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No 2

No 2 (17)

Editorial

Radu-Iulian Spataru
Emergency Clinical Hospital for Children „Maria Sklodowska Curie” Bucharest

 

 

In the last decade, cleft care in Romania has made significant steps towards progress and innovation. An important role in this process is our consistent collaboration along with the exchange of knowledge and experience with foreign partners through several European projects. Therefore, we came together and helped extend the existing European network of cleft professionals, so we can learn from each other and provide a better care for cleft patients.

Our dedication to care for children with cleft lip and palate at the Emergency Clinical Hospital for Children „Maria Sklodowska Curie” in Bucharest, started many years ago with Prof.Dr. Pesamosca who, over the years, gained a broad experience. Since 1984, the surgical protocol for cleft palate has been based on the Von Langerbeck principles with children being operated between 18 and 24 months. The delayed repair had minor disturbances on facial growth but in the cases with wide cleft defects, a high percentage of fistulas were noted and also abnormal speech due to short length and limited mobility of the soft palate.

In 2009, the protocol was replaced with the Gothenburg Delayed Hard Palate Closure (DHPC) technique. The transfer to the DHCP technique was performed in steps. We visited the cleft team in Gothenburg and the cleft team from Gothenburg (surgeons - Hans Mark and Jan Lidia, speech therapists, orthodontics) visited the cleft team in Bucharest twice every year for 5 years. This has been more than a teaching process. We set the first building bricks for a strong collaboration, the starting point to create a “twining cleft center” with a multidisciplinary team approach to treat patients with cleft lip and palate in Romania. A series of workshops were organized with national and international participation. During one of such meeting, together with colleagues from Iasi, Brasov, Timisoara and Craiova, the Romania Cleft Society was born in 2012.

The efforts to care and promote a multidisciplinary team continued with a multi institutional cooperation project called “Development of multidisciplinary innovative strategies for the care of the Romanian individuals with cleft lip and/or palate” having as partners the University of Medicine and Pharmacy “Carol Davila” from Bucharest, University of Oslo and Romania Cleft. The role of this project was to understand and apply the multidisciplinary approach of cleft lip and palate treatment, protocols of good practice taught by the members of the Cleft Team of University of Oslo (one of the most experienced cleft team around the globe); to follow and implement the Norwegian follow-up strategies of the cleft patients, to elaborate and to implement the management strategies of lip and palate malformations in Bucharest and the main centers in Romania. The result of this project was the elaboration of a Romanian guide of good practice regarding the multidisciplinary approach in cleft lip and palate called “Despicaturile labio-maxilo-palatine: diagnostic si tratament multidisciplinar”, an informative guide for the Romanian parents “Ingrijirea copiilor cu despicaturi labio-maxilo-palatine” and the organization of an international conference in Bucharest “Development of Multidisciplinary Innovative Strategies for the Care of the Romanian Individual with Cleft Lip and/or Palate”. This was followed by a COST European Cooperation in Science and Technology project called “Appearance Matters” with the aim to ensure that children born with orofacial clefts and other cranio- facial conditions receive optimum multidisciplinary care enabling them to grow up like any other children and attain equal status within their society. The project involved 34 countries over a 4 years period.

The 2nd COST project – European Cleft and Craniofacial Initiative for Equality in Care, with participation of 20 European countries including Romania has the aim to ensure that children born with orofacial clefts and other craniofacial conditions receive optimum multidisciplinary care enabling them to grow up like any other child and attain equal status within society. The Action will work with COST Inclusiveness Target Countries where limited or no national protocols exist in cleft and craniofacial care and will, via healthcare research, develop health-integrated networks which will manage and oversee the development of cleft and craniofacial services.

Currently, we became partners in 2 other projects: ERASMUS + “Cutting Edge Training - Optimizing medical outcomes for patients undergoing appearance altering procedures via innovative training of healthcare professionals” and “Early care training programme for health professionals working with children born with orofacial cleft”. The 1st project is based on the observation that there is a substantial need for the development of high quality work in the healthcare sector especially in the care of patients undergoing appearance altering procedures that results in scarring and body form changes (such as cancer, burns, limb loss, mastectomy, plastic surgery, birth defects). The paramount need to train healthcare professionals in the psychological and psychiatric aspects of care is crucial for the individuals undergoing appearance altering procedures.

The 2nd project has the aim to develop an early training programme for health professionals working with children with clefts and other craniofacial conditions. The participants will learn the necessary specialist skills to assess the infant, to determine whether there are any other associated anomalies needing specialized support and intervention, and formulate a treatment plan, that allows the infant to thrive normally.

During the same time other centers in Romania such as Brasov, Timisoara and Craiova became actively involved in raising awareness between healthcare professionals regarding multidisciplinary treatment of cleft patients. As an example, in 2015 Eduard Paraschivescu, a well renowned orthodontist, organized on international workshop in Timisoara and in 2017 during the National Conference for Pediatric Surgeons, an all-day session was dedicated to the care of patients with cleft lip and palate which was attended by a large number of international experts in cleft care.

In the last years our active involvement to care for children with cleft lip and palate has increased considerably with high impact on the life of our patients. The birth of Romania Cleft has been a turning moment in our progress pathway, but it needs to be constantly sustained by collaborations between all other centers from the country. We need to promote multidisciplinary treatment, continue to develop partnerships with European centers and support the long term needs of children and their families.

Catarina Ladeira, Aline Vaz-Silva, João Pascoal

Department of Pediatric Surgery, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central EPE. Lisboa, Portugal

 

Correspondence:

Catarina Ladeira
Avenida do Brasil, nº30, 6ºD
1700-070 Lisboa, Portugal
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Benign subcutaneous emphysema is a rare condition that commonly occurs secondary to trauma. We present a case of a child with a post-traumatic subcutaneous emphysema of the lower limb wrongly diagnosed as a necrotizing fasciitis. It is important to differentiate benign subcutaneous emphysema from serious soft-tissue infections such as gas gangrene and necrotizing fasciitis as the management, treatment and prognosis differ significantly.

Keywords: benign subcutaneous emphysema, necrotizing fasciitis

 

 

Introduction

Subcutaneous emphysema is defined as the presence of gas or air in the subcutaneous tissue plane [1]. It is usually associated with gas gangrene or necrotizing fasciitis, however not all subcutaneous emphysema signifies a life-threatening infection [2]. Benign subcutaneous emphysema is a rare condition that commonly occurs secondary to trauma [3].

Case report

We present the case of an 11-year-old female who was transferred to our hospital with a diagnosis of necrotizing fasciitis. She had suffered a puncture wound to the foot with a piece of glass. A few minutes later her mother noticed some crepitation on palpation of the leg and took her to the emergency room. On admission the doctor identified subcutaneous emphysema on palpation without any other signs of infection. Due to the presence of subcutaneous emphysema the diagnosis of necrotizing fasciitis was made. The girl was put on clindamycin, meropenem and vancomycin and transferred to our hospital.

On arrival she looked well, was hemodynamically stable and afebrile. Inspection of the left lower limb showed a puncture wound on the external malleolus, with discrete swelling and tenderness around it. Crepitation could be felt in the area and extending anteriorly and posteriorly all around the tibio-tarsal joint and superiorly to the lower third of the leg. The X-Ray showed air in the subcutaneous tissue, the ultrasound described air in the subcutaneous tissue without signs of necrotizing fasciitis and the laboratory work showed no alterations compatible with infection (Fig.1). The girl was discharged on the same day, medicated with oral flucloxacillin and was re-evaluated three days later showing no signs of complications.

Figure 1. X-Ray showing subcutaneous emphysema

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Discussion

Benign subcutaneous emphysema of the hand and upper limb of non-infectious aetiology has been documented in several case reports [2]. To the authors knowledge this is the first case in literatureregarding the lower limb and affecting children.

Subcutaneous emphysema can result from numerous benign causes, most of which are trauma related [4]. Reported causes to date include; high pressure injuries from power tools, blast injuries, air-gun injuries, dental extractions, hawthorn injuries, simple abrasive injuries (bike trauma, falls, etc.), insect bites and self-harm (factitious emphysema) [2,5–7]. Other rare causes include iatrogenic injuries (post-biopsy or insertion of peripheral vascular access) [3]. Speculation regarding mechanism of injury is that the trauma/puncture injury results in a one-way ball-valve effect causing accumulation of gas in the subcutaneous plane [2,8].

It is important to differentiate benign subcutaneous emphysema from serious soft-tissue infections such as gas gangrene (clostridial myonecrosis) and necrotizing fasciitis [9], especially in the early stages, as the management approaches vary significantly [1]. Benign subcutaneous emphysema is limited to the subcutaneous tissue, without systemic symptoms, and is more common in the upper extremities [1,2].

Some authors point out that infection from gas-forming organisms usually takes 12–18 h to develop and that gas in the subcutaneous tissue within 6–10 h of trauma is most likely to be indicative of a benign process [10,11]. Conservative treatment has been suggested for subcutaneous emphysema in patients who are systemically well, with minimal pain at the site of injury and in the absence of extensive cellulitis.In this cases it is acceptable to reassure the patient and discharge on oral antibiotics with an outpatient follow up appointment [2].

Conclusion

Benign causes of subcutaneous emphysema should be considered in patients presenting without any signs and symptoms of an infectious process or systemic illness. This will avoid the unnecessary aggressive treatment which is reserved for gas gangrene and necrotizing fasciitis.

 

 

References:

1. Onwochei VE, Kelly ME, Lyons R, Khan W, Barry KM. Benign subcutaneous emphysema: A case report with bite. Int J Surg Case Rep. 2015;9:89-91.

2. Fox A, Sheick H, Ekwobi C, Ho-Asjoe M. Benign surgical emphysema of the hand and upper limb: gas is not always gangrene - a report of two cases. Emerg Med J. 2007 Nov;24(11):798-9.

3. Yadav P, Benign subcutaneous emphysema following punch biopsy. J CutanAesthet Surg2013;6: 171–172.

4. Ozalay M, Akpinar S, Hersekli MA, et al. Benign subcutaneous emphysema of the hand. Arch. Orthop Trauma Surg 2003;203: 433–435.

5. Caspi I, Lin E, Nerubay J, et al. Subcutaneous emphysema following high-pressure injection injury of inert gas. J Trauma 1987;27: 1305–1306.

6. Eyres KS, Morley T. Subcutaneous emphysema of the upper limb: an air-gun injury. J Hand Surg1993;18: 251–253.

7. Kay PR, Kenny NW, Paul AIM, et al. Self-inflicted injection of air into the hand. Br J ClinPract1995;48: 336.

8. Winshall JS, Weissman BN. Benign subcutaneous emphysema of the upper extremity. N Engl J Med 2005;13: 352.

9. MisiakosEP, Bagias G, Patapis P, et al. Current concepts in the management of necrotizing fasciitis. FrontSurg2014;29: 1.

10. Filler RM, Griscom NT, Pappas A. Post-traumatic crepitation falsely suggesting gas gangrene. N Engl J Med 1968;278:758–61.

11. Butt M, Hird GF. Surgical emphysema of the dorsum of the hand. J Hand Surg 1990;15B:379–80.

Ahmed H. Al-Salem

Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia

 

Correspondence:

Ahmed Al-Salem

P.O. Box 61015

Qatif 31911, Saudi Arabia

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Mobile: 966505818009

 

Abstract

Congenital rectal atresia is an extremely rare malformation. It is characterized by a normally placed anus and well-developed sphincter muscles. There is no well established surgical technique to treat congenital rectal atresia and several operative procedures were described. We report four cases of congenital rectal atresia. The different surgical techniques are also discussed.

Keywords: congenital rectal atresia, treatment

 

Introduction

Congenital rectal atresia is a very rare condition that constitutes 1% - 2% of all anorectal malformations [1]. There is no standard surgical approach to manage congenital rectal atresia and several surgical techniques have been described [2 - 8]. This report describes our experience with four patients with congenital rectal atresia with emphasis on the different surgical techniques.

Case Series

Case 1

A full-term male newborn was referred to our hospital with abdominal distension and failure to pass meconium. Clinically, there were no other abnormalities apart from abdominal distension. Perineal examination revealed a normally looking anus; but a Foley's catheter could only be introduced into the anus for about 3 cm, suggesting rectal atresia. His abdominal X-ray showed dilated bowel loops. A contrast enema confirmed the diagnosis of congenital rectal atresia (Fig. 1A and 1B). He initially had a sigmoid colostomy and at the age of four months he underwent a posterior sagittal anorectoplasty to repair the rectal atresia. The superficial and deep layers of the striated muscle complex and levator ani were divided in the midline to explore the rectal pouch and continuity of the rectum was established by circumferential anastomosis after dividing the septum between the two pouches. Anal dilatation was carried out on the second postoperative week and four weeks later, the colostomy was closed. The patient was followed up in the clinic and the last time seen he was 2.5 years old passing stools daily with occasional constipation requiring suppositories from time to time.

Figure 1. A: Contrast enema showing congenital rectal atresia marked by the arrows. Note the dilated bowel loops. B: A lateral abdominal radiograph with a contrast enema showing congenital rectal atresia (marked by the arrows).

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Case 2

A 3 1/2-year-old girl was referred to our hospital with soiling her pants. One week prior to presentation she started to complain of abdominal pain and distension. Clinically, she was found to have mild abdominal distension with palpable fecal masses in the abdomen. Rectal examination revealed an empty rectum, but there was a large fecaloma in the pelvis. Several rectal washouts were done but did not clear the fecaloma totally and a repeat rectal examination showed the fecaloma in the proximal part of the rectum immediately above a rectal diaphragm with a central hole. This was confirmed with a barium enema and the diaphragm was about 4- 5 cm from the dentate line (Fig. 2). The washouts were continued till she was empty.

Figure 2. A lateral photograph of a barium enema showing congenital rectal stenosis (marked by arrows)

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She was operated on via a suprapubic transverse incision. The upper rectum and sigmoid colon were hypertrophied and dilated and the rectum was tapering down to the site of narrowing in the lower part. The lower sigmoid colon was opened longitudinally and the septum with the central hole was visualized. It was decided to resect this using an end-to-end anastomosis (EEA) stapler (#28). This was passed from the anus without the anvil and guided through the hole in the diaphragm and then out through the colotomy in the sigmoid colon. The anvil was screwed on the shaft of the EEA until it was flush at the site of the central hole where the gun was fired. The head was then released, and the entire EEA was recovered from the anus. A complete disc of tissue with a central hole was recovered (Fig. 3). The integrity of the anastomosis was confirmed and the colotomy was closed longitudinally. Histology of the resected part was lined by stratified sequamous non-keratinizing epithelium on the anal side and by rectal mucosa on the rectal side with an abrupt junction between the two. Beneath the epithelium there were striated muscle bundles arranged in a haphazard way without any clearly defined muscularis mucosa. Post operatively, she did well and was passing stools easily without soiling but occasionally she requires suppositories for constipation. Rectal examination five months later revealed an adequate anal opening at the anastomosis site.

Figure 3. Diagrammatic representation of the operative treatment of congenital rectal stenosis using end-to-end anastomosis stapler (EEA) which is marked by the green arrow. The site of congenital rectal stenosis is marked by the red arrow, the anvil of the EEA is marked with blue arrow and the site of the colotomy is marked with a black arrow.

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Case 3

A full-term male newborn was referred to our hospital because of abdominal distension and failure to pass meconium. He was a product of full term normal vaginal delivery. Clinically, there was abdominal distension but no other abnormalities. The anus was normally looking and at its normal position. A catheter was introduced into the anus but failed to pass distally and only about 4 cm of it could be introduced. The diagnosis of congenital rectal atresia was made and he underwent a defunctioning sigmoid colostomy. Post-operatively, he did well and started to pass meconium. His abdominal distension decreased gradually and was discharged home two weeks later. Unfortunately, he died at home at the age of two months from unknown reasons.

Case 4

A full-term male newborn, a product of normal vaginal delivery was sent home to be readmitted two days later to our hospital with abdominal distension and failure to pass meconium. Clinically, he had abdominal distension and a normally looking anus (Fig. 4). A Foley's catheter was passed into the anus but only up to five centimeters from the anal opening. The diagnosis of congenital rectal atresia was made and a sigmoid loop colostomy was done.

Figure 4. A clinical photograph showing a normal looking anus in a patient with congenital rectal atresia.

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Post-operatively, he did well and was discharged home to be followed up in the clinic. At the age of five months, he had a distal loop colostogram and a dilator was passed from below to demonstrate the length of the lower pouch as well as the gap between the two pouches (Fig. 5). It was decided to repair this via a limited posterior sagittal approach without dividing the muscle complex (Fig. 6). With the patient in the jackknife position, the site of congenital rectal atresia was exposed through a limited posterior saggittal incision away from the anal orifice and without excising the coccyx. A Hegar dilator was introduced into the distal part of the rectum via the colostomy and another one was introduced from the anal opening. The two dilators were pushed against each other to delineate the site of rectal atresia. The two pouches were opened under vision, the septum was excised and continuity of the rectum was established by circumferential anastomosis. Postoperatively, he did well and two weeks later he underwent regular dilation. There was a slight stricture at the site of anastomosis which was easily dilated. The colostomy was closed six weeks later and now he is five months post closure of colostomy and doing well.

Figure 5. Distal loop gram with a dilator in the anus showing congenital rectal atresia with a short gap between the two pouches

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Figure 6. Intra-operative photograph showing the site of the rectal atresia marked by the large arrows. Note also the extent of the incision to treat congenital rectal atresia via a posterior sagittal approach to avoid division of the muscle complex. The site of the normal anus is marked by the small arrows.

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Discussion

Congenital rectal atresia is a very rare type of anorectal malformations. Its incidence is low worldwide and comprises about 1%-2% of all anorectal malformations [1]. It is much more common in males with a male: female ratio of 7:1. The largest series of congenital rectal atresia was reported by Dorairjan from the southern part of India (State of Tamilnadu) where it constitutes about 14% of all anorectal malformations [9]. He reported 147 cases of congenital rectal atresia but only three of them had congenital rectal stenosis. The reason for this high incidence of congenital rectal atresia in that part of the world is not known.

Congenital rectal atresia is considered a rare variant of anorectal malformations but there are different types depending on the classification used. Dorairjan, based on the distance between the proximal rectum and distal anorectum, classified congenital rectal atresia into four grades: grade 1 – rectal atresia with a short gap between each end. This is the commonest variety. Grade 2- rectal atresia with a long gap, grade 3- membranous septal type, and grade 4 – rectal stenosis [9]. Gupta and Sharma on the other hand, classified congenital rectal atresia into five types. Type I is rectal stenosis, type II is rectal atresia with a septal defect, type III is rectal atresia with a fibrous cord between the two atretic ends, type IV is rectal atresia with a gap and type V is multiple rectal atresia with stenosis (A) or without stenosis (B) [1].

Congenital rectal atresia in contrast to anorectal malformations is characterized by a normally looking anus and the anal canal and lower rectum are surrounded by a normally developed sphincter and hence a good functional outcome is expected postoperatively. Unlike other anorectal malformations, congenital rectal atresia usually has no associated fistula communication with the urogenital system [1]. Lee et al. in 2007 described a case of congenital rectal atresia with rectourethral fistula [6]. Kulshrestha in 1997 reported a female with congenital rectal atresia and rectovestibular fistula [10]. Sharma et al. reported a case of rectal atresia with rectolabial fistula [11]. Saxena et al. reported a case of rectal atresia, choanal atresia and congenital heart disease [12]. These cases are the exception rather than the role as congenital rectal atresia commonly is an isolated malformation without any other additional congenital anomalies.

The treatment of congenital rectal atresia is controversial and several operative procedures have been described. These include simple perforation of the membrane [11], transanal end-to-end rectorectal anastomosis [3], mucosal proctectomy and coloanal anastomosis [7], posterior sagittal anorectoplasty [2, 6], Duhamel pull-through [5] and laparoscopic with transanal approach [8]. Since the anal canal and lower rectum are usually well developed and are surrounded by a normal sphincter, the long-term prognosis of these patients is excellent in term of bowel control and continence and every attempt should be made to preserve the sphincteric mechanism.

Although the reports on clinical results indicate that division of the sphincteric mechanism does not harm the function of the voluntary muscles there are major concerns about division of all the muscular structures as in the posterior sagittal anorectoplasty. This is especially so in patients with congenital rectal atresia where in contrast to high anorectal agenesis, the sphincter muscles are well developed. There is a definite increased exposure and definition of the entire sphincteric muscles by the posterior sagittal anorectoplasty. This is in contrast to the Stephens sacroperineal approach where the exposure is limited. Posterior sagittal anorectoplasty is also superior to the sacroperineal-sacroabdominoperineal pull-through [13].

Our patients were operated on differently. One of them (the first patient we saw) had a posterior sagittal anorectoplasty, while the other one had a limited posterior sagittal approach with preservation of the muscle complex. We feel this approach is feasible to repair congenital rectal atresia without dividing the muscle complex. Dividing the muscle complex is unnecessary to repair congenital rectal atresia and should be avoided. The exposure in our technique is adequate and the operative field is satisfactory. The pathology in congenital rectal atresia is localized and a limited exposure is sufficient to repair it. This is facilitated by the use of two dilators one passed from the distal loop of the colostomy and another passed through the anal canal. The third patient with congenital rectal stenosis in our series was treated using end-to-end anastomosis (EEA) to resect the rectal diaphragm with a hole. This approach is also safe and can be used to treat patients with congenital rectal diaphragm with a hole without interfering with the anatomy and function of the sphincters.

 

 

References:

1. Devendra K. Gupta, Shilpa Sharma. Rectal atresia and rectal ectasia. In: Alexander M. Hohlschneider and John M. Hustson, editors. Anorectal Malformations in Children Embryology, Diagnosis, Surgical Treatment, Follow-up. Springer Berlin Heidelberg 2006, p: 223-230.

2. Kisra M, Alkadi H, Ettayebi F, Benhammou M. Rectal atresia. J Pediatr Child Health 2005; 41:691-693.

3. Upadhyaya P. Rectal atresia: transanal, end-to-end, rectorectal anastomosis: a simplified, rational approach to management. J Pediatr Surg 1990; 25:535-537.

4. Sharma AK, Chaturvedi V, Wakhlu A. Anal agenesis associated with rectal atresia. J Pediatr Surg. 1995; 30(1):113-4.

5. Zia-w-Miraj Ahmad M, Brereton RJ, Huskisson L. Rectal atresia and stenosis. J Pediatr Surg. 1995; 30(11):1546-50.

6. Chien-Hsing Lee, Ming-Chih Chou, Hui-Min Yeh. Rectal atresia with rectourethral fistula: a rare anomaly. World J Pediatr 2007; 3(2):150-151.

7. Vaneerdeweg W, Hubens G, Deprettere A. Mucosal proctectomy and colo-anal anastomosis as treatment of rectal atresia. J Pediatr Surg 1995; 30: 1722-1723.

8. Nguyen TL, Pham DH. Laparoscopic and transanal approach for rectal atresia: a novel alternative. J Pediatr Surg. 2007; 42(11):E25-7.

9. Dorairajan T. Anorectal atresia. In: Stephens FD, Smith ED, Paul NW, editors. Anorectal malformations in children. New York, Liss, 1988, p: 105-110.

10. Kulshrestha S, Gangopadhyay AN, Sahoo SP. An unusual variant of rectal atresia with rectovestibular fistula. J Pediatr Surg 1997; 32:921-922.

11. Sharma SP, Upadhyaya A, Pandey A, Gangopadhyay AN. Rectal atresia with rectolabial fistula. J Indian Assoc Pediatr Surg 2008, 13: 75-76.

12. Saxena AK, Morcate JJ, Schleef J, Reich A, Willital GH. Rectal atresia, choanal atresia and congenital heart disease: a rare association. Tec Health Care 2004; 12:343-345.

13. Rintala RJ, Lindahl HG. Posterior sagittal anorectoplasty is superior to sacroperineal-sacroabdominoperineal pull-through: a long-term follow-up study in boys with high anorectal anomalies. J Pediatr Surg 1999; 34: 334–337.

Apoorva Kulkarni, Abhaya Gupta, Vishesh Dikshit, Paras Kothari

Department of Pediatric Surgery, Lokmanya Tilak Municipal Medical College and Government Hospital, Mumbai, India

 

Correspondence:

Apoorva Kulkarni

13 Akshay, KC Road, Bandra Reclamation, Bandra (W)

Mumbai-400050, Maharashtra, India

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

A 4-month-old male was referred to our tertiary care hospital with repeated episodes of lower respiratory tract infections and respiratory distress. The patient was operated for congenital lobar emphysema of right lung upper lobe at a private hospital 2 months prior. Patient developed respiratory distress again post operatively and had to be ventilated for 4 days. A high resolution contrast tomography (HRCT) of thorax was done which revealed emphysema of middle lobe of right lung. Patient underwent right middle lobe lobectomy via postero-lateral thoracotomy. Patient was relieved of respiratory distress post operatively and was discharged on post-operative day 7 after removal of intercostal drain.

Keywords: congenital, bilobar, emphysema, thoracotomy

 

Introduction

Congenital lobar emphysema is a developmental anomaly of lung causing over-inflation of one or more lung lobes. A rare variety results in emphysema of two lobes either of the same or of both lungs. The patient generally presents with respiratory distress requiring surgery. Both affected lobes can be removed either simultaneously or one after the other [1]. We describe a case where affection of the two lobes was discovered one after the other, both requiring removal.

Case report

A 4-month-old male was referred to our institution with a history of respiratory distress and previous episodes of lower respiratory tract infections. The patient has been previously operated for emphysema of upper lobe of right lung and an upper lobe lobectomy of the right lung was performed 2 months back in a private hospital. After the surgery, the patient was symptomatic with episodes of cough and respiratory distress, so much so that he had to be ventilated for a few days in the same private hospital. On examination, he had subcostal retractions but he was maintaining good oxygen saturation on nasal prongs.

Before surgery at the private hospital, X-ray of the chest revealed a right sided emphysema (Fig. 1). In our institution, we ordered a thoracic HRCT (high resolution contrast tomography) (Fig. 2 and 3) which revealed hyperinflation of the medial segment of right middle lobe with retrosternal extension and hyperinflation of lateral segment of right middle lobe reaching upto apical pleura posterior to the right upper lobe.

Figure 1. Pre- surgery chest radiograph

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The patient was scheduled for elective surgery. A posterolateral thoracotomy was done and the middle lobe, found to be emphysematous, was removed. An intercostal drainage was placed. It was removed on post-operative day 5. The patient was kept under observation for respiratory distress for 2 days following the removal of the chest tube and then discharged with no symptoms. The patient had followed up once one month after surgery and had no recurrence of symptoms since surgery.

Figure 2. Transverse section of CT thorax

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Figure 3. Longitudinal section of CT thorax

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Discussion

Congenital lobar emphysema (CLE) is a rare anomaly of lung development in which there is progressive over-inflation of one or more lung lobes [1]. It is usually unilateral and the most commonly affected lobe is the left upper (42%), followed by the right middle (35%) but any lung lobe can be affected. Bilobar involvement can affect the same lung or both lungs. Both are extremely rare and have been reported only in few cases in the literature [2]. There have been 7 cases of bilateral congenital lobar emphysema recorded in literature till date. Surgery was required mainly because of respiratory distress. The symptoms were successfully relieved with surgery. Lobar emphysema involving two lobes of the same lung has been observed only in 5 other cases in addition to ours [3].

The clinical presentation varies from asymptomatic to severe respiratory distress. Symptoms after the neonatal period occur mainly due to recurrent respiratory tract infections, resulting in cough, fever and intermittent dyspnea [4]. When a single lobe is involved, treatment of choice is conservative, if patient is asymptomatic. If symptoms persist, the patient requires resection of the affected lobe. In case of bilobar involvement affecting both the lungs, some authors advocate two-step sequential resection of both lobes [5], although there are authors who also propose simultaneous bilateral thoracotomies to resect both lobes at the same time [6]. In case of both lobes affecting the same lung, it is generally suggested that the worse affected be removed first and the other kept under observation [7]. But in our case, the situation was different. The middle lobe of right lung was found to be affected after the resection of upper lobe had already been done 2 months prior. There have been only 5 cases of bilobar emphysema reported in literature and no other case similar to ours. Our patient has been following up with us and has no more respiratory distress.

 

References:

1. Abel RM, Bush A. Congenital Lung Disease. In: Victor Chernick, Editor. Kendig’s disorders of the respiratory tract in children. 7th Ed. Philadelphia. Saudners; 2006. p. 280-316.

2. Floyd FW, Repici AJ, Gibson ET, Mcgeorge CK. Bilateral Congenital lobar emphysema surgically corrected. Pediatrics 1963;1:87-96.

3. Jesús Gutiérrez-Escobedo, Pedro Salvador Jiménez. Urueta. Congenital bilobar emphysema: An unusual presentation and literature review. PerinatolReprod Hum 2013; 27 (2): 129-133.

4. Schwartz MZ, Ramachandran P. Congenital malformations of the lung and mediastinum. A quarter century of experience from a single institution. J Pediatr Surg. 1997; 32: 44-77.

5. Maiya S, Clarke JR, More B, Desai M, Parikh D. Bilateral congenital lobar emphysema: How should we proceed? Pediatr Surg Int 2005;21:659-61.

6. Iodice F, Harban F, Walker I. Anesthetic management of a patient with bilateral congenital lobar emphysema. Paediatr Anaesth 2008; 18:340-1.

7. Hugosson C, Rabeeah A, Al-Rawaf R. Congenital bilobar emphysema. Pediatr Radiol. 1995; 25: 649-651.

Radu-Iulian Spataru, Dan-Alexandru Iozsa

Pediatric Surgery Unit, „Marie Sklodowska Curie” Emergency Clinical Hospital for Children, Bucharest, Romania

 

Correspondence:

Radu-Iulian Spataru

Pediatric Surgery Unit

„Marie Sklodowska Curie” Emergency Clinical Hospital for Children

20 C-tin Brâncoveanu Blvd., 041451, Bucharest, Romania

Phone: 0040788372478

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Spontaneous closure of the abdominal wall defect is a rare occurrence in the antenatal evolution of gastroschisis. This can compromise mesenteric blood supply to the extra-abdominal gut affecting the bowel in different forms from ischemia to necrosis. Intestinal atresia in a variable range is also known to coexist. We report the case of a newborn male admitted in our unit for neonatal bowel obstruction and a “mummified” tissue mass attached on the right side to the umbilical cord. Laparotomy revealed short length jejunal atresia and jejuno-ileal anastomosis has been performed. Despite the fact closed gastroschisis is frequently reported in association with short-bowel syndrome due to a vanishing midgut, our case had uneventful outcome.

Keywords: closed gastroschisis, intestinal atresia, neonatal bowel obstruction

 

 

Introduction

Gastroschisis (GS) is a congenital malformation in which the bowel (and occasionally other viscera) prolapses outside the abdominal cavity via a fullthickness wall defect, without a covering, most of the times right-sided to the umbilical cord which appears to be normal [1,2]. Gastroschisis occurs in very early gestation. Young mothers who do not follow proper prenatal care represent a group at risk. Development of early antenatal diagnosis together with improvement of postnatal treatment and increased survival in the last decades seems to have increased incidence of GS to 4-5:10,000 live births. This could be explained by a drop in the number of abortions, since certain risk factors aren’t clearly known [2,3]. Intestinal atresia and malrotation are well-documented associated conditions in GS [4].

In 6% of the GS cases antenatal closure of the abdominal wall may happen, condition being called closing/closed GS. This can lead to a “tourniquet effect” around the prolapsed bowel and a variable degree of vascular compromise of adjacent mesenteric vessels depending on the tightness of the abdominal ring. In exceptional cases, the abdominal wall may be entirely closed in conjunction with intestinal reabsorption due to severe chronic ischemia, situation known as vanishing midgut [5,6]. Nevertheless, intestinal atresia with consecutive short-bowel syndrome complications and sepsis remain the main risk factors for a poor outcome in GS [7].

Case Report

A full-term three-hour new-born male was admitted to our intensive care unit for neonatal bowel obstruction. No prenatal monitoring has been done and the baby was born via natural delivery in a secondary care center.

Clinical examination revealed abdominal distension and high output on the nasogastric tube. A „mummified” anhydrous aspect tissue, attached to a small circular scar through a short-narrow stalk was recognized just right to the umbilical cord (Fig. 1). No passage of meconium was noted so far.

Figure 1. Preoperative abdominal aspect

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Urgent midline laparotomy has been performed. A considerable dilated jejunum continued by 9 cm of type II atretic segment adjacent to a superior mesenteric artery branch vanishing into the abdominal wall could be observed (Fig. 2A and 2B). The distal bowel had a significantly reduced diameter. Exploration of the peritoneal cavity revealed approximately 80 cm of ileum and a normal length colon. Intestinal malrotation was noted. Maximum preservation of the small bowel in order to prevent intestinal failure was attempted with a jejuno-ileal anastomosis being done after resection of the atretic segment. The patient had an uneventful postoperative course tolerating early enteral feeds and no parenteral nutrition was needed (TPN). At 2 years follow-up, the patient has a normal height and weight gain.

Figure 2. Intraoperative aspect of the bowel illustrating complete jejunal atresia. Mesenteric artery branch vanishing into the abdominal wall.

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Discussion

The mechanisms of pathogenesis in GS are not clear and its relationship with intestinal atresia is not fully-understood [8]. Many assumptions are made in order to find a link between intestinal atresia and closing GS and the mainstay is the idea of a vascular accident in utero. This could be a spontaneous closure of the abdominal wall around the gut and mesenteric artery resulting into strangulation. Also, incarceration or volvulus within the closing defect and consecutive atrophy of the bowel are also postulated hypotheses [1,5,8].

During prenatal monitoring, closing GS main sign is a progressive and persistent dilatation of an intraabdominal bowel loop. This requires close observation and a good compliance of the mother since early delivery in order to salvage the bowel may be indicated when a high index of suspicion is considered [1].

Contrary to our situation, in many cases of closing/closed GS massive intestinal length is lost. Short-bowel syndrome demands further lengthening procedures and TPN with subsequent complications such as sepsis or intestinal failure associated liver disease [9]. However, in some cases partial compression can lead to “an amorphous nonviable appearing extra-corporeal mass of matted bowel” which some surgeons opt to resect considering it non-functional. Estrada JJ, et al. suggested reintegrating it into the abdominal cavity if a patent vascular pedicle is attached since a considerable segment development can surprise the surgeon in a second-look further intervention, so morbidity and mortality related to short-bowel syndrome may be reduced [3].

Completely closed abdominal wall defect in GS is extremely rare [8]. In 1999, Fowler CL, et al. reported an unique presentation of an antenatal closed gastroschisis with apparently normal abdominal wall outlined by a colo-cutaneous fistula which was proven by a barium enema performed for neonatal bowel obstruction differential diagnosis [10].

In our case, considering the narrow-stalk attaching the mummified bowel to the abdomen, the absence of antenatal monitoring of the pregnancy and the secondary care centre where our patient was delivered, if the necrotic external bowel would have been purposely or accidentally removed during or after birth, we simulated how the abdomen would have looked using computer assisted picture manipulation we illustrated the scenario (Fig. 3). We think that signs of neonatal bowel obstruction coexisting with a paraumbilical scar should be considered a suggestive sign for closed gastroschisis. In our case the circumstances that brought the most favourable outcomes were: early diagnosis and intervention, short bowel segment affected and localization to the jejunum, since this part is naturally shorter and exhibits less adaptive and absorptive capacity than the ileum [11].

Figure 3. Extra-abdominal mummified midgut removed using computer assisted picture manipulation

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References:

1. Houben C, Davenport M, Ade-Ajayi N, et al. Closing gastroschisis: diagnosis, management and outcomes. J Ped Surg 2009; 44: 343-7.

2. Holland AJ, Walker K, Badawi N. Gastroschisis: an update. Pediatr Surg Int 2010; 26: 871-8.

3. Estrada JJ, Petrosyan M, Hunter CJ. Preservation of extracorporeal tissue in closing gastroschisis augments intestinal length. J Ped Surg 2008; 43: 2213-5

4. Winter LW, Giuseppetti M, Breuer CK. A case report of midgut atresia and spontaneous closure of gastroschisis. Pediatr Surg Int 2005; 21: 415-6.

5. Davenport M, Haugen S, Greenough A, et al. Closed gastroschisis: antenatal and postnatal features. J Ped Surg 2001; 36: 1834-7.

6. Sisodiya RS, Panda SS, Gupta CK, et al. Closed gastroschisis with vanished small bowel and jejunal atresia. J Neonatal Surg 2016; 5: 65.

7. Kassa AM, Lilja HE. Predictors of postnatal outcome in neonates with gastroschisis. J Ped Surg 2011; 46: 2108-14.

8. Basaran UN, Inan M, Gűcer F, et al. Prenatally closed gastroschisis with midgut atresia. Pediatr Surg Int 2002; 18: 550-2.

9. Vogler SA, Fenton SJ, Scaife ER, et al. Closed gastroschisis: total parenteral nutrition-free survival with agressive attempts at bowel preservation and intestinal adaptation. J Ped Surg 2008; 43: 1006-10.

10. Fowler CL, Rothman EA, Congenital colocutaneous fistula as presenting sign of prenatally closed gastroschisis. Pediatr Radiol 1999; 29: 811-2.

11. Quiros-Tejeira, Ament ME, Reyen L, et al. Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome : a 25-year experience. J Pediatr 2004; 145: 157- 63.

Ali Ghribi, Manef Gasmi, Mourad Hamzaoui

Department of Pediatric Surgery, Children’s Hospital, Tunis El Manar University, Tunis, Tunisia

 

Correspondence:

Ali Ghribi

Department of Pediatric Surgery, Children’s Hospital, Tunis El Manar University

Place Bab Saadoun 1007 Tunis, Tunisia

Tel: 002167156810 Fax : 0021671562810

Email : This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Introduction: Priapism is persistent penile erection that is unrelated to sexual stimulation. Low-flow type is a urological emergency. The aim of this study is to illustrate our personal experience concerning the diagnostic and therapeutic management of priapism in children.

Patients and methods: A retrospective study was conducted involving 6 patients with priapism managed at our department, over a 20-year period, going from 1992 through 2012. Among them, 5 were because of sickle cell disease and 1 was of idiopathic origin in newborn.

Results: The study involved a 14-days-old neonate and 5 children whose ages ranged from 9 to 13 years. Mean duration of symptoms was 19.6 hours. The priapism was associated with confirmed sickle cell disease (SCD) and with newly diagnosed SCD in two others. It was of idiopathic origin in the newborn. The neonate was kept under observation and priapism disappeared after 18 hours without recurrence. In the older children, initial management was similar to that of other sickle cell crises (hyperhydration, oxygen, and analgesia). Because of the long duration of the priapism, emergency surgery was indicated under general anaesthesia. Successful detumescence was achieved after corporal aspiration in 4 patients. Caverno-glandular shunting, indicated after failure of corporal aspiration in one case. After surgery, spontaneous transient erection was self-reported by 4 patients without recurrence of priapism. One child presented after 6 months recurrence treated conservatively.

Conclusion: The first step of management of priapism is to distinguish ischemic from nonischemic type. Sickle cell disease is the commonest cause of ischemic priapism. Corporeal aspiration and modified Winter’s shunt were successful alternatives for patients with low-flow priapism. Management of idiopathic priapism in the newborn by observation alone seems to be safe. A long-term follow-up is necessary to assess erectile function.

Keywords: priapism, sickle cell disease, children, corporal aspiration

 

Introduction

Priapism is persistent penile erection for longer than 4 hours that is unaccompanied by sexual excitation. Low-flow or ischemic priapism is a urological emergency. Unlike high-flow priapism, it is excruciatingly painful and carries the risk of permanent impotence if treatment is delayed. A vast majority of the cases of priapism in children are due to sickle-cell disease. The aim of this study is to report our experience of management of six children with priapism over the past 20 years.

Patients and methods

A retrospective study was conducted involving 5 children and a neonate with priapism managed at the department of pediatric surgery of Tunis Children’s Hospital, over a 20-year period, going from 1992 through 2012. The priapism was associated with confirmed sickle cell disease (SCD)and with newly diagnosed SCD in two others. It was of idiopathic origin in the newborn. A workup for a secondary cause of priapism other than SCD was done including complete blood count, color Doppler ultrasonography (CDU) of the perineum and penis, and hemoglobin electrophoresis. Low-flow type priapism is characterized by painful rigid erection, a history of hemoglobinopathies or the presence of low-flow pattern in penile Doppler. Nonsurgical management was advocated only for the newborn with prolonged penile erection. The other patients underwent corporal aspiration (5 cases) or percutaneous caverno glanular shunt, Winter's shunt (1 case).

For corporal aspiration, a needle was inserted laterally at the 3 or 9 o’clock positions to avoid damaging the corpus spongiosum, the urethra and the dorsal neurovascular bundles. For the caverno glanular shunt, a needle was thrust into the corpora cavernosum through the glans. Multiple punctures are made in both corporal bodies, lateral to the urethra. After removing the needle, the puncture wound in the glans was closed with a single 6-0-catgut stitch. No patient received oral or intracorporeal alphasympathomimetics.

Results

The study involved 14-day-old neonate and 5 children whose ages ranged from 9 to 13 years (mean, 11.2 years). All of them presented with painful, rigid erection of penis that is characteristic of low-flow priapism. There were no signs of cyanosis in all cases. Mean duration of symptoms was 19.6 hours (range, 12-36 hours). Two children had been followed up, since early childhood, for homozygous sickle cell disease with chronic transfusion therapy programs and one for sickle β+ thalassemia with an enlarged spleen that required splenectomy for hypersplenism 2 years before. The 2 others hadn’t a history of SCD but in one case we found a history of unexplored anemia and jaundice. The other child reported that he had been the victim of a perineal trauma 7 days before the priapism occurred. CDU of the perineum and penis didn’t reveal anyarterio cavernous fistula. Homozygous SCD was confirmed by hemoglobin electrophoresis in these last two children. The newborn had no history of birth canal trauma or blood transfusion. Complete blood count didn’t show polycythemia (the hematocrit was at 39%) and penile CDU findings were normal. So, the patient was kept under observation and priapism disappeared after 18 hours without recurrence. After a follow-up period of 9 months, the penis was normal, and the mother reported periods with normal penile erection. In the older children, initial management was similar to that of other sickle cell crises (hyperhydration, oxygen, and analgesia). Because of the long duration of the priapism, emergency surgery was indicated under general anaesthesia. Successful detumescence was achieved after corporal aspiration in 4 patients (Fig. 1 and 2).

Figure1. Corporal aspiration. Two needles were inserted laterally at the 3 and 9 o’clock positions.

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Figure 2. Complete detumescence was achieved after corporal aspiration.

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Needle aspiration had to be repeated twice (in 2 cases) because of immediate recurrences. Caverno glandular shunting, indicated after failure of corporal aspiration in one case, produced improvement in cavernosal rigidity. Blood loss ranged from 100 to 150 ml and no patient required blood transfusion. One case of hematoma that developed in the subcutaneous tissue at the puncture site was controlled with direct pressure. After surgery, spontaneoustransient erection was self-reported by 4 patients without recurrence of priapism. One child presented after 6 months recurrence treated conservatively.

Discussion

There are two main kinds of priapism: low-flow ischaemic or veno-occlusive priapism, and highflow or arterial priapism that usually results from penile or perineal trauma injuring the wall of the cavernosal arteries and creating an arteriocavernous fistula [1-3]. The majority of cases of low-flow priapism are associated with SCD. Other predisposing factors including leukemia, lymphoma, Fabry’s disease, medication, and idiopathic causes have been reported. Stuttering priapism, describes recurrent priapism that usually due to SCD, lastes anywhere from minutes to just a few hours and typically resolves spontaneously. Neonatal priapism is also described, 18 cases have been reported [4]. It usually occurs in the first few days of life and persists for 2 to 12 days (average 5 days). Neonatal priapism is mostly idiopathic, like in our study. Identifiable etiologies are polycythemia, blood transfusions, congenital syphilis, cranial birth trauma, respiratory distress syndrome and umbilical artery catheterization.

There are no generalizable data on the prevalence of priapism in all children, which is considered rare. In children with sickle cell anemia, the incidence is reported to range from 6–27% and is higher in patients with HbSS [5]. Initial management aims to assess the type of priapism and to achieve detumescence. Low-flow priapism is typically painful with marked rigidity of the corpora cavernosa with a flaccid glans. Masturbation and nocturnal erections are common precipitators. However, high-flow priapism is characterized by painless, persistent semi-rigid erection. Perineal compression with the thumb resulting in immediate detumescence with relapse after withdrawal of the thumb is known as Piesis clinical sign and indicates high-flow priapism [1,2,6]. If there is difficulty in differentiating low-vs-high flow priapism, imaging studies or blood gas measurement from a cavernosal sample may help.

In ischaemic priapism CDU demonstrates a high resistance and low-flow arterial waveform. It detects nearly 100% of arteriosinusoidal fistulae in case of high-flow priapism. In blood gas analysis, values similar to arterial blood are indicative of high-flow priapism, whereas samples more consistent with venous blood sampling suggest the low-flow etiology [3]. Acute treatment for ischemic priapism should be instituted within hours given the increasing likelihood of cavernosal fibrosis and permanent erectile dysfunction (ED). Management involves corporeal aspiration, and phenylephrine injection to induce smooth muscle contraction and detumescence [3]. The corpora should be aspirated until bright red blood is seen (not exceeding 10% of the circulating blood volume). Simple intracorporeal aspiration will accomplish permanent detumescence in approximately half of patients. It was successful, in our study, in 80%. If conservative measures fail, penile shunt surgery should be performed.

Distal shunts, between corpora cavernosa and glans, are preferred over proximal ones (cavernospongiosal or cavernovenous). Distal shunt may be done either by open surgery (Al-Ghorab shunt) or by percutaneous techniques (Winter’s shunt).Several modifications of Winter's shunt have been introduced to get better results. Raveenthiran reported a modified technique using multiple punctures by a large bore needle in the tip of corpora cavernosa through the glans. The needle tracks functioned as temporary cavernoglandular fistula. This technique was almost always successful, like in our case report, and erectile capacity was retained in a high proportion of children (>71%)[7]. SCD patients may also benefit from hydration, blood transfusion,exchange transfusion, or hyperbaric oxygen. Non-operative management may be successful provided patients present early. Children with sickle cell anemia are at a higher risk of developing ED [7]. The lifetime probability of ED secondary to priapism is over 30%.

Expectant management in high-flow priapism is acceptable and safe. Because venous outflow is not compromised there is no damage to the corporal bodies. Up to 62% of non-ischemic priapism cases have spontaneous resolution [8]. For patients who request treatment or do not improve on observation, there are a few effective non-invasive options, like alpha adrenergic agonist injections, compression methods of the penile artery or compression of the fistula with a duplex ultrasound. Percutaneous transcatheter embolization is the most effective treatment.

Surgical options, involving resection of the fistula and ligation of the internal pudendal artery or cavernous artery are reserved for failed embolization attempts. The incidence of erectile dysfunction after embolization was about 25% [8]. Neonatal priapism may be treated less urgently. Initial evaluation of prolonged erections in newborns should include clinical examination and/or ultrasonography, full blood count, and C-reactive protein. The majorities of reported cases were idiopathic; they were managed conservatively with spontaneous resolution within a period of approximately 5 days. Phlebotomy and exchange transfusion for polycystic newborns were successful options. Priapism persisting for more than 5 days may be treated by ketamine hydrochloride anaesthesia or corporal aspiration. Long-term undesirable consequences, such as erectile dysfunction, have not been reported in the newborn group [9,10,11].

Conclusion

Priapism is a medical emergency commonly associated with significant long-term morbidity. The first step of management is to distinguish ischemic from nonischemic using findings from careful history taking, physical and finding from cavernosal blood gas determination and CDU examination.Sickle cell disease is the commonest cause of ischemic priapism in children and should be usually sought. There are many therapeutic modalities to achieve detumescence, depending on the etiology. Corporeal aspiration and modified Winter’s shunt were successful alternatives for patients with low-flow priapism who did not respond to conservative treatment. Management of priapism in the newborn by observational one seems to be safe. A long-term follow-up is necessary to assess erectile function.

 

 

References:

1. Mockford K, Weston M, Subramaniam R. Management of highflow priapism in paediatric patients: a case report and review of the literature. J Pediatr Urol2007; 3:404-12.

2. Volgger H, Pfefferkorn S, Hobisch A. Posttraumatic high-flow priapism in children: noninvasive treatment by color Doppler ultrasound- guided perineal compression. Urology 2007; 70:590.e3-5.

3. Donaldson JF, Rees RW, Steinbrecher HA. Priapism in children: a comprehensive review and clinical guideline. J Pediatr Urol2013; 5131:214-3

4. Palmer B, Frimberger D. Successful treatment of idiopathic priapism in a 13-year-old boy.J Pediatr Urol 2009; 5:145-6.

5. Vilke GM, Harrigan RA, Ufberg JW, Chan TC. Emergency evaluation and treatment of priapism.J Emerg Med 2004; 26:325-9.

6. Mahawong P, Srisuwan T. Idiopathic high-flow priapism in a pediatric patient. J Pediatr Urol 2011; 7:92-4.

7. Raveenthiran V. A modification of Winter's shunt in the treatment of pediatric low-flow priapism. J Pediatr Surg 2008; 43:2082-6.

8. Nabinger GB, Burttet LM, Lucena IR, et al. Child non-ischemic priapism, a conservative approach: case report and updated review. J Pediatr Urol 2013; 9:e99-101.

9. Burgu B, Talas H, Erdeve O, et al. Approach to newborn priapism: a rare entity. J Pediatr Urol 2007;3:509-11.

10. Walker JR, Casale AJ. Prolonged penile erection in the newborn. Urology.1997; 50:796-9

11. Meijer B, Bakker HH. Management of priapism in the newborn. Urology2003; 61:224

Sebastian Ionescu, Bogdan Andrei, Mihai Mocanu, Elena Licsandru, Daniela Pavel

Department of Pediatric Surgery, Emergency Clinical Hospital for Children “Marie S. Curie”, Bucharest, Romania

 

Correspondence:

Sebastian Ionescu

Department of Pediatric Surgery

Emergency Clinical Hospital for Children “Marie S. Curie”

20 Bd. Constantin Brancoveanu, Sector 4, 041451 Bucharest, Romania

Tel/fax: 021/4601040

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Aims: The most common complication in complete or partial pyeloureteral duplication is damage of the upper renal pole due to vesicoureteral reflux or ureterocele, condition that requires partial nephrectomy or ureteronephrectomy. The purpose of this study is to present and analyze the treatment results using the minimal invasive approach in complications of pyeloureteral duplication in children.

Materials and methods: We present 28 cases of upper pole nephrectomy in children with complete or partial pyeloureteral duplication. In all cases there was a non-functional renal upper pole due to the presence of vesicoureteral reflux in 20 cases and ureterocele in 8 cases. The laparoscopic approach was transperitoneal (in 26 cases) or retroperitoneal (in 2 cases). The mean age of the children was 6 (range 2-12). Preoperative evaluation included renal ultrasound, voiding cystourethrogram, intravenous urography, renal scintigraphy and blood analysis. We analyzed data concerning the operative time, the use of analgesics and antibiotics, the time to resume oral feeding, hospital stay and postoperative renal function.

Results: The mean operative time was 120 minutes but longer for the retroperitoneal approach. There were 2 types of complications: one intraoperative hemorrhage which required conversion and one postoperative urinoma treated with local drainage. Pain medication was required only in the first 2 days after surgery. The mean postoperative hospital stay was 4 days. The postoperative renal function of the lower pole, evaluated by blood samples (blood urea nitrogen, plasma creatinine) and scintigraphy was normal in all cases during 1 year postoperative follow-up.

Conclusion: Laparoscopic transperitoneal or retroperitoneal partial ureteronephrectomy is a safe and feasible procedure in children. We consider that laparoscopy offers a better visualization of the vessels to be ligated and the dissection plans. Using minimal access surgery, we had shorter postoperative recovery, good aesthetic postoperative scars and shorter hospital stay. For the reasons mentioned above, this technique is first choice over open partial nephrectomy.

Keywords: pyeloureteral duplication, minimally invasive, children, nephrectomy

 

Introduction

Nephrectomy, one of the first laparoscopic interventions performed the children, was reported for the first time in 1991 by Clayman [1] using a transperitoneal approach. Later on, in 1993 Jordan and Winslow [2] reported the first laparoscopic upper pole partial nephrectomy with ureterectomy. Soon after, laparoscopy became the first choice in the treatment of many renal duplex system complications in children [3].

The most common complication in complete or partial pyeloureteral duplication is damage of the upper renal pole due to vesicoureteral reflux or ureterocele, condition that requires partial nephrectomy / ureteronephrectomy [4].

The aim of this study is to analyze and report the results of minimal invasive treatment in 28 cases with non-functional renal upper pole, complications of complete pyeloureteral duplication, focusing on surgical indication, technical variants and functional postoperative results.

Materials and Methods

This retrospective, analytic study was performed in the Department of Pediatric Surgery of the Emergency Clinical Hospital for Children “Marie. S. Curie” between 2008-2014.

Preoperative evaluation was done using laboratory analysis (urea, creatinine, complete blood count, urinalysis and urine culture), renal ultrasound, voiding cystography and renal scintigraphy with DTPA (Fig. 1, 2, 3). Intravenous urography, computed tomography urography or magnetic resonance urography was performed in 7 patients for more accurate anatomical details. Indication for surgery was the presence of a nonfunctional upper pole, often with complete duplication of the ureter, dilated renal moiety and thinned renal parenchyma (1 mm-2 mm).

Figure 1. Renal scintigraphy with DTPA – non-functional left renal upper pole in complete pyeloureteral duplication with stenosis of the ureter

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Out of the 28 operated cases using the minimal invasive aproach, 26 were approached transperitoneal and 2 retroperitoneal. The upper moiety dilatation was caused by the presence of ureterocele in 8 cases and vesicoureteral reflux in 20 cases. Patient’s age ranged from 2-12 years, with an average of 6 years.

Figure 2. Voiding cystourethrography in a case with left pyeloureteral complete duplication with ureterocele of the upper moiety (A) and grade III vesicoureteral reflux in the lower moiety (B)

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Figure 3. Voiding cystourethrography – bilateral completepyeloureteral duplication with bilateral vesicoureteral reflux

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The operative technique for transperitoneal approach

The patient is positioned in lateral decubitus, with a side raised at about 45°. Pneumoperitoneum (10-12 mmHg) is obtained at a flow rate of 1- 2L/min through the umbilical trocar inserted by open access. The other three 5 mm trocars are placed in the epigastrium, right inferior abdominal flank or left inferior abdominal flank - on the lesion side and in the hypochondrium [5]. After incision of the peritoneum along the Toldt line, the colon is mobilized medially and Gerota fascia is opened. Dissection of the upper moiety together with the ureter is performed so as to avoid damage of the lower moiety and the ureter, followed by uncrossing the lower moiety vessels [6]. Next, we identify the upper moiety pedicle and transect it after clipping (in 6 cases) or using Ligasure (in 22 cases). The upper pole renal parenchyma is transected with electrocautery (6 cases) or with Ligasure (22 cases). We don’t perform the suture of the renal capsule above the sectioned upper kidney pole. The ureter of the upper moiety is sectioned close to the bladder in cases of vesicoureteral reflux, being ligated in advance or unligated in cases of ureteroceles. A drainage tube, in the renal fossa, was placed in all cases and was maintained between 1 and 3 days, depending on the drainage flow (Fig. 4).

In this study, we analyzed the operative time, intraoperative hemostasis problems, the need for analgesics and antibiotics, time to resume enteral nutrition, length of hospital stay and longterm functional evaluation of the remaining kidney.

Figure 4. Intraoperative aspects in complete pyeloureteral duplication with vesicoureteral reflux in the upper moiety during upper pole ureteronephrectomy by transperitoneal approach. (A) Upper ureter dissection from the vessels of the lower moiety. (B) Uncrossing the ureter from the lower moiety vessels. (C) The renal

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Results

From the total of 28 cases, the procedure was completed laparoscopically in 27 cases because one patient required conversion due to bleeding from the upper moiety hilum which could not be controlled with minimally invasive access. In the rest of the cases, the intraoperative blood loss was minimal.

Operating time ranged from 90-150 minutes with an average time of 120 minutes. One patient presented fever (38 °C) in the 5th postoperatively day, and ultrasonography revealed an urinoma with 7 cm diameter which required percutaneous drainage guided by ultrasound with a complete resolution after 4 days. All children received fluids orally in the night after surgery and were fed per mouth in the first postoperative day. Normal gastro-intestinal transit resumed 1-2 days after surgery.

The patients received intravenous antibiotics for 48 hours after surgery. In some cases, analgesics were administered orally 48 hours after surgery. The length of hospital stay was between 2 and 9 days, with an average of 4 days. Mean follow-up was 2 years, between 1 and 5 years. Urea and creatinine serum were in a normal range during follow up (1 month, 6 months, 12 months postoperatively), and the blood pressure was in normal range in all cases. In the cases associated with ureterocele, at the follow-up ultrasound, it was shown to be collapsed 1-6 months after surgery.

An ultrasound examination was performed after one month, 6 months, 12 months postoperatively, and then annually. Renal scintigraphy was performed after 6 months and it showed in all cases the maintenance or improvement of renal function, without damage of the remaining kidney. Aesthetic results were satisfactory for patients and family with no cases of abdominal wall complications or bowel obstruction.

Discussions

From early 90s, starting with the development and miniaturization of the laparoscopic instruments but also due to better understanding of the hemodynamic changes produced by pneumoperitoneum in children, indications for minimally invasive pediatric surgery have expanded continuously. Among these, the polar nephrectomy for renal congenital pathology emerged as a standard technique [7].

Heminephrectomy practiced using the open approach requires a large incision and mobilization of the entire kidney which can lead to damage of the remaining kidney [8].

During the minimal invasive technique, the kidney is approached "in situ" with a minimum mobilization, with better further results on the remaining kidney and obviously is virtually free of parietal complications. Good visualization of the vascular pedicle of the upper moiety allows sectioning of the demarcation between the two moieties with resection of the dysplastic upper renal pole and no damage of the residual kidney. Some authors recommend the use of ultrasonic dissector for parenchymal resection, but electrocautery may be used if vascular demarcation is clear. Ligasure is also a good option for vessels and parenchyma [9].

There is controversy over the transperitoneal or retroperitoneal approach. Proponents for the retroperitoneal approach believe that this provides good exposure of the posterior part of the kidney, avoiding the pedicle of the lower renal pole which can be left in place [10]. Retroperitoneal approach can be posterior or lateral. Posterior approach is preferred when ureterectomy is not necessary, the lateral approach providing better access for complete resection of the ureter [11]. The main disadvantages of the retroperitoneal approach are smaller workspace and a higher incidence of peritoneum perforation which prevents creation of an adequate retropneumoperitoneum and represents a cause of conversion [12].

On the other hand, the transperitoneal approach avoids these difficulties, providing a large working space, especially in young children (under 5 years), improving the surgeon’s comfort. We have obtained greater freedom of movement of the laparoscopic instruments, positioning the child on the edge of the operating table, on the surgeon and the cameraman side [13]. We prefer the transperitoneal approach, especially in young children, because the risk of intraoperative bowel injury is minimal and postoperative bowel obstruction is rare. In our series there were no complications related to the type of approach used. The patient who required conversion due to bleeding of the upper moiety vessels represented 5% of the total cases which is similar to results found in the literature. We also had a postoperative complication in a patient who underwent percutaneous drainage for urinoma in the upper renal pole; its frequency was also similar to data reported by other similar series [12].

The evaluation of postoperative pain in children is subjective, being hard to quantify, but the reduced need for analgesics was obvious. Antibiotic therapy was also administered only for 48 hours.

Most studies don’t show a statistically significant difference in the operative time, and when it is reported, depends on the learning curve [8]. We recorded an average duration of 2 hours in our patients and it is comparable with the average duration in the classic interventions. Postoperative follow-up (blood analysis, blood pressure, ultrasound and renal scintigraphy), showed no deterioration in any of these parameters, and about half of the patients showed an improvement in the remaining parenchyma at the renal scintigraphy at 6 months after surgery. This evolution is more probably due to reexpansion of the renal parenchyma, previously compressed by the dilated upper renal pole, than due to improving tubular function [14, 15].

Conclusion

Laparoscopic hemi-nephrectomy performed either trans or retroperitoneal is the first choice of treatment in children. Magnification provided by the telescope allows good highlighting of the upper renal pole structures, making the dissection safe and efficient. This approach allows distal section of the ureter without additional incisions and minimizes trauma to the vessels and the remaining lower renal parenchyma, reducing the possibility of long-term deterioration of the renal function. We prefer the transperitoneal approach, especially in young children because it offers a better working space, without complications such as intestinal lesions or postoperative bowel obstruction.

With this approach, postoperative recovery was shorter, there were no abdominal wall complications and the cosmetic results were satisfactory. For these reasons, we consider the laparoscopic approach as the first choice of treatment in children with indication for upper pole nephrectomy, because they can benefit from all the advantages of the minimal invasive technique.

 

 

References:

1. Clayman RV, Kavoussi LR, Soper NJ, Dierks SM, Meretyk Setal. Laparoscopic nephrectomy: initial case report. J Urol 1991;146(2): 278-82

2. Jordan GH1, Winslow BH. Laparoendoscopic upper pole partial nephrectomy with ureterectomy. J Urol 1993;150(3):940-3.

3. José M. Campero, Christián G. Ramos, Raúl Valdevenito, Alejandro Mercado, Juan Fullá.Laparoscopic partial nephrectomy: A series of one hundred cases performed by the same surgeon. Revista Chilena de Urologia 2015;80.

4. Ashcraft K. W, George W. Holcomb III, J. Patrick Murphy. Pediatric Surgery - 5th Edition. Saunders-Elsevier, Philadelphia 2009

5. Gao Z, Wu J, Lin C, Men C. Transperitoneal Laparoscopic Heminephrectomy in Duplex Kidney: Our Initial Experience. Urol 2011;77:231-36.

6. Abedinzadeh M, Nouralizadeh A, Radfar MH, Moslemi MK. Transperitoneal laparoscopic heminephrectomy in duplex kidneys: a one center experience. Ger Med Sci 2012;10:5.

7. Seibold J, Schilling D, Nagele U et al. Laparoscopic heminephroureterectomy for duplex kidney anomalies in the pediatric population. J Pediatr Urol 2008;4:345-47.

8. L. García-Aparicio, L. Krauel, X. Tarrado et al. Heminephroureterectomy for duplex kidney: laparoscopy versus open surgery. J Pediatr Urol 2010; 6 (2):157-160.

9. Singh RR, Wagener S, Chandran H. Laparoscopic management and outcomes in non-functioning moieties of duplex kidneys in children. J Pediatr Urol 2010; 6:66-69.

10. M. Castellan, R. Gosalbez, A. J. Carmack, J. C. Prieto, M. Perez- Brayfield, A. Labbie, Transperitoneal and retroperitoneal laparoscopic heminephrectomy-what approach for which patient?. J Pediatr Urol, 2006; 176 (6): 2636–2639.

11. Hamdan H. Al-hazmi, Hamzeh M. Farraj. Laparoscopic retroperitoneoscopic nephrectomy and partial nephrectomy in children. Urol Ann 2015; 7(2): 149–153.

12. Kieran K, Montgomery JS, Daignault S, Roberts WW, Wolf JS, Jr. Comparison of intraoperative parameters and perioperative complications of retroperitoneal and transperitoneal approaches to laparoscopic partial nephrectomy: support for a retroperitoneal approach in selected patients. J Endourol. 2007;21:754-59.

13. A. Schneider, M. Ripepi, C. Henry-Florence, and S. Geiss. Laparoscopic transperitoneal partial nephrectomy in children under 2 years old: a single-centre experience. J Pediatr Urol, 2010; 6 (2):166–170.

14. Jayram G, Roberts J, Hernandez A, et al. Outcomes and fate of the remnant moiety following laparoscopic heminephrectomy for duplex kidney: A multicenter review. J Pediatr Urol 2011;7:272-75.

15. Simmons MN, Hillyer SP, Lee BH, Fergany AF, Kaouk J, Campbell SC. Functional recovery after partial nephrectomy: Effects of volume loss and ischemic injury. J Urol. 2012;187:1667–73.

Kashif Chauhan, Omar Nasher, Richard WC Gan, Daniel W Colliver, Shailinder J Singh

Department of Paediatric Surgery, Nottingham University Hospitals NHS Trust, Queen’s Medical Centre, Nottingham, UK

 

Correspondence:

Kashif Chauhan

Paediatric Surgical Department

Nottingham University Hospital, Queen Mary Centre

Derby Road, Nottingham, UK

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Aims: Rectal prolapse has two main first line surgical treatments: injection sclerotherapy and Thiersch suture. We have used a technique in which injection sclerotherapy is combined with Thiersch suture as first line treatment. This study compares results between two groups of patients: 1) injection treatment alone and 2) injection treatment combined with Thiersch suture.

Method: A retrospective single institution study on children diagnosed with full thickness rectal prolapse and managed surgically during a period of 5 years (October 2009 – July 2014) using known International Classification of Disease (ICD) code. Results are compiled using chi square test and p-value.

Results: A total of 26 patients (18 males, 8 females) were identified during the study period with a median age at diagnosis of 4 years (range 1-9). Clinical details of 25 patients were available. Two out of 25 had rectal mucosa prolapse so they are excluded. Rest of 23 patients had full thickness rectal prolapse and were analysed very carefully. Fourteen patients were treated with injection sclerotherapy alone, 8/14 (57.14 %) patients developed reoccurrence ranging from 1 month to 9 months post sclerotherapy injection. Among those 5/8 required further phenol injections, 1/8 required phenol and Thiersch suture, 1/7 required rectopexy and 1/8 required rectal mucosal excision. Nine patients were treated with injection sclerotherapy and Thiersch suture. One in 9 (11 %) had re-occurrence 3 months later and was successfully treated with repeated injection sclerotherapy and Thiersch suture. All the 23 patients were on laxatives post procedure and follow up was more than 18 months. The chi-square statistic is 4.1972 with p-value of 0.040491. This result is significant at p- value < 0.05.

Conclusion: Combination of injection sclerotherapy and Thiersch procedure is more effective than injection sclerotherapy alone in the treatment of full thickness rectal prolapse in children. A post-operative laxative regimen is required in these patients as they can develop de novo constipation in post-operative period.

Keywords: Thiersch procedure, rectal prolapse, injection sclerotherapy

 

Introduction

Rectal prolapse is a common condition in children. Most of them can be managed conservatively. A significant number do require surgical intervention. There are various surgical options available in treating rectal prolapse in children. There is no consensus on the most effective surgical treatment. The three main surgical modalities to treat rectal prolapse are: A) Injection sclerotherapy [1-3] B) Thiersch procedure [4] and C) Rectopexy [5]. Injection sclerotherapy is the least invasive option. The success rate following initial following injection sclerotherapy alone as 67% [6]. Thiersch procedure is a relatively more invasive procedure. The success following a Thiersch procedure alone been reported as 61% [7]. Injection therapy is usually a first line of treatment in majority of the centres and the recurrence following the sclerotherapy are treated with repeat injection or with a Thiersch procedure. Rectopexy is reserved for children who have recurrent rectal prolapse despite Injection treatment and Thiersch procedure. There is reported use of combination of injection sclerotherapy with Thiersch procedure at the same sitting as first line treatment for rectal prolapse. It has shown 100% success rate [8]. However, this study involved only two patients and does not provide follow-up period. We have been using two techniques as first line initial treatment for rectal prolapse: 1) injection sclerotherapy on its own and 2) injection sclerotherapy combined with Thiersch procedure at the same sitting. This retrospective study aims to compare the outcome of children with rectal prolapse managed with injection sclerotherapy alone and in combination with Thiersch procedure.

Methods

This is a retrospective single institution study on children diagnosed with rectal prolapse and managed surgically during a period of 5 years (October 2009 – July 2014). We used the known International Classification of Disease (ICD-10) code for ‘Rectal prolapse’ (K623) to identify patients during the study period. We gathered clinical and operative details using our institution’s electronic database as well as clinical case notes. In particular, we looked at demographic information, symptoms, initial management outcome, type of surgical intervention and related complications. For injection sclerotherapy we use 5 ml of 5% phenol in almond oil. A park’s anal dilator or nasal speculum is used to open the Ano-rectal area for injection. The injection is administered in four equally divided doses around the four quadrants of lower rectum (above the anal canal). We use a spinal needle to inject in the submucosal plane. For injection sclerotherapy combined with Thiersch suture we used the same injection sclerotherapy as described above. The Thiersch stitch is then placed. Two small incisions (2-3 mm in length) are made through the skin with a Number 11 surgical blade, 1 cm from the mucocutaneous junction, at 12 o’clock and 6 o’clock position. We have utilized an absorbable suture in these procedures, as the need for the suture is only until the prolapsed rectal sub mucosa adheres to adjacent tissue. A 2/0 PDS stitch is paced circumferentially around the anal canal, utilizing the two incisions to insure its proper placement. The final tightening of the suture, before it’s tying, is made while the index finger of the operating surgeon in the anal canal. This maneuvered prevents over tightening. The PDS knot is then buried in the subcutaneous tissue in the incision at 6 o’clock or 12 o’clock position. Data on the patients who had undergone treatment with above two techniques was collected. Using the chi-square performed statistical analysis.

Results

A total of 26 patients (18 males, 8 females) with rectal prolapse were identified during the study period with a median age at diagnosis of 4 years (range 1-9 years).

Clinical details of 25 patients were available. Two out of 25 had rectal mucosal prolapse as presenting symptoms and was excluded from our study, as they do not have a full thickness rectal prolapse. Both had anorectal malformation for which a posterior sagittal ano-rectoplasty (PSARP) was done and then they had excision of rectal mucosa without any sclerotherapy or Thiersch procedure.

Rest of 23 patients who had full thickness rectal prolapse were analysed very carefully. Twenty patients had no underlying medical or surgical problems. One patient had cystic fibrosis (CF), one had spina bifida with neuropathic bladder and one had sacro-coccygeal teratoma excised. All had full thickness rectal prolapse. Sixteen patients had no history of constipation and were moving their bowel regularly with normal consistency having full thickness rectal prolapse on each occasion. The rest 7 patients had been constipated and were on laxatives with no relief of symptoms. All 23 patients were treated conservatively with or without laxatives and failed to improve clinically and subsequently required surgical intervention.

For data analysis we divided the 23 patients into two groups. First group had sclerotherapy (phenol) injections only and second group had phenol injection and Thiersch suture. Injection sclerotherapy alone was performed in 14/23 patients while 9/23 patients had a combination of injection sclerotherapy and Thiersch procedure. Out of 23 patients, pre-operatively 7 patients were constipated and were on laxative and had rectal prolapse. Five patients went for phenol injection only and 2 went for phenol and Thiersch suture.

Following treatment with injection sclerotherapy alone, 8/14 (57.1%) patients had reoccurrence of full thickness rectal prolapse ranging from 1 month to 9 months post sclerotherapy injection and all of them were on laxatives post injection. Among those 5/8 improved after another phenol injections, 1/8 improved after phenol and Thiersch suture, 1/8 required rectopexy and 1/8 required rectal mucosal excision. This group had 5 patients out of 14, who were constipated.

Following treatment with injection sclerotherapy and Thiersch suture 1/9(11%) had re-occurrence 3 months post-surgery and was successfully treated with repeated injection sclerotherapy (phenol) and Thiersch suture. This group had 2 patients out of 9 who were constipated were on laxatives and had rectal prolapse.

In total 23 patients with full thickness rectal prolapse were treated. Fourteen were treated with Phenol injection alone and 8 (57.1%) of them had a re-occurrence of rectal prolapse. As compared to 9 patients treated with phenol and Thiersch suture with only one patient (11%) had a re-occurrence of rectal prolapse. Follow up period in all 23 patients are more than 18 months (Table 1) and all of them were on laxatives post-surgery, which were weaned off slowly. Patients with cystic fibrosis and spina bifida had injection sclerotherapy and Thiersch suture in first instant with no postoperative complications or rectal prolapse where as one patient who had sacro-coccygeal teratoma excised and presented with full thickness rectal prolapse had injection sclerotherapy only (phenol) with re-occurrence of prolapse requiring excision of rectal mucosa.

Table 1

Total Patients

 

23

Re-Occurrence

Re-do Phenol

Re-do Phenol and Thiersch

Rectopexy

Rectal Mucosa Excision

Outcome

Phenol Injection

14

8 (57.1%)

5

1

1

1

Good

Phenol Injection and Thiersch Suture

 

09

1 (11%)

0

1

0

0

Good

We analyse our results and data using the chi-square statistic p-value. Our results are significant.

The chi-square statistic is 4.8735. The p-value is 0.0272. This result is significant at p value < 0.05. The chi-square statistic is 4.8735. The p-value is 0.02722. This result is significant at p value < 0.05. The chi-square statistic is 4.8735. The p-value is 0.02722.

Discussion

Rectal prolapse is a common condition in children. It affects both genders equally and normally manifests before the age of 4 years with the highest incidence in the first year of life [9]. In our cohort, the mean age at diagnosis was 4 years and there were more males than females affected by this condition. Predisposing conditions includes increased intraabdominal pressure as a result of chronic constipation and straining, infectious diarrhoea, parasitic and neoplastic disease of the rectum, malnutrition, pelvic floor weakness (Siafakas et al.)[10] and cystic fibrosis (Stern et al.)[11]. Other conditions are Ehlers Danlos syndrome (Douglas and Douglas et al.)[12] and Hirschsprung’s disease (Traisman et al.)[13]. In our series, only one patient had cystic fibrosis, two had previous Posterior sagittal Anorectoplasty (PSARP) for anorectal malformation, one had a previously excised sacrococcygeal teratoma and another had spinal bifida with neuropathic bowel. The condition can be self-limiting in some children.

There are conservative measures, which could be used if the rectal prolapse persists. These include manual reduction and modification of the position during defecation [1]. In addition, laxatives for constipation and anti-diarrhoeal medications to control loose stools may be useful in this condition. Following failure to manage rectal prolapse conservatively, different surgical options may be implemented depending on the clinical case.

Injection sclerotherapy is a common minimally invasive procedure used to treat rectal prolapse. There are several different sclerosing agents which include ethyl alcohol, 5% phenol, hypertonic saline solution, cow milk and dextranomer/hyaluronic acid copolymer (Deflux®) and have shown different outcomes [1-3]. This procedure has been reported having a 67% success rate following first injection of 5% phenol in almond oil [6].

In our series, we only used 5% phenol in almond oil with 57% recurrence following one injection treatment. Thus our recurrence rate following treatment with injection of 5% phenol in almond oils is similar to the one reported in literature.

Surgical option is anal encirclement (Thiersch’s procedure) which consisted of placing a suture internally around the anus with the aim of providing mechanical support and preventing the rectal prolapse [4]. This procedure is advocated to be the possible management option when the prolapse is due to an anatomical defect or in case of persistence (Oeconomopoulos and Swenson, 1960). In their study, Flum et al. showed a success rate of 61% when performing initial Thiersch procedure [7]. It is crucial that the Thiersch suture is not placed too tight or too loose. In the first instance, the child might experience difficulty in passing stool whereas in the second case rectal prolapse could occur followed by ischemia as the portion of bowel is unable to reduce due to the anal suture [14].

In our series we only had one case in which the Thiersch suture was too tight leading to extreme constipation necessitating its removal under general anaesthesia. However, in this child spontaneous rectal prolapse resolution occurred without the need of further surgical intervention. Thiersch procedure combined with injection sclerotherapy has also been reported successful in two patients in a retrospective study [8]. In our series, the combination of these two procedures lead to effective results in 89% of the patients compared to 52.9% achieved following injection sclerotherapy alone. These differences in the results are statistically significant. None of our patients underwent isolated Thiersch procedure. Laparoscopic rectopexy is another technique to treat recurrent rectal prolapse where the rectum is sutured to the sacral promontory fascia [5]. This procedure had been shown to have a very low recurrence rate (5%) [5]. In our study, rectopexy was only required in one case as patient developed multiple recurrences following phenol injection.

Other invasive procedures described in the literature include the Thiersch anal encirclement [4], linear cauterization [16], and packing of the pre sacral space with various materials [17]. More invasive options described include: trans sacral rectopexy [18,19], trans-coccygeal rectopexy [20], posterior sagittal ano-rectoplasty [21], and perineal procto-sigmoidectomy (Altemeier procedure) [22]. If little is known about the optimal initial operative management for rectal prolapse, far less is known about the best management approach following failure of the primary operative procedure, with only cases of recurrence following failure of injection sclerotherapy [23] and linear cauterization [16] being reported.

Following failure to manage rectal prolapse conservatively, different surgical options may be implemented depending on the clinical case. Injection sclerotherapy is a common minimally invasive procedure used to treat rectal prolapse. Widely used surgical option is Thiersch’s procedure, which consisted of placing a suture internally around the anus with the aim of providing mechanical support and preventing the rectal prolapse [4]. It is a minimally invasive procedure and is used in the treatment of rectal prolapse and faecal incontinence [24]. It was first described in 1891 by the German surgeon, Karl Thiersch [25]. He surrounded the anus with a ring of silver wire. Since the first description of this method, numerous modifications have been made. In 1896 making four small incisions through the skin with a knife, 1 cm from the muco-cutaneous junction laterally, and using a Nylon No. 1 stitch to pass around the perianal space cutaneously modify the procedure. This aims to narrow the relaxed anal sphincter and cause proliferation to form adhesions with the surrounding tissues. The success of the operation is probably due to the fact that placed suture ringbring the sphincter and pelvic floor higher, giving the sphincter a chance to contract normally. This procedure is advocated to be the possible management option when the prolapse is due to an anatomical defect or in case of persistence rectal prolapse [4].

Flum et al. showed a success rate of 61% when performing initial Thiersch procedure [7]. It is crucial that the Thiersch suture is not placed too tight or too loose. In the first instance, the child might experience difficulty in passing stool whereas in the second case rectal prolapse could occur followed by ischemia as the portion of bowel is unable to reduce due to the anal suture [14]. There is little agreement about the best management of rectal prolapse recurrence following initial surgical procedure [7]. Repeated sclerotherapy injections or Thiersch procedures may show to eventually succeed in some cases. Furthermore, modified Thiersch procedure has also been demonstrated to manage cases of recurrence with good outcomes [7]. Injection sclerotherapy is said to yield a success rate between 67%. The Thiersch procedure has a reported outcome of 61% success rate.The two procedures combined have a higher success rate than when performed separately [26]. In our series one patient out of eleven (11%) developed recurrence following combined treatment. This child had the combined procedure repeated twice and on the second occasion he had 5% phenol injection with a modified Thiersch procedure which consisted of three concentric sutures: two sutures placed above the dentate line and one suture below it. This patient was followed up for two and a half years and did not develop any adverse outcome. To best of our knowledge this type of modified Thiersch procedure has never been previously reported in the paediatric literature.

When the surgical management of rectal prolapse failed after phenol injection and Thiersch suture most of the surgeons recommend doing rectopexy. It is a major surgical procedure to treat recurrent rectal prolapse where the rectum is sutured to the sacral promontory fascia [5]. This procedure had been shown to have a very low recurrence rate (5%) [5]. In our study, rectopexy was only required in one case as patient developed multiple recurrences following phenol injection, but there is new technique and surgical procedure just been introduced to do modified 3 suture Thiersch technique. One PDS 1/0 (absorbable) Thiersch suture was placed just above the anal canal and below the dentate line, a second 1/0 Prolene (non-absorbable) Thiersch suture was inserted above the dentate line and a third 1/0PDS (absorbable) Thiersch suture was placed about 3 cm above the dentate line. At the end of the procedure, a soft jelonet dressing was left in the rectum, which self-ejects. This technique showed an excellent outcome results [27]. One can use this technique instead of major surgical procedures like Altemeier or Delorme procedures, or rectopexy.

In the paediatric population, rectal prolapse is most commonly encountered before 4 years of age, with the highest incidence found in the first year of life. In our cohort, the median age at diagnosis was 4 years and there were more males than females affected by this condition. The decision to perform surgery for prolapse is generally based on the duration of conservative management, recurrence of symptoms and the overall severity of symptoms, including pain, rectal bleeding and perianal excoriation.

We studied the retrospective data of the patients having full thickness rectal prolapse over the last 5 years. In our series, we divided patients into two groups one having phenol injection only and other group had phenol and Thiersch suture for full thickness rectal prolapse. We have excluded two patients who had mucosal prolapse and required excision of mucosa in the first instant.Our patient population was affected with conditions associated with rectal prolapse, including constipation, which was most common, as well as, ano-rectal malformation, spina bifida, sacro-coccygeal teratoma and CF. These conditions have all been described in the literature to be associated with rectal prolapse in addition to diarrheal diseases, imperforate anus (post-repair), rectal polyps, and Ehlers–Danlos syndrome [28-29]. It is interesting to note that only one of the patients included in our study group carried a diagnosis of CF. A prevalence of 11.1% has been reported for CF in children with rectal prolapse, and the prevalence of rectal prolapse in CF patients has been estimated to be 18.5– 22.6% [30-31]. The observation that only one of our patients had an established diagnosis of CF, suggests that CF in more recent years is better managed, allowing successful treatment with medical therapy alone.

We analysed the data using Chi Square and p-value and got significant result supporting our conclusion and outcome. The patients with full thickness rectal prolapse having phenol injection only had higher incidence of re-occurrence rate as compared to our other group who had phenol as well as Thiersch suture done at the same time. Patients with phenol injection only had a re-occurrence rate of 57% as compared to our Thiersch group with re-occurrence rate of 11%. In our series we found that the children with failed medical management of rectal prolapse will be treated better with phenol injection and Thiersch suture at the same time. It will give better outcome results, less traumatic to the children and chances of having another surgical intervention involving general anaesthesia and traumatising the child and family will be avoided. In our series, the combination of these two procedures lead to effective success rate of 89% in the patients compared to 42.8% achieved following injection sclerotherapy alone. None of our patients underwent isolated Thiersch procedure. Finally, laxative regime post-surgery is important in order to avoid developing new onset of constipation and hence possible recurrence [15]. We always start laxatives post operatively for our patients treated with full thickness rectal prolapse and then slowly wean them off. There are other studies advocating the use of laxatives for post-operative constipation following rectal prolapse [32-33]. This could be explained by the fact that post-operative pain might prevent the child from passing stools leading to constipation. In addition, post-operative constipation, may also potentially lead to recurrence of the rectal prolapse due to excessive straining.

Finally, laxative regime post-surgery is important in order to avoid developing new onset of constipation and hence possible recurrence [15]. This could be explained by the fact that post-operative pain might prevent the child from passing stools leading to constipation. In addition, post-operative constipation, may also potentially lead to recurrence of the rectal prolapse due to excessive straining. In this series, 33% of those patients who received the combination of injection sclerotherapy and Thiersch suture, developed complications post-operatively and required management with laxatives.

Conclusion

In conclusion, combination of injection sclerotherapy and Thiersch procedure for the treatment of rectal prolapse in children demonstrated much lower recurrence rate in our study when compared to injection sclerotherapy alone. Post-operative laxative management is important in these patients as they can develop de novo constipation.

 

 

References:

1. S. Fahmy MA. Ezzelarab. Outcome of sub mucosal injection of different sclerosing materials for rectal prolapse in children. Pediatr Surg Int 2004; 20:353-6.

2. Abeş M, Sarihan H. Injection sclerotherapy of rectal prolapse in children with 15 percent saline solution. Eur J Pediatr Surg 2004; 14:100-2.

3. Zganjer M, Cizmic A, Cigit I, Zupancic B, Bumci I, Popovic L, Kljenak A. Treatment of rectal prolapse in children with cow milk injection sclerotherapy: 30-year experience. World J Gastroenterol 2008; 14:737-40.

4. Oeconomopoulos CT, Swenson O; Thiersch's operation for rectal prolapse in infants and children. Am J Surg 1960; 100:457-61.

5. Potter DD, Bruny JL, Allshouse MJ, Narkewicz MR, Soden JS, Partrick DA. Laparoscopic suture rectopexy for full-thickness anorectal prolapse in children: an effective outpatient procedure. J Pediatr Surg 2010; 45:2103-7.

6. M. Sasaki Y. Iwai N. Kimura O. Hibi. The treatment of rectal prolapse in children with phenol in almond oil injection. Eur J Pediatr Surg 2004; 14:414-7.

7. DH. Flum AS. Golladay ES. Teitelbaum. Recurrent rectal prolapse following primary surgical treatment. Pediatr Surg Int 2010; 264:427-31.

8. R. Antao B. Bradley V. Roberts JP. Shawis. Management of rectal prolapse in children. Dis Colon Rectum 2005; 48:1620-5.

9. Corman ML. Rectal prolapse in children. Dis Colon Rectum 1985; 28:535-9.

10. Siafakas C, Vottler TP, Andersen JM. Rectal prolapse in pediatrics. Clin Pediatr (Phila) 1999; 38:63–72.

11. Stern RC, Izant RJ Jr, Boat TF, et al. Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterology 1982; 82:707–10.

12. Douglas BS, Douglas HM. Rectal prolapse in the Ehlers- Danlos syndrome. Aust Paediatr J 1973; 9:109–110.

13. Traisman E, Conlon D, Sherman JO et al. Rectal prolapse in two neonates with Hirschsprung’s disease. Am J Dis Child 137:1126–1127.

14. Pachl M, de la Hunt M, Jawaheer G (2013) Key Clinical Topics in Paediatric Surgery. Rectal prolapse. JP Medical Ltd, London, pp 296-297.

15. Sun C, Hull T, Ozuner G. Risk factors and clinical characteristics of rectal prolapse in young patients. J Visc Surg 2014; 151:425-9.

16. Hight DW, Hertzler JH, Philippart AI et al. Linear cauterization for the treatment of rectal prolapse in infants and children. Surg Gynecol Obstet 1982; 154:400–402.

17. Nwako F. Rectal prolapse in Nigerian children. Int Surg 1975; 60:284–285.

18. Chino ES, Thomas CG. Transsacral approach to repair of rectal prolapse in children. Am Surg 1984; 50:70–75.

19. Schepens MA, Verhelst AA. Reappraisal of Ekehorn’s rectopexy in the management of rectal prolapse in children. J Pediatr Surg 1993; 28:1494–1497.

20. Ashcraft KW, Amoury RA, Holder TM. Levator repair and posterior suspension for rectal prolapse. J Pediatr Surg 1977; 12:241– 245.

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22. Lee JI, Vogel AM, Suchar AM et al. Sequential linear stapling technique for perineal resection of intractable pediatric rectal prolapse. Am Surg 2006; 72:1212–1215.

23. Shah A, Parikh D, Jawaheer G et al. Persistent rectal prolapse in children: sclerotherapy and surgical management. Pediatr Surg Int 2005; 21:270–273.

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Total Patients

 

23

Re-Occurrence

Re-do Phenol

Re-do Phenol and Thiersch

Rectopexy

Rectal Mucosa Excision

Outcome

Phenol Injection

14

8 (57.1%)

5

1

1

1

Good

Phenol Injection and Thiersch Suture

 

09

1 (11%)

0

1

0

0

Good

Ubaidullah Khan, Murad Kitar, Imed Krichen, Kais Maazoun, Naglaa M Kamal, Rasha A, Mostafa YL Khalif

Pediatric Surgery, Department of Surgery, Alhada Armed Forces Hospital, Taif, Saudi Arabia

 

Correspondence:

Ubaidullah Khan

Pediatric Surgery, Department of Surgery

Alhada Armed Forces Hospital, Taif, Saudi Arabia

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Phone: 00966547005971

 

Abstract

Background and objectives: Soave transanal procedure for Hirschsprung disease (HD) is most commonly performed at early age with good outcome. In our center we adopted this technique in many patients in different age groups without assisted laparoscopic abdominal approach. We herein aim to describe different technical aspects, outcome and success rate of Soave procedure among our patients.

Methods: We retrospectively reviewed our series of HD patients who underwent the Soave transanal approach, for postoperative stricture, anastomotic leak, enterocolitis, and subsequent bowel functions. Patients were divided into two groups: group 1: the early neonatal group (0-1 month) and group 2: the late presentation group ( 3-14years).

Results: Among the 17 patients with HD operated in our center, 13 underwent Soave transanal resection, 1 was lost from follow up, 2 had a levelling colostomy prior to referral and 1 had ileostomy. The average length of resection was 20±10.5 cm. The mean follow-up period was 12.2 months (range 5–22 months). Seven patients were 3-14 years old. At follow-up none had fecal incontinence or constipation requiring laxatives. All patients had voluntary bowel movements.

Conclusions: Our results support the fact that a single stage Soave approach without transabdominal dissection is an excellent technique for patients presenting with late HD with an excellent outcome. A multicenter prospective study with larger number of patient is essential to validate our results.

Keywords: Hirschsprung disease, Soave, transanal


 

Introduction

Hirschsprung’s disease (HD) is the commonest congenital gut motility disorder and is characterized by a lack of ganglion cells (aganglionosis) in a variable length of the distal gut. Affected infants usually present shortly after birth with signs of distal intestinal obstruction that are invariably fatal if left untreated. It has been more than 60 years since the original description of the aetiology of HD, elucidated by Dr. Ovar Swenson [1]. Since then, numerous techniques have been described for removal of the aganglionic distal colon. Removal of the aganglionic bowel, pulling through ganglionic bowel, and preserving the anal canal and sphincter mechanism, remain the principles to surgical repair regardless of the technique.

The operative management of HD has evolved dramatically, from full-thickness rectosigmoid dissection (Swenson) [2], endorectal dissection (Soave) [3], retrorectal pouch procedure (Duhamel) [4,5], low anterior resection (Rehbein) [6] and more recently a primary repair [7,8] that can be done transanally [9,10] and using the laparoscopictechnique [11]. Despite all this, still there is debate about which technique provides the best short and long-term results.

Soave is most commonly done nowadays with good results in the young age group. The transanal Soave procedure is a natural evolution of the laparoscopic operation. Transanal resection of the rectum was shown to be possible in animal models and in the initial series of children with HD who were published by de la Torre and Ortega-Salgado [34] and Langer et al. [35] in the late 1990s. The transanal approach has the principal benefit of avoiding the need for intraabdominal mobilization of the rectum through either laparotomy or laparoscopy [27].

We herein aim to describe our experience with Soave technique in our cohort of HD and compare its outcomes in the early and late presenters groups.

Patients and methods

We retrospectively reviewed electronic records of pediatric patients, age 0 to 14 years, fulfilling the inclusion criteria of confirmed diagnosis of HD who underwent surgical repair in the period between January 2016 and July 2017 in the Pediatric Surgery department of Alhada Armed Forces Hospital, Taif, Saudi Arabia. The study was approved by the research and ethical committee of the hospital.

Exclusion criteria were patients with confirmed diagnosis of HD but not yet operated, and rectal biopsy histopathological result other than HD. Patients were divided into two groups according to the age at presentation and transanal resection: group 1 - before one month of age and group 2 - later than one month of age.

All patients’ files were reviewed for the following information: demographic data (age, gender), medical history (age at presentation, presenting symptoms, other medical problems), surgical history (for any operation done other than the primary repair of HD), confirmation of HD (contrast imaging of each patient was assessed for the level of transition zone, rectal biopsy with histopathological assessment), preoperative data (preoperative bowel preparation and intravenous antibiotics use), operative data(efficiency of bowel preparation, age at the time of surgical correction, surgical technique used for repair, performance of intra-operative full thickness frozen section biopsy, length of each resected specimen), hospital course and post-operative follow-up (information on early and late post-operative complications was evaluated as well as long-term outcomes especially fecal incontinence and constipation). Rates of stricture formation, pelvic abscess, and enterocolitis were noted as well.

Statistical analysis

Data were coded and statistical analysis was done using Statistical Package for Social Sciences software program (SPSS) Version 20. Quantitative data were expressed as means ± standard deviation. Although qualitative data were expressed as percentages. A Chi-square and Fisher exact tests were applied for bivariate data analysis to test the statistical significance of associations, the level of significance was P ≤ 0.05.

Results

Eighteen patients were diagnosed withHD but one female patient was excluded as she was above 14 years old and referred to adult surgeon services. The remaining 17 patients fulfilled all the study inclusion criteria and were enrolled in the study with 9 patients in group 1 and 8 patients in group 2.

Age at the time of operation ranged from 10 days to 14 years with mean age of 14±12 days in group 1 and 7.9 ± 5.8 years in group 2. There were 13 male and 4 female patients with a ratio of 3.2:1. Each group included two female patients.

Three patients didn’t go to direct primary repair of HD due to bowel perforation at few days of birth from which 2 of them underwent colostomy and one underwent ileostomy.

Preoperative evaluation with contrast enema was done in all patients which revealed a transition zone in the rectosigmoid in 12 patients (70.5%), left colon in 4 (23.5%) and transverse colon in 1 (5.8%) as shown in Fig. 1. HD was confirmed by preoperative rectal biopsy with histopathologic evidence of aganglionosis. All patients underwent mechanical bowel preparation along with rectal irrigation prior to their definitive operation aiming for best operative and post-operative outcomes. Six patients in group 2 had afecalom on presentation and three of them necessitated irrigation under anaesthesia to wash well.

Figure 1. Contrast study showing dilated proximal colon (blue arrow) and distal portion (black arrow) with clear transition zone

11.2 1 1

Preoperative intravenous antibiotics with third generation cephalosporin and metronidazole were given to all patients with provisions made for allergies.

One surgical technique was used in the studied cohort with transanal rectal dissection, short sleeve, Soave approach with careful preservation of the dentate line. The three patients with fecal diversion prior to definitive repair underwent transanal resection in the neonatal period. The transverse colostomy in two of them was left untouched for later reversal as a third stage; the ileostomy in the third patient required a stoma reversal in a next stage to pull-through procedure.

In the remaining 14 patients who had a well defined sigmoid or lower left colon transition zone on contrast enema (Fig. 2), a purely Soave transanal resection was performed without preoperative diversion. The transanal dissection was performed using a lithotomy position. Sutures were placed initially just inside the anal canal at the level of the mucocutaneous junction for retraction, in this way the distal 1.5 cm of anal canal was preserved. Interrupted sutures were placed 1.0 cm proximal to the dentate line in a circumferential fashion to provide uniform traction. Monopolar electrocautery was used to perform submucusal dissection leaving 3 cm cuff and then a full-thickness dissection along the traction line of sutures. The dissection was carried into the peritoneal cavity and the colon was pulled-through to the appropriate level. The anastomosis was created in two layers, 1.0 cm proximal to the dentate line. In all patients, full thickness frozen section intraoperative biopsies were checked in the operating theatre to assure that the pull-through segment had ganglionic cells and the muscularis and submucosa had normal sized nerve trunks.

Figure 2. Well excised specimen to demonstrate with contrast study (arrows)

11.2 1 2

Laparoscopy or laparotomy were not used to assist the dissection in patients with disease that extended even above the mid-sigmoid, except for the 3 patients who had previous levelling stomas. The average length of resected specimen was 22.6 ± 10.9 cm in both groups with no statistically significant difference (Fig. 2). The time consumed during the operation was slightly in group 2 but didn’t reach a statistical significance. Operative and post-operative complications were compared in both groups with no statistically significant difference. In both groups, none of the patients required perioperative blood transfusions, none had urinary retention, wound infection, enterocolitis, anastomotic leak or strictures, or intraabdominal abscesses.

All patients were followed up in the pediatricsurgey outpatient clinic at 2 weeks, 2 months and 6 months. At two weeks, rectal examination using Hagar dilators was done to assess the need for rectal dilations. Two patients from group 2 needed anal dilation in the first two clinic visits and improved after dilation. One patient in group 2 had constipation requiring intermittent laxative usage. All patients had multidisciplinary care follow-up plane with pediatric surgery, pediatric gastroenterology and clinical nutrition departments.

Group 2 patients were assessed for fecal and bowel functions on follow up where voluntary bowel movements and spontaneous voiding with good control was reported in 100% of patients (Table 1).

Table 1: Showing results of bowel function after Soave in both groups

 

Bowel Function

Total

Normal

NA

Age

less than a month

Count

9

0

9

% within age

100.0%

0.0%

100.0%

less than 5 years

Count

1

0

1

% within age

100.0%

0.0%

100.0%

less than 10 years

Count

6

0

6

% within age

100.0%

0.0%

100.0%

more than 10 years

Count

0

1

1

% within age

0.0%

100.0%

100.0%

Total

Count

16

1

17

% within age

94.1%

5.9%

100.0%

Discussion

HD is one of the most common congenital gut motility disorders facing pediatric surgeons with a mainstay of treatment involving removing the aganglionic bowel segment and pulling through the ganglionic bowel while preserving the anal sphincter. Many surgical techniques had evolved with many published HD series treated with either the laparoscopic or transanal pull-through techniques from almost every continent with excellent outcomes [16,17,18,33,28].

Few studies compared the laparoscopic to the transanal approach [19]. Miyano and his colleagues [27] adopted the laparoscopic approach, while Pratap and his group [36] highlighted the high feasibility of the transanal pull-through as it can be done by any pediatric surgeon, including those without laparoscopic skills, and by pediatric surgeons in parts of the world where access to appropriately laparoscopic equipment is limited.

In our center we adopted the Soave transanal technique in all our patients irrespective of the age at presentation and age at surgical intervention. We divided patients into two groups: neonatal and postneonatal, Soave procedure results were almost similar in both groups. None experienced anastomotic leaks, strictures or intraabdominal abscesses, this compares favourably with literature results which quotes leak rates of 5–7%, stricture rates of 5–24%, and abscess formation in 2–6% [20-25] which might be explained by the fact that we performed the Soave procedure without opening the abdomen or creating a stoma as compared to diversion before pull through done by others. The complications described by them beyond the neonatal age were attributable to the stoma done before Soave not the Soave itself [31]. In those series surgeons preferred preliminary defunctioning stoma in older children for resolution of the dilatation and thickening, so that a subsequent transanal approach can be safely used, however in our series like Onishi and his group, we used good colonic irrigations to decompress the dilated colon. We didn’t encounter any difficulty during transanal dissection to pull down this dilated segment of the rectum or colon (Fig. 2) and a single stage Soave procedure was performed without any appreciable complications in follow up as compared to the neonatal group (Table 1).

Full thickness frozen biopsies sections were performed intraoperative in all patients in our cohort. Based on the report of Muller et al. 2012 [22], we adopted our protocol where we don’t only depend on confirmation of presence of ganglion cells in intraoperative biopsies studies prior to performing an anastomosis, but also to have pathologic assurance of normal appearing nerves without hypertrophied nerve trunks in a circumferential biopsy sample which includes muscularis and submucosa. These protocols explain the slightly prolonged operative time in group 2 which was attributed to the wait time for the results of the intraoperative biopsies. This means that practically speaking the time of the technique itself is almost equal in both groups.

Only one patient in group 2 had constipation with intermittent laxative use and none had bowel dysfunction in both groups as excessive pull of the anal canal and external sphincter were avoided. In concordance with Levitt and his group [26], we completely removed the aganglionic bowel apart from the preserved 1 cm above the dentate line, without leaving behind a long cuff as we only leave around 3 cm cuff.

Although we have a study limit because of a small cohort population, we can conclude that the Soave approach is a safe, reproducible and elegant technique if properly planed irrespective of the patient’s age. A larger multicenter prospective study is warranted to solidify our conclusion.

 

 

References:

1. Swenson O, Rheinlander HF, Diamond I. Hirschsprung’s disease: a new concept of the etiology. N Engl J Med 1949;241:551-6.

2. Swenson O, Bill AH. Resection of rectum and rectosigmoid with preservation of sphincter for benign spastic lesions producing megacolon: an experimental study. Surgery 1948;24:212.

3. Soave F. A new operation for the treatment of Hirschsprung’s disease. Surgery 1964;56:1007-14.

4. Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35:38-9.

5. Duhamel B. Une nouvelle opération pour le mégacolon congénital: l’abaissement rétrorectal et trans-anal du colon, et son application possible au traitement de quelques autres malformations. Presse Med 1956;64:2249-50.

6. Rehbein F, Von Zimmermann H. Results with abdominal resection in Hirschsprung’s disease. Arch Dis Child 1960;35:29-37.

7. So HB, Schwartz DL, Becker JM, et al. Endorectal “pull through” without preliminary colostomy in neonates with Hirschsprung’s disease. J Pediatr Surg 1980;15:470-1.

8. Teitelbaum DH, Coran AG. Primary pull-through for Hirschsprung’s disease. Semin Neonatol 2003;8:233-41.

9.De la Torre-Mondrago’n L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 1998;33:1283-6.

10. Langer JC, Minkes RK, Mazziotti MV, et al. Transanal one-stage Soave procedure for infants with Hirschsprung’s disease. J Pediatr Surg 1999;34:148-52.

11. Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pull through for Hirschsprung’s disease in infants and children. J Pediatr Surg 1995;30:1017-22.

12. Levitt MA, Peña A. Imperforate anus and cloacal malformations. In: Holcomb GW, Murphy JP, editors. Ashcraft’s pediatric surgery. 5th ed. Philadelphia (Pa): Saunders Elsevier; 2010. p. 468-90.

13. Lawal T, Chatoorgoon K, Collins M, et al. Redo pull-through for obstructive symptoms due to residual aganglionosis and transition zone in Hirschsprung’s disease. Pediatr Surg 2011;46:342-7.

14. Swenson O. Follow-up on 200 patients treated for Hirschsprung’s disease during a ten-year period. Ann Surg 1957;146:706-14.

15. Langer JC, Durrant AC, de la Torre-Mondragón L, et al. One-stage transanal Soave pullthrough for Hirschsprung disease: A multicenter experience with 141 children. Ann Surg 2003;238:569-76.

16. Pratap A, Shakya VC, Biswas BK, et al. Single-stage transanal endorectal pull-through for Hirschsprung’s disease: Perspective from a developing country. J Pediatr Surg 2007; 42:532-35.

17. Sookpotarom P, Vejchapipat P. Primary transanal Swenson pull through operation for Hirschsprung’s disease. Pediatr Surg Int 2009; 25:767-73.

18. Vu PA, Thien HH, Hiep PN. Transanal one-stage endorectal pull through for Hirschsprung disease: Experiences with 51 newborn patients. Pediatr Surg Int 2010; 26:589-92.

19. Singh R, Cameron BH, Walton JM, et al. Postoperative Hirschsprung’s enterocolitis after minimally invasive Swenson’s procedure. J Pediatr Surg 2007; 42:885-9.

20. Fortuna RS, Weber TR, Tracy TF, et al. Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 1996;131: 520-24.

21. Somme S, Langer JC. Primary versus staged pull-through for the treatment of Hirschsprung disease. Semin Pediatr Surg 2004;13: 249-55.

22. Muller CO, Mignot C, Belarbi N, et al. Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung’s disease? Pediatr Surg Int 2012;28:597-601.

23. Nasr A, Langer JC. Evolution of the technique in the transanal pullthrough for Hirschsprung’s disease: Effect on outcome. J Pediatr Surg 2007;42:36-9.

24. Langer J, Caty M, de la Torre-Mondragon L, et al. IPEG colorectal panel. J Laparoendosc Adv Surg Tech A 2007;17:77-100.

25. Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung’s disease: A systematic review and meta analysis. Pediatr Surg Int 2011; 27:1053-7.

26.Marc A. Levitt, Miller C. Hamrick. Transanal, full-thickness, Swenson-like approach for Hirschsprung disease. Journal of Pediatric Surgery 2013; 48, 2289–2295.

27.Miyano G, Takeda M, Koga H, Okawada M Hirschsprung's disease in the laparoscopic transanal pull-through era: implications of age at surgery and technical aspects. Pediatr Surg Int. 2017: 5.

28.Zimmer J, Tomuschat C, Puri P. Long-term results of transanal pull-through for Hirschsprung's disease: a meta-analysis. Pediatr Surg Int. 2016 ;32(8):743-9.

29.Lu C, Hou G, Liu C, Geng Q, Xu X et al. Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and non neonates: A multicenter study. J Pediatr Surg. 2017 Jul;52(7):1102-1107.

30.Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood. Ann Surg. 2017 ;265(3):622-629.

31.Onishi S, Nakame K, Yamada K. Long-term outcome of bowel function for 110 consecutive cases of Hirschsprung's disease: Comparison of the abdominal approach with transanal approach more than 30 years in a single institution - is the transanal approach truly beneficial for bowel function? J Ped Surg 2016;51(12):2010-2014

32.Patrycja Sosnowska, Michał Błaszczyński. A 15-Year Experience with the One-Stage Surgery for Treatment of Hirschsprung’s Disease in Newborns, Infants, and Young Children. Indian J Surg 2015; 77(Suppl 3): S1109–S1114.

33.Amine Ksia, Houssem Yengui, Manel Ben Saad. Soave transanal one-stage endorectal pull-through in the treatment of Hirschsprung's disease of the child above two-year old: A report of 20 cases. Afr J Paediatr Surg 2013; 10 (4): 362—366.

34. de La Torre-Mondragon L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 1998;33: 1283-6.

35. Langer JC, Minkes RK, Mazziotti MV, et al. Transanal one-stage Soave procedure for infants with Hirschsprung disease. J Pediatr Surg 34:148-52.

36. Pratap A, Shakya VC, Biswas BK, et al. Single-stage transanal endorectal pull-through for Hirschsprung’s disease: perspective from a developing country. J Pediatr Surg 2007; 42:532-5.

 

Bowel Function

Total

Normal

NA

less than a month

Count

9

0

9

 

% within age

100.0%

0.0%

100.0%

 

less than 5 years

Count

1

0

1

 

% within age

100.0%

0.0%

100.0%

 

less than 10 years

Count

6

0

6

 

% within age

100.0%

0.0%

100.0%

 

more than 10 years

Count

0

1

1

 

% within age

0.0%

100.0%

100.0%

 

Total

Count

16

1

17

% within age

94.1%

5.9%

100.0%

Ubaidullah Khan, Murad Kitar, Imed Krichen, Kais Maazoun, Naglaa M Kamal, Rasha A, Mostafa YL Khalif

 

Abstract

Background and objectives: Soave transanal procedure for Hirschsprung disease (HD) is most commonly performed at early age with good outcome. In our center we adopted this technique in many patients in different age groups without assisted laparoscopic abdominal approach. We herein aim to describe different technical aspects, outcome and success rate of Soave procedure among our patients.

Methods: We retrospectively reviewed our series of HD patients who underwent the Soave transanal approach, for postoperative stricture, anastomotic leak, enterocolitis, and subsequent bowel functions. Patients were divided into two groups: group 1: the early neonatal group (0-1 month) and group 2: the late presentation group ( 3-14years).

Results: Among the 17 patients with HD operated in our center, 13 underwent Soave transanal resection, 1 was lost from follow up, 2 had a levelling colostomy prior to referral and 1 had ileostomy. The average length of resection was 20±10.5 cm. The mean follow-up period was 12.2 months (range 5–22 months). Seven patients were 3-14 years old. At follow-up none had fecal incontinence or constipation requiring laxatives. All patients had voluntary bowel movements.

Conclusions: Our results support the fact that a single stage Soave approach without transabdominal dissection is an excellent technique for patients presenting with late HD with an excellent outcome. A multicenter prospective study with larger number of patient is essential to validate our results.

Keywords: Hirschsprung disease, Soave, transanal

 

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