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No 1

No 1 (17)

Apoorva Kulkarni, Abhaya Gupta, Vishesh Dikshit, Paras Kothari, Geeta Kekre

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

 

Correspondence:

Apoorva Kulkarni

Department of Pediatric Surgery

Lokmanya Tilak Municipal Medical College and Government Hospital

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

Round ligament varicosities are a rare disorder seen in gravid females and is often mistaken for complicated inguinal hernia. We present a very rare case of a 2 years old female child who presented with intractable pain in the right inguinal region, found to be a round ligament varicosity. Relief was achieved only after excision of the mass. A diagnostic laparoscopy was also done to confirm the presence of normal ovaries.

Keywords: varicosity, inguinal, child, round ligament

doi: 10.21614/jpss.11.1.37

Citation: J Pediatr Surg Spec. 2017;11(1):37-39

 

Introduction

Round ligament varicosity is a rare occurrence in a child. It is seen mainly in pregnant females due to compression of the venous system. Our patient presented with swelling and pain the inguinal region. We describe this rare case in a 2-year-old female child, treated with excision of the varicosity.

Case report

A 2 years old female child presented with a painful swelling in the right inguinal region for 1 month. There was history of fine needle aspiration cytology (FNAC) done from the swelling which was followed by sudden increase in size. The FNAC was reported as “Blood mixed aspirate”. There was no history of reducibility of this swelling. Cough impulse was absent. The parents also complained of a slight difference in the size of the two calves since birth, with the right one seeming slightly bigger than the left. On examination, there was a 4 x 3 cm tender firm swelling in right inguinal region (Fig. 1). There was no discrepancy in lower limb length but the right calf circumference was bigger than left by 1 cm.

Figure 1. Swelling in right inguinal region

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Theultrasonography (USG) of the inguino-labial region revealed a well-defined hetero-genously hypoechoic lesion 5.6x2.6 cm in size. There was another similar lesion 1.6x0.9 cm adjacent but separate from it. These two possibly represented hematomas. There were also features suggestive of right sapheno-femoral junction varix (Fig. 2).

Figure 2. Ultrasonographic image of varicosity

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Right lower limb venous Doppler was suggestive of hematoma in the inguinal region, venous malformation of the saphenous veinand the popliteal vein. There was no evidence of arteriovenous fistula. MRI of the right lower limb showed abnormal vessels and vascular spaces in the right gluteus, thigh and leg suggestive of low flow venous malformations, sapheno-femoral varix and hematoma in the right inguinal region extending into the mons pubis with abnormal vessels around it representing part of the venous malformation. There was no evidence of AV fistula. All blood investigations were normal. As the patient was continuously in pain despite being on analgesics, she was taken up for excision of the swelling. A right supra inguinal incision was performedand deepened to expose the external oblique aponeurosis. A well-defined firm mass of 6x3 cm was seen just below the superficial inguinal ring and adherent to the round ligament (Fig. 3). This mass had areas of hematoma. At this point, there was a suspicion that the mass may be a herniated, torted and necrosed right ovary, hence a diagnostic laparoscopy was done by putting in a 5 mm umbilical port. The presence of both ovaries in their natural lie was confirmed. Both the deep inguinal rings were noted to be closed. The mass was then excised en-massand sent for histo-pathological analysis. No feeding vessels were found supplying the vascular mass, hence there was minimal bleeding. The mass was not extending retroperitoneally.Hemostasiswas attained and the incision closed.

Figure 3. Intra-operative photograph of varicosity

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Post op recovery was uneventful and the patient was given compression stockings to wear. The histopathology report came as “fibrocollagenous tissue with thick walled vessels of varying caliber, some with clots, suggestive of vascular malformation”.

Discussion

Round ligament varicosities are a rare entity and seen almost exclusively in pregnant females. Till 2016, less than 20 cases have been reported internationally in pregnant women [1,2] but no case has been reported in young children. They present in the early third trimester mimicking a complicated inguinal hernia and resolve soon after delivery. The lesion arises from the veins draining the round ligament and the inguinal canal and may result due to:progressive venous obstruction due to gravid uterus, progesterone-mediated smooth muscle relaxation, raised cardiac output during pregnancy causing increased venous return from the limbs and leading to engorgement of venous tributaries [3-6] thus in broad sense are part of pelvic congestion syndrome.

Congenital vascular malformations may be present in children as a part of certain syndromes like: Klippel-Trenaunay syndrome (capillary malformations (port-wine stains), soft-tissue or bony hypertrophy, and varicose veins or venous malformations without AV malformation or shunting [7], Parkes weber syndrome (venous malformations, cutaneous capillary malformations, and lymphatic malformations along with AV malformation) [7], Proteus syndrome (asymmetric overgrowth of skin, bones, muscles, fatty tissue, blood vessels and lymphatic channels) [8]. Our patient failed to fit into any of the overgrowth syndromes. Lower limb venous abnormalities can occur in the superficial, deep and perforating vein systems. Popliteal and superficial femoral veins are most frequently involved. Venous malformation involving the round ligament vessels in children is extremely rare. Conservative treatment like compression therapy and long term follow up is advised in asymptomatic patients.In patients who are symptomatic, like in our case surgical excision of the painful mass is indicated after confirming patency of the deep vein system.

At follow up 2 years later, our patient was found to have varicosities of the right great saphenous venous system, confirmed with venous Doppler imaging, which had to be treated with stripping of the vein.

 

 

References:

1. Andriessen MJ, Hartemink KJ, de Jong D. Round ligament varicosities mimicking inguinal hernia during pregnancy. J Am Coll Surg 2009;208:321.

2. Jaiswal et al. Round ligament varicosity in pregnancy.IJBAR (2012) 03(12).

3. IJpma FF, Boddeus KM, de Haan HH, van Geldere D. Bilateral round ligament varicosities mimicking inguinalhernia during pregnancy. Hernia 2009;13:85-8.

4. Chi C, Taylor A, Munjuluri N, Abdul-Kadir R. A diagnosticdilemma: round ligament varicosities in pregnancy.Acta Obstet Gynecol Scand 2005;84:1126-7.

5. Cheng D, Lam H, Lam C. Round ligament varices in pregnancymimicking inguinal hernia: an ultrasound diagnosis.Ultrasound Obstet Gynecol 1997;9:198-9.

6. McKenna DA, Carter JT, Poder L, Gosnell JE, Maa J, Pearl JM, et al. Round ligament varices: sonographic appearancein pregnancy. Ultrasound Obstet Gynecol 2008;31:355-7.

7. NoelAA, Gloviczki P, Cherry KJ Jr, Rooke TW, Stanson AW, Driscoll DJ. Surgical treatment of venous malformations in Klippel-Trenaunay syndrome. J Vasc Surg2000; 32: 840–847.

8. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0

Ramnik Patel, Isaac Philip

Department of Paediatric Surgery, The Royal Belfast Hospital for Sick Children, Belfast, Northern Ireland, UK

 

Correspondence:

Isaac Philip

Department of Paediatric Surgery

The Royal Belfast Hospital for Sick Children

180, Falls Road, Belfast BT12 6BE Northern Ireland UK

Phone: 00442890 632008

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

 

Abstract

We present a case of distal duodenal stenosis without a wind sock deformity in a Down’s syndrome presenting with non-bilious vomiting, constipation and bilious aspirates simulating Hirschsprung’s disease. Despite high index of suspicion and actively looking for associated congenital duodenal obstruction it eluded detection. The plain film and rectal biopsy in neonatal period were normal. It was the upper gastrointestinal study which helped in diagnosis. At exploration, distal duodenal stenosis without wind sock deformity was identified. Duodeno-jejunostomy was curative.

Keywords: duodenal stenosis, Down’s syndrome, duodeno-jejunostomy

doi: 10.21614/jpss.11.1.33

citation: J Pediatr Surg Spec. 2017;11(1):33-36

 

Introduction

There is very high incidence (36% or one third) of congenital duodenal obstruction in Down’s syndrome [1-2]. Congenital duodenal obstructions present with bilious vomiting and are relatively uncommon to be proximal to the ampulla of Vaterabout 30%, themost common site being periampullary [2]. Preampullary lesions with non-bilious vomiting simulating gastric outlet obstruction has been reported recently [1]. We describe a case in which despite high index of suspicion, the diagnosis of distal duodenal stenosis close to duodenojejunal flexure eluded prenatal and post natal detection posing diagnostic and therapeutic challenges.

Case Report

A first baby boy was born by normal vaginal delivery at 30+5/40 weeks with birth weight 1750 grams having trisomy 21 without polyhydramnios. He had mild respiratory distress syndrome requiring nasal continuous positive airway pressure for six weeks in special care baby unit. There was systolic heart murmur in neonatal period and echocardiography showed small ventricular septal defect which closed spontaneously. He passed meconium in first 48 hours but then required daily suppositories to open bowels. He had intermittent non-bilious vomiting up to 20 times a day, constipation with bowels opening every 4 to 5 days and occasional bilious aspirates. On examination, the abdomen was soft, non-distended and nontender. Abdominal film showed normal bowel gas pattern in the immediate post natal period while on continuous positive airway pressure (CPAP) (Fig. 1A).

With the help of suppositories, he had explosive bowel movements and Hirschsprung’s disease was suspected. He therefore underwent rectal suction biopsy which however showed plenty of normal ganglion cells. He was thus commenced on laxatives. Despite this he continued to have same symptoms and subsequently underwent abdominal films showing stomach and duodenum moderately distended but distal bowel gas visible. He previously had normal bowel gas distribution while he was on CPAP, for these reasons an upper gastro intestinal obstruction was not suspected (Fig. 1B-1D).

Figure 1. Plain abdominal films at various periods from birth to diagnosis

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He continued to have constipation, failure to thrive with centile dropping from 9th to 2nd centile with frequent vomiting 6 to 12 per day despite full antireflux medications. It was now noted that on occasions, vomits were bile stained. He therefore underwent upper gastrointestinal contrast study. This showeddilatation of the stomach and duodenum with delayed transit of contrast through a significantly narrowed third part of duodenum with a distinct change of caliber and without evidence of malrotation. The appearances did not appear typical of a duodenal web and duodenal stenosis or a ‘wind-sock’ was more thought to be more likely (Fig. 2).

Figure 2. Upper Gastrointestinal contrast study showing stenosis (arrows) at the junction of 3rd and 4th part of duodenum beyond the superior mesenteric pedicle impression

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He underwent exploratory laparotomy at 3 months corrected age. At exploration, there was grossly dilated stomach and duodenum to the third part and there was calibre change at the junction of 3rd part with the 4th part of duodenum but no evidence of a web or external band. The duodeno-jejunal flexure was normally positioned. There were multiple lacteals throughout the entire small bowel mesentery. The options of Strong’s manoeuvre converting normal rotation into malrotation, dudenoduodenostomy (technically impossible as the stricture was distal duodenal), and duodenojejunostomy were considered. On balance, the last alternative was selected. A transverse incision was made on the dilated duodenum and the absence of a diaphragm or web was confirmed. A feeding tube size 8 could not be passed into the 4th part of duodenum. A longitudinal incision was made on the proximal jejunum just beyond the flexure and tension free duodeno-jejunostomy was carried out uneventfully. No trans anastomotic tube was employed.

His initial post-operative period was uneventful and then he had nasogastric tube aspirates ranging from 400 to 750 ml in the following few days. A decision to start total parental nutrition was made and he underwent Broviac line insertion under general anaesthesia. The aspirates started falling in the second postoperative week. He was regaining bowel movements and feedings were started on 12th post-operative day which he tolerated slowly and steadily and was discharged home on 16thpost-operative day. At follow up at 3 months he was thriving well and was on antireflux medications. At 1 year, he is progressing well and weaning from his antireflux medications.

Discussion

Complete duodenal obstruction is often associated with polyhydramnios. This important clue could be absent in partial duodenal obstruction with stenosis. There is a very high incidence of duodenal anomalies in Down’s syndrome and although prenatal team was actively looking for it, the partial obstruction could not be detected antenatally. In duodenal atresia, passage of meconium at birth is exceptional and invariably a very good clinical indicator of the underlying complete obstruction [2]. Our patient passed meconium in the first 48 hours but then developed continued constipation. When the initial plain film was taken,the baby was on nasal CPAP, the gas ingested was significant and so was the distribution along the entire gut. This misled us to the possibility of Hirschsprung’s disease requiring rectal suction biopsy. It also misled the radiology team as each time reference was made to previous normal bowel gas pattern thus delaying early detection.

Congenital duodenal obstructions presents with bilious vomiting and are relatively uncommon in the distal duodenum, the most common site being just at the ampulla. Presentation with hematemesis has been reported [4]. Failure to thrive during weaning period may unmask the underlying partial obstruction as happened in our case [ 5]. The pitfalls in the diagnosis of partial duodenal obstructions have been described by previous authors [6-10]. Partial duodenal obstruction is known to present later on in life [11].

Plain radiographs that demonstrate the doublebubble appearance with no distal gas is characteristic of complete duodenal obstruction but not of stenosis or partial obstruction. The important lesson from our case was that even in the absence of bilious vomiting, an upper gastrointestinal contrast study is the gold standard to detect partial obstructions and that there needs to be a low threshold in performing this study. Upper gastrointestinal contrast studies will delineate the nature and site of the obstruction. Presence of a dimple on fluoroscopy suggests possibility of a duodenal web [12].

The distal duodenal stenosis was not only a diagnostic challenge with all the odds as mentioned above but has several therapeutic challenges and difficult options. Theoretically duodenal stenosis can best be treated with duodenoplasty with a longitudinal incision along the axis of the bowel and suturing it transversely. This is feasible in first and second parts as they are relatively mobile but the 3rd part is firmly fixed with pancreas superiorly, superior mesenteric pedicle on the medial aspect and the Duodenojejunal flexure on the lateral aspect making this option less attractive and more prone to leak and stenosis. Endoscopic duodenotomy at the stenotic site is not easily approachable and fraught with danger of perforation and bleeding in the stenotic area [13]. Laparoscopy may have the same difficulties [14].

The option of Strong’s manoeuvre to undo the normal rotation into non rotation is a major undertaking and is associated with prolonged ileus, chylous ascites and postoperative adhesions. Isoperistaltic duodeno-jejunostomy entails a larger loop of 4th part of duodenum and proximal jejunum predisposing to blind loop syndrome and malabsortion with bacterial overgrowth. Under the circumstances we selected antiperistaltic loop direct anastomosis which led to increased aspirates temporarily in the postoperative period.

The passage of trans anastomotic tube across the anastomosis may have helped in starting the nasojejunal feeds early and may have helped avoiding insertion of the central line as shown by recent studies in reducing the need for the central line and total parenteral nutrition [15-18].

In conclusion, distal duodenal stenosis in association with Down syndrome could be a diagnostic and therapeutic challenge despite high index of suspicion. We recommend actively look for partial obstruction and if the baby is on CPAP, the interpretation of the plain film, particularly the gas distribution should be with caution. The upper gastrointestinal contrast study remains the gold standard for early diagnosis and threshold should be low. Once diagnosed, all available surgical options should be considered and the best one implemented.

 

 

References:

1. Patel RV, Kumar H, More B. Preampullary duodenal web simulating gastric outlet obstruction. J NeonatSurg 2013; 2: 13-15.

2. Potts SR, Garstin WIH. Neonatal duodenal obstruction with emphasis on cases with Down's syndrome. Ulster Med J 1986; 55: 147–50.

3. Gray SW, Skandalakis JH: The embryological basis for treatment of congenital defects. In: Embryology for surgeons. Philadelphia, PA, Saunders, 1972, pp.: 177-217

4. Chabra R, Suresh BR, Weinberg G, et al. Duodenal atresia presenting as hematemesis in a premature infant with Downsyndrome. J Perinatal 1992; 12:25-27.

5. Sarkar S, Apte A, Sarkar N, Sarkar D, Longia. Vomiting and food refusal causing failure to thrive in a 2 year old: an unusual and late manifestation of congenital duodenal web. BMJ Case Reports 2011; doi:10.1136/bcr.01.2011.3779

6. Richardson WR, Martin LW. Pitfalls in the surgical management of the incomplete duodenal diaphragm. J Pediatr Surg 1969; 4:303- 312.

7. Oldham K, Aiken JJ. Congenital pyloric stenosis and duodenal obstruction. In: Fischer JE, Bland KI, editors. Mastery of Surgery. 5th ed. Vol. I Philadelphia: Lippincott Williams & Wilkins; 2007. p. 902- 09

8. Escobar MA, Ladd AP, Grosfeld JL, West KW, Rescorla FJ, Scherer LR 3rd, et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg. 2004; 39:867-71; 867-71.

9. SarinYK, Sharma A, Sinha S, Deshpande VP. Duodenal webs: an experience with 18 patients. J Neonat Surg 2012; 1: 20-24.

10. Applebaum H, Lee SL, Puapong DP. Duodenal Atresia and Stenosis; In: Grosfeld JL, O’Neill JA Jr, Coran AG, Fonkalsurd EW, editors. Pediatric Surgery, 6th ed., Vol II, Philadelphia: Mosby Elsevier; 2006. p. 1260-68.

11. Serracino-Inglott F, Smith GH, Anderson DN. Duodenal webs-- no age limit. HPB (Oxford). 2003; 5:186-7.

12. Dwek JR, Teich S. The duodenal dimple: a specific fluoroscopic correlate to the duodenal web. PediatrRadiol 1999; 29:467-8.

13. Blanco-Rodríguez G, Penchyna-Grub J, Porras-Hernández JD, Trujillo-Ponce A. Transluminal endoscopic electrosurgical incision of fenestrated duodenal membranes. PediatrSurgInt 2008; 24:711- 4.

14. Steyaert H, Valla JS, Van Hoorde E. Diaphragmatic duodenal atresia: laparoscopic repair. Eur J PediatrSurg 2003:414-416

15. Hall NJ, Drewett M, Wheeler RA, Griffiths DM, Kitteringham LJ, Burge DM. Trans-anastomotic tubes reduce the need for central venous access and parenteral nutrition in infants with congenital duodenal obstruction. PediatrSurgInt 2011; 27:851-5.

16. Patel RV, Govani D, Patel R, Dekiwadia DB. Bifid bile duct duodenal web bypass. Response to e-Letter submitted to BMJ case reports by D Sfoungaris, Aristotelian University of Thessaloniki, Patel RV, Govani D, Patel R, Dekiwadia DB. Neonatal dudeno-duodenostomy and missed duodenal stenosis with windsock deformity: a rare intraoperative error of technique and judgement by an unwary surgeon. BMJ Case Rep 2014: January

17. Patel RV, Govani D, Patel R, Dekiwadia DB. Neonatal dudenoduodenostomy and missed duodenal stenosis with windsock deformity: a rare intraoperative error of technique and judgement by an unwary surgeon. BMJ Case Rep 2014;15 Jan

Junaid Ashraf¹, Rakesh Thakur¹, Ruth Hallows¹, Varadaraja Kalidasan²

¹Department of Paediatric Urology, Leeds General Infirmary, Leeds, UK

²Department of Paediatric Surgery, Royal Alexandra Children's Hospital, Brighton, UK

 

Correspondence:

Junaid Ashraf

Department of Paediatric Urology

Leeds General Infirmary

Great George Street, Leeds

West Yorkshire, LS1 3EX, UK

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

 

Abstract

We are reporting first case of duodenal atresia occurring in both members of premature low birth weight monochorionic diamniotic twins of same sex. The twins were born at 28 weeks of gestations with no dyspmorphic features and normal chromosomes. One of the twins had type II duodenal atresia in the second part of the duodenum along with meckel’s diverticulum which was also excised. The other presented with duodenal web at the junction of second and third part of duodenum. He also underwent an elective left sided inguinal hernia repair at five months of age. They underwent delayed repair at 3rd and 4th weeks of age respectively. Both babies had an uneventful postoperative course.

Keywords: duodenal atresia, duodenal web, monozygotic twins

doi: 10.21614/jpss.11.1.29

citation: J Pediatr Surg Spec. 2017;11(1):29-32

 

Introduction

The incidence of duodenal atresia is about 1.4 per 10,000 live births in a singleton pregnancy [1] and there have been reports of occurrence in the same family [2]. However, duodenal atresia in identical twins is exceedingly rare. Duodenal atresia includes a range of anomalies from a simple duodenal diaphragm, duodenal stenosis to complete discontinuity. Associated conditions include Down’s syndrome (30%) and VACTERL anomalies (Vertebral, Anal, Cardiac, Tracheo Esophageal, Renal and Limb anomalies) in about 50% cases. We present a previously unreported case of isolated duodenal atresia and duodenal web in same sex mono-zygotic identical twins.

Family and Antenatal History

There is no history of consanguineous marriage in the family pedigree. Their mother was only 17 years old primipara white Caucasian. There is no history of any maternal ingestion of drugs, infections or irradiation exposure. Her antenatal scans at 14 weeks diagnosed monochorionic diamniotic pregnancy. At 21 weeks, polyhydramnios along with double bubble were seen in one foetus (case 1). Throughout the pregnancy, the umbilical vascular Doppler flow was reported to be normal. At 28 weeks of gestation, owing to failure to progress of spontaneous labour with breech presentation, an emergency caesarean section was carried out.

Case 1

A male baby weighing only 1010 grams was born at Gestation 28+4 weeks. Baby had to be ventilated because of respiratory distress of newborn; however he was extubated onto continuous positive airway pressure (CPAP) on day 4 of life. An echo done found a structurally normal heart with a small patent ductus arteriosus (PDA), though, the later scans showed no evidence of PDA. A plain abdominal X ray taken on 1st day of life showed a large stomach air bubble and dilatation of the first portion of the duodenum (Fig. 1). No air was seen distal to the proximal duodenum. An ultrasound abdomen was normal, ultrasound of brain prominent lateral ventricles and possibly small old intraventricular haemorrhage in left ventricle. Laparotomy was performed using transverse supraumbilical incision on the right side. Duodenal atresia with annular pancreas involving the junction of first and second part of duodenum was found and also a Meckel's diverticulum. A side to side wide duodeno-duodenostomy was carried out along with excision of Meckel's diverticulum and end to end bowel anastomosis. A feeding tube was passed through the anastomosis for post operative jejunal feeding.

Figure 1. Plain Abdominal X ray of Case 1, taken on 1st day of life showing a large stomach air bubble and dilatation of the first portion of the duodenum, the so called ‘Double Bubble’ of Duodenal Artesia. Note the absence of gas distal to the dilated duodenum.

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Case 2

The twin brother of case 1 baby weighed 1130 grams at birth. This baby was also ventilated at birth due to respiratory distress of newborn but extubated next day and weaned to optiflow by day 9. An echo done on day 1 showed a small PDA but a repeat echo after a week was absolutely normal. During first few days he did not tolerate enteral feeds and was developing abdominal distension with increased biliary aspirates. An abdominal X ray showed normal gas pattern with air up to the rectum (Fig. 2). An upper gastrointestinal contrast study showed grossly distended descending limb with a liner filling defect (Fig. 3) at the junction of second and third segment of the duodenum raising the possibility of a partially obstructing duodenal web. Contrast was seen to pass through the narrowed segment and opacify the proximal jejunum. He underwent laparotomy 5 days after his brothers surgery through the similar supraumbilical right transverse incision. A duodenal diaphragm with a central hole identified. The duodenum was mobilized, opened transversely in its first segment, duodenal diaphragm was identified and resected, a feeding tube passed into the jejunum before closing the duodenotomy. He also underwent an elective left sided inguinal herniotomy before discharge.

Figure 2. An abdominal x ray of Case 2 showing normal gas pattern with air upto the rectum.

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Both babies had an uneventful postoperative course and were on full feeds via bottle at discharge. Chromosomal studies in both babies were normal, had no dysmorphic features, no signs of Down’s syndrome or associated congenital abnormalies of the genitalia or anorectum and no limb deformities.

Figure 3. An upper gastrointestinal contrast study done in Case 2, showing grossly distended stomach as well as duodenal descending limb with a liner filling defect (black arrow) at the junction of second and third segment of the duodenum raising the possibility of a partially obstructing duodenal web. Contrast seen to negotiate the narrowed segment and opacify the proximal jejunum

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The twins are monozygous and monochorionic on the basis of a single, shared placenta, same sex and same blood group as well. They were oxygen dependent initially and stayed on optiflow or oxygen via nasal cannula but at discharge both mainted oxygen saturations without oxygen supplementation. Also, at discharge, both babies had resolving conjugated bilirubinaemia most likely secondary to parental nutrition. There was also evidence of metabolic bone disease requiring supplementation of phosphate and alfa calcidol at discharge.

Discussion

We are reporting first ever case of duodenal atresia (DA) in monochorionic diamniotic twins of same sex, one with unlinked type duodenal atresia in the second portion of duodenum and other with duodenal web between the second and third part of duodenum. Fonkalsrud et al. [2] reported duodenal atresia in six twin patients but they did not state whether both members of the pair had duodenal atresia. There is only one other report of monochorionic diamniotic twins with duodenal atresia by Enrico De Grazia [3], but their twins had intestinal atresia along with duodenal atresia. Also, the member twins were of different sexes, one male and other female. Takahide et al. [4] have also reported duodenal atresia in both members of the twins but their twins were diamniotic dichorionic gestation. There are at least 2 other reports of duodenal atresia in twins [5, 6] but they do not state whether the twins were identical or dizygotic. Our case therefore is the first reported case report of isolated duodenal pathology in monochorionic diamniotic twins involving same sex.

Duodenal atresia is an extremely uncommon congenital malformation. In a review by Cragan et al. [1] the incidence of duodenal atresia in singleton pregnancy has been estimated to be 1.4/10,000 but the incidence of DA in twin pregnancy is higher, 2.4 pre 10,000 (relative risk, 1.8; 95% CI, 0.6, 5.6). This higher incidence in familial DA supports the theory that this anomaly may be determined by genetic factors. Structural malformations that are not genetically determined obviously are seen in only one member of the monozygotic twins. It has been estimated that overall in around 15% of the cases both twins are affected by at least one structural anomaly while mostly only one twin is affected [7].

The etiology of duodenal atresia still remains unclear. Tandler J [8] has suggested that duodenal atresia is secondary to failure of recanalization of epithelia plugging by vacuolar coalescence in the solid developing duodenum. On the other hand, various authors including Louw and Barnard [8, 10, 11] have postulated a vascular theory which states that intestinal atresia in the mid gut is a result of interference of blood supply to a segment of the fetal bowel.

At the same time, there are certain environmental factors which are generally implicated in relation to congenital anomalies including various drugs ingestion during pregnancy, maternal infections and exposure to irradiation. Esterly JR et al. [12] have reported an association between jejunal atresia in both members of twins following interruption of the blood supply to the fetal jejunum secondary to maternal rubella infection.

Several clinical reports have been reported suggesting a genetic cause of congenital DA as well as other intestinal atresia with an autosomal recessive inheritance [13]. These occurrences of DA in both members of our identical twins may suggest that they may be expression of a single gene responsible for such rare condition. However, no specific gene has been identified so far that can be linked to the development of DA in humans.

Fairbanks TJ et al. [14] have published a study evaluating the role of Fibroblast growth factor receptor 2b (Fgfr2b) which is a critical developmental regulator of proliferation and apoptosis in multiple organ systems including the gastrointestinal tract (GIT). Fgfr2b invalidation results in an autosomal recessive intestinal atresia phenotype. The study done on pregnant mice suggests an innovative idea that absence of embryonic GIT Fgfr2b expression results in decreased proliferation and increased apoptosis resulting in GIT atresia including DA.

Fairbanks [15] also showed that vascular occlusion does not lead to all intestinal atresia but they can result from a genetic defect in the development as well as vascular occlusion; the antenatal expression of growth factor like FgF10 and Fgfr2b is a critical step towards normal gastrointestinal development, without this step and possibly associated critical genes failed expression, development defects are likely to occur.

 

 

References:

1. Cragan JD, Martin ML, Moore CA, et al. Descriptive epidemiology of small intestinal atresia, Atlanta, Georgia. Teratology 1993; 48:441- 450.

2. Fonkalsrud EW, de Lorimier AA, Hays DM: Congenital atresia and stenosis of the duodenum. Paediatrics 1969; 43:79-83.

3. De Grazia E, Di Pace MR, Caruso AM, Catalano P, Cimador M. Different types of intestinal atresia in identical twins. J Pediatr Surg 2008 ;43(12):2301-4.

4. Yokoyama T, Ishizone S, Momose Y, et al. Duodenal atresia in dizygotic twins. J Pediatr Surg 1997;32:1806–1808.

5. Best LG, Wiseman NE, Chudley AE. Familial duodenal atresia: a report of two families and review. Am J Med Genet 1989; 34:442- 444.

6. Gross E, Armon Y, Abu-Dalu K, et al. Familial duodenal atresia: a report of two families and review. Am J Med Genet 1989;34:442– 444.

7. Puri P, Fujimoto T. New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg 1988;23:221–225.

8. Tandler J. Zur Entwicklungsgeschichte des menschlichen Duodenums in fruhen embryonalstadien. Morph Jahrb 1900;29:187–216

9. Louw JH, Barnard CN. Congenital intestinal atresia. Lancet 1995;2:1065–1067.

10. Stefanutti G, Gamba P, Midrio P. Intestinal atresia and agenesis of the gallbladder in 2 siblings. J Pediatr Surg 2006; 41:E31–E32.

11. Rustico MA, Baietti MG, Coviello D, et al. Managing twins discordant for fetal anomaly. Prenat Diagn 2005; 25:766–771.

12. Esterly JR, Talbert JL. jejuna atresia in twins with presumed congenital rubella. Lancet 1969; 1: 1028-1029.

13. Mishalany HG, Idriss ZH, Der Kaloustian VM. Pyloroduodenal atresia (diaphragm type): An autosomal recessive disease. Pediatrics 1978; 62:419-421.

14. Fairbanks TJ, Sala FG, Kanard R, et al. The fibroblastic growth factor pathway serves a regulatory role in proliferation and apoptosis in the pathogenesis of intestinal atresia. J Pediatr Surg 2006;41(1):132–136.

15. Fairbanks TJ, et al. Colonic atresia without mesenteric vascular occlusion. The role of the fibroblastic growth factor 10 signaling pathway. J Pediatr Surg 2005;40:390–396.

Mohamed Shoukry, Munther Haddad

Paediatric Surgery Department, Chelsea and Westminster Hospital, London, UK

 

Correspondence:

Mohamed Shoukry

Paediatric Surgery Department

Chelsea and Westminster Hospital

369 Fulham Road, London, UK ,SW10 9NH

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

 

Abstract

Aim of study: Literature review of complications in laparoscopic repair of complete rectal prolapse in children and reporting of unusual presentation of adhesive bowel obstruction.

Materials and method: We present the case of an 11-year-old healthy girl has been referred with persistent rectal prolapse. Patient underwent laparoscopic rectosigmoidopexy with mesh. Two months post-surgery, she presented with symptoms of bowel obstruction. Laparotomy findings confirmed 2-bands of adhesions adherent to mesh. Post-operative was uneventful. We also review published English language literatures.

Discussions: Six series adopted laparoscopic treatment for persistent rectal prolapse in childhood. Median age was 6.9 years. No intraoperative complications were reported. Postoperative complications were reported with no significant morbidity and mortality. We report first case with adhesive bowel obstruction post laparoscopic mesh repair.

Conclusion: Small number of trials reported role of laparoscopy in management of complete rectal prolapse becomes common and improving functional outcome. Surgical complications can still happen; however current practise doesn’t show significant morbidity and mortality.

Keywords: laparoscopic repair, rectal prolapse, children, complications.

doi: 10.21614/jpss.11.1.25

Citation: J Pediatr Surg Spec. 2017;11(1):25-28

 

Background

Rectal prolapse is a relatively common condition in children. It is usually a self-limited problem and requires no surgical treatment. For children with persistent rectal prolapse, a variety of surgical procedures have been described with relative success. Recently, there are many reports addressing successful use of different laparoscopic approaches for complete rectal prolapse which become the standard choice for management. Laparoscopic non-absorbable mesh or suture rectopexy are frequently used. It is a feasible and safe procedure; however, complications may occur.

This article is reporting the first case of adhesive bowel obstruction post laparoscopic mesh repair of complete rectal prolapse in children and reviewing similar and further complications of the procedure which may happen.

Materials and Method

We reviewed English language articles published in Medline, PubMed and Cochrane database. The search is based on key words: complications, laparoscopy, complete rectal prolapse and children. Published reviews and articles are retrospective studies carried out after ethical approval is granted from local institutes. Permission from parent has been granted to report the case. The rest of published data have been gathered, tabulated and analysed using the percentage ratio, mean, median and the range for calculations.

Case report

An 11-year-old girl, well and healthy, has been referred from her GP with 10-month history of persistent rectal prolapse which becomes more frequent and difficult to reduce spontaneously. She has been brought to the A&E in few occasions for manual reduction. It is coming to an embarrassing point which is interfering with her usual daily physical activity. She has no previous history of diarrhoea or constipation. On physical examination, abdomen is soft, lax and no masses were felt. Digital rectal examination excluded rectal ulcers, fissures and any gross pathology. Oral laxative has been prescribed by gastroenterologist but with no successful response which was followed by unremarkable colonoscopy. Routine blood test and Sweat test results are normal.

The patient underwent laparoscopic rectosigmoidopexy with mesh applied to fix the redundant sigmoid colon to the sacral promontory. Postoperative period was uneventful and patient recovered smoothly and regained bowel function on second day before being discharged home. Oral laxative has been prescribed for three months. On follow-up appointment in 6 weeks, she looked well with no active abdominal complaint and no symptoms of recurrence.

A week after, she has been brought to the A&E with symptoms of bowel obstruction (vomiting, abdominal distension and no bowel movements for 3 days). After resuscitation, conservative management commenced with nasogastric tube on free drainage, IV drip and close monitoring for her response. Abdominal X-ray has shown distended bowel loops. Decision has been made for laparotomy and proceeds, which revealed thick fibrous bands obstructing distal ileal loops and firmly adherent to the mesh on the sigmoid colon. Excisions of the obstructing bands, release of intra-abdominal simple adhesions and partial omentectomy have been performed. Smooth post-operative recovery was reported. Patient had satisfactory outcome during out-patient follow-up.

Discussion

Scanty series and few case reports, 6 different series, adopted laparoscopic approach as a standard surgical treatment for persistent rectal prolapse in childhood. The trials are included with total number of 144 patients. All reviews are retrospective studies. Male to female ratio is variable, however boys are predominant. Their median age was 6.4 years (range, 0.4-19 years). The coincident pathologies and underlying pathologies are identified. Patients in available trials are exposed to stool softener, laxative and abundant straining during defecation as conservative management for variable period. However, the persistency of rectal prolapse caused significant distress, pain, occasionally anal bleeding and embarrassment for patients and their families. Post-operative complications are collected and categorized according to the laparoscopic procedure (Table 1). Rectal prolapse is ranging from intermittent protrusion of rectal mucosa that is spontaneously reduced to full thickness rectal wall that requires manual reduction. Complete rectal prolapse is the result of complete circumferential protrusion of 6 to 8 cm of full rectal wall thickness from the anal verge and advanced through the anal canal, everting onto the perineum [7]. It usually happens during defecation. Precipitating factors are weakness of pelvic floor muscles and loosely attached mucosa to underlying muscle coat. However, anatomic variation should be taken in consideration. Patients with primary prolapse tend to have more vertical course of rectum, flatter coccyx and relative low position of rectum [4] Other cofactors are: malnutrition, parasitic disease, straining during defecation and long period of sitting on toilet seat, diarrhoea or constipation [1,3,4]. Approximately 75% of rectal prolapse is associated with constipation. Boys are commonly affected than girls but no statistical significance [1-5]. Peak age is between 1 and 3 years old [3]. However, 20% of cases initially presented between 3 months to 6 years of age due to cystic fibrosis [7].

Table 1. Literature review

11.1 5 1

The vast majority of cases with rectal prolapse don’t need surgery. However, the indication for surgical intervention was warranted to some cases with intractable prolapse and not spontaneously cured. Basically, surgical intervention is not recommended until 1 year of conservative therapy. However, wait and watch period is preferred and could be individualized according to each case. Patients older than 4 years of age require surgery more than younger children [8]. Recently, the laparoscopic approach becomes standard choice for the management of complete rectal prolapse in children or failed other surgical procedures; e.g. posterior sagittal rectopexy and local nonsurgical forms of therapy; e.g.: sclerotherapy [4]. This procedure is used as replacement of trans-abdominal open technique. The rectum is mobilized, retracted from pelvis and fixed to the periosteum of sacral promontory. This can be carried out by using mesh applied in the retro-rectal space [2,3,7] or just suturing the recto sigmoid in multiple locations posteriorly and/or laterally [1,2,3,5,7]. Further retro-rectal dissection and division of lateral rectal ligaments are not recommended by Potter et al. [5]. This is due to risk of injury of blood supply and nerves running through this plane. However, carful exposure of sacral promontory can be achieved [2,3]. The specific goals are to eradicate the external prolapse, improve bowel motility and reduce recurrence [2].

Through laparoscopic view, cases showed redundant recto-sigmoid colon and sigmoid colon resection was reported [2]. The procedure is completed laparoscopically without any conversion to open [2,5]. Use of colonic preparatory rectal enema is documented [2,3] but evolved by Potter et al. later with no documented complications [5]. Antibiotic regime frequently used on induction is cephalosporin and metronidazole [2,5]. Preferable position is supine position [2] or Trendelenburg [5]. Procedure was carried out through three trocars 5mm which are applied by open method and under vision. However extra working trocar for laparoscopic retractor was occasionally applied [2,4]. 30۫ scope and pressure 10-12mmHg are sufficient [2,3,5], 45 scope was used by Laituri etal. [4]. Prolene sutures have been preferred by Ismail et al. [2] however silk suture has been used by Laituri et al. [4]. Choosing between solely suturing or mesh application depends on pelvic floor laxity [2]. The mean duration of surgery was 90 min (range, 40-110 minutes) for mesh and 60 min (range 50-90 minutes) for sutures. Short stay after surgery in most of case series and mean postoperative hospitalization ranges between one day and 6 days [2].

No intraoperative complications were reported such as bleeding, bowel injury [5] one case reported with port-site hernia and wound infection [1]. Postoperative complications and follow up events were reported with no significant morbidity and mortality. Although few cases were lost to follow-up, but the majority were available ranging between 7.2 months and 36 months [2,5]. Although relatively short follow-up period, recurrence is not documented with laparoscopic mesh [2]; however, it is documented when only sutures are used. 5% total recurrence and 11% partial mucosal prolapse have been reported (5).

Constipation happened that responded well to oral laxative treatment. Karvin et al reported 35% incidence of constipation after laparoscopic rectopexy (9). However, Demirbas et al reported reduction of colonic transit time in 50% of patients postoperatively (10). All patients with chronic constipation or anatomical anomalies have commenced laxative medications after surgery. However, only 16% were laxative free post rectopexy on long term follow up (5). Temporary colonic fistula treated conservatively was reported (2). Improvement of postoperative EMG studies during rest and squeezing in all cases was reported except those secondary to neuropathic origin (2).

In the current case, we report the first paediatric patient, published in literature, who developed adhesive bowel obstruction post laparoscopic mesh repair of primary complete rectal prolapse. No outstanding laparoscopic operative findings have been noticed. Optilene™ non-absorbable mesh has been applied with no intra-operative difficulties or complications. Surgical procedure was completed in 35 minutes. Post-operative period was uneventful. Laxative has been prescribed from day one and patient was reviewed well in out-patient clinic with no recurrence of rectal prolapse has been documented. Very short onset (less than 2 months), she developed symptoms of bowel obstruction which is not responding to usual conservative treatment. Intra-abdominal fibrous thick bands caused small bowel obstruction. These bands anchor on the mesh from one side and lateral abdominal wall from other end, however it compresses the small bowel underneath. Surprisingly no bowel ischemia has happened.

Conclusion

Small number of trials have been identified in the paediatric age group. Relative short period of follow-up was noticed. The use of laparoscopy in the management of complete rectal prolapse becomes common and is improving functional outcome. Surgical complications can still happen; in spite of this, current practise doesn’t show significant morbidity and mortality.

 

References:

1. Awad K, El Debeiky M, AbouZeid A, Albaghdady A, Hassan T, Abdelhay S. J. Laparoscopic Suture Rectopexy for Persistent Rectal Prolapse in Children: Is It a Safe and Effective First-Line Intervention? Laparoendosc Adv Surg Tech A 2016;26(4):324-7.

2. Ismail M, Gabr K, Shalaby R. Laparoscopic management of persistent complete rectal prolapse in children. J PediatrSurg 2010; 45(3):533-9.

3. Shalaby R, Ismail M, Abdelaziz M, Ibrahem R, Hefny K, Yehya A, Essa A.Laparoscopic mesh rectopexy for complete rectal prolapse in children: a new simplified technique. PediatrSurgInt 2010; 26(8):807-13.

4. Laituri CA, Garey CL, Fraser JD et al.15-year experience in the treatment of rectal prolapse in children.J PediatrSurg 2010; 45(8):1607-9.

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 PediatrSurg 2010; 45(10):2103-7.

6. Koivusalo A, Pakarinen M, Rintala R.Surg Endosc. Laparoscopic suture rectopexy in the treatment of persisting rectal prolapse in children: a preliminary report. 2006; 20(6):960-3.

7. Gourgiotis S, Baratsis S. Rectal prolapse. Int J Colorectal Dis 2007; 22 (3): 231-43.

8. Rintala RJ and Pakarinen M. Other disorders of the anus and rectum, anorectal function. Pediatric surgery. 6th edition, 2006; 1595-6.

9. KarvinY, Delancy CP, Casillas S. Longterm outcome after laparoscopic and open surgery for rectal prolapse. Surg Endosc 2005: 21.

10. Demirbas S, Akin ML,Kaemoglu M. Comparison of laparoscopic and open surgery for total rectal prolapse. Surg Today 2005; 35(6):446-52.

Kashif Chauhan, Gulwish Moghal, Myriam Guessoum, Nia Fraser, Manoj Shenoy

Pediatric Surgical Department, Queen Medical Centre, Nottingham University, Nottingham, UK

 

Correspondence:

Kashif Chauhan

Pediatric Surgical Department

Queen Medical Centre, Nottingham University NHS Trust

Derby Road, Nottingham, NG7 2UH, UK

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

 

Abstract

Background:Traditional teaching states that if the base of a sacral dimple is visible, associated spinal cord abnormalities are unlikely. In our experience, we noted that in several patients this was not true. We therefore set out to evaluate the incidence of spinal cord abnormalities in a cohort of children with sacral dimple.

Objective: Our objective was to determine the frequency of spinal cord abnormalities detected on whole-spine MRI in children with pure sacral dimple (cutaneous dimple without associated local pigmentation or hairy patch). The frequency of neurosurgical intervention in those with a spinal cord malformation was also calculated.

Materials and methods: We retrospectively reviewed all patients under the age of 16 years with clinically confirmed pure sacral dimple, who had undergone spinal MRI between 2005 and 2016. Patients with coccygeal pits were excluded from the study. We analyzed the MRI findings, calculated the incidence of cord abnormalities, and proportion of those who subsequently underwent neurosurgical procedures, such as spinal cord untethering. Other associated abnormalities were documented.

Results: During the 11-year study period, 33 patients (19 boys) with confirmed sacral dimple and whole-spine MRI were identified. The median age at the time of MRI was 21 months (range 2 months to 13 years). Eleven patients (33.3%) had abnormal cord detected on the MRI. Out of them, 4 had tethered cord, 2 had low lying cord (L2/3 level). Five had lesions related to the filum terminale (dermoid, cyst, lipoma). Of the four patients with tethered spinal cord, 2 had untethering procedures, 2 so far have been managed conservatively. Of the 7 patients with other cord abnormalities, 1 had excision of sacral dermoid. Nine of the 33 patients (27%) had other congenital anomalies, including 5 with syndromes.

Conclusion: In our experience, the incidence of spinal cord abnormalities in children with pure sacral dimple was 33.3%, some of which required surgery. Other congenital abnormalities were common. We recommend whole spinel MRI and a careful search for other congenital abnormalities.

Keywords: sacral dimples, spinal cord malformations, coccygeal pits, tethered spinal cord.

doi: 10.21614/jpss.11.1.20

Citation: J Pediatr Surg Spec. 2017;11(1):20-24

 

Introduction

A simple sacral dimple in children is defined as a dimple located within 2.5 cm from the anus, superficial, and less than 0.5 cm in diameter – with no additional associated cutaneous markers, such as local pigmentation or a hairy patch in children [1, 2]. Sacral dimples are common, occurring in up to 3-8% of the population [3]. The majority of these dimples are minor and do not represent any underlying disease [3]. However, they can manifest with serious and long term neurological, urological and cutaneous abnormalities [4]. They are usually detected in post-natal checks by a paediatrician, and the following features are assessed.

• Is the base of the dimple visible? If not, this may indicate that the neural tube is not completely closed.

• Is there a tuft of hair in the dimple? This may also indicate a neurological abnormality.

• How close to the buttocks is the dimple? The lower it is, the lower the risk of neurological abnormalities.

• Is the lower limb neurology, bladder and bowel function normal?

• Are there any other additional features of a congenital syndrome? Examples include Miller Dieker and Smith-Lemli-Opitz syndrome [5].

Sacral dimples are the most commonly seen cutaneous anomalies on paediatric spinal examination [5]. The more obvious lesions may be a manifestation of underlying tethered cord, justifying spinal cord imaging [1][2], such as spinal MRI scan [5], or in younger babies, spinal ultrasound initially.

However, given that the presence of a simple sacral dimple in an otherwise healthy child is not always associated with underlying spinal cord tethering [1], there have been studies suggesting that further evaluation with imaging is not necessary [2]. In fact, there is no clear guideline on an appropriate diagnostic work-up in the small subset of patients with pure sacral dimple and no overlying skin changes [1].

In our experience, we have found that many of our patients with a pure sacral dimple do have associated spinal cord abnormalities. Thus, the aim of this study was to evaluate the incidence of spinal cord abnormalities in children with a pure sacral dimple.

Material and Methods

We performed a retrospective study of all children with a clinically confirmed simple sacral dimple who underwent spinal MRI at Queens Medical Centre, Nottingham, between 2005 and 2016.

We requested the list of patients who had MRI of the spine to assess the sacral dimple in children under the age of 16, performed in the last 11 years. Patients with coccygeal pits and children in whom the dimples had overlying pigmentation or hairy patches were excluded from the study.

Details of all patients included were then accessed from our patient database. We collected data on patient demographics, presenting symptoms, and features of the dimple but also associated abnormalities, particularly of neurological, urological or bowel-related problems, in addition to making note of whether there were any clinical features suggestive of associated underlying syndromes (including whether these were later confirmed). Past medical and surgical history was also recorded.

Imaging performed in addition to the MRI was also recorded. In most cases, this included ultrasound (US) scans performed prior to referral, and a MRI of the brain in a few patients. The findings of the spine MRI were recorded, followed by indication for further management: “reassured and discharged” if MRI was normal, “conservative with regular follow up” if an abnormality was present where surgery was not required at that time or “surgical intervention”, for significant cord pathology.

We then calculated the incidence of spinal cord abnormalities within the 11-year period, and the proportion of these patients who subsequently underwent neurosurgical intervention.

Results

During the 11-years study period, 33 patients under the age of 16 underwent whole-spine MRI to further assess a simple sacral dimple. Of the 33 patients, 19 were male (57.5%) and 14 were female (42.4%). The age of the patients ranged from 2 months to 13 years with a median of 21 months. Five patients (15%) had associated syndromes, including Cornelia de Lange, Keratitisichthyosis- deafness (KID) syndrome, Aicardi- Goutières, caudal regression syndrome, one unknown syndrome. Nine patients (27%) had neurological abnormalities such as hypotonia, global developmental delay and gross motor problems. Thirteen patients (33%) had general urological problems such as a duplex kidney, hypospadias, urine incontinence or a bladder diverticulum. Three patients (9%) had cardiac abnormalities, such as tetratology of fallot (TOF), ventricular septal defect (VSD) or atrial septal defect (ASD) (Fig. 1).

Figure 1. Sacral dimple patients with related problems

11.1 4 1

In terms of previous surgical history, of the 33 patients, 1 had a duodenal atresia repaired, 2 had undergone pyloromyotomy, another 2 had major cardiac surgery (TOF, VSD and ASD repair, pulmonary valvutomy), 2 underwent hypospadias repair and 1 underwent a percutaneous nephrolithotomy (PCNL) of the right kidney.

Seven patients had a spinal ultrasound performed prior to being referred. Five of these were normal while 2 required a spinal MRI to further assess a low-lying cord. Three patients also had a renal ultrasound performed (all normal) and 8 also had brain MRI. Of the latter, 1 showed ventricular enlargement, and one showed mild prominence of extra cerebral CSF space. The remaining 6 patients had a normal MRI of the brain. In terms of neurological abnormalities, 11 patients (33.3%) had an abnormal cord detected on the whole spine MRI. Of the 11 patients, 4 (12.1%) had a tethered cord, 2 (6.06%) had a low-lying cord (L2/3 level) and 5 (15.2%) had lesions related to the filum terminale (dermoid, cyst, lipoma).

Of the four patients with a tethered spinal cord, 2 underwent untethering procedures for symptoms, whilst the remaining 2 are currently being managed conservatively with regular follow up. Of the remaining 7 patients with other cord abnormalities (low lying cord, filum terminale lesions), 1 underwent an excision of a sacral dermoid. All of these 7 patients had a good outcome (Fig. 2). It was noted that of the 11 patients with an abnormal cord found on MRI, 3 had associated syndrome (Cornelia de Lange, Keratitis-ichthyosisdeafness (KID) syndrome, Aicardi-Goutières) and there was a relatively higher incidence of urological related problems. In summary, of the 33 patients with a simple sacral dimple, 9 (27%) had congenital anomalies, 11 (33%) had spinal cord abnormalities (including 3 syndromic children) with only 4 (12%) found to have a tethered cord.

Figure 2. Spinal cord abnormalities; SC – spinal cord.

11.1 4 2

Discussions

Previously published studies have suggested that the risk of spinal malformations in asymptomatic, healthy infants with an isolated simple sacral dimple is exceedingly low [1]. Alongside this, several studies have suggested that there is little value in investigating a simple sacral dimple with further imaging [5], whilst others suggest simply performing a screening ultrasound scan [1]. Importantly, there is no clear guidance on an appropriate diagnostic workup in this group of children [1]. Nevertheless, through the 11-year period of our study, we have demonstrated a high yield (33.3%) of spinal cord abnormalities on a MRI of the spine, emphasizing the importance of imaging in patients with a simple sacral dimple. This has not been demonstrated in previous studies.

Kucera et al. [1] performed a 12-year study in children with a simple sacral dimple in USA. From a large cohort of asymptomatic children, only 2.1% of children were found to have spinal cord abnormalities on a spinal US or MRI scan. Out of 3,884 children, only 128 (3.3%) had an abnormal US of the spine, with 76 (2.1%) children found to have a spinal abnormality on follow up imaging. In this study, the large patient selection would allow the results of this study to be easily applied to other practices. Although our data was collected over an 11-year period, the number of patients who had a MRI of the spine to investigate a simple sacral dimple was only 33, which may be an important limitation in our study.

Although the Kucera et al. study [1] had a large patient sample, this consisted of patients who had a screening ultrasound of the spinal cord performed for a simple sacral dimple. A follow up MRI or US was then reviewed in all those with an abnormal screening US scan. However, this would exclude several patients who had a US reported as normal, although further imaging may have shown an abnormality. Our inclusion criteria consisted of all patients that had an MRI spine requested to investigate a simple sacral dimple. Within this group, a small proportion of patients had an US performed prior to referral. We found that not all of the US findings correlated with the subsequent MRI findings. In fact, at least one patient had an US scan reported as normal, only to have a follow up MRI scan showing tethering and a cutaneous sinus, requiring surgical un-tethering. Thus, this further emphasizes the importance of requesting an MRI of the spine for all patients with a simple sacral dimple, as an US scan alone can be inadequate. This may be due to the fact that an US is user - dependent, and requires an experienced sonographer.

Sonographic examination of the paediatric spinal canal is accomplished by scanning through the normally incompletely ossified posterior elements of spine. It is most successful in the new born period and in early infancy. Infants older than 6 months, the examination is very limited [6].

Another limitation is the retrospective nature of our study. Since the method of collecting data was to include all whole spinal MRI scans investigating a simple sacral dimple, this would not have included patients who may have been identified as having a simple sacral dimple, but either not referred, were investigated with an US scan with no follow up MRI, or had the MRI performed elsewhere. Thus, our patient selection could under-represent actual data. Our center is a tertiary center and we receive referrals from all over our region. Patients with sacral dimples who complains of symptoms like wetting, incontinence, urgency or urinary tract infections are referred to us for review and further investigations. They are reviewed in our urological department and we request spinal MRI scans to rule out any pathology. If there is any associated and related pathology we referred it to the neuro-surgical department for their opinion and review. All the patients referred to our urological department or neurosurgical department had a spinal cord imaging. As we are a tertiary center not all patients within our catchment area are referred to us, as they are managed locally.

Another recent study addresses concerns regarding investigating a simple sacral dimple for underlying spinal cord malformations with ultrasound. Albert GW [7] has looked into nine papers where 3.4% of 5,166 patients had an abnormal USS finding, although most of these findings were of no clinical significance. However, given the fact that a missed tethered cord in a patient with a simple sacral dimple can lead to significant neurological dysfunction, orthopaedic deformities, as well as genitourinary and gastrointestinal abnormalities [4], we believe that the low percentage of patients that require surgical intervention as shown in previous studies should not prevent further investigation in all patients with a simple sacral dimple. Of the 33 patients, 12% were found to have a tethered cord.

The percentage of children with a tethered cord in our dataset is exceptionally high, and this further supports our department protocol to obtain further imaging in patients with a simple sacral dimple. In our tertiary center, a selected group of patients are referred from primary care or district centers. These patients are of concern or are having urological problems. Initially they will have been reviewed by the local GP or a pediatrician, and managed at that level. Depending upon the initial examination and symptoms they are then referred to our urological department for review and further management. Only patients with problems are referred to our urological or neurosurgical department. They all have had a MRI of the spine as they were difficult to treat or having ongoing problems. This study has provided new information, and at present, will support our departmental guidelines in terms of organizing a whole-spine MRI scan for all children with a pure sacral dimple. Further research is required with a larger sample of patients, ideally in a prospective study, in order to provide results that can be applied nationally.

Conclusion

In our experience, the incidence of spinal cord abnormalities in children with a simple sacral dimple was 33.3%, with just over a third (12%) with a tethered cord. Surgery was only required in 3 (9.09%) patients. Other congenital abnormalities were also common in this set of patients. As a result, we recommend that all patients with a simple sacral dimple should undergo wholespinal MRI. There should also be a careful search for other congenital abnormalities, as these were common in our study group.



References:

1. Kucera JN et al. The simple sacral dimple: diagnostic yield of ultrasound in neonates. Pediatr Radiol 2015;45(2):211-6

2. Gomi A, Oguma H, Furukawa R. Sacrococcygeal dimple: new classification and relationship with spinal lesions. Childs Nerv Syst 2013;29(9):1641-5.

3. Flannigan C. A practical guide to managing pediatric problems on the postnatal wards. Eds: Radcliffe Publishing 2011: 43- 44.

4. Safavi-Abbasi S et al. History of the current understanding and management of tethered spinal cord. J Neurosurg Spine 2016; 11:1-10

5. Kumar A., Kanojia RK, Saili A. Skin dimples. Int J Dermatol 2014; 53(7): 789–797.

6. AIUM Practice parameters for the performance of an ultrasound examination of the neonatal and infant spine. 2016 http://www.aium.org

7. Albert GW. Spinal ultrasounds should not be routinely performed for patients with simple sacral dimples. Acta Paediatr 2016; 105(8):890-4.

Prisca Radu-Alexandru¹, Toth Tamas², Gozar Horea¹²

¹Department of Pediatric Surgery and Orthopedics, Emergency Clinical Hospital TârguMureş, Romania

²University of Medicine and Pharmacy TârguMureş, Romania 


Correspondence:

Radu-Alexandru Prisca

Department of Pediatric Surgery and Orthopedics

Emergency Clinical Hospital TârguMureş

Gheorghe Marinescu street, Nr 50

TârguMureş, Romania

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


Abstract

Introduction: Fingertip injuries are the most common injuries in pediatric hand trauma. The goal in fingertip amputation reconstruction is to cover the defect, establish maximum tactile gnosis, and protect the joint function and none the less to obtain a satisfactory cosmetic appearance. The plane of the amputation and the condition of the tissue at the injury site help to determine the best repair technique.

Materials and Methods: Thirty pediatric patients underwent fingertip reconstruction using the V-Y advancement flap and cross finger flap techniques at the Department of Pediatric Surgery in Târgu Mureş between January 2015 and December 2016. The used technique was chosen according to the nature of the injury, the size and condition of the defect, the affected fingertip, the plane and the zone of the traumatism. During the follow up visit the two point discrimination test was determined. The parents compared the cosmetic results with the contralateral fingertip and they rated it on a 1-10 scale.

Results: Cross finger flap was used in 14 cases and V-Y advancement flap in 16 cases. We corrected with cross finger flap technique 14 volar and 1 dorsal fingertip injuries. The operation average time was 83.21 minutes. The cosmetic outcome in this group was 8.28. Mostly dorsal (n=9) and transverse (n=7) injuries were corrected by V-Y advancement flap. The surgery to cover the deficiency took 36.56 minutes. A better cosmetic result was found in this group, the average rate was 9.37. There was a significant correlation between the plane of the traumatism and the cosmetic outcome of the operation. The best outcomes were found in transverse injuries. The quicker hospital presentation showed the better cosmetic outcomes at the 0.01 significance level.

Conclusion: The V-Y advancement flap can be used with a high liability in dorsal and transverse injuries. Cross finger flap is used more often in volar traumatism. We cannot apply an algorithm for the elective approach.

Keywords: V-Y advancement flap, cross finger flap, hand trauma

doi: 10.21614/jpss.11.1.15

Citation: J Pediatr Surg Spec. 2017;11(1):15-19

 

Introduction

The fingertips are always exposed to the external environment and play an important role in feeling delicate sensation. The children discover the surrounding world by touching and feeling everything. This is the reason why fingertip injuries constitute the largest part of hand traumas in children especially in toddlers and young children. The vast majority of injuries are due to traumatic crush-avulsion mechanism. The goal in fingertip amputation reconstruction is to cover the defect, establish maximum tactile gnosis, and protect the joint function and none the less to obtain a satisfactory cosmetic appearance. The plane of the amputation and the condition of the tissue at the injury site help to determine the best repair technique. Transverse and dorsal oblique fingertip amputations may be successfully treated with V-Y advancement flaps [1]. The V-Y technique preserves the normal contours of the dorsal finger and normal sensation [2]. It can be a volar or lateral lesion, a shortening of the bone, the chief competitor with the V-Y technique is the cross-fingerpulp flap in fingertip loss. The cross finger flap can be used primarily to replace an avulsed finger pad or to replace an inadequate skin graft [3]. The aim of this study is to compare the results of these two reconstructive techniques in pediatric population.

Materials and methods

Thirty pediatric patients underwent fingertip reconstruction using the V-Y advancement flap and cross finger flap techniques at the Department of Pediatric Surgery in Târgu Mureş between January 2015 and December 2016.

The used technique was chosen according to the nature of the injury, the size and condition of the defect, the affected fingertip, the plane and the zone of the traumatism. An injury classified as zone I occurred distal to the bony structures of the digit and the distal phalanx was preserved. Most of the nail bed and the integrity of the matrix were intact. Zone II injuries were located distally to the lunula of the nail bed and were commonly implicated by the bony exposure of the distal phalanx. Injuries of zone III involved the nail matrix and result in loss of the entire nail bed; usually they were not candidates for elaborate reconstruction (Fig. 1). According to the plane of the amputation injuries were also classified as dorsal, transverse or volar (Fig. 2). The crossed finger flap (Fig. 3) was designed on the opposite pulp area of the adjacent finger. The V-Y advancement flap (Fig. 4) used a triangular flap which was designed with the base at the edge of the amputation and the full thickness skin flap was advanced over the exposed bone. Regardless of which treatment option was chosen, the wound debridement was the first step for wound healing and infection prevention. Two-point discrimination measures the individual’s ability to perceive two points of stimuli presented simultaneously. Before surgical treatment two-point discrimination test was completed. The operations were performed under general anesthesia. During the follow up visit the two point discrimination test was determined again. The parents compared the cosmetic results with the controlateral fingertip and they rated it on a1-10 scale.

Figure 1. Zonal classification of amputation: Zone I is distal to the phalanx; Zone II distal to the lunula and Zone III proximal to the lunula

11.1 3 1

Figure 2. Injuries classified by the plane of the injury: dorsal oblique, transverse and volar oblique

11.1 3 2

Figure 3. Cross finger flap: The skin was elevated like a page of the book with the base on the controlateral side of the donor finger and it covered the exposed bone.

11.1 3 3

Figure 4. V-Y advancement flap: V shape incision with the apex at the baseline of the DIP joint. The distal edge of the volar skin is advanced and sewn into the dorsal skin. The donor site is closed in Y shape.

11 1 3 4

Results

The mean age of the patients is 7.23 with a minimum of one year and a half and a maximum of seventeen years. Out of the 30 patients 56.66% (n=17) are boys. The most frequent type of injury is due to crush mechanism 46.7% (n=14) followed by sharp injury in 30% (n=9) and avulsion in 23.3% (n=7) of the cases. A volar traumatism plane is observed in 12 patients, dorsal in 11 and transverse in 7. After statistical analyses we observed thatthere is a correlation between the plane of the traumatism and the time until hospitalization. In case of volar damage the time between the traumatism and surgical consult shortens. There is also a significant correlation between the plane of the traumatism and the cosmetic outcome of the operation. The best outcomes are found in transverse injuries. The mean cosmetic result is 8.87 (Fig. 5) however itdepends on variable factors. The quicker hospital presentation shows the better cosmetic outcomes at the 0.01significance level. The size of the wound correlates negatively with the cosmetic outcome (p<0.01). The mean size of the defect is 2.47 square centimeters.The biggest discontinuity is found in avulsion trauma with an average of 2.78 sqcm wound. There is a negative correlation between age and wound size at 0.05 level. The middle finger is injured in 8 traumas; the index along with the ring finger is damaged in 7 (23.35%) patients.

Figure 5. The cosmetic results of the cross finger flap technique show a median of 8.5 with a minimum of 7. The 75th percentile is below 9. The V-Y advancement flap shows better results: only 25% of the patients had lower scores than 9 with a minimum of 8.

11.1 3 5

The time between the injury and surgical consult is 1.43 hours. A statistically significant correlation is found between the age and the time until the hospitalization (p<0.01). Cross finger flap is used in 14 cases and V-Y advancement flap in 16 cases. The chosen technique is cross finger flap in 6 avulsion, 3 crush and 5 sharp injuries.The ring finger is affected the most.We corrected with this technique 14 volar and 1 dorsal fingertip injuries. The mean defect size is 2.51 sqcm. Parents arrive to the hospital after 1.5 hour (range of 30 minutes and 2 hours) after the accident. The patients wait in average 2.53 hours until the surgical treatment from the moment of the injury. The operation average time is 83.21 minutes. Before operation the two-point discrimination test is 5.45 and during the follow up consultsis 4.94. The follow up control takes place averagely 4.71 months after surgery. The cosmetic outcome in this group is 8.28. The best outcome is in crush injuries with an average of 8.33. Seven transverse and 9 dorsal injuries are corrected with V-Y advancement flap. The average defect size is 2.42 square centimeters (sqcm). The most frequent injured fingertip in this group is the middle one. This treatment was chosen in 1 avulsion, 11 crush and 4 sharp cases. Mostly the dorsal (n=9) and transverse (n=7) zone I injuries are corrected by this method. The surgery time to cover the deficiency takes 36.56 minutes. The time between the injury and surgical consult is 1.37 hours;2.28 hours until the operation. Before the operation the two-point discrimination test is 5.79 and during the follow up is 4.83. A better cosmetic result is found in this group, the average rate is 9.37. The best cosmetic result is given in the crush mechanism trauma (9.45).

Discussion

Fingertip injuries are the most common injuries in pediatric hand trauma. The major causes in children are due to traumatic-crush avulsion, such as when the affected digit is caught in the closing door or beneath heavy objects. In older children, we can see hand injures from sport related activities [4]. The fingertip being the end organ for touch, preserving maximal function is the utmost importance. When there is adequate skin and soft tissue available, primary wound closure should be attempted. A small uncovered distal phalangeal bone (< 1sqcm) will allow healing by secondary intention. The dressing is changed by the doctor; but once they confirm that there is no infection parents need to be instructed to change the dressings until healing. This strategy takes time and can be challenging in young patients. A bigger wound where the amputated fragment is not available, the optimal reconstruction has to preserve the finger length, sensation and functioning. The cosmetic result is not the last criteria since the hand is the second most conspicuous part of the body after the face [5].

The V-Y advancement flap and cross finger flap techniques are commonly used reconstructive techniques in pediatric population because they are simple solutions for wound covering and they can be used even in distress, nonverbal child in whom multiple procedures and anesthetics are to be avoided. The recovery is faster and better than in other techniques. However they are not at the top of the reconstructive ladder elaborated by Levin[6], they have good tactile gnosis and cosmetic outcomes. The two point discrimination test shows healing in both of the cases, but in the cross finger flap group the difference was lower. When dorsal skin was used for volar correction the sensory function decreased because of different tissue. To improve sensory recovery, maximal amounts of subcutaneous tissue must be included in the neurovascular island dissection [7].

The V-Y advancement flap has better cosmetic results. The advantage of this method is that it preserves the finger length with tactile gnosis and nail growth.It is a one stage surgery that uses similar colored and textured adjacent tissues. The child can move the injured finger after the operation; physical therapy isn’t usually needed for stiffness. The disadvantage can be the created tension in larger defects.

The other frequently used technique in pediatric population is the cross-finger flap which has acceptable cosmetic results. An advantage of this technique is that the abundant circulation in the skin of the finger offers a good blood supply; a pedicle vascular insufficiency is rare. This flap can easily cover dorsal digital defects if the adjacent finger is sufficiently long. The disadvantage is that there may be some residual scarring and stiffness in the donor finger. It needs skin grafting for donor site and a second surgery for flap separation. Another disadvantage is the uncomfortable positioning in case of terminal digital defects [8]. Children don’t support well to have stuck fingers for 2-3 weeks. Non innervated cross finger pulp flap has the weakness of poor sensory recovery [7]. Despite of the disadvantages this technique is a good option if other local flaps are not available.

Study Limitations

Our study is based on the outcome of 30 fingertip injuries, which may be small to conclude which reconstructive technique has better esthetic results.

Conclusion

Through this review we demonstrated that V-Y advancement flap can be used with a high liability in dorsal and transverse injuries. Cross finger flap is used more often in volar traumatism. We cannot apply an algorithm for the electiveapproach. The surgeon has to choose the reconstruction technique which gives a viable digital tip and if possible preserve the digital length.

 

 

References:

1. Jackson E. The V-Yplasty in the Treatment of fingertip amputations. Am Fam Physician 2001;64(3):455-8.

2. Scott W.Wolfe. Green’s operative hand surgery, vol.I, 6th ed. Philadelphia: Churchill Livingstone, 2011:1662-1709

3. Lemmon JA, Janis JE, Rohrich RJ. Soft tissue injuries of the fingertip: Methods of evaluation and treatment. An algorithmic approach. PlastReconstrSurg 2008; 122:105-17

4. Bae, Donald S. Pediatric hand and upper limb surgery: a practical guide. Philadelphia: Lippincott Williams & Wilkins. 2012.

5. Manske PR. Aesthetic hand surgery. J Hand Surg Am 2002; 27(3): 383.

6. Chao JD, Huang JM, Wiedrich TA. Local hand flaps.J Am SocSurg Hand 2001;1:25-44

7. Lee NH et al. Advantages of innervated cross-finger pulp flap. Arch PlastSurg 2012:637-642

8. Rahul K Patil, SachinChavre. Distally based cross-finger flaps for amputation stumps in avulsion amputations. Indian J PlastSurg 2012; 45:504-511

Ahmed H. Al-Salem, Hilal Matta

 

Abstract

Aim: Congenital paraesophageal (CPH) is rare in infants and children. It can unusually be large with herniation of most of the stomach which can lead to intrathoracic gastric volvulus. This report describes our experience with infants and children with large CPH.

Patients and Methods: Twelve infants and children with large CPH, three of them presented with intrathoracic gastric volvulus were evaluated. Their medical records were reviewed for age at diagnosis, sex, clinical features, diagnosis and management. Results: Between 1992 and 2012, twelve infants and children with large CPH were treated. There were 7 males and 5 females. Their age at diagnosis ranged from 2 days to 2.5 years (mean 16.3 months). Two presented acutely, immediately after birth with respiratory distress secondary to a large CPH. One of them had intrathoracic gastric volvulus. Four presented with recurrent chest infection and failure to thrive while the remaining three had repeated attacks of vomiting. Another patient was a case of repaired esophageal atresia and tracheoesophageal fistula. He presented at the age of 2.5 years with recurrent attacks of cough and vomiting for one-year duration and was found to have a large paraesophageal hernia. Two patients were sisters and both of them presented with recurrent chest infection and failure to thrive. At the time of presentation, both had large CPH with intrathoracic gastric volvulus.

Conclusions: CPH is rare in the pediatric age group and sometimes it is unusually large with herniation of most of the stomach which may result in intrathoracic gastric volvulus. Awareness of this and early diagnosis and treatment are important to avoid subsequent morbidity and mortality.

Keywords: paraesophageal hernia, infants and children, familial, fundoplication

doi: 10.21614/jpss.11.1.6

Citation: J Pediatr Surg Spec. 2017;11(1):6-10

 

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Soumyodhriti Ghosh, Ramendra Shukla, Vikram Singh Mujalde, Mushahid Ali , Sunil Mehra , Ayush Kumar , Arun Kumar Gupta, Vinita Chaturvedi

Department of Pediatric Surgery, SMS Medical College Hospital, Jaipur, India


Correspondence:

Soumyodhriti Ghosh

Department of Pediatric Surgery

JK Lon Hospital, SMS Medical College

Jaipur 302004, India

Phone: +91 9549339985

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

 

Abstract

Purpose: Pediatric patients represent nearly 10 percent of all trauma admissions in major trauma centers. The study attempts to study the different patterns of demography in patients suffering from trauma to the abdomen in a tertiary care hospital in a developing nation.

Methods: The study was done in a tertiary care center over a duration of one year. The study was retrospective based on hospital records of the patients. The demographic parameters were studied and compared to available literature.

Results: A total of 288 cases of blunt trauma to the abdomen were recorded in one year. The demographic trends with respect to the etiology, organ injured, management protocol and mortalities were comparable to standard literature.

Conclusion: Pediatric trauma is a neglected topic in developing nations due to absence of infrastructure and trained personnel. The high incidence illustrates the need to have proper training programs and education in this stream.

Keywords: pediatric, blunt trauma, abdomen, conservative, surgical exploration.

doi: 10.21614/jpss.11.1.11

citation: J Pediatr Surg Spec. 2017;11(1):11-14

 

Introduction

Blunt injuries are a common referral to any pediatric surgical emergency. Worldwide pediatric trauma accounts for 1.5 million injuries. Almost 90 percent of all trauma impacts are blunt in nature [1,2]. The head and extremity trauma account for majorities of all blunt injuries [1]. Abdominal blunt traumais a special concern due to their widespread and variable presentation and differing outcomes. The pediatric population are way different from their adult counterparts with regards to the body impact suffered from a similar trauma [3-5]. Children have poorly developed musculoskeletal system and the abdominal organs are in close proximity to each other. The abdominal wall is thin with less fat contents. Further, the small stature results in increased force per body surface area creating more damage to internal organs [5,6]. Evaluation and management protocols of blunt abdominal injuries in children have undergone drastic changes. Newer imaging modalities with better results obtained with conservative treatment have shifted the attention towards nonoperative modalities. The morbidities and mortalities should therefore be compared with caution. However, the trends have been partially limited in developing nations with constraints of infrastructure and proper trained health professional. Our study attempts to understand the trends of patient profile and management outlines in a tertiary care center of North West India.

Materials and Methods

The study was conducted in the Department of Pediatric Surgery, SMS Medical College Hospital, Jaipur. The study was retrospective, based on medical records of patients over a period of one year, from May 2015 to April 2016. All pediatric patients who were admitted with a provisional diagnosis of blunt trauma to the abdomen were included in the study. The maximum age limit was 18 years. All patients underwent evaluation and management by the same surgical team. The exclusion criteria included patients with penetrating injury to abdomen, patients with added head injuries needing neurosurgical care, patients with added extremity injury needing orthopedic care. The demography of these patients were studied with respect to different parameters as outlined next. The values obtained were analyzed and compared to available studies.

Results

The pediatric surgery emergency of SMS Medical college, Jaipur caters to all types of neonatal and pediatric emergency as per tertiary care protocols. Trauma in children accounts for a significant proportion of admitted children (10%). The total number of patients admitted in a span of one year was 288. The commonest age group was 3 to 6 years having 30% of all patients. More than half of all patients were less than 10 years of age (Table 1). Most of the children were referred from peripheral centers and district hospitals. This was expected as the department houses the only trauma center in the region (Table 2). The cause of injuries in children is different from those in adults. Direct injuries with impact over the abdomen are less common. Fall from height were noted in 45% of children, with motor vehicle accidents following it (Table 3). The patients underwent similar investigation protocols. Complete blood investigations were followed by imaging studies. X-ray, sonography and CT scan studies were performed as required. Based on the findings, single organ injury was found in more than half of all patients. Multi organ injuries are less common, in contrast to adult populations (Table 4). Liver was much more commonly injured than spleen and bowel (Table 5). This was the reason of better roles of conservative management in these patients. Nearly 61% of children were managed by non operative measures (Table 6). It is important that only 11 patients died, this accounts for 4% of total (Table 7). Patients with multi organ damage were the usual victims.

Table 1. Age distribution of patients admitted.

Age in years

No. of patients

Percentage

0-2

43

15%

3-6

86

30%

7-10

63

22%

11-14

58

20%

15-18

38

13%


Table 2. Distribution of patients referred to tertiary center.

Referral

Patients

percentage

Direct

46

16%

Indirect

242

84%

 

Table 3. Distribution of patients according to etiology of injury.

Etiology

Patients

Percentage

Fall from height

130

45%

Motor vehicle accident

107

37%

Direct trauma

51

18%

 

Table 4. Distribution of organ injuries based on number of internal organs injured.

Type of trauma

Patients

Percentage

Single organ

167

58%

Two   organs

72

25%

Multi organs

49

17%


Table 5. Distribution of the internal organs commonly injured.

Organs involved

Patients

Percentage

Liver

143

50%

Spleen

58

20%

Bowel

96

58%

Pancreas

43

15%


Table 6. Management protocol

Management

Patients

Percentage

Conservative

176

61%

Surgical

112

39%


Table 7.
Etiology of patients expired (MOD-multi organ damage)

Underlying cause

Expired Patients

N=11

Liver trauma

02

Splenic trauma

01

MOD

08


Discussion

A child is not a miniature adult. The principle applies to traumatology as well. The lesser body surface of area of children creates a higher impact for the same amount of force. Added to this, is the thin abdominal wall with poorly developed fat cushions [7-9]. Pediatric traumtology is a concern that should be managed with priority.Worldwide, 65% of all injury deaths results from unintentional injuries [10]. Approximately, 20000 pediatric patients die every year as a result of injuries [11, 12].

In developing nations, most injuries are observed in age groups of 13 to 18 years, particularly due to increased violence. This result is in contrast to our study where most trauma patients were less than 10 years of age. Motor vehicle accidents are the leading etiological factors causing abdominal blunt injuries [13,14]. However, this status does not hold true in most developing nations. In our study, children falling from height was the most common cause followed by motor vehicle accidents. The referral system has always been a concern in developing nations. The less number of trauma centers with poor inter center referrals result in delayed management in most cases. A large number of studies in children have quoted bowel injuries to be the most common injured internal organ followed by liver (52%) [15-17]. This might be related to the corresponding etiology in those cases. Rapid deceleration in car accidents cause compression injuries to the bowel [18]. In our study, we had maximum cases of liver contusions and laceration injuries. Spleen was less commonly injured in contrast to adults [19]. A recent study by Holmes et al. sought to follow the causes of failure of conservative management in trauma [1,2,20]. Around 85 to 90 % of children having liver damage could be managed by non-operative means [21]. The cause of failure included hemorrhage, shock, peritonitis among others [21,22]. Multi organ damage also necessitated surgical exploration in most cases.

Our study quotes resolution in 61% children with conservative measures alone. This might be related to the increased liver injuries in developing countries (50%). Patients treated nonoperatively had extensive monitoring with serial imaging and vitals statistics records. Standard conservative protocols of nasogastric suction, intravenous antibiotics and urine output monitoring was followed. Surgical repair was individualized with correction of etiology in most cases. One additional factor that we studied was the total mortality in one year. It is interesting that only 11 patients out of 288 died in this duration. Patients with severe multi organ damage with poor vitals were the usual victims.

Conclusion

Pediatric trauma derives special concern, not only for its subtle presentations but also for the specialized skills that it warrants in healthcare providers. Proper judgement, clinical evaluation and good imaging studies go a long way in proper diagnosis and prevention of unwanted surgeries.

 

References:

1. Holmes JF, Gladman A, Chang CH. Performance of abdominal ultrasonography in pediatric blunt trauma patients: a meta-analysis. J PediatrSurg 2007;42(9):1588-94.

2. Holmes JF, Sokolove PE, Brant WE, Palchak MJ, Vance CW, Owings JT, Kuppermann N. Identification of children with intraabdominal injuries after blunt trauma. Ann Emerg Med 2002;39(5): 500-9.

3. Wegner S, Colletti JE, Van Wie D. Pediatric blunt abdominal trauma. PediatrClin North Am 2006 ;53(2):243-56.

4. Sharpe RP, Pryor JP, Gandhi RR, Stafford PW, Nance ML. Abdominal compartment syndrome in the pediatric blunt trauma patient treated with paracentesis: report of two cases. J Trauma 2002;53(2):380-2.

5. Mirvis SE, Gens DR, Shanmuganathan KA. Rupture of the bowel after blunt abdominal trauma: diagnosis with CT. AJR Am J Roentgenol 1992;159(6):1217-21.

6. Coley BD, Mutabagani KH, Martin LC, Zumberge N, Cooney DR, Caniano DA, Besner GE, Groner JI, Shiels WE. Focused abdominal sonography for trauma (FAST) in children with blunt abdominal trauma. J Trauma 2000;48(5):902-6.

7. Fox JC, Boysen M, Gharahbaghian L, Cusick S, Ahmed SS, Anderson CL, Lekawa M, Langdorf MI. Test characteristics of focused assessment of sonography for trauma for clinically significant abdominal free fluid in pediatric blunt abdominal trauma. AcadEmerg Med. 2011;18(5): 477-82.

8. Gross M, Lynch F, Canty T, Peterson B, Spear R. Management of pediatric liver injuries: a 13-year experience at a pediatric trauma center. J PediatrSurg 1999;34(5):811-7.

9. Canty TG, Brown C. Injuries of the gastrointestinal tract from blunt trauma in children: a 12-year experience at a designated pediatric trauma center. J Trauma 1999;46(2):234-40.

10. Tataria M, Nance ML, Holmes IV JH, Miller III CC, Mattix KD, Brown RL, Mooney DP, Scherer III LR, Groner JI, Scaife ER, Spain DA. Pediatric blunt abdominal injury: age is irrelevant and delayed operation is not detrimental. J Trauma 2007;63(3):608-14.

11. Holmes JF, Brant WE, Bond WF, Sokolove PE, Kuppermann N. Emergency department ultrasonography in the evaluation of hypotensive and normotensive children with blunt abdominal trauma. J PediatrSurg2001;36(7):968-73.

12. Nance ML, Keller MS, Stafford PW. Predicting hollow visceral injury in the pediatric blunt trauma patient with solid visceral injury. J Pediatric Surg2000;35(9):1300-3.

13. Retzlaff T, Hirsch W, Till H, Rolle U. Is sonography reliable for the diagnosis of pediatric blunt abdominal trauma? J Pediatric Surg. 2010;45(5):912-5.

14. Milas ZL, Dodson TF, Ricketts RR. Pediatric blunt trauma resulting in major arterial injuries. Am Surg 2004;70(5):443.

15. Cook SH, Fielding JR, Phillips JD. Repeat abdominal computed tomography scans after pediatric blunt abdominal trauma: missed injuries, extra costs, and unnecessary radiation exposure. J Pediatric Surg 2010;45(10):2019-24.

16. Poletti PA, Mirvis SE, Shanmuganathan K, Takada T et al. Blunt abdominal trauma patients: can organ injury be excluded without performing computed tomography? J Trauma 2004;57(5):1072-81.

17. Feliz A, Shultz B, McKenna C, Gaines BA. Diagnostic and therapeutic laparoscopy in pediatric abdominal trauma. J Pediatric Surg 2006; 41(1):72-7.

18. Brown SL, Haas C, Dinchman KH, Elder JS, Spirnak JP. Radiologic evaluation of pediatric blunt renal trauma in patients with microscopic hematuria. World J Surg 2001;25(12):1557-60.

19. Christiano JG, Tummers M, Kennedy A. Clinical significance of isolated intraperitoneal fluid on computed tomography in pediatric blunt abdominal trauma. J Pediatric Surg 2009;44(6):1242-8.

20. Leone Jr RJ, Hammond JS. Nonoperative management of pediatric blunt hepatic trauma. AmSurg 2001;67(2):138.

21. Ameh EA, Chirdan LB, Nmadu PT. Blunt abdominal trauma in children: epidemiology, management, and management problems in a developing country. PediatrSurgInt 2000;16(7):505-9.

22. Richards JR, McGahan JP, Pali MJ, Bohnen PA. Sonographic detection of blunt hepatic trauma: hemoperitoneum and parenchymal patterns of injury. J Trauma 1999;47(6):1092.

Table 7- Etiology of patients expired (MOD-multi organ damage)

Horea Gozar MD PhD

Assistant Professor

Chief of Pediatric Surgery Department

Emergency Clinical Hospital Targu-Mures, Romania

 

Sad news disseminated quickly last October through the medical community: Professor Jay Grosfeld has died at age 81. The news had a profound impact upon the pediatric surgery community literally all over the world, as he was considered a giant and a founding father in the field.

Under these circumstances, many journals around the world considered writing an editorial about the work and the life of Dr. Grosfeld. For me was a moral duty to write one. I knew some things about Jay Grosfeld, but I started to search more information about him from papers or interviews and I have to confess that I remained charmed by his life, as well as his personality. It was not a barren work writing this editorial, but a very captivating one.

Every exposure about a great personality presumes some biographical data. Professor Grosfeld was born in New York City in 1935. He graduated from the New York School of Medicine in 1961, and then he started a residency in Surgery in the same city. He continued with a residency in Pediatric Surgery at Ohio State University College of Medicine, Columbus, OH, which he graduated in 1970. During that time, he was a Fellow in the American Cancer Society, Senior Clinical Traineeship. He began his career in Pediatric Surgery and between 1970 and 1972, Jay Grosfeld was an Assistant Professor of Surgery and Pediatrics at New York University. In 1972 he left New York City and followed his medical path in a different city, Indianapolis, IN. Over time, he became Professor and Director of Pediatric Surgery at Indiana University School of Medicine and Surgeon-in-Chief at James Whitcomb Riley Hospital for Children. Dr. Grosfeld was also Professor and Chairman in the Department of Surgery, at Indiana University School of Medicine.

He was guided to medicine by his mother and one of his uncles. Medicine was not a tradition in his family, but he wanted to open new doors. Family was always a strong support for him, a landmark for his formation, a loving, supportive and educational place. A good education sometimes presumes rigid rules, but is certainly a key for future successes. I imagine that Prof. Grosfeld was such a personality, that whatever he was to do and anywhere he was to go, he would have become very successful. Many areas of medicine fascinated him, but he endeared Surgery and Pediatrics, hence the decision to become a Pediatric Surgeon.

His long journey started at Columbus University, Ohio. He worked in the team led by Dr. Clatworthy, who had a profound contribution on his development. Dr. Clatworthy was the kind of person who could have a profound impact on many aspects of one’s personal and professional development: critical thinking, clinical skills, research conducting and scientific paper writting. The seed was launched and helped to produce a great harvest. Grosfeld had to leave Ohio and a temporary medical station was New York. His professor there was Frank Spencer, another giant in Pediatric Surgery, who also marked his personality. Spencer continued to nurture Grosfeld`s personality and his medical development. It was not for a long time though, as Grosfeld had to leave New York. As his family expanded (by that time he already had five children), he took the decision to move to a different place. That location was going to be the place in which he would become well-known all over the world: Indianapolis.

Riley Children`s Hospital from Indianapolis was an ordinary hospital before Dr. Grosfeld’s coming, where a small number of operations were performed on a regular basis. He exponentially increased the number of operations, as he performed new and pioneering operations. A great turning point was the reconsideration of the clinic work. The careful monitoring of patients day by day and round and round was one of his great achievements. He could work with abnegation many hours per day, with very little sleep. As a leader, one has to be an example for everybody in the team. One has to formulate questions and find the best solutions, particularly for those clinical cases that are not going well. Prof. Grosfeld put the basis of collaborative work with other surgeons, pediatricians and administrative staff, in order to dispose a wider clinical view and more resources for his patients. He was also the catalyzer for the development of different surgical subspecialties. Numerous residents and junior surgeons trained under his leadership, of which many eventually became great surgeons around the world. He traveled the world to disseminate his new concepts in Pediatric Surgery.

Jay Grosfeld dedicated significant time to research work. In Indianapolis, he put the basis of a new laboratory, virtually from scratch. He obtained grant funding for equipment and for an animal facility, which resulted in numerous animal studies. He tackled the most difficult unresolved medical problems, such as certain types of pediatric malignancies (hepatoblastoma, neuroblastoma), congenital malformations, short bowel syndrome, necrotizing enterocolitis, obesity, tissue ischemia and transplantation. He was especially dedicated to newborn pathology, which is the most difficult, as babies cannot express themselves. However, he had remarkable results, keeping in mind that those were the days where the usual quote amongst the neonatologists was “don`t let the surgeons touch the baby”. Dr. Grosfelt brought new concepts in trauma: quick interventions in some cases and a non-interventional “wait-and-watch” approach in others. I believe there is no important chapter of pediatric surgery, where Prof. Grosfeld had no contributions. He published numerous papers to disseminate his research. His name remained connected to the most respected journal in the field, “Journal of Pediatric Surgery”, where he functioned as editor-in-chief until his death.

Jay Grosfeld enjoyed being a teacher, as well. He definitely wanted to share his knowledge with his students, residents, fellows and junior faculty physicians. He always discussed medical problems, whether in large or small groups, in the hallways or in the operating rooms, standing by the children. He educated them how to formulate questions, how to solve them, how to “to read the baby” and simply how to be a good surgeon, the techniques and tactics in pediatric surgery.He brought the doctor closer to the patient and the family. Prof. Grosfeld had a tremendous success, being a traditional doctor and teacher, based on clinical experience, not only on digital support or high-tech equipment. He wrote many textbooks and chapters that now are regarded as “the bible” in pediatric surgery.

Prof. Grosfeld was Chairman of the Surgical Section of the American Academy of Pediatrics, President of the American Paediatric Surgical Association, Chairman of the American Board of Surgery, President of World Federation of Association of Paediatric Surgeons (WOPAS) and First vice- President of the American College of Surgeons. He received the William E Ladd Medal from American Academy of Paediatrics, the Sir Denis Browne Gold Medal, from British Association of Paediatric Surgeons and Fritz Rhebein Medal from European Association of Paediatric Surgeons. Prof. Grosfeld was Honorary Fellow of many surgical colleges around the world. His statue now stands at the entrance to the Riley Hospital for Children in Indianapolis, IN, a modest memento for a life dedicated to the well-being of children.

Unfortunately, I have never met Prof. Grosfeld in person, although now, after reading so much about him, I feel like I have... However, from the discussions I had with people who had the pleasure of knowing him, I understand that he was known as a friendly and kind person. He had a large family, including a loving wife of a lifetime, five children and seventeen grandchildren. He was very proud of his family and this was also an important part of his legacy. I know of at least two of his grandsons who are preparing for medical school, with the intention of becoming pediatric surgeons: life goes on!

Jay Grosfeld remains a giant in medicine, especially in the pediatric surgical community. Whether as a medical doctor, researcher or a teacher, he was a role model. He saved and improved the lives of many children in America, as well as around the world.He will live through the saved children, his disciples and his wonderful family. I conclude with the Grosfeld`s father phrase that traced his entire career: “you go to work every day and do the best job you can do and things will work out for you”.

Apoorva Kulkarni, Abhaya Gupta, Vishesh Dikshit, Paras Kothari, Geeta Kekre

 

Abstract

Round ligament varicosities are a rare disorder seen in gravid females and is often mistaken for complicated inguinal hernia. We present a very rare case of a 2 years old female child who presented with intractable pain in the right inguinal region, found to be a round ligament varicosity. Relief was achieved only after excision of the mass. A diagnostic laparoscopy was also done to confirm the presence of normal ovaries.

Keywords: varicosity, inguinal, child, round ligament

doi: 10.21614/jpss.11.1.37

Citation: J Pediatr Surg Spec. 2017;11(1):37-39

 

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