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Congenital Rectal Atresia: Which Surgical Approach?

Ahmed H. Al-Salem

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

 

Correspondence:

Ahmed Al-Salem

P.O. Box 61015

Qatif 31911, Saudi Arabia

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

Mobile: 966505818009

 

Abstract

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

Keywords: congenital rectal atresia, treatment

 

Introduction

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

Case Series

Case 1

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

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

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Case 2

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

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

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

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

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Case 3

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

Case 4

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

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

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

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

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

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Discussion

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

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

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

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

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

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

 

 

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