Congenital Rectal Stenosis: A Simple Approach to Management
From the Division of Pediatric Surgery, Department of Surgery, Qatif Central Hospital, Qatif.
AH Al-Salem, A Al-Faraj, V Talwalker, Congenital Rectal Stenosis: A Simple Approach to Management. 1994; 14(1): 56-57
Rectal atresia is a rare type of high anorectal malformation and of the variants of rectal atresia, congenital rectal stenosis is the least common type.1 Various surgical procedures2-5 have been described for the management of rectal atresia, but due to its rarity, there is no standard tested approach for its management. We present a case of congenital rectal stenosis in a 3-1/2-year-old female and describe a simple technique for its management.
Case
Case Report
A 3-1/2-year-old female was referred to Qatif Central Hospital because of soiling her pants with liquid stools since birth, and just prior to her presentation, she started to complain of abdominal pain and distention. On examination, she was found to have mild abdominal distention with palpable fecal masses in the abdomen. A hard fecaloma was palpated in the pelvis on rectal examination, while the rectum was empty. After repeated rectal washouts, it was noted that the fecaloma was in the proximal part of the rectum immediately above a diaphragm with a central hole about 3 cm from the dentate line. This was confirmed by a barium enema (Figures 1 and 2).
Surgical Technique
The patient underwent a laparotomy through a transverse suprapubic incision. The upper third of the rectum and sigmoid colon were dilated and hypertrophied, while the lower part of the rectum was normal in caliber and the junction between the two was clearly seen. There was no other pathology. The sigmoid colon was opened longitudinally to visualize the septum, which had a central hole. The #28 EEA gun (without anvil) was introduced transanally. The shaft was guided into the opening by a finger into the proximal rectum, through the opening in the sigmoid colon. The anvil was screwed on the shaft through the colotomy opening until it was flush at the site of the central hole. The gun was then fired. The head was released and the entire instrument was recovered from the anus (Figures 3a, b, and c) (Figure 3. Operative resection of the rectal diaphragm using the EEA autosuture: a) the EEA gun without anvil is introduced transanally and the shaft guided into the opening in the diaphragm, guided by a finger into the proximal rectum and sigmoid colon. b) the anvil was screwed on the shaft through the colotomy opening until it was flush at the site of the central hole. c) the gun was fired, released and then recovered from the anus. The opening in the sigmoid colon closed.). The gun contained a complete disc of tissue with a central opening. The integrity of the anastomosis was confirmed and the opening in the sigmoid colon was closed longitudinally in two layers.
The septum histologically was lined by stratified squamous non-keratinizing epithelium on the anal aspect 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.
Postoperatively, the patient was passing stools easily without soiling but from time to time she required glycerin suppositories because of constipation. Rectal examination five months postoperatively revealed an adequate opening at the anastomosis site.
Discussion
Rectal atresia is a rare type of high anorectal malformation and its incidence worldwide is low.1 Of the variants of rectal atresia, congenital rectal stenosis is the least common type. Dorairajan, in a series of 147 cases of rectal atresia, found only three cases of rectal stenosis.1
Congenital rectal stenosis has been classified either as a separate entity in the rare variants of high anorectal malformations6 or, more accurately, as Type IV in the classification of rectal atresia proposed by Dorairajan.1 Even though it has been classified as a variant of high anorectal anomalies, unlike them, the anal canal as well as the pelvic floor and surrounding sphincters is normally formed. Pathologically, congenital rectal stenosis actually resembles a duodenal diaphragm with a central hole, which is equivalent to Type I of colonic atresia according to the classification described by Sutton.7 Congenital rectal stenosis due to extrinsic causes, e.g., presacral teratoma and anterior sacral meningocele, has been reported previously.8
The optimal goal in the management of cases of high anorectal agenesis is acquisition of continence. Various surgical techniques, some of which are major, have been described for the management of rectal atresia,2-5 but because of its rarity, there is not a well proven and tested approach for its management. Congenital rectal stenosis, although a variant of rectal atresia, can be approached differently in its management. Cole9 reported a 10-year-old male with rectal stenosis due to a congenital diaphragm placed 8 cm from the anal margin. This patient was treated initially by a loop colostomy and ultimately required resection and end-to-end anastomosis. The EEA staplers have been used extensively in gastrointestinal surgery including stricture resection,10,11 but their use in the pediatric age group is limited. Our patient was operated upon successfully using a simple technique without interfering with the anatomy and function of the sphincters and obviating a long-term colostomy.
References
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6. Stephens FD. Widespread anomalies, rarities and super rarities of the anorectum and cloaca. In: Anorectal malformations in children: Update 1989, Chap 25. Stephens FD, Smith ED, eds. Alan R. Liss, Inc., New York, NY, USA 1988;581-5.
7. Sutton JB, cited by Philippart AI: In: Atresia, stenosis and other obstructions of the colon. Welch KJ, Randolph JG, Ravitch MM, et al. eds, Pediatr Surg Vol 2. Chicago IL, USA. Year Book 1986;984.
8. Malangori MA, Grosfeld JL, Ballantine TVN, Kleiman M. Congenital rectal stenosis: a sign of presacral pathologic condition. Pediatr 1987;62:584-7.
9. Cole GJ. Congenital diaphragm in the upper rectum. Br J Surg 1963;50:523-4.
10. Ravich MM, Steichen FM. Technique of staple suturing in the gastrointestinal tract. Ann Surg 1972;175:815-37.
11. Goligher JC. Recent trends in the practice of sphincter-saving excision for rectal cancer. Ann R Coll Surg 1979;61:169-76.
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