CHAPTER 20 Hirschsprung Disease
Swenson Pull-Through Procedure
Step 1: Surgical Anatomy
♦ Hirschsprung disease is the clinical manifestation of the absence of ganglion cells of the involved intestine. Ganglion cells are absent from the submucosal (Meissner) plexus as well as the intramuscular (Auerbach) plexus. In addition, there is an increase in the longitudinal nerve fibers, which stain intensely for acetylcholinesterase.
♦ The incidence of Hirschsprung disease ranges from 1 in 4000 to 1 in 7000 live births, with a male-to-female ratio of 4:1.
♦ The sporadic occurrence of Hirschsprung disease accounts for 80% to 90% of cases. There has been an explosion of genetic information regarding the complex inherited pattern of Hirschsprung disease and its association with neurocristopathies.
♦ In 80% of affected patients, the aganglionic segment of colon extends from the anus to the sigmoid colon. A longer segment of colon is involved in 10% to 15% of patients. Total colon Hirschsprung disease occurs in between 3% and 10% of patients, with extension into the small bowel in less than 3% of patients.
♦ Trisomy 21, trisomy 18, Ondine curse, and a variety of other cristopathologic disorders are associated with Hirschsprung disease.
♦ The diagnosis is most frequently made in the newborn period. The newborn has signs of bowel obstruction, including failure to pass meconium in the first 48 hours of life, abdominal distention, and vomiting. In infancy and childhood, symptoms of chronic constipation with or without episodes of explosive diarrhea can be present.
Step 2: Preoperative Considerations
Neonates
♦ Physical examination reveals a full-term infant with a distended abdomen. Examination of the perineum reveals an anus in the correct position, and rectal examination reveals an appropriate anal and rectal diameter. Withdrawal of the digit often results in the production of explosive liquid stool.
♦ Plain abdominal film reveals a distal bowel obstruction with dilated large bowel (Fig. 20-1). Barium enema reveals a narrow distal segment with dilated proximal bowel (Fig. 20-2). A postevacuation film reveals retained contrast material (Fig. 20-3).
Infants and Children
♦ Physical examination reveals a distended abdomen, normally placed anus, a rectal examination of normal caliber, a collapsed rectum, and production of explosive stool with removal of the digit. Barium enema reveals a collapsed distal colon and a dilated proximal colon. Postevacuation films reveal retained contrast material.
Management
♦ Most infants can be treated with colonic irrigations once or twice per day before scheduling a definitive procedure. Timing for a pull-through procedure is surgeon dependent, with some performing the procedure in the first several weeks of life and others waiting until infants are 2 to 3 months of age and weigh at least 10 pounds.
♦ If colonic irrigations do not sufficiently decompress the bowel and the infant is unable to tolerate feedings and gain weight, consideration should be made for the placement of a temporary colostomy.
♦ Infants with long-segment Hirschsprung disease or total colonic Hirschsprung disease are less likely to thrive with a colonic irrigation management scheme and will need a temporary colostomy placed in the colon or small bowel, where normal ganglion cells are present. A Kimura patch should be considered in infants with total colon Hirschsprung disease as a two-step procedure preceding pull-through.
Step 3: Operative Steps
♦ A preoperative admission is planned for a mechanical bowel preparation consisting of colonic irrigations every 4 hours and installation of a sufficient quantity of Go-Litely by oral or nasogastric route. A central venous line is placed for intravenous hydration, blood sampling, and postoperative total parenteral nutrition.
♦ The patient is placed on the operating table in the supine position, and the colon is irrigated with normal saline and dilute Betadine. Parenteral antibiotics are administered. The infant is placed at the end of the table in the frog leg position; an older child is placed in a lithotomy position. The abdomen and the perineum are prepared and a Foley catheter placed.
♦ A transanal Swenson pull-through is appropriate for most infants with rectosigmoid Hirschsprung disease. Minimally invasive techniques can be used alone or in combination with the transanal approach in infants or children with more proximal colonic involvement.
Transanal Operative Technique
♦ Four stay sutures are placed at the mucocutaneous junction and epinephrine injected at the dentate line (Fig. 20-4). A circumferential incision is then made through the full thickness of the bowel wall just proximal to the dentate line. Stay sutures are placed through the full thickness of the bowel wall circumferentially, and bipolar cautery is used to dissect proximally.
♦ Traction is placed on the bowel with the stay sutures, and the dissection is continued using the bipolar cautery to ligate and divide the feeding vessels staying on the bowel wall (Fig. 20-5). The transition zone can be visualized as the bowel begins to increase in size and thickness.
♦ Full-thickness biopsy of the bowel wall at this level will identify the transition zone with the presence of a few ganglion cells and hypertrophied nerves.
♦ Dissection is continued proximally until normal ganglion cells are identified. Full-thickness biopsies are taken in the anterior and posterior aspects of the bowel, with different-colored sutures placed on the anterior surface and the posterior surface to maintain orientation.
♦ At the point where normal ganglion cells are identified anteriorly and posteriorly and when there is no evidence of hypertrophied nerves, three fourths of the circumference of the bowel is divided (Fig. 20-6). Sutures are placed through the muscularis in four quadrants. A full-thickness anastomosis is then created with absorbable sutures (Fig. 20-7). The remainder of the bowel is divided and removed and the anastomosis completed (Fig. 20-8).
♦ A laparoscope can be introduced through the umbilicus to evaluate the orientation of the pulled-through bowel. In patients in whom the involved bowel extends more proximally than the sigmoid colon, minimally invasive techniques can be used to mobilize the colon and ensure that the pulled-through intestine has an adequate blood supply.
Operative Technique: Minimally Invasive Assisted
♦ After a pneumoperitoneum is created, two 5-mm and one 12-mm ports are placed. The suspected transition zone is identified and biopsies are taken to identify the presence of normal ganglion cells proximal to the transition zone (Fig. 20-9). Once this is established, the bowel is divided with the Endo-GIA stapling device.
♦ The pelvic peritoneum is incised and the mobilization of the rectum is accomplished by cauterizing the vessels with the harmonic scalpel on the bowel wall. This dissection is carried distally to 1 cm from the dentate line.
♦ The proximal bowel is mobilized to provide enough length to reach the anus. If necessary, mesenteric vessels are clipped at the base of the mesentery, preserving the arcade of vessels to the bowel to be pulled-through.
♦ The rectum is everted at the anus, and an incision is made 1 cm proximal to the dentate line over three fourths of the circumference.
♦ A clamp is placed through this opening, and the normal proximal colon is pulled through the incision under direct vision.
Step 4: Postoperative Care
♦ Nasogastric drainage is instituted intraoperatively and continues until there is return of bowel function. The diet is then advanced as tolerated.
♦ A Foley catheter is used for intraoperative and postoperative monitoring of urine output and is removed on postoperative day 3.
Follow-up Care
♦ The patient is seen 2 weeks following discharge, and a thorough physical examination is performed. No formal rectal examination is performed at this time. A gentle sizing of the rectal opening with small Hegar dilators occurs at the 4-week visit.
♦ Any evidence of obstruction from postoperative edema is treated with gentle irrigations at home, with an examination under anesthesia in the operating room when indicated.
♦ Postoperative stricture formation is identified at the postoperative visits and is treated with examination under anesthesia in the operating room. Late signs of dysmotility or relative obstruction are treated with examination under anesthesia and gentle dilation of the internal sphincter. Postoperative irrigations may be necessary in some infants, however, and if the need for irrigations or repeat dilation ensues, consideration should be made for re-evaluation of the extent of resection and possible repeat full thickness biopsy.
Step 5: Pearls and Pitfalls
♦ At the time of pull-through procedure, biopsies of the anterior and posterior aspect of the involved bowel will help avoid the complication of retained aganglionic bowel.
♦ Meticulous dissection and bipolar cautery use on the bowel wall will prevent injury to surrounding nerves.
♦ The need for increased awareness of possible enterocolitis, which is treated with immediate irrigation and general stretching of the internal sphincter under anesthesia, decreases as the child grows. Incidents of enterocolitis and the need for intervention usually decrease by the 3rd or 4th year following pull-through procedure.
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