Rectal myotomy with colectomy: A new radical operation for Hirschsprung's disease

doi:10.1016/0022-3468(71)90665-8

Journal of Pediatric Surgery

Volume 6, Issue 1, February 1971, Pages 36-41

https://doi.org/10.1016/0022-3468(71)90665-8 Get rights and content

Abstract

A new radical operation for Hirschsprung's disease, i.e., rectal myotomy with colectomy, the aim of which is to minimize pelvic dissection and to preserve the rectal wall as much as possible, was performed in 11 cases of Hirschsprung's disease. It can be used on young infants, and rectal sensation is preserved. The result was satisfactory in all but one child.

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Cited by (21)

Transanal rectal mucosectomy and partial internal anal sphincterectomy for Hirschsprung's disease
2014, Journal of Pediatric Surgery
Citation Excerpt :
No patient developed incontinence postoperatively. In 1971, Kasai et al [3] introduced the internal anal sphincterectomy and removal of the muscular layer in the posterior rectal wall for the treatment of HD, which had a good defecation results postoperatively. However, in this procedure, only the muscular layer of the posterior rectal wall was incised caudally as far as the internal anal sphincter, leaving the mucosal layer intact, and the friable mucosa of the myotomized rectum was then anastomosed to the proximal colon.
Hirschsprung-associated enterocolitis (HAEC) is a serious complication of Hirschsprung’s disease (HD), with generalized sepsis and high mortality rate. Although the surgical correction of HD is mostly successful, challenges remain in the management of children with repeated episodes of enterocolitis. The authors describe a novel modification of transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS) for HD.
One hundred twenty-seven HD children aged from 8 days to 16 years who successfully underwent TRM-PIAS were reviewed. TRM-PIAS was carried out circumferentially along the anorectal line. Anterior dissection was conducted between the rectal submuscosal layer and the rectal muscular sleeve. The posterior dissection was performed along the plane between internal and external anal sphincters. Normal colon was pulled through and anastomosed to anal mucosa. Aganglionic segment, rectal mucosa, part of internal anal sphincter and posterior rectal muscular cuff were removed. Twenty-five age-matched children without defecation dysfunction were used as the control group in the study of anal resting pressure.
Patients were followed up for 6–12 years (median: 8.2 years). The median age at last follow-up was 12.2 years (7.2–20.1 years). The incidence of enterocolitis decreased from 33.9% (43/127) preoperatively to 1.6% (2/127) postoperatively (P < 0.01). The incidence of constipation decreased from 100% (127/127) preoperatively to 2.4% (3/127) postoperatively (P < 0.01). Soiling rate on postoperative 1 month was 32.3%. It gradually decreased to 1.6% 6 months later. Anorectal manometries showed that mean anal resting pressure was significantly reduced from 37.9 ± 12.5 mm Hg preoperatively to 20.2 ± 6.4 mm Hg on postoperative 1 month and 24.8 ± 9.9 mm Hg on postoperative 6 months, which were similar to age-matched normal controls (27.9 ± 9.6 mm Hg, P > 0.05).
TRM-PIAS is effective in treatment of HD. It is associated with low postoperative HD-associated enterocolitis.
Morio Kasai: A remarkable impact beyond the Kasai procedure
2012, Journal of Pediatric Surgery
Citation Excerpt :
In 1948, Swenson and Bill [15] reported the use of a pull-through rectosigmoidectomy for children with Hirschsprung disease. Because Dr Kasai felt that this procedure was not feasible for small infants because of technical difficulties and because while awaiting this procedure many died of enterocolitis despite the use of a temporary colostomy, Dr Kasai therefore conceived of a novel procedure, a rectal myotomy with colectomy, for infants with Hirschsprung disease to specifically address what he described as the “anorectal achalasia” [16]. This procedure minimized pelvic dissection and preserved rectal sensation.
Morio Kasai is one of the most influential Japanese pediatric surgeons. He is best recognized in the United States for his pioneering efforts in the field of biliary atresia. His work revolutionized the treatment of infants born with biliary atresia throughout the world. Less is known about his remarkable impact in the fields of general surgery and pediatric surgery. This review highlights some of Dr Kasai's major contributions and highlights the influence he had in the establishment of pediatric surgery as a field.
The History of Hirschsprung's Disease: Then and Now
2006, Seminars in Colon and Rectal Surgery
THEN (From Hirschsprung to Swenson):
By 2000, more than 500 articles had been published to clarify Hirschsprung’s disease. Since it was not initially differentiated from other types of megacolon, the literature up to 1950 was confused by the inclusion of inappropriate material. In 1886, Hirschsprung presented his classic description, although he failed to recognize that the cause of the proximal megacolon was in the distal, narrow, aganglionic rectosigmoid. Sixty years later, classic articles by Swenson and others appeared that focused on clinical, radiological, surgical, and histological aspects of Hirschsprung’s disease and formed the basis of diagnosis and treatment as we practice it today.
NOW (From Swenson to Genetic Mapping and Molecular Biology):
The difficulties encountered in the early procedures were in large part due to technical errors resulting from a poor understanding of the pathophysiology of this disease. As our understanding of Hirschsprung’s disease improved, operations evolved into those that are presently performed. In the period following Swenson’s procedure, surgical techniques were refined and certain dogmas were abandoned. The pertinent events leading up to the current standards of practice are discussed in this section. Although a detailed review of the molecular biology of Hirschsprung’s disease is presented in another article in this journal (Goldstein), key discoveries along the journey to present therapy are discussed here.
Primary transanal rectosigmoidectomy for hirschsprung's disease: Preliminary results in the initial 33 cases
2001, Journal of Pediatric Surgery
Citation Excerpt :
Cuff (or rectal) strictures, diarrhea, and incontinence have been shown.8-10 Endorectal split sleeve11 or posterior rectal cuff myectomy12 have been used widely. Anorectal or rectal myectomy cures the persistent anorectal achalasia after a failed endorectal pull-through.13,14
Purpose: The authors describe their newly developed technique—primary transanal rectosigmoidectomy for Hirschsprung's disease (HD) and its preliminary results in neonates and infants. Methods: Thirty-four consecutive patients (26 boys) with biopsy-proven rectosigmoid HD, aged 18 days to 4 years, underwent this new procedure. Rectal mucosectomy started 1 to 1.5 cm posteriorly and 2 to 3 cm anteriorly proximal to the dentate line. The rectal muscular sleeve below the peritoneal reflection was resected to the level of the striated muscle complex, leaving a shorter muscular cuff, into which a partial internal sphincterotomy was made posteriorly. An oblique anastomosis was constructed between the pull-through ganglionic colon and the anus canal. Results: The mean time for the operation was 160 minutes, and the average length of bowel resected was 29.5 cm (range, 12.5 to 41 cm). Two children (6.06%, 2 of 33) had 2 to 5 episodes of postoperative enterocolitis (EC). One was cured by rectal irrigation and dilation, and the another by Lynn's myectomy. Eighty-four percent of patients had 1 to 6 bowel movements per day during a 6- to 18-month follow-up period. Conclusions: Primary transanal rectosigmoidectomy for HD is logical and associated with excellent early results. A long-term follow-up is required to determine bowel functions. J Pediatr Surg 36:1816-1819. Copyright © 2001 by W.B. Saunders Company.
Modified Soave pull-through for Hirschsprung's disease: Intraoperative internal sphincterotomy
1999, Journal of Pediatric Surgery
Background/Purpose: Anorectal achalasia (AA) may persist after pull-through (PT) for Hirschsprung's disease (HD), which may cause postoperative enterocolitis (POE) and constipation. The authors modified the Soave PT (modified Soave PT, MSPT) to eliminate AA, and present their results.
Methods: This was a 16-year retrospective review of 43 patients with histologically proven HD of the rectosigmoid or sigmoid colon treated by MSPT. The MSPT ivolves excision of the posterior rectal cuff and an intraoperative internal sphincterotomy, allowing the PT colon to fit nicely.
Results: Mean age at MSPT was 16.7 months (16 were ≤3 months old [37%]; 7 were neonates [16%]). Mean follow-up was 9.2 years. Six of 43 cases (14%) had preoperative enterocolitis; only 2 of 43 (5%) had single episodes of POE. At review, 37 of 43 were older than 4 years; 29 (78%) had normal bowel function (14 had experienced soiling after MSPT, which resolved after a mean of 6.4 years); and 8 (21%) had problematic bowel function: 3 had occasional soiling, 1 had soiling only before defecation, 3 (8%) had constipation requiring laxatives or enemas, and 1 had significant soiling.
Conclusion: MSPT is safe and may contribute to a reduction in the incidence of POE and constipation.
Intestinal neuronal dysplasia as a cause of surgical failure in Hirschsprung's disease: A new modality for surgical management
1996, Journal of Pediatric Surgery
Two hundred fifteen cases of Hirschsprung's disease (HD) had follow-up after definitive surgical treatment, which had been performed between September 1983 and August 1994. Of these, 20 (9.3%) had recurrence of obstructive symptoms that was unresponsive to conservative treatment. Nine cases were improved by posterior anorectal myectomy (PARM). The other 11 patients, who did not have improvement after PARM, had a re-pull-through. Reoperation consisted of excision of the descending and left transverse colon with pull-through of the right colon, regardless of the histology of the colon. The blood supply was based on the ileocolic vessels, with or without division of the right colic artery. To provide a smooth course for these blood vessels, without kinking, and to obtain adequate length of the right colon with less dissection, the entire bowel was derotated clockwise. Re-pull-through was then performed from the left side of the abdomen. The excised bowel from both operations was examined, using H&E staining, for possible evidence of intestinal neuronal dysplasia (IND) or acquired aganglionosis. All had ganglion cells in the remaining colon and at the distal limit of the pull-through. Histological signs of IND were present in all patients in the previously pulled-through colon. Apart from one mentally retarded child, all had satisfactory bowel movements in a 10 to 54-month follow-up period. IND should be considered a cause of surgical failure in HD. Subtotal colectomy and pull-through of the right colon after clockwise derotation of the entire bowel appears to be effective when symptoms persist after conservative therapy or PARM.

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^∗: Professor in Surgery, The Second Department of Surgery, Tohoku University School of Medicine, 1-1, Seiryo-cho, Sendai, Japan.

^†: Instructor in Surgery, The Second Department of Surgery, Tohoku University School of Medicine, 1-1, Seiryo-cho, Sendai, Japan.

^#: Surgeon, Division of Surgery, Okachi Central Hospital, Yuzawa, Japan.

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Rectal myotomy with colectomy: A new radical operation for Hirschsprung's disease

Abstract

J. Pediat. Surg.

J. Pediat. Surg.

Manometric and cineradiographic studies on anorectal motilityin Hirschsprung's disease before and after operation

Tohoku J. Exp. Med.