Soave Procedure Research Articles

The treatment and postoperative complications of Hirschsprung's disease

One hundred and ten patients with Hirschsprung's disease diagnosed and treated from 1981 to 1990 at the Royal Children's Hospital, Melbourne, were studied retrospectively. Eighty (72.7%) were treated by the Boley modification of the Soave procedure, 13 by the original Soave procedure, 13 had a Duhamel operation, and 4 had other procedures. Postoperative mortality was 2.7% (3 patients, 2 of whom died of early complications). Early postoperative complications occurred in 29 patients (26%); the most common was bowel obstruction, which included anastomotic obstruction (16 cases). Eight of these patients required anal dilatation to resolve obstruction. Enterocolitis occurred in 5 patients. There were no significant differences in incidence of early complications related to surgical procedure. Late postoperative complications occurred in 43 (39%) cases. Enterocolitis developed in 31 patients, with 19 requiring anal dilatation; 17 had bowel obstructions at various levels. The incidence of bowel obstruction was significantly higher in post-Boley patients (P <0.05). Length of follow-up was up to 10 years (average: 3.9 years). At the final follow-up (average age: 5.4 years) nearly 20% of patients were constipated, and 20% had some degree of incontinence or soiling. One half of the patients had normal bowel habits.

Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung's patient

One hundred seventy-eight of 326 patients had surgery for Hirschsprung's disease at Red Cross Children's Hospital (1957 to 1990) agreed to participate in a recall follow-up study. Assessment of the postoperative outcome of the Swenson, Duhamel, and Soave procedures included manometric evaluation in 43 patients by perfused-tube and balloonseries techniques. Rectal suction biopsies were performed for patients who had persistent problems with stool evacuation. One hundred fifteen of the 178 patients were more than 4 years of age, and long-term functional outcome could be assessed. Although good results were obtained in 94% overall, 16 had clinical evidence of a degree of perrsisting obstruction. Results of manometric assessment of anorectal function in these patients were not significantly different from those of 28 patients who had normal stool evacuation. A biopsy was performed in 14 of the 16 patients who had symptoms of obstruction postoperatively, and abnormal histological features were noted. There was aganglionosis in four, features of neuronal intestinal dysplasia in nine, ganlioneuromatosis of the colon in one. The results of two biopsies were entirely normal. Implications of Postoperative dysfunction after surgery for Hirschsprung's disease are discussed, and a protocol for investigation and management is proposed.

Posterior sagittal rectal myectomy for persistent rectal achalasia after the Soave procedure for Hirschsprung's disease

For the management of persistent rectal achalasia after the Soave endorectal pull-through procedure, we have used posterior sagittal myectomy of the remaining aganglionic rectal muscular cuff, and have had satisfactory outcomes in five patients. Via a posterior sagittal skin incision, the posterior aspect of the rectal muscular cuff is reached. With the striated muscular comlex retracted downward, the level of the dentate line is identified on the posterior wall of the rectum with the aid of the surgeon's finger inserted inside the anorectum. Two parallel longitudinal incisions are made on the rectal muscular cuff to create a muscular strip which is elevated and excised; the distal end of the myectomy strip is at the level of the dentate line and includes a part of the internal anal sphincter muscle. During the last 4 years, we performed this procedure in 5 patients with remarkable relief of constipation, distension, and enterocolitis. The advantages of this procedure include: (1) less technical difficulty than the transanal approach, (2) avoiding colostomy, and (3) promising results.

Management of Hirschsprung's disease: curative surgery before 3 months of age.

Curative surgery of Hirschsprung's disease (HD) was performed in 32 infants younger than 3 months of age from January 1, 1977, December 31, 1986. There were 24 males, seven of whom presented with total colonic aganglionosis. Preoperative relief of obstruction was carried out by only enemas in 25 patients (81%), with addition of total parenteral nutrition of 8 to 27 days in case of severe enterocolitis. Colostomy was never performed as a routine procedure. Preoperative morbidity occurred in three cases: one enterocolitis and two sigmoid performations that were successfully treated by colostomy. Weight at operation ranged from 3.3 to 6.0 kg (mean, 4.9 kg). Swenson's (25), Duhamel's (5), and Soave's procedures (2) were performed. There was no mortality. Follow-up varied from 2 to 10 years. Postoperative morbidity occurred in five patients (one anastomotic leak, two stenosis, one volvulus, and one recurrent enterocolitis). All other patients are considered as definitively cured. Comparison with data in the literature permits one to advocate primary corrective treatment of HD without decompression in infants less than 3 months of age.

Anal continence following soave procedure: Analysis of results in 100 patients: L.W. Martin, J.E. Fischer, H.J. Sayers, et al. Ann Surg 203:525–530, (May), 1986

Anal continence following soave procedure: Analysis of results in 100 patients: L.W. Martin, J.E. Fischer, H.J. Sayers, et al. Ann Surg 203:525–530, (May), 1986

Hirschsprung's disease

From 1972 to 1985, 214 patients were treated for Hirschsprung's disease. One patient underwent a Swenson pull-through operation, 13 a Soave procedure, and 195 had modifications of the Duhamel procedure. Six children are newly diagnosed and awaiting definitive therapy. Of the patients in whom a modified Duhamel procedure was performed, 153 were males (78%) and 7 (4%) had Down's syndrome. There was no operative mortality for the procedure, and only 4 late deaths have been observed (3 with Down's syndrome). Fecaloma occurred in 12 of 14 patients who underwent the classic operation described by Duhamel, and postoperative enterocolitis was noted in 4 of 6 patients who had Martin's “long” Duhamel modification for total colonic aganglionosis. No urologic problems or anastomotic leaks were identified, and only 1 patient developed a rectal stricture postoperatively. Growth and development and fecal continence have been excellent in the vast majority of patients. The modified Duhamel pull-through operation is associated with excellent survival and highly acceptable long-term function.

Hirschsprung's disease in young adults

Hirschsprung's disease is rarely seen in the young adult, and presents unique problems in management because of the massive dilatation and hypertrophy that occur proximal to the aganglionic rectum or the rectosigmoid colon. The diagnosis, which may be suspected by barium enema, is confirmed by suction or full-thickness biopsy of the rectum that may be complemented by anal manometry. Based on our experience with eight patients, a two-stage surgical reconstruction is recommended, with a preliminary sigmoid colostomy through the normally innervated colon and an associated defunctionalized stoma constituting the initial operation. The distal colonic stoma permits cleansing of the caudal colon while the normally innervated proximal colon reverts to near normal caliber, usually within 2 to 6 months. This approach is in accord with the recommendation of Fairgrieve [2]. Reconstruction using a Duhamel or Soave procedure has given good results. The Duhamel procedure seems preferable when a considerable discrepancy remains between the ganglionic and aganglionic segments of rectum [4].

Tubular duplication of the rectum treated by mucosal resection

This is a case report of an infant with tubular duplication of the rectum, extra lobular lung sequestration, gut malrotation, and a Meckel's diverticulum. The duplication was removed by a mucosal sleeve resection similar to that used in a Soave procedure for Hirschsprung's disease.

Acquired aganglionosis after Soave's procedure for Hirschsprung's disease: T. G. Cogbill and J. R. Lilly. Arch Surg 117:1346–1347, (October), 1982

Acquired aganglionosis after Soave's procedure for Hirschsprung's disease: T. G. Cogbill and J. R. Lilly. Arch Surg 117:1346–1347, (October), 1982

Acquired Aganglionosis After Soave's Procedure for Hirschsprung's Disease

• A patient had acquired aganglionosis after Soave's procedure for Hirschsprung's disease. Vascular compromise of the sigmoid pull-through at the time of the operation was the probable cause. Posterior myomectomy of the pull-through segment was curative. (Arch Surg1982;117:1346-1347)

Five years of the pediatric kock pouch

Since 1976, 16 teenagers at the Hospital for Sick Children, Toronto, have had a Kock pouch constructed. This group consists of 10 females and 6 males ranging in age from 13 to 19 yr. These were all elective pouches: 9 were converted from standard ileostomies, 5 were converted along with proctectomy or Hartmann procedure, and 2 had total colectomies along with a Kock pouch. Two of these conversions were for failed Swenson and Soave procedures. The complications directly related to the Kock pouch were stoma stricture, prolapsed nipple valve, long outflow tract, fecal fistula, salt loss, slipped nipple valve, chronic small bowel obstruction, and "pouchitis." These 8 complications required a total of 16 operations to correct (1.4 operations per patient). The 100% follow-up shows all but 3 followed for more than 1 yr. They are all well, continent, happy, and back to a virtually normal life that includes marriage in 3. The Kock pouch should be an elective procedure and must be done only when the total colon has been removed. There must be rigid selection of pediatric patients for this operation and no children younger than teenage should receive this pouch. Moreover, it may even by worthwhile letting such a child have a standard ileostomy for a while until his or her general condition (both physical and mental) is best able to cope with a Kock pouch.

The Story of Mellisaʼs Surgery

The Soave procedure involves a total colectomy, removal of rectal mucosa, ileorectal muscular wall anastomosis, and temporary ileostomy—which is later followed by a connection with the remaining bowel portion. The Soave, as well as other surgeries which begin with a temporary ileostomy, is still subject to clinical debate as “the” solution to ulcerative colitis.

Acquired aganglionosis

A documented case of acquired aganglionosis is presented in which transrectal biopsies taken 4 mo apart first showed the presence then subsequently the absence of ganglion cells. Colostomy followed by a Soave procedure was curative.

Anastomosis by invagination

The surgeon faced with cancer of the mid-third of the rectum who wishes to eradicate the tumor, yet avoid an abdominal colostomy, has three options. Goligher recommends the anal eversion pull-through technique of Cutait-Turnbull; however, an endorectal pull-through procedure similar to the Soave procedure where the mucosa is removed has also received favorable comment. Finally, there is the invagination technique utilized by Black.

Sodium balance following soave iloe-endorectal pull-through

Five children with ileostomies established in the neonatal period and modified Soave ileo-endorectal pull-throughs done at 7–14 mo of age have been followed. Episodes of salt and water depletion occurred both pre- and post-pull-through. Following the Soave procedures, all patients experienced recurrent episodes of abdominal distention with diarrhea, hypovolemia, and hyponatremia. Treatment included oral and intravenous sodium supplementation, as required. Sodium balance studies done (in three of the five) when the children were 5–9 yr of age suggested that although they appear well, the patients are in tenuous sodium balance. One hundred meq of dietary sodium were provided for 7 days followed by 7 meq sodium/day for 5 days. The children tolerated the sodium restriction poorly in spite of maximal renal sodium conservation. The response of the gastrointestinal tract to sodium restriction was a reduction of mean daily sodium output from 55 meq to 17 meq. This limited adaptation, which was not observed until the third day of restriction, was inadequate. The children lost weight and developed the signs and symptoms of salt and water depletion. Presumably renal and gastrointestinal sodium conservation was a response to circulating aldosterone which rose to levels 15–90 times those observed in normal children.

A critical look at the soave procedure for Hirschsprung's disease

S INCE THE FIRST U.S. PUBLICATION of endorectal pullthrough for Hirschsprung's disease by Soave in 1964,1 many pediatric surgeons have employed this procedure. It has been used in 20 patients between 1964 and June 1972 at the Children's Hospital of Pittsburgh. The immediate results have been far from satisfactory, and the purpose of this paper is critically to analyze our experience with the Soave procedure.

Hirschsprung's disease: pitfalls in diagnosis and surgical management.

Forty-seven (41 male) patients with biopsy-proved Hirschsprung's disease were seen in the past decade. The condition of more than half the patients was diagnosed within the first three months of life. These patients harbored bewildering clinical and radiographic manifestations and suffered a high mortality (22%), mainly from sepsis and enterocolitis. Patients whose condition was diagnosed after the first three months of life developed a more characteristic constipation pattern and none died. Sixteen of the 38 patients who underwent corrective operation received the modified Duhamel procedure and 15 the modified Soave. A comparison of these two groups at follow-up showed them to be similar, the modified Soave procedure having a slightly better record, 14 of 15 excellent to good results as opposed to 12 of 16 for the modified Duhamel.