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Managing Patients with Hirschsprung’s Disease

A. Mironescu, L. Muntean
Brasov, Romania

 

Abstract 

Purpose: The aim of this report is to present some of our particular cases with HD and the difficulties we had to face in managing them, but also an attempt to adapt the modern recommendations for the management of HD to the still frustrating technical conditions in a developing country.

Material and methods: In the present paper we are reconsidering a previous series of patients presented with constipation in the Pediatric Surgery Department of the Children’s Hospital of Brasov, but also the medical records of the last five years in the same surgical service.

Results: Between 1991 and 2005, over 300 children presented in the outpatients clinic, 150 were admitted for constipation, of which, 30 were confirmed as HD, 90 as functional constipation and 30 as constipation of unknown etiology. Our experience was limited until 2000 to Duhamel procedure with or without a previous colostomy. In the last five years we preformed several Soave-Boley endorectal pull-through in patients between 2 months and 1 year old , with good results.

Conclusions: Despite some technical problems and limited access to special histopathology tests, our results have been encouraging and new procedures could be adapted to local conditions until sufficient experience and improvement of surgical equipment will be achieved.

Key words: Hirschsprung’s disease, surgery, endorectal pull-through

 

 

Hirschsprung’s Disease (HD) surgical management is in continuing progress and new techniques are developed, based on the classical ones, but adapted for a minimally invasive strategy. The basic principles of the endorectal pull-through have been first described by Soave in 1964 (11), then modified by Boley (1) who performed the coloanal anastomosis during the pull through. Others have imagined combined techniques, laparoscopic and endorectal (Georgeson et al) 5 or just endorectal pull-through in newborns without a previous colostomy (Soave et al)(10). But the first ones to report HD patients managed by an exclusive transanal approach were De la Torre-Mondragón and Ortega-Salgado in 1998, who performed mucosectomy, colectomy and pull-through in this manner in 5 cases(2).

The aim of this report is to present some of our particular cases with HD and the difficulties we had to face in managing them. Our experience was limited until 2000 to Duhamel procedure with or without a previous colostomy. In the last five years we preformed several Soave-Boley endorectal pull-through in patients between 2 months and 1 year old, with good results. Still in one small patient, due to technical difficulties, we were forced to convert a Soave-Boley pull-through into a Duhamel. Thus we made steps for a next approach, the transanal technique, when technical conditions and more experience enabled us to perform such a delicate and difficult procedure.

Materials and metods

In the present paper we are reconsidering a previous series of patients presented with constipation in the Pediatric Surgery Department of the Children’s Hospital of Brasov, but also the medical records of the last five years in the same surgical service. We are also presenting some of the lately managed patients with atypical presentations and difficulties in their management. The diagnostic criteria included a thorough history, the clinical signs and symptoms and a contrast enema in all the patients. Rectal biopsy was performed in only 50% of the patients, those patients presenting with positive signs and a suggestive contrast enema for HD. No rectal manometry was performed.

Results

In 2000 we reported 90 patients admitted for different forms of constipation of over 200 children presented in the outpatients clinic for the same symptoms in the previous 10 years. Of the 90 patients admitted, 19 were confirmed as HD, 58 had functional constipation and the rest of 13 patients with constipation of unknown etiology. Five years later, 2005, of over 300 children presented in the outpatients clinic, 150 were admitted for constipation. Of those 150 patients admitted, 30 were confirmed as HD, 90 as functional constipation and 30 as constipation of unknown etiology. Among the 30 patients with HD, there were 20 with classical recto-sigmoid segment of aganglionosis, 5 with long segment and 5 with short segment. For the patients with constipation of unknown etiology, none had a conclusive classical histology for a short segment HD and in the absence of histochemistry capabilities or rectal manometry they could not be accurately diagnosed. The rectal biopsy was mainly performed as a full thickness rectal biopsy (70%), only a limited number of suction rectal biopsies were performed due to the lack of experience of the pathologist to work on very small submucosa specimens, but also to the unavailability of more specialized histochemistry or immunohistochemistry. No frozen sections were performed for our patients. The only staining available on full thickness biopsies was the classical HE staining, aiming to identify the lack or the paucity of ganglionic cells in the myenteric plexus (Auerbach).

Surgical treatment difficulties were determined initially by the lack of sufficient experience with small babies and the unavailability of certain technical facilities, such as diathermy or stapling devices. Until 5 years ago almost all HD patients had a Duhamell pull-through, initially staged procedure, then crushing clamp on the septum with single stage: long and difficult procedures for patients and parents, but results were good, with almost no soiling and only two patients needed reapplication of the crushing clamp for a residual septum. Stapling devices (GIA) for the septum had been used in two cases with improved results and short hospital stay, but devices not always available in the proper size of stapler and staples. In the last years, 5 patients had a Soave-Boley pull through, with encouraging results, shorter hospital stay (mean 7 days), but not possible in the newborn babies or the very small infants.

Patients

DENISA H., born February 2005. Admission at the age of 3 weeks for vomiting for a period of two days, 7-8 loose stools, 24 h prior to admission, high fever, sepsis signs. At 24 h past admission: distended abdomen, tympanism, small amounts of stool and gas only at introducing a rectal catheter. On plain abdominal x-ray largely distended left colon and few air/ fluid levels could be noticed (fig. 1). A suggested diagnostic was: Hirschsprung disease with enterocolitis. After fluid and electrolytes replacement therapy and broad spectrum antibiotics, stabilization afforded performing a contrast enema, which showed narrowing of the sigmoid with large descendent colon and a typical transition zone between the two segments. A full rectal biopsy confirmed rectal hypoganglionosis above the dentate line. Nursing was needed continually, and two weeks later a Soave-Boley pull-through was performed, with an uneventful recovery (fig. 2).

mamange hirsch 1 mamange hirsch 2
Figure 1. Abdominal x-ray Figure 2. ERPT

Three months later the child was well and thriving, had 3- 4 softer stools with very little perineal irritation. The pathology report confirmed normal innervations in the pulled-through segment, hypoganglionosis in the resected segment of sigmoid and at transition zone. Nine months later the girl is thriving well, no complaints, no local irritation, 1-2 normal stools in 24 h, no soiling between the bowel movements.

RARES E., born July 2005. At 40 gestational weeks, weighing 4.400g, otherwise normal newborn baby, first child of a young healthy couple. Since 2nd day he presented bilious vomiting, highly distended abdomen with tympanism and respiratory distress, but passed no meconium. On a plain abdominal x-ray free gas in the abdomen was detected confirming pneumoperitoneum (fig. 3). On emergency laparotomy: distended loops, bilious staining and peritoneal fluid from a large perforation at the cecum, with largely distended colon. 

mamange hirsch 3 mamange hirsch 4
Figure 3 Pneumoperitoneum Figure 4 Transition zone

In the left lower quadrant, a large sigmoid loop with narrowing at recto-sigmoid junction and a typical transition zone was found (fig. 4). The diagnostic of rectosigmoid Hirschsprung’s disease was obvious, severe neonatal form with diastasic cecal perforation and peritonitis. 

We performed debridment and suture of the perforation and the creation of a double-barrell colostomy on the transverse colon with immediate maturation. Biopsy from transverse colon showed normal innervation, but absent ganglion cells in the myenteric plexus from the transition zone. A postop. contrast enema confirmed typical aspect of rectosigmoid H.D. (fig. 5). A daily based anal dilatations were started after 10 days and continued for a month, than every other day until the pull-through operation. At 4 months the baby was well and thriving, and at 6 months, at 8 kg weight, he had a pullthrough performed. We started as an endorectal Soave pullthrough, but technical problems related to difficulties in developing a submucosal plane, forced us to convert to a Duhamel procedure with stapling of the septum with a GIA 60 stappler from Tyco inc. The recovery was uneventful, the patient discharged after seven days of hospitalization. After 8 weeks from the pull through, the transverse colostomy was closed without complications, the patient passed stools through the anus after 48 hours, without pain or perineal irritation. Dilatations have been maintained postoperatively with progressive Hegars. At two weeks after colostomy closure he was well, passing 2-3 soft stools without any complaints.

SZABALCS, born October 2005, from a twin couple, birth weigh 2300g, transferred from the Maternity Hospital for gastroschisis (fig. 6). At emergency surgery we found a jejunal perforation and performed a resection and anastomosis of the perforated bowel and successful primary closure of the gastroschisis. The patient had a difficult recovery, with massive discharge from the stomach, meconium passed initially, then no bowel movements for 7 days. At two weeks postop. he presented signs of obstruction, the contrast given via a nasogastric catheter did not progress to the ileal loops after 24 h. At a second look operations: multiple adhesions between the intestinal loops and to the other organs, insipated ileal content, difficult to evacuate towards the colon and through ileocecal valve. An enterotomy was performed with evacuation of dense contents and warm saline irrigation, then appendectomy, irrigation of colon through appendix stump before ligation and closure of abdominal wall.

mamange hirsch 5 mamange hirsch 6 mamange hirsch 7
Figure. 5 Contrast enema Figure. 6 Gastroschisis Figure. 7 Contrast enema

Again difficult postoperative evolution was present, with still large discharge from the stomach for 3-5 days, and then progressively reduced to 20ml/24h, when oral fluids were started. Still no stools passed for a week. On x-rays, plain and with contrast could be noticed pneumatisation of almost entire abdomen and passage of contrast down to the descendent colon (fig. 7). Evacuation of explosive stools and gas was observed at insertion of rectal catheter for more than 6-7 cm and warm saline irrigations. Contrast enema showed neat narrowing at and above the recto-sigmoid junction with enlargement of descendent colon. A third operation was decided and through a small left lower quadrant incision we confirmed the suggestive aspect for a recto-sigmoid HD and performed a transition zone, double ends, separated, sigmoid colostomy, distal end as a mucus fistula. Discharge of normal stools were noticed the same day. A month later, the baby was well and getting weight (2800g), minor local irritation around the stoma. Pathology report showed proximal end of colostomy with normal ganglionic structures, but hypoganglionic distal end. Suction rectal biopsy performed after a week could not confirm presence or absence of ganglionic cells in the sub mucosa above the dentate line. He is at home at present, thriving well and waiting for a pull-through when at least 5000g.

Discussions

The difficulties we encountered in having an accurate diagnostic of HD or other Hirschsprung-like entities are the same as in most of the developing countries.(3,7) The unavailability of frozen sections and special staining techniques makes it very difficult to confirm intraoperatively the extent of the aganglionic segment and the only resources for an approximation of the normal colon at the/or close to the transition zone are the experience and the contrast enema, if one available at the time of the operation. A lot of our patients came either as an emergency case with severe obstruction, or complicated with enterocolitis or diastasic perforation, forcing us to take immediate decisions and make those procedures that saved the patient’s life and offered the best protection against further complications. That is why we still have to perform a colostomy as an initial step, before the pull-through can be safely performed. Our results are encouraging and, so far, we had practically no mortality in patients with HD in the last 10-15 years. Described for the first time by Swenson et al in 1949 (12,13), the correlation between the radiographic transition zone on contrast enema and the level of the aganglionosis was confirmed by many other authors (9) as being accurate in 90% of cases. However, the need of a more accurate location of this level is absolutely needed when planning a one-stage pull through(8,16). The experience only and the correlation with the contrast enema gives just an approximate appreciation of the end of the transition zone and always forces the surgeon to resect the colon at a higher level to be sure that one brings normal ganglionic bowel to the anus. A lot of authors reported on the need for redo operations in patients with insufficiently resected bowel or a transition zone pull-through and persistent constipation after the initial pull-through(4,15).

As for the method to surgically manage the patients with confirmed HD, obviously the new methods prevail in most recent reports. As mentioned in the introduction, the laparoscopic assisted endorectal pull-through, described initially by Georgeson in 1995 or the one-stage mucosectomy and transanal endorectal pull-through (TEPT) described in 1998 by De la Torre Mondragon and Ortega-Salgado are now largely applied throughout the world and a lot of surgeons report large series with these procedures and encouraging reports. The aplication of these methods of treatment largely depend on certain technical conditions, magnification capabilities and experience with transanal disection. The endorectal pull-through described by Soave and modified by Boley can be a good step before performing the TEPT method especially in small infants.

An accurate and pertinent recommendation for an approach to patients with HD had been given by Hadidi in 2003 (6) and may easily be adapted to any technical conditions. If clinical diagnostic of HD is suggested, the next recommendation is for a rectal biopsy. If no ganglions are confirmed, a contrast enema is recommended. Depending on the aspect on the contrast enema, if a definite transition zone is located at the recto sigmoid junction, a TEPT procedure is recommended. If the transition zone is situated above the recto-sigmoid junction, the recommended procedure is a laparoscopic assisted TEPT, but for a less experienced surgeon with transanal dissection, an initial mini-laparotomy in the left lower quadrant, the same as the one recommended by Pena for a colostomy in anorectal malformations (17), will help visualise the opening of the seromuscular cuff and coagulate or clip the sigmoid vessels, then continue the transanal pull-through. In rare instances, when no definite transition zone can be noticed on the contrast enema, the recommendation is for laparoscopic biopsies and appendectomy. In the absence of laparoscopic facilities, both procedures can be achieved easily by a small transverse subombilical laparotomy. If ganglion cells are identified on one of the biopsies, either a laparoscopic assisted TEPT is recommended or a Soave-Boley ERPT through a limited suprapubic transverse incision. If no ganglion cells have been identified on the biopsies or in the appendix, serial biopsies from the entire intestine and a levelling ileostomy is recommended, followed later by a Martin pull-through.

Another clinical approach for HD is suggested by Teitelbaum and Coran in 2003, which start from a rectal biopsy that confirms absence of ganglion cells above the dentate line. If enterocolitis is present, they recommend considering a levelling colostomy or delay of pull-through with aggressive treatment for enterocolitis. If enterocolitis is absent, a contrast enema is recommended and if markedly dilated proximal colon is observed, consider the same as for enterocolitis. In the absence of much dilated proximal colon and the evidence of a low transition zone, the recommended procedure is a standard TEPT. If a high transition zone is observed or it cannot be determined on the contrast enema, as well as if during the transanal approach ganglion cells cannot be identified at most proximal point of dissection, a laparoscopic assisted transanal approach is recommended(14).

In our patients, a previous minilaparotomy, either in the left lower quadrant or subombilical, resulted in very mild adhesions between the intestinal loops, very easily dissected at the pull-through procedure and in a limited number of patients. Only those patients with very long aganglionosis segments, needing the dissection and pull-through of the right colon had a longer history of frequent liquid stools and perineal irritation, eventually controlled by adequate diet and nursing. All the patients that needed a left colon pullthrough had minor or no perineal complications and no soiling after the final operation. A routine dilatations program was maintained to all our patients up to one year postoperatively.

Concluding this report, despite some technical problems and limited access to special histopathology tests, our results have been encouraging and new procedures can be adapted to local conditions until sufficient experience and improvement of surgical equipment will be achieved.

 

 

 

REFERENCES 

  1. Boley SJ, New modification of the surgical treatment of Hirschsprung’s disease. Surgery 56:1015-1017, 1964
  2. De la Torre-Mondragón L, Ortega-Salgado JA, Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg, 33:1283-1286, 1998
  3. Elhalaby EA, Hashish A, Elbarbary MM, et al, Transanal one-stage endorectal pull-through for Hirschsprung’s disease: a multicentrer study. J Pediatr Surg 39: 345-351,2004
  4. Farrugia MK, Alexander N, Clarke S, et al, Does transitional zone pullthrough in Hirschsprung’s disease imply a poor prognosis? J Pediatr Surg, 38:1766-1769, 2003
  5. Georgeson KE, Fuenfer MM, Hardin WD, Primary laparoscopic pullthrough for Hirschsprung’s disease in infants and children. J Pediatr Surg 30:1017-1022, 1995
  6. Hadidi A, Transanal endorectal pull-through for Hirschsprung’s dis- ease:experience with 68 patients.J.Pediatr.Surg.38:1337-1340, 2003
  7. Krivchenya DY, Silcenko MI, Soroka VP, Endorectal pull-through for Hirschsprung’s disease: 17 year review of results in Ukraine. Pediatr Surg Int 18:718-722, 2002
  8. Langer JC, Durrant AC, de la Torre L, Teitelbaum DH, Minkes RK, Caty MG, Wildhaber BE, Ortega SJ, Hirose S, Albanese CT, One tage transanal Soave pull-through for Hirschsprung’s disease: a multicenter experience with 141 children. Ann Surg, 238:569-576, 2003
  9. Procter ML, Traubici J, Langer JC, et al, Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung’s disease: implications for surgical approach. J Pediatr Surg 38: 775-778, 2003
  10. So HB, Schwartz DL, Becker JM, et al, Endorectal pull-through without primary colostomy in neonates with Hirschsprung’s disease. J Pediatr Surg 15:470-471, 1980
  11. Soave F, Hirschsprung’s disease: a new surgical technique. Arch Dis Child 39:116-124, 1964
  12. Swenson O, Newhauser EBD, Picket LK, New concepts of etiology, diagnosis and treatment of congenital megacolon (Hirschsprung’s disease). Pediatrics 4:201-209, 1949
  13. Swenson O, Sherman JO, Fisher JH, Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 8;587-594, 1973
  14. Teitelbaum DH, Coran AG, Primary pull-through for Hirschsprung’s disease. Seminars in Neonatology 8:233-241, 2003
  15. Van Leeuwen K, Teitelbaum DH, Elhalaby EA, Coran AG, Long-term follow up of redo pull-through procedures for Hirschsprung’s disease: efficacy of the endorectal pull-through. J Pediatr Surg 35:829-834, 2000
  16. Weidner BC, Waldhausen JHT, Swenson Revisited: a one-stage, transanal pull-through procedure for Hirschsprung’s disease. J Pediatr Surg 38:1208-1211, 2003
  17. Pena A., Atlas of Surgical Management of Ano-Rectal Malformations, Springer Verlag, 17-24,1990