Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Long-segment Aganglionosis-Treatment and Results

M-A. Ardelean, J. Bauer, C. Schimke, J. Schnöll, C. Heil, B. Ludwikowski
Salzburg, Austria

 

Abstract 

Purpose: To assess the surgical progresses of patients (pts) with long segment aganglionosis.

Material and methods: During the past 13 years, 56 cases of Hirschsprung's disease have been treated at our clinic. We reviewed the therapy and outcome of 15 pts with long-segment aganglionosis (LSA): 3 with aganglionosis to the midtransverse colon (MTA), 7 to the ascendent and coecum (A/CA), 4 with total colonic and distal ileum involvement (TCA), and one child with total intestinal aganglionosis (TIA). The pt with TIA had only laparotomy and staged biopsies. Twelve were treated by Swenson technique and 2 pts by other procedures. All pts received total parenteral nutrition up to 10 days postoperative to ensure appropriate fluid and electrolyte status. The pts with TCA needed supplementary therapies (diet, drugs) up to 4 months to reduce the stool frequency.

Results: Two patients died. The pt with TIA died 6 months after biopsies. Another patient died 5 months after pullthrough: small bowel ileus with total intestinal necrosis. Nine of the 13 alive pts have normal stool pattern: 1 – 3 time per day. Two pts have 3 – 6 stools per day with 1 – 2 soiling per week. An adolescent pt has occasionally nightly soiling (1 – 2 per month). No actual data about one patient (A/CA pt). One TCA pt doing well immediately postoperative becomes obstipated 3 month later (after having enterocolitis). He had washout enemas for 9 months. Now he stools regularly.

Conclusions: Awareness of long-segment aganglionosis should lead to earlier diagnosis. Appropriate surgery and improvements in supportive care led to a significantly increased rate of survival. Pts with LSA even involving ileum (but no more than 50 cm of ileum) should attain a normal stooling behavior.

Key words: long-segment aganglionosis, total colonic aganglionosis, stooling behavior

 

Correspondence

Mircia Aurel Ardelean, MD, FEAPU

Paracelsus University, Department of Pediatric Surgery 

email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Introduction

The incidence of Hirschsprung’s disease was found to be between 1 in 4,417 (1) and 1 in 7,165 (2) live births with a male: female ratio of 4.1:1 in short segment, and 2.4:1 in long segment Hirschsprung's disease (2) The incidence of long-segment disease (including total colonic aganglionosis), in different studies, has a wide range of 9 % to 38 % from all Hirschsprung pts (3,4,5). The breakthrough in the surgery of congenital megacolon occurred in 1948: Swenson and Bill identified the distal segment of the colon as a benign spastic lesion producing megacolon (6). They resected the rectum and the rectosigmoid with preservation of the sphincter and pulled through the normal colon (6). Although other procedure like Duhamel, Soave, Rehbein, (or the modifications brought by Boley, Martin or Kimura mostly for long-segment disease) have been instituted to improve functional results, Swenson procedure proved to be as good as the others if the procedure is carried out correctly.

Materials and Methods

Hospital records of 57 patients with biopsy-proven aganglionosis from 07/1990 to 01/2004 were reviewed. We found 15 patients with long-segment aganglionosis: 3 with aganglionosis to the midtransverse colon, 7 to the ascendent and coecum, 4 with total colonic and distal ileum (15 to 45 cm) involvement, and one child with total intestinal aganglionosis extending to the duodenum (Fig. 1). All but the last had pull-through procedures performed at our institution. Ten patients were primarily operated by our team (all TCA included), the other 5 were reoperated by us. There were 13 boys and 2 girls. All patients with TCA and the patient with TIA were boys. From primarily operated pts, 6 were under 1 year of age, the other 3 pts were 2, 3 and 5 years old at the time of pull through. The pt with TIA had no pull-through operation but laparotomy and staged biopsies. Preoperative 5 pts had enterocolitis and 3 pts had ileus. The five reoperated pts were: one under 1 year, one 3, one 5 and two 7 years old. All these pts had the primary pull through when they were younger than 1 year.

Primary procedures used in these last 5 cases were Rehbein (n=3), Duhamel (n=1) and laparoscopic Swenson (n=1). The 3 patients formerly operated by Rehbein technique, presented with aganglionic rectum 6 to 10 cm proximal to dentate line. The patient operated by Duhamel technique had a 7 cm aganglionic rectum pouch with colorectal anastomosis 3 cm proximal to dentate line. The laparoscopic pull-trough (MTA-patient) was complicated by anastomosis fistula with pelvic abscess and totally stricture of the anastomosis. Also in this case was found an aganglionic distal segment 20 cm long. Before pull-through at our institution 5 primarily treated pts (3 with TCA) had ileostomies and the resection of aganglionic bowel (Fig. 2) as a first operation followed by pull-through 2 to 6 months later. Also 2 from 5 reoperated pts had colostomies before pull-through. The following pull-through procedures were achieved: Swenson in 12 pts, Duhamel in one and a combined approach (laparotomy and posterosagittal) in one.

long segment agang 1 long segment agang 2
Figure. 1. Total colonic and terminal ileum aganglionosis – intraoperative finding. The abdominal x-ray  in the corner, 60 hours after irrigoscopy, still shows the contrast inside the colon. Figure 2. Resected aganglionic bowel: all colon and a part of ileum (about 45 cm)

The last procedure was achieved in the patient with primary laparoscopic pull-through. The reason for posterosagittal approach was the cicatricial tissue in the pelvis after abscess by anastomosis fistula. Regarding only the TCA patients, 3 of them were treated by Swenson (total colectomy with ileum-rectum end-toend anastomosis) and one by Duhamel. The leaving rectum segment in these patients was 1 to 4 cm long. Postoperative management consisted of total parenteral nutrition 5 to 10 days, followed by gradual introduction of oral feeding beginning with a low-osmolarity low-residue diet. Three TCA patients needed Loperamide, and 2 of them washouts with saline for 3- and 6 months respectively.

Results

The pts were followed-up 6 month to 13 years postoperatively. There has been no operative-related mortality in these series. Two patients died: the pt with TIA died 6 months after biopsies, and another patient died 5 months after pull-through by small bowel ileus with total intestinal necrosis. There were 2 major and 8 minor complications in 13 surviving patients. The major complications occurred both after a secondary pull-through. They consisted in an anastomosis fistula 3 weeks postoperative needing a temporary ileostomy, and a volvulus developed 3 years postoperative needing surgery. As minor complications were registered subileus in one pt with spontaneous resolution, enterocolitis (one to three episodes) in 4 pts (2 TCA pts) and postoperative perineal skin irritation in 3 patients up to 1 year postoperative. Nine of the 13 alive pts have normal stool pattern: 1 – 3 time per day. Two pts have 3 – 6 stools per day with 1 – 2 soiling per week. An adolescent pt has occasionally night soiling (1 – 2 per month). There are no actual data about one patient (A/CA pt). The pts with TCA needed supplementary therapies (diet, drugs) up to 4 months to reduce the stool frequency. One TCA pt doing well immediately postoperative becomes obstipated 3 months later, after having enterocolitis. Thereafter he needed washout enemas for 6 months. Now he stools regularly (3 time/day), and has no symptoms. The levels of serum iron, serum ferritin, folic acid, and B12 vitamin were determinate in all TCA pts. Two of them needed iron, B12 vitamin and folic acid supplementation up to 1 year postoperative. The ferritin level was normal in all patients.

Discussion

The major advances that have occurred in the management of Hirschsprung's disease include the definitive management of the newborn, our understanding of Hirschsprung's-associated enterocolitis and the treatment of this entity, and the successful management of the very complex form of this disease, total colonic aganglionosis. (7) The most common surgical techniques used in Hirschsprung's disease are Swenson (introduced in 1948), Duhamel (introduced in 1956) Soave (introduced in 1963) and the modification of the last one (Boley 1968). In total colonic aganglionosis these operations have been performed without or with modifications: most known are Martin modification of Duhamel procedure, and Kimura procedure. Both of them preserve a part of the aganglionic colon aiming water resorbtion leading to the formed stools. There is no agreement about the need to preserve (8,9,10) or not (11,12,13,14) a patch of aganglionic colon in TCA patients. In our opinion there are some arguments against preservation of the aganglionic patch: the aganglionic patch alternate the motility with subsequent stasis and obstruction; and postoperative enterocolitis seems to be a frequent occurrence in patients having a patch of aganglionic bowel. There are four mainly causes leading to failure in surgical therapy of Hirschsprung disease:

  •  incomplete resection of aganglionic bowel
  • ischemia of pull-through segment 
  • associated intestinal neural dysplasia
  • postoperative enterocolitis

In 4 of reoperated pts we found a high made anastomosis, more than 4 cm above the dentate line. Ischemia may have a pathogenetic role in some cases of acquired Hirschsprung's disease. Vascular compromise of the distal bowel segment at the time of the initial pullthrough procedure may contribute to the selective loss of ganglion cells postoperatively because neural tissues are most sensitive to hypoxia. This finding must be considered when recurrent obstructive symptoms develop following pull-through for aganglionosis (15). The patients developing recurrent symptoms of Hirschsprung's disease, after adequate surgical correction, have to be fully studied with sequential biopsies in order to gain a better understanding of their condition (16).

The 5th reoperated pt (status post laparoscopic pull- through) had the anastomosis 1 cm above the dentate line. The proximal end of the resected colon at first operation was normal in histological examination. By redo operation a 20 cm aganglionic colon was found, very probably due to intraoperative ischemia. The key of success in the therapy of Hirschsprung’s disease is to pull-through a healthy bowel doing the anastomosis down within 1 - 2 cm to the dentate line. In TCA pts we preserved a little more from aganglionic rectum (2 to 3 cm). Even in this situation the pts developed diarrhea, needing diet and loperamide up to 4 month postoperative.

One of 4 TCA pts becomes obstipated 3 months postoperatively, after having enterocolitis. He had washout enemas for 6 months. However the long term results are good, showing us that the preservation of 2 – 3 cm of rectum in TCA-pts obviates the need to use an aganglionic colonic patch. The major postoperative complications occurred both in the patients having a second pull through operation: anastomotic fistula leading to ileostomy (1 pt) and volvulus needing surgery (1 pt). Two of these 5 patients have soiling 1-2 time per week. All reoperated pts had aganglionic segments. We didn’t find skip aganglionic area (17) in these children

Conclusions

Awareness of long-segment aganglionosis should lead to earlier diagnosis. Appropriate surgery and improvements in supportive care led to a significantly increased rate of survival. Regularly follow-up, early recognition and aggressive therapy of enterocolitis lead to good outcomes. Patients with long segment aganglionosis even involving a part of ileum, should attain a normal stooling behavior.

 

 

REFERENCES

  1. Spouge D, Baird PA. Hirschsprung disease in a large birth cohort. Teratology. 1985 Oct;32(2):171-7.
  2. Russell MB, Russell CA, Niebuhr E. An epidemiological study of Hirschsprung's disease and additional anomalies. Acta Paediatr. 1994 Jan;83(1):68-71.
  3. Sherman JO, Snyder ME, Weitzman JJ, Jona JZ, Gillis DA, O'Donnell B, Carcassonne M, Swenson O. A 40-year multinational retrospective study of 880 Swenson procedures. J Pediatr Surg. 1989 Aug;24(8):833-8.
  4. Foster P, Cowan G, Wrenn EL Jr Twenty-five years' experience with Hirschsprung's disease. J Pediatr Surg. 1990 May;25(5):5314.
  5. Klein MD, Philippart AI. Hirschsprung's disease: three decades' experience at a single institution. J Pediatr Surg. 1993 Oct;28(10):1291-3; discussion 1293-4.
  6. Swenson O, Bill A. Resection of rectum and rectosigmoid with preservation of sphincter for benign spastic lesion producing megacolon: experimental study. Surgery 1948, 24: 212
  7. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung's disease.Am J Surg. 2000 Nov;180(5):382-7
  8. Shermeta DW, Meller JL. Ideal treatment for total colonic Hirschsprung's disease. J Pediatr Surg. 1989 Jan; 24(1):88-91.
  9. Emslie J, Krishnamoorthy M, Applebaum H. Long-term follow-up of patients treated with ileoendorectal pull-through and right colon onlay patch for total colonic aganglionosis. J Pediatr Surg. 1997 Nov;32(11):1542-4
  10. Nishijima E, Kimura K, Tsugawa C, Muraji T. The colon patch graft procedure for extensive aganglionosis: long-term followup.J Pediatr Surg. 1998 Feb;33(2):215-9.
  11. Bergmeijer JH, Tibboel D, Molenaar JC. Total colectomy and ileorectal anastomosis in the treatment of total colonic aganglionosis: a long-term follow-up study of six patients. J Pediatr Surg. 1989 Mar;24(3):282-5.
  12. Tsuji H, Spitz L, Kiely EM, Drake DP, Pierro A. Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg. 1999 Jan;34(1):158-61
  13. Dodero P, Magillo P, Scarsi PL. Total colectomy and straight ileo-anal soave endorectal pull-through: personal experience with 42 cases. Eur J Pediatr Surg. 2001 Oct;11(5):319-23.)
  14. Rintala RJ, Lindahl HG. Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg. 2002 Jan;37(1):66-70
  15. West KW, Grosfeld JL, Rescorla FJ, Vane DW. Acquired aganglionosis: a rare occurrence following pull-through procedures for Hirschsprung's disease. J Pediatr Surg. 1990 Jan;25(1):104-8; discussion 108-9.
  16. Cohen MC, Moore SW, Neveling U, Kaschula RO. Acquired aganglionosis following surgery for Hirschsprung's disease: a report of five cases during a 33-year experience with pullthrough procedures Histopathology. 1993 Feb;22(2):163-8
  17. Berger S, Linke F, Heymanns M, Boor R, Coerdt W, Hofmann-v Kap-herr S. Complex long-segment intestinal dysganglionosis. J Pediatr Surg. 2000 Jul;35(7):1123-6.