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A proposal to preserve the ileocecal valve and right colon in total colonic aganglionosis
A Proposal to Preserve the Ileocecal Valve and Right Colon in Total Colonic Aganglionosis By Hugo Sauer and Ingrid Klos Graz, Austria 9 W e report two cases of total colonic aganglionosis in which the ileocecal valve and right colon w e r e preserved. The aganglionic ileal segment is removed leaving behind only a very short prececal remnant, which is anastomosed end-to-back to the remaining ileum. An ileal loop (of approximately 20 cm in length) is isolated end interposed between the right colon and rectum. The operation can be performed as a staged procedure. The advantage of preserving the ileocecal valve with respect to physiology is discussed. 9 1989 by Grune & Stratton, Inc. INDEX WORDS: Total colonic aganglionosis.
COLONIC aganglionosis or even small T OTAL bowel involvement is present in 5% to 15% of cases of Hirschsprung's disease according to various reports in the literature. ~-3 In the large series reported by Ikeda and Goto,3 this was true in 8.4% of 1,628 patients, with a mortality rate of 40.9%. Enterocolitis was a frequent finding (44.3%). Since the first publication4 of the Martin procedure, many attempts have been made to improve the patient's situation by preserving parts of the colon in order to maintain reabsorptive function.5s Martin himself improved his original method by performing a side-to-side anastomosis of the entire colon to the small bowel in 1982.9 Water reabsorption occurs mainly in the right colon and to a lesser degree also in the left colon, while no absorption takes place in the rectum. ~~ This would explain why Martin's original method using the left colon was not successful overall. The loss of the ileocecal region leads not only to malabsorption of vitamin B I2, fatty acids, and bile acids, but may also allow ascension of colonic bacteria into the small intestine. Therefore, the chance of survival in children with short bowel syndrome is clearly better if the ileocecal valve is preserved? As we demonstrated in three cases of total colonic aganglionosis without ileal involvement, preservation of the cecum and ileocecal valve is possible. ~2 The aganglionic cecum cannot be anastomosed to the aganglionic rectum, a problem that was solved by interposition of an ileal segment between the two parts of the colon. A protective cecostomy led to a significantly less complicated course than that in patients treated by ileostomy. The method published in 1972~3 cannot be applied to cases with ileal involvement (Fig 1). However, encouraged by the good results mentioned herein, Journal of Pediatric Surgery, Val 24, No 5 (May), 1989: pp 457-461
Fig 1. Extent of resection (left) and interposition of an ileal segment between the aganglionic ascending colon and rectum (right).
a technique was developed that allowed preservation of the ileocecal valve even in cases with ileal involvement. MATERIALS AND METHODS As in our original method, the continuity between the right colon and rectum is established by interposition of an ileal loop containing ganglion cells. The right colon remains in its normal position. Multiple frozen sections are taken from the ileum and colon to identify the extent of aganglionosis. Proximally, the entire aganglionic part of the ileum is removed and transected in an area of normal ganglion cell distribution, while distally about 2 to 3 cm of the affected prececal ileum is preserved. This remaining segment of aganglionic ileum is incised longitudinally on the antimesenteric side, almost down to the ileocecal valve. After isolating about 20 cm of normally innervated ileum for interposition, the proximal ileum is anastomosed end-to-back to the distal aganglionic ileal segment (Fig 2). Whether the anastomosis should be performed behind or in front of the isolated loop must be decided on an individual basis. A side-to-side anastomosis is then carried out between the isolated ileal loop and right colon in an isoperistaltic manner, if possible (Fig 3). However, it is imperative to orient the interposed ileal loop in order to direct peristalsis toward the rectum. To accommodate this procedure, the loop should he fixed to the cecum by stay sutures at an early phase. In cases of extensive aganglionosis, the distal aganglionic part of the ileum may be preserved and anastomosed side-to-side to the
From the Department of Paediatric Surgery, University of Graz, Austria. Date accepted: September 30, 1988. Address reprint requests to Professor Dr H. Sauer, Universit~tsklinik ff~r Kinderchirurgie, Heinrichstra[3e 31, A-8010 Graz, Austria. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2405-0010503.00/0 457
458
SAUER AND KLOS
C /
Fig 2. (A) Resection of aganglionic ileal segment. Resection line A is 2 to 3 cm proximal to the ileocecal valve in affected area, resection line s is in ganglionic area. (B) s to c: normal ileum to be used f o r interposition.
B
D
C Fig 3. Side-to-side anastomosis between ileal loop (B to c) and ascending colon. End-to-back anastomosis of ileum (A to 0).
Fig 4. Second step in staged procedure. A to D, ileo-ileal anastomosis; c to s, ileal conduit; 8, distal end of conduit as stoma. The proximal end of the distal colon is also exteriorized to facilitate later Ueorectal anastomosis.
TOTAL COLONIC AGANGLIONOSlS: ILEOCECAL VALVE
proximal ganglionic part of the ileum in order to improve reabsorption of water) 4'x5 The distal anastomosis between the ileal conduit and rectosigmoid can be performed either by using the Martin procedure or as an end-to-end anastomosis to the rectum (according to Rehbein). In the future, we suggest using the stapler after dilating the rectum to at least Hegar 22.
459
C
D
Patient Evaluation and Staging of Operations The procedure can be performed in one, two, or three stages. If there is suspicion of Hirschsprung's disease, we try to prove the diagnosis by suction biopsy and histochemical investigation as soon as possible. The operation takes place with an experienced pathologist present. When the extent of aganglionosis has been established, the remaining steps will depend on the patient's condition. In newborns or infants in poor condition, a loop ileostomy is performed in an area that contains ganglion cells, according to multiple extramucous biopsies. In order to rule out the danger of persisting enterocolitis, ~ the part of the transverse and descending colon that will not be used later is removed immediately. The second stage consists of ileo-ileal and proximal ileocolic anastomosis; the distal end of the ileal conduit is brought out as a terminal stoma in the left lower abdomen (Fig 4). If the patient is in a good condition, the operation is done as the first step, without previous ileostomy. The distal ileorectal anastomosis can be performed either as the third stage or in addition to the preceding step, depending on the patient's condition (Fig 5).
CASE REPORTS
B
Patient 1 A baby boy (born January 21, 1981) with a birth weight of 3,600 g was first seen by us at the age of 31/2 months. He had suffered from hemorrhagic diarrhea since birth, and had undergone a laparotomy elsewhere for signs of ileus, but no mechanical obstruction could be identified; the only finding was a narrow distal ileum. The child was operated on in our department at the age of 4 months; we found a narrow colon and terminal ileum, with ileal dilatation 20 cm proximal to the ileocecal valve. A rapid frozen section of this area showed marked hypoganglionosis and normal ganglion cells at 30 cm. As the first step, we removed the aganglionic part of the ileum, performed an ileo-ileal end-to-back anastomosis, and did a sideto-side anastomosis between the right colon and isolated ileal loop. One month later, the conduit was anastomosed to the rectosigmoid using the Martin procedure. During coloscopy 9 months after the last operation, the distal anastomosis was inconspicuous and so was the ileal conduit; the ileocecal valve could not be visualized. A defecation radiogram 3 years later showed a conical proximally-widening ampulla recti in the filling phase, and no relaxation of the internal sphincter. When trying to defecate, there was no coordinated relaxation of the puborectalis muscle, but in a later phase, the anal canal opened in a band-like fashion and the puborectalis relaxed. Seven years after surgery, it was evident that the patient had developed normally. He is clean during the day (with four or five stools), while at night there is some soiling from time to time. His anal sphincter is somewhat rigid, necessitating anal dilatation about twice a year.
Patient 2 A baby boy born June 15, 1985 had a birth weight of 3,200 g. The operation for this patient was also done in two stages--the first at the age of 17 days, and the second 6 months later. The rectal anastomosis was performed end-to-end, according to Rehbein. There were no
Fig 5. Procedure completed. A t o o: ileo-ileal a n a s t o m o s i s ; e: proximal closed end of ileum attached side-to-side to cecum and ascending colon; a: distal end of ileum conduit--ileorectal a n a s t o mosis.
complications, the child has developed normally, and appears to be continent. There are two bowel movements daily. A contrast enema performed 3 months after the second operation showed a short rectal cuff with a normal caliber, while the caliber of the interposed intestine was changing, but never exceeded that of the rectum. No reflux beyond the ileocecal valve could be demonstrated (Fig 6).
DISCUSSION Reports about the varying operative procedures that have been applied to treat total colonic aganglionosis a r e c o n t r o v e r s i a l . C a s s a n d M y e r s ~ b e l i e v e t h e r e is n o n e e d to p r e s e r v e p a r t o f t h e a g a n g l i o n i c c o l o n ; t h e y a p p e a r to a p p l y t h e M a r t i n m e t h o d o n l y i f w a t e r y i l e o s t o m y stools p e r s i s t f o r > 2 4 m o n t h s . F 6 k 6 t 6 e t al, 16 despite using this procedure regularly, believe that
460
SAUER AND KLOS
Fig 6. Consecutive phases of contrast study. (A) Site of ileorectal anastomosis (arrow). (B) Ileocecal pouch; no stanosis at site of ileorectal anastomosis (arrows). (C) Normal motility of ileum; no dilatation of proximal loops.
TOTAL COLONIC AGANGLIONOSIS: /LEOCECAL VALVE
461
" t h e best t r e a t m e n t is p r o b a b l y a low-pressure pouch system ensured by a low-placed wide a n d supple ileoa n a l - a n a s t o m o s i s . " In one of their patients, after the M a r t i n procedure, it was necessary to remove the colon and p e r f o r m an ileo-anal anastomosis. W e a r e convinced t h a t use of the left colon is responsible for the u n f a v o r a b l e results in some o f the cases. Good results a r e r e p o r t e d by o t h e r investigators, 2'~7 and by M a r t i n himself. However, being dissatisfied with his early results, Martingnow prefers to preserve the whole colon. In a c o m p a r a t i v e study on 50 colect o m i z e d children, K r a e f t et al is were u n a b l e to demons t r a t e a n y a d v a n t a g e o f one operative p r o c e d u r e over another. T h e n u m b e r of patients t r e a t e d by surgical m e t h o d s described by Boley, 6 K i m u r a et al, 7'19 and K o t t m e i e r et al Js is still too small for j u d g e m e n t ,
O n l y Stringel 2~ has used a side-to-side anastomosis between the ileum and colon t h r o u g h the valva ileocecalls (in one case; 1986). To date, no other proposed surgical m e t h o d s have d r a w n attention to the ileocecal valve and its specific role in protecting the ileum, a p r o p e r t y t h a t is well d o c u m e n t e d clinically and experimentally, n'22'22 The ileocecal valve acts as a physical b a r r i e r a g a i n s t reflux and ascension o f colonic bacterial flora into the small bowel; thus, it helps alleviate the absorption of bile salts and f a t t y acids. In neither of our two clinical cases could reflux o f c o n t r a s t m a t e r i a l into the ileum be d e m o n s t r a t e d . A l t h o u g h no f u r t h e r conclusions should be d r a w n from only two cases, we believe t h a t the a p p a r e n t a d v a n t a g e of a functioning ileocecal valve should be considered in the t r e a t m e n t of total colonic aganglionosis.
REFERENCES
1. Cass DT, Myers N: Total colonic aganglionosis: 30 years' experience. Pediatr Surg Int 2:68-75, 1987 2. Ikeda K, Goto S: Total colonic aganglionosis with or without small bowel involvement: An analysis of 137 patients. J Pediatr Surg 21:319-322, 1986 3. Ikeda K, Goto S: Diagnosis and treatment of Hirschsprung's disease in Japan. Ann Surg 199:400-405, 1984 4. Martin LW: Surgical management of Hirschsprung's disease involving the small intestine. Arch Surg 97:183-189, 1968 5. Applebaum H, Richardson R J, Wilkinson GA, et al: Alternative procedure for total colonic aganglionosis. J Pediatr Surg 23:4951, 1988 6. Boley SJ: A new operative approach to total aganglionosis of the colon. Surg Gynecol Obstet 159:481-484, 1984 7. Kimura K, Nishijima E, Muraji T, et al: A new surgical approach to extensive aganglionosis. J Pediatr Surg 16:840-843, 1981 8. Shandling B: Total colon aganglionosis--A new operation. J Pediatr Surg 19:503-505, 1984 9. Martin LW: Total colonic aganglionosis preservation and utilization of entire colon. J Pediatr Surg 17:635-637, 1982 10. Devroede GJ, Philipps SF: Failure of the human rectum to absorb electrolytes and water. Gut 11:438-442, 1970 11. Goto S, Gunter M, Scherer LR, et al: Surgical treatment of total colonic aganglionosis: Efficacy of aganglionic patch enteroplasty in the rat. J Pediatr Surg 21:601-607, 1986 12. Wilmore WD: Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 80:88-95, 1972
13. Saucr H: Diinndarmzwischenschaltung nach subtotaler Colektomie wegen Mb. Hirschsprung im Kindesalter. Chirurg 43:280-282, 1972 14. Schennach W, Sauer H, Menardi G: Die totale Aganglionose des Dickdarmes. Z Kinderchir 23:33-34, 1978 15. Kottmeier PK, Jongco, Velcek FT, et al: Absorptive function of the aganglionic ileum. J Pediatr Surg 16:275-278, 1981 16. F~k6t6 CN, Rieour C, Martelli H, et al: Total colonic aganglionosis (with or without ileal involvement): A review of 27 cases. J Pediatr Surg 21:251-254, 1986 17. Burrington JD, Wayne ER: Modified Duhamel procedure for treatment of total aganglionic colon in childhood. J Pediatr Surg 11:391-398, 1976 18. Kraeft H, Holschneider AM, Hecker W Ch, et al: Follow-up studies in 50 totally colectomised children. Z Kinderchir 40:85-86, 1985 19. Kimura K, Nishijima E, Muraji T, et al: Extensive aganglionosis: Further experience with the colonic patch graft procedure and long-term results. J Pediatr Surg 23:52-56, 1988 20. Stringel G: Extensive intestinal aganglionosis including the ileum: A new surgical technique. J Pediatr Surg 21:667-670, 1986 21. Myrvold HM, Tindel MS, Isenberg HD, et al: The nipple valve as a sphincter substitute for the ileocoecal valve: Prevention of bacterial overgrowth in the small bowel. Surgery 96:42-47, 1984 22. Wilkinson L, Rodning Ch B: Valva ileocoecalis: Preservation during ileocolonic surgery employing a rodent experimental model. Am Surg 52:269-272, 1986
COLONIC aganglionosis or even small T OTAL bowel involvement is present in 5% to 15% of cases of Hirschsprung's disease according to various reports in the literature. ~-3 In the large series reported by Ikeda and Goto,3 this was true in 8.4% of 1,628 patients, with a mortality rate of 40.9%. Enterocolitis was a frequent finding (44.3%). Since the first publication4 of the Martin procedure, many attempts have been made to improve the patient's situation by preserving parts of the colon in order to maintain reabsorptive function.5s Martin himself improved his original method by performing a side-to-side anastomosis of the entire colon to the small bowel in 1982.9 Water reabsorption occurs mainly in the right colon and to a lesser degree also in the left colon, while no absorption takes place in the rectum. ~~ This would explain why Martin's original method using the left colon was not successful overall. The loss of the ileocecal region leads not only to malabsorption of vitamin B I2, fatty acids, and bile acids, but may also allow ascension of colonic bacteria into the small intestine. Therefore, the chance of survival in children with short bowel syndrome is clearly better if the ileocecal valve is preserved? As we demonstrated in three cases of total colonic aganglionosis without ileal involvement, preservation of the cecum and ileocecal valve is possible. ~2 The aganglionic cecum cannot be anastomosed to the aganglionic rectum, a problem that was solved by interposition of an ileal segment between the two parts of the colon. A protective cecostomy led to a significantly less complicated course than that in patients treated by ileostomy. The method published in 1972~3 cannot be applied to cases with ileal involvement (Fig 1). However, encouraged by the good results mentioned herein, Journal of Pediatric Surgery, Val 24, No 5 (May), 1989: pp 457-461
Fig 1. Extent of resection (left) and interposition of an ileal segment between the aganglionic ascending colon and rectum (right).
a technique was developed that allowed preservation of the ileocecal valve even in cases with ileal involvement. MATERIALS AND METHODS As in our original method, the continuity between the right colon and rectum is established by interposition of an ileal loop containing ganglion cells. The right colon remains in its normal position. Multiple frozen sections are taken from the ileum and colon to identify the extent of aganglionosis. Proximally, the entire aganglionic part of the ileum is removed and transected in an area of normal ganglion cell distribution, while distally about 2 to 3 cm of the affected prececal ileum is preserved. This remaining segment of aganglionic ileum is incised longitudinally on the antimesenteric side, almost down to the ileocecal valve. After isolating about 20 cm of normally innervated ileum for interposition, the proximal ileum is anastomosed end-to-back to the distal aganglionic ileal segment (Fig 2). Whether the anastomosis should be performed behind or in front of the isolated loop must be decided on an individual basis. A side-to-side anastomosis is then carried out between the isolated ileal loop and right colon in an isoperistaltic manner, if possible (Fig 3). However, it is imperative to orient the interposed ileal loop in order to direct peristalsis toward the rectum. To accommodate this procedure, the loop should he fixed to the cecum by stay sutures at an early phase. In cases of extensive aganglionosis, the distal aganglionic part of the ileum may be preserved and anastomosed side-to-side to the
From the Department of Paediatric Surgery, University of Graz, Austria. Date accepted: September 30, 1988. Address reprint requests to Professor Dr H. Sauer, Universit~tsklinik ff~r Kinderchirurgie, Heinrichstra[3e 31, A-8010 Graz, Austria. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2405-0010503.00/0 457
458
SAUER AND KLOS
C /
Fig 2. (A) Resection of aganglionic ileal segment. Resection line A is 2 to 3 cm proximal to the ileocecal valve in affected area, resection line s is in ganglionic area. (B) s to c: normal ileum to be used f o r interposition.
B
D
C Fig 3. Side-to-side anastomosis between ileal loop (B to c) and ascending colon. End-to-back anastomosis of ileum (A to 0).
Fig 4. Second step in staged procedure. A to D, ileo-ileal anastomosis; c to s, ileal conduit; 8, distal end of conduit as stoma. The proximal end of the distal colon is also exteriorized to facilitate later Ueorectal anastomosis.
TOTAL COLONIC AGANGLIONOSlS: ILEOCECAL VALVE
proximal ganglionic part of the ileum in order to improve reabsorption of water) 4'x5 The distal anastomosis between the ileal conduit and rectosigmoid can be performed either by using the Martin procedure or as an end-to-end anastomosis to the rectum (according to Rehbein). In the future, we suggest using the stapler after dilating the rectum to at least Hegar 22.
459
C
D
Patient Evaluation and Staging of Operations The procedure can be performed in one, two, or three stages. If there is suspicion of Hirschsprung's disease, we try to prove the diagnosis by suction biopsy and histochemical investigation as soon as possible. The operation takes place with an experienced pathologist present. When the extent of aganglionosis has been established, the remaining steps will depend on the patient's condition. In newborns or infants in poor condition, a loop ileostomy is performed in an area that contains ganglion cells, according to multiple extramucous biopsies. In order to rule out the danger of persisting enterocolitis, ~ the part of the transverse and descending colon that will not be used later is removed immediately. The second stage consists of ileo-ileal and proximal ileocolic anastomosis; the distal end of the ileal conduit is brought out as a terminal stoma in the left lower abdomen (Fig 4). If the patient is in a good condition, the operation is done as the first step, without previous ileostomy. The distal ileorectal anastomosis can be performed either as the third stage or in addition to the preceding step, depending on the patient's condition (Fig 5).
CASE REPORTS
B
Patient 1 A baby boy (born January 21, 1981) with a birth weight of 3,600 g was first seen by us at the age of 31/2 months. He had suffered from hemorrhagic diarrhea since birth, and had undergone a laparotomy elsewhere for signs of ileus, but no mechanical obstruction could be identified; the only finding was a narrow distal ileum. The child was operated on in our department at the age of 4 months; we found a narrow colon and terminal ileum, with ileal dilatation 20 cm proximal to the ileocecal valve. A rapid frozen section of this area showed marked hypoganglionosis and normal ganglion cells at 30 cm. As the first step, we removed the aganglionic part of the ileum, performed an ileo-ileal end-to-back anastomosis, and did a sideto-side anastomosis between the right colon and isolated ileal loop. One month later, the conduit was anastomosed to the rectosigmoid using the Martin procedure. During coloscopy 9 months after the last operation, the distal anastomosis was inconspicuous and so was the ileal conduit; the ileocecal valve could not be visualized. A defecation radiogram 3 years later showed a conical proximally-widening ampulla recti in the filling phase, and no relaxation of the internal sphincter. When trying to defecate, there was no coordinated relaxation of the puborectalis muscle, but in a later phase, the anal canal opened in a band-like fashion and the puborectalis relaxed. Seven years after surgery, it was evident that the patient had developed normally. He is clean during the day (with four or five stools), while at night there is some soiling from time to time. His anal sphincter is somewhat rigid, necessitating anal dilatation about twice a year.
Patient 2 A baby boy born June 15, 1985 had a birth weight of 3,200 g. The operation for this patient was also done in two stages--the first at the age of 17 days, and the second 6 months later. The rectal anastomosis was performed end-to-end, according to Rehbein. There were no
Fig 5. Procedure completed. A t o o: ileo-ileal a n a s t o m o s i s ; e: proximal closed end of ileum attached side-to-side to cecum and ascending colon; a: distal end of ileum conduit--ileorectal a n a s t o mosis.
complications, the child has developed normally, and appears to be continent. There are two bowel movements daily. A contrast enema performed 3 months after the second operation showed a short rectal cuff with a normal caliber, while the caliber of the interposed intestine was changing, but never exceeded that of the rectum. No reflux beyond the ileocecal valve could be demonstrated (Fig 6).
DISCUSSION Reports about the varying operative procedures that have been applied to treat total colonic aganglionosis a r e c o n t r o v e r s i a l . C a s s a n d M y e r s ~ b e l i e v e t h e r e is n o n e e d to p r e s e r v e p a r t o f t h e a g a n g l i o n i c c o l o n ; t h e y a p p e a r to a p p l y t h e M a r t i n m e t h o d o n l y i f w a t e r y i l e o s t o m y stools p e r s i s t f o r > 2 4 m o n t h s . F 6 k 6 t 6 e t al, 16 despite using this procedure regularly, believe that
460
SAUER AND KLOS
Fig 6. Consecutive phases of contrast study. (A) Site of ileorectal anastomosis (arrow). (B) Ileocecal pouch; no stanosis at site of ileorectal anastomosis (arrows). (C) Normal motility of ileum; no dilatation of proximal loops.
TOTAL COLONIC AGANGLIONOSIS: /LEOCECAL VALVE
461
" t h e best t r e a t m e n t is p r o b a b l y a low-pressure pouch system ensured by a low-placed wide a n d supple ileoa n a l - a n a s t o m o s i s . " In one of their patients, after the M a r t i n procedure, it was necessary to remove the colon and p e r f o r m an ileo-anal anastomosis. W e a r e convinced t h a t use of the left colon is responsible for the u n f a v o r a b l e results in some o f the cases. Good results a r e r e p o r t e d by o t h e r investigators, 2'~7 and by M a r t i n himself. However, being dissatisfied with his early results, Martingnow prefers to preserve the whole colon. In a c o m p a r a t i v e study on 50 colect o m i z e d children, K r a e f t et al is were u n a b l e to demons t r a t e a n y a d v a n t a g e o f one operative p r o c e d u r e over another. T h e n u m b e r of patients t r e a t e d by surgical m e t h o d s described by Boley, 6 K i m u r a et al, 7'19 and K o t t m e i e r et al Js is still too small for j u d g e m e n t ,
O n l y Stringel 2~ has used a side-to-side anastomosis between the ileum and colon t h r o u g h the valva ileocecalls (in one case; 1986). To date, no other proposed surgical m e t h o d s have d r a w n attention to the ileocecal valve and its specific role in protecting the ileum, a p r o p e r t y t h a t is well d o c u m e n t e d clinically and experimentally, n'22'22 The ileocecal valve acts as a physical b a r r i e r a g a i n s t reflux and ascension o f colonic bacterial flora into the small bowel; thus, it helps alleviate the absorption of bile salts and f a t t y acids. In neither of our two clinical cases could reflux o f c o n t r a s t m a t e r i a l into the ileum be d e m o n s t r a t e d . A l t h o u g h no f u r t h e r conclusions should be d r a w n from only two cases, we believe t h a t the a p p a r e n t a d v a n t a g e of a functioning ileocecal valve should be considered in the t r e a t m e n t of total colonic aganglionosis.
REFERENCES
1. Cass DT, Myers N: Total colonic aganglionosis: 30 years' experience. Pediatr Surg Int 2:68-75, 1987 2. Ikeda K, Goto S: Total colonic aganglionosis with or without small bowel involvement: An analysis of 137 patients. J Pediatr Surg 21:319-322, 1986 3. Ikeda K, Goto S: Diagnosis and treatment of Hirschsprung's disease in Japan. Ann Surg 199:400-405, 1984 4. Martin LW: Surgical management of Hirschsprung's disease involving the small intestine. Arch Surg 97:183-189, 1968 5. Applebaum H, Richardson R J, Wilkinson GA, et al: Alternative procedure for total colonic aganglionosis. J Pediatr Surg 23:4951, 1988 6. Boley SJ: A new operative approach to total aganglionosis of the colon. Surg Gynecol Obstet 159:481-484, 1984 7. Kimura K, Nishijima E, Muraji T, et al: A new surgical approach to extensive aganglionosis. J Pediatr Surg 16:840-843, 1981 8. Shandling B: Total colon aganglionosis--A new operation. J Pediatr Surg 19:503-505, 1984 9. Martin LW: Total colonic aganglionosis preservation and utilization of entire colon. J Pediatr Surg 17:635-637, 1982 10. Devroede GJ, Philipps SF: Failure of the human rectum to absorb electrolytes and water. Gut 11:438-442, 1970 11. Goto S, Gunter M, Scherer LR, et al: Surgical treatment of total colonic aganglionosis: Efficacy of aganglionic patch enteroplasty in the rat. J Pediatr Surg 21:601-607, 1986 12. Wilmore WD: Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 80:88-95, 1972
13. Saucr H: Diinndarmzwischenschaltung nach subtotaler Colektomie wegen Mb. Hirschsprung im Kindesalter. Chirurg 43:280-282, 1972 14. Schennach W, Sauer H, Menardi G: Die totale Aganglionose des Dickdarmes. Z Kinderchir 23:33-34, 1978 15. Kottmeier PK, Jongco, Velcek FT, et al: Absorptive function of the aganglionic ileum. J Pediatr Surg 16:275-278, 1981 16. F~k6t6 CN, Rieour C, Martelli H, et al: Total colonic aganglionosis (with or without ileal involvement): A review of 27 cases. J Pediatr Surg 21:251-254, 1986 17. Burrington JD, Wayne ER: Modified Duhamel procedure for treatment of total aganglionic colon in childhood. J Pediatr Surg 11:391-398, 1976 18. Kraeft H, Holschneider AM, Hecker W Ch, et al: Follow-up studies in 50 totally colectomised children. Z Kinderchir 40:85-86, 1985 19. Kimura K, Nishijima E, Muraji T, et al: Extensive aganglionosis: Further experience with the colonic patch graft procedure and long-term results. J Pediatr Surg 23:52-56, 1988 20. Stringel G: Extensive intestinal aganglionosis including the ileum: A new surgical technique. J Pediatr Surg 21:667-670, 1986 21. Myrvold HM, Tindel MS, Isenberg HD, et al: The nipple valve as a sphincter substitute for the ileocoecal valve: Prevention of bacterial overgrowth in the small bowel. Surgery 96:42-47, 1984 22. Wilkinson L, Rodning Ch B: Valva ileocoecalis: Preservation during ileocolonic surgery employing a rodent experimental model. Am Surg 52:269-272, 1986