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A technique for preservation of the ileocecal valve in the neonatal short intestine
A Technique for Preservation of the I l e o c e c a l Valve in the N e o n a t a l Short Intestine By Ann M. Kosloske and Patrick F. Jewell Albuquerque, N e w Mexico 9 W e report a technique, appendiceal interposition, w h i c h permitted preservation of the ileocecal valve in an infant w i t h a congenitally short intestine (jejunum, 12 cm; ileum, 1 cm). The p r o c e d u r e was p e r f o r m e d on the first day of life in conjunction w i t h jejunal lengthening by the Bianchi technique. The result w a s a small intestine o f 21 cm in length w i t h an intact Ueocecal valve. 9 1 9 8 9 by Grune & S t r a t t o n . Inc. I N D E X W O R D S : S h o r t gut syndrome: Bianchi procedure: intestinal lengthening,
H E P R E S E N C E OF an intact ileocecal valve may be crucial to survival in infants who have sustained a massive loss of intestine in the newborn period. We report a new technique, appendiceal interposition, for preservation of the ileocecal valve in an infant with congenitally short intestine.
T
CASE REPORT
A full-term baby boy (birth weight, 2.5 kg) was explored at 23 hours of age. Jejunal atresia was found with the jejunum ending in a large blind bulb 12 cm distal to the ligament of Treitz. The
A
remaining small intestine was almost completely dissolved within a mass of congenital adhesions containing many calcifications from prenatal midgut volvulus. Most of the small intestinal mesentery was missing. The distal ileum was a thin 1-cm stump above the ileocecal valve. There was a long appendix and a small but unremarkable colon. The necrotic portion of the small intestine was removed and a Bianchi intestinal lengthening procedure ~was performed. The jejunum was split lengthwise with the needle cautery on both the antimesenteric and the mesenteric borders. The leaves of the mesentery were gently separated. The two flaps of bowel were fashioned into two narrower tubes over 12-F catheters, and anastomosed end-to-end isoperistaltically. The distal end was brought out as a tube jejunostomy. A tube gastrostomy was also performed. Appendieeal interposition was carried out in order to preserve the ileoceal valve. Any standard anastomosis or enterostomy would have sacrificed the valve. The appendix was divided at its base and the cecum closed. The ileal stump was opened at its proximal end, and an end-to-end anastomosis was carried out between the base of the appendix and the ileum (Fig 1A) using 5-0 and 6-0 silk sutures. The tip of the appendix was exteriorized adjacent to the tube j ejunostomy (Fig 1B). The entire operation was performed using a magnification of • The patient was sustained on central total parenteral nutrition (TPN). At 3 weeks of age, gastrostomy drip feedings of dilute elemental formula were begun. He developed early progressive cholestatic jaundice. At 9 weeks of age, a Bishop-Keep anastomosis was performed between the jejunum and the appendix (Fig 1C). Barium transit time (stomach to rectum) was ten minutes at three months of age and 20 minutes at 9 months of age. Although minimal enteral feedings were tolerated, he could never be weaned from TPN. Jaundice and hepatic failure were profound, and he died at 10 months of age following several episodes of massive upper gastrointestinal hemorrhage. DISCUSSION
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APPENDICOSTOMy &
+ APPENDtCOSTOMY
Fig 1. The appendiceal interposition procedure for preservation of the ileocecal valve, (A) Appendix divided at base, preserving its blood supply, and anastomosed end-to-end with a 1-cm stump of ileum. (B) Appendiceal tip and jejunostomy tube exteriorized. Bianchi jejunal lengthening has been completed, (C) Subsequent Bishop-Keep anastomosis, jejunum to appendix.
Journal of PediatricSurgery, Vol 24, No 4 (April), 1989: pp 369-370
I n 1972, in a n e x t e n s i v e r e v i e w o f o u t c o m e f o l l o w i n g massive intestinal resection in the neonatal period, Wilmore 2 concluded that among infants <2 months of age at the time of operation, survival was highly likely if the remaining segment of small intestine was >40 c m in l e n g t h , h i g h l y u n l i k e l y if it w a s < 1 5 c m in l e n g t h , a n d p o s s i b l e if 15 t o 4 0 c m in l e n g t h , if a n i n t a c t ileocecal valve remained. Although the absolute minim a l l e n g t h for s u r v i v a l is n o w k n o w n to b e < 4 0 c m ,
From the Departments of Surgery and Pediatrics, University of New Mexico School of Medicine, Albuquerque. Presented at the 21st Annual Meeting of the Pacific Association of Pediatric Surgeons, Taipei, Taiwan, ROC, April 11-15, 1988. Address reprint requests to Ann M. Kosloske, MD, Department of Surgery, Room 233, University of New Mexico Hospital, 2211 Lomas Blvd NE, Albuquerque, N M 87106. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2404-0011503.00/0 369
370
KOSLOSKE AND JEWELL
a n e c d o t a l survivors who u l t i m a t e l y had n o r m a l growth without p a r e n t e r a l nutrition, with as little as 11 to 16 c m of s m a l l intestine, were all infants with an intact ileocecal valve. 3'4 T h e short bowel s y n d r o m e of infancy has recently been reviewed. 46 In our infant, whose t e r m i n a l ileum was a tiny 1-cm stump, preservation of the valve was m a d e possible by a p p e n d i c e a l interposition. A s t a n d a r d operation, either a n a s t o m s i s or ileostomy, would have sacrificed the valve. Preservation of the valve m i g h t have p e r m i t t e d survival of our infant, whose j e j u n u m was lengthened from 12 to 20 cm, if he had not developed u n r e m i t t i n g h e p a t i c failure. T h e j e j u n a l lengthening procedure, described by Bianchi 1 in 1980, involves longitudinal splitting of the
d i l a t e d thickened p r o x i m a l bowel. It is based on the a r r a n g e m e n t of the blood vessels of the small bowel that divide into a n t e r i o r a n d posterior branches. T h e split gut is reconstructed into two n a r r o w e r tubes which, by bending, can be a n a s t o m o s e d isoperistaltically, doubling the original length. B o e c k m a n a n d T r a y l o r 7 r e p o r t e d the first successful clinical use in 1981. In a review by Bianchi 8 of the four clinical cases in which the p r o c e d u r e was used, the infants' ages r a n g e d from 3 weeks to 4 years. O u r p a t i e n t m a y be the youngest infant t r e a t e d in this fashion. T h e procedure was technically feasible in the dilated a n d thickened intestine of j e j u n a l atresia, even on the first d a y of life.
REFERENCES
1. Bianchi A: Intestinal loop lengthening--A technique for increasing small intestinal length. J Pediatr Surg 15:145-151, 1980 2. Wilmore DW: Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 80:80-95, 1972 3. Bell M J, Martin LW, Schubert WK, et al: Massive smallbowel resection in an infant: Long-term management and intestinal adaptation. J Pediatr Surg 8:197-204, 1973 4. Dorney SFA, Ament ME, Berquist WE, et al: Improved survival in very short small bowel of infancy with use of long-term parenteral nutrition. J Pediatr 107:521-525, 1985
5. Cooper A, Floyd TF, Ross AJ III, et al: Morbidity and mortality of short-bowel syndrome acquired in infancy: An update. J Pediatr Surg 19:711-718, 1984 6. Grosfeld JL, Rescorla FJ, West KW: Short bowel syndrome in infancy and childhood. Am J Surg 151:41-46, 1986 7. Boeckman CR, Traylor R: Bowel lengthening for short gut syndrome. J Pediatr Surg 16:996-997, 1981 8. Bianchi A: Intestinal lengthening: An experimental and clinical review. J R Soc Med 77:35-41, 1984
H E P R E S E N C E OF an intact ileocecal valve may be crucial to survival in infants who have sustained a massive loss of intestine in the newborn period. We report a new technique, appendiceal interposition, for preservation of the ileocecal valve in an infant with congenitally short intestine.
T
CASE REPORT
A full-term baby boy (birth weight, 2.5 kg) was explored at 23 hours of age. Jejunal atresia was found with the jejunum ending in a large blind bulb 12 cm distal to the ligament of Treitz. The
A
remaining small intestine was almost completely dissolved within a mass of congenital adhesions containing many calcifications from prenatal midgut volvulus. Most of the small intestinal mesentery was missing. The distal ileum was a thin 1-cm stump above the ileocecal valve. There was a long appendix and a small but unremarkable colon. The necrotic portion of the small intestine was removed and a Bianchi intestinal lengthening procedure ~was performed. The jejunum was split lengthwise with the needle cautery on both the antimesenteric and the mesenteric borders. The leaves of the mesentery were gently separated. The two flaps of bowel were fashioned into two narrower tubes over 12-F catheters, and anastomosed end-to-end isoperistaltically. The distal end was brought out as a tube jejunostomy. A tube gastrostomy was also performed. Appendieeal interposition was carried out in order to preserve the ileoceal valve. Any standard anastomosis or enterostomy would have sacrificed the valve. The appendix was divided at its base and the cecum closed. The ileal stump was opened at its proximal end, and an end-to-end anastomosis was carried out between the base of the appendix and the ileum (Fig 1A) using 5-0 and 6-0 silk sutures. The tip of the appendix was exteriorized adjacent to the tube j ejunostomy (Fig 1B). The entire operation was performed using a magnification of • The patient was sustained on central total parenteral nutrition (TPN). At 3 weeks of age, gastrostomy drip feedings of dilute elemental formula were begun. He developed early progressive cholestatic jaundice. At 9 weeks of age, a Bishop-Keep anastomosis was performed between the jejunum and the appendix (Fig 1C). Barium transit time (stomach to rectum) was ten minutes at three months of age and 20 minutes at 9 months of age. Although minimal enteral feedings were tolerated, he could never be weaned from TPN. Jaundice and hepatic failure were profound, and he died at 10 months of age following several episodes of massive upper gastrointestinal hemorrhage. DISCUSSION
\
\
C \, ~,~7:~f
APPENDICOSTOMy &
+ APPENDtCOSTOMY
Fig 1. The appendiceal interposition procedure for preservation of the ileocecal valve, (A) Appendix divided at base, preserving its blood supply, and anastomosed end-to-end with a 1-cm stump of ileum. (B) Appendiceal tip and jejunostomy tube exteriorized. Bianchi jejunal lengthening has been completed, (C) Subsequent Bishop-Keep anastomosis, jejunum to appendix.
Journal of PediatricSurgery, Vol 24, No 4 (April), 1989: pp 369-370
I n 1972, in a n e x t e n s i v e r e v i e w o f o u t c o m e f o l l o w i n g massive intestinal resection in the neonatal period, Wilmore 2 concluded that among infants <2 months of age at the time of operation, survival was highly likely if the remaining segment of small intestine was >40 c m in l e n g t h , h i g h l y u n l i k e l y if it w a s < 1 5 c m in l e n g t h , a n d p o s s i b l e if 15 t o 4 0 c m in l e n g t h , if a n i n t a c t ileocecal valve remained. Although the absolute minim a l l e n g t h for s u r v i v a l is n o w k n o w n to b e < 4 0 c m ,
From the Departments of Surgery and Pediatrics, University of New Mexico School of Medicine, Albuquerque. Presented at the 21st Annual Meeting of the Pacific Association of Pediatric Surgeons, Taipei, Taiwan, ROC, April 11-15, 1988. Address reprint requests to Ann M. Kosloske, MD, Department of Surgery, Room 233, University of New Mexico Hospital, 2211 Lomas Blvd NE, Albuquerque, N M 87106. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2404-0011503.00/0 369
370
KOSLOSKE AND JEWELL
a n e c d o t a l survivors who u l t i m a t e l y had n o r m a l growth without p a r e n t e r a l nutrition, with as little as 11 to 16 c m of s m a l l intestine, were all infants with an intact ileocecal valve. 3'4 T h e short bowel s y n d r o m e of infancy has recently been reviewed. 46 In our infant, whose t e r m i n a l ileum was a tiny 1-cm stump, preservation of the valve was m a d e possible by a p p e n d i c e a l interposition. A s t a n d a r d operation, either a n a s t o m s i s or ileostomy, would have sacrificed the valve. Preservation of the valve m i g h t have p e r m i t t e d survival of our infant, whose j e j u n u m was lengthened from 12 to 20 cm, if he had not developed u n r e m i t t i n g h e p a t i c failure. T h e j e j u n a l lengthening procedure, described by Bianchi 1 in 1980, involves longitudinal splitting of the
d i l a t e d thickened p r o x i m a l bowel. It is based on the a r r a n g e m e n t of the blood vessels of the small bowel that divide into a n t e r i o r a n d posterior branches. T h e split gut is reconstructed into two n a r r o w e r tubes which, by bending, can be a n a s t o m o s e d isoperistaltically, doubling the original length. B o e c k m a n a n d T r a y l o r 7 r e p o r t e d the first successful clinical use in 1981. In a review by Bianchi 8 of the four clinical cases in which the p r o c e d u r e was used, the infants' ages r a n g e d from 3 weeks to 4 years. O u r p a t i e n t m a y be the youngest infant t r e a t e d in this fashion. T h e procedure was technically feasible in the dilated a n d thickened intestine of j e j u n a l atresia, even on the first d a y of life.
REFERENCES
1. Bianchi A: Intestinal loop lengthening--A technique for increasing small intestinal length. J Pediatr Surg 15:145-151, 1980 2. Wilmore DW: Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 80:80-95, 1972 3. Bell M J, Martin LW, Schubert WK, et al: Massive smallbowel resection in an infant: Long-term management and intestinal adaptation. J Pediatr Surg 8:197-204, 1973 4. Dorney SFA, Ament ME, Berquist WE, et al: Improved survival in very short small bowel of infancy with use of long-term parenteral nutrition. J Pediatr 107:521-525, 1985
5. Cooper A, Floyd TF, Ross AJ III, et al: Morbidity and mortality of short-bowel syndrome acquired in infancy: An update. J Pediatr Surg 19:711-718, 1984 6. Grosfeld JL, Rescorla FJ, West KW: Short bowel syndrome in infancy and childhood. Am J Surg 151:41-46, 1986 7. Boeckman CR, Traylor R: Bowel lengthening for short gut syndrome. J Pediatr Surg 16:996-997, 1981 8. Bianchi A: Intestinal lengthening: An experimental and clinical review. J R Soc Med 77:35-41, 1984