Total Colonic Aganglonosis with Small Bowel Involvement How much is enough? Mike Florack R1

A clinical scenario - BPC 1 day old 38 1/7 WGA Male presents with billous emesis Born to 18y/o G1P1 Female, all prenatal care in El Salvador Apgar scores 8, 9 Birthweight 3.18Kg No passage of meconium AF VSS WNL Abdomen moderately distended, soft WBC 18 Bands 22 Repogle Decompression in NICU and plan for further imaging

Surgical Pathology – 11/4 Ex-lap Frank perforation of jejunum, resected 50cm proximal to ileocecal valve, SB distal to this filled with inspissated meconium. Abdomen/bowel irrigated and anastamosed Bowel also noted to be dilated/volvulized from 90cm prox to ICV until 50cm from LOT Path Report: Total aganglionosis of resected segment and both SB margins

What operation would you perform? How many stages? Which procedure? What margins on the bowel? Swenson Duhamel Martin Combined open reservoir J-pouch Z-anastamosis Soave Right Colon Patch (Kimura/Boley/Rebhein)

Image from: Elsiver Medical DIctionary

Procedure Types Main choices: 1 vs 2 vs 3 stage procedure Procedure controversy Some suggestion that large reservoir procedures are more likely to predispose to Recurrent Enterocolitis Symptoms of SBO, constipation nausea/vomiting Rarity of disease fails to have power. NO prospective studies 2008, 2012 metanalyses Spanish trial of 232 shows no difference between swenson and mod. martin

One vs Two Stage procedure Image: Pediatric Surgery international: http://link.springer.com.offcampus.lib.washington.edu/article/10.1007/s00383-011- 2960-y/fulltext.html

Complications of Neurogenic Bowel Rat models 40 subject retrospective studies Some evidence of decreased ostomy output and increased ability to gain weight Transition Zone/Residual Aganglionosis causative for recurrent symptoms in Hirschprung’s disease

So take all the aganglionic cells Direct ileoanal anstamosis impacts Lifestyle Nutrition Spectrum of Disease <ICV = Hirschsprung Disease ICV->30/50cm = Total Colonic Aganglionosis >30/50cm from ICV = Extensive Aganglionosis

Short Gut Syndrome Long Term deficiencies in TCA patients with SB involvement B12/Iron Poor Overall growth Morbidity correlates with amount of ileal involvement ~50-75cm from Ligament of Tritz cutoff Case series in attempting to leave neurogenic bowel to mid ileum OneFrench study found 50 as prognostic for 26 patients regarding need for nutritional support or not Japanese Study took f/u a step further following 7 patients for 10-26 years, requiring aggressive supplementation 3/15 dutch case series undergoing duhamel with involement to midileum and aganglionosis at proximal margin, all three with nl bowel function no soilage, but one dead of unknown causeas (possible enterocolitis vs. SIDS) Netherlands 2006, 2008, 2010, 1 episode of enterocolitics, distend bowel on xray, no distension sx

Extent of aganglionosis and mortality II III All patients 7.10% 4.90% 3.00% Colonic aganglionosis 4.10% 3.20% 1.70% TCSA 40.90% 21.50% 15.80% TCA 30.40% 8.00% EA 53.60% 33.30% 35.50% <75 cm from Treitz's band 70.60% 59.10% 83.30% >75 cm from Treitz's band 32.40% 10.70% 25.00% Large Japanese Census 4 year windows, approx 120 pts per group, 78-82, 88-92, 98-2002

Conclusions Use two-stage procedure for TCA Use the procedure you know Wait till potty-trained/toleration of rectal irrigation Use the procedure you know Question of how much bowel remains unresolved Close monitoring for <75cm of SB from the LOT

BPC Outcome POD 20 ex-lap, end jejunosotomy ~50CM from LOT, end of dilated SB tapered to form stoma, with normal ganglion cells at margin. Long MF connected to LLQ. Stamm Gastrostomy. Monitoring on TPN, Receiving G-tube feeds at low rate Rectal Irrigations Problems with both ostomies and erythema, will likely need revision

References Bischoff, A., M. Levitt and A. Peña (2011). "Total colonic aganglionosis: a surgical challenge. How to avoid complications?" Pediatric Surgery International 27(10): 1047-1052. Fouquet, V., P. De Lagausie, C. Faure, J. Bloch, S. Malbezin, L. Ferkhadji, C. Bauman and Y. Aigrain (2002). "Do prognostic factors exist for total colonic aganglionosis with ileal involvement?" Journal of Pediatric Surgery 37(1): 71-75. Friedmacher, F. and P. Puri (2011). "Residual aganglionosis after pull-through operation for Hirschsprung’s disease: a systematic review and meta-analysis." Pediatric Surgery International 27(10): 1053-1057. Goto, S. and J. L. Grosfeld (1987). "Is preserving the entire aganglionic colon reasonable in the surgicaltreatment of total colonic aganglionosis?" Journal of Pediatric Surgery 22(7): 623-627. Ieiri, S., S. Suita, T. Nakatsuji, J. Akiyoshi and T. Taguchi (2008). "Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan." Journal of Pediatric Surgery 43(12): 2226-2230. Ikawa, H., H. Masuyama, T. Hirabayashi, M. Endo and J. Yokoyama (1997). "More than 10 years' follow- up of total colonic aganglionosis-severe iron deficiency anemia and growth retardation." Journal of Pediatric Surgery 32(1): 25-27.

References (cont) Ishii, K., T. Doi, K. Inoue, M. Okawada, G. Lane, A. Yamataka and C. Akazawa (2012). "Correlation between multiple RET mutations and severity of Hirschsprung’s disease." Pediatric Surgery International: 1-7. Laughlin, D., F. Friedmacher and P. Puri (2012). "Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome." Pediatric Surgery International 28(8): 773-779. Marquez, T. T., R. D. Acton, D. J. Hess, S. Duval and D. A. Saltzman (2009). "Comprehensive review of procedures for total colonic aganglionosis." Journal of pediatric surgery 44(1): 257-265. Moore, S. W. and M. Zaahl (2009). "Clinical and genetic differences in total colonic aganglionosis in Hirschsprung's disease." Journal of pediatric surgery 44(10): 1899- 1903. Travassos, D. V. and D. C. van der Zee (2011). "Is complete resection of the aganglionic bowel in extensive total aganglionosis up to the middle ileum always necessary?" Journal of Pediatric Surgery 46(11): 2054-2059.

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