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

2. 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

4. 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

5. 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)

6. Image from: Elsiver Medical DIctionary

7. 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

8. One vs Two Stage procedure
Image: Pediatric Surgery international: y/fulltext.html

9. 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

10. 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

11. 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 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

12. Extent of aganglionosis and mortalityII
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,

13. 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

14. 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

15. References
Bischoff, A., M. Levitt and A. Peña (2011). "Total colonic aganglionosis: a surgical challenge. How to avoid complications?" Pediatric Surgery International 27(10):
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):
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):
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):
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):
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):

16. 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):
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):
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):
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):

17. Thank You