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Do prognostic factors exist for total colonic aganglionosis with ileal involvement?
V Fouquet1, P De Lagausie, C Faure
1Services de chirurgie Viscérale, de Gastro-entérologie, de Santé Publique, d'anesthésie, d'Anatomopathologie, de Génétique, Hôpital Robert Debre, Paris, France.
Background/Purpose:
Total colonic aganglionosis with ileal involvement is estimated at 1 case in 50,000 living births. This pathology has a very variable prognosis, and patients often need long-term parenteral nutrition. The aim of this study is to define prognostic factors for this disease.
Methods:
This is a retrospective study from 1980 to 1999, based on 26 cases of total colonic aganglionosis with ileal involvement. The authors analyzed birth term, sex, birth weight, ileal involvement in centimeters, delay to correct level enterostomy, the total parenteral nutrition duration, and the need for constant rate nutritional assistance. The authors studied the following items: Weight, height, complications, clinical state, and nutritional issues. The statistic test is: LOG RANK (analysis of censored data and comparison of survival diagram).
Results:
The only prognostic factor is the length of ileal involvement. All the children with ileal involvement less than 50 cm, except for 2, did not need long-term nutritional assistance; for those with ileal resection over 50 cm, long-term nutritional assistance was needed. Total colonic aganglionosis is a very serious illness (2 children had a small bowel transplantation, and 2 are waiting for one). The prognosis is even worse when associated with a polymalformation syndrome (50% of the children died).
Conclusion:
The major prognostic factor is the length of small bowel not involved in the total colonic aganglionosis.