Preoperative enterocolitis is associated with poorer long-term bowel function after Soave-Boley endorectal pull-through for Hirschsprung's disease.

BACKGROUND/PURPOSE: The purpose of the investigation was to apply a semiquantitative scoring system for bowel function to patients who had undergone endorectal pull-through (ERPT) for Hirschsprung's disease (HD) and to use this to analyse the clinical factors relating to functional outcome. METHODS: The case note details of 63 patients undergoing ERPT for HD were reviewed. A questionnaire using a semiquantitative scoring system for 6 aspects of bowel function was sent to parents of 55 patients. The total functional score (TFS) for each patient was calculated, and a statistical analysis was performed to determine which clinical factors were significantly related to bowel functional outcome. RESULTS: Fifty (91%) of the parents returned the questionnaire. TFS was "good" in 45%, "fair" in 33% and "poor" in 22%. There was a statistically significant difference in the scores of those who had preoperative Hirschsprung's associated enterocolitis (HAEC) TFS = 8.0 and those who did not, TFS 12.7 (P <.01) The sex of the patient, length of aganglionic segment, timing of ERPT (early/late), staging of ERPT (1 or 2), presence/absence of anastomotic stricture, and presence or absence of Down's syndrome did not statistically significantly affect TFS. CONCLUSIONS: Preoperative HAEC was the most important factor in relation to functional outcome after ERPT for HD. The reason remains unclear.

Twins with oesophageal atresia and the CHARGE association.

Twins are rarely concordant for oesophageal atresia and tracheo-oesophageal fistula. Such a case is presented and their similar associated anomalies described. The CHARGE association is considered as a possible diagnosis and the aetiological implications considered.