Pyloric stenosis: unusual presentations and rare assoclations.

Pyloric stenosis is a common paediatric surgical condition with a well-recognised pattern of clinical features. Diagnosis is usually straightforward and a Ramstedt pyloromyotomy is curative. We present three patients who developed pyloric stenosis incidentally during the management of other primary surgical conditions. Issues of diagnostic difficulty following presentation of pyloric stenosis in the post-operative period (all three patients), its rare syndromic associations (one patient) and its management in this unusual situation are discussed. An awareness of the unusual presentations of pyloric stenosis during treatment for other primary surgical conditions can help in earlier diagnosis and treatment.

Chromosomal translocation in mesenchymal hamartoma of liver: what is its significance?

Mesenchymal hamartoma of the liver is generally considered to be benign in nature, and surgical excision has been the treatment of choice. There have been 3 reports on the association of cytogenetic abnormalities with this tumor, and the possibility of malignant potential has been raised. The authors report a fourth case with a previously unreported complex chromosomal translocation between chromosomes 11, 17, and 19 at bands q12, p11, and q13.3, respectively in all cells examined and discuss the significance of this finding.

Congenital cystic mesoblastic nephroma.

With widespread use of antenatal ultrasound, an increasing number of urinary-tract lesions are being detected, and the clinician is called upon to make management decisions. Parenchymal lesions of the kidney pose a special challenge because of the associated risk of the occurrence of malignancy. A case of an antenatally-detected solid/cystic lesion of the kidney diagnosed as a congenital cystic mesoblastic nephroma following nephrectomy is presented and the issues involved in therapeutic decision-making are discussed.

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.

Perianal abscess in childhood.

Perianal abscess (PA) is a common condition encountered in childhood, but its optimal primary treatment is uncertain. Treatment of PA by incision and drainage (I & D) alone is associated with an unacceptably high recurrence rate, either as fistula-in-ano (FIA) or as PA. To identify possible causes of recurrence and assess the value of concomitant laying open of a fistulous tract at the time of primary incision and drainage, the case notes of all children who presented to our institution with a PA between January 1992 and January 1997 were reviewed retrospectively. Thirty-three cases were identified (29 boys and 4 girls). A fistulous tract was identified and laid open at the time of primary drainage in 20 cases, whilst 13 were treated by I & D alone. Following primary drainage, there were 7 recurrences (21.2%) (FIA 5 and PA 2). All recurrences had been primarily treated by drainage alone, whilst there were no recurrences in patients who had also undergone fistulotomy at the time of primary drainage. Thus the primary treatment of PA in childhood should involve a careful search for a coexisting fistula and treatment of this by fistulotomy.