History of the Pan African Paediatric Surgery Association (PAPSA).

The Pan African Paediatric Surgery Association (PAPSA) was formed in 1994. The need for an organisation in Africa to voice children's surgery and the trials and tribulations in forming this organisation was covered in this journal 2 years ago (Heinz R, Kyambi J, Lakhoo K. Surg Int 34(5):499-504, 2018). This article covers the history of the organisation post inception in 1994 to date. The near disbanding of the organisation due to political unrest and wars in Africa, to its success in the recent decade is highlighted in this manuscript.

Infants with radiologic diagnosis of gastric volvulus: are they over-treated?

Gastric volvulus (GV) is a rare condition in infants. The aim of this study was to define the management strategies of infants with GV based on their clinical and radiologic features. The medical records of 13 infants with a radiologically confirmed diagnosis of GV were retrospectively reviewed. Patients were divided into two groups according to the type of treatment (surgical vs conservative). Abdominal radiographs and upper gastrointestinal contrast studies allowed an unequivocal diagnosis in both groups. Group 1 included 3 infants with acute GV and 2 with chronic, intermittent secondary GV. Three patients had associated diaphragmatic defects, 1 had an ileocolic intussusception, and 1 had hypertrophic pyloric stenosis. The main presenting symptoms were vomiting, dehydration, respiratory distress, and abdominal pain and distention in acute cases and vomiting and failure to thrive in chronic cases. A laparotomy was required in all 5 infants with no recurrence of symptoms. Group 2 included 8 infants with idiopathic chronic GV, who were managed nonoperatively with gradual improvement of symptoms over 12 months. Based on our study, we conclude that: (1) laparotomy can be reserved for patients with either acute or chronic secondary GV; (2) conservative treatment is both safe and effective in infants with chronic idiopathic GV; and (3) routine gastropexy for all patients with a radiologic diagnosis of GV appears to be overtreatment.

Long-term follow-up of redo pull-through procedures for Hirschsprung's disease: efficacy of the endorectal pull-through.

BACKGROUND/PURPOSE: The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome. METHODS: From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-through at another hospital and 2 patients with an unsuccessful pull-through from C.S. Mott. All redo pull-throughs (n = 19) were performed in these patients, and their clinical courses are reviewed. RESULTS: Twelve patients required reoperation secondary to a mechanical problem with their first pull-through. The other 7 patients had evidence of residual segments of dilated colon leading to functional failure of their initial operation including 5 patients with documented aganglionic bowel present at the second pull-through. Ten of the patients requiring reoperation initially had an endorectal pull-through (ERPT), 5 had a Duhamel procedure, 3 had a Swenson procedure, and 1 had a Rehbein procedure. Choice of revision was an ERPT in 8 patients in whom an adequate rectal cuff could be developed. Additional redo procedures included a Duhamel in 8 patients and a Swenson in 3 patients. Follow-up ranges from 3 months to 23 years (mean, 13.8 years). There were no deaths in the series, and 1 patient required a third pull-through. All patients who are not neurologically impaired and are over age 3 are continent except one (94%). Stools per day range from 1 to 10 (mean, 3.2). CONCLUSIONS: Redo pull-through operations for Hirschsprung's disease appear to be as effective as primary procedures in terms of continence and stooling frequency. Distinct from other series, we found an ERPT to be the procedure of choice if an adequate rectal cuff was present.

Tube enterostomy in the management of intestinal atresia.

A simple technique was used successfully for retraining maximum bowel length in a premature baby born with type 3A jejunal atresia. Primary end-to-end anastomosis of the tip of the dilated proximal segment to the remaining viable distal 5 cm of ileum was performed. A tube passed via the cecum proximally into the small bowel acted as a stent for the anastomosis and decompression of the bowel contents in the proximal dilated segment. This simple method may be a viable option to avoid resection of the dilated segment when the bowel length is marginal.

Enterocolitis associated with Hirschsprung's disease: a clinical histopathological correlative study.

Enterocolitis associated with Hirschsprung's disease (HEC) remains a major source of morbidity and even mortality, both before and after definitive surgical treatment. This study was undertaken to investigate whether histopathologic mucosal changes, in the absence of clinical manifestations of HEC, could predict the subsequent development of this complication. The clinical data and histopathology of 25 patients who eventually developed clinical HEC were compared with a control group of 25 age- and sex-matched patients with Hirschsprung's disease (HD) but with no clinical HEC either preoperatively or postoperatively. The histopathologic findings of tissue obtained by rectal biopsy or during laparotomy were graded from 0 to V according to severity and compared with the eventual clinical course. This study showed that (1) histopathologic findings of HEC appear to predict the clinical development, but not the severity, of future episodes of clinical HEC; (2) a histological grade of > or = II (> or = 2 crypt abscesses per high power field) should raise suspicion for subsequent occurrence of HEC, whereas a grade of > or = III (multiple crypt abscesses) places a child at high risk for development of clinical HEC; and (3) contrary to what is generally thought, histopathologic changes of HEC occur in both ganglionic and aganglionic segments. Based on these findings, it is recommended that histopathologic documentation of HEC and its grade should be an integral part of the tissue diagnosis of HD.

Enterocolitis associated with Hirschsprung's disease: a clinical-radiological characterization based on 168 patients.

The enterocolitis associated with Hirschsprung's disease (HD) has not been clearly characterized. This study was undertaken to analyze the clinical and radiological findings of Hirschsprung's enterocolitis (HEC) in 168 patients treated from July 1974 through October 1992. HEC occurred in 57 patients (33.9%), either preoperatively (13; 7.7%) or postoperatively (36; 21.4%). In eight patients (4.8%), it occurred pre- and postoperatively. The number of bouts of HEC per patient ranged from one to six (mean, 2.2). The major presenting features were abdominal distension (83%), explosive diarrhea (69%), vomiting (51%), fever (34%), lethargy (27%), rectal bleeding (5%), and colonic perforation (2.5%). There were no deaths directly related to HEC. The analysis of 150 plain x-rays of the abdomen, taken at the onset of HEC or in between bouts, showed that colonic dilatation was the most sensitive radiological finding (90% sensitivity), but it had poor specificity (24%). However, an intestinal cutoff sign (gaseous intestinal distension with abrupt cutoff at the level of the pelvic brim) was both sensitive (74%) and specific (86%) for HEC. Barium enema was of limited value in the diagnosis of HEC bouts because most of the radiographic findings persisted for prolonged periods after cessation of such bouts. The authors conclude that (1) HEC can be characterized as abdominal distension and explosive diarrhea associated with the intestinal cutoff sign and (2) the occurrence of explosive diarrhea in any patient with HD is suggestive of HEC, even in the absence of systemic symptoms, and should be treated to avoid the morbidity and potential mortality of HEC.

Enterocolitis following endorectal pull-through procedure in children with Hirschsprung's disease.

The enterocolitis following a pull-through in Hirschsprung's disease can be life-threatening and difficult to distinguish clinically from gastroenteritis and post-operative complications. We reviewed retrospectively the abdominal radiographs in our series to identify specific radiographic characteristics of this syndrome in this population. A total of 55 episodes of enterocolitis with an abdominal series at presentation were located in the files of 43 patients following pull-through surgery for Hirschsprung's disease. There were 15 abdominal series with other complications of Hirschsprung's disease and surgery (seven cases of small bowel obstruction, one of fistula, one of abscess, six of severe constipation) and 71 surveillance follow-up studies. Radiographs were evaluated for bowel dilatation, air-fluid levels, intestinal cut-off sign, speculation, and pneumatosis. The intestinal cut-off sign with two or more air-fluid levels had sensitivity of 68% and specificity of 83%, with a positive predictive value of 0.71 and overall accuracy of 77%. Our review of enterocolitis following pull-through in children with Hirschsprung's disease concludes that the constellation of an intestinal cut-off sign and at least two air-fluid levels on the abdominal series strongly suggests the diagnosis.