Repeated pull-through surgery for complicated Hirschsprung's disease--principles derived from clinical experience.

BACKGROUND: In some patients, an initial pull-through procedure for Hirschsprung's disease fails, and obstructive symptoms persist or recur. Then a repeated pull-through operation may be necessary. METHODS: Seventeen patients with Hirschsprung's disease aged 2 to 9 years (median, 4.6 years) have undergone a repeated pull-through procedure because of unresponsive symptoms after an initial operation. The initial procedure was Soave in 3 patients, Rehbein in 13 patients, and Duhamel in 1 patient. Surgical revision was indicated by incomplete resection of the transition zone in 16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients. All 17 patients have undergone Redo Duhamel pull-through procedure. Median follow-up after Redo operation was 9 years (range, 1-23 years). RESULTS: In 15 patients, the stooling pattern normalized immediately after Redo procedure. Two, including 1 with Down's syndrome, are prone to constipation with occasional use of laxatives. Soiling is seen in the patient with Down's syndrome, but only with episodes of diarrhea. In spite of large formation of scars surrounding the neoanorectum in most patients, Duhamel pull-through reconstruction was possible in all children of this series. CONCLUSIONS: The predominant cause for persistent or recurrent unresponsive obstructive symptoms after initial pull-through procedure is incomplete resection of the transition zone. Less frequently, anastomotic strictures, rigidity of the anorectal cuff, and fistulas cause obstruction. Preoperative workup must focus on these complications. The courses after initial pull-through procedure show that laxatives, Malone procedure, dilatations of the anorectum, myectomy, V-Y-plasty, and injections of botulinum toxin cannot eliminate the mechanical or functional obstruction. Although a large formation of scars in the pelvis resulting from the initial operation, myectomies, dilatations, and other surgical modalities render a Redo revision more difficult, Redo Duhamel pull-through procedure is able to provide the definitive solution to the problem.

Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies.

Intestinal Dysganglionoses (IDs) represent a heterogeneous group of Enteric Nervous System anomalies including Hirschsprung's disease (HD), Intestinal Neuronal Dysplasia (IND), Internal Anal Sphincter Neurogenic Achalasia (IASNA) and Hypoganglionosis. At present HD is the only recognised clinico-pathological entity, whereas the others are not yet worldwide accepted and diagnosed. This report describes the areas of agreement and disagreement regarding definition, diagnosis, and management of IDs as discussed at the workshop of the fourth International Meeting on "Hirschsprung's disease and related neurochristopathies." The gold standards in the preoperative diagnosis of IDs are described, enlighting the importance of rectal suction biopsy in the diagnostic workup. The most important diagnostic features of HD are the combination of hypertrophic nerve trunks and aganglionosis in adequate specimens. Acetylcholinesterase staining is the best diagnostic technique to demonstrate hypertrophic nerve trunks in lamina propia mucosae, but many pathologist from different centers still use H&E staining effectively. Moreover, the importance of an adequate intraoperative pathological evaluation of the extent of IDs to avoid postoperative complications is stressed. Although it is not clear whether IND is a separate entity or some sort of secondary acquired condition, it is concluded that both IND and IASNA do exist. Other interesting conclusions are provided as well as detailed results of the discussion. Further investigation is needed to resolve the many controversies concerning IDs. The fourth International Conference in Sestri Levante stimulated discussion regarding these entities and led to the International guidelines to serve the best interest of our patients.