Immunohistochemical evaluations of ultrashort-segment Hirschsprung's disease
ABSTRACT PURPOSE: Unlike classic Hirschsprung's disease, short-segment and ultrashort-segment varieties are usually found to be latent and milder. Ultrashort-segment Hirschsprung's disease may present as intractable chronic constipation in children over one year of age, adolescents, and adults. Anorectal myectomy has been shown in many instances to provide effective long-term treatment for certain patients with ultrashort-segment Hirschsprung's disease. Histologically, the affected segment in Hirschsprung's disease has been shown to have increased cholinergic nerves, lack of nitric oxide synthase-containing neuronal elements, and show moderate to severe loss of myenteric neurons. METHODS: Here, we report three cases that showed clinical and manometric evidence of ultrashort-segment Hirschsprung's disease. Two of the three patients responded well to myectomy. RESULTS: Detailed histologic and immunohistochemical evaluation of the internal anal sphincter and a comparison with three normal controls revealed absence of nitric oxide synthase-containing neurons in both cases that responded well to surgery and continued presence of these neurons in the patient who did not respond. A review of the current literature on various treatment modalities is included. CONCLUSIONS: Anorectal myectomy provides long-term relief of this chronic problem in a subgroup of patients with ultrashort-segment Hirschsprung's disease who lack nitrinergic neurons at the internal anal sphincter.
- British Journal of Surgery 08/1988; 75(7):723. · 4.84 Impact Factor
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ABSTRACT: The distribution and colocalization of nitrinergic and peptidergic nerves were examined in six human colons. The tissues were fixed, cryosectioned, and standard immunohistochemistry was performed for several known neuropeptides. The same sections were stained for NADPH-diaphorase to denote nitric oxide synthase. NADPH-diaphorase-positive myenteric neurons were counted and colocalization noted for each peptide, as well as for peptide terminations. Galanin was the only neuropeptide that colocalized to a significant extent (23.0 +/- 7.21%) with NADPH-diaphorase-positive myenteric neurons. Many neuropeptide-containing nerve fibers had extensive terminations onto NADPH-diaphorase-positive neurons. Vasoactive intestinal peptide was the only neuropeptide that colocalized with NADPH-diaphorase to any extent in nerve fibers within circular muscle (59.5 +/- 9.3%). Fiber distribution in the longitudinal muscles showed a similar, but less dense pattern. These observations provide morphological evidence for the presence of nitric oxide, a candidate nonadrenergic noncholinergic neurotransmitter in the human colon.Neuropeptides 08/1995; 29(1):1-9. · 2.07 Impact Factor
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ABSTRACT: Hirschsprung's disease in the adolescent and adult is a rare and often misdiagnosed cause of lifelong refractory constipation. Two adolescent and three adult patients with Hirschsprung's disease treated between 1973 and 1987 at the University of Michigan Medical Center are reported. Each patient presented with chronic constipation requiring enemas, cathartics, and multiple hospital admissions for management. Diagnosis in each case was made with barium enema and full-thickness rectal biopsy. Four patients underwent endorectal pull-through procedures, all with good long-term results. The fifth patient, initially treated with a Duhamel retrorectal pull-through procedure, required reoperation for constipation secondary to a retained rectal septum. Review of 199 cases of adult Hirschsprung's disease enables comparison of the various operative procedures for this disorder with respect to postoperative complications and functional outcomes. Anorectal myectomy with low anterior resection, the Duhamel-Martin procedure, and the Soave endorectal pull-through procedure are the most acceptable methods for surgical management.Diseases of the Colon & Rectum 08/1990; 33(7):622-9. · 3.34 Impact Factor