Cases of adult onset hirschsprung s disease

Tel: ; Fax: ; E-mail: moc. This article has been cited by other articles in PMC. The primary pathogenic defect in adult HD is identical to that seen in infancy or childhood, and is characterized by the total absence of intramural ganglion cells of the submucosal Meissner and myenteric Auerbach neural plexuses in the affected segment of the bowel. Ninety-four percent of HD cases are diagnosed before the patient reaches 5 years of age, however, on rare occasion, mild cases of HD may go undiagnosed until he or she reaches adulthood. In this study, we describe four cases of adult HD with a history of longstanding recurrent constipation, relieved by laxatives, and presenting to the Department of Gastrointestinal Surgery with progressive abdominal distention, colicky pain or acute intestinal obstruction. Barium enema or computed tomography revealed a grossly distended proximal large colon with fecal retention.
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Adult Hirschsprung’s disease: report of four cases

Hirschsprung's Disease: Diagnosis and Management - American Family Physician

Immediate access to this article To see the full article, log in or purchase access. Address correspondence to Jennifer Kessmann, M. Reprints are not available from the author. Author disclosure: nothing to disclose.


Corresponding author. E-mail: ku. This article has been cited by other articles in PMC. However this may be as a result of misdiagnosis or late presentation as is the case with other causes of recurrent constipation in older age groups.
It is a rare condition in the adult, and usually mimicked by a long standing history of constipation, requiring great astuteness to diagnose. We present a case with all the clinical and radiologic characteristics of adult Hirschsprung disease, which had a final diagnosis of chronic constipation in a mentally impaired individual. The resulting aganglionic segment of the colon fails to relax, causing a functional obstruction [ 1 ]. We present a case of chronic constipation with a clinical history and radiologic findings [ 5 ] which mimicked Hirschsprung Disease diagnosed in the adult as described by case reports and series by others [ 3 , 6 - 9 ].
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