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Megarectosigmoid colon in an adolescent

Pediatrics and Neonatology(2023)

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摘要
A 12-year-old boy presented with abdominal distention since receiving supplementary foods at age 6 months. The average bowel movement was once every 5 days for 6 years, in conjunction with fecal soiling into the underwear for 3 years and occasional vomiting recently. During his visits to our hospital from October 2021 to January 2022, his body mass index was approximately 16.5 kg/m2, which was within the normal or healthy weight range for his age and hinted at no poor bodyweight gain. His stool usually looked like small pebbles; however, it could be hard and big enough to clog the toilet with enema use. The patient did not receive any examination or treatment for his clinical condition until recently, when his parents noticed the palpable mass localized in the middle abdomen. On physical examination, the abdomen was prominently protuberant, and a firm, fixed mass (25 × 15 cm) was localized in the middle abdomen. The abdominal radiograph revealed a mass of air mixed with feces in the middle of the abdomen (Fig. 1), and the contrast enema demonstrated a marked lengthening and dilatation of the rectosigmoid colon; however, neither an obvious transitional zone nor reverse rectosigmoid ratio was noted in this image (Fig. 2). A full-thickness rectal biopsy revealed the absence of ganglion cells alone 5.1 cm of the anorectal line (Fig. 3), indicating Hirschsprung's disease. The disease is commonly diagnosed in the newborn period or during early infancy; however, it can be seen in adolescence with a history of chronic constipation.1Wheatley M.J. Wesley J.R. Coran A.G. Polley Jr., T.Z. Hirschsprung's disease in adolescents and adults.Dis Colon Rectum. 1990; 33: 622-629Crossref PubMed Scopus (59) Google Scholar After the diagnosis was established, a primary transanal endorectal Soave's pull-through was arranged for the patient.2Ammar S.A. Ibrahim I.A. One-stage transanal endorectal pull-through for treatment of hirschsprung's disease in adolescents and adults.J Gastrointest Surg. 2011; 15: 2246-2250Crossref PubMed Scopus (11) Google Scholar The patient had gradually recovered to normal bowel movements a few days after the operation, and no soiling had been observed since.Figure 2A contrast enema demonstrated a marked lengthening and dilatation of the rectosigmoid colon.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Pathological results showed that the distal portion of the resected sigmoid colon had no ganglion cells (aganglionosis) (hematoxylin and eosin staining, × 100).View Large Image Figure ViewerDownload Hi-res image Download (PPT) The authors have no conflicts of interest relevant to this article.
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