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. 2018 Sep 4;11(1):644.
doi: 10.1186/s13104-018-3751-5.

Outcomes in patients with Hirschsprung disease following definitive surgery

Gunadi  1 , Stefani Melisa Karina  2 , Andi Dwihantoro  2
Affiliations

Outcomes in patients with Hirschsprung disease following definitive surgery

Gunadi et al. BMC Res Notes. .

Abstract

Objective: Several pull-through procedures have been described for Hirschsprung disease (HSCR) with varying outcomes. We aimed to describe the outcomes in HSCR patients < 18 year of age who underwent surgical procedures at Dr. Sardjito Hospital, Yogyakarta, Indonesia from January 2013 to December 2014.

Results: We utilized 67 HSCR patients, of whom 49 (73%) were males and 18 (27%) females. Neonatal presentation was seen in 57 cases (85%) and most patients (98.5%) had short-segment HSCR. The clinical manifestations were mainly abdominal distension (94%) and delayed passage of meconium (45%). The most common definitive treatment performed was transanal endorectal pull-through (TEPT) (54%), followed by Soave (18%) and Duhamel (13%) procedures. Enterocolitis occurred in 13% of the HSCR patients after endorectal pull-through, but did not reach a significant level (p-value = 0.65), while the constipation rate was significantly higher in HSCR patients who underwent posterior neurectomy compared with those other procedures (OR = 15.5, 95% CI = 1.8-132.5; p-value = 0.019). In conclusions, most HSCR patients in Indonesia were diagnosed in the neonatal period and underwent the TEPT procedure. Furthermore, the risk of constipation is increased in HSCR patients following posterior neurectomy compared with other definitive surgical techniques.

Keywords: Constipation; Definitive surgery; Enterocolitis; Hirschsprung disease.

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Figures

Fig. 1
Fig. 1
Histopathological findings of full-thickness rectal biopsy in a patient with Hirschsprung disease (HSCR) showed hypertrophic nerve trunk and no ganglion cells (arrow): a hematoxylin and eosin staining (×200), b S100 immunohistochemistry (×200)
See this image and copyright information in PMC

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