Abstract
ObjectiveSeveral 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.ResultsWe 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.
Highlights
Hirschsprung disease (HSCR), which is characterized by the absence of ganglion cells (Meissnerr and Auerbach) along variable lengths of the distal gastrointestinal tract, is a common cause of neonatal intestinal obstruction, which is of great interest to pediatric surgeons throughout the world [1]
Several definitive surgeries have been established for HSCR such as transabdominal endorectal pull-through (Soave), Duhamel, transanal endorectal pull-through (TEPT), transanal Swenson-like, and posterior neurectomy procedures [7–11], with varying outcomes [12]
We aim to describe the outcomes, constipation and enterocolitis, in HSCR patients following a definitive surgery in Indonesia
Summary
Hirschsprung disease (HSCR), which is characterized by the absence of ganglion cells (Meissnerr and Auerbach) along variable lengths of the distal gastrointestinal tract, is a common cause of neonatal intestinal obstruction, which is of great interest to pediatric surgeons throughout the world [1]. This disorder can be classified as follows: (1) short-segment (aganglionosis is confined to the rectosigmoid colon), (2) long-segment (aganglionic segment extends proximal to the sigmoid), and (3) total colonic aganglionosis [1]. A common variant within the RET gene, rs2435357, has been associated with HSCR across populations [2–6]. We aim to describe the outcomes, constipation and enterocolitis, in HSCR patients following a definitive surgery in Indonesia
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