Several pull-through procedures have been described for Hirschsprung disease (HSCR) with varying functional outcomes. The voluntary bowel movement (VBM) and the absence of soiling or constipation after pull-through remain the most important markers of good outcome. We aimed to compare the functional outcomes in HSCR patients following Soave and Duhamel procedures. Krickenbeck classification was utilized to determine VBM, soiling and constipation for patients who underwent Soave and Duhamel pull-through at Dr. Sardjito Hospital, Indonesia from 2013 to 2016. Fifty-three patients were ascertained (Soave: 23 males and 2 females vs. Duhamel: 22 males and 6 females, p = 0.26). Ninety-three and 88% patients had a VBM following Duhamel and Soave pull-through, respectively (p = 0.66). Constipation frequency was significantly higher in Soave than Duhamel groups (24% vs. 4%; p = 0.04) with OR of 8.5 (95% CI = 1.0–76.7), whereas soiling rate was similar between Duhamel (21%) and Soave (8%) groups (p = 0.26). Furthermore, the risk of constipation was increased ~ 21.7-fold in female patients after Soave procedure and was almost statistically significant (p = 0.05). The constipation rate is higher in patients who underwent Soave than Duhamel procedure, but the VBM and soiling frequencies are similar. The constipation risk following Soave pull-through might be increased by the female gender. Furthermore, a multicenter study with a larger sample of patients is necessary to clarify and confirm our findings.

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Functional outcomes in Hirschsprung disease patients after transabdominal Soave and Duhamel procedures

The objective of this study is to identify risk factors associated with the development of post-operative enterocolitis (HAEC), in short segment Hirschsprung’s disease (HSCR-S). A retrospective study was carried out for post-operative patients with HSCR-S from 1997 to 2017. HSCR-S was defined as the most proximal extension of aganglionosis limited to the sigmoid colon. An episode of HAEC was defined as the presence of (1) vomiting or explosive diarrhea; (2) abdominal distension; (3) fever and (4) leukocytosis. Risk factors for the development of HACE were determined using multivariate logistic regression. The medical records of 96 patients were reviewed. The overall incidence of HAEC was 20.8% (n = 20) and 65.0% (n = 13) of HAEC occurred within the first year of operation. After a univariate logistic regression analysis, three risk factors for HAEC were identified: (1) presence of other major anomalies [OR: 1.43 (1.12–2.32), p = 0.041]; (2) creation of pre-operative defunctioning stoma [OR: 2.28 (1.47–3.23), p = 0.035]; (3) extension of aganglionosis to the sigmoid colon [OR: 1.89 (1.05–3.19), p = 0.049]. After multivariate logistic regression analysis, a significant association was demonstrated for creation of pre-operative defunctioning stoma [OR: 1.81 (1.08–3.22), p = 0.045] and extension of aganglionosis to the sigmoid colon [OR: 1.91 (1.37–2.98), p = 0.038]. The requirement of pre-operative defunctioning stoma and a more proximal extension of aganglionosis are risk factors for the development of post-operative HAEC in HSCR-S. Patients with these risk factors should be closely followed up especially during the first year after the operation.

Risk factors for the development of post-operative enterocolitis in short segment Hirschsprung's disease.

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. 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.

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Outcomes in patients with Hirschsprung disease following definitive surgery

Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung disease (HSCR), that might occur preoperatively. We investigated the risk factors of preoperative HAEC. We retrospectively reviewed all medical records of HSCR patients admitted at Dr. Sardjito Hospital, Indonesia from March 2012 until March 2015. Diagnosis of HAEC was determined using the Delphi scoring system. Sixty-one HSCR patients were involved in this study, of whom 48 were males and 13 females. Eighteen percent (11/61) patients had a preoperative HAEC. The most common findings of the HAEC score found in our patients were distended abdomen (100%) and dilated loops of bowel (100%), followed by lethargy (72.7%), cut-off sign in rectosigmoid with absence of distal air (72.7%), leukocytosis (72.7%), and shift to left (63.6%). There was no association between gender, age of HSCR diagnosis, early/late diagnosis during neonatal period, aganglionosis type, albumin level nor body mass index with preoperative HAEC (p = 0.69, 0.76, 0.33, 1.0, 0.86, and 0.50, respectively). In addition, the maternal age, gestational age, and maternal education level also did not correlate with the development of preoperative HAEC (p = 0.71, 0.59, and 0.32, respectively). The incidence of preoperative HAEC in our hospital is considered relatively moderate, with the most common findings of distended abdomen and dilated loops of bowel. None of the identified risk factors have an association with the development of HAEC in our patients.

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Risk factors of preoperative Hirschsprung-associated enterocolitis

The risk factors for postoperative Hirschsprung-associated enterocolitis (HAEC) are still incompletely understood, especially age at which surgery is performed. Therefore, the aim of this study was to identify the risk factors for the development of postoperative HAEC in children operated during infancy. Thirty-five children who had undergone radical surgery for Hirschsprung disease (HD) during infancy were included in the study. They were divided into two groups; those who developed postoperative HAEC (HAEC, 14 patients) and those who did not (no HAEC, 21 patients). Their medical records were retrospectively reviewed for clinical details. Developing postoperative HAEC was significantly associated with long-segment HD (p = 0.020) and the age at radical surgery (p = 0.0241). No other factors had a significant association with postoperative HAEC. In the patients who developed postoperative HAEC (n = 14), those with Trisomy 21 had significantly longer hospitalizations than those without. Patients with long-segment HD had a higher hospitalization rate than those with short-segment HD. This study clearly showed that long-segment HD and older age at radical surgery are risk factors for developing postoperative HAEC.

Isidora Galuh Parahita

Papers

Comparison of Hirschsprung-associated enterocolitis following Soave and Duhamel procedures.