Most HD children are diagnosed and treated in the neonatal period and early infancy, and the main treatment principle of HD is resection of the diseased bowel segment. Despite the fact that has one-stage operation has become preferred practice in managing HD, some children require an enterostomy in neonatal period. Patients undergoing stage surgery had higher rates of readmissions and additional operations, therefore, to make the appropriate treatment decisions, it's essential to understand the factors that influence enterostomy. According to literature reports, the incidence of enterostomy in infants with HD is 15.4% − 53%, and presents a gradually decreasing trend[4, 8–10]. In our study, the incidence of enterostomy was 12.0%, which was relatively lower.
Few studies have reported the influencing factors of enterostomy. According to a retrospective United States analysis of a multi-institutional cohort of neonates with HD, showed that patients with low birth weights, premature delivery, and non-HD gastrointestinal anomalies were more likely to undergo a stoma before definite surgery. Previous studies showed that Down syndrome was strongly associated with stoma formation[8, 11]. In this study, the factors mentioned above were not independently related to the enterostomy, and we found that low serum albumin level, bowel perforation and level of aganglionosis with long-segment and TCA were significant independent risk factors of enterostomy in neonatal HD patients.
We found that the serum albumin level was significantly lower of patients with stoma than that of patients without stoma. The serum albumin was usually used to assess acute versus chronic malnutrition, but some studies showed it was likely more predictive of inflammation and morbidity rather than nutritional status[12, 13]. In our study, there was no significant difference in birth weight or hospital weight between the enterostomy group and the non-enterostomy group, but there were significant differences in many inflammation-related indexes such as CRP, WBC, NEUT% between the two groups, which seemed to indicate that the decrease of albumin was related to the poor state of the patient.
Commonly reported factors influencing the decision for enterostomy include the presence of preoperative enterocolitis[9, 14, 15]. However, Bradnock et al. indicated that enterocolitis were not independently correlated with stoma formation. This could be because the clinical definition of enterocolitis is still elusive, making it difficult to capture the case. We did not collect cases of enterocolitis, but we collected and analyzed clinical factors associated with the diagnosis of enterocolitis, such as diarrhea, distended abdomen, fever, CRP, WBC, NEUT%, radiographic results as gas-fluid level, and radiographic results as intestinal wall pneumatosis. The results showed that fever, CRP, NEUT%, and radiographic results were associated with enterostomy, but they were not significant in multivariate logistic regression analysis, possibly due to they were predictors of enterocolitis that might lead to enterostomy. Since previous studies have used different definitions of HD associated enterocolitis, factors for the relationship between enterocolitis and enterostomy are not absolute, and the conclusions are still controversial.
According to European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) guidelines for the management of rectosigmoid HD, a stoma is indicated if there is bowel perforation. In our study, the incidence of bowel perforation was 5.3%, and the multivariate logistic regression analysis showed that radiographic results as subphrenic free air was a vital risk factor of enterostomy. Although most HD patients mainly present with low intestinal obstruction in the neonatal period, there are also some children with intestinal perforation as the first symptom, mostly located in the cecum and appendix. However, we found that 53.3% of patients (8/15) had perforations in the sigmoid colon, which was similar to another research about perforated HD. The intestines of newborns with HD associated enterocolitis are very fragile and easy to perforate during colon enema. In this group, 4 cases had obvious abdominal distension on admission, which could not be relieved by diet resistance, and received colon enema. Unfortunately, the 4 patients developed intestinal perforation after colon enema. Therefore, for institutions with less experience in colon enema in the neonatal period, in order to avoid iatrogenic perforation and aggravation of the patient's condition, temporary enterostomy could be selected. The atypical manifestations of neonatal intestinal perforation make clinical diagnosis and treatment extremely difficult. Once a diagnosis has been made, an exploratory laparotomy should be performed as soon as possible, and pathological biopsy should be performed during the operation on children suspected of HD.
In addition, two patients needed preoperative mechanical, and both had bowel perforation. we found preoperative mechanical was associated with enterostomy, consistent with the previous study, which might mean that patients was seriously ill and needed emergency surgery. However, the number of events in this study was limited, necessitating additional research.
Compared to rectosigmoid HD, long-segment HD or TCA conferred a higher rate of stoma formation[9, 15, 19]. In this study, type of HD with long-segment and TCA was an independent risk factor in multivariate logistic regression analysis, which was in agreement with a prospective study. The intestinal obstruction in children with long-segment or TCA often appeared earlier, and the effect of colon enema was poor, which was easy to complicated with enterocolitis. As a result, if neonates with long-segment HD or TCA are in poor general condition, an enterostomy may be preferable. It is worth mentioning that not all long-segment HD require functioning stoma in the neonatal period, which is also reflected in our data.
The retrospective nature of this study limited it, and it was subjected to several confounding variables such as variation in the preoperative management protocol over the study period. Although some of the analyses in this study did not reach statistical significance, we believe they may be clinically important, and these risk factors should be closely monitored. Additionally, cases of HD that were treated on an outpatient basis during neonatal period would not be captured in our cohort. A prospective study is required to explore the relationship between additional risk factors and enterostomy.