Management of Meconium Ileus: 5 years' experience at Dhaka Shishu (Children) Hospital

Background : Meconium ileus (MI) is one of the common cause of intestinal obstruction in neonate. It can be present with various complications (about 50%) like volvulus, atresia, and gangrene of the gut, perforation and meconium cyst. 
Objective : This study aimed to compare various surgical procedures used in the treatment of meconium ileus and to assess their efficacy regarding survival and complications in our center. 
Methods : This retrospective study was done to all cases of meconium ileus admitted in the Department of neonatal surgery of Dhaka Shishu (Children) Hospital during the past 5 years (2011 to2016). The medical records of all patients with meconium ileus were studied. The surgical procedures were Mikulicz procedure, Bishop-Koop procedure done in case of complicated cases and Mikulicz procedure, Bishop-Koop and T tube ileostomy done in uncomplicated cases. Outcomes were compared between complicated and uncomplicated group and between the surgical procedures. 
Results : Total patients were 224. Among them 8 were excluded due to incomplete data. The mean age was 3.23 days. Twenty four neonates were preterm. Fifty-two percent were uncomplicatedMI and 48.15% were complicated. Among 112 uncomplicated cases, Mikulicz procedure done in 33 cases, Bishop-Koop procedure done in 37 cases and rest were treated by T tube ileostomy procedures. Among 104 cases of complicated meconium ileus, Mikulicz procedure done in 75 cases and 42 cases were treated by Bishop-Koop stoma. Predominant complications in Mikulicz procedures were high output fistula (67.6%), sepsis (34.84%) and skin excoriation (58.33%), while in Bishop-Koop procedure were sepsis (59.25%), anastomotic leak and reoperation (25.75%). In T tube ileostomy, complications were intra-peritoneal leak with reoperation and sepsis (9.52%). Twenty five (11.6%) stoma prolapse were found in Mikulicz ileostomy. The overall mortality of meconium ileus was 36.6%, in simple MI 23 out of 112 and in complicated MI 56 out of 104. This difference was significant. 
Conclusion : Considering the study result we concluded that complications occur more frequently in Mikulicz procedure and it is significantly associated with mortality, Bishop- Koop ileostomy can be considered but anastomotic leak is still an important complication of this procedure. In uncomplicated cases T tube ileostomy found as the best option. 
Northern International Medical College Journal Vol.10(1) Jul 2018: 326-329

Two form of meconium ileus can be described, uncomplicated and complicated meconium ileus. 1 In the uncomplicated form, thickened meconium obstructs the mid ileum and there is proximal dilatation, bowel wall thickening and congestion occurs. In complicated cases, this condition is complicated by volvulus, atresia, necrosis, perforation and meconium peritonitis and pseudocyst formation. 1 Uncomplicated meconium ileus can be treated with therapeutic contrast enemas with water-soluble and hyper or isoosmolar contrast. 3 Several complications have been reported following Gastrografin enema like perforation, necrotizing enterocolitis, shock and occasional death. 4 Therefore, some

Introduction
Meconium ileus (MI) is one of the common causes of intestinal obstruction in neonate, accounting for 9-33% of intestinal obstruction at this age. Obstruction occur secondary to the intraluminal accumulation of inspissated and desiccated meconium. 1 previously, meconium ileus was considered to be closely associated with cystic fibrosis. But, recent studies demonstrated that it can occur frequently without association of cystic fibrosis. Though the exact pathogenesis of meconium ileus in the absence of cystic fibrosis is unknown, a spectrum of genetic and pathological abnormalities may play a role. 2 data were assessed using student t-test. Associations of categorical data were assessed using Chi-square test and Fisher's exact test. For both test, p<0.05 was considered significant.

Results
During the study period total 3976 neonatal surgical cases were admitted, among them 224 were meconium ileus. The incidence of meconium ileus was 56.4 per 1000 neonatal surgical cases at the center. Eight patients were excluded for inadequate data. The mean age of presentation of neonates with meconium ileus was 3.23(± 1.08) days (range from 2 days to 6 days).
Male and female were same in number (108) and ratio. Twenty one neonates presented with meconium ileus were preterm, representing 11.11% of all cases. Among the studied cases, 112 cases (51.85%) were uncomplicated meconium ileus and 104 cases (48.15%) were complicated (Table: I).  (Table-II). criteria should be fulfilled before performing this procedure. 3 Reported success rate of non-operative management of uncomplicated meconium ileus is about 60 to 70% in newborn and current short-term operative treatment showed survival rate of 70 to 100%. 5 Options for surgical management of meconium ileus include resection of dilated ileum with primary anastomosis, Bishop-Koop ileostomy, Santulli procedure or Mikulicz procedure. 1 These are extensive operation, associated with gut reduction and high stoma output. A second surgery also required to close the stoma. 6,7 In uncomplicated meconium ileus, tube or T-tube ileostomy which includes enterotomy, evacuation of thick meconium and placement of T-tube for postoperative irrigation. 7,8 This procedure do not require gut resection and there is no intraperitoneal anastomosis. After extraction of T-tube the wound heal spontaneously and second operation to close the stoma is not required. 9 In our center, we do not have fluoroscopy to try non operative management of uncomplicated meconium ileus. Mikulicz ileostomy, Bishop Koop ileostomy has been practiced in our center for long time in both types of meconium ileus. In both of these procedures a second surgery to close the stoma is required. Recently we have started T tube ileostomy for uncomplicated meconium ileus.
This retrospective study was done to compare various surgical procedures used in the treatment of meconium ileus and to see their efficacy regarding survival and complications. Karimi A et al mentioned complication of meconium ileus is high may be due to ineffective prenatal diagnosis of meconium ileus and the consequent delayed diagnosis and referral. 10 In this study, Mikulicz ileostomy (50%) was the most common procedure then Bishop-Koop procedure (30.55%). Results were similar to Haithem H A. 11 In presence of contamination our surgeons preferred Mikulicz procedure as it requires less operation time and no intraperitoneal suture line. Therefore,it reduces duration of anesthesia and avoids intraperitoneal leakage in these sick patients.

Materials and Methods
Post-operative morbidities like high output fistula, skin excoriation and sepsis were more common in Mikulicz procedure. This is because Mikulicz procedure mostly performed in complicated cases. Intra peritoneal leakages were found more in  Table-III and IV.  Mean hospital stay was less in Mikulicz ileostomy procedure and more in Bishop-Koop Ileostomy procedure in both group (Figure-1). procedure. Further more, it was significantly associated with mortality. In Bishop-Koop procedure intra-peritoneal leakage and sepsis were more common but survival was higher than Mikulicz procedure. In uncomplicated cases T tube ileostomy is the best option to be performed due to less complication and high survival rate.
sepsis are less. 12 All patients survived after Mikulicz and Bishop Koop procedure required another operation to close stoma. This disadvantage is reported by most of the authors. 1,10,12 T-tube ileostomy only performed in uncomplicated cases. Intra peritoneal leakages were few (4 of 42 cases) and other complications were negligible. Stomas closed spontaneously after extraction of tube, therefore second operation did not require. After first description by Harbergeet al several authors described T tube ileostomy as a safe and effective procedure for uncomplicated meconium ileus. 6,8,9 Results of these studies were consistent with our study.
In both types of meconium ileus mean hospital stay for Mikulicz procedure was lower (7.2 to 7.44 days) than other procedures, as there was no intra-peritoneal suture line and stoma function establish earlier. The result is similar to Karimi et al and Haithem's studies. 10,11 The overall mortality of meconium ileus was 36.6%, which is lower than Haithem H A study, but was very high in comparison with other studies where the survival was between 85-100%. 1,11 Mortality (53.8%) was significantly higher in complicated group. Complicated patients mostly developed sepsis (61.5%) and it was very difficult to manage these patients without neonatal intensive care support.
The best survival was encountered in patients treated T tube ileostomy in uncomplicated cases and with Bishop Koop procedures in both uncomplicated and complicated meconium ileus. The worst result was Mikulicz procedure in both types. Ziegler MM and Karimi et al reported, resection with stoma creation is the safer procedure, preventing peritonitis due to anastomotic leakage but the need later on for closure of stoma being a relative disadvantage, although closure of stoma can be done later on as an elective safe procedure. 1