MECONIUM ILEUS ; A STUDY AND COMPARISON BETWEEN COMMON OPERATIVE PROCEDURES PERFORMED IN BASRAH

Meconium ileus accounts for 9–33% of all neonatal intestinal obstructions, with an incidence of 1:2500 newborns, representing the third most common cause of neonatal small bowel obstruction after atresia and malrotation. This study aimed to compare various surgical procedures used in the treatment of meconium ileus and to assess their efficacy regarding survival and complications. A retrospective study was done to all cases of meconium ileus admitted to the neonatal intensive care unit of Basrah hospital of maternity and children and Basrah children specialty hospital during the period of 10 years (2005 to 2015). The medical records of 57 cases of meconium ileus were studied. The comparison included: Mikulicz procedure, Bishop-Koop procedure and resection with primary anastomosis in both simple and complex meconium ileus. The parameters used for comparison were anastomotic leaks, high output diarrhea with dehydration and failure to thrive, sepsis, need for reoperation, wound complications, early adhesions, hospital stay and mortality. The mean age of presentation of neonates with meconium ileus was 3.9 days. Male to female ratio was 0.9: 1. About 10.5% were preterm. About 33.3% of cases were diagnosed as simple meconium ileus. Intestinal Volvulus is the predominant complications encountered (47.4%). Non-operative treatment was effective in 45.5%. The most common procedures done in our center were Mikulicz procedure (61.5%), followed by Bishop-Koop procedure (30.8%), and resection with primary anastomosis (7.7%). There was a significant association between mortality and high output fistula, anastomotic leaks, sepsis, and reoperation. Predominant complications in Mikulicz procedures were high output fistula (50%) and skin excoriation (53.1%), while in Bishop-Koop procedure were sepsis (75%), reoperation (50%), and adhesions (25%). In primary anastomosis, significant complications were anastomotic leak (75%), sepsis (50%), and reoperation (50%). Mortality was highest in primary anastomosis (75%), followed by Bishop-Koop procedure (62.5%), and lowest with Mikulicz procedure (40.6%). The overall mortality of meconium ileus was high 45.6% (42.9% for simple meconium ileus and 52.6% for complex meconium ileus). All neonates treated non-operatively survived, while the survival rate for those treated surgically was 50%. In conclusion, resection with stoma creation is superior to primary anastomosis. Mikulicz procedure is the safest procedure to be done with best survival and less complications. BishopKoop procedure is of value in a situation where the surgeon is afraid from high output diarrhea so proximal stoma is mandatory. Introduction M econium ileus is one of the most common causes of intestinal obstruction in the newborn, accounting for 9–33% of neonatal intestinal obstructions. It is characterized by the lack of excretion of meconium during the first 48 hours of life, associated with clinical and specific radiological findings of intestinal obstruction. It is the intestinal obstructive variant of cystic fibrosis, and it is considered as the earliest clinical manifestation of this genetically recessive lethal disorder. Meconium ileus seems to be more the


Introduction
M econium ileus is one of the most common causes of intestinal obstruction in the newborn, accounting for 9-33% of neonatal intestinal obstructions 1 .It is characterized by the lack of excretion of meconium during the first 48 hours of life, associated with clinical and specific radiological findings of intestinal obstruction 2 .It is the intestinal obstructive variant of cystic fibrosis 3 , and it is considered as the earliest clinical manifestation of this genetically recessive lethal disorder 4 .Meconium ileus seems to be more the result of the presence of viscous mucus in the bowel than of pancreatic insufficiency itself 5 , in which, the meconium contains a high amount of protein and becomes extremely thick, causing obstruction of the terminal ileum 6 .Two form of meconium ileus can be described, simple and complex meconium ileus 7,8 .Most of cases are simple meconium ileus but complicated meconium ileus is found in about 40% of patients e.g.volvulus, atresia, perforation, or meconial pseudocyst 9 .A contrast enema with watersoluble and hyper or iso-osmolar contrast is the medical treatment of choice for uncomplicated cases 9 .Non-operative management of simple meconium ileus is achieved in about 60% to 70% of newborns 10 .Current short-term operative survival rates of 70% to 100% are reported 10 .Many surgical options have been used in the surgical treatment of meconium ileus including resection with primary anastomosis, Mikulicz ileostomy, Bishop-Koop operation, and Santulli stoma 11 .

Aims:
To compare various surgical procedures used in the treatment of meconium ileus and to assess their efficacy regarding survival and complications.

Results
The mean age of presentation of neonates with meconium ileus was 3.9 days (range from 16 hours to 9 days).Male to female ratio was 0.9:1.Six neonates presented with meconium ileus were preterm, representing 10.5% of all cases.The total cases of meconium ileus studied were 57 patients.Of them, 19 cases (33.3%) were diagnosed as simple meconium ileus, while 38 cases (66.7%) were diagnosed as complex meconium ileus.Complications encountered including intestinal volvulus in 18 (47.4%),perforation/peritonitis in 15 patients (39.5%), intestinal atresia in 6 cases (15.8%), and meconium pseudocysts in 3 cases (7.9%).Some patients had more than one complication.Of the simple meconium ileus, only 11 cases treated with gastrographin enema.Five patients only responded to non-operative treatment (45.5%), while 6 patients did not respond to gastrographin enema and demanded operative treatment (54.5%).So that 14 cases (8 cases did not undergo conservative attempts plus 6 cases with failed conservative measures) of simple meconium ileus were treated by surgery.Those were treated by Mikulicz procedure (7 cases), Bishop-Koop procedure (4 cases), or resection with primary anastomosis (3 cases).Of the complicated meconium ileus, 25 cases treated by Mikulicz procedure, 12 cases treated by Bishop-Koop stoma, and 1 case treated by resection with primary anastomosis.

Discussion
The mean age of presentation of neonates with meconium ileus was 3.9 days (min= 16 hours, max=9 days).Male to female ratio was 0.9:1 which is similar to other study 12 .Six neonates presented with meconium ileus were preterm representing 10.5% of all cases, similar to other study 9 .Simple meconium ileus accounts for 33.3% of cases which is less than that seen in other studies, 47.1% 13 (58%) 14 .This high percentage of complex cases may be due to ineffective prenatal diagnosis of meconium ileus and the consequent delayed diagnosis and referral.In order of frequency, complications associated with meconium ileus were as follows: intestinal volvulus, perforation/ Meconium ileus (52 cases) Mikulicz (32) Bishop ( 16) Primary ( 4 [13][14][15] .Most authors consider enema reductions as the initial treatment of choice for simple meconium ileus 16,17 .Regarding simple meconium ileus, in this review, only 11 cases were treated with gastrographin enema (1:3 or 4 sodium chloride dilutions).About 45.5% of cases showed satisfactory response to nonoperative treatment with complete recovery, relatively similar to other study (40%) 14 , 39% 18 .When treated nonoperatively, all neonates survived with no complications encountered.In cases of complex meconium ileus or failed non-operative treatment, surgical intervention is mandatory.The most common procedures done in our center were Mikulicz procedure (61.5%),Bishop-Koop procedure (30.8%), and resection with primary anastomosis (7.7%).The small number of cases treated by primary anastomosis must be interpreted with cautions.In this review, we tied to study the association between mortality and complications of various surgical procedures to assess their efficacy.There was a significant association for certain parameters including high output diarrhea, anastomotic leaks, sepsis, and reoperation.On the opposite side, the association between mortality and the presence of early adhesion or wound infections was not significant.In our study, we found that complications including anastomotic leak, sepsis, adhesive intestinal obstruction, wound infections and even the need for reoperations (required in cases of anastomotic leaks or in cases of adhesive intestinal obstruction) occur in higher frequency, in both simple and complex meconium ileus, than what are seen in other study 18,19 .Regarding high output diarrhea, it occurred in 21.4% of Mikulicz patients in a study done by A. Karimi and in 50% of our patients 18 .Similarly, sepsis was seen in 26.7% of A. Karimi study and 34.6% of our patients 18 .In addition, certain surgical procedures have higher complication rate than others.High output diarrhea and skin excoriation occur mostly in neonates treated with Mikulicz procedures.Anastomotic leaks with peritonitis occur mostly in patients treated with resection and primary anastomosis.Reoperation was required especially in patients treated with Bishop-Koop procedure or primary anastomosis.Predominant complications in Mikulicz procedures were high output fistula (50%) and skin excoriation (53.1%), while in Bishop-Koop procedure were sepsis (75%), reoperation (50%), and adhesions (25%).In primary anastomosis, significant complications were anastomotic leak (75%), sepsis (50%), and reoperation (50%).The overall mortality was very high (45.6%) in comparison with other studies where the survival was between 85-100% [13][14][15] .The best survival was encountered in patients treated with Mikulicz procedures in both simple and complex meconium ileus, followed by Bishop-Koop procedure, and the worst result was with primary anastomosis.Since primary anastomosis cases have higher rate of complications and deaths than resection with stoma creation, we prefer stoma creation rather than primary anastomosis in both simple and complex meconium ileus.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 18,20 , although closure of stoma can be done later on as an elective safe procedure.Since high output diarrhea with failure to thrive occurred more frequently in patient treated with Mikulicz stoma (50%); furthermore, it is significantly associated with mortality (P value=0.000),Bishop-Koop procedure can be used in a situation where enterostomy is not desirable due to Bas J Surg, December, 22, 2016 very proximal stoma but anastomotic leak is still an important complication 20 .Bishop-Koop procedure has been widely used 17,21,22 .Escobar et al. suggests that resection with primary anastomosis should only be used in case of atresia, resection with enterostomy being preferable in complex meconium ileus patients 23 .In this review, resection with stoma creation has lower mortality and complication rate than primary anastomosis; furthermore, Mikulicz stoma seems to be better than Bishop-Koop stoma in the term of survival and complications (apart from high output diarrhea and skin excoriation), which is similar to other study 18 , so that Mikulicz procedure is the best procedure to be done in our center.In conclusion, resection with stoma creation is superior to primary anastomosis.
Furthermore, Mikulicz procedure is the safest procedure to be done with best survival and less complications.It is obvious that, high output fistula is significantly associated with mortality and it is the commonly occurred with Mikulicz procedure so that in a situation, where the surgeon afraid from this complications (i.e.proximal stoma is mandatory), it is preferable to use another procedures.Alternatively, we have to close the stoma as soon as possible if Mikulicz procedure has been done.Total parenteral nutrition is, of course, of value in those patients.
Resection with primary anastomosis should be used with caution, both in patients with simple and with complex meconium ileus, although, a large prospective multicenter studies seem to be warranted to better identify its results.