Jejuno-ileal atresia: its characteristics and peculiarities concerning apple peel atresia, focused on its treatment and outcomes as experienced in one of the leading South African academic centres.

PURPOSE: Jejuno-ileal atresia remains the most common form of intestinal obstruction in the neonatal and infantile age group and has an incidence of 1:300 to 1:1500. Apple peel atresia (APA) is the rarest of the five described types. The morbidity and mortality of patients with APA managed at our institution are high, and we review our experience with this paper. We compared our outcomes with other developed and developing countries. In addition, we looked at factors that affect outcome and how we can change them. METHODS: The study was a retrospective review of all patients treated with APA at IALCH between January 2002 and December 2010 and includes 34 patients. RESULTS: The results revealed a mortality in excess of 70%. There were poor antenatal screening, a high rate of prematurity and often delays in transfer to our institution. Relaparotomy and sepsis rates were high. CONCLUSION: This review represents a significant number of patients with APA from a single institution. Although survival rates have improved significantly over the years, APA remains a life-threatening malformation and results in significant morbidity and mortality in our setting.

Endoscopic balloon dilation in duodenal stenosis secondary to intestinal lymphoma in a heart transplant patient.

Intestinal lymphoma can appear as a result of a state of immunosuppression, secondary to the transplantation of solid organs. Treatment with chemotherapy can result in various complications such as intestinal stenosis due to fibrotic scar tissue, which is a complication barely described in the literature. We present the case of a 12-year-old male with a heart transplant, who was diagnosed with intestinal lymphoma. After chemotherapy treatment, he developed 1-2 daily vomiting episodes as a result of intestinal stenosis due to fibrosis without tumor infiltration. Endoscopic balloon dilation was used and the symptoms were resolved without the need for surgical treatment.

A novel rescue maneuver for a distal dislodged lumen-apposing metal stent (LAMS): "LAMS-in-LAMS" technique.

INTRODUCTION: the appearance of the lumen-apposing metal stent (LAMS) has meant an authentic revolution. To date, the results are promising but it is necessary to note the technical incidents and LAMS-related complications. CASE REPORT: an EUS-transmural guided drainage using a HotAXIOS was planned for a 36-year-old man with oral intolerance due to a voluminous walled-off necrosis. The distal flange was left in the collection, but a total distal malposition occurred during the proximal flange delivery, despite correct apposition with visualization of the black mark. A rescue technique was performed inserting a second LAMS over-the-guidewire salvaging the initial failed transmural drainage. DISCUSSION: This case is a reminder that in similar scenarios, extreme tension of the echoendoscope can cause a malfunction of the AXIOS stent delivery system, and lead to a total distal malposition. This "LAMS-in-LAMS" technique is feasible, effective, and a very helpful rescue technique in cases of dislodged LAMS.

[Therapeutic experience of type â ¢-b congenital intestinal atresia].

OBJECTIVE: To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA). METHODS: The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively. RESULTS: Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory. CONCLUSIONS: For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.

[Experience in treatment of complex congenital intestinal atresia in children].

OBJECTIVE: To summarize experience in the treatment of complex congenital intestinal atresia in children, so as to investigate the key points and effect of the operation. METHODS: Medical notes of 49 children with complex intestinal atresia treated between January 2012 and January 2018 were reviewed. The information of age, sex, age at operation, full-term or premature, birth weight, clinical manifestation, auxiliary examination, preliminary diagnosis, treatment process, discharge diagnosis, pathological results and prognosis of patients were analyzed. RESULTS: All patients underwent surgical treatment, including 42 cases with laparotomy (85.7%) and 7 with laparoscopic surgery (14.3%); 1 case undergoing laparoscopic surgery was converted to laparotomy due to meconium peritonitis. The mean operation time was (147±43) min (70-270 min); the mean fasting time after surgery was (8±3) d (4-16 d); the mean parenteral nutrition time was (12±6) d (3-30 d). Eleven cases were discharged against medical after operation and lost to follow-up. Among rest 38 children, 1 child (2.6%) received intestinal resection and ostomy five days after operation due to gastrointestinal perforation; 1 child (2.6%) received conservative treatment one month later due to adhered intestinal obstruction and left hospital with cure; 1 child (2.6%) received enterodialysis and ileostomy eight days after operation due to anastomotic leak, and received the operation for the closure of fistula after three months; 4 children had complications including fluid and electrolyte disorders, anemia, hypoproteinemia and so on, and recovered after conservative treatments. Postoperative follow-up showed that 1 child with duodenal atresia had lower body weight at 6 month after operation, but the body weight returned to normal when the child was one year old; 1 child with preterm labor of 32 weeks was treated with enteral nutrition, and gradually restored the normal diet after 6 months. Growth retardation was not observed in other children. CONCLUSIONS: With active treatment and reservation of normal bowel tube as much as possible during the operation, the prognosis of children with complex intestinal atresia is usually favorable.

What is this chocolate milk in my circuit? A cause of acute clotting of a continuous renal replacement circuit: Questions.

INTRODUCTION: One of the greatest problems associated with continuous renal replacement therapy (CRRT) is the early clotting of filters. A literature search revealed three case reports of lipemic blood causing recurrent clotting and reduced CRRT circuit survival time in adult patients, but no reports of cases in children. DIAGNOSIS/TREATMENT: A 23-month-old male infant with Martinez-Frias syndrome and multivisceral transplant was admitted to the hospital with severe sepsis and hemolytic anemia. He developed acute kidney injury, fluid overload and electrolyte imbalances requiring CRRT and was also administered total parenteral nutrition (TPN) and fat emulsion. The first circuit lasted 60 h before routine change was required. The second circuit showed acute clotting after only 18 h, and brownish-milky fluid was found in the circuit tubing layered between the clotted blood. The patient's serum triglyceride levels were elevated at 988 mg/dL. The lipid infusion was stopped and CRRT restarted. Serum triglyceride levels improved to 363 mg/dL. The new circuit lasted 63 h before routine change was required. CONCLUSION: Clotting of CRRT circuits due to elevated triglyceride levels is rare and has not been reported in the pediatric population. Physicians should be mindful of this risk in patients receiving TPN who have unexpected clotting of CRRT circuits.

Preservation of the native spleen in multivisceral transplantation.

The native spleen is usually removed in patients undergoing MTV. The consequential asplenic state is associated with a high risk of sepsis, especially in immunosuppressed children. In contrast, the inclusion of an allogeneic spleen in multivisceral grafts has been associated with a high incidence of GVHD. We propose an alternative technique for patients undergoing MTV, consisting of the preservation of the native spleen. This approach avoids the additional risk of infection that characterizes the asplenic state without the detrimental side effects of the allogeneic spleen.

Long-term outcomes for infants with intestinal atresia studied at Children's National Medical Center.

OBJECTIVES: Intestinal atresia is a common cause of intestinal obstruction in neonates. The predominant cause of late complications in these children is prolonged dependence on parenteral nutrition (PN). Our aims were to analyze the outcomes for patients with intestinal atresia at our institution, and to see how these changed with the implementation of an intestinal rehabilitation program (IRP). METHODS: This is a retrospective cohort study. The patient population is all children with intestinal atresias (118 patients) treated at our institution from July 2000 to June 2010, 20 of whom became PN dependent. RESULTS: Survival to hospital discharge was 95% for all patients, and 100% for those who had isolated atresia. Twenty of 118 patients (17%) were PN dependent beyond initial hospital discharge. At discharge, their median intestinal length was 22.5 cm, and they required PN for a mean of 88.5% of energy needs. Of these 20 patients, 2 died, 2 received transplants, and 2 transferred away for transplantation. The remaining 14 joined the IRP. Their mean energy requirement from PN is presently 10%, down from 87% at IRP enrollment, and 10 patients fully weaned off PN. Eleven of the 14 children had hyperbilirubinemia, with mean direct bilirubin of 7.5 mg/dL. All resolved their cholestasis during an average of 12 weeks. CONCLUSIONS: These results compare favorably with those reported in earlier periods. With programs such as the IRP, patients with short bowel secondary to intestinal atresia can show improvement in liver function and nutritional parameters, and discontinue PN, avoiding the need for transplantation.

Intestinal failure improve care project at Arkansas children's hospital.

Intestinal Failure is a complex and chronic condition that is challenging to patients, their families and their physicians. The intestinal rehabilitation team at Arkansas Children's Hospital adopts a multidisciplinary approach aimed at improving the outcomes of patients with Intestinal failure. The Intestinal Failure Improve Care Project will achieve these objectives through care coordination and monitoring, education and training of health care providers and families, pre-planned visits, and applications of evidence-based guidelines. The project will serve as an infrastructure for future clinical and translational research in the field.

Jejuno-ileal atresia: a 2-year preliminary study on presentation and outcome.

BACKGROUND/AIM: Intestinal atresia is a common cause of neonatal intestinal obstruction. Jejuno-ileum is the commonest site of intestinal atresia. Reports on Jejunoileal atresia in developing countries are still few. The purpose of this study is to determine the presentation and management outcomes of neonates with Jejunoileal atresia treated in our hospital. MATERIALS AND METHODS: Detailed data on all babies that presented and were treated within the study period (November2008-November, 2010) were kept and analyzed. A management protocol was put up and maintained. RESULT: A total of 9 babies (7males and 2 females) were treated. They were aged 2 hours to 13 days. Their weight ranged from 1.7kg to 3.3kg. Apart from one baby which presented within 2hrs with prenatal ultrasound diagnosis, others had bilious vomiting from the first day of birth, abdominal distension and delayed or absent passage of meconium. Even though symptoms developed on the first day of birth, presentation to the surgical unit was delayed 72hours and beyond in most patients.Type I atresia is commonest (no=4).There is associated gut malrotation in 2 babies. Five babies had complications which included surgical site infection, sepsis, prolonged vomiting post operatively, aspiration, rupture of dilated proximal segment after membrane excision, entero-cutaneous fistula and malnutrition. Three babies died giving a mortality of 33.3%. Mortality is commoner in types IIIb and IV. CONCLUSION: Mortality is higher in complex atresia which most times will require neonatal intensive care and parenteral nutrition facilities. These are still lacking in our institution. Providing these facilities will further improve outcome.

Cisapride improves enteral tolerance in pediatric short-bowel syndrome with dysmotility.

BACKGROUND AND OBJECTIVES: Gastrointestinal dysmotility is common in pediatric short-bowel syndrome, leading to prolonged parenteral nutrition dependence. There is limited literature regarding the safety and efficacy of cisapride for this indication. The aim of the study was to describe the safety and efficacy of cisapride for enteral intolerance in pediatric short-bowel syndrome. METHODS: Open-labeled pilot study in a limited access program for cisapride. Indications were short-bowel syndrome with underlying dysmotility and difficulty advancing enteral feeds despite standard therapies and without evidence of anatomic obstruction. Patients received cisapride 0.1 to 0.2 mg/kg per dose for 3 to 4 doses per day. We collected electrocardiogram, nutrition, and anthropometric data prospectively at study visits. RESULTS: Ten patients with mean (SD) age of 30.3 (30.5) months were enrolled in our multidisciplinary pediatric intestinal rehabilitation program. Median (interquartile range [IQR]) duration of follow-up was 8.7 (3.1-14.3) months. Median (IQR) residual bowel length was 102 (85-130) cm. Median (IQR) citrulline level was 14.5 (10.5-31.3) µmol/L. Diagnoses included isolated gastroschisis (n = 3), gastroschisis with intestinal atresia (n = 4), necrotizing enterocolitis (n = 2), and long-segment Hirschsprung disease (n = 1). Six subjects had at least 1 prior bowel-lengthening procedure. Median (IQR) change in percentage enteral energy intake was 19.9% (15.4%-29.8%) during follow-up (P = 0.01). Seven patients improved in enteral tolerance during treatment and 2 were weaned completely from parenteral nutrition. Complications during therapy were prolonged corrected QT interval (n = 2), gastrointestinal bleeding (n = 2), D-lactic acidosis (n = 1), and death due to presumed sepsis (n = 1). Longitudinal analysis (general estimating equation model) showed a strong positive association between cisapride duration and improved enteral tolerance. Mean percentage of enteral intake increased by 2.9% for every month of cisapride treatment (P < 0.0001). CONCLUSIONS: Cisapride is a potentially useful therapy in patients with pediatric short-bowel syndrome with gastrointestinal dysmotility. We observed modest improvement in feeding tolerance where prior treatments failed; however, patients treated with cisapride require careful cardiac monitoring because corrected QT prolongation occurred in 20% of our cohort.

How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review.

INTRODUCTION: Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. METHODS: All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. RESULTS: Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1-2) resections-anastomoses (RA) and 6 (1-10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15-25 cm). Median time to full oral feed was 46 days (14-626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2-8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. CONCLUSION: Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.

Parenteral fish oil-associated burr cell anemia.

We report the development of burr cell anemia in an infant with short bowel syndrome who received parenteral fish oil (Omegaven, Fresenius-Kabi, Graz, Austria) after development of total parenteral nutrition-associated liver disease. Parenteral fish oil was discontinued, and the burr cell anemia disappeared, suggesting that parenteral fish oil might be associated with hemolytic anemia.

Supplementation of Mother's Own Milk with Donor Milk in Infants with Gastroschisis or Intestinal Atresia: A Retrospective Study.

BACKGROUND: Mother's own milk (MOM) improves in-hospital outcomes for preterm infants. If unavailable, donor milk (DM) is often substituted. It is unclear if DM vs. formula to supplement MOM is associated with improved in-hospital outcomes in term/late preterm surgical infants with gastroschisis or intestinal atresia. METHODS: This retrospective study included infants born ≥33 weeks gestational age (GA) with a birth weight of >1500 g who were admitted to a quaternary neonatal intensive care unit (NICU). Using Chi square and Mann-Whitney u testing, we compared hospital outcomes (length of stay, parenteral nutrition and central line days) before and after a clinical practice change to offer DM instead of formula in this surgical population. RESULTS: Baseline characteristics were similar between eras for the 140 infants (median GA 37 weeks). Fewer infants in DM era were receiving formula at discharge (50.0% vs. 31.4%, p = 0.03). In sub-analyses including only small bowel atresia and gastroschisis infants, the median length of stay (35 vs. 25, p < 0.01) and the central line days (28 vs. 20, p < 0.01) were lower in the DM era. CONCLUSION: In this retrospective study, offering DM instead of formula was associated with less formula feeding at discharge, and in infants with gastroschisis or small bowel atresia, shorter length of stay and central line days.

Long-term surgical outcomes of apple-peel atresia.

PURPOSE: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis. METHODS: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed. RESULTS: Of the 39 APA neonates, 30 (76.9%) were born preterm, and 20 (51.3%) were diagnosed prenatally. All patients underwent primary anastomosis within the first week after birth: 10 laparoscopic-assisted (25.6%) and 29 open (74.4%). Postoperative complications occurred in 28 patients (71.8%), of which 20 (71.4%) developed cholestasis. Survival at hospital discharge was 94.9%. Median parenteral nutrition period was 59 days. Reoperation was required in 7 children (17.9%) owing to anastomotic obstruction (n = 3) and adhesive intestinal obstruction (n = 4). 32 children (82.1%) were followed up for an average of 5.7 years, of which 23 children (71.9%) showed normal growth and development. APA patients with low birth weight and associated anomalies had significantly worse outcomes. CONCLUSION: Most of the patients with apple-peel atresia have excellent long-term outcomes, though initial postoperative complications are common. Low birth weight and the presence of associated anomalies are independent prognostic factors in APA. TYPE OF STUDY: Prognosis study (case series). LEVEL OF EVIDENCE: Level IV.

Oesophageal atresia with tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease: outcome of a rare phenotype.

Oesophageal atresia with or without tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease are surgical malformations of the gastrointestinal tract typically diagnosed early in the neonatal period and varying in severity and prognosis. This report describes a full-term male newborn presenting simultaneous oesophageal atresia with distal tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease. In addition to the complex types of gastrointestinal malformations involved, the combination of ileal atresia and Hirschsprung's disease, as well as ganglion cells distal to intestinal atresia, resulted in a challenging diagnosis. Despite a successful outcome, the patient presented increased morbidity and prolonged hospitalisation. We highlight some important findings that may aid the early diagnosis of Hirschsprung's disease in this clinical setting. To our knowledge, the association of oesophageal atresia/tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease has not been previously reported.

A case of fetal midgut volvulus and jejunal atresia: nutritional support and maintenance of mucosal function and integrity.

Fetal midgut volvulus is a quite rare disease. Here, we report a case of a preterm newborn with fetal peritonitis, jejunal atresia with volvulus of post-atresic small bowel since about 30 cm from ileo-cecal valve (ICV) followed by a not-used microileus and microcolon. The surgical intervention consisted in the resection of volvulated necrotic small bowel followed by primary anastomosis. After surgery, total parenteral nutrition (TPN) has been started since 16(th) of post-operative days when enteral feeding (EN) was administered with a lactose-free formula containing hydrolyzed protein and medium-chain triglycerides (Pregestimil). Re-establishing intestinal continuity was preferred rather than stoma that would have required TPN. In fact, re-operation could have led to a shorter residual small bowel (RSB), since the anastomosis was at 15 cm from ICV. Our study provides evidence that not only the type of procedure (enteral versus parenteral) of nutritional support, but also the type of milk may modify the outcome. Early EN should be encouraged in newborn abdominal surgical patients because it is associated with reduced complications.

Multiple atresia of the small intestine: a 20-year review.

BACKGROUND: Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be balanced against preservation of the intestine's maximal length. MATERIAL AND METHODS: A retrospective analysis of the medical notes of 26 patients with MIA treated over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and surgical perspectives. RESULTS: All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an enterostomy performed at initial operation. Early and late postoperative complications requiring operative treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months, exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are alive and are on a full oral diet. CONCLUSION: Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.

Peripherally inserted central catheter-related complications in infants with intestinal failure.

BACKGROUND AND OBJECTIVES: For delivery of parenteral nutrition (PN), long-term central access is often required in infants with intestinal failure (IF). Compared to central venous catheters (CVCs), peripherally inserted central catheters (PICCs) are less invasive, as they are smaller, and they can even be placed without general anesthesia. In this study, we report the complications of long-term use of PICCs, and compare our results with previously published research. METHODS AND STUDY DESIGN: We reviewed the infants in the Xin Hua Hospital to determine the incidence of catheter-related bloodstream infections (CRBSIs) as well as other complication rates. RESULTS: A total of 43 infants diagnosed with intestinal failure and receiving PN through a PICC met the inclusion criteria. There were 66 PICCs accounting for 2563 catheter days, and a total of 29 complications were been recorded. The overall incidence of complications was 11.31 per 1000 catheter days, and the incidence of CRBSI was 5.85 per 1000 catheter days. Gram-positive bacterial species were the most common organisms growing in blood cultures. As for the risk factors, we find that low weight when PICC was inserted was associated with an increased risk of complications as well as low mean weight during the PICC dwelling time. CONCLUSIONS: We did not find an increased incidence rate of CRBSI in using PICC as an alternative to CVC. Also, as PICCs offer an advantage over CVCs in placing and nursing, we recommended PICCs as the first choice in patients with IF.

Is the Bishop-Koop procedure useful in severe jejunoileal atresia?

PURPOSE: The aim of this study was to report our experience using the Bishop-Koop procedure for the treatment of various surgical problems of jejunoileal atresia including luminal discrepancy, complex meconium peritonitis, type IIIb and type IV atresia which we defined as severe jejunoileal atresia. METHODS: This retrospective study was performed on the patients with severe jejunoileal atresia who underwent Bishop-Koop procedure at a tertiary center in China over a five year of period. The mortality, complication rate, nutrition status and the risk factors for postoperative adverse outcomes were explored. RESULTS: A total of 41 neonates underwent the Bishop-Koop procedure. The median duration of the hospital stay and total parenteral nutrition and the point at which oral feeding was initiated postoperatively were 24 days (95% CI =18.99-29.01), 13 days (95% CI = 9.03-16.97) and 11 days (95% CI = 10.17-11.83) respectively. The mortality rate was 7.32% (3/41). The complication rate was 41.4% (17/41) including anastomotic leak, intestinal obstruction, high output stoma and cholestasis. The weight for age Z-score at stoma closure was restored to normal levels (-0.86, 95% confidence interval (CI) = -1.44, -0.28). The main factor associated with adverse outcomes in severe jejunoileal atresia was premature delivery (odds ratio (OR) = 4.44, 95% CI = 1.06-18.67). CONCLUSIONS: Bishop-Koop procedure appears to be a technically efficient method for severe jejunoileal atresia, although larger studies are needed to compare Bishop-Koop procedure and other operation techniques. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: Level IV.