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.

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.

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.

[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.

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.

Complicated Gastroschisis Is Associated with Greater Intestinal Morbidity than Gastroschisis or Intestinal Atresia Alone.

AIM: The study aimed to compare outcomes and intestinal morbidity among complicated gastroschisis, isolated gastroschisis, and intestinal atresia. METHODS: In this retrospective observational single institution study, outcomes and intestinal morbidity were compared among gastroschisis complicated by intestinal atresia or perinatal bowel perforation, isolated gastroschisis, and isolated intestinal atresia. We included two cohorts; the first cohort included 68 consecutive patients with complicated gastroschisis (n = 9), isolated gastroschisis (n = 34), and intestinal atresia (n = 25) managed in our center. The second cohort included 20 patients (12 referred) with intestinal failure due to these underlying etiologies managed by our intestinal rehabilitation team. RESULTS: Patients with complicated gastroschisis had a significantly longer need for mechanical ventilation, primary hospital stay, and duration of parenteral nutrition (PN) and developed intestinal failure more often compared with other groups (p < 0.05 for all). Reoperations for surgical complications were also more frequent in patients with complicated gastroschisis (p < 0.05). Among those, who developed intestinal failure, autologous intestinal reconstruction (AIR) surgery was performed in 11 patients with comparable frequency in all groups. Repeated AIR surgery for bowel re-dilatation was required in 3/3 patients with complicated gastroschisis and 0/8 with isolated gastroschisis or intestinal atresia (p = 0.004). CONCLUSION: Complicated gastroschisis is associated with markedly increased intestinal morbidity, reflected by prolonged duration of PN, more frequent reoperations for intestinal complications, and bowel re-dilatation after AIR surgery, when compared with patients with isolated gastroschisis or intestinal atresia.

Presentation and outcome of treatment of jejunoileal atresia in Nigeria.

BACKGROUND: Intestinal atresia is a common cause of neonatal intestinal obstruction. Previous reports from Nigeria have indicated a high mortality rate. This is a report of current outcome review from one tertiary center. PATIENTS AND METHODS: A retrospective analysis of infants managed for jejunoileal atresia in 10 years (2005-2014). The information retrieved from patients' records was analyzed using SPSS 17. RESULTS: There were 38 patients (19 boys and 19 girls) aged 1-28 days (median 4 days). Twenty-four patients (63.2%) presented after 48 h of life. Twenty-five (65.8%) had jejunal atresia and 13 (34.2%) had ileal atresia. Six patients had associated anomalies. The most common atresia was type III (39.5%, 15 patients). Twenty-eight (73.7%) patients had a resection of the atresia and anastomosis and others had enterostomies. Total parenteral nutrition and neonatal intensive care support were not available during the period of the study. Bowel function was established within 1 week and 27 (71.1%) patients commenced oral feeding. Twenty-six (68.4%) patients had postoperative complications resulting in prolonged hospital stay of 2-44 days (median = 13). Mortality was 34.2% (13 patients). Factors that significantly affected mortality were intestinal necrosis at presentation, postoperative complications, and severe malnutrition. CONCLUSION: Intestinal atresia is still associated with unacceptably high morbidity and mortality, due to late presentation, and lack neonatal intensive care services and parenteral nutritional support. Efforts need to be intensified to address these factors to improve outcome.

The pediatric intestinal mucosal microbiome remains altered after clinical resolution of inflammatory and ischemic disease.

BACKGROUND: The pediatric intestinal microbiome is impacted by many factors, including age, diet, antibiotics, and environment. We hypothesized that in operative patients, alterations to antibiotics and mechanoluminal stimulation would demonstrate measurable changes in the intestinal microbiome and that microbial diversity would be reduced without normal mechanoluminal stimulation and with prolonged antibiotic treatment. METHODS: Bacterial 16s rRNA was extracted from swabbed samples of 43 intestines from 29 patients, aged 5 days to 13 years old. Swabs were obtained during initial resection or later stoma closure. Samples were compared using phylogenetic diversity whole tree alpha diversity and unweighted UniFrac distance beta diversity and by comparing significantly different taxonomic groups. RESULTS: Microbial community structure varied significantly between obstructive and inflammatory diseases (P = .001), with an effect size of 0.99 (0.97, 1.00). This difference persisted even 6 weeks after return to health. Family Enterobacter and Clostridiaceae predominated in patients with necrotizing enterocolitis or focal intestinal perforation; patients with an obstructive pathology had an abundance of Bacteroides. Comparison of UniFrac distance between paired proximal and distal intestines demonstrated that paired samples were significantly closer than any other comparison. CONCLUSION: In infants, inflammatory and ischemic intestinal pathologies treated with prolonged courses of antibiotics durably alter the intestinal mucosal microbiome. Diversion of mechanoluminal stimulation, however, does not.

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.

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.

The acute abdomen in the newborn.

The acute abdomen in the newborn provides challenging problems from many aspects, not only with regard to diagnosis, resuscitation and treatment, but also now with prenatal management. Most conditions are uncommon and treatment in specialist centres enables concentration of appropriate resources and expertise. Co-morbidity is common, particularly in the preterm or low birth weight infant. A multi-disciplinary team of surgeons, anaesthetists, neonatologists, radiologists, cardiologists, obstetricians, nurses, physiotherapists and other health professionals experienced in dealing with extremely small infants will provide the best outcome. The infant should be resuscitated and, as soon as conditions permit, transferred to a specialist surgical centre with intravenous fluids, gastric tube drainage and circulatory, respiratory and general support as needed. This involves close liaison within healthcare networks and readily available patient transfer facilities. Surgery itself should be carried out in a theatre fully equipped for neonatal surgery. A gentle touch is essential because of the fragility of the tissues, and painstaking care should be taken with blood loss.

[Percutaneous gastrostomy as an urgent method of treatment of a neonate with esophageal and duodenal atresia]. / Perkutana gastrostomija kao urgentna mera u lecenju novorodnenceta s atrezijom jednjaka i udruzenom atrezijom duodenuma.

The objective of this article was to present possibilities of percutaneous gastrostomy for management of a newborn with esophageal and duodenal atresia. Percutaneous gastrostomy is the most commonly employed technique for providing long-term enteral nutrition. In adults, it is also method of choice for decompression of digestive tract in palliative treatment of malignant and non malignant diseases. This is a case report of two-day old newborn with esophageal atresia with distal tracheoesophageal fistula and duodenal atresia. Percutaneous decompressive gastrostomy was used as a temporary bridge to definite surgery for management of respiratory dysfunction of this newborn, caused by dilated stomach. Surgical management (esophageal and duodenal reconstructions) as well as intra- and postoperative period were without any complications. Three months after surgical management, the infant is well and without any difficulties.

The effect of antenatal diagnosis on the management of small bowel atresia.

The aim of this study was to determine whether babies with small bowel atresia (SBA) diagnosed antenatally followed a different course from those diagnosed postnatally. We reviewed the records of neonates admitted to a single institution between 1985 and 2000 with a diagnosis of SBA. Thirty-nine neonates presented with SBA, with antenatal diagnosis (AND) being made in 12 (31%). There was no difference between the AND and postnatal diagnosis (PND) groups in terms of gestational age at birth, but the AND group had a lower mean birth weight. The babies in the AND group were operated on more quickly than those in the PND group. Ten out of 12 (83%) AND patients required parenteral nutrition compared with 12 out of 27 (44%) in the PND group, and the AND group had a significantly longer mean length of stay and spent a longer time on parenteral nutrition than the PND group.

Use of intraluminal stents in multiple intestinal atresia.

Multiple intestinal atresia presents a difficult technical problem because of extreme loss of intestinal length, disparity of residual bowel wall size, and discontinuity of multiple short intestinal segments. The authors report on a 3,000-g infant with gastroschisis complicated by intrauterine volvulus and multiple intestinal atresias who was treated successfully with intraluminal stenting and sutureless anastomoses. A total of 25 cm of small bowel was salvaged including 13 segments each measuring 1 to 8 cm in length. Subsequent radiographic studies showed spontaneous anastomosis with a compartmental configuration of the residual bowel and decreased transit time. Five months postoperatively, the patient was weaned off total parenteral nutrition completely and one year later is growing and gaining weight with 4 to 6 bowel movements per day.

Transanastomotic feeding tube after an operation for duodenal atresia.

UNLABELLED: The aim of this study was to answer the question whether or not, after an operation for duodenal atresia, a transanastomotic feeding tube reduces the time to full preanastomotic feeding. The method used was a retrospective study and a prospective observation. 18 consecutive newborns with duodenal atresia, nine from each of two different centres of paediatric surgery, were studied retrospectively. The patients in one centre received a nasogastric tube and a transanastomotic feeding tube during the operation, while in the other centre only a nasogastric tube was used. Seven control patients with duodenal atresia treated postoperatively with a nasogastric tube and a transanastomotic feeding tube were prospectively observed. The main outcome measure used to compare these two groups was the time required to achieve full preanastomotic feeding. RESULTS: The patients who were treated postoperatively with the transanastomotic feeding tube needed significantly less time to achieve full preanastomotic feeding than those with a nasogastric tube only (P < 0.001, Mann-Whitney U test). CONCLUSION: The use of a transanastomotic feeding tube, after an operation for duodenal atresia, leads to earlier full preanastomotic feeding.

Trends in the management and outcome of jejuno-ileal atresia.

The aim of this study was to evaluate contemporary patterns of presentation and trends in the management and outcome of newborn infants with jejuno-ileal atresia at a regional paediatric surgical centre in the United Kingdom. The hospital neonatal surgical registry was used to identify patients with jejuno-ileal atresia (n = 83) admitted between 1976 - 1998, excluding those associated with gastroschisis. The clinical records were reviewed and antenatal information, patient demographics, associated anomalies, operative treatment, post-operative management and outcomes were analysed in three time periods to identify trends in management and survival: Group 1 1976 - 1982 (n = 32), Group 2 1983 - 1990 (n = 21), and Group 3 1991 - 1998 (n = 30). Overall survival was 90 %. The number of patients with associated anomalies were Group 1, 10 (31 %); Group 2, 7 (33 %); and Group 3, 11 (37 %). Cystic fibrosis was encountered in 4 (13 %), 1 (5 %) and 4 (13 %) patients, respectively. Resection with primary anastomosis was the definitive management in most of patients: Group 1, 25 (78 %); Group 2, 17 (81 %); and Group 3, 27 (90 %). Initial stoma followed by delayed primary anastomosis was performed in 14 infants; eight patients had divided stomas while 6 had Bishop-Koop stoma. Tapering was used in 10 patients (12 %) with proximal jejuno-ileal atresia. Parenteral nutrition was increasingly utilised over the three time periods studied. There were no deaths in Group 3 compared to 6 deaths in Group 1 and 2 in Group 2 (P = 0.02). Most of the deaths were due to overwhelming sepsis. Mortality did not correlate significantly with the TYPE of atresia, presence of associated anomalies or the need for long-term total parenteral nutrition. The overall complication rate in survivors was 18 %. In the infants undergoing Bishop-Koop operation the complication rate was 50 %. This study has shown a significant reduction in mortality from jejuno-ileal atresia, which may be attributed primarily to advances in perioperative management, including parenteral nutrition. Generous resection of the atretic segment with primary anastomosis is more frequently employed in preference to initial stoma formation. Cystic fibrosis remains an important co-morbid condition that must be excluded promptly in all newborns.