CLINICAL FEATURES OF THE COURSE OF HIRSCHSPRING'S DISEASE INCHILDREN OF THE FIRST YEAR.

The purpose of the work - to investigate the peculiarities of the clinical course of Hirschsprung's disease in children of the first year of life and to determine the significance of symptoms in the verification of the disease. From 1980 to 2021, at the pediatric surgery clinic of the National Medical University named after O.O. Bogomolets on the basis of the National Children's Specialized Hospital "OKHMATDYT" and in the pediatric surgery clinic of the Ivano-Frankivsk National Medical University on the basis of the Ivano-Frankivsk Regional Children's Clinical Hospital, 483 children of the first year of life suffering from Hirschsprung's disease were examined and treated. The clinical manifestation and course of aganglionosis varied in length at the time of hospitalization and depended on the time after birth. During the first month of life, 97 (20.08%) patients were hospitalized, of which 39 (8.07%) hadatypical clinical picture due to: colonic atresia in 15 (3.10%), colonic atresia + gastroschisis in 3 (0.62%), ileal atresia in 9 (1.86%), esophageal atresia in 3 (0 .62%), clefts of the hard and soft palate in 9 (1.86%). Depending on the age, there were 280 (57.97%) patients under 6 months, and 203 (42.03%) patients between 6 months and 1 year. The classic typical clinical picture was in 444 (91.93%) patients, which was characterized by the absence of meconium excretion, abdominal distension in 444 (91.93%), delayed physiological weight gain against the background of nutritional insufficiency with the development of hypotrophy in 327 (67.70%) , vomiting of stagnant gastric and intestinal contents in 417 (86.34%). On the other hand, enterocolitis in 315 (65.22%), toxic megacolon in 16 (3.31%), and anemia of various degrees occurred in 241 (49.89%) patients among the complications that arose during the examination of patients with Hirschsprung's disease. According to the results of a comprehensive examination, the following extent of aganglionosis was established: rectal in 100 (20.70%), rectosigmoid in 192 (39.75%), subtotal in 150 (31.06%) and total in 41 (8.49%) patients. Concomitant malformations were found in 98 (20.29%) patients: renal malformations were diagnosed in 7 (1.45%) patients, concomitant heart malformations in 18 (3.73%) patients. Associated intraoperative findings were Meckel's diverticulum in 5 (1.03%) and congenital cyst of the right ovary in 1 (0.21%) patient. The clinical course was affected by concomitant malformations: incomplete bowel rotation in 10 (2.07%) and internal abdominal hernia in 2 (0.42%). The clinical manifestations and course of Hirschsprung's disease primarily depend on the presence of accompanying developmental defects, which may prevail during the examination due to vital disorders. In the clinical course of Hirschsprung's disease, it is necessary to distinguish between typical and atypical forms. Typical clinical symptoms were in 444 (91.93%), and atypical in 39 (8.07%).

The profile and outcome of small bowel atresia at Universitas Academic Hospital.

BACKGROUND: In patients with intestinal atresia, associated congenital anomalies and prematurity are the main predictors of mortality in high-income countries (HIC). In low-and middle-income countries (LMIC), late referrals and lack of resources contribute to mortality. This study aims to describe the outcome of small bowel atresia at a tertiary institution in a LMIC. METHODS: A retrospective descriptive and analytical study was done on all neonates admitted with small bowel atresia from 1 January 2010 to 31 October 2016. Birth demographics, status on arrival, atresia type, surgical interventions and clinical course were analysed in relation to outcomes including mortality. RESULTS: The study included 92 patients. Thirty-three patients had duodenal atresia and 59 jejunal atresia. Median gestation was 34 weeks, with average birth weight 2.2 kg. Seven were diagnosed antenatally and 13 (14.1%) were born in a tertiary institution. A cardiac congenital anomaly was present in 43.5%. Late referrals comprised 72.8%. Sixty per cent were dehydrated and 55.4% were septic on arrival. The median age at surgery was 7.5 days after being admitted, for a median of 2 days. The median age reaching full feeds was 18 days, and 28.6% had a feeding intolerance. The median number of septic episodes were two and the median length of stay 28 days. Of the 92 patients, 8.7% were palliated and 10.9% died. Mortality was strongly associated with prematurity and sepsis with hospital stay. CONCLUSION: Despite late referrals, our mortality was comparable to HICs. We overcame this burden due to access to resources, sophisticated equipment and a low surgical complication rate. The length of stay could be decreased with a feeding protocol and discharge goals.

Effect of trisomy 21 on long-term gastrointestinal outcomes in duodenal atresia.

PURPOSE: We aimed to determine if Trisomy 21 (T21) affected gastrointestinal outcomes for children with duodenal atresia (DA). METHODS: We identified children born with DA between 1991 and 2017. Cases were divided into DA with T21 and DA without T21. Ten healthy controls per case were included. Esophageal, ulcerative, obstructive and stomach complaints were assessed. Risk ratios (RR), rate ratios (RaR) and Cox models were constructed. Analyses were performed for cases versus controls, and for T21 cases versus non-T21 cases. RESULTS: DA cases totaled 52: 22 had T21 and 30 did not. There were 520 controls. DA cases had more gastrointestinal complaints than controls. T21 cases were at greater risk and frequency of esophageal disease than non-T21 cases (RR = 4.08, p = 0.002, RaR = 69.8, p < 0.001). T21 and non-T21 cases were equally likely to present with obstruction (RR = 0.91, p = 1), but T21 cases complained of obstructive symptoms less (RaR = 0.57, p = 0.003). T21 and non-T21 cases had the same risk of stomach diseases, but T21 cases complained more frequently (RaR = 6.20, p < 0.001). Cox models supported these observations. T21 did not affect ulcerative diseases. CONCLUSION: DA cases had more gastrointestinal problems than controls. T21 increased esophageal and gastric complaints in DA cases but did not affect ulcerative and obstructive complaints.

Incidence of late severe intestinal complications after bowel atresia/stenosis.

BACKGROUND: To determine the incidence and risk factors for late severe intestinal complications after surgical repair for intra-abdominal congenital intestinal atresia/stenosis. METHODS: We included 51 patients who underwent surgical repair for congenital intestinal atresia/stenosis. Late severe intestinal complications included adhesive ileus, incisional hernia, or volvulus. Whether surgical intervention was urgent or not was recorded. The location of the atresia/stenosis was classified into two groups: atresia/stenosis located at the oral or anal side from the Treitz ligament. The type of atresia/stenosis was classified as low-risk types (type I, mucosal web/II, fibrous cord/IIIa, mesenteric gap defect) and high-risk types (IIIb, apple peel/IV, multiple atresia). We compared the incidence of late intestinal complications between the location of intestinal atresia/stenosis at the oral and anal side of Treitz ligament, and between low- and high-risk types of atresia/stenosis using Fisher's exact test. RESULTS: Eight (15.7%) had late intestinal complications, all of which occurred in patients with intestinal atresia/stenosis located on the anal side of the ligament of Treitz. Urgent surgical intervention was needed in four cases. There was a significant difference in the location of atresia/stenosis (with vs. without late intestinal complications at oral/anal side of the Treitz ligament: 0/8 vs. 24/19; P = 0.005) and the type of intestinal atresia/stenosis (with vs. without that accompanying low-/high-risk type: 5/3 vs. 41/2; P = 0.023). CONCLUSIONS: Physicians should consider the presence of intestinal complications that require surgical intervention in patients undergoing surgical reconstruction for jejunal and ileal atresia/stenosis with abdominal symptoms.

Predictors of Survival: A Retrospective Review of Gastroschisis and Intestinal Atresia in Rwanda.

INTRODUCTION: Neonatal surgical diseases are prime examples of the global disparity in surgical access and outcomes, with survival for conditions like gastroschisis reaching above 95% in high-income settings but usually fatal in low-income settings. This study aims to examine outcomes and predictors of mortality in patients with two specific neonatal surgical conditions that often require early transfer and prolonged inpatient care (gastroschisis and intestinal atresia) at Rwanda's main pediatric referral hospital. METHODS: A single-institution retrospective chart review of neonates with gastroschisis and intestinal atresia was conducted between January 2016 and June 2019. Abstracted data included demographics, referral history, admission interventions, operative details, in-hospital complications, nutrition patterns, length of stay, and mortality. Daily logs were created to evaluate feeding status, infection status, and antibiotic usage. Descriptive and univariate analysis was conducted, with the primary outcome being survival to hospital discharge. RESULTS: A total of 112 patients met inclusion criteria (82% gastroschisis [n = 92] and 18% intestinal atresia [n = 20]). Median age at arrival was 0 d (GS) [IQR 0-1 d] and 8.5 d (IA) [IQR 4-10 d] (P < 0.0001). Survival to discharge was 22.8% (GS) (n = 21) and 60% (IA) (n = 12) with a mean length of stay of 28.3 d (GS) and 18.4 d (IA). The median number of days to initiation of oral feeds was 8.5 d [IQR 7-11] for gastroschisis survivors. CONCLUSIONS: Neonatal surgical conditions that require early transfer and prolonged nutritional intervention are challenging in low-resource settings, but through treatment by a comprehensive pediatric surgical service, improving survival is possible.

Gastroschisis with intestinal atresia leads to longer hospitalization and poor feeding outcomes.

OBJECTIVE: Compare in-hospital outcomes in gastroschisis with intestinal atresia versus simple gastroschisis (GS) using a national database. STUDY DESIGN: The Children's Hospitals Neonatal Database identified infants with gastroschisis from 2010 to 2016. RESULTS: 2078 patients with gastroschisis were included: 183 (8.8%) with co-existing intestinal atresia, 1713 (82.4%) with simple gastroschisis, the remainder with complex gastroschisis without atresia. Length of hospitalization was longer for those with atresia, and yielded higher rates of mortality, medical NEC, and intestinal perforation. They began enteral feedings later, were less likely to initiate feeds orally, and reached full feedings later. They were less likely to be receiving any maternal breast milk or breastfeeding at discharge and more likely than simple gastroschisis to be discharged with a feeding tube. CONCLUSION: A large multicenter cohort showed gastroschisis with atresia results in worse outcomes and complications, including necrotizing enterocolitis, feeding delays, and enteral feeding tube dependence.

[Anorectal malformations: a 6-years review at the University Clinics of Lubumbashi]. / Malformations anorectales: revue de 6 ans aux Cliniques Universitaires de Lubumbashi.

Anorectal malformations (ARM) are developmental anomalies of the genitor anal elements, they represent a wide range of anomalies. An early diagnosis allows a better management as it reduces mortality associated with ARM, especially in developing countries. A prospective cross-sectional study has been carried, including patients from 0 to one year, admitted in our service for ARM. Twenty-four (24) patients have been considered. The median age was 2 days, the sex ratio 1/3 for female. Intestinal occlusions were the most frequent circumstances of diagnosis (50%), low ARMs were the most frequent, diagnosed in 11 patients (45.7%) of which 10 did not have any fistula. The most common associated malformation was intestinal atresia (3 patients). Anoplasty was done using abdominoperineal pull-through associated to anal dilatations in 13 patients (54.1%). Six patients died from unknown etiology and 6 were lost from the follow-up. Concerning the functional outcome, 12 of the 24 patients concerned by the study attended review and 3 of them presented signs of fecal incontinence, 9 of them were continents among whom 8 were diagnosed low ARM and one with high ARM. ARMs are encountered in our environment, nonetheless, the diagnosis is still made most of the time, during an intestinal occlusion. The mortality rate is still high and measures should be taken to allow long-term reviews, which will surely reduce the number of lost patients.

Laparoscopy-Assisted Versus Open Surgery in Treating Intestinal Atresia: Single Center Experience.

INTRODUCTION: Surgical management of jejuno-ileal atresia/stenosis (JIA) is shifting to a minimally invasive approach. Our purpose is to evaluate the safety and feasibility of laparoscopy-assisted surgery (LAS) in JIA by comparing outcomes with a control group of open surgery (OS). METHODS: A retrospective review of JIA cases was performed. Demographic, surgical, and outcomes data were extracted. LAS cases were compared with OS. Fisher's exact-test for qualitative and Mann-Whitney-test for quantitative values were used. p values <0.05 were considered significant. RESULTS: Forty-seven patients (24/23, F/M) were included. In 19 (40%), the LAS technique was successfully performed, while 3 (17%) required conversion to OS. No differences were observed between the LAS and OS (n = 28) groups concerning demographic data (sex, mean gestational age, mean weight, associated anomalies) and type of JIA. Operative time was shorter in LAS (112 ± 46 min) compared to OS (138 ± 40 min), p = 0.04. Time to start enteral feeding and time to full enteral was shorter in LAS compared to OS, p = 0.04. No difference was observed between the two groups concerning duration of parenteral nutrition, length of hospitalization and weight at discharge. Overall rate of postoperative complications was 14% (n = 7), with a slightly prevalence in OS (18%) compared to LAS (10%), p = 0.68. CONCLUSIONS: The LAS technique in the treatment of neonatal JIA is safe and feasible. LAS is associated with shorter operative and restoration of enteral feeding times. The post-operative outcomes in LAS are similar with OS, with a lower rate of postoperative complications.

Immune function and infectious complications in children with jejunoileal atresia.

OBJECTIVE: Little is known about differences in immune function among children with multiple intestinal atresia (MIA) and those with isolated intestinal atresia (IA), and how such differences may manifest as infectious complications and patient outcomes. This study aimed to investigate the immune function and its impact on patient outcomes in IA and MIA children. METHODS: A single-center retrospective cohort study included children aged 0-19 years with intestinal atresia who were referred to a multidisciplinary intestinal rehabilitation program from 1/2000 to 12/2016. Data were collected for patient characteristics, surgical history, immunologic work-up, and infection-related hospitalizations. Groups of IA and MIA children were compared using chi-square test or Fisher's exact test for categorical variables and using Mann-Whitney test for continuous variables, as appropriate. RESULTS: Twenty-seven children (18 IA, 9 MIA) were included. More than half of the patients had low CD counts for age in IA and MIA groups: CD3 58.3% vs. 66.7% (p = 1.0), CD4 50.0% vs. 66.7% (p = 0.7), CD8 67.7% vs. 88.9% (p = 0.3), respectively. Six out of 12 IA children and 3 out of 8 MIA children had hypogammaglobulinemia (p = 0.7). Three out of 10 IA patients and 3 out of 5 MIA children had frequent bacteremia (≥5/year). Eight children (6 IA and 2 MIA) underwent intestinal and/or liver transplant; MIA children had a worse posttransplant outcome. CONCLUSIONS: IA children may have an immunodeficiency and associated infectious complications requiring hospitalization. We suggest performing immunologic evaluation not only in MIA but also in IA children presenting to an intestinal rehabilitation program to identify immunodeficiency. Early immunodeficiency screening may help initiate appropriate intervention and improve patient outcomes. LEVEL OF EVIDENCE: Level III.

Congenital segmental intestinal anomalies in calves.

This focus article has been prepared by Alwyn Jones, veterinary investigation officer with SRUC Veterinary Services.

Incidence of Congenital Spinal Abnormalities Among Pediatric Patients and Their Association With Scoliosis and Systemic Anomalies.

BACKGROUND: Congenital abnormalities when present, according to VACTERL theory, occur nonrandomly with other congenital anomalies. This study estimates the prevalence of congenital spinal anomalies, and their concurrence with other systemic anomalies. METHODS: A retrospective cohort analysis on Health care Cost and Utilization Project's Kids Inpatient Database (KID), years 2000, 2003, 2006, 2009 was performed. ICD-9 coding identified congenital anomalies of the spine and other body systems. OUTCOME MEASURES: Overall incidence of congenital spinal abnormalities in pediatric patients, and the concurrence of spinal anomaly diagnoses with other organ system anomalies. Frequencies of congenital spine anomalies were estimated using KID hospital-and-year-adjusted weights. Poisson distribution in contingency tables tabulated concurrence of other congenital anomalies, grouped by body system. RESULTS: Of 12,039,432 patients, rates per 100,000 cases were: 9.1 hemivertebra, 4.3 Klippel-Fiel, 56.3 Chiari malformation, 52.6 tethered cord, 83.4 spina bifida, 1.2 absence of vertebra, and 6.2 diastematomyelia. Diastematomyelia had the highest concurrence of other anomalies: 70.1% of diastematomyelia patients had at least one other congenital anomaly. Next, 63.2% of hemivertebra, and 35.2% of Klippel-Fiel patients had concurrent anomalies. Of the other systems deformities cooccuring, cardiac system had the highest concurrent incidence (6.5% overall). In light of VACTERL's definition of a patient being diagnosed with at least 3 VACTERL anomalies, hemivertebra patients had the highest cooccurrence of ≥3 anomalies (31.3%). With detailed analysis of hemivertebra patients, secundum ASD (14.49%), atresia of large intestine (10.2%), renal agenesis (7.43%) frequently cooccured. CONCLUSIONS: Congenital abnormalities of the spine are associated with serious systemic anomalies that may have delayed presentations. These patients continue to be at a very high, and maybe higher than previously thought, risk for comorbidities that can cause devastating perioperative complications if not detected preoperatively, and full MRI workups should be considered in all patients with spinal abnormalities. LEVEL OF EVIDENCE: Level III.

Fish consumption in early pregnancy and congenital gastrointestinal tract atresia in the Japan Environment and Children's Study.

Current evidence suggests that the aetiology of congenital gastrointestinal (GI) tract atresia is multifactorial, and not based solely on genetic factors. However, there are no established modifiable risk factors for congenital GI tract atresia. We used data from a Japanese nationwide birth cohort study launched in 2011, and examined whether fish consumption in early pregnancy was associated with congenital GI tract atresia. We analysed data of 89 495 women (mean age at delivery=31·2 years) who delivered singleton live births without chromosomal anomalies. Based on the results of the FFQ, we estimated the daily intake of fish and n-3 PUFA consumption in early pregnancy. We defined a composite outcome (oesophageal atresia, duodenal atresia, jejunoileal atresia and/or anorectal malformation) as congenital GI tract atresia. In this population, median fish intake was 31·9 g/d, and seventy-four cases of congenital GI tract atresia were identified. Fish consumption in early pregnancy was inversely associated with the composite outcome (multivariable-adjusted OR for the high v. low consumption category=0·5, 95 % CI 0·3, 1·0). For all the specific types of atresia, decreased OR were observed in the high consumption category, although not statistically significant. Reduced atresia occurrence was observed even beyond the US Food and Drug Administration's recommended consumption of no more than 340 g/week. Also, n-3 PUFA-rich fish and n-3 PUFA consumptions tended to be inversely associated with atresia. Fish consumption in early pregnancy may be a preventive factor for congenital GI tract atresia.

Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature.

PURPOSE: To determine the true incidence of associated intestinal atresia (AIA) in infants with duodenal atresia (DA) and to analyze whether the surgical approach, open versus laparoscopic, would impact on patient outcome when AIA is present. METHODS: Cohort study We review all DA infants treated at our institution (2001-2016) and analyzed the outcome of those with AIA. Systematic review/meta-analysis Using a defined search strategy and according to PRISMA guidelines, two investigators independently identified all studies on DA and searched cases of AIA to determine its incidence. Data are mean ± SD. RESULTS: Cohort study Of 140 DA infants, 10 (7%) had AIA (4 type I, 4 type III, 2 type II). All type I AIA (webs) were found in the duodenum. Systematic review/meta-analysis Of 840 studies, 18 were included (2026 infants). The incidence of AIA was 2.8 ± 1.6%. The incidence of missed AIA was 0.8 ± 2.4%. Three comparative studies (759 infants) showed higher risk of missed AIA following laparoscopic (2.9 ± 2.4%) than open repair (0.3 ± 0.1%; p < 0.01). CONCLUSIONS: The incidence of AIA in DA infants is low and the risk of missing it is higher at laparoscopy than at laparotomy. Regardless the approach, surgeons should carefully investigate bowel continuity to avoid the risk of missing AIA.

A safe and efficacious preventive strategy in the high-risk surgical neonate: cycled total parenteral nutrition.

INTRODUCTION: Hepatic dysfunction in patients reliant on total parenteral nutrition (TPN) may benefit from cycled TPN. A concern for neonatal hypoglycemia has limited the use of cycled TPN in neonates less than 1 week of age. We sought to determine both the safety and efficacy of cycled TPN in surgical neonates less than 1 week of age. METHODS: A retrospective chart review was conducted on surgical neonates placed on prophylactic and therapeutic cycled TPN from January 2013 to March 2016. Specific emphasis was placed on identifying incidence of direct hyperbilirubinemia and hypoglycemic episodes. RESULTS: Fourteen neonates were placed on cycled TPN; 8 were prophylactically cycled and 6 were therapeutically cycled. Median gestational age was 36 weeks (34, 37). Sixty-four percent (n = 9) had gastroschisis. There was no difference between the prophylactic and therapeutic groups in incidence of hyperbilirubinemia > 2 mg/dL (3 (37%) vs 5 (83%), p = 0.08) or the length of time to development of hyperbilirubinemia [24 days (4, 26) vs 27 days (25, 67), p = 0.17]. Time on cycling was similar though patients who were prophylactically cycled had a shorter overall time on TPN. Three (21%) infants had documented hypoglycemia, but only one infant became clinically symptomatic. CONCLUSION: Prophylactic TPN cycling is a safe and efficacious nutritional management strategy in surgical neonates less than 1 week of age with low rates of hypoglycemia and a shorter total course of TPN; however, hepatic dysfunction did not appear to be improved compared to therapeutic cycling.

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.

Retrospective study of umbilical cord ulceration related to congenital intestinal atresia: A single-center report.

AIM: Umbilical cord ulceration (UCU) is a disease in which an ulcer forms in the umbilical cord in the pregnant uterus and is accompanied by hemorrhaging from the same site. UCU occurs in fetuses with congenital upper-intestinal atresia (CUIA); however, its onset mechanism remains unclear. Here, we report our investigation of cases of UCU in our hospital. METHODS: Among the 9825 deliveries performed between 2007 and 2016 at this hospital, 20 fetuses were diagnosed with CUIA, 4 (20%) of which had UCU. There was no difference in the backgrounds of the fetuses with UCU (UCU group: 4 fetuses) and those without (non-UCU group: 16 fetuses). RESULTS: There was no intergroup difference in gestational age at delivery. Four cases in the UCU group had maternal age 35 weeks (26-39), weeks of delivery 35 weeks (35-36) and weight 2178.5 g (1600-2640); three out of four fetuses were female; and the location of gastrointestinal obstruction was in the duodenum in one case and in the jejunum in three cases. Death occurred in three of four fetuses in the UCU group versus none in the non-UCU group. CONCLUSION: We performed a retrospective statistical investigation on the risk of UCU onset in cases from this hospital; however, we could not identify any prognostic factors for its onset. We investigated a total of 27 past reported UCU cases and the 4 cases in this study. Mean gestational age at onset was 33.3 ± 2.7 for all 27 cases. Various methods for the early discovery of UCU have been reported in the past; however, there is currently no gold standard. Based on this report and a review of past papers, for CUIA, it is desirable to perform in-hospital management from gestational week 30 onward and decide proper delivery timing on a case-by-case basis.

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.

Risk factors and short outcome of bowel atresia in neonates at Tertiary Hospital.

BACKGROUND: Bowel atresia is a rare congenital anomaly that could affect any part of the bowel. It is categorized based on the type and location of the affected bowel with jejunoileal being the most common type worldwide. Risk factors for developing intestinal atresia are not well established, but we know that genetics, poor prenatal care, and low birth weight are considered risk factors. PATIENTS AND METHODS: This is a case-control study conducted in King Abdulaziz Medical City tertiary hospital in Nursery Intensive Care Unit from 2004 to 2015 with a total number of 50 cases. Each case was matched with two control groups: a female and a male; the matching was based on the date of birth. RESULTS: Our study showed an interesting results with esophageal atresia being the most common type (n = 16, 32%) after that jejunoileal (n = 11, 22%). Both genders were affected almost equally with no gender predominance. Twenty-six (51%) of the cases were having below normal birth weight (odds ratio [OR] =0.07; 95% confidence interval [CI] =0.03-0.2). One mother of the control groups had cesarean section in comparison to 15 mothers from the cases (OR 42.4; 95% CI 5.4-333.09). The study also showed that there is no correlation between the number of pregnancies and the risk of atresia (P = 0.798) (OR 0.9; 95% CI 0.72-1.3). CONCLUSION: Unlike western countries, esophageal atresia was the most common type with no gender differences. Interestingly, both groups had high rates of low birth weight.