Ileal atresia

BACKGROUND/OBJECTIVE: Small bowel atresia commonly causes neonatal intestinal obstructions. Technical problems are associated with the surgical management of atresia using primary end-to-end anastomosis. Furthermore, the significantly dilated proximal loop may be associated with the stasis of intestinal fluid, thus increasing pressure on the anastomosis and prolonging fasting time before initiation of oral intake. This study aimed to perform antimesenteric tapering of the proximal loop using a linear stapler to reduce its diameter and facilitate anastomosis with the distal loop...

Intestinal obstruction is one of the most common surgical emergencies in the neonatal period. Early diagnosis is vital for proper management and good outcome. Intestinal obstruction can be divided into high, for example, duodenal atresia and jejunal atresia, or low, for example, ileal atresia, colonic atresia, and Meckel's diverticulum. The most common cause of intestinal obstruction in neonates is midgut atresia. Surgical correction is needed and is a challenge in the developing countries where there is lack of pediatric surgeons, anesthesiologists, and intensive care...

INTRODUCTION: Biliary atresia (BA) is a rare, non-curable cholestasis-causing disease in infancy, due to progressive ascending bile duct sclerosis. Even after restoration of bile flow following Kasai portoenterostomy, about half of these children need a liver transplant by their 2nd birthday, due to progressive fibrosis. Toxicity of bile acids may play a central role in disease progression, but drug therapies are not yet available. With ileal bile acid transporter (IBAT) inhibitors, there is a potential novel drug option that inhibits the absorption of bile acids in the small intestine...

The ileal loops are anatomical location for the majority of congenital anomalies affecting the gastrointestinal tract. These include Meckel's diverticulum, ileal duplication, dysgenesis, atresia, mucosal diaphragm, and malposition of the ileum. Symptomatic lesions that often present with abdominal pain, intestinal obstruction or bleeding are usually diagnosed and treated during infancy and childhood. However, many of these congenital conditions may remain clinically silent and detected incidentally in adults undergoing radiological evaluation for unrelated medical reasons...

STUDY QUESTION: Can mutations of genes other than AMH or AMHR2, namely PPP1R12A coding myosin phosphatase, lead to persistent Müllerian duct syndrome (PMDS)? SUMMARY ANSWER: The detection of PPP1R12A truncation mutations in five cases of PMDS suggests that myosin phosphatase is involved in Müllerian regression, independently of the anti-Müllerian hormone (AMH) signaling cascade. WHAT IS KNOWN ALREADY: Mutations of AMH and AMHR2 are detectable in an overwhelming majority of PMDS patients but in 10% of cases, both genes are apparently normal, suggesting that other genes may be involved...

Small bowel atresia constitutes congenital obstruction of the lumen of the duodenum, jejunum or ileum, and is one of the most common causes of neonatal bowel obstruction with a reported incidence of between 1.3 and 2.8 per 10,1000 live births. Complete absence of the small bowel, or near total jejuno-ileal atresia (in the absence of malrotation or gastroschisis), are extremely rare. Mid-trimester prenatal sonographic finding of dilated fetal bowel led to the finding of interstitial 8q21.13q21.2 duplication...

PURPOSE OF REVIEW: Cholestasis in infants can indicate a serious hepatobiliary disease and requires timely assessment, diagnosis and intervention to prevent progression to serious liver decompensation. This report aims to highlight recently published studies regarding diagnosis and treatment of cholestasis in infants. RECENT FINDINGS: The evaluation of neonatal cholestasis can be challenging, requiring the assessment of a broad differential diagnosis in timely fashion...

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...

Fetal gastrointestinal tract obstruction (GITO) is the most frequently encountered gastrointestinal defect in the prenatal period. This study aimed to investigate the genetic disorders and pregnancy outcomes of fetal GITO. We reviewed data from 70 pregnancies that were referred for invasive prenatal testing because of fetal GITO. According to the level of obstruction, they were classified into esophageal atresia/stenosis, duodenal atresia/stenosis, jejunal or ileal atresia/stenosis, or anal atresia. Traditional karyotyping was performed on all the 70 pregnancies, and chromosomal microarray analysis (CMA) was performed on 32 of them in parallel...

Background: Gastrointestinal (GI) malformations have varied short-term and long-term outcomes reported across various neonatal units in India. Methods: This descriptive study was done to study the clinical profile, outcomes and predictors of mortality in neonates operated for congenital GI malformations in a tertiary neonatal care unit in South India between years 2011 and 2020. Details were collected by retrospective review of the case sheets. Results: Total of 68 neonates were included with esophageal atresia (EA) in 10, infantile hypertrophic pyloric stenosis (IHPS) in 9, duodenal atresia (DA) in 10, ileal atresia in 8, jejunal atresia in 5, anorectal malformations (ARM) in 11, meconium ileus/peritonitis in 9, malrotation in 2, and Hirschsprung's disease (HD) in 4...

Jejunoileal atresia (JIA) is one of the common etiologies of intestinal obtrusion in neonates. However, cases of concomitant ileal atresia and Hirschsprung disease (HD) rarely occur. We report the case of a male infant who had JIA concomitantly with HD that was re-anastomosed. The patient underwent an exploratory laparotomy to resect the dilated terminal ileum. Subsequently, owing to a significantly dilated proximal bowel, he underwent a second exploratory laparotomy. However, he continued to have feeding intolerance postoperatively...

Anorectal malformations and jejunoileal atresias are common causes of intestinal obstruction in neonates. Both have their own set of associated anomalies but it is extremely rare for the two to co-occur in the same patient. In this case report, we detail and describe this unusual incidence in a three-day-old neonate who was provisionally diagnosed with a case of simple imperforate anus. Per-op findings showed a type 4 Ileal atresia and an ileostomy was then created. Our experience stresses the importance of timely antenatal diagnosis and the presence of a high index of suspicion when encountering such patients...

Background Anatomical abnormalities leading to bowel movement failure are the major cause of intestinal obstruction. This study was done to assess the diagnostic efficacy of contrast enema in neonates with lower intestinal obstruction. Methodology This prospective study was conducted in The Children's Hospital and University of Child Health Sciences, Lahore from February 2021 to July 2021. Patients presenting with constipation, abdominal distension, vomiting, and unable to pass meconium, evaluated clinically and by X-rays, were included in the study...

BACKGROUND: Silicone-covered endoluminal stents have been applied to various hollow visceral disorders in adult patients with varying success. Efficacy of retrievable endoluminal stenting in children is less well-established. PURPOSE: The purpose of this study was to evaluate our experience with evolving applications of endoluminal silicone-covered stenting in children. RESEARCH DESIGN: Eight children 19 years and younger having silicone-covered stent placement for various indications at a single institution (2014-2021) were reviewed retrospectively...

INTRODUCTION: Intestinal atresia more common in the small bowel, apart from large intestine. Jejunal atresia characterized by complete occlusion of the intestinal lumen, is a rare congenital anomaly occurring in 1 in 12,000 live births. IMPORTANCE: The jejunal atresia can be single or multiple occurring anywhere from the ligament of Treitz to the jejuno-ileal junction, requiring immediate surgical attention to prevent mortality and morbidity among these neonates...

BACKGROUND: Mesenteric cysts are rare intra-abdominal masses and high index of suspicion is required to clinically suspect this pathology and make a definitive diagnosis. They are most commonly located in the ileal mesentery but can be located anywhere along the gastrointestinal tract mesentery and may extend into the retroperitoneum. The rarity of these anomalies and diversity of clinical picture pose challenges in the diagnosis and operative management. The objective of this study is to determine the demographic pattern, clinicopathological diagnosis and management outcome of mesenteric cysts in our hospital...

BACKGROUND: Bifidobacterium breve is widely used as a probiotic in preterm infants and children with congenital surgical conditions, however, some cases of probiotics-induced bacteremia have been reported recently. OBJECTIVES: To examine the clinical and bacteriologic features of Bifidobacterium breve bacteremia caused by a probiotic (BBG-01) in term and preterm infants. METHODS: We included 298 patients who were admitted to the neonatal intensive care unit of Miyagi Children's Hospital and were given BBG-01 as a probiotic within the period June 2014 to February 2019...

Maralixibat (Livmarli™) is an orally-administered, small-molecule ileal bile acid transporter (IBAT) inhibitor being developed by Mirum Pharmaceuticals for the treatment of rare cholestatic liver diseases including Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC) and biliary atresia. Maralixibat received its first approval on 29 September 2021, in the USA, for use in the treatment of cholestatic pruritus in patients with ALGS 1 year of age and older. Maralixibat is also under regulatory review for ALGS in Europe, and clinical development for cholestatic liver disorders including ALGS in patients under 1 year of age, PFIC and biliary atresia is continuing in several other countries...

BACKGROUND: Twin-to-twin transfusion syndrome (TTTS) is a significant cause of perinatal morbidity and mortality. Fetoscopic laser photocoagulation (FLP) is the optimal treatment option for twin-to-twin transfusion syndrome; but can cause central nervous system, extremity and intestinal system injury. CASE: We report the case report of ileal atresia and severe cerebral infarction co-occurrence after fetoscopic laser photocoagulation treatment. It is uncertain as to whether ileal atresia occurred due to ischemia associated with TTTS, the treatment with FLP, or a combination of both...

AIMS: Congenital colonic stenosis (CCS) is an extremely rare cause of low-intestinal obstruction in neonates/child. We report our experience with seven cases of CCS presenting with low-intestinal obstruction and diagnosed intraoperatively and also propose an algorithm for its appropriate treatment for the adequate outcome. MATERIALS AND METHODS: It was a retrospective study of seven patients of CCS including two neonates (5-days and 15-days old), four infants (age range - 2-11 months), and one 24-month-old child admitted from 2014 to 2019...