Apple peel atresia

BACKGROUND AND OBJECTIVE: Although complex atresias, such as apple-peel and multiple atresias, comprise a smaller percentage, they are usually associated with a higher incidence of postoperative complications and mortality rate. Contrary to simple atresias where the surgical technique of choice usually entails bowel resection and anastomosis with or without enteroplasty, managing apple-peel atresia remains more sophisticated. Decompressive and functionalizing stomas are sometimes mandatory to overcome problems such as increased wall thickness and the wide disparity among the anastomotic ends...

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...

OBJECTIVE: This study explored the feasibility of mesoplasty with end-to-side anastomosis in the treatment of different apple-peel mesenteric defects with high jejunal atresia. METHODS: A retrospective analysis was performed on 42 premature infants admitted to the hospital between 2014 and 2021. Prenatal ultrasound scans revealed bowel dilatation. The patients experienced vomiting after birth and produced white or no meconium. Plain radiography showed double or triple bubble signs and the patients underwent emergency laparotomy...

BACKGROUND: Gastroschisis is a closure defect of the abdominal wall classified as complex when it presents with necrosis, volvulus, or atresia of the gastrointestinal tract. Jejunoileal atresia is caused by abnormal closure, discontinuity, or narrowing of the intestine. Apple Peel or type IIIb is the rarest presentation, with an incidence of 1.3 per 10,000 live births. In addition to presenting a high mortality rate. PRESENTATION OF CASE: We present a preterm newborn patient of 30 weeks with a diagnosis of gastroschisis and jejunoileal atresia type IIIB...

INTRODUCTION: Duodenal atresia associated with apple peel is extremely rare. Duodenal atresia occurs as a result of absence of recanalization at an early stage, whereas intestinal atresia is seemingly due to vascular causes at later stages. The presence of abnormalities associated with diaphragmatic hernia is frequent, but association with duodenal atresia has been little explored. CASE REPORT: This is the case of a female neonate born at gestational week 31, with duodenal atresia and apple peel, associated with left diaphragmatic hernia and major heart disease...

The beta-actin gene (ACTB) encodes a ubiquitous cytoskeletal protein, essential for embryonic development in humans. De novo heterozygous missense variants in the ACTB are implicated in causing Baraitser-Winter cerebrofrontofacial syndrome (BWCFFS; MIM#243310). ACTB pathogenic variants are rarely associated with intestinal malformations. We report on a rare case of monozygotic twins presenting with proximal small bowel atresia and hydrops in one, and apple-peel bowel atresia and laryngeal dysgenesis in the other...

No abstract text is available yet for this article.

One-third of all intestinal obstructions in the newborn are caused by atresias. The most common site is the duodenum followed by jejunoileal and colonic locations. Herein we report the autopsy findings of a rare case of jejunoileal atresia associated with malrotation of gut. Autopsy performed on a 36 weeks old male fetus still birth, born of a non-consanguineous marriage, demonstrated jejunoileal atresia with apple peel deformity and malrotation of gut. Although the diagnosis was established in the prenatal period, in utero fetal demise occurred before definitive surgical intervention could be done...

OBJECTIVE: Bowel obstructions beyond the duodenum represent a heterogeneous group of congenital anomalies with a highly variable prognosis, the main issue being postnatal short bowel syndrome (SBS). The objective of our study was to evaluate the contributions of fetal MRI in cases of bowel obstruction. MATERIALS AND METHODS: A retrospective analysis of all newborns, for whom both ante-natal ultrasound and fetal MRI were available, referred to our center for suspected bowel obstruction was performed...

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...

Strømme syndrome is a rare autosomal recessive congenital disorder involving multiple systems. Centromeric protein F ( CENPF ) is the causative gene of the disease, and variants are usually linked to lethal outcomes either during the foetal stage or in early life. We present a young adult with a genetic diagnosis of Strømme syndrome who-in addition to classic microcephalia, microphthalmia and intestinal atresia (apple peel-type)-experienced slow and unexpected evolution to end-stage renal disease (ESRD). In conclusion, Strømme syndrome is a complex multiorgan disease that needs multidisciplinary clinical management, and potential evolution to ESRD should be taken into account...

No abstract text is available yet for this article.

This report describes siblings with Stromme syndrome, a rare genetic condition that primarily presents with a triad of intestinal atresia, cranial and ocular malformations, and other organ systems could be involved. This clinical triad was initially named after the first person to describe it in 1993. Here, we report a family with two siblings who presented with unusual intestinal atresia and ocular and CNS abnormalities. The first patient is a 6-year-old-boy with apple peel duodeno-jejunal atresia, unilateral microphthalmia and microcephaly...

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...

Apple peel intestinal atresia is a rare congenital malformation. It consists of a proximal jejunum ending in a blind pouch and distal small bowel wrapped around its vascular supply in a spiral fashion. A combination of type IIIb jejunoileal atresia (apple peel atresia) and type IV (multiple intestinal atresias) is a rare entity. The diagnosis and management of such complicated cases is a challenge, especially in resource-limited settings. We report a case of a four-day-old female who presented to the neonatal intensive care unit with complaints of vomiting, yellow discoloration of the skin, and failure to pass meconium since birth...

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...

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...

BACKGROUND: The sonographic whirlpool sign of volvulus due to midgut malrotation is well recognized. However, variations of the whirlpool sign may be seen in other conditions, but this observation has received little attention in the literature. OBJECTIVE: This study presents a series of neonates with a variety of causes of congenital intestinal obstruction, all associated with a whirlpool sign (unrelated to midgut volvulus), which was correctly recognized preoperatively on sonography...

Stromme syndrome.Jejunal atresia with microcephaly and ocular anomalies.Apple peel syndrome with microcephaly and ocular anomalies.Ciliopathy phenotype.Primary microcephaly and intellectual disability.OMIM# of the disease 243605.Name of the analysed genes or DNA/chromosome segments CENPF.OMIM# of the gene(s) 600236.Review of the analytical and clinical validity as well as of the clinical utility of DNA-based testing for mutations in CENPF genes in diagnostic, prenatal settings, and for risk assessment in relatives...

This is a case report of a neonate who was antenatally diagnosed with jejunal atresia which turned out to be duodenal atresia with apple peel syndrome. A previous sibling, who also had apple peel but with jejunal atresia, succumbed to sepsis after surgery. The first sibling had jejunal stenosis and had died of sepsis following surgery. Combination of duodenal atresia with apple peel is extremely rare. This coupled with a familial condition is rarer still. This case was challenging due to the short length of the gut and prolonged need for total parenteral nutrition and sepsis in postoperative period...