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Congenital atresia and stenosis of the intestine
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Sharon G. Cox, Alastair J.W. Millar, Alp Numanoglu
A much more severe condition, usually termed (hereditary or familial) multiple intestinal atresia, is characterized by a multiplicity of Type I (membranes) throughout the small bowel and also sometimes associated with a pyloric atresia. This is due to a genetic mutation (tetratricopeptide repeat domain of 7A gene). Affected infants invariably have a combined immunodeficiency and are only ever effectively treated by bone marrow and small bowel transplant.
Congenital and Perinatal Anomalies of the Gastrointestinal Tract
Published in Asim Kurjak, CRC Handbook of Ultrasound in Obstetrics and Gynecology, 2019
Fetal bowel obstruction may be secondary to a congenital malformation such as intestinal atresia, but may also be caused by meconium ileus, duplication of bowel, volvulus, and rupture of bowel wall. Atresias are most commonly encountered type of fetal bowel obstruction, occurring in 1 of every 2700 live births. The site of obstruction is the ileum in more than one half of cases, followed by, in the descending order of occurrence, the duodenum, jejenum, and colon (Figure 1).
Intestinal atresia and stenosis
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Jason S Frischer, Richard G Azizkhan
From the list of options above, choose which is the most appropriate for each of the following scenarios regarding the aetiology and genetics of jejunoileal atresia. Each option may be used once, more than once, or not at all. A full-term neonate is born with a jejunal atresia. His mother suffered from severe migraine headaches during the pregnancy.A neonate is born with congenital immunodeficiency.A newborn is noted to have a small-intestinal atresia with no other associated anomalies.Supports the well-accepted theory that jejunoileal atresia is most likely due to a vascular accident and occurs later in gestation.
Segmental Dilatation of Ileum Involving Bronchogenic Cyst in a Newborn
Published in Fetal and Pediatric Pathology, 2023
Özkan Okur, Malik Ergin, Akgun Oral, Munevver Hosgor
A 2-day-old 3300 g boy, born at 38 weeks of gestation, presented with bilious vomiting. There were no complications during his gestation. Prenatal history was unremarkable. The patient, who had meconium passage 12 h after birth, had a non-tense but massively distended abdomen. The right testis was not palpable. Laboratory values were within normal limits. Conservative management was started with intravenous fluids and antibiotics and nasogastric decompression. By radiograph, there was an air-fluid level in the entire abdomen with sharp borders resembling pneumoperitoneum(Fig. 1). The clinical picture was difficult to distinguish from more common causes of obstruction such as intestinal atresia or duplication. Ultrasound examination revealed abundant free fluid in the abdomen and no intra-abdominal cyst was detected. In the subsequent surgical exploration, a dilated jejunoileal segment of 10 cm in length and 8 cm in diameter, six times the normal bowel diameter on both sides, was detected 25 cm distal to the ligament of Treitz and 55 cm proximal to the cecum (Fig. 2). The dilated segment was filled with intestinal contents, and was in continuity with normal caliber afferent and efferent small intestine. There was no malrotation or Meckel’s diverticulum. Treatment was with segmental resection of the dilated segment and end-to-end anastomosis.
Prepregnancy overweight and obesity as risk factors for birth defects: a cross-sectional study over a 30-year period
Published in Journal of Obstetrics and Gynaecology, 2022
Carolina Bicudo Borrelli, Sirlei Siani Morais, Mariane M. Barbieri, Thayane Leme, Jessica Fernanda Teixeira Prado, Fernanda G. Surita
Neonatal outcomes were worse in the BD group and it was expected since some BD can be very severe. Researchers found that foetuses with BD had higher chances of being born preterm and with low weight than foetuses without BD. Preterm birth is associated with oesophageal and intestinal atresia, for example. The complex association of genetic and epigenetic factors could cause BD and growth restriction, like a vascular compromise (Miquel-Verges et al. 2015). We found that in our BD group the prevalence of low birth weight was higher, consistent with published data(Montes-Núñez et al. 2011; Cosme et al. 2017; Luz et al. 2019). Newborns with congenital heart defects have twice the risk of being born small for gestational age and this is maybe due to the severity of the disease (Miquel-Verges et al. 2015; Luz et al. 2019). BD also shown an association with preterm birth (Montes-Núñez et al. 2011). Research has suggested that the introduction of the prenatal screening program led to a decrease in perinatal mortality in infants with BD (Bardi et al. 2021) but this is not accessible for a large part of the population so we need to focus on prevention, like modifiable risk factors.
Delayed development of vacuoles and recanalization in the duodenum: a study in human fetuses to understand susceptibility to duodenal atresia/stenosis
Published in Fetal and Pediatric Pathology, 2022
Xuelai Liu, Yanbiao Song, Peiyu Hao, Xinghai Chen, Jun Zhang, Yandong Wei, Xianghui Xie, Long Li, Zhe-Wu Jin
Intestinal atresia (IA) and intestinal stenosis (IS) are congenital gastrointestinal malformations that cause intestinal obstruction requiring surgery in neonates, with a prevalence of approximately 1:1500–1:2000 [1]. During mid-gut development, the normal intestinal tract contains many vacuoles at embryonic weeks 6 to 7; these vacuoles expand and merge in the embryonic lumen obstructing, gradually recanalizing and forming the intestinal lumen [2–4]. The failure of vacuole-induced local recanalization keeps the intestinal duct occluded, leading to IA or IS in the fetus [5-]7 .IA and IS can occur throughout the entire intestinal tract, including the duodenum, jejunum, ileum, colon, and rectum, leading to atresia or stenosis in each of these anatomic locations. The frequency of duodenal atresia/stenosis is higher than that of jejunal/ileal atresia or stenosis, whereas colonic and rectal atresia/stenosis are infrequent [8–10]. This suggests that the frequency of atresia/stenosis decreases from the proximal to the distal bowel.