The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with the VATER association. Most interest has been centered on the foregut, where the model has clarified some aspects of the development of esophageal atresia (EA), tracheal agenesis, and other communicating bronchopulmonary foregut malformations. It has demonstrated aberrations in the nerve supply to the esophagus in EA and allowed the study of tracheomalacia. A relationship between an abnormal notochord, foregut abnormalities, and vertebral defects has been shown, and the model has reignited interest in the role of the notochord as a regional organizer of axial development. The normal temporospatial characteristics of apoptosis during fore- and hindgut development is disturbed in this model, resulting in abnormal morphology. The indications are that this model will continue to clarify the processes that lead to many of the structural congenital abnormalities that are seen in infants born with the VATER association.
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http://dx.doi.org/10.1007/s003830000426 | DOI Listing |
Health Sci Rep
December 2024
Department of Pediatric Surgery, New Children's Hospital Helsinki University Hospital and University of Helsinki Helsinki Finland.
Purpose: To evaluate possible problems during pregnancy or delivery in women with pediatric bladder augmentation.
Methods: Eleven of 59 women, who had undergone bladder augmentation in our pediatric hospital during 1990-2019, had given birth in our hospital district afterwards and their obstetrical records were evaluated.
Results: Median age at first delivery was 32 years (range 26-42).
Cureus
August 2024
Gastroenterology and Hepatology, Aberdeen Royal Infirmary Hospital, Aberdeen, GBR.
The VACTERL/VATER association is a rare congenital disorder characterized by the presence of at least three of its main components: vertebral defects, anal atresia, cardiovascular anomalies, tracheoesophageal fistulas, esophageal atresia, renal anomalies, and limb defects. The exact cause of the VACTERL association is not fully understood. Most cases occur randomly.
View Article and Find Full Text PDFChildren (Basel)
May 2023
Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, LMU Munich University, 80337 Munich, Germany.
VACTERL association is a rare malformation complex consisting of vertebral defects, anorectal malformation, cardiovascular defects, tracheoesophageal fistulae with esophageal atresia, renal malformation, and limb anomalies. According to current knowledge, VACTERL is based on a multifactorial pathogenesis including genomic alterations. This study aimed to improve the understanding of the genetic mechanisms in the development of VACTERL by investigating the genetic background with a focus on signaling pathways and cilia function.
View Article and Find Full Text PDFJ Med Case Rep
December 2022
Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan.
Pediatr Rep
April 2021
Department of Paediatric Surgery, Children's Hospital for Wales, Cardiff CF14 4XW, UK.
We present a case series of two patients with tracheo-oesophageal fistula with oesophageal atresia (TOF/OA), duodenal atresia (DA) and ano-rectal malformation (ARM). This constellation of abnormalities, dubbed triple atresia (TA), is a rare combination with few described cases in the literature. Here we describe our management of these cases, as well as the results of our literature review.
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