Insights into embryology and development of omphalocele

Khan, Faroque A.; Hashmi, Asra; Islam, Saleem

doi:10.1053/j.sempedsurg.2019.04.003

Cited by 41 publications

(30 citation statements)

References 23 publications

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“…In singletons, the etiopathogenesis of omphaloceles is still debated and causes are sought in aberrant formation and development of the ventral body wall, deviant formation of the amnion and/or the umbilical ring, or stagnant retractions of midgut structures (F. A. Khan, Hashmi, & Islam, 2019 ). In addition, malfunction of embryonic folding process due to defective cell–cell signaling at the critical transition point between the amnion and the ventral body wall or between the lateral and ventral body walls at the umbilical ring could interfere with ventral body wall formation, leading to separation of the left and right rectus abdominis and accompanying fascia (Brewer & Williams, 2004 ).…”

Section: Omphalocele and Oeis Complex In Ventrally And Caudally United Twinsmentioning

confidence: 99%

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Kim

Schepens‐Franke

Winter³

et al. 2021

Clinical Anatomy

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Shared anomalies, always located close to the area of coalescence and observable in virtually every type of conjoined twinning, are currently seen as separate anomalies caused by mostly unknown and seemingly unrelated pathways rather than being connected to the twinning mechanism itself. Therefore, most (case) reports about conjoined twins are mere descriptions of (external) dysmorphologies lacking reflections on the possible origin of their concomitant anomalies. As we will demonstrate in this article, shared anomalies are influenced, and in some cases solely and sequentially explained, by interaction aplasia and neo-axial orientation; two embryological mechanisms to which each set of conjoined twins is subjected and are responsible for their ultimate phenotypical fate.In this review, we consider how the ventral, lateral and caudal conjunction types and their intermediates determine the phenotypic presentation of the twins, including patterns of shared malformations and anomalies, which in themselves can be indistinguishable from those encountered in singleton cases. Hence, it can be hypothesized that certain anomalies in singletons originate in a fashion similar to that in conjoined twins.

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Section: Omphalocele and Oeis Complex In Ventrally And Caudally United Twinsmentioning

confidence: 99%

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Kim

Schepens‐Franke

Winter³

et al. 2021

Clinical Anatomy

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“…The exact mechanism for the development of omphalocele is not yet established. There are different theories explaining the failure of mesodermal closure when forming the abdominal wall that leaves a larger defect than the umbilical ring [7].…”

Section: Introductionmentioning

confidence: 99%

Omphalocele: national current birth prevalence and survival

et al. 2021

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Purpose The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients. Methods This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register. Results The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%. Conclusion The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high. Type of study National register study Level of evidence III.

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“…The midgut completed its rotation and returns to the abdominal cavity at the 10th WPC. Omphalocele is characterized by the failure of the physiological hernia to return to the abdominal cavity 17 . On the other hand, the cause of gastroschisis is not completely elucidated, but there is evidence of an abnormality in the formation and development of the ventral body wall during embryogenesis, resulting mainly in bowel herniation 18 .…”

Section: Discussionmentioning

confidence: 99%

Investigation of a connection between abdominal wall defects and severity of the herniation in fetuses with gastroschisis and omphalocele

Logsdon

Gallo

Favorito

et al. 2021

Sci Rep

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Analyze the biometric parameters and the size (area) of abdominal wall defect (AWD) in fetuses with gastroschisis and omphaloceles and correlate them with the herniated internal organs. We studied 22 fetuses (11 with AWDs and 11 without anomalies). In all fetuses we evaluated the xiphopubic distance (XPD) and iliac crest distance (ICD). In fetuses with AWDs we dissected the abdominal wall and measured the width and length of the defect for calculating its area and studying the correlation between the size of the defect with the organs that were herniated. For statistical analysis, the Anova and Tukey post-test were used (p < 0.05). The XPD in the control group had mean of 4.2 mm (2.3–5.9; SD ± 1.11), while in the AWDs it was 4.2 mm (2.9–5.5; SD ± 0.98) (p = 0.4366). The ICD had mean values of 2.5 mm (1.6–3.4; SD ± 0.58) in the control group, and 2.3 mm (1.2–3.0; SD ± 0.56) in AWDs fetuses (p = 0.6963). The number of herniate organs do not have significant correlation with the area of the defect (r2 = 0.2504, p = 0.5068). There is no correlation between the size (area) of abdominal wall defects and the number of the internal organs that herniated. Therefore, the hole size is not a predictor of the severity of the gastroschisis or omphalocele.

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Insights into embryology and development of omphalocele

Cited by 41 publications

References 23 publications

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Omphalocele: national current birth prevalence and survival

Investigation of a connection between abdominal wall defects and severity of the herniation in fetuses with gastroschisis and omphalocele

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