Association of Cloacal Anomalies, Caudal Duplication, and Twinning

Cloacal anomalies exhibit a wide variety of morphologic types and accompanying clinical severity. The association of malformations of the cloaca with partial, complete, or conjoined twinning has been appreciated for some time, but, with the advent of prenatal ultrasound technology, appears to occur with a greater frequency than once thought. This observation has important implications for pathogenesis. We present 2 representative cases, a 19-week-old female fetus with duplication of several caudal structures and a 21-week-old male fetus with cloacal exstrophy variant and demised co-twin with lower abdominal wall defect, extruded intestinal tract, absent external genitalia, and imperforate anus. These findings and previously published theories suggest that certain models of monozygotic twinning may apply to the pathogenesis of cloacal anomalies. Specifically, the partial or complete duplication of the organizing center within a single embryonic disc may increase the risk of mesodermal insufficiency and thus account for the failure of complete development of the cloacal membrane and consequent exstrophy or other aberration.

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Limb Body Wall Complex with Sacrococcygeal Mass and Agenesis of External Genitalia

Case Reports in Medicine ◽

10.1155/2013/218626 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3

Author(s):

Prabahita Baruah ◽

Pradipta Ray Choudhury

Keyword(s):

Abdominal Wall ◽

Body Wall ◽

Abdominal Wall Defect ◽

External Genitalia ◽

Wall Defect

Limb body wall complex (LBWC) is a rare clinicopathological entity, characterized by the presence of an abdominal wall defect associated with variable spectrum of limb and visceral anomalies. A stillborn baby of LBWC with placentoabdominal phenotype is reported here. Kyphoscoliosis, sacrococcygeal mass and agenesis of external genitalia are the associated features.

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Cloacal Exstrophy Repair with Primary Closure of Bladder Exstrophy: A Case Report and Review of Literature

Case Reports in Pediatrics ◽

10.1155/2016/8538935 ◽

2016 ◽

Vol 2016 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

George Bethell ◽

Navroop Johal ◽

Peter Cuckow

Keyword(s):

Case Report ◽

Primary Closure ◽

Abdominal Wall Defect ◽

Bladder Exstrophy ◽

Inpatient Stay ◽

Review Of Literature ◽

Cloacal Exstrophy ◽

Wall Defect ◽

Staged Approach ◽

Reduced Risk

Cloacal exstrophy is the most complex congenital, ventral, abdominal wall defect. Traditionally surgery consists of a staged approach to repair which takes place on many separate theatre visits. In this case a primary approach was undertaken resulting in a relatively short inpatient stay and a reduced risk from multiple surgical procedures under general anaesthesia.

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Pentology of Cantrell: a rare case report with review of literature

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20182917 ◽

2018 ◽

Vol 7 (7) ◽

pp. 2962

Author(s):

Rajesh Tile ◽

Supriya Jamkhandi

Keyword(s):

Abdominal Wall ◽

Anterior Abdominal Wall ◽

Abdominal Wall Defect ◽

External Genitalia ◽

Unknown Etiology ◽

Review Of Literature ◽

Wall Defect ◽

Rare Congenital Disorder ◽

Antenatal Ultrasonography ◽

Rare Case Report

Pentology of Cantrell a rare congenital disorder of unknown etiology consists of pentad of: defect in the diaphragm, abdominal wall defect, deficiency of the pericardium and intracardiac defects. The hallmark diagnostic findings being ectopia cordis and omphalocoele. The exact pathogenesis is not clear, and the prognosis of this lethal condition depends on the severity of anterior abdominal wall defect and associated cardiac anamoly. Antenatal ultrasonography is a reliable tool of diagnosis and allow for termination of pregnancy if done before 20weeks of pregnancy. First described by Cantrell in 1958, very few cases have been reported till now in the literature. Authors describe here a case of a neonate delivered at 32weeks of gestation weighing 2.3kg at their hospital with typical features of Cantrells’ Pentology associated with a rare interesting finding of absent external genitalia and presence of lumbar meningocoele.

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