Ueber Missbildungen mit Störungen des Körperverschlusses

Kermauner, Fritz

doi:10.1007/bf01981069

Cited by 23 publications

(2 citation statements)

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“…This is remarkable because it is most unlikely that the extremely small and thin purely epithelial membranes, which normally do not remain in embryos beyond a gestational age of 8.5 weeks (15 mm) [9], would become the large membranes observed in fetuses. The temporary character or the presence of remnants at birth [13,14] is, however, well in line with the defective amnion-like membrane.…”

Section: Discussionmentioning

confidence: 99%

“…The single phallic structures protruding from the urethral field were true, albeit defective, phalli pointing in a wrong, inward direction. The condition appears to be very rare, with the 1st case reported by Doran in 1881 [15] followed by only a few more or less unequivocal observations [8,13,14,16–21]. They appear to be unique for covered cloacal exstrophy, with the intraexstrophic position histologically proved by circumferential “urothelium” in some cases [15,21].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Common (“Classical”) and Covered Cloacal Exstrophy: A Histopathological Study and a Reconstruction of the Pathogenesis

2008

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Current opinion about structure and pathogenesis of cloacal exstrophy was challenged by histopathological findings and new insights into the normal development. Autopsy specimens of common (n = 3) and covered cloacal exstrophy (n = 4) with single intraexstrophic and -perineal phallic structures and perineo-exstrophic canals have been analyzed histopathologically. The findings were correlated to normal development to reconstruct the pathogenesis. By identifying a specific cloaca-derived urethra field as distinct from allantois-derived bladder fields, the exstrophic area is found to reflect the original hindgut configuration in embryos of approximately 26-29 postovulatory days gestational age (2-4 mm). Correlation to normal development suggests malfunctioning of the primitive streak/caudal eminence as a primary fault that leads to a defective cloacal region in the hindgut disturbing cloacal-intestinal-allantoic dissociation and also causes lengthening of the intestinal region into a blind-ending colon, teratoma-like lesions, and vertebral and muscular anomalies. The current idea that membranes in "covered cloacal exstrophy" represent persisting cloacal membranes is dismissed by finding an amnion-like structure, which suggests dysfunction of an umbilical ring placode as a simultaneous 2nd fault. This malfunctioning may cause omphalocele by defective demarcation of the umbilical cord and may replace midline stroma of the infraumbilical abdominal wall by extraembryonic tissue that stretches into a weak temporary membrane, may leave a perineo-extrophic canal, and may allow the formation of a single perineal or intraexstrophic phallus. Malfunctioning without replacement may result in a purely epithelial "allantoic" membrane, which by disintegrating in combination with the cloacal membrane will expose common cloacal exstrophy.

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Section: Discussionmentioning

confidence: 99%