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Abnormal Umbilical Vessels and Systemic Circulatory Reversal in Thoracopagus Twins

Published online by Cambridge University Press:  01 August 2014

B.D. Chaurasia*
Affiliation:
Department of Anatomy, G. R. Medical College, Gwalior, MP, India
*
Department of Anatomy, G.R. Medical College, Gwalior, M.P., India

Abstract

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A human thoracopagus of slightly unequal twins having a common umbilical cord with exomphalos is described, with special reference to the cardiovascular system. The larger twin had single umbilical artery. The smaller twin had a large left umbilical artery which was in direct continuity with the aorta, a small right umbilical artery with sole connection to the right external and internal iliac arteries (right common iliac artery was absent), missing umbilical vein, a rudimentary nonfunctional heart with atresia of the truncus arteriosus, and a right-sided aortic arch. Exomphalos of both twins is accounted by the possible defective growth of umbilical mesoderm caused by abnormal umbilical vessels. Reversal of systemic circulation in the upper part of the body of smaller twin is discussed. The importance of a careful study of umbilical veins is emphasized.

Riassunto

RIASSUNTO

Viene descritto un caso di gemelli toracopagi con cordone ombelicale unico, lievemente diversi con particolare riguardo al sistema vascolafe. Il gemello piú grosso presentava arteria ombelicale singola, mentre il piú piccolo presentava una grossa arteria ombelicale sinistra in diretta continuità con l'aorta, una piccola arteria ombelicale destra connessa soltanto alle arterie iliache esterna ed interna di destra (l'arteria iliaca comune destra era assente), ed in piú l'assenza della vena ombelicale, un cuore rudimentale non funzionale con atresia del tronco arterioso, e un arco aortico piegato a destra. La prominenza ombelicale può essere spiegata da un difettoso accrescimento del mesoderma ombelicale a causa dell'alterata vascolarizzazione. Viene inoltre discussa l'in-versione circolatoria nel gemello più piccolo e viene sottolineata l'importanza di un attento studio delle vene ombelicali.

Résumé

RÉSUMÉ

Description d'un cas de jumeaux thoracopages légèrement différents, avec cordon ombilical unique. Le plus gros des jumeaux présentait une artère ombilicale simple, alors que le plus petit présentait une grosse artère ombilicale gauche en continuité avec l'aorte, une petite artère ombilicale droite en connexion avec les artères iliaques externe et interne droites (l'artère iliaque commune droite étant absente) et en plus, l'absence de la veine ombilicale, un coeur rudimentaire non-fonctionnel avec atrésie du tronc artérieux, et un arc aortique vers la droite. La proéminence ombilicale peut être expliquée par un défaut de développement mésodermique ombilical à cause de la vascularisation altérée. L'inversion circulatoire dans le plus petit des jumeaux est discutée et l'importance d'un étude des veines ombilicales est soulignée.

Zusammenfassung

ZUSAMMENFASSUNG

Beschreibung eines Thorakopagus mit nur einer Nabelschnur und leichten, vor allem das Gefässystem betreffenden Unterschieden zwischen den beiden Zwillingen. Der grössere Zwilling hatte nur eine einzige Nabelarterie. Bei dem kleineren hingegen war die linke Nabelarterie gross und ging direkt in die Aorta über, die rechte war klein und nur mit den rechten AA. iliacae (externa und interna) verbunden, während die gemeinsame rechte A. iliaca fehlte. Es fehlte auch die Nabelvene, das Herz war rudimentär und nicht funktionell mit Atresie des Arterienstammes (Truncus arter.), der Aortenbogen nach rechts verbogen. Das Hervorstehen des Nabels erklärt sich vielleicht daraus, dass das Nabelkeimblatt aufgrund der veränderten Gefässverhältnisse nicht regulär gewachsen ist.

Type
Research Article
Copyright
Copyright © The International Society for Twin Studies 1975

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