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NJBMS - Volume 1, Issue 4, April - June 2011

Pages: 234-236
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SEVERE FORM OF ADAM COMPLEX - INTRANATAL MALFORMATION

Author: B. NARASINGA RAO , M. PRAMILA PADMINI , R .RAMANA.RAO

Category: Anatomy

Abstract:

ADAM complex (Amniotic Deformities / Adhesions / Mutilations) is a rare poly-malformation syndrome with an incidence of 0.33 for every 1000 births in the literature with thoraco- and/or abdomino-schisis associated with an eventration of the internal organs and variable variations in the extremities. The present foetus diagnosed with ADAMcomplex showed placenta forming an operculum at the cranial pole, severe craniofacial defects, absence of right upper limb and malrotation of right lower limbs, complete malrotation of the circulatory, respiratory and gastro-intestinal systems .The novel thing in this case is the presence of malrotation which has not been reported before. The cerebral hemisphere was partially present. A rudiment of brain stem without any differentiation was present. The cerebellum and spinal segments were absent. Postmortem foetogram revealed undifferentiated components of axial skeleton. The mechanism responsible for the Adam complex is the presence of Amniotic Band during early embryonic period.

Keywords: ADAM complex, thoraco/ abdominoschisis, malrotation and Amniotic Band