Cluster of Iniencephaly in Miami

Abstract

This report details a cluster of 5 cases of iniencephaly with anencephaly and rachischisis occuring over a 4-month period at Jackson Memorial Hospital/University of Miami Medical Center in Miami, Florida. All 5 cases of this rare, lethal, congenital malformation seen in the cluster included diaphragmatic defects with accompanying hernia, omphalocele, small adrenals, renal dysmaturity, gastrointestinal malformations, cleft lip and palate, and hypoplastic lungs. No single causative agent for this cluster was identified. A brief review of the literature regarding categorization of these malformations and as a discussion of the embryological basis for these lesions and possible etiologic factors are included.

Key Words:

• anencephaly
• congenital malformations
• iniencephaly
• neural tube defects
• rachischisis