During a four year period (1991-1994), 19 cases of iniencephaly were e
valuated at our institution. Associated cranial malformations include
anencephaly in 15 (79%), and posterior encephalocele in 5 (26.3%) of t
he cases. Other associated malformations include diaphragmatic defects
with a accompanying hernia, omphalocele, gastrointestinal malformatio
ns, cardiovascular and genitourinary malformations, single nostril, fa
cial cleft, spina bifida, hypoplastic lungs, club foot and single umbi
lical artery. No single causative agent for this group was identified.
A brief review of the literature is included regarding categorization
of these malformations and also a discussion of the embryological bas
is for these lesions and possible etiological factors.