Of 41 patients with craniofrontonasal syndrome, 35 were female and 6 w
ere male, Although most cases were sporadic, 7 familial instances were
found, Craniofrontonasal syndrome represents a unique, incompletely u
nderstood X-linked disorder, Unusual manifestations in females include
d thick, wiry, and curly hair (49%), anterior cranium bifidum (6%), ax
illary pterygia (9%), unilateral breast hypoplasia (postpubertal; 11%)
, and asymmetric lower limb shortness (14%). (C) 1996 Wiley-Liss, Inc.