Incomplete bilateral transverse facial cleft--a previously unreported associated defect of the Kallmann's syndrome.
In 1944 Kallmann et al described sex-linked inheritance of hypogonadotropic hypogonadism with anosmia and noted the co-occurrence cleft lip, cleft palate and cranio-facial asymmetry. Since then, other clinical cases associated with various skeletal, ophthalmic, urogenital, cardiovascular and central nervous system disorders have been reported. This article presents the case of a 17-year-old girl with a bilateral incomplete facial cleft in association with the Kallmann's syndrome, a previously unreported connection. We describe the plastic surgical reconstruction of the bilateral incomplete facial cleft. The mechanism of this embryopathologic association of the Kallmann's syndrome is, we suggest, due to a developmental disturbance in the region of the median forebrain organizer and associated structures, possibly in connection with a pleiotropic genetic mechanism.
Hönig
J F
JF
Department of Maxillo-Facial Surgery, University Hospital and Medical School, Göttingen, Germany.
D000293:Adolescent; D005260:Female; D006801:Humans; D017436:Kallmann Syndrome; D009056:Mouth Abnormalities / Q000601:surgery*