Median cleft lip without holoprosencephaly. Case report.
A patient with median cleft lip is reported, but the patient showed no evidence of holoprosencephaly, which was ruled out by MRI. When the neonate's head circumference is within two standard deviations of the mean and normotelorism occurs with median cleft lip, it can be assumed that the brain is not holoprosencephalic. The importance of this cannot be overemphasized because median cleft lip is commonly associated with holoprosencephaly and severe microcephaly. The mother developed overt diabetes mellitus after the patient was born. However, infants of diabetic mothers are known to be at increased risk of giving birth to infants with holoprosencephaly. Since a prediabetic woman may sometimes give birth to large and/or malformed infants before developing overt diabetes mellitus, it is intriguing to speculate that the prediabetic state might have resulted in a forme fruste of holoprosencephaly, affecting, in this case, only the face and not the brain.
van der Wal
K G
KG
Department of Oral and Maxillofacial Surgery, Medisch Centrum Leeuwarden, The Netherlands.
Wiebe Mulder
J
J
D000848:Anodontia / Q000150:complications; D002971:Cleft Lip / Q000150:complications*; D002972:Cleft Palate / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D016142:Holoprosencephaly; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications; D008297:Male; D009666:Nose / Q000002:abnormalities; D011236:Prediabetic State; D011247:Pregnancy; D011254:Pregnancy in Diabetics