A case of 4p-syndrome with a bilateral cleft lip and palate.

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4 p-syndrome (Wolf-Hirchhorn syndrome) is a rare congenital abnormality, characterized by delayed growth and development and facial dysmorphism associated with partial deletion of the short arm of chromosome 4 (4p-). Among many clinical findings, cleft lip and/or palate are the major manifestations. This is the case report of a male infant with 4p-syndrome, including a bilateral cleft lip and palate. Physical examination showed severe growth retardation, seizures, deafness, multiple craniofacial defects including a bilateral cleft lip and palate, inguinal hernia, hypoplastic dermal ridges, and so on. Roentgenographic investigation revealed dysplasia of the permanent dentition. Cytogenetic studies showed that the short arm of chromosome 4 was deleted and the band 4p 15.32 or 4p 15.33 was broken, although both parents had normal karyotypes. These findings are common features of this syndrome.<BR>Repairs of the bilateral cleft lip and palate were performed at the age of 9 months, 1 year and 4 months, and 3 years and 4 months, respectively. The postoperative course was good.<BR>Operations on cleft lip and/or palate for 4p-syndrome patients without serious complications are thought to be generally possible, although careful consideration is required.
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