A Case of Wolf-Hirschhorn Syndrome（4p-syndrome）with Cleft Palate Repair

Abstract

　　Wolf-Hirschhorn syndrome is a rare genetic disorder caused by partial deletion of the short arm of chromosome 4. Although our patient was born full-term, he had a low birth weight of 2079 g. The patient exhibited facial features indicative of Wolf-Hirschhorn syndrome, such as a“Greek warrior helmet appearance”, heart disease, hydronephrosis, cleft palate, and growth impairment that was evident during the pregnancy. The confirmatory diagnosis was made by gene testing. Cleft palate repair was performed by Furlow’s method when he was one year and six months old. At that time, his body weight was 7.1 kg (－3.0 SD) . The postoperative course was uneventful. His oral intake was increased. Five years after the surgery, he weighed 12.3 kg (－2.5 SD) . Cleft palate repair is a possible option to increase the oral intake and improve the quality of life of patients with Wolf-Hirschhorn syndrome.