J Korean Soc Neonatol.
2001 May;8(1):171-174.
A Case of Adams-Oliver Syndrome which was Observed in Two Brothers
- Affiliations
-
- 1Department of Pediatrics, College of Medicine, Dankook University, Cheonan, Korea.
- 2Department of Dermatology, College of Medicine, Dankook University, Cheonan, Korea.
- 3Department of Pathology, College of Medicine, Dankook University, Cheonan, Korea.
- 4Department of Pediatrics, College of Medicine, Pochon CHA University, Kumi CHA Hospital, Kumi, Korea.
Abstract
- Adams-Oliver syndrome is characterized by the presence of scalp aplasia cutis congenita with distal limb anomalies. Cutis mammorata and dilated scalp veins are additional frequent manifestations of the condition. In almost all reported patients with Adams- Oliver syndrome, the trait of inheritance was autosomal dominant. Only three reports suggested autosomal recessive inheritance characterized by multiple affected offsprings in unaffected parents. But a number of sporadic cases have been described. We have experienced a boy with this syndrome, who showed large congenital scalp defect with exposed dural membrane and bony defect beneath it, cutis mammorata on whole body, equinovarus, and no metatarsal ossification center of left foot. His brother also had these symptoms, but their parents were not affected. He was complicated by bacterial meningitis, and cured with antibiotics. The skin defect was closed spontaneously with atrophic scar. We report this case with the brief review of literature.
