A Case of Adams-Oliver Syndrome which was Observed in Two Brothers.

Kim,  Young Jun; Jee,  Youn Hee; Lee,  Chae Hyeok; Kim,  You Chan; Cinn,  Yong Woo; Kim,  Eo Jin; Kim,  Jung Are; Chang,  Young Pyo

Case Report

Journal of the Korean Society of Neonatology 2001;8(1):171-174.
Published online May 1, 2001.

A Case of Adams-Oliver Syndrome which was Observed in Two Brothers.

Young Jun Kim, Youn Hee Jee, Chae Hyeok Lee, You Chan Kim, Yong Woo Cinn, Eo Jin Kim, Jung Are Kim, Young Pyo Chang

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.

형제에게 발생한 Adams-Oliver 증후군 1례

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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.

Key Words: Adams-Oliver syndrome; Aplasia cutis congenita; Cutis mammorata