By Gary M. White, MD
Adams-Oliver syndrome (AOS) is characterized by the combination of congenital scalp defects (aplasia cutis congenita) and terminal transverse limb defects of variable severity.
- AOS is usually autosomal dominant, but autosomal recessive and sporadic cases have been described.
- AOS does not necessarily convey decreased mortality.
- Terminal transverse limb defects significantly affect the distal phalanges or entire digits. Shortening of the fingers and loss of the terminal phalanges of the foot are the most common defects.
- Cutis marmorata telangiectatica congenita was found in 25% of reported cases and often involves the entire skin including the scalp.
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