Aplasia cutis associated with coarctation of the aorta: could this be an incomplete form of Adams?Oliver syndrome?

Conflicts of interest: none declared. Sir, We report the association of aplasia cutis congenita (ACC) of the scalp and coarctation of the aorta in a newborn. A female child was born after suffering intrauterine growth retardation. Her mother’s obstetric history revealed a previous fetal death due to transposition of the great vessels. Physical examination of the patient revealed an alopecic and eroded plaque 2·5 cm in diameter, sited in the midline of the vertex. The lesion did not show a hair collar and was divided into two by a fibrous band (Fig. 1). A dimple in the lumbosacral region and a slightly dysmorphic ear were also found. No limb defects or cutis marmorata were observed. On the second day of life the patient presented with gasping, cyanosis and shortage of breath. Ultrasonography of the heart showed a severe coarctation of the aorta which was treated with a good outcome. Subsequent tests, including cranial and vertebral magnetic resonance imaging, were normal and a genetic study found a 46XX karyotype without microdeletions at 22q11. At present, the patient is 36 months old, healthy and follows a normal development. An alopecic and atrophic scar is still present on the scalp (Fig. 2).