Introduction
Adams-Oliver syndrome (AOS) is a rare congenital disorder characterized
by congenital cutaneous aplasia of the scalp and terminal transverse
limb anomalies (1). The lesions of congenital cutaneous aplasia are
generally located on the midline of the parietal or occipital regions,
where they may be associated with a parietal bone defect, but may also
appear on the abdomen or limbs(1–3). First described by Adams and
Oliver in 1945, this syndrome also presents severe forms of expression,
including central nervous system anomalies, cardiovascular disease, and
gastrointestinal malformations (1,4–6). Several modes of transmission
have been described: autosomal dominant, autosomal recessive, and
sporadic mutations (7–9). Six genes responsible for Adams-Oliver
syndrome have been identified, including ARHGAP31, RBPJ, NOTCH1, DLL4,
DOCK6, and EOGT (5,10,11). We report the case of a term newborn with
severe intrauterine growth retardation (IUGR) presenting with typical
signs of Adams-Oliver syndrome without associated visceral
complications. This syndrome remains rare and poorly described in the
scientific literature in Burundi.