Aplasia cutis congenita is a very rare and
heterogeneous disease. It is characterized by a localized
or extensive skin defect, isolated or associated with
damage to the underlying structures, including the
bone. The diagnosis is based on the clinical examination.
Other abnormalities of various etiologies and severities
may be associated with this pathology.
Adams-Oliver syndrome is a genetic
polymalformative syndrome. The typical form is
defined by the presence of Aplasia cutis congenita and
Managing Aplasia cutis congenita, especially in
severe cases, is a real challenge.
We report a case of a newborn, treated surgically,
for extensive occipito-parietal Aplasia cutis congenita.
The clinical presentation was suggestive of AdamsOliver syndrome. The evolution was favorable.
Describing our experience of managing a case of
Aplasia cutis congenita is interesting because: the
proposed treatment is very controversial, the rarity and
the high morbidity and mortality of Aplasia cutis
congenita and Adams-Oliver syndrome. As well as
clinical and genetic diversity.
Keywords : Aplasia cutis congenita, Adams-Oliver syndrome, surgery, scalp, treatment