Title:Approach in Management of Aplasia Cutis Congenita in a Newborn with Adam Oliver Syndrome
Volume: 2
Author(s): Ihab H. El Kadry*, Tarek Koratum and Laila Obaid
Affiliation:
- Departmentof Neonatology, Corniche Hospital, Abu Dhabi,United Arab Emirates
Keywords:
Aplasia cutis congenita, Adams-Oliver syndrome, Neonatal skin disorder, Congenital disorder, Cerebrospinal fluid, Scalp midline.
Abstract:
Introduction:
Aplasia Cutis Congenita (ACC) is a rare congenital disorder characterized by the absence of skin and adjacent tissue that usually affect the scalp.
A large scalp defect in ACC with adjacent tissue anomalies is associated with high morbidity and mortality. The management of these lesions can
be challenging. The treatment can be either conservative, surgical, or combined.
Case:
A full-term newborn girl delivered with a huge scalp defect. The dura mater was exposed but intact and there was no Cerebrospinal Fluid (CSF)
leakage. She also had bilateral talipes equinovarus and terminal reduction of multiple toes in the left foot. Genetic testing was positive for Adam
Oliver Syndrome. Conservative medical management was initiated at birth and continued until discharge at 4 weeks of age. The patient had a
regular follow-up with plastic surgery and neurosurgery teams. At 11 months old, the defect became smaller, hairless with good granulation tissue,
but still soft and compressible.
Conclusion:
Large scalp midline ACC is a rare, but potentially devastating disease when involved adjacent tissue. The choice of treatment is not
straightforward and it is mainly based upon the size, depth, and the risk of pre and post-treatment complications. Conservative management still is
an option with very complex ACC.
