• Congenital Abnorm
• Paediatric Practice
• Aplasia Cutis Congenita

Aplasia cutis congenita is an uncommon condition (estimated global incidence 0.5–1 in 10 000 neonates)1 characterised by a localised defect of all skin layers and subcutaneous tissue, sometimes extending to the underlying bone and meningeal layers.2 Some of the possible complications include haemorrhage, trauma, venous sinus thrombosis and infection (including meningitis).3 The management is controversial, and includes surgical and conservative treatment options.4

We present the case of a term male newborn, weighing 2615 g with normal delivery by a 24-year-old primipara. Prenatal ultrasounds were unremarkable as was prenatal history regarding infections and drug or medication use. Family history was also unremarkable. At the time of the delivery it was noted a large lesion of the scalp measuring 5×5 cm, involving all the layers of the skin, underlying subcutaneous tissue, bone and dura mater through which the sagittal venous sinus was visible (figure 1). There was no visible leak of cerebrospinal fluid. No other associated malformations were noted. The lesion was dressed initially twice a day with sterile fatty gauze bandage and after 2 weeks with soft silicone dressings (Mepitel) to minimise trauma and drying and to promote a re-epithelialisation of the lesion. Prophylactic antibiotics (flucloxacillin and gentamicin) were initiated. Progressive closure of the lesion issued without complications over the course of the next weeks.

Rapid identification, clinical assessment and institution of appropriate protection of the lesion are necessary to achieve the best outcome. A multidisciplinary approach is recommended.

• Download figure
• Open in new tab
• Download powerpoint

Figure 1

Aplasia cutis congenita of the scalp measuring 5×5 cm with sagital venous sinus exposure. Photo taken in the first hours of life (Israel Macedo, Maternidade Alfredo da Costa).