[Aplasia of the skull without scalp anomalies. A case report]

The authors report an extremely rare case of cranio-facial anomaly, which, to our knowledge has previously been reported only once (1993, Chakraborty and al.). This male infant presented with a giant congenital bone defect of the skull in the vertex region (10 x 20 cm) with no scalp deficiency. Minimal turricephaly and moderate telorbitism were associated with minor limb anomalies, but psychometric assessment appeared normal. Non-surgical follow-up was initially decided, but spontaneous reossification was so moderate that skull reconstruction was decided at 28 months of age, because of traumatological risks. A full-size resin cephalic skeletal reconstruction was obtained by 3D computerized tomography utilizing stereolithography techniques. A titanium plate was customized on the resin model for ideal adaptation to the convex skull defect (8 x 16 cm). Surgery was simply performed, consisting of preliminary undermining between the dura mater and scalp, and screwing of the custom titanium plate. The initial follow-up was uneventful.