Diagnosis, management and outcome in 9 children with unilateral posterior synostotic plagiocephaly.

Posterior synostotic plagiocephaly (PSP) represents a very rare challenging type of craniosynostosis, often misdiagnosed as a simple posterior positional plagiocephaly (PPP). Under-diagnosed forms may result in delayed diagnosis and neurosurgical management, with potential ophthalmological, cognitive and aesthetic sequelae in children.Here we retrospectively analyzed data of 9 PSP infants treated in our center over a 10-year period (January 2000-December 2009).Patients averaged 10 months of age, and there was a clear male predominance (8males/1 female). Cerebral CT-scan was performed in all patients, and the abnormal suture was located at the right side in 7cases (77.77%). Ipsilateral occipitomastoid bulge and occipital flattening were found in all cases (100%). Posterior ear displacement was found in 7 cases (77.77%), and there was a minimal facial asymmetry in 4 cases (44.44%). The ophthalmoscopical examination found a papillary edema grade-1 in 2 cases and grade-2 in 4 cases. There were No death or reoperation in our series. All of our patients underwent a neurosurgical correction, with total ophthalmic recovery in all patients with preoperative papillae edema. 8 patients had an early satisfactory aesthetic aspect. long-term follow-up in treated children found a normal childhood, with no major school dysfunctions, and normal social integration.An interesting finding in our series was the male gender predominance and the predilection of the right side synostotic lambdoid suture. We think early correct diagnosis and appropriate neurosurgical treatment may prevent potential complications such as neuro-cognitive and aesthetic sequelae in children with posterior synostotic plagiocephaly.