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

BACKGROUND: Posterior synostotic plagiocephaly represents a rare challenging type of craniosynostosis, often misdiagnosed as a simple posterior positional plagiocephaly. Underdiagnosed forms may result in delayed diagnosis and neurosurgical management, with potential ophthalmologic, cognitive, and aesthetic sequelae in children. METHODS: Here we retrospectively analyzed data of 9 posterior synostotic plagiocephaly infants treated in our center over a 10-year period (January 2000-December 2009). RESULTS: Patients averaged 10 months of age, and there was a clear male predominance (8 males/1 female). Cerebral computed tomography scan was performed in all patients, and the abnormal suture was located at the right side in 7 cases (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 minimal facial asymmetry in 4 cases (44.44%). The ophthalmoscopic examination found a papillary edema grade 1 in 2 cases and grade 2 in 4 cases. There were no deaths or reoperation in our series. All of our patients underwent a neurosurgical correction, with total ophthalmic recovery in all patients with preoperative papillae edema. Eight 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. CONCLUSIONS: Interesting findings in our series were male gender predominance and predilection of the rightside synostotic lambdoid suture. We think that early correct diagnosis and appropriate neurosurgical treatment may prevent potential complications such as neurocognitive and aesthetic sequelae in children with posterior synostotic plagiocephaly.