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 right-side 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.