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Methods: The authors reviewed their ongoing prospective craniofacial database (2/97-7/12) to document patients with PPP. Variables included age at diagnosis, syndromic association, head shape, serial head circumference (HC), radiographic findings, and operative interventions. Inclusion criteria included: 1) complete or partial fusion of all major cranial sutures on high resolution CT scan; 2) no prior craniofacial procedures at the time of CT diagnosis.
Results: A total of 10 out of 292 patients (3.5%) were identified. Each patient had confirmed fusion of all major cranial sutures. Mean age at diagnosis was 59.4 ± 42.3 months (range: 19-151 months). A syndrome was documented in 60% of patients, with Crouzon syndrome being most common (3 patients). Head shape was reported as normal or near normal in all patients. Serial HC, available in 90% of patients, documented sustained growth deceleration at the time of diagnosis. All patients had signs of increased ICP, such as severe endocortical erosion and loss of cisternal spaces; 6/7 patients with MRI had Chiari malformation. ICP monitoring in 4 patients revealed pressures ranging from 5 to >35 cm H2O. Calvarial expansion was performed in 70% of patients, two are awaiting definitive surgical repair, and one patient had only Chiari decompression and clinical monitoring.
Conclusion: The gradual progression of fusion in PPP results in a slow decrease in head circumference percentile and a relatively normal head shape, leading to delayed diagnosis and increased ICP. There should be a high clinical index of suspicion in children with a known craniosynostosis syndrome (especially Crouzon syndrome) and normal head shape. Any sustained deceleration in serial HC should prompt CT evaluation.