Progressive Postnatal Pansynostosis

• Published in: The Cleft palate-craniofacial journal 2015-11, Vol.52 (6), p.751-757
• Main Authors: Rogers, Gary F., Greene, Arin K., Proctor, Mark R., Mulliken, John B., Goobie, Susan M., Stoler, Joan M.
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnosis; Abnormalities, Multiple - pathology; Acrocephalosyndactylia - diagnosis; Acrocephalosyndactylia - pathology; Age; Babies; Child, Preschool; Craniofacial Dysostosis - diagnosis; Craniofacial Dysostosis - pathology; Craniosynostoses - diagnosis; Craniosynostoses - pathology; Dentistry; Disease Progression; Female; Growth Disorders - diagnosis; Growth Disorders - pathology; Hearing Loss, Sensorineural - diagnosis; Hearing Loss, Sensorineural - pathology; Humans; Infant; Infant, Newborn; Intellectual Disability - diagnosis; Intellectual Disability - pathology; Male; Medical imaging; Patients; Retrospective Studies
• ISSN: 1055-6656; 1545-1569
• DOI: 10.1597/14-092

Objective To describe the subtle clinical features, genetic considerations, and management of progressive postnatal pansynostosis, a rare form of multisutural craniosynostosis that insidiously occurs after birth and causes inconspicuous cranial changes. Design, Participants, Setting The study is a retrospective chart review of all patients diagnosed with progressive postnatal pansynostosis at a major craniofacial center between 2000 and 2009. Patients with kleebattschädel were excluded. Results Nineteen patients fit our inclusion criteria. Fifteen patients had a syndromic diagnosis: Crouzon syndrome (n = 8), Saethre-Chotzen syndrome (n = 5), and Pfeiffer syndrome (n = 2). With the exception of one patient with moderate turricephaly, all patients had a relatively normal head shape with cranial indices ranging from 0.72 to 0.93 (mean, 0.81). Patients were diagnosed at an average of 32.4 months; craniosynostosis was suspected based on declining percentile head circumference (n = 14), detection of an apical prominence (n = 12), papilledema (n = 7), and worsening exorbitism (n = 3). Nearly all patients had evidence of increased intracranial pressure. Conclusion Progressive postnatal pansynostosis is insidious; diagnosis is typically delayed because the clinical signs are subtle and appear gradually. All infants or children with known or suspected craniosynostotic disorder and a normal head shape should be carefully monitored; computed tomography is indicated if there is any decrease in percentile head circumference or symptoms of intracranial pressure.