title: Progressive Postnatal Pansynostosis.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
authors.forename: Gary F
authors.initials: GF
authors.lastname: Rogers
authors.forename: Arin K
authors.initials: AK
authors.lastname: Greene
authors.forename: Mark R
authors.initials: MR
authors.lastname: Proctor
authors.forename: John B
authors.initials: JB
authors.lastname: Mulliken
authors.forename: Susan M
authors.initials: SM
authors.lastname: Goobie
authors.forename: Joan M
authors.initials: JM
authors.lastname: Stoler
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000473:pathology; D000168:Acrocephalosyndactylia / Q000175:diagnosis / Q000473:pathology; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000175:diagnosis / Q000473:pathology; D003398:Craniosynostoses / Q000175:diagnosis* / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis / Q000473:pathology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000175:diagnosis / Q000473:pathology; D008297:Male; D012189:Retrospective Studies
keywords: craniosynostosis; normocephalic; pansynostosis; postnatal; progressive