Progressive Postnatal Pansynostosis

Cleft Palate Craniofac J. 2015 Nov;52(6):751-7. doi: 10.1597/14-092. Epub 2014 Oct 28.


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.

Keywords: craniosynostosis; normocephalic; pansynostosis; postnatal; progressive.

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Abnormalities, Multiple / pathology
  • Acrocephalosyndactylia / diagnosis
  • Acrocephalosyndactylia / pathology
  • Child, Preschool
  • Craniofacial Dysostosis / diagnosis
  • Craniofacial Dysostosis / pathology
  • Craniosynostoses / diagnosis*
  • Craniosynostoses / pathology
  • 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
  • Retrospective Studies

Supplementary concepts

  • Pfeiffer Kapferer syndrome