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Pre- and postnatal imaging of Pai syndrome with spontaneous intrauterine closure of a frontal cephalocele

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Abstract

Pai syndrome is a rare congenital disorder characterized by cutaneous polyps of the face, pericallosal lipoma and median cleft lip. We report on a newborn girl with a variant of Pai syndrome presenting with all typical findings except a median cleft. In addition, fetal sonography and MRI showed the unique intrauterine evolution of a cephalocele into an atretic cephalocele.

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Authors and Affiliations

  1. Department of Interventional, Pediatric and Diagnostic Radiology, Inselspital University Hospital, University of Bern, Freiburgstrasse 10, 3010, Bern, Switzerland

    Tomas Dobrocky, Lukas Ebner & Enno Stranzinger

  2. Department of Pediatric Surgery, Inselspital University Hospital, University of Bern, Bern, Switzerland

    Benjamin Liniger

  3. Department of Diagnostic and Interventional Neuroradiology, Inselspital University Hospital, University of Bern, Bern, Switzerland

    Christian Weisstanner

Authors
  1. Tomas Dobrocky
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  2. Lukas Ebner
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  3. Benjamin Liniger
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  4. Christian Weisstanner
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  5. Enno Stranzinger
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Correspondence to Tomas Dobrocky.

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Dobrocky, T., Ebner, L., Liniger, B. et al. Pre- and postnatal imaging of Pai syndrome with spontaneous intrauterine closure of a frontal cephalocele. Pediatr Radiol 45, 936–940 (2015). https://doi.org/10.1007/s00247-014-3205-8

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  • DOI: https://doi.org/10.1007/s00247-014-3205-8

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