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Atretic cephalocele - an uncommon cause of cystic scalp mass

Siong Lung Wong1, Huong Ling Law, Suzet Tan

  • 1Faculty of Medicine and Health Sciences, Universiti Malaysia Sarawak, Lot 77, Seksyen 22, KTLD, Jalan Tun Ahmad Zaidi Adruce, 93150 Kuching, Sarawak, Malaysia.

The Malaysian Journal of Medical Sciences : MJMS
|December 3, 2011
PubMed

Insights

Atretic cephalocele, a rare condition, was diagnosed in a young girl with a growing scalp swelling. Imaging revealed a cyst connected to the subarachnoid space, confirming the diagnosis.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pediatric Radiology

Background:

  • Atretic cephalocele is a rare congenital abnormality characterized by a defect in the skull with associated neural tissue malformation.
  • Early diagnosis is crucial for appropriate management and to prevent potential complications.

Observation:

  • A 6-year-old girl presented with a gradually enlarging midline parietal scalp swelling present since birth.
  • Magnetic resonance imaging (MRI) was utilized to investigate the scalp swelling.

Findings:

  • MRI revealed a cyst communicating with the subarachnoid space via a calvarial defect.
  • Associated findings included a vertically oriented straight venous sinus and subependymal grey matter heterotopia.
  • These imaging features are classical for atretic cephalocele.

Implications:

  • This case highlights the importance of advanced imaging in diagnosing rare congenital skull and brain malformations.
  • Understanding the imaging characteristics of atretic cephalocele is vital for pediatric neurosurgeons and radiologists.
  • Accurate diagnosis facilitates timely intervention and management planning for affected children.

Keywords:
cystsencephalocelemagnetic resonance imagingmedical sciencesscalp

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