A 1-month-old male infant presented with complaints of midline swelling in the postero-superior region of head.

A 1-month-old male infant presented with complaints of midline swelling in the postero-superior region of head.

FINDINGS ON MRI BRAIN WITHOUT IV CONTRAST

  • A small well-defined cystic lesion seen in the midline parietal region appearing T2 hyperintense T1 hypointense (CSF signal intensity) with an intracranial extension and associated bone defect at the cranial vertex.
  • CSF tract and primitive vertical falcine vein points towards the subcutaneous mass.
  • Prominence of the superior sagittal sinus with superior peaking of posterior tentorium is seen giving spinning top configuration.
  • Focal fenestration of the superior sagittal sinus.
  • Fibrous stalk connecting the cephalocele.
  • Atretic parietal cephalocele

ATRETIC PARIETAL CEPHALOCELE :

  • Atretic parietal cephaloceles, also known as atretic cephaloceles, are small subscalp lesions that consist of dura, fibrous tissue, and dysplastic brain tissue.
  • Infants and young children.
  • Palpable midline parietal soft tissue mass.
  • Thought to represent involuted true cephalocele (meningocele or encephalocele) connected to the dura mater via a fibrous stalk.
  • Increased incidence of intracranial anomalies.

IMAGING FEATURES:

  • Subgaleal soft tissue mass with an intracranial extension via a sharply demarcated calvarial defect (cranium bifidum)
  • CSF tract and vertical falcine vein point to the subcutaneous scalp mass
  • Vertically oriented primitive falcine vein
  • Fibrous stalk connecting the cephalocele
  • Focal fenestration of superior sagittal sinus at the atretic parietal cephalocele
  • Prominence of the superior cerebellar cistern and suprapineal recess
  • Superior peaking of the posterior tentorium
  • Spinning top configuration of the tentorial incisura

DIFFERENTIAL DIAGNOSES :

  • Sinus pericranii
  • Dermoid or epidermoid cyst
  • Cephalohematoma
  • Sebaceous  cyst
  • Vascular lesions (hemangioma)

References:

  1. Osborn AG, Salzman KL, Barkovich AJ. Diagnostic Imaging: Brain. Lippincott Williams & Wilkins. (2009) ISBN:1931884722.
  2. Wong SL, Law HL, Tan S. Atretic cephalocele - an uncommon cause of cystic scalp mass. Malays J Med Sci. 2012;17 (3): 61-3.
  3. Muralidharan CG, Aggarwal R, Singh D. Atretic parietal encephalocoele - An unusual diagnosis. Med J Armed Forces India. 2013;69 (1): 83-5.

Dr SRIRAM PATWARI
MBBS, MD, EDiNR
Senior Consultant Manipal Hospitals Radiology Group Yeshwantpur

Dr S Shreya
MBBS, MD
Cross section imaging fellow - MHRG