Sinus pericranii associated with syntelencephaly: a case report

Area of Science:

• Neurology
• Vascular Malformations
• Pediatric Neurosurgery

Background:

• Sinus pericranii is a rare cranial venous malformation characterized by abnormal intracranial and subperiosteal vein communication.
• This condition typically presents as a subcutaneous mass, often with positional changes in size.
• Association with syntelencephaly, a subtype of holoprosencephaly, has not been previously reported.

Observation:

• A 2-year-4-month-old female with pre-existing syntelencephaly presented with a 3x2 cm soft, nonpulsatile parietal subcutaneous mass.
• The mass enlarged when lying down and decreased when sitting.
• Diagnostic imaging, including Doppler ultrasound, MRI, and cerebral angiography, confirmed a dilated vessel through the parietal bone connecting the superior sagittal sinus to scalp veins.

Findings:

• The patient was diagnosed with sinus pericranii co-occurring with syntelencephaly and coronal craniosynostosis.
• Surgical transection of the sinus pericranii at 2 years and 7 months resulted in complete resolution of the mass.
• No complications or recurrences were observed for over 2.5 years post-surgery.

Implications:

• This case highlights the potential association between sinus pericranii and brain malformations like syntelencephaly.
• Early detection of sinus pericranii is crucial due to risks of intracranial hemorrhage and sinus thrombosis.
• Physicians should consider sinus pericranii in pediatric patients presenting with soft cranial masses exhibiting positional variations.