A giant head and neck venolymphatic malformation in a newborn: utility of post mortem MRI.

Posted By Alaa Mahmoud
A giant head and neck venolymphatic malformation in a newborn: utility of post mortem MRI.

A 26 week-gestational age female with poor maternal prenatal care was emergently delivered via cesarean section due to poor waveform on umbilical artery Doppler. A fetal ultrasound prior to delivery demonstrated a giant mass-like craniofacial deformity involving the left neck, face and eye. The patient passed away immediately after birth despite resuscitation efforts. Given potential implications for future pregnancies, the family sought a diagnosis but refused autopsy/invasive workup. Post-mortem MRI confirmed the presence of a large venolymphatic malformation involving the neck, left oral cavity and the left orbit with a massive intracranial component causing severe malformation and underdevelopment of the brain. This case reaffirms the utility of post mortem MRI and is to our knowledge the first case of a neonatal head and neck massive venolymphatic malformation with a large intracranial component causing ‘Early neonatal Mortality’. Figure 1A. T2 weighted coronal MRI demonstrating intracranial extra-axial multicystic mass involving the left neck (black arrow) consistent with a venolymphatic malformation. Figure 1B. Axial T2 weighted image demonstrating multicystic mass (black arrow) with fluid-fluid levels inside the calvarium (gray arrow) displacing rudimentary brain (black arrow head). There is also subgaleal hematoma (white arrow). Figure 1C. Axial T2 weighted image demonstrating ocular involvement of the venolymphatic malformation resulting in proptosis (white arrow). Figure 1D. T2 weighted axial image through the neck demonstrating extensive neck soft tissue involvement of the venolymphatic malformation with mass effect on the airway (black arrow).

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