Transverse sinus dural arteriovenous fistula: a reversible cause of severe pulmonary hypertension in an extremely premature infant

• Published in: BMJ case reports 2021-02, Vol.14 (2), p.e239544
• Main Authors: Jordan, Leah, Rodgers, Nathan, Roberts, Kari D
• Format: Article
• Language: English
• Subjects: Age; Carotid arteries; Case reports; Central Nervous System Vascular Malformations - complications; Central Nervous System Vascular Malformations - diagnostic imaging; Central Nervous System Vascular Malformations - therapy; Dura Mater; Embolization; Embolization, Therapeutic; Fistula; Humans; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - etiology; Images In; Infant; Infant, Extremely Premature; Infant, Newborn; Lung diseases; Medical imaging; Medical prognosis; Neuroimaging; Newborn babies; Ostomy; Patients; Pediatrics; Peptides; Premature babies; Premature birth; Pulmonary arteries; Pulmonary hypertension; Sinuses; Transverse Sinuses; Ultrasonic imaging; Veins & arteries
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2020-239544

On day of life (DOL) 95 (42-week postmenstrual age (PMA)), cardiac catheterisation revealed suprasystemic mean pulmonary artery pressure (65 mm Hg), high indexed pulmonary vascular resistance (8.4 Woods units×m2) and an exceedingly high superior vena cava (SVC) oxyhaemoglobin of 94% in the absence of partial anomalous pulmonary venous return, concerning for an arteriovenous malformation (AVM) of the head or neck. Intracranial AVMs are a rare but well-described cause of PH in neonates.1 While vein of Galen malformations are more commonly associated with PH in this age group, DAVF account for approximately 5.7%–10% of all paediatric intracranial AVMs and have been described to cause PH in neonates.2 3 Unlike vein of Galen malformations, the natural history of DAVF presenting in the neonatal period is less well understood and may include spontaneous regression.4 5 This is the first reported case of a DAVF resulting in severe PH in an extremely premature infant with chronic lung disease of extreme prematurity and normal head ultrasound evaluations. The most common sites of DAVF include the torcula, superior sagittal sinus, transverse sinus and cavernous sinus.2 Abnormalities in these deep structures may not be detected with traditional head ultrasound protocols. [...]in neonates with disproportionately severe and/or rapidly progressing PH, particularly those found to have elevated SVC oxyhaemoglobin on cardiac catheterisation, further evaluation for intracranial AVMs should be performed, even in the setting of a negative head ultrasound.