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Open access

Huge cerebral pial arteriovenous fistula in a newborn: illustrative case

Vadim Berestov, Pavel Seleznev, Natalya Obedinskaya, Alexandra Korostyshevskaya, Julia Gofer, Ilya Bondarenko, Roman Kiselev, Sergey Krasilnikov, Anna Brusyanskaya, and Kirill Orlov

BACKGROUND

Pediatric arteriovenous malformations (AVMs) and pial/dural arteriovenous fistulas (AVFs) are rare but life-threatening complications that can lead to congestive heart failure and hemorrhagic stroke in newborns and pediatric patients. The pronounced shunting in these conditions is associated with early complications and necessitates aggressive surgical management. Here, the authors describe endovascular treatment of an atypical cerebral pial AVF in a newborn.

OBSERVATIONS

This AVF formed direct communication between a major cerebral artery (basilar artery) and a large draining vein (dilated deep cerebral vein). The authors performed earlier subtotal embolization of the AVF using 0.020-inch coils, which led to progressive thrombosis of the fistula with restoration of normal arterial blood flow. The patient was discharged 18 days after surgery, examination at 1.5 and 6 months showed magnetic resonance imaging signs of blood flow absence through the fistula and satisfactory condition of the infant without physical and mental developmental delay.

LESSONS

Subtotal coiling of a high-flow pial AVF in a newborn can result in a good clinical outcome.

Open access

Tricuspid regurgitation and left ventricular eccentricity as a measure of heart failure in the newborn patient with a vein of Galen malformation: illustrative case

Jeremy A. Yarden, Lily I. Hauck, Kamlesh V. Athavale, Andrew W. McCrary, M. Jay Campbell, and Erik F. Hauck

BACKGROUND

Successful management of a vein of Galen malformation (VoGM) in the newborn patient requires a highly coordinated team approach involving neonatologists, pediatric cardiologists, pediatric neurologists, neurosurgeons, and interventionalists. Indication and timing of catheter intervention are topics of ongoing debate.

OBSERVATIONS

The authors highlighted two key echocardiographic markers believed to be practical indicators regarding the need for urgent catheter embolization in neonates with a VoGM. The first and preferred parameter was the tricuspid valve regurgitation (TR) gradient, an estimate of pulmonary artery hypertension. If the TR gradient exceeds systolic blood pressure (suprasystemic pulmonary hypertension [PH], i.e., >60 mm Hg), urgent intervention should be considered in eligible newborns. The second parameter was the left ventricular end-systolic eccentricity index (EI), a newly emerging echocardiographic marker and indirect correlate of PH. As an alternative to the TR gradient, an increased eccentricity index (>1.6) suggests severe right heart compromise, requiring emergency catheter embolization of the malformation. Postoperatively, the progressive reduction of both the TR gradient and the EI correlated with recovery.

LESSONS

In eligible newborns, urgent embolization of a VoGM is recommended in the presence of suprasystemic TR gradients and/or increased EI >1.6.

Open access

Removal of malformation in cerebral proliferative angiopathy: illustrative case

Gwang Yoon Choi, Hyuk Jai Choi, Jin Pyeong Jeon, Jin Seo Yang, Suk-Hyung Kang, and Yong-Jun Cho

BACKGROUND

Cerebral proliferative angiopathy (CPA) is a rare vascular disorder distinct from arteriovenous malformation. Because of the disorder’s rarity, there is still a controversy on the most promising treatment method for CPA. However, several meta-analysis articles suggest indirect vascularization such as encephalo-duro-arterio-synangiosis as an effective way of treating symptoms that are medically uncontrolled.

OBSERVATIONS

The authors describe a case of an 11-year-old boy with this disease, who had epilepsy that was intractable despite conservative management. The patient recovered from his symptoms after the vascular malformation was surgically removed. This is the first reported case of surgical removal in CPA.

LESSONS

Although further investigation on the best treatment for CPA is needed, the authors believe surgical intervention may also be an effective treatment modality when a patient presents with persisting symptoms.