• Junrao Li, Ting Wang, Seidu A Richard, Changwei Zhang, Xiaodong Xie, and Chaohua Wang.
• Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China.
• Medicine (Baltimore). 2020 Jun 26; 99 (26): e20991e20991.

RationalePial arteriovenous fistula (PAVF) occurs when intracranial arteries communicate directly with veins. PAVFs are very rare congenital vascular lesions that are commonly seen in infants and children. Arachnoid cysts are congenital cavitation often filled with cerebrospinal fluid. We present a very rare associated occurrence of bilateral pediatric PAVF and a giant arachnoid cyst presenting as torticollis in a child. So far, this is the first case.Patient ConcernsA 6-year-old male child was referred to our facility from a local hospital due to severe torticollis.DiagnosesAn enhanced computed tomography scan revealed 2 slightly high-density masses at the anterior pontine cistern, right circumferential cistern, as well as left posterior occipital region. The same computed tomography scan revealed a giant arachnoid cyst in the left occipital as well as the temporal region with a thin cerebral cortex adjacent to the cyst.InterventionsDigital subtraction angiography confirmed that the 2 high-flow lesions were PAVFs. The patient was treated with a combination of detachable coils and Onyx Liquid Embolic System (Onyx HD-500) (Covidien/ev3 Neurovascular) via the transarterial endovascular route while the giant arachnoid cyst was managed conservatively.OutcomesThe torticollis resolved 2 days after the procedure. He is currently well with no neurologic deficit.LessonsWe advocate that in cases of PAVF with accompanying cyst, the cyst should be managed conservatively if it is not associated with intracranial hemorrhage or focal neurologic deficit.

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