Spontaneous Intracranial Hypotension in Children: A Multi-Institutional Review of Spinal CSF Leaks Localized on Advanced Myelography

SUMMARY: Spontaneous intracranial hypotension is an increasingly recognized syndrome caused by a spinal CSF leak, with most reported cases occurring in adults. The use of specialized or advanced myelography to localize spinal CSF leaks has evolved substantially in recent years, particularly since the initial description of CSF-venous fistulas in 2014. To our knowledge, no prior series have evaluated the use of specialized myelographic techniques to localize CSF leaks in children with spontaneous intracranial hypotension, likely because the disease is rare in this patient population. This issue may be compounded by a hesitation to perform invasive procedures in children. In this clinical report, we conducted a multi-institutional review of pediatric patients with spontaneous spinal CSF leaks localized using advanced myelographic techniques, such as prone and decubitus digital subtraction and CT myelography, as well as dynamic CT myelography. We report the clinical features of these patients, as well as imaging findings, types of leaks discovered, and method of treatment. We found that the primary types of spontaneous spinal CSF leaks that occur in adults, including dural tears and CSF fistulas, can be seen in children, too. Furthermore, we show that specialized myelographic techniques can successfully localize these leaks and facilitate effective targeted treatment.