Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension

Abstract

Fulminant idiopathic intracranial hypertension (FIIH) is a subtype of idiopathic intracranial hypertension (IIH) characterized by rapid, severe, progressive vision loss. Surgical intervention is often performed either as a cerebrospinal fluid (CSF) shunt procedure or an optic nerve sheath fenestration or, at times, both. These surgical procedures carry a significant risk of morbidity and failure. We present 2 patients in whom a temporary lumbar drain was successfully used in the management of medically undertreated pediatric FIIH, and circumvented the need for surgical intervention.

Case Series of Rapid Surgical Interventions in Fulminant Intracranial Hypertension

“…Fulminant intracranial hypertension is a severe form of classic intracranial hypertension that is associated with rapidly progressive visual impairment or blindness secondary to increased intracranial pressure. 4,6,7 Prevention of this morbidity is the goal of rapid identification and treatment of fulminant intracranial hypertension. Reports in the literature have included medical therapies, lumbar drain placement, cerebrospinal fluid diversion with lumboperitoneal shunt or ventriculoperitoneal shunt, optic nerve sheath fenestration, combination of shunt and optic nerve sheath fenestration, or more rarely intracranial venous sinus stenting.…”

“…3 Fulminant intracranial hypertension is a rare form of intracranial hypertension, which is described as an acute onset of increased intracranial pressure with rapid progression to severe vision loss. 4 Fulminant intracranial hypertension can be thought of as the severe form in the spectrum of intracranial hypertension, and it is possible for patients to progress to fulminant intracranial hypertension after the initial diagnosis of primary or secondary intracranial hypertension.…”

“…There are only a few reports of temporary lumbar drain placement combined with medical therapy as the sole treatment of fulminant intracranial hypertension. 4…”

Pediatric Idiopathic Intracranial Hypertension

“…40 Temporary lumbar drains can also be considered as a way to lower the intracranial pressure quickly, while secondary causes can be adequately addressed and/or intracranial pressure lowering medications can take effect. 41 An alternative to the ventricular shunt is optic nerve sheath fenestration, which provides a conduit for cerebrospinal fluid to escape the subarachnoid space around the optic nerves. 42 This approach should only be conducted by a trained and experienced oculoplastic surgeon or neurosurgeon to reduce risk of the major potential complication of irreversible, severe visual loss in the operated eye.…”

Indication to use a non-pencil-point lumbar puncture needle

“…If it remains elevated, we undertake a further 24 hours of drainage, again clamping the drain tube for an hour and remeasuring pressure. Lumbar drainage has also resulted in immediate and sustained resolution of IIH in both children14 and adults 15…”