CSF Leak Associated with POEMS Syndrome

A 58-year-old Jamaican female presented with new onset diagnosis of papilledema. She had a prior history of an idiopathic peripheral neuropathy diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP) on intravenous immunoglobulin without improvement. In review of her history, she did have a high monoclonal gammopathy, high serum platelet count, and darker skin changes. The clinical history, laboratory testing and examination were suspicious for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes).

A magnetic resonance imaging (MRI) was done which showed a large empty sella and a left ethmoid sinus lesion which extended across the cribriform plate into the left anterior cranial fossa which was suspicious for a mass ([Figs. 1] and [2]). Sinus endoscopy revealed a nonpulsating, reddish mass with some exudate over it in the left olfactory cleft area. A computed tomography (CT) scan done shortly after the procedure confirmed an encephalocele ([Fig. 3]). The patient was already on acetazolamide 500 mg twice daily and undergoing treatment with Velcade for the POEMS syndrome with a hematologist. The plan was to observe the skull base defect since it was asymptomatic.

Eighteen months after the initial diagnosis of POEMS, she developed CSF leak from her nose. She underwent transfacial extradural resection of skull base meningoencephalocele with Alloderm closure of skull base defect/repair of CSF leak. She had a lumbar drain done prior to the surgery which measured the opening pressure at 32cmH2O.

Forty-two months later, she had recurrence of the CSF leak. Repeat CT ([Fig. 4]) and MRI showed a larger empty sella and now a right-sided meningoencephalocele. Repeat eye examination showed resolution of the initial papilledema. She underwent another repair of the CSF leak with findings this time showing a large meningoencephalocele reaching right nasal floor. Left-sided repair previously done looked intact.

Four years after the second CSF leak repair, she remains without leak. The most recent MRI did not show any anterior skull base defects.

POEMS syndrome is associated with papilledema due to intracranial hypertension as a result of high CSF protein.[1] CSF leak can be a complication of the chronic high cranial pressure.[2] We saw no prior cases of POEMS syndrome and CSF leak rendering this case unique. One case reported using ventriculoperitoneal shunt for symptoms and signs of high cranial pressure in POEMS.[3] We must consider POEMS syndrome in cases of high cranial pressure and other signs and symptoms atypical of classic idiopathic intracranial hypertension.

Publication History

Article published online:
07 February 2025

• 1 Liu LS, Zhang X, Zhao H. et al Reliability of optic disc edema area in estimating the severity of papilledema in patients with POEMS syndrome. Orphanet J Rare Dis 2020; 15 (01) 116
• 2 Hong CS, Kundishora AJ, Elsamadicy AA, Vining EM, Manes RP, Omay SB. A unique subset: idiopathic intracranial hypertension presenting as spontaneous CSF leak of the anterior skull base. J Neurol Surg B Skull Base 2021; 83 (02) 105-115
• 3 Alvarez-Breckenridge CA, Attiah MA, Zachariah M, Gummadavelli A, Yang J, Codd PJ. Ventriculoperitoneal shunt placement for POEMS syndrome. J Clin Neurosci 2015; 22 (10) 1672-1674