Occult spinal CSF leak: to patch or not to patch-a case-based update

Spontaneous intracranial hypotension (SIH) is a rare condition in the pediatric population with a classic clinical presentation of severe orthostatic headaches that improve when the patient is lying down. The cause of SIH is cerebrospinal fluid (CSF) leakage either around the brain or spinal cord. When working up a patient with suspected SIH, an MRI is done, which aims to visualize the location of the leak. Visualization of the leak on imaging confirms the diagnosis of SIH and can guide localized treatments like epidural blood patch (EBP) or surgery. There has never been a reported case of a pediatric patient that presents with classic symptoms of SIH but shows completely normal findings on brain and spine imaging. Cases of adult occult SIH (oSIH) have been successfully treated with an empiric EBP; however, the use of an empiric EBP in a child with suspected oSIH has not been reported before.

We describe the clinical features and management of a pediatric patient with an oSIH. A literature review was done for English articles, using PubMed, to identify previously reported cases of SIH and oSIH treated with empiric EBP.

A 9-year-old patient presented with worsening severe orthostatic headaches, nausea, phonophobia, a sensation of ear fullness, slight cognitive changes, and complete resolution of headaches when laying down. She received multiple MRIs of the brain and spine, which all found no abnormalities. She was treated for suspected post-viral syndrome and later for suspected migraines but had brief to no symptomatic relief. Her pain continued to worsen, debilitating her and causing her family distress. After being referred to our clinic, she was diagnosed with an oSIH and was treated with an empiric lumbar EBP. The patient made a profound recovery with a complete and sustained resolution of her symptoms. This shows the first pediatric case of a clinically suspected occult CSF leak with normal brain and spinal imaging, that was treated successfully with an empiric EBP after conservative treatments failed.

While epidural blood patch has become a routine treatment option for SIH and a proven treatment in adult oSIH, its use in pediatric oSIH is still unclear due to the rarity of this disease. This case shows that an empiric EBP is a viable treatment option in pediatric oSIH. In situations where clinical suspicion for oSIH is high, empiric EBPs should be considered, since they have a relatively low procedural risk and can offer sustained and significant symptomatic improvement.