Recurrent Spontaneous Cerebrospinal Fluid Leaks at Multiple Levels
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.333501
Source of Support: None, Conflict of Interest: None
Keywords: Craniospinal hypotension, CSF leak, CT myelography, epidural blood patch, nuclear scintigraphy
Spontaneous intracranial hypotension (SIH) is a commoner condition than realized. Spontaneous intracranial hypotension manifests with orthostatic headache, symptoms of low cerebrospinal fluid (CSF) pressure, and diffuse pachymeningeal enhancement on MRI. Spontaneous intracranial hypotension resolves spontaneously with conservative measures in most patients. Lumbar autologous epidural blood patch (EBP) has been used regardless of level of leak because of its safety. The injected blood travels many levels from the site of injection. In some patients, who do not respond to conservative measures, or have multiple levels of CSF leak, EBP is targeted to the site of CSF leak guided by imaging studies. Recently, we managed a girl with multiple spontaneous CSF leaks, who responded to autologous EBP under image guidance. We report this patient and highlight the difficulties in the diagnosis and management of multi-level recurrent CSF leaks.
A 19-year-old girl was admitted with a history of two attacks of paraparesis in the last two years. The last attack of paraparesis was three months back, which began with the left lower limb followed by right a day later. The weakness was associated with diffuse pulsatile headache on sitting and standing, and was relieved on lying down. She gave a history of similar weakness, episodes of fainting, visual blurring, tinnitus, and aural fullness rom the age of nine years and was diagnosed as psychogenic spell or seizure [Figure 1]. At the time of evaluation by us, she had persistent headache and watery nasal discharge for a week. Her lower limb power was grade 1–2 MRC (Medical Research Council) and knee and ankle reflexes were brisk and plantar extensor. She had loss of pin-prick sensation below D10 and joint position sense was impaired. She had retention of urine and was catheterized. Her blood counts and serum chemistry were normal. Spinal MRI showed dilated posterior epidural space displacing the spinal cord anteriorly [Figure 2]a. MRI of dorsal spine after recovery showed normal epidural space and position of the cord [Figure 2]b.
Cerebrospinal fluid opening pressure was 40 cm of water (normal 60). TC 99DTPA nuclear scan revealed multiple levels of CSF leak which were confirmed by CT myelography. The leaks were at the D1 and D10 levels on the right side and D11 and L2 levels on the left side [Figure 3].
After CT myelography, the patient developed complete paraplegia with sensory loss below the D9 level. She was treated with EBP at lumbar and lower thoracic level. Following EBP, she improved and was able to do her activities of daily living two weeks later. Her CSF rhinorrhea could not be localized because of the lumbar leak. Two months later, she again developed paraparesis and sensory loss below D2, and this time 3 ml of EBP was introduced at D1 level under fluoroscopic guidance. Following EBP, she developed quadriplegia (MRC grade 0) and severe root pain. Repeat imaging did not reveal any cord compression but the D2 nerve root was hyperintense on T2 sequence. The patient recovered completely by next month and has remained symptom free for the past 18 months.
The diagnosis of spontaneous intracerebral hypotension in this patient was based on orthostatic headache, a CSF pressure of 40 cm water, and multiple levels of CSF leak, which were demonstrated by TC99 DTPA nuclear scan and CT myelography. The cranial MRI of spontaneous intracranial hypotension is characterized by the signs of brain sagging resulting in diffuse pachymeningeal enhancement, descent of cerebellar tonsils, obliteration of basal cisterns, subdural fluid collection, enlargement of pituitary, engorged venous sinuses and reduced ventricular size. Various imaging modalities were used for the documentation of CSF spinal leak: CT myelography, radio isotope cisternography, and MR myelography. Spinal MRI shows spinal meningeal diverticulae or fluid collection, pachymeningeal enhancement, and engorgement of venous plexus. Radio isotope cisternography shows a rapid accumulation of tracer in the bladder and less radioactivity over cerebral convexities. CT myelography helps in the exact localization of CSF leak in one-third of patients. In spite of these imaging techniques, the leaks are not localized in half of the cases., Epidural blood patch not only seals the CSF leak but also helps in restoring the CSF blood gradient within the epidural space along the whole spinal cord. This patient presented with recurrent attacks of spontaneous CSF leaks at thoracic and lumbar regions, manifesting with paraplegia, quadriplegia, CSF rhinorrhea, fainting, postural headache, aural fullness, and tinnitus in last 10 years. These features are consistent with spontaneous intracranial hypotension (SIH) manifesting with varying severity. Multiple-level CSF leaks without any apparent cause may be due to underlying collagen tissue disorder, although we could not find a recognizable disorder. Hufner et al. have reported CSF leak in dorsal level (right D1, left D2) and the patient needed surgical repair although the authors could not find any definite cause such as connective tissue disorder. Recurrent CSF leaks have been reported in Ehlers–Danlos syndrome type II, Marfan syndrome, autosomal dominant polycystic kidney disease, and a number of unnamed syndromes or associations. Epidural patch is a simple and safe procedure, which is often used in unlocalized CSF leak. Our patient, however, had CSF leaks at multiple levels as evidenced by TC99 DTPA nuclear scan and CT myelography. The CSF leak at higher level required EBP under image guidance. In our patient, EBP was associated with worsening of weakness (quadriplegia) which resolved over three weeks. Thoracic and cervical EBP are associated with higher risk of cord and root complications. The epidural blood patch may be associated with the compression of spinal cord, nerve root inflammation, meningitis, or seizures. In the patients who do not respond to conservative measures and have persistent and disabling symptoms, surgical repair is recommended. Surgical repair, however, has limited role in the patients with multiple-level CSF leaks and if the leaks are not well localized.
In a patient with recurrent CSF leaks at multiple levels, an underlying cause should be investigated, but may not be always found. The epidural blood patch under image guidance is helpful in managing CSF leaks at multiple levels.
Informed consent was obtained from the patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
All the procedures performed in studies involving human participant were in accordance with the ethical standards of the institutional and or national research committee and with the 1964 Helinski Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from the patient.
We thank Mr. Shakti Kumar for secretarial help.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3]