Gorham-Stout disease affecting the spine with cerebrospinal fluid leakage and Chiari-like tonsillar herniation: a rare case report and review of literature.

BACKGROUND: Gorham-Stout disease (GSD) is a very rare disorder characterized by massive osteolysis of poorly understood aetiology. The association between GSD involving the skull base and cerebrospinal fluid (CSF) leakage has been reported in the literature. However, few cases of CSF leakage and Chiari-like tonsillar herniation in GSD involving the spine have been reported. CASE PRESENTATION: We present the case of a 20-year-old man with GSD involving the thoracic and lumbar spine, which caused CSF leakage and Chiari-like tonsillar herniation. The patient underwent four spinal surgeries for osteolytic lesions of the spine over a 10-year period. Here, we discuss the possible aetiology of the development of CSF leakage. Epidural blood patch (EBP) was performed at the T11-T12 level to repair the CSF leakage. After EBP treatment, rebound intracranial hypertension (RIH) developed, and tonsillar herniation disappeared 2 months later. CONCLUSIONS: GSD involving the spine with CSF leakage and Chiari-like tonsillar herniation is relatively rare. For patients who have undergone multiple spinal surgeries, minimally invasive treatment is an alternative treatment for CSF leakage. EBP can repair CSF leakage secondary to GSD and improve chronic brain sagging, with reversibility of Chiari-like malformations.

The status of diagnosis and treatment to intracranial hypotension, including SIH.

Intracranial hypotension, especially spontaneous intracranial hypotension (SIH), is a well-recognized entity associated with cerebrospinal fluid (CSF) leaks, and has being recognized better in resent years, while still woefully inadequate. An increasing number of factors including iatrogenic factors are realized to involve in development and progression of intracranial hypotension. The diagnosis remains difficult due to the various clinical manifestations, some of which are nonspecific and easily to be neglected. Multiple imaging tests are optional in CSF leakage identification while clinicians are still confronted with difficulties when making selection resulting from superiorities and disadvantages of different imaging tests. Treatments for intracranial hypotension are multifarious but evidence is anecdotal. Values of autologous epidural blood patching (EBP), the mainstay of first-line interventional treatment currently, is getting more and more regards while there are no systematic review of its efficacy and risks. Hereby, the purpose of this review was to reveal the present strategy of intracranial hypotension diagnosis and treatment by reviewing literatures, coupled with our experience in clinical work.

[Clinical characteristics of 42 cases of spontaneous intracranial hypotension with cerebrospinal leakage].

OBJECTIVE: To review the clinical characteristics of spontaneous intracranial hypotension (SIH) with cerebrospinal fluid (CSF) leakage. METHODS: Clinical data of 42 SIH patients with cerebrospinal leakage, whose diagnosis met the criteria of the International Headache Classification, were retrospectively reviewed. The patients were divided into short (n=27) and long (n=15) course groups. The clinical data and imaging features were compared between two groups. RESULTS: Thirty-nine patients (92.9%) had orthostatic headache. Compared with the short course group, the frequency of headache were significantly lower in patients with long disease duration (80% vs 100%, P =0.040); the ratio of high CSF opening pressure (>=60.0 mm H2O), the average CSF opening pressure, and the frequencies of subdural hematoma were higher in long course group than those in short course group [60.0% vs 20.8%, (64.7±42.1) vs (40.0±33.8)mm H2O, and 50.0% vs 11.6%; P=0.019, 0.038 and 0.018, respectively]. Forty-two patients underwent CT myelography;definite focal CSF leakage sites were found in all patients and multiple sites of CSF leakage in 38 patients. CONCLUSION: All SIH do not necessarily show the typical clinical manifestations, and cranial MRI and CT myelography are helpful in the diagnosis. Because of higher risk of subdural hemorrhage, patients with long disease duration require active intervention.

Case report: Epidural blood patches are effective in treating intracranial hypotension due to a subarachnoid-pleural fistula.

Background: Intracranial hypotension (IH) is usually associated with cerebrospinal fluid (CSF) leakage and/or CSF hypotension, and epidural blood patch (EBP) therapy has been proven to be effective for treating spontaneous IH and post-dural puncture headaches. Tarlov cysts (TCs) are common lesions of the sacral spine. They have rarely been reported in thoracic locations and are even less common in the posterior mediastinum, which can lead to their misdiagnosis as neurogenic tumors. Case presentation: Here, we report the case of a 60-year-old woman who developed an orthostatic headache after the thoracoscopic resection of a TC in the posterior mediastinum that was presumed to be a schwannoma preoperatively. The patient was finally diagnosed with IH caused by a subarachnoid-pleural fistula (SPF) and was cured by targeted EBP treatment. Conclusion: This is a case to show that a single targeted EBP treatment is effective for a patient with IH caused by an SPF after thoracoscopic resection of a TC. This case reminds us to be vigilant that a TC may be masquerading as a posterior mediastinal neurogenic tumor, and a detailed examination should be performed to identify it before deciding on a surgical procedure. In addition, postural headache after thoracoscopic spinal surgery should be alert to the possibility of IH induced by an SPF. Once it occurs, early treatment is necessary, and targeted EBP treatment can serve as a safe and effective alternative when conservative treatment fails.

Targeted Epidural Blood Patch Treatment for Refractory Spontaneous Intracranial Hypotension: A Case Report.

Purpose of Review Spontaneous intracranial hypotension (SIH) is recognized far more commonly than before, and it is well known that SIH is sometimes complicated by chronic subdural hematoma (SDH). We reported a patient who was treated with epidural blood patch (EBP) five times for refractory SIH and SDH surgery. Recent Findings We experienced that targeted EBP was useful in refractory SIH, and also can be performed safely prior to drainage of the hematoma. Summary We report the case of SIH patient with a bilateral SDH who came to our hospital and was discharged 2 weeks later with no neurologic deficit after trephination and five times EBP treatment. It shows that targeted EBP was useful in refractory SIH, and also can be performed safely prior to drainage of the hematoma.

Management of an intracranial hypotension patient with diplopia as the primary symptom: A case report.

BACKGROUND: Intracranial hypotension (IH) is a disorder involving cerebrospinal fluid (CSF) hypovolemia due to spontaneous or traumatic spinal CSF leakage and is easily being misdiagnosed or missed, especially in these patients without the prototypical manifestation of an orthostatic headache. At present, the management of IH with both cranial nerve VI palsy and bilateral subdural hematomas (SDHs) is still unclear. CASE SUMMARY: A 67-year-old male Chinese patient complained of diplopia on the left side for one and a half mo. Computed tomography revealed bilateral SDHs and a midline shift. However, neurotrophic drugs were not effective, and 3 d after admission, he developed a non-orthostatic headache and neck stiffness. Enhanced magnetic resonance imaging revealed dural enhancement as an additional feature, and IH was suspected. Magnetic resonance myelography was then adopted and showed CSF leakage at multiple sites in the spine, confirming the diagnosis of having IH. The patient fully recovered following multiple targeted epidural blood patch (EBP) procedures. CONCLUSION: IH is a rare disease, and to the best of our knowledge, IH with diplopia as its initial and primary symptom has never been reported. In this study, we also elucidated that it could be safe and effective to treat IH patients with associated cranial nerve VI palsy and bilateral SDHs using repeated EBP therapy.

Retinal nerve fiber layer characteristics in patients with spontaneous intracranial hypotension: a retrospective case series.

OBJECTIVE: To evaluate changes in retinal nerve fiber layer (RNFL) and macular thicknesses, included ganglion cell-inner plexiform layer (GCIPL) thickness, in patients with spontaneous intracranial hypotension (SIH). METHODS: This was a retrospective, nonrandom, observational case series study. Comprehensive ophthalmic examinations and systemic examinations were performed. Spectral domain optical coherence tomography angiography scanning was used to measure peripapillary RNFL thickness and macular volume. RESULTS: In total, 108 eyes in 54 patients with SIH were evaluated; these were compared with 108 eyes in 54 healthy controls. The mean ages were 38.2 ± 9.4 years (patients with SIH) and 38.9 ± 9.4 years (healthy controls). In both groups, 33 patients were women (61.1%). The peripapillary RNFL and GCIPL were thinner in patients with SIH than in healthy controls (100.08 ± 9.94 µm vs 104.83 ± 8.35 µm and 81.46 ± 5.67 µm vs 85.67 ± 4.57 µm, respectively). Among patients with SIH, the GCIPL was thinner in patients with visual field defects (79.81 ± 5.62 µm vs 82.39 ± 5.12 µm). CONCLUSIONS: The RNFL and GCIPL were thinner in patients with SIH than in healthy controls. The GCIPL was thinner in eyes with visual field defects among patients with SIH.

[Targeted epidural blood patch: an effective treatment for spontaneous intracranial hypotension].

OBJECTIVE: To report the authors' own experience in the treatment of spontaneous intracranial hypotension (SIH) with targeted epidural blood patch after locating the CSF leaks with CT myelography (CTM) and to discuss the mechanism and value of this method. METHODS: Six patients with SIH (3 males and 3 females aged 33-66 years) were included. All patients met the diagnostic criteria of 2nd edition of International Classification of Headache Disorders (2004). CT myelography revealed the CSF leak sites in all 6 patients. Autologous blood mixed with Omnipaque (300 mg/ml) was injected after selective puncture at the leak site indicated by CTM. RESULTS: All patients responded well to the treatment and achieved a complete resolution of symptoms. Nerve root irritation occurred in 2 patients. Three patients suffered from a temporary back pain. CONCLUSION: With the location of leak sites by CTM, targeted epidural blood patch is an effective way to treat spontaneous intracranial hypotension.

Targeted Epidural Blood Patch Treatment for Refractory Spontaneous Intracranial Hypotension in China.

Objective An epidural blood patch (EBP) is the mainstay of treatment for refractory spontaneous intracranial hypotension (SIH). We evaluated the treatment efficacy of targeted EBP in refractory SIH. Methods All patients underwent brain magnetic resonance imaging (MRI) with contrast and heavily T2-weighted spine MRI. Whole spine computed tomography (CT) myelography with non-ionic contrast was performed in 46 patients, and whole spine MR myelography with intrathecal gadolinium was performed in 119 patients. Targeted EBPs were placed in the prone position one or two vertebral levels below the cerebrospinal fluid (CSF) leaks. Repeat EBPs were offered at 1-week intervals to patients with persistent symptoms, continued CSF leakage, or with multiple leakage sites. Results Brain MRIs showed pachymeningeal enhancement in 127 patients and subdural hematomas in 32 patients. One hundred fifty-two patients had CSF leakages on heavily T2-weighted spine MRIs. CSF leaks were also detected on CT and MR myelography in 43 and 111 patients, respectively. Good recovery was achieved in all patients after targeted EBP. No serious complications occurred in patients treated with targeted EBP during the 1 to 7 years of follow-up. Conclusions Targeted and repeat EBPs are rational choices for treatment of refractory SIH caused by CSF leakage.