Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

BACKGROUND AND PURPOSE: Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS: This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS: Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS: Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.

Distinct Pattern of Membrane Formation With Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension.

BACKGROUND AND OBJECTIVES: To systematically describe pertinent, intraoperative anatomic findings encountered when approaching spinal cerebrospinal fluid (CSF) leaks and CSF-venous fistulas in spontaneous intracranial hypotension (SIH). METHODS: In a retrospective study, we included surgically treated patients suffering from SIH at our institution from April 2018 to March 2022. Anatomic, intraoperative data were extracted from operative notes and supplemented with data from surgical videos and images. Prominent anatomic features were compared among different types of CSF leaks. RESULTS: The study cohort consists of 120 patients with a mean age of 45.2 years. We found four distinct patterns of spinal membranes specifically associated with different types of CSF leaks: (i) thick, dorsal membranes, which were hypervascular and may mimic the dura (pseudodura); (ii) thin, lateral membranes encapsulating a ventral epidural CSF compartment (confining the spinal longitudinal extradural CSF collection); (iii) ventral membranes constituting a transdural funnel-like CSF channel; and (iv) lateral membranes forming spinal cysts/meningeal diverticulae associated with lateral CSF leaks. The latter three types resemble a layer of arachnoid herniated through the dural defect. CONCLUSION: We describe four distinct spinal (neo-)membranes in association with spinal CSF leaks. Formation of these membranes, or emergence by herniation of arachnoid through a dural defect, constitutes a specific pathoanatomic feature of patients with SIH and CSF leaks. Recognition of these membranes is of paramount importance for diagnosis and treatment of patients with spinal CSF leaks.

Recovery and long-term outcome after neurosurgical closure of spinal CSF leaks in patients with spontaneous intracranial hypotension.

INTRODUCTION: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid leak causes orthostatic headaches and impacts quality of life. Successful closure rates are often reported, whereas data on long-term outcome are still scarce. METHODS: Between April 2020 and December 2022 surgically treated patients completed the Headache Impact Test-6 prior to surgery and at 14 days, three months, six months, and 12 months postoperatively. In addition to the Headache Impact Test-6 score, we extracted data related to orthostatic symptoms. RESULTS: Eighty patients were included. Median Headache Impact Test-6 score preoperatively was 65 (IQR 61-69), indicating severe and disabling impact of headaches. At three months headache impact significantly improved to 49 (IQR 44-58) (p < 0.001) and remained stable up to 12 months (48, IQR 40-56), indicating little to no impact of headaches on quality of life. The need to lie down "always" or "very often" was reduced from 79% to 23% three months postoperatively (p < 0.001). CONCLUSIONS: Surgical closure of spinal CSF leaks significantly improves the impact of headaches in the long term. At least three months should be expected for recovery. Despite permanent closure of the CSF-leak, a quarter of patients still have relevant long-term impairment, indicating the need for further research on its cause and possible treatment.

Intraoperative Visualization of Flow in Direct Cerebrospinal Fluid-Venous Fistulas Using Intrathecal Fluorescein.

BACKGROUND: Cerebrospinal fluid-venous fistulas (CVFs) are a rare cause of spontaneous intracranial hypotension. Intraoperatively, CVFs are not readily identifiable and difficult to differentiate from normal veins. OBJECTIVE: To assess the utility of intrathecal fluorescein injection intraoperatively to visualize and identify the CVF. METHODS: We report a case series of patients treated surgically for a CVF. After surgical exposure, we injected intrathecal fluorescein (5-10 mg) through a lumbar catheter, which we placed immediately before surgery. RESULTS: Four patients with spontaneous intracranial hypotension with a suspected CVF underwent surgical ligation with adjunctive intrathecal fluorescein application. Intraoperative fluorescein injection confirmed the presence of a CVF in 3 cases. In 2 cases, we observed rapid fluorescein filling of a single epidural vein constituting the CVF. Other epidural vessels did not fill with fluorescein. In 1 case, fluorescein helped to identify a residual CVF after previous incomplete embolization. In the fourth case, no CVF was found intraoperatively. By contrast, a meningeal nerve root diverticulum was visualized, wrapped, and clipped. CONCLUSION: We demonstrate for the first time the direct intraoperative visualization of CVF using intrathecal fluorescein. CVF can be identified intraoperatively using fluorescein dye, which can be a valuable adjunct for the surgeon confronted with this disease.

Minimally invasive surgery for spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension.

OBJECTIVE: Spinal CSF leaks cause spontaneous intracranial hypotension (SIH). Surgical closure of spinal CSF leaks is the treatment of choice for persisting leaks. Surgical approaches vary, and there are no studies in which minimally invasive techniques were used. In this study, the authors aimed to detail the safety and feasibility of minimally invasive microsurgical sealing of spinal CSF leaks using nonexpandable tubular retractors. METHODS: Consecutive patients with SIH and a confirmed spinal CSF leak treated at a single institution between April 2019 and December 2020 were included in the study. Surgery was performed via a dorsal 2.5-cm skin incision using nonexpandable tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach. The primary outcome was successful sealing of the dura, and the secondary outcome was the occurrence of complications. RESULTS: Fifty-eight patients, 65.5% of whom were female (median age 46 years [IQR 36-55 years]), with 38 ventral leaks, 17 lateral leaks, and 2 CSF venous fistulas were included. In 56 (96.6%) patients, the leak could be closed, and in 2 (3.4%) patients the leak was missed because of misinterpretation of the imaging studies. One of these patients underwent successful reoperation, and the other patient decided to undergo surgery at another institution. Two other patients had to undergo reoperation because of insufficient closure and a persisting leak. The rate of permanent neurological deficit was 1.7%, the revision rate for a persisting or recurring leak was 3.4%, and the overall revision rate was 10.3%. The rate of successful sealing during the primary closure attempt was 96.6% and 3.4% patients needed a secondary attempt. Clinical short-term outcome at discharge was unchanged in 14 patients and improved in 25 patients, and 19 patients had signs of rebound intracranial hypertension. CONCLUSIONS: Minimally invasive surgery with tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach is safe and effective for the treatment of spinal CSF leaks. The authors suggest performing a minimally invasive closure of spinal CSF leaks in specialized centers.

Augmented reality-assisted craniofacial reconstruction in skull base lesions - an innovative technique for single-step resection and cranioplasty in neurosurgery.

Defects of the cranial vault often require cosmetic reconstruction with patient-specific implants, particularly in cases of craniofacial involvement. However, fabrication takes time and is expensive; therefore, efforts must be made to develop more rapidly available and more cost-effective alternatives. The current study investigated the feasibility of an augmented reality (AR)-assisted single-step procedure for repairing bony defects involving the facial skeleton and the skull base. In an experimental setting, nine neurosurgeons fabricated AR-assisted and conventionally shaped ("freehand") implants from polymethylmethacrylate (PMMA) on a skull model with a craniofacial bony defect. Deviations of the surface profile in comparison with the original model were quantified by means of volumetry, and the cosmetic results were evaluated using a multicomponent scoring system, each by two blinded neurosurgeons. Handling the AR equipment proved to be quite comfortable. The median volume deviating from the surface profile of the original model was low in the AR-assisted implants (6.40 cm3) and significantly reduced in comparison with the conventionally shaped implants (13.48 cm3). The cosmetic appearance of the AR-assisted implants was rated as very good (median 25.00 out of 30 points) and significantly improved in comparison with the conventionally shaped implants (median 14.75 out of 30 points). Our experiments showed outstanding results regarding the possibilities of AR-assisted procedures for single-step reconstruction of craniofacial defects. Although patient-specific implants still represent the gold standard in esthetic aspects, AR-assisted procedures hold high potential for an immediately and widely available, cost-effective alternative providing excellent cosmetic outcomes.