Introduction: Hemangioblastomas are rare, histologically benign, highly vascularized tumors of the brain, the spinal cord, and the retina, occurring sporadically or associated with the autosomal dominant inherited von Hippel-Lindau (VHL) disease. Children or adults with VHL disease have one of > 300 known germline mutations of the VHL gene located on chromosome 3. They are prone to develop hemangioblastomas, extremely rarely starting at age 6, rarely at age 12–18, and, typically and almost all, as adults. There is a plethora of VHL-associated tumors and cysts, mainly in the kidney, pancreas, adrenals, reproductive organs, and central nervous system. Due to a lack of causal treatment, alleviation of symptoms and prevention of permanent neurological deficits as well as malignant transformation are the main task. Paucity of data and the nonlinear course of tumor progression make management of pediatric VHL patients with hemangioblastomas challenging. Methods: The Freiburg surveillance protocol was developed by combining data from the literature and our experience of examinations of > 300 VHL patients per year at our university VHL center. Results: Key recommendations are to start screening of patients at risk by funduscopy with dilated pupils for retinal tumors with admission to school and with MRI of the brain and spinal cord at age 14, then continue biannually until age 18, with emergency MRI in case of neurological symptoms. Indication for surgery remains personalized and should be approved by an experienced VHL board, but we regard neurological symptoms, rapid tumor growth, or critically large tumor/cyst sizes as the key indications to remove hemangioblastomas. Since repeated surgery on hemangioblastomas in VHL patients is not rare, modern neurosurgical techniques should encompass microsurgery, neuronavigation, intraoperative neuromonitoring, fluorescein dye-based intraoperative angiography, intraoperative ultrasound, and minimally invasive approaches, preceded in selected cases by endovascular embolization. Highly specialized neurosurgeons are able to achieve a very low risk of permanent morbidity for the removal of hemangioblastomas from the cerebellum and spinal cord. Small retinal tumors of the peripheral retina can be treated by laser coagulation, larger tumors by cryocoagulation or brachytherapy. Conclusion: We consider management at experienced VHL centers mandatory and careful surveillance and monitoring of asymptomatic lesions are required to prevent unnecessary operations and minimize morbidity.

Purpose. To assess the frequency, risk factors, and management of accidental durotomy in minimally invasive transforaminal lumbar interbody fusion (MIS TLIF). Methods. This single‐center study retrospectively investigates 372 patients who underwent MIS TLIF and were mobilized within 24 hours after surgery. The frequency of accidental durotomies, intraoperative closure technique, body mass index, and history of previous surgery was recorded. Results. We identified 32 accidental durotomies in 514 MIS TLIF levels (6.2%). Analysis showed a statistically significant relation of accidental durotomies to overweight patients (body mass index ≥25 kg/m²; P = 0.0493). Patient age older than 65 years tended to be a positive predictor for accidental durotomies (P = 0.0657). Mobilizing patients on the first postoperative day, we observed no durotomy‐associated complications. Conclusions. The frequency of accidental durotomies in MIS TLIF is low, with overweight being a risk factor for accidental durotomies. The minimally invasive approach seems to minimize durotomy‐associated complications (CSF leakage, pseudomeningocele) because of the limited dead space in the soft tissue. Patients with accidental durotomy can usually be mobilized within 24 hours after MIS TLIF without increased risk. The minimally invasive TLIF technique might thus be beneficial in the prevention of postoperative immobilization‐associated complications such as venous thromboembolism. This trial is registered with DRKS00006135.

OBJECT: The aim of the study was to investigate the safety and efficacy of minimally invasive tubular microdiscectomy for the treatment of recurrent lumbar disc herniation (LDH). As opposed to endoscopic techniques, namely microendoscopic and endoscopic transforaminal discectomy, this microscopically assisted technique has never been used for the treatment of recurrent LDH. METHODS: Thirty consecutive patients who underwent minimally invasive tubular microdiscectomy for recurrent LDH were included in the study. The preoperative and postoperative visual analog scale (VAS) scores for pain, the clinical outcome according to modified Macnab criteria, and complications were analyzed retrospectively. The minimum follow-up was 1.5 years. Student t-test with paired samples was used for the statistical comparison of pre- and postoperative VAS scores. A p value < 0.05 was considered to be statistically significant. RESULTS: The mean operating time was 90 ± 35 minutes. The VAS score for leg pain was significantly reduced from 5.9 ± 2.1 preoperatively to 1.7 ± 1.3 postoperatively (p < 0.001). The overall success rate (excellent or good outcome according to Macnab criteria) was 90%. Incidental durotomy occurred in 5 patients (16.7%) without neurological consequences, CSF fistula, or negative influence to the clinical outcome. Instability occurred in 2 patients (6.7%). CONCLUSIONS: The clinical outcome of minimally invasive tubular microdiscectomy is comparable to the reported success rates of other minimally invasive techniques. The dural tear rate is not associated to higher morbidity or worse outcome. The technique is an equally effective and safe treatment option for recurrent LDH.

Presentation of a patient with acute subarachnoid hemorrhage from a ruptured spinal artery aneurysm attributable to exacerbated Sjögren syndrome. A 46-year-old woman with symptoms of exacerbated Sjögren syndrome experienced the acute onset of extreme headache accompanied by nuchal rigidity. A computed tomographic scan revealed subarachnoid hemorrhage. Angiography showed an isolated aneurysm of a branch of the right vertebral artery that was a feeding artery of the anterior spinal artery. Neither operative clipping nor endovascular coiling of the aneurysm was reasonable, owing to the high risk of occluding the anterior spinal artery during the intervention. Further diagnostic measures confirmed Sjögren syndrome and revealed cryoglobulinemic vasculitis, membranoproliferative glomerulonephritis with acute renal failure, Hashimoto thyroiditis, and acute hydrocephalus. In the course of conservative treatment, the patient recovered completely from the subarachnoid hemorrhage. One year after treatment with glucocorticoids and immunosuppressive agents, both the aneurysm and the vasculitis could no longer be detected on conventional angiography. Generally, spinal artery aneurysms are exceptionally rare, and few cases of rupture with subsequent subarachnoid hemorrhage have been published. We report on a ruptured spinal aneurysm attributable to Sjögren syndrome-associated cryoglobulinemic vasculitis. Conservative treatment with glucocorticoids and immunosuppressive agents led to resolution of the vasculitic spinal aneurysm.