scholarly journals Orthostatic hypotension after cervicomedullary junction surgery: illustrative case

2021 ◽  
Vol 2 (20) ◽  
Author(s):  
Kasper S. Jacobsen ◽  
Rico F. Schou ◽  
Frantz R. Poulsen ◽  
Christian B. Pedersen
Keyword(s):  
Orthostatic Hypotension ◽  
Vocal Cord Paralysis ◽  
Illustrative Case ◽  
Resonance Imaging ◽  
Von Hippel Lindau ◽  
Cervicomedullary Junction ◽  
Repeated Surgery ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Left Vagus

BACKGROUND Surgery at the cervicomedullary junction carries a risk of damaging vital brainstem functions. Because the nucleus of the solitary tract (NS) is involved in the baroreceptor reflex, damage to its integrity may lead to orthostatic hypotension. OBSERVATIONS A 56-year-old man with a medical history of hypertension, von Hippel-Lindau disease, and previous bilateral adrenalectomy due to pheochromocytoma was referred with symptoms of dysphagia and paralysis of the left vocal cord. Paralysis of the left vagus nerve was suspected. Magnetic resonance imaging revealed a contrast-enhancing cystic process in the cervicomedullary junction. Twenty-three years earlier, the patient had undergone surgical treatment for a hemangioblastoma in the same region. After repeated surgery, the patient temporarily developed orthostatic hypotension. At discharge, the patient no longer needed antihypertensive medication. LESSONS Surgery near the cervicomedullary junction can affect the NS, leading to disruption of the baroreceptor response that regulates blood pressure.

scholarly journals Sacral hemangioblastoma in a patient with von Hippel–Lindau disease

2003 ◽  
Vol 15 (2) ◽  
pp. 1-4 ◽  
Author(s):  
Ryszard M. Pluta ◽  
Scott D. Wait ◽  
John A. Butman ◽  
Kathleen A. Leppig ◽  
Alexander O. Vortmeyer ◽  
...  
Keyword(s):  
Nerve Root ◽  
Histopathological Diagnosis ◽  
Resonance Imaging ◽  
Nerve Roots ◽  
Von Hippel Lindau ◽  
Sacral Nerve ◽  
Left Lower Extremity ◽  
History Of ◽  
Von Hippel Lindau Disease

Hemangioblastomas are histologically benign neoplasms that occur sporadically or as part of von Hippel–Lindau disease. Hemangioblastomas may occur anywhere along the neuraxis, but sacral hemangioblastomas are extremely rare. To identify features that will help guide the operative and clinical management of these lesions, the authors describe the management of a large von Hippel–Lindau disease–associated sacral hemangioblastoma and review the literature. The authors present the case of a 38-year-old woman with von Hippel–Lindau disease and a 10-year history of progressive back pain, as well as left lower-extremity pain and numbness. Neurological examination revealed decreased sensation in the left S-1 and S-2 dermatomes. Magnetic resonance imaging demonstrated a large enhancing lesion in the sacral region, with associated erosion of the sacrum. The patient underwent arteriography and embolization of the tumor and then resection. The histopathological diagnosis was consistent with hemangioblastoma and showed intrafascicular tumor infiltration of the S-2 nerve root. At 1-year follow-up examination, pain had resolved and numbness improved. Sacral nerve root hemangioblastomas may be safely removed in most patients, resulting in stabilization or improvement in symptomatology. Generally, hemangioblastomas of the sacral nerve roots should be removed when they cause symptoms. Because they originate from the nerve root, the nerve root from which the hemangioblastoma originates must be sacrificed to achieve complete resection.

Hemangioblastoma diagnosis and surveillance in von Hippel–Lindau disease: a consensus statement

2021 ◽  
pp. 1-6
Author(s):  
Kristin Huntoon ◽  
Matthew J. Shepard ◽  
Rimas V. Lukas ◽  
Ian E. McCutcheon ◽  
Anthony B. Daniels ◽  
...  
Keyword(s):  
Age At Onset ◽  
Benign Tumors ◽  
Expert Committee ◽  
Level Of Evidence ◽  
Von Hippel Lindau ◽  
Assessment Development ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Cancer Network

OBJECTIVE Hemangioblastomas are a frequent underlying cause of neurological morbidity and death in patients with von Hippel–Lindau disease (VHL). Although these benign tumors can cause significant neurological debility when undetected and untreated, unified evidence-based surveillance recommendations for VHL patients have not been established. To develop consensus recommendations, the VHL Alliance established an expert committee, named the International VHL Surveillance Guidelines Consortium, to define surveillance recommendations. METHODS The Central Nervous System (CNS) Hemangioblastoma Subcommittee of the Guidelines Consortium was formed as a multidisciplinary team of experts in the diagnosis and management of hemangioblastomas. Recommendations were formulated using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) and National Comprehensive Cancer Network Categories of Evidence and Consensus categorization after a comprehensive literature review. RESULTS Published studies (n = 49) that discussed age at onset, MRI frequency, natural history of VHL, and the risks and benefits of surveillance were analyzed. Based on this analysis, the authors recommend that clinical evaluation (yearly) be used as the primary screening tool for hemangioblastomas in VHL. The subcommittee suggests that screening be performed between the ages of 11 and 65 years, or with the onset of symptoms, for synchronicity with other testing regimens in VHL. The subcommittee also recommends that baseline MRI be first performed at the age of 11 years (suggested 2B, level of evidence D) or after identification of neurological symptoms or signs (if earlier) and continue every 2 years (recommended 2A, level of evidence A). CONCLUSIONS The CNS Hemangioblastoma Subcommittee of the International VHL Surveillance Guidelines Consortium here proposes guidelines that aim to increase the early detection of VHL-associated hemangioblastomas to reduce their morbidity and mortality.

scholarly journals Extra and intradural spinal Hemangioblastoma

2012 ◽  
Vol 11 (3) ◽  
pp. 242-244
Author(s):  
Marcelo Campos Moraes Amato ◽  
Caio César Marconato Simões Matias ◽  
João Alberto Assirati Junior ◽  
Aline Paixão Becker ◽  
Carlos Gilberto Carlotti Junior ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Correct Diagnosis ◽  
White Male ◽  
Residual Tumor ◽  
Illustrative Case ◽  
Low Grade ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Spinal Hemangioblastoma

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.

scholarly journals von Hippel-Lindau Disease: an Update

2019 ◽  
Vol 7 (4) ◽  
pp. 227-235 ◽  
Author(s):  
Eamonn R Maher ◽  
Richard N Sandford
Keyword(s):  
Natural History ◽  
Patient Outcomes ◽  
Molecular Characterisation ◽  
Von Hippel Lindau ◽  
Functional Characterisation ◽  
History Of ◽  
Vhl Protein ◽  
Von Hippel Lindau Disease ◽  
Vhl Disease ◽  
Improve Patient

Abstract Purpose of Review In this review, we discuss the key molecular and clinical developments in VHL disease that have the potential to impact on the natural history of the disease and improve patient outcomes. Recent Findings Identifiable mutations in VHL underlie most cases of VHL and define clear genotype-phenotype correlations. Detailed clinical and molecular characterisation has allowed the implementation of lifelong screening programmes that have improved clinical outcomes. Functional characterisation of the VHL protein complex has revealed its role in oxygen sensing and the mechanisms of tumourigenesis that are now being exploited to develop novel therapies for VHL and renal cancer. Summary The molecular and cellular landscape of VHL-associated tumours is revealing new opportunities to modify the natural history of the disease and develop therapies. Drugs are now entering clinical trials and combined with improved clinical and molecular diagnosis, and lifelong surveillance programmes, further progress towards reducing the morbidity and mortality associated with VHL disease is anticipated.

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

1997 ◽  
Vol 87 (3) ◽  
pp. 445-449 ◽  
Author(s):  
Jean-Christophe Ouallet ◽  
Kathlyn Marsot-Dupuch ◽  
Remy Van Effenterre ◽  
Michele Kujas ◽  
Jean-Michel Tubiana
Keyword(s):  
Temporal Bone ◽  
Imaging Techniques ◽  
Endolymphatic Sac ◽  
Cystic Tumor ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Magnetic Resonance Imaging Techniques ◽  
Temporal Bone Tumor

✓ This report describes a patient with von Hippel—Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.

Clinical Features and Natural History of von Hippel-Lindau Disease

QJM ◽  
1990 ◽  
Vol 77 (2) ◽  
pp. 1151-1163 ◽  
Author(s):  
E. R. MAHER ◽  
J. R. W. YATES ◽  
R. HARRIES ◽  
C. BENJAMIN ◽  
R. HARRIS ◽  
...  
Keyword(s):  
Natural History ◽  
Clinical Features ◽  
Von Hippel Lindau ◽  
History Of ◽  
Von Hippel Lindau Disease

Retinal hemangioblastoma: prevalence, incidence and frequency of underlying von Hippel-Lindau disease

2017 ◽  
Vol 102 (7) ◽  
pp. 942-947 ◽  
Author(s):  
Marie Louise Mølgaard Binderup ◽  
Anne-Sophie Stendell ◽  
Michael Galanakis ◽  
Hans Ulrik Møller ◽  
Jens F Kiilgaard ◽  
...  
Keyword(s):  
National Study ◽  
Research Database ◽  
Von Hippel Lindau ◽  
Younger Age ◽  
National Patient ◽  
National Cohort ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Almost All ◽  
Retinal Hemangioblastoma

Background and aimsWe aimed to determine the frequency of von Hippel-Lindau disease (vHL) as the underlying cause of retinal hemangioblastoma and to estimate retinal hemangioblastoma incidence and prevalence in a national cohort study.MethodsThrough the national patient register and vHL research database, we identified 81 patients diagnosed with a retinal hemangioblastoma in Denmark between 1977 and 2014. Consent was obtained for 54 living and 10 deceased patients with retinal hemangioblastoma. For each participant, we collected medical records and family information. Almost all (63 of 64) participants were or had previously been tested for mutations in the VHL gene.ResultsOverall, 84% of the participants (54 of the 64) had vHL. Compared with the non-vHL patients, the vHL patients had their first retinal hemangioblastoma at a younger age (22.5 vs 40 years), and were more likely to have an asymptomatic first hemangioblastoma (80% vs 20%). Overall, 76% (41 of 54) of the vHL patients had a family history of vHL, while none of the patients without vHL did. Despite the rarity of the disease, on average more than eight new tumours are diagnosed each year due to multiple tumour development in vHL patients. The estimated prevalence of patients with retinal hemangioblastoma was up to 1 in 73 080 individuals.ConclusionIn the first national study in which almost all participants were genetically tested, vHL was the underlying cause of retinal hemangioblastoma in 84% of cases; more often than previously reported. We recommend that genetic and clinical vHL screening should be performed in all patients with retinal hemangioblastoma.

Bilateral Vocal Cord Paralysis with Shy-Drager Syndrome

1993 ◽  
Vol 109 (5) ◽  
pp. 911-914 ◽  
Author(s):  
Rohit Bawa ◽  
Hassan H. Ramadan ◽  
Stephen J. Wetmore
Keyword(s):  
Orthostatic Hypotension ◽  
Vocal Cord ◽  
Vocal Cord Paralysis ◽  
English Literature ◽  
System Failure ◽  
Bilateral Vocal Cord Paralysis ◽  
Vocal Cord Paresis ◽  
Median Position ◽  
History Of ◽  
Emergency Tracheotomy

Shy-Drager syndrome consists of progressive autonomic nervous system failure with Parkinson's disease-like symptoms and orthostatic hypotension. It can also result in airway compromise from bilateral vocal cord paralysis. Fewer than 30 cases of severe bilateral vocal cord paresis or paralysis associated with the Shy-Drager syndrome have been reported in the English literature. We present a case of a 72-year-old man who had a 2-year history of orthostatic hypotension, neurogenic bladder, impotence, anhydrosis, and extremity weakness and paresthesias. Hoarseness and dyspnea with stridor developed as a result of bilateral vocal cord paralysis in the median position and required an emergency tracheotomy. This combination of symptoms resulted in the diagnosis of Shy-Drager syndrome. We present the case along with literature review of bilateral vocal cord paralysis with the Shy-Drager syndrome.

Treatment of Intramedullary Hemangioblastomas, with Special Attention to von Hippel-Lindau Disease

Neurosurgery ◽  
2003 ◽  
Vol 53 (6) ◽  
pp. 1306-1314 ◽  
Author(s):  
Vera Van Velthoven ◽  
Peter C. Reinacher ◽  
Joachim Klisch ◽  
Hartmut P.H. Neumann ◽  
Sven Gläsker
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Timing Of Surgery ◽  
Vascular Tumors ◽  
Resonance Imaging ◽  
Surgical Morbidity ◽  
Von Hippel Lindau ◽  
Asymptomatic Patients ◽  
Von Hippel Lindau Disease

Abstract OBJECTIVE Hemangioblastomas of the central nervous system are rare vascular tumors that can occur as sporadic lesions or as component tumors of autosomal dominant von Hippel-Lindau disease. With the availability of magnetic resonance imaging, asymptomatic tumors are detected more frequently, especially among patients with von Hippel-Lindau disease, and the questions of whether and when these lesions should be treated arise. To identify surgical outcomes and the timing of surgery for intramedullary hemangioblastomas, we retrospectively analyzed data for a series of 28 consecutive patients whom we surgically treated for intramedullary hemangioblastomas in the past 10 years. METHODS All tumors were completely removed. Functional grades, according to the McCormick scale, were determined before and after surgery and in follow-up assessments. Several clinical characteristics were correlated with changes in functional grades in follow-up assessments, compared with preoperative grades. RESULTS Functional grades in follow-up assessments improved for 28.6% of the patients and remained unchanged for 71.4%. No patient was in worse condition, compared with preoperative status. Peritumoral edema on preoperative magnetic resonance imaging scans was correlated with significantly higher surgical morbidity rates. Four asymptomatic patients were surgically treated because of tumor or pseudocyst progression on serial magnetic resonance imaging scans. All of those patients remained asymptomatic postoperatively. CONCLUSION Intramedullary hemangioblastomas can be removed with low surgical morbidity rates and excellent long-term prognoses. The timing of surgery for patients with von Hippel-Lindau disease and multiple lesions remains a matter of debate. On the basis of our data, we established the strategy of operating also on asymptomatic lesions that exhibit radiological progression, before significant neurological deficits occur, which are often not reversible.

Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery

1996 ◽  
Vol 84 (6) ◽  
pp. 1046-1050 ◽  
Author(s):  
Paul D. Sawin ◽  
Kenneth A. Follett ◽  
B. Chen Wen ◽  
Edward R. Laws
Keyword(s):  
Residual Tumor ◽  
Subtotal Resection ◽  
Resonance Imaging ◽  
Subsequent Growth ◽  
Conventional Radiotherapy ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Adjuvant Therapies ◽  
Von Hippel Lindau Disease ◽  
Fine Print

✓ The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel—Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.

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