Cavernous malformations of the hypothalamus: a single-institution series of 12 cases and review of the literature

2021 ◽  
pp. 1-10
Author(s):  
Anadjeet S. Khahera ◽  
Yiping Li ◽  
Gary K. Steinberg
Keyword(s):  
Short Term Memory ◽  
Mass Effect ◽  
Senior Author ◽  
Functional Improvement ◽  
Acute Hemorrhage ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Radiological Measurements ◽  
Insight Into

OBJECTIVE There remains a paucity of literature on hypothalamic cavernous malformations (HCMs). Here, the authors present the largest series of HCMs to date and review the literature to gain additional insight into this rare disease subset. METHODS A prospectively managed database was retrospectively reviewed for patients diagnosed with symptomatic HCM and treated surgically between 1987 and 2019. Data gathered included demographics, presenting signs, radiological measurements, surgical approach, and postoperative events. Functional outcome was measured using the modified Rankin Scale (mRS) and Glasgow Outcome Scale–Extended (GOSE) pre- and postoperatively. A PRISMA guideline systematic review of HCM in the literature was performed. RESULTS Our cohort study consisted of 12 patients with symptomatic, and radiographically confirmed, HCM treated with microsurgery by the senior author (G.K.S.). An additional 16 surgically or conservatively managed patients were also identified from the literature, and the authors analyzed the data of all 28 patients (with 54% of patients being male; mean age 39 ± 16 years, range 10–68 years). Patients harboring HCMs most commonly presented with headache (16/28, 57%), short-term memory impairment (11/28, 39%), and gait disturbance (8/28, 32%). Radiographically, lesions most commonly involved the mammillary region (18/23, 78%), the tuberal/infundibulum region (13/23, 57%), and the preoptic/lamina terminalis region (12/23, 52%), with a mean diameter of 2.5 ± 1.4 cm (range 0.8–7 cm) at presentation. Acute hemorrhage was identified in 96% (23/24) of patients on presentation, with 96% (23/24) intraparenchymal and 29% (7/24) intraventricular. Of 24 patients who were managed surgically, gross-total resection (GTR) was achieved in 88% (21/24) of cases. There were no reports of perioperative infarction or mortality. With a mean follow-up period of 41 months (range 0.5–309 months), 77% (20/26) of patients experienced functional improvement, while 12% (3/26) had no change, and 12% (3/26) experienced increased disability. In our cohort of 12 patients, 83% (10/12) continued to report symptoms at the last follow-up (mean 4.8 years, range 0.1–25.7 years). However, there was a significant improvement in mRS score noted after surgery (mean 1.4 vs 3.1, p = 0.0026) and a trend toward improvement in GOSE score (mean 6.3 vs 5.1, p = 0.09). CONCLUSIONS Hemorrhage from HCMs can cause a symptomatic mass effect on adjacent eloquent structures. While patients are unlikely to be deficit free following surgery, GTR allows for functional improvement and reduces recurrent hemorrhage rates. Microsurgery remains a viable option for symptomatic HCMs in experienced hands.

Contralateral Interhemispheric Approach to Deep-Seated Cavernous Malformations

Neurosurgery ◽  
2014 ◽  
Vol 75 (1) ◽  
pp. 80-86 ◽  
Author(s):  
Hasan A. Zaidi ◽  
Shakeel A. Chowdhry ◽  
Peter Nakaji ◽  
Adib A. Abla ◽  
Robert F. Spetzler
Keyword(s):  
Clinical Outcomes ◽  
Chart Review ◽  
Short Term Memory ◽  
Demographic Data ◽  
Retrospective Chart Review ◽  
Senior Author ◽  
Cavernous Malformations ◽  
Memory Problems ◽  
Interhemispheric Approach

Abstract BACKGROUND: Deep-seated periventricular cavernous malformations of the basal ganglia or thalamus can be approached via an interhemispheric craniotomy. OBJECTIVE: To determine surgical efficacy and clinical outcomes of the contralateral interhemispheric approach. METHODS: Retrospective chart review was performed on patients undergoing an interhemispheric approach for the resection of deep-seated cavernous malformation by the senior author (R.F.S.) between 2005 and 2013. Demographic data and clinical outcomes were reviewed. Pre- and postoperative imaging were analyzed for lesion location, size, associated venous anomaly, proximity to ventricle, and presence of residual. RESULTS: Twenty-one patients underwent a contralateral interhemispheric-transventricular approach, 7 patients had a contralateral interhemispheric-transcingulate approach and 3 patients had a contralateral interhemispheric-transchoroidal approach. Mean age was 40.1 years, and the majority were female (58.1%). Mean maximum cavernoma diameter was 1.97 cm, and 43.8% reached the surface of the ventricle. Average follow-up was 8.9 months, with complete resection achieved in 96.8% of patients. At last follow-up, 61.3% of patients remained stable and 29.0% had improved. Of the patients, 6.5% experienced transient weakness that resolved at last follow-up, and 1 patient (3.2%) had short-term memory problems. There were no surgical mortalities. CONCLUSION: The contralateral interhemispheric approach is a safe, clinically well tolerated, and surgically efficacious approach to deep-seated cavernomas.

Cavernous malformations of the basal ganglia in children

2013 ◽  
Vol 12 (2) ◽  
pp. 171-174 ◽  
Author(s):  
Bradley A. Gross ◽  
Edward R. Smith ◽  
R. Michael Scott
Keyword(s):  
Basal Ganglia ◽  
Mass Effect ◽  
Neurological Impairment ◽  
Senior Author ◽  
Neurological Deterioration ◽  
Neurological Dysfunction ◽  
Lentiform Nucleus ◽  
Cavernous Malformations ◽  
Female Ratio

Object Cavernous malformations (CMs) of the basal ganglia are relatively rare lesions that can lead to considerable neurological impairment because of their eloquent location. The authors reviewed the clinical course and surgical outcome of a series of children with basal ganglia CMs. Methods The authors retrospectively reviewed the operative experience of the senior author (R.M.S.) and the 1997–2011 database of Boston Children's Hospital for children with CM of the basal ganglia (which includes CM of the caudate and/or lentiform nucleus and excludes CM of the thalamus). They evaluated baseline demographics, presenting signs, operative outcomes, and condition at long-term follow-up visits and compared these characteristics among patients who underwent surgery and those who were observed. Results Of 180 children with a diagnosis of CM, 11 (6%) had CM of the basal ganglia. The mean age at diagnosis was 9.3 years, and the male/female ratio was 1.8:1. Presenting signs were as follows: hemorrhage (8 children), incidental lesions (2), and seizures (1); 2 children had choreiform movement disorders. Treatment was observation or surgery. Observation was chosen for 5 children either because the lesions were asymptomatic (2 children) or because the risk for neurological dysfunction after attempted excision was believed to be high (3 children). These 5 children were observed over a combined total of 30.4 patient-years; none experienced neurological deterioration or symptomatic hemorrhage from their lesions. The other 6 children underwent microsurgical resection of the lesion because they were symptomatic from hemorrhage or increasing mass effect. All 6 of these children had hemorrhagic lesions, of which the smallest dimension was at least 1.5 cm. Of these 6 lesions, 5 were excised completely, and over a combined total of 46 patient-years of follow-up, no rebleeding or late neurological deterioration after surgery was reported. Conclusions In this patient population, the natural history of small and asymptomatic CMs of the basal ganglia was benign. The children with large (> 1.5 cm) symptomatic lesions underwent excision; neurological impairment was apparently minimal, and no hemorrhage or neurological deterioration occurred later.

scholarly journals Magnetic Resonance Thermometry-Guided Stereotactic Laser Ablation of Cavernous Malformations in Drug-Resistant Epilepsy: Imaging and Clinical Results

2015 ◽  
Vol 12 (1) ◽  
pp. 39-48 ◽  
Author(s):  
D Jay McCracken ◽  
Jon T Willie ◽  
Brad A Fernald ◽  
Amit M Saindane ◽  
Daniel L Drane ◽  
...  
Keyword(s):  
Laser Ablation ◽  
Magnetic Resonance ◽  
Minimally Invasive ◽  
Real Time ◽  
Refractory Epilepsy ◽  
Acute Hemorrhage ◽  
Cavernous Malformations ◽  
Remote Imaging ◽  
Medically Refractory Epilepsy

Abstract BACKGROUND Surgery is indicated for cerebral cavernous malformations (CCMs) that cause medically refractory epilepsy. Real-time magnetic resonance thermography (MRT)-guided stereotactic laser ablation (SLA) is a minimally invasive approach to treating focal brain lesions. SLA of CCM has not previously been described. OBJECTIVE To describe MRT-guided SLA, a novel approach to treating CCM-related epilepsy, with respect to feasibility, safety, imaging, and seizure control in 5 consecutive patients. METHODS Five patients with medically refractory epilepsy undergoing standard presurgical evaluation were found to have corresponding lesions fulfilling imaging characteristics of CCM and were prospectively enrolled. Each underwent stereotactic placement of a saline-cooled cannula containing an optical fiber to deliver 980-nm diode laser energy via twist drill craniostomy. MR anatomic imaging was used to evaluate targeting before ablation. MR imaging provided evaluation of targeting and near real-time feedback regarding the extent of tissue thermocoagulation. Patients maintained seizure diaries, and remote imaging (6-21 months postablation) was obtained in all patients. RESULTS Imaging revealed no evidence of acute hemorrhage following fiber placement within presumed CCM. MRT during treatment and immediate postprocedure imaging confirmed the desired extent of ablation. We identified no adverse events or neurological deficits. Four of 5 (80%) patients achieved freedom from disabling seizures after SLA alone (Engel class 1 outcome), with follow-up ranging 12 to 28 months. Reimaging of all subjects (6-21 months) indicated lesion diminution with surrounding liquefactive necrosis, consistent with the surgical goal of extended lesionotomy. CONCLUSION Minimally invasive MRT-guided SLA of epileptogenic CCM is a potentially safe and effective alternative to open resection. Additional experience and longer follow-up are needed.

Coexistence of intracranial and spinal cavernous malformations: a study of prevalence and natural history

2006 ◽  
Vol 104 (3) ◽  
pp. 376-381 ◽  
Author(s):  
Aaron A. Cohen-Gadol ◽  
Jeffrey T. Jacob ◽  
Diane A. Edwards ◽  
William E. Krauss
Keyword(s):  
Family History ◽  
Natural History ◽  
Mr Imaging ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Long Term Follow Up ◽  
History Of ◽  
Clinically Significant

Object The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients’ mean age at presentation was 50 years (range 13–82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

scholarly journals Pathological response of cavernous malformations following radiosurgery

2015 ◽  
Vol 123 (4) ◽  
pp. 938-944 ◽  
Author(s):  
Samuel S. Shin ◽  
Geoffrey Murdoch ◽  
Ronald L. Hamilton ◽  
Amir H. Faraji ◽  
Hideyuki Kano ◽  
...  
Keyword(s):  
High Risk ◽  
Stereotactic Radiosurgery ◽  
Therapeutic Option ◽  
Latency Period ◽  
Pathological Response ◽  
Recurrent Bleeding ◽  
Histopathological Analysis ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Insight Into

OBJECT Stereotactic radiosurgery (SRS) is a therapeutic option for repeatedly hemorrhagic cavernous malformations (CMs) located in areas deemed to be high risk for resection. During the latency period of 2 or more years after SRS, recurrent hemorrhage remains a persistent risk until the obliterative process has finished. The pathological response to SRS has been studied in relatively few patients. The authors of the present study aimed to gain insight into the effect of SRS on CM and to propose possible mechanisms leading to recurrent hemorrhages following SRS. METHODS During a 13-year interval between 2001 and 2013, bleeding recurred in 9 patients with CMs that had been treated using Gamma Knife surgery at the authors' institution. Microsurgical removal was subsequently performed in 5 of these patients, who had recurrent hemorrhages between 4 months and 7 years after SRS. Specimens from 4 patients were available for analysis and used for this report. RESULTS Histopathological analysis demonstrated that vascular sclerosis develops as early as 4 months after SRS. In the samples from 2 to 7 years after SRS, sclerotic vessels were prominent, but there were also vessels with incomplete sclerosis as well as some foci of neovascularization. CONCLUSIONS Recurrent bleeding after SRS for CM could be related to incomplete sclerosis of the vessels, but neovascularization may also play a role.

Pain outcomes after surgery in patients with intramedullary spinal cord cavernous malformations

2010 ◽  
Vol 29 (3) ◽  
pp. E15 ◽  
Author(s):  
Harel Deutsch
Keyword(s):  
Spinal Cord ◽  
Neurological Status ◽  
Posterior Column ◽  
Cervical Lesions ◽  
Acute Hemorrhage ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Pain Scores ◽  
Primary Indication

Object The objective of the study was to quantify the improvement in pain levels for patients who have undergone surgery for intramedullary spinal cord cavernous malformations (SCCMs). Methods The author reviewed medical records of patients who underwent surgery for an intramedullary SCCM between 2003 and 2010. Numerical pain scores (range 0–10) were recorded preoperatively and at follow-up. The follow-up period exceeded 1 year. Neurological status and subjective outcomes were assessed. Each patient underwent follow-up MR imaging. Results Five patients were identified with SCCMs who underwent surgery: 4 with thoracic and 1 with cervical lesions. Patients had been conservatively managed for an average of 5 years prior to surgery, and none had a history of acute hemorrhage or neurological deterioration during the observation period. The primary indication for surgery in each patient was pain, although 4 of 5 patients had some evidence of myelopathy on examination. Pain improved from a mean preoperative score of 8.6 to mean score of 2.0 (p < 0.01) at 1 month. Pain scores then increased to 3.7 (p < 0.01) at 1 year. All patients had some improvement in pain. No new motor weakness was noted, but all patients had increased symptoms of posterior-column dysfunction and numbness after surgery. Conclusions Spinal cord intramedullary cavernous malformations are increasingly being diagnosed early with patients presenting with mostly pain symptoms. Removal of the lesion is reliably associated with improvement in pain scores but often the pain improvement is transient. While long-term worsening of pain scores occurs, at 1-year follow-up, patients reported pain scores were improved over preoperative scores. In all patients some degree of postoperative posterior-column dysfunction was present. Some of the immediate pain relief may be due to analgesia related to the myelotomy of newly described posterior column pain pathways. In patients with severe pain, surgery to remove SCCMs reduced the overall pain level at 1 year.

scholarly journals Resection of brainstem cavernous malformation via perifacial zone through retrosigmoid supracondylar approach

2019 ◽  
Vol 1 (1) ◽  
pp. V16 ◽  
Author(s):  
Ken Matsushima ◽  
Michihiro Kohno ◽  
Helmut Bertalanffy
Keyword(s):  
Cavernous Malformation ◽  
Senior Author ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Inferior Surface ◽  
Complete Surgical Resection ◽  
Brainstem Cavernoma ◽  
Dissection Techniques ◽  
Pontomedullary Junction

Hemorrhagic brainstem cavernous malformations carry a high risk of progressive neurological deficits owing to recurrent hemorrhages and hence require complete surgical resection while minimizing damage to the dense concentration of nuclei and fibers inside the brainstem. To access lesions inside the lower pons, the senior author (H.B.) has preferred to approach the lesions via the “perifacial zone” through the pontomedullary sulcus from the inferior surface of the pontine bulge for more than 20 years.1,2 This video demonstrates a case of a cavernous malformation inside the lower pons, which was surgically treated via the pontomedullary junction through the retrosigmoid supracondylar approach in a half-sitting position. The lesion was completely removed in piecemeal fashion through a tiny incision on the sulcus, which did not cause any new neurological deficits. The modified Rankin Scale improved from 4 before the surgery to 1, and the patient had no recurrence during the 2 years of follow-up. The advantage of this access and the dissection techniques for this challenging lesion are introduced, based on our experience with more than 230 surgeries of brainstem cavernoma.The video can be found here: https://youtu.be/0H_XqkQgQ9I.

Cavernomata and angiographically occult lesions

2019 ◽  
pp. 651-658
Author(s):  
Hiren Patel ◽  
Janneke van Beijnum
Keyword(s):  
Vascular Malformations ◽  
Genetic Disorder ◽  
Positive Family History ◽  
Intractable Epilepsy ◽  
Venous Drainage ◽  
Mass Effect ◽  
Developmental Venous Anomaly ◽  
Cavernous Malformations ◽  
Capillary Telangiectasia

Cerebral cavernous malformations (CCMs), known in addition as cavernomata or cavernous (haem)angiomata, are vascular malformations consisting of abnormally enlarged capillary cavities without intervening parenchyma (see section on capillary telangiectasia, this chapter) or arterial feeders with venous drainage (see arteriovenous malformations, Chapter 50). About 10–20% of CCMs are associated with a developmental venous anomaly (DVA), which is an extreme variation of the normal venous anatomy. CCMs can occur as result of an underlying genetic disorder, and genetic analysis is recommended in patients with a positive family history or multiple lesions. A conservative approach for asymptomatic CCMs seems justified in the large majority of cases and follow-up imaging is only needed if patients become symptomatic. Indications for surgical treatment are multiples haemorrhages, CCMs with mass effect causing symptoms, and patients with intractable epilepsy with an identified epileptogenic focus. Stereotactic radiosurgery can be considered in exceptional cases with a very high surgical risk.

scholarly journals SAT-170 Clinical Course of Adrenal Myelolipoma: A Long-Term Longitudinal Follow-Up Study

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Oksana Hamidi ◽  
Ram Narayan Raman ◽  
Natalia Lazik ◽  
Nicole Ariza-Iniguez ◽  
Travis J McKenzie ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Tumor Size ◽  
Mass Effect ◽  
Tumor Growth Rate ◽  
Adrenocortical Adenoma ◽  
Size Change ◽  
Large Tumor ◽  
Acute Hemorrhage ◽  
Follow Up Study

Abstract Myelolipoma is the second most common adrenal tumor. Yet, systematic approach to these tumors remains poorly defined. Thus, we aimed to describe natural history of myelolipoma and to identify predictors of tumor growth and need for surgery. We conducted a retrospective longitudinal follow-up study of consecutive patients with myelolipoma. A total of 321 myelolipomas (median size, 2.3 cm [range, 0.5-18.0]) were diagnosed in 305 patients at median age of 63 years (25-87). Most myelolipomas were discovered incidentally (86.6%), whereas others were discovered on imaging done for cancer staging (8.8%) or during workup of mass effect symptoms (4.6%). Median duration of follow-up was 54 months (range, 0.03-267). Compared with myelolipomas &lt;6 cm, tumors ≥6 cm were more likely to be right-sided (59% vs 41%, P=0.02), bilateral (21% vs 3%, P &lt;.0001), cause mass effects symptoms (32% vs 0%, P&lt;.0001), have radiographic hemorrhagic changes (14% vs 1%, P&lt;.0001), and undergo adrenalectomy (52% vs 5%, P&lt;.0001). There was no difference in sex or age at diagnosis between the groups. Hemorrhagic changes were noted in 9 (3.0%) patients with median tumor size of 7.0 cm (range, 1.8-18.0). Concomitant adrenal hormone excess was diagnosed in 12/126 (9.5%) patients. Primary aldosteronism was noted in 9 patients: due to concomitant ipsilateral (n=3) or contralateral adrenocortical adenoma (n=3), or bilateral idiopathic adrenal hyperplasia (n=3). Autonomous cortisol excess was noted in 3 patients: due to concomitant contralateral (n=2) or ipsilateral adrenocortical adenoma (n=1). Of 162 patients with ≥6 months of imaging follow-up, tumor size change ranged from -10 to 115 mm (median, 0 mm) and tumor growth rate ranged from -5.6 to 140 mm/year (median, 0 mm/year). Tumor growth ≥1.0 cm (n=26, 16.0%) was associated with larger initial tumor size (3.6 vs 2.3 cm, P=0.02) and hemorrhagic changes on imaging (12% vs 2%, P=0.007), compared with &lt;1 cm size change. Myelolipomas with ≥1.0 cm growth were more likely to undergo adrenalectomy (35% vs 8%, P&lt;.0001). Among 37 (12%) patients that underwent adrenalectomy for myelolipoma, surgical indications included: large tumor size/tumor growth (32%), diagnostic surgery (27%), mass effect symptoms (14%), concomitant ipsilateral tumor leading to hormonal excess (11%), acute hemorrhage (8%), and concomitant resection during non-adrenal surgery (8%). In conclusion, most myelolipomas are discovered incidentally, whereas myelolipomas ≥6 are more likely to cause mass effect symptoms, have radiographic hemorrhagic changes, and more commonly undergo resection. Hormonal excess is rare and is usually attributed to concomitant adrenocortical adenoma or hyperplasia. Tumor growth ≥1.0 cm is associated with larger myelolipoma and presence of hemorrhagic changes. Surgical resection should be considered in symptomatic patients with large tumors, evidence of hemorrhage, or tumor growth.

scholarly journals Remarkable Clinical Improvement Following Microsurgical Resection of Left Lingual Gyrus Cerebral Cavernous Malformation: A Case Report

2020 ◽  
Author(s):  
Samer S. Hoz ◽  
Zahraa F. Al-Sharshahi ◽  
Mustafa M. Altaweel ◽  
Saja A. Albanaa
Keyword(s):  
Occipital Lobe ◽  
Surgical Removal ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Lingual Gyrus ◽  
Microsurgical Resection ◽  
Clonic Seizures ◽  
Magnetic Resonance Imaging Mri

Abstract Introduction Cerebral cavernous malformations (CCMs) are collections of dilated and irregular capillaries in the brain. Cerebral cavernous malformations are predominantly supratentorial; occipital CCMs are rare. Surgical removal is indicated for CCMs with recurrent hemorrhage, refractory seizures, and expanding lesions. Case Description We describe a case of a 15-year-old male who presented with repeated tonic-clonic seizures and right homonymous hemianopia of 3-week duration. Magnetic resonance imaging (MRI) showed a mass located on the left medial occipital lobe, specifically in the left lingual gyrus. The T2-weighted and T2-gradient echo images confirmed the diagnosis of a CCM. Total microscopic resection was achieved. There were no surgical complications. The visual deficit improved, and the patient was seizure-free on subsequent follow-up visits. Conclusion Surgical resection of an occipital CCM resulted in a remarkable improvement in terms of seizures and visual field deficits.

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