Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations

2019 ◽  
Vol 90 (6) ◽  
pp. 695-703 ◽  
Author(s):  
Anshit Goyal ◽  
Lorenzo Rinaldo ◽  
Redab Alkhataybeh ◽  
Panagiotis Kerezoudis ◽  
Mohammed Ali Alvi ◽  
...  
Keyword(s):  
Natural History ◽  
Surgical Resection ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Univariate Analysis ◽  
Case Series ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Increased Risk

ObjectiveThere is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management.MethodsWe retrospectively reviewed the clinical course of patients diagnosed with ISCMs at our institution between 1995 and 2016. Haemorrhage was defined as clinical worsening in tandem with imaging changes visualised on follow-up MRI. Outcomes assessed included neurological status and annual haemorrhage rates.ResultsA total of 107 patients met inclusion criteria. Follow-up data were available for 85 patients. While 21 (24.7%) patients underwent immediate surgical resection, 64 (75.3%) were initially managed conservatively. Among this latter group, 16 (25.0%) suffered a haemorrhage during follow-up and 11 (17.2%) required surgical resection due to interval bleeding or neurological worsening. The overall annual risk of haemorrhage was 5.5% per person year. The rate among patients who were symptomatic and asymptomatic on presentation was 9.5% and 0.8%, respectively. Median time to haemorrhage was 2.3 years (0.1–12.3). Univariate analysis identified higher ISCM size (p=0.024), history of prior haemorrhage (p=0.013) and presence of symptoms (p=0.003) as risk factors for subsequent haemorrhage. Multivariable proportional hazards analysis revealed presence of symptoms to be independently associated with haemorrhage during follow-up (HR 9.39, CI 1.86 to 170.8, p=0.013).ConclusionLarge, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.

scholarly journals Atypical Adrenaliemmas

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A117-A118
Author(s):  
Ahmad Al Maradni ◽  
Karyne Lima Vinales ◽  
Ricardo Rafael Correa
Keyword(s):  
Surgical Resection ◽  
Histological Examination ◽  
Adrenal Mass ◽  
Case Series ◽  
Adrenal Tumors ◽  
Histopathological Diagnosis ◽  
Nerve Sheath Tumor ◽  
Adrenal Incidentalomas ◽  
Increased Risk

Abstract Introduction: Adrenal Incidentalomas (AI) are increasingly found with recent proliferation in imaging techniques. The precise prevalence of AI is not well established but estimated to be about 5%. Such findings require thorough work and follow up plans to assess for hormonal functionality and differentiate between benign or a malignant entity. Adrenocortical carcinoma (AC), schwannoma (SCH), ganglioneuroma (GN), myelolipoma (ML), neuroblastoma (NB), and ganglioneuroblastoma (GNB) are a rare presentation of AIs. In this case series, we present 2 cases of uncommon AIs. Cases Series: Case 1: A 55-year-old man presented to the hospital with episodic dizziness, nausea, vomiting, and diaphoresis. His physical examination and blood workup were unremarkable. He takes multiple antihypertensive medications at home. CT abdomen revealed an incidental 5 cm mass adjacent to the left adrenal gland which was confirmed to be of adrenal origin on MRI. The hormonal workup was negative. and patient. Laparoscopic left adrenalectomy was performed. Histopathological diagnosis pointed to a benign nerve sheath tumor. Tumor cells stained positive for S-100 and negative for CD34 and epithelial membrane antigen. The morphologic features and immunohistochemical profile consistent with adrenal SCH diagnoses, confirmed by two histopathologists. Postoperatively, Blood pressure improved, and the patient has no evidence of recurrence on subsequent follow-ups. Case 2: A 38-year-old female presented to the hospital with severe right groin pain. During the workup, a CT abdomen revealed a homogeneous, lobulated, minimally enhanced, 5.8 cm right adrenal mass with no calcifications. The hormonal workup was negative. FDG-PET scan showed low uptake in the right adrenal mass. Adrenalectomy was performed and histological examination revealed Schwan cells and neurites with occasional ganglion cells compatible with benign GN. Discussion: SCHs originate from Schwann cells in peripheral nerve sheaths. Adrenal SCHs are rare, they represent about 0.5% of all SCHs. Surgical resection is the initial management as imagings are non-diagnostic and appearance does not distinguish benign from malignant lesions. Adrenal GNs originate from neural crest cells in the adrenal medulla and represents between 20–30% of all GNs. They may be primary or evolve from differentiating NB. The majority of them are sporadic. GNs are typically benign and asymptomatic. However, larger GNs are associated with an increased risk of malignancy, a 33% risk for 5 cm compared to 92% for 6 cm. Surgical resection is necessary, especially for larger masses. Conclusion: Besides the common adrenal incidentalomas and their proper work and follow up plans, a clinician should consider atypical adrenal tumors with unusual presentation. Surgical treatment and histological examination are essential for an accurate diagnosis.

The natural history of cerebral cavernous malformations

1995 ◽  
Vol 83 (5) ◽  
pp. 820-824 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
John R. W. Kestle
Keyword(s):  
Natural History ◽  
Management Strategies ◽  
Operative Management ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Content Type ◽  
Increased Risk ◽  
History Of ◽  
Fine Print

✓ To determine the natural history of brain cavernous malformations, the authors entered patients referred to their center into a prospective registry between 1987 and 1993. All patients underwent magnetic resonance imaging, which showed the typical appearance of this lesion, and conservative management was recommended in all. Patients or their referring physicians were contacted for follow-up data. The purpose of the study was to define the rate of symptomatic hemorrhage and to determine the outcome in those patients who had suffered seizures. Follow-up data were available for 122 patients with a mean age at entry of 37 years (range 4–82 years). The malformation was located in the brainstem in 43 cases (35%), the basal ganglia/thalamus in 20 (17%), and a hemispheric area in 59 (48%). Fifty percent of patients had never had a symptomatic hemorrhage, 41% had one bleed, 7% had two, and 2% had three. Seizures were reported in 23% of patients and headaches in 15%. Lesions were solitary in 80% of patients and multiple in 20%. The retrospective annual hemorrhage rate (61 bleeds/4550.6 patient-years of life) was 1.3%. The mean prospective follow-up period was 34 months. There were nine bleeds during this time, six with new neurological deficits. In patients without a prior bleed, the prospective annual rate of hemorrhage was 0.6%. In contrast, patients with prior hemorrhage had an annual bleed rate of 4.5% (p = 0.028). Patient sex (p = 0.97) or the presence of seizures (p = 0.11), headaches (p = 0.06), or solitary versus multiple lesions (p = 0.15) were not significant predictors of later hemorrhage. There was no difference in the rate of bleeds between brain locations. Four patients with seizures became seizurefree and four patients without seizures later developed seizures; only one patient developed intractable seizures. Fourteen patients (11%) underwent surgery (two after hemorrhage, five with seizures, and seven with progressive deficits), and five had radiosurgery. No patient died in the follow-up period. This study indicates that conservative versus operative management strategies may need to be redefined, especially in patients who present with hemorrhage and who appear to have a significantly increased risk of subsequent rehemorrhage.

The Impact of Incorporating Targeted Radioimmunotherapy (RIT) into Transplant Preparative Regimens on the Incidence of Therapy Related Myelodysplasia (t-MDS) or AML (t-AML) Following Autologous Stem Cell Transplant (ASCT) for Lymphoma.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1082-1082 ◽  
Author(s):  
Amrita Y. Krishnan ◽  
Joycelynne M. Palmer ◽  
Smita C. Bhatia ◽  
Auayporn Nademanee ◽  
Stephen J. Forman ◽  
...  
Keyword(s):  
Risk Factors ◽  
Chromosomal Abnormalities ◽  
Univariate Analysis ◽  
Case Series ◽  
High Dose ◽  
Cell Transplant ◽  
Bone Marrow Biopsies ◽  
Increased Risk ◽  
The Impact

Abstract The reported incidence of t-MDS/t-AML following traditional ASCT for lymphoma ranges between 0–12%. Previously identified risk factors include older age, prior alkylator therapy and use of radiation either prior to ASCT or as part of the preparative regimen. It is unclear whether novel conditioning regimens for ASCT that utilize targeted RIT with the potential to deliver higher radiation doses to the marrow are associated with a higher risk of t-MDS/t-AML. We identified a case-series of 83 pts who underwent RIT based ASCT between 06/00 and 01/06 to evaluate the incidence of t-MDS/t-AML; Forty-one pts received standard dose 90Y ibritumomab tiuxetan (0.4mci/kg: median dose 32.9 mci (range 20–40)) in combination with high dose BEAM (BCNU 450mg/m2, etoposide 800mg/m2, cytarabine 800mg/m2, melphalan 140mg/m2) and 42 pts received high dose 90Y based on dosimetry (median 70.8 mci range 36–105) in combination with etoposide 60mg/kg plus cyclophosphamide 100mg/kg. Pts were followed prospectively post ASCT with serial bone marrow biopsies approximately annually. The median age at ASCT was 54 years (range 19–78). Disease histology included diffuse large cell n=40, follicular NHL n=17, mantle cell n=21, transformed n=4, SLL n=1. Disease status at ASCT was 1st CR n=17, 1stPR n=14, induction failure n=14, 1st relapse or greater n=38. With a median follow-up of 39 months (range, 1.4–83), three patients (3.61%) have developed t-MDS/t-AML. The three pts also had associated complex chromosomal abnormalities including de1(13q), del(5q), del (20q). The median time to t-MDS/t-AML was 2.63 years (range, 1.51 – 8.41) post NHL diagnosis and 1.99 years (range, 0.56 – 5.10) post ASCT. The cumulative incidences of t-MDS/t-AML at 1 and 2 years were 1.20% (95%CI, 0.17– 8.1%) and 2.60% (95%CI 0.64–9.9%). None of the potential risk factors including age(>50 at ASCT) (p=0.33), prior radiotherapy (p=0.99), number of prior regimens (p=0.5) and 90Y dose (p=0.99) were statistically significant by univariate analysis. As 82/83 pts had received prior alkylator therapy this was not analyzed as a separate risk factor. Two year overall survival for the entire cohort is 90% (95%CI 83–95). Although the follow up is relatively short, the incidence of t-MDS/t-AML is consistent with our previous institutional experience in ASCT patients who received non-RIT based conditioning (Krishnan et al. Blood 2000) and with the 2.5% incidence of t-MDS/t-AML observed in pts receiving 90Y in registration and compassionate use trials (Czuczman et al JCO 2007 in press). In conclusion RIT based conditioning does not appear to confer an increased risk of t-MDS/t-AML above what has been previously reported with traditional ASCT preparative regimens. Incidence of t-MDS/t-AML Incidence of t-MDS/t-AML

Intramedullary spinal cord cavernous malformations

2010 ◽  
Vol 29 (3) ◽  
pp. E14 ◽  
Author(s):  
Bradley A. Gross ◽  
Rose Du ◽  
A. John Popp ◽  
Arthur L. Day
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Vascular Malformations ◽  
Case Reports ◽  
Case Series ◽  
Motor Deficits ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Management Algorithm ◽  
Surgical Data

Although originally the subject of rare case reports, intramedullary spinal cord cavernous malformations (CMs) have recently surfaced in an increasing number of case series and natural history reports in the literature. The authors reviewed 27 publications with 352 patients to consolidate modern epidemiological, natural history, and clinical and surgical data to facilitate decision making when managing these challenging vascular malformations. The mean age at presentation was 42 years without a sex predilection. Thirty-eight percent of the cases were cervical, 57% thoracic, 4% lumbar, and 1% unspecified location. Nine percent of the patients had a family history of CNS CMs. Twenty-seven percent of the patients had an associated cranial CM. On presentation 63% of the patients had motor deficits, 65% had sensory deficits, 27% had pain, and 11% had bowel or bladder dysfunction. Presentation was acute in 30%, recurrent in 16%, and progressive in 54% of cases. An overall annual hemorrhage rate was calculated as 2.5% for 92 patients followed up for a total of 2571 patient-years. Across 24 reviewed surgical series, a 91% complete resection rate was found. Transient morbidity was seen in 36% of cases. Sixty-one percent of patients improved, 27% were unchanged, and 12% were worse at the long-term follow-up. Using this information, the authors review surgical nuances in treating these lesions and propose a management algorithm.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Natural history and treatment options of radiation-induced brain cavernomas: a systematic review

2021 ◽  
Author(s):  
Gildas Patet ◽  
Andrea Bartoli ◽  
Torstein R. Meling
Keyword(s):  
Systematic Review ◽  
Natural History ◽  
Radiation Treatment ◽  
Neurological Deficits ◽  
Cochrane Library ◽  
Average Dose ◽  
Cavernous Malformations ◽  
Radiation Induced ◽  
The Mean

AbstractRadiation-induced cavernous malformations (RICMs) are delayed complications of brain irradiation during childhood. Its natural history is largely unknown and its incidence may be underestimated as RCIMS tend to develop several years following radiation. No clear consensus exists regarding the long-term follow-up or treatment. A systematic review of Embase, Cochrane Library, PubMed, Google Scholar, and Web of Science databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was performed. Based on our inclusion/exclusion criteria, 12 articles were included, totaling 113 children with RICMs, 86 were treated conservatively, and 27 with microsurgery. We were unable to precisely define the incidence and natural history from this data. The mean age at radiation treatment was 7.3 years, with a slight male predominance (54%) and an average dose of 50.0 Gy. The mean time to detection of RICM was 9.2 years after radiation. RICM often developed at distance from the primary lesion, more specifically frontal (35%) and temporal lobe (34%). On average, 2.6 RICMs were discovered per child. Sixty-seven percent were asymptomatic. Twenty-one percent presented signs of hemorrhage. Clinical outcome was favorable in all children except in 2. Follow-up data were lacking in most of the studies. RICM is most often asymptomatic but probably an underestimated complication of cerebral irradiation in the pediatric population. Based on the radiological development of RICMs, many authors suggest a follow-up of at least 15 years. Studies suggest observation for asymptomatic lesions, while surgery is reserved for symptomatic growth, hemorrhage, or focal neurological deficits.

scholarly journals Treatments and outcomes of untreated cerebral cavernous malformations in China: study protocol of a nationwide multicentre prospective cohort study

BMJ Open ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. e037957
Author(s):  
Fuxin Lin ◽  
Qiu He ◽  
Zhuyu Gao ◽  
Lianghong Yu ◽  
Dengliang Wang ◽  
...  
Keyword(s):  
Informed Consent ◽  
Natural History ◽  
Study Protocol ◽  
Treated Group ◽  
Secondary Outcome ◽  
Long Term Outcomes ◽  
Cavernous Malformations ◽  
Electronic Data Capture System

IntroductionThe treatment decision and long-term outcomes of previously untreated cerebral cavernous malformation (U-CCM) are still controversial. Therefore, we are conducting a nationwide multicentre prospective registry study in China to determine the natural history and effect of surgical treatment on long-term outcomes in Chinese people with U-CCM.Methods and analysisThis study was started on 1 January 2018 and is currently ongoing. It is a cohort follow-up study across a 5-year period. Patients will be followed up for at least 3 years after inception. Patients with U-CCM will be enrolled from 24 Grade III, level A hospitals distributed all over China. The cohort size is estimated to be 1200 patients. Patients are registered in surgically treated group and conservatively treated group. Clinical characteristics, radiology information and laboratory data are prospectively collected using an electronic case report form through an electronic data capture system. The primary outcome of this study is poor clinical outcome at the last follow-up (modified Rankin Scale score >2 lasting at least 1 year). The secondary outcome includes symptomatic haemorrhage, drug refractory epilepsy, focal neurological deficits, morbidity and all-cause mortality during follow-up. Univariate and multivariate regression analysis will be performed to determine the risk factors for poor outcomes in all patients, and to estimate the effect of surgery. Life tables, Kaplan-Meier estimates, log-rank test and proportional hazards Cox regression will be used to analyse the follow-up data of conservatively treated patients to determine the natural history of U-CCM. Initial presentation and location of U-CCM are prespecified subgroup factors.Ethics and disseminationThe study protocol and informed consent form have been reviewed and approved by the Research Ethical Committee of First Affiliated Hospital of Fujian Medical University (FAHFMU-2018-003).Written informed consent will be obtained from each adult participant or from the guardian of each paediatric participant. The final results will be published in peer-reviewed journals.Trial registration numberNCT03467295.

scholarly journals Eculizumab discontinuation in atypical hemolytic uremic syndrome: TMA recurrence risk and renal outcomes

2021 ◽  
Author(s):  
Gema Ariceta ◽  
Fadi Fakhouri ◽  
Lisa Sartz ◽  
Benjamin Miller ◽  
Vasilis Nikolaou ◽  
...  
Keyword(s):  
Family History ◽  
Hemolytic Uremic Syndrome ◽  
Univariate Analysis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Response ◽  
Pathogenic Variants ◽  
Increased Risk ◽  
History Of ◽  
Uremic Syndrome

ABSTRACT Background Eculizumab modifies the course of disease in patients with atypical hemolytic uremic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited. Methods Patients enrolled in the Global aHUS Registry who received ≥1 month of eculizumab before discontinuing, demonstrated hematologic or renal response prior to discontinuation and had ≥6 months of follow-up were analyzed. The primary endpoint was the proportion of patients suffering thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: eGFR changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function. Results We analyzed 151 patients with clinically diagnosed aHUS who had evidence of hematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants, or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; 7 (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation. Conclusions Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.

Malignant thyroid teratoma: an integrated analysis of case series and case reports

2021 ◽  
Author(s):  
Huy Gia Vuong ◽  
Truong P.x. Nguyen ◽  
Hanh T.t. Ngo ◽  
Lewis Hassell ◽  
Kennichi Kakudo
Keyword(s):  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Seer Program ◽  
Integrated Analysis ◽  
Continuous Variables ◽  
Chi Square ◽  
Large Tumor ◽  
Exact Test

Malignant thyroid teratoma (MTT) is a very rare thyroid malignancy. These neoplasms have been reported only in case reports and small-sized case series so far. In this study, we searched for MTTs in the Surveillance, Epidemiology, and End Result (SEER) program during 1975-2016. Subsequently, we incorporated the SEER data with published MTT cases in the literature to analyze the characteristics and prognostic factors of MTTs. Integrated data were analyzed using Chi-square or Fisher’s exact test for categorical covariates, and t-test or Mann-Whitney test for continuous variables. We included 28 studies with 36 MTT cases and found additional 8 cases from the SEER program for final analyses. Our results showed that MTT is typically seen in adult females. These neoplasms were associated with an aggressive clinical course with high rates of extrathyroidal extension (80%) and nodal involvement (62%). During follow-up, the development of recurrence and metastases were common (42% and 46%, respectively), and one-third of patients died at the last follow-up. Large tumor size (p = 0.022) and the presence of metastases during follow-up (p = 0.008) were associated with a higher mortality rate. In conclusion, our study demonstrated the characteristic features of MTT patients and outlined some parameters associated with a negative outcome which could help clinicians better predict the clinical course of these neoplasms.

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.

Sign in / Sign up

Export Citation Format

Share Document