CAVERNOUS MALFORMATIONS OF THE BASAL GANGLIA AND THALAMUS

Neurosurgery ◽  
2009 ◽  
Vol 65 (1) ◽  
pp. 7-19 ◽  
Author(s):  
Bradley A. Gross ◽  
H. Hunt Batjer ◽  
Issam A. Awad ◽  
Bernard R. Bendok
Keyword(s):  
Basal Ganglia ◽  
Natural History ◽  
French Literature ◽  
Outcome Data ◽  
Therapeutic Modality ◽  
Surgical Morbidity ◽  
Term Outcome ◽  
Cavernous Malformations ◽  
Long Term Outcome

ABSTRACT CAVERNOUS MALFORMATIONS OF the basal ganglia and thalamus present a unique therapeutic challenge to the neurosurgeon given their unclear natural history, the risk of surgical treatment, and the unproven efficacy of radiosurgical therapy. Via a PubMed search of the English and French literature, we have systematically reviewed the natural history and surgical and radiosurgical management of these lesions reported through April 2008. Including rates cited for “deep” cavernous malformations, annual bleeding rates for these lesions varied from 2.8% to 4.1% in the natural history studies. Across surgical series providing postoperative or long-term outcome data on 103 patients, we found an 89% resection rate, a 10% risk of long-term surgical morbidity, and a 1.9% risk of surgical mortality. The decrease in hemorrhage risk reported 2 years after radiosurgery might be a result of natural hemorrhage clustering, underscoring the unproven efficacy of this therapeutic modality. Given the compounded risks of radiation-induced injury and post-radiosurgical rebleeding, radiosurgery at modest dosimetry (12–14 Gy marginal doses) is only an option for patients with surgically inaccessible, aggressive lesions.

scholarly journals Cavernous Malformation of Brainstem, Thalamus, and Basal Ganglia

Neurosurgery ◽  
2012 ◽  
Vol 72 (4) ◽  
pp. 573-589 ◽  
Author(s):  
Paritosh Pandey ◽  
Erick M. Westbroek ◽  
Peter A. Gooderham ◽  
Gary K. Steinberg
Keyword(s):  
Basal Ganglia ◽  
Neurological Deficits ◽  
Term Outcome ◽  
Cavernous Malformations ◽  
Long Term Outcome ◽  
Brain Malformations ◽  
Hemorrhage Risk ◽  
Clinical Records

Abstract BACKGROUND: Cavernous malformations (CMs) in deep locations account for 9% to 35% of brain malformations and are surgically challenging. OBJECTIVE: To study the clinical features and outcomes following surgery for deep CMs and the complication of hypertrophic olivary degeneration (HOD). METHODS: Clinical records, radiological findings, operative details, and complications of 176 patients with deep CMs were reviewed retrospectively. RESULTS: Of 176 patients with 179 CMs, 136 CMs were in the brainstem, 27 in the basal ganglia, and 16 in the thalamus. Cranial nerve deficits (51.1%), hemiparesis (40.9%), numbness (34.7%), and cerebellar symptoms (38.6%) presented most commonly. Hemorrhage presented in 172 patients (70 single, 102 multiple). The annual retrospective hemorrhage rate was 5.1% (assuming CMs are congenital with uniform hemorrhage risk throughout life); the rebleed rate was 31.5%/patient per year. Surgical approach depended on the proximity of the CM to the pial or ependymal surface. Postoperatively, 121 patients (68.8%) had no new neurological deficits. Follow-up occurred in 170 patients. Delayed postoperative HOD developed in 9/134 (6.7%) patients with brainstem CMs. HOD occurred predominantly following surgery for pontine CMs (9/10 patients). Three patients with HOD had palatal myoclonus, nystagmus, and oscillopsia, whereas 1 patient each had limb tremor and hemiballismus. At follow-up, 105 patients (61.8%) improved, 44 (25.9%) were unchanged, and 19 (11.2%) worsened neurologically. Good preoperative modified Rankin Score (98.2% vs 54.5%, P = .001) and single hemorrhage (89% vs 77.3%, P < .05) were predictive of good long-term outcome. CONCLUSION: Symptomatic deep CMs can be resected with acceptable morbidity and outcomes. Good preoperative modified Rankin Score and single hemorrhage are predictors of good long-term outcome.

Hemorrhage risk, surgical management, and functional outcome of brainstem cavernous malformations

2013 ◽  
Vol 119 (4) ◽  
pp. 996-1008 ◽  
Author(s):  
Da Li ◽  
Yang Yang ◽  
Shu-Yu Hao ◽  
Liang Wang ◽  
Jie Tang ◽  
...  
Keyword(s):  
Neurological Function ◽  
Incomplete Resection ◽  
Developmental Venous Anomaly ◽  
Surgical Morbidity ◽  
Term Outcome ◽  
Cavernous Malformations ◽  
Long Term Outcome ◽  
Female Ratio

Object The aim of this study was to evaluate the pre- and postoperative rehemorrhage risk, neurological function outcome, and prognostic factors of surgically treated brainstem cavernous malformations (CMs) with long-term follow-up. Methods The authors conducted a retrospective review of the clinical data from 242 patients with brainstem CMs that were surgically treated between 1999 and 2010. Patient charts, imaging findings, and outcomes were examined. Results The study included 242 patients, with a male-to-female ratio of 1.3 and mean age of 32.6 years. The mean modified Rankin Scale scores on admission, at discharge, at 3 and 6 months after surgery, and at recent evaluation were 2.2, 2.6, 2.3, 1.8, and 1.5, respectively. The preoperative calculated annual hemorrhage and rehemorrhage rates were 5.0% and 60.9%, respectively. The complete resection rate was 95%. Surgical morbidity occurred in 112 patients (46.3%). Eighty-five patients (35.1%) demonstrated worsened condition immediately after surgery; 34 (41.0%) and 51 (61.4%) of these patients recovered to their baseline level within 3 and 6 months after surgery, respectively. At a mean follow-up of 89.4 months, the patients' condition had improved in 147 cases (60.7%), was unchanged in 70 cases (28.9%), and had worsened in 25 cases (10.3%). A total of 8 hemorrhages occurred in 6 patients, and the postoperative annual hemorrhage rate was 0.4%. Permanent morbidity remained in 65 patients (26.9%). The adverse factors for preoperative rehemorrhage were age ≥ 50 years, size ≥ 2 cm, and perilesional edema. The risk factors for postoperative hemorrhage were developmental venous anomaly and incomplete resection. The independent adverse factors for long-term outcome were increased age, multiple hemorrhages, ventral-seated lesions, and poor preoperative status. Favorable, complete improvement in the postoperative deficits over time was correlated with good preoperative neurological function and continuing improvement thereafter. Conclusions Favorable long-term outcomes and significantly low postoperative annual hemorrhage rates were achieved via surgery. Total resection should be attempted with an aim of minimal injury to neurological function; however, postoperative deficits can improve during the postoperative course. Close follow-up with radiological examination is proposed for patients with adverse factors predictive of rehemorrhage.

scholarly journals Long-term outcome and quality of life after CNS cavernoma resection: eloquent vs. non-eloquent areas

2021 ◽  
Author(s):  
Loay Shoubash ◽  
Jörg Baldauf ◽  
Marc Matthes ◽  
Michael Kirsch ◽  
Matthias Rath ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Short Form ◽  
Preoperative Evaluation ◽  
Patient Counseling ◽  
Surgical Morbidity ◽  
Term Outcome ◽  
Long Term Outcome

AbstractThe aim of this study is to analyze the long-term quality of life after surgery of cavernoma. A monocentric retrospective study was conducted on 69 patients with cavernoma treated microsurgically between 2000 and 2016. The eloquence was adopted from Spetzler-Martin definition. A most recent follow-up was elicited between 2017 and 2019, in which the quality of life (QoL) was evaluated with the Short Form-12 questionnaire (SF12). Forty-one lesions were in eloquent group (EG), 22 in non-eloquent group (NEG), 3 in orbit, and 3 in the spinal cord. Postoperative worsening of the modified Rankin scale (mRS) occurred in 19.5% of cases in EG versus 4.5% in NEG. After a mean follow-up of 6.5 years (SD 4.6), the neurological status was better or unchanged compared to baseline in 85.4% of EG and 100% of NEG. Regarding QoL assessment of 44 patients (EG n = 27, NEG n = 14) attended the last follow-up. Patients after eloquent cavernoma resection reported a non-inferior QoL in most SF12 domains (except for physical role) compared to NEG. However, they reported general health perception inferior to norms, which was affected by the limited physical and emotional roles. At a late follow-up, the surgical morbidity was transient in the NEG and mostly recovered in the EG. The QoL comparison between eloquent and non-eloquent cavernomas created interesting and new data after prolonged follow-up. These results add value for decision-making as well as patient counseling for future encountered cases. Preoperative evaluation of QoL is recommended for future studies to assess QoL dynamics.

scholarly journals Non-Surgical and Non-Radioiodine Techniques for Ablation of Benign Thyroid Nodules: Consensus Statement and Recommendation

2020 ◽  
Vol 128 (10) ◽  
pp. 687-692 ◽  
Author(s):  
Joachim Feldkamp ◽  
F. Grünwald ◽  
Markus Luster ◽  
Kerstin Lorenz ◽  
Christian Vorländer ◽  
...  
Keyword(s):  
Thyroid Nodules ◽  
Consensus Statement ◽  
German Society ◽  
Outcome Data ◽  
High Frequency Ultrasound ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ultrasound Ablation ◽  
General And Visceral Surgery

AbstractThyroid nodules and cysts are frequently diagnosed in Germany with a prevalence of about 20% in young adults reaching up to 70% in older adults. Surgery is the standard treatment of symptomatic nodules, nodules with suspicion of malignancy and thyroid cancer. Radioiodine treatment is applied for autonomously functioning nodules. During the last years new non-surgical and non-radioiodine techniques have been introduced to treat thyroid nodules. These techniques include ethanol/polidocanol treatment, radiofrequency, microwave, and laser ablation, and high frequency ultrasound ablation. A significant reduction in nodule size could be documented for these techniques in several studies, but long-term outcome data are missing. Until now, there is no general consensus regarding the appropriate indications for these methods. For this reason, the Thyroid Section (German Society for Endocrinology), the Thyroid Working Committee (German Society for Nuclear Medicine), and the German Association of Endocrine Surgeons (CAEK) for the German Society of General and Visceral Surgery (DGAV) reviewed the respective literature, discussed the pro and cons and developed a consensus statement and recommendation to help physicians and patients in their decision making.

Long-term outcome of children undergoing surgery for suspected perilymph fistula

2008 ◽  
Vol 123 (3) ◽  
pp. 298-302 ◽  
Author(s):  
R J Sim ◽  
A H Jardine ◽  
E J Beckenham
Keyword(s):  
Hearing Loss ◽  
Case Series ◽  
Outcome Data ◽  
Rank Test ◽  
Short Term ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Signed Rank ◽  
Signed Rank Test

AbstractA number of authors have suggested that surgery for suspected perilymph fistula is effective in preventing deterioration of hearing and in improving hearing in some cases in the short term. We present long-term hearing outcome data from 35 children who underwent exploration for presumed perilymph fistula at The Children's Hospital, Sydney, Australia, between 1985 and 1992.Methods:The pre-operative audiological data (mean of 500, 1000, 2000 and 4000 Hz results) were compared with the most recently available data (range two to 15 years) and the six-month post-operative data.Results:The short-term results showed no significant change in hearing at six months, with a subsequent, statistically significant progression of hearing loss in both operated and non-operated ears (Wilcoxon signed rank test: operated ear, p < 0.017; non-operated ear, p < 0.009).Conclusion:In this case series, exploratory surgery for correction of suspected perilymph fistula did not prevent progression of long-term hearing loss.

Menstrual, Sexual, and Obstetrical Outcomes after Vaginal Replacement for Vaginal Atresia Associated with Anorectal Malformation

2016 ◽  
Vol 27 (06) ◽  
pp. 495-502 ◽  
Author(s):  
Clare Skerritt ◽  
Alejandra Vilanova Sánchez ◽  
Victoria Lane ◽  
Richard Wood ◽  
Geri Hewitt ◽  
...  
Keyword(s):  
Anorectal Malformation ◽  
Outcome Data ◽  
Current Evidence ◽  
Vaginal Agenesis ◽  
Vaginal Reconstruction ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Vaginal Atresia ◽  
Rectovestibular Fistula

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.

The natural history of glycogen storage disease types VI and IX: Long-term outcome from the largest metabolic center in Canada

2014 ◽  
Vol 113 (3) ◽  
pp. 171-176 ◽  
Author(s):  
Anne Roscher ◽  
Jaina Patel ◽  
Stacy Hewson ◽  
Laura Nagy ◽  
Annette Feigenbaum ◽  
...  
Keyword(s):  
Natural History ◽  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Term Outcome ◽  
Long Term Outcome ◽  
History Of

scholarly journals Chronic Myeloid Leukemia: Long-Term Outcome Data in the Imatinib Era

2018 ◽  
Vol 35 (1) ◽  
pp. 37-42 ◽  
Author(s):  
Prasanth Ganesan ◽  
Trivadi S. Ganesan ◽  
Venkatraman Radhakrishnan ◽  
Tenali Gnana Sagar ◽  
Krishnarathinam Kannan ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Outcome Data ◽  
Term Outcome ◽  
Long Term Outcome

Abstract 20041: Single Ventricle Outcome Following Neonatal Palliation of Severe Ebstein’s Anomaly With Modified Starnes Procedure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
S. Ram Kumar ◽  
Nathan Noh ◽  
Novell Castillo ◽  
Brian Fagan ◽  
Grace Kung ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Nyha Class ◽  
Pacemaker Implantation ◽  
Outcome Data ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

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