Intramuscular cavernous venous malformation of extraocular muscles. Fractionated stereotactic radiotherapy as a therapeutic alternative. / Malformación venosa cavernosa en musculatura extrínseca ocular. Radioterapia estereotáctica fraccionada como alternativa terapéutica.

Intramuscular cavernous venous malformations affecting extraocular muscles are extremely uncommon. Due to their location, complete resection could be difficult. A clinical case is presented of an inferior rectus muscle orbital cavernous malformation treated with fractionated stereotactic radiotherapy after post-surgical excision recurrence. The malformation responded to radiotherapy with a reduction in size and symptoms. Fractionated stereotactic radiotherapy is an alternative and effective treatment for cavernous venous malformations that are surgically challenging due to their radiotherapy sensitivity.

Resection of cavernous angioma located in eloquent areas using functional cortical and subcortical mapping under awake conditions. Outcomes in a 50-case multicentre series.

INTRODUCTION: Surgical resection of supratentorial cavernous angiomas located in eloquent areas poses a significant risk to the patient of postoperative neurological impairment and justifies intraoperative functional monitoring. METHODS: Multicentre retrospective series of adult patients with cavernous angiomas located within eloquent areas and treated with functional-based surgical resection according to functional boundaries under intraoperative functional cortico-subcortical monitoring under awake conditions. RESULTS: Fifty patients (18 males, mean 36.3±10.8 year-old) underwent surgical resection with intraoperative cortico-subcortical functional mapping using direct electrostimulation under awake conditions for a cavernous angioma located in eloquent areas with a mean postoperative follow-up of 21.0±21.2 months. At presentation, the cavernous angioma had previously resulted in severe impairment (neurological deficit in 34%, seizures in 70%, uncontrolled seizures in 34%, reduced Karnofsky Performance Status score of 70 or less in 24%, inability to work in 52%). Functional-based surgical resection allowed complete removal of the cavernous angioma in 98% and of the haemosiderin rim in 82%. Postoperative seizures and other complications were rare, and similarly so across all centres included in this series. Postoperatively, we found functional improvement in 84% of patients (reduced Karnofsky Performance Status score of 70 or less in 6%, uncontrolled seizures in 16%, and inability to work in 11%). CONCLUSION: Functional-based surgical resection aids the safe and complete resection of cavernous angiomas located in eloquent areas while minimizing the surgical risks. Functional mapping has to be considered in such challenging cases.

Contralateral interhemispheric approach to deep-seated cavernous malformations: surgical considerations and clinical outcomes in 31 consecutive cases.

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.

Gamma knife stereotactic radiosurgery for thalamic & brainstem cavernous angiomas.

OBJECTIVE: To determine the safety and efficacy of Gamma Knife stereotactic radiosurgery (GKS) in the treatment of patients with symptomatic cavernous angiomas (CA) of the brainstem or thalamus, by comparing overall outcome to the natural history of the disease. METHODS: Over 10 years a series of 16 consecutively presenting patients (M = 9, F = 7) with thalamic or brainstem CA were treated with GKS to a single lesion, specifically excluding the haemosiderin ring from the target. Within the year prior to treatment eight patients had suffered one symptomatic haemorrhage and eight had suffered more than one symptomatic haemorrhage. Mean age at treatment was 38.9 (15-55) years. Mean prescription dose 13.31 Gray (11.0 Gy-16.0 Gy). Patients were followed up radiologically and, more importantly, clinically for a mean period of 43.8 (11-101) months, median 36 months. RESULTS: One patient suffered recurrent haemorrhage at 23 months post-GKS, but has not re-bled in the following 61 months. One patient died of thalamic haemorrhage from the treated lesion at 90 months. One patient was lost to follow up. There have been no other clinical episodes or radiological findings to suggest post-GKS haemorrhage in the remaining 13 patients, and no other complications were observed in the treated population. The annual haemorrhage rate within the first two years post GKS was 3.72% and the annual haemorrhage rate 2 years post GKS was 3.59% per annum. CONCLUSION: With the dose regimens described, GKS is safe and effective in the treatment of thalamic and brainstem CA, as assessed by significant reduction in observed rate of re-haemorrhage over that expected from the known natural history of those CAs which have already demonstrated a tendency to haemorrhage in highly eloquent areas.

Supracerebellar-supratrochlear and infratentorial-infratrochlear approaches: gravity-dependent variations of the lateral approach over the cerebellum.

OBJECTIVE: Lateral supracerebellar-infratentorial approaches are established for lesions in ambient cistern and posterolateral midbrain, but published surgical experiences do not describe results with this approach in the sitting position. Gravity retraction of the cerebellum opens this surgical corridor and dramatically alters exposure, creating 2 variations of the lateral supracerebellar-infratentorial approach: the supracerebellar-supratrochlear approach and the infratentorial-infratrochlear approach. METHODS: We reviewed our experience treating cavernous malformations and arteriovenous malformations (AVMs) of the posteroinferior thalamus and posterolateral midbrain by use of supracerebellar-supratrochlear and infratentorial-infratrochlear approaches. Microsurgical technique, clinical data, radiographic features, and neurological outcomes were evaluated. RESULTS: During an 11-year surgical experience with 341 cavernous malformation patients and 402 AVM patients, 8 patients were identified, 6 with cavernous malformations and 2 with AVMs. Infratentorial-infratrochlear approaches were used in 4 patients (50%), including 3 with inferolateral midbrain cavernous malformations. Supracerebellar-supratrochlear approaches were used in 4 patients (50%), including 2 with posterior thalamic lesions surfacing on pulvinar. Resections were radiographically complete in all cases. There were no new, permanent neurological deficits, nor were there any medical or surgical complications. There has been no evidence of rebleeding or recurrence. CONCLUSIONS: Gravity retraction of the cerebellum transforms the lateral supracerebellar-infratentorial approach, enhancing exposure and approach trajectories that can be achieved with patients in prone or lateral positions. The increased upward viewing angle of the supracerebellar-supratrochlear approach accesses the posteroinferior thalamus. The increased downward-viewing angle of the infratentorial-infratrochlear approach accesses cerebellomesencephalic fissure and posterolateral midbrain. These approaches open wide corridors for safe surgical resection of symptomatic cavernous malformations and AVMs.

Transanterior perforating substance approach to the thalamomesencephalic junction.

OBJECTIVE: Lesions in the thalamomesencephalic junction can be reached via an anterolateral approach, interhemispheric approach, transcortical (parieto-occipital lobule) approach, subtemporal approach, supracerebellar approaches, or transsylvian-insular approach. We now describe a new approach, a transanterior perforating substance approach, to this territory. METHODS: A 33-year-old man with progressive right arm tremors, mild hemiparesis, and a cavernous malformation of the thalamomesencephalic junction was followed for 5 years. Because of clinical progression, he underwent a left orbitozygomatic approach to the cavernous malformation, which could not be accessed because of a high-riding basilar artery. Hence, a new transsylvian corridor of exposure was developed using frameless neuronavigation. The trajectory, which was dorsal to M1, led through the perforating branches of M1. Care was taken to avoid violating any arterial perforators. To reach the lesion, a small opening into the brain was created near the optic tract. RESULTS: The cavernous malformation was resected totally. Postoperatively, the patient's tremors were cured. No visual deficits were encountered. Imaging showed a small ischemic stroke in the basal ganglia likely related to manipulation of a perforator. Initially, his hemiparesis worsened, but it improved significantly within 10 months with only a moderate decrease in strength. CONCLUSION: The transanterior perforating substance approach effectively allowed access to the thalamomesencephalic junction and was associated with significant morbidity. However, the safety of the approach needs further validation. Neuronavigation is indicated to choose the most direct trajectory through the M1 perforators. Tractography may help protect the optic tract.

Submandibular sialoadenectomy with local anesthesia in the era of minimally invasive surgery.

OBJECTIVE: To assess the feasibility and advantages of submandibular sialoadenectomy under local anesthesia. STUDY DESIGN: Retrospective clinical study. SUBJECTS AND METHODS: Twenty patients with benign submandibular masses underwent submandibular sialoadenectomy. The procedure was carried out under local anesthesia (LA) in 12 patients and general anesthesia (GA) in 8 patients. The patient and tumor characteristics, as well as treatment outcome or complications, were compared between the two groups. RESULTS: There was no conversion from LA to GA. Nine patients in the LA group could be discharged as day-case surgery versus none in the GA group, P = 0.001. The postoperative hospital stay was also much shorter in the LA group: 0.4 day vs 2.0 days, P < 0.001. Postoperative vomiting occurred in only two of the eight patients of the GA group. Complication rate was similar. CONCLUSION: Submandibular sialoadenectomy under local anesthesia is feasible. It can shorten the hospital stay and facilitate day-case surgery.

Thalamic cavernous angioma: paraculminar supracerebellar infratentorial transtentorial approach for the safe and complete surgical removal.

BACKGROUND: The thalamic cavernous angioma (CA) represents a neurosurgical challenge because of the critical neurologic functions of the thalamus and its surrounding structures and of their deep location inside the brain. Although the natural history of the thalamic CA remains undefined, several studies suggest the poor outcome of those patients especially if the symptomatic thalamic CA is treated conservatively. We describe the advantage of the paraculminar supracerebellar approach to the lesions in the brainstem. OBJECTIVE: We studied the usefulness and the safety of the paraculminar supracerebellar infratentorial transtentorial approach for the patients with thalamic CA. METHODS: One hundred and ninety two consecutive patients with CA were treated at the Department of Neurosurgery in the Zurich University Hospital between 1993 and 2003. Among these patients, we analyzed six patients (four female, mean age 43) with thalamic CA who underwent surgical removal with the paraculminar supracerebellar transtentorial approach. We retrospectively reviewed their medical charts, the neuroradiological images, and the operative notes/video records. RESULTS: Four patients of the six presented with thalamic hemorrhage. CA existed in the left thalamus in four patients and in the right in two. Preoperative symptoms included sensorimotor disturbance (three cases), double vision (three cases), Parinaud syndrome (one case), and thalamic pain (one case). All patients had the thalamic CA completely removed without any postoperative deterioration. CONCLUSIONS: This study suggests that for the removal of thalamic cavernous angioma the paraculminar supracerebellar infratentorial transtentorial approach provides the spacious surgical field with reduced risks of damaging and sacrificing surrounding vascular and neuronal system. This approach could proffer one of the best and safest surgical routes for the radical removal of thalamic cavernous angioma.

Imaging of a small bowel cavernous hemangioma: report of a case with emphasis on the use of computed tomography and enteroclysis.

Hemangiomas of the small bowel are rare benign tumors, that are dangerous since they may cause massive or occult gastrointestinal bleeding. We describe a case of a jejunum cavernous hemangioma detected by computed tomography (CT) and barium studies. An abdominal CT scan (with intravenous contrast agent) depicted a pronounced contrast enhanced lesion arising from the front wall of a loop of the proximal ileum. Enteroclysis revealed a small intramural nodular defect.

Autologous transfusion with modified total hepatic vascular exclusion for extracapsular resection of giant hepatic cavernous hemangioma.

BACKGROUND: This paper was to review the effects of intraoperative autologous transfusion during modified, normal-temperature, total hepatic vascular exclusion (THVE) for extracapsular resection of giant hepatic cavernous hemangioma. METHODS: The clinical data from 28 patients, who underwent hepatic resection requiring intraoperative autologous transfusion with the cell-saver apparatus, were analyzed retrospectively. The tumors in the 28 patients involved the proximal hepatic veins and inferior vena cava. The volume of these hemangiomas ranged from 12 x 15 cm to 18 x 40 cm. All patients had varying degrees of THVE. RESULTS: The 28 patients with hemangioma received integrated resection and recovered. One patient had rupture of tumors resulting in massive hemorrhage of 6000 ml during liver resection; 4 patients had blood transfusions of 400-800 ml; the other 23 patients had no blood transfusion. Only 6 patients underwent the Pringle maneuver with resection. The other 22 patients underwent THVE during the liver resection. The interval of THVE was 5-30 minutes (mean 16 minutes). CONCLUSIONS: Intraoperative autologous transfusion during modified, normal-temperature THVE for extracapsular resection of huge hepatic cavemous hemangioma is feasible.

Histopathological findings in a surgically resected thalamic cavernous hemangioma 1 year after 40-Gy irradiation.

Stereotactic radiosurgery is a controversial treatment modality in the management of cerebral cavernous hemangiomas (CHs), and results vary from center to center. Even the interpretation of treatment failure is controversial. It is suggested that the systematic pathological investigation of irradiated specimens could help to resolve the controversy. A hemorrhagic lesion in the posterior part of the thalamus had been diagnosed as a tumor and was treated with 40-Gy fractionated radiotherapy. One year after this treatment the case was reconsidered based on new imaging evidence, and the lesion was removed by conventional craniotomy. Histopathological examination revealed a CH with postirradiation changes. Compared with nonirradiated control CH tissue samples, there was endothelial cell destruction and marked fibrosis with scar tissue formation in the stroma of the treated lesion. The histopathological findings in this specimen were similar to those described in arteriovenous malformations after gamma knife surgery. The results of light microscopic investigations suggest that the ionizing effect of radiation energy evokes vascular and connective tissue stroma changes in CHs as well.

Diffuse cavernous hemangioma of the rectum: an atypical cause of rectal bleeding.

OBJECTIVE: cavernous hemangioma of the rectosigmoid colon is a rare disease, with no more than 200 cases reported in the literature. The rectosigmoid is the most common site of this disease in the gastrointestinal tract. CASE REPORT: we report the case of a 31-year-old male with recurrent episodes of rectal bleeding, who was finally diagnosed of diffuse cavernous hemangioma of the rectum. The tumor, of 12 x 10 x 9 cm in size, occupied the rectum to the margin of the anal sphincter. A surgical procedure was ruled out because of the inability to carry out a safe anastomosis while preserving anal sphincters. DISCUSSION: rectal hemangiomas are less frequent vascular malformations. The clinical presentation of a cavernous hemangioma of the rectum is usually acute, recurrent or chronic rectal bleeding. Other symptoms stem from the possible compression or invasion of adjacent structures, such as lumbar or perianal pain, metrorrhage, hematuria, etc. This diagnosis is commonly made in younger patients. Colonoscopy is without doubt the diagnostic technique of choice, and it allows to establish the localization, morphology, and total extension of the lesion; its characteristic image is a red-purplish nodule with great vascular congestion. According to the opinion of most authors, biopsy is not advisable during colonoscopy, since imaging techniques are sufficient for an accurate diagnosis, and the risk of bleeding while manipulating this lesion is not negligible. Computed tomography and particularly magnetic resonance imaging, given their high precision to delimit the lesion and its relations to adjacent structures, are imaging studies that are mandatory before surgical treatment. Other techniques such as selective angiography, barium enema, gastrointestinal transit, and upper-tract endoscopy may be supplementary and help locate more lesions along the gastrointestinal tract. Failure to recognize the exact diagnosis and extent of diffuse cavernous hemangioma may lead to failed surgical treatment and severe complications. Complete surgical excision of the lesion with a sphincter-saving procedure is the primary mode of treatment: conservative proctectomy with coloanal anastomosis.

Cavernous haemangioma of the rectum: an uncommon cause of rectal bleeding.

Cavernous haemangioma of the rectum is an uncommon cause of rectal bleeding. Initial diagnosis is often elusive because of lack of awareness. For accurate diagnosis, investigations such as endoscopy, plain X-ray of the abdomen, barium enema and selective angiography of the inferior mesenteric artery are required. Complete surgical excision of the haemangioma and colo-anal sleeve anastomosis is the most favoured operative procedure to eradicate the disease. We report a case of cavernous haemangioma of the rectum and discuss its salient clinical features, investigations and management.

Thalamic deep brain stimulation for disabling tremor after excision of a midbrain cavernous angioma. Case report.

Thalamic deep brain stimulation (DBS) has been demonstrated to be effective for the treatment of parkinsonian or essential tremor. To date, however, few data exist to support the application of this method to treat midbrain tremor. A 24-year-old right-handed man underwent radiosurgery and subsequent resection of a recurrently hemorrhaging cavernous angioma located in the left side of the midbrain. The surgery exacerbated severe choreoathetotic resting and action tremors of his right extremities and trunk. The patient underwent placement of a deep brain stimulator into the left ventral intermediate nucleus of the thalamus (Vim). Postoperatively, decreased truncal ataxia and right-sided choreoathetotic tremor were demonstrated, with a 57% increase in dexterity as measured by task testing. The authors demonstrate that DBS can be an effective treatment modality for disabling tremor after resection of a midbrain cavernous angioma.

[Gamma knife radiosurgery for the treatment of severe epilepsy]. / Rôle de la radiochirurgie Gamma Knife dans le traitement des épilepsies sévères.

The Gamma Knife radiosurgery is a neurosurgical approach having now demonstrated well its efficiency, its low morbidity and its comfort in the treatment of numerous neurosurgical disorders. These advantages of this type of intervention make it a method of great interest in functional neurosurgery and quite particularly in surgery of epilepsy. French experience is a pionner one in this domain. If for several years the positive evolution of the epilepsy associated to brain lesions had been noticed after the Gamma Knife radiosurgical treatment, the use of this approach in surgery of the epilepsy is systematically estimated since 1993. Data are today available concerning the surgical treatment of the epilepsies originating in temporomesiale area without occupying process, epilepsies associated to hypothalamic hamartomas and epilepsies associated to cavernous angiomas or to low grade gliomas. The quality of the epileptological result obtained in these various indications associated to a very reduced morbidity lets suppose that the Gamma Knife radiosurgery could indeed have tomorrow a place within the sample group of surgical approaches dedicated to the treatment of severe epilepsies. However, a larger number of treated patients and a more prolonged follow-up remains necessary to estimate in a more definitive way this approach.