Cavernomas de tronco encefálico: presentación de 8 casos operados, análisis y revisión del estado del arte / Brainstem cavernous malformations: a series of 8 surgicalcases, analysis and state of the art review

Introducción: Las lesiones del tronco cerebral son uno de los mayores desafíos neuroquirúrgicos. Los angiomas cavernosos de esta localización son lesiones de comportamiento agresivo, con alta morbi-mortalidad asociada, donde el neurocirujano tiene la posibilidad de curar al paciente pero con un nivel de riesgo que hacen que la oportunidad de la cirugía se mantenga en debate. Material y Método: Análisis retrospectivo de 8 casos de cavernomas de tronco operados entre los años 2009-2013 con sus características clínicas, quirúrgicas y de seguimiento. Se realiza además una revisión reflexiva sobre la evolución del manejo de estas lesiones y del estado del arte a nivel en el concierto internacional. Resultados: Los 8 casos presentaron evoluciones inmediatas y mediatas favorables tras la cirugía. No hubo mortalidad ni empeoramiento del status neurológico en relación al estado preoperatorio en ninguno de los 8 casos. Tres de los pacientes de la serie fueron operados tras caer en una condición clínica crítica y son los que registran los mayores déficits durante el seguimiento. El análisis de la evolución del manejo de este tipo de lesiones a nivel mundial revela una tendencia hacia el manejo quirúrgico precoz en lesiones sintomáticas. Conclusiones: Los resultados de esta serie así como la evolución del estado del arte permiten concluir que en pacientes sintomáticos una cirugía precoz ofrece en general mejores expectativas que intervenciones tardías para lesiones relativamente superficiales.

Introduction: Brainstem lesions are a major neurosurgical challenge. Cavernous angiomas of this location are lesions of aggressive behavior, with high morbidity and mortality associated, where the neurosurgeon has the possibility to cure the patient but with a level of risk that makes that the surgery timing remains under debate. Material and Methods: A retrospective analysis of 8 brainstem cavernomas cases operated in the period 2009-2013 is presented whit its clinical, surgical and follow-up characteristics. A thoughtful review of the evolution of the management of these lesions and the state of art in the international level is also carried out. Results: All 8 patients presented immediate and mediate favorable changes after surgery. There was no mortality or worsening of the neurological condition in relation to the preoperative status in any of the 8 cases. 3 patients in this series underwent surgery after falling in a critical condition and are those who have the highest deficit in the follow-up. The analysis of the evolution of the worldwide management of these injuries reveals a trend towards early surgical management in symptomatic lesions. Conclusions: The results of this series as well as the evolution of the state of the art let us conclude that in symptomatic patients an early surgery offers overall better expectations than later interventions in relatively superficial lesions.

The postirradiation incidence of cavernous angioma is higher in patients with childhood pineoblastoma or primitive neuroectodermal tumors than medulloblastoma.

PURPOSE: The purpose of this study is to investigate the incidence of cavernous angioma (CVA) in long-term survivors of childhood embryonal tumors treated by cranial irradiation. MATERIALS AND METHODS: Between 1990 and 2012, we treated 25 patients (13 males, 12 females) with embryonal tumors (17 medulloblastomas, 5 primitive neuroectodermal tumors (PNET), 3 pineoblastomas) with craniospinal irradiation. Follow-up ranged from 15.5 to 289.9 months, the irradiation dose to the whole neural axis from 18 to 36 Gy, and the total local dose from 49.6 to 60 Gy. All patients underwent follow-up magnetic resonance imaging (MRI) studies at least once a year, and the diagnosis of posttreatment CVA was based solely on MRI findings. RESULTS: At the time of this writing, 18 were alive and free of the recurrence of the original disease or the development of secondary neoplasms other than CVA; another 2 were alive with medulloblastoma or diffuse astrocytoma. Posttreatment, 14 patients developed CVAs in the course of a median of 56.7 months; 13 of these presented with multiple CVAs. Patients who underwent radiation therapy (RT) at an age younger than 6 years developed multiple CVAs significantly earlier than those treated at a later age (p = 0.0110). Patients with PNET or pineoblastoma developed Zabramski type 1 and 2 CVA significantly earlier than did medulloblastoma patients (p = 0.0042). CONCLUSION: We attribute the high rate of post-RT CVA in our long-term follow-up study of pediatric patients to the delivery of cranial irradiation for embryonal tumors, especially PNET and pineoblastoma, and recommend the regular, long-term follow-up of patients whose embryonal tumors were treated by cranial irradiation.

Management and outcome in adult intramedullary spinal cord tumours: a 20-year single institution experience.

BACKGROUND: Several uncertainties remain concerning the management of intramedullary spinal cord tumours (IMSCTs). These include the timing and extent of resection, its interrelated functional outcome, and the adequate use and timing of radiation therapy and/or chemotherapy. In this retrospective study we report on all adult cases involving IMSCTs treated from 1987 to 2007 in our institution to validate our treatment strategy for IMSCTs. Pre- and post-operative functional performance was classified according to the McCormick scale. RESULTS: A total of 70 adult cases with IMSCTs consisting of ependymoma (39), astrocytoma (11), carcinoma metastasis (8), haemangioblastoma (5), cavernoma (3) and others (4) were reviewed. Mean age was 46.8 years (range, 18-79 years), and mean follow-up was 4.5 years (range, 1-195 months). The proportion of localisation in descending order was thoracic (36%), cervical (33%), cervicothoracic (19%) and conus region (13%), with 45 gross total resections, 22 partial resections and three biopsies. Surgery-related morbidity with worsening postoperative symptoms occurred immediately in 13 patients (18.6%). The preoperative McCormick grade correlated significantly with the early postoperative grade and the grade at follow-up (χ2-test; p=0.001). None of the patients with preserved intraoperative evoked potentials exhibited significant postoperative deterioration. The degree of resection was correlated with progression-free survival (Duncan test; p=0.05). Most patients with malignant tumours, namely anaplastic ependymoma (3), astrocytoma (2) or metastatic lesions (5), underwent postoperative radiation therapy. Six patients (one anaplastic ependymoma, two anaplastic astrocytomas and three metastatic lesions) received postoperative chemotherapy. CONCLUSIONS: IMSCTs should be operated on when symptoms are mild. We recommend evoked potential-guided microsurgical total resection of ependymomas and other benign lesions; partial resection or biopsy followed by adjuvant therapy should be confined to patients with high-grade astrocytomas, whereas resection or biopsy with adjuvant therapy is the best option for metastatic lesions.

Gamma knife radiosurgery of brain cavernomas.

PURPOSE: Radiosurgery of cavernomas should prevent rebleeding, growth of the lesion, and deterioration of clinical symptoms. However, there is no direct diagnostic tool to verify the endpoints of treatment. At present, the positive effects of radiosurgery are identified by clinical observation and analysis of imaging changes on magnetic resonance imaging during a sufficiently long follow-up period. METHODS: Between 1992 and 2000, a total of 112 patients with brain cavernomas were treated with Gamma Knife radiosurgery at our center. In all, 59 patients experienced bleeding before radiosurgery; the remainder did not. The median age of patients was 42 years, the median volume of the cavernomas was 0.9 cm(3), and the median applied marginal dose was 16 Gy. RESULTS: After a 2-year latent interval after treatment (median follow-up 84 months), the risk of bleeding in the group of patients with bleeding before radiosurgery had decreased from 3.7 % to 0.2 %. For the patients without bleeding before radiosurgery, the annual risk of bleeding was 0.8 %. The cavernoma size decreased in 53.0 % of cases and increased in 6.4 %. Epilepsy, if present before the treatment, was alleviated in 45 % of cases. The risks of temporary or permanent morbidity caused by radiosurgery were 14.6 % and 0.9 %, respectively. CONCLUSION: Radiosurgery of cavernomas was associated with a low risk of permanent morbidity. The risk of rebleeding after the 2-year latent interval after radiosurgery had decreased. Treatment of cavernomas with no history of bleeding was halted at our center.

Feasibility and long-term evolution of TIPS in cirrhotic patients with portal thrombosis.

AIM: Many researchers consider portal thrombosis (PT) as a contraindication to transjugular intrahepatic portosystemic shunt (TIPS). The aim of this retrospective study was to compare the feasibility and long-term prognosis of TIPS in cirrhotic patients, with and without, complete PT. PATIENTS AND METHODS: Four hundred and thirty-six consecutive cirrhotic patients with portal hypertension were referred for TIPS, between 1990 and 2004. These patients were divided into two groups according to their portal patency. PT+: 34 patients with complete PT with cavernoma (19) or without (15) cavernoma versus PT-: 402 patients with normal portal patency (308) and partial PT (94). Epidemiological data were compared using the chi and Student's t-tests, and comparative evolution was made from actuarial data using the log-rank test. RESULTS: PT+ patients were more frequently women with viral hepatitis, and TIPS was performed more often for bleeding indications. The TIPS success rate was significantly lower in the PT+ group (79%) than in the PT- group (99.5%) (P<10). Presence of a cavernoma decreased the success rate to 63%. TIPS was always feasible in cases of recent PT and portal cavernoma with an accessible intrahepatic patent portal branch. Early and late outcome and complications were not significantly different between the two groups. CONCLUSION: Complete PT does not modify TIPS' long-term outcome. Rather than a contraindication, PT should be considered as an indication for TIPS in cirrhotic patients with accessible intrahepatic portal vein. Further randomized studies should be planned in cirrhotic patients with recent PT to better qualify TIPS and anticoagulation indications, respectively.

[Transjugular intrahepatic portosystemic shunt in patients with portal vein thrombosis]. / Transjugulärer intrahepatischer portosystemischer Shunt bei Patienten mit Pfortaderthrombose.

PURPOSE: To evaluate the feasibility of TIPS for portal vein thrombosis (PVT) with/without portal vein occlusion/cavernomatous transformation and with/without underlying cirrhosis. MATERIALS AND METHODS: 13 patients with PVT and refractory ascites (n=7) and variceal bleeding (n = 6) received TIPS placement. The function of the TIPS was controlled with Doppler US at 1 day, 1 week, 4 weeks and 4 months after TIPS placement and subsequently every 3 months. The rates of technical success, revision and mortality after TIPS were evaluated. RESULTS: The TIPS was successfully placed in 85% (11/13) of the cases. TIPS placement was successful in 87.5% (7/8) of patients with occlusion of the right portal vein and in 100% (5/5) of patients with non-occlusive right portal vein (p > 0.05), in 80% (8/10) of cirrhotic (x/10) and in 100% (3/3) of non-cirrhotic patients (p > 0,05), as well as in one patient with a fresh PVT and in 1 of 2 patients with cavernous transformation. 30 and 14.3% of patients needed a TIPS revision in the first and second year, respectively. No patient with occlusion of the right portal vein required a revision. 54.5% (6/11) of patients died within 6 months. Under exclusion of 4 patients with advanced malignant diseases, the mortality rate was 28.6%. CONCLUSION: TIPS should be considered for selected patients with symptomatic occlusive PVT with/without cavernous transformation and with/without underlying cirrhosis. The frequency of TIPS revision is not greater, and the mortality rate 6 months after PVT recanalization seems to be comparable with that of patients without PVT.

Does the psychological request of the patient justify the surgery for hepatic hemangioma?

BACKGROUND/AIMS: The incidental finding of hemangiomas has increased, but the problem of the correct surgical indications of this tumor has yet to be solved. The aim of this work is to establish whether the psychological request of surgery from patients known to have a benign tumor of the liver must be avoided or not. METHODOLOGY: Age, sex, symptoms, estroprogestinic oral therapy, methods of diagnosis, surgical procedures, morbidity, mortality, postoperative hospital stay and follow-up of the patients affected by hepatic hemangioma, observed from 1992 to 2002 in our institution, have been considered. RESULTS: Seventeen patients, with a mean age of 44 years (range 26-72), were hospitalized for hepatic hemangioma, 8 (47%) of them were operated on and 9 (53%) were managed by observation. The operated patients presented various symptoms. One patient was operated on for traumatic rupture of the hemangioma. Non-operated patients were asymptomatic or with slight dyspeptic symptoms not related with the tumor. The first diagnostic radiological examination was ultrasonography (US) in all cases. All lesions were larger than 4cm. The types of surgical procedures were 5 enucleations, and 3 hepatic resections. All operated patients resolved their clinical symptomatology, except two patients that had requested surgery for psychological implications. These patients presented their symptoms again after 2and 3 years of follow-up respectively. CONCLUSIONS: Our results suggest that liver hemangiomas should be operated for symptoms well related to the tumor or for bleeding. Psychological requests from the patients should be avoided every time.

Clinical experience of hepatic hemangioma undergoing hepatic resection.

The indications for surgery on cavernous hemangiomas, the most common benign tumors of the liver, remain unclear. This study reviewed 43 patients with cavernous hemangioma of the liver who underwent hepatic resection from 1984 to 2000. Patients were divided into three groups based on the reasons for surgery. Group I comprised 13 patients whose lesions presented symptoms and dimensions that were the main indications for operation. Group II consisted of 28 patients diagnosed with malignant tumors or who displayed malignant growth that could not be ruled out preoperatively. Group III comprised 2 patients with tumors found incidentally at laparotomy for other malignancies. No surgical mortality related to hepatectomy was noted. Postoperative bile leak was found in 2 (morbidity rate: 4.7%). Patients were followed up from 6 months to 12 years. Thirteen residual tumors progressed in size. The clinical status or symptoms changed only slightly in 10 patients with recurrence. The results suggest that resection therapy is an effective indicator for patients with symptoms and a questionable diagnosis. Hepatic resection may and should be carried out with no mortality and minimal morbidity risks since the lesion is benign.

Intermittent portal triad clamping in resection of liver tumors involving the hepatocaval confluence.

OBJECTIVE: To review our experience in and the results of resecting liver tumors involving the hepatocaval confluence under intermittent portal triad clamping (PTC). METHODS: Sixty-eight consecutive patients with liver tumors involving the hepatocaval confluence underwent hepatectomies with liver parenchymal transections under intermittent PTC. RESULTS: All the tumors were successfully resected under PTC, except for one in which the infrahepatic inferior vena cava was concomitantly occluded in addition to PTC. There was neither operative death nor uncontrollable massive bleeding or air embolism occurred in our patients. The bleedings from the main and short hepatic veins and right adrenal veins were properly managed during the operation, with a mean intraoperative blood loss of 1400 ml. Of the 68 tumors resected, 65 were hepatocellular carcinomas (HCC). Their 1-, 2-, 3- and 4-year survival rates were 64.11%, 52.82%, 44.90% and 36.98%, respectively, and the patients with HCC with capsules survived significantly longer than those with HCC without capsules. CONCLUSIONS: The liver tumors involving the hepatocaval confluence could be safely resected simply under PTC, without routine use of total hepatic vascular exclusion. As for HCCs in this area, the tumor with capsule is a better indicator for surgical resection than that without capsule.

[Intracranial cavernoma. Surgical results of 47 cases]. / Cavernomes intracrâniens. Résultats chirurgicaux de 47 cas opérés.

We report the surgical results in a series of 47 patients with cerebral cavernous malformation who had undergone surgery between 1973 and 1994, with a follow up ranging from 12 months to 24 years (mean: 4 years). They were divided in there groups according to their initial clinical presentation: epilepsy (31 cases), hemorrhage (11 cases) and neurological deficit (5 cases). Surgery consisted of cavernoma resection only (11 cases) or its extension to surrounding gliotic tissue (36 cases). Results are satisfactory: no surgical mortality, low morbidity (4 cases), no recurrent hemorrhage, seizures disappearance with anticonvulsant therapy stop (4 cases) or alleviation (20 cases). Only one patient died far from surgery (6 months) consequently to his initial bleeding, while all the others lead a normal active life. The therapeutic management, compared to the literature, pleads in favour of intentionally surgical attitude and gliotic tissue removal as often as reasonably possible.

Hepatic resection of giant cavernous hemangioma of the liver.

Surgical treatment of giant hemangioma of the liver is still controversial. The aim of this study is to examine the efficacy of hepatic resection for giant hemangioma of the liver. Twenty patients with giant cavernous hemangioma of the liver were treated by hepatic resection. The mean diameter of the hemangiomas was 13.9 cm (range, 6.5-30 cm). The surgical outcome was reviewed retrospectively. Major hepatectomy was performed in 14 patients and minor hepatectomy in 6 patients. Complications occurred in 7 of the 20 patients treated by hepatic resection. At a mean follow-up of 79 months (range, 12-173 months), 18 patients were symptom free whereas 2 patients had died--one died of pneumonia at 2 years and the other died of gastric cancer 6 years after surgery. Mean intraoperative hemorrhage and blood transfusion in all patients was 4,343 mL (range, 270-24,000 mL) and 1,860 mL (range, 0-8,800 mL) respectively. In the seven patients with preoperative high levels of fibrin degradation products (FDP), mean intraoperative hemorrhage and blood transfusion were markedly higher (9,371 mL and 3,714 mL respectively) than in the 13 patients without abnormal FDP (1,603 mL and 900 mL respectively). Preoperative hematologic status returned to normal after operation in all patients. Hepatic resection is a useful treatment for giant cavernous hemangioma of the liver. More careful management to reduce intraoperative hemorrhage is recommended to increase the safety of surgery, particularly in patients with preoperative abnormal FDP.

[Surgical treatment of giant hemangiomas of the liver]. / Chirurgische Therapie hepatischer Riesenhämangiome.

Giant liver hemangiomas are defined as hemangiomas with a diameter of more than 4 cm. They often require surgery due to bleeding tendencies or local compression. Between 1994 and 1998 we operated 11 patients with giant hemangiomas (median diameter 5.8 cm, range 4-12.5 cm). Average age was 50 years (range 23-85 years). 6 patients complained of pain, 1 patient sustained a bleeding. 7 hemangiomas were enucleated, 2 segmentally resected, 1 patient underwent a hemihepatectomy. 1 patient suffered from a bile leakage. Mortality was 0%. We conclude that clinically symptomatic liver hemangiomas can be safely resected. In a right sided localisation a enucleation has the best parenchyma sparing effect, in left sided locations a segmentectomy or bisegmentectomy can be performed with little blood loss.

[Cavernoma. Indications for surgical removal and outcome]. / Kavernome. Indikation zur operativen Entfernung und Ergebnisse.

Between July 1990 and October 1994 26 patients were operated on for 26 intracranial and 2 intraorbital cavernous hemangiomas. We found seizures in 62% of our patients, focal neurological deficits and unspecific complains (like headache or dizziness) in 19% each. The average follow-up period was 12 months, 24 patients could be included in this study. 12/14 patients of the seizure group improved, 10/14 reported a complete relieve of their epilepsy. All 5 patients with focal deficits improved, among them 2 with no residual deficit. Finally 3/5 patients with unspecific complains improved, 2/5 remained unchanged. The functional morbidity was 4% (one slight aphasic syndrome), we did not have any mortality. Therefore we conclude that a neurosurgical treatment is indicated in any case of cavernous hemangioma with focal deficits or intractable epilepsy. The operation should also be considered in patients with supressed seizures by anti-convulsants, if the malformation is not located in an eloquent area. Due to the risk of spontaneous bleeding (comparable to incidental aneurysms), the indication for a neurosurgical treatment mainly depends on the location of the cavernoma in cases of its accidental discovery.

Management strategies and surgical techniques for deep-seated supratentorial arteriovenous malformations.

The therapeutic options for arteriovenous malformations (AVMs) of the thalamus and the basal ganglia have expanded to include preoperative embolization, stereotactic radiation, and microsurgery. Adjuncts to surgery such as stereotactic guidance, electrophysiological monitoring, intraoperative ultrasound, intraoperative angiography, and induced hypotension have significantly reduced postoperative morbidity. We review the management and outcome of 65 consecutive patients who were treated for deep-seated supratentorial vascular malformations; 45 patients (69%) were treated surgically, 10 patients (15%) were treated conservatively, and 10 patients (15%) underwent radiosurgery. This retrospective study (1976-1993) includes 51 AVMs (78%), 14 cavernous angiomas (22%), and 10 associated vascular anomalies (15%). Initially, 59 (91%) of 65 patients presented with hemorrhage; 23 patients (39%) suffered recurrent hemorrhages. Malformations ranged in size from 1 to 7.5 cm (mean, 2.8 cm). AVMs were fed principally by the anterior and posterior choroidal, thalamoperforate, and lenticulostriate arteries. Venous drainage was uniform via the deep venous system. Among 39 patients who underwent surgery for AVMs, 26 (67%) improved, 7 (18%) remained unchanged, 5 (13%) worsened, and 1 (3%) died. Among six patients who underwent surgery for cavernous angiomas, four (66%) improved, one (17%) remained unchanged, and one (17%) worsened. Operative complications included transient neurological deficits in seven patients (16%), permanent neurological deficits in six patients (13%), and new bleeding from residual AVMs in four patients (9%). Among 10 patients treated conservatively, 3 (30%) had repeat hemorrhages, 2 (20%) had progressive neurological deficits, and 1 (10%) died.(ABSTRACT TRUNCATED AT 250 WORDS)

Cavernous malformations of the brain stem. A review of 139 cases.

A retrospective analysis of 139 patients with brain stem cavernous malformations is presented. The material consists of 41 cases from Bern and Phoenix and 98 further well-documented cases from the literature. Sixty-eight patients were male, 70 were female. The average age was 31.8 + 11.8 years. Sixty-two percent of the cavernous malformations were in the pons, 14% were in the mesencephalon, 12% were in the pontomesencephalic and in the pontomedullary junction, and 5% were in the medulla. Eighty-eight percent of the patients showed evidence of recent or previous hemorrhage, 55% had one hemorrhage. 17% had two hemorrhages, and 17% had three or more hemorrhages. Twelve patients died from a hemorrhage, 5 with the first bleeding and 7 with a rebleeding. The minimum bleeding rate was 2.7% per year and the average rebleeding rate 21% per year and per lesion. Most lesions had a diameter between 10 and 30 mm. Increase in size was observed in 12 of the patients; this corresponds to about 21% when only patients with a follow-up of at least one year are considered. In 93 patients the cavernous malformation was removed operatively while in 30 patients the lesion was not removed. In the group with conservative management at the end of the observation period (up to 25 and 32 years), 66.6% had no or only a slight neurological deficit, 6.7% were moderately disabled, 6.7% were completely dependent, and 20% had died. In the group treated surgically 83.9% had no or only a slight neurological deficit, and 15% were moderately disabled. One patient remained severely disabled, no patient died. The limitations of the retrospective nature of this study are stressed.

Solitary extradural cavernous hemangiomas in the spinal canal. Report of five cases.

Five cases of solitary extrathecal cavernous hemangioma in the spinal canal are reported. In one case, two coexistent massive cerebral venous medullary malformations were found. The symptomatology and prognosis of extrathecal spinal cavernous hemangiomas are discussed, as is the significance of various diagnostic measures. The patient material further permitted a rough calculation of the incidence of symptomatic extrathecal cavernous hemangiomas in the spinal canal.

Intraventricular cavernous angioma. A survey.

Since the availability of CT diagnosis 23 cases of intraventricular cavernous angioma (IVCA) have been published in the literature. Three additional cases have been operated upon in our Department. Based on these 26 cases the clinical data, radiological findings, treatment and outcome of IVCAs are reviewed.

Excisional therapy for benign hepatic lesions.

With the recent advances in imaging techniques, increased numbers of hepatic lesions are found today, and surgeons are asked frequently for the best course of management. Benign hepatic tumors sometimes cause life-threatening complications and more often trigger disabling or annoying symptoms in otherwise healthy individuals. Although various imaging techniques are quite accurate in identifying cysts and hemangiomas, other benign hepatic lesions, such as adenomas, focal nodular hyperplasia and other benign solid tumors, cannot be differentiated from malignant lesions with a high degree of confidence.