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.

Hemangioma of the rectum - How misleading can hematochezia be?

We present the case of an 18-year-old male patient that was referred to our gastrenterology department with history of intermittent painless hematochezia since childhood. During such instances, he was diagnosed with bowel intussusception, eosinophilic gastroenteritis and inflammatory bowel disease at 4, 6 and 8 years old, respectively. He underwent treatment with 5-aminosalicylic acid for two years, without improvement of symptoms. He was then lost to follow-up until our observation. His physical examination was unremarkable except for digital rectal examination which found a nodular compressible mass by the palpating finger. Blood tests revealed a mild iron deficiency anemia. The colonoscopy showed an extended reddish and bluish multinodular submucosal mass in the rectum, suggesting diffuse cavernous hemangioma of the rectum (DHCR). The Magnetic Resonance Imaging, showed diffuse thickening of the entire rectum extending into the distal sigmoid with the mesorectum revealing multiple serpiginous structures, corresponding to abnormal blood vessels. After discussion, we considered to perform a sphincter-sparing procedure, namely pull through transection and coloanal anastomosis. However, intervention was ruled out by the patient because of his fear of anal incontinence and permanent colostomy. We adopted a conservative strategy with clinical surveillance and iron supplementation. At the present, the patient remains with intermittent rectal bleeding, referring poor quality of life due to his ongoing symptoms. This is a rare case of DHCR. Despite of being a benign disease, the management of DHCR requires a sphincter mucosectomy and pull-through coloanal sleeve anastomosis which has become the first-line procedure. The surgical outcomes are non-expectable in 32% with permanent sphincter lesion or with incomplete DHCR removal. As in this case, the surgeons or patients refusal to perform the intervention is common which represents a challenge to the clinical follow-up.

En face mode of swept-source optical coherence tomography in circumscribed choroidal haemangioma.

PURPOSE: To describe the findings in circumscribed choroidal haemangioma (CCH) using en face swept-source optical coherence tomography (SS-OCT). METHODS: En face images were obtained employing DRI-1 Atlantis OCT (Topcon, Tokyo, Japan), using a three-dimensional volumetric scan of 12×9 mm. Images were obtained from the retinal pigment epithelium to 1000 µm in depth of the tumour. RESULTS: Twenty-two eyes from 22 patients with the clinical diagnosis of CCH were included. In 20 eyes (90.9%), a characteristic pattern was visualised in the en face image across the vascular tumour. A multilobular pattern, similar to a honeycomb, with hyporeflective, confluent, oval or round areas corresponding with the lumen of the tumour vascular spaces, and hyper-reflective zones, which may represent the vessels walls and connective tissue of the tumour. Ten eyes (45.4%) showed a hyper-reflective halo surrounding the tumour. Seventeen tumours (77.2%) showed small diameter vessels at the inner zone and larger vessels in the outer area. Twelve patients (54.5%) had previously received treatment (photodynamic therapy, transpupillary thermotherapy, dexamethasone intravitreal implant or brachytherapy with ruthenium-106). No differences were found between treated and untreated patients in any of the measured parameters. CONCLUSIONS: En face SS-OCT is a rapid, non-invasive, high-resolution, technology, which allows a complementary study to cross-sectional scans in CCH. A characteristic multilobular pattern, with a hyper-reflective halo surrounding the tumour, was found in en face SS-OCT images. No morphological differences were found between naïve patients and patients who received previous treatment.

Transcatheter Arterial Embolization Alone for Giant Hepatic Hemangioma.

Giant hepatic hemangioma is a benign liver condition that may be treated using surgery. We studied the digital subtraction angiographic (DSA) characteristics of giant hepatic hemangioma, and the effectiveness of transcatheter arterial embolization (TAE) alone for its treatment. This was a retrospective study of 27 patients diagnosed with giant hepatic hemangioma and treated with TAE alone (using lipiodol mixed with pingyangmycin) at the Division of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital, Zhejiang University, between January 2010 and March 2013. The feeding arteries were identified using DSA. All patients were followed up for between three weeks and 12 months. Changes in tumor diameter and symptoms were observed. The 27 patients included had giant hepatic hemangiomas ranging from 5.3 to 24.5 cm (mean, 11.24±5.08 cm) in the right (n = 13), left (n = 1) or both (n = 13) lobes. Preoperative hepatic angiography showed multiple abnormal vascular lakes in the early phase, known as the "early leaving but late returning, hanging nut on a twig" sign. On the day after TAE, hepatic transaminase levels were increased (ALT: 22.69±17.95 to 94.88±210.32 U/L; ALT: 24.00±12.37 to 99.70±211.54 U/L; both P<0.05), but not total bilirubin. Six patients complained of abdominal pain, and 12 experienced transient fever. In the months after TAE, tumor size decreased (baseline: 11.24±5.08; 3 months: 8.95±4.33; 6 months: 7.60±3.90 cm; P<0.05), and the patients' condition improved. These results indicated that TAE was effective and safe for treating giant hepatic hemangioma. TAE may be a useful alternative to surgery for the treatment of hepatic hemangioma.

Primary hepatic angiosarcoma: multi-institutional comprehensive cancer centre review of multiphasic CT and MR imaging in 35 patients.

OBJECTIVE: To assess the imaging features of primary hepatic angiosarcoma on multiphasic CT and MR. METHODS: Multi-institutional review identified 35 adults (mean age, 57.1 years; 22M/13F) with pathologically proven hepatic angiosarcoma and pretreatment multiphasic CT (n = 33) and/or MR (n = 7). RESULTS: Multifocal hepatic involvement was seen in all 35 cases, with at least 10 lesions in 74.3% (26/35). Mean size of the dominant mass was 8.9 ± 4.7 cm (range, 2.6-20 cm). Individual nodules were typically circumscribed. Arterial-phase foci of hypervascular enhancement without washout were seen in 89.7% (26/29). Heterogeneously expanding foci of enhancement generally followed blood pool in 88.6% (31/35). Progressive centripetal (n = 16) or diffuse "flash-fill" (n = 4) enhancement pattern resembling cavernous haemangiomas predominated in 20 cases, whereas a "reverse haemangioma" centrifugal pattern predominated in 11 cases. Rapid interval growth was seen in 24 (96.0%) of 25 cases with serial imaging. Vascular invasion was not seen in any case. Underlying cirrhotic morphology was seen in 42.3% (15/35). CONCLUSION: Primary hepatic angiosarcomas typically manifest as aggressive multifocal tumors containing small heterogeneous hypervascular foci that progressively expand and follow blood pool. The appearance can mimic cavernous haemangiomas, but distinction is generally possible. In the setting of cirrhosis, lack of tumour washout and vascular invasion argue against multifocal hepatocellular carcinoma. KEY POINTS: • Hepatic angiosarcoma manifests on CT and MR as rapidly progressive multifocal tumours • Multiphasic imaging demonstrates hypervascular foci that progressively expand and follow blood pool • Enhancement pattern can resemble cavernous haemangiomas or show a "reverse" centrifugal pattern • Lack of tumour washout of hypervascular lesions argues against multifocal hepatocellular carcinoma • Careful assessment of the cross-sectional imaging findings may suggest the diagnosis.

Single Cavernous Hemangioma of the Small Bowel Diagnosed by Using Capsule Endoscopy in a Child with Chronic Iron-Deficiency Anemia

Cavernous hemangiomas of the gastrointestinal tract are extremely rare. In particular, the diagnosis of small bowel hemangiomas is very difficult in children. A 13-year-old boy presented at the outpatient clinic with dizziness and fatigue. The patient was previously diagnosed with iron-deficiency anemia at 3 years of age and had been treated with iron supplements continuously and pure red cell transfusion intermittently. Laboratory tests indicated that the patient currently had iron-deficiency anemia. There was no evidence of gross bleeding, such as hematemesis or bloody stool. Laboratory findings indicated no bleeding tendency. Gastroduodenoscopy and colonoscopy results were negative. To obtain a definitive diagnosis, the patient underwent capsule endoscopy. A purplish stalked mass was found in the jejunum, and the mass was excised successfully. We report of a 13-year-old boy who presented with severe and recurrent iron-deficiency anemia caused by a cavernous hemangioma in the small bowel without symptoms of gastrointestinal bleeding.

Sclerosing cholangitis secondary to bleomycin-iodinated embolization for liver hemangioma.

Sclerosing cholangitis (SC) is a rarely reported morbidity secondary to transcatheter arterial chemoembolization (TACE) with bleomycin-iodinated oil (BIO) for liver cavernous hemangioma (LCH). This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO. Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts. Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy. Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy. Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area. Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy. Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.

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.

Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass.

We report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas.

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.

Vertebral and spinal cavernous angiomas associated with familial cerebral cavernous malformation.

BACKGROUND: Cerebral cavernous malformations are vascular malformations that affect the CNS and have been associated with cutaneous, retinal, and hepatic lesions. Until now, vertebral hemangiomas associated with CCM have been described only in one case. The coexistence of intracranial and spinal cavernous angiomas in familial CCM is extremely rare. In addition to previous studies, the occurrence of spinal, vertebral, and cutaneous cavernous angiomas is now described in different members of a large family with CCM. CASE DESCRIPTION: Our study reports a previously described family (IFCAS-07) with 12 members affected by autosomal dominant cavernous angiomas: 11 had CCM either alone or associated with hepatic or retinal angiomas, and one had only hepatic angioma. In all 11 members affected by CCM, the mutation of CCM1 gene was detected. During the follow-up, 8 subjects underwent a spinal MRI: 2 because they were symptomatic (thoracic paresthesias, enuresis, back pain) and 6 as a screening examination. Spinal MRI showed in 5 subjects spinal cavernous angiomas either alone or associated with vertebral hemangiomas. CONCLUSIONS: To our knowledge, this is the largest family reported with different subjects affected by CCM associated with multiple cavernous angiomas throughout (brain and spinal cord) and besides (retina, skin, liver, and vertebral column) the CNS. Comprehensive care of patients with familial CCM includes screening of all the tissues that can be affected and appropriate management by specialists. We emphasize the importance of spinal MRI in the diagnosis of spinal and vertebral cavernous angiomas in all patients affected by familial CCM.

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.