Paediatric giant cavernomas: report of three cases with a review of the literature.

INTRODUCTION: Cavernous angiomas of the brain (CCM) are being increasingly diagnosed, especially in the paediatric age group. Though classic presentations with haemorrhage or seizures are well recognised, presentation as a large lesion with mass effect is rare and creates difficulty in diagnosis as well as management. METHODS: Our cases of paediatric giant CCMs that presented as a 'mass lesion' are reported here, and the PubMed database for giant CCMs in the paediatric population is reviewed. All articles where the size of the lesion was reported to be > 4 cm were selected for analysis to study the varying modes of presentation, treatment, and outcome; to gain a proper perspective on this distinct entity of 'giant CCMs'. RESULTS: Analysis of a total of 53 cases (inclusive of our 3 cases) reported so far showed slight male preponderance (58.49%). The largest reported lesion was 14 cm in largest diameter. Most of the lesions (83.02%) occurred in the supratentorial region. In the infratentorial region, paediatric giant CCMs were more commonly seen in the cerebellum than in the brainstem. Seizures were observed in 47.17% at presentation. Features of mass effect were the mode of presentation in all our cases, and literature analysis has shown raised intracranial pressure in 37.74% (20 patients) and focal neurological deficit in 33.96% (18 patients) at presentation. Macrocephaly was seen in younger children up to the age of 7 years (16.98% or 9 patients). Gross total resection was carried out (with a good outcome) in all our cases and in 36 of the other 49 analysed patients who were operated on. DISCUSSION: About one-fourth of CCMs occur in paediatric patients. Giant CCMs are rare but can present in children even in the immediate post-natal period. Features of a mass lesion such as raised intracranial pressure, macrocephaly, and focal neurological deficit are much more common than their smaller counterparts. Their appearance on imaging also often causes diagnostic dilemmas with other intracranial mass lesions. Timely surgery with standard microsurgical principles leads to a favourable outcome in the majority. CONCLUSION: Giant CCMs, though rare, often present as a diagnostic challenge. Presentation with mass effect is common, and complete microsurgical excision remains the mainstay of treatment. Though transient neurological deficits may be encountered with this strategy, the long-term outcome remains favourable.

Could propranolol be beneficial in adult cerebral cavernous malformations?

Surgery is the only therapeutic option for cerebral cavernous malformations (CCM) and is proposed, whenever possible, after haemorrhagic events, neurological symptoms, or epilepsy, radiosurgery being a controversial alternative in some cases. However, there is no treatment for non-accessible lesions, such as brainstem CCM, multiple CCM, or those located in functional areas. Propranolol, a non-selective beta-blocker used as first-line treatment for infantile haemangiomas, has proved spectacularly effective in a few cases of adult patients with CCM. We herein review the histological, in vitro data and clinical findings that support the idea of propranolol as a potential treatment for CCM. Since one retrospective study has not been conclusive, we support the idea that prospective trials are necessary.

A case of cauda equina cavernous angioma coexisting with multiple cerebral cavernous angiomas.

The simultaneous presence of cavernous angiomas in both the brain and spinal cord is a very rare finding, as is the location of a cavernous angioma in the cauda equina. We reported a unique case of coexisting with multiple cerebral cavernous angiomas in the brain and cauda equina.

Cavernous Sinus Hemangioma: Imaging Diagnosis and Surgical Considerations.

BACKGROUND: Cavernous sinus hemangiomas (CSHs) are extraaxial vascular malformations that tend to bleed during surgery. METHODS: We reviewed 12 magnetic resonance imaging scans with CSH, 5 of them biopsy proven. RESULTS: In our review, CSH commonly presented as a lobulated mass with high, uniform signal intensity on T2-weighted images, a dumbbell shape, and a sellar extension. Two thirds presented a "filling-in" pattern of enhancement on dynamic imaging. These features should lead to a correct preoperative diagnosis, which is essential for surgical planning and avoiding hemorrhagic complications. CONCLUSIONS: A combination of low signal on T1, high signal on T2 and fluid-attenuated inversion recovery, no diffusion restriction and homogenous enhancement should place CSH at the top of the list of differential diagnoses. This is especially true when there is a "filling-in" pattern on dynamic or delayed imaging. Doing so may alert surgeons to the possibility of copious intraoperative bleeding and therefore avoid complications of hemorrhage.

Opto-chiasmatic apoplexy as a compartment syndrome? Anatomical and surgical considerations on two bleeding cavernous malformations.

OBJECTIVE: Opto-chiasmatic (OC) cavernous malformations are sporadic lesions that are often misdiagnosed clinically and radiologically. Presenting symptoms range from incidental findings to the more frequent and dramatic "chiasmal apoplexy." The present study aims to evaluate the potential role of arachnoidal membranes of the basal cisterns in the onset of OC apoplexy. A possible mechanism resembling a compartment syndrome is discussed through the description of two cases of bleeding cavernomas. METHODS: We describe clinical, radiological, intraoperative findings in two cases of young patients presenting with OC apoplexy from bleeding cavernoma. The first was a 38-year-old man diagnosed with optic neuritis at the first episode of visual acuity deterioration. The second patient was a 22 -year-old woman who suffered two OC apoplexy episodes from a recurrence, which also presented with bleeding. RESULTS: Both patients were operated on via pterional craniotomy and presented a postoperative improvement of visual symptoms. The second patient experienced deterioration 30 months after surgical resection due to rebleeding from a recurrence and required a second operation. Follow-up revealed a good recovery of visual disturbances; MRI at 6 and 3 years showed in both patients an apparent complete removal of the cavernous malformations. CONCLUSION: The cisternal environment where OC cavernous malformations develop and the paradigm of a compartment syndrome could explain the clinical presentation variability. This very rare subset of cavernomas would benefit from a classification system using ad hoc neuroimaging protocols and consistent indications.

Radiation-Induced Cervical Spinal Cord Cavernoma Following Head and Neck Radiotherapy: Case Report.

Cavernous angiomas are congenital vascular malformations that affect the central nervous system. Reports implicated radiation therapy as a triggering factor for the formation of cavernomas but not in relation with head and neck radiation therapy. Radiation-induced cavernomas (RIC) should be considered in the differential diagnosis of focal neurological symptoms in any patient who has received previous cranial-spinal or head and neck radiotherapy.

Cerebral Venous Malformations in a Chinese Population: Clinical Manifestations, Radiological Characteristics, and Long-Term Prognosis.

OBJECTIVE: We elucidated the clinical and radiological characteristics and analyzed the risk factors for hemorrhage and poor outcomes of cerebral venous malformations (CVMs) in a northern Chinese population. METHODS: We included 60 consecutive patients with CVM patients in Beijing Tiantan Hospital from January 2011 to February 2018. The clinical manifestations, radiological characteristics, management, and outcomes were elucidated and analyzed. The patients were followed up for 5-64 months (median, 26). Poor outcomes included repeat bleeding, secondary infarction, severe disability (modified Rankin scale score ≥3), and death. RESULTS: Infratentorial CVMs were more prone to intracranial hemorrhage (75% vs. 28.6%; P < 0.001), dizziness (37.5% vs. 10.7%; P = 0.017), and focal neurological deficits (65.6% vs. 25%; P = 0.002) than were supratentorial CVMs. Supratentorial CVMs were more prone to seizure (32.1% vs. 0%; P = 0.001) than were infratentorial CVMs. Multivariate logistic regression revealed that the major risk factors for intracranial hemorrhage in CVMs were infratentorial lesions (P = 0.003) and complicated cavernous angiomas (P = 0.016). Compared with conservative treatment, resection of hematoma or cavernous angiomas with CVM preservation did not increase the risk of poor outcomes (P = 0.646). However, CVM resection significantly increased that risk (odds ratio, 44.0; P = 0.003). CONCLUSIONS: Our results have shown that conservative treatment of CVMs results in a relatively good prognosis. For those complicated by hemorrhage or cavernous angiomas requiring surgical interventions, the integrity of the CVM should be preserved, irrespective of the treatment. In exceptional cases, before CVM resection, the CVM drainage should be comprehensively evaluated.

The Real Impact of an Intraoperative Magnetic Resonance Imaging-Equipped Operative Theatre in Neurovascular Surgery: The Sapienza University Experience.

The fundamental role of technological instruments in contemporary Neurosurgery is undisputed, and intraoperative magnetic resonance imaging (MRI) represents one of the best examples. The use of a modern high-field magnet and the possibility to match the MRI with an operative microscope and an integrated neuronavigation system has led to successful results in the surgical treatment of different diseases. At our institute, we have performed surgery routinely with the aid of intraoperative MRI over the last 15 years. The aim of this article is to report our experience in the management of neurovascular lesions with the use of this device. We experienced that intraoperative MRI enhanced the surgical experience, leading to an improved postoperative outcome in the treatment of different lesions, such as arteriovenous malformations, dural arteriovenous fistulas, intracranial cavernous angiomas, and intracranial aneurysms. There are several advantages provided by the use of intraoperative MRI. The use of intraoperative MRI coupled with the planning station and the neuronavigation system allows one to obtain preoperative 3-dimensional reconstructions of the vessels, which aids the definition of the anatomy of each neurovascular lesion. Furthermore, the possibility performing an intraoperative scan allows a comparison with preoperative images and, subsequently, the updating of the surgical strategy. Intraoperative diffusion-weighted imaging can detect possible territorial ischemia that would be amenable to intensive treatment. Although increased costs, increased surgical times, increased anesthesiology times, and the possible increased risk of surgical infection may represent some major limitation, the use of intraoperative MRI-equipped operative theaters with integrated neuronavigation systems can prove extremely helpful in the management of neurovascular conditions.

Clinical characteristics and surgical outcomes of solitary spinal epidural cavernous angiomas.

The majority of spinal cavernous angiomas (CAs) originate from the vertebral bodies with or without epidural space extension. Solitary epidural CAs are rare. In the present study, we retrospectively reviewed the records of 12 patients who underwent microsurgery for solitary spinal epidural CAs. All patients had performed pre- and postoperative magnetic resonance imaging. The patients were 7 females and 5 males with the mean age of 52.1 years. Two tumors were located in the cervicothoracic spine, nine in the thoracic spine and one in the lumbar spine, respectively. Solitary epidural CAs generally exhibited isointensity on T1-weighted images and hyperintesity on T2-weighted images. Contrast-enhanced T1-weighted images showed homogeneous markedly enhancement. Gross total resection (GTR) was achieved in 11 cases, and subtotal resection (STR) was achieved in 1 case. During an average follow up of 35.9 months, neurological function was improved in 11 patients and in one patient, preoperative status was maintained. No patient experienced tumor recurrence. These findings suggest that CAs should be considered in the differential diagnosis of spinal epidural lesions. Early surgery is advocated to prevent irreversible neurological deficits. When aggravated by a large amount of acute hemorrhage, neurological deterioration is usually acute and prompt surgical decompression is the optimal choice. Because of the excessive vascularity of CAs, en bloc resection is recommended. In addition, a good clinical outcome after GTR can be expected, and the risk of long-term recurrence is low.

Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb. Various vascular anomalies of the central nervous system have also been described in patients with KTWS. The English language literature to date contains 6 reports of associations between KTWS and spinal cord cavernous angioma (CA), but management of these patients has not been well described. CASE DESCRIPTION: A 23-year-old woman was admitted to our institution with acute onset of leg weakness accompanied by upper back pain. Thoracic magnetic resonance imaging of the spinal cord showed a heterogeneous mass with a slit component at the T1-2 level. The patient underwent left hemilaminectomy followed by removal of the tumor, and her neurologic symptoms improved postoperatively. Pathologic examination showed the spinal lesion was characterized by hemosiderin deposition and thin-walled vascular channels surrounded by fibrous tissue. CONCLUSIONS: This is the first report to provide a detailed pathologic description of the features of spinal CA in a patient with KTWS. Assessment of the clinical features and management of CA associated with KTWS are discussed. This syndrome is rare, and further experience in the treatment of these patients is needed. However, considering that the pathologic findings of spinal CA in patients with KTWS include the typical features of CA, the management of CA in patients with KTWS may be identical to management of isolated CA.

Occurrence of multiple Cerebral Cavernous Malformations in a patient with Neurofibromatosis type 1.

BACKGROUND: Neurofibromatosis 1 (NF1) belongs to the autosomal dominant neurocutaneous disorders' group, which mainly includes NF1 and NF2, tuberous sclerosis, von Hippel-Lindau disease and Cerebral Cavernous Malformations (CCMs). NF1 has a major impact on the nervous system, eye, skin, bone or cardiovascular system. Cerebrovascular lesions have been reported in NF1 including aneurysm, pseudoaneurysm, arteriovenous malformations, vascular stenosis or occlusion and Moya moya syndrome. OBJECTIVE: To report a case of an NF1 patient with multiple CCMs. OBSERVATION: A 47-year-old man with café-au-lait skin lesions, countless cutaneous neurofibromas, short stature and scoliosis was admitted for progressive spinal cord compression due to histologically proven neurofibroma. Systematic cerebral MRI screening including gradient echo sequences showed multiple asymptomatic CCMs. Screening of CCM1, CCM2 and CCM3 genes was negative while a deleterious frameshift mutation was identified in NF1 gene. CONCLUSION: While single CCM can occur in NF1 patients following radiation exposure, they are only rarely reported in non-irradiated NF1 brain. Even if it could be a fortuitous association, plausible links and explanations exist. If cerebral MRI can be systematic in NF1 to detect asymptomatic gliomas, used protocols in neuroradiology do not usually include gradient echo sequences, the most sensitive test for CCM detection, leading possibly to failure to detect these vascular lesions. More reports having this combination and further investigations of NF1 families will certainly provide a better understanding of links between these 2 phakomatoses, as recently reported with "multiple meningiomas" phenotype associated with multiple CCMs in patients with CCM3 gene mutations or café-au-lait skin lesions in CCM1 mutation carriers.

MRI diagnosis of intracranial extra-encephalon cavernous angiomas / 实用放射学杂志

Objective To investigate MRI diagnosis of intracranial extra-encephalon cavernous angiomas.Methods In 9 intracranial extra-encephalon cavernous angiomas,5 masses located in the parasellar,2 masses in the lateral ventricle triangle,1 mass in the fourth ventricle and 1 mass in temporal subdural space.The MRI features of the masses were analyzed.Results 5 parasellar cavernous angiomas enclosed the ipsilateral internal carotid artery and extended the saddle.The masses showed homogeneous low intensity on T1WI,high intensity on T2WI,obvious enhancement on enhanced scan.Of 2 cavernous angiomas in the right lateral ventricle triangle, 1 mass showed homogeneous low intensity on T 1WI,high intensity on T2WI,obvious enhancement on enhanced scan;the other mass showed isointensity on T1WI,slight high intensity on T2WI,high intensity on DWI,low intensity on SWI and patchy enhancement on enhanced scan.1 cavernous angioma located in the fourth ventricular showed mixed intensity on T 1WI and T2WI,low signal rim around the mass on T2WI,inhomogeneous high intensity on DWI sequence and little enhancement on enhanced scan.1 mass located in temporal subdural space showed homogeneous low intensity on T 1WI,high intensity on T2WI,low intensity on DWI,isointensity on SWI and homogeneous obvious enhancement on enhanced scan.Conclusion The MRI findings of the intracranial extra-encephalon cavernous angiomas are characteristic.Most masses show homogeneous low intensity on T1WI,high intensity on T2WI and obvious enhancement on enhanced scan.The masses should mainly be distinguished from meningiomas.