Cavernous hemangioma is a benign intraorbital tumor. Even though it is benign, the mass effect of the tumor causes different manifestations, including painless protrusion of the eye, hyperopia and diplopia.
Patients with cavernous hemangioma may not be aware of the condition because the benign tumor usually causes no symptoms other than a slowly progressing protrusion of the eye, which some people might not notice in early stages. Cavernous hemangioma usually presents in the middle age (around 40 years of age). The main symptom patients present with is a painless protrusion of the globe or around the eye progressing slowly, which might be accompanied by other symptoms, such as the feeling of fullness around the eye and changes in visual acuity including hyperopia and diplopia due to mass effect compression caused by the tumor on the extraocular muscles and surrounding tissues. Some studies suggest that the symptoms may be more severe among pregnant women . The onset of symptoms may differ depending on the location of the tumor, for example an extradural cavernous hemangioma behaves differently from the intramedullary ones, as well as from an extradural one in the spine  .
Entire Body System
- Vascular Disease
FFA) Retinal Macrovessels and Arteriovenous Communications Tortuous Vessels, Vascular Abnormalities Unilateral Optic Disc Pseudodoubling (Colour Images) Diseases of Hematopoesis (see under Systemic Diseases) Hereditary Degenerations and Dystrophies Infections [atlasophthalmology.net]
Occlusive cerebro-vascular disease 1144 33.1. Atherosclerotic cerebrovascular disease 1144 33.2. Cerebral arterial dissections 1160 33.3. Extracranial-intracranial (EC/IC) bypass 1165 33.4. Cerebrovascular venous thrombosis 1166 33.5. [worldcat.org]
[…] called a haemorrhagic stroke The severity and duration of symptoms can vary depending on the type of cavernoma and where it's located. [nhs.uk]
CCMs can bleed and cause strokes, seizures, and headaches. CCMs are often caused by an inherited gene mutation (alteration) in one of three CCM genes (CCM1, CCM2, or CCM3). [clinicaltrials.ucsf.edu]
Stroke 1010 29.1. Cerebrovascular hemodynamics 1010 29.2. Strokes: general information 1011 29.3. Stroke in young adults 1024 29.4. Lacunar strokes 1026 29.5. Collateral circulation 1027 29.6. "Occlusion" syndromes 1027 29.7. [worldcat.org]
While a cavernous angioma may not affect function, it can cause seizures, stroke symptoms, hemorrhages, and headaches. Approximately one in 200 people have a cavernoma. [mountsinai.org]
The workup in order to diagnose cavernous hemangioma includes several tools, such as CT scans, ultrasound studies, doppler flow studies, and MRI images.
The CT scan usually shows a homogenous lesion, which is oval or round in shape and sharply marginated. It usually enhances after injection of contrast; however, enhancement is not mandatory. CT scans alone are not sufficient to reach a definite diagnosis. In addition, a uniform high-echogenicity may be found in an ultrasound. Doppler flow studies may show low blood flow in the hemangioma and a specific honeycomb pattern of alternating strong and weak echos   . Because of the static blood in the vascular spaces, a negative doppler phenomenon may be observed . The bone usually appears normal on the CT scans  . Phleboliths may be detected as calcification on the CT scan.
Cavernous hemangioma does not contain large vessels, and that is why hypervascularity is not demenostrated on MRI in these cases  . T1-weighted MRI images usually show low intensity or they could be isointense, while T2-weighted images may show high intensity. Hypointensity suggests the presence of phleboliths. An extradural cavernous hemangioma is homogenous on CT and MRI studies because of its sinusoidal channels .
Angiography may be an option in the diagnosis of cavernous hemangioma; however, it is rarely used because CT scans, MRI images, and ultrasound studies suffice in reaching the diagnosis  .
Histopathologic studies may aid in the diagnosis of cavernous hemangioma, and they usually show engorged tightly knit vascular channels, which are lined by a layer of endothelial cells and separated by fibrous septae.
Cavernous hemangioma usually does not require any medical or surgical intervention, and observation with followup suffices. However, if mass effect symptoms develop, surgical intervention might be needed in order to relieve the compression on the other tissues and minimize the symptoms .
Several approaches and procedures can be used in the excision of the tumor and the choice depends on its location. They include midfacial degloving, lateral rhinotomy, trans-palatal, and trans-antral approach. Le Fort I osteotomy may also be used.
The most successful procedure involves complete excision of the tumor with ligation or cautery of the vessels feeding it . Other procedures like cryotherapy or resection with YAG laser may also be used. Emoblization of the hemangioma may be successful in the treatment . In case of partial removal of the tumor, a risk of recurrence remains. Therefore, it is advised to use radiotherapy as an adjuvant .
Lately, there have been advances in radiosurgery and radiosurgical equipment, which are becoming a good option for the management of cavernous hemangioma .
Radiotherapy was used in the past as a treatment for cavernous hemangioma but is now strongly advised against due to its adverse effects. It remains a treatment option where surgery is not possible.
Cavernous hemangioma is considered a benign tumor and has a very good prognosis. Usually, there is no need for any medical or surgical intervention and the tumor remains stable throughout the patient's life. However, in some cases, as the tumor grows slowly, it might cause mass effect and compress the surrounding tissues and structures, which affects the visual acuity. Furthermore, other symptoms like diplopia or hyperopia may be observed .
Surgical intervention is rarely required and observation can be sufficient; however, if mass effect symptoms occur and affect the patient, surgery may be needed in order to excise the tumor. Different surgical approaches may be used depending on the location of the tumor. The complete removal of the tumor ensures an excellent prognosis, whereas some risk of recurrence remains after partial excision. Some features and complications caused by the mass effect of the tumor, such as hyperopia, may persist even after successful full excision.
The cause of cavernous hemangioma is the formation of new vessels and proliferation of the vessel wall tissue components, which result in hyperplasia of cellular elements .
Cavernous hemangioma is the most common intraorbital tumor in adults . It appears that there is no difference in the incidence depending on race or gender; however, some studies suggest that the incidence is slightly higher among women than men  . The tumor usually presents in the middle age (around the age of 40) .
Cavernous hemangioma is usually an intraorbital tumor. While hemangiomata are most commonly seen on the extremities or the trunk, they may also present on other parts of the body, such as the head and the neck.
Even though cavernous hemangioma is a benign intraorbital tumor, it may be considered malignant because of its mass effect and the resultant complications, such as hyperopia and diplopia. The composition of cavernous hemangioma includes dilated cavernous vascular spaces separated by connective tissue stroma and lined with endothelium . Phleboliths identified in CT scans is due to calcified thrombi within these vascular spaces. The vascular spaces and channels bud into its surroundings, which results in tumor growth and thus leading to displacement and incorporation of the surrounding structures and tissue into the tumor. Lymphangioma may show similar histopathologic features as those seen in cavernous hemangioma.
The etiology of cavernous hemangioma is still being studied by researchers in order to understand more about the genes linked to the development of the condition. At the moment, it is not possible to prevent the condition. However, education and familial counseling are used to educate patients and families affected by the condition.
Cavernous hemangioma is the most common intraorbital tumor in adults. Patients usually complain about painless protrusion of the eye, which grows slowly. It belongs to a wide spectrum of hamartomas . The tumor may occur in any part along the neuroaxis  ; however, the most common locations are the cerebellum, cerebral hemisphere and the brainstem, and it is most commonly found in supratentorial compartments .
There are two forms of cavernous hemangioma, familial and sporadic. Sporadic cases are more common than the familial ones, accounting for about 90% of the cases .
The incidence of the tumor is almost equal between both genders; however, some studies suggest that the incidence is slightly higher among women.
The symptoms and features of the tumor develop very slowly starting with painless protrusion or bulging of the globe, accompanied by a feeling of fullness in the eyelid and around the eye. Vision acuity might be affected by the tumor. Hyperopia and diplopia may develop as a result of mass effect and compression of the tumor on the surrounding and adjacent structures and tissues.
The tumor is benign and usually has good prognosis without significant complications. However, there might be some complications due to the mass effect and the compression of the tumor on the adjacent structures, such as changes in vision acuity. Prognosis after surgical excision of the tumor is good and there is almost no risk of recurrence, especially if the tumor was removed completely.
Different workup tools may be used in the diagnosis of cavernous hemangioma, such as computed tomography (CT) scans, magnetic resonance imagingn (MRI), and ultrasound studies. Angiography may be used as well; however, it is not mandatory for the diagnosis.
Cavernous hemangioma is a benign tumor and it usually requires no treatment other than observation and followup. However, if mass effect symptoms such as changes in vision acuity develop, then surgical intervention may be needed in order to excise the tumor and relieve the compression caused by the tumor. In the past, radiotherapy was used as a treatment for cavernous hemangioma; however, it is not used anymore due to its complications.
Prevention of cavernous hemangioma is not easy and researchers are still studying the related genes. However, counselling and education for affected families may be helpful in raising awareness about the tumor and the possible complications.
Cavernous hemangioma is a benign tumor, which usually affects women more than men and presents around the age of 40 years. It is a collection of spaces filled with blood and usually develops intraorbitally, which means around the globe of the eye.
There are two types of cavernous hemangioma, the most common type is sporadic, which has no previous familial history. The second type is familial, which is associated with familial genes and usually runs in families.
Patients who have this tumor may not be aware of the condition because it is painless. The main symptom that patients may notice is a protrusion in the globe or around the eye, which grows slowly and compresses the adjacent structures and tissues. This may lead to other symptoms like vision problems or the feel of fullness in the globe of the eye.
Usually, cavernous hemangioma does not require any treatment or intervention. However, if symptoms like vision problems develop, surgical intervention may be considered as an option to relieve these symptoms and prevent other complications that may happen due to the compression of the tumor on the surrounding structures. Surgical intervention mainly involves complete excision of the tumor, which is done using different approaches, depending on the location of the tumor.
Cavernous hemangioma is a benign tumor and the outcome is very good. Patients who have the tumor can live a normal life without removing it. However, if surgery becomes necessary, the tumor is removed completely, resulting in almost no risk of recurrence.
It is hard to prevent the development of cavernous hemangioma because only little is known about the genes related to the condition. However, counselling and education for patients and their families may be useful to raise awareness about the condition, the outcomes, and the possible complications.
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