June 27, 2022

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Cavernous hemangioma: Cavernous Vascular Malformation (CCM)

Cavernous hemangioma: Cavernous Vascular Malformation (CCM)

 

Cavernous hemangioma: Cavernous Vascular Malformation (CCM). Also known as: Cavernous Vascular Malformation (CCM), the departments where the treatment is designed: Neurology, Hepatobiliary Surgery, and Ophthalmology.

Cavernous hemangioma: Cavernous Vascular Malformation (CCM)

 

Overview

Cavernous abnormal vascular clusters composed of numerous thin-walled blood vessels. Cavernous hemangioma is not a tumor. It can occur in any part of the central nervous system, including the brain and spinal cord. It often manifests as spontaneous bleeding, headache, coma, and hemiplegia

 

Disease definition

Cavernous hemangioma refers to a cavernous abnormal blood vessel group composed of numerous thin-walled blood vessels. The appearance is purple-red, and the cross section is sponge-like or honeycomb-like. Pathological structure:

  • The blood vessel wall is composed of a single layer of endothelial cells, lacking a muscle layer or elastic layer;
  • The lumen is filled with blood;
  • There may be fresh or old blood clots.

There is loose fibrous knotted tissue between abnormal blood vessels, and there is no substantial tissue between them.

 

Epidemiology

Incidence

The population incidence rate is 0.16%~0.50%.

Prone crowd

There are more women than men.

 

Type of disease

Cerebral cavernous hemangioma

Cavernous hemangioma that occurs in the brain is a congenital malformation of cerebrovascular development. When the embryo is about 4mm in size, the embryo’s blood vessel development has been deviated, resulting in cavernous hemangioma. Cerebral cavernous hemangioma can be single or multiple. This unhealthy malformed blood vessel group is prone to spontaneous rupture and bleeding, leading to clinical symptoms, disability and even life-threatening.

Hepatic cavernous hemangioma

It is the most common benign liver tumor. May be related to congenital developmental abnormalities

Orbital cavernous hemangioma

Cavernous hemangioma in the orbit is the most common benign tumor that originates in the orbit. The tumor is pathologically a hamartoma, not a real tumor.



Causes of CCM:

Cavernous hemangioma mainly occurs in the skull, but can also occur outside the skull, such as the liver and orbits.

 

Basic cause

The exact cause of most cavernous malformations is not clear. Some cavernous hemangioma has the characteristics of family inheritance.

 


Symptoms

Cavernous hemangioma can be asymptomatic if it does not rupture and bleed. However, there is a tendency to spontaneously rupture and hemorrhage. After rupture and hemorrhage, different symptoms such as epilepsy, neurological dysfunction, and headache will occur depending on the location.

Some patients have mild symptoms, but severe cases can cause patients to become unconscious and even die.


Typical symptoms

Cerebral cavernous hemangioma

Seizures

Seizures are the most common symptom of cerebral cavernous hemangioma. There are many types of seizures, which are related to the location of the lesion. Cavernous hemangioma is easy to induce epilepsy. The reason may be that the hemorrhage of cavernous hemangioma may cause bad stimulation of adjacent brain tissue.

The brain tissue around the lesion often becomes epileptic foci due to hemosiderin deposition, glial hyperplasia or calcification.

Headache symptoms

Cavernous hemangioma usually has headache symptoms after bleeding.

Focal neurological deficit symptoms

It is closely related to the location of the lesion.

  • If cavernous hemangioma is located in the brain stem, symptoms of paralysis on one side and hemiplegia on the opposite side of the upper and lower limbs can occur;
  • People in the sports area can cause paralysis of the side or weakness of the upper and lower limbs after bleeding;
  • Those located in the visual cortex of the occipital lobe can cause visual field defects;
  • Those located in the cavernous sinus can cause symptoms such as facial pain, drooping eyelids, and tilted eyeballs.

Hepatic cavernous hemangioma

Most patients have no clinical symptoms and are often found during physical examination or B-ultrasound, CT examination and laparotomy. The tumor develops slowly and the course of the disease can be as long as decades.

When the tumor gradually enlarges and oppresses adjacent organs, symptoms such as upper abdominal distension and nausea, fullness after eating, and belching may occur.

Anemia, total number of white blood cells and thrombocytopenia may occur in some patients, which may be related to the formation of intratumoral thrombosis, which destroys red blood cells and consumes a large number of platelets.

When a giant cavernous hemangioma forms an arteriovenous fistula in the liver, it can cause congestive heart failure. Obstructive jaundice is rare, and pedicle torsion occurs occasionally.

In most cases, the mass can be palpable in the abdomen, the surface is smooth, the texture is soft or medium hardness, the pressure can be reduced, there is a sense of elasticity, no tenderness, and vascular murmurs can be heard.

Orbital cavernous hemangioma

Cavernous hemangioma in the orbit can cause exophthalmos.

 


Seek medical attention

If cerebral cavernous hemangioma has no bleeding and no symptoms, the patient has no way of knowing it. If you have a history of bleeding, or cause epileptic seizures or focal neurological deficits, you should seek medical attention promptly and you should consider treatment.

When visiting a doctor, the doctor may ask the following questions:

  • Have epileptic seizures?
  • Have headaches and how long does it last?
  • Whether there are symptoms such as facial paralysis, hemiplegia, visual field defects, drooping eyelids, and skew eyeballs?

Hepatic cavernous hemangioma and orbital cavernous hemangioma can basically be diagnosed by physicians based on typical symptoms and imaging examinations.

 

Specialist visiting

Neurology, Hepatobiliary Surgery, Ophthalmology.

 

Related inspections

Physical examination

Combine medical history and detailed physical examination to make a preliminary judgment.

Film degree exam

The most important and effective inspection method.

Cotton-like hemangioma of the brain: CT can be manifested as iso-density or slightly high-density lesions, and may also have calcification. Magnetic resonance is very important for the diagnosis of cavernous hemangioma. Cavernous hemangioma at different bleeding periods has a big difference in MRI. The most typical feature is the iron-containing blood yellow ring around the lesion. Some people are asymptomatic, and cavernous hemangioma can be found during a head CT or MRI physical examination.

Hepatic cavernous hemangioma: Ultrasonography is the most commonly used method to diagnose the disease. Small cavernous hemangioma are mostly hyperechoic nodules with clear boundaries, while giant spongy hemangioma appear as a mixture of hyperechoic and hypoechoic images.

Orbital cavernous hemangioma: Ultrasonography has a typical echo image, which is of qualitative diagnostic significance. CT has the significance of location diagnosis.

EEG examination

Patients with cavernous hemangioma who have seizures need an EEG examination.

 

Differential diagnosis

Cerebral cavernous hemangioma should be differentiated from brain tumors and cerebral arteriovenous malformations.

brain tumor

Some brain tumors are small in size and may also be accompanied by hemorrhage. They need to be differentiated from cavernous hemangioma. On plain CT scan, cavernous hemangioma showed high-density nodules without edema and space-occupying effect, and may have calcification, and hemorrhage is rare.
Magnetic resonance imaging (MRI) can accurately show the size and location of the lesion. T1WI shows a “popcorn”-like mixed signal, T2WI is a mixed signal dominated by high signal, and the surrounding “black ring sign” is hemosiderin deposits. Characteristic.

Cavernous hemangioma has no obvious enhancement in the enhanced MRI scan, and many tumors can show tumor enhancement signs in the enhanced scan.

Cerebral arteriovenous malformations

Cerebral arteriovenous malformation is also a congenital vascular malformation. It can also rupture and bleed, causing headache symptoms or seizures, or focal neurological deficits.

However, it is difficult to distinguish the two from the symptoms alone. But cerebral angiography is easy to identify.

Cavernous hemangioma is often not visualized on cerebral angiography, while arteriovenous malformations can see short circuits between arteries and veins and early development of veins. Pathological examination can also easily distinguish the two.

 

Treatments

Treatment of cerebral cavernous hemangioma:

Symptomatic treatment: Authors with recurrent epilepsy can give anti-epileptic treatment, and patients with hemiplegia need to prevent complications. For the treatment of cavernous hemangioma: It is generally advocated to completely remove surgery and strive for a radical cure. For asymptomatic people, it can be observed.

Treatment of hepatic cavernous hemangioma:

Including surgical and non-surgical treatments. It is determined according to the size, location, growth rate of the tumor and the patient’s physical condition. Since clinically rare malignant changes, if there are no clinical symptoms, no special treatment is required, and regular follow-ups can be performed.

Orbital cavernous hemangioma treatment:

The tumor grows slowly, and it is relatively possible to stop growing, and there is no malignant change. Therefore, if the tumor is small and has not caused clinical symptoms, close clinical observation is feasible. When there are obvious clinical symptoms, surgical resection is often the choice.

 

Acute treatment

In the acute stage of cerebral cavernous hemangioma hemorrhage, symptomatic treatment, hemostasis, dehydration and lowering of intracranial pressure can be treated; authors with epilepsy need anti-epileptic treatment. Surgical treatment is generally recommended afterwards.

 

General treatment

Mainly rest, symptomatic hemostasis treatment, prevention of complications, such as:

Patients with hemiplegia should pay attention to turning over and slapping their backs regularly to prevent pneumonia and prevent venous thrombosis. For those with dysphagia, an indwelling gastric tube, nasogastric feeding, and even tracheotomy are required.

 

Medical treatement

Due to large individual differences, there is no absolute best, fastest, and most effective medication. In addition to commonly used over-the-counter drugs, the most appropriate drug should be selected under the guidance of a doctor in full consideration of individual circumstances.

There is no effective drug treatment for cavernous hemangioma itself. For cerebral cavernous hemangioma:
If there are symptoms of intracranial hypertension, mannitol, glycerol and fructose can be used to reduce intracranial pressure. Authors of chronic epileptic seizures need anti-epileptic treatment. Patients with pneumonia need to use antibacterial drugs.

 

Surgical treatment

Cerebral cavernous hemangioma

For cerebral cavernous hemangioma, surgical resection is the main treatment method. The purpose is to eliminate the risk of focal bleeding, reduce or prevent seizures, and restore nerve function.

The cavernous hemangioma must be removed completely, otherwise there is a possibility of recurrence.

Hepatic cavernous hemangioma

Hepatic cavernous hemangioma is rarely malignant, so if there are no clinical symptoms, no special treatment is needed, and regular follow-ups can be performed. Indications for surgical resection of hepatic cavernous hemangioma:

  • There are obvious clinical symptoms that affect normal life and workers;
  • giant cavernous hemangioma with a diameter of >10cm;
  • patients with unclear diagnosis and malignant tumors cannot be ruled out;
  • hemangioma that grows fast and increases significantly in a short period of time;
  • Rupture and bleeding occur Possibly:
  • For those under 40 years of age, the tumor is more than 5cm, and there may be those who continue to grow.

Orbital cavernous hemangioma

When there are obvious clinical symptoms, surgical resection is more choice. Preoperative tumor location can be performed according to CT, and anterior or lateral orbital surgery can be performed.

 

Other treatments

Stereotactic radiosurgery treatment (gamma knife treatment) is an adjuvant treatment. For those who have symptoms and cannot undergo surgical resection, it can be considered.

 

Prognosis

Cavernous hemangioma is a vascular malformation, not a tumor, and the overall prognosis is good.

  • Cavernous hemangioma located in the non-functional area is expected to be cured without sequelae.
  • Cavernous hemangioma located in the functional area may have sequelae and even life-threatening.

 

Sequelae

It is closely related to the location of the lesion and the amount of bleeding.

  • Cavernous hemangioma located in the non-functional area is expected to be cured without sequelae. Cavernous hemangioma located in the functional area may have sequelae and even life-threatening.
  • Bleeding from cavernous hemangioma in the brainstem may cause death, be in a vegetative state, or be permanently disabled. Cavernous hemangioma located in the functional area can cause different neurological deficits after bleeding.
  • Bleeding from cavernous hemangioma in the spinal cord can cause paraplegia or hemiplegia, dysfunction of urine and urine, and limb sensory disorders.
  • Cavernous hemangioma located in the non-functional area can leave no sequelae.

 

Recovery

For those with neurological impairment, active hyperbaric oxygen, acupuncture and other neurological rehabilitation treatments are required.

 

Complication

During the treatment of cavernous hemangioma, complications such as pneumonia, deep vein thrombosis and bedsores can occur.

 

Relapse

Cavernous hemangioma is not a true tumor and does not metastasize. Cavernous hemangioma can be cured after complete resection. Cavernous hemangioma that is not completely resected may recur and re-bleed.

 

Daily care

The exact cause of most cavernous malformations is not clear. The daily life management of this disease focuses on preventing postoperative complications, regular follow-up, and promoting postoperative recovery.

Home care

If you have a cavernous hemangioma of the brain, you need more than just medical treatment. For patients with sequelae, daily care is also very important.

Family members should take care of patients patiently and actively help patients build confidence in overcoming the disease. If a cavernous hemangioma suffers from physical impairments, poor speech and other problems, and often appears irritable or pessimistic and disappointed, the family should often enlighten the patient to make the patient cheerful, dispel worries, and maintain an optimistic mood to help the condition get better.

Pay attention to diet and ensure adequate supply of calories. Eat more fresh vegetables, fruits and soy products. The second will always be unobstructed. Patients with language dysfunction and hemiplegia symptoms should strengthen language training and passively move the affected limb to promote functional recovery.

When the condition is stable, family members should help the patient with language training and passive movement of the affected limb as soon as possible to promote the patient’s early recovery of language and motor functions.

Observe the changes in the condition carefully. When you find that the patient’s mind, language, or the function of the affected limb is gradually deteriorating, you should promptly ask a doctor for treatment.

 

Daily life management

Good living habits

Maintain a good schedule, adequate sleep, avoid staying up late, and avoid smoking and drinking.

Stable mood

The patient must maintain a good mood and avoid bad moods such as depression and irritability. If you have a mood disorder, you can go to the psychiatric and psychology department for medical treatment.

Reasonable eating habits

In normal life, patients with cavernous hemangioma should strive to achieve a balanced and healthy nutrition. Minimize frying, greasy, spicy, and irritating foods.

Follow-up review

Asymptomatic patients, it is recommended to review once every 1 year; for surgical patients three months, six months, one year after surgery, and once a year thereafter. If you have any symptoms, you should consult a doctor at any time.

 

Special considerations


Patients with hemorrhage of cavernous hemangioma are likely to have symptoms such as limb hemiplegia and poor language. We must build confidence, strengthen language training and passively move the affected limb to promote functional recovery.

 

Prevention

At present, there is no effective preventive method for this disease.

 

 

 

 

 

(source:internet, reference only)


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