Brain AVMs during pregnancy

An AVM (arteriovenous malformation) of the brain is an abnormal tangle of blood vessels in the brain connecting the arteries to the veins. The tangle of abnormal blood vessels is called a 'nidus'. If the arteries and veins are connected one-to-one without the intervening tangle of blood vessels, it is called a fistula. Because these blood vessels are abnormal, they have a tendency to rupture leading to bleeding within the brain. AVMs are not unique to the brain and can occur in other parts of the body. However, brain AVMs are unique in that they cause severe devastating effects if they rupture.

Brain AVMs diagnosed during pregnancy present a challenge to the neurosurgeon as well as to the obstetrician. The normal changes in the mother during pregnancy may predispose to rupture of the AVM, thus causing brain bleed. If untreated, the AVM runs the risk of bleeding and at the same time, treatment of the AVM may itself pose significant risk to the mother and the fetus. The overall risk of AVM rupture during pregnancy is about 1 in 100,000 deliveries.

What precautions should be taken in a pregnant mother diagnosed with brain AVM

• The highest risk of rupture of an AVM is in the second trimester due to the normal pregnancy related changes in the mother.
• The risk of AVM rupture does not increase during labor and delivery and hence, normal delivery should be encouraged
• As the changes occurring in the mother do not immediately revert following delivery, the risk of AVM rupture remains high immediately following delivery
• If the mother presents with seizures, due consideration should be given to the anti-seizure drug as it may harm the fetus
• The symptoms of AVM rupture mimic other common symptoms such as pregnancy induced hypertension and hence a high index of suspicion is required to diagnose a brain AVM
• when a mother presents with severe headache, seizures, speech difficulty or weakness of arm or leg, brain imaging is warranted
• MRI is the preferred method of choice to diagnose brain AVM in pregnancy as it avoids the radiation risk
• Surgery or endovascular therapy for an AVM in a pregnant mother should be carefully thought and undertaken with the understanding all the associated risks such as that of anesthesia, blood loss and radiation. There are no guidelines for the management of brain AVMs during pregnancy
• Stresses associated with normal delivery should be minimized. Primarily, the straining involved in the second stage of labor should be addressed. A combination of an outlet forceps delivery and the use of epidural anesthesia is recommended as means to reduce the duration of labor.
• There is no clear evidence to recommend that women with unruptured AVMs not have children.
• On the other hand, if a woman presents with a history of an AVM rupture in the recent past (within a year), there is evidence to support the recommendation that the patient undergo treatment for the AVM

Brain aneurysms in pregnancy

Pregnancy is a unique phase in a woman's life. It is associated with normal changes in almost every body system that assist fetal survival as well as preparation for labor. High blood pressure (hypertension) is the most common medical problem encountered during pregnancy and complicates 2%-3% of pregnancies. Diagnosis or rupture of a brain aneurysm during pregnancy poses serious life-threatening risk to the mother and the baby. It is therefore imperative that all Obstetricians are aware of this entity so that a high index of suspicion is maintained when pregnant women present with acute headache. The present article discusses some of the common questions about brain aneurysms in pregnancy.

Are brain aneurysms common in pregnancy?

Brain aneurysms rarely present during pregnancy.The reported incidence of brain aneurysm rupture during pregnancy ranges from 3-11 per 100,000 pregnancies. Of all the women presenting with ruptured brain aneurysm during pregnancy, 35% eventually die. It also results in death of the fetus in 17% of the cases. Although brain aneurysms are rare in pregnancy, they account for 5%-12% of all maternal deaths during pregnancy.

Is the risk of brain aneurysm rupture higher in pregnant women than in the general population?

It is unclear whether the risk of aneurysm rupture is higher in pregnant women than in general population. Many studies from Europe have reported increased risk of aneurysm rupture during pregnancy. However, a recent study from the United States reported no evidence of increased risk. The critical periods during pregnancy that can be detrimental to the mother and the fetus are labor and delivery.

How are brain aneurysms managed in pregnant women?

The management of brain aneurysms in pregnant women is similar to that in general population. Additional aspects that should be considered are

Asymptomatic unruptured intracranial aneurysm in pregnancy can be managed conservatively with close monitoring with noninvasive imaging techniques such as magnetic resonance imaging. However, expanding or symptomatic unruptured aneurysms in pregnant women should be treated

Radiation exposure to the fetus from CT scan and angiography should be minimized. MRI is the preferred imaging modality. If CT scan and angiography are absolutely required, a lead shield should be used to protect the fetus from radiation.

There are no evidence-based recommendations for child birth in patients with unruptured brain aneurysm, and there is no evidence to suggest that maternal or fetal outcome is improved by cesarean delivery in comparison with closely supervised vaginal delivery. Some studies have recommended caesarean delivery to avoid the stress of labor and delivery.

All women with ruptured brain aneurysms should be treated to prevent rebleeding. Clipping avoids the risk of radiation injury, but puts the mother and the fetus under stress during anesthesia and open surgery. Endovascular coiling poses radiation risk to the fetus.

The importance of support groups in the management of stroke

Stroke, for most people, is a life-changing event in life. Day-to-day activities may seem major obstacles to the stroke survivors. Writing a letter, going to the market, talking to people over telephone and commuting to work may be difficult to perform. Besides affecting the survivor, the family members may also face difficulties ranging from financial burden, strained relationships, frustration, anger and guilt feeling towards the survivor. All these difficulties hinder rehabilitation and recovery of the survivor following stroke.

Support groups allow stroke survivors to help themselves by sharing their experiences with other survivors and learn from others. They also provide a source for emotional and psychological support to survivors who often feel lonely and left out. They also allow the family members and caregivers to share concerns and help each other and bring people together with a sense of common purpose. The survivors and family members often feel empowered because of the warmth, acceptance and emotional support provided by the support group. The stigma associated with disability is eliminated when the survivors connect with other survivors with similar disabilities and this creates a positive attitude to face the problems together. The support groups also help in fundraising to support people in need and further research in the management of stroke.

Some of the popular support groups are

• The American Heart Association (AHA) is one of the oldest, largest voluntary organization devoted to fighting cardiovascular diseases and stroke. Founded in 1924, AHA now includes more than 22.5 million volunteers and supporters working to eliminate these diseases. The AHA also funds innovative research, advocates for stronger public health policies and provide lifesaving tools and information to save and improve lives. The American Stroke Association was created as a division in 1997 to bring together the organization’s stroke-related activities. (http://www.strokeassociation.org/STROKEORG/)

• The United States National Stroke Association provides stroke education and programs to stroke survivors, caregivers, and the healthcare community. (http://www.stroke.org)

• The Aphasia and Stroke Association of India, a nonprofit service organization, is dedicated to increasing public awareness of Aphasia and Stroke. It is committed to promoting the care of individuals with stroke and/or aphasia through educating the public, training families, and assisting with the networking of affected individuals. It is also dedicated to increasing resources that will help improve the quality of life for people with stroke and/or aphasia. (http://www.aphasiastrokeindia.com/index.php)

• The Brain Aneurysm Foundation is a nonprofit organization solely dedicated to providing critical awareness, education, support and research funding to reduce the incidence of brain aneurysm ruptures. (http://www.bafound.org)

• The Aneurysm and AVM Foundation (TAAF) is a nonprofit organization dedicated to bettering the lives, support networks, and medical care of those affected by aneurysm and other vascular malformations of the brain. It is an all-volunteer organization run by survivors, caregivers, and medical professionals. (http://www.taafonline.org)

Rehabilitation after Stroke

Rehabilitation after Stroke is one of the most important phases in recovery after stroke. It is also one of the most neglected aspects of stroke care in India. Annual estimated stroke incidence is 135 to 145 per 100,000, with early death rates ranging from 27% to 41%. This corresponds to ~1.5 million people having a stroke each year, leading to a further 500,000 people, each year, living with stroke-related disability. Hence, every patient being treated for stroke should have access to rehabilitation services. 

The long term goal of rehabilitation is to improve function so that the stroke survivor can become as independent as possible. This can be achieved by regaining the lost function as well as retraining other areas of the brain to take up the lost function (brain plasticity).

When does rehabilitation begin?

Rehabilitation should begin as soon as the treating doctor determines that the patient is medically stable. Depending upon the severity of stroke, rehabilitation may be undertaken in 

• Rehabilitation unit in the hospital
• At home, through home health personnel
• Outpatient rehabilitation facilities
• Long term care facilities such as nursing home

What are the different components of rehabilitation after stroke?

• Psychological - Depression and anxiety are the most common sequelae after stroke. Timely consultation with a psychiatrist and a psychologist will prevent interference with recovery process.
• Cognitive retraining - The psychiatrist and psychologist can also help in improving communication, spatial awareness, concentration and memory.
• Physiotherapy helps in improving muscle strength and overcoming walking difficulties.
• Occupational therapy involves adapting your home or using equipment to make everyday activities easier, as well as trying to find alternative ways of carrying out tasks that the patients have problems with.
• Speech and Language therapist works with improving difficulties with speech and communication.
• Swallowing difficulty - Some patients may have difficulty swallowing and require a feeding tube to prevent aspiration of food into the lungs. In long term, the speech and language therapist also works with the patient in starting oral feeds in a way to prevent aspiration.
• visual problems such as not being able to see one half of the visual fields may require eye movement exercises and can be addressed by simple ways by the physiotherapist or the ophthalmologist.
• Bladder and bowel problems are not uncommon after stroke and can be effectively be treated with retraining exercises, medications, pelvic floor exercises and the use of special products for incontinence.
• Erectile dysfunction can be a problem after stroke and there are many ways to overcome it.
• Not being able to drive is another major problem that may prevent the stroke survivor from returning to work. It should be borne in mind that the patients should start driving only after obtaining clearance from the doctor. If needed, specific training should be sought.
• Changes should be made in the lifestyle to prevent further strokes.

The ATTEND (Family-Led Rehabilitation After Stroke In India) trial aims to determine whether stroke recovery at home given by a trained family member is an effective, affordable strategy for those with disabling stroke in India when compared to usual care. Considering the cultural and family structure in India, this trial if it is positive it holds promise for a major change in practice in stroke rehabilitation both in developed and developing countries.

Vein of Galen Malformation

Vein of Galen Malformation (VOGM) is a rare condition in which there is an abnormal malformation involving one of the important veins in the brain that returns blood to the heart.

What causes VOGM?

The exact cause of VOGM is unknown. It is seen most often in infants and children. It can be detected as early as during pregnancy during routine ultrasound examination. The condition is not inherited and is not transmitted in the family. Rarely, it can be seen in older individuals.

Signs and Symptoms

The abnormal communication between arteries and veins in the brain leads to increased blood flow into the veins and to the heart. All the symptoms are as a result of this increased blood flow. the most common signs and symptoms are

• heart failure, often in the first few days of life
• abnormally increased head size and circumference due to excessive fluid accumulation within the brain
• prominent veins on the veins and scalp
• developmental delay
• failure to thrive
• seizures
• headache
• rarely, brain bleed
• if untreated, the condition may be life threatening

Diagnosis

• the condition may be detected on antenatal ultrasound
• CT scan, MRI may be required if there is a suspicion of VOGM
• cerebral angiography is the definitive test to confirm the diagnosis

Treatment

• A Neurosurgeon and neurointerventionist should be consulted to assess the extent and location of VOGM and plan management
• A Cardiologist should be consulted to assess heart function
• The neonatologist or pediatrician takes care of general health of the child
• Embolization is performed to close the abnormal connections. The procedure involves inserting a catheter through one of the arteries in the groin and navigating all the way into the tiny abnormal connections. Coils (metal threads) and glue are used to close the abnormal connections.
• Multiple sessions of embolization are often needed to close all the abnormal connections
• Open surgery for VOGM is never required. However, shunt surgery may be required to drain the fluid from accumulating in the brain.

Outcome

It is often possible to cure the malformation. The extent of brain and heart damage at the time of diagnosis and treatment determines the outcome.

Support Group

VOGM Patents' Alliance http://vogmparents.org/

The Road to Recovery after Brain Aneurysm Treatment - Emotional Effects

"I know all about time and wounds healing, but even if I had all the time in the world, I still don’t know what to do with all this hurt right now.” 
                                                   ------------― Nina Guilbeau, Too Many Sisters

It is impossible for others to understand the suffering that a patient with an illness goes through simply because the experience is unique to the person. The life-changing experience that one goes through after brain aneurysm treatment puts the patient through great emotional stress. Even though no one may truly understand the emotional effects following brain aneurysm treatment, all patients should know that help and support is available. Maintaining a positive attitude, healthy diet and following doctor's advice will help the patient overcome the emotional sequelae.

Common emotional disturbances following brain aneurysm treatment are

Depression: Many brain aneurysm survivors suffer from depression. Symptoms include mood swings, feeling of sadness, anxiety, emptiness, hopelessness, decreased energy and fatigue, difficulty concentrating, forgetfulness, lack of sleep, decreased appetite, loss of interest in pleasure or activities including sex and suicidal thoughts. 

The patients need to openly share their feelings with someone close to them and also to the treating doctor. Consultation with a psychiatrist or a psychologist may help. The patients should understand that patience and time are required to overcome some of these sequelae. The most important thing that the patients and their families should understand and keep in mind that having a brain aneurysm is not the end of the road and that help is available to face the emotional and physical disturbances. 

Loss of Emotional Control: Many patients experience increased anger, sadness or apathy. The anger or frustration of not being able to go back to the pretreatment state can be difficult to understand. In these circumstances, counselling should be sought.

Loneliness: Many patients feel lonely as they do not have someone to share their suffering. In such circumstances, sharing their feelings with the doctor and seeking appropriate counselling often helps.

Sleep Disturbances: Many patients experience decreased or excessive sleep due to prolonged stay in the hospital or the ICU. In most circumstances, the treating doctor is able to help.

Decreased Self-esteem: Many patients feel less capable of doing their work. In many patients, it is the lack of right attitude rather than physical incapability. Appropriate and timely treatment by a psychiatrist or a psychologist is very helpful.

Family and financial issues: Disputes in the family or financial insecurity may crop up and play a major role in worsening the above mentioned emotional effects. The patient and his/her family should understand that having a brain aneurysm is not the end of the road and, with appropriate and timely treatment, the physical and emotional effects can be minimized.

The Road to Recovery after Brain Aneurysm Treatment - Physical Effects

Having a brain aneurysm and undergoing treatment is a life changing event. Recovery is more difficult and longer in older patients, following brain aneurysm rupture and chronic medical problems. Patients recovering after treatment of brain aneurysm often have physical, emotional and psychological issues. Besides the patient, the family also suffers from many issues, one of the most important being financial burden in Indian scenario. In the coming few posts, I will discuss the issues faced by patients and family and how to get over them

Neurological

• weakness of arm, leg
• speech problems
• memory problems
• visual problems
• loss of smell or taste
• difficulty walking or balance problems
• seizures
• fatigue and generalized weakness
• sleep problems
• back pain

Many of these problems gradually improve over time. Evaluation by a physiotherapist and occupational therapist is recommended in patients with arm or leg weakness and balance problems. Speech therapy can help many patients with difficulty understanding and speaking. Appropriate neuropsychological testing and therapy can help patients with memory and behavioral problems to cope with the frustration. Some of the common memory problems include absorbing, storing and recalling information. The memory problem may be from the bleed or stroke during the acute period after aneurysm rupture. Organizing one's daily activities and writing down information is helpful.

Patients with visual problems and seizures should be careful not to drive and should avoid working near heavy machines that can cause injury. evaluation by an ophthalmologist is advised for visual rehabilitation.

Fatigue and generalized weakness is a common complaint in many patients. The patients should understand that it is normal to be sleepy and not interested in outdoor activities for a few weeks. The bleeding in the brain takes a few weeks to resolve. Eat well and engage your mind in activities to keep you busy. Trouble sleeping in the night is also very common and should get better with time.

Back pain may occur in some patients due to lying in the bed for many days or if the bleeding extended into the spine. The headache may also be from spinal tap in some patients. The pain usually resolves in a few weeks as the patients involve themselves in activities.

Common problems following surgery for brain aneurysms are

• incision site pain and numbness - may patients complain of local pain at the site of incision in the scalp following surgery. Some may have numbness over the incision site. The pain and numbness usually goes away with time.
• jaw pain - many patients may have jaw pain on the side of surgery, especially while eating or chewing hard food. For this reason, it is advisable to eat soft food immediately after the surgery. The jaw pain occurs due to manipulation of muscles during surgery and resolves slowly after surgery in about a month.
• clicking sound in the head - some patients may notice a click in the head following surgery. It is also a normal phenomenon and usually resolves in a few weeks
• headache - some patients may have intermittent mild headache that disturbs their daily activity. The pain usually resolves with pain pills or by itself. If headache is severe, associated with vomiting or blurring of vision, consult your doctor immediately.

Common problems following coiling or stenting for brain aneurysms are

• groin pain - there may be bruising or pain in the groin for a few days after the procedure. Please consult your doctor immediately if there is sudden bleeding from the puncture site or painful swelling.
• hair loss - is seen in some patients secondary to radiation. It is more common when the procedures last longer than about 3 hours. The hair loss is temporary in most of the patients.
• headache - may occur in some patients and is usually due to the bleed that occurred following aneurysm rupture. it resolves gradually on its own.
• general bruises over the body - may occur in some patients who take blood thinner pills following stenting. Try not to get hurt or cut yourself inadvertently. If you notice a bad bruise, contact your doctor immediately.