Jack’s Instant Replay

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Our 8 year old son, Jack, recently suffered a football injury.  What was at first thought to be a whiplash injury to his neck, resulted in the discovery, by way of MRI, of syringomyelia.

Up until that day of the injury, our son had no symptoms that usually present themselves with syringomyelia.  On the day of the accident, he had tingling in his arms and feet.

We visited a Neurologist at McMaster Pediatrics who diagnosed our son with the syrinx and advised us to immediately remove him from any type of contact sport or sport that would cause a whipping action of the neck.

We wanted a second opinion since this was such a rare disorder.  We contacted Dr. Sutherland who suggested that we upload our MRI images to iRapidConsult for a second opinion.

We were amazed at the speed of the consult and the report we received after a review of the MRI. We were given much more information that helped us to understand our son’s condition.

Our son has Chiari 1 Malformation with secondary Syringomyelia.  He has a minimal Chiari Malformation of 6-7 mm.  He also has a small syrinx extending from C4-C7 measuring 2 mm by 2 mm.  He is currently asymptomatic.

Since our son is very interested in playing competitive sports, including hockey, this was devastating to him and very worrisome for us.  We have removed him from physical sports since there is not enough information to suggest that he would not get injured further.

Jack continues to go out with his hockey team, which is a Rep/competitive travel team, to practice but does not engage in any of the games.   He will be playing ball hockey and golf this summer.  It’s still important for him to be a child and have fun playing sports.  We’ll just suggest other alternatives to contact sports.

Jack will have an annual MRI in the absence of symptoms but should he experience headaches, arm numbness or tingling we will have him re-evaluated sooner.

We are very thankful for the services of Starr Tze, iRapidConsult and Dr. Sutherland and his team.  We certainly received a more detailed analysis of our son’s condition which allowed us to digest and comprehend this rare disorder.

Through Faith and Neurosurgery

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My decision to have neurosurgery was both an emotional and an analytical one.  As a physician in training, I spent hours reading about the various treatment options and was drawn to surgery by its definitive nature. In the end, though, it was confidence in my surgeon that made surgery right for me.

I had neurological symptoms since early childhood, but was not formally investigated until early on in my medical training.  It was horrifying to learn that the constant “whooshing” in my ears, left-sided weakness and intense headaches were in fact a Spetzler-Martin grade III arteriovenous malformation (AVM) nudging up on my left motor cortex.  I had to have an angiogram before my surgical consult and was blown away by the cluster of vessels that appeared as the dye ran through my brain.  At that moment, I knew that something would have to be done about the AVM.

In my first neurosurgical consult, we discussed the options: surgery, combination therapies and watchful waiting.  Embolization in combination with surgery was not ideal.  I knew that embolization could de-stabilize the lesion and be cumbersome for my surgeon.   Radiation, given the size of my AVM was not realistic.  For me, watchful waiting was impossible.  As an avid Nordic skier and hiker, I found myself increasingly worried on my long outdoor excursions and was too nervous to fly long distances for vacations.  Similarly, with every severe headache, I found myself wondering, “is this it, the fatal bleed?”

Treatment planning also involved some knowledge of the probabilities.  I knew that AVMs bleed spontaneously at a rate of roughly 2 to 4% per year.  If an AVM does bleed, it imparts a 50% morbidity/mortality.  I compared this to surgery, which imparted a 10-20% risk of hemiparesis.  Given my age, I chose surgery with its higher initial risk up front rather than a smaller risk accrued over the years.

My surgeon gave me and my family the confidence we needed to make the right decision for us.  At the time, I had not yet read the plethora of literature that my surgeon had authored, but I mustered the courage to ask him whether he was the best surgeon for the job, or whether I should travel to an “AVM center”.  He was not at all offended by my question and answered thoughtfully that he too had pondered this and discussed my case with colleagues.  I learned that there was no AVM-centre per se and that the post-operative care offered at our centre was very strong.  This gave me confidence to proceed with surgery.

Despite a long road to recovery, I do not regret my decision and have accepted the new challenges that life has offered me.

The Acoustic Neuroma Odyssey

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For several years, my patient complained to her physicians of a ringing sensation in her right ear. More recently, she also developed impaired balance and loss of hearing in the same ear. She was investigated with a hearing test that confirmed the hearing loss that was associated with nerve damage. Her MR brain images showed a small tumor located within the passageway by which the hearing, balance and facial nerves exit the skull. With the probable diagnosis of acoustic neuroma, she was brought to the operating room where the tumor was removed under general anesthesia, while preserving her facial and auditory nerves.

Patients with acoustic neuroma often present with decreased hearing on one side, ringing in the ear (tinnitus) and unsteady gait. The tumors vary in size, from small to large. Small tumors may reside predominantly in the passageway called the internal auditory meatus, while larger tumors are located in a space called the cerebellar pontine angle and may compress the brain. In addition to hearing loss and balance impairment, the tumor may result in facial numbness, and with brain stem compression, weakness. There are many treatment approaches to acoustic neuroma that include observation, surgical excision, and focused radiation therapy called radiosurgery. Radiosurgery is often the treatment of choice for older patients, patients with other medical problems, and those with small tumors. Surgery remains the best option for larger tumors and for younger individuals who have an otherwise long life expectancy.  The proximity of the tumor to the facial nerve highlights the importance of preserving facial nerve function during treatment, in particular should surgery be recommended. More recently, it has also become important to preserve hearing in patients whose hearing is otherwise functional.

The patient’s decision for surgery was based on the desire to have the tumor no longer part of her body. She was fearful of radiation and recognized the increased risk associated with surgery.

The Second Opinion Letter

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Over the past several decades, an increasing amount of medical information has overwhelmed both physicians and patients. Interpreting this information is not only complex for patients, but also for physicians providing treatment. It is becoming imperative for physicians to interpret and translate this information to patients in a meaningful way. It is only in this way that a patient can make an informed decision about their course of treatment. Course of treatment is not only important in the context of the disease, but also in relation to the patient’s own lifestyle and values. The premise of a second opinion is not only to further understand the medical problem but also to help people navigate through complex medical information towards the most ideal, personalized treatment option. This decision-making paradigm is based on an analysis of an individual in relation to ongoing advancements in medical science. Connectivity helps patients not only with their decision towards a treatment option, but also provide a support system for sharing disease burden amongst people with similar problems.

The Coma Awakening

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A 55 year old man was brought to the emergency department with decreasing level of consciousness such that he required intubation for mechanical ventilation. He underwent emergency CT brain imaging that showed a large blood clot associated with a brain tumor.  While being imaged, his condition deteriorated with progressive loss of neurological function. He was rushed to the operating room where through an opening in the right side of his skull, the blood clot and tumor were removed.  Following the procedure, he rapidly improved such that he no longer needed life support and was able to be transferred to the neurosurgical ward for further recovery.

Neurosurgical disease may be accompanied by a decreased level of consciousness. Loss of consciousness often relates to an increased pressure within the head, such as that from a blood clot either within or outside the brain. Surgical evacuation of the clot and repair of damaged blood vessels often results in normalization of pressure levels. Should the brain not have been severely damaged by the elevated pressure, patients often rapidly improve such that they are able to be taken off life support systems. Subsequent improvement may however be slow, often requiring management in a rehabilitation unit. Recovery may plateau after one year. Multiple strategies are now underway to accelerate recovery, including the use of robotic technology. Furthermore, multiple centers are experimenting with neuromodulating techniques, including the use of stimulators, or biological implants such as stem cells, to improve recovery.

In this particular case time was and remains the most important variable. Rapid decision making for interventions predominate any elaborate discussions with family on the treatment choices, although medical management needs to include an individual’s personal directive.

A Tale of Grade Two Glioma

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A 25 year old nurse presented to the emergency department with mild personality change and headaches.  CT and MR brain imaging studies showed a large left sided brain tumor. Functional brain imaging showed the relationship of the tumor to adjacent speech centers. Following discussions, the decision was made to perform the operation awake so that the speech centers could be defined and protected during removal of the tumor. The pathological examination showed a grade II astrocytoma.

A Grade II glioma is a tumor that arises from brain cells called astrocytes, oligodendrocytes, or ependymal cells. Patients often present with seizures that may be focal or generalized, with or without loss of consciousness. Less often, such tumors present with headache or personality change. A probable diagnosis is established with CT and MR brain imaging studies. Surgeons will often recommend surgical removal of the abnormality, as extensive resection has been shown to correlate with outcome. Following surgery, the majority of patients are observed with serial MR imaging studies performed at 6-12 month intervals. Unfortunately, many patients with Grade II Glioma will progress to Grade III or IV, necessitating additional surgery and other adjuncts such as radiation and chemotherapy. Investigators have shown that certain genetic characteristics, in particular 1p19q deletions correlate with improved outcome. Investigators around the world are busy further defining the genetic nature of this tumor, which will ultimately lead to improved intervention and outcome.

In this particular patient, the decision to perform the operation under local anesthesia allowed maximum tumor resection while decreasing the surgical risks related to speech.