The Case Studies Series highlights Dr. Javahery’s hands-on experience as a practicing surgeon over the last 10 years and his most extraordinary cases, from patient diagnosis and treatment to their recovery and current condition.
This child was 9 years old when I first met her. She presented with progressive weakness on the left side, a weak voice, swallowing difficulties and impaired balance. Her imaging showed a lesion (tumor) in the posterior fossa compressing the brain stem.
Our initial impression was that this tumor was most consistent with a clival chordoma. We discussed the treatment options, and my plan was for a multi-staged approach for the resection of the lesion. I chose this treatment because I did not think it was possible to safely resect the entire lesion via a single surgery.
We began by doing an endoscopic endonasal approach to debulk the tumor and achieved about a 60 percent resection through this approach. Her pathology confirmed this tumor was indeed a chordoma. These tumors are benign, but they are extremely aggressive locally.
The best treatment for this type of tumor is what is called an en bloc resection, which means the tumor is removed with a cuff of surrounding normal tissue without ever actually seeing the tumor. However, this treatment is not possible when it is located in the clivus/skull base for two reasons. First, it is not technically possible, because it is impossible to get that wide of an exposure. It is also not possible neurologically, because you cannot remove all the neural elements and blood vessels that the tumor touches.
That is why for clival chordomas the goal is to remove all of the tumor, then treat the tumor bed with high doses of highly focused radiation — in this case proton beam therapy. Therefore, she needed more surgery in order to remove the remainder of the tumor.
She subsequently underwent a right-sided far lateral skull base craniotomy to approach the tumor from the lateral trajectory. We removed almost all of the residual tumor from this approach.
We did have to go after a few residual spots where the tumor was still visible on imaging. These included disease in the spinal canal on the left side, as well as disease in the petrous bone. She had a left-sided spinal approach from the base of the skull to the third cervical vertebrae to remove the left-sided spinal disease. For the tumor in her petrous bone, which was smaller than a centimeter in size, she underwent further endoscopic endonasal surgery.
We were able to achieve a gross total resection while avoiding any neurological deficits. She is now completely normal neurologically. Her pre-op weakness resolved soon after the endoscopic endonasal procedure. She did have transient worsening of her swallowing difficulty after the far lateral skull base craniotomy, but that resolved quickly as well.
She is now awaiting her proton beam therapy. I don’t know if her tumor will recur, but we are all very excited about how well she has done so far. I will post follow-up images on a delayed basis after her radiation treatment.
One of the most amazing patients I’ve treated was an incredibly brave 7-year old girl, who came in to the emergency room with her parents having extreme difficulties walking.
In addition to feeling numb in both of her legs, she had been experiencing chest pains at night for the past month. In search of answers, we ran emergency imaging and found a lesion on her T6 vertebrae with severe spinal cord compression.
This lesion was a cancerous form of Langerhans Cell Sarcoma. This is an incredibly rare disease with only 50 cases of it ever reported worldwide. Without quick treatment, this condition would leave this young girl paralyzed for the rest of her life.
The shaded area of the spine represents the lesion of the T6
This child was 10 years old when he initially presented to the hospital. He was with his grandmother when he began complaining of a headache, after which he lost consciousness. His grandmother called the paramedics but the sheriff got to the house first. The sheriff found the child not only unresponsive but also not breathing. According to the sheriff, he was purple. He initiated CPR and was able to get his pulse back. The sheriff saved him!
He was brought into the emergency room and was intubated. He had a CT scan of the brain that showed an intracranial hemorrhage. During the time in the ER, he arrested again and was brought back.
The ER called me after the CT scan on the way up to the Pediatric ICU.
I met him and his family in the ICU.
His CT head showed blood in the cerebellum with compression of the brain stem. There was also some hydrocephalus. His CT angiogram showed an associated arteriovenous malformation that had caused the hemorrhage.
This child was 3 months old when she presented to the emergency room with evidence of increased pressure inside of her head. She had rapid head growth and the soft spot at the top of her head (anterior fontanelle) was tense. Interestingly, she had multiple skin lesions that were consistent with cutaneous hemangiomas.
Based on her clinical condition, she underwent a CT scan of the brain, followed by an MRI of the brain. We could not do a contrast MRI since she was allergic to the contrast material for the MRI but we did a contrast CT and MRI without contrast. For a lot of tumors, it’s easier to see the tumors with the contrast dye but we had to proceed without it.
This patient was a 59 year-old when I initially met him. He presented with a 6 month history of left arm pain extending into the shoulder and upper arm. The pain did occasionally go down to his forearm. He had numbness around the left shoulder and mild weakness of the deltoid and biceps muscles. He had seen another spine surgeon who had recommended doing an anterior cervical discectomy and fusion between cervical 4 and 5 as well as between cervical 5 and 6 (ACDF of C4/5 and C5/6). He then saw me for a second opinion.
This patient was a 32 year-old with a history of multiple prior back surgeries. I met her in early 2017 when she presented with severe lower back pain and bilateral leg pain. Her pain was basically preventing her from a normal life. Walking any distance caused a rapid increase in her pain. Her leg pain went down the back of both legs and resulted in numbness in the feet.
This patient was the first case I ever did after completing my training. He was a 14 year-old high-school student on the local wrestling team. He was horsing around with a friend and the friend picked him up and dropped him on his head.
He was instantly unable to move his arms or legs.
He was brought in by the paramedics to the emergency room as a complete C4 quadriplegic. Complete means that he had no function (either motor or sensory) below the level of injury and being at the C4 level meant he had no muscle control in his arms or legs. He could only breathe spontaneously and move the muscles of his face and head.
This child is a 6 year-old who presented with progressive right-sided weakness. The weakness was initially subtle with some dragging of the right foot but it progressed to the point that he was falling and eventually could not walk
The weakness affected his face, arm, and leg. He was sent for an MRI and based on the results of the MRI, sent to me for an evaluation.
The MRI, unfortunately, showed a large tumor in the left side of his brain stem. The tumor extended from the midbrain (superiorly) to the pons (inferiorly).
Brain stem tumors in this age group can run the spectrum from very benign tumors to very malignant tumors. Some of these malignant brain stem tumors are so aggressive that they are invariably fatal.
This patient is a 19 year-old young woman who was involved in a high-speed motor vehicle crash. She suffered multiple fractures, including her leg and and her cervical spine (neck).
Fortunately, the fractures of her neck did not result in any neurological injury. The fracture pattern, however, was complex.
This patient was a 48-year-old woman who presented with a sudden transient loss of consciousness. When she regained consciousness, she had a severe headache. Fortunately, she was neurologically intact.
Her CT scan showed that she had an extensive subarachnoid hemorrhage (SAH). A SAH is bleeding inside the head into the subarachnoid space where cerebrospinal fluid circulates. This type of SAH is usually related to a cerebral aneurysm.
This patient was a 66 year-old women at the time of her initial presentation with Stage IV breast cancer and a new metastatic lesion in the 2nd lumbar vertebrae (L2).
She presented with new onset back pain about three months before I met her. She had radiation therapy for the lesion in L2 per the recommendation of her oncologist and another spine surgeon.
This case involves a previously normal child who, at 15 months old, presented with nystagmus, or abnormal eye movements. Her neurologist recommended she have an MRI, and the results showed large tumor in the posterior fossa compressing her brain stem.
This was one of the largest tumors I have ever seen. We initially did a biopsy since the lesion was so large and she was so young to see if there was a role for chemotherapy prior to surgery.
Another interesting case is this 18-month old child who presented with recurrent hemorrhages related to a cavernous malformation of her brain stem. Her parents discovered something was wrong when they noticed their child experiencing weakness on the left side of her body. They brought her into the emergency room for further evaluation.
This patient is a 66 year-old gentleman who previously had an L5-S1 instrumented fusion in 2007 (by me) for severe back pain and bilateral lower extremity pain. This pain was caused by an isthmic spondylolisthesis, a spinal condition in which one vertebra slips forward over the vertebra below, at L5/S1.
His symptoms were resolved after surgery but then in 2016 he developed recurrent back pain, leg pain, and foot numbness. His imaging showed that he had progressive spondylosis (degenerative changes) of the lumbar spine and adjacent level disease (from the prior fusion). He had significant spondylolisthesis at L4/5 and scoliosis in the lumbar spine.
This case is a 32 year-old woman who had persistent right sided headaches. She had a work-up done including an MRI of the brain. The MRI revealed that she had an arteriovenous malformation (AVM) of the right posterior temporal lobe going into the deep white matter. An arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system.
Arteriovenous malformation can be found anywhere arteries and veins operate but occur most often in the brain or spine. In general, brain AVMs are rare and affect less than 1 percent of the population.
They usually cause intense pain or bleeding and can lead to other serious medical problems like brain damage or stroke.