Chordoma surgery through the nose

A remarkable brain surgery at Saint John’s Health Center has restored vision to a seriously ill young woman from Spain—and the entire operation was performed through her nose without an incision.

“Tissue damage and scars left behind by the previous procedure made the surgery more complex as we had to work with fewer familiar anatomic landmarks”

The patient, referred to as Isabel R. for privacy reasons, came to the Saint John’s Neuroscience Institute in November 2009 with a rare medical problem: An aggressive tumor called a chordoma had wrapped itself around major arteries in her brain. This dangerous invasive tumor of the skull base was also adhering to the brain stem, pressing on the optic nerve, and damaging her pituitary—the master gland that controls hormonal function throughout the body.

“This is arguably one of the worst places to have a tumor growing,” said Amin Kassam, M.D., Medical Director of the Neuroscience Institute. “The area involved contains structures that control movement, vision, consciousness and nerve function.”

When she arrived at Saint John’s, Isabel had lost most of the vision in her left eye, was plagued by severe headaches and faced continued deterioration—even death—from progressive brainstem compression if her tumor could not be removed. After an unsuccessful attempt at removing the tumor, doctors in Spain had declared her condition inoperable. Even worse, the initial attempt at tumor removal had created a fluid leak requiring the placement of a permanent drain in her head and destroying healthy tissue that is normally needed for reconstruction.

The patient, a trained pharmacist whose father is a physician, had learned about the Neuroscience Institute via the Internet and by reviewing the medical literature for articles published by the surgical team at Saint John’s. With time running out, she came with her parents to Santa Monica in hopes that Saint John’s doctors could help.

The Neuroscience Institute specializes in minimally invasive brain surgeries that offer hope of a cure with reduced trauma and risk. Dr. Kassam and his colleague Daniel F. Kelly, M.D., chose the Expanded Endonasal Approach for their patient, an advanced strategy that provides surgical access to the brain through the nostril.

Together, Drs. Kassam and Kelly have more than two decades of combined experience pioneering endonasal surgery, and have used it on more than 2,000 patients. The procedure has many potential advantages over other techniques that require surgeons to make facial incisions, dismantle facial or orbital bones, or retract the brain. Instead, the endonasal route takes advantage of the natural nasal corridor to the base of the skull and the brain.

“Because it was not possible to treat my tumor in Spain, I wanted to find the best neurosurgeons in the world for this type of tumor,” the patient said. “With the help of my family, I found that the doctors with the most experience were Drs. Kassam and Kelly.”

The Expanded Endonasal Approach is one of several minimally invasive surgical procedures collectively called the 360º Approach that are practiced at the Institute to remove tumors of the brain, pituitary and skull base, and to treat other brain and cranial nerve disorders. In this approach, surgeons select the safest, most efficient corridor to the tumor, minimizing manipulation of nerves, blood vessels and the brain itself. Operations can be performed through a nostril, a small eyebrow incision, from behind the ear, or through a brain port device. This “keyhole” strategy can reduce complications and disfigurement while promoting a faster, less painful recovery.

Going through Isabel’s nose, the surgeons reached the tumor that was extending out from the skull base area into the nasal sinus area. Working between the two carotid arteries that supply the brain and repositioning one of them, a surgical “window” was created to allow tumor removal. To optimize this corridor, the pituitary gland that controls the hormone secretion was also moved and then returned to its normal position. This technique, referred to as “pituitary transposition,” was created by Dr. Kassam to access this hard-to-reach region of the brainstem. Using special instruments, they worked from the center of the tumor outwards, removing the majority of tumor without destroying critical structures surrounding it. The pressure that had been injuring the patient’s brain stem and optic nerve was relieved.

“Using the Expanded Endonasal Approach, we were able to access this very large skull base tumor through the nose, without facial incisions,” Dr. Kelly said. “We were also able to remove the spinal fluid shunt that had been placed there from her original operation in Spain.”

“Tissue damage and scars left behind by the previous procedure made the surgery more complex as we had to work with fewer familiar anatomic landmarks,” noted Chester Griffiths, M.D., a head and neck specialist and member of the Saint John’s minimally invasive skull base team.

According to Dr. Kelly, the patient recovered well. “She was awake and alert shortly after her surgery, eating and talking with her family,” he said.

At this time, Isabel’s vision has been restored and many of her other symptoms have already resolved. Meanwhile, she has no restrictions and can return to everyday activities.

“My recovery was very fast,” Isabel said. “In two days I was walking. Less than a month after surgery, I’m still feeling fine. I can lead a normal life, and I have practically regained all the vision in my left eye.”

Isabel has since returned to Spain, but will return to the Institute in March for further treatment. “Chordomas are complex, aggressive tumors that are rarely cured only by surgery,” Dr. Kassam said. “She may require a course of radiation to prevent further tumor growth.”

Still, the patient has a very positive opinion about her experience. “At Saint John’s I found a hospital where patients can receive treatment at the highest level,” Isabel said. “Dr. Kassam and Dr. Kelly are among the best neurosurgeons in the world, and I will be eternally grateful for their knowledge, support, kindness and compassion.”