Partial skull removal, used to relieve life-threatening pressure in the brain after a traumatic injury or severe stroke, raises questions about quantity versus quality of life

July 16, 2013

Skull Surgery Offers Perils and Potential

By KATIE HAFNER

Dr. Geoffrey T. Manley of San Francisco General Hospital held a cranial prosthesis to be fitted in a patient who suffered traumatic brain injury after falling from a window.

SAN FRANCISCO — Following the crash of Asiana Airlines Flight 214 here, one of the first victims rushed to San Francisco General Hospital and Trauma Center was a teenage girl, unconscious and gravely injured. Her brain was quickly swelling, with nowhere to go but through the small opening at the base of her skull. Such an event, known as “herniation,” crushes the brainstem and can be rapidly fatal. Unable to reduce the swelling with medications, neurosurgeons decided to remove a large portion of the girl’s skull. Once they had done so, her brain bulged through the opening. The operation relieved the pressure and saved her brain, but it was not enough to save her life. The girl, whose parents asked that she not be named to protect her privacy, died of the other injuries she sustained in the crash.The operation, called decompressive craniectomy, is a remarkable but controversial feat, increasingly used to treat victims of head trauma who once might not have been saved. Malala Yousafzai, the 16-year-old Pakistani schoolgirl targeted by the Taliban, and Gabrielle Giffords, the former Democratic congresswoman from Arizona, each underwent decompressive craniectomies after being shot in the head. Senator Mark Kirk, Republican of Illinois, had the procedure a year ago after suffering a severe stroke. He returned to work in January.

The brutality of the procedure vividly illustrates the adage that surgery is barbarism with a purpose. But decompressive craniectomy also raises difficult questions regarding trade-offs between quantity and quality of life. Despite many successful recoveries, some remarkable, significant numbers of patients who receive the operation die, or are left profoundly disabled. Some are minimally responsive, with no cognitive function; others are severely disabled with impaired cognitive and motor function, but can communicate.

“All of us have seen miracles in people we’ve done this on, but the truth is we’re also probably creating a larger population of patients who are significantly disabled,” said Dr. Karin M. Muraszko, the chairwoman of the neurosurgery department at the University of Michigan.

It is difficult for surgeons to know which patients might recover and which are likely to be left barely functional. But the decision must be made under unyielding time pressure, in emergency rooms and intensive-care units and battlefield hospitals.

“We don’t want to save lives if we’re saving people to a state where they can’t function,” said Dr. S. Andrew Josephson, a neurologist and the chairman of the ethics committee at the University of California San Francisco Medical Center.

Skull removal to address cerebral swelling for traumatic brain injury and severe stroke first became widespread in the 1970s. Over the years, surgeons have refined the technique to the point where death is averted in about half the cases.

In the past decade, the operation, also known as hemicraniectomy, has grown more common for injured soldiers as military neurosurgeons have moved their operating theaters closer to the battlefield. “Hemicraniectomy is a game changer for how we handle those combat casualties,” said Dr. Rocco A. Armonda, a neurosurgeon at Washington Hospital Center and Georgetown University Hospital.

Dr. Armonda, a retired colonel, was part of the first neurosurgery team on the battlefield in Iraq in 2003, performing what he calls “neuro-rescue.” “Hemicraniectomy is now a standard element of that resuscitation,” he said.

The surgery is no doubt macabre. Even experienced surgeons speak of it with a sense of wonder.

Once part of the skull is removed, it can remain off for several months, or however long it takes for the swelling to subside completely. The bone is stored in a freezer or sewn into the patient’s abdomen for safekeeping. If the skull is too damaged to preserve, a prosthesis is fitted.

Yet the uncertainty of the eventual outcome continues to give physicians pause.

“I’d say our enthusiasm peaked around 2008 or 2009,” said Dr. Geoffrey T. Manley, one of the neurosurgeons who performed the procedure on the injured airline passenger here last week. “Our exuberance was tempered by our complication rate, and we started looking at the procedure more critically.”

Yet Dr. Manley said he did not regret performing the surgery on the young passenger: “I believe we have to give everybody a chance.”

In 2011, The New England Journal of Medicine published results of a randomized trial that compared decompressive craniectomy with the best drug therapy in patients with traumatic brain injury and diffuse swelling who underwent the surgery within 72 hours of their injury. The study found that patients who had the surgery and survived were more severely disabled than those who received standard treatments. There was no difference in the mortality rate between the two groups.

“What we need to work out better with more trials and research is, ‘Can we predict the patients who will do well with craniectomy and those who won’t?’” said Dr. Jeffrey V. Rosenfeld, a neurosurgeon at the Alfred Hospital in Melbourne, Australia, and an author of the study. “We have a rough idea, but we still get surprises. We can do a beautiful craniectomy and a patient still ends up very disabled.

“We received an incredible amount of heat and criticism,” Dr. Rosenfeld added. “But we weren’t saying it should be abandoned. It’s not a black-and-white situation.” He said each case should be judged individually.

Just as much uncertainty swirls around the use of hemicraniectomies after a stroke.

About 700,000 people in the United States suffer ischemic strokes each year, and about 1,000 of those patients develop swelling so severe that skull removal is performed, according to unpublished data collected by a team of researchers led by Dr. Kevin N. Sheth, a neurologist at Yale.

Even if a life is saved, the part of the brain that is destroyed by the stroke will not recover. “No one gets away with no or very little disability from a huge hemispheric stroke,” said Dr. Neil Schwartz, a neurologist at Stanford.

In patients with major strokes, certain factors drive surgeons to favor hemicraniectomy. Early intervention is better than waiting, and removing a large segment of skull offers the highest chance of recovery.

Physicians are more hesitant to offer the surgery to patients with severe strokes involving the left side of the brain, where language ability resides.

In a systematic review of studies of hemicraniectomy for large strokes published last year in The Journal of Neurosurgery, the authors concluded that, despite leaving a significant fraction of patients with moderate or severe disability, many hemicraniectomy patients emerge with high levels of function and reasonable qualities of life.

Notably, the average age of the patients in the studies was 50, young for the average stroke victim. Indeed, many of the patients who receive the operation are young because younger patients are at higher risk for life-threatening brain swelling after a stroke. And physicians have found that patients under 60 have an increased likelihood of a good outcome.

Spencer Nuttall was just 18 when he had a huge stroke while working out at a gym in Taunton, Mass. His mother, Donna, consented to a hemicranectomy in a haze of panic and confusion.

“I just remember them explaining they would take part of his skull off,” Ms. Nuttall said. So you say, ‘O.K., sure,’ but you don’t really get it.”

Mr. Nuttall, now 23, lost the function of nearly two-thirds of the right side of his brain from the stroke. He walks with a cane but has recovered well. A senior at Bridgewater State University in Bridgewater, Mass., he plans to go to graduate school to study speech pathology.

“Spencer was very young, and I felt we could really help him,” said Dr. Clark Chen, who performed the surgery. “But in a lot of situations, it’s not so clear.”

Dr. Chen, now at the University of California, San Diego, said he recently conducted an informal poll among his surgical residents and the faculty, all of whom had experience in the care of craniectomy patients. He asked whether they would choose to have a decompressive craniectomy to save their lives after a severe stroke.

Half said they would not.

Dr. Chen said he would not be able to perform his job as a neurosurgeon were he to suffer a severe stroke, and his own advance directive specifies that a craniectomy should not be performed on him.