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For
many years, tumors within the skull-base's complicated,
bony anatomy were difficult for surgeons to access safely.
Patients with skull-base tumors, such as schwannomas,
often had a poor prognosis. Advances in both technology
and microsurgical techniques have dramatically increased
surgeons' ability to remove as much of a skull-base
tumor as possible.
When Danielle Weedo of
Belleville, New Jersey, was 20, her long-held fear was
confirmed: She, like her mother, her sister, and 11
other relatives, has neurofibromatosis. NF, an autosomal
dominant genetic condition, causes tumors to develop
along the body’s nerves. In Ms. Weedo’s
case, a large, benign tumor—a schwannoma—was
growing in an unusual and complex area: the skull-base.
The skull-base, upon
which the brain’s undersurface rests, has three
main regions. Located above the eyes, the anterior region
includes the olfactory bulbs, the nasal cavity, and
cranial nerves that control movement of the eyeballs.
The petrous internal carotid artery and sections of
the cranial nerve, involved with chewing and facial
sensation, travel through the middle region; it is also
where the cavernous sinus, an extremely difficult structure
from which to remove tumors, is located. The posterior
region is where the auditory canal and the cranial nerve
responsible for hearing are located; the jugular vein
also passes through this region.
For many years, tumors
within the skull-base’s complicated, vital and
bony anatomy were difficult for surgeons to access safely.
Patients with skull-base tumors often had a poor prognosis.
Advances in both technology and microsurgical techniques
have dramatically increased surgeons’ ability
to remove as much of a skull-base tumor as possible.
The technology available to neurosurgeons such as Dr.
Michael Schulder, an associate professor of neurosurgery
at New Jersey Medical School and Director of Image-Guided
Neurosurgery at NJMS/University Hospital, is impressive.
The ability to create three-dimensional, computer-generated
images of the brain and to map the patient’s brain
and its unique “eloquent” areas with functional
MRI have revolutionized the way brain surgeries are
approached. Intraoperative capabilities, whether image-guided
surgery or MRI, offer valuable information to the surgeon
in “real time.” Intraoperative MRI, for
example, can be used to ensure that surgical goals are
reached.
However, the best technology
without a skilled team utilizing the best techniques
is useless, even harmful. “Unquestionably, the
technology is getting better,” says Dr. Schulder.
“But technique is knowing how to use these tools.”
In addition to technology
and technique, there’s a third “T”
that has evolved in the realm of skull-base procedures:
teamwork. The multi-disciplinary skull-base team—which
includes specialists from neurosurgery, otolaryngology,
ophthalmology, interventional radiology, radiation oncology,
and plastic surgery—offers patients the best of
care for these very difficult cases.
The Power of
Teamwork
The skull-base team at
University Hospital made a world of difference to Ms.
Weedo. Although her tumor was benign, it was causing
a great deal of facial fullness and compressing her
right eye downward (Figure 1). “I could feel the
tumor inside my face,” says Ms. Weedo, now 23.
“I knew there was a problem, but considering my
family history and knowing what my relatives had been
through, I put off seeking treatment. When I finally
went to doctors in New York, I was told that my case
was ‘out of their league.’”
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Figure 1
Skull base tumor pressing on the right eye. |
Another
doctor recommended that Ms. Weedo see the skull-base
team of physicians at University Hospital. After a thorough
evaluation and consultation with them, Ms. Weedo received
a different perspective: They believed that at least
a portion of the large tumor, which had snaked its way
up from the nasal cavity, behind her right eye, and
around a facial nerve, could be removed. One of the
major risks, however, was facial paralysis. It was a
risk Ms. Weedo was willing to take.
Breaking Barriers
Among the 100,000 or
so brain tumors diagnosed each year, about 5,000 fall
into the category of skull-base tumors. Meningiomas,
tumors originating from the thin membranes that cover
the brain and the spinal cord; pituitary adenomas, tumors
of the pituitary gland; and craniopharyngiomas, tumors
near the brain’s pituitary gland, are three types
of skull-base tumors regularly seen at University Hospital.
While these tumors are benign and slow growing, they
can present significant problems: a pituitary adenoma,
for example, can cause overproduction of hormones. Excess
hormones can lead to a variety of conditions depending
on which hormone is being overproduced. Cushing’s
syndrome, for example, is associated with overproduction
of adrenocorticotropin, or ACTH, and can cause obesity,
high blood sugar, fatigue, and in women, irregular menstrual
periods.
The concept of a skull-base
team knocks down some of the barriers that specialists
working individually face. “Neurosurgeons are
used to operating in a totally sterile environment within
the brain. When a procedure crosses over to such areas
as the nose, the mouth, the external ear, and the eye,
which are not totally sterile, it creates both frustration
and concern,” says Dr. Schulder. “Similarly,
if otolaryngologists and ophthalmologists expose the
brain and inadvertently some spinal fluid escapes, they
have to reach out to a neurosurgeon on an emergency
basis or hope (usually in vain) that the matter resolves
itself. Many nerves and vital blood vessels also traverse
the skull-base, and their dissection and preservation
is crucial in ensuring a successful outcome for the
patient. Combining the expertise of several specialists
overcomes the difficulties of crossing these boundaries.”
As Dr.
Soly Baredes, associate professor of surgery and
chief of the division of otolaryngology–head and
neck surgery—at New Jersey Medical School, succinctly
explains, “Tumors in the skull-base region don’t
respect a specialist’s turf.”
University Hospital is
home to New Jersey’s largest head and neck cancer
program, and it’s not unusual for these tumors—by
virtue of their location—to require a multi-disciplinary
approach. “We encounter a broad gamut of lesions,
such as squamous cell carcinomas, salivary gland tumors,
or even large skin tumors that work their way down into
the nose, that are best handled by involving specialists
from neurosurgery and/or ophthalmology,” says
Dr. Baredes. From an ears, nose, and throat specialist’s
perspective, tumors within the skull-base can cause
significant problems, affecting a patient’s ability
to speak or swallow; proper nasal respiration; or critical
sensory nerves.
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Dr. Paul Langer |
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Dr. Roger Turbin |
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Similarly, a high percentage
of skull-base tumors involve the eye, and a change in
vision is often the first concern that causes patients
to seek treatment. The expertise of two ophthalmalic
sub-specialists at University Hospital, Dr.
Roger Turbin and Dr.
Paul Langer, frequently are called on when a patient’s
skull-base lesion directly or potentially impacts vision.
Both assistant professors of ophthalmology at New Jersey
Medical School, Dr. Turbin is a neuro-ophthalmologist
and orbital surgeon, and Dr. Langer is a specialist
in ophthalmic plastic surgery and orbital surgery.
“Although we aren’t
involved in every skull-base surgery, ophthalmology
has an important role in the medical decision-making
that occurs in these cases,” says Dr. Turbin.
“There are many important visual components that
travel through the skull-base, controlling sensory vision
and eye movement.”
“Skull-base patients
often need some type of ocular reconstructive surgery,
such as repair to the eyelids or the tear ducts, or
reconstruction of the bones of the orbit [the bony socket
that surrounds the eye]. We try to do as much reconstructive
work as we can at the time of the tumor removal, but
sometimes further surgery is needed,” says Dr.
Langer. “We try, as much as possible, to perform
these procedures with aesthetic considerations, placing
incisions in non-obvious places. Even when it is necessary
for the eye itself to be removed, an artificial eye
can be created that closely resembles the other eye.”
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Dr. Ramazi
O. Datiashvili |
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Dr.
Ramazi O. Datiashvili, an assistant professor of
surgery at New Jersey Medical School and a plastic surgeon,
is another member of the University Hospital skull-base
team. His expertise in free-flap reconstruction, an
intricate, microvascular technique, is invaluable for
healing on many levels. “Once a tumor has been
removed from the skull-base, large, deep wounds remain
that expose vital structures such as the brain, the
sinuses, soft tissues, bones, and muscles. To be protected,
these structures need to be covered with well-vascularized
tissues,” says Dr. Datiashvili.
During free-flap reconstruction,
the plastic surgeon takes tissue from another part of
the patient’s body—typically the back, abdomen,
or forearm—and connects tiny vessels, providing
blood supply to those tissues in the area left open
by the tumor’s removal. Connecting tiny, often
fragile blood vessels under a microscope and working
through tissue that in some cases is inflamed due to
previous radiation therapy is time consuming and meticulous
work. And while these surgeries are life saving in nature,
Dr. Datiashvili, like other members of University Hospital’s
skull-base team, has the patient’s quality of
life in mind. “My number one goal is to help the
patient to have a satisfactory life, and part of that
is covering the wound and protecting vital structures,”
he says. “Another part is making surgical decisions
that, to the best of my ability, preserve the patient’s
appearance.”
A New Concept
Just as the concept of
a skull-base team has evolved over the past couple of
decades, so has the physicians’ philosophy toward
removing tumors and how such procedures affect patients’
ability to function. “Today, most skull-base teams
are less willing to sacrifice the patient’s speech
and swallowing abilities, vision, or other important
functions for the quixotic goal of complete tumor removal,”
says Dr. Schulder. “There are other options, such
as stereotactic radiosurgery and chemotherapy, that
can be used to pursue remnants of tumors that cannot
be safely accessed by the neurosurgeon.”
The desire to preserve
function in skull-base patients raises questions that
weren’t necessarily asked 20 years ago. “Consider
the patient who has a benign tumor in the cavernous
sinus,” says Dr. Turbin, the neuro-ophthalmologist.
“Without surgery, there’s a chance that
the tumor could cause double vision. With the surgery,
double vision is almost certain. What’s the best
choice? If I were the patient, I’d want the tumor
to be watched and retain my normal vision for as long
as possible.”
The composition of a
skull-base team varies by the nature and location of
the tumor. At University Hospital, any specialist can
refer a patient to other members of the team, and the
person meets individually with each physician prior
to treatment.
Ms. Weedo’s complicated
case required the expertise of several members of University
Hospital’s skull-base team. From a pre-operative
MRI, her team determined that arterial embolization,
a non-invasive method of blocking arteries, would be
needed to help control bleeding during the surgery.
Dr. Jeffrey Farkas, University Hospital’s chief
of interventional neuroradiology and an assistant professor
of radiology at New Jersey Medical School, compares
embolization to “shutting off the water before
repairing a leaky pipe.”
“Embolization can
be necessary for two main reasons,” Dr. Farkas
says. “These tumors can be very vascular, with
numerous tiny, fragile vessels leading into them. Or,
there can be a major blood vessel leading to the brain
that is surrounded by or feeding into the tumor. Even
a small amount of blood, when magnified 50 or 60 times,
can be a big problem for a neurosurgeon, and the last
thing he needs is to stop the tumor resection to control
bleeding.”
Not every skull-base
surgery patient needs embolization. For those who do,
Dr. Farkas wants to ensure that these patients can withstand
having blood vessels blocked off. In one of the worst-case
scenarios for a skull-base team, the patient can’t
withstand the blockage during the operation, throws
a blood clot and has a stroke. So, under carefully monitored
conditions prior to embolization, Dr. Farkas temporarily
blocks blood flow with a balloon and gauges the patient’s
physiological response.
The day after Ms. Weedo’s
embolization, she underwent an 18-hour operation (figure
2) to remove the tumor. Dr. Schulder lifted Ms. Weedo’s
brain to access the right side of her face, and Dr.
Baredes, the otolaryngologist, used what’s known
as a facial degloving approach—an incision under
the lip and nose—to gain access to the tumor.
“Not only did this help preserve facial nerve
function, this technique did not require an incision
on her face,” says Dr. Baredes. The tumor had
also invaded the orbit, destroying the entire bone underneath
Ms Weedo’s right eye. Dr. Langer removed this
portion of the tumor, then reconstructed the entire
floor of the right orbit to support the eye, preventing
it from falling below the level of her other, normal
eye.
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Figure 2
Skull base surgical approach for tumor
removal, allowing for neurosurgeon, ENT surgeon,
and eye surgeon to combine efforts at one operation. |
Most of the tumor—a
mass about the size of an orange—was removed.
The outcome was successful, with no facial paralysis.
“They saved my life,” says Ms. Weedo of
the skull-base team. “And to look at me, you would
never know that I had brain surgery.” Although
the tumor cost Ms. Weedo vision in her right eye (“I
waited too long to see a doctor. It had nothing to do
with the surgery.”), she gained what the doctors
hoped for: removal of the tumor and full facial function
without damaging her appearance.
A Long Road
Due to the nature of
neurofibromatosis, Ms. Weedo’s first surgery at
University Hospital has not been her last. Her body
keeps producing tumors. In September 2003, Dr. Schulder
removed a meningioma from Ms. Weedo’s brain, and
currently, she has two acoustic neuromas. She views
her condition with a mixture of sadness, resignation,
and humor. “I’m getting tired of these terrible
haircuts. Every time my hair grows back, it’s
time for another shave,” she jokes, then turns
serious. “I’m the 14th person in my family
to have NF, and I’ve seen what they’ve been
through. I hate to do it, but when I start to experience
symptoms from my latest acoustic neuromas, I’ll
be back at University Hospital to have them removed,
too.”
To arrange
for a consultation with Dr. Soly Baredes, call (973)
226-3444.
To arrange
for a consultation with Dr. Ramazi O. Datiashvili, call
(973) 972-8071.
To arrange
for a consultation with Dr. Jeffrey Farkas, call (973)
972-6624.
To arrange
for a consultation with Dr. Paul Langer or Dr. Roger
Turbin, call (973) 972-2065.
To arrange
for a consultation with Dr. Michael Schulder, call (973)
972-2323.
The patient
profiled in this Healthlink feature, Ms. Danielle Weedo,
has offered her email address for those wishing to contact
her for information and support. Ms. Weedo can be reached
by emailing: Gypsygrl450@aol.com.
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