Nov. 28, 2003 Bloody noses and shifting teeth. Those were the only signs of a tennis ball-sized tumor growing behind 13-year-old Zachary White’s face.
It was an oral surgeon who first recognized something was growing around the boy’s nasal cavity and immediately referred Zach to the University of Michigan Health System. Doctors here diagnosed a benign tumor called juvenile nasopharyngeal angiofibroma. It was growing in the back of Zach’s nasal cavity and had spread through the skull base to push against the covering of his brain.
Traditionally, this type of tumor would have been removed by actually lifting up the brain to reach down beyond it. Lifting the brain, though, carries a high risk of permanent brain damage.
But surgeons at UMHS are using a technique to remove tumors like Zach’s without lifting the brain – thereby limiting the risk of brain damage, but also preventing any noticeable scars or damage to the patient’s sense of smell. With this technique, called cranial base surgery, doctors make an incision across the top of the head, peel the face down and cut through the bones of the forehead, cheek and jaw to access the tumor without touching the brain.
“The tumors we would approach with cranial base techniques are basically at the base of the brain, along the base of the skull, and are deep and difficult to get to. Typically they’re not malignant tumors, but sometimes they are. They’re tumors that are surgically treatable, but which without modern skull base techniques would be very difficult or impossible to remove,” says Greg Thompson Jr., M.D., associate professor of neurosurgery and otolaryngology at U-M Medical School and co-director of the Cranial Base Program at UMHS.
Days before the surgery at U-M’s C.S. Mott Children’s Hospital, Zach’s mom described the teen as a couch potato hooked on Gameboy, Nintendo and TV. It’s a charge Zach refuted by saying the tumor had slowed him down and sapped his energy.
“I like to be more active but it’s like this tumor’s been stopping me from doing a lot of stuff. Usually, me and my brother play football a lot in the backyard. But now we can’t because of my tumor,” Zach said at the time.
Cathy White says she initially assumed Zach’s bloody noses and stuffiness were allergies, since it mirrored the symptoms of her other children, who did experience allergies.
“I was very surprised at the diagnosis,” Cathy White says. “But there’s no pain. He doesn’t have headaches. He doesn’t have anything so you know it’s there. We were really in shock at first.”
Juvenile nasopharyngeal angiofibroma accounts for only 0.05 percent of all head and neck tumors, occurring almost exclusively in males ages 7 to 19. In Zach’s case, the tumor had already grown quite large, filling the sinuses and pushing up against the brain and onto the internal carotid arteries in his skull base.
The Cranial Base Program at UMHS pools together ear, nose and throat surgeons, neurosurgeons and facial plastic surgeons for a multidisciplinary approach to treating tumors and abnormalities at the cranial base. That region encompasses the base of the skull, the facial bones, the vaults of the skull and the soft tissue in this area.
Historically, problems in and around the base of the skull have been difficult or impossible to treat. But new surgical techniques and medical treatments have changed that. Surgeons use a variety of techniques, but the subcranial approach, which was used with Zach, is most common.
“First we had to make an incision that went across the top of the head so we could peel the scalp down. Additionally, Zach’s left cheekbone had to be moved out of the way because the tumor was deep in this area. So our second approach was to cut the cheekbone and move it off to the side,” says Lawrence J. Marentette, M.D., FACS, professor of otolaryngology and neurosurgery at U-M Medical School and director of the Cranial Base Program at UMHS.
“The third approach involved an incision that went underneath the lip on the face. The skin of the lower part of the face was lifted up in this fashion and then the upper jaw was cut and fractured down.”
At this point, with Zach’s forehead, cheekbone and upper jaw moved out of the way, surgeons were able to access the tumor, looking at the bottom of the brain. By going straight at it this way, they minimize the need to move the brain.
Once Zach’s tumor was removed, surgeons put his face back together, placing the bones in their normal position and holding them in place with titanium micro-plates and screws. Next, surgeons moved the upper jaw, cheekbone, nose and forehead back in place and then peeled the scalp back up. To finish, they sutured and stapled the scalp in place across the top of the head and closed the incision underneath the lip with stitches that will dissolve.
“It sounds like it’s quite a horrendous procedure and that these patients would be in excruciating pain,” Marentette says. “However, in gaining the access to the facial skeleton, a lot of the sensory nerves are stretched. So in the immediate postoperative period, the patients are numb and they don’t have much discomfort. By the time the sensation returns after about one or two months, all of the bones have already healed. So the patients actually do quite well.”
Indeed, four days after the surgery, Zach said he already felt much better and was less tired than when the tumor was pressing against his skull. He said the area above his top lip and under his nose was numb.
“This is a lot better than I expected,” he says. “I thought I’d feel really bad still. So it went a lot better than I expected it to go.”
The hidden incisions will make it difficult to see any outward signs of the surgery, and it helps make the recovery process easier, Thompson says.
“It’s remarkable because the incision is made behind the hairline, so you really don’t see the incision when it heals,” Thompson says. “Then the incision Dr. Marentette made is up under the gum line, so that’s hidden as well. The healing process takes place at the level of the bone, where the cuts are made. The skin incision is hidden and everything that’s healing is healing underneath the skin.”
This type of surgery is possible largely because of operating microscopes with high power magnification and a 3-D computer navigational guiding system that maps the area around the tumor. The navigational guiding allows surgeons to know where important arteries and areas of function are while working in a small area deep into the patient’s skull. This helps protect the functions of the brain that control intelligence, memory, judgment, orientation and personality.
That’s all good news for Zach. And, Marentette says, these tumors rarely recur.
“His long-term prognosis is excellent,” Marentette says.
About juvenile nasopharyngeal angiofibroma
• Occurs exclusively in males.
• Most often develops between ages 7 and 19. JNA is rare in people older than 25.
• Represents 0.05 percent of all head and neck tumors.
• Cause is unknown but a hormonal link is suggested because it occurs in adolescent boys.
• Most common symptoms are nasal congestion, nose bleeds, headaches and facial swelling.
About the U-M Cranial Base Program
• Formed in 1994 by independent specialists as a multidisciplinary approach to treating difficult abnormalities and tumors of the cranial base region.
• The cranial base region includes the base of the skull, the facial bones, the fossas or vaults of the skull, and the soft tissues of these regions.
• The program encompasses specialists in otolaryngology, neurosurgery, ophthalmology, radiation oncology and medical oncology.
• Referral hotline: (800) 962-3555
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