Man With Tumor for Face Braves Surgery

Jan. 11, 2008— -- To see the face of 32-year-old Huang Chuancai is to witness a rare genetic condition in its most terrible form.

Chinese doctors say Huang, of China's southern Hunan province, suffers from a disease known as neurofibromatosis, a genetic disorder of the nervous system that primarily affects the development and growth of neural cell tissues.

For many of its sufferers, the disease means abnormal growth of these tissues and, as a result, facial disfigurement. But Huang's case could well be the most extreme case of such disfigurement in the world today.

Before surgical intervention, 50 pounds of tumor tissue had pulled and twisted Huang's face into a distorted form, nearly unrecognizable as human. According to wire reports, doctors found that the heavy mass that droops from his head and face had deformed his backbone, stunting his growth. The tumor had totally covered his left eye, while his left ear hung down to his shoulder, and his right ear and jaw were completely engulfed.

Now, after more than two decades of suffering the horrific advance of his tumor, Huang is recovering from a second round of surgery on Monday to remove the massive growth. The initial operation, which took place last July, was a risky one-and-a-half-hour procedure that removed the largest of Huang's tumors, which weighed about 33 pounds.

While many more operations will be needed to remove all the excess tissue, Huang is reportedly hopeful that the treatments will allow him to lead a more normal life.

"I hope that when my illness is cured, I can go back home and have a nice spring festival with my family," he told Reuters.

Unfortunately, plastic and cosmetic surgeons say, it is highly unlikely that the surgeries will result in a normal appearance.

There is "zero chance of [Huang] looking normal, since the muscles, nerves, eye, bone and sometimes brain can be involved," notes Dr. Henry Kawamoto, clinical professor of plastic surgery at UCLA and director of the university's craniofacial clinic.

"I doubt that this patient will look 'normal' as suggested by [past media coverage]," agrees Park Avenue plastic surgeon Dr. Darrick Antell. "The patient would certainly be substantially better, but the degree of the problem is so large that 'normal' is unlikely in my opinion."

But doctors say the surgeries -- albeit risky -- could represent Huang's one best chance at leading as normal a life as possible.

"What he will get is a much more normal appearance," says Dr. Thomas Gampper, vice chair of plastic surgery at the University of Virginia. "Considering where he starts, the improvement will be dramatic, in that he will be recognizably human."

A Challenging Series of Surgeries

Though it only affects about one out of 4,000 people in the United States, neurofibromatosis is the most common genetic neurological disorder that is caused by a single faulty gene -- a mutation on chromosome 17. In total, it affects more than 100,000 people in the United States.

But few of those with the condition suffer the same degree of disfigurement that Huang experienced -- nor would many be forced to endure the same level of treatment. A major concern when dealing with surgery of this magnitude, surgeons say, is the bleeding that can accompany the removal procedure. One mistake could cause Huang to bleed to death.

"This would be as challenging as the face transplant cases from last year," says Dr. Brendan Stack, chief of head and neck surgery at the University of Arkansas for Medical Sciences. "The main challenges here will be blood loss, blood loss, blood loss, and underlying skeletal deformities."

Dr. David Song, chief of plastic surgery at the University of Chicago Pritzker School of Medicine -- who himself was part of a team that removed a 200-pound neurofibroma from a patient, agrees that there are many characteristics of such tumors that make them particularly difficult to work with.

"Massive bleeding is a major concern," he says. "Blood vessels from within and around a giant neurofibroma can be fibrotic (or hard), dilated and numerous. Any attempt to remove a giant neurofribroma must be taken with great care and planning, including the preparation and anticipation of major blood volume replacement, especially in the face."

But, Song adds, "When performed by the right team with experience, some of this difficulty can be mitigated."

And surgeons at the Fuda Hospital in China appear to be dealing well with the complexities of the surgeries thus far. Chief surgeon at Fuda Hospital Dr. Liu Lizhi told Reuters that Monday's operation, which aimed to remove 10 pounds of the tumor tissue, was not as risky as the larger July operation. But, he said, it was still a challenge.

"Compared to his first operation, the second one has some new issues," he told Reuters. "The main problem is the tumor is close to his ear, and his ear has already been invaded and extended by it ... So we will try to cut the tumor while keeping his ear and reconstruct it after the operation."

But considering the extent of Huang's deformities, surgeons will likely have to address the problem step by step -- and hope for the best.

"The biggest problem in reconstruction occurs when normal structures -- the mouth, ears, eyelids, nose, et cetera -- are stretched out of shape by the weight of a tumor this large," says Dr. Ricky Clay, plastic and reconstructive surgeon at the Mayo Clinic in Rochester, Minn.

"They have to be reshaped and repositioned to match the remainder of the face. There is no set method. It's a combination of anatomy, geometry, and imagination," he says.

"He has massive deformity. The challenge is to remove the tumor and leave normal tissue," says Dr. Brian Maloney, director of The Maloney Center for Facial Plastic Surgery in Atlanta. "He will need multiple surgeries and hopefully not have the huge flaps of tissue but will not look 'normal' ... If the bones of the face are involved he could even lose one of his eyes."

And as with any surgery, scarring will be an issue.

"This type of surgery would require multiple procedures and result in a significant potential scar," notes Dr. Julius Few, associate professor of plastic and reconstructive surgery at the Northwestern University Feinberg School of Medicine in Chicago. "People of Asian and African background have a higher risk for bad scars, such as keloids."

Surgery Will Not Be Last Challenge for Huang

And even after what promises to be an extensive battery of plastic and reconstructive surgery procedures, Huang will likely face continuing difficulties from his condition.

"There will be much tumor left behind," says Dr. Garry Brody, professor emeritus of plastic surgery at the University of Southern California's Keck School of Medicine.

"His entire jaw structure appears to be involved, with much or all of the bone being replaced by tumor. Bone grafts could not survive in that environment," he says. "Even after resection, the residual tumor will continue to grow and as there is elasticity in that type of skin it will sag again and require periodic redoing."

Brody adds that Huang will face a significant risk of any residual tumor tissue becoming malignant, potentially leading to life-threatening cancer.

But considering the profound impact that the tumors have already had on Huang's life, these additional challenges may seem par for the course.

The growth caused him to isolate himself from society. According to wire reports, the condition brought about bullying from his classmates early on, forcing him to leave school at the age of 10 -- and as his condition worsened, even the simple act of speaking became difficult.

Huang reportedly told Reuters that he was even approached several years ago by someone who wished to buy him and display him as part of a circus freak show.

It is a set of challenges that those with the condition in the United States will not likely have to face. Still, Kawamoto says even milder cases of the condition pose significant challenges for sufferers.

"Early treatment will decrease the amount of deformation but will not cure the disease," he says. "There is no cure. I am currently actively treating three children now. I wish the number was zero."

Reports from Reuters and the Australian Broadcasting Corporation contributed to this story.