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Five-month-old Bishop Uhlrich models his custom-made helmet about two months after surgery.

Husband-and-wife surgical team reshapes little lives

February 2005

by Will Sansom

The recent tsunami disaster in South Asia resulted from a powerful underwater earthquake along the line where the India and Burma plates converge. The same enormous pressures inside the earth that spawned this 9.0 temblor have done other amazing things in history, including the collision that forced up the Himalayas.

Imagine the developing infant brain under such forces. The brain triples in size from birth to 2 years of age and grows tremendously fast in the first six months. The human skull allows for this increase through the property of elasticity at five seams known as sutures. What would happen if the brain had nowhere to grow? Would a Mount Everest have to rise on the forehead or side of the head or top?

The answer is, many undesirable things can happen when just one of the sutures is closed at birth or even in the womb. Headaches, eye problems and permanent disfigurement affect babies born with craniosynostosis, the medical term for premature closure of a suture. The disfigurement occurs because the brain presses on other parts of the skull, resulting in elongation of the head, bulging forehead or other deformities.

"A lot of babies are born with an abnormal head shape due to the birthing process," says David F. Jimenez, M.D., professor of neurosurgery and director of the Center for Neurosurgical Sciences in the School of Medicine. "But it should remodel in a few days. If in a week the baby’s head shape is not normal, check with a physician."

Dr. Jimenez and his wife, Constance M. Barone, M.D., professor of surgery and head of the division of plastic and reconstructive surgery, joined the Health Science Center last summer from the University of Missouri. These surgeons are pioneers of a minimally invasive technique to correct the problem during a baby’s earliest months. They have treated dozens of infants in the neurosurgical operating suite at University Hospital. "We like to operate on our babies when they are under 5 months of age," Dr. Barone says. "Thus, there is urgency for pediatricians to diagnose this condition and refer the infants to us."

For decades, surgeons have corrected craniosynostosis by making an incision from ear to ear, cutting out pieces of bone, reshaping them, and placing them back together with plates and screws. The surgery averages five hours, requires a pint of blood (almost the whole volume of a baby’s blood), and usually results in several days’ stay in the intensive care unit. "After traditional surgery, the children look like they have been run over by a truck," Dr. Jimenez says.

He and Dr. Barone worked out a totally new approach, one they have now used in more than 300 children. "The standard operations made no sense to me," Dr. Barone says. "Why open a baby’s head from ear to ear? We brainstormed the original problem and developed a new algorithm." That new concept was to make incisions just large enough to insert an endoscope, a small tube with a camera on it. The endoscope provides the visual field a surgeon needs to snip areas of bone near a closed suture, remove a minimum amount of bone and cauterize the area to prevent post-operative bleeding. "David is a whiz at endoscopy," says Dr. Barone, herself a seasoned surgeon.

The endoscopic surgery takes an hour or less and the blood loss is about two tablespoons. Trauma is so minimized that most babies are discharged to go home the day after surgery. A recent study conducted by the surgeons showed that 94.5 percent of babies who had the endoscopic surgery stayed in the hospital only one day. For a year after discharge, the babies wear specially made, custom-fitted helmets to counteract the genetic forces that gave rise to the condition and to help the brain fill the space it should.

No one else in Texas does the minimally invasive operation. Dr. Jimenez came to the Health Science Center for the opportunity to lead a neurosurgical academic program to excellence. The Center for Neurosurgical Sciences now has foursurgeons and four research laboratories in the School of Medicine. The bone extracted from the endoscopic surgeries is being saved to create a genetic registry of babies born with craniosynostosis.

Three-month-old Bishop Uhlrich made history recently as the first San Antonio infant to have the endoscopic surgery at University Hospital. Bishop’s problem was a closed sagittal suture, the seam that runs from the soft spot to the back of the head. Only one child in 1,000 needs surgical correction for craniosynostosis, but Bishop’s elongated head, apparent even on prenatal ultrasound scans, left no doubt he was the one. His parents, Brian and Natasha Uhlrich, considered the traditional surgery before their Internet surfing led them to Drs. Jimenez and Barone.

In the waiting room the day of surgery, the Uhlriches admit the hardest thing to do is to wait for surgery to start. But the 55-minute surgery goes by quickly. "It would have been much more difficult to sit here if we knew that the traditional surgery was taking place," Brian says. When the surgeons come out to speak to the family, they report no complications and low blood loss. "His head already is rounder," Dr. Barone adds.

Within a week, Bishop is fitted with his helmet and life after craniosynostosis begins. It is life without the conflict of a brain straining to grow with nowhere to go.

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Surgery Gives the Infant Brain a Window in Which to Grow



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