By age 5 months, D.C. was having grand mal seizures. He now takes medication to control them, and he works with a speech therapist. While his conversation may be difficult for strangers to follow, D.C. is verbal and skilled at communicating by body language what he can't express in words, Christine says.
The seizures and speech problems are the result of benign tubers in the brain. Tubers may also form in the liver, kidneys or other organs, affecting their function. While at Children's Memorial Hermann Hospital, the family took the opportunity to consult with Hope Northrup, M.D., a nationally recognized tuberous sclerosis expert. The disease disables genes that control cell growth, explained Northrup, a pediatrics professor and director of the division of medical genetics at The UT Medical School at Houston.
Benign tumors, called rhabdomyomas, may form in the heart, particularly in very young patients, but these typically regress on their own, noted Regina L. Lantin, M.D. They can cause abnormal heart rhythms that can often be treated by medication, but if they obstruct blood flow, the tumors sometimes require heart surgery.
"Most patients will live a normal life span, but they must be monitored regularly for known complications," explained Lantin, medical director of pediatric echocardiography at Children's Memorial Hermann Hospital and an associate professor at the UT Medical School at Houston.
Aortic aneurysms are a much rarer complication. This dangerous ballooning of the body's most vital blood vessel is fairly common in older adults but almost unheard of in children as young as D.C.
As is usually the case with older patients, D.C.'s doctors discovered his aneurysm while looking for something else. His doctor had ordered an ultrasound to examine a tuber in the boy's abdomen. He found that D.C.'s aorta was 10 times wider than it should have been.
"We would never have known otherwise," Chrisitine said. "He is asymptomatic and very active."
The family lives in Fort Worth, where Christine works as a respiratory therapist and her career-Navy husband, Donald, is stationed at Carswell Joint Reserve Base. D.C.'s doctors suggested they come to Houston, where he could be treated at Children's Memorial Hermann Hospital by noted expert Hazim J. Safi, M.D.
Before making the trip, they logged onto the Internet and watched Safi's December 2004 webcast of a thoracoabdominal aortic aneurysm (TAA) repair. "After I saw the video, I felt really comfortable with Dr. Safi. I didn't want anyone else doing the surgery," Christine said.
In adults, physicians consider surgery to repair aneurysms when they reach 5 to 6 centimeters in diameter. At that size, the risk of a rupture in the weakened arterial wall exceeds the surgical risk. D.C.'s aorta measured 7 centimeters, meaning a life-threatening rupture could happen at any time, said Safi, chairman and chief of the department of cardiothoracic and vascular surgery at UT Medical School at Houston.
"I've been in this business about 35 years, and this is the first TAA I've seen in a tuberous sclerosis patient this young," Safi said.
In fact, Safi's team found fewer than 20 instances in the medical literature of extensive aneurysms in young children, and the other cases involved only abdominal segments of the aorta. D.C.'s, however, extended from his midchest to lower abdomen.
Since D.C.'s case was so unique, Safi and his team had to modify the procedure they usually perform on adults. "You have to move faster, and you have to protect the spine," Safi explained.
In the highly invasive TAA repair, physicians must work very near the spinal cord and briefly interrupt the blood flow to it. Historically, post-surgical paralysis was fairly common, but Safi and his team have refined the technique to greatly reduce that risk.
Surgeons also faced the challenge of factoring in D.C.'s future growth. The procedure involves replacing the weakened aortic segments with a Dacron/polyester graft.
"We had to fashion a way to give it a chance to elongate," said Anthony Estrera, M.D., a member of D.C.'s surgical team and an assistant professor of cardiothoracic surgery at UT Medical School at Houston. Physicians needed to leave the graft a little wider and longer than immediately necessary, in order to accommodate growth. Leaving too much material, however, could potentially block blood supply to vital organs. The surgical team struck a delicate balance.
"We will follow him very closely," Safi said.
D.C. might have another surgery someday, but his doctors feel confident this won't be necessary until he reaches his teenage years, if ever.
A couple of days after his two-hour surgery, D.C. was already having trouble keeping still.
"He actually tried to get out of bed," Christine said. "He recovered a lot quicker than I thought he would – than anybody thought he would."
Safi's team performed D.C.'s surgery on a Wednesday. By Saturday, the boy was eating pancakes. By Monday, the family was on their way back to Fort Worth.
D.C.'s parents may have their hands full trying to keep him off the trampoline while he continues to heal, but Christine has a stack of books and video games ready. "He's laughing, he's smiling, he's walking, he's mocking me!" she laughs.
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