Born with a rare and serious brain disorder, Troy is now a happy child who enjoys time at preschool.
Nationally recognized team at Rady Children’s helps boy overcome life-threatening brain abnormality.
Jodi Read, 47, an infant special education teacher with the San Diego City School District, first worked with Troy, a child with multiple physical problems, when he was just 6 months old. She admired the tiny baby who, despite his problems, was a spunky little fighter.
When Troy was a few weeks old, he was diagnosed with a basal encephalocele, a problem caused when the brain base fails to close during pregnancy. A portion of abnormal brain extended downward and pushed through the normal bone of the skull base. He needed immediate surgery because the encephalocele was causing him severe breathing problems.
Encephaloceles cases are very rare in the United States and other western countries – approximately one in 35,000 live births. They are more common in Southeast Asia and Russia, occurring in about one out of 6,000 births.
At 26 days old, Troy was operated on by Dr. Michael Levy, professor and director of Rady Children’s Neurosurgery, and Dr. Steven Cohen, director of Craniofacial Surgery at Rady Children’s and clinical professor of Plastic Surgery, UCSD. Dr. Levy successfully performed a craniotomy, removing a portion of the skull, removing some of the abnormal tissue and keeping the rest in place. Next, the team strategically reconstructed the normal brain covering and the bony floor of the skull.
“He was very young, so we didn’t remove all the abnormal brain tissue because it would cause so much blood loss,” explains Dr. Levy. “At the time, we thought we had taken enough that there wouldn’t be a future problem.”
Troy received a feeding tube and was diagnosed with “failure to thrive,” so he stayed at Rady Children’s Convalescent Hospital – the Helen M. Bernardy Center – until he was 20 months old. He joined the Read family as a foster child in 2007.
“When Troy was placed with us, he had clear water dripping from his nose,” says Jodi. “Dr. Levy told us that he was leaking cerebral spinal fluid, so they needed to do a second craniotomy to seal the leak. We were all very worried.”
The four- to five-hour surgery, which took place in February 2007, was extremely complicated and dangerous. Dr. Levy used a pediatric skull base approach to surgery because it was very difficult to access the area. Rady Children’s has one of the leading craniofacial and neurosurgical teams in the country and has extensive experience treating complex skull base and neurocraniofacial disorders in children.
“It was a complicated procedure that included removing all the bones of the orbital region of the face just to gain access to the encephalocele and remove it,” says Dr. Cohen. “We reconstructed the covering of his brain using a flap of tissue from the undersurface of the scalp that we sewed to the underside of the covering of the brain. Then we repaired the skull base of the brain with bone grafts taken from his skull and reassembled his facial bones.”
One week later, Troy leaked spinal fluid again and, in March 2007, they performed a third craniotomy to patch a portion of the skull floor to stop the leak. Basal encephaloceles are one of the most challenging pediatric skull base disorders. In fact, it is not uncommon for children to die from the disorder.
Although treated with several antibiotics, he developed osteomyelitis, a bone infection in the skull and surgeons operated in July 2007 to remove dead bone. Neurosurgeon Dr. Hal Meltzer also placed a lumbar shunt in Troy’s spine that drains into his abdomen.
Troy also must take medicine for the rest of his life for diabetes insipidus, commonly called water diabetes, which causes profuse urination. Because Troy has no growth hormones, he must take daily injections of them until he stops growing.
“Troy’s biological mother was overwhelmed by all his surgeries and illnesses and relinquished him for adoption,” says Jodi. “My husband, David, and our children Jessica, Michelle and Andrew were all very attached to him, so in December 2008, we adopted Troy. Now he’s our little boy.”
Although things were looking up for Troy, he would face even more challenges. In January of this year, Troy developed meningitis, causing increased pressure in his brain. Dr. Levy performed a procedure called endoscopic third ventriculostomy. A small perforation was made in the floor of the third ventricle of the brain to reduce the fluid that was causing the pressure. Troy spent 15 days in the hospital, where he received IV antibiotic treatment.
Because Troy has a 2 ½-inch circle in his forehead where bone is missing, he will undergo surgery this month. Smooth bone from his skull will be used to close the hole in his forehead and his rib bones will be used to close his skull.
Troy takes speech therapy, occupational therapy and physical therapy and sees Dr. Bretten Pickering, a special needs pediatrician, every six months. He will need ongoing craniofacial surgery to reposition his eyes and work around his nose as he grows, but it is unlikely he will have any more neurosurgery.
Despite all his medical hardships, 4-year-old Troy is a bright, happy child. He takes a gym class and swimming lessons, and he will attend a pre-kindergarten class this fall at Together We Grow, a special preschool for medically fragile children.
“We are dreading the surgery this fall because he’s old enough to understand it will be painful, but we know he’ll overcome it,” says Jodi. “The doctors feel he’s doing very well overall and will lead a fairly normal life. We’re very proud of this tough little guy and eager to see him continue to grow and learn.”
The San Diego Union-Tribune Kids’ NewsDay, October 2009