Estephan Zuniga is enjoying being a teenager. He smiles broadly as he talks about hanging out with his friends and what he might do for a career some day. But until he recently received specialized pediatric neurosurgery and orthopaedic surgery at Children’s Hospital Central California, all he could think about was what it would be like again to breathe normally, walk without slumping over and not be in constant pain.
“I just wanted to be like other kids,” explains Estephan, his mood now more somber as he recalls his life before getting medical treatment. “It was hard.”
Diagnosed with mild scoliosis in junior high school that progressed rapidly, Estephan soon suffered from a debilitating 100-degree curvature of the spine. Hunched forward, with a large prominence on his back and his right shoulder significantly higher than his left, he had trouble walking and endured frequent discomfort and fatigue. Scoliosis this severe puts extreme pressure on the heart and lungs, and can become life-threatening if untreated. Estephan also experienced ongoing headaches and muscle weakness due to other medical complications, including structural defects in the cerebellum that affected the normal flow of spinal fluid to and from his brain.
As Estephan’s health steadily declined, not surprisingly he became more socially withdrawn. Although he had loving support from his parents and two brothers, he felt embarrassed about his condition. “He would stay in his room a lot, not do things with his friends – he became very quiet,” says his mother, Llane Zuniga, glancing at her son in their Fresno home. “It was very difficult for him – and for us.”
Thanks to Dr. Meredith Woodward, a neurosurgeon, and Dr. Michael Elliott, an orthopaedic surgeon, at Children’s Hospital, Estephan, now 17, has a new life. Surgeries with each board certified physician resolved his spinal fluid issue and, using a new, safer technique called Internal Traction to improve spinal deformity, reduced his curvature by an impressive 80 degrees. “Both are excellent doctors,” says Estephan. “I don’t know where I would be without them.”
Estephan’s first experience with Children’s Hospital began soon after he was born. When he was about a year old, Dr. Woodward surgically removed a large cyst that developed on the back of his head near his neck, with a root that extended deep into his brain.
Llane said her son didn’t experience any health issues until years later when a test at his junior high school indicated he had scoliosis, which escalated quickly. Meanwhile, Estephan also struggled with very bad headaches and low energy, and was referred to Children’s Hospital for further evaluation.
As a result, Dr. Woodward diagnosed Estephan with Chiari malformation type 1. The congenital disorder – in which part of the cerebellum is located below the foramen magnum, or the opening at the base of the skull – affects the natural flow of cerebrospinal fluid, the clear liquid that surrounds and cushions the brain and spinal cord. The condition can cause headaches, fatigue, muscle weakness and sometimes paralysis.
“Essentially the brain gets pushed into the cervical spine because there is not enough room in the head for the brain, and this puts pressure on the brainstem,” says Dr. Woodward.
Spinal curvature is common among individuals with Chiari malformation, especially in children whose skeleton has not fully matured. Dr. Woodward also detected that Estephan has hydromyelia, an abnormal widening of the central canal of the spinal cord that creates a cavity in which spinal fluid can accumulate. As spinal fluid builds up, it may put abnormal pressure on the spinal cord and damage nerve cells and their connections.
To correct the issue, Dr. Woodward performed decompression surgery. She removed a small portion of bone from the back of Estephan’s skull and upper cervical spine and created a “patch,” thereby reducing the pressure and making more space for the brain tissue.
“The brain is like the motor that runs the body,” says Llane. “If done incorrectly, the brain procedures could have hurt Estephan’s ability to function. Dr. Woodward took care of Estephan very well, just like she did when he was a baby.”
To improve Estephan’s severe curve, Dr. Elliott turned to a cutting-edge technique. Used for curves of 100 degrees or more, Internal Traction is performed in two stages. The first surgery implants a rod that partially straightens the spine. About a week later, a second operation inserts double rods allowing further improvement. This gradual approach is safer for the patient and results in shorter hospitalization.
In Estephan’s case, the first surgery improved his curvature by about 50 degrees. Post-surgery, he was admitted to the pediatric intensive are unit (PICU) at Children’s. “I couldn’t walk or get out of bed for a week,” says Estephan. “The nurses and doctors were great. They made sure I was comfortable and joked around with me to keep my spirits up.”
Estephan remained in the PICU another week after the second surgery before going home. He began regaining his strength and did physical therapy for three months to improve function.
“Like with Dr. Woodward, I knew how risky a spinal surgery can be,” says Llane. “But Dr. Elliott explained everything. We had confidence in him and he didn’t let us down.” Following a short pause, with tears in her eyes, she continued, “Dr. Elliott changed our life. He gave us back our son.”
For best results, Dr. Elliott emphasized that these types of complex procedures should be done at a pediatric hospital such as Children’s that specializes in spinal surgery for patients like Estephan. “We have the best options available with modern technology that we can tailor to each patient,” says Dr. Elliott, one of five orthopaedic surgeons at Children’s.
Today, Estephan stands taller and breathes easier – literally. More confident and no longer wishing he could be more like “the other kids,” he enjoys socializing and school. “I can run now and even lift weights,” says Estephan proudly, referring to his improved activity. “I’m not in pain any more.”
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