The CHOP Chiari Clinic’s team of experts collaborated with Gladys’ family to provide the best and least invasive treatment to correct her condition.  

Gladys was diagnosed with scoliosis during a routine physical at age 5. A follow-up MRI revealed the root cause of the scoliosis was Chiari malformation. 

Chiari malformation is an anatomic abnormality in the back part of the head where the brain and spinal cord meet. Normally, the brain sits inside the skull and meets the spinal cord at an opening at the bottom of the skull. In Chiari malformation, the cerebellum (the lower part of the brain) dips down into the opening. This can block the normal flow of spinal fluid in and around the brain and lead to a buildup of spinal fluid within the spinal cord.  

Some kids with Chiari malformation develop a syrinx. This is an abnormal collection of fluid in the spinal cord. It can cause scoliosis and lead to neurological problems such as muscle stiffness, weakness and pain.  

Gladys' family were living in New York when they got the diagnosis. Their pediatrician said they could either see a neurology specialist an hour away in Syracuse, N.Y., or travel four hours south to Children’s Hospital of Philadelphia (CHOP).  

The Neuroscience Center at CHOP is world-renowned for providing precise diagnosis, individualized clinical care and cutting-edge surgical treatment. It has a specialized Chiari Clinic led by Alexander Tucker, MD, an expert on the condition. 

Going the distance for specialized care 

Gladys' parents, Theresa and Joshua, decided to go to CHOP. They traveled to Philadelphia for Gladys' evaluation and were instantly impressed with Dr. Tucker and his team.  

After the evaluation, Dr. Tucker and pediatric orthopedic surgeon Jason B. Anari, MD, met with the family to discuss their findings. Detailed imaging of the spinal cord showed a large syrinx.  

The team in the Chiari Clinic tries to use the least invasive approach possible to stop Chiari symptoms from interfering with a child’s life. They believed minimally invasive extradural surgery would be the best surgical approach for Gladys. (Extradural means between the skull and dura mater, the tough outer layer of tissue that covers and protects the brain.) In this procedure, a neurosurgeon makes a small incision in the skull and makes the blocked passage between the brain and spinal cord a little bigger. This relieves pressure on the spinal cord and restores the normal flow of cerebrospinal fluid, which in turn can help shrink the syrinx.  

It’s a lower risk surgery with great outcomes, and patients need to spend less time in the hospital recovering after the procedure. There is usually no need for additional surgery. 

Doing Well Since Surgery 

The procedure went well, and Gladys was able to return home just a day after surgery.  

She’s now 9 years old and thriving. There’s been a significant decrease in the size of the syrinx since surgery. In addition to the surgery, Dr. Tucker and Dr. Anari recommended Gladys wear a brace 23 hours a day, which has slowed the progression of scoliosis. 

The family now lives in Kansas, but they return to CHOP every year for a follow-up MRI and appointment with Dr. Tucker. His confident and caring bedside manner cemented a special bond with them.