Amelia Harks was born on January 28, 2009 with a previously undiagnosed neural tube defect called an encephalocele. She was whisked from Cat Scan to MRI to MRV and to appointments with all of the top neurosurgeons in the Chicagoland area. They each looked at different views of the posterior fontanelle, the excess brain matter that had developed outside of her skull, the blood supply to the area, as well as the particular way that her skull had malformed during early embryonic development to see what needed to be done.
Encephaloceles, the Harks’ learned, were a form of neural tube defects that occurred when the embryonic neural tube—the primitive spinal cord—failed to close during the fourth week of pregnancy. While they can occur along the spinal cord, causing disorders such as spina bifida and anencephaly, their daughter’s skull had been improperly formed and part of her brain had developed outside of her body. It hadn’t been detected during a routine prenatal ultrasound and because it had been covered with skin—a closed neural tube defect–the quad screen hadn’t given any warning of high levels Maternal Serum Alpha Fetoprotein (MSAFP). Amelia’s had developed in the posterior fontanelle of her skull, which was the area that was known to be associated with the least favorable outcomes. However, their daughter appeared to be neurologically intact. She was eating, sleeping, engaging and behaving like any normal newborn baby, which encouraged both the family and doctors.
Upon examination of the multiple images, the team of neurosurgeons concluded that the encephalocele needed to be removed as soon as possible so as to not cause any future problems as the skull grew. At three weeks of age, Amelia was checked into the satellite unit of a major children’s hospital, where she would undergo surgery to remove the portion of her brain that had developed outside of her body. While her surgeon, Dr. Andrew Chenelle, was in the operating room, he would perform a cranioplasty and repair her skull with an implant so that the tissue would not reherniate, causing future problems.
The surgery was a complete success and Amelia was released the following evening into the loving arms of her family
At nearly a year old, Amelia is being followed by teams of developmental therapists and doctors, who make sure that she has access to any therapies or procedures that she might need. The statistics for children who have encephaloceles are sobering: only 21% are born living and half of those live births survive. The survival rate for those with posterior encephaloceles—like Amelia’s—is only 55%. Of those that do survive, 75% have varying degrees of mental retardation.
Amelia Harks, even in the face of those statistics, is testing at or above normal across the board. She’s talking and walking well before either of her older brothers, her mother reports, and is a happy, well-adjusted child who loves music and making mischief. The only hint that something is a little different about this child is the scar on the back of her head, about 3 inches long and stark white. Her parents cannot believe her resiliency and her spirit. Her father, Dave, says, “Our Mimi, she’s here for a reason.”
And she is.