Rebecca Ackerman, a vivacious 12-year-old who likes to play softball and is a straight-A student, is a medical marvel. She has a rare congenital anomaly of the brain, and has undergone multiple surgeries at Children’s Memorial Hospital since she was a little girl.
Rebecca was born with a cleft lip, a skin tag in her mouth, unusually wide-set eyes — which all required surgery — and a mysterious dimple on her spine. She experienced a number of medical issues during her first few years that perplexed her parents, Michele and Sam, and her pediatrician.
From six months of age, Rebecca had a chronic runny nose and suffered frequent ear infections. At age 3 she began to fall down frequently. She also complained that her feet frequently fell asleep.
When Rebecca was 5, the Ackermans consulted with Children’s neurosurgeon Robin Bowman, MD, who recommended Rebecca undergo an MRI scan of both her spine and brain. “While she had undergone previous spinal scans to rule out spina bifida, this was Rebecca’s first brain scan, and I knew in my heart that we would find an answer to all these strange symptoms,” says Michele.
Although the scans indicated Rebecca’s spine was normal, her brain scan showed she had a basal encephalocele inside her skull. Put simply, Rebecca was born with a small part of her brain extending through a gap in her skull and into her nasal cavity. Amazingly, her brain had rerouted nerves to counter the defect. The constant runny nose that she experienced was caused by leaking cerebral spinal fluid from her brain, which left her vulnerable to developing meningitis — a potentially life-threatening condition. The scan also showed that the location of the anomaly threatened the function of her optic nerves and pituitary gland.
“Sam and I just stared at the picture of Rebecca’s brain on the computer screen trying to comprehend the enormity of what Dr. Bowman had told us,” says Michele. “But Dr. Bowman was wonderful, and gently explained all the implications.”
Because Rebecca’s condition was so rare, Dr. Bowman reached out to a colleague in Washington, D.C., Derek Bruce, MD, one of the few neurosurgeons with experience performing the delicate surgery. Dr. Bruce taught Dr. Bowman and plastic surgeon Frank Vicari, MD, the technique he had developed, and traveled to Chicago to observe the surgery.
Rebecca’s surgery took nearly 13 hours. Because of the size of the encephalocele, they surgeons were unable to move it back into her skull. Instead, they used a bone graft to create a “shelf” to protect the extruding part of her brain. Her pituitary gland was unscathed, and Rebecca’s vision remained intact. The surgeons corrected the spinal fluid leak and were able to preserve her sense of smell.
Unfortunately, months later the protective shelf the surgeons created failed. Rebecca continues to live with viable brain tissue lodged in her nasal cavity. Because Rebecca is doing so well, Dr. Bowman has recommended holding off on further surgery for the time being.
“Rebecca takes it all in stride,” says Michele, who says her daughter also sees experts in the hospital’s Divisions of Endocrinology, Otolaryngology and Ophthalmology. “She loves to come to Children’s, and it seems like everyone there is her friend. But it’s hard not knowing what the future will bring.”
The Ackermans have showed their gratitude for Rebecca’s care by participating in fundraising events for the hospital including the Step Up for Kids stair climb to the top of the Aon Center and the Move for the Kids 5K Run/Walk.
Rebecca is also a member of the hospital’s Kids’ Advisory Board, whose members have had input into the design of Lurie Children’s.
“While I’m going to miss Children’s, I’m excited about the new hospital for many reasons,” says Rebecca. “Also, all the doctors and nurses who have taken care of me at Children’s are moving to Lurie Children’s.”.