Every morning, Rebecca Ackerman's nose would run for a few hours, infuriating the 5-year-old. Then a doctor made a startling discovery: Those pesky drips weren't allergies but spinal fluid -- Rebecca had a hole inside her skull.
She was lucky. A dramatic surgical fix healed this rare birth defect, which allows brain tissue to grow in the back of the nose, before the Chicago girl contracted a brain infection. It is a technique that also can help children with certain hard-to-remove tumors, and the operation's developer is on a quest to teach more surgeons and parents about the option.
Too often, "neurosurgeons say nothing can be done," said Dr. Derek Bruce of the Children's National Medical Center in Washington, who coached Rebecca's surgeon in the painstaking operation.
At issue are encephaloceles, openings where the skull didn't form properly and parts of the brain can slip through. Some are large and fatal either before birth or soon after.
Rebecca's type, called a sphenoidal encephalocele, is sneaky. Thought to occur in a few dozen births a year, it often is undiagnosed until babies or children suffer repeated bouts of meningitis and puzzled doctors order a brain scan.
During fetal development, the pituitary gland -- the master gland that controls most hormone production -- forms in a little channel between the back of the throat and the brain. With this neural tube defect, bone doesn't form to close off that channel. The pea-sized pituitary slides from the bottom of the brain into the back of the nose, and bacteria easily can travel the other direction, into the brain.
Source
She was lucky. A dramatic surgical fix healed this rare birth defect, which allows brain tissue to grow in the back of the nose, before the Chicago girl contracted a brain infection. It is a technique that also can help children with certain hard-to-remove tumors, and the operation's developer is on a quest to teach more surgeons and parents about the option.
Too often, "neurosurgeons say nothing can be done," said Dr. Derek Bruce of the Children's National Medical Center in Washington, who coached Rebecca's surgeon in the painstaking operation.
At issue are encephaloceles, openings where the skull didn't form properly and parts of the brain can slip through. Some are large and fatal either before birth or soon after.
Rebecca's type, called a sphenoidal encephalocele, is sneaky. Thought to occur in a few dozen births a year, it often is undiagnosed until babies or children suffer repeated bouts of meningitis and puzzled doctors order a brain scan.
During fetal development, the pituitary gland -- the master gland that controls most hormone production -- forms in a little channel between the back of the throat and the brain. With this neural tube defect, bone doesn't form to close off that channel. The pea-sized pituitary slides from the bottom of the brain into the back of the nose, and bacteria easily can travel the other direction, into the brain.
Source