Chiari Malformation

Family credits Help Me Grow and preschool for prepairing boy for kindergarten.

By Richard Wilson Staff Writer for the Pulse Journal

Matthew Rought is one of about 765 kindergarteners at the Mason Early Childhood center.  He says he's making friends and likes his teacher, but starting school on time was not a certainty. 

Matthew, 4, underwent brain surgery in January to treat a condition known as Chiari Malformation.  The disease is becoming more common now that awareness among doctors is increasing and as more magnetic resonance imaging scans are conducted, said Matthew's doctor, pediatric neurosurgeon Francesco Mangano of Cincinnati Children's Hospital.

Mangano said the disease occurs when the brain is herniated and restricts the flow of spinal fluid at the base of the skull.  It occurs in people of all ages and can be brought on by an accident or a tumor, but the cause is often unknown.  Diagnosis is often missed by doctors because it has a wide variety of symptoms and can be identified only through a MRI scan.

"As more pediatricians become aware of this condition, hopefully it will lead to patients being treated and the alleviation of symptoms much sooner." Mangano said.

In Matthew's case, the disease gave him frequent headaches and affected his speech and fine motor skills.  His swift recovery after surgery seemed like a new beginning to his parents Merideth and Chris Rought, who watched their son's first years with growing apprehension.  They didn't know Matthew had the disease until a month before the surgery.
  Merideth and Chris worried about Matthew not developing at the same pace as other boys his age.  At 2 he knew only about three words and had a lot of trouble holding objects.  He also seemed to get headaches frequently.  As their health insurance wouldn't cover therapy for Matthew, they Roughts found help through the Help Me Grow program, provided by the Warren County Mental Retardation and Developmental Disabilities.  Merideth said Matthew showed progress right away as a therapist visited the Rought's home twice a week and worked with Matthew on learning words and using crayons and pencils.                                                                                                                            

" I about cried the first time I heard him say 'mom'," Merideth said.  The Roughts credit the Help Me Grow program and Mason City Schools' preschool program, where Matthew attended for two years, for getting him up to speed and ready for kindergarten.  Matthew was recently assessed by the school and was found to have motor deficits.  Merideth said the school is working on an idividualized education plan to address the issue.

Quick Facts about Chiari Malformation

Affects more than 300,000 people in the U.S.; 30 percent of children born with spina bifida are also born with Chiari Malformation.

Causes a wide range of diverse set of symptoms.  Research has shown that 95 percent of patients experience at least 5 symptoms.

Diagnosed using combination of MRI, neurological exam, physician's judgement, and other tests; patients often go five or more years before being properly diagnosed.

A study of more than 300 Chiari Malformation patients found that 57 percent had at one time been told by a doctor they were suffering from a mental or emotional problem.

Many patients undergo serious brain surgery in an attempt to stop symptoms from progressing.

Research has shown that surgery can improve symptoms for about 80 percent of patients, but fails about 20 percent of the time.

Source: Conquer Chiari and the C&S Patient Education Foundation

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Response to our article:

Thanks for bringing awareness

Thank you for the recent article bringing attention to the congenital condition, Chiari Malformation.  In January 2006, our son Guthrie was diagnosed with Chiari Malformation with Syringomyelia, a disorder that often in which a cyst forms within the spinal cord.  This cyst, called a syrinx, expands and elongates over time, destroying the center of the cord. Because the spinal cord connects the brain to the nerves in the extremities, this damage may result in sever pain and damage.  For a bright, active outgoing six-year old that was thought to be perfectly healthy, his family and friends were shocked and stunned!  We made the decision that he must undergo serious brain surgery in an attemp to stop symptoms from progressing.  After Valentine's Day surgery, a five-day hospital stay and a four-week recuperation at home, our son's surgery was deemed a success.

Thanks to the astute judgment of our local pediatrician, Dr. Todd Habel, early detection changed the outcome for our family.  As indicated in the article, we firmly believe that had it not been for the awareness of the medical staff to my son's condition, his future would have almost certianly held foreseen and chronic pain and debilitation.

As with many  diseases, Chiari manifests itself in such a wide range of symptoms it is extremely difficult to diagnose.  Based on my experience, I encourage all parents not to take lightly what may seem like mild symptoms or behaviors, as early detection is key to prevention.  When in doubt, persevere and continue to search for answers.

Amy R. (witholding lastname)

Mason, OH