Chiari Malformation in Pediatric Sleep Patients
It has been an interesting couple of weeks here in the sleep center. I mean, sleep is always interesting to me but the past week has been unusually so.
As you may recall, we do pediatric studies here about 50 percent of the time. We see a lot of very sick kids and many who are extremely challenged. The usual suspects include uncontrolled asthma, cystic fibrosis, Down's syndrome, duchenne muscular dystrophy, and then plain old poor sleep hygiene. Last week we added a new one, chiari malformation.
Now I was used to taking care of patients with this diagnosis in my other life as a nurse, but not so much since coming to sleep and not in kids. For those of you not familiar with the condition, chiari malformation is where brain tissue protrudes into the spinal canal. It occurs where part of the skull is misshapen or abnormally small so it presses on your brain and forces it downward. The condition is rare, but with improved imaging tests, we're seeing more frequent diagnosis. The adult form, Chiari malformation type I, develops as the skull and brain are growing so we don't see symptoms of the condition until late childhood or even adulthood. But the most common pediatric form, Chiari malformation type II, is present at birth (congenital). (Read more from Mayo Clinic by clicking here)
My experience with it as a sleep tech started earlier this week with a 10-year-old girl who showed severe sleep apnea with about 50 percent centrals. She went home with a prescription for nighttime oxygen and came back for CPAP. The centrals worsened. We proceeded with bilevel and bilevel with a back-up rate. My manager, who covers all of neurodiagnostics suggested we get an MRI. The MRI showed the chiari malformation.
Midweek, we had a 10-year-old boy come in who had similar symptoms and already had ENT surgery and was now on CPAP. He too has a chiari malformation. And finally, that same week I did a day sleep study on a 2-month-old infant diagnosed with chiari malformation. So now, when we see this particular pattern in children we will add a MRI to our recommendations.
It was amazing to have that bombardment of chiari malformations all in one week and gratifying to again have an immediate impact on someone's health. I think that is why I am so passionate about sleep medicine and being a sleep tech. We see such immediate results and can affect positive outcomes compared to the rest of healthcare.