Medical First: Boy’s Brainstem Saved by a Nose

15 year old boy with Type One Chiari Malformation Story

transnasalA young boy with a rare case of  Type One Chiari Malformation (CMI) was the first to undergo a new minimally invasive spine surgery through his nose.  Dr. Richard Anderson from the Pediatric Center and his colleagues presented this case at the annual Pediatric Neurosurgery Section meeting in December.

CMI is a birth defect where the bony opening at the base of the skull is malformed and, as the patient grows, can cause a dangerous build-up of pressure on the brain and spinal cord.

Surgeons usually uncrowd the area by removing bone from the back of the skull and neck. In some rare cases however, surgery has to be done on the front of the spine too because of pressure on the brainstem.  This was the case with this boy.

Typically, the front of the spine is accessed through the mouth.  This can be a problem when working with children because their mouths are so much smaller. Furthermore, when the surgical site is as high up on the spine as the brainstem is, surgeons sometimes have to split the palate to get there.  Even when they don’t, working through the mouth can cause considerable swelling that sometimes requires prolonged use of a breathing tube.  Additionally, if there is a deformity or some other problem, the mouth as a surgical entryway may not even be an option.

Surgeons have developed a much less invasive procedure using an endoscope through the patient’s nose.  An endoscope is a tube through which tiny cameras, lights, and surgical tools can be passed to perform a much less traumatic version of the same surgery.  The procedure has been used on adults and now for the first time, it has successfully been performed on a child.

The patient was a 15 year old boy, who complained of headaches, had difficulty swallowing and experienced weakness in his arms.  His speech was altered and he had lost his “gag “ reflex (an important reflex that keeps us from choking.)

Dr. Anderson and his team were able to use the endoscope through the boy’s nose to get to the front of his spinal column.  There they removed a piece of bone that took pressure off of his brainstem. He recovered well and by his one month checkup, his headaches were gone, he could swallow and speak normally, and his “gag” reflex had returned.

With the success of this procedure,  his surgeons encouraged fellow pediatric neurosurgeons at their recent meeting to consider this as a viable alternative for children with this kind of problem.

The Annual Pediatric Neurosurgery Section Meeting is a joint venture of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons. (See the full agenda from the December 2009 meeting).

See another one of our blogs about Chiari 1 Malformations: Pediatrics Added to this Year’s IML at the Congress of Neurosurgery Meeting