What is CCI / AAI?

CCI / AAI is a rare complication that  occurs in a small group of EDS patients.

It concerns neck instability resulting in CCI (Cranio-cervical instability), AAI (Atlanto-axial instability), hindbrain herniation (posterior brain hernia) and brain stem compression.

Extreme mobility of the neck is the cause of these problems. Ultimately, when there is a compression of the brain stem, the condition is also life-threatening because bodily functions are damaged or even completely fail.

Symptoms of CCI include: Headache, neck pain, double vision, memory loss, loss of concentration, dizziness, speech problems, difficulty swallowing, sensitivity to light, sound, smell. Diagnosis is commonly made using an upright MRI or a 3D CT scan, which are not available in The Netherlands. For Nanette, this meant getting her diagnosis in Barcelona by Dr. Gilete at the Quirado institute with a 3D CT scan (CBCT scan).

With AAI there are stretched bands around the atlanto-axial vertebrae, causing the vertebrae to shift and creating great mobility of the cervical vertebrae. In recent years, it has been found that this condition is more common in patients with EDS. This condition is also diagnosed with an upright MRI or with a CBCT scan. In people without a connective tissue disorder, the CCI / AAI is limited to C0-C1-C2, in people with a connective tissue disorder such as EDS, the lower vertebrae usually also participate.

The operation in Barcelona can solve this problem. Complete neck fusion can prevent further posterior brain prolapse and brainstem compression.

For Nanette, a full neck fusion, both at the back and at the front is necessary. After the operation, Nanette will not be able to move her neck at all, but it will ensure that other bodily functions cannot fail. This operation can give Nanette a future.