The Three Surgeries

Skull to Cervical Spine Fusion

This surgery is the most crucial to giving Julie an acceptable quality of life. This is a surgery which can only successfully be performed by a handful of surgeons across the world. It not only involves the ultra precise procedure of traction and aligning of the skull and vertebra to free the brainstem and spinal cord from compression, but also extremely delicate and precise surgical skills to navigate the unique structural, tissue, and systemic presentations as well as the plethora of potential complications in a patient with Ehlers Danlos Syndrome.

The minuscule adjustments of the bones into the correct position to both free the patient of brainstem and cordial stretching and compression along with placing the head and neck into a position in which the patient can have a functional angle of vision is also crucial. As the fusion is permanent, the position of the head will never again be able to change. Julie will no longer be able to turn her head nor able to move her head to look up or down. Therefore an incorrect angle would be a life long disability which cannot be repaired. Some recipients of this surgery elsewhere have had to face life with their head permanently turned to the side, upwards, or downwards.

Read more about the surgery here or on the images below.

Anterior Cervical Discectomy and Fusion (ACDF)

Julie requires both disk replacement due to ruptured disks and osteophytes (sharp bone protrusions inside the spinal column), as well as to stabilise the instability of the cervical vertebra. This surgery is performed from the front of the neck with the oesophagus and trachea being moved out of the way to gain access to the front of the spinal column. This gives more successful results for Julie’s particular case than the posterior (back of neck) approach. She will receive artificial disks plus plates but this could change to allograft (donor bone) if it is deemed to be a better choice during the surgical process.

Julie’s surgery will involve elements of both the ACDF and Atlantoaxial Instability surgeries. Each person is unique in their surgical needs and thus each surgery is unique.

Read more about the surgery below.

 Tethered Cord Syndrome

In the vast majority of people, the spinal cord floats in cerebrospinal fluid (CSF) within the spinal column. In some people, a high percentage in EDS people, the tail end of the spinal cord gets stuck to the inside of the spinal column. In rare cases it can herniate between vertebra as happens in Spina Bifida. Until further testing can be done in Barcelona to confirm or rule out, Julie is thought to have a tethered cord due to the symptoms she presents. A tethered cord means that it can no longer move up and down freely as the person bends their back. This causes the spinal cord to stretch causing issues with nerves exiting the cord near the base of the spine as well as contributing to further issues with the cord at the base of the skull and the brain stem.

The spinal cord can be freed from being tethered in a delicate spinal cord surgery. Once again, Ehlers Danlos patients pose unique issues and problems which a surgeon specialised and highly experienced is best posed to perform.

This surgery involves removing a part of the bone covering the cord to gain access before painstakingly dissecting the cord from the interior of the spinal column. Any errors made can leave the patient with many irreversible neurological issues such as leg movement, and rectal and urinary incontinence.

Click here or on the image below for more information on TCS

Click here for surgical images of Tethered Cord Release surgery NSFE