On this page you can find links to more medical information.
Conclusion: The craniovertebral junction and upper cervical spine continue to show normal parameters of growth, curvature, and alignment in children with cervical fusion of C0-C4.
Neurological and spinal manifestations of the Ehlers–Danlos syndromes
What spinal problems can occur in EDS patients.
Chiari I malformation management in patients with heritable connective tissue disorders
Conclusion: People with hypermobility and Chiari often have neck instability
Cervical instability operated on: 2007
Evolution of posterior cervical and occipitocervical fusion and instrumentation
A pdf can be downloaded at the top right. Here on page 2 it says “OCCIPITOCERVICAL FUSION” and on page 13 “CONCLUSIONS” which are especially interesting. The operation has been performed since 1927.
In the early 1900s occipitocervical instability and lesions
located at the occipitocervical junction were considered
inoperable and terminal. Since the first description
of an occipitocervical fusion by Forrester in 1927, multiple
methods of fusion in this region have been described.
Article is from 2004
5 year update after fusion due to brainstem compression and neck instability. Article from 2019.
A site with lots of information, measurements and articles.
Cranio-cervical Instability in Patients with Hypermobility Connective Disorders
That it is not often seen in combination with EDS and that other images and more precise measurements are needed for proper diagnosis and treatment.
Optimizing Alignment Parameters During Craniocervical Stabilization and Fusion: A Technical Note
Why it is important to position the neck correctly during surgery and not to fix the neck as it is at that moment. Also hypermobility is mentioned here. CXA should be >140.
It is a whole book that can be purchased for $45.
Among other things, retroflexed odontoid and pannus are discussed, both signs of hypermobility that can also be seen on the image and are often overlooked by radiologists.
3-5mm low-hanging tonsils is Chiari type 1. In the Netherlands, the guideline is that it is Chiari type 1 from 5mm. See page 14. Chiari 0 is described further on, this is not recognized at all in the Netherlands (see appendix “Chiari 0 for real” from 2006). This manual was written by, among others, a few doctors who are highly regarded in the US for their Chiari knowledge and good results in treatment. Ellenbogen and Klinge (for Tethered Cord and Ocult Tethered Cord she is THE doctor to go to). You will come across Benzel in many research articles.
-Chiari 0 for real
-Chiari cough headache. Here is the explanation of the reason and solution for this headache
The title of the article says it all. Article is from January 2024.
Chiari malformation and atlantoaxial instability: problems of co-existence
That fusion with realignment is the best solution for Chiari and atlantoaxial instability (AAI)
Spinal manifestations of Ehlers-Danlos syndrome: a scoping review
Which diseases of the spine occur in EDS, how to diagnose them, what treatment is needed and what is important to pay attention to during the operation. Also that the PLOD1 gene abnormality belongs to kEDS and that this is an abnormality in the LH1 collagen, something that is not recognized in the Netherlands.
What is it and what is the best treatment method.
1e alinea introduction: AAI can also occur with minimal or no trauma in children as a result of ligament laxity
Scientific article on CCI and EDS
A new hypothesis for the pathophysiology of symptomatic adult Chiari malformation Type I
Chiari Malformation type 1
Opiate-free surgery in EDS patients
Frequency and co occurrence of comorbidities in the Ehlers Danlos syndromes
88% POTS
81% MCAS
40% tethered cord
24% Chiari Malformation
21% Craniocervical instability
20% complex regional pain syndrome
English 
Dutch