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https://www.cdc.gov/nchs/data/icd/Topic-packet-Sept-2018.pdf
ICD-10-CM Coordination and Maintenance Committee Meeting September 11-12, 2018
Topic Packet One:
Ehlers-Danlos Syndromes (EDS)
A proposal to create thirteen new codes for Ehlers-Danlos Syndrome (EDS) was presented at the March 2018 Coordination and Maintenance Meeting.
The new codes that were proposed were in recognition of the thirteen specific types of EDS that were published by the International Consortium on EDS. The classification and manuscripts about EDS were published in the March 2017 Part C Seminars in Medical Genetics issue of the American Journal of Medical Genetics and are all available through the Ehlers-Danlos Society (
http://bit.ly/EDS2017papers).
However, based on public comment and the low prevalence of some types of EDS, a revised proposal to expand the more common and more severe types of EDS is being resubmitted for consideration.
Ehlers-Danlos syndromes are a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin hyperextensibility or laxity, and tissue fragility affecting virtually every organ system: skin, ligaments, joints, bone, muscle, blood vessels and various organs.
The prevalence and most common types of EDS cited in the GeneReview articles are:
hypermobile (hEDS) - 1:5000
https://www.ncbi.nlm.nih.gov/books/NBK1279;
classical (cEDS) - 1:20,000
https://www.ncbi.nlm.nih.gov/books/NBK1244
and vascular (vEDS) - 1:200,000 based on identification of 1500 affected individuals
https://www.ncbi.nlm.nih.gov/books/NBK1494/.
The most severe in presentation and the only one associated with early mortality is vascular (vEDS). “The long-term outlook (prognosis) for people with vascular Ehlers-Danlos syndrome is generally poor. It is typically considered the most severe form of EDS and is often associated with a shortened lifespan. Among affected people diagnosed as the result of a complication, 25% have experienced a significant medical complication by age 20 and more than 80% by age 40. The median life expectancy for people affected by vascular EDS is 48 years.[4][2]”
https://rarediseases.info.nih.gov/diseases/2082/ehlers-danlos-syndrome-vascular-type.
A specific ICD-10-CM code for the most common and severe types will be of value to the patient and the clinician. Regardless of the type experienced, EDS is a life- long progressive condition that has a major impact on the lives and daily function of most living with EDS.
This proposal is resubmitted jointly by Brad Tinkle, MD PHD, Division Chief of Clinical Genetics at Advocate Children’s Hospital and member of the Steering Committee of the International Consortium on EDS and Kay Jewell, MD Consultant, Acer Therapeutics Pharmaceutical Company.
(Proposed) TABULAR MODIFICATIONS
New/Revise subcategory
Q79.6 Ehlers-Danlos Syndromes
Add:
Q79.60 Ehlers-Danlos syndrome, unspecified
Q79.61 Classical Ehlers-Danlos syndrome
Q79.62 Hypermobile Ehlers-Danlos syndrome
Q79.63 Vascular Ehlers-Danlos syndrome
Q79.69 Other Ehlers-Danlos syndromes
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The following new codes were approved by NCHS and added to the FY 2020 ICD-10-CM Tabular List that was posted a few days ago:
FY 2020 ICD-10-CM Tabular List:
https://dxrevisionwatch.files.wordpress.com/2019/06/icd10cm_tabular_2020.pdf
[28MB]
Page 1148:
Q79.60 Ehlers-Danlos syndrome, unspecified
Q79.61 Classical Ehlers-Danlos syndrome
Q79.62 Hypermobile Ehlers-Danlos syndrome
Q79.63 Vascular Ehlers-Danlos syndrome
Q79.69 Other Ehlers-Danlos syndromes
[Edited to add link.]
