ORTHOPEDIC PROBLEMS IN THE EDS PATIENT
Edward G McFarland
The Wayne H Lewis Professor of Orthopeadic and Shoulder Surgery
The Johns Hopkins University
Baltimore MD
I. Introduction
A. Orthopaedic Surgery:
1. discipline for the study and treatment of disorders of the bones, muscles, joints,
ligaments (connect bone to bone and provide stability) and tendons (connect
muscles to bones)
2. Includes non-operative treatments and operative treatment
3. Surgeons have varying interest in non-surgical alternatives
4. Most surgeons are not familiar with EDS
5. Evolution of super-specialization there are few surgeons who treat every joint of
patients with EDS
II. Prevalence
A. Joint soreness (91%)
B. Joint swelling (39%)
C. Joint instability (76%)
a. Shoulders (78%)
b. Knees (85%)
c. Hips
d. Ankles (77%)
e. Sacroiliac joints
f. Fingers
g. Ribs
h. Elbows (50 %)
III. Etiology
A. Pain etiology not really known but it is real
B. Due to stretch alone?
C. Instability due to:
1. Laxity
2. Pain inhibition
3. Rarely bone deformity
4. Poor muscle control or tone
D. Often no cartilage damage at time of scope
IV. Evaluation
A. Having a diagnosis of EDS is first step---knowledge is power
B. History and physical examination---beware the MD who does not examine you
C. Physical examination laxity testing (testing on examination how loose the joints are or
are not) -----when does laxity become instability? Not easy to know or define
D. Other studies:
A. Should still start the evaluation with plain or regular radiographs
B. MRI , CT scan or bone scans as needed
C. Since joints loose, many studies such as MRI will not show any pathologies
1. The issue is typically that the lining of the joint and ligaments are too loose; this
cannot be easily be determined by MRI
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2. MRI scans are “over-read” by radiologists all the time—they see shadows but do
not have to correlate it to what is seen clinically
a. Example: labrum tears of the shoulder
1. The labrum is a cartilage around the shoulder that stabilizes it
2. It is rarely torn without some traumatic event
3. Nearly every shoulder MRI has some labrum pathology according to
radiologists
4. Orthopedic surgeon should read the scan personally
5. The finding on the scan should be consistent with your history and
examination
V. Treatment
A. The big issue with EDS (or any patient) is whether surgery is indicated or not
1. Bones heal normally in EDS typically, so bone operative procedures (fractures,
joint replacement) generally can do well
2. Soft tissue operations are a trade off: if you tighten the ligaments, the joint will
be tight for at least a little while—how long is difficult to predict in most cases
B. Non-operative
1. Modify activity but stay active as possible
2. Ice
3. Heat
4. Acetaminophen
5. Non-steroidal anti-inflammatory drugs (ibuprofen, naprosyn etc)
a. be very aware of complications
b. if long term should be followed
6. Physical therapy—the goal is a treatment plan you can do at home due to expense
1. Ultrasound
2. Electrical stimulation
3. Phonophoresis
4. Ionatophoresis
5. TENS units
6. Massage
7. Strengthening exercises
8. Aquatherapy
7. Yoga
8. Pilates
9. Cortisone shots
a. Limit to specific diagnosis---don’t use without a specific diagnosis
b. Long term affects not known
c. Number per year safe not known
d. Best to not rely upon these for pain relief
e. Many EDS patients have resistance/reaction to local anesthetics
10. Chondroitin etc---60% of people get pain relief
11. Injectable chondroitin sulfate (Synvisc etc)---one shot available now
12. Plasma rich protein
a. Little data to support efficacy but enthusiastic use
b. Widespread use not warranted
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c. Many studies but no clear answer
d. Expensive
C. Surgical
A. Few published studies
B. General sense among orthopaedic surgeons that should not operate upon patients
with EDS---not true but the risks and benefits have to be carefully assessed
1. Bone operations do better
2. Loose joint operations do less well due to stretching out over time---is short
term gain worth risking the complications of an operation?
C. Potential complications [1]: increased in “classic” type
1. Wound problems (11%)
a. Poor healing deep portions of soft tissues
b. Skin healing (6%) leave stitches I as long as possible
c. Infection (6%) increased risk if bleed
2. Stiffness—unusual complication in EDS but it can happen
3. Arthritis---change the way the joint works so not “physiological” for the EDS
patient
4. Medical complications---aneurysms etc; good medical work up preop
D. McFarland et al [2]: Weinberg J, Doering C, McFarland EG: Joint surgery in Ehlers-Danlos patients:
results of a survey. Am J Orthop (Belle Mead NJ). 1999 Jul;28(7):406-9.
1.
2.
3.
4.
5.
6.
Survey EDS patients
Not real science
Questions about different joints
High satisfaction rate: 67%
Low success rate: 53%
Due to the disease, if you tighten the ligaments, they stretch out again
eventually
7. As a result of this study, I recommend patients with EDS understand risks and
benefit for surgery
E. McFarland et al “Knee replacement in EDS”
1 Retrospective review of 10 patients 12 knees
2 Follow up 65 months
3 Successful in 7 of 10 patients (8 of 12 knees)
4 Concluded knee replacement can be beneficial; may need stabilized designs
F. Others [5&6]
G. Anecdotal success stories for certain indications
VI. My personal experience
A. EDS can be a challenging disease for everyone (you, family, me)
B. Most important thing is attitude of the person: “I can deal with this”
C. Support through EDS support group critical
D. Surgery should be the last resort and one’s expectations should be realistic
E. Try to exercise everyday—find ways to deal with the pain
F. Diet and weight control important for lower extremities
G. Example: Shoulder Surgery for looseness and pain
1. “demonstrable” shoulder instability
2. Can be devastating problem
3. Gerber study [4]:
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4.
5.
6.
7.
8.
9.
a. Two groups follow up 12 years
b. No surgery: no arthritis
c. Surgery: The instability recurred in 20% of shoulders, pain was common and
subjective satisfaction was less good
d. Subluxations generally do not lead to arthritis and pain usually tolerable
e. Surgery alters joint and causes bones to rub on each other
f. Relief is short term only
Undergo surgery only as very last resort for this problem
Surgery is operation to tighten ligaments
Usually restrict motion in brace for 6-8 weeks
PT often not necessary to regain motion
If the operation fails and there is recurrence, generally should avoid the impulse to
have it done again.
Fusion of joints---some joints fusions are tolerated better than others
EDS can be conquered----surgical interventions should be undertaken with a realistic expectation of
what is possible, but few surgeons are familiar with this disease and there is little published literature on
the results of surgery.
Bibliography:
1.
Weinberg J, Doering C, McFarland EG: Joint surgery in Ehlers-Danlos patients: results of a survey. Am J Orthop (Belle
Mead NJ). 1999 Jul;28(7):406-9.
2.
Rose PS, Johnson CA, Hungerford DS, McFarland EG: Total knee arthroplasty in Ehlers-Danlos syndrome. J
Arthroplasty. 2004 Feb;19(2):190-6.
3.
Voermans NC, Knoop H, Bleijenberg G, van Engelen BG: Pain in Ehlers-Danlos syndrome is common, severe, and
associated with functional impairment. J Pain Symptom Manage. 2010 Sep;40(3):370-8. Epub 2010 Jun 25.
4.
Huber H, Gerber C.Voluntary subluxation of the shoulder in children. A long-term follow-up study of 36 shoulders. J Bone
Joint Surg Br. 1994 Jan;76(1):118-22.
5.
Jasiewicz B, Potaczek T, Tesiorowski M, Lokas K: Spine deformities in patients with Ehlers-Danlos syndrome, type IV late results of surgical treatment. Scoliosis. 2010 Nov 25;5:26.
6.
Liu Y, Gao R, Zhou X, Yuan W.Posterior spinal fusion for scoliosis in ehlers-danlos syndrome, kyphoscoliosis type.
Orthopedics. 2011 Jun 14;34(6):e228-32. doi: 10.3928/01477447-20110427-28.
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