Local anaesthetic failure in joint hypermobility syndrome | J R Soc Med. 2005 Feb
When taking biopsies to assess skin strength in Ehlers–Danlos syndrome type III (EDS–III), a Danish group noticed that the patients experienced much pain despite conventional local anaesthesia.1
When asked, all these patients reported previous experience of partial or complete failure of local anaesthesia in dental or obstetric procedures—for which reason some had been dismissed as hysterics.
Pursuing this finding, Arendt-Nielsen et al.2 compared the effects of local anaesthesia in 8 patients with EDS–III and 8 controls.
Although the patients did gain analgesia from intradermal lidocaine the duration of effect was much shorter than in controls. EDS–III (now known as EDS–hypermobility type) is regarded by many authorities as identical to joint hypermobility syndrome (JHS).3,4
We wish to draw attention to the possibility of resistance to local anaesthesia in individuals with this common and under-diagnosed condition.
Box 2 Five-part questionnaire for identifying hypermobility (Ref. 7) Answers in the affirmative to 2 or more questions suggest hypermobility with sensitivity 80–85% and specificity 80–90%
Box 3 Brighton criteria for joint hypermobility syndrome (JHS) (Ref. 8) JHS is diagnosed in the presence of two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.
JHS is overrepresented in general rheumatology clinic populations and in our experience (RG and AJH)6 many of these patients report failure of local anaesthetics
A questionnaire was completed by 172 female Caucasian JHS patients, 53 non-hypermobile age-matched controls, and 28 individuals who showed evidence of hypermobility with insufficient features to qualify for JHS (HM-sine-JHS)
We asked ‘If you have ever had a local anaesthetic injection (dentist/minor surgery/epidural), did you think that it was as effective as it should have been?’.
58% of JHS patients, 21% of controls and 14% of the HM-sine-JHS group answered in the negative. For JHS versus controls the odds ratio was a highly significant 2.85.
Although the question was simplistic, non-exploratory, and reliant on recall and selfperception, the difference between JHS and controls was striking. Neither group had knowledge of any study hypothesis. At the time there was nothing of this nature documented in patient information sheets or published research.
We suspect that in many people JHS goes unrecognized. So, how can a busy clinician, mindful of possible resistance to local anaesthesia, make the diagnosis?
The nine-point Beighton hypermobility score (Ref. 9) One point may be gained for each side for manoeuvres 1–4 so that the hypermobility score will have a maximum of nine points if all are positive. A score of ≥4/9 indicates widespread hypermobility.
Although the pathophysiology of this phenomenon remains unresolved, an important clue that a patient is at risk of local anaesthetic failure might be in front of our eyes.
The study below indicates this lack of response to anesthetics has been known since at least 1991. It is even suggested as a diagnostic tool to differentiate EDS from generally hypermobile patients.
To make a differential diagnosis of Ehlers Danlos (EDS) Type III syndrome and Hypermobile patients has been difficult. In genetic advising and prognosis of the EDS patients there are need for new tools to separate them from hypermobile patients.
Topical analgesics (EMLA cream) was applied to seven EDS patients, ten hypermobile patients, and to fifteen controls.
The analgesic efficacy of cutaneous analgesia was evaluated by sensory and pain thresholds to brief argon laser stimuli, and the depth of the cutaneous analgesia was measured by sensory and pain threshold depth to controlled needle insertions.
Controls and hypermobiles did not differ in their response to cutaneous analgesia.
The thresholds to cutaneous laser stimulation and the depth of analgesia increased significantly less in the Ehlers Danlos patients compared to the other two groups.
In clinical practice a needle insertion test can easily be applied to investigate if patients are responders or non-responders to local analgesics.
This is a discussion from the EDS support forum on MDJunction:
Many patients with EDS are resistant to lidocaine, the most commonly used local anesthetic. Resistance to local anesthetics was proposed as a way of distinguishing benign hypermobility from Ehlers-Danlos syndrome, hypermobility type, but the research has not yet been replicated and the proposal has not been accepted.
I am not sure of the portion of EDS patients who have this, but think it is on the order of 10 to 20 percent.
Have you had a problem where a local anesthetic failed or where larger doses and more frequent renewal of the anesthesia was necessary?
Some folks with EDS have said that articaine (SeptocaineÂ®) works better than lidocaine. You might consider copying that name onto a small card carried in your wallet or purse so that, if you should encounter difficulty, you can tell the dentist or physician about the choice.
I came across an internet article that asserted the poor effect was the result of loose tissue and rapid reabsorption of the lidocaine from the area where it was deposited. The recommended solution was to use epinephrine to constrict the vessels locally, slowing reabsorption, or to use bupivacaine (Marcaine(R)), an inherently longer-lasting drug. I’m not at all sure I’m convinced by the theory of why lidocaine doesn’t work, but the recommended solutions might help some folks.