Xylocaine injections into the SED

Pain (or rather, pain ( s) is one of the major symptoms of Ehlers-Danlos disease. Their intensity, sometimes considerable, is responsible for many situations of disability. Their resistance to analgesics, even powerful ones, is usual. On the other hand, they are often accessible to local treatments (heat, orthoses, TENS, lidocaine plasters...). It is therefore consistent to favour " peripheral " treatments of pain, with the hypothesis that these repeated local actions can have a long-term result on its production mechanism.

The local injection of Lidocaine is a common technique in Physical and Rehabilitation Medicine to treat muscle pain (" trigger points " or " trigger zone "). Long and repeated practice has largely validated them. We have therefore been inspired by this therapeutic contribution.

The action of Lidocaine injections can be spectacular and long-lasting, which is surprising in a disease that is also characterized by the frequent ineffectiveness of local, dental, locoregional and epidural anaesthetics. This treatment appears, today, as a resource in large localized or generalized painful crises and in painful respiratory blockages of costal origin.

Description of pain in Ehlers-Danlos disease

They are diffuse, multifaceted and misleading, permanent with crises of exacerbations and, above all, rebellious to the usual treatments, even powerful ones.

The whole body's in pain. The association of the following pains is very evocative:

• Periarticular (mainly tendon or fascia): spine, pelvis, shoulders, elbows, wrists, fingers (especially thumbs), hips, thighs, knees, ankles and feet;
• Muscles with cramps and tearing sensations in the trapezius muscles, back muscles, thighs, calves, sometimes associated with involuntary contractions as part of dystonia.
• Thoracic (Xyphoid sterno-costals or low costals);
• Abdominals (often " stabbed ");
• Female genitalia (during menstruation) but also, much more rarely, in men;
• Cephalic, migraine-like;
• Cutaneous with a hyperesthesia, sometimes considerable, which can be an obstacle to injections, the crossing of the skin being particularly painful in a durable way.

This association of pain and its progressive characteristics constitute a very strong argument in favour of diagnosis, which is based solely on clinical arguments, in the absence of genetic testing.

Their impact on the quality of life is very important, due to their permanence and exacerbation during " crises ", which are often unpredictable. They are the cause of many functional limitations, compromising the functions of sitting or standing, walking, chewing and gripping, because of their periarticular location. They also disrupt communication due to hyperacusis or hypoacusis and, more generally, cognitive functions during migraines which can be very intense.

Injection technique

Material used: a 20 ml syringe, the thinnest needle possible, its length is chosen according to the depth of the area to be treated;

The place of the injections

The injection should always be given where it hurts the most. Injection sites are identified by careful but cautious palpation so as not to trigger lasting pain, guided by the patient.

A mapping of the pain points of Ehlers-Danlos disease can be established as follows:

• Cervico-occipital region: insertions of the sternocleidomastoid, occipital rim, in the path of the Arnold's nerve, interspinous spaces C6-C7 or, especially C8-D1, body of the upper trapezius, especially at the union medial-internal third, body of the supraspinous, and sub-spinous, of the shoulder rotators, of the Deltoid.
• Dorsal region: periscapular muscles (angular in particular), interspinous spaces (from T5 to T7), middle trapezius, paravertebral, lower ribs (costal margin).
• Pelvis: parasacral region, posterior border of the trochanter, ischia.
• Hips, thighs: external face, upper edge of greater trochanter (insertion of the gluteus medius), along the external face of the thigh Maissiat strip). Insertion of the seamstress and tensor of the fascia lata (EIAS).
• Knee: medial side of the knee, patellar fins, patellar tendon.
• Ankle: internal and external retro-malleolar gutters (sheaths of the posterior leg and short lateral peroneal), back of the foot (common extensor of the toes).
• Shoulder: low insertion of the deltoid, acromion and bicipital gutter, pectorals, large dorsal,
• Elbow: epicondyle, tricep insertion.
• Wrist hand: along the palmar tendons, radials, extensor tendons of the fingers, especially the thumb, in the anatomical snuffbox in particular.
• Thorax: right and left sterno-costal region, sterno-clavicular, xiphoid appendix in particular, lower ribs, costal margin, axillary region.

Practical implementation

Alcohol disinfection. Use of Lidocaine 5 mg per millilitre placed in a 20 ml syringe. The recommended total volume injected is one vial per injection session. Use needles that are as fine as possible. We recommend intradermal needles (0.3x13mm REF 304000) for easily accessible areas (trapezius, periscapular muscles, costal grill), elsewhere, needles for subcutaneous (0.5x16mm REF 300600) or intramuscular injections. Crossing the skin is the most difficult moment, due to frequent cutaneous hyperesthesia, especially in children. In this case, a skin anaesthetic(Emla) can be applied 10 to 15 minutes before injecting. Before injecting, the needle should be touched to the skin, which may be enough to cause very sharp pain. The " touch" of the needle will then be used to find a less painful area in order to access the place you want to reach. The passage through the skin should be very slow and gentle, and once the needle is implanted, it should not be moved. The injection is done very slowly because the contact of the liquid jet is also enough to cause pain. The volume to be injected varies according to the place of injection but can be limited to a few drops of the product (at the level of the ribs in particular).

With these techniques, 1 to 10 injections can be given in a single session. For the cervico-scapulo-thoracic regions, it is best to carry out the injection, with the patient in a sitting position, for the pelvic region, it will be in the ventral decubitus position, for the thighs, it will be in the lateral decubitus position.

The effect is rapid, usually within a few tens of seconds. For the trapezium, the test of effectiveness is to be able to perform a larger, easier and painless rotation of the head. Generally speaking, the reported sensation is that a vice loosens, that the trunk, the shoulder, the hip " unblock ", that one can breathe amply without pain, that one can open and close one's hand, squeeze objects, without pain too.

The effect is often lasting over several days, weeks, months or even years, as we have observed. The frequency of injections does not seem to have any limitation. It is better, however, to respect an interval of two days between two series. A reaction of fatigue and drowsiness sometimes accompanies the treatment and prompts a reduction in the number of injections. Intolerance to Lidocaine, with intense general or local reactions is exceptional but, of course, prohibits this therapy. They can be repeated, leaving an interval of 48 hours between two series.

The Lidocaine-Cortisone association retains indications in Ehlers-Danlos disease, particularly in cases of algoneurodystrophy with frozen shoulder or threatened to be frozen (injection, by the posterior route of Cyriax in the glenohumeral joint) and infiltration of the carpal tunnel in the shoulder-hand syndrome. The same is true for certain tendinitises (Achilles, lateral peroneal...).