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Childbirth and Ehlers-Danlos syndrome

Practical advice

Translation: Lucette Ducret, MD

 

The vagueness that continues to exist around this common collagen hereditary disease contributes to creating unfounded fears around childbirth in future parturients with Ehlers-Danlos syndrome (EDS), but also in obstetricians, midwives and anesthesiologists. Yet, its diagnosis on a set of quasi-pathognomonic clinical signs and the demonstration, by clinic alone (existence of other family case), of its hereditary nature with systematic transmission, is a major step forward. Anxieties are often based on disturbing descriptions found on Internet or transmitted by social networks. Obviously, childbirth must be the object of special attention with these patients with fragile tissues (possibility of perineal tears, hemorrhages) with peculiar uterine reactions due to proprioceptive disorders, including increased pain by hypersensoriality. However, if certain precautions are observed, these deliveries take place without unfortunate consequences for the mother, as for the child.

- During pregnancy uterine contractions can occur before term, requiring rest.

- At the time of delivery, many patients reported the dissociation between occurrence of uterine contractions, often very painful, and absence of cervix dilation. This can perfectly be explained by proprioceptive disorders which constitute the basis of the pathophysiological reasoning in this disease. Synchronization between uterine contractions and cervical opening is defective or absent due to the poor quality of the signals sent to the regulatory centers. This situation is extremely distressing and can be prolonged in the absence of local action and / or drug prescription facilitating the opening of the cervix. It appears that this point, which is quite specific to women with Ehlers-Danlos syndrome, is not known in obstetrics world.

- Joint instability and positional compression syndromes during childbirth. Due to hormonal impregnation linked to pregnancy, the already flexible, deformable, loose and fragile tissues are even more so. Usual risks of joints disorders (blockages, subluxations, dislocations) are increased. This implies installation precautions on the obstetric table, by mobilizing and positioning the hips with care to avoid their luxation. It is the same for the knees and the shoulders. If this occurs, replacing the joint is generally easy to perform, if necessary after intramuscular injections in the pain points ("trigger zones") found by gentle palpation (because the pain is very sharp) of the peri-joint muscles, a small amount (a few drops to half a milliliter depending on the volume of the muscle) xylocaine (10mg per ml) to relax them. For the same reasons, compression or stretching of superficial nerve trunks (ulnar / ulnar at the elbow, sciatic popliteal external / fibular joint at the knees), are possible, it is therefore necessary to protect these areas. Brachial plexus lesions (reversible) and scalene parade syndrome, are also observed, by muscle laxity, compression and stretching. Again, traction on the upper limbs to install the parturient, their position, especially if there are infusions, must be ensured to avoid risk positions.

- Hemorrhages. They represent the major risk. They are the consequence of the small vessels frailty and their difficulty in contracting to stop the bleeding. Platelets are involved in the formation of the platelet nail. They accompany perineal tears and cesareans. The blood clot formation can be late and sometimes significant in case of retained placenta, implying a quality uterine revision and postpartum monitoring. Their risk implies having possibilities of blood compensation.

- Epidural anesthesia. Alarmist opinions have propagated the idea that it was formally contraindicated because of the risk of meningeal (dural sheath) breach. This risk is very low and almost nonexistent in the history of parturients with EDS that we have followed. It is considerably reduced if you use fine, non-sharp needles, proceeding gently. In the event of a dural sheath breach, it can be blocked by the "blood patch" technique which consists of introducing blood into the meninges. It will be preferred to the attitude of waiting for spontaneous healing, which is the common fate (50%) of these breaches in the general population. Given the configuration of the tissues in EDS, we believe that the "blood patch" technique should be systematic if this incident occurs. The anesthetic effects of epidural are sometimes absent or incomplete (hemianesthesia for example), probably because of too rapid diffusion of the product in very "flexible" tissues. The solution is to re-inject more product. Importance of pain, which can be considerable at the time of work, sometimes longer, in these hyperalgesic women, imposes the resort to epidural. Phenomena of probably analogous cause are observed in general anesthesia: delay in induction, early awakenings.

- Cesarean section. It has been too easily and unnecessarily practiced by obstetricians wrongly concerned about major uterine risks (ruptures) but quite exceptional in EDS. In fact, caesarean section is contraindicated in the absence of dystocia. Bleeding, scarring difficulties with risk of disunity, frequent persistence of scar pains and its obstetric uselessness compel its reject in these parturients.

- Perineal tears must be repaired with non-absorbable wires, left in place long enough. They are not more frequent than in the general population (Professor Anne Gompel).

- The danger of anticoagulants. Hemorrhagic risk, generalized to all organs, is very important in these patients. The usual indication is phlebitis whose diagnosis can be difficult in a person with Ehlers-Danlos syndrome, due to the frequency of calves’ pain and the possibility of lower limbs edema, and ultrasound images are difficult to interpret. If decision is made to place the patient on anticoagulant therapy, it must be adapted so that it can be easily interrupted.

- False accusations of mistreatment and withdrawals of children. You should also know that newborn is very fragile, especially if his mother did not take vitamin D, during pregnancy, to compensate for the deficiency, often important, linked to a manufacturing deficit found in almost all cases of this pathology. Hemorrhages (bruises or even cerebral or retinal hemorrhages) can be observed in the absence of brutal gestures. Failure to understand the diagnosis too often leads to false accusations of parents’ violence. The consequence could then be the worst abuse that can be done to a child: to take him away from his parents. Ignorance of the diagnosis by pediatricians, social workers and judges for children favors this type of situation with considerable difficulties in recovering the child as was widely demonstrated during the 5th colloquium on Ehlers-Danlos disease, october 22, 2019 at the National Veterinary School of Maisons-Alfort.