Ehlers Danlos Syndrome and Anesthesia

Anesthesia for EDS patients is a little risky, and little evidence-based knowledge exists about it. Therefore, a registry of perioperative problems may help enhance anesthesia induction and perioperative medication safety in patients with EDS. However, proper preparedness, organizational and technical resources can be provided for safe anesthesia. An anesthetic must acquire a prospective anesthetic diagram in every EDS patient before making a realistic risk assessment about airway problems, bleeding issues, neuraxial procedures, and complication rates.

Anesthesia types and their effect on EDS patients

Regional anesthesia & local anesthesia

For general or regional/local anesthesia, there is no clear recommendation for EDS patients. The Orphanet UK Vascular EDS Emergency Guideline explicitly requires that neuraxial blockage be avoided.

However, multiple cases and series of adequate spinal and epidural anesthesia in both vascular EDS and other subtypes of patients. This could be due to an underreporting error and should not be seen as a general principle. Many trials are required to assess a possibly higher risk of perforation, nerve injury, or blood formation for tissue-fragile patients and an increased risk of bleeding. This topic should be discussed with the patient concerning the individual concerns (e.g., EDS subtype, bleeding diathesis, spinal pathology, a history of hematoma or spontaneous organ breakage) (early mobilization, pain control, the wish for awareness at cesarean delivery). A higher Headache post-dural puncture (PDPH) rate after neuraxial blockade may be expected because of tissue fragility, while no relevant research is available. A peridural blood patch can stop PDPH. With theory, this method is also available in EDS sufferers. In addition, EDS tissue fragility subtypes are associated with a significant prevalence of spontaneous dural ruptures with ensuing PDPH-like Headache. A peridural blood patch may be a less invasive solution in these specific, highly symptomatic individuals. It can be addressed between neurologists, neurosurgeons, and anesthetists (as most anesthetists regularly experience performing blood patches due to PDPH).

In patients with vascular EDS, neuraxial blockages are avoided because of a lack of apparent benefits and potential hazards compared to the anticipated general anesthesia. Neuraxial blockages are feasible in various EDS forms. However, a complete discussion of the potential advantages and risks should be conducted with the team and the unique patient.

Use of MRI in anesthetic EDS patients

Pre-operative ultrasound imaging (or the use of MRI) could assist exclude appropriate spinal pathologies in specific individuals planned for neuraxial blocking. There are limited reports of peripheral regional anesthesia in EDS, as indicated above. Nevertheless, in several EDS subgroups, the incidence of problematic airway status may imply peripheral regional anesthetic during limb surgery. As with neuraxial blockade, careful history and examination are essential. Theoretically, tissue scarring can hinder the transmission of local anesthetics. Ultrasound guidance is advised to reduce the incidence of arterial puncture and to improve local anesthetic deposition.

With regard to bleeding risk, peripheral nerve blockages in patients with vascular EDS are not recommended. Peripheral nerve blocks can be notified on a case-by-case shared-decision policy in patients with different EDS subtypes. Topical local anesthesias such as EMLA, or local infiltration anesthetic, proven by a clinical trial utilizing EMLA cream in EDS patients and healthy checks, could prove ineffectual. This may be important for youngsters to use EMLA to assist painless venous puncture.

Obstetrical anesthesia

There is no agreement on an optimal way of delivery as uterine rupture and postponed wound healing may complicate both vaginal and cesarean section. Severe bleeding must always be anticipated, especially in patients with vascular fragility. An elective cesarean section is ideal for those at high risk. It should be explored how to cross-match RBCs, utilization of autotransfusion systems, and DDAVP for prophylaxis. Episiotomy with pelvic prolapse is associated with hypermobile EDS and should be avoided. Instead of vaginal birth episiotomy, a cesarean section is preferable. There is no overall guideline for the distribution method of certain EDS forms covered elsewhere in detail. The advantages of the neuraxial blockade should be evaluated against the higher hazards in the individual EDS subtypes as described above.

In patients with bleeding hazards, such as in vascular EDS, some doctors favor general anesthesia. Nonetheless, neuraxial regional anesthesia (spinal and peridural anesthetic procedures) in vascular EDS should be avoided. In patients with a high risk of neuraxial problems, the use of patient-controlled remifentanil instead of neuraxial blockade may be a pragmatic strategy. Using inhalative 50% nitrous oxide in several countries is a common option to labor analgesia. However, because spontaneous pneumothorax is at risk in certain EDS patients, this treatment strategy should only be employed on a case-by-case basis.


Postoperative care of EDS patients after anesthesia

Careful positioning and mobilization of the patient are recommended to limit the danger of joint luxation and the lowering of shear pressures regarding skin fragility. Early mobilization is necessary for all EDS subtypes to prevent strength loss in the musculoskeletal and cardiovascular systems, particularly in the hypermobile subsystem. Patients should be closely monitored for bleeding and hematomas at the surgical site. In the first postoperative hours, POTS patients should be observed for cardiovascular symptoms unstable. Adequate postoperative nausea and vomiting (PONV) prevention is indicated due to vomiting with vascular EDS; spontaneous oesophageal rupture is observed.

Ambulatory anesthesia

All EDS patients should be operated in competent centers. Bleeding (including developing body disorders, etc.) should be anticipated with suspected or evident vascular fragility. Even after minor surgery, all patients should be observed for at least 24 hours.

Perioperative emergencies

Specific acute EDS scenarios such as vascular dissection (e.g., aortic dissection, peripheral arteries, and veins) occurring either spontaneously or iatrogenically should be known to all PACU and ward nurses, as should all EDS patients (especially during angiographic interventions). Pressure and shear stresses and bleeding can result in vascular puncture or rupture, which results in compartment syndromes and other complications. It is possible to experience uncontrollable bleeding from the surgical site or organ rupture (vascular EDS). Positive pressure ventilation and central venous access are associated with a pneumo- (hemo-) thorax risk, which must be expected. Furthermore, spontaneous rupture or rupture following trivial damage to the intestine, uterus, esophagus, or vagina has been described in some cases as well. This risk is increased in the postoperative period, and it can develop at sites that are distal to the place where the surgery was performed. All surgical team members should be aware of these possibilities throughout the postoperative period to minimize a delay in identification when odd symptoms arise after the procedure. In individuals with EDS other than vascular EDS, these outcomes are uncommon; hence, even elective vascular, gastrointestinal, and other surgical procedures should be avoided whenever possible in this patient population.

Possible anesthetic complications

Careless patient posture may increase the likelihood of developing brachial plexus neuropathy or experiencing sight loss due to direct pressure to the eye. It is possible to sustain skin injury and hematoma formation due to shear stresses and inadequate patient cushioning. Atlantooccipital instability can cause airway issues, and there is a risk of temporomandibular joint luxation when mask ventilation or intubation is performed on patients with this condition. Intubation attempts may be made more difficult by scoliosis and other forms of spinal disease. In patients with vascular EDS, spontaneous pneumothorax can occur due to mechanical breathing, and upper airway hemorrhage can occur due to repeated intubation efforts. Predural puncture headache (PDPH) or an increased incidence of epidural hematoma, as previously stated, may be associated with certain genetic factors. Both of these concerns should be shared with the client.

Nonetheless, this recommendation is based on professional judgment. There is also a case series of patients who experienced spontaneous CSF leaks and had high rates of underlying EDS to support this recommendation. Only one case of post-dural puncture headache (PDPH) has been reported in the published literature in patients with neuraxial blockade (mainly obstetrical anesthesia). That was in a patient with vascular EDS. Prospective studies are required for assessing the risk of PDPH and bleeding complications in patients with EDS who have neuraxial blockade syndrome.

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