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P110 Regional anesthesia and procedural sedation for Ilizarov apparatus placement in a boy with Ehlers-Danlos syndrome – a case report

rapm · 2025-09-10 · canonical JSON source

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Background and Aims Ehlers-Danlos Syndrome (EDS) is a genetic disorder affecting collagen formation and function. Afflicted patients often require corrective orthopedic surgery, with perioperative complications being more common than in the standard population, most prominently postoperative pain and discomfort. Skeletal anomalies may precipitate difficult intubation, mechanical ventilation, or weaning from the ventilator. Regional anesthesia (RA) may present a suitable solution for such patients, even though there are numerous reports of its failure.Methods A 10 year old boy, weighing 17 kg and suffering from EDS Type 4, was admitted for elective Ilizarov apparatus placement on the lower leg. He had significant thoracic and lumbar kyphosis/scoliosis with associated cranial dysmorphia. On induction he received Midazolam, Fentanyl, and Ketamine, while sedation was maintained via continuous Propofol and spontaneous breathing. A femoral nerve block, popliteal block, and adductor canal block were performed with 2% Lidocaine (3 ml, 3.53 mg/kg) and 0.25% Levobupivacaine (13 ml, 1.88 mg/kg).Results Throughout the 2 h procedure the patient maintained perfect hemodynamic and respiratory stability. Awakening was uneventful, and NSAID were started prn 12 h after surgery. No opioids were used postoperatively.Abstract P110 Figure 1Thoracic deformity, and Ilizarov apparatusAbstract P110 Figure 2Popliteal block ultrasound image (popliteal artery - PA, sciatic nerve - IN)Abstract P110 Figure 3Adductor canal block ultrasound image (femoral nerve - FN, femoral artery - FA)Conclusions This case highlights the benefits of peripheral nerve blocks in patients with Type 4 EDS, potentially in all patients with significant skeletal anomalies of the head, neck, and thorax. There is still no consensus on local anesthetic resistance. As patients with EDS are unpredictably sensitive to opioids, by maintaining perfect intraoperative analgesia and spontaneous breathing we can bypass all potential complications. Additionally we can minimize the opioid effects on gastric emptying, which may also be delayed.