Poster Presentation: Anesthetic Management in Fibrodysplasia Ossificans Progressiva: A Case Report-Anesthetic Management in Fibrodysplasia Ossificans Progressiva: A Case Report

Anesthetic Management in Fibrodysplasia Ossificans Progressiva: A Case Report

Pdf Summary

Fibrodysplasia ossificans progressiva (FOP) is a rare, disabling genetic disorder affecting 1 in 1-2 million individuals globally. Characterized by progressive heterotopic ossification (HO) of muscles, fascia, tendons, and ligaments, it typically begins around age 10. HO can be spontaneous or triggered by minor trauma, leading to progressive immobility and a median life expectancy of 40 years. There is no cure, and individuals are often wheelchair-bound by age 30. Death commonly results from thoracic insufficiency syndrome or pneumonia.<br /><br />The study reviews anesthetic management for FOP patients, particularly evaluating 10 relevant case reports and integrating guidelines from the International Clinical Council on FOP. It outlines a case of a 53-year-old male with FOP undergoing robotic bilateral inguinal herniorrhaphy, highlighting several challenges such as limited mobility, restricted cervical spine movement, minimal mouth opening, and reduced thoracic cage expansion. An awake nasal fiberoptic intubation was successfully employed, and intravenous hydrocortisone was administered intraoperatively and continued postoperatively. The patient experienced postoperative atelectasis but was discharged home on oxygen after eight days.<br /><br />Anesthesia recommendations for FOP patients include general anesthesia due to airway challenges, with awake nasal fiberoptic intubation considered optimal. An otolaryngologist should be present for emergent airway management. Measures to avoid tissue injury, such as using pillows and minimizing tourniquet time, are crucial. Medications should favor acetaminophen and NSAIDs over opioids to avoid respiratory depression. Regional anesthesia techniques are generally avoided due to risks, though some ultrasound-guided blocks like TAP and femoral-sciatic have been used without reported HO formation.<br /><br />The study concludes that FOP presents significant anesthetic challenges, and further research on the utility of ultrasound-guided blocks is needed to improve management strategies.

Keywords

Fibrodysplasia ossificans progressiva

FOP

heterotopic ossification

anesthetic management

awake nasal fiberoptic intubation

thoracic insufficiency syndrome

robotic bilateral inguinal herniorrhaphy

airway challenges

ultrasound-guided blocks

International Clinical Council on FOP