Anesthetic considerations for Myositis Ossificans Progressiva: a case report

Abstract

Background: Fibrodysplasia Ossificans Progressiva (FOP), or Myositis Ossificans Progressiva (MOP), is a rare disease that affects approximately 1 in every 2 million people. This hereditary condition is defined by the progressive, abnormal, and widespread ossification (i.e. formation of bone that is not normally present) of muscular and soft connective tissues (i.e. tendons and ligaments). Pathophysiology of the condition is unknown, though mutations of the ACVR1 gene have been identified and associated with the condition.

Case presentation: A 25-year-old male with a history of spinal trauma, along with 3 years of pain in the left hip, was studied. Diagnostic myositis ossificans were confirmed and subclassified as type B/C along with elevated inflammatory markers and mild anemia. A lump was detected in the left hip joint during the physical assessment.

Investigations: Heterotopic ossification around the hip joint was apparent on the X-ray and MRI scans. Full anesthetic preparation was required with a pre-operative assessment as the patient was expected to have airway problems concerning restricted neck movement.

Management: During the spinal anesthesia and the twelve-minute surgery (excision of Myositis), the patient was under close monitoring with Midazolam and Atropine prescribed to maintain stability in the hemodynamic parameters.

Conclusion: The potential complications of ectopic bone formation, coupled with the possibility of impaired respiratory function, necessitate extremely careful consideration of anesthesia and airway management for patients with FOP. The present case study, while making a case for the need for future research aimed at defining guidelines for anesthetizing patients with FOP, also underscores the need for individualized anesthesia in these very challenging patients.