Fibrodysplasia ossificans progressiva: anesthetic management in complex orthopedic spine procedures

Gorji, Reza; Li, Fenghua; Nastasi, Robert; Stuart, Sarah

doi:10.1016/j.jclinane.2010.11.004

Cited by 8 publications

(4 citation statements)

References 20 publications

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“…However, there are rare situations in the care of patients with FOP where operative treatment of fractures is the treatment of choice for conditions such as unstable spinal fractures, unstable appendicular fractures, or pending neurologic catastrophe. In fact, we are aware of two patients with FOP who sustained unstable fractures of the femoral diaphysis who underwent open reduction and internal fixation [7,29]. Both patients experienced postoperative flareups, substantial HO formed, and these patients lost movement of the ipsilateral hip and knee.…”

Section: Discussion Of Key Findingsmentioning

confidence: 99%

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility

Lindborg

Mukaddam²,

Baujat

et al. 2023

Clin Orthop Relat Res

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Background Fibrodysplasia ossificans progressiva (FOP) is an ultrarare genetic disorder with episodic and progressive heterotopic ossification. Tissue trauma is a major risk factor for flareups, heterotopic ossification (HO), and loss of mobility in patients with FOP. The International Clinical Council on FOP generally recommends avoiding surgery in patients with FOP unless the situation is life-threatening, because soft tissue injury can trigger an FOP flareup. Surprisingly little is known about flareups, HO formation, and loss of mobility after fractures of the normotopic (occurring in the normal place, distinct from heterotopic) skeleton when treated nonoperatively in patients with FOP. Questions/purposes (1) What proportion of fractures had radiographic evidence of union (defined as radiographic evidence of healing at 6 weeks) or nonunion (defined as the radiographic absence of a bridging callus at 3 years after the fracture)? (2) What proportion of patients had clinical symptoms of an FOP flareup because of the fracture (defined by increased pain or swelling at the fracture site within several days after closed immobilization)? (3) What proportion of patients with fractures had radiographic evidence of HO? (4) What proportion of patients lost movement after a fracture? Methods We retrospectively identified 36 patients with FOP from five continents who sustained 48 fractures of the normotopic skeleton from January 2001 to February 2021, who were treated nonoperatively, and who were followed for a minimum of 18 months after the fracture and for as long as 20 years, depending on when they sustained their fracture during the study period. Five patients (seven fractures) were excluded from the analysis to minimize cotreatment bias because these patients were enrolled in palovarotene clinical trials (NCT02190747 and NCT03312634) at the time of their fractures. Thus, we analyzed 31 patients (13 male, 18 female, The institution of one or more of the authors (MA, RJP) has received, during the study period, funding from the Radiant Hope Foundation.

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Section: Discussion Of Key Findingsmentioning

confidence: 99%

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility

Lindborg

Mukaddam²,

Baujat

et al. 2023

Clin Orthop Relat Res

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“…Most of the difficulty with the airway management of patients with FOP arises from the effects of the disease on the temporomandibular joints (TMJs) and the cervical spine. 3,18,19 Ankylosis of the TMJs is common in FOP 3,5,14 and overstretching of the jaw may cause enough trauma to the TMJs to result in localised flare-up and further ossification. 5 Neck stiffness may already be present at an early age in children with FOP and usually precedes heterotopic bone formation.…”

Section: Airway Managementmentioning

confidence: 99%

“…22,23 Ankylosis of the TMJs and the cervical spine may make the insertion of supraglottic airway devices as well as video-assisted and direct laryngoscopy impossible. 3,18,22 Forceful manipulation of the upper airway should always be avoided, as this may induce further flare-ups. 5 Tumolo et al 6 therefore recommend that direct laryngoscopy should not be performed during nonemergency airway management in patients with FOP, even if mouth opening is adequate.…”

Section: Airway Managementmentioning

confidence: 99%

“…32 The musculoskeletal deformities present in patients with FOP may also make it technically more difficult to insert needles with precision. 18,26 Furthermore, patients who have severe deformities should be positioned carefully on the operating table and pressure points should be padded to avoid soft tissue trauma. 6,24 Nutritional status Ankylosis of the TMJs may prevent mastication 3,6,8 and affected individuals may be restricted to soft or liquid diets.…”

Section: Vascular Access and Regional Anaesthesiamentioning

confidence: 99%

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Anaesthetic considerations in a child with fibrodysplasia ossificans progressiva

Kleyenstuber

2018

Southern African Journal of Anaesthesia and Analgesia

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Fibrodysplasia ossificans progressiva (FOP) is an extremely rare genetic disorder characterised by extraskeletal ossification of connective tissue. Affected individuals often become completely immobilised by their third decade of life. Amongst numerous anaesthetic concerns, the airway management of patients with FOP may prove to be the greatest challenge. This case report describes the anaesthetic management of a three-year-old girl with FOP and highlights the difficulties encountered during airway management.

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Heterotopic ossification on the volar surface of the distal radius in a child with fibrodysplasia ossificans progressiva: challenges in surgical excision of a rare condition

Şirin

Okay

Khalilov³

et al. 2021

Hand Surgery and Rehabilitation

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Fibrodysplasia ossificans progressiva: anesthetic management in complex orthopedic spine procedures

Cited by 8 publications

References 20 publications

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility

Anaesthetic considerations in a child with fibrodysplasia ossificans progressiva

Heterotopic ossification on the volar surface of the distal radius in a child with fibrodysplasia ossificans progressiva: challenges in surgical excision of a rare condition

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