When Limb Surgery Has Become the Only Life-Saving Therapy in FOP: A Case Report and Systematic Review of the Literature.

Autor: Botman E; Department of Internal Medicine Section Endocrinology, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Treurniet S; Department of Internal Medicine Section Endocrinology, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Lubbers WD; Department of Anesthesiology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Schwarte LA; Department of Anesthesiology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Schober PR; Department of Anesthesiology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Sabelis L; Department of Rehabilitation Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Peters EJG; Department of Internal Medicine Section of Infectious Diseases, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., van Schie A; Department of Radiology and Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., de Vries R; Medical Library, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Grunwald Z; Department of Anesthesiology, Jefferson Health System, Thomas Jefferson University, Philadelphia, PA, United States., Smilde BJ; Department of Internal Medicine Section Endocrinology, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Nieuwenhuijzen JA; Department of Urology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Visser M; Department of Neurology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Micha D; Department of Clinical Genetics, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Bravenboer N; Department of Clinical Chemistry, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Coen Netelenbos J; Department of Internal Medicine Section Endocrinology, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Teunissen BP; Department of Radiology and Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., de Graaf P; Department of Radiology and Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Raijmakers PGHM; Department of Radiology and Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Smit JM; Department of Plastic, Reconstructive and Hand Surgery, Amsterdam Bone Center, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Eekhoff EMW; Department of Internal Medicine Section Endocrinology, Amsterdam Bone Center, Amsterdam Movement Sciences, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands.
Jazyk: angličtina
Zdroj: Frontiers in endocrinology [Front Endocrinol (Lausanne)] 2020 Aug 21; Vol. 11, pp. 570. Date of Electronic Publication: 2020 Aug 21 (Print Publication: 2020).
DOI: 10.3389/fendo.2020.00570
Abstrakt: Fibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks. We report a patient with FOP suffering from life-threatening antibiotic resistant bacterial infected ulcers of the right lower leg and foot. The anesthetic, surgical and postoperative challenges and considerations are discussed. In addition, the literature on limb surgeries of FOP patients is systemically reviewed. The 44 year-old female patient was scheduled for a through-knee amputation. Airway and pulmonary evaluation elicited severe abnormalities, rendering standard general anesthesia a rather complication-prone approach in this patient. Thus, regional anesthesia, supplemented with intravenous analgosedation and N 2 O-inhalation were performed in this case. The surgery itself was securely planned to avoid any unnecessary tissue damage. Postoperatively the patient was closely monitored for FOP activity by ultrasound and [ 18 F]PET/CT-scan. One year after surgery, a non-significant amount of HO had formed at the operated site. The systematic review revealed seventeen articles in which thirty-two limb surgeries in FOP patients were described. HO reoccurrence was described in 90% of the cases. Clinical improvement due to improved mobility of the operated joint was noted in 16% of the cases. It should be noted, though, that follow-up time was limited and no or inadequate imaging modalities were used to follow-up in the majority of these cases. To conclude, if medically urgent, limb surgery in FOP is possible even when general anesthesia is not preferred. The procedure should be well-planned, alternative techniques or procedures should be tested prior to surgery and special attention should be paid to the correct positioning of the patient. According to the literature recurrent HO should be expected after surgery of a limb, even though it was limited in the case described.
(Copyright © 2020 Botman, Treurniet, Lubbers, Schwarte, Schober, Sabelis, Peters, van Schie, de Vries, Grunwald, Smilde, Nieuwenhuijzen, Visser, Micha, Bravenboer, Coen Netelenbos, Teunissen, de Graaf, Raijmakers, Smit and Eekhoff.)
Databáze: MEDLINE
Externí odkaz: