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

ABSTRACT: 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 N2O-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 [18F]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.

SUBMITTER: Botman E

PROVIDER: S-EPMC7472799 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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

Botman Esmée E Treurniet Sanne S Lubbers Wouter D WD Schwarte Lothar A LA Schober Patrick R PR Sabelis Louise L Peters Edgar J G EJG van Schie Annelies A de Vries Ralph R Grunwald Zvi Z Smilde Bernard J BJ Nieuwenhuijzen Jakko A JA Visser Marieke M Micha Dimitra D Bravenboer Nathalie N Coen Netelenbos J J Teunissen Bernd P BP de Graaf Pim P Raijmakers Pieter G H M PGHM Smit Jan Maerten JM Eekhoff Elisabeth M W EMW

Frontiers in endocrinology 20200821

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 a ...[more]

PMID: 32973683

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Project description:Background Breast cancer is the most common cancer in women. The tumor is prone to metastasize in the brain, lung, liver, bone, and other organs; however, generally, it is less likely to metastasize in the digestive tract. Thus, breast cancer metastasizes to colon is rarely happened and easily ignored by clinicians. Such misdiagnosis may lead to delay the further diagnosis and treatment of patients, or even cause the life danger of patients due to the progress of the disease. Therefore, we propose such case reports to arouse clinicians’ attention to the rare case of digestive tract metastasis after treatment of breast cancer. We also conducted a retrospective analysis of the relevant case reports. We suggest that because breast cancer with gastrointestinal metastasis rarely occurs, and because of the lack of specificity of syndromes, it is easily misdiagnosed. Thus, the attention of the receiving doctor needs to be drawn to this tumor. We also summarized the specificity and sensitivity of the commonly used immunohistochemical detection indicators of digestive tract metastasis of breast cancer. Case Description We presented a 67-year-old female went to hospital because of “acute pain in the right lower abdomen”, after computed tomography (CT) examination, the patient was diagnosed as “acute appendicitis” and underwent laparoscopic appendectomy (LA), the post-surgery pathology confirmed metastatic carcinoma to the appendix from the breast that was removed 10 years early. The patient first came to our hospital 10 years ago because of the right breast malignant tumor. After modified radical mastectomy for right breast cancer, the patient underwent chemotherapy for 6 cycles. Tamoxifen endocrine therapy was administered after chemotherapy. The patient had a regular follow-up, with no signs of distant metastasis and local recurrence. Conclusions Metastasis from breast cancer to the gastrointestinal tract is extremely rare. For patients with breast cancer, endoscopy should be adopted as a routine follow-up item. For suspicious lesions found under endoscopy, immunohistochemistry stain should be adopted to ensure the diagnosis. For lesions that are confirmed to be metastatic from breast cancer, proper treatment should be carried out based on patients’ condition to improve the prognosis.

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

Publications

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

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