Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
Health Design Lab, Thomas Jefferson University, Philadelphia, PA, USA.
Am J Case Rep. 2021 Jun 10;22:e931614. doi: 10.12659/AJCR.931614.
BACKGROUND Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of the connective tissue. Over time, patients with FOP experience decreased range of motion in the joints and the formation of a second skeleton, limiting mobility. Patients with FOP are advised to avoid any unwarranted surgery owing to the risk of a heterotopic ossification flare-up. For patients who do require a surgical procedure, a multidisciplinary team is recommended for comprehensive management of the patient's needs. CASE REPORT A 27-year-old woman with FOP underwent a hysterectomy for removal of a suspected necrotic uterine fibroid. To aid in presurgical planning and management, patient-specific 3-dimensional (3D) models of the patient's tracheobronchial tree, thorax, and lumbosacral spine were printed from the patient's preoperative computed tomography (CT) imaging. The patient required awake nasal fiberoptic intubation for general anesthesia and transversus abdominus plane block for regional anesthesia. Other anesthesia modalities, including spinal epidural, were ruled out after visualizing the patient's anatomy using the 3D model. Postoperatively, the patient was started on a multi-modal analgesic regimen and a course of steroids, and early ambulation was encouraged. CONCLUSIONS Patients with FOP are high-risk surgical patients requiring the care of multiple specialties. Advanced visualization methods, including 3D printing, can be used to better understand their anatomy and locations of heterotopic bone ossification that can affect patient positioning. Our patient successfully underwent supracervical hysterectomy and bilateral salpingectomy with no signs of fever or sepsis at follow-up.
纤维性骨发育不良进展性(FOP)是一种罕见的常染色体显性结缔组织疾病。随着时间的推移,FOP 患者会出现关节活动范围减小和第二骨骼形成,从而限制活动能力。由于异位骨化发作的风险,FOP 患者被建议避免任何不必要的手术。对于确实需要手术的患者,建议多学科团队综合管理患者的需求。
一名 27 岁女性患有 FOP,因疑似坏死性子宫肌瘤而行子宫切除术。为了辅助术前规划和管理,从患者的术前计算机断层扫描(CT)成像中打印出患者特定的气管支气管树、胸部和腰骶脊柱的 3 维(3D)模型。患者需要清醒鼻腔纤维光导插管全身麻醉和腹横肌平面阻滞进行区域麻醉。在使用 3D 模型观察患者的解剖结构后,排除了其他麻醉方式,包括脊髓硬膜外麻醉。术后,患者开始接受多模式镇痛方案和类固醇治疗,并鼓励早期活动。
FOP 患者是高风险手术患者,需要多个专业的护理。先进的可视化方法,包括 3D 打印,可以用于更好地了解他们的解剖结构和异位骨化的位置,这可能会影响患者的体位。我们的患者成功地接受了经宫颈子宫切除术和双侧输卵管切除术,随访时没有发热或败血症的迹象。