Surgical and Radiological Management of Complicated Uterine Leiomyoma Aided by 3D Models in a Patient with Fibrodysplasia Ossificans Progressiva
Unusual setting of medical care, Rare disease
Michelle Ho, Beau Y. Park, Norman G. Rosenblum, Mona Al Mukaddam, Frederick S. Kaplan, Victor Kucherov, Scott G. Hubosky, Gregory Kane, Vishal Desai, Michael R. Kramer, Bon S. Ku, Eric S. Schwenk , Jaime L. Baratta, Deepti Harshavardhana, Zvi Grunwald
Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
Am J Case Rep 2021; 22:e931614
Available online: 2021-05-10
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.
Keywords: airway management, Hysterectomy, Imaging, Three-Dimensional, Myositis Ossificans