Surgical management of ovarian endometrioma in a patient with fibrodysplasia ossificans progressiva: a case report

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare (prevalence ~ 1:2 million), devastating genetic disorder characterized by progressive heterotopic ossification. Crucially, surgical trauma is a potent trigger for catastrophic new bone formation, rendering any procedure exceptionally high-risk and generally contraindicated. The co-occurrence of FOP with a symptomatic ovarian endometrioma necessitating surgery represents an extraordinary clinical rarity. Critically, there exists a profound absence of published guidance on performing non-emergent, complex pelvic surgery like adnexectomy in FOP patients. This report details the first documented successful management of this unique comorbidity. Its significance lies in demonstrating the feasibility of achieving a safe outcome through innovative, tailored strategies: meticulous multidisciplinary planning, specialized surgical techniques (elective laparotomy, trauma-minimizing dissection/hemostasis), and rigorous perioperative care, providing a crucial management paradigm. CASE PRESENTATION: This article presents a highly uncommon case of concomitant FOP and ovarian endometrioma in a 27-year-old Asian female. The patient underwent pelvic ultrasound due to dysmenorrhea, revealing a large left adnexal cyst measuring 103 × 67 × 96 mm. Based on her medical history, clinical examination-characterized by significantly elevated CA125 and CA19-9 levels, along with imaging that indicated a cystic mass and pelvic heterotopic ossification-a diagnosis of ovarian endometrioma was established. Following comprehensive evaluation and preparation by a multidisciplinary team, the patient successfully underwent a transabdominal left adnexectomy and pelvic adhesion lysis under general anesthesia. Key aspects of the treatment strategy included: (1) selecting laparotomy over laparoscopy to accommodate the ankylosed position; (2) meticulous sharp dissection of adhesions supplemented by bipolar electrocoagulation for precise hemostasis, resulting in minimal intraoperative bleeding (5 ml); and (3) implementing targeted catheterization care, including positional adjustments to expose the urethra and stringent perineal care to prevent urinary tract infection. Postoperatively, the patient was transferred to the intensive care unit (ICU) for monitoring and received anti-infection therapy and fluid support with gradual dietary advancement. Vital signs remained stable, and she was discharged on the fifth postoperative day. CONCLUSION: This case detailed the surgical challenges, multidisciplinary collaboration model, perioperative meticulous care, and rapid recovery strategies for FOP patients with gynecological diseases, providing valuable practical experience for managing such rare and complex comorbidities.