Surgical Management of Gorham-Stout Disease of the Pelvis Refractory to Medical and Radiation Therapy
Gorham-Stout disease (GSD) is a rare condition characterized by spontaneous idiopathic bone resorption that can affect any part of the skeleton. Treatment is aimed at halting osteolysis and alleviating complications associated with bone loss. Often this can be achieved via observation and supportive management, medical treatment, and/or radiation therapy.
We report a case of GSD of the pelvis that was refractory to medical and radiation therapy and was managed successfully with surgery. A 30-year-old man presented to our clinic 3 years after being diagnosed at an outside institution with GSD of the pelvis that was managed with medical treatments and radiation therapy. Despite aggressive, multimodality treatment, he was unable to ambulate without crutches and was in significant pain. The patient opted for intralesional surgery and spinopelvic fusion. Sixteen months after surgery, the patient had only mild pain and was able to ambulate with a cane.
Very few cases have been reported of GSD involving the pelvis that necessitated surgical management. Significant functional impairment can occur as a result of pelvic osteolysis, and traditional management strategies focused on halting resorption may not be adequate. Surgical stabilization of the affected areas is an important treatment strategy for patients who have exhausted other options.
We present a case of a patient who continued to deteriorate after maximal medical and radiation therapy. Many reported cases of pelvic GSD have had good outcomes with some combination of conservative management, medical therapy, and radiation. However, in our patient, the pelvis and lumbosacral spine were unstable as a result of significant bone loss and fracture, and his clinical deterioration was dramatic. We considered reasonable surgical approaches, including local intralesional débridement and massive en bloc resection with structural allograft. We chose the less radical procedure given the patient’s age, minimal surgical history, and personal preference. Although structural pelvic allograft has been successful in a few cases, there remains a high risk of complications, such as fracture, resorption, or infection.17 We considered the addition of hip arthroplasty with either scenario, but we elected not to perform this component given his young age and lack of symptomatic improvement with diagnostic anesthetic hip injection. The key to this patient’s surgical reconstruction, aside from eliminating gross disease, was the stabilization of the spinopelvic junction and pelvic ring. His functional improvement as early as 6 weeks after surgery demonstrates that surgery can have an important role for patients with pelvic GSD who fail medical and radiation therapy.
