Gorham’s disease: treatment with an autologous iliac bone graft and a reverse total shoulder arthroplasty
Gorham’s disease (GSD) is a rare osteolytic disease with unclear etiology, and no known prevention or effective treatment. Here we report a new surgical treatment for a case of GSD in September 2017.
We report GSD in a 52-year-old woman. She had disappearance of her humeral head and a defect of the glenoid bone in her left shoulder joint, which were serious obstacles to joint function. We used an autologous iliac bone graft to repair the glenoid bone defect and a reverse total shoulder arthroplasty. After surgery, humeral osteolysis did not continue, and her shoulder function recovered well.
This case suggests that autologous bone grafting can still be used to treat GSD despite it being an osteolytic disease. The successful treatment suggests that this method could be used for GSD in other bones.
KeywordsGorham’s disease Autologous iliac bone graft Reverse total shoulder arthroplasty Shoulder joint function
Gorham’s disease (GSD) was first reported by Jackson in 1838 then later in 1872 [1, 2]. In 1955, Gorham and Stout defined a specific disease entity from a review of 24 cases in the literature . GSD can affect any bone in the body, but it has a predilection for bones of the shoulder and pelvic girdle [4, 5, 6, 7]. It presents as progressive idiopathic osteolysis of one bone or contiguous bones around one focus, without respect for joint boundaries [3, 4, 5, 6], and it may arrest spontaneously. Several methods have been reported to deal with this disease in the literature, such as radiotherapy, pharmaceuticals, complete resection, or a custom-made prosthesis to replace the resected bone and joint [8, 9, 10, 11, 12, 13, 14].
We report a case of GSD with extremely rapid progression of idiopathic osteolysis in the proximal humerus and glenoid in only 2 months . It was treated with a reverse total shoulder arthroplasty with simultaneous reconstruction of the glenoid bone defect using autologous iliac bone grafts. The shoulder joint function recovered well 6 months after surgery.
Discussion and conclusions
GSD is an unusual nonmalignant condition characterized by spontaneous and massive osteolysis. More than 300 cases of GSD have been described in the literature, but the underlying cause remains unknown [8, 9].
Various strategies are used to treat GSD [9, 10, 11], but in the present case it was important to preserve the function and shape of the shoulder joint. In particular, the problem of osteolysis had to be overcome, and the defect of the glenoid bone using autologous or allogeneic bone had to be considered. Surgery is a suitable treatment to reduce or stop the progression of GSD, but conservative treatment could result in loss of shoulder function or even amputation. In this case, the patient had extensive and rapid involvement of the glenoid and proximal humerus. We chose replacement prosthesis of the shoulder rather than amputation to preserve the function of the shoulder joint. Fortunately, the operation not only retained this function but also stopped the osteolysis damage. Up to now, the autologous bone graft was integrated with the shoulder glenoid and prosthesis, and no prosthesis loosening occurred. The patient was very satisfied with the treatment outcome.
Using an autologous bone graft for the reconstruction of the shoulder glenoid was a critical step in the present case that provided a basis for the installation of the shoulder prosthesis. This method of operation has important clinical significance and provides an operative reference for the treatment of GSD or similar conditions. The cause of GSD, duration of osteolysis, and the identification of factors that prevent or slow down development of the disease require further study.
We thank the patient for agreeing to publication of her case, and the Departments of Pathology and Radiology, North China University of Science and Technology Affiliated Hospital. We also thank Sarah Williams, PhD, from Liwen Bianji, Edanz Group China (www.liwenbianji.cn), for editing the English text of a draft of this manuscript.
No funding was obtained for this study.
Availability of data and materials
All data generated or analyzed during this study are included in this published article.
LJ, XQ, and WG analyzed and interpreted the patient data. YP and WG performed the operation. LJ and WG undertook patient rehabilitation. All authors read and approved the final manuscript.
Ethics approval and consent to participate
All procedures were part of the standard medical care, and the need for ethics approval and consent to participate was waived.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the consent form is available for review by the Editor of this journal.
The authors declare that they have no competing interests.
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