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. 2016:2:25.
doi: 10.1051/sicotj/2016015. Epub 2016 May 16.

Gorham-Stout syndrome of the shoulder

Ulrich Brunner  1 , Kilian Rückl  2 , Christian Konrads  2 , Maximilian Rudert  2 , Piet Plumhoff  2
Affiliations

Gorham-Stout syndrome of the shoulder

Ulrich Brunner et al. SICOT J. 2016.

Abstract

Introduction: Gorham-Stout syndrome (GSS) is a rare but severe subtype of idiopathic osteolysis. There are no guidelines for the treatment of GSS. We analysed different diagnostic and therapeutic regimes and we describe the sucessful treatment of GSS considering individual patient factors.

Methods: We diagnosed three patients with shoulder-specific GSS using clinical, radiological and histopathological examinations. Two out of three patients with similar clinical appearances were treated non-operatively. One patient was treated by reverse shoulder arthroplasty. All patients were analysed retrospectively using clinical and radiological evaluation with a mean follow-up of 42 (range 30-50) months.

Results: Two patients had few symptoms of GSS and were treated conservatively. One patient underwent arthroplasty, with a good clinical result. No additional therapy, such as radiation or anti-resorptive medications, was needed for the stable fixation of the prosthesis and the termination of osteolysis. In all patients we found good clinical outcomes with high patient satisfaction.

Discussion: GSS is diagnosed after exclusion of infectious, malignant, and systemic disorders. The diagnosis should be supported by clinical, radiological, and histopathological characteristics of patients. Different humoral and cellular changes have been reported in GSS, but lack sufficient supporting evidence. GSS is associated with angiomatous and lymphatic malformations. The changes in GSS and the theories of its pathophysiology may reveal.

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Figures

Figure 1.
Figure 1.
Left shoulder photographs of an 84-year-old female (patient 1) suffering Gorham-Stout syndrome in April 2011: the original contour of the shoulder vanished completely.
Figure 2.
Figure 2.
Antero-posterior radiographs of the involved shoulder of each of the three patients over time: (a) progressive osteolysis in all three patients; (b) implanted reverse shoulder prosthesis without loosening 12 months postoperatively.
Figure 3.
Figure 3.
CT and MRI of the left shoulder (patient 1): massive osteolysis and tumour. Multiple similar calcifications were found in all three patients in CTs.
Figure 4.
Figure 4.
Bone scan (patient 1): excessive accumulation in the mineralization phase in the left shoulder.
Figure 5.
Figure 5.
Overview of the existing theories regarding pathomechanism in Gorham-Stout syndrome. Broken lines and grey arrows show theoretical considerations still lacking proof. Role of trauma still remains uncertain. Elevated levels of Il-6 and differentiation factors Il-1β and RANKL may stimulate osteoclasts and lead to bone resorption while PDGF-BB, IL-8, and VEGF-A may activate endothelial cells and others via CD105 and TGF-beta1-receptor. This may result in vascular malformation and lymphangiomatosis. Both are characteristics of Gorham-Stout syndrome.
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References

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