Spinal Gorham-Stout syndrome: radiological changes and spinal deformities.

Area of Science:

• Radiology
• Orthopedics
• Rare Diseases

Background:

• Gorham-Stout syndrome (GSS) involving the spine is rare, with limited studies on its radiological features.
• Spinal GSS presents unique challenges in diagnosis and management due to its rarity.

Purpose of the Study:

• To detail the radiological changes and spinal deformities in patients diagnosed with spinal Gorham-Stout syndrome.
• To enhance understanding of the imaging characteristics and clinical presentation of spinal GSS.

Main Methods:

• Retrospective review of 11 consecutive spinal GSS patients (Jan 2005-Dec 2017).
• Utilized Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) for detailed spinal evaluation.
• Assessed spinal deformities using standing posteroanterior and lateral radiographs.

Main Results:

• CT revealed multi-level osteolytic lesions with adjacent fluid attenuation; MRI showed hyperintense T1- and T2-weighted signals.
• Kyphosis (63.6%) or scoliosis (36.4%) were the dominant deformities, averaging 94° for kyphosis.
• Deformities correlated with the most osteolytic segments; bracing showed stability, while spinal fusion had a high failure rate.

Conclusions:

• CT and MRI are essential for initial diagnosis and ongoing management of spinal GSS.
• Spinal GSS typically manifests as kyphosis or kyphoscoliosis, with deformity patterns closely mirroring osteolysis.
• Radiological findings are key to understanding and managing this rare spinal condition.