Surgical biopsy with histological identification of the vascular or lymphatic proliferation within a generous section of the affected bone is an essential component in the diagnostic process.Recognition of the disease requires a high index of suspicion and an extensive workup. Because of its serious morbidity, Gorhams must always be considered in the differential diagnosis of osteolytic lesions. Management
Treatment of Gorhams disease is for the most part palliative and limited to symptom management.Sometimes, the bone destruction spontaneously ceases and no treatment is required, but when the disease is progressive, aggressive intervention may be necessary. Duffy and colleagues reported that around 17% of people with Gorhams disease in the ribs, shoulder, or upper spine experience extension of the disease into the chest, leading to chylothorax with its serious consequences, and that the mortality rate in this group can reach as high as 64% without surgical intervention. A search of the medical literature reveals multiple case reports of interventions with varying rates of success as follows:Cardiothoracic (heart and lung):

Skeletal:

To date, no known interventions are consistently effective for Gorhams, and all reported interventions are considered experimental treatments, though many are routine for other conditions. Some people may require a combination of these approaches. Unfortunately, some people will not respond to any intervention. Epidemiology
Gorhams disease is extremely rare and may occur at any age, though it is most often recognized in children and young adults. It strikes males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.
This is known as the Adams forward bend test and is often performed on school students. If a prominence is noted, then scoliosis is a possibility and an X-ray may be done to confirm the diagnosis. As an alternative, a scoliometer may be used to diagnose the condition.When scoliosis is suspected, weight-bearing, full-spine AP/coronal (front-back view) and lateral/sagittal (side view) X-rays are usually taken to assess the scoliosis curves and the kyphosis and lordosis, as these can also be affected in individuals with scoliosis. Full-length standing spine X-rays are the standard method for evaluating the severity and progression of scoliosis, and whether it is congenital or idiopathic in nature. In growing individuals, serial radiographs are obtained at 3- to 12-month intervals to follow curve progression, and, in some instances, MRI investigation is warranted to look at the spinal cord. An average scoliosis patient has been in contact with around 50-300mGy of radiation due to these radiographs during this time period. The standard method for assessing the curvature quantitatively is measuring the Cobb angle, which is the angle between two lines, drawn perpendicular to the upper endplate of the uppermost vertebra involved and the lower endplate of the lowest vertebra involved. For people with two curves, Cobb angles are followed for both curves. In some people, lateral-bending X-rays are obtained to assess the flexibility of the curves or the primary and compensatory curves.Congenital and idiopathic scoliosis that develops before the age of 10 is referred to as early-onset scoliosis.