Gorham’s disease
Gorham’s disease, is a disorder in which one or more bones are progressively replaced by tissue consisting of lymph vessel tumours (lymphangiomatous tissue) [1] . The bone loss is often associated with painful swelling or abnormal growth of blood vessels [2]. Normal bone is renewed by a constant cycle of bone destruction and bone regrowth; in Gorham’s disease patients this regrowth process does not take place correctly.
Gorham’s disease is also known as ‘Vanishing Bone Disease’, massive osteolysis and Gorham-Stout disease [2]. It usually affects younger people. It is not known to be more common in one gender or the other, nor in any particular race. [3]
Signs and symptoms
The symptoms depend on the part of the body affected: this can include the hand, arm, shoulder, ribs, pelvis, thigh bone or jaw [2]. Fractures can be common, as can persistent pain in the part of the body that is affected.
Diagnosis
Gorham’s disease is commonly diagnosed after a bone fracture. There is a classic appearance on x-rays showing lowered bone density; MRI scans are less useful for diagnosis [2]. The skin in the affected area may have a damaged or odd appearance (skin lesions), which can be an indication.
Other diseases that may be related
Lymphangiomatosis is a disorder that refers to lymph vessel tumours growing in soft tissues and can also be used to describe lesions in bone. It is not clear whether lymphangiomatous bone lesions should always be described as Gorham’s disease or not. Many patients have both diseases. However, not all patients diagnosed with lymphangiomatosis have Gorham's and not all patients diagnosed with Gorham's have lymphangiomatosis.
Treatment
While bone is still growing in the affected area, doctors may advise no treatment [2]. If treatment is advised, then many different options have been tried, including [3]:
Different types of surgery may help to improve the functional use of the affected area, and/or improve the appearance [2].
- Bone grafts
- Artificial aids and bone replacements (prostheses)
- Radiotherapy, though some texts suggest caution is necessary here [2] [4]
Prognosis
There is a great deal of variation in how Gorham’s disease develops in different patients. The disease sometimes regresses without any obvious cause. However, if the vital structures of the body are affected, like the support for the lungs, then it can be much more serious. Disability is rare, but depends on the parts of the skeleton that are affected. [3]
Research
Very little is known of these diseases and no formal research has been conducted in patients with this diagnosis. Case reports are the primary format of all publications regarding both lymphangiomatosis and Gorham's disease. The number of patients is not known, as not all patients have case reports written about them.
History
Gorham’s disease was first described in 1838. It obtained the name from a case report by Gorham and Stout in 1955. [2]
References
- Pediatric chest imaging: chest imaging in infants and children ; with 19 tables. Celesino Aso. Springer 2008.
- Radiotherapy for Non-Malignant Disorders: Contemporary Concepts and Clinical ... By H. M. Seegenschmiedt, Michael H. Seegenschmiedt, Hans-Bruno Makoski, Klaus-Rudiger. 2007
- Orthopaedic Pathology By Vincent J. Vigorita, Bernard Ghelman, Douglas Mintz. 2007.
- Radiation Therapy of Benign Diseases: A Clinical Guide By Stanley E. Order, Luther W. (FRW) Brady, Sarah S. Donaldson, H. P. (FRW) Heilmann. 2003.
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