Synovial chondromatosis

Gelenkchondromatose is a rare orthopedic disease by up to one hundred individual chondromas (benign cartilage growths ) in the synovial membrane - the membrane lining the joint capsule - is marked. Ossification of the chondroma, one speaks of a synovial osteochondromatosis.

With the single name Chondromatosis a disease is called, in which as many findings chondromas (as En or Ekchondrome ) in the bone that can demonstrate the joints or other anatomical structures.

Synonyms

• Synovial Chondrometaplasie
• Reichel 's syndrome, according to Paul Friedrich Reichel (1858-1934)
• Henderson -Jones syndrome, according to Melvin Starkey Henderson (1883-1964) and Hugh Toland Jones ( 1892 -? )

Reichel reported on this disease for the first time in 1900 in the Archives of clinical surgery in his work " chondromatosis of the knee joint capsule ." Henderson described it in 1918, Jones 1924.,

Causes, incidence, symptoms

The causes and exact mechanisms that lead to the emergence of a synovial chondromatosis, are not yet known. We distinguish a primary from a secondary synovial chondromatosis. For the formation of the secondary form a chronic irritation or repetitive microtrauma ( small injuries ) can be assumed the capsule membrane, so that there is a metaplastic transformation of mesenchymal cells into cartilage circumscribed areas ( cartilage corpuscles ).

Also in orthopedic specialty clinics is a rarely seen disease. Exact figures on the incidence of the disease can therefore not be specified. In men, the disease is twice as common as in women occurs only sporadically in children.

Most often the Gelenkchondromatose in the large joints occurs ( knee, hip and shoulder joints ). Rarely the elbow, very rarely the temporomandibular joint, other joints, bursae, and tendon sheaths are affected.

The patients are often relatively long symptom-free. Finally, repeated impaction, mobility disability or pain caused by loose bodies, and synovial chondroma, and the possible damage to joint structures by abrasion (secondary osteoarthritis ) require clarification.

Diagnosis, therapy

The synovial chondromatosis or Clarification is done by means of conventional radiography and MRI. The diagnosis is made in the synopsis of clinical, radiological and histological findings. As feared complication the emergence of a secondary chondrosarcoma is seen.

In a larger study of 53 cases, which covered a period of 30 years, a chondrosarcoma was diagnosed in 3 cases, bringing a risk of over 5 % was calculated.

Sperling and employees cite a work of Blokx and employees, after a total of 34 chondrosarcomas associated with a synovial chondromatosis have been described in the literature up to the year 2000.

Problems arise not only by the possibility of malignant transformation, but also the fact that both diseases are extremely rare and a chondrosarcoma may falsely be diagnosed as a synovial chondroma, first, that a misdiagnosis but also in the other direction is possible. After the diagnosis of chondrosarcoma requires drastic therapeutic measures include a generous en- bloc resection or amputation of the affected limb. A conscientious cooperation of clinicians, radiologists and pathologists is therefore particularly important.

The treatment of synovial chondromatosis itself consists of the removal of loose bodies and an arthroscopic, as complete as possible synovectomy.

Giant solitary synovial chondromatosis

As a special subtype describes the so-called Giant solitary synovial chondromatosis. At this occurs by fusion of many individual chondroma, resulting in a solid tumor may form up to a diameter of 20 cm. Also, a corresponding enlargement of a single chondroma is considered possible. Histologically and radiologically the definition of a malignant tumor ( a chondrosarcoma or periosteal osteosarcoma ) is difficult.