Synovial chondromatosis
Encyclopedia
Synovial chondromatosis is a disease
affecting the synovium
, a thin flexible membrane around a joint. It is also known as Reichel's syndrome or Reichel-Jones-Henderson syndrome, named after Friedrich Paul Reichel
, Hugh Toland Jones and Melvin Starkey Henderson
.
injection or heat treatment, shows nothing on X-ray
, but shows a definite restriction of movement.
There are 3 defined stages to this disease:
In the early stages of the disease it is often confused with tendinitis and/or arthritis
. Once it reaches transitional the loose bodies become apparent with X-ray
in greater than 70% of cases, with MRI often showing where xray fails.
In the disease, the thin flexible membrane of the synovium gradually forms blisters which calcify and enlarge. These nodules eventually break free and float around the joint space becoming larger – these add to the discomfort and stiffness of the joint.
The disease is rare and little known and currently no known cure. The affected tissue will show up as a semi-solid mass in a MRI scan, final diagnosis is usually confirmed by taking a biopsy
.
Traditional medical journals conclude that individuals suffering from this disease are nearly always male, and usually in their forties. However, online communities for synovial chondromatosis patients have yielded a stark contrast, with equal representation from both genders and members diagnosed as young as late teenage/early 20s.
The disease generally affects only one of the larger weight bearing
joints (hip, ankle
, knee
) – although the elbow
, and wrist
can also be affected.
(removal of the synovium)
Full synovectomy is a moderately major operation and involves completely exposing the joint and removing the affected tissue. Partial synovectomy is normally done arthroscopically. Synovectomies are normally carried out by shaving the lining of the knee but there are other ways of achieving this by either freezing the synovium or by the use of radiation treatment.
The need for further procedures is greater than 25% although normally the frequency of the required removal of loose bodies is reduced by the previous synovectomy. There have been documented cases of malignant transformation however this is rare.
Whilst the condition can be described as a ‘benign growth’ it seldom affects more than one joint, and does not usually affect surrounding tissue.
Disease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...
affecting the synovium
Synovium
Synovial membrane is the soft tissue found between the articular capsule and the joint cavity of synovial joints....
, a thin flexible membrane around a joint. It is also known as Reichel's syndrome or Reichel-Jones-Henderson syndrome, named after Friedrich Paul Reichel
Friedrich Paul Reichel
Friedrich Paul Reichel was a German surgeon.1881 - 1885: Assistant 1882: Doctor of medicine...
, Hugh Toland Jones and Melvin Starkey Henderson
Melvin Starkey Henderson
Melvin Starkey Henderson was an American orthopedic surgeon, who was born in St. Paul, Minnesota .-Biography:Upon his father's death, he went to Winnipeg, Manitoba to live with relatives, the family of Edward Drewry and owner of the Drewry Brewery...
.
Symptoms
Patients usually complain of pain in one joint, which persists or months, or even years, does not ease with exercise, steroidSteroid
A steroid is a type of organic compound that contains a characteristic arrangement of four cycloalkane rings that are joined to each other. Examples of steroids include the dietary fat cholesterol, the sex hormones estradiol and testosterone, and the anti-inflammatory drug dexamethasone.The core...
injection or heat treatment, shows nothing on X-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
, but shows a definite restriction of movement.
There are 3 defined stages to this disease:
- early: no loose bodies but active synovial disease;
- transitional: active synovial disease, and loose bodies;
- late: loose bodies but no synovial disease;
In the early stages of the disease it is often confused with tendinitis and/or arthritis
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
. Once it reaches transitional the loose bodies become apparent with X-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
in greater than 70% of cases, with MRI often showing where xray fails.
In the disease, the thin flexible membrane of the synovium gradually forms blisters which calcify and enlarge. These nodules eventually break free and float around the joint space becoming larger – these add to the discomfort and stiffness of the joint.
The disease is rare and little known and currently no known cure. The affected tissue will show up as a semi-solid mass in a MRI scan, final diagnosis is usually confirmed by taking a biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
.
Traditional medical journals conclude that individuals suffering from this disease are nearly always male, and usually in their forties. However, online communities for synovial chondromatosis patients have yielded a stark contrast, with equal representation from both genders and members diagnosed as young as late teenage/early 20s.
The disease generally affects only one of the larger weight bearing
Weight bearing
In orthopedics, weight-bearing is the amount of weight a patient puts on the leg on which surgery has been performed. In general, it is described as a percentage of the body weight, because each leg of a healthy person carries the full body weight when walking, in an alternating fashion.After...
joints (hip, ankle
Ankle
The ankle joint is formed where the foot and the leg meet. The ankle, or talocrural joint, is a synovial hinge joint that connects the distal ends of the tibia and fibula in the lower limb with the proximal end of the talus bone in the foot...
, knee
Knee
The knee joint joins the thigh with the leg and consists of two articulations: one between the fibula and tibia, and one between the femur and patella. It is the largest joint in the human body and is very complicated. The knee is a mobile trocho-ginglymus , which permits flexion and extension as...
) – although the elbow
Elbow
The human elbow is the region surrounding the elbow-joint—the ginglymus or hinge joint in the middle of the arm. Three bones form the elbow joint: the humerus of the upper arm, and the paired radius and ulna of the forearm....
, and wrist
Wrist
In human anatomy, the wrist is variously defined as 1) the carpus or carpal bones, the complex of eight bones forming the proximal skeletal segment of the hand;...
can also be affected.
Treatment
Treatment is frequently by means of removal of the loose bodies and of a partial or full SynovectomySynovectomy
Synovectomy is the surgical removal of a part of the synovial membrane of a synovial joint.-Indications:It is one of the treatment options for certain diseases involving the synovium like:* Severe rheumatoid arthritis or juvenile idiopathic arthritis...
(removal of the synovium)
Full synovectomy is a moderately major operation and involves completely exposing the joint and removing the affected tissue. Partial synovectomy is normally done arthroscopically. Synovectomies are normally carried out by shaving the lining of the knee but there are other ways of achieving this by either freezing the synovium or by the use of radiation treatment.
The need for further procedures is greater than 25% although normally the frequency of the required removal of loose bodies is reduced by the previous synovectomy. There have been documented cases of malignant transformation however this is rare.
Whilst the condition can be described as a ‘benign growth’ it seldom affects more than one joint, and does not usually affect surrounding tissue.