Synovial chondromatosis

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Synovial chondromatosis
Other namesSynovial osteochondromatosis, synovial chondrosis, Reichel syndrome, synovial chondrometaplasia (not recommended) [1] primary synovial osteochondromatosis, Reichel-Jones-Henderson syndrome
ChondromatoseCoude.jpg
X-ray of an elbow affected by synovial chondromatosis
Specialty Orthopedics

Synovial chondromatosis is a locally aggressive bone tumor of the cartilaginous type. [1] It consists of several hyaline cartilaginous nodules and has the potential of becoming cancerous. [1]

Contents

Signs and symptoms

People usually complain of pain in one joint, which persists for months, or even years, does not ease with exercise, steroid 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 experienced hands, ultrasound is also useful for the diagnosis. [2]

Rare and little known, with currently no known cure, the disease gradually forms blisters in the thin flexible membrane of the synovium, 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 affected tissue will show up as a semi-solid mass in an MRI scan, final diagnosis is usually confirmed by taking a biopsy. The disease generally affects only one of the larger weight bearing joints (hip, ankle, knee) – although the elbow, and wrist can also be affected. It rarely involves the temporomandibular joint (TMJ) and most publications are case reports. [3]

Synovial chondromatosis occurs twice as commonly in males as females 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. Familial synovial chondromatosis with dwarfism introduces characteristics of dwarfism.

Cause

The exact underlying cause of synovial chondromatosis is unknown. Some evidence suggests trauma may play a role in its development as it mainly presents in weight-bearing joints. Infection has also been considered as a contributing factor. The condition is not inherited. [4]

Synovial chondromatosis can reportedly occur as either a primary or secondary form. Primary synovial chondromatosis, which is more rare, occurs spontaneously and does not appear to relate to any pre-existing conditions. Secondary synovial chondromatosis is the more common form and often occurs when there is pre-existent osteoarthritis, rheumatoid arthritis, osteonecrosis, osteochondritis dissecans, neuropathic osteoarthropathy (which often occurs in diabetic individuals), tuberculosis, or osteochondral fractures (torn cartilage covering the end of a bone in a joint) in the affected individual. [4]

Diagnosis

Proton density weighted MRI of a knee with synovial chondromatosis. There are multiple intra-articular loose bodies located in the suprapatellar recess. PD MRI of synovial chondromatosis of the knee.jpg
Proton density weighted MRI of a knee with synovial chondromatosis. There are multiple intra-articular loose bodies located in the suprapatellar recess.

Diagnosis is by medical imaging; X-ray, CT scan and MRI. [1] For diagnosis of the TMJ, Cone beam computed tomography (CBCT) can also be useful. [6]

Treatment

Treatment is frequently by means of removal of the loose bodies and of a partial or full synovectomy (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.

Names

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. [7]

Related Research Articles

Temporomandibular joint dysfunction Medical condition

Temporomandibular joint dysfunction is an umbrella term covering pain and dysfunction of the muscles of mastication and the temporomandibular joints. The most important feature is pain, followed by restricted mandibular movement, and noises from the temporomandibular joints (TMJ) during jaw movement. Although TMD is not life-threatening, it can be detrimental to quality of life; this is because the symptoms can become chronic and difficult to manage.

Knee Region around the kneecap

In humans and other primates, the knee joins the thigh with the leg and consists of two joints: one between the femur and tibia, and one between the femur and patella. It is the largest joint in the human body. The knee is a modified hinge joint, which permits flexion and extension as well as slight internal and external rotation. The knee is vulnerable to injury and to the development of osteoarthritis.

Temporomandibular joint Joints connecting the jawbone to the skull

In anatomy, the temporomandibular joints (TMJ) are the two joints connecting the jawbone to the skull. It is a bilateral synovial articulation between the temporal bone of the skull above and the mandible below; it is from these bones that its name is derived. This joint is unique in that it is a bilateral joint that functions as one unit. Since the TMJ is connected to the mandible, the right and left joints must function together and therefore are not independent of each other.

Joint Location at which two or more bones make contact

A joint or articulation is the connection made between bones, ossicles, or other hard structures in the body which link the skeletal system into a functional whole. They are constructed to allow for different degrees and types of movement. Some joints, such as the knee, elbow, and shoulder, are self-lubricating, almost frictionless, and are able to withstand compression and maintain heavy loads while still executing smooth and precise movements. Other joints such as sutures between the bones of the skull permit very little movement in order to protect the brain and the sense organs. The connection between a tooth and the jawbone is also called a joint, and is described as a fibrous joint known as a gomphosis. Joints are classified both structurally and functionally.

Septic arthritis Medical condition

Acute septic arthritis, infectious arthritis, suppurative arthritis, osteomyelitis, or joint infection is the invasion of a joint by an infectious agent resulting in joint inflammation. Generally speaking, symptoms typically include redness, heat and pain in a single joint associated with a decreased ability to move the joint. Onset is usually rapid. Other symptoms may include fever, weakness and headache. Occasionally, more than one joint may be involved, especially in neonates and younger children. In neonates, infants during the first year of life, and toddlers, the signs and symptoms of septic arthritis can be deceptive and mimic other infectious and non-infectious disorders.

Bakers cyst Medical condition

A Baker's cyst, also known as a popliteal cyst, is a type of fluid collection behind the knee. Often there are no symptoms. If symptoms do occur these may include swelling and pain behind the knee, or knee stiffness. If the cyst breaks open, pain may significantly increase with swelling of the calf. Rarely complications such as deep vein thrombosis, peripheral neuropathy, ischemia, or compartment syndrome may occur.

Avascular necrosis Death of bone tissue due to interruption of the blood supply

Avascular necrosis (AVN), also called osteonecrosis or bone infarction, is death of bone tissue due to interruption of the blood supply. Early on, there may be no symptoms. Gradually joint pain may develop which may limit the ability to move. Complications may include collapse of the bone or nearby joint surface.

Synovial osteochondromatosis Medical condition

Synovial osteochondromatosis (SOC) is a rare disease that creates a benign change or proliferation in the synovium or joint-lining tissue, which changes to form bone-forming cartilage. In most occurrences, there is only one joint affected, either the knee, the hip, or the elbow. Rarely involves the TMJ.

Ligamentous laxity Medical condition

Ligamentous laxity, or ligament laxity, means loose ligaments. Ligamentous laxity is a cause of chronic body pain characterized by loose ligaments. When this condition affects joints in the entire body, it is called generalized joint hypermobility, which occurs in about ten percent of the population, and may be genetic. Loose ligaments can appear in a variety of ways and levels of severity. It also does not always affect the entire body. One could have loose ligaments of the feet, but not of the arms.

Calcium pyrophosphate dihydrate crystal deposition disease Medical condition

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, also known as pseudogout and pyrophosphate arthropathy, is a rheumatologic disease which is thought to be secondary to abnormal accumulation of calcium pyrophosphate dihydrate crystals within joint soft tissues. The knee joint is most commonly affected.

Synovitis Medical condition

Synovitis is the medical term for inflammation of the synovial membrane. This membrane lines joints that possess cavities, known as synovial joints. The condition is usually painful, particularly when the joint is moved. The joint usually swells due to synovial fluid collection.

Osteochondritis dissecans Ischemic bone disease

Osteochondritis dissecans is a joint disorder primarily of the subchondral bone in which cracks form in the articular cartilage and the underlying subchondral bone. OCD usually causes pain during and after sports. In later stages of the disorder there will be swelling of the affected joint which catches and locks during movement. Physical examination in the early stages does only show pain as symptom, in later stages there could be an effusion, tenderness, and a crackling sound with joint movement.

Felty's syndrome (FS), also called Felty syndrome, is rare autoimmune disease characterized by the triad of rheumatoid arthritis, enlargement of the spleen and low neutrophil count. The condition is more common in those aged 50–70 years, specifically more prevalent in females than males, and more so in Caucasians than those of African descent. It is a deforming disease that causes many complications for the individual.

Plica syndrome is a condition that occurs when a plica becomes irritated, enlarged, or inflamed.

Synovectomy is a procedure where the synovial tissue surrounding a joint is removed. This procedure is typically recommended to provide relief from a condition in which the synovial membrane or the joint lining becomes inflamed and irritated and is not controlled by medication alone. If arthritis is not controlled, it can lead to irreversible joint damage. The synovial membrane or "synovium" encloses each joint and also secretes a lubricating fluid that allows different joint motions such as rolling, folding and stretching. When the synovium becomes inflamed or irritated, it increases fluid production, resulting in warmth, tenderness, and swelling in and around the joint.

Diastematomyelia is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra in the longitudinal (sagittal) direction. Females are affected much more commonly than males. This condition occurs in the presence of an osseous, cartilaginous or fibrous septum in the central portion of the spinal canal which then produces a complete or incomplete sagittal division of the spinal cord into two hemicords. When the split does not reunite distally to the spur, the condition is referred to as diplomyelia, which is true duplication of the spinal cord.

Rheumatoid nodule Medical condition

A rheumatoid nodule is a lump of tissue, or an area of swelling, that appear on the exterior of the skin usually around the olecranon or the interphalangeal joints, but can appear in other areas. There are four different types of rheumatoid nodules: subcutaneous rheumatoid nodules, cardiac nodules, pulmonary nodules, and central nervous systems nodules. These nodules occur almost exclusively in association with rheumatoid arthritis. Very rarely do rheumatoid nodules occur as rheumatoid nodulosis in the absence of rheumatoid arthritis. Rheumatoid nodules can also appear in other areas of the body other than the skin. Less commonly they occur in the lining of the lung or other internal organs. The occurrence of nodules in the lung of miners exposed to silica dust was known as Caplan’s syndrome. Rarely, the nodules occur at diverse sites on body.

Oral and maxillofacial pathology refers to the diseases of the mouth, jaws and related structures such as salivary glands, temporomandibular joints, facial muscles and perioral skin. The mouth is an important organ with many different functions. It is also prone to a variety of medical and dental disorders.

Attempts in the last decade to develop surgical treatments based on MRI and CAT scans now receive less attention. These techniques are reserved for the most difficult cases where other therapeutic modalities have failed. The American Society of Maxillofacial Surgeons recommends a conservative/non-surgical approach first. Only 20% of patients need to proceed to surgery.

Hip pain

Pain in the hip is the experience of pain in the muscles or joints in the hip/ pelvic region, a condition commonly arising from any of a number of factors. Sometimes it is closely associated with lower back pain.

References

  1. 1 2 3 4 "Synovial chondromatosis". Soft Tissue and Bone Tumours: WHO Classification of Tumours. International Agency for Research on Cancer. 2020. p. 368-369. ISBN   978-92-832-4502-5.
  2. Arend CF. Ultrasound of the Shoulder. Master Medical Books, 2013. Free chapter on ultrasound findings of synovial chondromatosis affecting the subacromial-subdeltoid bursa available at ShoulderUS.com
  3. Fuller E, Bharatha A, Yeung R, Kassel EE, Aviv RI, Howard P, Symons SP. Case of the month #166: synovial chondromatosis of the temporal mandibular joint. Canadian Association of Radiologists Journal. 2011 May; 62(2):151-153.
  4. 1 2 "Synovial Chondromatosis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. US National Institutes of Health. Retrieved 4 May 2020.PD-icon.svgThis article incorporates text from this source, which is in the public domain .
  5. Lima, Luana T. Barros de; Albuquerque Filho, Eolo Santana de; Batista, Laecio Leitão; Moraes, Talita Peixoto de; Pereira, Bruno Perez Guedes (2016). "Unusual lesions that distend the knee joint: pictorial essay". Radiologia Brasileira. 49 (5): 322–328. doi:10.1590/0100-3984.2015.0154. ISSN   0100-3984. PMC   5094822 . PMID   27818547. (CC BY 4.0)
  6. Florent Destruhaut, Antoine Dubuc, Aurélien Bos, Laurent Fabié, Philippe Pomar, Jean-Claude Combadazou, Antonin Hennequin, Sara Laurencin: Diagnosis of Synovial Chondromatosis of Temporomandibular Joint: Case Report and Literature Review: Healthcare (Basel).2021 May 18;9(5):601. doi: 10.3390/healthcare9050601.
  7. "Reichel's syndrome". www.whonamedit.com. Retrieved 2020-02-25.