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Haemophilic Arthropathy-Clinical and Radiological Findings

July 31, 2008 By Leave a Comment

The weight-bearing joints are most commonly affected, with frequency of involvement being, in descending order: knee, elbow, shoulder, ankle, wrist, and hip. The vertebral column is rarely involved.

Following injury, the synovial vessels rupture, and blood accumulates in the joint. Bleeding continues until the intra-articular hydrostatic pressure exceeds that of the arterial and capillary pressure in the synovium. The resultant tamponade of the synovial vessels causes ischemia of the synovium and subchondral bone.

With repeated hemorrhage, hyperplasia and fibrosis of the synovium will occur.

Pannus formation by the proliferating synovial tissue will erode the hyaline cartilage peripherally, and compression of its opposing cartilaginous surfaces will result in degeneration of articular cartilage centrally.

Inflammatory process invades and destroys cartilage. Loss of joint motion and contractural deformity occurs. Subchondral bone cysts are formed due to local ischaemia.

An attempt Stimulation of growth may be asymmetrical, resulting in valgus or varus deformity. Shortening of a limb may be produced by early closure of the physis.

Osteoporosis and muscle atrophy are common.

Clinical Findings

These depend on the severity of hemorrhage and whether the hemarthrosis is acute, subacute, or chronic. In acute hemarthrosis pain and swelling with distention of the joint capsule are the principle findings. A history of injury may not be elicited. With cessation of bleeding the intensity of pain decreases.

The joint will assume the position of minimal discomfort, which will also be the position of minimal intra-articular pressure.

For example the hip joint is held in 30 to 65 degrees of flexion, 15 degrees of abduction, and 15 degree of lateral rotation.Extension, wide abduction, and medial rotation of the hip are limited and painful, as they increase intra-articular hydrostatic pressure.

The knee joint is held in flexion with marked restriction of range of motion, caused by protective spasm, pain, and the hemarthrosis. Local tenderness and increased heat are present. Te overlying skin will be tense and shiny.

The intense pain of acute hemarthrosis subsides rapidly after the administration of factor VIII or IX.

Subacute hemarthrosis develops after several episodes of bleeding into the joint. Pain is minimal. The synovium is thickened and boggy. Restriction of joint motion is moderate. Sub-acute hemarthrosis does not respond rapidly to administration of clotting factor. Chronic hemarthrosis develops after six months of involvement. Progressive destruction of the joint leads to a fibrotic, stiff, totally destroyed joint.

Radiographic Finding

Radiograms will disclose soft-tissue swelling due to distention of the joint capsule. With repeated hemorrhage and resultant chronic synovitis there may be

  • Osteoporosis
  • Enlargement of the epiphysis
  • Subchondral cysts,
  • Narrowing of the articular cartilage space
  • Formation of peripheral osteophytes.

The final phase of hemophilic arthropathy is fibrous ankylosis.

Hemophilic arthropathy is classified into five stages.

In Stage I there is only soft-tissue swelling, but no skeletal abnormalities.

Stage II is characterized by overgrowth and osteoporosis of the epiphysis, but joint integrity is maintained. There are no bone cysts and no narrowing of the articular cartilage space.

The radiologic Stage II parallels the clinical stage of subacute hemophilic arthropathy.

In Stage III there is minimal to moderate joint space narrowing with subchondral cysts, which occasionally communicate with the joint space. There is widening of the intercondylar notch of the knee and the trochlear notch of the ulna. In the knee there may be squaring of the patella.

In Stage III the articular cartilage is still preserved, indicating that with treatment hemophilic arthropathy is still reversible.

In Stage IV there is destruction of articular cartilage with severe narrowing of the joint space. The other osseous changes found in Stage III-i.e., subchondral cysts, patellar squaring, and widening of intercondylar or trochlear notch are more pronounced.

Stage V is characterized by total loss of joint space with fibrous ankylosis of the joint. There is marked incongruity of the articular structures with severe irregular hypertrophy of the epiphysis.

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Hemophilia and Haemophilic Arthropathy

July 25, 2008 By Leave a Comment

Hemophilia is a  a genetically determined disorder and is characterized by abnormality of the coagulation mechanism due to functional deficiency of a specific factor, namely VIII or IX.

The name hemophilia was coined by Hopff in 1828. Wright demonstrated that disorder involves prolonged clotting time.

The estimated incidence is between 0.8-1 per 10,000 male births. The hemophiliac may be classified as follows:

Hemophilia A

This is classic hemophilia that results from a congenital deficiency of factor VIII antihemophilic factor (AHF) or antihemophilic globulin (AHG). This constitutes 80 percent of cases and is  X linked in inhertance. It occurs in the male and is transmitted by asymptomatic female carriers.

Hemophilia B

It is also known as Christmas disease and is due to a deficiency of factor IX (plasma thromboplastin component (PTC) or Christmas factor. Its clinical manifestations are quite similar to those of classic hemophilia. The hereditary transmission is also by an X-linked recessive gene. This type is the cause of about 15 percent of cases.

Van Willebrand’s Disease

In this bleeding disorder both factor VIII deficiency and platelet functional abnormality are present. It is inherited as an autosomal dominant trait, occurring in both males and females. The bleeding disorder is relatively mild.

Clinical Picture

Uncontrolled hemorrhage and repeated episodes of bleeding are the hallmarks of hemophilia. The severity of the disease varies from patient to patient, but it is constant in any one patient.

  • When the functional plasma level of the factor is 25 to 50 percent of normal the hemophilia is mild. Excessive bleeding occurs only after major trauma or during surgery
  • When the plasma level of the factor is 5 to 25 percent of normal, the hemophilia is moderate. Severe uncontrolled bleeding occurs after minor injury or during an operative procedure;
  • When plasma level of the factor it is 1 to 5 percent of normal the hemophilia is moderately severe with major hemorrhage taking place after minor injury or unrecognized mild trauma.
  • When the plasma levels of factor VIII or IX are below 1 percent, the hemophilia is considered very severe. There are repeated spontaneous hemorrhages into joints and bleeding into deep soft tissues.

Abnormal bleeding in hemophilia may occur in any area of the body. Joints are the most frequent sites of repeated hemorrhage followed by muscles and soft tissues.

When haemophila is severe,  the abnormal bleeding may manifest itself in the neonatal period or early infancy. Abnormal bleeding into joints and soft tissues is noted by the parents when child begins to crawl or walks.

About three fourths of bleeding sustained by hemophiliac is into either the joints, the deep soft tissues, or both.

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Types of Arthritis

July 24, 2008 By 3 Comments

Arthritic conditions can be divided into low-inflammatory or high-inflammatory types.

The classic types of low-inflammatory disease are primary osteoarthritis, produced by intrinsic degeneration of articular cartilage, and posttraumatic arthritis.

Rheumatoid arthritis is the classic type of high-inflammatory arthritis.

Other types include gout, psoriatic arthritis, Reiter’s syndrome, lupus erythematosus, ankylosing spondylitis, arthritis associated with bacterial infection, , and cccarthritis of ulcerative colitis.
Some articular diseases fit into neither category and that have unique characteristics, such as aseptic necrosis,“frozen” shoulder and other joint-stiffening conditions, as well as neuropathic joints.
Patients with the low-inflammatory type have low leukocyte counts in the synovial fluid and laboratory findings consistent with low-level inflammatory activity; the affected joint often shows focal degeneration.

Those with the high-inflammatory type have high leukocyte counts in the synovial fluid, laboratory findings consistent with high-level inflammatory activity, and usually show a more diffuse degeneration of the involved joints .

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Tuberculous Arthritis-Radiography, Laboratory Findings and Treatment

July 23, 2008 By 1 Comment

The earliest findings in the radiograms are regional bone atrophy, soft-tissue swelling, and capsular distention. These changes are due to synovitis and are nonspecific. As a rule, the bone decalcification in tuberculous arthritis is widespread, extending 3 to 5 cm. from the joint.

The joint space is widened and is preserved until late in the course of the disease.

Destruction of the hyaline cartilage by the tuberculous granulation tissue is a slow process. Eventually, with progression of the disease, the articular cartilage space will gradually narrow.

This is in contrast to pyogenic arthritis, in which the destruction of articular cartilage and narrowing of the joint space take place early in the course of the disease.

Bone destruction can be seen in the epiphysis or metaphysis as areas of radiolucency in which the normal trabecular structure of bone has disappeared

When affection of the joint is secondary to a tuberculous focus in the epiphysis or metaphysis the areas of bone destruction may be anywhere and are not distributed peripherally in the noncontact portions.

Reactive new bone formation is characteristically absent in the early stages of tuberculous arthritis; it is only in the late healing stages that it develops.

Sequestra may occasionally be present.

Eventually, in an untreated case, the entire articular cartilage will eventually be eroded and extensive destruction of subjacent bone will take place, resulting in gross deformity of the joint.

Laboratory Findings

The general findings are those of a chronic illness. Hypochronic anemia is common. The leukocyte count may be normal, or there may be slight leukocytosis. An elevated erythrocyte sedimentation rate and positive tubercular skin test are almost always present.

The synovial fluid shows an elevated leukocyte count, a lowered sugar level, and poor mucin.

Tubercle bacilli may be seen on microscopic examination of sediment of the joint fluid. A finding of great help in differential diagnosis is the marked reduction or absence of glucose in the synovial fluid. Cultural studies and guinea pig inoculations will be positive for tuberculosis.

The diagnosis is also confirmed by histologic examination of synovial tissue. Most of the diagnosis in India is made on clinicoradiological changes

Treatment

Early diagnosis and the use of antituberculous drugs have radically improved the prognosis of tuberculous arthritis. Other measures adopted are rest to the affected joint in functional position, traction when needed and dietary improvement.

Treatment consists of general medical measures, chemotherapy, local conservative orthopedic care, and surgery.

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Tuberculous Arthritis-Pathology and Clinical Features

July 22, 2008 By 5 Comments

Tuberculous ArthritisTuberculosis of bones and joints is a granulomatous inflammation caused by Myobacterium tuberculosis. It is a localized and destructive disease that is usually blood-borne from a primary focus such as infected peribronchial or mesenteric lymph nodes.

The infection may be either of the human or the bovine type. In countries where raw milk is used extensively, bovine transmission is common, whereas in areas where milk is pasterurized and there is rigid control of dairy herds, the bovine type is extremely rare and the human type is more common.

The incidence of tuberculosis has greatly declined in the past three decades owing to the discovery of antituberculous drugs and the enforcement of strict public health measures such as pasteurization and the reporting and isolation of patients with active tuberculosis.

But in economically well-developed countries it is still prevalent. [Read more...]

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Tuberculosis of Spine-An Overview

July 21, 2008 By 259 Comments

A scoliotic Spine With TuberculosisThis entity was first described by Percivall Pott. He noted this as a painful kyphotic deformity of the spine associated with paraplegia. Since then condition is often referred to as Pott’s disease.

The spine is the most common site of skeletal tuberculosis accounts for 50 percent of the cases. Lower thoracic region is the most common segment involved followed by lumbar, upper dorsal, cervical and sacral regionsin decreasing order of frequency. [Read more...]

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Lyme Arthritis

July 14, 2008 By Leave a Comment

Lyme disease is caused by the spirochete Borrelia burgdorferi and is transmitted by the tick Ixodes dammini (deer tick) or a related ixodid tick.

Arthritis is the presenting manifestation in the majority of cases.

The infection is endemic in certain areas of North America and has been described in 19 countries. The disease characteristically develops in the summer and autumn-periods when the ticks are very active.

Clinical Features

The arthritis is preceded in about half the cases by a characteristic rash-erythema chronicum migrans. The appearance of the rash in striking. Half of the affected children will definitely recollect being bitten by a tick.

Prodromal systemic illness in the form of low-grade fever, stiff neck, or headache is present in about 40 percent of cases.

The arthritis is pauciarticular, usually affecting one or a few large joints.

The knee is the most common site and is involved in over 95 percent of cases. Other joints that can be affected are the elbow, hip, ankle, shoulder, sternoclavicular, and interphalangeal.

The arthritis follows the skin rash or prodromal systemic symptoms usually within a few months (Range- One week to 12 months).

The synovitis manifests itself as joint swelling, increased local heat, joint tenderness, and pain on extremes of motion. When the knee, hip, or ankle is involved, the patient is able to bear weight and walk on the affected lower limb with an antalgic limp.

The typical pattern of synovitis is brief and intermittent. If untreated, however, it becomes chronic.

Other clinical features of Lyme disease are meningitis or neurologic disease in the form of nerve palsy such as Bell’s, and cardiac involvement, particularly conduction defect.

Diagnosis

Elevated titers of I&M and I&G antibodies against Ixodes dammini will establish the diagnosis of Lyme disease.

A nonspecific finding is elevation of the erythrocyte sedimentation rate.

In its initial stages Lyme disease should be differentiated from the monarticular or pauciarticular form of juvenile rheumatoid arthritis.

This may be difficult but following differences cn help to delineate.

  • The attacks of Lyme arthritis are usually brief and self-limited, but that of juvenile rheumatoid arthritis is unremitting for at least six weeks.
  • Chronic iridocyclitis does not occur in Lyme arthritis.

It is advisable to carry out serologic tests to rule out Lyme disease when one is working on JRA.

Pyogenic septic arthritis is another entity to be differentiated from Lyme disease.

In bacterial arthritis, the affected joint is acutely painful, red, and hot, and the patient is unable to bear weight on the lower limbs if the knee or hip is involved. In septic arthritis, joint fluid cultures are positive in 70 percent of cases.

Synovial fluid analysis is ordinarily not of great assistance in differentiating the two because in both conditions the leukocyte count is elevated with neutrophilia. The erythrocyte sedimentation rate is elevated in both.

In case of doubt the arthritis should be treated as if septic while serologic tests for Lyme disease are sent which should be available within one to two weeks.

Treatment

Therapy consists of administration of antibiotics-penicillin or tetracycline alone.

Initially, when the arthritic symptoms are mild, penicillin may be given orally (phenoxymethyl penicillin) 50 mg/kg/day for four weeks.

If tetracycline is administered, the dosage is 30 mg/kg/day.

If the arthritis fails to respond to oral antibiotics, or if it is acute, the parenteral route is employed. Intravenous penicillin G or benzathine penicillin is given for ten days.

Antibiotics do prevent or attenuate subsequent attacks.

They should be administered for at least one month in order to minimize the risk of recurrence with exacerbation of the arthritis.

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Ewing Sarcoma-Clinical Presentation, Pathology, Radiographic Findings and Treatment

July 7, 2008 By Leave a Comment

Ewing’s sarcoma is he second most common primary malignant tumor of bone found in children.Ewing Sarcoma is an uncommon primary malignant bone tumor.It has been named after Ewing, who first described it in 1921.

Ewing sarcoma has a characteristic predilection for an age group between 10 and 15 years. The tumor is more common in boys than in girls. [Read more...]

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