Rheumatoid Arthritis Rheumatoid arthritis is a well known clinical condition that results in apainful inflammatory synovitis most commonly in young adult femaleswith fair skin with a symmetric involvement of the hands, feet, wrists,ankles, knees, elbows and cervical spine. It is an autoimmune processthat has no clear cut etiology. However, it is suggested that the infamma-tory process is initiated by an external bacterial or viral source which thenresults in an antigen-antibody response in synovial, tenosynovial andbursal linings with the release of lysozymes that destroy joint cartilage,subchondral bone and juxta articular ligamentous structures. Rheumatoidfactor, an IgM immunoglobulin produced by plasma cells in thesynovial lining, is found in 70-80% of rheumatoid arthritic patients.These patients may have a genetic predisposition to their disease with anincreased frequency of possessing the histocompatibility antigens HLA-Cw3 and HLA-DRw4. The condition may start in a single joint such asone of the PIP or MP joints of the hand or foot and later spread to otherjoints in a symmetric fashion. The early radiographic findings include diffuse juxta articular osteo-
penia along with a universal loss of articular cartilagenous joint space.Later on with enzymatic softening of joint ligaments one will see jointdeformities such as ulnar deviation of fingers at the MP joints andswan neck deformities of fingers from flexion contractures of the MPjoints and hyperextension defects of the PIP joints. Another late changeis the formation of inflammatory subchondral granulomas and cystsseen also in infectious and gouty arthropathies. Chronic joint effusionscan lead to large juxta articular rheumatoid cysts about the knees, hipsand shoulder joints. Rheumatoid tenosynovitis is commonly seen aboutthe hands, wrists, feet and ankles. Large rheumatoid bursal sacs can beseen about the hips and shoulders. Rheumatoid nodules are seen sub-cutaneously about the extensor surfaces of joints such as the elbow andWrist. Rheumatoid arthritis can hit the upper cervical spine with dangerouflexion deformity at C1 on 2 with resultant spinal cord compression.Medical treatment consists of anti inflammatory drugs includingcortisone. Surgical treatment consists of early synovectomies, teno-synovectomies and bursectomies. Late treatment for chronic pain anddeformity consists of joint replacement arthroplasties.
Mechanism for rheumatoid synovitis and chondrolysis
Pathogenesis of rheumatoid knee joint destruction
Rheumatoid bursitis of shoulder in a 35 yr old female
Resected sub deltoid bursashowing hypertrophic andhyperemic changes seenin any rheumatoid synovitisPhotomicrograph of bursallining with prominentlymphoid nodule layingbeneath a hyperemic andhypertrophic synovial lining
Rheumatoid tenosynovitis and arthritis of wrist 40 year old female with 1 yr history of swelling about the dorsal wrist and x-ray evidence of severe carpitis
Surgical photos at time oftenosynovectomy of inflamed tendon sheaths of dorsal wristPhotomicrograph of resected tenosynovium
Several years later with volarwrist dislocation and surgical correction with a Voltz arthroplasty
Early swan neck deformity of rheumatoid fingers Littler release of oblique fibers of extensor hood
Early ulnar drift deformity in rheumatoid fingers Surgical correction by repositioning of the ulnardisplaced extensor tendons
Late MP joint dislocations in rheumatoid fingersSylastic MP rubber joint arthroplasties with PO x-ray
Late MP and PIP deformities of rheumatoid fingersFowler MP joint arthroplasty Metallic PIP joint arthroplasties
Rheumatoid extensor tendon ruptures at the wrist level Thumb MP joint fusion Surgical correction with for rheumatoid instability tendon grafts
Carpal tunnel syndrome 2nd to rheumatoid tenosynovitis Trigger finger deformity 2nd to rheumatoid tenosynovitis
Rheumatoid deformity of the footDorsal subluxation of MP joints & chondrolysis of forefoot joints
Rheumatoid instability at C1 on C2 Lateral x-ray Flexion view 2 yrs post fusion38 year female with rheumatoid arthritis and flexion instability at C1on C2 with mild paraparesis treated with a posterior spinal fusion
Pathogenesis of cord compression in C1-2 rh arthritisAutopsy specimen of 41 yr female with fatal quadriplegia 2nd to rheumatoid arthritis of C1 on C2
Rheumatoid synovial cysts arising from the knee3 cases of synovial cysts arising from rheumatoid arthritis of knees injected with contrast material
Rheumatoid synovial cysts arising from the knee Cor T-2 T-2 Gad Gad68 yr femalewith tender lumpin leg for 6 mos
Pathogenesis of rheumatoid nodules 40 yr female with severe form of rheumatoid arthritis withdiagnostic rheumatoid nodules overextensor surfaces of elbow & handMicroscopic evidence of fibrinoidnecrosis and pallisading round cells
Ankylosing Spondylitis Prior to 1963 this condition was referred to as rheumatoid spondylitissuggesting a common etiology with rheumatoid arthritis. However,ankylosing spondylitis is an autoimmune condition seen usually inyoung adult males, is usually seronegative for rh factor and most casespossess the histocompatibility antigen HLA-B27. Another eponym fromthe past is Marie-Strumpell’s disease. Early constitutional findings mayinclude anorexia, weight loss and low grade fever. Early symptomsinclude bilateral sacroiliac LBP which then gradually extends up thespine to the neck with diminished chest expansion and an exaggerateddorsal kyphosis. As many as 50% of cases will have rheumatoid likesymptoms asymmetrically in peripheral joints including the hips, knees,shoulders, ankles, wrists elbows and the small joints of the hands andfeet. Late changes that differ from rheumatoid arthritis includeossification in joint capsules that may progress to a complete bonyankylosis of the involved joint at which point the pain will disappear.Entheses is a term used to describe the ossification of ligamentous
attachments to bone seen commonly in ankylosing spondylitis and to alesser extent in other seronegative conditions such as psoriasis andReiter’s syndrome. Large entheses in the spine are referred to assyndesmophystes which create a bamboo appearance as a late change inspinal radiographs. The most common peripheral joint involvement isseen in the hip joints which may go on to a solid bony fusion. Attemptsto regain motion with a total joint replacement can lead to frustratingheterotopic bone formation which can be reduced with the use of postoperative radiation therapy. Treatment of severe dorsal kyphosis can behelped with a well planned spinal osteotomy.
Ankylosing Spondylitis (late)Classic radiographic and pathologic findings of a bamboo spine with associated bony fusion of the SI and hip joints
Differential diagnosis of syndesmophytes 1X-ray of autopsy specimen of L-spinein ankylosing spondylitis patient with classic bamboo findingsSchematic #1 shows syndesmophytesin ankylosing spondylitis compared to#2 showing those seen in psoriasis,Reiter’s syndrome and DISH syndrome
Late changes of anklyosing spondylitisSolid bony fusion of C-spine with later autopsy specimen
Early changes of ankylosingspondylitis with sclerotic changes on both sides of the SI joints in a young adult male with LBP Similar changes seen a youngpost partum female with sclerotic changes seen on just the iliac side of the SI joints with theDiagnosis of osteitis condensans ilii
Surgical correction of dorsal kyphosis in Anklyosing spondylitis Pre op Post opExtension lumbar osteotomy with Knot rod fixation posteriorly
Peripheral changes seen in ankylosing spondylitisTraction os calsis enthesmophyte Juxta articular synovial errosions about the MP joint of the hand
Psoriatic Arthritis About 5% of patients with cutaneous psoriasis will manifestsymptoms of inflammatory autoimmune arthritis of the peripheralextremities and spine. Heredity may play a role in the pathogenesisof psoriatic arthritis especially in cases of spinal and sacroiliac involve-ment where histocompatability typing can reveal a 25-60% incidenceof the HLA-B27 antigen. Rheumatoid changes are seen in the distalsmall joints of the hands and feet which are frequently asymmetric andthese patients are seronegative. The overlying skin lesions that precedethe arthritic syndromes consist of copper red plaques with silvery scalesat the periphery.The arthritic digits are frequently associated with nailchanges including pitting, discoloration, ridging and splintering.Spinal x-rays reveal asymmetric and large syndesmophytes with areasof blulky paravertebral ossification seen also in Reiter’s syndrome.
Radiographic changes of Psoriatic arthritis in hands and feet
Spinal changes seen in Psoriatic ArthritisLarge asymmetric secondary syndysmophytes with fluffy para- vertebral ossification seen also in Reiter’s syndrome
Reiter’s Syndrome Reiter’s syndrome is the most common rheumatoid variant to beinitiated by a bowel or lower genitourinary infection. As in ankylosingspondylitis there is a very high frequency of the histocompatibilityantigen HLA-B27. The disease is more common in young adult maleswho are sexually active. Urethritis is frequently the initial diseasesymptom following sexual intercourse which may then lead toasymmetric arthritis of the lower extremities. Skin lesions such askeratoderma blenorrhagicum can be seen in the hands and feet withmacular, vesicular and pustular skin formations. Keratosis of the nailssimilar to psoriasis may be seen. Early changes may include bilateralconjunctivitis. 10% of cases include ulcerations in the mouth and on thetongue. Spinal changes are similar to psoriatic arthritis with asymmetricsacroileitis and LD spinal syndesmophytes and paraspinous ossification.Bony proliferations are seen at ligamentous and tendonous attachmentsabout the pelvis, trochanters and anterior face of the os calcis.Asymmetric rheumatoid changes are seen more commonly in the MPand PIP joints of the feet and less common in the hands.
Clinical findings in Reiter’s SyndromeBilateral sacroileitis Acute conjunctivitis Vesicular and pustular lesions of hands and feet
Clinical findings of Reiter’s syndrome Bucal & tongue ulcerations Maculopapular lesion of penisVesicular and pustular lesions of feet Calcaneal heel spur
Pediatric Seronegative Arthritis (Still’s Disease) Seropositive adult rheumatoid arthritis can have its onset in olderchildren but the pediatric condition known as seronegative chronicarthritis or Still’s disease is an entirely separate disease process seen inyounger children and has been divided into three clinical groupsincluding classic systemic disease, polyarticular disease and pauci-articular disease. Classic Systemic Disease is seen in boys and girls under 5 yearswith acute febrile onset, anorexia, weight loss, skin rashes and general-ized lymphadenopathy and hepatosplenomegaly. Arthritic symptoms aremild with minimal radiologic findings. Lab studies may show anemia,leukocytosis and an elevated sed rate. Polyarticular Disease may occur at the onset of Still’s diseaseor may follow the systemic form in which case the prognosis is muchworse. The arthritis is symmetric as in adult rheumatoid arthritisinvolving the MP and PIP joints of the hands and feet, wrists,ankles,
and cervical spine. Osteoporosis and early loss of joint space leadsto early ankylosis with severe contractures. Premature closure ofgrowth plates results in a painful form of dwarfism. Pauciarticular Disease is a more common and milder form ofStill’s disease seen in younger children that may affect one thru five ofthe larger joints such as the knee, ankle, elbow and wrist. The arthritismay self arrest in several years with no residual deformity. Thesechildren are at risk of developing blindness from iridocyclitis.
Early Radiographic changes in Still’s DiseaseSevere MP and PIP joint changes Flexion contractures both knees Early osteoporotic changes Later mid tarsal osseous fusion
Late Changes of Still’s DiseaseYoung adult female with severechondrolysis of the hip and kneejoints from severe arthritis since childhood
Late changes of Still’s Disease57 year old female in bed for 5 yrs because of stiff knees
Scleroderma (Progressive Systemic Sclerosis) & Dermatomyositis Both scleroderma and dermatomyositis are autoimmune disordersseen in young adult females that show rheumatoid joint changes alongwith a unique peripheral microvascular Raynaud’s disorder that resultsin dystrophic eschemic calcifications about the hands and feet, wrists,ankles and knees.They both may have an elevated sed rate and testpositive for rheumatoid factor. Dermatomyositis is unique for thefinding of elevated muscle enzymes. They both can have radiographicchanges in the small joints of the hands and feet as seen in rheumatoidarthritis. The skin changes in scleroderma are unique with early edemafollowed later with a leathery indurated thickening and shiny appear-ance of the hands, feet and facial areas. At times the cutaneouscalcifications will ulcerate to the skin surface. Fibrosis of the esophaguscan result is dysphasia and other organs such as the heart, lung andkidneys can be affected.
Radiographic changes in SclerodermaSubQ calcifications about the knee, calf and hands
Pseudo scleroderma in lead synovitis from bullet wound Arrow defines retained lead bullet resulting in lead synovitis
Radiographic findings in dermatomyositis35 yr female with severe elbow contracture from chronicmyositis with subQ calcificationof the upper extremity, thighs and hands
Hemophiliac Arthropathy There are three type of hemophilia. The most common and classicform is an inherited x-linked recessive deficiency of antihemophiliacglobulin (AHG) or factor 8 which is almost exclusively seen in malesbut carried genetically by females. A far less common form of hemo-philia is Christmas disease which results from an x-linked recessivedeficiency of plasma thromboplastin component (PTC) or factor 9. Thethird form of hemophilia is an autosomal dominient deficiency ofplasma thromboplastin antecent or PTA which does not have an ortho-pedic problem with recurrent hemarthrosis. The factor 8 and 9 conditionsusually start their clinical problems with recurrent hemarthroses of thosejoints exposed to trauma such as the knees, ankles and elbows. Thehemorrhagic synovitis creates a similar pathologic change in the jointas one sees in other infammatory disorders such as severe forms of Still’sdisease, infectious arthropathies and PVNS. Radiologically one will seejuxta-articular osteoporosis. Progressive chondrolysis, bulbous enlarge-ment of epiphyseal bone, subchondral fibrocystic granulomas and in
very severe cases one may see an osseous anklyosis as is seen in Still’sdisease. Adjacent to the involved joint such as the knee one may seerecurrent subperiosteal hematoma formation that can produce a hemo-philliac pseudo tumor and even pathologic fracture simulating a sarcoma.Treatment programs include trauma reduction and possible prophylacticbracing. With an acute hemarthrosis the appropriate replacement factoris given followed by aspiration, icing down the swollen joint andtemporary immobilization. For chronic problems with advanced adultdisease, total joint replacement may be indicated.
Progressive hemophiliac knee arthropathy Early disease at age13 Surgical exposure for TKA age 25Micro of hemorrhagic synovitis Placement of a spherocentric knee
Hemophiliac Hip Arthropathy24 yr male with factor 9 diseasewith painful chondrolysis whichled to a THA with the resectedhead and hemorrhagic synovitisseen to your right.
Total ankle replacemnt for hemophiliac arthrosis
Early vs late changes in hemophiliac arthropathy 9 yr male with 12 yr male with late changes in the ankle withearly elbow changes complete bony ankylosis
Hemophiliac hip dislocation in a 3 yr old male
Pediatric Pseudo tumor formation in hemophilia15 yr male with a pseudo tumor of distalfemur with pathologic fracture plushemophilic arthropathy of adjacent kneealong with a pseudo tumor of the proximalphalanx of the middle finger
Degenerative Osteoarthritis & Spondylitis Degenerative osteoarthritis is the most common form of arthritis whichis really part of the normal aging process of articular cartilage fromnormal wear and tear activity of life time occupations and sports.Hereditary factors are significant as we see in collagen deficiencysyndromes such as multiple epiphyseal dysplasias. Metabolic diseasessuch as diabetes, alkaptonuria, hypercalcemic conditions and gout willincrease the rate of degenerative osteoarthritis. The initial degenerativeprocess begins with chondromalcia where the proteoglycan aggragateground substance is gradually lost thus exposing the supporting collagenfibers to mechanical breakdown and eventually the subchondral bonebecomes exposed to the joint surface in areas of greatest weight baring.Flakes of worn articular cartilage are swept to the joint periphery andbecome reattached and start the formation of osteophytes that are notfound in inflammatory arthritis where the primary source of the diseaseis synovial in nature. Cartilagenous flakes can also be phagocytised by
the synovial lining and later become ossified as we see in secondarysynovial osteochondromatosis. The breakdown products of articularcartilage can result in an autoimmune response in the synovium with aresultant secondary inflammatory synovitis with associated painsymptoms. Degenerative ossteoarthritis is frequently treated surgicallywith total joint arthroplasties. Degenerative spondylitis is the same aging process affected bywear and tear activities of normal life, occupational activities and sportswhich can be worsened by metabolic diseases as we saw in degenerativeosteoarthritis of the extremities. The primary source of this condition isa breakdown of the intervertebral disc which is composed of the samematerial as articular cartilage in peripheral joints. The degenerative discmaterial gradually extrudes thru the retaining annulus fibrosis with theresultant formation of reactive hypertrophic spurs to help stabilize theweakened disc. This process may result in nerve root compression thatmay require surgical decompression and stabilization procedures.
Osteoarthritis of the DIP finger jointsX-ray of hypertrophic spurformation of DIP joints of 70 male with OA Photo of ganglion cyst orHeberden’s node with macro- section of a similar case
Hypertrophic Spondylitis69 yr male with x-ray evidence of hypertrophic spurs and vacuum sign (yellow arrow) with macro section of resected specimen
Hypertrophic Spondylitis Cervical SpineTwo cases of severe hypertrophic spondylitis in older males
Pulmonary Osteoarthropathy There are two types of the disorder. The more common 96% form is asecondary pulmonary osteoarthropathy resulting from chronic disease ofthe lung, heart, liver, bloodstream or thymus carcinoma. The rarer 4%form or primary pulmonary osteoarthropathy is familial in males duringadolescence associated with enlargement of bones and joints, clubbing offinger tips and thickening of facial skin. The secondary form is mostcommonly seen in patients with bronchogenic carcinoma but can beseen associated with pulmonary TBc, lung abscess, chronic emphysemaand bronchiectasis. Clubbing of the finger tips results from swelling ofthe soft tissue about the distal phalanges and terminal osteolysis similarto that seen hyperparathyroidism. Synovitis of the small joints of thehands and feet with early juxta articular osteopenia can mimicrheumatoid arthritis. However, unlike rheumatoid disease, we see aperiostitis most common in the tibia, fibula, radius, ulna and lesscommon in the humerus, femur and phalanges. Once the primary diseaseis cured, the secondary problems of periostitis and synovitis disappear.
Secondary Pulmonary OsteoarthropathyPeriostitis of tibia Macro section of tibia
Neuropathic Arthropathy Neuropathic arthropathy is most likely a severe form of chronicdegenerative traumatic hypertrophic osteoarthritis induced by anyneurologic disorder that inhibits the normal protective sensation of painto the involved joint. The neurologic diseases include tabes dorsalis ofsyphilis, leprosy, syringomyelia, diabetes, paraplegia, myelodystrophy,spinal cord tumors, pernicious anemia, peripheral nerve damage, postop chordotomy patients and patients born with a congenital indifferenceto pain sensation. The process is initiated by an injury to a sensorydeficient joint usually in the lower extremity with damage to thestabilizing ligamentous structures and joint cartilage which goes unnoticed because of deficient protective pain sensation. The patientcontinues to use the joint before it has a chance to heal creating furtherdamage to the unprotected joint promoting a sequence of repetitivedamage to the joint surface with breaking off of osteochondralfragments that create loose bodies and synovitis with a large synovialeffusion, ligamentous laxity and joint instability. Secondary synovial
osteochondromatosis is seen on radiographic studies as the hypertrophicsynovium phagocytoses large segments of loose damaged articularcartilage which may create an image similar to that seen in tuberculousarthritis, ochronosis, gout or chondrosarcoma. If by chance a neuro-pathic joint becomes infected the resulting severe synovitis will createsymptoms of pain. The best treatment for neuropathic arthropathy is toprotect the sensory deficient joint from repeated injury with appropriatebracing. Surgical attempts at fusion or arthroplasty result in a highfailure rate second to infection, non union of attempted arthrodesis andaseptic loosening of arthroplasty components.
Neuropathic Hip Joints3 adult cases of neuropathic hipswith the upper two being second to syphilis and the lower to myelodysplasia
Syringomyelia induced Neuropathic Shoulders62 yr female 48 yr male
Syringomyelia induced Neuropathic Shoulder45 yr male with painless grinding sensations in shoulder due to syringomyelia of the cervicodorsal spine
Idiopathic Neuropathic Shoulder 62 yr male Bone scanCoronal MRI Axial MRI
Diabetic Neuropathic Feet Case #1 Case #1Case #1 is a diabetic mid tarsal neuropathic dislocation with resultant painless flat foot Case #2Case #2 is a more severe neuropathic diabetic pan talar dislocation
Luetic Neuropathic Spinal Deformity35 yr male with a neuropathic dislocation of L1 on L2 second to syphilis treated with a posterior spinal fusion
Luetic Neuropathic Spine Deformities52 yr male 59 yr male
Osteonecrosis & Osteochondroses Osteonecrosis and the osteochondroses are discussed together in mosttext books because in the past many of the heterogenous group ofosteochondroses where thought to be related to osteonecrosis when infact it is now known that only Perthes disease shows objective path-ologic findings of significant necrotic bone in the epiphysel area of theproximal femur in children. Osteonecrosis The most common cause for osteonecrosis is due to a traumaticdamage of the blood vessels that supply nutrients and oxygen to bone.many metabolic conditions can increase the risk of fracture with vasculardamage including osteoporosis, metastatic tumors to bone,myelogenoussarcomas and Gaucher’s disease. Osteomyelitis can result in vascularthrombosis and septic osteonecrosis. Elevated intra articular pressurefrom pyarthrosis or hemophiliac hemarthrosis of the hip joint can resultin avascular necrosis of the femoral head. Mobilization of neutral fatdroplets into the blood stream can result in small vessel occlusion and
osteonecrosis of the femoral and humeral heads in patients withCushing’s disease, alcoholism, sickle cell variants (SC and sickle Thal-assemia), dysbaric conditions (caisson disease), and patients on cortico-steroid medication. Other causes include gout, pancreatitis, radiationdamage and patients following renal transplantation. Collagen deficiencydisorders including Perthes disease have increased risk of pathologicfracture of the femoral head and resultant osteonecrosis. Idiopathicosteonecrosis is the terminology for those cases without a knownetiology. Epiphyseal bones such as the femoral head, medial femoralcondyle, humeral head, carpal lunate and narvicular and tarsal talus witha limited collateral circulation are more prone to avascular osteonecrosis.However, metadiaphyseal infarcts can be seen in long bones such as thetibia and femur. Osteochondroses The osteochondroses are a heterogenous group of conditions seen ingrowing children or young adults with painful disturbances seen radio-graphically in epiphyses, apophyses and epiphysoid bones (vertebralbodies, carpal and tarsal bones). They are are more common in boys and
trauma may be a precipitating factor that may result in avscular necrosisas in Perthes disease of the femoral head. Osteonecrosis is less of aproblem in osteochondroses of metatarsl heads, carpal lunate, tarsalnarvicular and the humeral capitalum. Perthes disease is the most outstanding of the osteochondrosesseen most commonly in boys between the ages of four and eight years.It is frequently seen in children with a retarded bone maturation patternsimilar to multiple epiphyseal dysplasias and hypothyoidism. Theclinical onset of a painful limp is frequently precipitated by a traumaticepisode. 40% of cases will involve both hips and even in the unilateralcases minor asymptomatic epiphyseal changes can be seen in theuninvolved side. A popular theory regarding the pathogenesis suggeststhat a preexisting weakened immature femoral epiphysis develops apathologic fracture with damage to the normally limited vascularity ina 4-8 year old resulting in avscular necrosis of the femoral headepiphysis. Radiologically one will then see fragmentation of theepiphyseal ossification center followed by flatening and sclerosis of thefemoral ossific nucleus and lateral extrusion and enlargement of the
femoral head as the necrotic bone is absorbed and gradually replaced bylive but deformed bone that sets the stage for early osteoarthritis andneed for a THA at an early age. During the early reparative stages onewill see lytic changes in the medial femoral neck metaphysis that cansuggest the diagnosis of TBc. Treatment consists of early none weightbaring in a brace of some type and maintaining acetabular coverage ofthe femoral head to avoid lateral extrusion of the repairing epiphysealbone which may require a corrective osteotomy. Freiberg’s infraction is seen most commonly in females betweenthe age of 13 and 18 years and consists of a pathologic collapse of thesecond metatarsal head that can result in minor avascular necrotic bonechanges. Treatment may require shortening of the second metatarsalbone or a partial condylectomy of the deformed metatarsal head. Kienbock’s disease is an osteochondrosis of the carpal lunate inpatients aged 20 thru 40 years and is common in manual laboringpatients suggesting a traumatic etiology in which a fracture may lead tominor changes of osteonecrosis. Surgical treatment may include radialshortening or ulnar lengthening to take pressure off the lunate or the
lunate can be replaced with a prosthesis. Kohler’s disease is an osteochondrosis of the tarsal narvicular boneseen more commonly in boys between the age of 3 and 7 years. Radio-graphically one sees flatening and sclerosis of the narvicular ossificationcenter associated with variable symptoms of pain which tends to correctitself spontaneously over a period of 2-4 years with mild reduction ofphysical activity. Some consider this condition as a normal variation ofepiphyseal maturation in the narvicular bone. Panner’s disease is an osteochondrosis of the capitulum of thehumerus seen usually in boys from 5-10 years and is linked to traumain which case it is referred to as “little leaguer’s elbow.” This conditioncan be seen in the Reinhardt-Pheiffer syndrome seen in Vol 24 undermesomelic dwarfism. Osgood-Schlatter disease is an osteochondrosis of the tibialtuberosity seen more commonly in boys involved in kicking andjumping sports. Painful swelling occurs over the tibial tuberosityassociated with radiographic evidence of fragmentation of the anteriortongue of the proximal tibial epiphysis. The pain usually resolves
spontaneously over a period of several years and in some cases anavulsion fracture separation of the weakened tuberosity requiressurgical replacement. Blount’s disease is an osteochondrosis of the medial aspect of theproximal tibial epiphysis. The more common severe infantile form ofBlount’s disease begins early at 1-3 years when normal physiologicbowing is seen. It is seen bilaterally in over half of the cases with obesityplaying a role by increasing the compressive forces on the medial tibialepiphysis and its adjacent growth plate resulting in damage to the weakgrowth cartilage with progressive varus deformity and shortening overthe growing years. Radiographically one will see early medialmetaphyseal beaking up to five years at which point fragmentation andresultant sclerosis will be seen breaking off and down from the medialedge of the medial tibial epiphysis. At age 11 years one will see atriangular ossific center formation in a split fork shaped growth platesimilar to the changes seen in congenital coxa vara of the hip. The lesssevere adolescent form begins at age 8-15 years, is bilateral in 90% ofcases and creates mild varus deformity and shortening. As with the
infantile form it is seen more commonly in blacks. Both forms mayrequire osteotomy corrective surgery. Scheuermann’s disease is an osteochondrosis of the ringapophyses of the mid and lower dorsal spine in adolescents with akyphotic deformity that may or may not be painful. 25% of cases arefamilial in nature suggesting that this condition may be a mild formof the more extensive spondyloepiphyseal dysplasias discussed in Vol20. Radiographically one will see irregularities and lytic changes in theanterior vertebral plates at three or five levels of the mid and lowerdorsal vertebrae associated with wedging and kyphosis followed laterwith anterior spurring an reactive sclerosis. Vertebral plate defects withintravertebral body herniations known as Schmorl’s nodes at the samelevel as the kyphosis might suggest the diagnosis of tuberculousspondylitis. These intravertebral hernations will lead to loss of discheight and early degenerative disease in later years. Sinding-Larsen-Johansson disease is an osteochondrosis ofthe lower pole of the patella similar to Osgood-Schlatter’s disease of thetibial tuberosity with the same fragmentation and sclerotic changes
seen at the attachment the patellar ligament to the lower patella. Sever’s disease is an osteochondrosis at the point of attachment ofthe heel cord to the os calsis seen in adolescents who may or may notexperience pain with radiographic findings of fragmentation andsclerosis similar to that seen in other traction apophyseal conditionsdiscussed above. Van Neck’s disease is a common osteochondrosis of the ischio-pubic synchondrosis in children ages 9 thru 11 years. Radiographicallyone will see evidence of enlargement and irregular ossification that maybe bilateral and could suggest an injury, infection or possible tumorformation. Femoral osteochondrosis & osteochondritis dissecansFemoral osteochondrosis is seen in children up through 17 years andusually occurs in the medial femoral condyle which may cause painwith activity with x-ray and MRI evidence of a subchondral defect dueto a localized retarded maturation of epiphyseal bone & can be bilateral,can be inherited as in multiple epiphyseal dysplasia, and because of theweakened subchondral area can go on to a traumatic sequestration of
a surface osteochondral fragment in teenagers or young adults resultingin the condition known as osteochondritis dissecans that usually requiressurgical intervention.
Femoral head necrosis in SC DiseaseX-ray of hip in 25 yr black male X-ray of cut resected specimenwith crescent sign in femoral of femoral head with crescenthead second to AVN in SC sign (yellow) and inflammatory disease reactive zone between dead bone above and live below (blue)
Pathology of femoral head necrosis in SC disease xGross specimen above ofresected femoral head withcrescent sign defect seen as wellin macro section to upper right.Lower right shows micro ofreactive bone formation aroundcentral necrotic bone lamina inreactive zone (x)
Avascular Necrosis in Caisson Disease45 yr old male caisson worker developed pain in L hip andshoulder with x-ray evidence of AVN in both areasMacro section of resected specimen
Metadiaphyseal Infarct of Distal FemurResection CT scan Macro section
Metadiaphyseal Infarct Distal Femur52 yr old female with a idiopathicdistal femoral infarct with resection specimen seen to your right.
Metaphyseal Infarct distal Femur & Tibia Sag T-1 MRI45 yr female with multiple metaphyseal steroid infarct about the knee
Metaphyseal Infarct Distal Femur38 year old male with incidental finding of a metaphyseal infarct
Metadiaphyseal Infarct Proxiaml Tibia Macro section Cor T-142 yr male with an incidental finding in the proximal tibia
Multi-tibial and femoral Idiopathic InfarctsSag T-1 Cor T-1 Cor T-1 74 yr female with tender lump mid pretibial area for 1 year
Six Year Radiographic Progression of Perthes Disease joint capsule 11th painful day 6 weeks 3 months 5 months5 year old male with early radiographic changes of Perthes disease
Later reparative change of Perthes Disease1 year 2 years 3 years 6 years
Pathogenesis of Perthes Disease Fragmentation epiphyseal ossificationdelamination necrotic bone Irregular growth plate Macro section of femoral head in early fragmentation stage
Frieberg’s Infraction18 year old female with pain at the base of the 3rd toe
Kohler’s Disease5 yr old male with slight pain in mid tarsal area for 1 yr
Panner’s Disease 6 yr 14 yr 20 yrOsteochondral defects seen in capitulum of different age groups
Femoral Osteochondrosis12 yr male with bilateral osteochondral defects with mild pain
Femoral Osteochondrosis17 yr male with a 5 yr historyOf knee pain with exercise andA large osteochondral defect inThe medial femoral condyle
Femoral Osteochondrosis12 year male with mild painin one knee for 6 months withMRI oateochondral defect in Cor T-1the lateral femoral condyle Axial T-1
Femoral Osteochondrosis16 yr male with 1 yr of knee pain & osteochondritic defect med condyle
Osteochondritis Dissecans17 yr male 30 yr male
Osgood-Schlatter’s Disease15 yr male soccer player withone yr history of pain andswelling over tibial tuberosityand x-ray evidence of osteo-chondral irregularities at thepoint of the patellar ligamentinsertion.
Kienbock’s Disease28 yr male laborer with wrist pain for one year
Severe Infantile form of Blount’s Disease 3 years 3 yr old obese black female withbowed legs and medial metaphyseal beaking on x-ray 11 years8 yrs later at age 11 yrs she developedpainful collapse and fragmentation of the medial tibial epiphysis
Scheuermann’s Disease Teen aged boy with lower dorsal kyphosis with multiple ringapophyseal defects and Schmorl’s nodes seen also on macro section
Three Separate Cases of Scheuermann’s Disease
Sever’s Disease Sag T-1 Sag T-212 yr old male with spontaneous heel pain for 4 months