oite 2010 disease

OITE 2010

DISEASES

Ortho Diseases

3. A 32-year-old man has had a 2-year history of left groin pain. Radiographs, MRI scans, and a biopsy specimen are seen in Figures 3a through 3f. What is the most likely diagnosis?

1- Enchondroma

2- Chondroblastoma

3- Giant cell tumor

4- Osteonecrosis

5- Clear cell chondrosarcoma

3 A

3 B

Ortho Diseases

3. A 32-year-old man has had a 2-year history of left groin pain. Radiographs, MRI scans, and a biopsy specimen are seen in Figures 3a through 3f. What is the most likely diagnosis?

1- Enchondroma

2- Chondroblastoma

3- Giant cell tumor

4- Osteonecrosis

5- Clear cell chondrosarcoma

Clear Cell Chondrosarcoma• Rare variant of chondrosarcoma(2% of all

chondrosarcoma, 0.2% of biopsy-analyzed primary bone tumors)

• Usually low grade, slow growing, infrequent metastases, but frequent local recurrence

• Male:Female 1.3:1• most commonly proximal humerus or femur

(75%). 15% around the knee(distal femur and proximal tibia). In about 10%, the lesion has been noted in the skull, spine, ribs, pelvis,ulna or phalanges.

• Epiphyseal +/- extension into metaphysis.• young adults, 20- 50 yo

• XR: expansile radiolucent bony lesion, absence of any periosteal reaction and absence of soft tissue mass +/- calcifications

• MR: Well demarcated, T1 -relatively homogeneous low to intermediate signal intensity, T2- heterogeneous high signal intensity

• Path: large round-to-oval tumor cells w/ distinct borders, abundant clear cytoplasm and a centrally located round nucleus. Typically see woven bone trabeculae within the microlobules or scattered between sheets of tumour cells. Giant cells present.

• S-100 positive• Tx: operative resection with a wide margin.

References:SPRINGFIELD DS, GEBHARDT MC, MCGUIRE MH: Instructional Course Lectures, The American

Academy of Orthopaedic Surgeons - Chondrosarcoma: a Review. J. Bone Joint Surg. Am., Jan 1996; 78: 141 - 9.

AYOUB KS, GRIMER RJ, CARTER SR, MANGHAM DC, DAVIES AM, TILLMAN RM:. Clear cell chondrosarcoma of bone. Sarcoma (1999) 3, 115-119.

Itälä A; Leerapun T, Inwards C, Collins M, Scully SP: An Institutional Review of Clear Cell Chondrosarcoma. Clin Orthopaedics and Related Research Number 440, pp. 209–212

Recommended Reading:• Seo SW, Remotti F, Lee FYI. Chondrosarcoma

of bone. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:185-195.

• Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:201.

Ortho Diseases

4. When patients with rheumatoid arthritis are being treated with the disease-modifying

antirheumatic drug adalimumab, what is the optimal time for surgical intervention?

1- Immediately after infusion2- Approximately 10 days after injection3- Only after a year of continuous therapy4- When drug levels are at their highest5- When the pharmacokinetic levels reach

equilibrium

Adalimumab (Humira)

• Mechanism: TNF α Inhibitor• TNFα - highly inflammatory macrophage-derived

cytokine, plays a critical role in the joint destruction of patients with RA

• TNF α Inhibitors provide symptomatic and functional improvement and slows radiographic progression of disease

• Enhances the risk of infection with mycobacteria and other opportunistic microorganisms, as well as increasing risk of postoperative infections.

• Half life 10-13.6 daysReferences:Giles JT, Bartlett SJ, Gelber AC, Nanda S, Fontaine K, Ruffing V, Bathon JM. Tumor necrosis factor

inhibitor therapy and risk of serious postoperative orthopedic infection in rheumatoid arthritis. Arthritis Rheum. 2006 Apr 15;55(2):333-7.

Pfeiffer M, Koller M, Wnivernhaus A, Trieb K. A postoperative infectious complication in a patient with rheumatoid arthritis treated with adalimumab. Z Rheumatol 2006 Dec;65(8): 725-7

Recommended reading:• Shojania K, Esdaile JM, Gredanus N. Arthritis.

In: Vaccaro AR, ed. Orthopaedic Knowledge Update 8. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2005:234-239.

• Giles JT, Bartlett SJ, Gelber AC, Nanda S, Fontaine K, Ruffing V, Bathon JM. Tumor necrosis factor inhibitor therapy and risk of serious postoperative orthopedic infection in rheumatoid arthritis. Arthritis Rheum. 2006 Apr 15;55(2):333-7. PubMed PMID: 16583385.

Ortho Diseases

21. Which of the following is a recognized complication of extra-articular corticosteroid injections?

1- Acne

2- Depression

3- Fat atrophy

4- Hypogonadism

5- Manic episode

Fat Atrophy

• More common with less soluble agents (eg. Triamcinilone)

• Effects can last >6mo• Other side effects steroid injections: Post-

injectional flare (1-10%), facial flushing (15%, women>men), infection, sepsis, skin depigmentation, perilymphatic atrophy, hyperpigmentation,tendon rupture.

Recommended Reading/References:• Cole BJ, Schumacher HR Jr. Injectable

corticosteroids in modern practice. J Am Acad Orthop Surg. 2005 Jan-Feb;13(1):37-46. Review. PubMed PMID: 15712981.

• Kumar N, Newman RJ. Complications of intra- and peri-articular steroid injections. Br J Gen Pract. 1999 Jun;49(443):465-6. PubMed PMID: 10562748; PubMed Central PMCID: PMC1313446.

Ortho Diseases

24.Figures 24a through 24c show the radiographs and CT scan of a 7-year-old girl who has had mild discomfort and a hard mass on the anterior tibia for 1 year. What is the most likely diagnosis?

1- Chondroblastoma2- Ewing’s sarcoma3- Ossifying fibroma4- Aneurysmal bone cyst5- Parosteal osteosarcoma

24A

24B

Ortho Diseases

24.Figures 24a through 24c show the radiographs and CT scan of a 7-year-old girl who has had mild discomfort and a hard mass on the anterior tibia for 1 year. What is the most likely diagnosis?

1- Chondroblastoma2- Ewing’s sarcoma3- Ossifying fibroma4- Aneurysmal bone cyst5- Parosteal osteosarcoma

Ossifying Fibroma• Typically occurs in the jaw bone, only rarely in long bones where they

have been termed Osteofibrous dysplasia (OFD)• OFD:rare (0.2% all bone tumors), benign, self-limited fibroosseous

lesion, usually in the diaphysis of the tibia but occasionally ipsilateral fibula is involved

• First two decades of life• benign lesion that rarely progresses during childhood and never

progresses after skeletal maturity• P/w lower leg swelling +/- pain, anterior bowing or path fx. Often

incidental finding• XR: intracortical lytic lesion, well marginated and is often surrounded

by a zone of sclerosis. Cortex may be expanded or thickened. Typically involves anterior cortex.

• .CT can provide more information on the amount of cortical destruction and may reveal occult pathologic fracture

• MRI can aid in diagnosis by identifying the lesion and assessing for extraosseous soft tissue or intramedullary extension. OFD generally contained within the cortex.

Pathoogy: •loose, often storiform fibrous background containing spicules of woven bony trabeculae that are lined by a layer of osteoblasts. •Osteoblastic rimming differentiates from fibrous dysplasia. •Devoid of devoid of epithelial differentiation, which differentiates fro AD

DDX: Adamantinoma(AD), fibrous dysplasia, nonossifying fibroma, UBC, ABC, chondromyxoid fibroma, Langerhans cell histiocytosis (ie, eosinophilic granuloma), osteomyelitis, osteosarcoma, chondrosarcoma, hemangioendothelioma, angiosarcoma, and metastatic carcinoma

• Consider open biopsy even if radiographically c/w OFD as could be early AD lesion.

• Treatment controversial– Traditionally tx with observation. Bracing may be

attempted to minimize deformity and prevent fracture. Surgery reserved for extensive or deforming lesion, or path fx.

– Some have recommended aggressive surgical approach with extraperiosteal resection in all cases. Advocates site sampling error in biopsy and theory that OFD could progress to AD

References:Most MJ, Sim FH, Inwards CY Osteofibrous Dysplasia and Adamantinoma J. Am. Acad. Ortho. Surg., June

2010; 18: 358 - 366.

Schoenecker PL, Swanson K, Sheridan JJ. Ossifying fibroma of the tibia. Report of a new case and review of the literature. JBJS 1981; 63:483-488.

Recommended Reading:• Pitcher JD Jr, Weber KL. Benign fibrous and

histiocytic lesions. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:121-132.

• Damron TA: Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:156-158.

Ortho Diseases

32. A 24-year-old woman reports a 4-month history of knee pain. Radiographs, an MRI scan, and biopsy specimens are seen in Figures 32a through 32e. What is the most likely diagnosis?

1- Chondrosarcoma

2- Ewing’s sarcoma

3- Giant cell tumor

4- Periosteal chondroma

5- Conventional osteosarcoma

32A

32B

32D

32E

Ortho Diseases

32. A 24-year-old woman reports a 4-month history of knee pain. Radiographs, an MRI scan, and biopsy specimens are seen in Figures 32a through 32e. What is the most likely diagnosis?

1- Chondrosarcoma


3- Giant cell tumor

4- Periosteal chondroma

5- Conventional osteosarcoma

Osteosarcoma

• Most common bone sarcoma• Peak incident 2nd decade, assoc w/ peak growth• Most commonly occurs at sites of rapid bone turnover

(eg. the distal femur, prox tibia, and prox humerus). Less commonly occurs in the pelvis, the spine, and the craniofacial bones.

• p/w pain, decreased ROM, mass, warmth or erythema, or path fx.

• XR: aggressive bone lesion arising from the metaphysis distal femur, proximal tibia, or proximal humerus. Illdefined borders, osteoblastic and/or osteolytic features, and an associated soft-tissue mass.

• MRI: demonstrates extent of tumor invasion of the surrounding soft tissues, neurovascular involvement, extent of bone marrow replacement, and presence of discontinuous metastases (ie, skip lesions)

• Biopsy

• Path: malignant mesenchymal cells w/ pleomorphic nuclei and osteoid

Osteosarcoma Subtypes• Conventional: most common, 1st/2nd decade. High grade,

originating from intramedullary cavity.• Telangiectatic osteosarcoma: <4%. Eccentric and osteolytic

lesion, may resemble ABC. Composed of blood filled sinusoids, with high signal on T2.

• Low Grade: 1-2%. 3rd/4th decade. Tumor may resemble fibrous dysplasia, but MRI shows cortical invasion.

• Small Cell: 1.5%. Lytic areas and variable amount os sclerosis. Path small, round, malignant ells w/in osteoid matrix.

• Parosteal: Arise surface, sparing intramedullary canal. Pead 3rd decade. Females>males. Low grade, well=differentiated fibrowus stroma with osseous components.

• Periosteal: 1-2%. More aggressive that paraosteal, Sunburst pattern or codman triangle commonly seen. Intermed grade tumor, mostly cartilaginous with areas of calcification.

• High Grade: <1%. Surface tumor. High grade spindle cells, w/ atypia & varying amounts of osteoid.

• Tx: multidisciplinary. • Low grade or parosteal: Wide surgical resection.• High grade lesions or mets: pre-op chemo, wide

surgical resection, post-op chemo• Commonly used chemo agents: doxorubicin,

cisplatin, MTX, and ifosfamide• Without mets, 70% survival rate• With clinically detectable mets, 20-30% survival

rate

Messerschmitt PT, Garcia RM, Abdul-karim FW, Greenfield EM, Getty P. Osteosarcoma. JAAOS Aug 2009; 17:515-527

Recommended Reading:• Damron TA. Orthopaedic Surgery Essentials,

Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:177-185.

• Hornicek FJ. Osteosarcoma of bone. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:163-174.

Ortho Diseases

45.A 13-year-old boy has had right knee pain for the past 4 months. Radiographs, bone scan, CT scan, and biopsy specimen are seen in Figures 45a through 45e. What is the most likely diagnosis?

1- Osteomyelitis

2- Chondroblastoma


4- Eosinophilic granuloma

5- Aneurysmal bone cyst

3 A

3 B

45A

45B

3 C

45C

Ortho Diseases

45.A 13-year-old boy has had right knee pain for the past 4 months. Radiographs, bone scan, CT scan, and biopsy specimen are seen in Figures 45a through 45e. What is the most likely diagnosis?

1- Osteomyelitis

2- Chondroblastoma


4- Eosinophilic granuloma

5- Aneurysmal bone cyst

Chondroblastoma• Benign neoplasm primarily affecting patients 10-

25 years old with a male predominance of 2:1 • Most common sites include distal femur,

proximal tibia, proximal humerus although in older populations it tends to involve flat bones.

• Characteristic radiographic findings include well-circumscribed lesions centered in an epiphysis of a long bone. In children, a well circumscribed epiphyseal lesion crossing an open growth plate is virtually diagnostic. There can be a surrounding rim of reactive bone and 30-50% will have some calcification evident on radiograph. Usually there is no soft tissue involvement.

• Microscopically, there are sheets of chondroblasts with background of chondroid matrix. Calcification is present giving a "chicken wire" appearance. Multinucleated giant cells are abundant.

• In adults, differential diagnoses include giant cell tumors (no rim of sclerotic bone or calcification) and clear cell chondrosarcoma

• Treatment includes extended curettage, bone grafting and/or bone cement.

Recommended Reading

- Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:135-137.

- Weber KL, O’Connor MI. Benign cartilage tumors. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:103-120.g:

Ortho Diseases

57. Optimal management of osteoporosis diagnosed after a hip fracture includes

1- urgent medical consultation.

2- administration of bisphosphonates with follow-up as needed.

3- referral to the patient’s primary care physician within a year of surgery.

4- initiation of vitamin D and calcium supplementation with follow-up as needed.

5- initial evaluation by the orthopaedic team with early postoperative referral to an osteoporosis clinic.

• Hip, wrist, vertebral fractures in the elderly from low energy mechanisms are termed fragility fractures as the forces involved would not fracture healthy bone.

• Miki et al's paper in JBJS 2008 was a randomized control trial involving 62 patients with fragility hip fractures. Their mean age was 79.2. Each patient was assigned to either an intervention or a control group. Patients in the intervention group were worked up for osteoporosis, treated and followed closely in a specialized orthopaedic osteoporosis clinic. Patients in the control group was started on treatment and told to follow up with their primary care doctors.

• The paper found that patients randomized to follow up in the specialized orthopaedic osteoporosis clinic had much a much high percentage of patients on osteoporotic medication and getting surveillance studies at 6 months as compared to the control group.

Recommended reading:

- Miki RA, Oetgen ME, Kirk J, Insogna KL, Lindskog DM. Orthopaedic management improves the rate of early osteoporosis treatment after hip fracture. A randomized clinical trial. J Bone Joint Surg Am. 2008 Nov;90(11):2346-53. PubMed PMID: 18978403.

- Murray MA. Bone metabolism and metabolic bone disease. In: Vaccaro AR, ed. Orthopaedic Knowledge Update 8. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2005:187-196.

Ortho Diseases

67. Which of the following diagnostic modalities is most useful for the diagnosis of proximal thigh deep venous thrombosis?

1- D-dimer assay

2- Physical examination

3- Venous ultrasonography

4- Computed tomography

5- Impedance plethysmography

Deep vein thrombosis

• 80-90% occur in operated limb• Peak incidence is 4 days after surgery and minimal after

day 17. • Clinical diagnosis of pain, tenderness, positive Homan

sign, swelling, erythema, low grade fever is absent in 50% of patients

• Venography is still considered most sensitive and specific test for calf and thigh DVTs but is costly, invasive, poses risk of contrast allergy or inducing thrombosis

• Ultrasound has a sensitivity of 79%, specificity of 98% and accuracy of 97% compared to venography but is not as useful in the diagnosis of calf and pelvic thrombi

Recommended Reading/References:

- Conduah AH, Lieberman JR. Thromboembolism and pulmonary distress in the setting of orthopaedic surgery. In: Einhorn TA, O’Keefe, RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:105-113.

- Haut ER, Chang DC, Pierce CA, Colantuoni E, Efron DT, Haider AH, Cornwell EE 3rd, Pronovost PJ. Predictors of posttraumatic deep vein thrombosis (DVT): hospital practice versus patient factors-an analysis of the National Trauma Data Bank (NTDB). J Trauma. 2009 Apr;66(4):994-9; discussion 999-1001. PubMed PMID: 19359905.

Ortho Diseases

78.

Paget’s disease of bone results from a defect in which of the following processes?

1- Bone resorption

2- Bone regeneration

3- Osteodifferentation

4- Matrix maturation and mineralization

5- Coupling of bone formation and resorption

Ortho Diseases

78.Paget’s disease of bone results from a defect in

which of the following processes?1- Bone resorption2- Bone regeneration3- Osteodifferentation4- Matrix maturation and mineralization5- Coupling of bone formation and resorption

Paget disease• Disorder of unregulated bone turnover. Excessive osteoclastic

resorption is followed by increased osteoblastic activity. An early lytic phase is followed by excessive bone production with cortical and trabecular thickening.

• More common in people over 55 years old of Anglo-Saxon descent. • Radiographic findings vary based on the stage of the disease. In the

lytic phase, the bone can take on a "blade of grass" or "flame" appearance at the end of the bone extending to the diaphysis. Later on, the bone is sclerotic with thickened cortices and trabeculae. Bone scans can be hot. MRI is used to differentiate between malignancy with the marrow in Paget disease appearing normal.

• Paget is managed medically with NSAIDs, calcitonin and bisphosphonates.

• Alkaline phosphatase levels and urine pyridinium cross-links is used to monitor disease activity.

• ~1% of patients with Paget disease develop a sarcoma, usually osteosarcoma.

Recommended Reading/References:- Zuscik MJ, Drissi MH, Chen D, Rosier RN. Molecular and

cell biology in orthopaedics. In: Einhorn TA, O’Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:3-24.

- Miller JD, McCreadie BR, Alford AI, Hankenson KD, Goldstein SA. Form and function of bone. In: Einhorn TA, O’Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:129-159.

Ortho Diseases

87.A 19-year-old college football player has had

suprapubic pain for the past 4 months. A radiograph and CT scans are show in Figures 87a through 87c. What is the most likely diagnosis?

1- Chronic adductor strain2- Osteoid osteoma3- Chronic pelvic instability4- Stress fracture5- Osteitis pubis

32A

32B

87A

87B

87C

Ortho Diseases

87.A 19-year-old college football player has had

suprapubic pain for the past 4 months. A radiograph and CT scans are show in Figures 87a through 87c. What is the most likely diagnosis?

1- Chronic adductor strain2- Osteoid osteoma3- Chronic pelvic instability4- Stress fracture5- Osteitis pubis

Osteitis Pubis

• Painful, noninfectious, inflammatory condition involving the pubic bone, symphysis and surrouding structures.

• Associated with urological/gynecological surgery and athletes.

• Gradual onset of pain in the pubic region is the primary symptom and pain may be severe enough to limit ambulation.

• Radiographic changes include symmetrical bone resorption at the medial ends of the pubic bones, widening of the pubic symphysis, and rarefaction or sclerosis along the pubic rami.

• Usually self limiting and treatment includes rest, anti-inflammatory medication. If symptoms persist, corticosteroid injections and wedge resection of the symphysis pubis may be necessary.

Recommended Reading/References:- Sekiya JK, Gibbs AE. Groin and pelvis injuries:

In: Kibler WB, ed. Orthopaedic Knowledge Update: Sports Medicine 4. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2009:83-90.

- Nuccion SL, Hunter DM, Finerman GAM. Hip and pelvis: adult. In: DeLee JC, Drez D Jr, Miller MD, eds. DeLee and Drez’s Orthopaedic Sports Medicine, 2nd ed. Philadelphia, PA: WB Saunders; 2003:1448.

Question 104

Paget’s disease is best characterized as increased bone turnover that results:1- from a sequenced missense genetic mutation.2- from an autosomal-recessive inheritance pattern.3- in significant deformity but is rarely painful.4- in subtle radiographic findings that necessitate advanced imaging.5- in increased urinary N-telopeptide and alpha-C-telopeptide.

Question 104

Paget’s disease is best characterized as increased bone turnover that results:

1- from a sequenced missense genetic mutation.2- from an autosomal-recessive inheritance pattern.3- in significant deformity but is rarely painful.4- in subtle radiographic findings that necessitate advanced imaging.5- in increased urinary N-telopeptide and alpha-C-telopeptide.

Question 104

• Paget’s disease - common geriatric problem, occurring in 3% to 4% of the population over 50 years of age

• Increased bone resorption leads to a compensatory increase in bone formation. The overall rate of bone remodeling is accelerated, resulting in a predominance of highly vascular bone that is structurally weak and prone to deformities and pathologic fractures

• The measurement of serum alkaline phosphatase activity is a good initial biochemical test for Paget’s disease. But, can be elevated in other diseases as well

• Most sensitive test is urinary N-telopeptide and alpha-C-telopeptide.

Question 104

- Metabolic bone markers relating type I collagen degradation products provide important information on Paget’s

- urinary N-telopeptide (NTX) and alpha-C-telopeptide (CTX) for bone resorption have emerged as the most sensitive ones

Type I Collagen

References

Murray MA. Bone metabolism and metabolic bone disease. In: Vaccaro AR, ed. Orthopaedic Knowledge Update 8. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2005:187- 196.

Delmas PD. Biochemical markers of bone turnover in Paget’s disease of bone. J Bone Miner Res. 1999 Oct;14 Suppl 2:66-9. Review. PubMed PMID: 10510216.

Question 112

A 7-year-old girl has activity-related leg pain. Radiographs and biopsy specimens are seen inFigures 112a through 112d. What is the most likely diagnosis?

1- Unicameral bone cyst2- Nonossifying fibroma3- Osteofibrous dysplasia4- Giant cell tumor5- Eosinophilic granuloma

Question 112• A few fun facts about

nonossifying fibromas:

– Most common fibrous lesion in children and adolescents

– Radiolucent, eccentric, cortically based lesions

– Most undergo spontaneous regression, usually starting at the end of adolescence and disappear by age 20 to 25 years.

Distinct, sclerotic margins often with scalloping

Most commonly located in the distal femoral metaphysis, followed by the proximal and distal tibial metaphyses.

There is no intralesional matrix mineralization, but nonossifying fibromas can have an internal “bubbly” appearance that is created by thin, white septae resulting from ridging of the cortex

References

• Pitcher JD Jr, Weber KL. Benign fibrous and histiocytic lesions. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:121-132.

• Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:149-151.

Question 131Patients with multiple hereditary exostosis have a greater risk of the development of what kind of mesenchymal tumor?

1- Hemangioma2- Enchondroma3- Chondrosarcoma4- Chondroblastoma5- Extra-abdominal desmoid

Question 131• Hereditary multiple exostoses (HME)

– an autosomal dominant condition, mutation of tumor suppressor genes EXT1 or EXT2

– prevalence 1/50,000

• Enlargement of exostoses rarely continues beyond skeletal maturity

• Presence of an enlarging mass in an adult patient with HME should raise concerns about malignant transformation

• 5% of cases go on to malignant transformation

– Most commonly chondrosarcoma– Lesions arising from the pelvic and

shoulder girdles are more likely to undergo sarcomatous transformation Erlenmeyer flask deformity of HME

Question 131

• Weber KL, O’Connor MI. Benign cartilage tumors. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:103-120.

• Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:133.

Question 150A 60-year-old patient who has diabetes mellitus with peripheral neuropathy has a plantar ulcer on the plantar surface of the second metatarsophalangeal joint. Examination reveals no fluctuance or purulent drainage, pulses are palpable, and the ulcer does not involve bone. Range of motion of the ankle reveals dorsiflexion to neutral and 15° of plantar flexion. Which of the following treatments offers the lowest recurrence rate of ulceration?

1- Early débridement and excision of the metatarsal head2- Second ray resection3- IV antibiotics with ulcer débridement4- Total contact casting5- Total contact casting with gastrocnemius recession

Question 150

• Key is recognizing that limited dorsiflexion has been implicated as a contributing factor to excessive plantar pressures and forefoot skin breakdown leading to recurrence

• Mueller et al:– Randomized clinical trial involving 64 DM pts with plantar foot ulcers– TCC vs TCC with percutaneous Achilles lengthening

TCC TCC with Achilles lengthening

Initial healing 88% 100% (p>0.05)

Recurrence < 7 months 59% 15% (p=0.001)

Recurrence 2 years 81% 38% (p=0.002)

Question 150• Lin SS, Lee TH, Wapner KL. Plantar forefoot ulceration with equinus

deformity of the ankle in diabetic patients: the effect of tendo-Achilles lengthening and total contact casting. Orthopedics. 1996 May;19(5):465-75. PubMed PMID: 8727341.

• Mueller MJ, Sinacore DR, Hastings MK, Strube MJ, Johnson JE. Effect of Achilles tendon lengthening on neuropathic plantar ulcers. A randomized clinical trial. J Bone Joint Surg Am. 2003 Aug;85-A(8):1436-45. PubMed PMID: 12925622.

Question 171Figures 171a through 171g show the radiographs, MRI scans, and biopsy specimens of a 9-year-old girl who reports right elbow pain that is worse at night. What is most likely diagnosis?

1- Ewing’s sarcoma2- Osteomyelitis3- Giant cell tumor4- Aneurysmal bone cyst5- Eosinophilic granuloma

Question 171• Ewing’s Sarcoma Clinical Features:

– Second most common bone tumor (after osteosarcoma) occurring in children

– t(11;22)(q24;q12) – Most common presenting symptoms of

ES are pain, swelling, and a mass– 20% have fever

• Radiographic Features on X-ray:– Location: 50/50 flat bones vs. long

bones– Long bones: typically at metadiaphysis

(58.7%) or diaphysis (35.4%)– Typically, ES appears as an ill-defined,

permeative, mottled, or focally motheaten, destructive intramedullary lesion.

– Periosteal reaction, with a laminated or “onion skin” appearance (a prominent multilayered reaction)

Question 171373 patients in theIntergroup Ewing’s Sarcoma

Study 7299, common radiographic findings

Included:1. poor margination (ie,

indistinct lesion borders 96%)

2. soft-tissue component(80.4%)

3. permeative component (76.1%)

4. laminated periosteal reaction (56.6%)

5. sclerotic component (39.7%)

Question 171

• On pathology: small round uniform cells with high nucleus-tocytoplasmic ratio and grow in dense, solid sheets

Question 171

• Patterson FR, Basra SK. Ewing’s sarcoma. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:175-183.

• Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:187-191.

204. A 26-year-old woman has had wrist pain for the past 3 weeks. Radiographs and an MRI scan are seen in Figures 204a through 204d. A biopsy specimen is seen in Figure 204e. What is the most likely diagnosis?

1- Osteosarcoma 2- Giant cell tumor 3- Large cell lymphoma 4- Metasatatic carcinoma 5- Nonossifying fibroma

Giant cell tumors are commonly seen in the knee (50%), distal radius (as in this case), and proximal humerus. They are more common in females. Radiographically, they are seen as well circumscribed purely lytic lesions. Pathology shows sheets of multi-nucleated giant cells.

-Osteosarcoma commonly occurs about the knee, proximal humerus, proximal femur and pelvis of children and young adults. Radiographs show poorly defined bone forming lesions with both bone destruction and formation. Path demonstrates lacelike mineralizing osteoid surrounding atypical osteoblasts.-Lymphoma is most frequently seen in the knee, pelvis, proximal femur, and vetebra, and can occur in all ages. Lymphoma of bone is typically diffuse large B-cell type. Xrays show poorly circumscribed lytic lesions. Path shows marrow replacement by a uniform population of lymphoid cells.-Metastatic disease is the most common lesion seen in older patients, particularly over the age of 40. Most common carcinomas to metastisize to bone are breast, lung, prostate, kidney and thyroid. Most common locations are the pelvis, vertebral bodies, ribs and proximal limb girdles. Radiographs can show lytic, mixed, or purely sclerotic lesions.-Nonossifying fibroma is a benign lesion most common seen in the distal femur, distal tibia, and proximal tibia of young patients. Characteristic radiographic appearance is a lucent lesion that is metaphyseal, eccentric, and surrounded by a sclerotic rim. Histology shows a whorled pattern of fibroblastic cells with giant cells.

204.

Mirra JM. Bone Tumors. Philadelphia, PA: Lea and Febiger; 1989:941-1022.

McDonald DJ, Weber KL. Giant cell tumor of bone. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:133-140.

References

224. Figures 224a and 224b show the radiographs of a 40-year-old woman with a familial history of ‘high arches’ with weakness and feelings of lateral ankle instability. Significant contribution to the development of this deformity comes from which of the following?

1- Hindfoot arthritis 2- Deltoid insufficiency 3- Contracture of the gastrocsoleus complex 4- Atrophy of the peroneus brevis muscle 5- Weakness of the posterior tibialis muscle

High longitudinal arches, or pes cavus, occur frequently in neurological disorders such as Charcot-Marie-Tooth. Weakness and atrophy of the tibialis anterior, extensor hallucis longus and the peroneus brevis are found in pes cavus. This leads to an imbalance in the plantar and dorisiflexors, as well as invertors and evertors of the foot and produces plantarflexion of the first ray, hindfoot varus, and the cavus malalignment.Patients tend to complain of painful calluses under the first and fifth metatarsals and the medial heel.The Coleman block test is important in determining the flexibility of the hindfoot varus. This is important in determining treatment.Work up should include a family history and neuro exam, as well as EMG to evaluate for neuromuscular disorders.Unilateral or asymmetric deformity can result from cerebral palsy, spinal cord tumors, or a tethered cord.Bilateral deformity can be caused by muscular dystrophy, cerebral palsy, CMT, Friedreich’s Ataxia, Polio, or be idiopathic.

224.

Alexander IJ, Johnson KA. Assessment and management of pes cavus in Charcot-Marie-Tooth disease. Clin Orthop Relat Res. 1989 Sep;(246):273-81. PubMed PMID: 2766615. Paulos L, Coleman SS, Samuelson KM. Pes cavovarus. Review of a surgical approach using selective soft-tissue procedures. J Bone Joint Surg Am. 1980 Sep;62(6):942-53. PubMed PMID: 7430182.

References

241. Figure 241 shows the radiograph of a 40-year-old man who reports a 1-year history of forefoot pain and swelling. Examination reveals synovitis and pitting of the nails. What is the most likely diagnosis?

1- Gout 2- Hallux rigidus 3- Reiter’s syndrome 4- Psoriatic arthritis 5- Rheumatoid arthritis

Psoriatic arthritis is an seronegative spondyloarthropathy that affects 10-30% of people with psoriasis. Classic findings include inflammatory arthritis of distal joints, sausage digits (dactylitis), changes in the nails (pitting, onycholysis, keratosis), pain the in the feet and ankles, and pain in the sacrum. Periarticular erosions can lead to the classic “pencil-in-cup” sign on xrays.

-Gout tends to affect the 1st MTP joint (50-75% initial cases). Characteristic radiographic signs are inordinate soft tissue enlargement and bony erosions on both sides of the joint.-Hallux rigidus, or stiff big toe, is a degenerative condition that affects the MTP joint.-Reiter’s Syndrome (Reactive arthritis) is an autoimmune phenomena that can develop after bacterial infection (Chlamydia, Salmonella, Shigella, etc). Characterized by triad of inflammatory arthritis of large joints, inflammation of the eyes (conjuctivitis or uveitis) and urethritis (“can’t pee, can’t see, can’t climb a tree”).-RA affects the forefoot > midfoot/hindfoot. Also tends to predominantly affect the MTP joint.

241.

Berberian WS, Najarian RG. Midfoot and forefoot arthritis and hallux rigidus. In: Pinzur MS ed. Orthopaedic Knowledge Update: Foot and Ankle 4. Rosemont, IL; American Academy of Orthopaedic Surgeons; 2008:201-214.

Shurnas PS, Coughlin MJ. Arthritic conditions of the foot. In: Coughlin MJ, Mann RA, Saltzman CL, eds. Surgery of the Foot and Ankle, 8th ed. Philadelphia, PA: Mosby Elsevier; 2007: 856-863.

References

253. A 43-year-old woman who is right-hand dominant fell onto her outstretched arm while rollerblading 1 day ago. She reports a painful wrist. Examination reveals swelling and tenderness dorsally. Radiographs reveal a nondisplaced transverse fracture of the distal radius. She is placed in a short arm cast. What can be done to reduce the risk of type 1 complex regional pain syndrome?

1- Transcutaneous electrical nerve stimulation 2- Occupational therapy treatment for finger dexterity 3- Strict elevation above the heart for 72 hours 4- Alpha adrenergic blockers for 2 weeks after injury 5- Daily oral vitamin C for 2 months

Type 1 complex regional pain syndrome (CRPS), formerly known as reflex sympathetic dystrophy, is characterized by pain, swelling and changes in the skin in the absence of an identifiable nerve lesion. Type II, or causalgia, by comparison is a result of a nerve injury. Classically, patients are thought to progress through three stages of disease (although they may be more accurately thought of as three distinct types of the disease):1 - severe burning pain, redness, warmth, hyperhydrosis, muscle spasm, rapid hair and nail growth, and vasospasm2 - intense pain, hard edema, muscle and skin atrophy3 - irreversible skin changes (cool, glossy, dry skin), unyielding pain, marked muscle atrophy and stiffness/joint contractures

Incidence of CRPS is higher in women, upper extremity>lower extremity. There is no correlation with age. Common in distal radius fractures

Vitamin C has been been shown to have a protective effect against CRPS. A randomized clinical trial published in JBJS in 2007 showed a decrease in the incidence of complex regional pain syndrome following distal radius fracture, with a recommended dosing of 500mg daily for 50 days. Across the treatment dosages, there was a prevalence of CRPS in the treatment group of 2.4% (8 of 328 patients) vs. 10.1% (10 of 99 patient) of those given placebo (p=0.002).

Treatments for CRPS include:NSAIDs, centrally acting analgesics (cymbalta, neurontin, amitryptilene, etc), vasodilators, steroids, transcutaneous electrical stimulation, neurolysis. Treatments are typically more effective the earlier in the disease process they are initiated.

253.

Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am. 2007 Jul;89(7):1424-31. PubMed PMID: 17606778.

Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet. 1999 Dec 11;354(9195):2025-8. PubMed PMID: 10636366.

References

263. The antirheumatic drug anakinra’s mode of action is via inhibition of which of the following?

1- IL-1 2- IL-6 3- Rheumatoid factor 4- Leukocyte production 5- TNF-α

Anakinra (brand name Kineret) is a competitive inhibitor of the interleukin-1 receptor. It is a approved for treatment of RA in patients with moderate or severe disease who have had an inadequate response to other disease-modifying antirheumatic drugs (DMARDs). By blocking IL-1, it prevents the inflammatory and immunologic effects of this mediator.It is administered as a subcutaneous injection daily, typically for 24 weeks. It can not be co-administered with TNF-a inhibitors, and no head-to-head studies comparing the two have been performed. Can be given in with methotrexate.Common side effects include infections (40%, 2% severe), nausea, diarrhea and GI upset, pain and erythema at injection site (70%), and a decrease in neutrophil count (8%).

263.

Shojania K, Esdaile JM, Greidanus N. Arthritis. In: Vaccaro AR, ed. Orthopaedic Knowledge Update 8. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2005:234-239.

Kalliolias GD, Liossis SN. The future of the IL-1 receptor antagonist anakinra: from rheumatoid arthritis to adult-onset Still’s disease and systemic-onset juvenile idiopathic arthritis. Expert Opin Investig Drugs. 2008 Mar;17(3):349-59. Review. PubMed PMID: 18321234.

References

275. A 10-year-old girl has a painful right tibial tubercle. She has swelling but no fever. Figures 275a through 275e show AP and lateral radiographs, CT scans, and an MRI scan. Figure 275f shows a biopsy specimen. What is the most likely diagnosis?

1- Healing fracture 2- Adamantinoma 3- Ewing’s sarcoma 4- Chondroblastoma 5- Eosinophilic granuloma

Eosinophilic granuloma is a form of Langerhans Cell Histiocysosis (Histiocytosis X). It is a condition that mimics primary bone neoplasm. Presents with pain and swelling and any bone can be involved. Most frequently, a single bone is involved, but multiple bones can be affected. Radiographs will show a highly destructive lesion with well defined margins. Histology shows characteristic proliferating eosinophilic Langerhans cells, as well as multiple bilobed eosinophils with bright eosinophilic cytoplasm. It is commonly a self-limiting process, although low dose radiation, or curettage with bone grafting when articular surface is at risk or fracture is imminent are options.

-Adamantinoma is a rare neoplasm of young adults found most frequently in the tibia, but the fibula, femur, ulna and radius are infrequently involved. Radiographs shows multiple “bubbly” sharply circumscribed lucent defects of different sizes, with sclerotic bone between.-Ewing’s Sarcoma is a small round cell sarcoma of children and young adults. Most commonly found in pelvis, distal femur, proximal tibia, femoral diaphysis, and proximal humerus. Xrays demontrate a large destructive lesion that may be purely lytic or have variable amounts of reactive new bone formation. May have characteristic “onion skin” appearance caused by periosteum lifted off in multiple layers.- Chondroblastoma is a benign cartilage tumor found in young patients with open physes. Most common locations are the distal femur, proximal tibia and proximal humerus. Radiographs show a central region of bone destruction separated from the medullary cavity by a rim of sclerotic bone. Histo shows chondroblasts and scattered multinucleated giant cells.

275.

Mirra JM. Bone Tumors. Philadelphia, PA: Lea and Febiger; 1989:1022-1045.

Pitcher JD, Weber KL. Benign fibrous and histiocytic lesions. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:121-132.

References

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