Presentation on theme: "Pathologic Fractures in Children"— Presentation transcript:
1Pathologic Fractures in Children Joshua Klatt, MDOriginal Author: Steven Frick, MD; March 20041st Revision: Steven Frick, MD; August 20062nd Revision: Joshua Klatt, MD; January 2010
2Pathologic Fracture = Fracture through abnormal bone
3Pathologic FracturesAbnormal bone lacks normal biomechanic and viscoelastic propertiesIntrinsic processesLocalized - Bone cyst, neoplasm, etc.Systemic - OI, osteopenia, osteopetrosis, rickets, etc.Extrinsic processesRadiation, biopsy, defects after plate removal, etc.
4Osteopetrosis - failed fixation of femoral neck fracture. No osteoclasts = No remodeling.
5With every fracture:Ask the question -Is this fracture through NORMAL bone?
6Often Need to Do More than Treat the Fracture The orthopaedic surgeon may be the first to have opportunity to make the diagnosis. (malignancy, metabolic disease, etc.)
7Often Need to Do More than Treat the Fracture Differ from fractures in normal bone in that one must take into account…EtiologyNatural historyTreatment of underlying abnormalityMust treat both fracture and underlying cause!
8History Minor or no trauma? Any antecedent pain? Recent illness? Less than anticipated for fracture patternAny antecedent pain?Only with activity vs. night painRecent illness?Weight loss?Fevers?
9History Ask about growth and development Dietary habits Kidney disease May suggest rickets or renal osteodystrophy, etc.Thyroid diseaseFamily historyDysplasias, metabolic disorders, osteoporosis, neuromuscular disorders, etc.
10History Ask about prior malignancies, even in the child! Families will not always volunteer this information
11Physical Exam Look for soft tissue mass vs. fracture hematoma Other systems- skin, lymphatics, solid organsHeight - weight percentiles
14Radiographs Be suspicious! OsteopeniaPhyseal width (rickets)Soft tissue calcificationsPresence of massAny periosteal reaction
15Radiographs Is pathology… Localized and isolated? Polyostotic? Generalized to entire skeletal system?A generalized condition with skeletal manifestations?
16Enneking’s 4 Questions Where is lesion located? What is lesion doing to bone?What is bone doing to lesion?Are there clues to type of lesion?Enneking, et al. The surgical staging of MSK sarcoma. JBJS 62-A: , 1980.Enneking. A System of Staging MSK Neoplasms. CORR 204:9-24, 1986.
17Benign vs. Malignant Mankin’s Criteria SizeMarginationCortexSoft tissue massGebhardt, Ready & Mankin. Tumors about the knee in children.Clin Orthop 255:86-110, 1980.
18Categorize/Make Diagnosis Benign bone lesionMalignant bone lesionInfectionMetabolic bone diseaseSkeletal dysplasiaNeuropathicOsteopenia-disuseOveruse
19TreatmentUnion best achieved by correcting biomechanical and biological environmentWhile chemo & radiation slow healing, they provide a beneficial response in presence of rapidly dividing malignant cells
20Simple Bone Cyst (Unicameral Bone Cyst) Not true neoplasms, etiology unknownOften loculated and not truly “unicameral”Most frequently contain serous fluidUsually metaphysealProximal humeral & femoral lesions account for 94% of all lesionsMost in patients 3-14 years old, average age 9Males > females (2:1)Baig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.
21SBC Pathologic Fracture Fallen leaf sign (or fragment) is virtually pathognomonicTreatmentFracture heals; cyst persist in 50-90%Humerus - treat fracture, address lesion after fracture is healed, if felt to be necessaryDisplaced proximal femur #s - Open reduction, grafting and rigid fixation, unless very youngPosterior facet #s of the calcaneus - Open reduction, if necessary with grafting and fixation
22SBC Treatment Controversial! Open ManagementCurettage/graftBone graft substitutesMinimally invasive techniques (injections)Steroid injectionsBone marrow injectionsAll seem to work with similar frequency (~90%)But can be recurrence with any of them!Disrupt hydraulics- puncture, screw, wires, rods, etc.
23SBC Injection 18 ga spinal needle C-arm Serous fluid, straw colored 2nd needle- ventDepo-Medrol 160 mgWatch for immediate drainage from large outflow veinsMay need multiple injections
24LJ, 8 yo with arm pain when throwing, injected once with methylprednisolone (multiple sites), healing at 3 months
25SBC - Risk Factors for Recurrence Only reliable predictor of treatment success is age of the patient> 10 yrs heal ~ 90% of time< 10 yrs heal ~ 60% of timeMost cysts tend to heal after skeletal maturityBaig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.Spence et al. Solitary unicameral bone cyst: treatment with freeze-dried crushedcortical-bone allograft. JBJS-A 58:636-41, 1976
27Aneurysmal Bone Cyst (ABC) ExpansileOften wider than physisEccentricAggressive at marginsCottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.
28Aneurysmal Bone Cyst (ABC) Symptoms usually present for < 6 monthsLesion may attain considerable size before recognizedCan exist as…primary bone lesion (70%)secondary lesion in other osseous conditions (30%)Pelvic lesions account for 50% of all flat bone lesions (~10% total)Treatment is difficult due to inaccessibility and integrity of acetabulumCottalorda et al. Aneurysmal Bone Cysts of the Pelvis in Children. J Pediatr Orthop. 25:471-5, 2005.
31ABC Look for fluid-fluid levels on MRI (however, not especially specific)Bur, et al. Fluid-fluid levels in a unicameral bone cyst: CT and MR findings. J Comput Assist Tomogr 17:134-6, 1993.Papagelopoulos, et al. Treatment of aneurysmal bone cysts of the pelvis and sacrum. JBJS-A 83: , 2001.
325 yo female with 1 year of hip pain and 4 prior steroid injections, progressive coxa vara. Biopsy = ABC
33Curettage, biopsy consistent with aneurysmal bone cyst
341 month after curettage, bone grafting, valgus/internal fixation, spica immobilization
35ABC Curettage and bone graft +/- internal fixation ? Injection of fibrosing agent (Ethibloc, Ethicon, etc.) is controversialHigh recurrenceCottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.Adamsbaum et al. Intralesional Ethibloc injection in primary ABCs. Skeltal Radiol. 32:559-66, 2003.Varshney et al. Is Sclerotherapy Better than Intralesional Excision for treating ABCs. CORR epib 2009.
36Nonossifying Fibroma (NOF) Benign, nonosteoid-producing lesionUsually found in metaphyses of long bonesPrediliction for lower extremitiesUsually asymptomaticOften incidental radiographic findingIt is speculated that up to a 1/3 of children may have at least a minor NOF/fibrous cortical defectAlmost always regress by early 20’sBetsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.
37Nonossifying Fibroma (NOF) Most treated non-op!Let fracture heal, excellent healing potentialMost NOF’s persist after #, but heal by skeletal maturityIf fractures once with minimal trauma, potential risk to fracture again unless bone changes with healingIf necessary, treat with curettage/bone graftBetsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.
3810 yo male - running during soccer. NOF fracture - at 4 weeks underwent allograft DBM / cancellous bone graft.Healed at 9 mos.
39NOF - Prophylactic Bone Graft? Are size parameters predictable?Arata and Peterson, JBJS 1981Review of 23 fractures over 50 yearsSuggest tx if greater than 50% diameter, >33 mm lengthEasley and Kneisl, JPO 1997Review of 22 lesions, many without #s, over 25 yearsOnly included large lesions (above criteria)Only 41% had fractures, no refracturesSuggest prophylactic surgery not necessary in manyCriteria for surgery still not well definedArata et al. Pathological fxs through NOFs. JBJS-A. 63:980-8, 1981.Easley & Kneisl. Pathologic fxs through NOFs: is prophylactic treatmentwarranted? J Pediatr Orthop 17:808-13, 1997.
40Fibrous Dysplasia Developmental disorder of bone, etiology unclear May be mutation leading to activation of c-fos oncogeneCan be associated with endocrine disorders (McCune-Albright syndrome)Can be mono- or polyostoticUsually affects adolescents and young adultsMany solitary asymp lesions found incidentallyMost do not require interventionIf increased fracture risk, treat with curettage, bone grafting and sometimes internal fixationParekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.
41Fibrous Dysplasia Surgical treatment for: Progressive lesions Large lesions with painPain & deformity suggest microfracturesFailure of conservative treatmentLess successful in:Younger patientsLarger and proximal femoral lesionsPolyostotic disease, esp McCune-Albright syndromeEnneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by corticalbone-grafting. JBJS-A 68: , 1986
42Fibrous Dysplasia Lesions are never eradicated, even with grafting All grafts are eventually absorbed into dysplastic boneCortical grafts at a much slower rate and therefore recommended for weight-bearing bonesEnneking suggested cortical struts alone for femoral neckFixation in WB bones seems to improve outcome in childrenEnneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by corticalbone-grafting. JBJS-A 68: , 1986.Stephenson et al. Fibrous dysplasia: An analysis of options for treatment. JBJS-A69:400-9, 1987.
4314 yo female - fell walking across front yard 3 months of left hip pain - MotrinReferred for “path fx through Ewing’s sarcoma”Dx -polyostotic fibrous dysplasia
443 Years PostopNotice resorption of graft and recurrence of cystic changes in femoral neck. Can have secondary ABC develop within fibrous dysplasia.
45Fibrous Dysplasia Consider other sites (polyostotic disease) Bone scan to help identify other lesionsAlthough lesions usually revealed on scan, a ‘cold’ bone scan does not rule out lesionsFor extensive involvement (McCune-Albright) consider intramedullary fixation/splinting
4611 yo male – fem neck path fx, nondisplaced 11 yo male – fem neck path fx, nondisplaced. Fibular allograft (neck) and titanium elastic nails (subtroch and shaft)
4713 yrs old – 2 years postop. lesions in Rt. femur and tibia 13 yrs old – 2 years postop. lesions in Rt. femur and tibia. No pain in hip, in karate. Fibular graft gone. Treat painful tibia? Nail? Pamidronate?
49Prophylactic Treatment of Fibrous Lesions (NOF /FD) Any mechanical pain?Location and size - relative issuesSupracondylar femur, proximal femur more worrisomePharmacologic approach (bisphosphonates) for painful fibrous dysplasia – some reported successesParekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.DiCaprio & Enneking. Fibrous dysplasia. Pathophysiology, evaluation andtreatment. JBJS-A87: , 2005.
50Osteogenesis Imperfecta (OI) Abnormal type I collagenCOL1A and COL2A defectsLocation and type of mutation in collagen molecule determine phenotype (Sillence)Severe types (II-IV)- multiple fractures prior to skeletal maturityAlso find joint laxity, gray-blue sclera, dentogenesis imperfecta, premature deafness, kyphoscoliosis & basilar invaginationLower extremity > upper extremitySillence et al. Genetic heterogeneity in osteogenesis imperfecta. J Med Gen. 16:101-16, 1979.Van Dijk et atl. Classification of Osteogenesis Imperfecta revisited. Eur J Med Genet 53:1-5, 2010.
51OI Early onset (fxs prior to walking)- more fractures (2x) Direct relationship between increased bone turnover and severity of diseaseClosed tx- limit immobilization time to reduce further osteopeniaIM fixation often neededAlso used for realignment surgery and to decrease fracture riskD’Astous & Carroll: Connective Tissue Diseases, in Vaccaro (ed):OKU 8. Rosemont, IL, AAOS, 2005.
55OI- New Methods Minimize disuse osteoporosis Early IM fixation New designExpandable nailsBisphosphonatesIncrease bone densityOsteoclast inhibitorsDecrease fracture rateOral and IV effectivePhillipi et al. Bisphosphonate therapy for OI. Cochrane Database Syst Rev 8(4), 2008Panigrahi et al. Response to zolendronic acid in children with type 3 OI.J Bone Biner Metab, Feb 4, [Epub ahead of print]
56Ollier’s Disease (Multiple Enchondromas) Linear masses of cartilage in metaphyseal and diaphyseal regions of long bonesAsymmetric, often unilateralUsually sporadic occurrencePathologic fx may occurLewis et al. Benign and malignant cartilage tumors.Instr Course Lect 36:87-114, 1987.
577 yo male - femur fracture jumping on bed Enchondromatosis Rt. femur/tibia/pelvis
58OsteomyelitisInfection should always be in differential of pathologic fracturesHowever, pathologic fracture uncommon in osteomyelitisOften delayed diagnosisMore common in weight-bearing bones (i.e. femur, tibia)Involucrum may be supportive of diagnosisTaylor et al. Childhood osteomyelitis presenting as a pathologic fracture.Clin Rad 63:348-51, 2008.Gelfand, et al. Path fx in acute osteo of long bones secondary to communityAcquired MRSA? Am J Med Sci 332:357-60, 2006.
623 yo with spina bifida, swollen leg Consult = DVT vs. infection?X-ray - healing fx may look like malignancy
6311 yo male - Duchene Muscular Dystrophy Hip pain for 2 months. Disuse fracture/nonunion
64Stress FracturesUsually through normal bone subjected to abnormal stressesMay be mistaken for more serious pathology (esp. longitudinal stress fxs)History of recent increased activityProximal tibia, distal fibula, metatarsals most commonTreat with rest, which can be challenging to achieveHeyworth & Green. Lower extremity stress fractures in pediatric andadolescent athletes. Curr Opin Pediatr 20:58-61, 2008.
6512 yr old male, activity related pain, training for baseball, running 6 miles per day, referred for Ewing’s sarcoma.Dx: Longitudinal femoral stress fracture
66Stress Fractures Can occur through pathologic bone Congenital abnormalities, metabolic disorders (osteoporosis, osteomalacia)
67Congenital Tibial Dysplasia Presented at age 10 after fracture from minor traumaHad “bowed leg”her entire lifeNo other msk abnormalitiesSakamoto et al. Congenital pseudarthrosis of the tibia: analysis of thehistology and the NF1 gene. J Orthop Sci 12:361-5, 2007.
68Malignant Appearing Pathologic Fracture Referral to musculoskeletal oncologistRequires complete stagingBiopsy needed - follow proper “rules” for biopsyNotify pathologist of fractureAvoid fracture callus (histology may look malignant)Biopsy soft tissue mass
73Pediatric Pathologic Fxs Be suspicious - scrutinize every fracture film!Most frequently a benign processMake the diagnosis to guide treatmentAppropriate referral / workup for suspected malignancyProphylactic treatment for benign lesions on an individual basis
74BibliographyEnneking, et al. The surgical staging of MSK sarcoma. JBJS 62-A: , 1980.Enneking. A System of Staging MSK Neoplasms. CORR 204:9-24, 1986.Gebhardt, Ready & Mankin. Tumors about the knee in children. Clin Orthop 255:86-110, 1980.Baig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.Spence et al. Solitary unicameral bone cyst: treatment with freeze-dried crushed cortical-bone allograft. JBJS-A 58:636-41, 1976Cottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.Cottalorda et al. Aneurysmal Bone Cysts of the Pelvis in Children. J Pediatr Orthop. 25:471-5, 2005.Bur, et al. Fluid-fluid levels in a unicameral bone cyst: CT and MR findings. J Comput Assist Tomogr 17:134-6, 1993.Papagelopoulos, et al. Treatment of aneurysmal bone cysts of the pelvis and sacrum. JBJS-A 83: , 2001.Adamsbaum et al. Intralesional Ethibloc injection in primary ABCs. Skeltal Radiol. 32:559-66, 2003.Varshney et al. Is Sclerotherapy Better than Intralesional Excision for treating ABCs. CORR epib 2009.Betsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.Arata et al. Pathological fxs through NOFs. JBJS-A. 63:980-8, 1981.Easley & Kneisl. Pathologic fxs through NOFs: is prophylactic treatement warranted? J Pediatr Orthop 17:808-13, 1997.Parekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.Enneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by cortical bone-grafting. JBJS-A 68: , 1986Stephenson et al. Fibrous dysplasia: An analysis of options for treatment. JBJS-A 69:400-9, 1987.DiCaprio & Enneking. Fibrous dysplasia. Pathophysiology, evaluation and treatment. JBJS-A87: , 2005.Sillence et al. Genetic heterogeneity in osteogenesis imperfecta. J Med Gen. 16:101-16, 1979.
75BibliographyVan Dijk et atl. Classification of Osteogenesis Imperfecta revisited. Eur J Med Genet 53:1-5, 2010.D’Astous & Carroll: Connective Tissue Diseases, in Vaccaro (ed): OKU 8. Rosemont, IL, AAOS, 2005.Phillipi et al. Bisphosphonate therapy for OI. Cochrane Database Syst Rev 8(4), 2008Panigrahi et al. Response to zolendronic acid in children with type 3 OI. J Bone Biner Metab, Feb 4, [Epub ahead of print]Lewis et al. Benign and malignant cartilage tumors. Instr Course Lect 36:87-114, 1987.Taylor et al. Childhood osteomyelitis presenting as a pathologic fracture. Clin Rad 63:348-51, 2008.Gelfand, et al. Path fx in acute osteo of long bones secondary to communit acquired MRSA? Am J Med Sci 332:357-60, 2006.Heyworth & Green. Lower extremity stress fractures in pediatric and adolescent athletes. Curr Opin Pediatr 20:58-61, 2008.Sakamoto et al. Congenital pseudarthrosis of the tibia: analysis of the histology and the NF1 gene. J Orthop Sci 12:361-5, 2007.
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