A 4 year old with hip pain: Legg-Calvé-Perthes Disease Cyndie Seraphin Harvard Medical School Year III
Our Patient A 4 year-old boy is complaining of severe L hip pain. The differential diagnosis of acute hip pain in children is quite broad. The categories include Tumor, Trauma, Infection, Inflammatory processes, or Infarction/mechanical derangement. Does this child require imaging? What would be the appropriate radiographic evaluation of this patient?
Imaging Modalities for Acute Hip Pain Radiographic evaluation is necessary in all patients with septic arthritis, skeletal injury, or tumor in the differential diagnosis.
Acute Hip Pain: Plain Films and Ultrasonography Plain radiographs - AP views of the pelvis and frog-leg views (lateral view of femur) Identify bony aberrations/abnormalities, but may miss small effusions After Plain radiographs: Ultrasonography - If plain film is normal and you are suspicious of septic arthritis or synovitis, it can be used to identify small effusions. If plain film is abnormal, it can be used to guide arthrocentesis.
Acute Hip Pain: Radionucleotide Scan Radionucleotide scan- Acute setting: can be used to differentiate joint inflammation (septic arthritis or tissue synovitis) from osteomyelitis. Chronic setting: can identify avascular necrosis before abnormalities are visualized on plain film, as well as early tumors and myelodysplastic disease. Evidence Based? 50 children with hip pain were evaluated in a prospective study that probed the usefulness of imaging protocols in the diagnosis of hip pathology. In this protocol of plain films followed, as needed, by ultrasound and three-phase radionucleotide scans, the diagnosis of 48 patients was successfully identified. (Clinical Pediatrics 1988)
Acute Hip Pain: MRI and CT MRI and CT can be used if: a) the other modalities have not identified a diagnosis b) to provide better detail of a diagnosed abnormality MRI- can identify signs of osteomyelitis, early Legg- Calvé-Perthes disease, early Slipped Capital Femoral Epiphysis and cartilage destruction. In some cases, MRI with contrast may be preferable to bone scan, though it faces complications of availability, cost, and need for sedation. CT - may identify an intraabdominal cause of hip pain, such as appendicitis or psoas abscess.
Our Patient: Frontal Pelvis He received an AP film of his pelvis. What is the abnormality on the radiograph? Image courtesy of Dr. Jennifer Song
Normal Hip Anatomy http://children.webmd.com/hip-anatomy-in-a-child
Normal Blood Supply and Avascular Necrosis http://www.eorthopod.com/images/contentimages /hip/hip_fracture/hip_fracture_treatment04.jpg http://www.zimmer.co.uk/ctl?template=pc&op=global&action=1&id=7997
There is: widening of the femoral head Back to Our Patient: Frontal Pelvis flattening of the femoral head (coxa plana) The infarction has extended across the growth plate and a radiolucent lesion is evident within the metaphysis In addition, the growth center of the femoral head has been damaged so that normal growth is arrested, and shortening of the femoral neck results. AP View Pelvis Image courtesy of Dr. Jennifer So
Differential Diagnosis for Femoral Head Irregularity and Collapse Bilateral Hypothyroidism Multiple Epiphyseal Dysplasia Spondyloepiphyseal dysplasia tarda Sickle Cell Gaucher s Disease Meyer s Disease Unilateral Legg-Calvé-Perthes-Disease* Septic Arthritis Spondyloepiphyseal dysplasia tarda Sickle Cell Gaucher s Disease Meyer s Disease Eosinophilic granuloma *Can be bilateral in 10-20% of cases transient synovitis
Legg-Calvé-Perthes Disease (LCPD) Idiopathic osteonecrosis of the femoral head described independently in 1910 by Legg, Calvé, and Perthes. Rare, affecting 1 in 1200 children Affects mostly males, only about 1:4-5 are girls. About 10-20% of all diagnosed develop the disease in both hips. Most of these children are very active and often very athletic. The age of diagnosis is usually between 2 and 12 years old, with the average age of 6. Legg-Calvé-Perthes children tend to be of shorter stature.
Pathophysiology of LCPD Approximately 10 percent of cases are familial Symptoms include painless limp, pain, and restriction in movement at hip. Proposed causes: (controversial) An unusually high frequency of factor V Leiden and inherited coagulopathies has been noted in some reports among patients with LCP, suggesting thrombophilia as a contributor to avascular necrosis. Structural abnormalities of epiphyseal cartilage Association with prenatal secondhand smoke exposure and birth weight less than 2.5 kg in boys.
Evolution of LCPD Early on, you can have normal frontal pelvis radiographs. Frontal Pelvis views of Femoral Head Three Stages of LCPD: 1. Ischemia disrupts growth and femoral head becomes more dense with possible fracture of supporting bone 2. Fragmentation and reabsorption of bone Initial Phase Reabsorbtive Phase 3. Reossification when new bone has regrown, often with residual deformity when new bone reshapes. Antero-lateral head most affected Reossification/Healed Phase http://nonf.org/perthesbrochure/perthes-brochure.htm
Companion Patient Frontal Pelvis Initial Presentation: Painless Limping What abnormality do you see? At Presentation Ten Months later AP View Pelvis Image courtesy of Dr. Carolynn DeBenede
Companion Patient Frontal Pelvis Identifying Abnormalities 1. Radiolucent lesion at femoral head 2. Fragmentation and reabsorption of bone 3. Flattening, Widening and Reossification with residual deformity At Presentation Ten Months later AP View Pelvis Image courtesy of Dr. Carolynn DeBenede
Radiographic Findings in LCPD 1. Small femoral ossification nucleus 2. Lateral displacement of the femoral ossification nucleus 3. Fissuring and fracture of the femoral ossification nucleus 4. Flattening and sclerosis of the femoral ossific nucleus 5. Metaphyseal changes: widening and shortening of femoral neck
Example #1: Lateral displacement of Femoral ossification nucleus A very early finding! Displacement may range from 1-4mm laterally. Seen in majority of cases Image courtesy of Dr. Carolynn DeBenedectis
Example #2: Fissuring and fracture of the femoral ossification nucleus Radiolucent areas are seen, beginning in the anterior margin of the epiphysis The fracture fragment is clearly seen. Image courtesy of Dr. Carolynn DeBenedectis
Companion Patient: Evolution of LCPD on Radionucleotide Normal Hip RN Scan Scan Legg-Calvé-Perthes RN Scan Image courtesy of Dr. Carolynn DeBenedectis cold spot on the R femoral head Image courtesy of Dr. Carolynn DeBenedectis At this phase, radionucleotide scan shows decreased perfusion to the femoral head.
2 years later, hip radiograph showed: continued flattening and fragmentation of the L femoral head broadening of the femoral neck Patient had hip stiffness and limited range of motion. Cyndie Seraphin, 2011 Back to Our Patient: Frontal Pelvis AP View Pelvis Image courtesy of Dr. Jennifer Son
Catterall Classification Used to predict prognosis by graded involvement of femoral head and proximal structures -Groups I and II have a better prognosis -Groups III and IV have a relatively poor prognosis http://otto.oxfordmedicine.com/content/vol2/issue1/images/large/grap hic013018006.jpeg
Treatment The principle of treatment is protection of the joint. If the joint is deeply seated within the acetabulum and normal joint motion is maintained, a reasonably good hip can result. This can range from conservative management (NSAIDS) to surgical intervention.
Cyndie Seraphin, 2011 Treatment for Our Patient AP View Pelvis Image courtesy of Dr. Jennifer Song Left Frog Leg Lateral View Image courtesy of Dr. Jennifer Son He required osteotomy (to maintain femoral head within the acetabulum).
Frontal Pelvis at Three Year Follow Up AP View Pelvis Image courtesy of Dr. Jennifer Song While femoral head is still somewhat flattened, it is mostly covered by the acetabulum.
References Frick SL: Evaluation of the child who has hip pain. Orthop Clin North Am 2006;37(2):133 140. Wiig O, Terjesen T, Svenningsen S: Prognostic factors and outcome of treatment on Perthes' disease: A prospective study of 368 patients with five-year follow-up. J Bone Joint Surg Br 2008;90(10):1364 1371. Alexander JE, Seibert JJ, Aronson J, et al. A protocol of plain radiographs, hip ultrasound, and triple phase bone scans in the evaluation of the painful pediatric hip. Clin Pediatr (Phila) 1988; 27:175. Wenger DR, Ward WT, Herring JA. Legg-Calvé-Perthes disease. J Bone Joint Surg Am 1991; 73:778. Glueck CJ, Crawford A, Roy D, et al. Association of antithrombotic factor deficiencies and hypofibrinolysis with Legg-Perthes disease. J Bone Joint Surg Am 1996; 78:3. Uno A, Hattori T, Noritake K, Suda H. Legg-Calvé-Perthes disease in the evolutionary period: comparison of magnetic resonance imaging with bone scintigraphy. J Pediatr Orthop 1995; 15:362. The National Osteonecrosis Foundation. Johns Hopkins University. Legg-Calvé-Perthes Disease Brochure. 2000. McQuillen KK. Musculoskeletal disorders. In: Marx JA, Hockberger RS, Walls RM, et al, eds. Rosen s Emergency Medicine: Concepts and Clinical Practice. 7th ed. Philadelphia, Pa: Mosby Elsevier; 2009:chap 174. Sankar WN, Horn BD, Wells L, Dormans JP. Legg-Calve-Perthes disease. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 670.3.
Acknowledgements Dr. Jennifer Son Dr. Carolynn DeBenedectis Dr. Gunjan Senapati Dr. Gillian Lieberman Dr. Elizabeth Asch