a 4 year old with hip pain: legg-calvé-perthes...

A 4 year old with hip pain:

Legg-Calvé-Perthes Disease

Cyndie SeraphinHarvard Medical School Year III

Gillian Lieberman, MD

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

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?

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Cyndie Seraphin, 2011Gillian Lieberman, MD

Imaging Modalities for Acute Hip Pain

Radiographic evaluation is necessary in all patients with septic arthritis, skeletal injury, or tumor in the differential diagnosis.

December 2011

Cyndie Seraphin, 2011Gillian Lieberman, MD

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.

December 2011

Cyndie Seraphin, 2011Gillian Lieberman, MD

Acute Hip Pain: Radionucleotide ScanRadionucleotide 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)

December 2011

Cyndie Seraphin, 2011Gillian Lieberman, MD

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.

Acute Hip Pain: MRI and CT

December 2011

He received an AP film of his pelvis.

What is the abnormality on the radiograph?

Our Patient: Frontal PelvisCyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Image courtesy of Dr. Jennifer Song

Normal Hip Anatomy

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

http://children.webmd.com/hip-anatomy-in-a-child

Cyndie Seraphin, 2011Gillian Lieberman, MD

Normal Blood Supply and Avascular Necrosis

http://www.zimmer.co.uk/ctl?template=PC&op=global&action=1&id=7997

December 2011

http://www.eorthopod.com/images/ContentImages /hip/hip_fracture/hip_fracture_treatment04.jpg

There is:widening of the femoral head

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.

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Image courtesy of Dr. Jennifer SoAP View Pelvis

Back to Our Patient: Frontal Pelvis

Cyndie Seraphin, 2011Gillian Lieberman, MD

Differential Diagnosis for Femoral Head Irregularity and Collapse

Bilateral UnilateralHypothyroidism Legg-Calvé-Perthes-Disease*

Multiple Epiphyseal Dysplasia Septic Arthritis

Spondyloepiphyseal dysplasia tarda Spondyloepiphyseal dysplasia tarda

Sickle Cell Sickle Cell

Gaucher’s Disease Gaucher’s Disease

Meyer’s Disease Meyer’s Disease

Eosinophilic granuloma

transient synovitis*Can be bilateral in 10-20% of cases

December 2011

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.

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Pathophysiology of LCPDApproximately 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 cartilageAssociation with prenatal secondhand smoke exposure and birth weight less than 2.5 kg in boys.

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Evolution of LCPDEarly on, you can have normal frontal pelvis radiographs.

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

3. Reossification when new bone has regrown, often with residual deformity when new bone reshapes.

Antero-lateral head most affected http://nonf.org/perthesbrochure/perthes-brochure.htm

Reossification/Healed Phase

Reabsorbtive Phase

Initial Phase

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Frontal Pelvis views of Femoral Head

Companion Patient Frontal Pelvis Initial Presentation: Painless

LimpingWhat abnormality do you see?

At Presentation Ten Months later

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

AP View Pelvis Image courtesy of Dr. Carolynn DeBenede

Companion Patient Frontal Pelvis Identifying Abnormalities1. Radiolucent lesion at femoral head2. Fragmentation and reabsorption of bone3. Flattening, Widening and Reossification with residual deformity

At Presentation Ten Months later

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

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

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

A very early finding!

Displacement may range from 1-4mm laterally.

Seen in majority of cases

Cyndie Seraphin, 2011Gillian Lieberman, MD

Example #1: Lateral displacement of Femoral

ossification nucleus

December 2011

Image courtesy of Dr. Carolynn DeBenedectis

Radiolucent areas are seen, beginning in the anterior margin of the epiphysis

The fracture fragment is clearly seen.

Example #2: Fissuring and fracture of the femoral

ossification nucleus

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Image courtesy of Dr. Carolynn DeBenedectis

Cyndie Seraphin, 2011Gillian Lieberman, MDCompanion Patient:

Evolution of LCPD on Radionucleotide Scan

Normal Hip RN ScanLegg-Calvé-Perthes RN

Scan

•At this phase, radionucleotide scan shows decreased perfusion to the femoral head.

“cold spot” on the R femoral head

December 2011

Image courtesy of Dr. Carolynn DeBenedectis Image courtesy of Dr. Carolynn DeBenedectis

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.

Back to Our Patient: Frontal Pelvis

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Image courtesy of Dr. Jennifer SonAP View Pelvis

Catterall Classification

http://otto.oxfordmedicine.com/content/vol2/issue1/images/large/grap hic013018006.jpeg

•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

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

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, 2011Gillian Lieberman, MD

December 2011

Treatment for Our Patient

He required osteotomy (to maintain femoral head within the acetabulum).

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

Image courtesy of Dr. Jennifer Song Image courtesy of Dr. Jennifer SonAP View Pelvis Left Frog Leg Lateral View

Cyndie Seraphin, 2011Gillian Lieberman, MD

Frontal Pelvis at Three Year Follow Up

While femoral head is still somewhat flattened, it is mostly covered by the acetabulum.

December 2011

Image courtesy of Dr. Jennifer SongAP View Pelvis

ReferencesFrick 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.

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011

AcknowledgementsDr. Jennifer Son

Dr. Carolynn DeBenedectis

Dr. Gunjan Senapati

Dr. Gillian Lieberman

Dr. Elizabeth Asch

Cyndie Seraphin, 2011Gillian Lieberman, MD

December 2011