Metaphyseal Sclerotic Bands Associated with Cyclical Bisphosphonate Therapy in Children

Clinical Cases 02.07.2008
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 13 months, female
Authors: Ankers D, Harvie J, Perry DC. Royal Liverpool Childrens' (Alder Hey) Hospital, Liverpool, UK.
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Clinical History

We present the case of a 13-month-old girl on cyclical bisphosphonate therapy who had unusual sclerotic lines throughout the metaphyses of her long bones.

Imaging Findings

A 13-month-old girl, with known osteogenesis imperfecta (type III), presented to the Emergency department with a painful right leg.

The child had previously presented on multiple occasions with long bone fractures. Medical therapy with cyclical bisphosphonates had been instigated.

Plain radiograph revealed a right proximal femoral fracture. Multiple transverse sclerotic lines were also noted through the metaphyses of the long bones. The aetiology and nature of these lines was unfamiliar to the orthopaedic team.

The fracture was successfully treated in a hip spica.

Discussion

Bisphosphonates have been at the cornerstone of cyclical OI management. Pamidronate in particular has been associated with improved clinical outcomes, reduced bone resorption, and increased bone density (1). The mechanism of action involves inhibition of bone resorption and stimulation of PTH-mediated anabolic effects on osteoblastic processes (2).

Numerous papers throughout the literature have reported similar sclerotic lines associated with cyclical bisphosphonates therapy in the immature skeleton (3,4,5) – recently termed ‘Zebra Lines’ (6). Each line represents a bisphosphonates treatment cycle and between these bands linear growth is demonstrated. Banding intervals are dependant upon patient age, bisphosphonate dosing regimen and rate of growth. The band is thought not to represent growth arrest but to to represent a layer of primary spongiosa from developing bones which has failed to appropriately remodel due to osteoclast inhibition (6). Another reported change associated with bisphosphonate is a subtle metaphyseal undertubulation (7)

The key differential with such sclerotic lines are those of Harris growth arrest lines (7). These occur in the immature skeleton during periods of systemic illness such as malnutrition, infection or chemotherapy. During this time bone growth ceases and the trabeculum thicken and fuse to form a dense band which becomes apparant once normal bone growth has resumed.

Both Harrison growth arrest lines and Zebra lines undergo remodelling and therefore disappear with progression into the disphysis. Owing to their nature zebra lines are more easily remodelled and hence with growth their presence is usually restricted to the metaphysis, whereas growth arrest lines may extend further in the diaphysis (6).

Other sclerotic lines with appear in the metaphysis may arise from trauma, lead poinsoning, healing rickets and chronic anaemia.

Differential Diagnosis List

Bisphosphonate associated sclerotic bands - "Zebra Lines"

Final Diagnosis

Bisphosphonate associated sclerotic bands - "Zebra Lines"

Liscense

Figures

AP Pelvis and Lower Extremities

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AP Pelvis and Lower Extremities