Case Author(s): Michael Quinn, M.D. and Tom R. Miller, M.D.,Ph.D. , 12/10/97 . Rating: #D2, #Q4

Diagnosis: Engelmann's disease

Brief history:

52 year-old man with pain in both feet.

Images:

Anterior and posterior whole body images are shown

View main image(bs) in a separate image viewer

View second image(xr). AP radiograph of left tibia

Full history/Diagnosis is available below

Diagnosis: Engelmann's disease

Full history:

52 year-old man with bilateral foot pain. He also has a family history which is remarkable for "congenital bone abnormality" involving his father.

Radiopharmaceutical:

Tc-99m MDP

Findings:

Both femurs and tibias are abnormal. There is increased uptake of radiopharmaceutical with widening of the diaphyses throughout their length with associated prominence of the cortices. The remainder of the axial and appendicular skelton also have increased tracer uptake, but to a lesser degree. Renal activity is decreased due to the increased bone uptake. The focus of increased uptake in the left medial tibial metaphysis is due to an exostosis.

Discussion:

Engelmann's disease, also known as progressive diapyseal dysplasia, is categorized with the sclerosing bone dysplasias such as osteopetrosis and melorheostosis. It is characterized by marked thickening of the cortices of the diaphyses of tubular bones, occuring on both periosteal and endosteal surfaces. It usually presents in the young child with gait disturbance and limb pain. Autosomal dominant transmission has been noted, though expression is variable. Males are affected more often than females.

Differential Diagnosis List

Infantile cortical hyperostosis (Caffey's disease), hyperphosphatasia, Van Buchem's disease, osteopetrosis, fibrous dysplasia

ACR Codes and Keywords:

References and General Discussion of Bone Scintigraphy (Anatomic field:Skeletal System, Category:Normal, Technique, Congenital Anomaly)

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Case number: bs088

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