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CASE 94

CASE 94

By: Dr. Aliya Sarhan Al Barwani.

HISTORY: Young boy, X-ray done after history of fall.

What are the findings?

What is the diagnosis?

Does it cross the joints?

 

FINDINGS:

Plain Radiograph: The anterior cortex of the tibia is significantly thickened with features of a dripping wax appearance. There is no evidence of cortical break or fracture.

CT shows similar findings of dripping wax appearance or flowing wax appearance.

 

DIAGNOSIS:

Melorheostosis of the tibia.

It can cross the joint and the patient may present with joint contracture or pain.

PEARLS AND DISCUSSION:

Melorheostosis is a sclerosing bony disease manifested by periosteal and cortical thickening with a characteristic dripping wax appearance or a flowing candle wax appearance. It usually starts during childhood and progresses to adulthood as joint pain, deformity and dysfunction secondary to contractures.

It has the tendency of sclerotome distribution and may associates with scleroderma skin distribution. Commonly seen in long bones, then hands and feet and rarely in axial skeleton.

 

FURTHER READING:

1.      Smith GC, Pingree MJ. Melorheostosis: A Retrospective Clinical Analysis of 24 Patients at the Mayo Clinic. PM R. (2016)

2.      Boulet C, Madani H. Sclerosing bone dysplasias: genetic, clinical and radiology update of hereditary and non-hereditary disorders. Br J Radiol. (2016)

3.      Ashish G, Shashikant J.  Melorheostosis of the Foot: A Case Report of A rare entity with a Review of Multimodality Imaging Emphasizing the Importance of Conventional Radiography in Diagnosis. J Orthop Case Rep. (2016)

4.      Nassar K, Mkinsi O. Melorheostosis. Pan Afr Med J. (2014)

CASE 93

CASE 93

CASE 95

CASE 95