Changes in the Head of the Femur in Children Suffering From Gaucher's Disease

Abstract

1. Two children suffering from Gaucher's disease, who developed changes in the femoral head typical of Perthes' disease, are reported. Similar changes have been recorded in the literature in seventeen children under the age of fifteen years. 2. The possible factors giving rise to the bone changes are discussed and it is considered that they result from aseptic necrosis. Splenectomy does not appear to hasten the development of bone changes in this disease. 3. In one of the patients, the blood Wassermann reaction was positive, but syphilis was not thought to play any part in the production of the bone changes.

Osseous Gaucher's Disease in Early Childhood

“…In none of the reported cases has the removal of the spleen accelerated the bone changes. 10 Because of the extreme variability of the clinical progression of Gaucher's disease, it is difficult to predict the future course of this patient. There is no direct relationship between intensity of cell de¬ posits and the clinical manifestations, however, decreased resistance subjects the patients to re¬ peated infections.…”

Systemic Lupus Erythematosus (SLE) and Aseptic Bone Necrosis

“…Cystic rarefaction, osteosclerotic changes and destruction of the femoral head have been reported in cases of systemic disease, e.g. sickle-cell anemia (22,24) and Gaucher's disease (8,25). The changes have been unilateral and bilateral.…”

Rare Features in a Case of Gaucher's Disease

“…The most common sites of these lesions are the hip-joint (e.g., Brill and others, 1905;Todd and Keidan, 1952;Vaughan-Jackson, 1952;Wood, 1952), the mandibular joint (Cecil, 1948;Snapper, 1943), and the knees (Draznin and Singer, 1948;James, 1952;Kroboth and Johnson, 1952;Markovits, 1949;Pick, 1927), including the lower part of the shaft of the femur with its typical flask-like deformity (Davies, 1952;Windholz and Foster, 1948). Affections of the shoulder bones seem to be less frequent; Pick (1927), reviewing the literature as a pathologist and adding four cases of his own, does not mention involvement of the upper extremity except in the case described by Brill and others (1905).…”

“…Apart from these, we have found only four cases with lesions at this site in the literature available to us (James, 1952;Kroboth and Johnson, 1952;Morgans, 1947;Snapper, 1943). Pre-existing osseous affections are prone to deterioration after splenectomy according to Snapper (1943), but the development of new osseous lesions after this operation has been described only rarely (Meakins, 1950;Todd and Keidan, 1952;Vaughan-Jackson, 1952), and then usually a few months to 2 years after removal of the spleen (Windholz and Foster, 1948;Wood, 1952).…”