Familial fibrous swelling of the jaws; a new case.

Abstract

An unusual multilocular “cyst-like” swelling of the jaws, apparently familial, was first described by Jones in 1933 (1). In 1938 he published a second account of his 3 patients, showing the progress of the condition in the intervening years (2). Seventeen years after the initial description, a further follow-up report appeared (Jones, Gerrie, and Pritchard, 3). In 1951, Caffey and Williams (4) described 5 cases of fibrous swelling of the jaws occurring in two families, and cited a case reported by Miller, for which, however, data were incomplete. We believe the following case to be the tenth recorded example of this unusual condition. It displays all the findings observed in the earlier cases, and the patient's father showed mandibular changes presumably due to a similar process in his youth. Case Report A five-year-old boy was referred for roentgen examination of the jaws, which were moderately enlarged in a generalized fashion (Fig. 1). His appearance otherwise was normal. The maxillae were particularly prominent, stretching the skin of the cheeks and pulling the lower eyelids down, producing the “cherubic” aspect first noted by Jones. The alveolar ridges were widened and the teeth were irregularly spaced and poorly aligned (Fig. 2). There was no pain or tenderness, but enlarged nodes were felt in the submandibular and cervical regions. The mother stated that the child had had normal deciduous dentition until recent months. He had, in fact, been seen by a dentist only ten months before, and no abnormalities had been noted at that time. For the past few months, however, the deciduous teeth had been falling out rapidly, and the extremely irregular placement of those still remaining had become apparent. The swollen appearance of the jaws had not been noticed by the parents until it was brought to their attention. Roentgen studies showed a multilocular cystic appearance of the entire mandible except for the condylar processes (Fig. 3). There was a diffuse increase in density of the maxillae and they were moderately enlarged, encroaching itpon the maxillary sinuses. The teeth were poorly aligned and had rudimentary roots. Dental follicles for the permanent teeth were irregularly placed and abnormal in size. The follicle for the second molar was absent, having been “crowded out” by the cystic areas. A skeletal survey disclosed no other lesions. The blood chemistry was as follows: total protein 7.7 gm.; albumin 4.9 gm.; globulin 2.8 gm.; phosphorus 3.24 mEq/L; calcium 6.1 mEq/L; alkaline phosphatase 7.32 units; acid phosphatase 0.24 units. The hemogram and urinalysis were normal in all respects. The child's father stated that he had had a localized swelling of the left side of his lower jaw in childhood.