Facial disfigurement due to osteitis fibrosa cystica or brown tumor from secondary hyperparathyroidism in patients on dialysis: A systematic review and an illustrative case report

Abstract

Osteitis fibrosa cystica (OFC) is the most frequent type of osseous change in renal osteodystrophy affecting the majority of dialysis patients. Brown tumors are a severe form of OFC. The involvement of the craniofacial skeleton causing facial disfigurement in patients on dialysis appears to be limited to case reports. After searching PubMed, we performed a systematic review of 127 cases with a severe form of OFC resulting in a facial disfigurement to understand possible determinants for this condition. We found that since the first published case in 1974, and after a peak in 1996, there appears to be an increase in published reported cases. Only 27.6% of these cases were published in nephrology journals. The most common region for reported cases was North America. Mean age of these patients was 31.2 years with a mean dialysis duration of 7 years. Almost 67% were women, and almost all were on hemodialysis. The disease tended to most commonly localize to the maxilla (73.2%) and mandible (57.5%). As part of the treatment, 59% of patients had a parathyroidectomy. More than one-third (35.4%) had symptomatic improvement at follow-up. Mean follow-up was 1.6 years. Clinicians should be aware of this clinical presentation of a severe form of OFC and/or brown tumors. Timely diagnosis and intervention may help to prevent or decrease destructive bone changes and reduce negative psychological consequences of facial disfigurement.

Brown tumor complicating end-stage kidney disease

“…The tumors are soft and brownish in color due to episodes of hemorrhages from microfractures of the thinned and demineralized bone. They present as either single or multiple lesions and are destructive by their lytic and expansive properties [ 5 , 14 , 15 ].…”

Epistaxis as an initial symptom of brown tumor in hard palate: a rare case report and review

“…More than one-third (35.4 %) of brown tumor cases have symptomatic improvement at follow-up. The mean follow-up duration was 1.6 years [30]. The only method for treating primary hyperparathyroidism is the surgical removal of the parathyroid adenoma or adenomas [31].…”

Oral and maxillofacial manifestations of chronic kidney disease–mineral and bone disorder: a multicenter retrospective study

“…16 Furthermore, our study identified a female preponderance, consistent with the literature, in what has been speculated to be a consequence of women's lower body mass, which would lead to an earlier clinical manifestation of the disease. 17,18 BTH may clinically present as either a painful exophytic mass or as an asymptomatic swelling, causing a well-defined radiolucent or hypodense image on radiographic and CT examinations but usually not producing cortical disruptions and periosteal reactions. 19,20 As found in this series, multifocal lesions may also be present.…”