Gorham disease (GD) is a very rare condition associated with spontaneous destruction and resorption of 1 or more bones anywhere in the body, Different regions of the skeleton may be infected, such as the pelvis, clavicle, spine, ribs and facial skeleton. The skull and mandible are the more frequently reported.
Matherial and methods : We review the experience of the Pediatric Department of Hospital la Paz in the treatment of Gorham disease in pediatric patients involving the craniofacial skeleton. 18 patients have been treated in these Department in collaboration with the Plastic Surgery Pediatric Service, affecting mainly skull (12), mandible(4) and ethmoid region (2). The therapeutic approach is specially important in the management of these patiens.
Results : The treatment in the active resorptive phase must be managed with medical therapy, using Intravenous bisphosphonate (Zometa) (IV) infusions were repeated once a month for 12 months. Calcium and vitamin D were administered once a day during the treatment. Surgical management of the disease must be delayed until no activity of the disease is present clinically and radiologically. In the paediatric patient, depending on the extent of the defect, the same as age, calvarian bone defects could be managed conservatively, and mandibular bone defects could be approach by mean of reconstruction plates associated to bone grafts in the less severe cases. Microvascular free flaps could be an option in severe cases or when growth development have been completed. Rreconstruction could be attempted after a sufficient period of time with careful clinical and radiologic observation.
Conclusions : GD is a clinical entity that should be suspected by the clinician, despite the difficulty in the diagnosis of this disease due to the few cases reported, histological difficulties and variety in the clinical and radiographic findings. Proper therapeutical management is mandatory to avoid complications and sequela.