Gorham’s Disease of the Maxilla and Mandible With Distinctive Cone Beam Computerized Tomographic Features

Rupam Sinha,Deepsikha Ramani,Soumyabrata Sarkar,Tanya Khaitan

doi:10.30699/ijp.2017.25855

Abstract

Gorham's disease is a rare and atypical disorder epitomized by progressive osteolysis of bone with eventual total disappearance of bone. The etiology is poorly understood with variable clinical presentation. Most times it is initially misdiagnosed as temporomandibular joint dysfunction, periodontal disease or odontogenic tumors clinically and radiographically in routine dental practice. Radiographic examination, such as Cone Beam Computerized Tomography (CBCT) play a vital role in diagnosing such disorder resulting in disappearance of the involved bone entirely, which is a definitive distinguishing feature of this condition. Regarding the rarity of the condition, the current study presents a case of Gorham's disease with distinctive clinical, radiological, and histological, features involving maxilla and mandible.

Highlights

Gorham’s disease is an uncommon disorder of ambiguous etiology, characterized by spontaneous and progressive massive osteolytic event involving one or more bones
The disorder envisages incident of ultimate osteoporosis ensuing complete dissociation in normal coupling mechanism of the involved bone, resulting in rapid destruction of osteoid matrix followed by proliferation of angiogenic components
The disorder came to light in medical literature in 1838 (Jackson), later on in 1924, a similar case occurring in jaws was reported by Romer

Summary

Introduction

Gorham’s disease is an uncommon disorder of ambiguous etiology, characterized by spontaneous and progressive massive osteolytic event involving one or more bones. Gorham’s disease is among few clinical entities, which are described under numerous headings, due to its obfuscating and diversifying etiopathological presentation. These entities include Gorham-Stout Syndrome, Morbus Gorham-Stout Disease, Massive Osteolysis, Idiopathic Massive Osteolysis, Progressive Massive Osteolysis, Vanishing Bone Disease, and Phantom Bone Disease [2]. The present study reports a rare case of Gorham’s disease in a 38-year-old male with distinctive clinical, radiological, and histological characteristics, involving maxilla and mandible. Orthopantomograph showed resorption of the mandibular arch with decreased vertical height of the mandibular body leaving a thin rim and discontinuation in the right lower border of mandible suggestive of pathological fracture (Figure 3). Axial section of computed tomographic image revealed almost completely resorbed lower arch along with hypodense areas in right upper maxilla (Figure 4). The patient was explicated about the rapidly progressive nature of the disease and recieved counselling and support as there is currently no definitive treatment

Discussion

Conclusion