Short Communication on Sleep Apnea Due to Rare Condition of Gorham’s Disease

Gorham’s disease or Phantom Bone Disease (PBD) is an extremely rare benign condition which is marked by the demolitian of bone matrix and the proliferation of vascular structures causing massive osteolysis of the affected bone [1]. The disorder can appear in any age group, but is usually seen in the second and third decades of life [1]. However, in the present case, the patient was in her 6^th decade of life which is unusual. Gorham's disease has no obvious gender, race or geographic predilection [1,2]. This disease was first reported by Jackson, who found gradual ‘vanishing’ of humerus in a youngmale patient following fracture [3]. Hence, he described the condition as ‘‘A boneless arm’’ in 1838 and also published his opinion on the same condition in 1872, where he postulated this condition to be a complication of traumatic injury. However, its first detailed description was given in 1954 by Gorham and in 1955 by Gorham and Stout [4,5]. A number of theories have been postulated to explain the etiopathogenesis of this disease including hydrolysis by enzymes due to local hypoxia and acidosis; stimulation of osteoclasts due to increased sensitivity of osteoclastic precursors to proliferation of vascular tissue; activation of silent hamartoma by minor trauma; disordered vascular malformations; and proliferation of lymphatic endothelium [3,6]. 

Gorham's disease is an extremely rare and relatively silent disease with potentially fatal complications. Since the clinical symptoms are not characteristic of the disease, radiographic investigations play a vital role in early diagnosis of this condition. CT scan revealed diffuse loss of bony structures involving the whole length of the mandible, giving rise to floating teeth appearance, may cause pathological fracture. The saggital section of CT scan revealed soft tissue impingement in the airway space leading to compromised airway space which might be the reason for sleep apnea. In order to rule out long bones involvement, which are more commonly affected by Gorham's disease, radiographic examination of long bones was done which revealed normal bone structures without any similar pathology. None of the treatment modalities have been proved to be very effective in the long run. Hence it is important for surgeons to be knowledgeable about the existence of this disease as one of the rare causes of massive osteolysis involving the maxillofacial skeleton.

1. Esper A, Alhoulaiby S, Emran A, Youssef S, Alshehabi Z (2021) Gorham Stout disease: A case report from Syria. Oxford Medical Case Reports 1: 8-12.
2. Mukhopadhyay S, Chattopadhyay A, Bhattacharya R, Roy U ((2016) Gorham’s disease of mandible-a rare case presentation in pediatric patient. J Indian Soc Pedod Prev Dent 34: 180-184.
3. Jackson JBS (1838) A boneless arm. Boston Med Surg J 10: 368-369.
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5. Gorham LW, Stout AP (1955) Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone) its relation to hemangiomatosis. J Bone Joint Surg Am 37: 985-1004.
6. Heyden G, Kindblom L-G, Nielsen JM (1977) Disappearing bone disease: A clinical and histological study. J Bone Joint Surg Am 59: 57-61.