Multicentric disappearing bone disease, or Gorham disease, is a rare entity. A middle age woman, presented to us with left sided antalgic gait and severe bony deformity of her left knee. Radiograph revealed massive bone defect of the medial condyle of the left tibia with subluxation of the knee joint. She was scheduled for knee replacement in six months. However, she developed another lesion over the right hip that typically mimicked the disease progression of disappearing bone disease. The right femoral head vanished progressively within three months without significant history of infection or trauma. Subsequent bone biopsy of the right femoral head and left tibia condyle confirmed the diagnosis. Total knee replacement was carried out for her left knee. She remained pain free on her left knee. A year later, after confirming by sequential radiographs that the osteolysis had stopped, total right hip replacement was performed. Five years later, she remained pain free and both the arthroplasties were stable.
Osteolysis, Essential

Osteolytic disease of the bones have a myriad range of aetiology. One rare cause is Gorham’s disease or disappearing bone disease. This disease is a diagnosis by exclusion using correlation made with clinical presentation, radiological findings and histopathological confirmation. Although many different therapies have been advocated, none have been successful in fully controlling this disease. We present a case that was detected in a Chinese lady using diagnostic imaging, confirmed with computed tomography guided biopsy and successfully treated with joint reconstruction using endoprosthesis.
Osteolysis, Essential

Gorham disease is a rare disorder characterized by proliferation of vascular channels resulting in destruction and resorption of osseous matrix. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. Antiresorptive medication, such as bisphosphonate, is used in Gorham disease because they suppress the course of osteolysis and angiogenisis. We report a 9-year-old boy with Gorham disease, who was presented with recurrent hemothorax and treated by pamidronate. After treatment, he showed no recurrence of hemothorax for more than 2 years.
Child ; Diphosphonates ; Hemothorax ; Humans ; Osteolysis ; Osteolysis, Essential ; Recurrence

Free Tissue Flaps ; Humans ; Mandible ; Osteogenesis ; Osteolysis ; Osteolysis, Essential

Gorham-Stout disease is a rare bone disorder that is characterized by angiomatous proliferation and results in destruction and resorption of the osseous matrix. It has variable presentations and a number of different sites may be affected, including the dermis, soft tissue, and viscera. The involved cutaneous and soft tissue lesions typically present with sponge-like, soft, brown or purple lesions. The clinical course is generally prolonged, with eventual stabilization of the affected bone; however, it is rarely fatal. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. We report a case of 24-year-old man with Gorham disease who presented with progressive cutaneous lesions and severe lymphedema adjacent to the diseased bone.
Dermis ; Humans ; Lymphedema ; Osteolysis, Essential* ; Viscera ; Young Adult

Bone Cements ; Femur ; pathology ; Humans ; Osteolysis, Essential ; therapy ; Syndrome

Gorham-Stout disease is a rare osteolytic disorder with an unclear pathophysiology. It presents as lesions characterized by the loss of the bony matrix and the proliferation of malformed vasculature. At present, there are no gold-standard diagnostic evaluation protocols and it is diagnosed through a mixture of clinical, histopathologic, and radiographic findings. We report a case of a 19-year-old female with Gorham-Stout disease presenting with an 8-year progressive soft tissue mass in the mandible. Extensive osteolysis of the mandible with clustering of the mandibular dentition is noted on computed tomography (CT) imaging. Her case was discussed in a multidisciplinary conference and her treatment was radiotherapy followed by surgery ± reconstruction. We used a CT-based three-dimensional planning technique to give 40 Gy over 20 treatment sessions to the involved areas. Post treatment, a repeat CT was done at six weeks to reassess for disease progression or stabilization, followed by surgical excision. As of 31 October 2021, no evidence of recurrence is noted 48 months after treatment. Arriving at a definitive diagnosis with GorhamStout disease is challenging and a multidisciplinary team approach can help determine the treatment choice with best outcomes.
Gorham-Stout disease ; disappearing bone disease ; osteolysis, essential

Gorham's syndrome (Gorham's disease, Massive osteolysis, Disappearing bone disease, phantom bone) is a rare disorder characterized by histologically benign proliferation of thin-walled vascular channels originating in bone associated with extensive lysis of regional bone matrix. It starts in bone, but it may secondarily involve soft tissues and adjacent bones. Although the osteolysis usually arrests spontaneously, its biologic behavior is unpredictable. The etiolgy is still in dispute and the treatment presents many problems. The author's case was a 20-year old girl, who had pathologic fracture in neck and supracondylar area of left femur with osteolysis of left pelvis, femur, and proximal fibula and tibia. She also had lymphangioma involving bones and adjacent soft tissues. To our knowledge, there is no report on Gorham's syndrome in Korea. The authors report a case of Gorham's syndrome with review of literatures.
Bone Matrix ; Dissent and Disputes ; Female ; Femur ; Fibula ; Fractures, Spontaneous ; Humans ; Korea ; Lymphangioma ; Neck ; Osteolysis ; Osteolysis, Essential ; Pelvis ; Tibia

Gorham's disease is a rare vanishing bone disease characterized by massive osteolysis and replacement with numerous wide engorged capillaries. The exact nature and effective treatment modalities for this condition are as yet unclear. A fifty-years old female, who had unexplained destruction of the left shoulder joint, was diagnosed as having Gorham's disease according to histopathological (capillary tissue aggregation) and immunopathological (immunoreactions with IL-1alpha and IL-6) studies. Although we treated her with antiresorptive medication and radiotherapy, which are current treatment modalities, the destructive process progressed. We report upon this case and provide a review of its literature.
Bone Diseases ; Capillaries ; Female ; Humans ; Osteolysis, Essential ; Radiotherapy ; Shoulder Joint* ; Shoulder*

Essential osteolysis is very rare, slowly progressive disease. Its etiology and pathophysiology is still unknown. Only one case has been reported in Korea. We experienced one case of essential osteolysis associated with renal insufficiency involving bilateral carpal, metacarpal, tarsal, metatarsal bones, elbow, ankle. We report a case of 24-year-old female who had manifestations of essential osteolysis with renal insufficiency with brief review of literatures.
Ankle ; Elbow ; Female ; Humans ; Korea ; Metatarsal Bones ; Osteolysis, Essential* ; Renal Insufficiency ; Young Adult