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A Rare Case of Gorham's Disease: Primary Ulnar Involvement with Secondary Spread to the Radius and Elbow.

Tavakoli Darestani R, Sharifzadeh A, Bagherian Lemraski M, Farhang Zanganeh R - Trauma Mon (2013)

Bottom Line: The etiology of the disease is unknown.As in this rare case the ulna may be affected first and then the disease may spread to adjacent bones.More studies are needed to better recognize the behavior of this rare disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopedics, Imam Hossein Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, IR Iran.

ABSTRACT

Introduction: Gorham's disease is a rare musculoskeletal disease which causes progressive osteolysis and is characterized by massive bone destruction due to proliferation of vascular elements along with a great number of osteoclasts. The etiology of the disease is unknown. Gorham's disease is essentially rare in the forearm bones. As far as we know, only 2 cases of Gorham's disease of the forearm have been reported with 1 of them in the radius and the other starting in the radius and spreading to the lower portion of the humerus.

Case presentation: This case report shows that Gorham's disease may affect the ulna primarily and spread to adjacent bones despite the fact that there are no such reports in the literature.

Conclusions: Gorham's disease has several manifestations as primary bone involvement. As in this rare case the ulna may be affected first and then the disease may spread to adjacent bones. More studies are needed to better recognize the behavior of this rare disease.

No MeSH data available.


Related in: MedlinePlus

These Figures Show Resorption of the Entire Ulna Also Fracture and Resorption of Distal Part of the Radius. A) Anterior posterior view of forearm in pronation; B) Lateral view of forearm in pronation
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fig3571: These Figures Show Resorption of the Entire Ulna Also Fracture and Resorption of Distal Part of the Radius. A) Anterior posterior view of forearm in pronation; B) Lateral view of forearm in pronation

Mentions: A 60 y/o female presented with Gorham’s disease in the ulna. The disease first started in the ulna and later involved the lower end of the radius and the lower humerus. The patient had referred to her doctor 9 years earlier with pain in the ulnar side of her forearm after getting hit by a heavy object. Clinical and radiographic examinations had failed to reveal any particular pathology and the patient was then referred to a physiotherapist. One year later, the patient returned to her physician with severe pain in the forearm. Plain X-rays were apparently normal, however MR imaging revealed nonspecific lesions in the ulnar bone marrow. These lesions were suspected to be chronic osteomyelitis. Radionuclide and CT scans revealed low density areas in the ulna. Laboratory, microbiological, immunological, hormonal and biochemical tests, including parathyroid tests were all within normal limits. The patient had a biopsy taken by her orthopedic surgeon. The biopsy was apparently consistent with the diagnosis of osteomyelitis, but the culture returned negative. After a while, the patient sustained a fracture in the same bone while asleep which was treated with a cast and healed without surgery. The patient has been complaining of pain and weakness in the left forearm for the past 5 years. Radiographs revealed bone absorption in parts of the ulna (Figure 1). Five months ago, while attempting to lift a glass, the patient twisted her forearm and broke her radius. Her radiographs showed complete resorption of the ulna (Figure 2); her arm was subsequently immobilized in a long arm cast. She came to our center and radiographs of her arm revealed non-union of her latest fracture. They also showed bony absorption of the lower end of the radius (Figures 3 A,B and C). The patient's elbow ROM was nearly normal, so was the wrist ROM except for the final degression of flexion and extension. MTPs and finger motion were normal and the patient's fingers were spared.The neurovascular status had been normal from the onset of the disease and the nerves and vessels were spared. The skin was normal and no soft tissue involvement was seen. All laboratory tests including serum complement levels, vasculitis tests, parathyroid, 24-hour urinary proteins and electrophoresis of plasma proteins were normal. Based on test results and the histopathology report of dead, edematous bony tissue and an abundance of inflammatory cells with a great number of thick-walled blood vessels and no evidence of malignancy, we considered the criteria proposed by Heffez et al. in 1983 ( 14 ) and Gorham’s disease was diagnosed.


A Rare Case of Gorham's Disease: Primary Ulnar Involvement with Secondary Spread to the Radius and Elbow.

Tavakoli Darestani R, Sharifzadeh A, Bagherian Lemraski M, Farhang Zanganeh R - Trauma Mon (2013)

These Figures Show Resorption of the Entire Ulna Also Fracture and Resorption of Distal Part of the Radius. A) Anterior posterior view of forearm in pronation; B) Lateral view of forearm in pronation
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3860655&req=5

fig3571: These Figures Show Resorption of the Entire Ulna Also Fracture and Resorption of Distal Part of the Radius. A) Anterior posterior view of forearm in pronation; B) Lateral view of forearm in pronation
Mentions: A 60 y/o female presented with Gorham’s disease in the ulna. The disease first started in the ulna and later involved the lower end of the radius and the lower humerus. The patient had referred to her doctor 9 years earlier with pain in the ulnar side of her forearm after getting hit by a heavy object. Clinical and radiographic examinations had failed to reveal any particular pathology and the patient was then referred to a physiotherapist. One year later, the patient returned to her physician with severe pain in the forearm. Plain X-rays were apparently normal, however MR imaging revealed nonspecific lesions in the ulnar bone marrow. These lesions were suspected to be chronic osteomyelitis. Radionuclide and CT scans revealed low density areas in the ulna. Laboratory, microbiological, immunological, hormonal and biochemical tests, including parathyroid tests were all within normal limits. The patient had a biopsy taken by her orthopedic surgeon. The biopsy was apparently consistent with the diagnosis of osteomyelitis, but the culture returned negative. After a while, the patient sustained a fracture in the same bone while asleep which was treated with a cast and healed without surgery. The patient has been complaining of pain and weakness in the left forearm for the past 5 years. Radiographs revealed bone absorption in parts of the ulna (Figure 1). Five months ago, while attempting to lift a glass, the patient twisted her forearm and broke her radius. Her radiographs showed complete resorption of the ulna (Figure 2); her arm was subsequently immobilized in a long arm cast. She came to our center and radiographs of her arm revealed non-union of her latest fracture. They also showed bony absorption of the lower end of the radius (Figures 3 A,B and C). The patient's elbow ROM was nearly normal, so was the wrist ROM except for the final degression of flexion and extension. MTPs and finger motion were normal and the patient's fingers were spared.The neurovascular status had been normal from the onset of the disease and the nerves and vessels were spared. The skin was normal and no soft tissue involvement was seen. All laboratory tests including serum complement levels, vasculitis tests, parathyroid, 24-hour urinary proteins and electrophoresis of plasma proteins were normal. Based on test results and the histopathology report of dead, edematous bony tissue and an abundance of inflammatory cells with a great number of thick-walled blood vessels and no evidence of malignancy, we considered the criteria proposed by Heffez et al. in 1983 ( 14 ) and Gorham’s disease was diagnosed.

Bottom Line: The etiology of the disease is unknown.As in this rare case the ulna may be affected first and then the disease may spread to adjacent bones.More studies are needed to better recognize the behavior of this rare disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopedics, Imam Hossein Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, IR Iran.

ABSTRACT

Introduction: Gorham's disease is a rare musculoskeletal disease which causes progressive osteolysis and is characterized by massive bone destruction due to proliferation of vascular elements along with a great number of osteoclasts. The etiology of the disease is unknown. Gorham's disease is essentially rare in the forearm bones. As far as we know, only 2 cases of Gorham's disease of the forearm have been reported with 1 of them in the radius and the other starting in the radius and spreading to the lower portion of the humerus.

Case presentation: This case report shows that Gorham's disease may affect the ulna primarily and spread to adjacent bones despite the fact that there are no such reports in the literature.

Conclusions: Gorham's disease has several manifestations as primary bone involvement. As in this rare case the ulna may be affected first and then the disease may spread to adjacent bones. More studies are needed to better recognize the behavior of this rare disease.

No MeSH data available.


Related in: MedlinePlus