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At least 145 records · Page 8Linked to original sources
PubMed2006

Gorham's disease of skull base and cervical spine--confusing picture in a two year old.

The unusual presentation of Gorham's disease of skull base and cervical spine in a two-year-old female child with radiological signs mimicking those of raised intracranial pressure is discussed. The differential diagnosis consists of skull base tumours, meningitis, osteomyelitis of the base of skull, congenital hydrocephalaus and congenital syndromes involving the skull base. Pathologically it can be very difficult to differentiate it from lymphangioma of the bone. Difficulty in establishing the diagnosis is discussed along with failure of radiotherapy and palmidronate therapy to cause arrest of the disease process and failure of surgery to provide stabilisation. We describe the course of the disease in this child over the period of last eight years. To the best of our knowledge this is the youngest case of Gorham's described so far.

Bone Density Conservation Agents↗
PubMedDate not supplied

Massive osteolysis of skull bones secondary to lymphangioma of paranasal sinuses and mastoid sinus: report of two cases in children.

Massive osteolysis (MO) is a rare condition in which progressive localized bone tissue resorption is associated with proliferating thin-walled vessels in the absence of inflammation. Rare cases have been reported to occur in the skull. This paper describes two patients with MO who presented with massive assymetric swelling of the skull. This was associated with extensive enlargement of the paranasal sinuses (frontal, ethmoidal, and sphenoidal in one and the mastoid air cells in the other). The second patient developed subcutaneous emphysema on several occasions and the Valsalva maneuver increased the swelling, indicating transmission of the air from the nasopharynx to the mastoid cells and from there to the subcutaneous tissue. In the first patient, the sinus mucosa was shown to be involved by an extensive lymphangioma, and a similar change was seen in the mastoid air cells (patient 2). We are proposing that MO of these two patients resulted from bone resorption due to progressive extension of sinus mucous lymphangiomata.

Adolescent↗
PubMed1987

Gorham's syndrome: a case report and review of the literature.

Gorham's syndrome is a rare disorder involving a proliferation of vascular channels associated with extensive loss of bony matrix. A case report is presented with a review of the 97 previously reported cases. The age of patients at presentation has ranged from less than one to 75 years (mean: 27 years). Sixty-four percent have been men. Fifty-seven percent have had a history of prior trauma. Laboratory values for systemic measures have usually been normal. The disease usually arrests spontaneously, but this is unpredictable. Sixteen patients (16 percent) have died of the disorder, with 10 deaths due to chest wall involvement, three to spinal cord transection, two to sepsis, and one to asphyxia and aspiration. Although the mechanism of bone loss is unknown, osteoclasts were focally increased in the case described herein. Further information and investigation are needed to better understand this unusual disorder.

Adult↗
PubMed1993

Massive osteolysis of the chest in children: an unusual cause of respiratory distress.

Massive osteolysis, also called Gorham's disease, is an uncommon disease in which bone virtually disintegrates and is replaced by vascular fibrous connective tissue. The authors treated two children with massive osteolysis of the chest. Both conditions responded well to radiation therapy; one even showed evidence of reossification. Massive osteolysis should be considered in children who have spontaneous hemothorax. The bony structures should be examined for evidence of lytic lesions.

Adolescent↗
PubMed1978

Total osteolysis of the mandibular condyle in progressive systemic sclerosis.

This report calls attention to the complete resorption of the mandibular condyle in progressive systemic sclerosis (scleroderma), a previously unreported finding. This was associated with osteolysis of the ipsilateral coronoid process, both mandibular angles, and autoamputation of the fingertips. The Panorex provides a simple, effective method for studying the mandible in systemic sclerosis. Similar mandibular osteolysis with vinyl chloride exposure is noted.

Bone Diseases↗
PubMed1983

Massive osteolysis of the maxillofacial skeleton: a clinical, radiographic, histologic, and ultrastructural study.

Since its initial description, there have been approximately sixty cases of massive osteolysis reported, involving virtually every bone in the body. This case represents only the fifteenth report of this disease affecting maxillofacial bones. Clinically and radiographically, the progressive nature of this disease was observed over a 5-year period. Attempts at treatment were without success. From the light and electron-microscopic studies, it can be concluded that this disease process is nonneoplastic in nature, showing features of an exuberant proliferative response of vascular and supportive elements.

Aged↗
PubMed1990

Massive osteolysis of the maxillofacial bones. Report of two cases.

Two cases of massive osteolysis were encountered, one affecting the mandible and the other the maxilla and mandible. Only 13 cases have been reported so far in the existing literature for massive osteolysis of the mandible. It is indeed a rare disease. Our two cases were surgically treated and were clinically and histopathologically assessed.

Aged↗
PubMed1990

Dental abnormalities associated with familial expansile osteolysis: a clinical and radiographic study.

Familial expansile osteolysis is a rare and possibly unique form of hereditary bone dysplasia with dental manifestations that has affected 42 members of a Northern Ireland family for five generations. Twenty-seven family members, aged 3 to 60 years, were examined clinically and radiographically, and it was found that cervical and/or apical root resorption is an early manifestation of the dysplasia. Of the 20 family members with skeletal manifestations of familial expansile osteolysis, 16 showed root resorption. Root resorption may therefore be a good prognosticator for later bone involvement.

Adolescent↗
PubMed1992

Winchester syndrome. A case report and literature review.

The mucopolysaccharidoses are a group of inherited lysosomal storage diseases that are caused by a deficiency of specific enzymes. The acid mucopolysaccharides are stored in tissue and excreted in large quantities in the urine. The storage of this material leads to effects on a wide variety of tissues and to remarkable changes in morphologic features. Winchester syndrome is a rare disorder in the group of mucopolysaccharidoses. This article is a report of a case with classic clinical, radiologic, and biochemical characteristics of the Winchester syndrome.

Abnormalities, Multiple↗
PubMed1991

Papillon-Lefèvre syndrome with acroosteolysis.

The Papillon-Lefèvre syndrome is characterized by palmoplantar hyperkeratosis and juvenile periodontitis that results in the precocious loss of both the deciduous and permanent teeth. We report a patient with Papillon-Lefèvre syndrome associated with acroosteolysis, an association that has not been previously described.

Adult↗
PubMed1993

Carpal tunnel syndrome presenting with ischemic skin lesions, acroosteolysis, and nail changes.

A 58-year-old woman had a chronic necrotic ulceration of the fingertip of the third finger and nail plate abnormalities of the first, second, and third fingers of the right hand. Roentgenograms of the hands showed acroosteolysis of the distal phalanges of the second and third right fingers. Skin and nail lesions showed considerable improvement after surgical treatment of carpal tunnel syndrome. The authors review the clinical and radiologic features of similar cases that have previously been reported.

Carpal Tunnel Syndrome↗
PubMedDate not supplied

Progressive vertebral collapse in diffuse angiomatosis.

A case of vanishing bone disease is presented, with radiologic, endoscopic, and peritoneoscopic evidence of angiomatous involvement of the bones, surrounding soft tissues, and gastrointestinal tract. The diagnosis was made by a combination of radiologic techniques and endoscopy. Areas of absent bone were shown to opacify after intralymphatic injections of contrast material, providing in vivo demonstrations that these areas of vanishing bone are occupied by abnormal lymphatics. The etiology remains obscure, but our review of the literature suggests that this condition may be one of a large spectrum of disorders due to a basic underlying endothelial dysplasia. The lymphatic system, vascular system, or both may be involved, and the condition may manifest itself as an abnormality of the bones, soft tissues, viscera, or a combination of these.

Adult↗
PubMed1995

A patient with carpotarsal osteolysis and nephropathy treated with long-term hemodialysis.

We describe a type III carpotarsal osteolysis patient with nephropathy who has been maintained on chronic hemodialysis for 12 years. The diagnosis of osteolysis was based on x-ray findings and bone biopsy. At the age of 12 years, she developed renal disease that progressed; she was placed on chronic dialysis at the age of 18 years. Blood pressure was controlled by a medical regimen, and her anemia has benefitted from erythropoietin up to a level of 28% to 30%. Her osteolysis has stabilized since the age of 20 years; she is presently 30 years old. The major problem affecting the patient's clinical outcome is hyperphosphatemia.

Adolescent↗