Gorham's disease of the spine.

BACKGROUND: Gorham's disease of the spine is very rare and usually associated with poor prognosis. Till date very few cases have been reported in the literature. It is characterized by osseous invasion by angiomatous vascular mass without skip areas, eventually causing lysis of affected bone. Morbidity and mortality are high in those with spinal and/or visceral involvement. Neurological complications increase the mortality to 33% whereas with chylothorax, mortality increases to more than 50%. OBJECTIVES: To describe the clinical course and the challenges in planning and implementing effective rehabilitation services for management of patients with Gorham's disease of the spine. METHODS: We report two patients with Gorham's disease who had neurological deficits secondary to spinal cord involvement, and their rehabilitation perspectives. RESULTS: Both patients achieved satisfactory ambulation and functional outcomes. CONCLUSION: Multiple revision surgeries may be required to ensure spinal stability. When working with these patients, one must remain vigilant about spinal stability as well as about possible serious pulmonary complications, and be prepared to make appropriate management decisions when necessary.

Enfermedad de Gorham como causa de lesión medular en la infancia: a propósito de un caso / Gorham's disease as a cause of spinal cord injury in childhood: a case report

Se presenta el caso de un niño de 11 años de edad, diagnosticado un año antes de enfermedad de Gorham con afectación ósea múltiple a nivel de tórax y columna dorsal. En el curso de la enfermedad apareció daño medular secundario. Fue intervenido quirúrgicamente realizándose una fusión vertebral para detener la progresión del cuadro clínico y la deformidad de columna. En el postoperatorio inmediato se evidenció cuadro de lesión medular completa motora e incompleta sensitiva precisando tratamiento rehabilitador precoz. En el síndrome de Gorham-Stout la afectación vertebral es infrecuente, siendo la mielopatía una grave complicación. La recuperación de las secuelas neurológicas precisa un programa de rehabilitación adecuado a la gravedad de las mismas. El síndrome de Gorham-Stout es un trastorno esquelético raro de etiología y patogénesis desconocidas. Puede afectar a cualquier hueso del esqueleto, siendo la afectación de la columna vertebral un hallazgo raro. Cuando aparece como complicación una mielopatía, esta se considera una complicación grave (AU)

Gorham-Stout syndrome is a rare skeletal disorder of unknown etiology and pathogenesis. It can affect any bone of the skeleton, involvement of the spine being a rare finding. Myelopathy, when it appears, is considered a serious complication. The case is presented of an 11- year old boy who had been diagnosed 1 year earlier of Gorham disease with multiple bone involvement on the chest and spine level. Secondary bone marrow damage appeared during the course of the disease. He was referred to our hospital where was underwent surgery, performing spinal fusion to halt the spinal deformity progression and the clinical course. In the immediate postoperative period, a complete motor and incomplete sensory spinal cord injury was observed, which required early rehabilitation treatment. In Gorham-Stout syndrome, vertebral involvement is uncommon, myelopathy being a serious complication. Neurologic recovery requires a comprehensive rehabilitation program adapted to its severity (AU)

[Clinico-pathological and orthopedic-surgical aspects of essential osteolysis (Gorham disease)]. / Az osteolysis idiopathica (Gorham-kór) klinikopatológiai és ortopédsebészi vonatkozásai.

Authors present two cases. One of their patient was followed for 30 years. They discuss the pathological characters and diagnostic difficulties of the disease. They have observed that with radical operations of--according to necessity--serial surgical interventions the outcome of patients can be properly controlled against recent pessimistic opinions.

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.