Viewpoints on vessels and vanishing bones in Gorham-Stout disease.

Gorham-Stout disease (GSD) is a rare disorder characterized by the proliferation of endothelial-lined vessels in bone and the progressive destruction of bone. Although Jackson described the first case of GSD in 1838, the clinical and histological features of GSD were not defined until Gorham and Stout published their report on massive osteolysis in 1955. In the years since Gorham and Stout's groundbreaking publication, more than 300 cases of GSD have been described in the literature. These reports have revealed that the progressive resorption of bone in GSD causes severe physical deformities, disabilities, and life-threatening complications. Unfortunately, the underlying cause of GSD remains unknown and, as a result, the therapeutic options for individuals with GSD are limited. Here we review the latest advances in GSD research and present strategies to address basic and clinical research questions related to GSD.

Mortalidad en pacientes con osteólisis de origen linfático. Revisión de 54 casos / Mortality in patients with osteolysis of lymphatic origin: a review of the experience with 54 patients and the literature

Introducción: La osteólisis de origen linfático es una enfermedad poco frecuente cuya alta mortalidad es difícil atribuir al propio cuadro o a la iatrogenia que genera su tratamiento. El objetivo del trabajo es revisar las causas de la mortalidad en nuestros pacientes y en la literatura médica en la osteólisis de origen linfático. Pacientes y métodos: Hemos revisado a todos los pacientes con osteólisis de origen linfático tratados en nuestro departamento y que han fallecido en los últimos 15 años, así como todos los casos publicados de defunción con esta enfermedad desde los años cincuenta. Resultados: De los 57 pacientes con osteólisis de origen linfático tratados en nuestro hospital, 3 han fallecido, 2 por infección y uno por distrés respiratorio agudo. Todos presentaban desnutrición y 2 de ellos quilotórax bilateral. Entre las 51 muertes revisadas de la literatura entre los años 1954 y 2010, 19 tenían quilotórax bilateral, 15 derecho, y sólo 2 tenían afectación exclusivamente izquierda. Once tuvieron complicaciones respiratorias en forma de neumonía y distrés, 10 presentaron fracturas patológicas y otros 10 quilopericardio y/o ascitis quilosa. La causa más común de defunción fue el fallo respiratorio en 25 pacientes, seguida de la neumonía y la sepsis. Las publicaciones no especifican la causa última de la insuficiencia respiratoria. Conclusiones: El paciente con osteólisis de origen linfático grave suele presentarse con 3 cuadros que se solapan: la malformación linfática de partes blandas, la reabsorción ósea y el quilotórax. Mientras que la mortalidad de los 2 primeros es excepcional, la del quilotórax es frecuente, sobre todo cuando es bilateral y no responde al tratamiento. El fallo respiratorio y la infección están agravados por la inmunosupresión, la desnutrición y el emplazamiento prolongado de catéteres centrales. A diferencia del fallo respiratorio, difícil de controlar tras el fracaso del tratamiento médico-quirúrgico, la morbimortalidad causada por las infecciones puede disminuirse mediante un adecuado soporte nutricional, una correcta profilaxis antibiótica y la reducción al mínimo indispensable de los accesos venosos y de la nutrición parenteral total (AU)

Introduction: Osteolysis of lymphatic origin is a rare disease with a high mortality which is difficult to attribute whether it is due to the disease itself or the therapeutic morbidity. The aim of this study is to review the causes of mortality in our group of patients with osteolysis of lymphatic origin, compared with the group of patients previously reported in the medical literature. Patients and methods: We reviewed all patients with osteolysis of lymphatic origin and treated in our department who had died in the last 15 years, and we reviewed all published cases of death with this disease since the 1950's.ResultsA total of 57 patients with osteolysis of lymphatic origin had been studied at our institution, of whom three died, two from sepsis, and one from acute respiratory distress syndrome. All of them suffered severe malnutrition from massive lymphatic loss and 2 had bilateral chylothorax. Among the 51 cases reviewed in the literature between 1954 - 2010, 19 had bilateral chylothorax, 15 had right side involvement and only 2 had an exclusive left chylothorax. Eleven patients had complications, such as respiratory distress and pneumonia. Ten of them suffered from pathological fractures, and 10 from chylopericardium and/or chylous ascites. The most common cause of death was respiratory failure in 25 patients, followed by pneumonia and sepsis. The publications do not specify the ultimate cause of respiratory failure. Conclusions: Patients with severe osteolysis of lymphatic origin suffer three main disorders with overlapping comorbidity: the lymphatic malformation of soft tissue, the bone resorption and the chylothorax. While mortality in the first two is exceptional, the mortality of the chylothorax is common, especially when it is bilateral and does not respond to different therapies. Respiratory failure and infection are aggravated by immunosuppression, malnutrition and the non-restrictive use of central catheters. Unlike respiratory failure, which is difficult to control after the failure of medical and surgical treatment, morbidity from infections can be reduced through careful nutritional support, rational antibiotic prophylaxis and a reduction to a minimum in the use of central catheters and total parenteral nutrition (AU)

Implicaciones anestésicas del síndrome de Gorham / Anaesthetic implications of Gorham syndrome

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Gorham Stout syndrome (disappearing bone disease): two additional case reports and a review of the literature.

Gorham-Stout syndrome, or disappearing bone disease, is characterized by the proliferation of thin-walled vascular channels associated with regional osteolysis. There have been fewer than 150 cases reported in the literature. In this clinical report, we describe 2 additional cases of Gorham-Stout syndrome affecting the maxillofacial skeleton. We provide a review of the clinical diagnosis of this syndrome and describe treatment options.

Gorham disease of the spine.

STUDY DESIGN: This is a case report of Gorham disease of the spine with review of the literature. OBJECTIVE: To review the diagnosis, therapy, clinical course, and prognosis of Gorham disease of the spine. SUMMARY OF BACKGROUND DATA: This is the 17th reported case of spinal involvement by this rare type of idiopathic osteolysis. METHODS: The patient was studied with radiographs, nuclear bone scans, computed tomography scans, magnetic resonance scans, and biopsies. His spine was stabilized by posterior and anterior rods, corpectomies, and bone grafts. RESULTS: The patient's spine had remained stable for 22 months after surgery, but intractable chylothoraces and spreading bone destruction were present. CONCLUSIONS: Spinal Gorham disease has high morbidity and mortality, but the course in an individual patient is difficult to predict. Early spinal stabilization should be considered before irreversible neurologic complications occur.

Artritis psoriasica asociada a acroosteolisis y neuropatia perférica

El diagnóstico de artritis psoriásica se hace cuandopsoriasis y artritis de presentan juntas, sin nódulos subcutáneos y usualmente seronegativa para factor reumatoideo (FR) (1). Wright en 1991 (2) simplificó la clasificación en tres grupos. Revisamos la literatura y no encontramos informes de neuropatía periférica ni osteólisi de la tibia, peroné y tarso en artritis psoriática. Informamos el caso de una paciente con las anteriores manifestaciones clínicas

Gorham's massive osteolysis: the role of radiation therapy and a review of the literature.

PURPOSE: This paper reviews the natural history and management of patients with Gorham's disease and presents four cases treated at The Massachusetts General Hospital since 1965. Gorham's disease is characterized by localized endothelial proliferation which results in destruction and resorption of bone. The etiology is undefined. There is no evidence of a malignant, neuropathic, or infectious component. This disease is progressive in most patients, but in occasional instances the process has been noted to be self-limited. The principal treatment modalities are surgery and radiation therapy. METHODS AND MATERIAL: Since 1965, four patients with Gorham's Disease have been treated at the Massachusetts General Hospital. Three received definitive radiation therapy in doses ranging from 31.5 to 45 Gy. The fourth patient underwent surgery primarily. RESULTS: Three patients are currently alive and fully functional with no evidence of disease at last follow-up. The fourth patient died of progressive disease despite treatment with both radiation therapy and surgery. CONCLUSION: The prognosis for patients with Gorham's disease is generally good unless vital structures are involved. Due to the rarity of this entity, there is no standard therapy. Definitive radiation therapy in moderate doses (40-45 Gy in 2 Gy fractions) appears to result in a good outcome and few long-term complications.

Pregnancy complicated by Gorham disease.

BACKGROUND: A pregnancy with Gorham disease (massive osteolysis syndrome) is described. To our knowledge, this is the first reported case of a pregnancy associated with this disease. CASE: A 25-year-old woman was seen for pre-conception counseling with the diagnosis of Gorham disease. Before pregnancy, magnetic resonance imaging confirmed a stable disease process. The woman and fetus were followed during pregnancy with ultrasound and consultations. Severe preeclampsia developed at delivery, with a significant thrombocytopenia. We delivered the infant by low forceps, with a good outcome. Subsequently, the mother and child were doing well at 18 months postpartum. CONCLUSION: We briefly review this rare bone disease as it relates to pregnancy and provide information for preconception counseling.