Gorham's syndrome: a usually fatal cause of pleural effusion treated successfully with radiotherapy.
نویسندگان
چکیده
A 21 year old man presented with a right sided pleural effusion. Destruction of the 11th and 12th right ribs and adjacent vertebral bodies was noted on computed tomographic scanning. An open rib biopsy revealed the histopathological changes of Gorham's syndrome. In view of the progressive vertebral destruction and inevitable spinal cord compromise, he was treated with high dose radiotherapy. The process was arrested and he remains well with no signs of recurrence after four years. Pleural effusion and vertebral destruction complicating Gorham's syndrome carry a poor prognosis but, in this case, high dose radiotherapy has been effective in controlling both the effusion and the progressive bony destruction.
منابع مشابه
Gorham's Disease With Chest Wall Involvement: A Case Report and a Review of the Literature
INTRODUCTION Gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones. Diagnosis of Gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial. No specific treatment is available. Management options include surgery, radiation therapy and medical therapy. We aimed to present the first case of Gorham's...
متن کاملConservative Management in a Ventilated Preterm Neonate with Pneumopericardium, Pleural Effusion, and Pulmonary Collapse: A Case Report
Background: Neonatal pneumopericardium (PPC) is a rare clinical condition usually associated with other air leak syndromes. It increases morbidity and mortality due to cardiac tamponade. Case report: A preterm male neonate weighing 1260 g was born with the gestational age of 28 weeks. Cardiopulmonary resuscitation was routinely performed without any medical therapy. The newborn was transferred ...
متن کاملChylothorax in Gorham's disease.
A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging show...
متن کاملAn Unusual Cause of Thoracic Mass.
A previously well 10 year old boy presented with scoliosis, a mass in the chest wall, and a pleural effusion. Chest radiography showed the triad of chronic consolidation, pleural effusion, and rib periostitis. Investigations confirmed thoracic actinomycosis. Tissue spread was evaluated by computed tomography. It was successfully treated with benzylpenicillin, which was later replaced by clindam...
متن کاملOctreotide for the Management of Chylothorax in newborns, case report
Chylothorax is the most common cause of pleural effusion in neonates. It is usually idiopathic. Neonatal chylothorax successfully respond to octreotide treatment and can reduce the duration of hospitalization. A number of therapeutic interventions have been used to reduce chyle production and promote resolution of a chylothorax. Initial management typically includes restriction or temporary ces...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Thorax
دوره 51 12 شماره
صفحات -
تاریخ انتشار 1996