Intraosseous CSF Fistula in a Patient with Gorham Disease Resulting in Intracranial Hypotension
Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA, USA.American Journal of Neuroradiology (Impact Factor: 3.59). 06/2011; 32(11):E198-200. DOI: 10.3174/ajnr.A2413
A 7-year-old girl with a history of headaches and Gorham disease was surgically treated in infancy for Chiari I malformation. Subsequent investigation revealed that her cerebellar tonsillar ectopia was due to a long-standing spinal CSF-lymphatic fistula causing intracranial hypotension. Percutaneous fistula closure was performed several times, resulting in transient symptomatic improvement.
Article: Lymphangiomatosis: Clinical Overview[Show abstract] [Hide abstract]
ABSTRACT: "Lymphangiomatosis" is a general term for excessive growth of aberrant lymphatic vessels. The impact of lymphangiomatosis can be devastating due to osteolysis and/or multi-organ involvement. The disorders are heterogeneous, and treatment is dependent upon disease location and symptoms. Most reports are single cases or small case series, predominantly in the orthopedic and radiologic literature. Basic research focused on lymphatic disorders may translate into new therapies for these disorders.Lymphatic Research and Biology 12/2011; 9(4):185-90. DOI:10.1089/lrb.2011.0020 · 1.71 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Objectives: To describe the clinical and radiographic manifestations of spontaneous intracranial hypotension, a rarely diagnosed cause of headache in children. Study design: This study included patients 19 years of age or younger evaluated between January 1, 2001, and June 30, 2012, for spontaneous intracranial hypotension. Results: We evaluated 24 children (18 girls and 6 boys) with spontaneous intracranial hypotension (age at onset of symptoms: 2-19 years, mean 14.3 years). Twenty-three patients presented with orthostatic headaches and 1 presented with a nonpositional headache. A generalized connective tissue disorder was diagnosed in 54% of patients. Magnetic resonance imaging showed the typical changes of spontaneous intracranial hypotension in most patients (79%). Spinal imaging demonstrated a cerebrospinal fluid (CSF) leak with or without an associated meningeal diverticulum in 12 patients (50%) and with dural ectasia or meningeal diverticula in 10 patients (42%), and it was normal in 2 patients (8%). Twenty-three patients initially underwent epidural blood patching, but 8 patients also were treated with percutaneous injections of fibrin glue and 11 patients eventually required surgical correction of the underlying CSF leak. There was no morbidity or mortality associated with any of the treatments, but 5 patients required acetazolamide for rebound high intracranial pressure headache. Overall, outcome was good in 22 patients (92%) and poor in 2 patients (8%). Conclusions: Spontaneous intracranial hypotension in childhood is rare. Most patients can be treated effectively using a combination of epidural blood patching and percutaneous injections of fibrin glue or surgical CSF leak repair in refractory cases.The Journal of pediatrics 02/2013; 163(2). DOI:10.1016/j.jpeds.2013.01.055 · 3.79 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Gorham's disease is a rare disorder characterized by proliferation of vascular channels. The clinical presentation of Gorham's disease is variable and depends on the site of involvement. A high index of clinical suspicion is needed for accurate diagnosis, and recognition of distinctive radiologic and histopathologic features can help facilitate and expedite diagnosis. The subsequent images were obtained from a 31-year-old male patient. Nuclear scintigraphy and computed tomography identified active bone destruction in the lower thoracic and upper lumbar spine in addition to the absence of the left lower rib cage. Gorham's disease was subsequently confirmed by biopsy.Clinical nuclear medicine 03/2013; 38(5). DOI:10.1097/RLU.0b013e318286bf93 · 3.93 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.