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戈勒姆-斯托特病与脑脊液耳漏

Gorham-Stout disease and cerebrospinal fluid otorrhea.

作者信息

Hernández-Marqués Carmen, Serrano González Ana, Cordobés Ortega Francisco, Alvarez-Coca Javier, Sirvent Cerda Sara, Carceller Lechón Fernando, Azorín Cuadrillero Daniel

机构信息

Department of Pediatric Hematology-Oncology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

出版信息

Pediatr Neurosurg. 2011;47(4):299-302. doi: 10.1159/000336877. Epub 2012 Mar 23.

Abstract

OBJECTIVE AND IMPORTANCE

Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician.

CLINICAL PRESENTATION

We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage.

INTERVENTION

Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased.

CONCLUSION

Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.

摘要

目的及重要性

戈勒姆-斯托特病是一种罕见病症,其特征为血管增生导致骨组织局部破坏。由于其发病率低且临床表现多样,诊断需要临床医生具备高度的警觉性。

临床表现

我们报告一例2岁男孩被诊断为戈勒姆-斯托特综合征,累及颞骨并继发脑脊液漏。

干预措施

由于脑脊液漏,该患者需要两次手术干预。第二次干预包括乳房切除术,并在50天内放置补片和腰椎引流装置,之后漏液停止。

结论

戈勒姆-斯托特病是一种罕见疾病,可累及颅底,甚至出现脑脊液漏。

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