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Erdheim-Chester disease with isolated neurological involvement

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http://dx.doi.org/10.1590/0100-3984.2016.0224 Felipe Mussi von Ranke1, Heloisa Maria Pereira Freitas1, Vanessa Dinoá1, Fernanda Miraldi1, Edson Marchiori1 1. Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil. Mailing address:… Click to show full abstract

http://dx.doi.org/10.1590/0100-3984.2016.0224 Felipe Mussi von Ranke1, Heloisa Maria Pereira Freitas1, Vanessa Dinoá1, Fernanda Miraldi1, Edson Marchiori1 1. Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil. Mailing address: Dr. Edson Marchiori. Rua Thomaz Cameron, 438, Valparaiso. Petrópolis, RJ, Brazil, 25685-120. E-mail: [email protected]. bullous disease, pneumatocele, Swyer-James syndrome, endobronchial mass, unilateral pulmonary agenesis, proximal interruption of the pulmonary artery, scimitar syndrome, diaphragmatic hernia, and Poland syndrome. It can also exclude an intrathoracic mass or vascular ring. HRCT is useful for confirming radiographic findings, delineating the affected lobe and showing relative narrowing of the bronchus associated with hyperinflation and attenuated vessels in the hyperlucent lobe, which facilitate the differential diagnosis. Lobectomy is the treatment for nearly all cases of CLE with respiratory distress. According to Karnak et al., lobectomy is the recommended treatment for CLE in all infants under two months of age and in older infants who present with severe respiratory symptoms. Apparently, the earlier the presentation is, the greater is the need for surgery. Conservative management, with close outpatient follow-up, can be used in older children who present with mild to moderate symptoms. Because our patient had remained asymptomatic throughout her life, her case was managed with clinical and radiographic follow-up.

Keywords: erdheim chester; neurological involvement; disease isolated; chester disease; disease; isolated neurological

Journal Title: Radiologia Brasileira
Year Published: 2018

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