Department of Pulmonology, Hospital Nossa Senhora do Rosário - HNSR - Barreiro, Portugal.
J Bras Pneumol. 2009 Oct;35(10):1053-6. doi: 10.1590/s1806-37132009001000013.
Madelung's disease, or multiple symmetric lipomatosis, is a rare disease, characterized by accumulation of unencapsulated fat, generally located symmetrically around the neck and shoulders. Here, we present the case of a patient with diffuse lipomatosis accompanied by obstructive sleep apnea due to cervical involvement and facial deformity, which made it necessary to use nasal pillows for ventilation. The patient was hospitalized with a diagnosis of pneumonia and required noninvasive ventilation due to severe hypercapnia. A brief review of the literature was made, and we describe and discuss the investigation of this rare clinical case.
马德隆病,或多发性对称性脂肪瘤病,是一种罕见疾病,其特征是无包膜脂肪的积聚,通常对称地位于颈部和肩部周围。在此,我们介绍了一例弥漫性脂肪瘤病患者,由于颈椎受累和面部畸形导致阻塞性睡眠呼吸暂停,因此需要使用鼻枕进行通气。该患者因肺炎住院,由于严重的高碳酸血症需要无创通气。我们对文献进行了简要回顾,并描述和讨论了这一罕见临床病例的检查。