肺淋巴管肌瘤病的HRCT诊断(附17例报告)

Diagnosis of pulmonary lymphangioleiomyomatosis by HRCT:report of 17 cases

目的探讨肺淋巴管肌瘤病的高分辨率CT(HRCT)表现,以提高对该病的认识。方法回顾性分析17例经病理证实的肺淋巴管肌瘤病胸部HRCT表现及临床资料,所有病例肺活检前均行胸部HRCT扫描及腹部B超检查,腹部B超发现异常病例者行腹部CT检查6例。结果 17例患者均为育龄期女性,胸部HRCT均表现为两肺弥漫、大小不等圆形薄壁囊状影(17/17,100%),2例部分融合成肺大泡(2/17,11.8%),4例合并气胸(4/17,23.5%),2例伴乳糜胸(2/17,11.8%)。腹部CT检查发现2例伴双侧肾错构瘤(2/17,11.8%),1例伴右侧肾错构瘤(1/17,5.9%),1例伴子宫错构瘤(1/17,5.9%),2例伴肝脏错构瘤(2/17,11.8%),3例伴腹膜后囊性淋巴管瘤(3/17,17.6%)。结论育龄期女性,胸部HRCT表现为两肺弥漫、均匀分布的圆形薄壁囊状影,应考虑肺淋巴管肌瘤病可能。应常规行腹部CT检查,腹部CT检查发现肝肾错构瘤,腹膜后囊性淋巴管瘤可协助诊断。

Objective To discuss high resolution CT （HRCT ） features of pulmonary lymphangioleiomyomatosis （PLAM ） and to make a futher understanding of this disease .Methods 17 patients with PLAM confirmed by pathological assessment were analyzed retrospectively for chest HRCT findings .They underwent chest high resolution CT （HRCT ） , abdominal type-B ultrasonic .Additional abdominal CT scanning was carried out in 6 cases .Results All the 17 cases were female .Chest high resolution findings included multiple diffuse lung parenchyma cysts in all cases （17/17 ,100% ） ,with some cysts coalesced together to form bullae （2/17 ,11 .8% ） or cause pneumothorax （4/17 ,23 .5% ） .Tow cases had chy-lopleura （2/17 ,11 .8% ） .Common abdominal CT findings included bilateral renal hamartoma in 2 of 17 patients and right renal hamartoma in 1 of 17 patients .One of the 17 patients had uterus hamartoma and tow cases had hepatic hamartoma and three cases had cystic lymphangioma in retroperitoneum .Conclusion If chest high resolution CT findings of child-bearing-age women included sporadic or disseminated multiple diffuse lung parenchyma cysts in bilateral lung , PLAM should be considered .As well as other routine abdominal and pelvic imaging examinations ,common abdominal CT findings showed renal hamartoma or hepatic hamartoma or cystic lymphangioma in retroperitoneum should be an important assistant effect in diagnosis .