Sailer M, Unsinn K, Fink C, Covi B, Gassner I
Universitätsklinik für Kinderheilkunde Innsbruck.
Klin Padiatr. 1995 Sep-Oct;207(5):302-4. doi: 10.1055/s-2008-1046556.
We report a case of Noonan syndrome associated with pulmonary stenosis and major lymphedema of the lower extremities. At the age of 15 yr spontaneous chylothorax with increasing dyspnea occurred> Chest-x-ray demonstrated increased interstitial markings restricted to the right lower lobe representing pulmonary lymphangiectasia. The chylothorax did not respond to repeated thoracocentesis and medium-chain-triglyceride diet. When a chest tube was inserted and total parenteral nutrition was supplied, the chylous effusion decreased within 32 days. The patient is still on diet and asymptomatic effusion remained during 12 months follow up. In conclusion, pulmonary lympgangiectasia should be considered in patients with Noonan syndrome and an abnormal interstitial pulmonary pattern similar to pulmonary congestion (without any hemodynamic abnormalities). In case of pleural effusion, chylothorax should be considered.
我们报告一例与肺动脉狭窄及下肢重度淋巴水肿相关的努南综合征病例。15岁时，患者出现自发性乳糜胸并伴有进行性呼吸困难。胸部X线显示，仅右肺下叶间质纹理增多，提示存在肺淋巴管扩张。反复胸腔穿刺及中链甘油三酯饮食对乳糜胸均无效果。插入胸管并给予全胃肠外营养后，乳糜性胸腔积液在32天内减少。患者仍在接受饮食治疗，在12个月的随访期间未出现有症状的胸腔积液。总之，对于患有努南综合征且肺部间质影像异常类似于肺充血（无任何血流动力学异常）的患者，应考虑存在肺淋巴管扩张。若出现胸腔积液，则应考虑乳糜胸。
