Takeda S, Miyoshi S, Inoue M, Omori K, Okumura M, Yoon H E, Minami M, Matsuda H
First Department of Surgery, Osaka University Medical School, Suita City, Japan.
Eur J Cardiothorac Surg. 1999 Jan;15(1):11-7. doi: 10.1016/s1010-7940(98)00262-0.
Congenital cystic lesions of the lung are uncommon but share similar embryologic and clinical characteristics. The purpose of this study is to review our institutional experience of congenital cystic lung disease, emphasizing the clinical spectrum of the disease related to age, and present some cases with unusual clinical manifestations.
Between 1962 and 1996, 26 patients (9 females and 17 males) under 15 years old underwent evaluation and surgical treatment for congenital cystic lung disease. Seven patients were under 1 year old, and 19 were in over 1 year old. There were 13 bronchogenic pulmonary cysts, 6 pulmonary sequestrations, 4 congenital cystic adenomatoid malformations (CCAM), and 3 congenital lobar emphysemas.
All patients under 1 year old showed respiratory distress with mediastinal shift but no episodes of infection. In contrast, 13 of the 19 patients over 1 year old had symptoms of recurrent infection without respiratory distress. Five patients over 1 year old were entirely asymtomatic from birth. There were significant differences (P < 0.05) in the frequencies of respiratory distress and infection between the two groups (chi2-test). Lobectomy was performed in 21 patients, excision in 3 patients, segmentectomy in one patient, and exploration in one patient. There was no incident of postoperative mortality or morbidity except for one patient with CCAM complicated by reexpansion lung edema. Twenty-one patients at long-term follow-up from 2 to 30 years after surgery are doing well with no subsequent limitation of physical activities due to lung resection.
In patients under 1 year old, cystic lesions were discovered by respiratory distress; and in patients over 1 year old signs of infection were the most important clinical features. Early recognition of these relatively rare congenital cystic lung lesions would lead to the immediate, proper surgical intervention.
肺先天性囊性病变并不常见，但具有相似的胚胎学和临床特征。本研究旨在回顾我们机构对先天性囊性肺病的经验，重点关注与年龄相关的疾病临床谱，并展示一些具有不寻常临床表现的病例。
1962年至1996年间，26例15岁以下患者（9例女性，17例男性）因先天性囊性肺病接受评估和手术治疗。7例患者年龄在1岁以下，19例患者年龄超过1岁。其中有13例支气管源性肺囊肿、6例肺隔离症、4例先天性囊性腺瘤样畸形（CCAM）和3例先天性大叶性肺气肿。
所有1岁以下患者均表现为呼吸窘迫伴纵隔移位，但无感染发作。相比之下，19例1岁以上患者中有13例有反复感染症状但无呼吸窘迫。5例1岁以上患者自出生起完全无症状。两组之间呼吸窘迫和感染的发生率存在显著差异（P<0.05）（卡方检验）。21例患者行肺叶切除术，3例患者行切除术，1例患者行肺段切除术，1例患者行探查术。除1例CCAM合并肺复张性肺水肿患者外，无术后死亡或发病事件。21例患者术后2至30年长期随访情况良好，未因肺切除而导致后续体力活动受限。
1岁以下患者中，囊性病变通过呼吸窘迫被发现；1岁以上患者中，感染迹象是最重要的临床特征。早期识别这些相对罕见的先天性囊性肺病变将导致立即进行适当的手术干预。
