- 期刊
Congenital Pulmonary Airway Malformation in an Adult: A Case Report and Review of the Literature
成人先天性肺氣道畸型－病例報告及文獻回顧
摘要
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare congenital cystic lung disease of the lower respiratory tract. Most cases are found in neonates and infants using pre-natal ultrasound screening or on presentation of respiratory distress. However, a few cases are diagnosed in adults with or without respiratory symptoms. Some of the CPAM cases may be related to recurrent pulmonary infection or malignancies. Complete resection, such as lobectomy, is usually suggested for children and adults who are symptomatic. We report a case of CPAM in an adult female presenting as recurrent hemoptysis. The patient underwent video-assisted thoracic surgery (VATS) with lobectomy. She has been regularly followed up at the clinic, and has maintained a stable condition. In addition to reporting this case, we summarize previously published articles regarding the epidemiology, histopathological classification, clinical presentation, radiology, and treatment of CPPAM in adults.
並列摘要
先天性肺氣道畸型（原名為先天囊狀類腺畸型）為罕見之先天肺部囊泡性疾病，主要發生於下呼吸道。大部份的病例為新生兒或嬰兒，乃經過產前超音波檢查或呼吸窘迫的症狀而被發現。然而少部份病人直至成人時期才被診斷，且不一定有症狀。一部份的病例可能會有反覆性的肺部感染，甚至是衍生出惡性腫瘤。因此，建議完全切除病灶，如肺葉切除手術，尤其針對已經有症狀的小孩或成人。我們在此報告一例成人之先天性肺氣道畸型，以反覆咳血為表現。經胸腔內視鏡輔助肺葉切除手術後，目前追蹤情況穩定。最後我們針對此病之流行病學、組織病理分類、臨床表現、放射線學變化與成人案例的處置等，回顧並整理過去的文獻以提出此報告。