선천성 폐 기도 기형의 임상적 양상에 대한 고찰: 단일기관 연구
Clinical characteristics of congenital pulmonary airway malformation of the lungs: A single-center study
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Purpose: Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lungs. We investigated clinical characteristics of CPAM based on patient age at diagnosis. Methods: In this retrospective study, we analyzed the medical records of 51 patients diagnosed with CPAM at Pusan National University Hospital between January 2000 and December 2019. Results: We investigated 39 children and 12 adults. The mean age at the diagnosis of the patients was 15.9±14.3 years. The mean ages of children and adults at the time of diagnosis of CPAM were 6.8±5.8 years and 31.2±10.2 years, respectively. Among the 51 patients investigated, 20 (39%) were asymptomatic and 31 (61%) showed clinical symptoms, such as dry cough, recurrent respiratory infections, and dyspnea at the time of diagnosis. Notably, clinical symptoms at diagnosis were observed in 28 children (72%) and in only 3 adults (25%) (P=0.006), and children were more symptomatic than adults. Children with large cysts tended to be more symptomatic than those with small cysts (P<0.001). Combined anomalies were detected in 12 patients (23%). Patients with cystic lesions in the right lower lobe of the lung showed a higher prevalence of combined anomalies (P=0.015). Surgical resection was performed in 40 patients (78%), and all patients showed good prognosis. Conclusion: This study revealed that the presence of clinical symptoms of CPAM differed between children and adults, depending on the age at diagnosis and that patients with cysts in the right lower lobe of the lungs tended to show a higher prevalence of combined anomalies.
Abstract
Purpose: Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lungs. We investigated clinical characteristics of CPAM based on patient age at diagnosis. Methods: In this retrospective study, we analyzed the medical records of 51 patients diagnosed with CPAM at Pusan National University Hospital between January 2000 and December 2019. Results: We investigated 39 children and 12 adults. The mean age at the diagnosis of the patients was 15.9±14.3 years. The mean ages of children and adults at the time of diagnosis of CPAM were 6.8±5.8 years and 31.2±10.2 years, respectively. Among the 51 patients investigated, 20 (39%) were asymptomatic and 31 (61%) showed clinical symptoms, such as dry cough, recurrent respiratory infections, and dyspnea at the time of diagnosis. Notably, clinical symptoms at diagnosis were observed in 28 children (72%) and in only 3 adults (25%) (P=0.006), and children were more symptomatic than adults. Children with large cysts tended to be more symptomatic than those with small cysts (P<0.001). Combined anomalies were detected in 12 patients (23%). Patients with cystic lesions in the right lower lobe of the lung showed a higher prevalence of combined anomalies (P=0.015). Surgical resection was performed in 40 patients (78%), and all patients showed good prognosis. Conclusion: This study revealed that the presence of clinical symptoms of CPAM differed between children and adults, depending on the age at diagnosis and that patients with cysts in the right lower lobe of the lungs tended to show a higher prevalence of combined anomalies.
참고문헌 (27)
- Baird R, "Congenital lung malformations : informing best practice", Semin Pediatr Surg, 23, pp.270~277 (2014)
- Patil A, "Congenital cystic adenomatoid malformation of lung in fetus : report of two cases with brief review of literature", J Assoc Chest Physicians, 3, pp.53~ (2015)
- Chikkannaiah P, "Congenital cystic adenomatoid malformation of lung : report of two cases with review of literature", Lung India, 30, pp.215~218 (2013)
- Giubergia V, "Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases", J Pediatr(Rio J), 88, pp.143~148 (2012)
- Durell J, "Congenital cystic lesions of the lung", Early Hum Dev, 90, pp.935~939 (2014)
- Xia B, "Surgical treatment of congenital cystic adenomatoid malformation : a retrospective study of single tertiary center experience", J Matern Fetal Neonatal Med, 30, pp.416~419 (2017)
- Hamanaka R, "Congenital cystic adenomatoid malformation in adults : report of a case presenting with a recurrent pneumothorax and a literature review of 60 cases", Respir Med Case Rep, 26, pp.328~332 (2019)
- Hyun Jung Kwak, "Congenital Cystic Adenomatoid Malformation with Bronchial Atresia in Elderly Patients", Tuberculosis and Respiratory Diseases, 72(6), pp.501~506 (2012), 대한결핵및호흡기학회[DOI]
- Kapralik J, "Surgical versus conservative management of congenital pulmonary airway malformation in children : a systematic review and meta-analysis", J Pediatr Surg, 51, pp.508~512 (2016)
- 정유영, "Life-Threatening Congenital Cystic Adenomatoid Malformation in the Premature Neonate", Journal of Chest Surgery, 49(3), pp.210~213 (2016), 대한심장혈관흉부외과학회
- Di Prima FA, "Antenatally diagnosed congenital cystic adenomatoid malformations(CCAM) : research review", J Prenat Med, 6, pp.22~30 (2012)
- 서영은, "조기수술이 필요한 폐 선천성 낭포성 샘 모양 기형아의 특성과 수술 전 중재적 시술", Neonatal medicine, 20(1), pp.81~89 (2013), 대한신생아학회
- Nagata K, "Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung", Pediatr Surg Int, 25, pp.753~757 (2009)
- Joo Hee Lim, "Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience", Neonatal medicine, 26(3), pp.138~146 (2019), 대한신생아학회[DOI]
- So Hyun Nam, "Minimally invasive surgery for congenital cystic adenomatoid malformations - early experience", Annals of Surgical Treatment and Research, 90(2), pp.101~105 (2016), 대한외과학회
- 전병우, "The Clinical Courses of Patients with Congenital Cystic Adenomatoid Malformation Complicated by Pneumonia", Yonsei Medical Journal, 56(4), pp.968~975 (2015), 연세대학교의과대학[DOI]
- "Congenital pulmonary airway (cystic adenomatoid)malformation" (2019)[URL]
- Stocker JT, "Congenital pulmonary airway malformation – a new name for and an expanded classification of congenital cystic adenomatoid malormation of the lung", Histopathology, 41(Suppl2), pp.424~430 (2002)
- Black AJ, "Sex differences in surgically correctable congenital anomalies : a systematic review", J Pediatr Surg, 55, pp.811~820 (2020)
- Parikh DH, "Congenital lung lesions : postnatal management and outcome", Semin Pediatr Surg, 24, pp.160~167 (2015)
- Ruchonnet-Metrailler I, "Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations", Pediatrics, 133, pp.e1285~e1291 (2014)
- Turan O, "Prenatally detected congenital cystic adenomatoid malformation and postnatally diagnosed trisomy 13 : case report and review of the literature", Turk J Pediatr, 53, pp.337~341 (2011)
- Stocker JT, "Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum", Hum Pathol, 8, pp.155~171 (1977)
- Morini F, "Current management of congenital pulmonary airway malformations : a"European Pediatric Surgeons’ Association"Survey", Eur J Pediatr Surg, 28, pp.1~5 (2018)
- Butterworth SA, "Postnatal spontaneous resolution of congenital cystic adenomatoid malformations", J Pediatr Surg, 40, pp.832~834 (2005)
- Stanton M, "The argument for a non-operative approach to asymptomatic lung lesions", Semin Pediatr Surg, 24, pp.183~186 (2015)
- Singh R, "The argument for operative approach to asymptomatic lung lesions", Semin Pediatr Surg, 24, pp.187~195 (2015)