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Weekly Chest CasesArchive of Old Cases

Case No : 647 Date 2010-03-22

  • Courtesy of Eun Kyoung Lee, MD, Jeung Sook Kim, MD / Dongguk University Ilsan Hospital
  • Age/Sex 20 / M
  • Chief ComplaintHigh fever, cough, and sputum.
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Congenital cystic adenomatoid malformation (CCAM)
Radiologic Findings
Chest radiography shows multiple variable sized thin walled cysts with air fluid level in left lower lobe. Chest CT scan shows multiloculated thin walled cysts (maximal 35 mm) with air fluid level and surrounding consolidation, mainly confined to LLL posterolateral portion. There is no evidence of abnormal blood supply from a systemic artery.
Brief Review
Congenital pulmonary airway malformations (CPAM) historically referred to as congenital cystic adenomatoid malformations (CCAM) is a rare anomaly of the lung. These lesions form masses of maldeveloped lung tissue and are identified most commonly in stillborn infants or newborns with respiratory distress. In adult, CCAM may be an incidental finding or may be accompanied by symptoms related to recurrent respiratory infections. Less common complications include pneumothorax and occasionally, the development of bronchioloalveolar carcinoma, adenocarcinoma, or squamous cell carcinoma. In older children and adults, CCAMs usually appear radiologically as a unilocular or multiloculated cyst or as a complex soft tissue and cystic mass. The CT manifestation in adults typically consist of a unilocular or multiloculated cyst or a complex soft tissue and cystic mass. CCAM have been classified into 5 subtypes (types 0 to 4) by Stocker. This classification was based on the histopathologic findings in the area of the tracheobronchial tree involved by the malformation. Of these, Types I-III have been well described in the imaging literature. Type I CCAM contains one or more cysts larger than 2 cm in diameter, usually surrounded by smaller cysts. In Type II lesions, the cysts are less than 2 cm in diameter. Type III CCAM is usually shows as a bulky, solid mass. This classification system also has prognostic significance. Type I CCAM is associated with the best overall prognosis and may go undetected clinically until later in life. Type II CCAM has an increased incidence of severe cardiac or renal anomalies. Type III CCAM is often a large mass associated with stillbirth or death in the neonatal period. Because the majority of cases are associated with recurrent infection and risk for development of carcinoma, CCAMs are usually removed surgically. Lobectomy is generally required.
References
1. Masashi et al. Congenital pulmonary airway malformation CT-pathologic correlation. J Thorac Imaging 2007;22:149?53
2. Patz EF Jr, Muller NL, Swensen SJ, Dodd LG. Congenital cystic adenomatoid malformation in adults: CT findings. J Comput Assist Tomogr 1995; 19:361-364.
3. William D. Winters and Eric L. Effmann. Congenital masses of the lung: prenatal and postnatal imaging evaluation. J Thorac Imaging 2001;16:196?06
Keywords
Airway, Congenital,

No. of Applicants : 106

▶ Correct Answer : 66/106,  62.3%
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - Inha University Hospital , Korea (South) Ju Won Lee
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) Sang Min Lee
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - CHRU Lille , France guillaume lefebvre
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - SMG-SNU boramae medical center , Korea (South) Kwang Nam Jin
  • - McGill University , Canada Ben Smith
  • - Konkuk university hospital , Korea (South) Jeong Geun Yi
  • - Yonsei University College of Medicine , Korea (South) Young Joo Suh
  • - Osaka University , Japan Osamu Honda
  • - Samsung Medical Center , Korea (South) Hye Sun Hwang
  • - radiologist, aditya imaging centre , India vivek patel
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - University of British Columbia , Canada Amr Ajlan
  • - freelancer , Korea (South) DAE HYOUN CHO
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Samsung Medical Center , Korea (South) Eun Kim
  • - SMC , Korea (South) Jihoon Cha
  • - Hospital Moinhos de Vento , Brazil Felipe Hertz
  • - KONARK DIAGNOSTIC CENTRE , India AHAMAD MUKARRAB
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Gangnam Severance Hospital , Korea (South) Min Jeong Kim
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) YOOKYUNG KIM
  • - KNUH , Korea (South) MyoungHun Ham
  • - MBAL-POPOVO EOOD , Bulgaria VLADISLAV RUSINOV
  • - Hopital du SacrCoeur , Canada Abraham Bohadana
  • - jiangsu province hospital , China yuan doctor
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - care hospital , India Chary D
  • - Uzunkopru Devlet Hastanesi , Turkey Armagan Sarac
  • - Hospital Universitario 12 de Octubre (Madrid) , Spain Constanza Liebana de Rojas
  • - Soonchunhyang university hospital, Bucheon , Korea (South) Hyelin Kim
  • - Assam Medical college , India samudra borah
  • - SMC , Korea (South) Yi Kyung Kim
  • - , Korea (South) Hun-cheol lim
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - Hospital M de Deus , Brazil Thiago Bento da Silva
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - sunder lal jain hospital , India sanjeev vachher
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - Chonbuk National University Hospital , Korea (South) Jisoo Song
  • - Severance , Korea (South) Kim Hee Yeong
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - BOLLINENI HOSPITAL NELLORE , India jignesh dubal
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - NASA SCANS , India RAKESH BHATIA
  • - IRSA , France jean-luc BIGOT
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - ROK air force aerospacemedical center , Korea (South) Daekeon Lim
  • - SacrCoeur , Canada Andrea Ojanguren
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - CHU Grenoble , France Fabrice Bing
  • - Pushpagiri Medical College , India Mangal Mahajan
  • - All India Institute of medical sciences , India Justin Moses
  • - Saga University , Japan Ryoko Egashira
  • - Hotel-Dieu Montreal , Canada nicolas gautier
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Armed Forces Chun-Cheon Hospital , Korea (South) Chae Lim
  • - Chungnam national university hospital , Korea (South) Jeoung Eun Lee
▶ Correct Answer as Differential Diagnosis : 14/106,  13.2%
  • - Mackay Memorial Hospital , Taiwan Sheng-Yeh Shen
  • - kovai medical centre , India k bhaskar
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OKUBO
  • - NTUH , Taiwan Kuei-pin Chung
  • - IRSA rochefort , France Viviane Pages
  • - Osaka University , Japan Hiromitsu Sumikawa
  • - Song-do Hospital , Korea (South) Ji-young Yun
  • - CHUM , Canada Laurent Letourneau-G.
  • - hospital , Korea (South) sung jo
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Doctors Hospital , Bahamas muneesh sharma
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Trakya University School of Medicine , Turkey Umut Ugur
▶ Semi-Correct Answer : 1/106,  0.9%
  • - Yashoda Superspeciality hospital , India Pravin Mahadevappa
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