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Weekly Chest CasesArchive of Old Cases

Case No : 52 Date 1998-10-26

  • Courtesy of Jin-Hwan Kim, M.D. / Chungnam National University Hospital
  • Age/Sex 16 / M
  • Chief Complaintmild fever, cough, and sputum for several days
  • Figure 1
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Chest PA

Diagnosis With Brief Discussion

Diagnosis
Congenital Cystic Adenomatoid Malformation, Type I
Radiologic Findings
Masslike lesion was discovered on chest radiograph. On CT scan, a large cystic mass was present in RLL, adjacent to major fissure, which had air-fluid level. Areas of low attenuation were noted around the cyst. RLL lobectomy was done. On gross specimen, a 6.5 x 6 cm sized cystic lesion filled with chocolate-colored fluid was present in superior segment of RLL. The cyst space was not connected with bronchial tree. Microscopically, the contents of cyst were old blood and hemosiderin
laden macrophages and the cyst was lined by psudostratified columnar epithelium. The surrounding parenchyme showed interstitial fibrosis, emphysematous change and lymphocyte infiltration.
Brief Review
Congenital cystic adenomatoid malformation(CCAM) is a developmental abnormality with immaturity and malformation of the small airways and distal lung parenchyma. Depending on the time and type of pulmonary insult, 4 - 26% of cases have associated congenital abnormalities. Three types of CCAM are recognized by Stocker et al. Patient with CCAM frequently present with mixed lesions of histologic type I & II.


Type Gross appearance Microscopic appearance
I (50%) One or more cysts 2 to 10 cm in diameter Broad fibrous septa Mucigenic cells Best prognosis

II (40%) Small uniform cyst < 2 cm in diameter Irregular proliferation of ectatic structures resembling bronchioles Renal and gastrointestinal anomaly
III (10%) Solid mass without gross cyst formation Irregular curving channels and small air spaces lined by plump cuboidal epithelium Poorer prognosis

The majority are found in neonatal period, and up to 90% are reported within the first 2 years of life. Presentation may be delayed until late childhood or adulthood. Patients present with respiratory distress, cyanosis, or pneumonia. Malignancies complicating CCAM are rarely seen including bronchioloalveolar carcinoma and rhabdomyosarcoma. It is unilateral in 98%, involves a single lobe in 95% and the lower lobes in 55%. They may be diagnosed on ultrasound antenatally. CT patterns of CCAM are a large solitay cyst, multiple cyst, areas of consolidation, and areas of low attenuation. Cysts of CCAM are fluid-filled, air-filled, or air-fluid levels in cysts, a combination of these
findings can result. The cysts expand because of collateral ventilation via cores of Kohn. Areas of consolidation shows enhancement and correspond to areas of glandular or bronchiolar structure. Areas of low attenuation around cystic lesions are microcysts with thin-walled bronchiolelike structures blended with normal lunt parenchyma. Because of
lack of cartilage in the involved airways, the malformed bronchi may collapse during expiration, predisposing to infection.
In neonates congenital diaphragmatic hernia and congenital lobar emphysema should be considered. Congenital lobar emphysema reveal the overdistended lobe with stretched and attenuated vascular structures. There are several other pulmonary abnormalities are included in the differential diagnosis. These include pneumothorax, pyopneumothorax,
cystic bronchiectasis, intralobar pulmonary sequestration, intrapulmonary bronchogenic cyst, or prior infection with pneumatocele formation. Intralobar pulmonary sequestration occurs in the posterior basal segment of the left lower lobe, has a large arterial supply from the aorta and prominant obliterative changes of the blood vessels histologically.
However overlapping CT features exist among these cases. The value of CT in the diagnosis of CCAM is to show the full extent of the disease not visible on the chest radiograph, thereby allowing accurate surgical planning.
Surgical excision is the treatment of choice. Prognosis depends on the size of the lesion and its effect on pulmonary development(pulmonary hypoplasia), fetal hydrops, and associated congenital abnormalities.
References
1. Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BKK, Han MC. Congenital cystic adenomatoid malformation of the lung:CT-pathologic correlation. AJR 1997;168:47-53
2. Patz Jr EF, uller NL, Swensen SJ, Dodd LG. Congenital cystic adenomatoid malformation in adults: CT findings. JCAT 1995;19(3):361-364
3. Spence LD, Ahmed A, Keohane C, Watson JB, Neill MO. Acute presentation of cystic adenomatoid malformation of the lung in a 9-year-old child. Pediatr Radiol 1995;25:572-573
4. Aurora P, McHugh K. Pleural pneumatocele mimicking congenital cystic adenomatoid malformation of the lung. A case report. Acta radiol 1998;39(5):520-522
5. Hubbard AM, Crombleholme TM. Anomalies and malformations affecting the fetal/neonatal chest. Semin Roentgenol 1998;33(2):117-125
6. Granata C, Gambini C, Balducci T, Toma P, Michelazzi A, Conte M, Jasonni V. Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation. Pediatr Pulmonol 1998;25(1):62-66
7. Rosado-de-Christenson ML, Stocker JT. Congenital cystic adenomatoid malformation. Radiographic 1991;11(5):865-886
Keywords
Lung, Congenital, Congenital Cystic Adenomatoid Malformation, Type I

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