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CCAM’s come and CCAM’s go?

CCAM’s come and CCAM’s go?. Dr Robert Dinwiddie Dr Sam Sonnappa Respiratory Unit Great Ormond Street Hospital London .

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CCAM’s come and CCAM’s go?

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  1. CCAM’s come and CCAM’s go? Dr Robert Dinwiddie Dr Sam Sonnappa Respiratory Unit Great Ormond Street Hospital London

  2. CCAMCongenital Cystic Adenomatoid Malformation(Congenital lung cysts)

  3. 5.3.90

  4. JAMES • Born at term on 22/2/1990 • BW 3.56 kg • Tachypnoea from birth • Moderate indrawing – in 30% oxygen • CXR “Congenital lobar emphysema on the right” • No prenatal ultrasound to suggest congenital anomaly

  5. 5.3.90

  6. Ventilation-perfusion V/Q scan Yes? No?

  7. V/Q scan Left lung normal Right lung shows matched ventilation-perfusion defect most likely in middle lobe Consistent with congenital lobar emphysema “The unlikely diagnosis of a CCAM cannot be excluded”

  8. CT scan Yes? No?

  9. CT scan Yes? No? Not done Would have been useful In retrospect this was a mistake

  10. Operation • On 16/3/05 aged 22 days • Very large 7x5 cm cyst in right upper lobe • “Rest of lung tissue seemed to be normal” • Right upper lobectomy perfomed • Histology – consistent with that seen in congenital cystic adenomatoid malformation (CCAM)

  11. 17.3.90

  12. ?Re-operationRisks of not operating • Infection – makes subsequent operation more difficult • Pneumothorax from rupture of cyst – rare but well described • Incidence in Canada 1/25000 • 30 new cases year in UK • Malignancy 1-2% - rhabdomyosarcoma • If no operation - continued surveillance and further radiation over many years • Successful operative removal – end of problem!

  13. Why no re-operation • Recent thoracotomy • “Some CCAMs resolve spontaneously” • Decision – “wait and see” • Re-operate later if not shrinking or if other complications such as recurrent infection

  14. 27.2.91

  15. Reoperate? Yes No

  16. Re-operation • 18/01/94 aged 3yrs 11mnths • Large cystic lesion in right middle lobe - removed • Right lower lobe appeared normal • No cysts seen in right lower lobe on CT • Histology “Type 1 CCAM”

  17. 20.1.94

  18. 5.9.94

  19. Re-re-operate? Yes No

  20. 22.6.97

  21. Lung Function age 8 FVC 72% FEV1 80% PEFR 83%

  22. 9.7.01

  23. 14.7.03

  24. 14.8.03

  25. Conclusions • Always do a CT scan on congenital lung malformations • Distinguishing lobar emphysema from CCAM on CXR can be very difficult • Re-operation x1 is probably appropriate if further cysts appear • Third re-operation may not be necessary if you wait but it may take 10 years to find out!

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