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Information for Parents

Congenital Pulmonary Airway Malformation (CPAM) or 

 Congenital Cystic Adenomatoid Malformation(CCAM) & Broncogenic Cyst 

  • What is this disease?

    • Congenital pulmonary airway malformation (CPAM) is a rare congenital birth defect that includes a cystic mass of abnormal lung tissue. This condition used to be referred to as congenital cystic adenomatoid malformation, or CCAM. In CPAM, the cystic mass does not function as normal lung tissue. On the other hand, Bronchogenic cyst are located near the pulmonary hilum or in the mediastinum (near the esophagus). While bronchogenic cysts arise from the airway and often remain intimately attached, they typically have lost their communication with the airway during development.  They appear as solid masses or rarely they are air filled (fluid level) when they communicate with the airways.

  • How it is diagnosed?

    • It is diagnosed by Chest X-ray in newborn. Sometimes, CECT is also needed. Both of these entities can be lined with ciliated mucosa. The bronchogenic cyst lacks connection with alveolar tissue, a feature that aids in its distinction from CPAM. On occasion, with inflamed cysts within the lung, the nature of the specific lesion or underlying disorder may be impossible to ascertain

  • How it is treated?

    • Treatment is surgical, in which the diseases part of lung is removed

  • When it should be operated?

    • The decision to operate will depend upon clinical condition at diagnosis..

  • Are there other alternative methods of treatment?

    • Surgery is only modality available.

  • What all I need to know before my child surgery?

    • Read “All you need to know before your child's surgery” information booklet in website.

  • How is the surgery done?

    • Surgery is thoracotomy and Lobectomy. In this, the surgeon makes a cut over chest of the child and removes the abnormal lobe of lung. It is a major surgery and need support of Pediatric anesthesia and NICU/PICU. After surgery, child is kept in ICU, sometimes on ventilator also.

  • Remarks

    • For more details of surgery, contact your surgeon

  • Related Photographs and videos

    • Few photographs of steps done by me  is given here for learning purposes

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