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Congenital Cystic Adenomatoid Malformation of

the Lung (CCAM)



What is it?
CCAM is a disorder affecting the foetus involving a cyst of abnormal lung tissue.  This tissue has increased cell proliferation and decreased apoptosis.  This means that the CCAM cells can reproduce quickly and the nuclei of the cells do not effectively instigate cell death as signalled by the condition of the cells.  This cystic tissue cannot function as lung tissue, and CCAM commonly develops during the seventh gestational week.  CCAM cells can be large enough to resemble bronchioles or can exist on a microscopic scale.



                                                                    
This is an ultrasound of a CCAM lung.
                                                                     The cyst is visible in the upper right lung.

 




Diagnosis
Initial symptoms of CCAM are evident exteriorly from the pregnant mother.  She appears too large for her stage in the pregnancy, and this is caused by the foetus' inability to swallow the amniotic fluid being produced.  These initial signs can be verified through an ultrasound examination in which bright mass will appear in the foetus' lungs.  Secondary indicators also include a dislodged heart and flattened diaphragm.  Often, the CCAM ceases its growth after the twenty seventh week while the lungs continue to develop and exceed the size of the CCAM.  In these situations the CCAM become less threatening and can be handled after birth.  However, certain CCAM cases result in pulmonary hypoplasia as the lungs do not have room to grow.  In these severe cases the CCAM can apply pressure to the trachea, thereby blocking the amniotic fluid from being swallowed, and the CCAM also applies pressure to the heart, stressing the circulatory system of the foetus (hydrops). 




                                  
A CT scan provides a detailed view of
                                  
the CCAM.








Treatment
In severe cases requiring inutero surgery, the cyst must be removed to save the foetus.  The procedure involves the opening of the womb as would be done in a caesarean section.  The amniotic fluid is collected and is reinserted following the removal of the CCAM.  After the foetus is exposed the heart rate is monitored by a pulse oximetre.  An incision is made under the armpit of the foetus, exposing the lung cavity.  The CCAM is then removed from the lungs.  The amniotic fluid is then replaced and the womb is stitched closed.  The trauma induced to the foetus through this procedure often results in preterm labour.  For this reason, mothers are ordered to bed rest to avoid any further labour stimulus and they must also take anti-contraction medications. 















         
The CCAM is accessed via an incision below                      A recovery time of approximately six weeks
         
the armpit.                                                                       is required for the lungs to recover and expand
                                                                                                 propery into their cavity
.
 

Additional Information
CCAM occurs in approximately one in every thirty thousand foetuses.  Only ten percent of these cases require inutero surgery, and CCAM usually becomes evident for diagnoses after twenty-two weeks.  There do not appear to be any genetic causes of CCAM.  The Fetal Treatment Center in San Francisco remains to be the only centre performing the foetal surgeries required to save foetuses diagnosed with severe CCAM.






Introduction (Homepage)

The Anatomy and Development of the Foetus


Medical Conditions Affecting the Foetus from initial diagnosis to treatment:
Congenital Diaphragmatic Hernia (CDH)
Congenital Cystic Adenomatoid Malformation of the Lung (CCAM)
Sacrococcygeal Teratoma (SCT)
Urinary Tract Obstruction

Related Technologies:
Ultrasound
Fetoscope
Radiofrequency Ablation (RFA)


Bibliography

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