Reply to the Letter to the Editor Reply to

We would like to express our appreciation to Mr Shanmugam for his letter regarding our article on congenital cystic adenomatoid malformation (CCAM) [1]. He pointed out that the paper failed to provide a new strategy for deciding the surgical timing in CCAM. His comments on the traditional strategy for CCAM are appropriate and we agree with them. As he mentioned, many patients will have antenatally diagnosed CCAM in the future, but many of them may not be present with any significant respiratory symptoms. These patients would not have been diagnosed antenatally in the past until a chest radiograph was taken with/without respiratory symptoms at some point of their life in order to make a diagnosis. Traditionally, we have recommended surgical resection for CCAM, diagnosed either incidentally or with minimal symptoms, at the time of diagnosis for various reasons mentioned in the article. However, there is no clear guideline of management strategy for antenatally diagnosed CCAM patients. Should we recommend surgery for those patients? If so, when is the best timing for surgery? We reviewed our experience, focusing on the timing of surgery and postoperative complications. Although, we found that minor complication rates increased with the patient’s age at the time of operation, the odd ratio per month increase of age was only 1.024. This indicates that the risk of complication will not be significantly increased even if the operation is delayed several months. We also suggested that waiting several years may result in a substantial increase of risk and such a lengthy delay should be avoided. In our experience, we did not find operative mortality nor morbidity among cases operated during the neonatal period. This may not be generalized for every hospital. However, in the major centers where neonatal surgeries have been established and performed routinely, we believe that surgery for CCAM can be performed during the neonatal period without a substantial increase of risk. Considering these factors, we recommended to perform elective surgery at 2–3 months of age for asymptomatic CCAM patients. With regards to the need of intensive care unit care, there will be many patients who will not need such care. However, until we are clearly able to predict the postnatal outcome of antenatally diagnosed CCAM, at least close observation for any respiratory symptoms should be mandatory. Adzick’s classification is based on the ultrasound size and appearance whereas Stocker’s classification is based on the histopathologic findings. It should be emphasized that there is a poor correlation between ultrasound classification and resected histology. It has been suggested that macrocystic lesions tend to be stable but can enlarge and mostly require postnatal surgical excision, whereas microcystic disease may diminish or resolve during pregnancy and, if so, can simply be followed-up and investigated appropriately [2]. However, in general, we agree with Mr Shanmugam’s comments about the macrocytic and microcytic lesions and acknowledge possible misunderstanding of our manuscript. We would like to thank Mr Shanmugan for his informative comments on our paper.