cystic lesions of the lungs encompass a wide spectrum of rare lung lesions comprising of congenital cystic adenomatoid malformation ( ccam ) , congenital lobar emphysema ( cle ) , bronchopulmonary sequestration , and bronchogenic cyst .
ccam with an incidence of 1 in 10,000 to 25,000 pregnancies is the most common lung cyst .
an abnormal antenatal ultrasound scan has now become the most common mode of presentation in europe and the united states . in a small proportion of the cases
fetal intervention , like thoracoamniotic shunts , laser ablation , and resectional surgery , is a possibility in some of these conditions .
management of an asymptomatic lesion is controversial ; while some advocate surgical excision , others recommend resection , only when symptoms occur .
this study retrospectively reviews our experience of congenital cystic lung lesions in terms of antenatal diagnosis , etiologies , presentation , management and complications .
the records of all patients managed at our center with a diagnosis of cystic lung lesions from january 2000 to june 2011 were reviewed .
the following data were compiled : antenatal diagnosis , symptomatology , treatment received prior to referral to our center , imaging findings , location , and type of lesions , type of surgery , postoperative ventilation , complications , and outcomes .
for the 10-year study period , 40 symptomatic patients with a diagnosis of congenital cystic lung lesions were managed at our center . only three ( 8% ) patients were diagnosed antenatally . among the remaining
, 34 had no antenatal ultrasound scan performed and it was reported as normal in three patients . out of the three patients with an antenatal diagnosis , two became symptomatic at birth and one at 48 months .
twenty - nine of the 40 ( 73% ) patients were male and 11 ( 27% ) were female and the median age of presentation was 25 months .
all patients were investigated with a standard chest x - ray ( cxr ) and computed tomography ( ct ) scan .
the cxr was accurate in identifying a cystic lesion in 18 patients ( 45% ) compared with a ct scan , which picked up the lesion in all 40 patients ( 100% ) .
nineteen ( 47.5% ) children became symptomatic in the neonatal period , 13 ( 32.5% ) between one month and one year , seven ( 18% ) between one and five years , and one ( 2% ) beyond five years of age .
respiratory distress was the most common presenting symptom in children less than one year of age , while respiratory infections were common in children presenting after one year of age .
eight ( 20% ) children had received a full course of prior antitubercular therapy due to misdiagnosis .
twelve ( 30% ) children had an intercostal drain ( icd ) inserted , prior to referral to our center , due to a mistaken diagnosis of pneumothorax .
all patients underwent open surgical resection by posterolateral thoracotomy , out of which eight were performed in the neonatal period .
the average duration of the postoperative icd drainage was 3.5 days ( 1 - 11 days ) and the average duration of hospital stay was five days ( 4 - 11 days ) .
the final histological diagnosis was ccam in 19 ( 48% ) , cle in 11 ( 28% ) , bronchogenic cyst in five ( 12% ) , and pulmonary sequestration in five ( 12% ) .
out of 40 patients , 21 ( 52% ) had lesions on the right side and 19 ( 48% ) on the left side , and four patients had multilobar involvement .
two ccams involved all the lobes , one right ccam involved both upper and middle lobes , and one right cle involved both middle and lower lobes [ figure 1 ] .
thirty patients underwent lobectomies , two underwent pneumonectomies , and eight underwent excisions for a bronchogenic cyst and for extralobar pulmonary sequestrations [ table 1 ] .
lobar distribution of the cystic lesions ( including four patients with multilobar involvement ) surgical procedures performed on the various cystic lesions complications included persistent non - expansion of the residual lobe requiring bronchoscopy and suction in two patients , pneumothorax necessitating re - icd reinsertion in one patient , persistent hyperinflation necessitating a redo thoracotomy , and excision of a small foregut duplication cyst in one patient ( this patient had been operated for a cle ) , persistent bronchopleural fistula requiring redo thoracotomy and repair in one patient .
the median period of follow up was 36 months and all patients had been asymptomatic .
antenatal diagnosis of cystic lung lesions is the norm in the western setup , leading to prenatal counseling and planned delivery .
the antenatally diagnosed lesions need to be evaluated postnatally with a ct scan , as the cxr may appear normal in spite of a pathological lesion .
the reported sensitivity of cxr as a test for the presence of ccam is about 61% , while it is 100% for a ct scan . in our series
the cxr picked up the lesions in only 18 patients ( 45% ) , whereas , the ct scan picked up all the chest lesions ( 100% ) .
occasionally , a ct or magnetic resonance ( mr ) angiography may be required for better delineation of the vascular supply in case of pulmonary sequestration .
symptomatically , neonates with cystic lesions tend to manifest with respiratory distress while the older children present with recurrent infections .
out of the 23 children with respiratory distress at presentation , 21 ( 92% ) were neonates , and out of the 27 patients with recurrent respiratory infections , only 10 ( 37% ) were neonates .
twelve ( 30% ) patients had prior icd insertion , due to mistaken diagnosis of pneumothorax .
likewise , eight ( 20% ) patients had been misdiagnosed as tuberculosis and had received complete antitubercular therapy before referral to our center .
antenatal diagnosis , coupled with an awareness of congenital cystic lesions of the lung , could have prevented this wrong treatment .
surgical resection is warranted for symptomatic lesions , but treatment recommendations for asymptomatic lesions are still unclear .
the main arguments for surgical resection in asymptomatic lesions are the potential risk of recurrent infections , pneumothorax , and even malignant transformation . about 4% of the pulmonary tumors
the tumors that develop are , sarcomas , pleuropulmonary blastoma , bronchogenic carcinoma , and mesenchymoma .
in a case report and literature review reported 33 cases of primary pulmonary rhabdomyosarcomas , 15 of which arose in a pre - existing pulmonary cystic malformation .
adzick et al . in their large series of 105 asymptomatic patients demonstrated that surgical resection is safe with no mortality and minimal morbidity .
in their review and meta - analysis of the postnatal management of cystic lesions , concluded that although the risk of asymptomatic cases developing symptoms is small ( 3% ) , elective surgery in asymptomatic cases has significantly reduced postoperative complications compared to emergency surgery in these cases . also they mentioned that segmentectomy was associated with more residual disease ( 15% ) compared to cases in which lobectomies were carried out ( 0% ) .
all our patients underwent lobectomies and none have shown evidence of residual disease on follow up .
minimally invasive surgery has emerged as the standard of care for many pediatric surgical conditions .
the stated advantages are : better postoperative pain control , shorter hospital stay , and improved cosmesis , compared to standard thoracotomy .
thoracoscopic resection of ccam has been reported to have a longer operative time , shorter hospital stay , and potentially reduced complications , with no additional costs , with the main risk factor for conversion to thoracotomy being a prior history of pneumonia .
all our patients underwent posterolateral thoracotomy and resection , with an average hospital stay of five days . probably with the increasing use of video - assisted thoracoscopic surgery ( vats ) , thoracoscopic resection of cystic lesions
although the treatment of cystic lung lesions is quite straightforward , lesions that are not diagnosed antenatally are quite a diagnostic challenge when they become symptomatic , especially in our country ( india ) , where pediatric surgical facilities are not readily available . increased awareness of this uncommon entity coupled with a wider antenatal ultrasonography ( usg ) cover for pregnant mothers would result in prompt referrals to a pediatric surgical center , thereby , obviating unnecessary prolonged and needless medical management like icd insertion and antitubercular therapy .
 background : a majority of cystic lesions in the western world are detected antenatally , whereas , the diagnosis in our setup occurs once the child becomes symptomatic . 
 surgical management is primarily dictated by the presence of symptoms , recurrent infection , and rarely by the potential risk of malignant transformation.materials and methods : a retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from january 2000 through june 2011 for antenatal diagnosis , presentation , diagnostic modalities , treatment , and complications.results:forty cystic lung lesions were identified . only 8% were antenatally detected . 
 out of 40 , the final diagnosis was congenital cystic adenomatoid malformation in 19 , congenital lobar emphysema in 11 , and bronchogenic cysts and pulmonary sequestration in five each . of these 
 , 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center . 
 postoperative morbidity in the form of bronchopleural fistula , pneumothorax , and non - expansion of the residual lung was noted in 10% of the patients.conclusion:antenatal diagnosis of these lesions is still uncommon in third world countries . 
 prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion , due to incorrect diagnosis . 
INTRODUCTION
MATERIALS AND METHODS
RESULTS
DISCUSSION
CONCLUSION