Indian J Pediatr DOI 10.1007/s12098-014-1462-z
SCIENTIFIC LETTER
Self Resolving Congenital Cystic Adenomatoid Malformation of the Lung in an Infant: Myth or Reality Arjun Sira Kamaraja Gupta & Prashanth Madapura Virupakshappa & Ramesh Hanumanthappa & Guruprasad Gowdar
Received: 26 September 2013 / Accepted: 10 April 2014 # Dr. K C Chaudhuri Foundation 2014
Introduction To the Editor: Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare developmental anomaly with an incidence of 1:25,000 to 1:35,000; males and females are equally affected [1]. We report a rare case of self resolving CCAM of the lung. A 12-d-old male neonate (birth weight of 2,250 g) presented with faulty feeding, respiratory distress and failure to thrive with admission weight of 1,670 g. Baby was diagnosed to have community acquired pneumonia (left mid zone ring lesion). Baby improved with co-amoxiclav and amikacin for 2 wk and was discharged on 25th day of life. Baby was readmitted on 31st day of life with similar complaints. He was diagnosed as a case of sepsis with aspiration pneumonia; treated with Inj ceftazidime, for 2 wk. Chest radiograph showed persistence of left mid zone ring lesion; CT scan of chest was done and pediatric radiologist reported -Type I Congenital cystic adenomatoid malformation (Fig. 1). Contrast CT was not done as baby’s renal parameters were altered. Ultrasound abdomen and A. S. Kamaraja Gupta (*) : R. Hanumanthappa Department of Pediatrics, JJM Medical College, #2811/1, 3rd main, 4th cross, MCC B Block, Davanagere 577004, Karnataka, India e-mail: [email protected] P. M. Virupakshappa : G. Gowdar Department of Neonatology, JJM Medical College, Davanagere, Karnataka, India P. M. Virupakshappa Department of Pediatrics, M.S. Ramaiah Medical College and Hospitals, Bangalore, India
Echocardiography were normal. Surgical intervention was postponed as the baby’s weight was 1,700 g. Baby was discharged on request with Syp coamoxiclav (discharge wt- 1,720 g). On follow-up (78th day of life) child was asymptomatic, weight −2,510 g. Repeat chest radiograph & CT chest were normal (Fig. 2). A diagnosis of self resolving CCAM was established. CCAM is a rare congenital cystic lung lesion arising from excessive disorganized proliferation of tubular bronchial structures. It is usually unilateral and restricted to a single lobe [2]. The CCAM develops during the pseudoglandular and saccular period (7–35 wk). Stocker using clinical and pathologic features divided CCAM into three subtypes in 1974 and later added two more subtypes in 2002: (1) type 0 – acinar dysplasia, (2) type I - multiple large cysts or a single dominate cyst, (3) type II - multiple evenly spaced cysts, (4) type III bulky firm mass, (5) type IV – peripheral cyst type [3]. Calvert et al. documented three cases in which abnormalities on postnatal imaging resolved by 9 mo of age [4]. For asymptomatic cases, some centre advocate conservative management. Surgery is postponed if the patient is asymptomatic but CT chest is done within 1 mo postnatally to demonstrate any connection with the tracheobronchial tree, evaluate the blood supply so that surgery can be performed in a planned manner due to long term risks [5].
Conflict of Interest None.
Indian J Pediatr Fig. 1 a Chest radiograph showing ring lesion in left lower lobe of the lung. b CT chest showing large air filled multicystic intralobar lesion in the left lower lobe and lingular lobe communicating with the left main bronchus with mediastinal shift Type I Congenital cystic adenomatoid malformation
Fig. 2 Radiological resolution of the cyst after 6 wk
Role of Funding Source None.
References 1. Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther. 2001;6:178–86. 2. Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K. Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg. 2004;39:549–56.
3. Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985;20: 483–8. 4. Calvert JK, Boyd PA, Chamberlain PC, Syed S, Lakhoo K. Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years’ experience 1991–2001. Arch Dis Child Fetal Neonatal Ed. 2006;91:F26–8. 5. Gornall AS, Budd JL, Draper ES, Konje JC, Kurinczuk JJ. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn. 2003;23:997–1002.
SCIENTIFIC LETTER
Self Resolving Congenital Cystic Adenomatoid Malformation of the Lung in an Infant: Myth or Reality Arjun Sira Kamaraja Gupta & Prashanth Madapura Virupakshappa & Ramesh Hanumanthappa & Guruprasad Gowdar
Received: 26 September 2013 / Accepted: 10 April 2014 # Dr. K C Chaudhuri Foundation 2014
Introduction To the Editor: Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare developmental anomaly with an incidence of 1:25,000 to 1:35,000; males and females are equally affected [1]. We report a rare case of self resolving CCAM of the lung. A 12-d-old male neonate (birth weight of 2,250 g) presented with faulty feeding, respiratory distress and failure to thrive with admission weight of 1,670 g. Baby was diagnosed to have community acquired pneumonia (left mid zone ring lesion). Baby improved with co-amoxiclav and amikacin for 2 wk and was discharged on 25th day of life. Baby was readmitted on 31st day of life with similar complaints. He was diagnosed as a case of sepsis with aspiration pneumonia; treated with Inj ceftazidime, for 2 wk. Chest radiograph showed persistence of left mid zone ring lesion; CT scan of chest was done and pediatric radiologist reported -Type I Congenital cystic adenomatoid malformation (Fig. 1). Contrast CT was not done as baby’s renal parameters were altered. Ultrasound abdomen and A. S. Kamaraja Gupta (*) : R. Hanumanthappa Department of Pediatrics, JJM Medical College, #2811/1, 3rd main, 4th cross, MCC B Block, Davanagere 577004, Karnataka, India e-mail: [email protected] P. M. Virupakshappa : G. Gowdar Department of Neonatology, JJM Medical College, Davanagere, Karnataka, India P. M. Virupakshappa Department of Pediatrics, M.S. Ramaiah Medical College and Hospitals, Bangalore, India
Echocardiography were normal. Surgical intervention was postponed as the baby’s weight was 1,700 g. Baby was discharged on request with Syp coamoxiclav (discharge wt- 1,720 g). On follow-up (78th day of life) child was asymptomatic, weight −2,510 g. Repeat chest radiograph & CT chest were normal (Fig. 2). A diagnosis of self resolving CCAM was established. CCAM is a rare congenital cystic lung lesion arising from excessive disorganized proliferation of tubular bronchial structures. It is usually unilateral and restricted to a single lobe [2]. The CCAM develops during the pseudoglandular and saccular period (7–35 wk). Stocker using clinical and pathologic features divided CCAM into three subtypes in 1974 and later added two more subtypes in 2002: (1) type 0 – acinar dysplasia, (2) type I - multiple large cysts or a single dominate cyst, (3) type II - multiple evenly spaced cysts, (4) type III bulky firm mass, (5) type IV – peripheral cyst type [3]. Calvert et al. documented three cases in which abnormalities on postnatal imaging resolved by 9 mo of age [4]. For asymptomatic cases, some centre advocate conservative management. Surgery is postponed if the patient is asymptomatic but CT chest is done within 1 mo postnatally to demonstrate any connection with the tracheobronchial tree, evaluate the blood supply so that surgery can be performed in a planned manner due to long term risks [5].
Conflict of Interest None.
Indian J Pediatr Fig. 1 a Chest radiograph showing ring lesion in left lower lobe of the lung. b CT chest showing large air filled multicystic intralobar lesion in the left lower lobe and lingular lobe communicating with the left main bronchus with mediastinal shift Type I Congenital cystic adenomatoid malformation
Fig. 2 Radiological resolution of the cyst after 6 wk
Role of Funding Source None.
References 1. Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther. 2001;6:178–86. 2. Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K. Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg. 2004;39:549–56.
3. Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985;20: 483–8. 4. Calvert JK, Boyd PA, Chamberlain PC, Syed S, Lakhoo K. Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years’ experience 1991–2001. Arch Dis Child Fetal Neonatal Ed. 2006;91:F26–8. 5. Gornall AS, Budd JL, Draper ES, Konje JC, Kurinczuk JJ. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn. 2003;23:997–1002.
