Case report of congenital cystic adenomatoid malformation type III with lethal outcome.

We present a rare case of congenital cystic adenomatoid malformation (CCAM) type III (mycrocystic) in young, 19 years old primigravida. Diagnosis has been accomplished by ultrasound examination in 26th week of gestation. Hyperechogenic, mycrocystic mass was found in left pulmonary lobe with contra lateral displacement of mediastinum, hypoplasia of the right lung and extensive polyhydramnios. Prenatal therapy was based upon a serial of amniodrainage procedures and pharmacological inhibition of amniotic fluid production and uterine activity. Spontaneous preterm labor occurred at 34 weeks of gestation, Diagnosis was confirmed postnatal. Infant was ventilated due to respiratory insufficiency and was operated at the age of 24 hours. Lethal outcome occurred during the second day due to heavy respiratory insufficiency provoked by pulmonary hypoplasia. We discuss about types of a disease, prenatal diagnostic and therapeutic possibilities and pregnancy outcome.

[1]  K. Lakhoo,et al.  Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years’ experience 1991–2001 , 2005, Archives of Disease in Childhood - Fetal and Neonatal Edition.

[2]  N. Andaç,et al.  Congenital cystic adenomatoid malformation of the lung. , 2004, JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie.

[3]  J. Deutinger,et al.  Longitudinal observation of antenatally detected congenital lung malformations (CLM): natural history, clinical outcome and long-term follow-up. , 2003, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[4]  E. Mark,et al.  Cystic adenomatoid change is common to various forms of cystic lung diseases of children: a clinicopathologic analysis of 10 cases with emphasis on tracing the bronchial tree. , 2002, Archives of pathology & laboratory medicine.

[5]  J. Rychik,et al.  Echocardiographic evaluation of the fetus with congenital cystic adenomatoid malformation , 2000, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[6]  A. Cavaliere,et al.  Congenital Cystic Adenomatoid Malformation of the Lung: Antenatal Ultrasound Findings and Fetal-Neonatal Outcome , 2000, Fetal Diagnosis and Therapy.

[7]  Fen-fen Chen,et al.  The glandular component in congenital cystic adenomatoid malformation of the lung , 1999, Respirology.

[8]  J. Estroff,et al.  Fetal lung masses: prenatal course and outcome. , 1995, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[9]  R. Filly,et al.  Fetal surgery for cystic adenomatoid malformation of the lung. , 1993, Journal of pediatric surgery.

[10]  M. Cloutier,et al.  Congenital cystic adenomatoid malformation. , 1993, Chest.

[11]  J. JiménezMorales,et al.  Congenital cystic adenomatoid malformation of lung , 1992 .

[12]  R. Drake,et al.  Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. , 1977, Human pathology.

[13]  C. Ky,et al.  Congenital adenomatoid malformation of one lobe of a lung with general anasarca. , 1949 .