ISSN - 0973-0958

Pediatric Oncall Journal

Cystic Adenomatoid Malformation of Lung in a Neonate 01/09/2014 00:00:00

Cystic Adenomatoid Malformation of Lung in a Neonate

Neeraj Jain, Deepak Seth, Vibha Magal, Varun Vij, Harijot.
Himalayan Hospital, Jolly Grant, Deharadun, India.

Neeraj Jain , 77 SBM Complex, Rishikesh- 249201 India.
This baby was born to a 26 year old gravida 3 mother at Himalayan Hospital, Jolly Grant, Deharadun. Her previous two pregnancies were spontaneous abortions at 2 to 3 month of gestation. This baby was delivered through emergency caesarean section (LSCS), indication being polyhydramnios and severe pregnancy induced hypertension (PIH). During her antenatal check up, ultrasound showed single live fetus having a large cystic mass in left lung (6.4 x 4.4 cm) with polyhydramnios. Following this MRI was done which showed congenital cystic adenomatoid malformation type 1 with polyhydramnios. MRI revealed small, irregular hyperintensities within the left lung parenchyma with few well defined cysts. Mass effect was also noted with inversion of the dome of the diaphragm and mediastinal shift to right. During her antenatal visits mother was prognosticated about the adverse fetal outcome and was advised termination but mother wanted to carry the pregnancy till term. Baby was delivered with meconium stained liquor. Baby cried immediately after birth. Oropharyngeal suctioning was done. APGAR score was 8, 9, 9 at 1, 5, 10 minutes respectively. Baby was shifted to nursery for further management. On examination general condition was stable. Vitals were maintained and baby was maintaining saturation at room air. Capillary filling time was less than 3 seconds. Respiratory examination showed decrease chest movements on the left side as compared to the right. On auscultation there was unequal air entry both the sides with left being less than the right. Other systems were normal. Baby was maintaining saturation till 6 hours of life, when he started having respiratory distress. Emergency lobectomy left side was done on Day 3 of life and baby was put on ventilatory support. Mass was sent for histopathological examination. Initially baby was maintaining on ventilatory support, but baby general condition deteriorated further. Blood investigations done revealed Hb-16.8gm/dl, total Leucocyte count -16810/cumm, CRP was negative, platelet count was 1 lakh, blood culture was sterile. Inspite of our best efforts, baby expired on Day 4 of life. Histopathological report of the mass confirmed cystic adenomatoid transformation of the lung.

Pulmonary cysts may either be single or multiple. The most common form of this type of anomaly in the neonate is cystic adenomatoid malformation which probably results from failure of a portion of the lung to develop. This disorder consists of hamartomatous or dysplastic lung tissue mixed with more normal lung and is usually confined to one lung. Lower lobes are frequently involved. Very large masses may compress the esophagus, thereby impairing swallowing of amniotic fluid and resulting in polyhydramnios. Hydrops occur in 45% of fetuses and is associated with combined fetal and postnatal mortality rates of 68% to 89%. Three histological pattern have been studied. TYPE 1 is macrocystic and consists of single or several large cysts >2 cm lined with ciliated pseudostratified epithelium. There are normal alveoli between the cysts. TYPE 2 consists of numerous small evenly spaced cysts that are less than 1 cm in diameter having a thin muscular coat which features large alveolar like structure between the cysts. TYPE 3 consists of bulky, firm masses of intricately folded ciliated and non ciliated cuboidal epithelium and a thick layer of smooth muscle (these occupy the entire lobe or lobes of the lung). Associated anomalies are noted in about 20% of cases including renal agenesis or dysgenesis, intestinal atresia, imperforate anus, diaphragmatic hernia and cardiac anomalies. This anomaly is readily detected by prenatal ultrasonography. In the fetus, the mass of cystic adenomatoid malformation may compress the adjacent normal lung, producing pulmonary hypoplasia. Newborns with respiratory distress might require intubation and ventilatory support. Definitive treatment of symptomatic patient requires resection of the affected lobe or lobes. Prognosis depends on the severity of pulmonary hypoplasia. In a literature review of babies whose condition was diagnosed in utero and who were then delivered, 26% died in the neonatal period before or after surgery.
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Funding None
Conflict of Interest None
Cite this article as:
Jain N, Seth D, Magal V, Vij V, H. Cystic Adenomatoid Malformation of Lung in a Neonate. Pediatr Oncall J. 2008;5: 106.
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