Congenital Cystic Adenomatoid Malformation: A Case Study

摘要

This paper examines a short case history of an infant born with a Congenital Cystic Adenomatoid Malformation (CCAM). CCAM is a rare congenital lung malformation representing 25% of congenital malformations and 95% of congenital lung lesions.1,2 CCAM may be diagnosed in utero by ultrasound examination, or after birth with the presentation of respiratory distress like symptoms. Differential diagnosis includes diaphragmatic hernia, pulmonary sequestration, bronchogenic cysts, and congenital lobar emphysema. Because most CCAM lesions are manageable with the proper assessment, diagnosis, and interventions, it is vital that members of the neonatal health care team be knowledgeable about this malformation. The natural history, differential diagnosis, pathophysiology and treatment of CCAM are discussed. Introduction Congenital Cystic Adenomatoid Malformation (CCAM) first identified in 1949 by Ch'in and Tang, is a rare congenital malformation of the lung representing 25% of congenital lung malformations and 95% of congenital lung lesions.1,2 This lesion occurs more often in males (1.8:1), and is primarily unilateral, but may occur bilaterally.3 Associated anomalies are rare. Since the technological advancement of ultrasound examination, CCAM has been increasingly diagnosed on routine prenatal examinations. Some CCAM lesions present only at birth with respiratory distress symptoms but are confirmed by an abnormal chest radiograph or a more definitive computed tomography scan. However, there are CCAM lesions that are not identified on routine ultrasound examination, and present without symptoms at birth. These lesions may not be identified until later in life.Whether diagnosed prenatally or postnatally, and with or without symptoms, most CCAM lesions are manageable with the proper assessment, diagnosis, and interventions. Moreover, with the advent of fetal therapy, infants with the more severe cases of CCAM are now surviving. Case History Baby boy C was born to a para 2 gravida 1 mother. The mother's prenatal history was unremarkable. On a routine prenatal ultrasound, a cystic lung lesion was noted in the left lower lobe of the lung. From ultrasound examination, the cyst appeared to measure 3cm by 4cm. The fetus and cyst were followed in utero by serial ultrasound examination until the time of delivery. No complications were documented throughout the pregnancy. Infant C was delivered vaginally at 382/7 weeks gestation, the amniotic fluid was clear and odorless. The Apgar scores were 8 and 9 at 1 and 5 minutes respectively. The birth weight was 3260 grams. The neonatology team was available at the delivery, but no resuscitation measures were needed. Infant C was admitted to the Neonatal Intensive Care Unit (NICU) for further evaluation, but stayed less than 24 hours. A computed tomography scan identified a lesion occupying half of the left lower lobe of the lung. Infant C showed no signs of respiratory distress and began nippling feeds without difficulty. The infant was evaluated by the pediatric surgery team, and transferred to the newborn nursery at approximately 24 hours of life, and then discharged to home.At 3 months of age, Infant C was admitted back into the hospital for intrathoracic surgery to resect the congenital cystic lung lesion. Infant C had no documented issues during these 3 months at home. Infant C underwent general anesthesia, and an epidural catheter was placed. A thoracotomy and lobectomy were performed with subsequent placement of a chest tube. The infant was extubated in the operating room, sent to the post-anesthesia care unit, and admitted to the level II nursery. Infant C's hospital course was uneventful. The infant received pain control per the epidural catheter with ibuprofen supplementation every 6 hours when necessary. The chest tube was removed on day 3 post-op; the epidural catheter was removed 3 hours after the chest tube. Tylenol with codeine was administered every 4 hours as needed after the epidural cat