Fetal pulmonary cyst: Intrauterine diagnosis and management Janice B. Asher, M.D., Rudy E. Sabbagha, M.D., Ralph K. Tamura, M.D., Susan Luck, M.D., and Albert B. Gerbie, M.D. Chicago, Illinois This case report describes the antenatal diagnosis of a fetal pulmonary anomaly. Early detection afforded detailed parental counseling and prompt neonatal diagnosis and treatment. (AM J OBSTET GYNECOL 1985; 151:97-8.)
Key words: Fetal pulmonary cyst, ultrasound, diaphragmatic hernia
We report a case illustrating how prenatal ultrasonic diagnosis of a major anomaly influenced patient management to bring about a favorable outcome.
Case presentation The patient, a 38-year-old primigravid woman, had a normal past medical history and initial physical examination at 12 weeks' gestation. Because of advanced maternal age, an amniocentesis was recommended and performed at the sixteenth week of pregnancy. Prior to the amniocentesis, a standard stage I ultrasound study was carried out to date the pregnancy, rule out twins, establish fetal life, localize the placenta, and define the most suitable location on the maternal abdomen for amniocentesis. The results of amniotic fluid analysis for fetal karyotype and level of a-fetoprotein were normal. A second ultrasound examination was performed at 23 weeks' gestation to evaluate fetal growth. Although the fetal head and trunk measurements were normal, it was noted that the heart and mediastinum were displaced to the right side of the chest (Fig. I). The stomach bubble could not be identified in the upper abdomen. Thus, the primary diagnosis was diaphragmatic hernia; the differential diagnosis included pulmonary cyst and adenomatoid cystic malformation of the lung. The patient and her husband were informed about the presence of a major anomaly in the fetal chest and the differential diagnosis of the abnormality. They were advised that all conditions could be associated with hypoplastic lungs and also that if the defect was, in fact, a diaphragmatic hernia the prognosis was quite grave. From the Departments of Obstetrics and Gynecology and Pediatric Surgery, Northwestern University and Northwestern and Children's Memorial Hospitals. Received for publication July 9, 1984; revised September 21, 1984; accepted October 1, 1984. Reprint requests:Janice B. Asher, M.D., 707 North Fairbanks Court, Suite 500, Chicago, IL 60611.
The couple elected to continue the pregnancy and expressed their desire to have treatment of the infant be as aggressive as necessary, including cesarean section for fetal distress if indicated. The remainder of the pregnancy was complicated by the development of gestational diabetes, which was well controlled by diet. Ultrasound examination at 32 weeks showed no change in the abnormal findings. The stomach bubble was still not visualized. Weekly nonstress tests begun at 36 weeks were reactive. To facilitate attendance of a team of specialists for optimal neonatal care, labor was induced with Pitocin at 39 + weeks of pregnancy, as defined by early ultrasonic parameters. Labor progressed normally, and a male infant weighing 7 pounds, I ounce was delivered by low forceps over a mediolateral episiotomy. Pudenda! anesthesia was used. The baby did not breathe spontaneously and was intubated immediately. Since it was known that the infant could be expected to have hypoplastic lungs, excessive pressure with the Ambou bag was avoided so as to prevent pneumothorax. No breath sounds were detected over the left aspect of the chest, and heart sounds were audible adjacent to the right midclavicular line. Chest and abdominal roentgenograms showed multiple air-filled pockets in the left lung area, a right mediastinal shift, and gas within a normally rotated colon within the abdomen (Fig. 2). With these findings, the presumptive diagnosis was a pulmonary cystic lesion. The baby was transferred to Children's Memorial Hospital and was taken directly to a previously prepared operating room. With the infant under general anesthesia, the pleural space was entered and a large, cystic, left upper lobe was seen along with a small but normal-appearing left lower lobe. A left upper lobectomy was performed. The pathologic diagnosis was cystic adenomatoid malformation of the lung. After operation, the baby did well. At l year of age, height was at the seventy-fifth percentile and weight at the fiftieth percentile.
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Fig. 1. Echogram showing cross section of fetal chest. Heart is markedly displaced to the right side by a large mass (arrows) occupying the left aspect of the chest.
Comment This case history demonstrates not only the feasibility of antenatal diagnosis of remediable fetal anomalies by ultrasound but also the importance of detailed counseling and individualized management. The differential diagnosis of the chest mass was presented to the parents, who understood that the prognosis might be quite unfavorable. However, they were able to make an informed decision based on available information and were emotionally prepared for the ensuing course of events. Furthermore, the timing of delivery was well controlled and expedited the attendance of a team of specialists to resuscitate the infant, prevent pneumothorax, execute a planned battery of tests, and perform immediate operation on the baby. REFERENCES
Fig. 2. Chest x-ray film of newborn infant.
I. Sabbagha RE, Tamura RK, Da!Compo S. Obstetric ultrasonography in perspective. Perinatal Neonatol 1982;53: 53-62. 2. Nishibayashi SW, Andrussy RJ, Woolley MM. Congenital cystic adenomatoid malformation: a 30-year experience. J Pediatr Surg 1981;16:704-6.