Prenatal treatment of type I congenital cystic adenomatoid malformation by intrauterine fetal thoracentesis

Abstract

Congenital cystic adenomatoid malformation of the lung is a developmental abnormality characterized by abnormal proliferation of terminal bronchioles forming cysts of varying sizes. Extensive lesions are associated with a poor prognosis due to the development of nonimmune hydrops and/or pulmonary hypoplasia. The advent of high-resolution ultrasonography has made it possible to identify these lesions during the antenatal period. With the prenatal identification of cystic adenomatoid malformations comes the potential for in utero intervention to modify the natural history of this process and prevent the development of hydrops and pulmonary hypoplasia. An instance of successful intrauterine treatment of congenital cystic adenomatoid malformation is presented. CASE REPORTA 32-year-old primagravida was referred at 28 weeks, menstrual age, because of a cystic chest mass noted on ultrasound examination. Ultrasound evaluation revealed a 28-week fetus with a 4 cm X 3.5 cm X 2.5 cm unilocular cystic mass filling the left side of the fetal thorax, displacing the heart into the right hemithorax ( Figure 1). The fetal stomach was present in the appropriate location and the diaphragm appeared intact. No evidence of fetal hydrops was identified. Based on the ultrasound findings, congenital cystic adenomatoid malformation was felt to be the most likely diagnosis followed by pulmonary sequestration or bronchogenic cyst. Given the relative size of the cyst, the fetus was felt to be at significant risk for pulmonary hypoplasia. Likewise, with the degree of mediastinal shift present, there was concern that cardiac function could ultimately be compromised leading to nonimmune hydrops. These concerns were discussed with the parents, who strongly desired that an attempt at intrauterine therapy be made. It was therefore planned to proceed with fetal thoracentesis followed by interval placement of an indwelling shunt only if there was significant fluid reaccumulation.Cessation of fetal movement was achieved by injecting 0.3 mg of pancuronium bromide into the fetal thigh under ultrasound guidance (Acuson 128). An 18-gauge Tuohy needle was then guided into the center of the cyst. Immediately on removing the stylet, there was brisk flow of clear viscous fluid. After removal of '22 mL of fluid, the cyst had completely disappeared and heart shifted back to the left. An umbilical vein blood sample for cytogenetic analysis was then obtained from the placental insertion of the cord. Fetal heart activity remained stable throughout the procedures.Analysis of the fluid included a cell count and differential with 545 red blood cells and 355 white blood cellskmm with the differential showing 13% segs, 36% lymphs, and 51% histiocytes. Protein was less than 1.0 g/dL. Cytologic examination of the fluid revealed numerous histiocytes, karyorrhectic debris, and ciliated columnar epithelial cells. Cytogenetic analysis of the fetal blood sample yielded a normal 46,XY karyotype.Serial follow-up ultrasound scans were obtained. On the first day following the pr...

Antenatal Diagnosis and Surgical Management of Congenital Cystic Adenomatoid Malformation of the Lung

“…There have been sev eral reports on aspiration and shunt opera tion. Aspiration was not effective as 3 of 4 cases showed re-expansion [3][4][5][6], while a cystamniotic shunt was reported to be effective. In particular, the first report was by Clark et al [5] who reported on the successful treat ment of a hydrops patient by using the double pigtail catheter.…”

Successful Outcome in a Case of Cystic Adenomatoid Malformation of the Lung Complicated by Fetal Hydrops, Using Extracorporeal Membrane Oxygenation

“…Our case demonstrated the unusual associa tion of fetal hydrops and a type 1 lesion. We did not attempt to drain the cystic mass in utero, although this procedure has been de scribed in 2 cases, leading to resolution of fetal hydrops in 1 [11,12], Spontaneous reso lution of the hydrops has also been reported in association with a macrocystic lesion [13]. Our case also demonstrated spontaneous re solution of hydrops, but it is not at all clear why this should have occurred.…”

Prenatal Catheter Placement for Fetal Cystic Adenomatoid Pulmonary Malformation: A Case Report

“…This present case demonstrates that prenatal placement of the catheter for continuous drainage may allow several additional weeks of lung and organ development to occur in utero [4,10] . Drainage and relief of the mass effect of the cyst appears to be suffi cient treatment in these cases, but may require several separate procedures if there is reaccumulation of fl uid.…”

“…Large unilateral lesions are often associated with devia-tion of the mediastinum in the contralateral side, cardiac compression, hydrops, and in about 30% of cases there is polyhydramnios which is likely to be due to decreased fetal swallowing, the consequence of esophageal compression [3] . Drainage procedures in the last decades have been successful in treating select cystic tumors [4] . Based on this experience, it was believed that a large type I CCAM causing deviation of the mediastinum could be successfully drained with prenatal placement of a catheter.…”