Cystic Hygroma of the Neck Pathologic study of 26 autopsy cases

Suh, Yeon Lim; Chi, Je Geun

Abstract

Cystic hygroma is a congenital malformation of the lymphatic system appearing single or multiloculated fluid-filled cavities, most often around the neck. They often progress to hydrops and cause fetal death, and frequently associated with chromosomal abnormalities and other congenital malformations. The purpose of our study is to delineate the nature of cystic hygroma and determine the relationship between cystic hygroma and associated anomalies including fetal hydrops. We used data from 26 cases of cervical cystic hygroma in autopsy files of SNU Children's Hospital. Most of cystic hygroma were found in stillborn or premature infants. The fetal cases had been dead for a quite a long period since there was discrepancy between the true gestational age and the developmental age estimated from the body length. Of 26 fetuses only 2 were studied chromosomally and both of them showed 45X. Of 26 cystic hygromas 23 occurred in the posterior neck and 3 in the anterior neck. They ranged from 2.5 to 14 cm (mean: 7.9 cm). The cystic hygroma of the posterior neck consisted of two symmetric sacs on both sides and in the nape and extended to the occipital region. The cystic hygromas of the anterior neck were unilateral or bilateral, and multiloculated and extended into the adjacent cheek. Cystic hygromas of posterior neck were always associated with hydrops, while no recognizable hydrops was found in cystic hygromas of anterior neck. The cystic hygromas were larger in patients with severe hydrops than in patients with less severe hydrops. Associated abnormalities, found in 88%, included hydrops fetalis(88%), growth retardation(80%), cardiovascular anomalies(27%), horseshoe kidney(23%), skeletal anomalies(12%) and hypoxic changes(31%) in visceral organs. In summary, when a hygroma is detected during fetal life, careful sonographic examination for associated congenital anomalies, fetal karyotyping and consideration of artificial abortion are indicated.