Prenatally diagnosed cystic hygroma and hydrops fetalis case

Cystic hygroma is also called as cystic lymphangioma. They are cystic lymphatic lesions that may arise in any location of the human body. It is a congenital malformation caused by incomplete connections between lymph veins and venous system, with or without septa, localized or common. It is frequently seen in head and neck region, mostly (75%) on left side. Although its incidence rate is 1/6000 births, it is 1/875 in aborted fetuses. The prognosis depends on many factors such as the karyotype, mass localization, week of gestation when diagnosed, depth of invasion, presence of septa. While it can be isolated, it is also frequently associated with chromosomal anomalies, and Turner syndrome is the most frequent concomitant chromosomal anomaly. Besides, it is also associated with trisomies, cardiac anomalies and hydrops fetalis. Progressive skin edema, pleural effusion, pericardial effusion, ascites and placental edema can be observed. In 43 – 75% of the cases may develop non-immune hydrops fetalis. In this case, we aimed to present non-immune hydrops fetalis associated with cystic hygroma which was prenatally diagnosed. Twenty-eight-year-old patient with G4P2A1 referred to our clinic and in the fetal examination, 17 weeks old live single fetus was observed. The case first referred to our clinic at 7 weeks of gestation and in her second visit at 17 weeks of gestation, cystic hygroma with septation in size of 60x48 mm originating from the posterior area of the neck was observed in the fetal ultrasonographic examination. No other systemic anomaly was observed in the fetus. There were no characteristics in the medical history of the case. The patient was recommended to refer a tertiary center for prenatal diagnosis and cardiac evaluation, but the patient did not follow any recommendation and decided to maintain the pregnancy. In her follow-ups, it was found in the fetal examination carried out on 23 weeks of gestation that hydrops fetalis developed and there was no fetal cardiac activity. The pregnancy of the patient was terminated in a tertiary center. Since the family refused, no fetal autopsy was performed. The diagnosis of cystic hygroma and non-immune hydrops fetalis can e established by ultrasonography easily. Fetal ultrasonographic examination including fetal cardiac evaluation should be done in case with cystic hygroma and hydrops fetalis. The patient should be evaluated in terms of family syndromes and karyotyping should be recommended.

Keywords

Prenatal diagnosis, cystic hygroma, hydrops fetalis.