P24.09: The role of computed tomography in the investigation of stillbirth due to congenital malformation

Abstract

Cystic hygromas are considered a form of vascular malformations of the lymphatic system, with an estimated incidence of 1 in 12,000 births (Fonkalsrud). They are fluid-filled sacs consisting of single or multiple cysts, that result from a blockage in the lymphatic system, mostly at the neck. They occur in approximately 1% of fetuses and have a high mortality rate when diagnosed prior to 30 weeks, because they can progress to hydrops and fetal death, or evolve to webbed neck, edema and lymphangioma. They may be an isolated finding or part of a syndrome such as Turner, Noonan and some trissomies. The success of the pregnancy depends on several factors, and the birth is a critical moment for the life of the patient. Primigesta, young Eastern European women, where a left cystic cervical hygroma was detected at 27 weeks of gestation in routine ultrasound. A medical interruption of pregnancy was proposed but refused and the pregnancy was carried out until full term. A thorough study was performed, with ultrasound scans and MRI, and no other congenital anomalies detected. Delivery was by a planned cesarean at full term, with Apgar scores 9 and 10 at first and five minutes, respectively, and the weight, height and head circumference were appropriate for gestational age. As shown by previous imaging studies, there was a clear airway and neither respiratory distress nor trouble feeding was observed. The karyotype was 46, XY and on day 16, he had surgical removal of the hygroma, which occurred without incidents. This case exemplifies the difficulty of pre-natal diagnosis and advising parents of a potential deadly condition. Even though this case was a surgical success, pre-natal findings of cystic hygroma are generally associated with a poor prognosis, especially when diagnosed prior to 30 weeks. The MRI assisted us in the decision taking process by reassuring a non-obstructed airway and normal fetal anatomy.