PP-035 Hydrops fetalis: etiologies, management and outcome

Hydrops fetalis (HF) is defined as excessive accumulation of amniotic fluid in the extravascular fetal compartment and at least two serous cavities. It manifests as subcutaneous edema, pleural or pericardial effusion and ascites. Etiologies are classified as immunological and non-immunological.
The objective of this study is to identify the clinical, etiological, therapeutic and evolutionary characteristics of HF in the neonatal period.

It is a descriptive and retrospective study, having included all newborns (NB) hospitalized in the neonatal intensive care unit of Farhat Hached Hospital of Sousse for management of HF, over a period of 7 years (January 2017- December 2023).

The study included 20 NB. Sex ratio (F/M) = 2/3. Mean gestational age was 37 weeks' gestation (WG). HF was diagnosed during pregnancy in 17 women at a mean term of 28 WG. Hydramnios was associated in 12 cases and pulmonary hypoplasia in 6 cases. The pregnancy was complicated by gestational diabetes in 6 cases. All NB required resuscitation with intubation in the delivery room. Clinical signs were dominated by subcutaneous oedema in 18 cases, large ascites in 12 cases, pleural effusion in 10 cases, hepatosplenomegaly in 9 cases and pallor in 5 cases. Other signs were facial dysmorphia in 6 cases, heart murmur in 2 cases and tachycardia in 1 case. Cardiac origin was the predominant etiology, with one case of atrial flutter, one case of Ebstein's disease, one case of transposition of great arteries and 2 cases of coarctation of the aorta. Intra-thoracic malformations were found in 3 patients (diaphragmatic hernia (2 cases) and digestive duplicity (1 case)). Anemia was the etiology of hydrops in 5 cases, of infectious (2 cases) or immunological (3 cases) origin, two of which received an in utero transfusion. Overload disease was found in 2 cases: mucopolysaccharidosis in 1 case, sialidosis type 2 in 1 case. One patient had congenital hypothyroidism and another a cystic lymphangioma. The etiology remained undetermined in 3 cases. Therapeutically, mechanical ventilation was used in all NB with surfactant instillation in 6 cases. Effusion puncture was performed in 8 cases. Exchange transfusion was used in cases of fetomaternal alloimmunization. Only 3 cases (atrial flutter after electrical and drug-induced cardioversion, rhesus alloimmunization and cystic lymphangioma) had a favorable outcome.

HF is a severe life-threatening neonatal disorder. The incidence of immunological etiologies has declined considerably since the prevention of Rhesus alloimmunization by anti-D immunoglobulin prophylaxis.