Primary fetal hydrothorax (PFHT) is a rare entity that refers to a primary accumulation of fetal pleural fluid without any underlying abnormality. It can present with a wide spectrum of severity and can be uni or bilateral.
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Epidemiology
The estimated prevalence is at ~ 1:10,000-15,000 pregnancies. There may be a greater male predilection.
Pathology
It is often characterized by a collection of chyle within the pleural cavity.
Location - distribution
There may be an increased incidence on the right side.
Associations
lymphovascular malformations 6
Radiographic features
Ultrasound
It is often a diagnosis of exclusion.
Ultrasound may show a fetal pleural effusion without any other associated sonographic abnormality. In some cases with dominant hydrothorax to one side, the fetal heart may be displaced to the opposite side.
Complications
Recognized complications include:
development of pulmonary hypoplasia
development of hydrops fetalis
Treatment and prognosis
The clinical course can be extremely variable. In selected cases a pleuro-amniotic shunt can be considered 6.
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