In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience.

BACKGROUND: Severe primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). OBJECTIVES: To compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. METHOD: A retrospective analysis was performed for all fetuses treated by TAS, between the years 2004-2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. RESULTS: 38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. CONCLUSION: TAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Dehydration as a Rare Cause of Pulmonary Artery Thrombosis in a 2-Week-Old Term Neonate.

Pulmonary arterial thrombosis is an extremely rare occurrence in the neonatal population. We describe a 2-week-old female neonate who presented in critical condition with severe cyanosis and dehydration and was found to have a large thrombus in the main branches of the pulmonary arteries. She was successfully treated with surgical embolectomy. Pulmonary arterial thrombosis should always be considered in the differential diagnosis of a dehydrated neonate presenting with severe cyanosis and evidence of pulmonary hypertension.