Clinician-to-Clinician Update Clinician-to-Clinician Update

Newborn with Idiopathic Hydrops and Chylothorax Successfully Treated with ECMO

June 2015 - Neonatology

Excessive fluid accumulation in body cavities, the condition known as hydrops, places infants at high risk, with survival rates of 22% to 45%.1,2,3 Survival for patients with hydrops depends on many factors, including the underlying disease, gestational age at delivery, and available fetal therapies.1 Among cases of hydrops fetalis, about 20% are idiopathic.4 Here we describe the successful treatment of a male infant with idiopathic nonimmune hydrops fetalis who was delivered at a little over 37-weeks gestation. The infant also presented with congenital chylothorax, placing him into an extremely high-risk category.

Fig1-Xray-infant-hydrops at-birth-350x350
— Figure 1

Patient

During a routine obstetrical ultrasound, hydrops fetalis was identified in the male fetus at 37 weeks gestation. The mother of the patient was referred to the University of Minnesota Maternal Fetal Medicine Center for management. After consultation with the team, physicians arranged transfer of the mother to the University of Minnesota Medical Center with delivery occurring via cesarean section at the Birthplace soon after her admission. Upon delivery at 37 weeks 3 days gestation, the patient weighed 4.9 kg. He experienced significant respiratory distress at birth (Figure 1) and was immediately admitted to the level IV neonatal intensive care unit (NICU) at University of Minnesota Masonic Children’s Hospital.

Fig-2-Xray-while-on-ECMO-350x350
— Figure 2

Management

The infant did not respond to highfrequency ventilation and inhaled nitric oxide therapy for severe hypoxemic respiratory failure with pulmonary hypertension. As a result, he was placed on extracorporeal membrane oxygenation (ECMO) for 7 days (Figure 2).

A full work-up, including a genetic evaluation, was performed to identify the etiology of the hydrops fetalis, but no causal factor was found besides the presence of congenital chylothorax, which was treated with intravenous octreotide.

ECMO was successfully discontinued after 7 days. Over the course of 3 months, the chylothorax and need for ventilation resolved (Figure 3), and the patient was extubated and discharged to home. A follow-up examination at 16 months of age revealed that the boy was developing within normal ranges for motor, social, and language skills.

Fig3-Xray-post-treatment-350x350
— Figure 3

Discussion

In a 1997 key trial of ECMO in infants with idiopathic nonimmune hydrops, survival rates increased to about 50% with the treatment. Early initiation of ECMO in the neonatal period was found to be critical.5 High-volume centers have since been identified as an important factor in improving survival rates.6,7 Early and aggressive treatment with ECMO at a high-volume center is of paramount importance in ensuring optimal outcomes for these patients.

References

1. Derderian SC, Jeanty C, Fleck SR, et al. The many faces of hydrops. J Pediatr Surg. 2015;50:50-54.

2. Turgal M, Ozyuncu O, Boyraz G, et al. Nonimmune hydrops fetalis as a diagnostic and survival problems: what do we tell the parents? J Perinat Med. 2014 Oct 8. doi: 10.1515/jpm-2014-0094.

3. Takci S, Gharibzadeh M, Yurdakok M, et al. Etiology and outcome of hydrops fetalis: report of 62 cases. Pediatr Neonatol. 2014;55:108-13.

4. Bellini C, Donarini G, Paladini D, et al. Etiology of non-immune hydrops fetalis: An update. Am J Med Genet A. 2015;167:1082-1088.

5. Bealer JF, Mantor PC, Wehling L, et al. Extracorporeal life support for nonimmune hydrops fetalis. J Pediatr Surg. 1997;32:1645-1647.

6. Freeman CL, Bennett TD, Casper TC, et al. Pediatric and neonatal extracorporeal membrane oxygenation: does center volume impact mortality? Crit Care Med. 2014;42:512-519.

7. Bokman CL, Tashiro J, Perez EA, et al. Determinants of survival and resource utilization for pediatric extracorporeal membrane oxygenation in the United States 1997-2009. J Pediatr Surg. 2015 Feb 19. doi: 10.1016/j. jpedsurg.2015.02.042.

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