Congenital diaphragmatic hernia with pneumothorax, a challenge for the neonatologist on call

Abstract

Objectives. Congenital diaphragmatic hernia (CDH) represents a developmental defect of the diaphragm, which allows the protrusion of the abdominal viscera into the thoracic cavity. In view of the fact that herniation occurs during a critical period of lung development, the pathological effect is pulmonary hypoplasia in different degrees, usually more severe on the ipsilateral side of the hernia, and also may be present contralateral if the mediastinum is bulged, compressing the lung. Material and methods. We present the case of a patient hospitalized in the Neonatology Department of “St. Andrew” County Emergency Clinical Hospital of Constanta, diagnosed with left CDH at birth. Outcomes. Full-term male newborn, gestational age (GA) 39 weeks, birth weight (BW) 3300g, Apgar Score 6. The 35-year-old mother, Gravida-VI, Para-III, has a pregnancy with inadequate prenatal care, with no structural abnormalities of the fetus detected by the 3rd-semester ultrasound. At birth, the newborn needed neonatal resuscitation, initially with positive pressure ventilation (on mask and bag) and after that, intubated and mechanical ventilated. Chest X-Ray showed a left diaphragmatic hernia, and the pediatric surgery team was called for further therapeutic management. Approximately 48 hours postoperatively, the chest X-Ray identified right upper lobe pneumothorax, with complete remission in 24 hours, under mechanical ventilation. Conclusions. CDH represents a condition with a challenging diagnosis and management. In the best cases, newborns have a very good clinical outcome with neonatal care and surgical treatment after birth. The management of infants with congenital diaphragmatic hernia requires the services of an interprofessional team. After the diagnosis in the antenatal period, parents should be allowed to discuss with a team, including maternal-fetal medicine, pediatric surgery, neonatology, and social work as appropriate. Genetic evaluation and counseling are recommended to identify risks in future pregnancies. Following the repair in the postnatal period, a standardized and interdisciplinary follow-up to provide surveillance, screening, and clinical care is recommended to improve outcomes.

Keywords

• congenital diaphragmatic hernia
• pulmonary hypoplasia
• pneumothorax