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Persistent pulmonary hypertension complicating cystic adenomatoid malformation in neonates
J B Atkinson1, E G Ford, H Kitagawa
1Division of Pediatric Surgery, Children's Hospital of Los Angeles, University of Southern California School of Medicine 90027.
Insights
Neonates with congenital cystic adenomatoid malformations (CCAM) can develop persistent pulmonary hypertension (PPHN) after surgery. Extracorporeal membrane oxygen (ECMO) support is crucial for these newborns.
Area of Science:
- Neonatal surgery
- Pediatric pulmonology
- Critical care medicine
Background:
- Congenital cystic adenomatoid malformations (CCAM) can cause neonatal respiratory distress.
- Persistent pulmonary hypertension (PPHN) is a known complication in neonates with congenital diaphragmatic hernia (CDH).
- PPHN is not commonly associated with CCAM.
Observation:
- Three neonates with CCAM presented with respiratory distress and underwent lobectomy.
- Post-lobectomy, all three patients developed PPHN within days.
- Cardiac echocardiography confirmed PPHN, necessitating extracorporeal membrane oxygen (ECMO) support.
Findings:
- Patients required ECMO for 66.5 to 120 hours.
- All patients were successfully weaned from ECMO to conventional ventilation.
- The clinical course mimicked that of CDH patients undergoing surgery.
Implications:
- Surgical stress, hypoxia, and hypercarbia in CCAM patients may trigger PPHN.
- ECMO is an effective supportive therapy for PPHN secondary to CCAM.
- This highlights a potential PPHN risk in neonates with CCAM undergoing surgery.
Abstract:
Neonates with congenital diaphragmatic hernia (CDH) are known to be susceptible to stress-induced persistent pulmonary hypertension (PPHN). Congenital cystic adenomatoid malformations (CCAMs) may also present as respiratory distress in the newborn. Intubation and mechanical ventilation cause clinical deterioration because of air trapping within cystic spaces; these patients require prompt lobectomy. PPHN has not been commonly associated with CCAM. Three patients with CCAM were encountered who developed PPHN postlobectomy. Three newborns, 36 to 38 weeks' gestation, presented with respiratory distress. Two had diagnosis of thoracic tumors on fetal ultrasound (22 and 33 weeks). Chest x-ray at birth confirmed cystic intrathoracic tumors in all and they underwent immediate thoracotomy and lobectomy (1 right upper, 1 left lower, 1 left upper). The patients were stable for 4 hours to 5 days postoperatively and then developed findings consistent with PPHN by cardiac echocardiography and required extracorporeal membrane oxygen (ECMO) support. ECMO was required for 66.5 to 120 hours. Each patient was successfully weaned to conventional ventilatory support. The clinical course of these patients was similar to those with CDH who undergo immediate surgery. The stress of surgical intervention combined with hypoxia and hypercarbia stimulates a hyperactive pulmonary vasculature and the development of PPHN. ECMO provides an effective adjunct to support patients with PPHN on the basis of congenital cystic adenomatoid malformations.