Pulmonary Malformations: Predictors of Neonatal Respiratory Distress and Early Surgery

Abstract

Objectives: The objective of our study is to retrospectively analyze a single-centre series of antenatally detected pulmonary malformations (PM) and to evaluate their postnatal outcome.

Materials and methods: We retrospectively reviewed all prenatally diagnosed PM patients referred to our Centre in the period between January 1999 and December 2014. All cases were diagnosed by one of our Maternal-Fetal Specialists by US examination. Congenital pulmonary airway malformation (CPAM) volume ratio (CVR), development of fetal complications, need for fetal therapy, need for neonatal resuscitation and timing of surgery were analyzed.

Results: A total of 70 fetuses were diagnosed with a PM in the period of study. An initial CVR higher than 1.6 was found in 16/70 patients (22.8%); 14/16 developed fetal complications (p less than .0001). Fifty-six fetuses (80%) did not develop any complications during pregnancy. To all complicated cases a prenatal treatment was offered, carried out in 12 (1 termination, 1 refusal). Survival rate was 100%. Sixty-three fetuses (90%) were asymptomatic at birth and did not require any neonatal resuscitation. Six patients submitted to fetal therapy and one untreated presented with neonatal respiratory distress, required mechanical ventilation at birth and early surgery in the neonatal period (7/70, 10%).

Conclusion: CVR > 1.6 and the presence of fetal complications can be considered as predictors of respiratory distress at birth and of the need for early surgery. Nevertheless, the vast majority of PM are asymptomatic at birth and only a small group of fetuses require prenatal and postnatal treatment and support.

Keywords: CPAM volume ratio; Fetal therapy; Neonatal resuscitation; Prenatal diagnosis; Pulmonary malformations.

Figure 1: Outcome of patients with prenatal complications.

Figure 2: Case prenatally treated with thoraco-amniotic shunt positioning. Fetal US imaging of hydrothorax (A); reduction of fetal hydrothorax after TAS positioning (B: US imaging and C: fetal MRI); CT-scan at birth (D).

Figure 3: Large left-sided complicated lesion not submitted to fetal therapy for parental refusal: fetal MRI (A), chest X-ray (B) and CT scan (C, D) at birth; the child needed mechanical ventilation at birth and required open surgery by the first 24 hours of life (E: postoperative chest X-Ray).

Table 1: Threshold values of CVR as reported in literature