Pierre Robin sequence: Management of respiratory and feeding complications during the first year of life in a tertiary referral centre.

OBJECTIVES: To review early clinical manifestations of Pierre Robin sequence (PRS) and their management during the first year of life in the University Hospitals Leuven. METHODS: Retrospective series of 48 patients with PRS born between 2001 and 2011 and treated at a tertiary referral hospital. Review of the current literature about management of respiratory and breathing difficulties in the early life of PRS patients. RESULTS: Of our cleft palate patients 15.3% presented with PRS. A syndrome was diagnosed in 14.6%, associated anomalies without a syndromic diagnosis in 56.3% and isolated PRS in 29.2% of the cases. Mortality rate directly related to PRS was 2.1%. Respiratory difficulties were observed in 83.3% and feeding difficulties in 95.6% of the patients. Respiratory problems were addressed in a conservative way in 75%, in a non-surgical invasive way in 42.5% and in a surgical way in 12.5%. A statistically significant relationship between the association of a syndrome or other anomalies, and a higher need for resuscitation and invasive treatment were found (chi-square test, p-values=0.019 and 0.034). Feeding difficulties were managed conservatively in 91.3%, invasively in 80.4% and surgically in 15.2%. CONCLUSIONS: PRS is frequently associated with other abnormalities or syndromes. Therefore routine screening for associated anomalies in neonates with PRS is recommendable. Respiratory and feeding complications are highly frequent and possibly severe, particularly in patients with associated anomalies or syndromes, and should be recognized and addressed appropriately in an early stage. There is a potential role for the nasopharyngeal airway in reducing the need for the more traditional surgical interventions for respiratory problems.

Conventional gas ventilation, liquid-assisted high-frequency oscillatory ventilation, and tidal liquid ventilation in surfactant-treated preterm lambs.

This study was designed to compare the efficacy and potential protective or injurious effects of tidal liquid ventilation (TLV), liquid-assisted high-frequency oscillatory ventilation (LA-HFOV), and high PEEP conventional mechanical ventilation (CMV) in neonatal respiratory distress syndrome. Preterm lambs (124-126 days gestation), prophylactically treated with natural surfactant, were allocated to one of the treatment modalities or to an untreated fetal control group (F), euthanised after tracheal ligation. LA-HFOV animals received an intratracheal loading dose of 5 mL x kg(-1) followed by a continuous intrapulmonary instillation of 12 mL x kg(-1);h(-1) FC-75 perfluorocarbon liquid. The ventilation strategies aimed at keeping clinically appropriate arterial blood gases for a study period of 5 hours. A histological lung injury score was calculated and semiquantitative morphometry was performed on lung tissue fixed by vascular perfusion. The alveolar-arterial pressure difference for O2 was significantly lower throughout the study in TLV compared to CMV lambs; at 1, 2, and 5 hours, oxygenation was better in TLV when compared to LA-HFOV. Total lung injury scores in TLV lambs were significantly lower than in either CMV or LA-HFOV animals, but higher when compared to F. CMV and LA-HFOV induced an excess of collapsed and overdistended alveoli, whereas in TLV alveolar expansion was normally distributed around predominantly normal alveoli. CMV and LA-HFOV, but not TLV, were associated with an excess of dilated airways. Thus, in the ovine neonatal RDS model, TLV compared favourably to either gas ventilation strategy by its more uniform ventilation, reduced lung injury, and improved gas exchange.