Hippokratia 2014, 18(4):381
Chatziioannidis I1,Mouravas V2, Babatseva E1
12nd Neonatal Intensive Care Unit, 22nd Department of Pediatric Surgery, Aristotle University of Thessaloniki, General Hospital “Papageorgiou”, Thessaloniki, Greece
Key words: Congenital diaphragmatic hernia, survival rate, timing
Congenital diaphragmatic hernia (CDH) is a rare developmental defect, associated with a mortality rate of 30-40%. Correct timing of surgical repair, in order to improve survival rate, remains controversial. We report a series of nine newborns admitted to our neonatal intensive-care unitwith CDH over a 5 year period. Gestation age was 31-39 weeks and birth weight was 1700-3420 gr. All newborns were born by elective caesarean section. Eight neonates presented with left-sided CDH (Bochdalek type), while in one case CDH was right-sided. In 5/9 CDH was identified prenatally. There were no coexisting anomalies found in any newborn. According to the standardized reporting system suggested by Lally KP et al1 our patients were categorised as follows: 5 at stage B (1 of which died), 2 at stage III. Two of nine neonates were intubated during resuscitation and were placed on mechanical ventilation subsequently, while the remaining developed respiratory distress later, within the first 2 hours of life. Persistent pulmonary hypertension (PPHN) was evident in 4/9 (3/4 died). Surgical repair took place in the first 10-72 hours of life and after they were stabilized using high-frequency oscillation ventilation, combined with administration of inhaled nitric oxide (iNO) in 5 cases and conventional ventilation in 4 cases. Criteria for surgical repairwere: no signs of PPHN, hemodynamic stability, pre-ductal saturation of 85-95% at FiO2< 0.5, diuresis 2 ml/kg/h and lactate <3mmol/Lt2.
Three newborns died (2 were not operated and 1 was surgically repaired within 10 hrs of life) with a total survival rate of 66.7%. All 6 neonates (34-37 weeks) who were surgically repaired after the first 24 hours of life (mean 37.5 hours) survived. Primary repair was applied in 5 cases, while Gore-Tex patch repair was applied in one case. Ventilatory support duration was 84-648 hours and mean length of stay was 17.5 days.
These results support a late (>24hrs) versus early surgical repair (<24hrs) for CDH treatment in newborn infants3. Lung hypoplasia due to CDH malformation could lead to PPHN. Surgical repair deteriorates lung compliance; therefore a period of respiratory stabilization is essential for the hypoplastic lung to develop.
Treatment strategy for CDH still remains a challenge for multi-skilled teams involved. Delayed surgical correction of CDH, after cardiopulmonary stability, along with novel operative techniques, seems to improve survival than early intervention.
1. Lally KP, Lasky RE, Lally PA, Bagolan P, Davis CF, Frenckner BP, et al. Standardized reporting for congenital diaphragmatic hernia--an international consensus. J Pediatr Surg. 2013; 48: 2408-2415.
2. Reiss I, Schaibe T, van den Hout L, Capolupo I, Allekaert K, van Heisjst A, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium consensus. Neonatology. 2010; 98: 354-364.
3. Moyer V, Moya F, Tibboel R, Losty P, Naqaya M, Lally KP. Late versus early surgical correction for congenital diaphragmatic hernia in newborn infants. Cochrane Database Syst Rev. 2002; 3: CD001695.