ABSTRACT
Sirenomelia is a rare developmental malformation and is incompatible to life. The incidence of sirenomelia, as recorded in the literature, is estimated to be approximately between 1.5 and 4.2 per 1,00,000 births. Around 15% of sirenomelia cases are associated with twin pregnancy, most often in monozygotic cases with an incidence of 7%. In monozygotic twins, the risk of sirenomelia is nearly 100-150 times higher as compared to dizygotic twins or singleton pregnancies. Until now, only two cases of sirenomelia associated with hypoplastic left heart have been reported in the literature. Here, we present a monozygotic twin pregnancy, where one fetus was diagnosed with sirenomelia associated with hypoplastic left heart syndrome and the co-twin was absolutely healthy.
ABSTRACT
OBJECTIVE: Developing less invasive methods for early detection of retinopathy of prematurity (ROP) is vital to minimizing blindness in premature infants. Lofqvist and colleagues developed a computer-based ROP risk algorithm (WINROP) (https://winrop.com), which detects downtrends in postnatal weight gain that correlate with the development of sight-threatening ROP. The aim of this study is to investigate the sensitivity and specificity of the WINROP algorithm to detect vision-threatening ROP. METHODS: This is a retrospective chart review study between January 2008 and December 2013. This study was conducted in the neonatal intensive care unit in Children's Hospital at Health Sciences Centre, Winnipeg, Manitoba, Canada. The study included preterm infants, less than 32 weeks' gestation, who were admitted to the hospital during the study period. The included 215 infants were eligible for ROP screening and had sufficient data to be entered into the WINROP algorithm. Infants were screened by a paediatric ophthalmologist for retinopathy of prematurity. The body weight of infants was measured weekly and entered into the WINROP algorithm; the sensitivity and the specificity of the WINROP algorithm were assessed. RESULTS: The mean gestational age was 28.6 ± 1.8 weeks. The mean body weight was 1244 ± 294 g. The sensitivity of the WINROP algorithm to detect vision-threatening retinopathy of prematurity in our cohort was 90% (P=0.021) with a specificity of 60% (P=0.002). CONCLUSION: The WINROP algorithm lacks sufficient sensitivity to be used clinically in our population. The algorithm needs to be reassessed in contemporary populations.
ABSTRACT
OBJECTIVE: Hypocarbia during the first 12 h of life is associated with mortality and disability in neonatal hypoxic ischemic encephalopathy (HIE). Notable variation in arterial carbon dioxide tension (PaCO2) during the first 4 d of life is related to severe intraventricular hemorrhages in preterm infants. We examined the association between PaCO2 during 72 h of whole-body therapeutic hypothermia for neonatal HIE and 2-year neurodevelopmental outcomes. METHODS: A retrospective review of 23 term neonates treated with whole-body hypothermia documented clinical, demographic and arterial blood gas data. Comparisons were made across good and severe neurodevelopmental outcome groups at 2 years of age. RESULTS: Severe neurodevelopmental outcomes were documented in 8 of 23 toddlers. There were no significant differences between outcome groups with regard to the number of patients with hypocarbic means or measurements. There were also no significant differences with mean PaCO2, PaO2, pH, time-weighted cumulative hypocarbia, and PaCO2 range. The severe neurodevelopmental outcomes group had a significantly higher mean PaCO2 standard deviation (p = 0.04; 95% CI, -5.46 to -0.39). CONCLUSION: Severe neurodevelopmental outcomes were significantly associated with high PaCO2 variability over 72 h in whole-body-cooled HIE neonates. Mitigating these fluctuations may be a potential management strategy.
