Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival.

PURPOSE: We describe the prevalence, associated anomalies, prenatal diagnosis and survival of patients with bladder exstrophy-epispadias complex. MATERIALS AND METHODS: Data were extracted from the Northern Congenital Abnormality Survey for patients delivered during 1985 to 2008. This survey collects data on congenital anomalies in fetuses, stillbirths and live-born infants of mothers residing in Northern England (Northumberland, North Cumbria, Tyne and Wear Durham, Darlington and Teesside). RESULTS: A total of 43 cases were identified from 824,368 registered births for a total prevalence of 5.22 per 100,000 (95% CI 3.77-7.03). Excluding 1 twin with cloacal exstrophy, 42 cases occurred in singleton pregnancies. A total of 29 cases (69%) were isolated and 13 (31%) were associated with other anomalies, of which 11 (26%) were other structural and 2 (5%) were chromosomal. Male-to-female ratio was 2.2:1 for all singleton cases and 1.4:1 for isolated cases. Total prevalence of bladder exstrophy-epispadias complex singleton cases was 5.10 per 100,000 registered births (95% CI 3.67-6.89) and overall live birth prevalence was 4.63 per 100,000 live births (95% CI 3.28-6.36). Total prevalence of isolated cases of bladder exstrophy-epispadias complex was 3.52 per 100,000 births (95% CI 2.36-5.05) and live birth prevalence was 3.29 per 100,000 (95% CI 2.17-4.79). Accuracy of prenatal diagnosis was low, with 4 cases (10%) being detected prenatally by routine ultrasound (bladder exstrophy in 3, cloacal exstrophy in 1). Overall survival of all infants at 1 year was 95%. CONCLUSIONS: This population based study demonstrates a prevalence rate similar to other studies, a low prenatal diagnosis rate and high survival.

Head trauma outcomes of verifiable falls in newborn babies.

Eleven newborn babies of normal weights sustained falls onto a hard surface in hospital. The one baby who fell from >1 m sustained clinical and radiological trauma and encephalopathy, with a skull fracture and cerebral contusion. No other baby demonstrated neurological signs despite the presence of parietal skull fractures in four of six who were x rayed; only two babies had scalp swelling. The findings suggest that parietal fractures can result from very low-level falls, and scalp swelling is a poor marker for underlying fracture.

Sudden Infant Death Syndrome and the time of death: factors associated with night-time and day-time deaths.

OBJECTIVE: To investigate the diurnal occurrence of Sudden Infant Death Syndrome (SIDS) and interaction with established risk factors in the infant sleeping environment. METHODS: A 3 year population-based case-control study, in five English Health Regions. Parentally defined day-time or night-time deaths of 325 SIDS infants and reference sleep of 1300 age-matched controls. RESULTS: The majority of SIDS deaths (83%) occurred during night-time sleep, although this was often after midnight and at least four SIDS deaths occurred during every hour of the day. The length of time from last observed alive until the discovery of death ranged from

Sudden infant death syndrome and sleeping position in pre-term and low birth weight infants: an opportunity for targeted intervention.

AIMS: To determine the combined effects of sudden infant death syndrome (SIDS) risk factors in the sleeping environment for infants who were "small at birth" (pre-term (<37 weeks), low birth weight (<2500 g), or both). METHODS: A three year population based, case-control study in five former health regions in England (population 17.7 million) with 325 cases and 1300 controls. Parental interviews were carried out after each death and reference sleep of age matched controls. RESULTS: Of the SIDS infants, 26% were "small at birth" compared to 8% of the controls. The most common sleeping position was supine, for both controls (69%) and those SIDS infants (48%) born at term or > or =2500 g, but for "small at birth" SIDS infants the commonest sleeping position was side (48%). The combined effect of the risk associated with being "small at birth" and factors in the infant sleeping environment remained multiplicative despite controlling for possible confounding in the multivariate model. This effect was more than multiplicative for those infants placed to sleep on their side or who shared the bed with parents who habitually smoked, while for those "small at birth" SIDS who slept in a room separate from the parents, the large combined effect showed evidence of a significant interaction. No excess risk was identified from bed sharing with non-smoking parents for infants born at term or birth weight > or =2500 g. CONCLUSION: The combined effects of SIDS risk factors in the sleeping environment and being pre-term or low birth weight generate high risks for these infants. Their longer postnatal stay allows an opportunity to target parents and staff with risk reduction messages.