Measured gap length and outcomes in oesophageal atresia

Aims

Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) is the most common congenital anomaly of the oesophagus. There is limited literature suggesting a linear relationship between increasing gap length and the incidence of all major complications. We sought to assess whether measured gap length at the time of surgery was related to outcomes in our patients.

Methods

All patients with a diagnosis of OA +/− TOF who underwent repair under a single surgeon between 1983 and 2012 were included. The length between the oesophageal pouches was measured at the time of surgery. Patients were then divided into three groups; short ≤ 1 cm, intermediate > 1–≤2 cm and long > 2–≤5 cm. Outcome measures were anastomotic leak, strictures requiring dilatation, gastrooesophageal reflux disease (GORD) and need for fundoplication.

Results

122 patients were included in the study. The outcomes for patients with short (n = 53), intermediate (n = 51) and long gaps (n = 18) were as follows: anastomotic leak — 1.9%, 2%, 5.5% (P = 0.66), strictures requiring dilatation — 32%, 33%, 50% (P = 0.67), GORD — 51%, 59%, 72% (P = 0.58) and need for fundoplication — 11%, 20%, 44% (*P = 0.02). There were no deaths related to the repair.

Conclusions

Measured gap length at the time of surgery did not have a linear relationship with leak or stricture rate. Our experience suggests that when primary repair is possible absolute gap length is irrelevant to the development of post-operative complications. There is however a significant increase in the need for fundoplication in those with a long gap.     

Triplets and higher-order multiple births. Time trends and infant mortality.

OBJECTIVES--To describe changes in rates of higher-order multiple births (triplets and higher) between 1972 and 1989, to compare infant mortality rates in infants of higher-order multiple births and singletons born from 1983 through 1985, and to compare infant mortality rates among higher-order multiples born from 1983 through 1985 with rates among those born in 1960. RESEARCH DESIGN--Population-based analysis of live births (1972 through 1989) and infant deaths (1960 and 1983 through 1985) in the United States. The rate of higher-order multiple births was calculated per 100,000 live births. DATA SOURCE--Computerized national natality files for 1972 through 1989 and national linked birth/infant death data sets for 1960 and 1983 through 1985 from the National Center for Health Statistics, Centers for Disease Control. POPULATION--Live births to white and black women in the United States. INTERVENTIONS--None. MAIN RESULTS--Between 1972 through 1974 and 1985 through 1989 the rate of higher-order multiple births increased by 113% among infants of white mothers and by 22% among infants of black mothers. In whites the increase was mostly age specific and was not due to the upward shift in the maternal age distribution. The increase was particularly large in white women aged 30 through 34 years (152%) and 35 through 39 years (165%) and in more highly educated mothers. In blacks the modest increase in the rate of higher-order multiple births was mostly due to an upward shift in the maternal age distribution. From 1983 through 1985, mortality of infants of higher-order multiple births was about 15 times that of singletons. This was due almost entirely to the lower birth weight distribution of infants of higher-order multiple births. Their weight-specific mortality compared favorably with that of singletons. At 500 through 999 g, mortality was about the same. In weight categories between 1000 and 1999 g, mortality rates in higher-order multiple births were much lower: weight-specific relative risks ranged from 0.30 to 0.73. Between 1960 and 1983 through 1985 infant mortality in higher-order multiple births declined by about 50%. CONCLUSIONS--It is likely that much of the increase in the incidence of higher-order multiple births is due to the rise in the use of ovulation-inducing drugs for the treatment of infertility. This increase and the decline in mortality risk have created a much greater need for medical and social services for infants of higher-order multiple births and their families.     

Double jeopardy: twin infant mortality in the United States, 1983 and 1984

The United States Linked Birth/Infant Death Data Sets: 1983 and 1984 Birth Cohorts from the National Center for Health Statistics were used to identify maternal and infant characteristics related to twin infant mortality; 41,554 white and 10,062 black live-born matched twin pairs were evaluated. Twin birth weight distribution was skewed with 48% of white and 63% of black twins born weighing less than 2500 gm. Overall infant mortality rates were 47.1 and 79.3 deaths per 1000 live births for white and black twins, respectively (five times the rates for singletons). Three fourths of deaths were among twins weighing less than 1500 gm. White like-gender twins had about twice the risk of both twins dying compared with unlike-gender twins. Likewise, white twin pairs with greater than 25% birth weight disparity had a 40% to 80% increased risk of both twins dying, compared with twins whose weights were within 10% of each other. Twins born to high-risk women (on the basis of demographic factors) were twice as likely to die as twins born to low-risk women. Thus strategies to decrease twin infant mortality must address both maternal and infant risk factors.     

Oesophageal atresia and associated anomalies.

Of 253 infants with oesophageal atresia treated over an eight year period, 122 (48%) had a total of 213 other anomalies. Most commonly affected were the cardiovascular (61 cases, 29%), anorectal (30 cases, 14%), and genitourinary (29 cases, 14%) systems. The VATER (or VACTERL) association was present in 10% of cases, but occurred more often in patients who had oesophageal atresia without an associated tracheo-oesophageal fistula (3/13, 23%). The level of the associated anorectal malformation was not associated with the type of oesophageal atresia. The presence and severity of other anomalies did not influence the basic approach to treatment of the oesophageal atresia--that is, primary repair whenever possible. Despite aggressive treatment, cardiac malformations were the most common cause of death. There were five infants with the CHARGE association, two with Potter''s syndrome, and two with ''SCHISIS'' syndrome (cleft lip and palate, omphalocoele, and hypogenitalism).     

Late-onset central hypoventilation syndrome: a family genetic study.

Congenital central hypoventilation syndrome is a rare disorder characterised by chronic alveolar hypoventilation, which becomes more pronounced during sleep and may be associated with neurocristopathies, such as Hirchsprung's disease. A mutation in the PHOX2B gene has recently been identified. In a family of both parents and five offspring, detailed clinical assessment, pulmonary function testing, overnight sleep studies and ventilatory responsiveness to progressive hypercapnia (V'(R,CO(2))) were performed, in addition to analysis of known genetic loci for this condition. The father and four of the offspring demonstrated features of central hypoventilation with nonapnoeic oxygen desaturation during sleep and diminished V'(R,CO(2)), despite normal pulmonary function. The lowest sleep saturation was median (range) 79% (67-83%) and V'(R,CO(2)) was 2.1 (0.03-4.3) L x min(-1) x kPa(-1). The normal values for the authors' centre (St Vincent's University Hospital, Dublin, Ireland) are 15-40 L x min(-1) x kPa(-1). An in-frame five amino acid polyalanine expansion of the PHOX2B gene was found in all affected subjects, while the mother and fifth child, who did not have features of central hypoventilation, had a normal PHOX2B gene. Magnetic resonance imaging of the brainstem in one severely affected child was normal. The present study of a unique family confirms that transmission of late-onset congenital central hypoventilation syndrome is autosomal dominant in nature.     

Changes in ocular accommodation when shifting between global and local attention

Background : The link between cognitive changes in attentional focus and the physiological parameters of the eye is not well understood. The aim of the current work was to examine the role of ocular accommodation, that is, the process of changing the shape of the crystalline lens in order to focus an image onto the retina, in active shifts of visual attention between global and local information. Method : Ten adult participants (aged 19 to 27) viewed sequences of complex global/local figures presented at the same location. They were asked to identify either a global or local red target letter and to look for either a global or local letter x (probe) in the sequence following. Target and probe items were separated by a temporal gap of approximately one second. Refraction was measured using a Canon Autorefractor Rl at the time of target appearance and again at the time of probe presentation. Particular interest was paid to trials where participants were required to shift attention either from a local to global level or in the reverse direction. The difference between the two measurements of refraction gave a ‘change score’ which provided an indication of the change of accommodation. Results : No significant change in refraction was observed when shifting from the local to the global condition. A mean change in refraction of ?0.128 dioptres was observed for the global to local condition. The 95 per cent confidence interval for this difference did not overlap zero, indicating a significant change in refraction, which was attributed to an increase in accommodation. Discussion : The results suggest that while a change in accommodation occurs in shifting attention from the global to the local aspect of the complex figures, a similar change is not observed in the reverse direction. These results are consistent with the hypothesis that it is more difficult to restrict attentional focus than it is to widen it.