Congenital anomalies are commonly associated with exomphalos minor

Aim

The objective of this study is to define the incidence of chromosomal and congenital anomalies in neonates with exomphalos major and minor.

Background

Incidence of major congenital anomalies varies from 35% to 81% in exomphalos. It is unclear whether these malformations are more common with exomphalos major.

Material and Methods

The case notes of 82 antenatal diagnoses of exomphalos, made between January 1998 and December 2004, were retrospectively reviewed. Exomphalos major was defined as a defect 5 cm or greater and exomphalos minor a defect less than 5 cm in diameter.

Results

There were 72 live births, 6 still births, and 4 terminations of pregnancy. There was no statistical significance between exomphalos major and minor regarding mode of delivery, gestational age at birth, birth weight, major cardiac anomalies (21% vs 23%), and renal and external genitalia abnormalities (11% vs 18%). Chromosomal anomalies, syndromes, and dysmorphism were common in exomphalos minor 17 (39%, P = .0001). Congenital malformations of the gastrointestinal tract (14% vs 27%), central nervous system (0 vs 21%), and Wilms' tumor (0 vs 5%) occurred commonly in exomphalos minor. Limb abnormalities (25% vs 5%), ectopia cordis (11% vs 0), and bladder exstrophy (7% vs 0) occurred predominantly in exomphalos major. Mean follow-up was 34 months. Three neonates with exomphalos major died. Overall mortality was 4%.

Conclusions

Chromosomal anomalies and syndromes occur more commonly in exomphalos minor. Exomphalos minor and major seem to have a predilection for associated anomalies of specific organ systems. This predisposition may help in counseling parents, planning investigations, and organization of multidisciplinary management strategy.     

Rising incidence of gastroschisis and exomphalos in New Zealand

Background

An apparent increase in the incidence of gastroschisis and exomphalos has been reported from several parts of the world. The exact mechanism of this trend is unknown. The aim of this study was to determine the regional and national trends in the incidence of gastroschisis and exomphalos in New Zealand.

Material and Methods

This retrospective multicenter study involved collection of data from all 4 tertiary care pediatric surgical centers in New Zealand. The incidence was calculated per 10,000 live births. Data were analyzed to determine the regional and national trends. The statistical analysis was done using linear regression model and Poisson distribution.

Results

The incidence of gastroschisis has increased from 2.96 per 10,000 live births to 5.16 per 10,000 live births between 1996 and 2004. During the same period, the incidence of exomphalos has increased from 0.69 per 10,000 live births to 3.27 per 10,000 live births. Gastroschisis was observed more in younger mothers, whereas exomphalos was associated with older mothers.

Conclusion

The incidence of gastroschisis and exomphalos is increasing in New Zealand, which is consistent with worldwide trends in showing the increasing incidence of anterior abdominal wall defects.     

Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain

Purpose

The aim of the study was to analyze the frequency and certain epidemiological characteristics of a consecutive series of conjoined twins born in Spain.

Material and Methods

We used data from the Spanish Collaborative Study of Congenital Malformations for the period April 1976 to 2006. Because the Spanish law permitting voluntary termination of pregnancies (TOP) when the fetus presented malformations was effective by the end of 1985, we analyzed the data in 4 periods, 2 before 1986 and 2 after. During the first period (1976-1979) only live births were recorded, whereas both still and live births were included in the other three (1980-1985, 1986-1995, and 1996-2006). In the present study, the cases were classified as symmetrical (16 pairs) and asymmetrical (1 pair) conjoined twins. Each pair of conjoined twins was considered as only one case for calculations, regardless of the type of union.

Results

Among a total of 2,281,604 consecutive births between 1980 and 2006, there were a total of 15 cases of symmetrical conjoined twins giving a frequency of 0.70 per 100,000 (1/152,107), whereas there was only 1 stillborn asymmetrical conjoined twin pair (0.04/100,000). Among the 13,418 consecutive stillborns surveyed, 6 cases of conjoined twins were identified (either symmetrical or asymmetrical) giving a frequency of 44.72 per 100,000, and 11 pairs were identified among the 2,425,583 total live births surveyed during the first period 1976 to 1979, a frequency of 0.45 per 100,000. Thus, the frequency among stillborn infants is 99.34 times higher than that observed among live births. However, the frequency for the total births (3 last periods) showed a decreasing trend from 1.47 per 100,000 birth in the first period (1980-1985) when TOP was illegal, to a value of 0.09 per 100,000 in the last period, more than 16-fold lower, probably because of the TOP of affected fetuses. Therefore, we consider that the frequencies observed in the period 1980 to 1985 are the basal values in our population. The most frequent type observed was thoracopagus, with an overall prevalence at birth of 0.44 per 100,000 (1/228,160) from 1980 to 2006, representing 58.82% of the total population of symmetric conjoined twin pairs. Diprosopus pairs were the next most common group (11.76%). Most of the cases were females (4 males/11 females), and although this appeared to be mainly because of the thoracopagus pairs (males-females, 2:8), in such a small number of cases, it is not possible to determine the ratios for the other groups. Gestational age was significantly shorter than in control twins for each type studied.

Conclusions

We conclude that it is incorrect to consider that all types of conjoined twins have the same epidemiological characteristics, such as the frequency at birth. The differences observed may be related with the distinct embryo-fetal mortality of each type of conjoined twins in different populations, and the sex ratio, among others.     

Definitive surgical management of antenatally diagnosed exomphalos

Background/Purpose

The management of exomphalos is controversial with many centers in the United Kingdom and elsewhere advocating a conservative nonsurgical approach for the larger examples. Nevertheless, this approach is not without problems or complication. The aim of the study was to ascertain the outcome of all infants with an antenatally diagnosed exomphalos treated recently at our institution using a policy of aggressive abdominal wall closure.

Methods

This is a retrospective review of all infants with exomphalos treated from January 1995 to September 2002.

Results

There were 35 infants, all of whom underwent surgery. These were separated into 3 groups: group A (all exomphalos minor) underwent primary closure (n = 11), group B (exomphalos major) underwent primary closure (n = 13), and group C (exomphalos major) underwent staged closure involving a silo (n = 11). Infants in group C had a lower birth weight (P = .05) and were less mature (P = .06). They required longer periods of ventilation (P < .001), a longer hospital stay (P = .001), and a longer period to achieve full enteral feeds (P < .001). Overall survival was 34 (97%) of 35 infants. One premature infant who was born with a ruptured exomphalos sac (birth weight, 862 g) died of nonsurgical complications (sepsis and respiratory failure) early after the creation of a silo.

Conclusions

An aggressive surgical approach in infants with exomphalos is a safe option resulting in effective abdominal wall closure. This requires a skilled multidisciplinary approach and possibly greater resources than other options.     

Omphalocele induction in the chick embryo by administration of cadmium

Background

Ventral body wall (VBW) defects occur in 1:2000 live births. We examined the association of VBW defect with somite abnormality and lordosis in the chick using in vitro and in ovo methods.

Methods

Explanted chick embryos were treated at 60 hours with 50 μL sodium acetate or 0.001% cadmium acetate solution to produce VBW defects. Mortality and abnormality rates were assessed. A further cohort of chicks was treated in ovo by dropping 50 μL 0.001% to 0.01% cadmium acetate onto the embryo and allowing development to 16.5 days for further assessment of the defect and skeletal staining with alcian blue and alizarin red.

Results

Cadmium treatment at 24 hours induced VBW defects in chicks treated in both shell-less culture and in ovo. Material herniating through the VBW defects was covered by a membrane in all fresh specimens. Membrane removal revealed large defects containing liver and bowel. These criteria clearly indicate that the defect observed is an omphalocele. Affected embryos had reduced somite numbers within 24 hours. Chicks exhibiting exomphalos at 16.5 days invariably had lumbosacral lordosis.

Conclusions

The cadmium-treated chick embryo is a reliable model for exomphalos. A positive association was found between exomphalos and lumbar lordosis in the chick.     

Long-term outcome of single institutional experience with conservative and surgical management for renal artery aneurysm

Background

Spontaneous rupture risk of a renal artery aneurysm (RAA) is extremely low. Indications for surgical repair of RAA remain uncertain.

Objective

Long-term outcomes of conservative therapy and surgical repair were evaluated.

Patients

The study included 58 patients (17 males, 41 females) who were diagnosed with RAA during the last 21 years. Median age at the time of diagnosis was 62 (19-85) years, and the median follow-up 69 months (range 3-216).

Methods

The patients were divided into two groups, conservative group (n = 30) who had been followed with blood pressure control, and treatment group (n = 29), who underwent an intervention.

Results

Multiple efferent aneurysmal branches were observed in seven conservative and 16 treatment cases (P = .002). The median maximum diameter of the aneurysm was lower in the conservative than the treatment group (15 versus 25 mm, P = .005). Two conservative group cases showed increases in aneurysm size during follow-up. The hypertensive state showed essentially no change in either group during the follow-up. Renal function decreased with age similarly both in conservative and treatment groups.

Conclusions

Our conservative management criteria for RAA are justifiable and even too strict.     

Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery

Background

We have previously reported the outcome of a cohort of cases over a 10-year period with antenatally suspected congenital cystic adenomatoid malformation (CCAM) and have recommended elective surgery within the first year of life for persistent, asymptomatic cases because of the risk of long-term complications. The aims of this study were to document optimal postnatal investigation and timing of surgery.

Methods

Cases were identified using the Oxford Congenital Anomaly Register, theatre records, and histopathology reports. Nineteen cases from 2003 onwards were reviewed with respect to postnatal imaging with chest x-ray and computed tomography scan and timing of surgery.

Results

Chest x-ray was poor at detecting CCAM with only 61% sensitivity. Computed tomography scan was 100% sensitive with no false-positive diagnoses.Of the 19 cases since 2003, 13 cases were asymptomatic and had elective surgery. Eight had surgery at 6 months of age or older, and of these, 4 (50%) had evidence of chronic inflammation/infection on histology including 1 case with multiple abscesses found at surgery and 1 case with multiple adhesions. Five cases had surgery at less than 6 months of age and only 1 of these, aged 5.5 months, had evidence of infection present at histology.

Conclusions

All cases of antenatally suspected CCAM should be followed up postnatally and investigated with computed tomography scan. Elective surgery in asymptomatic cases, at around 3 to 6 months of age, is recommended for persistent lesions because of the risk of long-term complications.     

Left-sided gallbladder with right-sided ligamentum teres hepatis: rare associated anomaly of exomphalos

Purpose

The aim of this investigation was to establish the association of left-sided gallbladder and right-sided ligamentum teres hepatis with exomphalos and to clarify the portal venous anomaly associated with right-sided ligamentum teres.

Methods

Three male infants with exomphalos major associated with left-sided gallbladder and right-sided ligamentum teres hepatis were identified during a retrospective study of exomphalos cases (n = 35) in which exomphalos major comprised 18 cases over 19 years.

Results

Three infants with exomphalos major (8.6%) had a left-sided gallbladder and right-sided ligamentum teres hepatis. One male infant survived. Computed tomography showed that there is anomalous intrahepatic portal venous branching associated with right-sided ligamentum teres hepatis. The first branch of the portal vein ran to the right posterior segment, and then the portal vein formed a trunk of the left portal vein and right anterior portal veins. The latter vein formed the umbilical segment of the portal vein and finally joined the ligamentum teres hepatis. The inferior vena cava was on the left side below the kidney and crossed to the right side at the level of the kidney.

Conclusions

Left-sided gallbladder with right-sided ligamentum teres hepatis may not be such a rare associated anomaly in infants with exomphalos. However, there are no reports describing this anomaly with exomphalos in the English medical literature. We believe this is the first report. As long-term survival after repair of exomphalos is increasing, perhaps some of the survivors may develop liver disease which requires liver resection later in life. Recognition of this anomalous relationship with exomphalos is clinically important because it is associated with abnormal intrahepatic portal venous branching and intraabdominal vascular anomalies.     

Postnatal spontaneous resolution of congenital cystic adenomatoid malformations

Purpose

Although antenatal resolution of congenital cystic adenomatoid malformations (CCAMs) is well documented, complete spontaneous postnatal resolution is rare, its existence even questioned by some.

Methods

All cases of antenatally diagnosed CCAMs over 7 years were retrospectively reviewed. Inclusion criteria were the following: (1) antenatal diagnosis of CCAM, (2) persistence on postnatal imaging, and (3) subsequent spontaneous resolution on postnatal imaging.

Results

Of 56 antenatally diagnosed CCAMs, 2 patients were identified. Both had macrocystic lesions. In case 1, the CCAM that filled the hemithorax on antenatal ultrasound was smaller on postnatal chest radiograph and disappeared by age 37 months on computed tomography. Case 2 had significant reduction of the CCAM at birth (persistence of the lesion on initial radiographs and ultrasound was documented). By 5 months, the lesion was not evident on computed tomography. Neither case was associated with symptoms, polyhydramnios, hydrops, or other abnormalities.

Conclusions

In patients with an antenatal diagnosis of CCAM, spontaneous resolution may occur postnatally in 4% of cases. Significant reduction in CCAM size may portend possible disappearance and therefore warrants an observational period before resection.     

Natural history and long-term follow-up of antenatally detected liver cysts

Background/Purpose

Antenatally detected liver cysts are rare; their diagnostic accuracy is unknown, and their management is controversial. This study assessed the natural history of these lesions.

Methods

We conducted a retrospective review of infants with isolated intrahepatic cysts that were detected antenatally. Data are expressed as median (range).

Results

Fifteen infants presented during the period 1991-2004 with an antenatally detected liver cyst. Their gestational age at detection was 22 (18-34) weeks, and the maximum diameter of their cyst was 23 (10-120) mm. Serial scans, which were performed in 9 fetuses, showed cyst enlargement in 5 cases, diminution in 1 case, and no change in 3 cases. In utero percutaneous aspiration was required in 1 infant. Three infants underwent postnatal surgery. One fetus (postfetal intervention) had a subtotal excision of a large subcapsular cyst filling the abdominal cavity on day 2. Another infant required partial excision and marsupialization (complex cyst arising from segment IV) at 5 months, and a third infant underwent a cyst cholecystostomy at 4 months. Postnatal investigations (including hepatic scintigraphy) suggested that the remaining lesions were either simple parenchymal (n = 10) or isolated intrahepatic choledochal (ie, type V; n = 2) cysts. The median follow-up for these patients was 44 (27-167) months. Serial postnatal ultrasonography showed cyst diminution in 4 cases, an enlargement in 1 case, and no dimensional change in 7 cases.

Conclusions

Most antenatally detected liver cysts appear to be simple and of parenchymal origin and do not require fetal intervention. Their postnatal history is variable, but regression without treatment is seen in most cases.     

Outcome of antenatally diagnosed sacrococcygeal teratomas: single-center experience (1993-2004)

Aims

Sacrococcygeal teratomas (SCTs) are the commonest neonatal tumors with an incidence of approximately 1:30,000. There are few large single-center series and even fewer describing both their antenatal and postnatal course. We report the outcome of all fetuses investigated at a tertiary fetal medicine center with this diagnosis.

Method

Demographic details were obtained from a prospectively maintained database. Patient records were examined for additional data including antenatal and postnatal interventions. Data were described as median (range).

Results

Forty-one SCTs were diagnosed antenatally during the period 1993 to 2004. Twelve were excluded from subsequent analysis (single antenatal visit or attending for second opinion [n = 6] and termination of pregnancy [n = 6]). Twelve underwent fetal intervention (laser vessel ablation [n = 4], alcohol sclerosis [n = 3], cyst drainage [n = 2], amniodrainage [n = 2], vesicoamniotic shunt [n = 1]) for fetal hydrops and polyhydramnios to aid in delivery and to prevent obstructive uropathy developing in the fetus. Of these, 3 died in utero and 9 survived to be born (median gestational age, 33 weeks [27-37 weeks]). A further 3 died in the neonatal period. There are 6 long-term survivors (50%) from this group. Seventeen infants, without intervention, were born at median gestational age 38 weeks (26-40 weeks). One infant with severe cardiac anomalies died on the day of birth. All surviving infants had definitive excisional surgery at a median of 2 days (1-16 days). Current median follow-up of survivors is 39 months (8-86 months). There have been no recurrences. One child has mild constipation, and 3 are awaiting cosmetic revision of their scars.

Conclusions

The overall survival of antenatally diagnosed SCT is approximately 77%, with the development of hydrops and others requiring in utero intervention carrying a poor prognosis. Otherwise, the outcome after surgical excision is excellent.     

Diaphragmatic repair through fetal tissue engineering: a comparison between mesenchymal amniocyte- and myoblast-based constructs

Purpose

We have previously shown that fetal tissue engineering is a preferred alternative to diaphragmatic repair in a large animal model. This study was aimed at comparing diaphragmatic constructs seeded with mesenchymal amniocytes and fetal myoblasts in this model.

Methods

Neonatal lambs (n = 14) underwent repair of an experimental diaphragmatic defect with identical scaffolds, either seeded with labeled autologous cells (mesenchymal amniocytes in group 1 and fetal myoblasts in group 2) or as an acellular graft (group 3). At 1 to 12 months postoperatively, implants were harvested for multiple analyses.

Results

Repair failure (reherniation or eventration) was significantly higher in group 3 than in groups 1 and 2, with no difference between groups 1 and 2. Seeded fetal myoblasts quickly lost their myogenic phenotype in vivo. All grafts contained cells with a fibroblastic-myofibroblastic profile. Elastin concentrations and both modular and ultimate tensile strengths were significantly higher in group 1 than in groups 2 and 3. There were no differences in glycosaminoglycans and type I collagen levels among the groups.

Conclusions

Diaphragmatic repair with a mesenchymal amniocyte-based engineered tendon leads to improved structural outcomes when compared with equivalent fetal myoblast-based and acellular grafts. The amniotic fluid is a preferred cell source for tissue-engineered diaphragmatic reconstruction.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Background

The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.

Method

We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.

Results

Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.

Conclusion

Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.     

Favorable Outcome of Orthotopic Liver Transplantation in Very High-Risk Situations

Background

Favorable outcomes of marginal liver grafts depends on optimal perioperative control and good recipient parameters. The aim of this study was to assess the results of marginal liver grafts associated with prolonged ischemic times and high-risk recipients.

Methods

We retrospectively reviewed data from patients who underwent orthotopic liver transplantation between 2001 and 2005. The patients were divided into two groups: group 1 received marginal liver grafts with ischemia times ≥ 12 hours and recipient United Network for Organ Sharing (UNOS) status 1, 2A, and 2B. Patients who had marginal liver grafts with ischemic times less than 12 hours and/or better UNOS status were classified as group 2. We compared initial graft function as well as patient and graft survivals at 1 year between the two groups.

Results

Among 31 patients who were reviewed, four were excluded because of incomplete data and 24/27 received marginal liver grafts. Seven patients were classified into group 1, and 17 into group 2. The initial poor function rate was 85.7% (6/7 patients) and 76.47% (13/17 patients) in groups 1 and 2, respectively. The 1-year survival rate in group 1 was 85.7% (6/7 patients) and 94.12% (16/17 patients) in group 2.

Conclusion

Marginal liver grafts can be used with favorable outcomes even in high-risk situations, such as prolonged ischemia times and high-risk recipients.     

Increasing prevalence of gastroschisis in Utah

Background

Recent studies provide conflicting information about gastroschisis prevalence trends. The authors proposed that prevalence of gastroschisis in live births has increased in Utah and that characteristics of these infants would provide clinically useful information about treatment and outcomes.

Methods

Primary Children's Medical Center (PCMC) is the sole pediatric surgical referral hospital for Utah. The authors used both pediatric surgical and neonatal databases to identify gastroschisis cases at PCMC from 1971 through 2002. Only infants whose mothers had a primary residence in Utah were included. Individual charts were reviewed for infant characteristics for cases from 1998 through 2002. Utah Vital Statistics Reports were used to determine live birth rates and general infant and maternal characteristics.

Results

Gastroschisis prevalence increased from 0.36 to 3.92 cases per 10,000 live births over 31 years (P < .001). Young maternal age, primigravida status, and tobacco use were associated risk factors. Using the time required to achieve full enteric feedings at targeted volume and caloric density as a measurement of outcome, we found no association between delivery mode or surgical closure type (primary or secondary) and time to full feedings. Higher birth weight was associated with decreased time to full feedings (P = .03).

Conclusions

Gastroschisis prevalence has increased 10-fold over the past 3 decades in Utah.     

Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years

Purpose

Biliary atresia (BA) is the leading cause of obstructive jaundice in the newborn and the major indication for liver transplantation in Japan. Viral infection has been implicated in its etiology because of seasonality and space clustering. However, this has been controversial among whites. The present study investigates space-time clustering of the incidence of BA in Japan.

Methods

Birth prevalence rates of BA were analyzed in infants born in Hyogo prefecture between 1985 and 2004 to assess time clustering among 3 seasons. The birth prevalence rates were also analyzed for space clustering between the northern (Hokkaido) and the southern islands (Kyushu) (difference of latitude, 10°) based on the Japanese Biliary Atresia Society Survey 1996-2004. We compared the prevalence rates between these groups using relative risks (RRs) calculated from 2 × 2 contingency tables.

Results

One hundred nineteen infants were born with BA in Hyogo (1.1/10 000 live births). Seasonal clustering in April to July did not significantly differ from that of the reference period (RR, 1.45; 95% confidence interval, 0.93-2.27). Based on Japanese registry data, space clustering between the northern and southern islands did not significantly differ (RR, 1.24; 95% confidence interval, 0.83-1.86).

Conclusions

Neither seasonal nor spatial clustering was statistically proven in Japan.     

Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia

Background

Congenital diaphragmatic hernia (CDH) is associated with significant neonatal morbidity and mortality. Although prenatal complete tracheal occlusion (cTO) causes hypoplastic CDH lungs to enlarge, improved lung function has not been demonstrated. Furthermore, cTO interferes with the dynamic pressure change and fluid flow associated with fetal breathing.

Purpose

The purpose of the study was to assess a novel dynamic tracheal occlusion (dTO) device that preserves pressure changes and fluid flow.

Methods

In this pilot study, CDH was created in fetal lambs at 65 days of gestational age (GA). At 110 days GA, a cTO device (n = 3) or a dTO device (n = 4) was placed in the fetal trachea. At 135 days GA, lambs were delivered and resuscitated. Unoperated lamb co-twins (n = 5), sham thoracotomy lambs (n = 2), and untreated CDH lambs (n = 3) served as controls.

Results

Tracheal opening pressure, lung volume, lung fluid total protein, and phospholipid were significantly higher in the cTO group than in the dTO and unoperated control groups. Maximal oxygenation and lung compliance were significantly lower in the cTO group when compared with the unoperated control and dTO groups.

Conclusion

Preliminary results suggest that in the fetal lamb CDH model, dTO restores normal lung morphometrics and function, whereas cTO leads to enlarged but less functional lungs.     

Intravenous landiolol, a novel β(1)-adrenergic blocker, reduces the minimum alveolar concentration of sevoflurane in women

Study Objective

To investigate the effect of intravenous (IV) landiolol, a novel β₁-adrenergic blocker, on the minimum alveolar concentration (MAC) of sevoflurane in adult women.

Design

Prospective, randomized study.

Setting

University hospital.

Patients

42 ASA physical status 1 and 2 women, aged 24-57 years, who were scheduled to undergo elective abdominal surgery.

Interventions

Anesthesia was induced in all patients by vital capacity rapid inhalation induction of sevoflurane. In the landiolol group, administration of landiolol began when patients took a vital-capacity breath: 0.125 mg/kg/min for one minute and then 0.04 mg/kg/min. Normal saline was administered in the control group.

Measurements

MAC was determined by a technique adapted from the conventional up-down method.

Main Results

The MAC of sevoflurane was 2.2% ± 0.2% in the control group and 1.7% ± 0.2% in the landiolol group, a statistically significant difference (P = 0.0005).

Conclusions

IV landiolol reduces the MAC of sevoflurane in women by approximately 20%.     

Increasing prevalence of gastroschisis repairs in the United States: 1996-2003

Purpose

Local and state registries have shown recent increases in the prevalence of gastroschisis in the United States and abroad. The purpose of this study was to use a nationally representative database to identify national trends in the prevalence of gastroschisis repairs.

Methods

Records of infants undergoing gastroschisis repair were identified in the Nationwide Inpatient Sample from 1996 to 2003. Birth data were obtained from the National Center for Health Statistics and used to calculate the rate of procedures/live births stratified by US census region. Survey statistics were used to account for the sampling design of the Nationwide Inpatient Sample database.

Results

Between 1996 and 2003, a total of 9459 gastroschisis repairs were performed in the United States (3 procedures for every 10,000 births). A significant increase in the population-based rate of these procedures was observed in each census regions and nationwide. A twofold higher procedure rate was observed in 2003 than in 1996 (rate ratio, 2.0; 95% confidence interval, 1.1-2.9; P < .001). No significant change in unadjusted hospital mortality was observed regionally or nationally.

Conclusions

The population-based rate of gastroschisis repairs significantly increased regionally and nationwide between 1996 and 2003, paralleling recent trends described at the local and state level.     

A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003

Purpose

The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the χ² test.

Results

The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality (P < .001).

Conclusions

This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.