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.     

Current outcome of antenatally diagnosed exomphalos: an 11 year review

Purpose

The aim of this study was to determine the outcome of antenatally diagnosed exomphalos.

Methods

The database of a tertiary referral Fetal Medicine Centre was searched for all cases of antenatally diagnosed exomphalos between January 1991 and December 2002. Patients, general practitioners, and hospitals were contacted for outcome details.

Results

In total, 445 cases of exomphalos were identified. In 250 (56%) cases, the fetal karyotype was abnormal (group A), in 135 (30%) cases, the karyotype was normal (group B), and in 60 (14%) cases, karyotyping was declined (group C). In group A, there were 248 (99%) terminations of pregnancy (TOP) or fetal deaths and 2 live births. In group B, 74 (54%) fetuses had other structural anomalies; 82 (61%) pregnancies resulted in TOP or fetal death, 42 (31%) in live births, and 11 (8%) were lost to follow-up. In group C, 38 (63%) fetuses had other structural anomalies; 41 (69%) pregnancies resulted in TOP or fetal death, 11 (18%) in live births, and 8 (13%) were lost to follow-up. Of the 55 live births, 11 died preoperatively and 44 had surgery. There were no postoperative deaths.

Conclusions

Less than 10% of the antenatal diagnostic workload reached operative repair. In our unit, these babies are a highly selected group, which is a factor in the high postoperative survival.     

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.     

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.     

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.     

Management of complicated gastroschisis with porcine small intestinal submucosa and negative pressure wound therapy

Introduction

In almost all cases of gastroschisis, fascial closure may be achieved primarily or after silo reduction. Rarely, fascial and skin closure are impossible. We report our experience with visceral coverage in complicated cases of gastroschisis with porcine small intestinal submucosa (SIS) augmented by negative pressure wound therapy (NPWT).

Methods

Over a 3-year period, 55 infants with gastroschisis were managed. In 3 of these cases, fascia and skin could not be approximated safely after complete reduction of abdominal viscera with a spring-loaded silo. Visceral coverage in each case was achieved with 0.42-mm-thickness Surgisis ES (Cook Surgical, Bloomington, Ind) that was sewn to the fascial edges. Negative pressure wound therapy was then initiated at 75 mm Hg over the exposed SIS using vacuum-assisted closure.

Results

In each case, granulation tissue developed quickly and was followed by complete epithelialization. Two patients subsequently developed umbilical hernias.

Conclusion

We have successfully used SIS augmented by NPWT in the management of 3 infants with complicated gastroschisis. In the rare situation in which fascial closure cannot be achieved, the combination of SIS and NPWT can provide a safe and effective means of abdominal wall closure.     

Pathogenic implications of remnant vitelline structures in gastroschisis

Purpose

The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis.

Methods

Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed.

Results

Paraumbilical bands, possible remnants of vitelline structures, were observed in 4 cases (9.8%). All 4 bands were attached to the skin edge of the abdominal defect without incorporation into the umbilical cord. The band ended at the mesentery in 3 cases and at the antimesenteric site of the ileum in the remaining case. Histologic findings showed fibrous tissues in all cases. One was possibly associated with the development of colonic atresia. Another was noticed after silo reduction when herniated bowels became strangulated by the band. The other 2 cases were uncomplicated.

Conclusions

Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally.     

Liver herniation in gastroschisis: incidence and prognosis

Purpose

Liver herniation is a rare occurrence in gastroschisis. We sought to determine the incidence and prognosis of liver herniation in patients with gastroschisis.

Methods

From December 1995 to March 2010, 117 patients with gastroschisis received care by our division. Operative reports were reviewed to identify patients with liver herniation. Logistic regression was used to determine the impact of liver herniation on survival, taking into account gestational age and birth weight.

Results

The incidence of liver herniation was 6%. Survival rates were 43% with liver herniation and 97% without liver herniation. Liver herniation was associated with a significantly higher rate of mortality, taking into account estimated gestational age and birth weight (P < .001). Patients who had liver herniation documented by prenatal ultrasound had significant liver herniation at birth and died postnatally. Patients with liver herniation who died required large silos and were noted to have comorbidities including lower birth weight, pulmonary hypoplasia, and sepsis. Biologic patches were necessary for closure in patients with greater extent of liver herniation.

Conclusions

Liver herniation was found in 6% of patients with gastroschisis and was associated with a high rate of mortality. Liver herniation appears to be a risk factor for poor outcome in gastroschisis. Documentation of liver herniation may be helpful in prenatal consultation for gastroschisis.     

Neonatal short bowel syndrome: population-based estimates of incidence and mortality rates

Background

Congenital or acquired neonatal short bowel syndrome (SBS) carries significant morbidity and mortality rates. No accurate population estimates of incidence and mortality exist because of differences in definition, follow-up, and regional referral patterns.

Methods

A retrospective cohort study was performed involving 175 surgical neonates admitted to our institution from January 1, 1997 to December 31, 1999 and followed up until July 1, 2001. Institution and population-based estimates of incidence and mortality were performed using postcensal population figures (1997) from Statistics Canada.

Results

The overall incidence of SBS was 22.1 per 1,000 neonatal intensive care unit (NICU) admissions (95% CI = 15.3, 28.9) and 24.5 per 100,000 live births (95% CI = 12.1, 36.9). The incidence was much greater in premature infants (less than 37 weeks). The SBS case fatality rate was 37.5% (95% CI = 22.5, 52.5) and the cause-specific and proportional mortality rates (for children less than 4 years old) were 2.0 of 100,000 population per year (0.4 to 3.6/100,000/year) and 1.4% (0.3% to 2.6%), respectively.

Conclusions

Patients with neonatal SBS pose a complex management challenge and are responsible for a significant cost to the health care system. To our knowledge, this study represents the first population-based estimates for neonatal SBS incidence and mortality rates. Accurate estimates will assist clinicians in counseling parents, allocating resources, and planning clinical trials.     

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.     

Mode of delivery and neonatal survival of infants with gastroschisis in Australia and New Zealand

Objective

The aim of the study was to examine the short-term outcome of infants with gastroschisis by route of delivery, comparing vaginal delivery vs elective and emergency cesarean delivery (CD).

Methods

Six hundred thirty-one infants with gastroschisis (International Classification of Diseases, 10th Revision: Q79.3) were admitted to the Australian and New Zealand Neonatal Network during 1997 to 2005. Multivariate Cox proportional hazards regression analysis was performed to adjust for case-mix and significant baseline characteristics.

Results

During the study period, 631 infants with gastroschisis were admitted to the collaborating centers. Of these, 343 (54.4%) infants were delivered vaginally, whereas 288 (45.6%) were delivered by cesarean birth. Of the latter, 148 (23.4%) were elective and 140 (22.2%) were emergency. There was an increasing trend of CD from 41.1% in 1997 to 69.0% in 2005.Forty-seven (7.4%) infants died; 30 (8.7%) in the vaginal, 9 (6.4%) in the emergency, and 8 (5.4%) in the elective CD group. There was no difference in rate of proven infection, duration of ventilation, or length of neonatal intensive care unit stay between the 3 groups.After controlling for prematurity, low birth weight, and outborn birth, the risk for neonatal demise was similar in both the vaginal and CD infants (adjusted hazard ratio, 1.486; 95% confidence interval, 0.814-2.713; P = .197). Stratifying the CD (emergency vs elective) gave similar results.

Conclusion

Infants with gastroschisis appear to be safely delivered vaginally.     

Gastroschisis: a national cohort study to describe contemporary surgical strategies and outcomes

Background

Information on adoption of newer surgical strategies for gastroschisis and their outcomes is largely limited to hospital-based studies. The aim of this study was to use a new UK national surveillance system to identify cases and thus to describe the contemporary surgical management and outcomes of gastroschisis.

Methods

We conducted a national cohort study using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System to identify cases between October 2006 and March 2008.

Results

All 28 surgical units in the United Kingdom and Ireland participated (100%). Data were received for 95% of notified cases of gastroschisis (n = 393). Three hundred thirty-six infants (85.5%) had simple gastroschisis; 45 infants (11.5%) had complex gastroschisis. For 12 infants (3.0%), the type of gastroschisis could not be categorized. Operative primary closure (n = 170, or 51%) and staged closure after a preformed silo (n = 120, or 36%) were the most commonly used intended techniques for simple gastroschisis. Outcomes for infants with complex gastroschisis were significantly poorer than for simple cases, although all deaths occurred in the simple group.

Conclusions

This study provides a comprehensive picture of current UK practice in the surgical management of gastroschisis. Further follow-up data will help to elucidate additional prognostic factors and guide future research.     

Amniotic fluid ferritin as a marker of intestinal damage in gastroschisis: a time course experimental study

Background/Purpose

Intestinal damage (ID) is closely related to morbidity and mortality in gastroschisis. This study was performed to determine the intraamniotic substances that may correlate ID and also to verify their time course levels that would be useful for determining when ID starts in gastroschisis.

Methods

In this study, 13-day-old fertilized chick eggs were used. The amnioallantoic membrane was perforated to create amnioallantoic cavity in all embryos. Gastroschisis was created in gastroschisis group to simulate human gastroschisis. Amnioallantoic fluid samples were collected from the embryos on the 13th to 19th gestational days, and the intestines of each group were harvested for evaluation. Amnioallantoic levels of interleukin-8, ferritin, alkaline phosphatase, and amylase were measured. Serosal thickness of the intestines in each group was evaluated.

Results

Increasing amnioallantoic fluid levels of interleukin-8, alkaline phosphatase, and amylase were found in both groups. In contrast to control group, ferritin levels, as a sign of inflammation, were found increased only in gastroschisis group. Histopathologic examination of intestines in the gastroschisis group showed a significant increase in the serosal thickness especially after the 16th day.

Conclusion

Increases in amnioallantoic fluid levels of ferritin show promise as a marker for determining ID encountered in gastroschisis but warrant further investigation.     

Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose

Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome.

Methods

A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994.

Results

Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years.

Conclusions

Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.     

Risk factors and management of intra-abdominal infection after extended radical gastrectomy

Background

This study elucidated risk factors and management for intra-abdominal infection after extended radical gastrectomy.

Methods

From 1988 to 2004, 2,076 patients with gastric cancer underwent extended radical gastrectomy at Taipei Veterans General Hospital. Risk factors for intra-abdominal infection were determined by analyzing clinicopathological factors, operative procedure, combined organ resection, operative time, blood loss, and associated disease(s). Management modalities were summarized.

Results

The overall complication rate was 18.7%. Eighty (3.9%) patients were found to have intra-abdominal infections. Age, prolonged operation time, and combined organ resection were the precipitating factors. These patients were categorized into 3 groups: intra-abdominal abscess with adequate drainage, intra-abdominal abscess without anastomotic leakage, and intra-abdominal abscess because of leakage. Adequate drainage was the primary treatment. Mortality rate was 22.5% (18), and the most common cause of mortality was intra-abdominal abscess caused by leakage.

Conclusions

Although expert surgical skills can minimize the incidence of intra-abdominal infection, management also requires experience and training.     

Using the Clavien Grading System to Classify the Complications of Right Hepatectomy in Living Donors

Introduction

The ratios of complications for living related liver donors after right hepatectomy differ widely among numerous single institutions. This study sought to use the Clavien classification system to define and graded the severity of these complications.

Materials and methods

This study retrospectively analyzed the outcomes of 160 consecutive living donor right hepatectomies performed between July 2002 and February 2008. Complications among living donors for liver transplantation after right hepatectomy were stratified according to the Clavien classification of postoperative surgical complications.

Results

Fifty-two living donors displayed one or more perioperative complications Grade 1 complications were recorded in 18.1%; grade 2 in 6.3%; grade 3a in 5%; and grade 3b in 3.1%. Biliary complications were the most frequent. No donor mortality was present in this series.

Conclusions

The Clavien grading system is useful to comparise surgical outcomes. This study demonstrated that donor right hepatectomy was a relatively safe procedure, but reducing donor complications after right hepatectomy has to be the first priority during the entire process of living related transplantation.     

Effects of prenatal dexamethasone on the intestine of rats with gastroschisis

Background/purpose

Intestinal lesions observed in gastroschisis (Gx) are accompanied by neonatal gastrointestinal dysfunction. This study examines the effects of transplacental dexamethasone on the eviscerated intestine of fetal rats with Gx.

Methods

Gx was created surgically in rat fetuses on gestational day 18, and the dams were treated either with 0.4 mg/kg intraperitoneal dexamethasone or with vehicle only on days 19 and 20. The intestine recovered on day 21 were processed for total DNA and protein. Immuno-histochemical staining for ki-67, TUNEL, and synaptophysin were used for assessing the proportions of proliferating and apoptotic cells and the density of intramural ganglia. Analysis of variance (ANOVA) was used for comparison among groups. Significance level was set at P less than .05.

Results

Body weight was reduced in Gx fetuses in comparison with controls. Intestinal weight per centimeter and mucosal and seromuscular layer thicknesses were increased in Gx and Gx + dexa groups. Total intestinal DNA was diminished in Gx animals but it was near normal in Gx + dexa ones. Total intestinal protein was similar in all groups. DNA and protein per centimeter of bowel were very increased in Gx animals but only slightly in Gx + dexa ones. Proliferating cells were decreased in Gx animals and increased in Gx+dexa ones, whereas the opposite was observed for apoptosis. Density of intramural ganglia was decreased significantly in both Gx groups.

Conclusions

Late intrauterine exposure to dexamethasone of rat fetuses with Gx decreased wall thickening, normalized total DNA, and induced proliferation in the exposed bowel while limiting apoptosis. This medication could have some yet incompletely defined beneficial effects on the wall of the eviscerated bowel in Gx.     

Gastroschisis: the cost of an epidemic

Background

The rising incidence of gastroschisis has been highlighted by the Department of Health as a growing concern. As well as the health implications for the increasing number of affected infants, this increase in incidence will have an impact of the costs of health care. This study was undertaken to estimate the financial cost of treating this condition in one tertiary neonatal surgical center.

Methods

A retrospective analysis was performed of all patients admitted to a tertiary neonatal surgical center with gastroschisis from January 1996 to December 2005. The main outcome measures were incidence, length of hospital stay, and total cost for all patients each year.

Results

The incidence of gastroschisis has risen 3-fold in 10 years. The median cost per patient is relatively constant. A few patients with severe intestinal dysmotility require prolonged hospital stay. As the condition becomes more common, there are an increasing number of complex patients and thus an increase in annual costs, which is disproportionate to the increase in numbers of cases. We estimate that the annual cost to the National Health Service (NHS) of this condition in England and Wales has risen from £3.6 million in 1996 to in excess of £15 million in 2005.

Conclusions

Urgent research is required into the etiology of gastroschisis and into the severe intestinal dysmotility that occurs in some complex patients.     

Incidence and risk factors for nonmelanoma skin cancer after heart transplantation

Introduction

The incidence of skin cancer in heart transplant (HT) patients is higher than in the general population, reversing the proportion of cutaneous squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) with a predominance of the former. The etiologic role of new immunosuppressants is not well known. We sought to ascertain the incidence of SCC and BCC in HT patients and the risk factors for its occurrence.

Patients and Methods

We report the incidence of all types of post-HT skin cancer, SCC, and BCC among adult HT patients in Spain (4089 subjects) as well as the influence of gender, age at heart transplant, immunosuppression, and sunlight exposure.

Results

The incidence rates of SCC and BCC, per 1000 persons/year, were 8.5 and 5.2, respectively. Males had a higher risk of SCC but not BCC. Induction therapy increased the risk of SCC and BCC. The relative risk of mycophenolate mofetil (MMF) was 0.3 (0.2-0.6; P < .0005) and azathioprine (AZA) 1.8 (1.2-2.7; P < .0032) for SCC, whereas tacrolimus and cyclosporine showed no difference. The relative risk of BCC was not affected by any immunosuppressant.

Conclusion

Age at transplantation >45 years, induction therapy use, and high sunshine zone were risk factors for both SCC and BCC. Different immunosuppressive agents have different risks of nonmelanoma skin cancer, as AZA increases the risk of SCC and MMF is a protective factor. The relative risk of BCC was not affected by any immunosuppressor.