Mortality in neonates with giant omphalocele subjected to a surgical technique in Barranquilla, Colombia from 1994 to 2019.

No studies of the efficacy and safety of surgical techniques for the primary closure of giant omphalocele have been performed in Colombia. To determine the mortality rate and factors associated with mortality in neonates with giant omphalocele subjected to the surgical technique of early closure with a surgical silo described by Abello in Barranquilla, Colombia from 1994 to 2019. Retrospective cohort study of 30 neonates diagnosed with giant omphalocele and subjected to early closure of the defect. Medical history data were collected, information bias was controlled for, and descriptive statistical analysis was performed using Fisher's exact test and the Mann-Whitney U test in SPSS 25.0. Of the patients in the cohort, 36.7% presented technique-related complications, 56.7% developed sepsis, 23.3% had low birth weight, 26.7% were preterm births, 43.3% had other malformations, 26.7% had congenital heart defects, and 13.3% presented pulmonary hypertension. The mean hospital stay was 26 days. The mortality rate was 16.7%; it was significantly higher among patients with other malformations, congenital heart defects, pentalogy of Cantrell and pulmonary hypertension. The Abello technique for the treatment of giant omphalocele showed a high neonatal survival rate and a low rate of procedure-related complications. The main factors associated with the death of neonatal patients were the presence of other malformations, congenital heart defect, pentalogy of Cantrell and pulmonary hypertension.

Omphalocele in Finland from 1993 to 2014: Trends, Prevalence, Mortality, and Associated Malformations-A Population-Based Study.

INRODUCTION: The aim of this study is to assess the changes in prevalence, mortality and termination pregnancy of omphalocele, and to identify associated anomalies. MATERIALS AND METHODS: A population-based nationwide register study. All cases with omphalocele were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded and analyzed, and perinatal and infant mortality and prevalence were calculated. RESULTS: There were 600 cases with omphalocele including 229 live births, 39 stillbirths, and 332 (55%) abortions. Birth prevalence in Finland was 1.96 per 10,000 births with no consistent trend over time. However, total prevalence was much higher (4.71/10,000) because more than half of these families chose option for the termination of pregnancy. Omphalocele is often complicated with other anomalies; most commonly chromosomal abnormalities (9.3%), heart defects (6.3%), central nervous system anomalies (3.0%), gastrointestinal, and urogenital malformations (both 2.0%). Proportion of chromosomal and central nervous system abnormalities were even higher in terminated pregnancies. Overall infant mortality was 22%. Total 1-year survival rates for isolated omphalocele, cases with multiple anomalies and neonates with chromosomal defects were 80, 88, and 17%, respectively. CONCLUSION: Omphalocele is a rare congenital anomaly, often associated with other malformations. Our data suggest that isolated cases may be more common than previously thought. In the absence of chromosomal defects, survival is reasonably good. Regardless, more than half of these pregnancies are often terminated.

Comprehensive Echocardiographic Assessment of Ventricular Function and Pulmonary Pressure in the Neonatal Omphalocele Population.

OBJECTIVE: Pulmonary hypertension (PH) has been described in the neonatal omphalocele population. This study was aimed to describe cardiac function and PH severity using echocardiography in newborns with giant omphalocele (GO) and with non-GO and determine if right ventricular (RV) dysfunction is associated with mortality. STUDY DESIGN: Retrospective, single-center analysis of first echocardiography among neonatal omphalocele patients born between 2004 and 2017 was conducted. Multivariate logistic and univariate Cox's regression was constructed to measure hazard ratio (HR) for death outcome. RESULTS: There were 32 newborns, of whom 18 were GO and 7 died. GO had increased systolic pulmonary arterial to systolic systemic blood pressure ratio (97% [isosystemic] vs. 73% [three-fourths systemic] p = 0.03). RV performance parameters (tricuspid annular plane excursion, HR = 0.40; fractional area change, HR = 0.90; and RV peak global longitudinal strain, HR = 1.39) were associated with mortality. These RV performance parameters remained associated in a multiple logistic regression accounting for gestational age and GO status. The overall population had abnormal eccentricity index and pulmonary artery acceleration time to RV ejection time ratio, two markers of PH. CONCLUSION: Patients with omphalocele have increased pulmonary pressure, with GO being worse than non-GO. RV dysfunction at initial echocardiography was significantly associated with mortality.

The validity of the viscero-abdominal disproportion ratio for type of surgical closure in all fetuses with an omphalocele.

OBJECTIVE: To determine the predictive value of the fetal omphalocele circumference/abdominal circumference (OC/AC) ratio for type of surgical closure and survival and to describe the trajectory of OC/AC ratio throughout gestation. METHODS: This cohort study included all live-born infants prenatally diagnosed with an omphalocele in our tertiary centre (2000-2017) with an intention to treat. The OC/AC ratio and liver position were determined using 2D ultrasound at three periods during gestation (11-16, 17-26, and/or 30-38 weeks). Primary outcome was type of closure; secondary outcome was survival. In the secondary analyses, the predictive value of the OC/AC-ratio trend for type of closure and survival was assessed. RESULTS: Primary closure was performed in 37/63 (59%) infants, and 54/63 (86%) survived. The OC/AC ratio was predictive for type of closure and survival in all periods. Optimal cut-off values for predicting closure decreased throughout gestation from 0.69 (11-16 weeks) to 0.63 (30-38 weeks). Repeated OC/AC-ratio measurements were available in 33 (73%) fetuses. The trend of the OC/AC ratio throughout gestation was not significantly associated with type of closure. All infants without liver herniation underwent primary closure. CONCLUSION: Type of omphalocele surgical closure and survival can be predicted prenatally on the basis of the OC/AC ratio and liver herniation independent of associated anomalies. LEARNING OBJECTIVE: The reader will be able to use the OC/AC ratio throughout gestation in all omphalocele cases for prediction of type of closure and survival and thus patient counselling.

Prenatally diagnosed omphalocele: characteristics associated with adverse neonatal outcomes.

OBJECTIVE: To characterize factors associated with adverse neonatal outcomes in prenatally diagnosed omphalocele cases. STUDY DESIGN: Prenatally diagnosed omphalocele cases at a single referral center from 1 January 2009 to 31 December 2017 were retrospectively reviewed. Clinical variables and antenatal imaging measurements were collected. Associations between prenatal and neonatal characteristics and the adverse outcome of death or prolonged length of stay (LOS) were analyzed. RESULTS: Out of 63 fetal cases, 33 were live-born, > 50% had other anomalies, and neonatal mortality was 12%. Adverse outcomes were associated with neonatal variables, including lower median 1-min Apgar score, initial mechanical ventilation, and late-onset sepsis, but not approach to omphalocele closure. With multivariate analysis, death or prolonged LOS was associated only with low lung volumes by fetal MRI (OR 34 (3-422), p = 0.006). CONCLUSION: Low lung volumes by fetal MRI were associated with death or prolonged LOS in neonates with prenatally diagnosed omphalocele and may guide clinicians with counseling families.

OEIS complex: Prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program.

OEIS is the acronym of a malformations complex association including omphalocele, exstrophy of bladder or cloaca, imperforate anus, and spinal defects. It has a very low prevalence, ranging from 1/82,000 to 1/200,000 live births (LB). The etiology of OEIS is unknown. Virtually all cases are sporadic, and specific associated risk factors uncertain. OBJECTIVES: This study aimed to determine the prevalence, clinical spectrum, possible early pregnancy exposures, and demographic characteristics as potentially associated risk factors in a sample of Mexican cases. METHODS: We conducted a multihospital based case-control study on 12 cases with the OEIS complex identified in 1,195,020 LB born from January 1978 to December 2015. All comparisons performed were matching 1:3 the relation of cases and controls, respectively, considering the p-value of ≤.05 as statistically significant. RESULTS: The prevalence of OEIS was 1.004/100,000 (1/99,585) LB. The frequency of bladder/cloacal exstrophy was 75 and 25%, respectively, omphalocele was 83.3%, and imperforate anus and spinal defects, 75.0% each. Two pairs of twins discordant for the defect exhibited the severest OEIS phenotype. Except for the higher frequency of maternal first pregnancy trimester influenza infection, early perinatal mortality and a twining trend association, none other variable differed significantly. DISCUSSION: The prevalence of OEIS in our sample is within the highest reported worldwide. First-trimester pregnancy maternal influenza infection and twining emerge as associated risk factors for OEIS. Although twin zygosity was not defined, the observed severest phenotypes in twins endorse the hypothesis that OEIS and monozygotic twinning are features of disturbances on early blastogenesis.

Anterior abdominal wall defects managed at a tertiary maternal-fetal medicine service in New Zealand: What counselling advice can we offer parents?

BACKGROUND: Anterior abdominal wall defects, including gastroschisis and omphalocoele, are common fetal anomalies. The management remains complicated, and their diagnosis may lead to significant parental distress. Effective parental counselling may impact on parental perceptions of the defect and help guide pregnancy management. AIMS: Using contemporary data, we aimed to describe clinical outcomes of patients with gastroschisis or omphalocoele in order to provide information for clinicians to assist in parental counselling. MATERIALS AND METHODS: We followed a case-series of patients with anterior abdominal wall defects referred to our regional Maternal Fetal Medicine services from 2011 to 2016. Outcomes of interest antenatally included details of diagnosis, associated anomalies and outcomes of pregnancy and postnatally included the nature of surgical repair, hospital stay and secondary complications until initial discharge. RESULTS: Eighty babies with gastroschisis were referred antenatally, and 72 were liveborn. Forty-nine babies with omphalocoele were referred antenatally, and 24 were liveborn. One further neonate with omphalocoele was postnatally diagnosed. Seventy-one neonates with gastroschisis progressed to operation, 30 developed complications post-surgery, and 68 survived until initial discharge. Twenty-two neonates with omphalocoele progressed to surgery, only two developed complications, and 21 survived until initial discharge. Eight of the surviving neonates with omphalocoele had associated structural abnormalities. The median hospital stay was 27 and eight days for gastroschisis and omphalocoele, respectively. CONCLUSION: Neonates with gastroschisis can have complex postnatal periods. Omphalocoele is associated with high antenatal mortality, especially in the presence of associated abnormalities; however, surviving neonates may have uneventful postnatal periods.

Outcomes in omphalocele correlate with size of defect.

BACKGROUND: Omphaloceles can be some of the more challenging cases managed by pediatric surgeons. Single center studies have not been meaningful in delineating outcomes due to the length of time required to accumulate a large enough series with historical changes in management negating the results. The purpose of this study was to evaluate factors impacting the morbidity and mortality of neonates with omphaloceles. METHODS: A multicenter, retrospective observational study was performed for live born neonates with omphalocele between 2005 and 2013 at nine centers in the United States. Maternal and neonatal data were collected for each case. In-hospital management and outcomes were also reported and compared between neonates with small and large omphaloceles. RESULTS: Two hundred seventy-four neonates with omphalocele were identified. The majority were delivered by cesarean section with a median gestational age of 37 weeks. Overall survival to hospital discharge was 81%. The presence of an associated anomaly was common, with cardiac abnormalities being the most frequent. Large omphaloceles had a significantly longer hospital and ICU length of stay, time on ventilator, number of tracheostomies, time on total parenteral nutrition, and time to full feeds, compared to small omphaloceles. Birth weight and defect size were independent predictors of survival. CONCLUSION: This is the largest contemporary study of neonates with omphalocele. Increased defect size is an independent predictor of neonatal morbidity and mortality. LEVEL OF EVIDENCE: Level II.

Accuracy and impact of prenatal diagnosis in infants with omphalocele.

BACKGROUND: Associated anomalies in omphalocele are common, but to which extent these anomalies are diagnosed before or after birth is less well documented. AIM: To investigate the different types of associated anomalies, long-term survival and the extent whether these are diagnosed pre- or postnatally in children with a prenatal diagnosis of omphalocele at a single institution. MATERIALS AND METHODS: Retrospective review of all pregnancies with omphalocele managed and/or born at our institution between 2006 and 2016. RESULTS: A total of 42 cases with prenatally diagnosed omphalocele were identified. Of those 14 (31%) decided to terminate the pregnancy (TOP). Of the remaining 28 that continued, 12 were giant omphaloceles. The overall mortality rate was 18, 25% for giant and 12% for non-giant omphaloceles. 64% had associated anomalies. Only 1/3 of these anomalies is diagnosed prenatally. CONCLUSION: The rate of associated malformations that are diagnosed postnatally is high, but the majority was malformations with a minor clinical significance or impact on future health. Beckwith-Wiedemann syndrome was present only in cases of non-giant omphalocele in our cohort.

Mortality and short-term morbidity in infants with exomphalos.

BACKGROUND: Infants with exomphalos major have a high mortality and morbidity. The aims of this study were to identify predictors of survival regardless of the size of the exomphalos, and to analyze morbidity in infants with exomphalos minor. METHODS: Patients were classified as having exomphalos major or minor based on whether the liver was in the exomphalos sac, and the size of the abdominal wall defect. The respiratory, gastrointestinal and surgical outcomes of 50 infants with exomphalos (including 27 with exomphalos major) were assessed. Receiver operating characteristic (ROC) curves were constructed to identify factors predictive of survival. RESULTS: No infant with exomphalos minor died; there were seven deaths in the exomphalos major group (P < 0.001). Infants with exomphalos minor who had chromosomal abnormalities (six had a genetic diagnosis of Beckwith-Wiedemann syndrome) developed severe respiratory distress or chronic respiratory morbidity. Nasogastric feeding at discharge was required in 37% of infants with exomphalos major and in 17% with exomphalos minor. Lower gestational age (area under the ROC curve [AUROC], 0.814) and birthweight (AUROC, 0.797), and longer duration of ventilation (AUROC, 0.853) and of supplementary oxygen (AUROC, 0.810) were predictive of mortality. CONCLUSIONS: Infants with exomphalos regardless of size can have chronic morbidity. Mortality is commonest in those with exomphalos major born at lower gestational age and birthweight.

Primary versus Staged Closure of Exomphalos Major: Cardiac Anomalies Do Not Affect Outcome.

AIM: The objective of the study is to describe management of exomphalos major and investigate the effect of congenital cardiac anomalies. METHODS: A single-center retrospective review (with audit approval) was performed of neonates with exomphalos major (fascial defect ≥ 5cm ± liver herniation) between 2004 and 2014.Demographic and operative data were collected and outcomes compared between infants who had primary or staged closure. Data, median (range), were analyzed appropriately. RESULTS: A total of 22 patients were included, 20 with liver herniation and 1 with pentalogy of Cantrell. Gestational age was 38 (30-40) weeks, birth weight 2.7 (1.4-4.6) kg, and 13 (60%) were male. Two were managed conservatively due to severe comorbidities, 5 underwent primary closure, and 15 had application of Prolene (Ethicon Inc) mesh silo and serial reduction. Five died, including two managed conservatively, none primarily of the exomphalos. Survivors were followed up for 38 months (2-71). Cardiac anomalies were present in 20 (91%) patients: 8 had minor and 12 major anomalies. Twelve (55%) patients had other anomalies. Primary closure was associated with shorter length of stay (13 vs. 85 days, p = 0.02), but infants had similar lengths of intensive care stay, duration of parenteral feeds, and time to full feeds. Infants with cardiac anomalies had shorter times to full closure (28 vs. 62 days, p = 0.03), but other outcomes were similar. CONCLUSION: Infants whose defect can be closed primarily have a shorter length of stay, but other outcomes are similar. Infants with more significant abdominovisceral disproportion are managed with staged closure; the presence of major cardiac anomalies does not affect surgical outcome.

Predictors of mortality in neonates with giant omphaloceles.

INTRODUCTION: This analysis performed a review of giant-omphaloceles to determine the predictors of mortality. EVIDENCE ACQUISITION: PubMed and KoBson databases were searched for terms "giant," "omphalocele," and "mortality." Primary end points included mortality correlation with gestational age (GA), birth weight (BW), eviscerated organs, associated anomalies and management. To calculate mean and median values IBM SPSS v. 23.0 was used. EVIDENCE SYNTHESIS: After de-duplication and review search revealed 42 articles of which 23 met the inclusion criteria with 396 giant-omphaloceles for this analysis. Median gestational age (GA) was 36 weeks for all neonates (range 21-41); 21 neonates were reported as premature with median GA 33.5 (range 21-36). Overall median birth weight (BW) was 3100 g (range 1100-4100 g). The diameter of abdominal wall defect was 4-15 cm with the average size of 7.6 cm except for non-giant giant omphaloceles (N.=7) where the defect was measuring between 2.7 and 4 cm. Amniotic sac contents beside intestines included liver (N.=154), stomach (N.=11), spleen (N.=2), pancreas (N.=1), gallbladder (N.=5), and 5 giant omphaloceles were reported to contain only liver; sac was ruptured in 22. Giant omphaloceles were associated with a variety of other anomalies, most often with cardiac anomalies (N.=93; 23.4%) and pulmonary hypoplasia and/or pulmonary hypertension (N.=39; 9.8%). Management included conservative treatment N.=264 (66.6%), primary closure (N.=17; 4.3%), staged closures (N.=98; 24.7%) primary or staged closure (N.=17; 4.3%). The most frequent complication was sepsis (N.=52). There were 90 (22.7%) lethal outcomes, 6 lethal outcomes in neonates even before final closure could be achieved and 12 in prematures. Leading cause of mortality was sepsis (N.=51; 56.6%), the cause of lethal outcome was not reported in 8 cases. CONCLUSIONS: Giant-omphaloceles have a lethal outcome in one-fifth of neonates. Predictors of mortality included pulmonary hypoplasia and respiratory failure with prematurity and ruptured sacs implicated within this group. Sepsis was the independent iatrogenic factor in mortality.

The use of ECMO for gastroschisis and omphalocele: Two decades of experience.

PURPOSE: The aim was to review the respiratory failure causes and outcomes of infants with omphalocele or gastroschisis receiving ECMO and reported to the Extracorporeal Life Support Organization (ELSO). METHODS: Gastroschisis and omphalocele infants supported with ECMO and reported to the ELSO Registry between 1992 and 2015 were retrospectively reviewed. Clinical variables, diagnosis of respiratory failure (pulmonary hypertension (PHN), congenital heart defects (CHD), congenital diaphragmatic hernia (CDH), and sepsis), and outcomes were recorded. Univariate analysis was performed using Student's t-test for continuous or Fisher's exact test for categorical variables. RESULTS: Fifty-two infants with gastroschisis (41) (79%) or omphalocele (11) (21%) were identified. The survival to discharge rate of 51% for gastroschisis remained stable and was significantly higher (P=0.05). The overall mortality rate for omphalocele was 82%. Omphalocele had significantly more PHN (P<0.01), CDH (P<0.01), and multiple anomalies (P=0.04) had significantly more sepsis (P=0.02), and none had a CDH. CONCLUSION: Infants with gastroschisis requiring ECMO support have significantly better survival than omphaloceles, and respiratory failure is significantly associated with sepsis. The majority of omphalocele infants die despite ECMO, and respiratory failure is associated PHN and CDH. The association of omphalocele, PHN, and CDH merits further investigation. STUDY TYPE AND EVIDENCE LEVEL: Retrospective comparative study of Registry Database, Level 3.

The effects of amoxicillin treatment of newborn piglets on the prevalence of hernias and abscesses, growth and ampicillin resistance of intestinal coliform bacteria in weaned pigs.

This study investigated the effects of a single amoxicillin treatment of newborn piglets on the prevalence of hernias and abscesses until the age of nine weeks. We also studied whether the treatment was associated with growth and mortality, the need for treatment of other diseases, the proportions of ampicillin resistant coliforms and antimicrobial resistance patterns of intestinal Escherichia coli (E. coli). A total of 7156 piglets, from approximately 480 litters, were divided into two treatment groups: ANT (N = 3661) and CON (N = 3495), where piglets were treated with or without a single intramuscular injection of 75 mg amoxicillin one day after birth, respectively. The umbilical and inguinal areas of weaned pigs were palpated at four and nine weeks of age. At the same time, altogether 124 pigs with hernias or abscesses and 820 non-defective pigs from three pens per batch were weighed individually. Mortality and the need to treat piglets for other diseases were recorded. Piglet faecal samples were collected from three areas of the floors of each pen at four weeks of age. The prevalence of umbilical hernias or abscesses did not differ between the groups at four weeks of age, but it was higher in the CON group than in the ANT group at nine weeks of age (2.3% vs. 0.7%, P < 0.05). Numbers of inguinal hernias and abscesses did not differ between the groups at four or nine weeks of age. The ANT group, when it compared with the CON group, increased the weight gain between four and nine weeks of age (LS means ± SE; 497.5 g/d ± 5.0 vs. 475.3 g/d ± 4.9, P < 0.01), and decreased piglet mortality (19.5% ± 1.0 vs. 6.9% ± 1.0, P < 0.05) and the need to treat the piglets for leg problems (3.4% ± 0.3 vs. 1.9% ± 0.3%, P < 0.01) but not for other diseases by the age of four weeks. The proportion of ampicillin resistant intestinal coliform bacteria and the resistance patterns of the E. coli isolates were not different between the ANT and CON groups. In conclusion, our results showed that the amoxicillin treatment of new-born piglets produced statistically significant effect in some of the parameters studied. However, as these effects were only minor, we did not find grounds to recommend preventive antibiotic treatment. Further, continuous antimicrobial treatment of newborn piglets could negatively influence the development of the normal microbiota of the piglet and promote selection of antimicrobial resistance genes in herds. Therefore we suggest rejection of the use of routine administration of antimicrobial agents at birth.

Thorax-to-head ratio and defect diameter-to-head ratio in giant omphaloceles as predictor for fetal outcome.

PURPOSE: To investigate the relationship between the thorax diameter and defect diameter of giant omphaloceles as a predictor for fetal outcome. METHODS: In a retrospective study, 17 fetuses with isolated giant omphaloceles were included for evaluation. The anterior-posterior thorax diameter and the defect diameter were measured from ultrasound images. For analysis, the thorax-to-head ratio (T/HC), the defect diameter-to-head ratio (DD/HC), and the quotient of the defect diameter and the thorax diameter (DD/T) were calculated. The days of ventilation (t ventilation), the duration until hospital discharge (t hospital), and the type of treatment were recorded as outcome parameters. RESULTS: No relationship was found between the calculated ratios (T/HC, DD/HC, or DD/T) and neither t hospital (r = -0.418, p = 0.095; r = -0.153, p = 0.556; and r = -0.023, p = 0.929; respectively) nor t ventilation (r = -0.391, p = 0.121; r = 0.041, p = 0.875; and r = 0.121, p = 0.645, respectively). The type of postnatal treatment was not associated with the three calculated ratios or t hospital (r = 0.155, p = 0.553; r = 0.019, p = 0.942; and r = 0.012, p = 0.965; r = -0.009, p = 0.973, respectively). In 53% of cases, t hospital was delayed due to additional and independent postnatal complications. CONCLUSION: Thorax diameter or defect diameter of giant omphaloceles is not predictive for fetal outcome. The perinatal care of these abdominal wall defects still remains a multidisciplinary challenge, but the outcome of giant omphaloceles is favorable at experienced centers.

Giant omphaloceles: surgical management and perinatal outcomes.

BACKGROUND: The purpose of this study was to describe the current management and outcomes of infants with omphalocele. METHODS: The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. RESULTS: Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4-23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. CONCLUSIONS: The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.

Management of umbilical hernias in patients with ascites: development of a nomogram to predict mortality.

BACKGROUND: The objective of this study was to develop an easy-to-use nomogram to assist clinicians in predicting patient-specific mortality in this patient population. METHODS: American College of Surgeons National Surgical Quality Improvement Program participant use files were used from 2005 to 2011. Multivariable logistic regression was used to model 30-day postoperative mortality in patients with ascites who underwent umbilical hernia repair. RESULTS: A total of 688 patients with ascites undergoing umbilical hernia repair were included. There were 643 (94%) survivors and 45 (7%) mortalities. A total of 300 (44%) patients were classified as emergent cases. Using logistic regression to predict 30-day mortality, preoperative Model for End-Stage Liver Disease score, albumin, white blood cell count, and platelet count were found to be significant predictors (P < .05) of mortality and were included in our model. CONCLUSION: We propose a nomogram to enable clinicians to better estimate mortality in patients with ascites undergoing umbilical hernia repair.

Pulmonary hypertension predicts mortality in infants with omphalocele.

OBJECTIVE: The objective of this study was to identify predictors of mortality in infants with omphalocele. METHODS: Medical records of infants with omphalocele born between January 1992 and June 2012, with follow-up toDecember 2012, were retrospectively reviewed. Survivors and non-survivors were compared. Evidence for pulmonary hypertension was sought between the second and seventh day after birth. All included infants had increased right ventricular pressures (RVP >40 mmhg) on echocardiogram on the second day of life with increased oxygen requirements, therefore, the finding of increased pressure was not considered a result of the transitional circulation. Logistic regression was used to evaluate the importance and independence of various factors. RESULTS: Of 51 infants whose records were reviewed, 13 died (25%) and 38 survived (75%). The median time to death was 34 days (range: 4 -408 days). The median follow-up time for those who died was 1.5 years (range: 0.01-15 years) and for survivors was 2.6 years (range: 0.08-15 years). Logistic regression revealed that respiratory insufficiency at birth (OR: 14.8; 95% CI: 2.5-85.0) and pulmonary hypertension (OR: 6.4; 95% CI: 1.1-39.0) were independently associated with mortality. CONCLUSION: Respiratory insufficiency after birth and pulmonary hypertension are independent predictors of mortality in infants with omphalocele.

Birth prevalence and survival of exomphalos in england and wales: 2005 to 2011.

BACKGROUND: Exomphalos occurs in 2.2 per 10,000 births with 76% of these babies surviving to discharge. The aim of this study was to determine the birth prevalence and survival of babies with this anomaly in England and Wales. METHODS: Six BINOCAR regional congenital anomaly registers in England and Wales (covering 36% of births) between 2005 and 2011 provided cases for this study. Cases included live births, stillbirths (24+ weeks' gestation), late miscarriages (20-23 weeks' gestation), and terminations of pregnancy with fetal anomaly. RESULTS: The overall birth prevalence was 3.8 (95% confidence interval [CI]: 3.6-4.0) per 10,000 births; 1.4 (1.2-1.6) for isolated cases, 1.2 (1.1-1.4) for cases with multiple anomalies, and 1.2 (1.1-1.4) for cases with chromosomal anomalies. The live birth prevalence was 0.8 (0.7-0.9), 0.5 (0.4-0.6), and 0.1 (0.0-0.1) per 10,000 live births, respectively. Edwards syndrome, congenital heart defects, and nervous system anomalies were the most common anomalies associated with exomphalos. A prenatal diagnosis was made in 83% of isolated, 95% of multiple, and 99% of chromosomal cases. Fifty-five percent of isolated and multiple cases were live born, whereas 85% of cases with chromosomal anomalies resulted in a termination of pregnancy with fetal anomaly. The 1-year survival of live born babies with an isolated exomphalos was 92% compared with 81% in cases with multiple anomalies and 27% in cases with chromosomal anomalies (p < 0.001). CONCLUSION: We report a higher birth prevalence than has previously been reported. The proportion of infants surviving with exomphalos remained unchanged over the time period.