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.

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.

Should we measure fetal omphalocele diameter for prediction of perinatal outcome?

OBJECTIVE: To evaluate the fetal omphalocele diameter/abdominal circumference ratio (OD/AC) as a predictor of adverse perinatal outcome. METHODS: Analysis involving 47 singleton pregnancies with fetal omphalocele, normal karyotype and absence of other major abnormalities. The OD/AC ratio was determined antenatally by ultrasound and the best cutoff for the prediction of neonatal death was determined by receiver operating characteristic curve analysis. Additional secondary outcomes included need for oral intubation in the first 24 h of life, two-step surgery or use of synthetic mesh, reoperation, parenteral feeding and need for respiratory assistance >21 days, time to first oral feed, and time to hospital discharge. RESULTS: Fetal OD/AC did not change significantly with gestational age. Postnatal death occurred in 10 (21.3%) cases and the best cutoff for prediction was an OD/AC ratio ≥0.26. In pregnancies with the first ultrasound evaluation performed before 31 weeks' gestation and an OD/AC ≥0.26, the likelihood ratio for needing intubation in the first 24 h of life was 2.6 (95% CI: 1.2-5.7), needing two-step surgery or use of mesh was 4.9 (95% CI: 1.9-14.4), and postnatal death was 4 (95% CI: 1.9-7.5). CONCLUSION: A fetal ultrasound OD/AC ratio ≥0.26 is associated with increased postnatal morbidity and mortality.

Defectos de la pared abdominal. Estudio comparativo entre onfalocele y gastrosquisis / Abdominal wall defects. A Comparative investigation between omphalocele and gastroschisis

Introducción: Onfalocele y gastrosquisis son los defectos de la pared abdominal (DPA) más frecuentes, que por su importancia y las diferencias entre ellos merecen ser estudiados en forma especial. Objetivos: Establecer las tasas de prevalencia de onfalocele y gastroaquisis al nacimiento. Verificar variaciones de ellas en distintos períodos. Estudiar la morbimortalidad asociada y su sobrevida. Pacientes y Método: Se revisó la base de datos ECLAMC (Estudio Colaborativo Latino Americano de Malformaciones Congênitas) para el período 1996-2010. Resultados: Se pesquizarón 33 recién nacidos (RN) con DPA. (11,6 por 10.000 nacimientos). De ellos 19 fueron onfalocele (6,7 por 10.000) y 14 (4,9 por 10.000) gastrosquisis. Los niños con onfalocele tenían significativamente mayor asociación con otras malformaciones, su frecuencia en el sexo masculino fue mayor y mostró mayor letalidad que la gastrosquisis. Todos los niños con gastrosquisis nacieron vivos, con predominio del sexo femenino, sus madres eran significativamente más jóvenes. Conclusión: Onfalocele y gastros-quisis presentaron una prevalencia al nacimiento mayor que lo publicado, probablemente por ser el HCUCH un Centro de Referencia. Ambas anomalías mostraron un aumento significativo en sus tasas de prevalencia al nacimiento al compararlas con las obtenidas anteriormente por nosotros en el mismo hospital. Se demostró diferencias significativas entre estas dos entidades: Onfalocele tiene mayor mortinatalidad, mortalidad y letalidad, mayor frecuencia de malformaciones asociadas y mayor promedio de edad materna.

Introduction: Omphalocele and Gastroschisis are the two most common congenital abdominal wall (AWD) defects. Objectives: To determine birth prevalence of Omphalocele and Gastroaquisis; to verify variations in different periods and to study the associated morbidity, mortality and survival. Patients and Method: The database of the Latin American Collaborative Study of Congenital Malformations (ECLAMC) was searched between the years 1996 and 2010. Results: 33 newborns (NB) with AWD were investigated (11.6 per 10,000 births). 19 of the cases corresponded to omphalocele (6.7 per 10,000 births) and 14 to gastroschisis (4.9 per 10,000 births). Children with omphalocele had significantly greater association with other malformations and the frequency in males was higher and showed higher mortality rates than gastroschisis. All children with gas-troschisis were born alive; they were predominantly female infants whose mothers were significantly younger. Conclusion: Birth omphalocele and gastroschisis prevalence were higher than published information, perhaps due to the fact that the HCUCH (Clinical Hospital of Universidad de Chile) is a reference center. Both anomalies showed a significant increase in their birth prevalence rates when compared with those previously obtained by us in the same hospital. Significant differences between these two entities were described. Omphalocele presented higher stillbirth, mortality and fatality rates; higher frequency of associated malformations and higher average maternal age.

Onfalocele, gastrosquisis: epidemiología, supervivencia y mortalidad en el Hospital Nacional Daniel A. Carrión, durante el periodo 2010-2013 / Omphalocele, gastroschisis: epidemiology, survival and mortality in National Hospital Daniel A. Carrion, during the period 2010-2013

Introducción: Los defectos de la pared abdominal anterior (onfalocele y gastrosquisis) conforman un espectro de malformaciones anatómicas estructurales de etiología diversa con severidad y pronóstico variable. Su incidencia es de 1:3000 a 5000 recién nacidos vivos. En los últimos años se ha evidenciado un aumento en la prevalencia de gastrosquisis, asociado con una creciente proporción de madres adolescentes y a nuevos factores etiológicos como exposición a teratógenos específicos y deficiencias nutricionales. Objetivo: Conocer las características clínicas y epidemiológicas de recién nacidos con onfalocele y gastrosquisis; y los resultados del manejo de estos pacientes, en un hospital nacional. Material y métodos: Se analizaron las historias clínicas de 15 neonatos en total, 12 con gastrosquisis y 3 con onfalocele, atendidos en el hospital en un lapso de tres años. Resultados: La prevalencia de los defectos de pared abdominal fue 14.67/10,000 nacidos vivos, con una frecuencia relativa de 2.97 y 11.90/10,000 nacidos vivos para onfalocele y gastrosquisis respectivamente. Los niños con gastrosquisis pesaron al nacer 2367.9±491 g y los de onfalocele 2040±1712 g; y la edad de gestación fue similar, 8 fueron sometidos a cesárea, mientras que 7 a parto vaginal; la permanencia en UCIN fue de 16 días en promedio, 9 para los casos de onfalocele y 18.6 para gastrosquisis. Siete murieron: cinco con gastrosquisis y dos con onfalocele. Conclusiones: Los factores asociados a estas enfermedades fueron similares a lo informado por otros. La mortalidad es aún alta por lo que se hacen cambios en el manejo de estos neonatos.

Introduction: Abdominal wall defects (omphalocele and gastroschisis) represent a spectrum of structural anatomical malformations with several etiology and variable prognosis. The incidence is 1: 3000 to 5000 live births. In the last years has been detected an increase of the prevalence, associated to an increasing proportion of teenage mothers and to new etiologic factors like exposition to specific teratogens and nutritional deficiencies. Objective: To know the clinical features and the neonatal survival of infants with gastroschisis and omphalocele in a general hospital. Subjects and methods: The hospital files of 12 neonates with gastroschisis and three with omphalocele during three years were review. Results: The prevalence of abdominal wall defects was 14.67/10,000 live births, with a relative frequency of 2.97/10,000 live births for omphalocele and 11.90/10,000 live births for gastroschisis. The birth weight of patients with gastroschisis was 2367.9±491 g and the other with omphalocele was 2040±1712 g they have similar gestational age. There were 8 cesarean section, whereas 7 vaginal delivery; stay in PICU were 16 days, 9 for the cases of omphalocele and 18.6 for gastroschisis. Seven of these neonates died, five with gastroschisis and two with omphalocele. Conclusions: The features of the neonates studied were similar to other reported. The mortality is still high but we are realized measures to respect.

[Morbidity and mortality in newborns with omphalocele and gastroschisis anterior abdominal wall defects]. / Morbilidad y mortalidad en recién nacidos con defectos de pared abdominal anterior (onfalocele y gastrosquisis).

OBJECTIVE: To identify morbidity and mortality in newborns with congenital defects of the anterior abdominal wall. DESIGN: Descriptive, comparative, and retrospective study. PATIENTS: Thirty nine patients with gastroschisis and 26 patients with omphalocele. RESULTS: Median size of the defect in the gastroschisis group was 4 cm. Infants underwent primary closure en 41% of cases. Post-surgical morbidity occurred in 74% of patients with sepsis the main complication in 61.5%. A total of 16.2% died mainly due to acute renal failure and sepsis. In the omphalocele group, median size of defect was 5.5 cm. Primary closure was done in 65% of patients. Complications occurred in 65% of newborns, sepsis was the most frequent complication (46%). Mortality rate was 16.6% related to acute renal failure and cardiogenic shock. CONCLUSIONS: The main causes of morbidity in the two groups were infections and acute renal failure. Mortality rate was similar to that reported in the world literature for gastroschisis and slightly lower for omphalocele.

Defectos de la pared abdominal / Abdominal wall abnormalities

Se analizan 44.736 nacimientos consecutivos, atendidos en la maternidad del Hospital Clínico de la Universidad de Chile, entre 1983 y 1995. La incidencia de Defectos de la Pared Abdominal fue de 4,24 por 10.000, siendo de 3,57 para Onfalocele y de 0,67 para gastrosquisis. La sobrevida fue de 66 por ciento para Onfalocele y de 100 por ciento para gastrosquisis; esta diferencia se debió a la mayor incidencia de malformacines incompatibles con la vida en la primera patología. Se concluye que los avances en las técnicas quirúrgicas, el mejor manejo del feto y recién nacido y el mejor diagnóstico prenatal han permitido una mejor sobrevida de estos pacientes. El tamaño y posición de los defectos de la pared anterior del abdómen, su contenido y asociación con otras anomalías son hechos que pueden ser diagnosticados in útero con ultrasonido y permiten hacer un diagnóstico diferencial prenatal, que es extremadamente importante para el manejo del paciente

Gastrosquise e onfalocele: revisäo de 16 casos e uma proposta de tratamento / Gastroschisis and omphalocele: review of 16 cases and a proposal of treatment

Oito casos com gastrosquise e oito com onfalocele foram tratados em um período de 38 meses (março/88 a abril/91) no Hospital Materno-Infantil Presidente Vargas. Quatro pacientes com onfalocele (50 por cento) e outros quatro com gastrosquise (50 por cento) nasceram de parto cesáreo. Apenas l caso de cada patologia teve diagnóstico pré-natal. Em 25 por cento de cada patologia estiveram presentes outras malformaçoes associadas. Seis RN com onfalocele (75 por cento) e 5 com gastrosquise (62,5 por cento) sobreviveram. Ventilaçao mecânica (VM) no pós-operatório (PO) foi usada em 5 casos de onfalocele e 6 de gastrosquise. O início da via oral nos RN que sobreviveram variou de 5 a 20 dias (onfalocele) e 15 a 18 gastrosquise), enquanto que o tempo de hospitalizaçao durou 6 a 34 dias (onfalocele) e 19 a 31 dias (gastrosquise). Nossa experiência e de outros autores sugerem que (l) o parto vaginal é o de escolha para malformaçoes da parede abdominal anterior, (2) a hidrataçao no pré-operatório deve ser 2 vezes a manutençao, (3) no tratamento cirúrgico a preferência é pelo fechamento fascial primário (FFP), (4) o uso da VM no PO segue as indicaçoes usuais (5), a nutricao parenteral total deve ser usada precocemente.

The pattern of gastro-intestinal obstruction in Trinidadian children

A review is presented of 156 children admitted to hospital with gastro-intestinal obstruction. The common underlying causes were obstructed hernia (46.8 percent), intussusception (23.7 percent) and pyloric stenosis (16 percent) of cases. There was a marked male proponderance in each of these three groups, and the predominance of patients of African extraction in the hernia group reached 100 percent when the hernia was umbilical. Well-known observations in children with obstructed inguinal hernias which were confirmed were a right-sided prediliction, and a tendency for tecurrent obstruction in the absence of early repair. All of the patients with intussusception were under the age of one year, and this was associated with an unusually low incidence of discomfort or irritability as a presenting complaint, and a paucity of anatomical lead points. Fifteen bowel resections were required in 14 children, seven for gangrenous or irreducible intussusceptions. There were nine fatalities, four in the group with intussusception, and the details of these suggest that all fatalities could be eliminated with improvements in respiratory care, and pre-and intra-operative management (AU)

Congenital defects of the abdominal wall. A review of the experience in New Mexico.

Omphalocele, umbilical cord hernia, and gastroschisis are surgically correctable defects of the abdominal wall. Each of these defects has a distinct embryologic basis that results in a characteristic clinical picture. Twenty-five infants with congenital defects of the abdominal wall were treated at the University of New Mexico Hospital in the past four years. Six infants had omphalocele, one had umbilical cord hernia, and 18 had gastroschisis. Survival among infants who underwent a corrective operation was as follows: omphalocele, 50%; umbilical cord hernia, 100%; and gastroschisis, 82%. Long-term survival for the entire group was 72% (18/25). Gastroschisis, which had a lower incidence of major associated anomalies, had a better prognosis than omphalocele. The mortality of congenital abdominal wall defects was related to presence of severe associated anomalies and to poor clinical condition on admission. Prompt and informed initial care may increase the chance of survival.