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.

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.

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.

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.

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.

Past and present in omphalocele treatment in Romania.

BACKGROUND: Omphalocele is a congenital abnormality whose prognosis has improved significantly over the last few decades, reaching a survival rate of 80-90% in developed countries. Currently, in Romania no comprehensive study on the incidence, treatment, and survival of patients with this defect of the anterior abdominal wall has been carried out. METHODS: This retrospective analytical study was conducted over a period of 23 years and included 105 children with omphalocele. Prenatal diagnosis, referral to our hospital, children age upon admission, associated diseases, medical and surgical management, early and late postoperative complications, and the length of hospital stay were analysed. RESULTS: The low rate of antenatal diagnosis (13.3%), the high frequency of associated congenital malformations (71.4%) and chromosomal abnormalities (27.6%), inadequate and delayed transport to a specialized pediatric surgery center together with an increased rate of sepsis (37.1%)resulted in a high mortality rate (54.3%). CONCLUSIONS: The significantly reduced length of hospital stay and higher survival rate despite the apparently more frequent medical complications plead for the surgical treatment of omphalocele whenever not contraindicated by the presence of severe pulmonary hypoplasia, cardiac defects, immaturity and other severe congenital anomalies, when conservative treatment is indicated.

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.

Umbilical hernia repair in patients with signs of portal hypertension: surgical outcome and predictors of mortality.

OBJECTIVES: To compare the outcomes of umbilical hernia repair in patients with and without signs of portal hypertension, such as esophageal varices or ascites; to assess the effect of emergency surgery on complication rates; and to identify predictors of postoperative mortality. DESIGN: Database search from January 1, 2005, through December 31, 2009. SETTING: North American hospitals participating in the American College of Surgeons National Surgical Quality Improvement Program initiative. PATIENTS: We studied patients who underwent umbilical hernia repair. Those with congestive heart failure, disseminated malignant tumor, or chronic renal failure while undergoing dialysis were excluded. MAIN OUTCOME MEASURES: Preoperative variables and perioperative course were analyzed. Main outcome measures were morbidity and mortality after umbilical hernia repair. RESULTS: A total of 390 patients with ascites and/or esophageal varices formed the study group, and the remaining 22 952 patients formed the control group. The overall morbidity and mortality rates for the study group were 13.1% and 5.1%, whereas these rates were 3.9% and 0.1% for the control group, respectively (P < .001). For the study group, the mortality after elective repair among patients with a model for end-stage liver disease (MELD) score greater than 15 was 11.1% compared with 1.3% in patients with a MELD score of 15 or less. The patients with ascites and/or esophageal varices underwent emergency surgery more frequently than the control group (37.7% vs 4.9%; P < .001). Emergency surgery for the study group was associated with a higher morbidity than elective surgery (20.8% vs 8.3%; P < .001) but not a significantly higher mortality (7.4% vs 3.7%; P = .11). However, logistic regression analysis showed that age older than 65 years, MELD score higher than 15, albumin level less than 3.0 g/dL (to convert to grams per liter, multiply by 10), and sepsis at presentation were more predictive of postoperative mortality. CONCLUSIONS: Umbilical hernia repair in the presence of ascites and/or esophageal varices is associated with significant postoperative complication rates. Emergency surgery is associated with higher morbidity rates but not significantly higher mortality rates. Elective repair of umbilical hernia should be avoided for those with adverse predictors, such as age older than 65 years, MELD score higher than 15, and albumin level less than 3.0 g/dL.

Omphalocele, gastroschisis: epidemiology, survival, and mortality in Imam Khomeini hospital, Ahvaz-Iran.

UNLABELLED: Gastroschisis and omphalocele are the most common malformation of the anterior abdominal wall.The aim of the study was to determine the abdominal wall defect frequencies, survival, and mortalities in Ahvaz, Khuzestan province of Iran.Material and methods. All cases born with omphalocele or gastroschisis whom born in Imam Khomeini hospital, were included in this study. Duration of study was 3 years from April 2005. All patients treated at Imam Khomeini hospital in Ahwaz, Iran.Results. Among 15321 consecutive births, 42 patients had abdominal wall deformity. Overall incidence was 27.41 per 10,000 live births. Of all cases, 18 (42.9%) of cases were male and 24 (57.1%) were female. Of all cases, 21.7% of patients with omphalocele and 10% patients with gastroschisis had other anomalies. Of all cases, 71.8% of patients with omphalocele and 60% with gastroschisis underwent surgery. The type of anomaly (omphalocele and gastroschisis) had correlation with post operation prognosis significantly (p<0.001). Of 66.7% of patients under went surgery, 46.4% with mesh and 53.6% without mesh performed. 80% of patients with omphalocele and 20% with gastroschisis were lived. CONCLUSIONS: In our study, mortality was significantly higher in cases with gastroschisis than cases with omphalocele.

Delayed management of giant omphalocele using silver sulfadiazine cream: an 18-year experience.

BACKGROUND/PURPOSE: To assess the value of topical silver sulfadiazine (SSD) cream in the treatment of babies with a giant omphalocele. METHODS: From 1991 to 2008 inclusive, 20 infants with giant omphalocele (defined as >10 cm diameter) were treated with SSD, leaving a large ventral hernia to be repaired at a later date. RESULTS: There were 12 boys and 8 girls. Thirteen had prenatal ultrasound diagnosis at a mean gestational age of 23 weeks. The mean gestational age at delivery was 37 weeks, and mean birth weight was 2.5 kg. Nineteen had other anomalies and/or medical problems, 18 of them multiple. The most common was pulmonary hypoplasia (70%). Mechanical ventilation and/or oxygen treatment was required in 15 (75%) for a mean of 10 weeks. SSD was used as primary sac treatment in 5 and secondary treatment in 15 (after Silon pouch 11, Op-site 3, povidone-iodine 1). Six omphalocele sacs were ruptured within the first 5 days of life. SSD was used for a mean of 6 months at a cost of $1 per day. Complications included 2 instances of staphylococcal sepsis and 1 jejunal perforation inside a Silon pouch. Six (30%) died from pulmonary hypoplasia at a mean age of 18 weeks. There were 14 (70%) survivors who went home after a mean of 14 weeks. Of the 14 survivors, 12 had ventral hernias repaired (18 operations with 2 recurrences), and 2 remain with their original ventral hernia. CONCLUSIONS: Initial topical coverage with SSD is associated with excellent outcomes for infants with giant omphalocele who cannot undergo immediate closure.

[Liver torsion leading to death in a 16-month-old infant treated neonatally for an omphalocele]. / Volvulus du foie d'évolution fatale 16 mois après la cure d'une omphalocèle géante.

We report the case of an infant who underwent surgery on the first day of life for a giant omphalocele. At the age of 16 months, he presented an acute abdominal syndrome and died a few hours later. Autopsy revealed a twisted left liver lobe (LL) including a suprahepatic vein. To our knowledge, this is the first case of giant omphalocele complicated by twisted liver lobe and fatal outcome.

Surgical repair of umbilical hernias in cirrhosis with ascites.

The most common complications of umbilical hernias in patients with cirrhosis and ascites include leakage, ulceration, rupture and incarceration. If such a complication is present, there is a high mortality rate after surgical repair. Elective repair is the most effective choice, as it prevents complications with a lower mortality. However, the control of ascites before and/or after repair is mandatory but may not always be possible with diuretics and paracentesis. Portal decompression by transjugular intrahepatic portosystemic shunt (TIPS) with better control of ascites may allow these patients to undergo surgery. Patients with cirrhosis and umbilical hernias should be referred for consideration of an elective surgical repair with mesh, preferably after optimal management of ascites. There should be a low threshold for placement of a TIPS to facilitate surgery and reduce the chance of severe recurrence of ascites. If surgery is contraindicated, a TIPS must be considered for control of ascites.

[Intrapartum operation on fetuses with birth defects and its outcome].

OBJECTIVE: To discuss the value of intrapartum operation in management of birth defects and the prognosis. METHODS: From August 2008 to November 2009, 11 fetuses were identified with birth defects through 3D color Doppler ultrasound and confirmed by MRI and fetal karyotype in the Maternal Fetal Medicine Center, Affiliated Shengjing Hospital, China Medical University including three lymphangiomas, two congenital diaphragmatic hernias (CDH), one sacrococcygeal teratoma, three omphalocele and two gastroschisi. All the above identified birth defects were indications for surgery. All fetuses were born abdominally and received intrapartum operations, including three intrapartum fetal operations with placental infusion (two repairs of CDH, one sacrococcygeal teratoma resection), six ex-utero intrapartum treatment (EXIT; two repairs of omphalocele, two repairs of gastroschisi, two lymphangioma resection) and two surgeries in house (one omphalocele repair and one lymphangioma resection). Both the mothers and fetuses were regularly followed up. RESULTS: (1) OPERATIONS: the average operating time for the three intrapartum fetal operations was 89 minutes, 5.5 minutes for the six EXIT, during which EXIT was performed first, followed by blocking the umbilical circulation and neonatal surgery, and 37 minutes for the two surgeries in house. All neonates survived except for one death from severe CDH at 3.5 hours after the operation. The average blood loss for cesarean section and fetal operation was 275 ml. All mothers recovered soon without fever or infection and were discharged three to five days after the operation. (2) Follow-ups: the ten survived neonates were followed up at 1-18 months at the pediatric clinics and all were growing and developing normally except for one baby with gastroschisi suffered from enteral torsion and feeding intolerance showed lower weight than babies at the same age, but caught up to normal at four months old after posture therapy. One baby with mild CDH developed pulmonary infection at two months after operation with 1/4 pneumothorax on chest X-ray, and were hospitalized for two weeks. At six months old, patent ductus arteriosus was diagnosed in the same baby and chest X-ray was normal. The baby with omphalocele was complicated with ventricular septal defect before operation and the cardiac function was normal during follow-ups for one year. The baby with sacrococcygeal teratoma was reported to have no automatic micturition, but recovered to normal at one month of age. CONCLUSION: Babies with certain birth defects can be managed through intrapartum operation with better outcomes.

Neonatal care in patients with giant ompholocele: arduous management but favorable outcomes.

OBJECTIVES: The objectives of the study were to provide a review of patients with giant omphalocele managed in a single institution (2001-2006), focusing on medical management in the neonatal period, and to evaluate short-term outcomes. METHODS: Data from 14 neonates with giant ompholocele (abdominal wall defect >5 cm and/or containing liver) and the absence of malformation and chromosomal anomalies during fetal screening were retrospectively reviewed. All were intubated and sedated before surgical treatment. Initial management consisted of progressive reduction of the herniated organs by gentle compression. After sequential reduction, abdominal wall closure was attempted at the skin and fascia level and, when necessary, with a Gore-Tex patch. RESULTS: Median gestational age was 39 weeks (38-40), and median birth weight was 3100 g (2470-3700). Median age at closure was 6 days (0-20). A central Gore-Tex patch was inserted in 10 cases. Median ventilation length was 26 days (2-78). Full enteral diet was achieved after an average of 33 days (8-82), and median time until discharge from the intensive care unit was 24.5 days (11-85). Nine patients developed sepsis in the postoperative course. In 10 patients, at least 1 associated malformation was diagnosed in the postnatal course, among which cardiac and diaphragmatic defects were the most common. Survival rate was 85.7%. CONCLUSION: Mortality rate of giant omphalocele without chromosomal anomaly or major malformations is low when treated by gradual reduction of the contents. Parents should be informed of the long hospitalization in the intensive care unit at birth, the potential nonthreatening associated malformations to be diagnosed after birth, and the high risk of sepsis.

Omphalocele: comparison of outcome following prenatal or postnatal diagnosis.

OBJECTIVES: To assess the impact of prenatal compared with postnatal diagnosis on outcome for liveborn infants with an isolated or with a non-isolated omphalocele. METHODS: This was a retrospective analysis of 101 prenatally and 45 postnatally diagnosed cases of omphalocele. Cases were collected from the ultrasound database of the Division of Obstetrics and Prenatal Medicine and the patient database of the Department of Pediatric Surgery. RESULTS: Following confirmation at delivery or autopsy, prenatally diagnosed omphaloceles included 21 isolated cases, 44 non-isolated cases with a normal karyotype and 36 non-isolated cases with an abnormal karyotype. Of the prenatally diagnosed apparently isolated cases (n = 31), 12 (39%; 95% CI, 22-58%) revealed associated anomalies after delivery. Liveborn infants with an isolated omphalocele had significantly worse short-term morbidity following prenatal diagnosis (n = 14) compared with diagnosis at birth (n = 29), having a lower gestational age at delivery, lower Apgar scores, longer duration of ventilation and parenteral nutrition, more readmissions and a longer hospital stay. The prenatally diagnosed subset contained more infants with a giant omphalocele (9/14 vs. 3/29, P = 0.001) and liver herniation (8/14 vs. 6/29, P = 0.02). The outcome of liveborn infants with a non-isolated omphalocele diagnosed prenatally (n = 17) was not different from that of those diagnosed at birth (n = 16), except for a greater need for ventilation and parenteral nutrition in the prenatal subset. CONCLUSION: When counseling patients with a prenatal diagnosis of isolated omphalocele, it is important to remember that over one third could turn out to have associated anomalies. Liveborn infants with an isolated omphalocele detected prenatally have worse short-term morbidity than do cases detected at birth. Those with non-isolated omphaloceles detected prenatally have an increased need for ventilation and parenteral nutrition compared with those detected at birth.