A rare case of neonatal intestinal obstruction

Omphalocele minor is often associated with the presence of other anomalies compared to that of omphalocele major. The occurrence of intestinal atresia is seldom associated with omphalocele minor. Prompt diagnosis and intervention can prevent morbidity and mortality. Following is a rare case report of combination of omphalocele minor and intestinal atresia with strangulation of the proximal bowel.

Craniocerebral and Spinal Dysraphism with Omphalocele – A Case Report of Primary Neurulation Defect

Background: Anencephaly and Spina bifida are the two most common types of neural tube defects (NTDs). Disrupted formation and closure of neural folds leads to Craniocerebral and spinal dysraphisms. Materials and methods: An 18 week old foetus was received in the department of Anatomy after elective medical termination of pregnancy due to the diagnosis of neural tube defect and associated congenital anomalies. Case Report: The foetus had anencephaly, thoracic-lumbar spina bifida, omphlocele and clubbed foot and hands. The foetus was dissected and studied, to look for abnormal internal structures. On meticulous dissection it was found that there were abnormalities in spine, gastrointestinal system and cranium. Conclusion: Anencephaly is a neural tube defect which has multiple neural and non-neural associated anomalies. A detailed description of the combination of associated anomalies goes a long way in updating knowledge on the same.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Divertículo de Meckel en asociación a onfalocele y tetralogía de Fallot: reporte de caso y revisión de la literatura / Meckel's diverticulum in association with omphalocele and tetralogy of Fallot: Case report and literature review

El onfalocele o exónfalos se definen como un defecto congénito de la pared abdominal, que consiste en la herniación de las vísceras abdominales a través del anillo umbilical. Esta entidad rara vez se asocia a la comunicación del divertículo de Meckel con el saco del onfalocele. Teniendo en cuenta la escasa prevalencia de dicha entidad, compartimos el reporte de caso de un paciente recién nacido, con diagnóstico de onfalocele menor, en quien se sospechaba ruptura del saco, sin embargo, de manera intraoperatoria se encontró que la aparente ruptura del saco, correspondía a la comunicación con un divertículo de Meckel. El caso además se asoció con hallazgos ecocardiográficos de tetralogía de Fallot

Omphalocele or exomphalos are defined as a congenital defect of the abdominal wall, which consists of the herniation of the abdominal viscera through the umbilical ring. This entity is rarely associated with the communication of Meckel's diverticulum with the omphalocele sac. Considering the low prevalence of this entity, we report the case of a newborn with a diagnosis of minor omphalocele, in whom rupture of the sac was suspected; however, intraoperatively it was found that the apparent rupture of the sac corresponded to a communication with a Meckel's diverticulum. The case was also associated with echocardiographic findings of tetralogy of Fallot

Bloqueo caudal más anestesia general balanceada en un paciente con pentalogía de Cantrell, presentación de un caso clínico / Caudal block plus inhaled general anesthesia in a patient with pentalogy of Cantrell, presentation of a clinical case

The pentalogy of Cantrell is a disorder characterized by congenital abnormalities in the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and cardiac anomalies. It is a rare disease with 250 cases registered around the world. The anesthetic implications will require a specialized management given the ventilatory mechanics and cardiac function which are compromised by the disease in the newborn. We present the case of a female patient with pentalogy of Cantrell without prenatal diagnosis, who had an operative procedure to correct patent ductus arteriosus and abdominal mesh placement under balanced general anesthesia with sevoflurane and fentanyl plus caudal block. This case is reported to provide our experience in the anesthetic management of this type of patients.

La pentalogía de Cantrell es una enfermedad caracterizada por anormalidades congénitas de la pared abdominal supraumbilical, esternón inferior, diafragma, pericardio diafragmático y anomalías cardiacas. Se trata de una enfermedad rara con 250 casos registrados alrededor del mundo. Las implicaciones anestésicas requieren de un manejo especializado debido a la mecánica ventilatoria y función cardíaca que se encuentran comprometidas en el recién nacido. Se presenta el caso de una recién nacida portadora de pentalogía de Cantrell, no diagnosticada prenatalmente, quien fue sometida a corrección de ductus arterioso persistente y colocación de malla abdominal bajo anestesia general balanceada con sevofluorano y fentanilo más bloqueo caudal. Se reporta el presente caso para brindar nuestra experiencia en el manejo anestésico de este tipo de pacientes.

Antenatal diagnosis of omphalocele and spinal dysraphism: a possible OEIS complex

OEIS complex, which comprises of omphalocele, exstrophy bladder, imperforate anus and spinal dysraphism, is a rare disorder. The prognosis depends upon the severity of the structural defects. Survival depends on the extension of the cloacal exstrophy and the neural tube defect. The antenatal diagnosis in early pregnancy is very tough, and a high level of suspicion should be kept for the possibility of its existence, even only if omphalocele and spinal dysraphism are seen on a prenatal sonography. Authors present the case of a 33-year-old, G1P0 female, who on her prenatal routine sonography scan, was found to have a fetus with omphalocele and spinal dysraphism. Despite the repeated attempts to follow her up, she refused and hence the possibility of the presence of other associated malformations could not be ascertained. Considering the difficulty of antenatal diagnosis of OEIS complex and its associated mortality, the objective of this case report is to discuss the vital sonographic findings of OEIS, along with its etiology and prognosis, which can aid in its early detection and subsequently help parents to make a decision regarding the continuation of pregnancy.

Bronchogenic cyst presenting as content of omphalocele – A case report

Bronchogenic cyst (BC) is a very rare congenital anomaly occurring due to budding of the primitive foregut, and its common location is the posterior mediastinum. BC when diagnosed prenatally can be treated if it is encroaching on the development of lungs. BC has been reported in other locations such as cervical, thoracic, abdominal sites and also as subcutaneous lesions. Omphalocele is a congenital malformation occurring due to a central defect in the abdominal wall with herniation of the viscera. The nonentity documented here was found in a female fetus with 20 weeks of gestational age. The mother was a primigravida who had antenatal ultrasound scan rendering diagnosis of a live fetus having abdominal wall defect with omphalocele. This case is exceptionally rare as the content of omphalocele was BC having a classical wall lined by pseudostratified ciliated columnar epithelium overlying band-like cartilage. The extensive search in the literature did not reveal another similar case.

Hernia of the umbilical cord: a clinical spectrum

Background: Hernia of the umbilical cord is a rare clinical entity which presents with hernia of the small bowel into the proximal part of the umbilical cord. This is sporadically associated with other congenital malformations. This is usually poorly identified and mistakenly termed as 'omphalocele minor. Inadvertent clamping of the cord in these cases leads to iatrogenic bowel wall injury. The aim of this study is to present a spectrum of cases presenting with umbilical cord hernia in a tertiary care Govt medical college, along with demography, intra operative findings, associated malformations and postoperative outcome and 1 year follow up.Methods: This is an ambispective study on neonates who attended the Outpatient Department (OPD) or Emergency department. The babies were evaluated by age, birth weight, gender, any gross clinical malformation. They were also evaluated intraoperatively for any associated anomalies. Post-surgery the babies were followed up until 2 years of age in pediatric surgery OPD of the same institute and outcome was recorded.Results: Out of 90 babies, 88 babies (97.77%) had body weight more than 2.5 kg, and rest 2(2.22%) had bodyweight of 1.5kg and 1.8 kg. Regarding content of contents of umbilical cord hernia, out of 87 patients, 43 patients (49.42%) had ileal loop as, 32 patients (36.78%) had ileum with Meckel's diverticulum, 6 patients (6.39%) had cecum with appendix.Conclusions: Most of this study cases had ileal loops as content of the hernial sacs, and 36.78% cases had Meckel's diverticulum associated, which is a remnant of Vitello intestinal duct. Prompt identification of the condition and early intervention and adequate post-natal care are affective to prevent long term morbidity.

The effects of multi-disciplinary team management on the outcome in neonates with omphalocele / 中国新生儿科杂志

Objective To study the effect of multi-disciplinary team (MDT) management on the outcome in neonates with omphalocele.Method A retrospective non-randomized controlled clinical study was conducted.Neonates who were diagnosed as omphalocele and admitted to the surgical neonatal intensive care unit of the Guangzhou Women and Children Medical Center from December 2010 to December 2017 were collected.Because MDT was established in December 2014,infants were assigned into non-MDT group and MDT group according to their dates of admission.The characteristics and outcomes between non-MDT group and MDT group were compared using x2,t-test or rank-sum test.Multivariate analysis was performed by Logistic regression.Result A total of 91 neonates were included in the study,50 were in non-MDT group and 41 were in MDT group.The mortality in MDT group (2.4％,1/41) was lower than that in non-MDT group (18.0％,9/50),the difference was statistically significant (P ＜ 0.05).The median time of mechanical ventilation of giant omphalocele in non-MDT group (18.3 hours) was longer than that in MDT group (41.7 hours),the difference was also statistically significant (P ＜ 0.05).After adjusting for the associated confounding risk factors,the risk of death in non-MDT group was 54 times higher than that in MDTgroup (OR=54.19,95％CI2.64 ～1 113.49,P＜0.05).Conclusion There was significant association between the MDT management and the decreased risk of death of omphalocele.

Discharge Outcomes of Liveborn Infants With Omphalocele (Isolated Vs Non-isolated)

@#Introduction: The mortality rate due to omphalocele cases remains high. The presence of other congenital anomalies is believed to be one of the factors causing death in patients with omphalocele. The objective of the study was to determine the influence of other congenital anomalies leading to mortality. Methods: We conducted a retrospective cohort study in the neonatal unit of Sardjito General Hospital from March 2008-April 2019. We collected data on factors including sex, mode of delivery, gestational age, birth weight, associated anomalies, management, neonatal complications, need for surgeries, and mortality from our registry and patients’ medical records. We then classified the infants into the isolated omphalocele and non-isolated omphalocele groups. Univariate and multivariate analyses were performed to determine the association of congenital anomalies and other confounding factors with mortality. Results: We identified 73 omphalocele cases during the study period. The hospital occurrence was 4.3/1000 livebirths. We found 45 cases (61.6%) of omphalocele associated with other congenital anomalies including several syndromes (46.7%) such as Patau syndrome and Beckwith-Wiedemann syndrome, congenital heart defects (42.2%), gastrointestinal anomalies (22.2%), neural tube defects (15.6%), cleft palate (11.1%), and syndactyly (0.07%). There was no difference in mortality (25/45 vs 15/28) between the groups (p=0.87). Interestingly, sepsis, pneumonia and gastrointestinal perforation were significantly associated with the survival of infants with omphalocele, with p-value of 0.05, 0.00, and 0.05, respectively. Conclusion: Certain complications might have an impact on survival, but the presence of other congenital anomalies was not significantly associated with the mortality of omphalocele cases.

Lower Midline Syndrome with Undescended Testes in the Tobacco Agricultural Environment: A Case Report

@#Lower midline syndrome is a rare congenital anomaly associated with omphalocele. Undescended testes is a congenital absence of one or both testes in the scrotum. Both of lower midline syndrome and undescended testes incidence have a positive correlation with exposure of cigarette and pesticide. Tobacco agricultural environment is a vulnerable area exposed by pesticides and cigarettes. This is the case report to associated tobacco agricultural environment’s exposure with lower midline syndrome and undescended testes. We describe a case of lower midline syndrome and undescended testes that occurs in the tobacco agricultural environment. In this case we found the exposures of paternal heavy smoker, second-hand smoker, pesticides from tobacco agricultural environment and daily use of bug spray in the house. As conclusion, the exposures from tobacco agricultural environment may take a contribution to lower midline syndrome and undescended testes incidence.

Apert Syndrome Presenting with Omphalocele

Apert syndrome is a congenital acrocephalosyndactylysyndrome. It is mainly presented by craniosynostosis,syndactyly of the hands and feet and dysmorphic facialfeatures. The condition has an autosomal dominantinheritance assigned to mutations in the FibroblastGrowth Factor Receptors (FGFR-2) gene. The reportedcase is a 9 months old boy with clinical suspicion ofApert syndrome as he had turricephaly, stubby handswith short fingers, omphalocele measuring 10 × 10 cm,bilateral undescended testes, Congenital TalipesEquinovarus (CTEV) and polydactyl and syndactyl ofgreat and second toes. He was developmentallynormal. The omphalocele was surgically repaired.Early diagnosis and intervention helps rehabilitation ofpatients with Apert syndrome and help them to lead abetter life.

Analysis of 2 cases of fetal congenital omphalo-enteric fistula with intestinal prolapse diagnosed by prenatal ultrasonography / 中国实用妇科与产科杂志

OBJECTIVE: To investigate the prenatal ultrasonographic manifestations of fetal congenital omphalo-enteric fistula with intestinal prolapse and improve the understanding and diagnosis of this disease.METHODS: A retrospective analysis of the prenatal ultrasonographic features was performed in 2 cases of fetal congenital omphalo-enteric fistula with intestinal prolapse comnfirmed after surgery between July 2016 and September 2017 in First Affiliated Hospital of Fujian Medical University,and the ultrasonographic manifestations were compared with postnatal looks and intraoperative conditions.Characteristics of prenatal ultrasonographic manifestations were summarized.RESULTS: Both cases were misdiagnosed as omphalocele by prenatal ultrasonography.Congenital omphalo-enteric fistula with intestinal prolapse was verified by surgery after delivery.A narrow pedicle,special bowel-shape and incomplete capsule were its prenatal ultrasonic characteristics.CONCLUSION: Fetal congenital omphalo-enteric fistula with intestinal prolapse is rare,but has particular prenatal ultrasonographic manifestations. Strengthening the understanding of the disease,reducing misdiagnosis and improving prenatal ultrasound diagnosis will provide help for prenatal counseling and clinical choice for time of delivery.

Prevalencia al nacimiento de gastrosquisis y onfalocele en Costa Rica / (Prevalence of gastroschisis and omphalocele in Costa Rica)

Resumen Justificación: En varios países se ha documentado un aumento de los defectos congénitos de pared abdominal, principalmente gastrosquisis. El objetivo es conocer la tendencia por edad materna de gastrosquisis y onfaloce en Costa Rica, durante 1996-2014. Métodos: Utilizando la base de datos del sistema nacional de vigilancia de defectos congénitos, se calcularon tasas de defectos congénitos de pared abdominal por edad materna (menores de 20, 20-34, 35 años o más), y período (1996-2002, 2003-2008 y 2009-2014) con sus respectivos IC 95%. Se realizó una regresión de Poisson, tomando como base el grupo 20-34 años y el período 1996-2002 y se compararon estimados mediante chi cuadrado de Wald. Se evaluó el impacto de la EM mediante fracción atribuible poblacional y se calcularon tasas de fecundidad y distribución proporcional de los nacimientos de acuerdo a esta variable. Resultados: La prevalencia de defectos congénitos de pared abdominal aumentó de 0,76 (IC95% 0,15-1,36) por 10 000 nacimientos en 1996 a 3,48 (IC95% 2,12-4,85) en el 2014. El riesgo relativo para gastrosquisis fue 3,76 (2,95-4,79) en madres menores de 20 años y la fracción atribuible poblacional 36,2%. El riesgo relativo para onfalocele fue 2,09 (1,22-3,59) en madres mayores de 35 años y la fracción atribuible poblacional 8,9%. Se documentó una disminución proporcional de los nacimientos y la tasa de fecundidad en madres menores de 20 y mayores de 35. Conclusión: La prevalencia de defectos congénitos de pared abdominal ha aumentado significativamente a través del tiempo. El aumento de gastrosquisis en las madres menores de 20 años fue mayor, a pesar que los nacimientos han disminuido en este grupo de edad.

Abstract Background: In several countries, an increase in abdominal wall defects, mainly gastroschisis, has been documented. The objective is to know the trend of gastroschisis and omphaloce by maternal age in Costa Rica, between 1996 and 2014. Methods: using the database of the national congenital defects surveillance system, congenital abdominal wall defects rates were calculated by maternal age (under 20, 20-34, 35 years or more), and period (1996-2002, 2003-2008 and 2009-2014) with their respective 95% CI. A Poisson regression was carried out, taking as a base the group 20-34 years and the period 1996-2002, and were compared estimated by Wald's chi-square. The impact of MS was evaluated by population attributable fraction and fertility and proportional distribution of births were calculated according to this variable. Results: abdominal wall defects prevalence increased from 0.76 (95% CI 0.15-1.36) per 10,000 births in 1996 to 3.48 (95% CI 2.12-4.85) in 2014. Relative risk for gastroschisis was 3.76 (2.95- 4.79) in mothers younger than 20 years and the population attributable fraction 36.2%. The relative risk for omphalocele was 2.09 (1.22-3.59) in mothers older than 35 years and population attributable fraction 8.9%. There was a decrease in the proportional distribution of births and the fertility rate in mothers under 20 and over 35. Conclusion: the prevalence of congenital abdominal wall defects has increased significantly over time. The increase in gastroschisis in mothers under 20 years was greater, despite the fact that births have decreased in this age group.

Omphaloceles in combination with a self-made hemispherical cushion: a report of 12 cases

The aim of this study was to discuss the curative effect of applying "capsule-reserved normal saline bag and self-made hemi-spherical cushion oppression" for treating giant omphaloceles. Twelve patients with giant omphaloceles who were admitted to our hospital between January 2008 and June 2016 were selected for treatment as follows: a capsule-reserved normal saline bag was used to promote the gradual return of the abdominal contents into the abdominal cavity in phase I, and a self-made hemi-spherical cushion was used for compression combined with a local dressing change in phase II to treat the giant omphaloceles without surgical treatment. All 12 patients in this group were cured, and after follow-up visits for >10 months, they had no abdominal infections, wound disruption, intestinal obstruction, or other complications, and their growth was normal. Two patients had abdominal hernias, and they recovered after herniorrhaphies. Giant omphaloceles in newborns were treated in stages, and in phase II, non-surgical treatment was applied, which was easily performed with a smaller wound, low cost, an obvious curative effect, and higher safety and effectiveness.

Prenatal ultrasound in diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex in first trimester / 中国医学影像技术

Objective To explore the value of prenatal ultrasound in diagnosis of omphalocele-exstrophy-imperforate anusspinal defects (OEIS) in first trimester.Methods Prenatal ultrasonic characteristics of 10 fetuses with OEIS complex in first trimester were retrospectively analyzed and compared with autopsy results.Results Cystic bulging in the lower anterior abdominal wall was observed in all 10 fetuses.Spinal scoliosis dysplasia was found in 10 fetuses,with myelomeningocele in 3 fetuses.No normal bladder was visualized in 8 fetuses.Thickened nuchal translucency was noticed in 5 fetuses,among which neck lymphatic hydrocele was found in 1 fetus.The bilateral clubbed feet and left lower mutilation was observed in 1 fetus,respectively.All 10 OEIS complex fetuses were found accompanied with short umbilical cord,while single umbilical artery and umbilical cord cyst were found in 4 and 1 fetus,respectively.Autopsy showed abdominal wall defects with exstrophy in 10 fetuses.However,no complete cystic bulging was found.Besides,autopsy also showed pubic symphysis separation and bladder exstrophy in 10 fetuses without obvious genitalia nor anus.Conclusion Cystic bulging in the lower anterior abdominal wall is the most common prenatal ultrasonic characteristic of OEIS complex in first trimester.

Celio-mesenteric trunk associated with giant omphalocele: surgical consequences / Tronco celíaco-mesentérico asociado con onfalocele gigante: consecuencias quirúrgicas

We report the first case of a newborn presenting with a celio-mesenteric trunk variation associated with a giant omphalocele. The celio-mesenteric trunk was unexpected and unseen during the staged surgical closure. After partial reintegration of the liver, the newborn presented refractory hypovolemia with anuria, leading to redo surgery. This procedure revealed ischemia of the liver and necrosis of the entire gastrointestinal tract except the colon. Despite treatment, including liver externalization, the infant did not survive. The autopsy revealed a celio-mesenteric trunk, a rare anomaly characterized by a common origin of the celiac axis and the superior mesenteric artery from the aorta. This association may explain the dramatic consequences of the staged closure procedure. Awareness of the association of celio-mesenteric trunk and omphalocele would allow the surgeon to take extra care during this delicate surgery.

Presentamos el primer caso de un recién nacido que presenta una variación del tronco celíaco-mesentérico asociada con un onfalocele gigante. El tronco celíaco-mesentérico fue inesperado y no se vio durante las etapas del cierre quirúrgico. Después de la reintegración parcial del hígado, el recién nacido presentó hipovolemia refractaria con anuria, lo que condujo a la repetición de la cirugía. Este procedimiento reveló isquemia del hígado y necrosis de todo el tracto gastrointestinal excepto el colon. A pesar del tratamiento, incluyendo la externalización hepática, el bebé no sobrevivió. La autopsia reveló un tronco celíaco-mesentérico, una rara anomalía caracterizada por un origen común del tronco celíaco y la arteria mesentérica superior, a partir de la aorta. Esta asociación puede explicar las dramáticas consecuencias del procedimiento durante las etapas del cierre. El conocimiento de la asociación de tronco celíaco-mesentérico y onfalocele permitiría al cirujano tomar especial cuidado durante esta delicada cirugía.

Partial hepatectomy in a calf with an omphalocele: case report / Hepatectomia parcial em bezerra portadora de onfalocele: relato de caso

An omphalocele is a defect in the closure of the abdominal wall during the embryonic stage, preventing the return of some abdominal organs to the cavity. In stillborn animals, the involvement of the liver in the omphalocele has been reported. The aim of the present study is to report the success of a partial liver resection in a female bovine at two days of age which presented an omphalocele at birth. Surgical intervention was indicated to reduce and suture the abdominal wall. During the surgical procedure, the presence of hard, reddish tissue was noted inside the sac covered by the amniotic membrane, with characteristics consistent with hepatic parenchyma. Due to the impossibility of reducing the contents added to the suggestion of tissue infection, we elected to perform a partial resection. The histopathological examination confirmed that the resected tissue was of the hepatic parenchyma. Antibiotic and systemic anti-inflammatory therapies were performed post-surgery. Fifteen days after surgery, infection was detected at the surgical site in addition to abdominal wound dehiscence; surgical reintervention was performed. On physical examination, carried out 4 months after the second operation, the heifer presented normal development for the species. In conclusion, omphalocele may contain liver. Ectopic liver is an extremely rare condition. Surgical treatment in the presented case focused on umbilicus with exploration of the abdominal cavity appeared to be sufficient.(AU)

A onfalocele é um defeito no fechamento da parede abdominal durante a fase embrionária, não permitindo o retorno de alguns órgãos abdominais à cavidade. O envolvimento do fígado em onfalocele tem sido relatado, porém em animais natimortos. Com o presente estudo, objetiva-se relatar uma ressecção parcial do fígado realizada com sucesso em uma fêmea bovina com dois dias de idade apresentando onfalocele ao nascimento. Foi indicada a intervenção cirúrgica para a redução e rafia da parede abdominal. Durante o procedimento cirúrgico, notou-se, no interior do saco recoberto pela membrana amniótica, a presença de tecido firme, de coloração avermelhada, com características compatíveis com parênquima hepático. Devido à impossibilidade de redução do conteúdo e sugestão de infecção do tecido encontrado, optou-se pela ressecção parcial dele. O exame histopatológico confirmou tratar-se de parênquima hepático o tecido resseccionado. No pós-cirúrgico, realizaram-se terapias antibiótica e antiinflamatória sistêmicas. Após 15 dias do procedimento cirúrgico, foi detectada infecção do sítio operatório e deiscência da sutura abdominal, sendo realizada reintervenção cirúrgica. No exame físico, realizado no animal após quatro meses da segunda intervenção cirúrgica, constatou-se desenvolvimento normal para a espécie. Conclui-se que o fígado pode ser conteúdo da onfalolocele, sendo uma condição extremamente rara. O tratamento cirúrgico no caso apresentado, com exploração da cicatriz umbilical e da cavidade abdominal, foi suficiente.(AU)

Parche de cordón umbilical versus cierre primario convencional en Gastrosquisis y Onfalocele / Umbilical cord patch versus conventional primary closure in Gastroschisis and Omphalocele

La Gastrosquisis y el Onfalocele son los defectos congénitos de la pared abdominal más frecuentes e importantes que afectan al recién nacido. En nuestro país son muy pocos los centros hospitalarios que cuentan con la infraestructura necesaria para atender a estos neonatos. Muchos de ellos tienen que esperar un tiempo valioso para poder ser operados mientras se ubica la institución en donde van a ser recibidos, aumentando el riesgo de Síndrome Compartimental Abdominal (SCA) y sepsis. Objetivo: Comparar los procedimientos quirúrgicos de Parche de Cordón Umbilical versus el Cierre Primario Convencional de la pared abdominal en recién nacidos. Método: En total se analizaron 20 pacientes los cuales fueron divididos en 2 grupos. El Grupo A, pacientes a los cuales se les realizó cierre de pared mediante la utilización de Parche de Cordón Umbilical (10). El Grupo B, pacientes a los cuales se les realizó Cierre Primario Convencional (10). Resultado: No hubo diferencia estadística significativa en cuanto a tiempo de hospitalización y a las complicaciones. La presión intraabdominal (PIA), inicio de la vía oral y la sobrevida si mostraron diferencia estadística significativa entre los dos procedimientos. Conclusiones: El uso de Parche de Cordón Umbilical pareciera ofrecer ventajas a estos neonatos en los que se ha retardado en cierre de la pared abdominal, ya que la técnica crea una superficie mesotelial en contacto con el intestino, es un material autologo, disponible, fácil de aplicar; y en nuestro estudio demostró mayores ventajas en cuanto al poco aumento de la PIA, inicio más precoz de la vía oral y mayor porcentaje de sobrevida comparándolo con el Cierre Primario Convencional.

Gastroschisis and Omphalocele are the most frequent and important birth defects of the abdominal wall in the newborn period. In our country there are few hospitals with the necessary infrastructure to care for these infants. Many of them have to wait valuable time to be operated while the institution where they will be transfered is located, increasing the risk of Abdominal Compartment Syndrome and sepsis. Objective: To compare the surgical outcome between the use of the Umbilical Cord Patch versus the Conventional Primary Closure of the abdominal wall in newborns. Methods: Twenty patients were equally divided into 2 groups. Group A patients underwent abdominal closure using the Umbilical Cord Patch and Group B patients underwent a Conventional Primary Closure of the abdominal wall (10). Results: No statistically significant difference was seen in hospitalization time and complications. Where as significant statistical difference was seen in intra-abdominal pressure (IAP), onset of oral intake and survival between the two procedures. Conclusion: The Umbilical Cord Patch seems to offer advantages to those infants with delayed closure of the abdominal wall because the technique creates a mesothelial surface in contact with the intestine, it is an autologous tissue, available for use at birth and easly applied. The study showed greater benefits in terms of lower increase of IAP, early onset of oral fedding, and higher survival rate compared with Conventional Primary Closure.

Meckel diverticulum in exomphalos minor

A congenital hernia into the base of the umbilical cord is known as an exomphalos and when the size of the defect is 5 cm or less and containing only bowel, it is called as exomphalos minor. We present a case of a newborn with an exomphalos minor within a Meckel diverticulum. He underwent surgical resection of the Meckel diverticulum and repair of the abdominal wall defect. To our knowledge, this is the first reported case of Meckel diverticulum in an exomphalos minor in Korea.