Giant omphalocele: A novel approach for primary repair in the neonatal period using botulinum toxin / Onfalocele gigante: Uma nova abordagem para reparo primário no período neonatal utilizando toxina botulínica

ABSTRACT Introduction: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience. Methods: patients were followed from August 2020 to July 2022. BTA was applied to the lateral abdominal wall in the first days of life followed by surgical repair of the abdominal defect. Results: while awaiting surgery, patients had minimal manipulation, without requiring mechanical ventilation, were on full enteral feeding, and in contact with their parents. The midline was approximated without tension and without the need for additional techniques or the use of a prosthesis. Patients were discharged with repaired defects. Conclusion: this approach represents a middle ground between staged and the nonoperative delayed repairs. It does not require aggressive interventions early in life, allowing maintenance of mother-child bonding and discharge of the patient with a repaired defect without the need for additional techniques or the use of a prosthesis. We believe that this technique should be considered as a new possible asset when managing this complex condition.

RESUMO Introdução: onfalocele gigante (OG) é uma condição complexa para a qual muitas alternativas terapêuticas foram desenvolvidas; no entanto, não há consenso sobre qual o melhor tratamento. Os benefícios e eficácia da toxina botulínica A (TBA) no reparo de grandes defeitos da parede abdominal em adultos foram comprovados, e o relato de uso em crianças cresceu recentemente. O objetivo deste estudo é descrever uma nova técnica para reparo primário de OG utilizando TBA durante o período neonatal e relatar nossa experiência inicial. Métodos: os pacientes foram acompanhados de agosto de 2020 a julho de 2022. A TBA foi aplicada na parede abdominal lateral nos primeiros dias de vida, seguida de correção cirúrgica do defeito abdominal. Resultados: enquanto aguardavam a cirurgia, os pacientes tiveram mínima manipulação, sem ser exigida ventilação mecânica, permaneceram em alimentação enteral plena e mantiveram contato com os pais. A linha média foi aproximada sem tensão e sem necessidade de técnicas adicionais ou uso de prótese. Os pacientes receberam alta com o defeito reparado. Conclusão: essa abordagem representa um meio-termo entre o reparo estagiado e o tratamento não-operatório. Não requer intervenções agressivas no início da vida, permitindo a manutenção do vínculo materno-infantil e a alta do paciente com defeito reparado sem a necessidade de uso de técnicas adicionais ou prótese. Acreditamos que esta técnica deva ser considerada como um novo possível recurso no manejo desta complexa condição.

Hernia umbilical en una gestante / Umbilical hernia in a pregnant woman

Se describe el caso clínico de una grávida de 26 años de edad y tiempo gestacional de 34,4 semanas, quien fue remitida del Hospital General de Rusape al Hospital Central de Harare, ambos en Zimbabwe, por presentar una hernia umbilical, que la aquejaba desde la infancia. La paciente había padecido ese volumen herniario en 3 embarazos anteriores, cuyos partos fueron normales. Luego de efectuar los estudios pertinentes, que demostraron el diagnóstico, y evaluar el caso en equipo multidisciplinario, se decidió practicar cesárea de urgencia. Durante el procedimiento quirúrgico se halló que todo el útero se encontraba dentro del saco herniario cubierto por el epiplón; se extrajo al recién nacido, el cual poseía buena vitalidad, índice de Apgar de 9-10 y peso de 2590 gramos; posteriormente, se resecó el tejido herniario redundante. La paciente evolucionó favorablemente en el período posoperatorio inmediato y recibió el alta hospitalaria a los 5 días.

The case report of a 26 years pregnant woman and gestacional age of 34.4 weeks is described, who was referred from General Hospital of Rusape to Harare Central Hospital, both in Zimbabwe, due to an umbilical hernia that afflicted her since the childhood. The patient had suffered from that hernial volume in 3 previous pregnancies whose childbirths were normal. After the pertinent studies that demonstrated the diagnosis, and evaluate the case in multidisciplinary team, it was decided to practice an emergency Cesarean section. During the surgical procedure it was found that the whole uterus was inside the hernial sack covered by omentum; the new born was extracted, who possessed good vitality, Apgar index 9-10 and 2590 grams weight; later on, the redundant hernial tissue was dried up. The patient had a favorable clinical course in the immediate postoperative period and she was discharged 5 days after.

Omphalocele and biliary atresia: chance or causality. A case report

ABSTRACT To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.

Tumor de ángulo hepático del colon incarcerado en hernia umbilical. Reporte de caso

RESUMEN Los tumores de colon ocupan la tercera causa de muerte por cáncer en Cuba. Los tumores de colon incarcerados en hernias umbilicales son raros. Se distinguen con más frecuencia en las hernias inguinales que en las umbilicales. Los síntomas más significativos son: astenia, pérdida de peso, alteraciones en las heces y dolor abdominal. Suelen presentarse como una masa palpable de difícil reducción. El método diagnóstico por excelencia es el colon por enema y el tratamiento es quirúrgico. La variedad histopatológica más frecuente es el adenocarcinoma. Se presenta el caso de una paciente anciana que acudió a consulta por presentar fatiga, pérdida de peso y deposiciones oscuras. Al examen físico se constató una hernia umbilical. Se le realizaron estudios complementarios y de diagnóstico por imagen. Se determinó una posible tumoración de vías digestivas. Se realizó procedimiento quirúrgico de urgencia. Se presenta este reporte con el objetivo de caracterizar un caso de tumor de ángulo hepático del colon incarcerado en una hernia umbilical debido a lo poco frecuente de su localización.

ABSTRACT Colon tumors are the third leading cause of cancer death in Cuba. Incarcerated colon tumors in umbilical hernias are rare. They are more often distinguished in inguinal hernias than in umbilical ones. The most significant symptoms are: asthenia, weight loss, alterations in the stool and abdominal pain. They usually present as a palpable mass that is difficult to reduce. The diagnostic method par excellence is the colon by enema and surgical treatment. The most common histopathological variety is adenocarcinoma. The case of an elderly patient who came to the clinic due to fatigue, weight loss and dark stools it is presented. The physical examination revealed an umbilical hernia. Complementary and diagnostic imaging studies were performed. A possible digestive tract tumor was determined. An emergency surgical procedure was performed. The aim of this report is to characterize a case of an incarcerated colon hepatic flexure tumor in an umbilical hernia due to its infrequent location.

New clues to understand gastroschisis. Embryology, pathogenesis and epidemiology / Nuevas pistas para comprender la gastrosquisis. Embriología, patogénesis y epidemiología

Abstract gastroschisis is a congenital structural defect of the abdominal wall, most often to the right of the umbilicus, through which the abdominal viscera protrude. Its developmental, etiological and epidemiological aspects have been a hot topic of controversy for a long time. However, recent findings suggest the involving of genetic and chromosomal alterations and the existence of a stress-inducing pathogenetic pathway, in which risk factors such as demographic and environmental ones can converge. . We have conducted a review of the medical literature that gathers information on the embryonic development of the ventral body wall, the primitive intestine, and the ring-umbilical cord complex, as well as on the theories about its origin, pathogenesis and recent epidemiological evidence, for which we consulted bibliographic databases and standard search engines

Resumen La gastrosquisis es un defecto estructural congénito de la pared abdominal, localizado con mayor frecuencia a la derecha del ombligo, a través del cual sobresalen las vísceras abdominales. Durante mucho tiempo, sus aspectos evolutivos, etiológicos y epidemiológicos han sido un tema candente de controversia, aunque hallazgos recientes sugieren la participación de alteraciones genéticas, cromosómicas, y la existencia de una vía patogénica inductora de estrés, en la que factores de riesgo como los demográficos y ambientales pueden converger. Con el objetivo de ampliar la frontera del conocimiento sobre una malformación que ha mostrado una creciente prevalencia global, hemos efectuado una revisión que incluye información, del desarrollo embrionario de la pared corporal ventral, el intestino primitivo, el complejo anillo-cordón umbilical, y de las teorías acerca de su origen, patogénesis e información epidemiológica reciente.

Hérnia umbilical primária: melhor manejo operatório no adulto / Primary umbilical hernia: Best surgical management in adults

RESUMO: Modelo do estudo: Revisão sistemática. Objetivo: Avaliar se é facultativo ou imprescindível o uso de malha cirúrgica no reparo das pequenas hérnias umbilicais primárias, com orifício menor que 2 cm, a fim de oferecer melhores evidências aos cirurgiões e, assim, aprimorar o método cirúrgico e o seu desfecho. Métodos: Trata-se de uma revisão da literatura, cuja busca foi direcionada aos artigos que abordassem o manejo operatório das hérnias abdominais, sobretudo das hérnias umbilicais de pequeno tamanho. A pesquisa foi realizada nas bases de dados primárias PubMed, LILACS, Cochrane Library e Periódicos CAPES. Resultados: No total, foram incluídos quatro es-tudos. Foram avaliadas as taxas de recorrência, bem como as de complicações pós-operatórias após a correção da hérnia umbilical com e sem o uso de tela, observando-se o tamanho do defeito abdominal. Foi observada diminuição da recorrência das hérnias após o reparo com tela. No entanto, complicações, como infecção da ferida operatória, foram mais comumente observadas com o uso da prótese. Não houve consenso quanto ao uso da tela em hérnias menores que 1 cm. Conclusão: O uso de próteses pode vir a ser o tratamento de escolha no reparo das hérnias umbilicais primárias. Contudo, mais estudos são necessários para avaliar o papel dessa estratégia no manejo das hérnias menores que 1 cm (AU)

ABSTRACT: Study design: Systematic review. Objective: Evaluate whether surgical mesh is optional or essential for the repair small primary umbilical hernias, with an orifice smaller than 2 cm, in order to provide better evidence for surgeons, thus enhancing surgical method and its outcomes. Methods: This is a literature review, whose search was directed towards papers that depicted surgical management of abdominal hernias, especially small umbilical hernias. The research was carried out in the primary the primary databases PubMed, LILACS, Cochrane Library, and Periódicos CAPES. Results: A total of four studies were included. Recurrence rates, as well as postoperative complications, were assessed after an umbilical hernia was corrected with or without the use of a mesh, observing the size of the abdominal defect. A reduction in the re-occurrence of hernias was observed when using a mesh. However, complications, such as surgical site infection, were more commonly noticed with the use of the prosthe-sis. There was no consensus regarding the use of the mesh in hernias smaller than 1 cm. Conclusion: The use of surgical mesh may prove to be the treatment of choice for the repair of primary umbilical hernias. However, more studies are needed to evaluate the role of this strategy in the management of hernias smaller than 1 cm. (AU)

La hernia umbilical en el paciente cirrótico: ¿tratamiento conservador o cirugía? / Umbilical hernia in cirrhotic patients: conservative treatment or surgery?

RESUMEN Antecedentes: la ascitis es una complicación de frecuente aparición en el paciente cirrótico que al producir un aumento de la presión intraabdominal puede originar una hernia de la pared abdominal; el defecto umbilical latente es su localización más habitual. Objetivo: presentar la incidencia y los beneficios que ofrece la resolución electiva de la patología um bilical en estos pacientes. Material y método: se presentan 15 pacientes, todos masculinos, que fueron intervenidos por hernia umbilical sintomática en forma electiva en el medio hospitalario. El período comprende desde enero del año 2015 a enero de 2019. El 100% de los enfermos cursa un cuadro de cirrosis hepática, con antecedentes de etilismo crónico. Se efectuó la reparación de la hernia con cierre del defecto y malla supraaponeurótica de polipropileno en la mayoría de los casos. Resultados: los pacientes fueron evaluados desde el punto de vista clínico con la escala (score) de Child en el preoperatorio. Se procedió a la evacuación de la ascitis en todos los casos y el control posoperatorio se efectuó cada 30 días los primeros 6 meses. Luego dos veces al año. No se evidenció recidiva. Dos pacientes no volvieron al control luego del año de la cirugía y 1 paciente falleció por la enfermedad de base a los 6 meses de la cirugía. Conclusiones: los enfermos con cirrosis hepática y hernia umbilical deben ser intervenidos quirúrgica mente en forma electiva. La observación y abstención quirúrgica conllevan el riesgo de rotura del saco herniario con alta morbimortalidad.

ABSTRACT Background: Background: Ascites is a common complication in patients with cirrhosis, and elevated intraabdominal pressure can lead to the development of abdominal wall hernias, particularly in patients with latent umbilical defects. Objectives: The aim of this study was to report the incidence and benefits of elective surgery for the management of umbilical hernias in cirrhotic patients with ascites. Material and methods: Between January 2015 and January 2019 15 patients with symptomatic umbi lical hernia underwent elective surgery in a public hospital; 100% were men with a history of alcoho lism and were hospitalized due to liver cirrhosis. The defect was closed, and a polypropylene mesh was placed in the supra-aponeurotic plane in most cases. Results: The preoperative risk was estimated using the Child-Pugh score. Ascites was evacuated in all the cases. Patients were followed-up every 30 days during the first 6 months and then twice a year. There were no hernia recurrences. Two patients were lost to follow-up 12 months after surgery and 1 patient died 9 months after the procedure due to progression of cirrhosis. Conclusions: Patients with liver cirrhosis and umbilical hernia should undergo elective surgery. Wat chful waiting is associated with higher risk of hernia rupture and high morbidity and mortality.

Técnica TARUP. Ventajas de la cirugía mínimamente invasiva de la pared abdominal asistida por robot / TARUP technique. Advantages of minimally invasive robot-assisted abdominal Wall surgery

La reparación de la hernia ventral/incisional mediante cirugía mínimamente invasiva asistida por robot ha aumentado exponencialmente en los últimos años. Este aumento probablemente esté relacionado con las ventajas que aporta, destacando una mejor visualización, la implementación de instrumentos articulados y la mejor ergonomía para el cirujano. La técnica TARUP (Robotic Transabdominal Retromuscular Umbilical Prosthetic Hernia Repair) combina los beneficios de la cirugía mínimamente invasiva asistida por robot con una menor morbilidad relacionada con la herida y la colocación de una malla en posición retromuscular. (AU)

The use of robot-assisted minimally invasive surgery in ventral/incisional hernia repair has increased exponentially in recent years. This increase is probably related to the advantages of robotic surgery, among which are better visualization, the implementation of articulated instruments and better ergonomics for the surgeon. The TARUP (Robotic Transabdominal Retromuscular Umbilical Prosthetic Hernia Repair) technique combines the benefits of minimally invasive surgery, in terms of less wound-related morbidity, also allowing the placement of a mesh in a retromuscular position facilitated by the use of the robotic platform. (AU)

New clues to understand gastroschisis. Embryology, pathogenesis and epidemiology.

gastroschisis is a congenital structural defect of the abdominal wall, most often to the right of the umbilicus, through which the abdominal viscera protrude. Its developmental, etiological and epidemiological aspects have been a hot topic of controversy for a long time. However, recent findings suggest the involving of genetic and chromosomal alterations and the existence of a stress-inducing pathogenetic pathway, in which risk factors such as demographic and environmental ones can converge. To expand the frontier of knowledge about a malformation that has showed a growing global prevalence, we have conducted a review of the medical literature that gathers information on the embryonic development of the ventral body wall, the primitive intestine, and the ring-umbilical cord complex, as well as on the theories about its origin, pathogenesis and recent epidemiological evidence, for which we consulted bibliographic databases and standard search engines.

La gastrosquisis es un defecto estructural congénito de la pared abdominal, localizado con mayor frecuencia a la derecha del ombligo, a través del cual sobresalen las vísceras abdominales. Durante mucho tiempo, sus aspectos evolutivos, etiológicos y epidemiológicos han sido un tema candente de controversia, aunque hallazgos recientes sugieren la participación de alteraciones genéticas, cromosómicas, y la existencia de una vía patogénica inductora de estrés, en la que factores de riesgo como los demográficos y ambientales pueden converger.Con el objetivo de ampliar la frontera del conocimiento sobre una malformación que ha mostrado una creciente prevalencia global, hemos efectuado una revisión que incluye información, del desarrollo embrionario de la pared corporal ventral, el intestino primitivo, el complejo anillo-cordón umbilical, y de las teorías acerca de su origen, patogénesis e información epidemiológica reciente.

TARUP technique. Advantages of minimally invasive robot-assisted abdominal Wall surgery. / Técnica TARUP. Ventajas de la cirugía mínimamente invasiva de la pared abdominal asistida por robot.

The use of robot-assisted minimally invasive surgery in ventral/incisional hernia repair has increased exponentially in recent years. This increase is probably related to the advantages of robotic surgery, among which are better visualization, the implementation of articulated instruments and better ergonomics for the surgeon. The TARUP (Robotic Transabdominal Retromuscular Umbilical Prosthetic Hernia Repair) technique combines the benefits of minimally invasive surgery, in terms of less wound-related morbidity, also allowing the placement of a mesh in a retromuscular position facilitated by the use of the robotic platform.

La hernia umbilical en régimen de cirugía mayor ambulatoria: procedimiento seguro y de menor coste / Umbilical hernia in major outpatient surgery: safe and lower-cost procedure

Introducción: La cirugía mayor ambulatoria (CMA) constituye una estrategia coste-efectiva comparada con la cirugía tradicional, al resolver el procedimiento quirúrgico sin comprometer la calidad de la atención ni la seguridad del paciente. Objetivo: Demostrar que la cirugía abierta de la hernia umbilical en régimen de CMA es un procedimiento seguro y de menor coste. Para ello realizamos un estudio de los factores de riesgo de ingreso no previsto en pacientes intervenidos de hernia umbilical en nuestro servicio, durante cuatro años. Material y métodos: Estudio observacional retrospectivo de 478 pacientes consecutivos intervenidos durante los años 2015 a 2018. Se revisaron las historias electrónicas de los pacientes y se creó una base de datos para el estudio de los resultados. Se utilizó un modelo de regresión logística para analizar el factor de riesgo de ingreso no previsto por edad, sexo, antecedentes personales, índice de masa corporal, ASA del paciente, tipo de reparación, tamaño del defecto, utilización de prótesis, causa del ingreso no previsto y complicaciones. Resultados: Edad media 48,1 ± 13 años con un índice de hospitalización global del 8,8 %. Hombres 319 (66,7 %) y mujeres 159 (33,3 %) con una tasa de ingresos no previstos del 6,9 % y 12,6 %, respectivamente ( p = 0,039). El análisis estadístico demostró que no se incrementa el riesgo de ingreso no previsto con la edad (0,678). El riesgo de hospitalización fue 2,78 veces mayor en las mujeres que en los hombres ( p = 0,005). Los pacientes ASA II tienen un riesgo de hospitalización 6,49 veces superior a los pacientes con ASA I (0,021) (AU)

Introducción: La cirugía mayor ambulatoria (CMA) constituye una estrategia coste-efectiva comparada con la cirugía tradicional, al resolver el procedimiento quirúrgico sin comprometer la calidad de la atención ni la seguridad del paciente. Objetivo: Demostrar que la cirugía abierta de la hernia umbilical en régimen de CMA es un procedimiento seguro y de menor coste.To this end, we conducted a study of the risk factors for unforeseen admission in patients operated on for umbilical hernia in our service, for four years. Material and methods: Retrospective observational study of 478 consecutive patients operated on during the years 2015 to 2018. Electronic patient records were reviewed and a database was created for the study of outcomes. A logistic regression model was used to analyze the risk factor for unplanned admission by age, sex, personal history, body mass index, patient ASA, type of repair, defect size, use of prostheses, cause of unplanned admission and complications. Results: Mean age 48.1 ± 13 years with an overall hospitalization rate of 8.8 %. Males 319 (66.7%) and females 159 (33.3%) with an unforeseen income rate of 6.9% and 12.6%, respectively (p = 0.039). Statistical analysis showed that the risk of unplanned admission does not increase with age (0.678) The risk of hospitalization was 2.78 times higher in women than in men (p = 0.005). ASA II patients have a 6.49-fold higher risk of hospitalization than patients with ASA I (0.021). A greater number of admissions was found in patients operated on with general anesthesia and spinal anesthesia, compared to those operated with local anesthesia and sedation or with a laryngeal mask (p = 0.000). Major outpatient surgery was cheaper than surgery with admission, between 45-52%, with a statistically significant difference (p = 0.000) (AU)

A propósito de un caso: Resolución quirúrgica en dos tiempos de Pentalogía de Cantrell / Case Report: Two-stage surgical resolution of Cantrell's Pentalogy

INTRODUCCIÓN: La pentalogía de Cantrell abarca la presencia de cinco defectos congénitos que representan un gran desafío para los cirujanos. Las anomalías del corazón, pericardio, diafragma, esternón y pared abdominal anterior son los hallazgos principales. Su incidencia es baja, sin embargo, es fundamental identificarla oportunamente para adoptar una terapia adecuada para todos los defectos descritos, puesto que se reporta una mortalidad elevada. CASO CLÍNICO: Se trató de un recién nacido a término, de sexo masculino, con antecedentes de defecto de la pared abdominal compatible con onfalocele detectado mediante ecografía prenatal. Tras cesárea programada, se evidenció defecto en la pared abdominal a través del cual emergían el lóbulo hepático izquierdo, intestinos y corazón; el esternón además presentaba una fisura baja. En ecocardiograma se evidenció foramen oval permeable, insuficiencia tricuspídea leve e hipertensión pulmonar severa. EVOLUCIÓN: Se decidió su manejo quirúrgico inmediato. Se colocó silo, con cierre progresivo de la línea media en 7 días. En segundo tiempo quirúrgico, se corrigió el defecto diafragmático y pericárdico con prótesis de pericardio bovino. Pese a la evolución adecuada de la cirugía, a los 28 días, el paciente presentó cianosis súbita, sin responder a maniobras de reanimación y falleció. CONCLUSIÓN: La Pentalogía de Cantrell es una enfermedad rara, con características clínicas, anatómicas y embriológicas peculiares, representa un desafío único para los cirujanos. Su diagnóstico temprano, así como el seguimiento durante el embarazo, la planificación de una cesárea en un centro de alto nivel y la aproximación quirúrgica inmediata con un equipo multidisciplinario, son componentes clave en el manejo integral de pacientes con Pentalogía de Cantrell.

BACKGROUND: Cantrell's pentalogy includes the presence of five birth defects that represent a great challenge for surgeons. Abnormalities of the heart, pericardium, diaphragm, sternumand anterior abdominal wall are the main findings. Its incidence is low, however, it is essential to identify Cantrell´s pentalogy timely to adopt an adequate therapy for all specific defects, since it has high mortality. CASE REPORT: The patient was a full- term male newborn, with a history of abdominal wall defect compatible with an omphalocele detected by prenatal ultrasound. After the caesarean section, the abdominal wall defect was notable, the left liver lobe, intestines and heart emerged through it, the sternum also had a low fissure. The echocardiogram revealed a permeable oval foramen, mild tricuspid regurgitation, and severe pulmonary hypertension. EVOLUTION: Immediate surgical management was decided. Silo was placed, with progressive closure of the midline in 7 days. During the second surgical procedure, the diaphragmatic and pericardial defect was corrected with a bovine pericardial prosthesis. Despite the adequate evolution after surgery, at day 28 he presented with sudden cyanosis and didn't respond to cardiopulmonary resuscitation and died. CONCLUSIONS: Cantrell's Pentalogy is a rare disease, with peculiar clinical, anatomical and embryological characteristics, it represents a unique challenge for surgeons. Early diagnosis, as well as follow-up during pregnancy, planning a cesarean section in a high-level center and immediate surgical approach with a multidisciplinary team, are the key components in the management of patients with Cantrell's Pentalogy.

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.

Hernia umbilical: un problema no resuelto / Umbilical hernia: an unsolved problem

RESUMEN La cirugía de la hernia umbilical ha sido considerada tradicionalmente como sencilla y de fácil ejecución. El cambio conceptual de la hernia umbilical y los avances en su tratamiento han modificado su abordaje, estableciéndose escenarios de complejidad variable (obesidad, embarazo, cirróticos, distasis de los rectos, etc.) que demandan un conocimiento más profundo del tema por parte de la comunidad médica. El impacto económico en el sistema de salud, debido a su alta prevalencia como patología quirúrgica, implica un uso racional de recursos, así como la necesidad de una nueva categorización dentro de la cirugía de la pared abdominal. Es necesario establecer modificaciones en los procesos diagnósticos y terapéuticos en una entidad que ha sido relegada a los niveles básicos del ejercicio quirúrgico habitual.

SUMMARY Umbilical hernia surgery has traditionally been considered simple and easy to perform. A conceptual change of the umbilical hernia and the advances in its treatment have modified its approach, establishing scenarios of variable complexity (Obesity, pregnancy, cirrhosis, diastasis of the rectus, etc.), which demand a deeper knowledge of the subject on the medical community. The economic impact on the health system, due to its high prevalence as a surgical pathology, implies a rational use of resources, as well as the need for a new categorization within the abdominal wall surgery. It is necessary to establish modifications in the diagnostic and therapeutic processes in an entity that has been relegated to the basic levels of the usual surgical practice.

Ectopia cordis associated with pentalogy of cantrell-a case report / Ectopia cordis associada à pentalogia de cantrell-relato de caso

Abstract Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by changes in the mesodermal median structures and congenital heart disease, often with a poor prognosis. In 1958, Cantrell et al2 defined the full spectrum of the syndrome with the following anomalies: defects of the anterior diaphragm, of the lower part of the sternum, of the supraumbilical region and the abdominal wall, of the diaphragmatic pericardium, and various intracardiac congenital abnormalities. The present report describes a case of ectopia cordis associated with PC and the importance of the participation of a multidisciplinary team in the treatment of this condition.

Resumo A pentalogia de Cantrell (PC) é uma rara anomalia congênita caracterizada por alterações nas estruturas medianas mesodérmicas e doenças cardíacas congênitas, cursando muitas vezes com um mau prognóstico. Em 1958, Cantrell et al2 definiram o espectro completo da síndrome com as seguintes anomalias: defeitos do diafragma anterior, da parte inferior do esterno, da região supraumbilical e parede abdominal, do pericárdio diafragmático, e várias anormalidades congênitas intracardíacas. O presente relato relaciona-se a um caso de ectopia cordis associado à PC e à importância da participação de uma equipe multidisciplinar no acompanhamento da doença.

Clinical effect of laparoscopic and open umbilical hernia repair for adult umbilical hernia / 国际外科学杂志

Objective To compare the clinical effect of laparoscopic and open umbilical hernia repair for adult umbilicalhernia.Methods Eighty-threepatients with adult umbilical hernia admitted to People's Hospital of Beijing Daxing District from Feb.2008 to Jan.2017 were randomly divided into laparoscopic umbilical hernia repair group (n =38) and open umbilical hernia repair group (n =45).Patients in laparoscopic umbilical hernia repair group accepted laparoscopic umbilical hernia repair with intraperitoneal onlay mesh (n =38),while patients in open umbilical hernia repair group accepted traditional umbilical hernia repairtreatment.Operation time,hospital stay,blood loss,seroma/hematoma formation,wound infection,incomplete bowel obstruction and recurrence were compared between the two groups with the software of SPSS 22.0.The outpatient consultation was performed at 2 weeks,1 month,3 months,and 6 months after operation.The follow-up contents were wound condition,pain,and whether there were any masses in the umbilicus.After every 3 months of telephone follow-up or outpatient review,whether there were any masses in the umbilicus,the average follow-up time was 14 months.Results All operations were successfully completed.The operation time of laparoscopic umbilical hernia repair group was shorter than open umbilical hernia repair group [(31 ± 8) min vs (48 ± 10) min,P ＜ 0.01],and hospital stay were significantly shortened in laparoscopic umbilical hernia repair group (P ＜ 0.05).The amount of bleeding of the laparoscopic umbilical hernia repair group decreased significantly than open umbilical hernia repair group [(40 ± 18) ml vs (62 ± 25) ml,P ＜ 0.01].The postoperative painof the laparoscopic umbilical hernia repair group was mild than open umbilical hernia repair group (P ＜ 0.05).The postoperative complications include seroma,wound infection and incomplete bowel obstruction.There were no significant differences between the two groups of seroma,incision infection and recurrence (P ＞ 0.05).The difference of the incidence of incomplete bowel obstruction between the two groups was statistically significant (P =0.019) . The total postoperative complication rate was significantly lower in laparoscopic umbilical hernia repair group than in open umbilical hernia repair group(x2 =5.328,P =0.021).Conclusions Laparoscopic umbilical hernia repair has short operation time,less bleeding,and satisfactory postoperative pain.It is worthy of advocacy for umbilical hernia patients who can tolerate general anesthesia.

Histerocele gravídico umbilical. Reporte de un caso / Umbilical gravid hysterocele. A case report

Resumen ANTECEDENTES: Aunque la hernia umbilical es relativamente frecuente en la población africana, la mayoría de los casos cursan asintomáticos. La situación en la que un útero grávido entra en un saco herniario representa una complicación excepcional de la hernia umbilical. CASO CLÍNICO: Paciente de 30 años, con antecedentes médicos de hernia umbilical y obstétricos de 5 embarazos, 4 partos y 1 aborto espontáneo. Acudió a consulta por dolor en la parte baja del vientre, de tres días de evolución. En el interrogatorio refirió amenorrea de 5 meses; 2 semanas antes percibió movimientos fetales y ausencia de los mismos 3 días previos a la consulta médica. Al examen físico se observó el abdomen con aumento de volumen irreductible, de 30 cm de diámetro, de forma pendular, que se protruía a través de un gran anillo herniario umbilical y llegaba hasta la mitad de los muslos; se palpó el feto, pero no se escucharon latidos cardiacos. Por los antecedentes médicos, hallazgos clínicos y ecográficos se estableció el diagnosticó de muerte fetal intrauterina, como complicación de útero grávido en una hernia umbilical. Se decidió la interrupción del embarazo mediante cesárea de urgencia. La hernia umbilical se reparó con reforzamiento del defecto mediante colgajos fascio-aponeuróticos, según la técnica de Mayo. La evolución de la paciente fue satisfactoria. CONCLUSIONES: El tratamiento de pacientes embarazadas con hernia umbilical incluye una conducta conservadora, control prenatal estricto, colocación de un corsé para rectificar el útero grávido, programación de la cesárea y reparación del defecto herniario.

Abstract BACKGROUND: Although umbilical hernia is relatively common in African patients, the majority of cases are asymptomatic. The situation in which a gravid uterus enters a hernia sac is one of the rarest complications of umbilical hernia. CLINICAL CASE: 30-year-old pregnant woman with a history of umbilical hernia and obstetric of 5 pregnancies, 4 deliveries and 1 spontaneous abortion. She reported pain in the abdomen that appeared 3 days ago, absence of menstruation 5 months ago, with fetal movements referred 2 weeks ago and absence of them 3 days ago. At the physical examination, an irreducible volume increase of approximately 30 cm in diameter with a pendulum shape that protruded through a large umbilical hernia ring and reached the middle of the thighs, fetal parts were palpated and absence of fetal heartbeats. Due to the antecedents, the clinic and the obstetric ultrasound, an intrauterine fetal death was diagnosed as a complication of a gravid uterus in an umbilical hernia. The interruption of pregnancy was performed by emergency caesarean section. The umbilical hernia was repaired with reinforcement of the defect using fascio-aponeurotic flaps, according to the Mayo technique. The evolution was satisfactory until hospital discharge. CONCLUSIONS: The treatment of pregnant patients with umbilical hernia includes a conservative behavior, strict prenatal control, placement of a brace to rectify the pregnant uterus, programming of the cesarean and repair of the hernia defect.

Características de los neonatos con onfalocele intervenidos quirúrgicamente con anestesia regional y local / Characteristics of neonates with omphalocele operated surgically with regional and local anesthesia

Introducción: el onfalocele es un defecto congénito consistente en una herniación en la pared abdominal. Los intestinos, el hígado u otros órganos sobresalen a través del ombligo y quedan envueltos en un saco delgado y casi transparente.Objetivo: caracterizar desde el punto de vista demográfico, clínico y epidemiológico a los neonatos con onfalocele, intervenidos quirúrgicamente con anestesia regional y local en el Hospital Central de Benguela, República de Angola.Método: se realizó una investigación observacional descriptiva prospectiva en el período comprendido entre septiembre de 2011 y agosto de 2013. La población quedó constituida por la totalidad de neonatos que nacieron con el defecto, y la muestra por los 17 que cumplieron los criterios de inclusión. Se cumplieron los procedimientos de preparación inicial, anestésicos, quirúrgicos y posquirúrgicos.Resultados: predominaron los neonatos masculinos (52,9 por ciento) y los menores de 24 horas de nacidos (41,2 por ciento). En 11 de los operados se identificaron malformaciones asociadas (64,7 por ciento), siendo las cardiovasculares las más frecuentes (35,3 por ciento). Más de la mitad presentó onfaloceles grandes (64,7 por ciento). Sin embargo, se logró realizar el cierre total en más de la mitad del total de operados (58,8 por ciento). La dehiscencia parcial de la herida quirúrgica fue la complicación más frecuente (37,5 por ciento). La mortalidad fue baja (17,65 por ciento).Conclusiones: la anestesia regional y local constituyó una opción terapéutica para el cierre quirúrgico del onfalocele ante la limitación tecnológica de los países en vías de desarrollo para evitar la muerte de los niños que nacen con esta malformación(AU)

Introduction: the omphalocele is a congenital defect consisting of a herniation in the abdominal wall. The intestines, liver or other organs protrude through the navel and are wrapped in a thin, almost transparent sac.Objective: to characterize from the demographic, clinical and epidemiological point of view the neonates with omphalocele, surgically treated with regional and local anesthesia in the Central Hospital of Benguela, Republic of Angola.Method: a prospective descriptive observational research was carried out in the period between September 2011 to August 2013. The population was constituted by the totality of neonates that were born with the defect, and the sample by the 17 that met the inclusion criteria. The initial preparation, anesthetic, surgical and postsurgical procedures were completed.Results: male neonates predominated (52,9 percent) and those under 24 hours of age (41,2 percent). In 11 of the operated patients, associated malformations were identified (64,7 percent), with the cardiovascular ones being the most frequent (35,3 percent). More than half presented large omphaloceles (64,7 percent). However, total closure was achieved in more than half of the total number of operated patients (58,8 percent). Partial dehiscence of the surgical wound was the most frequent complication (37,5 percent). Mortality was low (17,65 percent).Conclusions: regional and local anesthesia was a therapeutic option for the surgical closure of the omphalocele due to the technological limitations of the developing countries to avoid the death of children born with this malformation(AU)

Hernia umbilical en pacientes cirróticos con ascitis: ¿tratamiento conservador o quirúrgico? / Umbilical hernia in liver cirrhosis with ascites: conservative or surgical treatment?

Resumen La ascitis es la complicación más común en los pacientes con cirrosis hepática. Las hernias umbilicales (HU) ocurren en 20% de estos pacientes y 40% en aquellos con ascitis severa. La HU ocurre debido al aumento de presión intraabdominal, al debilitamiento de la fascia abdominal y la pérdida de masa muscular. Además, tienen tendencia a aumentar rápidamente y presentar alto riesgo de complicaciones que amenazan la vida del paciente. El tratamiento de la HU no complicada es controversial, tanto el manejo quirúrgico (herniorrafia) como el manejo conservador (control de ascitis) presentan alta tasa de complicaciones, en consecuencia incrementa la morbimortalidad. Actualmente, se recomienda herniorrafia umbilical con previo control de la ascitis en el manejo de HU no complicada, reduce el riesgo de infección de herida operatoria, evisceración, drenaje de ascitis, peritonitis y reduce hasta 41% la recidiva de HU. El éxito de este enfoque también depende del grado de disfunción hepática. El tratamiento de la HU complicada es quirúrgico (herniorrafia sin malla), con menor tasa de mortalidad respecto al manejo conservador. Estudios revelan ventajas de la herniorrafia umbilical laparoscópica (mínimamente invasiva y sin tensión) en comparación a la cirugía abierta, sin embargo, aún no hay evidencia al respecto.

Ascites is the most common complication in patients with liver cirrhosis. Umbilical hernias (HU) occur in 20% of these patients and 40% in those with severe ascites. HU occurs due to increased intra-abdominal pressure, weakening of the abdominal fascia and loss of muscle mass. In addition, they have a tendency to enlarge rapidly and present high risk of complications that threaten the patient's life. The treatment of the uncomplicated HU is controversial, both the surgical management (herniorrhaphy) and the conservative management (control of ascites) present high rate of complications, consequently high morbidity and mortality. Currently, umbilical herniorrhaphy is recommended with prior control of ascites in uncomplicated HU management, it reduces the risk of surgical wound infection, evisceration, ascites drainage, peritonitis, and it reduces up to 41% of HU recurrence. The success of this approach also depends on the degree of liver dysfunction. The treatment of complicated HU is surgical (herniorrhaphy without mesh), with lower mortality rate compared to conservative management. Studies reveal advantages of umbilical herniorrhaphy laparoscopy (minimally invasive and stress-free) compared to open surgery; however there is still no evidence about it.

Hernia de Littre estrangulada en un adulto / Littre strangulated hernia in an adult

Se describe el caso clínico de un adulto de 47 años de edad, quien acudió al cuerpo de guardia del Hospital Provincial Docente Clinicoquirúrgico Saturnino Lora Torres de Santiago de Cuba por presentar, desde hacía 3 días, dolor en epigastrio, de comienzo insidioso, que desapareció en las últimas 24 horas, y apareció nuevamente en la fosa ilíaca derecha y mesogastrio, pero esta vez se trasformó en fijo, punzante, de moderada intensidad, que no se aliviaba con analgésicos, y asociado a náuseas; igualmente, refirió y se corroboró al examen físico la presencia de una hernia umbilical que no lograba reducirse. El paciente fue operado y se encontró una hernia de Littre umbilical estrangulada. Se le realizó resección y anastomosis del íleon a nivel del divertículo de Meckel, así como herniorrafia umbilical. La evolución fue favorable y egresó sin complicaciones(AU)

The case report of a 47 years adult is described who went to the emergency room of Saturnino Lora Torres Teaching Clinical Surgical Provincial Hospital in Santiago de Cuba due to epigastric distress of insidious beginning for 3 days that disappeared in the last 24 hours, appearing again in the right iliac cavity and mesogastrium, but this time it was transformed in a fixed, sharp, of moderate intensity pain that was not relieved with analgesics, and associated with nausea; equally, he referred the presence of an umbilical hernia that was not able to decrease and it was corroborated in the physical exam. The patient was operated on and a strangulated umbilical Littre hernia was found. A resection and anastomosis of ilium at the Meckel diverticulum level was carried out, as well as umbilical hernia repair. There was a favorable clinical course and he was discharged without complications(AU)