Versatilidad del colgajo VRAM / Versatility of the VRAM flap

Los defectos extensos de la pared abdominal y de la zona perineal derivados de las resecciones tumorales o posteriores a procesos infecciosos pueden ser resueltos de una manera rápida y sin la ayuda de técnicas microquirúrgicas mediante el colgajo VRAM, una herramienta reconstructiva importante que ha entrado en desuso, pero que sin lugar a dudas es una estrategia que proporciona seguridad para el cirujano plástico en los casos indicados. En nuestro trabajo se describe una serie de casos en los que se utilizó al colgajo VRAM para la reconstrucción de defectos en pared abdominal y la zona perineal; defectos amplios que fueron satisfactoriamente resueltos, en donde se denotan las ventajas que ofrece este colgajo para la resolución de problemas reconstructivos de las áreas anatómicas en cuestión.

Extensive defects of the abdominal wall and perineal area derived from tumor resections or after infectious processes can be resolved quickly and without the help of microsurgical techniques using the VRAM flap, an important reconstructive tool that an important reconstructive tool that that has gone into disuse, but without a doubt it is a strategy that provides security for the plastic surgeon in the indicated cases. In our work, a series of cases are described in which the VRAM flap was used for the reconstruction of defects in the abdominal wall and the perineal area; large defects that were satisfactorily resolved, where the advantages offered by this flap for the resolution of reconstructive problems of the anatomical areas in question are denoted.

Anatomy and embryology of abdominal wall defects.

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.

Abdominal Wall Defects: A Review of Current Practice Guidelines.

The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Gastroschisis is a defect in the abdominal wall with exposed abdominal contents. Mortality rates are low but lengths of stay are often prolonged by bowel dysmotility and other intestinal abnormalities in complicated cases. Omphalocele is a defect through the umbilical cord with herniated abdominal contents covered by a sac. It is associated with other genetic abnormalities and other anomalies that can lead to significant morbidity and mortality. Prenatal diagnosis in both conditions allows for improved prenatal consultation and coordinated perinatal care to improve clinical outcomes.

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.

Pentalogy of Cantrell With a Left Ventricular Diverticulum and Muscular VSD.

Pentalogy of Cantrell (POC) is a rare congenital disorder characterized by defects of the anterior diaphragm, midline supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and heart. The low incidence and significant heterogeneity of POC make it difficult for most surgeons to gain consistent experience in treating these congenital heart defects. Here, we describe the treatment of a patient with POC, tetralogy of Fallot, left ventricular diverticulum, and a muscular ventricular septal defect. An innovative approach through a left ventricular diverticulotomy was utilized, which provided excellent exposure for repair of the muscular ventricular septal defect.

Gastrosquisis / Gastroschisis

Introducción: La gastrosquisis es un defecto de la pared abdominal anterior, paraumbilical, habitualmente a la derecha con la protrusión de vísceras intraabdominales, las que flotan libremente en el líquido amniótico durante la vida intrauterina, solo recubiertas por su peritoneo visceral. Objetivo: Analizar la entidad a propósito de un caso portador de gastrosquisis compleja. . Caso clínico: Se describe una neonato de 4 días remitido al servicio de urgencias del Hospital Provincial de Cabinda, República de Angola, por presentar deshidratación severa y "evisceración". La paciente fallece 36 horas posteriores al ingreso. Conclusiones: Los neonatos con esta enfermedad se presentan como una emergencia quirúrgica que plantean un reto difícil para el cirujano tratante. Tiene una alta mortalidad aun con el tratamiento apropiado. Técnicas novedosas de tratamiento se acercan a las tasas de sobrevida esperadas para esta entidad(AU)

Introduction: Gastroschisis is a defect of the paraumbilical anterior abdominal wall, usually on the right with protrusion of intraabdominal viscera, which float freely in the amniotic fluid during intrauterine life, only covered by its visceral peritoneum. Objective: To analyze the entity apropos a case of complex gastroschisis. Clinical case: The case is described od a four-day-old neonate referred to the emergency service at Provincial Hospital of Cabinda, Republic of Angola, for presenting severe dehydration and "evisceration." The patient died 36 hours after admission. Conclusions: Neonates with this disease are presented as a surgical emergency that poses a difficult challenge for the treating surgeon. It has a high mortality even with the appropriate treatment. Novel treatment techniques are close to the expected survival rates for this entity(AU)

Graphene Oxide Functionalized Double-Layered Patch with Anti-Adhesion Ability for Abdominal Wall Defects.

BACKGROUND: Effective repair of full-thickness abdominal wall defects requires a patch with sufficient mechanical strength and anti-adhesion characteristics to avoid the formation of hernias and intra-abdominal complications such as intestinal obstruction and fistula. However, patches made from polymers or bio-derived materials may not meet these requirements and lack the bionic characteristics of the abdominal wall. MATERIALS AND METHODS: In this study, we report a consecutive electrospun method for preparing a double-layer structured nanofiber membrane (GO-PCL/CS-PCL) using polycaprolactone (PCL), graphene oxide (GO) and chitosan (CS). To expand the bio-functions (angiogenesis/reducing reactive oxygen species) of the patch (GO-PCL/NAC-CS-PCL), N-acetylcysteine (NAC) was loaded for the repair of full-thickness abdominal wall defects (2×1.5cm) in rat model. RESULTS: The double-layered patch (GO-PCL/NAC-CS-PCL) showed excellent mechanical strength and biocompatibility. After 2 months, rats treated with the patch exhibited the desired repair effect with no hernia formation, less adhesion (adhesion score: 1.50±0.50, P<0.001) and more collagen deposition (percentage of collagen deposition: 34.94%±3.31%, P<0.001). CONCLUSION: The double-layered nanomembranes presented in this study have good anti-hernia and anti-adhesion effects, as well as improve the microenvironment in vivo. It, therefore, holds good prospects for the repair of abdominal wall defects and provides a promising key as a postoperative anti-adhesion agent.

Investigation of a connection between abdominal wall defects and severity of the herniation in fetuses with gastroschisis and omphalocele.

Analyze the biometric parameters and the size (area) of abdominal wall defect (AWD) in fetuses with gastroschisis and omphaloceles and correlate them with the herniated internal organs. We studied 22 fetuses (11 with AWDs and 11 without anomalies). In all fetuses we evaluated the xiphopubic distance (XPD) and iliac crest distance (ICD). In fetuses with AWDs we dissected the abdominal wall and measured the width and length of the defect for calculating its area and studying the correlation between the size of the defect with the organs that were herniated. For statistical analysis, the Anova and Tukey post-test were used (p < 0.05). The XPD in the control group had mean of 4.2 mm (2.3-5.9; SD ± 1.11), while in the AWDs it was 4.2 mm (2.9-5.5; SD ± 0.98) (p = 0.4366). The ICD had mean values of 2.5 mm (1.6-3.4; SD ± 0.58) in the control group, and 2.3 mm (1.2-3.0; SD ± 0.56) in AWDs fetuses (p = 0.6963). The number of herniate organs do not have significant correlation with the area of the defect (r2 = 0.2504, p = 0.5068). There is no correlation between the size (area) of abdominal wall defects and the number of the internal organs that herniated. Therefore, the hole size is not a predictor of the severity of the gastroschisis or omphalocele.

Congenital abdominal wall defects and cryptorchidism: a population-based study.

PURPOSE: Several studies have reported high prevalence of undescended testis (UDT) among boys with congenital abdominal wall defects (AWD). Due to rarity of AWDs, however, true prevalence of testicular maldescent among these boys is not known. We conducted a national register study to determine the prevalence of UDT among Finnish males with an AWD. METHODS: All male infants with either gastroschisis or omphalocele born between Jan 1, 1998 and Dec 31, 2015 were identified in the Register of Congenital Malformations. The data on all performed operations were acquired from the Care Register for Health Care. The register data were examined for relevant UDT diagnosis and operation codes. RESULTS: We identified 99 males with gastroschisis and 89 with omphalocele. UDT was diagnosed in 10 (10.1%) infants with gastroschisis and 22 (24.7%) with omphalocele. Majority of these required an operation; 8/99 (8.1%) gastroschisis and 19/89 (21.3%) omphalocele patients. UDT is more common among AWD patients than general population with the highest prevalence in omphalocele. CONCLUSIONS: Cryptorchidism is more common among boys with an AWD than general population. Furthermore, omphalocele carries significantly higher risk of UDT and need for orchidopexy than gastroschisis. Due to high prevalence testicular maldescent, careful follow-up for UDT is recommended.

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.

Development of a prenatal clinical care pathway for uncomplicated gastroschisis and literature review.

BACKGROUND: Gastroschisis is an abdominal wall defect wherein the bowel is herniated into the amniotic fluid. Controversy exists regarding optimal prenatal surveillance strategies that predict fetal well-being and help guide timing of delivery. Our objective was to develop a clinical care pathway for prenatal management of uncomplicated gastroschisis at our institution. METHODS: We performed a review of literature from January 1996 to May 2017 to evaluate prenatal ultrasound (US) markers and surveillance strategies that help determine timing of delivery and optimize outcomes in fetal gastroschisis. RESULTS: A total 63 relevant articles were identified. We found that among the US markers, intraabdominal bowel dilatation, polyhydramnios, and gastric dilatation are potentially associated with postnatal complications. Prenatal surveillance strategy with monthly US starting at 28weeks of gestational age (wGA) and twice weekly non-stress testing beginning at 32wGA is recommended to optimize fetal wellbeing. Timing of delivery should be based on obstetric indications and elective preterm delivery prior to 37wGA is not indicated. CONCLUSIONS: Close prenatal surveillance of fetal gastroschisis is necessary due to the high risk for adverse outcomes including intrauterine fetal demise in the third trimester. Decisions regarding the timing of delivery should take into consideration the additional prematurity-associated morbidity.

Proceso de atención de Enfermería en un paciente con gastroquisis. A propósito de un caso / Nursing care process in a patient with gastrochisis. Aproposs of a case

RESUMEN La gastroquisis, es un defecto congénito de cierre de la pared abdominal, en general justa a la derecha de la insertación normal del cordón umbilical en la pared del abdomen. Rara vez ocupa una posición especular a la izquierda del cordón umbilical; una cantidad variable de intestinos, y en ocasiones otros órganos abdominales se hernian, fuera de la pared abdominal, sin saco ni cobertura membranosa a diferencia del onfalocele. El Proceso de Atención de Enfermería, es el método científico de actuación de los profesionales de la Enfermería, vinculados a una base teórica que desarrollan habilidades analíticas que aplicadas en la práctica, repercuten en la mejoría de los pacientes. El objetivo de este trabajo es describir un caso poco frecuente de gastroquisis, y vincularlo al proceso de atención de Enfermería. Se trató de una recién nacida, sexo femenino, raza mestiza. Ingresó en la Sala de Terapia Intensiva del Hospital Pediátrico Provincial Eliseo Noel Caamaño de Matanzas con 5 horas de nacida. La vinculación de este caso con el Proceso de Atención de Enfermería permitió una atención integral al individuo enfermo, y desarrolló las habilidades prácticas de la asistencia de enfermería (AU).

ABSTRACT Gastrochisis is a congenital defect of the abdominal wall closure, generally just on the right of the umbilical cord normal insertion in the abdominal wall. It rarely occupies a specular position to the right of the umbilical cord; a variable amount of intestines, and sometimes other abdominal organs, are herniated outside the abdominal wall, without a sac or a membranous covering unlike the omphalocele. The nursing care process is a scientific method of nursing professionals behavior, on a theoretical basis, developing analytical skills that, applied in the practice, have repercussion on the patients´ improvement. Describing an infrequent case of gastrochisis, and linking it to the Nursing care process is the aim of this article. The patient was a mestizo female newborn who entered the Intensive Care Ward of the Pediatric Provincial Hospital "Eliseo Noel Caamaño" of Matanzas five hours after birth. Linking this case to the Nursing care process allowed the integral care of the sick person, and developed the nursing care practical skills (AU).

Decentralized surgery of abdominal wall defects in Germany.

PURPOSE: Neonatal surgery for abdominal wall defects is not performed in a centralized manner in Germany. The aim of this study was to investigate whether treatment for abdominal wall defects in Germany is equally effective compared to international results despite the decentralized care. METHODS: All newborn patients who were clients of the major statutory health insurance company in Germany between 2009 and 2013 and who had a diagnosis of gastroschisis or omphalocele were included. Mortality during the first year of life was analysed. RESULTS: The 316 patients with gastroschisis were classified as simple (82%) or complex (18%) cases. The main associated anomalies in the 197 patients with omphalocele were trisomy 18/21 (8%), cardiac anomalies (32%) and anomalies of the urinary tract (10%). Overall mortality was 4% for gastroschisis and 16% for omphalocele. Significant factors for non-survival were birth weight below 1500 g for both groups, complex gastroschisis, volvulus and anomalies of the blood supply to the intestine in gastroschisis, and female gender, trisomy 18/21 and lung hypoplasia in omphalocele. CONCLUSIONS: Despite the fact that paediatric surgical care is organized in a decentralized manner in Germany, the mortality rates for gastroschisis and omphalocele are equal to those reported in international data.

Major abdominal wall defects in the low- and middle-income setting: current status and priorities.

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30-100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.

Thoracic gout tophus with abdominal wall protrusion: A case report.

RATIONALE: A patient presented the abdominal wall protrusion due to tophaceous gout of the spine. Similar cases were not reported in the literature. This study aimed to report a case of tophaceous gout of the spine with abdominal wall protrusion. PATIENT CONCERNS: A 38-year-old male patient had a 10-year history of gout and hyperuricemia. He complained of back pain and abdominal wall protrusion. DIAGNOSES: The patient was diagnosed with tophaceous gout of the spine with abdominal wall weakness caused by T11 nerve root compression. INTERVENTIONS: A semi-lamina decompression was performed at T11-T12. The pathological examination of the specimen demonstrated tophaceous gout of the spine. OUTCOMES: After the surgery, the patient's back pain was completely relieved and the abdominal wall weakness significant improved. LESSONS: This case highlighted that axial gout could mimic thoracic disk herniation clinically. The abdominal wall weakness might also be due to single T11 nerve compression by the tophaceous gout of the spine. In patients with a history of gout, axial gout should be considered as one of the differential diagnoses.

An Algorithmic Approach to Complex Fetal Abdominal Wall Defects.

OBJECTIVE. The purpose of this article is to describe the imaging findings associated with complex fetal abdominal wall defects and provide an algorithmic method for arriving at a final diagnosis. CONCLUSION. Fetal ventral abdominal wall defects are a complex group of conditions with a broad spectrum of associated multisystem anomalies and manifestations. Correct characterization and classification of these defects require not only familiarity with imaging findings but also a systematic approach to avoid diagnostic confusion.

La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos / The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects

La supervivencia de los pacientes con antecedentes de malformaciones congénitas complejas ha aumentado en las últimas décadas. El pediatra de atención primaria debe conocer los problemas más habituales que pueden presentar este grupo de pacientes. Además, puede ofrecer una visión global que, a menudo, se pierde en las consultas especializada. En este trabajo se recogen algunas de las malformaciones congénitas digestivas y respiratorias más habituales, como atresia de esófago, defectos de pared abdominal, malformación anorrectal y enfermedad de Hirschsprung, y hernia diafragmática congénita. Se señalan los problemas de mayor interés para el pediatra, haciendo hincapié en las complicaciones a largo plazo y en todas las dimensiones de la persona

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual

[The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects]. / La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos.

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual.