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      Tratamiento de los defectos de la pared abdominal (gastrosquisis y onfalocele) en el Hospital Universitario San Vicente de Paúl, Medellín, 1998-2006 Translated title: Management of abdominal wall defects (gastroschisis and omphalocele) at Hospital Universitario San Vicente de Paúl, in Medellín, Colombia, 1998-2006

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          Abstract

          Introducción:la gastrosquisis y el onfalocele son malformaciones de la pared abdominal en neonatos que, a pesar de sus grandes diferencias, tienen en común el hecho de ser enfermedades graves caracterizadas por la herniación de las vísceras intrabdominales a través de un defecto de la pared abdominal. Los niños con estas enfermedades se presentan como emergencias quirúrgicas que plantean un reto difícil para el cirujano tratante. Tienen una tasa de mortalidad que oscila entre 20- 40%, aun con el tratamiento apropiado y se asocian a un amplio rango de malformaciones, principalmente en los niños con onfalocele. Objetivo: el objetivo de la presente revisión retrospectiva es describir el tratamientode los pacientes con gastrosquisis y onfalocele, y los resultados con él obtenidos, entre 1998 y 2006, en la Sección de Cirugía Pediátrica del Hospital Universitario San Vicente de Paúl (HUSVP), de Medellín. Pacientes y métodos: se evaluaron todos los pacientes que ingresaron al Servicio de Cirugía Pediátrica del HUSVP con diagnóstico de gastrosquisis u onfalocele, entre el 1 de enero de 1998 y el 31 de diciembre de 2006. Se definió el tipo de tratamiento llevado a cabo y, de acuerdo con este, se revisaron los resultados: las complicaciones posquirúrgicas, tales como infección del sitio operatorio, evisceración, sepsis, íleo e hipertensión intrabdominal; el tiempo de inicio de la vía oral y de la nutrición parenteral total (NPT); la permanencia en la unidad de cuidados intensivos (UCI) y la duración de la estancia hospitalaria. Resultados: se identificaron 55 pacientes, 32 con gastrosquisis y 23 con onfalocele; en todos se hizo tratamiento quirúrgico. En 31 pacientes (56,4%) se hizo cierre primario y en 24 (43,6%), cierre por etapas; en esta última modalidad el procedimiento más utilizado fue el silo (12 niños; (50%). En 42 pacientes (76,4%) se presentaron complicaciones la más frecuente de las cuales fue la sepsis. La frecuencia de complicaciones asociadas al procedimiento quirúrgico fue similar para el cierre primario y el cierre por etapas (49,9% y 49,7%, respectivamente). El inicio de la vía oral fue más temprano para los pacientes tratados con cierre primario. Los pacientes con gastrosquisis requirieron mayor tiempo de estancia en la UCI y en el hospital. Murieron 16 pacientes (29,1%); la mortalidad fue más alta en los que tenían onfalocele (10/23; 43,5%) que en los con gastrosquisis (6/32; 18,8%).

          Translated abstract

          Introduction: Gastroschisis and omphalocele are neonatal malformations of the abdominal wall. Despite their great differences, both are severe diseases characterized by herniation of viscera through the defect in the abdominal wall. Children with these defects present as surgical emergencies that pose a difficult challenge to the attending surgeon. Even with appropriate management, the mortality rate is between 20-40%. Omphalocele and, to a lesser degree gastroschisis, are associated with a wide range of malformations. Objective: The aim of this retrospective review was to describe the management of children with gastroschisis or omphalocele, and the results obtained with it, at the Pediatric Surgery Section, Hospital Universitario San Vicente de Paúl, in Medellin, Colombia. Patients and methods: We evaluated the charts of all patients admitted to the Pediatric Surgery Section, between January 1, 1998 and December 31, 2006, with a diagnosis of gastroschisis or omphalocele. The type of treatment was defined as either primary closure or closure by stages; accordingly, we reviewed the results of the operation, the surgical complications (surgical site infection, evisceration, sepsis, ileus and intraabdominal hypertension), the time of onset of oral and total parenteral nutrition (TPN), and the duration of hospital and UCI stay. Results: 55 patients were identified, 32 with gastroschisis and 23 with omphalocele, all of whom were surgically treated. In 31 patients (56.4%) primary closure was carried out, while in 24 ( 43.6%) the closure was done by stages; in the latter modality silo was most frequently used (12 cases). Complications, mostly sepsis, occurred in 42 patients (76.4%). The frequency of complications associated with the surgical procedure was similar for primary closure (49.9%) and for closure by stages (49.7%). Onset of the oral route was earlier in patients treated by primary closure. Patients with gastroschisis required longer hospital and UCI stays. Sixteen patients died (29.1%); mortality was higher in those with omphalocele (10/23; 43.5%) than in those with gastroschisis (6/32; 18.8%).

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          Most cited references32

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          Increasing prevalence of gastroschisis in Europe 1980-2002: a phenomenon restricted to younger mothers?

          Gastroschisis is an abdominal wall defect more prevalent in offspring of young mothers. It is known to be increasing in prevalence despite the general decrease in the proportion of births to young European women. We investigated whether the increase in prevalence was restricted to the high-risk younger mothers. We analysed 936 cases of gastroschisis from 25 population-based registries in 15 European countries, 1980-2002. We fitted a Bayesian Hierarchical Model which allowed us to estimate trend, to estimate which registries were significantly different from the common distribution, and to adjust simultaneously for maternal age, time (in grouped years) and the random variation between registries. The maternal age-standardised prevalence (standardised to the year 2000 European maternal age structure) increased almost fourfold from 0.54 [95% Credible Interval (CrI) 0.37, 0.75] per 10,000 births in 1980-84 to 2.12 [95% CrI 1.85, 2.40] per 10,000 births in 2000-02. The relative risk of gastroschisis for mothers <20 years of age in 1995-2002 was 7.0 [95% CrI 5.6, 8.7]. There were geographical differences within Europe, with higher rates of gastroschisis in the UK, and lower rates in Italy after adjusting for maternal age. After standardising for regional variation, our results showed that the increase in risk over time was the same for mothers of all ages--the increase for mothers <20 years was 3.96-fold compared with an increase of 3.95-fold for mothers in the other age groups. These findings indicate that the phenomenon of increasing gastroschisis prevalence is not restricted to younger mothers only.
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            Omphalocele and gastroschisis and associated malformations.

            The etiology of gastroschisis and omphalocele is unclear and their pathogenesis is controversial. Because previous reports have inconsistently noted the type and frequency of malformations associated with omphalocele and gastroschisis, we assessed these associated malformations ascertained between 1979 and 2003 in 334,262 consecutive births. Of the 86 patients with omphalocele, 64 (74.4%) had associated malformations. These included patients with chromosomal abnormalities (25, 29.0%); non-chromosomal syndromes including Beckwith-Wiedemann syndrome, Goltz syndrome, Marshall-Smith syndrome, Meckel-Gruber syndrome, Oto-palato-digital type II syndrome, CHARGE syndrome, and fetal valproate syndrome; malformation sequences, including ectopia cordis, body stalk anomaly, exstrophy of bladder, exstrophy of cloaca, and OEIS (Omphalocele, Exstrophy of bladder, Imperforate anus, Spinal defect); malformation complexes including Pentalogy of Cantrell, and non-syndromic multiple congenital anomalies (MCA) (26, 30.2%). Malformations of the musculoskeletal system (31, 23.5%), urogenital system (27, 20.4%), cardiovascular system (20, 15.1%), and central nervous system (12, 9.1%) were the most common other congenital malformations in patients with omphalocele and non-syndromic MCA. Of the 60 patients with gastroschisis, 10 (16.6%) had associated malformations. In contrast to omphalocele, gastroschisis was rarely associated with a complex pattern of malformation, that is, one each (1.7%) with a chromosomal abnormality (trisomy 21), sequence (amyoplasia congenita), unspecified dwarfism, and 7 (11.7%) with MCA. We observed a striking difference in the prevalence of total malformations (74.4% vs. 16.6%, P < 0.001) and specific patterns of malformations associated with omphalocele and gastroschisis which emphasizes the need to evaluate all patients with omphalocele and gastroschisis for possible associated malformations. Malformation surveillance programs should be aware that the malformations associated with omphalocele can be often classified into a recognizable malformation syndrome or pattern (44.2%). 2008 Wiley-Liss, Inc.
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              Current outcome of antenatally diagnosed exomphalos: an 11 year review.

              The aim of this study was to determine the outcome of antenatally diagnosed exomphalos. The database of a tertiary referral Fetal Medicine Centre was searched for all cases of antenatally diagnosed exomphalos between January 1991 and December 2002. Patients, general practitioners, and hospitals were contacted for outcome details. In total, 445 cases of exomphalos were identified. In 250 (56%) cases, the fetal karyotype was abnormal (group A), in 135 (30%) cases, the karyotype was normal (group B), and in 60 (14%) cases, karyotyping was declined (group C). In group A, there were 248 (99%) terminations of pregnancy (TOP) or fetal deaths and 2 live births. In group B, 74 (54%) fetuses had other structural anomalies; 82 (61%) pregnancies resulted in TOP or fetal death, 42 (31%) in live births, and 11 (8%) were lost to follow-up. In group C, 38 (63%) fetuses had other structural anomalies; 41 (69%) pregnancies resulted in TOP or fetal death, 11 (18%) in live births, and 8 (13%) were lost to follow-up. Of the 55 live births, 11 died preoperatively and 44 had surgery. There were no postoperative deaths. Less than 10% of the antenatal diagnostic workload reached operative repair. In our unit, these babies are a highly selected group, which is a factor in the high postoperative survival.
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                Author and article information

                Journal
                iat
                Iatreia
                Iatreia
                Universidad de Antioquia (Medellín, Antioquia, Colombia )
                0121-0793
                September 2010
                : 23
                : 3
                : 220-226
                Affiliations
                [03] Medellín orgnameHospital Universitario San Vicente de Paúl orgdiv1Hospital Pablo Tobón Uribe Colombia
                [02] Medellín orgnameUniversidad de Antioquia orgdiv1Facultad de Medicina Colombia
                [01] Medellín orgnameUniversidad de Antioquia orgdiv1Facultad de Medicina Colombia
                Article
                S0121-07932010000300004 S0121-0793(10)02300304
                20b3288b-78d6-4d0b-8909-2f3431e19559

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 02 June 2009
                : 12 April 2010
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 32, Pages: 7
                Product

                SciELO Colombia

                Self URI: Texto completo solamente en formato PDF (ES)
                Categories
                Investigación Original

                Silo,Abdominal wall defects in infants,Flat bag,Gastroschisis,Mesh,Omphalocele,Primary closure,Bolsa plana,Cierre primario,Defectos de la pared abdominal en neonatos,Gastrosquisis,Malla,Onfalocele

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