Atresia duodenal con apple peel asociada a herniadiafragmática congénita: un caso excepcional yrevisión de la literatura / Duodenal atresia with apple peel associated with congenital diaphragmatic hernia: an exceptional case and a literature review

Introducción: La atresia duodenal asociada con appel peel es extremadamente infrecuente. La primera se produce por un defecto en larecanalización en etapas tempranas, mientras que la atresia intestinalparece deberse a causas vasculares en etapas más tardías. La presenciade anomalías asociadas a la hernia diafragmática es común, pero laasociación con la atresia duodenal apenas está descrita.Caso clínico: Presentamos un recién nacido de 31 semanas de gestación y sexo femenino, con atresia duodenal y apple peel, asociada ahernia diafragmática izquierda y cardiopatía mayor. Se realizó un flapmuscular abdominal para el cierre del defecto diafragmático y anastomosis duodenoyeyunal tras la resección de parte del apple peel inviable.Comentarios: A nuestro entender, es el primer caso descrito conesta asociación singular. La combinación de atresia duodenal con apple peel se ha descrito previamente en 11 ocasiones; la asociación de ambascon hernia diafragmática congénita no había sido descrita.

Introduction: Duodenal atresia associated with apple peel is extremely rare. Duodenal atresia occurs as a result of absence of recanali-zation at an early stage, whereas intestinal atresia is seemingly due tovascular causes at later stages. The presence of abnormalities associatedwith diaphragmatic hernia is frequent, but association with duodenalatresia has been little explored.Care report: This is the case of a female neonate born at gestational week 31, with duodenal atresia and apple peel, associated withleft diaphragmatic hernia and major heart disease. An abdominal muscle flap was performed for diaphragmatic defect closure purposes, and duodenojejunal anastomosis was carried out following resection of partof the non-viable apple peel.Discussion: To our knowledge, this is the first case described with this rare association. The combination of duodenal atresia and applepeel had been previously described 11 times. However, the associationof both with congenital diaphragmatic hernia had not been reported yet.

Diagnóstico prenatal de atresia duodenal en feto con Síndrome Down. A propósito de un caso / Prenatal diagnosis of duodenal atresia in fetus with Down syndrome. About a case

RESUMEN La atresia duodenal (AD) es una patología congénita infrecuente. Es el resultado de una falla en la recanalización del duodeno y se puede encontrar asociada a cromosomopatías como el Síndrome Down en el 30% de los casos. El síndrome de down se produce por una trisomía del par cromosómico 21 y es la aberración cromosómica más frecuente. Debido a la necesidad de contar con reportes de esta alteración presentamos el diagnóstico prenatal por ultrasonido y estudio citogenético de una paciente de 25 años de edad a la que a las 28 semanas de gestación se le detectó en abdomen fetal una imagen ecolúcida dual por debajo del estómago (Imagen en doble Burbuja) compatible con atresia duodenal. Por sospecha de asociación de este tipo de malformación con enfermedad cromosómica, se le realizó diagnóstico prenatal citogenético, cuyo resultado fue: 47,XX, +21, inv(9)(p11;q12), feto femenino positivo para una trisomía 21 e inversión pericéntrica del cromosoma 9. Luego del asesoramiento genético, la pareja optó por la interrupción excepcional de la gestación. Se confirmó por anatomía patológica la presencia de la atresia duodenal y rasgos dismórficos compatibles con el síndrome down.

ABSTRACT Duodenal atresia (AD) is a rare congenital pathology. It is the result of a failure in the recanalization of the duodenum and can be found associated with chromosomopathies such as Down syndrome in 30% of cases. Down syndrome is caused by a trisomy of the 21 chromosome pair and is the most frequent chromosomal aberration. Due to the need to have reports of this alteration, we present the prenatal diagnosis by ultrasound and a cytogenetic study of a 25-year-old patient who, at 28 weeks pregnancy, had a dual echolucent image below the fetal abdomen under the stomach (double bubble image) compatible with duodenal atresia. On the bases of the suspicion of the association type of malformation with chromosomal disease, a prenatal cytogenetic diagnosis was performed, resulting: 47, XX, +21, inv (9) (p11; q12), female fetus positive for trisomy 21 and pericentric inversion of chromosome 9. After genetic counseling, the couple opted for the exceptional pregnancy interruption. The presence of duodenal atresia and dysmorphic features compatible with down syndrome were confirmed by pathological anatomy.

Anatomical variations of the superior mesenteric artery and its clinical and surgical implications in humans / Variações anatômicas da artéria mesentérica superior e suas implicações clínicas e cirúrgicas em humanos

ABSTRACT Introduction: Superior mesenteric artery (SMA) usually arises from the abdominal aorta, just below the celiac trunk and it supplies the midgut-derived embryonic structures. Anatomical variations in this vessel contribute to problems in the formation and/or absorption of this part of the intestine and its absence has been recognized as the cause of congenital duodenojejunal atresia. Objective: To analyze SMA anatomical variations in humans and the possible associated clinical and surgical implications. Methods: This is a systematic review of papers indexed in PubMed, SciELO, Springerlink, Science Direct, Lilacs, and Latindex databases. The search was performed by two independent reviewers between September and December 2018. Original studies involving SMA variations in humans were included. SMA presence/absence, level, place of origin and its terminal branches were considered. Results: At the end of the search, 18 studies were selected, characterized as for the sample, method to evaluate the anatomical structure and main results. The most common type of variation was when SMA originated from the right hepatic artery (6.13%). Two studies (11.11%) evidenced the inferior mesenteric artery originating from the SMA, whereas other two (11.11%) found the SMA sharing the same origin of the celiac trunk. Conclusion: SMA variations are not uncommon findings and their reports evidenced through the scientific literature demonstrate a great role for the development of important clinical conditions, making knowledge about this subject relevant to surgeons and professionals working in this area.

RESUMO Introdução: A artéria mesentérica superior (AMS), normalmente, tem sua origem a partir da aorta abdominal, um pouco abaixo do tronco celíaco e é responsável pela irrigação das estruturas derivadas, embrionariamente, do intestino médio. Variações anatômicas nesse vaso contribui para defeitos na formação e/ou absorção dessa parte do intestino e a sua ausência tem sido reconhecida como a causa da atresia duodenojejunal congênita. Objetivo: Analisar as variações anatômicas dela em humanos e as possíveis implicações clínicas e cirúrgicas associadas. Métodos: Trata-se de uma revisão sistemática de artigos indexados nas bases de dados PubMed, SciELO, Springerlink, Scienc Direct, Lilacs e Latindex. A busca ocorreu por dois revisores independentes entre setembro e dezembro de 2018. Foram incluídos artigos originais envolvendo as variações da AMS em humanos. Considerou-se para este estudo a presença/ausência da AMS, o nível, local de origem e seus ramos terminais. Resultados: Ao final da busca foram selecionados 18 artigos, caracterizados quanto à amostra, método para avaliar a estrutura anatômica e principais resultados. O tipo de variação mais comum foi aquele cuja AMS se originou da artéria hepática direita (6,13%). Dois estudos (11,11%) evidenciaram a artéria mesentérica inferior originando-se a partir da AMS, enquanto outros dois (11,11%) constataram ser ela compartilhada na mesma origem do tronco celíaco. Conclusão: Variações na AMS não são achados incomuns e seus relatos evidenciados através da literatura científica demonstram grande importância para o desenvolvimento de condições clínicas importantes, tornando o conhecimento sobre esse assunto relevante para os cirurgiões e profissionais atuantes nesta área.

Estado actual de la gastrosquisis en recién nacidos en el Hospital Nacional Daniel A. Carrión: Serie de casos de 5 años / Gastroschisis in newborns at Hospital Nacional Daniel A. Carrión: Case series in 5 years

La gastrosquisis es un defecto congénito de la pared abdominal que permite la exteriorización del contenido abdominal, cuyo pronóstico dependerá de las complicaciones intestinales asociadas. Es importante la detección prenatal de aquellos casos con riesgo de gastrosquisis compleja. Objetivo. Describir las características epidemiológicas de los recién nacidos con gastrosquisis atendidos en un hospital nacional. Diseño. Serie de casos. Institución. Hospital Nacional Daniel A. Carrión (HNDAC), Lima, Perú. Pacientes. Recién nacidos vivos. Metodología. Estudio de todos los recién nacidos vivos con el diagnóstico de gastrosquisis, en un periodo de 5 años. Se excluyó aquellos con nacimiento extrainstitucional o con datos insuficientes. Principales medidas de resultados. Características y manejo de los recién nacidos con gastrosquisis. Resultados. Se reportaron 17 casos. La incidencia fue 9,7/1 000 nacidos vivos y la mortalidad perinatal 14%; 64,3% nacieron de madres adolescentes, 78% primigestas. El nacimiento ocurrió principalmente por cesárea (71,4%). Existió un pobre registro de predictores ecográficos de complicaciones intestinales. Se realizó cierre primario en 28,6% de los casos. El primer caso de mortalidad ocurrió luego de manejo quirúrgico con silo, falleciendo por sepsis. El segundo caso presentó atresia intestinal requiriendo múltiples cirugías y falleció por atelectasia masiva. Conclusiones. Es necesario estandarizar la valoración y registro de los diversos marcadores pronósticos en gastrosquisis fetal. Es necesario establecer series más largas y mejor documentadas.

Gastroschisis is a congenital defect of the abdominal wall that allows externalization of the abdominal contents; prognosis depends on the associated intestinal complications. Prenatal detection of cases with risk of complex gastroschisis is important. Objective: To describe the epidemiological characteristics of newborns with gastroschisis occurring at a national hospital. Design: Case series. Institution: Hospital Nacional Daniel A. Carrion (HNDAC), Lima, Peru. Patients: Newborns with gastroschisis. Methodology: All live newborns with the diagnosis of gastroschisis in a period of 5 years were studied. Cases born in other institutions or with insufficient data were excluded. Main outcome measures: Characteristics of newborns with gastroschisis and their management. Results: 17 cases were reported with an incidence of 9.7/1 000 live newborns and with a perinatal mortality of 14%; 64.3% were born to an adolescent mother, of which 78% carried their first pregnancy. The main delivery mode was cesarean section (71.4%). There were poor records of ultrasonographic predictors of intestinal complications. Primary closure was performed in 28.6% of cases. The first case of death occurred after surgery with silo and died from sepsis. The second case had intestinal atresia requiring multiple surgeries and died due to massive atelectasis. Conclusions: It is necessary to standardize the assessment and registration of various prognostic markers in fetal gastroschisis. More and better documented series are necessary.

Hirschsprung disease and hepatoblastoma: case report of a rare association / Doença de Hirschsprung e hepatoblastoma: relato de caso de uma associação rara

ABSTRACT CONTEXT: Hirschsprung disease is a developmental disorder of the enteric nervous system that is characterized by absence of ganglion cells in the distal intestine, and it occurs in approximately 1 in every 500,000 live births. Hepatoblastoma is a malignant liver neoplasm that usually occurs in children aged 6 months to 3 years, with a prevalence of 0.54 cases per 100,000. CASE REPORT: A boy diagnosed with intestinal atresia in the first week of life progressed to a diagnosis of comorbid Hirschsprung disease. Congenital cataracts and sensorineural deafness were diagnosed. A liver mass developed and was subsequently confirmed to be a hepatoblastoma, which was treated by means of surgical resection of 70% of the liver volume and neoadjuvant chemotherapy (ifosfamide, cisplatin and doxorubicin). CONCLUSION: It is known that Hirschsprung disease may be associated with syndromes predisposing towards cancer, and that hepatoblastoma may also be associated with certain congenital syndromes. However, co-occurrence of hepatoblastoma and Hirschsprung disease has not been previously described. We have reported a case of a male patient born with ileal atresia, Hirschsprung disease and bilateral congenital cataract who was later diagnosed with hepatoblastoma.

RESUMO CONTEXTO: A doença de Hirschsprung é uma desordem do desenvolvimento do sistema nervoso entérico, que é caracterizada pela ausência de células ganglionares no intestino distal, ocorrendo em cerca de 1 a cada 500.000 nascimentos. O hepatoblastoma é uma neoplasia maligna do fígado que geralmente ocorre em crianças de 6 meses a 3 anos, com prevalência de 0,54 casos por 100.000. RELATO DE CASO: Um menino com diagnóstico de atresia intestinal na primeira semana de vida evoluiu com diagnóstico concomitante de doença de Hirschsprung. Catarata congênita e surdez neurossensorial foram diagnosticadas. Surgiu lesão hepática com posterior confirmação de hepatoblastoma, tratado com ressecção cirúrgica de 70% do volume hepático e quimioterapia neoadjuvante (ifosfamida, cisplatina e doxorubicina). CONCLUSÃO: Sabe-se que a doença de Hirschsprung pode estar associada a síndromes de predisposição ao câncer, da mesma forma que o hepatoblastoma já foi correlacionado a certas síndromes congênitas malformativas. No entanto, até o momento, a associação de hepatoblastoma com a doença de Hirschsprung não foi descrita. Relatamos o caso de um menino que nasceu com atresia ileal, doença de Hirschsprung, catarata congênita bilateral e com posterior diagnóstico de hepatoblastoma.

Estenosis congénita de íleon en un lactante de 5 meses de edad con síndrome de obstrucción intestinal / Congenital stenosis of the ileum in a 5-month-old infant with intestinal obstruction syndrome

Introducción. La atresia intestinal se refiere a la obstrucción completa de la luz del intestino y la estenosis al bloqueo parcial de ésta. Ocurre un caso por cada 4,000 a 5,000 nacimientos y no existen diferencias de presentación en cuanto al sexo. Cuando la obstrucción es incompleta los signos como vómito, la distensión abdominal y el estreñimiento pueden aparecer poco tiempo después del nacimiento o retrasarse de forma indeterminada. No se presentan grandes dificultades para diagnosticar la atresia intestinal; en cambio es más complicado diagnosticar la estenosis. El síndrome obstructivo del lactante obliga a descartar la estenosis congénita intestinal. Se han presentado casos en edades más avanzadas aunque esto es raro. Caso clínico. Se presenta el caso de un paciente femenino de 5 meses de edad con un cuadro clínico de obstrucción intestinal que fue manejado quirúrgicamente; se encontró una estenosis congénita de íleon. La paciente evolucionó sin complicaciones. Conclusiones. El síndrome obstructivo del lactante por estenosis intestinal es una entidad rara que no suele sospecharse de primera instancia; sin embargo, debe descartarse al realizar el protocolo de estudio para obstrucción intestinal.

Background. Intestinal atresia refers to complete obstruction or partial stenosis of the intestinal lumen. The prevalence is 1/4000-5000 births, without gender predilection. When the blockage is incomplete, signs such as vomiting, abdominal distension, and constipation may occur shortly after birth or delayed in an unspecifed manner. In intestinal atresia, the challenge may not be significant; however, stenosis may pose considerable diffculties. Obstructive syndrome must be ruled out in infant congenital intestinal stenosis. Even more rare cases have occurred during later ages. Case Report. We report on a 5-month-old female infant with clinical symptoms of intestinal obstruction, which was managed surgically. Surgical finding was a congenital stricture of the ileum. The patient recovered without complications. Conclusions. Obstructive syndrome in infant intestinal stenosis is a rare entity, which is usually not first suspected, but it should be ruled out as a study protocol for intestinal obstruction.

Atresia duodenal con fístula esofagopleural: reporte de caso / Esophago-pleural fistula with duodenal atresia: case report

Las fístulas esofagopleurales en el recién nacido son malformaciones raras, cuya etiología aún se desconoce, y cuya asociación a la atresia duodenal está pobremente documentada. Se reporta en el presente artículo el caso de un recién nacido con múltiples malformaciones congénitas, en quien se diagnosticó una fístula esofagopleural asociada a atresia duodenal. Además, se revisa brevemente la literatura disponible, con énfasis en la posible fisiopatología, así como en las posibles manifestaciones clínicas y radiológicas de este tipo de fístulas.

Esophago-pleural fistulas have been poorly understood and only a few cases of its association with duodenal atresia have been described. This case report describes a newborn with multiple congenital malformations; who exhibits poor clinical evolution with hydroneumothorax and an esophago-pleural fistula with duodenal atresia. In this particular case, we review the possible physiopathology, clinical and radiological manifestations of this rare type of fistulas.

Relato raro de atresia jejunoileal em dois irmãos nascidos com três anos de diferença / Rare jejunoileal atresia in two singleton brothers born three years apart

A atresia do intestino delgado é malformação congênita, conhecida como causa comum de obstrução intestinal no período neonatal. A atresia duodenal familiar é extremamente rara e tem sido atribuída a aberrações cromossômicas e a consaguinidade, sugerindo herança autossômica recessiva. As anomalias intestinais distais, como a atresia jejunoileal,são malformações congênitas raras do intestino delgado e têm sido relacionadas a oclusões vasculares tanto no início quanto no final da gestação além de causas genéticas. A atresia jejunoileal familiar em gêmeos é de ocorrência extremamente rara e é relacionada a algumas drogas e tinturas que são instiladas durante a gestação para diagnósticopor amniocentese e tem sido descrita como causa da atresia jejunoileal em irmãos gêmeos. Autores de dois diferentes estudos descrevem que esta atresia é significantemente mais frequente em gêmeos que em irmãos nascidos separadamente. No presente artigo descrevemos dois casos extremamente raros em dois irmãos nascidos com três anosde diferença, ambos com atresia jejunoileal sem outras malformações associadas ou anomalias cromossômicas, que foram acompanhados pela equipe em 2004 e 2007, respectivamente. Apesar das investigações, não foram encontradas nenhuma razão para essa ocorrência. Contudo, os autores estão seguindo os dois meninos desde então e não detectaramalterações no desenvolvimento de nenhum deles que pudessem sugerir qualquer malformação associada.

Small intestinal atresia is a common congenital malformation and it is a well-known cause of intestinal obstruction in neonates. Familial occurrence of duodenal atresia is extremely rare and has been attributed to chromosomal aberrations and parental consanguinity suggesting autosomal recessive inheritance. Distal intestinal anomalies, such as jejunoileal atresia, are a rare congenital malformation of the small bowel and have been related to vascular occlusion in the earlier or later stages in pregnancy and genetic causes. Familial jejunoileal atresia in twins is an extremely rareoccurrence that is attributed to the use of some chemicals and other dyes instillated during diagnostic amniocentesis and has been described as a cause of jejunoileal atresia in twin-brothers. Authors of two different research institutes stated that jejunoileal atresia is significantly more frequent in twins than in singletons. In the present article we describean extremely rare occurrence in two singleton infants, who were born three years apart, with similar jejunoileal atresia with no other associated malformations or chromosomal anomalies, who were treated in 2004 and 2007, respectively. Despite investigation, we did not find any reason for this particular occurrence; however they will be closely followed inorder to detect any development alterations that could indicate an associated malformation.

El manejo de la atresia intestinal tipo IIIb sigue siendo un reto / The management of Type III intestinal atresia still remains being a challenge

La atresia intestinal constituye una de las causas más frecuentes de obstrucción intestinal en el recién nacido y es causada por catástrofes vasculares mesentéricas tardías durante el desarrollo embrionario; sin embargo, puede tener otras causas. La atresia de tipo IIIb es rara y su manejo es un reto para todo el equipo médico. Este tipo de atresia sigue siendo de difícil manejo y requiere un equipo completo interdisciplinario para obtener un buen resultado: en primer lugar, lograr la supervivencia y, posteriormente, lograr un crecimiento y un desarrollo normales. La técnica quirúrgica incluye una anastomosis término-terminal; en complicaciones como las del caso presentado, se recurre a las plastias en YV, y a la colocación de férula en el intestino con sonda fenestrada para proteger la anastomosis, con el fin de mantener descomprimido el intestino y permeable distalmente.

Intestinal atresia constitutes one of the most frequent causes of intestinal obstruction in the newborn and it is caused by delayed mesenteric vascular catastrophes during embryonic development; nevertheless, it may have other causes. Atresia type IIIb is rare and its management is a challenge for all of the members of the medical team. This type of atresia continues being of difficult management, and needs a complete multidisciplinary equipment in order to attain good results: firstly, to ensure the survival of the child and subsequently; to achieve normal growth and development. The surgical technique includes endto- end anastomoses and, in complications like those presented in this case, we place YV shaped plasty and making an intestinal splint with a fenestrated probe in order to protect the anastomosis, maintaining bowel decompressed and distally-permeable.

Un caso raro de malformación del tubo neural: síndrome de hendidura notocordal / A rare case of neural tube defect: split notochord syndrome

Se trata de una embarazada de 33 años de edad, II gesta, I para, con 27 semanas de gestación, referida con el diagnóstico de ascitis fetal. Al estudio ecosonográfico se encuentra polihidramnios, abdomen fetal distendido ocupado por una gran masa líquida que inicialmente impresiona como ascitis fetal, además se encuentra intestino ecogénico compatible con peritonitis meconial y polimicrogiria. En una posterior evaluación ultrasonográfico se observa defecto en columna vertebral a nivel del sacro de donde emerge una imagen anecogénica equivalente a la conocida "espina bífida anterior" corroborándose restos de hallazgos ecográficos anteriores. A las 33 semanas de gestación se realiza cesárea extrayéndose recién nacido vivo, masculino, 2820 g y 45 cm de talla, con puntuación de Apgar de 6 y 7 al 1 y 5 minuto de vida respectivamente. Presentó síndrome de distrés respiratorio transitorio y sintomatología de obstrucción intestinal, la cual fue resuelta quirúrgicamente. Atresia intestinal y se confirma el síndrome de hendidura notocordal. El recién nacido falleció por sepsis. No se realizó autopsia. Los hallazgos ultrasonográficos dependen del tipo de lesión, pero frecuentemente se reportan como quistes abdomino-torácicos, mielo-meningocele y espina bífida. El pronóstico generalmente es ominoso, pero dependerá de la extensión de las lesiones y anomalías asociadas

A 33 years old woman, gravida II, para I, was referred at 27 weeks gestation with the diagnosis of fetal ascites. Ultrasound report showed, polyhydramnios, fetal abdomen distended occupied by a large liquid mass initially impressed as fetal ascites, echogenic bowel is also consistent with meconium peritonitis and polymicrogiria. In a subsequent ultrasonography was observed column defect on the sacrum where emerges a cystic mass equivalent to the known "anterior spina bifida", others previous ultrasound findings were confirmed. Cesarean section was performed at 33 weeks gestation, obtaining male live newborn, 2820 g and 45 cm in length, with Apgar score of 6 y 7 at 1 and 5 minutes, respectively. The newborn presented transient distress respiratory syndrome and symptoms of intestinal obstruction which was resolved surgically. Intestinal atresia, and confirming the split notochord syndrome. The newborn died of sepsis. No autopsy was performed. The ultrasound findings depend on the form of the lesion, but often are abdomino-thoracic cysts, myelomeningocele and spina bifida. The prognosis is usually ominous, but depends on the extent of the lesions and associated anomalies

Quiste mesentérico en el recién nacido / Mesenteric cyst present in newborn

El quiste mesentérico es una enfermedad rara en la etapa neonatal, y suele confundirse imagenológicamente con múltiples patologías de los órganos intraabdominales. Se presenta un caso clínico con diagnóstico prenatal por imagen anecoica del hemiabdomen superior. El paciente requirió múltiples intervenciones quirúrgicas por presentar un quiste mesentérico que coexistía con atresia intestinal ileal en forma de cáscara de manzana, y el tratamiento clínico y nutricional fue difícil.

Mesenteric cyst is a rare condition in neonatal stage and often is confounded with multiple pathologies of intra-abdominal organs. A clinical case diagnosed with prenatal diagnosis by anechoic image of superior hemi-abdomen. Patient required of many surgical interventions due to a mesenteric cyst coexisting with an apple shell ileal intestinal atresia. Clinical and nutritional treatment was difficult.

Quiste mesentérico en el recién nacido / Mesenteric cyst present in newborn

El quiste mesentérico es una enfermedad rara en la etapa neonatal, y suele confundirse imagenológicamente con múltiples patologías de los órganos intraabdominales. Se presenta un caso clínico con diagnóstico prenatal por imagen anecoica del hemiabdomen superior. El paciente requirió múltiples intervenciones quirúrgicas por presentar un quiste mesentérico que coexistía con atresia intestinal ileal en forma de cáscara de manzana, y el tratamiento clínico y nutricional fue difícil(AU)

Mesenteric cyst is a rare condition in neonatal stage and often is confounded with multiple pathologies of intra-abdominal organs. A clinical case diagnosed with prenatal diagnosis by anechoic image of superior hemi-abdomen. Patient required of many surgical interventions due to a mesenteric cyst coexisting with an apple shell ileal intestinal atresia. Clinical and nutritional treatment was difficult(AU)

Atresia duodenal associada a pâncreas anular: relato de caso / Duodenal atresia combined with annular pancreas: a case report

relatar o caso de um recém-nascido com síndrome de obstrução intestinal alta e múltiplas anomalias congênitas associadas. Relato do caso: neonato, pré-termo, apresentou quadro de vômitos biliosos e distensão abdominal progressiva, evoluindo com aspiração de conteúdo intestinal.Radiografia simples de abdome revelou o sinal da "dupla bolha". Foi submetido à laparotomia exploradora que identificou atresia duodenal e pâncreas anular associados à má-rotação intestinal. Realizou-se duodenojejunostomia com melhora do quadro clínico do paciente e evolução assintomática. Considerações Finais: a atresia duodenal e o pâncreas anular são importantes causas de obstrução intestinal neonatal e a combinação de ambas é rara.

to report the case of a newborn with intestinal obstruction syndrome and multiple congenital anomalies associated. Case Report: neonate, preterm, presented bilious vomiting and progressive abdominal distension,which evolved to aspiration of intestinal contens. Abdominal plain film showed the "double bubble" sign. The patient underwent an exploratory laparotomy that revealed duodenal atresia and annular pancreas associated with intestinal malrotation. Duodenojejunostomy was performed, with clinical condition improvement, whichevolved without symptoms. Final considerations: the annular pancreas and duodenal atresia are important causes of neonatal intestinal obstruction and the combination of both is rare.

Comorbilidad y letalidad en el primer año de vida en niños con atresia yeyunoileal / Co-morbidity and mortality during the first year of life in children with jejunoileal atresia

Objective. To identify the co-morbidity and mortality during the first year of life in children with jejunoileal atresia. Design. Descriptive, comparative cohort. Site. Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social. Population. Seventy children with jejunoileal atresia. Measures. Following variables were registered: gestational age, birth weight, sex, type and site of atresia, other congenital defects, surgical management, number of surgeries, duration of fasting, re-hospitalizations, weight and height at 6 months and one year of life, co-morbidity and mortality. Results. Intestinal atresia types I and II were present in 27% of the patients respectively and type IIIa in 24%. Co-morbidity in the neonatal period was 64.2%, being the most common conditions sepsis (47.1%) and anastomotic stricture (18.5%). During the first year of life co-morbidity was 51.4%, presented as functional intestinal obstruction (21.4%). Mortality was 11.4% (n = 8), the main causes of death were sepsis (n = 6) and liver failure (n = 2). The mortality rate according to the type of atresia was 44.4% for type IV, 33.3% for type IIIb and 1.3% for type I. Conclusions. Co-morbidity during the first year of life in children with jejunoileal atresia is high, and related to infections and anastomosis inherent problems. Mortality is higher for intestinal atresia type IV.

Objetivo. Identificar la comorbilidad y la letalidad durante el primer año de vida en niños con atresia yeyunoileal. Diseño. Cohorte descriptiva, comparativa. Lugar. Hospital de Pediatría, Centro Médico Nacional Siglo XXI, IMSS. Pacientes. Se estudiaron 70 niños. Mediciones. Se registraron las siguientes variables: edad gestacional, peso al nacimiento, sexo, tipo de atresia, sitio de la atresia, malformaciones asociadas, tratamiento quirúrgico, reintervenciones quirúrgicas, tiempo de ayuno, rehospitalizaciones, peso y talla a los seis meses y al año de edad, comorbilidad y letalidad. Resultados. La atresia tipo I y II se presentaron en 27% cada una y la tipo IIIa en 24%. La comorbilidad en el periodo neonatal fue de 64.2%, las causas más frecuentes fueron sepsis (47.1%) y estenosis de la anastomosis (18.5%) y en el primer año de vida fue de 51.4%; la causa más frecuente fue oclusión intestinal funcional (21.4%). La letalidad fue de 11.4% (n = 8); las principales causas de muerte fueron sepsis (n = 6), e insuficiencia hepática (n = 2). La letalidad por tipo de atresia fue de 44.4% en la tipo IV, 33.3% en la IIIb y 10.5% en la tipo I. Conclusiones. La comorbilidad en el primer año de vida en niños con atresia intestinal fue elevada, las principales causas fueron infecciosas y problemas inherentes a la anastomosis. La atresia con mayor letalidad fue la tipo IV.

Atresias intestinales: resultados de cinco años de trabajo (1999-2003) / Intestinal atresias: results of a five-year work (1999-2003)

Con el objetivo de contribuir al mejor conocimiento de la epidemiología, diagnóstico y evolución de las atresias intestinales en nuestro medio, se realizó un estudio retrospectivo de 11 pacientes operados de atresia intestinal en el Hospital Pediátrico Provincial Docente "Pepe Portilla" de Pinar del Río entre el 1 de Enero de 1999 y el 31 de Diciembre del 2003. En este estudio pudimos observar que el 63.6 % de los casos tuvieron un peso al nacer mayor de 2500 gramos, y el diagnóstico se realizó en las primeras 24 horas de vida en el 54.5 % de los casos. Hubo predominio del sexo masculino y los vómitos estuvieron presentes en todos los pacientes de nuestro estudio. La atresia intestinal estuvo localizada en el yeyuno íleon en el 63.6 % de los casos, siendo las complicaciones sépticas frecuentes. Hubo una supervivencia de 90.9 %.

In order to contribuye to a better understanding of the epidemiology diagnosis and natural history of intestinal atresias in our environment, a retrospective study was carried out with 11 patients operated on due to intestinal atresia at "Pepe Portilla" Provincial Teaching Pediatric Hospital in Pinar del Río, from January 1st. 1999 to December 31st. 2003. In such study we could obsrve that 63,6% of the cases had a birth weight greater than 2500 gr. And the diagnosis was performed during the first 24 hours alter birth in 54.5% of the cases. Masculine sex was predominant, and vomiting was present in all patients of our study. The intestinal atresia was located in jejunum ileum in 63,6% of the cases, and septic complicatins were frequent. There was a 90,9% survival.