Membrana duodenal: a propósito de un caso / Duodenal membrane: presentation of a case / Membrana duodenal: sobre o assunto de um caso

La atresia de duodeno es una causa poco frecuente de oclusión intestinal. Generalmente el diagnóstico se realiza en etapa prenatal o en el período neonatal principalmente para aquellos casos de oclusión completa, mientras que aquellos con oclusión incompleta pueden diagnosticarse más tardíamente. Se presenta el caso clínico de una paciente con una forma de presentación infrecuente, con diagnóstico luego del año de vida. Se realizó una duodeno-duodeno anastomosis, con buena evolución postoperatoria.

Duodenal atresia is a rare cause of intestinal occlusion. Generally, the diagnosis is made in the prenatal stage or in the neonatal period mainly for those cases of complete occlusion, whereas those with incomplete occlusion may be diagnosed later. We present the case of a patient with an uncommon presentation, with diagnosis after one year of life. A duodenum-duodenum anastomosis was performed, with good postoperative evolution.

A atresia duodenal é uma causa rara de oclusão intestinal. O diagnóstico geralmente é feito no estágio pré-natal ou no período neonatal principalmente para os casos de oclusão completa, enquanto aqueles com oclusão incompleta podem ser diagnosticados mais tarde. Apresentamos o caso de uma paciente com uma apresentação incomum, com diagnóstico após o ano de vida. Uma anastomose duodeno-duodeno foi realizada, com boa evolução pós-operatória.

Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association.

Tracheal agenesis (TA) is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel's diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.

Signo de la doble burbuja: marcador ecográfico asociado a aneuploidías / Double bubble sign. sonographic marker associated with aneuploidy

Introducción: Las anomalías cromosómicas ocurren en 0,1 a 0,2% de los recién nacidos (RN). En el ultrasonido se pueden observar marcadores ecográficos sugestivos de aneuploidia. El signo de la doble burbuja representa una anomalía estructural de la porción proximal del duodeno, generalmente debido atresia duodenal, que se asocia en un 30 – 40% a trisomía 21.Objetivos: Presentación de caso clínico y revisión de marcadores ecográficos que permiten detectar defectos estructurales que representen anomalías cromosómicas. Presentación de caso: Paciente de 38 años cursando embarazo de 32,3 semanas. Biometría fetal informó retardo de crecimiento intrauterino leve, con diámetro biparietal y circunferencia cefálica de 31 semanas, circunferencia abdominal y longitud femoral de 28 semanas. Scan fetal morfológico detallado visualiza cámara gástrica presente con dilatación de duodeno superior. Signo de la doble burbuja. Discusión: El signo de la doble burbuja suele reconocerse luego de la semana 24 de gestación. La detección lleva a la sospecha de un número de anomalías estructurales, las cuales causan obstrucción duodenal. La atresia duodenal es el tipo más común de atresia fetal del intestino delgado.

Introduction: Chromosomal abnormalities occur in 0.1 to 0.2% of newborns. By using ultrasound we can observe suggestive sonographic markers of aneuploidy. The double bubble sign represents a structural abnormality of the proximal duodenum, usually due to duodenal atresia, associated in a 30 to 40% with trisomy 21. Objectives: Presentation of a case and review of sonographic markers to detect structural defects that represent chromosomal abnormalities. Case presentation: 38 year old female patient, with a 32.3 weeks pregnancy. Fetal biometrics reported mild intrauterine growth retardation biparietal diameter and head circumference of 31 weeks, abdominal circumference and femur length of 28 weeks. Morphological fetal Scan shows gastric chamber with dilatation of the upper duodenum. Double bubble sign.Discussion: The double bubble sign is usually recognized after 24th week of pregnancy. Detecting this sign leads to suspicion of a number of structural abnormalities, which cause duodenal obstruction. Duodenal atresia is the most common type of fetal small bowel atresia.

Situs Inversus Abdominis Associated with Duodenal Atresia: A Case Report

Situs inversus abdominis is a rare congenital condition commonly associated with serious cardiac and splenic malformations. The importance of recognizing the presence of situs inversus abdominis preoperatively is emphasized by the fact that the surgical incision is placed on the incorrect side of the abdomen. A 6 day-old girl was referred to our hospital because of bile stained vomiting. A plain radiography of abdomen and chest showed the heart to be normal position and a reversed "double-bubble" picture with no other gas shadow in the rest of the abdomen. Abdominal computed tomography scan revealed situs inversus with the stomach and polysplenia on the right side and the liver on the left side. A laparotomy confirmed the diagnosis of situs inversus with duodenal atresia. The obstruction was bypassed by constructing a side-to-side duodenoduodenostomy. The postoperative course was uneventful.

Clinical Experience with Esophageal Atresia Combined with Duodenal Atresia

There is significant morbidity and mortality associated with the combination of esophageal atresia (EA) and duodenal atresia (DA). Nevertheless, the management protocol for the combined anomalies is not well defined. The aim of this study is to review our experience with the combined anomalies of EA and DA. From May 1989 to August 2006, seven neonates were diagnosed as EA with DA at Asan Medical Center. In all cases, the type of EA was proximal EA and distal tracheoesophageal fistula (TEF). The diagnosis of DA was made in theprenatal period in 1, at birth in 4, 4 days after birth in 1 (2 days after EA repair) and at postmortem autopsy in 1. Except the one case where DA was missed initially, primary simultaneous repair was attempted. DA repair with gastrostomy followed by EA repair in 2, EA repair followed by DA repair without gastrostomy in 2, and TEF ligation followed by DA repair with gastrostomy in 1. There were two deaths. One baby had a large posterolateral diaphragmatic hernia, and operative repair was not attempted. The other infant who had a TEF ligation and DA repair with gastrostomy expired from cardiac failure due to a large patent ductus arteriosus. Simultaneous repair of EA and DA appears to be an acceptable management approach for the combined anomalies, but more experience would be required for the selection of the primary repair of both anomalies.

Primer caso de corrección no quirúrgica de una atresia duodenal tipo membrana fenestrada en un recién nacido mediante sección y ampliación por endoscopia / First case of non surgical correction of fenestrated duodenal membrane in a newborn by endoscopic technique

Introducción. La obstrucción duodenal en el recién nacido es una patología frecuente; dentro de las causas más comunes se encuentran las membranas, algunas de ellas pueden estar fenestradas. La corrección de éstas tradicionalmente es con cirugía abierta, algunos casos por laparoscopia y de manera anecdótica resecciones completas con ondas de alta frecuencia o láser por vía endoscópica. Nos preguntamos si era posible la sección y ampliación de las membranas duodenales incompletas con un esfinterotomo de vías biliares por endoscopia como único tratamiento. Caso clínico. Recién nacido con atresia de esófago tipo III y malformación ano-rectal a quien realizaron plastia del esófago y colostomía; tardíamente se efectuó el diagnóstico de atresia duodenal tipo membrana fenestrada, la que se corrigió únicamente mediante un corte de ésta con un esfinterotomo por endoscopia. La evolución fue satisfactoria. Conclusión. Se presenta una nueva alternativa que puede ser usada en casos seleccionados de membrana duodenal fenestrada, que resuelve el problema y evita una cirugía abierta.

Introduction. Duodenal obstruction in the newborn is a frequent pathology; among the most common causes of obstruction are duodenal membranes and in some cases these membranes are fenestrated. Traditionally, correction of membranous duodenal obstruction is with open surgery although laparoscopic resection has been reported and, occasionally, complete resection has also been performed with laser endoscopic technique. Using a biliary tract sphincterotome we evaluated the resection and widening of a duodenal obstruction in a newborn infant. Case report. Newborn with type III esophageal atresia and anorectal malformation who underwent esophageal plasty and colostomy. Belatedly the diagnosis of duodenal atresia due to a fenestrated membrane was made and corrected endoscopically with a biliary tract sphincterotome. The child had a satisfactory recovery. Conclusion. A new non-invasive surgical alternative is presented that can be used in selected cases of fenestrated membranous duodenal obstruction that avoids an open surgery.

A Case of Congenital Duodenal Atresia Diagnosed by Prenatal Ultrasonography / 대한주산의학회잡지

Congenital duodenal atresia is the most common cause of perinatal intestinal obstruction. Abdominal distension and projectile vomiting are the specific symptoms of neonatal gastro-intestinal obstruction. The incidence of duodenal atresia is between 1 in 2,710 and 1 in 10,000 live births.1 Most cases are thought to be failure of recanalization of the duodenal lumen during embryonic period.2 Duodenal atresia is associated with a high incidence of other associational anomalies, including esophageal atresia, biliary atresia, congenital heart disease and vertebral anomalies. Prenatal diagnosis, the ealry operation and the facotrs such as prematurity, combined abnormalities, nutirtion have a significant influence on complication and mortality.3,4 We report a case of congenital duodenal atresia diagnosed by ultrasonography prenatally in Andong general hostpital at 26 gestational weeks and operated the 7th day of cesarean section in Yeungnam university hospital with a brief review of literature.

A Case of Tracheal Bronchus / 소아알레르기및호흡기학회지

Tracheal bronchus is an aberrant bronchus that arises most often from right tracheal wall above the carina. It is a rare congenital anomaly, which is usually asymptomatic but occasionally associated with recurrent pneumonia, chronic bronchitis and bronchiectasis. Anomalies found in association with tracheal bronchi include respiratory(tracheal hypoplasia, tracheal stenosis, cystic lung lesion), gastrointestinal and musculoskeletal systems. Tracheal bronchus has been diagnosed by conventional tomography, bronchography and bronchoscopy in the past. Technical advances have greatly enhanced the utility of this diagnostic modality. So chest CT, even three-dimensional reconstruction, is of particular importance in the evaluation of mediastinal, pleural and lung parenchymal lesions. We experienced a case of duodenal atresia and tracheal bronchus in 6-month-old child who suffered from recurrent pneumonia and dyspnea. So we report with a brief review and its related literatures.

A Clinical Study of Congenital Intestinal Atresia and Stenosis

Seventy neonates with congenital intestinal atresia and stenosis who were treated at pediatric surgical service, Hanyang University Hospital from September 1979 to December 1996 were analyzed retrospectively. The lesion occurred in 27 cases at the duodenum, in 26 cases at the jejunum, in 13 cases at the ileum, and in 2 cases at the pylorus and colon each. The atresia predominated over the stenosis by the ratio of 4 : 1. Male to female ratio was 1.3 : 1. The average gestational age was 38 weeks, and the average birth weight was 2,754 grams. Though 22.9% were borne prematurely and 34.3% had low birth weight, 92.3% of them had a weight appropriate for gestational age. The clinical manifestations were bile-stained vomiting (70%), abdominal distention (35.7%), jaundice (37.1%), and delayed passage of meconium (51.4%). Polyhydramnios (40%) was more frequently observed in duodenal and jejunal atresia while microcolon in ileal atresia (58.3%). Weight loss and electrolyte imbalance occurred more frequently in the duodenal stenosis cases because of delayed diagnosis. Twenty (55.6%) of 37 jejunoileal atresia cases had evidence of intrauterine vascular accident : 4 intrauterine intussusception, 3 intrauterine volvulus and 3 strangulated intestine in gastroschisis, and 10 cases of intrauterine peritonitis. There were one or more associated anomalies in 45 patients (64.3%). Preoperatively proximal loop volvulus developed in 3 cases and proximal loop perforation in 5 cases and one case each of distal loop perforation, duodenal perforation and midgut volvulus occurred in the jejunoileal atresia. There were 10 multiple atresia cases and 14 short bowel cases. Overall mortality rate was 20%.