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Introduction: Holt-Oram syndrome is a low frequency hereditary disease, with wide clinical heterogeneity. Characterized by skeletal defect in the upper limbs of variable and asymmetric degree, associated with congenital heart disease. Case presentation: a four-year-old boy was presented, a male with a clinical diagnosis of Holt-Oram Syndrome, who presents shortening of the left upper limb, congenital heart disease and esophageal atresia, the first affected in his family, so it was considered that it occurred by new mutation. Conclusions: due to the unusual occurrence of this syndrome associated with digestive malformation, it was difficult to reach the clinical diagnosis, where the clinical or pattern method was of great value to define the case. It was necessary to consult the case with other researchers, without being able to carry out a molecular study. Considering it is important to reach the clinical diagnosis of the genetic disease to provide adequate genetic counseling to this family. [ABSTRACT FROM AUTHOR]

The association of VACTERL (it is an acronym, V= vertebral anomalies, A= anal atresia, C= cardiac defects, T= tracheal anomalies, E= esophageal atresia, R= renal anomaly, L= other limb anomalies) is rare, however, it is a pathology with a high associated Morbi-mortality. This is a 30-year-old male patient with VACTERL's association, who has exceeded his life expectancy, based on his diagnosis. However, he has not escaped unscathed, since he is suffering from stage 4 chronic kidney disease and is under treatment for arterial hypertension and serial JJ catheter replacement. [ABSTRACT FROM AUTHOR]

Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista de Gastroenterología del Perú is the property of Sociedad de Gastroenterologia del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Esophageal atresia is an infrequent congenital malformation incompatible with life and a neonatal surgical urgency. The most common presentation is esophageal atresia with distal tracheoesophageal fistula (80-90 % of cases). It presents in one of every 3000 births. For these reasons, the case of a preterm infant, 48 hours of birth, 1245 grams weight, APGAR 8/9, with diagnosis of esophageal atresia and tracheo-esophageal fistula type IIIb / C is presented. She was diagnosed at birth, due to the presence of projectile vomiting, respiratory distress and the impossibility of introducing a naso-gastric tube and confirmed when seen on a chest x-ray. It was announced for surgery in which fistula repair was performed. We present the anesthetic considerations to be followed in the management of this case, related to: anesthetic drugs, volume replacement and mechanical ventilation strategy. Favorable results were obtained. [ABSTRACT FROM AUTHOR]

Copyright of Revista Chilena de Pediatría is the property of Revista Chilena de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Iatreia is the property of Universidad de Antioquia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Esophageal atresia is a relatively common congenital malformation of unknown etiology consisting of a lack of continuity of the esophagus with or without communication to the airway. The condition occurs in 1/2500--4500 live births. If the patient does not receive surgical treatment, this defect may lead to death. Abnormalities associated with esophageal atresia are present in ~50% to 60% of patients and include urinary tract and digestive, cardiac and musculoskeletal systems. Esophageal atresia is perhaps one of the classic pediatric surgical diseases and important developments have been reported in recent years regarding survival. This achievement is related to the specialized care provided in neonatal intensive care units (NICU) and the use of parenteral nutrition as well as improvement of anesthetic and surgical techniques. Currently, mortality in esophageal atresia patients is an indicator of the quality of care in NICUs. Postoperatively, overall prognosis is good because mortality is low in the absence of other associated malformations. This article synthesizes the general aspects that should be offered to all newborns suspected of having esophageal atresia, indicating the importance of a multidisciplinary approach to achieve the best results in this group of patients. [ABSTRACT FROM AUTHOR]

Copyright of Revista Chilena de Pediatría is the property of Revista Chilena de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Chilena de Pediatría is the property of Revista Chilena de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction. Spontaneous prenatal closure of a gastroschisis is rare; it is usually associated with atresia of the midgut. This anomaly is known as "closed gastrochisis". In Spanish we suggest the term antenatal paraumbilico-intestinal strangulation (APIS) or Grosfeld's syndrome. Material and method. A two-year review of 58 gastroschisis patients, identified four infants with this uncommon complication. Results. In these infants the extracorporeal midgut was invariably necrotic or fibrotic. The remnant midgut was surgically removed; during the procedure we observed jejunal atresia of 35 to 40 cm and atresia of the left colon. All patients developed short bowel syndrome and three of them died at less than four weeks of age. Conclusions. This series shows that this complication is potentially lethal. [ABSTRACT FROM AUTHOR]

Copyright of Revista Médica Clínica Las Condes is the property of Editorial Sanchez y Barcelo and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: The objective of the present work, is to inform the experience with two novel techniques to replace the esophagus when the separation of the esophagus ends to replace is not very long. Material and methods: From 1988 to 2007 five patients operated, two with the technique of Schärli and three with technique of Collis-Nissen. Four of them at institutional level and one private. Results: Of both patient patients with the technique of Schärli, one was by estenosis by caustic with four previous substitutions (three of colon and one of yeyuno), with 9 years of evolution with growth and acceptable development; the other patient, with atresia of esophagus type III and association VACTER, passed away in early form, by cause nonattributable to the operation. The three patients operated with Collis-Nissen had atresia of esophagus type I, with good evolution and pursuit of four, one year and the last one with two months. Conclusions: With both techniques good result has been obtained, with minimum postoperating complications. [ABSTRACT FROM AUTHOR]

Introduction: The objective of the work is to describe to the after repair complications of the patients with esophageal atresia type III. Material and methods: The patients with esophageal atresia type III a period of 6 years. The data were obtained: Sex, age, weight, cardiac anomaly. The complications were classified in early and delayed. We used the scale of Spitz. Results: We identified 45 patients with esophageal atresia type III: 25 female and 20 male, with age 2,2 average of days, weight average of 2012 g. 16 patients with cardiac malformation. The groups of Spitz: 29 patients for group I (64,5%), 16 patients for group II (35,5%). Group I the complications: early 11 and 25 delayed. Group II early complications 18 and delayed 9. Conclusions: The complications delayed appeared in group I most frequents and in group II the early complications were most frequents. [ABSTRACT FROM AUTHOR]

From 1972 to 2007 35 patients with esophageal atresia without fistulae were operated for substitution of the esophagus. Nineteen were children under six months of age (Group A), six (21%) of which died. The remaining 16 were over six months of age at operation (Group B), two of which (12.5%) died. Overall fatalities numbered five (17.1%). There were no deaths during surgery. In 31 patients the colon was used for substitution and the stomach in four. Surgical procedures were divided into five groups from 1 to 5. There were no deaths in Groups 1, 4 and 5 (nine patients). There were four deaths out of 21 patients (19%) in Group 2 and two deaths out of five patients (40%) in Group 3. There were no fatalities in 14 patients over three months of age operated in the last ten years; these were patients in anabolism, without heart disease. Three surgical procedures were used either with colon or with stomach replacement. They sustained minimal complications. Conclusion: Esophageal substitution can be performed in neonates but it is best to operate after three months of age provided the right surgical procedure and organ for replacement are chosen. [ABSTRACT FROM AUTHOR]

Introduction. The most used prognostic classifications in esophageal atresia are Waterston and Montreal. The purpose of this study was to search for prognostic factors for surgical complications such as dehiscence, refistulization, stenosis and mortality in our population. Methods. Retrospective case-control study on a series of type III esophageal atresia operated in our center over 10 years with a follow-up of at least 2 years. Statistically tests were chi square, Student's t test, and odds ratio. Results. Prognostic factors for mortality were gestational age, acidosis or pneumonia at admission, Waterston C and Montreal II classifications, and days of mechanical ventilation. Complications associated with ventilation were risk factors for dehiscence. This latter is in turn a risk factor for refistulization, and along with gastroesophageal reflux both are risk factors for esophageal stenosis. Conclusions. There are some specific factors in our population that enhance the risk of morbidity and mortality. [ABSTRACT FROM AUTHOR]

Introduction: The Atresia Esophagus is an alteration congenital and surgical treatment requires skill and experience, but are indispensable neonatal intensive care, the use of mechanical ventilation immediate postoperative and at times, the use of the food endovenosa total. Objective: Study of patients diagnosed with main Atresia Esophagus by accenting the form of treatment, results, surgical complications and mortality. Material and Methods: It was a retrospective study, observational and descriptive of the dossiers clinical patients diagnosed with main Esophageal Atresia in a period that includes January 1980 to September of 2005, taking a total of 80 cases of which 60 met the criteria for analysis. Results: The frequency was greater in the male, the 56.89 percent of children joined within the first 24 hours; its diagnosis with would clinical and radiographic, being more of the type III; the treatment by stages took place in the 40 percent and one time in 15 per cent. Discussion: Discussed the main parameters of prognosis and the vital role to play the congenital heart disease, as well as the main complications post-surgical and analysis of mortality. [ABSTRACT FROM AUTHOR]

Introduction: Intestinal Atresia is the main cause of obstruction in the digestive tract in new-born. Prenatal ultrasonography makes possible an early diagnosis and treatment with better survival. Objective: Study of new-born with diagnosis of AI, reviewing the intestinal lengthening procedures. Material and methods: A retrospective analysis of the pediatrics charts with principal diagnosis of IA in a period of 27 years. The variables included: age, sex, family background, clinical signs, diagnosis, treatment, and mortality. Results: Mean age was 4 days, without sex predominance; all patients had showed vomit presence of abdominal distention and they did not pass stools. The diagnosis was integrated with plain "X" rays of abdomen and in some cases we used medium contrast; with surgical treatment was possible the reconstruction of the intestinal tract. The mortality found 30.15% . Discussion: We enhanced the utility to make an early diagnosis, with the proper correlation of the clinical and radiological signs (95%). It reviewed some prenatal aspects that permits the surgical procedure in the first 24 hs of new-born. It is also analyzed, some techniques of intestinal anastomosis, the diamond type and its lengthening. Conclusions: IA of ileon was the most frequent type. Duodenal Atresia had associated the most serious malformations. Esophageal atresia was associated in the 3.17%. Prenatal control with ultrasonography improves the prognosis. [ABSTRACT FROM AUTHOR]

This paper reports the case of a male term-newborn, without problems during gestation process who died at birth and who was detected in autopsy with: upper third esophageal atresia, tracheo-esophageal fistula, tracheal atresia and arterious duct persistence. Esophageal atresia with tracheoesophageal fistula is a disorder affecting 1 per 5,000 births. This disease associated to tracheal atresia is even less common. Bibliography does not report more than 100 cases of these. Its early detection may increase life probabilities. [ABSTRACT FROM AUTHOR]