A difficult emergency surgical diagnosis: atresia of the common pulmonary vein.

A newborn female experienced severe respiratory distress immediately after delivery. She presented with intense cyanosis, refractory hypoxemia, and acidosis. The deterioration was rapidly progressive, leading to the child's demise. The autopsy showed common pulmonary vein atresia, the most severe form of pulmonary venous drainage obstruction. Subpleural and interstitial pulmonary lymphagiectasias also were present as well as evidence of pulmonary hypertension. The lung pathology probably resulted from pulmonary venous atresia during prenatal development.

Hemorragia digestiva masiva por fistulización al esófago de una colateral aortopulmonar / Massive digestive system haemorrhage due to the formation of oesophageal fistulas of the aortopulmonary collateral arter

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Diabetes neonatal transitoria y cardiopatía congénita tipo seudo-Fallot: a propósito de un caso / Transient neonatal diabetes and congenital cardiopathy pseudo-Fallot type: report of a case

Exponemos el caso de un recién nacido de 36 semanas de gestación y un peso al nacimiento de 1.680 g, que presenta a las10 h de vida hiperglucemias de 500 mg, por lo que precisa tratamiento con insulina regular en perfusión continua. En su evolución se observa una cardiopatía, seudo-Fallot, consistente en una comunicación interauricular tipo ostium secundum, una comunicación interventricular perimembranosa amplia y una estenosis pulmonar severa, que tolera bien y se trata confurosemida y aporte oral de potasio. A los 30 días de vida se administra insulina en forma de bolos cada 6 h y en la actualidad recibe insulina NPH cada 12 h, con buenos controles glucémicos .Los valores plasmáticos de insulina y péptido C son bajos y la hemoglobina glucosilada a los 2 meses de vida es normal(AU)

We present a newborn male of 36 weeks of gestation and a weight at birth of 1680 g. with an onset of hyperglycemia of500 mg at ten hours of life, requiring treatment with regular insulin in continued perfusion. In his development a Pseudo-Fallot cardiopathy is observed, consisting of ostium secundum type ASD (atrial septal defect), wide perimembranous ASD, and severe pulmonary stenosis, that he tolerates well and that is being treated with furosemide and oral contribution of K. At30 days of age, he receives bolus insulin every 6 hours and at present, NPH insulin every 12 hours, with good glycemics controls. The plasmatic values of insulin and C-peptide are low and the glycosylated hemoglobin at two months of life is normal(AU)

[Congenital anatomic gastrointestinal obstruction: prenatal diagnosis, morbidity and mortality]. / Obstrucción gastrointestinal anatómica congénita: diagnóstico prenatal, morbilidad y mortalidad.

OBJECTIVES: To describe the morbidity and mortality associated with congenital gastrointestinal obstruction, assess the usefulness of prenatal diagnosis, and analyze the results in relation to the site of obstruction. PATIENTS AND METHODS: We performed a retrospective case series study of 148 patients in a 13-year period (1990-2003). Sites of obstruction was classified into three groups: I) gastric, duodenal or jejunal; II) ileal or colonic; III) anorectal. RESULTS: There were 65 patients in group I, 38 in group II and 45 in group III. Surgery was performed in 137 patients. Trisomy was diagnosed in 15 patients, polymalformation syndrome in 16 patients, and cystic fibrosis in seven patients. More than one site of intestinal obstruction was found in 22 patients (15 %). Morbidity consisted of nosocomial sepsis in 32 %, necrotizing enterocolitis in 7 %, and short gut in 3.4 %. Prematurity (48 %) was associated with higher morbidity and mortality. Overall mortality was 14.2 %, mainly associated with other malformations and extraintestinal problems. When patients with other extraintestinal malformations were excluded, the highest mortality was found in group II (OR: 12.19; CI: 2.4-76.2). The overall sensitivity of prenatal diagnosis was 0.44 and the mean gestational age at diagnosis was 31.6 weeks (SD 5.2). According to the site of obstruction, sensitivity was 0.77 in group I, 0.39 in group II and 0.04 in group III. Prenatal diagnosis did not alter prognosis. CONCLUSIONS: Morbidity and mortality were strongly influenced by extraintestinal problems. The most severe intestinal complications were midgut volvulus and necrotizing enterocolitis. Prenatal diagnosis was late and showed good sensitivity only in group I.

Obstrucción gastrointestinal anatómica congénita: diagnóstico prenatal, morbilidad y mortalidad / Congenital anatomic gastrointestinal obstruction: prenatal diagnosis, morbidity and mortality

Objetivos Describir la morbimortalidad de la obstrucción gastrointestinal congénita, valorar la utilidad del diagnóstico prenatal y analizar los resultados según el nivel de obstrucción. Pacientes y métodos Estudio descriptivo retrospectivo de 148 pacientes en el período 1990-2003. Los niveles de obstrucción se clasificaron en tres grupos: I) gástrica, duodenal o yeyunal; II) ileocólica; III) anorrectal. Resultados La distribución por grupos fue: 65 en el grupo I, 38 en el grupo II y 45 en el grupo III. Se intervinieron 137 pacientes. Se diagnosticaron 15 trisomías, 16 síndromes polimalformativos y siete fibrosis quísticas. En 22 casos (15 %) se encontró más de una zona de obstrucción intestinal. La morbilidad fue la siguiente: sepsis nosocomial 32 %, enterocolitis necrosante 7 %, intestino corto 3,4 %. La prematuridad (48 %) se asoció con mayor morbimortalidad. La mortalidad global fue de 14,2 %, asociada fundamentalmente a otras malformaciones y a problemas extraintestinales. Excluyendo los pacientes con otras malformaciones, el grupo II presentó mayor mortalidad (OR: 12,19; IC: 2,4-76,2). La sensibilidad global del diagnóstico prenatal fue 0,44 y la edad gestacional media del diagnóstico 31,6 semanas (DE: 5,2). Según el nivel de la obstrucción, la sensibilidad fue 0,77 en el grupo I; 0,39 en el grupo II y 0,04 en el grupo III. El diagnóstico prenatal no modificó el pronóstico. Conclusiones La morbimortalidad estuvo muy condicionada por problemas extraintestinales. El vólvulo de intestino medio y la enterocolitis necrosante fueron las complicaciones intestinales más graves. El diagnóstico prenatal fue tardío y sólo tuvo buena sensibilidad en el grupo I

Objectives To describe the morbidity and mortality associated with congenital gastrointestinal obstruction, assess the usefulness of prenatal diagnosis, and analyze the results in relation to the site of obstruction. Patients and methods We performed a retrospective case series study of 148 patients in a 13-year period (1990-2003). Sites of obstruction was classified into three groups: I) gastric, duodenal or jejunal; II) ileal or colonic; III) anorectal. Results There were 65 patients in group I, 38 in group II and 45 in group III. Surgery was performed in 137 patients. Trisomy was diagnosed in 15 patients, polymalformation syndrome in 16 patients, and cystic fibrosis in seven patients. More than one site of intestinal obstruction was found in 22 patients (15 %). Morbidity consisted of nosocomial sepsis in 32 %, necrotizing enterocolitis in 7 %, and short gut in 3.4 %.Prematurity (48 %) was associated with higher morbidity and mortality. Overall mortality was 14.2 %, mainly associated with other malformations and extraintestinal problems. When patients with other extraintestinal malformations were excluded, the highest mortality was found in group II (OR: 12.19; CI: 2.4-76.2). The overall sensitivity of prenatal diagnosis was 0.44 and the mean gestational age at diagnosis was 31.6 weeks (SD 5.2). According to the site of obstruction, sensitivity was 0.77 in group I, 0.39 in group II and 0.04 in group III. Prenatal diagnosis did not alter prognosis. Conclusions Morbidity and mortality were strongly influenced by extraintestinal problems. The most severe intestinal complications were midgut volvulus and necrotizing enterocolitis. Prenatal diagnosis was late and showed good sensitivity only in group I