Duodenal atresia and stenosis: Reassessment of treatment and outcome based on antenatal diagnosis,pathologic variance,and long-term follow-up

Duodenal atresia and stenosis was observed in 103 infants and children from 1972 to 1991. There were 59 girls and 44 boys. Atresia was noted in 79 instances and stenosis in 24. Maternal hydramnios was detected in 33 cases, 46 babies were premature, and 31 had Down's syndrome. Fifty-four infants had significant associated anomalies including 35 with cardiac defects. Diagnosis was achieved by prenatal ultrasound examination in 14 cases, observation of a double-bubble sign on abdominal radiograph in 73, and contrast studies in 30 infants including 24 with stenosis. At operation annular pancreas was noted in 37 cases, malrotation in 37 cases, anterior portal vein in 4, and a second web in 3. Surgical treatment included duodenoduodenostomy in 85, duodenotomy and web excision in 8, and duodenojejunostomy in 10. Operative survival was 95%. Deaths were related to complex cardiac defects. Despite antenatal diagnosis, prompt intervention, and apparent early surgical success (95% survival), late deaths (5%) and late complications including motility disorders, megaduodenum, gastroesophageal reflux, duodenal-gastric reflux, gastritis, peptic ulcer disease, blind loop syndrome, and biliary-pancreatic conditions may be observed months to years after management during the neonatal period. Modifications in surgical technique including early tapering duodenoplasty may be useful, and close long-term follow-up is an essential component of patient care.

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Resumen Se encontró atresia duodenal y estenosis en 103 niños, 59 de sexo femenino y 44 de sexo masculino, en el período 1972–91: atresia en 79 casos y estenosis en 24. Se registró hidramnios materna en 33 casos; 46 niños fueron prematuros y 31 presentaban síndrome de Down; 54 exhibían anomalías de significación, incluyendo 35 con defectos cardiacos. El diagnóstico fue establecido por ultrasonido prenatal en 14 casos, mediante la observación de burbuja doble en la radiografía abdominal en 73 y por estudio con medio de contraste en 30, entre los cuales había 24 con estenosis. En la operación se encontró páncreas anular en 37 casos, malrotación en 37, vena porta anterior en 4 y segunda membrana en 3. El tratamiento quirúrgico incluyó duodenostomía en 85 pacientes, duodenotomía y resección de membrana en 8 y duodenoyeyunostomía en 10. La tasa de sobrevida operatoria fue de 95%. Las muertes estuvieron asociadas con los defectos cardfacos complejos. A pesar de su diagnóstico prenatal, una intervención quirúrgica precoz y un aparente éxito operatorio (sobrevida de 95%), se observan muertes tardías (5%) y complicaciones a largo plazo tales como desórdenes de la motilidad intestinal, megaduodeno, reflujo gastroesofágico, reflujo duodeno-gástrico, gastritis, enfermedad ulcerosa péptica, síndrome de asa ciega y alteraciones biliopancreáticas, meses a años después del tratamiento y manejo neonatales. Algunas modificaciones en la técnica quirúrgica, tales como duodenoplastia, pueden ser de utilidad; el seguimiento cuidadoso a largo plazo constituye un componente esencial de la atención de estos pacientes.

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Résumé Entre 1972 et 1991, on a observé 79 cas d'atrésie duodénale et 24 cas de sténose duodénale chez un total de 103 enfants, 59 filles et 44 garçons. Une hydramnios a été retrouvée chez 33 des mères, 46 enfants étaient des prématurés et 31 avait un syndrome de Down. Chez 54 enfants, il y avait des malformations associées, dont 35 cardiaques. Le diagnostic avait été établi dans 14 cas par une échographie anténatale, par l'existence du signe de la double bulle sur les abdomens sans préparation chez 73 patients et par une étude du transit intestinal en double contraste chez 34 enfants, dont 24 avec une sténose. A l'opération, on a retrouvé un pancréas annulaire chez 37 enfants, une malrotation chez 37, une veine porte antérieure chez 4, et une membrane intraluminale chez 3. Le traitement a été une anastomose duodénoduodénale chez 85 enfants, une excision de membrane après duodénotomie chez 8 et une duodénojéjunostomie chez 10. La mortalité opératoire était de 5%, en rapport essentiellement avec des malformations cardiaques complexes. En dépit d'un diagnostic anténatal, une intervention précoce et un succès chirurgical apparent, la morbidité tardive est toujours possible et comprend essentiellement des anomalies de la motilité duodénale, le mégaduodénum, un reflux gastroesophagien, un reflux duodénogastrique, une gastrite, la maladie ulcéreuse, un syndrome de l'anse borgne, et d'autres anomalies biliopancréatiques qui peuvent se voir des mois ou des années après la période néonatale. Les modifications de la technique chirurgicale et notamment la duodénoplastie précoce ainsi qu'une meilleure surveillance à distance, peuvent en améliorer le pronostic.

     

Anterior ischemic optic neuropathy: Classification of field defects by Octopus™ automated static perimetry

Visual fields of patients with anterior ischemic optic neuropathy (AION) were classified according to quantitative criteria, using the Octopus perimeter. Although a significant altitudinal pattern of field loss was found in 55% of perimetric examinations, the "spared" hemifields routinely showed some loss of sensitivity. This finding, along with the diffuse loss of sensitivity in a high percentage of visual fields, indicates more extensive involvement of the circulation of the anterior optic nerve head than has previously been suggested. Furthermore, patients with diabetes mellitus alone were found to have a statistically separable pattern of visual field loss. The pathophysiologic implications of the visual fields in AION and their relationship to the clinical findings were investigated.     

Shoulder dystocia: when to use suprapubic or fundal pressure.

"The delivery of the head with or without forceps may have been quite easy, but more commonly there has been a little difficulty in completing the extension of the head. The hairy scalp slides out with reluctance. When the forehead has appeared it is necessary to press back the perineum to deliver the face ... time passes. The child's head becomes suffused. It endeavors unsuccessfully to breathe. Abdominal efforts by the mother or by her attendants produce no advance; gentle head traction is equally unavailing. Usually equanimity forsakes the attendants. They push, they pull. Alarm increases. Eventually by greater strength of muscle or by some infernal juggle, the difficulty appears to be overcome, and the shoulders and trunk of a goodly child are delivered. The pallor of its body contrasts with the plum-colored cyanosis of the face, and the small quantity of freshly expelled meconium about the buttocks. It dawns upon the attendants that their anxiety was not ill-founded, the baby lies limp and voiceless, and too often remains so despite all efforts at resuscitation".     

“Reducing body”-like inclusions in skeletal muscle in childhood-onset acid maltase deficiency

Summary Unusual inclusions with some of the features of reducing bodies were encountered in the skeletal muscle biopsy of a 2.5-year-old boy with childhoodonset acid maltase deficiency. The biopsy revealed a vacuolar myopathy with lysosomal storage of glycogen and eosinophilic refractile inclusions in myofibers, which appeared dark blue with the menadione-nitroblue tetrazolium reaction. The significance of the association of inclusions with reducing properties in the setting of acid maltase deficiency is discussed.     

Current concepts in inguinal hernia in infants and children

Trends are changing in the management of infants and children with indirect inguinal hernias. Advances in neonatal intensive care have resulted in the survival of many small premature infants who have a high incidence of inguinal hernia. The rate of incarceration, strangulation, and gonadal infarction in these babies is twice that of the general pediatric age group. Respiratory immaturity, apnea, bradycardia, and associated neonatal conditions require special management at the time of hernia repair, usually performed just before discharge from the neonatal intensive care unit. New information concerning volume loss and depletion of germ cells beginning at 6 months of age in boys with undescended testes has stimulated the performance of orchiopexy when the patient is 1 year of age. More than 90% of boys with cryptorchid testes at the age of 1 year have an associated hernia that requires concomitant repair at the time of orchiopexy. The use of the peritoneal cavity for fluid absorptive purposes in hydrocephalus treated by venticuloperitoneal shunts or of peritoneal dialysis for renal failure and metabolic diseases such as hyperammonemia and lactic acidosis causes increased intraabdominal pressure and results in the appearance of a previously unrecognized hernia. Recognition of these and other conditions associated with a high incidence of hernial occurrence should allow early diagnosis and treatment before the development of complications. Most elective repairs of hernias are safely performed in the outpatient setting; however, some infants and children with concurrent illnesses are best managed in a morning admissions program, in which hospital admission occurs postoperatively.

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Resumen Este artículo se refiere a las tendencias en el manejo de lactantes y niños con hernias inguinales indirectas. Los avances logrados en el cuidado intensivo neonatal han resultado en la supervivencia de muchos pequeños prematuros, quienes exhiben una elevada incidencia de hernia inguinal. La tasa de incarceración, estrangulación, e infarto gonadal en estos pacientes es dos veces la de la población pediátrica general. La inmadurez respiratoria, apnea, bradicardia, y otras condiciones neonatales asociadas requieren un manejo especial con ocasión de la reparación herniaria, la cual generalmente se realiza inmediatamente antes de la salida de la unidad de cuidado intensivo neonatal. Nuevos conocimientos sobre pérdida de volumen y depleción de las células germinales, lo cual comienza a los 6 meses de edad en niños con testículos no descendidos, ha estimulado la realización de orquidopexia cuando el paciente llegue a la edad de un ano. Más del 90% de los niños con testículos criptorquídicos a la edad de un año tienen una hernia asociada que requiere reparación concomitante con la orquidopexia. El uso de la cavidad peritoneal para efectos de absorción de fluidos en el caso de hidrocéfalos tratados con la implantación de sistemas valvulares ventriculoperitoneales, o de diálisis peritoneal para falla renal y enfermedades metabólicas tales como hiperamonemia o acidosis láctica, causa un aumento en la presión intraabdominal y resulta en la aparición de una hernia previamente inaparente. La identificación de estas y otras condiciones asociadas con una elevada incidencia de hernia debe hacer posible el diagnóstico y tratamiento tempranos, antes de que se presenten complicaciones. La mayoría de las reparaciones herniarias pueden ser realizadas en forma adecuada como procedimientos ambulatorios. Sin embargo, algunos infantes y niños con enfermedades concurrentes pueden ser mejor manejados en un programa de admisión matinal, en el cual la hospitalización se produce en el postoperatorio.

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Résumé Le traitement des hernies inguinales obliques externes chez le bébé et l'enfant évolue. Les progrès accomplis dans les soins intensifs des nouveau-nés ont permis la survie d'un plus grand nombre d'enfants prématurés qui présentent une fréquence plus importante de hernies inguinales. Le taux d'engouement, d'étranglement, et d'ischémie gonadale chez cette population est deux fois celui de la population pédiatrique générale. Le développement respiratoire inachevé, l'apnée, la bradycardie, et d'autres conditions néonatales associées sont autant de facteurs qui nécessitent le travail d'une équipe spécialisée au moment du traitement de la hernie qui se situe juste avant la sortie de l'unité de soins intensifs. Certaines études ayant montré une perte de volume et une déplétion de cellules germinales à partir de l'âge de 6 mois chez le garçon avec une ectopie testiculaire, on préconise une orchidopexie tôt, à l'âge d'un an. Plus de 90% de ces enfants présentent une hernie associée qui doit être réparée en même temps. L'utilisation de la cavité péritonéale pour dériver l'hydrocéphalie par shunt ventriculopéritonéal ou pour dialyse péritonéale en cas d'insuffisance rénale ou autres maladies métaboliques comme l'hyperammonémie et l'acidose lactique sont des causes d'hyperpression intra-abdominale avec augmentation du nombre de cas de hernies jusqu'alors méconnues. Une meilleure connaissance de ces conditions propices à augmenter le nombre de hernies symptomatiques devrait permettre d'en faire le diagnostic plus tôt et de les traiter avant l'apparition des complications. La plupart des hernies non compliquées sont traitées sans hospitalisation. Cependant, quelques bébés et enfants ayant d'autres maladies concomitantes sont mieux traités avec un programme d'hospitalisation de jour, c'est-à-dire que l'enfant est hospitalisé pendant 24 heures après son opération.

     

Lung surfactant gelation induced by epithelial cells exposed to air pollution or oxidative stress

Lung surfactant lowers surface tension and adjusts interfacial rheology to facilitate breathing. A novel instrument, the interfacial stress rheometer (ISR), uses an oscillating magnetic needle to measure the shear viscosity and elasticity of a surfactant monolayer at the air-water interface. The ISR reveals that calf lung surfactant, Infasurf, exhibits remarkable fluidity, even when exposed to air pollution residual oil fly ash (ROFA), hydrogen peroxide (H2O2), or conditioned media from resting A549 alveolar epithelial cells (AEC). However, when Infasurf is exposed to a subphase of the soluble fraction of ROFA- or H2O2-treated AEC conditioned media, there is a prominent increase in surfactant elasticity and viscosity, representing two-dimensional gelation. Surfactant gelation is decreased when ROFA-AEC are pretreated with inhibitors of cellular reactive oxygen species (ROS), or with a mitochondrial anion channel inhibitor, as well as when A549-rho0 cells that lack mitochondrial DNA and functional electron transport are investigated. These results implicate both mitochondrial and nonmitochondrial ROS generation in ROFA-AEC-induced surfactant gelation. A549 cells treated with H2O2 demonstrate a dose-dependent increase in lung surfactant gelation. The ISR is a unique and sensitive instrument to characterize surfactant gelation induced by oxidatively stressed AEC.     

Cyclin alterations in giant cell tumor of bone.

Cyclins play an important role in regulating the passage of dividing cells through critical checkpoints in the cell cycle. Because alterations of several cyclins, especially cyclin D1, have been implicated in the development of many human neoplasms, we examined 32 cases of giant cell tumor of long bones for cyclin D1 gene amplification and protein overexpression using differential polymerase chain reaction and immunohistochemistry, respectively. In addition, the expression of cyclin D3, cyclin B1, and the proliferation-associated antigen Ki-67 (MIB-1) was assessed immunohistochemically. Low-level cyclin D1 gene amplification was detected in 61% of giant cell tumor cases. All tumors showed cyclin D1, cyclin D3, cyclin B1, and Ki-67 (MIB-1) staining; however, the distribution was very characteristic. Cyclin D1 protein expression was seen predominantly in the nuclei of the giant cells, with occasional mononuclear cells staining. There was no correlation between cyclin D1 gene amplification and protein overexpression. Cyclin D3 staining showed a similar distribution, with 88% of cases showing protein overexpression. Cyclin D1 and/or D3 staining in the giant cells was never associated with staining for either cyclin B1 or Ki-67 (MIB-1), as the expression of the latter two proteins was restricted to the mononuclear cells. Cyclin B1 overexpression was seen in 44% of cases. Ki-67 (MIB-1) staining was present in all cases, and between 10 to 50% of the mononuclear cells were positive. These results suggest that alterations in cyclin D1 and/or D3 might play a role in the pathogenesis of giant cell tumor of bone.