Sirenomelia associated with VACTERL and VACTERL-H syndrome / Sirenomelia asociaciada con síndrome de VACTERL y VACTERL-H

Sirenomelia or mermaid syndrome is an extremely rare congenital lethal malformation with a frequency between 1.5 and 4.2 per 1 000 000 pregnancies.The association of sirenomelia with the VACTERL association is very rare, with twenty cases reported in the literature and only two cases with VACTERL-H. We present two cases of sirenomelia, type I and type II associated with VACTERL-H and VACTERL syndromes and we review the literature. First time pregnancy women aged 15 and 40 years, without harmful habits and diseases, where between 25-27 gestational week (GW) the prenatal study identifies malformative fetus and the pregnancy is interrupted by medical evidence. The fetopathological examination in the first case identified sirenomelia type I associated with myelomeningocele, hydrocephalus, anal imperforation, single umbilical artery, bilateral renal agenesis, ureteral and bladder agenesis, tracheo-esophageal fistule, agenesis of external genitals, monkey fold of the left palm of the hand - VACTERL-H. In the second case, where genetic testing is normal, sirenomelia type II associated with agenesis of external genitalia, anal imperforation, myelomeningocele, dolichocrania, macroglossia, low set ears, left preauricular skin tag, long philtrum, lung hypoplasia, split cadiac apex, single umbilicalis artery, blind end colon, hepatomegaly, accessory spleen, polycystic horseshoe kidney, uterine and vaginal agenesis, presence of two ovaries and duodenal stenosis - VACTERL association. This two cases, lead us to believe that sirenomelia and the VACTERL association are probably different manifestations of a pathogenetic process leading to disorders of blastogenesis at different levels during embryonic development.

La sirenomelia es una malformación congénita y excepcionalmente rara, con una frecuencia entre 1,5 y 4,2 en un millón de embarazos. La combinación de la sirenomelia con el síndrome de VACTERL es igualmente rara. La literatura especializada informa sobre la existencia de una veintena de casos solamente; en lo que respecta a su asociación con el síndrome de VACTERL-H se conocen solo dos casos. Luego de realizar una revisión de la literatura presentamos dos casos de sirenomelia asociada con los síndromes de VACTERL-H y de VACTERL En el estudio se analizaron los primeros embarazos de dos mujeres, edad de 15 y de 40 años, respectivamente, ambas mujeres completamente sanas y sin hábitos viciosos. Entre la vigésima quinta y la vigésima séptima semana gestacional (SG) del embarazo ambas mujeres, el análisis prenatal comprueba la existencia de malformación del feto debido a lo cual los embarazos fueron interrumpidos por prescripción médica. El análisis fetopatológico del primer caso comprueba la existencia de sirenomelia de tipo I asociada con mielomeningocele, hidrocefalia, atresia anal, arteria umbilical única, agenesia bilateral de los riñones y de los ureteres que transportan la orina desde los riñones hasta la vejiga, fístula traqueoesofágica, agenesia de los órganos genitales externos, línea simiesca en la palma de la mano izquierda - VACTERLH. En el segundo caso, en que el análisis genético ha resultado normal, se observó la presencia de sirenomelia de tipo II asociada con agenesia de los órganos genitales externos, atresia anal, mielomeningocele, dolicocrania, macroglosia, orejas bajas, filtrum alargado, hipoplasia pulmonar, ápice cardíaco escindido, arteria umbilical única, colon terminado en ciego, bazo accesorio, poliquistosis renal, riñón en herradura, agenesia vaginal y de útero, presencia de dos ovarios y estenosis duodenal - VACTERL asociación. Los dos casos investigados permiten llegar a la conclusión de que la sirenomelia y su combinación con el síndrome de VACTERL probablemente sean manifestaciones diferentes de un proceso patogenético que conlleva la alteración de la blastogénesis en distintos niveles durante el proceso del desarrollo embrionario.

Juntos a la par: La acalasia en Síndrome de Down / Together at the same time: Achalasia in Down Syndrome

El síndrome de Down es la anomalía cromosómica más común, encontrándose asociado hasta en un 77 % de los casos con anomalías gastrointestinales que pueden ser de naturaleza estructural o funcional2. La Acalasia es un trastorno motor primario del esófago, de etiología desconocida e incurable1.La probabilidad de encontrar un paciente con síndrome de Down y acalasia es extremadamente rara. La prevalencia de Acalasia en el síndrome de Down es mayor, lo que implica una asociación única entre estas dos condiciones poco comunes(1). Los 3 tratamientos endoscópicos establecidos son: la aplicación de toxina botulínica y las dilataciones neumáticas, además de otro llamado POEM (per-oral endoscopic myotomy).Se presenta el caso clínico de paciente masculino de 45 años con diagnóstico de Síndrome de Down y Acalasia evaluado por el nuestro servicio. El principal objetivo es determinar la relación entre estas dos patologías, también revisamos las diversas opciones terapéuticas disponibles como así también como llegar al diagnóstico debido a que son pacientes poco colaboradores para la realización de estudios contrastados y manométricos. Se realizó una sesión de dilatación neumática como puente al tratamiento definitivo con posterior realización de Miotomía endoscópica perioral (POEM) presentando mejoría sintomatológica

Neuraxial analgesia in a parturient with the VACTERL association undergoing labor and vaginal delivery / Analgesia neuraxial em uma parturiente com síndrome de Vacterl em trabalho de parto normal

Abstract Introduction: The term VACTERL is an acronym for an association of congenital malformations: including vertebral, anal, cardiac, tracheo-esophageal, renal and limb anomalies. VACTERL anomalies pose a formidable challenge to anesthesiologists. We describe the anesthetic management of a parturient with VACTERL association, who underwent neuraxial analgesia for labor and vaginal delivery. Case report: A 23 year old primigravida at 39 weeks gestation presented in labor at 4 cm cervical dilatation, completely effaced, requesting labor analgesia. Past medical history included VACTERL association with an imperforate anus and a partial endocardial cushion defect, both repaired in early childhood. She also had significant dorso-lumbar scoliosis with an extra lumbar vertebra. An MRI performed at 14 years age revealed the above findings with no spinal cord abnormalities. With a normal neurologic exam, a combined spinal epidural technique was performed. Despite significant scoliosis, the epidural space was identified at approximately the L3-L4 interspace at a depth of 5 cm. Spinal Fentanyl 25 mcg was administered followed by continuous patient-controlled epidural analgesia. The patient experienced excellent pain relief throughout her labor, and had an uneventful vaginal delivery 5 h after epidural placement. Discussion: The rarity of VACTERL association in the obstetric population with its extensive anomalies mandates a multidisciplinary approach in the prenatal period as it can pose major challenges to all health care providers, including airway, ventilatory, cardiac and neuraxial problems. This is the first reported case of a successful and safe neuraxial technique in a laboring patient with the VACTERL association with albeit limited vertebral and spinal cord anomalies.

Resumo Introdução: O termo Vacterl é um acrônimo para uma associação de malformacões congênitas que inclui anomalias vertebral, anal, cardíaca, traqueal, esofágica, renal e dos membros (Limbs em inglês). As anomalias Vacterl representam um enorme desafio para os anestesiologistas. Descrevemos o manejo anestésico de uma parturiente com síndrome de Vacterl submetida à analgesia neuraxial para parto normal. Relato de caso: Paciente primípara, 23 anos, 39 semanas de gestação, apresentava em trabalho de parto 4 cm de dilatação cervical, apagamento completo, exigiu analgesia de parto. A história médica incluía síndrome de Vacterl com ânus imperfurado e defeito parcial do coxim endocárdico, ambos corrigidos na primeira infância. A paciente também apresentava escoliose dorso-lombar acentuada com uma vértebra lombar adicional. Uma RM feita aos 14 anos revelou os achados mencionados acima sem anormalidades na medula espinhal. Com um exame neurológico normal, a técnica anestésica combinada raquiperidural (CRP) foi usada. Apesar de escoliose significativa, o espaço peridural foi identificado próximo ao interespaço L3-L4 a uma profundidade de 5 cm. Fentanyl (25 mcg) foi administrado por via espinhal, seguido de analgesia peridural contínua controlada pela paciente. A paciente sentiu grande alívio da dor durante todo o trabalho de parto; o parto vaginal ocorreu 5 horas após a anestesia CRP sem intercorrências. Discussão: A raridade da síndrome de Vacterl na população obstétrica com suas extensas anomalias exige uma abordagem multidisciplinar no pré-natal porque pode representar grandes desafios para todos os prestadores de cuidados de saúde, inclusive problemas respiratórios, de ventilação, cardíacos e do neuroeixo. Este é o primeiro caso relatado de uma técnica neuraxial bem-sucedida e segura em uma paciente em trabalho de parto com síndrome de Vacterl, embora com anomalias limitadas da coluna vertebral e medula espinhal.

Técnica del tubo gástrico invertido como opción para la reconstrucción esofágica, en un hospital de tercer nivel en Bogotá, Colombia: presentación de dos casos y revisión de la literatura / Two Cases of Reversed Gastric Tube (RGT) Esophagoplasty at a Third Level Hospital in Bogota, Colombia and Literature Review

Introducción: las principales lesiones a nivel de la hipofaringe y el esófago son debidas a quemaduras por cáusticos y al cáncer esofágico. Estas pueden presentarse y comprometer las dos estructuras de un 17% a un 23%, lo que genera la necesidad de una gran resección y, a su vez, el reto de una gran reconstrucción. En este estudio presentamos nuestra serie de casos empleando la técnica del tubo gástrico invertido y una revisión de la literatura con discusión crítica de los aspectos principales de este procedimiento. Métodos: pacientes en quienes se llevó a cabo reconstrucción esofágica mediante la técnica del tubo gástrico invertido, desde enero de 2010 hasta enero de 2015. Resultados: un paciente presentó estenosis de la anastomosis cervical, la cual fue manejada con dilataciones endoscópicas. Ninguno de los pacientes tuvo disfagia con las modificaciones en la dieta o síntomas clínicos de síndrome de Dumping o retardo en el vaciamiento gástrico. Sin embargo, los dos pacientes presentaron reflujo gástrico y requirieron un inhibidor de la bomba de protones (IBP). Discusión: el tubo gástrico invertido no se utiliza a menudo para la reconstrucción después de una esofagectomía total. Su ventaja sobre la técnica convencional de ascenso gástrico, la interposición de colon o el tubo gástrico invertido supercargado es que es una operación de una etapa y un procedimiento simple que requiere solamente una anastomosis. Puede ser transferido a la región cervical o incluso al esófago faríngeo para crear una anastomosis. Conclusiones: esta técnica permite la creación de un conducto más largo para la reconstrucción esofágica, con una tasa de complicaciones baja y ausencia de mortalidad.

Introduction: The principal lesions in the hypharynx and esophagus are due to caustic burns and esophageal cancer which account for 17% to 23% of all events that compromise these two structures. They account for much of the surgery, especially for the challenge of major reconstruction. This study presents our series of cases using reversed gastric tube (RGT) esophagoplasty and presents a review of the literature and a critical discussion of the main aspects of this procedure. Methods: Patients underwent RGT esophagoplasties from January 2010 to January 2015. Results: One patient developed stenosis of the cervical anastomosis which was managed with endoscopic dilations. None of the patients developed dysphagia, clinical symptoms of dumping syndrome or delayed gastric emptying as the result of dietary modifications. Gastric reflux occurred in both patients and was teated with proton pump inhibitors. Discussion: RGT esophagoplasty is not often used for reconstruction after a total esophagectomy. Compared to the conventional technique of gastric ascent, interposition of the colon and supercharged reversed gastric tube techniques, it has the advantages that it is a one-step operation and is a simple procedure requiring only one anastomosis. It can be moved to the cervical region or even to the pharyngeal esophagus to create an anastomosis. Conclusions: This technique allows the creation of a longer duct for esophageal reconstruction and has a low complication rate with no mortality

Deficiência de hormônio de crescimento em um paciente com síndrome de VACTERL / Growth hormone deficiency in a patient with VACTERL syndrome

A Síndrome de VACTERL é um conjunto de malformações congênitas, que necessita para o diagnóstico pelo menos três das seguintes alterações: malformações vertebrais, atresia anal, anomalias cardiovasculares, fístula traqueoesofágica, atresia esofágica, malformações renais e displasia dos membros. É relatado caso de paciente com Síndrome de VACTERL e deficiência de hormônio de crescimento. A revisão da literatura aborda uma possível associação entre esses dois diagnósticos clínicos (AU)

The VACTERL syndrome is a set of congenital malformations, which requires at least three of the following changes to be diagnosed: vertebral defects, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal malformations and dysplasia of the limbs. Here we report the case of a patient with VACTERL syndrome and deficiency of growth hormone. A literature review discusses the possible association between these two clinical diagnoses (AU)

Achados audiológicos e eletrofisiológicos de indivíduos com a síndrome G/BBB / Auditory findings and electrophysiologics in individuals with G/BBB syndrome

A síndrome G/BBB é uma condição rara, caracterizada por hipertelorismo, fissura de lábio e palato e hipospádia. Não foram encontrados trabalhos sobre a audição em indivíduos com esta síndrome. OBJETIVO: Investigar a função auditiva em pacientes com síndrome G/BBB quanto à ocorrência ou não de perda auditiva e a condução nervosa auditiva periférica e central. MATERIAL E MÉTODO: Catorze pacientes de 7 a 34 anos, do gênero masculino, com a síndrome G/BBB, foram avaliados por meio de otoscopia, audiometria, timpanometria e potenciais evocados auditivos de tronco encefálico (PEATE). Forma de Estudo: Estudo de série clínico prospectivo. RESULTADOS: Limiares audiométricos normais em 12 (66,7 por cento) pacientes da amostra e alterados em dois (33,3 por cento), sendo um com perda condutiva e um neurossensorial. Quanto ao PEATE, foram encontrados: latências absolutas da onda I normais em todos os pacientes, aumento das latências absolutas da onda III e V em dois e seis pacientes respectivamente; latências interpicos I-III, III-V e I-V aumentadas em quatro, três e oito pacientes, respectivamente. CONCLUSÃO: Perdas auditivas periféricas podem ocorrer na síndrome G/BBB. Há evidências de comprometimento das vias auditivas centrais em nível do tronco encefálico. Estudos com exames de imagem são necessários para maior clareza dos achados clínicos.

The G/BBB syndrome is a rare condition characterized by hypertelorism, cleft lip and palate, and hypospadias. No studies were found on the hearing of individuals with this syndrome. AIM: To investigate the auditory function in patients with G/BBB syndrome, such as the occurrence of hearing loss, and central and peripheral auditory nerve conduction. METHODS: Fourteen male patients aged 7-34 years with the G/BBB syndrome were assessed by otoscopy, audiometry, tympanometry and evoked auditory brainstem response (ABR). Model: A retrospective clinical series study. RESULTS: Audiometric thresholds were normal in 12 (66.7 percent) of the sample and altered in two (33.3 percent), one with conductive and one with sensorineural loss. ABR resuts were: all patients had normal absolute wave I latencies; absolute wave III and V latencies were increased in two and six patients, respectively; interpeak latencies I-III, IV and V interpeak latencies were increased in four, three and eight patients respectively. CONCLUSION: Hearing loss can occur in the G/BBB syndrome. There is evidence of central auditory pathway involvement in the brainstem. Imaging studies are needed to clarify the clinical findings.

Aspectos clínicos e radiográficos de caprino com megaesôfago / Clinical and radiographic analyses of a goat with megaesophagus

A seven month old female goat showed neck swelling, apathy, appetite and weight loss, restlessness, increased salivation, cough, and regurgitation episodes which occurred post feeding. The animal was evaluated through both clinical and radiographic examinations. Plain radiography was performed and contrasted X-ray was done using barium sulfate. For radiological examination, the lateral projection was used for analysis. The results showed a radiopaque content in the esophageal lumen dorsally positioned to heart with density and appearance similar to the rumen. A dilation of the esophagus was reported and a diagnosis of megaesophagus was made. In conclusion, contrast esophagography must be included in the diagnosis of megaesophagus in goats.

Report of the first case of myxoid liposarcoma in Colombia: a rare tumor / Informe del primer caso de liposarcoma mixoide en Colombia: un tumor raro

Introduction: Primary esophageal myxoid liposarcoma is exceedingly rare. Sarcomas make up 1% of esophageal malignant tumors. There are only five (5) cases reported with this histological variant (myxoid) in previous literature. In Colombia, this is the first case reported and the sixth in the world. Objective: To report the first case in Colombia of myxoid liposarcoma of the esophagus with clinical, radiographic images, histology, surgical and to describe difficulties in the diagnosis. Methodology: We reviewed the clinical history of a 28-year old male patient. He was admitted to Hospital Universitario del Valle in Cali, Colombia with a clinical history of dysphagia, weight loss, and excessive salivation. The initial examination (esophagogram, cervical CAT scan and endoscopy) demonstrated a mass that was reported as a fibrovascular polyp. The finding of the pathological diagnosis was myxoid liposarcoma. Conclusions: The rarity of this condition recommends report of its detailed description. The myxoid liposarcoma of the esophagus can be diagnosed if a patient has a history of a slow-growing esophageal mass with a low tumor density in computed tomography in combination with surgical resection and histological examination.

Introducción: El liposarcoma mixoide es una neoplasia maligna del mesénquima con una presentación muy rara en esófago. Los sarcomas representan 1% de los tumores esofágicos malignos, y este tipo histológico es el menos frecuente. En la actualidad, se encuentran informes en la literatura de cinco (5) casos de esta variante histológica en el esófago. En Colombia, es el primer caso encontrado y el sexto (6º) a nivel mundial. Objetivo: Presentar el primer caso en Colombia de un liposarcoma mixoide en el esófago, sus características clínicas, imagenológicas, histología, manejo quirúrgico y las dificultades en su diagnóstico. Metodología: Se revisó la historia clínica de un paciente masculino de 28 años que ingresa por urgencias al Hospital Universitario del Valle en Cali, Colombia, con historia de disfagia, pérdida de peso y sialorrea. Los estudios imagenológicos como esofagograma, escanografía cervical y endoscopia de vías digestivas altas son consistentes con un pólipo esofágico y el manejo quirúrgico consistió en la resección parcial y luego la resección completa de la lesión. El informe histopatológico de la resección parcial comunicó un pólipo fibrovascular y el informe de la resección completa fue de liposarcoma mixoide. Las dificultades diagnósticas que surgieron en este caso se relacionan con otras encontradas en la literatura. Conclusiones: El liposarcoma mixoide del esófago es una entidad que presenta dificultades en su diagnóstico debido a que la presentación clínica no es específica. Aunque en estos casos las biopsias iniciales pueden suponer lesiones benignas, sólo hasta el procesamiento histológico de todo el espécimen, es posible realizar el diagnóstico de la entidad.

Duplicación esofágica: caso clínico / Esophageal duplication: a case report

We report an 11-month infant case, who consults electively to perform a laparoscopic Nissen fundoplication, given her background of gastro-esophageal reflux and hiatal hernia. Intraoperatively, a malformation of the distal third of the esophagus was found. The preoperative images showed no evidence of the lesión. The lesión was resected and sent to anatomopathologic analysis, confirming the diagnosis.

Se presenta el caso de una lactante de 11 meses con antecedentes de reflujo gastroesofágico y hernia hiatal que ingresa electivamente a la Sección de Cirugía Pediátrica, con el objetivo de realizar una fundoplicatura de Nissen laparoscópica. En el intraoperatorio se detecta una malformación en el tercio distal del esófago. En las imágenes realizadas previamente no se observó una alteración compatible. La lesión fue resecada y luego analizada en anatomía patológica, confirmándose el diagnóstico de una duplicación esofágica.

Laryngotracheoesophageal cleft; neonatal presentation and diagnostic challenges

Congenital laryngeal clefts are rare developmental disorders of the upper airway accounting for 0.3% to 0.5% of all congenital anomalies of the larynx. The study is reporting the research team's first experience with this anomaly at the Armed Force Hospital Southern Region to emphasize the difficulties and the challenges in the diagnosis as well as the treatment of such infants. This is a 32 week preterm female infant with antenatal ultrasound diagnosis of isolated esophageal atresia. After delivery the team faced a great difficulty in intubation and passing nasogastric tube. A large NGT size 12FR passed to the stomach, and contrast study showed abnormal anteriorly located esophagus, which suggested a common esophageo-tracheal tract. Esophagogram and soft fibro-optic endoscopy was done by ENT consultant which confirmed the presence of long segment laryngo-trachea-esophageal cleft. The infant was transferred to tertiary center where she died from respiratory failure at the age of 6 months

Pulmonary parenchymal alveolar histological study in experimental tracheo-oesophageal malformations / Estudio histológico alveolar y parenquimal pulmonar en malformaciones tráqueo-esofágicas experimentales

OBJECTIVE: Children operated on for tracheo-oesophageal malformation (TOM) often suffer from postoperative respiratory system difficulties. There is little current literature about this subject. This study aimed to investigate the causes of these problems in rats with experimental TOM by evaluating the lung alveolar histology. SUBJECTS AND METHODS: Twenty Wistar albino rats were used for the experiment. Twelve rats with a sperm positive vaginal smear received 1.75 mg/kg intraperitoneal adriamycin on days six to nine of gestation. A sham group was infused with saline instead of adriamycin. A control group was not subjected to any additional procedure. Their fetuses were dissected under surgical microscope. After examining the trachea and oesophagus, the lungs were dissected and fixed in 10% formalin. The groups were compared with respect to alveolar flat cell (Type-1), capillary density and air space percentage in the samples obtained under light microscopy. Statistical evaluation was performed through Mann-Whitney-U tests and Pearson Chi-squared tests. RESULTS: Type-1 cell ratio and air space percentage were the highest for the control and sham groups. However, the group that received adriamycin and developed TOM had the lowest values. There were no statistically significant differences between the groups with respect to capillary density. CONCLUSION: In rats with experimentally produced TOM, the pulmonary parenchyma showed delayed maturation. This could be the cause of the frequently seen respiratory system pathologies in children suffering from TOM. Further studies should be done to elucidate this.

OBJETIVO: Los niños operados por malformaciones tráqueo-esofágicas (MTE) sufren a menudo de dificultades postoperatorias en el sistema respiratorio. En el presente, existe poca literatura sobre este problema. Este estudio va encaminado a investigar las causas de estos problemas en ratas con MTE experimentales, mediante la evaluación de la histología alveolar del pulmón. SUJETOS Y MÉTODOS: Veinte ratas albinas Wistar fueron usadas en el experimento. Doce ratas con frotis vaginal positivo de esperma recibieron 1.75 mg/kg de adriamicina intraperitoneal en los días seis al nueve de gestación. A un grupo de simulación (sham group) se le dio una solución salina en lugar de adriamicina. El grupo de control no fue sometido a ningún procedimiento adicional. Sus fetos fueron disecados bajo microscopio quirúrgico. Luego de examinar la tráquea y el esófago, los pulmones fueron disecados y puestos en formaldehído al 10%. Recurriendo a la microscopía lumínica, los grupos fueron comparados con respecto a células alveolares planas (tipo 1), densidad capilar y porcentaje de espacio de aire en las muestras obtenidas. La evaluación estadística se realizó mediante tests U de Mann-Whitney y tests de Chi-cuadrado Pearson. RESULTADOS: La proporción de células de tipo 1 y el porcentaje de espacio de aire, fueron los más altos para los grupos de simulación y control. Sin embargo, el grupo que recibió adriamicina y desarrolló MTE, tuvo los valores más bajos. Con respecto a la densidad capilar, no hubo diferencias estadísticamente significativas entre los grupos. CONCLUSIÓN: En ratas con malformación MTE producida experimentalmente, el parénquima pulmonar mostró retardo en su maduración. Esto podría ser la causa de las patologías del sistema respiratorio observadas con frecuencia en niños que padecen de MTE. Se requieren estudios ulteriores a fin de dilucidar esta cuestión.

Unusual Bronchopulmonary Foregut Malformation Associated with Pericardial Defect: Bronchogenic Cyst Communicating with Tubular Esophageal Duplication

We report a case of unusual bronchopulmonary foregut malformation composed of a mediastinal bronchogenic cyst with sequestrated lung tissue and communicating tubular esophageal duplication associated with complete pericardial defect. A 18-yrold man, who had suffered from dry cough and mild dyspnea, was admitted because of an incidentally detected chest mass. A computed tomography scan demonstrated a cystic mass with an air fluid level connected with esophagus in the middle mediastinum. The surgically resected mass was a pleural invested accessory lobe of the lung (8.0x7.0x4.5 cm) connected with the esophageal wall by a tubular structure (3.0 cm in length and 2.0 cm in diameter). A complete left pericardial defect was also identified. Histologically, the cystic wall was composed of fibrovascular connective tissue with a smooth muscle layer, mixed seromucous glands and cartilage, and the inner surface of the cyst was lined by ciliated pseudostratified columnar epithelium. The inner surface of the tubular structure was lined by non-keratinizing or keratinizing squamous epithelium, and the wall contained submucosal mucous glands, muscularis mucosa, and duplicated muscularis propria. This case is important in understanding the embryological pathogenesis of the variable spectrum of the bronchopulmonary foregut malformation.

Pulmón esofágico: caso clínico / Esophageal lung: a case-report

Introducción: El pulmón esofágico es una malformación congénita muy infrecuente que se produce por una alteración en el desarrollo normal del intestino anterior. Consiste en la presencia de tejido pulmonar conectado con el esófago. Clínicamente se puede manifestar como neumonías recurrentes, dificultad respiratoria o disnea que aparece junto con la alimentación. Objetivo: Reportar el primer paciente portador de esta malformación tratado en nuestra institución. Caso Clínico: Lactante masculino, 4 meses de edad, con neumonías recurrentes del lóbulo superior derecho. El estudio con imágenes y endoscopía, confirmó la presencia de un pulmón supernumerario que estaba comunicado con el tercio medio del esófago. El paciente fue sometido a una resección quirúrgica de la malformación. Evoluciona favorablemente con mejoría de sintomatología. Conclusión: El pulmón esofágico es una malformación poco frecuente que debe ser sospechada en pacientes con sintomatología respiratoria recurrente. El estudio con imágenes y endoscopía permite precisar el diagnóstico.

A rare H-type of tracheo-oesophageal fistula in a young boy.

A 9-year-old male child presented with features of recurrent episodes of pneumonia. Following administration of different types of antibiotics, resolution of pneumonia was not complete. Antituberculous drugs were given on two occasions for sufficient period of time without any improvement. Following admission, history of a choking sensation during taking food pointed to perform barium swallow examination of oesophagus which revealed the diagnosis of tracheo-oesophageal fistula.

Efficacy of Combination of Intravenous Cyclosporin A and Steroid Therapy versus Prolonged Intravenous Steroid Therapy Alone in Patients with Severe Ulcerative Colitis Refractory to Initial Intravenous Steroid Therapy / 대한소화기학회지

BACKGROUND/AIMS: Maximal duration of intravenous (IV) corticosteroid (CS) treatment and efficacy of cyclosporin A (CsA) have not been clarified for patients with severe ulcerative colitis. We aimed to evaluate and compare the effectiveness of CS and CsA combination therapy with prolonged CS therapy alone in patients with severe UC refractory to initial CS therapy. METHODS: We retrospectively reviewed the medical records of 84 episodes of severe UC in 59 patients between April 1999 and May 2005. RESULTS: Among 84 episodes with IV CS therapy, 45 (53.6%) experienced an early response, while 39 (46.4%) did not respond within 2 weeks. The remaining 36 episodes excluding 3 which underwent colectomy were assigned to either combination therapy of IV CS and CsA or prolonged IV CS treatment alone for additional 2 weeks. Twelve of 16 episodes (75.0%) responded to therapy with combinations of IV CsA and CS, and 16 of 20 episodes (80.0%) to prolonged IV CS treatment alone. There was no statistical difference in response and colectomy rate after 4 weeks between CsA-use group and CsA-non-use group (p=1.00). CONCLUSIONS: These results suggest that CS and CsA combination has no additional benefit over prolonged CS therapy alone in terms of short-term response and that CS can be safely prolonged even after the first 14 days of treatment for severe UC.

Incomplete duplication of the esophagus: a case report.

A case of incomplete duplication of the esophagus diagnosed in a 20-month-old girl with an esophageal perforation is presented. X-ray films of the chest showed pneumomediastinum and subcutaneous emphysema. A water soluble contrast esophagogram revealed a contrast leakage from the cervical esophagus to the thoracic inlet. The endoscopic findings are described. Complete resection of the esophageal duplication was effective.

Imaging of congenital anomalies of the gastrointestinal tract.

The radiological imaging plays a vital role in the evaluation of patients with congenital anomalies of the gastrointestinal tract. The evaluation of these patients, most of which present early after birth, frequently requires the use of various imaging modalities for making the correct diagnosis and planning surgical correction. This article reviews the common congenital anomalies of the gastrointestinal tract including obstructive lesions, anomalies of rotation and fixation, anorectal anomalies, and intestinal duplications. The plain radiograph is often diagnostic in neonates with complete gastric of upper intestinal obstruction and further radiologic evaluation may be unnecessary. An upper gastrointestinal series should be performed in all patients with incomplete intestinal obstruction. Sonography is useful in the evaluation of many congenital anomalies affecting pediatric gastrointestinal tract especially hypertrophic pyloric stenosis, enteric duplication cysts, midgut malrotation, meconium ileus and meconium peritonitis. Moreover, CT and MRI has assumed a greater importance as these provide excellent anatomic details which may be necessary for correct diagnosis as well as treatment planning. This is particularly true in evaluation of congenital anomalies such as esophageal/enteric duplications, vascular rings and anorectal anomalies. It is important to be familiar with the role nad usefulness of the various imaging modalities so that these can be used judiciously to avoid unnecessary radiation exposure while minimizing the patient discomfort.

Incidental detection of tubular esophageal duplication in gastric cardia malignancy.

Congenital esophageal duplications represent about 15% of digestive tract duplications. We report a 38-year-old man who presented with longstanding heartburn and recent dysphagia. Endoscopy showed communicating tubular duplication of lower esophagus with ulceroproliferative growth at the gastric cardia extending into the lower esophagus. Histology of radical esophagogastrectomy specimen showed poorly differentiated adenocarcinoma of gastric cardia without evidence of Barrett's esophagus.

Oesophageal duplication cyst causing neonatal haemoptysis.

Duplication anomalies of the alimentary tract often present with myriad clinical symptoms and signs. There have been no reports of haemoptysis as the presenting feature and oesophageal duplication cyst is not considered as a cause for haemoptysis. An infant presented with this unusual manifestation. Chest X-ray showed persistent right upper zone haziness and contrast enhanced computed tomographic scan showed a multiloculated mass on the right side of the chest. The cystic mass was excised via a right thoracotomy and dense adhesions were noted with the oesophagus. The postoperative period was uneventful and histopathological examination revealed it to be a duplication cyst with ectopic gastric mucosa. Thus, the diagnosis was made retrospectively.