Recurrent acute pancreatitis caused by pancreatic biliary maljunction due to long common channel / Pancreatitis aguda recurrente causada por deficiente unión pancreático-biliar debido a un colédoco largo

The pancreatic biliary maljunction is a rare anomaly that affects mainly females, defined as an anatomical maljunction of the pancreatic duct and the biliary duct confluence, and may be a rare cause of recurrent acute pancreatitis. In order to early diagnosis and prompt treatment, ERCP has an important role in it

La Malformación de la unión biliopancreática es una afección rara y ocurre más en mujeres jóvenes. Es una causa de pancreatitis aguda de causa no conocida. CPRE es una herramienta eficiente para el diagnóstico y también para ser de la terapéutica

Validation of questionnaires to assess quality of life related to fecal incontinence in children with anorectal malformations and hirschsprung's disease / Validação de questionários para avaliação da qualidade de vida relacionada à continência fecal em crianças com malformações anorretais e doença de hirschsprung

Surgical treatment of anorectal malformations (ARMs) and Hirschsprung's disease (HD) leads to alterations in bowel habits and fecal incontinence, with consequent quality of life impairment. The objectives were to create and validate a Questionnaire for the Fecal Incontinence Index (FII) based on the Holschneider score, as well as a Questionnaire for the Assessment of Quality of Life Related to Fecal Incontinence in Children and Adolescents (QQVCFCA), based on the Fecal Incontinence Quality of Life. Methods: The questionnaires were applied to 71 children submitted to surgical procedure, in two stages. Validity was tested by comparing the QQVCFCA and a generic quality of life questionnaire (SF-36), and between QQVCFCA and the FII. A group of 59 normal children was used as control. Results: At two stages, 45.0% (32/71) and 42.8% (21/49) of the patients had fecal incontinence. It was observed that the QQVCFCA showed a significant correlation with the SF-36 and FII (Pearson's correlation 0.57), showing that the quality of life is directly proportional to improvement in fecal incontinence. Quality of life in patients with fecal incontinence is still globally impaired, when compared with control subjects (p<0.05, Student's t-test). There were also significant differences between the results of children with ARMs and children with HD. Conclusions: QQVCFCA and FII are useful tools to assess the quality of life and fecal incontinence in these groups of children. Children with ARMs submitted to surgical procedure and HD have similar quality of life impairment.

O tratamento cirúrgico das malformações anorretais (MAR) e da doença de Hirschsprung (DH) leva a alterações do hábito intestinal e incontinência fecal com prejuízo da qualidade de vida. Os objetivos foram criar e validar o Questionário para o Índice de Continência Fecal (ICF), baseado no Holschneider Criteria, bem como o Questionário para Avaliar a Qualidade de Vida Relativa à Continência Fecal em Crianças e Adolescentes (QQVCFCA), baseado no Fecal Incontinence Quality of Life. Métodos: Os questionários foram aplicados em 71 crianças operadas, em duas etapas. A validade foi testada por meio da comparação do QQVCFCA e um questionário genérico de qualidade de vida (SF-36) e entre o QQVCFCA e o ICF. Um grupo de 59 crianças normais foi usado como controle. Resultados: Nas duas etapas, 45,0% (32/71) e 42,8% (21/49) dos pacientes apresentaram incontinência fecal. Verificou-se que o QQVCFCA apresentou correlação significativa com o SF-36 e o ICF (correlação de Pearson 0,57) e mostrou que a qualidade de vida é diretamente proporcional à melhoria da continência fecal. A qualidade de vida no paciente com incontinência fecal está ainda comprometida globalmente, em comparação com os indivíduos controles (p<0,05; teste t de Student). Não houve ainda diferença significativa entre os resultados de crianças com MAR e crianças com DH. Conclusões: O QQVCFCA e o ICF são instrumentos úteis para a avaliação da qualidade de vida e da incontinência fecal nesses grupos de crianças. Crianças operadas de MAR e DH apresentam comprometimentos semelhantes da qualidade de vida.

Terminal ileum duplication: an unusual case of small bowel obstruction / Duplicación del ileon terminal: un caso inusual de obstrucción del intestino delgado

Duplications of the alimentary tract are uncommon congenital anomalies that usually present during infancy and early childhood. The case of an adolescent presenting with small bowel obstruction secondary to a duplication cyst is presented and the challenges in the management described.

Las duplicaciones del aparato digestivo constituyen anomalías congénitas poco frecuentes que suelen presentarse durante la infancia y niñez temprana. Se presenta el caso de un adolescente con obstrucción del intestino delgado secundaria a un quiste de duplicación, y se describen los desafíos del tratamiento.

Occult neurovesical dysfunction with anorectal malformations.

OBJECTIVE: To evaluate for the occurrence of occult NVD in children with anorectal malformations (ARM) using urodynamic evaluation. METHODS: This prospective study was carried out on children with ARM prior to and following definitive procedure. Urodynamic studies were performed on the Phoenix Griffon machine (Albyn Medical) using Phoenix plus software. RESULT: Nineteen children in the age range of 3 months to 156 months (mean = 19.2) were included in this study. Among these 19 children 13 underwent re-evaluation after definitive surgery for ARM. There were 11(57.9%) males and 8(42.1%) females. Of the 19 children 14 (73.7%) were cases of high anorectal malformation (HARM) and 5 (26.3%) were cases of low anorectal malformation (LARM). Baseline evaluation done in 19 children revealed seven urodynamic patterns: Normal capacity, compliant without uninhibited contractions (UIC) (21.1%); Normal capacity, compliant with UIC (5.3%); Normal capacity, poorly compliant without UIC (5.3%); Normal capacity, poorly compliant with UIC (10.5%); small capacity, compliant with UIC (5.3%); Small capacity, poorly compliant with UIC (26.3%) and large capacity, complaint with UIC (26.3%). Thirteen patients were evaluated post operatively also and in only 23% (3 of 13) no change in urodynamic pattern were observed. In the remaining 76.9% (10 of 13) some changes in urodynamics pattern were observed. The deleterious changes observed were appearance of UIC in 30.8% (4 of 13), decrease in the bladder capacity in 23% (3 of 13) and decrease in bladder compliance in 15.4% (2 of 13). CONCLUSION: Only 9 of of the 19 patients had normal urodynamics pre-operatively and post-operatively 3 more patients worsened. Incidence of occult NVD is high in patients with ARM even in the absence of clinical and radiological evidence of vertebral or lower urinary tract abnormalities. Though there seems to be a high incidence of changes in the neurovesical functions of these patients following definitive corrective surgery for ARM only time will show whether this has any deleterious effect on the upper tracts.

Duplicación gástrica / Gastric duplication

Introducción: La duplicación gástrica es una malformación poco frecuente del tubo digestivo, que en algunos casos puede dar sintomatología secundaria a una complicación. Objetivo: Reportar dos pacientes portadores de una duplicación gástrica que fueron resueltos por vía laparoscópica. Se detallan los aspectos clínicos, radiológicos y los beneficios de la técnica utilizada. Casos: Un lactante de 9 meses portador de una neurofibromatosis que en su estudio abdominal aparece como hallazgo una malformación quística del estómago, y un recién nacido que presenta vómitos frecuentes, dolor abdominal y masa palpable. Ambos tratados con cirugía laparoscópica, con buena evolución. Conclusión: La duplicación gástrica es una entidad poco frecuente que requiere tratamiento quirúrgico, ya sea por vía abierta o laparoscópica, siendo esta última una técnica recomendable y segura en la actualidad.

Congenital pyloric atresia: Isolated and associated with aplasia cutis congenita

Two cases of congenital pyloric atresia [CPA] are, hereby, reported. One was suspected on antenatal ultrasound and turned out to be an isolated anomaly. Other patient had a rare association of aplasia cutis congenita with congenital pyloric atresia. The lesions of aplasia cutis congenita were multiple while congenital pyloric atresia was of type II. The patient with an isolated lesion survived following surgery while the other baby died of sepsis in postoperative period

Gastrointestinal perforations in neonates with anorectal malformations.

We describe the presentation and management of gastrointestinal perforation in four neonates with anorectal malformations. Two neonates with high malformation had pneumoperitoneum on X-ray; surgery revealed sigmoid perforation in one patient and transverse colon perforation in the other. Colostomy was done, followed by posterior sagittal anorectoplasty at four months; both recovered satisfactorily. The third neonate had no radiological feature of gut perforation but cecal perforation was found at surgery; the neonate recovered following right hemicolectomy with stoma followed by anorectoplasty at five months. The fourth neonate presented with clinical and radiological features of perforation and recovered satisfactorily after anoplasty and colostomy.

Hernia diafragmática congénita: nuestra experiencia retrospectiva / Congenital diaphragmatic hernia: our restrospective experience

Se presenta un informe retrospectivo de 22 casos de hernia diafragmática congénita. Se dividieron en dos grupos de acuerdo al momento del diagnóstico, siendo prenatal en 11 pacientes y posnatal en la mitad restante. En el primer grupo el diagnóstico se hizo entre las 14 y 36 semanas de embarazo y los partos ocurrieron entre las 27 y 39 semanas. Seis niños fueron sometidos a cirugía correctora, sobreviviendo sólo 2; los otros 5 fallecieron antes de ser intervenidos, mostrando una mortalidad de 81,8 por ciento. En el segundo grupo se hizo diagnóstico entre el primer día de vida y los 11 meses de edad. Todos se sometieron a cirugía y dos fallecieron en el postoperatorio, con una mortalidad de 18,2 por ciento. La mortalidad general fue de 50 por ciento (11/22). Los resultados muestran que el diagnóstico prenatal de este defecto congénito se asocia a mayor mortalidad neonatal y se discute cuáles serían las razones de ello, así como sus implicancias para el tratamiento

Duplicaciones del tubo digestivo / Duplication of the digestive tube

Objetivo: Conocer la incidencia, clínica, histología, manejo y complicaciones de esta patología. Método: Estudio descriptivo retrospectivo de biopsias e historias clínicas de pacientes operados de duplicación en Hospital Roberto del Río en los últimos 30 años. Resultados: Se presentaron 25 casos. el doble en hombres que en mujeres, sin predominio etáreo, con mediana de 8 meses y rango de 1 a 14 años. Cuatro veces más frecuente las gastrointestinales que las esofágicas. En las primeras la clínica fue dolor abdominal, vómitos, distensión y HDA y mayoritariamente ubicadas en región ileal; en las esofágicas principalmente cuadros bronquiales a repetición. En sólo el 25 por ciento hubo sospecha preoperatoria. Como apoyo diagnóstico se ocupó Rx tórax y TAC. No hubo significativa asociación con otras malformaciones. En todos pudo realizarse resección de la duplicación. Histológicamente en 21 casos correspondió a tipo quístico y 4 tubulares (de ubicación ileal). No hubo mortalidad, si hubo morbilidad asociada como suboclusión intestinal, fístula esófago pleural e infección de herida operatoria. Conclusiones: La incidencia fue un caso por 790 biopsias, en razón de 2: 1 hombre: mujer. La clínica depende de su localización, siendo cuatro veces más frecuente las gastrointestinales que las esofágicas. Requieren un alto índice de sospecha. La malformación puede ser resecada en su totalidad en la mayoría de los casos, sin mortalidad, aunque no exento de morbilidad

Malformaciones congénitas anorrectales y sus asociaciones preferentes: experiencia del Hospital Clínico de la Universidad de Chile, período 1979-1999 / Anorectal congenital malformations and their associations in a Chilean University Hospital between 1979 and 1999

Background: Anorectal atresia is a relatively frequent malformation in the newborn. According to the Latin American Collaborative Study for Congenital Malformations (ECLAMC), its frequency is 4.1 per 10,000 born alive. Aim: To determine the frequency of anorectal malformations at birth, and compare the figures with those of other maternity hospitals in Chile participating in ECLAMC, with the figures of the whole ECLAMC and with figures from other worldwide monitoring systems. Patients and methods: All births occurred in the University of Chile Clinical Hospital between January 1979 and August 1999, were reviewed. Results: During the study period, 70,242 children were born, 4,486 had a malformation and 54 had an anorectal malformation (7.7 per 10,000 born alive). Fifty nine percent had other associated malformations (of the urinary tract in 42.5 percent, skeletal in 26 percent and cardiovascular in 18.5 percent). Five stillborn babies had other severe malformations. Twenty one children had a fistula. Forty three percent were male, 39 percent female and 18 percent had ambiguous sex. When compared with normal controls, malformed newborns had a lower birth weight, lower gestational age and a higher mean maternal age, a higher frequency of metrorrhagia during the first trimester of pregnancy, a higher number of siblings with malformations and a higher degree of consanguinity among parents. Conclusions: The participation of recessive genes in the etiology of anorectal malformations is suggested

Incidencia de las anomalías anorrectales en pacientes del Hospital Universitario de Los Andes 1982-1994 / Incidence of the abnormalities anusrrectal in patients of the Hospital Universitario of Los Andes 1982-1994

Se revisaron 60 historias clínicas del Servicio de Historias Médicas del Hospital Universitario de Los Andes y del Hospital Ambulatorio Urbano Tipo II "Venezuela", Mérida, correspondientes a los años 1982 a 1994. Se encontraron 42 casos con anomalías anorrectales y la mayor incidencia se presentó en los años 1991 (5 casos = 11.90 por ciento) y 1992 (6 casos = 14.28 por ciento). El sexo masculino fue el más afectado (76.19 por ciento), siendo las anomalías más frecuentes las de tipo alto (35.72 por ciento), en tanto que en el sexo femenino fueron las de tipo bajo (19.05 por ciento). La presencia de fístula se encontró en 19 casos (45.23 por ciento), 11 en el sexo masculino y 8 en sexo femenino. Además, el 83.33 por ciento de los pacientes, las anomalías anorrectales se encontraron asociadas a otro tipo de malformación, siendo el sistema urogenital el más afectado, con 27 casos.

Malformación anorrectal y anomalías congénitas asociadas en recién nacidos / Anorectal malformation and asociated congenital anormalies in newborn infants

Se estudiaron 50 recién nacidos con malformación anorrectal (MAR) que ingresaron en un período de dos años al servicio de neonatología del Hospital General Médico La Raza. El 60% de los casos correspondió a MAR alta, el 30% a MAR alta, el 30% a MAR baja y el 10% a MAR intermedia. En el 66% de los casos se encontró una o más anomalías congénitas asociadas a la MAR, el 42% de los casos presentó alteraciones del tracto genitourinario, el 38% fístula a uretra, vagina o periné, el 8% defecto de la pared abdominal y el 24% anomalías diversas; en el 12% se encontró la MAR como componente o asociada a entidades como el Síndrome de Down, el sSíndrome de "prune belly" y la asociación de VATER. Se hace énfasis en la necesidad de una búsqueda intencionada de malformaciones agregadas con objeto de identificar un sSíndrome genético que permita ofrecer un aseoramiento genético adecuado, así como la intervención multidisciplinaria para su tratamiento y manejo integral