Untreated Congenital Hypothyroidism Mimicking Hirschsprung Disease: A Puzzling Case in a One-Year-Old Child.

Congenital hypothyroidism is a clinical emergency due to its potential risk of mental retardation. Constipation might be present in hypothyroid children. However, Hirschsprung disease is rarely associated with congenital hypothyroidism. Herein, a case of congenital hypothyroidism in a one-year-old child mimicking Hirschsprung disease is described. Adequate treatment with levothyroxine sodium tablets controlled intestinal dysmotility that mimicked congenital intestinal aganglionosis due to the critical influence of thyroid hormones on bowel motility.

Delineamento para a construção de um projeto pedagógico do curso de Medicina: relato de experiência / Guidelines for elaborating a pedagogic project for the Medical School: a report of experience

Este estudo tem como objetivo relatar a experiência da construção de um projeto pedagógico para o ensino em medicina. Trata-se de um relato de experiência desenvolvido por um grupo de profissionais no direcionamento dos princípios para a construção de um projeto pedagógico. A proposta em questão tem como princípio norteador a busca de uma aproximação de conteúdos e práticas que, superando dicotomias que fragmentam tanto o domínio teórico, quanto a intervenção assistencial, sustentem uma atuação médica qualificada e eficiente em diferentes contextos de ação.

This study aims at describing the construction of an educational project for teaching in a medical school. This is a report of an experience developed by a group of professionals for elaborating guiding principles for the construction of an educational project. The proposal has as a guiding principle the search for an approach to content and practices that overcomes dichotomies that fragment both the theoretical domain and intervention assistance, that supports a qualified medical performance and efficient action in different contexts.

Clinical, manometric and profilometric evaluation after surgery for Hirschsprung's disease: comparison between the modified Duhamel and the transanal rectosigmoidectomy techniques.

PURPOSE: To evaluate fecal continence, anorectal manometry (AM) and profilometry (P), in patients operated for congenital megacolon, using either the modified Duhamel technique (MDT) or the modified transanal rectosigmoidectomy (MTR) technique. METHODS: 42 patients were evaluated clinically and via AM and P, for postoperative control. The resting, coughing, voluntary contraction, maintained voluntary contraction and perianal stimulation pressures were investigated. The rectosphincteric reflex was tested and the simple and enhanced pressure curves were evaluated. The three-dimensional profilometric outline was produced. Student's t, chi-squared and Fisher's exact tests were used for statistical analysis (p<0.05). RESULTS: AM showed mean resting pressures of 53.44 mmHg for MDT and 60.67 mmHg for MTR, and mean voluntary contraction pressures of 94.50 mmHg for MDT and 95.47 mmHg for MTR. There was no statistical difference between the groups. The shapes of the simple and enhanced pressure curves did not present any statistical difference, independent of the surgical technique used. CONCLUSION: The two surgical techniques were equivalent. MDT caused greater incidence of postoperative constipation that MTR did. AM and P were shown to be excellent tests for postoperative follow-up among these patients.

Clinical, manometric and profilometric evaluation after surgery for Hirschsprung's disease: comparison between the modified Duhamel and the transanal rectosigmoidectomy techniques / Avaliação clínica, manométrica e profilométrica após correção cirúrgica para doença de Hirschsprung: comparação entre as técnicas de Duhamel modificado e a retossigmoidectomia transanal modificada

PURPOSE: To evaluate fecal continence, anorectal manometry (AM) and profilometry (P), in patients operated for congenital megacolon, using either the modified Duhamel technique (MDT) or the modified transanal rectosigmoidectomy (MTR) technique. METHODS: 42 patients were evaluated clinically and via AM and P, for postoperative control. The resting, coughing, voluntary contraction, maintained voluntary contraction and perianal stimulation pressures were investigated. The rectosphincteric reflex was tested and the simple and enhanced pressure curves were evaluated. The three-dimensional profilometric outline was produced. Student's t, chi-squared and Fisher's exact tests were used for statistical analysis (p<0.05). RESULTS: AM showed mean resting pressures of 53.44 mmHg for MDT and 60.67 mmHg for MTR, and mean voluntary contraction pressures of 94.50 mmHg for MDT and 95.47 mmHg for MTR. There was no statistical difference between the groups. The shapes of the simple and enhanced pressure curves did not present any statistical difference, independent of the surgical technique used. CONCLUSION: The two surgical techniques were equivalent. MDT caused greater incidence of postoperative constipation that MTR did. AM and P were shown to be excellent tests for postoperative follow-up among these patients.

OBJETIVO: Avaliar a continência fecal, a manometria anorretal (MAR) e a profilometria (PFM), em pacientes submetidos a tratamento cirúrgico do megacolo congênito pelas técnicas de Duhamel modificado (DM) ou de retossigmoidectomia transanal modificada (RTM). MÉTODOS: 42 pacientes foram clinicamente avaliados e submetidos à MAR e PFM para controle pós-operatório. Foram pesquisadas as pressões no repouso (PR), à tosse (T), à contração voluntária (CV), à contração voluntária mantida (CVM) e à estimulação perianal (EPA). O reflexo reto-esficteriano (RRE) foi testado e as curvas pressóricas simples e potencializada foram avaliadas. Foi elaborado traçado tridimensional de PFM. Utilizamos os testes t de Student, Qui-Quadrado e exato de Fisher para análise estatística (p<0,05). RESULTADOS: A MAR mostrou médias de PR=53,44 mmHg para DM e 60,67 mmHg para RTM; CV média = 94,50 mmHg para o grupo DM e 95,47 mmHg para o grupo RTM. Não houve diferença estatística entre os grupos. A forma das CPS e CPP não apresentou diferença estatística, independentemente da técnica cirúrgica utilizada CONCLUSÃO: As duas técnicas operatórias foram manometricamente equivalentes. DM determinou maior incidência de constipação pós-operatória que RTM. A MAR e a PFM revelaram-se excelentes exames para acompanhamento destes pacientes após a cirurgia.

Posterior sagittal anorectoplasty in anorectal anomalies: clinical, manometric and profilometric evaluation.

CONTEXT AND OBJECTIVE: Anorectal malformations comprise a spectrum of anomalies that continue to be difficult to treat, even today. The aim was to evaluate the fecal continence of children who underwent posterior sagittal anorectoplasty due to anorectal malformations, via computerized anorectal manometry and profilometry. DESIGN AND SETTING: Prospective study at Universidade Federal de São Paulo. METHOD: 82 patients (56.1% boys; 43.9% girls) of mean age 85.5 months were evaluated. They were divided into continent, partially continent and incontinent groups. Age, sex, manometric variables and profilometric parameters were studied. The results were statistically analyzed. RESULTS: Among the 82 patients, 37.8% were continent, 25.6% were partially continent and 36.6% were incontinent. The overall mean resting pressure was 22 mmHg, and the means for the continent, partially continent and incontinent groups were, respectively, 30.7 mmHg, 23 mmHg and 14.7 mmHg. The overall mean pressure response to voluntary contraction was 56 mmHg, and the means for the groups were 65.4 mmHg, 55.8 mmHg and 46.6 mmHg, respectively. The rectosphincteric reflex was absent in 82.9% of the cases. In the profilometry analysis for all patients together, blue (20 to 50 mmHg) and yellow (50 to 80 mmHg) were predominant, and there was a similar distribution for the continent and partially continent patients. However, among the incontinent patients, green (< 20 mmHg) and blue prevailed. CONCLUSIONS: Manometric and computerized profilometric analyses were an excellent method for postoperative evaluations on patients with intermediate and high anorectal anomalies, and for therapeutic planning.

Posterior sagittal anorectoplasty in anorectal anomalies: clinical, manometric and profilometric evaluation

CONTEXT AND OBJECTIVE: Anorectal malformations comprise a spectrum of anomalies that continue to be difficult to treat, even today. The aim was to evaluate the fecal continence of children who underwent posterior sagittal anorectoplasty due to anorectal malformations, via computerized anorectal manometry and profilometry. DESIGN AND SETTING: Prospective study at Universidade Federal de São Paulo. METHOD: 82 patients (56.1 percent boys; 43.9 percent girls) of mean age 85.5 months were evaluated. They were divided into continent, partially continent and incontinent groups. Age, sex, manometric variables and profilometric parameters were studied. The results were statistically analyzed. RESULTS: Among the 82 patients, 37.8 percent were continent, 25.6 percent were partially continent and 36.6 percent were incontinent. The overall mean resting pressure was 22 mmHg, and the means for the continent, partially continent and incontinent groups were, respectively, 30.7 mmHg, 23 mmHg and 14.7 mmHg. The overall mean pressure response to voluntary contraction was 56 mmHg, and the means for the groups were 65.4 mmHg, 55.8 mmHg and 46.6 mmHg, respectively. The rectosphincteric reflex was absent in 82.9 percent of the cases. In the profilometry analysis for all patients together, blue (20 to 50 mmHg) and yellow (50 to 80 mmHg) were predominant, and there was a similar distribution for the continent and partially continent patients. However, among the incontinent patients, green (< 20 mmHg) and blue prevailed. CONCLUSIONS: Manometric and computerized profilometric analyses were an excellent method for postoperative evaluations on patients with intermediate and high anorectal anomalies, and for therapeutic planning.

CONTEXTO E OBJETIVO: As anomalias anorretais correspondem a um espectro de malformações de tratamento difícil mesmo nos dias de hoje. O objetivo foi avaliar crianças portadoras de anomalias anorretais altas e intermediárias, operadas pela anorretoplastia sagital posterior quanto à continência fecal através da manometria anorretal e profilometria computadorizadas. TIPO DE ESTUDO E LOCAL: Trabalho prospectivo, na Universidade Federal de São Paulo. MÉTODO: Avaliamos 82 pacientes agrupados em continentes, parcialmente continentes e incontinentes quanto a idade, sexo e variáveis padronizadas na manometria anorretal e profilometria. Os resultados foram analisados estatisticamente. RESULTADOS: Dos 82 pacientes 37,8 por cento eram continentes, 25,6 por cento parcialmente continentes e 36,6 por cento incontinentes. A média da pressão de repouso à manometria anorretal foi de 22 mmHg, sendo entre os continentes, parcialmente continentes e incontinentes, respectivamente de 30,7 mmHg, 23 mmHg e 14,7 mmHg. A média da resposta pressórica à contração voluntária foi de 56 mmHg, sendo entre os continentes 65,4 mmHg, parcialmente continentes 55,8 mmHg e incontinentes 46,6 mmHg. O reflexo reto-esfincteriano encontrava-se ausente em 82,9 por cento dos casos. Predominaram na profilometria as cores azul (20 a 50 mmHg) e amarela (50 a 80 mmHg), quando todo o grupo foi analisado conjuntamente, com padrão semelhante entre os continentes e parcialmente continentes; nos incontinentes, destacaram-se as cores verde (< 20 mmHg) e azul. CONCLUSÕES: A manometria anorretal computadorizada e a profilometria mostraram-se úteis na avaliação do comportamento pressórico esfincteriano, assim como no acompanhamento pós-operatório e planejamento terapêutico dos pacientes.

Modified transanal rectosigmoidectomy for Hirschsprung's disease: clinical and manometric results in the initial 20 cases.

PURPOSE: The authors describe a modified technique of primary transanal rectosigmoidectomy for Hirschsprung's disease (HD), using a Swenson like procedure to perform the anastomosis between the colon and the rectum, and the preliminary results from this in children. METHODS: Twenty children, of whom, 90% were boys and 10% girls, 50% white and 50% nonwhite, aged 15 days to 10 years and with HD proven via biopsy, underwent a transanal pull-through procedure over a 29-month period. Postoperative follow-up ranged from 29 to 5 months. The proximal cut edge of the mucosal and submucosal cuff was tagged with multiple polypropylene 4-0 sutures, which were used for traction of the intestinal layers outside. The rectal mucosa was incised circumferentially using cautery, to perform rectal dissection approximately 1.5 cm from the dentate line, except in newborn case, in which the proximal cut edge was 0.5 cm from the dentate line. The dissection extended in an upward direction around the entire rectal circumference as far as the opening of the peritoneal reflection. The full thickness of rectum and sigmoid were mobilized outside through the anus, with division and coagulation of the rectal and sigmoid vessels using cautery or ligatures with cotton 4-0. The dissected colon then was divided above the transition zone, which was confirmed via full-thickness biopsy sections and with frozen section confirmation of ganglion cell presence. The authors performed a modified Swenson anastomosis technique, using a seromuscular polyglactin 4-0 separate-stitch suture. No drains were used. RESULTS: Normal bowel movements were displayed by all patients at the follow-up. All patients underwent a defecogram and anorectal computerized manometry at 3 months after surgery that showed an absence of stenosis and good anorectal sphincter muscle complex function. The incidence of complications in our series was 10%. CONCLUSIONS: During the follow-up period of 29 months, all patients had normal bowel movements and normal anorectal manometric pressure profiles.

Retossigmoidectomia transanal modificada para o tratamento do megacólon congênito: resultados clínicos, radiológicos e manométricos / Modified transanal retossigmoidectomy for Hirschsprung's disease: clinical, radiological and manometric results

Introdução: Descreve-se, aqui, uma técnica modificada para a retossigmoidectomia transanal, em estágio único, para o tratamento da Doença de Hirschsprung, realizando-se a sutura colorretal de maneira semelhante à proposta por Swenson. Apresentam-se os resultados em 20 crianças. Objetivo: Avaliar clínica, radiológica e manometricamente os pacientes portadores de megacólon congénito clássico e de segmentos curto e longo, submetidos ao tratamento cirúrgico de abaixamento transanal, com anastomose colorretal semelhante à técnica de Swenson, em procedimento único. Métodos: No período de novembro de 1999 a abril de 2002, 20 crianças portadoras de megacólon congénito, com diagnóstico confirmado por exame anatomopatológico, foram submetidas a retossigmoidectomia transanal, sem colostomia prévia, e anastomose colorretal semelhante à técnica de Swenson. Dentre os pacientes, 90 por cento eram do sexo masculino, sendo 50 por cento brancos. A idade variou de 15 dias a 11 anos de idade. O acompanhamento pós-operatório estendeu-se de 29 meses para o primeiro paciente operado a 5 meses, para o último paciente operado. Após anestesia geral inalatória e adequado posicionamento, realizou-se a passagem de pontos com fio de poligalactina 4-0 a fim de reparar a borda retal a ser suturada. Realizou-se incisão mucosa e submucosa retal, reparando-se o cólon a ser ressecado com fios de polipropileno 4-0. A tração dos fios permite a dissecção da parede retal, em sua totalidade, até se atingir a reflexão peritoneal, que, após aberta, permite a sigmoidectomia por meio de criteriosa hemostasia do mesossigmóide. Após identificação da zona de transição, confirma-se o diagnóstico por meio de exame anatomopatológico de congelação, realizando-se a exerese do segmento aganglionar e a anastomose colorretal...(au)