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As far as we know, no report uses the Swenson transanal endorectal pull-through technique in an animal model. Our objective is to describe the use of this technique as an experimental model for training and research purposes. Ten Norfolk hybrid rabbits were randomly selected from our experimental laboratory, with a mean weight of 3539.3 (± 678.4) g. Neither colon preparation nor fast were used before the procedures. The surgical technique was based on the description performed by Levitt et al. (2013, J Pediatr Surg. 2013;48(11):2289-2295). Information related to the surgical procedures and the clinical evolution in the postoperative period were recorded and analyzed. There were no deaths or severe complications. The anesthetic and the surgical times were significantly higher for the first three animals of the experiment. Our animal model proved adequate to perform the transanal endorectal Swenson pull-through technique, allowing the training of surgical skills through a model similar to the human, with few anesthetic complications and good postoperative evolution, including postoperative follow-up. We believe that it will serve as a learning tool in many institutions that are continuously searching for improved new techniques and will support new researches in this area.

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INTRODUCTION: Hirschsprung's disease (HD) is characterized by the absence of ganglion cells in the submucosal and myenteric plexuses of the colon as a result of disorders in the migration and differentiation of enteric neural crest cells during embryogenesis. It is a cross-factor condition, with more than 11 genes identified in its pathogenesis, including the RET proto-onco gene. CASE REPORTS: We present the case of two siblings with total colon HD where a potentially pathogenic variant of the RET gene was found. Their father also had this condition. DISCUSSION: Prenatal diagnosis through genetic testing allows for informed decisions and care planning for the newborn, thus reducing delayed diagnosis and treatment, and minimizing long-term complications. Mutations such as the RET gene variant highlight the importance of the genetic approach in understanding and managing HD.

INTRODUCCION: La enfermedad de Hirschsprung (EH) se caracteriza por la ausencia de células ganglionares en los plexos submucoso y mientérico del intestino grueso, resultante de deficiencias en la migración y diferenciación de las células de la cresta neural entérica durante la embriogénesis. Es una condición multifactorial, con más de 11 genes identificados en su patogénesis, incluyendo el protooncogén RET. CASO CLINICO: Se presenta el caso de dos hermanos con EH de colon total, cuyo padre también padeció la enfermedad, y en quien se encontró una variante potencialmente patogénica en el gen RET. COMENTARIOS: El diagnóstico prenatal mediante pruebas genéticas permite decisiones informadas y la planificación de cuidados para el neonato afectado, reduciendo demoras en el diagnóstico y tratamiento, y minimizando las complicaciones a largo plazo. La identificación de mutaciones como la variante en el gen RET destaca la importancia del enfoque genético en la comprensión y manejo de la EH.

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BACKGROUND: Surgeons create a neorectum to repair patients with Hirschsprung's disease (HD), which should be formed from a normoganglionic bowel. However, the neorectum is occasionally created with a transition zone (TZ) bowel. A neorectum created with a TZ has been postulated as a cause of postoperative enterocolitis or constipation. This study compares the incidence of enterocolitis and constipation in patients with TZ neorectum and normoganglionic bowel. METHODS: We conducted a retrospective review of patients with rectosigmoid HD who underwent primary pull-through. Patients were divided into normoganglionic neorectum (NNR) and TZ neorectum. The diagnosis was based on the final histopathologic report of the proximal margin. The incidence of enterocolitis and constipation was compared between these two groups. RESULTS: A total of 98 HD patients were analyzed. Seventy-one patients fulfilled the inclusion criteria. 65 (92%) had a NNR, and six patients (8%) had a TZ neorectum. From these patients, 42 (59%) presented with enterocolitis or constipation. However, there was no significant difference between both groups. CONCLUSION: The present study showed no difference in the incidence of enterocolitis or postoperative constipation in HD patients with normoganglionic or TZ neorectum. These results suggest that TZ neorectum does not cause postoperative obstructive symptoms.

INTRODUCCIÓN: Los cirujanos crean un neo-recto para tratar a los pacientes con enfermedad de Hirschsprung (EH), que debe formarse con intestino normogangliónico; sin embargo, en ocasiones el neo-recto se forma con intestino de la zona de transición. Se ha postulado que un neo-recto en zona de transición causa enterocolitis o estreñimiento postoperatorio. El objetivo de este estudio fue comparar la frecuencia de enterocolitis y estreñimiento en pacientes con neo-recto en zona de transición y con neo-recto normogangliónico. MÉTODOS: Se llevó a cabo una revisión retrospectiva de pacientes con EH recto sigmoideo que se sometieron a descenso primario. Los pacientes se dividieron en el grupo neo-recto normogangliónico y el grupo con neo-recto en zona de transición. El diagnóstico del neo-recto se estableció con el informe histopatológico definitivo del margen proximal. Se comparó la frecuencia de enterocolitis y estreñimiento entre estos dos grupos. RESULTADOS: Se analizó un total de 98 pacientes con EH, de los cuales 71 pacientes cumplieron los criterios de inclusión; 65 (92%) con neo-recto normogangliónico y seis (8%) con neo-recto en zona de transición. Posteriormente, 42 (59%) pacientes presentaron enterocolitis asociada a Hirschsprung (HAEC) o estreñimiento; sin embargo, no hubo diferencia significativa entre ambos grupos. CONCLUSIONES: El presente estudio no demostró una diferencia en la frecuencia de HAEC o estreñimiento postoperatorio en pacientes con EH con neo-recto normogangliónico o en zona de transición. Estos resultados sugieren que un neo-recto en zona de transición no causa síntomas obstructivos postoperatorios.

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La enfermedad de Hirschsprung (EH) es infrecuente y la mayoría de los escasos casos se presentan en neonatos, raramente se diagnostica durante la adolescencia y menos son los casos documentados. Se presenta el caso de un varón de 16 años quien experimentó 15 días de estreñimiento, anorexia, vómitos postprandiales y distensión abdominal. Los exámenes imagenológicos mostraron un fecaloma masivo que obstruía el colon, y el examen anatomopatológico señaló EH. Ante el fracaso de medidas conservadoras de evacuación se realizó una hemicolectomía izquierda ampliada y una colostomía, más apendicectomía por obstrucción intestinal. El paciente fue dado de alta con antibioticoterapia. Este caso resalta la importancia de considerar EH en adolescentes con estreñimiento crónico y masas abdominales. Aunque los pacientes pueden experimentar problemas postoperatorios, la cirugía es el tratamiento más efectivo para la EH.

Hirschsprung's disease (HD) is uncommon, and most of the cases occur in neonates. It is rarely diagnosed during adolescence, and even fewer cases are documented. We present the case of a 16-year-old male who had 15 days of constipation, anorexia, postprandial vomiting and abdominal distension. Imaging examinations showed a massive fecaloma obstructing the colon, and histopathological examinations showed HD. Given the failure of conservative evacuation measures, an extended left hemicolectomy and colostomy were performed, plus appendectomy for bowel obstruction. The patient was discharged with antibiotic therapy. This case highlights the importance of considering HD in adolescents with chronic constipation and abdominal masses. Although patients may experience postoperative problems, surgery is the most effective treatment for HD.

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Abstract Background: Surgeons create a neorectum to repair patients with Hirschsprung´s disease (HD), which should be formed from a normoganglionic bowel. However, the neorectum is occasionally created with a transition zone (TZ) bowel. A neorectum created with a TZ has been postulated as a cause of postoperative enterocolitis or constipation. This study compares the incidence of enterocolitis and constipation in patients with TZ neorectum and normoganglionic bowel. Methods: We conducted a retrospective review of patients with rectosigmoid HD who underwent primary pull-through. Patients were divided into normoganglionic neorectum (NNR) and TZ neorectum. The diagnosis was based on the final histopathologic report of the proximal margin. The incidence of enterocolitis and constipation was compared between these two groups. Results: A total of 98 HD patients were analyzed. Seventy-one patients fulfilled the inclusion criteria. 65 (92%) had a NNR, and six patients (8%) had a TZ neorectum. From these patients, 42 (59%) presented with enterocolitis or constipation. However, there was no significant difference between both groups. Conclusion: The present study showed no difference in the incidence of enterocolitis or postoperative constipation in HD patients with normoganglionic or TZ neorectum. These results suggest that TZ neorectum does not cause postoperative obstructive symptoms.

Resumen Introducción: Los cirujanos crean un neo-recto para tratar a los pacientes con enfermedad de Hirschsprung (EH), que debe formarse con intestino normogangliónico; sin embargo, en ocasiones el neo-recto se forma con intestino de la zona de transición. Se ha postulado que un neo-recto en zona de transición causa enterocolitis o estreñimiento postoperatorio. El objetivo de este estudio fue comparar la frecuencia de enterocolitis y estreñimiento en pacientes con neo-recto en zona de transición y con neo-recto normogangliónico. Métodos: Se llevó a cabo una revisión retrospectiva de pacientes con EH recto sigmoideo que se sometieron a descenso primario. Los pacientes se dividieron en el grupo neo-recto normogangliónico y el grupo con neo-recto en zona de transición. El diagnóstico del neo-recto se estableció con el informe histopatológico definitivo del margen proximal. Se comparó la frecuencia de enterocolitis y estreñimiento entre estos dos grupos. Resultados: Se analizó un total de 98 pacientes con EH, de los cuales 71 pacientes cumplieron los criterios de inclusión; 65 (92%) con neo-recto normogangliónico y seis (8%) con neo-recto en zona de transición. Posteriormente, 42 (59%) pacientes presentaron enterocolitis asociada a Hirschsprung (HAEC) o estreñimiento; sin embargo, no hubo diferencia significativa entre ambos grupos. Conclusiones: El presente estudio no demostró una diferencia en la frecuencia de HAEC o estreñimiento postoperatorio en pacientes con EH con neo-recto normogangliónico o en zona de transición. Estos resultados sugieren que un neo-recto en zona de transición no causa síntomas obstructivos postoperatorios.

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Context: Hirschsprung's disease (HD) is one of the commonest problems requiring surgery in children. More than 95% of children present during new-born period, when they are treated with leveling colostomy and are followed with pull-through surgery a few months later, once the child has gained adequate weight to withstand a major surgery. The commonest pull through surgery done is the Duhamel retro-rectal pull-through (DRPT) repair. Settings and Design: This is a retrospective study of children who presented to one unit in our institute, a tertiary care referral hospital for children less than 12 years, with HD and underwent DRPT procedure during the period between July 2017 to June 2020. The children were evaluated after three years of follow-up for fecal incontinence and constipation. The study was conducted in children diagnosed with classical segment recto-sigmoid HD who underwent surgery. The children who were diagnosed with HD other than classical segment, who underwent primary pull through surgery and who underwent other repairs for HD were excluded from the study. Results: Thirty-two children underwent DRPT procedure during the study period. Of them, five (15.6%) children were lost on follow-up and one (3.1%) child had expired in the immediate post-operative period. Twenty-six children were included in the study. The bowel function score was calculated. The mean age of definitive surgery was 4.2 years. The follow-up period was a minimum of three years. Only two children had a "good" score of eighteen and above. Nineteen children had a "fair" score of 13-17. Five children had a "poor" score of less than thirteen, and among them, two had a "very poor" score of less than nine. The mean BFS was 13.72. Conclusions: Functional outcomes following Duhamel procedure are satisfactory, with 7.7% of children are in the fringe of requiring another surgery for constipation and pseudo-incontinence. (AU)

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BACKGROUND: Hirschsprung's Disease (HD) is characterized by intestinal sub-occlusion and the absence of enteric ganglion cells. A rectal biopsy examination is performed to confirm the diagnosis. In a recent study, we demonstrated that the analysis of 60 sections of rectal mucosa and submucosa stained by H&E may ensure a 90% diagnostic accuracy. Although the need to analyze so many sections makes the process of reading the slides more time-consuming, this encouraged us to study their distribution in the healthy rectal submucosa, to simplify the diagnosis. OBJECTIVES: To develop a method that facilitates HD diagnosis by studying the distribution of ganglion cells in the submucosal plexus. METHODS: Using the calretinin technique, we studied the distribution of plexuses in 60 fragments of rectal submucosa from 19 cadavers. After the study, the reading method created was used for diagnosis in 47 cases of suspected HD, using H&E staining. The accuracy was verified by comparing the results obtained with H&E to those obtained with the acetylcholinesterase technique, the golden standard in our laboratory. RESULTS: The study of submucosal plexus distribution showed that just by examining the submucosal region every 20 µm, approximately, it is possible to locate a ganglionic plexus, and we have already been able to diagnose HD with 93% accuracy. CONCLUSION: The study of ganglion cell distribution enabled the creation of a simplified method for reading the slides. The method applied achieved good accuracy and it can be used as an alternative method in HD diagnosis.

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Abstract Background: Hirschsprung's Disease (HD) is characterized by intestinal sub-occlusion and the absence of enteric ganglion cells. A rectal biopsy examination is performed to confirm the diagnosis. In a recent study, we demonstrated that the analysis of 60 sections of rectal mucosa and submucosa stained by H&E may ensure a 90% diagnostic accuracy. Although the need to analyze so many sections makes the process of reading the slides more time-consuming, this encouraged us to study their distribution in the healthy rectal submucosa, to simplify the diagnosis. Objectives: To develop a method that facilitates HD diagnosis by studying the distribution of ganglion cells in the submucosal plexus. Methods: Using the calretinin technique, we studied the distribution of plexuses in 60 fragments of rectal submucosa from 19 cadavers. After the study, the reading method created was used for diagnosis in 47 cases of suspected HD, using H&E staining. The accuracy was verified by comparing the results obtained with H&E to those obtained with the acetylcholinesterase technique, the golden standard in our laboratory. Results: The study of submucosal plexus distribution showed that just by examining the submucosal region every 20 µm, approximately, it is possible to locate a ganglionic plexus, and we have already been able to diagnose HD with 93% accuracy. Conclusion: The study of ganglion cell distribution enabled the creation of a simplified method for reading the slides. The method applied achieved good accuracy and it can be used as an alternative method in HD diagnosis.

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BACKGROUND: Hirschsprung's disease is characterized by the absence of enteric ganglia in the distal digestive tract; being usually diagnosed in neonates, it rarely debuts during adulthood, where it presents as chronic constipation refractory to treatment. CASE REPORT: 24-year-old female patient with Hirschsprung's disease who arrives to the emergency room with a sigmoid volvulus requiring urgent surgical therapy. CONCLUSIONS: Hirschsprung's disease doesn't frequently debut in adulthood, presenting a diagnostic and therapeutic challenge for the general surgeon, especially when dealing with its complications.

ANTECEDENTES: La enfermedad de Hirschsprung se caracteriza por un defecto de las células nerviosas en el intestino distal. Suele diagnosticarse durante los primeros años de vida, rara vez debutando en adultos, en quienes se manifiesta como una constipación crónica refractaria al tratamiento. CASO CLÍNICO: Mujer de 24 años con enfermedad de Hirschsprung no diagnosticada, quien acude a urgencias por un cuadro de vólvulo de sigmoides que requiere intervención quirúrgica. CONCLUSIONES: La enfermedad de Hirschsprung rara vez debuta en la edad adulta y generalmente se diagnostica secundaria a sus complicaciones, por lo que supone un reto diagnóstico y terapéutico para el cirujano general.

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Introdução: A doença de Hirschsprung, também conhecida como megacólon congênito, apresenta alterações na inervação entérica distal. Durante o período neonatal, apresenta-se através da tríade clássica de vômitos, distensão abdominal e atraso na eliminação meconial. Objetivo: Descrever quadro clínico de paciente do sexo masculino diagnosticado com megacólon congênito, brida congênita e má rotação intestinal. Comentário: Embora seja uma doença congênita, nem sempre o diagnóstico ocorre durante o período neonatal, devendo ser cogitada no raciocínio diagnóstico de pacientes mais velhos com história de constipação refratária ao tratamento. Aproximadamente 20% das crianças tem evolução pós-cirúrgica ruim, sendo a constipação a queixa mais comum, tendendo a melhorar com o passar dos anos. Não foi encontrado na literatura a associação de bridas congênitas, má rotação intestinal e doença de Hirschsprung. [au]

Introduction: Hirschsprung's disease, also known as congenital megacolon, presents alterations in the distal enteric innervation. During the neonatal period, it presents through the classical triad of vomiting, abdominal distension and delayed meconium elimination. Objective: To describe clinical case of a male patient diagnosed with congenital megacolon, whose initial presentation was neonatal intestinal obstruction attributed to congenital adhesion bands and intestinal malrotation and Hirschsprung's disease. Comments: Although it is a congenital disease, the diagnosis does not always occur during the neonatal period and should be considered in the diagnostic reasoning of older patients with a history of constipation refractory to treatment. Approximately 20% of children have negative outcomes on the postoperative course, with constipation being the most common complaint, tending to improve over the years. No association was found in the literature with congenital band, intestinal malrotation and Hirschsprung's disease. [au]

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OBJECTIVE: To determine the frequency with which inflammatory bowel disease (IBD) is diagnosed in persons with Hirschsprung disease in population-based datasets from 3 Canadian provinces. STUDY DESIGN: In study I, Ontario data were used to assess the incidence of IBD in a birth cohort of children with Hirschsprung disease relative to children without Hirschsprung disease. In study II, a case-control design was used in Alberta and Manitoba to determine the frequency of previously diagnosed Hirschsprung disease in persons with IBD, compared with the frequency of Hirschsprung disease in matched controls. Validated algorithms for Hirschsprung disease and IBD were applied to each provincial health registry. RESULTS: In study I, of the 716 children diagnosed with Hirschsprung disease in Ontario since 1991, 18 (2.5%) ultimately developed IBD (168.8 per 100 000 person-years), compared with 7109 of 3 377 394 children without Hirschsprung disease (0.2%, 14.2 per 100 000 person-years). The percentage of males with post-Hirschsprung disease IBD was 77.8%. The incidence rate ratio was 11.9 (95% CI, 7.5-18.8). In study II, the OR of having had Hirschsprung disease before a diagnosis of IBD compared with controls was 74.9 (95% CI, 17.1-328.7) in Alberta and 23.8 (95% CI, 4.6-123) in Manitoba. Crohn's disease was more common after Hirschsprung disease than ulcerative colitis. CONCLUSIONS: IBD can emerge in more than 2% of patients with Hirschsprung disease and, like Hirschsprung disease itself, is more common in males. IBD is much more common after a diagnosis of Hirschsprung disease than in the general population.

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PURPOSE: The gold standard for the diagnosis of Hirschsprung's disease (HD) is a rectal biopsy. The sample may be obtained using a transanal excisional biopsy (TEB) or suction technique. Rectal suction biopsy (RSB) is not a standard procedure in Latin-America. AIM: To evaluate the current practice in rectal biopsy for HD among pediatric surgeons. METHODS: We distributed an online questionnaire among Latin-American pediatric surgeons. RESULTS: One hundred forty-nine pediatric surgeons from 15 countries completed the anonymous survey (71.4% of Latin-American countries), grouped into 81.9% pediatric surgeons; 8.9% pediatric colorectal surgeons, 8.9% trainees, and 1 pediatric colorectal surgeon fellow. 50.4% reported less than 5 new patients with HD per year, 36.2% 5-10 new cases, and 13.4% more than 10. Only 14.1% of surgeons have access to perform a RSB in the diagnostic work-up of patients with suspected HD, 90% in our study perform an TEB under general anesthesia. When we ask if they could perform both procedures in babies up to 6 months, 52.3% indicate that they prefer an RSB, and for patients older than 6 months, 35.4% favor an RSB. Regarding the number of samples obtained performing an TEB, 30.9% get one biopsy, 29.5% two biopsies, and 39.6% three or more samples. Surgeons obtained the most proximal biopsy at a median of 2.3 cm (range 1-4 cm) above the pectinate line. 67.8% of surgeons prescribed antibiotic prophylaxis. Overall, 16.1% experienced complications, including rectal blood loss (n = 18), and rectal perforation (n = 3). The most frequently used staining methods for rectal biopsies are hematoxylin/eosin (87%), calretinin (56, 8%), and acetylcholinesterase (21.9%). CONCLUSIONS: In Latin-America, the accessibility for RSB is limited only 18 out of 149 surgeons have access to rectal suction tool. There is no consensus regarding sample number, site of proximal biopsy, and antibiotics use. The complications associated with the procedure seems to be less than reported with RSB. Therefore, we should standardize this common surgical practice and establish universal guidelines for rectal biopsy procedure (RBP).

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INTRODUCTION: Short-term international medical service trips (MSTs) provide specialized care in resource-constrained countries. There are limited data on immediate and long-term reported outcomes following specialty MST. We hypothesized that dedicated collaborative MST team and host institution produce outcomes and results comparable to those of high-income settings. Our primary aim was to analyze the long-term surgical and functional outcomes of our specialty-specific MSTs following five years of annual MST in Honduras. METHODS: We performed a single-institution retrospective analysis of 56 children who underwent colorectal and pelvic reconstructive operations between 2014 and 2018. Demographics, diagnosis, comorbidities, type of repair, long-term complications, and functional bowel and bladder results were recorded. RESULTS: We included a total of 56 children, 47 with ARM and 9 with HD, with a median age of 43.5 months (17-355) at the time of surgery. 25% (22) of the patients were lost to follow-up. Fecal continence was achieved by 23 (60%) patients <5 years who reported toilet training (n = 39) and by 5 (45%) patients >5 years (n = 11). Complications included constipation in 18 (42.9%) children with ARM and in 1 (12.5%) with HD. Eleven (19.6%) patients required revisional surgery for skin level anal stricture. Seventy-five percent of the patients with pediatric colorectal disorders attending the MST were compliant with continued long-term follow-up. CONCLUSION: We were able to demonstrate that with organized, dedicated site and surgeon, results achieved can be comparable to those in the high-income countries (HICs). We conclude that this type of specialized care is feasible and beneficial for affected pediatric colorectal patients in resource-limited settings, when a strong partnership with a system of preoperative assessments and peri- and postoperative care can be established. LEVEL OF EVIDENCE: Level IV (retrospective cohort study).

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La enfermedad de Hirschsprung ocurre en 1 de cada 5000 nacimientos. La falla de migración de las células ganglionares desde la cresta neural en dirección cefalocaudal genera su ausencia en parte o todo el colon. Se manifiesta con falta de eliminación de meconio, distensión abdominal y dificultades en la evacuación. Luego del tratamiento quirúrgico, existen complicaciones a corto y largo plazo. El objetivo de esta publicación es describir las principales causas de síntomas persistentes en los pacientes operados por enfermedad de Hirschsprung y presentar un algoritmo diagnóstico-terapéutico factible de ser realizado en nuestro medio

Hirschsprung disease is characterized by the lack of migration of intrinsic parasympathetic ganglia from neural crest and consequently absence of them at varying length of the bowel, resulting in functional obstruction. The incidence is 1 per 5000 births. After surgery, short term and long term comorbidity commonly occurs. The aim of this article is to revise the main causes of ongoing symptoms after surgery in Hirschsprung disease patients and to show a diagnostic and therapeutic algorithm that can be developed in our community

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Hirschsprung disease is characterized by the lack of migration of intrinsic parasympathetic ganglia from neural crest and consequently absence of them at varying length of the bowel, resulting in functional obstruction. The incidence is 1 per 5000 births. After surgery, short term and long term comorbidity commonly occurs. The aim of this article is to revise the main causes of ongoing symptoms after surgery in Hirschsprung disease patients and to show a diagnostic and therapeutic algorithm that can be developed in our community.

La enfermedad de Hirschsprung ocurre en 1 de cada 5000 nacimientos. La falla de migración de las células ganglionares desde la cresta neural en dirección cefalocaudal genera su ausencia en parte o todo el colon. Se manifiesta con falta de eliminación de meconio, distensión abdominal y dificultades en la evacuación. Luego del tratamiento quirúrgico, existen complicaciones a corto y largo plazo. El objetivo de esta publicación es describir las principales causas de síntomas persistentes en los pacientes operados por enfermedad de Hirschsprung y presentar un algoritmo diagnóstico-terapéutico factible de ser realizado en nuestro medio.

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La enfermedad de Hirschsprung es una malformación del sistema nervioso entérico, caracterizada por falta de células ganglionares en plexo submucoso y mientérico en pared distal del colon. En el 80% solo existe afectación del recto-sigma, sin embargo, pueden encontrarse casos de aganglionosis total. Reporte de Caso: Se presenta pre-escolar masculino de 4 años de edad, procedente de La Iguala, Lempira, Honduras, con historia de constipación y distensión abdominal de dos años de evolución, que ha empeorado progresivamente, y se atenuaba con el uso de enemas cada dos días para poder defecar. En mayo de 2016 llega al Hospital Mario Catarino Rivas, donde se le realizó una laparotomía exploratoria de emergencia debido a la presencia de deterioro clínico por constipación y distensión abdominal, acompañada de vómitos con restos alimenticios y fiebre alta de una semana de evolución. En dicha operación se realizó colectomía parcial izquierda, además se realizó una colostomía terapéutica y se diagnósticó enfermedad de Hirs-chsprung mediante biopsia, además del hallazgo incidental de apendicitis, se realizó apendicectomía obteniendo en general, una evolución satisfactoria.El estudio histopatológico de la biopsia del recto es el estándar de oro para realizar el diagnóstico. La ausencia de células ganglionares en el plexo submucoso con latinción hematoxilina y eosina establece el diagnóstico. El tratamiento de la enfermedad de Hirschsprung es de tipo quirúrgico y se busca la eliminación del segmento colónico afectado, de manera que se pueda lograr una anastomosis del colon proximal y distal al área agangliónica...(AU)

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ABSTRACT Background: Several types of complications including constipation, fecal soiling, perianal excoriation, were reported among different types of surgery for Hirschsprung's disease. Aim: To compare circular and oblique anastomoses following Soave's procedure for the treatment of Hirschsprung's disease. Methods: Children who underwent Saove's pull through procedure with oblique and circular anastomoses were included. Duration of the follow up was two years after surgery. Postoperative complications, such as wound infection, wound dehiscence, peritonitis, fecal soiling, perianal excoriation, were recorded for each patient. Results: Thirty-eight children underwent oblique anastomoses. Circular ones were done for 32 children. Perianal excoriation was seen in 57.89% and 46.87% of children in oblique and circular group, respectively. Enterocolitis was more frequent in circular (40.62%) than oblique (28.94%) group. Anastomotic stricture was more frequent in circular (15.62%) than oblique (7.89%). Conclusion: Perianal excoriation was the most common complication among patient in both groups. Oblique anastomoses had fewer complications than circular, and may be appropriate option for patient who underwent Soave's procedure.

RESUMO Racional: Vários tipos de complicações, incluindo constipação, secreção fecal, escoriação perianal foram relatadas entre diferentes tipos de operações para a doença de Hirschsprung. Objetivo: Comparar as anastomoses circulares e oblíquas realizadas no procedimento de Soave para o tratamento da doença de Hirschsprung. Métodos: Neste estudo, foram incluídas crianças submetidas ao procedimento pull-through de Saove com anastomoses oblíquas e circulares. A duração do acompanhamento foi de dois anos no pós-operatório. Complicações, como infecção da ferida, deiscência da ferida, peritonite, secreção fecal, escoriação perianal foram registradas para cada paciente. Resultados: Trinta e oito crianças foram submetidas à anastomoses oblíquas. As circulares foram realizadas em 32. Escoriação perianal foi observada em 57,89% e 46,87% das crianças nos grupos oblíquo e circular, respectivamente. Enterocolite foi mais frequente no grupo circular (40,62%) do que oblíquo (28,94%). A estenose anastomótica foi mais frequente na circular (15,62%) do que na oblíqua (7,89%). Conclusão: A escoriação perianal foi a complicação mais comum entre os pacientes nos dois grupos. A anastomose oblíqua teve menos complicações do que a anastomose circular e pode ser a opção adequada para o paciente submetido ao procedimento de Soave.

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Introduction: Hirschsprung's disease and anorectal malformations are pathologies that affect the pediatric population and compromise life from birth, making diagnosis and early treatment essential. The comorbidities they lead to in the long-term take parents and caretakers to situations that, without proper accompaniment, can generate serious complications in the child. Objective: To describe the meaning of the experiences of parents of children with Hirschsprung's disease or anorectal malformations who have had surgical treatment and are being followed-up. Materials and methods: This was a phenomenological qualitative research carried out through interviews with five parents of children with Hirschsprung's disease and five parents of children with anorectal malformations. Results: We found common experiences from which the following topics emerged: a) Difficult diagnosis, which involves the feelings and the impact generated by receiving this news; for health personnel it is not always a situation that is identified early; b) treatment: Social isolation due to hospitalizations and the presence of an ostomy, in addition to the channeling of resources for the child at the expense of those of the home; coping skills are required to achieve definitive surgical repair; and c) context: Health institutions may become barriers and health personnel must have more education in this regard; social support networks are necessary. Conclusion: The experiences reflected that diagnosis, treatment, and context generated great impact on the lives of parents and/or caretakers of children with these pathologies.

Introducción. La enfermedad de Hirschsprung y las malformaciones anorrectales son enfermedades que afectan a los niños y comprometen su vida desde el nacimiento, por lo que su diagnóstico y tratamiento temprano son esenciales. Las comorbilidades que implican a largo plazo llevan a los padres y cuidadores a situaciones que pueden generar graves complicaciones para el niño si no se cuenta con el acompañamiento adecuado. Objetivo. Describir y analizar las vivencias de los padres de niños con enfermedad de Hirschsprung o malformaciones anorrectales bajo seguimiento tras haber sido sometidos a cirugía. Materiales y métodos. Se hizo un estudio cualitativo con un enfoque fenomenológico mediante entrevistas a cinco padres de niños con enfermedad de Hirschsprung y a otros cinco de niños con malformaciones anorrectales. Resultados. Se encontraron experiencias comunes, de las cuales surgieron los siguientes temas: a) el diagnóstico difícil, debido a los sentimientos y al impacto generados por la noticia, situación que no siempre es detectada tempranamente por el personal de salud; b) el tratamiento, el cual provoca aislamiento social por las hospitalizaciones y la presencia de una ostomía, además de que los recursos se concentran en el niño en detrimento del hogar; se requiere capacidad de afrontamiento para lograr la reparación quirúrgica definitiva, y c) el contexto, ya que las instituciones de salud pueden convertirse en barreras; además, el personal de salud debe disponer de mayor educación al respecto y son necesarias las redes de apoyo social. Conclusión. Las vivencias reflejan que el diagnóstico, el tratamiento y el contexto generan un gran impacto en las vidas de los padres o cuidadores de niños con estas condiciones.

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BACKGROUND: Hirschsprung's disease (HSCR) is a common congenital malformation of the enteric nervous system (ENS). During fetal development, ganglion cells of the ENS are derived from neural crest cells that migrate to the bowel. These cells reside principally in two ganglionated plexus: 1) The myenteric plexus, extending from the esophagus to the anus, and 2) submucous plexus, extending from the duodenum to the anus. In large animal species, there is a third plexus called Henle's or Schabadasch's plexus. ENS ganglion cells play a key role in normal gastrointestinal motility, respond to sensory stimuli and regulate blood flow. Both plexus show a high degree of independence from the central nervous system. Alterations in the embryonic development of the ENS can induce multiple pathologies in animal models and humans. CASE PRESENTATION: The present case was a female the fifth born in a litter of 5 puppies. At about 2-3 weeks of age, she suffered from abdominal distension, pain, and constipation. At approximately 8-10 weeks of age, the puppy started to vomit abundantly, and the regurgitated food appeared undigested. Progressive abdominal distention was observed, with quite visible peristaltic movements and more frequent vomiting episodes. The abdominal radiographs, based on AP and side projections, revealed an enlargement of the abdominal diameter and an increased width in the epigastric region. At 12 weeks of age, exploratory surgery revealed a stenotic segment in the jejunum, followed by a small transition zone and then a significantly reduced diameter. Immunohistochemical examinations were performed using antibodies against calretinin, S-100 protein, CD56, neuron specific enolase (NSE) and synaptophysin, which are the biological markers for diagnosing HSCR. CONCLUSION: A reduced number of ganglion cells (1-3 cells per ganglion) were found. There was no specific staining pattern for many of these; while for others, the pattern was compatible with HSCR. Surgical intervention to remove the stenotic section prolonged the life of the puppy for 13 years. Extremely rare pathologies such as that discussed herein should be studied to understand the pathophysiology and be able to diagnose small species in veterinary medicine in a timely fashion. To our knowledge, this is the first report of congenital intestinal stenosis and Hirschprung's disease in a newborn puppy.

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. Antecedentes: La enfermedad de Hirschsprung (EH) es causa de obstrucción intestinal baja en neonatos. En 1998, De la Torre y Ortega publicaron una importante modificación a la técnica de Soave, realizando un descenso transanal endorrectal. Objetivo:Describir la experiencia en el tratamiento de la Enfermedad de Hirschsprung mediante la técnica descrita por De la Torre, Hospital Escuela, Tegucigalpa, 2013-2015. Metodología: Estudio descriptivo transversal. Se revisaron expedientes clínicos y se registró información sobre características sociodemográficas y clínicas de los pacientes. Los resultados se presentan como frecuencias y porcentajes de las variables estudiadas. La información personal de los pacientes se manejó confidencialmente. Resultados: Se identificó un total de26 casos intervenidos en el periodo del estudio. El 73.1% (19) pertenecía al sexo masculino, 46.2% (12) se diagnosticó antes de 12 meses de vida; sin enfermedades asociadas. La biopsia diagnosticó la enfermedad en el 100% de los casos. La longitud del colon resecado fue de 10-20 cm en 88.5% (23), con un tiempo quirúrgico de 4-5 horas 69.2% (18). El 19.2% (5) presentó complicaciones. Se inició alimentos en menos de 5 días en 96.1% (25), la estancia hospitalaria fue menor a 7 días en 88.5% (23). Discusión: El descenso endorrectal transanal es una operación segura con pocas complicaciones operatorias y postoperatorias. En este estudio, la estancia hospitalaria y el tiempo hasta la alimentación oral completa fueron más cortos que los procedimientos convencionales lo que generó menores costes hospitalarios. Consideramos que ésta es la técnica de elección para enfermos con la Enfermedad de Hirschsprung...(AU)

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