ABSTRACT
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)
Subject(s)
Humans , Male , Female , Treatment Outcome , Colon/surgery , Hirschsprung Disease/therapy , Quality of Life , Health Profile , Retrospective Studies , DefecationABSTRACT
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
Subject(s)
Humans , Hirschsprung Disease/surgery , Hirschsprung Disease/diagnosis , Hirschsprung Disease/therapy , Pediatrics , Chronic Disease , Constipation/diet therapy , Constipation/etiology , Enterocolitis/diet therapy , Enterocolitis/etiology , Fecal Incontinence/diet therapy , Fecal Incontinence/etiologyABSTRACT
La enfermedad de Hirschsprung es la causa más común de obstrucción intestinal inferior en el primer mes de vida. El objetivo de esta publicación es comunicar una forma de presentación frecuente de esta enfermedad y reforzar la importancia de la sospecha y acciones oportunas por parte del pediatra. Caso clínico: recién nacido varón 36hs de vida. Término, adecuado a la edad gestacional, vigoroso, sin dismorfias externas. A las 36 hs de vida instala vómitos, distensión abdominal progresiva, sin expulsión de meconio desde el nacimiento. Al examen abdomen distendido, dolor difuso a la palpación. Tacto rectal: ampolla vacía. Radiografía simple de abdomen compatible con oclusión intestinal. Radiografía de colon con contraste sugestiva de Enfermedad de Hirschsprung (EH) de segmento corto que se confirma con biopsia rectal. Se realizan lavados colónicos hasta resolución quirúrgica a los 6 meses de vida. Requirió plan de dilatación anal con buena evolución posterior sin complicaciones. Conclusiones: El pediatra cumple un rol fundamental en la sospecha diagnóstica de la EH y debe conocer sus diferentes formas de presentación. Es de suma importancia sospechar esta enfermedad en recién nacidos que presenten oclusión intestinal en las primeras horas de vida.
Hirschsprung's disease is the most common cause of lower intestinal obstruction in the first month of life. The objective of this publication is to communicate a frequent form of presentation of this disease and to reinforce the importance of timely diagnosis by pediatricians. Clinical case: male newborn 36hs of life. Term, Appropiate for gestational age, vigorous, without external dysmorphism. After 36 hours of life, he started vomiting, progressive abdominal distension, no meconium was observed. At the distended abdomen examination, diffuse pain on palpation. Rectal touch: empty blister. Simple abdominal radiography compatible with intestinal occlusion. Colon radiograph with contrast suggestive of Hirschsprung's disease (HD) of short segment that was confirmed with rectal biopsy. Colonic irrigations were performed until surgical resolution at 6 months of age. It required an anal dilatation plan with good posterior evolution without complications. Conclusions: The pediatrician has a fundamental role in the diagnostic suspicion of HD and must know its different forms of presentation. It is very important to suspect this disease in newborns who have intestinal occlusion in the first hours of life.
Subject(s)
Humans , Male , Infant, Newborn , Hirschsprung Disease/complications , Hirschsprung Disease/diagnosis , Intestinal Obstruction/etiology , Hirschsprung Disease/therapyABSTRACT
A identificação pré-operatória da zona de transição na Doença de Hirschsprung (DH) tornou-se um passo fundamental para o planejamento cirúrgico, especialmente para a técnica de abaixamento endorretal transanal. O presente estudo tem como objetivo investigar prospectivamente o valor da determinação colonoscópica da zona de transição na avaliação pré-operatória dos pacientes com DH. A colonoscopia foi realizada em doze pacientes com diagnóstico de DH previamente confirmado pela manometria anorretal, enema opaco e biópsia de sucção do reto. Endoscopicamente, o primeiro local com ausência de peristaltismo foi identificado como o início da zona agangliônica. Pouco acima deste ponto, a zona de transição foi marcada através de uma tatuagem com tinta nanquim. Durante o abaixamento endorretal transanal, uma biópsia de congelação envolvendo a espessura total da parede foi sempre realizada. Os resultados da determinação colonoscópica da zona de transição foram comparados com os obtidos pelo enema opaco. A colonoscopia permitiu a identificação da zona de transição em todos os 12 casos (100%). O enema opaco revelou a presença da zona de transição em apenas 7 pacientes (58,3%). A análise das amostras de congelação, obtidas pouco acima das áreas endoscopicamente marcadas, revelou a presença de células ganglionares em todos os casos. A análise histopatológica das peças cirúrgicas confirmou o diagnóstico de DH em todos os casos, assim como a localização da zona de transição no mesmo local previamente tatuado endoscopicamente...
Preoperative identification of the transition zone in Hirschsprungs disease (HD) has become an essential issue for surgical planning, especially for Transanal Endorectal Pull- Through (TEPT) procedure. The present study aimed to investigate prospectively, the value of endoscopic marking of the transition zone between normal and aganglionic bowel, as a landmark of the location of pull-through procedure for treatment of HD. Colonoscopy was performed on twelve patients with HD diagnosis, previously confirmed by anorectal manometry, contrast enema and rectal suction biopsies. Endoscopically, the first site with absence of motility was identified as the beginning of the aganglionic area. Just above this point, the transition zone was marked with an Indian Ink tattooing. During the TEPT, a full-thickness biopsy for frozen section analysis was performed just above this mark. The results of colonoscopic making were compared with contrast enema. Colonoscopy allowed the identification and tattooing of the junction between normal bowel with peristalsis and aganglionic bowel without peristalsis in all 12 cases (100%). Barium enema revealed the transition zone in 7 patients (58.3%). Frozen samples, obtained just above the marked areas revealed the presence of ganglion cells in all cases and the histopathological analysis of surgical specimens confirmed the diagnosis of HD in all cases and checked the location of the transition zone at the same site previously identified by colonoscopy...
Subject(s)
Humans , Male , Female , Child , Hirschsprung Disease/surgery , Hirschsprung Disease/diagnosis , Hirschsprung Disease/pathology , Hirschsprung Disease/therapy , Endoscopy/methods , Immunohistochemistry/methodsABSTRACT
Background: Hirschsprung's disease (HD) is a common cause of intestinal obstruction in children. Definitive treatments consist of excision of the aganglionic segment and anastomosing the normal colon to the anal remnant. The outcome of this approach in Nigerian children is not known. The aim of this study was to analyze the short to mid-term outcome of children who have undergone the Swenson Procedure (SPT) as a treatment of HD over a period of 10 years. Patients and Methods: The clinical data of biopsy-proven cases of HD managed at the Obafemi Awolowo University Teaching Hospital; Ile-Ife; Nigeria; between 1998 and 2007 were reviewed. Results: There were 33 children 28 were males and five were females (M 5.6: F = 1). The median age at presentation was eight months (range three days to 11 years). Eighteen (54.5) patients had primary pull-through without colostomy while 15 (45.5) patients had a two-staged operation. The operation was carried out at a median age of 14 months with a range of seven weeks to 11 years; four months. Twenty-one (64) of the patients were older than one year at the time of surgery. The level of aganglionosis was in the recto-sigmoid area in 30 (91) patients. The commonest post-SPT complication was intestinal obstruction from adhesions. Bowel opening varied from once daily to eight times daily; median thrice daily. Conclusion: The Swenson's Procedure is an effective procedure in the treatment of HD in children in Ile Ife; Nigeria
Subject(s)
Child , Hirschsprung Disease/therapy , Nigeria , Surgical Procedures, Operative , Treatment OutcomeABSTRACT
La enterocolitis asociada a enfermedad de Hirschsprung (EAEH) se define como la presencia de diarrea, deposiciones explosivas, distensión abdominal y signos radiológicos de obstrucción intestinal o edema de la mucosa. Es la causa más común de mortalidad en pacientes con enfermedad de Hirschsprung (EH). Puede ocurrir en cualquier etapa de la enfermedad, con una incidencia media del 25 por ciento. La fisiopatología es poco conocida. Etiologías potenciales son la obstrucción mecánica, infecciones, barrera mucosa defectuosa, y un sistema inmunológico deficitario. Microscópicamente la EAEH se caracteriza por criptitis y abscesos en las criptas, seguida de ulceración de la mucosa progresiva conduciendo eventualmente a la necrosis transmural. Los pilares del tratamiento son la reanimación con fluidos, la descompresión del intestino y la terapia antibiótica. A veces se requiere de una estoma cuando estas medidas han fracasado. La mayoría de los pacientes con EAEH siguen teniendo trastornos de la función intestinal muchos años después de la cirugía para la enfermedad de Hirschsprung. Investigaciones en la fisiopatología de la EAEH pueden conducir a medidas preventivas y mejores modalidades de tratamiento.
Hirschsprungs-associated enterocolitis(HAEC) is defined as the presence of diarrhea, explosive stools, abdominal distension and radiologic evidence of bowel obstruction or mucosal edema. HAEC is the most common cause of mortality in patients with Hirschsprungs disease. This condition may occur in any stage of the Hirschsprungs disease, with a mean incidence of 25 percent. The pathophysiology is poorly understood. Potential etiologies are mechanical obstruction, infections, defective mucosal barrier, and impaired immunological system. Microscopically HAEC is characterized by cryptitis and crypt abscesses followed by progressive mucosal ulceration eventually leading to transmural necrosis. The mainstays of treatment are fluid resuscitation, decompression of the bowel and antibiotic therapy. Sometimes a stoma is required when such measures have failed. The majority of patients with HAEC continue to have disturbances of bowel function many years after surgery for Hirschsprungs disease. Researchs into the pathophysiology of HAEC can lead to better preventative measures and treatment modalities.
Subject(s)
Humans , Hirschsprung Disease/complications , Hirschsprung Disease/physiopathology , Enterocolitis/complications , Enterocolitis/physiopathology , Hirschsprung Disease/diagnosis , Hirschsprung Disease/pathology , Hirschsprung Disease/therapy , Enterocolitis/diagnosis , Enterocolitis/pathology , Enterocolitis/therapy , Risk Factors , MucinsABSTRACT
Os autores destacam a importância da constipação intestinal, por sua alta incidência e tendência à cronificação, apresentando inicialmente sua conceituação e, a seguir, a definição dos distúrbios funcionais da defecação, os fatores etiológicos, apresentação clínica, diagnóstico diferencial e tratamento.Citam, igualmente, o papel da manometria anorretal na investigação do doença de Hirschsprung e a relação entre constipação e alergia ao leite de vaca. Finalmente, analisam as inter-relações entre constipação, prebióticos e probióticos.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Constipation/diagnosis , Constipation/etiology , Constipation/therapy , Hirschsprung Disease/diagnosis , Hirschsprung Disease/therapy , Digestive System Abnormalities/diagnosis , Digestive System Abnormalities/therapyABSTRACT
Se presenta el complicado curso clínico de una lactante, sus dificultades diagnósticas iniciales, las maniobras terapéuticas subsecuentes y la resolución satisfactoria con un adecuado seguimiento.
The complicated clinical course of an infant is presented, including its initial diagnostic difficulties, subsequent therapeutic management and satisfactory results with long term follow up.
Subject(s)
Humans , Female , Infant , Colostomy , Hirschsprung Disease/diagnosis , Hirschsprung Disease/therapySubject(s)
Humans , Infant, Newborn , Infant , Meckel Diverticulum/diagnosis , Meckel Diverticulum/therapy , Hirschsprung Disease/diagnosis , Hirschsprung Disease/therapy , Pyloric Stenosis/diagnosis , Pyloric Stenosis/therapy , Intussusception/diagnosis , Intussusception/therapy , Abdomen, Acute/etiology , Meckel Diverticulum/complications , Hirschsprung Disease/complications , Pyloric Stenosis/complications , Intussusception/complicationsABSTRACT
Under the specific environmental circumstances of a normal developing country, a surgical procedure can save patients born with Total Colonic Aganglionosis. This new one-stage procedure comprises preservation of the ileocaecal valve, partial colon resection and a long ileum patch of the remaining colon and rectum. Normal stool habits were established due to enhanced absorption function of the remaining colon. Normal growth etc. were also noted due to adequate transit time of nutrients in the small intestine. Long-term enterostomy is difficult to manage in any depressed economy and the little ones succumb easily to malabsorption syndrome. So far two patients have benefited from this procedure. One is now 8 years, while the other is 4 years old, 6 years, and over 2 years respectively after definitive surgery
Subject(s)
Elective Surgical Procedures , Hirschsprung Disease/surgery , Hirschsprung Disease/therapy , Surgical Procedures, OperativeABSTRACT
La técnica de Duhamel para el tratamiento quirúrgico de la enfermedad de Hirschsprung ha sido objeto de importantes modificaciones en el transcurso del tiempo. La introducción de la sutura mecánica en los últimos años ofrece algunas ventajas añadidas a la técnica original. En el servicio de pediatría quirúrgica del Hospital Universitario de Caracas se han realizado, en el lapso comprendido entre 1990 y 1992, trece descensos abdómino-perineales para el tratamiento quirúrgico de la enfermedad de Hirschsprung, utilizando como técnica quirúrgica, la operación de Duhamel modificada, realizada con suturas mecánicas PLC de la casa EThicon. Este procedimiento fue practicado en pacientes cuyas edades estaban comprendidas entre 4 meses y 30 años de edad, seis pacientes del sexo femenino y siete masculinos. En el seguimiento post-operatorio hemos observado resultados satisfactorios, ya que se han detectado complicaciones del procedimiento salvo un caso que presentó estenosis rectal debido a problemas en el momento de colocar las pinzas
Subject(s)
Infant , Child, Preschool , Child , Adolescent , Adult , Humans , Male , Female , Colorectal Surgery/trends , Hirschsprung Disease/surgery , Hirschsprung Disease/therapy , Surgical Procedures, OperativeABSTRACT
En la enfermedad de Hirschsprung existe ya una pauta de tratamiento de comprobada eficacia, que consiste en descender hasta el ano el extremo gangliónoco más distal del colon. A pesar de lo anterior, en algunos pacientes con variantes problemáticas de la enfermedad, principalmente deficiencias en la anatomía del colon distal, no es factible realizarles el procedimiento descrito. Se presentan dos casos en los que la ausencia de un colon izquierdo adecuado motivó un cambio en la estrategia quirúrgica, y un segmento de íleon distal aislado, vascularizado y properistáltico fue interpuesto entre el colon y el ano con la técnica descrita por Dohamel. Ninguno de los dos pacientes tuvo complicaciones posoperatorias, y una vez restaurado el tránsito intestinal, la función colo-rectal se catalogó como satisfatoria. La interposición de un asa ileal puede ser una buena alternativa en el tratamiento de casos complicados de enfermedades de Hirschsprung
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Colostomy , Colon/surgery , Hirschsprung Disease/therapy , LaparotomyABSTRACT
La técnica de Dahamel para el tratamiento quirúrgico de la enfermedad de Hirschsprung ha sufrido importantes modificaciones con el transcurso del tiempo. la introducción de la sutura mecánica en los últimos años ofrece algunas ventajas añadidas a la técnica original. En el servicio de pediatría quirúrgica del Hospital Universitario de Caracas se ha realzado en el lapso comprendido entre 1990 y el primer trimestre de 1992 diez descensos abdomino perineales. En el seguimiento post-operatorio de estos pacientes hemos observado resultados satisfactorios, ya que no se han detectado complicaciones del procedimiento,salvo un caso que presentó estenosis rectal debido a problemas en el momento de colocar las pinzas
Subject(s)
Hirschsprung Disease/surgery , Hirschsprung Disease/therapy , Surgical Procedures, Operative/methodsABSTRACT
Se estudiaron 29 casos clínicos de megacolon congénito en Nariño en un período de 1984 a 1988 en cinco instituciones médicas, encontrándose una incidencia de 1-5.000 nacidos vivos, con una relación de 4:1 con predominio del sexo masculino, sin asociaciones con anomalías congénitas, un compromiso predominante de segmentos ultracorto y corto de 96.5 por ciento; se hizo el diagnóstico con base en la clínica colon por enema y biopsia clásica; para segmento ultracorto se empleó como técnica de elección la rectomiectomía Lynn y para segmento corto y largo la técnica de Soave y de Ikeda; obteniéndose mejores resultados y una marcada disminución de complicaciones con el último método
Subject(s)
Humans , Hirschsprung Disease , Hirschsprung Disease/diagnosis , Hirschsprung Disease/epidemiology , Hirschsprung Disease/therapy , IncidenceABSTRACT
Foram estudados 12 recém-nascidos, admitidos na Unidade de Neonatologia do Hospital Infantil Joana de Gusmäo (HIJG) Florianópolis - SC, no período de janeiro de l980 e agosto de l985, com diagnóstico de Megacólon Congênito. Dez, eram do sexo masculino, dois do sexo feminino e o peso médio foi de 3.290g. Alteraçöes na eliminaçäo de mecônio (91,6%), distensäo abdominal (75%) e vômitos (58,3%) foram os sintomas principais. Os meios diagnósticos utilizados foram o RX simples de abdome (12 casos), o clister-opaco (11 casos) e a biópsia retal (3 casos). Todos os pacientes foram submetidos a Colostomia. Houve 2 óbitos por septicemia