Megacolon de etiología infecciosa poco frecuente en nuestro país: enfermedad de Chagas / No disponible

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Megacolon with infectious etiology that is infrequent in our country: Chagas disease.

We present the case of a 37-year-old male with constipation refractory to medical treatment, in the context of megacolon due to Chagas disease. The entire gastrointestinal tract may be affected but the digestive form is characterized by megaesophagus and megacolon. There is altered peristalsis due to the destruction of the neurons of the enteric nervous system caused by the parasite. Although the mortality rate is low, the disease can have a considerable impact upon quality of life. Chagas disease is poorly recognized and undertreated by healthcare providers in non-endemic regions.

Megacolon en la enfermedad inflamatoria intestinal: respuesta a infliximab / No disponible

El megacolon es una complicación grave de la enfermedad inflamatoria intestinal que con frecuencia requiere colectomía. Infliximab sería una alternativa terapéutica cuando fracasa el tratamiento convencional, antes de la cirugía. En la actualidad, su uso se basa en la publicación de casos aislados. Presentamos nuestra serie de 12 pacientes con megacolon (cinco con signos de toxicidad sistémica) tratados con infliximab. El 75% de los pacientes evitaron la colectomía durante el episodio agudo tras la instauración precoz del tratamiento con infliximab (2,45 días desde el diagnóstico del megacolon), apreciando un mayor riesgo de cirugía entre los pacientes con colitis ulcerosa y criterios de toxicidad. Pese a mantener infliximab a largo plazo, dos pacientes más requirieron cirugía en el seguimiento. Ningún paciente sufrió efectos adversos relevantes en relación con el tratamiento ni complicaciones posquirúrgicas significativas

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A case of severe megacolon due to acquired isolated hypoganglionosis after low anterior resection for lower rectal cancer.

Acquired isolated hypoganglionosis is a rare intestinal neurological disease, which presents in adulthood with the clinical symptoms of chronic constipation. A 39-year-old man underwent laparoscopic low anterior resection and covering ileostomy for locally advanced-rectal cancer. A 6-month course of postoperative adjuvant chemotherapy was completed, followed by closure of the ileostoma. After the closure, he developed severe colitis which required 1-month of hospitalization. Mucosal erosions and pseudo-membrane formation were evident on colonoscopy and severe mucosal damage characterized by infiltration of inflammatory cells and crypt degeneration were pathologically confirmed. Even after the remission of the colitis, he suffered from severe constipation and distention. At 4 years after the stoma closure, he decided to undergo laparoscopic total colectomy. Histopathologically, the nerve fibers and ganglion cells became gradually scarcer from the non-dilated to dilated regions. Immunohistochemical staining examination confirmed that the ganglion cells gradually decreased and became degenerated from the normal to dilated region, thereby arriving at the final diagnosis of isolated hypoganglionosis. The patient recovered without any complications and there has been no evidence of any relapse of the symptoms. We present a case of acquired isolated hypoganglionosis-related megacolon, which required laparoscopic total colectomy, due to severe enterocolitis following stoma closure.

Colonic Manifestations and Complications Are Relatively Under-Reported in Systemic Sclerosis: A Systematic Review.

OBJECTIVES: Although systemic sclerosis (SSc) is known to affect the gastrointestinal (GI) tract, most of the literature focuses on esophageal, small intestinal, or anorectal manifestations. There have been no reviews focused on large bowel SSc complications in over 30 years. The aim of this study is to perform a systematic review of colonic manifestations and complications of SSc. METHODS: An experienced librarian conducted a search of databases, including English and Spanish articles. The search used keywords including "systemic sclerosis," "scleroderma," and "colon." A systematic review was performed using Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines. Case reports/series were screened for validity by adapting from criteria published elsewhere. RESULTS: Of 1,890 articles, 74 met selection criteria. Fifty-nine of the 77 articles were case reports/series. The most common article topics on colonic SSc complications were constipation/dysmotility (15), colonic volvulus (8), inflammatory bowel disease (7), microscopic colitis (6), megacolon (6), and telangiectasia (6). Colonic manifestations constituted 24% of articles on GI complications of SSc. There were a total of 85 cases (84% women, with a median age of onset of colon complication of 52 years). Limited cutaneous SSc phenotype (65.6%) was more common than diffuse (26.2%). Patients frequently had poor outcomes with high mortality related to colonic complications (27%). Recent studies explore contemporary topics such as the microbiome in SSc and prucalopride for chronic constipation in SSc. DISCUSSION: Colonic complications comprise a large proportion of the published reports on GI symptoms afflicting patients with SSc and require raised diagnostic suspicion and deliberate action to avoid potentially serious complications including death.

Lymphocytic ganglionitis leading to megacolon in lymphocyte-rich glioblastoma.

T-cell immune attack of cancer cells underlies the efficacy of immune checkpoint inhibitors in many cancer subtypes, but is not yet well established in the primary brain cancer glioblastoma. Immune checkpoint inhibitor treatments that disinhibit the immune system to enhance immune clearance of cancer have in rare cases resulted in T-cell attack of peripheral ganglia causing lymphocytic ganglionitis. In glioblastoma, lymphocytic ganglionitis has not been reported and checkpoint inhibitors are not routinely used. Here we report a case of glioblastoma not treated with checkpoint inhibitors in which the primary tumor and peripheral ganglia of the celiac and sympathetic chains, as well as myenteric plexus, are infiltrated by CD8+ cytotoxic T-cells. In addition to the marked lymphocytic infiltrates, this case is also notable for an unusually long survival (8 years) after diagnosis with glioblastoma, but an ultimately fatal outcome due to ileus. The findings suggest T-cell immune attack of glioblastoma may prolong survival, but also suggest T-cell autoimmune diseases such as lymphocytic ganglionitis could become a risk with the future use of immune-targeted therapies for glioblastoma.

Pediatric sigmoid volvulus due to Chagas disease.

Exposure and infections by Trypanosoma cruzi are the fourth cause of loss of potential life years between parasitic and infectious diseases. We describe the case of a 11-year-old patient with intestinal occlusion, surgically treated with intestinal volvulus, the surgical specimen is sent to histopathology reporting Chagasic megacolon. The age range of presentation is a challenge in the absence of nonspecific symptoms. There is no pediatric statistical data that define trypanosomiasis in a latent or chronic state and will be diagnosed in adult stages due to the physiopathological alterations that they will present.

La exposición y las infecciones por Trypanosoma cruzi ocupan el cuarto lugar entre las causas de pérdida de años de vida potenciales por enfermedades parasitarias e infecciosas. Se describe el caso de un niño de 11 años, con cuadro de oclusión intestinal, intervenido quirúrgicamente con datos de vólvulo intestinal. La pieza quirúrgica se envió a histopatología, que reportó megacolon chagásico. El rango de edad de presentación es un reto ante la falta de síntomas inespecíficos. No se cuenta con datos estadísticos pediátricos que definan la tripanosomiasis en estado latente o crónico, y estos niños serán diagnosticados en la etapa adulta por las alteraciones fisiopatológicas que presentarán.

Chronic Megacolon Presenting in Adolescents or Adults: Clinical Manifestations, Diagnosis, and Genetic Associations.

OBJECTIVE: Chronic megacolon is rarely encountered in clinical practice beyond infancy or early childhood. Most cases are sporadic, and some are familial megacolon and present during adolescence or adulthood. There is a need for diagnostic criteria and identifying genetic variants reported in non-Hirschsprung's megacolon. METHODS: PubMed search was conducted using specific key words. RESULTS: This article reviews the clinical manifestations, current diagnostic criteria, and intraluminal measurements of colonic compliance to confirm the diagnosis when the radiological imaging is not conclusive. Normal ranges of colonic compliance at 20, 30, and 44 mmHg distension are provided. The diverse genetic associations with chronic acquired megacolon beyond childhood are reviewed, including the potential association of SEMA3F gene in a family with megacolon. CONCLUSIONS: Measuring colonic compliance could be standardized and simplified by measuring volume at 20, 30, and 44 mmHg distension to identify megacolon when radiology is inconclusive. Diverse genetic associations with chronic acquired megacolon beyond childhood have been identified.

Chagas disease: An underrecognized diagnosis.

Chagas disease is a parasite infection primarily transmitted to humans via the bite of triatomine insect vectors. Up to 8 million people are estimated to be infected with Chagas disease in the Americas. Patients who do not receive treatment can develop severe cardiac debility, gastrointestinal organ dysfunction, and may die. The changing demographics of the United States, a consequence of changing immigration patterns, means that healthcare providers are more likely to encounter patients with Chagas disease, and must understand its cause, pathogenesis, diagnosis, and treatment.

[Rehbein procedure for secondary megacolon in children]. / Operatsiia Rebeina pri vtorichnom megakolon u detei.

AIM: To evaluate the results of Rehbein procedure with extraperitoneal colorectal anastomosis combined with complex conservative treatment in children with secondary megacolon due to chronic constipation. MATERIAL AND METHODS: We carried out surgical interventions in 78 children aged 7-12 years with chronic colostasis resistant to conservative treatment. All patients underwent clinical, laboratory and instrumental examination. All patients were divided into 3 groups depending on type of surgery: group I - colon resection followed by intraperitoneal colorectal anastomosis (28 patients), group II - open Rehbein procedure (29 patients), group III - laparoscopic Rehbein procedure (21 patients). RESULTS: Absence of dyspeptic disorders and enterocolitis in remote postoperative period in patients receiving comprehensive treatment enhanced with new additions is the cause of improved outcomes. Type of surgery should be individualized in view of anatomical colon changes and secondary pathological conditions. Some pathological symptoms observed at preoperative period may be recurrent in long-term postoperative period due to wrong selection of surgery. The risk of recurrent chronic constipation is high after colon resection followed by intraperitoneal colorectal anastomosis. CONCLUSION: Rehbein procedure may be preferred for management of secondary megarectum and megasigma. Laparoscopic technique reduces surgical trauma and requires less postoperative analgesia.

Megacolon andino y vólvulo del sigmoides de la altura: Presentación de 418 casos entre 2008 - 2012 en el hospital C. Monge - Puno, Perú / Andean megacolon and sigmoid volvulus in the high altitude: Presentation of 418 cases between 2008 - 2012 at C. Monge Hospital, Puno, Peru

Objetivo: El presente estudio describe el manejo médico y quirúrgico del vólvulo de sigmoides debido a dolicomegacolon andino en un hospital a una altitud mayor a 3000 msnm. Material y métodos: Estudio descriptivo, observacional, transversal de 418 pacientes con diagnóstico de vólvulo de sigmoides; admitidos inicialmente por cuadros de obstrucción intestinal, en el Hospital de Juliaca Carlos Monge - Puno, Perú, durante el periodo 2008-2012. Los datos fueron procesados a través del programa SPSS versión 21. Resultados: Se registraron 418 pacientes, la media de edad fue de 60 años, rango 18-89 años, con una proporción hombre/mujer de 3,5/1. El manejo no quirúrgico se hizo en 64 (15,4%), el tratamiento empleado fue de enema salino 20 casos 31% y sonda rectal 44 (69%), se presentó recurrencia en 27 pacientes (45%), los cuales tuvieron cirugía con resección anastomosis primaria, de estos la mortalidad correspondió a 8 pacientes (30%). De los 354 pacientes sometidos a manejo quirúrgico de emergencia 325 fueron sometidos a sigmoidectomia con anastomosis primaria (92%), mientras 29 tuvieron colostomía a lo Hartmann (8%), la morbilidad para ambos procedimientos fue de 52 casos (14,7%), la mortalidad para ambos procedimientos fue de 45 casos (12,7%). Conclusiones: El vólvulo sigmoides debido a megacolon andino tuvo una edad media de 60 años. El 15,4% tuvo manejo no quirúrgico, la tasa de recurrencia fue de 45%, mortalidad de 30%. El 84,7% tuvo manejo quirúrgico; el 92% tuvo resección anastomosis primaria y 8% colostomía a lo Hartmann, la morbilidad fue de 14,7% y la mortalidad de 12,7%.

Objective: The present study describes the medical and surgical management of sigmoid volvulus due to Andean dolicomegacolon in a hospital at an altitude above 3000 m. Material and methods: A descriptive, observational, crosssectional study of 418 patients diagnosed with sigmoid volvulus; Admitted initially due to intestinal obstruction, in the Hospital of Juliaca Carlos Monge. Puno-Perú, during the period 2008-2012. The data were processed through the SPSS software version 21. Results: A total of 418 patients were enrolled, the mean age was 60 years, range 18-89 years, and the male/female ratio was 3.5/1. Nonsurgical management was done in 64 (15.4%), the treatment used was saline enema 20 cases (31%) and rectal catheter 44 (69%), recurrence was present in 27 patients (45%), who had surgery with primary anastomosis resection, of which the mortality corresponded to 8 patients (30%). Of the 354 patients undergoing emergency surgical management, 325 were submitted to sigmoidectomy with primary anastomosis (92%), while 29 had Hartmann's colostomy (8%), the morbidity for both procedures was 52 cases (14.7%), Mortality for both procedures was 45 cases (12.7%). Conclusions: In patients with sigmoid volvulus due to Andean megacolon the mean age was 60 years. The 15.4% had non-surgical management, the recurrence rate was 45%, and mortality 30%. Patients with surgical management was 84.7%, from this group; 92% had primary anastomosis resection and 8% Hartmann colostomy, morbidity was 14.7% and mortality was 12.7%.

Dolor abdominal en un adolescente. ¿Qué no debemos olvidar? / Abdominal pain in a teenager. Things we must not forget

El dolor abdominal supone un motivo de consulta habitual en las urgencias pediátricas. Los cuadros oclusivos son una causa muy poco frecuente de dolor abdominal en niños, pero son graves y precisan un tratamiento urgente. Una sospecha clínica precoz puede evitar posibles complicaciones, como la perforación intestinal, la peritonitis y el shock séptico. Se presenta el caso de un niño de 14 años de edad que acude al servicio de urgencias a causa de dolor abdominal y estreñimiento de 7 días de evolución, asociados en las últimas 12 horas a vómitos persistentes. En la exploración física presentaba un aspecto general regular, una ligera distensión abdominal, dolor a la palpación en el hipogastrio y ausencia de heces en la ampolla rectal. Se realizó una radiografía de abdomen, que mostraba una imagen en «grano de café» compatible con un vólvulo del sigma. Se realizó una devolvulación por vía endoscópica, con lo que se logró la resolución de los síntomas (AU)

Abdominal pain is a common admission in paediatric emergencies. Intestinal obstruction is an unusual cause of abdominal pain in children but is a serious condition and an urgent treatment is needed. An early diagnose could avoid complications such as intestinal perforation, peritonitis and sepsis. We report a case of a 14 year old boy who was admitted with abdominal pain, constipation for 7 days and vomiting during the last 12 hours. Examination showed a boy clinically affected, with abdominal distension, hipogastric pain and absence of stool in the rectum. The abdominal X-ray exam showed a "coffee bean sign" and a sigmoid colon volvulus was suspected. Endoscopic decompression was performed with symptoms resolution (AU)

Idiopathic Megacolon: Report of 2 Deaths With Review of the Literature.

Abnormal dilation of the colon and rectum can develop from a range of disease processes. When encountered at autopsy, its contribution to death requires assessment and a thorough investigation of its origins. Elimination of known causes elicits a diagnosis of idiopathic megacolon. This entity is uncommonly encountered and presents with similar gross anatomic findings as Hirschsprung disease. Although death is infrequent, it most commonly results from disruption of the bowel wall and subsequent peritonitis. The authors report 2 rare deaths from idiopathic megacolon with retained integrity of the bowel wall. The first was a 9-year-old girl who was administered a laxative and subsequently died the following day. She expressed difficulty passing stool since birth with a marked decline at the age of 7 years. The second case was a 16-year-old adolescent girl with recent diarrhea who collapsed after showering. She, too, had a long history of chronic constipation. Years before death, her rectum and sigmoid colon were found to be dilated on x-ray for an unrelated event, but follow-up was never pursued. Cases such as these require a thorough review of the medical history and exclusion of established conditions, such as infectious, inflammatory, metabolic, and neurogenic origins.

Relation between mast cells concentration and serotonin expression in chagasic megacolon development.

Chagas' disease is still reaching about 10 million people in the world. In South America, one of the most severe forms of this disease is the megacolon, characterized by severe constipation, dilated sigmoid colon and rectum and severe malnutrition. Previous data suggested that mast cells and serotonin (5-hydroxytryptamine [5-HT]) expression could be involved in intestinal homeostasis control, avoiding the chagasic megacolon development. The aim at this study was to characterize the presence of mast cells and expression of serotonin in chagasic patients with and without megacolon and evaluate the relation between mast cells, serotonin and megacolon development. Our results demonstrated that patients without megacolon feature a large amount of serotonin and few mast cells, while patients with megacolon feature low serotonin expression and a lot of mast cells. We believe that serotonin may be involved in the inflammatory process control, triggered by mast cells, and the presence of this substance in large quantities of the intestine could represent a mechanism of megacolon prevention.

Death Caused by Hepatodiaphragmatic Interposition of the Colon.

Hepatodiaphragmatic interposition of the colon is a rare, usually asymptomatic, anomaly and is typically an incidental radiologic finding. There are few cases in the literature describing the symptomatic form of the condition, known as Chilaiditi syndrome. In some cases, it may be accompanied by various severe complications. If symptoms are present, usually conservative treatment is given. However, conservative treatment only addresses the symptoms but does not prevent their recurrence and possible complications. Our present report shows that this anomaly may not only cause symptoms, but may also progress and cause severe complications, in our case-megacolon leading to right heart failure and, ultimately, death. To date, however, there have been no literature reports of death caused by colonic interposition. Therefore, it is important to draw attention to the importance of this anomaly and its appropriate diagnosis and treatment to ensure the most favorable patient outcomes.

[Andean megacolon and sigmoid volvulus in the high altitude. Presentation of 418 cases between 2008 - 2012 at C. Monge Hospital, Puno, Peru]. / Megacolon andino y vólvulo del sigmoides de la altura. Presentación de 418 casos entre 2008 - 2012 en el hospital C. Monge Puno, Perú.

OBJECTIVE: The present study describes the medical and surgical management of sigmoid volvulus due to Andean dolicomegacolon in a hospital at an altitude above 3000 m. MATERIAL AND METHODS: A descriptive, observational, crosssectional study of 418 patients diagnosed with sigmoid volvulus; Admitted initially due to intestinal obstruction, in the Hospital of Juliaca Carlos Monge. Puno-Perú, during the period 2008-2012. The data were processed through the SPSS software version 21. RESULTS: A total of 418 patients were enrolled, the mean age was 60 years, range 18-89 years, and the male/female ratio was 3.5/1. Nonsurgical management was done in 64 (15.4%), the treatment used was saline enema 20 cases (31%) and rectal catheter 44 (69%), recurrence was present in 27 patients (45%), who had surgery with primary anastomosis resection, of which the mortality corresponded to 8 patients (30%). Of the 354 patients undergoing emergency surgical management, 325 were submitted to sigmoidectomy with primary anastomosis (92%), while 29 had Hartmann's colostomy (8%), the morbidity for both procedures was 52 cases (14.7%), Mortality for both procedures was 45 cases (12.7%). CONCLUSIONS: In patients with sigmoid volvulus due to Andean megacolon the mean age was 60 years. The 15.4% had non-surgical management, the recurrence rate was 45%, and mortality 30%. Patients with surgical management was 84.7%, from this group; 92% had primary anastomosis resection and 8% Hartmann colostomy, morbidity was 14.7% and mortality was 12.7%.