RESUMO
Cicatricial pemphigoid (CP) is a rare autoimmune blistering disorder, which affects the skin, eyes, and mucous membranes. The annual incidence of CP is 1/100,000. Esophageal involvement is rare and usually occurs in disseminated disease. Esophageal disease presents with dysphagia and weight loss and can be difficult to treat. We present a case of a 65-year-old woman with esophageal CP whose symptoms improved with esophageal dilatation and rituximab infusions.
RESUMO
Numerous pulmonary conditions, such as aspiration pneumonia and acute respiratory distress syndrome (ARDS), may result from aspiration of gastric or oropharyngeal contents passing into the lower respiratory tract. ARDS is a type of diffuse lung injury that is distinguished by the abrupt onset of extensive pulmonary inflammation accompanied by the failure of multiple organ systems. Systemic sclerosis is an uncommon connective tissue disorder that presents with skin thickening, the etiology of which remains unknown. Esophageal luminal dilatation is observed in the distal third of the esophagus in most cases of systemic sclerosis. This dilatation is primarily attributed to the greater abundance of smooth muscle fibers in this area. Here, we present the case of a 70-year-old female patient who was diagnosed clinically with diffuse systemic sclerosis and fulfilled the 2013 European League Against Rheumatism/American College of Rheumatology classification criteria. She had esophageal dilatation, with an esophageal luminal diameter measured at the upper, middle, and lower thoracic esophagus of 2.5 cm, 2.5 cm, and 3.5 cm, respectively. The patient was admitted to the intensive care unit (ICU) due to ARDS from aspiration pneumonia. Our patient's complicated condition at the time of ICU admission with ARDS secondary to aspiration pneumonia was primarily due to esophageal dilatation and reflux. Aggressive anti-reflux pharmacotherapy and bed elevation may be beneficial in preventing pulmonary injury caused by aspiration. Esophageal complications are common in such patients and can have a substantial impact on the prognosis and quality of life. Regular medical attention is necessary to identify and manage any potential issues.
RESUMO
La acalasia es una patología de baja frecuencia que afecta la motilidad esofágica producto de la denervación mientérica del esófago y también, en gran proporción de casos, de estómago. Sus causas permanecen aún poco esclarecidas y su diagnóstico sigue siendo tardío, reportándose un desfase de al menos 5 años desde el inicio de la sintomatología, confundiéndose muchas veces con patologías como la enfermedad por reflujo gastroesofágico, entre otras. Es por ello, que requiere un alto índice de sospecha y un estudio acabado, siendo la manometría esofágica el estándar de oro. El tratamiento no es curativo en la actualidad, y está centrado en el alivio de los síntomas. Las alternativas quirúrgicas que se disponen son, la miotomía de Heller, dilatación endoscópica, y la más reciente miotomía endoscópica peroral (POEM). El tratamiento médico no ha demostrado buenos resultados y hoy en día presenta restringidas indicaciones.
Achalasia is a rare disease that affects esophageal motility as result of myenteric denervation of the esophagus and in a large proportion of cases, stomach. Its causes remain still poorly elucidated and its diagnosis remains late, reporting a delay of at least 5 years from the onset of symptoms, often confused with conditions such as gastroesophageal reflux disease, among others. Therefore, it requires a high index of suspicion and a comprehensive study, being esophageal manometry the gold standard. There is no curative treatment today and is focused on the relief of symptoms. The surgical available options are, Heller myotomy, endoscopic dilation, and the most recent peroral endoscopic myotomy (POEM). Medical treatment has not proved successful and today has restricted indications.
Assuntos
Humanos , Acalasia Esofágica/cirurgia , Acalasia Esofágica/diagnóstico , Esofagoscopia/métodos , Manometria , Acalasia Esofágica/etiologia , Acalasia Esofágica/fisiopatologia , Dilatação , Índice de Gravidade de DoençaRESUMO
La dilatación precoz en esofagitis caústica no está bien establecida. Objetivo: Evaluar el beneficio de la dilatación precoz, en la evolución y complicaciones de esofagitis caústicas grado II y III. Pacientes y Métodos: Estudio prospectivo de 32 niños, grupo A (dilatación precoz) y B (dilatación tardía). Se utilizo el índice de dilatación periódica para evaluar beneficio de la dilatación precoz. Resultados: edad promedio 2,3 años; 13 (40,62%) hembras y 19 (59,38%) varones; 21/32 (65,62%) desarrollaron estenosis esofágica, 6/15 (40,00%) grupo A, 15/17 (88,23%) grupo B (p=0,0041). Estenosis simples en 12/21 (57,14%), complejas 9/21 (42,85%), recurrentes 2/6 y refractaria 2/6, grupo A; 6/15 recurrente y 5/15 refractarias, 1/15 perforación esofágica, grupo B. El promedio de sesiones de dilatación 17 vs 44,6 (p=0,0297) e índice de dilatación periódica de 3,04 vs 4,11 (p=0.0002) grupo A y B respectivamente. Conclusiones: la dilatación precoz en esofagitis caústica es segura y contribuye a disminuir el número de sesiones de dilatación y complicaciones. Se destaca, la importancia de la prevención de la ingesta accidental de cáusticos.
Early dilatation in caustic esophagitis is not well established. Objective: Evaluate the benefits of early dilatation in the evolution and complications of grade I and II caustic esophagitis in children. Patients and Methods: Prospective study of 32 children, group A (early dilatation) and B (late dilatation). The periodic dilatation index was used to evaluate the benefits of early dilatation. Results: Average age 2,3 years old; 13 (40,62%) female and 19 (59,38%) male; 21/32 (65,62%) developed esophageal stenosis. 6/15 (40,00%) group A, 15/17 (88,23%) group B (p=0,0041). Simple stenosis in 12/21 (57,14%), complex 9/21 (42,85%), recurrent 2/6 and refractory 2/6, group A; 6/15 recurrent and 5/15 refractory, 1/15 esophageal perforation, group B. The average of dilatation sessions was 17 vs. 44,6 (p=0,0297) and periodic dilatation index was 3,04 vs. 4,11 (p=0.0002) group A and B respectively. Conclusions: early dilatation in caustic esophagitis is safe and contributes to decrease the number of dilatation sessions and complications.
Assuntos
Humanos , Masculino , Feminino , Criança , Dilatação Gástrica/complicações , Esofagite/diagnóstico , Esofagite/patologia , Estenose Esofágica/complicações , Gastroenterologia , PediatriaRESUMO
La ingestión de cáusticos en la edad pediátrica es un problema grave al producir una lesión progresiva y devastadora en el esófago y el estómago. Estudio retrospectivo y descriptivo de 421 pacientes en edades comprendidas de 2 meses a 14 años quienes ingirieron cáusticos entre 1.992 y 2.008, evaluados en el Hospital Universitario de Maracaibo. El 60,09% eran pre-escolares y el 62,23% eran del sexo masculino. La sustancia tipo álcali fue ingerida en el 90,73% de los casos y los cáusticos se encontraban almacenados en su envase original en 3,8% y trasegados en el 96,2% de los casos. La ingestión fue de origen accidental en el 99,53%. Todos los pacientes manifestaron alguna sintomatología como lesiones orofaríngeas, vómitos y sialorrea. La evaluación endoscópica se realizó en el 89,31% de los casos y se evidenció lesiones en el tracto gastrointestinal en 60,63%. Los pacientes con Esofagitis Grado III y estenosis esofágica fueron incluidos en el programa de dilatación, con una buena evolución en el 35,91% de ellos. La ingestión de cáusticos constituye un problema frecuente y serio, en su mayoría de origen accidental, afecta principalmente a menores de 6 años y el álcali es el principal agente involucrado. La Endoscopia Digestiva es el estudio por excelencia para evaluar estos pacientes. Las dilataciones esofágicas son moderadamente exitosas. El mejor tratamiento es la prevención.
The caustic ingestion in the pediatric population is a serious problem because produce a progressive and devastating injury to the esophagus and stomach. Retrospective and descriptive study of 421 patients with age from 2 months to 14 years who ingested caustic between 1.992 to 2.008 evaluated at the Hospital Universitario de Maracaibo. The 60.09% were pre-school and 62.23% were male. The substance type alkali was swallowed by 90.73% of cases and the caustics were stored in its original packaging by 3.8% and decanted in 96.2% of cases. Intake was accidental source in 99.53%. All patients showed some symptoms as oropharyngeal lesions, vomiting and drooling. Endoscopy evaluation was performed by 89.31% of cases and revealed gastrointestinal tract injuries in 60.63%. Patients with grade 3 Esophagitis and esophageal stricture were admitted in the dilatation program with a good success in 35.91% of them. The caustic ingestion is a common and serious problem, mostly from accidental origin, mainly affects children under 6 years and the alkali is the principal agent involved. The Esophagoscopy is the ideal mean for evaluate these patients. The esophageal dilatations are moderately successful. The best treatment is the prevention.