Hallazgos radiológicos en pseudoobstrucción intestinal por lupus eritematoso sistémico. Reporte de un caso / Radiological findings in pseudo-intestinal obstruction due to systemic lupus erythematosus. A case report

Resumen La pseudoobstrucción intestinal (POI) es una complicación poco frecuente de lupus eritematoso sistémico (LES), y su diagnóstico suele ser un reto, especialmente al diferenciarla de otras enfermedades abdominales. El cuadro clínico de la enfermedad se debe al daño muscular, vascular y nervioso de las paredes intestinales, lo que genera hipomotilidad y datos de obstrucción. En este artículo, se reporta el caso de una mujer de 19 años con dolor abdominal, distensión abdominal y vómito, con antecedentes de trombosis de vena cava inferior y LES. La tomografía computada (TC) de abdomen mostró signos de distensión intestinal, POI, ascitis y ureterohidronefrosis derecha, lo que descartó la obstrucción evitándose una intervención quirúrgica. El manejo fue con corticoesteroides. La evolución fue buena y se decidió el egreso de la paciente. Después de 12 meses no se reportaron complicaciones. La POI por LES es de difícil diagnóstico, pero la identificación y correlación de la semiología del caso con los datos inmunológicos y de imagenología, fue decisiva en el diagnóstico diferencial con otras patologías abdominales, como la obstrucción intestinal.

Abstract The pseudo-intestinal obstruction (PIO) is an uncommon complication due to systemic lupus erythematosus (SLE) and its diagnosis can be challenging, especially when trying to differentiate it from other abdominal diseases. Clinical manifestations are caused by muscular, vascular and nervous damage of the intestinal walls, causing hypomotility and symptoms of obstruction. This paper reports the case of a 19 year-old woman who first manifested abdominal pain, abdominal distention and vomits, plus she had a history of inferior vena cava thrombosis and lupus. An abdominal computed tomography (CT) scan showed intestinal distention, PIO, ascites and right hydronephrosis, therefore the diagnosis of a real obstruction was rejected, and surgical intervention was avoided. The patient was treated with steroids. The response to the treatment was good, the patient went into remission, and was discharged from the hospital. 12 months later, no complications were reported. SLE with PIO is hard to detect, but the proper identification and correlation of the semiology of the case with the immunological and imaging data was decisive in the differential diagnosis with other abdominal diseases, such as the intestinal obstruction.

Síndrome de Ogilvie (pseudo-obstrução intestinal aguda) - Relato de caso / Ogilvie's Syndrome (Acute pseudo-intestinal obstruction) - A case report

RACIONAL: A síndrome de Ogilvie é condição clínica com sinais, sintomas e aparência radiológica de dilatação acentuada do cólon sem causa mecânica e pode complicar com rompimento da parede do cólon e sepse abdominal. O tratamento na maioria das vezes é cirúrgico. RELATO DO CASO: Paciente feminina, 49 anos, internada com queixa de dor abdominal e diarréia e apresentando-se confusa, desidratada, taquicárdica, dispnéica, temperatura de 38ºC, abdômen distendido, ausência de ruídos hidro-aéreos e toque retal com fezes pastosas. Estudo radiográfico mostrou padrão de pseudo-obstrução intestinal. A paciente evoluiu com parada de eliminação de gases e fezes e sinais de abdômen agudo infeccioso. Foi submetida à laparotomia com achado de ceco e transverso bastante dilatados e sem sinal de obstrução mecânica. Realizada colectomia subtotal com fechamento do coto distal e ileostomia terminal. CONCLUSÃO: Pensar nessa possibilidade diagnóstica e agir mais rapidamente é a única possibilidade de diminuir a morbimortalidade desses pacientes.

BACKGROUND: The Ogilvie's Syndrome is a clinical condition with signals, symptoms and radiological appearance of large bowel swell without mechanical cause. This obstruction can complicate with disruption of the bowel and consequent evolution of abdominal sepse. The treatment is typically surgical. AIM: The aim of this work is report a case of Ogilvie's Syndrome. CASE REPORT: Feminine patient, 49 years-old, interned with a history of abdominal pain and diarrhea and presenting dehydratation, tachycardia , dyspnea, mental confusion, 38ºC of temperature, distended abdomen, absence of hydro-aerial noises and rectal touch with pasty excrements. The x-ray showed a standard of pseudo-intestinal obstruction. The patient evolved with stop of elimination of farts and excrements and signals of infectious acute abdomen. The laparotomy showed cecum and transverse very swelled without signal of mechanical obstruction. The treatment was a subtotal colectomy with closing of the rectal stump and terminal ileostomy. CONCLUSION: This is rare syndrome and cases like this must be described for a faster diagnostic and adequate treatment, reducing the morbimortality of these patients.

Unusual occurrence of intestinal pseudo obstruction in a patient with maternally inherited diabetes and deafness (MIDD) and favorable outcome with coenzyme Q10 / Pseudo-obstrução intestinal em paciente com diabetes mitocondrial que apresentou boa resposta ao tratamento adjunto com coenzima Q10

Maternally inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. This subtype of diabetes is characterized by maternal transmission, young age at onset and bilateral hearing impairment. Besides diabetes and deafness, the main diagnostic features, a wide range of multisystemic symptoms may be associated with the A3243G mutation. Organs that are most metabolically active, such as muscles, myocardium, retina, cochlea, kidney and brain are frequently affected. Gastrointestinal tract symptoms are also common in patients with mitochondrial disease and constipation and diarrhea are the most frequent manifestations. However, there are few prior reports of intestinal pseudo obstruction in MIDD patients. Here we report the case of a patient with MIDD associated with the mtDNA A3243G mutation who developed chronic intestinal pseudo obstruction, and the introduction of Coenzyme Q10 as adjunctive therapy led to a solution of the pseudo obstruction.

Diabetes mitocondrial ou diabetes e surdez de herança maternal (MIDD, acrônimo de maternally inherited diabetes and deafness) é freqüentemente associado à mutação mitocondrial A3243G. Esse subtipo de diabetes é caracterizado por transmissão materna, disacusia neuro-sensorial bilateral e idade precoce de aparecimento. Além do diabetes e da surdez, principais características diagnósticas, outros sintomas em diferentes órgãos podem também associar-se à mutação A3243G. Os órgãos que são metabolicamente mais ativos, tais como músculos, miocárdio, retina, cóclea, rim e cérebro, são freqüentemente afetados. Sintomas do trato gastrintestinal também são comuns em pacientes com doença mitocondrial, sendo diarréia e obstipação as manifestações mais freqüentes. Entretanto, há poucos relatos de pseudo-obstrução intestinal em portadores de diabetes mitocondrial. Este relato descreve o caso de uma paciente com diabetes mitocondrial que apresentou pseudo-obstrução intestinal e que com a introdução de coenzima Q10, como terapia adjunta, teve resolução o quadro.