Transvenous Obliteration Procedure in the Management of Parastomal Variceal Bleeding: A Case Report.

Introduction: Parastomal variceal bleeding (PVB) is a recognized complication of ostomized patients in the presence of portal hypertension. However, since there are few reported cases, a therapeutic algorithm has not yet been established. Case Presentation: A 63-year-old man, who had undergone a definitive colostomy, recurrently presented to the emergency department hemorrhage of bright red blood from his colostomy bag, initially assumed to be caused by stoma trauma. Accordingly, temporary success on local approaches such as direct compression, silver nitrate application and suture ligation was achieved. However, bleeding recurred, requiring transfusion of red blood cell concentrate and hospitalization. The patient's evaluation showed chronic liver disease with massive collateral circulation, particularly at the colostomy site. After a PVB with associated hypovolemic shock, the patient was submitted to a balloon-occluded retrograde transvenous obliteration (BRTO) procedure which stopped the bleeding successfully. The patient was subsequently proposed for a transjugular intrahepatic portosystemic shunt (TIPS) conjugated with percutaneous transhepatic obliteration (PTO). After an initial refusal by the patient, a new episode of self-limited PVB resulted in execution of the procedure. Four months later, in a routine consultation, the patient presented with grade II hepatic encephalopathy, successfully treated with medical therapy. After a 9-month follow-up, he remained clinically well and without further episodes of PVB or other adverse effects. Discussion: This report highlights the importance of a high index of suspicion when dealing with significant stomal hemorrhage. Portal hypertension as an etiology of this entity may compel to a specific approach to prevent recurrence of bleeding, including conjugation of endovascular procedures. The authors pre-sent a case of PVB, initially submitted to a variety of treatment options including BRTO, which was successfully addressed with conjugated treatment of TIPS and PTO.

Introdução: A hemorragia de varizes periestomais é uma complicação conhecida de doentes ostomizados com hipertensão portal. Contudo, devido ao pequeno número de casos descritos, ainda não foi estabelecido um algoritmo terapêutico. Apresentação do caso: Homem, 63 anos, com antecedentes de colostomia definitiva, recorre ao Serviço de Urgência recorrentemente por sangue vivo no saco de colostomia. Inicialmente, presumindo-se trauma do estoma, foi submetido a tratamentos locais, como compressão, aplicação de nitrato de prata e sutura, com sucesso temporário. Contudo, houve recorrência da hemorragia, com necessidade de suporte transfusional e hospitalização. A avaliação do doente evidenciou doença hepática crónica com circulação colateral exuberante, predominantemente junto da colostomia. Devido a hemorragia com choque hipovolémico, foi submetido a obliteração transvenosa retrógrada ocluída por balão (BRTO). Posteriormente, foi proposto para shunt portossistémico transjugular intra-hepático (TIPS) conjugado com obliteração transhepática percutânea (PTO). Após recusa inicial do doente, ocorreu novo episódio de hemorragia autolimitado, tendo o doente concordado em realizar o procedimento. Quatro meses depois, em consulta, apresentava sinais de encefalopatia hepática grau II, tendo sido controlada eficazmente com tratamento médico. Após nove meses de seguimento, mantém-se sem novos episódios de hemorragia ou efeitos adversos dos procedimentos. Discussão: É necessário um alto índice de suspeição clínica ao abordar a hemorragia significativa do estoma. A hipertensão portal como etiologia exige uma abordagem específica para prevenir a recorrência da hemorragia, incluindo a conjugação de procedimentos endovasculares. Os autores apresentam o caso de um doente com hemorragia de varizes periestomais submetido inicialmente a vários tratamentos, incluindo BRTO e que foi tratado com sucesso com TIPS e PTO.

Successful Endoscopic Closure of Esophageal Perforation in Boerhaave Syndrome Using the Over-the-Scope Clip.

Boerhaave syndrome (BS) is a rare but potentially fatal condition. Although surgery is considered the standard treatment, endoscopic therapy has acquired an important role as a minimally invasive management approach. The authors describe 2 cases of middle-aged male patients, presenting with spontaneous esophageal perforation after severe straining and vomiting. In the first case, the patient presented with a bone impaction in the upper esophagus successfully removed by rigid esophagoscopy. After the procedure, a chest X-ray/cervicothoracic computerized tomography scan (CT) showed a left hydropneumothorax and pneumomediastinum with oral contrast leak at the lower esophagus. In the second case, the patient presented to the Emergency Department with severe chest pain after an episode of vomiting. The CT showed a massive pneumomediastinum, subcutaneous emphysema, and an oral contrast leak compatible with BS. The patient was initially submitted to surgical suture, but contrast extravasation persisted after 12 days. After multidisciplinary team discussion of both patients, an upper gastrointestinal endoscopy was performed, which revealed pericentimetric wall defects at the distal esophagus. These were successfully closed using an over-the-scope clip (OTSC). After at least a 9-month follow-up, patients have remained clinically well with no relapse. The authors highlight the severity of these clinical cases and the endoscopic option that proved to be decisive in addressing BS. The favorable outcomes suggest a role for the OTSC approach in closing spontaneous esophageal perforation both as first-line and as rescue therapy after a surgical failure.

A síndrome de Boerhaave (SB) é uma entidade rara, mas potencialmente fatal. Embora a cirurgia seja o tratamento padrão, o tratamento endoscópico tem adquirido um papel importante como opção minimamente invasiva. Os autores descrevem dois casos de doentes do sexo masculino de meia-idade, que apresentaram perfuração esofágica espontânea após esforço emético intenso e vómitos. No primeiro caso, o doente apresentou impactação de um osso no esófago superior, que foi removido com sucesso por esofagoscopia rígida. Após o procedimento, o doente realizou radiografia de tórax e tomografia computorizada (TC) cervico-torácica que evidenciou hidropneumotórax esquerdo e pneumomediastino com extravasamento de contraste oral ao nível do esófago inferior. No segundo caso, o doente apresentou-se no Serviço de Urgência com toracalgia intensa após episódio de vómito. A TC mostrou pneumomediastino exuberante, com enfisema subcutâneo e extravasamento de contraste oral compatível com SB. O doente foi inicialmente submetido a rafia cirúrgica, mas o esofagograma ao 12º dia mostrou persistência de extravasamento do contraste. Após discussão em reunião multidisciplinar, ambos os doentes realizaram endoscopia digestiva alta, com visualização de orifícios pericentimétricos no esófago distal, encerrados com sucesso com a aplicação de clip overthe- scope (OTSC). Após seguimento de pelo menos 9 meses, os doentes permaneceram clinicamente bem, sem evidência de recidiva. Os autores destacam a gravidade desses casos clínicos, bem como a opção endoscópica que se mostrou decisiva no tratamento da SB. Os resultados favoráveis sugerem um papel para a abordagem com OTSC no tratamento da perfuração esofágica espontânea, tanto como tratamento de primeira linha, como terapêutica de resgate após falência do tratamento cirúrgico.

Lymphogranuloma Venereum-Associated Proctitis Mimicking a Malignant Rectal Neoplasia: Searching for Diagnosis.

Background: Chlamydia trachomatis-lymphogranuloma venereum (LGV) is a sexually transmitted infection (STI) and an uncommon cause of proctitis. The diagnosis requires a high index of clinical suspicion, since the clinical, imaging, endoscopic, and histological findings can mimic multiple benign or malignant conditions like inflammatory bowel disease and rectal neoplasms. Case Presentation: We present the case of a 48-year-old Caucasian male with no significant previous medical history who was admitted due to the suspicion of a rectal neoplasia. He underwent an abdominopelvic computed tomography (CT) scan and pelvic magnetic resonance imaging (MRI) before admission due to complaints of anorectal pain, hematochezia, and constipation over the previous 2 weeks. The examination revealed a circumferential rectal wall thickening, infiltration of the perirectal fat and invasion of the mesorectal fascia, associated with perirectal fat lymphadenopathy. A radiological diagnosis of a rectal malignant neoplasia staged as T4N2MX was stated. Digital rectal examination identified a circumferential rectal tumor. Rectosigmoidoscopy showed an extensive and circumferential ulceration of the rectal mucosa, with elevated geographical borders, exudate, and aphthoid erosions at the proximal limit of the endoscopic mucosal ulceration. Biopsy specimens revealed acute ulcerative proctitis with lymphoplasmocytic inflammatory infiltrate but no evidence of dysplasia or malignancy. A STI screening was positive for HIV-1 (CD4+ 251/mm3; N = 700-1,100) and C. trachomatis, with an elevated IgA-specific antibody titer (52.000; N < 5.0), suggesting LGV disease. The diagnosis was confirmed by the identification of C. trachomatis DNA on rectal swab. Other infectious causes of acute proctitis were excluded. When faced with these results, the patient ended up mentioning that he had unprotected anal sex with men. He started treatment with doxycycline 100 mg twice a day for 21 days, with a drastic improvement. Rectosigmoidoscopy was repeated and showed clear signs of progressive resolution of the ulcerative proctitis. Discussion: LGV-associated proctitis, often undervalued, is a reemerging disease which should always be considered a benign cause of rectal mass, in order to avoid delay in diagnosis and development of complications. Diagnosis becomes more challenging in patients with unknown HIV status. A detailed clinical history, including sexual behaviors, is a vital step to achieve the final diagnosis.

Introdução: A infeção por Chlamydia trachomatis-linfogranuloma venéreo (LGV) é uma doença sexualmente transmissível (DST), sendo uma causa incomum de proctite. O diagnóstico exige um elevado grau de suspeição, dado que os achados clínicos, radiológicos, endoscópicos e histológicos podem mimetizar múltiplas condições benignas ou malignas, como a doença inflamatória intestinal e as neoplasias retais. Caso clínico: Apresentamos o caso de um homem de 48 anos, caucasiano, sem antecedentes relevantes, admitido por suspeita de neoplasia retal. Por queixas de dor anorretal, hematoquézias e obstipação com 2 semanas de evolução, realizou uma tomografia computadorizada abdominopélvica e uma ressonância magnética pélvica, que revelaram espessamento retal circunferencial, infiltração da gordura periretal e invasão da fáscia mesoretal, associados a linfadenopatias locais, sugestivos de malignidade retal (T4N2MX). O toque retal identificou uma tumoração retal circunferencial.A retosigmoidoscopia mostrou mucosa retal com ulceração extensa e circunferencial, bordos geográficos elevados, exsudado e erosões aftóides no limite proximal da ulceração mucosa. As biópsias revelaram proctite ulcerada aguda com infiltrado linfoplasmocitário difuso, sem displasia ou neoplasia. O screening de DST foi positivo para VIH-1 (CD4+ 251/mm3; N: 700­1100) e título elevado de IgA para C. trachomatis (52 000; N< 5), sugerindo LGV. O diagnóstico foi confirmado pela identificação do DNA de C. trachomatis em zaragatoa retal. Outras causas infecciosas de proctite aguda foram excluídas. Perante estes resultados, o doente acabou por mencionar que tinha tido relações homossexuais anais desprotegidas. Iniciou doxiciclina (100 mg duas vezes por dia, por 21 dias) com melhoria sintomática drástica. Repetiu retosigmoidoscopia, com sinais de proctite ulcerada em resolução. Discussão: A proctite por LGV, frequentemente desvalorizada, é uma doença re-emergente, que deve ser sempre equacionada como causa benigna de massa retal, de modo a evitar o atraso diagnóstico e o desenvolvimento de complicações. O diagnóstico torna-se mais desafiante em doentes com status VIH desconhecido. A história clínica detalhada, incluindo comportamentos sexuais de risco, é fundamental para o diagnóstico.

POEMS syndrome: a rare cause of adrenal insufficiency in a young male.

SUMMARY: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. We describe a case of a 33-year-old patient, in whom the presenting symptoms were mandibular mass, chronic sensory-motor peripheral polyneuropathy and adrenal insufficiency. The laboratory evaluation revealed thrombocytosis, severe hyperkalemia with normal renal function, normal protein electrophoresis and negative serum immunofixation for monoclonal protein. Endocrinologic laboratory work-up confirmed Addison's disease and revealed subclinical primary hypothyroidism. Thoracic abdominal CT showed hepatosplenomegaly, multiple sclerotic lesions in thoracic vertebra and ribs. The histopathologic examination of the mandibular mass was nondiagnostic. Bone marrow biopsy revealed plasma cell dyscrasia and confirmed POEMS syndrome. Axillary lymphadenopathy biopsy: Castleman's disease. Gluco-mineralocorticoid substitution and levothyroxine therapy were started with clinical improvement. Autologous hematopoietic cell transplantation (HCT) was planned, cyclophosphamide induction was started. Meanwhile the patient suffered two ischemic strokes which resulted in aphasia and hemiparesis. Cerebral angiography revealed vascular lesions compatible with vasculitis and stenosis of two cerebral arteries. The patient deceased 14 months after the diagnosis. The young age at presentation, multiplicity of manifestations and difficulties in investigation along with the absence of serum monoclonal protein made the diagnosis challenging. We report this case to highlight the need to consider POEMS syndrome in differential diagnosis of peripheral neuropathy in association with endocrine abnormalities even in young patients. LEARNING POINTS: POEMS syndrome is considered a 'low tumor burden disease' and the monoclonal protein in 15% of cases is not found by immunofixation. Neuropathy is the dominant characteristic of POEMS syndrome and it is peripheral, ascending, symmetric and affecting both sensation and motor function. Endocrinopathies are a frequent feature of POEMS syndrome, but the cause is unknown. The most common endocrinopathies are hypogonadism, primary hypothyroidism and abnormalities in glucose metabolism. There is no standard therapy; however, patients with disseminated bone marrow involvement are treated with chemotherapy with or without HCT.