Minimally invasive surgical treatment in patients with immunological thrombocytopenic purpura: total splenectomy by a hybrid mini-laparoscopic technique

Duas pacientes, uma com 28 anos e outro com 61 anos de idade, apresentavam quadro de astenia, púrpura e trombocitopenia (ambos < 15.000 plaquetas/mm3). Após a exclusão de outras etiologias e com o estabelecimento do diagnóstico de púrpura trombocitopênica imunológica (PTI), ambos iniciaram terapia com corticoides. Entretanto, ambos pacientes permaneceram com plaquetas < 20.000 /mm3, caracterizando PTI refratária e com a indicação de esplenectomia. A equipe cirúrgica optou por uma abordagem minilaparoscópica híbrida. Com uma melhor visualização do campo cirúrgico, menos trauma abdominal, mais destreza nos movimentos e com alta hospitalar precoce, o uso de instrumentos minilaparoscópicos se provou uma abordagem segura e eficaz para esses pacientes.Two female patients, one 28 years-old, and the other 61 years-old, with a history of asthenia, purpura and thrombocytopenia (both <15,000 platelets / mm3). Once other etiologies were excluded, and the diagnosis of immune thrombocytopenic purpura (ITP) was established, corticoid therapy was started in both cases. However, the patients remained with a platelet count <20,000 / mm3, characterizing refractory ITP, and the splenectomy was indicated. The surgical team opted for a hybrid minimally invasive surgical procedure. Providing a better visualization of the surgical field, less abdominal trauma, greater technical dexterity, and early hospital discharge, the use of minilaparoscopic instruments proved to be a safe and effective approach for these patients

Insuficiência cardíaca grave de etiologia amiloidótica

Amyloidosis is a systemic disease caused by the extracellular deposition of insoluble fibrils of low molecular weight proteins in various tissues. It can be classified in the forms: primary, secondary, hereditary and senile systemic. We report the case of a 75-year-old male patient who was admitted to a cardiologic emergency with progressive edema in the lower limbs started four months ago. Thirty days before admission he presented dyspnea from medium to small effort, evolving to restlessness and in decubitus (cardiac failure - functional class IV). On physical examination he presented a regular, conscious, tachidispneic, no fever, hydrated, hypochromic (1 +/ 4 +), icteric (2 +/ 4 +), acyanotic and jugular stasis at 45°. The cardiac rhythm was regular in two times and normal heart sounds, with systolic murmur in the mitral focus (2 +/ 6 +) fixed unfolding of the second sound; blood pressure of 80 x 40 mmHg and heart rate of 84 bpm. The admission electrocardiogram showed regular sinus rhythm, 1st degree atrioventricular block, low voltage in frontal plane leads, electrically inactive zone in anteroseptal region and left bundle branch conduction disorder. Chest X-ray with increased cardiac area, pattern of pulmonary congestion and bilateral pleural effusion, more pronounced on the left. A transthoracic echocardiogram was performed too, showing right enlarged cardiac chambers, mildly depressed right ventricular systolic function, moderate to severe tricuspid insufficiency, moderate pulmonary hypertension, left ventricular hypertrophy, left ventricular dysfunction and bright granular echogenicity finding. Ultrasonography of abdomen showed liver and spleen with heterogeneous texture, suggestive of granulomatous disease. Subsequently, hepatic biopsy was performed, confirming the hypothesis of infiltrative disease determined by cardiovascular amyloidosis. Patient evolved with lowered level of consciousness, and acute renal failure, undergoing hemodialysis and transfer to Coronary Unit, where he presented cardiorespiratory arrest and evolution to death.A amiloidose é uma doença sistêmica causada pela deposição extracelular de fibrilas insolúveis de proteínas de baixo peso molecular em diversos tecidos. Pode ser classificada nas formas: primária, secundária, hereditária e sistêmica senil. Relatamos o caso de um paciente masculino, 75 anos, que deu entrada em emergência cardiológica com quadro progressivo de edema em membros inferiores iniciado há quatro meses. Trinta dias antes do internamento passou a apresentar dispneia de médios para pequenos esforços, evoluindo para dispneia ao repouso e em decúbito (insuficiência cardíaca – classe funcional IV). Ao exame físico apresentou-se em estado geral regular, consciente, taquidispneico, afebril, hidratado, hipocorado (1+/4+), ictérico (2+/4+), acianótico e presença de estase jugular a 45°. O ritmo cardíaco era regular em dois tempos e bulhas, normofonéticas, com sopro sistólico em foco mitral (2+/6+) desdobramento fixo da segunda bulha; pressão arterial de 80x40 mmHg e frequência cardíaca de 84 bpm. O eletrocardiograma de admissão mostrou ritmo sinusal regular, bloqueio atrioventricular de 1º grau, baixa voltagem em derivações de plano frontal, zona eletricamente inativa em região ântero-septal e distúrbio de condução pelo ramo esquerdo do feixe de His. Radiografia de tórax com área cardíaca aumentada, padrão de congestão pulmonar e derrame pleural bilateral, mais acentuado à esquerda. Também foi realizado ecocardiograma transtorácico que evidenciou câmaras cardíacas aumentadas à direita, função sistólica de ventrículo direito levemente deprimida, insuficiência tricúspide moderada a importante, hipertensão pulmonar moderada, hipertrofia de ventrículo esquerdo, disfunção de ventrículo esquerdo e achado de ecogenicidade granular brilhante. Ultrassonografia de abdome mostrou fígado e baço com textura heterogênea, sugestivo de doença granulomatosa. Posteriormente foi realizada biópsia hepática que confirmou a hipótese de doença infiltrativa determinada pela amiloidose cardiovascular. Paciente evoluiu com rebaixamento de nível de consciência e insuficiência renal aguda, sendo realizada hemodiálise e transferência para Unidade Coronariana, onde apresentou parada cardiorrespiratória e evolução para óbito.

Neonatal Atrial Flutter Approach: A Case Series

Objective: This study set out to analyze the therapeutic options of patients with neonatal atrial flutter (AFL), considering the diagnostic methods available and the prognosis of these patients. Methodology: A retrospective study was performed by reviewing the medical records of a series of seven patients with atrial fibrillation (AF) diagnosed during fetal or neonatal period. The follow-up time of these patients ranged from 7 months to 3 years and 8 months (mean: 1 year). The clinical data for the diagnosis included sustained heart rate greater than 180 bpm, which was confirmed in all patients by a 12-lead electrocardiogram. Results: Four (57.1%) of the 7 patients studied were male. Most of the patients revealed cardiac arrhythmia during the intrauterine period when screened by fetal ultrasound in the third trimester of gestation (5 patients, i.e. 71.2%). Only the mother of Patient 2 was administered digoxin before childbirth. The atrial rate of the tachyarrhythmia revealed a mean of 375 bpm, with an increase of up to 500 bpm. Atrioventricular conduction presented a 2:1 ratio in all patients, with variations of 3:1 and 4:1 observed in Patients 1, 3 and 6. The ventricular rate ranged from 188 to 250 bpm. All patients revealed typical and counter-clockwise electrocardiogram characteristics. Synchronized electrical cardioversion was the treatment of choice in 6 patients (85.7%), with a dose of 1 J/kg. Conclusion: Early diagnosis, prior treatment, and synchronized electrical cardioversion indicate an excellent prognosis, and prolonged maintenance treatment may be unnecessary

Neonatal Atrial Flutter Approach: A Case Series

Objective: This study set out to analyze the therapeutic options of patients with neonatal atrial flutter (AFL), considering the diagnostic methods available and the prognosis of these patients. Methodology: A retrospective study was performed by reviewing the medical records of a series of seven patients with atrial fibrillation (AF) diagnosed during fetal or neonatal period. The follow-up time of these patients ranged from 7 months to 3 years and 8 months (mean: 1 year). The clinical data for the diagnosis included sustained heart rate greater than 180 bpm, which was confirmed in all patients by a 12-lead electrocardiogram. Results: Four (57.1%) of the 7 patients studied were male. Most of the patients revealed cardiac arrhythmia during the intrauterine period when screened by fetal ultrasound in the third trimester of gestation (5 patients, i.e. 71.2%). Only the mother of Patient 2 was administered digoxin before childbirth. The atrial rate of the tachyarrhythmia revealed a mean of 375 bpm, with an increase of up to 500 bpm. Atrioventricular conduction presented a 2:1 ratio in all patients, with variations of 3:1 and 4:1 observed in Patients 1, 3 and 6. The ventricular rate ranged from 188 to 250 bpm. All patients revealed typical and counter-clockwise electrocardiogram characteristics. Synchronized electrical cardioversion was the treatment of choice in 6 patients (85.7%), with a dose of 1 J/kg. Conclusion: Early diagnosis, prior treatment, and synchronized electrical cardioversion indicate an excellent prognosis, and prolonged maintenance treatment may be unnecessary

Miocardiopatia de Takotsubo: importante diagnóstico diferencial de dor torácica na emergência

A miocardiopatia de Takotsubo é uma forma de insuficiência cardíaca aguda, geralmente reversível e desencadeada por um estresse físico ou gatilho emocional, cuja apresentação clínica é bastante similar às síndromes coronarianas agudas, porém sem a obstrução vascular característica da doença coronariana. Relatamos o caso de uma mulher de 53 anos que chegou à emergência cardiológica com dor retroesternal tipicamente coronariana, alterações dinâmicas no eletrocardiograma e aumento dos marcadores de necrose miocárdica. Cateterismo cardíaco mostrou coronárias isentas de ateromatose significativa, embora houvesse pequeno grau de aterosclerose, e achados sugestivos de miocardiopatia de Takotsubo do tipo apical. Foi realizado tratamento de suporte com drogas anti-hipertensivas, visando diminuir trabalho e remodelamento cardíacos. Os marcadores de necrose miocárdica acompanhados durante o internamento mostraram curva descendente. Paciente evoluiu com melhora clínica, recebendo alta hospitalar no 7º dia após internamento, em uso de inibidor da enzima conversora de angiotensina, beta bloqueador de 3ª geração, estatina e antiagregante plaquetário.Takotsubo cardiomyopathy is a form of acute, usually reversible, heart failure triggered by physical or emotional stress. The clinical presentation is very similar to that of acute coronary syndromes, but without the characteristic vascular obstruction of coronary disease. Here we report a case of a 53-year-old woman who presented at the cardiac emergency room with typical coronary chest pain, dynamic electrocardiogram changes, and increased markers of myocardial necrosis. Cardiac catheterization showed coronary arteries without significant atheromatosis, despite a slight degree of atherosclerosis, and findings suggestive of Takotsubo cardiomyopathy of the apical type. Supportive treatment with antihypertensive drugs was performed, aiming to reduce cardiac workload and remodeling. Markers of myocardial necrosis monitored during hospitalization showed a downward curve. The patient had clinical improvement, being discharged on the 7th day after hospitalization, using angiotensin-converting enzyme inhibitor, third-generation beta-blocker, statin and platelet antiaggregants

Causa incomum de abdome agudo na sala de emergência: lipoma intestinal - série de casos e revisão de literatura

Os lipomas são lesões benignas formadas por células gordurosas adultas e circundadas por cápsula fibrosa. Esses tumores podem ser assintomáticos ou apresentar-se com uma variedade de sintomas, podendo evoluir como uma emergência cirúrgica ou serem erroneamente diagnosticados como doença maligna. Assim, descrevemos três casos de pacientes diagnosticados com lipomas gastrointestinais (um caso de intestino delgado, um caso de ceco, e o terceiro de sigmoide) após início de quadro clínico sugestivo de abdome agudo, os dois primeiros casos foram inicialmente abordados por via laparoscópica e posteriormente a ressecção e anastomose foram realizadas por vias extracorpóreas, através de uma incisão de Pfannenstiel. O terceiro caso teve abordagem laparotômica. O estudo foi observacional e retrospectivo com análise de prontuários médicos. Os lipomas do trato gastrointestinal são raros, porém devem ser lembrados como diagnóstico diferencial, principalmente nos pacientes que apresentam quadro de dor abdominal aguda, geralmente compatível com abdome aguda obstrutivo.Lipomas are benign lesions formed by adult fatty cells, surrounded by a fibrous capsule. These tumors may be asymptomatic or present a variety of symptoms, which may occur as a surgical emergency, and may also be erroneously diagnosed as malignant disease. We report three cases of patients diagnosed with gastrointestinal lipomas (one case from the small intestine, another one from the cecum and the third one from the sigmoid) after the clinical presentation of acute abdomen, the first two cases were initially treated by laparoscopy and subsequently resection and anastomosis were performed by extracorporeal pathways through a Pfannenstiel incision. The third case had a laparotomic approach. The study was observational and retrospective with research of medical records. Lipomas of the gastrointestinal tract are rare, but should be included in the differential diagnosis, especially in patients presenting with acute abdominal pain, usually compatible with acute obstructive abdomen

Resection of giant hepatic cyst by hybrid minilaparoscopy

A female patient, 59-year-old, was complaining of abdominal pain in the right hypochondrium and mesogastrium for 6 months. Ultrasonography and abdominal computed tomography were performed, both confirming a large hepatic cyst (10.6 cm × 7.6 cm × 7.3 cm) on the left lobe. A hybrid minilaparoscopic resection was proposed. We opted for unroofing the cyst, and the procedure was uneventfully performed, with a total surgical time of 60 min. In the post-operative the patient did well, with minimal abdominal pain, being discharged on the 5th post-operative day, after drain removal due to the use of intravenous antibiotic therapy

Resection of a giant nonparasitic splenic cyst by minilaparoscopy

A female patient, 20 years old, with a history of a progressive increase in abdominal volume on the left side, starting 3 years ago, with no associated symptoms and no history of trauma. Ultrasonography and a computed tomography scan of the abdomen were performed, which revealed a large splenomegaly, and a partial minilaparoscopic splenectomy was indicated. We opted for unroofing of the cyst, and the procedure was uneventful, with a total surgical time of 47 min. The patient progressed clinically well, without abdominal pain, being discharged on the 2nd post-operative day

Colangite esclerosante em pacientes imunocomprometidos associada à infecção por Cryptosporidium

Introduction: Cryptosporidium is a protozoan who parasites the gastrointestinal tract, with a significantly higher incidence in children than in adults. It mainly infects the small intestine and causes acute diarrhea in immunocompetent patients. However, in immunocompromised patients, cryptosporidiosis can be a severe and chronic disease with persistent symptoms, and cause atypical manifestations, such as atypical gastrointestinal disease, biliary tract disease, respiratory tract disease and pancreatitis. Cryptosporidium parvum infection appears to be strongly associated with the development of cholangitis. Nonetheless, the available treatment modalities are limited, and prevention and risk reduction should be the main interventions. Objective: Report the current knowledge landscape and provide information on cholangitis associated with cryptosporidiosis in immunosuppressed patients in the pediatric age, thus contributing to the diagnosis and therapeutic behaviors. Methodology: It was reviewed the main databases: Institute of Health PUBMED, Scientific Electronic Library Online (SciELO) - searching for articles that considered the subject and using and crossing the descriptors: Cholangitis, Immunodeficiency, Cryptosporidiosis, Pediatrics. Articles were searched in Portuguese, English and Spanish, containing texts from 2001 to 2018. Discussion: The suspicion of chronic liver disease arises with the appearance of considerable hepatomegaly and laboratory abnormalities (hepatic transaminases, alkaline phosphatase and gamma GT with increased serum levels) in patients with previous immunodeficiency diagnosis. Several studies have shown that the therapeutic arsenal - antiparasitic agents and macrolide antibiotics - was not effective in eradicating infection and preventing the progression of the disease. Therefore, liver transplantation becomes necessary with the evolution of the disease. However, not even the procedure is capable of improving the survival rates of this group of patients, due to the complications of the procedure, such as absence of immunocompetence, use of medications, or graft rejection. Recurrence can reach a fifth of patients. Conclusion: Sclerosing cholangitis secondary to cryptosporidiosis should be considered in the differential diagnosis of chronic liver disease in children. Diagnosis can be made by associating protozoal infection in the hepatic and biliary tract with particular cholangiographic alterations of the bile duct. Such changes in children are often subtle. Therefore, prospective, controlled and collaborative trials in patients with cryptosporidiosis sclerosing cholangitis are necessary to provide a better understanding of the prevalence, pathogenesis, potential treatment and prognosis.Introdução: Crytosporidium é um protozoário parasita do trato gastrointestinal, com incidência significativamente maior em crianças do que em adultos. Infecta, principalmente, o intestino delgado e provoca diarreia aguda em imunocompetentes. Entretanto, em pacientes imunocomprometidos, a criptosporidiose pode ser uma doença grave e crônica com sintomas persistentes, além de causar manifestações atípicas, como doença gastrintestinal atípica, doença do trato biliar, doença do trato respiratório e pancreatite. A infecção por Cryptosporidium parvum parece estar fortemente associada ao desenvolvimento de colangite. No entanto, as modalidades disponíveis de tratamento são limitadas, devendo a prevenção e redução de risco configurarem as intervenções principais. Objetivo: Relatar o panorama de conhecimentos atuais e prover informações sobre colangite associada à criptosporidiose em pacientes imunodeprimidos na faixa etária pediátrica contribuindo desta forma para o diagnóstico e condutas terapêuticas. Metodologia: Foi feita uma revisão nas principais bases de dados, Institute of Health PUBMED, Scientific Electronic Library Online (SciELO), utilizando descritores, buscando artigos que contemplassem os assuntos: Colangite, Imunodeficiência, Criptosporidiose, Pediatria, sendo realizado o cruzamento entre eles. Foram pesquisados artigos nas línguas portuguesa, inglesa e espanhola, contendo textos compreendidos entre o período de 2001 a 2018. Discussão: A suspeita da doença hepática crônica surge com o aparecimento de hepatomegalia considerável e de alterações laboratoriais (transaminases hepáticas, fosfatase alcalina e gama GT com níveis séricos aumentados) em pacientes com diagnóstico prévio de imunodeficiência. Diversos trabalhos mostraram que o arsenal terapêutico – de agentes antiparasitários e antibióticos macrolídeos - não foi eficaz para erradicar a infecção e impedir a progressão da doença. Dessa forma, o transplante hepático se faz necessário com a evolução da doença. No entanto, nem mesmo o procedimento é capaz de melhorar os índices de sobrevida deste grupo de pacientes, devido às complicações inerentes ao transplante, como ausência de imunocompetência, uso de medicações e, rejeição do enxerto. A recorrência pode chegar a um quinto dos pacientes. Conclusão: A colangite esclerosante secundária à criptosporidiose deve ser considerada no diagnóstico diferencial de doença hepática crônica em crianças. O diagnóstico pode ser feito pela associação da infecção pelo protozoário na via hepática e biliar com alterações colangiográficas características do ducto biliar. Tais alterações em crianças são, frequentemente, sutis. Portanto, ensaios prospectivos, controlados e colaborativos em pacientes com colangite esclerosante por criptosporidiose são necessários para fornecer uma melhor compreensão da prevalência, patogênese, possível tratamento e prognóstico