Breast Cancer Presents With a Paraneoplastic Neurologic Syndrome

BACKGROUND: Paraneoplastic neurologic syndromes (PNS) pose quite an uncommon neurological complication, affecting less than 1% of patients with breast cancer. Nearly one third of these patients lack detectable onconeural antibodies (ONAs), and improvement in neurologic deficits with concomitant cancer treatments is achieved in less than 30% of cases. CASE PRESENTATION: A 42-year-old, premenopausal woman presented with facial paralysis on the central left side accompanied by a left tongue deviation, an upward vertical nystagmus, moderate spastic paraparesis, dystonic posturing of the left foot, lower limb hyperreflexia and bilateral extensor plantar reflex. After ruling out all other potential neurologic causes, PNS was suspected but no ONAs were found. A PET-CT scan detected increased metabolism in the right breast, as well as an ipsilateral thoracic interpectoral adenopathy. Core biopsy confirmed the presence of an infiltrating duct carcinoma. After breast surgery, the neurologic symptoms disappeared. One week later, the patient was readmitted to the hospital with a bilateral fatigable eyelid ptosis, and two weeks later, there was a noticeable improvement in eyelid ptosis, accompanied by a rapid and progressive development of lower spastic paraparesis. She started adjuvant treatment with chemotherapy with marked clinical and neurological improvement, and by the end of radiotherapy, there were no signs of neurologic impairment. CONCLUSION: This case study highlights the importance of a high level of vigilance for the detection of PNS, even when ONAs are not detected, as the rapid identification and treatment of the underlying tumor offers the best chance for a full recovery.

Association of Intestinal Malrotation and Bochdalek Hernia in an Adult: a Case Report

Background Late presentations of congenital diaphragmatic hernia are rare and differ from the classic neonatal presentation. The association with other congenital malformations in children, mainly intestinal malrotation, is well documented. The diagnosis of this association in adults is very rare, and depends on a high degree of suspicion. Case presentation We report a case of a 50-year-old female Caucasian patient with a previous history of intestinal malrotation diagnosed in adolescence and treated conservatively. She was referred to the hospital with signs and symptoms of intestinal obstruction. The patient undertook computed tomography that confirmed small bowel obstruction with no obvious cause, and a right subphrenic abscess with right empyema was also present. An exploratory laparotomy was performed that revealed an intestinal malrotation associated with a right gangrenous and perforated Bochdalek hernia. Resection of the affected small bowel, closure of the Bochdalek foramen and the Ladd procedure were carried out. Conclusion This case shows a rare association of two rare conditions in adults, and highlights the challenge in reaching the diagnosis and management options.

Oclusão Intestinal. Experiência da Urgência do H.S.José 1981-1991

Os Autores descrevem a experiência, em oclusão intestinal, da Unidade de Urgência Cirúrgica dos Hospitais Civis de Lisboa (UUC-HCL), no período de 10 anos, decorrido entre Novembro de 1981 e Novembro de 1991, estimando que esta entidade patológica tenha constituído cerca de 15% da casuística de cirurgia geral daquele Serviço. Os dados referentes aos 3679 doentes, operados de urgência por oclusão intestinal, foram reunidos num ficheiro informatizado e a sua análise global é descrita recorrendo à representação gráfica dos elementos mais relevantes. Foi semelhante a distribuição por sexos : feminino (49%) e masculino (5 1%). A forma mecânica de oclusão (94%)teve como patologias mais frequentes: a hérnia estrangulada (1604 casos), a brida (568 casos) e o cancro (713 casos); e a forma dinâmica ou funcional (6%) teve, como principal ocorrência, a isquémia intestinal (143 casos). A terapêutica cirúrgica foi avaliada de forma geral. No entanto, comenta-se, brevemente, a evolução registada no tratamento primário do cancro colorrectal em oclusão (625 casos). A taxa de mortalidade global foi de 10,8% (adulto). Em termos relativos, teve como principais expressões: (39%) na isquémia intestinal, (23%) no cancro e (22%) no volvo intestinal.

Femoral Approach: an Exceptional Alternative for Permanent Pacemaker Implantation

The classic transvenous implantation of a permanent pacemaker in a pectoral location may be precluded by obstruction of venous access through the superior vena cava or recent infection at the implant site. When these barriers to the procedure are bilateral and there are also contraindications or technical difficulties to performing a thoracotomy for an epicardial approach, the femoral vein, although rarely used, can be a viable alternative. We describe the case of a patient with occlusion of both subclavian veins and a high risk for mini-thoracotomy or videothoracoscopy, who underwent implantation of a permanent single-chamber pacemaker via the right femoral vein.

Hepatic Artery Thrombosis in Live Liver Donor Transplantation: How to Solve - a Case Report

The decrease in the number of cadaveric donors has proved a limiting factor in the number of liver transplants, leading to the death of many patients on the waiting list. The living donor liver transplantation is an option that allows, in selected cases, increase the number of donors. One of the most serious complications in liver transplantation is hepatic artery thrombosis, in the past considered potentially fatal without urgent re-transplantation. A white male patient, 48 years old, diagnosed with hepatocellular carcinoma in chronic liver failure caused by hepatitis B virus, underwent living donor liver transplantation (right lobe). Doppler echocardiography performed in the immediate postoperative period did not identify arterial flow in the right branch, having been confirmed thrombosis of the right hepatic artery in CT angiography. Urgent re-laparotomy was performed, which consisted of thrombectomy and re-anastomosis of the hepatic artery with segmental splenic artery allograft interposition. The patient started anticoagulation and antiplatelet therapy with acetylsalicylic acid. Serial evaluation with Doppler echocardiography showed hepatic artery patency. At present, the patient is asymptomatic. One of the most devastating complications in liver transplantation, and particularly in living liver donor, is thrombosis of the hepatic artery; thus, early diagnosis and treatment is vital. The rapid intervention for revascularization of the graft avoids irreversible ischemia of the bile ducts and hepatic parenchyma, thus avoiding the need for re-transplantation.

Anesthesia for Ex Utero Intrapartum Treatment: Renewed Insight on a Rare Procedure

The ex utero intrapartum treatment is a rare surgical procedure performed in cases of expected postpartum fetal airway obstruction. The technique lies on a safe establishment of a patent airway during labor in anticipation of a critical respiratory event, without interrupting maternal-fetal circulation. Anesthetic management is substantially different from that regarding standard cesarean delivery and its main goals include uterine relaxation, fetal anesthesia and preservation of placental blood flow. We present the case of an ex utero intrapartum treatment procedure performed on a fetus with a large cervical lymphangioma and prenatal evidence of airway compromise. Modifications to the classic ex utero intrapartum treatment management strategies were successfully adopted and will be discussed in the following report.

Pancreatic Involvement by Plasma Cell Neoplasms

INTRODUCTION: Pancreatic involvement by plasma cell neoplasms is an extremely rare event, with only 50 cases described in the literature. They can present as a primary solitary extramedullary plasmacytoma or plasmacytoma secondary to a plasma cell myeloma. Clinical manifestations are due to the presence of a pancreatic mass usually in the pancreas head, which causes extra-biliary obstruction and abdominal pain. METHODS: Abdominal imaging including CT scan or endoscopic ultrasound with fine-needle aspiration tissue sampling is essential for the initial diagnostic procedure. However, immunohistochemical analysis of the biopsy specimen or flow cytometry of the aspirated material is crucial to prove the monoclonality and the final diagnosis of a plasma cell neoplasm. DISCUSSION: Management of these situations include radiotherapy, chemotherapy, surgery or combined therapy. Novel medications including the immunomodulatory drugs or the proteasome inhibitors followed by consolidation with intensive chemotherapy and haematopoietic stem cell transplantation are nowadays used as upfront treatment in the cases associated to a plasma cell myeloma. CONCLUSION: Despite the rarity, plasma cell neoplasms should be considered in the differential diagnosis of obstructive jaundice and pancreatic neoplasms since they are potentially treatable situations.

Under-Report and Underdiagnosis of Chronic Respiratory Diseases in an African Country

BACKGROUND: Chronic respiratory diseases (CRD) are greatly underestimated. The aim of this study was to assess the burden associated with reported CRD and chronic obstructive pulmonary disease, as defined on the basis of various standardized criteria, by estimating their point prevalence in a sample of individuals attending the Primary Health Care (PHC) level and Emergency Room (ER) Departments in Cape Verde (CV) archipelago. The second aim of the study was to identify factors related to airways obstruction and reported CRD in this population. METHODS: A cross-sectional study was carried out in CV during 2 weeks. Outpatients aged more than 20 years seeking care at PHC level and ER answered a standardized questionnaire and were subjected to spirometry, independently of their complaint. Two criteria for airways obstruction were taken into account: forced expiratory volume (FEV(1)) <80% of the predicted value and FEV(1)/forced vital capacity (FVC) ratio <0.70. RESULTS: A total of 274 individuals with a satisfactory spirometry were included. 22% of the individuals had a FEV(1) < 80%. Individuals older than 46 years had a higher risk of having airways obstruction. Asthma diagnosis (11%) had a clear association with airways obstruction. Smoking was a risk factor for a lower FEV(1). Working in a dust place and cooking using an open fire were both related to chronic bronchitis and asthma diagnosis. CONCLUSION: Under-report and underdiagnosis of chronic respiratory conditions seem to be a reality in CV just as in other parts of the world. To improve diagnosis, our results reinforce the need of performing a spirometry

Cryptosporidiosis of the biliary tract mimicking pancreatic cancer in an AIDS patient

Diarrhea caused by Cryptosporidium sp is frequent in patients with AIDS, but involvement of other organs of the digestive tract is uncommon. We report a case of Cryptosporidium-associated obstruction of the biliary tract mimicking cancer of the head of the pancreas in a 43-year-old woman with AIDS.

Chagasic enteropathy

Involvement of the hollow organs of the digestive apparatus can occur in patients in the chronic phase of Chagas' disease. The basic mechanism is destruction of neurons of the enteric nervous system. Whereas megaesophagus and megacolon are the most notable and most extensively studied expressions of the digestive form of Chagas' disease, involvement of the small intestine (Chagasic enteropathy) is less frequent and less known than involvement of the two above mentioned entities. Chagasic enteropathy can be responsible for important clinical and laboratory manifestations resembling those of dyspeptic syndrome, intestinal pseudo-obstruction and bacterial overgrowth in the small intestine. Chagasic enteropathy also involves peculiar functional changes, especially those related to motor activity of the organ and to intestinal absorption of carbohydrates. In practice, the diagnosis is based on radiographic documentation of dilation of visceral segments. Treatment consists of clinical control of the above syndromes and, eventually, appropriate surgical operations.

Vibrios patogênicos em ostras (Crassostrea rhizophorae) servidas em restaurantes no Rio de Janeiro: um alerta para a Saúde Pública

Avaliaram-se 40 amostras de ostras (Crassostrea rhizophorae) servidas in natura em 15 restaurantes da Cidade do Rio de Janeiro, a fim de investigar a presença de Vibrio spp. As amostras de ostras foram analisadas e submetidas a enriquecimento em água peptonada alcalina adicionada de 1 e 3% de NaCl, incubadas a 37°C por 24 horas. Em seguida, os cultivos foram semeados em agar tiossulfato citrato bile sacarose e as colônias suspeitas foram submetidas à caracterização bioquímica. Vibrio parahaemolyticus, Vibrio carchariae, Vibrio alginolyticus e Vibrio vulnificus representaram as principais espécies (> 60%) isoladas a partir das ostras in natura.

Contributo da curva de débito-volume na detecção da obstrução da via aérea central

RESUMO: Introdução: A obstrução da via aérea central (OVAC) refere-se a um processo patológico que conduz a limitação do fluxo de ar ao nível do espaço glótico e subglótico, traqueia e brônquios principais. O seu correcto diagnóstico e tratamento constituem um território de interesse e preocupação para os profissionais de saúde, e requerem um profundo conhecimento da sua etiologia, fisiologia, diagnóstico e opções terapêuticas dado o potencial em originar significativa morbilidade e mortalidade. A avaliação da OVAC abrange múltiplas vertentes, entre as quais se salienta o componente clínico (sinais e sintomas), a repercussão fisiopatológica (função respiratória) e o estudo imagiológico (TC do tórax e broncoscopia). A compilação destes dados associada à etiologia, constituem factores importantes para estabelecer o prognóstico, determinar a necessidade de tratamento ou delinear uma futura intervenção terapêutica. A broncoscopia é o Gold Standard de avaliação desta condição, mas desde há cerca de 40 anos a curva de débito-volume constitui uma ferramenta não invasiva de detecção de OVAC. Apesar deste método ser utilizado até os nossos dias, poucos têm sido os estudos com o objectivo de verificar a sensibilidade e especificidade da curva de débito-volume na detecção de OVAC, bem como averiguar a relação entre as alterações morfológicas e quantitativas da mesma com a localização, o tipo e o grau da obstrução. Material e Métodos: Entre 1 de Novembro de 2009 e 30 de Abril de 2010, os doentes com indicação para a realização de broncoscopia diagnóstica ou terapêutica na Unidade de Técnicas Invasivas Pneumológicas (UTIP) do Centro Hospitalar Lisboa Norte – Hospital Pulido Valente (CHLN – HPV) foram seleccionados de forma consecutiva de acordo com os critérios de inclusão e exclusão. As avaliações (broncoscopia, curva de débito-volume e avaliação da dispneia) realizaram-se com um intervalo de tempo máximo de sete dias. A broncoscopia flexível foi realizada segundo as normas da British Thoracic Society e as curvas de débito-volume segundo as normas da ATS/ERS TaskForce 2005. Para a avaliação da dispneia recorreu-se à escala de dispneia MRC (Medical Research Council). Um painel de peritos realizou a avaliação da morfologia da curva de débito-volume (sugestiva ou não de OVAC) e um elemento independente a verificação dos critérios quantitativos e morfológicos (variáveis intra e extratorácica e fixa) da curva. O estudo foi aprovado pela Comissão de Ética para a Saúde do CHLN e todos os doentes assinaram um consentimento informado de participação. Resultados: Estudaram-se 82 doentes, 36 (44%) dos quais com OVAC. A predominância foi do género masculino, em relação ao feminino. A sensibilidade e especificidade dos critérios quantitativos da curva de débito-volume na detecção de OVAC foi de 91.3% e 88.9% respectivamente. Quando se utilizaram os critérios morfológicos da curva de débito-volume os valores foram de 93.5% e 30.6%. A agregação dos critérios morfológicos e quantitativos permitiu alcançar uma sensibilidade de 95.7% e especificidade de 86.1%. Nesta amostra, o critério quantitativo com maior ocorrência foi o FEF50/FIF50≥1 (83% dos doentes com OVAC). Este mostrou relacionar-se com todas as localizações de obstrução excepto o terço médio da traqueia. Mostrou, ainda, ter uma relação forte e positiva com o grau e tipo de obstrução (intra e extraluminal). O segundo foi o FEV1/PEF≥8, presente em 36% dos casos de OVAC. Relacionou-se com as obstruções no terço inferior da traqueia e brônquio principal direito (BPD). Também apresentou relação forte e positiva com o grau de obstrução e com os tipos de obstrução anteriormente descritos. Quanto à sintomatologia foi possível associar o grau de obstrução com o de dispneia e a presença de estridor com o grau e localização da obstrução na traqueia. Conclusões: Os resultados deste estudo demonstram que os critérios quantitativos da curva de débito-volume têm elevada sensibilidade e especificidade na detecção de OVAC. O critério FEV50/FIF50≥1 tem um bom poder discriminativo na detecção dessa condição, tendo sido relacionado com a localização, o grau e o tipo de obstrução. O critério FEV1/PEF≥8, embora com menor poder discriminativo, também se relaciona com o grau, a localização e o tipo de obstrução. A morfologia da curva tem uma boa sensibilidade mas baixa especificidade na detecção de OVAC, mas a agregação entre os critérios morfológicos e quantitativos aumenta a sensibilidade e especificidade. A dispneia e o estridor foram relacionados com o grau de obstrução e o último com a localização ao nível da traqueia.-------------ABSTRACT: Introduction: Central airway obstruction (CAO) refers to a pathological process that leads to restriction of airflow at the level of the glottis and subglottis, trachea and main bronchi. It’s proper diagnosis and treatment is an area of interest and concern to health professionals, and requires a deep knowledge of its etiology, physiology, diagnosis and treatment options, concerning the potential to cause significant morbidity and mortality. The evaluation of CAO covers multiple aspects: the clinical component (signs and symptoms), the pathophysiological effect (lung function) and the imaging study (bronchoscopy and chest CT). The compilation of this data associated with the etiology, are important for establishing prognosis, determine the need for treatment or outline a future therapeutic intervention. Bronchoscopy is the gold standard for evaluating this condition, but for the last 40 years the flow-volume loop has been used as a noninvasive tool for detecting CAO. Although this method is still in use, only few studies were made in order to verify its sensitivity and specificity in detecting CAO, and investigate the relation between morphological and quantitative changes of the curve to location, type and degree of obstruction. Methods: Between 1st November 2009 and 30th April 2010, patients with indication to perform diagnostic or therapeutic bronchoscopy in Interventional Pulmonology Unit - Hospital Pulido Valente (CHLN - HPV), were selected consecutively according to the inclusion and exclusion criteria. All assessments (bronchoscopy, flow-volume loop and dyspnea) were carried out within a period of seven days. The flexible bronchoscopy was performed according to the standards of the British Thoracic Society and the flow-volume loops in accordance with the standards of the ATS / ERS Taskforce 2005. For the evaluation of dyspnea was used to MRC dyspnea scale (Medical Research Council). A panel of experts evaluated the morphology of flow-volume loop (suggestive or non-suggestive of CAO) and an independent element established the quantitative criteria and morphological (intra and extrathoracic variables and fixed) of the curve. This study was approved by the Ethics Committee for Health CHLN and all the patients signed an informed consent to participate. Results: We’ve studied 82 patients, 36 (44%) of those with CAO. The majority of the patients were males, compared to females. The sensitivity and specificity of the quantitative criteria of the flow-volume curve in detecting CAO was 91.3% and 88.9% respectively. When we used the morphological criteria of flow-volume loop these values were 93.5% and 30.6%. The combination of quantitative and morphological criteria produced values of 95.7% sensitivity and 86.1% specificity. FEF50/FIF50≥1 was the most representative quantitative criterion (83% of patients with CAO) and it was correlated with all sites of obstruction except in the middle third of the trachea. It has shown a strong and positive association with the degree and type of obstruction (intra and extraluminal). The second was the FEV1/PEF ≥ 8, present in 36% of cases of CAO. It could be correlated with the obstruction in the lower third of the trachea and right main bronchus. It also showed a strong positive relation with the degree and types of obstruction described above. Regarding symptoms, we found a link between the degree of obstruction and dyspnea. The presence of stridor was correlated with the location and the degree of obstruction in the trachea. Conclusion: The results of this study demonstrate that the quantitative criteria of the flow-volume loop have a high sensitivity and specificity in detecting CAO. The criterion FEV50/FIF50 ≥ 1 has a good discriminative power to detect this condition and was related to the location, degree and type of obstruction. The criterion FEV1/PEF ≥ 8, although with a weaker discriminative power, also relates to the degree, location and type of obstruction. The morphology of the curve has a good sensitivity but low specificity in detecting CAO although the combination between the morphological and quantitative criteria increases sensitivity and specificity. Dyspnea and stridor were related to the degree of obstruction and the last one with its location in the trachea.

Primary biliary cirrhosis and myopathy: an uncommon association

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

Elimination of biliary stones through the urinary tract: a complication of the laparoscopic cholecystectomy

The introduction and popularization of laparoscopic cholecystectomy has been accompanied with a considerable increase in perforation of gallbladder during this procedure (10%--32%), with the occurrence of intraperitoneal bile spillage and the consequent increase in the incidence of lost gallstones (0.2%--20%). Recently the complications associated with these stones have been documented in the literature. We report a rare complication occurring in an 81-year-old woman who underwent laparoscopic cholecystectomy and developed cutaneous fistula to the umbilicus and elimination of biliary stones through the urinary tract. During the cholecystectomy, the gall bladder was perforated, and bile and gallstones were spilled into the peritoneal cavity. Two months after the initial procedure there was exteriorization of fistula through the umbilicus, with intermittent elimination of biliary stones. After eleven months, acute urinary retention occurred due to biliary stones in the bladder, which were removed by cystoscopy. We conclude that efforts should be concentrated on avoiding the spillage of stones during the surgery, and that no rules exist for indicating a laparotomy simply to retrieve these lost gallstones.

Laparoscopic treatment of retroperitoneal fibrosis: report of two cases and review of the literature

OBJECTIVES: We present the results of treatment by laparoscopy of two patients with retroperitoneal fibrosis and review the literature since 1992, when the first case of this disease that was treated using laparoscopy was published. We also discuss the contemporary alternatives of clinical treatment with corticosteroids and tamoxifen. CASE REPORT: Two female patients, one with idiopathic retroperitoneal fibrosis, and other with retroperitoneal fibrosis associated with Riedel's thyroiditis, were treated using laparoscopic surgery. Both cases had bilateral pelvic ureteral obstruction and were treated using the same technique: transperitoneal laparoscopy, medial mobilization of both colons, liberation of both ureters from the fibrosis, and intraperitonealisation of the ureters. Double-J catheters were inserted before the operations and removed 3 weeks after the procedures. The first patient underwent intraperitonealisation of both ureters in a single procedure. The other had 2 different surgical procedures because of technical difficulties during the first operation. Both patients were followed for more than 1 year and recovered completely from the renal insufficiency. One of them still has occasional vague lumbar pain. There were no abnormalities in the intravenous pyelography in either case. CONCLUSIONS: Surgical correction of retroperitoneal fibrosis, when indicated, should be attempted using laparoscopy. If possible, bilateral ureterolysis and intraperitonealisation of both ureters should be performed in the same operation.