Púrpura. Manifestação de Amiloidose Sistémica Primária

Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.

Abcesso Mediastínico Após Laceração Iatrogénica do Seio Piriforme - Caso Clínico

A hipofaringe e o esófago cervical são áreas particularmente vulneráveis a traumatismos por entubação oro-traqueal. Estes acidentes anestésicos são raros, pouco relatados e com maior incidência em situações de emergência. As perfurações faringoesofágicas podem passar despercebidas até ao aparecimento de sinais e sintomas característicos. Estes incluem dor cervical, febre, disfagia, leucocitose, enfisema subcutâneo e pneumomediastino. O tratamento cirúrgico vs conservador é controverso. É no entanto consensual que o intervalo de tempo entre o traumatismo e o diagnóstico e tratamento é, por si só, o factor mais importante para o prognóstico. Os autores apresentam um caso de uma parturiente submetida a cesariana de urgência em que ocorreu um acidente anestésico por entubação difícil, com laceração do seio piriforme e posterior formação de abcesso mediastínico.

Abcesso Renal na Gravidez

Apesar das infecções urinárias serem relativamente frequentes durante a gravidez, a ocorrência de um abcesso renal é muito raro. A patogénese do abcesso renal tem mudado ao longo dos anos. Actualmente, mais de 75% dos casos têm origem numa infecção do aparelho urinário. Os autores apresentam o caso clínico de uma grávida de 35 anos, internada às 38 semanas e cinco dias de gestação por febre, náuseas, vómitos e dor lombar à direita. Os dados clínicos e os exames complementares realizados levaram ao diagnóstico de abcesso renal. É feita uma revisão da literatura acerca do tema.

Abcesso Tubo-Ovárico. Análise de 20 Casos

Os autores procedem à análise retrospectiva dos casos de abcesso tubo-ovárico, internados no Serviço de Ginecologia da Maternidade Dr. Alfredo da Costa, num período de 3 anos (1991-1993). O número total de casos foi 20. A incidência em nulíparas foi 25%. Uma percentagem significativa das doentes (30%) tinha sido recentemente submetida a técnicas de instrumentação uterina. Em apenas 15% dos casos havia antecedentes de doença inflamatória pélvica. Nas doentes portadoras de DIU a incidência de abcessos unilaterias e bilaterais foi idêntica. Na maioria dos casos (85%) a apirexia surgiu até 48 horas após o início da antibioterapia. A maioria das doentes (90%) foi submetida a terapêutica cirúrgica. O tempo médio decorrido entre o início da antibioterapia e a intervenção cirúrgica foi 3 dias. Registou-se um caso de rotura de abcesso. Em 3 (15%) doentes verificou-se intraoperatoriamente a co-existência de um abcesso apendicular. Em 30% dos casos as doentes foram submetidas a histerectomia total com anexectomia unilateral ou bilateral. Uma das 2 (10%) doentes submetidas a terapêutica médica isolada apresentou recidiva 1 mês após a alta. A abordagem terapêutica do abcesso tubo-ovárico embora se tenha tornado mais conservadora, continua a incluir, na maioria dos casos, drenagem ou resecção cirúrgica após antibioterapia adequada.

Localized Abdominal Idiopathic Lipodystrophy

Localized loss of subcutaneous tissue can occur after panniculitis, injections of corticosteroids and other drugs, or associated with infectious, autoimmune or neurologic diseases. The "idiopathic lipoatrophies" are a group of poorly characterized diseases, with focal disappearance of subcutaneous fat, and usually the thighs, abdomen or the ankles are affected. Three subtypes have been described based on clinical presentation: lipoatrophia semicircularis, annular lipoatrophy of the ankles and centrifugal lipodystrophy. We describe a 52-year-old female patient who developed a localized atrophy of the abdominal areas over a period of 3 months without any inflammatory signs over the evolution of the disease. The patient denied any previous local trauma or medication of any type. The atrophy stabilized, showing no progression over the last 6 years. The histopathological examination was normal except for the absence of subcutaneous fat, although the biopsy was taken down to the fascia. There was no clinical or serologic evidence of autoimmune diseases and laboratory testing for Borrelia burgdorferi infection was negative. Other causes of localized lipoatrophies were excluded and the final diagnosis was localized idiopathic lipodystrophy. Our patient is the second report on an abdominal lipodystrophy, with no previous inflammatory signs, absence of subcutaneous fat and no associated pathogenic factor. There is no established treatment for idiopathic lipodystrophy, and the lesions do not tend to resolve spontaneously.

Abdominoplasty and Thoraco-Epigastric Flaps for Large Anterior Trunk Defects after Dermatofibrosarcoma Protuberans Wide Resection: Two Illustrative Cases

INTRODUCTION: Excision of large dermatofibrosarcoma protuberans in the anterior aspect of the trunk often results in large surgical defects that frequently dictate the need for microsurgical reconstruction. However, this option is not always available. PRESENTATION OF CASE: The authors describe two patients with very large anterior trunk dermatofibrosarcoma protuberans: one in the epigastric region and the other in the hypogastric region. In the patient with the hypogastric tumor, a classical abdominoplasty flap associated with umbilical transposition was used to cover the skin defect after muscle and fascial plication, and placement of a polypropylene mesh. In the patient with the epigastric tumor, a synthetic mesh was also placed, and the skin and subcutaneous defect was reconstructed with a reverse abdominoplasty flap and two thoraco-epigastric flaps. In both cases, complete closure was possible without immediate or late complications. DISCUSSION: The local options described in this paper present several potential advantages compared to microsurgical reconstruction, namely they are easier and faster to perform and teach; they provide a good skin color and texture match; they are not associated with distant donor site morbidity; follow-up is usually less cumbersome; the post-operative hospital stay tends to be shorter; they are less costly; they are less prone to complete failure. CONCLUSION: The authors believe that these two patients clearly show that local flaps, although frequently neglected, continue to be valid options for reconstructing large anterior trunk defects, even in the current era of microsurgery enthusiasm.

Sarcoidose Subcutânea. Um Caso Raro de Manifestação Cutânea Específica

A sarcoidose é uma doença granulomatosa multissistémica de etiologia desconhecida. O envolvimento cutâneo pode ocorrer, sendo classificado de específico ou inespecífico, dependendo da presença ou ausência de granulomas no exame histopatológico da pele. A forma subcutânea é uma forma particular e mais rara de apresentação e constitui o único subtipo que se crê estar associado a doença sistémica.

Abcesso Hepático Amebiano. A Propósito de um Caso Clínico

A amebíase é uma das doenças parasitárias mais comuns no mundo. As principais formas invasivas da doença são a colite amebiana e o abcesso hepático. Apresenta-se o caso clínico de um homem de 42 anos admitido com um quadro agudo de febre elevada e dor abdominal no hipocôndrio direito com dois dias de evolução. A tomografia axial computorizada do abdómen revelou a presença de 3 lesões abcedadas a nível do lobo direito do fígado. Tratando-se de um doente residente em área endémica de amebiase colocou-se o diagnóstico diferencial entre abcesso amebiano hepático versus abcesso piogénico, situações com abordagem terapêutica distinta. O quadro clínico e a serologia positiva para Entamoeba histolytica confirmaram o diagnóstico de abcesso amebiano hepático. Os autores apresentam uma breve revisão desta entidade, rara nos países desenvolvidos, que no adequado contexto epidemiológico deve ser considerada no diagnóstico diferencial dos abcessos hepáticos.

Implante Contraceptivo Não Palpável - Estratégias para a sua Localização e Remoção

Overview and Aims: The contraceptive implant is frequently used to provide contraceptive protection over three years. The implant is inserted into the subcutaneous tissue of the upper arm, and should be palpable and easily removed. We evaluated the best imaging strategy for non-palpable implant (Implanon®) localization and removal. Study Design: Retrospective study. Population: A total of 11 women referred to a tertiary care hospital, between October 2009 and January 2012, for localization and removal of their non-palpable implants. Methods: Different localization methods (ultrasound and magnetic resonance imaging) were evaluated for non-palpable rod. Results: Seven of the nonpalpable implants were inserted in a health care center, three in a district hospital and one in a private clinic. In three women, the reasons for requesting removal were the end of the implant validity, two wanted to become pregnant, two had weight gain, one had weight loss, one referred irregular bleeding, one had two implants and one did a hysterectomy. In 81.8% (9) of the women, the implants were identified and localized by ultrasound, and successfully removed. In two patients the implant was not found and therefore not removed. Conclusions: In our study, high resolution ultrasound proved to be a sensitive method in implants localization, being the primary choice for determining the location of nonpalpable implants.

Abcessos Retrofaríngeos de Causa Traumática na Criança

Os abcessos retrofaríngeos constituem uma entidade clínica relativamente rara, grave e por vezes fatal, devido à sua localização anatómica e consequente potencial para complicações graves, pelo que requerem diagnóstico e terapêutica precoces e correctos. Predominam na criança, podendo a su etiologia ser traumática ou não traumática, sendo esta a mais frequente. Pela sua raridade, os autores apresentam três casos clínicos de abcessos retrofaríngeos de causa traumática em crianças com menos de cinco anos de idade, a propósito dos quais tecem considerações acerca da clínica, diganóstico e terapêutica desta patologia.

Iatrogenic Aortic Pseudoaneurysm: a Forgotten Complication

Pseudoaneurysms of the ascending aorta are a rare complication of cardiac surgery. However, the poor prognosis associated with this condition if untreated makes early diagnosis and treatment important. We present the case of a 66-year-old woman who had undergone mitral valvuloplasty 12 days previously, who was admitted with a diagnosis of new-onset atrial fibrillation. The transthoracic echocardiogram showed a clot in the right atrium and anticoagulation was initiated, followed by antibiotic therapy. After further investigation, the patient was diagnosed with a pseudoaneurysm of the ascending aorta and underwent surgical repair, followed by six weeks of antibiotic therapy. She was readmitted six months later for an abscess of the lower sternum and mediastinum. After a conservative approach with antibiotics and local drainage failed, recurrence of a large pseudoaneurysm compressing the superior vena cava was documented. A third operation was performed to debride the infected tissue and to place an aortic allograft. There were no postoperative complications.

Calciphylaxis: a Literature Review Based in Two Case Reports

Calciphylaxis is a rare and devastating obliterative vasculopathy, leading to ischemia and subcutaneous necrosis. In most cases it affects patients with renal disease and is associated with high morbidity and mortality. We present two case reports followed recently in our department, and a literature review on this topic. Case one refers to an 80 -year -old Caucasian woman with chronic kidney disease stage 5 and primary hyperparathyroidism with secondary brown tumour and calciphylaxis. Case two refers to a 59 -year -old Caucasian woman admitted with severe nephrotic syndrome associated with amyloidosis, that developed a catastrophic picture of calciphylaxis, ending in the patient’s death. There is a critical need to understand the pathogenesis of calciphylaxis. Its comprehension is the only way to improve the survival of these patients, and may help to elucidate the pathophysiology of vascular calcification in general. Educating physicians in the prevention and early detection of calciphylaxis is crucial. Only by increasing the knowledge about risk factors, pathophysiology, response to treatment and outcome, will we be able to improve prophylaxis and therapy of patients with calciphylaxis, decreasing the high mortality of this entity.

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.

Phaeohyphomycosis Caused by Alternaria Infectoria Presenting as Multiple Vegetating Lesions in a Renal Transplant Patient

The genus Alternaria is one of the most common black moulds and appears to be increasing as a causative agent of subcutaneous phaeohyphomycosis, particularly among immunosuppressed patients. A 53-year-old patient who had received a kidney transplant presented with multiple verrucous lesions on the distal extremities. Positive histopathology and cultures, in addition to rDNA ITS region sequencing, identified the fungal isolate as Alternaria infectoria. Oral itraconazole was administered for 10 months. A follow-up at 15 months demonstrated no signs of infection. Clinical manifestations of cutaneous alternariosis vary significantly and only a few cases have been described in the literature. Although optimal treatment options remain controversial, this case of phaeohyphomycosis was successfully treated with itraconazole monotherapy.