Giant Conjunctival Cyst of the Orbit in a Patient with Previous Enucleation.

Introduction: Conjunctival cysts of the orbit development are a rare complication in enucleated patients. They result from the entrapment of conjunctival epithelium within the orbit. Case Presentation: We report the case of a 25-year-old man with a history of enucleation for a retinoblastoma of the right eye who presented with difficulty in fitting his eye prothesis. On his past medical records, there was no reference to the placement of any orbital implant at the time of the surgery. Biomicroscopy of the right eye revealed a thickened bulbar conjunctiva, an inferior symblepharon, and a translucid central area with vascularization. Imaging was remarkable for a cystic cavity filling the whole right orbit. Biopsy revealed the diagnosis of a conjunctival cyst, and drainage was performed, alleviating the patient's symptoms. Conclusion: Orbital conjunctival cysts may pose a clinical problem, and treatment modalities include surgical excision, absolute alcohol injection, and trichloroacetic acid injection.

Immunoparalysis in critically ill children.

Immunoparalysis is associated with poorer outcomes in the paediatric intensive care unit (PICU) setting. We aimed to determine the group of patients with higher chances of immunoparalysis and correlate this status with increased risks of nosocomial infection and adverse clinical parameters. We conducted an exploratory study with prospective data collection in a university-affiliated tertiary medical, surgical, and cardiac PICU. Fifteen patients with multiple organ dysfunction syndrome were included over a period of 6 months. Monocyte's human leucocyte antigen (HLA)-DR expression and tumour necrosis factor (TNF)-α and interleukin (IL)-6 production were measured by flow-cytometry at three time points (T1 = 1-2 days; T2 = 3-5 days; T3 = 6-8 days). Using the paediatric logistic organ dysfunction-2 score to assess initial disease severity, we established the optimal cut-off values of the evaluated parameters to identify the subset of patients with a higher probability of immunoparalysis. A comparative analysis was performed between them. Sixty per cent were males; the median age was 4.1 years. Considering the presence of two criteria in T1 (classical monocytes mean fluorescence intensity [MFI] for HLA-DR ≤ 1758.5, area under the curve (AUC) = 0.775; and frequency of monocytes producing IL-6 ≤ 68.5%, AUC = 0.905) or in T3 (classical monocytes MFI of HLA-DR ≤ 2587.5, AUC = 0.675; and frequency of monocytes producing TNF-α ≤ 93.5%, AUC = 0.833), a variable to define immunoparalysis was obtained (100% sensitivity, 81.5% specificity). Forty per cent of patients were assigned to the immunoparalysis group. In this: a higher frequency of nosocomial infection (p = 0.011), vasoactive inotropic score (p = 0.014) and length of hospital stay (p = 0.036) was observed. In the subgroup with the diagnosis of sepsis/septic shock (n = 5), patients showed higher percentages of non-classical monocytes (p = 0.004). No mortality was recorded. A reduction in classical monocytes HLA-DR expression with lower frequencies of monocytes producing TNF-α and IL-6 during the first week of critical illness, appears to be a good marker of immunoparalysis; these findings relate to an increased risk of nosocomial infection and deleterious outcomes. The increased frequency of non-classical monocytes in patients with sepsis/septic shock is suggestive of a better prognosis.

IgG4-related hepatic inflammatory pseudotumour: could MRI suggest the correct diagnosis?

We report a case of a 62-year-old woman, HIV positive, with a 3-week history of jaundice and elevated cholestatic enzymes. Imaging studies displayed intrahepatic biliary dilatation and a central liver lesion. Pathology described lesions of active cholangitis, lymphoplasmacytic infiltration and fibrosis, suggesting a hepatic inflammatory pseudotumour (IPT) IgG4 related. IgG4-related lymphoplasmacytic form of IPT belongs to IgG4-related diseases. We discuss the importance to include IgG4-related hepatic IPT as part of the differential diagnosis of any liver lesion, highlighting potential imaging clues that may help in establishing the correct diagnosis.

Spontaneous regression of hepatocellular carcinoma: myth or reality?

We present a case of a 68-year-old man with chronic hepatitis C infection, with no evidence of chronic liver disease during the first years of follow-up, diagnosed with a hepatocellular carcinoma (HCC) with 40 mm (α-fetoprotein (AFP) 205 ng/mL). He underwent segmental liver resection and pathology analysis was consistent with HCC and cirrhosis in the adjacent liver. Four months after surgery, AFP raised up to 126 661 ng/mL and abdominal MRI revealed a multinodular HCC. Patient rejected treatment with sorafenib and started megestrol and an herbal medicine, soursop (Annona muricata). Six months later, AFP markedly decreased (28 ng/mL) and abdominal MRI showed decreasing size and number of lesions. At 5 years of follow-up, he has no evidence of HCC. Spontaneous regression of HCC is a rare condition and the underlying mechanism is unclear. In this case there is a temporal relation between the start of megestrol and Annona muricata and HCC regression.

Anticoagulation in Cirrhosis and Portal Vein Thrombosis Is Safe and Improves Prognosis in Advanced Cirrhosis.

BACKGROUND: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. AIMS: We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. METHODS: Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. RESULTS: Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1-146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01-1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05-1.19, p < 0.001). CONCLUSIONS: Although AT did not improve overall OLT-free survival, it was associated with higher survival in advanced cirrhosis. Anticoagulation increased PVT recanalization and should be maintained after PVT recanalization to avoid rethrombosis.

An unusual fat-containing presacral tumor in an elderly patient.

The authors present a case of a presacral myelolipoma diagnosed in an 84-year-old male patient with longstanding pelvic pain and past medical history of bladder cancer. Pelvic computed tomography (CT) revealed a well-encapsulated and lobulated presacral mass, with mixed fat and soft-tissue attenuation. Magnetic resonance (MR) imaging provided further confirmation of macroscopic intralesional fat and excluded either adjacent bone invasion or bladder cancer recurrence. A presacral myelolipoma was suspected based on imaging findings, with liposarcoma and teratoma having also been considered for the differential diagnosis. The histological confirmation of the tumor was only attained postoperatively. This case report alerts to the possible presacral location of myelolipomas, which should be considered for every fat-containing lesion detected in this region. The main clinical, imaging, and differential diagnoses of this entity are reviewed in this paper.

Distribution and quantitation of skin iron in primary haemochromatosis: correlation with total body iron stores in patients undergoing phlebotomy.

Measurement of the concentration of iron in the skin, if correlated with total body iron stores, may enable better informed decisions on when to initiate, change or stop therapy in hereditary heamochromatosis. Naïve haemochromatosis patients with iron overload and with C282Y and/or H63D HFE mutations were evaluated at the following time-points: disease diagnosis, end of the therapy programme, and 6 months after the end of therapy. The distribution and concentration of iron in the skin were assessed by quantitative nuclear microscopy methods, in parallel with serum and plasma iron concentration. Iron content in the liver was determined by nuclear magnetic resonance. Iron accumulated in the epidermis; its concentration increased from outer to inner layers, being maximal in the basal layer (7.33 ± 0.98 µmol/g). At all 3 time-points, most of the iron was associated with the extracellular space. During the phlebotomy programme the iron content of the skin and the liver decreased by a factor of 2. These data suggest that measurements of iron concentration in the epidermis, which is a readily accessible tissue, reflect iron overload in the liver.

Tratamento conservador do pé torto congênito idiopático pela técnica de Ponseti / Conservative treatment of idiopathic clubfoot Ponseti's method

O pé torto congênito idiopático é uma patologia frequente, e seu tratamento padrão tem como início as manipulações e gessos seriados, seguidos de liberação cirúrgica póstero-medial extensa nos casos não corrigidos. Ponseti defende a possibilidade de se evitar a cirurgia em cerca de 89 por cento dos casos, através de sua técnica de manipulação que difere das outras descritas anteriormente. Essa forma de tratamento tem tido destaque nos trabalhos atuais devido à superioridade de seus resultados a longo prazo, com maior mobilidade e flexibilidade dos pés, menos dor e bom aspecto estético. Descreve-se a técnica, suas indicações e resultados

A artroplastia parcial e total do quadril. Sua evolução e a experiência no Hospital da Beneficência Portuguesa de São Paulo / The evolution and experience with partial and total hip replacement performed at the Beneficência Portuguesa Hospital of São Paulo

Os autores descrevem as próteses parcial e totais dos quadris, mostrando sua evolução até os dias atuais. A artroplastia parcial foi utilizada desde a década de sessenta (60) com resultados satisfatórios para a época. A partir de 1979 iniciou-se o emprego da artroplastia total cimentada e em 1985 as próteses não cimentadas tomaram posição definitiva e efetiva até nossos dias. Analisam os resultados obtidos com as próteses não cimentadas do quadril do tipo A.M.L. que vem sendo feitas desde 1985 na Beneficência Portuguesa de São Paulo, com excelentes resultados. os autores contam com mais de 3.000 cirurgias de artroplastias totais dos quadris já realizadas (cimentadas e não cimentadas), obtendo assim ampla experiência nesse campo da especialidade ortopédica

Luxaçao simultânea fechada interfalangeana em um dedo: relato de caso e revisäo bibliográfica / Simultaneous closed dorsal interphalangeal dislocation in one finger: report of a case and review literature

Os autores mostram um caso de luxacao simultanea fechada dorsal em um unico dedo, que ocorreu com um goleiro de futebol de salao. Esta e uma rara patologia e esta publicacao e a primeira relatada no Brasil

Cirurgia de Bristow-Latarjet na luxaçäo recidivante anterior do ombro: estudo de dez casos operados / Bristow-Latarjet's surgery in the anterior recurrent luxation of the shoulder: study of 10 operated cases

Os autores estudaram 10 casos de luxacao recidivante anterior do ombro, operados pela tecnica de Bristow-Latarjet. Obtiveram 90% de bons resultados, levando em consideracao a estabilidade articular e a volta as atividades fisicas habituais. Nao tiveram nenhuma recorrencia ou complicacao

Medida dos angulos de anteversao e inclinacao do componente acetabular na artroplastia total do quadril: estudo experimental / Measurement of inclination and anteversion of angle on acetabular component in total hip arthroplasty: experimental stydy

Os autores, atraves de medidas obtidas de um modelo experimental propoem o uso da anteversao planar, simplificando as medidas da anteversao ou retroversao do componente acetabular na artroplastia total do quadril

Fratura-luxaçäo da coluna torácica sem lesäo neurológica causando obstruçäo esofagiana extrínseca: relato de caso / Fracture-luxation of the thoracic spine with no neurologic lesion causing an extrinsic obstruction of the esophagus: a case report

Os autores apresentam um caso de fratura-luxaçäo ao nível da coluna tóracica em lesäo neurológica, diagnosticada pelas radiografias simples ântero-posterior e oblíqua, em que se apresentou complicaçäo rara com obstruçäo extrínseca do esôfago. O achado evidenciou-se pelo exame radiográfico contrastado, pela tomografia axial computadorizada e pela ressonância nuclear magnética. Os autores comentam as dificuldades diagnósticas da lesäo e a importância dos exames subsidiários

Tratamento cirúrgico tardio da fratura-luxaçäo de bacia com hemiascensäo da bacia / Late surgical treatment of fracture-dislocation of the pelvis with pelvic hemilifting

Propöe-se uma abordagem cirúrgica para tratamento da seqüela da fratura-luxaçäo com hemiascensäo da bacia. A consolidaçäo ocorrera viciosamente e com encurtamento de 4,5cm do membro inferior esquerdo. Após dois meses, foi proposta ao paciente a cirurgia, que constou da disjunçäo com osteotomia do ilíaco, da osteotomia do púbis e osteotomia do ísquio. Em seguida, foi instalada traçäo esquelética no fêmur, para que houvesse descenso da hemibacia esquerda. A articulaçäo sacroilíaca, após 30 dias de traçäo, achava-se reduzida, ocorrendo assim pleno êxito dessa cirurgia, muito pouco utilizada na prática ortopédica