Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

Espessamento do tendão muscular na oftalmopatia de Graves / Muscle tendon enlargment in Graves´ophthalmopathy

OBJETIVO: Estudar a frequência do espessamento do tendão muscular em pacientes com oftalmopatia de Graves buscando estabelecer correlações com as diferentes formas clínicas da doença. A diversidade clínica e laboratorial na oftalmopatia de Graves pode levar à confusão quanto ao diagnóstico, conduta e prognóstico. Os achados radiológicos variam desde o aumento isolado do tecido adiposo até o espessamento da musculatura extraocular, caracteristicamente poupando os tendões. Em 2004,no entanto, Ben Simon descreveu o espessamento do tendão muscular na oftalmopatia de Graves. MÉTODOS: Foram avaliados 20 pacientes, de ambos os sexos, com idades entre 20 e 80 anos, com formas clínicas designadas como :forma benigna (retração palpebral),forma intermediária (diplopia na posição primária do olhar) e forma maligna ou infiltrativa (sinais de comprometimento do nervo óptico).Todos os pacientes foram submetidos à tomografias computadorizadas de órbita. Os pacientes estavam eutiroideanos, há pelo menos um ano. Os padrões tomográficos foram estudados e divididos em dois grupos: com e sem espessamento dos tendões da musculatura extraocular. RESULTADOS: Uma relação estatisticamente significativa entre espessamento do tendão e a forma clínica intermediária foi encontrada (p <0,012). CONCLUSÃO: O espessamento do tendão extraocular, encontrado em 30 por cento dos pacientes com oftalmopatia de Graves, relaciona-se positivamente com a forma intermediária da doença, caracterizada pela presença de diplopia e que constitui um achado de grande valor clínico.

PURPOSE: The aim is therefore to study the frequency of tendon enlargment in Graves' ophthalmopathy, seeking to estabilish its clinical correlations. Clinical and laboratory diversity in Graves' Ophthalmopathy sometimes may mislead its diagnosis. Radiological findings are more reliable for the diagnosis of Graves' Ophthalmopathy. Since then, a number of patterns have been described. Extraocular muscle involvement in this pathology is considered as always sparing the tendons. In 2004, Ben Simon described extraocular muscles tendon enlargment in some patients with diplopia in Graves' orbitopathy. METHODS: 20 patients, aged between 20 and 80 years, of both sexes, designated as benign (eyelid retraction), intermediate(diplopia in primary sight position) and malignant or infiltrative(signs of optic nerve compromise) forms were evaluated by orbital tomography. All patients had already been euthyroidean for at least one year. Tomographic patterns were studied and divided into two groups: with or without extraocular muscle tendons enlargment. RESULTS: Statistically significant relationship was found between tendon enlargment and intermediate form (p<0.012). CONCLUSION: Extraocular tendon involvement present in 30 percent of the patients with Graves' ophthalmopathy, and is positively correlated to intermediary form of the disease, characterized by diplopia, a very important clinical landmark.

Disease spectrum in children presenting with Proptosis

The aim of this study was to provide the reader an overview of the subject of childhood proptosis with an emphasis on the systematic and practical approach for the work-up of proptosis in children. All children with proptosis who presented to the department of Paediatric Ophthalmology of The Children's Hospital and Institute of Child Health Lahore were included in the study from January 2004 to December 2005. A total of 64 patients were evaluated. There were 40 males and 24 females. Tumors were the most common [42.18%] cause of proptosis. Out of these vascular tumors, retinoblastoma and optic nerve gliomas were on the top of the list, followed by inflammatory diseases [28.15%]. Detailed history and clinical examination remain the useful tool to reach the correct diagnosis. Radio imaging plays an important role in coming to a diagnosis. Tumors form the most common cause of proptosis in our study followed by inflammatory lesions. A systematic and multidisciplinary approach is mandatory in managing a child presenting from proptosis

Retinoblastoma in Upper Egypt [1981-1991]

We conducted a retrospective study on 78 children with retinoblastoma who were admitted, diagnosed and treated at the Ophthalmology Department of Assiut University Hospital [AUH] in the period between October 1981 and November 1991. Forty-seven children were male and 31 female, forming a male/female ratio of 3:2. The tumor was unilateral in 68 [87%] and bilateral in 10 children [13%]. Retinoblastoma was primary in 67 [86%] and recurrent in 11 cases [14%]. The mean age at diagnosis in males with unilateral and bilateral retinoblastoma was 36 months and 17 months respectively, while it was 33 months in females with unilateral tumors. Bilateral retinoblastoma was seen in only two females, aged 3 and 4 years. The first presenting symptoms were leukokoria [44.9%] and proptosis [39.7%]. Echography showed a solid mass, with moderate to high refractile echoes, an orbital shadowing, and normal axial length of the globe. Retinoblastoma was treated by primary enucleation [50.0%], exenteration [16.7%], irradiation [3.8%], and chemotherapy [2.6%]; in 25.6% of cases the parents refused treatment. The late presentation by the patients, and the deficiency in specialized centers for the management of retinoblastoma in our region of the world worsen the prognosis, both for life and for vision

Evaluation of proptosis by computed tomography

Computed tomography [CT] was done for 30 patients complaining from proptosis whether unilateral or bilateral to assess its reliability in differentiating its causes and extent. Other radiological methods, histopathology and surgery were used for confirmation in addition to the clinical data. Three groups of retrobulbar, paraorbital and global lesions were defined. The CT was found to be an essential diagnostic tool for the detection of the global and orbital lesions, while it had to be combined with plain x-ray in dealing with parorbital lesions

Role of positive contrast orbitography in diagnosis of unilateral proptosis

The success of surgical removal of orbital tumours depends on pre-operative appraisal of the site, size and nature of the mass. Accurate clinical evaluation may be impossible in deeply seated retro-bulbar lesions, and even in superficial lesions with possible posterior extenion. Knowledge gained from plain radiography of the orbital space occupying lesions has its limitations. The changes that can be seen are; soft tissue shadow, bony erosions, osteoporosis, sclerosis or changes in the size of the orbit or orbital foramen. These suggestive changes were seen in 69% of the cases [Pfeiffer, 1943]. Most authors gave lower figures for diagnostic data gained from plain radiography; 46% by Mortada [1968] and 41% by Labib et al. [1970]. Those changes, mentioned above, are not specific and cannot indicate the exact site, size or nature of the tumour [Lombardi, 1971], particularly in cases of angioma of the orbit [Labib et aI., 1970]. The aim or this paper is to discuss the role of positive contrast orbitography in diagnosis and management of orbital space-occupying lesions