Uso terapéutico de la hialuronidasa y la triamcinolona en el pseudotumor orbitario / Therapeutic use of hyaluronidase and triamcinolone in orbital pseudotumor

RESUMEN El origen del pseudotumor orbitario no es del todo conocido. Se admite su naturaleza inflamatoria granulomatosa e inespecífica en diferentes localizaciones. El pseudotumor orbitario se define como una respuesta inflamatoria celular pleomórfica, que está usualmente confinado a estructuras de la órbita y tiene una evolución limitada. En este trabajo se presenta una paciente femenina de 16 años, con diagnóstico de pseudotumor orbitario corroborado por biopsia y tomografía axial computarizada, refractaria al tratamiento con esteroides sistémicos, por lo que se decide iniciar con la aplicación de hialuronidasa y triamcinolona en el espacio peribulbar. Los casos agudos casi siempre responden rápidamente al tratamiento con cortocoesteroides, como prednisona, pero debemos tener en cuenta que existen pacientes que son refractarios al tratamiento, por lo que es necesario buscar procedimientos alternativos. Una opción es el uso de hialuronidasa para destruir las uniones extracelulares, y difundir un esteroide de manera local, como la triamcinolona, más efectiva dentro del tejido inflamatorio para provocar un efecto localizado de este. A los tres meses del tratamiento hubo una regresión total del cuadro en esta paciente(AU)

ABSTRACT The exact etiology of orbital pseudotumor is unknown, but its granulomatous unspecific inflammatory nature at various locations has been recognized. Orbital pseudotumor is defined as a cellular pleomorphic inflammatory response of limited evolution often confined to orbital structures. A case is presented of a female 16-year-old patient diagnosed with orbital pseudotumor confirmed by biopsy and computerized axial tomography, refractory to treatment with systemic steroids, due to which it is decided to start treatment with hyaluronidase and triamcinolone in the peribulbar space. Acute cases often respond fast to treatment with corticosteroids such as prednisone. It should be borne in mind that there are patients who are refractory to treatment for whom alternative treatments should be sought. An option is the use of hyaluronidase to destroy extracellular junctions and locally spread a steroid such as triamcinolone, most effectively within the inflammatory tissue to ensure its localized effect. Total regression of the patient's status was observed at three months of treatment(AU)

Pseudotumor orbital bilateral recorrente / Recorrent bilateral orbits pseudo-tumor

Resumo O pseudotumor orbitário é uma doença inflamatória idiopática benigna. Os autores apresentam um caso manifestado em adolescente de 12 anos, diagnosticado por meio do exame clínico, laboratorial e radiológico. Houve boa resposta ao tratamento proposto com corticosteroides. O relato é seguido de breve retomada literária acerca do tema.

Abstract The orbital pseudotumor is a benign idiopathic inflammatory disease. The authors present a case manifested in 12 years old boy, diagnosed by clinical, laboratory and radiological examination. There was a good response to treatment with corticosteroids proposed. The report is followed by brief literary resume on the subject.

Pseudotumor esclerosante de órbita / Sclerosing orbital pseudotumor

RESUMO O pseudotumor esclerosante de órbita é um subtipo raro de pseudotumor inflamatório idiopático de órbita. É mais comum em adultos e apresenta diagnóstico de exclusão. A primeira linha de tratamento são os esteroides. O subtipo esclerosante apresenta resposta moderada aos esteroides devido à predominância de fibrose e colágeno na histologia. Relatamos o caso de um paciente com diagnóstico histológico de pseudotumor esclerosante de órbita que teve boa resposta ao tratamento com corticoide associado à azatioprina.

ABSTRACT Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.

Multifocal idiopathic fibrosclerosis mimicking tuberculosis of the abdomen.

We present a 13 year old girl from Assam who had been treated as abdominal tuberculosis for 2 years due to the presence of refractory lymphocyte-predominant ascites and multiple small bowel strictures associated with significant anorexia and weight loss. On evaluation she was found to have retroperitoneal fibrosis with hydroureteronephrosis, mediastinal fibrosis and a retro-orbital pseudotumour. Based on these findings the diagnosis of Multifocal Idiopathic Fibrosclerosis (MIFS) was made. Ascites and multiple bowel strictures have been only rarely been described in association with MIFS. The other unique features in this patient were the early age of presentation, the presence of mediastinal fibrosis in association with retroperitoneal fibrosis, extensive soft tissue fibrosis of the neck, axillae and the presence of trismus. In a country like ours where Tuberculosis is commonplace, one would not think twice about treating such a case with antituberculous therapy. However, with a constellation of findings suggestive of a diffuse fibrotic process, MIFS should be an important consideration.©

MR Imaging of Orbital Inflammatory Pseudotumors with Extraorbital Extension

OBJECTIVE: To demonstrate a variety of MR imaging findings of orbital inflammatory pseudotumors with extraorbital extension. MATERIALS AND METHODS: We retrospectively reviewed the MR features of five patients, who were diagnosed clinically and radiologically as having an orbital inflammatory pseudotumor with extraorbital extension. RESULTS: The types of orbital pseudotumors were a mass in the orbital apex (n = 3), diffuse form (n = 2), and myositis (n = 1). The extraorbital extension of the orbital pseudotumor passed through the superior orbital fissure in all cases, through the inferior orbital fissure in two cases, and through the optic canal in one case. The orbital lesions extended into the following areas: the cavernous sinus (n = 4), the middle cranial fossa (n = 4), Meckel's cave (n = 2), the petrous apex (n = 2), the clivus (n = 2), the pterygopalatine fossa and infratemporal fossa (n = 2), the foramen rotundum (n = 1), the paranasal sinus (n = 1), and the infraorbital foramen (n = 1). On MR imaging, the lesions appeared as an isosignal intensity with gray matter on the T1-weighted images, as a low signal intensity on the T2-weighted images and showed a marked enhancement on the post-gadolinium-diethylene triamine pentaacetic acid (post-Gd-DTPA) T1-sequences. The symptoms of all of the patients improved when they were given high doses of steroids. Three of the five patients experienced a recurrence. CONCLUSION: MR imaging is useful for demonstrating the presence of a variety of extraorbital extensions of orbital inflammatory pseudotumors.

Orbital myositis and rheumatoid arthritis: case report

Orbital myositis implies orbital inflammation confined to one or more of the extraocular muscles. The acute form responds well to high doses of oral corticosteroids tapered gradually, but it may recur or become chronic. We describe a 38 years old female who has been suffering from rheumatoid arthritis for six years. She developed diplopia as a result of a paralysis of the right and left rectus medialis muscle. MRI showed inflammatory process and thickness of the referred muscles. The patient had a total recovery with oral use of 80 mg methylpredinisolone daily. Two months after the first episode she developed a bilateral ophthalmoplegy. The patient improved with oral use of steroids the second time, but a paresis of the left rectus lateralis muscle remained. From the 156 cases we reviewed only three have been related to rheumatic diseases and none has been previously related to rheumatoid arthritis.

Enfermedad inflamatoria idiopática de la órbita: (pseudotumor orbitario) / Idiopathic orbital inflammatory disease: (orbitary pseudotumor)

El propósito de este estudio fue la revisión de nuestra experiencia clínica con 24 casos observados de enfermedad inflamatoria idiopática de la órbita entre 1980 y 1996, el cuadro clínico de los pacientes fue evaluado retrospectivamente en la Unidad de Neuro-Oftalmología del Hospital Vargas de Caracas. Pacientes con promedio de edades desde 13 a 76 (media 42,66) años. Los tres signos principales más comunes fueron: Proptosis (74,99 por ciento), Dolor periorbitario (58,33 por ciento), Diplopía (54,16 por ciento). El diagnóstico fue realizado por clínica y estudios complementarios. Patrones imagenológicos fueron observados en 10 casos, siendo la enfermedad inflamatoria difusa la más común, segudo por el Síndrome Tolosa Hunt (3 casos). Dacrioadenitis (3 casos) y periescleritis (1 caso). La respuesta a tratamiento fue conocida para 18 de los 24 casos clasificados imagenológicamente. El tratamiento con altas dosis de corticosteroides fue bueno en 11 de 24 (45,83 por ciento) de los casos. Dos casos resistentes a corticosteroides fueron tratados con radioterapia. Un caso resistente respondió a ciclofosfamida. Tres casos adicionales tuvieron remisión espontánea. Sin embargo, 16 de 24 pacientes (66,67 por ciento) tuvieron una eventual buen resulatdo. En base a estos resultados recomendamos el uso de corticosteroides como tratamiento inicial en pacientes con EIIO

Processos inflamatórios orbitais: avaliaçäo por tomografia computadorizada / Orbital inflamatory processes: computed tomography aspects

Os autores apresentam análise retrospectiva de 20 pacientes com processo inflamatório intra-orbital, estudados no Hospital Universitário Clementino Fraga Filho, da Universidade Federal do Rio de Janeiro. Os processos inflamatórios observados foram a celulite, os pseudotumores e a miosite. Na celulite, os principais achados tomográficos foram o aumento das partes moles e a exoftalmia. Outros achados que ajudam neste diagnóstico säo: velamento dos seios paranasais, velamento da gordura retrobulbar, comprometimento muscular, gás em partes moles e aumento da glândula lacrimal. Nos pseudotumores, ausência de erosäo óssea. Em relaçäo à miosite, encontraram-se proptose do globo ocular e espessamento muscular

Asociación entre síndrome de Parry Romberg o atrofia hemifacial progresiva y enfermedad inflamatoria idiopática de la órbita / Association between The Parry-Rombergïs Syndrome or progressive hemifacial atrophy and idiopathic inflammatory disease of the orbit

Enfermedad inflamatoria idiopática y atrofia hemifacial progresiva o síndrome de Parry-Romberg. La autora comunica el primer caso donde dos entidades de etiología desconocida se presentan en asociación, compartiendo características clínicas e histopatológicas. Se describe el caso de una paciente femenina cuya enfermedad se inicia a los 13 años de edad, con atrofia hemifacial izquierda, sin compromiso óseo, acentuándose en forma progresiva en el tiempo, hasta evidenciarse en la frente el signo "coup de sabre". A los 17 años presenta neuropatía óptica, restricción de la motilidad ocular y edema hemifacial izquierdo, simulando una celulitis orbitaria. Realizados los estudios adecuados y descartándose otras patologías inflamatorias de la órbita, se diagnostica una enfermedad inflamatoria aguda de la órbita, se inicia un tratamiento con prednisona y la respuesta terapéutica es favorable desde las primeras cuarenta y ocho horas, desapareciendo los signos inflamatorios, pero dejando como secuelas una neuropatía óptica residual expresada por la disminución de la agudeza visual, escotoma central y palidez del disco óptico izquierdo