Undifferentiated pleomorphic sarcoma of the orbital region: a case report.

Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

Synchronous conjunctival melanoma and lentigo maligna melanoma

Abstract: Lentigo maligna has an extensive and neoplastic character. It typically progresses slowly and may eventually develop into an invasive melanoma, which is called lentigo maligna melanoma. Ocular melanoma is the second most common type of melanoma. The uvea is the most common site of origin of ocular melanomas, while conjunctival melanoma accounts for about 1-5% of cases. In this article, we describe a rare case of synchronic conjunctival melanoma and lentigo maligna on the face.

[Periorbital liposarcoma in pediatric patient: a case report]. / Lipossarcoma periorbital em paciente pediátrico: relato de caso.

The purpose of this study is report a child with periorbital liposarcoma describing the clinical, epidemiological and therapeutic aspects. Six-months-old female baby with increasing tumor in the right fronto-zigomatic region wich was submitted to excision and the patologic and immunohistochemistry analisys observed typical findings of lipoblastoma. After that, there were three tumors relapse with the same diagnosis. One year after the last surgery there was a recurrence of the tumor but at this time the diagnosis was lipossarcoma and the patient was referred for additional treatment wilth radiotherapy and chemotherapy no new injuries so far Due to its rarity, liposarcoma usually does not enter the differencial diagnosis in the patients with orbital masses, however because of its local aggressiveess, it's vital the early identification and treatment to provide better therapeutic results and quality of life.

Lipossarcoma periorbital em paciente pediátrico: relato de caso / Periorbital liposarcoma in pediatric patient: a case report

O objetivo desse estudo é descrever uma criança com lipossarcoma periorbital, caracterizando seus aspectos clínico-epidemiológicos e terapêuticos. Menina de 6 meses de idade com tumoração crescente há dois meses em região fronto-zigomática direita, a qual foi submetida à exérese e cujas análises anatomopatológica (AP) e imuno-histoquímica (IH) observaram achados típicos de lipoblastoma. Após isso, apresentou mais três recidivas tumorais com diagnósticos similares. Um ano depois da última cirurgia, houve nova recorrência, porém, dessa vez, o resultado dos exames análises anatomopatológica e imuno-histoquímica foi de lipossarcoma, sendo, então, encaminhada para complementar o tratamento com radio e quimioterapia, sem novas lesões até o momento. Devido a sua raridade, geralmente o lipossarcoma não entra no diagnóstico diferencial em pacientes com massas orbitais, porém, por ser localmente agressivo, torna-se vital a pronta identificação e tratamento de forma a oferecer melhores resultados terapêuticos e influência sobre a qualidade de vida do paciente.

The purpose of this study is report a child with periorbital liposarcoma describing the clinical, epidemiological and therapeutic aspects. Six-months-old female baby with increasing tumor in the right fronto-zigomatic region wich was submitted to excision and the patologic and immunohistochemistry analisys observed typical findings of lipoblastoma. After that, there were three tumors relapse with the same diagnosis. One year after the last surgery there was a recurrence of the tumor but at this time the diagnosis was lipossarcoma and the patient was referred for additional treatment wilth radiotherapy and chemotherapy no new injuries so far Due to its rarity, liposarcoma usually does not enter the differencial diagnosis in the patients with orbital masses, however because of its local aggressiveess, it's vital the early identification and treatment to provide better therapeutic results and quality of life.

Canaliculite crônica supurativa - aspectos clínicos e terapêuticos: relato de 3 casos / Chronic suppurative canaliculitis - clinical and therapeutic aspects: report of 3 cases

A canaliculite lacrimal é uma afecção rara, cujo principal agente etiológico é o Actinomyces israelii. Ela deve ser considerada como um diagnóstico diferencial nos casos de conjuntivite crônica recorrente. O objetivo deste estudo é relatar 3 casos de pacientes com canaliculite crônica supurativa e diferentes formas de tratamentos. O primeiro paciente apresentou uma canaliculite superior esquerda e foi tratado com uma canaliculotomia. Entretanto, o mesmo desenvolveu uma canaliculite inferior ipsilateral após 6 meses e foi submetido a um esquema de injeção intracanalicular de cefazolina fortificada com resultado satisfatório. O segundo paciente apresentou uma canaliculite inferior esquerda e foi tratado com uma canaliculotomia. O terceiro paciente teve uma canaliculite inferior esquerda e foi submetido a um esquema de injeção intracanalicular de cefazolina fortificada. Ambos obtiveram completa resolução dos sintomas e sinais. O presente estudo demonstra que a irrigação intracanalicular de cefazolina fortificada pode ser uma forma útil de tratamento de canaliculite crônica supurativa com sintomatologia mais branda. O maior benefício desta abordagem é evitar o traumatismo cirúrgico da canaliculotomia.

Lacrimal canaliculitis is a rare disease caused mainly by Actinomyces israelii. It should be regarded as a differential diagnosis of recurrent chronic conjunctivitis. The purpose of this study was to report 3 cases of chronic suppurative canaliculitis and different treatment options. The first patient presented with an upper left canaliculitis and was treated with a canaliculotomy. Nevertheless, he had an ipsilateral lower canaliculitis after 6 months and underwent intracanalicular injections of fortified cefazolin with complete remission. The second patient presented with a lower left canaliculitis and underwent a canaliculotomy. The third patient had a lower left canaliculitis and underwent intracanalicular injections of fortified cefazolin. Both achieved complete remission. The present article demonstrated that intracanalicular irrigation of fortified cefazolin may be a helpful treatment of chronic suppurative canaliculitis with mild symptoms and signs. The most important benefit of this approach is to avoid injury to the lacrimal canaliculus.

[Chronic suppurative canaliculitis - clinical and therapeutic aspects: report of 3 cases]. / Canaliculite crônica supurativa - aspectos clínicos e terapêuticos: relato de 3 casos.

Lacrimal canaliculitis is a rare disease caused mainly by Actinomyces israelii. It should be regarded as a differential diagnosis of recurrent chronic conjunctivitis. The purpose of this study was to report 3 cases of chronic suppurative canaliculitis and different treatment options. The first patient presented with an upper left canaliculitis and was treated with a canaliculotomy. Nevertheless, he had an ipsilateral lower canaliculitis after 6 months and underwent intracanalicular injections of fortified cefazolin with complete remission. The second patient presented with a lower left canaliculitis and underwent a canaliculotomy. The third patient had a lower left canaliculitis and underwent intracanalicular injections of fortified cefazolin. Both achieved complete remission. The present article demonstrated that intracanalicular irrigation of fortified cefazolin may be a helpful treatment of chronic suppurative canaliculitis with mild symptoms and signs. The most important benefit of this approach is to avoid injury to the lacrimal canaliculus.

Ten-year follow-up of a case series of primary epithelial neoplasms of the lacrimal gland: clinical features, surgical treatment and histopathological findings.

PURPOSE: To describe and analyze the features of a cases series of patients with primary epithelial neoplasms of the lacrimal gland, its surgical treatment, and histopathological findings. METHODS: Retrospective evaluation of files from patients with primary epithelial neoplasms of the lacrimal gland in the period from 1997 to 2007. All patients with primary epithelial tumors of the lacrimal gland were included in this study. Data on gender, age, clinical features, surgical treatment, histopathological findings and follow-up were collected. The slides with histological sections of the tumors were reviewed by the same pathologist. RESULTS: During the study period, there were 12 patients, 5 (41.7%) with benign tumors, all pleomorphic adenomas (benign mixed tumor) and 7 (58.3%) with malignant neoplasms, thus distributed: four cases of adenoid cystic carcinoma, two of mucoepidermoid carcinoma and one carcinoma expleomorphic adenoma. Globally, patients mean age was 54.1 years-old (ranging from 14 to 70 years-old), with mean age of 52.4 years-old (ranging from 14 to 65 years-old) for benign neoplasms, and 55.3 years-old for malignant neoplasms (ranging from 26 to 70 years-old). Clinical follow-up information, ranging from 2 to 10 years-old, was available for all patients. Three patients developed distant metastasis and died of disease. CONCLUSIONS: The most frequent primary epithelial neoplasms of the lacrimal gland were pleomorphic adenoma and adenoid cystic carcinoma during the study period. Malignant tumors were more frequent than benign tumors. The histopathological diagnosis and the disease initial stage can play a significant role in patient's survival.

Ten-year follow-up of a case series of primary epithelial neoplasms of the lacrimal gland: clinical features, surgical treatment and histopathological findings / Dez anos de acompanhamento de uma série de casos de neoplasias epiteliais primárias da glândula lacrimal: características clínicas, tratamento cirúrgico e achados histopatológicos

PURPOSE: To describe and analyze the features of a cases series of patients with primary epithelial neoplasms of the lacrimal gland, its surgical treatment, and histopathological findings. METHODS: Retrospective evaluation of files from patients with primary epithelial neoplasms of the lacrimal gland in the period from 1997 to 2007. All patients with primary epithelial tumors of the lacrimal gland were included in this study. Data on gender, age, clinical features, surgical treatment, histopathological findings and follow-up were collected. The slides with histological sections of the tumors were reviewed by the same pathologist. RESULTS: During the study period, there were 12 patients, 5 (41.7 percent) with benign tumors, all pleomorphic adenomas (benign mixed tumor) and 7 (58.3 percent) with malignant neoplasms, thus distributed: four cases of adenoid cystic carcinoma, two of mucoepidermoid carcinoma and one carcinoma expleomorphic adenoma. Globally, patients mean age was 54.1 years-old (ranging from 14 to 70 years-old), with mean age of 52.4 years-old (ranging from 14 to 65 years-old) for benign neoplasms, and 55.3years-old for malignant neoplasms (ranging from 26 to 70 years-old). Clinical follow-up information, ranging from 2 to 10 years-old, was available for all patients. Three patients developed distant metastasis and died of disease. CONCLUSIONS: The most frequent primary epithelial neoplasms of the lacrimal gland were pleomorphic adenoma and adenoid cystic carcinoma during the study period. Malignant tumors were more frequent than benign tumors. The histopathological diagnosis and the disease initial stage can play a significant role in patient's survival.

OBJETIVO: Descrever e analisar as características de uma série de casos de portadores de neoplasias epiteliais primárias da glândula lacrimal, o tratamento cirúrgico, assim como os achados histopatológicos. MÉTODOS: Avaliação retrospectiva dos arquivos de pacientes com neoplasias epiteliais primárias da glândula lacrimal, no período de 1997 até 2007. Todos os pacientes com tumores epiteliais primários da glândula lacrimal foram incluídos neste estudo. Foram analisados os dados sobre sexo, idade, características clínicas, tratamento cirúrgico, achados histopatológicos e seguimento dos pacientes. As lâminas com secções histológicas dos tumores foram revisadas pelo mesmo patologista. RESULTADOS: No período do estudo, foram encontrados 12 pacientes, sendo 5 (41,7 por cento) portadores de tumores benignos, todos adenomas pleomórficos (tumor benigno misto), e 7 (58,3 por cento) com neoplasias malignas, assim distribuídos: quatro casos de carcinoma adenóide cístico, dois de carcinoma mucoepidermóide e um de carcinoma ex-adenoma pleomórfico. Analisando-se de modo global, a idade média dos portadores foi de 54,1 anos (variando de 14 a 70 anos); com média de idade de 52,4 anos (variando de 14 a 65 anos) para neoplasias benignas, e 55,3 para neoplasias malignas (variando de 26 a 70 anos). Informações do seguimento, variando de 2 a 10 anos, estavam disponíveis para todos os pacientes. Três pacientes desenvolveram metástases distantes e morreram devido à doença. CONCLUSÕES: A maioria das neoplasias epiteliais primárias da glândula lacrimal foi o adenoma pleomórfico e o carcinoma adenóide cístico no período de estudo. Os tumores malignos foram mais frequentes que os benignos. O diagnóstico histopatológico e o estadiamento inicial da doença podem desempenhar uma papel significante na sobrevida do paciente.

Dacrioadenite bilateral causada pelo vírus Epstein-Barr: relato de caso / Epstein-Barr virus bilateral dacryoadenitis: case report

Os autores apresentam um caso raro de dacrioadenite aguda bilateral associada à mononucleose. Paciente do sexo feminino, 27 anos, tinha aumento agudo da glândula lacrimal, bilateral, com sinais flogísticos. As alterações tomográficas e laboratoriais confirmaram o diagnóstico e o tratamento com esteróide sistêmico foi introduzido, com resolução completa dos sinais e sintomas. COMENTÁRIOS: A dacrioadenite é uma apresentação incomum da mononucleose infecciosa e pode minimizar outras manifestações desta doença. O diagnóstico de mononucleose deve ser considerado em casos de dacrioadenite aguda bilateral. Os esteróides sistêmicos contribuem para a resolução do quadro inflamatório.

The authors present a rare case of acute bilateral dacryoadenitis in association with infectious mononucleosis. A 27 year-old woman had acute bilateral lacrimal gland enlargement with inflammatory signs. The CT scan findings and laboratorial investigations confirmed the diagnosis of infectious mononucleosis. The systemic treatment with intravenous steroids was initiated leading to complete remission of the signs and symptoms. COMMENTS: Dacryoadenitis is an uncommon manifestation of infectious mononucleosis and may minimize other signs of the disease. Mononucleosis has to be considered in acute dacryoadenitis cases. Systemic steroids contribute to the fast regression of inflammatory condition.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report.

The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.

[Malignant fibrous histiocytoma of the eyelid: case report]. / Fibro-histiocitoma maligno da pálpebra: relato de caso.

Fibrous histiocytomas represent 1% of all orbital masses and may be classified into benign, locally aggressive and malignant - these representing nearly 11% of the total. In this study, the authors describe an unusual case of malignant fibrous histiocytoma of the eyelid, discussing clinical and histological features of this neoplasm.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report / Plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo: relato de caso

The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.

Os autores relatam um raro caso de plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo em uma paciente de 42 anos. A lesão foi removida cirurgicamente e o exame anatomopatológico, aliado à imuno-histoquímica, revelou positividade para imunoglobulina G e cadeias leve tipo Kappa. Após extensa avaliação sistêmica, nenhuma anormalidade foi observada, sendo feito o diagnóstico de plasmocitoma solitário ósseo. A paciente foi submetida à radioterapia, permanecendo livre da doença por 5 anos e 6 meses até 2007, quando apresentou uma fratura patológica devido à infiltração plasmocitária na coluna lombar, sendo feito diagnóstico de mieloma múltiplo. Uma vez que o plasmocitoma solitário ósseo pode ser a primeira manifestação do mieloma múltiplo, esses pacientes necessitam de adequado diagnóstico e seguimento a longo prazo, pois o tratamento e prognóstico dessas duas condições é diferente.

Fibro-histiocitoma maligno da pálpebra: relato de caso / Malignant fibrous histiocytoma of the eyelid: case report

Os fibro-histiocitomas representam 1 por cento das massas orbitárias e podem ser histologicamente classificados em benignos, localmente agressivos e malignos - os últimos representando aproximadamente 11 por cento do total. Nesse estudo, os autores descrevem um caso raro de fibro-histiocitoma maligno de pálpebra, ressaltando as características clínicas e histopatológicas desta neoplasia.

Fibrous histiocytomas represent 1 percent of all orbital masses and may be classified into benign, locally aggressive and malignant - these representing nearly 11 percent of the total. In this study, the authors describe an unusual case of malignant fibrous histiocytoma of the eyelid, discussing clinical and histological features of this neoplasm.

[Epstein-Barr virus bilateral dacryoadenitis: case report]. / Dacrioadenite bilateral causada pelo vírus Epstein-Barr: relato de caso.

UNLABELLED: The authors present a rare case of acute bilateral dacryoadenitis in association with infectious mononucleosis. A 27 year-old woman had acute bilateral lacrimal gland enlargement with inflammatory signs. The CT scan findings and laboratorial investigations confirmed the diagnosis of infectious mononucleosis. The systemic treatment with intravenous steroids was initiated leading to complete remission of the signs and symptoms. COMMENTS: Dacryoadenitis is an uncommon manifestation of infectious mononucleosis and may minimize other signs of the disease. Mononucleosis has to be considered in acute dacryoadenitis cases. Systemic steroids contribute to the fast regression of inflammatory condition.

Lagochilascariasis leading to severe involvement of ocular globes, ears and meninges.

A case report of a 31 year-old woman from Paraíba State (North-Eastern Brazil) that presented severe involvement of ocular globes, ears and meninges. Diagnosis was established after enucleation of her left eye, when adult worms were seen in the midst of a granulomatous inflammatory process. Her response to the initial treatment with levamisole and cambendazole was good, but there was a relapse after the fifth month of treatment even with maintenance doses of both medications. She later received ivermectin and albendazol and responded well.

Tumor neuroectodérmico primitivo periférico primário da órbita: relato de caso / Primary peripheral primitive neuroectodermal tumour of the orbit: case report

Os autores relatam o primeiro caso de tumor neuroectodérmico primitivo periférico primário da órbita apresentado na literatura nacional. Características clínicas, radiológicas e histopatológicas são discutidas. O diagnóstico foi confirmado por meio de análise imuno-histoquímica, etapa essencial no diagnóstico dos tumores de pequenas células redondas.

The authors present a case of primary peripheral primitive neuroectodermal tumor of the orbit in a 10-month-old girl, which is the first case in the Brazilian literature. Clinical, radiologic and histopathologic features are discussed. The diagnosis was confirmed by immunohistochemical analysis which is essential to the diagnosis of small round cell tumors in the orbit.

Lagochilascariasis leading to severe involvement of ocular globes, ears and meninges / Infecção humana por Lagochilascaris minor com envolvimento ocular, auditivo e das meninges

A case report of a 31 year-old woman from Paraíba State (North-Eastern Brazil) that presented severe involvement of ocular globes, ears and meninges. Diagnosis was established after enucleation of her left eye, when adult worms were seen in the midst of a granulomatous inflammatory process. Her response to the initial treatment with levamisole and cambendazole was good, but there was a relapse after the fifth month of treatment even with maintenance doses of both medications. She later received ivermectin and albendazol and responded well.

Paciente do sexo feminino, com 31 anos, procedente da Paraíba, apresentava envolvimento severo de ambos os globos oculares, com perda da visão à esquerda, comprometimento da audição e das meninges. Após enucleação do olho esquerdo fragmento de helminto identificado como Lagochilascaris minor foi observado em processo inflamatório granulomatoso. Inicialmente a paciente foi tratada com levamisol e cambendazol, com bom resultado. Verificou-se, todavia, piora do quadro após cinco meses, com eliminação de larvas do ascarídeo em lesão presente na órbita esquerda, embora a medicação fosse mantida com administração periódica. Houve boa resposta terapêutica, com regressão do quadro, após substituição dos anti-helmínticos anteriores pela associação ivermectina e albendazol.

[Primary peripheral primitive neuroectodermal tumour of the orbit: case report]. / Tumor neuroectodérmico primitivo periférico primário da órbita: relato de caso.

The authors present a case of primary peripheral primitive neuroectodermal tumor of the orbit in a 10-month-old girl, which is the first case in the Brazilian literature. Clinical, radiologic and histopathologic features are discussed. The diagnosis was confirmed by immunohistochemical analysis which is essential to the diagnosis of small round cell tumors in the orbit.

Epífora congênita nos pacientes com síndrome de Down / Congenital epiphora in patients with Down syndrome

OBJETIVO: Identificar as causas de epífora congênita em pacientes com síndrome de Down. MÉTODOS: Foram analisados os prontuários de 695 pacientes com epífora congênita, atendidos no Ambulatório de Vias Lacrimais da Clínica Oftalmológica da Santa Casa de Misericórdia de São Paulo, de outubro de 1989 a julho de 2005. Todos foram previamente submetidos a exame oftalmológico completo e apresentavam como queixa principal epífora e/ou secreção ocular constante, uni ou bilateral, desde o nascimento. Os pacientes foram divididos em: grupo A, 30 pacientes com síndrome de Down, e grupo B, 665 pacientes controle. A avaliação das vias lacrimais foi realizada com a prova de irrigação sob anestesia geral. RESULTADOS: Os grupos A e B são semelhantes estatisticamente quanto à idade (p=0,07), sexo (p=0,63) e raça (p=0,68). As queixas bilaterais foram mais freqüentes no grupo A (p=0,0008). A obstrução anatômica das vias lacrimais foi encontrada em 32,73 por cento do grupo A e em 85,51 por cento do grupo B (p<0,0001). CONCLUSÃO: A maioria das causas de epífora congênita em pacientes com síndrome de Down é decorrente de bloqueio funcional da bomba lacrimal. A hipotonia da musculatura palpebral e as alterações palpebrais, comuns à síndrome de Down, são as principais hipóteses para essa disfunção, mas são necessários estudos posteriores para confirmação.

PURPOSE: To describe the causes of congenital epiphora in patients with Down syndrome. METHODS: Retrospective study of 695 patients with congenital epiphora, of the Lacrimal Sector of the Department of Ophthalmology, São Paulo "Santa Casa", Brazil, between October 1998 and July 2005. This study analyzed: the main symptom of continuous epiphora or mucous discharge, which affected one or both eyes, since birth. Subjects were separated in to two groups: group A, with 30 patients with Down syndrome and group B, with 665 control patients. The lacrimal evaluation was performed by the throw irrigation test after general anesthesia. RESULTS: Both groups were statistically similar regarding age (p=0.07), sex (p=0.63) and race (p=0.68). Bilateral symptoms were more frequent in group A (p=0.0008). Anatomic obstruction of the lacrimal canal was present in 32.73 percent of group A and in 85.51 percent of group B (p<0.0001). CONCLUSIONS: The most frequent cause of congenital epiphora in Down syndrome is due to functional block of the lacrimal pump. Eyelid muscle hypotony and eyelid changes, common in Down syndrome, is the main hypothesis of lacrimal dysfunction, but further studies will be necessary for confirmation.