Ambliopia severa na infância relacionada à persistência da vasculatura fetal / Severe childhood amblyopia associated to persistence of fetal vasculature

RESUMO A persistência do vítreo primário hiperplásico, atualmente referida como persistência da vasculatura fetal, é uma anomalia congênita que resulta da não regressão do vítreo vascular primário e do sistema da artéria hialoide durante a embriogênese. Trata-se de uma anomalia unilateral na maioria dos casos, esporádica e comumente não associada a nenhum outro achado sistêmico. Clinicamente, essa condição pode ser classificada em persistência anterior e em persistência posterior da vasculatura fetal. A condição anterior está relacionada ao sistema da artéria ciliar, enquanto a persistência da vasculatura posterior associa-se à artéria hialoide e pode apresentar anormalidades, com desfecho visual desfavorável. A detecção da persistência do vítreo primário hiperplásico é de suma importância, visto que é um diagnóstico diferencial para retinoblastoma. O relato de caso a seguir descreve o acompanhamento ambulatorial em um Serviço de Oftalmologia de uma criança do sexo masculino com persistência da vasculatura fetal unilateral e sem alterações sistêmicas.

ABSTRACT Hyperplastic primary vitreous persistence, currently referred to as fetal vasculature persistence, is a congenital anomaly that results from non-regression of the primary vascular vitreous and hyaloid artery system during embryogenesis. It is a unilateral anomaly in the vast majority of cases, sporadic and commonly not associated with any other systemic finding. Clinically, this condition can be classified into anterior and posterior persistence of fetal vasculature. The anterior condition is related to the ciliary artery system, while the persistence of the posterior vasculature is associated with the hyaloid artery, which may present abnormalities with an unfavorable visual outcome. Detecting persistent hyperplastic primary vitreous is of paramount importance, as it is a differential diagnosis for retinoblastoma. The following case report describes the outpatient follow-up at the ophthalmology service of the Federal University of Triângulo Mineiro (UFTM) of a male child with persistent unilateral fetal vasculature and no systemic changes.

Persistência da vasculatura fetal diagnosticada pela ultrassonografia ­ Relato de caso / Vasculature persistence fetal diagnosed by ultrasound ­ case report

Contexto: A persistência da vasculatura fetal é uma malformação ocular rara em adultos, habitualmente unilateral, sendo uma condição não hereditária, com poucas manifestações sistêmicas e neurológicas. Descrição do caso: Homem de 45 anos queixando-se de dor no olho esquerdo, com pressão intraocular de 56 mmHg. A ultrassonografia do olho esquerdo demonstra aumento da ecogenicidade do cristalino inferindo catarata e redução da amplitude da câmara anterior, membrana posterior hiperecogênica no interior da câmara vítrea com intensa vascularização com fluxo arterial ao estudo com Doppler, caracterizando a persistência da vasculatura fetal. O paciente recebeu tratamento por três dias. Com a redução da pressão intraocular após esse período, realizou a cirurgia combinada de facoemulsificação com implante de lio e implante de tubo de Ahmed. Após o procedimento cirúrgico, o paciente não voltou a apresentar a sintomatologia. Discussão: Clinicamente, há duas condições de doença, dependendo da porção atingida do vítreo primário ­ as formas anterior e posterior. A persistência da vasculatura fetal não tratada frequentemente progride para phthisis bulbi ou enucleação devido a uma hemorragia intraocular recorrente e secundária ao glaucoma. Os esforços cirúrgicos têm o intuito de preservar a visão. Conclusão: Relatamos um caso de persistência da vasculatura fetal diagnosticado pela ultrassonografia e tratado cirurgicamente com sucesso devido ao alívio dos sintomas em olho esquerdo sem percepção luminosa.

Aqueous misdirection syndrome in persistent fetal vasculature (PFV) / Síndrome de mau direcionamento do humor aquoso em persistência da vasculatura fetal (PVF)

ABSTRACT We report a case of a 5-year-old girl with persistent fetal vasculature who presented with unilateral acute angle closure caused by aqueous misdirection syndrome that was apparently associated with regression of cycloplegia. Initial treatment with topical steroids, anti-glaucomatous drops, and atropine showed insufficient control of the intraocular pressure (IOP). Surgical treatment with lensectomy and vitrectomy resulted in satisfactory IOP control and no complications at the 6-month follow-up.

RESUMO Relatamos o caso de uma criança de 5 anos, sexo feminino com persistência do vítreo primário que apresentou crise de fechamento angular por síndrome do mau direcionamento do humor aquoso, aparentemente associado a regressão da cicloplegia. Tratamento inicial com corticoide tópico, colírios antiglaucomatosos e atropina mostraram controle insuficiente da pressão intraocular (PIO). Tratamento cirúrgico com lensectomia e vitrectomia anterior permitiu o controle satisfatório da PIO, sem intercorrências no seguimento de 6 meses.

Clinical Characteristics of Retinoblastoma Patients whose Diagnosis was Difficult due to Atypical Ocular Manifestation

PURPOSE: To report the clinical characteristics of retinoblastoma patients whose diagnosis was difficult due to atypical ocular manifestations. METHODS: Among retinoblastoma patients who were diagnosed and treated from January 1999 to December 2014 at Seoul National University Children's Hospital, 6 patients whose diagnosis was difficult were retrospectively reviewed. Factors including age, sex, family history, initial findings, time to final diagnosis, histopathologic examination, additional treatment, and survival rate were evaluated. RESULTS: Among 6 patients, 5 were male, and the mean age at the initial visit was 32.9 ± 19.1 months. None of the patients had family history, and all presented with unilateral lesion at the initial visit. The initial diagnoses were Coats' disease and uveitis in 2 patients, respectively, and persistent hyperplastic primary vitreous and traumatic hyphema in 1 patient, respectively. During an intensive short-term follow-up of 8.3 ± 5.3 weeks, 2 patients showed malignant cells after external subretinal fluid drainage procedure, and 4 patients demonstrated increasing ocular size or calcification in imaging. These patients received enucleation under suspicion of malignancy and were finally diagnosed with retinoblastoma after histopathologic examination. There were 2 patients with optic nerve involvement, and 3 patients underwent additional systemic chemotherapy. Five patients were followed-up for 7.6 ± 6.3 years after enucleation, and the mean age at final follow-up was 10.6 ± 7.4 years. CONCLUSIONS: Retinoblastoma is one of the diseases in which early diagnosis and treatment are important. However, some cases are difficult to diagnose, even for experienced clinicians. If there are no typical manifestations such as mass or calcification and early findings show retinal detachment, glaucoma, pseudohypopyon, or hyphema, intensive short-term follow-up to exclude retinoblastoma is needed.

Ophthalmic Doppler in persistent hyperplastic primary vitreous atypical presentation: case report / Doppler oftálmico na persistência hiperplásica do vítreo primário de apresentação atípica: relato de caso

ABSTRACTThe purpose is to document a case of persistent hiperplastic primary vitreous (PHPV) with atypical Bergmeister's papilla and patent hialoid artery that had its conclusive diagnosis done by doppler ultrasound. We report a case of child, who had unilateral leucokoria. This child's fundoscopic examination showed a white mass on the optic nerve that extended over to adjacent retina. Performed ultrasound that remained a doubt with the following diagnostic hypotheses: persistent hiperplastic primary vitreous, granuloma (toxocara), astrocytic hamartoma and retinoblastoma. The diagnosis was only established when the doppler ultrasound showed a blood flow inside of the membrane, thus confirming the diagnosis of persistent hiperplastic primary vitreous associated with the Bergmeister'spapilla.

RESUMOO objetivo é documentar um caso de persistência hiperplásica do vítreo primário (PHPV) com papila de Bergmeister atípica e artéria hialóide pérvia que teve seu diagnóstico conclusivo feito pelo exame ultrassonográfico com Doppler colorido. Relatamos um caso de uma criança com leucocoria unilateral, apresentando massa branca sobre o disco óptico observada à fundoscopia, que se estendia à retina adjacente. Após a realização do exame ultrassonográfico foi mantida a dúvida com as seguintes hipóteses diagnósticas: persistência hiperplásica do vítreo primário, granuloma (toxocaríase) e retinoblastoma. O diagnóstico foi estabelecido após a utilização do Doppler colorido que evidenciou fluxo sanguíneo no interior da membrana, confirmando o diagnóstico de persistência hiperplásica do vítreo primário associada à papila de Bergmeister.

Hyperplastic primary vitreous with hemorrhage manifested as a hyperechoic mass in the fetal orbit by prenatal ultrasound in a case of isolated unilateral microphthalmia

Congenital microphthalmia is a rare anomaly of the fetal orbit resulting from developmental defects of the primary optic vesicle. Chromosomal anomalies, genetic defect, infection, and prenatal drug exposure are the most common causes. Congenital microphthalmia is usually associated with other abnormalities, and cases of isolated microphthalmia are rarely reported. Congenital microphthalmia can be diagnosed by prenatal ultrasound by measuring the axial diameter of the eye ball, but the accuracy depends on fetal position and associated anomalies. We report a case of an isolated unilateral microphthalmia which was not diagnosed by prenatal ultrasound, because the only abnormal prenatal ultrasound finding was a small hyperechoic mass lesion in the eye ball and the subsequent scan of the orbits was limited due to fetal prone position. The hyperechoic mass lesion in the eye ball was finally diagnosed as a persistent hyperplastic primary vitreous with hemorrhage by neonatal magnetic resonance image.

Findings of Eye Screening Examinations with Wide-Field Digital Imaging System in Healthy Newborns

PURPOSE: To present the results of eye screening examinations using RetCam in healthy newborns. METHODS: Eye screening examinations were performed using the RetCam wide-field digital imaging system (Clarity Medical System, Pleasanton, CA, USA), as requested by parents, on healthy newborns within a week after birth in an obstetrics and gynecology hospital between June 2012 and September 2014. During the examination, photographs were taken showing red reflex and the fundus. The reading was conducted by a pediatric ophthalmologist. RESULTS: The examinations included a total of 10,023 newborn babies, and abnormalities were discovered in 2,916 patients (29.09%). The most commonly found abnormality was retinal hemorrhage, which was discovered in 2,796 babies (27.89%) and 471 patients showed a large amount of bleeding or macular hemorrhage. In 71 patients (0.7%), ophthalmologic examination was recommended after newborns presented with one of the following conditions: congenital cataracts, retinoblastoma, persistent hyperplastic primary vitreous, vitreous hemorrhaging, persistent pupillary membrane, choroidal nevus or albinism. Due to unclear photographs, 85 eyes of 49 patients (0.48%) could not be read. CONCLUSIONS: Ophthalmologic screening using RetCam is a safe, simple and useful technique for discovering ophthalmologic abnormalities. Additionally, when abnormalities are suspected, the role of a pediatric ophthalmologist is increasingly important in helping patients receive appropriate ophthalmologic treatment. Furthermore, retinal hemorrhage, which occurred in the majority of all observed abnormalities, requires further investigation to examine the possibilities of amblyopia in cases with a large amount of bleeding or macula involvement.

Persistent fetal vasculature: ocular features, management of cataract and outcomes / Persistência da vasculatura fetal: características oftalmológicas, manuseio da catarata e resultados cirúrgicos

PURPOSES: To describe ocular features, management of cataract and functional outcomes in patients with persistent fetal vasculature. METHODS: Retrospective, descriptive case series of patients with persistent fetal vasculature. Data were recorded from the Congenital Cataract Section of Federal University of São Paulo, Brazil from 2001 to 2012. All patients were evaluated for sex, age at diagnosis, systemic findings, laterality, age at surgery, and initial and final follow-up visual acuities. Follow-up and complications after cataract surgery were recorded. Ultrasound was performed in all cases and ocular eco-Doppler was performed in most. RESULTS: The study comprised 53 eyes from 46 patients. Age at diagnosis ranged from 5 days of life to 10 years-old (mean 22.7 months). Twenty-seven patients were male (58.7%). Persistent fetal vasculature was bilateral in 7 patients (15.2%). Forty-two eyes (79.2%) had combined (anterior and posterior forms) PFV presentation, 5 eyes (9.4%) had only anterior persistent fetal vasculature presentation and 6 eyes (11.3%) had posterior persistent fetal vasculature presentation. Thirty-eight eyes (71.7%) were submitted to cataract surgery. Lensectomy combined with anterior vitrectomy was performed in 18 eyes (47.4%). Phacoaspiration with intraocular lens implantation was performed in 15 eyes (39.5%), and without lens implantation in 5 eyes (13.2%). Mean follow-up after surgery was 44 months. Postoperative complications were posterior synechiae (3 cases), retinal detachment (2 cases), phthisis (3 cases), posterior capsular opacification (8 cases), inflammatory pupillary membrane (5 cases), glaucoma (4 cases), intraocular lens implantation displacement (1 case) and vitreous hemorrhage (2 cases). Complications were identified in 19 (50%) of the 38 operated eyes. Visual acuity improved after cataract surgery in 83% of the eyes. CONCLUSIONS: Patients with persistent fetal vasculature have variable clinical presentation. There is an association of persistent fetal vasculature with congenital cataract. Severe complications are related to cataract surgery in patients with persistent fetal vasculature, but 83% of the operated eyes improved visual acuity.

OBJETIVOS: Descrever as características oftalmológicas, o tratamento da catarata e os resultados funcionais em pacientes com o diagnóstico de persistência da vasculatura fetal. MÉTODOS: Estudo retrospectivo e descritivo de série de casos de pacientes com persistência da vasculatura fetal. Dados foram obtidos dos arquivos do Setor de Catarata Congênita da Universidade Federal de São Paulo, Brasil, durante o período entre 2001 a 2012. Todos os pacientes foram avaliados quanto ao sexo, idade ao diagnóstico, achados sistêmicos, lateralidade, idade à cirurgia e acuidade visual inicial e final ao seguimento. Complicações após a cirurgia da catarata foram analisadas. Ultrassom foi realizado em todos os casos e eco-Doppler foi realizado na maioria dos pacientes. RESULTADOS: O estudo incluiu 53 olhos de 46 pacientes. Idade ao diagnóstico variou de 5 dias de vida até 10 anos (média 22,7 meses). Vinte e sete pacientes eram masculinos (58,7%). A persistência da vasculatura fetal foi bilateral em 7 pacientes (15,2%). Quarenta e dois olhos (79,2%) apresentaram formas combinadas (anterior e posterior) da persistência da vasculatura fetal, 5 olhos (9,4%) tinham somente a forma anterior da persistência da vasculatura fetal e 6 olhos (11,3%) tinham a forma posterior de apresentação da persistência da vasculatura fetal. Trinta e oito olhos (71,7%) foram operados de catarata. Lensectomia com vitrectomia anterior foi realizada em 18 olhos (47,4%). Facoaspiração com implante de lente intraocular foi realizada em 15 olhos (39,5%) e sem implantação de lente em 5 olhos (13,2%). O seguimento médio após cirurgia foi de 44 meses. Complicações pós-operatórias foram: sinéquias posteriores (3 casos), descolamento da retina (2 casos), atrofia do globo ocular (3 casos), opacificação da cápsula posterior (8 casos), membrana pupilar inflamatória (5 casos), glaucoma (4 casos), deslocamento da lente implantada (1 caso) e hemorragia vítrea (2 casos). Complicações foram identificadas em 19 (50%) dos 38 olhos operados. Acuidade visual melhorou após a cirurgia da catarata em 83% dos olhos. CONCLUSÕES: Pacientes com persistência da vasculatura fetal tem apresentações clínicas variáveis. Existe uma associação da persistência da vasculatura fetal com catarata congênita. Complicações graves são associadas com a cirurgia da catarata nesses pacientes, mas 83% dos olhos operados melhoraram a acuidade visual nesse estudo.

Vítreo primario hiperplásico persistente: presentación de un caso / Persistent hyperplastic primary vitreous: a case report

El artículo presenta el caso de la persistencia del vítreo primario hiperplásico, evidenciado por una resonancia magnética, que se debe a una regresión incompleta de la suplencia sanguínea ocular embrionaria en el canal de Cloquet, ubicado dentro del globo ocular.

Incidental Abnormal Ocular Findings of Neonates in Ophthalmic Examinations

PURPOSE: To investigate incidental abnormal ocular findings that may cause visual impairment in neonates. METHODS: The medical records of patients requested for screening for retinopathy of prematurity (ROP) at our hospital's pediatrics department between March 2005 and July 2009 were retrospectively reviewed. Patients were classified into premature and mature neonates according to gestational age (under 37 weeks). RESULTS: A total of 2,090 eyes from 1,045 patients were comprised of 1,514 eyes of 757 premature neonates and 576 eyes of 288 mature neonates. Among 154 eyes of 78 patients (10.17%) who were diagnosed with ROP, 34 eyes of 18 patients had laser treatment. Seven eyes of 5 patients in the premature neonate group (0.46%), and 38 eyes of 23 patients in the mature neonate group (6.57%) were found to have abnormal ocular findings, excluding incidental ROP. Twenty-six eyes of 16 patients were found to have a retinal hemorrhage, and the majority had a birth history of perinatal distress, including birth asphyxia, meconium aspiration, and transient tachypnea of the newborn. Other findings included congenital cataracts, corneal opacity, macular coloboma, persistent hyperplastic primary vitreous, and abnormal fundus. CONCLUSIONS: The incidence of ocular abnormalities in neonates that cause visual impairment is low, but theses may threaten a child's life. Early detection and prompt treatment of ocular disorders in children are important in order to avoid permanent, lifelong visual impairment. Therefore, ophthalmic examinations are essential, even in normal neonates, for early detection of ocular disorders.

Bilateral persistent hyperplastic primary vitreous.

A case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 3-month-old male infant, who had bilateral leukokoria, is presented. The child was referred for imaging with a clinical suspicion of retinoblastoma. Gray-scale ultrasound evaluation revealed an echogenic band in the posterior segment of both globes, extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of arterial flow in the band in both globes. Associated echogenic hemorrhage was also seen, which was confirmed by computed tomography. Most cases of PHPV are sporadic and unilateral, and bilateral PHPV is rare. The imaging features in this case suggest the diagnosis of bilateral PHPV and differentiate it from retinoblastoma. This entity, although infrequent, should be considered in the differential diagnosis while evaluating bilateral leukokoria.

Persistência de vítreo primário hiperplásico posterior / Posterior persistent hyperplastic primary vitreous

Relato de um caso de persistência de vítreo primário hiperplásico posterior (PVPH) em um paciente de nove anos, com baixa acuidade visual em olho esquerdo, encaminhado pela escola para avaliação oftalmológica. A PVPH pode envolver um amplo espectro de alterações oculares envolvendo retina, vítreo, nervo óptico, mácula, cristalino, íris, corpo ciliar além de anomalias como a microftalmia e microcórnea. Ressaltamos a importância da propedêutica ocular precoce nas crianças, a fim de evitar o diagnóstico e tratamento tardios comprometendo o prognóstico visual e com isto minimizar os danos futuros à saúde destas crianças.

Report of a case of posterior persistent hyperplastic primary vitreous (PHPV) case on a nine years old patient with low visual acuity on the left eye, referred by the school for ophthalmic evaluation. The PHPV can embrace a wide spectrum of ocular alterations involving retina, vitreous, optic nerve, macula, crystalline, iris and ciliary body besides anomalies like mycroftalmia and microcornea. We point out the importance of early ocular evaluation in children, late diagnosis and late treatment, in order to minimize the possibility of future damages to the health of these children.

Clinical Findings and Prognosis of Persistent Hyperplastic Primary Vitreous

PURPOSE: To evaluate the clinical findings, visual improvement and preservation of eye according to treatment modality among children with persistent hyperplastic primary vitreous (PHPV) METHODS: A retrospective study, from 1982 to 2001, at Seoul National University Children's Hospital RESULTS: The subjects for investigation were 126 PHPV eyes in 114 children. The mean age at first exam was 3.6 +/- 3.5months, and the mean age at operation was 13.3 +/- 12.1months. Combined type (67%) was most common, followed by posterior (24%), and anterior (9%) types. The chief complaints in order of frequency were are leukocoria (35%), no fixation (23%), strabismus (21%), and microophthalmia (12%). Percentages of eye preservation and visual improvement over 20/1000 were 77%, and 57%, respectively. Nevertheless, the percentage of visual improvement over 20/200 in the anterior type was 25%. CONCLUSIONS: Combined type was most common in PHPV. Clinical findings and treatment modality varieds according to the type. Visual prognosis was poor. However, the post-treatment status was good in view of the treatment purpose. Therefore we can optain a good outcome through active treatment after the exact classification of PHPV eyes and by establishing the treatment purpose.

Persistência hiperplásica do vítreo primitivo: avaliação por métodos de imagem / Persistent hyperplastic primary vitreous: imaging evaluation

Persistência hiperplásica do vítreo primitivo (PHVP) é um desenvolvimento anormal, afetando crianças, resultante da falha de regressão do vítreo primitivo e dos vasos hialóides, com proliferação de tecido conectivo. A PHVP pode ter três variações: uma anterior, outra posterior e uma terceira com a combinação das duas. Clinicamente manifesta-se como leucocoria (reflexo branco pupilar) em um olho de dimensões reduzidas. Esta anomalia é usualmente unilateral e não associada com outros achados sistêmicos. Leucocoria, descolamento de retina, pregas retinianas e catarata podem confundir PHVP com outras afecções oculares que têm achados semelhantes. Retinoblastoma, catarata congênita, retinopatia da prematuridade e pseudogliomas são doenças com achados semelhantes aos da PHVP. A visualização direta dos remanescentes do sistema vascular hialóide fetal é a melhor evidência da lesão presente, se não fosse, muitas vezes impossivel, à fundoscopia, devido à opacidade do tecido. Nestes casos, a visualização indireta, por meio de ultra-sonografia, tomografia computadorizada e ressonância magnética, fornece o diagnóstico. As imagens obtidas por estes métodos proporcionam, ainda, informações e diagnóstico diferencial com outras doenças, particularmente com o retinoblastoma. Os autores fazem uma revisão na literatura e apresentam um estudo iconográfico das imagens e achados da PHVP

Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly of the eye that primarily affects premature infants. PHPV results from failure of regression of the embryogenic primary vitreous and hyaloid vasculature, and proliferation of connective tissue. Three types of PHPV may be found: anterior, posterior and a combination of anterior and posterior. The clinical findings include leukocoria (white pupillary reflex) and microphthalmic eye. This anomaly is usually unilateral and unassociated with other systemic findings. The differential diagnosis between PHPV and other ocular diseases may be difficult due to similar features such as leukocoria, detached retina, retinal folds and cataract. Other diseases with similar features include retinoblastoma, isolated congenital cataract, retinopathy of prematurity and pseudogliomas or leukocorias. Direct visualization of the remnants of the fetal hyaloid vascular system offers the best evidence of PHPV. However, diagnosis using the ophthalmoscope is sometimes impossible because of opaque tissues. In this circumstance an indirect visualization by ultrasound, computed tomography or magnetic resonance imaging may be useful. These imaging methods provide valuable information for the differentiation from other diseases, particularly retinoblastoma. The authors present a review of the literature and an iconographic study of the imaging findings in patients with PHPV.

Ocular Fundus Findings in Children with Strabismus

The authors have examined 980 children with strabismus who were 6 years of age or under at the Eye Clinic of Yeungnam University Hospital. This study was conducted during the 4-year period between January 1990 and December 1994. The main objective of this study was to determine the incidence and clinical characteristics of fundus abnormality in children with strabismus. The total number of subjects in this study are 980 cases. Sixty subjects(6.1%) are found to have optic disc, chorioretinal and vitreous anomalies. Among them, 26 cases(43%) are diagnosed as having optic nervehypoplasia, in 7 cases (12%) coloboma, in 4 cases(7%) morning glory syndrome, in another 4 cases persistent hyperplastic primary vitreous, and in 3 cases retinoblastoma. In our study, 6.1% of the patients with strabismus are found to have disorders in optic disc and vitreoretina. These finding suggests that infants and young children with strabismus should have been examined to detect other related disorders of the eye.

Ultrasonographic Findings of Persistent Hyperplastic Primary Vitreous

We analyzed 33 patients (39 eyes) who were surgically diagnosed as persistent hyperplastic primary vitreous (PHPV) with standardized B-scan ultrasonography. The eyes were evaluated for vitreous stalk or membrane, retrolental proliferation, retinal fold, vitreous opacity, and retinal detachment (64.1 %, 48.7 %, 15.4 %, 15.4 %, and 5.1 %, respectively) with the results. We classified these findings of PHPV into 5 types as follows, type 1: simple retinal fold or preretinal fold alone; type 2: retrolental proliferation alone; type 3: vitreous stalk or membrane; type 4: retrolental proliferation connected with vitreous stalk or membrane; type 5: complicated retinal detachment. The incidence of these types were 15.4%, 15.4%, 28.2 %, 33.3 %, and 5.1 %, respectively. Ultrasonographic findings would be used as a useful parameters to evaluate, document, and plan the management in PHPV.

Two Cases of Retinoblastoma Diagnosed by Fine needle Aspiration Biopsy

Retinoblastoma is a highly malignant intraocular tumor of childhood that requires accurate diagnosis and prompt treatment. The diagnosis of retinoblastoma is based on the fundus finding and noninvasive radiologic evaluation, but sometimes retinoblastoma is clinically misdiagnosed as nonneoplastic lesion such as Coats' disease, persistent hyperplastic primary vitreous, toxocariasis and old retinal detachment and sometimes vice versa. We performed fine needle aspiration biopsies in two cases, in whom retinoblastoma or Coats' disease was suspected but there was diagnostic uncertainty on fundus finding, orbit CT or MRI. The cytologic diagnosis of two cases was retinoblastoma. And after enucleation final histopathologic diagnosis correlated with the cytodiagnosis. We think that fine needle aspiration biopsy may be helpful in establishing the diagnosis in difficult cases of childhood leukocoria including retionblastoma.

Clinical Evaluation in Persistent Hyperplastic Primary Vitreous

We analysed a retrospective study of 23 patients (29 eyes) who were clinically and radiologically diagnosed as persistent hyperplastic primary vitreous from January 1984 to June 1992 at St. Mary's Hospital, Catholic University Medical College, by observing the clinical findings, results of CT or MRI, incidence of complications and postoperative visual acuity. The results were as follows: 1. The unilateral ocular involvement was 73.9% and bilateral ocular involvement was 26.1%. 2. The common presenting clinical signs were shallow anterior chamber, lens opacity, vitreous opacity and microphthalmos. 3. The main features of CT or MRI were lens defcrmity, shallow anterior chamber, vitreous opacity and microphthalmos. 4. The complications were glaucoma (31.0%), intraocular hemorrhages (10.3%), posterior synechiae (17.2%) and phthisis bulbi (6.9%). 5. The postoperative visual acuity was not improved than preoperative levels.

A Case of Bilateral Persistent Hyperplastic Primary Vitreous

Persistent hyperplastic primary vitreous(PHPV) is a congnital abnormality of the embryonic intraocular vasculature, resulting from the failure of regression of the primary vitreous in a full-term infant. PHPV is usually unilateral and characterized by leukocoria, elongated ciliary process, microphthalmia, cataract, rubeosis iridis, shallow anterior chamber, and secondary glaucoma. PHPV finally develops into retinal detachment, intractable glaucoma, and pthisis bulbi. Surgical treatment, involving lensectomy and vitrectomy, is not successful and poor visual result is obtained due to retinal abnormalities. A one-month-old girl was found to have persistent hyperplastic primary vitreous in both eyes, and she expired of unknown etiology at home 2 months after diagnosls.

Computed Tomography in the Evaluation of Childs with White Pupil

White pupil is one of the most disturbing problems in the pediatric age group and many conditions can cause white pupil, but the differential diagnosis between the retinoblastoma and other conditions simulating it, is the prime importance in the evaluation of the white pupil. The ophthalmoscopy is the most accurate diagnostic method for evaluating intraocular diseases because it is able to see directly. However, its usefulness seems to be limited in the case of extreme lens opacity or vitreous opacity and in the evaluation of orbital diseases extending outside of the globe. Recent advances of high resolution computed tomography(CT), the axial tomographic images of the orbit were markedly improved and represented good anatomic details of the orbit and the topographic relationship with surrounding structures. We reviewed CT findings of 15 children who presented white pupil and had results demonstrating specific findings in the cases of retinoblastoma, persistent hyperplastic primary vitreous and vitreous opacity due to retinal detachment and retrolental fibroplasia. The CT findings of each disease are discussed with detail.