Hallazgos clínicos y oftalmológicos en niños con neurofibromatosis 1 / Clinical and ophthalmological findings in children with type 1 neurofibromatosis

Este trabajo tuvo por objetivos determinar la incidencia en nuestro medio de los criterios diagnósticos del National Institutes of Health (NIH) en niños con Neurofibromatosis 1 (NF1), comparar con estadísticas publicadas, analizar los hallazgos oftalmológicos, el valor de los estudios complementarios y establecer criterios de seguimiento. Métodos: Se trata de un estudio retrospectivo que incluyó 245 pacientes que ingresaron al Hospital de Pediatría Garrahan entre los años 1988 y 2010. Se diagnosticó NF1 en la primera consulta multidisciplinaria, utilizando los criterios de NIH, efectuándose en algunos niños neuroimágenes y potencial evocado visual (PEV). Resultados: El 92% de los pacientes presentó manchas café con leche; 40.8% neurofibromas, 75.5% nódulos de Lisch; 38.8% efeliloides; 16.3% glioma del nervio óptico; 16.3% displasia esquelética y 49% fueron hereditarios. Evidenciamos 1.76 miopías por cada hipermetropía. Conclusiones: Las frecuencias halladas coinciden con reportes previos, a excepción de las efeliloides, con incidencia menor. Realizamos de elección resonancia magnética nuclear (RMN), aún en pacientes asintomáticos, repitiéndolas bianualmente y examen oftalmológico cada seis meses hasta los ocho años. No indicamos actualmente PEV (AU)

The objective of this study was to determine the incidence of the National Institutes of Health (NIH) diagnostic criteria in children with Neurofibromatosis type 1 (NF1) in our setting, to compare them with the published statistical data, ophthalmological findings, the importance of complementary studies, and to establish follow-up criteria. Methods: We conducted a retrospective study including 245 patients that were admitted to the Pediatric Hospital Garrahan between 1988 and 2010. NF1 was diagnosed at the first multidisciplinary visit, using the NIH criteria. Neuroimaging and visual evoked potentials (VEP) were performed in some of the children. Results: 92% of the patients had café au laity spots; 40.8% neurofibromas; 75.5% Lisch nodules; 38.8% ephelides; 16.3% optic-nerve glioma; and 16.3% skeletal dysplasia. The disorder was hereditary in 49%. For each hypermetry,1.76 myopias were observed. Conclusions: These rates found were according to previous reports, except for ephelides, which were less common. Elective magnetic resonance imaging (MRI ) was performed, even in asymptomatic patients, and was repeated biannually and ophthalmological examination was done every six months until nine years of age. Currently, we do not indicate VEP

Toxoplasmosis ocular. Descripción de tres pacientes con presentación atípica / Ocular toxoplasmosis. Description of three patients with an atypical presentation.

Objetivo: Describir una forma de presentación atípica de toxoplasmosis ocular, enfatizando la importancia de la jerarquización de los hallazgos clínicos y las limitaciones de la serología para realizar un diagnóstico temprano. Pacientes y Métodos: Estudio retrospectivo y descriptivo de tres casos clínicos de toxoplasmosis ocular activa, con presentación atípica (compromiso del nervio óptico), derivados al Servicio de Oftalmología del Hospital J. P. Garrahan en el periodo comprendido entre 2007 y 2010. Resultados: En los tres casos presentados la sospecha clínica de toxoplasmosis ocular no se correlacionó con evidencia serológica de infección reciente. En un caso, la terapéutica específica temprana, basada en la sospecha clínica, resultó en una excelente recuperación funcional. Un tratamiento tardío puede interferir en el resultado visual. Conclusiones: Basados en los hallazgos clínicos y la alta sospecha de esta patología debe iniciarse el tratamiento específico sin esperar que los resultados serológicos la confirmen. Eventualmente, la mejoría clínica confirmara el diagnóstico. El comportamiento de los títulos de anticuerpos en el curso de la enfermedad ocular no siempre es confiable, y en muchos casos retrasa el comienzo de la terapéutica con la consiguiente mala rehabilitación visual de estos pacientes (AU)

Objective: To describe an atypical presentation of ocular toxoplasmosis, emphasizing the importance of clinical findings and the limitations of serology in the early diagnosis. Patients and Methods: A retrospective, descriptive study was conducted of three cases with active ocular toxoplasmosis with an atypical presentation (optic nerve involvement), referred to the Department of Ophthalmology of Hospital J. P. Garrahan between 2007 and 2010. Results: In the three cases presented here clinical suspicion of ocular toxoplasmosis did not correlate with serological evidence of a recent infection. In one case, early treatment, based on clinical suspicion, resulted in excellent functional recovery. Late management may compromise visual outcome. Conclusions: Based on clinical findings and suspicion of the pathology, specific treatment should be started without waiting for serological confirmation. Eventually, clinical improvement will confirm the diagnosis. The behavior of antibody titres in the course of the ocular disease is not always reliable and often delays treatment initiation with subsequent difficulties in the visual rehabilitation of these patients (AU)

Retinopatía del prematuro / Retinopathy of Prematurity

La retinopatía del prematuro (RDP) es una complicación frecuente en los egresados de las UCIN y una causa importante de ceguera. Su prevención primaria depende de una tención reacional que respete límites de saturación en la oxigenoterapia. Su prevención secundaria requiere controles oftalmológicos oportunos y reiterados hasta completarse la vascularización de la retina. Estudios epidemiológcios realizados en el 2002 encontraron una situación alarmante por la frecuente afectación de pacientes de bajo riesgo (PN > 1500 g) y controles oftalmológicos tardíos. A partir del 2003 se conformó un grupo colaborativo multicéntrico para revertir esta situación. Objetivo: analizar las modificaciones de las características de los RN derivados por RDP al hospital JP Garrahan durante los períodos 1996-1999, 2000-2003 y 2004-2007. Métodos: diseño retrospectivo. Las variables analizadas fueron: PN, edad gestacional (EG), tiempo de tratamiento con 02 y ARM, edad de consulta, severidad de RDP, lugar dederivación (> o < a 70 Km). Resultados: el nº de consultas fue de 190, 613 y 489 respectivamente. La proporción de pacientes derivados de > 70 Km disminuyó del 50 al 35 por ciento. Los pacientes del último período han tenido > tiempo de ARM (P 0.04) y oxigenoterapia (p 0.001) pero no varió significativamente su PN ni EG. La proporción de pacientes con RDP severa aumentó del 56 por ciento al 67 por ciento. conclusión: ha disminuido la frecuentcia de derivaciones y las mismas coresponden a pacientes más graves. Continúa siendo la inaceptable la proporción de pacientes de bajo riesgo y la severidad de la RDP al momento de consulta.

[Retinopathy of prematurity in the XXI century in a developing country: an emergency that should be resolved]. / La retinopatía del prematuro en el siglo XXI en un país en desarrollo: una urgencia que debe ser resuelta.

INTRODUCTION: Retinopathy of prematurity (ROP) is the first cause of childhood blindness in Argentina and is reaching epidemic proportions. To gain greater insight into the issues involved in this epidemic, we analyzed the characteristics of the infants referred for treatment of very severe ROP to the Dr. Juan P. Garrahan Pediatric Hospital, located in the city of Buenos Aires. METHODS: We performed a retrospective study of patients referred for ROP from 01/01/96 to 12/31/03. Very severe ROP was defined as threshold disease or worse in both eyes, plus disease in zones 1 or 2, or Rush disease. Unusual patients were defined as those with very severe ROP with a gestational age (GA) of > 31 weeks or birth weight (BW) > 1500 g. Patients were divided into two groups according to the distance between the referring center and our hospital: group A consisted of those from nearby areas ( 70 km from the hospital. RESULTS: During the study period, the number of cases progressively increased while the proportion of "unusual" patients slightly decreased. The total number of infants referred was 809 and 14 % were unusual patients; 55 % had very severe ROP, 89 % were treated, and 7 % were referred too late and lost the opportunity for treatment. Group B comprised 61 % of the infants referred. Very severe ROP was present in 53 % of patients in group A and in 56 % of those in group B. The mean GA was 29.5 +/- 3.1 weeks in group A and 30.8 +/- 2.6 in group B (p < 0.001); 90.5 % of the patients in group A and 64 % of those in group B required mechanical ventilation (p < 0.001). No predictors for very severe ROP were found in multifactorial analysis. CONCLUSIONS: We report a childhood epidemic of blindness in which the prevalence of very severe ROP increased during the study period and the disease occurred in large, fairly mature newborns. The differences found in relation to the referring center and the lack of correlation between very severe ROP and prematurity or the use of mechanical ventilation suggests substantial disparity in healthcare, which should be taken into account when planning prevention strategies.

La retinopatía del prematuro en el siglo XXI en un país en desarrollo: una urgencia que debe ser resuelta / Retinopathy of prematurity in the XXI century in a developing country: an emergency that should be resolved

Introducción: La retinopatía del prematuro (RdP) es la primera causa de ceguera infantil en Argentina, de alcances epidémicos. Con el objetivo de mejorar el conocimiento de factores involucrados en esta epidemia, analizamos las características de los niños derivados para tratamiento de RdP severa al Hospital de Pediatría Juan P. Garrahan, ubicado en la ciudad de Buenos Aires. Métodos: Revisión retrospectiva de pacientes admitidos por RdP entre el 1 de enero de 1996 y el 31 de diciembre de 2003. RdP severa = enfermedad umbral en ambos ojos o peor, enfermedad plus en zona 1 o 2, o enfermedad de Rush. Pacientes “inusuales” son definidos aquéllos con RdP grave y edad gestacional (EG) > 31 semanas o peso al nacer (PN) > 1.500 g. Los pacientes se dividieron en 2 grupos según distancia de procedencia en relación a nuestro hospital: grupo A de procedencia cercana (≤ 70 km) y grupo B amás de 70 km. Resultados: Durante el período de estudio hubo un aumento progresivo del número de casos, con escasa reducción de casos “inusuales”. El total de niños referidos fue de 809, con 14% de casos “inusuales”; 55 % presentó formas severas, 89% recibió tratamiento y 7% perdió oportunidad por derivación tardía. El 61 % perteneció al grupo B; la incidencia de RdP severa fue de 53 % en A y 56 % en B. La media de EG fue 29,5 +/- 3,1 semanas en el grupo A y de 30,8 +/- 2,6 en el B (p < 0,001); 90,5 % en A y 64 % en B requirió asistencia ventilatoria mecánica (p < 0,001). No se encontraron factores predictivos de RdP grave en el análisis multivariable. Conclusiones: En esta epidemia de ceguera durante la niñez, la prevalencia de formas severas de RdP aumentó durante los años del estudio y la enfermedad ocurre aún en prematuros cercanos al término. Las diferencias según el lugar de procedencia y la falta de asociación de RdP grave con inmadurez o uso de ventilación mecánica, sugieren una importante disparidad en los cuidados de salud que debería tomarse en cuenta al planificar estrategias de prevención

Introduction Retinopathy of prematurity (ROP) is the first cause of childhood blindness in Argentina and is reaching epidemic proportions. To gain greater insight into the issues involved in this epidemic, we analyzed the characteristics of the infants referred for treatment of very severe ROP to the Dr. Juan P. Garrahan Pediatric Hospital, located in the city of Buenos Aires. Methods We performed a retrospective study of patients referred for ROP from 01/01/96 to 12/31/03. Very severe ROP was defined as threshold disease or worse in both eyes, plus disease in zones 1 or 2, or Rush disease. Unusual patients were defined as those with very severe ROP with a gestational age (GA) of > 31 weeks or birth weight (BW) > 1500 g. Patients were divided into two groups according to the distance between the referring center and our hospital: group A consisted of those from nearby areas (≤ 70 km) and group B comprised those from areas > 70 km from the hospital. Results During the study period, the number of cases progressively increased while the proportion of “unusual” patients slightly decreased. The total number of infants referred was 809 and 14 % were unusual patients; 55 % had very severe ROP, 89% were treated, and 7% were referred too late and lost the opportunity for treatment. Group B comprised 61 % of the infants referred. Very severe ROP was present in 53 % of patients in group A and in 56 % of those in group B. The mean GA was 29.5 +/- 3.1 weeks in group A and 30.8 +/- 2.6 in group B (p < 0.001); 90.5% of the patients in group A and 64 % of those in group B required mechanical ventilation (p < 0.001). No predictors for very severe ROP were found in multifactorial analysis. Conclusions We report a childhood epidemic of blindness in which the prevalence of very severe ROP increased during the study period and the disease occurred in large, fairly mature newborns. The differences found in relation to the referring center and the lack of correlation between very severe ROP and prematurity or the use of mechanical ventilation suggests substantial disparity in healthcare, which should be taken into account when planning prevention strategies

Validación de la prueba nacional de pesquisa de trastornos de desarrollo psicomotor en niños menores de 6 años / Validation for screening test of detecting psychomotor development problems in children under six

Introducción. El uso de una prueba de pesquisa en los primeros años de vida permite la detección temprana de retrasos en el desarrollo psicomotor y su tratamiento oportuno. En la Argentina contamos con una Prueba Nacional de Pesquisa preparada en base a un estudio nacional. El objetivo del trabajo fue validar la prueba, comparando sus resultados con evaluaciones diagnósticas, realizadas en forma simultánea por varios servicios del Hospital Garrahan. Pacientes y métodos. Se seleccionó una muestra de 106 niños de 0 a 5,99 años que concurrían al área de bajo riesgo del Hospital. Se realizaron los siguientes estudios diagnósticos: evaluación del desarrollo psicomotor (Bayley II), examen neurológico, salud mental, coeficiente intelectual (Wechsler, Terman), conducta adaptativa (Vineland), lenguaje (prueba de Gardner receptiva y expresiva, ITPA), audición (emisiones otoacústicas, audiometría tonal, PEAT), examen visual. Se utilizó el DSM-IV como referencia de trastornos del desarrollo. Se evaluó la sensibilidad y especificidad obtenidas según la aplicación de diferentes puntos de corte (número de ítems fracasados). Resultados. El mejor punto de corte se estableció en un ítem tipo A o 2 tipo B, con una sensibilidad del 80%, especificidad: 93%, valor predictivo positivo: 94%, valor predictivo negativo: 77%, porcentaje de coincidencia: 85%. Fue inesperada la elevada prevalencia de problemas de desarrollo encontrada en la muestra: 57%. La prueba es capaz de detectar problemas en las cuatro áreas del desarrollo, incluidos trastornos del lenguaje. Conclusión. Los resultados confirman a la Prueba Nacional de Pesquisa como un instrumento válido para ser usado en el primer nivel de atención para el reconocimiento de niños con sospecha de sufrir trastornos del desarrollo. Asimismo, el trabajo de información permite establecer diferentes puntos de corte y constituye un instrumento útil para su aplicación en la práctica pediátrica.

The use of a screening test in the first years of life allows the early detection of delays of psychomotor development and its treatment, thus contributing to improve the prognosis of the child with special needs. In Argentina, a screening test for detecting developmental problems in children under 6, made with local children and data is available (PRUNAPE). A validation procedure for this test was carried out on 106 children attending at low risk outpatient clinic in Hospital Garrahan. The test was administered to the children together with a battery of diagnostic examinations and studies, performed by experienced specialists from different Hospital services: psychomotor development, neurology examination, mental health, intellectual quotient (Wechsler, Terman), adaptive behaviour (Vineland), language (Gardner expressive and receptive, ITPA), hearing (otoacustic emissions, audiometry, BERA), vision. The DSM ­IV was used as a reference for developmental problems. Using as a failure criterion to the PRUNAPE, the failure of performing correctly one type A item or two type B item, sensitivity of the test was 80%, specificity, 93%; positive predictive value, 95%; negative predictive value, 77%; overall agreement, 85%. A very high prevalence of developmental problems was found: 57%. PRUNAPE was found to be capable of detecting a wide range of problems. These results confirm PRUNAPE as a valuable instrument for early detection of developmental problems in paediatric practice at the primary care level.

Retinoblastoma with low risk for extraocular relapse.

OBJECTIVE: To define a subgroup of patients with retino-blastoma and low risk of extraocular relapse through histopathological and clinical variables. PATIENTS AND METHODS: Inclusion criteria consisted of stage I (intraocular disease), stage IIb1 (without concomitant choroid and/or scleral invasion), and nonenucleated patients (according to the Grabowski-Abramson classification). A total of 112 consecutive patients admitted to Hospital JP Garrahan from 1987 to 1997 were evaluable. Treatment included enucleation or local therapy and no chemotherapy. RESULTS: Forty-one patients had stage Ia (intraretinal), 8 stage Ib (prelaminar optic nerve invasion), 40 stage Ic (uveal invasion), and 12 stage IIb1 (postlaminar optic nerve invasion and cut end free of tumor). Eleven patients had neither eye enucleated. Median follow-up was 60 months. Only two events occurred: one patient had progressive disease in the contralateral globe and died of CNS metastasis and another had an orbital relapse that was successfully treated. Both had choroidal invasion. Five-year pEFS and pOS were 0.97 and 0.98, respectively. Neither length of the optic nerve stump, tumor size, anterior chamber invasion, degree of differentiation, nor degree of ocular coat invasion correlated with increased risk of metastasis. CONCLUSIONS: A subset of patients with retinoblastoma with low risk of relapse can be determined using histopathological evaluation of the invasion of ocular coats. Adjuvant chemotherapy is not warranted for patients with intraretinal extension and prelaminar optic nerve invasion. It is also probable that those patients with isolated choroidal invasion and those with postlaminar optic nerve extension with surgical margins clear of tumor do not need chemotherapy.

Late diagnosis of retinoblastoma in a developing country.

OBJECTIVES: To assess the diagnostic process of retinoblastoma in a developing country. STUDY DESIGN: Prospective survey of 95 consecutive parents of patients with retinoblastoma. RESULTS: Fifty six parents consulted initially with a paediatrician. Their children tended to be younger, with a significantly higher frequency of advanced disease. Only half of the patients who consulted with a paediatrician were appropriately referred to an ophthalmologist; the paediatrician underestimated the complaints in the remainder. Children taken to an ophthalmologist were older and had less advanced disease. In about three quarters of these children, a diagnosis of retinoblastoma was suspected by the ophthalmologist on the first visit. Parents of patients with more advanced disease consulted significantly later. Poor parental education correlated significantly with late consultation. Lack of health insurance and living outside Buenos Aires City correlated significantly with an increased risk of extraocular disease. CONCLUSIONS: Paediatricians are the first health professional seen by most children with retinoblastoma. However, the diagnosis is not readily established. There is also a delay in consultation by parents, which is significantly longer in cases with advanced extraocular disease. Socioeconomic factors and access to health care might play a role in delayed diagnosis.

Clinical, cytogenetic, and molecular testing of Argentine patients with retinoblastoma.

PURPOSE: The purpose of this study is to determine the clinical, chromosomal, and molecular characteristics of Argentine patients with unilateral and bilateral retinoblastoma. STUDY DESIGN: Eighty-six patients belonging to 82 families were studied; 59% of them were examined during the first year of life. Leukocoria was the most common reason for consultation. Other presenting signs were strabismus and glaucoma. Enucleation of the affected eye was performed in 85% of the cases and the complication rate was 13%. RESULTS: An appropriate therapy allowed the survival of 84 of the 86 patients. Two children with malformations and growth retardation had an abnormal karyotype with a deletion in 13q14. Segregation analysis of polymorphic sites within the retinoblastoma gene and the parental origin of the allele lost in the tumor were analyzed in 30 of the 82 families. Five mutant alleles transmitted through the germline and six de novo germline mutant alleles were identified in 12 patients with hereditary retinoblastoma. Most de novo germline mutant alleles were paternally derived. Molecular analysis of nonhereditary retinoblastoma showed loss of heterozygosity in three of eight cases. From these, two maternal alleles and one paternal allele were lost, thus not indicating a significant difference in the parental origin for the lost allele. CONCLUSIONS: These data are useful for deoxyribonucleic acid diagnosis of susceptibility to retinoblastoma in relatives of hereditary patients, even if mutations have not been identified.

Papel del pediatra en el proceso diagnóstico del retinoblastoma / Role of pediatrician in the diagnostic process of retinoblastoma

Objetivos: describir el proceso diagnóstico en pacientes con retinoblastoma y el papel del pediatra en el mismo. Valorar los conocimientos acerca del retinoblastoma en una población de médicos residentes de pediatría. Material y métodos: estudio prospectivo transversal. Encuesta administrada a los médicos residentes e interrogatorio a familias de pacientes con retinoblastoma. Resultados: se estudiaron 69 familias. Consultaron al peidatra 39, siendo derivadas al oftalmólogo 17. Treinta pacientes consultaron al oftalmólogo. En 12 de ellos, no se práctico fondo de ojos se restó importancia al cuadro. Los pacientes que consultaron al pediatra eran significativamente más jóvenes y tenían mayor frecuencia de enfermedad bilateral. La mediana del tiempo transcurrido entre la aparición de los síntomas y la consulta fue de 9 semanas. De los cuales 41 médicos residentes encuestados, 30 reconocieron a la leucocoria como manifestación más frecuente del retinoblastoma y sólo 11 al estrabismo. Solo 9 residetes respondieron adecuadamente la edad de presentacion del retinoblastoma bilateral. No hubo asociación significativa entre el año de residencia y la probabilidad de responder acertadamente las pregutas. La gran mayoría había recibido información sobre el retinoblastoma en la facultad o en la residencia. conclusiones: La mayoría de los pacientes con retinoblastoma son llevados inicialmente al pediatra, en especial los niños más jovenes. En un alto porcentaje el pediatra no derivó al niño al oftalmólogo para su evaluación. Existió además una demora en llevar a la consulta a los niños por parte de la familia. Los médicos residentes de pediatría tienen escasa información acerca del retinoblastoma, independientemente del año de residencia o del hecho de haber visto pacientes con esta entidad.

Results of a stage-based protocol for the treatment of retinoblastoma.

PURPOSE: To describe the treatment of retinoblastoma at a single institution using a prospective protocol based on histopathologic staging. PATIENTS AND METHODS: We included 116 consecutive patients (101 eligible, 46 bilateral) from August 1987 to December 1993. Treatment was enucleation or conservative therapy for intraocular disease (stage I patients). Stage II patients (orbital or postlaminar invasion) received vincristine, cyclophosphamide, and doxorubicin for 57 weeks. Patients with orbital mass and extension beyond the cut end of the optic nerve also received orbital radiotherapy (45 Gy). The latter received intrathecal therapy. In those with CNS (stage III) or hematogenous metastasis (stage IV), cisplatin and etoposide were added along with cranial (in patients with a CNS mass and prophylactically in stage IV) or craniospinal (in patients with positive CSF) radiotherapy. RESULTS: The median follow-up time was 39 months (range, 12 to 84). The overall survival rate was 0.84. Survival rates according to stage were as follows: stage I probability of overall survival [pOS] = 0.97) (alive/total), 59 of 60; stage II (pOS = 0.85) including patients with scattered episcleral cells, three of three; orbital mass, one of one; postlaminar invasion up to and beyond the cut end of optic nerve, 10 of 11 and 11 of 14, respectively; of stage III (pOS = 0), zero of six; and stage IV (pOS = 0.50), three of six. Only those patients with preauricular adenopathy as the only metastatic site survived in the latter group. Acute toxicity was mild. CONCLUSION: Chemotherapy is not warranted to prevent systemic metastasis for intraocular disease. Patients with extraocular orbital disease and had a good outcome with this therapy. Patients with metastatic disease fared poorly, except for those with isolated malignant preauricular adenopathy.

Multinational clinical and pathologic registry of retinoblastoma. Retinoblastoma International Collaborative Study report 2.

BACKGROUND: The Retinoblastoma International Collaborative Study is a multinational prospective clinical and pathologic registry designed to collect baseline clinical information, data on methods of treatment, and information on the subsequent clinical course of children with retinoblastoma. METHODS: 206 children with newly diagnosed retinoblastoma were evaluated in the participating clinical centers between 1 July 1987 and 31 December 1989. All of the children underwent comprehensive baseline assessment of multiple clinical variables prospectively according to a standard protocol. The recorded values were tabulated and analyzed. RESULTS: The 206 children with retinoblastoma included 99 boys (48.1%) and 107 girls (51.9%). The mean age at diagnosis was 21.2 mo in the total study group. One hundred twenty-seven of the 206 children (61.7%) had unilateral disease, while 79 (38.3%) had bilateral involvement. The mean age at diagnosis in the bilateral cases (14.6 mo) was substantially less than in the unilateral ones (23.5 mo). The great majority of patients (approximately 75%) had advanced disease (group V in both Reese-Ellsworth and Essen prognosis classifications) in the affected eye (unilateral cases) or the more severely affected eye (bilateral cases). CONCLUSIONS: This study shows that there is a dedicated group of international ophthalmologists and other physicians who are willing to participate in multicenter cooperative clinical studies of retinoblastoma and that a relatively large group of children with this ocular malignancy can be enrolled within this framework during a reasonably short time for the purposes of future studies.

Meningeal dissemination of retinoblastoma: CT findings in eight patients.

Thirty-seven patients with retinoblastoma were evaluated prospectively by clinical examination, lumbar puncture, and CT. Eight (22%) of the 37 were found to have meningeal dissemination. The tumor was bilateral in three patients. Two cases showed no CT signs of local recurrence. Headache, nausea/vomiting, and restlessness were the most common symptoms. CT scans in these patients showed diffuse meningeal contrast enhancement, nodular masses, ependymal-subependymal enhancement, and ventricular dilatation. Our series of eight patients with meningeal spread illustrates a considerable range of dissemination patterns referable to retinoblastoma. One case illustrated the CT finding of multiple epidural metastases.