La prematuridad: un antecedente de obligada consideración a la hora de valorar el complejo de células ganglionares de la retina / Prematurity: a medical history of obligatory consideration when assessing the retinal ganglion cell complex

Introducción: La supervivencia de los niños prematuros es cada vez más frecuente siendo cada vez más habitual encontrar pacientes con este antecedente en las consultas de oftalmología. El parto prematuro puede conllevar cambios estructurales a nivel ocular, pudiéndose afectar entre otras estructuras el complejo de células ganglionares (CCG), que puede ser estudiado mediante la tomografía de coherencia óptica.Materiales y métodosRealizar una revisión bibliográfica de los estudios que analizan el CCG en pacientes con antecedente de prematuridad y lo comparan con pacientes nacidos a término.ResultadosSe referencian varios estudios que analizan el CCG en población con antecedente de prematuridad y se estudian los distintos resultados obtenidos.ConclusionesEn nuestra práctica clínica, conocer el antecedente de prematuridad es fundamental en la valoración del CCG medido por tomografía de coherencia óptica ya que el grosor de esta capa es distinta en la población con antecedente de prematuridad comparada con la población a término. (AU)

Introduction: Premature children birth and survival is becoming more frequent due to the improvement in obstetric and neonatal care. This makes it increasingly common to find patients with history of preterm birth in ophthalmology clinics, both in pediatric and adult ages. Premature birth can lead to ocular structural changes, being possible to affect the ganglion cell complex (GCC), among other structures, which can be studied using optical coherence tomography.Materials and methodsTo carry out a bibliographic review of the studies that analyze GCC in patients with a history of prematurity compared with patients born at term.ResultsSeveral studies that analyze GCC in patients with a history of prematurity are referenced and their results are studied.ConclusionsIn our clinical practice, knowing the history of prematurity is fundamental in the assessment of GCC measured by optical coherence tomography, since this layer is different in the patients with a history of prematurity compared to patients born at term. (AU)

Prematurity: A medical history of obligatory consideration when assessing the retinal ganglion cell complex.

INTRODUCTION: Premature children birth and survival is becoming more frequent due to the improvement in obstetric and neonatal care. This makes it increasingly common to find patients with history of preterm birth in ophthalmology clinics, both in pediatric and adult ages. Premature birth can lead to ocular structural changes, being possible to affect the ganglion cell complex (GCC), among other structures, which can be studied using optical coherence tomography. MATERIALS AND METHODS: To carry out a bibliographic review of the studies that analyze GCC in patients with a history of prematurity compared with patients born at term. RESULTS: Several studies that analyze GCC in patients with a history of prematurity are referenced and their results are studied. CONCLUSIONS: In our clinical practice, knowing the history of prematurity is fundamental in the assessment of GCC measured by optical coherence tomography, since this layer is different in the patients with a history of prematurity compared to patients born at term.

Manifestaciones oftalmológicas en 6 pacientes con síndrome de Williams-Beuren y revisión de la literatura / Ophthalmological manifestations in 6 patients diagnosed with Williams-Beuren syndrome and literature review

Se revisó la historia clínica de 6 pacientes diagnosticados de síndrome de Williams-Beuren en la edad pediátrica. Todos los pacientes presentaron la facies de elfo característica y anomalías cardiovasculares. Todos presentaron buena agudeza visual, excepto un caso de ambliopía unilateral. El error refractivo más frecuente fue la hipermetropía (n=6; 100%) y el astigmatismo (n=5; 83,3%). En 2 pacientes se encontraron alteraciones de la motilidad ocular (un caso de exoforia con hiperfunción de oblicuo inferior derecho y otro de endotropía congénita con hiperfunción del oblicuo inferior bilateral). Cognitivamente un 66,7% (n=4) tenía alteraciones visoperceptivas. Otros hallazgos fueron epicantus (n=6; 100%) y obstrucción congénita del conducto nasolagrimal con epífora unilateral (n=1; 16,7%). El síndrome de Williams-Beuren es un trastorno poco frecuente con manifestaciones oftalmológicas y sistémicas complejas. Por ello, es recomendable realizar un seguimiento oftalmológico a estos niños (AU)

Medical history of 6 patients diagnosed with Williams-Beuren Syndrome (SWB) in pediatric age was revised. All the patients presented characteristic elf facies and cardiovascular abnormalities. All presented good visual acuity, except one case of unilateral amblyopia. The most frequent refractive error was hyperopia (n=6; 100%) and astigmatism (n=5; 83.3%). Ocular motility alterations were found in 2 patients (1 case of exophoria with hyperfunction of right inferior oblique and another of congenital endotropia with bilateral hyperfunction of inferior oblique). On the cognitive function, 66.7% (n=4) had visoperceptive disorders. Other findings were epicanthus (n=6; 100%) and congenital obstruction of the nasolacrimal duct with unilateral epiphora (n=1; 16.7%). SWB is a rare disorder with complex ophthalmological and systemic manifestations. For this reason, ophthalmological follow-up of these children is recommended (AU)

Ophthalmological manifestations in 6 patients diagnosed with Williams-Beuren syndrome and literature review.

Medical history of 6 patients diagnosed with Williams-Beuren Syndrome (SWB) in pediatric age was revised. All the patients presented characteristic elf facies and cardiovascular abnormalities. All presented good visual acuity, except one case of unilateral amblyopia. The most frequent refractive error was hyperopia (n = 6; 100%) and astigmatism (n = 5; 83.3%). Ocular motility alterations were found in 2 patients (1 case of exophoria with hyperfunction of right inferior oblique and another of congenital endotropia with bilateral hyperfunction of inferior oblique). On the cognitive function, 66.7% (n = 4) had visoperceptive disorders. Other findings were epicanthus (n = 6; 100%) and congenital obstruction of the nasolacrimal duct with unilateral epiphora (n = 1; 16.7%). SWB is a rare disorder with complex ophthalmological and systemic manifestations. For this reason, ophthalmological follow-up of these children is recommended.

Septo-optic dysplasia: Ophthalmological abnormalities in a series of 5 cases.

Septo-optic dysplasia (SOD) is a rare congenital condition of unknown cause, with a characteristic triad that includes optic nerve hypoplasia, pituitary function abnormalities and midline brain defects, in addition to a broad spectrum of symptoms and associations. A total of five clinical cases are presented, four of which met the complete classic triad. All of them showed a wide variety of ophthalmological, endocrinological and neurological alterations. Within the ophthalmological spectrum of SOD, papillary hypoplasia and ocular motility alterations (nystagmus, strabismus) stand out. Other less frequent ones may also appear, such as pupillary alterations, microphthalmia and coloboma. Given the suspicion of SOD, brain MRI scan should be performed, as well as consultation with the paediatric department in order to complete the study and indicate, if necessary, systemic treatment.

Displasia septoóptica: alteraciones oftalmológicas en una serie de 5 casos / Septo-optic dysplasia: ophthalmological abnormalities in a series of 5 cases

La displasia septoóptica (DSO) es una entidad congénita, rara, de causa desconocida, con una tríada característica que incluye hipoplasia de nervio óptico, anomalías en la función hipofisaria y defectos en la línea media cerebral, además de un amplio espectro de manifestaciones y asociaciones.Se presenta una serie de cinco casos clínicos, de los cuales cuatro presentaron la tríada clásica completa. Todos mostraron gran variedad de alteraciones oftalmológicas, endocrinológicas y neurológicas.Dentro del espectro oftalmológico de la DSO, destacan la hipoplasia papilar y las alteraciones de la motilidad ocular (nistagmos estrabismo). También pueden aparecer otras menos frecuentes, como son alteraciones pupilares, microftalmia y coloboma.Ante la sospecha de DSO, conviene realizar una resonancia magnética cerebral y una interconsulta con el servicio de pediatría para completar el estudio e indicar, si fuera preciso, tratamiento sistémico.

Septo-optic dysplasia (SOD) is a rare congenital condition of unknown cause, with a characteristic triad that includes optic nerve hypoplasia, pituitary function abnormalities and midline brain defects, in addition to a broad spectrum of symptoms and associations.A total of five clinical cases are presented, four of which met the complete classic triad. All of them showed a wide variety of ophthalmological, endocrinological and neurological alterations.Within the ophthalmological spectrum of SOD, papillary hypoplasia and ocular motility alterations (nystagmus, strabismus) stand out. Other less frequent ones may also appear, such as pupillary alterations, microphthalmia and coloboma.Given the suspicion of SOD, brain MRI scan should be performed, as well as consultation with the paediatric department in order to complete the study and indicate, if necessary, systemic treatment.

Orbital apex syndrome secondary to herpes zoster ophthalmicus.

Herpes zoster ophthalmicus usually presents with ocular manifestations, but neurological complications are much more infrequent. An 84-year-old woman with herpes zoster of the left first trigeminal branch developed herpetic keratouveitis in her left eye despite treatment with oral valaciclovir. Seven days later, a progressive and total left ophthalmoplegia appeared, requiring hospital admission and intravenous treatment with acyclovir and corticosteroids. The neuroimaging was suggestive of an orbital apex syndrome. The evolution of the ophthalmoplegia was favourable, with complete resolution at 5 months, but with decreased visual acuity due to the optic nerve involvement.

Síndrome de ápex orbitario por herpes zoster ophthalmicus / Orbital apex syndrome secondary to herpes zoster ophthalmicus

El herpes zoster ophthalmicus suele cursar con manifestaciones oculares, siendo mucho más infrecuentes las complicaciones de tipo neurológico. Una mujer de 84 años con herpes zóster en el dermatomo de la primera rama trigeminal izquierda, desarrolló una queratouveítis herpética en el ojo izquierdo a pesar del tratamiento con valaciclovir oral. A los siete días, además apareció una oftalmoplejia progresiva y total izquierda que requirió ingreso hospitalario y tratamiento intravenoso con aciclovir y corticoides. La neuroimagen fue sugestiva de un síndrome de ápex orbitario. La evolución de la oftalmoplejia fue favorable con resolución completa a los cinco meses de seguimiento, dejando una disminución de la agudeza visual por la afectación del nervio óptico. (AU)

Herpes zoster ophthalmicus usually presents with ocular manifestations, but neurological complications are much more infrequent. An 84-year-old woman with herpes zoster of the left first trigeminal branch developed herpetic keratouveitis in her left eye despite treatment with oral valaciclovir. Seven days later, a progressive and total left ophthalmoplegia appeared, requiring hospital admission and intravenous treatment with acyclovir and corticosteroids. The neuroimaging was suggestive of an orbital apex syndrome. The evolution of the ophthalmoplegia was favourable, with complete resolution at 5 months, but with decreased visual acuity due to the optic nerve involvement. (AU)

Microftalmia bilateral asociada con quistes orbitarios. Manejo individualizado según el potencial visual. Reporte de caso clínico / Bilateral microphthalmos with bilateral orbital cysts: individualising the treatment according to the visual potential. A case report

Niño de tres meses diagnosticado de microftalmia y quistes orbitarios bilaterales. En la resonancia magnética se objetivó una microftalmia bilateral con quiste moderado en ojo derecho (OD) y quiste severo en ojo izquierdo. La exploración sugería un posible potencial visual del OD. Se decidió solo observación. A los dos años, la exploración mostraba una disminución significativa de los quistes con una agudeza visual de movimiento de manos gracias a la visión residual del OD. La microftalmia con quistes orbitarios es una anomalía congénita rara. Los quistes tienden a crecer con el tiempo. Actualmente no existe un protocolo estandarizado de manejo de esta enfermedad. A diferencia de nuestro caso, la agudeza visual en estos pacientes es normalmente de no percepción de la luz. La observación puede ser una alternativa terapéutica en casos severos ya que existe la posibilidad de una disminución, desplazamiento o regresión espontánea de los quistes

The case is presented of a 3-month-old infant diagnosed with microphthalmos and orbital cysts. Magnetic Resonance Imaging revealed a bilateral microphthalmia with a moderate right cyst and a severe left cyst. Visual potential of the right eye was uncertain. Non-surgical treatment was decided. At the age of 2 years old, physical examination showed a significant decrease of the cysts and visual acuity of hand movements due to the residual vision of his right eye. Microphthalmos with orbital cyst is a rare congenital anomaly. Cysts tend to enlarge with time. At the moment, no standard protocol for the management of this pathology has been described. Unlike in our patient, visual acuity in these patients is usually of no-light perception. The observation could be an alternative in severe cases, since there is a possibility of spontaneous reduction, displacement, or regression of the cysts

Bilateral microphthalmos with bilateral orbital cysts: individualising the treatment according to the visual potential. A case report. / Microftalmia bilateral asociada con quistes orbitarios. Manejo individualizado según el potencial visual. Reporte de caso clínico.

The case is presented of a 3-month-old infant diagnosed with microphthalmos and orbital cysts. Magnetic Resonance Imaging revealed a bilateral microphthalmia with a moderate right cyst and a severe left cyst. Visual potential of the right eye was uncertain. Non-surgical treatment was decided. At the age of 2 years old, physical examination showed a significant decrease of the cysts and visual acuity of hand movements due to the residual vision of his right eye. Microphthalmos with orbital cyst is a rare congenital anomaly. Cysts tend to enlarge with time. At the moment, no standard protocol for the management of this pathology has been described. Unlike in our patient, visual acuity in these patients is usually of no-light perception. The observation could be an alternative in severe cases, since there is a possibility of spontaneous reduction, displacement, or regression of the cysts.

Bases de datos normativas en edad pediátrica para tomografía de coherencia óptica: una clara necesidad / Paediatric optical coherence tomography normative databases: A real need

INTRODUCCIÓN: La tomografía de coherencia óptica (OCT) se ha convertido en una herramienta imprescindible en las consultas de oftalmología pediátrica. Sin embargo, las OCT disponibles en la actualidad no incluyen ninguna base de datos normativa para población pediátrica, lo que puede conllevar errores importantes de interpretación. OBJETIVO: Realizar una revisión bibliográfica sobre las bases de datos normativas para OCT realizadas en población pediátrica. MATERIAL Y MÉTODOS: Se revisan las aplicaciones e implicaciones del uso de las bases de datos normativas pediátricas para OCT. RESULTADOS: Se referencian varios estudios realizados para extraer estas bases de datos con diferentes modelos de OCT en población española y europea con el fin de disponer de ellos de manera resumida. CONCLUSIONES: En nuestra práctica clínica diaria es muy importante el conocimiento y manejo de las bases de datos normativas pediátricas para la correcta interpretación de los mapas de grosores de OCT

INTRODUCTION: Optical coherence tomography (OCT) has become an essential tool in paediatric ophthalmology. However, none of the currently available OCT devices include any kind of normative database for the paediatric population, which can lead to important interpretation errors. OBJECTIVE: To review the paediatric OCT normative databases. MATERIAL AND METHODS: The applications and implications of the use of paediatric OCT normative databases are reviewed. RESULTS: The paediatric normative databases that have been published so far in scientific literature with different OCT devices for Spanish and European population are presented. CONCLUSIONS: The knowledge and interpretation of paediatric OCT normative databases in our daily clinical practice is crucial in order for the correct interpretation of OCT thickness maps

Paediatric optical coherence tomography normative databases: A real need. / Bases de datos normativas en edad pediátrica para tomografía de coherencia óptica: una clara necesidad.

INTRODUCTION: Optical coherence tomography (OCT) has become an essential tool in paediatric ophthalmology. However, none of the currently available OCT devices include any kind of normative database for the paediatric population, which can lead to important interpretation errors. OBJECTIVE: To review the paediatric OCT normative databases. MATERIAL AND METHODS: The applications and implications of the use of paediatric OCT normative databases are reviewed. RESULTS: The paediatric normative databases that have been published so far in scientific literature with different OCT devices for Spanish and European population are presented. CONCLUSIONS: The knowledge and interpretation of paediatric OCT normative databases in our daily clinical practice is crucial in order for the correct interpretation of OCT thickness maps.

Sarcoma pleomorfo indiferenciado de alto grado orbitario: a propósito de un caso / Undifferentiated high-grade pleomorphic sarcoma of the orbit: A case report

CASO CLÍNICO: Mujer de 27 años con proptosis del ojo derecho. Las pruebas de imagen mostraron una lesión extracónica en el tercio anterior medial de la órbita que afectaba al músculo recto interno. Se realizó exéresis del tumor con radioterapia adyuvante. El diagnóstico fue de sarcoma pleomorfo indiferenciado de alto grado. Tras 6 años de seguimiento no se ha observado recidiva. DISCUSIÓN: El sarcoma pleomorfo indiferenciado es una neoplasia maligna muy infrecuente en la región orbitaria. Tiene un rápido crecimiento local y es potencialmente metastatizante, por lo que debe hacerse estudio de extensión. El tratamiento de elección es la cirugía (AU)

CASE REPORT: A 27-year-old female presented to us with a complaint of proptosis of her right eye. Imaging tests showed an extraconal mass in the medial orbital wall, which affected the medial rectus. The tumor was removed and adjuvant radiotherapy was required. The histological examination showed an undifferentiated high-grade pleomorphic sarcoma of the orbit. There has not been recurrence after 6 years of follow-up. DISCUSSIÓN: Undifferentiated high-grade pleomorphic sarcoma is an uncommon cause of malignant orbital mass. It grows fast and can metastasize, so extension studies are required. Surgical excision is the treatment of choice (AU)

[Undifferentiated high-grade pleomorphic sarcoma of the orbit: a case report]. / Sarcoma pleomorfo indiferenciado de alto grado orbitario: a propósito de un caso.

CASE REPORT: A 27-year-old female presented to us with a complaint of proptosis of her right eye. Imaging tests showed an extraconal mass in the medial orbital wall, which affected the medial rectus. The tumor was removed and adjuvant radiotherapy was required. The histological examination showed an undifferentiated high-grade pleomorphic sarcoma of the orbit. There has not been recurrence after 6 years of follow-up. DISCUSSION: Undifferentiated high-grade pleomorphic sarcoma is an uncommon cause of malignant orbital mass. It grows fast and can metastasize, so extension studies are required. Surgical excision is the treatment of choice.