Brown syndrome: clinical features and results of superior oblique tenotomy / Síndrome de Brown: quadro clínico e resultados da tenectomia do oblíquo superior

ABSTRACT Purpose: This study was conducted to further define the specific clinical characteristics of patients with Brown syndrome and evaluate the outcomes of superior oblique tenotomy in its surgical management. Methods: A retrospective analysis of the medical charts of 45 patients with Brown syndrome was performed, which revealed that 11 patients underwent superior oblique tenotomy due to abnormal head posture and/or hypotropia and 1 patient underwent bilateral superior oblique tendon elongation with a silicone band due to abnormal head posture. In the last patient, silicone bands were removed at the postoperative 3rd month due to the lack of improvement in the abnormal head posture and the limitation of elevation in adduction. Simultaneous horizontal rectus muscle surgery was performed in four patients. Results: There was a predominance of female gender, right eye, congenital form, unilaterality, A-pattern, and an abnormal head posture type with a combination of chin up and head tilting. Bilateral form was observed only in female patients. Amblyopia was detected in two patients. Among patients aged >5 years, 40% had reduced stereopsis. Abnormal head posture was found in 60% of patients. More than half of them were diagnosed with a vertical and/or horizontal deviation. Tenotomy procedure eliminated the abnormal head posture in all patients and significantly improved the mean limitation of elevation in adduction and hypotropia (p=0.001, p=0.012). Two patients developed inferior oblique overaction in the operated eye. There was complete spontaneous resolution in two patients. Conclusions: The clinical features of patients with Brown syndrome in our study are considerably consistent with those of previous reports. The present study demonstrated the effectiveness of superior oblique tenotomy with less overcorrection in the surgical treatment of Brown syndrome.(AU)

RESUMO Objetivo: Definir mais detalhadamente as características clínicas específicas de pacientes com síndrome de Brown e avaliar os resultados da tenectomia do músculo oblíquo superior no manejo cirúrgico da síndrome de Brown. Métodos: Prontuários de 45 pacientes com síndrome de Brown foram analisados retrospectivamente. Onze pacientes submetidos à tenectomia do músculo oblíquo superior devido a postura anormal da cabeça ou a hipotropia e um paciente submetido ao alongamento bilateral do tendão do oblíquo superior com uma faixa de silicone devido a postura anormal da cabeça. Neste último paciente, a faixa de silicone foi removida no terceiro mês pós-operatório devido à ausência de melhora na postura anormal da cabeça e à limitação da elevação em adução. Quatro pacientes submeteram-se simultaneamente à cirurgia do músculo reto horizontal. Resultados: Houve predominância de sexo feminino, olho direito, forma congênita, acometimento unilateral, padrão em "A" e um tipo de postura anormal da cabeça combinando queixo elevado e inclinação da cabeça. A forma bilateral foi vista apenas em pacientes do sexo feminino. Foi constatada ambliopia em 2 pacientes. Dentre os pacientes acima de 5 anos de idade, 40% tinham estereopsia reduzida. Postura anormal da cabeça estava presente em 60% dos pacientes. Mais da metade dos pacientes foi diagnosticada com um desvio vertical, horizontal ou ambos. O procedimento de tenectomia eliminou a postura anormal da cabeça em todos os pacientes e melhorou significativamente a limitação média da elevação em adução e a hipotropia (p=0,001 e p=0,012). Dois pacientes desenvolveram hiperação do músculo oblíquo inferior no olho operado. Resolução completa ocorreu espontaneamente em 2 pacientes. Conclusões: O quadro clínico dos pacientes com síndrome de Brown no nosso estudo é bastante consistente com os relatos iniciais na literatura. Este estudo mostrou a eficácia da tenectomia do oblíquo superior, com menor hipercorreção no tratamento cirúrgico da síndrome de Brown.(AU)

Síndrome de flutter ocular e ataxia de tronco pós-infecciosa / The post-infectious ocular flutter syndrome and truncal ataxia

OBJETIVO: Descrever um quadro clínico subagudo pós-infeccioso caracterizado por ataxia de tronco e flutter ocular. RELATO DE CASO: Um homem de 37 anos previamente hígido, pouco após um quadro sistêmico inespecífico que se resolveu espontaneamente, deu início a movimentos involuntários hipercinéticos dos olhos, da cabeça e desequilíbrio importante. Os sintomas evoluíram em poucos dias. Ao exame, apresentava ataxia de tronco e presença de flutter ocular. O líquor mostrou pleocitose discreta. Ressonância magnética (RM) de crânio e exames laboratoriais normais. Houve melhora espontânea e total dos sintomas em cerca de três semanas. CONCLUSÃO: A síndrome de flutter ocular e ataxia de tronco é rara e é destacado a benignidade do quadro.

OBJECTIVE: To describe a post-infectious subacute clinical picture characterized by truncal ataxia and ocular flutter. CASE REPORT: A healthy 37-year-old man, a few days after spontaneously resolved nonspecific systemic disease, initiated involuntary hyperkinetic movements of the eyes, head, and major imbalance. The symptoms progressed within a few days. He presented ataxia of the trunk and presence of ocular flutter. CSF showed pleocytosis. Magnetic resonance of the skull and laboratory tests were normal. There was spontaneous and total improvement of symptoms in about three weeks. CONCLUSION: The ocular flutter syndrome and truncal ataxia is rare and the benignity of the condition is highlighted.

Fundamentos para una plataforma nacional de interconsulta digital en trastornos de la motilidad ocular / Foundations for a national platform of digital consultation related to eye movement disorders

Se realizó estudio descriptivo de 187 pacientes recibidos en atención terciaria de Neuroftalmología, desde fuera de la capital, por trastornos de la motilidad ocular, entre enero/2010 y diciembre/2011, con el objetivo de caracterizar las interconsultas físicas por trastornos de la motilidad ocular solicitadas a servicios de Neuroftalmología de nivel terciario. Las variables estudiadas describieron particularidades epidemiológicas, origen y finalidad de las remisiones. Se evaluó además la posibilidad de interconsulta digital como opción alternativa en cada uno de los casos y sus beneficios potenciales. El 63,1 por ciento de las remisiones procedieron desde fuera de la región occidental, donde se encuentran enclavados los dos centros de atención terciaria de la especialidad. La distancia global recorrida por remisiones físicas fue de 70 286,9 Km. Del total de remisiones realizadas, 131 (70,1 por ciento) fueron clasificadas tributarias de interconsulta digital, lo que representaba una reducción de 54 823,2 Km en términos de distancia global recorrida. Las 56 remisiones restantes pudieron beneficiarse de interconsulta previa a la remisión física según criterio de experto, fundamentalmente para inicio de tratamiento e indicación de estudios complementarios. La Telemedicina aplicada a los trastornos de la motilidad ocular constituye una realidad necesaria y posible con los recursos humanos y estructurarles actuales con los que cuenta la especialidad en el país(AU)

This paper presents a descriptive study in 187 patients with eye movement disorders, received in Third-level Neurophthalmology Units, from far outside Havana, between January/2010 and December/2011, in order to characterize office consultation related to eye movement disorders in Third-level Neurophthalmology Units. Various subjects connected to epidemiological elements, genesis and purpose of the office evaluation were studied. The possibility and advantages of digital consultation were evaluated in each case. Interconsultation from outside the west region, where are both Third-level Neurophthalmology Units, were 63,1 percent. Total traveled-length owing to office evaluation was 70 286, 9 Km. 131 (70,1 percent) patients were classified as candidate for digital cases, reducing 54 823,2 Km the global traveled-length. For the remaining 56 cases, telemedicine consultation previous to the office one could be helpful according to the expert consensus, mainly to begin treatment or to do complementary tests. Telemedicine for eye movement disorders is in fact necessary. It is also possible oriented to all people and structural tools that have the specialty around the country(AU)

Synergistic convergence and split pons in horizontal gaze palsy and progressive scoliosis in two sisters.

Synergistic convergence is an ocular motor anomaly where on attempted abduction or on attempted horizontal gaze, both the eyes converge. It has been related to peripheral causes such as congenital fibrosis of extraocular muscles (CFEOM), congenital cranial dysinnervation syndrome, ocular misinnervation or rarely central causes like horizontal gaze palsy with progressive scoliosis, brain stem dysplasia. We hereby report the occurrence of synergistic convergence in two sisters. Both of them also had kyphoscoliosis. Magnetic resonance imaging (MRI) brain and spine in both the patients showed signs of brain stem dysplasia (split pons sign) differing in degree (younger sister had more marked changes).

Botulinum toxin in the management of acquired motor fusion deficiency.

Acquired disruption of motor fusion is a rare condition characterized by intractable diplopia. Management of these patients is extremely difficult. Prisms in any combination or even surgery may not help relieve their symptoms. We describe a longstanding case of acquired motor fusion disruption which was managed successfully with botulinum toxin injection.

Preferred clinical practice in convergence insufficiency in India: a survey.

PURPOSE: Convergence insufficiency (CI) is a common binocular vision disorder. However, there is a lack of consensus regarding the treatment most appropriate for CI. The aim of the study was to investigate the treatment for CI by surveying the ophthalmologists regarding the most common treatment modalities used in India. MATERIALS AND METHODS: Four hundred questionnaires were distributed amongst ophthalmologists attending different sessions of the Delhi Ophthalmological Society annual conference held in April 2007. Two hundred and three ophthalmologists responded (response rate 50.75%). The responders included 109 private practitioners, 57 consultants attached to teaching institutes and 37 residents. RESULTS: The majority of ophthalmologists (66.7%) claimed encountering > 5% outpatient department patients with CI. Pencil push-ups therapy (PPT) was the most common first line of treatment offered by ophthalmologists (79%) followed by synoptophore exercises (18%). Only 3% referred the patients to optometrists. Thirty per cent ophthalmologists claimed good results with PPT, which was significantly higher in private practitioners (35%). Only 26% ophthalmologists explained physiological diplopia to patients on a regular basis and reported significantly higher percentage of patients (46.3%) with good results. Only 12.3% ophthalmologists needed to refer > 30% patients for synoptophore exercises. For failure of PPT 86.7% considered lack of compliance as the major reason as perceived by ophthalmologists. CONCLUSIONS: This survey suggested that most ophthalmic practitioners prescribed PPT as the initial treatment for CI and had satisfactory results with PPT. The majority of the practitioners did not explain to the patient about physiological diplopia. Explaining physiological diplopia may improve outcome, as perceived from the survey.

Surgical Outcomes in Correction of Brown Syndrome

PURPOSE: To evaluate the outcomes of surgery for Brown syndrome. METHODS: We reviewed the charts of 15 patients who underwent surgery for Brown syndrome. The limitation of elevation in adduction (LEA) ranged from -2 to -4 degrees. A superior oblique muscle (SO) tenotomy was performed in 4 patients, a silicone expander was inserted in the SO of 9 patients, and a SO recession was performed in 2 patients. The results of surgery were analyzed with a follow-up period of more than 6 months, 42.3+/-48.42 months on average. RESULTS: Nine female patients and 6 male patients with unilateral Brown syndrome were selected for this study. The left eye was the affected eye in 9 patients. The degree of preoperative LEA was -2 to -4 in 4 patients in whom SO tenotomy was performed, -3 to -4 in 9 patients treated with the silicone expander, and -2 to -4 in 2 patients treated with SO recession. The LEA was released after surgery in all patients without postoperative adhesion. However, unilateral overaction of the inferior oblique muscle due to excessive weakening of the SO occurred in 1 patient with tenotomy (25%) and in 1 patient with insertion of a silicone expander (11%). CONCLUSIONS: LEA was released after tenotomy, insertion of a silicone expander and recession of the SO in 13 of 15 patients with Brown syndrome. SO palsy due to overcorrection and under-correction with postoperative adhesion should be avoided.

Torsiones oculares: seudohiperfunción de los oblicuos inferiores / Ocular torsions: inferior oblique overaction

Se plantea que la extorsión bilateral produce modificaciones globales de las acciones de los músculos rectos que pueden determinar el desarrollo de una desviación tipo -V- y simular una sobreacción de los oblicuos inferiores, con elevación en adducción. El análisis del siguiente caso clínico sugiere los planteamientos anteriores. Se trata de una paciente con endotropia alternante, hipermetropia alta y astigmatismo, con doble elevación en addución con lentes, la cual desaparece al retirarlos. Se obtiene ortoposición postoperatoria, con lentes persiste la elevación en addución. El cuadro se mantiene estable desde los 6 años (fecha de la operación) hasta los 32 (último control). El diagnóstico de extorsión se hizo mediante el test de Maddox modificado y fotografías del fondo de ojo, la cual puso en evidencia una extorsión de 15º en cada ojo en PPM y una disminución total de 4º durante la adducción. Esta pérdida de extorsión produce una reconversión del movimiento horizontal en elevación con modificación de los planos de acción de los rectos medios y upshoot en adducción, sin sobreacción de los oblicuos inferiores, los cuales no se contraen en las miradas horizontales.

Bilateral ocular extorsion may produce global modifications on recti eye muscles action with V pattern strabismus and bilateral upshoot, with apparent inferior oblique overaction. The report of the following case is supportive of this concept. We present a patient having alternant esotropia, high hyperopia and astigmatism, with bilateral upshoot with glasses, that disappears without them. The patient is orthophoric after surgical treatment. This patient remain in the same condition from the age of six (when she was operated on) until today (32 years old). The double red Maddox lenses and fundus photography were used to reveal a 15º bilateral extorsion diagnosis, and the further loss of abnormal torsion in extreme adduction. This degree of torsional change is converted in hyperdeviation, explaining the upshoot in adduction without any inferior oblique muscle overaction.

Síndrome de Fells-Waddel / Waddell Fells syndrome

Objetivo: Describir la fenomenología, génesis y alteraciones musculares del Síndrome de Fell-Waddel (FW) como parte de los desórdenes inervacionales congénitos. Se presentan dos casos. Método: Se realizó un análisis de la genética del rombencéfalo para comprender las posibles alteraciones del FW. Se analizaron dos casos, a los que se realizó la cirugía bajo anestesia tópica para el análisis de las funciones musculares. Resultados: En ambos casos se demostró la restricción en las diferentes rotaciones oculares, siendo mayor en el adulto. El debilitamiento de los rectos inferiores provocó una exotropia importante al frente, la divergencia en el intento de depresión y convergencia en el intento de elevación que tuvo sólo leve mejoría con el debilitamiento de los oblicuos superiores, en el segundo caso se analiza con cirugía bajo anestesia tópica su persistencia con los oblicuos superiores desinsertados. Conclusiones: Considerando la anomalía reportada de la división superior del III nervio en las disgnesias congénitas, podemos atribuir los cambios presentados en ambos casos a inervaciones aberrantes de la división inferior del III nervio craneal a los rectos laterales lo que correspondería con la divergencia e el intento de depresión, y a su vez la convergencia en el intento de elevación seria secundaria a la orden inhibitoria a los rectos laterales, quedando funcionando los restos mediales. El debilitamiento de los rectos inferiores, cirugía más frecuente en los síndromes de fibrosis de músculos extraoculares tipo I provocará una exotropia al frente que debe ser corregida.

Objective: To describe clinical characteristics, genetic and muscular anomalies in Fells-Waddel syndrome (FW), a congenital cranial dysinnervation disorder. Method: We analyzed the genetics of rhombencephalon to explain alterations in FW. We present two cases of FW which underwent surgery where orbital alterations were demonstrated. Topical anesthesia was used in the second case for analysis of muscular functions. Results: Restriction was demonstrated in both cases, but it was worse in the adult. Recession of the inferior rectus caused a big exotropia. Divergence in depression and convergence in elevation were improved only partially after recession of superior oblique. In case 2, the patient was alert under topical anesthesia, and while these muscles were free, the anomalies persisted. Conclusions: There is an anomaly of superior division of III nerve in these cases, so these movements can be secondary to aberrant innervations of inferior division of the III nerve to the lateral rectus, this can explain divergence during depression. Convergence in elevation is explained because of the inhibitory effect on the lateral rectus while de medial rectus are still functional. Recession of inferior rectus is the most common surgery in these cases, which will cause a big exotropia that must be corrected.

Síndrome de Brown bilateral / Bilateral Brown syndrome

Objetivo: El síndrome de Brown (SB) se caracteriza por restricción activa y pasiva de la elevación en adducción. Puede ser congénito o adquirido, intermitente o permanente, uni o bilateral; este último se presenta en el 10 por ciento de los casos. Nuestro objetivo es describir 9 casos de SB bilateral y revisar la literatura al respecto. Pacientes: se describen características clínicas de 9 casos y se revisa la literatura. Resultados: De los 9 casos, 2 eran de sexo masculino, y 7 de sexo femenino. Edad promedio: 5.7 años. 1 caso intermitente. Los casos publicados en diferentes series son: 13/126 (Brown), 3/8 (Clark y Noel), 7/36 (Waddell ) y 1/30 (Eustis). Entre los casos bilaterales hay alta incidencia de casos familiares. Conclusión: Se confirma que el cuadro es más común en mujeres. 7/9 tenía alineamiento en posición primaria. Todos tenían déficit bilateral de elevación y anisotropía en V. Frente a un caso de SB bilateral es conveniente examinar a los familiares en busca de casos asintomáticos.

Objective: The motility defect known as Brown´s síndrome is characterized by active and passive restriction of elevation in adduction. It may be either congenital or acquired, intermittent or permanent, uni or bilateral. It is bilateral in 10 percent of cases. Our purpose is to report 9 cases with bilateral Brown´s syndrome and review the cases previously published. Patients: 9 cases are described: 2 males and 7 females, average age 5,7 years who had bilateral Brown´s syndrome and review. Results: 9 cases are described: 2 males and 7 females, average age 5,7 years who had bilateral Brown´s syndrome, 1 of them was intermittent. Bilateral cases published are: 13/126 in Brown´s series, 3/28 in Clark and Noel´s, 7/36 in Waddell´s and 1/30 in that of Eustis. Among bilateral cases there is a striking incidence of familial cases. Conclusion: Brown´s statement that the disorder is more common in females than in males is confirmed in our series. It should be noted that 7 out of 9 of these patients had normal alignment in primary gaze. All cases had bilateral elevation deficit and V pattern. In bilateral cases examination of the relatives should be performed in order to detect asymptomatic familial cases.

Modificaciones del ángulo de desviación horizontal en posición primaria de la mirada después del debilitamiento de oblicuos inferiores / Modifications of the horizontal deviation angle in vision primary position after inferior obliques weakening

En este trabajo retrospectivo se estudiaron los cambios del ángulo de desviación horizontal en posición primaria de la mirada (PPM), después del debilitamiento bilateral de oblicuos inferiores. Se evaluaron 21 pacientes con un año de evolución postoperatoria a quienes se les había efectuado, como única operación debilitamiento bilateral de oblicuos inferiores. Diez presentaban exotropia, 7 esotropia y 4 ortotropia. Se registró el ángulo en PPM, en extrema mirada arriba y en extrema mirada abajo; antes de la cirugía y al año de la operación. En el 48 porciento de los casos, en PPM se observó desplazamiento de los ojos hacia la convergencia, tanto en pacientes esotrópicos como exotrópicos (p=0,001)