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)

Clinical Manifestations and Surgical Outcomes of Double Elevator Palsy

PURPOSE: To evaluate the clinical manifestations of double elevator palsy and describe the surgery outcomes in patients. METHODS: We performed a retrospective chart review of all patients who were treated surgically for double elevator palsy between 1999 and 2012 at Yonsei University, Severance Hospital in Seoul, Korea. RESULTS: Overall, 15 subjects (7 males and 8 females) with a mean age of 14.6 years (range, 3-40 years) underwent their first surgery during the study period. All patients received inferior rectus recession as a primary procedure. Nine patients (60.0%) underwent a secondary procedure which included 4 cases of horizontal muscle transposition, 2 cases of correction of exotropia, and 3 cases of correction of hypotropia and exotropia simultaneously. The mean preoperative hypotropia was decreased from 29.9 +/- 8.4 prism diopter (PD) to 4.7 +/- 5.3 PD postoperatively. Mean follow-up period was 40.9 +/- 48.2 months. Seven patients (46.7%) underwent eyelid surgery for true ptosis. At last follow-up, a majority of patients showed mild or no amblyopia. CONCLUSIONS: Primary inferior rectus recession and additive secondary horizontal muscle transposition surgery was effective in treatment of double elevator palsy. The clinical manifestations and surgical outcomes of monocular elevation deficiency in the present study can help in the treatment of Korean patients.

Doble parálisis de los elevadores y su comportamiento clínico-quirúrgico en Ciudad de La Habana, Cuba / Double elevador palsy and its clinical surgical behavior in the City of Havana, Cuba

OBJETIVO: Describir los resultados clínico-quirúrgicos de los pacientes con doble parálisis de los elevadores. MÉTODOS: Se realizó un estudio descriptivo retrospectivo a 16 pacientes que acudieron al servicio de oftalmología pediátrica y estrabismo del Instituto Cubano de Oftalmología "Ramón Pando Ferrer", desde septiembre de 2004 hasta septiembre de 2006, en el que se analizaron las siguientes variables: etiología, motilidad ocular, ausencia de signo de Bell, ptosis o pseudoptosis palpebral, resultados de la técnica quirúrgica aplicada. La técnica utilizada fue la transposición completa de los rectos horizontales al recto superior. RESULTADOS: La etiología fue congénita en todos los pacientes. El 100 por ciento de la muestra presentó hipotropía en posición primaria de mirada con marcada limitación de la elevación activa, tanto en aducción como abducción, con elevación del mentón y signo de Bell ausente. El 62,5 presentaba pseudoptosis y el 37,5 ptosis. Alcanzaron ortotropia 14 pacientes y dos quedaron con hipotropía menor de 15 dioptrías prismáticas. CONCLUSIONES: Se obtuvieron buenos resultados quirúrgicos en casi la totalidad de los pacientes.

OBJECTIVE: To describe the clinical surgical outcomes of patients with double elevator palsy. METHODS: A retrospective descriptive study of 16 patients, who went to the ophthalmologic pediatric strabismus service at "Ramón Pando Ferrer" Cuban Institute of Ophthalmology from September 2004 to September 2006, was carried out based on the following variables: etiology, ocular motility, lack of Bell´s sign, palpebral pstosis or pseudoptosis, and results of the applied surgical technique. The surgical method was the complete transposition of horizontal recti to upper rectus. RESULTS: There was congenital etiology in all the patients. The whole sample presented with hypotropia in the primary position of the look, with significant restriction of active elevation both in adduction and abduction, rise of chin and lack of Bell´s sign. Pseudoptosis was seen in 62,5 percent of patients whereas 37,5 percent showed ptosis. Fourteen patients managed to reach orthotropia and only two remained with hypotropia under 15 prismatic dioptres CONCLUSIONS: Surgical results were satisfactory in most of the patients.

Evaluation and Treatment of True Ptosis in Double Elevator Palsy

PURPOSE: To estimate the predictable factors of postoperative true ptosis remaining after squint surgery in patients with double elevator palsy (DEP), and to analyze the factors that must be considered before the correction of true ptosis. METHODS: We retrospectively reviewed the medical records of 6 patients who had undergone strabismus surgery for DEP at the Department of Ophthalmology, Yeungnam University College of Medicine, between 1989 and 2003. The MRD (margin reflex distance) of paretic and nonparetic eye with each eye fixation, palpebral aperture, levator function, and Bell's phenomenon were analyzed before and after squint surgery. RESULTS: True ptosis was noted in 3 patients, one of whom underwent external levator resection. Postoperative Bell's phenomenon improved in 2 patients who had poor Bell's phenomenon preoperatively. Scleral show remained in 2 patients who underwent inferior rectus recession, and was taken into account before the correction of ptosis. CONCLUSIONS: Upper lid drooping in DEP patients is expected to be improved after squint surgery if preoperative MRD of paretic eye with paretic eye fixation has the same measured amount as that of non-paretic eye. MRD, Bell's phenomenon and the amount of scleral show after the squint surgery should be carefully examined and assessed before the correction of residual ptosis.

A Case of Double Elevator Palsy Associated with Blepharoptosis

Double elevator palsy(DEP) is characterized by monocular limitation of upgaze in both abducted and adducted position. It is often associated with hypotropia, blepharoptosis, and pseudoblepharoptosis of the affected eye. The authors experienced a case of primary inferior rectus restrictive type of DEP, which has showed hypotropia of 40 prism diopters, and blepharoptosis on the right eye. Examinations revealed poor Bell's phenomenon, and forced duction test showed the limitation of elevation on the right eye. Surgery was performed with 6mm recession of the right inferior rectus. Postoperatively the elevation of glove was satisfactory, Bell's phenomenon was improved and primary position was also orthophoric. Correction of blepharoptosis with frontalis slinging could safely performed in the presence of adequate Bell's phenomenon after the inferior rectus recession.

Congenital Double Elevator Palsy in Two Brothers

Double elevator palsy (DEP) is an unusual anomaly of ocular motility in which both elevator muscles of the same eye are weak with a resultant inability or reduced ability to elevate the eye and hypotropia. This anomaly is occured congenitally, but occasionally occured as an acquired form. Congenital DEP, which is main cause of the monocular elevation paralysis, is characterized by the progressive deterioration and the absence of diplopia. The etiology is obscure, but is thought to be supranuclear lesion. We observed DEP of the same side of eyes in two patients who were brothers. The limitation of elevation and hypotropia were improved after Knapp's transposition procedure or Jensen's procedure.