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 Clinical Experience of Postoperative Double Elevator Palsy after Surgical Removal of Schwannoma in Orbit and Cavernous Sinus

A patient who had been complaining of slowly progressive proptosis for two months came to the authors`hospital. Brain MRI was taken promptly to show an intraorbital lesion highly suspicious of schwannoma. We performed an operation in concert with neurosurgeons in which the intraorbital mass was totally resected through orbitozygomatic and extradural approach. After the surgery, ptosis of right upper eyelid and right hypertropia of 22 prism developed as a complication. Another operation was performed 7 months after the initial resection of the mass, in which right inferior rectus muscle was recessed and a silicone sleave was inserted for right frontalis sling. No further complication developed thereafter. Presently, the patient has right hypotropia of 8 prism and only a few creases on right upper eyelid. Orbital schwannoma is a rare disease and comprises approximately 1 percent of all orbital tumors. The authors experienced postoperative double elevator palsy as well as sinus and intraorbital schwannoma. We report this case with review of the literature.

Differential Diagnosis of Atypical Brown Syndrome and Primary Inferior Rectus Restriction of Double Elevator Palsy

Both atypical Brown syndrome (ABS) and inferior rectus restriction of double elevator palsy (DEP) are characterized by monocular limitations of upgaze in primary, abducted position as well as abducted position. Futhermore, they exhibit resistance of elevation in adduction during forced duction test and should be considered in the differential diagnosis. We gained several differential points from 6 year-old female with ABS and a 12 year-old female with DEP due to inferior rectus restriction (IRR). In forced duction test under general anesthesia, ABS exhibited resistance of elevation in adduction and IRR of DEP showed resistance of elevation in all upward direction. DEP showed an impairment of Bell`s phenomenon and mild pseudoptosis preoperatively in which ABS never showed. ABS displayed divergence in upgaze producing V-pattern, and further limitation of upgaze in adduction than DEP. Superior oblique lengthening procedure using silicone expander was performed for ABS. Ipsilateral inferior rectus and contralateral superior rectus recession were performed for IRR of DEP. The results for elevation in adduction, primary position and abduction were satisfactory in both diseases. In conclusion, the direction of resistance in forced duction test, Bell` s phenomenon, the degree of elevation in abducted and abducted position, presence of pseudoptosis and divergence in upgaze producing 3 V-pattern may be helpful to differentiate ABS from IRR of DEP.

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.

4 Cases of Double Elevator Palsy

Double elevator palsy(DEP) is rare paralytic anomaly of ocular motility due to monocular paresis of both elevator muscles. Clinically, DEP is classified into the pure paralytic, restricted and mixed types. The authors describe the clinical experiences of 2 cases of purely paralytic type of DEP and 2 cases of restricted type of DEP treated at Department of Ophthalmology, Pusan Pail, Hospital, Inje Medical College from January 1988 to January 1991. The results were as follows: 1) In the pure paralytic type of DEP, the hypotropia was below 30 prism diopters in the primary position and in the restricted type, greater than 60 prism diopters preoperatively. 2) Preoperatively, the pseudoptosis was shown in 2 cases of purely paralytic type of DEP and it was disappeared postoperatively. The Bell's phenomenon was shown the negative result in 2 cases of restricted type of DEP. 3) For the pure paralytic type, the both vertical and horizontal deviation were corrected completely in primary position by the transposition and recession of the horizontal rectus muscles at one surgery and the limitation of ocular motility remained more and less in the elevation postoperatively but no limitation in the adduction and abduction. For the restricted type, the tenectomy of the inferior rectus muscle corrected about 40 prism diopters of hypotropia without the limitation of the infraduction.