Unusual and Rare Causes of Monocular Elevation Deficit.

INTRODUCTION: To study the rare and unusual causes of monocular elevation deficit. METHODS: Five patients presenting to us with diplopia and elevation deficit were thoroughly examined and were found to have monocular elevation deficit due to rare causes. OBSERVATIONS: All five were found to have different underlying etiologies - iatrogenic, sphenoid wing meningioma, cysticercosis, sarcoidosis and mid brain infarct, and were managed appropriately. DISCUSSION: Monocular Elevation Deficit can occur due to a variety of causes. Having a high index of suspicion for the more serious etiologies is of utmost importance. Thorough clinical examination and imaging help clinch the diagnosis.

Marcus Gunn Jaw-Winking Phenomenon and Monocular Elevation Deficiency in Association With Congenital Ptosis.

Marcus Gunn jaw winking (MGJW) is an uncommon entity and is associated with congenital ptosis. It is a neurogenic congenital ptosis, which is also called the Marcus Gunn phenomenon, trigeminal-oculomotor synkinesis, or pterygoid-levator synkinesis. Congenital ptosis can be associated with MGJW syndrome, blepharophimosis syndrome, and monocular elevation deficiency (MED). MED is a condition where there is a unilateral congenital abnormality in the elevation of the eye in abduction and adduction. The MGJW phenomenon, congenital ptosis, and double-elevator palsy may be associated with and represent a congenital misdirection syndrome. Together, it can be challenging, and surgery is recommended in severe cases, depending on the degree of ptosis and jaw winking. We hereby want to bring to light one such case of a 14-year-old female with congenital ptosis, MGJW, and double-elevator palsy and want to highlight how both MGJW and double-elevator palsy are both parts of the same disease spectrum and how such cases can be treated.

Congenital monocular elevation deficiency associated with a novel TUBB3 gene variant.

BACKGROUND: The genetic basis of monocular elevation deficiency (MED) is unclear. It has previously been considered to arise due to a supranuclear abnormality. METHODS: Two brothers with MED were referred to Leicester Royal Infirmary, UK from the local opticians. Their father had bilateral ptosis and was unable to elevate both eyes, consistent with the diagnosis of congenital fibrosis of extraocular muscles (CFEOM). Candidate sequencing was performed in all family members. RESULTS: Both affected siblings (aged 7 and 12 years) were unable to elevate the right eye. Their father had bilateral ptosis, left esotropia and bilateral limitation of elevation. Chin up head posture was present in the older sibling and the father. Bell's phenomenon and vertical rotational vestibulo-ocular reflex were absent in the right eye for both children. Mild bilateral facial nerve palsy was present in the older sibling and the father. Both siblings had slight difficulty with tandem gait. MRI revealed hypoplastic oculomotor nerve. Left anterior insular focal cortical dysplasia was seen in the older sibling. Sequencing of TUBB3 revealed a novel heterozygous variant (c.1263G>C, p.E421D) segregating with the phenotype. This residue is in the C-terminal H12 α-helix of ß-tubulin and is one of three putative kinesin binding sites. CONCLUSION: We show that familial MED can arise from a TUBB3 variant and could be considered a limited form of CFEOM. Neurological features such as mild facial palsy and cortical malformations can be present in patients with MED. Thus, in individuals with congenital MED, consideration may be made for TUBB3 mutation screening.

Vertical Strabismus - Indication of Surgical Techniques on the Inferior Rectus Muscle.

Aid: Familiarize themselves with the operating techniques in their own modulation to solve hypotropic states with elevation or hypotropic states with depression. METHODS: Surgery technique “cul-de-sac” is a procedure with fixed adjustable sutures (non-absorbable suture), which are guided in parallel on both peaks original insertion of inferior rectus muscle. The node itself of suture is done in cutting the muscle and through the original insertion only leads arc suture. During „counterclockwise transposition“ procedure sec. Knapp, the horizontal rectus muscles are fixed at the straight inferior rectus muscle level. The author included their graphical diagrams. MATERIAL: At the Department of Ophthalmology in the Faculty Hospital Královské Vinohrady in Prague (Czech Republic, EU) in the years 1996 - 2014 a release relaxing operation of the inferior rectus musle by the technique “cul-de-sac” on 49 eyes. Indication was double elevator palsy in 31 patients, congenital fibrosis of the extraocular muscles in 15 patients and in 3 cases, it was thyroid eye disease. “Counterclockwise transposition” procedure sec. Knapp was indicated twice for paresis of the inferior rectus muscle in 2015 and 2016. For the first time, it was a congenital form. It was detected histologically atrophy of stripped muscle with hypertrophy collagen. The second traumatic form was formed after an orbital injury. RESULTS: We provided adequate relaxation of the inferior rectus muscle and practical restoration of eye elevation by the technique “cul-de-sac“ in 18 preschool children with the double elevator palsy and three adult patients with thyreiod eye disease. We had to restore motility to complete the procedure by classical transposition procedure sec. Knapp of both horizontal rectus muscles to the direct superior rectus muscle in elderly children and adults with the double elevator palsy, as well as in all patients with the congenital fibrosis of the extraocular muscles. Indicate the inclusion of this transposition influenced the degree of fibrotic rectus inferior muscle given by age. “Counterclockwise transposition“ procedure sec. Knapp ensured the practical disappearance hypertropie in both cases of paresis of the inferior rectus muscle. The alignment of the position of the eyes without diplopia in the direct view was ensured by prismatic correction. CONCLUSIONS:   To release the fibrotically altered inferior rectus muscle in the double elevator palsy, the congenital fibrosis of the extraocular muscles and the thyroid eye disease, the operation of adjustable sutures was necessary in accordance with our experience with the technigue “cul-de-sac“ in its own modification. The transposition procedure sec. Knapp, either classical or “”counterclockwise” “ in its own modification, was of paramount importance for the solution of the vertical deviation of a paretic ethiology. Key words: adjustable sutures, congenital fibrosis of the extraocular muscles, double elevator palsy, thyroid eye disease, transposition procedure sec. Knapp, vertical strabismus.

Clinical features and surgical treatment of double elevator palsy in young children.

AIM: To describe the clinical features of congenital double elevator palsy (CDEP) and to evaluate various surgical outcomes between the standard Knapp and augmented Knapp procedures, based on improvements in primary eye position and ocular motility. METHODS: Twenty-two patients with CDEP at Shanghai Children's Hospital were enrolled from July 2014 to January 2018. The forced duction test (FDT) was negative in 21 patients, aged 8mo to 12y (mean 5.4y). Patients were divided into two treatment groups: 16 patients underwent the standard Knapp procedure (group A), with or without horizontal squint procedure; and 5 patients underwent the augmented Knapp procedure (Foster procedure; group B). One patient underwent inferior rectus recession in the affected eye and superior rectus recession in the sound eye because of a positive FDT. The pre- and postoperative vertical deviations in the primary position and ocular motility were compared in the two groups. RESULTS: Twenty-one eyes of the 22 patients (95%) were aligned within 10 prism diopters (PD), and all patients (100%) reached ≥25% elevation improvement after surgery. The average corrected vertical deviation in group B was statistically better than that of group A. For group A, the vertical deviation in the primary position decreased from 24.75Δ±8.35Δ to 4.56Δ±8.07Δ after surgery, for an improvement of 23.06Δ±6.51Δ (P<0.05). In group B, the decrease was from 35.00Δ±5.00Δ (range 30Δ-40Δ) to 1.00Δ±2.24Δ, for an improvement of 34.00Δ±4.18Δ (P<0.05). There were significant differences between the pre- and postoperative elevation in each group (group A, P<0.05; group B, P<0.05). The average scale of improved elevation in group B (1.80±0.45) was not significantly better than that of group A (1.69±0.87; Z=-0.732, P=0.548). The average follow-up periods lasted 21mo in group A and 18mo in group B. CONCLUSION: For vertical deviations <30Δ, the standard Knapp procedure can be chosen. For deviations greater than 30Δ-40Δ, the Foster procedure should be chosen. Because of our early interference, the inferior rectus (IR) muscle did not show mechanical restriction. Monocular elevation deficiency (MED) should be diagnosed early so that complications will be reduced and the procedure will be easier for the surgeon.

Ocular-muscle surgery for filamentary keratitis that developed in double elevator palsy.

PURPOSE: To report a case of filamentary keratitis occurring in the cornea hidden under the eyelids by squint surgery. METHODS: A 69-year-old female patient with a history of amblyopia was referred for intractable filamentary keratitis in the left eye. The strabismus angle was 35Δ hypertrophic, and ocular motility was within the normal range. Slit-lamp examination of her left eye revealed filamentary keratitis in more than one-third of the upper cornea behind the upper eyelid. Her right eye was diagnosed as supranuclear double elevator palsy. We performed strabismus surgery on her right eye, including inferior rectus muscle recession (5 mm) in combination with superior rectus muscle resection (5 mm) under local anesthesia. Following surgery, the left eye squint angle was improved. The filamentary keratitis of the left eye disappeared, and there was no recurrence over the following 5 years. CONCLUSION: The squint surgery of paralyzed right eye decreased the strabismus angle, subsequently resulting in the disappearance of the filamentary keratitis in the left eye via the resolution of the relative blepharoptosis. Although the squint operation performed was not for the purpose of improving binocular function, we want to conclude that it can treat the filamentary keratitis behind the eyelid.

Congenital Double Elevator Palsy with Sensory Exotropia: A Unique Surgical Management.

PURPOSE: To report a unique surgical approach for congenital double elevator palsy with sensory exotropia. CASE REPORT: A 7-year-old boy with congenital double elevator palsy and sensory exotropia was managed surgically by Callahan's procedure with recession and resection of the horizontal recti for exotropia without inferior rectus recession, followed by frontalis sling surgery for congenital ptosis. CONCLUSIONS: Favourable surgical outcome was achieved without any complication.

Surgery for Supranuclear Monocular Elevation Deficiency.

PURPOSE: We report a novel approach to surgery for monocular elevation deficiency (MED). METHODS: A retrospective review of 5 patients undergoing surgery for supranuclear MED between 2003 and 2014. All patients had intact Bell's phenomenon, hypotropia, limited elevation above the primary position, and negative forced duction testing of the paretic eye. Preoperatively all patients preferred chin-up head posture and three had pseudoptosis or ptosis. One of the 5 had prior vertical muscle surgery. Surgery correction for the MED consisted of near maximal superior rectus recession on the contralateral sound eye. RESULTS: Compensatory chin-up head position and alignment in primary position was improved in all patients. Average age at surgery was 5.3 years. Average superior rectus recession was 9.7 mm. Mean follow-up was 4.8 years (range 12 months to 11.5 years). The vertical deviation of the paretic eye in primary position postoperatively was orthotropic for 2, hypotropic for 2, and overcorrected for 1. CONCLUSIONS: In cases of supranuclear MED (double elevator palsy) contralateral superior rectus recession based on the innervational principle is a simple and reliable alternative surgical approach compared to published results of the Knapp transposition procedure. Additionally, it holds the possibility for decreased complications and less complicated future surgical treatment options.

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.