Superior Oblique Palsy Research Articles

Inferior oblique muscle anteriorization in congenital superior oblique palsy.

Inferior oblique muscle overaction of variable amounts is usually present with congenital superior oblique palsy. Inferior oblique muscle anteriorization has been described as a suitable surgical procedure in this entity. The aim of this study was to investigate the effect of inferior oblique muscle anteriorization in patients with congenital superior oblique palsy on vertical, torsional and horizontal alignment. The study was designed as an institutional retrospective cohort study. 45 patients with congenital superior oblique palsy (15 female, 30 male; mean age 36 years ± 19.2 SD, ranging from 6 to 75 years) underwent inferior oblique muscle anteriorization between 2000 and 2010. Preoperative amounts of vertical, torsional and horizontal deviation (using Harms tangent screen), measurements of Bielschowsky head tilt phenomenon as well as stereopsis (Lang test) were compared with findings three months and one year postoperatively. Preoperative vertical deviation in primary position measured 10.1° (mean; range 0-19). Three months postoperatively vertical deviation was significantly reduced (p<0.001) to 4° (mean; range 0-20). After one year vertical deviation measured 3.5° (mean; range 0-15). The values three months postoperatively did not significantly differ from those one year postoperatively (p=0.46). Inferior oblique muscle anteriorization leads to a significant and sustained improvement of ocular alignment in patients with congenital superior oblique palsy of various degrees of severity. Thus the procedure is recommendable as a first line treatment in this clinical situation.

Operative management of patients with radiosurgery-related trigeminal neuralgia: Analysis of the surgical morbidity and pain outcome

ObjectTrigeminal neuralgia (TGN) occurring after radiosurgical treatment of cerebellopontine or petroclival tumors may be very difficult to control. Our aim was to determine the efficacy of neurosurgical treatment in regards to pain control and to evaluate the procedure-related complication and morbidity rates. MethodsRetrospective study of a series of operated patients with radiosurgery-induced TGN. The primary goal of the surgery was to inspect and decompress the trigeminal nerve; the second goal was to remove the tumor remnant completely, if safely feasible. The main outcome measures were pain control, time to onset of pain relief and its duration, occurrence of new neurological deficits or worsening of the existing one and completeness of tumor removal. ResultsThe four patients met the inclusion criteria: 2 with vestibular schwannomas, 1 with petroclival meningioma and 1 with an epidermoid. TGN occurred 12–60 months after radiosurgery (mean 39 months). At presentation the pain attacks occurred multiple timesdaily and lasted from a few seconds to 2–3min. The Complete tumor removal via the retrosigmoid approach was achieved in all cases. There were no major operative complications or persistent morbidity, besides one patient with trochlear nerve palsy. All patients experienced immediate pain relief after surgery. At follow-up (median duration – 42.5 months) the three patients reported complete pain resolution. One patient had occasional slight pain but did not need any medications. ConclusionSurgery is safe and effective treatment option of patients with intractable radiosurgery-induced TGN. It leads to excellent pain control and is curative in regards to the neoplastic disease.

Bilateral Superior Oblique Palsy: Etiology and Therapeutic Options

To study the causes of bilateral superior oblique palsy (BSOP), treatment with botulinum toxin and/or surgery, and outcome of treatment. This was an 11-year retrospective study of patients with BSOP treated with injections of botulinum toxin (Botox), surgery, or both. Treatment was considered successful when anomalous head turn and diplopia in primary gaze position and downgaze resolved. Bilateral superior oblique palsy was diagnosed in 12 patients (8 male; mean age, 29.5 years). Palsy was secondary to a neoplasm in 3 cases and to head trauma in 2. In 2 cases, it was ischemic, in 2 it was congenital; the remaining cases were iatrogenic (hydrocephalus secondary to meningitis, 1), hemorrhagic (1), and idiopathic (1). The clinical manifestations recorded were diplopia (10), anomalous head posture (9), V pattern (12), subjective excyclotorsion (8), and objective excyclotorsion (6). Recovery was spontaneous in 1 case with neoplastic disease. Botox was injected in 8 cases (inferior oblique and/or inferior rectus muscles [successful in 2]), and subsequent surgery was required in 6. Two patients underwent surgery without prior injection of Botox. The most common surgical technique was recession of the inferior oblique muscle (6 patients), either as the only operation or associated with other procedures. The final result was good in 72.72% (8/11). Mean follow-up was 62.6 months (range 9-99 months). Causes of BSOP were varied (most frequently neoplastic). Botox was effective as the only treatment in 25% (2/8). Outcome was good in a high percentage of cases with Botox, surgery, or both.

Herpes zoster ophthalmicus with isolated trochlear nerve palsy in an otherwise healthy 13-year-old girl

Herpes zoster ophthalmicus is rare in healthy children. It is occasionally associated with extraocular muscle palsies and rarely with isolated trochlear nerve palsy. We report a case of unilateral isolated trochlear nerve palsy associated with herpes zoster ophthalmicus in an immunocompetent 13-year-old girl who presented with diplopia and blurred vision in her right eye. The right cornea had multiple subepithelial opacities. Ocular motility returned to normal and diplopia and corneal opacification resolved with steroid therapy. To our knowledge, this is the first such case involving the troclear nerve in a child.

Cyclotropia to Differentiate Longstanding and Acute Fourth Cranial Nerve Palsy

IntroductionPalsy of the fourth cranial nerve is the most common isolated cyclovertical extraocular muscle palsy, and the most common isolated cranial nerve palsy. Fourth cranial nerve palsy may be acquired or “congenital.” Without a clear history of acute onset vertical diplopia in a patient without risk factors, it can be difficult to distinguish between congenital and acquired superior oblique palsy. The weight of evidence following a thorough sensorimotor exam is used to support the presumed etiology. The purpose of this study was to determine if the ratio of excyclotropia to hypertropia in primary position could be used to distinguish acquired from congenital unilateral superior oblique palsy.MethodsA computer database search was done to identify patients with unilateral fourth cranial nerve palsy. Patients were sorted into two groups: those with documented acute fourth nerve palsy (Group A), and those with presumed congenital onset (Group B).ResultsAlthough the degree of cyclotropia was not statistically different between Groups A and B, the amount of hypertropia was significantly larger in the congenital group. Group B patients had an average of 0.3° of excyclotropia per 1Δ of hypertropia in primary, compared to 1.16° per 1Δ in Group A (P < 0.001).ConclusionThe degree of cyclotropia is correlated to the severity of the SO weakness in acute fourth nerve palsy, but not in presumed congenital fourth nerve palsy. The ratio of degrees of cyclotropia per prism diopter of hypertropia may be helpful in differentiating longstanding from acute fourth nerve palsy.

Superior Oblique Palsy: Epidemiology and Clinical Spectrum From a Tertiary Eye Care Center in South India.

The objective of this study was to describe the epidemiologic characteristics and clinical spectrum of superior oblique palsy (SOP) at a tertiary eye care center. This was a retrospective case review. Retrospective analysis of case records of 181 consecutive patients diagnosed with SOP seen from January 2001 to December 2008 was performed. The mean age of the patients was 31.78 ± 19.28 years (range, 1-85 years). One hundred thirty-six (75.1%) were males, and 45 (24.9%) were females. Most common presenting symptom was manifest vertical deviation (81.7%). Most common etiology was trauma (33.7%) followed by a congenital (32.6%), idiopathic (23.7%), and ischemic (8.8%) etiology. One patient each had multiple myeloma and central nervous system tuberculoma. Bilateral SOP was noted in 11 cases; 7 (63.4%) of them were posttraumatic, 3 (27.3%) were congenital, and 1 developed after cerebrovascular accident. Neuroimaging performed in 67 patients (37%) with suspected central nervous system pathology was positive in 10 (14.9%) of patients. The distribution of neuroimaging abnormalities was traumatic sequelae in 5 patients (50%), intracranial space-occupying lesion in 1 (10%), granuloma in dorsal midbrain in 1 (10%), and ischemic lesions (lacunar infarcts, occipital lobe infarcts) in 3 (30%) patients. Superior oblique palsy etiology tends to show an age and sex distribution. Trauma and congenital etiologies are the most common in young males and females, respectively, whereas ischemic etiology is more frequent in middle-aged and elderly people. Detailed history, meticulous ocular and systemic examination, appropriate neuroimaging, and systemic investigations help elucidate the etiology.

Risk Factors and Prognosis of Isolated Ischemic 3rd, 4th, 6th Cranial Nerve Palsy

Purpose: To investigate the clinical features and risk factors of ischemic third, fourth, sixth cranial nerve palsy. Methods: Retrospectively, we reviewed the medical records of 46 eyes of 46 patients who were diagnosed with ischemic third, fourth, sixth nerve palsy alone such as age of onset, risk factors, recovery rate and recovery time. Results: The mean age of onset was 64.9 years. Of the 46 patients, 15 patients (32.6%) in third cranial nerve palsy group, 15 patients (32.6%) in fourth cranial nerve palsy group, 16 patients (34.8%) in sixth cranial nerve palsy group. The risk factor of hypertension in 30 patients (65.2%) was the most common than other risk factors such as diabetes, hyperlipidemia, elevated blood hematocrit, ischemic heart disease, left ventricular hypertrophy, smoking. The mean number of risk factors was 2.3 ± 0.5 in third cranial nerve palsy group, 1.6 ± 1.1 in sixth cranial nerve palsy group, 1.4 ± 1.1 in fourth cranial nerve palsy group. Of the 46 patients, 42 patients (91.3%) were recovered. There was no significant difference in recovery rate by cranial nerve palsy. Recovery time of intracranial abnormalities group (10.5 ± 2.9 weeks) in brain imaging study was late as compared with that of no intracranial abnormalities group (7.5 ± 5.1 weeks). Conclusions: The overall recovery rate of isolated ischemic third, fourth, sixth cranial nerve was high. But if there are intracranial abnormalities in imaging study, it took a long time to recover. Also ischemic third cranial nerve palsy had multiple risk factors characteristically. J Korean Ophthalmol Soc 2014;55(5):740-745

Correction of Hypertropia Coexisting with Intermittent Exotropia

Purpose: To investigate the clinical features associated with hypertropia and report the surgical outcomes of hypertropia coexisting with exotropia. Methods: We reviewed the medical records of 148 patients with intermittent exotropia coexisting with hypertropia over 4 PD who received exotropia surgery. The cases accompanied by apparent paralytic strabismus such as superior oblique palsy were excluded. Patients were divided into groupI(clinically diagnosed hypertropia) and group II (non-specific hypertropia) and the clinical features of coexisting hypertropia and surgical outcomes were analyzed. Results: Among the 148 patients, groupIconsisted of 38 patients (26%) and group II of 110 patients (74%). The average amount of preoperative hypertropia angle in primary gaze was 9.58 ± 3.89 PD and 6.62 ± 2.69 PD in group I and II, respectively. Group I included 12 patients with dissociated vertical deviation (DVD), 10 patients with unilateral inferior oblique overaction, 13 patients with asymmetric bilateral inferior oblique overaction and 3 patients with superior oblique overaction. Group II included 19 patients with comitant hypertropia (17%), head tilt positive pattern (simulated superior oblique palsy) was found in 84 patients (76.3%) and variable incomitance was observed. In groupI, 29 patients received simultaneous horizontal muscle with hypertropia surgery. Postoperative hypertropia angle in groupI was 1.41 ± 2.93 PD and 4 cases were considered surgical failure. In groupII, hypertropia was resolved with horizontal muscle surgery only and the amount of postoperative hypertropia was 0.45 ± 1.60 PD. Conclusions: In this study, vertical deviations in intermittent exotropia with concomitant hypertropia related to obvious oblique muscle dysfunction or DVD were corrected effectively by oblique or vertical rectus muscle surgery. Nonspecific hypertropia can be resolved after horizontal muscle surgery alone, however, for precise differential diagnosis, careful examination for variable clinical features is necessary before determining surgery. J Korean Ophthalmol Soc 2014;55(12):1883-1889

The Middle Fossa Approach for the Removal of a Trochlear Schwannoma

Objectives. Schwannomas originating from the trochlear nerve are extremely rare; only 30 cases have been reported in the literature. Many operative approaches have been utilized for lesion resection, but the advantages of the anterior transpetrosal approach are numerous and include excellent exposure, minimal extradural retraction of the temporal lobe, and minimal cerebrospinal fluid leaks. We report the second case of a trochlear schwannoma resected via the anterior transpetrosal approach. Setting. A 64-year-old male presented with 3-month history of diplopia and headaches. On physical examination, he was found to have a right fourth nerve palsy. Brain magnetic resonance imaging revealed a mass within the right ambient cistern compressing the adjacent midbrain. A right-sided anterior transpetrosal approach was used—which confirmed that the trochlear nerve entered the mass—to achieve gross total resection. Pathological examination confirmed diagnosis of schwannoma. The patient was discharged on postoperative day 3. He experienced a persistent fourth nerve palsy postoperatively with an otherwise normal neurological examination. Follow-up imaging confirmed complete removal of the tumor. Conclusion. The anterior transpetrosal approach is an excellent approach for removal of trochlear schwannomas involving the cisternal course of the trochlear nerve. It affords complete visualization of this anatomical region while introducing minimal morbidity.

Effect of strabismus surgery on torticollis caused by congenital superior oblique palsy in young children

Purpose:To evaluate the outcome of strabismus surgery for congenital superior oblique palsy (SOP) in relation to correction of head tilt and hypertropia. The cohort of patients mainly involved very young children. This is the first study to use a standardized instrument to objectively measure torticollis before and after surgery.Materials and Methods:A non-comparative interventional case series of 13 cases of congenital superior oblique palsy with head tilt, who underwent simultaneous superior oblique tuck and inferior oblique recession between Jan 2000 and Dec 2008, were studied.Results:The mean duration of SOP until surgery was 36.8 months. Of the 12 unilateral cases, 8 were right-sided. Mean follow-up period was 17 months (range 7-36). The outcome was determined at the last follow-up. Mean pre-and post-operative hypertropia (p.d.) in forced primary position was 19 ± 7 and 2 ± 6, respectively (P < 0.0001). The head tilt reduced from mean of 17 ± 9 to 2 ± 2 degrees (P < 0.0001). Success, defined as hypertropia <5 PD and head tilt less than 5 degrees, was achieved in 69% (9/13. C.I. 42-88%) and 85% (11/13. C.I. 56-96%), respectively. The success rate for achieving both criteria was 61.5% (C.I. 35-88%). Five patients required additional surgery; usually a contralateral inferior rectus muscle recession, which was successful in all cases. One case developed asymptomatic Brown syndrome (7.69% - C.I. 6.7-22.2).Conclusions:Simultaneous superior oblique tuck and inferior oblique muscle recession can successfully treat selected cases of congenital superior oblique palsy. About one-third required an additional procedure, which led to total normalization of the head position.

A Case of Bilateral Trochlear Nerve Palsy Following Cisternography

A Case of Bilateral Trochlear Nerve Palsy Following Cisternography

Duane′s retraction syndrome with severe upshoot and ipsilateral superior rectus contracture: A rare presentation

Type III Duane's retraction syndrome (DRS) is a rare condition and represents only 1% of all cases of Duane's syndrome. It consists of limited/absent abduction as well as adduction of the affected eye. A characteristic upshoot/downshoot/both, in adduction, and globe retraction may occur in DRS patients. Secondary muscle changes like medial rectus contracture or superior rectus contracture (SRC) may rarely be associated with DRS. SRC has been reported in patients with unilateral superior oblique palsy and dissociated vertical deviation. However, no case report of association of SRC with DRS has been reported. We report a rare case of type III DRS with severe upshoot, globe retraction and ipsilateral SRC. It is hypothesized that a long standing severe upshoot could be the reason for development of secondary SRC. The condition was treated with Y-split of lateral rectus and superior rectus recession.

Does the Clinical Phenotype of Fatal Familial Insomnia Depend on PRNP codon 129 Methionine-Valine Polymorphism?

Fatal familial insomnia (FFI) is a rare, hereditary prion-protein disease. Methionine-valine polymorphism at codon 129 of the prion-protein gene (PRNP) determines the phenotype in other hereditary prion-protein diseases, but association with the clinical phenotype in FFI remains uncertain. Early clinical findings in FFI comprise disturbances of the sleep-wake cycle and mild neuropsychiatric changes which typically emerge during middle to late adulthood. Here we describe an unusually early onset and rapid progression of FFI associated with dorsal midbrain involvement in a female patient with PRNP mutation at codon 178 and homozygote methionine polymorphism at codon 129. Early dorsal midbrain involvement became apparent by total loss of REM sleep and isolated bilateral trochlear nerve palsy. Early onset and rapid progression disease type associated with dorsal midbrain involvement may indicate a different spatiotemporal distribution of the neurodegenerative process in FFI patients with PRNP mutation and codon 129 methionine homozygosity compared to methioninevaline heterozygosity.

Erratum to: Effects of vertical muscle surgery on differences in the orientation of Listing’s plane in patients with superior oblique palsy

Erratum to: Effects of vertical muscle surgery on differences in the orientation of Listing’s plane in patients with superior oblique palsy

Clinical utility and assessment of cyclodeviation

This article reviews and updates basic concepts, diagnosis and treatment of cyclotorsion. Cyclodeviation in congenital superior oblique palsy (SOP) seems to correlate with the extent of superior oblique muscle hypoplasia. Genetic polymorphisms such as PHOX2B polymorphism, considered to be risk factors for congenital fibrosis of the extraocular muscles, may play a role in SOP and consequently in cyclotorsion. Two components of the ocular tilt reaction, ocular torsion and tilt of subjective visual vertical, seem to share similar sites of impairment in the brainstem. Harada-Ito surgery continues to be the procedure of choice in patients with isolated cyclodeviation, evidencing better outcome if less than 10° of preoperative excyclotorsion and preoperative fusion exist. Ocular torsion is not infrequent in patients with intermittent exotropia, especially in the most exo-deviated eye, emphasizing a possible role in pathogenesis. A new device for the assessment of dynamic torsion during ocular counter roll response using after-image has been described. Similarly, a new method to measure objective ocular torsion using retinal arcade tilt as a reference has been proposed. Finally, torsional data transformation such as the sum of angles of excyclodeviation, rather than using the angle of excyclodeviation of the paretic eye, is becoming increasingly popular among studies on torsion. Exciting developments on ocular torsion have been described recently, and new ways to access and interpret ocular torsion have been devised as well.

Prediction of trochlear nerve absence by superior oblique muscle volumetry in congenital superior oblique palsy

Prediction of trochlear nerve absence by superior oblique muscle volumetry in congenital superior oblique palsy

Unusual Presentation of Multiple Nerve Palsies with Granulomatous Encephalitis Due to Acanthamoeba Species

Acanthamoeba spp is one of the common protozoa in soil, usually found in fresh water and other habitats. We are presenting a case of Granulomatous amoebic encephalitis presented with unusual symptoms of third, fourth, sixth, and seventh nerve palsy with diplopia, ptosis, and migraine. Differential diagnosis of tuberculous (TB) meningitis was initially considered but was ruled out by CSF TB PCR. There was no history of exposure to sources such as fresh water ponds and contaminated swimming pools. CSF wet mount and culture confirmed the diagnosis of Acanthamoeba spp. Specific antiparasitic therapy was started and patient improved with successful resolution of symptoms.

Pearls and Oy-sters: Central fourth nerve palsies

Editor's Note: Miller and Gold et al. provide additional pearls that may help clinicians suspect a central origin of a fourth nerve palsy. In response to Yufe, Saposnik et al. clarify the methodology of their study on Stroke Prognostication using Age and NIH Stroke Scale (NIHSS). The latter 2 combined …

Persistent diplopia and superior oblique muscle dysfunction following dissection of the orbital periosteum in cranial base surgery

Background/aimsPersistent diplopia secondary to a fourth cranial nerve palsy is poorly documented after open cranial base surgery.MethodsSix cases of fourth cranial nerve palsy after cranial base surgery were drawn from...

Combined resection and anterior transposition of the inferior oblique muscle for treatment of large primary position hypertropia caused by unilateral superior oblique muscle palsy

To evaluate the efficacy of combined resection and anterior transposition of the inferior oblique muscle for treatment of unilateral superior oblique muscle palsy with hypertropia from 20(Δ) to 25(Δ) in primary position. The medical records of consecutive patients operated on for unilateral superior oblique muscle palsy and hypertropia from 20(Δ) to 25(Δ) in primary position were retrospectively reviewed. All patients had overaction of the inferior oblique muscle. The inferior oblique muscle was disinserted and 4mm of its distal end was resected and transposed to the lateral border of the inferior rectus muscle insertion. The prism and alternate cover test was used to measure hypertropia. Surgical results were evaluated at 6months' follow-up. A total of 27 patients were included. The mean hypertropia in primary position was 22.6±0.4(Δ) preoperatively, which decreased to 1.4±0.6(Δ) after surgery. None of the patients developed hypotropia in primary position. Mild limitation of elevation was recorded in 1 patient, and 4 patients developed lower eyelid fullness. In this patient cohort, combined resection and anterior transposition of the inferior oblique muscle effectively treated unilateral superior oblique muscle palsy with hypertropia from 20(Δ) to 25(Δ) in primary position.