Effect of inferior oblique myectomy on ocular torsion according to the absence of the trochlear nerve in unilateral congenital superior oblique palsy.

OBJECTIVES: To evaluate the effect of inferior oblique (IO) myectomy on ocular torsion according to the absence of the trochlear nerve in unilateral congenital superior oblique palsy (UCSOP). METHODS: We retrospectively reviewed the clinical data of patients who had been diagnosed with UCSOP and underwent ipsilateral IO myectomy (n = 43). Patients were classified into the present and absent groups according to the absence of the trochlear nerve and superior oblique hypoplasia on magnetic resonance imaging (MRI). For quantitative analysis of ocular torsion, disc-fovea angles (DFA) were collected in both eyes using fundus photographs taken within three months before surgery and one month after surgery. RESULTS: DFA of the paretic eye did not differ according to the absence of the trochlear nerve (9.4±5.6° in the present group vs. 11.0±5.4° in the absent group, p = 0.508). However, the present group had a larger DFA in the non-paretic eye than the absent group (14.1±6.7° in the present group vs. 8.0±5.0° in the absent group, p = 0.003). The change of ocular torsion after IO myectomy in the paretic eye was -5.3±3.7° in the present group and -4.8±3.5° in the absent group, respectively (p = 0.801). In the non-paretic eye, the change in DFA was -1.5±3.0° in the present group, which was larger than that in the absent group (0.7±2.6°, p = 0.047). In the multivariate analysis, the change in DFA was correlated with only the preoperative DFA (standardized ß = -0.617, p<0.001 in the paretic eye, and standardized ß = -0.517, p<0.001 in the non-paretic eye). CONCLUSIONS: In the paretic eye, there was no significant difference in the change of ocular torsion between both groups, whereas in the non-paretic eye, the present group had a larger change in DFA after IO myectomy than the absent group. However, in the multivariable analysis, the change in ocular torsion was significantly correlated with preoperative excyclotorsion but not with the presence of the trochlear nerve itself.

Unilateral Isolated Trochlear Nerve Palsy due to Ipsilateral Midbrain Infarction.

Ischemic stroke is a very rare etiology in cases of isolated trochlear nerve palsy, and no reports of ipsilateral trochlear nerve palsy caused by unilateral stroke have so far been published. However, we now report a case of isolated trochlear nerve palsy due to ipsilateral dorsal small midbrain infarction in a 70-year-old woman who presented with acute onset of diplopia. There were no other clinical manifestations, but brain magnetic resonance imaging revealed a small ischemic lesion in the right dorsal midbrain, showing that isolated trochlear nerve palsy can be caused by stroke.

Segmental Imaging of the Trochlear Nerve: Anatomic and Pathologic Considerations.

BACKGROUND: The trochlear nerve (the fourth cranial nerve) is the only cranial nerve that arises from the dorsal aspect of the midbrain. The nerve has a lengthy course making it highly susceptible to injury. It is also the smallest cranial nerve and is often difficult to identify on neuroimaging. EVIDENCE ACQUISITION: High-resolution 3-dimensional skull base MRI allows for submillimeter isotropic acquisition and is optimal for cranial nerve evaluation. In this text, the detailed anatomy of the fourth cranial nerve applicable to imaging will be reviewed. RESULTS: Detailed anatomic knowledge of each segment of the trochlear nerve is necessary in patients with trochlear nerve palsy. A systematic approach to identification and assessment of each trochlear nerve segment is essential. Pathologic cases are provided for each segment. CONCLUSIONS: A segmental approach to high-resolution 3-dimensional MRI for the study of the trochlear nerve is suggested.

[Diplopia: An important symptom in internal medicine!] / Diplopie : un symptôme important en médecine interne !

Diplopia is defined as "double vision" when looking at a single object. Monocular diplopia is related to an ocular disorder and must be differentiated from binocular diplopia which is secondary to ocular misalignment. The examination of the patient with binocular diplopia is often challenging for non-specialists. However, a careful and systematic clinical examination followed by targeted ancillary testing allows the clinician to localize the lesion along the oculomotor pathways. The lesion may involve the brainstem, the ocular motor nerves III, IV or VI, the neuromuscular junction, the extraocular ocular muscles, or the orbit. Causes of binocular diplopia are numerous and often include disorders typically managed by internal medicine such as inflammatory, infectious, neoplastic, endocrine, and metabolic disorders. In addition to treating the underlying disease, it is important not to leave diplopia uncorrected. Temporary occlusion of one eye by applying tape on one lens or patching one eye relieves the diplopia until more specific treatments are offered should the diplopia not fully resolve.

Synchronous ipsilateral cavernous malformations of the trochlear nerve.

BACKGROUND: Cranial nerve cavernous malformations (CM) are rare benign congenital vascular anomalies, with approximately 44 preceding cases in the literature. We report the fifth case of trochlear CM, as well as the first instance of two discrete CM occurring simultaneously along the same cranial nerve. METHODS: Case report. RESULTS: A fifty-seven year-old man presented with several years of diplopia; physical examination identified a complete left trochlear nerve paralysis. MRI revealed a 1cm enhancing lesion within the left ambient cistern, and the patient underwent left pretemporal transcavernous resection. Intraoperatively, a second, discrete CM of the trochlear nerve was also discovered; wide excision of the intrinsic trochlear lesions was completed, allowing both tumors to be removed en bloc with negative margins. Pathologic analysis confirmed both to be CM of the trochlear nerve. The patient recovered with a persistent left trochlear paralysis only, and follow-up MRI was negative for residual or recurrent disease. CONCLUSION: Cranial nerve CM are rare but potentially morbid mass lesions, with the capacity to precipitate significant neuropathies. Differential diagnosis includes schwannoma and hemangioblastoma. Definitive diagnosis may not be possible preoperatively; however, resection is recommended in symptomatic patients, potentially accompanied by nerve repair.

Central Trochlear Palsy: Report of Two Patients With Ipsilesional Palsy and Review of the Literature.

BACKGROUND: The trochlear (fourth) nerve is the only cranial nerve that decussates before emerging from the posterior aspect of the brainstem. Lesions involving the trochlear nucleus or fascicles mostly give rise to contralesional superior oblique palsy (SOP). METHODS: We report 2 patients with SOP on the side of intraaxial lesions with a literature review on central trochlear palsy. RESULTS: The lesions are more commonly located posterior to the cerebral aqueduct in patients with ipsilesional SOP than in those with contralesional SOP. CONCLUSIONS: Intraaxial lesions may cause ipsilesional or contralesional SOP depending on the lesion location along the course of trochlear fascicle in the brainstem.

Surgical Outcomes of Inferior Oblique Myectomy in Unilateral Congenital Superior Oblique Palsy with or without Trochlear Nerve.

OBJECTIVES: To compare the surgical outcomes of inferior oblique (IO) myectomy in congenital superior oblique palsy (SOP) according to the presence of the trochlear nerve identified with high-resolution MRI. DATA EXTRACTION: Forty-one congenital SOP patients without a trochlear nerve (absent group) and 23 patients with a trochlear nerve (present group) who underwent IO myectomy as the primary surgical treatment were retrospectively reviewed. "Motor success" was defined as postoperative ipsilateral hypertropia ≤ 4 prism diopter (PD). "Head tilt improvement" was regarded as postoperative angle of head tilt < 5 degrees (°). Success rates for motor alignment and head tilt improvement, cumulative probabilities of success, and factors influencing surgical responses were evaluated. RESULTS: The cumulative probabilities of motor success at 2 years after IO myectomy were 92% in patients with a trochlear nerve and 86% in patients without a trochlear nerve (P = 0.138). The cumulative probabilities of undercorrection and recurrence of hypertropia after 2 years were 0% in the present group versus 21% in the absent group (P = 0.014). The cumulative probabilities of persistent head tilt after 2 years were 14% in the present group and 20% in the absent group (P = 0.486). A younger age at operation was associated with reduced probabilities of motor success and head tilt improvement (P = 0.009, P = 0.022 respectively). A greater preoperative angle of head tilt was associated with persistent head tilt after surgery (P = 0.038). CONCLUSIONS: Congenital SOP without a trochlear nerve had a higher risk of hypertropia undercorrection after IO myectomy compared to patients with a trochlear nerve. A younger age at operation and larger preoperative head tilt was related to poor outcomes.

Imaging demonstration of trochlear nerve agenesis in superior oblique palsy emerging during the later life.

BACKGROUND: Congenital trochlear palsy may manifest with sudden vertical diplopia due to decompensation during the later life, which may bring a diagnostic challenge. CASE PRESENTATION: Two men with vertical diplopia for several years after age of 50 were referred with persisting or suddenly aggravating diplopia. Findings were consistent with unilateral superior oblique palsy (SOP) in both patients with a contraversive head tilt. Facial asymmetry was suggestive of a congenital cause in a patient. High resolution magnetic resonance image (MRI)s disclosed atrophic superior oblique and absent trochlear nerve in the side of SOP in both patients. CONCLUSION: Imaging demonstration of superior oblique atrophy and absent trochlear nerve may aid in diagnosis of congenital SOP presenting sudden vertical diplopia during the later life due to delayed decompensation.

[Brown syndrome: clinical and radiological correlation].

OBJECTIVE: Brown syndrome is characterized by limitation of elevation in adduction, with complex mechanisms involving muscle, tendon, and trochlea. Here, we investigated mechanisms of Brown syndrome by magnetic resonance (MR) imaging. METHODS: It was a retrospective case series study. Fourteen patients with unilateral Brown syndrome between 3 and 54 years of age (10 cases of congenital and 4 cases with acquired disease) were included in the study. All patients underwent complete ophthalmic and orthoptic evaluation. Imaging of the ocular motor nerves at the brainstem was performed on 3D-FIESTA sequence, the orbits were imaged with FSE T1, T2WI using surface coils. RESULTS: Nine of 10 with congenital Brown syndrome demonstrated hypoplasia of the superior oblique (SO) of the affected side. Abnormal low signal intensity in the trochlea area was found in one patient. Three of 4 acquired patients had a history of trauma and were demonstrated fracture of the trochlea, extensive scarring, and superior orbital fracture. One acquired case was demonstrated scarring of anterior part of the SO and hypoplasia of the posterior part. CONCLUSION: Brown syndrome consists of a series of diseases. Their clinical features are quite similar while their anatomical mechanism varies in numerous ways. Therefore, based on patient's individual pathophysiology, the management in Brown syndrome should be personalized.

Infratrochlear neuralgia.

INTRODUCTION: The infratrochlear nerve supplies the medial aspect of the upper eyelid, the superolateral aspect of the nose and the lacrimal caruncle. This nerve may contribute to the pain stemming from the trochlea, but infratrochlear neuralgia has not been identified as a specific cause of pain. METHODS: Over a 10-year period we have been recruiting patients with pain in the internal angle of the orbit that did not show features of trochlear pain. RESULTS: Seven patients (six female, one male; mean age, 46.1 ± 18.9) presented with pain in the territory of the infratrochlear nerve. The pain appeared in the internal angle of the orbit and upper eyelid (n = 3), the superolateral aspect of the nose (n = 3), or the lacrimal caruncle (n = 1). All patients had a paroxysmal pain, with the attacks lasting five to 30 seconds. Pain attacks were mostly spontaneous, but two patients had triggers. Between attacks, all patients had local allodynia. Pain did not increase with vertical eye movements. Six patients were treated with gabapentin with complete response, and one patient experienced long-lasting relief with an anesthetic blockade of the infratrochlear nerve. CONCLUSION: Infratrochlear neuralgia should be considered as a possible cause of pain in the internal angle of the orbit.

Morphometry of the trochlear nerve and superior oblique muscle volume in congenital superior oblique palsy.

PURPOSE: To infer the pathogenic mechanism of congenital superior oblique palsy (SOP) by evaluating trochlear nerve diameter (CN4D) and superior oblique muscle (SO) volume in patients with congenital SOP. METHODS: The medical records of 125 patients diagnosed with unilateral congenital SOP and 34 age-matched healthy controls were reviewed retrospectively. Using thin-section high-resolution magnetic resonance imaging, we evaluated the presence of the trochlear nerve, CN4D, SO volume, and their relationship. RESULTS: Of the 125 patients with congenital SOP, 87 showed absence of the trochlear nerve (n = 87, 70%, absent group) and 38 showed bilateral presence of the trochlear nerve (n = 38, 30%, present group). The nonparetic side CN4D was smaller than controls in the absent group (P = 0.001), and larger than controls in the present group (P = 0.001). Trochlear nerve diameter positively correlated with SO volume in controls (P = 0.014, R(2) = 0.174) and in the nonparetic sides of congenital SOP (present group P = 0.023, R(2) = 0.135; absent group, P = 0.008, R(2) = 0.079). The paretic side SO volume did not show a linear correlation between CN4D and SO volume in the present group (P = 0.243). CONCLUSIONS: In congenital SOP patients without a trochlear nerve, the nonparetic side CN4D was also reduced in contrast to those with a trochlear nerve. The relatively weaker relationship of CN4D and nonparetic side SO volume in the absent group than that of the present group suggests different pathogenic mechanisms underlying these two entities of congenital SOP.

Microvascular decompression for superior oblique myokymia: case report.

BACKGROUND: Superior oblique myokymia (SOM) is a rare disorder in which the patient suffers episodic uniocular torsional eye movement associated with diplopia and oscillopsia . Although the pathophysiology has been narrowed down to erratic discharge of the trochlear nerve, yet the exact etiology remains unclear; a handful of cases have been described in association with an identifiable space occupying lesions or dural AV fistulae. Neurovascular compression theory has been postulated in the early 1980s and to our knowledge, very few reports exist in the literature accrediting this hypothesis in the pathogenesis of superior oblique myokymia. CASE REPORT: We report a case of successful resolution of severe medication refractory SOM following microvascular decompression of the trochlear nerve. The clinical response has been sustained for a follow-up period of 18 months to date. CONCLUSION: Microvascular decompression may be considered as a definitive and least destructive surgical option for the treatment of medication refractory superior oblique myokymia.

Extended subtemporal transtentorial approach to the anterior incisural space and upper clival region: experience with posterior circulation aneurysms.

BACKGROUND: Although most posterior circulation aneurysms are currently treated by endovascular means, some are not amenable to this treatment modality. The narrow working window afforded by the anterolateral and lateral surgical approaches often translates into suboptimal visualization and limited maneuverability. OBJECTIVE: We present a modified technique of tentorial incision and reflection that optimizes the exposure achieved with the traditional subtemporal approach and report our clinical experience in a series of posterior circulation aneurysms. METHODS: Retrospective review of patients operated via an extended subtemporal transtentorial approach for posterior circulation aneurysms. The modified tentorial incision implies dissection of the trochlear nerve along its dural canal up to its entrance into the cavernous sinus and incision of a tentorial flap that extends up to Meckel cave, which is then reflected far anterolaterally. Clinical and radiological data were reviewed. RESULTS: This series comprises 18 patients (21 procedures). Ten patients presented (56%) with a subarachnoid hemorrhage. Aneurysms most frequently arose from the basilar tip (61%) and were of small size (50%) and saccular morphology (72%). Two patients underwent surgery following unsuccessful endovascular treatment. Aneurysm treatment was successful on the first attempt in 90% (19/21) and after a second attempt in 10% (2/21). Documented postoperative palsies of the oculomotor (n = 3) and trochlear (n = 1) nerves were all transient. No procedure-related mortality occurred. CONCLUSION: This modified technique of tentorial incision and reflection optimizes visibility, anatomic orientation, and maneuverability by increasing the rostrocaudal and anterolateral exposure obtained via the extended subtemporal transtentorial route without permanent postoperative trochlear nerve deficit.

Towards understanding ocular motility: III, IV and VI.

The study of the ocular-motor nerves must be exhaustive from their source (nuclei in the brainstem) down to the effector muscles (orbit). Visual disturbances have to be analysed by differentiating between a decrease in visual acuity and ocular-motor disorders. Imaging tests are dominated by MRI, including fine slices and gadolinium injection. A study of the Circle of Willis vessels is often useful, and essential in the case of type III impairment. A further CT scan is essential for analysis of the foramina, base of the skull and orbital walls. Impairment of CN VI requires a CT scan of the apex of petrous. The study of the cavernous sinuses must be in-depth (T2 and T1 after gadolinium and elimination of fats) and always comparative. Impairment of CN III is often complex, difficult to identify precisely (complete or partial, with or without a pupil impairment, associated with other neurological signs) and requires a reasoned study based on anatomical, semiological and pathological knowledge. Other than tumour diseases, it is necessary to consider less well known malformative, ischemic and inflammatory aetiology.

The extended subtemporal transtentorial approach: the impact of trochlear nerve dissection and tentorial incision.

The subtemporal transtentorial approach provides excellent exposure of the incisural space. Incision of the tentorium improves access to the interpeduncular cistern, basilar artery, and rostral ventral pons. Description of the starting and termination points of the tentorial incision has varied greatly. We assessed the impact on surgical exposure of freeing the trochlear nerve (TN) from its dural canal (DC) in addition to dividing and retracting the tentorium. A subtemporal approach was performed on 10 hemispheres of cadaveric specimens. Following exposure of the middle tentorial incisura, the TN is dissected from its DC over a few millimeters. Two retraction sutures are placed along the tentorial edge, posterior to the TN entrance in its DC. The tentorial incision is started between the sutures. Dissection of TN from its DC continues for a short distance. The tentorial incision is extended straight up towards the superior petrosal sinus. Dissection of the TN DC continues anteriorly, up to its entry into the cavernous sinus. The tentorial incision can then be extended, just over the entrance to Meckel's cave, and the flap reflected far anterolateraly. Using this technique, the exposure of the interpeduncular cistern and its content increased by a mean of 8.2 mm (standard deviation [SD] 3.9 mm) in the anteroposterior axis and by 5.5mm (SD 1.9 mm) in the rostrocaudal axis. Tentorial incision following dissection of the TN from its DC optimizes reflection of the tentorium flap anterolateraly, maximizes the exposure, and improves lighting and visibility as well as maneuverability within the interpeduncular and rostral pre-pontine cisterns.