[Vertical retraction syndrome caused by anomalous orbital structures].

OBJECTIVE: To described the clinical feature and MRI imaging of six children with vertical retraction syndrome. METHODS: Six children with unilateral vertical retraction syndrome between 15 months and 8 years of age, mean age was (5.01 ± 1.27) years old. Strabismus examination included diopter, prism diopters, eye movement examination, binocular vision and fundus examination. Imaging of the ocular motor nerves at the brainstem was performed in 0.8 mm thickness image planes using 3D-FIESTA sequence, the orbits were imaged with FSE T1, T2WI using surface coils, and within 2.0 mm thick planes. RESULTS: Four children showed hypertropia, characterized by limited depression, a light retraction of the globe during downward gaze and eyelid lag. The MRI imaging showed anomalous orbital structure in the superonasal quadrant that between medial rectus and superior rectus or adjacent to the superior rectus. Two children showed intermittent exotropia, characterized by limited elevation, retraction of the globe and narrowing of the palpebral fissure during upward gaze. The MRI imaging showed anomalous orbital structure was present in the inferotemporal quadrant, one originate in inferior rectus and another close to the lateral rectus. CONCLUSION: Anomalous orbital structures are a main cause of vertical retraction syndrome. The presence of specific unusual eye movement and MRI imaging may assist in diagnosis. When the eyelid lag was found since the early age, anomalous orbital structures were implied.

[What is the role of MR imaging in the diagnosis of strabismus?].

The advent of magnetic resonance imaging (MRI) has given us previously unavailable opportunities to visualize the functional anatomy of extraocular muscles and corresponding ocular motor nerves along the intraorbital and intracranial course of living subjects. Ophthalmologists should be aware of these new MRI techniques. We believe that MRI will play a more marked role in the diagnosis and management of incomitant strabismus.

[Magnetic resonance imaging of the ocular motor nerves in normal volunteers].

OBJECTIVE: To display the detail functional anatomy of ocular motor nerves in the orbits, cavernous sinus and brainstem by high-resolution MRI. METHODS: Twenty normal subjects between 24 and 38 years of age, mean age was 28.25 +/- 4.07 (SD). Imaging of the ocular motor nerves at the brainstem was performed in 0.8 mm thickness image planes using 3D-FIESTA sequence; nerves in the cavernous sinus was imaged with a head coil, the FSE/T(1)WI was obtained in coronal planes; nerves to extraocular muscles (EOMs) in the orbits were imaged with FSE/T(1)WI oblique-coronal planes using surface coils, and within 2.0 mm thick planes. A human orbit was celloidin embedding and serially sectioned at 0.1 mm of thickness for HE stain, which would be used to distinguish motor nerves to the EOMs. RESULTS: Oculomotor nerves (CN3) and abducens (CN6) of the cisternal segment were well demonstrable in all normal subjects. Trochlear nerves (CN4) were depicted 95%. CN3 could be demonstrated consistently in the cavernous sinus, because of their small diameter, CN4 and CN6 not always detectable. The larger inferior division of CN3 and branches to the target EOMs and CN6 were demonstrated well in all species. The superior division of CN3 was demonstrated 60%, CN4 was showed 40%. CONCLUSION: 3D-FIESTA Sequence combined with MPR (Multiplanar Reformation) could precisely show the ocular motor nerves, as well as the relationships with adjacent structures in the cistern. The inferior division of CN3 and the CN6 could be well depicted in the orbits. But of the small sizes and complicated neighboring structures in the orbit, the superior division of the CN3 and the CN4 could not be demonstrated consistently. The capabilities of MRI can be fully used only with thorough knowledge of the complicated topographic relationships in the corresponding region.

Outcomes of secondary sulcus intraocular lens implantation in unilateral anterior persistent fetal vasculature.

AIM: To evaluate the surgical results of sulcus intraocular lens (IOL) implantation in children with unilateral anterior persistent fetal vasculature (PFV) underwent primary vitrectomy combined with lensectomy and preservation of the peripheral anterior capsule. METHODS: Twenty-two eyes of 22 children with unilateral anterior PFV who underwent sulcus secondary IOL implantation were analyzed. Main outcome measures were preoperative and postoperative visual acuity, and complications both intraoperatively and postoperatively. RESULTS: Review of 22 consecutive patients identified best-corrected visual acuity (BCVA) improvement from 1.37±0.84 to 0.73±0.57 logarithm of the minimal angle of resolution (logMAR) after IOL implantation (P<0.001) with a mean follow-up was 16.55±5.86mo. Average age at secondary IOL implantation was 41.05±15.41mo. Three eyes (13.64%) achieved BCVA of 0.3 logMAR at the final visit. Transient intraocular pressure rise (4 eyes; 18.18%), postoperative increased inflammation (3 eyes; 13.64%) and postoperative hypotony (2 eyes; 9.09%) were common complications. CONCLUSION: Properly preservation of the anterior lens capsule during the primary surgery facilitated secondary sulcus IOL implantation in pediatric patients with anterior PFV, with favorable postoperative visual outcomes and compatible percentage of complications.

Congenital Monocular Strabismus Fixus.

PURPOSE: To investigate the clinical characteristics and magnetic resonance imaging (MRI) findings of the extraocular muscle and ocular motor nerves in congenital monocular strabismus fixus. METHODS: The retrospective observational case series of three patients with congenital monocular strabismus fixus were reviewed between January 1, 2006, and December 31, 2016. Ophthalmologic examination and thin-sectioned MRI of the ocular motor nerve and the orbit were performed on the three patients. RESULTS: Three patients presented with unilateral non-progressive strabismus fixus with marked limitations of movement in all directions since birth. Of the three patients, one presented with esotropia, one with a large degree of exotropia and hypertropia, and one with an almost normal primary position. All three patients had normal ocular motor nerves, but adherences among the extraocular muscles, posterior Tenon's capsule, and the globe within the muscle cone on MRI. Two patients underwent strabismus surgery, but there were no postoperative improvements in the primary position and eye movements. CONCLUSIONS: Extensive adherences among the extraocular muscles, posterior Tenon's capsule, and globe may partially explain the cause of congenital monocular strabismus fixus and why strabismus surgery was ineffective. The findings further highlight the importance of MRI in detecting and characterizing atypical forms of strabismus. [J Pediatr Ophthalmol Strabismus. 2018;55(6):363-368.].

[A family of congenital fibrosis of extraocular muscles associated with juvenile canities].

OBJECTIVE: To analyze the clinical manifestations of affected individuals in a family of congenital fibrosis of the extraocular muscles (CFEOM) with juvenile canities. METHODS: All affected and unaffected individuals were retrospectively analyzed in this study. The clinical features include genetic aspects, sex, age, ptosis, restriction of eye movement, aberrant innervation and surgical procedures, were evaluated. RESULTS: This pedigree was inherited as autosomal dominant. There were 14 cases suffering from congenital fibrosis of extraocular muscles in four generations. They had congenital blepharoptosis, head-tilt, chin lift and primary gaze fixed in a hypotrophic position. But vertical and horizontal positions of the eye and restriction of eye movement were different among affected individuals. Some of them also had pupillary abnormally, aberrant innervation and juvenile canities. Inferior rectus recession improved hypotropia in patients with infraducted eyes and chin elevation. Horizontal muscle recession corrected horizontal strabismus satisfactorily in most cases. Ptosis was repaired by frontalis sling or levator resection. CONCLUSIONS: This is the first report of CFEOM associated with juvenile canities. There was phenotypic heterogeneity in this CFEOM pedigree. So the phenotype alone is not sufficient to distinguish among the 3 genotypically distinct CFEOM syndromes. The combination of clinical characteristics and genetic analysis are the basis for the establishment of diagnosis.

Outcomes of small gauge pars plicata vitrectomy for patients with persistent fetal vasculature: a report of 105 cases.

AIM: To evaluate the surgical outcomes in eyes with persistent fetal vasculatures (PFV) managed by small gauge pars plicata vitrectomy. METHODS: Consecutive patients with PFV treated by small gauge pars plicata vitrectomy at Beijing Tongren Eye Center between January 2010 and January 2013 were retrospectively reviewed. RESULTS: A total of 118 eyes of 105 patients with PFV were included and undergone small gauge pars plicata vitrectomy, of which 84 (71.2%) eyes had lensectomy and 16 (13.6%) eyes had lens aspiration and immediate intraocular lens implantation. The percentage of sutured scleral incision of 23 gauge vitrectomy (71.7%, 33/46) was higher than that of the 25 gauge vitrectomy (18.1%, 13/72). At last follow-up, visual acuity remained stable in 34 eyes (28.8%) and improved in 84 eyes (71.2%). Age at surgery (less than 2y), anterior type of PFV, and immediate IOL implantation were associated with postoperative improved visual acuity. Sixty five (55.1%) eyes had retinal detachment preoperatively, among which 33 (50.8%, 33/65) eyes had retinal reattachment or partial retinal reattachment. CONCLUSION: The results suggest that cases with PFV have a potential for developing good visual acuity after small gauge pars plicata vitrectomy with favorable anatomic outcomes and acceptable rate of serious surgical complications.

Magnetic resonance imaging findings in sporadic Möbius syndrome.

BACKGROUND: Although neuroradiological findings of Möbius syndrome have been reported as a result of brain and brainstem abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of the cranial nerve (CN) and branches in the orbits. This study presents the MRI findings in patients with sporadic Möbius syndrome. METHODS: Prospectively, CNs were imaged in the cistern using head coils and three dimensional fast imaging employing steady-state acquisition (3D-FIESTA), yielding a 0.5 mm(2) resolution in planes of 0.8 mm thickness in seven patients with sporadic Möbius syndrome. The cavernous and intraorbital segment of the CN and the extraocular muscles (EOMs) were imaged with T1 weighting in all patients. The cavernous segment was imaged in coronal planes, while the intraorbit in quasicoronal planes were imaged using surface coils. Intraorbital resolution was 0.16 mm(2) within 2.0 mm thick planes. RESULTS: In the seven patients, the CN were absent or showed hypoplasia in the cistern, cavernous sinus, and orbit. Abducens (CN VI) and facial (CN VII) nerves were absent on the affected sides. Unilateral CN IX (glossopharyngeal nerve) in two cases displayed dysplasia. Branches from the inferior division of CN III were observed to innervate the lateral rectus (LR) bilaterally in three cases and unilaterally in one case, and had intimate continuity with the LR muscle in two cases bilaterally and two cases unilaterally. Hypoplasia of EOMs was shown in five cases. Dysplasia of the medulla on the left side was found in one patient. CONCLUSIONS: Direct imaging of CNs and EOMs by MRI is useful in diagnosis of Möbius syndrome. It can directly demonstrate the abnormalities of the CN and orbital structures. The absence or hypoplasia of CN VI and CN VII may be the most common radiologic features in sporadic Möbius syndrome, and hypoplasia of CN IX may be an associated feature. The abnormality of EOMs and aberrant innervations in the orbit should be observed, and may be important for the study of the etiology.

Vertical deviation and A pattern of type III Duane retraction syndrome.

BACKGROUND: Researches in ocular electromyography (EMG) and Magnetic resonance imaging (MRI) of patients with Duane retraction syndrome (DRS) suggest that there may be additional abnormalities such as paradoxical innervation between horizontal rectus muscles and vertical rectus muscles, hypoplasia of vertical rectus muscle and that oblique muscles may also contribute to the heterogeneity of the clinical manifestation of DRS. This paper reports the results of superior rectus recession for vertical deviation and A pattern in DRS Type III and discusses the pathogenesis of the disease. METHODS: Superior and lateral rectus recession were performed in 5 cases of Huber type III DRS to treat vertical deviation and A pattern strabismus. Before operation, MRI of the brain, brainstem, cavernous sinus, and orbits were performed. RESULTS: All subjects had unilateral limitation of both abduction and adduction, with palpebral fissure narrowing and globe retraction in adduction. Three cases had A pattern of strabismus, three cases had hypertropia. The abducens nerves (CN6) were either absent or hypoplasitic in the brainstem in all patients. Two eyes had larger oculomotor foramen. Two eyes had hypoplasia of the superior rectus and the inferior rectus. There was presumably a branch of the third cranial nerve (CN3) innervating the lateral rectus (LR) in one eye. While in another eye, two branches of CN3 sent into medial rectus were revealed. After surgery, vertical deviation in the primary position was reduced in all patients and A pattern was eliminated in 3 patients. One patient developed 10Δ consecutive esotropia postoperatively. CONCLUSION: The results suggest that structural abnormalities of vertical muscle and abnormal orbital innervation may be related to vertical deviation and the presence of A pattern in DRS type III. Recession of the superior rectus muscle seems to be a safe and effective treatment for vertical deviation and A pattern strabismus in DRS Type III.

Magnetic resonance imaging of unilateral vertical retraction syndrome with atypical strabismus.

We report two patients with unilateral vertical retraction syndrome. Magnetic resonance imaging (MRI) of the orbits of the two cases showed similar size and location of the orbital structure, but with dramatically different strabismus type. MRI sagittal reconstruction of the orbits suggested that abnormal muscle tissue arised from the inferior rectus, which might be associated with retraction and narrowing of the palpebral fissure and atypical strabismus as well.

Magnetic resonance imaging of the extraocular muscles and corresponding cranial nerves in patients with special forms of strabismus.

BACKGROUND: With the technical advances, magnetic resonance imaging (MRI) is now sensitive enough to detect subtle structural abnormalities of ocular motor nerves arising from the brainstem and orbits of living subjects. This study was designed to delineate the MRI characteristics in patients with special forms of strabismus. METHODS: A total of 29 patients with special forms of strabismus underwent orbital and intracalvarium MRI. Imaging of the ocular motor nerves in the brainstem was performed in 0.8 mm thickness image planes using the three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) sequence. Nerves to extraocular muscles (EOMs), EOMs and their associated connective tissues were imaged with T1 weighting in tri-planar scans by dual-phased coils within 2.0 mm thick planes. RESULTS: Patients with congenital fibrosis of the extraocular muscles exhibited hypoplasia of the oculomotor (CN3), abducens (CN6), trochlear (CN4) nerves, and the EOMs; hypoplasia of CN6 in the brainstem and an extra branch of the inferior division of CN3 to the lateral rectus were the most common but not the only presentation of Duane's retraction syndrome. Hypoplasia of CN6, facial (CN7) and hypoglossal (CN12) nerves were revealed in patients with Möbius syndrome. In a rare case of bilateral synergistic convergence and divergence, an enlarged branch of CN3 to the medial rectus and a questionable branch of CN3 to the inferior rectus bilaterally were found. CONCLUSION: MRI can reveal subtle structures of the ocular motor nerves and their corresponding EOMs. This can provide valuable information regarding pathogenesis in some special forms of strabismus.