Unilateral lid retraction during pregnancy

A 32-year-old woman noted left lid retraction during pregnancy. Examination revealed unilateral enophthalmos without symptoms of diplopia or sinus disease. Orbital imaging showed characteristic features of the silent sinus syndrome, which were confirmed intraoperatively. The clinical and imaging attributes of this syndrome are discussed, including possible mechanisms of disease development. Management strategies are summarized. A brief discussion of the differential diagnosis of enophthalmos is also included.     

Lid retraction or no lid retraction in optic neuropathy with proptosis

A 58-year-old woman had a history of treated hyperthyroidism and presented with unilateral proptosis and visual loss. The diagnostic evaluation and differential diagnosis were discussed and the treatment of dysthyroid optic neuropathy was outlined.     

Ependymal Tissue in Microphthalmia with Cyst

Purpose: To report the nature of the contents of a cyst associated with microphthalmia and optic nerve coloboma in a male infant. Design: Clinicopathologic report. Case report: A male infant in good general health presented with a massively swollen, protruded left lower eyelid since birth. The globe was not identifiable. MRI disclosed a microphthalmic globe in the upper nasal orbit and an orbital cystic mass consisting of various compartments. Examination of the fellow eye showed a large optic nerve coloboma. As the protrusion of the left lower eyelid was progressive, aspiration of the cyst was performed twice. Finally, the cyst was removed surgically due to repeated filling. Results: Examination of the aspirated fluid showed resemblance to cerebrospinal fluid (CSF). During surgery, the cyst was not found to communicate with the cerebrospinal fluid system. However, tissue resembling choroidal plexus lined the wall of the cyst that was adherent to the optic nerve. Histological evaluation of the tissue revealed ependymal cells. Discussion: The presence of CSF in microphthalmic cysts does not necessarily imply a connection to the CNS, but may be due to local CSF production by ectopic ependymal cells.     

Unilateral eyelid retraction secondary to contralateral ptosis in dysthyroid ophthalmopathy

A patient with dysthyroid eye disease presented with unilateral lid retraction secondary to a contralateral ptosis. While others have reported similar findings in various underlying disease processes, to the best of our knowledge this is the first case in the literature with dysthyroid ophthalmopathy, and the first where the results of surgical management are presented. The importance of testing for secondary lid retraction by manual elevation of the ptotic partner is stressed.     

Ophthalmoplegic migraine with superior ramus oculomotor paresis

We report a 13-year-old girl with recurrent paroxysmal superior division oculomotor palsy as a manifestation of ophthalmoplegic migraine. Neurodiagnostic tests (including head computed tomography scan, cerebral angiography, and Tensilon tests) were normal. This case demonstrates that ophthalmoplegic migraine may selectively affect superior division fibers of the oculomotor nerve.     

Blepharophimosis syndrome: association with colobomatous microphthalmos

Objective: To highlight the association of colobomatous microphthalmos with blepharophimosis syndrome.
Result: We present a case of blepharophimosis syndrome associated with bilateral optic disc, retinochoroidal and iris colobomas, and microphthalmos, in a Caucasian boy. Inheritance in this case was autosomal dominant from the maternal side.
Conclusion: Colobomatous microphthalmos is an infrequent ocular abnormality in the blepharophimosis syndrome. Ophthalmologists should be aware of this association.     

Retinitis pigmentosa,mental retardation,marked short stature,and brachydactyly in two sibs

We present two siblings with retinitis pigmentosa, mental retardation, markedly short stature, and brachydactyly. This association of clinical findings appears to be distinct from previously described syndromes and seems to represent the pleiotropic effects of a single autosomal recessive gene.     

局部皮瓣在眼睑成形手术中的灵活运用

目的探讨在眼睑成形手术中灵活运用各种局部皮瓣的方法及效果。方法在87例（108眼）眼睑成形手术中合理设计和灵活运用多种眼睑局部皮瓣,以修复各种眼睑缺损、治疗复杂的倒睫、睑内翻。结果87例（108眼）睑局部皮瓣全部成活,术后随访3—12个月,瘢痕不明显,皮瓣颜色与周同睑皮肤相近,双眼对称,形态良好。结论在不同的眼睑病变的成形手术中,设计并灵活运用简便而合理的睑局部皮瓣,可较好地恢复眼睑的形态和功能,具有良好的临床疗效。     

Horner's syndrome associated with contralateral superior oblique paresis

A patient presented with Homer's syndrome together with a contralateral superior oblique paresis. Both conditions had been present for at least 12 years. While the patient had suffered what he considered to be minor head trauma on the sporting field and admitted to several altercations during his life, there was no specific event in his medical history that could account for this presentation.     

Unilateral congenital fibrosis of the extraocular muscles with lid retraction: surgical treatment with a silicon plate on the orbital floor

Fibrosis of the extraocular muscles can be an acquired or congenital disorder (CFEOM). The congenital disorder(1) is a complex strabismus with congenital restrictive ophthalmoplegia with or without ptosis. The surgery is challenging because the eye muscles are replaced by fibrous tissue or fibrous bands and in most cases the results are not satisfactory. We present the first case report of unilateral CFEOM with palpebral adherence and hypotropia, which was managed with our technique of a silicon plate implant on the orbital floor. The purpose of the implantation of the silicon plate in the orbital floor is to improve the hypotropia caused by CFEOM.     

Superior division paresis of the oculomotor nerve

Five patients with isolated ptosis and a paresis of ocular elevation in abduction consistent with an isolated superior division III nerve palsy are reported. In all instances the III nerve appeared to be involved before its reported anatomic bifurcation into a superior and an inferior division, in the anterior cavernous sinus.     

Electroretinogram and visual-evoked potentials in children with optic nerve coloboma

The aim was to evaluate alterations in Visual-Evoked Potentials (VEP) and Electroretinogram (ERG) and discover whether these tests are useful for determining residual visual acuity in cases where a patient is unable to collaborate. Flash and, when possible, Transient Pattern Reversal Visual-Evoked Potentials and Maximal Response ERG were recorded in 8 children (under three years old) affected by different aspects of optic nerve coloboma. None of them had visual acuity evaluated because of poor collaboration. All examinations were carried out using skin electrodes. Amplitude of the a and b component of ERG, amplitude, morphology and latency of the major components of Flash VEP and amplitude and latency of P100 Pattern Reversal VEP were evaluated. Four of the patients were examined three years later and visual acuity was compared with the previous electrofunctional results. Alterations in ERG were found only in the case of significant retinal anomalies (great coloboma, retinal detachment), huge alterations were found in both Flash VEP and in Pattern Reversal VEP. The retrospective study of VEP traces and visual acuity showed a good correlation between electrofunctional data and visual capability. Electrofucntional examinations can identify important conductive retinocortical anomalies. The possibility of correlating electrophysiological results with residual visual acuity is important for rehabilitative management in such optic disc malformations. This revised version was published online in July 2006 with corrections to the Cover Date.     

Intracranial aneurysms with superior division paresis of the oculomotor nerve

Five patients with intracranial aneurysms had unilateral ptosis, limitation of supraduction, and normal pupillary reactivity. The most common aneurysm location for superior division paresis of the oculomotor nerve was the superior cerebellar-posterior cerebral artery junction, where aneurysms (3) of the basilar artery compressed and flattened the interpeduncular oculomotor nerve from below. A superior cerebellar artery aneurysm and brain stem infarction affected the intra-axial course of the oculomotor nerve producing superior division paresis. Only an aneurysm of the intracavernous carotid artery resulted in superior division paresis at the expected anatomic site of bifurcation of the oculomotor nerve into its superior and inferior divisions. Because the superior division supplies innervation exclusively to the superior rectus muscle and levator palpebrae, an aneurysm involving this branch does not affect the pupillo-constrictor fibers. Consequently, cerebral angiography should be considered in the initial evaluation of this ocular motor deficit.     

Intracranial oculomotor nerve paresis with anisocoria and pupillary parasympathetic hypersensitivity

Three patients developed anisocoria as a result of third nerve palsies caused by intracranial lesions: two in the cavernous sinus and one in the mesencephalon. Pharmacologic testing using low concentration pilocarpine showed apparent preganglionic parasympathetic supersensitivity of the abnormal pupil. These findings are compatible with the existence of a proposed direct pathway from the midbrain to the eye, without synapse at the ciliary ganglion. Cholinergic hypersensitivity can occur, not only with postciliary ganglionic but also with preciliary ganglionic parasympathetic lesions.     

Superior oblique paresis with contralateral relative afferent pupillary defect

Background: The purpose of this study is to report a case of superior oblique paresis and contralateral relative afferent pupillary defect (RAPD) with normal vision in a patient with brainstem astrocytoma. Methods: We correlated the pa-tient’s clinical findings with anatomical substrates on magnetic resonance imaging (MRI) findings. Results: The patient had right-sided superior oblique paresis. There was a left-sided RAPD, although visual acuities and visual fields were normal in both eyes. T₁-weighted, gadolinium-enhanced MRI demonstrated a hyperintense area in the right dorsal midbrain. Conclusion: It is suggested that the lesion damaged both the pretectal afferent pupillary pathway and fascicles of the trochlear nerve, causing a unique combination of neuro-ophthalmologic findings. Received: 31 January 2000 Revised: 10 July 2000 Accepted: 10 July 2000     

A single transcutaneous injection with Botox for dysthyroid lid retraction

PURPOSE: To evaluate the safety and efficacy of injections with botulinum toxin type A (BTTA,Botox), given transcutaneously, in the treatment of upper lid retraction associated with thyroid eye disease (TED). METHODS: A total of 15 patients (21 eyes) with a stable (TED) condition, and a euthyroid state, were enrolled into the study. There were 12 females and three males from ages 23 to 52 years. A single injection, at the centrally superior tarsal border transcutaneously, aiming at the levator aponeurosis and Müller muscle, was administered into each eyelid with 5-6 U of Botox. All patients were followed regularly for 4-6 months. Any complications, such as ptosis, diplopia, pain,or lid ecchymosis were recorded. RESULTS: All patients, except one, experienced much reduction of palpebral fissure. The mean difference of MRD1 between pre- and postinjections of Botox at the first week was -3.1 mm, and the effect remained, at least, for 2 months. There were temporary complications of ptosis in three patients and vertical diplopia in two patients, lasting 3-4 weeks. CONCLUSIONS: A single transcutaneous injection with Botox for the treatment of thyroid lid retraction is safe and effective. Some minor complications may occur, such as ptosis and diplopia; however, it may offer an alternative and temporary method for patients with dysthyroid lid retraction, who are waiting for a staged operation of either an orbital decompression or a strabismus surgery or both.