Blepharoptosis As an Early Manifestation of Neuronal Intranuclear Inclusion Disease.

Neuronal intranuclear inclusion disease (NIID) exhibits diverse clinical manifestations. Our patient was a 64-year-old woman with bilateral ptosis as the chief complaint. She had bilateral miosis, and the pupil was only slightly dilated 60 min after 1% phenylephrine administration, suggesting autonomic dysfunction secondary to preganglionic sympathetic impairment. A head-up tilt test revealed asymptomatic orthostatic hypotension. She was diagnosed with NIID based on a skin biopsy and genetic testing. This study suggests that blepharoptosis is an early manifestation of NIID. Furthermore, patients with suspected NIID should be examined carefully for autonomic dysfunction.

Juvenile ocular myasthenia gravis: a report of two cases.

We report 2 cases of pediatric ocular myasthenia gravis. The first case was a 7-year-old girl who presented with bilateral ophthalmoplegia and ptosis that correlated with the onset of upper respiratory symptoms. Neuroimaging and acetylcholine receptor antibody testing were unremarkable. The ice pack test was positive. Symptoms greatly improved with pyridostigmine, with full resolution of ophthalmoplegia achieved by 8-month follow-up. The second case was a 4-year-old girl who presented emergently with ptosis and bilateral ophthalmoplegia. Acetylcholine receptor antibodies testing was positive. The patient was started on pyridostigmine and intravenous immunoglobulin and is scheduled to follow-up with pediatric ophthalmology in the outpatient setting.

A review of nonsurgical facial rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1% in periorbital revitalization.

BACKGROUND: The demand for nonsurgical facial rejuvenation options is growing, yet the periorbital region remains an area of relative unmet need. This review explores nonsurgical options for facial rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1%, in treating age-related blepharoptosis as part of periorbital rejuvenation. METHODS: Advisors experienced in facial rejuvenation met to discuss existing literature on the upper face and periorbital rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1%, in treating facial aging. RESULTS: An array of nonsurgical options exist to address the signs of aging, including minimally invasive treatments, such as botulinum toxin injections and dermal fillers, and noninvasive therapy, such as lasers, chemical peels, and microdermabrasion. However, treating age-related ptosis in periorbital rejuvenation is mainly addressed surgically. The newly approved α-adrenergic receptor agonist oxymetazoline hydrochloride ophthalmic solution, 0.1%, provides a novel non-interventional approach to blepharoptosis. CONCLUSIONS: Facial rejuvenation is highly sought-after in this post-pandemic era. Each nonsurgical treatment option has its advantages and drawbacks. A patient-centered approach is necessary to select the appropriate procedure considering the patient's concerns and aesthetic sensibilities. The eyes are an area of primary concern for patients, yet surgery is the gold standard for treating ptosis. Oxymetazoline hydrochloride ophthalmic solution, 0.1%, is a safe and effective nonsurgical treatment for blepharoptosis.

Pediatric Ocular Myasthenia Gravis: Single-Center Experience.

BACKGROUND: Currently, there is no universally accepted standard treatment for ocular myasthenia gravis (OMG) in children. We aimed to investigate the possible proper regimens and timing of treatment for pediatric OMG cases based on the clinical manifestations: OMG with ptosis only and OMG with other features. METHODS: One hundred and forty two OMG cases attended at the Department of Pediatrics, Xiangya Hospital, Central South University, from 2010 to 2019 were included, and information from medical records was reviewed and recorded. Comparisons of clinical characteristics between patients with OMG with ptosis only and patients with OMG with other features as well as between patients treated with glucocorticoid (GC) within or after six months from disease onset were performed. RESULTS: OMG with other features constituted about 54.9% of the cases, and 66.2% of the patients achieved optimal outcome. Patients with OMG with ptosis only responded to pyridostigmine alone more than patients with OMG with other features who required several therapies (P < 0.001). Patients with OMG with ptosis only had a larger proportion of optimal outcome than the patients with OMG with other features (P = 0.002), and the difference remained significant even when the individual outcome groups were compared (P < 0.001). Patients who received GC within six months had a greater proportion of optimal outcome than those who received it after six months (P < 0.001). CONCLUSIONS: Although OMG with other features is a more common subtype of OMG, it is also more severe than OMG with ptosis only. An earlier addition of GC leads to optimal outcome.

Older Woman With Proptosis, Ptosis, and Blurred Vision.

A woman in her early 70s with a history of coronary artery disease, hypertension, and colon polyps presented to oculoplastic surgery with 1 week of progressive right-sided proptosis, headache, right eyelid ptosis, and blurry vision. Outside magnetic resonance imaging demonstrated an infiltrative mass involving right greater than left orbital apices, the right optic nerve, and right extraocular muscles. What would you do next?

Blepharoptosis and cognitive performance: a population-based study of 1.4 million adolescents.

The purpose of this study is to examine the association between blepharoptosis and cognitive performance in late adolescence. This population-based, retrospective, cross-sectional study included 1,411,570 Israeli-born adolescents (620,107 women, 43.9%) aged 16-19 years who were medically examined before compulsory military service between 1993 and 2017. The diagnosis of blepharoptosis was verified by an ophthalmologist. Cognitive performance was assessed by a validated intelligence-quotient-equivalent test, comprising four domains (problem-solving, verbal abstraction and categorization, verbal comprehension, and mathematical abilities). Cognitive Z-scores were calculated and categorized as high (≥ 1 standard deviation (SD)), medium (- 1 to < 1 SD), and low (less than - 1 SD). Relationships were analyzed using regression models adjusted for sociodemographic variables including sex, year of birth, residential socioeconomic status, education level, body mass index, and familial country of origin. A total of 577 (41 per 100,000, 32.2% women) adolescents were diagnosed with blepharoptosis. The proportions of unilateral and bilateral visual impairment among adolescents with blepharoptosis were 13.0% and 3.5%, respectively. In a multivariable analysis, blepharoptosis was associated with a 0.18 SD reduction in cognitive Z-score (p < 0.001). The adjusted odds ratios for low and high cognitive Z-scores in adolescents with blepharoptosis were 1.54 (1.25-1.89) and 0.80 (0.62-1.04), respectively. This relationship persisted when adolescents with normal best-corrected visual acuity or unimpaired health status were analyzed separately.  Conclusions: Blepharoptosis is associated with reduced cognitive performance determined in late adolescence. Future prospective studies should investigate the causes of this link and their underlying mechanisms. What is Known: • While earlier investigations have examined the effects of blepharoptosis on vision and quality of life, the association between blepharoptosis and cognitive outcomes in youth has remained unexplored. What is New: • This nationwide study involving 1.4 million Israeli adolescents found a correlation between blepharoptosis and reduced cognitive performance. • Our findings suggest a potential interplay between blepharoptosis and cognitive development in the pediatric population, calling for increased focus on the educational needs of affected individuals.

Orbital Fibrotic Band as Cause of Monocular Elevation Deficiency.

Monocular elevation deficiency is a congenital, unilateral inability of elevation. It is classified as innervational, restrictive, or combine types. Here, we report a rare case of monocular elevation deficiency who had 60 PD left hypotropia and left ptosis with limited elevation (-5) both on abduction and adduction. Orbital MRI revealed a hypointense fibrotic band between the superior oblique and superior rectus muscles extending obliquely in the superonasal direction between the sclera and orbital roof. She was successfully treated after severing the fibrotic band between the sclera and bony orbit.

A simplified technique for correction of complete ptosis secondary to palpebral neurofibromatosis.

PURPOSE: To present a simplified technique in management of complete ptosis secondary to neurofibromatosis. METHODS: This prospective, non-comparative, clinical interventional study included 13 patients with complete ptosis secondary to histologically proved plexiform neurofibromas. It was conducted at the Orbital Unit of Assiut University Hospital, the referral center of Upper Egypt in the period between June 2013 and October 2021. In all cases, a simplified technique of 5 surgical steps was applied: (A) Division of the involved eyelid surgically into three parts by drawing 2 curvilinear lines, the superior line 11 mm below and parallel to the lower eyebrow hairline and the inferior one 10 mm above the lid margin, (B) Resection (full-thickness) of the large middle part which involves the main pathology and lies between the 2 lines, (C) Preservation of the upper part with identification, dissection and clamping of the levator muscle, (D) Refinement of the lower part by removal of any tissue between the skin and the debulked tarsus and (E) Re-suturing of the upper and lower parts in layers; conjunctiva to conjunctiva, levator to tarsus (after resection of a part that corrects the ptosis) and skin to skin. RESULTS: Ptosis was completely corrected in 8 cases (61.5%) and residual mild ptosis occurred in 5 patients (38.5%). No exposure keratopathy or tumor growth was reported during the follow-up period of minimum 1 year. CONCLUSIONS: This simplified technique could be considered as a surgical basis for correction of complete ptosis in neurofibromatosis.

Case Report: Unilateral Hemispheric Hemorrhage with Bilateral Ptosis and Upgaze Palsy.

SIGNIFICANCE: Determining the anatomic location of insult in cases of concurrent bilateral upgaze palsy with bilateral ptosis can be challenging because of the various overlapping pathways and shared functions. It is more commonly related to bilateral oculomotor nerve palsies and myasthenia gravis. However, the possibility of unilateral cerebrovascular events may be overlooked because of the lack of laterality of disease manifestations. PURPOSE: This report documents the uncommon presentation of bilateral ptosis and upgaze palsy in unilateral hemispheric hemorrhage with the corresponding clinical and anatomical review. CASE REPORT: A 46-year-old gentleman presented to the emergency department with left-sided hemiplegia, concurrent bilateral ptosis, and upgaze palsy. He was found to have acute hemorrhagic stroke secondary to significantly elevated blood pressure. Computed tomography of the brain revealed acute extensive intraparenchymal hemorrhage involving the right basal ganglia, frontal lobe, and temporal lobe. There was an extension of hemorrhage into the third ventricle and subarachnoid extension to the Sylvian fissure with obstructive hydrocephalus. An emergency right craniotomy was performed to evacuate the blood clot, and the hydrocephalus subsequently resolved. Post-operatively, bilateral ptosis and upgaze palsy improved and then resolved. CONCLUSIONS: Acute bilateral ptosis and upgaze palsy suggest the possibility of unilateral hemispheric hemorrhage, even though there is no direct involvement of the brainstem and its nuclei.

Severe Upper Lid Fornix Prolapse After a Ptosis Repair in Floppy Eyelid Syndrome: A Case Report.

ABSTRACT: Patients with profound floppy eyelid syndrome (FES) may experience challenges achieving eyelid and tarsal stability in the setting of severe ptosis. A 43-year-old woman with sleep apnea presented to clinic with difficulties driving because of droopy eyelids. She also experienced chronic tearing, crusting, and discharge of the eyes. She was diagnosed with severe FES and significant ptosis. She underwent a wedge excision of the upper lid in the medial and lateral aspect of the upper lid. This was followed by a ptosis repair that resulted in complete upper lid fornix obliteration causing the conjunctiva to obstruct the patient's pupils. The patient's symptoms ultimately improved after a definitive fornix reconstruction. Her visual field symptoms were improved with 1-year follow-up. To our knowledge, this is the first report in the literature of a prolapsed fornix resulting in vision obstruction after floppy eyelid repair via a wedge excision followed by a ptosis repair via an external levator advancement. Given these unusual findings for the patient's age, further investigations into treatment management may be necessary to mitigate eyelid reconstruction complications in younger patients with FES.

[A man with hanging eyelids]. / Een man met hangende oogleden.

A 73-year-old man came to the outpatient clinic because of diplopia and hanging eyelids. Neurological examination revealed fatigueable bilateral ptosis. The patient had a strong positive ice pack-test. Myasthenia gravis preached on top of the differential diagnosis. Subsequent blood tests showed antibodies against ACh-receptors and confirmed the diagnosis.

Pachydermoperiostosis with bilateral ptosis and its associated systemic comorbidities: a rare case report.

Pachydermoperiostosis is a rare genetic disease known as primary or idiopathic hypertrophic osteoarthropathy (HOA)/Touraine-Solente-Gole syndrome. It is an autosomal dominant or recessive disorder comprising digital clubbing, periostosis, hyperhidrosis, and pachydermia (thickening of facial skin). Ocular manifestations are uncommon; however, blepharoptosis may occur. This case presented with severe bilateral ptosis due to the disease progression. A large 20 mm upper lid resection with levator advancement was performed to improve his ability to see. This is the first reported case of pachydermoperiostosis (PDP) in Jamaica. We present a rare case of pachydermoperiostosis with severe blepharoptosis, who attained a good result with surgical intervention.

Clinical Reasoning: A 74-Year-Old Woman Presenting With Monocular Ptosis and Binocular Diplopia.

A 74-year-old woman presented with acute-onset right ptosis and binocular diplopia. CT scan showed low-density lesions in the bilateral basal ganglia and adjacent to lateral ventricles. Intracranial aneurysm was not detected. This case highlights the importance of neurologic localization of ophthalmoplegia based on physical examination and the microanatomy of the oculomotor nerve.

Clinical Reasoning: An Unusual Cause of Diplopia and Ptosis in a 67-Year-Old Woman.

Diplopia is a relatively common chief complaint encountered in an outpatient neurology clinic and carries a broad differential diagnosis. In this case, a 67-year-old woman presented with new horizontal, binocular diplopia and ptosis of 8-month duration, which persisted without significant progression. This case highlights the need for a comprehensive list of differential diagnoses for patients with acquired ophthalmoplegia and ptosis. Key learning points include an illustration of the stepwise diagnostic approach to evaluate for common etiologies, the importance of interpreting test results in the appropriate clinical setting, and the significance of recognizing specific signs and symptoms in achieving the correct diagnosis.

Efficacy of apraclonidine eye drops in treating ptosis secondary to myasthenia gravis: A pilot clinical trial.

INTRODUCTION/AIMS: Most patients with myasthenia gravis (MG) develop ocular manifestations during their illness and up to 22% may have isolated ocular myasthenia gravis (OMG). Apraclonidine elevates the eyelid by activating alpha-2 receptors on Muller's muscle, an accessory eyelid elevator muscle. In this study we evaluate the effect of apraclonidine in alleviating ptosis secondary to MG. METHODS: This clinical trial (NCT05045248) was done at the American University of Beirut Medical Center. Patients with ptosis secondary to MG were administered two drops of apraclonidine 0.5% solution to the most affected eye. We measured palpebral fissure height (PF), marginal reflex distance-1 (MRD1), marginal reflex distance-2 (MRD2), and levator function (LF) before drug administration and at 1, 5, 30, and 60 minutes after administration. RESULTS: Ten participants were enrolled in the study. Improvement in all eyelid measurements was noted in all participants as early as 1 minute after apraclonidine administration. From baseline to 60 minutes after administration, average PF increased from 8.8 ± 1.9 mm to 14.2 ± 2.6 mm, MRD-1 from 1.7 ± 1.4 mm to 5.4 ± 2.9 mm, MRD-2 from 7.1 ± 1.3 mm to 8.8 ± 1.7 mm, and LF from 13.4 ± 2.9 mm to 17.5 ± 2.4 mm. All increases were statistically significant. DISCUSSION: Apraclonidine may alleviate ptosis secondary to MG and may be an effective alternative treatment for this group of patients.

[Research progress of iatrogenic blepharoptosis repair after double eyelid surgery].

Objective: To summarize the etiology mechanism and treatment of iatrogenic blepharoptosis after double eyelid surgery in Asia. Methods: To extensively review the literature related to iatrogenic blepharoptosis after double eyelid surgery, and to summarize and analyze the related anatomical mechanism, existing treatment options, and indications. Results: Iatrogenic blepharoptosis is a relatively common complication after double eyelid surgery, sometimes it is combined with other eyelid deformities such as sunken upper eyelid and wide double eyelid, which makes it difficult to repair. The etiology is mainly caused by improper adhesion of tissues and scars, improper removal of upper eyelid tissue, and injury of a link of levator muscle power system. Whether blepharoptosis occurs after double eyelid surgery by incision or suture, it should be repaired by incision. The principles of repair include surgical loosening of tissue adhesion, anatomical reduction, and repair of damaged tissues. The key is to use surrounding tissues or transplanted fat to prevent adhesion. Conclusion: When repairing iatrogenic blepharoptosis clinically, appropriate surgical methods should be selected based on the causes and severity of the blepharoptosis, combined with treatment principles, in order to achieve better repair results.

Acute unilateral isolated ptosis as a complication of sinusitis in a post-COVID-19 patient.

A fit and well young man presented to our emergency department in the UK. On examination, he had an isolated left-sided ptosis; he had a 3-day history of frontal headache which was worse on head movement. He lacked any clinical signs of cranial, orbital, or preseptal infection, and his eye movements were normal. Ten days before presentation, he tested positive for SARS-CoV-2. Inflammatory markers were moderately raised, and CT of the head did not reveal any vascular abnormality or intracranial lesion. Imaging revealed opacification, predominantly in the left facial sinuses, keeping with sinusitis. He was discharged the same evening with oral antibiotics and made a full recovery over the next few days. He remained well at 6-month follow-up. The authors convey their findings to raise awareness of a rare complication of sinusitis and to demonstrate the utility of CT imaging for diagnosing sinusitis and ruling out severe pathology.