Surgical Management of Overgrowth Syndrome With Bilateral Vision-Threatening Ptosis.

We describe the first case reported in ophthalmological literature of the surgical management of a 17-month-old boy with bilateral vision-threatening ptosis, tarsomegaly, ectropion, and euryblepharon secondary to suspected overgrowth syndrome. We elaborate on the major challenges associated with surgical management including the natural and asymmetric growth of oversized tissue, the high likelihood of scarring and formation of disorganized tissue, and risks of frequent intubation in these patients who may have lesions that compromise critical structures such as the airway. Ultimately, surgical intervention is encouraged primarily if vision or ocular health is threatened and secondarily to achieve good cosmesis.

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.

Clinical characteristics and surgical outcomes of transcutaneous versus transconjunctival excision of Wolfring gland ductal cysts.

PURPOSE: To analyze the clinicopathological characteristics and surgical outcomes of patients with Wolfring gland ductal cysts (WGDCs). METHODS: A retrospective, consecutive, interventional comparative case series was performed over a period of 7 years. Data on demographic and clinical characteristics, pathological findings and outcomes of surgically excised cysts were collected. A comparison between the transconjunctival and transcutaneous approaches was also assessed. RESULTS: Forty-eight patients (48 eyelids) were included in the final analysis. The most common presenting symptom was painless eyelid swelling (81.3%). The median symptom duration was 11.5 months (IQR, 18.25). The upper eyelid was involved in 31 (64.6%) patients, 29/31 of whom had cysts in a medial or centromedial location. Forty-five (93.8%) cysts were bluish gray and transilluminable with clear contents on lid eversion and a median largest dimension of 22 mm (IQR, 8). A transverse conjunctival fibrotic band was observed along the proximal tarsal border in the cyst area in all patients. Signs of chronic trachoma were noted in 38 (79.2%) patients. Preoperative significant ptosis was present in 28/31 (90.3%) of the upper eyelid cysts. Thirty cysts (62.5%) were excised through the skin, and 18 cysts (37.5%) were excised transconjunctivally. Intraoperative cyst rupture, the need for conjunctival grafting and postoperative residual upper lid ptosis were significantly greater in the transconjunctival group (p = 0.009, p < 0.001, and p = 0.016, respectively). CONCLUSION: The present study highlights the clinicopathological characteristics of a relatively large series of surgically excised WGDCs. Transcutaneous excision of WGDCs has proven to be an effective treatment with fewer adverse sequelae than the transconjunctival approach.

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.

Effect of Tranexamic Acid on Intra- and Postoperative Bleeding in Eyelid Surgery: A Prospective, Randomized, Multicenter, Double-Masked, Control Trial.

PURPOSE: Pilot studies suggest that waiting 15 minutes after a subcutaneous tranexamic acid injection is associated with decreased intraoperative bleeding and postoperative ecchymosis in eyelid surgery. The outcomes of commencing eyelid surgery immediately after injection without a waiting period remain unexplored. METHODS: This prospective, randomized, multicenter, double-masked, controlled study examined bilateral symmetric upper and/or lower lid blepharoplasty or ptosis repair. Patients received tranexamic acid in 1 eyelid and control in the contralateral eyelid. The surgeon recorded the side with more intraoperative bleeding. Two masked graders evaluated periocular ecchymosis at postoperative day 0 and postoperative week 1 (POW 1) with a 5-point scale. At POW 1, patients reported subjective grading of bruising as increased on 1 side or similar on both sides. Results were analyzed with Wilcoxon signed-rank and sign tests. RESULTS: Of 130 patients, there was less eyelid ecchymosis on the tranexamic side at postoperative day 0 ( p = 0.001) and POW 1 ( p < 0.001). By surgery type, the 69 levator advancement surgeries had significantly less ecchymosis at postoperative day 0 ( p < 0.001) and POW 1 ( p = 0.001), while upper eyelid blepharoplasty, combined upper and lower lid blepharoplasty, and conjunctivomullerectomy trended toward significance. Of 68 patients reporting a POW 1 grading, 69% reported less bruising on the tranexamic side ( p < 0.001). Intraoperative bleeding was not significantly different between sides ( p = 0.930). CONCLUSIONS: Without a postinjection waiting period, subcutaneous tranexamic acid for eyelid surgery significantly decreased postoperative ecchymosis on postoperative day 0 and POW 1 but did not affect intraoperative bleeding. Subcutaneous tranexamic acid was not associated with any complications.

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Head rotation and the perception of eyelid height and contour.

PURPOSE: Effective visual perceptual processing is one of the many components of surgical competence. Human face identification is most efficient when viewed upright. However, it is not yet clear how this perception sensitivity impacts eyelid symmetry. This study investigates surgeons' and laypeople's accuracy and efficiency in perceiving eyelid asymmetry from different spatial perspectives. METHODS: A prospective psychometric experiment was conducted where oculoplastic surgeons were recruited from the American Society of Ophthalmic Plastic and Reconstructive Surgery and the Brazilian Oculoplastic Surgery Society, and control participants were recruited via crowdsourcing (Amazon's Mechanical Turk). Standard illustrations of the human face with varying degrees of eyelid abnormality, laterality, gender and rotation were presented to participants who were asked to judge whether the eyelids were symmetric or asymmetric. RESULTS: The survey was completed by 75 oculoplastic surgeons (49.33% male; mean age of 46.9±10.7) and 192 lay individuals (54.6% male; mean age 34.6±11.3 years). Among oculoplastic surgeons, deviation from upright was significantly associated with increased reaction time and decreased proportion correct (OR per 45° for peak 0.68, 95% CI 0.60 to 0.77, p<0.001; OR per 45° for ptosis 0.52, 95% CI 0.32 to 0.87, p=0.012; OR per 180° for aggregate responses 0.56, 95% CI 0.51 to 0.61, p<0.001). Oculoplastic surgeons demonstrated increasing accuracy and decreasing reaction time with additional trials for both peak and ptosis. CONCLUSION: Oculoplastic surgeons perceive eyelid asymmetries more accurately and can better compensate for inverted sensory information. However, accuracy increases and reaction time decreases with additional trials, suggesting trainability and potential for improvement in inversion disability.

Quantitative analysis of the eyelid curvature in patients with blepharoptosis.

BACKGROUND: The aim of this study was to evaluate the ability of two novel eyelid curvature measurements to distinguish between normal eyes and different severities of blepharoptosis. METHODS: A comparative cross-sectional analysis of upper eyelid curvature was performed for different severities of patients with unilateral blepharoptosis (congenital and aponeurotic) and normal controls. Mean upper lid contour index (ULCI) and area circularity index (ACI) were calculated for each group by dividing the intercanthal distance by upper eyelid margin length (ULCI) and dividing the interpalpebral area by the area of a circle enclosing the eye (ACI). The ratio of each index for the study and fellow normal eye of each patient was also calculated and compared between groups. RESULTS: A total of 106 eyes including 30 eyes in the control group and 25, 27, and 24 eyes in the mild, moderate, and severe ptosis groups were enrolled in the study. ULCI and ACI showed a statistically significant difference between the groups (p < 0.001, p < 0.001). The inter-eye ratio (ULCI-ratio and ACI-ratio) of indices was also significantly different between groups (p = 0.002, p < 0.001). Pairwise comparisons revealed that ACI and ACI-ratio were significantly different between all pairs of study groups. CONCLUSION: The results of our study showed that ACI based on area measurements may distinguish blepharoptosis patients from normal controls and from each other. Including the data from the fellow normal eyes in the form of ratio indices may improve the differentiating power. These results can be useful in designing the optimal eyelid curvature measurements.

Oculomasticatory rhythmic movements, insomnia and stroke-like episodes in a patient with POLG mutation.

The POLG mutation, a leading cause of mitochondrial diseases, exhibits a wide-ranging age of onset and a complex clinical presentation. We encountered an atypical clinical profile in an elderly man with a POLG mutation, characterised by a stroke-like episode, chronic insomnia and transient oculomasticatory rhythmic movement. History revealed chronic constipation since his 50s and progressive bilateral ophthalmoplegia since his early 60s. Subsequently, he had experienced acute encephalopathy and later developed chronic insomnia. The present neurological examination showed bilateral complete ophthalmoplegia, ptosis, and rhythmic ocular and jaw movements. Imaging indicated findings suggestive of a stroke-like episode and eventual genetic analysis revealed a homozygous missense mutation in the POLG gene. This case expands the clinical spectrum of POLG mutations in individuals over 60 years, showcasing the rare combination of a stroke-like episode, chronic insomnia and oculomasticatory rhythmic movement.

Ptosis secondary to orbital metastasis undetected by magnetic resonance imaging: A case report.

We report a patient with isolated ptosis secondary to orbital metastasis but no evidence of a neoplastic process on magnetic resonance imaging (MRI). A 69-year-old male was referred to our hospital with ptosis of the right upper eyelid and secondary frontalis muscle overaction on the left side for six months. The palpebral fissure was 3mm on the right and 16mm on the left, and levator function was 6mm and 19mm respectively. Three years previously, he had undergone surgical resection of the left lower lobe of the lung due to a T1 N0 M0 adenocarcinoma, and no recurrence had since been observed. An MRI was performed to rule out ptosis secondary to metastasis. Thyroid dysfunction and myasthenia were also ruled out. The ptosis was attributed to an isolated microvascular third nerve palsy. A standard external levator advancement was performed. Six months after the surgery, the patient presented with intense pain, minimal ptosis, edema and diplopia. A new MRI revealed an orbital metastasis measuring 2.4×1.0×1.6cm in the superior orbit, exerting mass effect on the superior rectus and the levator palpebrae superioris muscle. A positron emission tomography scan revealed a recurrence of the lung cancer with multiple bony metastases. The patient underwent chemotherapy consisting of the taxol-carboplatin protocol. Our report highlights the importance of a high index of suspicion of a neoplastic etiology in patients with mild symptoms and a history of cancer even if imaging does not initially reveal a mass or infiltration.

Recovery of subcutaneous orbital fascia under preserved skin below the original crease in the repair of excessively high double-eyelid folds.

BACKGROUND: High-eyelid fold is a common complication of upper eyelid surgery. This study proposed a novel technique for correcting high-eyelid fold in Asian patients with little eyelid skin and thick orbital fascia to improve cosmetic outcomes and patient satisfaction. METHODS: We retrospectively analysed the clinical data of 38 patients with high-eyelid fold repaired at the Plastic Surgery Hospital, Chinese Academy of Medical Sciences, from July 2017 to April 2022. All patients were treated using this method of preserving the high-eyelid fold and reconstructing the subcutaneous orbital fascia. Postoperative outcomes were reviewed and analysed using objective measures and subjective surgeon and patient evaluations. RESULTS: Thirty-eight patients (71 eyes) were included. Postoperative follow-up was for 6-25 months. At 6 months postoperatively, the mean anatomic crease height decreased from 10.45 mm to 6.22 mm, mean pretarsal show decreased from 3.20 mm to 1.61 mm, and mean margin reflex distance in 1 of the 31 patients with ptosis increased from 2.93 mm to 3.87 mm (P < 0.001). The compliance rate between the surgeon's assessment and patient satisfaction was 89.5%, with 31 cases considered good by both the surgeon and patient and one case considered poor by both (undercorrected ptosis), though a satisfactory result was achieved after reoperation. Neither recurrence of the original crease nor serious complications were reported. CONCLUSION: By preserving high-eyelid folds and reconstructing the subcutaneous orbital fascia, unnaturally high- and deep-eyelid folds were converted to lower, nondepressed folds.

Congenital ptosis with aponeurotic maldevelopment: clinical and surgical perspectives : Aponeurotic maldevelopment in congenital ptosis.

PURPOSE: Levator muscle dystrophy has been commonly accused being the main pathology of congenital ptosis, nevertheless, few reports drew attention to the existence of congenital aponeurotic defects. This study aims at highlighting the detailed clinical and surgical features of aponeurotic maldevelopment together with the efficacy of simple aponeurosis repair. METHODS: This is a retrospective nonrandomised study including patients with congenital ptosis who underwent levator muscle surgery throughout 4 years. Patients' records were reviewed for the preoperative clinical assessment and photographs, intraoperative recorded data, and videos as well as postoperative data and photographs. RESULTS: Twenty-seven eyes of 22 patients (9.4%) out of 287 eyes were recorded to have absent levator muscle at its typical anatomical insertion site intraoperatively. The mean preoperative MRD1 was (0.44 ± 1.17 mm). The mean levator function was 8.56 ± 3.89 mm, with higher-than-normal crease position (mean value 10.07 ± 1.62 mm). 25 eyes of included cases (92.6%) showed total absence of the levator aponeurosis edge which only was revealed after cutting through the orbital septal covering of the preaponeurotic fat. CONCLUSION: Congenital aponeurotic defect is an established yet under reported entity of congenital ptosis with reproducible characteristic intraoperative findings. Simple aponeurosis repair could achieve ptosis correction in such cases.

A novel hybrid BonT-A and hyaluronic acid cannula-based technique for eyelid ptosis and periorbital rejuvenation.

BACKGROUND: Eyelid ptosis is characterized by an inferior displacement of the upper eyelid when the eye assumes its primary position. Besides its aesthetic implications, ptosis can also adversely affect visual acuity. OBJECTIVE: This study aimed to evaluate the simultaneous administration of IncobotulinumtoxinA (IncoBonTA) and hyaluronic acid effect in eyelid ptosis and ocular rejuvenation. METHODS: A novel, non-surgical technique for eyelid ptosis management involving IncoBonTA and hyaluronic acid the co-administration within a single syringe, and applied using a cannula. RESULTS: The dual action of IncoBonTA and hyaluronic acid in conjunction with the exact injection sites approaches improves overall aesthetic outcomes but also optimizes the restoration of eyelid functionality in palpebral ptosis. CONCLUSIONS: The functional balance achieved among the contributory muscles-primarily the orbicularis oculi (OO) and its antagonists, the frontal muscle and levator palpebrae superioris (LPS), yields to both, cosmetic and functional.

Sebaceous carcinoma in a 54-year-old Black African man after cancer chemotherapy: a case report.

BACKGROUND: Sebaceous carcinoma is a very rare malignant skin adnexal tumor that is occasionally aggressive. We have not seen a case of sebaceous carcinoma in our center in the last 10 years. It is extremely rare in Black Africans. CASE PRESENTATION: We described the case of a 55-year-old man African man who presented to our ophthalmologist with complaints of growth on the right upper eyelid for 8 months. He had surgery and chemotherapy for rectal carcinoma 6 years prior to presentation and received his last dose of chemotherapy 5 years before seeing our ophthalmologist. There was a history of spontaneous unprovoked bleeding from the lesion. He subsequently underwent surgical excision under general anesthesia. Histology of the mass showed an effaced architecture due to proliferating malignant epithelial cells disposed as trabecules, solid nests, and tongues. The microscopic features of widespread multivacuolated cytoplasm of the neoplastic cells led us to conclude that the tumor was a sebaceous carcinoma. The patient is alive and well. CONCLUSION: Sebaceous carcinoma is a rare malignant skin adnexal tumor in Black Africans. It can present as an eyelid mass with spontaneous bleeding. It can follow cancer chemotherapy either because of its association with other tumors in Muir-Torre syndrome or because of mutagenic effects of chemotherapeutic agents.

Sling for the sling: a new technique for long-term correction of severe congenital ptosis.

INTRODUCTION: Severe congenital ptosis poses a complex challenge for oculoplastic surgeons, requiring meticulous surgical intervention to restore eyelid function and improve aesthetic outcomes mainly by using frontalis sling approach. A crucial issue in frontalis sling surgeries is the sustainability of effect. PURPOSE: This retrospective study reports the outcomes of two surgical techniques for treating severe congenital ptosis in the paediatric age group: Silicon rods ptosis sling and a novel technique involving the use of Silicon rods with green braided polyester (Ethibond) sutures to secure the rods in place "sling for the sling". METHODS: The medical records of children who underwent frontalis suspension were reviewed in a retrospective fashion. We identified two groups; the first group (20 patients: 35 eyelids) had the traditional frontalis suspension surgery using silicone suspension set, the second group (14 patients: 25 eyelids) was operated using the new "sling for sling" technique. We used the postoperative marginal reflex distance-1 (MRD-1) as the primary outcome measure while the frequency of both wound related complications and recurrence were considered as secondary outcome measures. Post operative data were collected and compared after 1 month, 6 months, 12 months, and 18 months. RESULTS: Preliminary results indicate promising outcomes for both techniques, with significant improvement in eyelid elevation observed in both groups. However, the novel technique using Silicon rods with Ethibond sutures demonstrated enhanced sustainability, leading to a more durable outcome with significantly less recurrence. CONCLUSION: This study highlights the potential benefits of the novel technique in treating severe congenital ptosis and introduces an innovative approach to Silicone rods fixation to achieve a long-term corrective effect.

Digital analysis of unilateral ptosis repair: external levator advancement vs. Müller's muscle conjunctival resection.

PURPOSE: Evaluation of lid contour and marginal peak point changes to compare outcomes of external levator advancement and Miiller's muscle conjunctival resection surgery in unilateral ptosis. METHODS: We reviewed the charts of unilateral ptosis patients who underwent external levator advancement or Miiller's muscle conjunctival resection. Eyelid contour analysis was conducted on preoperative and 6-month postoperative digital images. This was performed with the multiple margin reflex distances technique, measuring the vertical distance from a line intersecting the center of the pupil to the eyelid margin at 10 positions at 2 mm intervals. The marginal peak point changes were analyzed digitally using the coordinates of the peak point according to the pupil center. Each position's mean distance was compared preoperatively, postoperatively, and with the fellow eyelid. RESULTS: Sixteen patients underwent external levator advancement and 16 patients had Miiller's muscle conjunctival resection. The mean margin reflex distance was improved by both techniques (1.46 vs. 2.43 mm and 1.12 vs. 2.25 mm, p=0.008 and p=0.0001 respectively) and approached that of the fellow eyelid (2.43 vs. 2.88 and 2.25 vs. 2.58 mm, p=0.23 and p=0.19, respectively). However, statistically significant lid margin elevation was limited to between the N6 and T6 points in the external levator advancement group. Whereas, significant elevation was achieved along the whole lid margin in the Miiller's muscle conjunctival resection group. The marginal peak point was shifted slightly laterally in the external levator advancement group (p=0.11). CONCLUSIONS: Both techniques provide effective lid elevation, however, the external levator advancement's effect lessens toward the canthi while Müller's muscle conjunctival resection provides more uniform elevation across the lid margin. The margin reflex distance alone is not sufficient to reflect contour changes.

A Systematic Quality Assessment of Online Resources on Eyelid Ptosis Using the Modified Ensuring Quality Information for Patients (mEQIP) Tool.

BACKGROUND: Eyelid ptosis is an underestimated pathology deeply affecting patients' quality of life. Internet has increasingly become the major source of information regarding health care, and patients often browse on websites to acquire an initial knowledge on the subject. However, there is lack of data concerning the quality of available information focusing on the eyelid ptosis and its treatment. We systematically evaluated online information quality on eyelid ptosis by using the "Ensuring Quality Information for Patients" (EQIP) scale. MATERIALS AND METHODS: Google, Yahoo and Bing have been searched for the keywords "Eyelid ptosis," "Eyelid ptosis surgery" and "Blepharoptosis." The first 50 hits were included, evaluating the quality of information with the expanded EQIP tool. Websites in English and intended for general non-medical public use were included. Irrelevant documents, videos, pictures, blogs and articles with no access were excluded. RESULTS: Out of 138 eligible websites, 79 (57,7%) addressed more than 20 EQIP items, with an overall median score of 20,2. Only 2% discussed procedure complication rates. The majority fail to disclose severe complications and quantifying risks, fewer than 18% clarified the potential need for additional treatments. Surgical procedure details were lacking, and there was insufficient information about pre-/postoperative precautions for patients. Currently, online quality information has not improved since COVID-19 pandemic. CONCLUSIONS: This study highlights the urgent requirement for improved patient-oriented websites adhering to international standards for plastic and oculoplastic surgery. Healthcare providers should effectively guide their patients in finding trustworthy and reliable eyelid ptosis correction information. LEVEL OF EVIDENCE V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .