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PURPOSE: A levator dissection-resection technique (LDR) in which the levator is dissected free from all fibrous attachments including Whitnall's ligament was compared to two commonly used frontalis-based procedures in the management of congenital ptosis with poor levator function. METHODS: Thirty patients having congenital ptosis with poor levator function were randomized to one of the three surgical groups (ten patients for each group), namely, frontalis sling (FS), frontalis advancement flap (FAF), and (LDR) technique. Marginal reflex distance-one (MRD-1), levator function, symmetry in different levels of vertical gaze, lagophthalmos, and any other surgical complications were assessed 6 months following the intervention. RESULTS: Patients in the three groups achieved statistically significant improvement in MRD-1 in the primary position of gaze (p = 0.001 for FS, 0.003 for FAF, 0.001 for LDR). Patients who underwent a frontalis-based procedure acquired an additional ability to elevate the upper eyelid by using their eyebrows. Patients who underwent LDR technique have acquired an additional mean of 5.79 ± 1 mm improvement in levator function with better symmetry during up and down gaze in unilateral cases. Patients from all groups had an equal degree of lagophthalmos with forced eyelid closure, and during sleep. CONCLUSION: Patients with ptosis and poor levator function who were managed with LDR technique achieved a similar degree of eyelid elevation in the primary gaze to that of frontalis-based procedures, acquired additional levator function, achieved more symmetry in up and down gaze in unilateral cases, and had no additional risk to the cornea.

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PURPOSE: To identify a correlation between the degree of congenital ptosis and levels of refractive error, visual stimulus deprivation and anisometropic amblyopia, and any improvement with surgical intervention. METHODS: A prospective investigation of 30 patients aged 3 months to 8 years, with a diagnosis of unilateral or bilateral congenital ptosis. A full orthoptic assessment and cycloplegic refraction were performed and patients requiring surgical intervention for ptosis were at surgeon discretion. RESULTS: The mean age at presentation was 27.1 months. The prevalence of amblyopia ranged from 19 to 29%. There was no significant difference in the levels of astigmatism throughout the follow-up period between patients who underwent surgical intervention and who did not. There was a significant improvement in the visual acuity of the affected eye (p = .03) and both eyes open (p = .02), in patients who did not undergo surgery. This was then repeated on patients who underwent surgical intervention which showed no significant difference in the affected eye or both eyes open; p = .27, p = .32, respectively. CONCLUSION: There is no significant change in the levels of astigmatism in both patients who underwent surgery and those who did not. Conservative management of congenital ptosis, while counterintuitive, proves not to deleteriously affect the visual potential of these patients.

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PURPOSE: To analyze the effect of congenital ptosis on corneal topography and total aberrometry and to compare these variables between ptotic and normal fellow eyes. METHODS: The study included 32 eyes of 16 patients with unilateral congenital blepharoptosis. A Shack-Hartmann wavefront sensor was employed to assess Zernike coefficients and root-mean-square. Computerized corneal topography, Orbscan and aberrometry were measured in the healthy and ptotic eyes. Data were analyzed using SPSS version 16. P<0.05 was considered significant. RESULTS: The mean patient age was 21.31±6.3 years. The mean margin to light reflex distance-1 (MRD-1) was 0.6±1.44mm in the ptotic eyes. Among topography variables, surface regularity index (SRI), cylinder power, irregular astigmatism index (IAI), and flat meridian keratometry were significantly different between ptotic and non-ptotic fellow eyes (P<0.05). Some Orbscan parameters, including simulated keratometry, maximum and minimum corneal power, and astigmatism power were significantly different between ptotic and normal fellow eyes (P<0.05). There was no statistically significant difference in total aberrometry variables between paired eyes. However, in a comparison between ptotic eyes with over 1 diopter astigmatism vs. less than 1 D, high-order Zernike modes without spherical aberration at 6mm (HOW/O Z400 6mm) were significantly different between the 2 groups (P=0.02). CONCLUSION: Unilateral congenital ptosis significantly affects corneal topography and aberrometry, especially in eyes with astigmatism≥1 D. Such differences need to be considered before keratorefractive surgery (KRS).

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BACKGROUND: Blepharoptosis is a common symptom in ophthalmology clinic, but eyelid retraction when smiling in a ptosis eye is a rare manifestation. Here we report a novel manifestation that eyelid retraction during smiling in a patient with monocular congenital ptosis. CASE DESCRIPTION: A 10-year-old girl with isolated and mild unilateral congenital ptosis showed eyelid retraction in ptotsis eye when smiling together with a lid lag on downgaze. She didn't have any systematic and ocular diseases other than myopia and astigmatism.Eyelid retraction during smiling is 5 mm, resulting in a significant difference in the height of bilateral palpebral fissures.As for ptosis, is mild.The margin to reflex distance 1 is 1.0 mm on the right eye(ptosis eye) and 3.0 mm on the left eye. A lid lag of 1.0 mm on downward gaze was noted on the right, she could close her eyes fully while sleeping.The ice pack test, laboratory test for thyroid function, whole-exome sequencing (WES) and magnetic resonance imaging(MRI) of the orbital and ocular motor nerves showed normal results.Her symptoms alleviated after 6 months, with the retraction of the right upper eyelid when smiling was approximately 3 mm, thus the difference in the palpebral fissure height when smiling was smaller than that at the initial presentation. CONCLUSION: Blepharoptosis may accompanied with abnormal innervation like eyelid retraction, this phenomenon can be alleviated with age.The results of the levator muscle function test should be carefully examined to determine whether it is ptosis in an impaired innervation eyelid.

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PURPOSE: To carry out a comprehensive critical review of the peer-reviewed literature on the refractive changes associated with oculoplastic surgeries. METHODS: This review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) statement recommendations. Following specific inclusion and exclusion criteria, 20 articles were selected for the current scoping review. Each of them was analyzed carefully and their risk of bias was assessed with the Quality Assessment Tool for case Series Studies from the National Heart, Lung and Blood Institute. RESULTS: Data of 1428 eyes from 1051 patients were analyzed. All studies were case series. Regarding the oculoplastic pathologies, five articles focused on dermatochalasis, four on chalazion, five on ptosis and six on congenital ptosis. Most articles did not report changes in the spherical equivalent (SE), astigmatism changes of less than 0.5 diopters (D), changes in astigmatism axis of less than 11 degrees and changes in uncorrected distance visual acuity (UDVA) of less than 0.06 logMAR. Out of the 20 articles reviewed, 6 achieved a risk of bias score between 6 and 8, representing a low level of evidence and highlighting the limitations in the study design. CONCLUSIONS: Based on the findings of this study, there is no evidence to suggest clinically significant refractive changes following oculoplastic surgeries. The most notable changes may occur after surgery for severe ptosis; however, further research is needed to confirm this observation.

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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.

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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.

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Introduction and importance: Allgrove syndrome (AS) (AAA syndrome) is a rare autosomal recessive disease caused by mutations in the AAAS gene located on chromosome 12q13. The AAAS gene encodes for the ALADIN protein (alacrima, achalasia, adrenal insufficiency, neurologic disorder). AS can manifest with a plethora of symptoms. Early recognition of the syndrome remains challenging due to its rarity and progressive nature. This report presents an unusual case of triple-A syndrome (TAS) with concurrent neuromuscular manifestations. Understanding the atypical presentation of this syndrome is vital for early diagnosis and appropriate management. Case presentation: We report a 16-year-old boy with severe malnutrition presented with painful swallowing, fatigue, and bilateral congenital ptosis. Barium swallow, upper gastrointestinal endoscopy, and Shimmer test were performed, which led to the diagnosis of TAS. Treatment included laparoscopic Heller's procedure, artificial tears, hydrocortisone. Clinical discussion: TAS, also known as AS, is a rare multisystem disorder characterized by achalasia, Addison's disease, and alacrima. This syndrome is occasionally referred to as 4A syndrome due to the inclusion of autonomic dysfunction. There is no treatment for AS. Management includes artificial tears for alacrima, glucocorticoid replacement therapy to treat adrenal insufficiency, and treatment of achalasia. Conclusion: This case emphasizes the importance of considering atypical presentations of TAS. Early diagnosis and treatment are paramount in addressing the varied components of this rare disorder. Understanding the clinical complexities of this syndrome aids in improved patient care and underscores the necessity for comprehensive evaluation and management in similar cases.

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Elastin expression in the conjoint facial sheath (CFS) of patients of different ages with severe ptosis has been extensively studied, but its expression in the CFS of pediatric patients with severe ptosis with different muscle strengths remains poorly understood. The aim of the present study was to investigate the expression of elastin in the CFS and levator palpebrae superioris muscle (LM) of children with severe congenital ptosis with different LM strengths. In total, 20 pediatric patients with unilateral severe congenital ptosis (20 eyes) were included, who underwent CFS + LM complex suspension surgery from June 2020 to February 2022. Among these patients, the LM strength was 0-1 mm in 10 patients and 2-3 mm in the other 10 patients. Excess CFS and LM tissue samples were obtained from the patients during surgery, before the protein expression levels of elastin in the specimens were measured by western blotting. During the 6-month postoperative follow-up period, the good correction rate, the degree of incomplete eyelid closure and the incidence of complications were observed. Western blotting results showed that, compared with that in the 0-1 mm group, elastin expression was not significantly different in the CFS, whereas it was significantly increased (P=0.021) in the LM of the 2-3 mm group. In addition, elastin expression in the CFS was markedly higher compared with that in the LM in both groups (in the 0-1 mm group, P=0.005; in the 2-3 mm group, P=0.009). Additionally, the curative effect evaluation revealed that the good correction rates in the 0-1 and 2-3 mm groups were 90 and 100%, respectively. In total, 3 patients experienced conjunctival prolapse during the follow-up period, including 2 patients in the 0-1 mm group and 1 patient in the 2-3 mm group, but there were no other complications. To conclude, elastin expression in the CFS was found to be higher compared with that in the LM of children with severe congenital ptosis. Although elastin expression in the LM was positively associated with LM strength, its expression in the CFS displayed no clear association with LM function. Therefore, these observations suggested that CFS + LM complex suspension surgery is viable to correct severe congenital ptosis in pediatric patients.

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AIM: to assess the outcomes of a novel algorithm for the calculation of the amount levator muscle plication in congenital blepharoptosis surgery. METHODS: this retrospective comparative study included 34 patients with congenital ptosis subjected to levator muscle plication surgery during the period from October 2021 to November 2022. They were divided into two groups. Group A: the amount of levator muscle plication was calculated by a traditional formula [(amount of ptosis x 3) + 9 mm in cases with good levator function or (amount of ptosis x 3) + 11 mm in cases with fair levator function]. Group B: the amount of levator muscle plication was calculated by a novel nomogram [the result of the traditional formula was modified by subtracting 4 mm if the calculated amount was ≥ 15 mm or subtracting 3 mm if the calculated amount was < 15 mm]. Demographic data, baseline ptosis characteristics and postoperative results at 1st week, 1st month, 3rd month and 6th month were compared between the groups. Primary outcome measure was postoperative Marginal Reflex Distance (MRD1). Secondary outcome measures were lid contour, lid crease and any reported complications. RESULTS: Group A included 20 eyes of 18 patients while Group B included 20 eyes of 16 patients. The mean amount of levator muscle plication was 16.98 ± 2.44 mm and 13.48 ± 2.42 mm in group A and group B respectively. The difference between the two groups was highly statistically significant (p < 0.001). Mean MRD1 at the 1st postoperative week was 4.95 ± 0.37 mm in group A and 4.08 ± 0.64 mm in group B. This difference was highly statistically significant (P < 0.001). Overcorrection was seen in 8 (40%) eyes in group A and 1 (5%) eye in group B. The difference was statistically significant between the two groups (p = 0.008). Undercorrection was seen in only 1 (5%) eye in group B. No other complications were reported. Surgical success was achieved in 12 (60%) eyes in group A versus 18 (90%) eyes in group B. The difference between the two groups was statistically significant (p = 0.03). CONCLUSION: our novel nomogram for the calculation of the amount levator muscle plication in congenital blepharoptosis surgery is effective in achieving a satisfactory postoperative MRD1.

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Congenital cranial dysinnervation disorders(CCDDs)are a group of diseases with congenital non-progressive developmental abnormalities or absence of one or more cranial nerves, resulting in primary or secondary abnormalities of cranial nerves innervating the extraocular muscles. CCDDs can be sporadic or hereditary, and may be accompanied by systemic abnormalities. In recent years, with the research progress of neuropathology, neuroimaging, and genetics, it has not only been clarified that the cause of eye movement disorder in CCDDs is neurogenic, but also been found the pathogenic genes of CCDDs, including SALL4, HOXA1, KIF21A, PHOX2A, TUBB3, and HOXB1, etc. In this review, the relevant domestic and international literatures on the molecular genetics and neuroscience of CCDDs in recent years are reviewed, aiming to address how the causing gene mutations of CCDDs affect brain neural development and further lead to congenital abnormal cranial nerve innervation, in order to provide references for the clinical and basic research of CCDDs.

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PURPOSE: To evaluate the corneal topography and aberrometry and their changes following ptosis surgery in patients suffering from unilateral congenital blepharoptosis. METHODS: The study included 15 eyes of 15 patients with unilateral congenital blepharoptosis who underwent surgical correction through anterior levator resection. Shack-Hartmann wavefront sensor was employed to assess Zernike coefficients and root-mean-square. Computerized corneal topography, Orbscan and aberrometry were measured pre-and 3 months post-surgery in the healthy and ptotic eyes. Aberrometric changes were compared between patients with more than one diopter of astigmatism and subjects with less amount of astigmatism. Data were analyzed using the SPSS version 16. P < 0.05 was considered as significance level. RESULTS: Following anterior levator resection, the amount of astigmatism decreased. However, the difference was not statistically significant. Changes in topographic indices were not statistically significant. Regarding tomographic indices, the change in minimum keratometry was significant. The amount of change in total higher-order aberrations (HOAs) was not statistically significant. However, there was a decrease in the amount of third-order aberrations (vertical coma and vertical trefoil) which was statistically significant for vertical coma (-0.002 ± 0.001 vs. -0.08 ± 0.02 µm, P = 0.02). Comparing aberrometric changes in patients with more than 1 diopter astigmatism and less, there was a significant difference in the amount of HOA w/o Z400. CONCLUSION: Surgical correction of ptosis could reduce the ocular aberrations, despite no significant change in astigmatism. Nonaxial high-order aberrations are mostly affected probably due to the meridional effect of ptosis on the cornea.

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Purpose The purpose of the study is to assess short- and long-term functional outcomes after frontalis suspension using expanded polytetrafluoroethylene (ePTFE) sling for congenital ptosis repair. Methods A retrospective, observational case review was conducted on pediatric patients who underwent frontalis suspension using ePTFE sling from 2008 to 2020. Functional success was assessed by lid height, lid symmetry, and parental satisfaction with the cosmetic outcome. Clinical course and long-term functional outcomes after surgery were assessed. Results Twenty-one cases met the inclusion criteria and were assessed. The follow-up time ranged from 13 months to 11 years (mean: six years). Functional success after one surgery was 62% at early and late postoperative periods. Six of 21 cases (29%) required revisional surgery in the early postoperative period due to undercorrection. Three cases (14%) were complicated by infection and/or granuloma formation. There were no cases of ptosis recurrence in the long term if success was seen in the early postoperative period. Conclusion ePTFE slings remain an excellent option for severe congenital ptosis repair with frontalis sling, demonstrating long-term functional success, with satisfactory lid symmetry and acceptable cosmetic outcome. This is of important consideration in patients younger than three years of age, where autogenous materials may not be recommended. The need for early revisional surgery for undercorrection is not uncommon. The current authors also demonstrate a low but considerable risk for infection and/or granuloma formation.

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The levator palpebrae superioris is the primary muscle responsible for elevation of the eyelid. This muscle is innervated by the third intracranial nerve. Any pathology affecting the muscle or the supplying nerve can lead to blepharoptosis. In this study, we share our experience of a two-year-old baby boy patient who presented with a rare congenital disorder manifested as blepharoptosis increased with adduction bilaterally with no limitation of ocular muscles action except bilateral underaction of inferior oblique muscles. To our knowledge, this unusual presentation has not been previously reported in the literature. We aim in this report to build more knowledge on such a rare clinical presentation. Based on the findings, this could be a case of congenital innervation dysgenesis syndrome (CID)/congenital cranial dysinnervation disorders (CCDDs). CCDDs/CID is a group of conditions that includes blepharoptosis as part of their clinical presentation. This group of conditions includes Duane's retraction syndrome, congenital fibrosis of extraocular muscles, and monocular elevation defect.

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BACKGROUND: Several types of surgeries have been used in the treatment of congenital ptosis, but the optimal methods and materials for this treatment are unknown. PURPOSE: The study aims to evaluate the comparative effectiveness and safety of various surgical methods and materials for treatment of congenital ptosis. METHODS: We performed comprehensive searches of five databases, two clinical trial registries and one gray literature database from inception to January 2022 for related trials to include in this study. Meta-analysis was performed to evaluate the effect of surgical methods and materials on the primary outcomes: margin reflex distance 1 (MRD1), palpebral fissure height (PFH), and degree of lagophthalmos; and secondary outcomes: undercorrection, entropion, corneal epithelial defects, wound dehiscence, recurrence, infection, and cosmetic outcomes. RESULTS: A total of 14 trials evaluating 909 eyes of 657 patients were included in our study. Compared with the levator plication, the frontalis sling significantly increased the MRD1 (MD = - 1.21; 95% CI [- 1.69, - 0.73]), and the levator resection significantly increased the PFH (MD = 1.30; 95% CI [0.27, 2.33]). For the frontalis sling surgical patterns, the fox pentagon was significantly better than the double triangle at improving the degree of lagophthalmos (MD = 0.70; 95% CI [0.32, 1.08]), while the opened pattern provided statistically better cosmetic outcome than the closed frontalis sling. Analysis of surgical material showed that absorbable sutures significantly increased the MRD1 (MD = 1.16; 95% CI [0.60, 1.72]) compared to non-absorbable sutures when used in levator plication; frontalis sling surgeries performed with silicon rods significantly increased the PFH (MD = 0.88; 95% CI [0.29, 1.47]) compared to those performed with Gore-Tex strips, while autogenous fascia lata provided statistically better aesthetic outcome for lid height symmetry and contour. CONCLUSION: Different surgical methods and materials appear to affect different aspects of the congenital ptosis treatment outcome. LEVEL OF EVIDENCE III: 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 .

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PURPOSE: Comparing the surgical and refractive outcomes of congenital ptosis repair by different surgical techniques. METHODS: This longitudinal cohort study reviewed medical records of 101 patients who underwent congenital ptosis repair, from 2006 to 2022 in a single center. Analysis was performed for demographic background, co-morbidities, pre-operative and post-operative ocular examinations and refraction, complications, reoperations, and success rates. RESULTS: Following exclusion criteria, we remained with 80 patients (103 eyes) who underwent either frontalis muscle suspension surgery (FMS) (55 eyes) or levator muscle surgery (LM) (48 eyes). Patients in the FMS group were younger (mean age of 3.1 vs. 6.0 years, p < 0.001) and had worse pre-operative ocular assessments including prevalence of visual axis involvement, chin-up head position, ptosis severity, and levator muscle function (LF) (p < 0.001). Both groups had a 25% rate of reoperation, however while in the LM group reoperation was required solely due to undercorrection, in the FMS group various indications prompted reoperation. Success rate was higher in the FMS group (87.3% vs. 60.4%, p = 0.002). While pre-operative astigmatism was higher in the LM group (p = 0.019), no significant differences were observed post-operatively. Spherical and spherical equivalent changes over time were significant only in the FMS group (p = 0.010 and p = 0.004, respectively). CONCLUSIONS: Within our cohort, a higher success rate of congenital ptosis repair was observed among patients who underwent FMS compared to LM, despite similar reoperation rates. In cases of severe ptosis and moderate LF, LM demonstrated a lower-than-anticipated success rate. Astigmatic changes following ptosis repair were not consistent in either group.

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PURPOSE: To introduce a novel technique for small-incision levator resection in ptosis surgery and evaluate its efficacy in a pilot study among patients affected by congenital or aponeurotic ptosis. METHODS: We prospectively enrolled congenital and aponeurotic ptosis patients if their levator function was not poor (≥5 mm) from June 2021 through October 2022. Surgical technique involved a 1-cm lid crease incision, minimal dissection, and creating a loop passing through the tarsus and levator aponeurosis. Success was defined as postoperative MRD-1 ≥3 mm and inter-eyelid MRD-1 difference ≤1 mm. Eyelid contour quality was scored excellent, good, fair, and poor according to its curvature and symmetry. RESULTS: Sixty-seven eyes (35 congenital and 32 aponeurotic) were included in the study. Mean age was 34±19 years (range, 5-79 years). Mean preoperative levator function and levator resection amount were 9.53 mm and 8.39 mm in the congenital group and 12.34 mm and 4.15 mm in the aponeurotic group, respectively. Mean pre- and postoperative MRD-1 was 1.61 mm and 3.27 mm respectively (P<0.001). The overall success rate was 82.1% (95% C.I: 71.7-89.8%); the result was failure in 12 cases, of which 11 had under-correction. Preoperative MRD-1 was correlated with a success rate (P=0.017). CONCLUSION: The described technique shows non-inferior results to the previously described surgical methods and also it shows very good lid contour outcome and minimal lag. The findings suggest that the double mattress single suture technique can be used in both congenital and aponeurotic ptosis.

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BACKGROUND: To examine the astigmatism characteristics and surgical outcomes in patients with unilateral severe congenital ptosis following frontalis suspension surgery. METHODS: We included 53 congenital ptosis patients who underwent frontalis suspension surgery in Hunan Children's Hospital. Each patient underwent a refractive examination before and after surgery to assess astigmatism. We also evaluated the effects and complications associated with the procedure. RESULTS: Degree of astigmatism in ptotic and fellow eyes was - 1.45 ± 0.59 D and - 0.66 ± 0.51 D before surgery. Ratio of severe astigmatism in ptotic and fellow eyes was 51.3 and 12.8%. The fellow eyes presented with with-the-rule astigmatism (WR; 71.8%) and against-the-rule astigmatism (AR; 20.5%) types, with no cases of oblique astigmatism (OA). Ptotic eyes demonstrated higher frequencies of AR (59.0%) and OA (10.2%) than did fellow eyes. Furthermore, the former showed increased astigmatism, followed by a gradual decrease at the 6-month, before significantly decreasing at the 1-year postoperatively. The ratio of postoperative AR and OA astigmatism cases in ptotic eyes decreased to 35.9 and 7.7% 1 month postoperatively. However, there was a postoperative increase in the WR ratio from 30.8 to 56.4% after 1 month. Kaplan-Meier survival analysis showed a success rate of 81.4% at 6 months and 62.9% at 12 months which was influenced by the following complications: suture reaction, epithelial keratopathy, infection and granuloma, lid lag, and recurrence. CONCLUSION: Monocular congenital ptosis could develop severe astigmatism and higher frequency of AR or OA, early surgery may ameliorate astigmatic amblyopia.

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Marcus Gunn jaw winking (MGJW) is an uncommon entity and is associated with congenital ptosis. It is a neurogenic congenital ptosis, which is also called the Marcus Gunn phenomenon, trigeminal-oculomotor synkinesis, or pterygoid-levator synkinesis. Congenital ptosis can be associated with MGJW syndrome, blepharophimosis syndrome, and monocular elevation deficiency (MED). MED is a condition where there is a unilateral congenital abnormality in the elevation of the eye in abduction and adduction. The MGJW phenomenon, congenital ptosis, and double-elevator palsy may be associated with and represent a congenital misdirection syndrome. Together, it can be challenging, and surgery is recommended in severe cases, depending on the degree of ptosis and jaw winking. We hereby want to bring to light one such case of a 14-year-old female with congenital ptosis, MGJW, and double-elevator palsy and want to highlight how both MGJW and double-elevator palsy are both parts of the same disease spectrum and how such cases can be treated.

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PURPOSE: To compare the surgical outcomes of tarsofrontalis sling surgery using silicon rod versus supramaximal levator resection in patients of unilateral congenital ptosis with poor levator function (≤ 4 mm). MATERIALS AND METHODS: This randomized control study was conducted over a period of 18 months. 44 patients were enrolled in the study group. The study population was randomly divided into two groups (22 in each group). In Group A, congenital ptosis was treated with tarsofrontalis sling surgery using silicon rod and supramaximal levator resection was done in the group B. Follow-up examination was done at postoperative day 1, one week, 1 month, 6 months and 12 months. Surgical outcome measures were change in marginal reflex distance-1 (MRD 1), vertical palpebral fissure height (VPFH) and inter-eyelid difference of margin reflex distance 1 and complications in two groups. RESULTS: The mean age of patients in group A was 7.25 ± 6.10 years ranging from 1-18 years and mean age of patients in group B was 5.64 ± 2.44 years ranging from 3 to 11 years. Good surgical outcome was obtained in 73-77% of both groups. MRD1and VPFH increased significantly after surgery from baseline in both the groups (p value < 0.001). Inter-eyelid MRD 1 difference also improved significantly in both groups following ptosis surgery. CONCLUSION: Both supramaximal levator resection and tarsofrontalis sling surgery are equally effective in cases of unilateral ptosis patients with poor levator function and should be part of the surgical armamentarium for treating congenital ptosis.

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