Congenital eyelid imbrication syndrome: A rare occurrence in Pakistan.

Congenital eyelid imbrication syndrome (CEIS) is a rare condition presenting at birth and is characterised by overriding of the upper lid on the lower lid. It is due to longer upper lid, than the lower lid. Overriding leads to spontaneous eversion of the upper lids. In our patient, examination revealed canthal tendon laxity and hyperaemia of the tarsal conjunctiva. All the rest of the structures in the eyeball and adnexa were normal. Spontaneous eversion occurred in two weeks as the upper lid grew with time. No treatment was required.

Meibography-Guided Electroepilation for Congenital Distichiasis: A Novel Surgical Technique.

PURPOSE: Congenital distichiasis is managed either by ablation, using laser, cryotherapy, or electroepilation, or by surgical excision with mucous membrane grafting. Ablative procedures are usually blind as the exact depth of distichiatic eyelashes is unknown. The described surgical technique utilizes meibography for imaging the root and depth of distichiatic eyelashes that aided in performing electroepilation. METHODS: Six patients (n = 24 eyelids; mean age 15.5 ± 12.2 years) underwent infrared meibography (Oculus Keratograph 5 M) and noninvasive tear breakup time prospectively. Eyelashes were electroepilated using a premarked needle inserted at a depth based on meibography findings in 4 patients. Surgical success was defined as no distichiatic eyelash regrowth and functional success was defined as the resolution of symptoms at a minimum of 3 months of follow-up. RESULTS: All 6 patients had all 4 eyelids involved to varying degrees, with a total of 230 distichiatic eyelashes. The median number of distichiatic eyelashes was 9 in the upper eyelids and 4.5 in the lower eyelids. Meibography revealed visible distichiatic eyelash roots in 70% of eyelashes in the upper eyelid and 87.8% in the lower eyelid, respectively. The median eyelash root depth was 2.7 mm (mean 2.9 mm, range 1.8-5.4 mm). The mean noninvasive tear breakup time was 12.2 seconds despite absent or rudimentary meibomian gland segments seen on meibography. The anatomical success was 75% (12/16 eyelids), and functional success was 87.5% (7/8 eyes) at a median follow-up of 5.5 months. CONCLUSION: Preoperative infrared meibography in eyelids with congenital distichiasis helps estimate the eyelash depth and can be used to guide eyelash ablation procedures.

Congenital Eyelid Imbrication and Floppy Eyelid Syndromes Complicated by Eversion due to Prostaglandin E Infusion in a Patient With Trisomy 21 and Tetralogy of Fallot.

Congenital eyelid imbrication syndrome is a rare eyelid finding where a long upper lid overlaps the lower lid when the eyes are closed. To date, congenital eyelid imbrication syndrome has been described in the literature less than 10 times. We present a case of congenital eyelid imbrication syndrome in a patient with trisomy 21 and tetralogy of Fallot on a prostaglandin E infusion to maintain a patent ductus arteriosus prior to definitive heart surgery. While on the infusion, the patient developed peripheral edema and flushing due to vasodilation. This coincided with eyelid swelling, conjunctival chemosis, and eversion of the eyelids. Upon cessation of the prostaglandin E1 infusion, his eyelid eversion resolved.

Orbicularis muscle fixation downward for the correction of congenital epiblepharon in Chinese patients.

BACKGROUND: Congenital epiblepharon (CE) is a congenital eyelid deformity unique to young individuals of Asian descendants, and it might cause a sequence of other complications such as corneal damage and ocular foreign body sensation. Surgery is the most effective approach to treat this condition. Here, this study evaluated the effectiveness and safety of the orbicularis muscle fixation downward (OMFD) procedure for correcting CE. METHODS: This study enrolled 29 patients who were diagnosed with CE. These patients experienced significant ocular irritation leading to epiphora and corneal injury and, therefore, they underwent the OMFD procedure that was performed by the same surgeon. The cases were divided into 3 grades according to postoperative outcomes. RESULTS: The OMFD procedure was performed on 55 lower lids of the 29 patients. The average age of the patients was 9.6 years. The mean follow-up duration was 17 months. No perioperative complications were observed, and successful surgical outcomes were achieved in all 29 patients. No postoperative necrosis, infection, or corneal epithelial injury occurred. None of the patients exhibited significant scarring. Three patients showed residual 1-3 eyelashes touching the nasal corneal or bulbar conjunctiva at the inner canthus during the last postoperative examination. These patients did not undergo further surgery as there were no obvious irritation symptoms or corneal damage. CONCLUSIONS: The fixation of the pretarsal orbicularis muscle is a simple and effective modification of the Hotz procedure to correct CE. The key aspect of this procedure is the suturing of the pretarsal orbicularis muscle and the lower eyelid retractors.

Successful Ocular Surface Reconstruction in Complete Ankyloblepharon With the Simple Oral Mucosal Epithelial Transplantation Technique: A Case Report.

PURPOSE: To report an outcome of a patient with complete ankyloblepharon successfully managed with simple oral mucosal epithelial transplantation (SOMET). METHODS: A 55-year-old woman presented with complete adhesion of both lids to the ocular surface as a complication from Stevens-Johnson syndrome. We performed 2-staged reconstructive surgeries: the first stage was to perform ankyloblepharon lysis and surface reconstruction with a mucosal graft on the palpebral area and an amniotic membrane on the bulbar area, and the second stage was to reconstruct the bulbar area with a transplantation of small pieces of oral mucosa (SOMET technique). Postoperatively, the patient was evaluated for ocular surface stability, recurrent symblepharon, in vivo confocal microscopy, and impression cytology with immunofluorescence staining. RESULTS: Complete epithelialization of cornea-like epithelium was observed within 6 weeks after SOMET was performed. The ocular surface was stable over 1 year. Both fornices remained deep. In vivo confocal microscopy showed cornea-like epithelium mixed with conjunctival epithelium, as confirmed with immunofluorescence staining, which revealed cytokeratin 3, cytokeratin 7, and cytokeratin 12 positivity. CONCLUSIONS: SOMET is a simple modified technique using minimal oral mucosal tissue to regenerate epithelialization for complicated ocular surface reconstruction such as a complete ankyloblepharon repair. Although there was evidence of conjunctival invasion, stable ocular surface and deep fornices can be achieved for further visual rehabilitative procedure.

Effect of Congenital Lower Eyelid Epiblepharon Surgery on Asymmetric Margin Reflex Distance 1.

Objectives: To investigate the characteristics of margin reflex distance 1 (MRD1) asymmetry in congenital lower eyelid epiblepharon and its resolution after surgical correction of epiblepharon.Methods: Among patients who underwent lower eyelid epiblepharon surgery from November 2015 to September 2017, patients with a preoperative MRD1 difference of more than 1.0 mm between the two eyes according to medical photographs were defined as having MRD1 asymmetry. A postoperative MRD1 difference of less than 1.0 mm between the two eyes was regarded as MRD1 asymmetry resolution. The preoperative MRD1 difference was compared between subgroups with resolved or sustained MRD1 asymmetry. Astigmatism and amblyopia were also assessed.Results: Among 432 patients, MRD1 asymmetry was observed in 24 patients (5.6%). MRD1 was always lower in the side with more extensive epiblepharon. At 6 months after surgery, the mean MRD1 difference between the two eyes was significantly decreased (1.8 ± 0.7 mm to 0.5 ± 0.8 mm, p < .001, paired t-test) and MRD1 asymmetry resolution occurred in 19 patients (79%). In the resolved MRD1 asymmetry group, the preoperative MRD1 difference was 2.0 mm or less and was significantly smaller than that in the sustained MRD1 asymmetry group (p = .010, Mann-Whitney U test). Six patients had preoperative aniso-astigmatism ≥ 1.50 D. Unilateral amblyopia presented in nine patients (38%) and improved within 1 year postoperatively.Conclusions: MRD1 asymmetry can be resolved after epiblepharon surgery, especially when the preoperative MRD1 difference is 2.0 mm or less. Unilateral amblyopia was frequent, but the treatment outcome was good.

Analysis of corneal real astigmatism and high order aberration changes that cause visual disturbances after lower eyelid epiblepharon repair surgery.

This retrospective, cross-sectional study investigated changes in corneal low and high order aberrations (LOAs and HOAs) after lower eyelid epiblepharon repair surgery. In total, 108 eyes from 54 patients were evaluated. Wavefront analyses for calibrated LOAs and HOAs were performed using a Galilei G4 Dual Scheimpflug Analyzer before surgery and during the first and second follow-ups (f/u), adjusting for several risk factors. Flat keratometry (K) and axis values decreased significantly from baseline at the first f/u. At the second f/u, mean K and axis values decreased. Coma and trefoil increased from baseline at the first f/u and normalized by the second f/u. Spherical aberrations (SA) only decreased at the second f/u. After correction for risk factors, cylinder, coma, trefoil, and SA significantly increased at the first f/u; axis and flat K values decreased. At the second f/u, cylinder increased while axis and mean K values significantly decreased. Epiblepharon repair surgery may result in a shift from 'with-the-rule' to 'against-the-rule' axis change. Flat K, coma, and trefoil may be affected by mechanical force changes immediately post-surgery, while mean K values and SA may also change with corneal state changes including corneal erosion healing after the second f/u during the postoperative period.

Thyroid Eye Disease-Related Epiblepharon: A Comparative Case Study.

PURPOSE: This study describes the clinical features and management of epiblepharon as a manifestation of thyroid eye disease (TED). In addition, we compare the frequency and age in Asian and non-Asian patients, and discuss pathophysiologic implications. DESIGN: Retrospective case-control study. METHODS: This is a single-center retrospective review that identified 172 adult patients (age 19 to 83) with TED that were consecutively evaluated by 1 author (T.J.M.) between December 2015 and July 2018. Diagnosis of TED and epiblepharon was based upon clinical assessment as documented in the medical record. RESULTS: In a cohort of 172 patients (mean age 52; 138 female), 3 patients with acquired epiblepharon were identified, all of whom were Asian. The proportion of affected Asian patients (3/of 17, 17.6%) was significantly higher than that of non-Asian patients (0/155, P < 0.001). Patients with epiblepharon were also significantly younger than those without epiblepharon, 29.7 ±â€Š2.1 versus 48.7 ±â€Š13 years of age (P = 0.026). All 3 patients underwent surgical correction with lateral canthoplasty and anterior lamellar pretarsal fixation with successful outcomes. CONCLUSIONS: Lower eyelid epiblepharon may occur in TED. In our clinic-based population, this finding was significantly more frequent in Asian patients and in younger patients. Relieving horizontal tension in conjunction with anterior lamella pretarsal fixation is an effective method of correcting TED-associated epiblepharon.

Treatment of symptomatic epiblepharon with Botulinum toxin type A in patients under 2years of age. / Experiencia del uso de la toxina botulínica tipo A para el tratamiento sintomático del epiblefaron en pacientes menores de 2 años.

INTRODUCTION: Epiblepharon is a congenital eyelid malposition due to a horizontal skin fold and a redundant orbicular muscle, resulting in the inward positioning of the eyelashes. OBJECTIVE: Personal experience is presented of the non-surgical correction of symptomatic epiblepharon using a pretarsal injection of 5 IU of botulinum toxin type A (BoNT-A) into the orbicular muscle. MATERIAL AND METHODS: Patients with epiblepharon younger than 2 year were included in the study. A review was made of their clinical charts and the symptoms and signs of epiblepharon were evaluated before and after treatment with BoNT-A. RESULTS: A total of 40 patients were included (28 girls [70%]). The mean age at treatment was 11 months (range, 4-24 months). A total of 76 eyelids were treated with BoNT-A. A statistically significant improvement in symptoms, lash-corneal touch, and punctate corneal epitheliopathy were reported after the treatment with 5IU BoNT-A. The mean final follow-up was 25.5 weeks (range, 4-92 months). CONCLUSIONS: The present study provides evidence that a pretarsal BoNT-A injection is an effective and safe treatment for the correction of symptomatic epiblepharon in patients younger than 2 years of age.

Modified Hotz procedure with CO2 laser ablation of the orbicularis oculi muscle for epiblepharon.

OBJECTIVE: To introduce the use of a CO2 laser for ablation of the orbicularis oculi muscle (OOM) in a modified Hotz procedure for correction of epiblepharon and to report its clinical outcome. METHODS: A retrospective consecutive study was conducted from August 2014 to January 2018. Patients who had undergone surgical correction for epiblepharon with the modified Hotz procedure and a CO2 laser ablation of OOM were included. The procedure includes a transverse subciliary incision with removal of a very small amount of skin using CO2 laser ablation. The ablation was carried out continuously on parts of the pretarsal portion and parts of the preseptal portion of the OOM inferior to the lower edge of the incised skin. Finally, the incised skin was closed and secured to the tarsus. The patients were followed for direct inspection of the wound, the direction of the lashes, and the status of the cornea. RESULTS: One hundred eyelids from 50 patients were enrolled. The average age was 6.4 years. The mean postoperative follow-up period was 14.3 months. In 90 eyelids (90.0%), the eyelashes were successfully outwardly everted. Undercorrections were noted in 10 eyelids (10.0%). To date, the cosmetic outcome was satisfactory in all cases without complications such as wound dehiscence, ectropion, and eyelid retraction. CONCLUSIONS: A CO2 laser is a safe and effective alternative to the scalpel in the modified Hotz procedure to correct epiblepharon. Its advantages include limited skin excision, minimal bleeding during surgery, short operation time, and a satisfactory clinical outcome.

Reporting outcomes of hyaluronic gel filler as a treatment for epiblepharon.

PURPOSE: Epiblepharon occurs when an extra skin fold overlaps on the eyelid margin with the isolated form mainly seen in children of east Asian origin. If symptomatic and the use of conservative measures such as lubricants have failed, surgery is usually indicated. This traditionally involves everting sutures or combined skin excision, such as a modified Hotz procedure. However, a temporizing non-surgical alternative to a skin removal procedure, especially if the natural history is for improvement as the child grows older, would be ideal. METHODS: This is a retrospective single-centre case review of epiblepharon cases treated with hyaluronic acid (HA; Restylane, Galderma UK) treated in the past 5 years by a single surgeon (RM). Institutional review board approval was obtained. Success is defined as improvement or stabilization of the class and/or keratopathy score of the epiblepharon. RESULTS: Five patients were identified with epiblepharon between 2012 and 2017 who had hyaluronic acid filler to 8 eyelids. Six eyelids had improvement, 1 remained stable and 1 was worse equating to an 87% success rate; however, 2 opted for reversal using hyaluronidase due to aesthetic reasons. Two went on to have further surgery as they partially responded to filler treatment. CONCLUSION: This study provides further proof of concept that HA is a simple non-surgical and reversible option that may avoid the need for surgery for epiblepharon in selected cases. It may even be considered in older children or adolescents with the caveat that fullness may require hyaluronidase to dissolve.

P63-related disorders: Dermatological characteristics in 22 patients.

In P63-related ectodermal dysplasias (ED), the clinical characteristics focus on extra-cutaneous manifestations. The dermatological phenotype remains incompletely characterized. We report the dermatological features of 22 patients carrying a TP63 mutation. Erosions, erythroderma and pigmentary anomalies are characteristics of P63-related ED. Our data suggest that patients might be classified into two major P63-related disorders: AEC and EEC. RHS and ADULT represent mild AEC and EEC forms, respectively.

Reconstruction strategy in isolated complete Cryptophthalmos: a case series.

BACKGROUND: The present study sought to introduce clinical characteristics and stepwise surgical strategies of isolated complete cryptophthalmos, a rare, congenital ocular anomaly. CASE PRESENTATION: Retrospective, noncomparative, clinical study. Six patients with isolated complete cryptophthalmos were diagnosed at the Beijing Tongren Hospital between 2010 to 2018. The presentation age of patients ranged from 1 month to 68 years. This study includes two males and four females, and involvement was noted to be bilateral in two cases and unilateral in four cases. According to orbital CT scan and ocular CDI results, two patients were combined with ocular cyst. Reconstruction surgeries were performed in three patients, involving the eyeball enucleation, creation of fornix, eyelid reconstruction with skin flaps/amniotic membrane, and implantation of prosthesis. Besides, implantation of hydroxyapatite was performed in one pediatric patient to promote orbit development. Good outcomes in terms of cosmetic satisfaction were achieved in all patients during follow-up. CONCLUSIONS: Surgical intervention could only improve the cosmetic appearance in isolated complete cryptophthalmos. The surgical strategies may be planned to use three-stage approaches described in this study. Meanwhile, orbital development must be taken into consideration in pediatric cases.

Sex-specific difference in age distribution of congenital lower eyelid epiblepharon in a Japanese population.

PURPOSE: To examine the sex-specific differences in age distribution of Japanese patients with congenital lower eyelid epiblepharon. STUDY DESIGN: A retrospective, observational study. METHODS: A total of 291 patients (87 men, 204 women) who underwent modified Hotz for correction of congenital lower eyelid epiblepharon were included in this study. Data on sex, age at surgical intervention, and affected side were collected. Patients' age was classified according to the orbital growth pattern (0-3 years, 4-6 years, 7-12 years, 13-15 years, and ≥ 16 years). The sex-specific difference in age distribution was analysed using Pearson's chi-squared test. RESULTS: Among male patients, 75 (86.2%) underwent surgery during 4-12 years of age. Only 2 (2.3%) boys had surgery at 13-15 years and 1 had it while he was ≥ 16 years. Among female patients, 11 (5.4%) underwent surgery at 13-15 years and 59 (28.9%) at ≥ 16 years. The age distribution was significantly different between sexes (P < 0.001). CONCLUSION: Among patients aged ≥13 years, there were more women compared to men. This implies that congenital lower eyelid epiblepharon is more persistent in females and may reflect slower and less orbital growth from 13 years of age and onwards in females. The results suggest that as long as there are no severe corneal complications and no risk for amblyopia, conservative management may be a good option for males since spontaneous resolution is likely to occur. However, for females aged 13 years or more, the threshold for surgical intervention should be lower.

Epiblepharon correction in Korean children based on the epicanthal pathology.

PURPOSE: This study aims to investigate the surgical outcomes of a novel epiblepharon correction method based on the pathologic features of epicanthal tissue. The correlation between the pathology and clinical presentations was also studied. METHODS: This retrospective study included 72 eyelids of 36 children with epiblepharon and epicanthal folds. From July 2013 to March 2016, epicanthal tension-releasing procedure of subdermal fibrinolysis with orbicularis oculi ring myotomy were performed in 36 children. Photographs before and after operation were analyzed with Image J program. A clinical severity of the epiblepharon was scored based on the lower eyelid skin fold height (mild to severe). Subdermal fibrosis index was defined as the portion of fibrosis in low power field (× 40) and eyelid contour was assessed with custom software (MATLAB). Eighty eyelids without eyelid pathology were analyzed to establish the normal eyelid contour. The epicanthal tissues were classified into three categories: mild, moderate, and severe fibrosis. RESULTS: The mean age of the patients was 5.5 years. Intercanthal distance (ICD) ratio was defined as Inner ICD (IICD)/outer ICD (OICD). The IICD/OICD was 0.41 in the severe epiblepharon group that is significantly longer compared to that of the mild epiblepharon group (0.38). Subdermal fibrosis index was higher in severe epiblepharon group. Eyelid contour was normalized and the cilia touch was resolved after the epicanthal tension-releasing procedure. CONCLUSION: Epicanthal tension-releasing procedure with orbicularis oculi ring myotomy achieved favorable surgical outcomes. Clinical correlations with pathologic findings and satisfactory results of the procedure suggest that medial epicanthal fibrosis is the main pathologic feature causing epiblepharon and epicanthal folds.

Lower eyelid tension balance reconstruction: A new procedure for the repair of congenital epiblepharon with epicanthus.

BACKGROUND: Congenital epiblepharon with epicanthus (CEE) is a common eyelid malposition in Asian children, which generally involves the lower eyelid. The induced cilial entropion may cause constant ocular irritation that requires surgical repair. The purpose of this study is to report the outcomes and surgical details of a novel procedure, lower eyelid tension balance reconstruction (LETBR), for the correction of CEE. METHODS: Patients diagnosed with CEE underwent LETBR, which consists of modified half Z epicanthoplasty, and fixation between marginal orbicularis oculi muscle and lower eyelid retractor was reviewed retrospectively. The outcomes were classified as 'good' with no cilia-ocular surface contact, 'fair' with 5 or fewer asymptomatic cilia-ocular surface points of contact and 'poor' with most of the eyelashes remaining in contact with the eyeball. The surgical outcomes (good, fair or poor), recurrence and complications were evaluated. RESULTS: One hundred and forty-nine eyelids of 78 patients (43 females and 35 males; mean age 6.6 ±â€¯2.4 years, range 4-17 years) were evaluated in this study. The mean follow-up time was 14 months (range 9-24 months). At the last follow-up time, 144 of 149 eyelids were judged as 'good' (96.6%), the other 5 eyelids were judged as 'fair' (3.4%) and no eyelid was assessed with a 'poor' outcome. There were no significant complications or complaints about scarring on eyelids from patients or their parents. CONCLUSION: LETBR is effective, safe and stable for patients with CEE.