Prenatal ultrasound detection of collodion membrane in association with an autosomal recessive congenital ichthyosis due to transglutaminase 1 deficiency.

We describe a case of collodion baby diagnosed prenatally by ultrasound. Classic signs (ectropion, flattened nose, and eclabion) were detected on routine ultrasound at 21 weeks of gestation. At birth, the presence of collodion membrane was confirmed and subsequently, the diagnosis of an autosomal recessive congenital ichthyosis due to compound heterozygosity of the TGM1 gene was made.

Lateral canthal reconstruction for the treatment of macroblepharon/diamond eye conformation in dogs.

PURPOSE: To introduce a novel surgical technique for treatment of macroblepharon and diamond eye conformation in dogs. METHODS: Lateral canthal reconstruction was used in dogs with prominent eyelid malformations resulting in ocular surface disease. Lateral canthus was resected and new lateral canthus was created using a two-layer closure. This technique was performed either alone or in combination with additional procedure(s). Additionally, this technique was used to resect lateral canthal dermoid. RESULTS: One hundred and fifty-three eyes of 85 dogs were included in the study. Procedure was done for macroblepharon and/or lateral canthal entropion (149 eyes of 81 dogs) or for lateral canthal dermoid (four eyes of four dogs). Procedure was done either alone (n = 68 eyes) or in combination with additional procedure (n = 85 eyes). Favorable cosmetic outcome and client satisfaction was achieved in all dogs. Good to excellent functional outcome was recorded in all but 6 eyes of 4 dogs, which required additional surgery. Most common complications included slight undercorrection or overcorrection. CONCLUSIONS: Lateral canthal reconstruction is a simple yet effective surgical procedure for macroblepharon and/or lateral canthal entropion. If done early, it prevents development of secondary eyelid malformation ("pagoda defect") in giant breed dogs. If done after severe eyelid malformation has developed, combining this technique with concurrent pagoda resection is recommended to achieve ideal eyelid conformation. None.

Enhancing Lower Eyelid Suspension Outcomes Through Pre-surgical Facial Nerve Reanimation: A Comparative Study.

BACKGROUND: Lower eyelid suspension, a common therapeutic procedure for facial paralysis-induced eyelid retraction, faces challenges due to high recurrence in patients lacking facial muscle function and impedes wider adoption. This research aims to explore the potential effects of restoring orbicularis oculi muscle tension through facial nerve reanimation prior to lower eyelid suspension and to define the indications for lower eyelid suspension. METHODS: The study encompassed 32 individuals with complete facial paralysis, segmented into group A (reanimation group) and group B (non-reanimation group), based on whether the orbicularis oculi muscle's tension was restored through facial nerve reconstruction prior to lower eyelid suspension. Subjective assessments of eyelid closure (the inter-eyelid gap upon gentle closure) and objective methods measures of scleral show (the distance from the pupil's center to the lower eyelid margin, MRD2) were used to provide a comprehensive analysis of long-term effectiveness. RESULTS: The group A exhibited significantly greater long-term improvement in lagophthalmos and lower eyelid ectropion. The alterations in MRD2 measured 2.66 ± 0.27 mm in the group A versus 2.08 ± 0.53 mm in the group B, denoting a statistically significant variance (p < 0.001). Moreover, while the ratio of MRD2 preoperative 6 months postoperative revealed no significant difference between groups, a significant difference emerged in 12 months postoperative (group A: 1.02 ± 0.21; group B: 1.18 ± 0.24; p < 0.05), with the values in group A closer to 1, indicative of enhanced symmetry. CONCLUSIONS: Restoring the tension in the orbicularis oculi muscle through facial nerve reconstruction prior to palmaris longus tendon sling could effectively sustain long-term outcomes of lower eyelid retraction correction and reduce the recurrence rate. LEVEL OF EVIDENCE IV: 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 .

Resolution of Pityriasis rubra pilaris induced ectropion with oral dexamethasone. A case report.

An 82-year-old male presented with generalised Pityriasis rubra pilaris (PRP) managed initially by dermatology team. The patient did not respond to first- and second-line treatment, including oral acitretin, steroid creams, and methotrexate, and developed bilateral cicatricial ectropion, for which he was referred to oculoplastic team for surgical management. A head injury resulting in subacute subdural haematoma, managed with a week course of low dose oral dexamethasone, resulted in the improvement of his skin condition and complete resolution of the cicatricial ectropion within a few weeks. Thus, systemic treatment of PRP with oral dexamethasone may be considered sooner in the treatment of cicatricial ectropion in similar cases.

Recalcitrant postoperative bleeding after ectropion repair in the setting of undiagnosed chronic disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) is characterized by abnormal activation of the coagulation cascade, which leads to simultaneous hypercoagulation and excessive bleeding. While it typically occurs in systemic diseases, such as infection, inflammation, obstetric complications, and malignancy, it can rarely manifest postoperatively. This case report describes a patient who presented with prolonged, refractory bleeding after ectropion repair via a lateral tarsal strip procedure. Due to the inability to control the patient's bleeding with conservative measures followed by surgical exploration and electrocautery, the patient underwent a hematologic work-up. Laboratory studies were consistent with DIC, attributed to his large burden of endovascular stents. He was treated with anticoagulation using apixaban in addition to tranexamic acid to achieve lasting hemostasis. This case highlights the importance of thorough preoperative assessments, even for minor surgical procedures, and systemic workup for atypical postoperative bleeding.

Evaluation of dry eye disease and corneal epithelial mapping in patients with lower eyelid ectropion.

PURPOSE: To evaluate the corneal epithelial mapping, ocular surface parameters and their relationship with each other in lower eyelid ectropion patients according to severity. METHODS: This retrospective study included 48 lower eyelid ectropion patients and 63 healthy individuals as control group. Ocular surface and tear functions were evaluated with ocular surface staining score, tear film break-up time (BUT), non-invasive tear break-up time (NIBUT) and ocular surface disease index (OSDI). Meibography scores and corneal epithelial thickness (CET) mapping were evaluated. The lower eyelid ectropion was classified and divided into subgroups as follows: mild, moderate and severe. RESULTS: There was no significant differences between the groups for age and gender. Compared to controls, CET was significantly thinner at inferior (p = 0.047) and inferior nasal quadrants (p = 0.023) in the ectropion patients. Lower BUT and NIBUT, higher OSDI scores and higher ocular surface staining were observed in the ectropion patients. In the subgroups determined according to the severity of ectropion, ocular surface damage was found to be significantly higher as the severity of ectropion increased (p < 0.05). CONCLUSION: Patients with lower eyelid ectropion had worse ocular surface findings and more ocular complaints. Furthermore, the inferior and inferior nasal CETs were thinner in patients with lower ectropion.

Correction of severe medial ectropion using a novel osseous fixation technique.

PURPOSE: To study the feasibility of osseous medial canthal fixation for severe medial ectropion. METHODS: Retrospective analysis over a period of 3 years identified 10 patients who had undergone the modified medial canthoplasty. Outcome measures were based on the presence of complications and cosmetic/functional results. RESULTS: Post-operative evaluations of all patients were significant for excellent functionality and cosmetic results in the medial canthal area following the novel osseous fixation technique. No complications were reported intra-operatively or post-operatively. CONCLUSIONS: For repair of severe medial ectropion, especially cicatricial and paralytic ectropion, the modified medial "puncture hole" canthoplasty is an effective alternative to traditional repair techniques and does not necessitate the use of anchoring systems such as wiring or microplates.

Ocular involvement in facial nerve paralysis: risk factors for severe visual impairment and ocular surface exposure in 1870 patients.

PURPOSE: To identify the risk factors associated with the development of ocular surface exposure and severe visual impairment (SVI) in patients with facial nerve palsy (FNP). METHODS: Ocular data of all consecutive FNP patients (N = 1870) presenting to an eye-care network over the past 10 years were reviewed. Risk factors associated with SVI (best-corrected visual acuity <20/200) and ocular surface exposure at presentation were evaluated using multivariate analysis and odds ratios (OR). RESULTS: The prevalence of SVI was 15%, and 47% had ocular surface exposure at the first presentation. The presence of corneal scar (28% vs. 10.2%, p < .001; OR 3.05), corneal ulcer (12.9% vs. 2.3%, p < .001; OR 4.67), older age at presentation (p < .001; OR 1.02), >10 mm lagophthalmos (p < .001; OR 8.7), male sex, and duration of FNP (p = .021; OR 1) were independent risk factors for developing SVI. Of the 893 eyes with ocular surface exposure, 75 (3.9%) had a corneal ulcer, and 11 (0.6%) were perforated, with the rest having epithelial defects and punctate keratopathy. Patients with neoplastic etiology (15.9% vs. 5.7%; p < .001; OR 2.39), lagophthalmos (49% vs. 29.7%; p < .001; OR 2.25) and poor Bell's phenomenon (9.4% vs. 4.2%; p = .005; OR 1.8) had twofold risk for developing ocular surface exposure. CONCLUSION: Eyes with lagophthalmos, poor Bell's phenomenon, and FNP of neoplastic etiology have a higher risk of developing ocular surface exposure. Male sex, increasing age, longer duration of FNP, and >10 mm lagophthalmos are associated with SVI in FNP patients; hence, early and timely intervention is necessary.

Ophthalmic findings in patients with autosomal recessive lamellar ichthyosis due to TGM1 mutations in an isolated population.

PURPOSE: To describe the ocular clinical characteristics of a group of Mexican patients with lamellar ichthyosis (LI) arising from TGM1 pathogenic variants. METHODS: Ophthalmological exploration, pedigree analysis and genetic screening were performed in patients with an established clinical diagnosis of lamellar ichthyosis from families located in a small community in the Southeast of Mexico. RESULTS: Nine patients with LI in five families were identified. There were six affected females. All patients (9/9) demonstrated eye lid abnormalities with eight patients showing lid margin abnormalities. Madarosis was present in only three individuals and corneal scarring was documented in two. All nine individuals carried biallelic TGM1 variants, either homozygously or as compound heterozygous. CONCLUSION: Ocular anomalies are common in individuals with TGM1-related LI. The occurrence of a variety of private or rare mutations hampers the identification of a genotype-phenotype correlation for ocular anomalies in this disorder.

Involutional ectropion: etiological factors and therapeutic management.

PURPOSE: Involutional Ectropion is commonly prevalent disorder of eyelid malposition caused by age-related degeneration of the periocular tissues. This study conducted to provide a summary and review of surgical practice for the management of lower eyelid involutional ectropion and enlist various causative factors that explain the pathogenesis. METHODS: The review of literature on risk factors and surgical approaches for involutional lower eyelid ectropion, searched on PubMed from 1980 onwards. RESULT: Multiple factors contribute to horizontal and vertical lower eyelid involutional ectropion. Several surgical practices have been described over the last years to address these factors. Lateral tarsal strip is the most used and effective surgery to treat horizontal laxity. CONCLUSION: Knowledge of various contributing factors and surgical procedures will enable to design the most effective therapeutic management for lower eyelid involutional ectropion. surgical approaches are individualized depending on preoperative clinical evaluation of possible causative factors and concerning the predominant location of the ectropion will result in a high success rate.

ImageJ assessment of double-frequency YAG laser efficacy in treatment of mild lower eyelid ectropion with punctal eversion.

PURPOSE: To describe a simple and minimally invasive technique using double-frequency YAG laser for correction of mild medial lower eyelid ectropion with punctal eversion and assess the efficacy of the technique by measuring the tear film meniscus pre- and post-laser treatment using imageJ software. METHODS: This study included 23 eyes of 19 patients with lower eyelid ectropion with punctal eversion. All patients were treated by double-frequency YAG laser applied to lower medial conjunctiva. Tear film thickness was assessed using imageJ software pre- and post-argon laser treatment. RESULTS: There was a highly significant change detected after argon laser treatment as regarding the mean of the height of tear film which was found to be significantly lower after argon laser treatment compared to before it (81.1 pixels versus 193.1 pixels, respectively) (P < 0.001). CONCLUSION: Double-frequency YAG laser applied to lower medial palpebral conjunctiva is a simple, easy, safe, and effective procedure which can be used as a solo treatment in early cases of punctal eversion with no or mild medial canthal tendon laxity.

[Modern opportunities of lagophthalmos correction]. / Sovremennye vozmozhnosti korrektsii lagoftal'ma.

The main goal in the treatment of lagophthalmos is prevention of serious corneal complications. Based on the results of 2453 surgeries performed in patients with lagophthalmos, a detailed analysis of modern surgical techniques was carried out highlighting their advantages and disadvantages. The article describes in detail the most effective methods of static correction of lagophthalmos, their features and indications, and presents the results of using an original palpebral weight implant.

Tarsoconjunctival-Skin Flap as Another Option in Correcting Ectropion for Oro-Ocular Cleft Reconstruction: Report of 2 Cases.

Facial clefts are rare facial anomalies. Among them, oro-ocular cleft remains a challenging malformation due to ectropion of the inferior palpebra which can constitute an emergency when the patient's globe is exposed leading to exposure keratitis and blindness. Here we report surgical procedures to correct lower eyelid ectropion using tarsoconjunctival-skin flap performed on 2 cases. In conclusion, tarsoconjunctival-skin flap can be provided to effectively correct lower eyelid ectropion and is a better option when nose correction is not involved simultaneously.

Lower eyelid malposition following repair of complex orbitofacial trauma.

PURPOSE: To compare the incidence of lower eyelid malposition following repair of isolated orbital floor fractures with that of complex orbitofacial fractures (defined as multi-wall fractures or prior orbital fracture repairs requiring revision) by oculofacial plastic surgeons via a transconjunctival or swinging eyelid approach. METHODS: Retrospective review of 175 patients who underwent surgical repair of orbital fractures at our institution. The primary outcomes were the occurrence of lower eyelid malposition (ectropion, entropion, and eyelid retraction) and the need for subsequent surgical correction. RESULTS: Of 95 patients with isolated orbital floor fractures, 4 developed eyelid malposition (4.2%), 1 of which required surgical repair (1.1%). Of 80 patients with complex orbitofacial fractures (48 multi-wall fractures, 32 secondary revisions), 10 had pre-operative eyelid malposition and were excluded from further analysis. Fourteen of the remaining 70 patients developed postoperative eyelid malposition (20%), 3 of which required surgical repair (4.3%). The difference in the occurrence of eyelid malposition between groups was statistically significant (p = .001), but the difference in rates of those requiring subsequent repair was not (p = .182). There was no statistically significant difference in the occurrence of eyelid malposition when considering other surgical factors including lateral canthotomy, conjunctival closure, implant material, or anterior rim screws. CONCLUSIONS: The incidence of lower eyelid malposition following orbital fracture repair via a fornix-based approach was significantly higher for the repair of complex orbitofacial fractures than for isolated floor fractures. However, very few patients in either group required surgical repair for eyelid malposition. Surgical factors including implant material did not affect outcomes.

Consensus recommendations for the use of retinoids in ichthyosis and other disorders of cornification in children and adolescents.

Topical and systemic retinoids have long been used in the treatment of ichthyoses and other disorders of cornification. Due to the need for long-term use of retinoids for these disorders, often beginning in childhood, numerous clinical concerns must be considered. Systemic retinoids have known side effects involving bone and eye. Additionally, potential psychiatric and cardiovascular effects need to be considered. Contraceptive concerns, as well as the additive cardiovascular and bone effects of systemic retinoid use with hormonal contraception must also be deliberated for patients of childbearing potential. The Pediatric Dermatology Research Alliance (PeDRA) Use of Retinoids in Ichthyosis Work Group was formed to address these issues and to establish best practices regarding the use of retinoids in ichthyoses based on available evidence and expert opinion.

Calcium-sensing receptor promotes calcium oxalate crystal adhesion and renal injury in Wistar rats by promoting ROS production and subsequent regulation of PS ectropion, OPN, KIM-1, and ERK expression.

OBJECTIVES: To explore the mechanism of calcium-sensing receptors (CaSRs) during the development of nephrolithiasis. MATERIALS AND METHODS: Wistar rats were treated with ethylene glycol to induce calcium oxalate crystallization, and gadolinium chloride (GdCl3, an agonist of CaSR) and NPS 2390 (an antagonist of CaSR) were added. Oxidative stress (OS) and calcium oxalate crystals in the kidney were observed. CaSR expression and the expression of extracellular signal-regulated protein kinase (ERK), OPN, and KIM-1 were determined by western blotting. In addition, renal tubular epithelial cells were isolated from the kidney to observe phosphatidylserine (PS) ectropion using flow cytometric analysis. Various biochemical parameters were assessed in serum and urine at the end of the experiment. RESULTS: Calcium oxalate increased OS, crystal adhesion, PS ectropion, and the expression of CaSR and ERK, OPN, and KIM-1 in vivo. In addition, lower levels of urine citrate as well as increased serum creatinine and urea levels were observed after treatment with calcium oxalate (p < .05). Compared with calcium oxalate treatment alone, the above deleterious changes were further significantly confirmed by GdCl3 but were reversed by NPS-2390. However, urine calcium excretion was decreased after ethylene glycol treatment but was significantly reduced by NPS 2390 and increased by GdCl3 (p < .05). CONCLUSIONS: The results suggest that CaSR might play significant roles in the induction of nephrolithiasis in rats by regulating reactive oxygen species (ROS) and PS ectropion and the composition of urine, OPN, KIM-1, and ERK expression.

The impact of cryotherapy for symptomatic cervical ectropion on female sexual function and quality of life.

In rare cases, cervical ectropion causes symptoms such as abundant leucorrhoea, postcoital bleeding, recurrent cervicitis, pelvic pain, and dyspareunia. Cryotherapy is a commonly used treatment for symptomatic cervical ectropion. We assessed the impact of cryotherapy on sexual function and quality of life among patients with symptomatic cervical ectropion. In this prospective observational study, 73 patients were assessed before and six months after cryotherapy treatment using the Female Sexual Function Index (FSFI) and Short Form-12 Health Survey questionnaires. The double-freeze cryotherapy procedure was performed using a cryotherapy unit, and liquid nitrogen was used as a refrigerant. The mean physical and mental quality of life scores were significantly improved after treatment. With the exception of the pain domain, the overall and domain FSFI scores exhibited no significant differences before and after cryotherapy. The sexual pain domain scores were significantly increased after treatment. There was a statistically significant improvement in vaginal discharge, pelvic pain, and postcoital bleeding symptoms after the cryotherapy. We concluded that cryotherapy is an effective and feasible treatment for symptomatic cervical ectropion. Although cryotherapy results in improved quality of life scores, it has no significant impact on female sexual function.Impact statementWhat is already known on this subject? Cryotherapy is the most preferred treatment option for symptomatic cervical ectropion. Its feasibility and effectiveness with respect to symptom relief have been observed in previous studies. No study has evaluated quality of life and sexual function after cryotherapy among patients with symptomatic cervical ectropion.What do the results of this study add? Although the patients' quality of life scores were significantly improved after treatment, no significant improvement was observed in overall and domain sexual function scores, with the exception of the pain domain. The sexual pain domain scores were significantly improved after cryotherapy.What are the implications of these findings for clinical practice and/or further research? Patients should not expect better sexual function after cryotherapy. Comparative studies should seek to identify the ideal treatment option, which would result in both symptom relief and better sexual function.

Successful correction of ichthyosis-related ectropion by autografts.

Ectropion is a rather frequent complication of ichthyosis with negative functional and esthetic impact. Lid surgery can provide significant improvement. We report on a series of four ichthyosis patients with ongoing bilateral eye problems despite intense medical treatment (mean age 27.8 ± 14.1 years). All patients suffered from lagophthalmos. Two of the patients had only lower lid ectropion. In two of the patients' ectropion was forming on the upper lid in addition to the lower lid when closing the eye. In three of four patients, ectropion was repaired by skin grafts from the supraclavicular region. In a child with lower and upper lid ectropion prepuce was used for repair surgery. At the end of the follow-up period of about 23.0 ± 12.7 months, none of the patients revealed lagophthalmos or corneal exposure. No intraoperative or postoperative complications have been observed in our patients. Ichthyosis patients with ectropion resistant to medical treatment, benefit from surgical treatment with full layer autografts. In boys, prepuce can be successfully used as autograft.

Essential skin shrinkage: cicatricial ectropion, a histopathologic evaluation and clinical analysis.

Purpose: To describe common risk factors in patients with Essential Skin Shrinkage (ESS) and identify corresponding histopathologic changes in lower eyelids.Methods: A case-control study was performed after an Internal Review Board approval was obtained. Consecutive patients who underwent surgical repair for ectropion with ESS of the lower eyelid were enrolled along with 10 control patients. Informed consent was obtained on all patients. Fitzpatrick skin type, history of sun exposure and non-melanoma skin cancer was obtained along with relevant physical exam findings. Skin samples obtained during surgical repair were evaluated by light microscopy for the extent of dermal actinic change. Statistical analysis was performed.Results: Sixteen study subjects and 10 control patients were enrolled. Subjects were found to be predominantly male, older than controls (p = 0.0011) and have Fitzpatrick skin type (FST) I or II while controls had type I, II or III (p = 0.0221). Hours of sun exposure reported by subjects ranged from 23,165 to greater than 125,000 h, versus 1,459 to 46,890 h in controls (p = 0.0002). Nine of 16 (56%) subjects had a history of skin cancer compared to only 3/10 controls (30%) (p = 0.2475). Histopathologic evaluation using the Fritschi scale for dermal actinic damage identified an average grade of 3.6 for subjects and 2.4 for controls (p = 0.0095).Conclusions: ESS is predominantly seen in male individuals with FST I or II and a history of extensive sun exposure. Histopathologic evaluation shows moderate to severe actinic damage. These individuals frequently have concomitant non-melanoma skin cancer.

Modified cheek advancement flap for medial lower eyelid, nasal sidewall and infraorbital cheek reconstruction: a case series.

Aim: To describe and evaluate surgical outcomes of the Modified Cheek Advancement Flap (MCAF) to reconstruct defects involving medial lower eyelid, nasal sidewall and infraorbital cheek following Mohs micrographic surgery for basal cell carcinoma (BCC).Materials and Methods: All patients who underwent MCAF between 2012 and 2018 under the care of a single surgeon (LCA) were identified. The technique described in this report is a significant modification of the traditional cheek advancement flap. The MCAF was undertaken without subciliary or infraorbital incisions, less extensive dissection and use of flap advancement rather than rotation. A retrospective chart review was completed using a pro-forma which included risk factors for flap failure, early and late complications and additional reconstructive procedures. Patient satisfaction was rated using a five-point Likert-type scale.Results: 42 patients underwent the MCAF. Mean follow up was 28.4 months (±19.9 months). Early complications were found in 11.9% of the patients and resolved completely within the first four post-operative weeks. There were no long term complications secondary to the reconstruction. No lower eyelid ectropion was noted. 78.6% of the patients cited they were 'extremely satisfied' with the final cosmetic outcome.Conclusion: The MCAF proves a valuable and safe option in periocular reconstructive surgery with excellent cosmetic results and no post-operative ectropion. The necessity for eyelid tightening as part of the reconstructive process should encourage oculoplastic surgeons to use the MCAF when reconstructing defects involving medial lower eyelid, infraorbital cheek and nasal sidewall.