Subciliary Rotating Sutures Combined With Modified Hotz Procedure for Correction of Congenital Lower Eyelid Entropion.

PURPOSE: Standard techniques for the treatment of congenital lower eyelid entropion may not yield suitable outcomes or may result in overcorrection if disinsertion of the lower eyelid retractors is not the primary cause. Herein, we propose and evaluate a technique using subciliary rotating sutures combined with a modified Hotz procedure for repair of lower eyelid congenital entropion that addresses these concerns. METHODS: A retrospective chart review was conducted of all patients who underwent lower eyelid congenital entropion repair by a single surgeon using subciliary rotating sutures combined with a modified Hotz procedure between 2016 and 2020. Study variables included patient demographics, follow-up period, postoperative complications, operative success, and recurrence. RESULTS: Twelve patients (19 eyelids) met the study inclusion criteria. The mean patient age was 7.1 ± 6.1 years (range, 0.2-22 years). Nine of the patients were female (75%) and 3 were male (25%). The distribution of eyelids was 8 right (42%) and 11 left (58%). The mean follow-up time was 19.5 ± 15 (range 2.5-45) months. There were two eyelids (11%) that had entropion recurrence after initial repair in patients with concomitant compound disease processes. Repeated repair resulted in success with no recurrence at last follow-up. Overall, the described entropion repair technique was successful and without recurrence in 17 eyelids (89%). There were no cases of ectropion, lid retraction, or other complications. CONCLUSIONS: Subciliary rotating sutures combined with a modified Hotz procedure are effective for correction of congenital lower eyelid entropion. As the technique does not manipulate the posterior layer of the lower eyelid retractors, it may be useful for when retractor reinsertion does not yield adequate improvement and may also reduce the risk of eyelid retraction and overcorrection in particular cases.

A genome-wide significant association on chromosome 15 for congenital entropion in Swiss White Alpine sheep.

Entropion is a known congenital disorder in sheep presumed to be heritable but no causative genetic variant has been reported. Affected lambs show a variable inward rolling of the lower eyelids leading to blindness in severe cases. In Switzerland, the Swiss White Alpine (SWA) breed showed a significantly higher prevalence for entropion than other breeds. A GWAS using 150 SWA sheep (90 affected lambs and 60 controls), based on 600k SNP data, revealed a genome-wide significant signal on chromosome 15. The 0.2 Mb associated region contains functional candidate genes, SMTNL1 and CTNND1. Pathogenic variants in human CTNND1 cause blepharocheilodontic syndrome 2, a rare disorder including eyelid anomalies, and SMTNL1 regulates contraction and relaxation of skeletal and smooth muscle. WGS of a single entropion-affected lamb revealed two private missense variants in SMTNL1 and CTNND1. Subsequent genotyping of both variants in 231 phenotyped SWA sheep was performed. The SMTNL1 variant p.(Asp452Asn) affects an evolutionary conserved residue within an important domain and represents a rare allele, which occurred also in controls. The p.(Glu943Lys) variant in CTNND1 represents a common variant unlikely to cause entropion as the mutant allele occurred more frequently in non-affected sheep. Therefore, we propose that these protein-changing variants are unlikely to explain the phenotype. Additionally, WGS of three further disconcordant pairs of full siblings was carried out but revealed no obvious causative variant. Finally, we conclude that entropion represents a more complex disease caused by different non-coding regulatory variants.

Efficacy of Inverted "V" Release Surgery in the Correction of a Special Type of Epicanthus.

OBJECTIVE: To evaluate the efficacy of a new correction method (inverted "V" release surgery) for epicanthus as a cause of congenital entropion/trichiasis in the lower eyelid. METHODS: From May 2014 to May 2017, 60 eyes of 30 patients (13 male and 17 female patients, with the age of 6.85 ± 4.52 years underwent inverted "V" release surgery to correct epicanthus. The incision in the epicanthus and entropion was continuous. Lower eyelid entropion/trichiasis was corrected by a two-layered suture. We separately assessed the width of the palpebral fissure, exposed ratio of the lacrimal caruncle, eyelash direction, and incision scar at one and 6 months after surgery. RESULTS: All patients were followed up for 6-18 months. At 6 months after surgery, the palpebral fissure width of all patients was extended by 3-4 mm. The ratio of the exposed lacrimal caruncle was enhanced from 0.24 ± 0.22 to 0.75 ± 0.08. All patients presented with natural inner canthal angles, and their eyelashes clearly extended outward, with a 100% total effective rate. The inner canthal incision scars in 83% of patients were invisible 6 months after surgery. CONCLUSION: Based on our experience, both functionality and aesthetics can be obtained by using inverted "V" release surgery to correct a special type of epicanthus that aggravates congenital entropion/trichiasis in the lower eyelid. Thus, this technique is suitable for clinical application. 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 .

A Simple New Technique for Treatment of Tarsal Kink Syndrome.

Congenital tarsal kink syndrome is a rare type of upper eyelid entropion associated with keratitis due to trichiasis. The authors describe a new technique for treatment of congenital horizontal tarsal kink syndrome by means of absorbable everting sutures via a posterior approach. A neonate was referred to eye clinic with right eye redness and corneal opacity since birth. Clinical examination revealed inversion of the eyelid margin with a horizontal kink in the tarsal plate and corneal ulcer. Surgical treatment resulted in successful correction of upper eyelid malpositioning, rapid resolution of the corneal ulcer with excellent anatomical outcome, visual development and cosmesis, with no evidence of recurrence after 6 years. Absorbable everting sutures via a posterior approach is a simple, minimally invasive, and effective surgical technique for treatment of congenital horizontal tarsal kink syndrome.

Surgical correction of congenital entropion in related Boer goat kids using a combination Hotz-Celsus and lateral eyelid wedge resection procedure.

Five related Boer goat kids (≤4 months of age) were presented to the University of Missouri, Veterinary Teaching Hospital (MU-VMTH) with epiphora and blepharospasm of several weeks duration and commencing prior to 1 month of age in all animals. Clinical examination confirmed euryblepharon and entropion bilaterally in two females and one male and unilaterally in two female kids. Deep stromal corneal ulceration was present in two eyes, and corneal granulation tissue and fibrosis were present in half (5/10) the affected eyes. A combination Hotz-Celsus and lateral eyelid wedge resection procedure was performed on all affected eyelids. Recheck examinations and long-term follow-up confirmed resolution of the entropion, preservation of normal eyelid conformation, and restoration of ocular comfort. Pedigree analysis ruled out sex-linked and autosomal dominant inheritance patterns; a specific mode of inheritance could not be determined. The Boer goat breed may be at increased risk for the development of entropion. This cases series represents the first report of entropion in the caprine species.

Congenital entropion: outcome of posterior layer advancement of lower eyelid retractors and histological study of orbicularis oculi muscle hypertrophy.

PURPOSE: To examine surgical outcomes of the posterior layer advancement of the lower eyelid retractors (LER) in patients with congenital entropion and to ascertain whether the pretarsal orbicularis oculi muscle (OOM) was hypertrophic or not. MATERIALS AND METHODS: This retrospective study included three Japanese patients with congenital entropion who underwent posterior layer advancement of the LER. We reviewed the surgical outcomes and postoperative complications, such as lower eyelid ectropion and retraction for each patient. We also measured the diameters of the pretarsal OOM fibers in these patients and compared them with those measured in the previously reported 67 eyelids of 41 Japanese patients with congenital epiblepharon. RESULTS: Congenital entropion was successfully corrected in all three patients without recurrence during 12-months of follow-up. No patient exhibited lower eyelid ectropion or lower eyelid retraction. The mean diameter of the pretarsal OOM fibers was 21.9 µm (range, 20.5-23.7 µm), which was not significantly different from that of the congenital epiblepharon (25.3 µm; range, 18.1-34.7 µm; p = 0.272, Mann-Whitney U test). CONCLUSIONS: The surgical outcomes in the present study suggest that posterior layer advancement of the LER is a useful surgical option for treatment of congenital entropion. No histological evidence of pretarsal OOM hypertrophy was shown in congenital entropion, which demonstrated that debulking of the pretarsal OOM may not be significant for correction of this entity.

Anatomical characteristics and surgical treatment of bilateral congenital upper eyelid entropion in an infant with neonatal progeroid syndrome.

Neonatal progeroid syndrome (NPS) is a rare, autosomal recessive disorder characterized by an aged face, wrinkled skin, decreased subcutaneous fat, and neonatal teeth. The authors describe herein a 2-month-old infant with NPS who underwent eyelid surgery for bilateral upper eyelid entropion and stromal keratitis. The preoperative examination revealed thin, narrow, and soft tarsal plates. The eyelids were large and floppy, and there was no preaponeurotic fat tissue. The entropion was successfully treated by the levator muscle advancing onto the tarsus, anterior lamellar repositioning, horizontal eyelid shortening, and canthal resuspension. Upper eyelid entropion may occur relatively frequently in infants with NPS, and the special anatomical features of the eyelid should be considered while performing entropion surgery.

Bilateral congenital entropion with cutis laxa.

Cutis laxa is a rare connective tissue disorder characterized by redundant and pendulous skin due to a defect in the elastic fiber network. Two cases of entropion associated with cutis laxa have been reported, although entropion was due to elongation of the anterior lamella or horizontal lid laxity. Thorough systemic and ophthalmic evaluations were performed, as well as chart review for the perinatal period. Surgical correction of entropion through posterior tarsotomy was done. An infant boy with dysmorphic features and furrowing of the skin of the entire body without hyperelasticity, which is typical for cutis laxa, presented with bilateral congenital entropion. We report here for the first time a different etiology of congenital entropion with cutis laxa: the eyelashes were abnormally directed due to the unusual location of their roots, which were embedded within the tarsus. Moreover, this is the only case of cutis laxa with congenital entropion involving both upper and lower eyelids. Congenital entropion can be associated with cutis laxa. Although elongation of the anterior lamella and horizontal lid laxity predispose to such an entropion, abnormal location of the roots of the eyelashes might be encountered and marginal eyelid rotation surgery is indicated.

The surgical treatment of congenital tarsal kink syndrome associated with corneal ulcer.

The authors present a case of unilateral eyelid congenital tarsal kink associated with corneal ulcer. The tarsal kink was corrected by a horizontal tarsal incision, performed half the thickness of the tarsus along its entire horizontal extent. Terminal levator aponeurosis fibers were reattached to the tarsal incision line to create sufficient everting force for correction of the entropion. The tarsal kink disappeared and eyelid positions remained stable during a follow-up period of 2 years.

Congenital entropion.

Entropion is a malposition of the eyelid causing an abnormal inversion of the eyelid margin. This results in symptomatic contact of the eyelashes with the surface of the globe, resulting in corneal irritation. Symptoms primarily arise from irritation of the ocular surface. Corneal abrasions and scarring can occur. Although common in many canine breeds, it is rare in the pediatric congenital form and rarely reported in the literature. Success in treatment depends on diagnosing the appropriate cause and selecting a surgical procedure that adequately addresses the underlying abnormality. The authors describe a pediatric patient with congenital entropion and review the nature of the problem and its treatment options.

Congenital horizontal tarsal kink with microphthalmos corrected by intermarginal suture tarsorrhaphy.

Congenital horizontal tarsal kink is extremely rare. Only one case of associated microphthalmos has been reported. Various surgical techniques have been described with successful results. Few reported cases have been managed with intermarginal suture tarsorrhaphy. The current case is probably the third patient with severe tarsal kink to be managed with this simple technique.

Cilial entropion: surgical outcome with a new modification of the Hotz procedure.

PURPOSE: To report the surgical outcome with a new modification of the Hotz procedure for cilial entropion repair. DESIGN: Retrospective case series. PARTICIPANTS: Forty-nine lower eyelids of 29 patients (22 female, 7 male; average age 8.4 years, range 2-27 years). METHODS: The study included all patients diagnosed with cilial entropion and significant ocular irritation causing epiphora, photophobia, and ocular discharge, with or without keratitis. Surgery is based on dividing the anterior layer of the lower eyelid retractors from the anterior tarsal plate surface, definite identification of the inferior tarsal plate border, and reinforcement of the skin to the tarsal plate and lower eyelid retractors. Surgical outcome was defined as "good" or "fair" when there was no contact between the eyelashes and the globe or when less than 5 asymptomatic eyelashes had direct contact with the globe, respectively. A "poor" outcome was defined as either the majority of eyelashes remained in contact with the globe or persistence of irritation/keratitis in the presence of residual in-turned eyelashes. A successful outcome ("good" or "fair") required no additional surgical intervention. MAIN OUTCOME MEASURES: Surgical outcome ("good," "fair," or "poor"), postoperative complications, recurrence. RESULTS: During a mean follow-up period of 27.4 months (range, 3-50 months), 40 eyelids (82%) were judged postoperatively as "good," 7 eyelids (14%) were judged as "fair," and 2 eyelids (4%) were judged as "poor." The 2 eyelids with a poor outcome were reoperated successfully using the same technique. No postoperative complications were recorded. There were no cases of recurrence, except the 2 eyelids with a "poor" outcome, during the follow-up period. CONCLUSIONS: Our modification of the Hotz procedure is based on identifying and dividing the anterior layer of the lower eyelid retractors and reinforcing the skin to the lower border of the inferior tarsal plate and lower eyelid retractors. This is a useful and predictive technique for cilial entropion repair and results in a high success rate.

Congenital horizontal tarsal kink: a single-center experience with 6 cases.

PURPOSE: To describe the clinical features, surgical management, and outcome of congenital horizontal tarsal kink. DESIGN: Single-center retrospective interventional case series. PARTICIPANTS: Six patients with congenital horizontal tarsal kink. METHODS: Medical records of all patients with congenital horizontal tarsal kink of the upper eyelid managed at a tertiary care referral center were retrospectively reviewed. The referral diagnosis, presenting clinical features, associated ocular abnormalities, and outcome of entropion surgery were analyzed. Surgical treatment consisted of transconjunctival horizontal tarsotomy with marginal rotation. MAIN OUTCOME MEASURES: Evaluation of clinical presenting features and surgical correction of entropion. RESULTS: Six patients were diagnosed with congenital horizontal tarsal kink. Five of 6 (83%) patients were males. The referral diagnosis was congenital entropion in 2 (33%) patients. Corneal opacity or infiltrate was the commonest associated presenting symptom, seen in 5 of 6 (83%) patients. Absence of upper eyelid crease as compared with contralateral eye was noted in all 6 (100%) patients. The tarsal kink was severe with an acute angle (defined as inability to visualize the inverted upper eyelid margin) in 4 (67%) patients that presented within 8 weeks of birth. The tarsal kink was mild with an obtuse angle (easy visualization of upper eyelid margin) in 2 (33%) patients that presented late, at 3 and 5 years of age. Transconjunctival horizontal tarsotomy with marginal rotation resulted in permanent correction of entropion (mean follow-up, 43.3 weeks; range, 16-89 weeks) in all patients. Visual acuity remained subnormal in 5 of 6 (83%) patients. CONCLUSION: The diagnosis of congenital horizontal tarsal kink was suspected only in one third of patients at referral. Associated corneal scar or infiltrate was seen in 83% of cases, and absent eyelid crease in all cases. Congenital horizontal tarsal kink was severe in patients who presented early (within 8 weeks of birth), and mild in patients who presented late. An absent upper eyelid crease, corneal opacity at birth, and nonvisibility of upper eyelid margin are important diagnostic clues to identify this rare disorder. Transconjunctival horizontal tarsotomy with marginal rotation corrected the entropion in all patients. Visual acuity remained subnormal in 83% of patients owing to corneal opacity.

Eyelid malposition: lower lid entropion and ectropion.

Correcting entropion and ectropion successfully requires knowledge of the eyelid problems, because understanding of these abnormalities is a key to planning a successful surgical procedure. Entropion is a condition in which the eyelid margin turns inwards against the globe. It is divided into following categories: congenital and acquired, which may be involutional or cicatricial. Ectropion is a malposition in which the lid falls away or is pulled away from its normal apposition to the globe. The condition is classified as congenital and acquired, which is divided into following categories: involutional, cicatricial, paralytic, and mechanical. Therefore, there are some common anatomic changes for both entropion and ectropion as well as specific changes that are unique to each eyelid malposition. Typically, instability of the eyelid is caused by either horizontal laxity or disinsertion or attenuation of the lower eyelid retractors to the inferior tarsal border, so surgical procedures should be directed at correcting the horizontal and vertical instability of the lid. Classification, etiology, underlying anatomic changes in the lid, principles of surgical treatment of entropion and ectropion are reviewed in this article.

Congenitally enlarged extraocular muscles: can congenital thyroid eye disease exist in a euthyroid infant?

A 2-month-old boy presented with hypotropia, eyelid retraction, and proptosis of the left eye. CT and ultrasound demonstrated enlarged extraocular muscles. Both the infant and mother were euthyroid. The patient underwent inferior rectus recession, lower eyelid retractor disinsertion, and entropion repair. Biopsy of the inferior rectus and oblique muscles was normal. The clinical presentation and workup appear to be most consistent with thyroid eye disease, which, to our knowledge, would be the first reported case of euthyroid congenital thyroid eye disease with a euthyroid mother.

A simple correction for congenital tarsal kink associated with distichiasis.

Congenital tarsal kink is a rare condition. The folded edge of the upper tarsus, or the inturned lashes, may traumatize the cornea causing ulceration. We describe a case of unilateral upper eyelid horizontal tarsal kink associated with distichiasis and congenital corneal ulceration. Several different surgical treatment options have been previously alluded to in the literature including lamellar tarsoplasty, full-thickness eyelid fracture, and rotation and excision of the kink with tarsal reapposition. Repair by means of eyelid everting sutures via an anterior lamellar approach is presented as a novel technique to correct this congenital anomaly.

Correction of congenital entropion of the lower eyelid: incisional versus rotational surgery.

PURPOSE: When, at birth, the eyelid margin is rolled inward against the globe, the condition is referred to as congenital entropion. Upper eyelid involvement is commonly associated with a tarsal abnormality, while lower eyelid entropion is often associated with epiblepharon. Entropion does not resolve spontaneously, and may cause corneal pathology if untreated. The purpose of this study is to compare the two common techniques for the correction of congenital entropion. METHODS: The authors performed a pilot study of 24 consecutive patients with lower bilateral congenital entropion to compare the results of incisional versus rotational surgery. RESULTS: The rotational procedure was carried out in 14 patients; incisional surgery was performed in 10 patients. Twenty-one patients had good functional and cosmetic results. There were only three case of relapse after 3, 4, and 3 months. CONCLUSIONS: The authors consider both techniques satisfactory, but the procedure of choice, considering the age of the patients and previous studies, remains rotational sutures because of its simplicity, quickness, and low risk of complication.