Congenital eversion of upper eyelids: case report and management

Congenital eversion of the upper eyelids is a rare condition, the exact cause of which remains unknown. It is more frequently associated with Down's syndrome and black babies. If diagnosed early and treated properly, the condition can be managed without surgery. We report a case of congenital bilateral severe upper eyelid eversion in a normal infant, born by vaginal delivery. The case was conservatively managed by lubricants, antibiotics and eyelid patching.     

Surgical management of persistent congenital eversion of the upper eyelids

Most cases of congenital eversion of the upper eyelids resolve spontaneously within two weeks of birth. We present a case of persistent upper-eyelid eversion that did not even after two months of conservative management. A previously undescribed mechanism, consisting of a vertical elongation of the eyelid in its posterior aspect creating a relative skin deficiency, was probably responsible for this eversion. Skin grafts provided rapid resolution with a good cosmetic result.     

Congenital ectropion: three case reports and literature review

To report 3 cases of congenital ectropion because of their rarity and confusing classification. Case 1: JPT, 2 days old, male, negro. Left upper eyelid eversion with chemosis was present, passive to mechanic reduction. Compressive occlusion was done with ectropion regression in 48 hours. Case 2: AJL, 6 years old, female, Caucasian, with Down syndrome. The left eye had hyperemia, lagophthalmos and inferior leucoma. She received horizontal shortening (superior and inferior tarsal strip) and skin grafts, and after 2 months the patient did not return. Case 3: GSD, 4 years old, male, Caucasian, with Down syndrome. His signs and treatment were the same as in case 2. According to Picó's classification the first case is classified as grade II due to eyelid eversion during the passage through the birth canal, more frequent in black people. Cases 2 and 3 represent grade III that is due to eyelid skin alteration, and the association with Down syndrome is observed. Treatment for ectropion grade III is always surgical, as it was done in these cases. We do not agree with Picó's classification, the only one found in medline, because there are no articles confirming the existence of grade I (absent tarsus), grade II should be called congenital upper eyelid eversion, grade IV (microphthalmos and orbital cyst) is a disease of the orbit. Grade III refers to true ectropion, because horizontal enlargement of superior and inferior eyelids (megaloblepharon).     

Floppy eyelid syndrome

PURPOSE OF REVIEW: Although floppy eyelid syndrome causes significant ocular symptoms and morbidity, the condition is often underdiagnosed. This review will highlight diagnostic features of the condition, emphasizing recent advances in the understanding of its pathophysiology. Current therapeutic strategies and surgical techniques are discussed. RECENT FINDINGS: Current concepts regarding the underlying pathophysiology of floppy eyelid syndrome revolve around upregulation of elastin degrading enzymes and mechanical factors. Together, these forces cause instability of the eyelid scaffold, resulting in eyelid malposition and ocular symptoms. Newer surgical treatments aim to preserve tarsus to improve eyelid stability and position. SUMMARY: Floppy eyelid syndrome--an underdiagnosed condition--produces significant ocular morbidity. Symptoms range from occasional redness and irritation to corneal ulcer. Diagnosis is based on ocular signs, including easy or spontaneous eversion of the upper eyelids in conjunction with conjunctivitis and keratitis. The condition, associated with body mass index and obstructive sleep apnea, should be suspected in any obese patient with a chronic red and tearing eye. Treatment consists of supportive measures such as ocular lubrication, eyelid taping or a shield, and surgery to address horizontal laxity and redundant eyelid tissues.     

An unusual case of floppy eyelid syndrome

A 51-year-old obese man with a history of nocturnal upper eyelid eversion secondary to floppy eyelids presented with a Staphylococcus aureus corneal ulcer. No papillary conjunctivitis was present. The corneal ulcer was treated successfully. This case demonstrates the importance of ruling out spontaneous nocturnal lid eversion as a cause for unexplained ocular surface pathology and emphasizes the fact that the floppy eyelid syndrome may present in the absence of classic papillary conjunctivitis.     

A CASE OF CONGENITAL ECTROPION IN DOWN'S SYNDROME

A rare case of primary congenital ectropion of all 4 eyelids in a child with Down's syndrome is reported to emphasise the problems of surgical management and to distinguish the condition from congenital eversion of the eyelids. Congenital ectropion is associated with other eyelid abnormalities and usually requires surgical measures to protect the cornea in contrast to congenital eversion which is characterised by the protrusion of oedematous conjunctiva from everted eyelids. This usually resolves spontaneously with simple supportive measures and no structural or functional eyelid abnormality remains.     

A case of congenital ectropion in Down's syndrome

A rare case of primary congenital ectropion of all 4 eyelids in a child with Down's syndrome is reported to emphasise the problems of surgical management and to distinguish the condition from congenital eversion of the eyelids. Congenital ectropion is associated with other eyelid abnormalities and usually requires surgical measures to protect the cornea in contrast to congenital eversion which is characterised by the protrusion of oedematous conjunctiva from everted eyelids. This usually resolves spontaneously with simple supportive measures and no structural or functional eyelid abnormality remains.     

Eyelid crease approach for chalazion excision

Chronic chalazia that predominantly involve the anterior eyelid lamellae are most easily approached through a transcutaneous incision. We describe the use of an eyelid crease incision for chalazia of the upper lid as an alternative to incision directly over the lesion. The technique has been used successfully in 16 patients with anteriorly situated upper eyelid chalazia. The advantages of this technique include excellent exposure with minimal patient discomfort. There is no need for eyelid eversion or compression with a chalazion clamp. The inflamed tissue can be directly inspected and the controlled excision will yield a superior specimen if biopsy is contemplated.     

Operation of the floppy eyelid. Symptomatic cases require surgical eyelid stabilization

BACKGROUND: Floppy Eyelid is a rare condition causing chronic papillary conjunctivitis and chronic corneal disorders (superficial punctate keratitis, epithelial and stromal ulcers). It is characterized by an extremely enlarged and "floppy" upper eyelid which can be easily everted by slight elevation. Usually obese men are affected who use to sleep face down either on the right or on the left side. Pushing the eyelid against the pillow, the lid is intermittently everted at sleep. This lagophthalmus with rubbing of the exposed eye and lid structures causes all pathologic disorders. Shielding the eye at night may help temporarily. PATIENTS: We performed surgery on 7 men with symptomatic floppy eyelid in the age of 42 to 61 years. The patients had been symptomatic between 1 month and 4 years prior to the definite diagnosis. Follow-up time has been 7 months to 4.5 years. RESULTS: In all cases surgery achieved improvement. CONCLUSIONS: Cases of unclear conjunctival or corneal damage and inflammation should led consider Floppy Eyelid as a possible cause. The typical clinical findings make diagnosis easy. As complete stopping of eyelid-rubbing by changing the patient's sleeping habits is mostly not easily possible, for acute therapy of threatening damages to cornea and conjunctiva a surgical shortening of the lid is necessary which stabilizes the lid in order to avoid nightly spontaneous eversion for a long time.     

Complications of exposed monofilament sutures

Exposed monofilament suture ends caused a variety of symptoms and signs in 18 patients. These included foreign-body sensation, pain, contact lens intolerance, giant papillary conjunctivitis, tarsal ulceration, conjunctival granuloma, corneal infiltrate, and corneal vascularization. These changes followed cataract surgery, corneal transplantation, and pars plana vitrectomy. Diagnoses in these cases were made by careful slit-lamp examination and by eversion of the upper eyelid. In every case, removal of the sutures or trimming the suture ends resulted in the immediate relief of all symptoms with complete resolution of all signs within two months.     

Medial upper eyelid shortening to correct medial eyelid laxity in floppy eyelid syndrome: a new surgical approach

PURPOSE: To describe and present the results of a new surgical technique for patients with floppy eyelid syndrome, based on the medial upper eyelid stretching encountered in this condition. METHODS: A case series of 24 patients with floppy eyelid syndrome who where found to have symptomatic predominately medial upper eyelid laxity was analyzed. The history, clinical features, histopathology, and outcome were reviewed after patients underwent medial upper eyelid shortening with or without upper eyelid skin reduction as the first surgical procedure. RESULTS: Of the 24 patients, 18 were men (75%) with a mean age at referral of 56 years, having ocular discomfort and conjunctival irritation/papillary conjunctivitis as the main complaints at presentation. Obesity was present in 96% of cases, with lower eyelid laxity/ectropion (50%) and upper eyelid eyelash ptosis (29%) in conjunction with the upper eyelid laxity. The affected side was related to sleeping habits or recurrent mechanical eyelid trauma. Histologic studies showed a nonspecific inflammatory cell infiltrate and loss of elastin with loose dermal connective tissue. After surgery, complete relief of ocular symptoms and good functional and cosmetic results were present in all cases after 18 months of follow-up. CONCLUSIONS: This new surgical approach is based on the presence of predominately medial upper laxity in patients with floppy eyelid syndrome. The excision of this stretched area stabilized the upper eyelid in an anatomic fashion, providing a good and stable long-term result. The possible mechanisms involved in the medial upper eyelid stretching are discussed.     

Keratoconus associated with floppy eyelid syndrome.

Floppy eyelid syndrome is a recently described entity, which characteristically involves overweight individuals. The characteristic findings are an upper lid that may be readily everted, tarsal laxity, and diffuse papillary conjunctival changes. The cause of floppy eyelid syndrome is believed to be a mechanical disorder due to the eversion of the lids while sleeping. The cause of keratoconus remains uncertain. There are strong proponents to a mechanical etiology for this disease. The authors report five cases of floppy eyelid syndrome with concomitant keratoconus. One patient with bilateral keratoconus had bilateral symmetric floppy eyelid syndrome. The other four patients had asymmetric keratoconus and floppy eyelid syndrome. In all four patients, the keratoconus was significantly worse in the eye with the more severe case of floppy eyelid syndrome. In addition, these four patients all gave a history of sleeping with their head facing predominantly on the side with the floppy eyelid syndrome and keratoconus. Two patients with keratoconus and floppy eyelid syndrome were able to undergo successful contact lens rehabilitation of their keratoconus after treatment of the floppy eyelid syndrome.     

External ophthalmic findings in multiple endocrine neoplasia type 2B

PURPOSE: To identify external ophthalmic abnormalities in multiple endocrine neoplasia type 2B (MEN2B), which may facilitate early detection and prophylactic thyroidectomy to prevent medullary thyroid carcinoma. METHODS: Three patients with MEN2B were examined by an oculoplastic surgeon and external ophthalmic findings were recorded. RESULTS: All patients had prominent corneal nerves, thickened eyelids, mild ptosis and eversion of the upper eyelids. Two patients displayed eyelid nodules and one exhibited lower lid margin eversion. CONCLUSION: Ptosis and lower lid margin eversion are previously unreported findings in patients with MEN2B. Medullary thyroid carcinoma is the most serious consequence of MEN2B and has a high mortality if untreated. Early diagnosis and prophylactic thyroidectomy may be lifesaving. Gene mutations can be identified but the sporadic tendency of the syndrome emphasizes the importance of early clinical detection. MEN2B is one of a number of systemic malignancies with ophthalmic manifestations. Ophthalmologists should be aware of the external features of this rare but lethal malignancy.     

两种术式治疗重度上睑瘢痕性睑内翻的疗效比较

目的：比较显微镜下Hotz法联合睑缘切开皮瓣转位术和单纯Hotz法治疗重度上睑瘢痕性睑内翻的临床效果。

方法：回顾性分析2017-07/2019-07在我院接受上睑内翻倒睫矫正术的患者60例84眼,试验组32例42眼采用显微镜下Hotz法联合睑缘切开皮瓣转位术,对照组28例42眼采用单纯Hotz法。术后随访观察至12mo, 记录患者主观症状、睑缘位置、睫毛外翻情况及满意度。

结果：试验组单眼平均手术时间长于对照组(40.8±2.57min vs 28.5±2.64min,P<0.01)。术后1、12mo,试验组治愈率分别为100%、95%,对照组治愈率分别为95%、76%,术后12mo试验组治愈率优于对照组(P=0.013)。 术后1mo两组患者满意度无差异(Z=1.1825,P=0.2371),术后12mo试验组患者满意度高于对照组(Z=3.7346,P<0.01)。

结论：对于重度上睑瘢痕性睑内翻,显微镜下Hotz法联合睑缘切开皮瓣转位术虽手术时间长于单纯Hotz法,但其远期疗效优于单纯Hotz法,术后12mo患者满意度较高。     

Giant papillary conjunctivitis following cataract extraction

Out of a series of 600 cataract extractions, 14 patients were found to have giant papillary conjunctivitis (GPC) due to 10-0 nylon sutures. When renewed conjunctival irritation appears weeks or months after surgery, GPC should be suspected and confirmed by eversion of the upper eyelid at the slit lamp. Free edges of protruding corneoscleral nylon sutures should be looked for. Signs and symptoms of GPC disappear within one to four weeks after removal of the offending suture.     

异体硬脑膜额肌悬吊术治疗上睑下垂远期效果

目的观察异体硬脑膜额肌悬吊术治疗重度先天性上睑下垂的远期疗效。方法对28例（49眼）术后患者随访3～5年观察。结果上睑下垂完全矫正或基本矫正，睑缘弧度美观，符合生理，睑裂闭合良好。结论异体硬脑膜额肌悬吊术治疗重度先天性上睑下垂，术后效果良好、且较稳定。     

Trisomy 13: a rare case of congenital tarsal kink

We describe the management of the eyelid anomaly associated with Patau syndrome. Trisomy 13 is the genotype of the syndrome's phenotype. The eyelid anomaly was a tarsal kink, a congenital malformation of the tarsus that causes entropion. A 2-month-old white girl presented with unilateral upper eyelid entropion and central corneal ulceration. To correct this condition, two 6-0 polyglactin sutures were passed through the gray line of the upper and lower eyelids and tied. Correction of the entropion and improvement in the corneal condition were achieved after surgery. No recurrence of the entropion or corneal ulceration was noted after 2 months of follow-up. This simple technique, which corrected the eyelid malposition, providing an excellent cosmetic result without incision of the tarsus, has been previously reported in other cases of tarsal kink but not in a patient with Patau syndrome.     

Ectropion

Ectropion is the most frequent eyelid malposition, characterized by the eversion of the margin and exposure of the conjunctiva and the cornea. It is classified as congenital (primary and secondary) and acquired (involutional, paralytic, mechanical and cicatricial). Congenital ectropion is rare and it is usually associated with other malformations such as euryblepharon, ptosis, epicanthus inversus, and blepharophimosis syndrome. Involutional ectropion is the most frequent form of acquired eyelid eversion and a result of multiple factors. The mechanisms involved in its pathogenesis are discussed in this review. Cicatricial ectropion is caused by the shortening of the anterior lamella of the lid, secondary to congenital malformations, trauma, burns, skin conditions, scarring skin tumors, medications, allergies, blepharoplasty complications, and involutional changes that result in loss of skin elasticity. Mechanical ectropion is caused by eyelid tumors or inflammatory disorders that cause orbicularis spasm. The evaluation of the patient, selection of treatment and surgical techniques are described in detail.     

应用自体硬腭黏膜修复睑板缺损

目的 观察自体硬腭黏膜修复睑板缺损的效果.方法 各种原因所致睑板缺损26例（26眼）.其中上睑10例,下睑14例,单眼上下睑2例.睑板完全缺损10只睑,缺损1/2～2/3者10只睑,缺损1/3～＜1/2者8只睑.取自体硬腭黏膜修剪后代替睑板修复眼睑缺损.结果 术后随访6～36月.16只睑外形及功能完全恢复,4只睑轻度睑内翻,3只睑轻度闭合不全,1只睑闭合不全为3 mm,4只睑轻度睑缘切迹.无严重并发症发生.结论 自体硬腭黏膜修复睑板缺损外形及功能均良好,自体硬腭黏膜是一种很好的睑板替代物.     

三种术式治疗重度先天性上睑下垂的疗效对比

目的:探讨提上睑肌缩短术、额肌瓣悬吊术、阔筋膜悬吊术治疗重度先天性上睑下垂的效果。方法:手术治疗重度先天性上睑下垂患者42例47眼,其中提上睑肌缩短术25例28眼、额肌瓣悬吊术10例12眼、阔筋膜悬吊术7例7眼,比较手术效果,上睑弧度、双重睑成形情况及手术并发症情况。结果:采用提上睑肌缩短术者,术后外观自然美观,兔眼恢复时间短,效果满意;采用额肌瓣悬吊术者术后外形欠自然、兔眼恢复时间长;采用阔筋膜悬吊术者,上睑迟滞和眼睑闭合不全较明显,易发生矫正不足。结论:三种手术方式均能有效治疗先天性上睑下垂。提上睑肌缩短术更符合生理状态,手术效果最好且并发症少。