Mucous membrane graft for cicatricial ectropion in lamellar ichthyosis: an approach revisited

A 26-year-old woman with congenital lamellar ichthyosis presented with bilateral cicatricial ectropion of the upper and lower eyelids. There was severe exposure keratopathy with corneal perforation in the left eye. The generalized skin involvement necessitated the use of an alternative to correct the cicatricial ectropion. Mucous membrane graft harvested from the oral cavity was used to correct the cicatricial ectropion of upper and lower eyelid of both eyes. Six months after treatment, the mucous membrane graft was keratinized and a significant reduction in lagophthalmos was seen. Mucous membrane graft offers a promising alternative to correct cicatricial ectropion in lamellar ichthyosis.     

Combined medical and surgical management for cicatricial ectropion in lamellar ichthyosis: A report of three cases

Ichthyosis is a rare inherited skin disorder characterized by abnormal keratinization of the epidermis. Cicatricial ectropion is the most common ophthalmic feature of congenital ichthyosis. Progressive subepithelial cicatrization and abnormal cornification of eyelid skin cause progressive ectropion in both eyelids, leading to lagophthalmos and corneal exposure. Surgical correction of cicatricial ectropion in these cases is challenging with unsatisfactory results. Proper processing of the donor and recipient site with lubricants and topical retinoids before surgery makes grafting easier and its survival better. We present three cases of lamellar ichthyosis with cicatricial ectropion managed with combined preoperative topical therapy followed by surgery. All patients had extremely good surgical outcomes, with none of them requiring repeat surgery.     

Congenital ichthyosis in a Libyan child with ophthalmic manifestations

This report presents a Libyan child with congenital ichthyosis of the recessive lamellar variety complicated by severe ectropion of both upper and lower eyelids. Over a period of 12 months the degree of ectropion diminished and the anterior segments of the eyes were preserved using regular lubricating drops, vitamin A ointment and intermittent topical antibiotic therapy. Despite the pronounced initial upper eyelid ectropion no surgery was required to relieve the ectropion or prevent corneal exposure.     

Surgical treatment of cicatricial ectropion in lamellar ichthyosis

BACKGROUND. Lamellar ichthyosis is a skin disorder that is frequently associated with cicatricial ectropion and lagophthalmos. The authors present the surgical management of cicatricial ectropion in a case with lamellar ichthyosis. PATIENT AND METHODS. A 2-year-old male presented with bilateral cicatricial ectropion of the upper and lower eyelids. He had lagophthalmos and corneal punctuate staining. His skin was totally involved with the disease process except his prepuce. The patient was circumcised, and after the correction of horizontal eyelid laxity, the penile skin graft was sutured into the defects in all four eyelids. RESULTS. 18 months following surgery the patient had no lagophthalmos or corneal exposure, but had slight ectropion at the temporal side of the left lower eyelid. He had no complications from the circumcision. CONCLUSION. Prepuce is an alternative donor tissue in male patients with no other available donor site. To our knowledge, this is the second report of cicatricial ectropion corrected with a penile skin graft.     

Surgical treatment of cicatricial ectropion in lamellar ichthyosis

BACKGROUND . Lamellar ichthyosis is a skin disorder that is frequently associated with cicatricial ectropion and lagophthalmos. The authors present the surgical management of cicatricial ectropion in a case with lamellar ichthyosis. PATIENT AND METHODS . A 2-year-old male presented with bilateral cicatricial ectropion of the upper and lower eyelids. He had lagophthalmos and corneal punctuate staining. His skin was totally involved with the disease process except his prepuce. The patient was circumcised, and after the correction of horizontal eyelid laxity, the penile skin graft was sutured into the defects in all four eyelids. RESULTS . 18 months following surgery the patient had no lagophthalmos or corneal exposure, but had slight ectropion at the temporal side of the left lower eyelid. He had no complications from the circumcision. CONCLUSION . Prepuce is an alternative donor tissue in male patients with no other available donor site. To our knowledge, this is the second report of cicatricial ectropion corrected with a penile skin graft.     

Angeborenes Ektropium bei Ichthyosis congenita mitis und gravis

BACKGROUND: Congenital ichthyosis is a generalized hyperkeratinization of the skin at birth. Depending on clinical aspects and severity, three forms of congenital ichthyosis have been defined: mitis, tarda, and gravis. Desquamation of the parchment-like hyperkeratinized skin begins shortly after birth and may require several weeks to complete. Skin alterations in the eyelid cause shortening of the anterior lamella, subsequently resulting in ectropion. This affects the upper eyelid more often than the lower and can lead to complications such as chronic palpebral or bulbar conjunctivitis and keratinization or exposure keratopathy. Here we present two case reports illustrating the course of ichthyosis congenita mitis and gravis. PATIENTS AND METHODS: Patient 1 (ichthyosis congenita mitis): a male baby prematurely born at 34+2 weeks of gestation was delivered by cesarean section. The entire body was covered by a parchment-like hyperkeratinized skin. Both eyes showed ectropion of the upper and the lower eyelid, which was more obvious with enforced lid closure. Frequent application of external ointment and spontaneous desquamation led to resolution of the ectropion. Patient 2 (ichthyosis congenita gravis): a male baby prematurely born at 35+4 weeks of gestation was delivered by cesarean section. At birth the child showed the signs of a collodion baby with ectropion of all four eyelids in combination with a characteristic "fish mouth" and rudimentary external ears. The child died on the 14th day of life of septicaemia. CONCLUSION: In mild forms of congenital ichthyosis surgical treatment of eyelid ectropion is not required. In more severe cases a skin graft may become necessary. Various although limited sources of graft material which are discussed can be considered.     

Eyelid abnormalities in lamellar ichthyoses

PURPOSE: To describe eyelid abnormalities in lamellar ichthyoses (LI). DESIGN: Retrospective observational case series. PARTICIPANTS: Eight patients with classic LI and two patients with congenital ichthyosiform erythroderma. METHODS: Results of eyelid and corneal examinations of 10 patients with LI were reviewed and analyzed with emphasis on the relationship between eyelid ectropion and corneal damage. RESULTS: All patients presented with cicatricial lagophthalmos. Of the eight patients with classic LI, five had ectropion of the four eyelids, one had only lower ectropion, and two had no degree of ectropion. Two patients with congenital ichthyosiform erythroderma had distinct eyelid abnormalities, including madarosis and eyelash retraction. Loss of vision caused by corneal damage was found in three patients with classic LI. Of these three patients, two did not have upper eyelid ectropion. CONCLUSIONS: Severe corneal damage can occur in LI even if there is no upper or lower eyelid ectropion.     

Surgical correction of entropion and excess upper eyelid skin in congenital cutis laxa: a case report

Congenital cutis laxa is a rare generalized inherited elastosis, characterized by the appearance of premature aging and skin laxity with mild to severe systemic anomalies. Ocular manifestations include excess skin in the eyelids, ptosis and lower lid ectropion. Of the hyperelasticity syndromes - Ehlers Danlos, Pseudoxanthoma elasticum and cutis laxa - only cutis laxa has normal skin wound healing. The diagnosis must therefore be established before surgical options for treatment are considered. We report an unusual case of a 5-month-old male child with cutis laxa who presented with upper lid entropion secondary to severe redundant upper eyelid skin. An anterior lamellar repositioning procedure successfully corrected the lid margin malposition with complete relief of symptoms.     

Surgical correction of entropion and excess upper eyelid skin in congenital cutis laxa: a case report

Congenital cutis laxa is a rare generalized inherited elastosis, characterized by the appearance of premature aging and skin laxity with mild to severe systemic anomalies. Ocular manifestations include excess skin in the eyelids, ptosis and lower lid ectropion. Of the hyperelasticity syndromes – Ehlers Danlos, Pseudoxanthoma elasticum and cutis laxa – only cutis laxa has normal skin wound healing. The diagnosis must therefore be established before surgical options for treatment are considered. We report an unusual case of a 5-month-old male child with cutis laxa who presented with upper lid entropion secondary to severe redundant upper eyelid skin. An anterior lamellar repositioning procedure successfully corrected the lid margin malposition with complete relief of symptoms.     

Role of the lower lid retractors in involutional ectropion repair

AIM. To outline the role of the lower lid retractors in correction of involutional ectropion. METHODS. Eight eyelids with a tarsal ectropion were included in the study. Clinical clues to help identify weakness of the lower lid retractors were documented. A transconjunctival lower lid retractor reattachment with concommitant correction of horizontal lid laxity and lamellar dissociation was performed. RESULTS. Stable eyelid position was obtained in 7 of the 8 cases. One case had a lateral ectropion due to a wound dehiscence. CONCLUSIONS. This small study helps better define the clinical presentations of retractor weakness and provides evidence of a systematic approach in correcting involutional ectropion.     

Spontaneous bilateral corneal perforation in a patient with ichthyosis

We present an 8-month-old female patient with bilateral spontaneous corneal perforation and lamellar ichthyosis. On examination, the skin of the body and face of the patient was completely dry and scaly. Cicatricial ectropion and descemetocele with small perforations were seen. Double-layered amniotic membrane transplantation on the cornea with eyelid construction was performed on both eyes. At the 6-month follow-up, lower eyelid minimal ectropion formation and inferior corneal leukoma were seen bilaterally. In ichthyosis patients, the cornea should be monitored closely due to the risk of severe visual loss and corneal perforation.     

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.     

Eyelid retractor surgery as an adjunct to cicatricial ectropion repair

Purpose : To evaluate the effectiveness of eyelid retractor repair in cicatricial ectropion of the lower eyelid. Methods : The study design was a prospective case series. One hundred and twenty eight eyelids were operated on in 100 consecutive patients with cicatricial ectropion. All patients underwent lower eyelid retractor repair via a conjunctival approach combined with skin replacement to the anterior lamella with or without a horizontal lid tightening procedure. When only medial ectropion was present, a medial‐based transpositional skin flap was used to repair the anterior lamella (26 eyelids). The remaining eyelids with ectropion involving all or most of the eyelid underwent upper‐to‐lower eyelid lateral‐based transpositional skin flap repair (92 eyelids), or full thickness free skin grafting (10 eyelids). Horizontal lid tightening was performed by lateral canthoplasty in 123 eyelids. Results : Relief of cicatricial ectropion symptoms was reported in 90% of patients overall. A normal punctum position was achieved in 70% of eyelids, overall, and was highest (88%) with a medial‐based transpositional skin flap. Conclusions : Eyelid retractor repair combined with skin replacement and horizontal lid shortening is an effective procedure for cicatricial ectropion.     

Corneal topography in involutional ectropion of the lower eyelid: preoperative and postoperative evaluation

PURPOSE: The shape and position of the eyelids affect corneal topography. This study evaluated preoperative and postoperative corneal topography in involutional ectropion of the lower eyelid. METHODS: Eighteen patients with unilateral involutional lower eyelid ectropion underwent ophthalmic examinations and corneal topography before surgical correction and at the 6-month postoperative interval. Corneal topographies were evaluated with the Holladay Diagnostic Summary package. The fellow eyes served as controls. Parameters evaluated included the regularity of astigmatism (RA), steep refractive power (SRP), flat refractive power (FRP), and total astigmatism (TA). RESULTS: Preoperatively, RA was found significantly decreased in the eyes with ectropion compared with the fellow eyes, whereas differences in other parameters were statistically nonsignificant. Postoperatively, RA was significantly increased, whereas SRP was significantly reduced. The percentage of eyes with with-the-rule astigmatism (WTRA) was increased postoperatively, although astigmatic axis changes were not systematic. CONCLUSIONS: Postoperative topographic changes may be related to either restoration of symmetry in the upper and lower lid apposition on the cornea or to rearrangement of the tear film. Further research will be required to assess whether corneal topographic findings could be used as an index of the severity of eyelid laxity and to evaluate the effects of topographic changes on corneal and total optical aberrations.     

Use of topical cutaneous N-acetylcysteine in the treatment of major bilateral ectropion in an infant with lamellar ichthyosis

PURPOSE: To report for the first time bilateral ectropion treatment in an infant with severe lamellar ichthyosis associating N-acetylcysteine applied directly to the skin and oral acitretin. METHODS: An 8-week-old male child with major bilateral ectropion due to lamellar ichthyosis was given treatment associating oral acitretin (Soriatane) and topical N-acetylcysteine. Though the precorneal tear film quality could be maintained, after 1 month of initial treatment with acitretin only, bilateral upper eyelid ectropion remained threatening for the child's cornea. The adjunction of topical N-acetylcysteine enabled a complete regression of ectropion. No complementary surgery was needed and the eyelids remained well positioned. CONCLUSION: Topical N-acetylcysteine has been proved to have an antiproliferative effect on keratinocytes in vitro and in vivo. It may be useful in the treatment of major forms of ectropion in children with lamellar ichthyosis. Its association with conventional acitretin treatment may prevent unnecessary surgery.     

Cicatricial ectropion: repair with myocutaneous flaps and canthopexy

BACKGROUND: To evaluate the effectiveness of myocutaneous upper eyelid flaps combined with canthopexy to treat cicatricial lower eyelid ectropion. METHODS: A prospective non-comparative case series undertaken in a private practice setting. Consecutive patients with moderate lower eyelid cicatricial ectropion and upper eyelid dermatochalasis underwent transfer of a bipedicle or monopedicle flap from the upper eyelid combined with canthopexy. The main outcome measures included the occurrence of complications, eyelid position and cosmesis. RESULTS: Sixty-two consecutive cases of cicatricial ectropion repair using myocutaneous flaps and canthopexy. After a mean follow up of 20 months, 58 (93.5%) of the cases had the lower lid punctum facing posterosuperior into the tear lake, showed lid globe apposition and satisfactory eyelid position. There was mild recurrence of cicatricial ectropion in four patients (6.5%). There were no cases of graft failure or granuloma formation. CONCLUSION: The use of a myocutaneous flap from the upper eyelid combined with a canthopexy suspension suture for repair of cicatricial ectropion may offer good eyelid position and function. This technique has the advantage of avoiding full thickness blepharotomy and was associated with a low incidence of early recurrence.     

全身麻醉下额肌瓣悬吊治疗小儿单眼重度先天性上睑下垂

目的:观察全身麻醉下额肌瓣悬吊术治疗小儿单眼重度先天性上睑下垂的临床效果和特点。方法:对23例小儿单眼重度先天性上睑下垂行全身麻醉下额肌瓣悬吊术,男13例,女10例,2.5～7(平均4.6)岁,患眼提上睑肌力2～4(平均3.1)mm;术前确定健眼平视前方时上睑缘的位置(在上提患眼睑缘至与对侧眼等高时)。术中根据全身麻醉下眼位略上转的状态及术前健眼上睑缘的位置,将患眼睑缘上提至上角巩缘以下1～3mm。结果:随访1wk～1a,20例(87%)双眼睑缘完全或基本等高(双眼上睑缘高度差在1mm内),3例(13%)欠矫,双眼睑缘高相差约2～3mm。所有病例外观术后均较术前有明显改善或改善,均无严重的睑缘畸形、睑外翻和暴露性角膜炎等并发症,但有6例患儿术后外侧睑缘稍低,与对侧眼略不对称。结论:全身麻醉下额肌瓣悬吊术治疗小儿单眼重度先天性上睑下垂关键在于术前确定健眼上睑缘的位置,并根据术中实际眼位的变化确定上睑缘上提的量。     

Repair of cicatricial ectropion in an infant with harlequin ichthyosis using engineered human skin

PURPOSE: To report the use of an Apligraf (Organogenesis, Inc., Canton, Massachusetts, USA) human skin equivalent for repair of cicatricial ectropion in a patient with harlequin ichthyosis. DESIGN: Interventional case report. METHODS: A 6-week-old male child with harlequin ichthyosis and severe bilateral upper eyelid cicatricial ectropion underwent repair with Apligraf grafts. RESULTS: After the initial repair with Apligraf grafts, recurrent bilateral upper eyelid ectropion developed, requiring repeat Apligraf grafting at age 61 days. After the second graft, the eyelids remained well positioned until the child's sudden death from respiratory failure at age 6 months. CONCLUSION: Apligraf human skin equivalent facilitated the repair of cicatricial ectropion in a child with harlequin ichthyosis.     

Surgical management of deep chemical burns of the eyelids

Chemical burns of the eyelids are common, and this may lead to ocular damage. A direct insult of the eyes that result in permanent damage, is rare in facial burns. The majority of the chemical burns of eyelids are partial-thickness that heal spontaneously in 1 week. Whereas, 10 percent are full-thickness burns that require release of contractures and grafts. Wound contracture can cause ectropion of the eyelid, resulting in exposure keratitis, conjunctivitis, corneal ulcers, perforation, and even blindness. At our departments, thirteen patients with 28 chemical burns of eyelids of third-degree, were reviewed. The eyelids had burns wounds with granulation and necrotic tissue. All patients had severe cicatrical ectropion. The eyelids were released with incisions running along the eyelid margin, down to the orbicularis muscle, including the distal part of the levator palpebrae superioris muscle, when necessary. To cover the resulting defects, we use generous full-thickness skin grafts, if available, for both the upper and lower eyelids. Rarely has a tarsorrhaphy been required, and properly constructed dressing provides satisfactory eyelid margin immobilization and conjunctival hygiene. Eighteen full-thickness grafts in 10 patients are reported 8 to 12 weeks after grafting. In seven eyelids, 3 patients developed ectropion and required reconstruction of the eyelids. Our series demonstrates that the early grafting of eyelid burns with full-thickness grafts, can prevent the development of recurrent cicatrical ectropion. Split-thickness grafting should be limited to cases where we can not find the hairless donor site for full-thickness skin grafts.     

Lagophthalmus mit Oberlidektropium bei kongenitaler Ichthyosis

This report concerns the case of a 10-year-old female patient with congenital ichthyosis who developed bilateral ectropion of the upper eyelids resulting in massive ocular surface alterations due to lagophthalmos. A complex surgical approach with partial excision, tarsal strip refixation and a free skin graft led to normalization of the eyelid positioning and healing of the ocular surface.