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.     

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.     

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.     

Ocular manifestations of congenital lamellar ichthyosis

PURPOSE: To describe the ophthalmic manifestations in a series of children with congenital lamellar ichthyosis. These cases presented with varying types of eyelid abnormality associated with the systemic disease. The clinical features and ophthalmic management were studied. METHODS: The case histories of three children presenting to the oculoplastic clinic were reviewed. All were diagnosed with congenital lamellar ichthyosis and under the care of the Dermatology department. Family history and pedigree analysis was performed to determine mode of genetic inheritance. Ocular examination for visual acuity, eyelid and eyelash malposition, lid function and closure were carried out. Corneal examination including tests for exposure was also done. RESULTS: All three patients had eyelid position abnormalities from the systemic disease. There was no clinical evidence of conjunctival involvement. One patient required full thickness skin grafts to treat corneal exposure secondary to lower lid ectropion. One had mild lower lid ectropion but without corneal exposure. The third case had the unusual finding of inward turning of the anterior lamella of the upper eyelid with a marked lash ptosis and only mild ectropion of the lower lid. CONCLUSIONS: Congenital lamellar ichthyosis is a heterogeneous disorder with phenotypic variability. The most common eyelid abnormality is cicatricial ectropion of the upper and mainly lower eyelids. Most cases are managed conservatively although in severe cases secondary corneal exposure may require surgical correction. In this condition, to the best of our knowledge, the tendency for the eyelids to turn inwards has not previously been described.     

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.     

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.     

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.     

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.     

颞区水平皮瓣修复瘢痕性下睑外翻的临床观察

目的探讨外眦角颞侧水平带蒂皮瓣在下睑瘢痕性外翻修复中的应用。方法对11例（11只眼）伴有皮肤组织缺损的下睑外翻患者实施该手术,其中3只眼为局部疖肿溃破后皮肤瘢痕挛缩,2只眼为铝水烫伤后,6只眼为皮肤挫裂伤后。年龄15～58岁。局麻下手术,根据下睑皮肤缺损范围,在颞区设计一以肌肉组织为蒂的水平向皮瓣。皮瓣穿过供、受区之间的皮下隧道后缝合于缺损区。结果术后第2天,有1只眼皮瓣的鼻侧端微紫,1周后色泽日趋正常。术后随访3～6个月,11只眼下睑皮瓣色泽良好,与周围组织差异小,无外翻发生。颞区伤口瘢痕细而淡。结论颞区水平皮瓣在伴有皮肤组织缺损的瘢痕性下睑外翻修复中疗效良好,供区水平伤口愈合佳,瘢痕小。但供皮量受颞区皮肤松弛程度的限制,且应严格控制皮瓣长度,避免因血供不足造成组织坏死,因而更适合于下睑颞侧半皮肤缺损的修复。     

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.     

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.     

Anthrax as the cause of preseptal cellulitis and cicatricial ectropion

A 54-year-old female farmer with anthrax infection of the eyelids is presented. She was initially managed with high dose intravenous penicillin G treatment. Following complete healing of the eyelid lesions, significant cicatricial ectropion resulted. Her right lower eyelid ectropion was corrected by surgical reconstruction using full thickness skin graft after a period of 6 months during which the cicatrization process stabilized. Satisfactory cosmetic and functional improvement was achieved. Anthrax of the eyelid must be considered in the differential diagnosis of preseptal or orbital cellulitis and any reconstructive procedure should be attempted only after the cessation of the healing process.     

Eyelid reconstruction with hard palate mucosa grafts.

Hard palate mucosa grafts are an excellent replacement for tarsus and conjunctiva in eyelid reconstruction. Twenty-five eyelids from 18 patients underwent eyelid reconstruction using hard palate mucosa grafts. Patients were treated for a variety of disorders including postblepharoplasty lower eyelid retraction, cicatricial entropion, eyelid retraction secondary to thyroid eye disease, and lagophthalmos following surgery for paralytic ptosis. Surgical results were evaluated, grafts were measured for postoperative shrinkage, and donor site healing was recorded. Several patients had hard palate biopsy specimens evaluated. One of these patients also had a graft biopsied after it had been in place for 3 months. A review of hard palate anatomy and histology and a discussion of surgical technique are presented.     

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.     

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.     

Cicatricial ectropion in mycosis fungoides and sézary syndrome: Successful surgical repair

A case of cicatricial ectropion was encountered in a 76-year-old woman with mycosis fungoides that had progressed to Sézary syndrome. The ectropion required urgent surgical repair due to corneal compromise. Although the graft appeared to fail, the donor site healed well, with no sign of spreading cellulitis suggestive of infection. To our knowledge, the successful surgical repair and outcome in such infiltrated skin has not been previously described.     

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).     

Comprehensive, combined anterior and transcaruncular orbital approach to medial canthal ligament plication

PURPOSE: To assess the efficacy of a comprehensive technique for correction of severe punctal and medial lower eyelid ectropion and lower eyelid retraction associated with medial canthal ligament (MCL) laxity. METHODS: A comprehensive technique that plicates the anterior and posterior crura of the MCL was performed on 8 eyelids of 6 patients with punctal ectropion and MCL laxity. Preoperative and postoperative symptoms, punctal ectropion, medial lower eyelid ectropion, lower eyelid retraction, lagophthalmos, and exposure keratopathy were evaluated. RESULTS: At an average of 13 months (range, 8-17 months), preoperative symptoms of epiphora and discomfort improved or resolved in all eyes. Punctal ectropion improved in all eyes and completely resolved in 75% of eyes. Medial lower eyelid ectropion was corrected in all eyes, when present. Lower eyelid retraction, lagophthalmos, and exposure keratopathy improved in all eyes. In 1 case, edema of the caruncle and semilunar fold persisted for 6 months. CONCLUSIONS: Combined anterior and transcaruncular MCL plication is an effective and safe procedure for addressing severe punctal and medial lower eyelid ectropion that accompanies MCL laxity and is difficult to correct by other methods. This procedure provides stable, 3-dimensional support to the medial lower eyelid and punctum.