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.     

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.     

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.     

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.     

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.     

Reversible cicatricial ectropion associated with EGFR inhibitors

The management of cicatricial ectropion resulting from epidermal growth factor receptor (EGFR) inhibitors is unclear. We describe two cases of bilateral cicatricial ectropion following the use of an EGFR inhibitor who were treated with oral doxycycline, topical ophthalmic steroid and antibiotic ointment to the eyelids, and topical facial steroid cream with lubrication. The first case resolved with discontinuation of panitumumab infusions along with institution of the aforementioned regimen. However, it is unclear whether the resolution was from discontinuation of the infusions or from the instituted regimen. The second case resolved without a dose adjustment of cituximab. This case may provide support for the use of this regimen prior to discontinuation of the offending agent, as there was a successful outcome without alteration of the infusions. Additional cases are necessary to determine if this is a successful means of treating bilateral lower-lid cicatricial ectropion from EGFR inhibitors.     

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.     

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 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.     

Ectropion correction with the bucket-handle technique (Tripier flap)

A bridge-pedicled flap from the upper lid (bucket-handle) has been used to correct certain types of ectropion of the lower lid. The cicatricial variety of senile ectropion is by far the most important indication for the application of this technique. Environment, such as dry climate and excessive exposure to sunlight seems to play an important role in the increased frequency of this type of ectropion. Twelve cases were operated upon with a follow-up period of up to two years. Results have been very satisfactory and lasting. Other indications for the application of bucket-handle technique include secondary cicatricial type of ectropion complicating the treatment of lower lid lesions with surgery, radiation or cryotherapy.     

Cicatricial ectropion in progressive skin diseases

PURPOSE . To report the clinical course, patient care and treatment of cicatricial ectropion in patients with progressive skin diseases. METHOD . Review and photo series of three typical cases, which were followed for up to 10 years. RESULTS . In certain severe progressive skin diseases, tissue shrinkage may progress permanently. The soft lid tissue cannot withstand the forces of vertical lid traction. As a result, recurrent ectropion occurs. Patients with lamellar ichthyosis and with eruptive Grzybowski-type keratoacanthoma were followed for up to 10 years. Free skin grafts of severely involved donor skin were repeatedly transplanted to the lids. The lid margins were fixed by traction sutures in order to spread out the wound and to allow rapid vascular ingrowth and undisturbed healing. In this way, early wound contracture could be prevented. Nevertheless, follow-up revealed progressive shrinkage of the transplanted lid skin. Eversion of the lacrimal punctum was the first sign of progression. Epiphora was the leading complaint of the patients. Bacterial superinfection of the deepened lacrimal lake was more frequent in advanced ectropion. CONCLUSION . Patients should understand the natural history of their disease in order to accept multiple surgical procedures. Ectropion should be re-operated in time in order to reduce epiphora, to prevent corneal complications, and to avoid metaplasia and keratinization of the conjunctiva and thickening of the lid margin. The elasticity of the lid skin should be improved pharmacologically and by increasing the relative humidity of the home environment, especially in winter. Consistent vertical lid massage can delay recurrence.     

Resolution of pityriasis rubra pilaris-induced cicatricial ectropion with systemic low-dose methotrexate

PURPOSE: To report a case of pityriasis rubra pilaris (PRP)-induced bilateral lower eyelid cicatricial ectropion that resolved with systemic low-dose methotrexate. DESIGN: Observational case report. METHODS: A retrospective case review of a patient with cicatricial ectropion caused by PRP. RESULTS: A 82-year-old female presented with bilateral lower eyelid cicatricial ectropion secondary to PRP. The patient did not systemically respond to conventional oral retinoid therapy. She was treated with low-dose methotrexate and experienced dramatic resolution of her signs and symptoms, including complete resolution of her bilateral cicatricial ectropion. CONCLUSION: Systemic treatment of PRP with low-dose methotrexate may result in successful treatment of cicatricial ectropion without surgery.     

Cicatricial ectropion in progressive skin diseases

PURPOSE. To report the clinical course, patient care and treatment of cicatricial ectropion in patients with progressive skin diseases. METHOD. Review and photo series of three typical cases, which were followed for up to 10 years. RESULTS. In certain severe progressive skin diseases, tissue shrinkage may progress permanently. The soft lid tissue cannot withstand the forces of vertical lid traction. As a result, recurrent ectropion occurs. Patients with lamellar ichthyosis and with eruptive Grzybowski-type keratoacanthoma were followed for up to 10 years. Free skin grafts of severely involved donor skin were repeatedly transplanted to the lids. The lid margins were fixed by traction sutures in order to spread out the wound and to allow rapid vascular ingrowth and undisturbed healing. In this way, early wound contracture could be prevented. Nevertheless, follow-up revealed progressive shrinkage of the transplanted lid skin. Eversion of the lacrimal punctum was the first sign of progression. Epiphora was the leading complaint of the patients. Bacterial superinfection of the deepened lacrimal lake was more frequent in advanced ectropion. CONCLUSION. Patients should understand the natural history of their disease in order to accept multiple surgical procedures. Ectropion should be re-operated in time in order to reduce epiphora, to prevent corneal complications, and to avoid metaplasia and keratinization of the conjunctiva and thickening of the lid margin. The elasticity of the lid skin should be improved pharmacologically and by increasing the relative humidity of the home environment, especially in winter. Consistent vertical lid massage can delay recurrence.     

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。     

Medical ectropion repair. A new procedure

The lazy-T procedure described by Smith corrects medial ectropion of the lower lid by combining a horizontal full-thickness shortening of the lid with excision of conjunctiva and tarsus inferior to the punctum to invert the lid. However, some cases of senile medial ectropion involve an element of vertical traction on the skin as well as horizontal lid laxity. A new procedure is described that makes use of skin gained from horizontal lower lid shortening in the form of a medially based transposition flap to produce vertical skin lengthening. It is useful to treat medial ectropions that have horizontal laxity along with cicatricial components and has successfully relieved this condition in eight patients.     

Cicatricial ectropion due to essential skin shrinkage: treatment with rotational upper-lid pedicle flaps

Ectropion is sometimes due to a shortage of skin of the lower lid (cicatricial) and may result from previous surgery, trauma, burns, skin diseases etc. Excessive exposure to the sun has also been incriminated. Vertical traction lines in the skin of the lower lid, accentuated by gazing up or by opening the mouth, suggest this condition. This paper describes the use of pedicle skin flaps rotated from the upper lid to treat cicatricial ectropion occurring in the absence of any predisposing factor and not responding to conservative treatment--that is, due to essential skin shrinkage. All 10 patients had an improved appearance, and epiphora persisted in only 1 patient, who subsequently underwent a punctum-enlarging procedure. In an 11th patient there was insufficient redundant upper-lid skin, so a free skin graft was used instead.     

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.     

Frontal osteomyelitis presenting as upper eyelid ectropion: A cautionary tale

Frontal osteomyelitis is a rare clinical entity that can occur as sequelae to frontal sinusitis, head trauma, as a postoperative complication following sinus surgery or due to haematogenous spread. It usually presents with a soft, fluctuant forehead swelling with pain and fever. Cicatricial ectropion is an extremely rare feature of frontal osteomyelitis. We present a young male patient presenting with cicatricial ectropion that occurred as the sole manifestation of an underlying frontal osteomyelitis. Extensive Medline search did not find any such reported case. We feel that frontal osteomyelitis should be kept in mind as a possible etiology when considering the differential diagnosis of cicatricial ectropion.