Spectrum approach to congenital ectropion / Abordagem espectral do ectrópio congênito

ABSTRACT In the literature, there is a confusing classification among congenital floppy eyelid, eyelid eversion and ectropion. They are described as eyelid malposition with laxity and out-turning of the eyelids in newborns, usually associated with conjunctival prolapse and chemosis. Although the underlying pathophysiology of these rare conditions is obscure, they share anatomic characteristics. Thus, instead of a plethora of denominations, a spectrum approach should join these entities. In this paper, the authors present a case series of four patients that illustrates distinctive presentations of this condition and advocate that it should be considered as variants of a spectrum of congenital ectropion. Mild cases, when promptly treated, can benefit from clinical treatment. On the other hand, severe and delayed cases will need surgical correction as in the case of acquired ectropion.

RESUMO Na literatura, existe uma classificação confusa entre floppy eyelid congênita, eversão palpebral e ectrópio congênito. Essas afecções são similarmente descritas como pálpebras frouxas e evertidas em recém-nascidos e geralmente associadas a prolapso de conjuntiva e quemose. Embora a fisiopatologia dessas raras afecções seja incerta, elas apresentam íntimas características anatômicas em comum. Assim, ao invés dessa nomenclatura variada, seria interessante incluí-las em um espectro de uma só doença. Neste artigo. apresenta-se uma série de quatro casos que ilustram diferentes apresentações dessa afecção e propõe-se que todas devam ser consideradas variações do espectro de ectrópio congênito. Casos leves são beneficiados quando tratados precocemente. Por outro lado, casos mais graves ou que são tratados tardiamente necessitarão de procedimento cirúrgico semelhante ao ectrópio adquirido.

Vision and Ocular Surface Salvage in Extreme Postburn Cicatricial Ectropion With Infectious Exposure Keratitis.

Prevention and early management of postburn cicatricial ectropion is the best strategy to avoid ocular complications, with poor visual prognosis in extreme cases. A 51-year-old man presented with diminution of vision and absolute inability to close both eyes, 3 months after thermal burn injuries to face, upper limbs, and trunk. His best corrected visual acuity (BCVA) was 1/60 in BE. He had bilateral extremely severe ectropion involving both upper and lower lids with complete inability to close the eyes. The ensuing exposure keratitis developed secondary infection by Methicillin-resistant Staphylococcus aureus (MRSA) in the right eye and multidrug-resistant Pseudomonas aeruginosa in the left eye. His extreme ectropion prevented infection healing, so its release and full-thickness skin grafting was done when partial resolution of infection was noted. After 3 months, he had moderate residual ectropion in BE; vascularized corneal scar in the inferior part of the right eye (BCVA:20/40) and adherent leucoma in left eye (BCVA:HM). Prioritizing ectropion surgery in our extreme case for infection control, facilitated corneal healing. Our case highlights the extreme consequences of not taking preventive measures or of managing incident ectropion following thermal injury to the face.

Sight-threatening Complication of Cicatricial Ectropion in a Patient with Lamellar Ichthyosis - Case Report.

We report a case of lamellar ichthyosis and sight-threatening complications of cicatricial ectropion in an adult male patient which was surgically managed with tectonic penetrating keratoplasty. We present a case of autosomal-recessive lamellar ichthyosis in a 47-year-old man who was referred to our outpatient eye clinic for treatment of primary keratouveitis of the right eye with keratolysis and exudation in the anterior chamber. A diagnosis of cicatricial ectropion with serious lagophthalmos was established on examination. The patient underwent tectonic penetrating keratoplasty, cataract extraction, and intra-ocular lens placement with no perioperative complications. The patient was subsequently treated with oral fluconazole 200 mg once daily for 12 days due to a positive fungal culture for Candida albicans and systemic oral acyclovir 250 mg 3 times per day for 12 days as prophylaxis for a labial herpetic infection. Post-operative complications included corneal rejection and nonhealing neurotropic epithelial defect of the graft. Long-term treatment with topical cyclosporine (Ikervis®) and dexamethasone led to resolution of the corneal rejection. Lubrication with artificial tears containing hyaluronic acid, perfluorohexyl octane (Evotears®), and vitamin A ointment led to symptomatic relief of dry eye disease. The patient was referred to a dermatologist and was started on systemic retinoid acitretin at a dose of 0.5 mg/kg per day. Ten months after surgery, the patient's visual acuity was 0.1 based on the Snellen chart and the corneal graft was stable. Infection in the cornea can rapidly progress to corneal melting in patients with severe cicatricial ectropion. A good patient outcome depends on the interdisciplinary approach to patient management by the ophthalmologist, dermatologist, and plastic surgeon.

Congenital Total Eversion of Upper Eyelids in a Normal Newborn.

Here, the authors report a rare case of congenital bilateral upper eyelid eversion with severe chemosis that was successfully managed by nonsurgical ways. A 2-hour-old male newborn with bilateral congenital upper eyelid eversion and severe chemosis was properly treated with conservative management consisting of the application of the antibiotic, steroid, and artificial tear drops and ointments in combination with padding the exposed conjunctiva with normal saline-soaked gauzes. The management was resulted in prompt and satisfactory resolution on the 14th day. Congenital upper eyelid eversion being typically a rare clinical entity is reported more frequently in African population. Although this unfavorable condition could end up with destructive complications like loss of vision, a reasonable anatomical outcome with good cosmetic results can be effectively achieved by conventional methods. Therefore, a thorough ocular examination should be carried out to exclude other ocular or systemic complications.

Conservative management of congenital upper eyelid eversion.

We report 2 cases of congenital upper eyelid eversion and highlight nonsurgical treatment options, including a novel approach.

Management of ocular manifestations of autosomal recessive congenital ichthyosis 4B, harlequin type, in the perinatal period.

Autosomal recessive congenital ichthyosis (ARCI4B [OMIM #242500]), also known as harlequin ichthyosis, presents at birth with extreme hyperkeratosis and thick-fissured plaques, leading to tightness of the skin around the eyes, mouth, ears, chest, abdomen, and extremities. Ocular manifestations include cicatricial ectropion and exposure keratitis. We present 2 infants with ARCI4B and cicatricial ectropion who were managed with aggressive nonsurgical therapy. Both infants avoided severe ocular sequelae and maintained corneal clarity, highlighting that management of exposure keratopathy with frequent ophthalmic ointment application can prevent severe ocular surface pathology in ARCI4B.

Congenital Bilateral Eyelid Eversion and Chemosis: A Case Study.

This case reports the findings and management of a late preterm female infant born with congenital bilateral eyelid eversion with chemosis. The pathogenic process remains unknown but typically presents at birth, predominantly affecting the upper eyelid of both eyes. Black males, patients with trisomy 21, and collodion infants have a higher incidence of eyelid eversion. Treatment modalities range from conservative therapy including eye patching with antibiotic and lubricating ointment to invasive surgical eyelid suturing. In this case report, successful resolution of chemosis and eyelid inversion occurred with conservative management.

Bilateral congenital upper eyelid eversion: the clinical course and outcome of conservative management.

A case of bilateral congenital upper eyelid eversion (CUEE) in an otherwise normal healthy 6 day old neonate of African descent (Nigeria). Pregnancy and delivery history were uneventful. The baby recovered completely 5 days after the commencement of conservative management. This case further gives credence to the usefulness of conservative therapy in the management of CUEE.

Management of cicatricial ectropion secondary to pyoderma gangrenosum.

Pyoderma gangrenosum is a rare condition with few cases of eyelid involvement reported in the literature. Pathergy is a well-recognised phenomenon that can trigger this condition. Pyoderma gangrenosum should be considered in cases of progressive cribriform cicatrisation where there is a history of antecedent trauma. Surgical management of a resultant ectropion may be challenging as a result of aggressive scarring and the risk of provoking a recurrence. We report a case of pyoderma gangrenosum causing a cicatricial ectropion, and discuss the underlying aetiology of iatrogenic incitement, and its implications for surgical management.

Congenital bilateral upper eyelid eversion: report of a case.

Congenital bilateral upper eyelid eversion is a rare condition and the definite cause is not known. It is often seen in Black babies or babies with Down's syndrome. With early diagnosis and appropriate treatment, the condition can be managed without surgery. We report a case of congenital upper eyelid eversion in an otherwise healthy Caucasian neonate, born by normal vaginal delivery. The case responded well to conservative treatment, including eyelid repositioning, lubricants, antibiotic ointment, and eyelid patching.

Ichthyosis in the newborn.

The ichthyoses encompass a variety of genetic disorders marked by abnormal epidermal differentiation. The neonatal period is critical for patients with ichthyosis because of the risk for significant associated morbidity and mortality, with the majority of complications arising as a result of impaired barrier function. This article reviews presentations of ichthyosis in the neonate, outlines risks and complications, and provides strategies for management.

The incidence of lower eyelid malposition after facial fracture repair: a retrospective study and meta-analysis comparing subtarsal, subciliary, and transconjunctival incisions.

BACKGROUND: Many incisional techniques have been described for access to the craniofacial skeleton for traumatic fracture repair, including subciliary, subtarsal, and transconjunctival approaches. Each is associated with a distinct set of complications. The goal of the authors' study was to examine lower lid malposition associated with these incisional approaches at the authors' institution and in the literature. METHODS: A retrospective chart review of all operative orbital and/or zygomatic fractures at the Beth Israel Deaconess Medical Center from 1998 to 2008 was performed. The surgical approach and postoperative lower eyelid complications were noted. A literature review was performed of all peer-reviewed studies in the English language examining the incidence of complications of lower eyelid incisions in operative facial trauma. RESULTS: A total of 180 cases were treated with a lower eyelid incision; these were identified as subtarsal (41 percent), subciliary (31 percent), and transconjunctival (25 percent). The risk of ectropion was highest in subciliary incisions (12.5 percent, p = 0.018); however, only two cases required operative management. Entropion was found in two cases after transconjunctival incisions; both required operative management. Seventeen studies were included in the meta-analysis, representing 2086 patients. The risk of ectropion was highest in subciliary incisions (14 percent), the risk of entropion was highest in transconjunctival incisions (1.5 percent), and the risk of hypertrophic scarring was highest in subtarsal incisions (3.4 percent). A majority of these complications resolved with conservative management. CONCLUSIONS: Lower eyelid complications can be seen with all three incisional approaches to facial fracture repair. Based on their findings, the authors favor the subtarsal approach for zygomaticomaxillary fractures and the transconjunctival incision for isolated orbital floor fractures.

Congenital bilateral eversion of the eyelids.

The authors report a case of congenital bilateral eversion of the eyelids with secondary conjunctival prolapse in a 4-hour-old neonate. Conservative treatment with hypertonic normal saline, lubricants, antibiotics, and padding was instituted. No surgery was performed. Complete eye opening was achieved by the 10th day after birth. Medical management is advantageous in that it is inexpensive, noninvasive, and can be performed even in a center with poor surgical facilities, as is commonly encountered in developing countries. The sociopsychological aspect of the disease, possible pathophysiology, and treatment are discussed.

Facial nerve palsy: anatomy, etiology, evaluation, and management.

The ophthalmologist may be the first clinician to see a patient who presents with acute facial nerve palsy. Under such circumstances the ophthalmologist should make every effort to establish the underlying cause of the facial palsy and ensure that the patient's cornea is adequately protected. This article reviews the anatomy of the facial nerve, the varied disorders that may cause a facial palsy, a detailed evaluation of such a patient, and the various medical and surgical treatments available.