A symblepharon is a fibrous adhesion of the palpebral conjunctiva and the bulbar conjunctiva of the eye. It is most commonly caused by a response to inflammation, as a result of inherited or systemic disease, or trauma.
Presentation
The symptoms of symblepharon are dependent on the degree of the fibrous adhesions [1]. The adhesions that are located between the palpebral conjunctiva and the bulbar conjunctiva may occur only in the anterior region (anterior symblepharon), in the fornix (posterior symblepharon), or comprise the whole eyelid (total symblepharon). Patients may complain of mucous build up around the corners of the eye, eye redness, frequent tearing, feeling of 'dry eye' that is attributed to an inability to close the eyelid, restriction of ocular motility, reduced visual acuity, and/or involvement of the cornea. Additionally, diplopia (which occurs secondary to restricted ocular motility), entropion (eyelid inverted or folded inward), inability to fully close the eyelids (lagophthalmos), and altered cosmetic appearance may also be present [2].
Entire Body System
- Pain
Symptoms of pathology depend on primary disease, pains, gripes, dacryagogue, existence of cosmetic defect and so forth are possible. [medicine-for-you.com]
Patients with ocular involvement may present with pain or the sensation of grittiness in the eye and conjunctivitis. Erosions may be seen on the conjunctival surface. [emedicine.medscape.com]
By the next visit, the patient's pain had improved, and her vision stabilized at counting fingers OD and hand motions OS. She complained of debilitating vision loss and wanted to discuss her options for visual recovery. [eyeworld.org]
Signs: asymptomatic, reduced vision, ocular pain. Diagnosis: history, clinical signs, ophthalmic examination. Treatment: surgical if the condition is severe but re-adhesions complicate the repair. Prognosis: depends on severity. [vetstream.com]
- Fever
[…] disease associated with a 5-15% mortality rate Ocular involvement in 50% Associated with various infections and medications most notably sulfa Ocular involvement in 50% Pathogenesis: Angiitis-->Erythematous lesion-->Bullae-->Rupture-->Scar Prodrome: Fever [eyeplastics.com]
She had a history of fever from malaria 2 months back for which she was treated with oral chloroquin phosphate 500 mg. [ijo.in]
The most frequent manifestation of an ocular adenoviral infection is epidemic keratoconjunctivitis, followed by pharyngoconjunctival fever. [ncbi.nlm.nih.gov]
Skin
- Flushing
Finally, the excess amniotic membrane and fibrin gel are trimmed to make the graft flush with the conjunctival or oral mucosal graft edges. [healio.com]
Eyes
- Diplopia
To report the occurrence of diplopia and symblepharon following conjunctival Muellerectomy for ptosis repair in 3 patients under chronic antiglaucoma treatment. [ncbi.nlm.nih.gov]
All 3 patients reported diplopia after surgery. Two of the patients were treated with prismatic correction and one underwent surgical correction. [journals.lww.com]
- Lagophthalmos
The aim of eyelid surgery in firework-related injuries is to prevent delayed damage, such as lagophthalmos, symb… Publisher Full Text Ocular surface repair using decellularized porcine conjunctiva. [unboundmedicine.com]
Clinical picture It is characterised by difficulty in lid movements, diplopia (due to restricted ocular motility), inability to close the lids (lagophthalmos) and cosmetic disfigurement. [ikeyes.blogspot.com]
Additionally, diplopia (which occurs secondary to restricted ocular motility), entropion (eyelid inverted or folded inward), inability to fully close the eyelids (lagophthalmos), and altered cosmetic appearance may also be present. [symptoma.com]
However, in more severe cases, the fornix of the eye may become obliterated, cicatricial entropion may form, or there may be permanent lagophthalmos with exposure of the cornea.[15] Obliteration of the fornix can cause insufficient tear reservoir and [eyewiki.aao.org]
In cases combined with an eyelid abnormality, eyelid surgery should be performed to correct entropion, trichiasis, or lagophthalmos. Surgical failure may more easily happen in symblephara at higher grades [6, 13, 23]. [bmcophthalmol.biomedcentral.com]
- Burning Eyes
Methods Thirty eyes of 29 patients had severe symblephara resulting from eye burns. [link.springer.com]
The symblephara were caused by chemical burns in 36 eyes (acid burn, n = 11; alkali burn, n = 25) and thermal burns in 44 eyes (metal solution burn, n = 31; firework burn, n = 13). [bmcophthalmol.biomedcentral.com]
- Blepharitis
Pertinent findings BCVA 20/20 OD/OS Anterior segment Mild posterior blepharitis Symblepharon nasally OD, inferior and superior palpebral conjunctiva scars OU Laboratory studies Cultures: Bacterial, EKC, Herpes, Cytomegalovirus, Chlamydia all negative [aaopt.org]
Secondary ankyloblepharon following herpes infection Trauma - Burns, chemical or physical injuries Cicatrizing diseases like Stevens-Johnson syndrome or cicatricial ocular pemphigoid Inflammatory diseases such as herpes simplex infection or ulcerative blepharitis [eyewiki.aao.org]
- Congenital Ankyloblepharon
Surgical therapy Congenital ankyloblepharon The surgical management of congenital ankyloblepharon depends on the type and extent of the lid margins adhesions. [eyewiki.aao.org]
Neurologic
- Irritability
The ring helps to reduce scarring and keep the eyelids away from an irritable or irritated eye, helping to keep the patient more comfortable. [guldenophthalmics.com]
A bandage lens was placed in case mechanical irritation from conjunctival papillae was contributing to the non-healing epithelial defect. [nzma.org.nz]
Entropion causes great discomfort, as the eyelashes continuously rub against the cornea causing irritation. This is turn causes secondary negative effects on the ocular surface. [eyecyte.com]
The liquid bandage created by these devices can offer significant relief from the pain and photophobia that result from ocular surface dryness and the irritation associated with trichiasis and distichiasis, he says. [reviewofoptometry.com]
- Burning Sensation
For most patients, the common symptoms include: Redness Tearing Burning sensation Foreign body sensation Photophobia Lid heaviness Blepharospasm Finally, patients can also present with ocular motility disorders, diplopia, entropion, and inadequate lid [visualdx.com]
Patients may complain of dry eyes, burning sensation, photophobia, or decreased vision.[5] Physical examination Symblepharon has variable severity and tissue involvement. [eyewiki.aao.org]
Workup
Workup consists of a review of medical history, assessment of current medications, and an ocular examination [3]. Laboratory tests may be performed when they are clinically indicated or to rule out a disease that is associated with symblepharon [4]. Symblepharon is a physical finding that can be a result of one of many etiologies. Therefore, the patient's medical history should include questions that evaluate presence of conditions commonly associated with symblepharon like ocular cicatricial pemphigoid, history of trauma, ocular chemical exposure, or conjunctival burns, recent ocular surgeries, pseudopemphigoid conditions, Stevens-Johnson syndrome, sarcoidosis, atopic keratoconjunctivitis, porphyria cutanea tarda, epidemic keratoconjunctivitis, squamous papilloma of the conjunctiva, ocular rosacea, and xeroderma pigmentosum [5] [6] [7] [8] [9].
The ocular examination should consist of the following:
- Assessment of extraocular movements (EOMS): EOMS will show restricted ocular movements.
- An external and slit-lamp examination: This is done by everting the eyelids and inspecting the palpebral conjunctiva for any scarring or symblepharon during an upward gaze.
- A visual acuity test: In most patients with symblepharon, their visual acuity is usually normal, nearly normal, or unaffected by the condition itself.
- Intraocular pressures (IOP): IOP should also be evaluated to rule out other conditions.
Depending on the clinical findings, laboratory tests to rule out associated systemic diseases may be performed. These include complete blood count (CBC), erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA) levels, rheumatoid factor (RF), rapid plasma reagin (RPR), and uric acid level. Patients who are suspected of having dry eye syndrome should be tested for fodrin (a cytoskeletal protein), muscarinic M3 receptor, and sjögren syndrome (SS) specific antibodies (anti-RO [SS-A] and anti-LA [SS-B]) to confirm the diagnosis. Patients who have signs and symptoms of Stevens-Johnson syndrome will have increased levels of tumor necrosis factor (TNF)–alpha by the local tissue dendrocytes and T-cell proliferation [10] [11]. The following antibodies can be tested to rule out cicatricial pemphigoid: bullous pemphigoid antigen 1 and 2 (BPAG, BPAG2), type VII collagen, laminin 5, laminin 6, and b4 integrin subunit [12].
Treatment
Cryptophthalmos: surgical treatment of the congenital symblepharon variant. Abstract A case of the congenital symblepharon variant of cryptophthalmos is reported. Surgical treatment is described. [doi.org]
Our aim was to evaluate the efficacy of amniotic membrane transplantation in the surgical treatment of symblepharon by determining the rate of epithelization of the graft, the incidence of complications, and the rate of recurrence of cicatrization. [ncbi.nlm.nih.gov]
Prognosis
[…] title features more than 150 color illustrations throughout and a short, succinct format which in most cases, includes: Epidemiology and Etiology, History, Physical Examination, Differential Diagnosis, Laboratory and Special Examinations, Diagnosis, Prognosis [books.google.es]
Prognosis: depends on severity. Pathogenesis Etiology Previous inflammation, especially from feline herpes virus (FHV-1) infection Cornea: herpesvirus keratitis. Eyelid/conjunctival injury, especially chemical. Abscessation of eyelid. [vetstream.com]
Depending on the severity, etiology, and management of the symblepharon, there is a variable prognosis. In some cases, there may be permanent blindness, in others there may be full resolution with good visual outcome. References ↑ Glossary. [eyewiki.aao.org]
Etiology
A very rare tumor presentation might be considered, but more significant is the determination of the underlying etiology. [visualdx.com]
When discovered, the specific etiology of the symblepharon must be investigated. Management Medical therapy Medical management of symblepharon aims to prevent or decrease symblepharon formation and to treat the underlying pathology. [eyewiki.aao.org]
RESULTS: Etiology of symblepharon included chemical burn (n = 16), thermal burn (n = 7), Stevens-Johnson syndrome (n = 5), mucous membrane pemphigoid (n = 2), xeroderma pigmentosum (n = 1), and graft-versus-host disease (n = 1). [ncbi.nlm.nih.gov]
Etiologies include congenital, traumatic, postsurgical or inflammatory causes. While the first three etiologies present as a stable adhesion, inflammatory symblepharon may show a progressive course. [jaypeedigital.com]
Epidemiology
Epidemiology of epilepsy in developing countries. Bull World Health Organ. 1993; 71(2): 247–258. Preux PM, Druet-Cabanac M. Epidemiology and aetiology of epilepsy in sub-Saharan Africa. Lancet Neurology 2005; 4(1): 21-31. Anyanniyi AA. [informaticsjournals.com]
April 2009 Volume 50, Issue 13 Free ARVO Annual Meeting Abstract | April 2009 Epidemiology of Symblepharon in a Mexican-mestizo Population Author Affiliations & Notes T. N. [iovs.arvojournals.org]
Each title features more than 150 color illustrations throughout and a short, succinct format which in most cases, includes: Epidemiology and Etiology, History, Physical Examination, Differential Diagnosis, Laboratory and Special Examinations, Diagnosis [books.google.es]
You are ( * ) If you have selected the Other category, please specify which type of user you are: * Email address: * Topic of your comment * Epidemiology data Summary and related texts Related genes Clinical signs Nomenclature and/or coding Your message [orpha.net]
Pathophysiology
Pathophysiology Adhesions of conjunctival tissue (palpebral, bulbar or nictitating) to another conjunctival surface or to the cornea → reduced eyelid mobility → exposure keratopathy and/or impaired vision. [vetstream.com]
The pathophysiology is still unknown, although there are several theories proposed, ranging from autoimmune to infectious. [Read more...] EyeWiki News 2019 Residents and Fellows Contest is now open. [eyewiki.aao.org]
Shimazaki (co-chair), (None) Anatomy, Physiology, and Pathophysiology Subcommittee: E. Knop (chair), (None) N. Knop, (None) T. Millar, Alcon (F), Allergan (F) H. Obata, (None) D.A. [tearfilm.org]
Prevention
Such a device was described by Ridley2 in 1967 and others.3The symblepharon ring will work in the prevention of mild adhesions, but will not prevent symblepharon in severely pathologic conjunctiva, such as chemical burns or ocular pemphigoid. [jamanetwork.com]
Symblepharon rings are designed to prevent retraction of the conjunctival cul-de-sacs without touching the cornea. The edges are highly polished and atraumatic. [fci-ophthalmics.com]
Amniotic membrane has been studied in preventing recurrence. [eyecyte.com]
Application of a gelatin sponge proved to be a simple and effective way of preventing symblepharon formation after an alkali burn. [ncbi.nlm.nih.gov]
References
- Holsclaw DS. Ocular cicatricial pemphigoid. Int Ophthalmol Clin. 1998;38:89-106.
- Romanes GJ. Treatment of established symblepharon with split skin homograft. Brit J Opthal. 1953; 37: 236-41.
- Gruchalla RS. 10. Drug allergy. J Allergy Clin Immunol. 2003 Feb. 111(2 Suppl):S548-59.
- King MJ. Ocular lesions in Boeck′s sarcoid. Trans Am Ophthalmol Soc. 1939;37:422–58.
- Moin M, Kersten RC, Bernardini F, Kulwin D. Destructive eyelid lesions in sarcoidosis. Ophthal Plast Reconstr Surg. 2001;17:123–5.
- Flach A. Symblepharon in sarcoidosis. Am J Ophthalmol. 1978;85:210–4.
- Assier-Bonnet H, Aractingi S, Cadranel J, Wechsler J, Mayaud C, Saiag P. Stevens-Johnson syndrome induced by cyclophosphamide: report of two cases. Br J Dermatol. 1996; 135:864-6.
- Vajpayee RB, Shekhar H, Sharma N, Jhanji V.Demographic and clinical profile of ocular chemical injuries in the pediatric age group. Ophthalmology. 2014;121:377-80
- Ondas O, Keles S. Central corneal thickness in patients with atopic keratoconjunctivitis. Med Sci Monit. 2014 Sep 21;20:1687-90.
- De Rojas MV, Dart JK, Saw VP. The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. 2007;91:1048-53.
- Morel E, Escamochero S, Cabañas R, Díaz R, Fiandor A, Bellón T. CD94/NKG2C is a killer effector molecule in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Allergy Clin Immunol. 2010;125:703-10.
- Inachi S, Mizutani H, Shimizu M. Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration. Arch Dermatol. 1997;133:845-9.