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A 6-week-old baby boy with discharge
Digital Journal of Ophthalmology 2011
Volume 17, Number 3
August 15, 2011
DOI: 10.5693/djo.03.2011.07.001
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| Diagnosis and Discussion | Histopathology of ligneous lesions typically shows fibrin deposition, mixed inflammatory infiltrate, and amorphous, hyaline-like eosinophilic material resembling amyloid but found to be negative with Congo red stain. The inflammatory component in ligneous conjunctivitis is a result of the pseudomembrane, unlike in other etiologies, where the inflammatory component is the cause of pseudomembrane. Our patient’s characteristic histopathologic findings in conjunction with his severely decreased level of plasminogen activity and classic woody pseudomembranes confirmed the diagnosis of ligneous conjunctivitis. Whereas the other etiologies of pseudomembrane are primarily inflammatory or infectious in origin, the origin of ligneous conjunctivitis is genetic.
Pseudomembranes covering both eyes were first described by Bouisson in 1847. By 1933 the term ligneous conjunctivitis was proposed because the pseudomembrane had a woody appearance. Plasminogen deficiency was genetically linked with ligneous conjunctivitis in 1997.(1) A missense mutation at location K19E on the plasminogen gene (PLG) was found to be the most common mutation found in 34% of patients.(5) At this time there appears to be no racial predisposition in this genetic disorder, although there is a slightly higher incidence in females, and it has been suggested that there may be a higher incidence in areas where consanguinity is more common.(4,5)
The pathophysiology proposed for ligneous conjunctivitis is that a deficiency in plasminogen leads to deficient extravascular fibrin clearance, with wound-healing arrested at the granulation stage: minor insults to the conjunctiva may lead to an accumulation of the characteristic woody material.(4,5) The median age of clinical manifestation for plasminogen deficiency is 9.54 months, with ligneous conjunctivitis being the most frequently observed association, in 80% of cases, followed by ligneous gingivitis (34%), respiratory involvement (16%), and ligneous vaginitis (8%). Congential occlusive hydrocephalus has been associated with plasminogen deficiency in up to 8% of cases.(5) The clinical course of plasminogen deficiency depends on the site of involvement. When considering ligneous conjuncitivitis, erythema of the lid margin precedes epiphora, followed by development of woody conjunctival membranes. The condition is bilateral in roughly 50% of patients. Vision loss occurs in 20-30% of cases, primarily due to corneal involvement. Although most patients present as children, there are case reports of ligneous conjunctivitis in patients over age 55.
The natural history of these lesions is variable. Some resolve without treatment over months, whereas others may last many years. Patients with plasminogen deficiency may have a reduced life expectancy but this is most particular to the groups manifesting respiratory or cerebral involvement. Pseudomembranes involving the larynx or tracheobronchial tree can lead to recurrent pneumonia and airway obstruction. Pseudomembranes can also lead to a congenital occlusive hydrocephalus, which has a particularly poor outcome.(4,5)
Surgical excision often results in regrowth, as in our case, and many therapies have been attempted to prevent this, including amniotic membrane transplantation (AMT), local heparin, corticosteroids, cyclosporine, azathioprine, hyaluronidase, oral contraceptives, topical plasminogen, systemic plasminogen, fresh frozen plasma, and allogeneic serum drops. The AMT used in our patient prevented recurrence for 7 months. Barabino and Rolando first used AMT in the treatment of ligneous conjunctivitis; they reported no membrane recurrence 36 months postoperatively.(6) Recently, Lee and Himmel reported success combining allogeneic serum with topical heparin every two hours plus prednisolone acetate 1% drops 4 times daily.(1) Resolution of a ligneous membrane occurred without surgical intervention over a 2-month period, at which time all drops were discontinued. At 2 year’s follow-up there was no membrane recurrence. Many case reports describe good success with plasminogen drops; however this therapy is not available in the US.(1,2,4,5)
Acknowledgments
The author thanks Kyle Acosta, MD, Maria Vives, MD, and Mathew Stark, MD, for their assistance. | |
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