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A seven month old baby with a right sticky eye
Digital Journal of Ophthalmology 2006
Volume 12, Number 3
May 10, 2006
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Imran Rahman | Manchester Royal Eye Hospital
ABHA GUPTA
Diagnosis and Discussion
Discussion

Ligneous conjunctivitis is a rare form of idiopathic membranous conjunctivitis first reported in 1847 (2). Over 100 cases have been reported worldwide, but the true incidence remains unknown.
Although the exact etiology is unknown, local injuries, infections, lime burns and surgical interventions have all been associated with ligneous conjunctivitis (3). Most cases are sporadic although an autosomal recessive inheritance pattern has been described (4). It has since been found that ligneous conjunctivitis is strongly associated with homozygous or compound-heterozygous type I plasminogen deficiency (5).
Most often the upper tarsal conjunctiva is involved, presenting with thick viscous, mucopurlent discharge. The membranes are firm, sessile and affect both eyes in 50% of cases. Visually impairing corneal involvement may be seen in up to 30% of cases (6). The disease duration can be variable and may extend from a few months to as long as 36 years (7).
Importantly, additional sites such as the kidneys, respiratory tract, female genitalia, ear and gingival maybe involved (3).

The precise mechanism of membrane formation in ligneous conjunctivitis has been suggested to centre on the plasminogen activator system and wound healing. The mechanism behind wound healing is highly regulated to remove injured tissue and replace it with normal healing tissue. A tissue injury results in plasma protein exudation and immediate coagulation. This clot provides haemostasis and is eventually replaced by granulation tissue, which in turn is replaced by a secondary collagenous matrix. Granulation tissue degradation is dependent on the plasminogen-activator system and matrix metalloprotease (MMPs). In fact, it has been shown that patients suffering ligneous conjunctivitis, healing in mucus membranes is markedly impaired with membranes largely consisting of fibrinogen. This may signify a deficiency of extracellular fibrinolysis (3). The role of plasminogen deficiency in the pathogenesis of ligneous conjunctivitis is further supported by a case of membrane formation in a patient taking tranexamic acid, a commonly used antifibrinolytic drug (8).

Spontaneous resolution of this condition is rare (9) and several treatment modalities have been tried. In light of hypoplasminogeneaemia being a strong aetiological factor, the number of ocular surgical procedures should be kept to a minimum to prevent membrane formation in such patients. Many forms of local treatment have been used, often with disappointing results. Topical hyaluronidase has been used successfully in some patients but not all. Cyclosporine A in combination with a topical corticosteroid may decrease severity and recurrences following surgical excision, without systemic side effects (10). De Cock et al (1) showed aggressive initial treatment of membranes with surgical excision and immediate intensive topical heparin and prednisolone until complete reepithelisation of the conjunctiva controlled disease in 13/17 cases. This is the regimen we used in the presented case. Watts et al (11) showed the use of topical plasminogen concentrate in softening membranes for ease of removal.

The treatment regimen utilized in this case resulted in successful resolution of symptoms. Plasminogen deficiency was noted in the child and has been strongly associated with ligneous conjunctivitis in the literature (5, 11, 12). Hypoplasminogeneaemia may point towards a diagnosis of ligneous conjunctivitis before a formal histology report is available and is therefore an important investigation to consider in cases of membranous conjunctivitis.
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