CASE REPORT

A 69 year old woman attending the eye clinic with primary open angle glaucoma complained of a 2 month history of sore eyes. She was already using gel tears for dry eyes, in addition to betaxolol and latanoprost for her glaucoma. Visual acuities were 6/9 in each eye unaided, improving to 6/6 with a pinhole. On examination she had marked bilateral, symmetrical lid retraction, lid lag, and some mild punctate corneal epithelial staining. She had no other features suggestive of thyroid orbitopathy, or of generalised thyroid dysfunction and no goitre. Her free thyroxine and thyroid stimulating hormone were normal.

Eight months earlier she had presented with a large epigastric mass and was found to have advanced primary hepatocellular carcinoma. She had a raised alkaline phosphatase (930 IU/l) and alanine aminotransferase (169 IU/l) and an iron deficient anaemia (haemoglobin 91g/l). Hypercalcaemia (corrected serum calcium 3.4 mmol/l) had been treated with rehydration and a pamidronate infusion 4 months previously. She was also taking prednisolone 5 mg twice daily, morphine sulphate, lansoprazole, metoclopramide, and codanthrusate.

Despite increasing the frequency of the gel tears her symptoms did not improve and 2 months later her vision had deteriorated to counting fingers (CF) in each eye (6/24 and 6/60 right and left with a pinhole). She had 2–3 mm of upper lid retraction, markedly reduced blink, lid lag on downgaze, 3 mm of lagophthalmos, and marked interpalpebral corneal staining (Figs 1 and 2). Vertical upgaze was slightly reduced bilaterally, but felt to be typical of that commonly seen in older patients rather than indicative of a gaze palsy. Saccades were slightly jerky, particularly on upgaze. Convergence retraction nystagmus was not demonstrated. Convergence was poor, as may be expected with markedly reduced vision. Smooth pursuit on downgaze and horizontal gaze was normal. Corneal sensation was reduced bilaterally.

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Figure 1

Bilateral lid retraction.

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Figure 2

Corneal epitheliopathy

The latanoprost was discontinued, simple eye ointment prescribed at night, in addition to the gel tears, and her relatives taught her how to tape the lids closed. Thermal occlusion of both lower puncta was also performed.

A month later her eyes were more comfortable but vision no better. The lubricants were changed to frequent simple eye ointment only and urgent lateral tarsorrhaphies and medial canthoplasties were performed as a palliative procedure. This led to a significant subjective improvement in vision, reduction in inflammation, and an improvement in the corneal appearance. Her overall condition had deteriorated, however, and she died 2 weeks later.

COMMENT

Bartley1 has classified the causes of lid retraction as neurogenic, myogenic (including disorders affecting the neuromuscular junction), mechanical, and miscellaneous. It is generally accepted that thyroid orbitopathy is the commonest cause of acquired unilateral or bilateral lid retraction.2 A minority of such patients may be both clinically and biochemically euthyroid.

The most likely cause of lid retraction in this case is either neurological or “miscellaneous”. Thyroid orbitopathy was an unlikely diagnosis on clinical grounds and further investigations, such as orbital computed tomograph scanning and thyroid antibody testing, were deemed unnecessary.

Poor upgaze is common in older patients and the patient had no other features suggestive of Parinaud's dorsal midbrain syndrome (her poor convergence was felt not to be significant). Decreased blink rate and lid lag are seen in both progressive supranuclear palsy and in Parkinson's disease, together with “cogwheel” pursuit and slow, hypometric saccades. While the patient had rather jerky saccades, she had no other features suggestive of either condition and, in particular, had no parkinsonian features and normal downgaze.2 ,3

Bilateral lid retraction and lid lag with minimal impairment of vertical gaze have been described with a circumscribed lesion involving the periaqueductal grey matter in the region of the nucleus of the posterior commissure (nPC).2 ,4 It is possible that the patient had a brain metastasis involving the nPC, although it should be noted that she had no other overt clinical evidence of this. The patient had not had neuroimaging of her brain, and it was not appropriate to perform this in the terminal stages of her disease.

Eyelid retraction has also been reported in association with a number of conditions with an undetermined aetiological relation. An example is the association with hepatic cirrhosis (Summerskill's sign),1 ,2 which Walsh and Hoyt suggest may cause a myopathy of the levator.5 An association with hepatocellular carcinoma has not been described previously, although we postulate that a similar mechanism may be involved. While prolonged high dose steroids may also cause lid retraction,6 this patient was on only a low palliative dose.

The severe keratopathy was probably multifactorial. Her eyes were exposed due to lid retraction, markedly reduced blink, and lagophthalmos, and a neurotrophic element was likely because of reduced corneal sensation. A toxic element could not be ruled out and therefore all topical medication was eventually stopped, apart from simple eye ointment that contains no preservatives. This, together with surgery to reduce the palpebral apertures, finally led to palliation of the patient's ocular symptoms.