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Spontaneous hyphaema in hereditary haemorrhagic telangiectasia
  1. Warrington Hospital NHS Trust, Lovely Lane, Warrington WA5 1QG
  1. Mr Cota.

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Editor,—Hereditary haemorrhagic telangiectasia (HHT) is characterised by multiple dilatations of capillaries and venules in the skin, mucous membranes, and viscera that have a tendency to bleed. Although a relatively rare condition, ocular involvement is common in these patients (45-65%).1 Intraocular involvement is however extremely rare.

We report a patient with HHT with spontaneous bilateral hyphaemas


A 69 year old man presented with sudden onset of blurred vision in the left eye associated with a “red streak” under his left pupil. There was no history of any trauma. He had suffered from intermittent epistaxis, haematuria, gastrointestinal bleeds, and post-coital penile bleeding associated with telangiectasia on his face, external ear, chest, and glans penis. Past medical history included HHT, iron deficiency anaemia, and hypertension. Current medication included captopril, omeprazole, and ferrous sulphate. He recently had blood transfusion for severe iron deficiency anaemia.

On ophthalmic examination, visual acuity was 6/12 right eye and 6/18 left eye. Slit lamp examination showed hyphaema in the left eye and microhyphaema in the right. Mulberry-like iris vascular malformations were present at 12 o’clock position of both pupillary margins (Fig 1). Intraocular pressures were normal. He had moderate cataract and fundi were normal.

Figure 1

Iris photograph of the right eye showing mulberry-like iris vascular malformation at the 12 o’clock position. The left iris showed a similar appearance.

The hyphaemas resolved spontaneously within 2 weeks with no sequelae. A month later, spontaneous subconjunctival haemorrhage occurred in the left eye. Conjunctival telangiectasia were seen on the palpebral conjunctiva in both eyes. This haemorrhage resolved spontaneously.


HHT is a rare autosomal dominant disorder characterised by abnormal multiple dilatations of capillaries and venules which have a tendency to bleed. These occur in the skin, mucous membranes, and other viscera. Common presentations are epistaxis, dyspnoea on exertion, and gastrointestinal bleeding due to haemorrhage from, or shunting through, abnormal vessels in the nasal mucosa, lung, and the gastrointestinal tract. The most serious consequence of HHT is the potential for paradoxical embolisation and stroke. This occurs when venous thrombi, originating in the leg or pelvis, pass through arteriovenous malformations in the lung and lodge in the brain.

Common ophthalmic features are conjunctival and eyelid telangiectasia. Intraocular involvement is extremely rare. In a study of 20 patients with HHT conjunctival telangiectasis was seen in seven patients and retinal vascular malformation in two.1 Although retinal lesions seldom cause symptoms, retinal haemorrhages, beading of retinal arteries, branch, and central retinal artery occlusion,2vitreous haemorrhage, secondary to areas of retinal neovascularisation, and inner retinal ischaemic atrophy can occur. Spontaneous hyphaema has been reported in juvenile xanthogranuloma, retrolental fibroplasia, persistent hyaloid, blood dyscrasias, and delayed contusional injury.3 In patients of all ages, Doggart found spontaneous hyphaema associated with intraocular neoplasia, herpes simplex keratitis, zoster involvement of the iris, retrolental fibroplasia, persistent hyperplastic primary vitreous, and retinoschisis.4 Other causes of spontaneous hyphaema include inflamed iris vessels, rubeosis, iris haemangioma,5 iris neovascular tufts,6neovascularisation of cataract surgery incisions, both small incision7 and extracapsular,8 and aneurysmal dilatation from persistent strand of vascularised pupillary membrane.9 Iris vascular tufts are uncommon acquired lesions associated with non-insulin dependent diabetes, myotonic dystrophy, retinal vein occlusion, and old age. They are elevated, and confined to the pupillary border. Recurrent hyphaema arising from vascular tufts can be successfully prevented by laser.6Cataract surgery in such patients may be complicated by “in the bag hyphaemata”.8

Our case demonstrates that iris vascular malformations may occur in HHT and give rise to spontaneous hyphaema. Patients with HHT are frequently not aware of having the disease unless it has been previously diagnosed in their family.10 Hence it would be prudent to look for evidence of HHT in patients with iris vascular malformations and consider HHT in the differential diagnosis of spontaneous hyphaema.