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Figure 1
Figure 2. Hyphema grading table (Brandt et al.) (1)
| Grade | Anterior chamber filling | Diagram | Best prognosis for 20/50 vision or better |
|---|---|---|---|
| Microhyphema | Circulating red blood cells by slit lamp exam only | Slit lamp view | 90 percent |
| I | <33 percent | (Diagram I) | 90 percent |
| II | 33-50 percent | (Diagram II) | 70 percent |
| III | >50 percent | (Diagram III) | 50 percent |
| IV | 100 percent | (Diagram IV) | 50 percent |
| Case report A sixty-year old man came to the outpatient clinic with redness and pain in the right eye, without any trauma. It was thought to be episcleritis and was initially treated with prednisone eye drops and prednisone 30 mg tablets for three days. He came back after a week with no improvement. The advice was to wait and to use cold compresses. There was no headache and no general illness at the time. After three months, there was still no improvement. The patient had the same redness and pain of the right eye, but additionally there was general malaise and severe weight loss. He had a strong headache and loss of vision of the right eye. On physical examination, he looked ill; the vital signs were normal. He seemed to be disorientated and suffered from memory loss. No abnormalities were found during auscultation of the heart or lungs nor on abdominal examination. There was a general weakness of the extremities. His HIV status was negative. Consult online was asked for a differential diagnosis and advice on treatment. |
| Setting Kikori rural hospital is a district hospital in Papua New Guinea. It is located in the jungle and it can only be reached by boat or plane from Port Morseby. The nearest referral hospital can be reached after two days of travel. In case of emergency, a helicopter of the nearby oil company can be used, provided that the weather conditions are good. Annually, this hospital provides healthcare to 30,000 patients and has a capacity of 80 clinical beds. There are two doctors working in the hospital, and there is one operating room. At the time of presentation of this patient, there was a tuberculosis epidemic in this area. |
Advice from the specialists
Two of the specialists responded within a day. They said that the photo (Figure 1) was suggestive for hyphema, blood in the anterior chamber of the eye. Presumably, the intraocular pressure was raised, and this can cause headache, nausea and general malaise. However, the cornea looked clear. This argued against a highly raised intraocular pressure, as the cornea would then show a greyish glow and there was no evidence for hematocornea, a discoloration of the cornea. To test if the eye pressure is raised, the cornea can be palpated while the patient is looking down. The resistance must be compared to the other normal eye.
If the intra-ocular pressure is raised, the pressure can be released by making a small incision in the anterior chamber of the eye, after retrobulbar anaesthesia. Then the blood can be rinsed out of the anterior chamber with a cannula. If this does not lower the eye pressure and the eye stays painful and blind, there are a few options such as atropine eye drops and prednisone eye drops. If there is no vision left at all, a retrobulbar injection with alcohol 70% can be given after an injection with lidocaine. If this is still not adequate, enucleation may be considered.
The underlying cause of the hyphema was still not clear, as hyphema alone does not cause a general illness. Presumably, it was caused by a rubeosis iridis (neovascularization of the iris), after a vascular occlusion of the eye. In most cases, there is a longstanding hypertension, but an infection or a tumour is also a possibility. Tuberculosis and syphilis can cause vascular complications and could be the underlying cause of the hyphema. It was suggested to test for TB and syphilis and to start treatment if the results were positive.
Treatment and follow-up
The patient was admitted to the hospital. Because of his poor condition and the unknown cause, he was treated with intravenous broad-spectrum antibiotics, TB medication, prednisone, and corticosteroid eye drops.
At first, his general condition seemed to improve, but the eye stayed painful and red. Unfortunately, after a few weeks, there was a deterioration and he eventually passed away. The cause of death remains unknown; there was no fever, hypertension or neurological symptoms at the time of death.
Discussion
Spontaneous hyphema is an uncommon condition with debilitating consequences. The classification system for hyphema defines grade I-V (Figure 2), and this patient had a grade II-III hyphema.
Hyphema can occur spontaneously or after minor trauma in patients with bleeding tendency or conditions that cause neovascularization (rubeosis iridis) or vascular anomalies of the anterior chamber structures. (1, 2, 5) This includes diabetes mellitus, iris melanoma, clotting disorders (e.g. thrombocytopenia, haemophilia, von Willebrand’s disease), and the use of antiplatelet drugs. (2,3,4) Infections that may cause vascular complications can also cause hyphema, such as tuberculosis, HIV and syphilis. Furthermore, several malignancies (or metastases) can also cause vascular abnormalities. (4)
The prognosis for hyphema depends on the grade (Figure 2), and the treatment of the underlying cause. Moreover, some patients are at higher risk of permanent complications and vision loss, such as patients with sickle cell disease or sickle cell trait. (2) Patients with clotting disorders or on anticoagulants are also at increased risk of vision loss because of the greater frequency of rebleeding. If possible, the clotting disorder must be treated immediately, and the patient should stop taking anticoagulants. (2,3)
In this case, the underlying cause was not found, which can be difficult in a rural setting. One must keep in mind the broad differential diagnosis of the underlying diseases and start, as in this case, a broad systemic treatment if there are not enough resources to confirm a diagnosis.
References
- Brandt MT, Haug RH. Traumatic hyphema: a comprehensive review. J Oral Maxillofac Surg 2001; 59:1462.
- Walton W, Von Hagen S, Grigorian R, Zarbin M. Management of traumatic hyphema. Surv Ophthalmol 2002; 47:297.
- McDonald CJ, Raafat A, Mills MJ, Rumble JA. Medical and surgical management of spontaneous hyphaema secondary to immune thrombocytopenia. Br J Ophthalmol 1989; 73:922.
- Arentsen JJ, Green WR. Melanoma of the iris: report of 72 cases treated surgically. Ophthalmic Surg 1975; 6:23.
- Blanksma LJ, Hooijmans JM. Vascular tufts of the pupillary border causing a spontaneous hyphaema. Ophthalmologica 1979; 178:297.
