Spontaneous unilateral hyphema from a strand of persistent pupillary membrane.

A 48-year-old female presented to us with sudden onset dimness of vision in her right eye (RE). There was no history of trauma. Her best corrected visual acuity (BCVA) was 6/9 (partial) (Snellen's) in her RE and 6/6 in her left eye (LE). Her RE showed strands of PPM crossing the pupil. There was active bleeding seen from a point source on one of the strands of the PPM resulting in hyphema formation, 3 mm in height [Fig. 1]. The patient was seen and pupillary dilatation had been done at a local clinic and thus her RE was pharmacologically dilated at presentation. Both eyes had nuclear sclerosis grade II cataract and the intraocular pressures (IOPs) were 16 mmHg and 14 mmHg in the RE and LE, respectively. Gonioscopy showed wide open angles without any new vessels at the angle in both eyes. Fundus examination of both eyes was normal.

Figure 1

This is a photograph of the right eye of the patient showing clear cornea, dilated pupil with strands of PPM crossing the pupillary axis. Active bleeding can be seen from a point source on one of the strands of the PPM (white arrow). The resultant hyphema, 3 mm, due to the bleeding from the strand of PPM can be seen in the inferior aspect of the anterior chamber (white arrow with black outline). Plasmoid aqueous can also be noted in the anterior chamber

Her systemic examination was normal with normal blood pressure and random blood sugar levels. Gentle compression of the right eye with a pinkie-ball for 3 min was done and the bleeding stopped completely. She was prescribed topical steroids (Loteprednol etabonate 0.5% w/v) four times daily and a mydriatic/cycloplegic eye drop twice daily in the RE for 7 days. She was advised routine blood tests: complete hemogram, fasting and post-prandial blood sugar, serum urea and creatinine, lipid profile and coagulation profile and advised follow-up after 1 week with the reports. At her follow-up after 1 week her BCVA was 6/6 in both eyes and there were no signs of hyphema or active bleeding. PPM in RE was noted as fine strands crossing the pupillary axis [Fig. 2]. Her blood work-up was normal. She refused to undergo an iris angiography since it was an invasive procedure. At her next follow-up visit after 1 month she was asymptomatic with no recurrence of bleeding from the PPM.

Figure 2

These photographs show both the eyes of the patient at one week follow-up. In the right eye, strands of PPM can be seen crossing the pupillary axis (white arrows). There is no active bleeding noted from the PPM strands and the hyphema has completely resolved (a). The left eye is normal with no evidence of any PPM (b)

The pupillary membrane is a transitory vascular connective tissue covering the anterior surface of the lens during embryonic development. By eight and a half months, the central arcades gets fragmented and disappear. Clinically the remnants of the pupillary membrane are often seen as white strands, in 17.6–31.9% of the cases, but persistent functional vessels are found in only 0.3% which may very rarely bleed.[1234] Our case presented with active bleeding from such a PPM strand.

Spontaneous hyphema in the anterior chamber is rare and can occur due to certain reasons like intraocular neoplasms, blood disorders (leukemia, hemophilia), uveitis, rubeosis iridis, fibrovascular membranes in retrolental or zonular area (retrolental fibroplasia, persistent primary vitreous, retinoschisis), juvenile xanthogranuloma, occult trauma or trauma with late effect, hydrophthalmos, vascular anomalies of the iris, histiocytosis X, post-glaucoma surgery and very rarely from PPM.[1234]

Literature search revealed that there are very few cases of bleeding from a strand of PPM in adults and it is extremely rare.[12345] Some case reports have documented an iris angiography which showed the anomalous vasculature or aneurysmal dilatation within the PPM strand.[2345] In most of the previously reported cases a predisposing factor, either strenuous effort immediately preceding the bleeding[12] or systemic hypertension[34] contributed in the causation of hyphema; however, Kotamarthi et al. reported the only case of truly spontaneous hyphema similar to our case.[5]

This case illustrates that spontaneous bleeding may occur from a strand of PPM. To our knowledge, this is the first case of truly spontaneous bleeding from a strand of PPM being reported from India.

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