Spontaneous microscopic hyphema secondary to iris vascular tufts: Case report with video documentation.

A 71-year-old woman presented with spontaneous microhyphema in her left eye, causing blurry vision. Bleeding stopped spontaneously shortly after several cycles of digital compression on the upper eyelid, (which were documented in video), and therefore, did not require laser photocoagulation, a possible approach previously explained to the patient. A microhemangioma at the edge of the iris was identified to be the cause of the condition. The hemorrhage did not recur during the follow-up period (9 months).

Spontaneous hyphema is very unusual condition.[123456789] When there are no obvious iris vascular tumors or rubeosis, and certain systemic conditions (like sickle cells disease or coagulopathies) or excessive anticoagulation are not present, a frequent cause of spontaneous hyphema are iris microhemangiomas (iris vascular tufts).[12345689] Herein we present a video recorded case of spontaneous microhyphema related to iris microhemangiomatosis.

Case Report

A 71-year-old female attended the emergency department referring 2 days of blurry vision in her left eye (OS), without other ocular symptoms. She denied diabetes mellitus, ocular trauma, and other ocular or systemic diseases. Her OS presented a continuous filiform hemorrhage originating from the pupillary margin (at 12 o'clock position), without any signs of rubeosis iridis. Anterior chamber was full of erythrocytes floating in the aqueous humor with no visible layered blood (microhyphema or grade 0 hyphema). Intraocular pressure (IOP) was normal (OS = 14 mmHg). Pupil dilation was performed, however the bleeding did not stop [Fig. 1 and Video 1]. Fundus examination did not show retina abnormalities. The patient was advised to rest at home in semisitting position during the night and return the next day. Around 18 h after the initial examination, with a nondilated pupil, the steady hemorrhage continued to flow from the same point [Fig. 2 and Video 2]. Initially, pressure applied with a four-mirror goniolens for approximately 15 s was not successful [Video 3]. Then, digital compression on the superior eyelid was performed for 1 min with interruption of the bleeding during the maneuver; however, when the pressure was released, it reactivated. The compression time was sequentially increased until reaching 6 min, but the result was the same. These procedures were video documented [Video 4]. Compressive maneuvers were abandoned and the possible approach of laser photocoagulation was considered. However, around 1 h later, the bleeding spontaneously halted, while the patient was in the waiting room; therefore, photocoagulation was not required.

Figure 1

OS: Slit-lamp photograph, dilated pupil, 1 h after initial consultation. Active bleeding is flowing from the pupillary margin at 12 o'clock. Close-up image (right) shows bleeding apparently coming posteriorly from the iris though the effect was due to a partial pupil margin fold back, which hid the pupillary ruff

Figure 2

OS: Photograph taken in the slit lamp around 18 h after initial consultation. Active hemorrhage persisted from the pupillary margin at 12 o'clock, but with miosis, it was evident that the origin of the blood was located in the anterior iris surface, in the stroma adjacent to the pupillary ruff

One week later, there was no evidence of bleeding recurrence. A very small reddish vascular anomaly was visible in the iris at 12 o'clock position, near to the pupillary margin [Fig. 3]. Gonioscopy did not reveal any abnormal vessels in the angle. There was no recurrence over a 9-month period. In the last examination, other four small microhemangiomas were found, visible in the pupillary margin between 8 and 10 o'clock positions. We carefully checked again the pictures taken 3 days after the initial consultation and discovered that the lesions already existed, but they were extremely small, and had grown, especially one of them [Fig. 4]. We recommended the patient to consult in case of having again similar visual symptoms.

Figure 3

OS: One week later a small, irregular, vascular lesion (iris microhemangioma, Cobb's tuft or iris vascular tuft) was observed at 12 o'clock position, in the anterior iris stroma adjacent to the pupillary margin (marked with yellow circles). Details are better seen in the close-up image on the right

Figure 4

OS: Nine months later (right), besides the superior (red arrows), four other microhemangiomas were detected (black and yellow arrows). A new revision of the initial photograph (left) showed that the lesions already existed though smaller. The four originally overlooked lesions seemed larger, especially one of them (orange arrow)

Discussion

Spontaneous hyphema was first described by Bell in 1806.[1] Most cases are associated with microhemangiomatosis of the iris.[234568910] However, spontaneous hyphema has also been associated with various ocular conditions including other vascular tumors of the iris (such as racemose, cavernous, and capillary hemangiomas), rubeosis, and uveitis.[28] In addition, they have likewise been related although very rarely with hematological alterations such as thrombocytopenia, xanthogranuloma, sickle cell disease, leukemias, lymphomas, and excessive anticoagulation.[7]

Iris microhemangiomatosis, also known as iris vascular tufts or Cobb's tufts, are benign lesions that usually occur in or close to the pupillary margin, consisting of clusters of narrow coiled vascular loops and are frequently bilateral. Age of presentation varies between 40 to 80 years. Pathogenesis and prevalence remain unknown; however, systemic associations including diabetes mellitus, myotonic dystrophy, Sturge–Weber syndrome, hemangioma of the orbit or eyelid, or cerebral aneurysms have been reported.[9] Patients are usually asymptomatic until blurry vision due to spontaneous hyphema presents. This may also be accompanied by high IOP.[2345689]

Video documentation of an active bleeding episode has been rarely published.[9] In our case, the patient did not have systemic comorbidities and iris vascular tufts were evident only in one eye. We were able to record in video the entire process: the active hemorrhage at the initial consultation and the maneuvers performed to stop it [Videos 1–4].

When bleeding occurs, laser photocoagulation has been used by some authors as the first therapeutic option, but since usually other less invasive measures are effective, probably it could be reserved for nonrespondent or recurrent hemorrhages, as those reported by Sarmad et al., Strauss et al., Goyal et al., Williams et al., and Ni et al.[45689]

Spontaneous hyphema secondary to iris tufts, usually only require medical treatment to either treat or avoid IOP spikes. When active bleeding occurs, simple measures, such as digital pressure which we used in the present case, may halt the hemorrhage. Direct pressure exerted by the finger on the eyelid was more effective than compression achieved with a gonioscopy lens in the patient herein reported. Both failed and successful results have been published using compression gonioscopy.[39] However, in our case, active bleeding ceased around 1 h after the digital compression; therefore, we may not clearly establish whether these maneuvers did help. However, since they stopped the active bleeding at least temporarily [Video 4], we believe that performing them repeatedly could have eventually helped the blood hemostasis process to occur and finally achieved to control the hemorrhage definitively. Since photocoagulating lesion at the pupillary margin have, at least theoretically, the risk of causing a macular injury, we preferred less invasive approaches, and if the bleeding recurs in the future, we will think about using a 3-mirror Goldman lens to apply laser energy.

Conclusion

Spontaneous microscopic hyphema can be caused by iris vascular tufts. These patients may present with sudden blurring of vision, and mostly need conservative medical management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.