Hyphema is defined as the presence of blood in the anterior chamber. It is one of the most common sequelae of blunt injury to the eye and its presence can signify major damage to the eye’s blood vessels and intraocular structures. The blood may fill a portion of the anterior chamber, partially obstructing vision, or may entirely fill the anterior chamber and cause severe vision loss. A microhyphema occurs when there is no layering of blood, but red blood cells are seen within the anterior chamber. An 8–Ball Hyphema is the result of a large amount of clotted, deoxygenated blood often giving a purple/black appearance [2].

Hyphema is most commonly caused by blunt trauma, from a projectile or blunt trauma to the anterior portion of the eye. The hemorrhage is thought to be caused by a tear of the iris and/or ciliary body after the sudden posterior displacement of the lens and iris during injury [3]. A tear at the anterior aspect of the ciliary body is the most common site of bleeding and occurs in about 71% of cases [4]. As with most traumatic injuries to the eye, males are affected more than females. Non-traumatic causes of hyphema include surgery, hemodynamic abnormalities, and bleeding of abnormal vessels (neovascularization) in the iris or angle.

Patients with hyphema typically present after blunt trauma with complaints of pain, photophobia, and visual acuity changes. Hyphema is diagnosed by direct visualization of blood in the anterior chamber via penlight or slit lamp exam (Fig. 3.2). Because a hyphema may signify significant damage to the intraocular tissues, it is essential to perform a complete anterior and posterior examination in order to rule out the possibility of globe rupture. A B-scan ultrasound is often necessary, as the posterior segment may be obscured by blood, and a CT scan of the orbits may be indicated if the suspicion for open globe is high. Examination of the angle structures by gonioscopy is important to determine the severity of the blunt trauma that precipitated the hyphema. This is usually delayed until after the high-risk five-day rebleed period. Angle abnormalities and synechiae are commonly found. A useful way to grade the hyphema is by measuring its vertical height. This measurement can be repeated at each follow-up visit to monitor for resolution of the hyphema.

Increased intraocular pressure, as a result of red blood cells and fibrin clogging the trabecular meshwork, can occur in hyphemas of any size, and so tonometry must be performed on initial presentation and at each follow-up visit. It has been reported that a rise in intraocular pressure can occur in 32% of hyphemas, and patients with preexisting glaucoma are at higher risk [4]. For partial or microhyphemas, intraocular pressure is usually highest in the first 24 hours after injury and then normalizes after day 2. Larger or total hyphemas may cause intraocular pressure to be elevated for several days.

Secondary hemorrhage, or rebleeding after hyphema, may occur secondary to lysis of the clot which served to occlude the previously traumatized vessel. It can be seen in 25% of all patients with hyphema and typically occurs between 2 and 5 days after the initial trauma [5]. Studies have shown that rebleeds are seen more commonly in African American patients [6]. A rebleed is signified by an obvious increase in hyphema size during the follow-up period, and may result in vision threatening complications such as increased intraocular pressure, corneal blood staining, amblyopia and generally a worse visual prognosis [7].

Special consideration must be given to hyphema in the sickle cell patient, as the sickle-shaped red blood cells cannot effectively pass through the trabecular meshwork, leading to higher intraocular pressures for longer periods of time. These patients are also more prone to optic atrophy. Thus, it is important to obtain a sickle cell prep and hemoglobin electrophoresis in all patients with hyphema, and those positive for sickle cell disease or trait must be followed extremely closely.

The four complications of traumatic hyphema include: posterior synechiae, peripheral anterior synechiae, corneal blood staining, and optic atrophy.

Hyphema results in intraocular inflammation which may lead to the formation of posterior synechiae (iris adhesions to the lens), and/or peripheral anterior synechiae (iris adhesions to the cornea). Both situations can ultimately lead to angle closure glaucoma. Posterior synechiae, if substantial, may affect the movement of aqueous from the posterior to the anterior chamber causing secondary angle closure and iris bombe. Peripheral anterior synechiae may cause shallowing of the angle and blockage of outflow through the trabecular meshwork, also resulting in angle closure glaucoma.

Corneal blood staining is defined as the deposition of hemoglobin and its breakdown products into the cornea, and typically occurs in the setting of total hyphema with increased intraocular pressure. It has been reported that an IOP of 25 mm Hg or greater for more than 6–7 days increases the incidence of corneal blood staining. Patients with prior endothelial dysfunction are also at higher risk [4]. Corneal blood staining causes persistent reduction of vision, even after the hyphema has cleared, due to the presence of hemoglobin degradation products causing endothelial dysfunction. It is seen initially as a central yellow discoloration of the deep stroma which later spreads centrifugally to the periphery. The blood staining may extend to Bowman’s layer and even the epithelium in severe cases. Clearance of the blood staining begins peripherally and progresses centrally, and can take up to 3 years.

Optic nerve atrophy is one of the most dreaded complications associated with hyphema as the damage caused is irreversible. Glaucomatous optic neuropathy may result from chronically elevated intraocular pressure while diffuse optic nerve pallor and atrophy may result from the acute transient rise in intraocular pressure, or from the initial direct trauma to the optic nerve. The risk for glaucomatous optic neuropathy is highest if the IOP remains 50 mm Hg or greater for 5 days or 35 mm Hg or greater for 7 days for the general healthy population [3]. However, in a sickle cell patient, the risk of developing optic nerve atrophy is much higher, even when the IOP is lower than 35 mm Hg [8].

Management of hyphema is directed at reducing the incidence of rebleeding, and reducing the risk of corneal blood staining and optic atrophy. Traditionally, management included maintaining an atraumatic environment, which consisted of strict bed rest with the head in an upright position, bilateral patching, and sedation. More recent studies have shown that patients may remain ambulatory with a shield only on the injured eye, and achieve the same therapeutic results [9]. Medical treatment includes cycloplegic agents (such as atropine 1% solution, once daily) and topical steroids (prednisolone acetate 1%, four to six times daily) in order to control inflammation and prevent synechiae. If analgesics are needed, aspirin-containing products or NSAIDs are avoided as their antiplatelet effect can increase the risk of rebleeding [10]. Hospitalization may be considered in patients with severe trauma, or when noncompliance to the medical regimen is of concern.

Several studies have shown that systemic and topical aminocaproic acid (ACA) prevents rebleeding in patients with hyphema [11, 12]. ACA retards clot lysis by preventing plasmin from binding to lysine in the fibrin clot. The oral preparation, while effective, is not recommended for patients who are pregnant, or who have renal or hepatic insufficiency. The topical preparation appears to be just as effective in preventing rebleeds, with no systemic adverse effects [13].

Elevated intraocular pressure may be treated with topical IOP-lowering medications such as prostaglandin analogs, beta-blockers, or alpha-agonists. Oral carbonic anhydrase inhibitors may be needed if the intraocular pressure does not respond to topical therapy. In patients with sickle cell trait or sickle cell disease, where carbonic anhydrase inhibitors are contraindicated, methazolamide may be substituted.

While most hyphemas can be managed medically, some require surgical evacuation, or washout of the anterior chamber, in order to prevent severe vision loss. However, even total hyphemas can resolve spontaneously and it has been recommended to wait until after day 4 to surgically intervene. Surgery may be indicated in the following situations; microscopic corneal blood staining at any time, total hyphema with intraocular pressures of 50 mm Hg or more for 5 days (to prevent optic atrophy), total hyphemas or hyphemas filling greater than 75% of the anterior chamber present for 6 days with pressures of 25 mm Hg or more (to prevent corneal blood staining), hyphemas filling greater than 50% of the anterior chamber retained longer than 8–9 days (to prevent peripheral anterior synechiae), and patients with sickle cell trait or sickle cell disease who have hyphemas of ANY size that are associated with intraocular pressures of greater than 35 mm Hg for more than 24 hours. Surgery must also be considered in children who are at risk for developing amblyopia [6, 14].

The prognosis after hyphema resolution is mostly dependent on the associated injuries sustained from the initial trauma, and less so on the presence of rebleeding and the other described complications. Studies have shown that the majority of patients with decreased vision (<20/40) after hyphema are not the result of the hyphema itself, but from damage to the intraocular structures. This emphasizes the importance of hyphema as a marker for severe eye injury.

Symptoms of traumatic iritis occur within 24 hours of injury and consist of ocular pain, photophobia, tearing, redness, and decreased vision [16]. Irritation of the iris and its attachment to the anterior ciliary body causes spasm of accommodation, sustained miosis and is often associated with a poorly dilating pupil. However, sometimes a larger mydriatic pupil can be seen when the iritis is associated with an iris sphincter muscle tear. The presence of cell and flare (i.e., white blood cells and proteins) in the anterior chamber will be seen on slit lamp examination (Fig. 3.3). These findings, with miosis and photophobia, are the hallmark findings of traumatic iritis. Cells and flare are seen due to intraocular inflammation causing the breakdown of the blood–aqueous barrier. However, the anterior chamber reaction may be surprisingly minimal. Intraocular pressure is often found to be low due to decreased aqueous production from ciliary body shock; however, it can sometimes be increased due to damage to the trabecular meshwork or from clogging of the trabecular meshwork by inflammatory debris. A rise in intraocular pressure can cause a secondary glaucoma, and if it goes unnoticed, can lead to optic neuropathy and vision loss. Other diseases that can be easily confused with traumatic iritis are infectious and noninfectious causes of anterior uveitis such as HLA B-27 related and HSV uveitis. Careful history and exam will help differentiate between these entities.

Traumatic iritis is a self-limiting entity and usually resolves on its own within 7–14 days [16]. Topical corticosteroid use has been the standard therapy since the 1950s, although the clinical evidence to support its use is sparsely documented in the literature [17, 18]. Currently, most practitioners chose to observe mild cases of traumatic iritis with close follow-ups. For moderate to severe cases, most physicians initiate topical steroid treatment to avoid complications associated with prolonged inflammation. If steroids are continued for more than approximately 2 weeks, it is important to look for a steroid induced rise in intraocular pressure. The treatment for moderate-to-severe cases includes cycloplegic agents (homatropine or cyclopentolate) to decrease ciliary spasm and relieve ocular pain and decrease the formation of posterior synechiae, and topical corticosteroids (prednisolone acetate) to control inflammation.

Rare complications from traumatic iritis include cataract, synechiae, and glaucoma. While these complications may be the result of prolonged inflammation, it is also important to note that cataract and increased intraocular pressure often result from the long-term use of topical corticosteroids. Because of this, it is important to start topical corticosteroids in appropriate cases and taper the dose accordingly.

Classic clinical features associated with traumatic mydriasis include a fixed, dilated pupil with a diminished direct or consensual pupillary reaction to light and accommodative stimuli. The patient may complain of photophobia, as more light enters the pupil. The pupil may appear irregular and slit lamp examination may reveal small tears at the pupillary margin.

While sphincter tears and traumatic mydriasis can resolve after several weeks, they can often be permanent. Clinical and pathological studies have demonstrated failure of traumatic and surgical iris tears to heal spontaneously due to the absence of bridging iris stromal cells, fibroblasts, and pigmented cells to migrate between the wounded edges of iris, and to subsequently create a scar [19]. Treatment of mydriasis is largely dependent on symptoms of glare, diplopia, and rarely, on cosmesis. Medical management often includes use of miotics (i.e., pilocarpine or brimonidine), contact lenses, and sunglasses. However, due to the functional loss of the iris sphincter muscle, there is often minimal improvement with the use of miotic agents for traumatic mydriasis when compared to their use in paralytic or pharmacological causes of mydriasis. Colored contact lenses with a clear pupillary zone and opaque periphery are often used to hide iris defects and to artificially create a small iris pupillary diameter and minimize visual symptoms. The risks of contact lens wear can include infectious keratitis, and so these patients must be followed appropriately.

Surgical repair of traumatic mydriaisis is indicated when significant visual disturbance (glare, photophobia, and diplopia) is not correctable with medical management alone, when iris diaphragm support is needed for IOL placement, and rarely, for cosmetic reasons [19]. The standard and preferred surgical technique for permanent traumatic mydriaisis is Ogawa’s iris suture cerclage using a 10–0 Prolene running suture technique [20].

Management of Iridodialysis involves controlling associated symptoms. Sunglasses, tinted glasses, or colored contact lenses may help reduce symptoms of glare. If symptoms still persist and/or a large dialysis is present, then surgical repair should be considered. For sectoral iris defects or small iridodialysis (i.e., less than 3 clock hours), the McCannel Iris suturing method is the preferred technique [23]. This involves suturing the avulsed iris segment to the adjacent sclera and ciliary body junction using a 10-0 prolene or nylon suture. For larger iridodialyses and iris defects, prosthetic iris devices (PID) can be used. Burke et al., reported visual acuity improvement in 79% of patient using a PID. Although the use of such devices have been available in Europe for more than 15 years, they are still not FDA approved in the United States and are not widely available in other parts of the world [24].