It is a rare, idiopathic, chronic, low-grade iridocyclitis, without synechiae, with heterochromia, and with low-grade anterior chamber reaction with diffuse, small, stellate keratic precipitates, posterior subcapsular cataract, and secondary open-angle glaucoma. Glaucoma is believed to be the major long-term threat to vision, generally persisting after uveitis has subsided. Treatment of the inflammation with corticosteroids may further increase the IOP. Medications, particularly aqueous suppressants, may be effective in controlling glaucoma initially, although the glaucoma is resistant to medical therapy and may require surgery when chronic [9, 10].

It is also known as glaucomatocyclitic crisis and presents typically with unilateral recurrent episodes of mild cyclitis and heterochromia. Though inflammatory signs may be minimal, rise of IOP may be in the range of 40–70 mmHg during an acute attack. There may be a relationship between this syndrome and the development of primary open-angle glaucoma [11]. Vascular incompetence due to segmental iris ischemia is associated with the release of prostaglandins, inflammation, and a subsequent rise in IOP. Although the pathogenesis of Posner-Schlossman syndrome remains unknown, possible associations include an immunogenetic factor involving HLA-Bw54 [9], viral infections (herpes simplex and cytomegalovirus) [12], gastrointestinal disease, and various allergic conditions [13]. The prognosis for control of IOP in patients with glaucomatocyclitic crisis is good [14]. Currently, the favored initial treatment is a topical nonsteroidal anti-inflammatory drug (NSAID) to control inflammation [14]. Topical steroids, oral NSAIDs, or carbonic anhydrase inhibitors may also be used.

The prevalence of glaucoma in JIA-associated uveitis has been reported in the range of 14–27 % [15]. Patients with persistent low-grade inflammation are at greatest risk for developing glaucoma and an aggressive approach to treatment may reduce the risk of blindness. In JIA, glaucoma commonly occurs with open angles but may be of pupillary block, secondary angle-closure type, as a result of formation of posterior synechiae.

Medical management includes topical steroids, cycloplegics, regional steroid injections, and brief systemic steroid therapy. An oral NSAID may be used if inflammation recurs with steroid withdrawal. Immunomodulatory therapy with methotrexate has a high efficacy and low toxicity. Biologic response modifiers may also be tried in treatment failures. The management of glaucoma may be medical or surgical. Medical treatment is with topical beta blocker and sympathomimetics. Carbonic anhydrase inhibitors and prostaglandin analogues may be added if required [1]. However, the use of PG analogues can often result in an inflammatory response and they should be withheld as a last resort.

Glaucoma surgery secondary to uveitis presents challenges as the postoperative inflammatory response is often magnified and complicates both control of the uveitis and IOP. Although goniotomy surgery is successful in many cases of childhood uveitic glaucoma, it was found to be less successful in eyes that were aphakic or eyes that had peripheral anterior synechiae [16]. In eyes with significant peripheral anterior synechiae and a predominantly closed angle, a glaucoma drainage device as initial surgery may be considered. Thus, in determining appropriate surgical management of uveitic glaucoma, it is important to take into consideration whether the angle is open or not and the extent of synechia formation.

Compared with other surgical options, goniotomy offers higher rates of success, with fewer risks of complications such as infection, exacerbation of uveitis, or hypotony. It requires less operating time and preserves conjunctiva for future interventions. Goniosurgery is considered an effective, low-risk surgical intervention in the face of failure with maximal medical therapy. However, it requires expertise, a predominantly open angle, and a gonioscopic view, which may be difficult in cases of elevated IOP or severe band keratopathy. The placement of a glaucoma drainage device, particularly a valved implant, offers immediate IOP reduction and can be considered in eyes with significant synechia formation or those that immediately failed goniotomy. Glaucoma drainage devices are standard treatment for adult uveitic glaucoma [17–19]; however, studies on their use in children are limited by small patient numbers and relatively short follow-up [20, 21]. Problems with drainage devices include strabismus, corneal decompensation, increased inflammation, cataract, and pupil peaking [22, 23]. It has been found that in eyes with adequate IOP and uveitis control after goniotomy or glaucoma drainage device placement, cataract removal did not exacerbate the glaucoma [24]. However, any surgical intervention for a child with glaucoma must consider a strategy that minimizes ocular damage while maximizing chances for vision preservation and IOP control over decades.

Secondary glaucoma is the most common complication in patients with herpetic uveitis [25]. An estimated 28–45 % of patients with herpes simplex virus (HSV) keratouveitis develop transient elevated IOP [26], and 10–54 % may present with secondary glaucoma [25].

An acute rise in IOP in the presence of active iridocyclitis is the hallmark of a herpetic etiology [27]. These hypertensive episodes are attributed to inflammation of the trabecular meshwork (TM) [28]. This is supported by normalization of IOP in response to topical corticosteroids. The increase of IOP can also be secondary to swelling and obstruction of the TM by inflammatory cells and debris. Synechia formation may be seen typically during an episode of severe herpetic iridocyclitis [29, 30].

Inflammation of cells in the TM (trabeculitis) due to HSV-1 infection during corneal endotheliitis and uveitis is a major risk factor for glaucoma. Immunoreactivity for HSV-1 in the trabeculum has been demonstrated to induce inflammation of human TM cells, which impedes aqueous outflow and increases IOP. Cultured human TM cells are susceptible to HSV-1 entry and replication. HSV entry into cells is a complex process that is initiated by specific interactions of viral envelope glycoproteins and host cell surface receptors. Three classes of HSV entry receptors have been identified. They include herpes virus entry mediator (HVEM), nectin 1 and 2, and isoforms of 3-O-sulfotransferases. The relative abundance of the receptors varies with cell type, and this variation might influence the course of HSV-1-related trabeculitis [31].

Management of glaucoma secondary to herpetic uveitis includes long-term antiglaucoma therapy and surgery may be warranted after the active inflammation has subsided. Along with the management of glaucoma, long-term prophylactic antiviral therapy is required to prevent recurrences [32, 33].