Aniridic Fibrosis Syndrome

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Disease Entity

Aniridic fibrosis syndrome is a rare complication of intraocular surgery (especially after cataract surgery with intraocular lens implantation) that can occur in patients with congenital aniridia. It is characterized by a fibrotic membrane that originates at the rudimentary iris root and can extend over the intraocular lens (IOL), ciliary body, and anterior retina[1][2][3].

Etiology

The etiology of aniridic fibrosis syndrome is unknown. All cases have been reported in patients with congenital aniridia who have undergone intraocular surgery[1][2][3].

Risk Factors

Risk factors for aniridic fibrosis syndrome are unestablished. However, 88% of reported cases have been female, and all have undergone intraocular surgeries. The large majority of affected patients have had cataract extraction with IOL insertion. Many patients also have other intraocular hardware including glaucoma drainage implants[1][2][3].

Primary prevention

Patients with congenital aniridia frequently have poor baseline visual acuity, and often must undergo multiple ocular procedures to address sequela of their underlying condition. However, as cases are associated with multiple procedures and intraocular hardware, limiting intraocular devices or surgeries as appropriate may help to prevent this rare complication.

Diagnosis

Diagnosis of aniridic fibrosis syndrome is made by clinical observation in patients with congenital aniridia and previous intraocular surgery

History

The chief complaint in patients with aniridic fibrosis syndrome is a decrease in visual acuity from baseline. The process is painless and gradual. Observant individuals may notice the membrane in more advanced disease[1][2][3].

Physical examination

On slit lamp examination a fibrous membrane should be observed originating at the iris root and affecting the IOL if present. If left untreated, or in presentations with late disease, the IOL may be pulled forward into contact with the cornea. Additionally, the membrane may extend to cover the ciliary body and retina which may cause hypotony and retinal detachment respectively. Finally, endothelial decompensation may be present[1].

Differential diagnosis

The differential for patients with aniridic fibrosis syndrome involves other causes of intraocular membranes, postsurgical inflammatory response, and associated sequela of the underlying aniridia[1][3][4].

Management

Surgery

Early surgical membranectomy via penetrating keratoplasty is recommended to prevent further membrane growth and destruction. As recurrence of this postsurgical complication has only been noted in patients who underwent membranectomy and IOL exchange, IOL membranectomy and IOL explantation may help to prevent recurrence[1]. The Boston KPro type 1 has not been associated with recurrence[3] and may be a viable treatment option. Surgical follow up is necessary to assess for recurrence.

Prognosis

Early surgical intervention may allow patients to return to, or close to, their baseline visual acuity[3]
  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Tsai JH, Freeman JM, Chan CC, et al. A progressive anterior fibrosis syndrome in patients with postsurgical congenital aniridia. Am J Ophthalmol. 2005. doi:10.1016/j.ajo.2005.07.035
  2. 2.0 2.1 2.2 2.3 Kothari M, Rao K, Moolani S. Recurrent progressive anterior segment fibrosis syndrome following a descemet-stripping endothelial keratoplasty in an infant with congenital aniridia. Indian J Ophthalmol. 2014. doi:10.4103/0301-4738.128635
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Bakhtiari P, Chan C, Welder JD, De La Cruz J, Holland EJ, Djalilian AR. Surgical and visual outcomes of the type i Boston Keratoprosthesis for the management of aniridic fibrosis syndrome in congenital aniridia. Am J Ophthalmol. 2012. doi:10.1016/j.ajo.2011.10.027
  4. Pavilack MA, Foster CS, Kowal VO, Dutt JE, Albert DM. Peripseudophakic Membrane: Pathologic Features. Arch Ophthalmol. 1993. doi:10.1001/archopht.1993.01090020094031