Congenital Aniridia: Clinic, Genetics, Therapeutics, and Prognosis

Table 1

Results of studies with keratoprosthesis.

Authors	No. Patients /number of eyes	Age (years)	Complications (intraoperative; /follow-up)	Best Corrected Visual Acuity			Best final corrected visual acuity	Associated procedures /number of patients	% prosthesis retention	Follow-up (months)
				Improvement	Without improvement; /worsening	Worsening
Akpek et al. [10]	15/16	25–66; Median: 50	0/1 glaucoma [GW] + 3 CD + 1 RD [after CD] + 2 RPM + 1 CTM^a	14	1	0	HM to 20/60; Median: 20/200	LE, IOLE, GDD, PPV/10	100	2–85; Median: 17
Bakhtiari et al. [14]	9/9	11–71; Mean: 33,8	0/1 RD + 1 SH + 5 RPM	9	0	0	2/500 to 20/200	IOLE/7; PPV/9	100	6–48; Mean: 26,1
Rixen et al. [15]	7/7	12–85; Mean: 52	0/1 glaucoma [GW] + 1 WD + 3 RPM	6	0	1^b	LP to 20/100; Median: 20/200; (with spectacles)	GDD/4; GDD revision/1; LE/1; IOLE/2; PPV/3	100	3–30; Median: 18
Greiner et al. [16]^c	4/5; (35/40)	(2–86; Mean: 52,9)	(0/22 RPM + 34 glaucoma + 5 endophthalmitis + 6 CTM + 6 keratoprosthesis extrusion)	2	3		2 eyes maintained VA ≥ 20/200; 2 eyes had initial VA improvement that was not substantial (did not maintain VA ≥ 20/200); 1 eye never achieved VA ≥ 20/200	(LE/13; pupilloplasty/1; tarsorrhaphy/1; GDD/3; AMT/1; AV/1)	80; (80)	(5–72; Mean: 33,6)
Kang et al. [17]^cd	5/5; (19/21)	(27–83; Mean: 51,9)	(0/8 glaucoma [3 GW + 5 IOPE + 3 CD + 2 PED + 1 endophtalmitis + 7 MO + 3 VH + 2 EM + 10 RPM + 1 CTM + 2 LLR)	(19)	(2)		(6 patients—≤20/200; 11 patients—≥20/200; 4 patients—≥20/50)	(LE/14; iridectomy/7; IOLE/3; tarsorrhaphy/3; GDD/2; AV/8; PPV/3)	80^e; (90,5)	(6–36, 3; Mean: 14,6)

AMT (amniotic membrane transplant); AV (anterior vitrectomy); CD (choroidal detachment); CTM (corneal tissue melt); EM (epiretinal membrane); GDD (glaucoma drainage device implantation); GW (glaucoma worsening); HM (hand movement); IOLE (intraocular lens extraction); IOPE (intraocular pressure elevation); LE (lens extraction or cataract removal); LLR (lower lid retraction); LP (light perception); MO (macular oedema); PED (persistent epithelial defects); PPV (pars plana vitrectomy); RD (retinal detachment); RPM (retroprosthetic membrane); SH (suprachoroidal hemorrhage); VH (vitreous hemorrhage); WD (wound dehiscence).
^aThis patient received an older type of the keratoprosthesis which justified that complication. After that he had a scleral patch reinforcement of the prosthesis and there was no need to extract the device neither affectation of VA.
^bInitially, the patient had VA improved with BCVA 20/300 with spectacles but suffered a massive occipital hemorrhagic stroke which the authors point to be the reason for the final VA (light perception only).
^cThis study used patients with keratopathy who had different diagnoses (including patients with aniridia). When specific data for aniridic patients was available, it is shown in the table. Otherwise, data from the overall patients is used and is specified between brackets.
^dIn this study, the keratoprosthesis was used as the primary penetrating corneal procedure.
^eThe aniridic patient who had not retained the prosthesis developed corneal tissue melt surrounding the device as a complication. Further on he had two more replacement procedures.