Table 1.
Age | Sex | Ethnicity | Axial Length (mm) | Presence of Retrobulbar Cyst (Y/N) | Phenotype | FA | Pre-Sx BCVA | Pre-Sx Rx | Pre-Sx Function | Pre-Sx IOP | Last BCVA | Last Rx | Last IOP | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patient 1 | ||||||||||||||
OD | 3 years | M | Greek | 10.5 | Y | Chorioretinal coloboma, opaque microcornea 5.5 mm × 5 mm, microophthalmos, retrobulbar cyst | Not good image quality | NLP | Not possible | Noticed diminished vision | 10 mmHg | HM | Not possible | 19 mmHg |
OS | 21.64 | N | Chorioretinal coloboma, subluxated cataract, microcornea 7.5 mm × 6.5 mm, cataract | Coloboma: hypo;PFV: hyper;FAZ: present | 2/200 | Not possible | 24 mmHg | 20/60 (far)20/20 (near) | +17.25 | 9 mmHg* | ||||
Patient 2 | ||||||||||||||
OD | 3 years | F | Saudi Arabian | 24.2 | N | Chorioretinal coloboma, subluxated crystalline lens, microcornea 9 mm, microophthalmos, closed angles. |
Coloboma: hypo PFV: hypo; FAZ: not identified |
8/400 | Not possible | Non-independent | 27 mmHg | Fix and follow | Not possible | 18* |
OS | 28.2 | N | Not done | Fix and follow (difficult) | Not possible | 50 mmHg | Fix and follow | Not possible | 28* | |||||
Patient 3 | ||||||||||||||
OD | 6 weeks | M | African American | 19.8 | N | Chorioretinal coloboma, subluxated crystalline lens, microcornea 7 mm, microophthalmos, closed angles. | Coloboma: hypo PFV: stalk hypo, anterior insertion hyper | Fix and follow | −12 D | Plays and uses toys | 16 mmHg | HM | Not possible | STP |
OS | 22.4 | N |
Coloboma: hypo PFV: stalk 6hypo; anterior insertion hyper; FAZ: present |
20/800 | −12 D | 22 mmHg | 20/250 ecc | Not possible | STP |
Summarized information of the pre and post surgical functional status of our three cases with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microophthalmia
BCVA-best corrected visual acuity; EL-endolaser; FAZ-foveal avascular zone; HM-hand motion; IOP- intraocular pressure; IVB- intravitreal bevacizumab; NLDO- nasolacrimal duct obstruction; NLP- no light perception; PPL-pars plana lensectomy; PPV- pars plana vitrectomy; Rx-refraction; STP- soft to palpation; STT-sub-tenon's triamcinolone; Sx-surgery.
*using glaucoma eyedrops.
** 3 months of follow up at BPEI. Now IOP control in Saudi Arabia by glaucoma specialist.