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. 2020 Oct 1;15(10):e0240016. doi: 10.1371/journal.pone.0240016

Table 3. Overview of visual function in children with craniopharyngioma at diagnosis.

Study Children with availability of vision data Visual disturbance as symptom Visual impairment Decreased VA with description Decreased VF with description Orthoptic examination Fundoscopy Other vision related defects Ophthalmological examination Ophthalmological definitions
1 Al-Mefty, 1985 [99] VA: 15/20 NR NR 15/15: Bitemporal defects 6/9, only central VF 1/9 NR Papilledema 7, optic atrophy 7, Foster-Kennedy syndrome 3 NR NR NR
VF: 9/20 Severe decrease in both eyes 8/15, totally blind in both eyes 5/15, totally blind in one eye with decreased VA in the other eye 2/15
2 Albright, 2005 [51] NR NR Intracavitary irradition P-32: 4/44 NR NR NR NR NR NR NR
Microneurosurgical tumor resection and GKSR: VI was one of the predominant neurological symptoms
3 Ali, 2013 [54] NR 2/7 4/7 Blurry vision 1/7 NR Diplopia 1/7 NR NR Yes (not specified) NR
4 Ammirati, 1988 [71] 2/3 1/3 2/3 2/3: Bitemporal hemianopia 2/3 NR NR NR NR NR
VA 20/100: 1/4 eyes, VA 20/200: 1/4 eyes, LP: 1/4 eyes
5 Anderson, 1989 [53] 2/2 1/2 2/2 2/2: Homonymous hemianopia 1/2, temporal hemianopia 1/2 NR NR NR NR NR
VA 20/25; CF at 1 foot 1/2, VA: 20/300; CF at 2 feet 1/2
6 Ansari, 2016 [62] NR 5/9 5/9 NR NR NR NR NR NR NR
7 Artero, 1984 [107] VA: 21/24, VF: 18/24 5/24 22/24 Decreased VA 17/21 16/18: Diplopia 2/24 Abnormal ocular fundus 19/24, optic atrophy or pallor 14/24 (unilateral 6/24), papilledema 7/24 NR VA and campimetric determinations, funduscopy and examination of ocular motility when the age and/or clinical condition allowed them to be performed. NR
Homonymous defects 5/18, temporal defects 14/18 (of whom 7/18 had bitemporal defects)
8 Ashkenazi, 1990 [82] NR 5/12 NR Decrased VA 5/12 Reduction 3/12 NR NR NR NR NR
9 Bartlett, 1971 [35] NR 23/30 NR NR NR NR Papilledema 13/30 NR NR NR
10 Behari, 2003 [81] 2/2 2/2 2/2 2/2 NR Bilateral sixth nerve palsy 1/2 Bilateral papilledema 1/2 NR NR NR
6/24: 1/4 eyes, 6/18: 2/4 eyes, 6/12: 1/4 eyes
11 Bialer, 2013 [84] 13/20 1/11 4/11 ≤ 20/200 in at least one eye 7/11 Bilateral temporal 4/15, unilateral temporal 3/15, right inferior homonymous quadrantopia 1/15 RAPD 8/13, unilateral exotropia 6/13, sixth nerve palsy 2/13, monocular nystagmus 1/13, diplopia 3/13 Papilledema 3/13, optic disc pallor 10/13: bilateral 7, unilateral 3 NR BCVA, Humphrey Field Analyzer NR
12 Boekhoff, 2019 [70] NR Symptomatic CP 54/214 Symptomatic CP 54/214, Incidental CP 1/4 NR Incidental CP: impaired VF right side 1/4 NR NR NR NR NR
13 Cai, 2019 [76] NR NR 3/5 NR NR NR NR NR VA and VF testing. Visual outcome was graded as improved, stable, or deteriorated.
14 Caldarelli, 2005 [90] NR NR 17/52 Reduction of VA 13/52 9/52 Sixth nerve deficit 5/52, third nerve deficit 1/52, nystagmus 2/52 NR NR NR NR
15 Capatina, 2018 [106] NR NR NR 22/35: 18/35 NR NR NR Yes (not specified) NR
Defect 11/35, decrease 15/35, uni- or bilateral blindness 7/35
16 Chamlin, 1955 [52] NR NR NR Loss of central VA 12/18 Bitemporal hemianopia 18/18 NR Optic atrophy 14/18, papilledema 4/18 Proptosis 1/18 VF, optic discs, central VA (Snellen) and other ocular signs (extraocular muscle palsies, pupillary changes, involvement of NV, papilledema, proptosis, nystagmus) As an indication of visual loss they took a very definite drop in VA (e.g. from a known 20/20 to 20/40 or less), or a reliable statement from the patient that his vision was definitely failing.
17 Chen, 2003 [6] 16/17 15/17 13/17 < 6/12: 6/17 (40%), ≥ 6/12: 10/17 (60%) Bitemporal hemianopia 9/17, unilateral hemianiopia 1/17, homonymous hemianopia 1/17, normal VF 3/17, data NA 3/17 Strabismus 3/17, RAPD 10/17 Bilateral (optic) atrophy 10/17, bilateral papilledema 4/17 NR BCVA, Ishihara colour testing, RAPD, fundoscopy, cranial nerve examination, perimetry testing with Humphrey field analyser, Goldman perimetry or Bjerrum screen. Normal if BCVA ≥ 6/12
18 Cherninkova, [1990] [102] NR NR NR Reduced VA 32/46. Bilaterally reduced VA: under 0.1 6/50; over 0.1 17/50, amaurosis in one eye and reduced VA in the other 2/50, amaurosis in one eye and normal VA in the other 3/50, bilateral amaurosis 1/50, reduced VA in one eye and normal in the oter 3/50 VF defects 21/31. Bitemporal hemianopia 5/31, amaurosis of one eye and temporal defect of the other eye 3/31, bilateral temporal narrowing of perimeters 5/31, homonymous hemianopia 2/31, bilateral concentric narrowing of the visual field 5/32, other defects 1/31 Nystagmus 4/50, paresis of cranial nerve 6/50 Optic nerve atrophy: unilateral 7/50, bilateral 19/50, congestive optic papilla 11/50 NR Ophthalmological examinations were performed by routine methods. In small children and patients in a serious condition a thorough study was not always possible. NR
19 d’Avella, 2019 [91] NR 7/8 NR Left eye VA reduction 1/8 Bitemporal hemianopia 3/8, bilateral superior quadrantopia 1/8, right temporal hemianopia 1/8 NR NR Right amaurosis 1/8 VA, computerized VF examination. NR
20 Drimtzias, 2014 [72] VA: 20/20 12/20 12/20 11/20: 10/14: NR Optic atrophy 12/20, bilateral papilledema 6/20 NR BCVA (Logmar or Preferential Looking charts). VF with Goldmann perimetry VA: normal (grade 8), mild-moderate visual loss (grade 5, 6, 7), severe visual loss (grade 1, 2, 3, 4).
VF: 14/20 Mild-moderate 8/40 eyes, severe 13/40 eyes, normal 19/40 eyes bitemporal hemianopia 5/14
21 Erşahin, 2005 [101] NR NR NR Blindness 13/87, visual disturbance and decreased VA 21/87 10/87 Abducens paralysis 2/87, nystagmus 3/87, diplopia and squint 7/87 Papilledema 5/87, optic atrophy 29/87 NR NR NR
22 Fisher, 1998 [43] NR 19/30 19/30 Loss of VA 17/30 Loss of VF 14/30 NR NR NR NR NR
23 Fouda, 2019 [63] NR 56/135 56/135 Impaired VA 26/135 Impaired VF 39/135 NR Papilledema 51/135 NR NR NR
24 Gautier, 2012 [73] 53/65 40/53 40/53 Only reported together with VF 40/53 Only reported together with VA 40/53 NR NR NR NR Blindness: VA of 1/10 or less in both eyes.
25 Gerganov, 2014 [67] NR 1/1 1/1 NR NR NR NR NR Uniformly subjected preoperative ophthalmological assessment NR
26 Goldenberg-Cohen, 2011 [83] 3/4 2/4 3/4 3/4 NR Diplopia 1/4, esotropia 1/4, exotropia 1/4, mono-nystagmus 1/4 Papilledema 1/4, optic atrophy 2/4, mild pallor 1/4 NR BCVA and a comprehensive neuro-ophthalmologic evaluation. Severe visual loss was defined as counting fingers or less.
27 Gonc, 2004 [31] 64/66 23/66 NR 20/64: Bitemporal hemianopia 22/64 Diplopia 3/66 Optic atrophy 27/64, papilledema 26/64 NR NR NR
Unilateral 14/64, bilateral 6/64
28 Greenfield, 2015 [60] NR NR 21/24 VA and VF deficits 16/24, legally blind (BCVA <20/200) 1/24 VA and VF deficits 16/24 Diplopia 8/24 NR NR NR: only at follow-up. Legally blind if corrected VA <20/200 in the better eye.
29 Haghighatkhah, 2010 [104] NR NR 2/5 Visual loss 1/5, left eye blindness 1/5 NR NR NR NR NR NR
30 Hakuba, 1985 [86] NR 1/3 1/3 Failing vision 1/3 Bitemporal hemianopia 1/3 NR Papilledema 2/3 NR NR NR
31 Hoff, 1972 [37] 12/16 NR 7/12 NR NR NR Papilledema 2/12, optic atrophy 7/12 NR Satisfactory eye examination in 12/16 children. NR
32 Hoffman, 1977 [25] NR 47/48 47/48 Significantly reduced unilaterally 17/48, significantly reduced bilaterally 11/48 Hemianopia 33/48: bitemporal 25/48, homonymous 4/48, unilateral temporal 4/48 Seesaw nystagmus 3/48 Papilledema 13/48 NR NR VA ≥ 20/40 bilaterally (mild visual loss); VA < 20/40 in one eye (moderate visual loss); VA < 20/40 bilaterally (severe visual loss).
33 Hoffman, 1992 [89] NR 29/50 29/50 Decreased in one or both eyes 21/50, blind in one eye 4/50 19/50: Diplopia 4/50, seesaw nystagmus 2/50 NR NR No NR
bitemporal hemianopia 8/50
34 Hoffmann, 2015 [68] 130/411 161/291 NR NR NR NR NR NR NR NR
35 Honegger, 1999 [26] NR 10/30 NR NR NR NR NR NR NR NR
36 Hoogenhout, 1984 [93] NR 7/12 NR NR NR NR NR NR Visual fields NR
37 Im, 2003 [97] NR 5/6 NR NR NR NR NR NR NR NR
38 Jane Jr., 2010 [64] NR NR NR NR 4/11 NR NR NR Formal visual field testing for patients with visual complaints. NR
39 Jung, 2010 [98] NR NR 4/17 NR NR NR NR NR Method used described by Fahlbusch and Schott to analyze ophthalmological findings (VA and VF). NR
40 Karavitaki, 2005 [48] 41/42 NR NR Decreased 16/39 19/41 VF defects: bitemporal hemianopia 11/41 NR Papilledema 12/41, optic atrophy 2/41 NR NR NR
41 Kennedy, 1975 [39] VA 12/14, VF NR NR NR Diminished vision 7/14 7/14 Strabismus 5/14; rotatory nystagmus 1/14 Optic atrophy 9/14, papilledema 6/14 NR VA, ocular movements, pupil reactions, ophthalmoscopy and VF testing using the Bjerrum screen. NR
42 Kiran, 2008 [79] 2/2 1/2 1/2 6/9 bilateral: 1/2 NR NR Optic atrophy 1/2 NR VA, pupils and fundus examination NR
43 Kramer, 1960 [38] 6/6 3/6 3/6 2/6: Bitemporal hemianopia 2/6, loss of right nasal field 1/6 Right vertical and left rotatory nystagmus 1/6 Papilledema 3/6, optic atrophy 3/6 NR Yes (not specified) NR
6/60: 1/6
44 Lee, 2008 [108] NR 21/66: NR NR NR NR NR NR NR NR
Intrasellar 15/27; third ventricular 6/39
45 Lena, 2005 [24] NR 32/47 32/47 Blindness: bilateral 2/47, unilateral 3/47 Pure VF defect 3/47, VF defect and decreased VA 23/47 NR Papilledema 13/47 NR Yes (not uniformly performed on all patients due to young age and emergency presentation). NR
46 Leng, 2012 [58] NR 1/3 1/3 NR NR NR NR NR Neuro-ophthalmological evaluation and formal visual field testing when possible. NR
47 Merchant, 2002 [65] NR 17/30 NR NR NR NR NR NR NR NR
48 Mohd-Ilham, 2019 [7] 11/11 4/11 4/11 BCVA ≥ 6/6-6/12: 13/22 eyes, BCVA 6/15-6/60: 3/22 eyes, BCVA < 6/60: 6/22 eyes Temporal hemianopia 5/22 eyes: bilateral 2/22 eyes, unilateral 3/22 eyes; scotoma 3/22 eyes: central 2/22 eyes, inferior 1/22 eyes; quadrantanopia 2/22 eyes; constricted 1/22 eyes. VF is NA in 5 patients Squint 2/11, diplopia 1/11, RAPD 7/11 Optic atrophy 11/11, papilledema 2/11 Color defect 4/11 BCVA, VF (confrontational test or Humphrey visual field test), color vision, light brightness, RAPD, fundus examination and cranial nerves examination. BCVA ≥ 6/12 (good) during presentation. Visual loss was defined as blurring of vision in both eyes.
49 Mottolese, 2001 [33] NR 2/20 2/20 NR NR NR NR NR NR NR
50 Nielsen, 2012 [103] NR NR NR Reduction 21/32, blindness 1/39 Reduction 12/26 NR NR Ophthalmoplegia 5/31 VA and VF testing. NR
51 Ohmori, 2007 [28] NR 15/27 (55%) NR NR NR NR NR NR For 21 patients only initial outcome data is available. 6 patients underwent extensive outcome analysis, including ophthalmological testing. NR
52 Ono, 1996 [87] NR NR 15/19. Mean visual score at diagnosis 68.4. NR NR NR NR NR VA and VF testing. Visual scores were assigned in order to evaluate visual functions digitally assessing both VA and VF (0–100 points). NR
53 Pascual, 2018 [55] NR 35/35 35/35 NR NR Diplopia 14/35, sixth nerve palsy 1/35 NR NR Yes (not specified) NR
54 Patel, 2017 [56] NR 7/10 7/10 NR NR NR Papilledema 1/10 NR NR NR
55 Puget, 2007 [14] NR RS cohort: 30/66 NR Blindness 10/66 NR NR NR NR NR NR
PS cohort: 14/22
56 Qi, 2012 [77] NR Group A: 34/34 57/81 Unilateral/bilateral blindness or light perception: NR NR NR NR NR NR
Group B: 24/47
- Group A: 12/34
- Group B: 3/47.
57 Quon, 2019 [57] NR NR 11/16 Vision loss 4/16, blurry vision 2/16 Bilateral hemianopia 1/16, bitemporal hemianopia 2/16; VF deficit 3/16 NR Papilledema 3/16, optic nerve compression 1/16 Proptosis 1/16 Complete work-up by an ophthalmologist when visual symptoms or signs were present. NR
58 Rath, 2013 [96] 10/10 4/10 7/10 Mild VA deficit or field cut 4/10, unilateral blindness, homonymous hemianopia or bitemporal hemianopia 2/10, bilateral blindness or near functional blindness (unrelated) 1/10 Mild VA deficit or field cut 4/10, unilateral blindness, homonymous hemianopia or bitemporal hemianopia 2/10, bilateral blindness or near functional 2 blindness (unrelated) 1/10 Right exotropia 1/10 Papilledema 1/10 NR NR 1) Normal acuity and fields (3/10); 2) Mild acuity deficit or field cut (4/10); 3) Unilateral blindness, homonymous hemianopia or bitemporal hemianopia (2/10); 4) Bilateral blindness or near functional blindness. (1/10)
59 Richmond, 1980 [61] NR 9/21 NR Decreased VA: bilateral 2/21, unilateral 7/21 (3 blind). Unilateral blind 3/21. 12/21 (3 of them had a combination of two findings). Temporal: unilateral 4/21; bilateral 3/21, homonymous 4/21, scotoma 1/21 NR Optic atrophy: unilateral 2/21; bilateral 5/21, papilledema 2/21 NR Yes (not further specified) NR
60 Salunke, 2016 [80] 2/2 2/2 2/2 2/2: Bitemporal hemianopia 2/2 NR NR NR NR NR
6/18: 2/4 eyes, 6/24: 1/4 eyes, PL plus 1/4 eyes
61 Sankhla, 2015 [27] NR 3/6 NR NR NR NR NR NR NR NR
62 Shammari, 2012 [100] NR 2/2 1/2 NR NR Rotatory nystagmus 1/2, horizontal pendular nystagmus 1/2 Temporal disc pallor both eyes 2/2 NR Ophthalmic records were reviewed. NR
63 Shi, 2017 [78] NR NR 99/348 NR NR NR NR NR NR NR
64 Sogg, 1977 [109] 2/2 2/2 2/2 2/2: Bitemporal hemianopia 2/2 NR Papilledema 1/2, optic pallor 1/2 NR VA and VF testing (red test and large white test objects; Goldmann perimetry), fundoscopy. NR
20/100: 1/4 eyes, 20/50: 1/4 eyes, 20/400: 1/4 eyes, 20/200: 1/4 eyes
65 Stahnke, 1984 [29] NR 12/28 24/28 Decreased VA 11/28 VF defect 16/28 NR Optic atrophy 11/28, papilledema 4/28 NR Ophthalmological examination. NR
66 Suharwardy, 1997 [9] 5/5 1/5 5/5 6/24 2/10 eyes, NPL 1/10 eyes, 6/12 2/10 eyes, 6/9 1/10 eyes, 6/6 2/10 eyes, 1/60 1/10 eyes, HM 1/10 eyes VF in the better eye: RAPD 5/5 Bilateral optic atrophy 1/5, papilledema 2/5, left disc pallor 1/5, bilateral disc pallor 1/5 NR A full ophthalmological examination including VA and VF testing (in most cases possible with a Snellen chart and Goldmann field respectively), optic discs, colour vision and pupil responses. NR
temporal defect 1/5, bitemporal hemianopia 1/5, asymmetric binasal loss with enlarged blind spots 1/5, right probable temporal loss 1/5, left supero-temporal loss 1/5
67 Synowitz, 1977 [69] 3/3 3/3 3/3 Fingerzahlen right/1 m and Handbewegung left/50 cm (1/3), LP (1/3), NR (1/3) NR Nystagmus 2/3 Optic atrophy 2/3, papilledema 2/3 NR Yes (not further specified) NR
68 Tamasauskas, 2014 [105] 7/9 4/9 4/9 RE = LE = 5/10: 1, RE = 1 and LE = 1/1000: 1 Bitemporal hemianopia 2/9, homonymous hemianopia 1/9 NR NR NR VA and VF testing before surgery and after surgery. NR
69 Tan, 2017 [42] 136/185 90/136 90/136 NR NR NR NR NR VA and VF were assessed by experienced ophthalmologists. Children unable to cooperate were given a score based on visual evoked potentials. NR
70 Taphoorn, 2002 [94] 3/3 1/3 3/3 Decreased VA 1/3 Bitemporal hemianopia 1/3, partial homonymous hemianopia 2/3 Diplopia 1/3 Papilledema 2/3 NR NR NR
71 Taylor, 2012 [74] NR 27/56 NR Reduced VA NR Strabismus, nystagmus NR NR NR NR
72 Thomsett, 1980 [32] NR 15/42 15/42 Decreased VA 14/33 VF defect 13/32 Cranial nerve palsy 12/42 Optic atrophy 11/29, papilledema 9/42 NR NR NR
73 Tomita, 2005 [66] NR 23/54 23/54 Decreased VA 13/54: monocular 10, binocular 11. VF defect 2/54 Diplopia 3/54, strabismus 2/54 NR NR NR NR
74 Villani, 1997 [92] NR 16/27 16/27 Decreased VA 16/27 11/27 NR NR NR NR NR
75 Vries de, 2003 [85] NR 8/36 NR NR NR NR NR NR NR NR
76 Wan, 2018 [10] 59/59 18/59 25/59 Visual impairment in at least 1 eye 25/59, binocular visual impairment 16/59, legally blind in both eyes 4/59 NR Diplopia or strabismus 7/59 Optic nerve edema 25/59, optic nerve pallor 24/59 NR VA (preferential looking if vision too poor or pre-verbal) and VF testing (automated Humphrey, dynamic Goldmann or confrontation), fundoscopy. Visual decline: defined as a move from a higher to lower category of visual function in 1 or 2 eyes. Visual outcomes were grouped normal, impaired and legally blind.
77 Weiss, 1989 [59] NR 24/31 24/31 VA and/or VF 19/31 VA and/or VF 19/31 Sixth nerve deficit 4/31: unilateral 2, bilateral 2 Asymptomatic papilledema or optic atrophy 5/31 NR NR NR
78 Wijnen, 2017 [95] VA: 46/63 NR NR 33/46 23/39 NR NR NR VA was determined after correction for refraction disorders. Goldmann perimetry for VF testing. NR
VF: 39/63
79 Winkfield, 2011 [34] NR 46/79 NR NR NR NR Papilledema 25/76 NR NR NR
80 Yamada, 2018 [88] 42/45 (3 patients could not be assessed due to their young age) 12/45 28/42 NR NR NR NR NR Yes (except the youngest in whom testing was difficult): VA and VF testing before and 2 weeks after surgery. NR
81 Yano, 2016 [19] NR 14/26 NR NR NR NR NR NR NR NR
82 Yu., 2015 [46] 14/15 NR 13/15 Decreased vision 13/14 NR Cranial nerve palsy 3/15 NR NR NR NR
83 Zhang, 2008 [30] NR NR 113/202 NR NR NR NR NR NR NR
84 Zhou, 2009 [75] NR 4/5 1/5 NR Hemianopia 1/5 Diplopia 1/5, bidiplopia 1/5, dilated pupil on the left 2/5 Bipapilledema 3/5 NR NR NR

Studies in italics indicate studies retrieved by reference screening.

BCVA = best corrected visual acuity. GKSR: gamma knife stereotactic radiosurgery. HM = hand motion. NA: not available. NPL = no perception of light. NR: not reported. OU = both eyes.

PL = perception of light. P-32: phosphorus-32. VA: visual acuity. VF: visual field. VI: visual impairment.