- - - 3 gene. This makes This 3 RB1 Due to specializedto Due 3 Importantly, evenImportantly, 3 In 2008, Marmor et al introducedal et Marmor 2008, In Enucleation of the left eye was per was eye left the of Enucleation remained eye right the follow-up, On inoccur to begins development Foveal displacement of cone photoreceptorscone of displacement aforming cells, layer retinal inner and pit. foveal complete thein cones absent, is pit this when morphologicallystill can central tothem enabling narrow, and elongate numbersgreater with compactly align acuity. visual high-resolution for formed, and retinoblastoma was con was retinoblastoma and formed, firmed with no evidence of uveal or optic The histopathologically. invasion nerve theto due altered was anatomy foveal revealedtesting Genetic tumor. massive the in germline (Figure).plana fovea stable with stable, albi aniridia, of evidence no was There diseases.other or nanophthalmos, nism, in20/60 was VA follow-up, 3-year At wasplana fovea the and eye right the unchanged. DISCUSSION intocontinues and gestation of 25 week period. postnatal the region,foveal the in circuitry “midget” singlea to connects cone each where cell,ganglion single a and cell bipolar betweenform connections lateral fewer zoneavascular foveal the in neurons retina. the in elsewhere than to susceptible zone avascular foveal the ------

A 6-month-old white boy with leftwith boy white 6-month-old A fundusand segment anterior The enhancing mass with possible distalpossible with mass enhancing invasion. nerve optic evidence of tumor and with minimallywith and tumor of evidence ring- foveal subtle , pigmented foveo minimal and reflex, light shaped dem eye left The reflex. light central lar eso left diopter 30–prism onstrated Funduscopically,leukocoria. and tropia exophytic multinodular, a was there measuringD) (group retinoblastoma inmm 8.3 and diameter in mm 22.0 mac the through extending thickness, andnerve, optic the overhanging ula, Magneticseeds. subretinal viable with thedemonstrated imaging resonance the foveal pit in this 6-month-old infant. CASE REPORT toreferred was months 4 for Willsat Service Oncology Ocular the retinoblas possible for Hospital Eye wasacuity visual examination, On toma. fix- no and eye right the in fix-and-follow tensionFinger eye. left the in and-follow eyes.both in normal were pressures nowith normal were eye right the in boy with unilateral advanced retino advanced unilateral with boy Atenucleation. necessitating blastoma tonoted was he evaluation, of time the eyeuninvolved his in plana fovea have knowledge,our of best the To OCT. on foveaof case reported earliest the is this ofloss microstructural clear with plana,

1 - 2 - 6,7 - - - - 1,2,5 refers Rare cases ofcases Rare refers to refers 6 1,3 2 Foveal hypoplasia Foveal 4 An estimated 3% of 3% estimated An 1 fovea plana fovea

foveal hypoplasia foveal

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eyes have an underdeveloped an have eyes he term term he a of absence anatomic the pit. foveal normal clinically with children We recently cared for a 6-month-olda for cared recently We Fovea plana is generally discovered indiscovered generally is plana Fovea By contrast, contrast, By A foveal pit is not necessarilynot is pit foveal A BY KEVIN GEORGE, BS; ANTONIO YAGHY, MD; AND CAROL L. SHIELDS, MD MD; AND CAROL L. SHIELDS, MD BY KEVIN GEORGE, BS; ANTONIO YAGHY, This may be the youngest patient in which this abnormality has been noted. been has this abnormality which patient in the youngest This may be T unilateral fovea plana have been report been have plana fovea unilateral indepen that suggesting however, ed, mosaicismgenetic as such factors dent aplay might environment tissue local or plana. fovea of development the in role reported in children as young as 4 years. 4 as young as children in reported pro bilateral a typically is plana Fovea findings structural symmetric with cess asuggesting OCT, spectral-domain on inresults that process developmental maturation. foveal arrested has been seen with conditions such assuch conditions with seen been has nan , albinism, aniridia, pigmenti,incontinentia ophthalmos, prematurity. of and routineduring adults older or younger beenalso has it but evaluation, OCT tional disability. It is certainly possiblecertainly is It disability. tional inacuity visual adequate maintain to plana. fovea with eye an asso with fovea underdeveloped an to loss. vision ciated foveal pit on OCT. on pit foveal specialization. cone foveal for required plana fovea of diagnosis a own, its On func portend automatically not does

6-MONTH-OLD INFANT 6-MONTH-OLD FOVEA PLANA IN A A IN PLANA FOVEA RETINA TODAY

32 s OCULAR ONCOLOGY OCULAR ONCOLOGY s

A B On evaluation, fovea plana can be associated with aniridia, albinism, nanophthalmos, incontinentia pigmenti, retinopathy of prematurity, and achromatopsia, or can be seen in an oth- erwise healthy eye. In the case presented here, there was initial suspicion for albinism due to a blunted foveal contour, notice- able reduction in uveal pigmentation, and prominent choroidal vessels. However, there was no or transillumina- C tion defect. Thus, this case is likely fovea plana in an otherwise healthy eye, and a good visual outcome is anticipated.

CONCLUSION Fovea plana is typically a bilateral fundus finding on OCT imaging. It can occur in patients with excellent visual acuity. Figure. Funduscopy of the right eye showing blonde fundus and prominent choroidal This rare presentation of fovea plana diagnosed in an eye of vessels (A), with flat retina on ultrasonography and axial length of 22.3 mm (B). a 6-month-old child could represent the youngest patient to On OCT, there was minimal foveal pit, consistent with fovea plana, and the retinal be recognized with this abnormality. n layers appeared intact (C). 1. Marmor MF, Choi SS, Zawadzki RJ, Werner JS. Visual insignificance of the foveal pit: reassessment of foveal hypoplasia as the term fovea plana in a description of four patients, ages fovea plana. Arch Ophthalmol. 2008;126(7):907-913. 10 to 26 years, with OCT-evident fovea plana. These patients 2. Noval S, Freedman SF, Asrani S, El-Dairi MA. Incidence of fovea plana in normal children. J AAPOS. 2014;18(5):471-475. 3. Provis JM, Dubis AM, Maddess T, Carroll J. Adaptation of the central retina for high acuity vision: cones, the fovea and the had VA of 20/20 to 20/50 and no evidence of nystagmus or avascular zone. Prog Retin Eye Res. 2013;35:63-81. abnormalities on multifocal electroretinogram. The authors 4. Pakzad-Vaezi K, Keane PA, Cardoso JN, Egan C, Tufail A. Optical coherence tomography angiography of foveal hypopla- sia. Br J Ophthalmol. 2017;101(7):985-988. concluded that foveal cone specialization can be preserved 5. Dubis AM, Hansen BR, Cooper RF, Beringer J, Dubra A, Carroll J. Relationship between the foveal avascular zone and independently from foveal contour.1 They proposed that the foveal pit morphology. Invest Ophthalmol Vis Sci. 2012;53(3):1628-1636. 6. Villegas VM, Schwartz SG, Hamet TD, McKeown CA, Capó H, Flynn HW. Variable clinical profile of fovea plana in normal term fovea plana is anatomically descriptive, based on OCT, children. Ophthalmic Surg Lasers Imaging Retina. 2018;49(4):251-257. and that it can appear in patients with related conditions such 7. Bechet L, Hage R, Vest A, Jean-Charles A, Merle H. Fovea plana in a 9-year-old boy presenting with decreased vision in the left eye. Am J Case Rep. 2019;20:537-541. as aniridia, albinism, and achromatopsia, or might appear in 8. Dolz-Marco R, Phasukkijwatana N, Sarraf D, Freund K. Optical coherence tomography angiography in fovea plana. patients with no underlying disease. Ophthalmic Surg Lasers Imaging Retina. 2016;47(7):670-673. Thus, the clinician should not infer that the lack of a foveal pit on OCT signifies poor visual potential. However, any child or adult with poor pit morphology should be evaluated clini- KEVIN GEORGE, BS cally for underlying related conditions. n Medical student, Sidney Kimmel Medical College, Thomas Jefferson University, Since that initial description, several studies of this entity Philadelphia have been published. In 2014, Noval et al reviewed the n [email protected] OCTs of 286 normal children and found an absent foveal n Financial disclosure: None pit in nine patients (3%).2 All nine children with fovea plana had bilateral findings and VA of 20/20 in both eyes, with CAROL L. SHIELDS, MD normal stereoacuity.2 The measured mean foveal thickness n Director, Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson was greater in eyes with fovea plana (294.5 μm) compared University, Philadelphia with age-matched controls (219.8 μm, P = .029).2 In 2016, n Editorial Advisory Board Member, Retina Today Dolz-Marco et al noted both the loss of the foveal avascu- n [email protected] lar zone and preserved fusion of the superficial and deep n Financial disclosure: None capillary plexuses around the foveal center in three patients with fovea plana.8 ANTONIO YAGHY, MD In 2018, Villegas et al noted that fovea plana presented as a n Research Intern, Ocular Oncology Service, Wills Eye Hospital, Philadelphia bilateral disease in five of six patients, all of whom maintained n [email protected] 20/40 or better BCVA.6 In the one patient with asymmetric n Financial disclosure: None manifestation, an 8-year-old girl, her right eye with 20/25 VA showed obvious fovea plana, whereas her left eye with Support provided in part by the Eye Tumor Research Foundation, 20/25 VA showed a normal foveal contour. Both eyes showed Philadelphia, PA (CLS). The funders had no role in the design and exactly the same refraction, +1.00 +1.50 X 90°. The authors conduct of the study, in the collection, analysis and interpretation noted that of +1.50 D or greater was present in of the data, and in the preparation, review or approval of the 45% of eyes, suggesting that astigmatism may be more preva- manuscript. Carol L. Shields, MD, has had full access to all the data lent in patients with fovea plana than initially suspected.6 in the study and takes responsibility for the integrity of the data.

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