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Chromosome 16q12 duplication syndrome

MedGen UID:
1794292
Concept ID:
C5562082
Disease or Syndrome
Synonym: CONE DYSTROPHY WITH EARLY-ONSET TRITANOPIC COLOR VISION DEFECT
 
Monarch Initiative: MONDO:0859210
OMIM®: 619649

Definition

Chromosome 16q12 duplication syndrome is characterized by early-onset progressive cone dystrophy, with early blue cone involvement. Patients report reduced visual acuity in the first decade of life, as well as difficulty differentiating colors, photophobia, and reduced night vision (Kohl et al., 2021). Tritanopia can also be caused by heterozygous mutation in the OPN1SW gene (613522) on chromosome 7q32 (see 190900). [from OMIM]

Clinical features

From HPO
Photophobia
MedGen UID:
43220
Concept ID:
C0085636
Sign or Symptom
Excessive sensitivity to light with the sensation of discomfort or pain in the eyes due to exposure to bright light.
Anisocoria
MedGen UID:
1944
Concept ID:
C0003079
Finding
Anisocoria, or unequal pupil size, may represent a benign physiologic variant or a manifestation of disease.
Night blindness
MedGen UID:
10349
Concept ID:
C0028077
Disease or Syndrome
Inability to see well at night or in poor light.
Cataract
MedGen UID:
39462
Concept ID:
C0086543
Disease or Syndrome
A cataract is an opacity or clouding that develops in the crystalline lens of the eye or in its capsule.
Blue color blindness
MedGen UID:
57827
Concept ID:
C0155017
Disease or Syndrome
Tritanopia is an autosomal dominant disorder of human vision characterized by a selective deficiency of blue spectral sensitivity (Weitz et al., 1992).
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
High myopia
MedGen UID:
78759
Concept ID:
C0271183
Disease or Syndrome
A severe form of myopia with greater than -6.00 diopters.
Paracentral scotoma
MedGen UID:
75740
Concept ID:
C0271197
Finding
Temporal optic disc pallor
MedGen UID:
576209
Concept ID:
C0344299
Disease or Syndrome
A pale yellow discoloration of the temporal (lateral) portion of the optic disc.
Retinal pigment epithelial mottling
MedGen UID:
347513
Concept ID:
C1857644
Finding
Mottling (spots or blotches with different shades) of the retinal pigment epithelium, i.e., localized or generalized fundal pigment granularity associated with processes at the level of the retinal pigment epithelium.
Central thinning of the outer nuclear layer of the retina
MedGen UID:
1052433
Concept ID:
CN376963
Finding
Reduced thickness of the outer nuclear layer in the middle region of the retina. The outer nuclear layer (ONL) of the retina contains the nuclei of the cone and rod photoreceptors. Loss of the cellular machinery found in these nuclei causes irreparable loss of the photoreceptors and the capacity for visual function. This feature can be appreciated by directional optical coherence tomography.
Paracentral hyper-autofluorecence
MedGen UID:
1052698
Concept ID:
CN377918
Finding
Increased autofluorescence in areas adjacent to the central macula (but not including the very center).

Recent clinical studies

Diagnosis

Zerem A, Vinkler C, Michelson M, Leshinsky-Silver E, Lerman-Sagie T, Lev D
Am J Med Genet A 2011 Dec;155A(12):2991-6. Epub 2011 Nov 3 doi: 10.1002/ajmg.a.34316. PMID: 22052843

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