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Intellectual developmental disorder with ocular anomalies and distinctive facial features(IDDOF)

MedGen UID:
1824011
Concept ID:
C5774238
Disease or Syndrome
Synonym: IDDOF
 
Gene (location): MTSS2 (16q22.1)
 
Monarch Initiative: MONDO:0859303
OMIM®: 620086

Definition

Intellectual developmental disorder with ocular anomalies and distinctive facial features (IDDOF) is characterized by global developmental delay, mildly impaired intellectual development, ophthalmologic anomalies, microcephaly or relative microcephaly, hearing loss, and characteristic facial features (Huang et al., 2022). [from OMIM]

Clinical features

From HPO
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Intellectual disability, mild
MedGen UID:
10044
Concept ID:
C0026106
Mental or Behavioral Dysfunction
Mild intellectual disability is defined as an intelligence quotient (IQ) in the range of 50-69.
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Autistic behavior
MedGen UID:
163547
Concept ID:
C0856975
Mental or Behavioral Dysfunction
Persistent deficits in social interaction and communication and interaction as well as a markedly restricted repertoire of activity and interest as well as repetitive patterns of behavior.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Upslanted palpebral fissure
MedGen UID:
98390
Concept ID:
C0423109
Finding
The palpebral fissure inclination is more than two standard deviations above the mean for age (objective); or, the inclination of the palpebral fissure is greater than typical for age.
Epicanthus
MedGen UID:
151862
Concept ID:
C0678230
Congenital Abnormality
Epicanthus is a condition in which a fold of skin stretches from the upper to the lower eyelid, partially covering the inner canthus. Usher (1935) noted that epicanthus is a normal finding in the fetus of all races. Epicanthus also occurs in association with hereditary ptosis (110100).
Narrow forehead
MedGen UID:
326956
Concept ID:
C1839758
Finding
Width of the forehead or distance between the frontotemporales is more than two standard deviations below the mean (objective); or apparently narrow intertemporal region (subjective).
Ptosis
MedGen UID:
2287
Concept ID:
C0005745
Disease or Syndrome
The upper eyelid margin is positioned 3 mm or more lower than usual and covers the superior portion of the iris (objective); or, the upper lid margin obscures at least part of the pupil (subjective).
Nystagmus
MedGen UID:
45166
Concept ID:
C0028738
Disease or Syndrome
Rhythmic, involuntary oscillations of one or both eyes related to abnormality in fixation, conjugate gaze, or vestibular mechanisms.
Optic atrophy
MedGen UID:
18180
Concept ID:
C0029124
Disease or Syndrome
Atrophy of the optic nerve. Optic atrophy results from the death of the retinal ganglion cell axons that comprise the optic nerve and manifesting as a pale optic nerve on fundoscopy.
Iris cyst
MedGen UID:
124385
Concept ID:
C0271119
Finding
An iris cyst is composed of a single cell layer of epithelium and is filled with fluid.
Foveal hypoplasia
MedGen UID:
393047
Concept ID:
C2673946
Finding
Underdevelopment of the fovea centralis.

Recent clinical studies

Etiology

Sakaguchi Y, Yoshihashi H, Uehara T, Miyama S, Kosaki K, Takenouchi T
Am J Med Genet A 2021 Mar;185(3):884-888. Epub 2020 Dec 27 doi: 10.1002/ajmg.a.62020. PMID: 33369122

Diagnosis

Karamik G, Tuysuz B, Isik E, Yilmaz A, Alanay Y, Sunamak EC, Durmusalioglu EA, Ozkinay F, Cetin GO, Ozturk N, Mihci E, Nur B
Am J Med Genet A 2023 Jul;191(7):1814-1825. Epub 2023 Apr 13 doi: 10.1002/ajmg.a.63207. PMID: 37053206
Al-Hinai A, Al-Hashmi S, Ganesh A, Al-Hashmi N, Al-Saegh A, Al-Mamari W, Al-Murshedi F, Al-Thihli K, Al-Kindi A, Al-Maawali A
Am J Med Genet A 2022 Aug;188(8):2485-2490. Epub 2022 May 14 doi: 10.1002/ajmg.a.62778. PMID: 35567578
Coyan AG, Dyer LM
Eur J Med Genet 2020 Dec;63(12):104083. Epub 2020 Oct 9 doi: 10.1016/j.ejmg.2020.104083. PMID: 33039685
Yates TM, Turner CL, Firth HV, Berg J, Pilz DT
Clin Genet 2017 Jul;92(1):3-9. Epub 2016 Nov 30 doi: 10.1111/cge.12864. PMID: 27625340
Gropman AL, Duncan WC, Smith AC
Pediatr Neurol 2006 May;34(5):337-50. doi: 10.1016/j.pediatrneurol.2005.08.018. PMID: 16647992

Prognosis

Eker HK, Derinkuyu BE, Ünal S, Masliah-Planchon J, Drunat S, Verloes A
Eur J Med Genet 2014 Jan;57(1):32-6. Epub 2013 Nov 7 doi: 10.1016/j.ejmg.2013.10.005. PMID: 24211661

Clinical prediction guides

Winter-Paquette LM, Al Suwaidi HH, Sajjad Y, Bricker L
Eur J Med Genet 2022 May;65(5):104501. Epub 2022 Apr 1 doi: 10.1016/j.ejmg.2022.104501. PMID: 35378319
Sakaguchi Y, Yoshihashi H, Uehara T, Miyama S, Kosaki K, Takenouchi T
Am J Med Genet A 2021 Mar;185(3):884-888. Epub 2020 Dec 27 doi: 10.1002/ajmg.a.62020. PMID: 33369122
Martín-Rivada Á, Pozo-Román J, Güemes M, Ortiz-Cabrera NV, Pérez-Jurado LA, Argente J
Horm Res Paediatr 2020;93(9-10):567-572. Epub 2021 Mar 23 doi: 10.1159/000514280. PMID: 33756487
Gropman AL, Duncan WC, Smith AC
Pediatr Neurol 2006 May;34(5):337-50. doi: 10.1016/j.pediatrneurol.2005.08.018. PMID: 16647992

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