Rare diseases · Sign or symptom
Esotropia
Inward turning cross eyed
HP:0000565
What it means
A form of strabismus with one or both eyes turned inward ('crossed') to a relatively severe degree, usually defined as 10 diopters or more.
Esotropia is analogous to but more severe than esophoria. Affected children are more likely to have amblyopia or require corrective eye muscle surgery than children with esophoria.
Rare diseases that can present with this66
Very common80–99%
5Common30–79%
22- 3-phosphoglycerate dehydrogenase deficiency, infantile/juvenile form
- 9p13microdeletion syndrome
- ALG2-CDG
- Alternating hemiplegia of childhood
- Autosomal recessive cerebellar ataxia-pyramidal signs-nystagmus-oculomotor apraxia syndrome
- Autosomal recessive congenital cerebellar ataxia due to MGLUR1 deficiency
- Autosomal recessive frontotemporal pachygyria
- Bilateral frontoparietal polymicrogyria
- Bilateral polymicrogyria
- Blindness-scoliosis-arachnodactyly syndrome
- Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation
- Hao-Fountain syndrome due to 16p13.2 microdeletion
- Infantile hypotonia-oculomotor anomalies-hyperkinetic movements-developmental delay syndrome
- Intellectual disability-obesity-prognathism-eye and skin anomalies syndrome
- Late-infantile/juvenile Krabbe disease
- Microcephaly-seizures-intellectual disability-heart disease syndrome
- Orofaciodigital syndrome type 6
- PMM2-CDG
- S-adenosylhomocysteine hydrolase deficiency
- Ullrich congenital muscular dystrophy
- X-linked intellectual disability, Cantagrel type
- Xp21deletion syndrome
Sometimes5–29%
34- 7q11.23microduplication syndrome
- Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
- AHDC1-related intellectual disability-obstructive sleep apnea-mild dysmorphism syndrome
- ALG9-CDG
- Autosomal recessive methemoglobinemia
- CDKL5-deficiency disorder
- Congenital fibrosis of extraocular muscles
- Congenital limbs-face contractures-hypotonia-developmental delay syndrome
and 26 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.