Rare diseases · Sign or symptom
Narrow palate
Narrow roof of mouth
HP:0000189
What it means
Width of the palate more than 2 SD below the mean (objective) or apparently decreased palatal width (subjective).
Palatal width is measured as the distance between the maxillary first permanent molar on the right and left sides, at the lingual cervical line, using a specific device. Palate width is typically assessed subjectively in routine clinical practice. Narrowing is often associated with a High palate, but this should be assessed and coded separately. Gingival overgrowth can give the impression of a narrow palate but should be distinguished and coded separately. The term gothic palate is used to indicate that the roof of the palate is not round but rather has an inverted V-shape, and therefore, only the upper part of the palate is narrow.
Rare diseases that can present with this26
Very common80–99%
4Common30–79%
13- Alpha-mannosidosis
- Apert syndrome
- Böök syndrome
- Coffin-Lowry syndrome
- Cutis gyrata-acanthosis nigricans-craniosynostosis syndrome
- Developmental and speech delay due to SOX5 deficiency
- Down syndrome
- Intellectual disability-muscle weakness-short stature-facial dysmorphism syndrome
- Koolen-De Vries syndrome
- Rubinstein-Taybi syndrome due to 16p13.3 microdeletion
- Rubinstein-Taybi syndrome due to CREBBP mutations
- Rubinstein-Taybi syndrome due to EP300 haploinsufficiency
- Saethre-Chotzen syndrome
Sometimes5–29%
9- Bannayan-Riley-Ruvalcaba syndrome
- Classical-like Ehlers-Danlos syndrome type 2
- Crouzon syndrome
- DOORS syndrome
- FLNA-related X-linked myxomatous valvular dysplasia
- Leukocyte adhesion deficiency type II
- Oculocerebrorenal syndrome of Lowe
- Splenogonadal fusion-limb defects-micrognathia syndrome
and 1 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
Also called: Decreased palatal width · Decreased transverse dimension of palate
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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.