Rare diseases · Sign or symptom
Dolichocephaly
Long, narrow head
HP:0000268
What it means
An abnormality of skull shape characterized by a increased anterior-posterior diameter, i.e., an increased antero-posterior dimension of the skull. Cephalic index less than 76%. Alternatively, an apparently increased antero-posterior length of the head compared to width. Often due to premature closure of the sagittal suture.
Cephalic index is the ratio of head width expressed as a percentage of head length. The normal range is 76-80.9%. Head length is measured between the glabella (the most prominent point on the frontal bone above the root of the nose) and the most prominent part of the occiput in the midline, using spreading calipers. Head width is measured between the most lateral points of the parietal bones on each side of the head, using spreading calipers. Cephalic index standards are derived from Caucasians and have limited relevance for other races and ethnicities. Current norms have limited validity because of changes in infant sleeping position and consequent changes in head shape. New data should be developed. Dolichocephaly is distinct from Prominent occiput, but both can be present in the same individual and should be coded separately. Scaphocephaly is a subtype of dolichocephaly where the anterior and posterior aspects of the cranial vault are pointed (boat-shaped).
Rare diseases that can present with this93
Very common80–99%
26- Bonnemann-Meinecke-Reich syndrome
- Campomelia, Cumming type
- Cranioectodermal dysplasia
- Craniosynostosis-Dandy-Walker malformation-hydrocephalus syndrome
- Cryptorchidism-arachnodactyly-intellectual disability syndrome
- Cutis gyrata-acanthosis nigricans-craniosynostosis syndrome
- Distal duplication 18q syndrome
- German syndrome
- Hypotonia-cystinuria syndrome
- Isolated megalencephaly
- Lateral meningocele syndrome
- Mevalonic aciduria
- Microcephaly-brachydactyly-kyphoscoliosis syndrome
- Neonatal adrenoleukodystrophy
- Neonatal Marfan syndrome
- Non-syndromic bilambdoid and sagittal craniosynostosis
- Non-syndromic sagittal craniosynostosis
- Prominent glabella-microcephaly-hypogenitalism syndrome
- Radioulnar synostosis-developmental delay-hypotonia syndrome
- Shprintzen-Goldberg syndrome
- Spinocerebellar ataxia-dysmorphism syndrome
- Trisomy 18 syndrome
- Trisomy 5p syndrome
- Unilateral ocular duplication
- White forelock with malformations
- Wolf-Hirschhorn syndrome
Common30–79%
33- 3M syndrome
- 7q11.23microduplication syndrome
- Acromesomelic dysplasia, Maroteaux type
- Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome
- Antenatal multiminicore disease with arthrogryposis multiplex congenita
- Birk-Barel syndrome
- Branchio-oculo-facial syndrome
- Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome
- Cardiocranial syndrome, Pfeiffer type
- Familial scaphocephaly syndrome, McGillivray type
- Hajdu-Cheney syndrome
- Hurler syndrome
- Hypocalcemic vitamin D-resistant rickets
- Marfanoid habitus-autosomal recessive intellectual disability syndrome
- Microcephalic osteodysplastic primordial dwarfism types I and III
- Mosaic trisomy 8 syndrome
- Mucopolysaccharidosis type 1
- Nasopalpebral lipoma-coloboma syndrome
- Oculocerebral hypopigmentation syndrome, Cross type
- Otoonychoperoneal syndrome
- Phelan-McDermid syndrome
- Proteus syndrome
- Ring chromosome 22 syndrome
- Schneckenbecken dysplasia
- Sotos syndrome
- Temtamy syndrome
- Tetralogy of Fallot
- Thomas syndrome
- Tricho-dento-osseous syndrome
- Trisomy 10p syndrome
- Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia
- X-linked intellectual disability-limb spasticity-retinal dystrophy-arginine vasopressin deficiency
- Yunis-Varon syndrome
Sometimes5–29%
21- 15q overgrowth syndrome
- 16p13.11microduplication syndrome
- 19p13.13microdeletion syndrome
- 6q terminal deletion syndrome
- Autosomal dominant deafness-onychodystrophy syndrome
- Autosomal recessive multiple pterygium syndrome
- Bainbridge-Ropers syndrome
- Bannayan-Riley-Ruvalcaba syndrome
and 13 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: Large dolichocephalic skull · Narrow cranium shape · Narrow head shape · Narrow skull shape · Tall and narrow skull · Turridolichocephaly
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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.