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Start free with EleplanCraniosynostosis-anal anomalies-porokeratosis syndrome
ORPHA:85199Malformation syndrome
Also called CAP syndrome · CDAGS syndrome
What it is
Craniosynostosis - anal anomalies - porokeratosis, or CDAGS, is a very rare condition characterized by craniosynostosis and clavicular hypoplasia, (C), delayed closure of the fontanel (D), anal anomalies (A), genitourinary malformations (G) and skin eruption (S).
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Infancy, Neonatal
- Inheritance
- Autosomal recessive
- Classified as
- Malformation syndrome
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
24- Abnormal dental morphology
- Abnormality of dental enamel
- Abnormality of the clavicle
- Abnormal palate morphology
- Absent eyebrow
- Absent eyelashes
- Anal atresia
- Aplastic clavicles
- Brachycephaly
- Coronal craniosynostosis
- Delayed cranial suture closure
- Delayed skeletal maturation
- Ectopic anus
- Eczematoid dermatitis
- Frontal bossing
- Hypospadias
- Large posterior fontanelle
- Parietal foramina
- Porokeratosis
- Short thorax
- Tarsal synostosis
- Thin fingernail
- Urogenital fistula
- Wide anterior fontanel
Common30–79%
8These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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