Closed spinal dysraphism

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Closed spinal dysraphism

ORPHA:645202Clinical group

Also called Closed spina bifida · Occult spina bifida · Spina bifida occulta

What it is

A rare group of spinal dysraphisms, also referred to as spina bifida occulta, with a high variability in terms of severity,characterized by the absence of exposed neural tissue. The skin overlying the abnormality remains intact although the skin itself may be abnormal with features such as hairy patch of skin, crater or haemangioma. These skin features are known as the cutaneous stigmata of spinal dysraphism.

Key facts

Classified as
Clinical group

Recorded for the broader condition

Prevalence
1-5 / 10 000 (Europe)Spina bifida and other spinal dysraphisms
Age of onset
Infancy, NeonatalSpina bifida and other spinal dysraphisms
Inheritance
Multigenic/multifactorial, Not applicableSpina bifida and other spinal dysraphisms

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Genes reported in subtypes

FUZVANGL1

Orphanet records these genes on 1 more specific entry under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

ICD-10 codes

Q76.0ICD-10 names this disease exactly — shared with 9 other rare diseases

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.

Benefit programs to look at

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

MEDDRA 10041525MESH D016136MONDO 859UMLS C0080174

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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