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Start free with EleplanCloacal exstrophy
ORPHA:93929Clinical subtype
Also called OEIS complex · Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome
What it is
A rare congenital genitourinary malformation characterized by urogenital, gastrointestinal, skeletal and neurospinal anomalies. It is the most severe part of the spectrum of the exstrophy-epispadias complex (EEC) and is also referred as the OEIS complex (omphalocele, exstrophy, imperforate anus and spinal defects).
Key facts
- Prevalence
- 1-9 / 1 000 000 (at birth, United States)
- Age of onset
- Infancy, Neonatal
- Inheritance
- Multigenic/multifactorial
- Classified as
- Clinical subtype
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%
6Common30–79%
11These 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.
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.
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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