MCID: OSC001
MIFTS: 29

Oeis Complex

Categories: Genetic diseases, Nephrological diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Oeis Complex

MalaCards integrated aliases for Oeis Complex:

Name: Oeis Complex 54 24 56
Omphalocele-Cloacal Exstrophy-Imperforate Anus-Spinal Defect Syndrome 56
Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects 24
Omphalocele Exstrophy Imperforate Anus 69
Cloacal Exstrophy 56

Characteristics:

Orphanet epidemiological data:

56
cloacal exstrophy
Inheritance: Multigenic/multifactorial; Age of onset: Infancy,Neonatal; Age of death: normal life expectancy;

OMIM:

54
Inheritance:
isolated cases

Miscellaneous:
majority of cases are sporadic
oeis is an acronym for omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects


HPO:

32
oeis complex:
Inheritance sporadic


Classifications:



Summaries for Oeis Complex

MalaCards based summary : Oeis Complex, also known as omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome, is related to omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex and exstrophy of bladder, and has symptoms including hemivertebrae, talipes equinovarus and omphalocele. An important gene associated with Oeis Complex is MNX1 (Motor Neuron And Pancreas Homeobox 1). Affiliated tissues include kidney, colon and uterus.

Description from OMIM: 258040

Related Diseases for Oeis Complex

Diseases related to Oeis Complex via text searches within MalaCards or GeneCards Suite gene sharing:

id Related Disease Score Top Affiliating Genes
1 omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex 11.6
2 exstrophy of bladder 11.4
3 omphalocele 10.4
4 imperforate anus 10.3
5 cloacal exstrophy 9.9
6 pentalogy of cantrell 9.9
7 transposition of the great arteries 9.8
8 nail-patella syndrome 9.8
9 lumbosacral lipoma 9.8
10 limb-body wall complex 9.8

Graphical network of the top 20 diseases related to Oeis Complex:



Diseases related to Oeis Complex

Symptoms & Phenotypes for Oeis Complex

Symptoms via clinical synopsis from OMIM:

54

Neurologic- Central Nervous System:
hydrocephalus
chiari malformation
meningomyelocele
tethered cord
lipomyelocystoceles

Genitourinary- External Genitalia Female:
ambiguous genitalia
hypoplastic labia
widely separated labioscrotal folds

Genitourinary- Kidneys:
hydronephrosis
renal agenesis
pelvic kidney

Skeletal- Spine:
hemivertebrae
absent sacrum
sacral segmentation defects

Genitourinary- Internal Genitalia Female:
bifid uterus

Skeletal- Pelvis:
congenital hip dislocation
symphysis pubis diastasis

Chest- Ribs Sternum Clavicles And Scapulae:
11 pairs of ribs

Genitourinary- External Genitalia Male:
ambiguous genitalia
small penis
epispadias
abnormal or absent scrotum

Skeletal- Feet:
club feet

Genitourinary- Internal Genitalia Male:
cryptorchidism

Abdomen- External Features:
omphalocele

Abdomen- Gastroin testinal:
intestinal malrotation
imperforate anus
anal atresia
rectovaginal fistula
anteriorly placed anus
more
Genitourinary- Ureters:
hydroureter
duplicated collecting system

Genitourinary- Bladder:
vesicovaginal fistula
cloacal exstrophy


Clinical features from OMIM:

258040

Human phenotypes related to Oeis Complex:

56 32 (show all 47)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hemivertebrae 56 32 Frequent (79-30%) HP:0002937
2 talipes equinovarus 56 32 Frequent (79-30%) HP:0001762
3 omphalocele 56 32 Very frequent (99-80%) HP:0001539
4 intestinal malrotation 56 32 Frequent (79-30%) HP:0002566
5 hydroureter 56 32 Occasional (29-5%) HP:0000072
6 anal atresia 56 32 Frequent (79-30%) HP:0002023
7 myelomeningocele 56 32 Frequent (79-30%) HP:0002475
8 bladder exstrophy 56 32 Very frequent (99-80%) HP:0002836
9 cloacal exstrophy 56 32 Very frequent (99-80%) HP:0010475
10 hydrocephalus 32 HP:0000238
11 micropenis 32 HP:0000054
12 hip dislocation 56 Frequent (79-30%)
13 hydronephrosis 32 HP:0000126
14 cryptorchidism 32 HP:0000028
15 horseshoe kidney 56 Occasional (29-5%)
16 absent scrotum 32 HP:0008707
17 vesicoureteral reflux 56 Very frequent (99-80%)
18 spina bifida 56 Frequent (79-30%)
19 bifid uterus 32 HP:0000136
20 renal agenesis 32 HP:0000104
21 ureteropelvic junction obstruction 56 Occasional (29-5%)
22 congenital hip dislocation 32 HP:0001374
23 11 pairs of ribs 32 HP:0000878
24 duplicated collecting system 32 HP:0000081
25 ureterocele 56 Occasional (29-5%)
26 epispadias 32 HP:0000039
27 ectopic kidney 56 Occasional (29-5%)
28 vesicovaginal fistula 32 HP:0001586
29 arnold-chiari malformation 32 HP:0002308
30 pelvic kidney 32 HP:0000125
31 rectovaginal fistula 32 HP:0000143
32 tethered cord 32 HP:0002144
33 anteriorly placed anus 32 HP:0001545
34 labial hypoplasia 32 HP:0000066
35 renal hypoplasia/aplasia 56 Occasional (29-5%)
36 hypoplasia of penis 56 Very frequent (99-80%)
37 duplicated colon 32 HP:0005223
38 ambiguous genitalia, male 32 HP:0000033
39 abnormality of the tibia 56 Frequent (79-30%)
40 abnormality of the fibula 56 Frequent (79-30%)
41 intestinal duplication 56 Frequent (79-30%)
42 ambiguous genitalia, female 32 HP:0000061
43 abnormality of the clitoris 56 Very frequent (99-80%)
44 abnormality of the fallopian tube 56 Occasional (29-5%)
45 absence of the sacrum 32 HP:0010305
46 absent foot 56 Frequent (79-30%)
47 sacral segmentation defect 32 HP:0008490

Drugs & Therapeutics for Oeis Complex

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Prospective Cohort of Transitional Urology Patients Recruiting NCT03061084

Search NIH Clinical Center for Oeis Complex

Genetic Tests for Oeis Complex

Genetic tests related to Oeis Complex:

id Genetic test Affiliating Genes
1 Oeis Complex 24

Anatomical Context for Oeis Complex

MalaCards organs/tissues related to Oeis Complex:

39
Kidney, Colon, Uterus

Publications for Oeis Complex

Articles related to Oeis Complex:

(show all 30)
id Title Authors Year
1
Discordant Anomalies with Combined Features of Pentalogy of Cantrell and OEIS Complex: A Case Report in Monochorionic Twins. ( 28617177 )
2017
2
Penile duplication as genital abnormality in OEIS complex: a fetal autopsy case report. ( 26513516 )
2016
3
Fetal and neonatal presentation of OEIS complex. ( 26520699 )
2015
4
Multimodality Renal Failure in a Patient with OEIS Complex. ( 26495176 )
2015
5
Re: Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 24703152 )
2014
6
Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex). ( 25400352 )
2014
7
Early prenatal diagnosis of the OEIS complex with different appearance in early compared with late pregnancy before spontaneous rupture of the cloacal membrane. ( 24623061 )
2014
8
Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 22640865 )
2013
9
OEIS complex. ( 24839537 )
2013
10
Prenatal findings of omphalocele-exstrophy of the bladder-imperforate anus-spinal defects (OEIS) complex. ( 22925220 )
2012
11
Comprehensive genetic analysis of OEIS complex reveals no evidence for a recurrent microdeletion or duplication. ( 21204209 )
2011
12
Prenatal diagnosis of OEIS (omphalocele, bladder exstrophy, imperforate anus, clubfeet) variant associated with increased nuchal translucency and OEIS complex with ambiguous genitalia associated with corrected transposition of the great arteries: case series and review of the literature. ( 21475965 )
2011
13
Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. ( 21453858 )
2011
14
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in a child with nail-patella syndrome. ( 20880310 )
2010
15
OEIS complex associated with chromosome 1p36 deletion: a case report and review. ( 20101692 )
2010
16
Double lumbosacral lipomas of the dorsal and filar types associated with OEIS complex: case report. ( 19855150 )
2009
17
Supernumerary kidney in a child with OEIS complex. ( 19371928 )
2009
18
Discordant omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex and cardiac malformations in monochorionic twins. ( 19842883 )
2009
19
Neurosurgical management of occult spinal dysraphism associated with OEIS complex. ( 17962955 )
2008
20
Syndromes and disorders associated with omphalocele (II): OEIS complex and Pentalogy of Cantrell. ( 17638617 )
2007
21
OEIS complex with major cardiac malformation: a case report. ( 17883075 )
2007
22
Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review. ( 17702047 )
2007
23
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex associated with increased nuchal translucency. ( 12858313 )
2003
24
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases. ( 11251992 )
2001
25
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins. ( 10213043 )
1999
26
Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects. ( 9259943 )
1997
27
Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam. ( 7711456 )
1995
28
The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. ( 1433234 )
1992
29
OEIS complex (cloacal exstrophy): prenatal diagnosis in the second trimester. ( 2456564 )
1988
30
The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). ( 728566 )
1978

Variations for Oeis Complex

Expression for Oeis Complex

Search GEO for disease gene expression data for Oeis Complex.

Pathways for Oeis Complex

GO Terms for Oeis Complex

Sources for Oeis Complex

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....