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Ectrodactyly-ectodermal dysplasia-clefting syndrome

MedGen UID:
98357
Concept ID:
C0406704
Disease or Syndrome
Synonyms: Ectrodactyly-cleft lip/palate syndrome; Ectrodactyly-ectodermal dysplasia-cleft lip/cleft palate; Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome; EEC syndrome; RUDIGER SYNDROME; Rudiger syndrome 1; Walker-Clodius syndrome
SNOMED CT: Ectodermal dysplasia with ectrodactyly and cleft lip or palate (39788007); Rudiger's syndrome (39788007); EEC - Ectodermal dysplasia with ectrodactyly and cleft lip or palate (39788007); Ectrodactyly-ectodermal dysplasia-clefting syndrome (39788007); EEC syndrome (39788007)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Monarch Initiative: MONDO:0010004
OMIM®: 268650
Orphanet: ORPHA1896

Definition

EEC syndrome is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia, and orofacial clefts (cleft lip/palate). [from ORDO]

Clinical features

From HPO
Ovarian cyst
MedGen UID:
14540
Concept ID:
C0029927
Disease or Syndrome
The presence of one or more cysts of the ovary.
Bicornuate uterus
MedGen UID:
78599
Concept ID:
C0266387
Congenital Abnormality
The presence of a bicornuate uterus.
Ureterovesical stenosis
MedGen UID:
870198
Concept ID:
C4024633
Anatomical Abnormality
Micropenis
MedGen UID:
1633603
Concept ID:
C4551492
Congenital Abnormality
Abnormally small penis. At birth, the normal penis is about 3 cm (stretched length from pubic tubercle to tip of penis) with micropenis less than 2.0-2.5 cm.
Single transverse palmar crease
MedGen UID:
96108
Concept ID:
C0424731
Finding
The distal and proximal transverse palmar creases are merged into a single transverse palmar crease.
Short digit
MedGen UID:
893063
Concept ID:
C4023124
Finding
One or more digit that appears disproportionately short compared to the hand/foot, whereby either the entire digit or a specific phalanx is shortened.
High axial triradius
MedGen UID:
871318
Concept ID:
C4025806
Anatomical Abnormality
Inguinal hernia
MedGen UID:
6817
Concept ID:
C0019294
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Frontal bossing
MedGen UID:
67453
Concept ID:
C0221354
Congenital Abnormality
Bilateral bulging of the lateral frontal bone prominences with relative sparing of the midline.
Flexion contracture
MedGen UID:
83069
Concept ID:
C0333068
Anatomical Abnormality
A flexion contracture is a bent (flexed) joint that cannot be straightened actively or passively. It is thus a chronic loss of joint motion due to structural changes in muscle, tendons, ligaments, or skin that prevents normal movement of joints.
Hoarse voice
MedGen UID:
5602
Concept ID:
C0019825
Sign or Symptom
Hoarseness refers to a change in the pitch or quality of the voice, with the voice sounding weak, very breathy, scratchy, or husky.
Depressed nasal bridge
MedGen UID:
373112
Concept ID:
C1836542
Finding
Posterior positioning of the nasal root in relation to the overall facial profile for age.
Prominent forehead
MedGen UID:
373291
Concept ID:
C1837260
Finding
Forward prominence of the entire forehead, due to protrusion of the frontal bone.
Coarse facial features
MedGen UID:
335284
Concept ID:
C1845847
Finding
Absence of fine and sharp appearance of brows, nose, lips, mouth, and chin, usually because of rounded and heavy features or thickened skin with or without thickening of subcutaneous and bony tissues.
Hypoplastic fingernail
MedGen UID:
347304
Concept ID:
C1856786
Finding
Underdevelopment of a fingernail.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEctrodactyly-ectodermal dysplasia-clefting syndrome
Follow this link to review classifications for Ectrodactyly-ectodermal dysplasia-clefting syndrome in Orphanet.

Professional guidelines

PubMed

Fassihi H, Eady RA, Mellerio JE, Ashton GH, Dopping-Hepenstal PJ, Denyer JE, Nicolaides KH, Rodeck CH, McGrath JA
Br J Dermatol 2006 Jan;154(1):106-13. doi: 10.1111/j.1365-2133.2005.07012.x. PMID: 16403102

Recent clinical studies

Etiology

Di Iorio E, Kaye SB, Ponzin D, Barbaro V, Ferrari S, Böhm E, Nardiello P, Castaldo G, McGrath JA, Willoughby CE
Ophthalmology 2012 Jan;119(1):74-83. Epub 2011 Sep 28 doi: 10.1016/j.ophtha.2011.06.044. PMID: 21959367
Wojcicki P, Wysocki M, Wojcicka K
J Craniofac Surg 2010 Sep;21(5):1388-92. doi: 10.1097/SCS.0b013e3181ebccc5. PMID: 20856026
Birgfeld CB, Glick P, Singh D, LaRossa D, Bartlett S
Plast Reconstr Surg 2007 Jul;120(1):144-150. doi: 10.1097/01.prs.0000263567.62923.fd. PMID: 17572556
Johnson SE, Tatum SA, Thomson LL
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):309-13. doi: 10.1016/s0165-5876(02)00278-1. PMID: 12443822
Fernandes B, Ruas E, Machado A, Figueiredo A
Pediatr Dermatol 2002 Jul-Aug;19(4):330-2. doi: 10.1046/j.1525-1470.2002.00094.x. PMID: 12220279

Diagnosis

Childs AJ, Mabin DC, Turnpenny PD
Am J Med Genet A 2020 Aug;182(8):1939-1943. Epub 2020 May 31 doi: 10.1002/ajmg.a.61628. PMID: 32476291
Gün I, Kizilaslan C, Atalay MA
Int J Gynaecol Obstet 2012 Oct;119(1):86-7. Epub 2012 Jul 12 doi: 10.1016/j.ijgo.2012.05.014. PMID: 22795755
Johnson SE, Tatum SA, Thomson LL
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):309-13. doi: 10.1016/s0165-5876(02)00278-1. PMID: 12443822
Kelman GJ, Aronoff RC
J Am Podiatr Med Assoc 2000 Oct;90(9):460-4. doi: 10.7547/87507315-90-9-460. PMID: 11055171
Annerén G, Andersson T, Lindgren PG, Kjartansson S
Clin Genet 1991 Oct;40(4):257-62. doi: 10.1111/j.1399-0004.1991.tb03093.x. PMID: 1756598

Therapy

Ota Y, Matsumoto Y, Dogru M, Goto E, Uchino Y, Endo K, Tsubota K
Optom Vis Sci 2008 Sep;85(9):E795-801. doi: 10.1097/OPX.0b013e31818527cd. PMID: 18772710

Prognosis

Labunski A, Carrasquillo KG, Brocks D
Eye Contact Lens 2023 Jun 1;49(6):262-265. Epub 2023 Apr 13 doi: 10.1097/ICL.0000000000000987. PMID: 37053073
Wojcicki P, Wysocki M, Wojcicka K
J Craniofac Surg 2010 Sep;21(5):1388-92. doi: 10.1097/SCS.0b013e3181ebccc5. PMID: 20856026
Paranaíba LM, Martelli-Júnior H, de Miranda RT, Bufalino A, Abdo Filho RC, Coletta RD
Cleft Palate Craniofac J 2010 Sep;47(5):544-7. doi: 10.1597/09-063. PMID: 20180707
Johnson SE, Tatum SA, Thomson LL
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):309-13. doi: 10.1016/s0165-5876(02)00278-1. PMID: 12443822
Fernandes B, Ruas E, Machado A, Figueiredo A
Pediatr Dermatol 2002 Jul-Aug;19(4):330-2. doi: 10.1046/j.1525-1470.2002.00094.x. PMID: 12220279

Clinical prediction guides

Di Iorio E, Kaye SB, Ponzin D, Barbaro V, Ferrari S, Böhm E, Nardiello P, Castaldo G, McGrath JA, Willoughby CE
Ophthalmology 2012 Jan;119(1):74-83. Epub 2011 Sep 28 doi: 10.1016/j.ophtha.2011.06.044. PMID: 21959367
Wojcicki P, Wysocki M, Wojcicka K
J Craniofac Surg 2010 Sep;21(5):1388-92. doi: 10.1097/SCS.0b013e3181ebccc5. PMID: 20856026
Paranaíba LM, Martelli-Júnior H, de Miranda RT, Bufalino A, Abdo Filho RC, Coletta RD
Cleft Palate Craniofac J 2010 Sep;47(5):544-7. doi: 10.1597/09-063. PMID: 20180707
Vilain C, Mortier G, Van Vliet G, Dubourg C, Heinrichs C, de Silva D, Verloes A, Baumann C
Am J Med Genet A 2009 Jul;149A(7):1476-81. doi: 10.1002/ajmg.a.32678. PMID: 19504604
Ota Y, Matsumoto Y, Dogru M, Goto E, Uchino Y, Endo K, Tsubota K
Optom Vis Sci 2008 Sep;85(9):E795-801. doi: 10.1097/OPX.0b013e31818527cd. PMID: 18772710

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