U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Treacher Collins syndrome 2(TCS2)

MedGen UID:
462333
Concept ID:
C3150983
Disease or Syndrome
Synonyms: POLR1D-Related Treacher Collins Syndrome; TCS2; TREACHER COLLINS SYNDROME 2, AUTOSOMAL RECESSIVE
 
Gene (location): POLR1D (13q12.2)
 
Monarch Initiative: MONDO:0013385
OMIM®: 613717

Disease characteristics

Excerpted from the GeneReview: Treacher Collins Syndrome
Treacher Collins syndrome (TCS) is characterized by lower eyelid abnormalities, malar hypoplasia, downslanted palpebral fissures, and micro- or retrognathia due to symmetric hypoplasia of the zygomatic bones, maxilla, and mandible. External ear anomalies include absent, small, malformed, and/or posteriorly rotated ears and atresia or stenosis of the external auditory canals. About 40%-50% of individuals have conductive hearing loss attributed most commonly to malformation of the ossicles and hypoplasia of the middle ear cavities. Inner ear structures tend to be normal. Significant respiratory and feeding difficulties can be present in infancy. Other, less common abnormalities include cleft palate and unilateral or bilateral choanal stenosis or atresia. Typically, intellect is normal. [from GeneReviews]
Authors:
Mafalda Barbosa  |  Ethylin Wang Jabs  |  Sara Huston   view full author information

Additional description

From OMIM
Treacher Collins syndrome is a disorder of craniofacial development characterized by a combination of bilateral downward slanting of the palpebral fissures, colobomas of the lower eyelids with a paucity of eyelashes medial to the defect, hypoplasia of the facial bones, cleft palate, malformation of the external ears, atresia of the external auditory canals, and bilateral conductive hearing loss (Dauwerse et al., 2011). For additional phenotypic information and a discussion of genetic heterogeneity of Treacher Collins syndrome, see TCS1 (154500).  http://www.omim.org/entry/613717

Clinical features

From HPO
Conductive hearing impairment
MedGen UID:
9163
Concept ID:
C0018777
Disease or Syndrome
An abnormality of vibrational conductance of sound to the inner ear leading to impairment of sensory perception of sound.
Microtia
MedGen UID:
57535
Concept ID:
C0152423
Congenital Abnormality
Underdevelopment of the external ear.
Anotia
MedGen UID:
152377
Concept ID:
C0702139
Congenital Abnormality
Complete absence of any auricular structures.
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
Motor delay
MedGen UID:
381392
Concept ID:
C1854301
Finding
A type of Developmental delay characterized by a delay in acquiring motor skills.
Micrognathia
MedGen UID:
44428
Concept ID:
C0025990
Congenital Abnormality
Developmental hypoplasia of the mandible.
Retrognathia
MedGen UID:
19766
Concept ID:
C0035353
Congenital Abnormality
An abnormality in which the mandible is mislocalised posteriorly.
Microretrognathia
MedGen UID:
326907
Concept ID:
C1839546
Finding
A form of developmental hypoplasia of the mandible in which the mandible is mislocalised posteriorly.
Malar flattening
MedGen UID:
347616
Concept ID:
C1858085
Finding
Underdevelopment of the malar prominence of the jugal bone (zygomatic bone in mammals), appreciated in profile, frontal view, and/or by palpation.
Fusion of middle ear ossicles
MedGen UID:
349423
Concept ID:
C1862068
Finding
Bony fusion of malleus, incus, and stapes.
Hypoplasia of the zygomatic bone
MedGen UID:
866886
Concept ID:
C4021242
Anatomical Abnormality
Underdevelopment of the zygomatic bone. That is, a reduction in size of the zygomatic bone, including the zygomatic process of the temporal bone of the skull, which forms part of the zygomatic arch.
Choanal atresia
MedGen UID:
3395
Concept ID:
C0008297
Congenital Abnormality
Absence or abnormal closure of the choana (the posterior nasal aperture). Most embryologists believe that posterior choanal atresia results from a failure of rupture between the 35th and 38th day of fetal life of the partition which separates the bucconasal or buccopharyngeal membranes. The resultant choanal atresia may be unilateral or bilateral, bony or membranous, complete or incomplete. In over 90 per cent of cases the obstruction is bony, while in the remainder it is membranous. The bony type of atresia is commonly located 1-2 mm. anterior to the posterior edge of the hard palate, and the osseous septum varies in thickness from 1 to 10 mm. In the membranous form of choanal atresia the obstruction usually occurs further posteriorly. In approximately one third of cases the atresia is bilateral.
Downslanted palpebral fissures
MedGen UID:
98391
Concept ID:
C0423110
Finding
The palpebral fissure inclination is more than two standard deviations below the mean.
Choanal stenosis
MedGen UID:
108427
Concept ID:
C0584837
Finding
Abnormal narrowing of the choana (the posterior nasal aperture).
Lower eyelid coloboma
MedGen UID:
373417
Concept ID:
C1837826
Disease or Syndrome
A short discontinuity of the margin of the lower eyelid.
Cleft palate
MedGen UID:
756015
Concept ID:
C2981150
Congenital Abnormality
Cleft palate is a developmental defect of the palate resulting from a failure of fusion of the palatine processes and manifesting as a separation of the roof of the mouth (soft and hard palate).
Preauricular pit
MedGen UID:
120587
Concept ID:
C0266610
Congenital Abnormality
Small indentation anterior to the insertion of the ear.

Professional guidelines

PubMed

Johns AL, Stock NM, Costa B, Billaud Feragen K, Crerand CE
Cleft Palate Craniofac J 2023 Sep;60(9):1090-1112. Epub 2022 Apr 5 doi: 10.1177/10556656221091699. PMID: 35382590Free PMC Article
Chan CH, Hu AC, Jain NS, Dang BN, Liu MT, Bertrand AA, Wilson LF, Lee JC
J Craniofac Surg 2021 Nov-Dec 01;32(8):2771-2773. doi: 10.1097/SCS.0000000000007938. PMID: 34727477
Lee HJ, Yang IH, Baek SH
J Craniofac Surg 2021 Nov-Dec 01;32(8):e773-e778. doi: 10.1097/SCS.0000000000007769. PMID: 34727453

Recent clinical studies

Etiology

Gonçalves Ferraz B, Vendramini-Pittoli S, Gomes LP, Madeira Brandão M, Alonso N, Tonello C
J Craniofac Surg 2023 Jun 1;34(4):e398-e401. Epub 2023 May 1 doi: 10.1097/SCS.0000000000009326. PMID: 37126414
Beaumont CA, Dunaway DJ, Padwa BL, Forrest C, Koudstaal MJ, Caron CJJM
Int J Oral Maxillofac Surg 2021 Nov;50(11):1471-1476. Epub 2021 Mar 19 doi: 10.1016/j.ijom.2021.03.001. PMID: 33752939
Ribeiro AA, Smith FJ, Nary Filho H, Trindade IEK, Tonello C, Trindade-Suedam IK
Cleft Palate Craniofac J 2020 Mar;57(3):371-377. Epub 2019 Dec 5 doi: 10.1177/1055665619885555. PMID: 31801369
Levasseur J, Nysjö J, Sandy R, Britto JA, Garcelon N, Haber S, Picard A, Corre P, Odri GA, Khonsari RH
J Craniomaxillofac Surg 2018 Feb;46(2):305-311. Epub 2017 Dec 8 doi: 10.1016/j.jcms.2017.11.028. PMID: 29275073
Dimitrov B, Balikova I, Jekova N, Vakrilova L, Fryns JP, Simeonov E
Am J Med Genet A 2005 May 15;135(1):81-5. doi: 10.1002/ajmg.a.30673. PMID: 15793832

Diagnosis

Ulhaq ZS, Nurputra DK, Soraya GV, Kurniawati S, Istifiani LA, Pamungkas SA, Tse WKF
Clin Genet 2023 Feb;103(2):146-155. Epub 2022 Oct 17 doi: 10.1111/cge.14243. PMID: 36203321
Spineli-Silva S, Sgardioli IC, Dos Santos AP, Bergamini LL, Monlleó IL, Fontes MIB, Félix TM, Ribeiro EM, Xavier AC, Lustosa-Mendes E, Gil-da-Silva-Lopes VL, Vieira TP
Am J Med Genet C Semin Med Genet 2020 Dec;184(4):970-985. Epub 2020 Nov 20 doi: 10.1002/ajmg.c.31857. PMID: 33215817
Birgfeld C, Heike C
Clin Plast Surg 2019 Apr;46(2):207-221. doi: 10.1016/j.cps.2018.12.001. PMID: 30851752
Alfi D, Lam D, Gateno J
Atlas Oral Maxillofac Surg Clin North Am 2014 Sep;22(2):167-73. Epub 2014 Jul 15 doi: 10.1016/j.cxom.2014.04.003. PMID: 25171997
Buchanan EP, Xue AS, Hollier LH Jr
Plast Reconstr Surg 2014 Jul;134(1):128e-153e. doi: 10.1097/PRS.0000000000000308. PMID: 25028828

Therapy

Sakamoto Y, Miwa T, Yoshida K, Kishi K
J Craniofac Surg 2019 Jan;30(1):e58-e60. doi: 10.1097/SCS.0000000000004974. PMID: 30444784
Hopper RA, Kapadia H, Susarla S, Bly R, Johnson K
Plast Reconstr Surg 2018 Aug;142(2):447-457. doi: 10.1097/PRS.0000000000004606. PMID: 29870506
Golinko MS, LeBlanc EM, Hallett AM, Alperovich M, Flores RL
J Craniofac Surg 2016 Sep;27(6):1408-11. doi: 10.1097/SCS.0000000000002821. PMID: 27607112
Nguyen PD, Caro MC, Smith DM, Tompson B, Forrest CR, Phillips JH
J Plast Reconstr Aesthet Surg 2016 Mar;69(3):402-8. Epub 2015 Oct 29 doi: 10.1016/j.bjps.2015.10.036. PMID: 26638831
AlSalloum A, El Mouzan MI, AlHerbish A, AlOmer A, Qurashi M
Ann Saudi Med 2015 Mar-Apr;35(2):107-10. doi: 10.5144/0256-4947.2015.107. PMID: 26336015Free PMC Article

Prognosis

Gomes LP, Ferraz BG, Brandão MM, Alonso N, Pittoli SV, Tonello C
J Craniofac Surg 2023 Jun 1;34(4):1283-1286. Epub 2023 Jan 20 doi: 10.1097/SCS.0000000000009192. PMID: 36658685
Lu M, Yang B, Chen Z, Jiang H, Pan B
Cleft Palate Craniofac J 2022 Aug;59(8):1038-1047. Epub 2021 Aug 16 doi: 10.1177/10556656211037509. PMID: 34397304
Liu J, Dong J, Li P, Duan W
Int J Pediatr Otorhinolaryngol 2021 Aug;147:110765. Epub 2021 May 11 doi: 10.1016/j.ijporl.2021.110765. PMID: 34058530
Rosa RF, Guimarães VB, Beltrão LA, Trombetta JS, Lliguin KL, de Mattos VF, Zen PR
Pediatr Int 2015 Apr;57(2):e69-72. Epub 2015 Mar 25 doi: 10.1111/ped.12562. PMID: 25808856
Dimitrov B, Balikova I, Jekova N, Vakrilova L, Fryns JP, Simeonov E
Am J Med Genet A 2005 May 15;135(1):81-5. doi: 10.1002/ajmg.a.30673. PMID: 15793832

Clinical prediction guides

Erlandson KM, Geng LN, Selvaggi CA, Thaweethai T, Chen P, Erdmann NB, Goldman JD, Henrich TJ, Hornig M, Karlson EW, Katz SD, Kim C, Cribbs SK, Laiyemo AO, Letts R, Lin JY, Marathe J, Parthasarathy S, Patterson TF, Taylor BD, Duffy ER, Haack M, Julg B, Maranga G, Hernandez C, Singer NG, Han J, Pemu P, Brim H, Ashktorab H, Charney AW, Wisnivesky J, Lin JJ, Chu HY, Go M, Singh U, Levitan EB, Goepfert PA, Nikolich JŽ, Hsu H, Peluso MJ, Kelly JD, Okumura MJ, Flaherman VJ, Quigley JG, Krishnan JA, Scholand MB, Hess R, Metz TD, Costantine MM, Rouse DJ, Taylor BS, Goldberg MP, Marshall GD, Wood J, Warren D, Horwitz L, Foulkes AS, McComsey GA; RECOVER-Adult Cohort
Ann Intern Med 2024 Sep;177(9):1209-1221. Epub 2024 Aug 13 doi: 10.7326/M24-0737. PMID: 39133923Free PMC Article
Gomes LP, Ferraz BG, Brandão MM, Alonso N, Pittoli SV, Tonello C
J Craniofac Surg 2023 Jun 1;34(4):1283-1286. Epub 2023 Jan 20 doi: 10.1097/SCS.0000000000009192. PMID: 36658685
Ulhaq ZS, Nurputra DK, Soraya GV, Kurniawati S, Istifiani LA, Pamungkas SA, Tse WKF
Clin Genet 2023 Feb;103(2):146-155. Epub 2022 Oct 17 doi: 10.1111/cge.14243. PMID: 36203321
Lu M, Yang B, Chen Z, Jiang H, Pan B
Cleft Palate Craniofac J 2022 Aug;59(8):1038-1047. Epub 2021 Aug 16 doi: 10.1177/10556656211037509. PMID: 34397304
Liu J, Dong J, Li P, Duan W
Int J Pediatr Otorhinolaryngol 2021 Aug;147:110765. Epub 2021 May 11 doi: 10.1016/j.ijporl.2021.110765. PMID: 34058530

Recent systematic reviews

Aiyar A, Pedersen TK, Resnick CM, Nørholt SE, Verna C, Stoustrup PB
Orthod Craniofac Res 2024 Jun;27 Suppl 1:131-140. Epub 2023 Nov 21 doi: 10.1111/ocr.12729. PMID: 37987216
Ulhaq ZS, Nurputra DK, Soraya GV, Kurniawati S, Istifiani LA, Pamungkas SA, Tse WKF
Clin Genet 2023 Feb;103(2):146-155. Epub 2022 Oct 17 doi: 10.1111/cge.14243. PMID: 36203321
Glaeser AB, Santos AS, Diniz BL, Deconte D, Rosa RFM, Zen PRG
Am J Med Genet A 2020 Nov;182(11):2624-2631. Epub 2020 Sep 7 doi: 10.1002/ajmg.a.61841. PMID: 32893956
Amirhassankhani S, Lloyd MS
J Craniofac Surg 2018 Mar;29(2):372-375. doi: 10.1097/SCS.0000000000004201. PMID: 29239919
Renkema RW, Caron CJJM, Wolvius EB, Dunaway DJ, Forrest CR, Padwa BL, Koudstaal MJ
Int J Oral Maxillofac Surg 2018 Jan;47(1):27-34. Epub 2017 Jul 20 doi: 10.1016/j.ijom.2017.06.009. PMID: 28736116

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...