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Chondrodysplasia punctata, MT type

MedGen UID:
98147
Concept ID:
C0432224
Congenital Abnormality
Synonyms: Chondrodysplasia punctata, tibia-metacarpal type; Chondrodysplasia punctata, tibial-metacarpal type
SNOMED CT: Chondrodysplasia punctata, tibia-metacarpal type (254083002); Chondrodysplasia punctata, MT type (254083002)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0007322
OMIM®: 118651
Orphanet: ORPHA79346

Definition

A rare, non-rhizomelic, chondrodysplasia punctata syndrome characterized, radiologically, by stippled calcifications and disproportionate, short metacarpals and tibiae (with characteristic overshoot of the proximal fibula), clinically manifesting with severe short stature, bilateral shortening of upper and lower limbs, flat midface and nose, in the absence of cataracts and cutaneous anomalies. Neonatal tachypnea, hydrocephalus and mild developmental delay have been seldomly associated. Additional radiologic features include bowed long bones, platyspondyly and/or vertebral clefts. [from ORDO]

Clinical features

From HPO
Micromelia
MedGen UID:
10031
Concept ID:
C0025995
Congenital Abnormality
The presence of abnormally small extremities.
Limb undergrowth
MedGen UID:
116086
Concept ID:
C0239399
Finding
Limb shortening because of underdevelopment of one or more bones of the extremities.
Short 4th metacarpal
MedGen UID:
327074
Concept ID:
C1840309
Finding
Short fourth metacarpal bone.
Short tibia
MedGen UID:
338005
Concept ID:
C1850259
Finding
Underdevelopment (reduced size) of the tibia.
Short 3rd metacarpal
MedGen UID:
338089
Concept ID:
C1850631
Finding
Short third metacarpal bone.
Short 2nd metacarpal
MedGen UID:
370004
Concept ID:
C1969397
Finding
Short second metacarpal bone because of developmental hypoplasia.
Coronal cleft vertebrae
MedGen UID:
320483
Concept ID:
C1834954
Finding
Frontal schisis (cleft or cleavage) of vertebral bodies.
Calcific stippling
MedGen UID:
340441
Concept ID:
C1849993
Finding
An abnormal punctate (speckled, dot-like) pattern of calcifications in soft tissues within or surrounding bones (as observed on radiographs).
Short long bone
MedGen UID:
344385
Concept ID:
C1854912
Finding
One or more abnormally short long bone.
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.
Epiphyseal stippling
MedGen UID:
349104
Concept ID:
C1859126
Finding
The presence of abnormal punctate (speckled, dot-like) calcifications in one or more epiphyses.
Depressed nasal ridge
MedGen UID:
334631
Concept ID:
C1842876
Finding
Lack of prominence of the nose resulting from a posteriorly-placed nasal ridge.
Midface retrusion
MedGen UID:
339938
Concept ID:
C1853242
Anatomical Abnormality
Posterior positions and/or vertical shortening of the infraorbital and perialar regions, or increased concavity of the face and/or reduced nasolabial angle.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChondrodysplasia punctata, MT type
Follow this link to review classifications for Chondrodysplasia punctata, MT type in Orphanet.

Professional guidelines

PubMed

Mathijssen IB, Henneman L, van Eeten-Nijman JM, Lakeman P, Ottenheim CP, Redeker EJ, Ottenhof W, Meijers-Heijboer H, van Maarle MC
Eur J Med Genet 2015 Mar;58(3):123-8. Epub 2015 Jan 30 doi: 10.1016/j.ejmg.2015.01.004. PMID: 25641760
Schramm T, Gloning KP, Minderer S, Daumer-Haas C, Hörtnagel K, Nerlich A, Tutschek B
Ultrasound Obstet Gynecol 2009 Aug;34(2):160-70. doi: 10.1002/uog.6359. PMID: 19548204
Ruano R, Molho M, Roume J, Ville Y
Ultrasound Obstet Gynecol 2004 Aug;24(2):134-40. doi: 10.1002/uog.1113. PMID: 15287049

Recent clinical studies

Etiology

Bosselut H, Panuel M, Sigaudy S, Gorincour G, Chaumoitre K, Bretelle F
Prenat Diagn 2019 Jun;39(7):549-562. Epub 2019 May 10 doi: 10.1002/pd.5469. PMID: 31046133
Morota N, Ihara S, Ogiwara H, Tamura G
J Neurosurg Pediatr 2017 Oct;20(4):378-387. Epub 2017 Aug 11 doi: 10.3171/2017.5.PEDS16554. PMID: 28799855
Schramm T, Gloning KP, Minderer S, Daumer-Haas C, Hörtnagel K, Nerlich A, Tutschek B
Ultrasound Obstet Gynecol 2009 Aug;34(2):160-70. doi: 10.1002/uog.6359. PMID: 19548204
Bams-Mengerink AM, Majoie CB, Duran M, Wanders RJ, Van Hove J, Scheurer CD, Barth PG, Poll-The BT
Neurology 2006 Mar 28;66(6):798-803; discussion 789. doi: 10.1212/01.wnl.0000205594.34647.d0. PMID: 16567694
Connor JM, Connor RA, Sweet EM, Gibson AA, Patrick WJ, McNay MB, Redford DH
Am J Med Genet 1985 Oct;22(2):243-53. doi: 10.1002/ajmg.1320220205. PMID: 3901754

Diagnosis

Thistlethwaite LR, Li X, Burrage LC, Riehle K, Hacia JG, Braverman N, Wangler MF, Miller MJ, Elsea SH, Milosavljevic A
Sci Rep 2022 Apr 21;12(1):6556. doi: 10.1038/s41598-022-10415-5. PMID: 35449147Free PMC Article
Gregersen PA, McKay V, Walsh M, Brown E, McGillivray G, Savarirayan R
Mol Genet Genomic Med 2020 Jun;8(6):e1173. Epub 2020 Apr 18 doi: 10.1002/mgg3.1173. PMID: 32304187Free PMC Article
Chummun S, McLean NR, Nugent M, Anderson PJ, David DJ
J Craniofac Surg 2012 Jul;23(4):986-90. doi: 10.1097/SCS.0b013e31824e2683. PMID: 22777437
Schramm T, Gloning KP, Minderer S, Daumer-Haas C, Hörtnagel K, Nerlich A, Tutschek B
Ultrasound Obstet Gynecol 2009 Aug;34(2):160-70. doi: 10.1002/uog.6359. PMID: 19548204
Miyazaki O, Nishimura G, Sago H, Watanabe N, Ebina S
Pediatr Radiol 2007 Nov;37(11):1151-4. Epub 2007 Sep 6 doi: 10.1007/s00247-007-0568-0. PMID: 17805524

Therapy

Chummun S, McLean NR, Anderson PJ, David DJ
J Plast Reconstr Aesthet Surg 2013 Nov;66(11):1477-81. Epub 2013 Jul 30 doi: 10.1016/j.bjps.2013.07.004. PMID: 23910911

Prognosis

Thistlethwaite LR, Li X, Burrage LC, Riehle K, Hacia JG, Braverman N, Wangler MF, Miller MJ, Elsea SH, Milosavljevic A
Sci Rep 2022 Apr 21;12(1):6556. doi: 10.1038/s41598-022-10415-5. PMID: 35449147Free PMC Article
Shabbir RMK, Nalbant G, Ahmad N, Malik S, Tolun A
J Med Genet 2018 Jul;55(7):489-496. Epub 2018 Mar 7 doi: 10.1136/jmedgenet-2017-105003. PMID: 29514872
Koç U, Karakaş P
Turk J Pediatr 2017;59(5):604-609. doi: 10.24953/turkjped.2017.05.018. PMID: 29745127
Morota N, Ihara S, Ogiwara H, Tamura G
J Neurosurg Pediatr 2017 Oct;20(4):378-387. Epub 2017 Aug 11 doi: 10.3171/2017.5.PEDS16554. PMID: 28799855
Connor JM, Connor RA, Sweet EM, Gibson AA, Patrick WJ, McNay MB, Redford DH
Am J Med Genet 1985 Oct;22(2):243-53. doi: 10.1002/ajmg.1320220205. PMID: 3901754

Clinical prediction guides

Thistlethwaite LR, Li X, Burrage LC, Riehle K, Hacia JG, Braverman N, Wangler MF, Miller MJ, Elsea SH, Milosavljevic A
Sci Rep 2022 Apr 21;12(1):6556. doi: 10.1038/s41598-022-10415-5. PMID: 35449147Free PMC Article
Shabbir RMK, Nalbant G, Ahmad N, Malik S, Tolun A
J Med Genet 2018 Jul;55(7):489-496. Epub 2018 Mar 7 doi: 10.1136/jmedgenet-2017-105003. PMID: 29514872
Bams-Mengerink AM, Majoie CB, Duran M, Wanders RJ, Van Hove J, Scheurer CD, Barth PG, Poll-The BT
Neurology 2006 Mar 28;66(6):798-803; discussion 789. doi: 10.1212/01.wnl.0000205594.34647.d0. PMID: 16567694
Al-Gazali LI, Bakir M, Hamid Z, Varady E, Varghes M, Haas D, Bener A, Padmanabhan R, Abdulrrazzaq YM, Dawadu A
Birth Defects Res A Clin Mol Teratol 2003 Feb;67(2):125-32. doi: 10.1002/bdra.10009. PMID: 12769508
Connor JM, Connor RA, Sweet EM, Gibson AA, Patrick WJ, McNay MB, Redford DH
Am J Med Genet 1985 Oct;22(2):243-53. doi: 10.1002/ajmg.1320220205. PMID: 3901754

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