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Cloverleaf skull-multiple congenital anomalies syndrome

MedGen UID:
375942
Concept ID:
C1846671
Disease or Syndrome
Synonym: Multiple congenital anomalies syndrome with cloverleaf skull
SNOMED CT: Cloverleaf skull with multiple congenital anomalies syndrome (717771007)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
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 individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0011788
OMIM®: 607161
Orphanet: ORPHA93267

Definition

This syndrome has characteristics of cloverleaf skull, limb anomalies, facial dysmorphism and multiple congenital anomalies. It has been described in three siblings from one family. Dysmorphic features include protruding forehead, hypertelorism, broad nasal bridge, wide anterior fontanelle and short philtrum, down turning mouth, micrognathia and low-set ears. The limbs show rhizomelic shortening. Additional malformations are not constant: omphalocele, bilateral microphthalmia, cataract, narrow chest, ambiguous genitalia, cardiac ventricular septal defect and agenesis of the corpus callosum. The condition seems to be inherited as an autosomal recessive trait. Prognosis is poor. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCloverleaf skull-multiple congenital anomalies syndrome
Follow this link to review classifications for Cloverleaf skull-multiple congenital anomalies syndrome in Orphanet.

Professional guidelines

PubMed

Rosato S, Unger S, Campos-Xavier B, Caraffi SG, Beltrami L, Pollazzon M, Ivanovski I, Castori M, Bonasoni MP, Comitini G, Nikkels PGJ, Lindstrom K, Umandap C, Superti-Furga A, Garavelli L
Genes (Basel) 2022 Jan 28;13(2) doi: 10.3390/genes13020261. PMID: 35205306Free PMC Article
Rubio EI, Blask A, Bulas DI
Pediatr Radiol 2016 May;46(5):709-18. Epub 2016 Feb 25 doi: 10.1007/s00247-016-3550-x. PMID: 26914936
Tubbs RS, Sharma A, Griessenauer C, Loukas M, Shoja MM, Watanabe K, Oakes WJ
Childs Nerv Syst 2013 May;29(5):745-8. Epub 2012 Dec 12 doi: 10.1007/s00381-012-1981-8. PMID: 23233211

Recent clinical studies

Etiology

Vinchon M, Guerreschi P, Karnoub MA, Wolber A
Childs Nerv Syst 2021 Oct;37(10):3113-3118. Epub 2021 Mar 13 doi: 10.1007/s00381-021-05117-8. PMID: 33713165
Liu T, Liu G, Jiang S, Hu Y, Zhang M, Liu X
Med Hypotheses 2020 Nov;144:109837. Epub 2020 May 23 doi: 10.1016/j.mehy.2020.109837. PMID: 32512489
Giancotti A, D'Ambrosio V, Marchionni E, Squarcella A, Aliberti C, La Torre R, Manganaro L, Pizzuti A; PECRAM Study Group*
J Matern Fetal Neonatal Med 2017 Sep;30(18):2225-2231. Epub 2016 Oct 20 doi: 10.1080/14767058.2016.1243099. PMID: 27762162
Machado G, Di Rocco F, Sainte-Rose C, Meyer P, Marchac D, Macquet-Nouvion G, Arnaud E, Renier D
Childs Nerv Syst 2011 Oct;27(10):1683-91. Epub 2011 Sep 17 doi: 10.1007/s00381-011-1508-8. PMID: 21928032
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Diagnosis

Mavridis IN, Rodrigues D
Childs Nerv Syst 2021 Feb;37(2):367-374. Epub 2020 Oct 20 doi: 10.1007/s00381-020-04934-7. PMID: 33083874
Giancotti A, D'Ambrosio V, Marchionni E, Squarcella A, Aliberti C, La Torre R, Manganaro L, Pizzuti A; PECRAM Study Group*
J Matern Fetal Neonatal Med 2017 Sep;30(18):2225-2231. Epub 2016 Oct 20 doi: 10.1080/14767058.2016.1243099. PMID: 27762162
Tubbs RS, Sharma A, Griessenauer C, Loukas M, Shoja MM, Watanabe K, Oakes WJ
Childs Nerv Syst 2013 May;29(5):745-8. Epub 2012 Dec 12 doi: 10.1007/s00381-012-1981-8. PMID: 23233211
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Therapy

Noto M, Imai K, Masuoka T, Sakahara D, Kunihiro N
J Craniofac Surg 2021 Jan-Feb 01;32(1):322-324. doi: 10.1097/SCS.0000000000007151. PMID: 33156168
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Prognosis

Mavridis IN, Rodrigues D
Childs Nerv Syst 2021 Feb;37(2):367-374. Epub 2020 Oct 20 doi: 10.1007/s00381-020-04934-7. PMID: 33083874
Peña-Padilla C, Viramontes-Aguilar L, Tavares-Macías G, Bobadilla-Morales L, L Cunningham M, Park S, Zapata-Aldana E, Corona-Rivera JR
Fetal Pediatr Pathol 2019 Oct;38(5):412-417. Epub 2019 Apr 19 doi: 10.1080/15513815.2019.1603256. PMID: 31002276
Machado G, Di Rocco F, Sainte-Rose C, Meyer P, Marchac D, Macquet-Nouvion G, Arnaud E, Renier D
Childs Nerv Syst 2011 Oct;27(10):1683-91. Epub 2011 Sep 17 doi: 10.1007/s00381-011-1508-8. PMID: 21928032
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Clinical prediction guides

Rosato S, Unger S, Campos-Xavier B, Caraffi SG, Beltrami L, Pollazzon M, Ivanovski I, Castori M, Bonasoni MP, Comitini G, Nikkels PGJ, Lindstrom K, Umandap C, Superti-Furga A, Garavelli L
Genes (Basel) 2022 Jan 28;13(2) doi: 10.3390/genes13020261. PMID: 35205306Free PMC Article
Vinchon M, Guerreschi P, Karnoub MA, Wolber A
Childs Nerv Syst 2021 Oct;37(10):3113-3118. Epub 2021 Mar 13 doi: 10.1007/s00381-021-05117-8. PMID: 33713165
Mavridis IN, Rodrigues D
Childs Nerv Syst 2021 Feb;37(2):367-374. Epub 2020 Oct 20 doi: 10.1007/s00381-020-04934-7. PMID: 33083874
Ettinger N, Williams M, Phillips JA 3rd
J Craniofac Surg 2013;24(5):1829-32. doi: 10.1097/SCS.0b013e3182997df5. PMID: 24036790
Hall BD, Cadle RG, Golabi M, Morris CA, Cohen MM Jr
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Recent systematic reviews

Tcherbbis Testa V, Jaimovich S, Argañaraz R, Mantese B
Acta Neurochir (Wien) 2021 Nov;163(11):3083-3091. Epub 2021 Sep 27 doi: 10.1007/s00701-021-04980-3. PMID: 34570275
Mavridis IN, Rodrigues D
Childs Nerv Syst 2021 Feb;37(2):367-374. Epub 2020 Oct 20 doi: 10.1007/s00381-020-04934-7. PMID: 33083874

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