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Facial dysmorphism-shawl scrotum-joint laxity syndrome

MedGen UID:
419099
Concept ID:
C2931522
Disease or Syndrome
Synonyms: Hypertelorism, downslanting palpebral fissures, malar hypoplasia, and apparently low-set ears associated with joint and scrotal anomalies; Seaver Cassidy syndrome
SNOMED CT: Seaver Cassidy syndrome (716337006)
 
Monarch Initiative: MONDO:0015781
Orphanet: ORPHA1778

Definition

This syndrome has characteristics of facial dysmorphism (hypertelorism, telecanthus, downslanting palpebral fissures, ptosis, malar hypoplasia, broad nasal bridge, thin upper lip, smooth philtrum, and low-set prominent ears) and associated with joint anomalies (genu valgum or cubitus valgus, hyper-extensible joints). It has been described in two patients (a mother and her son). The boy also had hypoplastic shawl scrotum and cryptorchidism, and the mother had mild intellectual deficit. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFacial dysmorphism-shawl scrotum-joint laxity syndrome

Professional guidelines

PubMed

Joseph AW, Joseph SS, Francomano CA, Kontis TC
JAMA Facial Plast Surg 2018 Jan 1;20(1):70-75. doi: 10.1001/jamafacial.2017.0793. PMID: 29121166
Schrander-Stumpel CT, Spruyt L, Curfs LM, Defloor T, Schrander JJ
Am J Med Genet A 2005 Jan 30;132A(3):234-43. doi: 10.1002/ajmg.a.30331. PMID: 15690368
Sepulveda W, Dezerega V, Horvath E, Aracena M
J Ultrasound Med 1999 Oct;18(10):707-10. doi: 10.7863/jum.1999.18.10.707. PMID: 10511304

Recent clinical studies

Etiology

Klaniewska M, Bolanowska-Tyszko A, Latos-Bielenska A, Jezela-Stanek A, Szczaluba K, Krajewska-Walasek M, Ciara E, Pelc M, Jurkiewicz D, Stawinski P, Zubkiewicz-Kucharska A, Rydzanicz M, Ploski R, Smigiel R
Mol Genet Genomic Med 2023 Dec;11(12):e2265. Epub 2023 Sep 1 doi: 10.1002/mgg3.2265. PMID: 37658610Free PMC Article
Joseph AW, Joseph SS, Francomano CA, Kontis TC
JAMA Facial Plast Surg 2018 Jan 1;20(1):70-75. doi: 10.1001/jamafacial.2017.0793. PMID: 29121166
Dentici ML, Di Pede A, Lepri FR, Gnazzo M, Lombardi MH, Auriti C, Petrocchi S, Pisaneschi E, Bellacchio E, Capolino R, Braguglia A, Angioni A, Dotta A, Digilio MC, Dallapiccola B
Arch Dis Child 2015 Feb;100(2):158-64. Epub 2014 Oct 3 doi: 10.1136/archdischild-2013-305858. PMID: 25281733
Mack WP
Facial Plast Surg Clin North Am 2010 Aug;18(3):435-56. doi: 10.1016/j.fsc.2010.05.002. PMID: 20659676
Murray J, Cuckle H, Taylor G, Hewison J
Health Technol Assess 1997;1(4):i-iv, 1-71. PMID: 9414543

Diagnosis

Salinas R, Puig M, Fry CL, Johnson DA, Kheirkhah A
Ocul Surf 2020 Jan;18(1):31-39. Epub 2019 Oct 5 doi: 10.1016/j.jtos.2019.10.002. PMID: 31593763
Bari A, Sadaqat N, Nawaz N, Bano I
J Coll Physicians Surg Pak 2019 Jun;29(6):S41-S42. doi: 10.29271/jcpsp.2019.06.S41. PMID: 31142417
Joseph AW, Joseph SS, Francomano CA, Kontis TC
JAMA Facial Plast Surg 2018 Jan 1;20(1):70-75. doi: 10.1001/jamafacial.2017.0793. PMID: 29121166
Dentici ML, Di Pede A, Lepri FR, Gnazzo M, Lombardi MH, Auriti C, Petrocchi S, Pisaneschi E, Bellacchio E, Capolino R, Braguglia A, Angioni A, Dotta A, Digilio MC, Dallapiccola B
Arch Dis Child 2015 Feb;100(2):158-64. Epub 2014 Oct 3 doi: 10.1136/archdischild-2013-305858. PMID: 25281733
Matsumoto N, Niikawa N
Am J Med Genet C Semin Med Genet 2003 Feb 15;117C(1):57-65. doi: 10.1002/ajmg.c.10020. PMID: 12561059

Therapy

Zoumpou T, Samuel S, Torun N, Yadav P, Jones DB
Obes Surg 2023 Feb;33(2):523-529. Epub 2022 Dec 26 doi: 10.1007/s11695-022-06410-4. PMID: 36567378
Cheong AJY, Ho OTW, Wang SKX, Woon CY, Yap K, Ng KJY, Xu FWX, See A, Ng ACW, Loh SRH, Chee SP, Toh ST
Surv Ophthalmol 2023 Mar-Apr;68(2):257-264. Epub 2022 Nov 23 doi: 10.1016/j.survophthal.2022.11.006. PMID: 36427560
Vahdani K, Ford R, Garrott H, Thaller VT
Eye (Lond) 2018 Jun;32(6):1117-1122. Epub 2018 Feb 21 doi: 10.1038/s41433-018-0048-9. PMID: 29463864Free PMC Article
Macchiaiolo M, Mennini M, Digilio MC, Buonuomo PS, Lepri FR, Gnazzo M, Grandin A, Angioni A, Bartuli A
Am J Med Genet A 2014 Mar;164A(3):760-3. Epub 2013 Dec 19 doi: 10.1002/ajmg.a.36327. PMID: 24357341
Mack WP
Facial Plast Surg Clin North Am 2010 Aug;18(3):435-56. doi: 10.1016/j.fsc.2010.05.002. PMID: 20659676

Prognosis

Klaniewska M, Bolanowska-Tyszko A, Latos-Bielenska A, Jezela-Stanek A, Szczaluba K, Krajewska-Walasek M, Ciara E, Pelc M, Jurkiewicz D, Stawinski P, Zubkiewicz-Kucharska A, Rydzanicz M, Ploski R, Smigiel R
Mol Genet Genomic Med 2023 Dec;11(12):e2265. Epub 2023 Sep 1 doi: 10.1002/mgg3.2265. PMID: 37658610Free PMC Article
Zoumpou T, Samuel S, Torun N, Yadav P, Jones DB
Obes Surg 2023 Feb;33(2):523-529. Epub 2022 Dec 26 doi: 10.1007/s11695-022-06410-4. PMID: 36567378
Joseph AW, Joseph SS, Francomano CA, Kontis TC
JAMA Facial Plast Surg 2018 Jan 1;20(1):70-75. doi: 10.1001/jamafacial.2017.0793. PMID: 29121166
Muniesa MJ, Huerva V, Sánchez-de-la-Torre M, Martínez M, Jurjo C, Barbé F
Br J Ophthalmol 2013 Nov;97(11):1387-90. Epub 2013 Apr 12 doi: 10.1136/bjophthalmol-2012-303051. PMID: 23584721
Bergin DJ, McCord CD, Berger T, Friedberg H, Waterhouse W
Br J Ophthalmol 1988 Nov;72(11):863-7. doi: 10.1136/bjo.72.11.863. PMID: 3207663Free PMC Article

Clinical prediction guides

Ruiters S, Mombaerts I
Eye (Lond) 2021 Jul;35(7):1909-1914. Epub 2020 Sep 14 doi: 10.1038/s41433-020-01178-2. PMID: 32929179Free PMC Article
Joseph AW, Joseph SS, Francomano CA, Kontis TC
JAMA Facial Plast Surg 2018 Jan 1;20(1):70-75. doi: 10.1001/jamafacial.2017.0793. PMID: 29121166
Rasmussen M, Vestergaard EM, Graakjaer J, Petkov Y, Bache I, Fagerberg C, Kibaek M, Svaneby D, Petersen OB, Brasch-Andersen C, Sunde L
Am J Med Genet A 2016 Nov;170(11):2934-2942. Epub 2016 Jul 13 doi: 10.1002/ajmg.a.37848. PMID: 27409573
Willatt L, Cox J, Barber J, Cabanas ED, Collins A, Donnai D, FitzPatrick DR, Maher E, Martin H, Parnau J, Pindar L, Ramsay J, Shaw-Smith C, Sistermans EA, Tettenborn M, Trump D, de Vries BB, Walker K, Raymond FL
Am J Hum Genet 2005 Jul;77(1):154-60. Epub 2005 May 25 doi: 10.1086/431653. PMID: 15918153Free PMC Article
Murray J, Cuckle H, Taylor G, Hewison J
Health Technol Assess 1997;1(4):i-iv, 1-71. PMID: 9414543

Recent systematic reviews

Cheong AJY, Ho OTW, Wang SKX, Woon CY, Yap K, Ng KJY, Xu FWX, See A, Ng ACW, Loh SRH, Chee SP, Toh ST
Surv Ophthalmol 2023 Mar-Apr;68(2):257-264. Epub 2022 Nov 23 doi: 10.1016/j.survophthal.2022.11.006. PMID: 36427560

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