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Fetal trigonocephaly

MedGen UID:
1842024
•Concept ID:
C5826881
•
Congenital Abnormality
Synonyms: Foetal trigonocephaly; Strawberry sign; Strawberry skull; Strawberry-shaped skull
 
HPO: HP:4000142

Definition

A fetal ultrasound finding defined by flattening of the occiput with pointing of the frontal bones and brachycephaly with an increased cephalic index. It is characterized by keel shaped deformity of forehead with midline ridge, bilateral fronto temporal constriction with compensatory biparietal expansion, supra orbital and lateral orbital retrusion and hypotelorism. This finding is said to resemble a strawberry. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFetal trigonocephaly

Professional guidelines

PubMed

Differential diagnosis of syndromic craniosynostosis: a case series.
Casteleyn T, Horn D, Henrich W, Verlohren S
Arch Gynecol Obstet 2022 Jul;306(1):49-57. Epub 2021 Oct 11 doi: 10.1007/s00404-021-06263-9. PMID: 34633507Free PMC Article
Craniosynostosis: Clinical Presentation, Genetics, and Prenatal Diagnosis.
Brah TK, Thind R, Abel DE
Obstet Gynecol Surv 2020 Oct;75(10):636-644. doi: 10.1097/OGX.0000000000000830. PMID: 33111964
Prenatal diagnosis: detailed chromosomal analysis in 500 cases.
Atkins L, Milunsky A, Shahood JM
Clin Genet 1974;6(4):317-22. doi: 10.1111/j.1399-0004.1974.tb02092.x. PMID: 4216438

Recent clinical studies

Etiology

Feeding considerations for infants with craniofacial malformations.
Green MA, Resnick CM
Semin Fetal Neonatal Med 2021 Dec;26(6):101280. Epub 2021 Sep 20 doi: 10.1016/j.siny.2021.101280. PMID: 34561178
Sonographic indicators of isolated fetal sagittal craniosynostosis during pregnancy.
Constantine S, Kiermeier A, Anderson P
J Med Imaging Radiat Oncol 2020 Oct;64(5):626-633. Epub 2020 Jun 12 doi: 10.1111/1754-9485.13068. PMID: 32530572
22q11.2 deletion syndrome and congenital heart disease.
Goldmuntz E
Am J Med Genet C Semin Med Genet 2020 Mar;184(1):64-72. Epub 2020 Feb 12 doi: 10.1002/ajmg.c.31774. PMID: 32049433
Craniofacial disorders associated with airway obstruction in the neonate.
Cielo CM, Montalva FM, Taylor JA
Semin Fetal Neonatal Med 2016 Aug;21(4):254-62. Epub 2016 Mar 17 doi: 10.1016/j.siny.2016.03.001. PMID: 26997148Free PMC Article
Mortality and pathological findings in C (Opitz trigonocephaly) syndrome.
Opitz JM, Putnam AR, Comstock JM, Chin S, Byrne JL, Kennedy A, Frikke MJ, Bernard C, Albrecht S, Der Kaloustian V, Szakacs JG
Fetal Pediatr Pathol 2006 Jul-Aug;25(4):211-31. doi: 10.1080/15513810601015753. PMID: 17162528

Diagnosis

Genetics of craniofacial malformations.
Schmetz A, Amiel J, Wieczorek D
Semin Fetal Neonatal Med 2021 Dec;26(6):101290. Epub 2021 Sep 17 doi: 10.1016/j.siny.2021.101290. PMID: 34561177
Polydactyly.
Society for Maternal-Fetal Medicine, Rac MWF, McKinney J, Gandhi M
Am J Obstet Gynecol 2019 Dec;221(6):B13-B15. doi: 10.1016/j.ajog.2019.09.023. PMID: 31787158
Non-invasive prenatal sequencing for multiple Mendelian monogenic disorders using circulating cell-free fetal DNA.
Zhang J, Li J, Saucier JB, Feng Y, Jiang Y, Sinson J, McCombs AK, Schmitt ES, Peacock S, Chen S, Dai H, Ge X, Wang G, Shaw CA, Mei H, Breman A, Xia F, Yang Y, Purgason A, Pourpak A, Chen Z, Wang X, Wang Y, Kulkarni S, Choy KW, Wapner RJ, Van den Veyver IB, Beaudet A, Parmar S, Wong LJ, Eng CM
Nat Med 2019 Mar;25(3):439-447. Epub 2019 Jan 28 doi: 10.1038/s41591-018-0334-x. PMID: 30692697
Prenatal Imaging of Craniosynostosis Syndromes.
Ketwaroo PD, Robson CD, Estroff JA
Semin Ultrasound CT MR 2015 Dec;36(6):453-64. Epub 2015 Jun 16 doi: 10.1053/j.sult.2015.06.002. PMID: 26614129
Saethre-Chotzen syndrome.
Reardon W, Winter RM
J Med Genet 1994 May;31(5):393-6. doi: 10.1136/jmg.31.5.393. PMID: 8064818Free PMC Article

Therapy

PIN1 is a new therapeutic target of craniosynostosis.
Shin HR, Bae HS, Kim BS, Yoon HI, Cho YD, Kim WJ, Choi KY, Lee YS, Woo KM, Baek JH, Ryoo HM
Hum Mol Genet 2018 Nov 15;27(22):3827-3839. doi: 10.1093/hmg/ddy252. PMID: 30007339Free PMC Article
Fetal Valproate Syndrome - Still a Problem Today!
Wiedemann K, Stüber T, Rehn M, Frieauff E
Z Geburtshilfe Neonatol 2017 Oct;221(5):243-246. Epub 2017 Oct 26 doi: 10.1055/s-0043-107619. PMID: 29073690
Craniosynostosis Following Fetal Methotrexate Exposure.
Zarella CS, Albino FP, Oh AK, Wood BC, Oluigbo CO, Myseros JS, Magge SN, Keating RF, Rogers GF
J Craniofac Surg 2016 Mar;27(2):450-2. doi: 10.1097/SCS.0000000000002423. PMID: 26890458
Maternofetal Trauma in Craniosynostosis.
Swanson J, Oppenheimer A, Al-Mufarrej F, Pet M, Arakawa C, Cunningham M, Gruss J, Hopper R, Birgfeld C
Plast Reconstr Surg 2015 Aug;136(2):214e-222e. doi: 10.1097/PRS.0000000000001468. PMID: 26218396
Cloverleaf skull and multiple congenital anomalies in a girl exposed to cocaine in utero: case report and review of the literature.
Esmer MC, Rodriguez-Soto G, Carrasco-Daza D, Iracheta ML, Del Castillo V
Childs Nerv Syst 2000 Mar;16(3):176-9; discussion 180. doi: 10.1007/s003810050488. PMID: 10804054

Prognosis

Polydactyly.
Society for Maternal-Fetal Medicine, Rac MWF, McKinney J, Gandhi M
Am J Obstet Gynecol 2019 Dec;221(6):B13-B15. doi: 10.1016/j.ajog.2019.09.023. PMID: 31787158
Non-invasive prenatal sequencing for multiple Mendelian monogenic disorders using circulating cell-free fetal DNA.
Zhang J, Li J, Saucier JB, Feng Y, Jiang Y, Sinson J, McCombs AK, Schmitt ES, Peacock S, Chen S, Dai H, Ge X, Wang G, Shaw CA, Mei H, Breman A, Xia F, Yang Y, Purgason A, Pourpak A, Chen Z, Wang X, Wang Y, Kulkarni S, Choy KW, Wapner RJ, Van den Veyver IB, Beaudet A, Parmar S, Wong LJ, Eng CM
Nat Med 2019 Mar;25(3):439-447. Epub 2019 Jan 28 doi: 10.1038/s41591-018-0334-x. PMID: 30692697
Apert syndrome with fused thalami.
Ludwig K, Salmaso R, Manara R, Cosmi E, Baldi M, Rugge M
Fetal Pediatr Pathol 2012 Dec;31(6):410-4. Epub 2012 Mar 23 doi: 10.3109/15513815.2012.659407. PMID: 22443264
Mortality and pathological findings in C (Opitz trigonocephaly) syndrome.
Opitz JM, Putnam AR, Comstock JM, Chin S, Byrne JL, Kennedy A, Frikke MJ, Bernard C, Albrecht S, Der Kaloustian V, Szakacs JG
Fetal Pediatr Pathol 2006 Jul-Aug;25(4):211-31. doi: 10.1080/15513810601015753. PMID: 17162528
Transvaginal Doppler assessment of fetal intracranial venous flow.
Pooh RK, Pooh KH, Nakagawa Y, Maeda K, Fukui R, Aono T
Obstet Gynecol 1999 May;93(5 Pt 1):697-701. doi: 10.1016/s0029-7844(98)00546-8. PMID: 10912970

Clinical prediction guides

Fetal Trigonocephaly (Strawberry Skull) in Early Pregnancy.
Gover A, Khatib N, Beloosesky R, Bronshtein M
J Ultrasound Med 2022 Feb;41(2):505-509. Epub 2021 Mar 26 doi: 10.1002/jum.15707. PMID: 33769585
Non-invasive prenatal sequencing for multiple Mendelian monogenic disorders using circulating cell-free fetal DNA.
Zhang J, Li J, Saucier JB, Feng Y, Jiang Y, Sinson J, McCombs AK, Schmitt ES, Peacock S, Chen S, Dai H, Ge X, Wang G, Shaw CA, Mei H, Breman A, Xia F, Yang Y, Purgason A, Pourpak A, Chen Z, Wang X, Wang Y, Kulkarni S, Choy KW, Wapner RJ, Van den Veyver IB, Beaudet A, Parmar S, Wong LJ, Eng CM
Nat Med 2019 Mar;25(3):439-447. Epub 2019 Jan 28 doi: 10.1038/s41591-018-0334-x. PMID: 30692697
Central nervous system malformations and deformations in FGFR2-related craniosynostosis.
Khonsari RH, Delezoide AL, Kang W, Hébert JM, Bessières B, Bodiguel V, Collet C, Legeai-Mallet L, Sharpe PT, Fallet-Bianco C
Am J Med Genet A 2012 Nov;158A(11):2797-806. Epub 2012 Sep 17 doi: 10.1002/ajmg.a.35598. PMID: 22987770
Progressive development of sonographic features in prenatal diagnosis of Apert syndrome--case report and literature review.
Respondek-Liberska M, Smigiel R, Zielinski A, Sasiadek MM
Ginekol Pol 2010 Dec;81(12):935-9. PMID: 21391441
Ultrasound diagnosis of craniosynostosis.
Miller C, Losken HW, Towbin R, Bowen A, Mooney MP, Towbin A, Faix RS
Cleft Palate Craniofac J 2002 Jan;39(1):73-80. doi: 10.1597/1545-1569_2002_039_0073_udoc_2.0.co_2. PMID: 11772173

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