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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
Prenatal diagnosis of Greig Cephalopolysyndactyly Syndrome. When to suspect it.
Garcia-Rodriguez R, Rodriguez-Rodriguez R, Garcia-Delgado R, Romero-Requejo A, Medina-Castellano M, Garcia Cruz L, Santana Rodriguez A, Garcia-Hernandez JA
J Matern Fetal Neonatal Med 2022 Jun;35(11):2162-2165. Epub 2020 Jun 4 doi: 10.1080/14767058.2020.1774541. PMID: 32495660
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

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
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
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
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

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
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
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
Craniosynostosis update 1987.
Cohen MM Jr
Am J Med Genet Suppl 1988;4:99-148. doi: 10.1002/ajmg.1320310514. PMID: 3144990

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
Craniosynostosis and fetal exposure to sodium valproate.
Lajeunie E, Barcik U, Thorne JA, El Ghouzzi V, Bourgeois M, Renier D
J Neurosurg 2001 Nov;95(5):778-82. doi: 10.3171/jns.2001.95.5.0778. PMID: 11702867

Prognosis

Prenatal diagnosis of Pfeiffer syndrome and role of three-dimensional ultrasound: case report and review of literature.
Vimercati A, Olivieri C, Dellino M, Gentile M, Tinelli R, Cicinelli E
J Matern Fetal Neonatal Med 2022 Dec;35(25):7840-7843. Epub 2021 Jun 28 doi: 10.1080/14767058.2021.1937984. PMID: 34182859
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 sonographic findings and prognosis of craniosynostosis diagnosed during the fetal and neonatal periods.
Harada A, Miyashita S, Nagai R, Makino S, Murotsuki J
Congenit Anom (Kyoto) 2019 Jul;59(4):132-141. Epub 2018 Sep 5 doi: 10.1111/cga.12308. PMID: 30132994
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

Clinical prediction guides

CYP26B1-related disorder: expanding the ends of the spectrum through clinical and molecular evidence.
Silveira KC, Fonseca IC, Oborn C, Wengryn P, Ghafoor S, Beke A, Dreseris ES, Wong C, Iacovone A, Soltys CL, Babul-Hirji R, Artigalas O, Antolini-Tavares A, Gingras AC, Campos E, Cavalcanti DP, Kannu P
Hum Genet 2023 Nov;142(11):1571-1586. Epub 2023 Sep 27 doi: 10.1007/s00439-023-02598-2. PMID: 37755482Free PMC Article
De novo nonsense variant in ASXL3 in a Chinese girl causing Bainbridge-Ropers syndrome: A case report and review of literature.
Wang Q, Zhang J, Jiang N, Xie J, Yang J, Zhao X
Mol Genet Genomic Med 2022 May;10(5):e1924. Epub 2022 Mar 11 doi: 10.1002/mgg3.1924. PMID: 35276034Free PMC Article
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
Alobar holoprosencephaly associated with a rare chromosomal abnormality: Case report and literature review.
Ionescu CA, Calin D, Navolan D, Matei A, Dimitriu M, Herghelegiu C, Ples L
Medicine (Baltimore) 2018 Jul;97(29):e11521. doi: 10.1097/MD.0000000000011521. PMID: 30024536Free PMC Article

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