A Case Report on Beckwith Wiedemann Syndrome
International Journal of Research and Reports in Dentistry,
Introduction: Beckwith Wiedemann Syndrome (BWS) is a congenital disorder that involves a somatic overgrowth during the patient’s first years of life and associated with visceromegaly, macroglossia, abdominal wall defects, pre and postnatal overgrowth, and neonatal hypoglycemia.
Case Report: This is a case report of a 14-year-old male patient who presented with macroglossia and Wilm’s Tumor. Diagnosis was made after karyotyping, which showed an abnormality in chromosome 11p15 and dental treatment was done.
Conclusion: Pediatric dentists should be aware of Beckwith Wiedemann Syndrome and its oral manifestations as they can encounter this condition in their clinical practice.
- Beckwith Wiedemann Syndrome
- Wilm’s tumour
How to Cite
Brioude F, Kalish JM, Mussa A, Foster AC, Bliek J, Ferrero GB, Boonen SE, Cole T, Baker R, Bertoletti M, Cocchi G. Clinical and molecular diagnosis, screening and management of beckwith–wiedemann syndrome: An international consensus statement. Nature Reviews Endocrinology. 2018;14(4):229-49.
Elliott M, Bayly R, Cole T, Temple IK, Maher ER. Clinical features and natural history of Beckwith‐Wiedemann syndrome: presentation of 74 new cases. Clinical Genetics. 1994;46(2):168-74.
Engström W, Lindham S, Schofield P. Wiedemann-beckwith syndrome. European Journal of Pediatrics. 1988;147(5): 450-7.
McManamny DS, Barnett JS. Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plastic and Reconstructive Surgery. 1985;75(2):170-6.
Weng EY, Mortier GR, Graham Jr. JM. Beckwith-Wiedemann syndrome: An update and review for the primary pediatrician. Clinical Pediatrics. 1995;34 (6):317-26.
Lim D, Bowdin SC, Tee L et al. Clinical and molecular genetic features of beckwith-wiedemann syndrome associated with assisted reproductive technologies. Hum Reprod. 2009;24(3):741-747.
Cooper WN, Luharia A, Evans GAet al. Molecular subtypes and phenotypic expression of beckwith-wiedemann syndrome. Eur J Hum Genet 2005;13(9): 1025-1032.
Lapunzina Badía P, del Campo Casanelles M, Delicado Navarro A et al. Clinical guide to the management of patients with beckwith-wiedemann syndrome. An Pediatr (Barc) 2006;64(3):252-259.
Beckwith JB. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas, and leydig-cell hyperplasia: Another syndrome? Abstract presented at: 11th Annual Meeting of the Western Society for Pediatric Research. Los Angeles, Calif; 1963.
Wiedemann HR. Familial malformation complex with umbilical hernia and macroglossia: A “new syndrome”? (in French). J Genet Hum. 1964;13:223-232.
Beckwith JB. Macroglossia, Omphalocele, adrenal cytomegaly, gigantism and hyperplastic visceromegaly. Birth Defects.1969; 5:188-196.
Therese Garvey M, Dympna Daly, Triona McNamara. Beckwith weidmann syndrome: Dental management. Int Journal of Paediatric Dent. 1997;7:95-99.
Cielo CM, Duffy KA, Taylor JA, Marcus CL, Kalish JM. Obstructive sleep apnea in children with Beckwith-Wiedemann syndrome. J Clin Sleep Med. 2019; 15(3):375–381.
Published 2019 Mar 15.
Cohen JL, Cielo CM, Kupa J et al. The utility of early tongue reduction surgery for macroglossia in beckwith-wiedemann syndrome. Plast Reconstr Surg. 2020;145(4):803e–813e.
Aldo Giancotti, Gaia Romanini, Raffaella Docimo, Claudio Arcuri. Clinical treatment of oral manifestations of beckwith-wiedemann syndrome in a child. J Clin Paediatr Dent. 2003;4: 377-380.
Gazzin A, Carli D, Sirchia F, Molinatto C, Cardaropoli S, Palumbo G, Zampino G, Ferrero GB, Mussa A. Phenotype evolution and health issues of adults with Beckwith‐Wiedemann syndrome. American Journal of Medical Genetics Part A. 2019;179(9):1691-702.
Santos TFR, Coradini CDB et al. Knowledge and practice of oral health in child patients with cancer. Arquivos em Odontologia. 2010;46(1):5–10.
Guideline on dental management of pediatric patients receiving chemotherapy, hematopoietic cell transplantation, and/or radiation therapy. Pediatr Dent. 2016; 38(6):334-342.
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