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2001, Number 5

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Rev ADM 2001; 58 (5)

Beckwith-Wiedemann syndrome. Three case report

Toranzo FJM, Duarte HS, Rodríguez PA
Full text How to cite this article

Language: Spanish
References: 9
Page: 170-172
PDF size: 166.26 Kb.


Key words:

Beckwith-Wiedemann, syndrome, macroglosia.

ABSTRACT

Beckwith-Wiedemann syndrome was first described in 1963. It is a rare congenital disease associated with macrosomy, onfalocele, craniofacial dismorfism, macroglosia, visceromegaly, hemihypertrophy and Wilms tumors. Head and neck signsare of variable expression including dental and cranio-facial anomalies. Three cases are reviewed and treated with partial glossectomy.


REFERENCES

  1. Beckwith JB. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas and leydig-cell hyperplasia: another syndrome? Personal communication. Annual Meeting of Western Society for Pediatric Research. Los Angeles, C.A Nov. 1965.

  2. Salmon MA. Beckwith-Wiedemann Syndrome. Developmental defects and syndromes. 89-91.

  3. Menard RM, Delaire J, Schendel SA. Treatment of the craniofacial complications of Beckwith-Wiedemann syndrome. Plastic Reconstructive Surgery 1995; 96(1): 27-31.

  4. Wiedemann HR. Complexe malformatif familial avec hernie ombilicale et macrogossie: Un syndrome nouveau. JO Genetic Hum 1964; 13: 223.

  5. Rimell FL, Shapiro AM, Shoemaker DL, Kenna MA. Head and neck manifestations on Beckwith-Wiedemann syndrome. Department of Pediatric Otolaryngology, Children’s Hospital of Pittsburgh. USA.

  6. Elliott M, Bayly R, Cole T, Temple IK, Maher ER. Clinical features and natural history of Beckwith-Wiedemann syndrome: presentation of 74 new cases. Clinic genet 1994; 46(2): 168-74.

  7. Henry V Kruchinsky. A new tongue reduction method. J Oral maxillofacial Surgery 1990; 48: 756-757.

  8. Chan KC, Cheung WK, Chen YC. Incomplete forms of Beckwith-Wiedemann syndrome: Report of a case. J Formos med Assoc 1994; 93(9): 813-5.

  9. Pedro Diz Dios. Treatment of macroglossia in a child with Beckwith-Wiedemann Syndrome. Universidad Santiago de Compostela. Spain. J Oral Maxillofacial Surgery 2000; 58: 1058-1061




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C?MO CITAR (Vancouver)

Rev ADM. 2001;58