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Abstract

Objective

Macroglossia is a common feature of Beckwith Wiedemann Syndrome (BWS). Tongue reduction surgery (TRS) is advocated to overcome, or reduce, the secondary effects of macroglossia. Macroglossia may affect a child's cosmetic appearance, feeding, and drooling function. However, no study has systematically reported on feeding, drooling, or psychosocial outcomes preoperatively and postoperatively in this group. This study aimed to describe the presurgical and postsurgical psychosocial, feeding, and drooling outcomes of children with macroglossia associated with BWS and to determine the effect of TRS on these areas.

Design

Clinical cohort study.

Participants

Ten consecutively admitted children with BWS (age at surgery from 9 months to 4 years, 9 months [4;9]; mean, 2;7) were assessed preoperatively, 3 months postoperatively, and at long-term follow-up.

Main Outcome Measures

A parental report questionnaire, the Brodsky drooling scale, and a feeding rating scale.

Results

Parents reported that macroglossia had a negative impact cosmetically that was ameliorated following surgery. Macroglossia caused a range of feeding difficulties presurgically by preventing lip seal and bolus manipulation during the oral preparatory phase. Excessive drooling was present in all cases presurgically. This resolved partially across the group postsurgically, with almost complete recovery at longer-term follow-up assessment.

Conclusions

Presurgically, children show a common profile of feeding and drooling impairment with negative effects on cosmetic appearance. Our preliminary results demonstrate that TRS has a positive impact on these features with good outcomes for children with BWS.

Keywords

Beckwith Wiedemann cosmetic drooling feeding,macroglossia psychosocial surgery

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References

Berger

Z.E.

Dalton

L.J.

Coping with a cleft: psychosocial adjustment of adolescents with a cleft lip and palate and their parents. Cleft Palate Craniofac J. 2009; 46: 435–443.

Berger

Z.E.

Dalton

L.J.

Coping with a cleft II: factors associated with psychosocial adjustment of adolescents with a cleft lip and palate and their parents. Cleft Palate Craniofac J. 2011; 48: 82–90.

Blasco

P.A.

Allaire

J.H.

Drooling in the developmentally disabled: management practices and recommendations. Consortium on Drooling. Dev Med Child Neurol. 1992; 4: 849–862.

Brodsky

Drooling in children. In: Arvedsen

Brodsky

, eds. Paediatric Swallowing and Feeding. San Diego, CA: Singular; 1997: 395–398.

Cotton

R.T.

Richardson

M.A.

The effect of submandibular duct rerouting in the treatment of sialorrhea in children. Otlaryngol Head Neck Surg. 1981; 89: 535–541.

Crysdale

W.S.

Management options for the drooling patients. Ear Nose Throat J. 1989; 68: 820–830.

Crysdale

W.S.

White

Submandibular duct relocation for drooling: a 10 year experience with 194 patients. Otolaryngol Head Neck Surg. 1989; 101: 87–92.

Davalbhakta

Lamberty

B.G.H.

Technique for uniform reduction of macroglossia. Br J Plast Surg. 2000; 53: 294–297.

Egyedi

Obwegeser

Zur operativen zungen-verkleinerung. Dtsch Zahn Mund Kieferheik. 1964; 41: 16–25.

10.

Elliot

Maher

E.R.

Beckwith Wiedemann syndrome. J Med Genet. 1994; 31: 560–564.

11.

Elliott

M.R.C.

Bayly

Cole

Temple

I.K.

Maher

E.R.

Clinical features and natural history of Beckwith-Wiedemann syndrome: presentation of 74 new cases. Clin Genet. 1994; 46: 168–174.

12.

Engstrom

Lindham

Schofield

Wiedemann-Beckwith syndrome. Eur J Paediatr. 1988; 147: 450–457.

13.

Gisel

E.G.

Alphonce

Classification of eating impairments based on eating efficiency in children with cerebral palsy. Dysphagia. 1995; 10: 268–274.

14.

Hunt

Burden

Hepper

Stevenson

Johnson

Parent reports of the psychological functioning of children with cleft lip and/or palate. Cleft Palate Craniofac J. 2007; 44: 304–311.

15.

Kacker

Honrado

Martin

Ward

Tongue reduction in Beckwith Wiedemann syndrome. Int J Paediatr Otorhinolaryngol. 2000; 53: 294–297.

16.

Knapke

S.C.

Bender

R.N.

Prows

Schultz

J.R.

Saal

H.M.

Parental perspectives of children born with cleft lip and/or palate: a qualitative assessment of suggestions for healthcare improvements and interventions. Cleft Palate Craniofac J. 2010; 47: 143–150.

17.

Kveim

Fisher

J.C.

Jones

K.L.

Gruer

Early tongue resection for Beckwith Wiedemann macroglossia. Ann Plast Surg. 1985; 14: 142–144.

18.

Lespargot

Langevin

M.F.

Muller

Guillemont

Swallowing disturbances associated with drooling in cerebral-palsied children. Dev Med Child Neurol. 1993; 35: 298–304.

19.

Lew

K.M.

Younis

R.T.

Lazar

R.H.

The current management of sialorrhea. Ear Nose Throat J. 1991; 70: 99–105.

20.

Martinez

Clinical features in the Wiedemann-Beckwith syndrome. Clin Genet. 1994; 50: 273–274.

21.

McManamny

D.S.

Barnett

J.S.

Macroglossia as a presentation of the Beckwith Wiedemann syndrome. Plast Reconstr Surg. 1985; 75: 170–176.

22.

Mixter

R.C.

Fisher

J.C.

Jones

K.L.

Gruer

Central tongue reduction for macroglossia. Plast Reconstr Surg. 1993; 91: 1159–1162.

23.

Merks

J.H.

Caron

H.N.

Hennekam

R.C.

High incidence of malformation syndromes in a series of 1,073 children with cancer. Am J Med Genet. 2005; 134: 132–143.

24.

Murthy

Laing

M.R.

Macroglossia. Br Med J. 1994; 309: 1386–1387.

25.

Noar

J.H.

Questionnaire survey of attitudes and concerns of patients with cleft lip and palate and their parents. Cleft Palate Craniofac J. 1991; 28: 279–284.

26.

Pettenati

M.J.

Haines

J.L.

Higgins

R.R.

Wappner

R.S.

Palmer

C.G.

Weaver

D.D.

Wiedemann Beckwith syndrome: presentation of clinical and cytogenetic data on 22 new cases and review of the literature. Hum Genet. 1986; 74: 143–154.

27.

Rizer

F.M.

Schechter

G.L.

Richardson

M.A.

Macroglossia: etiological considerations and management techniques. Int J Paediatr Otorhinolaryngol. 1985; 8: 225–236.

28.

Salman

R.A.

Oral manifestations of Beckwith-Weidemann syndrome. Spec Care Dent. 1988; 8: 23–24.

29.

Shipster

Oliver

Morgan

Speech and oral motor skills in children with Beckwith Wiedemann Syndrome: pre- and post-tongue reduction surgery. Adv Speech Lang Pathol. 2006; 8: 45–55.

30.

Siddiqui

Pensler

J.M.

The efficacy of tongue resection in the treatment of symptomatic macroglossia in the child. Ann Plast Surg. 1990; 25: 14–17.

31.

Sokoloski

P.M.

Ogle

R.G.

Waite

D.E.

Surgical correction of Beckwith Wiedemann syndrome. J Oral Surg. 1978; 36: 212–215.

32.

Van Borsel

Van Snick

Leroy

Macroglossia and speech in Beckwith Wiedemann syndrome: a sample survey study. Int J Lang Commun Disord. 1999; 34: 209–221.

33.

Van De Heyning

P.H.

Marquet

J.F.

Creten

W.L.

Drooling in children with cerebral palsy. Acta Otorhinolaryngologica Belgica. 1980; 34: 691–705.

34.

Vogel

J.E.

Mulliken

J.B.

Kaban

L.B.

Macroglossia: a review of the condition and a new classification. Plast Reconstr Surg. 1986; 78: 715–723.

35.

Weksberg

Shuman

Smith

A.C.

Beckwith-Wiedemann syndrome. Am J Med Genet. 2005; 137: 12–23.

36.

Weng

E.Y.

Mortier

G.R.

Graham

Jr . Beckwith-Wiedemann syndrome: an update and review for the primary paediatrician. Clin Paediatr (Phil). 1995; 34: 317–326.

37.

Wolford

L.M.

Cottrell

D.A.

Diagnosis of macroglossia and indications for reduction glossectomy. Am J OrthodDentofac Orthop. 1996; 110: 170–177.

38.

Young

J.L.

What information do parents of newborns with cleft lip, palate or both want to know?

Cleft Palate Craniofac J. 2001; 38: 55–58.

Psychosocial,Feeding,and Drooling Outcomes in Children with Beckwith Wiedemann Syndrome following Tongue Reduction Surgery

Abstract

Objective

Design

Participants

Main Outcome Measures

Results

Conclusions

Keywords

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References