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A case of severe obstructive sleep apnoea in Madelung's disease treated by lateral pharyngoplasty

Published online by Cambridge University Press:  23 May 2017

V Pinto ,
P G Morselli ,
D Tassone  and
O Piccin
V Pinto
Affiliation:
Department of Plastic and Reconstructive Surgery, S Orsola-Malpighi University Hospital, Bologna, Italy
P G Morselli
Affiliation:
Department of Plastic and Reconstructive Surgery, S Orsola-Malpighi University Hospital, Bologna, Italy
D Tassone
Affiliation:
Department of Plastic and Reconstructive Surgery, S Orsola-Malpighi University Hospital, Bologna, Italy
O Piccin*
Affiliation:
Department of Otolaryngology Head and Neck Surgery, S Orsola-Malpighi University Hospital, Bologna, Italy
*
Address for correspondence: Dr Ottavio Piccin, Department of Otolaryngology, S Orsola-Malpighi University Hospital, Via Massarenti 9, Bologna 40138, Italy Fax: +39 051 636 3525 E-mail: ottavio.piccin@gmail.com
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Abstract

Background:

Madelung's disease is a rare disorder characterised by the presence of multiple, symmetric, non-capsulated fat masses in the face, neck and other areas of upper extremities. In some cases, severe clinical complications such as upper airway compression can occur.

Case report:

A 56-year-old man affected by Madelung's disease complained of snoring and severe daytime sleepiness. Polysomnography revealed severe obstructive sleep apnoea. An attempt to treat sleep apnoea by continuous positive airway pressure failed because of poor compliance. Functional expansion pharyngoplasty was carried out as an initial treatment. Marked improvement of neck movements and normalisation of somnographic parameters were observed at six months’ follow up.

Conclusion:

Patients with Madelung's disease should be examined carefully for potential obstructive sleep apnoea. Although continuous positive airway pressure remains the treatment of choice, specific surgery can be used in those patients who cannot tolerate continuous positive airway pressure therapy.

Keywords

Madelung's DiseaseApneaObstructive SleepPharyngoplastyPolysomnographySleep Endoscopy

Information

Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 131 , Issue 9 , September 2017 , pp. 834 - 837
Copyright
Copyright © JLO (1984) Limited 2017 

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References

1 Josephson, GD, Sclafani, AP, Stern, J. Benign symmetric lipomatosis (Madelung's disease). Otolaryngol Head Neck Surg 1996;115:170–1CrossRefGoogle ScholarPubMed
2 Nisoli, E, Regianini, L, Briscini, L, Bulbarelli, A, Busetto, L, Coin, A et al. Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 2002;198:378–87Google Scholar
3 Costantinidis, J, Steinhart, H, Zenk, J, Gassner, H, Iro, H. Combined surgical lipectomy and liposuction in the treatment of benign symmetrical lipomatosis of the head and neck. Scand J Plast Reconstr Hand Surg 2003;37:90–6CrossRefGoogle Scholar
4 Ali, S, Kishore, A. Dysphagia and obstructive sleep apnoea in Madelung's disease. J Laryngol Otol 2007;121:398–400 CrossRefGoogle ScholarPubMed
5 Piccin, O, Pinto, V, Sorrenti, G. Surgical correction of lateral pharyngeal wall collapse in sleep-related disordered breathing: functional expansion pharyngoplasty. Oper Tech Otolaryngol Head Neck Surg 2015;26:221–4CrossRefGoogle Scholar
6 De Weerdt, S, Haentjens, P, Van Binst, AM, Baron, I, Clement, P, Vincken, W. Is there any relationship between nCPAP therapy and signs of sinus hyperpneumatization? B-ENT 2010;6:171–5Google ScholarPubMed
7 Smith, PD, Stadelmann, WK, Wassermann, RJ, Kearney, RE. Benign symmetric lipomatosis (Madelung's disease). Ann Plast Surg 1998;41:671–3CrossRefGoogle ScholarPubMed
8 Jalladeau, E, Crestani, B, Laissy, JP, Vecchierini, MF. A case of obstructive sleep apnea syndrome: unusual comorbidity. Clin Respir J 2008;2:239–41CrossRefGoogle ScholarPubMed
9 Oliven, A, Kaufman, E, Kaynan, R, Oliven, R, Steinfeld, U, Tov, N et al. Mechanical parameters determining pharyngeal collapsibility in patients with sleep apnea. J Appl Physiol (1985) 2010;109:1037–44CrossRefGoogle ScholarPubMed