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Published in: Aesthetic Plastic Surgery 3/2021

01-06-2021 | Liposuction | Review

Madelung Disease Epidemiology and Clinical Characteristics: a Systemic Review

Authors: Quanzhe Liu, Haozhen Lyu, Bin Xu, Jae Hyup Lee

Published in: Aesthetic Plastic Surgery | Issue 3/2021

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Abstract

Background

Madelung disease is a rare lipid metabolic disorder, and most cases are sporadically reported. There are currently no systematic reviews summarizing the epidemiological and clinical characteristics of this disease. The purpose of the current article is to extract and analyze the existing evidence concerning Madelung disease derived from case series in order to provide adequate treatments for patients based on a more comprehensive understanding of the disease.

Methods

PubMed, Embase, and Web of Science databases were queried for relevant articles using the search terms “Madelung disease,” “multiple symmetric lipomatosis,” “Launois-Bensaude syndrome” and synonyms until Aug 31,2020. Data statistics of Madelung disease epidemiology and clinical characteristics are summarized in different tables or charts with Microsoft Office software.

Results

Patients exhibiting Madelung disease were mostly located in Europe, although some records existed in Asia as well. Average patient age was between 45 and 65 years old. Type I was the most common form of the disease, and the neck was the most common location for tumors. Madelung disease is associated with various metabolic disorders, and hematoma and seroma were the most common complications. Overall recurrence rate was 18.3%, with similar recurrence rates after lipectomy and liposuction. Fewer complications occurred after liposuction compared with lipectomy, but relapse was more common after liposuction.

Conclusion

Madelung disease consists of specific epidemiological and clinical characteristics, knowledge of which can be helpful for diagnosis and cognition. Lipectomy and liposuction are considered to be the most effective treatment methods for Madelung disease; however, choice of surgery should be based on comprehensive consideration of the disease, such as severity, mass location, and patient expectations.

Level of Evidence IV

This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.​springer.​com/​00266.
Literature
1.
go back to reference Brodie BC (1842) Clinical lectures—delivered at St. George’s Hospital. Provincial Med Surg J 3:443 Brodie BC (1842) Clinical lectures—delivered at St. George’s Hospital. Provincial Med Surg J 3:443
2.
go back to reference Ramos S, Pinheiro S, Diogo C, Cabral L, Cruzeiro C (2010) Madelung disease: a not-so-rare disorder. Ann Plast Surg 64:122–124CrossRef Ramos S, Pinheiro S, Diogo C, Cabral L, Cruzeiro C (2010) Madelung disease: a not-so-rare disorder. Ann Plast Surg 64:122–124CrossRef
3.
go back to reference Morinaka S, Sato T, Miyoshi H, Iwashita K (1999) A case of multiple symmetrical lipomatosis (Madelung’s disease). Auris Nasus Larynx 26:349–353CrossRef Morinaka S, Sato T, Miyoshi H, Iwashita K (1999) A case of multiple symmetrical lipomatosis (Madelung’s disease). Auris Nasus Larynx 26:349–353CrossRef
4.
go back to reference Abenavoli FM, Corelli R, D’Erme G (2002) Madelung’s disease: which surgical treatment? Br J Plast Surg 55:536–537CrossRef Abenavoli FM, Corelli R, D’Erme G (2002) Madelung’s disease: which surgical treatment? Br J Plast Surg 55:536–537CrossRef
5.
go back to reference Parmar SC, Blackburn C (1996) Madelung’s disease—an uncommon disorder of unknown aetiology. Br J Oral Maxillofac Surg 34:467–470CrossRef Parmar SC, Blackburn C (1996) Madelung’s disease—an uncommon disorder of unknown aetiology. Br J Oral Maxillofac Surg 34:467–470CrossRef
6.
go back to reference Torres M, Rodríguez J, Salvatierra B, Gilsanz F (2009) Airway management with the Airtraq in a patient with Launois-Bensaude syndrome. Rev Esp Anestesiol Reanim 56:579–581CrossRef Torres M, Rodríguez J, Salvatierra B, Gilsanz F (2009) Airway management with the Airtraq in a patient with Launois-Bensaude syndrome. Rev Esp Anestesiol Reanim 56:579–581CrossRef
7.
go back to reference Mégevand C, Savoy J, Boudrama A, Meier D (2000) Madelung disease and obstructive sleep apnea syndrome: apropos of a case. Schweiz Med Wochenschr Suppl 116:123s–126sPubMed Mégevand C, Savoy J, Boudrama A, Meier D (2000) Madelung disease and obstructive sleep apnea syndrome: apropos of a case. Schweiz Med Wochenschr Suppl 116:123s–126sPubMed
8.
go back to reference Fedriani JJ (2014) Two different airway-management strategies in patients with Launois-Bensaude lipomatosis. Rev Esp Anestesiol Reanim 61:353–354CrossRef Fedriani JJ (2014) Two different airway-management strategies in patients with Launois-Bensaude lipomatosis. Rev Esp Anestesiol Reanim 61:353–354CrossRef
9.
go back to reference Conroy JP (2006) Airway management: a patient with Madelung disease. Aana J 74:281–284PubMed Conroy JP (2006) Airway management: a patient with Madelung disease. Aana J 74:281–284PubMed
10.
go back to reference Plummer C, Spring PJ, Marotta R, Chin J, Taylor G, Sharpe D, Athanasou NA, Thyagarajan D, Berkovic SF (2013) Multiple Symmetrical Lipomatosis—a mitochondrial disorder of brown fat. Mitochondrion 13:269–276CrossRef Plummer C, Spring PJ, Marotta R, Chin J, Taylor G, Sharpe D, Athanasou NA, Thyagarajan D, Berkovic SF (2013) Multiple Symmetrical Lipomatosis—a mitochondrial disorder of brown fat. Mitochondrion 13:269–276CrossRef
11.
go back to reference Enzi G, Inelmen EM, Baritussio A, Dorigo P, Prosdocimi M, Mazzoleni F (1977) Multiple symmetric lipomatosis—a defect in adrenergic-stimulated lipolysis. J Clin Invest 60:1221–1229CrossRef Enzi G, Inelmen EM, Baritussio A, Dorigo P, Prosdocimi M, Mazzoleni F (1977) Multiple symmetric lipomatosis—a defect in adrenergic-stimulated lipolysis. J Clin Invest 60:1221–1229CrossRef
12.
go back to reference Moher D, Liberati A, Tetzlaff J, Altman DG, Prisma Group (2009) Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med 6:e1000097CrossRef Moher D, Liberati A, Tetzlaff J, Altman DG, Prisma Group (2009) Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med 6:e1000097CrossRef
13.
go back to reference Jüni P, Altman DG, Egger M (2001) Systematic reviews in health care—assessing the quality of controlled clinical trials. BMJ 323:42–46CrossRef Jüni P, Altman DG, Egger M (2001) Systematic reviews in health care—assessing the quality of controlled clinical trials. BMJ 323:42–46CrossRef
14.
go back to reference Pinto CI, Carvalho PJ, Correia MM (2017) Madelung’s disease: revision of 59 surgical cases. Aesthetic Plast Surg 41:359–368CrossRef Pinto CI, Carvalho PJ, Correia MM (2017) Madelung’s disease: revision of 59 surgical cases. Aesthetic Plast Surg 41:359–368CrossRef
15.
go back to reference Luscher NJ, Prein J, Spiessl B (1986) Lipomatosis of the neck (Madelung’s neck). Ann Plast Surg 16:502–508CrossRef Luscher NJ, Prein J, Spiessl B (1986) Lipomatosis of the neck (Madelung’s neck). Ann Plast Surg 16:502–508CrossRef
16.
go back to reference Wong DS, Lam LK, Chung JH, Ng RW, Li GK, Chan VS (2003) Aesthetic considerations in the cervicofacial management of Madelung syndrome. Scand J Plast Reconstr Surg Hand Surg 37:34–40CrossRef Wong DS, Lam LK, Chung JH, Ng RW, Li GK, Chan VS (2003) Aesthetic considerations in the cervicofacial management of Madelung syndrome. Scand J Plast Reconstr Surg Hand Surg 37:34–40CrossRef
17.
go back to reference Constantinidis J, Steinhart H, Zenk J, Gassner H, Iro H (2003) Combined surgical lipectomy and liposuction in the treatment of benign symmetrical lipomatosis of the head and neck. Scand J Plast Reconstr Surg Hand Surg 37:90–96CrossRef Constantinidis J, Steinhart H, Zenk J, Gassner H, Iro H (2003) Combined surgical lipectomy and liposuction in the treatment of benign symmetrical lipomatosis of the head and neck. Scand J Plast Reconstr Surg Hand Surg 37:90–96CrossRef
18.
go back to reference Enzi G, Angelini CO, Negrin P, Armani MA, Pierobon S, Fedele DO (1985) Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis. Medicine (Baltimore) 64:388–393CrossRef Enzi G, Angelini CO, Negrin P, Armani MA, Pierobon S, Fedele DO (1985) Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis. Medicine (Baltimore) 64:388–393CrossRef
19.
go back to reference Gao Y, Hu JL, Zhang XX, Zhang MS, Lu Y (2017) Madelung’s disease: Is insobriety the chief cause? Aesthetic Plast Surg 41:1208–1216CrossRef Gao Y, Hu JL, Zhang XX, Zhang MS, Lu Y (2017) Madelung’s disease: Is insobriety the chief cause? Aesthetic Plast Surg 41:1208–1216CrossRef
20.
go back to reference Bassetto F, Scarpa C, De Stefano F, Busetto L (2014) Surgical treatment of multiple symmetric lipomatosis with ultrasound-assisted liposuction. Ann Plast Surg 73:559–562CrossRef Bassetto F, Scarpa C, De Stefano F, Busetto L (2014) Surgical treatment of multiple symmetric lipomatosis with ultrasound-assisted liposuction. Ann Plast Surg 73:559–562CrossRef
21.
go back to reference Verhelle NA, Nizet JL, Guelinckx P, Heymans O (2003) Liposuction in benign symmetric lipomatosis: sense or senseless? Aesthetic Plast Surg 27:319–321CrossRef Verhelle NA, Nizet JL, Guelinckx P, Heymans O (2003) Liposuction in benign symmetric lipomatosis: sense or senseless? Aesthetic Plast Surg 27:319–321CrossRef
22.
go back to reference Ujpal M, Nemeth ZS, Reichwein A, Szabo GY (2001) Long-term results following surgical treatment of benign symmetric lipomatosis (BSL). Int J Oral Maxillofac Surg 30:479–483CrossRef Ujpal M, Nemeth ZS, Reichwein A, Szabo GY (2001) Long-term results following surgical treatment of benign symmetric lipomatosis (BSL). Int J Oral Maxillofac Surg 30:479–483CrossRef
23.
go back to reference Lee HW, Kim TH, Cho JW, Ryu BY, Kim HK, Choi CS (2003) Multiple symmetric lipomatosis: Korean experience. Dermatol Surg 29:235–240PubMed Lee HW, Kim TH, Cho JW, Ryu BY, Kim HK, Choi CS (2003) Multiple symmetric lipomatosis: Korean experience. Dermatol Surg 29:235–240PubMed
24.
go back to reference Enzi G, Favaretto L, Martini S, Fellin R, Baritussio A, Baggio G, Crepaldi G (1983) Metabolic abnormalities in multiple symmetric lipomatosis: elevated lipoprotein lipase activity in adipose tissue with hyperalphalipoproteinemia. J Lipid Res 24:566–574CrossRef Enzi G, Favaretto L, Martini S, Fellin R, Baritussio A, Baggio G, Crepaldi G (1983) Metabolic abnormalities in multiple symmetric lipomatosis: elevated lipoprotein lipase activity in adipose tissue with hyperalphalipoproteinemia. J Lipid Res 24:566–574CrossRef
25.
go back to reference Tremp M, Wettstein R, Tchang LA, Schaefer DJ, Rieger UM, Kalbermatten DF (2015) Power-assisted liposuction (PAL) of multiple symmetric lipomatosis (MSL)—a longitudinal study. Surg Obes Relat Dis 11:155–160CrossRef Tremp M, Wettstein R, Tchang LA, Schaefer DJ, Rieger UM, Kalbermatten DF (2015) Power-assisted liposuction (PAL) of multiple symmetric lipomatosis (MSL)—a longitudinal study. Surg Obes Relat Dis 11:155–160CrossRef
26.
go back to reference Brea-García B, Cameselle-Teijeiro J, Couto-González I, Taboada-Suárez A, González-Álvarez E (2013) Madelung’s disease: comorbidities, fatty mass distribution, and response to treatment of 22 patients. Aesthetic Plast Surg 37:409–416CrossRef Brea-García B, Cameselle-Teijeiro J, Couto-González I, Taboada-Suárez A, González-Álvarez E (2013) Madelung’s disease: comorbidities, fatty mass distribution, and response to treatment of 22 patients. Aesthetic Plast Surg 37:409–416CrossRef
27.
go back to reference Mevio E, Sbrocca M, Mullace M, Viglione S, Mevio N (2012) Multiple symmetric lipomatosis: a review of 3 cases. Case Rep Otolaryngol 2012:910526PubMedPubMedCentral Mevio E, Sbrocca M, Mullace M, Viglione S, Mevio N (2012) Multiple symmetric lipomatosis: a review of 3 cases. Case Rep Otolaryngol 2012:910526PubMedPubMedCentral
28.
go back to reference Enzi GI (1984) Multiple symmetric lipomatosis—an updated clinical report.pdf. Medicine 63:56–64CrossRef Enzi GI (1984) Multiple symmetric lipomatosis—an updated clinical report.pdf. Medicine 63:56–64CrossRef
29.
go back to reference Busetto L, Sträter D, Enzi G, Coin A, Sergi G, Inelmen EM, Pigozzo S (2003) Differential clinical expression of multiple symmetric lipomatosis in men and women. Int J Obes Relat Metab Disord 27:1419–1422CrossRef Busetto L, Sträter D, Enzi G, Coin A, Sergi G, Inelmen EM, Pigozzo S (2003) Differential clinical expression of multiple symmetric lipomatosis in men and women. Int J Obes Relat Metab Disord 27:1419–1422CrossRef
30.
go back to reference Leung NW, Gaer J, Beggs D, Kark AE, Holloway B, Peters TJ (1987) Multiple symmetric lipomatosis (Launois-Bensaude syndrome)-effect of oral salbutamol. Clin Endocrinol 7:601406 Leung NW, Gaer J, Beggs D, Kark AE, Holloway B, Peters TJ (1987) Multiple symmetric lipomatosis (Launois-Bensaude syndrome)-effect of oral salbutamol. Clin Endocrinol 7:601406
31.
go back to reference Faga A, Valdatta LA, Thione A, Buoro M (2001) Ultrasound assisted liposuction for the palliative treatment of Madelung’s disease: a case report. Aesthetic Plast Surg 25:181–183CrossRef Faga A, Valdatta LA, Thione A, Buoro M (2001) Ultrasound assisted liposuction for the palliative treatment of Madelung’s disease: a case report. Aesthetic Plast Surg 25:181–183CrossRef
32.
go back to reference DiBernardo BE (2010) Randomized, blinded split abdomen study evaluating skin shrinkage and skin tightening in laser-assisted liposuction versus liposuction control. Aesthet Surg J 30:593–602CrossRef DiBernardo BE (2010) Randomized, blinded split abdomen study evaluating skin shrinkage and skin tightening in laser-assisted liposuction versus liposuction control. Aesthet Surg J 30:593–602CrossRef
33.
go back to reference Parlette EC, Kaminer ME (2008) Laser-assisted liposuction: here’s the skinny. Semin Cutan Med Surg 27:259–263CrossRef Parlette EC, Kaminer ME (2008) Laser-assisted liposuction: here’s the skinny. Semin Cutan Med Surg 27:259–263CrossRef
34.
go back to reference Glottic CT, Neck G, Lipectomy NT, Supraglottic CT (2012) Laryngeal involvement in Madelung’s disease with acute airway compromise. Kaohsiung J Med Sci 28:462–463CrossRef Glottic CT, Neck G, Lipectomy NT, Supraglottic CT (2012) Laryngeal involvement in Madelung’s disease with acute airway compromise. Kaohsiung J Med Sci 28:462–463CrossRef
Metadata
Title
Madelung Disease Epidemiology and Clinical Characteristics: a Systemic Review
Authors
Quanzhe Liu
Haozhen Lyu
Bin Xu
Jae Hyup Lee
Publication date
01-06-2021
Publisher
Springer US
Published in
Aesthetic Plastic Surgery / Issue 3/2021
Print ISSN: 0364-216X
Electronic ISSN: 1432-5241
DOI
https://doi.org/10.1007/s00266-020-02083-5

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