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Madelung's Neck: Cross-Sectional Imaging Observations

University Hospital Antwerp Edegem 2650, Belgium

A 53-year-old man with a history of alcohol abuse and with masses in the cervical region that had been gradually enlarging for several years presented to the surgeon. Contrast-enhanced helical CT of the neck was performed (Fig. 2A, 2B, 2C). Axial CT scans show symmetric fatty deposits under the trapezius and sternocleidomastoid muscles, between the paraspinal muscles, and in the anterior part of the neck. No extensive fat appears in the subcutaneous soft tissues.

View larger version (123K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A. —53-year-old man with history of alcohol abuse. Axial contrast-enhanced CT scans of neck show symmetric fatty deposits under trapezius and sternocleidomastoid muscles, between paraspinal muscles, and in anterior part of neck. Subcutaneous soft tissues do not have extensive fat.

View larger version (149K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B. —53-year-old man with history of alcohol abuse. Axial contrast-enhanced CT scans of neck show symmetric fatty deposits under trapezius and sternocleidomastoid muscles, between paraspinal muscles, and in anterior part of neck. Subcutaneous soft tissues do not have extensive fat.

View larger version (110K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C. —53-year-old man with history of alcohol abuse. Axial contrast-enhanced CT scans of neck show symmetric fatty deposits under trapezius and sternocleidomastoid muscles, between paraspinal muscles, and in anterior part of neck. Subcutaneous soft tissues do not have extensive fat.

Madelung's neck—or benign symmetric lipomatosis—is a rare lipodystrophic disease of unknown origin that was first mentioned by Brodie in 1846 and further defined by Madelung in Germany (1888) and Launois and Bensaude in France (1898). The main features are painless symmetric unencapsulated compartmentalized fatty deposits diffusely involving the head and neck region, shoulder girdle, and upper body. It usually presents in middle-aged men of Mediterranean descent and is associated with a history of alcohol abuse, malignant tumors of the upper airway, neuropathy, diabetes mellitus, hyperlipidemia, and other metabolic disorders [1–4]. Occurrence is usually sporadic, but some investigators believe an underlying hereditary factor may be present in the form of a mitochondrial dysfunction that disturbs the lipid metabolism [5].

These deposits tend to grow slowly over a period of years and in most cases have only a cosmetic impact. In rare cases, however, dyspnea and dysphagia can result from compression of the larynx, trachea, or cervical esophagus and require treatment [6]. Furthermore, Enzi et al. [7] recently reported an association with significant morbidity and mortality in a large long-term follow-up series, which brings into question the benign nature of the disease. The compartmentalization of the fatty deposits distinguishes this disease from obesity. No calcification, ossification, or invasion of adjacent vascular structures is associated with it [1].

These fatty masses can appear anywhere in the neck region but tend to be located deeply in the neck posterior relative to the trapezius and sternocleidomastoid muscles, in the supraclavicular fossa, or between the paraspinal muscles. Rarer locations include the anterior neck, superior mediastinum, pretracheal and prevertebral spaces, and cheeks [1, 2].

Surgical debulking is the treatment of choice; liposuction is used for small lesions. Recurrence is common, and surgical treatment often results in a less-than-optimal aesthetic outcome.

References

1. Delman BN, Weissman JL, Som PM. Skin and soft tissue lesions. In: Som PM, Curtin HD, eds. Head and neck imaging, 4th ed. St. Louis, MO: Mosby, 2003:2184
2. Ahuja AT, King AD, Chan ES, et al. Madelung disease: distribution of cervical fat and preoperative findings at sonography, MR, and CT. AJNR 1998;19:707 –710[Abstract]
3. Chan ES, Ahuja AT, King AD, Lau WY. Head and neck cancers associated with Madelung's disease. Ann Surg Oncol1999; 6:395 –397[Abstract]
4. Smith PD, Stadelmann WK, Wassermann RJ, Kearney RE. Benign symmetric lipomatosis (Madelung's disease). Ann Plast Surg 1998;41:671 –673[Medline]
5. Wu TP, Tsai JG, Chan PH, Lee HC, Wei YH. Mitochondrial respiratory function in multiple symmetrical lipomatosis: report of two cases.J Formos Med Assoc1994; 93:513 –518[Medline]
6. Adamo C, Vescio G, Battaglia M, Gallelli G, Musella S. Madelung's disease: case report and discussion of treatment options. Ann Plast Surg 2001;46:43 –45[Medline]
7. Enzi G, Busetto L, Ceschin E, Coin A, Digito M, Pigozzo S. Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study. Int J Obes Relat Metab Disord2002; 26:253 –261[Medline]

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