Laryngeal involvement in multiple symmetric lipomatosis: The role of computed tomography in diagnosis

Laryngeal Involvement in Multiple Symmetric Lipomatosis: The Role of Computed Tomography in Diagnosis Alexandra Borges, MD, Fernando and Elliot Abemayor, MD, PhD

Torrinha, MD, Robert B. Lufkin, MD,

Madelung’s disease (MD) is a rare lipodystrophy characterized by the presence of multiple, nonencapsulated fatty, infiltrative lesions that occur in a symmetric distribution. The disease predominately involves the neck, face, supralavicular and deltoid regions, the mediasti-

num, and the tongue. l Men are affected more often than women, and for unclear reasons, Mediterranean males are predominately afflicted.2-4

Patients

generally

seek medical

atten-

tion for cosmetic or compressive symptoms. Symptoms related to the larynx have been reported2-lo and are generally caused by extrinsic compression or, more rarely, compression of the recurrent of fat, leading

laryngeal

nerve

by infiltration

to dyspnea and dysphonia. Direct involvement of the larynx by proliferating adipose tissue is rare, with only five cases

reported in the literature.4,6~7,sJ0 We report two pathologically proven of MD, with striking direct involvement larynx, diagnosed by computed tomography

cases of the (CT).

CASE REPORTS

sinoatrial nodal disease, and a transvenous pacemaker was placed at that time. Physical examination showed generalized enlargement of the neck without discrete masses. Laryngoscopic evaluation showed a marked decrease in the caliber of the lumen in the region of the vestibule and rima glottidis with bulging of the mucosa apparently secondary to extrinsic compression. No mucosal lesions were seen, and no cervical or supraclavicular adenopathy was detected. Except for enlargement of the superficial thoracic veins, the general physical examination was unremarkable. A CT scan (GE 9800 scanner; GE, Rochester, NY) was performed and showed massive infiltration of all infrahyoid neck spaces by tissue with an attenuation value of -98 Hounsfield units, consistent with fat. This tissue showed submucosal infiltration at all laryngeal levels and extended from the preepiglottic space inferiorly through the true and false vocal cords and anterior and posterior commissures to the inferior surface of the vocal cords, markedly reducing the laryngeal lumen. Mediastinal involvement was also noted that could, in part, account for the engorged thoracic veins. No soft tissue component was seen (Figs 1 through 3). The patient was offered laser resection of the intralaryngeal lesion, but he refused for fear of needing a tracheostomy.

Case 1 Case 2 A 69-year-old nonobese man with a history of moderate alcohol consumption was referred for evaluation of progressive chronic dysphonia. The patient complained of dyspnea on exertion and paroxysmal nocturnal dyspnea for the past 4 years. A cardiac work-up done 3 years previously showed From the Department of Radiology, lnstituto Portugues de Oncologia, Lisboa, Portugal, and the Department of Radiological Sciences and the Division of Head and Neck Surgery, University of California-Los Angeles School of Medicine, Los Angeles, CA. Address reprint requests to Elliot Abemayor, MD, PhD, Professor, Division of Head and Neck Surgery, UCLA Center for Health Sciences, Rm 62-l 58, 10833 Le Conte Ave, Los Angeles, CA 90095. Copyright o 1997 by W.B. Saunders Company 0196-0709/97/l 802-0011$5.00/O American Journal of Otolaryngology,

A 66-year-old man was referred for evaluation of progressive neck swelling, dysphonia, and nonproductive cough of 8 months’ duration. There were no complaints of dyspnea or dysphagia, and no history of alcohol consumption or smoking was elicited. Results of the physical examination showed painless discrete masses in the anterolateral and posterior compartments of the neck and the postauricular area. Laryngoscopic evaluation showed reduction of the laryngeal lumen and prominent but mobile vocal cords. The mucosa covering the true and false vocal cords had a yellowish glistening appearance, but no discrete lesions or friability were apparent. General physical examination was otherwise unremarkable. The CT examination again showed striking infiltration of all compartments of the infrahyoid and

Vol 18, No 2 (March-April),

1997: pp 127-l 30

127

128

Fig 1. CT axial scan at the supraglottic level obtained during quiet breathing. This image shows marked infiltration of the supraglottic compartment by a tissue with fat density (- 98 HU), which is reducing the lumen of the laryngeal vestibule. Fatty infiltration is also noted in the prelaryngeal soft tissues, carotid space, and posterior triangle. The carotid arteries, jugular vein, and sternocleidomastoid muscle seen to float in a sea of fat.

BORGES

ET AL

Fig 3. CT axial scan at the supraglottic level. This image shows fatty infiltration of the preepiglottic space, reducing the lumen of the laryngeal vestibule. Marked adipose tissue infiltration is also seen involving the retropharyngeal space.

DISCUSSION suprahyoid neck by adipose tissue. Laryngeal infiltration was also noted involving the supraglottic and glottic levels, with marked involvement of the vocal cords by the presence of discreet lipomatous masses (Figs 4 and 5). A biopsy sample of a postauricular discrete, mobile mass was taken, and the pathological diagnosis was “adipose tissue,” consistent with the diagnosis of Madelung’s disease. No further surgery was performed.

Fig 2. CT axial scan at the infraglottic level. This image shows the inferior extension of the fat tissue infiltration though the infraglottic compartment with fat deep to the true vocal cord.

Multiple symmetric lipomatosis is generally easily diagnosed on physical examination. However, the diagnosis may be in question when the disease occurs in unusual organs, such as the larynx. 1 Symptoms related to the larynx are generally the result of extrinsic compression from adjacent proliferating tis-

Fig 4. CT axial vocal cords showing

scan through the level of the adipose tissue infiltration.

false

LARYNGEAL

LIPOMATOSIS

129

Fig 5. CT axial scan at the level of the cords during breath holding also showing infiltration along all the extent of the cords.

other cases of clinically proven direct involvement of the larynx (Table 1). All patients, including the two described in the current study, were men between the ages of 44 and 71 years old, and the symptoms necessitating medical care included dysphonia, laryngeal dyspnea, wheezing, and nonproductive cough. Physical examination showed involvement of the supraglottis (5 patients), glottis (7 patients), and infraglottis (3 patients). Reduction of the laryngeal lumen was seen in all patients. Clinically, there was a yellowish glistening appearance of the laryngeal mucosa in three patients and decreased mobility of the true vocal cords with associated edema also in three patients. Radiological evaluation was performed in six patients. One patient had plain radiographs and fluoroscopic examination only. This showed enlargement of the larynx and reduction of the laryngeal air column. Two patients had laryngeal linear tomograms. One examination showed nonspecific fullness of the supraglottic and glottic levels with reduction of the laryngeal lumen and the

true vocal fat tissue

sue or, more rarely, compression of the recurrent laryngeal nerve. The latter can be seen when fat infiltrates the surrounding tissues. Review of the literature5-*Jo disclosed five TABLE 1.

Laryngeal

Involvement

in Madelung’s

Disease:

of the Literature

Patient

Sex/ Age

1

Ml63

Dyspnea, dysphagia

Supraglottic, glottic

Edema of the true vocal cords and yellowish, glistening masses

Linear

2

M/57

Progressive dyspnea, stridor, neck swelling

Transglottic

None

3

Mi71

Dyspnea

Glottic

4

M/53

Dyspnea, stridor, dysphonia

Supraglottic, glottic

Hypertrophy of the false vocal cords, yellowish glistening masses, hypomobility of TVC Hypertrophy of the TVC, hypomobility of the TVC Edema of the TVC and FVC, hypomobility of the TVC

5

M/44

Dysphonia, cough, swelling

Glottic,

6*

M/66

Dysphonia, dyspnea

Supraglottic, glottic

Hypertrophy of the FVC with a yellowish glistening appearance

Computerized tomography

7*

M/69

Dysphonia, cough,

Transglottic neck

Hypertrophy and TVC mucosa

Computerized tomography

F, female;

FVC,

Abbreviations:

Laryngeal Involvement

Review

Symptoms

false

vocal

Laryngoscopic Findings

infraglottic

cord:

M, Male;

Edema

TVC,

Imaging

of the TVC

cord.

Radiologic

Findings

tomography

Reduction of the lumen at the level of the vestibule, rima and glottis -

tomography

Vocal cord thickening with discrete masses in both vocal cords Symmetric reduction of the laryngeal lumen at the vestibule, rima and glottis Infiltration of the false vocal cords and infraglottis by adipose tissue Infiltration of the supraglottic and glottic levels by adipose tissue Submucosal fatty infiltration at all laryngeal levels

Simple radiograms of the neck

Computerized tomography

of the FVC with bulging

true vocal

Linear

Modality

130

other, enlargement of the vocal cords. CT was performed in three patients and accurately determined not only the extent of laryngeal involvement, but also the adipose nature of the infiltrating tissue. There are few data about the radiological evaluation of laryngeal involvement in Madelung’s disease. CT is clearly superior to laryngeal radiographs and linear tomograms, because the latter two lack contrast resolution, No data are currently available concerning imaging of the larynx in Madelung’s disease with magnetic resonance imaging (MRI). However, MRI was used to evaluate neck disease in two patients,ll with good results on spinecho Tl-weighted sequences but more data will have to be collected to evaluate this matter. Imaging these patients with CT has three main goals: differential diagnosis from diseases with a similar clinical picture (submucosal infiltration of the larynx by granulomatous diseases or amyloid); determining the extent of the infiltrating adipose tissue; and excluding malignancy. The diffuse infiltrative pattern cannot by itself differentiate fat from other tissue (eg, amyloid). However, the lack of cartilage invasion or effacement of surrounding tissue planes may be used to exclude malignancy. Perhaps more sensitive anatomic delineation would result with use of gadolinium-enhanced MRI scanning. A biopsy needs to be performed in all cases because liposarcoma can not be excluded solely on radiologic basis.12 Only one case of malignant degeneration of Madelung’s disease is reported in the literature,4 corresponding to an incidence of 0.5% (1:ZOO cases), which could justify radiologic follow-up of these

BORGES

ET AL

patients. Among these six patients, only two had surgical treatment: laryngofissure operation in one7 and resection of a vocal cord lipoma in the other. lo All the remaining were managed conservatively with improvement of their clinical condition.

REFERENCES 1. Katou F, Shirai N, Motegi K, et al: Symmetrical lipomatosis of the tongue presenting as macroglossia. J Cranio maxi110 fat Surg 21:298-301,1993 2. Plotnicov NA, Babayev TA, Lamberg MA, et al: Madelung’s disease (benign symmetric lipomatosis). Oral Surg Oral Med Oral Path01 66:171-175,198s 3. Boozan JA, Maves MD, Schuller DE: Surgical management of massive benign symmetric lipomatosis. Laryngoscope 102:94-99,1992 4. Norem-Coker JE, Bryan JA: Madelung’s disease: A case report. J Fam Pratt 39:283-285,1994 5. Tizian C, Berger A, Vykoupil KF: Malignant degeneration in Madelung’s disease [benign lipomatosis of the neck): Case report. Br J Plast Surg 36:187-189,1993 6. Greiner GR, Klotz G, Conraux C, Dillenschneider E: Localization laryngee d’une adenolipomatose. J Fr ORL 17:561-563,1967 7. Moretti JA, Miller D: Laryngeal involvement in benign symmetric lipomatosis. Arch Otolaryngol 97:495496,1993 8. Roche G, Wahl D, Pottecher G, et al: Les localizations respiratoires de la lipomatose symmetric de LaunoisBensaude: A propos de trois cas. Le Poumon et le Coeur 34:411-415,197s 9. Enzi G, Biondetti PR, Fiori D, Mazzoleri F: Computed tomography of deep fat masses in multiple symmetric lipomatosis. Radiology 144:121-124,1982 10. Birnholz JC, Macmillian AS: Advanced laryngeal compression due to diffuse symmetric lipomatosis (Madelung’s disease). Br J Radio1 46:245-249,1973 11. Williams DW, Ginsberg LE, Moody DM, McCain BL: Madelung’s disease; MRI findings. Am J Neuroradiol 14:1070-1073,1993 12. Gritzmann N, Schratter M, Traxler M, Helmer M: Sonography and computed tomography in deep cervical lipomas and lipomatosis of the neck. J Ultrasound Med 7:451-456,198s