An Unusual Cause of Carotid Sinus Syndrome: Multiple Symmetric Lipomatosis MASSIMO ROMANO,* ACHILLE SPINELLI,* SANDRO FELLER,** VITO TIBY,** SERGIO SBRASCINI,** and LUCIANO MUZIO* From the *Department of Cardiology and the **Department of Neurology, Ospedale Civile, Carbagnate Milanese. Italy ROMANO, M., ETAL.: An Unusual Cause of Carotid Sinus Syndrome: Multiple Symmetric Lipomatosis.

A 45-year-o!d man wiih multipie symmetric iipomalosis suffered recurrent syncope attributed lo cavoWd sinus syndTome caused by extrinsic compression of the carotid body by [he Jipomutous masses. SurgicaJ removal reduced bul did not stop syncope, which was then controlled by implantation oj Q DDD pacemaker. {PACE, Vol. 15, February 1992} muJtipJe symmetric iipomatosis, carotid sinus syndrome, DDD pacemaker

Introduction Multiple symmetric lipomatosis is a rare disease (about 200 cases have been reported), first described by Launois and Bensaude' in 1898 transmitted by a dominant autosomal gene, and mainly involving the adult male between 20 and 50 years of age.^^ It is the most common of the lipomatoses and is characterized by a typical distribution within the neck, the nape of the neck, the shoulders, supraclavicular and deltoid regions, and with progression, the abdomen and the groin. Clinically, deep locations are most important, especially in the mediastinum where compression of the mediastinal structures can occur.^'^ Carotid sinus syndrome caused by lipomatous compression of the carotid body has not previously been reported. This report is of a male with lipomatosis and associated carotid sinus syndrome who required cardiac pacing because of recurrent syncope. Case Report A 45-year-old male without a family history of neurological disease and who consumed '% of a

Address for reprints; Dr. Massimo Romano, via Ausonio 6, 20123, Milano, Italy. Received January 16, 1991; revision September 2. 1991; revision October 24. 1991; accepted October 25. 1991.

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liter of wine daily had undergone surgical removal of bulky bilateral subcutaneous lipomas from the carotid area at the age of 33 years. Thereafter he underwent repeated removal of local recurrences, which eventually spread to the entire neck. In 1983 and again in 1988 he complained of an episode of severe fatigue and di2:ziness that was then ascribed to vertebrobasilar insufficiency. He was hospitalized for the first episode of syncope, accompanied by head trauma, in the spring of 1990. On physical examination he was in good general condition, with bilateral muscular wasting of the scapular girdle, especially of the pectoral and deltoid muscles and lesser wasting of the pelvic girdle. Moderate weakness was present with reduction of the deep tendon reflexes. EEG and Doppler ultrasonography of the carotid and vertebrobasilar vessels, and visual and acoustic evoked potentials were all normal. Cerehral and lateral cervical MRI excluded an expansive intracranial process and other neurological disease and confirmed the presence of lateral cervical lipomatous tissue (Fig. 1). The baseline ECG was normal while carotid sinus massage caused more than 3 seconds of asystole and lipothymia (Fig. 2). Fortyeight hour Holter monitoring, head-up tilt test, and tests for autonomic function including deep breathing, Valsalva maneuver, and tests for orthostatic hypotension, i.e., lying to standing, were all normal. The diagnosis of carotid sinus syndrome

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Figure 1. A magnetic resonance imaging scan showing the large lipomas in the lateral aspect of the neck and fheir proximity to the carotid bifurcation.

was made and the patient underwent surgical removal of the lipoma. No pacemaker was implanted as it was assumed that the removal of the lipomatous mass would stop the syncope. Postoperatively, the bradyarrhythmic response to carotid sinus massage decreased, with neither prolonged asystole nor symptoms related to vagal stimulation. But. within the next 3 months the lipoma partially recurred accompanied by two syncopal episodes. The patient was rehospitalized and a cardiac electrophysiological study was performed, the results of which were entirely normal. No ventricular or supraventricular tachycardias could be induced. A diagnosis of the mixed type of carotid sinus syndrome was made as carotid sinus

massage, during atrioventricular sequential cardiac stimulation, at a rate of 70 beats/min, produced a 40 mmHg reduction in systolic arterial pressure. The patient then underwent implantation of a DDD pacemaker and has been asymptomatic during the succeeding 6 months.

Discussion Multiple systemic lipomatosis is a syndrome characterized by the development of noncapsulated lipomatous masses that begin in the subcutaneous fatty tissue and eventually extend deeply to the muscular sheaths and to spaces between organs. The most typical locations are the neck.

.fr.

Figure 2. The BCG during manuai carotid sinus massage demonstrated sinus arrest and asystole for 4.8 seconds.

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shoulders, supraclavicular, and deltoid regions with later involvement of the abdomen and groin. As organs may be compressed, macrocytic anemia, chronic liver disease, peripheral neuropathy, and metabolic abnormalities eventually appear.^ The most important radiologically detectable location is the mediastinum** in which the pathological findings are caused by compression of the trachea and neurovascular structures.^"^ Signs of autonomic neuropathy often occur, probably metabolically determined and histologically similar to diabetic neuropathy. ^-^ Cardiovascular involvement is usually related to autonomic nervous system disturbance and a tendency to tachycardia at rest. Only a single unexpected, fatal cardiac arrest unrelated to acute myocardial infarction has been described.^ There are no reports of carotid sinus syndrome caused by compression of the carotid body, though the growth of bulky lipomatous masses in the lateral region of the neck is common. It was reasonable to exclude the independent association of the two conditions, as the volume of the lipomatous mass was large and caused severe symptomatic bradycardia with only moderate ca-

rotid sinus massage. The removal of the fatty tissue resulted in brief improvement of the clinical response to vagal stimuli witb return when the fatty mass recurred. Carotid sinus syndrome returned when the recurrent fatty mass once again compressed the carotid body. The absence of autonomic neuropathy and atrial and ventricular tacbyarrhythmias, documented by the negative tests, Holter monitoring, and programmed atrial and ventricular stimulation confirmed the vagus mediated bradyarrhythmic genesis of the presyncopal and syncopal episodes. Autonomic nervous system dysfunction was excluded in this patient though it is reported to be present in a significant proportion of patients with multiple symmetric lipomatosis^ and may then cause the repeated syncopal episodes.^ The normal autonomic evaluation, normal sleeping heart rate, and the negative results of head-up tilt test^" allowed the exclusion of autonomic pathology. In the presence of bulky lipomatous masses in the lateral portion of the neck, even minor disturbances of consciousness should suggest the consideration of carotid sinus syndrome.

References 1. Launois PE, Bensaude R. De radenolipomatose symetrique. Bull Mem Soc Hop 1898; 1:298-318. 2. Enzi G. Digito M, Bellina L, et al. Le lipomatosi. Alimentazione, Nutrizione, Metabolismo 1983; 4:245-251. 3. Enzi G. Multiple symmetric lipomatosis; An update clinical report. Medicine 1984; 63; 56-64. 4. Enzi G. Biondetti PR, Fiore D, et al. Gomputed tomography of deep fat masses in multiple symmetric lipomatosis. Radiology 1982; 144:121-124. 5. Ghahrokhi K. La lipomatose symetrique diffuse a predominance cervicale. Rev Laryngol Otol Rhinol 1960; 81:551-568.

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6. Gomings DE, Glenchur H. Benign symmetric lipomatosis. J Am Med Assoc 1968; 203:305-312. 7. Mounier-Kuhn P, Hagenauer JP. Localization laringee d'une maladie de Launois-Bensaude. J Franc Otorhinol 1967; 16:603-607. 8. Fessel WJ. Fat disorders and peripheral neuropathy. Brain 1971; 94:531-540. 9. Sobel B, Roberts R. Hypotension and syncope. In E Braunwald [ed.): Heart Disease. A Textbook of Cardivovascular Medicine. 3rd Ed. Philadelphia, PA, W.B. Saunders Go.. 1988. pp. 884-895. 10. Kenny RA, Ingram A. Bayliss ). et al. Head-up tilt test: A useful test for investigating unexplained syncope. Lancet 1986; 1:1352-1355.

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An unusual cause of carotid sinus syndrome: multiple symmetric lipomatosis.

A 45-year-old man with multiple symmetric lipomatosis suffered recurrent syncope attributed to carotid sinus syndrome caused by extrinsic compression ...
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