A case of obstructive sleep apnea syndrome: unusualcomorbidityE. Jalladeau1, B. Crestani2, J.-P. Laissy3 and M. F. Vecchierini1

1 Laboratoire du Sommeil, Groupe Hospitalier Bichat Claude Bernard, AP-HP, Paris, France2 Service de Pneumologie, Groupe Hospitalier Bichat Claude Bernard, AP-HP, Paris, France3 Service de Radiologie, Groupe Hospitalier Bichat Claude Bernard, AP-HP, Paris, France

AbstractIntroduction: Launois-Bensaude syndrome (LBS) is a rare disease, characterized bythe accumulation of fatty tissue predominantly in the neck, shoulders and thorax,whose diagnostic is clinical.Objectives: We describe a new case in a 73 year-old man. As the patient was obese,complained of snoring and was treated for hypertension, we looked for a morbidassociation with an obstructive sleep apnea syndrome (OSAS).Methods: A polysomnography (PSG) and a cervical magnetic resonance imaging(MRI) were performed.Results: PSG demonstrated OSAS with an apnea-hypopnea index of 43/h. CervicalMRI showed fatty infiltration resulting in airway narrowing at the pharyngeal butnot at the tracheal level. Only, 3 cases of such a morbid association have alreadybeen published; in 2 of these patients was a tracheal compression. More over ametabolic syndrome was present.Conclusions: This observation draws attention to the need for seeking OSASamong patients affected by LBS, even in a moderate form, and emphasises the rolesof the upper airway narrowing by the fat infiltration as well as the role of themetabolic syndrome in the genesis of OSAS.

Please cite this paper as: Jalladeau E, Crestani B, Laissy J-P and Vecchierini MF. Acase of obstructive sleep apnea syndrome: unusual comorbidity. The Clinical Res-piratory Journal 2008; 2: 239–241.

Case history

A 73-year-old man complained of snoring, chronicexcessive daytime sleepiness (EDS) with an Epworthscore of 17. He had hypertension. He abused alcohol,did not smoke and did not take any psychotropic treat-ment. He became gradually obese [body mass index of31 kg/m2] since he was 20 years old. The patient’s neckcircumference was 58 cm.

What are the diagnoses?

Figure 1 shows fat masses, localized at the neck, shoul-ders and higher part of the thorax, the typical distri-bution of lipomata in Launois–Bensaude syndrome(LBS) or Madelung’s disease.

Snoring, obesity and EDS are symptoms of obstruc-tive sleep apnea syndrome (OSAS). The diagnosis wasconfirmed by a polysomnography showing a respira-tory event’s index of 43 per sleep hour (27 obstructiveapneas, 16 hypopneas) associated to micro arousals(42/h). SaO2 under 90% lasted 3% of total sleep dura-tion and snoring 77%.

The pulmonary function testing highlighted only arestrictive syndrome (total lung capacity 70% of thetheoretical value). Blood gases were normal.

Is there a link between OSAS and LBS?

A cervical magnetic resonance imaging was per-formed, the patient being awake in a supine position. Itshowed an important fatty infiltration, mainly behind

Key wordsBMI – Launois–Bensaude syndrome –lipomatosis – metabolic syndrome – sleepdisorder breathing

CorrespondenceMarie Francoise Vecchierini, MD, GroupeHospitalier Bichat, Paris, France.Tel: +33 140 258 401Fax: +33 140 258 800email: marie-francoise.vecchierini@bch.aphp.fr

Received: 09 April 2008Revision requested: 14 April 2008Accepted: 16 May 2008

DOI:10.1111/j.1752-699X.2008.00071.x

The Clinical Respiratory Journal CASE REPORT

239The Clinical Respiratory Journal (2008) • ISSN 1752-6981© 2008 The Authors. Journal compilation © 2008 Blackwell Publishing Ltd

the pharynx, associated with a thickening of the pha-ryngeal wall, reducing the pharyngeal airway caliberpredominantly at the retroglossal level (Fig. 2). Theanteroposterior (11.5 mm) and lateral (13.2 mm)diameters were reduced with a trifoliate aspect ofpharyngeal airway in axial imaging (Fig. 3). The cross-sectional area of the pharynx was 117 mm2, consid-ered as very small even if a great variability of thecaliber of the pharynx has been reported in OSASpatients as well as in healthy subjects (1). The com-puted tomography did not show any respiratory tracknarrowing at the level of the thorax.

A metabolic syndrome according to NCEP-ATP III(2) was present. The biological follow-up showed onseveral occasions hypertriglyceridemia, fasting hyper-glycemias up to 1.53 g/L. However, glycated hemo-globin, total and low density lipoprotein (LDL)cholesterol remained normal.

Discussion

LBS is an uncommon excessive development of brownfat tissue whose etiology and prevalence are still

Figure 1. Typical Launois–Bensaude syndrome appearance inour patient.

Figure 2. Cervical magnetic resonance imaging; sagittal T2 –weighted image shows fat infiltration (light gray) behind thepharynx, the thickness of posterior soft tissues of the pharynx atthe level of the lower jaw was 14.1 mm (white dash 1).

Figure 3. Cervical magnetic resonance imaging; axial T1 –weighted image at the level of the lower jaw shows transversestenosis of the oropharynx lumen, with trifoliate patternof pharyngeal airway. Lateral diameter was 13.2 mm (whitedash 1).

Obstructive sleep apnea syndrome Jalladeau et al.

240 The Clinical Respiratory Journal (2008) • ISSN 1752-6981© 2008 The Authors. Journal compilation © 2008 Blackwell Publishing Ltd

unknown. The diagnosis is clinical. Our subject pre-sents the usual ground of this affection: male gender,ripe age, history of obesity and alcohol abuse. Fat infil-tration in the mediastinum is often associated. Onlythree cases associating severe OSAS and LBS have beenpreviously reported (3, 4) with fat infiltration aroundupper airway. In two of the three patients, there was atrachea compression. In our patient, the narrowingwas not at the tracheal level but at the pharyngeal level.It could be a mechanical factor responsible for OSAS.The narrowing is not only explained by the posteriorfatty infiltration, but also by a thickening of the mus-cular pharyngeal wall and a reduction of the lateraldiameter of pharynx. These findings have beenreported in some patients with OSAS without LBS (5).A metabolic syndrome is frequent in OSAS (6) as wellas in LBS, and could be another link between these twosyndromes. Treatment of OSAS in LBS seems similar toOSAS without LBS. A patient with trachea compres-sion was treated successfully by continuous positiveairway pressure (CPAP) (4). At the moment, no effec-tive medication against lipomatosis is known. So, atreatment by CPAP was initiated and dietetic ruleswere emphasized in our patient.

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Jalladeau et al. Obstructive sleep apnea syndrome

241The Clinical Respiratory Journal (2008) • ISSN 1752-6981© 2008 The Authors. Journal compilation © 2008 Blackwell Publishing Ltd