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Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 21e23

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Journal of Cranio-Maxillo-Facial Surgery

journal homepage: www.jcmfs.com

LaunoiseBensaude syndrome involving the orbits

Boris Laure a,b,*, Florent Sury a,b, Talel Tayeb a,b, Pierre Corre a,b, Dominique Goga a,b

aMaxillo-facial and Facial Plastic Surgery Department, Trousseau Hospital, 37044 Tours, FrancebUniversity of Medicine François Rabelais, 2bis Boulevard Tonnellé, 37000 Tours, France

a r t i c l e i n f o

Article history:Paper received 14 December 2009Accepted 7 April 2010

Keywords:ExophthalmosOrbital diseaseLaunoiseBensaude syndromeOrbit

* Corresponding author. Service de Chirurgie MaxFace, CHU Trousseau, 37044 Tours cedex, France. Tel.247 478 529.

E-mail address: laure@med.univ-tours.fr (B. Laure

1010-5182/$ e see front matter � 2010 European Assdoi:10.1016/j.jcms.2010.04.003

a b s t r a c t

Background: We report the first description of a LaunoiseBensaude syndrome involving the orbit.Case report: LaunoiseBensaude syndrome was diagnosed in a 70-year-old-man who presented withsevere bilateral proptosis (Hertel value 26 mm). We performed a bilateral transpalpebral orbitaldecompression by resection of intraorbital fat without bone removal. The surgery was uneventful. Thevolume of resected orbital fat was 16 cc in both sides. Proptosis reduction was 7 mm. PostoperativeHertel values were 19 mm in both eyes.Conclusion: The proptosis was managed successfully. Orbital lipectomy lead to minimal sequelae and,may be repeated if necessary in this case.

� 2010 European Association for Cranio-Maxillo-Facial Surgery.

1. Introduction

First described in 1848 as a benign symmetric lipomatosis, thisentity was then detailed by Launois and Bensaude in 1898. Lau-noiseBensaude syndrome has the same pathology as Madelung’sdisease. It is an uncommon disease with about 300 cases reportedin the medical literature. The disorder is characterised by multiple,symmetric and non-encapsulated lipomas affecting various areaspredominantly around the neck and the trunk. Its pathogenesisremains unclear, although several theories have been suggested,such as congenital or acquired defects of lipid metabolism in themitochondria (Vankoningsloo et al., 2005).

LaunoiseBensaude syndrome is more common in middle agedmales from the mediterranean area, usually with alcohol abuse orchronic liver diseases (Colella et al., 2005). The commonest sitesinclude the cervico-facial region, shoulder girdle, and arms (Parmarand Blackburn, 1996).

The aim of this case report was to describe the manifestation ofLaunoiseBensaude disease involving the orbits.

2. Case report

A 70-year-old-man with a history of LaunoiseBensaudesyndrome presented to us with a 2-year history of two majorsymptoms: daily headaches and episodes of eyeball luxation (twice

illo-faciale et Plastique de la: þ33 247 474 640; fax: þ33

).

ociation for Cranio-Maxillo-Facial

a month) which were self reduced by the patient. The patient’sbody mass index was 32.4 (weight: 83 kg and height: 1.6 m).Lipomatous deposits had been present in the head, the neck and theproximal part of the lower extremities for 30 years. He had previ-ously had excision of lipomatous masses on three occasions. Therewas a 50-year history of alcohol abuse.

Examination revealed severe bilateral and symmetric ocularproptosis (Hertel value: 26mm). There was no keratitis, excessivelacrimation, sandy sensation, or photophobia. There was a fattyhernia under the right conjunctiva and bilateral lipoptosis with vol-uminous pockets in the inferior and superior eyelids (Figs. 1 and 2).

Preoperative visual acuitywas 9/10Monoyer, Parinaud 2 right eyeand 10/10 Monoyer, Parinaud 2 left eye. Visual field and fundoscopicexamination were normal. Intraocular pressure measurements were12 mmHg right eye and 15 mmHg left eye. Extra-ocular musclefunction was subnormal. Evoked potentials were not performed.

Theorbital CT-scanassessed theproptosis asgrade III and revealedthe existence of diffuse and homogenous non-encapsulated adiposetissue in the intra and extraconal spaces of the orbit (Fig. 3). Proptosisis quantified by reference to bicanthal external line on the NeuroOcular Plan slice (Cabanis et al., 1981). In Grade III, the globe and partof the retrobulbar fat are in front of this line. The optic nerves wereelongated bilaterally but they had a normal diameter. The extra-ocular muscles were stretched and had a filiform aspect.

Biochemical investigations revealed the patient was euthyroid(TSH¼ 1, 1 mU/ml) and Graves’ orbitopathy was therefore excluded.

We chose to perform single stage bilateral orbital decompres-sion with intra and extraconal fat removal as described by Olivari(1988). We used a transpalpebral approach for the inferior eyelidsand for the upper eyelids we performed a blepharoplasty approach

Surgery.

Fig. 1. Preoperative view of the patient showing the bilateral proptosis and lipoptosisof inferior and superior eyelids.

Fig. 2. Preoperative view of the right orbit showing the significant proptosis and thefatty hernia under the conjunctiva.

Fig. 3. Preoperative CT-scan showing grade III bilateral proptosis due to increase of fatin intra and extraconal spaces of the orbits.

Fig. 4. Preoperative blepharoplasty approach on upper lids showing the significantquantity of fat.

B. Laure et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 21e2322

with removal of surplus skin (Fig. 4). Sixteen cc of fat were excisedfrom both sides. Surgery was uneventful.

There were no complications following the surgical proceduresuch as diplopia, optic nerve damage or alteration of the visual field.Macroscopically, the fat excised had the same appearance of normalorbital adipose tissue. Histologically the tissue had the same char-acteristics of a classic lipomatosis.

Twelve days after the surgery, once the oedema had resolved,bilateral inferior eyelid ectropions appeared. They were thesequelae of the lid distension due to proptosis. To avoid compli-cations (conjunctival irritation and inferior keratitis) successfulsurgical correction was undertaken. After 1 month with the Kuhnt-

Szymanowski procedure which involved excision of a tarso-conjunctival triangle and a skin triangle in the external canthus.

Nine months postoperatively, eye projection was symmetricaland measured 19 mm. The ectropion correction remained stable,ophthalmological and orthoptic exams were normal.

The patient was satisfied by the outcome and he noticed thatheadaches improved after surgery (Fig. 5).

3. Discussion

We describe on the first case of LaunoiseBensaude syndromeinvolving the orbits and that was characterised by episodes ofbilateral proptosis with recurrent luxation of the eyeballs.

In our case, the differentiation between dysthroid eye diseaseand orbital LaunoiseBensaude syndrome was not difficult becausetherewere others lipomatous deposits located in the head, the neckand the proximal part of the lower extremities. Nevertheless thisdiagnosis must be a diagnosis of exclusion after dysthroid eyedisease has been excluded.

Fig. 5. Postoperative view at 9 months showing the reduction of proptosis and thebetter look of the patient.

B. Laure et al. / Journal of Cranio-Maxillo-Facial Surgery 39 (2011) 21e23 23

Rare localisations of LaunoiseBensaude disease have beenreported including the tongue (Lopez-Ceres et al., 2006), themediastinum, the larynx (Borges et al., 1997) and the scrotum(Poggi et al., 2006). Currently there is no way of preventing thelesions and. surgical removal of the lipomatous lesions by resectionand/or liposuction (depending on location) remains themainstay oftreatment.

In this case, because the proptosis was of a lipomatous origin,we opted for lipectomy rather than orbital expansion, which wasconsidered to be the treatment of choice had the lipectomy beenunsuccessful. Liposuction is contraindicated in the orbits.

Although the presentation of the proptosis in this case wassimilar to a Graves’ orbitopathy, the volume of fat excised here washigher than the average 6 ml of resected orbital fat in Graves’orbitopathy (Olivari,1988, Richter et al., 2007). In our case proptosisreductionwas 7 mm for both eyeballs. This suggests that 1 ml of fatremoved results in 0.5 mm of improvement in proptosis, unlike theobservations of Adenis (Adenis et al., 1998) who report 1 mm of

improvement every 1 ml removed. This may be explained by theexistence of major fat hernias and preseptal filling in the superiorlid. Both orbits were managed in the same operation (Wu et al.,2008).

4. Conclusion

The course of LaunoiseBensaude syndrome is unpredictable andfrequent recurrences of excised lipomas are seen. We managed thisproptosis successfully with orbital lipectomy with no recurrenceafter 9 months of follow-up. This technique lead had no compli-cations and may be repeated if necessary in the case of relapse.

Patient confidentiality

The Patient signed a permission form to publish his photographs.

Source of support

No grant.

Conflict of interestThe authors have no conflict of interest.

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