Launois–Bensaude syndrome involving the orbits

Laure, B.; Sury, F.; Tayeb, Talel; Corre, Pierre; Goga, D.

doi:10.1016/j.jcms.2010.04.003

Cited by 11 publications

(7 citation statements)

References 9 publications

(9 reference statements)

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“…Clinical types of MSL [37,43] Type I, head and/or neck with extension down the back, or only on the back: In rare cases, MSL SAT can invade the lingual muscles of the tongue [44,45] , or the vocal cords and compress the recurrent laryngeal nerve causing hoarseness [46] , or increase periorbital fat [47] . Tracheal or esophageal compression and the superior vena cava syndrome can be found in 15%-20% of patients [48] .…”

Section: Diagnosismentioning

confidence: 99%

Rare adipose disorders (RADs) masquerading as obesity

2012

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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidencebased data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.

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Section: Diagnosismentioning

confidence: 99%

Rare adipose disorders (RADs) masquerading as obesity

2012

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“…It is noteworthy that medico-legal autopsies in adults with a positive record of heavy alcohol consumption give histological evidence of the presence of multi-locular adipose cells resembling brown adipose tissue around the thoracic aorta and the carotids. Unusual locations of lipomatous tissue in MSL have been reported in the tongue (12,13), in the scrotum (14) and in the retro-orbital space (15).…”

Section: Clinical Aspectsmentioning

confidence: 99%

Multiple symmetric lipomatosis: A rare disease and its possible links to brown adipose tissue

Enzi

Busetto

Sergi

et al. 2015

Nutrition, Metabolism and Cardiovascular Diseases

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“…Two prior case reports with orbital involvement were found in the literature (Table 1 ). The first case was reported in 2011 by Laure et al [ 14 ] He described a 70-year-old Caucasian male patient with bilateral symmetric proptosis and recurrent episodes of luxation of the eyeball for 2 years. The patient was diagnosed with Launoise–Bensaude syndrome and had a 50-year history of alcohol abuse.…”

Section: Discussionmentioning

confidence: 99%

“…A subsequent bilateral Kuhnt–Szymanowski procedure was also carried out 12 days after surgery to correct a secondary bilateral lower lid ectropion. [ 14 ]…”

Section: Discussionmentioning

confidence: 99%

“…A similar concept of fat removal surgery was introduced in 1988 by Olivari [ 17 ] to treat orbital fat hypertrophy caused by Grave's ophthalmopathy (GO). Olivari's technique was utilized in Laure's case [ 14 ] to treat the lipomatous exophthalmos. The procedure achieved a successful surgical result, with a total of 7 mm reduction of proptosis of both eyes.…”

Section: Discussionmentioning

confidence: 99%

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Application of lateral tarsoconjunctival flap to correct ocular complications of Madelung's disease

et al. 2018

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Rationale:Madelung's disease is a specific type of benign symmetric lipomatosis, which is characterized by overgrowing fat distributed at the neck and shoulder. This excessive fat accumulation may occur in different regions, including vital organs, such as the larynx, trachea, or the orbits. Surgery is usually performed to correct the esthetic or functional concern of the affected area. There are only few case reports mentioned about the orbital involvement of this disease. This study aimed to describe a case of Madelung's disease with ocular complications and the successful treatment using tarsoconjunctival flap.Patient concerns:A 90-year-old obese male presented with decreased visual acuity accompanied with severe band form punctate epithelial erosions and ciliary injections in both eyes. Exophthalmos and ectropion of the lower eyelids were observed.Diagnoses:Orbital computed tomography revealed a rare entity of Madelung's disease with significant massive deposits of lipomas located symmetrically around the orbit and neck region.Interventions:Bilateral lateral tarsoconjunctival flaps with lateral tarsal strip procedures were performed to correct the lower eyelid ectropion-related exposure keratopathy.Outcomes:The patient obtained improved visual acuity, intact and clear corneal surfaces, and perfect lower eyelid positions over both eyes.Lessons:Madelung's disease is a rare condition. There are few reports to describe its ocular complications, due to lipoma overgrowing in the orbit. We shared our successful experience to correct ocular complications using tarsoconjunctival flap technique.

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Launois–Bensaude syndrome involving the orbits

Cited by 11 publications

References 9 publications

Rare adipose disorders (RADs) masquerading as obesity

Rare adipose disorders (RADs) masquerading as obesity

Multiple symmetric lipomatosis: A rare disease and its possible links to brown adipose tissue

Application of lateral tarsoconjunctival flap to correct ocular complications of Madelung's disease

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