Madelung’s disease: A case report of laryngeal localization and a review of the literature

Madelung’s Disease (MD), also known as benign multiple symmetric lipomatosis or Launois-Bensaude syndrome, is a rare condition. The characteristic feature is the presence of numerous diffuse lipomas at the level of the neck or upper limbs. The etiology is unknown, but a close association with ethyl abuse has been observed. The use of radiological examinations such as ultrasound, computed tomography and magnetic resonance imaging is helpful for the differential diagnosis. There are two classifications mainly used: The Enzi classification and the Donhauser classification. In this article we present a case of MD with laryngeal localization. A 50-year-old man was referred to our department complaining of dysphonia for about 6 months. An abnormal and diffuse enlargement of the anterior part of the neck was noticed, compatible with “Madelung’s Collar”. Fiberoptic examination of the larynx revealed the presence of a lipomatous-like neoformation of the left false vocal cord. The patient underwent surgical removal of the laryngeal neoformation with transoral laser microsurgery. With this article we suggest a possible diagnostic and therapeutic procedure for the treatment of laryngeal lipomatosis. Keywords: Lipoma; TML; Larynx; Dysphonia; ENT

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Madelung's disease – a case report

Perspectives in Surgery ◽

10.33699/pis.2019.98.6.260-264 ◽

2019 ◽

Vol 98 (6) ◽

pp. 260-264

Keyword(s):

Case Report ◽

Clinical Findings ◽

Surgical Removal ◽

Resonance Imaging ◽

Laboratory Methods ◽

Madelung's Disease ◽

Madelung’S Disease ◽

Magnetic Resonance Imaging Mri ◽

Biopsy Examination ◽

Large Formation

Introduction: Madelung’s disease is a rare illness manifested by the uncontrollable proliferation of unencapsulated adipose tissue which accumulates symmetrically in the hypodermis in the area of the neck, shoulders, back and thighs. As part of differential diagnosis it is necessary to investigate tumours in the area of the neck, dysfunction of the thyroid gland, Cushing’s syndrome and rare lipomatosis. Case report: In the case report, the authors present the case of a 52-year-old male patient with a large formation on the neck, trunk and the scrotal area. A suspicion of liposarcoma was expressed based on the imaging examinations performed. Results: Excisions of tumorous loci on several occasions were indicated for the patient. Lipomas without abnormalities were proven in all histological examinations. Conclusion: The diagnosis of Madelung’s disease is based on the clinical findings, imaging (computed tomography − CT, magnetic resonance imaging − MRI) and biopsy examination. Laboratory methods tend to focus rather on associated internal diseases. Treatment is only symptomatic and consists in surgical removal of the foci. However, it is often associated with the risk of recurrence.

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Multiple Symmetric Lipomatosis: A Diagnostic Dilemma

Case Reports in Medicine ◽

10.1155/2013/836903 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 6

Author(s):

Mladen Mimica ◽

Danijel Pravdic ◽

Emina Nakas-Icindic ◽

Maja Karin ◽

Emil Babic ◽

...

Keyword(s):

Rare Condition ◽

Fat Distribution ◽

Diagnostic Dilemma ◽

Disease Etiology ◽

Neck Masses ◽

Multiple Symmetric Lipomatosis ◽

Madelung's Disease ◽

Madelung’S Disease ◽

High Incidence ◽

History Of

Introduction. Multiple symmetric lipomatosis, or Madelung's disease, is a rare condition which is characterized with large symmetrical accumulation of noncapsulated fat tissue in upper arms, neck, and shoulder areas. The disease etiology is unknown, with the highest incidence in the Mediterranean region.Case Presentation. Here, we present the case of Madelung's disease with symmetric fat distribution throughout the neck and history of alcoholism. The patient was treated from several diseases associated with alcoholism and hospitalized several times, but the diagnosis of Madelung's disease was omitted. The thyroid gland disease was excluded, while enlargement of the neck adipose tissue was attributed to obesity.Conclusions. This study points out possible diagnostic mistakes when a physician is not aware of a differentiation diagnosis of symmetrically enlarged neck masses, especially in geographic regions with high incidence of this disease.

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Partial cystectomy in young male for a urachal tumor masquerading a bladder leiomyoma

Archivio Italiano di Urologia e Andrologia ◽

10.4081/aiua.2019.1.51 ◽

2019 ◽

Vol 91 (1) ◽

pp. 51-52

Author(s):

Maurizio Sodo ◽

Lorenzo Spirito ◽

Roberto La Rocca ◽

Umberto Bracale ◽

Ciro Imbimbo

Keyword(s):

Clinical Symptoms ◽

Bladder Neoplasms ◽

Young Male ◽

Surgical Removal ◽

Resonance Imaging ◽

Ultrasound Computed Tomography ◽

Urinary Tract Symptoms ◽

Magnetic Resonance Imaging Mri ◽

Bladder Leiomyoma ◽

Lower Urinary

Leiomyoma of the bladder is a very rare disorder that accounts for 0.43% of all bladder neoplasms. Although the pathophysiology of the bladder leiomyoma is unknown, there are some theories on it. The patients can be asymptomatic; when present, clinical symptoms (lower urinary tract symptoms and\or hematuria), are associated with tumor size and location. For diagnosis, imaging plays an important role: ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI) are the examinations most frequently performed. Treatment consists of surgical removal of the tumor, and the prognosis is excellent.

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Multiple Symmetric Lipomatosis: A Review of 3 Cases

Case Reports in Otolaryngology ◽

10.1155/2012/910526 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 8

Author(s):

Emilio Mevio ◽

Michele Sbrocca ◽

Mauro Mullace ◽

Silvia Viglione ◽

Niccolò Mevio

Keyword(s):

Mediterranean Area ◽

Upper Body ◽

Cervical Region ◽

Unknown Etiology ◽

Surgical Removal ◽

Upper Aerodigestive Tract ◽

Madelung's Disease ◽

Madelung’S Disease ◽

History Of ◽

Symmetrical Lipomatosis

Multiple symmetrical lipomatosis, or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and in the upper body. The neoformations grow slowly and their initial consequence is purely esthetic. They can, however, lead to compression of the laryngotacheal area and of the esophagus. This disease usually affects middle-aged males from the Mediterranean area with a history of alcohol abuse. Although most cases have been sporadic, a few authors have indicated that the disorder may be hereditary. It is thought that this pathology originates from an alteration in lipid metabolism. Since the patients were asymptomatic temperance and diet was proposed, surgical removal of the lipomatose mass is the treatment of choice in case of complications due to fat mass compression on upper aerodigestive tract. The authors present three cases of Madelung's disease with different and particular manifestations.

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Massive intracranial immature teratoma: report of a case with polyhidramnios and intense pelvic pain

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x1996000200021 ◽

1996 ◽

Vol 54 (2) ◽

pp. 309-312 ◽

Cited By ~ 3

Author(s):

Fernando A. Soares ◽

Sandra Elisabeth Fischer ◽

Marlene Antonia Dos Reis ◽

Edson Garcia Soares

Keyword(s):

Pelvic Pain ◽

Healthy Woman ◽

Rare Condition ◽

Immature Teratoma ◽

Normal Brain ◽

Resonance Imaging ◽

Histologic Diagnosis ◽

Continuous Growth ◽

Ultrasound Computed Tomography ◽

Rapid Enlargement

A case of massive intracranial immature teratoma in a female stillborn is reported. She was the product of the second pregnancy of a 25-year-old healthy woman. The pregnancy was unremarkable until the 25th week of gestation when the mother noticed a rapid enlargement of her abdomen and intense pelvic pain. Because of the pain, a cesarean section was indicated, and a stillborn weighing 2750g with macrocephaly was delivered. The cranial contents weighed 1350g and showed a huge tumoral mass with only a rim of normal brain. A histologic diagnosis of immature teratoma was made. Massive intracranial teratomas are rare tumors and their occurrence in intrauterine life is even rarer. Their histogenesis is unknown, and there is no explanation for their continuous growth during embryogenesis. A prenatal diagnosis of this rare condition can be made by ultrasound, computed tomography, or magnetic resonance imaging.

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Benign notochordal cell tumor of the clivus with chordoma component: report of 2 cases

Journal of Neurosurgery ◽

10.3171/2019.6.jns19529 ◽

2020 ◽

Vol 133 (5) ◽

pp. 1355-1359

Author(s):

Maria Peris-Celda ◽

Laura Salgado-Lopez ◽

Carrie Y. Inwards ◽

Aditya Raghunathan ◽

Carrie M. Carr ◽

...

Keyword(s):

Histological Analysis ◽

Cell Tumor ◽

Surgical Removal ◽

Anatomical Site ◽

Adjuvant Radiation ◽

Resonance Imaging ◽

Adjuvant Radiation Therapy ◽

Notochordal Cell ◽

Transsphenoidal Resection

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.

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A non–alcohol-related case of Madelung's disease: Challenging patient with progressive jugular vein distension

Radiology Case Reports ◽

10.1016/j.radcr.2021.02.050 ◽

2021 ◽

Vol 16 (5) ◽

pp. 1183-1187

Author(s):

Cecilia Gozzo ◽

Federica Galioto ◽

Stefano Palmucci ◽

Salvatore Santo Signorelli ◽

Antonio Basile

Keyword(s):

Jugular Vein ◽

Related Case ◽

Madelung's Disease ◽

Madelung’S Disease

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Prenatal findings and postnatal follow-up of a midline dural sinus malformation

Acta Radiologica Open ◽

10.1177/20584601211006315 ◽

2021 ◽

Vol 10 (4) ◽

pp. 205846012110063

Author(s):

Hana Shabana ◽

Johannes Leidinger ◽

Johan Wikström ◽

Ove Axelsson

Keyword(s):

Magnetic Resonance Imaging ◽

Spontaneous Regression ◽

Rare Condition ◽

Present Knowledge ◽

Magnetic Resonance Imaging Examination ◽

Dural Sinus ◽

Resonance Imaging ◽

Prenatal Magnetic Resonance Imaging ◽

Dural Sinus Malformation

Dural sinus malformation is a rare condition. We describe a prenatally detected case followed by repeated ultrasound scans and a prenatal magnetic resonance imaging examination. A substantial spontaneous regression was observed, which is associated with a favorable outcome. We believe that our observations, including a long postnatal follow-up, will add to the present knowledge of prenatally detected cases, and thus improve management of the pregnancies as well as our possibilities to counsel the parents-to-be.

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