Qiuyuan FENG ,Xueyao CAI ,Yucheng CAI ,Guoyou ZHANG ,Jie LIAN ,Lian ZHU,*

ABSTRACT Multiple symmetric lipomas are a rare disease characterized by multiple symmetrical deposits of adipose tissue associated with alcohol abuse.A 68-year-old man was admitted to our hospital for being operated on for a mass growth on the neck and back.Based on the patient’s medical history and preoperative magnetic resonance imaging findings,we performed surgical resection.There was no recurrence at one year postoperatively.

KEY WORDS Multiple symmetric lipomas;Symmetric adipose tissue;Alcohol abuse;Surgical resection

INTRODUCTION

Multiple symmetric lipomas (MSL),also known as Madelung’s disease (MD),benign symmetric lipomatosis,and Launois-Bensaude syndrome[1-5],is a rare disease characterized by the peculiar appearance of an excessive,progressive,and symmetrical accumulation of adipose tissue in the subcutaneous layer.Here we present a recent case of MSL.

CASE PRESENTATION

A 68-year-old man visited the clinic with a 9-year history of a mass growing on the neck and back that had grown rapidly for the past 1 year.According to his medical history,he had undergone a surgical resection in another hospital for a neck tumor 9 years prior,and part of the tumor remained after the surgery with unknown postoperative pathology.This tumor in the neck and back had grown significantly in the past year.At presentation,his neck tilting and rotation function were slightly limited without dyspnea,dysphagia,or upper-extremity numbness.He had a 3-year history of hypertension with irregular medication,a 50-year drinking history (more than 500 g per day),and a 45-year smoking history (20 cigarettes per day).He had quit smoking and drinking for 1 year before this hospital admission.

The patient was conscious with a blood pressure of 158/93 mmHg during the physical examination.The mass was mainly distributed across the patient’s neck,upper arm,upper back,and chest.It was soft and bilaterally symmetrical with poor mobility and unclear borders.No redness,swelling,or ulceration was seen on the local skin,but an operative scar was seen in the middle of the back of the neck (Fig.1),and his neck tilting and rotation abilities were restricted.

Fig.1 Patient’s appearance at presentation

Laboratory tests on admission showed a reduced leukocyte count (2.0 × 109/L;reference:3.5-9.5 × 109/ L),platelet count (68 × 109/L;reference:100-300 × 109/L),and serum albumin level (33 g/L;reference:35-55 g/ L).The monocyte count was 10.7% (reference:3%-8%).Neck magnetic resonance imaging (MRI) confirmed that the soft tissue in the back of the neck was significantly thickened,presenting high signals on T1WI and T2WI and low signals on a STIR sequence (Fig.2).Computed tomography (CT) suggested excessive fat deposition with fibrous septa and no marked pseudocapsule (Fig.3).

Fig.2 Magnetic resonance images of the patient’s neck

Fig.3 CT scan images

The diagnosis of type I MSL was confirmed.A preoperative examination suggested that the lipoma had grown symmetrically,penetrating the sternocleidomastoid muscle,trapezius muscle,and cervical triangle regions.It has no capsule,clear boundary,or regular shape.Laboratory tests revealed low leukocyte and platelet counts.In this case,careful preoperative preparation was performed,including increasing the leukocyte count,lowering blood pressure,and preparing blood.The surgical plan prioritized the removal of the posterior neck lipoma to modify the local malformation and dysfunction.Any injury to important vessels and nerves in the neck would be avoided.The patient was placed in the prone position during routine intubation.We cut along the original incision at the back of the neck and made an accessorial incision on both sides to create a T-shaped incision to reveal the tumor completely.During the operation,bleeding was obvious due to the large tumor,so we staunched blood thoroughly and placed a drainage tube.The wounds were dressed under pressure after surgery.The mass cut was approximately 2 kg with dense tissue and small particles (Fig.4).The significant fluid draining postoperatively could easily lead to a hematoma.Therefore,some sutures were removed later to increase the drainage.The removal of stitches at 12 days postoperatively showed that most of the incisions had healed well,but a dressing change was still needed.Thus,we instructed the patient to continue pressure bandaging and regularly change the dressing (Fig.5).

Fig.4 Intraoperative views of the MSL

Fig.5 Postoperative views

Fig.6 One-year postoperative views

DISCUSSION

MD,also known as MSL and benign symmetric lipomatosis,is a rare disease of fat metabolism.The disease was first discovered by Brodie[4]in 1846.In 1888,Madelung[5]conducted a detailed analysis and summary of 33 cases,so it was first named Madelung’s disease.It is characterized by the growth of multiple symmetrical fat masses without a capsule in the maxillofacial region,neck,shoulders,trunk,extremities,and other parts.In most patients,neck involvement usually causes a specific symptom called“horse neck”[6].

According to the literature[7,15],the disease is mainly prevalent among middle-aged men with a long-term history of alcohol abuse.It is more common in men living in the Mediterranean or Eastern European areas.The male-to-female ratio is 15:1.Asian patients are rare.However,cases of non-drinking patients[3],women,and even children[8]have been reported.

Clinically,the disease can be classified into several types according to the location of fat accumulation,and Donhauser’s classification[9]made in 1991 is one of the most widely accepted.The four types are summarized in total.Type Ⅰ mainly includes the neck,upper back,shoulders,and upper limbs.Type Ⅱ covers the shoulders,deltoid region,upper limbs,and chest.Type Ⅲ includes the thigh and medial knee.Type Ⅳ mainly includes the abdomen.Regardless of how it is divided,fat rarely accumulates in the throat,mediastinum,and distal limbs[14].

The etiology and pathogenesis of MD remain unclear.Factors related to its occurrence and development may include enzyme deficiency,fat deposition induced by catecholamines,sympathetic denervation,and brown fat cell hypertrophy caused by mitochondrial DNA mutations.Some scholars have recently reported that the type and distribution of fat cells in MD patients are similar to those in the brown adipose tissue of infants[10-12].Among patients with MD,this is not due to the increasing volume of existing adipocytes,but rather the appearance of new adipocytes[13].Other scholars[1,14]have claimed that the disorder of human brown adipose tissue proliferation and differentiation may be the basis of the disease.Alcoholism is an important contributing factor.Approximately 60% to 90% of patients are alcoholics.Alcohol can reduce the number and activity of β-adrenergic receptors,thereby promoting fat synthesis and reducing lipolysis.Alcohol can also directly affect mitochondrial activity,leading to premature oxidation of mitochondrial DNA or mutation of mitochondrial DNA (A8344G)[16],resulting in fat accumulation in different parts of the body.

Patients with MD often have other metabolic-related diseases,such as liver dysfunction,hypercholesterolemia,hyperuricemia,hypothyroidism,hypertension,and impaired glucose tolerance,which tends to progress to diabetes[2,13].Except for the characteristic appearance,MD patients may also suffer from the mediastinal syndrome,dysphagia,dyspnea,sleep apnea syndrome[17],and restricted neck movement due to the large fat accumulation.Approximately 85% of MD patients may also have sensory,motor,and autonomic polyneuropathy[18,19].

MD is diagnosed based on medical history,physical signs,pathology,and imaging examinations[1,20].Among them,imaging examinations,including CT,MRI,and ultrasound,can determine the main location and infiltrative range of the tumor as well as the relationship with surrounding important blood vessels and nerves.MD should also be distinguished from morbid obesity,Cushing’s syndrome,lymphoma,liposarcoma,lipoedema,angiolipoma,and neurof ibroma[21-22].

The treatment of MD mainly includes surgical and nonsurgical treatments.Surgical treatment,primarily resection and liposuction,is currently the most commonly used and effective treatment.The principle of surgical treatment should be based on restoring function and improving appearance instead of complete tumor excision.Compared with liposuction,surgical resection can provide a wider surgical field in which as much abnormal fat tissue can be removed as possible and side injuries can be reduced.However,as the postoperative bleeding time is long and the amount of bleeding is increased,it is also likely to cause local large-scale ecchymosis and hematoma.Therefore,more attention should be paid to hemostasia during the operation,postoperative compression of hemostasis,and placement of a negative pressure drainage tube.Taking this patient as an example,due to a large amount of drainage fluid,some sutures were removed later.Because his postoperative recovery was good and the physiological curvature of his neck recovered,the patient’s satisfaction was high in this case.Liposuction is relatively less injurious,simpler to operate,and can achieve better cosmetic results[23].However,because the diseased adipose tissue is denser and the presence of fibrous septa may cause suction difficulties,liposuction usually requires more experienced operators.Both surgical resection and liposuction are susceptible to recurrence.Thus,as reported here,some scholars believe that surgical resection combined with liposuction can reduce recurrence[24].

Non-surgical treatments include intra-fat injections,alcohol cessation,and lifestyle adjustments.Intra-fat injection refers to the injection of one or several drugs into the subcutaneous fat layer to achieve lipolysis.These drugs mainly include phosphatidylcholine,multivitamins,pentoxifylline,aminophylline,hyaluronic acid,and collagenase.Some researchers have reported that this method may limit tumor growth but cannot effectively reduce tumor volume.Moreover,fibrosis caused by lipolysis increases surgical resection or liposuction difficulty[25].Alcohol cessation can slow the rate of swelling and reduce postoperative recurrence,but it cannot reverse the disease course.Controlling blood glucose and blood lipids may also slow down swelling.However,diet control has no effect.Concerning non-surgical treatments,oral β-receptor agonists,and injections of anticoagulant drugs in local lesions[26]could also be effective,but this remains to be confirmed in the future.

There are also many other treatments,such as Bassetto[27],using ultrasound-assisted liposuction to prevent damage to the surrounding neurovascular structures.This technology provides gentle,precise liposuction with less damage to the subcutaneous tissues and vascular structures,but its cosmetic effect is not ideal.

In conclusion,MD is a rare disease.Due to its painless course,the masses of most patients are huge at discovery.Currently,the most effective treatment for MD is surgical resection and liposuction,each of which has its own advantages and disadvantages.Therefore,the patient’s condition must be accurately evaluated before each surgery.The surgical plan should depend on tumor size and depth,patient expectations,and operator experience.Future research will focus on the pathogenesis of MD to guide its clinical prevention and treatment.

ACKNOWLEDGMENTS

We appreciate the patient,who provided informed consent for the publication of his case.

ETHICS DECLARATIONS

Ethics Approval and Consent to Participate

The need for ethics approval was waived as it was a case report.The participant provided written informed consent prior to enrollment in the study.

Consent for Publication

All the authors have consented to the publication of this article.

Competing Interests

The authors declare that they have no competing interests.The authors state that the views expressed in the article are their own and not the official position of the institution or funder.