Laryngeal involvement in Madelung's disease with acute airway compromise
Journal
Kaohsiung Journal of Medical Sciences
Journal Volume
28
Journal Issue
8
Pages
462-463
Date Issued
2012
Author(s)
Abstract
Madelung's disease is a metabolic disorder characterized by multiple fatty accumulations in the neck and upper trunk. After first described by Brodie in 1846, nearly 200 cases have been reported. Most cases were middle-aged European white men with chronic alcoholism. Diagnosis is based on clinical history and physical examination [1]. Madelung's disease is very rare in Eastern populations. Although most cases were asymptomatic, laryngeal involvement of the disease can results in a life-threatening airway problem and warrant immediate surgical intervention [2]. We present a rare oriental patient with Madelung's disease who had laryngeal involvement resulting in airway compromise. We will also discuss disease management strategies. A man 50 years of age with typical appearances of Madelung's disease came to our emergency department and presented with severe dyspnea and stridor. Laryngoscopy showed swollen false cords, marked lumen compromise, and bilateral limited vocal fold movement. A computed tomography (CT) scan revealed fatty attenuation involving the glottic larynx and neck spaces. After general anesthesia with endotracheal intubated at the operating room, an extended apron-flap incision was made over the anterior neck. Bilateral neck levels III, IV, V, and VI lipomas were resected. Operation was finished following a tracheotomy. Pathology revealed benign adipose tissue. He was discharged with a tracheostomy under stable course. Further surgical removal of the laryngeal lipoma was suggested but he refused because of his stable airway condition. His condition remains stationary after 10 months follow-up. Reports about surgeries in patients with Madelung's disease with laryngeal involvement are very rare. Review of the English literature revealed only four other cases reported (Table 1) [2]-[5]. All patients were men between the ages of 50 and 71 and laryngeal involvements included supraglottis in one patient, glottis in two, and transglottic in one. CT imaging was performed in two. Follow-up periods ranged from 6 months to 2.4 years. Otolaryngologists may have difficulty performing a tracheostomy under local anesthesia due to anterior neck fat deposits. A small airway is also a challenge for endotracheal intubation. Surgical modalities mentioned previously include transoral lipectomy and transcervical lipectomy with temporary tracheostomy. The transoral approach is indicated when lipomas only involve the supraglottis [5]. Lee DH and others [2] also described a case of isolated laryngeal lipoma removed trans orally with early recurrence. Thus, they preferred the transcervical approach with laryngofissure procedure when glottic or subglottic lipomas were present [2]. Clinicians should be aware of difficulties in completely excising the lesion and the disease's high recurrence rate. In patients with partial lipectomy, repeated operations have been reported [3]. Moreover, despite patent airway, laryngeal function after surgery has not been mentioned. Laryngeal involvement in Madelung's disease can become a life-threatening condition and is a clinical challenge to physicians. Respiratory symptoms should be evaluated for need of surgical interventions. A transcervical procedure is preferred when lipomas involve the glottis or subglottis. A transoral approach is indicated when the disease only involves the supraglottis. However, due to limited cases, long-term clinical courses after laryngeal lipomas removal remain unpredictable.
SDGs
Other Subjects
adult; airway dynamics; case report; computer assisted tomography; dyspnea; endotracheal intubation; general anesthesia; human; laryngoscopy; larynx tumor; letter; lipoma; lipomatosis; male; stridor; tracheotomy; Humans; Laryngeal Diseases; Lipomatosis, Multiple Symmetrical; Male; Middle Aged
Type
letter