Giant Cervicothoracic Madelung Disease Presenting with Airway Compromise: A Case Report from Ethiopia

Madelung's disease, or multiple symmetric lipomatosis, is an extremely rare disease and very likely to be under-diagnosed. It is characterized by multiple symmetrical non-encapsulated fat accumulations mainly located around the neck, shoulders, upper extremities and upper parts of the trunk. The disorder predominantly affects middle-aged men of Mediterranean origin with a history of ethanol intake. Reports of this uncommon disorder in women are very rare. The aim of this article is to report the endocrine and metabolic workup leading to the diagnosis of this uncommon disorder. A 55-year-old woman with osteoporosis was referred to our outpatient clinic with a suspected diagnosis of Cushing's disease. The patient complained of undesired weight gain with atypical fat accumulations predominantly in the upper trunk region during the previous 10 weeks. She presented with the characteristic physical features of Madelung's disease and underwent a thorough examination with endocrine and metabolic evaluation of this rare condition and was finally diagnosed with Madelung's disease. This report demonstrates how a diagnosis of this rare disorder can be reached efficiently. A history of osteoporosis in combination with weight gain and atypical fat accumulations ultimately led to the diagnosis of Madelung's diseases, a rather unknown disorder likely to be under-diagnosed. Although treatment options are limited, a diagnosis is still important for the affected individual.

Madelung's disease associated with polyneuropathy and symptomatic hypokalemia

Madelung’s disease is a rare disorder described for the first time in the year 1846 by Brodie. It is characterized by the occurrence of progressive, excessive, and symmetrical deposits of adipose tissue in the subcutaneous layer. Most often, these changes concern the neck, the nape of the neck, arms, and upper back, giving the patients a specific, pseudoathletic appearance. Madelung’s disease is also known as multiple symmetrical lipomatosis, benign symmetrical lipomatosis, and Launois-Bensaude syndrome. The most commonly affected ones are men who drink alcohol in excessive amounts. The fat masses emerging in the course of the disease are painless but can lead to adverse repercussions. Patients may experience dysphagia, dysphonia, difficulty in breathing, and limited mobility of the neck. The reasons for the willingness to take up treatment are also often esthetic reasons. The disease is usually accompanied by numerous metabolic disorders. The etiology of the disease has not been sufficiently explained so far, which creates diagnostic and therapeutic difficulties. Currently used treatment is limited to surgical resection of the resulting lesions or liposuction. Unfortunately, the effectiveness of these activities is limited. Most patients experience recurrence after treatment. This paper discusses the essence of Madelung’s disease, numerous aspects of etiology, the manner of diagnosis, and treatment based on current literature data.

Madelung's disease, or multiple symmetric lipomatosis, is a rare disease, characterized by accumulation of unencapsulated fat, generally located symmetrically around the neck and shoulders. Here, we present the case of a patient with diffuse lipomatosis accompanied by obstructive sleep apnea due to cervical involvement and facial deformity, which made it necessary to use nasal pillows for ventilation. The patient was hospitalized with a diagnosis of pneumonia and required noninvasive ventilation due to severe hypercapnia. A brief review of the literature was made, and we describe and discuss the investigation of this rare clinical case.

Madelung Disease

The proportion of early extubation after esophagectomy varies among hospitals; however, the impact on clinical outcomes is unclear. The aim of this retrospective study was to evaluate associations between the proportion of early extubation in hospitals and short-term outcomes after esophagectomy. Because there is no consensus regarding the optimal timing for extubation, the authors considered that hospitals' early extubation proportion reflects the hospital-level extubation strategy. Data of patients who underwent oncologic esophagectomy (July 2010-March 2019) were extracted from a Japanese nationwide inpatient database. The proportion of patients who underwent early extubation (extubation on the day of surgery) at each hospital was assessed and grouped by quartiles: very low- (<11%), low- (11-37%), medium- (38-83%), and high-proportion (≥84%) hospitals. The primary outcome was respiratory complications; secondary outcomes included reintubation, anastomotic leakage, other major complications, and hospitalization costs. Multivariable regression analyses were performed, adjusting for patient demographics, cancer treatments, and hospital characteristics. A restricted cubic spline analysis was also performed for the primary outcome. Among 37983 eligible patients across 545 hospitals, early extubation was performed in 17931 (47%) patients. Early extubation proportions ranged from 0-100% across hospitals. Respiratory complications occurred in 10270 patients (27%). Multivariable regression analyses showed that high- and medium-proportion hospitals were significantly associated with decreased respiratory complications [odds ratio, 0.46 (95% CI, 0.36-0.58) and 0.43 (0.31-0.60), respectively], reintubation, and hospitalization costs when compared with very low-proportion hospitals. The risk of anastomotic leakage and other major complications did not differ among groups. The restricted cubic spline analysis demonstrated a significant inverse dose-dependent association between the early extubation proportion and the risk of respiratory complications. A higher proportion of early extubation in a hospital was associated with a lower occurrence of respiratory complications, highlighting a potential benefit of early extubation after esophagectomy.

Symmetrical lipomatosis of the tongue: Case report and literature review

Obesity, obstructive sleep apnoea, and diabetes mellitus: anaesthetic implications

Multiple symmetric lipomatosis (MSL)—a rare metabolic condition characterized by diffuse, nonencapsulated, painless fat deposits—remains a challenging condition to treat. In addition, the clinical manifestation of MSL in women has not been fully understood due to the male-dominated prevalence of the disease. Here, we report a rare case of MSL with vulvar involvement and provide a review of the literature to increase the understanding of both the diagnosis and treatment of the disease. A 46-year-old Japanese woman, who had a history of chronic alcohol consumption for over 20 years, presented with bilateral giant enlargement of the labia major and complained of difficulty in walking and urination. Magnetic resonance imaging revealed nonencapsulated fat deposits involving the vulvar region and extending around the rectum. The patient underwent pubic lipectomy followed by episioperineoplasty, which resulted in a good esthetic outcome with sufficient patient satisfaction. The morphological presentation of MSL in women is different from that in men, and clinical expression of MSL in the vulvar region is rare but cannot be overlooked owing to the potential of serious sequelae such as walking difficulty, urination disorder, and sexual dysfunction. In such cases, lipectomy followed by episioperineoplasty is indicated for the treatment of the condition and its sequelae, while ensuring less chance of relapse and better esthetic outcome compared with other treatment strategies.Level of evidence: Level V, therapeutic, diagnostic, risk/prognostic study.

Background. In recent years, various methods of combined anesthesia during abdominal surgery have been introduced into clinical practice.Aim. To demonstrate the possibilities of a combination of high prolonged spinal anesthesia and endotracheal anesthesia during abdominal surgery.Materials and methods. A clinical case of combined use of high prolonged spinal anesthesia and endotracheal anesthesia in a 48-year-old patient with a tumor in the right half of the ascending part of the right half of the colon is presented.Results. The patient received a puncture of the spinal space at a standard point and was installed a spinal catheter in the cranial direction for 3 cm. An isobaric solution of marcaine in the initial dose of 20 mg was injected into the catheter. The regulation of the development of the block was regulated by the inclination of the head end of the table by 60°. After that endotracheal anesthesia was performed on the basis of fentanyl and propofol. This combination allowed to expand the scope of surgical intervention, provided adequate pain relief intraoperatively and in the postoperative period, without the use of narcotic analgesics. With the appearance of signs of recovery of pain sensitivity, intraoperatively or in the postoperative period, re-introduction of the anesthetic into the spinal catheter was performed in half of the initial dose with liquor barbotage. In the early postoperative period, the patient was on strict bed rest with a head end of the bed raised at 30–45°. The method provides complete segmental blockade and muscle relaxation in the area of operation, stability of central hemodynamics during surgery and in the postoperative period.Conclusion. This type of anesthesia is more easily tolerated by patients, accompanied by early awakening and extubation, characterized by stability of central hemodynamics, reduced risk of complications, the possibility of prolonging anesthesia with lower doses of narcotic analgesics in the intraoperative period, providing high-quality anesthesia in the postoperative period without resorting to the use of narcotic analgesics.

Post-operative recurrence after ileo-colic resection for Crohn's disease (CD) is somehow inevitable. Several studies investigated risk factors for recurrent disease. The introduction of laparoscopic surgery for CD has improved the quality of life of patients undergoing ileo-colic resection, however the need for protective ileostomy has remained substantially unchanged. Ileostomy diverts the stools before the anastomosis, and could potentially have a role in the disease relapse. Aim of the study was to correlate the presence of a protective ileostomy with the postoperative recurrence rate after ileo-colic resection for CD. All the patients who underwent ileo-colic resection for CD from 2008 onwards have been prospectively enrolled. Patients who had a so-called “protective” ileostomy or those requiring a postoperative ileostomy due to complications, were retrieved from the database and endoscopic recurrence in 1 and 3 years after surgery was compared with patients who did not receive any diversion. Other risk factors for recurrent disease were also considered. Recurrence was assessed through ileocolonoscopy and classified by the Rutgeerts scoring system. Chi-square and t Student tests were used for statistical analysis. Twenty out of 185 patients who underwent ileo-colic resection for CD, received an ileostomy (16 “protective” ileostomies and 4 for postoperative complications.). Endoscopy was performed in 166 patients at 1 year and in 115 patients at 3 years after surgery. Endoscopic recurrence (assessed as a Rutgeerts score > 2) was found in 25% of patients who had an ileostomy vs. 86% of those without ileostomy at 1 year (p < 0.03) and in 54% vs. 88% at 3 years (p < 0.04). Patients underwent closure of ileostomies from 1 to 5 months (3 months on average) away from surgery, with no major complications. The presence of an ileostomy in our CD patients undergoing ileo-colic resection appeared to represent a protective factor for post-operative endoscopic recurrence both at 1 and 3 years from surgery. Although the main bias represented by the limited number of patients treated with ileostomy, this result seems worthy of further investigation to better understand a possible role of intestinal diversion in CD recurrence.

Doppler ultrasound assessment of common femoral vein (CFV) flow could represent a simple, fast, and noninvasive technique to identify right ventricular dysfunction and venous congestion in patients undergoing cardiac surgery. Our primary objective was to determine the prevalence of abnormal CFV flow pulsatility before cardiac surgery. Secondary objectives included investigating its association with postoperative outcomes. We conducted a single-centre prospective cohort study and recruited adult patients undergoing cardiac surgery with cardiopulmonary bypass. We performed pulsed-wave Doppler ultrasound assessments of CFV flow at four timepoints: before surgery, after induction of anesthesia, immediately after surgery, and on postoperative day 1. When the Doppler profile showed cardiophasic variations in velocities, abnormal pulsatility was defined as a CFV pulsatility fraction ≥ 100%. Outcomes included postoperative complications, duration of postoperative support, and the cumulative time of persistent organ dysfunction after surgery (TPOD). Among the 150 patients included, 30 (20%) presented abnormal CFV pulsatility (pulsatility fraction [PF] ≥ 100%) before surgery. Preoperative abnormal CFV pulsatility was associated with a higher proportion of postoperative acute kidney injury (35% vs 16%; P = 0.02), major complications (21% vs 7%; P = 0.02), and a longer duration of TPOD (median [interquartile range]; 24 [9-56] hr vs 5 [3-19] hr; P < 0.001). After adjusting for the European System for Cardiac Operative Risk Evaluation II, the association between PF ≥ 100% before surgery and TPOD remained statistically significant (β = 0.72; 95% confidence interval, 0.21 to 1.23; P = 0.01). An abnormal CFV Doppler was associated with higher right atrial pressure and tricuspid regurgitation. Abnormal CFV flow pulsatility occurred in 20% of patients before cardiac surgery and was associated with a higher rate of major postoperative complications, longer durations of postoperative support and care, and a longer cumulative time of persistent organ dysfunction after surgery. ClinicalTrials.gov ( NCT05038267 ); first submitted 28 August 2021.

Introduction: An early and specific indicator is needed to prognosticate acute myocardial infarction (MI). This indicator should be simple, quick, reliable, non-invasive, inexpensive, and easily applicable to all the patients especially in a resource-constrained setting. The present study tried to evaluated efficacy of ST score on electrocardiography as a prognostic indicator in acute ST-elevated MI (STEMI). Materials and Methods: A prospective study was conducted on consecutive thrombolysed patients of STEMI admitted in teaching hospital. ST score defined as sum of ST-segment elevation in all leads related to infarct location was calculated in standard 12-lead electrocardiographic tracings immediately on admission and then serially postthrombolysis at 90 min, 6 h, 24 h, and day 3 and 5 of admission. Primary end-point was all-cause mortality at 30 days. Results: The mean ST score between survivor and nonsurvivor was 19.27 mm and 16.16 mm, respectively. The score on admission in patients who had poor outcome was significantly higher than those who had good outcome (poor = 20.27 mm vs. good outcome = 12.47 mm; P = 0.002). This difference persisted throughout but was maximum at 90-min postthrombolysis (13.82 mm vs. 7.39 mm; P = 0.0001). The optimal cutoff point maximizing sensitivity and specificity was found at 11 mm for both anterior- and inferior wall MI (IWMI) with a sensitivity of 73% and specificity of 58%. Conclusion: This study concludes that an increase of 1 mm in ST score increased the odds of complications by 1.06 (95% confidence interval [CI], 1.01–1.2) for anterior-wall MI and by 1.09 (95% CI, 0.96–1.2) for IWMI. In a resource-constrained health facility where electrocardiography may be the only available equipment, ST score can easily and effectively be used to stratify patients into high-risk and low-risk categories.

The aim of the present study was to evaluate the residual lesions after conization and the clinical outcome of patients with multifocal micro-invasive squamous cell cervical carcinomas (MMSCCs) treated with different surgical strategies. A retrospective study was carried out in 98 patients with MMSCCs diagnosed by conization and treated between January 2010 and December 2016 in 2 institutions. The patients underwent further different surgeries as therapeutic conization, extrafascial hysterectomy (ES), modified radical hysterectomy (MRH), radical hysterectomy (RH) with or without pelvic lymph node dissection (PLND) and regular follow-up. The clinicopathological characteristics of all of the patients were recorded. The risk factors of residual lesions and that of the recurrence were also analyzed in the present study. The logistic regression analysis revealed that cone margins (P=0.001) were correlated with residual disease after conization whereas parameters including age, gravidity, parity, menopause, stage, LVSI and the number of lesions were not predictors of residual lesions. The cone margin status also indicated the incidence of residual disease as follows: The risk of residual disease was lower with a negative margin when compared with the margin with micro-invasive carcinoma [MIC; odds ratio (OR)=0.064, P=0.012] and was lower in margin with a high-grade intraepithelial lesion than the margin with MIC (OR=0.297, P=0.287). The Cox regression analysis revealed that there were no significant correlations between the following surgery scales and postoperative recurrences, nor were any significant correlations found between the recurrences and the gravidity and parity, postmenopausal state, stage, residual disease after conization, margin status, LVSI and number of lesions (P>0.05). Positive cone margin was the only predictive factor for residual disease in patients with MMSCCs. There were no significant correlations between the surgical scales and postoperative recurrences. This result may be due to the excellent prognosis of MICs despite multiple lesions, regardless the treatment.

Managing difficult airways in patients with complex comorbidities and language barriers requires a strategic, multidisciplinary approach. We present the case of a 60-year-old non-smoking female with a history of multinodular euthyroid goiter causing significant tracheal stenosis, scheduled for an elective procedure. Her sole documented comorbidity was a newly diagnosed diabetes mellitus. No additional health conditions were reported. A notable challenge was the language barrier, as the patient did not speak the local language, necessitating the presence of a translator to facilitate all communication and informed consent. Given the significant tracheal compression observed in imaging studies, an awake fiberoptic intubation was planned to reduce airway compromise risks. This approach allowed for continuous patient cooperation and monitoring, minimizing the chance of sudden airway obstruction. Throughout the procedure, maintaining clear communication and patient cooperation was essential for the safe execution of the intubation. This case emphasizes the importance of thorough preoperative planning and individualized airway management strategies, particularly in patients with significant anatomical challenges. It highlights the need to adapt standard airway techniques to address complex physiological conditions and underscores the value of a methodical approach to ensure safe and successful outcomes in difficult airway cases.