Leiomyomatosis masquerading as sigmoid achalasia: a rare occurrence.
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ABSTRACT:
SUBMITTER: Nabi Z
PROVIDER: S-EPMC7947316 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
Publications
Leiomyomatosis masquerading as sigmoid achalasia: a rare occurrence.
Nabi Zaheer Z Chavan Radhika R Darisetty Santosh S Kotla Rama R Reddy D Nageshwar DN
VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy 20201219 3
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Project description:Peroral endoscopic myotomy (POEM) has been recently considered as the first treatment option for achalasia. The standard POEM procedures are often successful in most patients, but sometimes technical challenges are encountered. We report a new technique that is divided between two tunneling sites in the esophagus for sigmoid-type achalasia. A 40-year-old male patient with dysphagia for 10 years was diagnosed with a sigmoid-shaped esophagus at our hospital. We devised a two-stage myotomy technique to treat sigmoidtype achalasia. The myotomy was first performed in the upper part of the greater flexion area and then in the lower part of the flexion. We termed this method "two-stage POEM", which was successfully performed without any complications. This new POEM method can also be used to improve symptoms in patients with achalasia who have a structural deformity that may result in a high change of treatment failure.
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Project description:Background/aimsPatients with achalasia-related esophageal motility disorders (AEMDs) frequently present with dilated and sigmoid esophagus, and develop esophageal diverticulum (ED), although the prevalence and patients characteristics require further elucidation.MethodsWe conducted a multicenter cohort study of 3707 patients with AEMDs from 14 facilities in Japan. Esophagography on 3682 patients were analyzed.ResultsStraight (n = 2798), sigmoid (n = 684), and advanced sigmoid esophagus (n = 200) were diagnosed. Multivariate analysis revealed that long disease duration, advanced age, obesity, and type I achalasia correlate positively, whereas severe symptoms and integrated relaxation pressure correlate negatively with development of sigmoid esophagus. In contrast, Grade II dilation (3.5-6.0 cm) was the most common (52.9%), while grade III dilation (≥ 6 cm) was rare (5.0%). We found early onset, male, obesity, and type I achalasia correlated positively, while advanced age correlated negatively with esophageal dilation. Dilated and sigmoid esophagus were found mostly in types I and II achalasia, but typically not found in spastic disorders. The prevalence of ED was low (n = 63, 1.7%), and non-dilated esophagus and advanced age correlated with ED development. Patients with right-sided ED (n = 35) had a long disease duration (P = 0.005) with low integrated relaxation pressure values (P = 0.008) compared with patients with left-sided ED (n = 22). Patients with multiple EDs (n = 6) had lower symptom severity than patients with a single ED (P = 0.022).ConclusionsThe etiologies of dilated esophagus, sigmoid esophagus, and ED are considered multifactorial and different. Early diagnosis and optimal treatment of AEMDs are necessary to prevent these conditions.
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Project description: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 evidence-based 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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Project description:Background: The efficacy and safety of peroral endoscopic myotomy (POEM) in the treatment of sigmoid-type achalasia is unknown. This meta-analysis aims to explore the clinical outcomes of POEM for sigmoid-type achalasia. Method: We searched all relevant studies published up to September 2020 in PubMed, Embase, and Cochrane library databases. Meta-analyses for clinical success, Eckardt score, angle of esophageal tortuosity, diameter of esophagus, lower esophageal sphincter (LES) pressure, integrated relaxation pressure (IRP), adverse events, and gastroesophageal reflux diseases were performed based on random or fixed-effects models as needed. Results: We found a total of eight studies that provided data on 248 patients. Overall, the pooled clinical success was achieved in 211 sigmoid-type achalasia patients [90.4%; 95% confidence interval (CI), 85.5%-93.8%]. The pre- and post-POEM Eckardt scores, angle of esophageal tortuosity, diameter of esophageal, LES pressure, and IRP were significantly improved (All p < 0.05). The pooled adverse events rate was 13.0% (95% CI, 3.6%-37.4%). The pooled objective confirmation of reflux rate was 41.5% (95% CI, 26.5%-58.3%), and symptomatic reflux rate was 12.5% (95% CI, 8.3%-18.4%). Conclusions: Our current evidence indicated that POEM is an effective and safe therapeutic modality for the treatment of sigmoid-type achalasia.
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Project description:Colorectal cancer is a very common disease process, as is ureteral obstruction, but the two are typically mutually exclusive. The case report presented details a 35-year-old male with left sided flank pain and hydroureteronephrosis caused by metastatic spread of previously diagnosed and treated sigmoid adenocarcinoma. About two years previously, he had been diagnosed with Stage IV, T4a sigmoid colon cancer with liver metastasis and had resection of his primary tumor as well as metastatic sites and several rounds of chemotherapy. This case is an example of the different ways that cancer can grow, evading detection by spreading in atypical patterns.
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Project description:Background/aimsSigmoid esophagus and/or megaesophagus are considered as an advanced stage in the natural history of achalasia cardia. The role of peroral endoscopic myotomy (POEM) in these subset of patients is emerging. We performed a systematic review and metanalysis to study the efficacy of POEM in advanced achalasia cardia with sigmoid and megaesophagus.MethodsA literature search in PubMed and Embase was done from inception till August 3, 2021 to look for studies reporting exclusively on the role of POEM in advanced achalasia cardia with sigmoid and/or megaesophagus. The random effect method with inverse variance approach was used for the computation of pooled prevalence. For 2 groups' analysis of continuous outcome standardized mean difference was used as the summary measure.ResultsEleven studies with 428 patients were included for analysis. The pooled technical and clinical success was 98.27% (95% CI, 96.19-99.22; I2 = 0%) and 89.38% (95% CI, 84.49-92.86; I2 = 26%) and on subgroup analysis into sigmoid and megaesophagus it was (98.06% [95% CI, 95.41-99.19; I2 = 0%], 98.47% [95% CI, 92.72-99.69; I2 = 0%] and 87.92% [95% CI, 80.68-92.70; I2 = 37%], 88.36% [95% CI, 62.62-97.17; I2 = 77%]) respectively. The clinical success at < 1 year and 1-3 year follow-up was 89.37% (95% CI, 82.82-93.61; I2 = 0%) and 88.66% (95% CI, 81.65-91.22; I2 = 46%) respectively. There was a significant reduction in the post-POEM scores with standardized mean difference for Eckardt score (4.81), for integrated relaxation pressure at 4 seconds (1.93), and for lower esophageal sphincter pressure (2.06).ConclusionsPOEM is an effective modality of treatment even in the subset of patients of advanced achalasia cardia with sigmoid and megaesophagus.
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