History

Fact Explanation
Dysphagia Long lasting non-progressive dysphagia is characteristic of motility disorders of the esophagus, which lead to megaesophagus. Dysphagia to liquids is commoner than to solids. [3]
Stridor Stridor is a relatively rare but significant complication of megaesophagus. Dilated esophagus causes pressure effects and compresses the upper airway, leading to stridor. Compression of the superior vena cava leads to venous congestion of the airway and airway edema, which further narrows the airway. [1,3]
Symptoms of esophageal carcinoma Patients with megaesophagus may progress to squamous cell carcinoma of the esophagus, due to chronic stasis of food and irritation of the epithelium. Patients present with short duration of progressive dysphagia on top of chronic non-progressive dysphagia. [2]
Symptoms of achalasia cardia Patients with achalasia cardia are at risk of developing megaesophagus. Achalasia presents with dysphagia and regurgitation of undigested food particles. Typically dysphagia is prominent for liquids than for solids.
History of diabetic neuropathy Patients with poorly controlled diabetes develop diabetic autonomic neuropathy and uncoordinated peristalsis of the esophagus and megaesophagus. Nausea, vomiting, change in bowel habits, and dizziness are symptoms of diabetic autonomic neuropathy. [1]
History of Chagas disease Patients with chronic Chagas disease develop megaesophagus as a complication. Patients with acute Chagas disease have fever, fatigue, body aches, headache, rash, loss of appetite, diarrhea, and vomiting. After an asymptomatic phase, patients develop cardiac and intestinal complications.
History of Parkinson's disease Parkinson's disease is also associated with increased risk of megaesophagus.
References
  1. NATESH B. G., CATON N., KIM D., SHETTY A.. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus. Case Reports in Otolaryngology [online] 2013 December, 2013:1-3 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504
  2. HENRY MA, LERCO MM, RIBEIRO PW, RODRIGUES MA. Epidemiological features of esophageal cancer. Squamous cell carcinoma versus adenocarcinoma. Acta Cir Bras [online] 2014 Jun, 29(6):389-93 [viewed 13 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24919048
  3. NATESH BG, CATON N, KIM D, SHETTY A. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus Case Rep Otolaryngol [online] 2013:368504 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504

Examination

Fact Explanation
Signs of respiratory distress Patients with megaesophagus and tracheal compression can present in respiratory distress. These patients are tachypnoec cyanosed and gasping for breath. Stridor is a common finding in patients with tracheal obstruction.
Swelling of the neck Some patients with significant megaesophagus and food accumulation may have neck swelling. The swelling is diffuse, firm and non-tender. [1,2]
Dilated neck veins Dilatation of the superficial veins in the neck occurs due to esophageal dilatation and compression of the superior vena cava. [1]
Signs of lower respiratory tract infection Patients with megaesophagus can develop secondary respiratory tract infection due to aspiration. Lobar consolidation can be detected commonly over the right lower lobe. The affected lobe shows reduced chest expansion, dull percussion note and reduced breath sounds. Patients are often febrile due to infection.
References
  1. NATESH B. G., CATON N., KIM D., SHETTY A.. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus. Case Reports in Otolaryngology [online] 2013 December, 2013:1-3 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504
  2. NATESH BG, CATON N, KIM D, SHETTY A. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus Case Rep Otolaryngol [online] 2013:368504 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504

Differential Diagnoses

Fact Explanation
Achalasia Achalasia is a common etiological factor for megaesophagus.
Pseudoachalasia Pseudoachalasia is an esophageal motility disorder. Patients present with short duration of dysphagia. This condition is often associated with submucosal gastric cancer. [1]
Chagas disease Chronic Chagas disease can result in megaesophagus due to destruction of intramural autonomic ganglia. [2]
Carcinoma of the esophagus Esophageal carcinoma should be considered a differential diagnosis in patients presenting with dysphagia, especially in elderly.
References
  1. CAMPO SM, ZULLO A, SCANDAVINI CM, FREZZA B, CERRO P, BALDUCCI G. Pseudoachalasia: A peculiar case report and review of the literature. World J Gastrointest Endosc [online] 2013 Sep 16, 5(9):450-4 [viewed 14 August 2014] Available from: doi:10.4253/wjge.v5.i9.450
  2. ANIS RASSI, JOSÉ ANTONIO MARIN-NETO. Chagas disease. The Lancet [online] 17 April 2010: 375 (9723) 1388 – 1402. [viewed 14 August 2014] Available from: doi:10.1016/S0140-6736(10)60061-X

Investigations - for Diagnosis

Fact Explanation
Uppergastrointestinal endoscopy (UGIE) UGIE reveals grossly dilated and tortuous oesophagus. [1]
Barium swallow test Barium swallow shows dilated esophagus. Bird beak appearance is seen in achalasia. [2]
Chest X-ray Chest X-ray shows the megaesophagus as a large air- filled swelling. [1]
CT scan CT scan can also detect the dilated esophagus. There is no demonstrable esophageal stenosis.
References
  1. NATESH BG, CATON N, KIM D, SHETTY A. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus Case Rep Otolaryngol [online] 2013:368504 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504
  2. EL-TAKLI I, O’BRIEN P, PATERSON W. Clinical diagnosis of achalasia: How reliable is the barium x-ray? Can J Gastroenterol [online] 2006 May, 20(5):335-337 [viewed 13 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659891

Management - General Measures

Fact Explanation
Airway management Patients who present with signs of acute respiratory distress should be given oxygen. Assessment and maintenance of the patency of the airway is lifesaving. Timely endotracheal intubation or emergency tracheostomy are necessary in some patients. Nasogastric decompensation of the esophagus is an effective in restoring the airway patency in an emergency. [1]
Decompression of the esophagus Decompression of the food filled esophagus with a wide-bore nasogastric tube provides immediate symptomatic relief, especially in patients with stridor due to tracheal compression. Nasogastric decmopression can be difficult in some patients due to impacted solid food particles, in which instances intensive manual disimpaction is necessary. [1]
References
  1. NATESH BG, CATON N, KIM D, SHETTY A. Idiopathic Oesophageal Dysmotility Disorder: Stridor Secondary to Megaesophagus Case Rep Otolaryngol [online] 2013:368504 [viewed 13 August 2014] Available from: doi:10.1155/2013/368504

Management - Specific Treatments

Fact Explanation
Surgery Esophagectomy and esophagogastric anastamosis is indicated in the treatment of symptomatic megaesophagus. Heller's myotomy and fundoplication, or esophageal resection is used in advanced disease. [1,2]
References
  1. HERBELLA FA, AQUINO JL, STEFANI-NAKANO S, ARTIFON EL, SAKAI P, CREMA E, ANDREOLLO NA, LOPES LR, DE CASTRO POCHINI C, CORSI PR, GAGLIARDI D, DEL GRANDE JC. Treatment of achalasia: lessons learned with Chagas' disease. Dis Esophagus [online] 2008, 21(5):461-7 [viewed 13 August 2014] Available from: doi:10.1111/j.1442-2050.2008.00811.x
  2. AQUINO JL, CAMARGO JG, SAID MM, MERHI VA, MACLELLAN KC, PALU BF. [Cervical esophagogastric anastomosis evaluation with a mechanical device versus manual suture in patients with advanced megaesophagus]. Rev Col Bras Cir [online] 2009 Feb, 36(1):19-23 [viewed 14 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20076864