1. Abstract Achalasia is a rare motility disorder of the esophagus and present by failure of lower esophageal sphincter (LES) relaxation during swallow. This rare disease of esophagus with it symptoms can mimic a variety of common illnesses of esophagouse,but not the mediastinum A man with 39 years old was referred to our chest clinic with complaints of dysphagia to solid foods and liquids food, chest pain and cough after eating, Dyspnea and regurgitation present latter at night with cough. These symptoms present for the past 2 years and in resent three months becoming progressively worse. A CXR and computer tomography (CT)of chest were performed which show right lung cystic or mass lesion with necrosis. After visit by a pulmonologist and a thoracic surgeon, the patient was transferred to the thoracic Surgery department for a planned surgical intervention. After postero-lateral thoracotomy and exploration, the diagnosis was achalasia whit huge megaesophaguse. Esophagectomy and gastric pull-up was performed. The patient died three day post operation because of pulmonary sepsis due to aspiration
Keywords: Achalasia; Dysphagia; Regurgitation; Diverticula; Megaesophagus
2. Introduction In vast majority of patients, the etiology of achalasia remains unknown [1, 2]. Achalasia is an unusual motility disorder of esophagus which involve the smooth muscle of the esophagus wall and (LES). The incidence of achalasia about 1 in 100,000 of people and the age of most patients on the time of diagnosis are between 25 to 60 years old [1-3]. In more than 80% of patients, major symptoms of achalasia are usually dysphagia to solid and liquid food, regurgitation, weight loss and chest pain [1,4,5]. The cause of These symptoms are the absence or weak of peristalsis in esophageal body, failure relaxation of LES and pressure in the (LES) is higher than normal, on swallowing time [5, 6] One of primary diagnostic methods are CXR and barium swallow in liquid form and then the movement of barium may follow by fluoroscopy to LES [1, 2]. Other methods of diagnosis are an endoscopy end esophageal ultrasonophrapy and manometry [6, 3]. Several surgical and nonsurgical methods have been described for disruption muscle of LES [3,6] But in recent years, minimally invasive techniques as laparoscopy or thorascopy is the surgical treatment of choice [6 ,7]. First, cardiomyotomy performed by Heller through an abdominal approach which in this method both anterior and posterior side of LES muscle was divisions [6,1]. Palliation of dysphagia was the first goal of therapy but the motor dysfunction persists after the cardiomyotomy [8]. A modified Heller's myotomy performed at present time and is the most efficient for long-term solution [6,8]. In some reports recommended often as the first line of treatment [9]. When the esophagus was re-evaluation with barium after more than 10 years, diverticulum formation may present through the myotomized area, and the size of diverticulum depend to the length of the myotomy [8,9]. This complication need for re-operation r (29%) [7,9]. For treatment of this complication, need to take down the Nissen fundoplication or to perform an esophagectomy [7]. The main aim of the esophagogastric myotomy is to reduce the pressure of LES relaxation by division of slingand clasp fibers. One complication of this operation is, pathologic gastero-esophageal reflux and for prevention of reflux a fundoplication procedure remains necessary [10] After the advent of minimally invasive technology, attempts at a pure thoracoscopic approach were problematic and commonly led to incomplete LES division even under endoscopic guidance [2]. More recently, laparoscopic myotomy with partial fundoplication has gained popularity for the treatment of achalasia [3]. Controversy still persists regarding the ideal length of myotomy to treat esophageal achalasia [2,8]. In sigmoid or megaesophagousin form, esophagectomy and gastric pull-up or colon bypass indicated [4,12]. The aim of this case report is to show the rare clinical presentation and imaging finding in achalasia. Because with our knowledge and review of literatures we don’t find such case. With this imaging presentations and very huge megaesophagousin.
3. Case Presentation A man with 39 years old was referred to our chest clinic with complaints of dysphagia to solid foods and liquids food, chest pain and cough after eating, Dyspnea and regurgitation present latter at night with cough. These symptoms present for the past 2 years and in resent three months becoming progressively dysphagia, was overcome by drinking water, a constant weight loss of 23 kg, which had developed over the past two years. Additional symptoms were retrosternal pain, regurgitations, night coughing, dyspnea, right side chest pain, heaviness of chest wall and recurrent respiratory infection. While the laboratory results did show anemia (9mh /l), (albumin 2mg/l) others was normal, a CXR and computed tomography (CT) scan of the chest show a huge cystic lesion with air fluid level in right hemithorax and widening of the mediastinum up to 10 × 8 cm in diameter accompanied by a slit shaped of compress and mild deformity of cranial and narrowing of the trachea (Figure1-6). Differential diagnosis in radiologist report was complicated hydatid cyst, bronchogenic cyst and hiatal hernia, esophageal duplication or mediastinal necrotic masses which compress the esophagus and trachea. Upper endoscopy and bronchoscopy did not performed because the patient cannot tolerated this procedures. Whit above diagnosis, we performed a right postero-lateral thoracotomy whit double lumen for prevention of aspiration during operation, thoracic cavity in 5th intercostal space was opened, during exploration we found a very dilated esophagus which in the proximal portion was very dilated from than other portion of esophageouse. The dilated portion was opened and the content of cavity was full of debris and remnant of swallowing food, after we find two lumen one above and one in the lower portion of this cavity. Very huge megaesophagouse due to achalasia was the definitive diagnosis (Figure 7,8). Esophagectomy was performed, next the position of patient was changed to supine, after laparotomy, for esophagus reconstruction , gastric tube was made, than gastero-esophagostom was performed in the left side of the neck. A feeding joujnostomy tube was performed and abdomen, chest and neck incision was closed. Patient referred to intensive care, three day post-operative the patient was died because of pulmonary sepsis due to aspiration problem.
4. Dicussuion The majority (80%) of patients with achalasia complain of dysphagia to solids and liquids food and these symptoms may be the primary compliant in the achalasia patients [1,4]. In 40-60% of patients weight loss, reurgitations, chest pain, cough and heartburn are reported [2,4]. Occasionally, respiratory complications, such as aspiration pneumonia, lung abcess and bronchiectasis, are another complications of achalasia [3,4]. In advanced stages of achalasia in the few publications report dyspnea, stridor and swelling of the neck due to dilatation of esophagouse [6]. In the literature However, acute total airway compression and death are reported [11]. In this case, the patient present with high weight loss of 21 kg over two years, respiratory problem and hypoalbonomia which show the achalasia is very advanced with delay diagnosis and treatment [4,12]. In this case the CT scan of chest show an extensively widening of mediastinum and a cystic and solid mass with air –fluid level with compression mild of the trachea and carina and bronchial system, respiratory system was significantly compromised. However, in such case difficulties intubation may happen and should be take care of aspiration during induction of anesthesia and before or during and poste surgical intervention, for prevention of sever and fatal aspiration, we insert a tracheal tube in the esophagus and aspiration all content of esophagus In our experience we used this method in four case with successfully. Diagnosis tools are esophageal manometry, and Chest-X-ray or barium swallow and perform an endoscopy. Another diagnostic method swallow barium should in liquid form. And radiologist will then track the barium’s movement down your esophagus through X-rays [1-4]. In our case we used computer- tomography of chest, neck and CXR, but patient can.t tolerate, esophacoscopy, bronscopy and barium swallow. Several types of treatment can either temporarily or permanently reduce symptoms of patients and alter the function of the LES. However most achalasia treatments involve of the LES [7-10]. The main aim of achalasia treatment is to reduce patient’s symptoms and to improve their quality of life [1,2,4]. Achalasia is an irreversible disease, and the restoration of esophageal peristalsis is unreliable [4]. Therefore, the ultimate goal of therapy is to relieve the obstruction at the level of the EGJ by either drugs which can produce LES relaxation, botulinum toxin injection, by pneumatic dilatation, per oral endoscopic myotomy, surgical techniques laparoscopic, thorascopy, open thoracotomy or laparatomy Heller myotomy [8-12]. Although the one of fatal complicatin in advanced achalasia during intubation is aspiration. In this patients, if adequate treatment perfome with delay, sever pulmonary complications such as fibrosis of lung occur because of long time of aspiration Tracheal function such as tracheomalacia due to long time of compression might have been possible, compressed of trachea due to mega esophagus might result in life-threatening complication also in earlier courses of postoperative time [3,4,6]. Thus, even more severe problems associated with endotracheal intubation and postoperative extubation, may need for tracheostomy [4,6]. In older patients and possible respiratory or other co-morbidities and, this distinct finding of a slit-shape and For safety reasons, extubation was performed on the intensive care units [8-12]. Therefore, proper therapy may perform in order to restore and prevention of megaesophagus as early as possible [4,6]. Achalasia is a rare esophageal disease such as gastroesophageal reflux and usually diagnosed too late, which often have some difficulty on the time of correct and tratment for many physicians [3,4,6]. However, we recommend that patients with suspicion of achalasia with typical or atypical, especially symptoms should be referred for further diagnostic work-up such as high resolution manometry and barium swallow [4,6,7].
5. Conclusion This case report show that achalasia with megaesophagousin, mediastinum widening, cystic lesion and mediastinum mass may be in differentiated diagnosis of megaesophagouse due to achalasia and early treatment need in order to prevent severe and fatal complications, becaused the compression of tracheal due to megaesophagus and sever pulmonary aspiration. If diagnosed by radiologic imaging only, in such patients tracheal obstraction by megaesophagouse or tracheomalacia can difficulty intubation for by anesthesiologists during intubation for surgical interventions and prevention of life-threatening aspiration during surgery, we putt a tube in esophagus and aspiration the content of esophagus.
References 1. Richter JE. Achalasia- an update. J Neurogastroenterol Motil. 2010; 16(3): 232-42.
2. Park W, Vaezi MF. Etiology and pathogenesis of achalasia: The current understanding. Am J Gastroenterol 2005; 100: 1404-14.
3. Chuah SK, Hsu PI, Wu KL, Wu DC, Tai WC, Changchien CS. 2011 update on esophageal achalasia. World J. Gastroenterol. 2012; 14:1573-1578.
4. Moritz Kaths J, Daniel B. Foltys, Uwe Scheuermann, Mari Strempel, Stefan Niebisch, Maren Ebert, et al. Achalasia with megaesophagus and tracheal compression in a young patient: A case report. International Journal of Surgery Case Reports. 2015; 14: 16-18.
5. Doshi AH, Aw J, Costa F, Cohen L, Som PM. Cervical tracheal compression in a patient with achalasia: an uncommon event. AJNR Am. J. Neuroradiol.2009; 4: 813-814.
6. Eckardt VF. Clinical presentations and complications of achalasia. Gastrointest. Endosc. Clin. N Am. 2001; 2: 281-292.
7. Zonca P, Cambal M, Labas P, Hrbaty B, Jacobi CA. The role of laparoscopic Heller myotomy in thetreatment of achalasia. Bratisl Lek Listy. 2014; 115: 156-60.
8. Yaghoobi M, Mayrand S, Martel M, Roshan-Afshar I, Bijarchi R, Barkun A. Laparoscopic Heller’s myotomy versus pneumatic dilation in the treatment of idiopathic achalasia: A meta-analysis of randomized, controlled trials. Gastrointest Endosc. 2013; 78: 468-75.
9. Huffmanm LC, Pandalai PK, Boulton BJ, James L, Starnes SL, Reed MF, et al. A safe operation with higher postoperative quality-of-life indices. Surgery. 2007; 142: 613-18.
10. Boeckxstaens GE, Annese V, des Varannes SB, Chaussade S, Costantini M, Cuttitta A, et al. Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. N Engl J Med. 2011; 364: 1807-16.
11. Giustra PE, Killoran PJ, Wasgatt WN. Acute stridor in achalasia of the esophagus (cardiospasm) Am. J. Gastroenterol. 1973; 2: 160- 164.
12. Manouchehr Aghajanzadeh, Anoush Dehnadi Moghadam, Hossein Hemmati, Sara Massahnia. Results of Short- and Long-Segment Cardioesophageal Myotomy for Achalasia, Saudi Journal of Gastroenterology. 2012; 18(4): 237-40.
13. Ho V, Whiting P. Acute airway obstruction secondary to achalasia mega-oesophagus. Anaesth. Intensive Care. 2008; 36 (4): 582-584. 14. Al-Abdoulsalam T., Anselmo M.A. An 11-year-old male patient with refractory asthma and heartburn. Can. Respir. J. 2011; 18(2).Manoucheher Aghajanzadeh. Repot a Rare Case of Achalasia Which Present as Mediastinal Cysts or Masses. Annals of Clinical and Medical Case Reports 2021