Peroral endoscopic myotomy in a pediatric patient with refractory achalasia: a case report and review of the literature

A 15-year-old male patient presented with dysphagia, regurgitation, weight loss and retrosternal pain. The diagnosis of achalasia was made 4 years before. The esophagogram revealed severe esophagus dilatation and the classic “bird-beak” termination. A Heller myotomy plus fundoplication and endoscopic balloon dilatation were conducted four months previously. Nevertheless, the symptoms persisted and the last high-resolution manometry study still showed achalasia type II. The patient underwent a peroral endoscopic esophageal myotomy (POEM). POEM is a feasible and safe procedure for experienced and properly- equipped health care delivery centers and could be used as a rescue treatment in refr e present the youngest patient with achalasia in our region who had a successful response to rescue POEM.

Atelectasis: An Uncommon Sign of Achalasia in Childhoo

An 11-year-old boy presented to our pediatric pulmonology clinic with a 3-month history of atelectasis evident on his chest radiography. Breath sounds revealed fine crackles in the right lower zone and rhonchi in the upper zones. His initial pulmonary function test was compatible with restrictive pulmonary disease. Chest tomography revealed that the trachea, right intermediate, and middle lobe bronchi were narrowed by megaesophagus. Esophagogram determined dilatation of the esophagus and “bird-beak” sign in the esophagogastric junction but it was not sufficient to diagnose. Esophageal manometry which is the gold standard test for achalasia was performed and type 2 achalasia was diagnosed. His symptoms improved following Heller myotomy conducted together with Dor fundoplication. Although respiratory problems are more common in infants and younger children, atypical respiratory presentations may also occur during adolescence. Achalasia should be one of the rare differential diagnoses of pediatric restrictive pulmonary disease.

Caecal Volvulus

Colonic volvulus is the third leading cause of large bowel obstruction. About 35% of these are located in the caecum. Though, relatively, a rare cause of obstruction, the incidence of caecal volvulus is steadily increasing at a rate of about 5% per year. Mortality due to caecal volvulus may be as high as 40% especially in the presence of gangrene and sepsis. Clinical presentation may be acute and fulminant or as a mobile caecum syndrome with intermittent abdominal pain. “Whirl,” “Coffee bean,” and “bird beak” signs seen on computed tomography are pathognomonic. Colectomy is the preferred treatment as it obviates any chance of recurrence. A conservative approach to colectomy such as limited ileocaecal resection and ileostomy formation in critically ill patients or in those with poor physiological reserve may be associated with better postoperative outcomes.

Association between aortic arch angulation and bird-beak configuration after thoracic aortic stent graft repair of type B aortic dissection

OBJECTIVES The goal of this study was to investigate factors favouring the bird-beak configuration after thoracic endovascular aortic repair (TEVAR) for type B aortic dissection. METHODS We retrospectively analysed 76 patients with type B aortic dissection who underwent landing zone 1 and 2 TEVAR from December 2015 to January 2018. Preoperative aortic arch geometry (aortic arch length, maximal diameter and angulation), stent graft details and operative details were evaluated. A bird-beak configuration was defined as a ≥5-mm gap between the proximal end of the stent and the aortic wall of the lesser curvature. RESULTS Patients were stratified into those with (n = 46) and without (n = 30) a bird-beak configuration. The baseline demographics, dissection chronicity, clinical features and implanted devices were largely similar between the 2 groups. No significant difference was observed in the arch length or maximal arch diameter. However, the mean aortic arch angulation was greater in patients with than without a bird-beak configuration (61.4° vs 51.3°; P < 0.001). No influence of either the stent graft brand or the proximal stent graft type was observed. The multivariable analysis showed that the aortic arch angulation was an independent risk factor for a bird-beak configuration (odds ratio 1.15, 95% confidence interval 1.07–1.24; P < 0.001). A cut-off angle of 59.15° was predictive of a bird-beak configuration (sensitivity 59%; specificity 77%). CONCLUSIONS The preoperative aortic arch angulation was an independent predictor of a postoperative bird-beak configuration in patients with type B aortic dissection who underwent TEVAR that involved the aortic arch. An angle of >59.15° may imply a relatively hostile anatomy with a higher risk of a bird-beak configuration.

A Rare Cause of Left Atrium Compression: Esophageal Achalasia

Background Extrinsic compression of left atrium (LA) due to esophageal achalasia is uncommon. Patients might present with dysphagia, dyspnea, and even hemodynamic compromise. Prompt detection with thorough differential diagnosis is crucial for subsequent management. We presented a case with LA compression by esophageal achalasia, and literature review regarding the clinical manifestation, diagnosis, and treatment strategy was performed to provide an updating knowledge of the disease.Case presentation A 59-year-old relatively healthy man presented with dysphagia accompanied by chest tightness and breathlessness after a large meal. His chest X-ray film disclosed a widened mediastinum. The barium swallow esophagogram revealed contrast pooling at the esophagogastric junction with a bird beak shape. Meanwhile, the transthoracic echocardiogram showed a round-shaped, well bordered, hyperechogenic, and heterogeneous mass (5.1 cm x 3.8 cm in size) compressing the LA irrespective of the systolic or diastolic phase. A chest contrast-enhanced computed tomography scan showed diffuse esophageal dilatation with a smoothly thickening wall aligned compressing the LA. Due to the abovementioned image findings, extrinsic compression of LA by esophageal achalasia was diagnosed.ConclusionLA compression due to esophageal achalasia is not common. Remarkably, given a patient presenting dysphagia and concurrent dyspnea after meals, the clinicians should keep this differential diagnosis in mind. Echocardiography and esophagography are useful to ensure the diagnosis promptly.