Bassi Raghav, Saeed Yasir
Internal Medicine, Lincoln Medical and Mental Health Center, New York, USA.
Cureus. 2021 Feb 7;13(2):e13204. doi: 10.7759/cureus.13204.
Achalasia is a relatively rare motor disorder characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter. In only 10% of patients, untreated or poorly managed achalasia can progress to esophageal dilation and eventual loss of total functionality resulting in a characteristic sigmoid dolichomegaesopahagus. In extremely rare instances, this sigmoid dolichomegaesopahagus can present clinically as acute airway obstruction or a fatal, life-threatening hemorrhage requiring immediate intervention. We present the case of a 65-year-old female with a past medical history of long-standing achalasia who had complaints of shortness of breath, chest pain, and two episodes of life-threatening hematemesis requiring a blood transfusion. An angiography illustrated significant distention of the esophagus occupying most of the right hemithorax and non-specific intraluminal fluid with a small amount of gas. Emergent esophagogastroduodenoscopy showed fibrosis and necrosis of the esophageal mucosa with food debris, suggesting that the bleeding was likely coming from an ulcer caused by pressure necrosis. The patient was hemodynamically unstable after the procedure and was transferred to another facility the next day for an esophagectomy. Patients with achalasia have an increased susceptibility to develop pressure ulcers due to increased shear force on the esophageal wall, increased moisture of the esophageal wall from prolonged contact of food boluses, and underlying malnutrition and weight loss from the indigestion of food causing atrophy of the mucosal barriers. The management of these ulcers is to treat and manage the underlying cause. Although there are no curative treatments for achalasia, symptomatic relief through both surgical and medical therapies are the mainstay of management, with an esophagectomy reserved for refractory cases or in patients who develop end-stage complications.
贲门失弛缓症是一种相对罕见的运动障碍性疾病，其特征为食管无蠕动以及食管下括约肌不完全松弛。仅10%的患者中，未经治疗或管理不善的贲门失弛缓症会进展为食管扩张，最终导致完全丧失功能，形成典型的乙状巨食管。在极其罕见的情况下，这种乙状巨食管在临床上可表现为急性气道梗阻或致命的、危及生命的出血，需要立即干预。我们报告一例65岁女性病例，她有长期贲门失弛缓症病史，主诉呼吸急促、胸痛以及两次危及生命的呕血，需要输血治疗。血管造影显示食管显著扩张，占据了大部分右胸腔，管腔内有非特异性液体及少量气体。急诊食管胃十二指肠镜检查显示食管黏膜纤维化和坏死，伴有食物残渣，提示出血可能来自压力性坏死导致的溃疡。术后患者血流动力学不稳定，第二天被转至另一机构进行食管切除术。贲门失弛缓症患者由于食管壁上的剪切力增加、食物团块长时间接触导致食管壁水分增加以及食物消化不良引起黏膜屏障萎缩导致的潜在营养不良和体重减轻，发生压疮的易感性增加。这些溃疡的治疗方法是治疗和处理潜在病因。虽然贲门失弛缓症没有治愈性治疗方法，但通过手术和药物治疗缓解症状是主要的治疗手段，食管切除术仅用于难治性病例或出现终末期并发症的患者。
