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现代对假性贲门失弛缓症的病理生理学和治疗的认识。

Modern insights into the pathophysiology and treatment of pseudoachalasia.

机构信息

Department of Surgery, Escola Paulista de Medicina, Federal University of São Paulo, Rua Diogo de Faria 1087 Cj 301, São Paulo, 04037-003, Brazil.

Department of Surgery, University of South Florida, Tampa, USA.

出版信息

Langenbecks Arch Surg. 2024 Feb 17;409(1):65. doi: 10.1007/s00423-024-03259-2.

Abstract

BACKGROUND

Secondary achalasia or pseudoachalasia is a clinical presentation undistinguishable from achalasia in terms of symptoms, manometric, and radiographic findings, but associated with different and identifiable underlying causes.

METHODS

A literature review was conducted on the PubMed database restricting results to the English language. Key terms used were "achalasia-like" with 63 results, "secondary achalasia" with 69 results, and "pseudoachalasia" with 141 results. References of the retrieved papers were also manually reviewed.

RESULTS

Etiology, diagnosis, and treatment were reviewed.

CONCLUSIONS

Pseudoachalasia is a rare disease. Most available evidence regarding this condition is based on case reports or small retrospective series. There are different causes but all culminating in outflow obstruction. Clinical presentation and image and functional tests overlap with primary achalasia or are inaccurate, thus the identification of secondary achalasia can be delayed. Inadequate diagnosis leads to futile therapies and could worsen prognosis, especially in neoplastic disease. Routine screening is not justifiable; good clinical judgment still remains the best tool. Therapy should be aimed at etiology. Even though Heller's myotomy brings the best results in non-malignant cases, good clinical judgment still remains the best tool as well.

摘要

背景

继发性贲门失弛缓症或假性贲门失弛缓症在症状、测压和影像学表现方面与贲门失弛缓症无明显区别,但与不同且可识别的潜在病因相关。

方法

在 PubMed 数据库中进行文献回顾,限制结果为英文。使用的关键词为“贲门失弛缓症样”,有 63 个结果;“继发性贲门失弛缓症”,有 69 个结果;“假性贲门失弛缓症”,有 141 个结果。还手动审查了检索到的论文的参考文献。

结果

对病因、诊断和治疗进行了回顾。

结论

假性贲门失弛缓症是一种罕见的疾病。关于这种疾病的大多数可用证据都是基于病例报告或小的回顾性系列研究。虽然有不同的病因,但都导致流出道梗阻。临床表现、影像学和功能检查与原发性贲门失弛缓症重叠或不准确,因此可能会延迟继发性贲门失弛缓症的诊断。常规筛查没有道理;良好的临床判断仍然是最好的工具。治疗应针对病因。即使非恶性病例中 Heller 肌切开术带来的效果最好,但良好的临床判断仍然是最好的工具。

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