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贲门失弛缓症相关的肠系膜上动脉综合征伴巨大胃扩张

Achalasia-Associated Superior Mesenteric Artery Syndrome with Massive Gastric Dilatation.

作者信息

Gashi Zaim, Shabani Leutrim, Shatrolli Endrit

机构信息

Gastroenterology clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo.

Radiology clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo.

出版信息

Eur J Case Rep Intern Med. 2025 May 20;12(6):005461. doi: 10.12890/2025_005461. eCollection 2025.

DOI:10.12890/2025_005461
PMID:40502947
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12151572/
Abstract

BACKGROUND

Achalasia and superior mesenteric artery (SMA) syndrome are both uncommon gastrointestinal disorders. When they occur together-a rare clinical scenario-they can cause serious complications, including severe gastric distension.

CASE PRESENTATION

We report the case of a 62-year-old man with a known history of achalasia, previously managed with botulinum toxin injections. He presented with progressively worsening abdominal bloating, unintentional weight loss, and abdominal discomfort. Endoscopy revealed a strikingly dilated oesophagus and stomach, filled with nearly 9 litres of fluid. Further imaging with computed tomography angiography showed a narrowed aortomesenteric angle measuring just 16.7°, consistent with a diagnosis of SMA syndrome. Remarkably, despite the massive distension, no perforation was identified.

DISCUSSION

This case highlights a rare but important overlap between achalasia and SMA syndrome, leading to extreme gastric dilatation in the absence of rupture. Despite the extreme degree of gastric dilatation observed on both endoscopy and imaging, the stomach wall remained intact, and there were no signs of ischemia or perforation. Although SMA syndrome may occur secondary to other health conditions, this case underscores how variable and potentially dangerous this dual pathology can be.

CONCLUSION

Clinicians should remain vigilant for the possibility of SMA syndrome in patients with achalasia who develop new or worsening upper gastrointestinal symptoms, particularly if there is significant gastric dilatation. Early imaging and recognition can be crucial in preventing severe outcomes like perforation.

LEARNING POINTS

The patient has a very rare combination of achalasia and superior mesenteric artery syndrome, presenting with massive dilatation of the stomach with no perforation.Early detection through computed tomography angiography and early decompression are essential to prevent potentially fatal complications, including gastric rupture.This case adds to the limited literature describing achalasia-superior mesenteric artery overlap and reinforces the need for a high degree of clinical suspicion when symptoms diverge from baseline.

摘要

背景

贲门失弛缓症和肠系膜上动脉(SMA)综合征均为罕见的胃肠道疾病。当它们同时出现——这是一种罕见的临床情况——可能会导致严重并发症,包括严重的胃扩张。

病例报告

我们报告一例62岁男性,有贲门失弛缓症病史,此前接受过肉毒杆菌毒素注射治疗。他出现进行性加重的腹胀、非故意体重减轻和腹部不适。内镜检查显示食管和胃显著扩张,充满近9升液体。计算机断层血管造影进一步成像显示主动脉肠系膜角狭窄,仅为16.7°,符合SMA综合征的诊断。值得注意的是,尽管有大量扩张,但未发现穿孔。

讨论

该病例突出了贲门失弛缓症和SMA综合征之间罕见但重要的重叠,导致在无破裂的情况下出现极度胃扩张。尽管在内镜检查和成像中观察到胃扩张程度极高,但胃壁保持完整,没有缺血或穿孔迹象。虽然SMA综合征可能继发于其他健康状况,但该病例强调了这种双重病理情况的多变性和潜在危险性。

结论

对于出现新的或加重的上消化道症状的贲门失弛缓症患者,临床医生应警惕SMA综合征的可能性,特别是在有明显胃扩张的情况下。早期成像和识别对于预防穿孔等严重后果可能至关重要。

学习要点

该患者患有非常罕见的贲门失弛缓症和肠系膜上动脉综合征组合,表现为胃大量扩张且无穿孔。通过计算机断层血管造影进行早期检测和早期减压对于预防包括胃破裂在内的潜在致命并发症至关重要。该病例补充了描述贲门失弛缓症 - 肠系膜上动脉重叠的有限文献,并强化了在症状偏离基线时需要高度临床怀疑的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/4d249ab491c0/5461_Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/ebc7010e8a3e/5461_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/558dfb2d2fac/5461_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/21dbd8a4baf2/5461_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/4d249ab491c0/5461_Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/ebc7010e8a3e/5461_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/558dfb2d2fac/5461_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/21dbd8a4baf2/5461_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2bf/12151572/4d249ab491c0/5461_Fig4.jpg

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本文引用的文献

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Unusual cause of intra-abdominal perforation.腹腔内穿孔的罕见病因。
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