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先天性纵隔神经肠囊肿作为出生后即出现呼吸窘迫的病因。

Congenital Mediastinal Neuroenteric Cyst as a cause of respiratory distress since birth.

作者信息

Gillani Saima, Khan Muhammad Junaid, Akram Faiza, Shahjahan Raabiya

机构信息

Department of Paediatrics, Ayub Teaching Hospital, Abbottabad, Pakistan.

Department of Radiology, Ayub Teaching Hospital, Abbottabad, Pakistan.

出版信息

J Pak Med Assoc. 2022 Nov;72(11):2298-2301. doi: 10.47391/JPMA.3507.

Abstract

Mediastinal Neuroenteric Cyst is a rare congenital presentation in infants and is associated with a high mortality rate. It is a very uncommon benign lesion and usually develops from abnormal embryological development of the foregut. Till now, only 106 cases have been reported worldwide. In Pakistan only three cases have been published, with varying presentations. The clinical presentation and age at presentation vary from asymptomatic and coincidental finding on chest x-ray, to limb numbness or early presentation with severe symptoms like those in our case. In fact, it poses an important challenge for paediatricians. We present a rare case with emphasis on clinical presentation and diagnostic criteria.

摘要

纵隔神经肠囊肿是婴儿中一种罕见的先天性疾病,死亡率很高。它是一种非常罕见的良性病变,通常由前肠胚胎发育异常形成。到目前为止,全球仅报道了106例。在巴基斯坦仅发表了3例,表现各异。临床表现和发病年龄各不相同,从胸部X线检查时无症状的偶然发现,到肢体麻木,或像我们病例中那样早期出现严重症状。事实上,它给儿科医生带来了重大挑战。我们报告一例罕见病例,重点介绍其临床表现和诊断标准。

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