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迟发性先天性膈疝

Late-presenting congenital diaphragmatic hernia.

作者信息

Hamid Raashid, Baba Aejaz A, Shera Altaf H, Wani Sajad A, Altaf Tahleel, Kant Mohd H

机构信息

Department of Paediatric and Neonatal Surgery, SKIMS, Srinagar, Jammu and Kashmir, India.

出版信息

Afr J Paediatr Surg. 2014 Apr-Jun;11(2):119-23. doi: 10.4103/0189-6725.132799.

Abstract

BACKGROUND

This study was undertaken to highlight the clinical profile, misdiagnosis, surgical treatment,and prognosis of late-presenting congenital diaphragmatic hernia (CDH) cases in a tertiary level hospital.

PATIENTS AND METHODS

This retrospective study included all the babies and children >1 month of age with CDH who were admitted in our Hospital (Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India) during the period between January 2008 and December 2013. Babies with age <1 month were excluded from the study. Data regarding clinical profile, operative records, and follow-up was reviewed and analysed statistically.

RESULTS

A total of 20 patients were included in this study. The clinical picture ranged from respiratory distress (13 patients) to non-specific gastrointestinal complaints (5 patients). In two patients, CDH was misdiagnosed as pneumothorax and had got chest tube inserted in other hospitals before referral to this tertiary care centre. In 14 patients chest, X-ray revealed the diagnosis of CDH and in remaining five patients (including the two patients with misdiagnosis) further investigations were undertaken to establish the diagnosis. Age ranged from 45 days to 17 years with an average age of 58.9 months. There were 12 male and 8 female patients. In all the 20 patients, surgical procedures were undertaken with the retrieval of herniated contents from the thoracic cavity and repair of the diaphragmatic defect. There was no mortality in our series. All the 20 patients were followed-up for a period ranging from 6 months to 5 years (median 3.1 years).

CONCLUSIONS

Late-presenting CDH can have diverse clinical presentation. Late diagnosis and misdiagnosis can result in significant morbidity and potential mortality if these cases are not managed properly at an appropriate stage. Outcome is favourable if these patients are expeditiously identified and surgically repaired.

摘要

背景

本研究旨在突出三级医院中迟发性先天性膈疝(CDH)病例的临床特征、误诊情况、手术治疗及预后。

患者与方法

这项回顾性研究纳入了2008年1月至2013年12月期间在我院(印度克什米尔斯利那加的谢里 - 克什米尔医学科学研究所)收治的所有年龄大于1个月的CDH患儿。年龄小于1个月的婴儿被排除在研究之外。对临床特征、手术记录及随访数据进行回顾并进行统计学分析。

结果

本研究共纳入20例患者。临床表现从呼吸窘迫(13例患者)到非特异性胃肠道症状(5例患者)不等。2例患者的CDH被误诊为气胸,在转诊至该三级医疗中心之前已在其他医院插入胸管。14例患者胸部X线检查确诊为CDH,其余5例患者(包括2例误诊患者)进行了进一步检查以明确诊断。年龄范围为45天至17岁,平均年龄为58.9个月。男性12例,女性8例。所有20例患者均接受了手术,从胸腔内回纳疝入内容物并修复膈肌缺损。本系列中无死亡病例。所有20例患者均接受了6个月至5年(中位时间3.1年)的随访。

结论

迟发性CDH可表现出多样的临床表现。如果这些病例在适当阶段未得到妥善处理,晚期诊断和误诊可能导致严重的发病率和潜在死亡率。如果这些患者能被迅速识别并接受手术修复,预后良好。

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