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先天性膈疝合并支气管肺隔离症:两例报告及文献复习

Congenital diaphragmatic hernia with associated broncho-pulmonary sequestration: A report of two cases and a literature review.

作者信息

Alhamad Moath, Anand Dhullipala

机构信息

Sidra Medicine, Doha, Qatar.

Weill Cornell Medicine, Doha, Qatar.

出版信息

J Neonatal Perinatal Med. 2024;17(1):123-131. doi: 10.3233/NPM-230042.

Abstract

BACKGROUND

Congenital Diaphragmatic Hernia (CDH) is a severe congenital anomaly with significant morbidity and mortality. It can be isolated or can be associated with other congenital anomalies, including broncho-pulmonary sequestration (BPS). The association of CDH with BPS (CDH+BPS) is uncommon but has been previously reported, and it can complicate the course of the disease in patients with CDH. We report two cases of CDH+BPS that were recently treated at our CDH-Qatar (CDH-Q) program.

METHODS

We reviewed CDH-Q program registry to search for CDH+BPS and extracted the data for the identified cases. We also reviewed the previously published literature available on PubMed for similar cases.

RESULTS

Out of 53 cases of CDH referred to CDH-Q from January 2018 to December 2022, two cases of CDH+BPS were identified, with an estimated prevalence of 3.8% of this association in our CDH population. Both cases were born at term. Case 1 was diagnosed with CDH+BPS postnatally, while case 2 was diagnosed with CDH antenatally but BPS was diagnosed after birth. Both cases underwent a surgical repair of the CDH with resection of the associated BPS, and the histopathology of the resected lung tissue confirmed the presence of BPS in both. Both cases survived to discharge.

CONCLUSION

The association of CDH+BPS is uncommon; however, it can have significant consequences on the management and the prognosis of patients with CDH. Reporting these cases is important to provide a better understanding of this association and its impact on CDH patients.

摘要

背景

先天性膈疝(CDH)是一种严重的先天性异常,具有较高的发病率和死亡率。它可以是孤立性的,也可与其他先天性异常相关,包括支气管肺隔离症(BPS)。CDH与BPS的关联(CDH+BPS)并不常见,但此前已有报道,它会使CDH患者的疾病进程复杂化。我们报告两例最近在我们的卡塔尔先天性膈疝(CDH-Q)项目中接受治疗的CDH+BPS病例。

方法

我们查阅了CDH-Q项目登记册以寻找CDH+BPS病例,并提取了确诊病例的数据。我们还在PubMed上查阅了此前发表的关于类似病例的文献。

结果

在2018年1月至2022年12月转诊至CDH-Q的53例CDH病例中,确诊了2例CDH+BPS病例,在我们的CDH患者群体中,这种关联的估计患病率为3.8%。两例均为足月出生。病例1出生后被诊断为CDH+BPS,而病例2产前被诊断为CDH,但出生后诊断出BPS。两例均接受了CDH手术修复并切除相关的BPS,切除的肺组织病理检查证实两例均存在BPS。两例均存活至出院。

结论

CDH+BPS的关联并不常见;然而,它会对CDH患者的治疗和预后产生重大影响。报告这些病例对于更好地理解这种关联及其对CDH患者的影响很重要。

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