Double H-type congenital tracheoesophageal fistulas: two cases report and literature review

doi:10.3877/cma.j.issn.1673-5250.2024.02.013

Objective

To investigate the clinical characteristics of children with double H-type congenital tracheoesophageal fistulas (DCTEFs).

Methods

Two cases of DCTEFs (patient 1 and 2)treated at Xi′an Children′s Hospital were selected in this study. A retrospective analysis was conducted to examine the clinical data, diagnostic methods, and treatment plans for these two patients. Literature searches were performed in Chinese databases such as CNKI and Wanfang using Chinese keywords including " tracheoesophageal fistula" " H-type tracheoesophageal fistula" " double tracheoesophageal fistula" " double H-type tracheoesophageal fistula" " V-type esophageal atresia" " simple tracheoesophageal fistula" and " esophageal tracheal fistula". Additionally, English databases in PubMed and Embase were searched using English keywords such as " tracheoesophageal fistula" " H-type tracheoesophageal fistula" " double H-type tracheoesophageal fistula" " double tracheoesophageal fistula" and " V-type esophageal atresia". The search period was set from January 1952 to November 2022.

Results

① Patient 1 (4 months and 2 days old) was admitted to our hospital on August 29, 2018, with symptoms of choking on milk, vomiting, and recurrent respiratory infections after birth. Patient 2 (2 years and 11 months old) was admitted on March 24, 2020, with symptoms of choking cough, vomiting, and recurrent respiratory infections after birth. Both patients underwent bronchoscopy and were found to have two H-type tracheoesophageal fistulas. They both underwent endoscopic tracheoesophageal fistula closure (TFC), with argon beam coagulation of the mucosa around the inner fistula of the esophagus. Patient 1 was cured, while patient 2 experienced a recurrence 8 months after surgery. ② According to the search strategies established in this study, a total of 10 relevant articles were retrieved, involving 10 cases with DCTEFs. Among them, 7 patients (patients 4, 6-10, 12) had relatively complete clinical case data, all showing respiratory and gastrointestinal symptoms, such as cyanosis after feeding, choking cough, vomiting, or abdominal distension. One patient (patient 12) had a history of recurrent respiratory infections. Among the 10 patients, only one patient (patient 5) had two fistulas revealed by esophagography, three patients (patient 7, 9, 12) were found to have two fistulas by bronchoscopy, one patient (patient 11) was found to have two fistulas by combined bronchoscopy and gastroscopy, one patient (patient 3) had two fistulas discovered at autopsy, one patient (patient 4) had the second fistula discovered at autopsy, and three patients (patients 6, 8, 10) had the second fistula discovered during or after surgery. Four patients (patients 6-8, 10) were cured by open surgery, and one patient (patient 12) was cured by thoracoscopic surgery.

Conclusions

DCTEFs is a rare disease with nonspecific clinical manifestations, prone to missed diagnosis. Careful search for the second fistula during examination is essential, and once diagnosed, surgical treatment should be performed promptly.

Key words: Tracheoesophageal fistula, congenital, Double H-type tracheoesophageal fistula, Bronchoscopy, Tracheoesophageal fistula closure, Child

图1　DCTEFs患儿1(男性，4个月2 d龄)支气管镜检查结果[图1A、1B：声门下气管上段膜部偏右侧可见2个瘘口(C8～T1)，右主支气管狭窄，镜体不能通过，左主支气管稍狭窄(黑色箭头所示)]　图2　DCTEFs患儿2(女性，2岁11个月龄)支气管镜检查结果[气管上段可见2个瘘口(C8～T1)，瘘口内可见较多分泌物涌出(黑色箭头所示)]注：DCTEFs为双H型先天性气管食管瘘

表1 文献检索涉及的10例DCTEFs患儿及2例本研究纳入的DCTEFs患儿的临床特征比较

患儿编号	文献(第1作者，发表年)	性别	诊断年龄	临床表现	诊断			治疗方式及定位	预后及转归
患儿编号	文献(第1作者，发表年)	性别	诊断年龄	临床表现	影像学	食管造影	支气管镜	治疗方式及定位	预后及转归
1	本研究	男	4个月2 d	呛奶、吐奶	胸部常规CT提示双肺内散在斑片影及实变	－	2个瘘口位于声门下气管上段膜部偏右侧	内镜下TFC	治愈
2	本研究	女	2岁11个月	呛咳、呕吐	胸部＋喉部常规CT及三维重建提示右肺上叶少许炎症	－	2个瘘口位于气管上段	内镜下TFC(无定位)	术后8个月余瘘复发
3	Leven等^[7]，　1952	女	死亡后	－	－	－	－	－	死亡
4	Babbitt等^[8]，1957	男	死亡后	进食后发绀、呕吐、腹胀	胸部X射线摄片示右肺中上叶无菌性肺炎	颈部可见1个瘘口	1个直径为3 mm瘘口位于气管下1/3处	开胸TFC(无定位)	死亡
5	Benjamin等^[9]，　1991	－	－	－	－	2个瘘口	－	－	－
6	Schulte等^[10]，　2009	男	生后20 d	呕吐、呛咳	胸部X射线摄片示右上肺叶炎症浸润	T2下方1个瘘口	1个瘘口位于距隆突上方1.5 cm处	开胸TFC远端瘘管＋瘘管切断缝合(支气管镜)	治愈
7	Mattei等^[11]，　2012	男	新生儿期	呛咳	胸部X射线摄片未见明显异常	胸内TEF	远、近端瘘口分别位于距隆突上方2 cm与气管上段	开胸TFC＋经右颈部TFC＋瘘管切断缝合(Fogarty气囊导管)	治愈
8	Oosthuizen等^[12]，　2014	女	生后18 d	呼吸困难	胸部X射线摄片提示左下肺不张	阴性	1个瘘口位于气管下段	开胸TFC远端瘘管＋单纯结扎(支气管镜)	治愈
9	Carolis等^[13]，2016	－	－	呼吸困难、发绀、咳嗽	胸部常规CT示右肺散在斑片影、实变	－	2个瘘口位于颈段气管内	－	－
10	Sharman等^[14]，2020	女	生后14 d	体重不增、发绀	－	1个瘘口	阴性	经右颈部入TFC＋瘘管切断缝合＋填充组织(支气管镜)	治愈
11	Matthew等^[15]，2021	男	13岁	－	－	－	联合胃镜发现2个瘘口	开胸TFC(无定位)	－
12	赵家伟等^[16]，　2022	女	4个月17 d	呛咳、腹胀	胸部CT及气管重建示T3水平气管后壁与食管前壁交通	T3水平可见1个瘘口	远、近端瘘口分别位于隆突上方1 cm与1.5 cm	胸腔镜TFC＋瘘管切断缝合(支气管镜导丝)	治愈

表1 文献检索涉及的10例DCTEFs患儿及2例本研究纳入的DCTEFs患儿的临床特征比较

患儿编号	文献(第1作者，发表年)	性别	诊断年龄	临床表现	诊断			治疗方式及定位	预后及转归
患儿编号	文献(第1作者，发表年)	性别	诊断年龄	临床表现	影像学	食管造影	支气管镜	治疗方式及定位	预后及转归
1	本研究	男	4个月2 d	呛奶、吐奶	胸部常规CT提示双肺内散在斑片影及实变	－	2个瘘口位于声门下气管上段膜部偏右侧	内镜下TFC	治愈
2	本研究	女	2岁11个月	呛咳、呕吐	胸部＋喉部常规CT及三维重建提示右肺上叶少许炎症	－	2个瘘口位于气管上段	内镜下TFC(无定位)	术后8个月余瘘复发
3	Leven等^[7]，　1952	女	死亡后	－	－	－	－	－	死亡
4	Babbitt等^[8]，1957	男	死亡后	进食后发绀、呕吐、腹胀	胸部X射线摄片示右肺中上叶无菌性肺炎	颈部可见1个瘘口	1个直径为3 mm瘘口位于气管下1/3处	开胸TFC(无定位)	死亡
5	Benjamin等^[9]，　1991	－	－	－	－	2个瘘口	－	－	－
6	Schulte等^[10]，　2009	男	生后20 d	呕吐、呛咳	胸部X射线摄片示右上肺叶炎症浸润	T2下方1个瘘口	1个瘘口位于距隆突上方1.5 cm处	开胸TFC远端瘘管＋瘘管切断缝合(支气管镜)	治愈
7	Mattei等^[11]，　2012	男	新生儿期	呛咳	胸部X射线摄片未见明显异常	胸内TEF	远、近端瘘口分别位于距隆突上方2 cm与气管上段	开胸TFC＋经右颈部TFC＋瘘管切断缝合(Fogarty气囊导管)	治愈
8	Oosthuizen等^[12]，　2014	女	生后18 d	呼吸困难	胸部X射线摄片提示左下肺不张	阴性	1个瘘口位于气管下段	开胸TFC远端瘘管＋单纯结扎(支气管镜)	治愈
9	Carolis等^[13]，2016	－	－	呼吸困难、发绀、咳嗽	胸部常规CT示右肺散在斑片影、实变	－	2个瘘口位于颈段气管内	－	－
10	Sharman等^[14]，2020	女	生后14 d	体重不增、发绀	－	1个瘘口	阴性	经右颈部入TFC＋瘘管切断缝合＋填充组织(支气管镜)	治愈
11	Matthew等^[15]，2021	男	13岁	－	－	－	联合胃镜发现2个瘘口	开胸TFC(无定位)	－
12	赵家伟等^[16]，　2022	女	4个月17 d	呛咳、腹胀	胸部CT及气管重建示T3水平气管后壁与食管前壁交通	T3水平可见1个瘘口	远、近端瘘口分别位于隆突上方1 cm与1.5 cm	胸腔镜TFC＋瘘管切断缝合(支气管镜导丝)	治愈

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