Clinical treatment strategies of children with severe congenital diaphragmatic hernia

doi:10.3877/cma.j.issn.1673-5250.2021.03.014

Objective

To explore clinical treatment strategies of children with severe congenital diaphragmatic hernia (CDH).

Methods

A total of 7 cases of children with severe CDH who were treated in Northwest Women′s and Children′s Hospital from January 2015 to October 2019 were selected as research subjects. Their general clinical data, treatment outcomes, prognosis and postoperative follow-up results were collected and analyzed by retrospective method. This study met the requirements of World Medical Association Declaration of Helsinki revised in 2013.

Results

The analysis results of clinical data of 7 children with severe CDH in this study were as follows. ①General clinical data: 4 cases of male children (No.2, 5-7) and 3 cases of female children (No.1, 3-4) respectively. Among them, 1 case (No. 4) was a full-term infant with low birth weight, 1 case (No. 7) was a full-term infant with low birth weight and multisystem malformations, and 5 cases (No.1-3, 5-6) were premature infants with low birth weight. 2 cases (No.2, 7) were infants born with assisted reproductive technology and complicated with chromosomal abnormalities. Delivery mode of pregnant mothers: 5 cases (No. 1-4, 7) were delivered by cesarean section, and 2 cases (No. 5-6) had natural birth. 4 cases of pregnant mothers (No. 1-2, 6-7) had abortion history. ②Treatment effects: except for the No.6 child with 10 scores of Apgar score at 1, 5, and 10 min after birth, the other 6 cases received tracheal intubation and high frequency oscillatory ventilation (HFOV) in the operating room or delivery room, and also received fasting and gastrointestinal decompression therapy. Due to multiple complex malformations, the guardian of No.1 child gave up treatment. The other 6 children were treated with CDH repair surgery, 3 cases (No.4-6) were operated through the left posterolateral thoracic incision, and 3 cases (No. 2-3, 7) were operated through the left upper abdominal incision. ③Prognosis: 4 cases had a poor prognosis during perioperative period, of which 1 case (No. 1) died after giving up treatment before operation, and 3 cases (No. 3-4, 7) died after operation. Among them, except for No.4 child, No.1, 3, and 7 children have complicated congenital heart disease and other system malformations. The other 3 children (No. 2, 5-6) were cured and discharged from hospital after surgery, and they all had premature delivery, low birth weight, and other uncomplicated deformities. Postoperative follow-up results of these 3 cured children showed that 1 case (No. 2) had CDH recurrence in 1 month after operation, and after receiving CDH repair surgery again, the diaphragm was intact and without recurrence of CDH at the end of follow-up. And No. 5-6 children grew well at the end of follow-up.

Conclusions

Complication of congenital heart disease may be one of the main factors affecting the prognosis of children with severe CDH. As to children with severe CDH, treatment strategies such as immediate tracheal intubation, HFOV, and delayed surgical repair (DSR) after delivery may be helpful to improve the surgical cure rate of them.

Key words: Hernia, diaphragmatic, Intubation, intratracheal, High-frequency ventilation, Congenital diaphragmatic hernia, High-frequency oscillatory ventilation, Delayed surgical repair, Child

表1 本组7例重症先天性膈疝患儿临床资料

患儿(No.)	性别	出生体重(g)	出生胎龄(周)	发现CDH时胎龄(周)	LHR	合并畸形	出生后1、5 min Apgar评分(分)	通气时间(h)		疝入脏器	转归
患儿(No.)	性别	出生体重(g)	出生胎龄(周)	发现CDH时胎龄(周)	LHR	合并畸形	出生后1、5 min Apgar评分(分)	术前	术后	疝入脏器	转归
1	女	1 620	36⁺⁵	20	0.9	复杂先天性心脏病，小脑蚓发育不良	7、9	120	0	—^b	死亡
2	男	1 670	36	30	2.4	染色体异常，遗传性呼吸道纤毛运动障碍	7、8	60	74	胃、结肠、小肠、脾	治愈
3	女	1 900	34	25⁺²	1.7	复杂先天性心脏病，股骨骨折	4、7	12	260	肝左叶、胃、小肠、脾	死亡
4	女	2 340	38⁺³	33	—^a	无	8、9	25	12	小肠、脾、结肠	死亡
5	男	2 450	34⁺²	32	1.8	肠旋转不良	9、9	72	120	小肠、结肠、脾	治愈
6	男	2 460	36⁺³	26	3.6	右手拇指畸形，右侧睾丸重度发育不良	10、10	0	120	胃、肠管	治愈
7	男	2 470	39⁺³	23	1.4	染色体异常，复杂先天性心脏病，多发运动系统畸形	7、8	103	480	胃、小肠、结肠、脾	死亡

表1 本组7例重症先天性膈疝患儿临床资料

患儿(No.)	性别	出生体重(g)	出生胎龄(周)	发现CDH时胎龄(周)	LHR	合并畸形	出生后1、5 min Apgar评分(分)	通气时间(h)		疝入脏器	转归
患儿(No.)	性别	出生体重(g)	出生胎龄(周)	发现CDH时胎龄(周)	LHR	合并畸形	出生后1、5 min Apgar评分(分)	术前	术后	疝入脏器	转归
1	女	1 620	36⁺⁵	20	0.9	复杂先天性心脏病，小脑蚓发育不良	7、9	120	0	—^b	死亡
2	男	1 670	36	30	2.4	染色体异常，遗传性呼吸道纤毛运动障碍	7、8	60	74	胃、结肠、小肠、脾	治愈
3	女	1 900	34	25⁺²	1.7	复杂先天性心脏病，股骨骨折	4、7	12	260	肝左叶、胃、小肠、脾	死亡
4	女	2 340	38⁺³	33	—^a	无	8、9	25	12	小肠、脾、结肠	死亡
5	男	2 450	34⁺²	32	1.8	肠旋转不良	9、9	72	120	小肠、结肠、脾	治愈
6	男	2 460	36⁺³	26	3.6	右手拇指畸形，右侧睾丸重度发育不良	10、10	0	120	胃、肠管	治愈
7	男	2 470	39⁺³	23	1.4	染色体异常，复杂先天性心脏病，多发运动系统畸形	7、8	103	480	胃、小肠、结肠、脾	死亡

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