Clinical analysis of neonatal refractory congenital chylothorax

doi:10.3877/cma.j.issn.1673-5250.2021.04.010

Objective

To explore the clinical characteristics and treatment strategies of refractory congenital chylothorax (CC) in neonates.

Methods

Eleven neonates with refractory CC who were hospitalized in Children′s Hospital, Capital Institute of Pediatrics from June 2015 to December 2019 were selected as research subjects. The clinical manifestations, ancillary tests, treatment strategies, regression and follow-up were retrospectively analyzed. The procedures followed in this study were in accordance with the World Medical Association Declaration of Helsinki revised in 2013, and informed consent for clinical study was signed with the guardians of neonates.

Results

①Among the 11 cases of refractory CC, 8 were term infants and 3 were preterm infants, with gestational age ranging from 31 to 40 weeks; 6 were boys and 5 were girls; 4 received prenatal diagnosis and 3 were combined with fetal edema. ② Seven cases developed within 10 min after birth, 11 cases showed tachypnea, weakening of respiratory sound in affected lung, chest X-ray, chest CT or chest ultrasonography all suggested pleural effusion, including 7 cases of bilateral pleural effusion and 4 cases of unilateral pleural effusion (left side), and the pleural effusion test showed celiac fluid. ③ After failure of 2-4 weeks of conservative treatment (dietary modification, respiratory support, thoracic close drainage, octreotide, anti-infective therapy, etc.), 10 cases were treated with chemical pleural fixation (intra-thoracic injection of erythromycin), of which 7 cases were treated with octreotide during the same time, and the number of erythromycin intrathoracic injections was 3-7 for those who did not combine with octreotide. During intrapleural injection of erythromycin, 4 children showed tachycardia and irritability, and 1 patient showed significantly increased blood glucose (18.8 mmol/L). Two cases underwent thoracoscopic exploration and repair of chylous fistula. ④After treatment, 11 cases of refractory CC were absorbed and improved, and the total length of hospital stay was 40-73 days. Among them, 1 case died after automatic discharge, and 1 case was lost to follow-up because of suspected parents of tracheoesophageal fistula. No recurrence occurred after discharge.

Conclusions

Refractory CC is common in full-term children. For children who have failed in early dietary regulation, intravenous nutrition, thoracic close drainage and octreotide intravenous infusion for 2-4 weeks, chemical pleural fixation (intrathoracic injection of erythromycin) can be combined. For patients with ineffective treatment for more than 4 weeks, thoracoscopic exploration can be used to repair lymphatic fistula after identifying the leak in order to improve the cure rate of refractory CC.

Key words: Chylothorax, Injections, intrathoracic, Erythromycin, Octreotide, Microinjections, Surgical procedures, operative, Infant, newborn

表1 11例难治性CC患儿一般临床情况

患儿编号(No.)	性别	出生胎龄(周)	出生体重(kg)	起病时间	入院时日龄(d)	生后Apgar评分(分)		产前诊断胎儿胸腔积液
患儿编号(No.)	性别	出生胎龄(周)	出生体重(kg)	起病时间	入院时日龄(d)	1 min	5 min	产前诊断胎儿胸腔积液
1	男	40	4.16	10 min	51	9	10	是
2	女	33	2.43	10 min	1	6	8	是
3	男	31	2.32	10 min	13	5	8	是
4	女	35	2.25	10 min	33	9	9	否
5	女	38	3.43	10 min	42	9	10	是
6	男	39	3.75	7 d	31	10	10	否
7	男	40	3.40	10 min	10	10	10	否
8	女	37	3.57	10 min	52	10	10	否
9	男	40	3.32	12 d	103	10	10	否
10	女	38	3.35	10 d	90	10	10	否
11	男	39	2.99	12 d	12	10	10	否

表1 11例难治性CC患儿一般临床情况

患儿编号(No.)	性别	出生胎龄(周)	出生体重(kg)	起病时间	入院时日龄(d)	生后Apgar评分(分)		产前诊断胎儿胸腔积液
患儿编号(No.)	性别	出生胎龄(周)	出生体重(kg)	起病时间	入院时日龄(d)	1 min	5 min	产前诊断胎儿胸腔积液
1	男	40	4.16	10 min	51	9	10	是
2	女	33	2.43	10 min	1	6	8	是
3	男	31	2.32	10 min	13	5	8	是
4	女	35	2.25	10 min	33	9	9	否
5	女	38	3.43	10 min	42	9	10	是
6	男	39	3.75	7 d	31	10	10	否
7	男	40	3.40	10 min	10	10	10	否
8	女	37	3.57	10 min	52	10	10	否
9	男	40	3.32	12 d	103	10	10	否
10	女	38	3.35	10 d	90	10	10	否
11	男	39	2.99	12 d	12	10	10	否

表2 11例难治性CC患儿合并症情况

患儿编号(No.)	合并症
1	无
2	胎儿水肿、NRDS、败血症、气胸、动脉导管未闭(4.3 mm)
3	胎儿水肿、NRDS、支气管肺发育不良、气胸
4	弥散性血管内凝血、毛细血管渗漏综合征、NRDS、败血症、休克、脓气胸
5	胎儿水肿、颈璞、肺动脉瓣狭窄、硬膜下积液、房间隔缺损、胆汁淤积症、气胸
6	败血症
7	疑似气管食管瘘、气胸
8	右心增大
9	败血症
10	气胸
11	败血症

表2 11例难治性CC患儿合并症情况

患儿编号(No.)	合并症
1	无
2	胎儿水肿、NRDS、败血症、气胸、动脉导管未闭(4.3 mm)
3	胎儿水肿、NRDS、支气管肺发育不良、气胸
4	弥散性血管内凝血、毛细血管渗漏综合征、NRDS、败血症、休克、脓气胸
5	胎儿水肿、颈璞、肺动脉瓣狭窄、硬膜下积液、房间隔缺损、胆汁淤积症、气胸
6	败血症
7	疑似气管食管瘘、气胸
8	右心增大
9	败血症
10	气胸
11	败血症

表3 11例难治性CC在本院住院时第1次胸腔穿刺液的实验室检查结果

患儿编号(No.)	外观	乳糜试验	胆固醇(mmol/L)	三酰甘油(mmol/ L)	胆固醇比(胸腔穿刺液/血浆)	三酰甘油比(胸腔穿刺液/血液)	白细胞计数(×10⁶/L)	淋巴细胞比例(%)	总蛋白(g/L)
1	橘黄色	阳性	1.73	18.75	0.94	45.70	21 634	94	34
2	淡黄色	阴性	0.89	0.16	0.36	0.19	305	94	23
3	淡黄色	阳性	1.07	0.24	0.46	0.61	5 615	99	29
4	乳白色	阳性	0.54	3.21	0.32	1.96	9 120	90	23
5	橘黄色	—	1.10	2.22	0.54	1.32	6 000	95	27
6	乳白色	—	0.24	9.01	0.10	8.42	13 421	87	35
7	淡黄色	阴性	1.16	0.63	0.43	0.48	2 312	96	38
8	橘黄色	阳性	0.73	7.83	0.53	3.21	11 234	92	32
9	乳白色	阳性	0.45	8.01	0.41	4.35	14 421	94	26
10	乳白色	—	0.64	5.01	0.34	3.12	9 421	95	27
11	乳白色	阳性	0.68	6.32	0.15	7.32	19 226	94	32

表3 11例难治性CC在本院住院时第1次胸腔穿刺液的实验室检查结果

患儿编号(No.)	外观	乳糜试验	胆固醇(mmol/L)	三酰甘油(mmol/ L)	胆固醇比(胸腔穿刺液/血浆)	三酰甘油比(胸腔穿刺液/血液)	白细胞计数(×10⁶/L)	淋巴细胞比例(%)	总蛋白(g/L)
1	橘黄色	阳性	1.73	18.75	0.94	45.70	21 634	94	34
2	淡黄色	阴性	0.89	0.16	0.36	0.19	305	94	23
3	淡黄色	阳性	1.07	0.24	0.46	0.61	5 615	99	29
4	乳白色	阳性	0.54	3.21	0.32	1.96	9 120	90	23
5	橘黄色	—	1.10	2.22	0.54	1.32	6 000	95	27
6	乳白色	—	0.24	9.01	0.10	8.42	13 421	87	35
7	淡黄色	阴性	1.16	0.63	0.43	0.48	2 312	96	38
8	橘黄色	阳性	0.73	7.83	0.53	3.21	11 234	92	32
9	乳白色	阳性	0.45	8.01	0.41	4.35	14 421	94	26
10	乳白色	—	0.64	5.01	0.34	3.12	9 421	95	27
11	乳白色	阳性	0.68	6.32	0.15	7.32	19 226	94	32

表4 11例难治性CC患儿治疗情况

患儿编号(No.)	胸腔积液	禁食天数(d)	MCT含量	治疗时间(d，呼吸支持治疗方式)	胸腔引流时间(d，引流部位)	引流量[mL/(kg·d)]
1	宫内双侧，生后为左侧	12	40% → 87%	吸氧	30(左侧)	50
2	左侧，复发后转为双侧	10	50%→60%~90% ^a	38(有创)	45(左侧)，15(右侧)	50~90
3	双侧，以左侧为主	12	87%	34(有创)	33(左侧)	50~100
4	双侧	38	40%	21(有创)	62(双侧)	60
5	左侧，复发后转为双侧	27	50%→60%~90% ^a	4(有创)	54(左侧)，10(右侧)	60
6	左侧，复发后转为双侧，以右侧为主	14	50%	—	21(左侧)，5(右侧)	50~65
7	左侧，复发转为双侧，右侧少量	20	40%	5(有创)	21(左侧)	50~60
8	左侧	35	40%	5(无创)	28(左侧)	80
9	左侧	24	40%	10(无创)	35(左侧)	50~80
10	双侧，以左侧为主	12	50%	10(无创)	31(左侧)	80
11	左侧	14	60%~90% ^a	吸氧	21(左侧)	50~60
患儿编号(No.)	奥曲肽		红霉素胸腔内注射		手术	住院天数(d)	转归
患儿编号(No.)	剂量[μg/(kg·h)]	治疗天数(d)	剂量(mg/kg)	胸腔部位×次数(次)	手术	住院天数(d)	转归
1	1~8	28	25	左侧×1	—	53	治愈
2	1~10	30	25	左侧×5	—	50	出院后2个月吸收
3	—	—	25	左侧×7	—	58	出院后1个月吸收
4	1~7	14	25	双侧×6	—	63	出院后5个月吸收
5	1~9	32	25	左侧×3，右侧×5	—	73	好转后自动出院，死于其他疾病
6	—	—	25	左侧×7，右侧×3	—	44	出院后1个月吸收
7	—	—	25	左侧×7	—	42	好转后因可疑气管食管瘘自动出院，失访
8	8	14	—	—	+^b	51	术后14 d出院，出院后1个月吸收
9	6	19	30	左侧×7	+^b	46	术后14 d出院，出院后1个月吸收
10	1~8	14	30	左侧×3	—	42	出院后2个月吸收
11	1~8	14	30	左侧×4	—	40	出院后2个月吸收

表4 11例难治性CC患儿治疗情况

患儿编号(No.)	胸腔积液	禁食天数(d)	MCT含量	治疗时间(d，呼吸支持治疗方式)	胸腔引流时间(d，引流部位)	引流量[mL/(kg·d)]
1	宫内双侧，生后为左侧	12	40% → 87%	吸氧	30(左侧)	50
2	左侧，复发后转为双侧	10	50%→60%~90% ^a	38(有创)	45(左侧)，15(右侧)	50~90
3	双侧，以左侧为主	12	87%	34(有创)	33(左侧)	50~100
4	双侧	38	40%	21(有创)	62(双侧)	60
5	左侧，复发后转为双侧	27	50%→60%~90% ^a	4(有创)	54(左侧)，10(右侧)	60
6	左侧，复发后转为双侧，以右侧为主	14	50%	—	21(左侧)，5(右侧)	50~65
7	左侧，复发转为双侧，右侧少量	20	40%	5(有创)	21(左侧)	50~60
8	左侧	35	40%	5(无创)	28(左侧)	80
9	左侧	24	40%	10(无创)	35(左侧)	50~80
10	双侧，以左侧为主	12	50%	10(无创)	31(左侧)	80
11	左侧	14	60%~90% ^a	吸氧	21(左侧)	50~60
患儿编号(No.)	奥曲肽		红霉素胸腔内注射		手术	住院天数(d)	转归
患儿编号(No.)	剂量[μg/(kg·h)]	治疗天数(d)	剂量(mg/kg)	胸腔部位×次数(次)	手术	住院天数(d)	转归
1	1~8	28	25	左侧×1	—	53	治愈
2	1~10	30	25	左侧×5	—	50	出院后2个月吸收
3	—	—	25	左侧×7	—	58	出院后1个月吸收
4	1~7	14	25	双侧×6	—	63	出院后5个月吸收
5	1~9	32	25	左侧×3，右侧×5	—	73	好转后自动出院，死于其他疾病
6	—	—	25	左侧×7，右侧×3	—	44	出院后1个月吸收
7	—	—	25	左侧×7	—	42	好转后因可疑气管食管瘘自动出院，失访
8	8	14	—	—	+^b	51	术后14 d出院，出院后1个月吸收
9	6	19	30	左侧×7	+^b	46	术后14 d出院，出院后1个月吸收
10	1~8	14	30	左侧×3	—	42	出院后2个月吸收
11	1~8	14	30	左侧×4	—	40	出院后2个月吸收

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