新生儿难治性先天性乳糜胸2例报告并文献复习

doi:10.12372/jcp.2025.24e1306

Abstract

Abstract:

Congenital chylothorax (CC) is the most common cause of pleural effusion during the neonatal period, with a reported perinatal mortality rate ranging from 15% to 57%. When associated with fetal hydrops, this mortality rate can increase dramatically, reaching as high as 98%. Currently, there are no universally accepted standardized treatment guidelines for CC. This study retrospectively reviewed the clinical presentation, diagnosis, treatment, and follow-up outcomes of two neonates diagnosed with refractory congenital chylothorax. Additionally, a comprehensive literature search was conducted using relevant keywords in both domestic and international databases from their inception to September 2024, aiming to summarize the clinical features and recent advances in the management of refractory congenital chylothorax in neonates. Both infants were born as near-term preterm babies and had prenatal ultrasound findings of bilateral pleural effusion. Following birth, they exhibited signs of respiratory distress and cyanosis of the lips, necessitating immediate transfer to the neonatal intensive care unit (NICU). The presence of elevated triglyceride levels in the pleural fluid confirmed the diagnosis of congenital chylothorax. Despite more than four weeks of conservative management—including endotracheal intubation with mechanical ventilation, closed thoracic drainage, dietary modifications, parenteral nutrition, and intravenous octreotide infusion—clinical improvement was not observed. Subsequently, oral propranolol was initiated. In case 1, complete resolution of pleural effusion was achieved before discharge, and no recurrence was observed during follow-up until six months of age. In case 2, the effusion showed partial improvement at discharge. However, after discontinuation of propranolol, recurrence occurred. The infant was managed with close observation, and the effusion resolved spontaneously within one week. No recurrence was noted during follow-up until seven months of age. This study suggests that propranolol may serve as a potentially effective and safe therapeutic option for refractory congenital chylothorax when conventional conservative treatments fail.

Key words: neonate, refractory, congenital chylothorax, propranolol

CLC Number:

ZHENG Ruixue, SUN Xiaodong, WU Guilan, SHEN Leilei. Neonatal refractory congenital chylothorax: two case reports and literature review[J].Journal of Clinical Pediatrics, 2025, 43(10): 775-781.

Figures/Tables 5

Figure 1

Figure 2

Figure 3

Figure 4

Table 1

General status and treatment outcomes of 30 cases of refractory CC"

编号	性别	出生胎龄/ 周	体重/g	产前是否诊断胸腔/腹腔积液	Apgar评分		治疗与结局
编号	性别	出生胎龄/ 周	体重/g	产前是否诊断胸腔/腹腔积液	1min	5min	治疗与结局
患儿1	男	36	3 100	是	7	8	治愈，口服普萘洛尔
患儿2	男	35⁺³	3 460	是	6	8	治愈，口服普萘洛尔
3^[6]	—	34⁺¹	2 047	是	1	3	治愈，口服依替福林
4^[7]	—	36	5 275	是	4	7	治愈，口服米多君
5^[8]	女	33⁺⁶	—	否	—	—	治愈，大剂量奥曲肽（30.0μg·kg^-1·h^-1）
6^[9]	男	37⁺⁴	3 155	是	6	10	治愈，大剂量奥曲肽（20.0μg·kg^-1·h^-1）
7^[10]	女	36	2 700	是	—	—	治愈，化学胸膜固定术（胸腔内注射聚维酮碘）
8^[11]	男	足月	3 290	否	—	—	治愈，胸膜穿刺
9^[11]	女	足月	2 700	否	—	—	治愈，胸膜穿刺
10^[11]	男	36	3 020	否	—	—	治愈，胸膜穿刺
11^[12]	男	36	3 000	否	—	—	死亡（败血症），胸膜穿刺
12^[13]	男	35⁺²	3 040	是	7	8	死亡，胸腔镜下探查术
13^[14]	女	31	—	是	—	—	治愈，化学胸膜固定术（胸腔内注射聚维酮碘）
14^[14]	女	35	2 300	是	—	—	治愈，化学胸膜固定术（胸腔内注射聚维酮碘）
15^[14]	—	36	—	—	—	—	治愈，化学胸膜固定术（胸腔内注射聚维酮碘）
16^[14]	—	36	—	是	—	—	死亡（肾衰竭），化学胸膜固定术（胸腔内注射聚维酮碘）
17^[15]	男	37	3 000	是	8	9	治愈，注射索马托司他丁
18^[15]	女	33	2 510	是	7	9	治愈，注射索马托司他丁
19^[16]	男	40	4 160	是	9	10	治愈，化学胸膜固定术（胸腔内注射红霉素）
20^[16]	女	33	2 430	是	6	8	治愈，化学胸膜固定术（胸腔内注射红霉素）
21^[16]	男	31	2 320	是	5	8	治愈，化学胸膜固定术（胸腔内注射红霉素）
22^[16]	女	35	2 250	否	9	9	治愈，化学胸膜固定术（胸腔内注射红霉素）
23^[16]	女	38	3 430	是	9	10	死亡（因其他疾病），化学胸膜固定术（胸腔内注射红霉素）
24^[16]	男	39	3 750	否	10	10	治愈，化学胸膜固定术（胸腔内注射红霉素）
25^[16]	男	40	3 400	否	10	10	失访（因可疑食管气管瘘），化学胸膜固定术（胸腔内注射红霉素）
26^[16]	女	37	3 570	否	10	10	治愈，淋巴管瘘修补术
27^[16]	男	40	3 320	否	10	10	治愈，淋巴管瘘修补术
28^[16]	女	38	3 350	否	10	10	治愈，化学胸膜固定术（胸腔内注射红霉素）
29^[16]	男	39	2 990	否	10	10	治愈，化学胸膜固定术（胸腔内注射红霉素）
30^[17]	女	36⁺⁵	3 281	是	1	8	治愈，口服西地那非

Table 1

References 34

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Neonatal refractory congenital chylothorax: two case reports and literature review

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