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

[1]	Resch B, Sever Yildiz G, Reiterer F. Congenital chylothorax of the newborn: a systematic analysis of published cases between 1990 and 2018[J]. Respiration, 2022, 101(1): 84-96.
[2]	Attar MA, Donn SM. Congenital chylothorax[J]. Semin Fetal Neonatal Med., 2017, 22(4): 234-239. doi: S1744-165X(17)30040-9 pmid: 28351595
[3]	Bellini C, Boccardo F, Bellini T. Congenital chylothorax of the newborn[J]. Respiration, 2022, 101(8): 793-794.
[4]	Shao XM, Ye HM, Qiu XS. Practical neonatology[M]. People's Medical Publishing House, 2011.
[5]	Kasdallah N, Kbaier H, Salem B, et al. Povidone iodine pleurodesis for refractory congenital chylothorax: a review of literature[J]. Tunis Med, 2016, 94(12): 834.
[6]	Tomobe Y, Mizuguchi U, Shimotakahara A, et al. Combination therapy with etilefrine and pleurodesis for refractory congenital chylothorax[J]. Biomedicine hub, 2020, 5(3): 1-5.
[7]	Tamaoka S, Osada A, Kin T, et al. Midodrine, an oral alpha-1 adrenoreceptor agonist, successfully treated refractory congenital chylous pleural effusion and ascites in a neonate[J]. Chest, 2021, 159(4): e189-e191.
[8]	Sahoo T, Mangla MK, Sethi A, et al. Successful treatment of congenital chylothorax with skimmed milk and long course octreotide[J]. BMJ Case Reports CP, 2018, 11(1): bcr-2018-226347.
[9]	Vass G, Evans Fry R, Roehr CC. Should newborns with refractory chylothorax be tried on higher dose of octreotide?[J]. Neonatology, 2021, 118(1): 122-126.
[10]	Hmami F, Oulmaati A, Bouchikhi C, et al. Congenital chylothorax: rapid and complete response to polyvidone iodine[J]. Arch Pediatr, 2014, 21(9): 1002-1005.
[11]	Altuncu E, Akman İ, Kiyan G, et al. Report of three cases: congenital chylothorax and treatment modalities[J]. Turk J Pediatr, 2007, 49(4): 418-421. pmid: 18246745
[12]	Salem P. A neonate with bilateral refractory chylothorax[J]. Med J Malaysia, 1991, 46(4): 376-378. pmid: 1840449
[13]	Lyu M, Liao ZC, Yu XH, et al. Fetal pleural effusion in the uterus and dyspnea after birth[J]. Zhongguo Dangdai Erke Zazhi, 2020, 22(8): 892-896.
[14]	Brissaud O, Desfrere L, Mohsen R, et al. Congenital idiopathic chylothorax in neonates: chemical pleurodesis with povidone-iodine (Betadine)[J]. Arch Dis Child Fetal Neonatal Ed, 2003, 88(6): F531-F533.
[15]	González Santacruz M, Tarazona Fargueta JL, Muñoz Alvarez P, et al. Use of somatostatin in five neonates with chylothorax[J]. An Pediatr (Barc), 2005, 63(3): 244-248.
[16]	华敏敏, 夏磊, 霍婉莹, 等. 新生儿先天性乳糜胸的临床及预后影响因素分析[J]. 临床儿科杂志, 2023, 41(1): 25-29.
	Hua MM, Xia Lei, Huo WY, et al. Analysis of clinical and prognostic factors of neonatal congenital chylothorax[J]. Linchuang Erke Zazhi, 2023, 41(1): 25-29.
[17]	Malleske DT, Yoder BA. Congenital chylothorax treated with oral sildenafil: a case report and review of the literature[J]. J Perinatol, 2015, 35(5): 384-386. doi: 10.1038/jp.2015.10 pmid: 25917021
[18]	Yang C, Peng J, Chi J, et al. Comparison of the clinical characteristics of congenital chylothorax in preterm and term infants[J]. Chinese Journal of Neonatology, 2021: 12-16.
[19]	Itkin M, Chidekel A, Ryan KA, et al. Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: diagnosis and treatment[J]. Paediatr Respir Rev, 2020, 36: 15-24. doi: 10.1016/j.prrv.2020.07.001 pmid: 32792289
[20]	王斯瑶, 李娟. 新生儿淋巴管发育异常研究进展[J]. 国际儿科学杂志, 2019, 46(1): 48-52.
	Wang SY, Li J. Research progress on neonatal lymphatic dysplasia[J]. Guoji Erkexue Zazhi, 2019, 46 (1): 48-52.
[21]	Pérez-Pérez A, Vigil-Vázquez S, Gutiérrez-Vélez A, et al. Chylothorax in newborns after cardiac surgery: a rare complication?[J]. Eur J Pediatr, 2023, 182(4): 1569-1578.
[22]	Kambara M, Ibara S. Chylothorax as a complication of congenital myotonic dystrophy: a retrospective cohort study[J]. Early Hum Dev, 2021, 158: 105396.
[23]	Ball PL, Nethercott S, Beardsall K. Rare case of congenital chylothorax and challenges in its management[J]. BMJ Case Rep, 2019, 12(5): e228023.
[24]	Takahashi Y, Kinoshita Y, Kobayashi T, et al. Management of refractory chylothorax in the neonatal intensive care unit: a 22‐year experience[J]. Pediatr Int, 2022, 64(1): e15043.
[25]	Wang B, Feng Y, Guo Y, et al. Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China[J]. J Cardiothorac Surg, 2022, 17(1): 276. doi: 10.1186/s13019-022-02009-z pmid: 36303149
[26]	Marino LV, Bell KL, Woodgate J, et al. An international survey of the nutrition management of chylothorax: a time for change[J]. Cardiol Young, 2019, 29(9): 1127-1136. doi: 10.1017/S1047951119001525 pmid: 31414980
[27]	Kobeisy SAN, Alkhotani A, Barzanji MM. Octreotide infusion for the treatment of congenital chylothorax[J]. Case Rep Pediatr, 2020, 2020(1): 8890860.
[28]	Church JT, Antunez AG, Dean A, et al. Evidence-based management of chylothorax in infants[J]. J Pediatr Surg, 2017, 52(6): 907-912. doi: S0022-3468(17)30159-8 pmid: 28342580
[29]	Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference[J]. Pediatrics, 2013, 131(1): 128-140. doi: 10.1542/peds.2012-1691 pmid: 23266923
[30]	Ozeki M, Kanda K, Kawamoto N, et al. Propranolol as an alternative treatment option for pediatric lymphatic malformation[J]. Tohoku J Exp Med, 2013, 229(1): 61-66. pmid: 23257321
[31]	Thornbury KD, McHale NG, McGeown JG. Alpha-and beta-components of the popliteal efferent lymph flow response to intra-arterial catecholamine infusions in the sheep[J]. Blood Vessels, 1989, 26(2): 107-118. pmid: 2758107
[32]	Omri S, Tahiri H, Pierre WC, et al. Propranolol attenuates proangiogenic activity of mononuclear phagocytes: implication in choroidal neovascularization[J]. Invest Ophthalmol Vis Sci, 2019, 60(14): 4632-4642.
[33]	Handal-Orefice R, Midura D, Wu JK, et al. Propranolol therapy for congenital chylothorax[J]. Pediatrics, 2023, 151(2): e2022058555.
[34]	Corda R, Chrisomalis-Dring S, Crook S, et al. Propranolol treatment for chylothorax after congenital cardiac surgery[J]. J Thorac Cardiovasc Surg, 2022, 163(5): 1630-1641.

Neonatal refractory congenital chylothorax: two case reports and literature review

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