Research & Literature

Novel mutations in PIEZO1 cause an autosomal recessive generalized lymphatic dysplasia with non-immune hydrops fetalis.

Fotiou E, Martin-Almedina S, Simpson MA, et al.

Nature communications 2015; (6()):8085 doi:10.1038/ncomms9085.

A Multiplex Kindred with Hennekam Syndrome due to Homozygosity for a CCBE1 Mutation that does not Prevent Protein Expression.

Jackson CC, Best L, Lorenzo L, et al.

Journal of clinical immunology 2016; (36(1)):19-27 doi:10.1007/s10875-015-0225-6.

Refractory tension pneumothorax as a result of an internally displaced thoracoamniotic shunt in an infant with a congenital pulmonary airway malformation.

Law BH, Bratu I, Jain V, Landry MA

BMJ case reports 2016; (2016()).

Acute pancreatitis after thoracic duct ligation for iatrogenic chylothorax. A case report.

Bédat B, Scarpa CR, Sadowski SM, et al.

BMC surgery 2017; (17(1)):9 doi:10.1186/s12893-017-0204-3.

Pseudoamniotic Band Syndrome Post Fetal Thoracoamniotic Shunting for Bilateral Hydrothorax.

Han M, Afshar Y, Chon AH, et al.

Fetal and pediatric pathology 2017; (36(4)):311-318 doi:10.1080/15513815.2017.1313915.

Effects of somatostatin/octreotide treatment in neonates with congenital chylothorax.

Yin R, Zhang R, Wang J, et al.

Medicine 2017; (96(29)):e7594 doi:10.1097/MD.0000000000007594.

Fetal Aneuploidy: A Comparison of Dichorionic Twins and Monochorionic Twins.

Shi X, Li L, Huang X, et al.

Fetal diagnosis and therapy 2018; (44(2)):124-128 doi:10.1159/000479685.

Fetal Thoracoamniotic Shunting in a Case of Congenital Pulmonary Airway Malformations with Hydrops Fetalis.

Nitta H, Taira Y, Kinjo T, et al.

AJP reports 2017; (7(3)):e185-e187 doi:10.1055/s-0037-1606830.

Prenatal factors associated with neonatal survival of infants with congenital chylothorax.

Dorsi M, Giuseppi A, Lesage F, et al.

Journal of perinatology : official journal of the California Perinatal Association 2018; (38(1)):31-34 doi:10.1038/jp.2017.150.

Octreotide for congenital and acquired chylothorax in newborns: A systematic review.

Bellini C, Cabano R, De Angelis LC, et al.

Journal of paediatrics and child health 2018; (54(8)):840-847 doi:10.1111/jpc.13889.

[Outcomes of severe primary fetal hydrothorax treated by prenatal intervention].

Xia B, Yu G, Hong C, et al.

Zhonghua fu chan ke za zhi 2018; (53(8)):522-527 doi:10.3760/cma.j.issn.0529-567x.2018.08.003.

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.

Nørgaard LN, Nygaard U, Damm JA, et al.

Fetal diagnosis and therapy 2019; (46(2)):81-87 doi:10.1159/000489775.

Long-Term Outcomes After Thoracoamniotic Shunt for Pleural Effusions With Secondary Hydrops.

Chon AH, Chmait HR, Korst LM, et al.

The Journal of surgical research 2019; (233()):304-309 doi:10.1016/j.jss.2018.08.022.

Successful treatment of congenital chylothorax with skimmed milk and long course octreotide.

Sahoo T, Mangla MK, Sethi A, Thukral A

BMJ case reports 2018; (11(1)) doi:10.1136/bcr-2018-226347.

Association between mucopolysaccharidosis Type VII and hydrops fetalis.

Holtz M, Montaño AM, Sly WS

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology 2020; (55(3)):416-417 doi:10.1002/uog.20371.

Lymphovenous anastomosis for the treatment of persistent congenital chylothorax in a low-birth-weight infant: A case report.

Hayashida K, Yamakawa S, Shirakami E

Medicine 2019; (98(43)):e17575 doi:10.1097/MD.0000000000017575.

A rare case of congenital chylothorax in a Palestinian neonate.

Abuhamda AF, Elsous A, Al-Saadi N, Al-Dadah M

Respiratory medicine case reports 2019; (28()):100937 doi:10.1016/j.rmcr.2019.100937.

Thoracic duct-to-vein anastomosis for the management of thoracic duct outflow obstruction in newborns and infants: a CASE series.

Reisen B, Kovach SJ, Levin LS, et al.

Journal of pediatric surgery 2020; (55(2)):234-239 doi:10.1016/j.jpedsurg.2019.10.029.

Congenital chylothorax: Current evidence-based prenatal and post-natal diagnosis and management.

De Angelis LC, Bellini T, Witte MH, et al.

Lymphology 2019; (52(3)):108-125.

Necrotising enterocolitis in a newborn infant treated with octreotide for chylous effusion: is octreotide safe?

Chandran S, Agarwal A, Llanora GV, Chua MC

BMJ case reports 2020; (13(2)) doi:10.1136/bcr-2019-232062.

Atypical cadherin FAT4 orchestrates lymphatic endothelial cell polarity in response to flow.

Betterman KL, Sutton DL, Secker GA, et al.

The Journal of clinical investigation 2020; (130(6)):3315-3328.

Efficacy of Early Pleurectomy for Severe Congenital Chylothorax.

Rubalcava NS, Perrone EE, Church JT, et al.

The Journal of surgical research 2020; (256()):433-438 doi:10.1016/j.jss.2020.07.005.

Management and outcomes of congenital chylothorax in the neonatal intensive care unit: A case series.

Healy H, Gipson K, Hay S, et al.

Pediatric investigation 2017; (1(1)):21-25 doi:10.1002/ped4.12007.

Causes and manifestations of chylothorax in children in China: Experience from a children's medical center, 2007-2017.

Guo Y, Chen J, Xu B, et al.

Pediatric investigation 2018; (2(1)):8-14 doi:10.1002/ped4.12019.

Causes and manifestations of chylothorax in children.

Bellini C, De Angelis LC, Bellini T, Ramenghi LA

Pediatric investigation 2018; (2(3)):203 doi:10.1002/ped4.12057.

Reply to "Causes and manifestation of chylothorax in children".

Shen K

Pediatric investigation 2018; (2(3)):204 doi:10.1002/ped4.12058.

Recurrent non-traumatic idiopathic chylothorax: a diagnostic dilemma with therapeutic challenge.

Vadala R, Talwar D, Talwar D

Respirology case reports 2020; (8(7)):e00637 doi:10.1002/rcr2.637.

Nonimmune Hydrops Fetalis - More Than Meets the Eye?

Kilby MD

The New England journal of medicine 2020; (383(18)):1785-1786 doi:10.1056/NEJMe2029914.

Should Newborns with Refractory Chylothorax Be Tried on Higher Dose of Octreotide?

Vass G, Evans Fry R, Roehr CC

Neonatology 2021; (118(1)):122-126 doi:10.1159/000512461.

Combination Therapy with Etilefrine and Pleurodesis for Refractory Congenital Chylothorax.

Tomobe Y, Mizuguchi U, Shimotakahara A, et al.

Biomedicine hub 2020; (5(3)):907-911 doi:10.1159/000509903.

Congenital Chylothorax and Hydrops Fetalis: A Novel Neonatal Presentation of RASA1 Mutation.

Gallipoli A, MacLean G, Walia JS, Sehgal A

Pediatrics 2021; (147(3)) doi:10.1542/peds.2020-011601.

Staged Management of Congenital Chylothorax With Hydrops Fetalis: An Insight Into EXIT Related Procedures.

Tai HL, Mok TYD, Chao AS, et al.

Frontiers in pediatrics 2021; (9()):633051 doi:10.3389/fped.2021.633051.

The use of high dose octreotide in management of neonatal chylothorax: Review.

Alhasoon MA

Journal of neonatal-perinatal medicine 2021; (14(4)):457-461 doi:10.3233/NPM-200644.

Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum.

Ngeow AJH, Chan MY, Teoh OH, et al.

BMJ case reports 2021; (14(4)) doi:10.1136/bcr-2020-240688.

Thoracic Empyema Secondary to Congenital Chylothorax in a 14-Month-Old Boy with Noonan Syndrome.

Oikawa T, Ota C, Iwasawa S, et al.

Case reports in pediatrics 2021; (2021()):6620353 doi:10.1155/2021/6620353.

Dietary treatment of congenital chylothorax with skimmed breast milk.

Höck M, Höller A, Hammerl M, et al.

Italian journal of pediatrics 2021; (47(1)):175 doi:10.1186/s13052-021-01125-1.

Milky pleural effusion in a neonate and approach to investigating chylothorax.

Senarathne UD, Rodrigo R, Dayanath BKTP

BMJ case reports 2021; (14(9)) doi:10.1136/bcr-2021-245576.

Chylothorax: A Stepwise Approach to Diagnosis and Treatment.

Jackson S, Jnah AJ

Neonatal network : NN 2021; (40(6)):386-392 doi:10.1891/11-T-705.

A case report of RASA1-associated inherited lymphoedema with recurrent life-threatening lymphangitis.

Westphal DS, Bergmann K, Martens E, Ibrahim T

European heart journal. Case reports 2021; (5(11)):ytab451 doi:10.1093/ehjcr/ytab451.

Clinical Course and Outcome of Non-Immune Fetal Hydrops in Singleton Pregnancies.

Reischer T, Muth B, Catic A, et al.

Journal of clinical medicine 2022; (11(3)) doi:10.3390/jcm11030702.

Changing pleural fluid triglyceride levels in cirrhotic chylothorax.

Chan KP, Yip WH, Chan TYC, Law CY

Respirology case reports 2022; (10(3)):e0907 doi:10.1002/rcr2.907.

Novel use of a Synovis™ coupler device for a surgical lymphovenous anastomosis for the treatment of refractory chylothorax in a patient with central lymphatic flow disorder.

Rezkalla J, Husain M, Slack G, Sinha S

Clinical case reports 2022; (10(3)):e05636 doi:10.1002/ccr3.5636.

Review of chylopericardium.

Rochefort MM

Mediastinum (Hong Kong, China) 2022; (6()):3 doi:10.21037/med-20-64.

Efficacy of hyperthermia pleurodesis: A comparative experimental study on serous membrane of abdominopelvic and thoracic cavities of rats.

Rivas F, Penin RM, Macía I, et al.

Cirugia espanola 2022; (100(4)):209-214 doi:10.1016/j.cireng.2022.04.001.

Multidisciplinary Management of Chylothorax.

Agrawal A, Chaddha U, Kaul V, et al.

Chest 2022; (162(6)):1402-1412 doi:10.1016/j.chest.2022.06.012.

Utility of contrast-enhanced magnetic resonance lymphangiography for yellow nail syndrome with lymphangiopathy: a case report.

Shimanuki Y, Miyoshi S, Anzai N, et al.

Oxford medical case reports 2022; (2022(7)):omac077 doi:10.1093/omcr/omac077.

Lymphangiography as a Treatment for Refractory Congenital Chylothorax Due to RASopathies: A Report of Two Cases.

Kinota N, Kodama H, Moriyama T, et al.

Interventional radiology (Higashimatsuyama-shi (Japan) 2022; (7(1)):17-20 doi:10.22575/interventionalradiology.2021-0014.

Fetal Distress and Neonatal Death After Thoracoamniotic Shunting Therapy Due to Hydrops Associated With Transient Abnormal Myelopoiesis.

Muraoka J, Yoshimoto N, Ohsawa A, et al.

Cureus 2022; (14(9)):e28991 doi:10.7759/cureus.28991.

Recurrent chylothorax treated with thoracic duct-venous anastomosis: A retrospective review of medical records.

Tang H, Zhou X, Xu M, Zhao J

JTCVS techniques 2022; (15()):199-205 doi:10.1016/j.xjtc.2022.07.015.

Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China.

Wang B, Feng Y, Guo Y, et al.

Journal of cardiothoracic surgery 2022; (17(1)):276 doi:10.1186/s13019-022-02009-z.

Propranolol Therapy for Congenital Chylothorax.

Handal-Orefice R, Midura D, Wu JK, et al.

Pediatrics 2023; (151(2)) doi:10.1542/peds.2022-058555.

Neonatal Lymphatic Flow Disorder.

Toptan HH, Ozalkaya E, Karadag N, et al.

Indian journal of pediatrics 2024; (91(3)):248-253 doi:10.1007/s12098-023-04531-x.

Case report of generalized lymphatic dysplasia with PIEZO1 mutation and review of the literature.

Alhazmi W, Qurban A, Alrashidi E

Respiratory medicine case reports 2023; (44()):101872 doi:10.1016/j.rmcr.2023.101872.

Pleurodesis using OK-432 for persistent pleural effusion after cardiac surgery in the neonatal period or early infancy.

Nakata T, Tachi M, Yasuda K, et al.

Asian cardiovascular & thoracic annals 2024; (32(2-3)):83-90 doi:10.1177/02184923231219606.

Chylopericardium following esophagectomy: a case report and systematic review.

Yang X, Zhang J, Sun P, et al.

Journal of cardiothoracic surgery 2024; (19(1)):50 doi:10.1186/s13019-024-02536-x.

Chromosomal abnormalities associated with fetal pleural effusion (I): General overview.

Chen CP

Taiwanese journal of obstetrics & gynecology 2024; (63(2)):165-167 doi:10.1016/j.tjog.2024.01.009.

Indocyanine green lymphography in the congenital chylothorax and chylous ascites.

Lin T, Shibasaki J, Yamamoto K, et al.

Journal of neonatal-perinatal medicine 2024; (17(2)):247-254 doi:10.3233/NPM-230171.

Chylothorax in Infants and Children After Congenital Heart Surgery: Approach and Review.

Kumar A, Agarwal S, Joshi RK, et al.

World journal for pediatric & congenital heart surgery 2024; (15(5)):644-652 doi:10.1177/21501351241237952.

Neonatal perspective on central lymphatic disorders.

Dulmovits BM, Taha DK

Seminars in pediatric surgery 2024; (33(3)):151424 doi:10.1016/j.sempedsurg.2024.151424.

An Updated Classification of Primary Lymphedema Based on Age of Onset, Lymphatic Anomalies, and Genetics.

Liu NF, Gao MZ, Maltese PE, Bertelli M

Lymphology 2023; (56(3)):99-109.

Successful Treatment of Congenital Chylothorax by Early Use of Prednisolone: A Case Report.

Hirano K, Nakae K, Matsunaga M, et al.

Cureus 2024; (16(5)):e60628 doi:10.7759/cureus.60628.

Pericardial fenestration and thoracic duct ligation for treatment of chylopericardium as first symptom of underlying generalized lymphatic anomaly: a case report.

Makarian RS, Mirea O, Verhamme P, et al.

Acta chirurgica Belgica 2025; (125(1)):40-43 doi:10.1080/00015458.2024.2406606.

Persistent Postoperative Chylothorax in a Neonate Undergoing Primary Esophageal Atresia Repair Successfully Treated by Open Thoracic Duct Ligation: A Case Report.

Zvizdic Z, Pilav A, Terzic S, et al.

Cureus 2024; (16(9)):e70421 doi:10.7759/cureus.70421.

Propranolol As a Treatment Option for Chylous Effusions and Chylous Ascites in Fetuses and Neonates: A Systematic Review.

Courteau B, Zarlenga G, Narciso-Owen SP, et al.

The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2024; (29(5)):468-474 doi:10.5863/1551-6776-29.5.468.

Case Report of Meconium Peritonitis: A Rare Cause of Non-immune Hydrops Fetalis.

Maddali R, Alvarez-Gell C, Balasundaram P, Sakr M

Cureus 2024; (16(11)):e72860 doi:10.7759/cureus.72860.

Retrograde thoracic duct embolization in an idiopathic case of chylopericardium.

Mak RK, Fung HS, Chan TK, Chum HL

Journal of cardiothoracic surgery 2025; (20(1)):26 doi:10.1186/s13019-024-03208-6.

Impact of enhanced chest CT with three-dimensional reconstruction combined with preoperative olive oil administration on the incidence of postoperative chylothorax: a retrospective study.

Luo X, Lu D, Wang Z, et al.

Journal of cardiothoracic surgery 2025; (20(1)):112 doi:10.1186/s13019-024-03319-0.

Antenatal phenotype associated with PAK2 pathogenic variants: bilateral pleural effusion as a warning sign.

Domenach L, Rooryck C, Legendre M, et al.

BMC medical genomics 2025; (18(1)):35 doi:10.1186/s12920-025-02096-6.

PIEZO1 variant implications for biological understanding and human health.

Cheng CW, Earle SL, Povstyan OV, et al.

Open biology 2025; (15(7)):240345 doi:10.1098/rsob.240345.

Near-infrared intraoperative fluorescence imaging using indocyanine green in thoracic duct ligation surgery in patients with chylothorax.

Shao H, Ji S, Yao Y, Liu H

Journal of cardiothoracic surgery 2025; (20(1)):381 doi:10.1186/s13019-025-03591-8.

Autologous Blood Versus Talc Pleurodesis and the Influence of Non-steroidal Anti-inflammatory Drugs.

Dittberner FA, Diaz GM, Börnsen LS, Licht PB

Interdisciplinary cardiovascular and thoracic surgery 2025; (40(11)) doi:10.1093/icvts/ivaf264.

Maternal MCT Oil-Based Diet in the Treatment of Primary Fetal Pleural Effusion: Case Reports.

Roozmeh S, Shakiba AM, Vafaei H, et al.

Clinical case reports 2025; (13(11)):e70918 doi:10.1002/ccr3.70918.

Causes and risk factors for death in infants with congenital chylothorax.

Kubo Y, Ibara S, Tokuhisa T, et al.

Frontiers in pediatrics 2025; (13()):1699515 doi:10.3389/fped.2025.1699515.

Necrotizing enterocolitis following a single very low dose of octreotide in a patient with congenital hyperinsulinism: a case successfully managed with 18F-DOPA PET/CT-guided surgery.

Uehara T, Mizutani H, Hatabu N, et al.

Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology 2026; (35(1)):92-97 doi:10.1297/cpe.2025-0023.

Effectiveness of medium-chain triglycerides in chylothorax management: A review.

Nasir S, Siddique J, Ahmed Z, et al.

Nutrition and health 2026; (32(3)):787-797 doi:10.1177/02601060251411827.