Neonatal Lymphatic Flow Disorder.

OBJECTIVE: To examine and discuss patients diagnosed with acquired and congenital chylothorax in the neonatal period in the light of the literature. METHODS: The files of newborns followed-up in the neonatal intensive care unit (NICU) and diagnosed with congenital and acquired chylothorax were reviewed retrospectively. Patients with isolated chylothorax were classified as Group 1 and those with multiple lymphatic flow disorders were classified as Group 2. Antenatal and clinical features were recorded and compared between the groups. RESULTS: Thirteen infants were diagnosed with chylothorax; 92.3% (n = 12) of the patients were congenital. The rate of antenatal diagnosis was 61.5% (n = 8). Eight patients (61.5%) were diagnosed with hydrops fetalis. Among the cases in Group 1 and Group 2, receiving ocreotide and the incidence of sepsis (p = 0.05) were partially significant. Seven of the patients (66.6%) responded to medium chain triglycerides (MCT), and complete resolution was seen in 6 (85.7%) of the responders. Complete resolution of chylothorax fluid was observed in 7 (77.7%) of nine patients who responded to ocreotide treatment. CONCLUSIONS: In neonatal chylothorax, the postnatal period includes a multidisciplinary approach that requires drug therapy, dietary modifications, drainage of pleural fluid, and rarely, surgery.

Propranolol Therapy for Congenital Chylothorax.

Congenital chylothorax is a rare and often severe anomaly without well-established medical therapies. Previously, propranolol use in patients with lymphatic malformations and secondary chylothorax was associated with improvement in clinical signs. We hypothesized that propranolol treatment would be beneficial for severe congenital chylothorax. We reviewed medical records of neonates born from 2015 to 2019 at our tertiary center with a prenatal diagnosis of congenital chylothorax for whom either prenatal or postnatal propranolol therapy was initiated. Inclusion was limited to fetuses diagnosed with severe congenital chylothorax without significant genetic, infectious, or cardiac anomalies, and who underwent prenatal interventions to mitigate consequences of the condition. Propranolol was administered orally to pregnant women at 20 mg 4 times daily and increased to a maximum dose of 40 mg 4 times daily, or to infants at 0.3 mg/kg/d and increased to 1 to 2 mg/kg/d. Primary outcomes were the time course of resolution of ultrasonographical, clinical, and/or radiologic signs of chylothorax after treatment with propranolol. Four neonates met the inclusion criteria. In 2 cases, prenatal initiation of propranolol led to resolution of the chylothoraxes before delivery (38 and 32 days after treatment) on a dose of 40 mg/day 4 times daily. Neonates had a normal postnatal course. Postnatal propranolol was initiated in 2 neonates with respiratory failure when chylothoraces were refractory to standard management. Stabilization and improvement of their pleural effusion was observed by imaging at 29 and 13 days after initiation of propranolol. There were no significant maternal or neonatal complications from prenatal or postnatal propranolol use. Propranolol may be efficacious in treating severe fetal congenital chylothorax.

Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018.

BACKGROUND: Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy. MATERIAL AND METHODS: A PubMed search was performed according to the PRISMA criteria. All cases were analyzed according to prenatal, perinatal, and postnatal treatment modalities and follow-ups. RESULTS: We identified 753 cases from 157 studies published between 1990 and 2018. The all-cause mortality rate was 28%. Prematurity was present in 71%, male gender dominated 57%, mean gestational age was 34 weeks, and birth weight was 2,654 g. Seventy-nine percent of newborns had bilateral CCT, the most common associated congenital anomalies with CCT were pulmonary lymphangiectasia and pulmonary hypoplasia, and the most common chromosomal aberrations were Down, Noonan, and Turner syndromes, respectively. Mechanical ventilation was reported in 381 cases for mean 17 (range 1-120) days; pleural punctuations and drainages were performed in 32% and 64%, respectively. Forty-four percent received total parenteral nutrition (TPN) for mean 21 days, 46% medium-chain triglyceride (MCT) diet for mean 37 days, 20% octreotide, and 3% somatostatin; chemical pleurodesis was performed in 116 cases, and surgery was reported in 48 cases with a success rate of 69%. In 462 cases (68%), complete restitution was reported; in 34 of 44 cases (77%), intrauterine intervention was carried out. CONCLUSION: Respiratory support, pleural drainages, TPN, and MCT diet as octreotide remain to be the cornerstones of CCT management. Pleurodesis with OK-432 done prenatally and povidone-iodine postnatally might be discussed for use in life-threatening CCT.

Dietary treatment of congenital chylothorax with skimmed breast milk.

BACKGROUND: Congenital chylothorax (CC) is a rare but potentially life-threatening condition in newborns. It is defined as an accumulation of chyle in the pleural cavity. The few publications regarding medical management and therapeutic dietary intervention motivated us to share our experience. METHODS: Neonates diagnosed with congenital chylothorax and treated at Innsbruck Medical University Hospital between 2013 and 2020 (n = 6, gestational age: 36 3/7, 32 5/7, 36 4/7, 35 0/7, 35 4/7, 37 3/7 weeks) were eligible for this report. The cornerstones of treatment for chylothorax conventionally consist of chest tube drainage (CTD), respiratory support, dietary restriction of long-chain triglycerides (LCT) or total parenteral nutrition (TPN). In further course the introduction of a medium-chain triglyceride (MCT)-based formula followed by an overlapping switch to a formula with low LCT and high MCT, containing the essential long-chain fatty acids (LCFA), is attempted. In three patients we used fat-modified (skimmed) breast milk to provide a high protein and low fat diet and to avoid the discontinuation of breast milk. RESULTS: The outcome of an early introduction of LCFA in the form of skimmed breast milk after resolution of chylothorax diverse. One patient had a favourable outcome, meaning no recurrence of pleural effusion, adequate weight gain and a content mother, while another patient had a relapse of pleural effusion after the administration of skimmed milk and was therefore transitioned back to Basic F® . The CC of patient 5 was difficult due to Noonan syndrome. Two weeks after the introduction of skimmed breast milk the mother wanted to stop to express breast milk, so nutrition was changed to Basic F®. CONCLUSION: The first-line therapy of chylothorax is a combination of respiratory stabilization and dietary modification. The use of skimmed breast milk is advisable in CC and feasible by means of a simple milk defatting procedure. It offers benefits to mothers who wish to resume breast feeding after resolution of chylothorax and has proven positive effects, above all in preterm infants as optimal nutrition with protective components superior to formula feeding. However, the nutritional analysis of the skimmed milk and the correlation to a re-accumulation of pleural fluid remains a question to be answered.

Midodrine, an Oral Alpha-1 Adrenoreceptor Agonist, Successfully Treated Refractory Congenital Chylous Pleural Effusion and Ascites in a Neonate.

A trisomy 21 neonate presented with congenital chylous pleural effusion and ascites that was refractory to conventional pharmacotherapy. Midodrine, an oral alpha-1-adrenoreceptor agonist, achieved remission of chylous effusion without any adverse effects. To the best of our knowledge, this is the first neonatal case of successful management of congenital chylous pleural effusion and ascites with midodrine.

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

A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.

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

Mutations in the RASA1 gene are known to cause arteriovenous malformations (AVMs), with evidence of associated lymphatic malformations. We report for the first time, to the best of our knowledge, an infant with RASA1 mutation presenting with hydrops fetalis and chylothorax, but without an associated AVM. Previously, researchers studying rodents have found chylothorax associated with RASA1 mutations, and, in previous case reports, researchers have reported on infants with RASA1 mutations born with hydrops fetalis and AVMs. In this report, we describe the case of a "late preterm" female infant born with nonimmune hydrops fetalis and congenital chylothorax who was detected to have a RASA1 deletion on genetic workup. Although classically described phenotypes of RASA1 mutations present with venous malformations, no such malformations were found in this infant on extensive imaging. This combination is a novel and nonclassic presentation of RASA1 mutation. In cases of congenital chylothorax, especially with nonimmune hydrops fetalis, RASA1 mutations should be considered as part of the differential diagnosis and genetic testing should be included as part of a complete workup to allow for screening for associated vascular anomalies.

Hydrothorax in fetal cases of Opitz G/BBB diagnosis: Extending the phenotype?

We report two fetal cases carrying a de novo MID1 mutation and presenting with severe hydrothorax, suggesting the expansion of the phenotype of Opitz GBBB syndrome.

Efficacy of Early Pleurectomy for Severe Congenital Chylothorax.

BACKGROUND: Severe congenital chylothorax (SCC) may result in respiratory failure, malnutrition, immunodeficiency, and sepsis. Although typically managed with bowel rest, parenteral nutrition, and octreotide, persistent chylothoraces require surgical management. At our institution, a pleurectomy, unilateral or bilateral, in combination with mechanical pleurodesis and thoracic duct ligation is performed for SCC, and we describe our approach and outcomes. MATERIALS AND METHODS: We reviewed over 15-year period neonatal patients with SCC managed surgically with pleurectomy after medical therapy was unsuccessful. Patients were divided into two groups: those who underwent pleurectomy within 28 d of diagnosis (early group) and those who underwent pleurectomy after 28 d (late group). Resolution of chylothorax was defined by the absence of clinical symptoms as well as absent or minimal pleural effusion on chest X-ray. RESULTS: Of 40 patients diagnosed with SCC over the study period, 15 underwent pleurectomy, eight early [mean time to operation = 20 (IQR 17, 23) d] and 7 late [59 (42, 75) d, P = 0.001]. Overall survival was 67% (10 of 15). Seven of 8 (88%) neonates who underwent early pleurectomy survived versus 3 of 7 (43%) who underwent late pleurectomy (P = 0.07). Length of stay was lower in the early group than the late group [73 (57, 79) versus 102 (109, 213) d, P = 0.05]. All patients who survived to discharge had resolution of their chylothorax. CONCLUSIONS: Pleurectomy with mechanical pleurodesis and thoracic duct ligation is effective in the management of severe congenital chylothorax. When performed earlier, pleurectomy for severe congenital chylothorax may be associated with improved survival and shorter hospital length of stay.

Suturas adhesivas mediante cianoacrilato en drenajes pleurales de prematuros / Adhesive sutures using cyanoacrylate in pleural drains of premature infants

No disponible

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

Octreotide is a somatostatin analogue used for treating congenital chylothorax and congenital hyperinsulinism in infants. By increasing splanchnic arteriolar resistance and decreasing gastrointestinal blood flow, octreotide indirectly reduces lymphatic flow in chylous effusions.Splanchnic ischaemia following octreotide predisposes infants to necrotising enterocolitis (NEC). Although NEC occurrence in infants treated with octreotide for hyperinsulinaemic hypoglycaemia has been reported widely, its incidence in infants with chylothroax is low. We describe a case of congenital chylothorax in a preterm infant who had poor response to thoracentesis. Although octreotide initiation lead to resolution of chylothorax, he developed NEC. Cessation of octreotide and medical management resulted in rapid resolution of NEC. Since octreotide is generally used as the first-line treatment for chylous effusion, the risk of NEC should be considered, especially when the dosage is increased. Infants on octreotide should be closely observed for early signs and symptoms of NEC to avert surgical emergency.

In utero congenital chylothorax treatment with fetal thoracoamniotic shunt: Case report.

BACKGROUND: Fetal pleural effusions are a rare fetal anomaly that may result from congenital chylothorax. Severe cases lead to chest compression with resulting pulmonary hypoplasia and possible neonatal demise. Fetal thoracoamiontic shunt (TAS) placement may decrease the amount of pleural effusion and improve lung expansion. CASE: A 30-year-old primigravida at 29 2/7 weeks' gestation presented with fetal bilateral pleural effusions with no identifiable genetic or structural abnormalities. TAS placement accomplished decompression of the left fetal chest. The neonate was delivered at 33 3/7 weeks and required minimal respiratory support with no apparent long term complications at discharge. CONCLUSION: This case demonstrated that fetal intervention with TAS placement can improve neonatal outcomes. Referral to an MFM specialist capable of TAS should be considered for isolated fetal bilateral pleural effusion.