Propranolol for the Treatment of Lymphatic Malformations in a Neonate – A Case Report and Review of Literature

Abstract

Lymphatic malformations in neonates often manifest as a chylothorax, and although rare, morbidity and mortality can be significant. First-line treatment with medium-chain triglyceride–enriched formulas, or enteric rest with total parenteral nutrition, are not always successful. We describe the case of a premature neonate with trisomy 21 who presented with bilateral pleural effusions and a pericardial effusion that worsened with the initiation of enteral nutrition. Clinical improvement was not seen until the initiation of treatment with oral propranolol at a maximum dosage of 0.5 mg/kg/day divided every 8 hours with extubation 8 days after propranolol initiation. Two case reports have described the use of propranolol in similar patients receiving 2 mg/kg/day; however, our experience is the first to report treatment success at a much lower dose. A review of the literature for alternative medication treatments uncovered numerous case reports and series documenting variable results with incongruent definitions of treatment success in a diverse patient population. The rarity of this disease state makes accrual of patients difficult and more robust treatment data unlikely. Therefore, selection of the optimal adjunctive treatment must be based on individual patient and disease state characteristics as well as safety and efficacy profile of the medication.

The Update of Treatment for Primary Intestinal Lymphangiectasia

“…Propranolol can be started orally at a dose of 1 to 4 mg/kg/day. Some cases reported that propranolol treatment was effective after 12 weeks [ 37 38 ].…”

Chylothorax in the neonate—A stepwise approach algorithm

“…Propranolol has been used in neonates to treat chylothoraces associated with lymphatic malformations; descriptions of propranolol use are limited to case reports and small case series with variable degrees of success (LoE C) 60–63 . The effects of propranolol may be caused by the suppression of proangiogenic factors, which are also lymphangiogenic factors (VEGF1, bFGF, and MMP‐2), in the short term, and by the increase in the apoptosis of endothelial cells in the long term 64 …”

“…The pharmacological agents used to treat chylothorax in neonates, as well as doses and adverse effects are summarised in Table 2 54,59,63,71,72 …”

Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease

“…The pathway of function is speculative, with a reduction in both splanchnic blood flow and gastrointestinal secretion, an antagonistic effect on lymphatic vessel function, and reduced uptake of triglycerides being proposed as possible mechanisms [46,47]. Other treatment options include nitric oxide, etilenephrine, corticosteroids, propranolol, and high positive-end expiratory pressure ventilation [48][49][50][51][52]. However, evidence supporting the efficacy of these treatments remains sparse.…”