Inpatient Clinical Pathway for Management of Infants with Lymphatic Disorders
Lymphatic Flow Disorder Suspected
- Review of history and physical
- Draw Initial Labs
- Cell count (body fluid)
- Triglycerides (body fluid)
- Alpha 1 antitrypsin (stool)
- Albumin (serum)
- Obtain Initial Imaging
- CXR and/or chest ultrasound for pleural effusions
- Abdominal ultrasound for ascites
- Place chest tube/peritoneal drain as needed
- Types of Lymphatic Flow Disorders
Assess Diagnostic Criteria
- Physical finding: edema or anasarca
- Abnormal lab values
- Abnormal fluid collection on imaging
Lymphatic Flow Disorder Confirmed
- Consult Lymphatics Team
- Consider additional consults as indicated
- Obtain
- Perform
Performed by interventional radiologist/ cardiologist in the Cardiac Cath Lab
Ongoing Management
Individualized for Each Patient
- Lymphatic Drainage Tubes/Catheters
- Medical Management
- Fluid Replacement
- Replace drainage when > 20 mL/kg/day
- Fresh frozen plasma (FFP) and Normal
- Saline (NSS), ½ NSS, or lactated Ringer's
- Serum albumin goal > 2.5 or 3 g/dL
- 0.5-1 g/kg/dose 25% IV albumin
- Fluid Replacement
- Nutrition Guidelines
- Parenteral Nutrition
- Enteral Nutrition
- Assess Feeding Readiness
- Initiate Trophic Feeds
- Low Long Chain Fat/High Medium Chain Fat Formulas
- Skimmed Human Milk
- Essential Fatty Acid (EFA)
- Vitamin/Mineral/Trace Element Monitoring and Supplementation
- PT/OT
- Consult PT/OT
- PT/OT Guidelines for Manual Lymph Drainage
Assess Discharge Readiness
- Drain removal for at least 24 hours
- Stable or decreased effusions on CXR
- Oxygen: requirement stable for home
- Diet: tolerating full feeds or home diet plan in place
- Schedule follow-up
- Lymphatics Team
- Nutrition
- PT/OT
Posted: July 2022
Revised: September 2022
Authors: D. Taha, DO; E. Pinto, CRNP; M. Begany, MS; A. Branas, PT; M. Burkhardt, MS; V. Bustard; E. Cross, PT; R. DeLeo, MS; C. McCabe, MSN; E. Young, MSN; M. Savoca, MS; M. Snyder, MSN; M. Stetzer, MSN; K. Zbarsky, CRNP
Evidence
- ARAF Recurrent Mutation Treatable with MEK Inhibitor
- Imaging of Central Lymphatic Abnormalities
- Intrahepatic Dynamic Contrast MR Lymphangiography
- Pathogenic Variant in EPHB4
- Lymphovenous Anastomosis for Treatment of Chylothorax in Infants
- Recognition of Neonatal Lymphatic Flow Disorder
- Post-Operative Chylothorax
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