🧬 Chapter 538 · Abnormalities of Lymphatic Vessels

Nelson Textbook of Pediatrics 22nd Edition | Lymphatic malformations (LMs): cystic LMs (macrocystic, microcystic), often in head/neck/axilla. Complex lymphatic anomalies (CLAs): generalized lymphatic anomaly (GLA), Gorham-Stout disease (progressive osteolysis), kaposiform lymphangiomatosis (KLA, consumptive coagulopathy), central conducting lymphatic anomaly (CCLA). Genetics: PIK3CA mutations (LMs), NRAS/KRAS/ARAF (CLAs). Treatment: sirolimus (mTOR inhibitor), sclerotherapy, surgery, trametinib (MEK inhibitor) for RAS-mutated CLAs. Lymphedema, lymphangioleiomyomatosis (LAM), lymphangitis.

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📋 30 Clinical Scenarios — Lymphatic Vessel Abnormalities

📇 High‑Yield Review Cards (Lymphatic Anomalies)

🩺 Clinical Recognition: Lymphatic Vessel Abnormalities

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Lymphatic Anomalies

🔑 Key Principles — Nelson Chapter 538
• Lymphatic malformations (LMs): Macrocystic (sclerotherapy with OK432, bleomycin, ethanol), microcystic (sirolimus). Surgical excision if symptomatic.
• Complex lymphatic anomalies (CLAs): Generalized lymphatic anomaly (GLA), Gorham-Stout (progressive osteolysis), kaposiform lymphangiomatosis (KLA, consumptive coagulopathy), central conducting lymphatic anomaly (CCLA). First-line systemic therapy: sirolimus (mTOR inhibitor).
• Genetics: PIK3CA mutations (LMs, PROS). NRAS (KLA), KRAS (Gorham-Stout), ARAF (CCLA). Trametinib (MEK inhibitor) for RAS-mutated CLAs.
• Lymphedema: Primary (Milroy, Meige, FOXC2) or secondary. Treatment: compression, manual lymphatic drainage, surgery (lymphovenous anastomosis).
• Lymphangioleiomyomatosis (LAM): Women with tuberous sclerosis. Sirolimus stabilizes lung function.
• Lymphangitis: Antibiotics (streptococci/staphylococci).

    ⚡ Reflex Prompts — Clinical Decisions in Lymphatic Anomalies

    📖 Summary: Abnormalities of Lymphatic Vessels — Nelson Ch 538