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Cystic Hygroma

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Cystic Hygroma, Hygroma Cysticum, Cystic Lymphangioma, Cavernous Lymphangioma, Lymphangioma Cavernosum, Lymphangioma Cysticum, Lymphangioma

  • Definitions
  1. Lymphangioma
    1. Benign tumor developing as a Congenital Abnormality of the lymph system
    2. Results in dilated lymph channels
  • Epidemiology
  1. Onset under age 2 years (typically as a young infant)
  • Pathophysiology
  1. Congenital lymphatic malformation
    1. Lymphatic vessel fails to connect and drain
    2. Results in large multiloculated cyst
  2. Distribution
    1. Neck (60%)
    2. Other locations
      1. Chest Wall
      2. Mediastinum
      3. Axilla
    3. Rare locations
      1. Inguinal region
      2. Retroperitoneal region
  • Associated Conditions
  1. Turner's Syndrome
  2. Noonan's Syndrome
  3. Down Syndrome
  • Symptoms
  1. Initial
    1. Asymptomatic
    2. Painless lesion unless infected or bleeding
  2. Progression: Continues to increases in size
    1. 90% symptomatic by age 2 years
  • Signs
  1. Fluctuant mass usually in neck
  2. Mass transilluminates
  • Complications
  1. Infection
  2. Bleeding
  3. Airway compromise (due to compression)
  • Differential Diagnosis
  1. See Neck Masses in Children
  • Imaging
  1. Chest XRay
    1. Identify mediastinal extension
    2. Pleural Effusion
  • Management
  1. Deliver where NICU available if diagnosed prenatally
    1. Risk of airway compromise
  2. Surgical excision
  • Prognosis
  1. Recurrence rate after surgical excision: 10-15%
  • References
  1. Townsend (2001) Sabiston Surgery, Saunders, p. 546-7
  2. Muensterer (2000) Pediatr Rev 21(4):139-43 [PubMed]
  3. Weintraub (2000) Otolaryngol Clin North Am 33:1171-89 [PubMed]