Cystic Hygroma

Aka: Cystic Hygroma, Hygroma Cysticum, Cystic Lymphangioma, Cavernous Lymphangioma, Lymphangioma Cavernosum, Lymphangioma Cysticum, Lymphangioma

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II. Definitions

  1. Lymphangioma
    1. Benign tumor developing as a Congenital Abnormality of the lymph system
    2. Results in dilated lymph channels

III. Epidemiology

  1. Onset under age 2 years (typically as a young infant)

IV. 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

V. Associated Conditions

  1. Turner's Syndrome
  2. Noonan's Syndrome
  3. Down Syndrome

VI. 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

VII. Signs

  1. Fluctuant mass usually in neck
  2. Mass transilluminates

VIII. Complications

  1. Infection
  2. Bleeding
  3. Airway compromise (due to compression)

IX. Differential Diagnosis

  1. See Neck Masses in Children

X. Imaging

  1. Chest XRay
    1. Identify mediastinal extension
    2. Pleural Effusion

XI. Management

  1. Deliver where NICU available if diagnosed prenatally
    1. Risk of airway compromise
  2. Surgical excision

XII. Prognosis

  1. Recurrence rate after surgical excision: 10-15%

XIII. 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]

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