Diagnosis

Cystic hygroma

Etiology

Cause [fyy]

• Congenital benign malformation of the lymphatic system

Pathophysiology [fyy]

1. Abnormal embryonic development of lymphatic system --> aberrant proliferation of lymphatic vessels

2. The failure of communication between lymph and venous pathways --> an accumulation of lymph --> formation of a cystic structure

3. Histologically: large macroscopic cystic spaces.

4. Often identified at birth or after airway infection --> swelling (usually in the neck) --> risk of airway compression.

Associated with

• Turner syndrome, trisomy 13/18/21, Noonan syndrome, Fryns syndrome,  Multiple-pterygium syndrome, Neu-Laxova syndrome, hydrops fetalis and genetic predisposition with autosomal recessive pattern.

Complications [yb4]

• Damage to neurovascular structures

• Chyle leak and chylothorax

• Hemorrhage

• Infection

• Recurrence

Epidemiology

Incidence per 100.000 [1bn][fyy]

Approach

• Ultrasound

• CT collum

• MRI

• Fine needle aspiration --> cytology: yellow fluid with mature lymphocytes and histiocytes

• Biopsy

Treatment

1. Asymptomatic --> no treatment (benign lesion) [fyy]

2. Infection:

   • Drainage + antibiotics

   • 3 months later --> surgical removal [1bn]

3. Hemorrhage, respiratory distress, dysphagia, or disfigurement: [fyy]

   • Sclerosis by chemical drug [1bn]

     • ethanol, doxycycline, quinine, hypertonic glucose, corticosteroids, sodium morrhuate, bleomycin, and OK-432 (picibanil)

   • Radiotherapy

   • Laser therapy (superficial microcystic lesions)

   • Cryotherapy

Differential diagnoses

Branchial cleft cyst, Carotid aneurism, Dermoid Cyst, Encephalocele, Hematoma, Lipoma, Lymphadenopathy, Lymphoma, Meningocele, Paraganglioma, Peritonsillar abscess, Sarcoidosis, Sarcoma, Teratoma, Thyroglossal duct cyst, Thyroid abscess, Toxic thyroid adenoma