Introduction
A cystic hygroma, also known as a cystic lymphangioma, is a rare, congenital, benign tumour of the lymphatic system. They may arise in any location of the lymphatic system, but are most common in the cervical-fascial region and particularly common in the posterior triangle of the neck. The tumours are typically filled with multiple cysts containing lymph.
Epidemiology
- Incidence: 0.50 cases per 100,000 person-years
- Most commonly see in infants
- Sex ratio: 1:1
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Clinical features
Cystic hygromas typically present at birth or within a few years following, with 90% being diagnosed before the age of 2 years. They are known associations with Turner syndrome and environmental factors such as alcohol abuse and viral illness during pregnancy.
Typical examination features:
Typical examination features:
- occur anywhere within lymphatic system, but most common in the posterior triangle of the neck
- cystic hygromas are more common on the left-side
- soft and compressible
- typically not painful
- no movement on swallowing
- transillumination is detected
- may interfere with respiration or compromise the airway
Investigations
Diagnosis and investigations:
- consider and exclude other malignancy
- ultrasound, CT or MR imaging
Differential diagnosis
Differential diagnosis of a neck lump in children:
- congenital: branchial cyst, thyroglossal cyst, dermoid cyst, vascular malformation
- inflammatory: reactive lymphadenopathy, lymphadenitis,
- neoplastic: lymphoma
Management
Management:
- Observation (some tumours are small, static or slowly regress)
- Surgical excision