Prevalence:

• 1 in 6,000 births.

Ultrasound diagnosis:

• Multiseptated, multicystic irregular mass, which is usually located on the neck (75%), axillary region (20%), chest wall, abdominal wall and extremities (5%); in <1% of cases the tumor is in the mesentery or retroperitoneum.

Associated abnormalities:

• The incidence of chromosomal abnormalities and genetic syndromes is not increased.

Investigations:

• Detailed ultrasound examination.
• In case of cervical and thoracic lymphangioma, there is an increased risk of central venous compression leading to hydrops and esophageal compression resulting in polyhydramnios.

Follow-up:

• Ultrasound scans every 2-3 weeks to monitor the size of the tumor and assess amniotic fluid volume. Amniodrainage may be necessary if there is polyhydramnios and cervical shortening.

Delivery:

• Place: hospital with neonatal intensive care and pediatric surgery.
• Time: 38 weeks.
• Method: cesarean section with EXIT procedure if there is polyhydramnios and hyperextension of the neck due to a large cervical tumor.

Prognosis:

• Prognosis is favourable, unless there is associated cardiac failure, hydrops or high airway compression.
• Some mesenchymal lesions may partially regress spontaneously after birth. The treatment of choice is surgical excision.

Recurrence:

• No increased risk of recurrence.