Cystic hygroma is a benign, multiloculated cystic
structure. Cystic hygromas form as a result of budding lymphatics. They
may occur anywhere in the body, although they are most frequently
encountered in the neck and axilla. Cystic hygromas frequently abut and/or
encompass neurovascular structures. Surgical excision remains the therapy of
Cystic hygroma of the neck (outlined arrows) resulting in deviation of the trachea (solid arrow) to
the contralateral side.
Facial asymmetry as a result of a big cystic hygroma in an infant
Cystic Lymphangioma; Familial Nuchal Bleb; Fetal Cystic
Hygroma; Hygroma Colli.
Approximately 1:6,000 live births suffers from cystic
Cystic hygroma with a normal karyotype may be
inherited as an autosomal recessive trait.
Cystic hygromas can range from increased nuchal
translucency to thin-walled cystic masses that can become larger than the
fetal head. The cysts may result from a lymphatic abnormality, possibly
because of absent or inefficient connections between the lymphatic and
venous systems. The concept that correct communications between these two
systems develop later during gestation in some fetuses may account for
cystic areas that resolve with merely a residual of redundant skin
(pterygium colli). Other theories for the development of cystic hygromas
include possible abnormal sequestration of embryonic lymphatic tissue that
does not communicate with normal lymph flow channels and budding of
lymphatics that canalize to form lymph-filled cysts.
Prenatal diagnosis is often possible (ultrasound). Approximately
two thirds of children present at birth or within the first year
of life and in 90% within 2 years of age. Most often, the lesion is a
cosmetic problem, however, compression of the airway, esophagus, and/or
great vessels may occur as the hygroma invades the deeper structures of the
neck or the mediastinum. The clinical course is characterized by
intermittent or progressive growth, spontaneous regression, hemorrhage, and
Cystic hygromas, most often occurring in the neck
(75%) and axilla (20%), belong to a group of diseases that now are
considered lymphatic malformations. Occasionally, cystic hygromas become
very large and extend into the mediastinum, axillae, mouth, and chest.
Occasionally, they occur exclusively in those areas. Cystic hygromas are
often associated with lymphedema, congenital cardiac defects, and
chromosomal anomalies. Other features may include cleft lip/palate, single
umbilical artery, and horseshoe, fused, or ectopic kidneys. Umbilical
hernia, thymic aplasia, and holoprosencephaly/arrhinencephaly have also been described.
The risk of infection of a cystic hygroma is approximately 16%
and may result in additional swelling, pain, fever, and localized erythema.
The risk of hemorrhage is approximately 13%. Consider this complication
with an enlarging and painful cystic hygroma in a patient with evidence of