Cystic hygroma

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Cystic hygroma

Cystic Hygroma.jpg
A child with cystic hygroma

Classification and external resources

Specialty
oncology, medical genetics

ICD-10
D18.1, Q87.8 (ILDS D18.140

ICD-9-CM
228.1

ICD-O
M9173/0

OMIM
257350

DiseasesDB
3374

MedlinePlus
000148

eMedicine
ped/536

Cystic hygromas are classically found in the posterior triangle of the neck
(purple).
A cystic hygroma, also known as cystic lymphangioma and macrocystic
lymphatic malformation, is an often congenital multiloculated lymphatic lesion
that can arise anywhere, but is classically found in the left posterior triangle of
the neck and armpits. This is the most common form of lymphangioma. It
contains large cyst-like cavities containing lymph, a watery fluid that circulates
throughout the lymphatic system. Microscopically, cystic hygroma consists of
multiple locules filled with lymph. In the depth, the locules are quite big but they
decrease in size towards the surface.

Cystic hygromas are benign, but can be disfiguring. It is a condition which usually
affects children; very rarely it can present in adulthood.[1]

Cystic hygroma is also known as lymphatic malformation. Nowadays, the medical

field prefers to use the term lymphatic malformation because the term cystic
hygroma means water tumor[citation needed]. Lymphatic malformation is more
commonly used now because it is a sponge-like collection of abnormal growth
that contains clear lymphatic fluid. The fluid collects within the cysts or channels,

usually in the soft tissue. Cystic hygromas occur when the lymphatic vessels that
make up the lymphatic system are not formed properly. There are two types of
lymphatic malformations. They are macrocystic lymphatic malformations, large
cysts, and microcystic, small cysts. A person may have only one kind of the
malformation or can have a mixture of both macro and micro cysts.

Cystic hygroma can be associated with a nuchal lymphangioma or a fetal

hydrops.[2] Additionally, it can be associated with Turner syndrome[3] or with
Noonan syndrome.

A lethal version[4] of this condition is known as Cowchock Wapner Kurtz

syndrome that, in addition to cystic hygroma, includes cleft palate and
lymphedema, a condition of localized edema and tissue swelling caused by a
compromised lymphatic system.[5]

Contents
1 Signs and Symptoms
2 Treatment

Signs and Symptoms[edit]

Cystic hygromas are increasingly diagnosed by prenatal ultrasonography. A

common symptom is a neck growth. It may be found at birth, or discovered later
in an infant after an upper respiratory tract infection.[6] Cystic hygromas can
grow very large and may affect breathing and swallowing. Some symptoms may
include a mass or lump in the mouth, neck, cheek, or tongue. It feels like a large
fluid-filled sac. In addition, cystic hygromas can be found in other body parts
such as the arm, chest, legs, groin, and buttocks. Cystic hygromas are also often
seen in Turner's syndrome, although a patient who does not have Turner's
syndrome can present with this condition.

Treatment[

A baby with a prenatally diagnosed cystic hygroma should be delivered in a

major medical center equipped to deal with neonatal complications, such as a
neonatal intensive care unit. An obstetrician usually decides the method of
delivery. If the cystic hygroma is large, a cesarean section may be performed.
After birth, infants with a persistent cystic hygroma must be monitored for airway
obstruction. A thin needle may be used to reduce the volume of the cystic
hygroma to prevent facial deformities and airway obstruction. Close observation
of the baby by a neonatologist after birth is recommended. If resolution of the
cystic hygroma does not occur before birth, a pediatric surgeon should be
consulted.[7]

Cystic hygromas that develop in the third trimester, after thirty weeks gestation,
or in the postnatal period are usually not associated with chromosome
abnormalities. There is a chance of recurrence after surgical removal of the cystic
hygroma. The chance of recurrence depends on the extent of the cystic hygroma
and whether its wall was able to be completely removed.[7]

Treatments for removal of cystic hygroma are surgery or sclerosing agents which
include:
Bleomycin
Doxycycline
Ethanol (pure)
Picibanil (OK-432)
Sodium tetradecyl sulfate