Definition and Characteristics
- Cystic hygroma is an abnormal growth that appears on a baby's neck or head.
- It consists of one or more cysts that tend to grow larger over time.
- The disorder usually develops while the fetus is still in the uterus but can also appear after birth.
- Cystic hygroma is also known as cystic lymphangioma and macrocystic lymphatic malformation.
- It is a congenital lymphatic lesion with multiple locules filled with lymph.
Benign Nature and Associations
- Cystic hygromas are benign but can be disfiguring.
- It usually affects children, but rarely can be present in adulthood.
- It is associated with nuchal lymphangioma or fetal hydrops.
- It can be associated with Down syndrome, Turner syndrome, or Noonan syndrome.
- Cowchock-Wapner-Kurtz syndrome is a lethal version that includes cystic hygroma, cleft palate, and lymphedema.
Signs and Symptoms
- Cystic hygromas are classically found in the posterior triangle of the neck.
- They are increasingly diagnosed by prenatal ultrasonography.
- A common sign is a neck growth that may be found at birth or later in an infant.
- Cystic hygromas can grow very large and may affect breathing and swallowing.
- Other body parts like the mouth, cheek, tongue, arms, chest, legs, groin, and buttocks can also be affected.
Diagnosis
- Lymphatic malformations can be detected by ultrasound during pregnancy.
- Further investigation, such as amniocentesis, may be done to evaluate for genetic abnormalities.
- Postnatal or incidental findings during medical imaging can also lead to the diagnosis.
- Imaging techniques like ultrasound or MRI can provide more information about the size and extent of the lesion.
- Multiple cystic masses can arise in children, requiring verification of the diagnosis.
Treatment
- Babies with prenatally diagnosed cystic hygromas should be delivered in a major medical center.
- Cesarean section may be performed for large cystic hygromas.
- Close observation by a neonatologist is recommended after birth.
- Thin needle aspiration may be used to reduce the volume of the cystic hygroma.
- Surgical removal of the cystic hygroma may be necessary, but recurrence is possible depending on the extent of the condition.
A cystic hygroma is an abnormal growth that usually appears on a baby's neck or head. It consists of one or more cysts and tends to grow larger over time. The disorder usually develops while the fetus is still in the uterus, but can also appear after birth.
| Cystic hygroma | |
|---|---|
| Other names | Lymphangioma |
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| Specialty | Oncology, medical genetics  |
Also known as cystic lymphangioma and macrocystic lymphatic malformation, the growth is often a congenital lymphatic lesion of many small cavities (multiloculated) that can arise anywhere, but is classically found in the left posterior triangle of the neck and armpits. The malformation 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. Deep 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 be present in adulthood.
Currently, the medical field prefers to use the term lymphatic malformation, because the term cystic hygroma means water tumor. 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. The two types of lymphatic malformations are macrocystic (large cysts) and microcystic (small cysts) lymphatic malformations. A person may have only one kind of the malformation or can have a mixture of both macro- and microcysts.
Cystic hygroma can be associated with a nuchal lymphangioma or a fetal hydrops. Additionally, it can be associated with Down syndrome, Turner syndrome, or Noonan syndrome. If it is diagnosed in the third trimester, then chances of association with Down syndrome are increased, but if diagnosed in the second trimester, then it is associated with Turner syndrome.
A lethal version of this condition exists, 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.
