A child with cystic hygroma
|Classification and external resources|
|ICD-10||D18.1, Q87.8 (ILDS D18.140|
A cystic hygroma, also known as cystic lymphangioma and macrocystic lymphatic malformation, is often a 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 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. 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.
A lethal version 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.
Signs and Symptoms
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. 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.
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.
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.
Treatments for removal of cystic hygroma are surgery or sclerosing agents which include:
Ultrasound images of a cystic hygroma
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- Schwartz's principles of surgery: self assessment and board review, 8th edition, chapter 38, page 257; textbook p.1476
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- Syndromes By Bruno Bissonnette, Igor Luginbuehl, Bernard J. Dalens, Bruno Marciniak, http://books.google.com.au/books?id=uRR1MYa-w5wC&pg=PT235&lpg=PT235&dq=%22Cowchock+Wapner+Kurtz+syndrome%22&source=bl&ots=bEeJU7SsDU&sig=EzUmEHwBeRmM-atleoQxSc0fxuw&hl=en&ei=TR4zSr2iApPusQOOqdHYDg&sa=X&oi=book_result&ct=result&resnum=7#PPT235,M1
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- Epstein, Michael, Sherman, Stephanie, Warren, Stephen F. "Cystic Hygroma". Emory University School of Medicine, Department of Human Genetics.