|Classification and external resources|
A child with cystic hygroma
|ICD-10||D18.1, Q87.8 (ILDS D18.140|
A cystic hygroma (also known as cystic lymphangioma and macrocystic lymphatic malformation) is a congenital multiloculated lymphatic lesion that can arise anywhere, but is classically found in the left posterior triangle of the neck. This is the most common form of lymphangioma. It contains large cyst-like cavities containing watery fluid. 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 are filled with lymph, which is the fluid that travels in the lymphatic system of the body. Cystic hygromas occur when the lymph vessels that make up the lymphatic system aren't 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 found in healthy babies or in babies born with chromosome abnormalities. The outcome is generally better for a baby that does not have a chromosome abnormality. About 60% of cystic hygromas result from chromosome abnormalities. The baby may have a set of abnormalities known as "genetic syndrome".
Genetic syndromes with cystic hygroma as a clinical feature:
- The majority of prenatally diagnosed cystic hygromas are associated with Turner syndrome,
a chromosome abnormality in which a female has only one X chromosome instead of two.
- Chromosome abnormalities such as trisomies 13, 18, and 21
- Noonan syndrome
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.
Many cystic hygromas are usually found at birth, but some may appear after birth.
Cystic hygromas can grow very large and may affect breathing and swallowing. The following are the most common symptoms of a lymphatic malformation. However, each child may experience symptoms differently.
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.
A baby with a prenatally diagnosed cystic hygroma should be delivered in a major medical center equipped to deal with neonatal complications. An obstetrician usually decides the method of delivery. If the cystic hygroma is large, a cesarean section may be performed. After birth, infants with 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 neonatalogist 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 the wall of the cyst was able to be completely removed.
Treatments for removal of cystic hygroma are surgery or sclerosing agents which include:
- OK-432 (Picibanil)
- Pure Ethanol
- Sodium Tetradeyl Sulfate
Ultrasound images of a cystic hygroma
- Gow L, Gulati R, Khan A, Mihaimeed F (2011). "Adult-onset cystic hygroma: a case report and review of management". Grand Rounds 11: 5–11. doi:10.1102/1470-5206.2011.0002.
- Schwartz's principles of surgery: self assessment and board review, 8th edition, chapter 38, page 257; textbook p.1476
- Alpman A, Cogulu O, Akgul M, et al. (March 2008). "Prenatally Diagnosed Turner Syndrome and Cystic Hygroma: Incidence and Reasons for Referrals". Fetal. Diagn. Ther. 25 (1): 58–61. doi:10.1159/000199869. PMID 19202339.
- 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
- Vorvick, Linda F. "Cystic Hygroma". MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine.
- Epstein, Michael, Sherman, Stephanie, Warren, Stephen F. "Cystic Hygroma". Emory University School of Medicine, Department of Human Genetics.
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