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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 choice.

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Cystic Hygroma
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Cystic hygroma of the neck (outlined arrows) resulting in deviation of the trachea (solid arrow) to the contralateral side.

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Cystic Hygroma
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Facial asymmetry as a result of a big cystic hygroma in an infant boy.

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Cystic Lymphangioma; Familial Nuchal Bleb; Fetal Cystic Hygroma; Hygroma Colli.

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Approximately 1:6,000 live births suffers from cystic hygroma.

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Cystic hygroma with a normal karyotype may be inherited as an autosomal recessive trait.

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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.

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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 infection.

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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 acute blood loss. ...

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