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KEY POINTS
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Key Points

  • Cystic hygroma and lymphangioma are older commonly used terms for a specific type of vascular malformation.

  • Most often seen in the soft tissue of the neck, axilla, thorax, and lower extremities.

  • Different prenatal natural history if diagnosed in the first trimester versus the third.

  • Lymphangiomas presenting in the third trimester usually seen in the anterior or anterolateral neck are not usually associated with other anomalies or hydrops.

  • Lymphatic vascular malformations consisting of cysts separated by fine septa.

  • While the mortality of lymphatic vascular malformations prior to 30 weeks’ gestation is high, due to hydrops and karyotype abnormalities, those presenting later have an excellent prognosis.

  • Cervical lymphangiomas are at risk for airway compromise, and an EXIT procedure should be considered if there is evidence of airway compression or displacement.

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CONDITION
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Traditionally referred to as lymphangioma or if in the neck cystic hygroma, these are currently considered a form of vascular malformation of the lymphatic system characterized by localized or diffuse malformations of lymphatic channels that can be characterized as microcystic, macrocystic, or both (Christison and Fishman, 2006).

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Lymphangioma is a benign type of vascular malformation composed predominantly of dilated cystic lymphatics (Isaacs, 1997). These malformations are often present at birth and are second only to hemangioma as a cause of soft tissue mass in the newborn (Potter and Craig, 1975; Isaacs, 1983, 1991, 1997). Lymphangiomas can occur in almost any location but are most commonly seen in the soft tissue of the neck, axilla, thorax, and lower extremities (Isaacs, 1997). Isaacs reported a series of 97 consecutive lymphangiomas seen at Children’s Hospital of Los Angeles in which 45 occurred in the neck, 22 in the chest wall, 12 in the extremities, and 4 in the abdominal wall. Other less common sites included the omentum, mesentery, larynx, tongue, bowel, retroperitoneum, mediastinum, conjunctiva, and mouth (Isaacs, 1991). These lesions can vary in size from tiny subepidermal skin blebs to large dilated cystic fluid filled– masses that, when presenting in the neck, are commonly referred to as cystic hygromas.

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There is a disparity between lymphangiomas that are diagnosed at birth as isolated findings in otherwise healthy infants and those detected prenatally during the first or second trimester (see chapter 31). Prenatal sonographic examination in the first and second trimester identifies a group of fetuses with cystic hygroma in which 60% have associated chromosomal abnormalities and are often associated with other structural anomalies that have an extremely high mortality rate (Romero et al., 1988; Cohen et al., 1989; Welborn and Timm, 1994). In this group of fetuses, cystic hygromas are distinguished by posterior triangle location of the lymphangioma, chromosomal abnormalities, structural anomalies, hydrops fetalis, a high incidence of intrauterine death, and rare postnatal survival. In contrast, isolated cystic hygroma presenting during the third ...

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