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Inservice Exam - 2005
Lymphedema




A 6-year-old girl has had swelling of the right cheek for the past five years. Her mother says that the area of swelling has grown commensurately with the child and enlarges when the child has a sore throat. Physical examination shows a 4-cm mass that is soft and doughy on palpation. Which of the following is the most likely diagnosis?

(A) Arteriovenous malformation
(B) Branchial cleft cyst
(C) Capillary malformation
(D) Lymphatic malformation
(E) Venous malformation

The correct response is Option D.

The most likely diagnosis is lymphatic malformation, a localized malformation in the development of the lymphatic system. Approximately 50% of lymphangiomas are diagnosed at birth, and 90% are diagnosed before age 2 years. Lymphangiomas are soft and doughy to palpation and typically occur in the head and neck. They may vary in size from a few millimeters to larger than 30 cm. Although the overlying skin is usually normal, it may be thin, atrophic, or have bluish discoloration.

Patient symptoms correlate with the size, location, and extent of the lymphangioma. Respiratory compromise is the most common significant complication associated with lymphangiomas affecting the head and neck. Viral infections can cause enlargement of the lesion, as in this patient.

Arteriovenous malformations are high-pressure, high-flow lesions that grow in rapid spurts. These lesions are often pulsatile and warmer than the surrounding skin. A port-wine-stainBlike appearance of the skin is commonly associated. Branchial cleft cysts present as smooth, nontender masses overlying the anterior border of the sternocleidomastoid. They can fluctuate in size. Capillary malformations, or port-wine stains, are red macular lesions that are present at birth and do not regress. They can be localized or extensive. In patients with capillary malformations of the face, further darkening of skin and hyperplastic skin changes occur over time.

Hemangiomas are vascular anomalies that appear shortly after birth, proliferate rapidly during infancy, and then spontaneously regress during childhood. They do not occur in adults.

References:
1. Achauer BM. Cutaneous vascular lesions and lasers. In: Bentz ML, ed. Pediatric Plastic Surgery. Stamford, CT: Appleton & Lange; 1998:557-570.
2. Giguere CM, Bauman NM, Smith RJ. New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol. 2002;111:1066-1075.
3. Mulliken JB. Vascular anomalies. In: Aston SJ, Beasley RW, Thorne CH, eds. Grabb & Smith’s Plastic Surgery. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 1997:191-203.

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