(A) Alexandrite
(B) Diode
(C) Erbium
(D) Nd:YAG
(E) Pulsed dye

The correct response is Option C.

Because treatment of lymphatic malformations typically centers on relief of symptoms, including obliteration of lymphatic bullae, the erbium laser, with a wavelength of 2910 nm, is most appropriate. Lymphatic malformations are frequently larger than their skin manifestations would indicate; the intradermal and subcutaneous vessels are often extensive. The aforementioned bullae tend to drain intermittently, causing chronic infection. By eliciting an inflammatory reaction, the erbium laser causes scarring within the superficial dermis, resulting in obliteration of superficial channels and temporary relief of symptoms.

The alexandrite laser (755 nm) is indicated for removal of hair, blue and green tattoo pigments, and some pigmented lesions; the diode laser (810 nm) is used similarly. The 532-nm Nd:YAG and 585-nm pulsed dye lasers are appropriate for ablation of small vascular lesions; the 1064-nm Nd:YAG laser is recommended for removal of tattoo pigments and other dermal pigments. Because these lasers do not penetrate to the depths of the erbium laser, they are less likely to cause an inflammatory reaction and to result in obliteration of superficial channels.

References
1. Balakrishnan A, Bailey CM. Lymphangioma of the tongue: a review of pathogenesis, treatment and the use of surface laser photocoagulation. J Laryngol Otol. 1991;105:924.
2. Bass LS. Understanding laser-tissue interactions helps predict clinical effects (letter). Plast Reconstr Surg. 1995;95:607.
3. Eliezri YD, Sklar JA. Lymphangioma circumscriptum: review and evaluation of carbon dioxide laser vaporization. J Dermatol Surg Oncol. 1988;14:357.
4. White B, Adkins WY. The use of carbon dioxide laser in head and neck lymphangioma. Laser Surg Med. 1986;6:293.

Which of the following is most characteristic of large cervicofacial lymphatic malformations?

(A) Associated bruit
(B) Bony enlargement of the maxilla and mandible
(C) Frequent mediastinal involvement
(D) Rapid growth phase followed by steady involution
(E) Skin ulceration

The correct response is Option B.

Lymphatic malformations, previously known as cystic hygromas and lymphangiomas, are lesions composed of lymph-filled dysplastic vessels that occur in infancy; 90% of these lesions are noted by age 2 years. Males and females are affected equally. These malformations enlarge commensurately with growth of the child; cellulitis and respiratory infection are most likely to cause rapid growth of the lesion. Large lymphatic anomalies of the face can result in airway compromise, feeding difficulties, bleeding, and infection.

Approximately 80% of patients with large cervicofacial malformations have hypertrophy and distortion of the facial bones, typically resulting in an open bite, prognathism, and/or malocclusion. The mandible is affected most frequently, and bony overgrowth often persists despite soft-tissue resection. Examination of hypertrophied bone has shown the presence of lymphatic channels, providing a link between the malformations and involvement of osseous structures. Although orthognathic procedures are generally not performed until bony growth has been completed, patients with gross facial disharmony may require urgent treatment.

Unlike hemangiomas, lymphatic malformations do not have proliferative capacity, but enlarge in response to fluid accumulation, inadequate drainage, or anomalous lymphatic channels. Bruits are not associated, and mediastinal involvement occurs in only 2% of affected patients. Although the lesions can shrink spontaneously, this is not considered involution or regression but is more likely caused by lymphaticovenous shunting.

References
1. Mulliken JB. Cutaneous vascular malformations. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3191-3274.
2. Padwa BL, Hayward PG, Ferraro NF, et al. Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy. Plast Reconstr Surg. 1995;95:951-960.

For each patient, select the corresponding malformation (A-D).

(A) Hemangioma
(B) Lymphatic malformation
(C) Pyogenic granuloma
(D) Vascular malformation

1) A 3-month-old infant has an enlarging, bluish mass over the proximal third of the leg. Plain radiographs show a soft-tissue mass with accompanying skeletal overgrowth of the tibia.

2) A 2-year-old boy has the new onset of a rapidly growing 7-mm lesion on the nasal tip. The lesion is pedunculated and bleeds easily.

The correct response for Item 1 is Option D and for Item 2 is Option C.

The 3-month-old infant has a vascular malformation, which is comprised of anomalous vascular structures. Vascular malformations are present at birth and do not regress; they grow commensurately with the child. Skeletal deformities are associated. Treatment is often difficult, and recurrence rates are high. Surgical excision of the entire lesion is required to remove all residual tissue, decreasing the risk for recurrence.

The 2-year-old boy has a pyogenic granuloma, a rapidly growing, pedunculated lesion in the central face that often bleeds easily. Pyogenic granulomas can be differentiated from hemangiomas because they break down spontaneously with bleeding and do not typically develop during the first year of life. Curettage or excision is appropriate.

Hemangiomas are true neoplasms that develop by age 1 year, grow rapidly for the next four to eight months, and then regress spontaneously. Less than 5% of hemangiomas break down spontaneously with bleeding. Skeletal deformities are rarely associated.

Lymphatic malformations can be superficial or deep. Superficial lymphatic malformations are comprised of anomalous lymphatic remnants that manifest as clear vesicles. Deep lymphatic malformations appear similar to hemangiomas but are soft and compressible on palpation. Bony overgrowth is associated.

References
1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412.
2. Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases. Pediatr Dermatol. 1991;8:267-276.

For each patient, select the most likely diagnosis (A-E).

(A) Klippel-Feil syndrome
(B) Klippel-Trenaunay syndrome
(C) Maffucci syndrome
(D) Rendu-Osler-Weber syndrome
(E) Sturge-Weber syndrome

1) A 15-year-old girl has a short neck, a low posterior hairline, and fusion of the cervical vertebrae.

2) A 14-year-old boy has enlargement of the left leg with a port-wine stain and venous malformations in the affected area.

The correct response for Item 1 is Option A and for Item 2 is Option B.

The 15-year-old girl has findings consistent with Klippel-Feil syndrome, which is characterized by a short neck and a low posterior hairline. Affected patients have fusion of the cervical vertebrae. Vascular anomalies are not typically associated.
The 14-year-old boy has Klippel-TrŽnaunay syndrome. Characteristic findings include limb hemihypertrophy, most frequently affecting the leg, with an associated port-wine stain. Lymphatic and venous malformations also occur.
Patients with Maffucci syndrome have multiple enchondromas, most frequently affecting the hand, and venous malformations. The bones of the fingers, toes, and proximal extremities are typically deformed.

Rendu-Osler-Weber syndrome, or hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder that manifests as telangiectasias of the lips, tongue, oral mucosa, and gastrointestinal tract. The pulmonary and nervous systems may also be affected. Profuse bleeding can be life-threatening in these patients.

Sturge-Weber syndrome is associated with a port-wine stain that often occurs in the distribution of the trigeminal (V) nerve. Other findings include intracranial calcifications and leptomeningeal venous malformations.

References
1. Elster AD. Quadriplegia after minor trauma in the Klippel-Feil syndrome. J Bone Joint Surg. 1984;66A:1473.
2. Mulliken JB, Young AE, eds. Vascular Birthmarks: Hemangiomas and Malformations. Philadelphia, Pa: WB Saunders Co; 1988.