CLINICAL HISTORY:

Signs and symptoms:  The mother states that the lesion has been present since the baby was born and has been getting bigger. The child demonstrates full range of motion of the arm, no crying,crankiness or failure to thrive.

Previous Treatment:  none

Other information:  the patient was born premature at 35 weeks and demonstrated no postnatal complications

PHYSICAL EXAM:

On physical, an erythematous and violaceous papule extending from the forearm to the wrist to the hand and thumb was noted. Easy compressability and blanching were present. No ulcerations or active bleeding were noted. Wrist circumference of the right was 10 ½ cm and the left was 10cm. forearm to the wrist 9 cm on the right and 9 ½ cm on the left. Pt was noted to have a sacral dimple and overlying Mongolian spot as well

LABORATORY TESTS:

Ultrasound of the lumbosacral spine was unremarkable.

Head CT was also unremarkable.

Genetic testing showed a normal 46 XX karyotype.

All other tests at birth were normal.

DERMATOHISTOPATHOLOGY:

No biopsy was done on this baby.

DIFFERENTIAL DIAGNOSIS:

1.   infantile hemangioma
2.   vascular malformation
3.   Kasabach-Merritt syndrome
4.   angioma
5.   Klippel- Trenaunay syndrome

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Infantile Hemangiomas

DISCUSSION:

Infantile hemagiomas manifest at birth in 30% of patients. It is the most common and benign tumor of infancy. Other patients may manifest at 2 weeks to 2 months of age with a pale macule. Usually 1 – 60 mm in diameter hemangiomas are found on the head and neck (60% of cases) though may present anywhere on the body. Rarely an entire extremity is involved.

Clincally the lesion appears as a dull red dome shaped lesion, with sharp borders and is easily compressable. When involution occurs one may see streaks or islands of white. Hemangiomas tend to grow over the first year of life then stabilize before involuting. 50% will involute by 5 years of age and 70% by 7 years of age. Depending on size and duration of the hemagioma the patient may be left with atropy, telangiectasias and an anetoderma type redundancy.

Malformation are associated with with 7% of hemagiomas and may include such syndromes as PHACES and Kippel-Trenaunay Syndrome. PHACES is associated with posterior fossa malformations, hemangiomas, arterial malformations, coartation of the aorta and eye abnormalities. Kippel-Trenaunay Syndrome has a triad of nevus flammeous (confined to skin extremity), varicose veins (venous malformations) and soft tissue hypertrophy of the affected limb.

Multiple Hemangiomas, ranging in size from 1-10 mm, are referred to as benign neonatal hemagiomatosis. Diffuse Neonatal Hemangiomatosis has a poor prognosis and involves the viscera including CNS, lung and liver. These lesions can lead to complications including GI or CNS bleeding, and respiratory failure.

Histologically hemangiomas resemble primitive endothelial cells though they lack Weibel-Palade bodies. Crystalloid inclusions typical of embryonic endothelium are appreciated. There is a sequence of events that may be appreciated as the intralumin proliferates, flattens and the lumen becomes more apparent. Once fibrosis begins the lumen shrinks and involution progresses.

TREATMENT:

Hemangiomas should be treated aggressively if they hemorrhage, ulcerate, thrombose, compromise airways or vision. Early intervention for comestic results is important especially for school-age children with lesions on the face. Cryotherapy or laser ablation of early lesions are unaffective. Pulse dye lasers (PDL) helps with residual telangiectasia. Surgery is an excellent option for small pedunculated lesions or patients with the Cyrano defect. Cyrano defects are those hemangiomas at the tip of the nose that have become bulbous. In addition, compressive wraps may improve the outcome of hemangiomas on an extremity.

Treatment if not surgical may include intralesion steroids or oral prednisone. Hemangiomas stabilize (stop growing) in 30% of patients on 2-3mg/kg/day of po prednisone within 3-21 days. Ulcerated lesions take 2 weeks to respond. Lesions go on to involute at approximately 30-90 days of po treatment. Repeat courses may be required with rebound growth. Maintance with low dose prednisone for 1 year may prevent rebound. 30% of patients don’t respond to po prednisone.

Recombinant interferon alpha 2a or 2b has been successful in 80% of patients on 1-3 million units/m2/day. Responses may take 6-10 months. Side affects include thyroid dysfunction and neurotoxicity (spastic diplegia).

REFERENCES:

1. Andrews Diseases of the Skin, 9th Edition. pages 740, 750-751

Additional Comment:

© Copyright 2003-2006 by AOCD Grand Rounds

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