Page 21 - SPD Review Winter 2014

Literature Review - Winter 2014

Babajanyan A, Nanan R, Liu A. Isolated gigantism of digits. J Pediatr. supericial and mixed, but not purely deep. The authors called hemangiomas

2014;165(4):874.
that involved multiple digits but excluded the distal portion of the digits the

“biker-glove” pattern; this was seen in 73% of the cases. Hemangiomas with

Isolated macrodactyly is a rare, nonhereditary, congenital malformation pre- minimal or arrested growth (IH-MAG) accounted for 38% of cases, and over

senting with localized, asymmetric gigantism caused by ibroadipose tissue half of these had a reticular morphology. All 24 cases that were complicated

hypertrophy of one or many digits or even an entire limb. The authors report (ulcerated or required treatment) were segmental and 50% of these cases were

a term newborn with isolated gigantism of the right second and third toes in- reticular IH-MAG. The authors note reticular IH-MAG had a higher risk of ul-

volving the surrounding soft tissues and metatarsal bones. Other physical exam ceration, were the cases in their series with associated structural anomalies

indings and family history were unremarkable. The cause of the condition is (LUMBAR and PHACE syndrome in 5 patients), and more commonly required

unknown, but one researcher suggested mosaicism as well as activation of the therapy. They predict age of development of the hemangiomas during gestation

P13K/AKT pathway as is found in other overgrowth syndromes. This case high- based on pattern and posit differing oxygen demands of tissues.

lights the differential diagnosis of macrodactyly: neuroibromatosis type I, ibro- (Submitted by Carrie C. Coughlin, MD)

lipomatous hamartoma, lymphangiomatosis, Ollier disease, Klippel-Trenaunay,

Mafucci, Proteus, and CLOVE syndromes. As in this case, diagnosis of isolated Waelchli R1, Aylett SE, Robinson K, et al. New vascular classiication of port-

macrodactyly is made when there is no family history suggestive of overgrowth wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol. 2014;

syndrome or other cutaneous or systemic manifestations associated with it. 171(4):861-7.

(Submitted by Maria Elena Miyar, MD)

This study suggested that facial port-wine stain (PWS) distribution follows the

embryonic vasculature of the face, rather than the trigeminal nerve. Investiga-

 VASCULAR LESIONS
tors looked at 192 children with a facial PWS over a 2 year period and mea-

sured their clinical (i.e. seizures, abnormal neurodevelopment, glaucoma) and

Weitz NA, Bayer ML, Baselga E, et al. The “biker-glove” pattern of segmen- radiological adverse outcomes. The best predictor of adverse outcomes was a

tal infantile hemangiomas on the hands and feet. J Am Acad Dermatol. 2014 PWS involving any part of the forehead, delineated at its inferior border by a line

Sep;71:542-547.
joining the outer canthus of the eye to the top of the ear, and including the upper

eyelid. Although this anatomically involves all three divisions of the trigeminal

This multicenter (4 sites, including patients in the United States and Spain), nerve, it corresponds well to the embryonic vascular development of the face.

retrospective study characterizes the pattern, location, and complications in Bilateral distribution was not an independently signiicant phenotypic feature.

infantile hemangiomas on the hands and feet. These hemangiomas (n=73) were
(Submitted by Joseph Lam, MD)

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