Hamartomatous dysplasias with hemihypertrophy. 2 cases including one with Proteus syndrome

Hamartomatous dysplasias with hemihypertrophy are a part of several congenital diseases, sometimes difficult to classify when their symptomatology is relatively poor. Case 1. A girl, 11 year-old, suffered from gigantism of the feet, left hemihypertrophy of the limbs, scoliosis and kyphosis. X-rays s...

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Bibliographic Details
Published in:Archives de pédiatrie : organe officiel de la Société française de pédiatrie 1995-01, Vol.2 (1), p.52-56
Main Authors: Delaporte, E, Wendehenne, R, Descamps, S, Bitbol-Duneton, V, Hatron, P Y, Piette, F, Bergoend, H
Format: Article
Language:fre
Subjects:
Adolescent
Child
Extremities - pathology
Female
Hamartoma Syndrome, Multiple - complications
Hamartoma Syndrome, Multiple - diagnosis
Humans
Hypertrophy
Klippel-Trenaunay-Weber Syndrome - diagnosis
Proteus Syndrome - diagnosis
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Summary:Hamartomatous dysplasias with hemihypertrophy are a part of several congenital diseases, sometimes difficult to classify when their symptomatology is relatively poor. Case 1. A girl, 11 year-old, suffered from gigantism of the feet, left hemihypertrophy of the limbs, scoliosis and kyphosis. X-rays showed frontal hyperostose, bony and soft tissues overgrowth involving the left limbs and pelvis with hip dislocation and coxa valga. The vertebrae were hypertrophic and dysplastic. No tumor could be found. Case 2. A girl, 18 year-old, suffered from left hemihypertrophy of limbs and face associated with homolateral cutaneous hemangioma. X-rays showed enlargement of bone and soft tissue. No tumor or venous abnormalities were observed. A diagnosis of Proteus syndrome was made in the first patient while the second patient was considered to have Klippel-Trenaunay syndrome even though hemangiomatous involvement has been seen in Proteus syndrome.
ISSN:0929-693X