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Russell-Silver Syndrome: Anaesthetic Implications and Management

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127–29

INTRODUCTION

The Russell-Silver Syndrome (RSS) is a form of dwarfism characterized by intrauterine growth retardation with subsequent severe postnatal growth impairment (ie low-birthweight dwarfism). The characteristic features have been classified as universal, common and uncommon (1). Universal features include cranio-facial disproportion, facial dysmorphia, lateral asymmetry and incurved fifth digit (clinodactyly).
The typical facial features are frontal bossing, triangular face with hypoplastic mandible and a downward incurving of the mouth, the so-called “shark” mouth (2). Endocrine abnormalities are common traits and include hypoglycaemia, hypopituitarism, adrenal insufficiency and various patterns of hypogonadism (3–6).

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e-Published: 10 Jun, 2013
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