Prader-Willi Syndrome: A Case Report

Erika Tayal*, KR Indushekar, Bhavna G. Saraf, Neha Sheoran and Ashima Doda

Prader-Willi Syndrome: A Case Report.

The clinical features of the Prader–Willi Syndrome (PWS) are complex, variable and characterized by severe neonatal hypotonia, feeding problems, childhood-onset hyperphagia, obesity, short stature, cranio facial disharmony, hypogonadism, learning and behavioral difficulties (because of hypothalamus dysfunction). Clinical features may alter with age. Scoliosis, hypopigmentation of skin and hair, juvenile diabetes, narrow forehead, sleep apnoea (because of obesity), delayed healing, small hands and feet and self inflicted injuries are some of the other characteristic features of this syndrome.

The worldwide incidence is between 1:15000 and 1:50000. It usually affects both males and females equally with a large preponderance for boys. Oro-facial manifestations reported in PWS include almond-shaped eyes with up-slanting palpebral fissures and a triangular mouth. Intraoral findings include the presence of hypoplastic enamel, rampant caries, low basal salivary secretion, delayed tooth eruption and excessive tooth wear.

Early diagnosis and thorough orofacial examination of such cases is important to optimize treatment planning and management and to minimize the risk of progression of developing symptoms. Once the diagnosis of syndrome is confirmed, dental consultations should be started, in which education of the parents on the possible dental problems and instructions on how to prevent them should be given. Early introduction of good dietary and oral hygiene practices and
use of fluoride supplements, when appropriate, would help prevent the deleterious dental consequences, as experienced here in this case report. Early interventions also include physical
and occupational speech therapy. Therapeutic interventions to manage growth, regular exercises, dietary and behavioral concerns can enhance the child’s potential and can cause a significant impact on health.

Public Health Open J. 2016; 1(1): 12-15. doi: 10.17140/PHOJ-1-104

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