Online ISSN: 2515-8260

Case Series: Variable Genetic Expressivity In Crouzon Syndrome- A Comparative Report Of Three Cases

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Dr. Puneeta Vohra1 , Dr. Shaik Rabbani2 , Dr. Archna Nagpal3 , Dr.Vundela Muni Sravani4 , Dr. Gaurav Sharma5 , Dr. Sourabh Ramesh Joshi6 , Dr. Rahul VC Tiwari7

Abstract

Despite the intense research, the clinical manifestation of syndromic craniosynostosis patients remains an enigma. Human skull is made up of various bone joints connected by sutures. The sutures fuse in later life after the complete growth ofthe brain. If any of these sutures close early, it may encumber the normal growth of the brain. In craniosynostosis there are disturbances in the growth of the skull which are caused by premature synostosis at one or more cranial sutures, resulting in typical deformities of the vault called as dyscrania. Crouzon syndrome is an emblematic example of craniosynostosis. Cranial malformations in crouzon syndrome depend on the order and rate of progression of sutural synostosis. Thus, physical appearance varies from patient to patient. In this article we intend to compare two patients with crouzon syndrome and a clinically non syndromic patient with beaten metal appearance radiographically. The idea behind this is that each syndromic patient is distinctive and a separate approach for treatment of such patients is desired.

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