Abstract

Pycnodysostosis is a sclerosing skeletal dysplasia characterized with short stature, osteosclerosis, frequent fractures, acroosteolysis of the distal phalanges, clavicular dysplasia, and skull deformities with delayed suture closure. In this paper a 10-year-old boy with pycnodysostosis is reported. He had typical facial features, short stature, open fontanel, short stubby hands, high arched palate as well as radiological features including open cranial sutures, obtuse angle of mandible, and generalized skeletal sclerosis. We think that even pycnodysostosis is infrequent; it may be one from the causes of delayed open fontanel. The making of differential diagnosis of pycnodysostosis is important in the prognosis of disease and determining of treatment

Keywords: Pycnodysostosis, dwarfism, open fontanel

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Copyright and license

How to cite

1.
Sert A, Atabek ME, Pırgon Ö. DELAYED OPEN ANTERIOR FONTANEL IN A 10-YEAR-OLD BOY: PYCNODYSOSTOSIS. Turk J Pediatr Dis [Internet]. 2007 Jun. 1 [cited 2025 May 24];1(1):33-7. Available from: https://turkjpediatrdis.org/article/view/6