Craniofacial Dysostosis (Crouzon's Syndrome)

Crouzon's craniofacial dysostosis in Kenya.

A 5-year-old child with Crouzon's disease presented with luxation of the eyeballs and advanced endophthalmitis as a result of which both the eyes had to be enucleated. This case led us to study the family pedigree. The factors influencing the pattern of clinical presentation, and hence the ultimate treatment of orbital disease, are discussed. The case is presented to emphasise the different con...

The dentist’s role in diagnosing craniofacial dysostosis

Introduction: The Crouzon syndrome is a rare clinical condition that affects the craniofacial skeleton development. It accounts for about 4.8% of all the cases of craniosynostosis, and it is the most common syndrome presenting with craniosynostoses. The Crouzon syndrome’s early diagnosis is critical to avoid cranial hypertension as well as visual disturbances and blindness. Children who have Cr...

Breathing patterns in children with craniofacial dysostosis and hindbrain herniation.

In the past few years, hindbrain herniation has become recognized as a frequent feature of the child with syndromic craniofacial dysostosis. The clinical significance of hindbrain herniation in these disorders is unclear. Abnormalities of respiratory control have frequently been reported. The aim of the present study was to document the incidence of obstructive and central respiratory abnormali...

Postaxial acrofacial dysostosis (Miller) syndrome.

Mandibulo-facial dysostosis (Treacher Collins syndrome).

Mandibulo-facial dysostosis has been described with increasing frequency in recent years. Once known it is readily recognized and may be more common than is sometimes imagined. We describe in this article a case seen in a newlyborn infant who died at the age of 21 months and in whom careful dissections were made of the facial region. The infant (A.R., 456/1954) was admitted to the Royal Aberdee...