Crouzon syndrome and the eye: An overview

Abstract

The current literature review aims to evaluate the ocular findings and associated ophthalmic features in Crouzon syndrome. Craniosynostoses are syndromes characterized by premature fusion of sutures of the skull and Crouzon syndrome is the most common of the craniosynostosis syndromes. Early fusion of sutures results in craniofacial anomalies, including abnormalities of the orbits. To prepare this review of the ophthalmic findings in this disorder, an organized search on online databases such as PubMed, Scopus, Cochrane Library, and Ovid was carried out. The key terms searched were “Crouzon”, “craniosynostosis”, “eye” and “ophthalmic”, and 51 research items were found. A total of 17 articles were included after scrutiny of the databases and a further 25 articles were added after augmented search. A detailed review was performed from the final 42 articles. A comprehensive description of associated anomalies is given along with the author’s own technique of surgical management in cases with Crouzon syndrome having bilateral luxation bulbi with exposure keratopathy. However, for optimum management of cranial and oculo-facial dysmorphisms, a multidisciplinary team of specialists is required.

Dynamic Morphological Growth Trajectory of Crouzon Population: A Geometric Morphometric Analysis

“…Of note, the cohort in this study ranged from infanthood to adulthood, allowing comprehensive and chronological development. Our study suggested that maxillary retrusion rapidly aggravated during the 3 to 6 years old, which corroborates with what Lu et al found, confirming that therapeutic interventions, including surgical advancement 22 or nonsurgical orthodontic maxillary expansion 23,24 should be initiated to slow pathologic deterioration. The delay of mandibular rapid aggravation indicates mandibular counterclockwise rotation might be triggered by maxillary retrusion and the covariation of maxillary and mandibular morphological variation should be further investigated to determine whether intervention should be enforced on the mandible independently or not.…”

First Arch Syndrome: A Case Report on Crouzon Syndrome

“…A new technique, craniofacial disjunction, followed by gradual bone distraction (Ilizarov procedure) can be carried out to correct exophthalmos and improve the functional and esthetic aspects of the middle third of the face without requirement for bone graft in patients aged 6-11 years. [5,6]…”

“…Proper treatment plan by multidisciplinary approach utilizing modern methods will achieve good outcome and improve quality of patient's life. [5,6]…”

“…[4,5]It represents the most common syndromic craniosynostosis and is caused by premature obliteration and ossification of two or more sutures, most often coronal and sagittal resulting in brachycephaly, midface hypoplasia and wider anterior skull base. [6] Other anomalies include shallow orbit, maxillary hypoplasia, and occasional upper airway obstruction. [7] Besides these features, it is characterized by hypertelorism, exophthalmos, strabismus, beaked nose, short upper lip and relative mandibular prognathism with no digital abnormalities.…”

Mysterious Bilateral Foot Pain in a Child With Crouzon Syndrome

“…Surgical management typically involves several phases targeting specific structures. The first phase usually involves remodeling the fronto-orbital area and releasing stenotic sutures so that proper brain growth can occur [ 7 , 11 ]. Once the child reaches late adolescence, corrections including midfacial, orthodontic, and orthognathic surgery are performed to ensure a more normal cosmetic appearance [ 7 , 11 ].…”