Triad of blepharophimosis, blepharoptosis, and
epicanthus inversus. Occasional presence of spinal bifida occulta, cranial
asymmetry, occipital bone flattening, and cleft lip/palate.
Oculopalatoskeletal Syndrome; Clefting-Ocular Anterior
Chamber Defect-Lid Anomalies Syndrome.
Eyelid traits are usually blepharophimosis,
blepharoptosis, and telecanthus inversus, plus a developmental defect of the
anterior segment of the eye. Craniosynostosis with cranial asymmetry,
skeletal defects, hearing deficit, cleft lip and palate, and mild mental
retardation; hydronephrosis is described.
Creatinine and blood urea nitrogen
should be obtained. The presence of raised intracranial pressure cannot be
excluded in presence of craniosynostosis. It is reported that in presence of
a one-suture fusion, the intracranial pressure is elevated in 18% of patients
and when two or more are involved, 40% of patients have an increase in
Sculerati N, Gottlieb MD, Zimbler MS, et al: Airway management in
children with major craniofacial anomalies. Laryngoscope