The Morning Glory syndrome associated with sphenoethmoidal encephalocele.
A 10-month-old infant was referred to the ophthalmic department of the Royal Victoria Hospital for assessment. Pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.
Department of Ophthalmology, Royal Victorial Hospital, Belfast, Northern Ireland.
M
M
Hope-Ross
S S
SS
Johnston
D000015:Abnormalities, Multiple; D061085:Agenesis of Corpus Callosum; D002971:Cleft Lip / Q000150:complications / Q000601:surgery; D003103:Coloboma / Q000150:complications*; D004677:Encephalocele / Q000150:complications* / Q000601:surgery; D005145:Face / Q000002:abnormalities; D006801:Humans; D007223:Infant; D008297:Male; D009898:Optic Disk / Q000002:abnormalities*; D013577:Syndrome