Very rare congenital disorder characterized by the
association of strabismus, nonrhegmatogenous retinal detachment, or remnants
of the hyaloid system of the eyes. Basal encephalocele has been encountered
in a few cases. The CHARGE (coloboma of iris, heart deformities, choanal
atresia, retarded growth, genital and ear deformities) association or isolated
congenital defects have been demonstrated with this condition.
Unknown; females affected twice
as often as males.
Funnel-shaped, excavated optic disc surrounded by
chorioretinal pigmentary anomalies; it is generally unilateral and isolated
but has been observed in association with midline facial defects (cleft lip
or palate, hypertelorism, basal meningomyelocele, agenesis of corpus
callosum), renal anomalies, the CHARGE association, and isolated cardiac
defects (i.e., atrial septal defect [ASD], ventricular septal defect [VSD], and patent ductus arteriosus [PDA]). In a few cases, basal encephalocele was also
associated with the defect.
Check for associated anomalies, mainly
airway and cardiac malformations. Anesthetic management is dictated by
associated anomalies; one case of unexpected difficult tracheal intubation
has been reported. The presence of a basal encephalocele might complicate
airway management and positioning. Cardiac anomalies may be present and have
their own considerations.
Eustis HS, Sanders MR, Zimmerman T: Morning glory syndrome in children.
Association with endocrine and central nervous system anomalies. Arch Ophthalmol 2:204,
Shevchenko Y, Rehman M, Dorsey AT, et al: Unexpected difficult intubation in
the patient with morning glory syndrome. Paediatr Anaesth