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Anales de la Sociedad Mexicana de Oftalmología y Archivos de la Asociación Para Evitar la Ceguera en México

2009, Number 3

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Rev Mex Oftalmol 2009; 83 (3)

Síndrome de Shone con encefalocele, anomalía de 'morning glory' pulsátil y estrabismo. Reporte de un caso

Prado-Serrano A, Camas-Benitez JT, Ponce-Martínez JR

Language: Spanish
References: 26
Page: 176-180
PDF size: 189.34 Kb.


ABSTRACT

Shone’s anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supra valvular mitral ring, parachute mitral valve, subaortic stenosis, and coarctation, being the survival rates for the untreated patients generally poor.
The Morning glory syndrome is a birth defect of the optic nerve which includes coloboma of the optic disc. It results in a funnel-shaped optic nerve head with a white dot in the center, an elevated ring of pigment around the disk, and vessels radiating out from the ring like spokes. Vision in the affected eye is severely impaired.
The multi systemic association between cardiac, neurological and ophthalmological features is quite uncommon.
We report a case of a 22 year old male who was diagnosed with incomplete Shone’s anomaly, encephalocele and hiperviscosity syndrome who complained of poor left eye visual function. The ocular evaluation confirmed secondary esotropia, and a congenital optic disc coloboma, most likely a pulse morning glory disc anomaly.


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