Table 4.
Various syndromes and their anesthetic and ocular implications
| Syndrome | Ocular Features | Anesthetic implication | ||
|---|---|---|---|---|
| Apert syndrome | Glaucoma, cataract, strabismus, proptosis | Possible difficult intubation and choanal atresia, cervical spine fusion, CHD# | ||
| Crouzon syndrome | Glaucoma, cataract, strabismus and proptosis | Possible difficult intubation and elevated intracranial pressure | ||
| Cystinosis | Corneal opacities, retinal degeneration | Chronic renal failure, diabetes mellitus, esophageal varices, hyperthermia | ||
| Downs syndrome | Cataract, strabismus | Airway obstruction, atlantoaxial instability, CHD, more sensitive to atropine | ||
| Ehlers's Danlos syndrome | Retinal detachment, blue sclera, ectopia lentis, keratoconus | Laryngeal trauma possible with intubation, careful positioning, avoid arterial and central lines | ||
| Goldenhar syndrome | Glaucoma, cataracts, dermoids, lacrimal drainage defects | Hemifacial microsomia, possible cervical spine abnormalities, possible difficult intubation, and mask ventilation | ||
| Hallermann-Strieff syndrome | Congenital cataracts, coloboma, microphthalmia, glaucoma | Major craniofacial abnormalities with likely difficult intubation, upper airway obstruction, chronic lung disease | ||
| Hunters’ syndrome | Retinal detachment, optic atrophy | Often difficult intubation, copious secretions, macroglossia, Stiff temporomandibular joint, limited neck mobility, Possible ischemic and valvular heart disease | ||
| Hurlers syndrome | Corneal clouding, retinal degeneration, optic atrophy | Often difficult intubation and mask ventilation, possible cervical spine instability, possible ischemic or valvular heart disease | ||
| Juene syndrome | Retinal degeneration | Limited thoracic excursion, pulmonary hypoplasia, possible renal and hepatic insufficiency | ||
| Lowes syndrome | Cataracts, glaucoma | Renal failure, renal tubular acidosis | ||
| Marfan syndrome | Ectopia lentis, glaucoma, retinal detachment, cataract | Aortic or pulmonary root dilatation, mitral valve prolapse, pectus excavatum, risk for pneumothorax | ||
| Myotonic dystrophy | Cataract, ptosis, strabismus | Prone to myotonic and succinylcholine induces contractions, cardiac conduction abnormalities, sensitive to CNS depressants | ||
| Rubella spectrum | Cataract, microphthalmia, optic atrophy, glaucoma, | Neonatal anemia, pneumonia, thrombocytopenia, CHD, hypopituitarism | ||
| Sticklers’ syndrome | Vitreous degeneration, retinal detachment, cataract | Possible difficult intubation, micrognathia, pulmonary hyperplasia, CHD | ||
| Sturge–Weber syndrome | Choroidal hemangioma, glaucoma | Airway angiomas, CHD, High output failure, seizure disorder, Hyperkalemic response to succinylcholine | ||
| Treacher collins syndrome | Lid defects, microphthalmia, dermoids | Often difficult intubation, mandibular hypoplasia, CHD | ||
| Turners’ syndrome | Ptosis, strabismus, cataracts, corneal scars, blue sclera | Possible difficult intubation and IV access, CHD | ||
| Von Hippel-Lindau syndrome | Retinal hemangioma | Possible increased intracranial pressure | ||
| Von Recklinghausen's disease | Ptosis, proptosis, optic nerve glioma, meningioma, optic atrophy, glaucoma, lisch nodules | Possible difficult mask ventilation and intubation, possible airway tumors, restrictive lung disease, renovascular hypertension, sensitivity to neuromuscular blockers | ||
| Zellweger syndrome | Glaucoma, cataracts, optic atrophy, optic nerve hypoplasia | Micrognathia, possible CHD, renal and adrenal insufficiency | ||
| CHD: Congenital heart disease, CNS: Central nervous system | ||||