title: PERIOPERATIVE CARE OF AN INFANT WITH GOMEZ-LOPEZ-HERNANDEZ SYNDROME.
abstract: Gomez-Lopez-Hernandez syndrome, also known as cerebello-trigeminal dermal dysplasia, is a rare neurocutaneous syndrome classically characterized by the triad of rhombencephalosynapsis, trigeminal anesthesia, and bilateral parietal alopecia. Associated clinical features include a characteristic facial appearance (mid-face hypoplasia, hypertelorism, and low-set, posteriorly rotated ears), brachycephaly, strabismus, ataxia, developmental delay, short stature, and corneal opacities. Given the associated congenital anomalies, anesthetic care may be required for various surgical interventions. We report a 7-month-old with Gomez-Lopez-Hernandez syndrome scheduled for laparoscopic gastrostomy with tube placement and frenulotomy under general anesthesia. The potential perioperative implications of such patients are reviewed and options for anesthetic care discussed.
authors.forename: Onur
authors.initials: O
authors.lastname: Balaban
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
mesh_terms: D000015:Abnormalities, Multiple / Q000503:physiopathology*; D000505:Alopecia / Q000503:physiopathology*; D000758:Anesthesia; D002531:Cerebellum / Q000002:abnormalities* / Q000503:physiopathology; D019465:Craniofacial Abnormalities / Q000503:physiopathology*; D006130:Growth Disorders / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D020752:Neurocutaneous Syndromes / Q000503:physiopathology*; D019990:Perioperative Care; D012249:Rhombencephalon / Q000503:physiopathology