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Perioperative care of a patient with Beare–Stevenson syndrome
Author(s) -
UPMEYER SARA,
BOTHWELL MARCELLA,
TOBIAS JOSEPH D.
Publication year - 2005
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2005.01585.x
Subject(s) - medicine , craniosynostosis , choanal atresia , palpebral fissure , hypertelorism , crouzon syndrome , craniofacial , surgery , anatomy , atresia , psychiatry
Summary Beare–Stevenson syndrome is a craniofacial syndrome consisting of a specific pattern of craniosynostosis resulting in a cloverleaf skull deformity and hydrocephalus, down‐slanting palpebral fissures, proptosis, hypertelorism, strabismus, dysmorphic ears, choanal atresia, cleft palate, cutis gyratum, acanthosis nigricans, and abnormal genitalia. Its primary cause has been identified as a single amino acid substitution in fibroblast growth factor receptor 2. Of primary importance to the anesthesiologist are issues related to airway management resulting from midface hypoplasia, choanal atresia, and airway abnormalities (tracheal stenosis). Additional issues affecting airway management include associated cervical spine and foramen magnum abnormalities. The authors present their experience caring for a patient with Beare–Stevenson syndrome and discuss the anesthesia care of these patients.