Pitfall of Monobloc Advancement in Beare-Stevenson Syndrome.

ABSTRACT

Beare-Stevenson syndrome (BSS) is an extremely rare form of craniosynostosis. Half of patients with BSS died within the first year of life due to cardiorespiratory arrest or unexpected sudden death. In addition, there have only been fewer than 30 cases, which limits prognostication for clinicians. We currently have an 8-year-old patient who is being followed up. She underwent tracheostomy, cranioplasty, and shunting. After waiting for growth, for improvement of ocular herniation, monobloc advancement was performed at the age of 5 years. The temporal fossa was quite narrow due to severe midface hypoplasia. In addition, even though midface advancement, it was difficult to remove tracheostomy. This was because midfacial hypoplasia was so severe that it was hard to notice, but there was also micrognathia. We report our experience with this midface treatment.