Anesthetic Management of Cleft Palate Associated with Meier-Gorlin Syndrome

Abstract

Meier-Gorlin syndrome (MGS) is a very rare autosomal recessive primordial dwarfism disorder, characterized by microtia, patellar aplasia/hypoplasia, and a proportionate short stature. Typical facial characteristics during childhood comprise a small mouth with full lips and micro-retrognathia. Presence of cleft palate in these patients is usual condition. Due to associated congenital pulmonary emphysema and sudden cardiac death during anesthesia these patients present major perioperative challenges to anesthesiologist. We report successful anesthetic care in a 4-year-old male child diagnosed with Meier-Gorlin syndrome and admitted for repair of cleft palate. Because of dysmorphic features, we faced difficulties during intubation. Being a multisystem disorder, each patient of Meier-Gorlin syndrome requires meticulous preoperative evaluation and high level of intraoperative and postoperative continuous monitoring regardless of any surgical procedure. Presented case report highlights the significance of aggressive perioperative management in MGS which can result in successful outcome. As for additional features, it is worth to note that in our patient we didn't reveal short stature and patellar anomalies.