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This article examines the relationship between pediatric obstructive sleep apnea syndrome in Apert syndrome and congenital structural abnormalities of the respiratory system and skull. It reviews both invasive and non-invasive treatment methods for pediatric obstructive sleep apnea syndrome. The analysis includes various perspectives on the appropriate age for implementing invasive therapies in Apert syndrome.

This report describes an especially rare postoperative pseudoaneurysm of the sphenopalatine artery following midface advancement using Le Fort III in an Apert syndrome patient. The Apert patient referenced in this report was a 13-year-old boy with severe midface hypoplasia, significant obstructive sleep apnea, and Angle Class III malocclusion. The patient underwent a low Le Fort III osteotomy, subsequently followed by gradual anterior distraction with an external halo distractor. One month postoperatively, the patient experienced recurrent, massive blood loss due to unilateral epistaxis. In order to immediately address life-threatening hemorrhaging and maintain advanced segment stability, the external distractors were removed, and a custom protraction face mask was applied for retention. A computerized tomography angiography showed a 4×3 mm saccular pseudoaneurysm from a branch of the left sphenopalatine artery in the pterygopalatine fossa near the posterior maxillary sinus wall. Careful microcatheterization and embolization achieved complete occlusion with preservation of adjacent collateral flow, resulting in immediate and sustained cessation of epistaxis, hemoglobin stabilization, and recovery without further complication. After 12 weeks of protraction face mask retention, achieved midface advancement remained stable; there was no recurrent bleeding over the next 6 months, and the patient's occlusion was classified as Angle Class II. This case underscores that recurrent severe postoperative epistaxis occurring after Le Fort III distraction requires immediate computerized tomography angiography to detect pseudoaneurysm and supports endovascular embolization as a safe, minimally invasive, highly effective first-line therapy within a multidisciplinary care model.

Apert syndrome is a rare genetic disorder characterized by premature fusion of the cranial sutures, syndactyly of the extremities, and distinct craniofacial deformities. The condition results from mutations in the FGFR2 gene, which disrupt normal craniofacial growth and lead to complex functional and morphological abnormalities. Patients with Apert syndrome commonly present with stomatognathic abnormalities, which significantly affect oral function and facial development. The management of Apert syndrome requires a multidisciplinary therapeutic approach. Surgical treatment strategies are typically staged according to the patient's age and clinical severity. Early interventions focus on cranial vault expansion procedures, such as fronto-orbital advancement and posterior vault distraction osteogenesis, aiming to relieve intracranial pressure and improve cranial morphology. During childhood and adolescence, midface advancement techniques are commonly performed to address midfacial hypoplasia and associated functional impairments. Early diagnosis and appropriate surgical planning play a crucial role in preventing complications and improving the functional, aesthetic, and psychosocial outcomes of patients with Apert syndrome. This narrative review summarizes current evidence while highlighting areas of ongoing controversy, particularly regarding surgical sequencing, orthodontic management and the integration of digital technologies into multidisciplinary care.

BackgroundSyndromic midface hypoplasia often includes a high or fragile palate that is vulnerable during midfacial disimpaction.SolutionWe created a patient-specific palatal protection plate produced through a fully digital workflow to stabilize and protect the palate.What we didAfter using conventional plates from 2022 to 2024, we implemented a digital design and printing workflow in 2025 and added suction-catheter sleeves to the Rowe forceps branches to improve retention.