Orbitofrontal monobloc advancement for Crouzon syndrome

Area of Science:

• Craniofacial surgery
• Orthognathic surgery
• Pediatric plastic surgery

Background:

• Crouzon syndrome often presents with coronal suture synostosis, exophthalmia, hypertelorism, and midface hypoplasia.
• Standard frontofacial monobloc advancement can exacerbate maxillo-mandibular dysmorphia in patients with class I or II occlusion.
• A modified approach, orbitofrontal monobloc advancement minus the dental arch, is proposed to mitigate these issues.

Observation:

• A 12-year-old girl with Crouzon syndrome underwent orbitofrontal monobloc advancement without splitting pterygoid plates.
• The procedure utilized four KLS Martin distractors, achieving 10.2 mm cranial and 10.5 mm fronto-zygomatic advancement.
• Distraction devices were removed after 8 months.

Findings:

• The modified orbitofrontal monobloc advancement successfully corrected craniofacial deformities while preserving the maxillary dental arch.
• This technique avoids the need for pterygoid plate splitting, simplifying the procedure.
• The patient experienced significant advancement without worsening dental occlusion.

Implications:

• This modified technique offers a valuable alternative for Crouzon syndrome patients with class I or II occlusion, preventing iatrogenic maxillo-mandibular dysmorphia.
• It highlights the importance of considering dental occlusion in surgical planning for craniofacial syndromes.
• Further research may explore long-term outcomes and applicability in diverse patient populations.