Monoblock craniofacial internal distraction in a child with Pfeiffer syndrome: a case report
Jaiho Chung1, Dong Ha Park, Soo Han Yoon
1Department of Neurosurgery, Ajou University School of Medicine, San 5 Wonchon-dong, Yongtong-gu, Suwon, Korea.
Insights
Internal monoblock distraction osteogenesis effectively treated Pfeiffer syndrome type 1 in a child. This craniofacial surgery expanded airway spaces and improved aesthetic and functional outcomes.
Area of Science:
- Craniofacial Surgery
- Pediatric Orthodontics
- Genetics
Background:
- Pfeiffer syndrome type 1 presents with complex craniofacial abnormalities impacting airway, aesthetics, and development.
- Early intervention is crucial for managing syndromic craniosynostosis and its associated complications.
Observation:
- A 7-year-old boy with Pfeiffer syndrome type 1 exhibited macrocrania, midfacial retrusion, airway obstruction, and developmental delay.
- Symptoms included exophthalmos, tongue protrusion, and malocclusion, alongside anthrophobia.
Findings:
- Internal monoblock distraction osteogenesis successfully increased intracranial, intraorbital, and airway volumes.
- Computed tomography confirmed brain expansion and proper ossification 12 months post-surgery.
- The patient demonstrated improved aesthetics, reduced protrusion, resolved respiratory difficulty, and enhanced learning.
Implications:
- Internal distraction osteogenesis offers a viable alternative to external methods for craniofacial expansion.
- This technique may lead to superior bone fusion rates and more successful craniofacial reconstruction in pediatric patients.
- Improved craniofacial volume and airway patency positively impact neurodevelopmental outcomes.
Abstract:
A 7-yr-old boy visited our surgical center with Pfeiffer syndrome type 1, presenting with macrocrania, broad big toe and thumb, exophthalmos, tongue protrusion, malocclusion with midfacial retrusion, mild respiratory difficulty due to minor upper airway obstruction, and developmental delay. He also exhibited anthrophobia with a passive character. The patient was treated with internal monoblock distraction osteogenesis to increase the intracranial and intraorbital volumes, and the nasal and pharyngeal airway spaces using two modular mid-facial internal distractors. For distraction, the latency period was 1 week, the daily activation of 1.0 mm was 20 days (total advancement 20 mm at the midline), and the consolidation period was 3 months. The follow-up computed tomography 12 months after surgery showed expansion of the brain and proper ossification in the distracted area. The patient also showed aesthetically good cranial contours, improved tongue and eyeball protrusion, no respiratory difficulty, and improved learning. We suggest that the internal distraction may last longer than an external type, resulting in a better bone fusion rate and successful expansion of craniofacial bones.
