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[Pancraniofacial synostosis--indications for early craniofacial operation]
P Heeckt1, W Mühlbauer, J Fairley
1Abteilung für Plastische, Wiederherstellende und Handchirurgie, Städtisches Krankenhaus München-Bogenhausen.
Handchirurgie, Mikrochirurgie, Plastische Chirurgie : Organ Der Deutschsprachigen Arbeitsgemeinschaft Fur Handchirurgie : Organ Der Deutschsprachigen Arbeitsgemeinschaft Fur Mikrochirurgie Der Peripheren Nerven Und Gefasse : Organ Der V
|March 1, 1994
Summary
Pancraniofacial synostosis (PCS) requires early surgery for severe cases, improving outcomes. Emergency operations in infants carry high risks, while elective surgery at 3-12 months is safer and effective.
Area of Science:
- Craniofacial Surgery
- Pediatric Neurosurgery
- Plastic Surgery
Background:
- Premature fusion of all major cranial sutures, termed pancraniofacial synostosis (PCS), is a rare condition.
- PCS often presents with syndromic features like Kleeblattschädel-syndrome and affects both cranial vault and facial skeleton.
- Surgical intervention is frequently necessary in infancy for vital indications.
Purpose of the Study:
- To present the results and complications of surgical management in children with PCS.
- To evaluate the safety and efficacy of early craniofacial reconstruction in PCS.
- To compare outcomes between emergency and elective surgical approaches.
Main Methods:
- Retrospective review of 12 children with PCS.
- Subtotal craniectomy and fronto-orbito-facial skeleton advancement in 11 patients.
- Preoperative shunting for hydrocephalus and emergency surgery for impending vision loss in 4 infants.
Main Results:
- Emergency surgery in 4 infants resulted in lethal complications in 3 cases.
- Elective craniofacial surgery in 11 children significantly improved intracranial hypertension, exorbitism, airway obstruction, and aesthetics.
- One child managed non-surgically showed no complications over ten years.
Conclusions:
- Total craniofacial correction for PCS can be safely performed between 3 and 12 months of age.
- Emergency surgery in PCS is associated with significantly higher risks.
- Simultaneous mid-face advancement should be cautiously considered in emergency PCS cases.