A Surgical Management Algorithm for Type 4 Parry-Romberg Syndrome

J Craniofac Surg. 2023 Oct 10. doi: 10.1097/SCS.0000000000009780. Online ahead of print.

Abstract

Background: Type 4 Parry-Romberg syndrome (PRS) affects multiple facial esthetic units involving many tissue types, requiring both bone and soft tissue restoration and requiring different surgical procedures over a long period of time. To date, the timing and sequence of these operations remains controversial.

Methods: A retrospective analysis was performed to introduce our surgical management algorithm for type 4 PRS and to evaluate its safety and efficacy according to long-term follow-up results.

Results: Six Chinese patients (5 females and 1 male) fulfilled the inclusion criteria. The follow-up period ranged from 23 to 90 months (mean 51.83 mo). All patients were satisfied with the improvement in facial appearance after staged bone and soft tissue reconstruction. No major complications were reported except for 1 case of infection after Medpor implantation.

Conclusions: For severe PRS, both bone and soft tissue reconstruction is recommended. Enlargement of the bony framework provides a good foundation for subsequent soft tissue restoration. Alloplastic implants in the maxilla can be problematic if the patient has undergone either a zygomatic or maxillary osteotomy. The anterolateral thigh adipofascial flap is a reliable choice for large soft tissue deficits in type 4 PRS. We hope that reporting our surgical management algorithm with long-term follow-up results will improve the personalized treatment of these patients.