Rare Facial Clefts - Clinical Findings and Surgical Management

Objectives: The wide variation of craniofacial clefts, their complexity as well as the rarity of these conditions make classification difficult and require adjusted surgical strategies. Among the different structures involved in the cleft formation the orbit and the cranial base need special consideration as in most of these cases development of encephalomeningoceles results. The aim of this study was to evaluate the short and long term outcome after early and staged surgical treatment.

Patients and Methods: 62 children with rare facial clefts (32 female, 30 male) were evaluated according to the extension of their cleft formation and the individual surgical management. Seven out of 62 suffered from a combination of different clefts based on the Tessier classification. In 25 cases the neurocranium was involved in the cleft formation.

Results: Depending on the extension of the cleft formation the surgical management was based on three major requirements:(1): Establishment of normal eye-lid function to preserve visual acuity, (2): Repair of perioral structures to achieve oral competence. (3): Closure of the bony gap to the neurocranium to avoid a relapse of the encephalomeningoceles after their removal.
In order to deal with soft tissue deficiency local flaps, different patterns of rotation flaps were mostly used.
The decision management, technical notes as well as dealing with side effects of the surgical treatment and long term results will be presented in detail.

Conclusion: The presentation focuses on the clinical findings, classification and outcome after surgical treatment of rare craniofacial cleft cases. The soft tissue repair mainly aims for improvement of eyelid function and perioral repair to achieve oral competence. The surgical management allowed definitive treatment of the encephalomeningoceles and improvement of function and aesthetics but long term effects due to scar contracture and further growth of the children have to be considered.