Craniofacial fasciitis of the face: Case report and review of the recent literature.

INTRODUCTION

Craniofacial fasciitis (CFFF) an uncommon, benign pseudosarcomatous proliferation of unknown etiology that occurs almost exclusively in children less than 6 years old. CFF lesions occur most frequently in the deep fascial layers and the periosteal layer of the calvarium, resulting in a rapidly enlarging scalp mass with potential underlying bony erosion. Presentation of CFF on the midface is rare.

OBJECTIVE

The objective of this report is to describe a case of paranasal CFF involving the nasolacrimal duct in an infant and perform a literature review of cases of CFF of the midface in children.

METHODS

A literature review of children ≤18 years old who were diagnosed with CFF of the midface was performed. A single case report is presented.

RESULTS

A 7-month-old girl presented with a rapidly enlarging facial mass with edema, epiphora, difficulty breathing, and issues with feeding. She underwent urgent biopsy which revealed spindle cells with fibrous background. Patient was diagnosed with paranasal CFF based on clinical presentation and pathology report. She subsequently underwent near complete surgical excision. Patient is doing well with no progression of disease with follow up MRI revealing minimal residual disease in the nasal cavity. Review of the literature identified 4 additional cases of CFF of the midface which presented on sites including the mandible, frontonasal region, nasal process of the maxilla, and orbit/maxilla. The patient in this case is the first presentation of CFF involving the nasolacrimal duct. Lesions presented anywhere from 2 days to 3 months prior to treatment, and immunohistochemistry was positive for either vimentin or beta-catenin. Interestingly, all cases in this series occurred within the first year of life and were treated with complete or near complete resection with no evidence of recurrence.

CONCLUSION

Although CFF is a rare diagnosis, it should be considered in the evaluation and treatment of soft-tissue masses of the midface in children. Surgical excision is curative and there appears to be a low rate of recurrence based on the small series presented in literature.