Hitno stanje u pedijatriji neočekivanog uzroka - infantilna fibromatoza - prikaz slučaja

Abstract

Introduction: Infantile fibromatosis (IF) is a rare benign mesenchymal tumor of early childhood , located solitarily or multicentrically in the skin, soft tissues, muscles, bones, or visceral organs. The cause is unknown, and some cases are linked to mutations in two different genes. Rapid growth is typical, and while there are reports of spontaneous regression, relapses have also been recorded. Treatment depends on the location of the lesions, with surgery being the main treatment option. Case report: This paper presents an unusual emergency presentation of infantile fibromatosis in a 16-month-old girl, initially manifested as acute laryngitis. The rapid development of respiratory failure necessitated immediate life-saving treatment. Emergency diagnostics revealed a large mass deep within the neck structures, causing significant compression and endangering the airways. The child's condition was critical, and the multidisciplinary team thoroughly discussed available treatment options. Eventually, after careful preparations, the tumormass was surgically removed on the sixth day. The postoperative course was challenging, but the outcome was positive. Pathohistological diagnosis confirmed infantile fibromatosis, and the treatment was successfully completed. Conclusion: Despite its rarity, infantile fibromatosis must be considered a potential cause of urgent, life-threatening conditions in children. Treatment requires individual adaptation and collaboration with a multidisciplinary team.