Miofibromatosis infantil sistémica en un lactante

Résumé

Infantile myofibromatosis is characterized by the presence of solitary or multicentric fibrous nodules in skin, muscle and/or internal organs. Despite being an infrequent entity, and consequently little known by pediatricians, it constitutes the most frequent fibrous tumor in children. Solitary cutaneous lesions have a very good prognosis but in some cases there is systemic involvement. In these cases the disease can produce serious complications and even put the patient’s life at risk, especially during the first months of life. These complications are due to the locally invasive nature of the nodules, obstruction of vital organs, growth retardation or infection. We present the case of an infant who at birth presented a skin nodule only. Over time, the infant presented lesions in skin, bone and internal organs. The infant showed respiratory distress requiring mechanical ventilation due to diaphragmatic paralysis. We recommend close follow-up of all patients with infantile myofibromatosis to prevent or make an early diagnosis of these complications.