Obregón MJ, Santillán AA

Language: Spanish
References: 7
Page: 69-72
PDF size: 3302.87 Kb.

ABSTRACT

Neurofibromatosis is the most common neurocutaneous syndrome, and is divided into two genetic variants. Neurofibromatosis type 1 (von Recklinghausen’s disease, NF-1) is autosomic dominant with a penetrance of 80-90% and has occurs in one of every 4,000 childbirths. Neurofibromas are the most commonly tumors found in patients with NF-1. This is a case report of a seven year-old male presenting with NF-1. Two sibling were also affected by NF-1. The patient showed the classic cafe-au-lait skin lesions and multiple bilateral cervical neurofibromas. Such tumors had rapidly progressed within the previous six months, provoking dyspnea and dysphagia two weeks prior to his admittance to the Hospital. One of the neurofibromas was located in the left anterior and posterior cervical triangles, underneath the esternocleidomastoid and causing important tracheal displacement and esophageal compression. The tumor was surgically resected using the Fendi approach. The present case is discussed in light of the current approach to the management of this type of lesion. Special consideration is given to the secondary problems that may arise upon attempting to resect all neurofibromas present in the surgical field.

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