A 10-year-old black male patient was referred to the Department of Stomatology, Oral and Maxillofacial Surgery of the University Hospital of Cartagena de India (Colombia), presenting a nodule in the anterior portion of the floor of the jaw slightly compromised.
During this time, the lesions grew slowly and asymptomatically.
As family history, the patient's father, aunt and sister had clinically confirmed neurofibromatosis.
Regarding personal history, no important data related to the current disease were found.
In the general physical examination the patient was conscious, with good muscle and nutritional status, with growth and development according to age.
On the skin level, numerous café au lait spots scattered throughout the body and neurofibromas were found.
The rest of the test did not reveal important data for the disease.
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He also had a slightly low ear pinna, a slight increase in perimeter, left temporal fossa depression and facial implantation compromised the middle and lower thirds of the left side.
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In the oral examination a 3x2 cm diameter nodule was observed, located in the anteromedial portion of the floor of the mouth, asymptomatic, covered by a mucosa of normal appearance and color, of firm consistency.
At the level of the left mandibular alveolar ridge, he had a 4x2 cm diameter tumor, located in the mandibular body, from the first temporal molar to the retromolar region.
In the left hemimaxillary region, there was a palatal bulging that compromised from the first bicuspid to the tuberosity, with similar clinical characteristics as the nodule on the floor of the mouth.
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The patient's family history, the numerous café au lait spots, and some signs such as school delay, low implantation of ears and depression of the temporal fossa led us to diagnose a clinical neurofibromatosis type 1 and neurofibromatosis.
Radiologically, an image of extraosseous compression at the level of the left mandibular body and ascending ramus was observed, with displacement of teeth 34, 35, 36, 37 and germ 38.
The upper jaw showed bone resorption at the level of the tooth 26.
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Pathological examination with slit lamp revealed numerous Lisch nodules, which confirmed another diagnostic criterion.
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The patient was taken to the operating room to perform, under general anesthesia, an excisional biopsy of the tumor lesions as well as placement of alveolar ridges.
Histopathological analysis confirmed the diagnosis of neurofibromas.
In order to rule out the presence of optic nerve glioma, an orbital CT scan was performed, without revealing any pathology of these nerves.
