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Case Report | DOI: https://doi.org/10.31579/2690-8808/007
*Corresponding Author: Haidar Maojil, Departement of Otolarygology, Hassan II Hospital University, Fez, Morocco
Citation: M. Haidar, J. Ziani, A. Charai, M. Bennani, D. Kamal, (2020) A Pediatric Case of Parotid Manifestation of Recklinghausen Neurofibromatosis. Journal of Clinical Case Reports and Studies, 1(2):DOI: 10.31579/2690-8808/007
Copyright: © © 2020. Jihane Ziani. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Received: 07 March 2020 | Accepted: 16 March 2020 | Published: 23 March 2020
Keywords: pigmented skin macules ; neurofibromatic
A 12-year-old girl is refered to our ORL department. The disease started in childhood with the appearance of multiple hyper pigmented skin macules. Dermatological exam showed of hyper pigmented skin macules on the chest and back, multiple pigmented spots with diameter> 1.5 cm (Figure 1), axillary freckling.
A 12-year-old girl is refered to our ORL department. The disease started in childhood with the appearance of multiple hyper pigmented skin macules. Dermatological exam showed of hyper pigmented skin macules on the chest and back, multiple pigmented spots with diameter> 1.5 cm (Figure 1), axillary freckling.
During oral examination, we saw a formation of neurofibromatic mass on the left side of her face (Figure 2) mucous membranes were not affected.
MRI showed the presence of a hypodense left intraparotideal process before and after injection of contrast medium, molding the intraparotidic vascular structure and extending along the facial vascular bundle from the mandibular homolateral angle below, and the region pretrageal at the top, with significant narrowing of the external auditory canal of the homolateral ear.
Neurofibromatosis are genetic diseases that predispose to the development of tumors in the nervous system. There are two essential forms: neurofibromatosis type 1 and neurofibromatosis type 2. This disease cannot be treated. Only complications are treated. The parotido-jugual neurofibroma is a readily unique lesion, centered on the parotid region and more or less invading the jugular region. The parotid tissue is invaded by the neurofibroma and the position of the facial nerve is unpredictable, the neurofibroma can be developed from the facial nerve. We can also find a posterior tilt of the pavilion of the ear and occipital osteolytic lesions. Endo-oral extensions should be systematically sought [1].
Bone deformities of the upper and lower jaw are clearly associated with the presence of facial neurofibromas that invade the branches of the trigeminal. A progressive widening of the superior orbital fissure and the oval foramen. The mandible on the affected side is asymmetrical just as the adjacent part of the maxilla and the external wall of the orbit are destroyed [2]. A parotid swelling that can reflect a nervous attack, while thinking of the facial nerve even if its involvement is rare (0.5 to 1%) [3], and studies, as far as possible, its path in front of any tumor in the region parotid. Imaging plays an important role in positive diagnosis, prognostic evaluation, price orientation in therapeutic charge and progressive follow-up of lesions. Treatment is surgical but abstention and monitoring may be indicated because the progress is slow.
The particularity of our observation is that it a rare presentation of the disease in children as well as the interest of imagery to evoke the diagnosis of parotid neurofibroma and assessment of locoregional extension and the follow-up of these tumors.
Author declares that there is no conflict of interest.
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