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The terminal nerve (cranial nerve zero, cranial nerve XIII, the nerve “N”) was discovered in fish in 1894. In the early 90’s, it was found in human embryos and human adults. In the anterior fossa, it courses on the inner side of the olfactory tract and bulb; it then spreads fibers through the cribriform plate to distribute beneath the nasal septum mucosa. Being provided with intrinsic ganglion cells, its functions are weakly suggested by studies in different species. It may be connected with the visual system, it could act upon the intracranial vascular system, or it could ensure the pathway for pheromone-mediated behaviours. The cranial nerve zero deserves a better attention equally from anatomists and ENT specialists.
Hereditary angioedema (HAE) is a rare autosomal dominant disease consisting of recurrent angioedema attacks, varying in severity, possibly life-threatening and with frequent involvement of the head and neck areas. The patophysiology of HAE differs from histamine-mediated allergic angioedema. Three types of reduced quality or quantity in various complement or coagulation factors, leading to massive release of bradykinin, increase vascular permeability and produce capilary leakage. Clinical manifestations of HAE include swelling located predominantly in the head and neck area, hands, feet and urogenital area and abdominal pain caused by edema of the gastrointestinal tract mucosa. Diagnosis requires laboratory tests for complement components and genetic tests. A timely and correct diagnosis in the emergency room is of utmost importance, the medical treatment of HAE being substantially different from that of allergic angioedema. Although new therapies are available and in development, airway intervention and surgery are still life-saving procedures and the ENT surgeon is an important part of the multidisciplinary team managing an HAE attack.
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