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. 2022 Jun 3;64(8):1483–1509. doi: 10.1007/s00234-022-02986-x

Table 1.

Clinico-epidemiological features of developmental and inflammatory sphenoid bone lesions. M male. F female. CN cranial nerve

Developmental and inflammatory sphenoid lesions Prevalence Age (decade) of peak incidence of onset Gender predilection Clinical manifestations
Ecchordosis physaliphora [13] Found in about 0.4–2% of autopsies Congenital M = F Usually asymptomatic and does not require any treatment
Neurenteric cyst [21, 22] Intracranial localization is rare (0.15–0.35% of all intracranial tumours); clivus localization is described in very few sporadic cases III–IV decade M = F Often symptomatic with headache and diplopia
Arrested pneumatization [11, 24] 2% in the general population; 10% in patients with blood-red cell diseases (sickle cell anaemia-thalassemia) Congenital and developmental lesion M = F Headache and obstructive symptoms due to alterations of normal sinus drainage
Epidermoid cyst [31, 32] Located in the head-neck area in 7% of cases Often congenital. It can also occur in adult age for metaplasia or trauma M = F Symptoms are very rare. They generally occur in III-V decade and include visual disturbances (compression of CN II), infection due to adjacent sinusitis, and pituitary apoplexy in case of sellar extension
Fibrous dysplasia [37] The skull is involved in 10–30% and 50% of monostotic and polyostotic forms respectively. Sphenoid is one of the main craniofacial areas Congenital M = F Supraorbital headache is the most frequent symptom
Fungus ball (mycetoma) [7] Typically affects the maxillary sinus. The sphenoid sinus is involved in about 20% of paranasal fungus balls V-VI decade M:F ratio = 3:7 Frontal, retro-orbital, and occipital headache. Visual disturbances due to the CN II and VI impairment. Rarely asymptomatic
Mucocele [8, 9] Sphenoid sinus is a rare localization for mucocele, accounting for 1–2% of all paranasal mucoceles Any age M = F Posterior headache is the most common symptom. Visual disturbances may be associated when mucocele compresses CN II into the orbit and/or CN III, IV, and VI into the cavernous sinus
Osteomyelitis [10, 12]  < 2% and around 10% of all osteomyelitis in developed and developing countries, respectively Any age M:F ratio = 2:1 Headache is commonly the only initial symptom