Introduction Extensive craniocervical pneumatization (CCP) refers to an abnormal pneumatization extended from the temporal bone into adjacent bone structures, especially the skull base and the craniocervical junction. The etiology remains controversial; however several studies reported a correlation with recurrent Valsalva maneuvers or Eustachian tube dysfunction. Although some cases requiring surgical treatment have been reported, conservative treatment remains the gold standard. The authors aimed to describe a case of CCP, complicated by a spontaneous fracture of a pneumatized left occipital condyle. Furthermore, they reviewed all previously reported cases of fractures in CCP in order to propose a standardized approach to this pathology.

Methods A total of 148 studies were retrieved. Of those, 23 studies (including 26 patients in addition to our case) were included in the review. These studies consisted of case reports or small case series (up to 3 patients).

Results In 3 patients (11.1%), bone pneumatization involved C0; all remaining patients had both C0 and C1 pneumatization, while in 7 cases (25.9%), an extension to C2 and/or C3 was reported. Radiological follow-up was performed in 20 patients (74.1%), showing in all of the cases either stability (6 patients, 22.2%), improvement, or complete resolution (6 patients, 22.2% vs 8 patients, 29.7%). Two patients underwent surgical intervention.

Conclusions This review suggests that fractures secondary to CCP are extremely rare and are associated to a good clinical and radiological outcome with conservative treatment. Ear, nose, and throat (ENT) evaluation is recommended to detect cases who need treatment for a subjacent middle ear disease.