Coronal Clival Cleft: Estimated Prevalence and Clinical Associations in a Pediatric Cohort

Coronal clival cleft is a congenital corticated defect traversing the basioccipital portion of the clivus, beneath the spheno-occipital synchondrosis. It has been reported in cases of CHARGE syndrome, Cornelia de Lange syndrome, anencephaly, hemifacial microsomia, Chiari deformities, and in asymptomatic patients, but it may be underdiagnosed and underestimated on imaging. This study aims to estimate the prevalence of coronal clival cleft and expand its genetic and clinical associations.

In this retrospective study, the imaging report database from a single children's hospital was queried for the terms "clival cleft," "clivus cleft," "clefts of the clivus," and "cleft of the clivus." The search was restricted to head and neck, brain, and cervical spine CTs and MRIs, and reports from a consecutive 2-year period (May 2022 to June 2024) authored by either of 2 neuroradiologists with expertise in the diagnosis of clival clefts. Electronic medical records were reviewed for demographics and to confirm final diagnosis and genetic disorders. Descriptive statistics were used to calculate frequency, demographic characteristics, and percentage distribution.

The search yielded 13 patients with coronal clival cleft (estimated prevalence: 4.2 per 1000; 95% CI, 1.67-10.52 per 1,000). The distribution between the sexes was 7 girls and 6 boys. Partial coronal clival cleft (n=9) was more frequent than complete coronal clival cleft (n=4). Clival clefts were associated with 9 different disorders, including CHARGE syndrome (n=4), Chiari I deformities (n=2), Cornelia de Lange syndrome (n=1), and others (n=6).

Coronal clival clefts are potentially more common than previously anticipated. Radiologists should be able to recognize and differentiate coronal clival clefts from anatomic variants in the skull base and, when a coronal clival cleft is found, must actively search for additional cerebral and craniovertebral junction abnormalities, often found in combination.