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Neuroradiology Case of the Week

Case 228

Sam McCabe, MD, and Sudhir Kathuria, MD

Clinical Presentation: Patient with known cleft lip and palate.

Imaging Findings: The palatine process of the maxilla and horizontal plate of the palatine bone are absent. 

Diagnosis: Cleft palate

Discussion: Cleft palate is the most common facial birth defect, occurring in 1 in 1000 live births. The incidence is higher in Native American and Japanese populations and lower amongst African Americans. It results from a failure of the nasomedial process to fuse with maxillary processes on one or both sides during fetal growth, resulting in a bony or soft tissue defect involving the hard palate and upper lip. The defect may involve the lip or palate independently, but most commonly involves both. The lesion may be to the right or left of midline or bilateral, but left-sided lesions are the most common. The defect is seen as an element of more than 300 recognized clinical entities but is non-syndromic in 50-70% of cases. Genetic as well as environmental etiologies have been proposed. The incidence increases to 4% if a first degree relative is affected and jumps to 17% if two first degree relatives have cleft palates. Potentially teratogenic factors include drugs (cortisone, phenytoin, salicylates), maternal alcohol ingestion and smoking, maternal diabetes, rubella infection, and advanced maternal age.
     The maxillary alveolar process is split in the region of the lateral incisor, either unilaterally or bilaterally, again most commonly on the left. On the clefted side, the anterior hemimaxilla has a narrowed curvature and the anterior portion is tilted upward. There may be missing, deformed, displaced or supernumerary teeth. The lip may display a vertical cleft, continuous intra-orally with the palate cleft. Besides the obvious aesthetic impact of a cleft lip/palate, the lesion may also cause speech problems. There is often a limited phonemic repertoire, poor speech intelligibility, and expressive and receptive language developmental delays.

References:

1. Som P, Curtin D. Head and Neck Imaging, 4th Ed., Mosby 2003
2. Larheim TA, Westesson PL. Maxillofacial Imaging, Springer 2005