Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management

Numb chin syndrome (NCS) is a rare sensory neuropathy resulting from inferior alveolar or mental nerve injury. It manifests as hypoesthesia, paraesthesia, or, rarely, as pain in the chin and lower lip. Several case reports suggest that sickle cell disease (SCD) could be a cause of NCS. However, information about NCS is scarce in this population. Our objectives were to synthesize all the available literature relevant to NCS in SCD and to propose recommendations for diagnosis and management based on the best available evidence. A systematic review was performed on several databases to identify all relevant publications on NCS in adults and children with SCD. We identified 73 publications; fourteen reports met the inclusion/exclusion criteria. These described 33 unique patients. Most episodes of NCS occurred in the context of typical veno-occlusive crises that involved the mandibular area. Radiological signs of bone infarction were found on some imaging, but not all. Neuropathy management was mostly directed toward the underlying cause. Overall, these observations suggest that vaso-occlusion and bone infarction could be important pathophysiological mechanisms of NCS. However, depending on the individual context, we recommend a careful evaluation to rule out differential causes, including infections, local tumors, metastatic disease, and stroke.