LAUSR

A 61-year-old male patient presented with a two-day history of diarrhea and vomiting, reporting dyspnea, as well as severe lower abdominal and thoracic pain with irradiation to the precordium and left shoulder. Physical examination showed a rigid abdomen and reduced breath sounds, with coarse crackles in both lung bases. Computed tomography (CT) of the thorax showed posterior pneumomediastinum and periesophageal densification primary osseous dysplasia or pressure-induced responses to neurofibromas. Although the diagnosis of NF1 often relies on cardinal clinical findings, cross-sectional imaging studies can provide valuable information in sundry settings. Particularly for NF1 patients with skull defects, CT is essential for detecting and following up the lesions, given that progressive bone erosion occurs in more than half of all cases and such erosion can require calvarial reconstruction with bone grafts or titanium mesh. However, progressive bone resorption can predispose to long-term implant instability, the best approach to NF1 calvarial defects therefore remaining undetermined.