Objective: Williams-Campbell syndrome (WCS) is a rare congenital disorder, which leads to bronchiectasis affecting fourth to sixth order of bronchial divisions. Symptoms include cough, sputum, wheeze and recurrent pulmonary infections, classically seen in the pediatric age group with selective bronchiectasis of the mid-order bronchioles. The literature describing diagnosis of Williams-Campbell syndrome in adult population is very sparse. Methods: This report presents a 62-year-old female with cough, fever, dyspnea and generalized body ache. She has had multiple admissions to the hospital since her childhood due to recurrent lower respiratory tract infections. Imaging findings demonstrated multiple cystic thin walled airways, compatible with bronchiectatic changes in the upper, middle and lower lobes bilaterally, bronchial wall thickening with air-fluid levels prominent in the fifth and sixth generation bronchial divisions, with normal caliber trachea and central bronchi. These radiologic findings are consistent with diagnosis of Williams-Campbell syndrome, which was diagnosed after ruling out the other common causes of bronchiectasis. Conclusion: Williams-Campbell syndrome is a rare congenital cystic lung disease, the diagnosis of which is made by exclusion of common causes of bronchiectasis such as cystic fibrosis, allergic bronchopulmonary aspergillosis, tuberculosis, dyskinetic cilia syndrome and alpha-1 antitrypsin deficiency. Whenever the clinical picture is consistent with bronchiectasis, especially involving the mid-order bronchioles and recurrent pulmonary infections, it is wise to include WCS in the list of differential diagnoses, even in the adult population.
Background
Certain factors have been linked to lateral skull base demineralisation or erosion, which may predispose to spontaneous cerebrospinal fluid leak. There are relatively few quantitative reports of skull base changes in patient populations.
Method
A novel refined measurement algorithm for quantification of tegmen bone mineral density was developed, and bone mineral density between obese and non-obese patient groups was compared. Computed tomography scans were analysed by three blinded reviewers, and tegmen bone mineral densities were compared.
Results
There were 23 patients in the obese group and 27 matched controls in the non-obese group. Inter-rater reliability was ‘strong’ to ‘near complete’ (κ = 0.75–0.86). No differences in tegmen bone mineral density were found between the groups (p = 0.64). The number of active blood pressure medications correlated positively with lateral skull base bone mineral density.
Conclusion
A novel, refined, quantitative measurement algorithm for the assessment of tegmen bone mineral density was developed and validated. Obesity was not found to significantly affect tegmen bone mineral density.
BACKGROUND
Frontal mucoceles develop due to accumulation of mucoid secretions within the frontal sinuses. They can lead to serious consequences with further expansion and destruction of the surrounding bones or infection that might spread intracranially.
OBSERVATIONS
The authors present a case of a 37-year-old male with a frontal mucocele and the rare presentation of Pott’s puffy tumor and an epidural-cutaneous fistula, as well as a literature review of previously reported cases of epidural cutaneous fistula and sinocutaneous fistula, their predisposing factors, and their management.
LESSONS
A mucocele is a benign entity that can rarely present with potentially significant complications. Open surgery is required in patients who have frontal sinus posterior wall involvement, osteomyelitis, or intracranial involvement.
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