Cano-Cevallos Leonardo, Cortes-Telles Arturo, Calderon Juan C, Robles-Velasco Karla, Rivadeneyra Nelson, Cherrez-Ojeda Ivan
Respiralab Research Group, Av. Francisco Boloña, Torre Médica Xima, Guayaquil, Ecuador.
Clinica de Enfermedades Respiratorias. Hospital Regional de Alta Especialidad de la Peninsula de Yucatan, Calle 7 #433 por Calle 20 y 22. Col, Altabrisa, C.P. 97130, Mérida, Yucatán, Mexico.
Respir Med Case Rep. 2024 Sep 27;52:102126. doi: 10.1016/j.rmcr.2024.102126. eCollection 2024.
Williams-Campbell syndrome (WCS), traditionally recognized in childhood, is a rare congenital disorder characterized by subsegmental bronchial cartilage deficiency, leading to bronchiectasis. However, its occurrence in adults presents unique diagnostic complexities. We present two cases: Case 1, a 53-year-old male with recurrent rhinosinusitis and COVID-19-associated lung cysts; Case 2, a 59-year-old cyclist with pulmonary bullae. Diagnostic evaluations included pulmonary function tests and imaging studies. Both cases underwent extensive diagnostic evaluations before WCS diagnosis. Management was focused on symptom alleviation and pneumococcal vaccination. Adult-onset WCS poses diagnostic challenges, often mimicking other respiratory conditions. Pathology confirmation is a gold standard for definitive diagnosis; however, in the case of WCS, the literature supports a diagnostic approach primarily based on clinical and radiological findings. Early recognition and tailored management strategies are essential to enhance patient outcomes.
威廉姆斯-坎贝尔综合征（WCS）传统上在儿童期被认识，是一种罕见的先天性疾病，其特征为节段性支气管软骨缺乏，导致支气管扩张。然而，其在成人中的发生呈现出独特的诊断复杂性。我们报告两例病例：病例1，一名53岁男性，患有复发性鼻窦炎和新冠病毒相关肺囊肿；病例2，一名59岁的自行车运动员，患有肺大疱。诊断评估包括肺功能测试和影像学检查。两例病例在诊断为WCS之前均接受了广泛的诊断评估。治疗重点是缓解症状和接种肺炎球菌疫苗。成人起病的WCS带来诊断挑战，常与其他呼吸道疾病相似。病理确诊是明确诊断的金标准；然而，对于WCS，文献支持主要基于临床和影像学表现的诊断方法。早期识别和量身定制的管理策略对于改善患者预后至关重要。
