Abstract：
BACKGROUND: It is estimated that approximately one out of 200 boys has the diagnosis of lichen sclerosus (LS), previously referred to as BXO (balanitis xerotica obliterans). Severe progressive disease is rare however, mismanagement of urethral tissues may contribute to progression of LS.
METHODS: The current literature regarding the management of severe lichen sclerosus was reviewed alongside our management of seven patients with ages ranging from six to ten years of age with severe lichen sclerosus who required surgical intervention. These patients were identified out of a busy pediatric practice that saw 5507 patients during the four-year span. Based on the pathophysiology of lichen sclerosus, urethral anatomy, and our management an algorithm was developed for medical and surgical management.
RESULTS: All patients received initial medical treatment with topical steroids. Three patients underwent urethral mobilization and serial biopsy. One of these patients with severe disease required a second distal urethral mobilization. Three patients were treated with circumcision, and one is responding well to topical steroids after complex reconstruction. None developed postoperative urethral disease.
CONCLUSIONS: Lichen sclerosus affects squamous epithelium but can extend to unaffected tissue if traumatized via the Koebner phenomenon. There are no randomized control trials for the management of the disease. Thus, appropriate early management with avoidance of urethral dilation or incision may prevent extension down the urethra that can lead to severe stricture disease. Several authors identified this as one of the worst forms of stricture diseases to manage. Based on the pathophysiology of the disease and our 4-year experience treating patients, we propose an algorithm for management of severe lichen sclerosus in boys. The diagnosis of lichen sclerosus in boys requires a high level of suspicion, and early biopsies should be obtained if suspected. If identified before circumcision or meatotomy, initial treatment should be medical. If the patient fails topical steroid therapy, circumcision and biopsy are the initial recommended surgical approach. Optimally, a biopsy with the first meatotomy establishes the diagnosis. If the disease persists, urethral mobilization may represent a curative treatment as it advances healthy urethra and allows complete removal of distal squamous epithelium instead of traumatic repeated dilations or incisions.
CONCLUSIONS: This paper summarizes the available literature on the management of severe LS and provides a flow diagram based on the pathophysiology of the disease and our experience sever cases.
METHODS: The current literature regarding the management of severe lichen sclerosus was reviewed alongside our management of seven patients with ages ranging from six to ten years of age with severe lichen sclerosus who required surgical intervention. These patients were identified out of a busy pediatric practice that saw 5507 patients during the four-year span. Based on the pathophysiology of lichen sclerosus, urethral anatomy, and our management an algorithm was developed for medical and surgical management.
RESULTS: All patients received initial medical treatment with topical steroids. Three patients underwent urethral mobilization and serial biopsy. One of these patients with severe disease required a second distal urethral mobilization. Three patients were treated with circumcision, and one is responding well to topical steroids after complex reconstruction. None developed postoperative urethral disease.
CONCLUSIONS: Lichen sclerosus affects squamous epithelium but can extend to unaffected tissue if traumatized via the Koebner phenomenon. There are no randomized control trials for the management of the disease. Thus, appropriate early management with avoidance of urethral dilation or incision may prevent extension down the urethra that can lead to severe stricture disease. Several authors identified this as one of the worst forms of stricture diseases to manage. Based on the pathophysiology of the disease and our 4-year experience treating patients, we propose an algorithm for management of severe lichen sclerosus in boys. The diagnosis of lichen sclerosus in boys requires a high level of suspicion, and early biopsies should be obtained if suspected. If identified before circumcision or meatotomy, initial treatment should be medical. If the patient fails topical steroid therapy, circumcision and biopsy are the initial recommended surgical approach. Optimally, a biopsy with the first meatotomy establishes the diagnosis. If the disease persists, urethral mobilization may represent a curative treatment as it advances healthy urethra and allows complete removal of distal squamous epithelium instead of traumatic repeated dilations or incisions.
CONCLUSIONS: This paper summarizes the available literature on the management of severe LS and provides a flow diagram based on the pathophysiology of the disease and our experience sever cases.
摘要：
背景：据估计，大约每200个男孩中就有一个被诊断为硬化性苔藓（LS），以前称为BXO（干燥龟头炎）。然而，严重的进行性疾病很少见，尿道组织管理不善可能导致LS的进展。
方法：回顾了当前有关严重硬化性苔藓治疗的文献，同时回顾了我们对7名年龄在6至10岁之间的严重硬化性苔藓患者的治疗，这些患者需要手术干预。这些患者是在繁忙的儿科实践中确定的，在四年的时间里看到了5507名患者。根据硬化性苔藓的病理生理学，尿道解剖,我们的管理开发了一种用于医疗和外科管理的算法。
结果：所有患者都接受了局部类固醇的初始药物治疗。三名患者接受了尿道动员和连续活检。其中一名患有严重疾病的患者需要第二次远端尿道动员。三名患者接受了包皮环切术治疗，一个在复杂的重建后对局部类固醇反应良好。无术后尿道疾病。
结论：硬化性苔藓影响鳞状上皮，但如果通过Koebner现象受到创伤，则可以扩展到未受影响的组织。目前尚无治疗该疾病的随机对照试验。因此，适当的早期治疗避免尿道扩张或切口可以防止尿道向下延伸，这可能导致严重的狭窄疾病。几位作者认为这是最糟糕的狭窄疾病之一。根据疾病的病理生理学和我们治疗患者的4年经验，我们提出了一种管理男孩严重硬化性苔藓的算法。诊断为男孩硬化性苔藓需要高度怀疑，如果怀疑，应进行早期活检。如果在包皮环切术或切肉术前被发现，最初的治疗应该是医学的。如果患者局部类固醇治疗失败，包皮环切术和活检是最初推荐的手术方法.Optimally,第一次切肉手术的活检确定了诊断。如果疾病持续存在,尿道动员可能是一种治愈性治疗，因为它可以促进尿道健康，并允许完全切除远端鳞状上皮，而不是创伤性的重复扩张或切口。
结论：本文总结了有关严重LS的处理的现有文献，并根据疾病的病理生理学和我们的经验提供了流程图。
方法：回顾了当前有关严重硬化性苔藓治疗的文献，同时回顾了我们对7名年龄在6至10岁之间的严重硬化性苔藓患者的治疗，这些患者需要手术干预。这些患者是在繁忙的儿科实践中确定的，在四年的时间里看到了5507名患者。根据硬化性苔藓的病理生理学，尿道解剖,我们的管理开发了一种用于医疗和外科管理的算法。
结果：所有患者都接受了局部类固醇的初始药物治疗。三名患者接受了尿道动员和连续活检。其中一名患有严重疾病的患者需要第二次远端尿道动员。三名患者接受了包皮环切术治疗，一个在复杂的重建后对局部类固醇反应良好。无术后尿道疾病。
结论：硬化性苔藓影响鳞状上皮，但如果通过Koebner现象受到创伤，则可以扩展到未受影响的组织。目前尚无治疗该疾病的随机对照试验。因此，适当的早期治疗避免尿道扩张或切口可以防止尿道向下延伸，这可能导致严重的狭窄疾病。几位作者认为这是最糟糕的狭窄疾病之一。根据疾病的病理生理学和我们治疗患者的4年经验，我们提出了一种管理男孩严重硬化性苔藓的算法。诊断为男孩硬化性苔藓需要高度怀疑，如果怀疑，应进行早期活检。如果在包皮环切术或切肉术前被发现，最初的治疗应该是医学的。如果患者局部类固醇治疗失败，包皮环切术和活检是最初推荐的手术方法.Optimally,第一次切肉手术的活检确定了诊断。如果疾病持续存在,尿道动员可能是一种治愈性治疗，因为它可以促进尿道健康，并允许完全切除远端鳞状上皮，而不是创伤性的重复扩张或切口。
结论：本文总结了有关严重LS的处理的现有文献，并根据疾病的病理生理学和我们的经验提供了流程图。
