Treatment algorithm for the comprehensive management of severe lichen sclerosus in boys based on the pathophysiology of the disease.

INTRODUCTION: 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. STUDY DESIGN: 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. DISCUSSION: 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. CONCLUSION: 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.

Fimosi refractària a tractament mèdic amagant una balanitis xeròtica obliterant / Fimosis refractaria a tratamiento médico escondiendo una balanitis xerótica obliterante / Refractory phimosis due to balanitis xerotica obliterans

INTRODUCCIÓ: La balanitis xeròtica obliterant és una malaltia crònica inflamatòria progressiva de baixa incidència en la població pediàtrica. CAS CLÍNIC: Es presenta un pacient de 4 anys afectat de fimosi greu I aspecte xeròtic del prepuci que no respon a tratament tòpic amb corticoides. Davant la sospita de balanitis xeròtica obliterant es practica una postectomia completa, I l'anatomia patològica confirma el diagnòstic. COMENTARI: És important conèixer aquesta malaltia en l'edat pediàtrica ja que el seu diagnòstic de sospita és clínic I pot comportar greus complicacions per als pacients. La refractarietat al tractament mèdic amb corticoides tòpics, la recidiva de la fimosi en pacients intervinguts, l'aparició de lesions blanquinoses en la mucosa prepucial o el gland I l'estenosi meatal són complicacions possibles que ens han de fer sospitar aquesta entitat. El tractament de les lesions en pacients intervinguts previament de fimosi amb diagnòstic posterior de balanitis xeròtica obliterant pot ser mèdic I de forma ambulatòria amb pomada de corticoides. És necessari un seguiment estricte d'aquests pacients per detectar possibles complicacions més greus, com l'estenosi meatal

INTRODUCCIÓN: La balanitis xerótica obliterante es una enfermedad crónica inflamatoria progresiva de baja incidencia en la población pediátrica. CASO CLÍNICO: Se presenta un paciente de 4 años afectado de fimosis grave y aspecto xerótico del prepucio que no responde a tratamiento tópico con corticoides. Ante la sospecha de balanitis xerótica obliterante, se realiza una postectomía completa y la anatomía patológica confirma el diagnóstico. COMENTARIO: Es importante el conocimiento de esta enfermedad en la edad pediátrica ya que su diagnóstico de sospecha es clínico y puede conllevar graves complicaciones para los pacientes. La refractariedad al tratamiento médico con corticoides tópicos, la recidiva de la fimosis en pacientes intervenidos, la aparición de lesiones blanquecinas en mucosa prepucial o glande y la estenosis meatal son complicaciones posibles que nos deben hacer sospechar esta entidad. El tratamiento de las lesiones en pacientes previamente intervenidos de fimosis con diagnóstico posterior de balanitis xerótica obliterante puede ser médico y de forma ambulatoria con pomada de corticoides. Es necesario un seguimiento estricto de estos pacientes para la detección de posibles complicaciones más graves, como la estenosis meatal

INTRODUCTION: Balanitis xerotica obliterans is a chronic progressive inflammatory disease of low incidence in the pediatric population. CASE REPORT: We present the case of a 4-year-old patient with stenosis and xerotic aspect of the foreskin that did not respond to medical treatment with topic corticosteroids. Balanitis xerotica obliterans was suspected, a complete postectomy was performed and the diagnosis was confirmed by pathology. COMMENTS: It is important to recognize this entity in children since its diagnosis is clinical and its mismanagement may result in several complications. The refractoriness to medical treatment with corticosteroid ointment, the recurrence of phimosis in operated patients, the appearance of whitish lesions in the mucosa of the foreskin or glans, and meatal stenosis are the complications that should raise suspicion of this entity. The treatment of lesions in patients who have previously undergone surgical treatment of phimosis can be medical with corticosteroid ointment. A close follow-up of these patients is required in order to detect severe complications such as meatal stenosis