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.

Could a careful clinical examination distinguish physiologic phimosis from balanitis xerotica obliterans in children?

The diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for phimosis from 2015 to 2019. Histology was routinely performed. Cohen's kappa coefficient, sensitivity, specificity, predictive values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p < 0.001) and it showed a diagnostic accuracy of 75%. The specificity and positive predictive value of abnormal macroscopic findings at examination, together with a positive clinical history for other allergic or immune diseases, and/or for balanitis, were 100% and the positive likelihood ratio was greater than 10. Conversely, sensitivity decreased to 4.5% (95% CI 0-11%).Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended. What is Known: • Occurrence of balanitis xerotica obliterans may be underestimated in children and it could lead to long-term complications. • The diagnostic accuracy of clinical examination is controversial. What is New: • Clinical signs together with patients' medical history present high specificity and positive predictive values but low sensitivity. • When suggestive clinical aspects are present, patient should be referred to surgery avoiding prolonged conservative treatment; and preputial histology is highly recommended.

Balanitis Xerotica Obliterans:an underestimated cause of secondary phimosis.

Balanitis Xerotica Obliterans is a chronic, progressive, sclerosing inflammation of unclear etiology. It involves the external genitalia of males and more specifically the prepuce and its frenulum, the glans, and the external urethral meatus while it may extend to the peripheral part of the urethra. Recent studies have noted an increasing incidence in the paediatric population. It is the most common cause of secondary (pathologic) phimosis. Even more, in boys with physiologic phimosis that does not respond to conservative treatment, Balanitis Xerotica Obliterans should be considered as the underlying condition. In this study, we present all the latest data and attempt to create a diagnostic and curative algorithm regarding this condition.

Lichen Sclerosus and Phimosis - Discrepancies Between Clinical and Pathological Diagnosis and Its Consequences.

OBJECTIVE: To assess the accuracy of clinical diagnoses and the true incidence of lichen sclerosus (LS) in patients with phimosis. MATERIALS AND METHODS: The 92 adult male patients who were qualified for circumcision due to phimosis, were included in the study. The patients were diagnosed clinically by a urologist and dermatologist before the surgical procedure. After the circumcision, the resected foreskins were examined by 2 independent uropathologists. RESULTS: Preoperative clinical diagnosis of LS was established in 54 patients (58.7%); healthy-looking skin in 26 (28.3%) and other penile diseases in 12 (13.1%) patients. After histopathological examination, the diagnosis of LS was established in 62 patients (67.4%), but only in 44 patients with previous LS clinical diagnosis. LS was histopathologically confirmed in 18 other patients with clinically diagnosed healthy skin (n = 17) or lichen planus (n = 1). Healthy skin was histopathologically confirmed in 10 cases in patients diagnosed clinically before as LS. Other 15 histopathological diagnoses were Zoon balanitis (n = 3), nonspecific balanitis (n = 5), lichen planus (n = 1), psoriasis (n = 1), invasive penile cancer (n = 3), Bowen's disease (n = 1), penile intraepithelial neoplasia 2 usual type (n = 1). CONCLUSION: LS has been revealed as the most common histopathological diagnosis in patients undergoing circumcision in our study. Histopathological examination seems to be necessary to exclude this disease.

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

Treatment of balanitis xerotica obliterans in pediatric patients. / Tratamiento de la balanitis xerótica obliterante en pacientes pediátricos.

OBJECTIVES: Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease with a little known incidence in pediatric population. The objective of this work was to describe our experience in the treatment of BXO. MATERIALS AND METHODS: Retrospective study carried out in 419 patients undergoing circumcision surgery between January 2014 and January 2017. Demographic, clinical, therapeutic, and anatomical and pathological variables, as well as complications during follow-up, were analyzed. RESULTS: Of the 419 patients, 41 (9.78%) were diagnosed with BXO. 6 patients were excluded owing to lack of follow-up, so 35 patients were analyzed. Mean age at diagnosis was 8.6 years. Suspicion diagnosis was clinical at physical exploration in 17 patients (48.6%), and at surgery in 18 patients (51.4%). Anatomical and pathological confirmation was performed in a total 35 patients (100%). During follow-up, 6 patients (17.14%) had lesions in the glans, 3 (8.57%) in the urethra, and 9 (25.71%) in both. 6 meatotomies (17.14%) and 5 new circumcisions (14.28%) had to be carried out. Mean recurrence time was 32.43 months. In 19 patients (54.28%), topical corticoids - ointment - were applied, and 1 patient (2.85%) received topical immunosuppressants. CONCLUSIONS: A close follow-up of patients with clinical or anatomical and pathological diagnosis of BXO is required given its high morbidity. The complications described in pediatric population include meatal and urethral stenosis, as well as recurrent phimosis, unless a sufficient amount of foreskin is resected.

OBJETIVOS: La balanitis xerótica obliterante (BXO) es una enfermedad crónica inflamatoria de incidencia poco conocida en la población pediátrica. El objetivo de este trabajo es describir nuestra experiencia en el tratamiento de las BXO. MATERIAL Y METODOS: Estudio retrospectivo de 419 pacientes intervenidos de circuncisión en el periodo comprendido entre enero de 2014 y enero de 2017. Analizamos variables demográficas, clínicas, anatomopatológicas, terapéuticas y complicaciones durante el seguimiento. RESULTADOS: De los 419 pacientes, 41 fueron diagnosticados de BXO (9,78%). Seis pacientes fueron excluidos por falta de seguimiento, por lo que se analizaron 35 pacientes. La media de edad al diagnóstico fue de 8,6 años. El diagnóstico de sospecha fue clínico durante la exploración física en 17 pacientes (48,6%) y durante la intervención en 18 (51,4%), realizando la confirmación anatomopatológica en un total de 35 pacientes (100%). Durante el seguimiento 6 pacientes (17,14%) presentaron lesiones en glande, 3 (8,57%) en uretra y 9 (25,71%) en ambas localizaciones, siendo necesaria la realización de 6 meatotomías (17,14%) y de nueva circuncisión en 5 (14,28%). El tiempo medio de recidiva fue de 32,43 meses. En 19 pacientes (54,28%) se aplicaron corticoides tópicos en pomada y en 1 paciente inmunosupresores tópicos (2,85%). CONCLUSIONES: Es necesario un seguimiento estrecho de los pacientes con diagnóstico clínico o anatomopatológico de BXO dada su elevada morbilidad. Las principales complicaciones descritas en la población pediátrica son la estenosis meatal y uretral, así como la recidiva de la fimosis si no se reseca el prepucio suficientemente.

Balanitis xerotica obliterans: an update for clinicians.

Lichen sclerosus (LS) is a severe, chronic, dermatosis characterised by inflammatory, sclerotic, pruritic lesions that causes significant morbidity in patients of all genders and ages. In boys, the lesions typically affect the foreskin and glans (termed balanitis xerotica obliterans (BXO)), leading to phimosis and potentially meatal stenosis. The incidence of the disease is not well reported but the average age of affected boys is 8 years (range 1-16). Diagnosis can often be made clinically, although histological study remains important to rule out important differential diagnoses. Complications include genital scarring, urinary and sexual dysfunction as well as the development of carcinomas in adult life. Circumcision has been regarded as definitive management of BXO in boys, but this may be supplemented with medical therapies such as topical steroids, immune modulators, intralesional triamcinolone and ozonated olive oil. Supportive measures including emollients, avoidance of irritants, surveillance of complications and recurrence as well as education and counselling remain important.Conclusion: BXO remains an important cause of phimosis in boys. The frequency of this condition appears unclear but seems likely to be less than 1% of males. Treatment generally involves circumcision, with some evidence that topical steroids or immunomodulators may decrease the incidence of recurrent meatal stenosis.What is Known:• Surgical circumcision is considered the definitive management of BXO• Many aspects of BXO are still in contention or require further study including the epidemiology and aetiology.What is New:• There is increasing awareness of non-surgical modalities that may be used in adjunct to surgery including topical corticosteroids, immune modulators, intralesional triamcinolone and ozonated olive oil• Awareness of meatal stenosis-related BXO has led to the development of surgical techniques such as preputioplasty as well as buccal mucosal inlay grafts.

Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review.

OBJECTIVE: To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options. MATERIALS AND METHODS: The Medline/PubMed and Embase databases were systematically reviewed for publications pertaining to LS. After applying inclusion and exclusion criteria, references were assessed for relevance to the pathophysiology, presentation, and treatment of LS by title and abstract review by 2 independent reviewers, yielding 186 articles for assessment. RESULTS: The contemporary understanding of the epidemiology and histology of LS is reviewed herein. Additionally, we explore in detail the 3 hypotheses regarding the pathophysiological mechanism contributing to disease presentation: infectious etiology, primary immune dysregulation, and the isotraumatopic response. We summarize the available biological evidence supporting each hypothesis. This discussion provides context for understanding LS morbidity and may spur new avenues of research. For the clinician, we review the clinical presentation of disease, including the risk of progression to squamous cell carcinoma. The current medical and surgical treatment options are also detailed. CONCLUSION: LS remains a potentially insidious disease which may lead to debilitating urinary and sexual dysfunction. Cross disciplinary research should aim for earlier detection, as well as more effective and durable treatment. The exact cause of LS remains unknown.

Balanitis xerotica obliterans: a review of diagnosis and management.

Balanitis xerotica obliterans (BXO), or penile lichen sclerosus, is a progressive sclerosing inflammatory dermatosis of the glans penis and foreskin. It is associated with significant morbidity and may result in impaired urinary and sexual function. It was initially described by Stuhmer in 1928, named after its pathological features, and is considered the male equivalent of vulvar lichen sclerosis (LS).3,40 The etiology of BXO is uncertain; however, autoimmune disease, local trauma, and genetic and infective causes have been proposed. BXO occurs most commonly on the prepuce and glans penis. It is considered to have premalignant potential to transform into squamous neoplasia. This postulation rests on retrospective studies and parallels drawn with vulvar LS and squamous cell carcinoma (SCC) development. Histologically, BXO and vulvar LS are considered the same disease.41 There is a paucity of evidence-based guidelines to assist with appropriate follow-up for patients with BXO.

10 Years' Experience in Balanitis Xerotica Obliterans: A Single-Institution Study.

INTRODUCTION: Balanitis xerotica obliterans (BXO) is uncommon in children. Diagnosis of the condition is almost always clinical and supported by histology. Our aim was to evaluate the outcomes of children undergoing circumcision for BXO and explore the correlation between surgical and histological findings. MATERIALS AND METHODS: A 10-year retrospective review (2007-2017) of all children, aged 16 and less, undergoing circumcision at a tertiary teaching hospital was conducted. Statistical analysis was performed using Fisher's exact test. RESULTS: BXO occurred in 91/1025 (8.9%) children. The highest incidence of BXO was in the 5 to 10 age group (13.3%; p < 0.0001). The commonest symptom was foreskin scarring (62.6%). Intraoperatively, involvement of foreskin alone was seen in 26.4%, foreskin and meatus in 47.2%, and foreskin, meatus, and glans in 26.4%. Preoperatively, 24.2% of patients were prescribed steroid cream. Histologically, all patients showed microscopic changes confirming BXO. A total of 87.9% of patients only had a circumcision, and 11% required a meatal procedure along with the circumcision. Postoperatively, 19.8% of patients required a further procedure after an average duration of 5.8 months (range: 2-12 months); the majority of whom (83.3%) were prescribed postoperative steroid cream. CONCLUSION: Clinical correlation by surgeons has a high degree of accuracy (>90%). Meatal involvement is more common than previously reported. The use of pre- or postoperative steroids does not obviate the need for further surgical procedures. Patients can have recurrent symptoms 1 year following surgery, and prolonged follow-up is necessary.

Utility of uroflowmetry during the follow-up of children affected by balanitis xerotica obliterans (BXO).

INTRODUCTION: To evaluate the outcome of circumcised patients with balanitis xerotica obliterans (BXO) using uroflowmetry (UF). METHODS: Between 2011 and 2013, 180 children underwent a circumcision for phimosis. The foreskin was examined on microscopy. Patients with an histological diagnosis of BXO were included in the study. Patients with BXO underwent UF two weeks after surgery and treatment with clobetasol propionate ointment. Patients were re-evaluated at 6, 12, 18 and 24 months postoperatively clinically and using UF. RESULTS: 75 of 180 circumcised patients (41.6%) were included. At two weeks, Thirtytwo of 75 patients (42.7%) displayed a pathological UF. At six months, 15 patients (20%) had pathological UF and a new cycle of clobetasol was prescribed. At one year, 10 patients (13.3%) displayed patholgocial UF and underwent progressive urethral dilatation or meatoplasty. At 18 months, 71 patients (94.7%) displayed regular UF, 3 underwent a meatoplasty and one a staged urethroplasty for a severe urethral stenosis. At two years, UF was normal in 74 out of 75 (98.7%). CONCLUSIONS: We recommend to send for hystological examination all foreskins excised after circumcision. We believe that a clinical and uroflowmetric follow-up of pediatric patients with BXO is mandatory for a prompt identification of post-voiding dysfunction.

Single-stage urethroplasty with buccal mucosal inlay graft for stricture caused by balanitis xerotica obliterans in boys: Outcomes in the medium term.

INTRODUCTION: Balanitis xerotica obliterans (BXO) is a common condition that can affect the foreskin, glans, meatus, and urethra, and rarely can also involve urethra leading to stricture. Numerous procedures have been suggested to treat urethral stricture secondary to BXO but these have had variable results. OBJECTIVE: We describe the first prospective study of a single stage buccal mucosal inlay grafting in children with urethral strictures resistant to existing remedies. MATERIALS AND METHODS: We treated five boys with resistant urethral strictures secondary to BXO with a single stage buccal mucosal inlay graft (BMIG, Figure). Uroflowmetry was performed both pre- and post-operatively. Functional outcome was the prime measure determining success. RESULT: All the boys had a successful functional outcome and all expressed satisfaction with cosmetic outcome as well. Uroflow parameters improved remarkably, with maximum and mean urinary flow rates significantly improved from 4.2 mL/s to 26 mL/s (p = 0.0002) and from 1.6 mL/s to 12.2 mL/s (p = 0.003), respectively. Median follow-up was 34 months (range 30-42 months). DISCUSSION: This paper is the first to describe the successful use of buccal mucosal inlay grafts to treat refractory BXO stricture in children. Various surgical techniques have been proposed particularly in adults including single vs. staged procedures, preputial and post auricular grafts, circular mucosal buccal grafts, as well as double mucosal grafts placed both ventrally and dorsally. All of these procedures are not without problems, and had variable results and outcome. Most of the procedures have been described in adult urethra and are not suitable for paediatric small calibre urethra. Our technique of dorsal inlay graft gives a robust structure to the distal urethra avoiding diverticula, pooling or urine spraying and can be placed onto proximal urethra as well. We have not seen recurrence of BXO in our buccal grafts, which is reported in other grafts from prepuce and post auricular grafts. The number of patients presented in our study is limited, nonetheless an excellent result in all our cases makes this a compelling approach to manage BXO urethral strictures. An early intervention in such cases is paramount to convert a salvage operation to a pre-emptive procedure. CONCLUSION: A single stage buccal mucosal inlay grafting in children with BXO stricture can offer an excellent outcome.

The diagnosis and management of male genital lichen sclerosus: a retrospective review of 301 patients.

INTRODUCTION: Male genital lichen sclerosus (MGLSc) is an acquired, chronic, inflammatory skin disease that is associated with significant morbidity and squamous cell carcinoma of the penis (PSCC). However, some clinical, diagnostic and management controversies endure, including the relationship with penile intraepithelial neoplasia (PeIN). OBJECTIVES: To clarify clinical presentations, diagnostic approaches, histological findings, response to treatment and the relationship with PeIN. METHODS: Retrospective review of patients with a diagnosis of MGLSc who attended a specialist male genital dermatoses clinic. RESULTS: 301 patients were identified: 260 had isolated MGLSc and 41 both MGLSc and PeIN. Referrals were made from the local Urology and Andrology departments (128), primary care (89), GUM (54), other dermatology departments (28) and other specialties (2). In isolated MGLSc, 94.6% were diagnosed clinically with 93.5% accuracy (based on data from subsequent circumcisions). In combined MGLSc/PeIN, 85.4% were diagnosed following diagnostic biopsy and 14.6% retrospectively after circumcision. In isolated MGLSc, 50% were treated topically, and 50% required surgery. In MGLSc/PeIN, 78% required surgical interventions. In isolated MGLSc, 92.2% achieved resolution of symptoms, 3.5% were awaiting procedures, and 4.8% were receiving ongoing topical therapy. In MGLSc/PeIN, 90.2% achieved clearance, 2.4% were waiting surgery, and 7.3% were treated topically. Only 2.7% reported ongoing symptoms, all in patients treated surgically. None progressed to PSCC. DISCUSSION: MGLSc is generally a disease of the uncircumcised; the majority of cases of MGLSc are accurately diagnosed clinically; suspected PeIN or PSCC requires histological confirmation; circumcision histology can be non-specific; most men are either cured by topical treatment with ultrapotent corticosteroid (53.1%) or by circumcision (46.9%); surgical intervention is required in most cases of concomitant MGLSc and PeIN; the majority of patients with MGLSc alone or with MGLSc and PeIN remit with this approach; effective management appears to negate the risk of malignant transformation to PSCC.

[Clinical and immunohistochemical correlation of balanitis xerotica obliterans]. / Correlación clínica e inmunohistoquímica de la balanitis xerotica obliterans.

AIM: Balanitis xerotica obliterans (BXO) is a disease of the skin and mucosa of male genitals of unknown etiology that may affect children of any age. It has a low incidence (9-19%) and in adults is considered a potential premalignant lesion. The aim of our study is to establish the incidence of BXO in our center and to determine its correlation between the clinical and immunohistochemical (IHC) findings. METHODS: Prospective cohort including all children < 14 years with foreskin pathology that required a circumcision between 2014-2016. Statistical analysis of the clinical characteristics, histological and IHC findings searching for inflammatory response, premalignant lesions and microbiological findings. RESULTS: A total of 176 boys with phimosis had circumcision with a mean age of 7 ± 3 years (Range 2-14). Presurgical diagnosis of BXO was suspected in 28.4% (n= 50) whereas the AP confirmed a total of 29.5% (n= 53) with a very good interobserver concordance (kappa= 0.81: p< 0.01). Previous treatment with corticoids in BXO was found in 63.5% (n= 33/52). Meatal stenosis was found in 7.69% (n= 4/52) requiring meatal/urethral dilations. Patients with BXO had a T-Lymphocytes CD3+ mediated inflammatory response with a positive correlation between tumor suppressing protein (p53) expression and chronic inflammation. CONCLUSIONS: BXO is a chronic inflammatory disease mediated by T-lymphocytes with an incidence greater than previously reported. Surgeons' criterion has a very good concordance with the AP findings. The elevation of p53 in children with BXO may indicate a plausible malignant potential that may require a surgical treatment (circumcision) and an adequate follow-up.

OBJETIVO: La balanitis xerotica obliterans (BXO) es una enfermedad de etiología incierta, que afecta a piel y mucosa de genitales masculinos de cualquier edad. La incidencia en niños es baja (9-19%) y en adultos se considera una lesión premaligna. El objetivo de este estudio es establecer la incidencia de BXO en nuestro centro y determinar la correlación entre las características clínicas y los hallazgos inmunohistoquímicos (IHQ). METODOS: Cohorte prospectiva de niños ≤14 años con fimosis circuncidados entre 2014-2016. Análisis estadístico de las características clínicas e histológicas e IHQ para valorar la respuesta inflamatoria, presencia de lesiones premalignas y asociaciones microbiológicas. RESULTADOS: Se incluyeron 176 pacientes circuncidados con una edad media de 7 ± 3 años (rango 2-14 años). La sospecha clínica de BXO, 28,4% (n= 50), se confirmó mediante anatomía patológica en 29,5% (n= 52) con muy buena fuerza de concordancia interobservador (kappa= 0,81: p< 0,01). El 63,5% (n= 33/52) recibieron corticoterapia como tratamiento inicial. El 7,69% (4/52) presentaron estenosis meatal requiriendo dilataciones meatales/uretrales. Los casos de BXO presentaron una respuesta mediada por linfocitos-T: CD3+ (p< 0,01) y correlación positiva con la sobreexpresión de proteína supresora de tumores (p53) (p< 0,01). CONCLUSIONES: BXO es una enfermedad inflamatoria crónica mediada por linfocitos-T con una incidencia mayor a la reportada. La concordancia interobservador entre la sospecha de BXO y la confirmación histológica es muy buena. La elevación de p53 en los pacientes con BXO indica un posible potencial maligno que requiere tratamiento quirúrgico (circuncisión) y un seguimiento adecuado.

Bleeding after circumcision is more likely in children with lichen sclerosus (balanitis xerotica obliterans).

INTRODUCTION: Over 27,000 circumcisions were performed in England in 2012-13. The complication rate is generally perceived to be low, although published figures vary widely. Balanitis xerotica obliterans, more correctly termed Lichen Sclerosus et atrophicus (LS), is one of the commonest indications for medical circumcision. To test the hypothesis that children undergoing circumcision for LS have a higher rate of postoperative bleeding than those undergoing the procedure for other reasons, we retrospectively reviewed records for patients undergoing circumcision. METHODS: The disease and procedure coding system was used to identify patients who underwent circumcision (ICD10 code N303) between 2000-2010. Cases with a diagnosis unrelated to circumcision and children circumcised during hypospadias repair were excluded. Bleeding which required return to theatre for surgical arrest was considered significant. Cases were identified by review of medical records if there was: a second procedure during the same admission, or readmission coded for circumcision within 2 weeks. Only cases with histologically confirmed LS were included in the LS cohort. GraphPad online calculator was used for statistical analysis (two tailed Fisher's exact test. RESULTS: 2385 boys with a median age of 4 years (range 0-16) were included in the study. Indication for circumcision included religious (1305, 54.7%), phimosis or redundant prepuce (512, 21.5%), suspected LS (366, 15.4%) and balanoposthitis (202, 8.5%). LS was histologically confirmed in 262 (10.9%) boys. Fourteen (0.6%) patients returned to theatre for surgical arrest of bleeding following circumcision; 6 had LS and 8 did not (Table 1). The bleeding rate was higher in those with LS (2.3%) than in those without (0.3%), P = 0.0003 with a relative risk of 6.08. CONCLUSION: Post-operative complications are distressing, especially if further surgery is required. Published figures for complications following circumcision vary widely making counseling regarding risk difficult. Since LS includes an inflammatory element and circumcision in widespread LS can be challenging, the observation of more post-operative bleeding in patients with histologically confirmed LS during a previous audit prompted the hypothesis that this may be a significant finding. Thus we reviewed all patients requiring return to theatre within 2 weeks of circumcision, finding that whilst the overall bleeding rate was low, circumcision for LS significantly increased the risk. Although factors such as the severity of LS and surgical technique were not assessed, this is still a notable finding which should be reflected during pre-operative counseling.

Clinical Features, Complications and Autoimmunity in Male Lichen Sclerosus.

Lichen sclerosus is a chronic inflammatory disease associated with substantial morbidity. Knowledge of the aetiology and progression of lichen sclerosus is therefore needed. In this cross-sectional study, 100 male patients diagnosed with lichen sclerosus were interviewed and examined. Since there is a possible link between lichen sclerosus and autoimmunity, blood tests were analysed for thyroid disease, antinuclear antibodies and antibodies to extracellular matrix protein 1, but autoimmunity was found to be infrequent. In 72 participants active genital lichen sclerosis was observed and complications were common; 27 patients had preputial constriction and 12 meatal engagement. In total, 13 patients needed a referral to the Department of Urology, including 1 patient with suspected penile cancer. In conclusion, despite available treatment with ultra-potent steroids and circumcision, lichen sclerosus in males is frequently complicated by phimosis and meatal stenosis. However, the disease can also go into remission, as seen in 27% of our patients.

Common urological problems in children: prepuce, phimosis, and buried penis.

Parents often bring their children to the family doctor because of urological problems. Many general practitioners have received little training in this specialty. In this review, we aimed to provide a concise and informative review of common urological problems in children. This review will focus on the prepuce.

Incidence of balanitis xerotica obliterans in boys younger than 10 years presenting with phimosis.

OBJECTIVE: Evaluation of the true incidence of balanitis xerotica obliterans (BXO) among boys younger than 10 years. METHODS: In a period of 13 months, 75 boys younger than 10 years were treated for phimosis. Suspicion of BXO was raised in phimosis grade 2 or 3 (classification by Kikiros). Patients were offered primarily either circumcision or conservative therapy and circumcision secondarily (if treatment failed in the conservative group). Each circumcision specimen was examined histopathologically. RESULTS: Circumcision was primarily performed in 29 and secondarily in 17 patients. The mean age was 3.7 years (range 1-10). BXO, chronic inflammation, and normal histological results were found in 8/26/12 (17.4/56.5/26.1%) cases, respectively. The mean follow-up was 8.1 months. No recurrences were reported. CONCLUSIONS: The incidence of BXO appears to be higher than previously reported. The clinical appearance in children may be confusing. The preoperative BXO suspicion did not correlate with the final histopathological results.

Diagnosis and treatment of lichen sclerosus: an update.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.