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.

Post-operative steroids in boys with histologically proven balanitis xerotica obliterans reduce the need for subsequent meatal dilatation.

AIM: Post-circumcision topical steroids may reduce the number of patients with balanitis xerotica obliterans (BXO) who require subsequent meatal dilatation. However, their use has only been investigated in the presence of an abnormal urethral meatus. The aim of this study is to determine if post-operative topical steroids reduce the need for subsequent meatal dilatation in patients with histologically proven BXO independent of the appearance of the urethral meatus. To determine if post-operative topical steroids reduce the need for subsequent meatal procedures in patients with histologically proven BXO. To determine if the appearance of the meatus at circumcision affects the need for subsequent meatal procedure. METHODS: We performed a retrospective review of all paediatric patients with histologically confirmed BXO post circumcision. We excluded all those with 'chronic inflammation' or 'early BXO'. Patients were divided into groups based on whether post-operative topical steroids were prescribed. The appearance of the urethral meatus at circumcision was recorded. Primary outcome was progression to subsequent meatal dilatation. Statistical significance was calculated using Fisher's exact test. A P value < 0.05 was considered significant. RESULTS: One hundred and seventeen patients with histologically confirmed BXO were identified between October 2012 and December 2017. Fifty-nine patients were prescribed post-operative topical steroids (50%). Nine patients required subsequent meatal dilatation procedure (8%) and only one of these had been prescribed post-operative topical steroids (P = 0.0165). Abnormal meatus was noted in 24 (21%) patients. Three patients in this group required subsequent meatal dilatation compared with 6 in the group with a normal meatal appearance (P = 0.385). CONCLUSIONS: Post-operative topical steroids can reduce the need for subsequent meatal dilatation in boys with BXO. The appearance of the urethral meatus at circumcision does not predict the need for subsequent meatal dilatation.

Circumcision versus preputioplasty for balanitis xerotica obliterans: a randomised controlled feasibility trial.

OBJECTIVES: To determine: (i) feasibility for a randomised controlled trial (RCT) comparing circumcision to preputioplasty and intralesional triamcinolone (PIT) to treat balanitis xerotica obliterans (BXO) and (ii) patient outcomes to inform future study design. PATIENTS AND METHODS: Approval was obtained from the UK Health Research Authority and local Research Ethics Committee (Reference 16/NW/0364) and the trial protocol registered with ClinicalTrials.gov (NCT02854995). A total of 20 boys (aged 2-16 years) with BXO were randomised to either circumcision or PIT (online parallel group 1:1 allocation, non-blinded). Exclusion criteria were: (i) previous penile surgery and (ii) contraindication for either treatment. Follow-up (including satisfaction questionnaire) was at 6 weeks, 3 and 12 months. Data are presented as median (interquartile range [IQR]), continuous variables were compared by t-test. RESULTS: A total of 54 boys were approached over 18 months: 23 (45%) were recruited and randomised. The commonest reason for non-entry was treatment preference: 12 preferred circumcision, 18 preferred PIT. Four patients withdrew after randomisation, three did not want circumcision and one did not want PIT. The groups were similar in terms of age (median [IQR] 11 [6-12] vs 8 [7-10] years, P = 0.53) and duration of symptoms (median [IQR] 6 [6-15] vs 6 [2-24] months, P = 0.77). There were no protocol breaches, serious adverse events or postoperative meatal stenosis. There was one self-resolving haematoma after PIT and one suture granuloma after circumcision. Two boys went on to have a circumcision after PIT. Overall, satisfaction levels were high for both groups. CONCLUSION: A definitive RCT of circumcision vs PIT for BXO appears feasible, with 39% of those approached completing the trial. More families preferred PIT. A robust comparison in the form of a multicentred RCT is required.

Long-term outcomes for 2-stage urethroplasty: an analysis of risk factors for urethral stricture recurrence.

PURPOSE: To report long-term results and patient reported outcomes of staged anterior urethroplasties, and isolate risk factors for recurrence. METHODS:  We reviewed urethroplasty database for all patients who underwent staged urethroplasty from 2000 to 2017. Follow-up included a cystoscopy 4 months after their 2nd stage to assess early success, and then annual follow-up thereafter with post-void residual and symptom assessment. Stricture characteristics, etiology and graft type were analyzed with regards to success. RESULTS:  Forty-nine patients were eligible for inclusion. The median stricture length was 7 cm (3-17 cm). The early success rate demonstrated by cystoscopy at 4 months was 100%. Long-term success was 96.4% in buccal graft (BMG) only patients; however, long-term success fell considerably to 53% in patients requiring any use split thickness skin graft (STSG) in the first stage. Median follow up time was 57 months (6-240 months). On analysis, age, increased stricture length and especially the use of STSG all appeared to be associated with late recurrence. The recurrence group had longer stricture length and were more likely to be panurethral. All recurrences occurred after the initial 4-month cystoscopy with a median time to recurrence of 78 months. CONCLUSION: Staged repairs that are amenable to BMG-only repairs have high long-term success rates. Increasing stricture length and the addition of split-thickness skin graft were associated with lower success rate in staged urethral reconstruction. Patients requiring staged repairs often experience recurrence in a very delayed fashion reinforcing the need for close, long-term follow up.

Nerve-sparing circumcision: Myth or reality?

BACKGROUND: Circumcision is a common procedure. Recently, tissue-sparing approaches have become a matter of interest, and a nerve-sparing approach is described in adults. Although circumcision is common in the practice, the nerve-sparing approach has not been evaluated in the pediatric age group. OBJECTIVE: To give a contemporary evaluation of the preputium histology, challenge the phenomenon of a genuine nerve-sparing approach, and report the results of a prospective cohort contrasting the tissue-sparing fine dissection technique to the regular sleeve circumcision in the pediatric age group. STUDY DESIGN: A total of 20 healthy children between 7 and 12 years of age were enrolled in the study. All circumcisions were carried out for religious purposes, and children with any anatomical anomaly, skin lesions, or Balanitis Xerotica Obliterans were not included in the study. The first 10 children underwent regular sleeve circumcision, while the latter 10 children underwent tissue-sparing fine dissection modification of the sleeve technique. All materials obtained from the circumcision were examined by a single pathologist, and relevant tissue structures were counted and compared between the groups. RESULTS: Both techniques were satisfactory in terms of final cosmetic results, without significant complications, such as bleeding, massive edema, iatrogenic chordee, or unacceptable cosmetics. None of the children required readmission or medical intervention other than analgesics and topical moisturizing creams. Preservation of all nervous system structures, including the receptors, appeared to be not possible with macroscopic dissection techniques due to micrometer scale depth of the touch receptors. Nerve trunks were also located in less than 1-mm depth. The tissue-sparing technique could preserve significantly more vascular structures, nerve trunks, and Pacinian Corpuscles, which can be a matter of further long-term research. CONCLUSION: We propose the term "tissue-sparing" instead of "nerve-sparing" for the available techniques. The tissue-sparing technique did not affect the clinical outcomes and the postoperative course in our study. However, it showed to be superior in terms of preserving the vascular structures, nerve trunks, and Pacinian Corpuscles.

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.

Morphea patients with mucocutaneous involvement: A cross-sectional study from the Morphea in Adults and Children (MAC) cohort.

BACKGROUND: Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge. OBJECTIVE: To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort. METHODS: Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018. RESULTS: A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5 years; P < .001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P = .004). LIMITATIONS: Selection bias and limited number of affected subjects. CONCLUSION: Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.

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

Recent advances in understanding and managing Lichen Sclerosus.

Lichen sclerosus (LS), or balanitis xerotica obliterans as it was previously known, is a chronic inflammatory lymphocyte-mediated scarring dermatosis that often affects the preputial skin and glans, leading to phimosis and urethral strictures if left untreated. We present a narrative review of the literature assessing its aetiology and pathogenesis and discuss its links to penile cancer and its medical and surgical management. Possible hypotheses for the development of LS include chronic exposure to trapped urine, leading to changes in the epithelial structure. This is supported by the fact that circumcision is often curative in the early stages of the disease. Although circumcision can be curative, the use of topical steroids is typically the first-line treatment and may preserve the foreskin and forgo the need for circumcision altogether. Patients should be made aware of a possible association with penile cancer. Although the majority of cases can be treated by medical therapy and circumcision, a significant number of patients may also require penile reconstructive procedures.

Balanitis Xerotica Obliterans Associated With Differentiated Penile Intraepithelial Neoplasia and "Bramble-Bush" Elastic Fibers Accumulation: A Finding Not Specific for Penicillamine Intake.

We describe the association of balanitis xerotica obliterans and differentiated penile intraepithelial neoplasia (PeIN) with dermal elastosis and "bramble-bush" elastic fibers in a diabetic patient with no history of penicillamine intake. An 84-year-old man presented with urethral obstruction syndrome that required postectomy, meatotomy, and foreskin excision. Histopathological examination revealed changes of lichen sclerosus and differentiated PeIN. Orcein staining showed striking dermal elastosis and transepidermal elimination of elastic fibers. At higher magnification, multiple serrations and buds arising from the borders of the elastic fibers were observed giving the so-called bramble-bush appearance. Balanitis xerotica obliterans is considered a premalignant disease, and 14% of patients have evidence of PeIN. The differentiated subtype can be difficult to diagnose because of the minimal basal cell atypia, so a striking dermal elastosis, which may even occur before the neoplasm becomes invasive, may facilitate its diagnosis. The term "acquired perforating dermatosis" seems appropriate to describe those cases of perforating elastosis that occurs in adults with systemic diseases. The bramble-bush appearance of elastic fibers is not specific for penicillamine-induced elastopathy, and it may occur in other diseases, such as diabetes mellitus. This peculiar morphology of elastic fibers may be related to the enzymatic imbalance between matrix metalloproteinases and lysyl oxidase, an enzyme required for the cross-linking of elastic fibers.

Primary Urethral Tuberculosis With Urethrocutaneous Fistula in Association With Balanitis Xerotica Obliterans.

We present an unusual case of isolated urethral tuberculosis. The patient had a history of urethral strictures and persistent discharge from two peno-scrotal fistulas which was confirmed on urethrogram. He was treated with antitubercular treatment and a two stage urethroplasty.

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.

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

Objetivos: La balanitis xerótica obliterante (BXO) es una enferme-dad 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 métodos: 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

Objectives. Balanitis xerotica obliterans (BXO) is a chronic inflam-matory 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

Topographical Evaluation of Penile Lichen Sclerosus Reveals a Lymphocytic Depleted Variant, Preferentially Associated With Neoplasia: A Report of 200 Cases.

Since the seminal study of Hart and Helwig in 1975, there are few detailed pathological studies of lichen sclerosus (LS). The aims of this study were to provide a detailed histopathological description of penile LS, as well as to explore its relationship with penile intraepithelial neoplasia (PeIN) or invasive carcinoma. We evaluated 200 patients and designed a topographical approach for the histological evaluation focusing in alterations of the following anatomical layers: squamous epithelium, lamina propria, dartos, and corpus spongiosum. We documented the quantity and topographical location of stromal lymphocytes. The prevalent lesions found were epithelial hyperplasia, atrophy, PeIN, basal cell vacuolization, lamina propria sclerosis, and variable patterns of lymphocytic infiltration. Various unique patterns of stromal sclerosis were described: perivascular, globular, linear, and solid fibrosis/hyalinization; any of them were found to be diagnostic for LS. The variation in the topography and density of lymphocytes was determinant for the identification of LS morphological variants: lichenoid, band-like, lymphocytic depleted, and mixed. A major finding was the identification of the variant designated as lymphocytic depleted LS, which we considered as the morphological prototype of LS associated with penile neoplasia. The detailed description of this complex lesion presented in this study may help pathologists in practice to identify and better define LS. The identification of the special variants suggests a role of the stromal lymphocytes in the process of carcinogenesis. Confirmation of the observations with more studies is necessary to determine the significance of these findings.

Ultrasound evidence of bladder outlet obstruction secondary to lichen sclerosus et atrophicus in boys (balanitis xerotica obliterans).

BACKGROUND: Lichen sclerosus (LS), (balanitis xerotica obliterans), causes pathological phimosis. Many boys present with obstructive symptoms, the cause is usually obvious on examination so ultrasound scans (USS) of the urinary tract are not routinely indicated. We review a series of abnormal USS in boys with LS. METHODS: Retrospective note review for boys undergoing surgical treatment for LS between 2000 and 2017. Seventy-eight boys had a USS prior to surgery, those with abnormal USS form the study population. Boys with neuropathic bladder or congenital urinary tract abnormalities were excluded. RESULTS: Nineteen of 78 boys (24%), mean age 9 years, were included. Seventeen had obstructive symptoms, 13 had culture proven UTIs, 12 had new onset incontinence. On USS 3 (17%) had acute retention, 8 (78%) had an isolated post-void residual volume (PVR) >10% of estimated bladder capacity (EBC); 3 had bladder wall thickening +/- PVR >10%, 5 had upper tract changes. Symptoms resolved with successful treatment of LS. Six boys had post treatment USS, abnormalities resolved in 5. CONCLUSIONS: Clinicians should consider LS in boys presenting with UTIs, new onset incontinence and obstructive urinary tract symptoms. Routine USS are not indicated though should be considered in those with an atypical history or examination. TYPE OF STUDY: Case Series. LEVEL OF EVIDENCE: Level 4.

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.