Balanitis Xerotica Obliterans in Boys

Balanitis xerotica obliterans (BXO) is a chronic dermatitis of unknown etiology that occurs on the penile skin. It most often involves the glans and prepuce but sometimes extends into the meatus and anterior urethra. It was first described in 1928 by Stuhmer in 9 adult patients, and at that time was thought to be etiologically related to exposure of the glans after circumcision.¹ This inference was later refuted by Freeman and Laymon, who noted in 1941 that the disease entity described as BXO was a variant of lichen sclerosus et atrophicus confined to the male penis.² The first published report of BXO in a child is credited to Catterall and Oates, who in 1962 described a 7-year-old boy with disease isolated to the foreskin.³ Since then, there have been multiple reports of this entity in children.^4-7

Although circumcision is curative in the majority of boys with BXO confined to the prepuce, progression of the disease to involve the meatus and anterior urethra is not uncommon. Furthermore, previous studies have failed to define the optimum treatment strategy for patients who have meatal or glanular involvement. We sought to examine a cohort of pediatric patients with a pathological diagnosis of BXO with regard to referral history, clinical presentation, operations performed and postoperative course. We also sought to determine which factors, if any, predispose children with BXO to a more aggressive disease course.

MATERIALS AND METHODS

The pathology database at our institution was retrospectively reviewed for all cases with a tissue diagnosis of BXO from 1992 to 2002. Patients were excluded if they were older than 18 years.

Patient charts were reviewed and the referral diagnosis, specialty of the referring physician, clinical presentation, symptoms, medical history and radiographic studies were recorded. Operative notes were reviewed to assess the degree of penile involvement with BXO. Specific attention was given to glans involvement, meatal stenosis, urethral stricture and involvement of the shaft skin. The severity of phimosis found at the time of surgery (if reported by the surgeon) was graded according to criteria defined by Meuli et al, ie grade 1—full retractability of the prepuce and stenotic ring in the shaft, grade 2—partial retractability with partial exposure of the glans, grade 3—partial retractability with exposure of the meatus only and grade 4—no retractability.⁸ Postoperative records were reviewed to assess recurrence of disease and the need for further interventions.

Patients were divided into 3 groups based on the clinical course of the BXO (table 1). Group 1 (mild course) included patients who were cured after undergoing circumcision and had no recurrence. Group 2 (moderate course) consisted of patients who required 1 major or more than 1 minor surgical procedure, or required emergent treatment (acute urinary retention). Group 3 (severe course) included patients who required more than 1 major reconstructive procedure.

The Mann-Whitney test was applied to compare age distributions between the 3 groups. The unpaired Fishers exact test was used to calculate the 2-sided p value comparing meatal stenosis in patients with and without involvement of the glans with BXO.

RESULTS

A total of 42 patients with pathological confirmation of BXO were identified between 1992 and 2002. All specimens showed lymphocytic infiltration in the upper dermis, hyali- nosis and homogenization of collagen, basal cell vacuolation, atrophy of the stratum Malpighii and hyperkeratosis. One patient who was 21 years old was excluded, leaving a cohort of 41 patients for analysis. Average patient age was 10.6 years (range 4 to 17) and 85% of patients were 4 to 13 years old. All patients had complete medical records available for review.

A total of 40 patients were referred to our department for evaluation. Two referrals were made by urologists, and the remainder were made by pediatricians (35) or emergency medicine physicians (3). The most common referral diagnoses were phimosis (52%), balanitis (13%) and buried penis (10%). None of the referrals by pediatricians or emergency medicine physicians identified BXO as the primary diagnosis. After evaluation by a pediatric urologist 36 patients (88%) were correctly diagnosed with BXO preoperatively. Of the 5 patients who were not diagnosed by a pediatric urologist preoperatively only 1 had the characteristic white, sclerotic, distal preputial ring.

A total of 19 patients (46%) with an average age of 9.5 years were categorized into group 1 (mild disease course) and underwent circumcision or redo circumcision. The most common presenting symptoms for these children were dysuria (42%), ballooning of the foreskin with voiding (32%) and splaying or deviation of the urinary stream (26%, table 2). Of these patients 32% had grade 1 or 2 phimosis at surgery and 68% had grade 3 to 4 phimosis. The glans was involved by disease in 26% of the patients. One patient had meatal stenosis. These patients had no recurrence of BXO after circumcision at a mean followup of 9 months (range 1 to 55).

A total of 16 patients (mean age 11 years) were categorized into group 2 (moderate disease course). Three of these patients (19%) presented in acute urinary retention and underwent emergent surgery (circumcision, dorsal slit or circumcision with meatoplasty). The 3 most common symptoms in this group were dysuria (50%), obstructive symptoms or complete obstruction (44%) and ballooning of the foreskin with voiding (25%, table 2). Of these patients 12% had grade 1 or 2 phimosis at surgery and 88% had grade 3 to 4 phimosis. The glans was involved in 81% of patients and there was meatal stenosis in 56%. Of these patients 37% underwent circumcision combined with meatotomy, meatoplasty or distal urethral cutback,⁶ and 44% required reconstructive operations to release a concealed penis with concomitant meatoplasty required in 3 (table 3).⁷

Six patients (mean age 13 years) were categorized into group 3 (severe disease course). Of these patients 67% presented with dysuria.⁴ Half of the patients had a completely entrapped penis at presentation and 4 had undergone multiple surgical procedures before referral. Operative course is outlined in table 4. There was a significant difference in age at presentation between patients in group 1 and patients in group 3 (p <0.05), with patients in group 3 being older.

Of the 41 patients 23 (56%) had glans involvement in the form of keratotic plaque(s) and 15 (37%) had meatal stenosis. Of the 15 patients with meatal involvement 13 (87%) also had glans involvement. In addition, 13 (57%) of the 23 patients with glans involvement also had meatal stenosis. Patients with glans involvement had significantly more meatal stenosis than those without glans involvement (p = 0.0036, table 5). Of the 15 patients with meatal involvement 1 underwent circumcision alone, and 7 underwent circumcision and meatoplasty. The remaining 7 patients underwent concealed penis release, redo circumcision and meatoplasty, meatotomy and lysis of penile adhesions, circumcision and distal urethral cutback (2), and urethroplasty with buccal mucosa (2). Two of the patients who underwent circumcision and meatoplasty had recurrence of disease and required further surgery.

DISCUSSION

BXO is a poorly understood dermatological disease involving the prepuce, glans, urethral meatus and sometimes the anterior urethra. Once thought to be a rare entity in children, it is now apparent that its incidence is more common than previously realized. We examined a cohort of patients seen at our department during a 10-year period with histologically confirmed BXO, with specific interest in referral patterns, referral diagnoses and clinical outcomes.

The onset of BXO is usually without symptoms. Initial symptoms might be mild, such as dysuria or ballooning of the foreskin. More severe symptoms usually indicate disease progression. Rickwood et al reported on 23 patients referred for phimosis in 1970, of whom 20 were found to have BXO.⁹ The 3 most common presenting symptoms were poor urinary stream, dysuria and preputial pain. Bale et al identified 54 patients with BXO in a circumcision series.¹⁰ The most common presenting symptom was inability to retract the foreskin. Discomfort with voiding occurred in 27 patients and obstructive symptoms were noted in 23. Two patients were hospitalized with acute retention and 2 had hematuria.

We examined the presenting symptoms of patients and also aimed to determine whether patients with more complicated clinical courses (groups 2 and 3) had different or higher incidences of certain symptoms than those with milder courses (group 1). The most common symptom for all groups was dysuria (49% of all boys) but a greater proportion of patients in groups 2 (50%) and 3 (66%) had dysuria than in group 1 (41%). There were also higher percentages of patients in groups 2 and 3 with obstructive symptoms, including complete obstruction requiring emergent surgery in 3 (table 2). Complete urinary obstruction in adults and children with BXO has been described previously, and obstructive symptoms are not unusual.^8,11 Catterall and Oates reported symptoms of urinary obstruction in 8 of 41 patients (20%).³ Bainbridge et al reported symptoms of urinary obstruction in 47% of their patients.¹² A greater percentage of patients in our groups 2 and 3 had deflection or changes in the urinary stream (including incontinence) than in group 1, perhaps implying worsening phimosis and meatal or urethral involvement.

We believe that recognition and early referral for treatment of BXO while the lesion is small and local are important due to the extensive tissue involvement that may ensue if diagnosis and treatment are delayed. A review of the available literature revealed an alarming incidence of missed diagnoses of BXO by pediatricians and general practitioners. Schinella and Miranda found 15 cases of BXO in children 13 years or younger.⁶ In none of these patients was a preoperative clinical diagnosis of BXO made. Huntley et al prospectively examined a group of 100 patients referred for circumcision by general practitioners.¹³ Six patients were identified as having clinical evidence of BXO, which was confirmed histologically in 4. In none of these cases was the diagnosis made by the referring clinician. Meuli et al prospectively followed 100 patients referred for circumcision by family practitioners or pediatricians.⁸ The referring provider diagnosed none of these patients with BXO, although 2 presented with urinary retention. In our study only 2 patients were correctly diagnosed by the referring provider as having BXO, both by urologists.

There are unifying clinical characteristics of BXO that should make its diagnosis relatively straightforward. Perhaps the most important of these is a ring-like distal sclerosis of the prepuce with white discoloration or plaque formation. McKay et al found this white ring in two thirds of cases.⁷ Meuli⁸ and Bale¹⁰ et al found this sign in 100% of their patients. We noted this sign in 74% of patients (14 of 19) in group 1, 88% (14 of 16) in group 2 and 33% (2 of 6) in group 3. The lower proportion of this finding in patients in group 3 is due to the fact that a majority (5 of 6) had been circumcised previously.

Age at presentation and a history of circumcision provide further clues to the diagnosis of BXO and to the possible significant variable for the diagnosis of BXO in 100 children referred for phimosis.⁸ Of children with phimosis secondary to BXO 1.8% were younger than 6 years, while 21.6% were older than 6 years. In this same series 90% of boys with BXO had development of secondary phimosis or the inability to retract a once retractile foreskin during a period of several months. In contrast only 11% of boys without BXO had development of secondary phimosis. The authors concluded that secondary phimosis with onset at school age is highly suggestive of BXO.⁸ In our study children with a more severe clinical course (group 3, mean age 13 years) were significantly older than those with a milder course (group 1, mean age 9.5 years, p <0.05). Whether this finding represents more aggressive disease in older children or a longer delay in diagnosis and institution of appropriate treatment is not clear.

It has not been established previously whether glans and/or meatal involvement with BXO is of clinical relevance. Given that the disease is locally progressive, it is possible that glans involvement leads to meatal involvement, which may eventually lead to urethral involvement. To our knowledge a relationship between glans involvement, meatal involvement and urethral involvement has not been demonstrated in the literature.

There are various anecdotal reports of glans and/or meatal involvement by BXO but the clinical relevance of these, if any, has yet to be established. Schinella and Miranda reported that 7 of 22 cases (32%) in their series had clinical evidence of glans involvement and 4 of 22 (18%) had meatal involvement.⁶ They speculated that all of the patients with meatal involvement plus 3 others who had severe adhesions of the prepuce to the glans had BXO involvement of the glans. However, they provided no concrete evidence for this conclusion.

In our study 13 of 15 boys (87%) with meatal stenosis also had glanular disease (table 5). Meatal stenosis was significantly higher in patients with glanular involvement (p 0.05). Given the locally progressive nature of this disease, this finding may be expected. Two of these boys had disease progression after meatoplasty and required additional surgery. Frank et al reported that 10 of 12 boys with meatal and submeatal stenosis had undergone previous circumcision for BXO.¹⁴ Our data, combined with the observation by Bale¹⁰ and Frank¹⁴ et al, suggest that children with BXO and meatal stenosis tend to have a more severe clinical course than those with preputial involvement alone. Thus, these patients require more frequent followup, and families should be instructed about the possible signs and symptoms of disease progression or recurrence.

Depasquale et al have proposed an interesting hypothesis.¹⁵ In a series of 300 adult and pediatric patients with BXO they noted that circumcision alone was curative in 96%. They advocated that the main purpose of the circumcision was to desiccate the glans, stating, BXO has a predilection for the warm, moist, urine exposed environment that exists under the foreskin.¹⁵ They noted several recurrences in conservative circumcisions after thorough removal of the diseased skin, and in obese patients in whom the shaft skin rolled up to form a pseudo foreskin as the penis invaginated into the pubic fat pad. Interestingly, we noted that 4 of 6 patients (67%) in group 3 were obese and had prominent pubic fat. Although in this study this observation was purely subjective, there may be some association between obesity and a more severe clinical outcome. A more likely explanation for the apparent more severe course in obese children might be that because of the invagination of the penis into the pubic fat pad, the penis is not easily visible to the parent or primary provider, and, thus, the diagnosis is delayed.

CONCLUSIONS

BXO encompasses a spectrum of clinical disease, and rapid progression of BXO is not unusual. The unpredictable nature of the disease warrants prompt diagnosis and referral to an appropriate specialist. Failure to do so may lead to extensive damage of the glans, meatus and urethra, necessitating significant reconstructive surgery. Primary care providers fail to differentiate the pathological presentation of BXO from benign phimosis in the majority of cases. Improved education of these clinicians regarding BXO and its clinical appearance is clearly warranted to prevent referral delays.

BXO should be suspected in any child with phimosis (particularly of a progressive nature) and a white sclerotic ring in the distal prepuce. Our study shows that older patients, those with BXO involvement of the meatus and those with a history of surgery for BXO tend to have a more severe and morbid clinical course. Therefore, these patients require more frequent followup.

Submitted for publication December 22, 2004.
* Correspondence: Department of Urology, Childrens Hospital Boston, 300 Longwood Ave., Boston, Massachusetts 02115 (telephone: 617-355-7796; FAX: 617-730-0474; e-mail:  David.diamond@childrens.harvard.edu).

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