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Abstract

Posterior urethral hemangioma (PUH) is a rare congenital lesion, included in group of polypoid or papillary lesion of the prostatic urethra. This lesion is responsible for a variety of symptoms in children that may be associated; sometimes it is an incidental finding. The diagnosis is usually made by ultrasonography and cystourethrogram, but urethrocystoscopy is diagnostic and therapeutic. We report a case of one year old boy with persisting haematuria, in whom a previous cystoscopy didn’t find any cause of haematuria. An accurate urethrocystoscopy led to diagnosis of a prostatic urethral polyp; transurethral resection was performed and pathological assessment confirmed the diagnosis of PUH.

Introduction

Macroscopic haematuria represents a common cause of pediatric urologist or nephrologist referral (1), although its prevalence is low in the general pediatric population, occurring in around to 0.5% to 2% of unselected children (2). The differential diagnosis in these cases is extensive, including glomerular disorder, tumors, calculi, hydronephrosis and trauma. 

Among the rare cause of haematuria is posterior urethral hemangioma (PUH). This rare congenital lesion, is more often seen in the male, although in the literature there are also few cases in females (3). It is included in the group of polypoid or papillary lesions of the prostatic urethra with different histopathological features (4). 

Because of their location (the pedicle is attached to the superior part of the verumontanum), PUHs are responsible for a variety of different symptoms in children, ranging from haematuria  to dysuria with stranguria, to infections of the urinary tract (5): patient can have one or more of these symptoms. In other cases, in the absence of symptoms, its finding is fortuitous (6). The diagnosis is usually made by ultrasonography and cystourethrogram, but in doubtful cases, urethrocystoscopy is appropriate, for  diagnosis and therapy. We report a case of child who presented because of persisting haematuria.

Case report

A one year old boy presented to our Pediatric Surgery Unit with persisting haematuria for 3 months. The child had been subjected to ultrasonography and cystoscopy at another hospital with negative results.

During hospitalization clinical examination revealed no abnormalities. Ultrasonography showed  a normal bladder with a echogenic structure (diameter of about 5 x 8 mm) projecting from bladder wall into lumen.

Subsequently the child underwent cystourethrography, showing a filling defect arising from the prostatic urethra (Fig. 1). Urethrocystoscopy, performed with special attention to the study of the urethra, detected the presence of a smooth pedunculated lesion localized on posterior urethra and extending into the bladder (Fig. 2). The lesion was treated and completely removed by transurethral resection and cauterization of the stalk (Fig. 3). The catheter was removed 24 h later and the child voided without difficulty. Histopathological study revealed a haemangiomatous polyp.

At 6-month follow up the patient was fine; urethrocystoscopy demonstrated a normal bladder, without pathological changes in the posterior urethra.

Discussion 

Mustafa (4) classified the polyps of prostatic urethra into two groups according to the presence of prostatic glandular tissues or not, and the possibility of malignant transformation. In the first group are included types with prostatic glandular tissues with possibility of transformation and recurrence, like ectopic prostatic tissue of urethra, or papillary adenoma. In the second group are included those without prostatic glandular tissues, such as fibroepithelial polyp, congenital urethral polyps and urethral hemangioma. In these cases, as in the present report, there is not risk of malignancy. However, some Authors (7) proposed the use of chromosomal analysis to distinguish the benign lesion from a malignant botryoid embryonal rhabdomyosarcoma, commonly arising in the prostate or urinary bladder of the children. Regarding the risk of recurrence, there is no agreement, but some Authors stress it is possible (5). 

PUHs are located between the verumontanum and the external sphincter. In a recent study, Saito (8) proposed that a high intraurethral pressure exists around the sphincter, so that the hemangioma formation may result from high venous pressure.

The embryological origin remains unknown. PUH may arise from an embryonic rest of unipotent angioblastic cells that fail to develop into normal blood vessels (9); congenital origin is supported by the presence of polyps also in newborns and in infants (10).

Ultrasonography and cystourethrogram may represent the first step for diagnosis, but are not always conclusive, as in our case. Thus an accurate urethrocystoscopy is necessary, not only to make the diagnosis, but also to treat the lesion (11). Two different techniques of endoscopic resection (electrocautery, like in our case, or laser energy) are used with similar success rate; open cystotomy is required only for selected cases (12).

In conclusion, the authors want to stress the possibility of posterior urethral hemangioma in cases of children with persisting haematuria and the need of an accurate and complete urethral evaluation for diagnosis. 

References

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3. Klee LW, Rink RC, Gleason PE, Ganesan GS, Mitchell ME, Heifetz SA. Urethral polyp presenting as interlabial mass in young girls. Urol 1993; 41:132-3

4. Mustafa M. Capillary haemangioma of verumontanum: Case report and review of the literature. Inter Urol Nefrol 2006; 38:  493-4.

5. Natsheh A, Prat O, Shenfeld OZ, Reinus C, Chertin B. Fibroepithelial polyp of the bladder neck in children. Pediatr Surg Int 2008; 24: 613-5

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7. Isaac J, Snow B, Lowichik A. Fibroepithelial polyp of the prostatic urethra in an adolescent. J Pediatr Surg 2006; 41: 29-31

8. Saito S. Posterior urethral hemangioma: one of the unknown causes of hematuria and/or hematospermia. Urol 2008; 71: 168e11- 168e14

9. Hayashi T, Igarashi K, Sekine H. Urethral hemangioma: case report. J Urol 1997;158: 539-0

10. Demircan M, Ceran C, Karaman A, Uguralp S, Mizrak B. Urethral polyps in children: a review of the literature and report of two cases. Int J Urol 2006; 13:  841-3

11. Redman JF, Lightfoot ML. Congenital posterior urethral polyp in a boy. Urol 2003; 61: 460 -1

12. Reuter VE. Urologic surgical  pathology: Chapter 9, Urethra, St. Louis, MO: Mosby-year Book, Inc; 1997: 434-59

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