Urethral Anomalies and Urethral Prolapse in Children Clinical Presentation

Updated: Jul 10, 2019
  • Author: Jyoti Upadhyay, MD, FAAP; Chief Editor: Marc Cendron, MD  more...
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Presentation

History

Because lacuna magna, anterior urethral valves (AUVs), congenital urethral polyps, and Cowper duct cysts present with quite similar complaints, careful consideration of these urethral anomalies, along with a complete physical examination and appropriate imaging studies, is required to reach the correct diagnosis.

Children with lacuna magna can report intermittent postvoid spotting, hematuria, dysuria, or severe pain in the vicinity of the glans during voiding.

Most patients with incomplete urethral duplications are asymptomatic; however, those with complete duplications may complain of urinary incontinence, discharge from the accessory urethra, double urinary stream, or symptoms of urinary tract infections (UTIs).

Infants with AUVs present with severe bladder outlet obstruction. Older children present with irritative voiding symptoms, weak stream, incontinence, dribbling, or UTIs. Emptying of the valve diverticulum or overflow incontinence may cause dribbling.

Diverticula of the anterior urethra can also present in infancy with urinary obstruction or retention and, later, as UTIs or dribbling.

Symptoms of congenital urethral polyps in children include intermittent bladder obstruction, hesitancy, hematuria, dysuria, splayed urinary stream, and UTI.

Cowper duct cysts are manifested by hematuria, passage of mucous plugs, dysuria, hesitancy, intermittent obstructions, and postvoid dribbling. These symptoms become more apparent in adolescent males.

Female patients with urethral prolapse usually present with vaginal bleeding, [10, 11] spotting, dysuria, urinary frequency, introital pain, and, occasionally, urinary incontinence or retention.

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Physical Examination

Many of the conditions discussed in this article may be suggested by the history; however, the anomalies may not be obvious upon physical examination. Some of these conditions are clearly evident.

In patients with urethral duplications, dorsal curvature of the penis (in the epispadiac type), double stream, or multiple meatus can be present.

In children with anterior urethral valves and congenital urethral diverticula, the urethra may bulge on the ventral aspect of the penis while voiding and the stream may be weak.

In boys with fusiform megalourethra, the penis is flabby and soft because of the absence of corpora cavernosa, and the urethra balloons during micturition. Unfortunately, fusiform megalourethra is usually an autopsy finding because this condition is often associated with stillbirths.

Occasionally, urethral polyps prolapse through the meatus, or a perineal mass can be palpated in patients with a Cowper duct cyst.

The physical findings in girls with urethral prolapse are quite striking. A doughnut-shaped anterior vulvar mass completely surrounding the urethra is pathognomonic. Urethral prolapse is the only condition in which a circumferential mass surrounding the urethra can be found. The mass appears as an edematous fleshy rosette that may become ulcerated, necrotic, or gangrenous.

Demonstration of the meatus in the center of the edematous tissue is important for diagnosis. Finding the urethral meatus is critical in making the diagnosis of urethral prolapse. The meatus can be located by inspecting the urethra, placing a Foley catheter, or watching the child void. 

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Complications

Morbidity of the sinus of Guérin (ie, lacuna magna) is limited to those few symptomatic patients who present with dysuria, hematuria, or hematospermia.

Although most patients with incomplete urethral duplications are asymptomatic, patients with complete duplications can present with urinary obstruction, incontinence, infection, or double stream.

Epispadiac duplications are associated with a significant dorsal curvature of the penis. In general, associated upper urinary tract anomalies are infrequent but should be investigated.

In at least 50% of patients with AUVs, associated hydronephrosis or vesicoureteral reflux (VUR) is present. Although considerably more attention has been focused on posterior urethral valves (PUVs), AUVs can be just as obstructing and devastating, in some cases leading to chronic renal failure or end-stage renal failure.

The prognosis for patients with megalourethra depends on the number and severity of the associated anomalies. Often, patients with fusiform megalourethras have associated lethal congenital malformations.

Urethral diverticula, polyps, and Cowper duct cysts are benign conditions that may cause urinary retention, hematuria, irritative voiding symptoms, dribbling, or urinary tract infections (UTIs). Because these lesions may be obstructive, evaluation of the upper urinary tracts is essential.

Urethral prolapse may result in dysuria, hematuria, blood spotting on the underwear, or bleeding and, if unattended, can lead to necrosis of the prolapsed portion of the urethra.

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