Epispadias

EpispadiasEpispadias Description, Causes and Risk Factors: A malformation in which the urethra opens on the dorsum of the penis; frequently associated with exstrophy of the bladder. Epispadias is a rare congenital defect in which the opening of the urethra is in an abnormal location. In boys with epispadias, the urethra generally opens on the top or side, rather than the tip, of the penis, though it is possible for the urethra to be open the entire length of the penis. In girls, the opening is usually between the clitoris and the labia but may be in the abdomen. For boys with penopubic epispadias and girls with epispadias, the bladder neck is invariably involved; it is often wide and incompetent. This involvement directly impacts the continence mechanism of these patients and impacts the ability to achieve urinary continence. In untreated boys with epispadias continence is possible if the epispadias is located distally and the bladder neck is not involved. In girls, however, continence is invariably impact to some degree because the urethral and bladder neck ectasia are invariably involved in female epispadias. Because of the shared features with bladder exstrophy, clinicians consider epispadias as part of a spectrum of the exstrophy/epispadias complex. The causes of the disease are unknown at this time. It is believed to be related to improper development of the pubic bone. It is often associated with bladder exstrophy. However, it can also occur alone or with defects other than exstrophy. Epispadias occurs in 1 in 117,000 Newborn boys and 1 in 484,000 Newborn girls. The condition is usually diagnosed at birth or shortly thereafter. The authors believe that penopubic epispadias in the boy and epispadias in the girl should be repaired completely with a complete primary repair technique at birth, similar to classic exstrophy, to maximize the potential for urinary continence. Early primary reconstruction may decrease this risk. Uterine suspension procedures such as sacrocolpopexy procedure can be employed in these situations as well. Symptoms: Urinary incontinence is the main clinical symptom in both sexes, ranging from urine permanently dripping through the meatus in severe forms, to involuntary urine loss with stress (coughing and strenuous effort) in milder forms. Urinary incontinence may not be observed in patients with the most distal forms of epispadias. Epispadias results from an anomaly during early embryologic development associated with abnormal partitioning of the cloacal membrane causing displacement of the genital tubercle and resulting in non-closure of the urethral plate and an abnormal dorsal urethral location. Diagnosis: Diagnosis may include the following tests: Pelvic x-ray.
  • Intravenous pyelogram (IVP).
  • Ultrasound of the Genitourinary system.
  • Complete blood count & serum electrolytes.
Treatment: The ideal approach for epispadias repair should return the Genitourinary anatomy to its normal location and con?guration. This will involve the following goals: Correction of dorsal chordee.
  • Creation of a straight urethra to allow easy.
  • Negotiation during catheterization or Cystoscopy.
  • Satisfactory cosmesis.
  • Minimal complications, especially regarding urethrocutaneous ?stulas.
  • Maintenance of erectile function.
  • Creation of urinary continence (penopubic epispadias).
Epispadias repair can be challenging. The modi?ed Cantwell-Ransley and complete penile disassembly (CPD) procedures can be employed as salvage procedures for patients who have undergone previous operations for epispadias. NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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