Refot-Arka: Belgrad, Yugoslavia; 1999. A positive attitude towards micropenis and the male gender role can be achieved by patients and parents, but mental success mainly depends on parental enthusiasm, openness, and sufficient knowledge about the anomaly. In this case, further evaluation with a multidisciplinary team is recommended. Gargollo PC, Borer JG. In cases with bad bladder development, upper tract deterioration and continence is not achievable over a reasonable period and a well-balanced benefit-effort-analysis urinary diversion should be performed. It may be related to improper development of the pubic bone. During cystoscopy, a defect of the external sphincter can be identified as a longitudinal attenuated tissue strip from the bladder neck through to the urethral sphincter. Philadelphia, PA: Elsevier; 2020:chap 556. Mathews RI, Gan M, Gearhart JP. Gearhart JP. As your child nears adulthood, it is especially important that the care they receive remains effective and streamlined. That is why many babies are diagnosed after they are born. The treatment of bladder exstrophy consists of a series of corrective surgeries performed over several years. Most girls with exstrophy also have epispadias. The position of the meatus is important because it predicts the degree to which the bladder can store urine (continence). This urethral tissue must be surgically removed. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Due to normal internal genitalia, mainly not affected by the reconstructive bladder surgery, fertility should usually be normal. World J Urol. Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls. Read more about causes of Epispadias. Classic bladder exstrophy is intermediate in severity and the bladder is open from the top of the bladder through the urethra and to the tip of the penis. Gearhart JP, Di Carlo HN. Furthermore, as a consequence of a low cervical insertion an even a higher chance for pregnancy is assumed. Less often, the opening is at the middle or the base of the penis. Epispadias is associated with bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall., Bladder Exstrophy-Epispadias-Cloacal Exstrophy Complex. Still, there is a paucity of knowledge about pelvic floor anatomy after reconstruction and sparse reports have failed to determine risk factors for this major complication. Updated by: Scott Miller, MD, Urologist in private practice in Atlanta, GA. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. However, Matthews reported a series of 83 female exstrophy-epispadias complex patients, who had a late onset of sexual activity with a mean age of 20.2 years despite appropriate sexual desire 32). Curr Opin Urol. However, gaining anal continence in childhood after urinary diversion is an individual process until the child is about 5-7 years old. In this condition, the urethra does not develop into a full tube. Urologic disorders. Urology. A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission ( Review provided by VeriMed Healthcare Network. Woodhouse was the first who revealed that bladder function in exstrophy-epispadias complex is not stable over time, and late failure with muscular atony may occur 8). Pediatric Urology. If the bladder neck resistance is low, injectable materials like dextranomer/hyaluronic acid can enforce urethral resistance 14). If left untreated, more severe forms of hypospadias can interfere with sexual intercourse when your child is an adult. Who gets epispadias? The urine exits the body from the wrong place. J Urol. Footnote: Male newborn with classic bladder exstrophy. 's editorial policy editorial process and privacy policy. (2015). Epispadias can occur in both boys and girls who are otherwise healthy with no other abnormalities. Continence is often achieved by several operations, and is not a result of learning and developing processes 42). Patient age, social background and life style should be taken into consideration to decide whether a catheterizable pouch or a sigma-rectum-pouch is chosen for urinary diversion. 2001 May; 87(7):623-8. 2006 Oct; 98(4):849-53. Recent long-term results regarding fertility in the exstrophy-epispadias complex do sparsely exist 24). The causes of epispadias are not known. The bladder exstrophy-epispadias-cloacal exstrophy complex. Some people with epispadias may continue to have urinary incontinence, even after surgery. The urine exits the body from the wrong place with epispadias. Further relevant information on causes of Epispadias may be found in: By using this site you agree to our Terms of Use. There are some reports about adenocarcinomas and squamous cell carcinomas occurring in unreconstructed, environment-exposed exstrophic bladders 47). Valerio, Enrico & Vanzo, Valentina & Zaramella, Patrizia & Salvadori, Sabrina & Castagnetti, Marco & Baraldi, Eugenio. Exposed, everted bladder template is clearly visible immediately below umbilical stump; a completely dorsally opened (epispadic) urethral plate runs from bladder neck down to the open glans; left and right corpora cavernosa are visible beneath and alongside urethral plate; the scrotum is caudally displaced. The first surgery is closure of the bladder to allow it to hold urine, placement of the bladder inside the pelvis and closure of the abdominal wall. Figure 4. In: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Osteotomy, however, is hardly recommended in epispadias. Hypospadias is a birth defect (congenital condition) in which a baby boy’s urethra is located on the under side of his penis rather than at the tip 52). Boys with epispadias have a urethra that is extremely short and split and the opening is on the upper surface of the penis. 5. In most cases, the exact cause of hypospadias is unknown. Woodhouse CR, North AC, Gearhart JP. Exstrophy-epispadias complex is characterized by a visible defect of the lower abdominal wall and other problems. Nelson Textbook of Pediatrics. These include a separation of the pubic bones, absence of the lower portion of the bladder causing lack of bladder control (incontinence) and abnormal position of the tubes that carry urine from the kidneys to the bladder (ureters) causing back up of urine in the kidneys (reflux). Causes of Epispadias. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. Normal urethra in boys, that tube goes to the tip of the penis and also carries semen. Exstrophy-epispadias complex refers to a spectrum of birth defects that includes epispadias, classical bladder exstrophy, and exstrophy of the cloaca and several variants 1). Long-term data suggest that functional bladder neck reconstruction with a consequent anatomical placement of the colliculus seminalis in the posterior urethra, however, allows antegrade ejaculations in 94.1% of the patients 28). Identifying the medical problem, with simultaneous consideration of the individual and family history, should lead to further therapeutic recommendations. In epispadias with relevant urinary incontinence and a wide sphincter defect, a complete bladder neck procedure is needed; in mild defects, approximation of the pelvic floor during penile procedure might be sufficient. Ebert AK, Reutter H, Ludwig M, Rösch WH. Causes of Epispadias. Rösch WH, Schott G, Scheuering S, Schrott KM. Bladder exstrophy (classic bladder exstrophy). Epispadias can also occur with other birth defects. In general, female exstrophy-epispadias complex patients require comparatively little surgery with mostly acceptable cosmetic outcome.