Epispadias is a congenital condition whereby the vagina or penis is malformed, in particular the urethra. Epispadias can even include a double clitoris.
When it involves the bladder and urethra, and other pelvic structures, epispadias is more often called the exstrophy-epispadias complex.
Epispadias is rarely found by itself. Exstrophy is where the bladder and other pelvic structures are outside the abdomen, with other pelvic malformations.1
While the foetus is developing, certain events happen at different, but very specific, times. Epispadias is essentially a timing error that results in the external genitalia malforming, and sometimes the bladder and large intestine too. It varies in severity.
The more structures involved, the more severe the epispadias. Epispadias affects boys and girls, however this article only deals with girls, for whom epispadias is far rarer. Epispadias affects only one in 484,000 live births.2
How epispadias affects girls
Epispadias results in the pubic bones being separated, with the result being that the clitoris doesn’t fuse during development leaving two halves.
The bladder neck is almost always affected, which causes leaking urine when the bladder is pressured (coughing, lifting). In most cases, surgery resolves these problems.
Diagnosis of epispadias in girls
Epispadias is typically observed at birth, but very minor epispadias can be missed, only becoming apparent when the child remains wet after toilet training.
There is no evidence to suggest that babies born with epispadias are any worse off in any other way, and the incidence of other issues is low. It can be expected that the child’s problems remain isolated to the pelvic region.
Deeper issues need not be examined without cause, though boys may experience problems with fertility logistics.3 Girls generally see no such issues, as their reproductive organs are intact.
Any child born with severe exstrophy-epispadias complex is at a slightly increased risk of other malformations (ureters, vesicoureteral reflux).
Treatment of epispadias in girls
Treatment depends on the problems in the particular child, and is surgery in all cases.
Some children will have complete exstrophy-epispadias, and there is an increased risk of urinary tract infections (vesicoureteral reflux), in which case, the children remain on antibiotics until the reflux is corrected as a preventative for UTIs.
In girls the main treatment outcomes are cosmetically-pleasing outer genitalia, and if the bladder and bladder neck are involved, urinary continence and fertility preservation techniques as needed.4,5
Surgical options for epispadias in girls
The surgical techniques differ in boys and girls, with the female reconstruction of the bladder less complex compared to boys. The urethra and vagina can be short and nearer to the front of the body, and the clitoris split into two parts.6
Other internal reproductive organs are normal (uterus, fallopian tubes, ovaries). If diagnosed at birth, the clitoris can be brought together and the urethra replaced in a normal position. If the operation happens early enough, continence can be saved.
If diagnosis is missed and early repair not performed, incontinence can be surgically corrected later in life. A narrow vaginal entrance in older girls or younger women can be reconstructed after puberty.7
Exstrophy-epispadias complex
If exstrophy-epispadias complex is present, further surgery is likely to be required to improve urethral resistance. This involves bladder neck repairs. Newer methods of surgical repairs at birth mean that almost one-third of girls can achieve complete urinary control without further surgeries.
Urinary control of girls with epispadias8
With children who don’t potty train normally, other methods can be used to get the urinary function up to standard. This could include injecting a bulking material around the bladder neck, solving leakage problems.
Other surgical methods may include creating a longer urethra or wrapping other materials around the bladder neck. Early repair is the best course of action, with early bladder storage and emptying promoting proper bladder growth and function.
Surgery used to be delayed until one year after birth, but some surgeons are indicating a preference for earlier repair for better outcomes. Discuss this with your surgeon.
Outcomes of epispadias in girls
Old treatments are being phased out as new surgical methods improve outcomes.9 The formation of an abnormal hole in the urethral tube used to be common, but is now much less common (about six per cent).
Natural medical support for epispadias in girls
The role of natural medicine and alternative treatments in epispadias is in support only, since the appropriate treatment is surgical in nature. Natural medicine can play a role in supporting healing after surgery, and proper tissue repair, reduction in scarring, and calming parents down.
Supporting a young body to repair itself fully is the end goal, but supporting parents throughout these surgeries is important too. Parents may need extra support during this time, to cope with sleep deprivation, stress, and fear.
Having your new baby undergo serious surgery for an anatomical abnormality is frightening, and fear for your child’s wellbeing not just at that time, but for the future, weighs heavy.
Your surgeon and specialists are the only people who will know precisely the situation of your child, so they are the best people to speak to.
Ensure any natural medicine practices you choose to employ are done so under the direction of a qualified, experienced paediatric practitioner.
Helpful natural medicine treatments for a baby may include:
- Herbal creams applied externally for wound healing
- Reflexology on the feet or hands to support healing and relaxation
- Extra probiotic support if antibiotics are given
- Breastfeeding
Natural medicine treatments for older children may include:
- Herbal medicine for urinary tract support
- Herbal creams applied to assist wound healing and tissue repair
- Reflexology on the feet or hands
- Extra probiotic support if given antibiotics
- Counselling to help them manage any unexpected feelings associated with their genitals, any disability they face, or fears about surgery
Natural medicine treatments for parents may include:
- Herbal medicine for stress relief and sleeping
- Counselling to support parents through difficult choices, fear, and stressful family situations
See your healthcare practitioner for advice.
References
- 1.Ebert AK, Reutter H, Ludwig M, Rösch WH. The Exstrophy-epispadias complex. Orphanet J Rare Dis. Published online October 30, 2009. doi:10.1186/1750-1172-4-23
- 2.Jonuzi A, Popović N, Zvizdić Z, Milišić E, Karavdić K, Dewan P. Female Epispadias Presenting as Urinary Incontinence. APSP J Case Rep. Published online March 21, 2017:10. doi:10.21699/ajcr.v8i2.548
- 3.Ansari M, Cervellione R, Gearhart J. Sexual function and fertility issues in cases of exstrophy epispadias complex. Indian J Urol. Published online 2010:595. doi:10.4103/0970-1591.74477
- 4.Zhu X, Klijn AJ, de Kort LMO. Urological, Sexual, and Quality of Life Evaluation of Adult Patients With Exstrophy-Epispadias Complex: Long-term Results From a Dutch Cohort. Urology. Published online February 2020:272-277. doi:10.1016/j.urology.2019.10.011
- 5.Merino-Mateo L, Cabezalí Barbancho D, Tordable Ojeda C, Gómez Fraile A. Sexual function in adolescents and young adults (AYA) with Bladder exstrophy-epispadias complex: Influence of the incontinence. Journal of Pediatric Surgery Open. Published online January 2024:100078. doi:10.1016/j.yjpso.2023.100078
- 6.Inouye BM, Tourchi A, Di Carlo HN, Young EE, Gearhart JP. Modern Management of the Exstrophy-Epispadias Complex. Surgery Research and Practice. Published online 2014:1-9. doi:10.1155/2014/587064
- 7.Oyinloye AO, Wabada S, Abubakar AM, Rikin CU. Delayed presentation of isolated female epispadias with urinary incontinence: a case report. Ann Pediatr Surg. Published online September 12, 2022. doi:10.1186/s43159-022-00206-y
- 8.van Geen FJ, Nieuwhof-Leppink AJ, Wortel RC, de Kort LMO. Bladder Exstrophy-Epispadias Complex: The effect of urotherapy on incontinence. Journal of Pediatric Urology. Published online May 2024. doi:10.1016/j.jpurol.2024.05.013
- 9.Morrill CC, Haffar A, Harris TGW, Crigger C, Gearhart JP. Current management of classic bladder exstrophy in the modern era. Afr J Urol. Published online May 22, 2023. doi:10.1186/s12301-023-00356-4
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