Hypospadias
Hypospadias | |
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Different types of hypospadias | |
Pronunciation | |
Specialty | Urology, medical genetics |
Hypospadias is a common variation in fetal development of the
In most cases, the foreskin is less developed and does not wrap completely around the penis, leaving the underside of the glans uncovered. Also, a downward bending of the penis, commonly referred to as chordee, may occur.[4] Chordee is found in 10% of distal hypospadias[3] and 50% of proximal hypospadias[5] cases at the time of surgery. Also, the scrotum may be higher than usual on either side of the penis (called penoscrotal transposition).
The cause of hypospadias is unknown; however, there is likely to be a genetic component.[6] It most often occurs by itself, without other variations, although in about 10% of cases it may be part of disorder of sex development condition or a medical syndrome with multiple abnormalities.[7][8]
The most common associated difference is an
Hypospadias can be a symptom or indication of a difference in sex development,[12] but some consider that the presence of hypospadias alone is not enough to classify someone as a person with a difference/variation in sex development or as intersex. In most cases, hypospadias is not associated with any other condition.[13] Hypospadias is sometimes considered as an intersex condition by several intersex rights activist groups, who consider the repositioning of a working urethra on a child too young to consent to be a human rights violation.[14][15][16]
Presentation
Complications
There is noted to be an increase in erectile problems in people with hypospadias, particularly when associated with a chordee (down curving of the shaft). There is usually minimal interaction with ability to ejaculate in hypospadias providing the meatus remains distal. This can also be affected by the coexistence of posterior urethral valves. There is an increase in difficulties associated with ejaculation, however, including increased rate of pain on ejaculation and weak/dribbling ejaculation. The rates of these problems are the same regardless of whether or not the hypospadias is surgically corrected.[17]
Diagnosis
Presentation
A penis with hypospadias usually has a characteristic appearance. Not only is the meatus (urinary opening) lower than usual, but the foreskin is also often only partially developed, lacking the usual amount that would cover the glans on the underside, causing the glans to have a hooded appearance. However, newborns with partial foreskin development do not necessarily have hypospadias, as some have a meatus in the usual place with a hooded foreskin, called "chordee without hypospadias".[18]
In other cases, the foreskin (prepuce) is typical and the hypospadias is concealed. This is called "megameatus with intact prepuce". The condition is discovered during newborn circumcision or later in childhood when the foreskin begins to retract. A newborn with typical-appearing foreskin and a straight penis who is discovered to have hypospadias after the start of circumcision can have circumcision completed without concern for jeopardizing hypospadias repair.[19][20]
Treatment
Intersex topics |
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Where hypospadias is seen as a
Surgery can extend the urinary channel to the end of the penis, straighten bending, and/or change the foreskin (by either circumcision or by altering its appearance to look more typical ("preputioplasty"), depending on the desire of the patient. Urethroplasty failure rates vary enormously, from around 5% for the simplest repairs to damage in a normal urethra by an experienced surgeon, to 15-20% when a buccal graft from the inside of the mouth can be used to extend a urethra, to close to 50% when graft urethral tubes are constructed from other skin.[21]
When the hypospadias is extensive–third degree/penoscrotal–or has associated differences in sex development such as chordee or cryptorchidism, the best management can be a more complicated decision. The world standard (UN and WHO) forbids nonessential surgery to produce a "normal" appearance without the informed consent of the patient,[22] and the American Academy of Pediatrics currently recommends but does not require the same standard. The AAP Textbook of Pediatric Care states "Gender assignment in patients with genital ambiguity should be made only after careful investigation by a multidisciplinary team; increasingly, surgical decisions are delayed until the child is able to participate in the decision-making process."[23] A karyotype and endocrine evaluation should be performed to detect intersex conditions or hormone deficiencies that have major health risks (i.e. salt-wasting). If the penis is small, testosterone or human chorionic gonadotropin (hCG) injections may be given with consent to enlarge it before surgery if this will increase the chance of a successful urethral repair.[3]
Surgical repair of severe hypospadias may require multiple procedures and
Age at surgery
The results of surgery are probably not influenced by the age at which repair is done.[28][29] Teens and adults typically spend one night in the hospital after surgery.[citation needed]
Preoperative hormones
Hormones potentially increase the size of the penis, and have been used in children with proximal hypospadias who have a smaller penis. Numerous articles report testosterone injections or topical creams increase the length and circumference of the penis. However, few studies discuss the impact of this treatment on the success of corrective surgery, with conflicting results.[29][30]
Surgery
Surgery is not always required for minor hypospadias like glanular hypospadias and coronal hypospadias if the meatus is of good caliber, urine flow is in good stream & forwardly directed.[31]
Hypospadias repair is done under general anesthesia, most often supplemented by a nerve block to the penis or a caudal block to reduce the general anesthesia needed, and to minimize discomfort after surgery.[citation needed]
Many techniques have been used during the past 100 years to extend the urinary channel to the desired location. Today, the most common operation, known as the tubularized incised plate or "TIP" repair, rolls the urethral plate from the low meatus to the end of the glans. TIP repair, also called the Snodgrass Repair (after the creator of the method, Dr. Warren Snodgrass), is the most widely used procedure and surgical method for hypospadias repair worldwide. This procedure can be used for all distal hypospadias repairs, with complications afterwards expected in less than 10% of cases.[32][33]
Less consensus exists regarding proximal hypospadias repair.[34] TIP repair can be used when the penis is straight or has mild downward curvature, with success in 85%.[32] Alternatively, the urinary channel can be reconstructed using the foreskin, with reported success in from 55% to 75%.[35]
Most distal and many proximal hypospadias are corrected in a single operation. However, those with the most severe condition having a urinary opening in the scrotum and downward bending of the penis are often corrected in a two-stage operation. During the first operation the curvature is straightened. At the second, the urinary channel is completed. Any complications may require additional interventions for repair.[citation needed]
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Example of penis with hypospadias
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Penis with hypospadias (1) and two fistulae (2)
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Adult penis with hypospadias
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Adult penis with hypospadias
Outcomes
Problems that can arise include a small hole in the urinary channel below the meatus, called a fistula. The head of the penis, which is open at birth in children with hypospadias and is closed around the urinary channel at surgery, sometimes reopens, known as glans dehiscence. The new urinary opening can scar, resulting in meatal stenosis, or internal scarring can create a stricture, either of which cause partial blockage to urinating. If the new urinary channel balloons when urinating a child is diagnosed with a diverticulum.[citation needed]
Most complications are discovered within six months after surgery, although they occasionally are not found for many years. In general, when no problems are apparent after repair in childhood, new complications arising after puberty are uncommon. However, some problems that were not adequately repaired in childhood may become more pronounced when the penis grows at puberty, such as residual penile curvature or urine spraying due to rupture of the repair at the head of the penis.[citation needed]
Complications are usually corrected with another operation, most often delayed for at least six months after the last surgery to allow the tissues to heal sufficiently before attempting another repair. Results when circumcision or foreskin reconstruction are done are the same.[36][37] (Figure 4a, 4b)
Patients and surgeons had differing opinions as to outcomes of hypospadias repair, that is, patients might not be satisfied with a cosmetic result considered satisfactory by the surgeon, but patients with a cosmetic result considered not very satisfactory by the surgeon may themselves be satisfied. Overall, patients were less satisfied than surgeons.[17]
Living with hypospadias can present challenging emotional obstacles. Many men, whether they have had surgical repair of their hypospadias as a child or not often are very guarded in school bathrooms or locker rooms. Secrecy about the condition can complicate emotional pain, because talking about the penis is often a taboo subject. Worry, anxiety and feelings of shame are common among adult men born with hypospadias.[38]
Epidemiology
Hypospadias is among the most common birth defects in the world and is said to be the second-most common birth defect in the male reproductive system, occurring once in every 250 males.[39]
Due to variations in the reporting requirements of different national databases, data from such registries cannot be used to accurately determine either incidence of hypospadias or geographical variations in its occurrences.[3]
Adults
While most hypospadias repairs are done in childhood, occasionally, an adult desires surgery because of urinary spraying or unhappiness with the appearance.
A direct comparison of surgical results in children versus adults found they had the same outcomes, and adults can undergo hypospadias repair or reoperations with good expectations for success.[29]
Society and culture
Notable individuals with hypospadias: (sources and citation needed)
- Henry II of France[40]
- Tiger Devore[41][42]
- Gabriel J. Martín[43][44]
- Scout Schultz[45]
- Maurice Duplessis[citation needed]
- Aprilio Manganang[citation needed]
- Lil Dicky[46]
See also
- Pediatric urology
- Andrology
- Bladder exstrophy, cloacal exstrophy
- pseudovaginal perineoscrotal hypospadias
- Intersex surgery
- Androgen insensitivity syndrome
- Testicular dysgenesis syndrome
References
- ^ Entry "hypospadias" in Merriam-Webster Online Dictionary.
- OED2nd edition, 1989 as /hɪpəʊˈspeɪdɪəs/~/haɪpəʊˈspeɪdɪəs/
- ^ ISBN 978-1-4160-6911-9.
- ISBN 978-0-702-04292-8.
- PMID 19692004.
- ^ "Hypospadias - Symptoms and causes". Mayo Clinic. Retrieved 2023-12-13.
- PMID 2231648.
- PMID 3746833.
- ^ Wu H, Wei Z, M G (2002). "Hypospadias and enlarged prostatic utricle". Chinese Journal of Urology. 12: 51–3.
- PMID 9120962.
- PMID 11796295.
- ^ "Explaining Disorders of Sex Development & Intersexuality".
- ^ Tidy, Colin (January 19, 2016). "Hypospadias". Patient. Patient Platform Ltd. Retrieved October 18, 2018.
- ^ "What is intersex? Frequently Asked Questions and Intersex Definitions". interACT. Retrieved May 7, 2021.
- ^ "Alice Dreger: Do you have to pee standing up to be a real man? – Intersex Human Rights Australia". ihra.org.au. 2014-02-14. Retrieved 2022-03-22.
- ^ "IGM". OII Europe. 2021-02-04. Retrieved 2022-03-22.
- ^ PMID 15713818.
- PMID 23798865; PMCID: PMC3685753.
- PMID 16753427.
- PMID 24534572.
- ^ "Urethroplasty". Department of Urology. 2016-06-06. Retrieved 2019-06-14.
- ^ "United Nations Fact Sheet-Intersex" (PDF).
- ISBN 978-1-61002-579-9.
- ^ "Re operation with Dr Nicol Bush & Dr Warren Snodgrass". PARC Urology Hypospadias Center. 2016-07-15. Retrieved 2019-05-09.
- ISBN 978-1-4160-4572-4.
- PMID 23878686.
- ^ "What are some of the risks of penile surgery?". ISSM. 2012-02-21. Retrieved 2019-06-14.
- ISBN 978-1-4614-6910-0.
- ^ PMID 24999242.
- S2CID 22512786.
- ^ "Hypospadias". Retrieved 2023-03-21.
- ^ PMID 19837000.
- PMID 21159560.
- PMID 20727541.
- ISBN 978-1-4614-6910-0.
- PMID 18790424.
- PMID 22854388.
- ISBN 978-1638372172.
- ^ Gatti JM. "Epidemiology". Medscape Reference. Retrieved 22 January 2013.
- ^ "The "infertility" of Catherine de Medici and its influence on 16th century France" (PDF).
- British Broadcasting Corporation. 11 October 2011. Retrieved 2016-05-12.
- Rewire.News. Retrieved 2018-11-30.
- ^ Intersexualidad: «Nunca me sentí niña y mi comportamiento masculino era un problema». ABC. 20 October 2014.
- ^ La barba me dio la razón: aunque me criaran como una niña, yo era un niño. El País. 30 June 2016.
- ^ Lieberman, Hallie (August 29, 2018). "The Trigger Effect". The Atavist Magazine. No. 82. Archived from the original on September 1, 2018. Retrieved 2018-08-31.
- ^ Heifetz, Danny, When a Dick Joke Isn't a Joke, theringer.com, March 2020
Further reading
- Austin PF, Siow Y, Fallat ME, Cain MP, Rink RC, Casale AJ (October 2002). "The relationship between müllerian inhibiting substance and androgens in boys with hypospadias". The Journal of Urology. 168 (4 Pt 2): 1784–8, discussion 1788. PMID 12352359.
- Patel RP, Shukla AR, Snyder HM (October 2004). "The island tube and island onlay hypospadias repairs offer excellent long-term outcomes: a 14-year followup". The Journal of Urology. 172 (4 Pt 2): 1717–9, discussion 1719. PMID 15371798.
- Retik AB, Atala A (May 2002). "Complications of hypospadias repair". The Urologic Clinics of North America. 29 (2): 329–39. PMID 12371224.
- Shukla AR, Patel RP, Canning DA (August 2004). "Hypospadias". The Urologic Clinics of North America. 31 (3): 445–60, viii. PMID 15313054.
- International Trends in Rates of Hypospadias and Cryptorchidism
- "Special issue on hypospadias". Indian Journal of Urology. 24 (2). 2008.