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Because polyorchidism is very uncommon, there is no standard treatment for the condition. Prior to advances in ultrasound technology, it was common practice to remove the supernumerary testicle. Several cases have been described where routine follow-up examinations conducted over a period of years showed that the supernumerary testicle was stable.
A meta-analysis in 2009 suggested removing non-scrotal supernumerary testicles because of the increased risk of cancer, and regular follow-up in the remaining cases to ensure that the supernumerary testicle remains stable.
The primary management of cryptorchidism is watchful waiting, due to the high likelihood of self-resolution. Where this fails, a surgery, called orchiopexy, is effective if inguinal testes have not descended after 4–6 months. Surgery is often performed by a pediatric urologist or pediatric surgeon, but in many communities still by a general urologist or surgeon.
When the undescended testis is in the inguinal canal, hormonal therapy is sometimes attempted and very occasionally successful. The most commonly used hormone therapy is human chorionic gonadotropin (HCG). A series of hCG injections (10 injections over 5 weeks is common) is given and the status of the testis/testes is reassessed at the end. Although many trials have been published, the reported success rates range widely, from roughly 5 to 50%, probably reflecting the varying criteria for distinguishing retractile testes from low inguinal testes. Hormone treatment does have the occasional incidental benefits of allowing confirmation of Leydig cell responsiveness (proven by a rise of the testosterone by the end of the injections) or inducing additional growth of a small penis (via the testosterone rise). Some surgeons have reported facilitation of surgery, perhaps by enhancing the size, vascularity, or healing of the tissue. A newer hormonal intervention used in Europe is the use of GnRH analogs such as nafarelin or buserelin; the success rates and putative mechanism of action are similar to hCG, but some surgeons have combined the two treatments and reported higher descent rates. Limited evidence suggests that germ cell count is slightly better after hormone treatment; whether this translates into better sperm counts and fertility rates at maturity has not been established. The cost of either type of hormone treatment is less than that of surgery and the chance of complications at appropriate doses is minimal. Nevertheless, despite the potential advantages of a trial of hormonal therapy, many surgeons do not consider the success rates high enough to be worth the trouble since the surgery itself is usually simple and uncomplicated.
In cases where the testes are identified preoperatively in the inguinal canal, orchiopexy is often performed as an outpatient and has a very low complication rate. An incision is made over the inguinal canal. The testis with accompanying cord structure and blood supply is exposed, partially separated from the surrounding tissues ("mobilized"), and brought into the scrotum. It is sutured to the scrotal tissue or enclosed in a "subdartos pouch." The associated passage back into the inguinal canal, an inguinal hernia, is closed to prevent re-ascent.
In patients with intraabdominal maldescended testis, laparoscopy is useful to see for oneself the pelvic structures, position of the testis and decide upon surgery ( single or staged procedure ).
Surgery becomes more complicated if the blood supply is not ample and elastic enough to be stretched into the scrotum. In these cases, the supply may be divided, some vessels sacrificed with expectation of adequate collateral circulation. In the worst case, the testis must be "auto-transplanted" into the scrotum, with all connecting blood vessels cut and reconnected ("anastomosed").
When the testis is in the abdomen, the first stage of surgery is exploration to locate it, assess its viability, and determine the safest way to maintain or establish the blood supply. Multi-stage surgeries, or autotransplantation and anastomosis, are more often necessary in these situations. Just as often, intra-abdominal exploration discovers that the testis is non-existent ("vanished"), or dysplastic and not salvageable.
The principal major complication of all types of orchiopexy is a loss of the blood supply to the testis, resulting in loss of the testis due to ischemic atrophy or fibrosis.
With prompt diagnosis and treatment the testicle can often be saved. Typically, when a torsion takes place, the surface of the testicle has rotated towards the midline of the body. Non-surgical correction can sometimes be accomplished by manually rotating the testicle in the opposite direction (i.e., outward, towards the thigh); if this is initially unsuccessful, a forced manual rotation in the other direction may correct the problem. The success rate of manual detorsion is not known with confidence.
Testicular torsion is a surgical emergency that requires immediate intervention to restore the flow of blood. If treated either manually or surgically within six hours, there is a high chance (approx. 90%) of saving the testicle. At 12 hours the rate decreases to 50%; at 24 hours it drops to 10%, and after 24 hours the ability to save the testicle approaches 0. About 40% of cases result in loss of the testicle. Common treatment for children is surgically sewing the testicle to the scrotum to prevent future recurrence (orchiopexy).
Small cysts are best left alone, as are larger cysts that are an asymptomatic condition. Only when the cysts are causing discomfort and are enlarging in size, or the patient wants the spermatocele removed, should a spermatocelectomy be considered. Pain may persist even after removal.
Spermatocelectomy can be performed on an outpatient basis, with the use of local or general anesthesia.
A spermatocelectomy will not improve fertility.
The fluid accumulation can be drained by aspiration, but this may be only temporary. A more permanent alternative is a surgical procedure, generally, an outpatient ambulatory (same-day) procedure, called a hydrocelectomy. There are two surgical techniques available for hydrocelectomy.
- Hydrocelectomy with Excision of the Hydrocele Sac: Incision of the hydrocele sac after complete mobilization of the hydrocele. Partial resection of the hydrocele sac, leaving a margin of 1–2 cm. Care is taken not to injure testicular vessels, epididymis or ductus deferens. The edge of the hydrocele sac is oversewn for hemostasis (von Bergmann's technique) or the edges are sewn together behind the spermatic cord (Winkelmann's or Jaboulay's technique). Hydrocele surgery with excision of the hydrocele sac is useful for large or thick-walled hydroceles and multilocular hydroceles.
- Hydrocele Surgery with Plication of the Hydrocele Sac: The hydrocele is opened with a small skin incision without further preparation. The hydrocele sac is reduced (plicated) by suture Hydrocele surgery: Lord's technique. The plication technique is suitable for medium-sized and thin-walled hydroceles. The advantage of the plication technique is the minimized dissection with a reduced complication rate.
If the hydrocele is not surgically removed, it may continue to grow. The hydrocele fluid can be aspirated. This procedure can be done in a urologist's office or clinic and is less invasive but, recurrence rates are high. Sclerotherapy, the injection of a solution following aspiration of the hydrocele fluid may increase success rates. In many patients, the procedure of aspiration and sclerotherapy is repeated as the hydrocele recurs.
Patients with Leydig cell hypoplasia may be treated with hormone replacement therapy (i.e., with androgens), which will result in normal sexual development and the resolution of most symptoms. In the case of 46,XY (genetically "male") individuals who are phenotypically female and/or identify as the female gender, estrogens should be given instead. Surgical correction of the genitals in 46,XY males may be required, and, if necessary, an orchidopexy (relocation of the undescended testes to the scrotum) may be performed as well.
The two most common surgical approaches are retroperitoneal (abdominal using laparoscopic surgery), infrainguinal/subinguinal (below the groin) and inguinal (groin using percutaneous embolization). Possible complications of this procedure include hematoma (bleeding into tissues), hydrocele (accumulation of fluid around the affected testicle), infection, or injury to the scrotal tissue or structures. In addition, injury to the artery that supplies the testicle may occur, resulting in a loss of a testicle.
Whether having variocele surgery or embolization improves male fertility is controversial, as good clinical data are lacking. There is tentative evidence that varicoceletomy may improve fertility in those with obvious findings and abnormal sperm; however, this has a number needed to treat of 7 for varicoceletomy and 17 for embolization. There are also studies showing that the regular surgery has no significant effect on infertility. A 2012 Cochrane review found tentative but unclear evidence of improved fertility among males treated for varicocele.
Ultrasound is useful if the cause is not certain based on the above measures. If the diagnosis of torsion is certain, imaging should not delay definitive management such as physical maneuvers and surgery.
Surgery is sometimes performed to alter the appearance of the genitals. However many surgeries performed on intersex people lack clear evidence of necessity, can be considered as mutilating, and are widely considered to be human rights violations when performed without the informed consent of the recipient.
To some extent, it is possible to change testicular size. Short of direct injury or subjecting them to adverse conditions, e.g., higher temperature than they are normally accustomed to, they can be shrunk by competing against their intrinsic hormonal function through the use of externally administered steroidal hormones. Steroids taken for muscle enhancement (especially anabolic steroids) often have the undesired side effect of testicular shrinkage.
Similarly, stimulation of testicular functions via gonadotropic-like hormones may enlarge their size. Testes may shrink or atrophy during hormone replacement therapy or through chemical castration.
In all cases, the loss in testes volume corresponds with a loss of spermatogenesis.
Most cases of polyorchidism are asymptomatic, and are discovered incidentally, in the course of treating another condition. In the majority of cases, the supernumerary testicle is found in the scrotum.
However, polyorchidism can occur in conjunction with cryptorchidism, where the supernumerary testicle is undescended or found elsewhere in the body. These cases are associated with a significant increase in the incidence of testicular cancer: 0.004% for the general population vs 5.7% for a supernumerary testicle not found in the scrotum.
Polyorchidism can also occur in conjunction with infertility, inguinal hernia, testicular torsion, epididymitis, hydrocele testis and varicocele. However, it is not clear whether polyorchidism causes or aggravates these conditions, or whether the existence of these conditions leads sufferers to seek medical attention and thus become diagnosed with a previously undetected supernumerary testicle.
Complications are often diagnosed post-operatively, which can be differentiated through duplex ultrasound scanning and are bit observed until 24 to 48 hours for early complications such as drainage, infection, formation of haematocele, rupture, etc., but also for 1 to 6 weeks during follow-up on out-patient basis.
XX females with lipoid CAH may need estrogen replacement at or after puberty. Active intervention has been used to preserve the possibility of fertility and conception in lipoid CAH females. In a case report in 2009, a woman with late onset lipoid CAH due to StAR deficiency underwent hormone replacement therapy in combination with an assisted fertility technique, intracytoplasmic sperm injection. This led to ovulation and with implantation of the in vitro fertilized egg, a successful birth.
One of the strongest arguments for early orchiopexy is reducing the risk of testicular cancer. About 1 in 500 men born with one or both testes undescended develops testicular cancer, roughly a 4 to 40 fold increased risk. The peak incidence occurs in the 3rd and 4th decades of life. The risk is higher for intra-abdominal testes and somewhat lower for inguinal testes, but even the "normally descended" testis of a man whose other testis was undescended has about a 20% higher cancer risk than those of other men.
The most common type of testicular cancer occurring in undescended testes is seminoma. It is usually treatable if caught early, so urologists often recommend that boys who had orchiopexy as infants be taught testicular self-examination, to recognize testicular masses and seek early medical care for them. Cancer developing in an intra-abdominal testis would be unlikely to be recognized before considerable growth and spread, and one of the advantages of orchiopexy is that a mass developing in a scrotal testis is far easier to recognize than an intra-abdominal mass.
It was originally felt that orchidopexy resulted in easier detection of testis cancer but did not lower the risk of actually developing cancer. However, recent data has resulted in a paradigm shift. The New England Journal of Medicine published in 2007 that orchidopexy performed before puberty resulted in a significantly reduced risk of testicular cancer than if done after puberty.
The risk of malignancy in the undescended testis is 4 to 10 times higher than that in the general population and is approximately 1 in 80 with a unilateral undescended testis and 1 in 40 to 1 in 50 for bilateral undescended testes. The peak age for this tumor is 15–45 yr. The most common tumor developing in an undescended testis is a seminoma (65%); in contrast, after orchiopexy, seminomas represent only 30% of testis tumors.
The testicle or testis is the male reproductive gland in all animals, including humans. It is homologous to the female ovary. The functions of the testes are to produce both sperm and androgens, primarily testosterone. Testosterone release is controlled by the anterior pituitary luteinizing hormone; whereas sperm production is controlled both by the anterior pituitary follicle-stimulating hormone and gonadal testosterone.
Although there is no cure for 13q deletion syndrome, symptoms can be managed, usually with the involvement of a neurologist, rehabilitation physician, occupational therapist, physiotherapist, psychotherapist, nutritionist, special education professional, and/or speech therapist. If the affected child's growth is particularly slow, growth hormone treatment can be used to augment growth. Plastic surgeries can repair cleft palates, and surgical repair or monitoring by a pediatric cardiologist can manage cardiac defects. Some skeletal, neurological, genitourinary, gastrointestinal, and ophthalmic abnormalities can be definitively treated with surgery. Endocrine abnormalities can often be managed medically. Special educators, speech and occupational therapists, and physiotherapists can help a child develop skills in and out of school.
Management of salt-wasting crises and mineralocorticoid treatment are as for other forms of salt-wasting congenital adrenal hyperplasias: saline and fludrocortisone.
Glucocorticoids can be provided at minimal replacement doses because there is no need for suppression of excessive adrenal androgens or mineralocorticoids. As with other forms of adrenal insufficiency, extra glucocorticoid is needed for stress coverage.
This can be due to:
- One testicle not descending into the scrotum during normal embryonic or fetal development (3–4% of 'normal' live births), also known as undescended testis or cryptorchidism. In this case the testis is within the abdominal cavity, somewhere along the normal route of descent – most commonly, within the inguinal canal. Such a testis has an increased risk of malignancy.
- One testicle may disappear during development (the so-called vanishing testis) due to some intrauterine insult. This is thought to be most likely vascular, such as testicular torsion.
- One testicle may have been surgically removed through orchiectomy.
- One testicle may be injured.
Treatment is often with NSAIDs and antibiotics however, this is not always effective.
An individual having monorchism can be referred to as "monorchid".
Surgical correction of inguinal hernias is called a hernia repair. It is not recommended in minimally symptomatic hernias, for which watchful waiting is advised, due to the risk of post herniorraphy pain syndrome. Surgery is commonly performed as outpatient surgery. There are various surgical strategies which may be considered in the planning of inguinal hernia repair. These include the consideration of mesh use (e.g. synthetic or biologic), open repair, use of laparoscopy, type of anesthesia (general or local), appropriateness of bilateral repair, etc. Laparoscopy is most commonly used for non-emergency cases, however, a minimally invasive open repair may have a lower incidence of post-operative nausea and mesh associated pain. During surgery conducted under local anaesthesia, the patient will be asked to cough and strain during the procedure to help in demonstrating that the repair is without tension and sound.
Constipation after hernia repair results in strain to evacuate the bowel causing pain, and fear that the sutures may rupture. Opioid analgesia makes constipation worse. Promoting an easy bowel motion is important post-operatively.
Surgical correction is always recommended for inguinal hernias in child.
Emergency surgery for incarceration and strangulation carry much higher risk than planned, "elective" procedures. However, the risk of incarceration is low, evaluated at 0.2% per year. On the other hand, surgery has a risk of inguinodynia (10-12%), and this is why males with minimal symptoms are advised to watchful waiting. However, if they experience discomfort while doing physical activities or they routinely avoid them by the fear of pain, they should seek surgical evaluation. For female patients, surgery is recommended even for asymptomatic patients.
These hernias should be repaired because of the high risk of strangulation; fortunately, surgery is straightforward, with only larger defects requiring a mesh prosthesis. Varied Spigelian hernia mesh repair techniques have been described, although evidence suggests laparoscopy results in less morbidity and shorter hospitalization compared with open procedures. Mesh-free laparoscopic suture repair is feasible and safe. This novel uncomplicated approach to small Spigelian hernias combines the benefits of laparoscopic localization, reduction, and closure without the morbidity and cost associated with foreign material.
Most hydroceles appearing in the first year of life seldom require treatment as they resolve without treatment. Hydroceles that persist after the first year or occur later in life require treatment through open operation for removing surgically, as these may have little tendency towards regression. Method of choice is open operation under general or spinal anesthesia, which is sufficient in adults. General anesthesia is the choice in children. Local infiltration anesthesia is not satisfactory because it cannot abolish abdominal pain due to traction on the spermatic cord. If a testicular tumor is suspected, a hydrocele must not be aspirated as malignant cells can be disseminated via the scrotal skin to its lymphatic field. This is excluded clinically by ultrasonography. If a tumor is not present, the hydrocele fluid can be aspirated with a needle and syringe. Clear straw-colored fluid contains mostly albumin and fibrinogen. If the fluid is allowed to drain in a collecting vessel, it does not clot but can be coagulated if small amounts of blood come in contact with the damaged tissue. In long standing cases, hydrocele fluid may be opalescent with cholesterol and may contain crystals of tyrosine and a palpable normal testis confirms the diagnosis; other wise surgical exploration of testis is needed.
The scrotum should be supported post-operatively and ice bags should be placed to soothe pain. Regular changes of surgical dressings, observation of drainage, and looking for other complications may be necessary to prevent re-operation. In cases with presence of one or more complications, open operation with/without Orchidectomy is preferred depending on the complications.
Jaboulay’s procedure
After aspiration of a primary hydrocoele, fluid reaccumulates over the following months and periodic aspiration or operation is needed. For younger patients, operation is usually preferred, whereas the elderly or unfit can have aspirations repeated whenever the hydrocoele becomes uncomfortably large. Sclerotherapy is an alternative; after aspiration, 6% aqueous phenol (10-20 ml) together with 1% lidocaine for analgesia can be injected and this often inhibits reaccumulation. Several treatments may be necessary. Aspiration of the hydrocele contents and injection with sclerosing agents sometimes with Tetracyclines is effective but it can be very painful. These alternative treatments are generally regarded as unsatisfactory treatment because of the high incidence of recurrences and the frequent necessity for repetition of the procedure.
The three basic types of treatment are surgery, radiation therapy, and chemotherapy.
Surgery is performed by urologists; radiation therapy is administered by radiation oncologists; and chemotherapy is the work of medical oncologists. In most patients with testicular cancer, the disease is cured readily with minimal long-term morbidity. While treatment success depends on the stage, the average survival rate after five years is around 95%, and stage 1 cancers cases, if monitored properly, have essentially a 100% survival rate.
The initial treatment for testicular cancer is surgery to remove the affected testicle (orchiectomy). While it may be possible, in some cases, to remove testicular cancer tumors from a testis while leaving the testis functional, this is almost never done, as the affected testicle usually contains pre-cancerous cells spread throughout the entire testicle. Thus removing the tumor alone without additional treatment greatly increases the risk that another cancer will form in that testicle.
Since only one testis is typically required to maintain fertility, hormone production, and other male functions, the afflicted testis is almost always removed completely in a procedure called inguinal orchiectomy. (The testicle is almost never removed through the scrotum; an incision is made beneath the belt line in the inguinal area.) In the UK, the procedure is known as a radical orchidectomy.