SCROTAL AND TESTICULAR DISEASES. 7 Hydrocele 7 Epididymal cyst 9 Varicocele 9 Cryptorchidism 11 Testicular tumors 13

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1 Table of Contents Subject Page Scrotal pain Testicular torsion Torsion of the appendages Epididymitis Fournier gangrene 7 Hydrocele 7 Epididymal cyst 9 Varicocele 9 Cryptorchidism 11 Testicular tumors P a g e 1

2 Scrotal Pain Scrotal pain is a condition with a lot of differential diagnoses; Torsion of the testes Torsion of appendages Acute epididymitis. Bleeding or infection in a testicular tumor "tumors per se are usually painless" Fournier gangrene. Trauma Strangulated hernia. Put in your mind that scrotal pain could be a referred pain from the abdomen, for example, a case of appendicitis with long appendix that extends into the pelvis, so you should always examine the abdomen in a patient with scrotal pain. A. Testicular Torsion Coverings of the testis from inside to outside; tunica vaginalis (visceral and parietal), internal spermatic fascia, cremaster muscle (middle spermatic fascia), external spermatic fascia, Dartos muscle, skin. Testicular torsion occurs due to twisting of the spermatic cord and blood supply to the testicles, which causes a sudden severe pain and the scrotum will suddenly increase in size. It is a urological emergency; early diagnosis and treatment are vital to saving the testicles and preserving future fertility. 2/3 of cases are adolescents (peak age at 14), another commonly affected age group is neonates. 1:160 is the lifetime incidence. P a g e 2

3 It has three types; Intravaginal torsion; torsion of the spermatic cord inside the tunica vaginalis, the tunica vaginalis itself is not twisted. It is the commonest overall. Normally, with mature attachments, the tunica vaginalis is attached securely to the posterior lateral aspect of the testicle, and, within it, the spermatic cord is not very mobile. If the attachment of the tunica vaginalis to the testicle is inappropriately high, the spermatic cord can rotate within it, which can lead to intravaginal torsion. This defect is referred to as the bell clapper deformity. Intravaginal torsion most commonly occurs in adolescents. It is thought that the increased weight of the testicle after puberty, as well as sudden contraction of the cremasteric muscles, is the impetus for acute torsion. Extravaginal torsion; the spermatic cord and tunica vaginalis undergo torsion as a unit. Neonates more often have extravaginal torsion. This occurs because the tunica vaginalis is not yet secured to the gubernaculum and, therefore, the spermatic cord, as well as the tunica vaginalis, undergo torsion as a unit. Extravaginal torsion is not associated with bell clapper deformity. This can occur up to months prior to birth and, therefore, is managed differently depending on presentation. In this type of torsion, most of the time there's complete infarction at the time of exploration, This happens because the baby will not tell you he's having severe pain in his testicle, but what happens is that the mother brings him complaining of loud continuous crying. P a g e 3

4 The third type of the torsion is called Isolated Torsion. This happens when the mesoepididymis that connects the testicle to the epididymis is long which causes the testicle to go around itself. This type of course is rare because the prevalence of long mesoepididymis is very low. History - Left : right = 2:1 - Sudden onset of severe pain followed by inguinal and/or scrotal swelling, some cases may suffer of a gradual onset of pain, and a small number of cases will have abdominal pain only. - Could be associated with nausea, anorexia, vomiting and low grade feve, especially in pediatric age group. - Torsion can occur with trauma in about 20% of cases, masturbation is one form of trauma. Physical examination - Tender and swollen testis. - Elevated testis; as the cord is twisted, it becomes shorter. - Horizontal lie. - Redness of the overlying skin. - Loss of cremasteric reflex. Cremaster reflex; scratching the upper medial side of the thigh will stimulate the femoral branch of the genitofemoral nerve and ilioinguinal nerve. These synapse in the spinal cord and activates the motor fibers of the genitofemaoral nerve leading to contraction of the cremaster muscle that pulls the epsilateral testis. Management After developing the diagnosis we should send him the Operation Room directly. We don't use ultrasound to detect testicular torsion; - May cause delay, so the possibility to save the testis will be less - More inaccurate in smaller child. - Can give false negative results. - Report will never rule out torsion. If you explore the testis within the first within the first 6 hours, the survival rate will be 85-97%, but after 24 hours, the survival rate will be less than 10%. Note; the time to save the testis varies according to the degree of twisting (in general it is 6 hours), the more the twisting the less time to save the testis. do a small excision, put the testicle in its place and fix the torsion, fixate the testicle very well so we prevent other torsions, and finally we should fixate the other testicle because we will be afraid of torsion in the future to the other testicle too P a g e 4

5 When the testicle gets affected by the torsion, the venous drainage will be the first vascular component to be affected. Following it by few minutes will be the arterial supply, and finally in few hours a complete infarction will occur, and the testicle may become gangrenous if not treated. That's why surgical intervention in cases of testicular torsion should be very fast to prevent any complication. As you know from your previous surgical knowledge, any cut in blood supply to an organ will take only 6 hours before a complete infarction happens and death of the organ starts. For this reason, we shouldn't allow any case of torsion to be left without urgent intervention to correct it. We should remember that the pain is because of venous and then arterial obstruction. This will cause sudden severe pain after movement. That's why you will always face cases where a child was playing with his friends and suddenly he stops playing and feels severe pain in his scrotum. On examining the testicle, you will notice that it is high and tender, with horizontal lie. B. Torsion of appendages The appendages are embryological remnants that are associated with the testicle, epididymis, and vas deferens. The appendages are The superior and inferior aberrans of haller "originate from the body and tail of epididymis". Appendix of the epididymis "from the head of epididymis". Appendix testis "hydatid of morgagni". If one of these appendages become twisted, this will cut off the blood supply and cause ischemia, prolonged torsion will result in the death of the appendix. The peak incidence is below 2-3 years which is below the ages of testicular torsion, and it's important to know that these appendages has no function because they are remnants. 95% of the cases occur in the Hydatid of Morgagni: the appendix of the testis, remnant of mullerian duct, present in the upper pole of the testis and attached to the processus vaginalis. When doing a physical examination we can see (blue dot sign) with the swelling which is palpable dots with blue or black in color. -pain is confined to the upper pole (origin of the appendages) and with time it become indistinguishable from testicular torsion because of scrotal enlargement and the inflammation that spread over the testis P a g e 5

6 C. Torsions Vs epididymitis : - It is frequently difficult to distinguish these two conditions; Because of the edema and pain associated with both acute epididymitis and testicular torsion. Epididymitis usually: - Pain is Gradual because it s inflammation (infection). - Sharp pain (not dull aching). - Epididymitis is might be preceded by UTI (in a lot of cases) or urethral discharge. - Can be associated with fever and turbid urine. Torsion is a vascular pain so: - It s a dull aching (can t be described well). - It s not relived by changing the position. - Spontaneous, can be preceeded by trauma, or physical activity. - Any testicular pain below 20 y age is torsion until proven otherwise. - The patient usually presents with the sudden onset of pain and swelling of the involved testis. The pain may radiate into the groin and lower abdomen. Most appropriate cause Torsion Adolescent who is not sexually active Epididymitis STD (sexual transmitted disease) Onset Sudden Gradual Duration Short Few days Severity More severe Less severe Pattern Constant Intermittent Increasing or decreasing factors Any movement may aggravate the pain Cremaster reflex Loss of cremaster reflex Present Prehn's sign* negative Positive *Prehn's sign: is a medical diagnostic scale which determine if the injured testicles is because of epididymitis or testicular torsion. According to it the physical lifting of the testicles relieves the pain of epididymitis not the pain of testicular torsion. P a g e 6

7 D. Fournier Gangrene A type of necrotizing infection or gangrene that affects the perineum. It is a mixed infection that involves both aerobic and anaerobic bacteria. Most of the patients are in their 60s or 70s and about half of them are diabetic. The hallmark of the disease is intense pain and tenderness in the genitalia, usually associated with edema of the overlying skin, pruritis may also be present. It progresses till it becomes an obvious gangrene of a portion of genitalia and purulent drainage from wounds. Hydrocele Hydroceles are collection of fluids inside the scrotum that happens for many reasons, and can be classified into: Hydrocele due to patent processus vaginalis, which includes; Primary Vaginal hydrocele, infantile hydrocele, congenital hydrocele, encysted hydrocele of the cord. Processus vaginalis is a pouch of peritoneum that is carried into the scrotum by the descent of the testicle and which in the scrotum forms the tunica vaginalis. In children, patency of the processus vaginalis is the main and commonest cause. In this case the processus, which normally closes and becomes the tunica vaginalis, will fail to close after delivery and it will stay patent, causing fluids from the peritoneum to pass to the scrotum and fill it. P a g e 7

8 Secondary hydrocele to other nearby lesions like inflammations, tumors, traumas and torsion. In adults, the problem lies in the lymphatic drainage of the testicles, where there will be obstruction in the drainage that causes fluids to stay in the scrotum causing the enlargement. Communicating and non-communicating types We can differentiate between the 2 types clinically by asking the patient to lie on his back, and if the hydrocele decreases then it's communicating because the fluids in this case would go to the peritoneal cavity, while in case of non-communicating hydrocele the fluids will stay in the scrotum. Symptoms We should know that the hydrocele is painless and rarely complicated, but we are usually asked to remove it for cosmetic reasons, and because of its heaviness that will make the patient feel uncomfortable. Hydrocele is managed by surgical excision for it to remove the fluids and fix the problem. We do not aspirate the fluids because it will come back, and sometime aspiration will cause infection in the testicle (orchitis) which will end with infertility. P a g e 8

9 in the figure to the left, you can see what happen when using light to transilluminate the hydrocele. in the figure to the right, you can see a case of hydrocele caused by acute epididymitis that blocked the lymphatic drainage of the testicles and caused the accumulation of the fluids. Epididymal Cysts: It occurs at the head of the epididymis, and it usually contains clear fluid. However, if it contains turbid fluid and sperms, then we call it spermatocele. It transilluminate when exposed to light, contrary to the spermatocele which doesn't transilluminate. When we have a young patient with epididymal cyst, we don't advise him to remove it because in surgical excision we might face fibrosis and thus obstruction of the duct, causing infertility. So we delay the operation till the end of fertility period, because this condition is painless and rarely develops any complications. When should we take an action? If the cyst is large and interferes with the patient's life. In this case, we do complete excision not aspiration, because aspirating it puts the patient in risk of recurrence and developing infections. Varicocele: Varicocele is abnormal tortuosity and dilatation of the testicular veins within the spermatic cord due to incompetency of the valves of the venous drainage of the testicles. It is present in about 15-20% of normal fertile male population and 40% of infertile men. Although the majority of men with varicocele are fertile, it is considered as the most common correctable cause of male infertility. This happens because of increased temperature in the scrotal sac due to increased pressure and amount of blood around the testes. The most common effect of it is Asthenospermia, which is decreased motility of the sperms. Decreased sperm density and presence of abnormal forms will follow, and later on the patient may have Azoospermia. P a g e 9

10 90% in the left side, only less than 2 percent is bilateral. It is more common in the left side because the left gonadal vein drains to the renal vein, while the right one drains directly to the inferior vena cava, and so the more perpendicular lie of the left vein makes it more susceptible to be varicose. Because of this, any case of right varicocele should be well investigated for any other causes like renal or retroperitoneal tumors. Symptoms; Dragging like or aching pain in the scrotum Feeling of heaviness in the testicles. Infertility. Atrophy (shrinking) of the testicles. visible or palpable veins described as (bag of worms) Investigations; Renal ultrasound (+/-) CT if suspecting obstructive causes. When to suspect obstructive causes if the varicocele is; Acute onset Doesn't disappear on supine position Palpable abdominal mass Solitary right varicocele. History of RPF. Clinical suspicion of renal tumor (hematuria) Varicoceles grading; 1st grade is when it's small and palpable only on Valsalva maneuver when we ask the patient to cough. 2nd grade is when it's palpable on standing position without coughing 3rd grade you will see it also in standing position. So you should always remember that varicoceles, just like hernias, should be examined in standing position. Treatment Scrotal support Varicocele embolization Varicelectomy P a g e 11

11 Cryptorchidism It is the absence of one or both testis from the scrotum. It is a common condition affecting about 3% of full-term babies, and about 30% of premature infants, with a family history seen in about 10% of cases. A 7% percent incidence rate seen in siblings of boys with undescended testis. Predisposing factors; Prematurity Low birth weight Small size for gestational age Maternal exposure to estrogen during the first trimester Twinning 70% of cases spontaneously descend after 3 months, spontaneous descend after the first year is uncommon. Physical examination It should be performed in a worm room. The patient should be relaxed in a frog-like position. Start the examination by observation, the milk down "palpating from the iliac crest to the scrotum". Examine the scrotum "hypoplastic, bifid, rugae, transposition and pigmentation" Check the penis for any other abnormality "chordee, hypospadiad, and length of stretched penis" Classification Testis in general are either palpable or non-palpable; 1. Palpable testis a. Normal, when the patient is supine (or standing), the testes should be both visible and palpable at the base of scrotum, no manipulation needed to put it there. The ipsilateral hemiscrotum equally well-developed in comparison to the contralateral side. b. Retractile, when the patient is supine (or standing), the testis is not in its normal position at the base of scrotum, but can be located in the inguinal area and normally moved to the base of scrotum where it remains for some minutes without traction. It descends in response to hormonal manipulation (hcg, GnRH) The ipsilateral hemiscrotum is equally developed in comparison to the contralateral side. P a g e 11

12 c. Ectopic testes, these ones have left the path of normal descent, they reside in the superficial inguinal pouch, the perineum, the femoral canal, penopubic area or transversely ectopic. The ipsilateral hemiscrotum is usually smaller than the contralateral side. d. Undescended, located somewhere along the normal path of descent, the ipsilateral hemiscrotum is small 2. Non-palpable a. Canalicular, detected by ultrasound b. Intra-abdominal c. Emergent testis, which lies in the inguinal canal and may sometimes project beyond the superficial inguinal ring but again slips back into the inguinal canal. d. Absent, agenesis "never formed embryologically" or atrophic "which is either unilateral - monorchia- or bilateral anorchia-". We use hormonal test to distinguish bilateral anorchia from bilateral undescended. Complications Using laparoscopy to detect nonpalpable testis carries 100% specificity. On the other hand, MRI and CT scan don't distinguish between the undescended testis and a lymph node, so that the specificity in this case doesn't exceed 80%. One of the most important complications of cryptorchidism is neoplasia. Intra-abdominal testis is more likely to undergo malignancy than inguinal (4times). Orchidopexy (surgery for the testes to put them in their appropriate place) doesn t decrease the risk of malignancy (we only do it for better surveillance of the testes) Hernia (patent processus vaginalis) in 90% of cases Infertility (the process of impaired spermatogensis start for day on in life and become irreversibleat 18 years old) Torsion Associated malformation; Non-urological Cerebral lesion, myelomeningocele, mental retardation (prader-willi syndrome) Urological: epididymal (36-43%) hypospadias up to 9% P a g e 12

13 Management 1. Surgical, common and better 2. Hormonal, no longer used. Note: Very important that the doctor said that we should put the testis in its places at the ages of one year to 1.5 year (possible MCQ). Purpose of treatment Cosmotic Fertility. It doesn't affect the risk of tumor development. Testicular Tumors: They compromise 2% of all male tumors. Its etiology in general is unknown, but we know that patients with cryptorchidism have much higher risk (up to 14 times), patients with intersex problems have higher risk too, and Africans have much lower risk [And of course being a male is the most important risk factor :P ]. Most of the testicular tumors (90-95%) arise from germinal elements, and the rest from non-germinal elements. Neonates will have Embryonal Cell Carcinoma or Yolk Sac Tumor. In young adults we will see teratoma and seminoma, with the former peaking at the age of 27 and the latter at 37. In old ages, after the age of 50 years, we will see lymphomas and seminomas. So knowing the age of the patient is very important to consider the diagnosis. Seminoma is the most common type, crypto-orchidism increases the risk of seminoma up to 50 times. Types of Testicular Tumors: We classify the testicular tumors according to the organ of origin into: Primary, Secondary, or Paratesticular tumors. Primary tumors can be either of a germinal origin (like seminoma, teratoma, combined, or yolk sac tumor), or of a non-germinal origin from other cells in the testes (like interstitial cells tumors or lymphoma). Secondary tumors can come from one of 2 origins: either of a Reticuloendothelial origin, or is a metastasis from another tumor, most commonly RCC. Paratesticular tumors are anything that doesn't involve the testicle in it, but may affect it in other ways. P a g e 13

14 2-3% of testicular tumors are bilateral, and in about 5% of the cases we will have testicular tumor in one side and CIS in the other testes. Mixed tumors of seminoma and teratoma will be seen in about 14% of the cases. Staging When classifying tumors into stages, we will be depending on Royal Marsden System where we have 4 grades of tumors: 1. First stage: There is no metastasis outside the testes to any other organ. stage 1M: As above but with persistently raised AFP & /or b-hcg after orchiectomy 2. Second stage: Metastasis is limited to infra-diaphragmatic and abdominal lymph nodes. IIA: Max. diameter < 2 cm IIB: 2-5 cm IIC: 5 cm 3. Third stage: Metastasis will go also to supra-diaphragmatic lymph nodes (like supraclavicular and thoracic LNs). 4. Fourth stage: Here the metastasis will go other organs other than LNs, like Liver. Tumor Markers Tumor markers are very important for us to help in the diagnosis: 1. Oncofetal substances: AFP & B-hCG AFP is produced by trophoblastic cells, has a half-life of 5-7 hours and has the following features: Increase in cancers of testis, liver, pancreas, lung, pregnancy, benign liver diseases, ataxia & tyrosinemia. Produced by pure embryonal CA, terato CA, Yolk sac tumor, or combined tumors. Indicate presence of a teratomatous element. Not produced by pure choriocarcinoma, or pure seminoma. B-hCG is a glycoprotein with half-life of hours, and has the following features also: Increase in malignancies of liver, pancreas, stomach, lung, breast, kidney, and bladder & marijuana smoker. Increase in all types with choriocarcinoma % of patients with embryonal ca. 5-10% of patients with pure seminoma. 2. Cellular enzymes: LDH, PLAP LDH marks the presence of bulky tissue, PLAP which indicates advanced diseases, and GGT which might be present in seminomas. P a g e 14

15 Clinical Manifestations: % of patients present with painless testicular mass. - Some may present with heavy sensation in the lower abdomen, anal area, or scrotum. - Pain can be due to Epdidymitis or bleeding in the tumor. - 10% of patients come with manifestations of metastasis. - Gynecomastia: as an effect of B-hCG, Androgens & Estrogens. Differential Diagnosis: Epididymo-orchitis Torsion Hydrocele Syphilitic gumma TB Granulomatous orchitis - Spread from the tumor can be either direct, vascular, or through lymphatic drainage. Investigations -CBC, U&E, LFT. -Tumor Markers. -Imaging: - CXR: to exclude metastasis - Ultrasound for the scrotum - CT-scan: Thorax & Abdomen to look for LNs. -Surgical exploration: Trans-inguinal not trans-scortal to prevent spreading tumor cells to the scrotum -No FNA or Tru-cut Biopsy should be done, although some physicians say that performing FNA is not dangerous. Treatment: It's agreed that the basic treatment in cases of testicular tumors is Radical Orchiectomy. After performing the surgery, our next step should be clinically grading the tumor by doing histopathological studies and imaging investigations. It was found that with seminomas, it's usually confined to the testes without any metastasis to other tissues. In non-seminoma germinal cell tumors (NSGCTs), however, recognizable distant metastasis is usually seen in about 60-70% of cases. P a g e 15

16 When classifying the tumors into seminomas and NSGCTs, it was found that seminomas will have much better prognosis. This is due to the fact that they are highly radiosensitive, in addition to the previously mentioned fact of low rate of metastasis. When seminomas metastasize, they mostly go to the liver and lung. In such cases of seminomas, we have to perform inguinal orchiectomy as soon as possible, and then depending on the stage we will continue the treatment. Inguinal approach to the tumor is needed because if we go through the scrotum, we are putting the patient in risk of spreading the tumor cells all over the scrotum. In case staging gave us good prognosis of stage 1 or early stage 2, then abdominal radiotherapy will be the next step. However, if we found out that the grade of the tumor was late grade 2, grade 3, or grade 4, then we should start chemotherapy, with BEP (Bleomycine, Etoposide, and Cis-Platinum) being the drug combination of choice. What should be our aim in treatment? Our main aim of course should be total cure of the patient. We should make sure that we finish the treatment of the patient only when total recovery. Another aim for us should be maintenance of fertility in the patient, especially if he was young. And the third aim is to prevent any future complication in the patient. In cases of NSGCTs, the treatment should be extensive after performing the orchiectomy. Chemotherapy of BEP for 4-6 courses is needed directly after the surgery. Retroperitoneal Lymph Node Dissection (RPLND) is needed also to prevent any metastasis that have reached them and may cause complications. Use of radiotherapy is limited in NSGCTs because the tumor is not sensitive to it. Follow-Up: After finishing treatment and making sure the body of the patient is clear from any metastasized tissue, we should follow him up for 2 years. In the first year after recovery, we should perform a CXR every month to make sure the chest of the patient is clear. In the second year the CXR will be taken every 2 months, and then annually after that. Another follow-up technique should be Abdominal and Chest CT scans perform to look for any metastasis to the LNs or organs. P a g e 16