As another article on men's health, I would like to focus on the health conditions which may arise from the dysfunction of the male genitalia. There are numerous differential diagnoses when it comes to a lump over the scrotum. However, as with differential diagnoses in other systems, and running in accordance with the maxim of 'common things come first', we need to pay attention to the more likely diagnosis given the patient's anthropometric factors. In the case of scrotal mass or lump, we focus on the age of the patient initially. We work our way through by considering relevant family history, past medical history and associated symptoms. The main focus of this article is what I like to call, the 'Red Nut Syndrome' since it is a painfully accurate (no pun intended) visual description of what one can see and, frankly, it is the most painful condition known to the male sex.
We dive into the case of Elmo*, a seven-year old boy who presents to the clinic with acute groin pain and vomiting. His parents takes him to the hospital after Elmo complained about the searing pain and that they discovered a lump over one side of the scrotum. Elmo is a taciturn boy and has not spoken a word when his parents are telling me what they know about his condition. He needs a lot of prompting and does not squeal or shout like other kids when faced with a similar issue. He winces hard as he tries with difficulty to suppress the pain arising from his groin.
Figure showing an acute scrotum in a neonate (newborn baby boy) - extracted from: https://www.consultant360.com/articles/testicular-torsion-neonate.
We call it 'acute scrotum' since it has a very early onset and presents with serious symptoms. Immediate treatment is required.
An illustration of testicular torsion (extracted from: http://sinaiem.org/wp-content/uploads/2016/12/torsion.png)
Patient Particulars: 7 Year Old Boy, in full-time education. It is Sunday so naturally his parents are with him.
Chief Complaint(s) and Physical Examination Features**:
Scrotal Pain and Lump;
Vomiting.
The scrotal pain has occurred 3 hours ago spontaneously and is persistent in nature. It is not episodic and there are no associated past episodes. It has progressed in severity throughout. It is described as 10 / 10 on the Visual Analogue Scale, meaning that the pain is very severe. It is initially localised over the right scrotum, but progresses to the abdomen. It is thought that the abdominal pain is referred pain, instead of another hotspot, due to the nerve endings present in the spermatic cord. The character of the pain is sharp. It is not relieved by anything (including postural changes) and is exacerbated by coughing (the exertion of abdominal pressure).
The scrotal lump arises at the same time as the scrotal pain. It is also persistent but is not progressive in size.
The scrotal lump is located on the right and is elevated. The left side is normal. The right lump is measured at 6 cm in the longest diameter (ideally, the longer and shorter diameters should be measured. But then, the child is moving and it's difficult to obtain both measures). The skin is erythematous on the right. There are no associated masses elsewhere. There are no scars on both sides. Blue dot sign is absent (this sign is indicative of a related condition called torsion of the Hydatid of Morgagni, which is rare but congenital).
Extra tests are performed. Upon stroking the skin of the right inner thigh, the right testicle remains static. The response is normal on the left, where the left testicle is elevated. The patient has lost his cremaster reflex on the right. Moreover, when gently elevating the right testicle, the pain remains and is not mitigated. The Prehn's Sign is thus negative in this patient.
The mass is extremely tender and cannot be palpated properly. Palpation of the left side reflects no underlying abnormality.
An image of a real patient presenting with the 'Blue Dot Sign', which is a classical sign of a condition called 'torsion of the hydatid of Morgagni'. The Hydatid of Morgagni is a vestigial portion of the Mullerian Duct, also known as appendix testis. It is likened to a tiny tail of the testicle (remember Henley the Caterpillar from Disney's A Bug's Life, and his tiny wings towards the end of the show as he transformed into a 'beautiful butterfly'? Something similar). (Extracted from: https://pamadaydotnet.files.wordpress.com/2016/09/fig-1-blue-dot-sign-in-patients-with-a-left-torted-hydatid-or-mor-gagni.png?w=440)
[2] Clinical Picture showing right erythematous scrotal mass, where the right scrotum is elevated relative to the left. This case concerns a transwoman who cross-dresses and has the habit of hiding their (I am using a gender-neutral pronoun here) male genitalia by pushing the testicles up the spermatic cord and tucking the empty scrota and the penis to their buttock folds.
The vomiting has started in episodic fits at around the time of occurrence of the scrotal pain. There are no reported abnormalities regarding the consistency and colour of the vomit. It amounts to roughly one cup (roughly 250 mL) in amount per episode. The child is not dehydrated (no dry mucous membranes; capillary refill time within 2 seconds; no skin turgor).
The history is mainly obtained from the parents. Attempts have been made to obtain more information from Elmo. However, the pain was so intense that he only nodded in assent to the facts given.
Past Medical History: Mildly asthmatic, well-controlled by inhalers;
Developmental History: Unremarkable, with Elmo having met all developmental milestones on time. His height and weight also correspond to the normal ranges on the growth chart.
Past Surgical History: Unremarkable;
Family History: Grandfather died of testicular cancer ten years ago; Father presented with same issue when he was a child - also remarked by the attending physician (of the time) as having a horizontally-lying left testicle (the same testicle that presented with intense pain).
Drug History: Unremarkable, no known drug allergies particularly;
Sexual History: Not sexually active (children these days know more than those in my days...moreover, this must be confirmed explicitly since prior sexual exposure is a risk factor of a competing differential diagnosis);
Autoimmune History: Asthma, as stated above; no known otherwise;
Social History: Living with parents, no smoking/alcoholism/recreational drug use (seems dumb but this has to be routinely asked- it's not nice to assume), normal diet;
Infectious History: Unremarkable; no recent travel; Elmo has also taken all vaccines on time as prescribed by the NHS according to the vaccination schedule;
Having gathered so much information, there are several differential diagnoses that have popped up in my mind. However, one particularly stands out and I have to act fast since the top differential is an extremely serious problem.
Differential Diagnoses:
Testicular Torsion (intra-vaginal);
Extra-Vaginal Testicular Torsion;
Torsion of Hydatid of Morgagni;
Epididymitis or Epididymo-orchitis (respectively meaning inflammation of the Epididymis only, or both the Epididymis and the ipsilateral Testicle);
Testicular Cancer;
Trauma
(Unlikely, since it happens spontaneously; however, if it does happen, there's a condition called 'haematocoele' which means blood is accumulated over the scrotum, leading to bruising; to be honest, looking at it alone makes me wince)
Diagram showing the anatomy of the scrotum (extracted from: https://carolinapelvichealth.com/wp-content/uploads/2018/07/Testicle-anatomy-1024x853.jpg)
The tunica albuginea (not labelled) is the brown covering immediately covering the testis. It is underneath the visceral layer of the tunica vaginalis.
Testicular cancer is crossed out quite early on in the game, since the issue has arisen 3 hours ago and testicular cancer is a chronic disease. Testicular cancer can arise in the paediatric population and the most common subtype is the yolk sac tumour, which is a type of germ cell tumour. [3] It is simply not likely to be the case here.
Epididymitis and Epididymo-orchitis are both inflammatory conditions and can occur against the backdrop of post-infectious syndrome (where the child has had a cold a few weeks earlier) or infection. Infection of the epididymis can occur if there is a primary urinary tract infection or sexually-transmitted disease. Since the patient is neither sexually active, nor presented with relevant medical history, these are unlikely. Moreover, it has been suggested that anatomical aberrations, possibly leading to the reflux of sterile urine to the ejaculatory ducts, can contribute to these conditions as well. However, this cannot be confirmed through clinical exam alone. More importantly, the patient is Prehn's Sign negative. This significantly points us away from epididymitis and epididymo-orchitis. [4]
Sole orchitis is rare and is likely to occur if there is prior Mumps infection (such as sialadenitis, particularly parotitis; inflammation of the parotid gland which secretes saliva). This patient has received both doses of MMR vaccine. The MMR vaccine has an efficacy of 88 per cent against Mumps and confers lifelong protection. [5]
Torsions pertinent to the scrotum are more likely in this case due to spontaneous and acute onset, as well as the emergence of a scrotal lump accompanied by excruciating pain.
I have already covered the torsion of the Hydatid of Morgagni above. The patient does not have a blue dot sign and considering that this condition is rare epidemiologically, it is at best a 'backup' diagnosis.
For extra-vaginal torsion, we go back to the anatomy of the scrotum. In the diagram above, we can see that the tunica albuginea is underneath the tunica vaginalis. The tunica vaginalis is usually affixed to the inner surface of the scrotal skin via the scrotal ligament. However, this is not always the case, especially in neonates. In neonates, the scrotal ligament (originating from the lower part of an embryonic structure called the gubernaculum) is not as strong. [6] Therefore, the tunica vaginalis, by theory, is relatively mobile. Any undue movement can lead to twisting and turning of the tunica vaginalis, with the contents within following it.
However, as a child ages, this is less likely since the scrotal ligament increases in strength. Intra-vaginal torsions are more common, meaning that the torsion takes place due to the twisting of contents within the tunica vaginalis. The tunica vaginalis itself remains static. There are some risk factors associated with this. They include positive family history, cryptochordism (meaning undescended testes), and the Bell-Clapper Deformity (where the testis is horizontally-lying, rather than vertically-lying). As a side note, cryptochordism is also a risk factor for testicular cancer. [7]
Diagram showing the differences between a normal testis and a testis afflicted by the Bell-Clapper deformity. (Source unidentified; contributor(s) welcomed to approach me for acknowledgement). The Bell-Clapper deformity occurs in 12 per cent in all testicles autopsied in an autopsy series done in California. [8]
Treatment Administered:
Testicular Torsion is a very serious surgical emergency and MUST be treated with care. There is no time to play about and do a Doppler ultrasound. The patient has to be wheeled straight into the surgical theatre so that a procedure called 'orchidopexy' can be performed. It basically means untwisting the twisted spermatic cord and fixing the structures to the underlying scrotal tissue to prevent it from happening again. An interval between presentation and symptom occurrence of over 4-8 hours risks a condition called 'testicular death'. It means the ship has sailed and there is nothing you can do about it. The patient is not, de facto, infertile, since there is still the other testicle left. A procedure called orchidectomy is performed to remove the dead testicle to avoid infectious complications (any necrotised tissue has to be removed since it serves as a nidus for microbial growth). [9]
We should also not forget about supportive therapy. While we are evaluating the patient and doing a surgical plan (which should be prompt anyway), we should give anti-emetics and painkillers to the child for symptomatic relief. If the child is dehydrated, then fluid resuscitation is required. It is done by administration of predominantly normal saline (0.9% NaCl), which is a crystalloid solution.
After the surgery, Elmo stays in the hospital for a couple of days and does not report of any surgical complications. He is discharged from the hospital shortly afterwards.
Illustration of Orchidectomy (removal of the afflicted testicle) (Extracted from: https://marlin-prod.literatumonline.com/cms/attachment/3492e275-40e9-42cc-8db6-603a9a1aec81/gr2.jpg)
Concluding Remarks: In children, there are multiple differential diagnoses concerning an acute scrotum. However, in many cases, testicular torsion is the most likely differential, with epididymitis and trauma being close alternatives. Testicular torsion is a very severe disease leading to excruciating pain and impaired quality of life. If not treated promptly, it can lead to testicular death and possible infertility (especially when both testicles are affected). Family history and anatomical variants, such as the Bell-Clapper deformity, must be noted during history-taking and physical examination since they are risk factors of testicular torsion.
* A fake name is used. In all honesty, this is a case I've made up to illustrate what we usually do when a patient presenting with a scrotal mass comes to the hospital. It's quite interesting, though heart-wrenching at times. For me, I do feel empathy pain whenever I see patients wincing at their flaming scrotum.
**Normally, I like to keep them separate. However, in this case report, I think putting them together is better for the ease of reference.
References and Further Reading:
[1] Bowlin P, Gatti J, Murphy J. Pediatric Testicular Torsion. Surgical Clinics of North America. 2017;97(1):161-172. doi:10.1016/j.suc.2016.08.012.
[2] Debarbo C. Rare cause of testicular torsion in a transwoman: A case report. Urol Case Rep. 2020;33:101422. doi:10.1016/j.eucr.2020.101422.
[3] Alanee S, Shukla A. Paediatric testicular cancer: an updated review of incidence and conditional survival from the Surveillance, Epidemiology and End Results database. BJU Int. 2009;104(9):1280-1283. doi:10.1111/j.1464-410X.2009.08524.x.
[4] McConaghy JR, Panchal B. Epididymitis: an overview. Am Fam Physician. 2016 Nov 1;94(9):723-726.
[5] MMR (measles, mumps and rubella) vaccine. NHS. https://www.nhs.uk/conditions/vaccinations/mmr-vaccine/. Published 2020. Accessed December 13, 2020.
[6] Djahangirian O, Ouimet A, Saint-Vil D. Timing and surgical management of neonatal testicular torsions. J Pediatr Surg. 2010;45(5):1012-1015. doi:10.1016/j.jpedsurg.2010.02.032.
[7] Sharp VJ, Kieran K. Testicular Torsion: Diagnosis, Evaluation, and Management. Am Fam Physician. 2013 Dec 15;88(12):835-840.
[8] Caesar R, Kaplan G. Incidence of the bell-clapper deformity in an autopsy series. Urology. 1994;44(1):114-116. doi:10.1016/s0090-4295(94)80020-0.
[9] Moore S, Chebbout R, Cumberbatch M et al. Orchidopexy for Testicular Torsion: A Systematic Review of Surgical Technique. Eur Urol Focus. 2020. doi:10.1016/j.euf.2020.07.006.