Testicular Torsion

Aims and objectives

• Target audience: medical students in clinical years, and PAs

• Duration: 70 minutes

• Cover 2 key urological emergencies • Provide important differential diagnoses • Pathophysiology, clinical features, investigations, management, prognosis • Multi-step SBAs: for a full understanding of the patient journey • Summary and Q&A

• Slides and previous recordings: app.bitemedicine.com

2 Case-based discussion: 1

History and examination A 14-year-old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma.

Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

3 Case history History and examination A 14-year old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma. Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

Q1 Q2 Q3 Q4

What is the most likely diagnosis?

Epididymal hypertension

Testicular torsion

Varicocele

Torsion of the testicular appendage

Epididymo-orchitis

app.bitemedicine.com 4

Explanations Q1 Q2 Q3 Q4

What is the most likely diagnosis?

Epididymal hypertension Usually presents with aching testes following arousal without ejaculation; ‘blue balls’

Testicular torsion Severe, unilateral testicular pain and swelling with vomiting in a young male and absent cremasteric reflex

Varicocele Usually only a dull ache and associated with a dragging or ‘bag of worms’ sensation

Torsion of the testicular appendage Mimics testicular torsion. Not associated with vomiting, ‘blue dot sign’ and cremasteric reflex is present

Epididymo-orchitis Gradual onset testicular pain in sexually active men, LUTS, vomiting is rare, Phren’s positive and cremasteric reflex is present

app.bitemedicine.com 6 Case-based discussion: 1

History and examination A 14-year old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma.

Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

7 Case-based discussion: 1

Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

8 Conditions to cover

Today… • Testicular torsion • Priapism

9 10 Introduction and pathophysiology

• Twisting of the testicle on its spermatic cord à ischaemia and necrosis à non-viable testis

11 Introduction and pathophysiology

Epidemiology and risk factors • Young age: two peaks; one in neonatal period and one in puberty (peak 13-15 years old) • Bell clapper deformity • Cryptorchidism • Trauma: accounts for < 10% of cases

12 Introduction and pathophysiology

Normal Bell-clapper deformity Torsion

13 Case history History and examination A 14-year old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma. Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

Q1 Q2 Q3 Q4

The young man is noted to have a bell clapper deformity. What is the cause of this?

Fibrosis of the tunica albuginea

Gubernaculum is not firmly attached to the scrotum

Failure of the tunica vaginalis to properly fix to scrotum

Dysregulation of the NO/cGMP signalling pathway in the penis

Abnormal enlargement of the pampiniform venous plexus

app.bitemedicine.com 14

Explanations Q1 Q2 Q3 Q4

The young man is noted to have a bell clapper deformity. What is the cause of this?

Fibrosis of the tunica albuginea This is the mechanism underlying Peyronie’s disease

Gubernaculum is not firmly attached to the scrotum This contributes to cryptorchidism (undescended testes)

Failure of the tunica vaginalis to properly fix to the scrotum Lack of proper fixation à high attachment of tunica vaginalis to spermatic cord à ’bell-clapper’

Dysregulation of the NO/cGMP signaling pathway in the penis This is the primary molecular mechanism of recurrent ischemic priapism

Abnormal enlargement of the pampiniform venous plexus This describes varicocele formation

app.bitemedicine.com 16 Anatomy

17 Introduction and pathophysiology

Normal Bell-clapper deformity Torsion

18 Clinical features

Symptoms Signs Testicular pain: unilateral, sudden, Swollen, high-riding, tender testicle excruciatingly painful

Nausea and vomiting: common Absent cremasteric reflex

Lower abdominal pain: referred Prehn’s negative: pain is not relieved on lifting the ipsilateral testicle

19 Cremasteric reflex

20 Prehn’s sign

21 Testicular torsion vs. epididymo-orchitis

Testicular torsion Epididymo-orchitis

Neonates, adolescent and young men Often sexually active men

Sudden onset of scrotal pain Gradual onset of scrotal pain (over days)

Tender testis +/- cord Tender along epididymis

Nausea and vomiting common Nausea and vomiting are rare

LUTS and fever usually absent LUTS and fever may be present

Testis may be high-riding or transverse Testis not high-riding, with normal lie

Cremasteric reflex absent Cremasteric reflex present

Prehn’s sign negative Prehn’s sign positive

22 Case history History and examination A 14-year-old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma. Prehn’s sign is negative and the cremasteric reflex is absent.

Observations HR 130, BP 102/70, RR 20, SpO2 94% on air, Temp 37.3

Q1 Q2 Q3 Q4

What is your next step?

FBC and CRP

Urinalysis

Urgent testicular ultrasound

Refer to urology for surgical exploration

Urgent abdominal X-ray

app.bitemedicine.com 23

Explanations Q1 Q2 Q3 Q4

What is your next step?

FBC and CRP Will not add anything if torsion is strongly suspected, and will delay definitive management

Urinalysis May be considered if the diagnosis is uncertain but is not required in this case

Urgent testicular ultrasound As per NICE, imaging studies would not be performed in this case (see next slide)

Refer to urology for urgent surgical exploration Suspected torsion is a urological emergency and delay will result in loss of the testis

Urgent abdominal X-ray Not indicated and will not provide any useful information

app.bitemedicine.com 25 “In patients with a history and physical examination suggestive of torsion, imaging studies should NOT be performed as they may delay treatment, therefore prolonging the ischaemic time”

NICE Guidelines August 2019

app.bitemedicine.com 26 Investigations

Primary investigations: • Surgical exploration: should be performed immediately if there is high clinical suspicion; perform within 6 hours to prevent irreversible damage

Investigations to consider: if clinical suspicion is high, do not delay surgery • Testicular ultrasound: operator-dependent; ‘whirl-pool’ sign suggests torsion • Urinalysis: may suggest an alternative diagnosis, e.g. leukocytes and nitrites in epididymo-orchitis

27 Case history History and examination A 14-year old male presents to the emergency department with a 3-hour history of sudden-onset, severe pain in his right testicle. This was initially intermittent but is now constant. He has vomited twice. There is no history of trauma. Prehn’s sign is negative and the cremasteric reflex is absent.

Intra-operatively, the affected testicle is found to be viable.

Q1 Q2 Q3 Q4

What is the most appropriate management of this patient?

Bilateral orchiopexy

Ipsilateral orchiectomy and contralateral orchiopexy

Ipsilateral orchiopexy and contralateral orchiectomy

Manual detorsion

Ipsilateral orchiectomy

app.bitemedicine.com 28

Explanations Q1 Q2 Q3 Q4

What is the most appropriate management option for this patient?

Bilateral orchiopexy The affected testicle is untwisted and fixed, as is the contralateral testicle to prevent contralateral torsion

Ipsilateral orchiectomy and contralateral orchiopexy Used in the case of a non-viable testicle (e.g. necrotic)

Ipsilateral orchiopexy and contralateral orchiectomy Not appropriate

Manual detorsion Considered in cases of surgical delay; not appropriate in this case as the patient is in theatre

Ipsilateral orchiectomy May be performed if the ipsilateral testicle is non-viable, however the contralateral testicle should be fixed (orchiopexy)

app.bitemedicine.com 30 Management

Viable testicle • Bilateral orchiopexy: affected testis is untwisted and fixed; contralateral testis always fixed too

Non-viable testicle (e.g. necrotic) • Ipsilateral orchiectomy and contralateral orchiopexy: removal of the affected testis; contralateral testis is always fixed

In cases of surgical delay • Manual detorsion: a temporary measure only performed if surgery is not available within 6 hours

31 Complications

System Complication

Urological • Recurrent torsion: may occur despite orchiopexy (very rare) • Subfertility/infertility: orchiectomy results in decreased spermatogenesis

32 Prognosis

Within 4-6 hours: testis can be saved in the majority of cases A delay of >10-12 hours: irreversible ischaemia and necrosis ≥24 hours: testis is salvageable in <10% of cases

33 Case-based discussion: 2

History and examination A 30-year-old Afro-Caribbean male presents to the emergency department with a painful, sustained erection for the past 5 hours, without sexual arousal. He has never had an episode like this previously.

The penis is painful and rigid.

Observations HR 129, BP 120/80, RR 21, SpO2 96%, Temp 36.9

34 Case history History and examination A 30-year-old Afro-Caribbean male presents to the emergency department with a painful, sustained erection for the past 5 hours, without sexual arousal. He has never had an episode like this previously. The penis is painful and rigid.

Q1 Q2 Q3

What is the most likely diagnosis?

High-flow priapism

Low-flow priapism

Non-ischaemic priapism

Stuttering priapism

Adult-type priapism

app.bitemedicine.com 35

Explanations Q1 Q2 Q3

What is the most likely diagnosis?

High-flow priapism Also known as ‘non-ischaemic’ priapism and is painless, rare, and often due to blunt perineal trauma

Low-flow priapism Also known as ’ischaemic’ priapism, a urological emergency with a painful, sustained erection

Non-ischaemic priapism As described above (also known as ‘high-flow’ priapism)

Stuttering priapism Rare and associated with frequent prolonged and painful erections; unlikely as this is the patient’s first episode

Adult-type priapism This is not a recognised classification system or subtype of priapism

app.bitemedicine.com 37 38 Case-based discussion: 2

The penis is painful and rigid.

Observations HR 129, BP 120/80, RR 21, SpO2 96%, Temp 36.9

39 Case-based discussion: 2

The penis is painful and rigid.

Observations HR 129, BP 120/80, RR 21, SpO2 96%, Temp 36.9

40 Introduction

• Definition: a prolonged penile erection (>4 hours) which is maintained without sexual stimulation and persists despite ejaculation and orgasm (BAUS 2018)

Categorised into • Low-flow: due to inadequate venous outflow from corpus cavernosum à painful penile ischaemia • High-flow: excessive arterial inflow + sufficient venous outflow à no penile ischaemia à painless • Stuttering/recurrent: frequent, prolonged, painful erections, ischaemic, usually self limiting (often in SCD)

41 Pathophysiology: low-flow (ischaemic) vs. high-flow (non-ischaemic) (Veno-occlusive)

42 Low-flow priapism High-flow priapism

More common Rare

Inadequate venous outflow Excessive arterial influx with sufficient (ISCHAEMIA) venous outflow (NO ISCHAEMIA)

Aetiology: Aetiology: • Drugs: sildenafil (for erectile • Blunt perineal trauma: e.g. saddle dysfunction), trazodone, prazosin injury • Hypercoagulable state: SCD, • Penetrating injury: e.g. local penile thalassaemia injections • Neoplastic: e.g. bladder/prostate • Congenital vascular malformations cancer • Neurological: e.g. spinal cord stenosis, cauda equina

43 Case history History and examination A 30-year-old Afro-Caribbean male presents to the emergency department with a painful, sustained erection for the past 5 hours, without sexual arousal. He has never had an episode like this previously. The penis is painful and rigid.

Q1 Q2 Q3

Which of the following is the most likely underlying cause of this patient’s presentation?

Straddle injury

Marijuana use

Sickle cell disease

Coital trauma

Anaemia

app.bitemedicine.com 44

Explanations Q1 Q2 Q3

Which of the following is the most likely underlying cause of this patient’s presentation?

Straddle injury Causes high-flow priapism (painless)

Marijuana use No evidence to suggest that marijuana use causes priapism (alcohol and cocaine may predispose)

Sickle cell disease A very important cause of low-flow priapism, particularly in Afro-Caribbean patients

Coital trauma Causes high-flow priapism (painless)

Anaemia Anaemia alone should not cause priapism

app.bitemedicine.com 46 Clinical features

Low-flow priapism High-flow priapism

Early presentation May be up to 72-hour delay between initial injury and priapism onset

Painful, rigid erection Painless or uncomfortable

Progressive increase in pain as Penis not completely rigid duration of priapism increases

No evidence of perineal or penile Evidence of perineal or penile trauma trauma (e.g. perineal swelling, haematuria)

Recurrent (stuttering) priapism

Frequent, prolonged, usually self- limiting, painful erections in men with SCD 47 Investigations

Diagnose and differentiate low-flow vs. high-flow: as per BAUS (2018) • Corpus cavernosum aspiration: • Low-flow: dark red, hypoxic, hypercapnic, acidotic • High-flow: bright red, oxygenated • Urgent penile Doppler study: can help to confirm the diagnosis (do not delay aspiration + washout) • Low-flow: poor arterial influx • High-flow: high arterial influx and adequate outflow

Other primary investigations: • FBC: leukocytosis (? infection), anaemia/raised reticulocytes (? SCD)

Investigations to consider: • Blood film: in select cases to diagnose haematological disorders • CT/MRI Abdomen and Pelvis: if suspected underlying pelvic/abdominal malignancy • Penile MRI: in refractory cases 48 Case history A 30-year-old Afro-Caribbean male presents to the emergency department with a painful, sustained erection for the past 5 hours, without sexual arousal. He has never had an episode like this previously. The penis is painful and rigid.

Corpus cavernosum aspiration: dark red, hypoxic, hypercapnic, acidotic blood

Q1 Q2 Q3

What is the most appropriate management option for this patient?

Winter shunt

Corporal washout and phenylephrine injections

Corporal aspiration

Penile prosthesis

Analgesia and monitor

app.bitemedicine.com 49

Explanations Q1 Q2 Q3

What is the most appropriate management option for this patient?

Winter shunt Usually considered when aspiration, washout and phenylephrine injections are unsuccessful

Corporal washout and phenylephrine injections Most important next step in management to achieve detumescence

Corporal aspiration Diagnostic and therapeutic, but washout and phenylephrine injections are also required

Penile prosthesis Considered if other options fail or duration of symptoms >72 hours

Analgesia and monitor Low-flow priapism is a surgical emergency

app.bitemedicine.com 51 Management: low-flow priapism

First-line: • Aspiration of sludged blood and corporal washout • Repeated intracavernosal injections of phenylephrine

52 Management: low-flow priapism

First-line: • Aspiration of sludged blood and corporal washout • Repeated intracavernosal injections of phenylephrine

Second-line: • Shunt: decompression of the penis by creating a shunt

Winter shunt T-shunt 53 Management: low-flow priapism

First-line: • Aspiration of sludged blood and corporal washout • Repeated intracavernosal injections of phenylephrine

Second-line: • Shunt: decompression of the penis by creating a shunt

Other considerations: • Analgesia • Antibiotic cover • Role of penile prosthesis?

Winter shunt T-shunt 54 Management: low-flow priapism

• Conservative management • Usually no treatment is needed • Persistent high-flow priapism: consider superselective embolisation

56 Complications

System Complication

Urological • Penile / cavernous fibrosis à irreversible impotence • Penile ischaemia • Erectile dysfunction

57 Conditions covered

Today… • Testicular torsion • Priapism

58 Top-decile question

Testicular torsion vs. torsion of appendix testis

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66 References

Slide 9/27: Kalumet / CC BY-SA (http://creativecommons.org/licenses/by-sa/3.0/). https://commons.wikimedia.org/wiki/File:Hodendrehung_Post-OP.jpg Slide 15: Henry Vandyke Carter / Public domain. https://commons.wikimedia.org/wiki/File:Gray1143.png Slides 18/19: Creator / Public domain. https://commons.wikimedia.org/wiki/File:Hanging_testicles.JPG Slide 33: https://en.wikipedia.org/wiki/File:Pompeya_er%C3%B3tica6.jpg#filelinks Slide 33: https://en.wikipedia.org/wiki/File:Pompeya_er%C3%B3tica5.jpg Slide 36: Henry Vandyke Carter / Public domain. https://commons.wikimedia.org/wiki/File:Gray1158.png Slide 52: Henry Vandyke Carter / Public domain. https://commons.wikimedia.org/wiki/File:Gray1148.png

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