Post circumcision bleeding

Cite this article as:
Andrew Tagg. Post circumcision bleeding, Don't Forget the Bubbles, 2013. Available at:

Sam, a 9-day old boy, is rushed into the paediatric emergency room by his distraught parents.  Bright red blood is soaking through the front of his cloth nappy.


Bottom Line

  • The incidence of neonatal circumcision is on the decline though it may still be performed for religious reasons.
  • Circumcision reduces the transmission of HIV and HPV and reduces the risk of UTI’s.
  • The commonest complications are pain, bleeding and later infection.


What is a bris?

A brit milah, or bris, is the traditional Jewish circumcision ceremony that usually takes place on the 8th day of life.  It is carried out by a mohel. The mohel may not be a medical practitioner.  Followers of Islam are also circumcised (Khitan) though there is no prescribed time after birth in which must take place as long as it is before the age of 10.

What proportion of newborn boys are circumcised?

Whilst a number of religions including Islam and Judaism require newborn boys to be circumcised the proportion of boys that undergo the procedure is declining.  Currently, about 10-20% of boys born in Australia and less than 10% in New Zealand are circumcised.

The Royal Australasian College of Physicians’ policy statement on male circumcision states that “there is no evidence that the benefits outweigh the risk of the procedure”. The American Academy of Pediatrics holds the opposite view.


Other than religious reasons, why might a boy be circumcised?

  • Treatment of true phimosis
  • Prevention of recurrent balanoposthitis
  • Prevention of recurrent UTI’s
  • Prevention of STI transmission

A 2009 Cochrane meta-analysis found that male circumcision in sub-Saharan Africa reduced male-to-female rates of HIV transmission by 36-66%. Males who have been circumcised are unlikely to pass on HPV to their partners in life and will not get skin cancer of the penis.


Are there any contraindications to circumcision?

It is generally contraindicated if there is any genital developmental abnormality such as hypo- or epispadias or if the patient has ambiguous genitalia.  It is also not recommended in the children of parents with haemophilia until the child has been tested. It goes without saying that it should not be performed on the sick or jaundiced infant either.


What are the possible acute complications of such a circumcision and how would you treat them?

  • Pain
  • Bleeding
  • Infection

Complications occur following approximately 1 in 500 procedures. The tip of the penis is often crusted and inflamed. Sucrose should be used prior to the removal of the dressing in the neonate.

If there is profuse bleeding this may be a marker of an underlying coagulopathy and so should be tested for.  Bleeding may be due to a snipped vessel or localized inflammation/infection.  Direct pressure with a surgical dressing such as Kaltostat should halt the bleeding. Very rarely a single suture is needed to tie off a bleeding vessel.

Should there be any cellulitis to the penis in the neonate then they should be admitted for IV antibiotics.



Once the nappy was removed it was obvious that there was active bleeding to the area where the foreskin had been removed.  After giving some sucrose the vaseline gauze dressing was removed and a bleeding point identified.  When the application of a surgical dressing failed to stop the bleeding a penile block was placed and a single stitch tied off the guilty blood vessel. The clotting profile was normal and Sam was discharged to follow up with his primary care provider.



Royal Australasian College of Physicians, Paediatrics & Child Health Division. Circumcision of infant males. [cited 2013 Jul 22]

American Academy of Pediatrics Task Force on Circumcision. Male circumcision. Pediatrics. 2012 Sep;130(3):e756-85.

Siegfried N, Muller M, Deeks JJ, Volmink J. Male circumcision for prevention of heterosexual acquisition of HIV in men. Cochrane Database Syst Rev. 2009 Apr 15;(2).

Anaphylaxis and dosing errors

Cite this article as:
Tessa Davis. Anaphylaxis and dosing errors, Don't Forget the Bubbles, 2013. Available at:

Medication errors are a particular area of interest for me, so this paper caught my eye….here’s my summary of it.


It’s a paper by Benkelfat et al and is published in the September 2013 issue of the Journal of Emergency Medicine.


Benkelfat R, Gouin S, Larose G, Bailey B. Medication errors in the management of anaphylaxis in a pediatric emergency department. J Emerg Med. 2013 Sep;45(3):419-25.


It looked at using standard order forms to reduce medication errors when managing anaphylaxis in paediatric emergency.


What’s the need for the study?

It may seem surprising, but most doctors do not know the correct dose of adrenaline (epinephrine) to give in the management of anaphylaxis.

Tain and Rubython (2007) showed, in a New Zealand study, that only 20% of doctors actually knew the right dose and route of administration of adrenaline for anaphylaxis.  And Drost and Narayan (2010) found that only 15% of UK doctors would give adrenaline as recommended by the UK resuscitation guidelines.  These studies were all in adults, and one would expect that in children there would be even more error due to weight variation and low frequency of presentation.

We need to be able to treat anaphylaxis quickly, safely and optimally, as patients can deteriorate rapidly and die from this.  And an overdose of adrenaline comes with its own set of side effects.


What was the intervention?

The authors introduced a standard order form (SOF) which was given to doctors when prescribing medications for anaphylaxis (in their Paediatric Emergency Department in Canada).

They then looked at the frequency of medication errors before introducing the SOF and after introducing the SOF.


How did they find the patients?

This was done retrospectively through searching for patients coded with anaphylaxis or anaphylactic shock in their ED database.  The notes were then cross-checked with the National Institute of Allergy and Infectious Disease diagnostic criteria for anaphylaxis to make sure the patients did actually have anaphylaxis.


How did they decide what constituted an error?

Incorrect medication dosages (10% and 25% margin of error for doses); wrong drug administration; and a delay in administration (15 min delay for adrenaline, 30 min delay for other drugs).


How many patients were included?

96 patients were included – 31 in the Pre-SOF group and 65 in the Post-SOF group.  In the Post-SOF group 30 patients were SOF negative – this means that even though SOF had been introduced in the department, the SOF was not used for that patient.


What did they find?

A whopping 60% of medication charts contained at least one medication error (59% post-SOF).

The number of dosage errors did reduce significantly when the SOF was used (this was the same using either the 10% error margin or the 25% one).


Perhaps most importantly for our learning, the correct adrenaline doses for managing anaphylaxis in paediatric emergency are…

Give IM doses of 1 in 1000 adrenaline into the lateral thigh (can repeat after 5 mins if not improving). Avoid subcutaneous administration and do not use IV bolus adrenaline unless cardiac arrest is likely.  Nebulized adrenaline can be used as adjunctive therapy (to IM) but not as 1st line.

Dosing can be 0.01ml/kg of 1 in 1000, or if it is easier to remember:

  • <6 years old: 150mcg (0.15 mL) IM
  • 6-12 years old: 300mcg (0.3 mL) IM
  • >12 years old: 500mcg (0.5 mL) IM
  • Adult: 500mcg (0.5 mL) IM



Thain S, Rubython J. Treatment of anaphylaxis in adults: results of a survey of doctors at Dunedin Hospital, New Zealand. N Z Med J, 2007;120:1252.

Droste J, Narayan N. Hospital doctor’s knowledge of adrenaline (epinephrine) administration in anaphylaxis in adults is deficient. Resuscitation 2010;81:1057–8.

Anaphylaxis guidelines, Royal Children’s Hospital, Melbourne.

Penile problems

Cite this article as:
Andrew Tagg. Penile problems, Don't Forget the Bubbles, 2013. Available at:

A 5-year-old boy, Kayden, is brought in by his mother as she is concerned that there is something wrong with his penis – every time he tries to pass urine it balloons in his foreskin and goes all over the floor.

Bottom Line

  • A non-retractile foreskin may be present in up to 10% of 4-year-olds
  • Parents should not try to forcibly retract the foreskin of their child as it may lead to paraphimosis
  • Most cases of phimosis resolve with time
  • Paraphimosis occurs when a retracted foreskin is unable to be returned to its normal position due to oedema of the glans and prepuce
  • Good hygiene and avoidance of irritants are the mainstays in treating balanitis

What is phimosis?

A non-retractile foreskin is the norm in neonates and may be present in 60% of boys under one year of age.  By 4 years of age, 90% of boys are able to retract their foreskin. The majority of cases that present to either primary care or the emergency department are physiological phimosis.  Forceful retraction of the foreskin can lead to a tight circular band of scar tissue. It can then get stuck. This is true phimosis and there is some evidence that it may lead to an increased incidence of balanitis xerotica obliterans (BXO).

Penile problem - phimosis

When is phimosis a problem?

  • The foreskin is non-retractable by puberty
  • Previously retractable foreskin gets stuck
  • Presence of ballooning of urine under foreskin on micturition

How do you treat phimosis?

Application of 0.05% betamethasone ointment bd applied to the tip of the foreskin for 4-6 weeks reduces localized inflammation. It also helps loosen any inner preputial adhesions from the underlying tissue thus making it easier to retract.  The majority of children with phimosis will not need any surgical intervention.  Children with BXO may be referred early for circumcision though it may recur.

What is paraphimosis?

If the foreskin is left in the pulled back position venous return from the glans can be impaired. This leads to oedema and potential ischaemia and necrosis if left untreated.  It is commonly related to previous phimosis where a ring of tough fibrous scar tissue forms around the foreskin.  Whilst its presentation is usually obvious do not forget to consider the diagnosis of a hair tourniquet.


How do you treat paraphimosis?

Return the foreskin to its normal position as quickly as possible. It can be a painful procedure so both intranasal and topical analgesia are useful.  Lignocaine jelly (similar to that used for catheterisation) may be enough to numb the pain whilst an ice pack is applied to help reduce oedema. Once these have had ten minutes to work use the thumb of your dominant hand to push down on the glans whilst sliding the foreskin back in place. If this does not work a flexible, self-adhering bandage may be used to compress oedema.

What surgical options are available?

In the majority of occasions, the simple measures described above should be effective for returning the foreskin to its natural position. It is worth being aware of some of the surgical options available though these should only be carried out by experienced practitioners. For the older child, it may be necessary to perform a penile block (under sedation) in order to aid reduction.  More brutal surgical techniques have been described. They include multiple punctures of the glans to reduce oedema or performing a dorsal slit (cutting the fibrous ring of tissue).  The child should then be referred to a paediatric surgeon for consideration for circumcision at a later date.

How can I remember which is which?

PARAchutes come down and so does the foreskin in PARAphimosis.

What is balanitis?

Balanitis is inflammation of the glans of the penis that may be accompanied by inflammation of the overlying foreskin.  It is more common in boys that have not been circumcised.

What causes balanitis?

Balanitis may be either

  • Contact or irritant balanitis – presents as generalized redness and swelling and is often due to detergents or bubble baths
  • Candidal balanitis – often presents as redness around the glans with sparing of the meatus and  cottage cheese-like debris that is easily rubbed off
  • Bacterial balanitis – presents as redness and pain with a purulent exudate.  It may be caused by Staph. aureus or Group A beta haemolytic strep species

A penile skin swab is not needed as the majority of cases clear up with empiric treatment.

How can it be treated?

  • General methods – Advise the parents to encourage washing in lukewarm, saltwater baths and dry the penis without forcibly retracting the foreskin.  They should avoid detergents and bubble baths and if the boy is still in nappies they should change him frequently
  • Suspected irritant balanitis with/without candidal colonisation – topical hydrocortisone cream 1% with added imidazole (miconazole/clotrimazole) bd for 14 days or until settled
  • Suspected bacterial balanitis – Oral flucloxacillin for 7 days ± topical hydrocortisone 1% for discomfort.  Topical antibiotics have no proven efficacy.

Most cases of balanitis are irritant in origin and respond well to simple measures.  It can be tough to distinguish between irritant and infective forms. Treatment may need to be escalated if simple hygiene methods fail. If the symptoms are not improving after 7 days then take a penile skin swab, looking for unusual organisms.

Children with recurrent or chronic balanitis should be referred to a paediatrician or a dermatologist.

Kayden is diagnosed with phimosis and is prescribed four weeks of topical steroid cream.  When he is seen for another matter a month later you enquire as to his problem and find that he no longer misses his target.


McGregor TB, Pike JG, Leonard MP. Pathologic and physiologic phimosis:approach to the phimotic foreskin. Can Fam Physician. 2007 Mar;53(3):445-8.

Shahid SK. Phimosis in children. ISRN Urol. 2012;2012:707329.

Pohlman GD, Phillips JM, Wilcox DT. Simple method of paraphimosis reduction revisited: point of technique and review of the literature. J Pediatr Urol. 2013 Feb;9(1):104-7.

Febrile neutropenia

Cite this article as:
Henry Goldstein. Febrile neutropenia, Don't Forget the Bubbles, 2013. Available at:

You’re working as the paeds reg overnight at a regional centre when ED phones you about an incoming patient – Josef, 8 – who’s in a delayed intensification cycle of his treatment for ALL and has a fever of 38.6oC. Josef’s last chemotherapy was last week, and an FBC done 2 days ago showed WCC 4.0×109/L with an absolute neutrophil count 0.9×109/L.


Bottom Line

  • Fever in the setting of neutropaenia may be the only herald of a severe, potentially life-threatening infection.
  • Febrile neutropenia is an oncologic emergency.
  • A thorough history and physical examination are essential in cases of febrile neutropaenia.
  • Find and follow your local protocol and discuss it with a senior early.
  • Isolate the child to reduce the chances of further infection.


Why is fever in an oncology patient dangerous?

Febrile neutropaenia may be the only feature of a life-threatening infection, a major cause of morbidity and mortality in paediatric oncology patients. A 2005 study of over 12000 children established a mortality rate as high as 3%. Around 1 in 5 children will have microbiologic evidence of infection during induction chemotherapy, and this number jumps to ~40% if the fever returns upon ceasing antibiotic therapy.


You move Josef to a resus bay with isolation (and cytotoxic) procedures in place. He is febrile to 38.6oC, mildly tachycardic but normotensive. You do not identify an immediate threat to life after considering shock, overwhelming sepsis, respiratory compromise.  You take a thorough history and examine Josef.


What is the criteria for febrile neutropenia?

Fever is any temperature >38.5°C, or >38.0°C for one hour.

Neutropenia is an absolute neutrophil count  <0.5×109/L, or <1×109/L with a predicted decline to less than 0.5×109/L within 48 hours.

What are the specific features of the history?

What is the child’s oncology diagnosis and where in the treatment course are they? Different chemotherapeutic agents have relatively stronger myeloablative effects, leading to more fulminant and predictable neutropenia. As chemo agents vary with diagnosis and cycle, it’s important to clarify which medications have been given and the number of many days since last chemotherapy.

Compliant with antifungal & pneumocystis prophylaxis? Think about PCP pneumonia in any oncology child presenting with work of breathing.

What kind of central venous access +/- last accessed? Central venous access is a double-edged sword – an essential access for chemo that allows a reduction in the number of peripheral venipuncture, but also the most common source of bacterial infection in chemo kids.

Sick contacts? Remember, just because your patient has an oncology diagnosis doesn’t mean they don’t catch other age-appropriate illnesses, like gastroenteritis, upper respiratory infections and the like. Of course, they’re often more severe, but it’s important to look!


Specific features on examination?

Examine for signs of dehydration, sepsis and anaemia.

Examine the central line site. A good time to look is when the line is being accessed for cultures. Check the age of the dressing and note any erythema or cracks in the line.

Look at the skin all over.

Examine as for a fever without source.

Have a good look in the mouth – mucositis is common and a possible entry site.

Likewise, the perianal area is susceptible to skin breakdown, with or without perianal abscesses.

Take particular note of any areas of erythema.


Why is skin erythema of particular importance?

It’s worth considering the pathophysiology of erythema; local inflammatory mediators (IL-1, IL-6, TNFa) signal neutrophils to marginate, roll and undergo diapedesis to the area of action. But in the absence of a full neutrophil response, any localized erythema will likely be reduced, and pus not formed in the usual volume.

Thus, the smallest area of erythema should be considered as a possible source for infection, especially around central access sites or surgical wounds.


Which investigations are indicated?

FBC (are they actually neutropaenic?)

Blood culture (central lines/PICC)

Urea & electrolytes, consider calcium, magnesium, phosphate (dehydration, renal dysfunction, tumor lysis syndrome)

Liver function tests (liver dysfunction secondary to chemotherapy agents)

Urinalysis (clean catch)

CXR if increased work of breathing, poor SpO2

Nasopharyngeal aspirate if rhinorrhoea (make sure the PLT >50×109/L beforehand)

Consider coagulation profile

Focused investigations as per history and examination

Stool sample if diarrhoea, with C. difficile toxin if on recent antibiotics


IV access is gained, via Josef’s tunneled central line, by an experienced staff member. Bloods are sent for FBC, culture and UEG, LFT, Ca/Mg/PO4, coags.


What is the management?

Most hospitals have well-established protocols for the treatment of febrile neutropenia. Be aware of where to find yours and the choice of anti-infective agents.

Start antibiotic treatment promptly; it may be life-saving. This is not a time to faff about waiting for the results of investigations as antibiotics are the treatment irrespective of any preliminary results.

Some protocols advise anti-fungal treatment in addition to antibiotics. These protocols will vary between centres and over time with changing resistance patterns.

Remember to discuss your patient with the oncologist on call; these kids will usually need admission and, on occasion, transfer to the tertiary oncology centre.

Antibiotics are the mainstay of treatment in febrile neutropaenia. Miadema and her Dutch colleagues are presently undertaking a Cochrane Review of intravenous vs oral empiric treatment of febrile neutropaenia.

The Therapeutic Guidelines currently recommends:

Piperacillin+tazobactam 100+12.5mg/kg (Max 4+0.5g) IV q8h

or cefipime 50mg/kg (Max 2g) IV q8h

or ceftazidime 50mg/kg (Max 2g) IV q8h

If you have a suspicion of MRSA, central line infection or haemodynamically unstable, add vancomycin. Gentamicin or amikacin may be indicated.

Treat dehydration with the appropriate fluids and if the child is nauseated or vomiting, antiemetics.



Basu, K et al. Length of stay and mortality associated with febrile neutropenia among children with cancer. J Clin Oncol. 2005 Nov 1;23(31):7958-66.

RCH Melbourne CPG – Febrile Neutropenia 

Management of Fever in the Paediatric Oncology Patient v3.0 17102012 Febrile Neutropenia Protocol QPHON QCCC

Miadema et al. [Protocol] Empirical antibiotic therapy for febrile neutropenia in pediatric cancer patients. Cochrane Library.

Lehrnbecher, T. Guideline for the Management of Fever and Neutropenia in Children With Cancer and/or Undergoing Haematopoetic Stem-Cell Transplantation. JCO Dec 10, 2012, vol. 30, no 35 4427-4438

Afzal, S. et al. Risk Factors for Infection-Related Outcomes During Induction Therapy for Childhood Acute Lymphoblastic Leukemia, The Pediatric Infectious Disease Journal • Volume 28, Number 12, December 2009 pp 1064-68

Therapeutic Guidelines : Antibiotic. Severe Sepsis: empirical therapy (no obvious source of infection): febrile neutropenic patients. Therapeutic Guidelines Group. Revised June 2010. (etg40 July 2013) 


ITP – Idiopathic Thrombocytopenic Purpura

Cite this article as:
Tessa Davis. ITP – Idiopathic Thrombocytopenic Purpura, Don't Forget the Bubbles, 2013. Available at:

A 4-year-old girl presents with bruising over her legs, trunk and face.  Mum has noticed them appear over the last week.  She has been completely well with no other symptoms.  There is no history of trauma.  After an anxious 1 hour wait, the bloods are back-Hb 113, WCC 7.3, Plt 8 x 109/L.


Bottom Line

  • Uncomplicated idiopathic thrombocytopenic purpura (ITP) is new-onset bruising and bleeding with a platelet count <100 x 109/L in the absence of other symptoms
  • It generally resolves itself in 80% by six months
  • 5% will have a recurrence
  • Only treat if there is active bleeding, not just because of a low platelet count
  • Advise parents to avoid NSAIDS and lookout for signs of bleeding
  • Follow up regularly for the first six weeks or until platelet count stabilises


What is it and how did she get it?

Idiopathic thrombocytopenic purpura (ITP) is a reduction in platelet count in the absence of any other cause (<100 × 109/L).  Whilst normal platelets last eight to ten days, in ITP there are autoantibodies that destroy them in the first few hours. It has a peak incidence of two to five years of age (chronic ITP peaks in adolescence).  There is often a recent history (one to six weeks) of a viral illness or immunisation.

What are the commons symptoms and signs?

The most common sign is petechiae (1-5 mm red or purple non-blanching spots) on the skin or mucosa – these indicate capillary haemorrhages.  Some mucocutaneous bleeding is often seen, but it is rare for this to be severe (<5%).

Other symptoms of autoimmune disorders should NOT be present in ITP – e.g. no weight loss, rashes, alopecia, joint swelling. The examination should be normal with no hepatosplenomegaly or lymphadenopathy.

How is it diagnosed?

It is diagnosed by having a low platelet count with a normal haemoglobin (unlike in leukaemia, TTP, HUS and DIC). If there is a history of previous bleeding then consider other diagnoses. Bone marrow aspirate is only recommended if there is persistent bleeding in spite of a platelet count >20 × 109/L.


What treatment should we use?

The answer is simple: treat the patient not the platelet count.  Assess if the patient has haematuria, melaena, menorrhagia, epistaxis, mucosal bleeding or tonsillar purpura/petechiae.

Although there is variation between specialists, they will all be more concerned with the signs of wet purpura or haematuria rather than just the petechiae on the skin.


Prednisolone 1-2mg/kg OD for at least three weeks then taper


Methylprednisolone 30mg/kg/day for three days, then 20mg/kg /day for four days


IVIG (intravenous immunoglobulin)

Consider where there is significant bleeding (0.8-1g/kg) – can rapidly raise the platelet count Effects takes place in one to five days and lasts for two to four weeks



Only give platelets if there is an intracranial hameorrhage (ICG) or significant bleeding.  Can be effective after IVIG administration and this can prolong platelet survival (otherwise transfused platelets are quickly destroyed)


When to admit?

Admit if there is significant bleeding: epistaxis>1 hour; haematemesis; haemoptysis, intracranial haemorrhage, melaena.  Or if there is an unclear diagnosis or problematic social circumstances.

When will it go away?

Most ITP self resolves.  80% will have resolved by six months (with or without treatment).  5% of ITP patients will have a recurrence. Although it seems counterintuitive, the lower the platelet count at the beginning, the better.  Uncomplicated ITP normally has a platelet count of <20 × 109/L. Chronic ITP does not resolve within six months and accounts for 10% of ITP.

Could it be anything else?

Confirmation is based on excluding other differentials such as acute leukaemia, aplastic anaemia, HUS.  A full blood count and film us usually adequate to make the diagnosis.

What do you need to inform the parents to look out for?

While the platelets are low, the patient is at risk of bleeding.  ICH is a serious but rare (1%) side effect.  Parents should watch out for any signs of ICH, urinary bleeding, GI bleeding, excessive mucosal bleeding and menorrhagia (in older patients).

They should avoid NSAIDs while the platelet count is low.

Older children should avoid contact sports.  This is completely impractical for young children so is not helpful advice – will only stress out the parents!

When to follow up?

Patients should be reviewed within two weeks of initial presentation and have a repeat FBC. Aim for weekly GP follow up initially and then PRN until resolution.

Paediatric outpatient review at six weeks three months and six months. Refer to haematology if unclear diagnosis, unresolved after six months or a haematological malignancy is suggested by the blood count.


Selected references

Pediatric EM Morsels – Wet purpura and ITP

UMEM Educational Pearls – ITP

Royal Children’s Hospital, Melbourne – Guidelines for ITP

Princess Margaret Hospital for Children – ITP Guideline

BMJ BestPractice – ITP

Grainger JD, Rees, JL, Reeves M, Bolton-Maggs PHB.  Changing trends in the UK management of childhood ITP. Arch. Dis. Child. 2012;97:8-11.[/toggle]

Testicular trouble

Cite this article as:
Andrew Tagg. Testicular trouble, Don't Forget the Bubbles, 2013. Available at:

Jaxxon, a 13-year-old boy, forgot to wear his box whilst at cricket training and a high-speed ball swings in just before the crease and hits him in the groin. He thought nothing of it because the pain disappeared after an hour. The next day it was back and worse than before so he stumped up the courage to tell his Dad.

Bottom Line

  • Testicular torsion is a true surgical emergency
  • Do not neglect the inguino-scrotal exam in the inconsolable infant
  • No part of the history or clinical exam can rule out torsion with 100% reliability
  • Doppler US can aid diagnosis in equivocal cases but if not immediately available should not prevent a trip to the OR

What is your differential diagnosis?

What is the incidence of testicular torsion and who is at risk?

Torsion occurs in 1 in 4000 men less than 25 years old. It has a bimodal distribution. 65% of cases occur during puberty due to hormone-induced changes in size.

10% of cases occur in boys under one year of age. Some of these torsions may have occurred before delivery!

An undescended testis is at increased risk of torsion as is a testicle subjected to trauma.

What is a bell-clapper deformity?

The tunica vaginalis extends over the epididymis and spermatic cord forming a cavity in which the testicle can hang and swing freely – like the clapper of a bell.

They have an increased risk of torsion.

12% of men have this deformity at post mortem.

What factors on history or examination may help you rule out torsion?

Nothing in the history can reliably let you rule out a torsion. The pain is often acute in onset and unremitting. It may wake the patient in the middle of the night and be associated with nausea and vomiting.

Pain due to trauma should settle within an hour or so.

They may also give a history of previous similar incidents when the testis has torted and detorted spontaneously.

The classical exam finding is of an exquisitely tender, high riding testicle with a horizontal lie though  secondary hydrocele may mask this.

What is Prehn’s sign?

This is the absence of the cremasteric reflex on the side of the affected testicle. It was once thought that if the cremasteric reflex was present then it could not be torsion. Unfortunately, a number of case reports have since refuted this. Relying on the presence of the reflex to rule out torsion will lead to trouble.

What about imaging to rule out torsion?

Ischemia and infarction of the testis may occur within 4 hours of torsion though one study has suggested a 90% salvage rate if operated on within 6 hours of onset.

Rates of success drop to 50% by 12 hours.

Time is testicle and if you have a high degree of clinical suspicion then the patient should go to the operating room (regardless of fasting state) for surgical exploration.

If the history is greater than 12 hours and there is some diagnostic doubt then two methods of imaging modalities may be considered.

Colour-flow doppler ultrasound has a quoted sensitivity of 88% and a specificity of 90% and may also be useful in making alternative diagnoses such as epididymo-orchitis, rupture, or bleeding. However, if the testicle has spontaneously detorted the resultant hyperaemia on ultrasound can be confused for epididymo-orchitis. Tc-99 scintigraphy is 100% sensitive but is not widely available.

Is there anything I can try whilst waiting for the surgeons?

You could try to externally detort the testis.  This does not negate the need for scrotal exploration but may buy you some time.

The key is good procedural sedation and the rotating the affected testicle as if you were opening the pages of a book.

What about a torsion of the appendix of the testicle?

The hydatid of Morgagni (one of five possible testicular appendages) is an embryological remnant of the Mullerian system found in the upper pole of the testis. As puberty hits raging hormones make this, and other appendages swell.  This makes them more likely to twist on their precarious blood supply.

The pain of a torted hydatid is supposed to be more insidious in onset and less intense. As it becomes more ischaemic it can be visible as a small blue dot on the testicle though this may be masked by a reactive hydrocele.

Treatment is conservative with supportive underwear and NSAID’s but the diagnosis can be hard to make and so exploration is often needed.

Given the greater than 24 hour history and the possibility of testicular rupture, a colour-flow Doppler was performed. This confirmed the presence of a large haematocoele and a non-viable testis. It was removed in the operating room later the same day.

Selected references on testicular torsion

Ringdahl E, Teague L. Testicular torsion. Am Fam Physician. 2006 Nov 15;74(10):1739-43. Review. Free full text

Cuckow PM, Frank JD. Torsion of the testis. BJU Int. 2000 Aug;86(3):349-53.

Mellick LB. Torsion of the testicle: it is time to stop tossing the dice.Pediatr Emerg Care. 2012 Jan;28(1):80-6. Free full text