1. Pediatric Endocrine and Genitourinary Emergencies
Gavin Greenfield

2. Objectives Endocrine Genitourinary Diabetic Ketoacidosis Phimosis
Paraphimosis
Penile Entrapment
Balanoposthitis
Epididymitis
Testicular (spermatic cord) torsion
Torsion of appendix testis

3. Pediatric Type 1 DM General Info
characterized by pancreatic islet beta-cell destruction mediated by immune mechanisms in predisposed individuals
classic presentation is polyuria, polydipsia, polyphagia, unexplained weight loss
presents clinically when insulin secreting reserve is 20% of normal
DKA is the initial presentation of the disease in 25% of children

4. Case
6 year old male presents with polyuria, polydipsia, vomiting, fruity breath odour. You suspect DKA. Before you are allowed to treat her son the mother wants to know how diabetic ketoacidosis develops.

5. Diabetic Ketoacidosis Pathophysiology
progressive insulin deficiency
leads to excessive glucose production and impaired glucose utilization
results in osmotic diuresis
resulting dehydration (stress) activates counter-regulatory stress hormones (epinephrine, glucagon, cortisol, GH)
insulin deficiency and elevated stress hormones results in lipolysis and protein metabolism
lipids to fatty acids to ketone bodies (beta-hydroxybutyrate and acetoacetate)
protein to ketoacids
ketone bodies and ketoacids result in metabolic acidosis
ketone bodies utilized for fuel

6. Diabetic Ketoacidosis Pathophysiology
Physiology Text p. 558

7. DKA Presentation polyuria, polydipsia vomiting, dehydration
Kussmaul’s respiration
odour of acetone on breath (fruity)
abdominal pain or rigidity
cerebral obtundation and ultimately coma
seek out precipitating event like infection
others include trauma, vomiting, psychologic disturbances, deliberate insulin omission
consider cultures

8. Case
Mom wants to know how you can be sure of the diagnosis and what tests you will do.

9. Case glucose 36 Na 130, K 5.5, HCO3 15, Cl 90 WBC 20
urine for glucose and ketones

10. DKA Diagnosis hyperglycemia and glucosuria ketonemia and ketonuria
anion gap metabolic acidosis
Other Lab Findings
leukocytosis common
normal or elevated serum potassium
total body K is almost universally low because of urinary excretion
often low measured serum sodium
explain
Hyperglycemia leads to dilutional hyponatremia
A normal serum sodium in the face of marked hyperglycemia suggests severely elevated plasma osmolality
Correction Formula:
For every rise in serum glucose of 5.6 above 5.6 the sodium should reduce by 1.6
“Hypertonic hyponatremia”

11. Case
How are you going to treat this 6 year old boy who has DKA?

12. DKA Treatment Principles
Ensure adequate ventilation and circulation (cardiovascular function)
Correct fluid deficits and electrolyte disturbances (fluid therapy)
Interrupt ketone and ketoacid production with insulin therapy and lower plasma glucose to minimize ongoing osmotic diuresis
Correct metabolic acidosis (fluids and insulin)
Assess for and treat any underlying causes of DKA (e.g., infection)
Closely monitor for and treat any complications of DKA (vital signs, neurologic monitoring)

13. DKA Treatment Fluids and Electrolytes – Initial Volume Resuscitation White, Diabetic Ketoacidosis in Children, Endocrinol Metab Clin North Am, Dec 01, 2000; 29(4): Rutledge J Initial Fluid Management of Diabetic ketoacidosis in children, Am J Emerg Med, Oct 01, 2000; 18(6):
if clinical evidence of shock
10-20 cc/kg NS over minutes and repeat only if shock persists
if no clinical evidence of shock
no bolus or bolus < 10 cc/kg
Why less aggressive fluid resuscitation?
less aggressive fluid resuscitation because of concerns re cerebral edema

14. DKA Treatment Fluids and Electrolytes – Subsequent Resuscitation
Following bolus give fluids evenly over next 24 – 48 hours
Consider giving X maintenance over next 24 hours and decrease to 1-1.5X maintenance after first 24 hours
Felner Improving management of diabetic ketoacidosis in children Pediatrics Sept 01, 2001; 108(3):
sodium, potassium, phosphate
excess chloride may aggravate acidosis so consider giving some potassium as potassium-phosphate
glucose containing solution once glucose < ~ 15
probably no role for bicarb therapy
Above study compared two groups of pt’s prior to and after a revision to their fluid replacement protocol
All pt’s received bolus of 20 cc/kg
Prior to protocol revision pt’s had fluid replacement calculated based on percentage dehydration (based on clinical exam)
After fluid protocol revision all pt’s simply given 2.5 x the maintenance rate based on admission weight. Degree of dehydration not accounted for
Second group received decreased total fluid, needed fewer IV fluid changes, decreased cost, quicker resolution of acidosis, no change in cerebral edema rate
Confounding factor was that group 1 used 0.45% saline and group 2 used 0.675% saline
UCLA group gives small boluses prn then 1.5 x maintenance only, regardless of level of dehydration
total body potassium low regardless of serum level
hypophosphatemia can shift oxygen dissociation curve to left resulting in impaired tissue oxygen delivery
Bicarb shifts oxygen dissociation curve to L which may result in decreased tissue perfusion predisposing to lactic acidosis, alkalosis accelerates entry of potassium into cells and may produce hypokalemia, bicarb may worsen cerebral acidosis while plasma pH is being restored to normal because HCO3 combines with H and dissociates to CO2 and H20. Whereas bicarb passes blood-brain barrier slowly, CO2 diffuses freely, thereby exacerbating cerebral acidosis

15. DKA Treatment - Insulin
Bolus vs. No Bolus
steady state reached in 30 min even without bolus
no clinical trials comparing the two directly
if decide to bolus dose is unit/kg R IV
Infusion Dose
0.1 unit/kg/h R (how was this number arrived at?)
if no improvement in 4 hours (pH, anion gap, bicarb, glucose) then double infusion rate
as ketosis and acidosis resolve can lower infusion rate (usually no lower than 0.05 unit/kg/h R)
0.1 u/kg/h results in a circulating level of insulin that will achieve near maximal insulin receptor saturation – except in the most severely insulin resistant patients

16. Case
You have started your treatment with intensive monitoring, fluids and insulin. Labs are slowly normalizing. 4 hours later you note the patient to have a decreased level of consciousness. Mom says “what is happening??? what did you do???”
hypoglycemia
cerebral edema

17. DKA - Complications hypoglycemia, aspiration, fluid overload with CHF
all can be avoided with careful attention to details of treatment
Cerebral Edema
complication of DKA that is restricted to children
incidence 1-2%
poor prognosis: 1/3 die, 1/3 permanent neurological impairment
usually occurs during treatment of DKA

18. DKA Complications – Cerebral Edema
Presentation
Coma or declining or fluctuating mental status
Dilated, unresponsive, sluggish, or unequal pupils
Papilledema (a late finding)
Sudden development of hypertension not detected at presentation
Development of hypotension or bradycardia
An unexpected decline in urine output without clinical improvement or tapering of intravenous fluids (SIADH)

19. DKA Complications – Cerebral Edema
Proposed Mechanisms
rapid shifts in extracellular and intracellular fluid and osmolality
CNS acidosis
cerebral hypoxia
excess fluid administration
Glaser et al. Risk factors for cerebral edema in children with diabetic ketoacidosis. NEJM Vol 344 Jan 25, 2001 No.4: 264-9
independent risk factors for cerebral edema in children with DKA: low pCO2, increased BUN, treatment with bicarbonate
Above study is a case control study. 61 children hospitalized over 15 year period in whom cerebral edema had developed. Two additional groups with DKA but without cerebral edema were also identified. Logistic regression used to compare the three groups.
Some authors have questioned aggressive fluid resuscitation as being a cause of cerebral edema in that many pt’s who get aggressively resuscitated are more dehydrated (increased BUN is one measure of dehydration)

20. Case
How can we treat this 6 year old’s swollen brain?

21. DKA Complications – Cerebral Edema - Treatment
IV Mannitol g/kg over 30 minutes, repeat prn
decrease IV rate
Hyperventilation
ICU

22. Case 2
6 year old sister of above pt presents with 3 weeks of polyuria, polydipsia and minimal weight loss. Glucose 20, Na 140, K 4.0, Cl 105, HCO3 25, urine glucose +, no urine ketones. Manage.

23. 1st presentation of Type 1 DM, not in DKA (75% of patients)
subcutaneous injections of insulin
usually start with regular insulin q 6-8 hours, total daily dose of units/kg
simultaneous monitoring of blood glucose concentration and adjustment of insulin dosing
after 1-2 days of regular insulin estimate total daily requirement and change to combined intermediate and short acting forms
Referral and Education

24. Pediatric Genitourinary Emergencies
Phimosis and Paraphimosis
Penile Entrapment
Balanoposthitis
Epididymitis
Testicular Torsion and Torsion of Appendages

25. Genitourinary Emergencies

26. Which one of the two is the emergency?
phimosis is inability to retract the prepuce (foreskin)
paraphimosis is inability to reduce the proximal edematous prepuce (foreskin)

27. Genitourinary Emergencies - Phimosis
inability to retract the prepuce
in 90% of uncircumcised males the prepuce becomes retractable by age of 3 years
can be pathologic from inflammation and scarring at the tip of the foreskin
causes include infection, poor hygiene, previous preputial injury with scarring (see next point)
forceful retraction of the foreskin can result in phimosis in the future from scarring
only reason to treat in emerg is if scarring at the tip of the foreskin occludes the preputial meatus resulting in urinary retention
dilate preputial meatus with hemostat
What is the prepuce? – fold of skin covering the glans penis (called also foreskin)
at birth phimosis is physiologic, over time the adhesions between the prepuce and glans lyse and the distal phimotic ring loosens so that in 90%...
definitive treatment is dorsal slit or circumcision

28. Genitourinary Emergencies - Paraphimosis
inability to reduce the proximal edematous foreskin (prepuce) distally over the glans penis into its naturally occurring position
resulting venous engorgement of glans can progress to arterial compromise and gangrene
true urologic emergency
What is the glans? the cap shaped expansion of the corpus spongiosum at the end of the penis (also called balanus)

29. Genitourinary Emergencies - Paraphimosis

30. Genitourinary Emergencies – Paraphimosis - Treatment
Proximal foreskin needs to be reduced distally over the glans
compress glans for several minutes to reduce edema in glans and allow foreskin to be pulled over
tightly wrap glans with elastic bandage
22-25G needle to produce several puncture wounds in glans to drain edema fluid
local infiltration of constricting band with lidocaine followed by superficial vertical incision of band; this decompresses the gland and allows foreskin reduction

31. Genitourinary Emergencies - Penile Entrapment
various objects can be placed around penis, initially occluding venous and subsequently arterial supply
hair is probably most common in kids
usually entrapped behind coronal (glans) ridge
hair may be invisible in edematous skin
manage with careful removal or consultation

32. Genitourinary Emergencies - Balanoposthitis
Balanitis is inflammation of glans
Posthitis is inflammation of foreskin (prepuce)
Treat
cleanse area with mild soap
assure adequate dryness
antifungal creams
possible circumcision
if secondary bacterial infection is present use broad spectrum antibiotic (cephalosporin)

33. Genitourinary Emergencies - Balanoposthitis

34. Case
10 year old boy presents with 3 hours of lower abdominal pain and scrotal pain (L>R). What is differential diagnosis?
What historical features can we use to sort out diagnosis?
Kadish and Bolte, A retrospective review of pediatric patients with epididymitis, testicular torsion and torsion of testicular appendages. Pediatrics 1998; 102(1):73-6
Differential Diagnosis
Epididymitis
Torsion of appendix testis
Testicular malignancy (can present with acute pain from hemorrhage into tumour)
Testicular Trauma
Above study: 90 pt’s (64 with epididymitis, 13 with testicular torsion, 13 with torsion of appendage) reviewed retrospectively to compare historical features, physical examination findings, and testicular colour Doppler ultrasound results among the 3 groups
Historical Features
Above study: only historical factor that was significantly different among the three groups of patients was duration of symptoms (testicular torsion and torsion of appendix testis had shorter duration (<12h) of symptoms compared to epididymitis)
Epididymitis pain usually not as acute onset as torsion (days rather than minutes or hours), often associated dysuria and urethral discharge
Testicular torsion is suggested by testicular pain with rapid onset and short duration

35. Genitourinary Emergencies – Epididymitis - Anatomy

36. Genitourinary Emergencies - Epididymitis
Presentation
unilateral scrotal swelling and/or tenderness, maximal over the head of the epididymis
often associated orchitis
occasionally bilateral
may have erythema and edema of overlying skin
with/without discharge
redness, swelling, fever only in severe cases

37. Genitourinary Emergencies - Epididymitis
major differential diagnosis is torsion
urinalysis usually reveals pyuria
true infectious epididymitis rare pre-puberty
if occurs pre-pubertal consider chemical cause from anatomic abnormality
like ectopic ureter entering vas
retrograde urine flow up urethra to vas
after puberty becomes most common cause of acute painful scrotal swelling in young, sexually active boys

38. Genitourinary Emergencies – Epididymitis - Anatomy

39. Genitourinary Emergencies - Epididymitis
Infectious
usually STD post pubescent (Chlamydia, Gonorrhea)
non STD causes include gram negative organisms associated with UTI, viruses, TB
investigate with urethral swab and urine culture
ultrasound can potentially be helpful
treat with Ceftriaxone or Cefixime + doxycycline if STD
ofloxacin if enteric organisms
in addition to antibiotics, treat with bedrest, scrotal elevation, analgesics
Investigations
Urethral swab for gonorrhoeae and Chlamydia
Midstream urine to look for urinary tract pathogens
?Ultrasound
Treatment
Enteric organisms
Ofloxacin 300 mg po bid for 10 days
N. gonorrhoea / C.trachomatis
Cefixime 800 mg po once + doxycycline 100 mg po bid x 10 days
Partners
treat partners of sexually transmitted within 60 days of onset of symptoms with Cefixime 400 mg po once + Azithromycin 1 g po once

40. Case Kadish and Bolte, A retrospective review of pediatric patients with epididymitis, testicular torsion and torsion of testicular appendages. Pediatrics 1998; 102(1):73-6
10 year old boy presents with 3 hours of lower abdominal pain and scrotal pain (L>R). What is differential diagnosis?
What historical features can we use to sort out diagnosis?
What features on physical examination can we use to sort out diagnosis?
Above study: 90 pt’s (64 with epididymitis, 13 with testicular torsion, 13 with torsion of appendage) reviewed retrospectively to compare historical features, physical examination findings, and testicular colour Doppler ultrasound results among the 3 groups

41. Genitourinary Emergencies – Testicular (spermatic cord) Torsion

42. Genitourinary Emergencies – Testicular (spermatic cord) Torsion
most common cause of testicular pain in boys 12 years and older
uncommon in boys less than 10 but may occur at any age (torsion of appendix testis most common cause of testicular pain between 2-10)
typically, the at risk testis is aligned along a horizontal rather than a vertical axis
2 types: intravaginal and extravaginal
intravaginal: associated with a congenital anomaly: the tunica vaginalis extends high on the spermatic cord (bell-and-clapper deformity). Testicle lies in a more horizontal lie. The most common cause beyond the neonatal period
extravaginal: much less common, occurs in perinatal period, the defect is believed to be inadequate adherence of the tuncia vaginalis to the scrotal wall. This allows the spermatic cord and entire tunica vaginalis, with its contents, to twist

43. Genitourinary Emergencies – Testicular (spermatic cord) Torsion
Presentation
torsion typically preceded by strenuous activity or trauma but does occur at rest
pain usually sudden, severe, felt in lower abdominal quadrant, inguinal canal, or testis
often associated vomiting

44. Case Kadish and Bolte, A retrospective review of pediatric patients with epididymitis, testicular torsion and torsion of testicular appendages. Pediatrics 1998; 102(1):73-6 Robinowitz R. The importance of the cremasteric reflex in acute scrotal swelling in children. J. Urol. 1984;132:89-90
All 13 patients (100%) with testicular torsion had a tender testicle and an absent cremasteric reflex
patients with testicular torsion had significantly greater incidence of tender testicle, abnormal testicular lie and absent cremasteric reflex when compared with patients with epididymitis
Rabinowitz reviewed 245 boys with acute scrotal swelling (over 7 years), no patients with a cremasteric reflex had a testicular torsion
How do you test cremasteric reflex?
stimulation of skin on front and inner side of the thigh retracts the testis on the same side; the presence indicates integrity of the first lumbar nerve segment of the spinal cord or its root
Rabinowitz – I don’t know the number of patients in the study that actually had a testicular torsion

45. Genitourinary Emergencies – Testicular (spermatic cord) Torsion
Management
if high suspicion emergent urological consultation for surgical exploration
if low or equivocal suspicion consider colour-flow duplex Doppler ultrasound or radionuclide scintigraphy
while awaiting transport attempt manual detorsion
need definitive treatment within 6 hours for testis to survive
Colour flow duplex Doppler ultrasound highly sensitive and specific for testicular torsion. Diagnosis made when intratesticular blood flow is visualized on the normal side but absent or greatly reduced on the affected side.

46. Genitourinary Emergencies – Testicular (spermatic cord) Torsion Manual Detorsion
most testes torse in a lateral to medial fashion, therefore initially attempt in medial to lateral motion (right testes counterclockwise, left testes clockwise)
painful procedure but can’t use anesthesia because won’t be able to assess relief of pain
worsening of patient’s pain should result in detorsion being done in the opposite direction

47. Genitourinary Emergencies – Testicular (spermatic cord) Torsion
Left testicular torsion in a newborn. Left hemiscrotum is ecchymotic, and the testis was slightly enlarged, quite firm, and nontender.

48. Genitourinary Emergencies – Testicular (spermatic cord) Torsion
torted necrotic testis

49. Genitourinary Emergencies – Torsion of the Appendages
appendages of the epididymis and testis have no known physiologic function
appendix testis is present in 80% of men
they are pedunculated structures and are capable of torsion
pain often more intense near head of epididymis or testis
isolated tender nodule often present
“blue dot sign”
blue dot sign: when the involved infarcted appendage is brought close to the thin, prepubertal nonhormonally stimulated scrotal skin, a blue reflection may be seen when light shines upon it. Blue dot sign is pathognomonic of torsion of the appendix testis or epididymis

50. Genitourinary Emergencies – Torsion of Appendages - Management
if diagnosis absolutely assured and confirmed by colour Doppler ultrasound (showing normal testicular blood flow) immediate surgery is not necessary
most appendages will calcify or degenerate over days and cause no harm
treat with bed rest, analgesia, NSAIDS
if any doubt about diagnosis need surgical exploration to exclude testicular torsion

51. Genitourinary Emergencies – Torsion of the Appendages
torsion of the appendix testis which is necrotic

52. Take Home Messages DKA Genitourinary Emergencies
judicious fluid use: 10 cc/kg bolus prn for shock, followed by 1.5 x maintenance
never use bicarb
probably no role for insulin bolus
Genitourinary Emergencies
if prepubescent epididymitis refer for potential anatomic abnormalities
a present cremasteric reflex makes diagnosis of testicular torsion far less likely
attempt manual detorsion while awaiting urology transfer