Pediatric Endocrine and Genitourinary Emergencies

1. Pediatric Endocrine and Genitourinary Emergencies Gavin Greenfield

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

3. Pediatric Type 1 DMGeneral 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

6. Diabetic Ketoacidosis Pathophysiology

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

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

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 ResuscitationWhite, Diabetic Ketoacidosis in Children, Endocrinol Metab Clin North Am, Dec 01, 2000; 29(4):657-82Rutledge J Initial Fluid Management of Diabetic ketoacidosis in children, Am J Emerg Med, Oct 01, 2000; 18(6): 658-60 • if clinical evidence of shock • 10-20 cc/kg NS over 30-60 minutes and repeat only if shock persists • if no clinical evidence of shock • no bolus or bolus < 10 cc/kg

14. DKA Treatment Fluids and Electrolytes – Subsequent Resuscitation • Following bolus give fluids evenly over next 24 – 48 hours • Consider giving 1.5 - 2.5 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): 735-40 • 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

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 0.05-0.1 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)

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???”

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

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

21. DKA Complications – Cerebral Edema - Treatment • IV Mannitol 0.2-1.0 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 0.3-1.0 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

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

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

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

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

40. CaseKadish 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?

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

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. CaseKadish and Bolte, A retrospective review of pediatric patients with epididymitis, testicular torsion and torsion of testicular appendages. Pediatrics 1998; 102(1):73-6Robinowitz 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

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

46. Genitourinary Emergencies – Testicular (spermatic cord) TorsionManual 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

48. Genitourinary Emergencies – Testicular (spermatic cord) Torsion

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”

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 10-14 days and cause no harm • treat with bed rest, analgesia, NSAIDS • if any doubt about diagnosis need surgical exploration to exclude testicular torsion