Pediatric Endocrine and Genitourinary Emergencies

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Pediatric Endocrine and Genitourinary Emergencies

• Gavin Greenfield

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Objectives

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

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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

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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.

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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

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Diabetic Ketoacidosis Pathophysiology
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DKA Presentation

• polyuria, polydipsia
• vomiting, dehydration
• Kussmauls 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

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Case

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

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Case

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

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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

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Case

• How are you going to treat this 6 year old boy
  who has DKA?

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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)

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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 lt 10 cc/kg

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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 lt 15
• probably no role for bicarb therapy

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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)

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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???

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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

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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)

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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

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Case

• How can we treat this 6 year olds swollen brain?

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DKA Complications Cerebral Edema - Treatment

• IV Mannitol 0.2-1.0 g/kg over 30 minutes, repeat
  prn
• decrease IV rate
• Hyperventilation
• ICU

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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.

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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

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Pediatric Genitourinary Emergencies

• Phimosis and Paraphimosis
• Penile Entrapment
• Balanoposthitis
• Epididymitis
• Testicular Torsion and Torsion of Appendages

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Genitourinary Emergencies
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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

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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

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Genitourinary Emergencies - Paraphimosis
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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

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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

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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)

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Genitourinary Emergencies - Balanoposthitis
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Case

• 10 year old boy presents with 3 hours of lower
  abdominal pain and scrotal pain (LgtR). 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

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Genitourinary Emergencies Epididymitis - Anatomy
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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

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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

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Genitourinary Emergencies Epididymitis - Anatomy
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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

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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 (LgtR). 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?

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Genitourinary Emergencies Testicular (spermatic
cord) Torsion
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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

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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

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CaseKadish and Bolte, A retrospective review
of pediatric patients with epididymitis,
testicular torsion and torsion of testicular
appendages. Pediatrics 1998 102(1)73-6Robinowit
z R. The importance of the cremasteric reflex in
acute scrotal swelling in children. J. Urol.
198413289-90

• 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

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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

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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 cant use anesthesia
  because wont be able to assess relief of pain
• worsening of patients pain should result in
  detorsion being done in the opposite direction

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Genitourinary Emergencies Testicular (spermatic
cord) Torsion
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Genitourinary Emergencies Testicular (spermatic
cord) Torsion
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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

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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

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Genitourinary Emergencies Torsion of the
Appendages
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Take Home Messages

• DKA
• 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