Pediatric Endocrine and Genitourinary Emergencies

Gavin Greenfield

Objectives

• Endocrine– Diabetic Ketoacidosis

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

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

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.

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

Diabetic Ketoacidosis Pathophysiology

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

Case

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

Case

• glucose 36

• Na 130, K 5.5, HCO3 15, Cl 90

• WBC 20

• urine for glucose and ketones

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

Case

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

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)

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

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

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)

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???”

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

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)

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

Case

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

DKA Complications – Cerebral Edema - Treatment

• IV Mannitol 0.2-1.0 g/kg over 30 minutes, repeat prn

• decrease IV rate

• Hyperventilation

• ICU

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.

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

Pediatric Genitourinary Emergencies

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

Genitourinary Emergencies

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

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

Genitourinary Emergencies - Paraphimosis

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

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

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)

Genitourinary Emergencies - Balanoposthitis

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

Genitourinary Emergencies – Epididymitis - Anatomy

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

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

Genitourinary Emergencies – Epididymitis - Anatomy

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

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?

Genitourinary Emergencies – Testicular (spermatic cord) Torsion

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

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

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

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

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

Genitourinary Emergencies – Testicular (spermatic cord) Torsion

Genitourinary Emergencies – Testicular (spermatic cord) Torsion

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”

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

Genitourinary Emergencies – Torsion of the Appendages

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