PEDIATRIC GI EMERGENCIES

Kevin Levere

Jan 16, 2003

Objectives

• To appreciate differences from adults in• GI bleeds• Pancreatitis• Liver disease

• To review common pediatric abdominal emergencies

• IBD, toxic megacolon, metabolic disease, constipation, colic, and jaundice are not discussed

Pediatric GI Bleeds

• Many etiologies similar to adult• Frequency differs significantly• Some uniquely pediatric causes

• Mortality lower than adult counterparts• Fewer comorbidities• Relatively greater physiological flexibility

• Division between upper and lower• Ligament of Treitz

Case

• 4wo ex35wker girl, breast feeding. Spat up a shot-glass worth of red blood.

• Apart from oral thrush, her exam is normal.• Hx: Got Vit K. No meds. Growing. No BRBPR.• FHx: No bleeding diatheses. Mom not on

meds, but has mastitis.• Apt test turns yellow-brown

Case

• 2yo boy passing several fairly large maroon coloured BMs, x12hrs. Painless. Normal BM Hx until then.

• 37, 140, 90/70, 24, pale but playful. OB+. Hgb 60, rest of CBC normal.

• Which is best course of action?• A) cross-match, radionuclide scan, IV bolus, surgery• B) cross-match, IV bolus, scan, surgery• C) cross-match, IV bolus, surgery, scan• D) IV bolus, 0- transfusion, surgery, forget the scan

 DIFFERENTIAL DIAGNOSIS OF GI BLEEDING *

Upper GI Bleed

Newborns Infants Children

Swallowed maternal blood Epistaxis Epistaxis

Hemorrhagic gastritis Gastritis Tonsillitis/sinusitis

Stress ulcer Esophagitis Gastritis

Idiopathic Stress ulcer Mallory-Weiss tear

Coagulopathy Gastric/duodenal ulcer Gastric/duodenal ulcer

Gastric outlet obstruction Foreign bodies Medications

Gastric volvulus Gastric volvulus Tumors

Pyloric stenosis Esophageal varices Hematologic disorders

Antral/pyloric webs   Esophageal varices

    Munchausen by proxy

Lower GI Bleed

Anal fissure Anal fissure Anal fissure

Allergic proctocolitis Infectious diarrhea Infectious diarrhea

Infectious diarrhea Allergic proctocolitis Polyp

Hirschsprung’s disease Meckel’s diverticulum Hemorrhoids

Necrotizing enterocolitis Intussusception Inflammatory bowel disease

Volvulus GI duplication Henoch-Schonlein purpura

Stress ulcer Peptic ulcer Meckel’s diverticulum

Vascular malformation Foreign body Peptic ulcer

GI duplication  

Hemolytic uremic syndrome

    Vascular malformations

From Mezoff AG, Preud Homme DL. Contemp Pediatr 1994; 11:60-92.

* In order of frequency, most common in bold.

Pediatric GI Bleeds

• Hemorrhagic disease of the newborn• Early (<1wk)

• Vitamin K deficiency• Rare now administration of vitamin K shortly after birth

has become routine

• Maternal anticoagulant and intrapartum antiepileptic drug use

• Late onset (2-6mos)• Fat malabsorption

Pediatric GI Bleeds

• Ingestion of maternal blood• Apt Downey test

• 1:1 stool with tap water, spin• 5:1 supernatant with 1% NaOH• After 2 mins

• Pink = fetal Hgb• Yellow = maternal Hgb

Pediatric UGI Bleeds

• Upper GI Bleeds• No good epidemiologic data outside PICU

• 6-25%, depending on prophylactic therapy• 0.4% considered significant

• Commonest endoscopic findings• Gastritis• Esophagitis• Varices• Ulcers• Mallory-Weiss tears

Pediatric UGI Bleeds

• Ulcers and Gastritis• Gastric acid production begins shortly after birth• Ulcers relatively rare

• Most are associated with NSAIDs and stress

• H. pylori infection• Diffuse nodular gastritis commonest presentation

• Infection increases with age• <5yo rare

• ~20-50% by 10yo (SES dependent)

• 40-80% adults (SES dependent)

Pediatric UGI Bleeds• Esophagitis

• Severe GERD• FB or chemical injury• Infection

• Vascular anomalies• Hemangiomas• Hereditary telangiectasia• Aortoenteric fistulas

• Congenital malformations• Duplications, obstructions

• Predisposed to mechanical injury

Pediatric UGI Bleeds• Management

• Similar to adults• ABC’s, history and physical• NG

• Same dilemma as in adults, but no pediatric data• No longer use ice cold lavages

• Diagnostics• CBC, PTT/INR, LFT’s, cross-match• Limited roles for imaging – CXR, U/S, angiography

• Medications• Acid-suppressive +/- visceral vasoconstrictive• Limited but supportive data; dosing is the key difference

Pediatric UGI Bleeds

• Management• Endoscopy

• Indications less standardized than in adults• For severe or persistent/recurrent bleeding

• Diagnostic• No data on risk of rebleeding based on findings

• Interventional as in adults• Size the limiting factor for techniques

• Safety similar to adult data• Complication rate 0.3% in retrospective study of 2026

Pediatric UGI Bleeds

• Management• Surgery as back-up

• Failed endoscopic therapy• Surgical lesion, e.g. Dieulafoy’s lesion

• Take home point• Non-GI sources as common as GI sources in

“pediatric UGI bleeds”

Pediatric LGI Bleeds

• Lower GI Bleeds• 0.3% of ED visits• 50% <1yo

• Allergic colitis, fissures commonest

• >1yo• Infectious GE, fissure, polyps commonest

• Ann Emerg Med, 1994

Pediatric LGI Bleeds

• Diagnosis• Nature of bleeding helps localize origin

• N.B. hematochezia unreliable in infants, with their faster GI transit times

• History and physical• If doubt bleeding, or suspect false-pos guaiac

• Immunodiffusion of fecal Hgb• Sensitivity and specificity ~70% each, as with guaiac

• Fecal alpha-1-AT measurement• Sensitivity 88%, specificity 90%• Also elevated in protein-losing enteropathy???

Pediatric LGI Bleeds

• Numerous laboratory and imaging options• Blood• Stool• Urine• Plain films, nuclear scans, endoscopy

• Commonly used in painless bleeding

• Non-barium contrast studies, U/S, CT, MRI, angiography

• Dependent on suspected etiology

Pediatric LGI Bleeds

• Management• Similar to adults

• ABC’s, history and physical• NG

• Aspirate can help identify if blood from UGI source

• Diagnostics• CBC, PTT/INR, LFT’s, cross-match, etc

• Medications• Visceral vasoconstrictive• Supportive data for role in LGI bleeds

• Treat underlying cause

Pediatric LGI Bleeds

• Neonatal• NEC

• Risk factors• Prematurity (87% of cases)• Hypoxia• Sepsis• Acidosis• Early enteral feeds• Umbilical vascular catheter• PDA

• Epidemics support infectious component

Pediatric LGI Bleeds

• Neonatal• NEC

• Onset• Typically <4wks of age; can be late as 3mos

• Manifestation• Abdominal distention, gastric retention• Poor feeding, V/D, lethargy, apnea• Gross blood in stools in only 25%• Severe cases lead to SIRS

Pediatric LGI Bleeds

• Pneumatosis intestinalis in 50-75% at diagnosis

• Portal venous gas in severe disease

Pediatric LGI Bleeds

• Neonatal• NEC

• Complications• Mortality up to 5%• Strictures in 10%

• Treatment• Supportive• Antibiotics, gut rest• Surgical back-up

Pediatric LGI Bleeds

• Neonatal• Hirschsprung disease

• Congenital aganglionic megacolon• Delayed (>48hr) passage of meconiom• History of (often progressive) constipation• 25% have blood in stool

• Diagnosis• Contrast enema – proximal dilation = normal bowel• Rectal manometry• Biopsy with absent ganglion cells

• Treatment is surgical resection

Pediatric LGI Bleeds

• Infants• “Allergic” colitis

• Intolerance to cow’s milk protein• 0.2-7.5% prevalence• Soy protein intolerance in 14-25% of these• Typically not IgE mediated• Resolves in most by 2yo

• Treatment is dietary restriction

• Volvulus• Intussusception

Pediatric LGI Bleeds

• Children• Infectious enterocolitis• HUS, HSP, pseudomembranous colitis• IBD• Vascular malformations

• Hemangiomas, telangiectias, varices, hemorrhoids

• Polyps• Other tumors, e.g. colon cancer, are rare

• Trauma• FB, NAT

Pediatric LGI Bleeds

• Meckel’s diverticulum• Vitellointestinal duct remnant

• 50% have gastric mucosa

• Most important source of small bowel bleeding• Gastric mucosal ulceration vs intussusception

• Rule of 2’s• 2% of population• 2:1 male:female• 2 feet from IC valve• Under 2yo commonest

• Diagnosed by radionuclide scan• Treatment is surgical resection

Pediatric LGI Bleeds

• Take home point• Think of NEC and Meckel’s

• Both relatively common• Both potentially serious

Case

• 13yo girl with 3days fever, despite Tylenol used round the clock. Also c/o N/V, anorexia, moderate epigastric pain. Today a bit jaundiced. Denies EtOH or other drug use. Her boyfriend has Mono.

• 38.7, 100, 120/85, 22. Abdo tender, a bit distended, else exam normal.

Pancreatitis

• Differential• Gastroenteritis• Ulcer• Hepatitis• Pneumonia• Biliary tract obstruction

Pancreatitis

• Much less common than in adults• Pathogenesis

• Cell injury (toxin or otherwise) sets off pancreatic autodigestion and inflammatory response

Pancreatitis

• Acute• Interstitial edema

• Usually resolves within 2-7 days – mortality 5%

• Complications – rare• Pseudocyst – slow to mature and resolve (weeks)• Phlegmon, necrosis +/- hemorrhage• SIRS – 50-80%+ mortality

• Chronic/recurring• Endocrine and exocrine insufficiencies• Calcification

Pancreatitis

• Etiology – the key difference in pediatrics• Trauma

• Blunt injury – commonest cause; think of NAT• Infection

• Viral (not just mumps), et al• Multisystem disease

• CF, collagen vascular disease, vasculitits, metabolic• Obstructive

• Congenital anomalies, biliary microlithiasis• Drugs and Toxins

• EtOH, acetaminophen• Hereditary – autosomal dominant• Idiopathic (25%)

Pancreatitis

• Clinical Picture• Abdominal pain

• Steady, epigastric, with tenderness, distention

• Persistent vomiting• Proportional to abdominal pain

• Fever• Associations in complicated picture

• Mass, e.g. in 50% of cases with pseudocyst• Asictes, pleural effusions, hypocalcemia, hyperglycemia,

jaundice• Grey Turner (flank) and Cullen (periumbilical) signs• MSOF and shock

Pancreatitis

• Diagnosis• Serum lipase

• sensitivity 86% to 99%; specificity of 50% to 99% • Elevated 1-2 weeks longer than amylase

• Serum amylase• sensitivity 75% to 92%; specificity 20% to 60%

• General lab evaluation• Urinary trypsin activation peptide (TAP)

• Investigational prognosticator

• Ranson and APACHE-II criteria• Not reliable in pediatrics

Pancreatitis

• Diagnosis• Diagnostic imaging – 20% normal at first

• Plain films• Pleural effusions, “sentinel loop”, “cut-off sign”

• U/S• Biliary tract evaluation, or follow-up of cysts/abcesses

• CT• Usually only if poor U/S visualization

• ERCP/MRCP• Considered if recurrent/undiagnosed problems• For suspected stones

Pancreatitis

• Acute pancreatitis • Pseudocyst 5 months after acute episode

Pancreatitis

• Management• “Pancreatic rest”

• NPO +/- NG

• Pain control• Meperidine – opioid causing least enterobiliary pressure by

contraction of Sphincter of Oddi

• Fluid and electrolyte homeostasis• Surgical and antibiotic interventions rare

• Abcess, infected pseudocyst, necrosis, hemorrhage

• Address underlying cause if able

Pancreatitis

• Take home point• Trauma, including non-accidental, is the

commonest cause

Liver Failure

• Fulminant hepatic failure• Acutely impaired hepatocyte function with

• Encephalopathy within 8 weeks of initial symptoms, with a previously healthy liver, or

• Encephalopathy within 2 weeks of jaundice, even if previously underlying liver dysfunction

Liver Failure

• Cirrhosis• End-stage of acute or chronic disease

• Fibrosis following injury from hepatitis, necrosis, or biliary obstruction

• Restricts blood flow, creating portal hypertension, and ischemia further impairing heptocyte function

• Biliary atresia• Commonest cause of liver failure in pediatrics

Fulminant Hepatic Failure

• Etiology• Viral

• HBV likely the commonest of these• TORCH

• Toxins• Acetaminophen the most common toxin• Amanita phalloides, EtOH, PHT, VPA…

• Vascular• Veno-occlusive disease, ischemia, thrombosis

Fulminant Hepatic Failure

• Etiology• Metabolic

• Wilson’s, neonatal iron storage disease, tyrosinemia, galactosemia, fatty acid oxidation defects, mitochondrial disease

• Reye’s syndrome

• Others• Malignancy, autoimmune, idiopathic (20-40%)

Fulminant Hepatic Failure

• Clinical Picture• Jaundice• Fetor hepaticus• Fever• Anorexia• Vomiting• Abdominal pain• Encephalopathy

• Grades I-IV = mild confusion to coma

Fulminant Hepatic Failure

• Note about ALT• Most specific for hepatocellular toxicity,

BUT…• Falls as exhaust hepatocyte supply

• Markers of function• Bilirubin, albumin, INR, glucose, NH3

Fulminant Hepatic Failure

• Complications• Cerebral edema• Renal failure• Coagulopathy

• GIT commonest site of bleeding

• Hypoglycemia• Metabolic instability• Infection• MSOF

Fulminant Hepatic Failure

• Treatment in ED• Eliminate or treat cause if able

• N-acetylcysteine, charcoal

• Supportive therapy• ABC’s, then

• Increased ICP• Mannitol or hyperventilation; no Dexamethasone

• Bleeding• FFP; FVIIa being studied• H2 blocker > sucralfate; PPI role being studied

Fulminant Hepatic Failure

• Treatment thereafter• Ongoing supportive care

• Corticosteroids shown to worsen outcome

• For encephalopathy• Lactulose• Neomycin• Low protein diet• Plasmapheresis

• Specific therapies• Antivirals, TIPS, thrombolysis, transplant

Fulminant Hepatic Failure

• Prognosis• 50% experience serious infection• Mortality with medical care ~70%

• Worse prognosis if <10yo or >40yo

• Transplant survival 50-80%• Spontaneous recovery

• More likely with low grade encephalopathy• Viral and acetaminophen 50-60%• Wilson’s and some idiosyncratic reactions 10-20%

Liver Failure

• Take home point• Infants with fulminant hepatic failure typically

have congenital problems, i.e. underlying liver disease

• They don’t strictly fit the definition for fulminance

Case

• 5yo with 1day of vomiting and occasional diarrhea. Severe abdo pain on and off every 15 mins or so. No sick contacts.

• 37.9, 120, 110/75, 22. Abdo diffusely tender, no peritonitis, no mass. CBC normal, OB+.

Intussusception

• Differential Diagnosis• GE• Formula intolerance• Volvulus• Incarcerated hernia

• inguinal>internal

Intussusception

• Definition• Invagination of proximal intussusceptum into

distal intussuscipiens• Ileocolic>cecocolic>ileoileal

• Epidemiology• 1-4/1,000 live births• 50% by 1yr,80% by 2yrs, 90% by 5yrs• Peak incidence 10mos (2mos-2yrs)• 2-4:1 male:female

Intussusception

• Etiology• 90-95% idiopathic in <2yo’s

• Peyer patches’ hypothetical role

• Pathologic lead points commoner in >5yo’s• Meckel’s diverticulum commonest• Polyps, HSP lesions, lymphomas, appendix, HUS,

parasite, hemangiomas, metastases, CF, fecolith

• Entrapped mesentery, bowel edema cause ischemia

Intussusception

• Clinical Picture• Classic triad seen in <20%

• 13% have less than or equal to 1 of these features

• Intermittent abdominal pain (50-90%)• Vomiting (60-80%)• Blood and mucous in stools (20-60%)

• Currant jelly stools are a late sign (20%)

Intussusception

• Clinical Picture• Nonspecifics

• Fever• Poor feeding• Lethargy

• Physical Exam• “Dance’s sign” = sausage in RLQ (up to 70%)• Abdominal mass in 25-85%

Intussusception

• Diagnosis• Plain films

• Normal in <30%; suspect mass in 70%; obstruction

• Barium or H2O-soluble enema• Gold standard for diagnosis and therapy• Misses ileoileal cases, e.g. Meckel’s

• U/S • High accuracy• Adjunct to enemas, to monitor therapeutic effect

Intussusception

• Plain film

Intussusception

• Coil-spring appearance

• Target on U/S

Intussusception

• Therapy• Barium or H2O-soluble enema

• Successful reduction in 80% <48hrs, 50% >48hrs

• Air-contrast enema• Studies suggest at least as effective as barium• Bowel perforation in 0.1-0.2% vs 0.5-2.5% with Ba

• Surgical• Reduction vs resection

• Dexamethasone• Hypothesis that it might reduce recurrence

Intussusception

• Initial misdiagnosis in up to 60%• GE commonest• Consider in differential of lethargy

• Spontaneous resolution rare

• Mortality increases after 24-48hrs

• Recurrence rate 5-8%• Higher if pathologic lead point• Less after surgery

Intussusception

• Take home points• Be suspicious of this diagnosis• Add it to your differential for lethargy

Volvulus

• Definition• Closed loop obstruction from twist in GIT from

a predisposing embryonic malrotation

• Epidemiology• Malrotation in 1/500 births• 70% experience volvulus• 2/3 of volvulus occurs by 1mo, 75% by 12mo

Volvulus

• Clinical Picture• Bilious emesis (80-100%)• Abdominal pain

• Peritonitis, shock as ischemia progresses• Necrosis can occur within 2hrs

• Feeding intolerance• Normal abdominal examination in 50%

• Since obstruction is usually high and proximal

Volvulus

• Differential Diagnosis• Small bowel obstruction (most are duodenal)

• Web• Stenosis• Atresia• Hernia

• Large bowel obstruction• Adynamic ileus

Volvulus

• Diagnosis• Plain films

• Variable: normal, SBO, LBO

• UGI• Taper/beak of contrast; Ligament of Trietz

abnormally located

• Malrotation can be suspected by U/S• Abnormal position of superior mesenteric vessels

• Malrotation work-up includes contrast enema• Cecum most common element of malrotation

Volvulus

• Management• Volvulus is a surgical emergency

• Take home point• Bilious emesis is volvulus until proven

otherwise

Appendicitis

• Definition• Appendicitis!

• Pathophysiology• Luminal obstruction vs mucosal ulceration

leads to inflammation or infection, edema and ischemia, then perforation, and subsequent peritonitis or abcess formation

Appendicitis

• Epidemiology• Incidence: 2/10,000 <4yo, 25/10,000 teens

• 1/10th incidence where high fiber diets

• Seasonal variability• Family history has RR of 3.5-10• Lifetime risk: 9% males, 7% females• 1-8% of ED Diagnoses of abdominal pain• Perforation: ~90% <3yo, <15% teens

Appendicitis

• Pathophysiology Encore• Appendix’s function - ? immunologic• Funnel shaped tube in infants

• Less likely to obstruct

• Maximal lymphoid hyperplasia in teens• Corresponds to peak incidence

• More mobile than in adult population• Hence more confounding clinical features

Appendicitis

• Clinical Picture – age dependent• Classic progression

• Periumbilical pain – N/V – RLQ pain• Occurs in 50% of adult cases, less in pediatric• Hence initial misdiagnosis 28-57%

• Neonates (~80% mortality)• Features are nonspecific

• Lethargy, irritability (20%)• Vomiting (60%)

• Abdominal distention (60-90%)• Mass (20-40%), abdominal cellulitis, dyspnea, shock

Appendicitis

• Clinical Picture• Infants – misdiagnosis 70-100%

• Commonest features• Vomiting (85-90%)• Pain and diffuse tenderness (35-92%)• Fever (50%)• Diarrhea (18-46%)

• Right hip complaints (3-23%)

Appendicitis

• Clinical Picture• Pre-schoolers – misdiagnosis 19-57%

• Vomiting often noted before pain• RLQ pain and tenderness become more prominent

than diffuse pain• Rebound and guarding evident

Appendicitis

• Clinical Picture• School-aged kids – misdiagnosis 12-28%

• Clearer communication of features• Non-classic features

• Vomiting prior to pain (18%)• Dysuria (up to 20%)• Constipation or diarrhea (~20% each)

• Adolescents – misdiagnosis <15%• Challenge in female population

• Pelvic pathology

Appendicitis

• Clinical Picture• Rebound most sensitive (83%) and (with percussion)

most specific (82%) for peritonitis (adult data)• No pediatric data on psoas, obturator, cough, and

Rovsing’s signs, or cat’s-eye symptoms• Rectal exam adds little outside of infant population

(except legal fodder in the USA)• Alvarado’s MANTRELS scoring system not accurate

in pediatrics

Appendicitis

• Differential Diagnosis• GE• Mesenteric adenitis• Tubo-overian pathology• UTI• Intussusception• Meckel’s diverticulitis• Testicular pathology• RLL pneumonia• Sepsis

Appendicitis

• Diagnostic Studies – limited role, nothing 100%• WBC

• Non-specific, non-sensitive – might alter suspiscions >48hrs

• Neutrophilia• More sensitive at <24hrs of symptoms than WBC

• CRP• Studies suggest more sensitive than WBC if perforation

• Beta-HCG• The only “mandatory” test, in teen girls

• U/A• 7-25% abnormal due to appendicitis

Appendicitis

• Radiology• Plain films

• Fecolith in 13-20% (1-2% without appendicitis)• Role if suspect free air, obstruction, or mass

• WBC scans• Variable results, therefore not recommended

• U/S• Sensitivity 80-92%, specificity 86-98%• Don’t visualize appendix in 10% with appendicitis

• CT (+/- contrast)• Sensitivity 87-100%, specificity 83-97%• Sometimes used to follow-up negative U/S

Appendicitis

• Management• Controversy over use of “prophylactic” antibiotics in

uncomplicated appendicitis• Often decision deferred to surgeon

• Fluid resuscitation and broad-spectrum antibiotics if suspect perforation

• Observation in equivocal cases

• Rare cases sound like recurrent appendicitis• Think IBD!

Appendicitis

• Take home points• Young children and adolescent girls are

hardest to diagnose• In the CT vs U/S debate

• U/S is often better in thin patients• CT is often better in fat patients • Utilization is centre and resource dependent

Incarcerated Inguinal Hernia

• Hernias• Indirect in >99% of pediatric cases

• Epidemiology• 1-2 inguinal hernias/100 live births

• Up to 30% in prems• Most right-sided; 10% bilateral (more in girls!)

• 10% complicated by incarceration• Commonest cause of obstruction in infants• 70% of these by 1yo

• 4-6:1 male:female

Incarcerated Inguinal Hernia

• Hernia vs Hydrocele

Incarcerated Inguinal Hernia

• Clinical picture• Bulge in groin• Progresses to off-colour, firm, tender mass• Symptoms and signs of intestinal obstruction• Unlike hydrocele, can feel neck of mass at

distal inguinal ring• Transillumination suggests hydrocele

Incarcerated Inguinal Hernia

• Differential Diagnosis• Hydrocele• Undescended or retractile testes• Lymphadenopathy

• Management• Manual reduction

• 95% success, less if ovary entrapped

• Surgery for all hernias• Emergent if not reducible

Case• 6 wk old boy with 2/52 forceful non-bilious vomiting

after feeds. Responsive, feeds hungrily.• 36.5, 150, 28, 90/50. CRT>2secs, AF depressed.

Abdo soft, non-tender.Skin turgor diminished. Na 125, K 3.8, Cl 85, CO2 30.

• Which of these is correct?• A) Congenital adrenal hyperplasia• B) Hypertrophic pyloric stenosis• C) Malrotation with volvulus• D) Milk allergy

Pyloric Stenosis

• Differential Diagnosis• GERD• Milk allergy• Infection (e.g. GE, sepsis)• Hydrocephalus• Metabolic disorders• Surgical causes

• Malrotation, webs, duplications, atresias, annular pancreas

Pyloric Stenosis

• Definition• Idiopathic hypertrophy of pyloric muscle

• Epidemiology• 1-4/1,000 live births• 2-5:1 male:female• Family history; genetic syndromes

• Etiology• Poor innervation of pyloric musculature• Presumed molecular cause (e.g. NO, GrwthF)

Pyloric Stenosis

• Clinical Picture• Presents 1-10wks• Peak incidence 3-5wks• Progressive nonbilious projectile emesis post

feeds• Earlier onset but slower progression in prems

• Hungry until too dehydrated

Pyloric Stenosis

• Physical Examination• Up to 90% specific, but <50% sensitive

• RUQ “Olive”• 60-80% found “by an experienced examiner”

• LUQ peristalsis• Saw it at last!

Pyloric Stenosis

• Diagnosis• Hypochloremic metabolic alkalosis

• +/- hypokalemia• +/- hyponatremia• +/- paradoxical aciduria

• Imaging• U/S vs UGI

• Both >90% sensitive

Pyloric Stenosis

• Management• Correct hydration and electrolyte disorders

• Focus on chloride replacement else alkalosis resistant to therapy

• Severe alkalosis and hyponatremia need slow correction to decrease risk of pontine myelinolysis

Pyloric Stenosis

• Management• Pyloromyotomy commonest choice• Natural history

• spontaneous resolution over weeks to months• Controversial option – long-term TPN• Atropine has reportedly hastened this resolution

• No good trials to date for this option

• No long-term differences in gastric emptying

Pyloric Stenosis

• Take home point• This is a medical, not a surgical emergency

DEHYDRATION IN PEDIATRICS

Kevin Levere

Jan 16, 2003

Definition

• Loss of H2O and salt from ECF• Isonatremic – 70-80%• Hyponatremic – 10-20%• Hypernatremic – 10-20%

Question

• Which of the following is associated with greater fluid loss per KG for a given severity of clinical manifestations of dehydration?• A) Fever• B) Hypernatremia• C) Male gender• D) Obesity• E) Older age

Etiology

• Infectious gastroenteritis• Commonest by far (>1 episode/yr/pediatric

patient)• Compounded by malnutrition

• Pyloric stenosis

• DKA or DI

• Starvation

Pathophysiology

• Lean Body Mass (LBM) is 65-80% H2O• Infants

• 25% ECF, 45% ICF

• Children by 5yo (equivalent to adults)• 20% ECF, 50% ICF

• Plasma is within ECF• 6% of LBM at all ages

• Total Body Weight is ~10% fat in older children and adults

• So Total Body Water is ~60% of Total Body Mass

Pathophysiology

• Renal and hormonal homeostasis• Preserve H2O, regulate Na

• Losses• Insensible, GI, renal

• Intake• Deficient• Hypoosmolar vs hyperosmolar

Clinical Picture

• Isonatremic• Isoosmolar state, no fluid compartment gradient• ICF preserved

• Hyponatremic• Further H2O loss as shifts from ECF into ICF• Exacerbates circulatory depletion, enhancing signs

• Hypernatremic• ECF preserved by shifts from ICF• Attenuates circulatory signs; doughy skin; lethargy

Clinical Picture

• Signs arise at 3% - Lancet, 1989• Clinical overestimation is common

• Clinical Physician estimates ~70% sensitive• Pediatr Emerg Care, 1997

• Tachycardia presents early• Not specific, and surprisingly not sensitive

• Decreased U/O occurs by 5%• Therefore poor predictor of severity

Clinical Findings of Dehydration (Adapted from WHO)Signs and Symptoms

Degree of Impairment

None or Mild Moderate Severe

General Condition

Infants Thirsty; alert; restless Lethargic or drowsy Limp; cold, cyanotic extremities; may be comatose

Older Children Thirsty; alert; restless Alert; postural dizziness

Apprehensive; cold, cyanotic extremities;  muscle cramps

Quality of Radial Pulse

Normal Thready or weak Feeble or impalpable

Quality of Respiration

Normal Deep Deep and rapid

Skin Elasticity Pinch retracts immediately

Pinch retracts slowly Pinch retracts very slowly (>2 sec)

Eyes Normal Sunken Very sunken

Tears Present Absent Absent

Mucous Membranes Moist Dry Very dry

Urine Output (by report of parent)

Normal Reduced None passed in many hours

Clinical Picture

• Validity and Reliability of Clinical Signs• Pediatrics, 1997

• Individual findings lack sensitivity• Included capillary refill time and tachycardia

• For dehydration of 5% or more• 3 or more findings 87% sensitive and 82% specific

• For dehydration of 10% or more• 7 or more findings 82% sensitive and 90% specific

Clinical Picture

• Validity and Reliability of Clinical Signs• Pediatrics, 1997

• Factors independently associated with dehydration (in logistic regression)

• Capillary refill >2 sec• Dry mucous membranes• Absent tears• Ill appearance

• For dehydration of 5% or more• 2 of 4 findings 79% sensitive and 87% specific

lablablab

• Common lab tests• Recommended in moderate to severe cases• Not sensitive or specific for degree of dehydration

• Na• Reflects relative losses of H2O and salts

• K• Low if lost in diarrhea or if alkalotic (vomiting)• High if renal compromise or acidosis

• HCO3• Lost in renal compromise or stool losses• Might replace if renal compromise and acidosis

More Labs

• Cl• Alkalosis resistant to therapy if not replaced

• Urea• Estimates degree of renal compromise

• Ca• Reduced if phosphate retention or hypernatremia• Rarely significant

• U/A• Specific gravity

Fluid Therapy

• “Coconut Water!”

Fluid Therapy

• 150 years of History• Parenteral concoctions assayed with varying success• Early theories were that ICF was dehydrated because of

excess K in diarrhea – 1915• Principle of cellular homeostatic limits led to maintenance

and deficit therapy, IV therapy as mainstay – NEJM, 1953• Greater success after realized ECF dehydration was the

greater contributor, with potassium restored during maintenance – Pediatrics, 1956

• Affect of net Na and glucose relationship on reabsorption appreciated, oral rehydration therapy (ORT) success realized – NEJM, 1968

• Free water and concentrated ORT solutions found to have increased mortality, hence reduced osmolar questions

Oral Rehydration Therapy (ORT)

 

OsmolesmOsm/L

Glucosemmol/L

NamEq/L

ClmEq/L

HCO3mEq/L

KmEq/L

WHO formulation 330 110 90 80 30 20

Pedialyte 270 140 45 35 30 20

AJ 730(CHOs)

690 5 x x 32

Sports drink 330(CHOs)

255 20  x 3 3

D5W /

0.45% saline 454 300 77 77 0 0

Fluid Therapy

• Rice-based oral solution with WHO’s electrolyte balance:• More effective than the WHO ORS in reducing

stool output in people with cholera. This effect was not apparent in infants and children with non-cholera diarrhea

• Cochrane Database Syst Rev, 2000

• Starch and small proteins as Na cotransport, with less osmolar effect on diarrhea

Fluid Therapy

• Reduced osmolarity solutions• ~270mOsm/L, Na ~60-75mEq/L

• 33% fewer IV infusions• Decreased stool losses• Less vomiting• No additional risk of hyponatremia

• Cochrane Database Syst Rev, 2001

• Only significant incidence of hyponatremia found in adults with cholera, but none were symptomatic

• CHOICE Study Group, 1999

Fluid Therapy

• For severe dehydration (~10% or 100ml/kg)• Rapid intravenous ECF restoration

• Over 2-6hrs• Improves renal perfusion and function• Reduces vomiting• Earlier resumption of oral intake, including food• Food and milk might shorten duration of diarrhea

despite transient lactase deficiency• Avoid fatty food and simple sugars

Fluid Therapy

• For severe dehydration• IV restoration followed by ORT to replace K,

restore ICF status• For maintenance and ongoing losses

• Deficit calculations in isonatremic situations not found to improve care

Fluid Therapy

• For hypernatremic dehydration• Restore circulation (treat shock)• Deliver maintenance and deficit over 48hrs to

avoid cerebral sequelae• Hyperglycemia often accompanies

hypernatremia• Slower restoration increases risk of

hypocalcemia, especially in young infants

Fluid Therapy

• For mild to moderate dehydration (5-9%)• ORT is 90-95% effective• Oral fluid challenge only if moderate (i.e.

borderline for IV therapy)

Fluid Therapy• NG vs IV

• Pediatrics, 2002

• 93 patients prospectively enrolled• Moderate dehydration, failed oral fluid challenge

• Randomized to rapid NG vs IV hydration• 50ml/kg over 3hrs of Pedialyte vs NS

• Both found to be safe and efficacious• Significantly more emesis in NG group• Significantly less cost• Improved acidosis in NG vs worsened in IV

• Not clinically significant; no other lab variance

Fluid Therapy

• NG vs IV vs ORT• Failure meant getting admitted

• 1 of the 47 NG arm failed (~2%)• 2 of the 46 IV arm failed (~5%)

• ORT is 90-95% successful, and cheapest• Still the first line therapy in moderate dehydration• Oral fluid challenge poorly defined

• Only useful if borderline for IV/NG intervention

Medications

• Antidiarrheals• None recommended in younger children

• AAP Guidelines, 1996

• Loperamide and other opiate derivatives• Limited efficacy vs high rate of side effects

• Anticholinergics• No clear efficacy, high rate of side effects

• Bismuth compounds• Limited benefit vs lack of data on rates of side effects

• Adsorbents and lactobacillus• No clear benefit, though little concern of toxicity

Medications

• Antiemetics• Concensus opinion – they aren’t needed

• Little efficacy vs side effects• CHMC Guidelines, 1997

• Ondansetron effective in reducing emesis from GE, increasing success of ORT

• Ann Emerg Med, 2002

Medications

• Zinc• Role in the malnourished

• Reduced duration and severity of diarrhea• Independent of Vitamin A supplementation

• Pediatrics, 2002

• Regular Zn supplementation reduced frequency of diarrheal illnesses (and pneumonias)

• J Pediatrics, 2002

Ad(sub)mission

• Significant dehydration and…• Persistent and/or prolonged ongoing losses• Care-givers far from help, or unable to help

• Ill despite fluids – consider other diagnoses• i.e. back to the beginning

Thank you

Liver Disease

• Chronic manifestations• Hepatomegaly• Jaundice• Pruritis• Spider hemangiomas• Palmar erythema• Ascites• Portal hypertension• Xanthomas• Encephalopathy• Renal dysfunction• Endocrinopathies (rarer than in adults)

Liver Disease

• Hepatomegaly• Inflammation

• Infections, toxins, autoimmune disease

• Storage• Glycogen, lipids, Wilson’s, iron

• Infiltration• Primary and secondary neoplasia

• Congestion• CHF, veno-occlusive disease, post-hepatic

obstruction

Liver disease

• Jaundice (aka icterus)• In neonates at 80-100micromol/L

• Occurs at lower levels in children and adults

• Unconjugated bilirubin• Lipid soluble; unbound crosses BBB

• Conjugated bilirubin• Unbound is renally excreted• Increased if >20% total bilirubin

Approach to Neonatal Jaundice

Unconjugated Hyperbilirubinemia

• Hemolytic disease• Hereditary or acquired• Sepsis, UTI

• Decreased hepatic conjugation• Decrease hepatic intake

• Breast milk, hypothyroidism

• Decreased hepatocellular function• Hepatitis• Physiologic, Crigler-Najjar, Gilbert

• Enterohepatic recirculation

Neonatal Cholestasis

• Infectious• Sepsis, hepatitis, TORCH

• Toxin• TPN, sepsis, drugs

• Metabolic• Galactosemia, tyrosinemia, alpha-1 AT defiency, CF

• Intrahepatic diseases• Alagille, bile duct paucity, congenital fibrosis

• Extrahepatic biliary diseases

Extrahepatic biliary diseases

• Biliary atresia• Commonest cause of liver failure in pediatrics

• Sclerosing cholangitis• Association with IBD

• Bile duct stenosis• Choledochal-pancreaticoductal junction anomaly• Spontaneous perforation of the bile duct• Choledochal cyst• Mass (neoplasia, stone)• Bile/mucous plug ("inspissated bile")

Liver Disease

• Evaluation• ALT most specific for hepatocellular toxicity

• AST might have role in evaluation of EtOH etiology, not a typical pediatric concern

• 5’ nucleotidase and GGT sensitive markers of biliary obstruction or inflammation

• Alk phosphatase not so specific

• Bilirubin, albumin, INR, glucose, NH3• Markers of function

Liver Disease

• Evaluation• Metabolic survey

• Electrolytes, Ca, Mg, PO4• Renal function, U/A (ketones)• Glucose, U reducing substances• NH3, lactate, urate• Blood gas• Serum AA’s, urine AA’s and OA’s

Liver Disease

• Evaluation• Imaging

• U/S, CT, MRI, ERCP• Masses, gall bladder, biliary tree

• Nuclear med studies now very rarely used

• Liver biopsy