Gastrointestinal
Cleft Lip and Palate
Incidence
Cleft lip 1:750
Cleft palate 1:2,500
Frequently occurs together
Male > Females
Familiar
Asians
Left > Right – may be bilateral
Treatment
Feeding – soft nipple, large opening, squeezable bottle
Surgery
Cleft lip by 3 months – Z-plasty
Cleft palate – individualized – depending on extent of the defect
Usually before 1 year of age
Team: pediatrician, plastic surgeon, otolaryngologist, pediatric dentist,
prosthodontist, orthodontist, speech therapist
Problems
Recurrent otitis media
Speech problems
Dental Caries
Sugar exposure
Sucrose by-product is glucan that help bacteria adhere to enamel
Mutans streptococcus – adheres to enamel – produces acid
Lactobacilli – produces acid
Prevention – fluoride
Incidence is decreasing – but still in 50% of children
Complications of dental cavities – dental abscess
Treatment of dental cavities – dental referral
Medication
Acetaminophen or Ibuprofen
Antibiotics oral for abscesses or cellulitis
Penicillin
Alternative Clindamycin
Prevention
Brushing teeth 2 or more time a day with fluoride toothpaste
Decreasing sugar ingestion
Oral fluoride
Dental sealants
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Oropharyngeal Candidiasis
White plaques on tongue and buccal mucosa that are difficult to remove from the areas involved. When
removed they have an inflamed base
Seen in newborn, after antibiotic use, after steroid use
Treatment – Nystatin oral suspension
Complications and spread if patient is immunocompromised
Herpetic Gingivostomatitis
Small vesicles that develop into ulcers in anterior part of mouth
Child may have fever, malaise, and refuse to drink
Treatment
Acetaminophen or Ibuprofen
Acyclovir or Valacyclovir
Recurrent Herpes Labialis (cold sores)
Limited to the lip
Treatment – antiviral therapy not recommended, acetaminophen or Ibuprofen
Ankyloglossia (“tongue-tie”)
Short lingual frenulum
Treatment – cut frenulum if child unable to suck or has trouble pronouncing certain words
Parotid Glands
Swelling
Mumps – redness of Stensen’s duct
Recurrent Parotitis – cause unknown
Suppurative Parotitis – usually Staphylococcus aureus
Fever, swelling, tender, and painful gland
Treatment – appropriate antibiotics
Esophageal Atresia
Incidence 1:4000 - over 90% have tracheoesophageal fistula
May be associated with VATER/VATECRL
(Vertebral, Anorectal, Trachea, Esophagus, Cardiac, Renal, Radial, and Limb abnormalities)
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Tracheoesophageal Fistula
Clinical
May have had polyhydramnios
Presents with excessive mucous – in mouth and nose
coughing, cyanosis, and respiratory distress
Feeding make symptoms worse and may cause aspiration pneumonitis
Diagnosis
Unable to pass a feeding tube into the stomach
It coils in the esophagus. May also have an air distended stomach
Treatment
Prone position
Esophageal suctioning from the blind pouch
Avoid intubation
Surgery
Ligation of the fistula and end to end anastomosis of the esophagus
Complications
Stricture of surgical site
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Gastroesophageal Reflux Disease
Gastroesophageal Reflux – normal in newborn, have no weight loss, no respiratory symptoms, no esophagitis,
and no esophageal symptoms
Begins early in life – peaks at 4 months, and is usually gone by 12 months
Gastroesophageal Reflux Disease – GERD
Due to excessive relaxation of the Lower Esophageal Sphincter (LES)
May be autosomal dominant – on chromosome 13 q 14 and chromosome 9
Clinical
Esophagitis – irritability, arching, choking, gagging, feeding aversion
Failure to thrive
Abdominal and chest pain
Neck contortions (Sandifer syndrome)
Respiratory
Apnea – obstructive
Stridor
Asthma
Laryngitis
Sinusitis
Diagnosis
Good history and physical – Infant Gastroesophageal Reflux Questionnaire (I-GERD)
Upper GI – contrast study
Extended esophageal PH monitoring
Endoscopic – permits diagnosis of esophagitis, stricture, or Barrett esophagitis
Radionucleotide – demonstrates aspiration and delayed gastric emptying
Laryngotracheobronchoscopy – demonstrates bronchial lipid-laden macrophages
Treatment
Feeding – Infant – thicken 15 cc of cereal per ounce of formula
Older children avoid tomatoes, chocolate, mint, juices, carbonated and caffeinated
drinks, alcohol
Positioning – seated – worse – increased intra-abdominal pressure
Medication
Antacids
Histamine-2 receptor antagonist (H2RAs) – cimetidine, ranitidine
Proton pump inhibitors (PPI’s) – omeprazole
Surgery
Fundoplication – Nissen
Foreign Body in the Esophagus
Usually occurs between 6 months and 3 years of age
Coins most common objects
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Lodge at cricopharyngeal muscle, aortic arch level, or at the gastroesophageal junction
Clinical
30% no symptoms
Choking, gagging and coughing initially
Followed by excessive salivation, dysphagia, food refusal, emesis, or pain in neck, throat, or
sternal notch areas
Respiratory symptoms - stridor, wheezing, cyanosis and/or dyspnea
Diagnosis
Anteroposterior radiograph of neck, chest, and abdomen
Lateral views of neck and chest
Treatment
Endoscopic visualization and removal of the object
Removal by using a Foley catheter under fluoroscopy
Urgency in removing button batteries – will erode esophagus
Hypertrophic Pyloric Stenosis
Occurrence 3 per 1000 infants
Most common in Caucasians
Higher incident if a parent had it – especially the mother (20% of Male, 10% of female)
Higher incident if B or O blood type
Increased if Erythromycin was previously used
Clinical
Nonbilious vomiting – progressing to projectile vomiting
Occurs immediately after feeding – child wants to eat again
Begin after 1st weeks, (usually after 3rd weeks), and before 5 months of age
Develops dehydration, hypochloremic metabolic alkalosis
Total body loss of potassium
Malnutrition
5% develop jaundice
Diagnosis
Palpable pyloric mass – firm, movable 2 cm in length – “olive”
Located above and to the right of the umbilicus in the mid-epigastric area
Easiest to feel right after the child vomits
Visible gastric peristaltic wave
Ultrasound – confirms the diagnosis – sensitivity of 95%
Treatment
Initial – correct fluids and electrolytes
D5 in ½ NSS with 40 meg KCL per liter
Must correct Alkalosis before surgery to prevent post-operative apnea
Surgery – Ramstedt pyloromyotomy – done laparoscopically
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Duodenal Obstruction
May be due to annular pancreas or from Ladd band
A membranous atresia is the most common form
Down syndrome occurs in 20-30% of duodenal atresia
Clinical
Bilious vomiting without abdominal distension
Usually observed in the first day of life
Polyhydramnios is present in ½
Jaundice in 1/3
Diagnosis
Abdominal radiograph – “Double-bubble” sign
Echocardiogram – to rule out other significant abnormalities
Radiograph of chest and spine – to rule out other significant abnormalities
Treatment
Nasogastric decompression
I.V. fluid
Surgery – duodenostomy
Malrotation
Clinical
Most present in first year of life
Acute or chronic obstruction (recurrent abdominal pain, vomiting, or both)
Infants in first week of life
Bilious vomiting
Diagnosis
Ultrasound
Upper GI study – malposition of the Ligament of Treitz
Treatment
Emergency surgery to prevent ischemia to the bowel
Meckl Diverticulum
Remnant of the embryonic yolk sac (omphalomesenteric duct)
Occurs in 2-3% of population
3-6 cm outpouching, 50-75 cm from ileocecal valve
Clinical
Usually in first 2 years of life, but common anytime in first decade
Intermittent painless rectal bleeding (brick colored or currant jelly)
Bowel obstruction – Meckel acts as a lead point for intussusceptions
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Diverticulitis
Diagnosis
Meckel radionuclide scan – I.V. technetium – 99m pertechnetate
Enhanced with cimetidine, glucagon, or gastin
Enhanced sensitivity 85% specifically 95%
Treatment
Surgical excision
Functional Constipation
Clinical
Begins after the neonatal period
Caused by passage of painful bowel movement
Voluntary withholding of feces
Stool gets harder (less water) and larger
That produces a painful bowel movement
Encopresis (stool soiling of diaper or underwear)
Examination
Mass in left lower quadrant
Rectal – stool just inside rectum, large stool in rectal vault
Treatment
Parent education
Establish bowel habits – sit on toilet for 5-10 minutes after each meal
Polyethylene glycol-miralax
Congenital Aganglionic Megacolon (Hirschsprung’s Disease)
Caused by absence of ganglion cells in the bowel wall beginning in the internal anal sphincter and
extending proximally
Incidence 1 in 5,000
Male : female = 4 : 1
Familial incidence in long segment diseases
Entire colon involved in only 10%
Clinical
Not pass stools in first 24 (normal 97% of infants)
or 48 hours (99% of infants)
Chronic constipation
Failure to thrive
Hypoproteinemia
Abdominal distention
May develop an enterocolitis (Toxic Megacolon)
(Clostridium Difficile, Staphylococcus aureus, anaerobes, coliforms)
Examination
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Large fecal mass in left abdomen
Rectum is empty of feces
Diagnosis
Rectal suction biopsy
Barium Enema – shows a transition zone – after several weeks
Treatment
Surgery
Swenson
Duhamel
Boley
Intussusception
Is a telescoping of one portion of the gastrointestinal tract into an adjacent segment
It is the most common cause of intestinal obstruction between 3 month and 6 years of age.
80% occur before 2 years of age
Incidence 1-4/1000 live births
Male : female = 4 : 1
Increase incidence
Spring and autumn (adenovirus season)
Otitis Medius
Gastroenteritis
Henoch-Schönlein Purpura
Upper respiratory infection
Rotavirus vaccine
Cystic fibrosis
Symptoms
Paroxysmal colicky pain
Lethargic
Vomiting
Currant Jelly stools – late
Mass – sausage shaped – usually in right upper quadrant
Complication
Necrotic bowel
Perforation
Peritonitis
Recurrent intussusceptions
Diagnosis
History and Physical
Radiograph – density in the area of the Intussusception
Ultrasound – tubular mass in longitudinal view - doughnut in transverse view
Treatment
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Reduction by “Air” enema
Peptic Ulcer Disease
Associated with
Sepsis
Head or body trauma (Cushing ulcers)
Burns (Curling ulcers)
Steroid
Nonsteroidal anti-inflammatory drugs
No associated disease
Usually due to Helicobacter pylori
May lead to gastric adenodorcinoma or mucosal associated lymphoid tissue lymphoma
(MALT)
Clinical
Poorly localized abdominal pain – periumbilical
Gastrointestinal bleeding – hematemesis and/or melena
Pancreatitis
Recurrent vomiting
Slow growth
Epigastric pain in children over 6 years of age
Relieved by food – only in 1/3
Iron deficiency anemia
Diagnosis
Endoscopy
Testing for H. pylori – detection of H. pylori antigen in the stool
Treatment
2 antibiotics
Proton pump inhibitor
Zollinger-Ellison Syndrome
Rare – islet cell tumor or hypertrophy
Causes multiple recurrent duodenal and jejuna ulcers
Diarrhea
Treatment
Removal of tumor
Proton Pump Inhibitors
Lansoprazole
Omeprazole
H2 Receptor Antagonist
Famotide
Ranitidine
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Inflammatory Bowel Disease
Crohn Disease
Ulcerative colitis
Both occur more often in certain families
Chromosome 6, NOD2
Environmental factors are important – use of antibiotics
Extraintestinal manifestations – more common with Crohn disease
Growth retardation 15-35% of Crohn Disease at diagnosis
Colitis of either associated with – joint (arthritis), skin (Erythema nodosum, pyoderma
gangrenosum), eye (uveitis), mouth, and hepatobiliary disease
Chronic Ulcerative Colitis
Begin in rectum and extends proximally – limited to colon
Incidence has remained constant
Males more frequently involved than females
Only mucosa is involved
Clinical – usually has episodes of exacerbation
Blood in stools
Diarrhea
Tenesmus
Urgency
Cramping abdominal pain
Nocturnal bowel movements
Fulminant colitis
Fever, severe anemia, hypoalbuminaemia, leukocytosis, > 5 bloody stools per
day
Extraintestinal manifestations
Pyoderma gangrenosum
Sclerosing cholangitis
Chronic active hepatitis
Ankylosing spondylitis
Iron Deficiency anemia or anemia of chronic disease
Secondary amenorrhea
Complication
Colon cancer begins after 8-10 years of the disease and increases by 0.5 to 1% / year
thereafter
Diagnosis
Endoscopic and histologic examination of the colon
Cryptitis, crypt abscesses, separation of crypts by inflammatory cells, edema,
branching of crypts
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Treatment
Sulfasalazine 50-75 mg/Kg/day divided in 4 doses
Prednisone 1-2 mg/Kg/day taper to every other day
Immunomodulators – Azathioprine or 6-mercaptopurine
Colectomy – for intractable disease
Crohn Disease (Regional Enteritis)
May involve entire gastrointestinal tract from mouth to anus
Eccentric and segmental with skip areas
Transmural
Incidence is increasing
Clinical
Appear ill
Weight loss
Malnourished
Cramping abdominal pain
Diarrhea
May have right lower abdominal pain
Fever, malaise, easy fatigability
Growth failure with delayed bone maturation and delayed sexual development and secondary
amenorrhea
Perianal disease (tags, fistula, abscess)
Small bowel obstruction (strictures)
Enteroenteric or enterocolonic fistulas (results in malabsorption)
Enterovaginal fistulas
Perianal fistulas
Oral aphthous ulcers
Erythema nodosum
Digital clubbing
Renal stones and gall stones
Cancer risk increases after 10 years of the disease
Diagnosis
Anemia
Elevated sed. rate
Elevated platelet count
Low serum albumen
Stool - α1 antitrypsin is elevated
Anti-Saccharomyces cerevisiae antibodies in 55%
Upper Gastrointestinal Contrast Examination with a small bowel follow-through
Colonoscopy – if colon is involved
Treatment
Mesalamine (40-60 mg/Kg/day)
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Sulfasalazine – for mild Crohn colitis – not help small bowel disease
Prednisone 1-2 mg/Kg/day – taper to alternate day dose
Immunomodulators – Azathioprine or 6-mercaptopurine
Infliximab
Metronidazole
Nutritional therapy – enteral – High – caloric
Surgery – only special situations – perforation, stricture, intractable bleeding, abscess
Psychological consult and Social support
Eosinophilia Gastroenteritis
Eosinophils infiltration of stomach and small intestine, mucosa, muscularis, or serosa
Peripheral eosinophilia
IgE is elevated
Clinical
Nausea, vomiting, diarrhea, abdominal pain, gastrointestinal bleeding, protein losing
enteropathy, malabsorption
Treatment
Elimination diets
Cromolyn
Corticosteroids
Malabsorptive Disorders
Symptoms – abdominal distension, pale, foul-smelling bulky stools, muscle wasting, decrease
subcutaneous fat, poor weight gain or weight loss, growth retardation, lethargy, edema,
clubbing, depigmentation of skin and hair, eczema, bleeding, follicular hyperkeratosis, cheilosis,
stomatitis, glossitis, and diffuse abdominal pain
Conditions that cause malabsorption
Cystic fibrosis
Chronic protein-caloric malnutrition
Biliary Atresia
Massive resection
Stagnant loop syndrome
Short bowel
Giardiasis
Celiac Disease
Dietary protein intolerance (milk, protein)
Shwachman’s-Diamond syndrome
Chronic pancreatitis
Pearson Syndrome
Other cholestatic state – Alagille Syndrome, and familial neonatal hepatitis
Congenital short gut
Immunodeficiency
Tropical Sprue
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Idiopathic diffuse mucosal lesions
Abatalipoproteinemia
Enterokinase deficiency
Amino acid transport defects (cystinuria, Hartnup disease, methionine malabsorption, blue
diaper syndrome)
Disaccharidase deficiencies (sucrose-isomaltose, lactose)
Glucose-galactose malabsorption
Glucoamylase deficiency
Vit B12 malabsorption
Folic acid malabsorption
Chloride-losing diarrhea
Congenital sodium diarrhea
Acrodermatitis enteropathica (zinc)
Menkes Syndrome (copper)
Vit D-dependant rickets
Primary hypomagnesaemia
Drug induced
Sulfasalazine (folic acid malabsorption)
Cholestyramine (calcium, fat malabsorption)
Phenytoin (calcium malabsorption)
Specific Pancreatic enzyme deficiencies
Laboratory
Stool for fat – microscopic examination
72 hour quantitive test (> 7% is abnormal)
Sweat test
ELISA for Elastase 1
Trypsinogen
CBC
Albumin – serum
IgG, IgA, IgM (Immunodeficiencies)
Clintest of stool
Stool Ph. (Abnormal < 5.6)
“stool osmotic gap”
Hydrogen breath test
Stool – α1 antitrypsin, (protein losing enteropathy)
Iron
Folic Acid
Calcium
Zinc
Magnesium
Vit A, B12, D2, and K (prothrombin time)
D-xylose (proximal small bowel)
Giardiasis antigen test
HIV tests
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Small bowel biopsy
Imaging studies
Plain film of abdomen
Barium contrast studies
Ultrasound
Retrograde studies of pancreatic and Biliary tree – rare cases
Intestinal Infections
Giardiasis is the most common infection, cause of chronic malabsorption, obtained from family members or at
daycare
Other microorganism – rotavirus, campylobacter, shigella, salmonella, cryptosporidiosis, coccidiosis
Immunodeficiency
AIDS
T and B-cell immune deficiencies
Deficiency of Neutrophils – neutropenia
Stagnant Loop Syndrome
Exceeding high number of bacteria in loop areas
Chronic incomplete bowel obstruction – distention, pain, vomiting
Treatment – Metronidazole
Short Bowel Syndrome
Loss of at least 50% of small bowel
Clinical
Malabsorption, diarrhea, failure to thrive, dehydration, hyponatremia, hypokalemia, acidosis
Treatment
Parenteral nutrition
Enteral feeding begin at 1-2 mL/hour then increase
Breast milk is best, amino acid-based formula is acceptable
Bacterial overgrowth may be treated with Metronidazole
Gluten-sensitive Enteropathy (Celiac disease, Celiac Sprue)
Proximal small bowel is damaged by dietary exposure to gluten
Affects people of northern European ancestry mainly
Presents at 6 mos to 2 years
Occurs after long-term dietary exposure to gluten (wheat, rye, and barley)
Villus atrophy, crypt hyperplasia, and damage to the surface epithelium
Decrease in absorption and digestive capacity
Pancreatic secretion is decreased
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Clinical
Presentation is variable
Diarrhea, failure to thrive, vomiting, anorexia, clingy, irritable, unhappy, abdominal distension, large
bulky stools, digital clubbing, ataxia and dermatitis herpetiform
Increased incidence in child with IgA deficiency, diabetes mellitus, idiopathic arthritis, thyroiditis,
hypothyroidism, Addison disease, pernicious anemia, alopecia, and Down Syndrome
Laboratory
CBC (for anemia), total protein, albumen, (for hypoproteinemia) Prothrombin for
hypoprothrombinemia, antiendomysial antibodies (IgA & IgG), tissue transglutaminase (tTG)
(IgA & IgG)
Biopsy of small bowel is the gold standard. It shows short, flat villi, deep crypts, irregular vacuolated surface
epithelium with increased number of lymphocytes.
Treatment
Lifelong strict gluten-free diet (no wheat, rye, or barley)
Dietary consult
Iron and vitamin supplementation
Prognosis
Good
If diet not adhered to several types of malignancy many occur
Disaccharidase Deficiencies
Lactase deficiency
Late – onset genetic lactose deficiency is the most common condition associated with reduced disaccharidase
activity
Clinical
Expulsion of hydrogen gas, bloating, cramping abdominal pain, osmotic diarrhea with Low pH (<5.6)
stools, excoriated the buttocks
Tests
Breath hydrogen test after ingestion of the disaccharide (Lactose)
Mucosal biopsy may also be performed
Treatment
Removal of milk and milk products from the diet
A tablet with Lactase may be ingested with meals
Sucrose-Isomaltose Deficiency
Inherited as autosomal recessive trait – on chromosome 3
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Clinical
Bloating, watery diarrhea and failure to thrive
Tests
Breath hydrogen test shows increased hydrogen after ingesting sucrose
Treatment
Avoid sucrose
Give sucraid
Gastroenteritis
Caused by
Viruses
Astroviruses
Celiciviruses
Norovirus
Enteric adenoviruses
Rotavirus
Bacteria
Aeromonas
Bacillus cereus
Campylobacter jejuni
Clostridium perfringens
Clostridium difficile
Escherichia coli
Plesiomonas shigelloides
Salmonella
Shigella
Staphylococcus aureus
Vibrio cholerae 01 and 0139
Vibrio parchaemolytious
Yersinia enterocolitica
Parasites
Balantidum coli
Blastocystis hominis
Cryptosporidium parvum
Cyclospora cayetanensis
Encephalitozoon intestinalis
Entamoeba histolytica
Giardia lamblia
Isospora belli
Strongyloides stercoralis
Trichuris trichiura
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Diarrhea lasting 14 days or more may be due to Giardia lamblia, Cryptosporidum parvum, or Excherichia coli
Viral Gastroenteritis
The main cause of diarrhea in children
Bacterial gastroenteritis
Antibiotics may be administered to selected patients with bacterial gastroenteritis to shorten the clinical
course
Parasitic Gastroenteritis
Giardia Lamblia is the most common parasite causing diarrhea in the United States
Symptoms
Vomiting, diarrhea, dehydration, decrease urine output
Treatment
Fluids with glucose, sodium, and potassium
Usually given orally, occasional given IV if severely dehydrated
Chronic Nonspecific Diarrhea (Toddler’s diarrhea)
Occurs in well appearing toddlers between 1 and 3 years of age
The diarrhea is brown and watery
Frequently follows an episode of viral Gastroenteritis
Treatment
Decrease fluid intake to less than 90 mL/Kg/day
Discontinue fruit juices (Sorbitol or excessive fructose)
Increase fat intake
Stop lactose and sucrose if the child has carbohydrate intolerance or add lactose (Lact Aid)
Consider other causes for malabsorption
Recurrent Abdominal Pain of Childhood – (Chronic Recurrent Abdominal Pain)
Pain is either organic or non-organic (functional)
Non-organic pain cannot be explained on a structural or biochemical basis.
Chronic Recurrent Abdomen Pain of childhood is defined as
Occurring monthly for 3 consecutive months that is severe enough to interrupt routine function
It affects 10-18% of middle and high school students
Accounts for 5% of pediatric office visits
Most common chronic pain entity in children and adolescents
10% of recurrent abdominal pain is organic
Disorder of the entire family and child
In children the gastrointestinal tract is the target system for psychosocial involvement
Onset between 5 and 12 years of age
Organic more likely before 5 years or older than 13 years
The pain is periumbilical and involves a broad area
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Pain is varied and erratic
Well between episodes
Precipitated by stress
Improves with hospitalization
Other symptoms – 20% headaches, 50% pallor, dizziness, anorexia, constipation
Past History
Difficult pregnancy and/or delivery
Setting them up to have
20% had neonatal problems
Vulnerable Child Syndrome
31% had colic – 16% the colic lasted longer than 6 months
Only 9% have regular school attendance
May have GI and GU complaints but have normal GI development
}
Family history
40% oldest child, 30% youngest child, 50% have a family history of functional GI complaints – usually
“spastic colitis” (Irritable Bowel Syndrome)
25% have family history of migraine headaches
Personality
Pseudomature; superachievers, over conscientious, obsessive, compulsive, impulsive, demanding, long
for attention, sensitive to others needs, “the best child”, tolerates criticism poorly, feels rejected
Examination
Look well
Cold extremities, mottled skin, sweaty palms, nails and cuticles bitten, tachycardia, pale,
All sympathomatic response - Epinephrine affect
Differential diagnosis
Constipation
Genitourinary tract – infection or stones
Lactase Deficiency
Irritable Bowel Syndrome
Inflammatory Bowel Disease – Crohn Disease or Ulcerative Colitis
Parasitic Disease
Celiac Disease
Intussusception
Henoch-Schönlein Purpura
Cystic Fibrosis
Cholelithiasis
Depression – withdrawn, irritable, poor school performance
Conversion reaction – rare
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Testing
CBC & Differential, Sed Rate, CRP, Amylase, Lipase, Basic Metabolic panel, Liver function tests, Calcium,
Phosphorous, magnesium, tTG, antiendomycial antibodies, urinalysis, urine culture, lactose
Hydrogen Breath test, stool for ova and parasites and Giardiasis antigen, ultrasound of abdomen
and pelvis
Treatment
Counseling – (sympathetic and supportive)
Demystify what is occurring
Pain is real
Expression of anxiety
Teach them to accept the pain
Desensitize to the pain (slow or rapid)
Send them back to do those things that cause the pain
Rewards for not having the pain
Prognosis
⅓ gets better
⅓ gets other somatic illnesses
⅓ continue to have abdominal pain into adulthood
Acute Appendicitis
Acute appendicitis is the most common condition that requires emergency surgery. The biggest risk is
perforation and that risk is greatest in the 1-4 year old child (70-75%) and lowest in the adolescent (3040%). The highest incidence of appendicitis is in the adolescent. It occurs more often is males and in
the Spring and Fall.
The cause is obstruction of the appendix lumen by a fecalith.
The fecalith may calcify which may be detected on a plain abdominal x-ray
Pathology
3 phases
Luminal obstruction
Venous congestion progresses to mucosal ischemia (Bacteria enter all layers of the appendix)
Necrosis and ulceration (perforation occurs) followed by peritonitis
Clinical
Pain (first periumbilical then right lower quadrant)
Nausea and vomiting
Fever – low grade unless perforation with peritonitis has occurred
Other symptom – frequency and urgency of urination
The symptoms progress from onset to perforation usually requires 36-48 hours
Examination
Child walks slowly, often bent forward, often with a limp. Hyperactive bowel sounds early, followed by
hypoactive sounds. Persistent direct tenderness to palpation and rigidity at McBurney point.
Test for rebound tenderness. Rectal examination should be done.
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Imaging studies
May not want to be done if one is certain of the diagnosis
X-ray of abdomen to look for appendicolith
small bowel distension, obstruction, or a mass
Ultrasound
CT scan – more specific and sensitive – significant radiation
Laboratory
CBC
Urinalysis
Differential Diagnosis
Gastroenteritis – vomiting precedes the pain
Pneumonia of right lower lobe
Henoch-Schönlein Purpura
Hemolytic – uremic syndrome
Inflammatory bowel disease
Torsion of undescended testes
Follicular cyst of the ovary – mid cycle
Pelvic inflammatory disease
Typhlitis – children with malignancy on chemotherapy
Meckel diverticulitis
Treatment
IV fluids
Antibiotics – Ampicillin 100 mg/Kg/24hr, Gentamicin 5mg/Kg/24 hr, Clindamycin 30 mg/Kg/24 hr or
Metronidazole 30 mg/Kg/24 hr
Appendectomy – open or Laparoscopy
Anorectal Malformation
Main concern is bowel control, urinary and sexual function
Male
Perineal fistula – small orifice in the perineum anterior to the center of the external sphincter – “bucket
handle” or “black ribbon”
Rectourethral fistula
Rectovesical fistula
Both sexes
Imperforated Anus – more frequent in Down syndrome
Rectal Atresia
Female
Vestibular fistula – rectum open in the vestibule of the female genitalia immediately outside the hymen
Persistent Cloacae – Rectum, vagina, and urinary tract meet and fuse into a common channel
Most important question are:
1. Does the child have any other abnormalities (50% have another urological problem)
2. Is any abnormality life threatening
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Anal fissure
Small laceration of the mucocutaneous junction of the anus
Caused by forceful passage of a hand stool
Clinical
The child usually has constipation; the child had a hard, large painful bowel movement
Examination
Fissure is seen; also a skin tag may be seen
Treatment
Miralax – enough to keep the stool soft
Perianal Abscess and Fistula
Those < 2 years of age usually have no underlying condition and will heal spontaneously (even if they reoccur)
Those > 2 years of age – often have an underlying disease
Autoimmune neutropenia, leukemia, AIDS, diabetes, Crohn disease, or prior rectal surgery
Clinical
<2 years of age – low grade fever, rectal pain, cellulitis, pustule, drainage
>2 years of age – rapidly expanding cellulitis – warmth, erythema, induration, tenderness, fluctuation,
toxic, septic
Treatment
Drain abscess
Antibiotics
Fistulotomy– if fistule forms
Hemorrhoids
Rare in children
Suspect portal hypertension
Treat constipation with Miralax
Rectal prolapse
The rectal mucosa protrudes through the anus
May be idiopathic
Must exclude underlying conditions – intestinal parasites, malnutrition, diarrhea, ulcerative colitis, pertussis,
Ehlers-Danlos, meningocele, Cystic fibrosis, and chronic constipation, previous anal surgery
Treatment
Reduce Prolapse
Look for underlying conditions – and treat them
Future
Avoid excessive pushing with defecation
Stool softener
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Pilonidal Sinus and Abscess
Occurs in adolescents
Treatment
Incision and Drainage during the acute phase
Block resection to remove the entire epithelial tract
Tumors of the Digestive Tract
Familial Polyposis Syndrome – malignant potential
Juvenile colonic polyps
Clinical – bright red painless rectal bleeding, during or immediately after defecation. Polyp may
prolapse
(dark, beefy red)
Diagnosis and Treatment – colonoscopy – at same time remove them
Multiple juvenile colonic polyps (more than 3 to 5) – autosomal dominant
Associated with congenital anomalies
Increased risk of gastrointestinal cancer (50%)
Clinical – rectal bleeding, failure to thrive, malabsorption, anemia, hypoalbuminaemia, abdominal pain
Treatment – colonoscopy every 3 years
Hemangioma
Clinical – painless bleeding, massive hemorrhage
Leiomyoma – rare – stomach, jejunum, and distal ileum
Carcinoma – rare – seen in patients with FAP, heredity nonpolyposis colon carcinoma, Peutz-Jeghers syndrome,
juvenile polyposis coli, ulcerative colitis and Crohn disease
Lymphoma – most common gastrointestinal tract malignancy in children
Predisposing condition – AIDS, ataxia-telangiectasia, Wiskott-Aldrich syndrome, agammaglobulinemia,
severe combined immunodeficiency syndrome, bone marrow or organ transplantation, celiac
disease
Clinical – crampy abdominal pain, vomiting, distention, abdominal mass, acute intussusception
Carcinoid Tumors – diarrhea, flushing, wheezing, right sided heart failure
Diagnosis – high urinary levels of 5-hydroxy indolacetic acid (5-HIAA)
Inguinal Hernias
Due to patency of the processus vaginalis – indirect hernia
Incidence 3.5-5.0% in full term infants 9-11% in preterm infants
Boys: Girls = 6:1
60% on right side
10% bilateral
22
Clinical
Bulge or mass in inguinal region that may extend into the scrotum
It may come and go. Larger when child cries.
Examination
Smooth, firm mass in inguinal area that may extend into the scrotum
It is normally easily reduced
The hernia may become incarcerated (non-reducible)
May develop irritability, inguinal and abdominal pain, vomiting, abdominal distension, as the
hernia progresses to a strangulated hernia (ischemic or gangrenous)
Management
Surgical repair – early
Reduable – elective
Incarcerated – reduce – surgery 48 hours later
Strangulated – immediate surgery
Contralateral Exploration
Controversial in boys 10-40% change of developing a contralateral hernia
Girls have a high incidence of bilateral inguinal hernias - recommended
Direct Inguinal Hernia – rare in children – acquired defect
May have a connective tissue disorder as Ehlers-Danlos syndrome or Marfan syndrome
Femoral Hernia – rare in children Girls: Boys = 2:1
Exocrine Pancreas
Anatomic Abnormalities
Pancreatic agenesis – rare – neonatal diabetes
Annular pancreas – complete or partial bowel obstruction
associated with Down syndrome, intestinal atresia, imperforated anus, pancreatitis, and malrotation
Treatment – Duodenojejunostomy
Choledochal cysts – dilation of biliary tract
causes jaundice, pain, and fever
Diagnosis by ultrasound
Cystic Fibrosis – see Pulmonary section
Schwechman-Diamond
Pancreatic insufficiency, neutropenia, metaphyseal dysostosis
failure to thrive, short stature, recurrent pyrogenic infections, thrombocytopenia, anemia, may develop
myelodysplastic syndrome, acute myeloid Leukemia – normal sweat test
Treatment – oral enzyme replacement (Pancreas, Creon, Ultrase)
23
Pancreatitis
Acute pancreatitis is the most common pancreatic disease in children
It may result from blunt abdominal trauma, mumps, viral illnesses, multisystem disease, congenital
abnormalities, biliary sludge or unknown causes
Clinical
Abdominal pain, (epigastric and steady), vomiting, fever, hips and knees flexed , or lying on side, appears
ill; abdomen is distended and tender, mass may be present, dehydrated
Children rarely have severe pancreatitis (shock, high fever, jaundice, ascites, hypocalcaemia and pleural
effusion, Cullen sign and Grey Turner sign)
Laboratory
Elevated amylase, elevated amylase isoenzymes, elevated lipase, leukocytosis, hyperglycemia,
glucosuria, hypocalcaemia, elevated gamma glutamyl transpeptidase and hyperbilirubinemia
Ultrasound and CT scan
Pancreatic enlargement, edematous pancreas, pancreatic mass, fluid collections or abscesses
Treatment
Pain relief
Fluid, electrolyte and mineral deficits corrected
Nasogastric tube if patient is vomiting
Usually lasts 2 to 5 days
Begin feedings when the vomiting has stopped and the amylase is falling
Chronic or recurrent Pancreatitis
Frequently hereditary (Autosomal dominant) (Chromosome 7) or due to congenital abnormalities of the
pancreatic or biliary ducts, less common causes are hyperlipidemia (Types I, IV, and V),
hyperparathyroidism, and ascariasis
Evaluation
Serum lipids, calcium, phosphorus, stools for ascaris, sweat test, HP, SPINK 1 and CFTR genes
Abdominal x-ray, ultrasound or CT scan, ERCP and MRCP
Pseudocyst of the Pancreas
Rare sequel of acute and chronic pancreatitis
Clinical – pain, nausea, vomiting, palpable mass, jaundice, ascites, pleural effusion
Evaluation – ultrasound
Treatment
Observe – most resolve
Non-resolving – percutaneous and endoscopic drainage
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Pancreatic Tumors
Endocrine or Nonendocrine
Endocrine – autosomal dominant multiple endocrine neoplasia Type I
Insulinomas – hypoglycemia and elevated insulin level (MEN-1)
Gastrinomas – refractory gastric ulcers (Zollinger-Ellison syndrome)
Non-endocrine
Watery diarrhea – hypokalemia – acidosis syndrome
due to secretion of vasoactive intestinal peptide (VIP)
Pancreatoblastomas
Frantz tumor – girls – abdominal pain, mass, or jaundice
Cyst of the pancreas – seen in Hippel-Lindau disease
Treatment
Surgery if all can be removed
Medication for those that cannot be completely removed
Neonatal Cholestasis
Elevation of serum conjugated bilirubin beyond the first 14 days of life.
Must consider: 1). Biliary atresia (1/10,000 – 1/15,000), 2). Neonatal hepatitis (1/500 – 1/10,000), 3).
intrahepatic Cholestasis (1/50,000 – 1/75,000)
Clinical
Jaundice, dark urine, light or acholic stools, hepatomegaly
May develop hypoprothrombinemia and bleeding disorders
Laboratory
More than 20% of Total bilirubin is conjugated bilirubin
Need to exclude sepsis, hypothyroidism, panhypopituitarism, galactosemia, tyrosinemia, α1 antitrypsin
deficiency, cystic fibrosis, syphilis, toxoplasmosis, rubella, CMV, herpes, and hepatitis viruses (A,
B, C)
Neonatal Hepatitis syndrome
Idiopathic neonatal hepatitis – unknown cause
Infectious hepatitis in a neonate – due to a specific virus
rubella, CMV, herpes simplex, enterovirus, hepatitis B
Intrahepatic Cholestasis – heterogeneous group of diseases and causes
Intrahepatic Bile Duct Paucity – absence or marked reduction in the number of interlobular bile ducts
Alagille syndrome – most common cause of bile duct paucity
Clinical – broad forehead, deep-set wide spaced eyes, long straight nose, underdeveloped mandible, peripheral
pulmonary stenosis, or Tetralogy of Fallot, vertebral abnormalities, and nephropathy
Byler disease – Type 1, 2, 3
Aegenaes – Cholestasis and Lymphedema of the lower extremities
Zellweger
25
Neonatal Iron Storage Disease – increased iron deposition in Liver, heart, and endocrine organs
Deficiency of delta4 – 3 – oxosteroid -5 β redustase
3 β hydroxy c27 – steroid dehydrogenase – isomer (3-HSD)
Progressive familial intrahepatic Cholestasis
Oxysterol 7 α-hydroxylase
Biliary Atresia
Complete obliteration of the entire extra hepatic biliary tree at or above the portahepatics
Clinical
Persistently acholic stools
Ultrasound of abdomen may demonstrate choledcholithiasis, perforation of the bile duct, Choledochal
cyst, polysplenia
Hepatobiliary Scintigraphy – uptake is normal, but no excretion into the intestines, due follow-up at 24
hours
Give Phenobarbital (5mg/Kg/day) for 5 days before the scan
Percutaneous liver biopsy – most reliable test
bile duct proliferation, bile plugs, portal or perilobular edema and fibrosis, basic hepatic lobular
architecture is intact
Treatment
Laparotomy and direct cholangiography
Kasal procedure – when no correctable lesion is found
should be done before 8 weeks of age (success rate of 90%)
May eventually need a liver transplant
Cholestasis – idiopathic neonatal hepatitis
Sporadic cases 60-70% recover with no consequences
Familial only 20-30% recover
Treatment
Medium-chain triglyceride containing formula
Vitamin A, D, E, K supplementation
Ursodeoxycholic acid increased bile – use if any bile duct patency
Transplantation – success rate 85%
May lead to portal hypertension, ascites, variceal with hemorrhage, bacterial peritonitis
Inherited Deficient Conjugation of Bilirubin
Gilbert syndrome – benign disease
Crigler-Najjar syndrome – Type I autosomal recessive
mutation of UDP (B) – GT gene – glucuronyl transferase activity is absent
Clinical
Jaundice in first 3 days, Kernicterus, pale stools
26
Laboratory - >20% of Total bilirubin is direct, no hemolysis
Diagnosis measuring glucuronyl activity in liver tissue
Treatment – keep Total bilirubin less than 20mg/dL phototherapy, exchange transfusion
Calcium phosphate, Cholestyramine or agar to bind photobilirubin
Crigler-Najjar syndrome – Type II
Clinical
Jaundice in first 3 days of life. No Kernicterus
Stool color is normal
Treatment
Phenobarbital
Inherited Conjugated Hyperbilirubinemia – autosomal recessive
Normal liver function, jaundice with infection, pregnancy, oral contraceptives, alcohol consumption, or surgery
Dubin-Johnson syndrome – liver cells contain black pigment
Roto – urinary coproporphyrin is increased
Wilson Disease – autosomal recessive, Kayser-Fleischer rings in the cornea
1/100,000 to 1/500,000 births
Excessive accumulation of copper in the liver then affects brain, kidneys
On Chromosome 13 at q14-q21
Clinical
Hepatomegaly, chronic hepatitis, cirrhosis, portal hypertension, ascites, edema, variceal bleeding,
delayed puberty, tremor, dysarthria, dystonia, deterioration of school performance, behavioral
changes, Kayser-Fleischer rings, hemolytic episodes, renal failure
Diagnosis
Decreased serum ceruloplasmin
Elevated serum copper (early), urinary copper excretion
Liver biopsy – high liver copper
Treatment
Copper-chelating agent – penicillamine
Restrict copper intake to less than 1mg/day
Vitamin B6
Hepatic Copper Overload syndrome
Indian Childhood Cirrhosis
Neonatal Iron Storage disease
27
Α1 Antitrypsin Deficiency
Clinical
1/2000 – 1/4000
Jaundice, acholic stools, hepatomegaly, during first week of life
Jaundice goes away by 2-4 months of life
See Pulmonary chapter
Diagnosis
P1 phenotype
Biopsy of liver
Treatment
Liver transplant
Cystic Fibrosis
Liver disease occurs in 20% of patients with cystic fibrosis
Clinical
Cholestasis, steatosis, focal biliary cirrhosis, multilobular cirrhosis, micro gallbladder, gallstones
See Pulmonary chapter
Treatment
Ursodeoxycholic acid improves bile flow
Nonalcoholic Steatohepatitis
Caused by obesity
Associated with obesity, insulin resistance, hyperlipidemia, hepatomegaly, elevated aspartate aminotransferase
Ultrasound shows fatty liver
Treatment
Dietary consult to lose weight and Vitamin E
Viral Hepatitis
Hepatotropic viruses are designated hepatitis A, B, C, D, E, and G
Other viruses can cause hepatitis as part of their multiple system disease – herpes simplex virus,
cytomegalovirus, Epstein-Barr virus, Varicella-zoster, HIV, rubella, adenoviruses, enteroviruses,
parvovirus B 19, arboviruses
A and E cause only acute disease – not chronic disease
B, C, and D can cause acute and chronic disease
G can cause acute and chronic disease – no morbidity or mortality
Symptoms
Jaundice
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Differential Diagnosis
Above infectious agents
Hemolytic – uremic syndrome
Reye syndrome
Malaria
Heptospinosis
Brucellosis
Severe infections (malignancy, immunodeficiency)
Gall stones
Hemolytic anemia
Wilson’s disease
Cystic Fibrosis
Jamaican Vomiting sickness
Collagen Disease – S.L.E.
Medications – acetaminophen overdose, valproic acid, etc
Hepatitis A
Occurs throughout the world
Developing countries – 100% of children by 5 years of age
USA – 30-40% of adult population
Causes only acute disease – not chronic disease
Causes 50% of clinical apparent viral hepatitis in the USA
Spread – person to person contact – fecal – oral route
infected people, child care centers, homosexual population, food and water, travel to endemic areas
Incubation period 15-50 days (Average about 4 weeks)
Fecal excretion of the virus occurs late in the incubation period. Peak just before symptoms
Clinical
Jaundice, tiredness, fever, nausea, anorexia, abdominal discomfort, right upper abdominal pain, dark
colored urine
Diagnosis
IgM anti-HAV positive for 4-6 months
IgG anti-HAV positive after 4 months
Virus isolated in stools 2 weeks before and 1 week after symptoms
ALT, AST, bilirubins, ALP, 51 nucleotidase, GGT are all elevated. Vit K may be prolonged
Treatment
Gets better with time – no specific treatment
IV fluids if needed
Prevention
Vaccine – 2 doses 6 to 12 months apart for children over 2 years of age
Immunoglobulin
Pre-exposure – travelers to country where HAV is endemic
29
Post-exposure – household contacts, sexual contacts, newborn of HAV infected mother
Child care staff, employees, and children
Outbreaks in institutions and hospitals
Hepatitis B
Worldwide – highest prevalence in Africa, China, Middle East, Amazon, Pacific Islands, Alaska Eskimo
New cases in USA 300,000/year – highest incidence in 20-29 year age group
Less than 10% in children but account for 20-30% of all chronic cases
Most children acquire HBV in perinatal period from a mother who is HBsAg- positive
The risk is greatest if the mother is also HBcAg – positive
70-90% becomes chronically infected, if untreated in the first day of life
Also found in breast milk of infected mothers – but not increase risk of baby acquiring the disease
Other risk factors – IV drugs abuse, blood products, sexual contact, institutional care, contact with carriers
Chronic infection risk is inversely proportional to age of acquisition
Leads to chronic Liver disease and hepatocellular carcinoma
Incubation period – 45 to 160 days – mean 120 days
Clinical
Many cases are asymptomatic
Lethargy, anorexia, malaise, elevated ALT o????, arthralgia, skin lesions, Gianotti-Crosti syndrome,
polyarteritis, glomulonephritis, Aplastic anemia
May occur 6-7 weeks after exposure
Jaundice begins 8 weeks after exposure and lasts 4 weeks
Chronic form – (chronic active hepatitis) – can progress to cirrhosis and hepatocellular carcinoma (2530 years after initial infection)
Exam
Icteric skin and mucous membranes, liver enlarged and tender
Splenomegaly and Lymphadenopathy are common
Diagnosis
HBsAg (first to rise), HBeAg (acute phase highly infectious status)
IgM antibodies to HBcAg (IgM anti-HBcAg), rises early and persists for many months
IgG (persists for years)
IgM anti HBcAg is usually not present in perinatal HBV infections
Anti-HBsAg is the only test positive in immunized person
Anti-HBsAg and anti-HBcAg are present in patients with resolving hepatitis
Treatment
No treatment in the acute phase
Chronic hepatitis in patients over 18 years of age with compensated liver disease and HBV replication –
Interferon – α – 2b and Lamivudine
Liver transplantation – patients with end-stage disease
Complications
Acute Fulminant hepatitis with Coagulopathy, encephalopathy, and vertebral edema
30
Chronic hepatitis that can progress to cirrhosis and primary hepatocellular carcinoma
Membranous glomerulonephritis – rare
Prevention
Hepatitis B vaccine
Hepatitis B immunoglobulin – only for specific post exposure
provides only temporary protection
Infants of HBsAg positive mothers should receive both as soon possible after birth
Hepatitis C
The most common cause of chronic liver disease in the United States – causes 8,000 to 10,000 deaths per year
4,000,000 people in the US are infected
25% of infected people are persistently infected
Infected – main cause – IV drug use, sexual transmission, imprisonment, occupational exposure, blood
transfusion (rare now, common in past)
Genotype 1b is the most common type in the US
Incubation period 7-9 weeks (range 2-24 weeks)
Clinical
Similar to the other hepatitises – usually mild and insidious initially. Most likely hepatotropic virus to
cause chronic infection – 85% become chronic
After 20-30 years – 25% progress to cirrhosis, liver failure, and occasionally to hepatocellular carcinoma
Child disease is milder and has a slower progression than adults
Chronic hepatitis may lead to essential mixed cryoglobulinemia, and nephrotic syndrome
Diagnosis
Antibodies to HCV – EIA’s
Detecting RNA (polymerase chain reaction), (very sensitive – detect recent, perinatal infection, and
immunosuppressed infection). DNA (bronchial-chain DNA)
Recombinant immunoblot assay – confirmatory test
Elevated ALT – means liver disease and justifies a liver biopsy to confirm and assess the presence of
fibrosis
Should screen all patients at high risk and children born of HCV positive women
Anti-HCV EIA after 12 months or PCR in infancy
Treatment
IFN-α2b – 10-15% effective – normal ALT and negative PCR 6 months after completion of therapy
Interferon and ribavirin - ⅓ have good response – preferred treatment
Should be immunized for Hepatitis A and B to minimize
Complications
Risk of chronic hepatitis is the highest of the hepatotropic viruses
Risk factors for progression to hepatic fibrosis – older age, male, moderate alcohol ingestion (1g/day)
31
Prevention
Not use IV drugs, limit sexual contacts, use condoms, not share toothbrushes or razors
No immunization available
Gamma globulin does not contain antibodies to Hepatitis C
Hepatitis D
Smallest known animal virus
Cannot produce disease without a Hepatitis B infection
Can make Hepatitis B infection worse
Consider it in patients with fumining hepatitis, parenteral drug abusers, hemophilia, and emigrants
Incubation period – 45-160 days – mean 120 days
Clinical
In co-infection Acute hepatitis – more severe than other hepatitis – risk of chronic hepatitis is low
In superinfection Acute illness is rare and chronic hepatitis is common – risk of fulminant hepatitis is
highest
Diagnosis
IgM antibody to HDV
Complication
Fulminant hepatitis
Prevention
No vaccine for HDV
Vaccine for Hepatitis B
Hepatitis E
Seen only in other countries, visitor or emigrants from other countries
Incubation period – 15-60 days – mean 40 days
Produce only acute disease
More severe than Hepatitis A
High fatality rate in pregnant women
Diagnosis
IgM – IgG – Hepatitis E antibodies
Viral ANA in stool and serum
Prevention
No vaccine – gamma globulin is not effective
Hepatitis G
Risk factors – HIV infection, chronic hepatitis B & C, transfusion, organ transplantation recipients, IV drug
abuse, hemodialysis, sexual activity
32
Clinical
Not associated with hepatic inflammation
Co-infection does not worsen the course of concurrent HBV or HCV infection
May not cause symptomatic disease or may cause an Epstein-Barr of CMV type illness
Does not cause chronic hepatitis
Diagnosis
PCR to detect HGV RNA
Delays development of AIDS in patients infected with both
Prevention
None
Hepatitis in Newborn
Greatest risk of perinatal transmission is with HBV
Nearly all are asymptomatic
Usually caused by bacteria, syphilis, enteroviruses, CMV, HSV, HIV, and Toxoplasm???
Clinical
Jaundice, vomiting, poor feeding, elevated liver enzymes
Differential diagnosis
Intrahepatic and extrahepatic biliary atresia, Choledochal cyst, cystic fibrosis, disorders of bile acid
metabolism, galactosemia, tyrosinosis, α1 antitrypsin deficiency, hyperalimentation, or drugs as
cause
Treatment
Ampicillin and Gentamicin for bacterial infections
Acyclovir and Foscarnet for CMV infection
Pleconaril for enteroviruses
Prevention
If mother is HBsAg positive, baby should receive both hepatitis B vaccine – HB1G within 12 hours of
birth. Then receive the 2nd dose at least 1 month after the first dose and the third dose at least
4 months after the first dose (and at least 2 months after the second dose)
Liver Abscess
Infants – see with sepsis, umbilical vein infection or vessel cunnulation
Children – immunosuppressed
40% chronic granulomatous disease
20% immunosuppressed – Leukemia, etc
Organisms – staphylococcus aureus, E. Coli, Salmonella, anaerobes, candidemia, cat-scratch disease or
Entamoeba histolytica
33
Clinical - fever, right upper quadrant pain, enlarged liver that is tender
Laboratory - sed rate is high, ALT, AST, and Alk phosphatase may be mildly elevated
X-ray – chest – elevated right hemidiaphragm
Ultrasound or nuclear scan – site of abscess
Treatment
Based on infectious cause
Liver Disease associated with Systemic Disorders
Inflammatory Bowel Disease
Bacterial Sepsis – E. coli, Klebsiella pneumonia, Pseudomonas aeruginosa
Cardiac Disease
Hemoglobinopathies – sickle cell anemia or trait
Cholestasis Associated with Total Parental Nutrition
incidence is inversely proportional to the birth weight and proportional to the duration of treatment
Bone Marrow Transplantation
Collagen Vascular Disease – rare – seen with S.L.E.
Drugs – NSAIDS, chemotherapy
Obesity – (nonalcoholic Steatohepatitis – NASH), fatty liver, inflammation and fibrosis
Reye Syndrome
Acute encephalopathy and fatty degeneration of the liver
Loss of mitochondrial function that leads to abnormal fatty acid and carnitine metabolism
Highest incidence 1974 – 400 cases – now rare
Related to viral epidemics – especially Influenza B and Varicella
especially associated with the use of aspirin
Usually between 4 and 12 years of age – mean 6 years
Clinical
Preceded by a febrile viral upper respiratory tract infection (90%) or Varicella (5-7%). Then child seemed
well. Then protracted vomiting, delirium, combative behaviors, abnormal liver function tests,
not jaundiced, normal spinal fluid
Diagnosis
Elevated AST, ALT, CPK, Aldolase, Lactic acid, ammonia, glutamate dehydrogenase
Prothrombin prolonged
Differential Diagnosis
Organic acidurias
Disorders of oxidative phosphorylation
Urea cycle defects (carbamoyl phosphate synthetase ornithine transcarbamoylase)
Defects in fatty acid oxidation metabolism
Acyl-CoA dehydrogenase deficiencies
34
Systemic carnitine deficiency
Hepatic carnitine palmitoyltransferase deficiency
3-OH, methylglutaryl – CoA Lyase deficiency
Fructosemia
CNS infections or intoxications
Hemorrhagic shock with encephalopathy
Drug or toxin ingestion (salicylates, valproate)
Treatment
10-15% glucose IV
Restrict fluid – especially in patients with cerebral edema
Avoid hyperthermia
Vit K, fresh frozen plasma and platelet transfusion
Severe, respiratory support, hyperventilation
Monitor ICP
Pentobarbital
Cystic Diseases of the Biliary Tract and Liver
Choledochal Cyst – congenital dilatations of the common bile duct
May cause biliary obstruction and biliary cirrhosis
Clinical
Jaundice, liver dysfunction, ascites, Coagulopathy
Older child – abdominal pain, jaundice, mass, fever, right upper quadrant tenderness Leukocytosis
Diagnosis
Ultrasound
Treatment
Roux-en-Y choledochojejunostomy
Cystic Dilation of Intrahepatic Bile Duct (Caroli Disease)
Congenital Hepatic Fibrosis
Autosomal recessive
Clinical
Hepatosplenomegaly
Portal hypertension with bleeding
Autosomal Dominant Polycystic Kidney Disease (ADPKD)
Multiple hepatic cysts are associated with this condition but rarely occurs before 16 years of age
Cholangiocarcinoma may occur
35
Autosomal Recessive Polycystic Disease (ARPKD)
Associated with hepatic fibrosis and biliary duct ectasia
Later may develop portal hypertension
Hepatosplenomegaly leads to portal hypertension
Gall Bladder
Hypoplasia or absence of the gall bladder – associated with extrahepatic biliary atresia or cystic fibrosis
Acute hydrops
Seen in patients who received parental nutrition over a long period of time, and Kawasaki disease
Clinical
Fever, vomiting, and jaundice
Diagnosis
Ultrasound
Treatment
Spontaneous resolution
Cholecystitis and Cholelithiasis
Acute acalculous Cholecystitis – rare in children
Caused by Group A and B streptococci, salmonella, leptospira interrogans, ascaris, Giardia lamblia, burns or
systemic vasculitis
Clinical
Right upper quadrant or epigastric pain, nausea, vomiting, fever and jaundice
Diagnosis
Ultrasound – enlarged thick-walled gallbladder without calculi
Alkaline phosphate, bilirubin and WBC are elevated
Treatment
Cholecystectomy
Cholelithiasis
Rare in children – seen in patients who have a hemolytic anemia, Wilson’s disease, cirrhosis, chronic
Cholestasis, Crohn disease, cystic fibrosis, and premature infants
Adolescents who are or have been pregnant or are obese
Clinical
Recurrent abdominal pain – colicky and in the right upper quadrant
Intolerance to fatty foods
Acute Cholecystitis
Fever, pain in right upper quadrant, and palpable mass
36
Diagnosis
Ultrasound
Treatment
Laparoscopic cholecystectomy
Portal Hypertension
Portal pressure above 12 mm of Hg (normal 7mm)
Causes
Portal vein thrombosis, splenic vein thrombosis, A-V fistula, acute and chronic viral hepatitis, cirrhosis,
congenital hepatic fibrosis, Wilson disease, α1 antitrypsin deficiency, glycogen storage disease
type IV, hepatotoxicity (methotrexate, Parenteral nutrition), biliary atresia or paucity, cystic
fibrosis, Choledochal cyst, Sclerosing cholangitis, Budd-Chiari Syndromes, veno-occlusive disease
Main cause is Portal Vein thrombosis secondary to neonatal umbilical infections in infants
In older children may be caused by intra-abdominal infections, hypercoagulable states, and cirrhosis
Clinical
Bleeding from esophageal varices – may be life threatening – especially if patient has liver disease
In patients with liver disease, jaundice, palmar Erythema,
Vascular telangiectasis, dilated cutaneous collateral veins
Splenomegaly with hypersplenism
Diagnosis
Ultrasound – with Doppler flow
Endoscope – detect esophageal varices
Treatment
Of variceal hemorrhage – IV fluids – crystalloid initially then red blood cells, Vit K, platelets, and fresh
frozen plasma
Insertion of NG tube – monitor blood loss
H2 blocker – ranitidine
Vasopressin
Nitroglycerin – decreases portal pressure
Octreotide
Sengtaken-Blakemore tube – if bleeding persists
Beta-blockers – propranolol
Surgical
Portacaval shunt
Risk of hepatic encephalopathy in patients with liver disease
Mesocaval or distal splenorenal shunt
Liver transplant
Prognosis
Poor if have concurrent liver disease – may need liver transplant
37
Liver Transplant
Indications
End stage liver disease, hepatic metabolic disease, and cancers of the liver, extrahepatic biliary atresia
after a failed Kasai procedure, acute hepatic necrosis, fulminant hepatic failure, ascites,
encephalopathy, variceal bleeding, and renal failure
Pretransplantation management
Nutrition – 150 K cal/Kg/24 hrs, medium-chain triglycerides, vitamins, ursodeoxycholic acid, all
immunizations
Post transplant
Prevent rejection with steroids, cyclosporine (Neoval) and/or tacolimus
Complication
Increases risk of Lymphoproliferative disease (4-11%)
Hypertension
Renal failure
Peritoneal bands
Cause intestinal obstruction and intra-abdominal herniation
Ascites
Accumulation of serous fluid within the peritoneal cavity
Causes
Hepatic
Renal
Cardiac
Clinical
Abdominal distension, bulging flanks, flank dullness, shifting dullness, fluid wave, “puddle sign”, and
umbilical hernia
Diagnosis
Ultrasound
Complication
Bacterial peritonitis
Chylous Ascites
Cause
Abnormality, injury, or obstruction of the abdominal portion of the thoracic duct
Diagnosis
Obtaining milky ascetic fluid by paracentesis parenteral alimentation after a fatty meal
38
Treatment
High-protein, low fat diet, medium-chain triglycerides
Parenteral alimentation
Laparotomy – search for site of the leak
Peritonitis
Inflammation of the peritoneal lining of the abdominal cavity
Causes
Infection, autoimmune, or chemical
Acute Primary Peritonitis
No intra-abdominal cause
Occurs in children with ascites from nephrotic syndrome or cirrhosis
Usually streptococcus pneumonia, but can be due to group A streptococcus, Enterococci, staphylococcus, gram
negative bacterial E. coli – Klebsiella pneumonia
Clinical
Fever, abdominal pain, vomiting, diarrhea, toxic appearance, hypotension, tachycardia, shallow rapid
respiration
Rebound tenderness, rigidity of abdomen
Absent or hypoactive bowel sounds
Diagnosis
Leukocytosis with mainly polymorphonuclear cells
X-rays – dilation of the large and small bowel
Paracentesis > 250cells/mm3 > 50% polymorphonuclearcytes
Ph <7.35, elevated lactate, positive gram stain
Treatment
Cefotaxime and Gentamicin – change with respect of organism involved and its sensitivities
Acute Secondary Peritonitis
Usually occurs because of necrotic intestines, or other visceral that permit enteric bacteria into the peritoneum
perforation of the appendix, Meckel diverticulum, incarcerated hernias, volvulus, intussusception, hemolytic
uremic syndrome, peptic ulcer, inflammatory bowel disease, Cholecystitis, MEC, Typhlitis, traumatic
perforation, or foreign body
In postpubertal females gonorrhea or Chlamydia may cause it
Clinical
Fever, abdominal pain, nausea, and vomiting
Rebound tenderness, abdomen wall rigidity, not moving, absent or decreased bowel sounds, toxic
appearing, irritability, restlessness, shock
39
Diagnosis
Elevated WBC with mainly polymorphonuclear cells
X-ray – free air, ileus, obstruction, peritoneal fluid and obliteration of the psoas shadow
Treatment
Fluids – IV – normal saline
Antibiotics – Ampicillin, Gentamicin, and Clindamycin
later as sensitivities dictate
Surgery – repair the perforation
Peritoneal Abscess
May occur anywhere within the abdomen
Symptoms
Similar to Acute Secondary Peritonitis
Diagnosis
Ultrasound or CT scan
Treatment
Antibiotics – same as Acute Secondary Peritonitis
Drainage – insertion under ultrasound or CT guidance
Diaphragmatic Hernia
May be congenital or traumatic
Location may be hiatal, paraesophageal, retrosternal (Morgagni) or posterolateral (Bochdalek)
Congenital diaphragmatic hernia – are posterolateral – present with severe respiratory distress in the newborn
period
Are associated with hypoplastic lungs, pulmonary hypertension and malrotation of the intestines
The lung on the side of the hernia is the most hypoplasia
Have a significant mortality (40-50%)
70-80% are on the left side, 5% are bilateral
Associated with other congenital abnormalities and with chromosomal and non-chromosomal syndromes
Clinical
May be diagnosed by prenatal ultrasound
Most develop severe respiratory distress within the first hour of life
A few will not present until after the neonatal period with intestinal obstruction or respiratory
symptoms
Diagnosis
Prenatal diagnosis by ultrasound
Need to evaluate for other abnormalities – echocardiogram and amniocentesis
Absence of breath sounds on one side
Shift of heart sounds
40
Scaphoid abdomen
Chest x-ray is diagnostic
Treatment
Extracorporeal membrane oxygenation (ECMO)
Preoperative stabilization
Delayed repair
Stabilization with sedation and paralysis
Mild hyperventilation (PCO2 of 25-30 mm Hg)
Volume resuscitation, dopamine, bicarbonate
Repair when stable usually 24-72 hours of life
ECHO if child is severely ill or not respond to above treatment, may need to 7 to 14 days
Dopamine
Nitric oxide
Dipyridamole
Surfactant
Abdominal surgical approach
Prognosis
Survival – 42 to 75%
Factors associated with poor prognosis
1. Symptoms before 24 hours of age
2. Severe pulmonary hypoplasia
3. Herniation to the contralateral lung
4. Required ECMO
5. Delivered in a non tertiary center
Most survivors have decreased lung function in the neonatal period and years afterwards
High incidence of reactive airway disease
Neurological complication in 24% if required ECMO 67%
40-50% is less than the 5th percentile
Gastroesophageal reflux
Pectus excavatum
Scoliosis
Fixed pulmonary hypertension
Recurrent hernia – 20-40% of those with large defects that require patches
Foramen of Morgagni Hernia
Accounts for 2% of Diaphragmatic hernias
Eventration of the Diaphragm – elevation of the entire hemidiaphragm
Epigastric Hernia
Intermittent bulge or midline abdominal mass
Treatment
Repair if symptomatic
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References:
Behrman RE, Kliegman RM, Jenson HB Nelson Textbook of Pediatrics. 17th Edition Saunders, The Curtis Center
Philadelphia 2004 1197-1356
Boyle, JT Recurrent Abdominal Pain: An Update Pediatrics in Review 1997; 18(9):310-321
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