PEDIATRIC SURGERY

Schwartz 11th Edition

Eve Queennie May R. Balbon, MD

GASTROINTESTINAL
TRACT
 Approach to the Vomiting Infant
● Differentiate between normal and abnormal vomiting
● Characterize what the vomit looks like and the timing
○ Looks like feeds and comes up immediately after a
feeding: gastroesophageal reflux
○ Occurs short after feeding and projectile: pyloric
stenosis
○ Green in color: intestinal volvulus, an underlying
infection, or some other cause of intestinal
obstruction
 Hypertrophic
Pyloric Stenosis
Hypertrophic pyloric stenosis
● Due to progressive thickening of the pylorus
muscle
● Approximately 1 in 300 live births
● Common in infants between 3 and 6 weeks of age
● Male-to-female ratio of 5:1
● Familial link
● Pyloric muscle thickening -> complete gastric
outlet obstruction
Hypertrophic pyloric stenosis
CLINICAL MANIFESTATIONS
● Nonbilious vomiting
○ Projectile over days to weeks
● Increasingly hungry d/t unsuccessful feeding
● Dehydration
● Less passage of flatus
● Associated with jaundice
DIAGNOSIS

● PE: palpation of olive

mass in RUQ; (+) visible
gastric waves

● UTZ: (1) channel length of over 16

mm; (2) pyloric thickness over 4 mm
● Upper GI series: delayed passage of
contents from the stomach through
the pyloric channel and a typical
thickened appearance to the
pylorus.
 TREATMENT
● Pyloric stenosis is never a surgical emergency
● Fluid resuscitation and correction of electrolyte
imbalance & metabolic alkalosis
○ 5% dextrose and 0.45% saline with added
potassium of 2 to 4 mEq/kg over 24 hours at
a rate of approximately 150 to 175 mL/kg for 24
hrs
● Ensure UO of >2 cc/kg/hr
● Fredet-Ramstedt pyloromyotomy
○ splitting the pyloric muscle while leaving the
underlying submucosa intact
○ incision extends from just proximal to the pyloric
vein of Mayo to the gastric antrum, between 1
and 2 cm in length
● POST-OPERATION
○ Continue IV fluids for several hrs -> Pedialyte ->
formula or breast milk 60 cc every 3 hrs
○ Discharged w/in 24 to 48 hrs following surgery

● COMPLICATIONS
○ perforation of the mucosa (1–3%)
○ bleeding
○ wound infection
○ recurrent symptoms due to inadequate myotomy
Intestinal Obstruction
in the Newborn
Intestinal obstruction in the newborn
● 1 in 2000 live births
● Consider surgical etiology in neonates with bilious
vomiting

CLINICAL MANIFESTATIONS

● Bilious emesis: cardinal symptom

Intestinal obstruction in the newborn
DIAGNOSIS

● Determine whether obstruction is proximal or distal to

ligament of Treitz
○ Proximal intestinal obstruction
■ Bilious emesis, (+) abdominal distension
■ Flat or scaphoid abdomen
■ Abdominal X-ray: paucity or absence of bowel
gas
○ Distal obstruction
■ Bilious emesis, (+++) abdominal distension
Duodenal Obstruction
Duodenal obstruction
● Causes of duodenal obstruction include duodenal
atresia, duodenal web, stenosis, annular pancreas,
or duodenal duplication cyst

● Associated polyhydramnios

CLINICAL MANIFESTATION
○ Nonbilious vomiting

○ Abdominal distention is not usually present

 Duodenal obstruction

DIAGNOSIS
○ UTZ

■ Fluid-filled stomach and proximal

duodenum as two discrete cystic

structures in the upper abdomen
■ Classic sign: “double bubble sign”
TREATMENT
○ Orogastric tube insertion (decompress stomach and
duodenum)
○ Fluid resuscitation
○ Workup for other associated anomalies
○ Duodenoduodenostomy
■ Surgical treatment of choice for duodenal obstruction

due to duodenal stenosis or atresia or annular pancreas

■ Annular pancreas is never divided but rather is bypassed

to avoid injury to the pancreatic ducts

TREATMENT

○ Vertical duodenotomy, excision of the web, oversewing of

the mucosa, and closing the duodenotomy horizontally
■ Treatment of duodenal web

○ In premature infants with duodenal obstruction duodenal

repair can be performed in children as small as 1 kg
Intestinal Atresia
Intestinal atresia
● Result of developmental defects in normal intestinal
organogenesis due to disruption of various signaling pathways
○ Fibroblast growth factor, bone morphogenic protein, and

β-catenin pathways

● 1 in 2000 to 1 in 5000 live births

● Male = female
Intestinal atresia
CLINICAL PRESENTATION
○ Infant w/ bilious vomiting and progressive abdominal

distention
DIAGNOSIS
○ Abdominal X-ray

■ The more distal the obstruction, the more

distended the abdomen; and greater number of

obstructed loops on upright abdominal films
○ Barium enema

■ Establish whether a microcolon is present and to

diagnose the presence of meconium plugs, small

left colon syndrome, Hirschsprung’s disease, or
meconium ileus
 Intestinal atresia
● Types of atresia:
○ Type 1
■ Mucosal atresia with intact muscularis
○ Type 2
■ Atretric ends are connected by a fibrous band
○ Type 3A
■ Two ends of the atresia are separated by a V-
shaped defect in the mesentery
○ Type 3B
■ “Apple-peel” deformity or “Christmas tree”
deformity in which the bowel distal to the atresia
receives its blood supply in a retrograde fashion
from the ileocolic or right colic artery
○ Type 4
■ Multiple atresias with a “string of sausage” or “string
of beads” appearance
Intestinal atresia
TREATMENT
○ Laparotomy

○ Anastomosis using the end-to-back technique

■ Distal, compressed loop is “fish-mouthed” along

its antimesenteric border

Malrotation and
Midgut volvulus
Malrotation and Midgut volvulus
● Failure of normal 270° counterclockwise rotation of the GI
tract around the superior mesenteric artery during
embryologic development
○ Mutations in BCL6 gene, FOXF1

● Incomplete rotation
○ Cecum remains in the epigastrium

○ Ladd’s bands form and extends from the cecum to the

lateral abdominal wall and crossing the duodenum,

which creates the potential for obstruction
○ Volvulus may occur which causes obstruction and cuts

off blood supply to the midgut

Malrotation and Midgut volvulus

CLINICAL PRESENTATION
○ Bilious vomiting in the 1st few weeks of life

○ Bloody stools: from vascular compromise of midgut

○ erythema and edema of the abdominal wall

○ shock and death

○ Often asymptomatic (malrotation w/o volvulus)

Malrotation and Midgut volvulus

DIAGNOSIS
○ Abdominal X-ray

■ paucity of gas throughout the

intestine with a few scattered air-

fluid levels in distal bowel
○ Upper GI series

■ incomplete rotation with the

duodenojejunal junction displaced

to the right
TREATMENT
○ Fluid resuscitation
○ Exploratory laparotomy: Ladd’s procedure

1. Volvulus is untwisted counterclockwise

(“turn back the hands of time”)
2. Division of Ladd’s bands
3. Broadening of the mesentery (open
folded mesentery like a book and divide
congenital adhesions)
4. Positioning the colon on left side and the
entire small bowel on the right side to
prevent any future recurrence of volvulus
5. Appendectomy
Meconium ileus
Meconium ileus
● Production of viscous, water-poor meconium In infants with
cystic fibrosis
● This thick, highly viscous meconium becomes impacted in the
ileum and leads to high-grade intestinal obstruction leading to
meconium ileus

CLINICAL MANIFESTATIONS
○ Failure to pass meconium

○ Abdominal distension

○ Intermittent bilious vomiting

Meconium ileus
DIAGNOSIS
Abdominal X-ray
1. Dilated loops of
intestine
2. Soap bubbles or
“ground glass”
appearance
3. Microcolon in
contrast enema
Meconium ileus
DIAGNOSIS
○ Uncomplicated
■ pellets of meconium in terminal ileum
■ no intestinal perforation
○ Complicated
■ intraperitoneal calcification producing eggshell pattern
■ Intestinal perforation and vascular compromise
MANAGEMENT
○ Uncomplicated:
■ Dilution of water-soluble contrast or infusion of N-acetylcysteine (Mucomyst)
transanally via catheter under fluoroscopic guidance; repeat at 12 hr
interval
○ Complicated:
■ Exploratory laparotomy and operative irrigation of contrast and N-
acetylcysteine
■ Resection of distended terminal ileum and anastomosis
Techniques of intestinal anastomosis
Necrotizing Enterocolitis
Necrotizing enterocolitis
● NEC is the most frequent and lethal gastrointestinal
disorder affecting the intestine of the stressed, preterm
neonate
● Survivors of neonatal respiratory distress syndrome are at
risk for developing NEC
● Precursors for NEC are prematurity and enteral
alimentation
● Risk factors:
○ Prematurity
○ Initiation of enteral feeding
○ Bacterial infection
○ Intestinal ischemia resulting from birth asphyxia
○ Umbilical artery cannulation
○ Persistence of a patent ductus arteriosus
○ Cyanotic heart disease
○ Maternal cocaine abuse
CLINICAL MANIFESTATIONS
○ Prematurity

○ Period of mild illness to severe, life-

threatening sepsis
○ Bell stages
PATHOGENESIS OF NEC
○ Prematurity -> bacterial receptor, Toll-like receptor 4
(TLR4) is elevated -> activation of TLR4 by colonizing
bacteria -> severe pro inflammatory response ->
NEC

○ Breast milk is able to suppress TLR4 signaling and

known to be protective against NEC
TREATMENT
○ Feedings are discontinued, a nasogastric tube is placed,
and broad-spectrum parenteral antibiotics are given
○ Fluid resuscitation and inotrope to maintain perfusion
○ Intubation and mechanical ventilation to maintain
oxygenation
○ TPN is started
○ Bell 1: NPO; IV antibiotics for 7 to 10 days
○ Bell 2: assess for peritonitis, foxed mass, abdominal
cellulitis, systemic sepsis; paracentesis; laparotomy
○ Bell 3: laparotomy; resection and anastomosis; drainage
catheters
NEC IN OLDER INFANTS
○ Pattern of disease is different from that found in premature
infants
○ Typically is localized to the end of the small intestine and
beginning of the colon
○ 4 pertinent associations:
■ congenital heart disease

■ in utero growth restriction

■ polycythemia

■ perinatal hypoxic-ischemic events

○ Also associated with formula consumption

○ Typically present with bloody stools
● SPONTANEOUS INTESTINAL PERFORATION (SIP) VERSUS NEC
○ SIP is a distinct clinical entity from NEC
○ Essentially a perforation in the terminal ileum
○ Mucosa is intact and not necrotic with no signs of
ischemia
○ Submucosa is thinned at the site of perforation
○ Pneumatosis intestinalis is absent
○ Demographics: tend to be slightly more premature,
smaller, and have been on inotropic support
○ Occurs in two separate time points
■ Few days after birth and approximately 10 days later

■ Free air will be present

○ Respond better to peritoneal drainage

○ Have better outcome
TREATMENT (SIP)
○ Intestinal resection and stoma creation

○ Stoma reversal once the child is stable

■ Timing of stoma closure: approximately 38 to 40 weeks

or approximately 6 weeks after the initial surgery

OUTCOME
○ Survival by stage is approximately 85%, 65%, and 35% for

stages I, II, and III, respectively

○ At risk of developing short bowel syndrome (total length of

viable intestine is <40 cm)

Short Bowel Syndrome
Short Bowel Syndrome
● Extremely morbid condition with an
increasing incidence
● It can result from extensive surgical resection
or congenital intestinal diseases
○ Gastroschisis

○ Malrotation

○ Atresia

○ NEC may lead to SBS

Short bowel syndrome

CLINICAL MANIFESTATIONS
○ primary adverse outcome of SBS is malabsorption secondary to
decreased intestinal absorptive surface area and rapid intestinal
transit
○ most common signs, symptoms, and findings include malnutrition,
weight loss, diarrhea, steatorrhea, dehydration, vitamin
deficiencies, and electrolyte imbalance

TREATMENT
○ Improve nutrition: parenteral nutrition
○ Prevent infection
○ Reduce cholestasis
Intussusception
Intussusception

● Condition whereby a segment of

intestine becomes drawn into the
lumen of the more proximal bowel
● Begins in the region of terminal ileum
and extends distally into descending,
transverse or descending colon
○ May prolapse through the rectum
(rarely)

● Leading cause of intestinal obstruction

in young child
Intussusception

ETIOLOGY
○ Antecedent viral infection
■ Viral infection -> hypertrophy of Peyer’s patches acts as
lead point drawing the proximal bowel into the distal
bowel by peristalsis
○ Idiopathic in ages 6 and 24 months
○ Beyond this age group consider pathologic lead points
(polyps, malignant tumors such as lymphoma, enteric
duplication cysts, or Meckel’s diverticulum)
 Intussusception
CLINICAL MANIFESTATIONS

Paroxysms of crampy Dance’s sign: Currant-

abdominal pain & RUQ or epigastric jelly stool
intermittent vomiting elongated mass
(sausage-shaped)
with absence of
NOTE: Infant may act normally
bowel in RLQ between episodes
DIAGNOSIS
 Plain abdominal X-ray ○ Ultrasound
 Coiled-spring sign ■ Target sign or doughnut
sign
● TREATMENT
○ Assess for presence of peritonitis & systemic illness
○ Assess for suitability to undergo radiographic vs surgical
reduction
■ (-) peritonitis: radiographic reduction
■ (+) peritonitis: laparotomy
○ In stable px (radiographic reduction)
■ Air enema: preferred method of diagnosis and treatment
● Air is introduced w/ a manometer (should not exceed
120 mmHg)
● Successful reduction: (1) free reflux of air into multiple
loops of small bowel; (2) symptomatic improvement

NOTE: Unless both signs are observed, it cannot be assumed that intussusception is
reduced, reattempt at reduction after few hrs.
Intussusception
○ In stable px:
■ Hydrostatic reduction w/ barium: if pneumatic
reduction is unsuccessful
○ Failure reduction mandates surgery
■ Open
● RLQ incision
● Reduction by milking out
■ Laparoscopic
● Using 5mm laparoscopes placed in:
○ Umbilicus, RLQ, LLQ
■ Appendectomy is often performed.
 ○ IV fluids continued until postoperative ileus subsides
○ Start on clear liquids, diet as tolerated

● Recurrent intussusception occurs in 5-10%

○ Independent of radiographic or surgical reduction
○ Symptoms recur in the immediate postoperative
period
○ Treatment: repeat air enema
○ If 3 or more episodes of intussusception: suspect for
pathologic lead points
Appendicitis
Appendicitis
PRESENTATION
○ Classic: generalized abdominal pain that localizes to the RLQ
followed by nausea, vomiting, fever, and localized peritoneal
irritation in the region of McBurney’s point
■ However, children often do not present in this manner

DIAGNOSIS IN CHILDREN
○ UTZ: good visualization of appendix; exclude ovarian causes
○ Despite radiographic measures, the diagnosis remains largely
clinical
■ Localized RLQ tenderness associated with low-grade fever

and leukocytosis
■ In girls: consider ovarian or uterine pathology
Appendicitis

TREATMENT
○ Appendectomy: definitive treatment

■ Most common complication: surgical site infection

○ Most are back to school 1 wk from surgery; allowed to

return to full physical after 2-3 wks
Appendicitis
TREATMENT OF CHILD WITH PERFORATED APPENDICITIS
○ Signs and symptoms: abdominal pain, vomiting, diarrhea, intestinal
obstruction; abdominal mass may be present in the lower
abdomen
○ Suspect abscess if symptoms present for more than 4 or 4 days
DIAGNOSIS
■ CT scan of abdomen and pelvis with contrast
TREATMENT
■ Appendectomy
■ In abscess without evidence of generalized peritonitis
● Perform image guided percutaneous drainage of the
abscess followed by broad spectrum antibiotic therapy;
Appendectomy 6 to 8 wks later (high failure rate)
● If no improvement: appendectomy
■ 80% certain children will respond to antibiotics alone
Appendicitis
OTHER CAUSES OF ABDOMINAL PAIN THAT MIMICS APPENDICITIS IN CHILDREN
○ UTI
■ Are less likely to present with vomiting
■ Likely to experience difficulty with urination, characterized by pressure,
burning, and frequency
○ Constipation
■ Patients with constipation rarely have fever
■ (-) Abnormalities in their blood work
○ Ovarian torsion
■ Patients are generally asymptomatic until the acute onset of severe pai
■ Appendicitis: gradual onset of pain associated with nausea and vomiting
○ Gastroenteritis
■ Persistent vomiting and diarrhea precedes abdominal pain
Intestinal Duplications
Intestinal duplications
● Represent mucosa-lined structures that are in continuity with
the gastrointestinal tract
● Occur at any level in GI tract
○ Most common: ileum

● May be tubular or cystic (mostly)

● Share a common wall with the intestine

CLINICAL MANIFESTATIONS
○ Recurrent abdominal pain,

○ Emesis from intestinal obstruction

○ Hematochezia: from ulcerations if contains

ectopic gastric mucosa

○ Palpable mass
Intestinal duplications

DIAGNOSIS
○ CT, UTZ, technetium pertechnetate scanning

TREATMENT
○ Short duplication: resection of the cyst and adjacent

intestine with end-to-end anastomosis

○ Long duplication: multiple enterotomies and mucosal

stripping in the duplicated segment; alternatively division of
the common wall using the GIA stapler, forming a common
lumen
Meckel’s Diverticulum
Meckel’s diverticulum
● Remnant of a portion of the embryonic vitelline duct
● Located within 2 ft of the ileocecal valve
● Incidental finding during surgery or may mimic appendicitis
● Perforation occurs when outpouching is impacted with food ->
distention and necrosis
● Bands of tissue extend from the Meckel’s diverticulum to the
anterior abdominal wall
○ May represent lead points around which internal hernias

may develop
● Important cause of intestinal obstruction in the older child who
has a scarless abdomen
● Ectopic gastric mucosa -> produce ileal ulceration -> bleeding
-> passage of maroon-colored stools
● Ectopic gastric mucosa -> produce ileal ulceration -> bleeding ->
passage of maroon-colored stools

DIAGNOSIS
○ Technetium pertechnetate scans

TREATMENT
○ Narrow base: (-) mass in lumen: wedge resection of the

diverticulum with transverse closure of the ileum

○ Wide base: (+) inflammation, (+) palpable ectopic tissue:

resection of the involved bowel and end-to-end
ileoileostomy
Mesenteric Cyst
Mesenteric cyst
● Are similar to duplications in location within the mesentery
● Do not contain any mucosa or muscular wall

● Can cause intestinal obstruction or may present as an

abdominal mass
DIAGNOSIS
○ Abdominal UTZ

○ CT scan

TREATMENT
○ Resection of adjacent intestine

○ Partial excision or marsupialization: when complete excision

is not possible (d/t close proximity to vital structures)

Hirschsprung’s Disease
Hirschsprung’s Disease
● Incidence: 1in 5000 live births

PATHOGENESIS
○ Absence of ganglion cells in Auerbach’ plexus and
hypertrophy of associated nerve trunks
○ Defect in migration of neural crest cells
■ Mutations in GDNF or Rete genes
Hirschsprung’s Disease

CLINICAL PRESENTATION
○ Functional distal obstruction
○ Failure of infant to pass meconium >48 hr of life must
be investigated for Hirschsprung’s disease.
○ Abdominal distension, failure to pass meconium,
bilious emesis: in newborn
○ May present with enterocolitis
■ Abdominal distension and tenderness + fever, failure to
thrive, lethargy
CLINICAL PRESENTATION
○ Dehydration and leukocytosis
○ Rectal examination: forceful expulsion of foul-smelling

liquid feces
○ Initial treatment while awaiting diagnosis: rehydration,

antibiotics, nasogastric compression, rectal irrigation

■ Unresponsive to non operative management:
decompressive stoma
○ Diagnosed beyond the newborn period in 20% of cases
■ Severe constipation, abdominal distension, failure to
thrive
Hirschsprung’s Disease

DIAGNOSIS
○ Rectal biopsy: definitive diagnosis

■ samples of mucosa and submucosa obtained at 1 cm,

2 cm, and 3 cm from the dentate line

○ Histopathologic findings: (1): absence of ganglion cells in

the myenteric plexuses (2) increased acetylcholinesterase
staining (3) presence of hypertrophied nerve bundles.
Hirschsprung’s Disease

DIAGNOSIS
○ Obtain a barium enema in suspected patients

■ May demonstrate the location of the transition zone

● Between the dilated ganglionic colon and the
distal constricted aganglionic rectal segment
■ Done before instituting rectal irrigations
■ Useful in excluding other causes of distal intestinal
obstruction
■ May show a markedly shortened colon In total colonic
aganglionosis
Hirschsprung’s Disease
● TREATMENT
○ Nonoperative management while awaiting confirmation of dx

■ Rehydration, antibiotics, nasogastric decompression, and

rectal irrigations

○ Multiple stage surgical approach

■ (1) Colostomy: in newborn

■ (2) Definitive pull-through operation: after child is >10 kg

○ Single-staged pull-through (primary pull-through)

■ Using laparoscope
3 Types of Pull-through:
Duhamel Swenson Soave procedure
procedure procedure

Endorectal dissection and

Leaves the rectum Resection with end- removal of mucosa from the
in place and to-end anastomosis aganglionic distal segment
brings ganglionic performed by and bringing the ganglionic
bowel into the exteriorizing bowel bowel down to the anus
retrorectal space ends through the within the seromuscular
anus tunnel
Hirschsprung’s Disease
TREATMENT
○ Anastomosis should be performed at least 5 cm from the

point at which ganglion cells are found

● Main complications of procedures:

○ Postoperative enterocolitis, constipation, and anastomotic

stricture

● Recurrent Hirschsprung’s disease may reflect:

○ Residual aganglionic bowel left behind after the pull-

through
○ Presence of ischemia in the pulled-through segment

leading to ganglion cell loss

Anorectal Malformations
Anorectal malformations
● Spectrum of congenital anomalies that include imperforate
anus and persistent cloaca
● Approximately 1 in 5000 live births
● Males = females
● Failure of descent of the urorectal septum
● Imperforate anus
○ The rectum fails to descend through the external sphincter complex
○ The rectal pouch ends “blindly” in the pelvis, above or below the
levator ani muscle
○ Most cases, the blind rectal pouch communicates more distally with
the genitourinary system or with the perineum through a fistulous
tract
Anorectal malformations
● 2 types depending on whether the rectum ends above or partially
descends through the levator ani:
○ Low type imperforate anus
○ High type imperforate anus

Low High

Male • Fistula to the perineum • Rectum ends as fistula

• Connects with the median into the membranous
raphe of the scrotum or penis urethra
Female • Fistula to the perineum • Persistent cloaca
• Fistula end within the vestibule of
the vagina
Anorectal malformations
Peña Classification: classification system that describes the location
of the fistulous opening
Anorectal malformations

Peña Classification: classification system that describes the location

of the fistulous opening

• Imperforate anus with rectourethral fistula: most common in

defect in males

• Rectovestibular fistula: most common in females

Anorectal malformations
Peña classification describes the location of the fistulous opening.
Anorectal malformations

● ASSOCIATED MALFORMATION
○ Found in 60%
○ Most common: urinary tract defect
○ Skeletal defect: sacrum is commonly involved
○ Spinal cord anomalies in high lesions
○ Esophageal atresia
○ VACTERLL syndrome
Anorectal malformations

MANAGEMENT
○ Wait for some period of time before surgery is undertaken
■ It may take up to 24 hours before the presence of a fistula on the skin is noted
○ Place orogastric tube
○ Monitor for appearance of meconium in perineum or
urine
○ Diagnostics:
■ UTZ of abdomen: for presence of urinary tract anomaly
■ Echocardiogram
■ Spinal radiographs: look for tethered cord
■ Lateral abdominal radiograph: classify low vs high
Anorectal malformations
MANAGEMENT
○ Low lesion: perineal operation w/o colostomy

○ High lesion: colostomy during newborn; pull-through

at 2 months
○ Persistent cloaca: evaluate urinary tract at the time

of colostomy this is to: (1) ensure normal emptying;

(2) determine whether the bladder needs to be
drained by means of a vesicostomy

○ Posterior sagittal anorectoplasty (PSARP procedure)

■ most favored type of pull-through used
Anorectal malformations