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Surgery Rotation > Pediatric Surgery- GI Malformation > Flashcards

Pediatric Surgery- GI Malformation Flashcards

1
Q

Slow colon (FTPM vs. Constipation)

A

Failure to pass meconium: (24-48 hours after birth)

  • def: nothing (stool) comes out in 24-48 hours
  • most infants pass meconium by 24 hours
  • caused by: obstruction anywhere from esophagus to anus (billar emesis)
  • Types:
    1. Imperforate anus (level of obstruction: Anus)
    2. Meconium ileus (AKA: Cystic Fibrosis) (level of obstruction: ileum)
    3. Hirschsprung’s (level of obstruction: RectoSigmoid)

Constipation: (year 2)

  • less than 2 times pass stool per week
  • caused by:
    1. Medication
    2. Diet
    3. Anatomy
    4. Neurologic problem
  • types:
    1. Voluntary holding:
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2
Q

Imperforate anus

A

Def:

  • part of a syndrome called VACTERL
    1. V: Vertebral defect (ultrasound of sacrum)
    2. A: Anorectal malformation/ other bowel atresias (X-ray)
    3. C: Cardiac anomalies (ECHO-cardiogram)
    4. TE: Tracheal-esophageal fistula + esophageal atresia (catheter with X-ray)
    5. R: Renal (voiding cysto-urethrogram)
    6. L: Limb defect (x-ray of wrist)

Sign/symptoms:

  1. No anal opening
  2. Maybe stool present, if fistula present

Diagnosis:
1. X-ray (Cross Table)

Treatment:

  1. Depends if mild or severe
    1. first evaluate child for VACTERL syndrome ( specific procedure)
    * mild: imperforate anal pouch ends are closed together: 1. fix now
* severe: imperforate anal pouch is far from anal verge: 1. Colostomy, 2. wait until child gets bigger to fix
         * *note: reverse colostomy, before they start toilet training
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3
Q

Meconium ilues

A

Def:
1. Associated with Cystic fibrosis

Sign/symptoms:

  1. Failure to pass meconium
  2. No “new-born” screening test performed

Diagnosis:
1. X-ray (to visualize level of obstruction: terminal ileum; & gas-filled plug)
** (soap-bubble) or (ground-glass) appearance of
intestine caused by mixture of meconium/gas
2. Water-contrast enema (gastrografin) for diagnosis

Treatment:

  1. Water enema (gastrografin to dissolve meconium)
  2. If enema does not breakup meconium = surgery required (bowel resection + ileostomy)

Follow-up (F/U):
1. Confirm with chloride test (because if they have CF, need to supplement them with ADEK & pancreatic enzymes; perform pulmonary-hygiene to clear airways of mucus to prevent respiratory infection)

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4
Q

Cystic fibrosis

A

Def:

  1. Mutation in “cystic fibrosis transmembrane-conductance regulator (CFTR)” on chromosome 7
  2. Common mutation (delta F508 Deletion)

Pathophysiology:

  • Dysfunction of the (CFTR) protein found in the epithelial cells of respiratory, gastrointestinal, reproduction system & sweat glands
  • Dysregulation of chloride, bicarbonate & sodium channel transport
  • Decrease water concentration of secretion
  • Impaired clearance

Clinical manifistation:
1. Respiration:
1. Mucous plugging —> chronic airway inflammation & infection
(bronchiectasis)
2. Common pathogen: S. Aureus; H.influenza; P.aeruginosa; B.cepacia

  1. Gastrointestinal:
    1. Intestinal obstruction
    2. Steatorrhea
    3. Pancreatic insufficiency (1st exocrine—> 2nd endocrine) (CF related
      diabetes)
    4. Fat soluble vitamin deficiency (ADEK)
    5. CF related liver disease
  2. Others:
    1. Failure to thrive
    2. Delay puberty & infertility
    3. Dehydration & sodium depletion
    4. Stress incontinence
    5. Low bone mineral density
    6. Clubbing

Investigation:

  1. New born screening test
  2. Sweat chloride test
  3. CF genetic testing

Management:

  1. Airway clearance technique
  2. Nutrition to maintain BMI > 50TH percentile
  3. Pancreatic enzyme supplement
  4. ADEK vitamin supplement
  5. Increase salt intake
  6. Oral glucose tolerance test
  7. No hot tub or humidifier
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5
Q

Hirschsprung’s

A

Def:

  1. Congenital defect
  2. M:F = 4:1
  3. Limited to the rectosigmoid segment of the colon
  4. Can present with an early failure to pass meconium or late constipation

Pathophysiology:

  1. Migration failure of the neural crest cells to the distal colon (inhibitory neurons)
  2. Absence of ganglion cells in the submucosal & myenteric plexuses
  3. Absence of peristalsis in the Aganglionic colon (muscle can’t relax= no stool can enter that space/leaving)
  4. Functional obstruction

Clinical manifistation:
* newborn:
1. Failure to pass meconium int he first 24 hours
after birth
2. Bowel obstruction with bilious vomiting
* older child:
1. Severe chronic constipation
2. Failure to thrive, malnutrition & feeding problem

Physical examination:

  1. Abdominal destination
  2. Digital rectal exam:
    • empty rectum
    • explosive ejection of stool as the finger is
      removed (squirt sign)

Investigation:
1. TSH (rule out hypothyroidism)
2. Electrolytes (rule out hypercalcemia, hypokalemia)
3. Abdominal X-ray (shows dilated loops of intestine; good colon is dilated & bad colon looks normal)
4. Contrast enema (shows “transition zone” between narrow rectum/distal colon & dilated proximal colon
** contraindicated if enterocolitis is suspected
(risk of perforation)
** for infants ( 12 mths): anal manometry shows
absence of recto-anal inhibitory reflex
(increased tone)
** lack of this reflex in patient with chronic
constipation indicates Hirschsprung’s
Diagnosis:
1. Rectal biopsy: shows Aganglionosis, hypertrophy of nerve fiber/trunks & abnormal Calretinin staining

Treatment:
1. Surgical “pull-through” procedure (removal of the Aganglionic segment -the segment that looks normal on X-ray- & anastomosis between the normally innervated part of the intestine & the anus, preserving the anal sphincter)

Hirschsprung’s associated enterocolitis:

  1. Life-threatening complication
  2. Inflammation of the colon, which causes bowl obstruction & sepsis
  3. Can occur before or after “pull-through” surgery

Sign/symptoms:

  1. Abdominal dissension
  2. Explosive diarrhea
  3. Vomiting
  4. Others: fever, rectal bleeding, shock

Diagnosis:
1. X-ray: megacolon (severely dilated colon)

Treatment:

  1. Broad spectrum antibiotics
  2. IV fluid
  3. Labs: CBC, CRP, Blood culture
  4. Nasogastric decompression
  5. Rectal irrigations (wash out colon of stool & prevent stasis)
  6. General surgery consultation
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6
Q

Voluntary holding

A

Pathway:

  1. Pain
  2. Embarrassment
  3. Congenital impairment (at higher risk)

Sign/symptoms:

  1. Overflow of incontinence
  2. Encopresis ( stooling in the bed)

Diagnosis:
1. Clinical (check bowel habits)

Treatment:

  1. Bowel regimen: stool softener & motility agent
  2. Change behavior (tell kid it is okay to poop)
  3. Dis-impaction: done under anesthesia for kids
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7
Q

Congenital anomalies in newborn infants

A

*Upper GI tract:

  1. Anatomic relation: proximal to ligament of Treitz
  2. Embryonic source: foregut
  3. Blood supply: celiac axis
  4. Viscera: esophagus, stomach, duodenum, biliary ducts, liver, pancreas

*Lower GI tract:

  1. Anatomic relation: distal to ligament of Treitz
  2. Embryonic source: midgut
  3. Blood supply: SMA
  4. Viscera: jejunum, ileum, cecum, ascending colon, proximal 2/3 of transverse colon
    ———————————————
  5. Anatomic relation: distal to ligament of Treitz
  6. Embryonic source: Hindgut
  7. Blood supply: IMA
  8. Viscera: Distal 1/3 of transverse colon, descending colon, sigmoid, rectum , anal canal
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8
Q

Foregut disorders

A

Types:

  1. Esophageal atresia +/- tracheal fistula
  2. Duodenal atresia
  3. Pyloric stenosis
  4. Biliary atresia
  5. Choledochal cysts
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9
Q

Midgut disorders

A

Types:

  1. Small intestine atresia
  2. Malrotation & midgut volvulus
  3. Meckel’s diverticulum
  4. Omphalocele & gastrochisis
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10
Q

Hindgut disorders

A

Types:

  1. Hirschsprung’s disease
  2. Anorectal malformation/imperforate anus
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11
Q

Esophageal atresia (EA) (+/-) tracheal-esophageal fistula (TEF)

A

Embryology:

  • 4th week of gestation: ventral foregut differentiate into esophageal & trachea
  • 9th week of gestation: muscular/neurovascular development of the esophageal is completed
  • Esophageal malformation can occur within this period

Types:

  1. Type A: proximal EA with distal TEF (common)
  2. Type B: only proximal EA (no fistula)
  3. Type C: only TEF (no atresia)
  4. Type D: both a proximal & distal TEF in the setting of atresia (rare)
  5. Type E: proximal EA with TEF & distal esophageal pouch (rare)

Clinical presentation:

  1. Drooling
  2. Attempts at feeding causes: coughing, choking, regurgitation
  3. Type B/E (Proximal obstruction): scaphoid abdomen (anterior wall of abdomen is sunken & hollow) & gas is not seen in distal bowel
  4. Type C: recurrent aspiration pneumonia (food, saliva, liquid, vomit inhaled into lung) (diagnosed late in life)

Diagnosis:

  1. Prenatal ultrasound (shows polyhydramnios & blind end of esophageal pouch)
  2. After birth: failure to pass an oro- OR naso- gastric tube (tip of the tube is seen in the esophageal pouch with radiography)
  3. Further investigation of VACTERL phenomena ( Ventricular septal defect is most common anomaly with EA/TEF)

Management:

  1. Surgical correction
    * single procedure:
    * staged procedure: decompressive gastrostomy & fistula takedown, followed by esophageal reconstruction at later date.

Outcome:
Lifelong issues:
1. Gastro-esophageal reflex (GER)
1. Asymptomatic
2. Symptomatic (persistent cough, respiratory
problem, or esophageal stricturing)
3. Manage with: anti-reflux drugs & pro-kinetics

  1. Esophageal stricture
    1. Can form many years after repair of (EA/TEF)
    2. Managed with:
      1. endoscopic dilation
      2. Recurrent/refractory: surgical resection & re-
        anastomosis

Note:

  • infant with scaphoid abdomen:
    1. can indicate congenital diaphragmatic hernia
    2. Esophageal atresia (type B/E with no passage of air into GI tract)
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12
Q

Duodenal atresia

A

Embryology:

  1. Failure of the duodenal recanalization
  2. Most common at: second portion of duodenum distal to ampulla of Vater

Clinical presentation:

  1. First 24-48 hrs: emesis & feeding intolerance
    • non-bilious vomiting: atresia above Ampulla of
      vater
      *bilious vomiting (green): atresia below Ampulla
      of vater
  2. Palpable mass at the epigastrium
  3. No abdominal distention

Diagnosis:

  1. X-Ray: “double-bubble” (air in stomach & proximal duodenum)
  2. UGI series: (to rule out malrotation; present with bilious emesis; surgical emergency)
    * shows: duodenal web (intraluminal diverticulum; windsock sign)
  3. Echocardiogram & renal ultrasound: (rule out other defects -trisomy 21)

Management:
1. Surgical correction via Diamond-Duodenodenostomy (transverse incision at proximal duodenum + vertical incision distal to it + anastomosis)

Outcome:

  1. Persistent obstruction (indicates missed duodenal web & required re-operation)
  2. Delayed gastric emptying (in early post-operative period; resolved by time)
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13
Q

Pyloric stenosis

A

Embryology:

  1. Etiology is unknown
  2. Exposure to erythromycin (antibiotics) (risk factor)

Clinical presentation:

  1. Non-bilious emesis (projectile) (level of obstruction: pylorus, proximal to ampulla of vater)
  2. Feeding intolerance
  3. Present at 2-4 weeks of life & until 6-12 weeks
  4. First born Caucasian male

Diagnosis:
1. Physical examination: Palpable & mobile “olive-like” mass on epigastrium or RUQ; Soft & non-distend abdomen

  1. Ultrasound: pyloric channel length >16 mm, wall thickness >4 mm
  2. Lab: Hypocholoremia, hypokalemia metabolic alkolosis, dehydration (repeated vomiting of gastric acid - HCL; hypovolemia stimulates aldosterone secretion causing Na resorption & K secretion)
  3. Acidic urine “paradoxical aciduria” ( worsening hypokalemia stimulates renal H+/K+ pump to resorb K & secretes H)

Management:

  1. Not surgical emergency (operative intervention is delayed until electrolytes have normalized first, Cl > 95, Bicarbonate <30)
  2. Resuscitation (volume & gastric juice loss —> 10-20 cc/kg normal saline bolus)
  3. K-containing fluid (D5 1/2NS + 10 K/L) (at maintenance rate)
  4. Pyloromyotomy + laparoscopy (longitudinal incision along anterior surface of pylorus + inner lining bulging through incision) + oral feeding initiated 6-8 hrs post-operative

Outcome:
1. Incomplete myotomy: shows persistent feeding intolerance in peri-operative period (requires re-operation)
1.

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14
Q

Biliary atresia

A

Embryology:

  1. From 4-10 weeks of gestation: normal, Extra-hepatic biliary tracts develop from hepatic diverticulum.
  2. In post-natal period: inflammatory process causing fibrosis of the extra-hepatic biliary duct

Clinical presentation:

  1. During the first 2 weeks after birth: worsening jaundice
  2. Lab: direct hyperbilirubinemia + elevated Alkaline phosphate (biliary obstruction)
  3. Sign of Cholestasis
  4. Dark urine & light/gray stool

Diagnosis:

  1. (99-Tc IDA): shows technetium taken up by liver, but obstruction of extra-hepatic duct (due to biliary atresia) prevents outflow of radio-tracer into duodenum.
  2. Abdominal ultrasound: show small/obliterated gallbladder
  3. MRCP: (rule out intra-hepatic atresia or choledocal cysts)

Management:
1. Kasai porto-enterostomy: best performed in infants < 60 days

Outcome:
1. Successful at early intervention (infant <60 days)

*Post -operative complication:
2. Require liver transplant due to liver failure
* presence of intra-hepatic atresia
* fat-soluble (ADEK) vitamin deficiency (failure to
thrive & variceal bleeding secondary to portal
hypertension)
3. Cholangitis: decrease bile flow (elevated total bilirubin, fever, leukocytosis) (managed with: IV antibiotic & fluid resuscitation)

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15
Q

Choledochal cysts

A

Embryology:

  1. Etiology is unknown
  2. Abnormal pancreaticobiliary junction near duodenal wall

Types:

  1. Type 1: saccular/fusiform dilation of CBD (intrahepatic ducts are normal) (common)
  2. Type 2: isolated CBD diverticulum
  3. Type 3: choledochocele (cystic dilation of the supra-duodenal CBD, prior to its junction with pancreatic duct)
  4. Type 4: intra- & extra-hepatic bile ducts are dilated
  5. Type 5: only intra-hepatic duct is dilated.

Clinical presentation:

  1. Symptoms of biliary obstruction (progressive jaundice, dark urine, light colored stool)
  2. Palpable & Tender abdominal mass in RUQ
  3. Can present with: cholangitis or pancreatitis

Diagnosis:

  1. Lab: elevated direct bilirubin & alkaline phosphate
  2. MRCP ( test of choice; 1st)
  3. Abdominal ultrasound or 99-Tc IDA

Management:

  1. Risk for cholangiocarcinoma (highest in type 1 & 4)
  2. Excision of choledocal cysts (depends on the type)
  3. Liver transplant

Outcome/ complication:

  1. Biliary tract malignancy (occurs with incomplete excision)
  2. Cholangitis
  3. Stricture formation
  4. Choledocolithiasis
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16
Q

Small intestine (jejunoileal) atresia

A

Embryology:

  1. During 5th week of gestation: development of midgut
  2. Ischemic event can lead to jejunoileal atresia

Clinical presentation:

  1. Structural obstruction (failure to pass meconium in the first 24-48 hrs)
  2. Bilious emesis
  3. Abdominal distention

Diagnosis:

  1. Abdominal X-ray: shows dilated proximal bowel to the site of obstruction with collapsed loop & paucity of air in the distal bowel.
  2. Contrast enema: shows abrupt transition from the filling to non-filling segments of small bowel + small colon < 1 cm diameter due to lack of use
  3. UGI series: to rule out malrotation causing bilious emesis

Management:

  1. Bowel decompression
  2. Fluid resuscitation
  3. Resection of atresic segment with end-to-end anastomosis

Outcome:

  1. Intestinal dysmotility (few weeks after repair)
  2. Short bowel syndrome: require parental nutrition (carries risk for sepsis & liver damage)
17
Q

Malrotation & midgut volvulus

A

Embryology:

  1. Failure of the normal 270 degree rotation & fixation of the midgut
  2. Lack of rotation places the duodenum/small bowel to the right of midline & the large bowel to the left.
  3. Ladd’s band (lateral peritoneal attachment) attaches cecum (anterior to the duodenum) to the abdominal wall & compresses the duodenum (causing obstruction & bilious emesis)
  4. The root of mesentery is narrowed & act as fulcrum , in which the bowel can twist around (volvulize): kinking SMA & causing ischemia

Clinical presentation:

  1. First week of life: feeding intolerance & bilious emesis
  2. Abdominal rigidity & overlying erythema (signs of peritonitis= indicates ischemic bowel)
  3. No abdominal distention

Diagnosis:

  1. Abdominal X-ray:
  2. UGI series: contrast exists pylorus & descending straight down to the right of the midline into small bowel

Management:

  1. Fluid resuscitation
  2. Bowel decompression (oro- or naso-gastric tube)
  3. IV- antibiotics
  4. emergency surgery intervention: Ladd’s band is incised to released obstruction; any necrotic bowel segment is resected; healthy/viable bowel are anastomosed.

Outcome:
1. Adhesive small bowel obstruction (occurs years later)

18
Q

Meckel’s diverticulum (true diverticulum)

A

Embryology:

  1. Between week 5-7 of gestation: Failure of the omphalomesenteric (vitelline) duct to completely involute.
  2. Most common congenital GI malformation
  3. true diverticulum: involve all 4 layers of the bowel

Clinical presentation:

  1. Painless lower GI bleeding in children (brisk & bright red)
  2. Present by age of 2-12
  3. Male > female
  4. Anemia
  5. Abdominal pain & distention
  6. Inability to pass flatus (gas) or move bowel

Diagnosis:

  1. Lab: low Hg (anemia)
  2. Abdominal pain & distention
  3. Inability to pass gas
  4. Meckel’s scan: localize bleeding ulcer (diverticulum located 2 feet proximal to ileocecal valve, contain heterotropic mucosa -gastric & pancreatic origin)
    * *ulceration/bleeding occur secondary to acid secretion from the heterotropic mucosa

Management:

  1. Ileal resection with primary anastomosis: if bleeding is the primary symptom
  2. Segmental resection: in case of complicated diverticulitis, perforation, obstruction, volvulus, or if base of the diverticulum is narrow or if diverticulitis does not involve the base.

Outcome:
1. Successful

19
Q

Omphalocele & gastroschisis

A

Embryology:

  1. Congenital defects of the abdominal wall
  2. Associated with malrotation

Clinical presentation:
* Omphalocele:
1. midline abdominal wall defect
2. larger defect (from 2-15 cm): allow for herniation
of liver & spleen
3. Herniated organs covered by amniotic sac
4. Higher association with chromosomal abnormalities
* Gastroschisis
1. right of the umbilicus- abdominal wall defect
2. smaller defect (<3 cm)
3. Herniated small bowel not covered by amniotic
sac; exposure to amniotic fluid lead to thickened
& fibrotic mesentery
4. Intestinal atresia

Diagnosis:

  1. Prenatal ultrasound
  2. Chest radiography, Echocardiogram, renal ultrasound & Karyotyping (rule out other anomalies, in case of omphalocele)

Management:

  1. Infant placed under warmer, fluid resuscitation, urinary catheter to monitor volume status
  2. Bowel decompression
  3. Intestinal content wrapped in a moist, sterile plastic dressing to prevent evaporative losses
  4. Surgical reduction: Return the herniated content into abdominal cavity & close the defect

Outcome:

  1. Gastroschisis complication with intestinal atresia:
    1. Dysmotility
    2. Malabsorption
    3. Necrotizing enterocolitis

Surgery Rotation (7 decks)

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