Pediatrics

Is bilious vomiting a surgical emergency in the newborn and why?

• Must rule out malrotation with mid-gut volvulus in a patient with proximal obstruction because the gut can be strangulated (time = bowel).

• Differential diagnosis—(memorize this list—the ABSITE will point you toward one of these!) annular pancreas, malrotation, jejunoileal atresia, meconium ileus, meconium plug syndrome, duodenal web/atresia, Hirschsprung, hypoplastic left colon. Always remember 1 anomaly begets additional associated anomalies.

Meconium ileus is associated with what anomaly?

• Cystic fibrosis

Duodenal atresia and malrotation are associated with what anomaly?

• Down syndrome

What other abnormalities are associated with malrotation?

• Diaphragmatic hernia, jejunoileal atresia, abdominal wall defects

Which abdominal wall defects are associated with malrotation?

• Both omphalocele and gastroschisis

What is the diagnostic study of first choice to rule in malrotation with midgut volvulus?

• Upper gastrointestinal

Surgical procedure for malrotation:

• Ladd procedure

• Counterclockwise rotation, divide peritoneal bands crossing the duodenum, position SB to right and colon to the left

• Look for other abnormalities (see above)

• Appendectomy (cecum is now on the left side of the abdomen, perhaps delaying the diagnosis of acute appendicitis.)

Why do patients with malrotation have bilous vomiting?

• Ladd bands cross the duodenum

• Remember that BILIOUS vomiting in newborn is malrotation until proven otherwise. (most common presentation)

Why is blood flow to the bowel compromised in malrotation?

• SMA twisted on its axis—detorsion counterclockwise will relieve this problem. (Malrotation results from failure of normal counterclockwise rotation in utero.)

Duodenal atresia—diagnosis and treatment (Rx):

• "Double bubble sign"

• Associated anomalies? Prematurity, polyhydramnios (most common), malrotation, annular pancreas, biliary atresia

• Resuscitation and then duodenojejunostomy

Rx of duodenal atresia:

• Decompression and resuscitation first and then duodenoduodenostomy or duodenojejunostomy

Rx of jeunoileal atresia:

• Decompression and resuscitation first then resect atretic portion.

• (Run the bowel [common ORALS mistake])!

Patient fails to pass meconium in first 24 hours, bilious vomiting, perforate anus, abdominal distention. "Babygram" shows terminal ileum is dilated; bowel contents have ground glass/soap bubble appearance without air/fluid levels. What is the diagnosis and Rx?

• Simple meconium ileus

• Rx is gastrograffin enema, pancreatic enzymes, and mucomyst

• Get a chloride sweat test post-op

• If patient is toxic → (Complicated meconium ileus) resuscitate and go to the OR (Don't mess with mucomyst when a patient is toxic!).

What is the difference between "meconium plug" and "meconium ileus?"

• Meconium ileus is a misnomer. It is actually a distal obstruction. Meconium plug may be secondary to maternal condition, Hirschsprung or cystic fibrosis. It is also a distal obstruction. With passage of the meconium, the obstruction may be relieved.

Called to see a newborn, 27 weeks' gestation, acidotic, fever, free air on AXR, first stool was bloody. Diagnosis and Rx:

• Necrotizing enterocolitis (NEC)!

• Make the ABSITE push you to the OR on this one.

Absolute indication for OR:

• Free air

• Relative indications for OR. Clinical deterioration, worsening acidosis, persistent fixed loop on abdominal x-ray (AXR), thrombocytosis, portal venous air, abdominal wall erythema/cellulitis, positive paracentesis cultures. Portal venous gas and pneumatosis alone are not absolute indications for OR.

• Nonoperative management is NPO, IVF, nasogastric tube (NGT), broad-spectrum IV antibiotics, correct coagulopathy, correct electrolytes, appropriate resuscitation, serial AXR, and bedside evaluations.

Operative management options:

• In the neonatal intensive care unit: On high-frequency jet ventilator and can't be moved to OR ± extreme prematurity (<1000 g). Peritoneal drain at the bedside.

• In the OR: Exploratory laparotomy, resection of dead bowel, bring up ostomies

Before establishing continuity, what study is needed and why?

• A distal limb study because of the high incidence of associated strictures.

What is the expected urine output in this patient with NEC?

• 2 cc/kg

Called to L&D to see a patient with bowels outside the abdomen from defect to right of umbilical cord. Diagnosis and Rx:

• Gastroschisis

• The first step is always resuscitation.

• Cover bowel with moist Kerlix, bowel bag.

• "Bad bowel, good baby"—only 10% with associated anomalies. The bowel was exposed to amniotic fluid and looks bad.

• Remember they will all have malrotation.

• Place bowel in silo and slowly cinch the silo bag over time. The bowel may look congested/ purplish in the silo.

• Do not try to reduce all viscera into the abdomen at the first operation—will result in respiratory distress and possibly abdominal compartment syndrome

Associated anomalies with omphalocele and gastroschisis:

• Gastroschisis—intestinal atresia, malrotation

• Omphalocele—skeleton, gastrointestinal (GI) tract, nervous system, genitourinary, GI tract. The most common and worrisome is cardiac, so get a STAT echo before going to the OR.

Which condition is always associated with a sac, gastroschisis, or omphalocele?

• Omphalocele always has a sac (may be ruptured); gastroschisis never has a sac

Where can the umbilical cord be found in gastroschisis compared to omphalocele?

• In gastroschisis the umbilical cord if found to the left of the defect; in omphalocele the umbilical cord is attached to the sac

Infant with poor PO intake, life-threatening episodes of apnea (near miss SIDS), chronic cough, arching, recurrent pneumonia, asthma, hoarseness. Most likely diagnosis:

• Gastroesophageal reflux disease (GERD)

Preoperative evaluation for GERD:

• UGI—exclude other causes of vomiting

• Gold standard is pH monitoring.

• Endoscopy of esophagus, larynx, and trachea

Management of GERD:

• Nonoperative: Thicken formulas, small feeds, acid suppression, modify posture

• Operative: Nissen fundoplication

• Indication for operative management: Failure of medical management, multiple aspiration, near miss SIDS, esophageal stricture, FTT

Most common TEF:

• Gross type C (Type C—90%, Type A—7%, Type E—3%)

Describe Type C TEF:

• Proximal EA (blind pouch)—distal tracheoesophageal fistula (TEF)

What is the 2nd most common TEF?

• Type A

Describe Type A TEF:

• Pure EA—no TEF

What is the common presentation of Type A TEF?

• Newborn spits up feeds, excessive drooling, respiratory symptoms with feeds. NGT won't pass.

AXR finding with Type A TEF:

• Gasless abdomen, possibly with a scaphoid abdomen

Describe Type E TEF:

• Normal esophagus—distal TEF

Most likely TEF to present in adulthood:

• Type E

TEF is part of the VACTERL group of anomalies. Describe VACTERL:

• Vertebral (ultrasound), Anorectal (rectal examination, imperforate anus), Cardiac (ECHO), TE Fistula, Renal (check renal ultrasound), Limb anomalies

Surgical management of TEF:

• Rigid bronchoscopy, right extrapleural thoracotomy, divide fistula, close trachea, primary repair of esophagus, azygous often divided, ± G-tube. Prior to OR—Sump (NGT) for oral secretions, elevated head of bed, antibiotics, resuscitate, H2 blockers, NPO. Esophagogram to identify length of pouch

Patient with diaphragmatic hernia on the left. Do you expect both lungs to be dysfunctional or just the left?

• Both are usually dysfunctional secondary to primary pulmonary hypertension

What percent of diaphragmatic hernia present on the left?

• 85%

Percent of diaphragmatic hernias associated with other congenital defects?

• 80%. Again, get an ECHO for associated cardiac defects before going to the OR!

Most common diaphragmatic hernia—located posterior-lateral:

• Bochdalek

Rare diaphragmatic hernia—located anteriormedially, posterior to sternum:

• Morgagni

When are most diaphragmatic hernias identified?

• On prenatal ultrasound (typical presentation—no gastric bubble, bowel in chest, polyhydramnios)

NGT placed in child with Bochdalek hernia. CXR shows:

• NGT in chest. Bowels in chest.

Failure of the diaphragm to fuse is called:

• Eventration

What branchial cleft cyst is anterior to sternocleidomastoid muscle?

• 2nd branchial cleft cyst

Where does a 2nd branchial cleft cyst usually pass?

• Fistula usually passed through carotid bifurcation and into tonsillar pillar.

Most common branchial cleft cyst?

• 2nd

Lateral neck, lymphatic malformation:

• Cystic hygroma (75% present in posterior neck, 20% in the axilla)

Which branchial cleft cyst presents clinically as a firm mass in suprasternal notch or clavicular region?

• 3rd

The 1st branchial cleft cyst is often associated with which nerve?

• 1st branchial cleft cyst found at angle of mandible is often associated with facial nerve.

Midline neck mass draining pus:

• Infected thyroglossal duct cyst. Give antibiotics to clear infection then Sistrunk procedure (excise tract and central portion of hyoid bone)

Patient presents with cervical lymphadenopathy suspected to be from URI who subsequently fails to respond to 10 days of antibiotics. Possible etiology:

• Lymphoma (if it persists). Always check the scalp for a hidden infection.

Patient presents with head persistently rotated. Head noted to be rotated in the opposite direction of the affected muscle. Diagnosis and Rx:

• Torticollis. ROM exercises (physical therapy)

What size airway?

• Uncuffed endotracheal tube if less than 8 to 10 years of age. Size is (4 + pt age)/4. Or can use the size of their pinkie as a clue! Remember, no cricothyroidotomy less than 12 years of age

IVF bolus? How much?

• 20 mL/kg. Blood if no response to 2 IVF boluses

Number 1 cause of death for children ages 1 to 20 years of age:

• Trauma

Patient's mother gives a history of a fall that does not match the developmental stage of the child:

• Suspect child abuse

Patient presents with history of fall from 15 feet, hypotensive, distended abdomen, open long bone fracture, blood draining from both ears, and an EMS report of decreased breathe sounds on the right. Next step in management:

• Airway (ABC's)

Best indicator of shock:

• Capillary refill/tachycardia. Pediatric trauma—children will look OK and then suddenly become very unstable.

Ultrasound findings:

• Pyloric wall thickness

• Greater than 3 mm thick

• Pyloric channel—greater than 15 mm long. All bilious vomiting does not equal pyloric stenosis. (For example, 20% of duodenal atresia occurs before the ampulla.)

Paradoxical aciduria:

• Know the mechanism. Remember that they are volume-contracted and have lost H⁺ in their vomit. The metabolic alkalosis and dehydration are the first things you need to treat. Use 0.9% NaCl boluses. When the baby is urinating, start 0.5% NaCl at 1.5 maintenance. Then replace the K⁺. Again, we talk about the importance of K⁺ levels in these patients, but you need a normal HCO₃⁻ before going to the OR. This is a semielective procedure.

Electrolyte findings:

• Hypokalemic, hypochloremic, metabolic alkalosis

3-year-old presents with a history of inconsolable crying from abdominal pain followed by periods in which they are happily playing. What is the diagnosis?

• Intussusception

How to make diagnosis?

• Start with plain AXR, then ultrasound

What are you looking for on ultrasound?

• Target sign

Etiology:

• Lead point is usually Peyer patches (classically secondary to viral gastroenteritis, URI, or administration of rotavirus vaccine).

When to operate?

• Again, if the patient is toxic, resuscitate then go to the OR. Failure of nonoperative management with hydrostatic reduction/contrast or air enema. Bloody stool is not an indication for OR but ominous sign.

Success rate with nonoperative reduction:

• 80%

Push or pull?

• Always push bowel out. Never pull bowel out. Recurrence after surgical reduction is low.

Incidental finding of Meckel:

• Resect

What is intussusception?

• Telescoping/invagination of a proximal segment of bowel into an adjacent distal segment of bowel

What is the most common cause of intestinal obstruction in infants/children ages 9 months to 4 years?

• Intussusception

Where does intussusception most commonly occur?

• At or near the ileocecal valve (ileocolic intussusception)

What percentage of pediatric intussusception cases are considered idiopathic?

• 95%

How many attempts at reduction of intussusception are usually tried before stopping?

• 3 attempts at reduction, may attempt more if progress being made

How long should each attempt at reduction of intussusception be in duration?

• No longer than 3 minutes

When should a patient with intussusception undergo surgical exploration?

• Presence of peritonitis and small bowel obstruction; incomplete reduction with retrograde enema; bowel perforation during diagnostic/therapeutic enema

What other structure is often routinely removed when operating on a patient with intussusception?

• The appendix

What percentage of infants experience recurrent intussusception after either nonoperative or operative reduction?

• ~5% of infants

When trying to reduce an intussusception, what is the maximum barium column height or maximum air pressure that should be used?

• Maximum column height of barium: 1 m

• Maximum air pressure: 120 mm Hg

Caloric intake for 0 to 1 year of age?

• 90 to 120 kcal/kg/d

• Breast milk and formula is 20 kcal/ounce = 20 kcal/30 cc = 0.68 kcal/cc.

• Infants should gain 30 g/d or about 1% of their body weight per day. For example, a 2-week-old, 1-kg, 34-week-gestation infant should gain about 10 g/d.

• Weight gain is an important indicator of sufficient caloric intake.

• Requirements for protein and calories are much higher for infants.

Other ages (not high-yield):

• 1 to 7 years: 75 to 90 kcal/kg/d

• 7 to 12 years: 60 to 75 kcal/kg/d

• 12 to 18 years: 30 to 60 kcal/kg/d

Temperature? Higher risk for hypothermia—large BSA.

• Respond by nonshivering thermogenesis and mobilization of brown fat

Immune system:

• Relatively immunodeficient, IgA from mother's breast milk. IgG is the only immunoglobulin to cross the placenta.

Woman in trauma bay, 30 weeks pregnant, with strong, regular contractions, cervical dilation without vaginal bleeding on speculum examination. Diagnosis and management? Patient is in labor-strong, regular contractions with cervical dilatation:

• General considerations: Check ultrasound (fetal position, previa, abruption, etc), give steroids to help if premature lungs, tocolytics, hydration (number one cause of preterm labor is dehydration/hypovolemia!), antibiotics for GBS, check ratio of lecithin to sphingomyelin (L/S), check Rh, speculum examination before bimanual. Give mom oxygen and put the LEFT SIDE DOWN—insisting on putting the left side down to get the baby off the IVC. This alone will dramatically improve outcomes.

Insensible losses in an infant:

• Higher! Notice the difference below (don't memorize)

• Premature infant (<1500 g)—45 to 60 mL/kg/d

• Term infant—30 to 35 mL/kg/d

• Adult—15 mL/kg/d

Best indicator of adequate fluid intake:

• Urine output: 1 to 2 mL/kg/d

• Weigh diapers and weigh the baby

• Urine osmolarity? Decreased. Max concentration for infant is 700 mOsm/kg

• Know the 4, 2, 1 rule for maintenance fluids

• No K⁺ to any neonate/infant until making urine and potassium within normal serum levels

What structures are derived from the foregut?

• Lungs, esophagus, stomach, pancreas, liver, gallbladder, bile duct, duodenum proximal to ampulla of Vater.

What structures are derived from the midgut?

• Duodenum distal to ampulla, small intestine, colon to distal one-third of transverse colon.

What structures are derived from the hindgut?

• Distal one-third of transverse colon to anal canal

What is the primary energy substrate for the fetus?

• Glucose

What might you see with a linoleic acid deficiency?

• Dryness/thickening of the skin, hair loss, and delayed wound healing

What is the most important carbohydrate source for infants?

• Lactose

What is the most common cause of respiratory distress in neonates?

• Respiratory distress syndrome (hyaline membrane disease)

What is the most common index of lung maturity?

• The ratio of lecithin (phosphatidylcholine) to sphingomyelin in amniotic fluid (L/S ratio):

• L/S ratio = 2.0, normal with extremely low risk of RDS

• L/S ratio = 1.5 to 2.0 considered "immature" with low risk

• L/S ratio <1.5 increased risk

• L/S ratio <1.0 high risk

What drug inhibits prostaglandin synthetase and promotes closure of a patent ductus arteriosus if given within the first 2 weeks of life?

• Indomethacin

Describe the fetal circulation:

• Well-oxygenated blood from the placenta/umbilical cord passes through the hepatic ductus venosus → IVC → Right atrium.

• More oxygenated blood flows through foramen ovale into left atrium where it is pumped by the left ventricle into the aortic arch to supply the heart, brain, and upper body.

• Less-oxygenated blood is pumped into pulmonary artery by right ventricle and shunted through ductus arteriosus into descending aorta, which then supplies the organs of the lower body and also gives blood back to the placenta through 2 umbilical arteries.

What is the most serious complication of neck masses in children?

• Airway obstruction

What is the recurrence rate for thyroglossal duct cyst after the Sistrunk procedure?

• <10%

What percentage of ectopic thyroid tissue is found to have thyroid neoplasia?

• <1%

What is the most common presentation of a thyroglossal duct cyst?

• A midline cystic neck mass versus a draining sinus

How might a thyroglossal duct cyst be differentiated from a branchial cleft cyst on physical examination?

• A thyroglossal duct cyst often moves with protrusion of the tongue and swallowing because the cyst and tract are attached at the foramen cecum at the base of the tongue; branchial cleft cysts should not change position with tongue movement.

What is the most common deformity of the anterior chest wall?

• Pectus excavatum

What is pectus excavatum?

• A posterior curve in the body of the sternum from the manubrium to the xiphoid, resulting from abnormal regulation of costal cartilage growth

What do patients with pectus excavatum typically complain of clinically?

• Chest pain or shortness of breath with exertion or both

What is the pectus index?

• The ratio of the maximum internal transverse diameter of the thorax to the minimum sternovertebral distance

What is the optimal timing for repair of pectus excavatum?

• Late childhood and early adolescence (ossification of key components of anterior chest wall not completed until late teenage years)

What procedure used to treat pectus excavatum utilizes a rigid U-shaped bar inserted transversely across the chest to push the deformity back into place?

• The Lorenz bar/Nuss procedure

What chest wall defect features a protrusion of the sternum and ribs?

• Pectus carinatum

What is included in Cantrell pentology?

• Distal sternal cleft, omphalocele, (morgagni) diaphragmatic defect, pericardial defect, and intracardiac defect

What is a pulmonary sequestration?

• Abnormal lung tissue that may or may not communicate with the trachea/bronchus

What is the usual vascular supply to an extralobar pulmonary sequestration?

• Arterial blood supply: Systemic (frequently from aorta below diaphragm)

• Venous drainage: Systemic

What is the usual Rx for a pulmonary sequestration?

• Thoracotomy and lobectomy

What is the usual vascular supply to an intralobar pulmonary sequestration?

• Arterial blood supply: thoracic or abdominal aorta

• Venous drainage: pulmonary vein (can be systemic)

How may intralobar pulmonary sequestration present during childhood?

• As recurrent localized pneumonia

What are the 3 stages of empyema?

• Stage I: Exudative: Thin low cell count free-flowing fluid

• Stage II: Fibrinopurulent: Encasement of lung parenchyma with thick purulent material with multiple loculations

• Stage III: Organizing: A fibrotic peel from fibroblasts growing into the exudate

What are the 3 most common pathogens in pediatric pneumonia?

• Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus

What pleural fluid cell count differential makes the diagnosis of chylothorax in patients who are not being fed?

• >90% lymphocytes

What may be administered to a patient prior to the OR that will make identification of the thoracic duct and an associated leak more obvious?

• Cream

Are inguinal hernias more common in males or females?

• Males (6:1)

How might acute, noncommunicating hydrocele be distinguished from incarcerated inguinal hernia?

• Absence of symptoms of bowel obstruction; can usually palpate normal cord structures above the scrotal mass and transilluminate the hydrocele

When might you perform operative exploration of the contralateral asymptomatic groin in a pediatric patient with an inguinal hernia?

• Controversial, but consider in males younger than 2 years (60%–70% incidence of a contralateral patent processus vaginalis) or female up to age 4.

What complications may follow repair of a pediatric inguinal hernia?

• Complications from general anesthesia; wound infection; recurrence; injury to vas deferens/testicular vessels

What is a hydrocele?

• A fluid collection in the processus vaginalis in the inguinal canal or the tunica vaginalis in the scrotum

What is the difference between a communicating versus a noncommunicating hydrocele?

• A communicating hydrocele features a direct patency between the hydrocele and the peritoneal cavity, whereas a noncommunicating hydrocele does not increase or decrease in size over time.

How might a communicating hydrocele be distinguished from a noncommunicating hydrocele by history?

• The communicating hydrocele will generally have a history of fluctuating size.

When should a communicating hydrocele be repaired?

• Shortly after they are diagnosed (like an inguinal hernia)

When is operative management of a noncommunicating hydrocele indicated?

• Acutely enlarging hydrocele; lesions that persist after age 12 months (usually spontaneously resolve before this time); question of communication of the hydrocele

What causes of neonatal intestinal obstruction have a higher incidence in infants with trisomy 21 compared to the general population?

• Duodenal atresia/stenosis; Hirschsprung disease

What are the 2 most common anorectal malformations observed in males?

• Low imperforate anus with perineal fistula; high anorectal agenesis with rectoprostatic urethral fistula

What is the most common anorectal malformation observed in females?

• Low imperforate anus with fistula from rectum to either perineal body or vaginal vestibule

Which anorectal malformation has a more favorable prognosis for fecal continence: low, intermediate, or high anorectal lesion?

• Low has a more favorable prognosis for fecal continence

What structure is used to delineate a low from high anorectal malformation?

• Pubococcygeal line

What is the most common associated abnormality with anorectal malformation?

• Renal anomaly (including both upper and lower tract)

What types of anorectal malformations are considered high lesions in females?

• Anorectal agenesis with/without rectovaginal fistula; rectal atresia

What types of anorectal malformations are considered intermediate lesions in females?

• Rectovestibular fistula, rectovaginal fistula, anal agenesis without fistula

What types of anorectal malformations are considered low lesions in females?

• Rectovestibular fistula, anal stenosis

What types of anorectal malformations are considered high lesions in males?

• Anorectal agenesis with/without rectoprostatic urethral fistula, rectal atresia

What types of anorectal malformations are considered intermediate lesions in males?

• Rectobulbar urethral fistula, anal agenesis without fistula

What types of anorectal malformations are considered low lesions in males?

• Anocutaneous fistula, anal stenosis

What do infants with an intermediate, high, or indeterminate anorectal malformation generally require as initial surgical management?

• Divided diverting proximal sigmoidostomy, mucous fistula to study distally

When is anorectoplasty generally performed in an infant with an intermediate or high anorectal malformation?

• 8 to 12 months of age

What is the most widely used procedure for repair of high and intermediate anorectal malformations in the United States?

• Posterior sagittal anorectoplasty

What complications may follow a repair of an anorectal malformation?

• Infection, leak, anastomotic stricture, recurrent fistula

What is the most frequently encountered neonatal surgical emergency?

• NEC

What percentage of NEC cases occur after the initiation of feeding?

• >90%

What are the most common sites of involvement of necrotizing enterocolitis?

• Terminal ileum, right colon

What causes of neonatal intestinal obstruction have a higher incidence in infants with trisomy 21 compared to the general population?

• Duodenal atresia/stenosis Hirschsprung disease

What is the most common cause of colon obstruction in a pediatric patient?

• Hirschsprung disease (vs constipation)

Which portion of the GI tract is most commonly involved in the aganglionosis of Hirschsprung disease?

• Distal rectosigmoid colon (75%–80% of affected infants)

What is the characteristic lesion in the distal bowel seen with Hirschsprung disease?

• Aganglionosis of the intermuscular and submucosal plexuses

What finding on rectal biopsy excludes a diagnosis of Hirschsprung disease?

• The finding of ganglion cells on a rectal biopsy performed 1 to 1.5 cm above the dentate line

How do patients with Hirschsprung disease present?

• Failure to pass meconium within the first 24 to 48 hours of life. Infants can have abdominal distension, feeding intolerance, bilious emesis, malnutrition, and failure to thrive. They can have enterocolitis with forceful expulsion of foul-smelling, liquid stool on rectal examination progressing to sepsis, transmural necrosis, and perforation; can have chronic constipation if older children/adults

When would you perform a full-thickness rectal biopsy under general anesthesia rather than a bedside suction biopsy for the diagnosis of Hirschsprung disease?

• Inadequate suction biopsy in infants, older children

How would you treat a patient with Hirschsprung disease with enterocolitis or multiple associated anomalies/medical problems?

• Resuscitation and broad-spectrum IV antibiotics

• Diverting colostomy versus rectal irrigation and decompression followed by proximal diversion after stabilization

What is the most frequently encountered congenital anomaly of the GI tract?

• Meckel diverticulum

What is a Meckel diverticulum?

• A malformation resulting from persistence of the omphalomesenteric duct (vitelline duct)

Regarding Meckel diverticulum, what is the rule of 2's?

• Found 2 ft from the ileocecal valve; In 2% of the population; 2% are symptomatic; 2 tissue types (gastric—most common and likely to be symptomatic, pancreatic); 2 presentations (bleeding, diverticulitis)

What is the #1 cause of painless lower GI bleeding in children?

• Meckel diverticulum

Is a Meckel diverticulum a true diverticulum?

• Yes, a Meckel diverticulum is a true diverticulum

What is the arterial blood supply to a Meckel diverticulum?

• Persistent vitelline vessels (from SMA)

What is the test of choice to diagnose GI bleeding secondary to a Meckel diverticulum?

• ^99mTc-pertechnetate radioisotope scan (Meckel scan)

What is the Rx of choice for a symptomatic Meckel diverticulum?

• Antimesenteric wedge excision or segmental bowel resection with primary closure versus anastomosis

How does a patient with pyogenic liver abscess present?

• Fever; jaundice; leukocytosis; may find tender liver/hepatosplenomegaly on physical examination

What is the Rx for a pyogenic liver abscess?

• Broad-spectrum antibiotics and percutaneous drainage; rarely, may require open operative drainage

What parasite is responsible for amebic abscess of the liver?

• Entamoeba histolytica

What is the most common cause of chronic cholestasis in infants?

• Biliary atresia

What is the most frequent indication for pediatric liver transplantation?

• Biliary atresia

What is biliary atresia?

• Replacement of the extrahepatic/intrahepatic/both biliary tract ± gallbladder with dense, fibrous inflammatory tissue secondary to an inflammatory process

What is the cardinal sign of biliary atresia?

• Progressive neonatal jaundice during the first few weeks of life

What degree of conjugated hyperbilirubinemia in an infant requires investigation?

• Direct fraction of bilirubin >50%; direct bilirubin >2 mg/dL

What drug is given to infants orally to improve the sensitivity of a hepatobiliary scan in a patient with suspected biliary atresia?

• Phenobarbital (5 mg/kg daily); increases hepatocyte processing ^99mTc-IDA and induces hepatic microsomal enzymes. Workup is ultrasound, laboratories, HIDA

What is the most common ultrasonographic finding in biliary atresia?

• A diminutive/absent gallbladder without associated intrahepatic duct dilatation

What will you see on liver biopsy in a patient with biliary atresia?

• Periportal fibrosis; bile plugging; eventual cirrhosis

What medical condition should be ruled out before operative exploration in all patients with suspected biliary atresia?

• α1-antitrypsin deficiency (difficult to differentiate from biliary atresia); check plasma α1-antitrypsin levels

What is the recommended initial procedure for the Rx of biliary atresia?

• Portoenterostomy (Kasai procedure)

What fraction of patients undergoing the Kasai procedure for biliary atresia get better?

• One-third

What fraction of patients undergoing the Kasai procedure for biliary atresia go on to liver transplant?

• One-third

What fraction of patients undergoing the Kasai procedure for biliary atresia die?

• One-third

What are indications for liver transplantation in a patient with biliary atresia?

• Progressive hepatic failure despite portoenterostomy, growth retardation, complications of portal HTN

What condition is characterized by 2 separate pancreatic ducts resulting from the failure of the dorsal and ventral ducts to fuse normally?

• Pancreas divisum

What is the most common exocrine pancreatic anomaly secondary to defective development during embryogenesis?

• Annular pancreas (circumferential pancreatic tissue around duodenum)

What is the most common disorder of the pancreas in infants/children?

• Acute pancreatitis

How is the definitive diagnosis of pancreas divisum made?

• The demonstration of 2 separate, parallel ductal systems (magnetic resonance cholangiopancreatography)

If indicated, what would be the surgical management for pancreas divisum?

• Sphincterotomy of the accessory duct (open dorsal duct sphincterotomy, endoscopic sphincterotomy)

In what percentage of hypospadias cases is the defect distal to the midshaft?

• >85%

What are the principles of hypospadias repair?

• To advance the urethral meatus to a normal position in the glans; correct penile chordee

When performing resection of a Wilm tumor, what is the consequence of intraoperative spillage of tumor?

• Lesion is upstaged; will need whole-abdomen radiation

By what factor does intraoperative tumor spillage of Wilm tumor increase local abdominal recurrence?

• 6X

Which patients with resectable Wilm tumor should receive postoperative chemotherapy?

• All patients with resectable Wilm tumor receive postoperative chemotherapy.

What is the surgical Rx of testicular torsion?

• For torsed testis: orchiopexy if viable; orchiectomy if necrotic

• For contralateral testis: orchiopexy

If suspect a torsed testes, don't waste time with Doppler's → go to OR!