Pediatric surgery

Question 1

Embryology

1) what develops from foregut
2) from midgut
3) from hind gut
4) which of the above rotates 270 degrees CCW normally

Answer 1

1) lungs, esophagus, stomach, pancreas, liver, gallbladder, bile duct and duodenum proximal to ampulla
2) duodenum distal to ampulla, small bowel and large bowel to distal 1/3 of transverse colon
3) distal 1/3 of transverse colon to anal canal
4) midgut rotates

Question 2

1) what is low birth weight
2) what is premature
3) what immunoglobulins from where provide immunity at birth
4) #1 cause of childhood death
5) what are the umbilical vessels
6) why is alk phos increased in children compared to adults

Answer 2

1)

Question 3

Trauma in Peds

1) what is a trauma bolus and when to give blood and how much?
2) what is the best indicator of shock
3) definition of tachycardia in neonate, 1yo
4) ideal UOP in children
5) GFR of children

Answer 3

1) 20cc/kg x2. then give blood at 10cc/kg
2) tachycardia
3) neonate >150, 120, rest >100
4) 2-4cc/kg/hr
5) 25% that of adults- poor concentrating ability
6) 4cc/kg/hr for 1st 10kg; 2cc/kg/hr for 2nd 10kg; 1cc/kg/hr for everything after that

Question 4

Congenital Cystic disease of the lung
1) Pulmonary sequestration
a- arterial supply and veneous drainage of lung tissue for this dz
b-presentation
c- difference bw intra-lobar and extra-lobar types
d- rx

Answer 4

1) a- lung tissue has anomalous systemic arterial supply (thoracic aorta or abdominal aorta through inferior pulmonary ligament). Either systemic venous or pulmonary venous drainage
b- infection, respiratory compromise or abnormal CXR
c- intra-lobar is more likely to have pulmonary vein drainage. extralobar is more likely to have systemic drainage.
d- lobectomy

Question 5

Congenital cystic disease of the lung
1) Congenital Lobar overinflation (emphysema
a- pathophysiology
b- MC lobe affected
c- Complications
d- rx

Answer 5

1) a-cartilage fails to develop in bronchus-> air trapping with expiration. Vascular supply and other lobes are normal except compressed by hyperinflated lobe.
b- Left upper lobe
c-can devt hemodynamic instability (same mech as tension PTX) or respiratory compromise
d-lobectomy

Question 6

Congenital cystic disease of Lung:
1) Congenital cystic adenoid malformation
a- what does it communicate with
b-sx
c-lobectomy

Answer 6

1) a-communicates with airway: alveolar structure is poorly developed, although lung tissue is present
b- respiratory compromise or recurrent infection
c- lobectomy

Question 7

Congenital cystic dz of lung: Bronchiogenic cyst

1) location
2) pathophysiology
3) presentation
4) rx

Answer 7

1) MC cysts of the mediastinum. usually posterior to the carina
2) extra-pulmonary cysts formed from bronchial tissue and cartilage wall
3) usually present with a mediastinal mass filled with milky liquid. can compress adj structures or become infected. have malignant potential. occasionally intra-pulmonary
4) resect cyst

Question 8

Mediastinal Masses in children

1) MC mediastinal tumor in children and MC location
2) presenting symptoms
3) Anterior mediastinal tumors incl MC anterior tumor
4) Middle mediastinal tumors/masses
5) Posterior mediastinal masses
6) T/F: thymoma is common in children

Answer 8

1) Neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma), usually posterior
2) respiratory symptoms, dysphagia
3) T cell lymphoma, teratoma, other germ cell tumors (MC), thyroid CA
4) T cell lymphoma, teratoma, cyst (bronchiogenic or cardiogenic)
5) T cell lymphoma, neuroblastoma, neurogenic tumor
6) False, thymomas are rare in children

Question 9

Choledochal cyst

1) cause
2) rx
3) risk of having cyst

Answer 9

1) reflux of pancreatic enzymes into the bilary system in utero
2) resect + hepaticojejunostomy. May need liver lobectomy or TXP for type IV and V bc partially intrahepatic
3) risk of cholangiocarcinoma, pancreatitis, cholangitis and obstructive jaundice

Question 10

Lymphadenopathy in kids
1) MC cause
2) management if fluctuant and chronic causes
3) management if asymptomatic and what if it doesn’t improve with rx?
4) cystic hygroma (lymphangioma)-
a-where is it found usually
b- rx

Answer 10

1) usually acute suppurative adenitis associated with URI or pharyngitis
2) if fluctuant-> FNA, cx and sensitivity and abx. may need I&D if that fails. CHronic causes include cat scratch fever, atypical mycoplasma
3) abx for 10 days. excisional bx if no improvement bc worry about lymphoma
4) a- found in lateral cervical regions in neck, usually lateral to the sternocleidomastoid muscle. gets infected.
b-resect

Question 11

Diaphragmatic hernias peds

1) overall survival of diaphragmatic hernia
2) which side is MC and side effect
3) associated anomalies
4) how to dx
5) sx
6) rx
7) Bochdalek’s hernia- location
8) Morgagni’s hernia- location
9) which is MC of 7 and 8

Answer 11

1) 50%
2) increased on left side (80%)-> severe pulm HTN
3) 80% have associated anomalies incl cardiac and neural tube defects mostly. also malrotation
4) prenatal US, CXR- bowel in chest
5) respiratory distress
6) high-frequency ventilation, inhaled nitric oxide, may nee ECMO. stabilize before OR. In OR-> reduce bowel and repair defect (abdominal approach) + look for visceral anomalies
7) posterior. MC
8) anterior, rare.

Question 12

Chest wall disorders in Peds

1) Pectus Excavatum- reason for repair and how to repair
2) Pectus carinatum- appearance and how to repair

Answer 12

1) repair for cosmesis/emotional stress MC, or resp symptoms. Sternal osteotomy, need strut.
2) repair for emotional stress (pigeon chest). strut not necessary

Question 13

Branchial Cleft cyst

1) 1st brachial cleft cyst- location, associated nerve and where it may fistulize to
2) 2nd brachial cleft cyst- location and where it can fistulize
3) 3rd brachial cleft cyst. location
4) rx o all cysts

Answer 13

1) angle of mandible (may connect with external auditory canal), often associated with facial nerve
2) MC. on anterior border of mid-SCM muscle goes through carotid bifurcation into tonsillar pillar
3) lower neck, medial to or through the lower SCM
4) resection

Question 14

Thyroglosal duct cyst

1) pathophysiology
2) presentation
3) rx

Answer 14

1) from the descent of the thyroid gland from the foramen cecum. may be the only thyroid tissue the pt has.
2) midline cervical mass. goes through the hyoid bone
3) excision of cyst, tract, and hyoid bone (at least the central portion)

Question 15

Hemangioma

1) presentation
2) rx

Answer 15

1) appears at birth or shortly after, rapid growth during first 6-12 months then begins to involute.
2) observation. most resolve by age 7-8
- if lesion has uncontrollable growth or imparis fnc (eyelid or ear canal), or persistent after age 8-> rx with oral steroids. Laser or resection if steroids don’t work

Question 16

Neuroblastoma

1) MC solid malignancy where in kids?
2) presentation/sx
3) MC location
4) MC age of presentation and who has best prognosis
5) serum tests
6) cell origin
7) where do mets go

Answer 16

1) MC solid abdominal malignancy
2) usually asx mass. sometimes secretory diarrhea, racoon eyes (orbital mets), HTN, and opsomyoclonus syndrome (unsteady gate)
3) MC on adrenals, but can occur anywhere along the sympathetic chain
4) in 1st 2 yr of life,

Question 17

Neuroblastoma

1) what factors have worse prognosis
2) what is increased in all pts with mets
3) abdominal XR findings
4) rx

Answer 17

1) NSE, LDH, HVA, diploid tumors and N-myc amplification (>3 copies) have worse prog
2) NSE
3) may show stippled calcifications in tumor
4) resection (adrenal gland and kidney taken-> cures 40%). initially unresectable tumors-> give doxorubicin-based chemo then try to resect

Question 18

Wilms Tumor (nephroblastoma)

1) presentation
2) mean age at dx
3) what is prognosis based on?
4) sites of mets and rx
5) abdominal CT scan findings
6) rx

Answer 18

1_ asx mass, can have hematuria or HTN, 10% bilateral
2) 3yo
3) tumor grade (anaplastic and sarcomatous variations have worse prognosis)
4) frequent mets to bone and lung. can resect pulm mets if resectable
5) replacement of renal parenchyma and NOT displacement (differentiates from neuroblastoma which displaces)
6) nephrectomy (cures 90%). examine C/L kidney. avoid rupture of tumor with resection bc will inc stage.
ALL get chemo unless Stage I and <500g tumor: Actinomycin and vincristine

Question 19

hepatoblastoma

1) serum marker
2) presentation
3) prognosis compared to hepatocellular CA
4) rx
5) which histology has best prognosis

Answer 19

1) increased AFP in 90%
2) fractures, precocious puberty (from Beta-HCG release)
3) better prognosis than HCC
4) resection if can. Otherwise doxorubicin and cisplatin chemo 1st to try to make resectable. survival primarily related to resectability
5) fetal histology

Question 20

MOST COMMON in peds
1) #1 children's malignancy overall
2) #1 solid tumor class
3) #1 general surgery tumor overall
a- in child 2yo
4) #1 cause of duodenal obstruction in newborns 1 wk) and overall

Answer 20

1) leukemia (ALL)
2) CNS tumors
3) neuroblastoma; a- neuroblastoma; b- Wilms tumor
4) duodenal atresia
5) malrotation

Question 21

MOST COMMON in peds

1) MC cause of colon obstruction
2) MC liver tumor
3) MC lung tumor
4) MCC of painful lower GI bleed
5) MCC of painless lower GI bleed
6) MCC of upper GI bleed at a-0-1yr; b-1+yr
7) MCC of duodenal obstruction in newborns
8) MCC of airway obstruction in infants

Answer 21

1) hirschsprung’s
2) hepatoblastoma, 2/3 of liver tumors in children are malignant
3) carcinoid
4) #1 benign anorectal lesions (fissures, etc)
5) meckel’s diverticulum
6) a-gastritis, esophagitis; b- esophageal varices, esophagus.
7) duodenal atresia
8) laryngomalacia

Question 22

Meckel’s diverticulum

1) what side of small bowel is it found on
2) embyologic cause
3) rule of 2’s
4) MC type
5) most likely type to be symptomatic
6) presentations
7) best way to locate
8) rx

Answer 22

1) antimesenteric border
2) persistent vitelline duct
3) 2ft from ileocecal valve, 2% of population, 2% symptomatic, 2 tissue types (pancreatic and gastric), 2 presentations (diverticulitis= pancreatic, and bleeding=gastric)
4) pancreatic
5) gastric
6) #1 cause of painless lower GI bleeding
7) Meckel’s diverticulum scan with pertechnetate if suspicious and having trouble locating
8) resection with sx, suspicion of gastric mucosa or narrow neck. If diverticulitis involving the base or if the base is >1/3 the size of the bowel need to perform segmental resection

Question 23

Pyloric stenosis:

1) age of onset, MC sex
2) presentation signs/sx
3) metabolic abnormality
4) Dx
5) how to resuscitate
6) rx

Answer 23

1) 3-12 weeks, firstborn males
2) projectile vomiting/can feel olive mass in stomach.
3) get hyochloremic, hypokalemic metabolic alkalosis
4) US- pylorus 4+ mm thick, 14+ mm long
5) resuscitate with NS boluses until making urine, then switch to D5NS with 10mEq K. avoid fluid resuscitation with K-containing fluids. avoid non-salt containing solutions as hyponeatremia can quickly develop. Infants should always have maintenance fluid with glucose because of limited reserves for gluconeogenesis
6) pyloromyotomy (RUQ incision; proximal extent should be the circular muscles of the stomach

Question 24

Intussusception
1) typical age of presentation
2) presentation signs/sx
3) causes of lead points in children
4) risk of recurrence
5) rx
6) a- max pressure with air-contrast enema b4 you need to go to OR
b- max column height with barium enema before need to go to OR

Answer 24

1) 3mo to 3years
2) current jelly stools (from vascular congestion, not an indication for resection), sausage mass, abdominal distention, RUQ pain and vomiting
3) enlarged Peyer’s patches (#1), lymphoma and Meckel’s diverticulum
4) 15% recurrence
5) reduce with air-contrast enema (80% success), if doesn’t work need to go to OR. OR also if peritonitis or free air. In OR apply pressure to DISTAL limb. Do not require resection unless associated with lead point (ie- Meckels)
6) a- 120mm HG; b-1meter (3feet). high perforation risk beyond these values

Question 25

Intestinal Atresia

1) cause
2) symptoms
3) MC location
4) what should you get before surgery
5) rx

Answer 25

1) result of intrauterine vascular accidents
2) bilious emesis, distention, most do not pass meconium
3) Jejunum, can be multiple
4) get rectal bx to r/o hirschsprung’s before surgery.
5) resection

Question 26

Duodenal atresia

1) where does it typically occur
2) sx
3) what disease in mother is associated with it
4) what other abnormalities are associated with it
5) AXR findings
6) rx

Answer 26

1) distal to ampulla of vater
2) bilious vomiting, feeding intolerance
3) polyhydramnios
4) cardiac, renal and other GI anomalies, 20% have Down’s syndrome (check chromosomal studies)
5) double-bubble sign
6) resuscitation, duodenoduodenostomy or duodenojejunostomy

Question 27

Tracheoesophageal fistula
1) MC type
2) Type A
a-anatomy; b- sx; c-AXR findings
3) Type C
a-anatomy, b-sx, c-AXR findings
4) describe the components of VACTERL syndrome
5) TE fistula rx
6) complications of Rx

Answer 27

1) Type C (85%); Type A (5%)
2) a-esophageal atresia and no fistula
b-newborn spits up feeds, excessive drooling and respiratory symptoms with feeding. Can’t place NG tube in stomach.
c- gasless abdomen
3) a- Proximal esophageal atresia (blind pouch), distal TE fistula
b- same as type A
c- distended, gas-filled stomach
4) vertebral, anorectal (imperforate anus), cardiac, TE fistula, radius/renal and limb anomalies
5) right extrapleural thoracotomy with primary repair and G_tube. azygous vein often needs to be divided
6) GERD, leak, empyema, stricture and fistula

Question 28

Malrotation

1) presentation and at what age
2) what vessel gets compromised/pathophysiology
3) dx
4) rx

Answer 28

1) sudden onset of bilious vomiting. 75% in 1st month, 90% by 1yo
2) failure of nl CCW rotation 270 degrees of the midgut. Ladd’s bands cause duodenal obstruction, coming out from the right retroperitoneum. Volvulus-> SMA compromise->infarction of the intestine
3) UGI- duodenum does not cross midline, duodenal-jejunal jnc displaced to the right
4) resect Ladd’s bands, CCW rotation, cecopexy (place cecum in LLQ), place duodenum in RUQ and appendectomy

Question 29

Meconium Ileus

1) location of obstruction and sx
2) what disease is it seen in and what test should you get to dx the disease
3) AXR findings
4) complications
5) dx and rx

Answer 29

1) distal ileal obstruction, abd distention, bilious vomiting and distended loops of bowel
2) 10% of Cystic Fibrosis kids. get sweat chloride test or PCR for CL-channel defect
3) dilated loops of small bowel without air-fluid levels (BC meconium is too thick to separate from the bowel wall). can have ground glass or soapsuds appearance
4) can cause perforation-> meconium pseudocyst or free perforation requiring laparotomy
5) gastrografin enema (Effective 80%), can make the dx and potentially treat the pt. Can also use N-acetylcysteine enema. If surgery required-> manual decompressin and create a vent for N-acetylcysteine antegrade enemas

Question 30

Necrotizing enterocolitis

1) classic presentation
2) risk factors
3) AXR findings
4) initial rx
5) indications for surgery
6) what do you need to do before taking down ostomies to r/o distal obstruction from stenosis

Answer 30

1) bloody stools after 1st feeding in premature neonate, lethargy, respiratory decompensation, abd distention, vomiting, blood per rectum
2) prematurity, hypoxia, sepsis
3) pneumatosis intestinalis, free air or portal vein air. need serial lateral decubitus films to look for perf
4) resuscitation, NPO, abx, TPN and orogastric tube
5) free air, peritonitis, clinical deterioration-> resect dead bowel and bring up ostomies
6) barium contrast enema

Question 31

Congenital vascular malformation

1) rx
2) indications for surgery

Answer 31

1) embolization (may be sufficient on own) and/or resection

2) surgery for hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment or limb-length discrepancy

Question 32

Imperforate Anus
1) MC sex
2) associated anomalies
3) High imperforate anus (above levators)-
a- sx; b- rx
4) Low (below levators) imperforate anus:
a-sx; b-rx

Answer 32

1) males
2) VACTERL: vertebral, cardiac, TE fistula, renal limb anomalies
3) a-meconium in urine or vagina (fistula to bladder/vagina/prostatic urethra)
b-colostomy, later anal reconstruction with posterior sagittal anoplasty
4) a-meconium to perineal skin
b- perform posterior sagital anoplasty (pull anus down into sphincter mechanism). no colostomy needed
* both need postop anal dilation to avoid stricture, prone to constipation

Question 33

Gastroschisis

1) pathophysiology and PE
2) rx

Answer 33

1) intrauterine rupture of umbilical vein. does not have peritoneal sac. Only 10% have other congenital anomalies except malrotation. to the right of midline, no peritoneal sac, stiff bowel from exposure to amniotic fluid
2) initially place saline-soaked gauzes and resuscitate pt. TPN, NPO. repair when stable. Try to place bowel back in abdomen. may need vicryl mesh silo with primary closure at later date

Question 34

Omphalocele

1) pathophysiology and PE
2) Cantrell Pentology
3) rx
4) prognosis compared to gastroschisis

Answer 34

1) failure of embryonal development-> has peritoneal sac with cord attached. inc congenital anomalies (50%), midline defect. sac can contain intra-abdominal structures other than bowel, can also have malrotation
2) Cardiac defects, Pericardium defects (usually at diaphragmatic pericardium), sternal cleft or absence of lower sternum, diaphragmatic septum transversum absence, Omphalocele
3) initially place saline-soaked gauzes and resuscitate the pt. TPN, NPO. Repair when stable. Same as Gastroschisis repair
4) worse overall prognosis compared with gastroschisis 2/2 to congenital anomalies.

Question 35

Hirschsprung’s disease

1) MC sign and other sx
2) anorectal exam findings
3) dx
4) pathophysiology
5) rx

Answer 35

1) infant fails to pass meconium in 1st 24 hours. Can also present in older ages 2-3 with chronic constipation. Get distention, ocassionally colitis
2) explosive release of watery stool
3) rectal bx- absence of ganglion cells in the myenteric plexus
4) 2/2 failure of neural crest cells (ganglion cells) to progress in caudal direction
5) may need to bring up a colostomy initially, eventually connect the colon to the anus (Soave or Duhamel procedure)

Question 36

Hirschsprung’s colitis

1) presentation
2) rx

Answer 36

1) may be rapidly progressive, abdominal distention, foul-smelling diarrhea, lethargy, signs of sepsis
2) rectal irrigation to try and empty colon, may need emergency colectomy

Question 37

Umbilical Hernia

1) pathophysiology
2) risk factors
3) rx

Answer 37

1) failure of closure of linea alba. most close by age 3, rare incarceration
2) african americans and premature infants
3) surgery if not closed by age 5, incarceration or if pt has VP shunt

Question 38

Inguinal hernia

1) embyrologic cause
2) how to differentiate from hydrocele
3) rx

Answer 38

1) persistent processus vaginalis, 3% of infants M>F
2) extension of the hernia into the internal ring
3) emergent operation if not able to reduce. otherwise elective repair with high ligation. Consider exploring C/L side if L-sided, female or child <1yr. Need OR within 24 hr after reduction

Question 39

Hydrocele

1) PE
2) natural history
3) rx

Answer 39

1) transilluminate
2) most disappear by 1 year, noncommunicating will resolve
3) surgery at 1yr if not resolved or if thought to be communicating (waxing and waning size). resect hydrocele and ligate processus vaginalis

Question 40

Cystic dupplication

1) MC location
2) rx

Answer 40

1) MC in ileum on mesenteric border

2) resect cyst

Question 41

Biliary Atresia

1) presentation
2) dx
3) complications
4) rx

Answer 41

1) progressive jaundice persisting >2wk after birth suggests atresia. can involve extrahepatic or intrahepatic biliary tree or both
2) liver bx-> periportal fibrosis, bile plugging, eventual cirrhosis. US and cholangiography can reveal atretic biliary tree
3) cholangitis, continued cirrhosis and eventual hepatic failure
4) Kasai procedure (hepaticoportojejunostomy)- 1/3 get better, 1/3 need liver txp; 1/3 die. Resect the atretic extrahepatic bile duct segment. Must do before age 3mo o/w get irreversible liver damage

Question 42

Teratoma
1) serum markers
2) MC location in a-neonates; b-adolescents
3) rx
4) sacrococcygeal teratomas
a-benign or malignant
b-at what point do they transition from benign to malignant
c- rx

Answer 42

1) inc AFP and beta-HCG
2) a-sacrococcygeal; b-ovarian
3) excision
4) a-90% benign at birth (almost all with exophytic component), great potential for malignancy
b- usually benign 2mo
c- coccygectomy and long-term f/u

Question 43

Undescended Testicles

1) when to treat
2) what are these pts at higher risk for?
3) if both are undescended what further workup do you need?
4) rx

Answer 43

1) wait until 2yo
2) higher risk of testicular CA (seminoma)- doesn’t change after testicles brought into scrotum
3) chromosomal studies
4) orchipexy through inguinal incision. if not able to get testicles down-> close, wait 6 mo and retry. if will not come down, perform division of spermatic vessels

Question 44

Tracheomalacia

1) pathophysiology/sx
2) surgical indications
3) surgery

Answer 44

1) elliptical, fragmented tracheal rings instead of C-shaped. Wheezing, usually gets better after 1-2yr
2) dying spell, failure to wean from vent, recurrent infections
3) aortopexy (aorta sutured to back of sternum, opens up trachea)

Question 45

Laryngomalacia
a- sx
b-pathophysiology
c- rx

Answer 45

a- intermittent respiratory distress and stridor exacerbation in the supine position
b- immature epiglottis catilage with intermittent collapse of the epiglottis airway
c- most outgrow by age 12, surgical tracheostomy reserved for small number of pts

Question 46

Choanal Atresia

1) what is it
2) sx
3) rx

Answer 46

1) obstruction of choanal opening (nasal passage) by either bone or mucous membrane. usually unilateral
2) respiratory distress, poor suckling
3) surgical correction

Question 47

Laryngeal papilomatosis

1) T/F: frequently involutes after puberty
2) rx
3) cause

Answer 47

1) true
2) endoscopic removal or laser, but frequently comes back
3) HPV in mother during passage through the birth canal

Question 48

What GI disease do cerbral palsy pts often develop

Answer 48

GERD