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484 Cards in this Set

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What is the estimated blood volume of infants and children?
About 8% of body weight or approximately 80 cc/kg
What is the maintenance IV fluid for children?
D5 1/4 NS + 20 mEq KCl
Why 1/4 NS for maintenance IV fluid?
Children (especially those younger than 4 years of ago) cannot concentrate their urine and cannot clear excess sodium
What are the maintenance fluid rates calculated in children?
4, 2, 1 per hour:
- 4 cc/kg for the 1st 10 kg of body weight
- 2 cc/kg for the 2nd 10 kg of body weight
- 1 cc/kg for every kg over the 1st 20

(e.g. the rate for a child weighing 25 kg is 4 x 10 = 40 PLUS 2 x 10 = 20 PLUS 1 x 5 = 5, for an IVF rate of 65 cc/hr
What is the minimal urine output for children?
From 1 to 2 mL/kg/hr
What is the best way to present urine output measurements on rounds?
urine output total per shift THEN cc/kg/hr
What is the major difference b/w adult and pediatric nutritional needs?
Premature infants/ infants/ children need more calories and protein/kg/day
What are the caloric requirements by age for the following patients?
- Premature infants
- Children < 1 yr
- Children 1-7 yrs
- Children 7-12 yrs
- Youths 12-18 yrs
- Premature infants: 80 Kcal/kg/day (90-120)
- Children < 1 yr: 100 Kcal/kg/day (75-90)
- Children 1-7 yrs: 85 Kcal/kg/day (75-90)
- Children 7-12 yrs: 70 Kcal/kg/day (60-75)
- Youths 12-18 yrs: 40 Kcal/kg/day (30-60)
What are the protein requirements by age for the following patients?
- Premature infants
- Children < 1 yr
- Children 1-7 yrs
- Children 7-12 yrs
- Youths 12-18 yrs
- Premature infants: 3 g/kg/day (2-3.5)
- Children < 1 yr: 2 g/kg/day (2-2.5)
- Children 1-7 yrs: 2 g/kg/day
- Children 7-12 yrs: 2 g/kg/day
- Youths 12-18 yrs: 1.5 g/kg/day
How many calories are in breast milk?
20 Kcal/30 cc (same as most formulas)
Give blood volume per kilogram:
- Newborn Infant:
- Infant 1-3 months:
- Child:
- Newborn Infant: 85 cc
- Infant 1-3 months: 75 cc
- Child: 70 cc
What is the number of umbilical veins?
1 (usually)
What is the number of umbilical arteries?
2
Which umbilical vessel carries oxygenated blood?
umbilical veins
The oxygenated blood travels through the liver to the IVC through which structure?
Ductus venosus
Oxygenated blood passes from the right atruim to the left atrium through which structure?
Foramen ovale
Unsaturated blood goes from the right ventricle to the descending aorta through which structure?
Ductus arteriosum
What is the adult structure of the ductus venosus?
Ligamentum venosum
What is the adult structure of the umbilical vein?
Ligamentrum teres
What is the adult structure of the umbilical artery?
Medial umbilical ligament
What is the adult structure of the ductus arteriosus?
Ligamentum arteriosum
What is the adult structure of the urachus?
Median umbilical ligament
What is the tongue remnant of thyroid's descent?
Foramen cecum
What is the persistent remnant of vitelline duct?
Meckel's diverticulum
What is ECMO?
ExtraCorporeal Membrane Oxygenation chronic cardiopulmonary bypass - for complete respiratory support
What are the types of ECMO?
- Venovenous: Blood from vein --> oxygenated --> back to vein
- Venoarterial: Blood from vein (IJ) --> oxygenated --> back to artery (carotid)
What are the indications for ECMO?
Severe hypoxia, usually from congenital diaphragmatic hernia, meconium aspiration, persistent pulmonary HTN, sepsis
What are the contraindications for ECMO?
Weight < 2kg, IVH (intraventricular hemorrhage in brain CI b/c of heparin in line)
What is the major differential diagnosis of a pediatric neck mass?
Thyroglossal duct cyst (midline)
Branchial cleft cyst (lateral)
Lymphadenopathy
Abscess
Cystic hygroma
Hemangioma
Teratoma/dermoid cyst
Thyroid nodule
Lymphoma/Leukemia
(also parathyroid tumors, neuroblastoma, histiocytosis X, rhabdomyosarcoma, salivary gland tumors, neurofibroma)
What is a thyroglossal duct cyst?
Remnant of the diverticulum formed by migration of thyroid tissue; normal development involves migration of thyroid tissue from the foramen cecum at the base of the tongue through the hyoid bone to its final position around the tracheal cartilage
What is the average age at diagnosis for a thyroglossal duct cyst?
Usually presents around 5 years of age
How is the diagnosis of a thyroglossal duct cyst made?
Ultrasound
What are the complications of a thyroglossal duct cyst?
Enlargement, infection, and fistula formation b/w oropharynx or salivary gland; aberrant thyroid tissue may masquerade as thyroglossal duct cyst, and if it is not cystic, deserves a thyroid scan
What is the anatomic location of a thyroglossal duct cyst?
Almost always in the MIDLINE
How can one remember the position of the thyroglossal duct cyst?
Think: thyroGLOSSAL = TONGUE midline sticking out
What is the treatment for a thyroglossal duct cyst?
Antibiotics if infection is present, then excision, which must include the midportion of the hyoid bone and entire tract to the foramen cecum (Sistrunk procedure)
What are branchial cleft anomalies?
Remnant of the primitive branchial clefts in which epithelium forms a sinus tract b/w the pharynx (2nd cleft), or the external auditory canal (1st cleft), and the skin of the anterior neck; if the sinus ends blindly a cyst may form
What is the common presentation of a branchial cleft cyst?
Infection b/c of communication b/w the pharynx and external ear canal
What is the anatomic position of a branchial cleft cyst?
2nd cleft anomaly - LATERAL TO THE MIDLINE along the anterior border of the sternocleidomastoid, anywhere from the angle of jaw to clavicle
1st cleft anomaly - less common than 2nd cleft anomaly; tend to be located higher under the mandible
What is the most common branchial cleft remnant?
2nd; thus these are found most often laterally vs thyroglossal cysts, which are found centrally

think: Second = Superior
What is the major anatomic difference b/w thyroglossal cyst and branchial cleft cyst?
Thyroglossal cyst = MIDLINE
Branchial cleft cyst = LATERAL

think: brAchial = lAteral
What is stridor?
A harsh, high-pitched sound heard on breathing caused by ostruction of the trachea or larynx
What are the signs/symptoms of stridor?
Dyspnea, cyanosis, difficulty with feedings
What is the DDx of stridor?
- Laryngomalacia: leading cause of stridor in infants; results from inadequate development of supporting laryngeal structures; is expectant unless respiratory compromise is present
- Tracheobronchomalacia: similar to laryngomalacia, but involves the entire trachea
- Vascular rings and slings: abnormal development or placement of thoracic large vessels resulting in obstruction of trachea/bronchus
What are the symptoms of vascular rings?
Stridor, dyspnea on exertion, or dysphagia
How is the diagnosis of vascular rings made?
Barium swallow revealing typical configuration of esophageal compression
Echo/arteriogram
What is the treatment of vascular rings?
Surgical division of the ring, if the pt is symptomatic
What is a cystic hygroma?
Congenital abnormality of lymph sac resulting in lymphangioma
What is the anatomic location of a cystic hygroma?
Occurs in the sites of primitive lymphatic lakes and can occur virtually anywhere in the body, most commonly in the floor of the mouth, under the jaw, or in the neck, axilla, or thorax
What is the treatment of a cystic hygroma?
Early total surgical removal b/c they tend to enlarge; sclerosis may be needed if the lesion is unresectable
What are the possible complications of a cystic hygroma?
Enlargement in critical regions, such as the floor of the mouth or paratracheal region, may cause airway obstruction; also, they tend to insinuate onto major structures (although not malignant), making excision difficult and hazardous
What bronchus do FBs go into more commonly (left or right)?
Younger than the age 4: 50/50
Age 4 and older: most go into right bronchus b/c it develops into a straight shot (less of an angle)
What is the most commonly aspirated object?
Peanut
What is the associated risk with peanut aspiration?
Lipod pneumonia
How can an FB result in "air trapping and hyperinflation"?
By forming a "ball valve" (i.e. air in, no air out) as seen on CXR as a hyperinflated lung on expiratory film
How can you tell on A-P CXR if a coin is in the esophagus or trachea?
Coin in esophagus results in the coin lying "en face" with face of the coin viewed as a round object because of compression by anterior and posterior structures
If coin is in the trachea, it is veiwed as a side projection due to the U-shaped cartilage with membrane posteriorly
What is the treatment of tracheal or esophageal FB?
Remove FB with rigid bronchoscope or rigid esophagoscope
What is the DDx of a lung mass
Bronchial adenoma (carcinoid is most common)
Pulmonary sequestration
Pulmonary blastoma
Rhabdomyosarcoma
Chondroma
Hamartoma
Leiomyoma
Mucus gland adenoma
Metastasis
What is the DDx of a mediastinal tumor/mass
1. Neurogenic tumor (ganglioneuromas, neurofibromas)
2. Teratoma
3. Lymphoma
4. Thymoma
(T's: Teratoma, Terrible lymphoma, Thymoma, Thyroid tumor)
Rare: pheochromocytoma, hemangioma, rhabdomyosarcoma, osteochondroma)
What heart abnormality is associated with pectus abnormality?
Mitral valve prolapse (many pts receive preoperative echocardiogram)
What is pectus excavatum?
Chest wall deformity with sternum caving inward

think: exCAVatum = CAVE
What is the cause of pectus excavatum?
Abnormal, unequal outgrowth of rib cartilage
What are the signs/symptoms of pectus excavatum?
Often asymptomatic; mental distress, dyspnea on exertion, chest pain
What is the treatment of pectus excavatum?
Open perichondrium, remove abnormal cartilage, place substernal strut; new catilage grows back in the perichondrium in normal position; remove strut 6 months later
What is the NUSS procedure?
Placent of metal strut to elevate sternum without removing cartilage for pectus excavatum
What is pectus carinatum?
Chest wall deformity with sternum outward (pectus = chest, carinatum = pigeon), much less common than pectus excavatum
What is the cause of pectus carinatum?
abnormal, unequal growth of rib cartilage
What is the treatment of pectus carinatum?
Open perichondrium and remove abnormal cartilage
Place substernal strut
New cartilage grows into normal position
Remove strut 6 months later
What is esophageal atresia without tracheoesophageal (TE) fistula?
Blind-ending esophagus from atresia
What are the signs of esophageal atresia without tracheoesophageal (TE) fistula?
Excessive oral secretions and inability to keep food down
How is the diagnosis of esophageal atresia without tracheoesophageal (TE) fistula made?
Inability to pass NG tube; plain x-ray shows tube coiled in upper esophagus and no gas in abdomen
What is the primary treatment for esophageal atresia without tracheoesophageal (TE) fistula?
Suction blind pouch, IVFs, (gastrostomy to drain stomach if prolonged preoperative esophageal stretching is planned)
What is the definitive treatment of esophageal atresia without tracheoesophageal (TE) fistula?
Surgical with primary anastomosis, often with preoperative stretching of blind pouch (other options include colonic or jejunal interposition graft or gastric tube formation if esophageal gap is long)
What is esophageal atresia with tracheoesophageal (TE) fistula?
esophageal atresia occuring with a fistula to the trachea; occurs in more than 90% of cases of esophageal atresia
What is the incidence of esophageal atresia with tracheoesophageal (TE) fistula?
One in 1500 to 3000 births
Define the following types of fistulas/atresias
- Type A:
- Type B:
- Type C:
- Type D:
- Type E:
- Type A: Esophageal atresia without TE fistula (8%)
- Type B: Proximal esophageal atresia with proximal TE fistula (1%)
- Type C: Proximal esophageal atresia with distal TE fistula (85%); most common type
- Type D: Proximal esophageal atresia with both proximal and distal TE fistulas (2%) (think: D = Double connection to trachea)
- Type E: "H-type" TE fistula w/o esophageal atresia (4%)
How do you remember which type of fistula/atresia is most common?
the most Common type is type C
What are the symptoms of esophageal atresia with tracheoesophageal (TE) fistula?
Excessive secretions caused by an accumulation of saliva (may not occur with type E)
What are the signs of esophageal atresia with tracheoesophageal (TE) fistula?
Obvious respiratory compromise, aspiration pneumonia, postprandial regurgitation, gastric distension as air enters the stomach directly from the trachea
How is the diagnosis of esophageal atresia with tracheoesophageal (TE) fistula made?
Failure to pass an NG tube (although this will not be seen with type E); plain film demonstrates tube coiled in the upper esophagus; "pouchogram" (constant esophageal pouch); gas on AXR (TE fistula)
What is the initial treatment of esophageal atresia with tracheoesophageal (TE) fistula?
Directed toward minimizing complications from aspiration:
1. Suction blind pouch (NPO/TPN)
2. Upright position of child
3. Prophylactic antibiotics (amp/gent)
What is the definitive treatment of esophageal atresia with tracheoesophageal (TE) fistula?
surgical correction via thoracotomy, usually through the right chest with division of fistula and end-to-end esophageal anastomosis if possible
What can be done to lengthen the proximal esophageal pouch in esophageal atresia with tracheoesophageal (TE) fistula?
Delayed repair with or w/o G-tube and daily stretching of proximal pouch
Which type of esophageal atresia with tracheoesophageal (TE) fistula should be fixed via a right neck incision?
"H-type" (type E) is high in the thorax and can most often be approached via right neck incision
What is the work up of a patient with a TE fistula?
To evaluate the TE fistula and associated anomalies: CXR, AXR, U/S of kidneys, cardiac echo (rest of work-up directed at physical exam)
What are the associated anomalies of a TE fistula?
VACTERL cluster (present in about 10% of cases):
- Vertebral or Vascular, Anorectal, Cardiac, TE fistula, Esophageal atresia
- Radial limb or Renal anomalies, Lumbar or Limb
- Previously known as VATER: Vertebral, Anus, TE fistula, Rasial
What is the significance of a "gasless" abdomen on AXR?
No air to the stomach, and thus, no TE fistula
What is a congenital diaphragmatic hernia?
Failure of complete formation of the diaphragm, leading to a defect through which abdominal organs are herniated
What is the incidence of a congenital diaphragmatic hernia?
One in 2100 live births, males are more commonly affected
What are the types of congenital diaphragmatic hernias?
Bochdalek and Morgagni
What are the associated positions of congenital diaphragmatic hernias?
Bochdalek: posterolateral with L>R
Morgagni: anterior parasternal hernia, relatively uncommon
How to remember the position of Bochdalek hernia?
think BOCH DA LEK = "BACK TO THE LEFT"
What are the signs of a congenital diaphragmatic hernia?
Respiratory distress, dyspnea, tachypnea, retractions, and cyanosis; bowel sounds in the chest; rarely, maximal heart sounds on the right; IL chest dullness to percussion
What are the effects on the lungs caused by a congenital diaphragmatic hernia?
1. Pulmonary hypoplasia
2. Pulmonary hypertension
What inhaled agent is often used for effects on the lungs caused by congenital diaphragmatic hernias?
Inhaled nitric oxide (pulmonary vasodilator), which decreases the shunt and decreases pulmonary HTN
What is the treatment for a congenital diaphragmatic hernia?
NG tube, ET tube, stabilization, and if pt is stable, surgical repair;
If pt is unstable: nitric oxide +/- ECMO then to the OR when feasible
What is pulmonary sequestration?
Abnormal benign lung tissue with separate blood supply that DOES NOT communicate with the normal tracheobronchial airway
Define interlobar pulmonary sequestration
Sequestration in the normal lung tissue covered by normal visceral pleura
Define extralobar pulmonary sequestration
Sequestration not in the normal lung covered by its own pleura
What are the signs/symptoms of pulmonary sequestration?
Asymptomatic, recurrent pneumonia
How is the diagnosis of pulmonary sequestration made?
CXR, chest CT, A-gram, U/S with Doppler flow to ascertain blood supply
What is the treatment of extralobar pulmonary sequestration?
Surgical resection
What is the treatment of intralobar pulmonary sequestration?
Lobectomy
What is the major risk during operation for sequestration?
Anomalous blood supply from below the diaphragm (can be cut and retracted into the abdomen and result in exsanguination!); always document blood supply by A-gram on U/S with Doppler flow
What is the DDx of pediatric upper GI bleeding?
Gastritis
Esophagitis
Gastric Ulcer
Duodenal ulcer
Esophageal varices
Foreign Body
Epistaxis
Coagulopathy
Vascular malformation
Duplication cyst
What is the DDx of pediatric lower GI bleeding?
Upper GI bleeding
Anal fissures
NEC (premature infants)
Midgut volvulus (usually children < 1 yr)
Strangulated hernia
Intussusception
Meckel's diverticulum
Infectious diarrhea
Polyps
IBD
Hemolytic Uremic Syndrome
Henoch-Schonlein purpura
Vascular malformation
Coagulopathy
What is the DDx of neonatal bowel obstruction?
Malrotation with volvulus
Intestinal atresia
Duodenal web
Annular pancreas
Imperforate anus
Hirschprung's disease
NEC
Intussusception (rare)
Meckel's diverticulum
Incarcerated hernia
Meconium ileus or plug
Maternal: narcotic abuse, hypermagnesemia, sepsis --> ileus
What is the DDx of infant constipation?
Hirschsprung's disease
CF
Anteriorly displaced anus
Polyps
What is the most commonly performed procedure by US pediatric surgeons?
Indirect inguinal hernia repair
What is the MC inguinal hernia in children?
Indirect
What is an indirect inguinal hernia?
Hernia lateral to Hesselbach's triangle into the internal inguinal ring and down the inguinal canal

(think: through the abdominal wall indirectly into the internal ring and out through the external inguinal ring)
What is Hesselbach's triangle?
A triangle formed by:
1. Epigastric vessels
2. Inguinal ligament
3. Lateral border of the rectus sheath
What type of hearnia goes through Hesselbach's triangle?
A direct hernia from a weak abdominal floor; rare in children (0.5% of all inguinal hernias)
What is the incidence of indirect inguinal hernia in all children?
Approximately 3%
What is the incidence of indirect inguinal hernia in premature infants?
up to 30%
What is the male to female ratio of indirect inguinal hernia?
6:1
What are the risk factors for an indirect inguinal hernia?
Male gender
Ascites
V-P shunt
Prematurity
Family History
Meconium ileus
Abdominal wall defect elsewhere
Hypo/epispadias
Connective tissue disease
Bladder exstrophy
Undescended testicle
CF
Which side is affected more commonly in an indirect inguinal hernia?
Right (about 60%)
What percentage of indirect inguinal hernia are bilateral?
Approx 15%
What percentage of indirect inguinal hernia have a family history of indirect hernias?
Approx 10%
What are the signs/symptoms of an indirect inguinal hernia?
Groin bulge, scrotal mass, thickened cord, silk glove sign
What is the silk glove sign?
The hernia sac rolls under the finger like the finger of a silk glove
Why should an indirect inguinal hernia be repaired?
Risk of incarcerated/strangulated bowel or ovary; will not go away on its own
How is a pediatric inguinal hernia repaired?
High ligation of hernia sac (no repair of the abdominal wall floor, which is a big difference b/w the procedure in children and adults; high refers to high position on the sac neck next to the peritoneal cavity)
Which infants after repair of inguinal hernia need overnight apnea monitoring/ observation?
Premature infants; infants younger than 3 months of age
What is the risk of recurrence after high ligation of an indirect pediatric hernia?
Approx 1%
Describe the steps in the repair of an indirect inguinal hernia from skin to skin
Cut skin, then fat, then Scarpa's fascia, then External Oblique Fascia through the External Inguinal Ring;
Find hernia sac anteriomedially and bluntly separate from the other cord structures;
Ligate sac high at the neck at the Internal Inguinal Ring;
Resect sac and allow sac stump to retract into the peritoneal cavity;
Close External Oblique;
Close Scarpa's fascia;
Close Skin
Define Cryptorchidism
Failure of the testicle to descend into the scrotum
Define Hydrocele
Fluid-filled sac (i.e. fluid in a patent processus vaginalis or in the tunica vaginalis around the testicle)
Define Communicating Hydrocele
Hydrocele that communicates with the peritoneal cavity and thus fills and drains peritoneal fluid or gets bigger, then smaller
Define Noncommunicating Hydrocele
Hydrocele that does not communicate with the peritoneal cavity; stays about the same size
Can a hernia be ruled out if an inguinal mass transilluminates
NO; baby bowel is very thin and will often transilluminate
From what abdominal muscle layer is the cremaster muscle derived?
Internal oblique muscle
From what abdominal muscle layer is the inguinal ligament (a.k.a. Poupart's ligament) derived?
External oblique
What nerve travels with the spermatic cord?
Ilioinguinal nerve
What is in the spermatic cord (5 structures)?
1. Cremasteric muscle fibers
2. Vas deferens
3. Testicular artery
4. Testicular pampiniform venous plexus
5. With or without hernia sac
What is the hernia sac made of?
Basically peritoneum or a patent processus vaginalis
What is the name of the fossa b/w the testicle and epididymis?
Fossa of Geraldi
What attaches the testicle to the scrotum?
The gubernaculum
How can the opposite side be assessed for a hernia intraoperatively?
Many surgeons operatively explore the opposite side when they repair the affected side
- A laparoscope is placed into the abdomen via the hernia sac and the opposite side internal inguinal ring is examined
Name the remant of the processus vaginalis around the testicle
Tunica vaginalis
What is a Littre's inguinal hernia?
Hernia with a Meckel's diverticulum in the hernia sac
What may a yellow/orange tissue that is not fat be on the spermatic cord/testicle?
Adrenal rest
What is the most common organ in an inguinal hernia sac in boys?
Small intestine
What is the most common organ in an inguinal hernia sac in girls?
Ovary/fallopian tube
What lies in the inguinal canal in girls instead of the vas?
Round ligament
Where in the inguinal canal does the hernia sac lie in relation to the other structures?
Anteriomedially
What is a "cord lipoma"?
Preperitoneal fat on the cord structures (pushed in by the hernia sac); not a real lipoma
Should be removed surgically, if feasible
Within the speramtic cord, do the vessels or the vas lie medially?
The vas is medial to the testicular vessels
What is a small outpouching of testicular tissue off of the testicle?
Testicular appendage (a.k.a. the appendix testes); should be removed with electrocautery
What is a "blue dot sign"?
A blue dot on the scrotal skin from a twisted testicular appendage
How is a transected vas treated?
Repair with primary anastomosis
How do you treat a transected ilioinguinal nerve?
Should not be repaired; many surgeons ligate it to inhibit neuroma formation
What happens if you cut the ilioinguinal nerve?
Loss of sensation to the medial aspect of the inner thigh and scrotum/labia; loss of cremasteric reflex
What is an umbilical hernia?
Fascial defect at the umbilical ring
What are the risk factors for an umbilical hernia?
1. Black or African American infant
2. Premature infant
What are the indications for surgical repair of an umbilical hernia?
1. > 1.5 cm defect
2. Bowel incarceration
3. > 4 years of age
What are the causes of GERD?
LES malfunction/malposition
Hiatal hernia
Gastric outlet obstruction
Partial bowel obstruction
Common in cerebral palsy
What are the signs/symptoms of GERD?
Spitting up, emesis, URTI, pneumonia, laryngospasm from aspiration of gastric contents into the tracheobronchial tree, failure to thrive
How is the diagnosis of GERD made?
24 hr pH probe, bronchoscopy, UGI (manometry, EGD, U/S)
What cytologic aspirate finding on bronchoscopy can diagnose aspiration of gastric contents in GERD?
Lipid-laden macrophages (from phagocytosis of fat)
What is the medical/conservative treatment of GERD?
H2 blockers
Small meals/ rice cereal
Elevation of head
What are the indications for surgery in GERD?
"S.A.F.E.":
- Stricture
- Aspiration, pneumonia, Asthma
- Failure to thrive
- Esophagitis
What is the surgical treatment for GERD?
Nissen 360 degree fundoplication, with or w/o G tube
What is congenital pyloric stenosis?
Hypertrophy of smooth muscle of pylorus, resulting in obstruction of outflow
What are the associated risks of congenital pyloric stenosis?
Family history, firstborn, males are affected most commonly, decreased incidence in the african american population
What is the incidence of congenital pyloric stenosis?
One in 750 births, male to female ratio = 4:1
What is the average age at onset of congenital pyloric stenosis?
Usually from 2 weeks after birth to about 2 months (2 to 2)
What are the symptoms of congenital pyloric stenosis?
Increasing frequency of regurgitation, leading to eventual nonbilious projectile vomiting
Why is the vomiting nonbilious in congenital pyloric stenosis?
The obstruction is proximal to the ampulla of Vater
What are the signs of congenital pyloric stenosis?
Abdominal mass or "olive" in epigastric region (85%), hypokalemia hypochloremic metabolic alkalosis, icterus (10%), visible gastric peristalsis, paradoxic aciduria, hematemesis (<10%)
What is the DDx for congenital pyloric stenosis?
Pylorospasm
Milk allergy
Increased ICP
Hiatal hernia
GERD
Adrenal insufficiency
Uremia
Malrotation
Duodenal atresia
Annular pancreas
Duodenal web
How is the Dx of congenital pyloric stenosis made?
Usually by H&P alone
U/S - demonstrates elongated (>15mm) pyloric channel and thickened muscle wall (>3.5mm)
If U/S is nondiagnostic, then barium swallow - shows "string sign" or "double railroad track sign"
What is the initial treatment of congenital pyloric stenosis?
Hydration and correction of alkalosis with D10 NS plus 20 mEq of KCl

Note: the infant;s liver glycogen stores are very small; therefore, use D10; Cl- and hydration will correct the alkalosis
What is the definitive treatment of congenital pyloric stenosis?
Surgical, via Fredet-Ramstedt pyloromyotomy (division of the circular muscle fibers w/o entering the lumen/mucosa)
What are the postoperative complications of congenital pyloric stenosis?
Unrecognized incision through the duodenal mucosa, bleeding, wound infection, aspiration pneumonia
What is the appropriate postoperative feeding for congenital pyloric stenosis?
B/w 6 and 12 hrs postoperative feeding wtih Pedialyte; advanced to full-strength formula over 24 hrs
Which vein crosses the pylorus?
Vein of Mayo
What is duodenal atresia?
Complete obstruction or stenosis of duodenum caused by ischemic insult during development or failure of recanalization
What is the anatomic location of duodenal atresia?
85% are distal to the ampulla of Vater
15% are proximal to the ampulla of Vater (these present with nonbilious vomiting)
What are the signs of duodenal atresia?
Bilious vomiting (if distal to the ampulla), epigastric distension
What is the DDx of duodenal atresia?
Malrotation with Ladd's bands
Annular pancreas
How is the Dx of duodenal atresia made?
Plain abdominal film revealing "double bubble: with one air bubble in the stomach and the other in the duodenum
What is the treatment of duodenal atresia?
Duodenoduodenostomy or Duodenojejunostomy
What are the associated abnormalities of duodenal atresia?
B/w 50% and 70% have cardiac, renal, or other GI defects; 30% have trisomy 21
What is meconium ileus?
Intestinal obstruction from solid meconium concretions
What is the incidence of meconium ileus?
Occurs in about 15% of infants with CF
What percentage of pts w/ meconium ileus have CF?
More than 95%
What are the signs/symptoms of meconium ileus?
Bilious vomiting, abdominal distension, failure to pass meconium, Neuhauser's sign, peritoneal calcifications
What is Neuhauser's sign?
a.k.a. "soap bubble" sign; ground glass appearance in the RLQ on AXR from viscous meconium mixing with air
How is the Dx of meconium ileus made?
Family history of CF, plain abdominal films showing significant dilation of similar-sized bowel loops, but few if any air-fluid levels, BE may demonstrate "microcolon" and inspissated meconium pellets in the terminal ileum
What is the treatment of meconium ileus?
70% nonoperative clearance of meconium using Gastrografin enema, +/- acetylcysteine, which is hypertonic and therefore draws fluid into lumen, separating meconium pellets from bowel wall (60% success rate)
What should you remove during all operative cases of meconium ileus?
Appendix
What is the surgical treatment of meconium ileus?
If enema is unsuccessful, then enterotomy with intraoperative catheter irrigation using acetylcysteine (Mucomyst)
What is the long-term medical treatment of meconium ileus?
Pancreatic enzyme replacement
What is CF?
Inherited disorder of epithelial Cl- transport defect affecting the sweat glands, airways, and GI tract (pancreas, intestine); Dx by sweat test (elevated levels of NaCl >60 mEq/L) and genetic testing
What is DIOS?
Distal Intestinal Obstruction Syndrome: intestinal obstruction in older pts with CF from inspissated luminal contents
What is meconium peritonitis?
A sign of intrauterine bowel perforation; sterile meconium leads to an intense local inflammatory rxn w/ eventual formation of calcifications
What are the signs of meconium peritonitis?
Calcifications on plain films
What is meconium plug syndrome?
Colonic obstruction from unknown factors that dehydrate meconium, forming a "plug"
What is meconium plug syndrome also known as?
Neonatal small left colon syndrome
What are the signs/symptoms of meconium plug syndrome
Abdominal distension and failure to pass meconium within the first 24 hours of life; plain films demonstrate many loops of distended bowel and air-fluid levels
What is the nonoperative treatment of meconium plug syndrome?
Contrast enema is both diagnostic and therapeutic; it demonstrates "microcolon" to the point of dilated colon (usually in transverse colon) and reveals copious intraluminal material
What is the major DDx in meconium plug syndrome?
Hirschsprung's disease
Is meconium plug highly associated with CF?
No; less than 5% of pts have CF, in contrast to meconium ileus, in which nearly all have CF (95%)
What are anorectal malformations?
Malformations of the distal GI tract in the general categories of anal atresia, imperforate anus, and rectal atresia
What is imperforate anus?
Congenital absence of normal anus (complete absence or fistula)
Define a "high" imperforate anus
Rectum patent to level above puborectalis sling
Define a "low" imperforate anus
Rectum patent to below puborectalis sling
Which type of imperforate anus is much more common in women
Low
What are the associated anomalies of imperforate anus?
Vertebral abnormalities, Anal abnormalities, Cardiac, TE fistulas, Esophageal Atresia, Radial/Renal abnormalities, Lumbar abnormalities

(VACTERL; most commonly TE fistula)
What are the signs/symptoms of imperforate anus?
No anus, fistula to anal skin or bladder, UTI, fistula to vagina or urethra, bowel obstruction, distended abdomen, hyperchloremic acidosis
How is the diagnosis of imperforate anus made?
PE, the classic Cross table "invertogram" plain x-ray to see level of rectal gas (not very accurate), perineal U/S
What is the treatment of a low imperforate anus with anal fistula?
Dilataion of anal fistula and subsequent anoplasty
What is the treatment of an high imperforate anus
Diverting colostomy and mucus fistula; neoanus is usually made at 1 yr of age
What is Hirschsprung's disease also known as?
Aganglionic megacolon
What is Hirschsprung's disease?
Neurogenic form of intestinal obstruction in which obstruction results from inadequate relaxation and peristalsis; absence of normal ganglion cells of the rectum and colon
What are the associated risks of Hirschsprung's disease?
Family history; 5% chance of having a 2nd child with the affliction
What is the male to female ratio in Hirschsprung's disease?
4:1
What is the anatomic location of Hirschsprung's disease?
Aganglionosis begins at the anorectal line and involves rectosigmoid in 80% of cases (10% have involvement to splenic flexure, and 10% have involvement of entire colon)
What are the signs/symptoms of Hirschsprung's disease?
Abdominal distension and bilious vomiting; more than 95% present with failure to pass meconium in the first 24 hours; can also present later with constipation, diarrhea, and decreased growth
What is the classic history of Hirschsprung's disease?
Failure to pass meconium in the first 24 hours of life
What is the DDx of Hirschsprung's disease?
Meconium plug syndrome
Meconium ileus
Sepsis with adynamic ileus
Colonic neuronal dysplasia
Hypothyroidism
Maternal narcotic abuse
Maternal hypermagnesemia (tocolysis)
What imaging studies should be ordered in Hirschsprung's disease?
AXR: reveals dilated colon
Unprepared barium enema: reveals constricted aganglionic segment with dilated proximal segment, but this picture may not develop for 3 to 6 weeks; BE will also demonstrate retention of barium for 24 to 48 hours (normal evacuation = 10 to 18 hrs)
What is needed for definitive diagnosis of Hirschsprung's disease?
Rectal biopsy: for definitive Dx, submucosal suction Bx is adequate in 90% of cases; otherwise full-thickness Bx should be performed to evaluate Auerbach's plexus
What is the "colonic transition zone"?
The transition (taper) from aganglionic small colon into the large dilated normal colon seen on BE
What is the initial treatment for Hirschsprung's disease?
In neonates, a colostomy proximal to the transition zone prior to correction, to allow for pelvic growth and dilated bowel to return to normal size
What is a "leveling" colostomy
The colostomy performed for Hirschsprung's disease at the level of normally innervated ganglion cells as ascertained on frozen section intraoperatively
Describe the Swenson procedure
Primary anastomosis b/w the anal canal and healthy bowel (rectum removed)
Describe the Duhamel procedure
The anterior, aganglionic region of the rectum is preserved and anastomosed to a posterior portion of healthy bowel; a functional rectal pouch is thereby created

think: DUHA = DUAL barrels side by side
Describe the Soave procedure
(a.k.a. endorectal pull-through); this procedure involves bringing proximal normal colon through the aganglionic rectum, which has been stripped of its mucosa but otherwise present

think: SOAVE = SAVE the rectum, lose the mucosa
What is the new trend in surgery for Hirschsprung's disease?
No colostomy; remove aganglionic colon (as confirmed on frozen section) and perform pull-through anastomosis at the same time (Boley modification)
What is the prognosis of Hirschsprung's disease?
Overall survival rate >90%;
>96% of pts continent
Postoperative symptoms improve with age
What is malrotation and midgut volvulus?
Failure of the normal bowel rotation, with resultant abnormal intestinal attachments and anatomic positions
Where is the cecum in malrotation?
usually ends up in the RUQ
What are Ladd's bands?
Fibrous bands that extend from the abnormally placed cecum in the RUQ, often crossing over the duodenum and causing obstruction
What is the usual age at onset of malrotation and midgut volvulus?
1/3 present by 1 week of age, 3/4 by 1 month of age, and 90% by 1 year of age
What is the usual presentation of malrotation?
Sudden onset of bilious vomiting

(BILIOUS VOMITING IN AN INFANT IS MALROTATION UNTIL PROVEN OTHERWISE)
Why is the vomiting bilious in malrotation?
The "twist" is distal to the ampulla of Vater
How is the Dx of malrotation made?
Upper GI contrast study showing cut off in duodenum; BE showing abnml position of cecum in the upper abdomen
What are the possible complications of malrotation?
Volvulus with midgut infarction, leading to death or necessitating massive enterectomy (rapid Dx is essential)
What is the treatment for malrotation?
IV ABX and fluid resuscitation with LR, followed by emergent laparotomy with Ladd's procedure; second-look laparotomy if bowel is severely ischemic in 24 hrs to determine if remaining bowel is viable
What is the Ladd's procedure?
1. Counterclockwise reduction of midgut volvulus
2. Splitting of Ladd's bands
3. Division of peritoneal attachments to the cecum, ascending colon
4. Appendectomy
In what direction is the midgut volvulus of malrotation reduced - clockwise or counterclockwise?
Rotation of the bowel in a counterclockwise direction
Where is the cecum after reduction in malrotation?
In the LLQ
What is the cause of bilious vomiting in an infant until proven otherwise?
malrotation with midgut volvulus
What is omphalocele?
Defect of abdominal wall at the umbilical ring; sac covers extruded viscera
How is omphalocele diagnosed prenatally?
It may be seen on fetal U/S after 13 wga, with elevated maternal AFP
What comprises the "sac" of an omphalocele?
Peritoneum and amnion
What organ is often found protruding from an omphalocele, but almost never found with a gastroschisis?
the Liver
What is the incidence of an omphalocele?
approx 1 in 5000 births
How is the diagnosis of omphalocele made?
prenatal U/S
What are the possible complications of an omphalocele?
Malrotation of the gut, anomalies
What is the treatment for an omphalocele?
1. NG tube for decompression
2. IV fluids
3. Prophylactic ABXs
4. Surgical repair of the defect
What is the treatment of a small defect (>2cm) omphalocele?
Closure of abdominal wall
What is the treatment of a medium defect (2-10 cm) omphalocele?
Removal of outer membrane and placement of a silicone patch to form a "silo," temporarily housing abdominal contents; the silo is then slowly decreased in size over 4 to 7 days, as the abdomen acommodates the viscera; then the defect is closed
treatment of a "giant" defect (>10 cm) omphalocele?
Skin flaps or treatment with Betadine spray, mercurochrome, or silver sulfadiazine over the defect; this allows an eschar to form, which epithelializes over time, allowing opportunity for furture repair months to years later
What are the associated abnormalities of omphalocele?
50% of cases occur with often severe abnormalities of the GI tract, CV system, GU tract, musculoskeletal system, CNS, and chromosomes
Of what "pentalogy" is omphalocele a part?
Pentalogy of Cantrell
What is the pentalogy of Cantreall?
"D COPS"
- Diaphragmatic defect (hernia)
- Cardiac abnormalitiy
- Omphalocele
- Pericardium malformation/absence
- Sternal cleft
What is gastroschisis?
Defect of abdominal wall; sac does not cover extruded viscera
How is gastroschisis diagnosed prenatally?
Possible at fetal U/S after 13 wga, elevated maternal AFP
Where is the gastroschisis defect?
Lateral to the umbilicus

think: gAstrochisis = lAteral
What is the treatment for an omphalocele?
1. NG tube for decompression
2. IV fluids
3. Prophylactic ABXs
4. Surgical repair of the defect
What is the treatment of a small defect (>2cm) omphalocele?
Closure of abdominal wall
What is the treatment of a medium defect (2-10 cm) omphalocele?
Removal of outer membrane and placement of a silicone patch to form a "silo," temporarily housing abdominal contents; the silo is then slowly decreased in size over 4 to 7 days, as the abdomen acommodates the viscera; then the defect is closed
treatment of a "giant" defect (>10 cm) omphalocele?
Skin flaps or treatment with Betadine spray, mercurochrome, or silver sulfadiazine over the defect; this allows an eschar to form, which epithelializes over time, allowing opportunity for furture repair months to years later
What are the associated abnormalities of omphalocele?
50% of cases occur with often severe abnormalities of the GI tract, CV system, GU tract, musculoskeletal system, CNS, and chromosomes
Of what "pentalogy" is omphalocele a part?
Pentalogy of Cantrell
What is the pentalogy of Cantreall?
"D COPS"
- Diaphragmatic defect (hernia)
- Cardiac abnormalitiy
- Omphalocele
- Pericardium malformation/absence
- Sternal cleft
What is gastroschisis?
Defect of abdominal wall; sac does not cover extruded viscera
How is gastroschisis diagnosed prenatally?
Possible at fetal U/S after 13 wga, elevated maternal AFP
Where is the gastroschisis defect?
Lateral to the umbilicus

think: gAstrochisis = lAteral
On what side of the umbilicus is the gastroschisis defect found most commonly?
the right
What is the usual size of the gastroschisis defect?
b/w 2 and 4 cm
What are the possible complications of gastroschisis?
Thick edematous peritoneum from exposure to amnionic fluid; malrotation of the gut
Other complications include hypothermia; hypovolemia from third-spacing; sepsis; metabolic acidosis from hypovolemia and poor perfusion; NEC; prolonged ileus
How is the diagnosis of gastroschisis made?
prenatal U/S
What is the treatment of gastroschisis?
Primary - NG tube decompression, IVF (D10 LR), and IV ABX
Definitive - surgical reduction of viscera and abdominal closure; may require staged closure with silo
What is a "silo"?
Silastic silo is a temporary housing for external abdominal contents; silo is slowly tightened over time
What is the prognosis of gastroschisis?
more than 90% survival rate
What are the associated anomalies of gastroschisis?
Unlike omphalocele, relatively uncommon except for intestinal atresia, which occurs in 10% to 15% of cases
How do you remember that omphalocele is associated with abnormalities in 50% of cases?
think: Omphalocele = "Oh no, lots of abnormalities"
What are the major differences of gastroschisis when compared with omphalocele?
No membrane coverings
Uncommon associated abnormalities
Lateral to umbilicus not on umbilicus
How can you remember the position of omphalocele vs gastroschisis?
think: OMphalocele = ON the umbilicus
What are the differences b/w omphalocele and gastroschisis in terms of ANOMALIES?
Common in omphalocele (50%), uncommon in gastroschisis
What are the differences b/w omphalocele and gastroschisis in terms of PERITONEAL/AMNION COVERING (sac)?
Always with omphalocele, never with gastroschisis
What are the differences b/w omphalocele and gastroschisis in terms of POSITION OF UMBILICAL CORD?
On the sac with omphalocele, from skin to the left of the gastroschisis defect
What are the differences b/w omphalocele and gastroschisis in terms of THICK BOWEL?
common with gastroschisis, rare with omphalocele (unless sac ruptures)
What are the differences b/w omphalocele and gastroschisis in terms of PROTRUSION OF LIVER?
Common with omphalocele , almost never with gastroschisis
What are the differences b/w omphalocele and gastroschisis in terms of LARGE DEFECT?
omphalocele
What is appendicitis?
Obstruction of the appendiceal lumen (fecalith, lymphoid hyperplasia), producing a closed loop with resultant inflammation that can lead to necrosis and perforation
What is appendicitis's claim to fame?
Most common surgical disease requiring emergency surgery in children
What is the affected age of appendicitis?
very rare before 3 years of age
What is the usual presentation of appendicitis?
Onset of referred or periumbilical pain followed by anorexia, nausea, and vomiting (Note: unlike gastroenteritis, pain precedes vomiting); pain then migrates to the RLQ, where it becomes more intense and localized from local peritoneal irritation
If the pt is hungry and can eat, serisouly question the Dx of appendicitis
How is the diagnosis of appendicitis made?
H&P
What are the signs/symptoms of appendicitis?
Signs of peritoneal irritation may be present - guarding, muscle spasm, rebound tenderness, obturator and Psoas signs; low-grade fever rising to high grade if perforation occurs
What is the DDx for appendicitis?
Intussusception
Volvulus
Meckel's diverticulum
Crohn's disease
Ovarion torsion
Cyst
Tumor
Perforated ulcer
Pancreatitis
PID
Ruptured ectopic pregnancy
Mesenteric lymphadenitis
What is the common bacterial cause of mesenteric lymphadenitis?
Yersinia enterocolitica
What are the associated lab findings in appendicitis?
Increased WBC (> 10,000 per mm^3 in >90% of cases, with a left shift in most)
What is the role of urinalysis in appendicitis?
to evaluate for possible pyelonephritis or renal calculus, but mild hematuria and pyuria are common in appendicitis b/c of ureteral inflammation
What is the "hamburger" sign?
ask pts with suscepted appendicitis if they would like a hamburger or favorite food; if they can eat, serisouly question the Dx
What radiographic studies may be performed in appendicitis?
Often none; CXR to rule out RML or RLL pneumonia; AXR are usually nonspecific, but calcifed fecalith is present in 5% of cases; U/S to evaluate for ovarian/gynecologic pathology
What is the treatment for appendicitis?
Nonperforated: prompt appendectomy and cefoxitin to avoid perforation
Perforated: triple ABX, fluid resuscitation, and prompt appenedctomy; all pus is drained and cultures obtained, with postoperative ABX continued 5-7 days +/- drain
How long should ABX be administered in appendix is nonperforated?
24 hours
How long should ABX be administered in appendix is perforated?
Usually 5-7 days or until WBCs are normal and pt is afebrile
If normal appendix is found upon exploration, what must be examined/ ruled out?
Meckel's diverticulum, Crohn's disease, Intussusception, Gynecologic disease
What is the approximate risk of perforation in appendicitis?
~25% after 24 hrs from onset of symptoms
~50% by 36 hours
~75% by 48 hours
What is intussusception?
Obstruction caused by bowel telescoping into the lumen of adjacent distal bowel; may result when peristalsis carries a "leadpoint" downstream
What is intussusception's claim to fame?
most common cause of small bowel obstruction in toddlers (< 2 yo)
What is the usual age of at presentation of intussusception?
Disease of infancy; 60% present from 4-12 months; 80% by 2 years of age
What is the most common site of intussusception?
Terminal ileum involving ileocecal valve and extending into ascending colon
What is the most common cause of intussusception?
Hypertrophic Peyer's patches, which act as a lead point, many pts have prior viral illness
What are the signs/symptoms of intussusception?
Alternating lethargy and irritability (colic), bilious vomiting, "currant jelly" stools, RLQ mass on plain AXR, empty RLQ on palpation (Dance's sign)
What is the intussuscepiens?
The recipient segment of bowel

(think: RECIPIENS = intussusCIPIENS)
What is the intussusceptum?
The leading point or bowel that enters the intussuscepiens
What is the treatment for intussusception?
Air or barium enema; 85% reduce with hydrostatic pressure (i.e. barium = meter elevation air = maximum of 120 mmHg); if unsuccessful, then laparotomy and reduction by "milking" the ileum from the colon should be performed
What is the causes of intussusception in older patients?
Meckel's diverticulum, polyps and tumors, all of which act as a lead point
What is Meckel's diverticulum?
Remnent of omphalomesenteric duct/ vitelline duct, which connects the yolk sac with the primitive midgut in the embryo
What is the usual location of Meckel's diverticulum?
B/w 45 and 90 cm proximal to the ileocecal valve on the antimesenteric border of the bowel
What is the major DDx for Meckel's diverticulum?
Appendicitis
Is Meckel's diverticulum a true diverticulum?
Yes; all layers of the intestine are found in the wall
What is the incidence of Meckel's diverticulum?
2% of the population at autopsy' but more than 90% of these are ASx
What is the gender ratio for Meckel's diverticulum?
2-3x more common in males
What is the usual age at onset of symptoms in Meckel's diverticulum?
most frequently in the first 2 years of life, but can occur at any age
What are the possible complications of Meckel's diverticulum?
- Intestinal hemorrhage (painless): 50%, accounts for 50% of all lower GI bleeding in pts younger than 2 yrs, bleeding results from ectopic gastric mucosa secreting acid --> ulcer --> bleeding
- Intestinal obstruction: 25%; most common complication in adults; includes volvulus and intussusception
- Inflammation (+/- perforation): 20%
What percentage of cases of Meckel's diverticulum have heterotopic tissue?
More than 50%; usually gastric mucosa (85%), but duodenal, pancreatic, and colonic mucosa have been described
What is the most common ectopic tissue in a Meckel's diverticulum?
Gastric mucosa
What other pediatric disease entity besides Meckel's diverticulum can also present with GI bleeding secondary to ectopic gastric mucosa?
Enteric duplications
What is the most common cause of lower GI bleeding in children?
Meckel's diverticulum with ectopic gastric mucosa
What is the "rules of 2s" in Meckel's diverticulum?
- 2% are symptomatic
- Found about 2 feet from ileocecal valve
- Found in 2% of the population
- Most symptoms occur before age 2
- One of 2 will have ectopic tissue
- Most diverticula are about 2 inches long
- Male:female ratio = 2:1
What is a Meckel's scan?
Scan for ectopic gastric mucosa in Meckel's diverticulum; uses technetium Tc 99m pertechnetate IV, which is preferentially taken up by gastric mucosa
What is necrotizing enterocolitis?
Necrosis of intestinal mucosa often with bleeding; may progress to transmural intestinal necrosis, shock/sepsis, and death
What are the predisposing conditions to necrotizing enterocolitis?
PREMATURITY
Stress: shock, hypoxia, RDS, apneic episodes, sepsis, exchange transfusions, PDA and cyanotic heart disease, hyperosmolar feeding, polycythemia, indomethacin
What is the pathophysiologic mechanism of necrotizing enterocolitis?
Probable splanchnic vasoconstriction with decreased perfusion, mucosal injury, and probably bacterial invasion
What is necrotizing enterocolitis claim to fame?
most common cause of emergency laparotomy in the neonate
What are the signs/symptoms of necrotizing enterocolitis?
abdominal distension, vomiting, heme positive or gross rectal bleeding, fever or hypothermia, jaundice, abdominal wall erythema (consistent with perforation and abscess formation)
What are the radiographic findings of necrotizing enterocolitis?
fixed, dilated intestinal loops; pneumatosis intestinalis (air in the bowel wall); free air; and portal vein air (sign of advanced disease)
What are the lab findings in necrotizing enterocolitis?
Low hematocrit, glucose, and platelets
What is the treatment of necrotizing enterocolitis?
Most are managed medically:
1. Cessation of feedings
2. OG tube
3. IVF
4. IV ABX
5. Ventilator support, as needed
What are the surgical indications for necrotizing enterocolitis?
Free air in abdomen revealing perforation, and positive peritoneal tap revealing transmural bowel necrosis
What is the operation for necrotizing enterocolitis?
1. Resect
2. Stoma
What is an option for bowel perforation in <1000 gram NEC pt?
placement of percutaneous drain (w/o laparotomy!)
Is portal vein gas or pneumatosis intestinalis alone an indication for operation with NEC?
no
What are indications for peritoneal tap in necrotizing enterocolitis?
Severe thrombocytopenia, distended abdomen, abdominal wall erythema, unexplained clinical downturn
What are the possible complications of necrotizing enterocolitis?
Bowel necrosis, gram-negative sepsis, DIC, wound infection, cholestasis, short bowel syndrome, strictures, SBO
What is the prognosis for necrotizing enterocolitis?
>80% overall survival rate
What is "physiologic jaundice"?
Hyperbilirubinemia in the first 2 weeks of life from inadequate conjugation of bilirubin
What enzyme is responsible for conjugation of bilirubin
Glucuronyl transferase
How is hyperbilirubinemia from "physiologic jaundice" treated?
UV light
What is Gilbert's syndrome?
Partial deficienncy of Glucuronsyl-transferase, leading to intermittent asymptomatic jaundice in the second or third decade of life
What is Crigler-Najjar syndrome?
Rare genetic absence of Glucuronsyl-transferase activity, causing unconjugated hyperbilirubinemia, jaundice, and death from kernicterus (usually w/in the 1st year)
What is biliary atresia?
Obliteration of extrahepatic biliary tree
What is the incidence of biliary atresia?
one in 16,000 births
What are the signs/symptoms of biliary atresia?
Persistent jaundice (normal physiologic jaundice resolves in <2 weeks), hepatomegaly, splenomegaly, ascites, and other signs of portal HTN, acholic stools, biliuria
What are the lab findings in biliary atresia?
Mixed jaundice is always present (i.e., both direct and indirect bilirubin increased), with an elevated serum alkaline phosphatase level
What is the classic "rule of 5s" of indirect bilirubinemia?
Bizarre: with progressive hyperbilirubinemia, jaundice progresses by levels of 5 from head to toes:
- 5 mg/dL = jaundice of head
- 10 mg/dL = jaundice of trunk
- 15 mg/dL = jaundice of leg/feet
What is the DDx of biliary atresia?
Neonatal hepatitis (TORCH)
Biliary hypoplasia
How is the diagnosis of biliary atresia made?
1. U/S to r/o choledochal cyst and to examine extrahepatic bile ducts and gallbladder
2. HIDA scan - shows no excretion into the GI tract (with phenobarbital preparation)
3. Operative cholangiogram and liver biopsy
What is the treatment of biliary atresia?
Early laparotomy by 2 months of age with a modified form of the Kasai hepatoportoenterostomy
How does a kasai work?
the anastomosis of the porta hepatis and the small bowel allows drainage of bile via many microscopic bile ducts in the fibrous structure of the porta hepatis
What is the kasai fails?
Revise or liver transplantation
What are the possible postoperative complications of Kasai procedure for biliary atresia?
Cholangitis (manifested as decreased bile secretion, fever, leukocytosis, and recurrence of jaundice), progressive cirrhosis (manifested as portal HTN with bleeding varices, ascites, hypoalbuminemia, and fat-soluble vitamin K, A, D, E deficiencies)
What are the associated abnormalities of biliary atresia?
B/w 25% and 30% have other anomalies, including annular pancrease, duodenal atresia, malrotation, polysplenic syndrome, situs inversus, and preduodenal portal vein; 15% have congenital heart defects
What is a choledochal cyst?
Cystic enlargement of bile ducts; most commonly arises in extrahepatic ducts, but can also arise in intrahepatic ducts
What is the usual presentation of a choledochal cyst?
50% present with intermittent jaundice, RUQ mass, and abdominal pain; may also present with pancreatitis
What are the possible complications of a choledochal cyst?
Cholelithiasis, cirrhosis, carcinoma, and portal HTN
What is the type I anatomic variant of a choledochal cyst?
Dilation of common hepatic and common bile duct, with cystic duct entering the cyst; most common type (90%)
What is the type II anatomic variant of a choledochal cyst?
Lateral saccular cystic dilation
What is the type III anatomic variant of a choledochal cyst?
Choledochocele represented by an intraduodenal cyst
What is the type IV anatomic variant of a choledochal cyst?
Multiple extrahepatic cysts, intrahepatic cysts, or both
What is the type V anatomic variant of a choledochal cyst?
Single or multiple intrahepatic cysts
How is the diagnosis of a choledochal cyst made?
U/S
What is the treatment of a choledochal cyst?
Operative cholangiogram to clarify pathologic process and delineate the pancreatic duct, followed by complete resection of the cyst and a Roux-en-Y hepatojejunostomy
What condition are pts with choledochal cyst at increased risk of developing?
Cholangiocarcinoma often arises in the cyst; therefore, treat by complete prophylactic resection of the cyst
What is cholelithiasis?
The formation of gallstones
What are the common causes of cholelithiasis in children?
The etiology differs somewhat from that of adults; the most common cause is cholesterol stones, but there is an increased percentage of pigmented stones from hemolytic disorders
What is the DDx of cholelithiasis?
Hereditary spherocytosis
Thalassemia
Pyruvate kinase deficiency
Sickle-cell disease
CF
Long-term PTN
Idiopathic
What are the associated risks of cholelithiasis?
Use of oral contraceptices, teenage, positive family history
What is the treatment of cholelithiasis?
Cholecystectomy is recommended for all children with gallstones
What is an annular pancreas?
Congenital pancreatic abnormality with complete encirclement of the duodenum by the pancreas
What are the symptoms of annular pancreas?
Duodenal obstruction
What is the treatment of annular pancreas?
Duodenoduodenostomy bypass of obstruction (do not resect the pancreas!)
What is the DDx of pediatric abdominal mass?
Wilms' tumor
Neuroblastoma
Hernia
Intussusception
Malrotation with volvulus
Mesenteric cyst
Duplication cyst
Liver tumor (hepatoblastoma/ hemiagnioma)
Rhabdomyosarcoma
Teratoma
What is Wilms' tumor?
Embryonal tumor of renal origin
What is the incidence of Wilms' tumor?
Very rare: 500 new cases in the US per year
What is the average age at diagonsis for Wilms' tumor?
usually b/w 2-4 years of age
What are the symptoms of Wilms' tumor?
Usually ASx except for abdominal mass; 20% of pts present with minimal blunt trauma to mass
What are the signs of Wilms' tumor?
Abdominal mass (most do not cross the midline); hematuria (10-15%); HTN in 20% of cases, related to compression of juxtaglomerular apparatus; signs of Beckwith-Wiedemann syndrome
What are the diagnostic radiologic tests for Wilms' tumor?
Abdominal and chest CT
Define Wilms' tumor Stage I
Limited to kidney and completely resected
Define Wilms' tumor Stage II
Extends beyond kidney, but completely resected; capsule invasion and perirenal tissues may be involved
Define Wilms' tumor Stage III
Residual nonhematogenous tumor after resection
Define Wilms' tumor Stage IV
Hematogenous metastases (lung, distal lymph nodes, and brain)
Define Wilms' tumor Stage V
Bilateral renal involvement
What are the best indicators of survival with Wilms' tumor?
Stage and histologic subtype of tumor; 85% pf pts have favorable histology (FH); 15% have unfavorable histology (UH); overall survival for FH is 85% for all stages
What is the treatment for Wilms' tumor?
Radical resection of affected kidney with evaluation for staging, followed by chemotherapy (low stages) and radiation (higher stages)
What is the neoadjuvant treatment for Wilms' tumor?
Large tumors may be shrunk with chemotherapy/XRT to allow for surgical resection
What are the associated abnormalities of Wilms' tumor?
Aniridia, hemihypertropy, Beckwith-Wiedemann syndrome, neurofibromatosis, horseshoe kidney
What is Beckwith-Wiedemann syndrome?
Syndrome of:
1. Umbilical defect
2. Macroglossia (big tongue)
3. Gigantism
4. Visceromegaly (big organs)

think: Wilms' = beckwith-Wiedmann
What is neuroblastoma?
Embryonal tumor of neural crest origin
What are the anatomic locations for neuroblastoma?
Adrenall medulla - 50%
Paraaortic abdominal paraspinal ganglia - 25%
Posterior mediastinum - 20%
Neck - 3%
Pelvis - 3%
With which type of neuroblastoma tumor does a patient with Horner's syndrome present?
Neck, superior mediastinal tumors
What is the incidence of neuroblastoma?
One in 7,000 to 10,000 liver births; most common solid malignant tumor of infancy; most common solid tumor in children outside the CNS
What is the average age at Dx for neuroblastoma?
Approx 50% are Dx by 2 years of age
Approx 90% are Dx by 8 years of age
What are the symptoms of neuroblastoma?
Vary by tumor location - anemia, FTT, weight loss, and poor nutritional status with advanced disease
What are the signs of neuroblastoma?
ASx abdominal mass (palpable in 50% of cases), respiratory distress (mediastinal tumors), Horner's syndrome (upper chest and neck tumors), proptosis (w/ orbital mets), subcutaneous tumor nodules, HTN (20-35%)
CABS in neuroblastoma?
24-hour urine to measure VMA, HVA, and metanephrines (elevated in >85%), neuron-specific enolase, N-myc oncogene, DNA ploidy
What are the diagnostic radiologic tests in neuroblastoma?
CT, MRI, I-MIBG, somatostatin receptor scan
What is the classic abdominal plain x-ray finding in neuroblastoma?
Calcifications (approx 50%)
How do you access bone marrow involvement in neuroblastoma?
Bone marrow aspirate
What is the difference in position of tumors in neuroblastoma versus Wilms' tumor?
neuroblastoma may cross the midline, but Wilms' tumors do so only rarely
What is the treatment of neuroblastoma?
depends on staging
Define neuroblastoma stage I
tumor is confined to organ of origin
Define neuroblastoma stage II
Tumor extends beyond organ of origin but not across the midline
Define neuroblastoma stage III
Tumor extends across the midline
Define neuroblastoma stage IV
Metastatic disease
Define neuroblastoma stage IVS
Infants: localized primary tumor does not cross the midline, but remote disease is confined to the liver, subcutaneous/skin, and bone marrow
What is the treatment of neuroblastoma stage I
Surgical resection
What is the treatment of neuroblastoma stage II
Resection and chemotherapy +/- XRT
What is the treatment of neuroblastoma stage III
Resection and chemotherapy/ XRT
What is the treatment of neuroblastoma stage IV
chemotherapy/ XRT --> Resection
What is the survival rate of neuroblastoma stage I
Approx 90%
What is the survival rate of neuroblastoma stage II
Approx 80%
What is the survival rate of neuroblastoma stage III
Approx 40%
What is the survival rate of neuroblastoma stage IV
Approx 15%
What is the survival rate of neuroblastoma stage IVS
Survival rate is more than 80%
Note: these tumors are basically stage I or II with metastasis to liver, subcutaneous tissue, or bone marrow; most of these pts, if younger than 1 year of age, have a spontaneous cure

think: stage IVS = Special condition
What are the laboratory prognosticators in neuroblastoma?
Aneuploidy is favorable! The lower the number of N-myc oncogene copies, the better the prognosis
Which oncogene is associated with neuroblastoma?
N-myc oncogene

think: N-myc = Neuroblastoma
What is rhabdomyosarcoma?
Highly malignant striated muscle sarcoma
What is rhabdomyosarcoma's claim to fame?
most common sarcoma in children
What is the age distribution of rhabdomyosarcoma?
Bimodal
1. 2-5 years
2. 15-19 years
What are the most common sites of rhabdomyosarcoma?
1. Head and neck (40%)
2. GU tract (20%)
3. Extremities (20%)
What are the signs/symptoms of rhabdomyosarcoma?
Mass
How is the diagnosis of rhabdomyosarcoma made?
Tissue biopsy, CT, MRI, bone marrow
What is the treatment of a resectable rhabdomyosarcoma?
Surgical excision +/- chemotherapy and radiation therapy
What is the treatment of a unresectable rhabdomyosarcoma?
Neoadjuvant chemo/XRT then surgical excision
What is hepatoblastoma?
Malignant tumor of the liver (derived from embryonic liver cells)
What is the average age of diagnosis of hepatoblastoma?
presents in the first 3 years of life
What is the male to female ratio for hepatoblastoma?
2:1
How id the diagnosis of hepatoblastoma made?
PE - abdominal distension; RUQ mass that moves with respiration
Elevated serum alpha-fetoprotein and ferritin (can be used as tumor markers)
CT scan of abdomen, which often predicts resectability
What percentage of hepatoblastoma will have an elevated AFP level?
approx 90%
What is the treatment of hepatoblastoma?
Resection by lobectomy or trisegmentectomy is the treatment of choice (plus postoperative chemo); large tumors may require preoperative chemo and subsequent hepatic resection
What is the overall survival rate of hepatoblastoma?
approx 50%
What is the major difference in age presentation b/w hepatoma and hepatoblastoma?
hepatoblastoma presents at younger than 3 yo; hepatoma presents at older than 3 yo and in adolescents
What is the leading cause of death in pediatric pts?
Trauma
How are the vast majority of splenic and liver injuries treated in children?
Observation (i.e. nonoperatively)
What is a common simulatory of peritoneal signs in the blunt pediatric trauma victim?
Gastric distension (place an NG tube)
How do you estimate normal systolic blood pressure (SBP) in a child?
80 plus 2 x age (e.g a 5 yo child should have an SBP of about 90)
What is the 20-20-10 rule for fluid resuscitation of the unstable pediatric trauma pt?
Fluid resuscitation in the unstable pediatric pt: first give a 20 cc/kg LR bolus followed by a 2nd bolus of 20 cc/kg LR if needed, if the pt is still unstable after the 2nd LR bolus, then administer a 10 cc/kg bolus of blood
What CT scan findings suggest small bowel injury?
free fluid with no evidence of liver or spleen injury, free air, contrast leak, bowel thickening, mesentary streaking
What is the treatment for duodenal hematoma?
observation with NGT and TPN
What is bilious vomiting in an infant?
Malrotation until proven otherwise! (about 90% of pts w/ malrotation present before the 1st year of life)
What does TORCHES stand for?
Nonbacterial fetal and neonatal infections:
- TOxoplasmosis
- Rubella
- Cytomegalovirus (CMV)
- Herpes
- Syphilis
What is the common pediatric sedative?
Chloral hydrate
What are the contraindications to circumcision?
Hypospadias, ect., b/c the foreskin might be needed for future repair of the abnormality
When should an umbilical hernia be repaired?
After 4 years of age; otherwise observe b/c most close spontaneously; repair before school age if persists
What is the cancer risk in the cryptorchid testicle?
more than 10x the nml testicular cancer rate
When should orchidopexy be performed?
all pts with undescended testicle should undergo orchidopexy after 1 yr of age
What are the signs of child abuse?
Cigarette burns, rope burns, scald to posterior thighs and buttocks, multiple fractures/ old fractures, genital trauma, delay in accessing health care system
What is the treatment of child abuse?
Admit the pt to the hospital
What is Dance's sign
Empty RLQ in pts with ileocecal intussusception
What is the treatment of hemangioma?
Observation, b/c most regress spontaneously
What is the indications for operation in hemangiomas?
Severe thrombocytopenia, congestive heart failure, functional impairment (vision, breathing)
What are the treatment options for hemangiomas?
Steroids, radiation, surgical resection, angiographic embolization
What is the most common benign liver tumor in children?
Hemangioma
What is Eagle-Barrett's syndrome?
a.k.a. Prune belly; congenital inadequate abdominal musculature (very lax and thin)
What is the Pierre-Robin syndrome?
Classic Triad:
1. Big, protruding tongue (glossoptosis)
2. Small mandible (micrognathia)
3. Cleft palate
What is the major concern with Pierre-Robin syndrome?
Airway obstruction by the tongue
What are the most common cancers in children?
1. Leukemia
2. CNS tumors
3. Lymphomas
What is the most common solid neoplasm in infants?
Neuroblastoma
What is the most common solid tumor in children?
CNS tumors
What syndrome must you consider in the pt with abdominal pain, hematuria, history of joint pain, and a purpuric rash?
Henoch-Schonlein syndrome; pt may also have melena (50%) or at least guaiac-positive stools (75%)
What is Apley's law?
The further a chronically recurrent abdominal pain is from the umbilicus, the greater is the likelihood of an organic cause for the pain
What is the most common cause of SBO in children?
Hernia
What is a patent urachus?
persistence of the urachus, a communication b/w the bladder and umbilicus; presents with urine out of the umbilicus and recurrent UTIs
What is a "Replogle tube"?
10 French sump pump NG tube for babies (originally designed by Dr. Replogle for suction of the esophageal blind pouch of esophageal atresia)
What are "A's and B's"?
Apnea and Bradycardia episodes in babies
What is the "double bubble" sign on AXR?
Gastric bubble and duodenal bubble on AXR; seen with duodenal obstruction (web, annular pancreas, malrotation w/ volvulus, duodenal atresia, etc.)
What is Poland's syndrome?
Absence of pectoralis major muscle
Absence of pectoralis minor muscle
Often associated with IL hand malformation
Nipple/breast/right-breast hypoplasia
What is the treatment of ATYPICAL mycobacterial lymph node infection?
surgical removal of node
What is the most common cause of rectal bleeding in infants?
anal fissure
What chromosomal abnormality is associated with duodenal web/atresia/stenosis?
Trisomy 21
Which foreign body past the pylorus must be surgically removed?
Batteries
What is the usual age at presentation:
- Pyloric stenosis:
- Intussusception:
- Wilms' tumor:
- Malrotation:
- Neuroblastoma:
- Hepatoblastoma:
- Appendicitis:
- Pyloric stenosis: 2 wk - 2 mo
- Intussusception: 4 mo - 2 yrs (>80%)
- Wilms' tumor: b/w 1 - 4 yo
- Malrotation: birth - 1 yr (>85%)
- Neuroblastoma: ~50% by 2 yrs, >80% by 8 yo
- Hepatoblastoma: < 3 yo
- Appendicitis: > 3 yo (but must be considered at any age!)