PEDS

Question 1

A newborn infant presents in severe respiratory distress. A CXR shows: complete lucency of hemithorax

A chest tube is then placed and the child decompensates further

what is next step

what is dx

Answer 1

Immediate thoracotomy and lobectomy

Congenital lobar emphysema (CLE)

results from overdistention of one or more lobes within a histologically normal lung due to abnormal cartilaginous support of the feeding bronchus.

This focal area of bronchial collapse results in a ball- valve with air trapping and a progressive increase in lobar distention.

Question 2

The symptoms of congenital lobar emphysema

Answer 2

range from

none to severe respiratory distress within the neonatal period.

Asymptomatic patients are often identified during a routine chest radiograph as an area of hyperlucency.

In these cases, observation without pulmonary resection may be prudent.

Occasionally, CLE is identified in a patient with recurrent or persistent pneumonia or with progressive dyspnea. Resection of the involved lung is therapeutic and well tolerated.

The presentation of CLE in a neonate may include severe respiratory distress. In these cases, the clinical and radiographic picture may mimic a tension pneumothorax with severe mediastinal shift. Inadvertent placement of a chest tube into the distended lung would be catastrophic. Immediate thoracotomy with resection of the involved lobe may be lifesaving

Bottom Line: Congenital lobar emphysema can present in many ways. The worst presentation is severe respiratory distress which can look like a tension pneumothorax on CXR. Lobectomy is the only treatment for this severe presentation.

Question 3

jejunoileal atresia

Answer 3

intrauterine focal mesenteric vascular accident (

A preoperative barium enema may be useful to exclude multiple atresias which may be present in 10% to 15% of cases.

usually NOT associated with other anomalies - EXCEPT is cystic fibrosis, which may be present in roughly 10% of cases. (maybe gastroschesis)

This may require multiple anastomoses over an endoluminal stent!
saving as much bowel length as possible.

Question 4

used to make the diagnosis of a patent ductus arteriosus

Answer 4

Transthoracic echocardiography

Question 5

patent ductus arteriosus natural history in term versus premature

Answer 5

Spontaneous closure of the ductus occurs within 4 days in 90% to 95% of full-term infants

80% to 90% of premature infants at 30 to 37 weeks’ gestational age.

The long-term risks of PDA in term infants include development of endocarditis, congestive heart failure, and, eventually, of irreversible pulmonary hypertension (choice D).

Question 6

treatment of PDA

Answer 6

Asymptomatic infants may undergo elective closure between 1 and 2 years of age to facilitate VATS or transcatheter closure

symptomatic infants should undergo prompt evaluation and closure.

NO! COX inhibitors in TERM babies - because of the lack of prostaglandin-responsive contractile smooth muscle in their ductal tissue

Patent ductus arteriosis can be treated with COX inhibitors in PREmature infants but need surgical management in term infants.

Question 7

Testicular torsion in the neonatal and prenatal period

Answer 7

extravaginal–the testicle and both layers of the tunica vaginalis rotate

Testicular torsion in neonates may not produce symptoms and is usually only noted after the testicle has atrophied.

Testicular salvage after neonatal torsion is rare

Question 8

Testicular torsion in children and young adults

Answer 8

(most common in 12-18 year olds)

intravaginal–the testicle and inner layer of the tunica vaginalis rotate.

Adolescents present with severe pain

The diagnosis of testicular torsion is made principally on clinical grounds

If the diagnosis is strongly suspected, the best “test” is operative scrotal exploration.

Surgery should be performed within 4-6 hours of the onset of pain.

The testicular salvage rate if detorsion is performed within 6 hours of symptoms is up to 97%.

An orchiopexy should be performed by fixing the testicle to the scrotal wall at three points.

The anatomic predisposition to torsion affects both testicles; therefore, the contralateral testicle should be similarly repaired

Question 9

mechanisms have been proposed to explain the findings of jejunoileal atresia

Answer 9

prevailing theory is that of an intrauterine focal mesenteric vascular accident.

Question 10

omphalocele mech

Answer 10

11th week of gestation, the midgut returns back into the abdominal cavity and undergoes normal rotation and fixation, along with closure of the umbilical ring.

If the intestine fails to return, the infant is born with abdominal contents protruding directly through the umbilical ring, termed an omphalocele.

Question 11

An umbilical hernia occurs as a result of persistence of the

Answer 11

umbilical ring.

Question 12

The variable persistence of the embryonic processus vaginalis offers a spectrum of abnormalities, including

Answer 12

a scrotal hernia,
communicating hydrocele,
hydrocele of the cord,
simple hydrocele.

Question 13

Gastroschisis results from

Answer 13

an abdominal wall defect associated with normal involution of the

second umbilical vein.

Question 14

cyanotic heart defect pathophys

Answer 14

deoxygenated blood bypassing the lungs and entering the systemic circulation.

This can be caused by right-to-left or bidirectional shunting, or malposition of the great arteries.

Question 15

Cyanotic heart defects include

Answer 15

• Tetralogy of Fallot
• Total anomalous pulmonary venous connection
• Hypoplastic left heart syndrome
• Transposition of the great arteries
• Truncus arteriosus (Persistent)
• Tricuspid atresia
• Interrupted aortic arch
• Pulmonary atresia
• Pulmonary stenosis
• Eisenmenger syndrome (reversal of Shunt due to Pulmonary Hypertension)
• Patent ductus arteriosus (may cause cyanosis in late stage)

Question 16

Non cyanotic heart defects

Answer 16

more common because of higher survival.

In these, the shunt is initially from left (oxygenated) to right (non-oxygenated). These include:

• Atrial septal defect
• Ventricular septal defect
• Coarctation of the aorta
• Double outlet left/right ventricle

Question 17

Infantile hemangiomas

Answer 17

Bottom Line: Strawberry hemangiomas appear at birth and are characterized by a rapid growth phase that then begin to involute. Treatment is observation, as most resolve by age 7 to 8.

characteristic evolution,
with early rapid growth (proliferation)
followed by slow involution.

Rapid growth during the neonatal period (birth to 4 wk) is the historical hallmark of infantile hemangiomas.

superficial dermal lesions (strawberry hemangiomas) appear raised and profoundly erythematous with an irregular texture,

whereas deep lesions (cavernous hemangiomas) appear smooth, slightly raised, with a bluish hue or a faint telangiectatic pattern on the overlying skin.

Observation remains the standard of care for uncomplicated cases.

Fifty percent will involute without treatment by age 5 years, 70% by 7 years

Steroid therapy hastens the rate of proliferation of hemangiomas by 30% to 90% and is indicated for complicated lesions (i.e., those causing severe physiological or anatomical abnormalities)

Question 18

Vascular malformations

Answer 18

normal endothelial cell turn-over

tend to grow with the child.

They are usually visible at birth but may take years or even decades to manifest.

Some lesions can be excised or injected with a sclerosing solution

Question 19

Capillary malformations

Answer 19

port-wine stain (nevus flammeus),
nevus flammeus neonatorum (angel’s kiss),
nevus flammeus nuchae (stork bite, salmon patch),
angiokeratomas, or telangiectasias (spider, hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber syndrome)).

They are prone to infection and are treated aggressively with intravenous antibiotics??

Question 20

A hydrocele what is it

Answer 20

a collection of fluid in the space surrounding the testicle

between the layers of the tunica vaginalis.

communicating = patent processus vaginalis with free flow of fluid

noncommunicating =usually scrotal in males, and may extend to the external inguinal ring

Question 21

associated findings Hydroceles

Answer 21

indirect inguinal hernia
bilateral
RIGHT side

Question 22

diagnosis of hydrocele

Answer 22

A history of intermittent fluctuation in the size, intermittent inguinal bulging, and transillumination is generally diagnostic for hydrocele.

Question 23

A hydrocele is a

Answer 23

collection of fluid in the space surrounding the testicle between the layers of the tunica vaginalis.

Question 24

management of Hydroceles

Answer 24

need SURGERY if:
communicating (patent processus vaginalis with free flow of fluid)

or noncommunicating (usually scrotal in males, and may extend to the external inguinal ring).

Question 25

if bilat Hydrocele which side is most likely side for recurrence

Answer 25

often bilateral

higher rate of occurrence on the right side (sigmoid butress the left?). (careful, Varicocele more often on left (because left gonadal drain into renal instead of directly into IVC)

Occasionally, a hydrocele may extend through the inguinal canal into the retroperitoneum as an abdomino-scrotal hydrocele.

Question 26

Varicocele

Answer 26

upper part of the scrotum.

80% to 90% of cases, they present on the left side,

In the supine position, a redundant left hemiscrotum and horizontal lie of the left testis may be noted.

On standing, the varicocele fills with blood to produce the typical “bag of worms” appearance.

The lesion is NOT painful

however, the boy may complain of a dragging sensation.

Question 27

most common type of solid organ tumor in the pediatric population.

Answer 27

neuroblastoma

Question 28

Children often present with a palpable flank mass, and may have symptoms such as diarrhea, hypertension, or ataxia. what is the diagnosis

Answer 28

neuroblastoma

Question 29

neuroblastoma Workup will reveal

Answer 29

an elevation in catecholamines
and
metanephrines,

easily diagnosed by CT scan or MRI.

Question 30

neuroblastoma -poor prognosis is associated

Answer 30

age greater than 1 year old,
elevations in neuron specific enolase (NSE),
Incr LDH,
Incr N-myc amplification.

Tumors that are hyperdiploid will have more DNA within the tumor cells which make them more susceptible to chemotherapy and portends a favorable prognosis.

Question 31

initial management NEC

Answer 31

bowel rest with nasogastric tube decompression,
fluid resuscitation,
blood and platelet transfusion,
administration of broad-spectrum antibiotics.

Medical management continues for 7 to 10 days and is successful in roughly half of cases.

Question 32

The general principles of surgical management of NEC include

Answer 32

resection of all nonviable segments of intestine with

creation of a stoma.

Question 33

single most important risk factor for NEC

Answer 33

Prematurity

other factors such as 
ischemia, 
bacteria, 
cytokines, 
enteral feeding

Question 34

The absolute indication for operative management of NEC

Other relative indications

Answer 34

the presence of intestinal perforation

as revealed by the identification of free air on plain abdominal radiographs.

Other relative indications for surgery
overall clinical deterioration, 
abdominal wall cellulitis, 
worsening acidosis, 
falling white blood cell 
or 
falling platelet count, 
palpable abdominal mass, or a persistent fixed loop on repeated abdominal radiographs.
Bottom Line: The biggest risk factor for NEC is premature birth. Medical management is successful in 50% of cases. Surgical intervention should be undertaken when there are signs of perforation and resection of all necrotic bowel with stoma creation is the procedure of choice.

Question 35

Describe order of prevelance of esophageal atresia TEF

Answer 35

A 7%
PURE atresia - long gap

B 2%
Proximal TEF
long gap atresia

C 86%
Prox atresia
Distal TEF

D 1%
Proximal AND distal TEF
Distal atresias
N-type

E 4%
PURE TEF
NO atresia

Question 36

natural history of severe pulmonary hypoplasia seen with CDH

Answer 36

Severe pulmonary hypoplasia is irreversible and fatal.

Pulmonary hypertension is potentially reversible.

Question 37

CDH is typically diagnosed by

Answer 37

prenatal ultrasound

associated with polyhydramnios secondary to an acute esophagogastric angle with resulting distal esophageal obstruction.

Question 38

frist steps in management of CDH after birth

Answer 38

After birth, nasogastric decompression is crucial to prevent further distention of the viscera within the chest and additional cardiovascular compromise.

Surgical intervention is not emergent following delivery but should be planned for day 2-3 of life when the neonate is free of significant respiratory or cardiovascular compromise.