Pestana Chap 5 - Pediatric Surgery

Question 1

Do most congenital anomalies require surgical correction?

Answer 1

Yes

Question 2

How does esophageal atresia first present?

Answer 2

Esophageal atresia shows up with excessive salivation noted shortly after birth, or choking spells when first feeding is attempted

Question 3

When an NG tube is placed to help first treat esophageal atresia, what will be seen on x-rays?

Answer 3

A small NG tube is passed, and it will be seen coiled in the upper chest

Question 4

How many types of esophageal atresia are there? Which is the most common and what is seen in the bowel?

Answer 4

1) 4 types
2) If there is normal gas pattern in the bowel, th baby has the most common form of the four tpes, in which there is a blind pouch in the upper esophagus and a fistula between the lower esophagus and the tracheobronchial tree

Question 5

What should be ruled out before treating esophageal atresia? How can this be done?

Answer 5

1) Before therapy is undertaken, associated anomalies (the vertebral, anal, cardiac, tracheal, esophageal, renal, and radial [VACTER] constellation) have to be ruled out
2) Look at the anus for imperforation, check the x-ray for vertebral and radial anomalies, do echocardiogram looking for cardiac anomalies, and sonogram looking for renal anomalies

Question 6

What is the preferred treatment for esophageal atresia? What must be done if this treatment is to be delayed?

Answer 6

1) Primary surgical repair is preferred

2) If it has to be delayed, a gastrostomy has to be done to protect the lungs from acid reflux

Question 7

What is a gastrostomy?

Answer 7

Gastrostomy - an opening into the stomach from the abdominal wall, made surgically for the introduction of food (PEG tube = Percutaneous Endoscopic Gastrostomy)

Question 8

Before treating imperforate anus, what should be ruled out? What should you look for near the anus? What if this finding is present? What if it is not? How is the level of the pouch determined?

Answer 8

1) Rule out the VACTER collection of anomalies
2) Look for a fistula nearby (to vagina or perineum)
3) If fistula is present, repair can be delayed until further growth (but before toilet training time)
4) If fistula is not present, a colostomy needs to be done for high rectal pouches (and later the repair), or a primary repair can be done right away if the blind pouch is almost at the anus
5) The level of the pouch is determined with x-rays taken upside down (so that the gas in the pouch goes up), with a metal marker taped to the anus

Question 9

On which side of the body is congenital diaphragmatic hernia always found? What is the problem with a congenital diaphragmatic hernia? How is this problem addressed? When are patients usually diagnosed?

Answer 9

1) Congenital diaphragmatic hernia is always on the left, and the bowel will be up in the chest
2) The real problem is not the mechanical one, but the hypoplastic lung that still has fetal-type circulation
3) Repair must be delayed 3 or 4 days to allow maturation of the lung. Babies are in respiratory distress and need endotracheal intubation, low-pressure ventilation (careful not to blow up the other lung), sedation, and NG suction. Difficult cases may require extracorporeal membrane oxygenation (ECMO)
4) Many patients currently are diagnosed before birth by sonogram

Question 10

Where is the defect found with gastroschisis and omphalocele?

Answer 10

Gastroschisis and omphalocele show up with an abdominal wall defect in the middle of the belly

Question 11

What occurs in gastroschisis? What occurs in omphalocele?

Answer 11

1) In gastroschisis the cord is normal (it reaches the baby), the defect is to the right of the cord, there is no protective membrane, and the bowel looks angry and matted
2) In omphalocele the cord goes to the defect, which has a thin membrane under which one can see normal-looking bowel and a little slice of liver

Question 12

How are gastroschisis and omphalocele treated? What do babies with gastroschisis additionally need?

Answer 12

1) Small defects can be closed primarily, but large ones require construction of a Silastic “silo” to house and protect the bowel
2) The contents of the silo are then squeezed into the belly, a little bit every day, until complete closure can be done in about a week
3) Babies with gastroschisis also need vascular access for parenteral nutrition, because the angry-looking bowel will not work for about 1 month

Question 13

What is exstrophy of the urinary bladder? How urgent must treatment be done and why?

Answer 13

1) It is an abdominal wall defect over the pubis (which is not fused), with a medallion of red bladder mucosa, wet and shining with urine
2) The baby has to be transferred immediately to a specialized center where a repair can be done within the first 1 or 2 days of life. Delayed repairs do not work

Question 14

Why is green vomiting in the newborn important to take note of?

Answer 14

Green vomiting in the newborn has ominous significance. A serious problem exists

Question 15

What are shared signs found in duodenal atresia, annular pancreas, or malrotation? Do these anomalies require surgical correction? Which is the most dangerous and why?

Answer 15

1) Green vomiting and a “double-bubble” picture in x-rays (a large air-fluid level in the stomach, and a smaller one to its right in the first portion of the duodenum)
2) All of these anomalies require surgical correction
3) Malrotation is the most dangerous because the bowel can twist on itself, cut off its blood supply, and die

Question 16

How is malrotation diagnosed? When can malrotation occur?

Answer 16

1) Malrotation is diagnosed with contrast enema (safe, but not always diagnostic) or upper GI study (more reliable, but more risky)
2) Although described here as a problem of the newborn, the first signs of malrotation can show up at any time within the first few weeks of life

Question 17

How does intestinal atresia present? Do any other congenital anomalies have to be ruled out?

Answer 17

1) Intestinal atresia shows up with green vomiting, but instead of a double bubble there are multiple air-fluid levels throughout the abdomen
2) There may be more than one atretic area, but no other congenital anomalies have to be suspected because this condition results from a vascular accident in utero

Question 18

When does necrotizing enterocolitis occur? What does the baby present with?

Answer 18

1) Necrotizing enterocolitis is seen in premature infants when they are first fed
2) There is feeding intolerance, abdominal distention, and a rapidly dropping platelet count (in babies, a sign of sepsis)

Question 19

What is the treatment of necrotizing enterocolitis? When is surgical intervention required?

Answer 19

1) Treatment: stop all feedings and administer broad-spectrum antibiotics, IV fluids, and IV nutrition
2) Surgical intervention is required if the infant develops abdominal wall erythema, air in the portal vein, intestinal pneumatosis (presence of gas in the bowel wall), or pneumoperitoneum (signs of intestinal necrosis and perforation)

Question 20

In which patients is meconium ileus seen? How do they present? What does imaging show? What is both the diagnostic and therapeutic approach?

Answer 20

1) Meconium ileus is seen in babies who have cystic fibrosis (often hinted at by the mother having it)
2) They develop feeding intolerance and bilious vomiting
3) X-rays show multiple dilated loops of small bowel and a ground-glass appearance in the lower abdomen
4) Gastrografin enema is both diagnostic (microcolon and inspisated pellets of meconium in the terminal ileum) and therapeutic (Gastrografin draws fluid in, dissolves the pellets)

Question 21

At what age and in which patients does hypertrophic pyloric stenosis occur? How does it present?

Answer 21

1) Hypertrophic pyloric stenosis shows up at age 3 weeks, more commonly in firstborn boys
2) Nonbilious projectile vomiting occurs after each feeding. The baby is hungry and eager to eat again after he vomits. By the time they are seen they are dehydrated, with visible gastric peristaltic waves and a palpable “olive-size” mass in the right upper quadrant

Question 22

What is the diagnostic test for hypertrophic pyloric stenosis if a mass cannot be felt? What is treatment?

Answer 22

1) If the mass cannot be felt, a sonogram is diagnostic
2) Therapy begins with rehydration and correction of the hypochloremic, hypokalemic metabolic alkalosis, followed by Ramstedt pyloromyotomy or balloon dilatation

Question 23

In which patients would you suspect biliary atresia? What tests can you conduct to confirm your suspicion?

Answer 23

1) Biliary atresia should be suspected in 6- to 8-week-old babies who have persistent, progressively increasing jaundice (which includes a substantial conjugated fraction)
2) Do serologies and sweat test to rule out other problems, and do HIDA scan after 1 week of phenobarbital (which is a powerful choleretic)

Question 24

When is surgical exploration done for biliary atresia and what fraction of patients need a liver transplant?

Answer 24

If no bile reaches the duodenum even with phenobarbital stimulation, surgical exploration is needed. About 1/3 can get a long-lasting surgical derivation, about 1/3 need a liver transplant after they survived a while with a surgical derivation, and about 1/3 need the transplant right away

Question 25

When does Hirschsprung disease (aganglionic megacolon) present? What is the cardinal symptom? What will a rectal exam cause?

Answer 25

1) Hirschsprung disease can be recognized in early life or may go undiagnosed for many years
2) The cardinal symptom is chronic constipation
3) With short segments, a rectal exam may lead to explosive expulsion of stool and flatus, with relief of abdominal distention

Question 26

How can Hirschsprung disease be differentiated from psychogenic problems in older kids?

Answer 26

In older kids in whom differential diagnosis with psychogenic problems is an issue, presence of fecal soiling suggests the latter

Question 27

What do X-rays show in Hirschsprung disease?

Answer 27

X-rays show distended proximal colon (the normal one) and “normal-looking” distal colon, which is the aganglionic part

Question 28

How is diagnosis of Hirschsprung disease made? What have new surgical advancements created for treatment?

Answer 28

1) Diagnosis is made with full-thickness biopsy of rectal mucosa
2) Ingenious operations have been devised to preserve the unique sensory input of the motor-impaired rectum, while adding the normal propulsive capability of the innervated colon

Question 29

In which patients do you see intussusception and how do they present?

Answer 29

1) Intussusception is seen in 6- to 12-month-old chubby, healthy-looking kids who have episodes of colicky abdominal pain that makes them double up and squat
2) The pain lasts for about 1 minute, and the kid looks perfectly happy and normal until he gets another colic

Question 30

What does physical exam show in patients with intussusception? What test is both diagnostic and therapeutic? When is surgery required?

Answer 30

1) Physical exam shows a vague mass on the right side of the abdomen, an “empty” right lower quadrant, and “currant jelly” stools
2) Barium or air enema is both diagnostic and therapeutic
3) If reduction is not achieved radiologically (or if there are recurrences), surgery is done

Question 31

When is child abuse suspected? What are some classic presentations? What must you always do when you suspect child abuse?

Answer 31

1) Child abuse should always be suspected when injuries cannot be properly accounted for
2) Some classic presentations include subdural hematoma plus retinal hemorrhages (Shaken baby syndrome), multiple fractures in different bones at different stages of healing, and all scalding burns, particularly burns of both buttocks (the child was held by arms and legs and dipped into boiling water)
3) Refer to the proper authorities

Question 32

In which patients should Meckel diverticulum be suspected in? How can it be diagnosed?

Answer 32

1) Meckel diverticulum should be suspected in lower GI bleeding in the pediatric age group
2) Do radioisotope scan (technetium) looking for gastric mucosa in the lower abdomen

Question 33

At what age must undescended testicle be repaired and how is the repair done?

Answer 33

Undescended testicle that has not reached the scrotum by the age of 1 year old needs to be surgically brought down there and fixed in place (orchiopexy)

Question 34

How can an undescended testicle be differentiated from an overactive cremasteric muscle?

Answer 34

A testicle that is in the canal at birth, but can be easily pulled down where it belongs, is not an undescended testicle; it is an overactive cremasteric muscle. Parents need to be reassured of the benign nature of the latter

Question 35

Who most commonly diagnoses abdominal masses in children and why?

Answer 35

Abdominal masses in children are typically discovered by the mother. Mothers constantly handle their babies, are very familiar with their anatomy, and immediately recognize a change

Question 36

How can some of the typical abdominal masses be differentiated in children? What test can be used to differentiate them further?

Answer 36

1) If, on examination, the mass moves up and down with respiration, it is most likely a malignant liver tumor (hepatoblastoma or hepatocellular carcinoma). Alpha fetoprotein is likely to be elevated
2) If the mass is deeper and nonmobile, there is an even chance that it is a Wilms tumor arising from the kidney or a neuroblastoma growing in the adrenal gland
3) Modern imaging devices (CT scan, MRI) can easily clarify what it is

Question 37

How are most abdominal masses in children treated?

Answer 37

Most of these malignancies are treated with surgical resection, if possible, and often they are subjected to additional chemotherapy or radiation

Question 38

How is the prognosis of neuroblastoma in children determined? How does cure rate change with age? What is a curious feature of neuroblastoma?

Answer 38

1) The prognosis in neuroblastoma depends on the child’s age when it is discovered
2) The younger the patient is, the better the cure rate
3) A curious added feature of the neuroblastoma is that it may involute and spontaneously revert to a benign neuroma