Gi: Pediatric GI Surgery - (Safaoui) - JCN
Safaoui TEST = Important
Terms in this set (75)
OVERVIW: List the 8 gastrointestinal pathologies that will be covered on the exam
1) Pyloric stenosis
4) Annular pancreas
7) Intestinal atresia
What is an esophageal atresia?
Blind esophageal POUCH with OR without a fistula between the esophagus and the airway
TEST: Esophageal atresia is commonly be associated with (oligo/poly)-hydramnios seen in pregnancy
TEST: Esophageal atresia is associated with what kind of anomalies in the baby?
What is the mnemonic to remember this and which one of the anomalies is most commonly seen?
Cardiac defects (MOST COMMON)
TEST: What is the most common type of tracheoesophageal fistula?
What is the second most common type of tracheoesophageal fistula?
: Esophageal atresia WITH a DISTAL tracheoesophageal fistula
- Type C
Second most common
: Esophageal atresia WITHOUT a tracheoesophageal fistula (the esophagus just ends in a blind sac with no connection between the distal esophagus and the trachea)
- Type A
How do newborns with esophageal atresia present?
With type C esophageal atresia, crying will result in abdominal (shrinking/distention). Why?
- Excessive DROOLING and secretions
- CHOKING w/ attempted feeding
With type C esophageal atresia, crying will result in abdominal DISTENTION.
This is because the distal esophagus is connected to the trachea so that
each time the baby takes a breath to cry, air will enter the stomach --> DISTENTION!
After taking an X-ray, how could you clinically determine if a baby has an esophageal atresia?
Failure to put in the NG tube (it will start coiling on itself)
An esophageal atresia (with/without) a fistula will produce a gasless abdomen
- Type A
Note: With Type C, there will be GAS in the abdomen
AGAIN: What is the most common in-utero finding with esophageal atresia?
(can't swallow the amniotic fluid and recirculate, so it will build up in the amniotic cavity)
AGAIN: What is the most common anomaly associated with an esophageal atresia?
CARDIAC anomalies (VACTERL)
When ligating the esophageal atresia with the rest of the esophagus, it is important to note the ____ vein as that is commonly cut during the procedure
When part of the intestine or stomach comes above the diaphragm this abnormality is called?
Congenital diaphragmatic hernia
CDH is present in 1:___ - ___ live births and is ___% of all major anomolies.
It is associated with ___ ____ and pulmonary HTN
What is the most common type of CDH?
This is found ___ and is associated with __ ___ CDH
associated with Left Sided CDH
The less common type of CDH is a ___- hernia. This is foung in the ANTERIOR and herniates through the ___ __ _____...it is associated with a ___ ___ CDH
Through the FORAMEN OF MORGAGNI
RIGHT SIDED CDH
TEST: Define omphalocele
Failure of the LATERAL FOLDS of the abdomen to close, creating an UMBILICAL RING in which bowel and solid viscera can protrude (since it occurs right at midline, the UMBILICAL CORD will be PIERCING it)
TEST: An omphalocele is covered by ______ and ______ ______.
What is this condition called when it is not covered by these things?
An omphalocele is covered by PERITONEUM and AMNIOTIC MEMBRANE
If not covered = GASTROSCHISIS
TEST: What is the most common congenital abnormality associated with an omphalocele?
CARDIAC defects (theme here)
The first thing you need to do in treating an omphalocele is what?
MAINTAIN BODY TEMPERATURE
Then you repair by resecting membrane and close fascia
AGAIN: What is a protrusion of bowel and solid and viscera due to failure of the lateral folds of the abdomen to close called when it is NOT covered by peritoneum and an overlying sac?
TEST: In terms of the location, omphaloceles are _______ located so the ______ _____ will be piercing it, while with gastroschisis it is almost always to the (right/left) of the _______
In terms of the location, omphaloceles are CENTRALLY located so the UMBILICAL CORD will be piercing it, while with gastroschisis is is almost always to the RIGHT of the umbilicus
TRUE or FALSE: Just like with omphalocele, the most commonly associated congenital defect with gastroschisis is cardiac defects
Gastroschisis usually isn't associated with congenital anomalies!
- 10% of the time it is associated with INTESTINAL ATRESIA, but not common
RECAP/TEST: List 3 differences between omphalocele and gastroschisis
1) Omphaloceles are CENTRALLY located right through the umbilicus (umbilical cord pierces it) while gastroschisis is usually to the RIGHT of the umbilicus
2) Omphaloceles are COVERED by peritoneum and amniotic sac, gastroschisis is NOT
3) Omphaloceles are associated with congenital anomalies (cardiac defects) while gastroschisis usually is not (and if it is, it is associated w/ intestinal atresia)
BOARDS: What is the FIRST step in treating gastroschisis?
What is the next step after that?
RESUSCITATION is always FIRST when you are operating on a patient (make sure they are hemodynamically stable)
Next step: Primary surgical repair
TEST: What is the most common cause of vomiting in childhood?
Acute VIRAL gastroenteritis (enteroviruses)
TEST/TEST: With pediatric vomiting, you need to determine if it is _____ or non-_____. What does it indicate if it is ______ emesis?
BILIOUS (green) or NON-BILIOUS (not green)
BILIOUS EMESIS indicates OBSTRUCTION (think MALROTATION and MIDGUT VOLVULUS)
AGAIN: Bilious emesis in a newborn is _______ until proven otherwise
Pyloric stenosis is a postnatal muscular ________ of the pyloris, causing a ______ _______ obstruction
Postnatal muscular HYPERTROPHY of the pyloris, causing a GASTRIC OUTLET obstruction
TEST: List the key words for pyloric stenosis
- PROJECTILE (due to pylorus being closed up)
- NON-bilious emesis
- Palpable OLIVE on RUQ (where thickened pylorus is)
To do an ultrasonographic dx of a pyloric stenosis, a channel length of ____ and a pyloric thickness of ____ are needed.
TEST/TEST/BOARDS: What acid-base disorder is associated with pyloric stenosis?
Hypochloremic Hypokalemic metabolic alkalosis with a PARADOXICAL aciduria
Pyloric stenosis is diagnosed by _______ and is treated by ________
Pyloric stenosis is diagnosed by ULTRASOUND and is treated by PYLOROMYOTOMY (Fredet-Ramstedt)
- Making an incision on the pylorus until the submucosa bulges upward (so it has some give to allow it to stretch more)
AGAIN: The cardinal symptom of intestinal obstruction in the newborn is ______ ______. You must rule out ______ with _____ ______!!
- You must rule out MALROTATION with MIDGUT VOLVULUS
What are the two main types of intestinal obstruction in the newborn?
What is the main difference between the two?
1) Proximal obstruction
- Emesis first, then abdominal distention
2) Distal obstruction
- Abdominal distention, emesis later
- Delayed stooling
RECALL: On imaging, large bowel can be seen with _____ that are (centrally/peripherally) located and small bowel can be seen with _____ ______ that are (centrally/peripherally) located
On imaging, large bowel can be seen with HAUSTRA that are PERIPHERALLY located and small bowel can be seen with PLICAE CIRUCLARIS that are CENTRALLY located
ONE MORE TIME: Bilious emesis indicates ______ until proven otherwise, and the most common cause of this is ______
Bilious emesis indicates MALROTATION until proven otherwise, and the
most common cause of this is NONROTATION
AGAIN: With any patient undergoing surgical intervention, you need to _____ them first before operating on them
Which malrotation is a surgical emergency?
Malrotation of the midgut can present initially with or without a midgut ______
VOLVULUS (but eventually will manifest as a volvulus)
What is the gold standard in detecting malrotation of the midgut? What is the caveat to this?
Upper GI series (barium swallow)
- Caveat: Do NOT do this if they have a midgut volvulus!
TEST: What is the surgical treatment for malrotation of the midgut?
AGAIN: Distal obstruction of the intestinal tract presents with delayed ______
TEST: What is an annular pancreas?
Incomplete rotation (during development) of the VENTRAL pancreatic bud resulting in the pancreas encircling the 2nd part of the duodenum
"ring encircling the 2nd part of the duodenum"
Annular pancreas is associated with what genetic abnormality?
TEST: Duodenal atresia is a form of (distal/proximal) obstruction, which means there will be bilious emesis (with/without) abdominal distention
PROXIMAL obstruction, which means there will be bilious emesis WITHOUT abdominal distention (initially)
TEST/TEST: What is the most common cause of obstruction in newborns within 1 week?
What sign can be seen on plain film with this? If there is ____ ____, then you need to rule out a _____ ______ as well
- DOUBLE BUBBLE sign
- If there is DISTAL AIR, then you need to rule out a MIDGUT VOLVULUS
What is the most common cause of duodenal atresia?
FAILURE TO RECANNALIZE
BOARDS: Which type of duodenal atresia is the most common?
Type IIID (Corkscrew shape)
bilious emesis and abdominal distension presenting in the first 2-3 days of life...which shows a MICROCOLON with no reflux into the dilated intestines is???
RECAP: What is the most frequent cause of intestinal obstruction in the NEWBORN?
Newborn: Duodenal atresia
What is the most frequent cause of intestinal obstruction in the first TWO years of life?
In the first 2 years:
Intussusception (terminal ileum "telescoping" into the colon")
TEST/TEST: What physical finding is indicative of intussusception?
CURRANT JELLY STOOL
- "Bloody bowel movement w/ mucus"
What is the diagnostic and therapeutic methods for intussusception?
BARIUM or AIR enemas
TEST: What is the key association with meconium ileus?
TEST: With a meconium ileus, small bubbles of gas can become entrapped and produce a characteristic _____-____ appearance and on contrast enema it will show an _____ pattern
With a meconium ileus, small bubbles of gas can become entrapped and produce a characteristic GROUND-GLASS appearance and on contrast enema it will show an EGGSHELL pattern
What is the most frequent and lethal GI disorder in preemies?
What part of the intestinal tract does it commonly involve?
Necrotizing enterocolitis (NEC)
- Most commonly involves the TERMINAL ILEUM and COLON
TEST: What finding on plain film is pathognomonic for necrotizing enterocolitis (NEC)?
(invasion of ischemic mucosa by gas-producing microbes)
What is the most common cause of a short gut in the neonate?
Intestinal resection (from resecting a necrotizing enterocolitis)
What is the bell criteria?
Define Hirschsprung Disease
Absence of GANGLION cells
in the MUCOSAL and MUSCULAR layers of the colon (Congenital Aganglionic MEGACOLON)
"Think of Hirschsprung as a giant spring that has sprung in the colon. Risk INCREASES w/ Down syndrome"
The ganglion cells are absent in both the ___ and the ____ layers of the involved bowel in a pt with Hirschsprung
SUBMUCOSAL and MUSCULAR
What clinical finding is common with Hirschsprung?
Failure of the newborn to pass the meconium within the first 48 hours of life.
What is the definitive diagnosis of Hirschsprung Disease?
Suction rectal biopsy
A fibroproliferative obliteration of the BILIARY TREE that progresses toward HEPATIC FIBROSIS, CIRRHOSIS and END STAGE liver failure is?
This presents in the first ___ ____ of life
first 3 months
Biliary atresia obliterates the biliary tree and ultimately progresses towards what three clinical manifestations?
1) Hepatic fibrosis
3) End-stage liver failure
All happening in the first 3 months of life
List the two types of biliary atresia
1) Perinatal form (80% of cases)
2) Fetal-embryonic form (20% of cases)
Biliary atresia present with _____ at birth or shortly thereafter
What do the stools look like in a pt with biliary atresia?
ACHOLIC - Pale gray stool
What imaging test can help in your diagnosis of biliary atresia?
- Hepatobiliary scintigraphy
- Looking for a lack of intestinal excretion
Biliary atresia is the leading indication for pediatric _____ ________
What are two operative procedures for biliary atresia?
1) Hepatoportoenterostomy (Kasai procedure)
2) Portocholecystostomy (gallbladder Kasai)
What is the leading indication for PEDIATRIC liver transplant
Without surgical correction or transplantation, what can occur in biliary cirrhosis
Death by 18 to 24 months