-This is when Tracheal and pulmonary tissue develop from the esophagus
-A fistula represents a dysgenesis of this tissue
Dysgenesis occurs at 4-6 weeks of life, is it genetic?
Is not thought to be genetic due to lack of twin concordance.
What is the clinical presentation of Tracheoesophageal Fistula
1) Excessive salivation (drooling)
2) **Cough, choking and respiratory distress & cyanosis—most common
3) Worsening symptoms with attempted feedings
What other anomalies are Tracheoesophageal Fistulas associated with?
Vertebral (segmentation defects), Anal (imperforate), Cardiac (VSD), TE Fistula, Renal, Limb (radial anomalies)
What may difficult cases of Tracheoesophageal Fistula for diagnosis?
1) Gastrograffin swallow
2) Methylene blue challenge
What is the treatment of a Tracheoesophageal Fistula?
1) Needs prompt surgical fixation
2) May require G-tube placement and feedings until surgery can be performed
What is the #1 Cause of Intestinal Obstruction before 3 months of age?
Describe pyloric stenosis?
Progressive gastric outlet obstruction due to hypertrophy of the pylorus musculature
What is the clinical Presentation of pyloric stenosis?
1) 2-6 w.o. post-prandial, progressive nonbilious "projectile" vomiting
- Hypochloremic, hypokalemic metabolic alkalosis (common in all vomiting disorders)
How is the diagnosis of Pyloric Stenosis made?
1) Classic history (2-6 weeks old with vomiting/FTT)
2) Abd exam: Palpate an "olive" in RUQ
3) U/S: confirmed by thickened pyloric channel (thickness, length, diameter)
4) UGI: antral "shoulder" sign with either "string" sign or "double channel" sign
What is the management of pyloric stenosis?
1) Correct electrolyte disturbances
2) OG tube for slow feedings until surgery
3) Pyloromyotomy (open vs. lap)
- Simple surgery with excellent prognosis
4) British standard of care is more conservative
In patients with pyloric stenosis, what can be used for a patient that is a poor surgical candidate?
What is a Bochdalek foramen?
Bowel herniates through the diaphragm during fetal development
- Since bowel develops before lung, bowel impedes lung development
- Left > Right (Back to the left)
What is the clinical presentation of a congenital diaphragmatic hernia?
1) Progressive, severe respiratory distress shortly after delivery
2) "Scaphoid" abdomen
3) Bowel sounds in chest
How is the diagnosis of a congenital diaphragmatic hernia made?
**Infant abdomen scaphoid =
RED FLAG for congenital diaphragmatic hernia
What is the treatment for a congenital diaphragmatic hernia?
1) Intubation and respiratory support
2) OG decompression
3) Immediate tertiary transfer with consideration for surgical fixation
What is the prognosis of a congenital diaphragmatic hernia?
Depends on severity of pulmonary hypoplasia
This is a Failure of the intestines to rotate normally during fetal development?
What does Intestinal malrotation predispose a patient to?
Well what is Midgut volvulus?
Intestines twist on themselves causing obstruction, ischemia and necrosis
What is the clinical presentation Intestinal Malrotation & Volulus?
1) Severe, progressive vomiting
2) Tender, "surgical" abdomen with acute progression
3) Because of this, bilious vomiting in a neonate is a surgical emergency until proven otherwise
- 2/3 of presentation are in children < 1 month
- Vomiting may occur without malrotation due to duodenal constriction from Ladd's bands
What is a surgical abdomen?
Guarding, rebound tenderness, rigidity, maybe distention...
What would a Barium Enema show due to malrotation?
The cecum in RUQ
What would a UGI show in a Volvulus?
What is the treatment for intestinal Malrotation & Volvulus?
1) Fluid resuscitation
2) OG decompression
3) Immediate laparotomy
What would be called a double bubble?
Neonatal Duodenal Atresia
What causes Neonatal Duodenal Atresia?
Caused due to failure of midgut to recanalize at 8-10 weeks after lumen obliteration at 6-7 weeks at level of the Ampulla of Vater (where pancreatic and bile ducts empty into duodenum)
What percentage is Neonatal Duodenal Atresia associated with down syndrome?
What is the clinical presentation of Neonatal Duodenal Atresia?
1) Maternal polyhydramnios
2) Failure at attempts to feed
3) Vomiting (bilious in 80%)
4) Upper abdominal distention
6) "Double bubble" sign on x-ray
What is the management of Neonatal Duodenal Atresia?
1) OG tube decompression and I.V fluid resuscitation until
2) Surgical correction
What are the causes for Neonatal Jejunoileal Atresia?
1) Possibly due to intrauterine mesenteric vascular event
- Increased risk: smoking & vasoconstrictors
2) Frequently the only congenital anomaly
**Most common form of intestinal atresia
What are the clinical manifestations of Neonatal Jejunoileal Atresia?
1) Cardinal signs of neonatal obstruction
- Maternal polyhydramnios
- Bilious vomiting
- Abdominal distention
- Failure to pass meconium in first 24 hours
What would be seen on abdominal CXR with Neonatal Jejunoileal Atresia?
Multiple dilated loops of small bowels with air fluid levels
What should be used to assess the extent of the lesion in Neonatal Jejunoileal Atresia?
What is the management of Neonatal Jejunoileal Atresia?
1) OG tube decompression, IV fluids until
2) Surgical correction
This is a herniation of peritoneal sac through the umbilical ring
-50% affected have other associated anomalies (trisomy 13, 16, 18)
This is a herniation of GI tract without peritoneal sac through ant. abd wall defect lateral to the umbilical ring
-Due to OMA or umbilical vein thrombosis
-Intestines develop a "peel" due to amniotic fluid exposure in utero
What is the treatment Omphalocele vs. Gastroschisis?
1) Tertiary care center
2) OG tube (decompress stomach), IV fluids
3) Cover with sterile dressing (reduce heat and water loss)
4) Small defects repaired immediately (<2cm)
5) Larger defects need staged procedure
______ usually needs staged procedure while _________usually does not (defects are smaller)
This is a true diverticulum on anti-mesenteric border of ileum
-Failure of omphalomesenteric duct obliteration
What is the rule of 2's regarding a Meckel's Diverticulum?
- 2% of the population
-2 ectopic mucosas
- Gastric & pancreatic
- Presents by 2 y.o.
- Within 2 feet of the cecum
- 2 inches long
- Only 5% with a Meckel's diverticulum actually have symptoms
What is the most common clinical presentation of a Meckel's Diverticulum
Painless rectal bleeding most common presentation
What accompanying conditions are associated it a Meckel's Diverticulum?
25% have intestinal obstruction (either intussusception or volvulus)
How is the diagnosis of a Meckel's Diverticulum made?
Meckel's scan (Tc Pertechnetate taken up by ectopic gastric mucosa)
What is the treatment of a Meckel's Diverticulum?
What are the complications of a Meckel's Diverticulum?
This is Meconium becomes impacted in the distal ileum leading to obstruction
What does Meconium ileus prompts search for?
Cystic Fibrosis (occurs in 15% of CF neonates)
What is the clinical presentation of meconium ileus?
1) Abdominal distention
2) Progressive bilious emesis
3) Failure to pass meconium
Would meconium ileus cause jaundice?
How is the diagnosis of meconium ileus made?
Gastrograffin to confirm diagnosis
- If diagnosis found, perform a sweat chloride test or DNA study to test for CF
What is the treatment for meconium ileus?
- May use a Gastrograffin enema as both a diagnostic and therapeutic modality
- Surgical removal if BE unsuccessful
This is Congenital aganglionic megacolon
What causes Hirshsprung's Disease?
1) Lack of parasympathetic neurons results in muscular stricture at site (Parasympathetic nervous system stimulates the gut therefore, lack thereof -->no forward progress of intestinal contents.)
2) Often begin at anus and extends proximally for varying degrees
3) Short segment disease involving rectosigmoid region most common
**In Hirshsprung's disease, where does the dilation occur?
Dilation occurs proximal to aganglionic segment
What is the clinical presentation of Hirshsprung's disease
1) Abd distention, bloating and absence of meconium usually in the first week of life
- May also have progressive, bilious vomiting
2) Mild (smaller) cases may present later in life with chronic constipation (ribbon-like stool)
3) 1/3 of cases present with diarrhea
Now this is the 3rd things we've seen that can cause failure of timely passage of meconium...What were they again?
2) Intestinal atresia
3) and now Hirschprung's.
What does X-ray show in Hirshsprung's Disease?
dilated loops of bowel
What does Barium enema show in Hirshsprung's Disease?
Impaction and megacolon
**Well dammit, what is the DEFINITIVE diagnosis for Hirshsprung's Disease?
Colonoscopic or suction biopsy showing aganglionic mucosa
What is the treatment for Hirshsprung's Disease?
1) Decompressive colostomy (normal bowel)
2) ****Definitive surgical procedure
Usually 2nd year of life
- What is a large collection of anal dysgenesis abnormalities
- Usually associated with other GU abnormalities
What is the management of a imperforate anus?
-If fistula present, dilate fistula with definitive surgical fixation later in life
-If no fistula, diverting colostomy (temporary fix)
What is the #1 Cause of Intestinal Obstruction age 3 months to 6 years
What is the physiology of Intussusception?
1) "Telescoping" of one segment of bowel into another
2) Resulting in:
What is Intussusception associated with?
- Hypertrophied Peyer's patches
- Rotavirus infection
What is the frequent site of intussusception?
What is the clinical presentation of intussusception?
1) 5-12 months-old
2) **Paroxysmal abdominal pain
4) Drawing up legs
6) May follow viral infection (esp GI)
7) Abdominal distention
8) *"Currant jelly" stool (late finding)*
9) "Sausage-shaped" abdominal mass
10) Fever, leukocytosis
What is the management of Intussusception?
Prior to Barium Enema, stabilize the patient:
- Decompress (NG Tube)
- IV Hydration
- Broad spectrum abx
Then: Barium Enema
- Both diagnostic and therapeutic
- Gastrograffin used most often
- Prevents peritonitis if bowel perforates from necrosis
- May also use air enema
What is the lobster claw sign?
When the Barium has come to an end of the obstruction
What is the most common reason for emergency abdominal surgery in child?
Describe appendicitis in a young child
- Pain often poorly localized or absent
- Rupture is more common (40%) than adults
- Rare in children < 3 y.o.
How is the diagnosis made for appendicitis?
- Diagnosis is clinical, not based on labs
- Leukocytosis in only ~30-50%
- UA can be mildly abnormal without UTI
What is the quickest, cheapest, and least invasive way of diagnosing appendicitis?
What other imagine studies can diagnose appendicitis?
- CT and US similar efficacy, although CT with IV contrast likely more accurate
- However, US quicker, cheaper and less invasive (contrast)