Luminal GI Flashcards
What is the most common esophageal atresia?
Type N (also know as proximal atresia, distal fistula and also known as type C TE fistula).
Association with esophageal atresia
VACTERL
CHARGE
T18
T21
VACTERL
V: vertebral anomalies: hemivertebrae, congenital scoliosis, caudal regression
spina bifida
A: anorectal anomalies, anal atresia
C: cardiac anomalies; cleft lip,
TE: tracheo-esophageal fistula +/- esophageal atresia
R: renal anomalies; radial ray anomalies
L: limb anomalies: polydactyly, oligodactyly
Cardiac (77%) and renal (72%)anomalies: Most common
Coronal oritnetation of coin
Esophagus
Sagittal orientation of coin
Trachea
When to remove swallowed maget
If there are 2, if there is 1 it is ok.
When to remove AA and AAA battery?
2 days
When to remove disc battery
2 hours if esophagus
2 days if stomach
Coins (and pennies <1982)
24 hours in the esophagus
24 days in the stomach
Pennies >1982
Remove from stomach
Lead
Immediate removal from stomach
Sharp objects
Remove immediately if esophagus or stomach
Surgery/follow up if postpyloric
Associated with tracheal stenosis (primary) and narrowing between the esophagus and trachea.
Pulmonary sling (aberrant left pulmonary artery)
failure of formation of the 6th aortic arch.
“sling” is best used when the proximal portion of the anomalous vessel impinges on the right main bronchus and causes air trapping of the entire right lung
Hypoplastic right lung, TE fistula, imperforated anus.
Tx Surgical repositioning of the artery.
Shortness of breath (newborn), or difficulty swallowing (adult) most common symptomatic aortic arch variant
4th arch anomaly
No associated with congenital cardiac abnormalities
Most commonly dominant RIGHT
Innominate artery compression syndrome
Normal anatomy + stenosis of trachea and obstructive symtoms.
What causes posterior esophagus compression on esophagogram?
Double aortic arch AND aberrant subclavian (right aberrant with a left arch or left aberrant with right arch, most common aortic arch anomaly)
Diverticulum of Komerell
Pro
Single bubble
Pyloric atresia (image) Antral atresia
Double bubble
HIGHLY SPECIFIC for duodenal atresia
Tripple bubble
Double bouble + distal gas
Exclude atresia
Differential includes midgut volvulus, duodenal stenosis and duodenal webb
Diffusely dilated bowel loops
= Barium enema
If negative= Upper GI (exclude atypical volvulus)
What is most commonly associated with?
Heterotaxy, omphalocele, duodenal atresia, internal hernias
Most common presentation in infants: Volvulus
Ladd bands: Older
SMA in the right and SMV in the left
Midgut volvulus
Ladd procedure:
Release of abnormal bands in second portion
Pexy second and cecum
Appendectomy
Intestinal nonrotation
small bowel occupying the right side of the peritoneal cavity and the colon predominantly on the left
Wandering duodenum
Duodenum inversus
Ladds band
Fibrous staff fixing the cecum, by mistake takes the second portion of the duodenum and causes a partial obstruction
Ladds procedure
Preudoduodenal portal vein
Duodenal obstruction
Heterotaxy syndrome or polysplenia syndrome or associated with:
situs inversus bowel malrotation biliary atresia duodenal atresia annular pancreas
Hypertrophic pyloric stenosis
2-12 weeks
4 mm single wall
14 mm length
Organoaxial volvulus
Old, paraesophageal hernia
Mesenteroaxial volvulus
Children
Two buble of air within the chest
Triad of Borchard
Epigastric pain
Retching without vomiting
Inability to pass a nasogastric tube
Occurs in the second part of the duodenum and is associated with Down syndrome/intestinal malrotation//annular pancreas
Duodenal web
Anterior (two) buds unable to migrate from right to left posteriorly.
Annular pancreas, associated with Down’s syndrome, pancreatitis, duodenal obstruction, etc.
What is short microcolon
Colonic atresia
What is a long microcolon
Ileal atresia or cystic fibrosis (meconium ileus)
Having a microcolon and not terminal ileum
Ileal atresia
Vascular insult
Increased insident of chromosomal abnormalities
25% CF
Having long microcolon and poop in the terminal ileum
Meconium ileus= Cystic fibrosis
20% CF PRESENT WITH Meconium ileu at birht
What is an small left colon?
Meconium plug syndrome
Diabetic mothers
Eclampsia
Hirschprung disease
most common cause of neonatal colonic obstruction
definitive diagnosis requires a full-thickness rectal biopsy (2 cm above the dentate line as the region below the dentate line is normally aganglionic)
- short segment disease: ~75%: rectal and distal sigmoid colonic involvement only
- long segment: ~15%: typically extends to splenic flexure / transverse colon
- total colonic aganglionosis: ~7.5% (range 2-13%) also known as Zuezler-Wilson syndrome occasional extension of aganglionosis into the small bowel
- ultrashort segment disease: 3-4 cm of internal anal sphincter only controversial entity
Associated with CF, intestinal atresia and polyhydramnios
Meconium peritonitis
Associated with meconium peritonitis
Meconium pseudocyst
Who gets tehtered cords?
Imperforated annus:
Associated to other atresias, esophageal atresia, VACTERL association, caudal regression syndrome (associated sacral agenesis and lower limb hypoplasia),
Currarino’s triad (anorectal malformations with sacral anomalies and presacral mass lesion), fistulous tracts to the urethra or vagina may be present or may have a single cloacal opening
Invetrograms high vs low
High: >2 cm: colostomy and subsquent repair
Low: < 2 cm: anoplasty
Most common cause of bowel obstruction in > 4 year old
Appendicitis
Most common cause of obstruction 1 month to 1 year
Indirect inguinal hernia
When intusuception occurs?
3 months to 3 years (lead points outside this age range)
> 2.5 cm
Intusucipiens (recipiens)
What is persistent omphalomesenteric duct?
Meckels 2% of population 2 mucosas (gastric and pancreatic) 2 cm long 2 feet from IC valve <2 years present with symptoms
No surrounding membran, always in the right side of the abdomen
Gastroschisis
Elevated AFP
No congenital shit
+ Intestinal shit
Trisomy 18, surrounded by membranes, base umbilical cord insertion
Omphalocele
High mortality
Antithyroid drugs early pregnancy
Ruptured omphalocele, unable to differentiate from gastroschisis, herniated liver as well.
UmbilIcal cord cysts
50% cardiac
Turner, Klinefelters, Beckwith Wiedeman, Penthalogy of Cantrell
What herniates normally before 13 weeks
Physiologic gut herniation:
While the bowel is within the umbilical cord, the midgut rotates 90 degrees counter-clockwise (facing the embryo). At approximately 10-11 weeks the abdomen enlarges and the intestines return to the abdominal cavity. The midgut then rotates an additional 180 degrees counter-clockwise, fixing to the posterior retroperitoneum.
Omphalocele, ectopia cordis, diaphragmatic defect, pericardial/sternal defect and cardiac malformation
Pentalogy of Cantrell
Intrabadominal cyst with gut signature and vertebral anomaly
Enteric duplication cyst
If not signature: Omental cyst
Duodenal hematoma
Handleblar or NAT
Post ERCP
Cystic fibroiss patient not taking meds
Distal intestinal obstruction syndrome
20-30 years old
Most commonly cause by Yersinia enterocolitica (also salmonella, m tb, h. jejuni, campylo jejuni, shigella, among others)
Mesenteric adenitis
< 36 weeks (preterm), <1500g, cardiopathy
Necrotizing enterocolitis:
- Inflammation, ischemia, and permeability of the neonatal bowel wall to bacteria.
- 2-3 days following birth, with 90% developing within the first 10 days of life
- Medical
- Surgery: Pneumoperitoneum (stage IIIb), portal venous gas, fixed dilated loop on serial x-rays, and abdominal wall erythema
- 10-30% mortality
- 20% develop stricture (more common in large bowel)
Enlarged pancreas without fibrosis causing mass effect on surrounding structures
Lipomatous pseudohypertrophy of the pancreas:
- cystic fibrosis (although some authors exclude this arbitrarily by definition)
- Shwachman-Bodian-Diamond syndrome
- Bannayan syndrome
-Johanson-Blizzard syndrome
Without association with obesity, diabetes mellitus, or chronic pancreatitis
Exocrine pancreatic insuficiency (also pancreatic pseudohypertrophy) + metaphyseal chondrodysplasia + bone marrow hypoplasia (cyclic neutropenia)
Schwachman-Bodian-Diamond syndrome
+main pancreatic duct + lipomatosis
Fatty replacement/infiltration
Absent main pancreatic duct + “fatty replacement”
Pancreas agenesis (dorsal pancreas agenesis)
Absent pancreatic tail + diabetes
Dorsal pancreatic agenesis (most beta cells are in the tail)
Normal ventral bud
Polysplenia
What is the most common and second cause of pancreas fatty replacement in kids?
Cystic fibrosis (First) Shwachman, Diamond syndrome (Second)
What tumor is possibly associated with pancreatic dorsal agenesis?
Solid pseudopapillary epithelial neoplasm (SPEN): TAIL, CYSTIC NECROTIC MASS.
20-30 year old asian, howver kids too.
~15% can be malignant. Complete resection is associated with long-term survival even in the presence of metastatic disease.
Exocrine pancreatic insuficiecny (fatty repl), intellectual disability, fascial staff, short stature,rectourogenital anomalies, hypothyroid
Johanson-Blizzard syndrome
Most frequent mass in infants (<6 months)
Infantile hepatic hemangioma:
- Hepatomegaly
- Hydrops fetalis (av shunting)
- Hemolytic anemia, thrombocytopenia, and consumptive coagulopathy (Kasabach-Merritt sequence)
- Hypothyroidism (elevated type 3-iodothyronine deiodinase activity)
Rapid, proliferative growth phase in the first six months of life, followed by regression and involution
Regression is expected, if symptoms propanolol or AV shunting embolization.
Most common malignant liver neoplasm + Beckwith Wiedeman/Hemihypertrophy/Wilms +Venous extension + Calcifications
Hepatoblastoma: Elevated AFP, male
Lung mets, venous invasion
Negative AFP and <2 year old
Hepatic mesenchymal hamartoma (part of the benigns mesenchymal tumors in childrne, along side the Infantile hepatic hemangioma.
10-14 years old, elevated AFP
Hepatocellular carcinoma, associated with hepatitis B infection
Fanconi, glycogen storage disease, biliary atresia
Second most common pediatric liver malignancy.
Adolescent with normal AFP
Non enhancing scar, T2 dark, photopenic defect with tc99 sulfurcolloid.
Tumor is gallium avid. Tumor calciifes most often than HCC
6-10 year old children with complex liver mass (mucoid, cystic and solid) and normal AFP
Undifferentiated embryonal sarcoma
Children liver massess with elevated AFP
HCC and Hepatoblastoma
Children liver epithelial maassess
Fibrollamelar, hepatoblastoma and HCC
Children liver epithelial maassess
Fibrollamelar, hepatoblastoma and HCC
Trisomy 21 + hepatomegaly
Transient abnormal myelopoiesis
10% of T21
Preleukemic syndrome
20% progresses to leukemia
Hepatomegaly + placentomegaly + splenomegaly
First hepatomegaly, last hepatomegaly (After tx)
Hepatomegaly in congenital syphilis is first seen at 18 weeks
Fetal intrahepatic calcifications
Hepatomegaly + congenital hemocrhomatosis + hydrops
GALD (Gestational alloimmune liver disease)
What is the most common Todani choledochal cyst?
Type 1: Focal dilatation of the CBD
What are the rarest choledocal cysts?
Type 2 and 3.
Type 2: Diverticulum from the CBD
Type 3: Choledechocele
rare todani
Type III
What has cysts within and outside?
Todani IV: next most common
What has cysts insides and is called disease
Caroli disease (Type V) Association with liver fibrosis
What is a dilated cystic duct ?
Todani VI
What todani undergoes surgical resection?
Type I, II and IV
Rpux en Y Hepaticojejunostomy
Roux limb connects to the biliary ducts.
What todani undergoes this procedure?
Todani III (choledococele)
What is the difference between caroli disease and caroli syndrome
The syndrome includes hepatic fibrosis.
Caroli disease is just the dilatation.
Carolid disease is associated with caroli syndrome, ARPKD, ADPKD, and medullary sponge kidney
Autosomal dominant syndrome associated with epistaxis
Hereditary hemorrhagic telangiectasia.
AVM in the lung: Brain abscess
10% die of strokes, cerebral abscess or massive hemorrhage.
Proliferation of intrahepatic biliary ducts + 2 weeks neontal inctericia
Biliary atresia
90% spontaneous
10% syndromic: heterotaxy, polysplenia, inverte IVC
Ecogenic triange: atretic extrahepatic biliary ducts
Fenobarbital 5mg/kg/5 days
Obliteration of intrahepatic biliary ducts + > 2 weeks jaundice
Alagille syndrome (aRTERIOhepatic dysplasia)
Peripheral pulmonary artery stenosis, nephrocalcinosis, coarctation of arota.
Splenomegaly + gallstones in kids
Sickle cell disease.
If associated heart failure: Acute splenic sequestration (most common cause of death ins SCD kids)
If assocaited abdomina pain: Acute infarct.
Splenomegaly may lead to abscess in this kids.
Transposition of organ within the chest and abdomen with associated absent IVC and asplenia/polysplenia syndromes
Situs inversus totalis
Asplenia, transposition of IVC (To the left), bilateral eparterial bronchi, severe congenital heart diseases, bilateral right atria, intestinal malrotation, bilateral pulmonary/left atria, malrotation
Right isomerism
Immunocompromised due to absent spleen
Severe congnital cardiac anomalies
Polysplenia, azygous continuation, not many congenitl cardiac anomalies and bilateral hyparterial bronchus.
Left isomerism
MeTAnephros
Renal Tubules (Kidney)
MeSonephros
Sobras (Wolfian ducts, Mullerian ducts).
Renal agenesis in abscense of meSonpehrosi is due to lack of induction of the ureteric bud to the Metanephrosi to produce renal tissue.
Most common type of renal fusion anomaly
Inferior mesenteric artery prevents normal ascend.
Wilms, RENAL CARCINOID and TCC
Turner syndrome
Predominantly occurs in the right side
Crossed fuse renal ectopia
Ectopic kidney is inferior
Most common congenital genitourinary anomaly in neonates
Ureteropelvic junction obstruction
MAG 3 agent of choice (HIGH extraction when compared to DTPA, especially necessary in the context of obstruction)
40% accessory aberrant lower pole early branching vessels compress the ureter.
Liver fibrosis is always present
ARPKD:
Mutation PKHD1, chromosome 6 p
Most common vascular anomaly in neonates
Neonatal renal vein thrombosis: Dejhydrated, SCKD, Mother with Diabetes mellitus.
Usually left
Increased RI, hypoechoic kidney
Severe hypertension
Renal artery thrombosis: Associated with umbilical catheters
Cyptorchidism, hydroureteronephrosis (+renal dysplasia) and …. deficiency of abdominal musculature
Prune Belly syndrome
Non extrinsic ureteral compression or obstruction.
Primary (congenital) megaureter:
- Obstructive (Hirschprung/Acalasia like).
- Reflux: From VCUR
- Non obstructive nonreflux: Unknown etiology
Fishhook / reverse J appearance…
Circumcaval (Retrocaval) ureter
Girl with urinary incontinence
Upper pole of duplicated kidney inserted in the vagina\
Duplication is due to two separate ureteric buds arise from a single Wolffian duct. The future lower pole ureter separates from Wolffian duct earlier and thus migrates superiorly and laterally as the urogenital sinus grows.
Association: Fanconi anemia
Cobra head
Ureterocele: Upper pole duplicated system
Most common cause of urethral obstruction in male/Wolfian duct remant
Posterior urethral valve
Failure of mesonephros to resolve (Wolfian remant)
Craniospinal defects, bowel atresia and Down synd
Staging
Vesicoureteral reflux 1 ureter 2 calyceal 3 calyceal blunting 4 tortuous 5 very tortous
Median umbilical ligament (if not obliterated)
Patent urachus.
Most common complication: Infection
Most common in males
Adenocarcinmoa
Umbilicus…
Umbilicus sinus
Urachal..
Urachal cyst
Vesicourachal….
Vesicourachal diverticulum
Associated with Hurley stick ureters and cryptorchidism
Bladder extrophy (in the spectrum of epispadia/cloacal extrophy) Increased risk of adenocarcinoma
