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GI Board Review
2019
Joan K. Zawin, MD
Nasal Obstruction in
Newborns
• Choanal atresia
• Pyriform aperture stenosis
• Nasolacrimal duct mucocele
• Nasoethmoid encephalocele
• Nasal dermoid
Choanal Atresia
• Atresia of posterior nasal cavity
• Buccopharyngeal membrane persistence vs.
faulty neural crest cell migration
• Most common congenital abnl. of nasal cavity
• 29% bony; 71% membranous and bony
Choanal Atresia
• 50 - 60% unilateral; chronic purulent rhinorrhea
• 75% bilateral cases have other anomalies
• CHARGE, DiGeorge, Aperts, Fetal ETOH,
Treacher Collins
• Bilateral cases present with severe respiratory
distress and cyanosis
• Newborns are obligate nasal breathers (6 mos.)
Choanal Atresia: Imaging
• High resolution non-contrast CT, bone filters
• Suction nares, nasal vasoconstrictors
• Findings:
• medial bowing and thickening of lateral nasal
wall
• enlarged posterior vomer
Congenital Nasal Pyriform
Aperture Stenosis
• Obstructed nasal inlet
• Isolated or associated with other findings:
• solitary central maxillary incisor
• Holoprosencephaly
• Hypopituitarism
• Deletion short arm chromosome 18
Pyriform Aperture Stenosis:
Imaging
• Non-contrast high resolution CT, bone filters
• Findings:
• thickened anteromedial maxilla
• narrowed anterior and inferior nasal passage
• small triangular hard palate
Esophageal Atresia
• Faulty division of foregut into tracheal and
esophageal channels, first month embryogenesis
• 1/3,000 live births
• Associations:
• Trisomy 21 [EA only], VACTREL
• cardiac (up to 49%), MSK (up to 25%)
• CCAM, DA, CDH, biliary atresia, sirenomelia
EA +/- TEF: Imaging
• CXR:
• air filled proximal esophageal pouch
• repogle fails to pass into stomach
• Differential diagnosis:
• posterior pharyngeal perforation - look for
pneumomediastinum
EA +/- TEF
• KUB:
• air in gastrointestinal tract indicates presence
of distal TEF - types III, IV, V
• No air in GI tract implies either no TEF or only
a proximal TEF - types I and II
EA/TEF Type III
EA + DA
Posterior Pharyngeal Perforation
Isolated TEF
• Presents later: recurrent pneumonia, difficult
feeding
• Esophagram:
• NG tube, lateral position, cine study
• FOV to include glottis - carina
• Called “H” -shaped but really “N”-shaped
Vomiting Infants
• Non-bilious:
• Gastroesophageal reflux
• Antral spasm
• Pyloric Stenosis
Gastric Outlet Obstruction
• Single Bubble
• Differential diagnosis:
• hypertrophic pyloric stenosis - acquired
• pylorospasm - ↑ incd. in adrenogenital syndrome
• gastric atresia - failure of recanalization
• assoc. with epidermolysis bullosa
• antral web
Hypertrophic Pyloric
Stenosis
• Idiopathic hypertrophy of circular muscles
• 2-12 wks of age; ♂: ♀ = 4/5:1
• Projectile non bilious vomiting;
• “olive”, visible gastric peristaltic wave
Hypertrophic Pyloric
Stenosis
• Etiology: unknown
• familial
• PG, erythromycin exposure
• higher incidence in CF patients
HPS: Imaging
• KUB:
• +/- distended stomach
• paucity of distal bowel gas
• gastric peristaltic wave
HPS: Imaging
• Ultrasound:
• Gallbladder fossa, right side down, glucose
water
• Single wall > 3mm, channel length> 16 mm
• Assess gastric peristalsis and emptying
• SMA/SMV orientation
Normal Pylorus
Pyloric
Stenosis
Normal Pylorus
HPS: Imaging
• Upper GI Series:
• Signs:
• shoulder, pyloric tit, beak, string, double
tract, mushroom
• delayed gastric emptying, 70% have GER
BiliousVomiting
• Duodenal atresia or stenosis
• Malrotation with midgut volvulous
• Duodenal hematoma
• Jejunal atresia
• Any distal bowel obstruction
Double Bubble
• Intrinsic:
• duodenal atresia, stenosis, webs
• duodenal hematoma
• Extrinsic:
• annular pancreas, Ladds bands, pre duodenal
portal vein, malrotation/midgut volvulus
Duodenal Atresia
• #1 upper GI tract obstruction in neonates
• Failure of recanalization (vs. other atresias which
are due to vascular accidents)
• Etiology unknown
• 50% associated with other malformations
• 30%DA have Trisomy 21, 28% malrotation, 33%
annular pancreas
Duodenal Atresia
• No distal bowel gas unless anomalous bile duct
anatomy
• Vomiting within hours of birth:
• 85% bilious, 15% non bilious
• Scaphoid abdomen
• No imaging Required
Duodenal Stenosis/Webs
• Less common than duodenal atresia
• Failure of recanalization
• Web with pin hole lumen→ windsock
diverticulum
• Associations: Trisomy 21, malrotation, annular
pancreas, CDH, VACTREL
Duodenal Stenosis
Duodenal Stenosis
Duodenal Stenosis
Normal Upper GI
• AP: DJJ to left of spine at level of pylorus
• Lateral: duodenum courses posterior →
inferior→ anterior→superior
• DJJ quite mobile in young children
Normal DJJ
Malrotation
• Abnormal fixation of small bowel mesentery
• Short mesenteric base prone to twisting along axis
of SMA (volvulus)
• Ligament of Treitz = where duodenum passes thru
transverse mesocolon and becomes
intraperitoneal
• Ladds bands : fibrous peritoneal bands, cecum to
porta hepatis
Malrotation
• Normally bowel rotates 270 degrees
counterclockwise as it returns into the fetal
abdominal cavity
• Associations:
• CDH
• omphalocele, gastroschisis
• heterotaxy syndromes
Malrotation/Midgut Volvulus
• 40% present within first 10 days of life
• > 90% within first 3 months
• Vovulus can occur at any age = SURGICAL
EMERGENCY
• Symptoms:
• bilious vomiting, abdominal pain
• failure to thrive
Malrotation/Midgut Volvulus
• Ladd Procedure:
• reduction of volvulus
• resection of dead bowel
• transection of Ladd’s bands
• small bowel placed on right
• colon placed on left
Midgut Volvulus: Imaging
• Plain films:
• might be normal
• distention of stomach and proximal duodenum
• diffuse small bowel dilatation due to ischemia
• pneumatosis, pneumperitoneum, PV gas
Malrotation
• UGI:
• DJJ to right of midline
• DJJ too low +/- extra duodenal loops
• Jejunal loops in RUQ
• If UGI equivocal do SBFT to asses position and mobility
of cecum
• Volvulus: “corkscrew” distal duodenum/proximal jejunum
Normal Duodenojejunal Junction
Malrotation
Malrotation
2 day old with bilious vomiting
Malrotation/Midgut volvulus
• Ultrasound: abnormal sma/smv orientation
• Volvulus:
• “whirl” sign of sma/smv
• dilated stomach and proximal jejunum
Normal Malrotation
Malrotation
Omphalocele
• Herniation of abdominal viscera into base of
umbilical cord
• Failure of migration of lateral mesodermal body folds
• Increased incidence:
• advanced maternal age, in vitro fertilization,
maternal obesity, bicornuate uterus
• 50% with chromosomal abnormalities
Omphalocele
• Midline sac, umbilical cord insertion at apex
• Sac = peritoneum + wharton’s jelly + amnion
• Contains liver, small bowel, rarely bladder
• Spectrum includes: ectopic cordis, bladder
extrophy, cloacal extrophy, epispadias
• Elevated 𝛂 fetoprotein
Omphalocele
• Trisomy 13 and 18
• Prune Belly Syndrome
• Beckwith Weidermann
• Pentology of Cantrell
• OEIS Complex
• bladder extrophy, imperforate anus, omphalocele,
spinal defects
Omphalocele
Omphalocele S/P Repair
Gastroschisis
• Congenital full thickness defect in paraumbilical
anterior abdominal wall - almost all right sided
• Usually small bowel herniated, occasionally
stomach, colon
• No sac → thickened bowel wall, poor peristalsis
• Not associated with chromosomal abnormalities
• Elevated 𝛂 fetoprotein
Gastroschisis
Gastroschsis
Closed Gastroschisis
• Abdominal wall defect closes in utero
• Herniated bowel loops infarct and either vanish
or mummify.
Congenital Diaphragmatic
Hernia
• Failure of fusion of the pleuroperitoneal folds
• Bochdalek = posterolateral
• Morgagni = anteromedial
• L:R = 5:1; #Boys>Girls
• Bowel, liver, spleen, kidney can herniate into
chest
Congential Diaphragmatic
Hernia
•Pleuroperitoneal canal remains open
•# Lt > Rt, #Bochdalek > Morgagni
•CXR: opaque hemithorax, bowel gas in
hemithorax, abnl NGT, contralateral shift heart
and mediastinum
•Complication: pulmonary hypoplasia
86
Congenital Diaphragmatic
Hernia
• Severe respiratory distress, schaphoid abdomen
• Bilateral pulmonary hypoplasia, no bowel fixation
• DDX:
• CPAM
• necrotizing pneumonia
Left Congenital Diaphragmatic Hernia
Left CDH: Pre and Post OP
Left CDH 15 months
Right CDH
Right CDH Post-OP
Right CDH UGI
Jejunal Atresia
• Secondary to in utero vascular accident
• No microcolon if isolated
• Triple bubble sign on KUB
• No further imaging required
Jejunal atresia
Jejunal Atresia
AIM X 2
• Appendicitis
• Adhesions
• Intussusception
• Internal/external hernia
• Meckels diverticulum
• Malrotation/midgut volvulus
Meckel’s Diverticulum
• Omphalomesenteric duct remnant (vitelline duct)

• Most common malformation of GI tract

• Antimesenteric, true diverticulum, communicates with ileal
lumen

• #1 sx = painless rectal bleeding

• Obstruction, diverticulitis +/- perforation, intussusception,
stone formation, tumors (carcinoid)

• Lifetime risk sx = 5%
Meckel’s Diverticulum
• Rule of 2’s:

• 2% population, #♂ = 2 x ♀

• 2 feet from ileocecal valve, 2 inches long

• 2 types of ectopic mucosa (gastric, pancreatic)

• Sx usually by 2 yrs. of age

• Littre hernia = meckel’s constricted in inguinal hernia
Meckels Diverticulitis
Abdominal pain and bilious vomiting
SBO - Internal hernia
Intussusception
• invagination of proximal loop (intussusceptum)
into distal loop (intussuscipiens)
• 90% idiopathic, ileocecal, < 3 y.o.
• winter and spring
• Clinical triad: vomiting, colicky pain, currant jelly
stool (< 30%)
Intussusception
• 10% secondary to lead point
• < 3 mos. and > 3 yo
• SB duplication, Meckels, polyp, hematoma
(HSP), stool (CF), lymphoma
• Untreated → bowel ischemia, infarction,
perforation.
Intussusception
Intussusception
• KUB:
• Soft tissue mass (crescent sign), SB obstruction,
pneumoperitoneum
• Ultrasound: 98 - 100% accurate
• “Target” or “Doughnut” signs TX
• “Pseudokidney” sign on longitudinal
• ileocolic 3 - 5 cm, ileoileal < 2.5 cm
• 50% ascites, not def. assoc. with perforation
Intussusception
Intussusception
Intussusception
Intussusception
Severe abdominal pain
Ileocolic Intussusception
Ileocolic Intussusception
Necrotizing Enterocolitis
• VLBW infants
• Infection + ischemia
• #1 location= TI and ascending colon
• Can occur anywhere
Necrotizing Enterocolitis
•Usually premature, 10% term infants
•Etiologies: enteral feeds, mesenteric ischemia,
infection
•KUB - best diagnostic tool
•Pneumatosis is dx of NEC
122
Necrotizing Enterocolitis
•KUB:
•distention, bowel wall thickening, single
distended loop, gasless abdomen
•Pneumatosis is dx of NEC
•Portal venous gas seen in 1/3 cases
123
NEC: Radiographic findings
• Normal
• Gaseous distention GI tract
• Asymmetric bowel distention
• Featureless bowel loops
• Separated bowel loops
• Fixed configuration of bowel loops
Necrotizing Enterocolitis
• Pneumotosis = definitive finding
• submucosal = bubble-like
• subserosal = curviliner
• Pneumoperitoneum → Surgery
• Portal venous gas - might be fleeting on KUB →
try ultrasound
126
Nonspecific Gaseous Distention
127
Wednesday, March 27, 2013
Abnormal Separation of Bowel Loops
128
Wednesday, March 27, 2013
Pneumatosis
NEC - Pneumatosis
Necrotizing Enterocolitis
•Pneumoperitoneum :
•football sign
•Riglers sign
•1/3 who perf will not have free air on KUB
•US: used to look for complex ascites, air in
bowel wall, PV gas, ? free air
135
Football Sign
137
138
139
140
142
Spontaneous Ileal
Perforation
143
Perforated Meckels
Air in Biliary Tree
147
148
Wednesday, March 27, 2013
PortalVenous Gas
149
Wednesday, March 27, 2013
X - Table Lateral
Portal Venous Gas
Pneumatosis - Ultrasound
Failure to Pass Meconium
•94% pass meconium during first 24 hrs.
•Exceptions: premies, perinatal anoxia, maternal
analgesia, maternal diabetes
•Initial evaluation = obstructive series
•bowel gas pattern, Ca2++, abnl air collections,
osseous abnl
•Exam of choice = contrast enema
167
Newborn Constipataion
•Functional causes:
•maternal meds, anesthesia
•maternal diabetes
•neonatal anoxia
•prematurity
168
Newborn Constipation
•Surgical:
•Imperforate anus, colonic atresia
•Hirschsprungs
•Meconium plug small left colon
•Meconium ileus
•Ileal atresia
169
170
Normal Neonatal BE
•Redundant colon
•Rectum wider than sigmoid at all ages
•Cecum may be high riding
•Difficult to reflux into TI
171
Normal Size Colon
•Hirschsprungs
•Meconium plug small left colon
•might be initial presentation of
Hirschsprungs
172
Hirschsprungs
•Short ( 80% in rectosigmoid) or long segment,
complete aganglionosis (3%)
•Absence of myenteric and submucosal ganglion
cells
•1/5000 births, # ♂ ≻ ♀
•↑ incd. in families, Down syndrome
173
Hirschsprungs
•Change in colon caliber subtle
•Aganglionic segment narrower, lacks stool, and
is spastic
•corregated rectum sign 2° to dennervation
hypersensitivity
174
Hirschsprung Disease
• Colonic aganglionosis → functional obstruction
• defective caudal migration of neural crest cells -
• myenteric (Auerbach) + submucosal
(Meissners) plexus
• always involves anus, continues proximally
without skips!
• sporadic, 8 - 10% familial, ↑ incd. in Trisomy 21
Hirschsprung Disease
• SX: distention, bilious vomiting, no meconium 24 - 48 hrs.
• 90% dx in newborns (enema might be normal)
• Contrast enema:
• neonates - water soluble; older - barium
• denervated bowel is small, spastic
• normal bowel is dilated TRANSITION ZONE
• Recto-sigmoid index < 1
• short segment, long segment, total colonic aganglionosis
• intaluminal SB calcification with total colonic HD
177
178
179
180
181
Hirschsprung Disease
Hirschsprung Disease
Hirschsprung Disease
Hirschsprung Disease
• DX: suction rectal biopsy, full thickness biopsy
• RX: surgical resection
• Untreated → enterocolitis, toxic megacolon,
sepsis, death
• DDX based on KUB:
• imperforate anus, colon atresia, meconium plug-
small left colon, meconium ileum, ileal atresia
Meconium Plug - Small Left
Colon Syndrome
• Transient functional obstruction of newborn colon
• Same sx as H.D.
• Etiology: immature ganglion cells?
• ↑ incd. with diabetic mothers and mag. sulfate
• NOT ASSOCIATED with CYSTIC FIBROSIS
• Temporary, excellent prognosis
Meconium Plug - Small Left
Colon Syndrome
• Water soluble contrast enema
• Rectum > sigmoid colon (R:S ratio > 1)
• left colon small to splenic flexure (transition
zone)
• +/- meconium plugs
Meconium Plug Small Left
Colon
Meconium Plug Small Left
Colon
190
Meconium Plug
191
Meconium plug
Hirschsprung’s
Microcolon
•Normal but unused colon, nl proportions
•Causes:
•ileal atresia, meconium ileus, proximal colon
atresia, complete aganglionosis
•Gastrograffin enema with reflux in dilated
loops
192
193
194
Prone
Supine
195
Ileal Atresia
Meconium Ileus
• distal ileal obstruction due to tenacious meconium
• complications: meconium peritonitis, ileal atresia
• essentially all have cystic fibrosis
• 15% CF patients present with MI
• Cystic Fibrosis:
• autosomal recessive, chromosome #7
• incidence 1/3000 live births
Meconium Ileus
• KUB:
• distal bowel obstruction
• bubbly radiolucencies RLQ
• few if any air fluid levels
• if complicated, calcification from meconium peritonitis
• Water soluble contrast enema:
• smallest microcolon
• pellets in TI
• Rx = serial hyper osmotic enemas; 70 - 80% success rate, 1-3% perforation
198
Meconium Ileus
199
Microcolon, Meconium Ileus
Meconium Ileus
Meconium Ileus
Meconium Ileus
Meconium Ileus
Meconium Peritonitis
• chemical peritonitis secondary to inter bowel perforation; 1/35,000 live
births
• leakage of sterile meconium → calcifications in 86% cases within 1 - 8
days of perforation
• Associations:
• obstructions including MI, volvulus, vascular insufficiency
• KUB: calcifications, soft tissue mass (giant cystic), dilated bowel,
ascites
• check scrotum - patent processes vaginalis
• Rx = surgery
Meconium Peritonitis
Cystic Meconium Peritonitis
Meconium Peritonitis
Ileal Atresia
• secondary to in utero ischemia
• more common that ileal stenosis
• rarely hereditary, French Canadians
• 10% assoc. with:
• malrotation, volulus, MI, omphalocele, gastroschisis
• Contrast enema :
• microcolon
• cannot reflux into dilated small bowel loops.
Ileal Atresia
Ileal Atresia
Imperforate Anus
• clinical diagnosis, no anal opening
• Low: no GU tract communication
• High: rectouretheral fistula in ♂, rectovaginal or
rectovestibular fistula in ♀
• KUB: dilated loops of bowel +/- intraluminal calcification
(especially in ♂)
• VATER or VACTREL
• Currarino Triad: abnl sacrum + ARM + presacral mass
Rectourethral Fistula
Rectovaginal Fistula
Button Battery Ingestion
• Children 6 years of age or less
• Lithium batteries 20 mm or larger
• Liquification necrosis due to NaOH generated by
current at the anode surface (narrower, negative
side)
• Esophageal perforation can occur within 6 hrs
• Perforation, strictures, TEF, esophageal-aortic fistula
Button Battery
Button Battery Perforation
Button Battery TEF
Button Battery Stricture
Vascular Ring
Vascular Ring
Vascular Ring
Thank you!
NewbornsWith Respiratory
Distress
•Transient tachypenia
•Hyaline membrane disease
•Meconium aspiration
•Neonatal pneumonia
•Space occupying lesions
•Congenital heart disease
223
CXR Assessment
•Lines and tubes
•Lungs, trachea, and pleural spaces
•Heart - size, shape, position
•Pulmonary vascularity
•Osseous structures
•Subphrenic structures
224
Normal CXR
•Inspiration:
•6 ribs anteriorly, 8 - 9 ribs posteriorly
•Trachea
• Heart: apex left, 50% of chest
•Thymus
225
226
Normal Newborn CXR
227
Normal Thymus
228
Thymic Atrophy
229
Normal CXR 5 year old
Transient Tachypnea
•Delivery before labor or c-section
•Excess fetal lung fluid
•Mild sx, resolves within 48 hrs.
•CXR: over inflated hazy lung, prominent
fissures, pleural effusion
230
Newborn TTN
232
48 hrs. old
233
TTN & Pneumomediastinum
Angel Wing Deformity
Hyaline Membrane Disease
•Premature infants
•Surfactant deficiency
•Sx immediate or soon after birth
•CXR: ground glass lungs, small volumes,
central air bronchograms
•Complications: PNTX, PDA/ CHF, BPD
236
237
238
239
240
HMD
PIE + PNTX
241
242
243
244
245
246
HMD Pulmonary
Hemorrhage
247
HMD BPD
248
Chronic Lung Disease
Meconium Aspiration
•Fetal distress, post-dates
•Chemical pneumonitis + air blocks
•CXR: over inflation with coarse bilateral
infiltrates
•Complications: PNTX, pulmonary hemorrhage
249
Meconium Aspiration
Neonatal Pneumonia
•Acquires in utero - CMV
•Acquired during birth - Herpes, Group B Strep
•CXR: Can mimic HMD with confluent areas,
pleural effusion
252
Group B Strep Pneumonia
Round Pneumonia
Congenital Lobar Emphysema
•Over distention of a lobe →
•mediastinal shift
•atelectasis of neighboring lung
•Initially fluid filled, the air filled
•LUL > RML > RUL
256
257
258
Congenital Lober
Emphysema
259
Congenital Lobar Emphysema
Congential Diaphragmatic
Hernia
•Pleuroperitoneal canal remains open
•# Lt > Rt, #Bochdalek > Morgagni
•CXR: opaque hemithorax, bowel gas in
hemithorax, abnl NGT, contralateral shift heart
and mediastinum
•Complication: pulmonary hypoplasia
260
263
CDH 6 wks. of age
264
265
Rt. CDH post op
LLL Sequestration
267
Posterior UrethralValves
268
PUV, bilateral PNTX & Pneumomediatinum
269
PUV, Pneumopericardium
TAPVR III
Pericardial Effusion
Asplenia, 2 minor fissures
Choanal Atresia
276
277
278
279
Wednesday, March 27, 2013
Cross Table Lateral
280
Wednesday,March27,2013
281
282
Wednesday, March 27, 2013
PortalVenous Gas
283
Wednesday, March 27, 2013
Necrotizing Enterocolitis
284
2013
Wednesday, March 27, 2013
Pneumatosis
285
Wednesday, March 27, 2013
Wednesday, March 27, 2013
286
Duodenal Atresia
UAB GI board review

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