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UAB GI board review
- 1. GI Board Review 2019 Joan K. Zawin, MD
- 2. Nasal Obstruction in Newborns • Choanal atresia • Pyriform aperture stenosis • Nasolacrimal duct mucocele • Nasoethmoid encephalocele • Nasal dermoid
- 3. 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
- 4. 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.)
- 5. 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
- 7. 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
- 8. 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
- 10. 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
- 12. EA +/- TEF: Imaging • CXR: • air filled proximal esophageal pouch • repogle fails to pass into stomach • Differential diagnosis: • posterior pharyngeal perforation - look for pneumomediastinum
- 13. 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
- 16. EA/TEF Type III
- 19. EA + DA
- 21. 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
- 23. Vomiting Infants • Non-bilious: • Gastroesophageal reflux • Antral spasm • Pyloric Stenosis
- 24. 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
- 25. 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
- 26. Hypertrophic Pyloric Stenosis • Etiology: unknown • familial • PG, erythromycin exposure • higher incidence in CF patients
- 28. HPS: Imaging • KUB: • +/- distended stomach • paucity of distal bowel gas • gastric peristaltic wave
- 31. 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
- 32. Normal Pylorus
- 37. HPS: Imaging • Upper GI Series: • Signs: • shoulder, pyloric tit, beak, string, double tract, mushroom • delayed gastric emptying, 70% have GER
- 40. BiliousVomiting • Duodenal atresia or stenosis • Malrotation with midgut volvulous • Duodenal hematoma • Jejunal atresia • Any distal bowel obstruction
- 41. Double Bubble • Intrinsic: • duodenal atresia, stenosis, webs • duodenal hematoma • Extrinsic: • annular pancreas, Ladds bands, pre duodenal portal vein, malrotation/midgut volvulus
- 42. 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
- 44. 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
- 46. 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
- 50. 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
- 51. Normal DJJ
- 52. 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
- 54. Malrotation • Normally bowel rotates 270 degrees counterclockwise as it returns into the fetal abdominal cavity • Associations: • CDH • omphalocele, gastroschisis • heterotaxy syndromes
- 55. 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
- 56. 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
- 57. 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
- 58. 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
- 60. Malrotation
- 61. Malrotation
- 62. 2 day old with bilious vomiting
- 69. Malrotation/Midgut volvulus • Ultrasound: abnormal sma/smv orientation • Volvulus: • “whirl” sign of sma/smv • dilated stomach and proximal jejunum
- 71. Malrotation
- 72. 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
- 73. 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
- 75. Omphalocele • Trisomy 13 and 18 • Prune Belly Syndrome • Beckwith Weidermann • Pentology of Cantrell • OEIS Complex • bladder extrophy, imperforate anus, omphalocele, spinal defects
- 76. Omphalocele
- 79. 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
- 81. Gastroschisis
- 82. Gastroschsis
- 84. Closed Gastroschisis • Abdominal wall defect closes in utero • Herniated bowel loops infarct and either vanish or mummify.
- 85. 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
- 86. 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
- 87. Congenital Diaphragmatic Hernia • Severe respiratory distress, schaphoid abdomen • Bilateral pulmonary hypoplasia, no bowel fixation • DDX: • CPAM • necrotizing pneumonia
- 90. Left Congenital Diaphragmatic Hernia
- 91. Left CDH: Pre and Post OP
- 92. Left CDH 15 months
- 93. Right CDH
- 95. Right CDH UGI
- 96. Jejunal Atresia • Secondary to in utero vascular accident • No microcolon if isolated • Triple bubble sign on KUB • No further imaging required
- 97. Jejunal atresia
- 98. Jejunal Atresia
- 99. AIM X 2 • Appendicitis • Adhesions • Intussusception • Internal/external hernia • Meckels diverticulum • Malrotation/midgut volvulus
- 100. 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%
- 101. 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
- 105. Abdominal pain and bilious vomiting
- 107. SBO - Internal hernia
- 108. 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%)
- 109. 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.
- 110. Intussusception
- 111. 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
- 112. Intussusception
- 113. Intussusception
- 114. Intussusception
- 115. Intussusception
- 121. Necrotizing Enterocolitis • VLBW infants • Infection + ischemia • #1 location= TI and ascending colon • Can occur anywhere
- 122. Necrotizing Enterocolitis •Usually premature, 10% term infants •Etiologies: enteral feeds, mesenteric ischemia, infection •KUB - best diagnostic tool •Pneumatosis is dx of NEC 122
- 123. 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
- 124. NEC: Radiographic findings • Normal • Gaseous distention GI tract • Asymmetric bowel distention • Featureless bowel loops • Separated bowel loops • Fixed configuration of bowel loops
- 125. Necrotizing Enterocolitis • Pneumotosis = definitive finding • submucosal = bubble-like • subserosal = curviliner • Pneumoperitoneum → Surgery • Portal venous gas - might be fleeting on KUB → try ultrasound
- 127. 127 Wednesday, March 27, 2013 Abnormal Separation of Bowel Loops
- 128. 128 Wednesday, March 27, 2013 Pneumatosis
- 132. NEC - Pneumatosis
- 135. 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
- 136. Football Sign
- 137. 137
- 138. 138
- 139. 139
- 140. 140
- 146. Air in Biliary Tree
- 147. 147
- 148. 148 Wednesday, March 27, 2013 PortalVenous Gas
- 149. 149 Wednesday, March 27, 2013 X - Table Lateral
- 151. Portal Venous Gas
- 167. 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
- 168. Newborn Constipataion •Functional causes: •maternal meds, anesthesia •maternal diabetes •neonatal anoxia •prematurity 168
- 169. Newborn Constipation •Surgical: •Imperforate anus, colonic atresia •Hirschsprungs •Meconium plug small left colon •Meconium ileus •Ileal atresia 169
- 170. 170
- 171. Normal Neonatal BE •Redundant colon •Rectum wider than sigmoid at all ages •Cecum may be high riding •Difficult to reflux into TI 171
- 172. Normal Size Colon •Hirschsprungs •Meconium plug small left colon •might be initial presentation of Hirschsprungs 172
- 173. 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
- 174. Hirschsprungs •Change in colon caliber subtle •Aganglionic segment narrower, lacks stool, and is spastic •corregated rectum sign 2° to dennervation hypersensitivity 174
- 175. 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
- 176. 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. 177
- 178. 178
- 179. 179
- 180. 180
- 181. 181
- 182. Hirschsprung Disease
- 183. Hirschsprung Disease
- 184. Hirschsprung Disease
- 185. 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
- 186. 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
- 187. 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
- 188. Meconium Plug Small Left Colon
- 189. Meconium Plug Small Left Colon
- 190. 190 Meconium Plug
- 192. 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. 193
- 194. 194 Prone Supine
- 195. 195 Ileal Atresia
- 196. 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
- 197. 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. 198 Meconium Ileus
- 200. Meconium Ileus
- 201. Meconium Ileus
- 202. Meconium Ileus
- 203. Meconium Ileus
- 204. 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
- 205. Meconium Peritonitis
- 207. Meconium Peritonitis
- 208. 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.
- 209. Ileal Atresia
- 210. Ileal Atresia
- 211. 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
- 213. Rectovaginal Fistula
- 214. 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
- 215. Button Battery
- 217. Button Battery TEF
- 219. Vascular Ring
- 220. Vascular Ring
- 221. Vascular Ring
- 222. Thank you!
- 223. NewbornsWith Respiratory Distress •Transient tachypenia •Hyaline membrane disease •Meconium aspiration •Neonatal pneumonia •Space occupying lesions •Congenital heart disease 223
- 224. CXR Assessment •Lines and tubes •Lungs, trachea, and pleural spaces •Heart - size, shape, position •Pulmonary vascularity •Osseous structures •Subphrenic structures 224
- 225. Normal CXR •Inspiration: •6 ribs anteriorly, 8 - 9 ribs posteriorly •Trachea • Heart: apex left, 50% of chest •Thymus 225
- 227. 227 Normal Thymus
- 228. 228 Thymic Atrophy
- 229. 229 Normal CXR 5 year old
- 230. 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
- 231. Newborn TTN
- 232. 232 48 hrs. old
- 233. 233
- 235. Angel Wing Deformity
- 236. 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. 237
- 238. 238
- 239. 239
- 240. 240 HMD PIE + PNTX
- 241. 241
- 242. 242
- 243. 243
- 244. 244
- 245. 245
- 247. 247 HMD BPD
- 249. Meconium Aspiration •Fetal distress, post-dates •Chemical pneumonitis + air blocks •CXR: over inflation with coarse bilateral infiltrates •Complications: PNTX, pulmonary hemorrhage 249
- 250. Meconium Aspiration
- 252. Neonatal Pneumonia •Acquires in utero - CMV •Acquired during birth - Herpes, Group B Strep •CXR: Can mimic HMD with confluent areas, pleural effusion 252
- 253. Group B Strep Pneumonia
- 255. Round Pneumonia
- 256. 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. 257
- 260. 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. 263 CDH 6 wks. of age
- 264. 264
- 265. 265 Rt. CDH post op
- 266. LLL Sequestration
- 268. 268 PUV, bilateral PNTX & Pneumomediatinum
- 271. TAPVR III
- 272. Pericardial Effusion
- 273. Asplenia, 2 minor fissures
- 274. Choanal Atresia
- 276. 276
- 277. 277
- 278. 278
- 279. 279 Wednesday, March 27, 2013 Cross Table Lateral
- 281. 281
- 282. 282 Wednesday, March 27, 2013 PortalVenous Gas
- 283. 283 Wednesday, March 27, 2013 Necrotizing Enterocolitis
- 284. 284 2013 Wednesday, March 27, 2013 Pneumatosis
- 285. 285 Wednesday, March 27, 2013 Wednesday, March 27, 2013
- 286. 286
- 287. Duodenal Atresia