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Congenital gastrointestinal anomalies
- 1. DEVELOPMENTAL ANOMALIES OF GASTROINTESTINAL TRACT DR. DEV LAKHERA
- 2. Classification of developmental anomalies of GIT STRUCTURAL EMBRYOLOGICAL MALDEVELOPMENT Malrotation Oesophageal/ pyloric/ duodenal/ anorectal atresia Duplication cystIN UTERO (ISCHEMIC) COMPLICATIONS FUNCTIONAL • Meconium plug syndrome • -intestinal hypoperistalsis BOTH • Midgut volvulus • Agangliosis • Hypertrophic pyloric stenosis
- 3. Disorders of oesophagus Oesophageal atresia +/- Tracheo-oesophageal fistula Congenital oesophageal stenosis, webs and diverticula Extrinsic compression –foregut duplication cyst
- 4. Tracheo-oesophageal fistula Tracheo-oesophageal septum (5wks) 1 in 5000 births M:F VACTERL anomalies Down’s syndrome
- 5. Types Most common EA with distal fistula
- 6. Chest X-ray Dilated proximal esophageal pouch with coiled nasogastric tube within is diagnostic air in the stomach and the small bowel
- 8. ANTENATAL USG : Oesophageal atresia • polyhydramnios • Distended proximal esophageal pouch • Small gastric bubble
- 9. CONTRAST STUDIES: Should be avoided, fear of aspiration • Nonionic isoosmolar contrast medium • H-type fistulas are mostly at the thoracic inlet, between C7 and T2 vertebral bodies
- 10. Congenital stomach disorders Microgastria Gastric Atresia Antral Mucosal Diaphragm Duplication Cyst Malrotation
- 11. Microgastria Small, tubular, midline stomach Always associated with anomalies Failure to thrive
- 12. Antral Diaphragm Mucosal web positioned in the antrum If large enough, can cause gastric outlet obstruction.
- 13. Congenital Hypertrophic Pyloric Stenosis • 1 in 500, M>>F • Present between 2-12 wks • Clinical diagnosis : Mass palpation /Antral peristaltic waves Ultrasonography is the primary imaging method
- 14. On USG • Thickened hypoechoic pyloric muscle • Double layer of echogenic mucosa • Length >16mm • Thickness >3.5 mm
- 15. Transverse section shows the– “Bull’s eye” sign.
- 16. Xray and Barium
- 17. • ‘STRING SIGN’ - hypertrophied muscle mass causes elongation and narrowing of pyloric canal • “SHOULDER SIGN” -hypertrophy of the pyloric muscle
- 18. Duodenal obstruction (Atresia ,Stenosis, Webs) Duodenal atresia (1 in 10000) Most common of all intestinal atresia 25% Downs syndrome
- 20. Double bubble on antenatal USG
- 21. Duodenal web Incomplete duodenal obstruction
- 22. Duodenal web intraluminal diverticulum Windsock sign
- 23. MALROTATION Normal intestinal rotation Two Processes involved : Physiological midgut Herniation and Rotation : 6 wks -12 wks Fixation of mesentery :12 wks -20 wks
- 24. 6 weeks -physiologic herniation of the midgut through the umbilical orifice (UO). Superior mesenteric artery (SMA) acts as the axis prearterial segment postarterial limb
- 25. 90-degree counterclockwise rotation Predominant pre-arterial elongation By 12th week
- 26. Fixation By 3rd to 5th month there is resorption of dorsal mesentery The base of the normal small bowel mesentery
- 27. NONROTATION arrest of the midgut rotation after the first 90 degrees of rotation.
- 28. entire colon lies in the left side of abdomen
- 29. INCOMPLETE ROTATION AND MALFIXATION Failure to complete the final 180-degree rotation. Shortened mesenteric root -allows formation of elongated and mobile segments of colon. Midgut volvulus.
- 30. Classic malrotation Cecum lies left of the midline Fixed by Ladd bands (aberrant peritoneal bands )
- 31. REVERSED INTESTINAL ROTATION – Transverse colon lie behind the descending duodenum and the superior mesenteric artery cecum is can be medially placed
- 32. Midgut volvulus Narrow mesentery Suddenly presents with bilious vomiting Ischemia and necrosis Plain radiograph
- 33. corkscrew sign tapering or beaking of the bowel in complete obstruction malrotated bowel configuration Fluoroscopy: contrast study
- 34. Ultrasound clockwise whirlpool sign abnormal bowel dilated duodenum proximal to obstruction dilated fluid-filled loops of small bowel free intra-abdominal fluid
- 36. CT scan whirlpool sign malrotated bowel configuration bowel obstruction free fluid/free gas in advanced cases
- 37. Meckel’s Diverticulum congenital intestinal diverticulum omphalomesenteric duct fails to be completely obliterated Present with obstruction or ulceration Antimesenteric border Litters hernia
- 38. Xray – non specific SBFT with a large Meckel diverticulum
- 39. 99MTC (TECHNETIUM -99M PERTECHNETATE) SCANNING: ectopic gastric tissue is found in a Meckel's diverticulum
- 40. Mid to distal bowel defects High bowel obstruction – Bilious vomiting Low bowel obstruction – Failure to pass meconium (< 48 hrs)
- 41. Small Bowel Atresia / High intestinal obstruction Utero-vascular insults Decreased intestinal perfusion Ischaemia
- 42. Dilated bowel loops proximal to atresia Triple bubble PLAIN RADIOGRAPHY
- 43. Enema may demonstrate Microcolon
- 44. Meconium peritonitis Bowel perforates as a result of bowel obstruction, such as atresias or meconium ileus Meconium peritonitis and small bowel obstruction is highly suggestive of atresia.
- 45. Low bowel obstruction Difficult to differentiate on X-ray Contrast enema is usually required Water soluble contrast is preferred
- 46. Meconium ileus Meconium consists of succus entericus Cystic fibrosis > 80% Meconium – viscid distal ileum and colon
- 49. Enteric Duplication Cyst embryological abnormalities that are lined by intestinal mucosa distal ileum (35%) > distal esophagus (20%) > stomach (9%) > duodenum > jejunum.
- 51. ULTRASONOGRAPHY: Well defined, unilocular anechoic mass
- 52. Functional immaturity of colon Meconium plug syndrome/ small left colon syndrome Immaturity of bowel innervation Change in caliber in splenic flexure
- 53. Hirschsprung’s Disease Absence of ganglion cells in bowel wall Transition point found in the rectosigmoid (73%) > descending colon (14%) > more proximal colon (10%).
- 54. Barium enema Narrowed aganglionic segment irregular saw-toothed mucosal pattern Recto-sigmoid ratio <1 abnormal
- 55. Delayed radiographs (24 hours) prolonged retention of barium (strong indicator) when enema findings – inconclusive Confirmatory – rectal biopsy
- 56. Colonic Atresia Distended loops of bowel similar to those seen in low small bowel obstruction.
- 57. Anorectal Anomalies Anal atresia: Vacterl association range from a membranous separation to complete absence of the anus. RADIOGRAPH: Invertogram
- 58. ULTRASOUND: Delineating distance from the distal pouch to perineum CYSTOGRAPHY: Delineates associated fistulas between terminal bowel and urinary tract. CT & MRI Modalities of choice Help determine presence of puborectalis muscle, external sphincter and rectal pouch.
- 59. THANK YOU
- 60. fusiform manner and then with preferential growth of its dorsal wall
- 61. Mesenteric Cyst (Lymphangioma) congenital malformation arising due to sequestration of lymphatic vessels. SONOGRAPHY: thin-walled unilocular or multilocular cystic lesion useful to demonstrate the thin septations which may not be well seen on CT. CT and MRI: demonstrate variable characteristics of the cyst contents (usually water-to fat) depending upon whether fluid is chylous, infected or haemorrhagic.
- 62. Megacystis-microcolon-intestinal Hypoperistalsis Syndrome (Berdon Syndrome) pseudoatresia. functional small bowel obstruction with a microcolon, malrotation and a large unobstructed bladder UPPER GI CONTRAST STUDY: hypomotility of small bowel with retrograde peristalsis.
- 63. • “DOUBLE TRACT SIGN” – this refers to fluid, trapped in the mucosal folds in the center of an elongated pyloric canal seen as two sonolucent streaks in the center
- 66. THANK YOU
Notas del editor
- Megacystis-microcolon //• Jejuno-ileal atresia // Colonic atresia or stenosis….I will be discussing the common disorders…
- Esophageal atresia is the M/C congenital anomaly of esophagus >90 % have associated TEF
- Trachea and oesophagus arise from the foregut. these structures are separated by a septum . Any anomaly in separation of these structures will lead to esophageal atresia with or without a trachea-oesphageal fistula…..Equal incidence in males and females….Isolated esophageal’tresia is more commonly aassociated with down,s syndrome..complte imaging is required to assess other associated anomalies
- More than 80 %esophageal atresia and trachea is connected to the esophagus by a fistula …9-10 % have only esophageal atresia…6% ty no atresia only an abberent connection H type ( If small unlike others it may present later in childhood with episodes of choking coughing or cyanosis on feeding )
- When there is a possibility of esophageal atresia Presence atresia with a distal tracheo esophageal fistula. Absence eliminates the possibility of a distal fistula
- A feeding tube is coiled in the upper esophageal pouch (open arrows). Eleven ribs are present on the left and 12 on the right. Segmentation anomalies, including a hemivertebra, are seen at the thoracolumbar level (solid arrow). The right hemithorax is opacified, and the mediastinal contents are shifted to the right because the right lung is agenetic.
- USG of the fetus in the sagittal plane at the level of neck shows an anechoic pouch (anechoic, dilated, blind-ending proximal esophageal pouch) ..polyhydramnios
- best way to demonstrate H-type: Others can be bronchoscopy and endoscopy H-type tracheoesophageal fistula. The contrast agent injected into the upper esophagus fills the esophagus (E), the fistula (arrow), and the trachea (T). Isoosmolar –iodixanol visipaque
- All the stomach anomalies present with non billous vomiting
- severe GOR megaoesophagus// VACTERL sequence, small bowel malrotation, upperlimb reduction defects and asplenia …The moderately dilated duodenum is in the normal position.
- cause vomiting or failure to thrive in infants`…Antral web. Barium study demonstrates a thin, linear filling defect across the antrum (arrows).
- Vomiting non billous …If not in this timeline and bilious malrotation is suspected//
- Outllet function must be observed ,can be confused with pylorospasm (Passage of gastric contents into duodenum)
- The pyloric length ( is abnormally elongated to more than 17 mm)//The stomach is distended with fluid
- nonbilious vomiting shows gastric distention with relative paucity of gas distally A single streak of barium (the string sign,arrow) within the narrowed, elongated pyloric channel is seen…Pyloric stenosis. Barium meal showing an elongated pyloric canal and shouldering of the antrum due to the hypertrophied pyloric muscle
- Due to incomplete recanalization// Location : Just below the ampulla of vater Associated with :anomalies of pancreas, CBD and portal vein //
- Air / aflevel is present in the stomach and proximal duodenum …Absence of distal air
- patients also develop polyhydramnios - as was seen in this case.
- is not complete; rather there is a diaphragm or membrane or web that causes duodenal stenosis
- thin membrane or web can become stretched and propelled toward the distal duodenal lumen by constant peristalsis, creating the so-called windsock deformity//smoothly-marginated oval collection of barium with a thin lucent rim around it
- Any Arrest in these processes lead to malrotation and malfixation of intestine
- that the superior mesenteric artery (SMA) acts as the axis for midgut rotation/// (OMD) omphalomesenteric duct divides the`midgut into prearterial and postarterial limbs///The celiac axis (CA) is the major artery of the foregut; the inferior mesenteric artery (IMA) supplies the hindgut. CB, Cecal bud.
- Liver enlarges and pushes pre-arterial segment down…..Midgut elongates predominantly prearterial ….postarterial segment occupies the left side of the umbilical hernia. Viewed from the front, this represents a 90-degree counterclockwise rotation……By the 12th week, the colon completes a 270-degree counterclockwise rotation.
- Duodenojejunal juction (DJF) to the ileocaecal valve. It has a broad bse any defect in rotation or fixation acan cause a narrow mesentry which later causes volvulus.
- demonstrated incidentally on barium studies in older children or adults ..bowel is not very mobile and volvulus is not a common complication …small bowel (prearterial ) right side and colon (post arterial) left side.
- Single-contrast barium enema
- Incomplete resorption of the dorsal mesentery that //twist of malfixed intestines around the short mesentery
- extend from the malpositioned cecum across the duodenum and attach to the hilum of the liver, posterior peritoneum or abdominal wall Malrotation demonstrated by barium enema
- 180 degrees clockwise
- Fixation of mesentery does not occur and, as a result, the mesentery has a short root, which allows it to act as a pedicle (through which the SMA and SMV pass) around which volvulus can occur. //distended stomach and proximal duodenum with a tapered end
- spiral appearance of the distal duodenum and proximal jejunum seen in midgut volvulus. //congenital malrotation of the midgut, the distal duodenum and proximal jejunum do not cross the midline and instead pass inferiorly
- clockwise spiralling of the mesentery and superior mesenteric vessels.
- CECT images through the upper abdomen revealed abnormal bowel position around the SMA, with a characteristic swirling pattern -this is consistent with Midgut Volvulus. //thick-walled loops of ischemic right-sided small bowel loops with potential pneumatosis intestinalis and mesenteric edema
- apex of the physiologic herniation//wide spectrum of variation..May contain ectopic gastric tissue
- //Fluoroscopy may demonstrate the narrowed neck blind-ending sac on the antimesenteric border.
- It is actively accumulated and secreted by the mucoid cells of the gastric mucosa,[2] and therefore, technetate(VII) radiolabeled with Tc99m is injected into the body when looking for ectopic gastric tissue as is found in a Meckel's diverticulum with Meckel's scans
- child presenting with billous vomiting// In contrast to duodenal atresia they are due to ..rather than failure of canalization //Supine radiograph in a neonate shows three dilated loops of bowel
- Proximal jejunal atresia triple bubble sign, ///more distal atresia uniform dilatation & air-fluid levels.
- The colon appears smaller callibre and unused,. //This colonic appearance depends upon gestational timing of inujry earlier will present with microcolon
- Usually the perforation seals off and the bowel is intact at birth. Intraperitoneal meconium usually calcifies
- Failure to pass meconium in 48 hrs ( safer if perforation is suspected // any contrast has a therapeutic benefit in meconium ileus and functional immaturity of colon.
- (bile salts, bile acids, and debris from the intestinal mucosa) //granular/bubbly appearance of rlq
- . Abdominal scout radiograph shown marked distention of the small bowel and a “soap bubble”appearance in the right side of the abdomen a finding suggestive of mottled air and meconium///The caliber of the colon is slightly diminished on contrast enema. As contrast material refluxes into the dilated and obstructed terminal ileum (I), it is diluted by the intraluminal meconium
- ) US image shown dilated, fluid-filled intestinal loops containing echogenic material ( calcified meconium.
- and are contiguous with the small intestine wall// uncommon & occur anywhere in the gastrointestinal tract.//noncontrast CT hypodense rounded mass due to a jejenual duplication cyst.
- Barium study shows extrinsic impression on the body of the stomach in a case of gastric duplication
- Rarely the contents are reflective or contain septations secondary to hemorrhage or inspissated material within the lumen.
- Contrast enema i n a newborn term infant showing a relatively small left colon, transition zone at the splenic flexure and a large coiled meconium plug which was dislodged from the splenic flexure to the hepatic flexure during colonic filling
- always involves the anal canal and extends proximally for a variable distance.// upto splenic flexure//total colonic agangliosis//Palin radiograph shows markedly dilated colon, with no gas in the pelvis / rectum
- Normally in neonates rectum is of a greater caliber// Barium enema shows an abrupt transition from the narrow caliber rectosigmoid (aganglionic) to the larger caliber more proximal sigmoid colon//
- huge and disproportionately dilated loop of bowel//Image from a barium enema study demonstrates microcolon with complete obstruction to the retrograde flow of a barium enema study demonstrates microcolon with complete obstruction to the retrograde flow of barium in the transverse portion of the colon.
- A coin/metal piece is placed over the expected anus and the baby is turned upside down (for a minimum 3 minutes
- fourth week.. straight, hollow tube in the midsagittal plane