Download - Congenital gastrointestinal anomalies
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DEVELOPMENTAL ANOMALIES OF GASTROINTESTINAL TRACT
DR. DEV LAKHERA
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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
BOTHMidgut volvulus
AgangliosisHypertrophic pyloric stenosis
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Disorders of oesophagus
Oesophageal atresia +/- Tracheo-oesophageal fistula
Congenital oesophageal stenosis, webs and diverticula
Extrinsic compression –foregut duplication cyst
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Tracheo-oesophageal fistula
Tracheo-oesophageal septum (5wks)
1 in 5000 births
M:F
VACTERL anomalies
Down’s syndrome
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Types
Most common EA with distal
fistula
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Chest X-ray
Dilated proximal esophageal pouch with
coiled nasogastric tube within is diagnostic
air in the stomach and the small bowel
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ANTENATAL USG
: Oesophageal atresia
• polyhydramnios
• Distended proximal esophageal
pouch
• Small gastric bubble
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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
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Congenital stomach disorders
Microgastria Gastric Atresia Antral Mucosal Diaphragm Duplication Cyst Malrotation
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Microgastria
Small, tubular, midline stomach
Always associated with anomalies
Failure to thrive
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Antral Diaphragm
Mucosal web positioned in the antrum
If large enough, can cause gastric outlet obstruction.
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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
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On USG
• Thickened hypoechoic pyloric muscle
• Double layer of echogenic mucosa
• Length >16mm
• Thickness >3.5 mm
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Transverse section shows the– “Bull’s eye” sign.
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Xray and Barium
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• ‘STRING SIGN’ - hypertrophied muscle mass causes elongation and narrowing of pyloric canal
• “SHOULDER SIGN” -hypertrophy of the pyloric muscle
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Duodenal obstruction (Atresia ,Stenosis, Webs)
Duodenal atresia (1 in 10000)
Most common of all intestinal atresia
25% Downs syndrome
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ABDOMINAL RADIOGRAPH:
TYPICAL “DOUBLE-BUBBLE SIGN”
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Double bubble on antenatal USG
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Duodenal web Incomplete duodenal obstruction
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Duodenal web intraluminal diverticulum Windsock sign
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MALROTATION
Normal intestinal rotation
Two Processes involved :
Physiological midgut Herniation and Rotation : 6 wks -12 wks
Fixation of mesentery :12 wks -20 wks
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6 weeks -physiologic herniation of the midgut through the umbilical orifice (UO).
Superior mesenteric artery (SMA) acts as the axis
prearterial segment
postarterial limb
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90-degree counterclockwise rotation Predominant pre-arterial elongation
By 12th week
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Fixation
By 3rd to 5th month there is resorption of dorsal mesentery
The base of the normal small bowel mesentery
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NONROTATION arrest of the midgut rotation after the first
90 degrees of rotation.
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entire colon lies in the left side of abdomen
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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.
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Classic malrotation Cecum lies left of the midline
Fixed by Ladd bands (aberrant peritoneal bands )
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REVERSED INTESTINAL ROTATION –
Transverse colon lie behind the descending duodenum and the superior mesenteric artery
cecum is can be medially placed
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Midgut volvulus
Narrow mesentery
Suddenly presents with bilious vomiting
Ischemia and necrosis
Plain radiograph
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corkscrew sign
tapering or beaking of the bowel in
complete obstruction
malrotated bowel configuration
Fluoroscopy: contrast study
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Ultrasound
clockwise whirlpool sign
abnormal bowel
dilated duodenum proximal to obstruction
dilated fluid-filled loops of small bowel
free intra-abdominal fluid
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CT scan
whirlpool sign
malrotated bowel configuration
bowel obstruction
free fluid/free gas in advanced
cases
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Meckel’s Diverticulum congenital intestinal diverticulum
omphalomesenteric duct fails to be completely obliterated
Present with obstruction or ulceration
Antimesenteric border
Litters hernia
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Xray – non specific
SBFT with a large Meckel diverticulum
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99MTC (TECHNETIUM -99M PERTECHNETATE) SCANNING:
ectopic gastric tissue is found in a Meckel's diverticulum
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Mid to distal bowel defects
High bowel obstruction – Bilious vomiting
Low bowel obstruction – Failure to pass meconium (< 48 hrs)
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Small Bowel Atresia / High intestinal obstruction
Utero-vascular insults
Decreased intestinal perfusion
Ischaemia
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Dilated bowel loops proximal to atresia
Triple bubble
PLAIN RADIOGRAPHY
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Enema may demonstrate Microcolon
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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.
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Low bowel obstruction
Difficult to differentiate on X-ray
Contrast enema is usually required
Water soluble contrast is preferred
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Meconium ileus Meconium consists of succus entericus
Cystic fibrosis > 80%
Meconium – viscid distal ileum and colon
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Ultrasound appearance
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Enteric Duplication Cyst
embryological abnormalities that are lined by intestinal mucosa
distal ileum (35%) > distal esophagus (20%) > stomach (9%) > duodenum > jejunum.
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ULTRASONOGRAPHY: Well defined, unilocular anechoic mass
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Functional immaturity of colon
Meconium plug syndrome/ small left colon syndrome
Immaturity of bowel innervation
Change in caliber in splenic flexure
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Hirschsprung’s Disease
Absence of ganglion cells in bowel wall
Transition point found in the rectosigmoid (73%) > descending colon (14%) > more proximal colon (10%).
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Barium enema
Narrowed aganglionic segment
irregular saw-toothed mucosal pattern
Recto-sigmoid ratio <1 abnormal
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Delayed radiographs (24 hours) prolonged retention of barium (strong indicator) when enema findings – inconclusive
Confirmatory – rectal biopsy
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Colonic Atresia
Distended loops of bowel similar to those seen in low small bowel obstruction.
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Anorectal Anomalies
Anal atresia: Vacterl association
range from a membranous separation to complete absence of the anus.
RADIOGRAPH: Invertogram
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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.
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THANK YOU
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fusiform manner and then with preferential
growth of its dorsal wall
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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.
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Megacystis-microcolon-intestinalHypoperistalsis 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.
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• “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
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THANK YOU