congenital gastrointestinal anomalies

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DEVELOPMENTAL ANOMALIES OF GASTROINTESTINAL TRACT DR. DEV LAKHERA

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Page 1: Congenital gastrointestinal  anomalies

DEVELOPMENTAL ANOMALIES OF GASTROINTESTINAL TRACT

DR. DEV LAKHERA

Page 2: Congenital gastrointestinal  anomalies

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.

Page 63: Congenital gastrointestinal  anomalies

• “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