Congenital Stomach Anomalies

• Note: presentation depends on degree of obstruction. Complete obstruction leads to significant symptoms within hrs, partial obstruction may not be discovered until later in childhood or even into adulthood.
  
• 2 main types: Pylric atresia and Antral web
  
• Pyloric Atresia
• infants presenting with non-bilious vomiting, feeding difficulties, can lead to metabolic dergangments similar to IHPS
  
• abdominal distension during the first day of life (this helps to differentiate from IPHS which does not appear within the first few days of life)
  
• Rupture of stomach may occur in first 12hrs if Dx delayed or missed
  
• large gastric aspirate, >20mL, should be removed to prevent aspiration
  
• Polyhydramnios
• low birth weight is common
• Assoc w/
• epidermlysis bullosa
• icthyosis
• aplasia cutis congenita
• pyloric web may be missed in duodenal atresia
  
• Antral Web (1cm or more proximal to the pylorus)
  
• typically less dramatic Sx, depends on degree of obstruction
• nausea
• vomiting
• abd pain
• weight loss
  
• Diagnosis of gastric outlet obstruction
• Prenatal polyhydramnios and Large dilated stomach and narrowed outlet seen on prenatal ultrasound
• Postnatal Plain films reveal "single bubble" with large distended stomach and absence of distal intestinal gas.
• If partial obstruction (e.g. distal gas noted), contrast studies or US may be helpful
  
• Incomplete prepyloric membrane is seen as thin, linear filling defect on contrast studies
• Upper GI series can reveal pyloric dimple sign for pyloric atresia which appears as a shallow cavity at the proximal point of the atresia
  
• US reveals echogenic band extrending centrally from the lesser and greater curvatures into the prepyloric region
• Endoscopy can directly visualize the defect
• Note: Obstruction is proximal to the ampulla of Vater - so patient will have passage of meconium stool. some may see this and think the diagnosis is not obstruction.
  
• Treatment consists of restoring volume and correcting metabolic derangements followed by surgical or endoscopic repair
• Endoscopic advances
• alternative treatment strategies (limited outcome data available) including
• balloon dilation
• laser web excisions
• laser radial incisions of a type I membrane
  
• Surgery
• Gastrostomy and distal passage of cath may be required to detect membranous obstructions
• Excision with Heineke-Mikulicz or Finney Pyloroplasty
• Gastrododenostomy is necessary if pyloric atresia has atretic ends seperated by a cordlike or discontinuous segment
  
• Recent reports of gastroduodenal mucosal advancement anastomosis, with reconstruction of the pyloric sphincter have acheived some success
• Post-op stomach decompression (NG-tube)
  
• Enteral nutrition is commenced during the first pot-op week if no complications present
• Long term follow up is essential
• DDx:
• obstruction of gastric outlet with
  
• intrinsic lesions - chronic granulomatous disease or infantile myofibromatosis
• extrinsic lesions - annular pancreatic tissue or congenital perintoneal bands compressing gastric outlet
• Outcomes
• Death is associated with delayed diagnosis
• Junctional epidermolysis bullosa/pyloric atresia syndrome has poor outcomes (54 of 70 Cases reported ended in death by 11mo of age)
  
• This is due to risk of:
• sepsis
• malabsorption
• renal disease
• Failure to thrive
• Mutations of PLEC1 gene
  
• History
• 1749, Calder reported first case of congenital pyloric atresia
• 1940, Touroff performed the first successful operation
  

• Cyctic or tubular structures that occur within the wall of the stomach
• Gastric duplications account for 2-7% of all GI duplications. It is uncommon
  
• Etiology
• several hypotheses
• notochord theory
• diverticulation
• canalization defects
• caudal twinning
  
• Features
• most commonly found over greater curvature
• <12cm in diameter
• usually does not communicate with the stomach, but they do have a common blood supply
  
• Female: Male = 2:1
  
• Histology
• alimetary -columnar epithelium
• submucosa
• smooth muscle coat
• Clinical Manifestations
• 33% with palpable cyst - may be confused with "olive" of IHPS
  
• symptoms of Gastric outlet obstruction (nausea, emesis, abd pain, weight loss)
• If communicating, can produce gastric ulceration with hematemesis or melena
• Bleeding can also occur is ectopic gastric tissue present (30%)
• volvulus
• perforation
• May have co-existing pancreatic duplication - pancreatitis
  
• Dx
• Upper GI series may show extrinsic defect on lesser curve
• Radiographic studies usually show a paragastric mass displacing the stomach
  
• CT or US may outline cystic structure
• US may show inner hyperechoic and outer hypoechoic muscle layers typicall of GI duplications
• Direct visualization via endoscopy has been descibed
• MRI - becoming more popular
  
• Tx
• Surgical Excision
  
• common vasculature and common wall complicate surgery
• outcomes following surgery are usually excellent

• Acute gastric volvulus (esp intrathoracic) is a surgical emergency requiring reduction of volvulus to prevent gastric eschemia, necrosis, and perforation.
• Etiology
• The stomach is generally resistant to rotation because it is fixed at the gastroesophageal junction and the pylorus. The stomach is also tethered by 4 gastric ligaments:
• gastrohepatic
• gastrosplenic
• gastrocolic
• gastrophrenic
• Volvulus occurs when an attachment is absent or elongated which allows the stomach to rotate around itself resulting in obstruction of the pylorus or cardia
  
• organoaxial - around the longitudinal esophagogastro-pyloric axis
• mesentroaxial - around the transverse axis, through the greater and lesser curvatures
• Combined - through both
• Sometimes associated with:
• intestinal malrotation
• diaphragmatic defects (i.e. congenital diaphragmatic hernia)
  
• hiatal hernia
• adjacent organ abnormalities: asplenia
• Clinical Manifestations
  
• Borchardt Triad As noted in Walker (Per Fellows Review) difficult to elicit in infants
  
• Unproductive retching (Intractable emesis and retching)
  
• Localized epigastric distention (Sudden, severe epigastric pain)
  
• Inability to pass NG Tube (Inability to pass tube into the stomach)
  
• Typically presents in the first few months of life, consider in children with:
• chronic reflux, emesis, FTT
• nonspecific, high obstructive symptoms
• Dx
• UGI series (contrast studies may be most helpful)
• dilated stomach
• Double fluid level on upright abd film with characteristic beak near lower esophageal junction in mesenteroaxial volvulus
• Single air fluid level is seen without the characteristic beak with stomach lying in a horizontal plan in organoaxial voluvlus
• CT
  
• Tx:
• Acute Cases
  
• Emergent surgical correction with gastropexy and correction of any associated anomalies/defects.
  
• Chronic Cases
• Patients typically older
• Surgical repair is controversial
• Conservative management with gradual improvement reported

• Very Rare - only 60 cases reported ever (first reported in 1842)
• Failure of gastric enlargement during embryogenesis results in tubular stomach with smaller capacity
• Commonly associated with other defects/malformations:
• 84% - Intestinal
• 43% - Cardiovascular
• 33% - Pulmonary
• 31% - Skeletal
• 28% - Urogenital
• 12% - Neuronal
• Specific Defects:
• intestinal malrotation, asplenia, transverse liver, TE anomalies, AV septal defects, upper limb and spinal deformities, micrognathia - Pierre Robin sequence, renal dysplasia or aplasia, corpus callosum agenesis, and anopthalmia
• Exclude Microgastria in patients with VACTERL and midline defects (Situs Inversus)
• Etiology
• Genetic Cause Unkown
• BMP signaling pathway may play a role - overexpression in BMP4 results in the microgastria phenotype in animal models
• Clinical Manifestations
• Symptoms secondary to reduced capacity of the stomac
• postprandial vomiting
• GER
  
• aspiration with recurrent infections
• rapid gastric emptying leading to diarrhea
• malnutrition
• dumping syndrome
• FTT, developmental delay
• Dx
• Upper GI Contrast study
• small, tubular stomach
• abnormal, usually midline position
• dilated, poorly peristaltic esophagus
• Tx:
• Conservative management with frequent small volume, high calorie feeds
• NJ Tube feeds or Jejunostomy with continuous feeds
• Treat GER
• Prokinetic agents to assist with gastric emptying
• Surgery
• Reserved for severe cases or failure of conservative small frequent feeds
• Goal: increase capacity and draining of the stomach as well as prevent or resolve dialtion of esophagus
• Attach jejunal puch to stomach and form distal roux-en-Y jejunojejunostomy (Hunt-Lawrence Pouch) - variable outcome data
  
• Correct associated anomalies

• Very Rare
• Occur in posterior wall, antrum, and pylorus
• Usually through all layers of stomach wall
• Can be associated with hiatal hernia or aberrant pancreatic tissue
• Clinical Manifestations
• usually presents in adult life
• children with recurrent abd pain and vomiting
• Dx
• Upper Endoscopy
  
• Contrast studies
• reveal outpouching
• Differentiate from Gastric Ulcer and Malignancy
• Tx
• Surgical Excision
  

• Spontaneous gastric perforation usually occurs in the first 3-5 days of life
• Cause believed to be multifactorial leading to common end pathway of ischemia
• congenital muscular defect of the gastric wall
• bacterial colonization of gut with pathogenic organisms
• immaturity of the immune system
• fetal stress, hypoxia, hypovolemia may trigger redistribution of blood flow
  
• Clinical Manifestations:
  
• abrupt, rapidly progressive abd distension is the hallmark symptom
• tachycardia and lethargy secondary to hypovolemia and decreased perfusion
• resp difficulty and massive pneumoperitoneum may be the first sign
• Severe fetal stress increases risk. Careful observation in the following cases:
  
• abruptio placentae
• placenta previa
• amnionitis
  
• infants delivered by emergency cesarean section
• Dx:
• free air on abd plain films
• eval should include:
• leukocyte and platelet counts
• arterial pH
• blood gas
• Tx:
• Patients can rapidly decompensate so early identification and treatment are essential
• ABC -intubate and ventilate if necessary
  
• rapid fluid resuscitation
• blood transfusion
• correction of acidosis
• administration of antibiotics
• orogastric suction
• maintenance of body temperature
• Labs
• limit to essential diagnostic studies
• Paracentesis (blunt needle or platic catheter) -remove pneumoperitoneum and prevent elevation of diaphragm to relieve potential life threatening resp distress
• Emergency Laparotomy through transverse supraumbilical abdominal incision
• aerobic, anaerobic, and fungal Cx of peritoneal contents
• Perf may be occult: careful exam of stomach, esophageal hiatus to lesser omentum necessary
• most perforations occur along greater curvature
• perforation due to duodenal ulcer appears on the anterior wall of the duodenum or near the pyloroduodenal junction
• gastric ulcer may perforate along lesser curvature near antral fundic junction
• Stomach perforations can usually be debrided and undergo primary closure
• if greater curvature extensively involved with more necrosis - resection is indicated
• extensive necrosis of the gastric antrum, a partial gastrectomy and gastroenterostomy may be necessary
• A bilroth I (gastroduodenostomy) is preferable
• Survival is 75-80%
• Multiple organ dysfunction leads to higher mortality

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