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34 Cards in this Set
- Front
- Back
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congenital disorder
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a disorder that you are born with
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developmental disorder
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a disorder that develops after birth
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Development of the GI Tract in utero
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GI tract formed during fourth week of gestation
Appears as 4 mm hollow tube Buccopharyngeal and cloacal membranes rupture during the third and seventh weeks respectively By end of 2nd trimester, organs well formed and display early function |
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Foregut
Components |
Esophagus
Stomach Duodenum Liver Gallbladder Pancreas |
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Midgut
Components |
Jejunum
Ileum Ascending Colon Transverse Colon |
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Hindgut
Components |
Descending Colon
Rectosigmoid Colon |
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Esophageal Atresia and Tacheoesophageal Fistula
Epidemiology |
1 in 3000-5000 live births
M=F 50% of infants have associated anomalies many varieties |
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Esophageal Atresia and Tacheoesophageal Fistula
Embryology |
Foregut is partitioned by a septum into two separate tubes
The anterior trachea develops cartilaginous rings and lung buds The posterior esophagus stretches from pharynx to stomach Incomplete tubular separation may occur leading to anomalies |
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Esophageal Atresia and Tacheoesophageal Fistula
Associated Anomalies |
Polyhydraminos in 50% of mothers (b/c baby cannot swallow fluid)
Cardiovascular-PDA, VSD, ASD Gastrointestinal-imperforate anus, duodenal atresia Skeletal-vertebral anomalies Genitourinary-hypospadias Other-trisomy |
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What is the most common esophageal atresia, tracheoesophageal fistula?
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in 85-90% of babues with disorders
esophageal atresia with distal fistula |
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What is the H tracheo-esophageal fistula?
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only 3% of babies
no esophageal atresia, but there is a tracheo-esophageal fistula |
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Esophageal atresia with Distal Tracheoesophageal Fistula
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85% of total esophageal defects
Esophageal gap 1 to 2 cm Distal TEF joins trachea at carina Diagnosis made by placing nasogastric tube (that cannot pass beyond upper chest) Cough, choking, aspiration with first feed |
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Pure Esophageal Atresia
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10% of congenital esophageal defects
Absence of gas within the GI tract (on xray b/c lungs are not connected and you cannot swallow air) 20% will have Down’s syndrome If the gap between the esophageal pouches is > 4 cm primary anastomosis is difficult ... therefore stretch colon or insert colon |
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Why is it a problem if you put a part of the colon in the esophagus?
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because there is no parastalsis... dysphagia
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H-Tracheoesophageal Fistula
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3% to 5% of cases
Rarely have other anomalies REPEATED episodes of PNEUMONIA Esophagus and trachea otherwise normal |
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Pyloric Stenosis
Epidemiology |
Developmental Problem
1 in 900 to 1000 live births Nonbilious vomiting (b/c obstruction is prior to bile duct) Hypochloremic alkalosis (b/c vomiting acid) Presents 1 week to 5 months of life (usually at 6 wks) Pyloric mass on PE |
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What is the most frequent surgical disorder of the stomach in babies?
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Pyloric stenosis
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Pyloric Stenosis
Incidence and Heredity |
Most frequent surgical disorder of the stomach
Incidence variable 1 per 1,000 live births Males to females 4-6:1 Positive family history 13% of time |
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Pyloric Stenosis
Pathogenesis |
Pyloric muscle hypertrophy from gastric peristalsis against closed pyloric canal
Hypergastrinemia with hyperacidity Elevations of prostaglandins leading to smooth muscle constriction |
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How do you surgically treat pyloric stenosis?
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cut the hypertrophied muscle to allow mucosa to balloon out
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Pyloric Stenosis
Clinical Presentation |
Nonbilious, progressive vomiting
Dehydration Failure to thrive 1 week to 5 months of age Elevated serum bicarbonate, decrease in serum chloride |
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Pyloric Stenosis
Diagnosis |
Physical examination reveals pyloric mass or “olive”
UGI Sonography (can see enlargement) |
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Are atresias congenital or developmental?
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CONGENITAL
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Duodenal Atresia
Epidemiology |
Thought to arise from failure of recanalization of duodenal lumina
Incidence is 1 in 10,000 50% infants premature. F to M 2:1 Down’s syndrome occurs in up to 30% Presents with BILIOUS VOMITING and abdominal distension |
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Duplications of the GI Tract
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Rare anomalies consisting of well developed tubular, or spherical structures
Can present in adulthood Located on the mesenteric border Symptoms related to OBSTRUCTION, intussusception, volvulus, perforation, or hemorrhage |
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Malrotation
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Failure of midgut to achieve normal position (does not cause any symptoms)
Predisposes to VOLVULUS of midgut ... can occlude SMA and cause midgut necrosis... unable to absorb nutrients Acute bowel obstruction Bilious vomiting Surgical emergency |
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How much does the midgut rotate during development?
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270 degrees counterclockwise rotation
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Meckel's Diverticulum
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Remnant of vitellointestinal duct
Antimesenteric border of ileum 2-3% of population Painless rectal bleeding in children < 2 (excess mucosa that ulcerates and bleeds) Crampy abdominal pain |
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How do we diagnose Meckel's Diverticulum?
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tracer goes to gastric mucosa
lights up stomach and meckel's diverticulum |
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Hirschsprung's Disease
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absence of intramural ganglion cells
1 in 5,000 live births M>F Associated with Down's Syndrome 75% rectosigmoid colon Aganglionic segment permanently contracted |
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What is the main symptom of Hirschsprung's Disease?
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constipation because you lack the ganglions to relax
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Symptoms of Hirschsprung's Disease
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Delayed passage of meconium
83% have problems within first month 96% have problems within the first year Failure to thrive Vomiting (bilious), intermittent diarrhea Abdominal distension |
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Hirschsprung's Disease
Diagnostic Procedures |
Unprepared Barium Enema (BE)
Mucosal Suction biopsy Anorectal Manometry |
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Unprepared Barium Enema
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unprepared means that you do not clear out the poop before the study
because clearing out the poop can cause dilation and stop you from seeing the constriction of Hirschsprung's Disease |
