3. How many (it’s relative…)
Resuscitation (it works every time!)
Simple vs Strangulating (countdown to a Deadline)
Development of the Midgut (What goes wrong)
Specific diagnosis
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4. In the 2006 population census we had 2.5 millions
babies born
Luckily most will be born healthy
Unfortunately a small percentage will be born with lesser
chances at surviving.
All they need is some TIMELY help to put them back on
the road to life.
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5. Birth defects are quite uncommon
Conditions requiring surgery at birth are rare
But rare loses its meaning here…
Something that happens once every 12000
babies occurs >200 times a year
Cases of conjoined twins happen once every
half a million births
We get at least 2 or 3 every year
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6. Normal
need help
•Congenital anomalies
requiring surgery are
small percentage of live
births.
•It’s a very small slice,
less than 0.5%.
•But it’s one huge pie!
•This small slice
represents around
100000 babies
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11. Any baby presenting with persistent, bile
stained vomiting should be considered to be
surgical until proven otherwise.
Clinical picture:
The cardinal signs are 2C, 2V, 2D
Colics, Constipation
Vomiting, Visible peristalsis
Distention, Dehydration
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12. Gastric decompression (naso-gastric
tube)
IV line (for fluid replacement)
Urinary catheter (to monitor urine output)
Fluids:
◦ 10-20ml/kg over an hour, to be repeated according to
response
Drugs:
◦ To cover anaerobes as well as Gram stained bacteria
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Resuscitation
13. After initial resuscitation, every effort is
directed towards rapid diagnosis and
treatment of the specific cause
Imaging:
◦ Plain X ray
◦ Contrast studies etc
12345
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14. Any neonate presenting with projectile, non bilious
vomiting, associated with hunger and constipation
should be considered CHPS
Incidence:
◦ 8:1000
◦ M/F ratio=4:1
◦ More in first born babies
◦ More in infants born to a mother who had suffered from
CHPS
◦ More during spring and Autumn!
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15. Etiology:
◦ Unknown, but appears to be polygenic
(environmental, and genetic)
Pathology:
◦ Progressive hypertrophy of circular pyloric
muscles
◦ Persistent vomiting leads to development of
hypochloremic hyponatremic alkalosis and
dehydration
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16. Clinical picture:
◦ Symptoms:
projectile, progressive, non bilious vomiting after
which the baby is hungry and ready to suckle again.
Classically the symptoms start 2-3 weeks after birth
◦ Signs:
Signs of dehydration which may be severe an life
threatening.
Visible peristalsis in the upper abdomen can usually
be seen after the baby is given a test feed followed by
projectile vomiting.
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19. Differential diagnosis:
◦ Gastroenteritis
◦ Gastro-esophageal reflux
◦ Other obstructive lesion of the gut
◦ Increased Intracranial tension
Investigations:
◦ Imaging: sonography and contrast X ray
◦ Laboratory: to detect and correct electrolyte
and metabolic disturbances
Treatment:
◦ First aid treatment to correct dehydration and
metabolic disturbances
◦ Definitive surgery, the Ramstedt’s
pyloromyotomy
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21. Atresia
◦ Types:
Type I: mucosal diaphragmatic (web) membrane
Type II: short fibrous cord connects two atretic ends
Type III: complete separation of two atretic ends
◦ Commomly associated with Down’s syndrome 30%
◦ Vomiting is bilious in around 85% of cases
◦ Shows characteristic double bubble sign in X ray
◦ Treatment is by duodeno-duodenostomy with high success rate
Annular pancreas
◦ Incidence is around 1:7000
◦ Failure of normal fusion of the two
◦ pancreatic buds which may obstruct
◦ the duodenum from without
It might be Malrotation!
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22. Stage II (Rotation):
◦ Non-rotation: leaving the major part of the colon on
the left side and the small intestines to the right of
the midline
◦ Incomplete rotation: the coecum is situated in the
sub-hepatic region
◦ Reversed rotation: the final 180o
rotation occurs in a
clockwise manner so that the colon is lying posterior
to the duodenum and the superior mesenteric artery
◦ Hyper-rotation: the rotation continues to 360o
or 450o
so that the coecum rests in the region of the splenic
flexure.
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26. Stage I (Return):
◦ Exomphalos major (umbilical defect
>5cm)
◦ Exomphalos minor (umbilical defect<5cm)
◦ Gastroschisis, refers to extrusion of
intestines through a defect to the right of
a normally formed umbilicus
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28. Stage III:
◦ Non fixation may predispose to volvulus of the
gut or caecum
◦ Interference with the blood supply of a segment
of gut during rotation may result in an atresia of
a segment or segments of intestines
◦ The Yolk sac which was attached to the midgut by
the vitello-intestinal duct normally disappear
completely. The common remnant of this system
is the Meckel’s diverticulum
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32. Obstruction of Ilieum due to thick inspissated
meconium
Occurs in 10% of patients with cystic fibrosis
Gives the characteristic “Ground glass” appearance
in plain X ray
Gastrografin enema is both diagnostic and has
potential therapeutic effect
Operative intervention may be needed in non
responsive cases
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34. It is the commonest cause of intestinal
obstruction in infancy
Epidemiology:
◦ Affects 1:1000 live births
◦ Male/Female ratio = 4:1
◦ Most cases are sporadic
◦ Long segment and total colonic aganlionosis have
strong familial association (15% &25%)
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35. Pathology:
◦ Failure of caudal migration of neuroblasts
derived form the neural crest
◦ There is absence of ganglions in the submucous
and the myenteric plexuses
◦ There is failure of propulsive peristalsis
manifesting by obstruction
◦ Grossly there is a spastic distal segment with a
funnel connecting it to a dialated segment
◦ In about 75% of cases the affected region is the
recto sigmoid junction
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36. Clinical picture:
◦ History of delayed passage of meconium and
persistent constipation
◦ On examination there is abdominal distention
◦ Rectal examination reveals a tight spastic
rectum
Investigations:
◦ Imaging: Ba enema (Without preparation!!)
◦ Anal manometry
◦ Rectal biopsy is the gold standard
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37. Treatment:
◦ Temporary measures include laxatives and
enemas.
◦ Colostomy may be a life saving procedure in
some cases
◦ Definitive treatment is surgical excision of the
spastic segment an re-anastomosis to the anal
canal. Various procedures can be done e.g
Swenson’s, Duhamel’s and Soave’s procedures
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40. Definition:
The main pathology is atresia of the esophagus! Tracheoesophageal fistula is secondary
to that.
Incidence:
•1-2500 to 1-10000
•males slightly more affected than females
•the second sibling of an affected child has 0.2-0.5% chance of being also affected
Associations:
Other congenital anomalies occur in 50-70% of infants affected with esophageal atresia.
These are most common with esophageal atresia and distal fistula. Anomalies include
cardiovascular (the majority), genitourinary, gastrointestinal and skeletal.
VACTERL association consists of Vertebral, Anorectal, Cradiac, Tracheo-Esophageal,
and radial Limb deformities. It is not correlated with a known genetic abnormality or
syndrome.
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43. DDiagnosis
Antenatal:
Maternal ultrasound
Postnatal:
Tracheoesophageal fistula present within the first few hours of life by:
- excessive salivation
- respiratory distress
- cyanosis
- inability to pass a nasogastric tube.
- Attempts at feeding cause choking, coughing, cyanosis, regurgitation.
Radiography:
•Plain X-ray
•Contrast study(pouchogram)
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45. The treatment is ultimately surgical but management strategy
include also preoperative, operative and postoperative
measures
Surgical treatment:
Division and closure of the fisula and primary anastomosis of
the two esophageal segments.
If the “Gap” between the two segments is too long, the infant
is managed temporarily with an esophagostomy and
gastrostomy. Later in life an procedure to replace the
esophagus is considered e.g. colon interposition bypass.
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46. Definition:
Strictly the term should be agenesis or atresia but the term is
widely used.
Incidence:
Affects 1:4500 live births
Classification:
Can be broadly classified into
High anomaliesLow anomalies:
-Anorectal agenesis-Covered anus
-Rectal atresia-Ectopic anus
-Cloaca-Stenosed anus
-Membranous stenosis
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49. Management
As there are frequent association with other anomalies these should be also
looked for, diagnosed or excluded
•Clinical examination:
Usually the clinical diagnosis is made a birth either by inspection or by
failing to pass a thermometer easily into the anus
•Radiography:
Invertogram, done after six hours of birth
Contrast studies (distal loopogram): in a clolostomized child to
diagnose the site of any fistulous connection to the genito-urinary
system
•Treatment:
-Colostomy: early in the neonatal period for high anomalies
-Local procedure: for low anomalies
-Full correction of high anomalies is done later in life starting from the
6th month after which the colostomy maybe closed
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