DEFINITION

congenital anomalies are also birth defect ,congenital diso

rder or congenital malformation .it can be define as struc
tural or functional anomalies ,including metabolic disord
ers,(hypothyrodism,Dm,phenyketnuria) which are prese
nt at the birth.
COMMON CONGRNITAL GASTROINT
ESTINAL ANOMALIES
 The following GI malformation arise when this process is disrupted.
 Pyloric stenosis .
 Omphalocele VS Gastrochisis
 Intussusceptions’ disease
 Duodenum Artesia
 Hirschsprung’s disease
 Intestinal obstruction
 Hiatus hernia
 Imperforate hernia
 Esophagus Artesia
PYLORIC STENOSIS
 Pyloric stenosis
INTRODUCTION
 Pyloric stenosis is a narrowing of the opening fro
m the stomach to the first part of the small intestine
 ). It is most common intra-abdominal condition du
ring the neonatal period which requires surgery. It s
eems to occur more frequently in first –born males a
nd Caucasian infants .Symptoms include projectile 
vomiting without the presence of bile.
 DEFINITION
It is defined as narrowing and obs
truction of the lower portion of the sto
mach (pylorus) that prevent food fro
m the stomach to the intestine .it is ca
used due to hypertrophy and hyperpl
asia of circular muscle of the stomach.
 ETIOLOGY

 1.Pyloric stenosis seems to be multifactorial:-multifactoial inheritan

ce means that many factors are involved in causing a brith defect, th
e factors are usually genetic and some environmental components.
 2. It is four times more likely to occur in males,[8] and is also
more common in the first born.
 3. pyloric stenosis can occur as an autosomal dominant cond

ition.
 4.It is uncertain whether it Is a congenital anatomic narrow

ing or a functional hypertrophy of the pyloric sphincter musc

le.
pathophysiology
 Clinical feature
 Babies with this condition usually present any time in the
first weeks to months of life with progressively worsening 
vomiting. The vomiting is often described as non-bile stai
ned ("non bilious") and "projectile vomiting.
 2. the initial presentation is regurgitation and non-bilious
vomiting which may occur both during and after feeding .
 3 .wt loss
 4.dehydartion
 5.Constipation
 6. fewer bowel movement
• 7.  epigastric fullness with visible peristalsi
s in the upper abdomen from the person's l
eft to right..
• 8.the infant always seems to be hungry .
• 9.starvation
• 10.loos of skin subcutaneous tissues, dryne
ss of oral mucosa membrane ,
• 11.loss of electricity of skin ,lethargy
DIAGNOSTIC EVALUATION
 medical history .
 physical examination
 Blood test
 Abdominal x-ray
 Abdominal ultrasound
 Barium swallow/upper GI series
MEDICAL MANAGEMENT

 Medications used to treat pyloric stenosis sympt

omatically are:
 IV atropine. The intravenous dose of atropine f

or treatment of pyloric stenosis ranges in studies

from 0.04 to 0.225mg/kg/day and is given for 1
– 10 days.
Oral atropine. Oral atropine (0.08 – 0.45mg/kg/day) is
continued, after IV therapy has been deemed successful,
for 3 weeks to 4 months.
 Pyloric stenosis treat in two stages .initially fluid are give

n intravenous to treat dehydration and electrolyte imbala

nce and baby’s restore normal chemistry .

To give initial 20ml/kg bolus isotonic solution .
SURGICAL MANAGEMENT
 pyloromytomy or fredet –ramstedt surgery is performed
.
 A pyloromyotomy is a surgical procedure in which an i
ncision is made in the longitudinal and circular muscles
of the pylorus. It is used to treat hypertrophic 
pyloric stenosis. Hypertrophied muscle is cut along the w
hole length until the mucosa bulges out. If the mucosa is
injured, it is sutured horizontally using interrupted vicryl
 or silk sutures.[.
 This surgery can be done through a single incisio
n (usually 3–4 cm long) or laparoscopically(thro
ugh several tiny incisions).
 
NURSING MANAGEMENT

Preoperative nursing management :- 

 Maintain adequate nutrition and fluid intake.

 TO check vital sign

 Promote skin integrity.

 Promote family coping.

 Collecting specimens

 Withholding oral feeding

 Administering i.v. fluid

 To take operation consent .

Post operative nursing management :-
 Observe complication.
 Management pain
 Provision adequate fluid and nutrition
 Parental preparation and follow up-after s

urgery
ABDOMINAL WALL DEFECTS
OMPHALOCELE VS GASTROSCHISIS

Rusila Tikoitoga
MBBS 4
2016
Definition:-
 GASTROSCHISIS:- it is a congenital anomaly characterize

d by a defect in the anterior abdominal wall through which t

he abdominal content freely protrude .it defect usually less t
han 4 cm.
 OMPHALOCEL :- it is a congenital birth defect that involv

es the umbilical cord itself ,and the organs remain enclosed

in the visceral peritoneum .with omphalocel the defect is usu
ally much larger than in gastroschisis.
  
ETIOLOGY

 GASTROSCHISIS:-
 Failure of migration and fusion of the lateral folds of the embryoni
c disc on the 3rd -4 th week of gestation.
 Disruption of the right omphalomesenteric artery as midgut retur
ns to abdomen by the 10th week causing ischemia of the abdomina
l wall and weakness then herniation.
 Rupture of omphalocele.
 OMPHALOCELE :-.
 Malrotataion of the bowels while returning to the abdomen during
development .
 Genetic disorder: such as.-Edward’s syndrome.
 Risk Factors
Omphalocele Gastroschisis
Increased maternal age Young maternal age
Twins Low gravida
High gravida Prematurity
Consecutive children Low birth-weight
secondary to IUGR
OMPHALOCELE GASTROSCHISIS
 Clinical Features
OMPHALOCELE GASTROSCHISIS
• •
central defect of the abdominal wall beneath the Defect to the right of an intact umbilical cord allowing
umbilical ring. extrusion of abdominal content

•Defect may be 2-12 cm (Small-<5cm)(Large>8cm) • •Umbilical cord arises from normal place in abdominal wall •
Always covered by sac Opening <=5 cm

• Sac is made of amnion, Wharton’s jelly and •No covering sac (never has a sac )
peritoneum
•Evisceration usually only contains intestinal loops
• The umbilical cord inserts directly into the sac in an apical
•Bowels often thickened, matted and edematous
or lateral position.
• Small contains intestinal loops only. Large may • 10-15% have associated anomalies

involve liver, spleen and bladder, testes/ovary • 40% are premature/SGA

• >50% have associated anomalies
 Diagnosis
• Alpha-feto-protein-synthesized in fetal liver and
excreted by fetal kidneys and crosses placenta by 12
weeks.
• Elevated maternal AFP - neural tube defects,
abdominal wall defects, duodenal or esophageal
atresia
•40% false positive rate
• Fetal ultrasound after 14 weeks gestation is the
confirmatory test.
 Prenatal Ultrasound
Gastroschisis
• Normal umbilical cord
insertion site
• Small bowel loops seen in the
amniotic cavity
• No covering membrane over
the loops of bowel
• Can include stomach and large
bowel
• Majority occur to the right of
the umbilical cord
 Prenatal Ultrasound
Omphalocele

• Umbilical cord
insertion is typically
midline on the mass
•Located centrally
•Contents are intestinal
loops and maybe liver,
spleen and gonads.
 MANEGEMENT
•ABC
•Heat Management
– Sterile wrap or sterile bowel bag
– Radiant warmer
• Fluid Management
– IV bolus 20 ml/kg LR/NS
– D10¼NS 2-3 maintenance rate
• Nutrition
– TPN (central venous line )
• Abdominal Distention
– OG/NG tube
– urinary catheter
• Infection Control
Broad-spectrum antibiotics - Ampicillin and Gentamycin
• Closure of the Defect
 Omphalocele
• Conservative
1. Large omphalocele (10-12cm) o Primary Closure
apply topical application - Small defects (<4cm)
Betadine ointment or silver
sulfadiazine to the intact sac.  excision of the sac and closure
2. Secondary eschar formation and
granulation. of the fascia and skin over the
3. Healing lasts for 12 months then abdominal contents
repaired as ventral hernia.
o Mesh patch
 Medium defects (6-8cm)
• Post operative care
o NICU
o Ventilation
o Feeding:
– Minimal volume
o 48 hrs Antibiotics o
Hernia dealt with at 1
yr old
 Gastroschisis
• Primary closure
o If bowel easily
reduced
• Staged closure
o Silo
fashioning:
Sac excised
Silo sewn to rectus fascia/full thickness
• Post operative care
o NICU
o Feeding delayed for
weeks
o Oral stimulation/sucking
reflex
o Broad spectrum
antibiotics
Intussusception
 INTRODUCTION:-
 It is the most common abdominal emergency in early childh
ood, particularly in children younger than two years of age.
 The majority of cases in children are idiopathic. Epidemiol
ogy
 Most common cause of intestinal obstruction in infants bet
ween 6 and 36 months of age.
 Approximately 60% < 1 year old 80 to 90%.
 DEFFINITION:- Intussusception – telescoping of a proxi

mal segment of the intestine(intussusceptum) into a distal s

egment (intussuscipien).
 CLASSIFICATION

 ILEOCOLIC most common in children and where ileum

invaginated into the caecum and then into the acending colo
n.
 ILEOILEAL:- second most common and one portion of t

he ileum invaginated into the other portion of ileum.

 ENTEROENTERIC ILEO-ILEAL,

JEJUNO-JEJUNAL more common in adults

 CAECOCOLIC:-the caecum invaginates into the colon .
 AETIOLOGY

  Approximately 75% of cases are idiopathic because there is no cl

ear disease trigger or pathological lead point.
  Viral infections can stimulate lymphatic tissue in the intestinal t
ract, resulting in hypertrophy of Peyer patches in the lymphoid ric
h terminal ileum, which may act as a lead point for ileocolic intuss
usceptions
  Postoperative- The intussusceptions is thought to be caused by
uncoordinated peristaltic activity and/or traction from sutures or
devices such as a gastrojejunal feeding tube. .
CLINICAL FEATURES
 Patients with intussusception typically develop the su
dden onset of intermittent, severe, cramp, progressive
abdominal pain, accompanied by inconsolable crying
and drawing up of the legs toward the abdomen.
  Between symptoms child will be playing and doing
normal activity.
  Vomiting Later
  Continuous abdominal pain
 The stool may contains gross or occult blood or be a
mixture of blood and mucous and sloughing mucosa,
giving it the appearance of currant jelly.  Lethargy
  Palpable abdominal mass. Physical
  A sausage shaped abdominal mass
 .  Abdominal distension
  Dehydration
  Classic triad ( < 1%. If left untreated, this conditio
n is uniformly fatal in 2-5 days. .
 DIAGNOSIS:-

 Ultrasonography — Ultrasonography is the method of choice to detect in

tussusception. A Doughnut or ‘target sign’ is seen, representing layers of
the intestine within the intestine Dx accuracy is approx 85%. May also b
e visible on
 Abdominal CT with IV contrast.- Abdominal plain film – low sensitivity

and specificity  Signs of intestinal obstruction  Pneumoperitoneum

 Contrast x-ray Patients with typical presentation can proceed directly to

contrast study (enema) advantage of being diagnostic (barium will outlin

e a concave ‘meniscus’) and therapeutic.
 
 TREATMENT

  Stabilize and resuscitate with intravenous fluids  Hydration, electrolyte, aci

d-base balance.
  NBM and Stomach should be decompressed with a nasogastric tube
  Antibiotics- if signs of infection (fever, peritonitis)
  Ampicillin 50mg/kg IM/IV 4x per day
  Gentamycin 5mg/kg IM/IV OD
  Metronidazole 7.5mg/kg IV TDS
 Duration – uncomplicated reduced with air enema 24-48 hrs
 - perforated bowel with resection 1 week
 post-op
 
NON-OPERATIVE TREATMENT

  Stable patients and no evidence of bowel perfora

tion should be treated with Non-operative reducti
on
  Non-operative reduction using hydrostatic or p

neumatic pressure by enema

SURGICAL MANEGEMENT

  Indicated for patients with peritonitis or evidence of perfo

ration or in whom non-operative reduction is unsuccessful.
  Manual reduction at operation is attempted

  If manual reduction fails or in case of perforation,

 necrosis, pathological lead point – bowel resection and prim

ary anastomosis is performed.  The risk of recurrence is ap

proximately 1 percent after manual reduction and virtually
nonexistent after surgical resection.
 NURSING MANAGEMENT

 Preoperative nursing management :-

 To check the vital sign.
 Keep the patient is semi up right position.
 Intravenous iv fluid administered.
 Maintain intake and output.
 To take operation consent.
 To collect all report.
 To administered oxygen if necessary.
 NBM
Post operative nursing management

 To check the vital sign

 To administered oxygen .
 Ventilator support may be provided
 Change the infant position frequently
 feeding is started as soon as ordered.
 Administered prescribed medication.
 To maintain intake and output .
 Gastrointestinal suctioning is done to keep the stomach empty .
 Dressing is incision site with strict aseptic pracution.
 COMPLICATIONS
 Rarely occur when diagnosis is prompt.
  Necrosis and bowel perforation from strangulated intussus
ceptions
  Peritonitis and Sepsis
  Hypovolaemia and circulatory shock
  Electrolyte imbalance  Perforation during non operative re
duction.
  Wound infection.
  Adhesions causing bowel obstruction.
INTRODUCTION
DEFFINITION: Duodenal atresia is the congenital absence
or complete closure of a portion of the lumen of the
duodenum due to the defective fusion of foregut and
midgut with failure of the recanalisation.

EPIDEMIOLOGY:
1 Per 5000 to 10000 live births.
Affecting boys more commonly than girls.
ETIOLOGY
 Intrinsic lesion: Caused by failure of recanalization of
the fetal duodenum
 Extrinsic lesion: Defects in the development of the
neighbouring structures
Teratogens:
Chlordiazepoxide
Thalidomide
Flurouracil
ASSOCIATIONS
Prematurity (45%)
Growth retardation (33%)
Annular pancreas
Down’s syndrome (30%) – Trisomy 21
Miller-Dieker syndrome
More than 50% patients have associated
congenital anomalies –
Congenital heart disease (30%)
Incomplete rotation of gut (20%)
Anorectal malformations (10%)
 CLASSIFICATION
ANATOMICAL CLASSIFICATION:
1TYPE 1 (92%):Complete atresia

1TYPE 2 (1%):Fibrous cord separating two ends

1TYPE 3 (7%):Incomplete or partial obstruction

Stenosis
 Mucosal web with intact muscular wall-
WINDSOCK DEFFORMITY
Anatomical Types
CLASSIFICATION CONTD…
SITE WISE
CLASSIFICATION:

PREAMPULLARY
(10-15% )

POSTAMPULLARY
(85-90% )
PATHOPHYSIOLOGY
Failure of canalization of the duodenal lumen

Improper formation of vacuoles

Atresia or web may develop

Accumulation of gases and secretions above the

blockage
In intra luminal pressure , venous pressure
Circulatory stasis or edema

Gangrene, Peritonitis
CLINICAL FEATURES
 Bilious or nonbilious vomiting immediately after
birth
Jaundice
Features of gastric outlet obstruction
Dehydration and electrolytes changes
Abdominal distension and tenderness
Absence of flatus
No passage of stool
Respiratory distress
Shock
 DIAGNOSIS
History: Maternal polyhydramnios
Investigations:
1. MATERNAL -
 Prenatal
1ultrasonography
Detects duodenal: atresia between 7th and
8th month of gestation.
1 Polyhydramnios and associated anomalies can also
be detected.
2. CHILD
Plain X ray abdomen: DOUBLE BUBBLE SIGN
with absence of air in the distal part
 INVESTIGATION CONTD…
Upper GI contrast study:
a. Partial obstruction
with the presence of
air in the distal loop

b. Other associated
anomalies –
Malrotation,Mid gut
volvulus etc
INVESTIGATION CONTD…
 Ultrasonography abdomen:
Distended stomach and proximal
Duodenum – RAIL ROAD TRACK
DUODENUM
MANAGEMENT
GENERAL MANAGEMENT :
IV fluids
Oro or nasogastric aspiration
Stomach wash
NPM
Antibiotics,antiemetics,vitamin K
supplement,PPI
SURGICAL MANAGEMENT :
PREOPERATIVE CARE :

Appropriate resuscitation
 Correction of fluid imbalance and electrolyte
abnormalities
Monitoring of the complete metabolic profile
 Gastric decompression
Perenteral nutrition via central catheter line
Two dimensional echocardiographic monitoring
TREATMENT
PROPER:
Surgery can be done
by:
LAPAROTOMY: Supraumbilical
transverse incision is given to the right
upper quadrant of abdomen

LAPAROSCOPY: Neonatal laparoscopic

instruments (3mm ) and Trocars are
used
TREATMENT CONTD…
 Different Methods are :
DUODENODUODENOSTOMY: Surgical bypass for
the duodenal obstruction is done by Side to side
Duodenoduodenostomy.Associated malrotation
should also be corrected (LADD’S
Megaduodenum OPERATION).
or anstomotic
can occur.dysfunction
Kimura’s diamond shaped anstomosis:
Done between transversly opened proximal pouch
and longitudinally opened distal pouch.Presence
of bile and concomittent distal atresia should be
confirmed by Less chances
saline of the problems
irrigation.
anastomosis.of
Treatment contd..
Tapering Duodenoplasty: Done with staples or
sutures to reduce the Duodenal caliber when the
proximal Duodenum is markedly dialated.
HEINEKE-MICULICZ DUODENOPLASTY: Partial web
resection is done transduodenally in Duodenal
mucosal web.
DUODENOJEJUNOSTOMY: Previously preferred
technique.
May cause blind loop problem.

GASTROJEJUNOSTOMY: Done previously.

High incidence of marginal ulceration and bleeding.
POST OPERATIVE CARE
Continuation of Total parenteral nutrition (TPN)
Monitoring of Nasogastric tube output
Feeding via transanastomotic nasojejunal or
gastrostomy tube after diminution of nasogastric
tube output
Small feeding with volume and concentration
advanced as tolerated
 Discharge within one to three weeks
COMPLICATIONS
 INTRAOPERATIVE :
 Windsock defformity (Floppy web) : Incorrect identification
of site of obstruction
Bypass anstomosis entirely distal to true web attachment
 Multiple obstructions
 POST OPERATIVE :
Megaduodenum (30%)
Prolonged feeding intolerance
Poor peristalsis
Residual obstruction
Anastomotic stenosis
HIRSCHSPRUNG’S DISEASE
 DEFINITION

Also known as
CONGENITAL A GANGLIONIC MEGACOLON

Present since birth

missing cluster of nerves
enlarged colon

It is a congenital anomaly in which inadequate

motility causes obstruction of the intestine.
INCIDENCE

• The disease occur approx. 1 in 5000 live

births
ETIOLOGY

• Congenital absence of aganglion cells in the

wall of variable segment of rectum and colon

• RET –proto- oncogene and EDTRB genes

are a major gene responsible for the disease.
PATHOPHISIOLOGY:-

 Due to absence of ganglionic cells

  

There is lack of peristalsis in the affected portion

 

Function obstruction of colon

 

Accumulation of the gas and feces proximal to the defect

 
 Enlargement of the colon occurs ,so it is also know s megacolon
CLINICAL MANIFESTATIONS
•Newborns
•Failure to pass meconium within first 48 hours
• Abdominal distension
•Bilious vomiting
•Failure to thrive
•Fever and bloody diarrhoea
•enterocolitis ( inflammation of intestines)
•Older children

•Failure to gain weight

•Malnutrition
•Chronic progressive
constipation
•Recurrent fecal impaction
•History of pencil thin stools
•Abdominal distension
•Child may pass ribbon like / fluid/pellet form
stools
 DIAGNOSIS
•History
•Physical examination
•Bowel pattern
• Anorectal manometry- reaction of anal spinchter to
distension of rectum.
•Rectal biopsy – presence/absence of ganglion cells
• Contrast enema – narrow distal segment, funnel
shaped dilation, marked dilation in proximal colon
• Rectal biopsy :definitive diagnostic test
MANAGEMENT

• Surgical correction after 9-12 months or till

child gains weight
•Definitive procedures
•Swenson procedure
•Duhamel procedure
•Soave procedure
•Laparoscopy
•Transanal endorectal pull through
SWENSON PROCEDURE

•Extra rectal dissection down to 2 cm above the canal

•aganglionic colonic segment is resected

•End to end anastomosis of normal proximal colon to anal canal

• Completely removes aganglionic colon
DUHAMEL PROCEDURE

Posterior portion of defective colon segment is

resected

Side to side anastomosis to left over portion of

rectum

Constipation is a complication due to

remaining aganglionic tissue

This is a simple surgery with less dissection

 SOAVE PROCEDURE

• The circumferential cut through muscular coat

of colon at peritoneal reflection
• Mucosa is separated from muscular coat down
to anal canal
• Proximal normal colon is pulled through
retained muscular sleeve
• Telescoping anastomosis of normal colon to
anal canal is done.
 LAPAROSCOPY

•Transanal endorectal pull through

• Excised aganglionic tissues are removed through

anal canal
•There is no abdominal incision
• Has better results in terms of pain, return of bowel
movements and shortens hospital stay.
NURSING MMANGEMENT
 Preoperative nursing management :-
 To check the vital sign.
 Keep the patient is semi up right position.
 Intravenous iv fluid administered.
 Maintain intake and output.
 To take operation consent.
 To collect all report.
 To administered oxygen if necessary.
 NBM
Post operative nursing management
 To check the vital sign
 To administered oxygen .
 Ventilator support may be provided
 Change the infant position frequently
 feeding is started as soon as ordered.
 Administered prescribed medication.
 To maintain intake and output .

 
 
NURSING DIAGNOSIS:-

 Abdominal pain related to abdominal distention

 Hyperthermia related to disease condition.
 Fluid volume deficit related to inadequate intake of food .
 Dehydration related inadequate intake of food
 Risk of aspiration related to disease condition .
 Risk of complication related to disease condition.
 Anxiety of parents related to disease condition.
 Deficit knowledge of parents regarding disease and its management
.
THANK YOU