GI Disorders

OMPHALOCELE
Are congenital malformations in which intra abdominal
contents herniate through the umbilical cord.
Cause
failure of the abdominal contents to return to the abdomen
when the abdominal wall begins to close by the 10th week of
gestation.
The protrusion is covered by a translucent sac into which
the umbilical cord inserts.
Rupture of the sac results in evisceration of the abdominal
contents.

Omphalocele
associated anomalies
cardiac defects,
genito urinary anomalies,
chromosomal defects, craniofacial
abnormalities &
diaphragmatic abnormalities.

Incidence: 1 in 3000- 10,000 births.

Treatment:Surgery is done to replace the

abdominal contents & close the
abdominal wall.

Pre op:
Immediately after birth, the sac is covered with sterile
gauze soaked in normal saline to prevent drying of
abdominal contents,
a layer of plastic wrap is placed over the gauze to
provide additional protection against moisture loss.
Monitor V/S every 2 to 4 hours, particularly
temperature because the infant can lose heat through
the sac.
Maintain NPO status.
Administer IV fluids as prescribed to maintain
hydration & electrolyte balance.
Monitor for signs of infection. Daily dressing changes.
Handle the infant carefully to prevent rupture of the
sac.

Post Op

Control pain
Prevent infection
Maintain fluid & electrolyte balance
Ensure adequate nutrition

Gastrochisis
Both gastrochisis and omphalocele allow
herniation of intraabdominal contents
through the abdominal wall
Herniation of intestines lateral to umbilical ring
usually right of umbilicus
Peritoneal sac is not present

Diagnosis

Increased MSAFP levels

normal- 40 micro gm/L
Prenatal ultrasound
Amniocentesis

Operation
Preferred within first 24 hrs
Complication fluid loss, infection,
hypothermia
First stage is an operative placement bowel
in silo
Intermittent tie down of silo is done
Pushes bowel in abdominal cavity
When bowel reaches skin level, defect is
closed

Preop care- abdominal wall defect

Prevent infection
Sterile barriers, antibiotics, bath silo with warm
antibiotic solution, monitor for redness , drainage

Prevent hypothermia
Cover with bowel bag, warm all solutions,
maintain temp above 36.5 C, us warm humidified
oxygen

Protect bowel
Prevent kinking of bowel, gastric decompression
tube

Pre op
Hydration
Administer iv fluids, weigh infant, I/O, lab tests
(CBC,ABG, electrolytes), BP, urine output

Respiratory support
SaO2>95%, avoid bag and mask ventilation
(distends bowel), auscultate breath sounds
bilaterally

Post op

Respiratory support
Infection prevention
Fluid therapy
Pin management
Skin care
GI decompression
Nutrition gastrostomy feedings, TPN

Malrotation
During embryonic life rotation of duodenojejunal
loop and rotation of cecocolic loop has to take place
simultaneously for normal intestinal development
failure to complete normal rotation and fixation of
the bowel leads to malrotation
Superior mesentric artery may get strangulated
Intestinal obstruction due to malrotation is volvulus
Complication
Midgut volvulus, Intestinal ischemia, bowel
necrosis, perforation, shock, respiratory failure
death

 When the normal 270 degree rotation is

interrupted duodenum lies behind SMA
Cecum does not reach right iliac fossa
Adhesions form, running from cecum
across the duodenum to the R lateral wall
of abdomen - Ladds Bands

Sign and symptoms

Abdomianal pain
Bilious vomiting
Melena, currant jelly stools
Abdominal distension

Diagnosis

UGI series
Lab tests
US
CT Scan

Surgery

Division of Ladds bands

Resection of necrotic bowel
ETE anastomosis
Mobilisation of intestine
Appendicectomy

Preop
Correct hypovolemia, metabolic
imbalances
Antibiotics
NG decompression,
foleys catheter
Prevention of hypothermia

Post op
IV fluids and electrolytes
NG tube to low intermittent suction
Pain - morphine, acetaminophen,
distraction
Nutrition- TPN, gastrostomy feedings

ARM
ARM encompasses multiple congenital
anomalies of the rectum, urinary and
reproductive structures with varying
degree of complexity
Most children with ARM have abnormal
communication with rectum, GU tract and
perineum called fistulas.

Signs and symptoms

Imperforate anus
Abdominal distension, vomiting
Meconium in perineum or urine

Types of male defects

1. Perineal fistula
2. Rectobulbar urethral
fistula
3. Rectoprostatic urethral
fistula
4. Rectobladder neck
fistula

1. No colostomy, minimal
PSARP
2. Colostomy, PSARP
3. Colostomy, PSARP

5. Imperforate anus
without fistula
6. Rectal atresia and
stenosis

5. Colostomy, PSARP

4. Colostomy, PSARP+
laparotomy

6. Colostomy, PSARP

Types of female defects

1. Perineal fistula
2. Vestibular fistula
3. Imperforate anus
without fistula
4. Rectal atreaia
5. Persistent cloaca

1. No colostomy,
minimal PSARP
2. Colostomy, PSARP
3. Colostomy, PSARP
4. Colostomy, PSARP
5. Colostomy,
PSARVUP

Diagnosis
Abdominal US - to detect urinary
obstruction
Invertogram to assess height of defect

Perineal fistula
When rectum opens into perineum
Low malformation
No colostomy, minimal PSARP during
newborn period

Rectourethral bulbar fistula

Rectum communicates with the lower
posterior portion of the urethra called
bulbar urethra
Colostomy
PSARP with first yr of life

Rectourethral prostatic fistula

Rectum communicates with the upper part
of the posterior portion of the urethra
passing through prostatic tissue

Rectobladder neck fistula

Highest defect in male
Rectum communicates with bladder neck
Abnormal sacrum

Imperforate anus without fistula

Rectum is completely blind

Ends 2cm from perineum
No meconium in urine
Associated with Down syndrome

Female defects
Perineal fistula- rectum opens through an
abnormal orifice located in the perineum
between the genitalia and anal dimple
Vestibular fistula- rectum opens through
an abnormally narrow orifice located in the
vestibuleof the genitalia, outsid the hymen

 Vaginal fistula- meconium comes out

through the vagina from inside the hymen
orifice

Persistent cloaca
rectum, vagina and urinary tract are fused
together into a single common channel
that communicates exteriorly through a
single perineal orifice located at normal
urethral site
Diverting colostomy, urinary diversion
(vesicotomy, ureterostomy)
3 months - PSARVUP

Cloacal extrophy
Rare malformation
Includes omphalocele, 2 extrophied hemi
bladders with cecum between them,
imperforate anus and abormal sexual
structures

Anoplasty
For low defects or perineal fistulas
NPO for 4 hrs
Fistula is moved to anatomically correct position,
at the center of anal sphincter
Larger anal opening is created
Nothing inserted in rectum
Antibiotic ointment perineum TDS for 2 weeks
IV antibiotics- 2-3 days
Breastfeeding
Anal dilatations at 2 weeks

Colostomy

Descending colostomy
Functional stoma,
Non functional stoma
Site care

PSARP
Generally done after 1 month, at approx. 6-12
months of age
Sx done in prone position with foleys in place
Midline posterior sagittal incision is made
running from middle portion of the sacrum to the
anterior edge of anal sphincter
Rectum is mobilised, fistula is closed
Rectum is placed in its optimal location
Laparotomy is done for very high defect

 Anal dilatations after 2 weeks of surgery,

for 6-8 weeks
Colostomy closure