Download MBBS Pediatric Surgery Presentations 1 Abdominal Wall Defects Lecture Notes

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OMPHALOCELE AND GASTROSCHISIS

ABDOMINAL WALL DEFECTS

? A type of congenital defect that allows the abdominal organs

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to protrude through an unusual opening (blue arrows)that

forms on the abdomen.

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CONTENTS

? Embryology
? Types

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? Gastroschisis
? Omphalocele
? Management
? Outcome
? Differences

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EMBRYOLOGY

? Closure of the body wall begins at 3 weeks' gestation and results

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from growth and longitudinal infolding of the embryonic disks.


? The cephalic fold forms the thoracic

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and epigastric wall.

? The lateral folds form the lateral

abdominal walls.

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? The caudal fold contributes the

hindgut, bladder, and hypogastric

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wall.

? These four folds meet in the midline to

form the umbilical ring.

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? During 6th week of gestation, rapid growth

of intestines causes herniation of the

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midgut into the umbilical cord.

? Week 10, the midgut is returned to the

abdominal cavity and the small bowel and

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colon assumes a fixed position.

? Any disruption in process may result in an

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abdominal wall defect.

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TYPES

1. Ectopia cordis thoracis ? cephalic

fold defect.

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2. Pentalogy of Cantrell- cephalic fold

defect.

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3. Omphalocele ? Failure of folding.
4. Umbilical cord Hernia ? Small

defect and normal abdominal wall.

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5. Gastroschisis ?
6. Cloacal exstrophy ? caudal fold
defect.

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GASTROSCHISIS- Most common

? Incidence : 2 to 4.9 per 10,000 live

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births.

? Herniation of intestinal loops

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through full-thickness defect in

anterior abdominal wall.

? Defect lateral to the

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umbilicus (right>left), usually

less than 4cm in size.

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? No sac covers the extruded

viscera (usu. only intestines).

? Preterm babies (28%).

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? Young mothers (<25years).

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Etiology:

? In-utero vascular accident.

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2 theories
1. Involution of the right umbilical vein causes

necrosis in the abdominal wall leading to a

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right-sided defect.
2.Right omphalomesenteric artery prematurely

involutes

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? Other theories:

?In-utero rupture of omphalocele.

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?Abnormal midline fusion of the

abdominal folds.

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ASSOCIATED ANOMALIES

? 10-20% - intestinal stenosis or

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atresia that results from

vascular insufficiency to the

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bowel.

? `Vanishing bowel'- very small

defect strangulates bowel

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development.

ANTENATAL CONSIDERATIONS

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? Diagnosis can often be made < 20 weeks

of pregnancy by ultrasound.

? Amniotic fluid and serum tests of AFP and

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amniotic fluid acetylcholinesterase

(AChE)- raised in abdominal wall defects.

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? Opportunity to counsel the family

(Increased risk :

- Intrauterine growth retardation

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(IUGR),

- Fetal death, and

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- Premature delivery).

? Prepare for optimal postnatal care.

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Mode of delivery.

? Optimal mode- debated.

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- Proponents of LSCS: Vaginal delivery

may damage bowel.

- Studies have failed to show difference

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in outcome between Caesarean and

vaginal delivery.

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- The delivery method should be at the

discretion of the obstetrician and the

mother

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Timing of delivery

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? Considerations :
1. Because bowel edema and peel formation increase

as pregnancy progresses.

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2. LBW and preterm negatively influences outcome,

with neonates weighing <2 kg having

- increased time to full enteral feeding,

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- ventilated days, and

- duration of parenteral nutrition.

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? The presumption is that earlier delivery based on serial

measurements of the bowel may decrease the incidence

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of intestinal complications.

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PERINATAL CARE

? Outcome depends on - amount of intestinal damage that

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occurs during fetal life.

? Combination of exposure to amniotic fluid and constriction

of the bowel at the abdominal wall defect.

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? Intestinal damage impaired motility and mucosal absorptive

function prolonged need for total parenteral nutrition and

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severe irreversible intestinal failure.



? Prenatal diagnosis provides a potential opportunity

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to modulate mode, location, and timing of delivery
in order to minimize these complications.

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Neonatal resuscitation and management

? Gastroschisis causes significant

evaporative water losses from

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the exposed bowel.

1. Warm saline-soaked gauze,

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placed in a central position on the

abdominal wall and wrapped with

plastic wrap.

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2. IV Fluid resuscitation.
3. Gastric decompression.
4. Baby right side down- prevent

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mesenteric pedicle kinking.

5. IV antibiotics.

SURGICAL MANAGEMENT

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? The primary goal of every surgical repair is to

return the viscera to the abdominal cavity while

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minimizing the risk of damage to the viscera.

? Options include:

(i) Primary reduction with operative closure of the

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fascia;

(ii) silo placement, serial reductions, and delayed

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fascial closure;

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Primary closure ? with fascial closure

? In neonates considered to possess sufficient intraabdominal

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domain to permit full reduction of the herniated viscera.

? Warm bowel and clean the peel; check quickly for intestinal

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anomalies.

Primary closure- without fascial closure

? Umbilicus as an

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allograft.

? Prosthetic non

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absorbable mesh.

? Prosthetic biosynthetic

absorbable options ?

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dura or porcine small

intestinal submucosa.

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Staged closure

? Bowel placed into
? Spring loaded silo

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- Silastic sheet silo

? Delivery room or OT.

? Bowel is reduced once or twice

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daily into the abdominal cavity

as the silo is shortened by

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sequential ligation.

? Once contents entirely

reduced, definitive closure.

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? Usually takes 1-14 days.

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Intra-abdominal pressure

? Either as intravesical or intragastric pressure, can be used to

guide the surgeon during reduction.

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? Pressures >20 mmHg are correlated with decreased perfusion

to the kidneys and bowel.

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? Following reduction, monitor:
- Physical examination,
- Urine output, and
- lower limb perfusion

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With a low threshold to reopen a closed abdomen for signs of

abdominal compartment syndrome

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? Gangrenous intestinal loop within the silo.

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Management of associated intestinal
atresia or perforation

? Upto 10 % cases associated.

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? Usually jejunal and ileal.

? Options
- Resection and end to end anastomosis
- Stoma

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- Initial gastroschisis repair and 4-5 weeks later, atresia

surgery.

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Postoperative Course

? Abnormal intestinal motility.
? Abnormal nutrient absorption.

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? Delayed enteral feeding.
? Prokinetics.
? Parenteral nutrition.

OMPHALOCELE- 2nd Most common

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? Incidence is 1.5 to 3 per 10,000 live births.

? Omphalocele represents a failure of the body folds to complete

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their journey.

? Herniated viscera covered by a membrane consisting of

peritoneum on the inner surface, amnion on the outer surface,

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and Wharton's jelly between the layers.

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OMPHALOCELE (EXOMPHALOS)

? The umbilical vessels insert into the

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membrane and not the body wall.

? The hernia contents include a variable

amount of intestine, often parts of the

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liver, and occasionally other organs.

?

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OMPHALOCELE (EXOMPHALOS)

? Whatever the insult may be that causes it, this aberration

occurs early in embryogenesis- more associated anomalies.

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ANTENATAL CONSIDERATIONS

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? Distinguished by presence of sac and

presence of liver.

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? Other associated anomalies-
ultrasound especially for cardiac and

chromosomal studies.

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? Increased levels of AFP and AChE

? Risks of :
- IUGR (5-35%)
- Fetal death

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- Premature labour (5-60%)

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PERINATAL CARE

? Neither caesarean nor vaginal delivery superior.

? Most practitioners choose to deliver neonates with large

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omphaloceles by cesarean section because of the fear of liver

injury or sac rupture during vaginal delivery.

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? Delivery at tertiary perinatal centre- immediate access to expert

care.

? No advantage of preterm delivery.

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NEONATAL RESUSCITATION AND MANAGEMENT

? Careful attention to cardiopulmonary status- unsuspected

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pulmonary hypoplasia- requires immediate intubation and

ventilation.

? Directed cardiac evaluation:

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- auscultation,

- four-limb blood pressures, and

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- peripheral pulse examination.

? Dressed with saline soaked gauze and an impervious dressing to

minimize fluid and temperature losses.

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? If sac ruptured, then treat as gastroschisis.

? IV fluids and nasogastric tube.

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SURGICAL MANAGEMENT

? Treatment options in infants with omphalocele

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depend on:

- The size of the defect,

- gestational age, and

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- the presence of associated anomalies.

? Options:

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1. Primary closure
2. Staged closure

PRIMARY CLOSURE

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? Only when the baby is stable and defect is small.
? Steps:
- Excising the omphalocele membrane,
- reducing the herniated viscera, and
- closing the fascia and skin.

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STAGED CLOSURE

? If the covering sac is intact, then there is no urgency to perform

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operative closure.

? `Escharotic therapy', which results in gradual epithelialization of

the omphalocele sac.

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? Usually takes many

months for the sac to

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granulate and epithelialize.

? Options:

1. Silver sulfadiazine

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2. Mercurochrome

3. Povidone iodine

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4. Gentian violet

? Mercurochrome

- scarificant and disinfectant.

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- reports of mercury poisoning.

? Povidone iodine - systemic
absorption of the iodine-

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transient hypothyroidism.

? Gentian violet ?
Antibacterial and
antifungal.

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STAGED CLOSURE

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? Sac is epithelialized or sturdy enough to withstand external

pressure

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Compression is done with elastic bandages and serially

increased until the abdominal contents are reduced.

VENTRAL HERNIA REPAIR

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VENTRAL HERNIA REPAIR

1. Flaps that mobilize the muscle, fascia, and

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skin of the abdominal wall toward the

midline and allow midline fascial

closure.

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2. Tissue expanders-to

create an abdominal

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cavity big enough to

house the viscera.

3. Prosthetic patches in

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abdominal wall.


Long-term outcomes

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GASTROSCHISIS

? Generally excellent.

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? Many patients with atresia do very

well as long

as the bowel is not irreversibly

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damaged during fetal life.

? Majority - will achieve normal

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growth and development after an

initial catch-up period in early
childhood.

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Long-term outcomes

OMPHALOCELE

? Most infants recover well with no long

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term issues, provided that there are no
significant structural or chromosomal abnormalities.

? Long term medical problems occur in patients with large

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omphaloceles:

- gastroesophageal reflux,

- pulmonary insufficiency,

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- recurrent lung infections or asthma, and
- feeding difficulty with failure to thrive,

reported in up to 60% of infants with a giant omphalocele.

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OMPHALOCELE

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GASTROSCHISIS

INCIDENCE

1.5-3: 10,000

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2 -4.9: 10,000

SAC

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Present

Absent

ASSOCIATED ANOMALIES

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Common

Uncommon

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DEFECT

At umbilicus; 1-15 cm Right of umbilicus; <4cm

MATERNAL AGE

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Average

Younger

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SURGICAL MANAGEMENT

Non urgent

Urgent

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PROGNOSTIC FACTORS

Associated anomalies

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Bowel condition

MORTALITY

<5%

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~ 25%