Abdominal wall defects

1. Abdominal wall defectsAbdominal wall defects

2. EmbryologyEmbryology
 Normal 2wk embryo is a flat disc that containsNormal 2wk embryo is a flat disc that contains
ectoderm, endoderm & mesodermectoderm, endoderm & mesoderm
 Intraembryonic coelom divides mesoderm intoIntraembryonic coelom divides mesoderm into
sphlancnoplueric & somatoplueric mesodermsphlancnoplueric & somatoplueric mesoderm
 4 folds appear4 folds appear
Cephalic fold: thoracic & epigastric wallCephalic fold: thoracic & epigastric wall
Caudal fold: hindgut, bladder & hypogastricCaudal fold: hindgut, bladder & hypogastric
wallwall
Lateral folds: lateral abdominal wall.Lateral folds: lateral abdominal wall.
Four folds meet to form the umbilical ring byFour folds meet to form the umbilical ring by
4rth week4rth week

4.  Physiological herniation of gut during the 6Physiological herniation of gut during the 6
– 10th week.– 10th week.
 Small defects at umbilicus: probablySmall defects at umbilicus: probably
failure of intestine to return into thefailure of intestine to return into the
peritoneal cavityperitoneal cavity
 Large defects: Failure of development ofLarge defects: Failure of development of
body wall.body wall.
ExomphalosExomphalos
GastroschisisGastroschisis
Extrophy bladderExtrophy bladder

5. ExomphalosExomphalos
 Central defect at the site of the umbilicalCentral defect at the site of the umbilical
ringring
 Eviscerated contents are covered by a sacEviscerated contents are covered by a sac
formed by peritoneum, whartons jelly &formed by peritoneum, whartons jelly &
amnionamnion

6.  Size 4 – 12cmsSize 4 – 12cms
 Umbilical cord is inserted onto the sacUmbilical cord is inserted onto the sac
 Contents: Usually small & large bowel,Contents: Usually small & large bowel,
sometimes stomach & liversometimes stomach & liver
 Abdominal muscles are well developed,Abdominal muscles are well developed,
coelom not well developedcoelom not well developed

7.  Congenital hernia of the cord:Congenital hernia of the cord:
Less than 4 cms diameterLess than 4 cms diameter
Contain few loops of intestineContain few loops of intestine
May be missed at birthMay be missed at birth
Careless clamping may result in injuryCareless clamping may result in injury
 Giant omphalocoeles:Giant omphalocoeles:
Massive sac containing most of theMassive sac containing most of the
abdominal viscera including liver,abdominal viscera including liver,
spleen, gall bladder, gonads,spleen, gall bladder, gonads,
intestines.intestines.

8.  GastroschisisGastroschisis::
 Smooth edged defect locatedSmooth edged defect located
adjacent to a normal umbilical cord.adjacent to a normal umbilical cord.
Ocassionaly separated from theOcassionaly separated from the
cord by a strip of skin.cord by a strip of skin.
 Almost always to the right of theAlmost always to the right of the
umbilicusumbilicus
 Size 2-5 cms, often dangerouslySize 2-5 cms, often dangerously
small compared to the size of thesmall compared to the size of the
eviscerated organs.eviscerated organs.

9.  Stomach, small & large intestine areStomach, small & large intestine are
commonly herniated.commonly herniated.
 There is no sac, hence exposed toThere is no sac, hence exposed to
amniotic fluid.amniotic fluid.
 Exposed bowel often foreshortened,Exposed bowel often foreshortened,
edematous, covered by thickedematous, covered by thick
exudates. May be ischemic.exudates. May be ischemic.

11. Associated anomalies:Associated anomalies:
 Pentalogy of CantrellPentalogy of Cantrell
( defect of cephalic fold)( defect of cephalic fold)
OmphalocoeleOmphalocoele
Anterior diaphragmaticAnterior diaphragmatic
herniahernia
Sternal cleftSternal cleft
Ectopia cordisEctopia cordis
Cardiac anomaliesCardiac anomalies
 Lower midline defect:Lower midline defect:
Bladder / cloacalBladder / cloacal
extrophyextrophy
ARMARM
MMCMMC
Sacral vertebralSacral vertebral
anomaliesanomalies
Major congenital anomalies are often seen

12. ManagementManagement
 Immediate post natal :Immediate post natal :
 NG aspirationNG aspiration
 IV Fluid managementIV Fluid management
 CatheterisationCatheterisation
 Maintain body temperatureMaintain body temperature
 DressingDressing

13. Surgical managementSurgical management
 Could be in single / multiple stagesCould be in single / multiple stages
 Exomphalos:Exomphalos:
 Excise the sacExcise the sac
 Put the contents back into thePut the contents back into the
abdomen after inspectionabdomen after inspection
 Measure abdominal pressureMeasure abdominal pressure

14.  If pressure lower than 20cms of HIf pressure lower than 20cms of H2200
proceed with primary repair of theproceed with primary repair of the
defectdefect
 If pressure is high, close only theIf pressure is high, close only the
skin to make a ventral hernia forskin to make a ventral hernia for
repair laterrepair later
 If peritoneal cavity is small & notIf peritoneal cavity is small & not
accepting contents, apply prostheticaccepting contents, apply prosthetic
closureclosure

15.  Single running suture is applied at theSingle running suture is applied at the
top of the sac. Suture reapplied everydaytop of the sac. Suture reapplied everyday
and contents are gradually reduced overand contents are gradually reduced over
a period of 8 – 10 days. Then defect isa period of 8 – 10 days. Then defect is
repairedrepaired..
Dacron reinforcedDacron reinforced
silastic sheet is used as asilastic sheet is used as a
prosthetic sac.prosthetic sac.
It is sutured to theIt is sutured to the
fascia around thefascia around the
circumference of thecircumference of the
defect.defect.

16. Extrophy – Epispadias ComplexExtrophy – Epispadias Complex
 Abnormal over-development of cloacalAbnormal over-development of cloacal
membrane preventing migration ofmembrane preventing migration of
mesenchymal tissue and development ofmesenchymal tissue and development of
lower abdominal wall.lower abdominal wall.
 Incidence: 1 in 20,000 live births.Incidence: 1 in 20,000 live births.

17. AnatomyAnatomy
 Musculoskeletal defect:Musculoskeletal defect:
Outward rotation of iliac bonesOutward rotation of iliac bones
results in wide pubic diastasis. Pelvicresults in wide pubic diastasis. Pelvic
diaphragm is open (divergent) anddiaphragm is open (divergent) and
incompetent. High incidence of rectalincompetent. High incidence of rectal
prolapseprolapse

18.  Urinary defectsUrinary defects
Anterior wall of bladder absentAnterior wall of bladder absent
Mucosa of posterior wall , trigone, uretericMucosa of posterior wall , trigone, ureteric
orifices & bladder neck exposedorifices & bladder neck exposed
Bladder plate may be large & elastic orBladder plate may be large & elastic or
small, fibrosed & unelastic.small, fibrosed & unelastic.
Mucosa may be normal, polypoid or undergoMucosa may be normal, polypoid or undergo
squamous metaplasia.squamous metaplasia.
Upper tracts & kidneys are usually normal.Upper tracts & kidneys are usually normal.

19.  Anorectal:Anorectal:
Perineum is short & broad. Anus displacedPerineum is short & broad. Anus displaced
anteriorlyanteriorly
 Male genital defect:Male genital defect:
Severe - EpispadiasSevere - Epispadias
Phallus is foreshortened because of widePhallus is foreshortened because of wide
separation of crural attachmentseparation of crural attachment
Prominent dorsal chordeeProminent dorsal chordee

20. Short urethral grooveShort urethral groove
External sphincter deficientExternal sphincter deficient
 Female genital defectFemale genital defect
Short vagina. Stenosis commonShort vagina. Stenosis common
Clitoris is bifid and labia divergentClitoris is bifid and labia divergent

22. Problems in managementProblems in management
 Bladder plate may be inadequateBladder plate may be inadequate
 Large fascial defect on bladder closure. DifficultLarge fascial defect on bladder closure. Difficult
to repair inspite of osteotomiesto repair inspite of osteotomies
 Chances of continence after surgery is poorChances of continence after surgery is poor
 Extremely difficult to attain cosmeticallyExtremely difficult to attain cosmetically
satisfying reconstruction of genitaliasatisfying reconstruction of genitalia
 Fertility poor.Fertility poor.

23. ManagementManagement
 Staged repairStaged repair
 Stg 1: Bladder closure atStg 1: Bladder closure at
presentationpresentation
 Stg 2: Epispadias repair at 6 – 12Stg 2: Epispadias repair at 6 – 12
monthsmonths
 Stg 3: Bladder neck repair at 4 yrsStg 3: Bladder neck repair at 4 yrs