Abdominal wall defectsAbdominal wall defects

EmbryologyEmbryology Normal 2wk embryo is a flat disc that contains Normal 2wk embryo is a flat disc that contains

ectoderm, endoderm & mesodermectoderm, endoderm & mesoderm Intraembryonic coelom divides mesoderm into Intraembryonic 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 & hypogastric wallCaudal fold: hindgut, bladder & hypogastric wall

Lateral folds: lateral abdominal wall.Lateral folds: lateral abdominal wall.

Four folds meet to form the umbilical ring by Four folds meet to form the umbilical ring by 4rth week4rth week

Physiological herniation of gut during the 6 Physiological herniation of gut during the 6 – 10th week.– 10th week.

Small defects at umbilicus: probably Small defects at umbilicus: probably failure of intestine to return into the failure of intestine to return into the peritoneal cavityperitoneal cavity

Large defects: Failure of development of Large defects: Failure of development of body wall.body wall.

ExomphalosExomphalos

GastroschisisGastroschisis

Extrophy bladderExtrophy bladder

ExomphalosExomphalos

Central defect at the site of the umbilical Central defect at the site of the umbilical ringring

Eviscerated contents are covered by a sac Eviscerated contents are covered by a sac formed by peritoneum, whartons jelly & formed by peritoneum, whartons jelly & amnionamnion

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

Congenital hernia of the cord:Congenital hernia of the cord: Less than 4 cms diameterLess than 4 cms diameterContain few loops of intestineContain few loops of intestineMay be missed at birthMay be missed at birthCareless clamping may result in injuryCareless clamping may result in injury

Giant omphalocoeles:Giant omphalocoeles: Massive sac containing most of the Massive sac containing most of the

abdominal viscera including liver, abdominal viscera including liver, spleen, gall bladder, gonads, spleen, gall bladder, gonads, intestines.intestines.

GastroschisisGastroschisis::

Smooth edged defect located Smooth edged defect located adjacent to a normal umbilical cord. adjacent to a normal umbilical cord. Ocassionaly separated from the Ocassionaly separated from the cord by a strip of skin.cord by a strip of skin.

Almost always to the right of the Almost always to the right of the umbilicusumbilicus

Size 2-5 cms, often dangerously Size 2-5 cms, often dangerously small compared to the size of the small compared to the size of the eviscerated organs.eviscerated organs.

Stomach, small & large intestine are Stomach, small & large intestine are commonly herniated.commonly herniated.

There is no sac, hence exposed to There is no sac, hence exposed to amniotic fluid. amniotic fluid.

Exposed bowel often foreshortened, Exposed bowel often foreshortened, edematous, covered by thick edematous, covered by thick exudates. May be ischemic.exudates. May be ischemic.

Associated anomalies:Associated anomalies:

Pentalogy of Cantrell Pentalogy of Cantrell

( defect of cephalic fold)( defect of cephalic fold)

OmphalocoeleOmphalocoele

Anterior diaphragmatic Anterior diaphragmatic herniahernia

Sternal cleftSternal cleft

Ectopia cordisEctopia cordis

Cardiac anomaliesCardiac anomalies

Lower midline defect:Lower midline defect:

Bladder / cloacal Bladder / cloacal extrophyextrophy

ARMARM

MMCMMC

Sacral vertebral Sacral vertebral anomaliesanomalies

Major congenital anomalies are often seen

ManagementManagement

Immediate post natal :Immediate post natal :

NG aspirationNG aspiration IV Fluid managementIV Fluid management CatheterisationCatheterisation Maintain body temperatureMaintain body temperature Dressing Dressing

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 the Put the contents back into the abdomen after inspectionabdomen after inspection

Measure abdominal pressureMeasure abdominal pressure

If pressure lower than 20cms of HIf pressure lower than 20cms of H220 0 proceed with primary repair of the proceed with primary repair of the defectdefect

If pressure is high, close only the If pressure is high, close only the skin to make a ventral hernia for skin to make a ventral hernia for repair laterrepair later

If peritoneal cavity is small & not If peritoneal cavity is small & not

accepting contents, apply prosthetic accepting contents, apply prosthetic closureclosure

Single running suture is applied at the top Single running suture is applied at the top of the sac. Suture reapplied everyday and of the sac. Suture reapplied everyday and contents are gradually reduced over a contents are gradually reduced over a period of 8 – 10 days. Then defect is period of 8 – 10 days. Then defect is repairedrepaired..

Dacron reinforced Dacron reinforced silastic sheet is used as a silastic sheet is used as a prosthetic sac.prosthetic sac.

It is sutured to the fascia It is sutured to the fascia around the circumference around the circumference of the defect.of the defect.

Extrophy – Epispadias ComplexExtrophy – Epispadias Complex

Abnormal over-development of cloacal Abnormal over-development of cloacal membrane preventing migration of membrane preventing migration of mesenchymal tissue and development mesenchymal tissue and development of lower abdominal wall.of lower abdominal wall.

Incidence: 1 in 20,000 live births.Incidence: 1 in 20,000 live births.

AnatomyAnatomy

Musculoskeletal defect:Musculoskeletal defect: Outward rotation of iliac bones Outward rotation of iliac bones

results in wide pubic diastasis. Pelvic results in wide pubic diastasis. Pelvic diaphragm is open (divergent) and diaphragm is open (divergent) and incompetent. High incidence of rectal incompetent. High incidence of rectal prolapseprolapse

Urinary defectsUrinary defects

Anterior wall of bladder absentAnterior wall of bladder absent

Mucosa of posterior wall , trigone, ureteric Mucosa of posterior wall , trigone, ureteric orifices & bladder neck exposedorifices & bladder neck exposed

Bladder plate may be large & elastic or Bladder plate may be large & elastic or small, fibrosed & unelastic.small, fibrosed & unelastic.

Mucosa may be normal, polypoid or undergo Mucosa may be normal, polypoid or undergo squamous metaplasia.squamous metaplasia.

Upper tracts & kidneys are usually normal.Upper tracts & kidneys are usually normal.

Anorectal:Anorectal:Perineum is short & broad. Anus displaced Perineum is short & broad. Anus displaced

anteriorlyanteriorly

Male genital defect:Male genital defect:

Severe - EpispadiasSevere - Epispadias

Phallus is foreshortened because of wide Phallus is foreshortened because of wide separation of crural attachmentseparation of crural attachment

Prominent dorsal chordeeProminent dorsal chordee

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

Problems in managementProblems in management Bladder plate may be inadequateBladder plate may be inadequate

Large fascial defect on bladder closure. Difficult Large 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 cosmetically Extremely difficult to attain cosmetically satisfying reconstruction of genitaliasatisfying reconstruction of genitalia

Fertility poor.Fertility poor.

ManagementManagement Staged repairStaged repair

Stg 1: Bladder closure at Stg 1: Bladder closure at presentationpresentation

Stg 2: Epispadias repair at 6 – 12 Stg 2: Epispadias repair at 6 – 12 monthsmonths

Stg 3: Bladder neck repair at 4 yrsStg 3: Bladder neck repair at 4 yrs