1. The document discusses various abdominal wall defects including omphalocele, gastroschisis, umbilical hernia, prune belly syndrome, bladder extrophy, cloacal exstrophy, and Pentalogy of Cantrell. 2. It describes the embryology, clinical findings, diagnosis, and management of each condition. Common abdominal wall defects like omphalocele and gastroschisis are discussed in detail including their differences. 3. Rare syndromes associated with abdominal wall defects are also mentioned such as Prune Belly Syndrome, Beckwith-Wiedemann Syndrome, and Pentalogy of Cantrell. The document provides an overview of different abdominal wall birth defects.

1. Dr. Md. Jakir Hossain Bhuiyan
(Sohel)Resident Phase–A
Dr MD AB QUIYUM
Resident,HBS,BSMMU
ABDOMINAL WALL DEFECTS
Welcome

2. OBJECTIVES
 Anatomy of abdominal wall in short
 What are the types of abdominal wall defects
 Embryology of different abdominal wall defects
 Diagnosis and management of different abdominal
wall defects

3. Borders of the Abdomen
• Superior:
Costal cartilages 7-12.
Xiphoid process:
Level of 10th cartilage = L3
• Inferior:
Pubic bone and iliac crest =Level of L4.
• Umbilicus:
Level of IV disc L3-4

4. Abdominal Quadrants
Formed by two intersecting lines:
Intersect at umbilicus.
Quadrants:
Upper left.
Upper right.
Lower left.
Lower right.

5. ABDOMINAL REGIONS
Divided into 9 regions by two pairs of planes:
●Vertical Planes:
Left and right lateral planes
= midclavicular planes
●Horizontal Planes:
Transpyloric plane: tip 9 th CC
Intertubercular plane:Through tubercles of iliac
crests.

6. Fascia
• Superficial:Camper’s fascia Continuous with
fascia over thorax and thigh Fatty layer.
• Deep Superficial:Scarpa’s fascia
-Membranous layer:Continues into perineum
as:Superficial perineal fascia = Colle’s fascia.
• Deep: Thin layer covering abdominal muscles.

7. Muscle LayersMuscle Layers
General Characteristics:
-Three large flat sheets connecting rib cage to
hip bone.
-Muscular posteriorly and laterally.
-Aponeurotic anteriorly and medially.

8. Muscle Layers
• Include:
External oblique.
Internal oblique.
Transversus abdominus.
Rectus abdominus.

9. Arterial Supply
Above the Umbilicus:
●PIA 10-11.●Subcostal artery●LA 1-4.
●MA●Superior epigastric arteries.
●Inferior epigastric arteries.
Below the Umbilicus:
●3s

10. Venous Drainage
• Superficial veins are paired with arteries.
• Above the umbilicus:
Drain into the azygos venous system.
• Below the umbilicus:
Drain into the femoral system (via great
saphenous).

11. OMPHALOCOELE
●Anterior abdominal wall defect at the base of
the umbilical cord with herniation of the
umbilical contents
Small omphalocoele 1:5000
Large omphalocoele 1:10000
Male to female ratio 1:1
Pacific Islanders have low risk for omphalocoele

12. Embryology of Omphalocele
• Failure of the midgut to return to abdomen by
the 10th
week of gestation
(Somatic layers of cephalic, caudal, and lateral
folds join to close abdominal wall)

13. Embryology of Omphalocele

14. Clinical Findings
• Covered clinical defect
of the umbilical ring
• Defect may vary from 2-
10 cm
• Sac is composed of
amnion, Wharton’s jelly
and peritoneum
• Associate ẽ
BA,IA,TTF,SD,VIF,CL

15. Clinical Findings
• 50% have
accompanying liver,
spleen, testes/ovary
• >50% have associated
defects
• Location:
– Epigastric
– Central
– Hypogastric
• Cord attachment is on
the sac

16. Clinical Findings

17. GASTROSCHISISGASTROSCHISIS
Defect of the anterior abdominal wall just
lateral to the umbilicus
1:20,000-30,000
Sex ratio 1:1
10-15% have associated anomalies
40% are premature/SGA

18. Embryology of Gastroschisis
Abnormal involution of right umbilical vein
Rupture of a small omphalocoele
Failure of migration and fusion of the lateral folds of
the embryonic disc on the 3rd
-4th
week of gestation
Lateral folds form
-Lateral abdominal wall
-Future umbilical ring

19. Clinical Findings
• Defect to the right of an
intact UC allowing extrusion
of abdominal content
• Opening ≅ 5 cm
• No covering sac

20. Clinical Findings
• Bowels often
thickened, matted and
edematous
• 10-15% with intestinal
atresia

22. MANAGEMENT
 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

23. MANAGEMENT
Nutrition
 NPO and TPN
Gastric Distention
 OG/NG tube
Infection Control
 Ampicillin and Gentamicin
Associated Defects

24. MANAGEMENT
 Conservative treatment
 Reduction by squeezing the sac
 Painting sac with escharotic agent
0.25% Silver nitrate
0.25% Merbromin (Mercurochrome)

26. MANAGEMENT
 Surgical Management
 Skin Flaps
 Primary Closure
 Staged Closure
Staged repair using silo pouch

27. Skin Flaps

28. Primary Closure

33. Staged Closure

38. Omphalocoele Gastroschisis
Incidence 1:6,000-10,000 1:20,000-30,000
Delivery Vaginal or CS CS
Covering Sac Present Absent
Size of Defect Small or large Small
Cord Location Onto the sac On abdominal wall
Bowel Normal Edematous, matted

39. Omphalocoele Gastroschisis
Other Organs Liver often out Rare
Prematurity 10-20% 50-60%
IUGR Less common Common
NEC If sac is ruptured 18%
Associated
Anomalies
>50% 10-15%
Treatment Often primary Often staged
Prognosis 20%-70% 70-90%

40. UMBILICAL HERNIA
• Defect in linea alba,
subcutaneous tissue
and skin covering the
protruding bowel
• Frequent in premature
infants

41. Pathophysiology
● In intrauterine life, there is a small opening
in the abdominal muscles that allows the UC
to pass through, connecting mother to baby.
● After birth, this opening in the abdominal
muscles closes.
●Sometimes, these muscles do not meet and
grow together completely,

42. Clinical findings
• Bulge or swelling in the belly button area
• May become more noticeable when the baby
cries.
• May become smaller or disappear when the
baby is quiet
• Usually get smaller or go back into the
abdomen when gently pushes on the bulge
• Sometimes incarcerated hernia

43. MANAGEMENT
●Many umbilical hernias close spontaneously by
ages 3 to 4.
●If closure does not occur by this time, surgical
repair is usually advised
●In younger children, if there is an episode of
incarceration or if the hernia is very large, surgical
repair may be recommended.

44. PRUNE BELLY SYNDROME
(AMDS,CAAM, EBS,OS,FS & TS)
A group of birth defects that deal with three main
issues-
•Thin, flaccid abdominal wall
•Dilation of bladder, ureter and renal collecting
system
•UDT
•1:30,000-50,000
•95% are male

45. EMBRYOLOGY
• Myotomal cells from somites form ventral
and dorsal group migrating cells called
hypomeres
• If for some reasons hypomeric group does
not migrate,proliferate or differenciate
within the Abdominal resons

46. • “Little Buddha” appearance
• Constipation
• Delay in sitting and walking
• Difficulties coughing
• Difficulties in micturation
CLINICAL SYMPTOMS

47. BLADDER EXTROPHY
• Defective enfolding of
caudal folds
• 3.3 in 100,000 births
• Associated with prolapsed
vagina or rectum,
epispadias, bifid clitoris or
penis

48. BLADDER EXTROPHY
●Spectrum of severity
-Small defect can result in epispadias
-large defect can result in exposure of
posterior bladder wall
●Exposed bladder mucosa is edematous and
friable

49. EMBRYOLOGY
• It is partial form of spectrum of failure of
abdominal and pelvic fusions in the 1st
month
of embryogenesis
• It occurs as a result of defective migration of
genital tubercle primodii to the clocal
membrane and so malformation of the genital
tubercle at about 5th
week of gestation

50. MANAGEMENT
●Moist, fine-mesh gauze or vaseline gauze to cover
exposed bladder
●Antibiotics
●Renal ultrasound
●Transfer infant to surgical center
-Want surgical correction by 48-72 hours of life
-Sacroiliac joints are still pliable and the pelvis can be
“molded” to allow better approximation of the pubic
rami
●IVP of limited utility b/c of poor concentrating ability
of neonatal kidney

51. MANAGEMENT
●Turn-in of the bladder to preserve bladder
function
●Symphysis pubis is approximated
Iliac osteotomies aren’t necessary if ●repair
is within 48 HOL (bones are still pliable
from circulating maternal estrogens)
●Epispadiac urethra is reconstructed later

52. Cloacal Exstrophy
●Very rare: 1 in 200,000 births
●Sporadic occurrence
●Complex of GI and GU anomalies:
-Imperforate anus
-Exstrophy of the bladder
-Omphalocele
-Vesicointestinal fistula
-Frequently prolapse of bowel thru the
fistula on bladder mucosa

53. EMBRYOLOGY
• Abnormal over development of the clocal
membrane which prevents medial
migration of the mesenchymal tissue and
proper lower abdominal wall development

54. MANAGEMENT
●Cover exposed mucosa (vaseline/saline gauze)
and/or plastic wrap to minimize heat loss
●Gender assignment / diagnosis
-Karyotype / FISH
-Controversial regarding assignment
●NG suction to relieve partial intestinal
obstruction.
-Stool frequently excreted through a vesico-
intestinal fistula that is often partially
obstructed

55. Cloacal Exstrophy - Operative
●Survival 80% - mortality due to sepsis and
bowel obstruction
●Prompt surgery to separate fecal and urinary
streams
●Bladder can be closed during initial procedure
if baby stable
●Subsequent procedures to reduce the number
of stomas and create genitalia

56. COMPLICATIONS
Multiple Long term problems:
Psychosocial / Gender Identification
Recurrent UTIs
Bowel/Bladder Incontinence
Sexual function in later life

57. PENTALOGY OF CANTRELL
• Omphalocoele
• Anterior diaphragmatic hernia
• Sternal cleft
• Ectopia Cordis
• Intracardiac defect

59. BECKWITH-WIEDEMANN
SYNDROME (MOEAM)
• Macrosomia
• Macroglossia
• Organomegaly
• Abdominal wall
defects
• Embryonal tumors

60. Thanks to
all