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Hakim J. Jamba
Moderator: Dr. Dawit Teare, Consultant Surgeon
Contents
 Perinatal Management
 Preoperative
 Gastroschisis
 Exomphalo
 Umbilical hernia
 Other anomalies
 Summary
 References
2
Perinatal Management
 Maternal screening
 - Alpha fetoprotein
 90% exoph , 10% gastro
 U/S scanning
 dilated bowel; 7-25 mm
 Prenatal Counselling
 Mode of delivery
3
Preoperative Manag.
 Fluid Management
 IV bolus 20 ml/kg LR/NS
 D10¼NS 2-3 maintenance rate
 Heat Management
 Sterile wrap or sterile bowel bag
 Radiant warmer
 Hypoglycaemia (Beikwith Weidemann Synd)
 Positioning
4
 Nutrition
 NPO and TPN
 Gastric Distention
 OG/NG tube
 Infection Control
 Associated Defects
5
Gastroschisis 6
 Primary closure
 If bowel easily reduced
 Fascial grafts with skin mobilization and closure
 May need skin flaps and secondary healing of the
new defects
 Silastic covering to prevent evisceration then closed
4-5 days later after improvement of the pulmonary
state and intra-abdominal pressure
7
 Staged closure:
 Silo/Springed, sequential ligation
 Taking 1-14 days
 hospitalization, ventilation & early feeding
 Definitive closure;
 Skin flaps
 Horizontal fascia closure
 Linear skin closure (keyhole)/Purse-string
 Umbilical Plastic closure
 Cord/bowel directly covered by adhesive dressing
 60-84% ventral hernia, majority close spontaneously
8
 Associated atresia:
 10%
 Treated at wall closure or 4-6 weeks after
 Stoma for distal atresias
 Perforation:
 REEA if minimal inflammation
 Ostomy and closure at a later date
 Exteriorized through the silo
9
10
 Vanishing bowel;
 a very small
abdominal wall
defect
 Necrosis and
disappearance of
some or all of the
intestine
 it usually results
in short bowel
syndrome.
Postoperative course
 Feeding delayed for weeks
 Oral stimulation/sucking reflex
 Dysmotility;
 prokinetics may improve i.e. erythromycin,
cisapride, metocloprimide
11
Long term outcome
 Generally excellent if no atresia
 NEC:
 18.5% of neonates more with formula
 Bowel loss short bowel syndrome
 Cryptorchidism:
 15-30%
 Due either being outside/prematurity
 Replacement and orchidopexy by 1 yr
 60% have psychosocial stress if umbilicus scrificed
12
Exomphalo 13
 Treatment options depend on the size, gestational
age, and associated anomalies
 Escharotic therapy/ Conservative;
 Gradual epithelialization of the omphalocele sac
 Mercurochrome, iodine, polymer membrane, silver
sulfadiazine, 70-90% alcohol, and Nystatin powder
 It usually takes many months to granulate and
epithelialize then managed as a ventral hernia
14
 70-90% alcohol
 Closed dressing for intact sacs
 Abdomen cleansed using plain antiseptic
 2-3 layers of soft cotton gauze placed
 Soft crepe bandage 4-6 inches applied
 Kept for 24-48 hrs
 Changed daily if moist
 Twice daily if infection is evident
 7-10 days hospitalization only
 Leaves a ventral hernia
15
 Primary closure may be appropriate for small
defects
 consists of excision of the sac and closure of the
fascia and skin over the abdominal contents
 Delayed closure
 medium-sized,
 the hepatic veins are located just under the
epithelium/sac interface in the midline and can be
injured
 haemorrhage can result from tears in Glissen’s
capsule or bladder injury
16
 Large defects;
 Bilateral flaps
 Component separation
 Tissue expanders in the cavity
 Fascial patch;
 Marlex, Gore-Tex infection
 Dura, SB submucosa,
17
 Staged closure:
 Historically; skin flaps with resultant large ventral
hernias
 Moderate ones with thick sacs can be considered
as silo
 Silo fashioning;
 Sac excised
 Silo sewn to rectus fascia/full thickness
18
Postoperative course
 Ventilation
 Feeding;
 No bilious output
 Minimal volume
 Bowel activity
 48 hrs Antibiotics
 Hernia dealt with at 1 yr old
 12% increased IAP & acute hepatic congestion
 25% skin and fascia dehiscence
19
Long-Term outcome
 Small; recover well
 Large;
 Gastro-oesophageal reflux - 43%
 Majority improve over time
 20% pulmonary insufficiency
 Respiratory Infections
 Asthma
 Feeding difficulties;
 60% with giant Exomph
 May need gastrostomy for feeding
 Failure to thrive
20
UMBILICAL HERNIA
 Defect in linea
alba,
subcutaneous
tissue and skin
covering the
protruding bowel
 Frequent in
premature infants
21
PENTALOGY OF CANTRELL
 Omphalocoele
 Anterior diaphragmatic hernia
 Sternal cleft
 Ectopia Cordis
 Intracardiac defect
22
23
BECKWITH-WIEDEMANN
SYNDROME
 Macrosomia
 Macroglossia
 Organomegaly
 Abdominal wall
defects
 Embryonal tumors
24
Compendium
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
25
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%
26
References 27
28
Intra-abdominal pressure
 10-15 mmHg kidney & bowel hypoperfusion
 Use Silo/Patch if
 IAP: >20 mmHg
 CVP: > 4 mmHg increment
 Bladder: >20 mmHg
 Splanch Perfusion pressure: <44 mmHg
29
30
Dilatation 31
Component separation 32
Conservative 33
Skin Flaps
34
Primary Closure
35
36
37
Primary closure 38
39
Bandage 40
Staged Closure 41
42
Staged 43
44
Primary Omph 45
46
Silo bag 47
Fascial graft 48
49
Umbilicus 50
Prenatal Counselling
 Description and cause of
abdominal wall defects
 Size of the defect and
how that may influence
management
 What organs are involved
in the abdominal wall
defect
 Other anomalies the
infant may have
 Timing of delivery
 Management following
birth, including
resuscitation and the
possible need for
intubation
 Neonatal intensive care
unit
 Treatment goals
 Surgical options
 Anticipated fetal course
 Treatment options and
timeline of treatment
 Potential complications
 Prognosis and quality-of-
life issues
51
Anomalies 52
53
GASTROSCHISIS
 Defect of the anterior abdominal wall just lateral
to the umbilicus
54
Incidence
 1:20,000-30,000
 Sex ratio 1:1
 10-15% have associated anomalies
 40% are premature/SGA
55
Pathophysiology
 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
56
Lateral folds
 Form
 Lateral abdominal wall
 Future umbilical ring
57
58
Clinical Findings
 Defect to the right
of an intact
umbilical cord
allowing extrusion
of abdominal
content
 Opening  5 cm
 No covering sac
59
 Bowels often
thickened, matted
and edematous
 10-15% with
intestinal atresia
60
61
OMPHALOCOELE
 Anterior abdominal wall defect at the base of the
umbilical cord with herniation of the umbilical
contents
62
Incidence
 Small omphalocoele 1:5000
 Large omphalocoele 1:10000
 Male to female ratio 1:1
 Pacific Islanders have low risk for omphalocoele
63
Pathophysiology
 Failure of the midgut to return to abdomen by the
10th week of gestation
64
65
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
66
 50% have
accompanying
liver, spleen,
testes/ovary
 >50% have
associated defects
 Location:
 Epigastric
 Central
 Hypogastric
 Cord attachment is
on the sac
67
PRUNE BELLY SYNDROME
 Thin, flaccid abdominal wall
 Dilation of bladder, ureter and renal collecting
system
 1:30,000-50,000
 95% are male
68
69
BLADDER EXTROPHY
 Defective enfolding
of caudal folds
 3.3 in 100,000 births
 Associated with
prolapsed vagina or
rectum, epispadias,
bifid clitoris or penis
70

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Management of abdominal wall defects

  • 1. Hakim J. Jamba Moderator: Dr. Dawit Teare, Consultant Surgeon
  • 2. Contents  Perinatal Management  Preoperative  Gastroschisis  Exomphalo  Umbilical hernia  Other anomalies  Summary  References 2
  • 3. Perinatal Management  Maternal screening  - Alpha fetoprotein  90% exoph , 10% gastro  U/S scanning  dilated bowel; 7-25 mm  Prenatal Counselling  Mode of delivery 3
  • 4. Preoperative Manag.  Fluid Management  IV bolus 20 ml/kg LR/NS  D10¼NS 2-3 maintenance rate  Heat Management  Sterile wrap or sterile bowel bag  Radiant warmer  Hypoglycaemia (Beikwith Weidemann Synd)  Positioning 4
  • 5.  Nutrition  NPO and TPN  Gastric Distention  OG/NG tube  Infection Control  Associated Defects 5
  • 7.  Primary closure  If bowel easily reduced  Fascial grafts with skin mobilization and closure  May need skin flaps and secondary healing of the new defects  Silastic covering to prevent evisceration then closed 4-5 days later after improvement of the pulmonary state and intra-abdominal pressure 7
  • 8.  Staged closure:  Silo/Springed, sequential ligation  Taking 1-14 days  hospitalization, ventilation & early feeding  Definitive closure;  Skin flaps  Horizontal fascia closure  Linear skin closure (keyhole)/Purse-string  Umbilical Plastic closure  Cord/bowel directly covered by adhesive dressing  60-84% ventral hernia, majority close spontaneously 8
  • 9.  Associated atresia:  10%  Treated at wall closure or 4-6 weeks after  Stoma for distal atresias  Perforation:  REEA if minimal inflammation  Ostomy and closure at a later date  Exteriorized through the silo 9
  • 10. 10  Vanishing bowel;  a very small abdominal wall defect  Necrosis and disappearance of some or all of the intestine  it usually results in short bowel syndrome.
  • 11. Postoperative course  Feeding delayed for weeks  Oral stimulation/sucking reflex  Dysmotility;  prokinetics may improve i.e. erythromycin, cisapride, metocloprimide 11
  • 12. Long term outcome  Generally excellent if no atresia  NEC:  18.5% of neonates more with formula  Bowel loss short bowel syndrome  Cryptorchidism:  15-30%  Due either being outside/prematurity  Replacement and orchidopexy by 1 yr  60% have psychosocial stress if umbilicus scrificed 12
  • 14.  Treatment options depend on the size, gestational age, and associated anomalies  Escharotic therapy/ Conservative;  Gradual epithelialization of the omphalocele sac  Mercurochrome, iodine, polymer membrane, silver sulfadiazine, 70-90% alcohol, and Nystatin powder  It usually takes many months to granulate and epithelialize then managed as a ventral hernia 14
  • 15.  70-90% alcohol  Closed dressing for intact sacs  Abdomen cleansed using plain antiseptic  2-3 layers of soft cotton gauze placed  Soft crepe bandage 4-6 inches applied  Kept for 24-48 hrs  Changed daily if moist  Twice daily if infection is evident  7-10 days hospitalization only  Leaves a ventral hernia 15
  • 16.  Primary closure may be appropriate for small defects  consists of excision of the sac and closure of the fascia and skin over the abdominal contents  Delayed closure  medium-sized,  the hepatic veins are located just under the epithelium/sac interface in the midline and can be injured  haemorrhage can result from tears in Glissen’s capsule or bladder injury 16
  • 17.  Large defects;  Bilateral flaps  Component separation  Tissue expanders in the cavity  Fascial patch;  Marlex, Gore-Tex infection  Dura, SB submucosa, 17
  • 18.  Staged closure:  Historically; skin flaps with resultant large ventral hernias  Moderate ones with thick sacs can be considered as silo  Silo fashioning;  Sac excised  Silo sewn to rectus fascia/full thickness 18
  • 19. Postoperative course  Ventilation  Feeding;  No bilious output  Minimal volume  Bowel activity  48 hrs Antibiotics  Hernia dealt with at 1 yr old  12% increased IAP & acute hepatic congestion  25% skin and fascia dehiscence 19
  • 20. Long-Term outcome  Small; recover well  Large;  Gastro-oesophageal reflux - 43%  Majority improve over time  20% pulmonary insufficiency  Respiratory Infections  Asthma  Feeding difficulties;  60% with giant Exomph  May need gastrostomy for feeding  Failure to thrive 20
  • 21. UMBILICAL HERNIA  Defect in linea alba, subcutaneous tissue and skin covering the protruding bowel  Frequent in premature infants 21
  • 22. PENTALOGY OF CANTRELL  Omphalocoele  Anterior diaphragmatic hernia  Sternal cleft  Ectopia Cordis  Intracardiac defect 22
  • 23. 23
  • 24. BECKWITH-WIEDEMANN SYNDROME  Macrosomia  Macroglossia  Organomegaly  Abdominal wall defects  Embryonal tumors 24
  • 25. Compendium 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 25
  • 26. 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% 26
  • 28. 28
  • 29. Intra-abdominal pressure  10-15 mmHg kidney & bowel hypoperfusion  Use Silo/Patch if  IAP: >20 mmHg  CVP: > 4 mmHg increment  Bladder: >20 mmHg  Splanch Perfusion pressure: <44 mmHg 29
  • 30. 30
  • 36. 36
  • 37. 37
  • 39. 39
  • 42. 42
  • 44. 44
  • 46. 46
  • 49. 49
  • 51. Prenatal Counselling  Description and cause of abdominal wall defects  Size of the defect and how that may influence management  What organs are involved in the abdominal wall defect  Other anomalies the infant may have  Timing of delivery  Management following birth, including resuscitation and the possible need for intubation  Neonatal intensive care unit  Treatment goals  Surgical options  Anticipated fetal course  Treatment options and timeline of treatment  Potential complications  Prognosis and quality-of- life issues 51
  • 53. 53
  • 54. GASTROSCHISIS  Defect of the anterior abdominal wall just lateral to the umbilicus 54
  • 55. Incidence  1:20,000-30,000  Sex ratio 1:1  10-15% have associated anomalies  40% are premature/SGA 55
  • 56. Pathophysiology  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 56
  • 57. Lateral folds  Form  Lateral abdominal wall  Future umbilical ring 57
  • 58. 58
  • 59. Clinical Findings  Defect to the right of an intact umbilical cord allowing extrusion of abdominal content  Opening  5 cm  No covering sac 59
  • 60.  Bowels often thickened, matted and edematous  10-15% with intestinal atresia 60
  • 61. 61
  • 62. OMPHALOCOELE  Anterior abdominal wall defect at the base of the umbilical cord with herniation of the umbilical contents 62
  • 63. Incidence  Small omphalocoele 1:5000  Large omphalocoele 1:10000  Male to female ratio 1:1  Pacific Islanders have low risk for omphalocoele 63
  • 64. Pathophysiology  Failure of the midgut to return to abdomen by the 10th week of gestation 64
  • 65. 65
  • 66. 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 66
  • 67.  50% have accompanying liver, spleen, testes/ovary  >50% have associated defects  Location:  Epigastric  Central  Hypogastric  Cord attachment is on the sac 67
  • 68. PRUNE BELLY SYNDROME  Thin, flaccid abdominal wall  Dilation of bladder, ureter and renal collecting system  1:30,000-50,000  95% are male 68
  • 69. 69
  • 70. BLADDER EXTROPHY  Defective enfolding of caudal folds  3.3 in 100,000 births  Associated with prolapsed vagina or rectum, epispadias, bifid clitoris or penis 70

Notas del editor

  1. Induced at 36-37 wks
  2. Ampicillin and Gentamicin
  3. care must be taken when excising the portion of the sac covering the liver
  4. Curtis graft