ABDOMINAL WALL DEFECTS

& MALROTATION
OMPHALOCOELE

 It is a defect in abdominal wall musculature and

skin with protrusion of abdominal viscera
contained within a membranous sac.
 3/23/2014
 Exomphalos major (umbilical defect

Prof Khaled H.K. Bahaaeldin

>5cm)
 Exomphalos minor (umbilical
defect<5cm)

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3/23/2014 Prof Khaled H.K. Bahaaeldin
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 Abdominal wall

OMPHALOCELE
defect
INCIDENCE

 Small omphalocoele 1:5000

 Large omphalocoele 1:10000

 Male to female ratio 1:1

 Pacific Islanders have low risk for omphalocoele

PATHOPHYSIOLOGY

 Failure of the midgut to return to abdomen by

the 10th week of gestation
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
 50% have
accompanying liver,
spleen, testes/ovary
 >50% have
associated defects
 Location:
 Epigastric
 Central
 Hypogastric
 Cordattachment is
on the sac
 The sac may rupture in utero in 10-18% or from
the delivery process in4%.
 The incidence of associated major congenital
anomalies in up to 81%.
DEFECTS OF CRANIAL FOLD
 congenital heart disease
 diaphragmatic hernia

 ectopia cordis

 sternal cleft,
DEFECTS OF CAUDAD FOLD
 Imperforate anus
 Genitourinary malformations

 Bladder or cloacal exstrophy

 Colon atresia

 Sacral and vertebral anomalies, and

 Meningomyelocele.
GASTROSCHISIS

 It is the defect in the abdominal wall was

displaced to the right of the umbilicus and
eviscerated bowel was not covered by a
membrane.
 Abdominal wall

GASTROSCHISIS
defect
INCIDENCE

 1:20,000-30,000
 Sex ratio 1:1

 10-15% have associated anomalies

 40% are premature/SGA

 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
CLINICAL FINDINGS
 Defect to the right
of an intact
umbilical cord
allowing extrusion
of abdominal
content
 Opening  5 cm

 No covering sac
 Bowels often
thickened, matted
and edematous
 10-15% with
intestinal atresia
 Evisceration of the bowel leads to malrotation.
 Constriction of the base may cause intestinal
stenosis, atresia, and volvulus
 Undescended testicles

 preterm or small for gestational age (SGA)

CAUSES
Folic acid deficiency
 hypoxia

 salicylates
DIAGNOSIS
 History : Prenatal U/S
 Polyhydramnios

 MSAFP

 Amniocentesis
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
 Nutrition
 NPO and TPN (central venous line )
 Gastric Distention
 OG/NG tube
 urinary catheter
 Infection Control
Broad-spectrum antibiotics
 Associated Defects
 Conservative treatment
 Reduction by squeezing the sac
 Painting sac with escharotic agent
0.25% Silver nitrate
0.25% Merbromin (Mercurochrome)
 Surgical Management
 Skin Flaps
 Primary Closure
 Staged Closure
 Staged repair using silo pouch
SKIN FLAPS
PRIMARY CLOSURE
 In 1967, Schuster technique
 A circumferential incision along the skin-
omphalocele junction; the omphalocele membrane
is left intact
 Teflon sheets

 DualMesh patch (Gore-Tex)

 AlloDerm patch (acellular human dermis)

PRIMARY CLOSURE
STAGED CLOSURE
In 1969, Allen and Wrenn adapted Schuster's
technique to treat gastroschisis
Silo Procedure.
STAGED CLOSURE
 Technically
Gastroschisis and
Omphaloceles are all
malrotated.
 Are they at increased
risk of volvulus after
closure of the defect?
UMBILICAL HERNIA

 Defect in linea alba,

subcutaneous tissue
and skin covering
the protruding
bowel
 Frequent in
premature infants
PRUNE BELLY SYNDROME
 1:30,000-50,000
 95% are male
 A partial or complete lack of abdominal wall muscles.
There are wrinkly folds of skin covering the abdomen.
 Undescended testicles
 Urinary tract abnormality such as unusually
large ureters, distended bladder, vesicoureteral
reflux
 Frequent urinary tract infections
 VSD
 Malrotation of the gut
 Club foot
 Tx:
 Treatment of the associated anomalies
 Usually end up with a cystostomy for urinary
drainage
BLADDER EXTROPHY
(ECTOPIA VESICAE)
 A defect in the abdominal wall
occupied by both the
exstrophied bladder as well as
a portion of the urethra
 3.3 in 100,000 births
 Separation of the pubic
symphysis
 Shortening of the pubic rami
 External rotation of the pelvis.
 Associated with prolapsed
vagina or rectum, epispadias,
bifid clitoris or penis
 Tx: Reconstruction
 PENTALOGY OF CANTRELL

 Omphalocoele
 Anterior diaphragmatic hernia

 Sternal cleft

 Ectopia Cordis

 Intracardiac defect
BECKWITH-WIEDEMANN
SYNDROME

 Macrosomia

 Macroglossia

 Organomegaly

 Abdominal wall
defects
 Embryonal tumors
Have coarse, rounded facial features
 hyperplasia of the pancreatic islet cells with
hypoglycemia; visceromegaly
 genitourinary abnormalities
 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

 Omphalocoele Gastroschisis
Other Organs Liver often out Rare
Prematurity 10-20% 50-60%
IUGR Less common Common
NEC If sac is ruptured 18%
Associated >50% 10-15%
Anomalies
Treatment Often primary Often staged
Prognosis 20%-70% 70-90%
Baby with an
umbilical cord
hernia.
Baby with
gastroschisis and
associated
intestinal atresia
Silo closure of a
baby with
gastroschisis.
Completed
reduction of the
bowel contained
within the silo; the
silo is about to be
removed and the
abdominal wall
closed.
Case A. Baby with
a giant
omphalocele.
Case A. Closure of
the giant
omphalocele using
a synthetic patch
Case A.
Tightening the
abdominal wall
closure
Case A. Flank
flaps were used to
close the giant
omphalocele in the
baby whose patch
became infected.
Case A. The flank
wounds were skin
grafted and
closure of the
giant omphalocele
obtained.
Baby with cloacal
exstrophy.
Note the bifid
genitalia in this
baby with cloacal
exstrophy.
Closure of the
bladder exstrophy.
Baby with bladder
exstrophy and
epispadias; note
the appearance of
the bladder
mucosa, indicating
chronic
inflammation.
Another view
demonstrating the
epispadias shown
in the previous
image.
Baby with isolated
epispadias.
Closure of a giant
omphalocele with
an AlloDerm patch
Two months after
implantation:
epithelialization of
the AlloDerm
patch.
Eight months
after implantation:
epithelization is
nearly complete,
but a huge ventral
hernia has
developed
Baby with an
omphalocele.
Complicated gastroschisis.
Following
reduction of
eviscerated viscera
(and lysis of
adhesions,
tubularization of
the viable,
mesenteric portion
of the proximal
jejunum).
MALROTATION
ERRORS OF MIDGUT
DEVELOPMENT AND ROTATION

3/23/2014
 Non-rotation: leaving the major part of the colon on
the left side and the small intestine to the right of the
midline

Prof Khaled H.K. Bahaaeldin

 Incomplete rotation: the coecum is situated in the
sub-hepatic region
 Reversed rotation: the final 180o rotation occurs in a
clockwise manner so that the colon is lying posterior to
the duodenum and the superior mesenteric artery
 Hyper-rotation: the rotation continues to 360o or 450o
so that the coecum rests in the region of the splenic
flexure.

78
 Normal rotation of the human intestine requires
transformation from a simple, straight alimentary tube
into the mature fixed and folded configuration present at
birth.

 The duodenojejunal junction becomes fixed in the left

upper abdomen while the cecum is anchored in the right
lower quadrant.

 The midgut, defined as the portion of the intestine

supplied by the superior mesenteric artery, is thus
suspended from a wide mesenteric base.
EMBRYOLOGY
 The development of the midgut begins with the
differentiation of the primitive intestinal tract into the
foregut, midgut, and hindgut at the fourth week of
gestation.
 The mature alimentary tract and all associated digestive
organs are formed from this primitive tube.
 The most accepted model of midgut maturation involves
four distinct stages:
 (1) herniation.
 (2) rotation.
 (3) retraction.
 (4) fixation
 The intestinal loop can be divided into the cephalic
(duodenojejunal) limb and the caudal (cecocolic) limb,
which rotate separately but in parallel.
NON
ROTATION
INCOMPLETE
ROTATION
PRESENTATION
 The incidence of malrotation has been
estimated at 1 in 6000 live births.
 Up to 75% of patients present during the first month
of life.
 Another 15% will present within the first year.
 Volvulus, intestinal gangrene, and mortality have
been noted regardless of the patient’s age or
chronicity of symptoms.
 Bilious vomiting remains the cardinal sign of
neonatal intestinal obstruction, and malrotation
must be the presumed diagnosis until proven
otherwise.
 Other signs in the neonate include abdominal
pain and distention.
 The inconsolable infant may rapidly deteriorate
as metabolic acidosis quickly advances to
hypovolemic shock.
 Late signs include abdominal wall erythema and
hematemesis or melena from progressive mucosal
ischemia.
 Many other cases will present less dramatically.

 Failure to thrive, gastro esophageal reflux, early

satiety, and mild abdominal discomfort are
routinely reported.
DIAGNOSIS
 Plain X-Ray.
 Doppler study.

 Upper contrast study.

 CT- scan.
TREATMENT:
(LADD’S PROCEDURE)
 The aim of surgery:
 1. Entry into abdominal cavity and evisceration
(open)
 2. Counterclockwise detorsion of the bowel (acute
cases)
 3. Division of Ladd’s cecal bands

 4. Broadening of the small intestine mesentery

 5. Incidental appendectomy

 6. Placement of small bowel along the right

lateral gutter and colon along the left gutter
LAPAROSCOPY

 The laparoscopic treatment for intestinal rotation

anomalies in neonates, infants, and children with
or without midgut volvulus has been proposed by
several authors since van der Zee’s original
report in 1995.
 Reverse trendelenberg position

 4 port technique used.

SUMMARY
 Rotational anomalies are the result of arrest of
normal rotation of the embryonic gut
 Early diagnosis and surgical intervention reduces

morbidity and mortality .

 Must have a high index of suspicion for infants with
bilious vomiting.
 Malrotation, regardless of age and presence of

symptoms is treated surgically with Ladd

procedure
3/23/2014 Prof Khaled H.K. Bahaaeldin
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