Transcript Gastroschisis

Case Management Conference
November 11, 2009
 This
is the case of a preterm baby boy
from Sapampalay, Bulacan, delivered at
PGH LRDR via low segment cesarean
section to an 18 y/o G1P0 mother,
presenting with protrusion of the
intestines.

On the day of delivery, at 29 1/7 weeks AOG by
amenorrhea, the 18 y/o G1P0 mother of the
patient was referred to PGH by a local health
center for control of preterm labor. Ultrasound
was done revealing gastroschisis.
Dexamethasone 1 dose IM was given to the
mother and a live baby boy was delivered via
low segment cesarean section for gastroschisis,
35 weeks by pediatric aging, 1550 grams, SGA.
Tactile stimulation, suctioning, and
thermoregulation were done and APGAR scores
were noted to be 9,9.
 No
family history of asthma, allergy, DM,
hypertension, PTB, cancer
 No family history of any congenital
anomalies
 The
patient was born to an 18 y/o G1P0
mother, who is single, unemployed, High
School undergraduate, with no vices nor
pollution exposures.
 The father of the patient is a 19 y/o
unemployed, elementary graduate,
heavy smoker, and occasional alcoholic
beverage drinker.
 The
mother of the patient had her first
coitus when she was 17 y/o with 1 NPSP.
She had no comorbidities nor illnesses
during gestation. She had regular PNCU
since 3 months AOG c/o a local health
center. She took multivitamins and FeSO4
as advised to her. She denies intake of
any antibiotic and teratogens nor
exposure to radiation.
 Patient
was received stable, acrocyanotic
with good cry, good activity, good muscle
tone
 HR 140, RR 40, afebrile
 Anicteric sclerae, pink conjunctivae, formed
nose, ears, lips, tongue and palate
 Equal chest expansion, no retractions, clear
breath sounds
 Adynamic precordium, no precordial bulge,
distinct heart sounds, normal rate and
regular rhythm, no murmurs
 Abdomen
with ~7x8 cmwall defect with
erythematous and edematous bowels
protruding lateral to the umbilical cord
without sac
 Grossly male genitalia with descended
testes
 Full and equal pulses, acrocyanotic, no
clubbing, no edema
Preterm, 35 weeks by pediatric aging, 1550
grams, small for gestational age, cephalic
presentation, delivered via primary low
segment cesarean section for
gastroschisis, live baby boy, APGAR 9,9
Gastroschisis
Rule out Sepsis
Omphalocoele
 Congenital herniation of abdominal
contents at the umbilicus into the
umbilical cord.
 Amniotic sac (amnion & peritoneum) is
always present but it may have ruptured
at or before birth exposing the contents.
Gastroschisis
 Full thickness abdominal wall defect
situated almost always to the right of the
umbilicus without a covering membrane.
A bridge of skin separates it from the
umbilicus.
Umbilical Cord Hernia
 Congenital herniation of abdominal
contents at the umbilicus into the
umbilical cord with less than 4 cm in size.
 Amniotic sac is present but it may also
have ruptured at or before birth
exposing the contents.
Prune Belly Syndrome
 Congenital deficiency of abdominal
musculature, urinary tract dilatation and
cryptorchidism
First Day of Life
 Diagnostics including CBC, blood CS, blood typing
and babygram APL were done.
 Started on Meropenem (40) and Amikacin (15).
Patient was placed on NPO with IVF D10W at
9.5cc/hr (TFI 150)
 Patient was brought to the OR for SILO closure of
gastroschisis and right IJ cutdown. Findings at
operation include edematous, erythematous bowels,
abdominal defect extending superiorly and
inferiorly.
 Patient was intubated ET 3, level 7.5, maintained on
MV settings: FiO2 60%, PIP 20, PEEP 5, RR 40, IT 0.4.
Second Day of Life
 Patient was started on albumin (1) 25% to
run at 8cc for 24 hours with furosemide
while on albumin.
 Noted to have no urine output. Inotropes
Dopa (5) and Dobu (8) were started to
enhance renal perfusion. TFI was
increased to 200 cc/kg.
Third Day of Life
 PPN was started at D10 Na3 K2 Ca400
AA0.5.
 Weaning from mechanical ventilator was
started. Patient was able to tolerate
weaning and was eventually extubated.
8th Day of Life
 Awaiting second stage closure.
 Patient was started on Fluconazole (12)
and Ciprofloxacin (20).
Date
11/03
11/09
WBC
15.93
27.47
RBC
5.27
4.09
Hgb
186
139
Hct
0.526
0.400
MCV
99.8
97.8
MCH
35.3
34.0
MCHC
354
348
RDW
20.3
18.7
Platelet
169
14
Neutrophils
0.526
0.683
Lymphocytes
0.276
0.201
Monocytes
0.187
0.107
Eosinophils
0.006
0.003
Basophils
0.005
0.006
Date
11/3
11/5
11/6
11/9
Ca
1.53
1.87
1.91
2.09
Na
129
136
139
135
K
3.9
4.6
5.3
4.6
Cl
101
101
106
103
Plasma K
Blood CS
Blood Type
3.4
No growth after 2 days
A+
Date
11/3
11/4
PH
7.346
7.433
PCO2
29.9
21.0
PO2
172.5
118.1
HCO3
18.2
17.8
Beb
-7.8
-7.9
O2sat
99.3%
98.5%
2D echo
Normal echo cardiographic findings
& doppler studies
Transabdominal Single intrauterine live pregnancy of about
30 weeks one day sonal AOG, cephalic
UTZ (11/2/09)
presentation, anterior placenta, grade 2
maturity, normohydramnios
Incidantal finding of Gastroschisis, Eagle
barette syndrome
Preterm, 35 weeks by pediatric aging, 1550
grams, small for gestational age, cephalic
presentation, delivered via primary low
segment cesarean section for
gastroschisis, live baby boy, APGAR 9,9
Gastroschisis
Rule out Sepsis

Herniation of bowel loops & other abdominal
organs through a defect in the abdominal wall
w/ no associated covering /sac

Defect just to the right of the umbilical cord






strongly associated with very low maternal age
More frequent in primipara
Associated w/ low social economic status
Poor maternal education
Drug abuse (alcohol,cigarettes,cocaine)
may have vasoreactive effects during embryo
development
baseline birth prevalence is 1 in 50,000 births and has
increased since between 10- and 20-fold.



occurs in approximately two infants per 10,000 live
births in the United States and internationally.
Mothers below the age of 20 are birth to affected
babies.
Gastroschisis occurs slightly more often in males than
in females.




Maternal intake of aspirin or ibuprofen causes 4 to 5
times increased risk for gastroschisis.
Both medications are inhibitors of the cyclooxygenase
enzyme and influence blood flow to the fetus.
Decongestants, especially pseudoephedrine and
phenylpropanolamine,double the risk (cause
constriction of blood vessels and decrease blood flow
to the fetus)
Illness and fever have no association with the
development of gastroschisis.
 During the 4th week of development, the lateral
body folds move ventrally and fuse in the midline to
form the anterior body wall.
 Incomplete fusion defect that allows abdominal
viscera to protrude through the abdominal wall.
 The bowel typically herniates through the rectus
muscle, lying to the right of the umbilicus.
A
mother carrying a fetus with gastroschisis
will not experience any unusual signs or
symptoms in early pregnancy.
 suspected
when maternal serum screening
reveals an elevated AFP level
(not indicative of gastroschisis specifically, but
this does alert for a detailed ultrasound)

Ultrasonogram
 the primary method of diagnosis (noninvasive, rapid, and
allows for real-time fetal monitoring)
 May reveal:
-loops of fetal intestines floating exposed to amniotic fluid w/
or w/o other organs
-signs of intestinal obstruction
-defect in the middle of the abdominal wall to the right of a
normal umbilical cord.
Diagnose gastroschisis before 20 weeks of gestation

Transvaginal Sonogram
diagnosis has been made as early as 12 weeks of gestation.

AFP levels
 elevated maternal serum AFP level is present in approximately
75 to 80% of cases
Upon birth

radiographs and bowel contrast studies may be
necessary to diagnose intestinal complications.