Transcript Haddow JE, Palomaki GE, Holman MS. (1993) Young maternal age

blocketch.crisostomo.dejoras.delmundo.delossantos.
History of Present Illness
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Patient was born 10/30/09, full term, 37 3/7
weeks by LMP to a 23 y/o G2P0 (0-0-1-0)
mother, at home assisted by a midwife.
No known maternal illness during
pregnancy.
Regular monthly prenatal consults c/o local
health center ; no ultrasound studies done
(+) intake of folic acid, ferrous sulfate,
multivitamins
(-)
smoking/alcohol/drugs/exposure
to
radiation
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At birth:
 Good cry and activity
 (+) eviscerated bowels at umbilical area
 (-) fever/cyanosis/apnea/vomiting
 (-) other gross deformities
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Patient was immediately brought to
Trece Martires Hospital but was advised
transfer to PGH for further evaluation
and management.
Review of Systems
(-) fever/cough/colds
 (-) jaundice
 (-) cyanosis/apnea
 (-) vomiting
 (-) hematemesis/hematochezia/melena
 (+) urine output
 (-) seizure
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Birth and Maternal History
As above
 G1 2007 spontaneous abortion, noninduced, no known maternal illness at
the time
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Family Medical History
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(+) Polydactyly (hands and toes) – mother
(-) Hypertension
(-) DM
(-) Pulmonary TB
(-) Cancer
(-) Bronchial asthma
(-) Allergies
(-) Similar condition
(-) Other congenital defects
Immunization History
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None
Developmental History
(+) Moro reflex
 (+) Rooting reflex
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Good cry, fair activity
Good pulses, HR 140-150, RR 40-50, T 36.7
Pink conjunctivae, anicteric sclerae,
(-) nasoaural discharge,
(-) tonsillophayngeal congestion,
(-) anterior neck mass,
(-) cervical lymphadenopathy,
(-) neck vein engorgement,
trachea midline, (-) carotid bruits
Equal chest expansion, (-) retractions,
clear breath sounds, (-) rales, (-) wheezes
Adynamic precordium, (-) precordial bulge,
(-) heaves, (-) thrills, distinct heart sounds, normal
rate, regular rhythm, (-) murmurs
Globular abdomen,
(+) eviscerated erythematous bowels
 Grossly female genitalia
 Full and equal pulses, pink nail beds,
CRT <2sec, (-) cyanosis, (-) edema,
(-) clubbing, (-) jaundice
 Neuro: awake, alert, pupils 2 mm EBRTL, full
EOMs, (+) corneal reflex, (-) facial asymmetry,
(+) gag reflex, uvula midline, tongue midline,
spontaneous movement of extremities,
withdraws to pain, DTRs +2, (+) Moro reflex,
(+) rooting reflex, (+) Babinski bilateral,
(-) clonus, (-) nuchal rigidity, (-) nystagmus
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Point of comparison
Incidence
Peritoneal sac
GASTROSCHISIS
OMPHALOCOELE
• 1 in 5,000 live births • Small – 1 in 5,000
• Occurs more often
• Large – 1 in 10,000
in babies born to
younger mothers
(usually under 20
years of age)
Absent
Present
Location of defect
Right Periumbilical
Base of umbilical cord
Contents
Edematous bowels
Abdominal viscera
• Low
• 10% intestinal
atresia
• High
• Associated w/
other congenital
anomalies
Associated
anomalies
GASTROSCHISIS
OMPHALOCOELE
GASTROSCHISIS
Full term 37 weeks by pediatric aging,
2040 grams small for gestational age,
Cephalic presentation,
Delivered via spontaneous vaginal delivery,
Live baby girl, APGAR 9-9,
Non-institutional delivery
FLUIDS/FEEDING
• 1st HD: At the ER, initially given IV bolus
20 cc/kg pLR then maintained on D10W
at 17 cc/hr (TFI = FM + 100%).
• 2nd HD: In the wards, IVF revised to PPN
D10-Na3-K2-Ca300-AA0.5 at 12 cc/hr
(TFI = FM + 50%)
• 3rd HD: IVF shifted to D10IMB-Ca300
(TFI = FM + 50%) at 12-13 cc/hr.
RESPIRATORY
• 1st HD: Intubated ET3L8, MV FiO2 100%
PIP 18 PEEP 5 RR 40 IT 0.5
• 3rd HD: Patient being weaned off MV.
FiO2 decreased to 60%.
• ABG showed pH 7.497, pCO2 20.9, pO2
225.9, HCO3 19.6, Beb -5.7, O2st
99.8%.
• 4th HD: FiO2 30 PIP 16 PEEP 5 RR 33 IT
0.5
INFECTIOUS
• 1st HD: At the ER, started on Meropenem (40)
80 mg IV q12 and Amikacin (15) 30 mg IV OD.
• 2nd HD: Shifted to Cefuroxime (100) 70 mg IV
q8 and Metronidazole (15) 30 mg IV OD.
CARDIAC/CIRCULATORY
HEMATOLOGIC
CBC
(10/31) Hgb 194 Hct 0.68 Plt 158 WBC 14.6 (N
0.793, L 0.176)
(11/01) Hgb 186 Hct 0.553 Plt 276 WBC 12.6
(N 0.516, L 0.307)
METABOLIC
Blood chemistry
(10/31) BUN 4.67 Crea 80 Na 136 K 5.1 Cl 103
(11/01) Crea 95 Ca 1.89 Na 147 K 5.1 Cl 118
(11/ 02)BUN 3.94 Crea 45
ONCOLOGIC
NEUROLOGIC
DEVELOPMENTAL
Surgical
For ‘E’ closure of abdominal wall defect
 s/p silo bag closure (10/31/09 Rimando)
 s/p fascial closure (11/03/09 Rimando)
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Diagnostics
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CBC (10/31)
Hgb 194 Hct 0.68
Plt 158 WBC 14.6
(N 0.793, L 0.176)
(11/01)
Hgb 186 Hct 0.553
Plt 276 WBC 12.6
(N 0.516, L 0.307)
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Blood type O+
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Blood chemistry (10/31)
BUN 4.67 Crea 80
Na 136 K 5.1 Cl 103
(11/01)
Crea 95 Ca 1.89
Na 147 K 5.1 Cl 118
(11/02)
BUN 3.94 Crea 45
Definition
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A herniation of abdominal contents
through a paramedian full-thickness
abdominal wall fusion defect usually to
the right of the umbilical cord.
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A gastroschisis usually contains small
bowel and has no surrounding
membrane.
Embryology
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Human embryo initially has 2 layers that
looks like a disc. As it acquires a third cell
layer, it becomes cylindrical; it then
elongates and invaginates over the
umbilical ring. The body folds (cephalic,
caudal, lateral) centrally fuse, where the
amnion invests the yolk sac.
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Defective development at this critical
location results in a spectrum of abdominal
wall defects.
Pathophysiology
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Theories:
1.
2.
3.
4.
5.
Failure of mesoderm to form in the body wall
Rupture of the amnion around the umbilical ring with
subsequent herniation of bowel
Abnormal involution of the right umbilical vein leading to
weakening of the body wall and gut herniation
Disruption of the right vitelline (yolk sac) artery with
subsequent body wall damage and gut herniation
Failure of the yolk sac and related vitelline structures to
be incorporated into the umbilical stalk providing a
connection through the ventral wall and acts as the
egress point for the gut
Maternal Risk Factors
1.
Maternal Young Age
2.
Smoking History
3.
Maternal Infection
4.
Recreational Drug Use
5.
Maternal Medications
1. Young Maternal Age
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Pregnancies younger than 20 years of
age were at 7.3 times greater odds for
being affected with gastroschisis than
pregnancies in women aged 25 or older.
For pregnant women aged 20 to 24
years, the odds were 1.9 times greater.
Haddow JE, Palomaki GE, Holman MS. (1993) Young
maternal age and smoking during pregnancy as risk factors
for gastroschisis. Teratology 47:225–8
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Evidence from the present study and
other
published
studies
clearly
establishes a greater risk for fetal
gastroschisis in pregnant women
younger than age 20, even after
adjustment for smoking status.
Haddow JE, Palomaki GE, Holman MS. (1993) Young
maternal age and smoking during pregnancy as risk factors
for gastroschisis. Teratology 47:225–8
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In a case-control surveillance program of births
defects (76 gastroschisis cases versus 2581
malformed controls), Werler et al (1992) found a
strong inverse association between maternal age
and gastroschisis. Compared with women 30
years or older, the relative risks of gastroschisis for
25–29, 20–24 and younger than 20-year-old
women were 1.7 (95% CI: 0.7, 4.1), 5.4 (95%
CI: 2.6, 11) and 16 (95% CI: 8.1, 30)
.
Werler MM, Mitchell AA, Shapiro S. (1992) Demographic, reproductive, medical, and
environmental factors in relation to gastroschisis. Teratology 45:353–60
2. Smoking History
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Pregnant
women
who
smoked
cigarettes were at 2.1 times greater
odds than non-smokers.
Haddow JE, Palomaki GE, Holman MS. (1993) Young
maternal age and smoking during pregnancy as risk factors
for gastroschisis. Teratology 47:225–8
3. Maternal Infections
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There is a significant association
between self reported urinary tract
infection plus sexually transmitted
infection just before conception and in
early pregnancy and gastroschisis.
Case-control study of self reported genitourinary infections
and risk of gastroschisis: findings from the national birth
defects prevention study, 1997-2003 ML Feldkamp, et al.
BMJ 2008 336: 1420-1423
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Crude odds ratios were:
 2.0 (95% confidence interval 1.6 to 2.6) for sexually
transmitted infection or urinary tract infections
 1.7 (1.0 to 3.0) for sexually transmitted infection only
 1.9 (1.5 to 2.6) for urinary tract infection only
 6.8 (2.6 to 17.5) for sexually transmitted infection
and genitourinary infection
Case-control study of self reported genitourinary infections and risk of
gastroschisis: findings from the national birth defects prevention study,
1997-2003 ML Feldkamp, et al. BMJ 2008 336: 1420-1423
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Urinary tract infections are common during pregnancy,
probably share common risk factors with sexually
transmitted infections and also are more common
among adolescent girls who are sexually active.
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Our finding that the risk was highest for exposure to
both types of infection, particularly among younger
women, suggests a combined role of infection and
early sexual activity.
Case-control study of self reported genitourinary infections and risk of gastroschisis:
findings from the national birth defects prevention study, 1997-2003 ML Feldkamp, et
al. BMJ 2008 336: 1420-1423
4. Recreational Drug Use
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Statistically significant adjusted odds ratios
for gastroschisis were associated with firsttrimester use of:
Any recreational drug (odds ratio (OR) = 2.2,
95% confidence interval (CI): 1.2, 4.3) and
2. Vasoconstrictive recreational drugs (defined as
cocaine, amphetamines, and ecstasy) (OR =
3.3, 95% CI: 1.0, 10.5).
1.
Recreational Drug Use: A Major Risk Factor for Gastroschisis? ES Draper et
al., American Journal of Epidemiology 2008 167(4):485-491
5. Medications
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This retrospective study evaluated the relation between
maternal use of cough/cold/analgesic medications and risks
of gastroschisis.
Drug
Odds Ratio
Aspirin
2.7
Pseudoephedrine
1.8
Acetaminophen
1.5
Pseudoephedrine combined with
Acetaminophen
4.2
Maternal Medication Use and Risks of Gastroschisis and
Small Intestinal Atresia MM Werler, et al.; American Journal
of Epidemiology 2002 Vol. 155, No. 1 : 26-31
Early Detection During Pregnancy
1.
Elevated maternal serum alphafetoprotein levels in 2nd trimester
2.
Evidence on Ultrasonography
3.
Amniocentesis
FETAL ULTRASOUND
•
Bowel protruding from
abdominal wall defect
•
A 2-5 cm right
paramedian
paraumbilical
abdominal wall defect
•
Normal insertion of the
umbilical cord
Goals of Management
1.
Prenatal Monitoring
2.
Delivery
3.
Preoperative Management
4.
Surgery
5.
Fluids/Nutrition
6.
Prevention/Treatment Of Complications
1. Prenatal Monitoring
Daily fetal movement count
 Serial UTZ
 Fetal Non-stress test/Biophysical profile
 3rd trimester – at risk for gastroschisisrelated complications such as bowel
dilatation/inflammation, intestinal
damage, IUGR, oligohydramnios
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2. Delivery
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There was no significant relationship
between mode of delivery and:
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Rate of primary fascial repair
Neonatal sepsis
Pediatric mortality
Time until enteral feeding
Length of hospital stay
Segel SY, Marder SJ, Parry S, et al: Fetal abdominal wall
defects and mode of delivery: A systematic review. Obstet
Gynecol 98(5 Pt 1):867- 873, 2001
3. Preoperative management
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OGT insertion – for gastric decompression
Endotracheal intubation – for respiratory
distress
Minimize and correct fluid, electrolyte, and
heat losses
Place under a radiant heater
Cover exposed bowels
Foley catheter insertion – for urine output
monitoring
IV BOLUS: 20 cc/kg pLR to replace
significant ongoing fluid losses
4. Surgery
Primary repair: reduction of the bowel
and complete abdominal wall closure in
one operation immediately after birth
 Staged repair:
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 Silo – placed around the herniated bowel,
which is then reduced daily at the bedside
until the abdominal contents are level with
the skin.
 Final fascial closure
5. Fluids/Nutrition
Maintenance fluids: FM + 50-100 %
 Nutrition: A central venous line is placed
intraoperatively to provide parenteral
nutrition, thereby minimizing catabolic
protein loss during the period of GI
dysfunction which may take up to 3
months.
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6. Prevention/Treatment of
Complications
Infection: Broad-spectrum antibiotics are
administered to prevent contamination of
the peritoneal cavity.
 Hemodynamic/circulatory compromise :
ensure adequate hydration, monitor renal
status
 Respiratory distress : ensure adequate
ventilation
 Watch
out for hepatotoxicity from
prolonged parenteral nutrition
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Factors
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Severity of associated problems
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Prematurity
Intestinal atresia
Intestinal inflammatory dysfunction
Short gut syndrome
Hemodynamic stability
 Pulmonary growth and development
 GI maturity
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Thank you! 