A baby girl with eviscerated bowels
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blocketch.crisostomo.dejoras.delmundo.delossantos. A BABY GIRL WITH EVISCERATED BOWELS. clinical history. History of Present Illness. 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.

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A BABY GIRL WITH EVISCERATED BOWELS

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blocketch.crisostomo.dejoras.delmundo.delossantos.

A BABY GIRL WITH EVISCERATED BOWELS


clinical history


History of Present Illness

  • 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


  • At birth:

    • Good cry and activity

    • (+) eviscerated bowels at umbilical area

    • (-) fever/cyanosis/apnea/vomiting

    • (-) other gross deformities

  • 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


Birth and Maternal History

  • As above

  • G1 2007 spontaneous abortion, non-induced, no known maternal illness at the time


Family Medical History

  • (+) Polydactyly (hands and toes) – mother

  • (-) Hypertension

  • (-) DM

  • (-) Pulmonary TB

  • (-) Cancer

  • (-) Bronchial asthma

  • (-) Allergies

  • (-) Similar condition

  • (-) Other congenital defects


Immunization History

  • None


Developmental History

  • (+) Moro reflex

  • (+) Rooting reflex


physical examination


  • Good cry, fair activity

  • Good pulses, HR 140-150, RR 40-50, T 36.7

  • Pink conjunctivae, anictericsclerae,

    (-) 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

  • Adynamicprecordium, (-) 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


differentials


GASTROSCHISIS

OMPHALOCOELE


present working impression


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


course in the ER/wards


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)


Diagnostics

  • 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)

  • Blood type O+

  • 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


CASE DISCUSSION


what is gastroschisis?


Definition

  • A herniation of abdominal contents through a paramedian full-thickness abdominal wall fusion defect usually to the right of the umbilical cord.

  • A gastroschisis usually contains small bowel and has no surrounding membrane.


Embryology

  • 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.

  • Defective development at this critical location results in a spectrum of abdominal wall defects.


Pathophysiology

  • Theories:

    • 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


diagnosis/evaluation


Maternal Risk Factors

  • Maternal Young Age

  • Smoking History

  • Maternal Infection

  • Recreational Drug Use

  • Maternal Medications


1. Young Maternal Age

  • 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


  • 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


  • Ina case-control surveillance program of births defects (76 gastroschisiscases versus 2581 malformed controls), Werler et al (1992) founda strong inverse association between maternal age and gastroschisis.Compared with women 30 years or older, the relative risks ofgastroschisis for 25–29, 20–24 and younger than20-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

  • 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

  • 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


  • 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


  • 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.

  • 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

  • Statisticallysignificant adjusted odds ratios for gastroschisis were associatedwith first-trimester use of:

    • Any recreational drug (odds ratio(OR) = 2.2, 95% confidence interval (CI): 1.2, 4.3) and 

    • Vasoconstrictiverecreational drugs (defined as cocaine, amphetamines, and ecstasy)(OR = 3.3, 95% CI: 1.0, 10.5).

      Recreational Drug Use: A Major Risk Factor for Gastroschisis? ES Draper et al., American Journal of Epidemiology 2008 167(4):485-491


5. Medications

  • This retrospective study evaluated the relation between maternaluse of cough/cold/analgesic medications and risks of gastroschisis.

    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

  • Elevated maternal serum alpha-fetoprotein levels in 2nd trimester

  • Evidence on Ultrasonography

  • 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


management


Goals of Management

  • Prenatal Monitoring

  • Delivery

  • Preoperative Management

  • Surgery

  • Fluids/Nutrition

  • Prevention/Treatment Of Complications


1. Prenatal Monitoring

  • Daily fetal movement count

  • Serial UTZ

  • Fetal Non-stress test/Biophysical profile

  • 3rd trimester – at risk for gastroschisis-related complications such as bowel dilatation/inflammation, intestinal damage, IUGR, oligohydramnios


2. Delivery

  • There was no significant relationship between mode of delivery and:

    • 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. ObstetGynecol 98(5 Pt 1):867- 873, 2001


3. Preoperative management

  • 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:

    • 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.


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


prognosis


Factors

  • Severity of associated problems

    • Prematurity

    • Intestinal atresia

    • Intestinal inflammatory dysfunction

    • Short gut syndrome

  • Hemodynamic stability

  • Pulmonary growth and development

  • GI maturity


Thank you! 


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