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Duodenal atresia

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Duodenal atresia

  1. 1.  CASE PRESENTATION
  2. 2. Objectives:  Present a case of congenital duodenal atresia  Discuss the embryology, clinical presentation, and diagnosis of the malformation  Give recommendations on obstetrical antenatal and intrapartal management of patients carrying fetuses with such malformations
  3. 3. Introduction  Duodenal atresia is the most common cause of fetal small bowel lumen obstruction  Duodenal atresia is lack of revacuolization during the solid-core stage of embryonic intestinal development
  4. 4. 4  THE CASE
  5. 5. General information  35 years old G3P1(1011)  Single  Filipino  Roman Catholic  Government Employee (Nursing Attendant at Sta. Ana Hospital)  Was referred to High Risk Clinic on 2/14/17 for co- management
  6. 6. Menstrual History  LMP: unsure  AOG: 32 5/7 weeks by EUTZ  EDC: April 6, 2017
  7. 7. OB History  Gravida 3 Para 1 (1011) G1 2003 Full term boy NSD Alive, No complications G2 2015 D&C Early Embryonic Demise (8weeks) G3 Present pregnancy
  8. 8. Prenatal History 1st trimester:  No PNCU  Folic acid 5 mg OD
  9. 9. Prenatal History 2nd trimester:  PNCU: Ospital ng Maynila(2X)  CBC: Hgb:128 Hct:0.37 WBC:15.30 Platelets: 340  UA: pus cells-0-2 RBC-0-1
  10. 10. Prenatal History 2nd trimester:  HBsAg: non-reactive  RPR: non-reactive  HIV:non-reactive
  11. 11. Prenatal History 2nd trimester:  UTZ: Sta. Ana Hospital (11/25/17)  SLIUP, Cephalic, 21 Weeks and 1 day by Fetal Biometry, Anterior placenta, Grade 1, Normohydramnios, SEFW:314g  Patient was given MVTS and FeSO4 with Folic Acid
  12. 12. Prenatal History 3rd trimester:  PNCU: Dr. Jose Fabella Memorial Hospital (at 31 4/7 weeks)  75 g OGTT: FBS-87.28 1st hour-153.65 2nd hour-123.02
  13. 13. UTZ (2/09/17) DJFMH-Women’s Clinic at around 32 Weeks Gestation UTZ result: Dr. Jose Fabella Memorial Hospital
  14. 14. UTZ (2/09/17) DJFMH-Women’s Clinic
  15. 15. UTZ (2/09/17) DJFMH-Women’s Clinic
  16. 16. UTZ (2/09/17) DJFMH-Women’s Clinic
  17. 17. UTZ (2/09/17) DJFMH-Women’s Clinic Congenital Anomaly Scan: Single live intrauterine pregnancy cephalic presentation 31 5/7 weeks by biometry AGA (2080 gms) Anterior high lying placenta grade I Polyhydramnios (35.06) Consider Duodenal Atresia
  18. 18. Past Medical History (-) Asthma (-) diabetes (-) hypertension (-) previous hospitalization
  19. 19. Family History (+) asthma-Maternal aunt (-) diabetes (+) hypertension-Father (-) cancer
  20. 20. Personal and Social History (-) smoker (-) alcoholic beverage drinker (-) allergies to food and drugs
  21. 21. Physical Examination  conscious, coherent, oriented to 3 spheres  BP 110/80 CR 81 RR 19 T 36.9  HEENT: Pink palperbral conjunctivae, anicteric sclerae  Chest and Lungs: SCE, NR, RR  Abdomen: globular FH: 34 cm FHT 130 RLQ  No gross deformities  IE: cervix soft closed, uneffaced, (-) BOW cephalic st-3
  22. 22. 22 WORKING DIAGNOSIS: Gravida 3 Para 1 (1011) Pregnancy Uterine 32 5/7 weeks of gestation by EUTZ Cephalic not in labor Congenital Anomaly (Duodenal Atresia) Polyhydramnios
  23. 23. Recommendation:  Dexamethasone  Fetal well-being studies  Biometry every 2 weeks to monitor growth  BPS with NST weekly  WOF signs of Preterm Labor  Fetal echocardiography  Refer to newborn medicine and pediatric surgeon prior to delivery
  24. 24. Long Term Plan:  To bring pregnancy to term  Karyotyping of the Newborn  Mode of Delivery: Normal Spontaneous Delivery
  25. 25. Emergency Room (34 5/7 weeks gestation)  Chief Complaint: labor pains
  26. 26. Physical Examination  conscious, coherent, oriented  BP 110/70 CR 82 RR 19 T 36.6  HEENT: Pink palperbral conjunctivae, anicteric sclerae  Chest and Lungs: SCE, NR, RR  Abdomen: globular FH: 30 cm FHT 140 RLQ  No gross deformities  IE: cervix soft 5 cm dilated, 50% effaced, (-) BOW, clear AF, cephalic st-3
  27. 27. Admitting Diagnosis (2/28/17) G3P2(1011) Pregnancy Uterine 34 5/7 Weeks by EUTZ Cephalic in Preterm Labor Congenital Anomaly (Duodenal Atresia) Polyhydramnios
  28. 28. Course in the Ward(2/28/17)  Almost 1 hour after admission, patient delivered via Normal Spontaneous Delivery to an alive preterm boy 35 Weeks by BS (2160g, 48 cm) AS 8,9  Baby was admitted at the NICU with the ff. diagnosis: t/c Trisomy 21, Intestinal Obstruction 2°to Duodenal Atresia, Congenital Heart Disease(PDA, stretched PFO, TR mild, MR mild)
  29. 29. Course in the Ward(3/2/17)  On the 4th HD, baby underwent duodeno- duodenostomy
  30. 30. 30  DISCUSSION
  31. 31. Duodenal Atresia  Congenital birth defect wherein the 1st part of the small intestines, known as the duodenum fails to develop  Duodenal atresias occur in approximately 1/5,000- 10,000 live births  Incidence: associated with Trisomy 21 in 30-50% :isolated anomaly in 30-52% of cases
  32. 32. Duodenal Atresia  The exact etiology is still unknown  Equal male-to-female predisposition  No racial predilection  Recurrence is not known to be increased
  33. 33. Duodenal Atresia Nuchal Translucency Trisomy 21 33
  34. 34. Duodenal Atresia Double-bubble sign Polyhydramnios 34
  35. 35. Embryogenesis of Duodenal Atresia
  36. 36. Embryogenesis of Duodenal Atresia
  37. 37. Embryogenesis of Duodenal Atresia
  38. 38. Embryogenesis of Duodenal Atresia
  39. 39. Embryogenesis of Duodenal Atresia- Type 1 Defect
  40. 40. Embryogenesis of Duodenal Atresia- Type 2 Defect
  41. 41. Embryogenesis of Duodenal Atresia- Type 3 Defect
  42. 42. 42 Associated anomalies: : Trisomy 21 (30-50%) : Polyhydramnios : Congenital heart defects(ASD and VSD) : Malrotation : Meconium ileus and peritonitis : Volvulus : Annular pancreas : Esophageal atresia : Genitourinary malformations : Anorectal atresia : Ventral wall defects and skeletal defects 1 Creasy and Resnik’s Maternal Fetal Medicine
  43. 43. 43 Antenatal Monitoring  Congenital Anatomy Scan  Amniocentesis for karyotyping, cystic fibrosis status  Fetal Echocardiography for cardiac malformations  MRI to determine level of obstruction  Fetal NST and Biophysical profile (or both) in the 3rd trimester  Newborn medicine and Pediatric surgery consult
  44. 44. Prognosis:  Overall prognosis depends on the presence of associated anomalies particularly aneuploidy  Survival data improved from 60%(1960s-1970s) to 90%- 100%(1990s-2000s)  Chronic total parenteral nutrition may lead to cholestasis and subsequent liver damage Creasy and Resnik’s Maternal Fetal Medicine
  45. 45. 45 Obstetric Management  Mode of delivery:  Delivery in a tertiary care hospital is recommended  Therapeutic amnioreduction may reduce the risk of preterm labor, cord prolapse, and abruption with rupture of membranes  Cesarean delivery is reserved for obstetrics indications 1 Creasy and Resnik’s Maternal Fetal Medicine
  46. 46. 46 Summary/Conclusion:  Prenatal diagnosis provides the opportunity to plan the delivery of a baby at a center where immediate and definitive care can be provided.  Pediatric surgeon, neonatologist should be readily on standby once the baby is delivered  Timely diagnosis and appropriate interventions may lead to a favourable outcome
  47. 47.  HAVE AN AWESOME DAY!

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