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
CASE PRESENTATION
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
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

THE CASE
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
Menstrual History
 LMP: unsure
 AOG: 32 5/7 weeks by EUTZ
 EDC: April 6, 2017
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
Prenatal History
1st trimester:
 No PNCU
 Folic acid 5 mg OD
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
Prenatal History
2nd trimester:
 HBsAg: non-reactive
 RPR: non-reactive
 HIV:non-reactive
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
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
UTZ (2/09/17) DJFMH-Women’s Clinic
at around 32 Weeks Gestation
UTZ result:
Dr. Jose Fabella Memorial Hospital
UTZ (2/09/17) DJFMH-Women’s
Clinic
UTZ (2/09/17) DJFMH-Women’s
Clinic
UTZ (2/09/17) DJFMH-Women’s Clinic
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
Past Medical History
(-) Asthma (-) diabetes (-) hypertension
(-) previous hospitalization
Family History
(+) asthma-Maternal aunt
(-) diabetes
(+) hypertension-Father
(-) cancer
Personal and Social History
(-) smoker
(-) alcoholic beverage drinker
(-) allergies to food and drugs
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
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
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
Long Term Plan:
 To bring pregnancy to term
 Karyotyping of the Newborn
 Mode of Delivery: Normal Spontaneous Delivery
Emergency Room (34 5/7 weeks
gestation)
 Chief Complaint:
labor pains
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
Admitting Diagnosis (2/28/17)
G3P2(1011)
Pregnancy Uterine 34 5/7 Weeks by EUTZ
Cephalic in Preterm Labor
Congenital Anomaly (Duodenal Atresia)
Polyhydramnios
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)
Course in the Ward(3/2/17)
 On the 4th HD, baby underwent duodeno-
duodenostomy
30

DISCUSSION
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
Duodenal Atresia
 The exact etiology is still unknown
 Equal male-to-female predisposition
 No racial predilection
 Recurrence is not known to be increased
Duodenal Atresia
Nuchal Translucency Trisomy 21
33
Duodenal Atresia
Double-bubble sign Polyhydramnios
34
Embryogenesis of Duodenal Atresia
Embryogenesis of Duodenal Atresia
Embryogenesis of Duodenal Atresia
Embryogenesis of Duodenal Atresia
Embryogenesis of Duodenal Atresia-
Type 1 Defect
Embryogenesis of Duodenal Atresia-
Type 2 Defect
Embryogenesis of Duodenal Atresia-
Type 3 Defect
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
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
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
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
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

HAVE AN AWESOME DAY!

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Duodenal atresia

  • 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. 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
  • 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. Menstrual History  LMP: unsure  AOG: 32 5/7 weeks by EUTZ  EDC: April 6, 2017
  • 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. Prenatal History 1st trimester:  No PNCU  Folic acid 5 mg OD
  • 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. Prenatal History 2nd trimester:  HBsAg: non-reactive  RPR: non-reactive  HIV:non-reactive
  • 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. 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. UTZ (2/09/17) DJFMH-Women’s Clinic at around 32 Weeks Gestation UTZ result: Dr. Jose Fabella Memorial Hospital
  • 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. Past Medical History (-) Asthma (-) diabetes (-) hypertension (-) previous hospitalization
  • 19. Family History (+) asthma-Maternal aunt (-) diabetes (+) hypertension-Father (-) cancer
  • 20. Personal and Social History (-) smoker (-) alcoholic beverage drinker (-) allergies to food and drugs
  • 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 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. 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. Long Term Plan:  To bring pregnancy to term  Karyotyping of the Newborn  Mode of Delivery: Normal Spontaneous Delivery
  • 25. Emergency Room (34 5/7 weeks gestation)  Chief Complaint: labor pains
  • 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. 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. 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. Course in the Ward(3/2/17)  On the 4th HD, baby underwent duodeno- duodenostomy
  • 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. Duodenal Atresia  The exact etiology is still unknown  Equal male-to-female predisposition  No racial predilection  Recurrence is not known to be increased
  • 39. Embryogenesis of Duodenal Atresia- Type 1 Defect
  • 40. Embryogenesis of Duodenal Atresia- Type 2 Defect
  • 41. Embryogenesis of Duodenal Atresia- Type 3 Defect
  • 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 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. 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 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 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

Notas do Editor

  1. This is the sagittal view of the fetus. This is the fetal spine, the thoracic cavity with the heart. The diaphragm was noted to be intact. This is the ub. We noted 2 anechoic, cystic structures within the abdominal cavity.
  2. Adjacent to the gastric bubble is a cystic structure w/c on further exam showed a connection to the gastric bubble. At this time we are already considering duodenal atresia with the classic double-bubble sign. We then proceeded to do full CAS to look for other congenital anomalies, however, no other anomalies were noted.
  3. Adjacent to the gastric bubble is a cystic structure w/c on further exam showed a connection to the gastric bubble. At this time we are already considering duodenal atresia with the classic double-bubble sign. We then proceeded to do full CAS to look for other congenital anomalies, however, no other anomalies were noted.
  4. A finding of polyhdramnios was noted at 35.06 cm
  5. Because of the UTZ findings, patient was referred to Perinatology for comanagement.
  6. Px was first seen at the high risk clinic on 2/13/17. The PE was unremarkable. FH was 34 cm with good FHT
  7. On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
  8. On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
  9. On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
  10. The duodenum is the first part of the small intestines. The second part is the jejunum, and the third part is the ileum
  11. Starting at the 6th week of pregnancy or 4th embryonic week, the duodenum starts to develop below the stomach.
  12. This picture portrays the formation of the definitive gut lumen. Starting at the 6th week of pregnancy or 4th embryonic week, the duodenum starts to develop below the stomach. Proliferation of the endodermal lining completely occludes the gut tube during the sixth week. Recanalization is completed by week 9. Incomplete or abnormal recanalization may result in duplication of the lumen or stenosis of the gut tube.
  13. Type I Defect-mucosal web with muscular wall(most common)
  14. Type 2 Defect-a short fibrous cord connecting the 2 atretic ends of the duodenum
  15. Type 3 Defect-one in which there is complete separation of the atretic ends