This case presentation discusses the diagnosis and management of congenital duodenal atresia in a 35-year-old pregnant patient. Prenatal ultrasound revealed signs of duodenal atresia including polyhydramnios. After being admitted in preterm labor at 34 weeks, the patient delivered a baby boy with duodenal atresia who underwent surgery on postnatal day 4. Duodenal atresia results from failure of embryonic development and is often associated with other anomalies. Prenatal diagnosis allows delivery at a center prepared to care for the newborn.
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
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
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
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
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
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
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.
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.
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.
A finding of polyhdramnios was noted at 35.06 cm
Because of the UTZ findings, patient was referred to Perinatology for comanagement.
Px was first seen at the high risk clinic on 2/13/17. The PE was unremarkable. FH was 34 cm with good FHT
On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
On the 34 5/7 Weeks Gestation, patient came in at the ER with chief complaint of labor pains
The duodenum is the first part of the small intestines. The second part is the jejunum, and the third part is the ileum
Starting at the 6th week of pregnancy or 4th embryonic week, the duodenum starts to develop below the stomach.
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.
Type I Defect-mucosal web with muscular wall(most common)
Type 2 Defect-a short fibrous cord connecting the 2 atretic ends of the duodenum
Type 3 Defect-one in which there is complete separation of the atretic ends