Volume 69, Number 3 OBSTETRICAL AND GYNECOLOGICAL SURVEY Copyright © 2014 by Lippincott Williams & Wilkins
CME REVIEW ARTICLE
8
CHIEF EDITOR’S NOTE: This article is part of a series of continuing education activities in this Journal through which a total of 36 AMA PRA Category 1 Credits TM can be earned in 2014. Instructions for how CME credits can be earned appear on the last page of the Table of Contents.
Umbilical Artery Aneurysm: A Case Report, Literature Review, and Management Recommendations Pooja Doehrman, MD,* Brenna J. Derksen, BS,† Jordan H. Perlow, MD,‡ William H. Clewell, MD,§ and Harris J. Finberg, MD} *Obstetrics and Gynecology Resident, Department of Obstetrics and Gynecology, Banner Good Samaritan Medical Center, Phoenix, AZ; †Medical Student, University of Arizona College of Medicine, Phoenix, AZ; ‡Director, Maternal-Fetal Medicine, Banner Good Samaritan Medical Center, Phoenix, AZ; §Maternal-Fetal Medicine and Director of Obstetric Ultrasound, Banner Good Samaritan Medical Center, and Clinical Professor of Obstetrics and Gynecology, University of Arizona College of Medicine, Phoenix, AZ; }Diagnosic Radiology, Banner Good Samaritan Medical Center, Phoenix, AZ Background: Umbilical artery aneurysm is a rare and often lethal condition frequently associated with fetal anomalies, fetal demise, and neonatal complications. Case: We report a case of umbilical artery aneurysm discovered at 21 weeks 2 days of gestation in a fetus of normal karyotype. Maternal hospitalization occurred at 28 weeks for antenatal testing, betamethasone administration, and monitoring for expansion of the aneurysm. Delivery of a live neonate by repeat cesarean delivery was performed at 32 weeks 2 days. Pathology confirmed a 3-vessel cord with an umbilical artery aneurysm. Neonatal course was complicated by respiratory distress of the newborn, hyperbilirubinemia, anemia, difficulty feeding, and cardiac defects. The newborn was discharged from the neonatal intensive care unit on day of life 19. Conclusions: Umbilical artery aneurysm is highly associated with fetal complications including trisomy 18, single umbilical artery, cardiac anomalies, and intrauterine fetal demise. A normal karyotype, antenatal monitoring, and early delivery have been suggested to impact the likeliness of survival. Antenatal management strategies include consideration of nonstress testing 3 times daily, serial ultrasound assessments, testing to identify intrauterine growth restriction, and delivery by planned cesarean delivery between 32 and 34 weeks. We recommend that patients be counseled on the high risks associated with umbilical artery aneurysm and be included in discussions regarding antenatal management and delivery planning. Target Audience: Obstetricians and gynecologists, family physicians Learning Objectives: After completing this CME activity, physicians should be better able to diagnose umbilical artery aneurysm using ultrasound and manage pregnant women whose fetuses have umbilical artery aneurysm.
All authors and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interests in, any commercial organizations pertaining to this educational activity. Correspondence requests to: Jordan H. Perlow, MD, Department of Obstetrics and Gynecology and Maternal-Fetal Medicine, Banner Good Samaritan Medical Center, 1111 E McDowell Rd, Phoenix, AZ 85006. E-mail: [email protected].
A 30-year-old Native American, G3P1011, at 21 weeks 2 days of gestation was found on routine second trimester obstetrical ultrasound to have a hypoechoic mass of the fetal umbilical cord measuring 2.7 4.0 3.4 cm. The patient was referred to a maternal fetal medicine group for further evaluation of the mass. At 26 weeks 6 days, a detailed level II ultrasound was performed for assessment of the
www.obgynsurvey.com | 159
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
160
Obstetrical and Gynecological Survey
FIG. 1. The umbilicus is to the left within the image, with 2 umbilical arteries straddling the fetal bladder. The cord widens 3 cm from the umbilicus, with the umbilical arteries splayed around a masslike region of mixed echogenicity.
fetal anatomy; it demonstrated a 3 5-cm enlargement of the umbilical cord beginning approximately 3 cm from the cord insertion at the umbilicus. During the ultrasound assessment, color Doppler flow demonstrated a swirling pattern of blood within the umbilical aneurysm and normal systolic to diastolic ratio (Figs. 1–3). The outer third of the cord defect at the level of the aneurysm appeared solid with echogenic heterogeneous material absent of vascularity. This solid material was hypothesized to be either a mural thrombus or focal thickening of Wharton jelly. Ultrasound examination raised the possibility of other abnormalities including membranous ventricular septal defect, overriding aorta, and calcifications adjacent to the umbilical vein at the level of hepatic
FIG. 3. Umbilical artery aneurysm of a neonate 3 cm from the cord insertion at the umbilicus on DOL 1.
insertion. A Doppler waveform obtained of the umbilical artery near the aneurysm demonstrated a normal systolic to diastolic ratio of 2.6. The patient was counseled regarding the association between umbilical artery aneurysm and aneu ploidy, specifically trisomy 18.1 She was also counseled on the significantly increased risk for intrauterine fetal demise (IUFD).2–8 After counseling the patient in regard to the variety of management options, the patient was hospitalized at 28 weeks 3 days. Inpatient management consisted of daily fetal movement assessments, nonstress testing 3 times daily, weekly fluid measurements, weekly umbilical artery Doppler assessments, and growth ultrasounds every 3 weeks. A fetal echocardiogram was performed at
FIG. 2. A, The cord mass has a well-circumscribed cystic area and an adjacent solid area of somewhat heterogeneous echogenicity, consistent with thrombus abutting the liquid blood portion of the aneurysm. B, Color flow mapping demonstrates the presence of swirling blood within the aneurysm.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
US at 30 wk
US at 35 wk
After delivery
US at 22 wk
US at 22 wk
US at 27 wk
2002
2002
2002
2006
2006
2007
2009
2011
2013
Weber
Sentilhes et al Hill et al
Olog et al
Our case
32 wk 2 d
31 wk 3 d
28 wk
34 wk
32 wk
26 wk
38 wk
36 wk
40 wk
37 wk
35 wk 5 d 27 wk
36 wk
Age at Delivery
CS
CS
CS
IUFD
IUFD
IUFD
NSVD
CS
NSVD
NSVD
IUFD IUFD
IUFD
Delivery Method
3VC
3VC
2VC
2VC
2VC
2VC
3VC
2VC
2VC
2VC
2VC NA
3VC
No. Vessels
PDA, normal variant PFO vs ASD
None
None
Cardiomegaly
Multicystic dysplastic kidney Cardiomegaly
None
Multiple
Multiple
Multiple
Cardiomegaly IUGR
None
Fetal Anomalies
Normal Normal
46,XX
Oligohydramnios
Oligohydramnios
NA
46,XY
46,XY
Oligohydramnios
Normal
46,XY
46,XY
Normal
Polyhydramnios
Polyhydramnios
Polyhydramnios
Oligohydramnios
NA
Amniotic Fluid
46,XY
47, XX+18
47, XX+18
47, XX+18
N/A N/A
N/A
Karyotype
Live born
Live born
Live born
IUFD
IUFD
IUFD
Live born
Death DOL 1
Death DOL 18
Death DOL 50
IUFD IUFD
IUFD
Outcomes
5
8
3
2.5
2.5
2.6
4
3
2.3
1.9
5 NA
3
UAA Size, cm
At placental insertion At placental insertion At placental insertion At placental insertion 3 cm from fetus
12 cm from fetus At placental insertion At placental insertion At placental insertion At placental insertion Midlength of cord N/A
N/A
UAA Location
N/A
Yes
No
Yes
Yes
Yes
Yes
No
No
No
No No
No
UAA Thrombus
ASD, atrial septal defect; CS, cesarean section; GA, gestational age; IUGR, intrauterine growth restriction; N/A, not available; NSVD, normal spontaneous vaginal delivery; PDA, patent ductus arteriosus; PFO, patent foramen ovale; UAA, umbilical artery aneurysm; VC, vessel cord.
US at 21 wk 2 d
US at 27 wk 2 d
At time of IUFD
US at 32 wk
1992 2001
2002
US at 30 wk NA
1978
Fortune and Ostor Siddiqi et al Jeffrey
Sepulveda et al: 1 Sepulveda et al: 2 Sepulveda et al: 3 Myosorekar et al Shen et al
At delivery
Year
Reference
GA Discovery of UAA
TABLE 1 Case Reports and Umbilical Artery Aneurysm Features
Umbilical Artery Aneurysm • CME Review Article 161
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
162
Obstetrical and Gynecological Survey
28 weeks 4 days, demonstrating low velocity flow within the umbilical artery aneurysm but no evidence of ventricular septal defect or other cardiac abnormalities. After informed consent was obtained, genetic amniocentesis was performed, confirming normal fetal karyotype. Her antenatal course was complicated by maternal obesity (body mass index, 35.2), gestational diabetes, and urinary tract infection. In addition, the patient and male partner reportedly had a consanguineous relationship of unknown degree. An interdisciplinary committee including neonatologists, maternal fetal medicine specialists, obstetrician gynecologists, nurses, and case managers convened to consider the risk for IUFD and benefits of various management strategies for this relatively rare condition. The decision on the timing and mode of delivery was made after careful review of the limited literature available. Twenty days after maternal hospitalization, the neonate was delivered by scheduled repeat cesarean delivery, at which time the estimated gestational age was 32 weeks 2 days. The birth weight of the neonate was 2180 g (48th percentile); Apgar scores were 6 at 1 minute and 8 at 5 minutes. Neonatal complications included respiratory distress of the newborn, hyperbilirubinemia of prematurity, anemia of prematurity, and difficulty feeding. Neonatal calcifications of the left portal vein were found on ultrasound and were hypothesized to be a calcified thrombus possibly related to the aneurysm. Neonatal echocardiogram showed a large patent ductus arteriosus and small atrial septal defect that possibly represented a normal variant of patent foramen ovale. These findings were likely related to prematurity given the gestational age at delivery. The newborn continued to do well and was discharged home from the neonatal intensive care unit on day of life (DOL) 19 in good condition with no significant morbidity either associated with premature delivery or as a result of the umbilical artery aneurysm. REVIEW OF UMBILICAL ARTERY ANEURYSM DIAGNOSIS AND MANAGEMENT A review and summary of the world literature were performed. The terms fetal and umbilical and artery and aneurysm were entered into the PubMed database. A total of 12 case reports were identified (Table 1).1,2,4–13 Including the case presented herein, there are only 4 live births reported with survival, 6 intrauterine fetal deaths, and 3 neonatal deaths. With the addition of this case, 2 of the 4 cases of surviving newborns were born at our institution.9 On the
basis of our acknowledged limited experience of this relatively rare condition, we propose consideration of the following with respect to antenatal management. Once the prenatal ultrasound occurs, karyotype should be considered. Trisomy 18 is associated in 3 (23%) of the 13 cases.1 Level II ultrasound to identify other coexisting congenital anomalies should also be used to aid in antenatal management. A single umbilical artery is a relatively common finding occurring in 0.2% of all births and of limited significance compared with the exceedingly rare finding of umbilical artery aneurysm. However, single umbilical artery is the most commonly associated congenital anomaly in fetuses with umbilical artery aneurysm and normal karyotypes; in our review of the literature, it was present in 4 cases (67%) (Table 2).5,6,8,9 There is no definitive evidence on the influence of a single umbilical artery on the development of an umbilical artery aneurysm. It has been speculated that, in cases of a single umbilical artery, the artery may undergo a compensatory increase in size resulting in thinning of the arterial wall and aneurysm formation.1,9 Four cases, including the one describe herein, also had cardiac abnormalities.5,7,8 Three cases of cardiomegaly have been reported in fetuses with umbilical artery aneurysm.5,7,8 Given our limited understanding of this rare congenital anomaly, it is difficult to assess the role of umbilical artery aneurysm on development of cardiomegaly. Our recommendation, based on the limited number of cases managed at our institution and review of the literature, is to perform antenatal TABLE 2 Umbilical Artery Aneurysm Features and Outcomes Outcomes (N = 13)
Delivery by cesarean delivery Delivery by vaginal delivery Delivery after 32 wk Delivery at 32 wk or earlier Trisomy 18 Normal karyotype Prenatal diagnosis Diagnosis at delivery 2-vessel cord 3-vessel cord Abnormal AFI Thrombosis
Live Born, n (%)
Neonatal Death, n (%)
IUFD, n (%)
3 (23%)
1 (8%)
0 (0%)
1 (8%)
2 (15%)
0 (0%)
2 (15%) 2 (15%)
3 (23%) 3 (23%)
3 (23%) 0 (0)
0 3 3 1 1 3 1 2
3 0 3 2 3 0 3 0
0 3 3 0 4 1 3 3
(0%) (23%) (23%) (8%) (8%) (23%) (8%) (15%)
(23%) (0%) (23%) (15%) (23%) (0%) (23%) (0%)
(0%) (23%) (23%) (0%) (31%) (8%) (23%) (23%)
AFI, amniotic fluid index; IUFD, intrauterine fetal demise.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Umbilical Artery Aneurysm • CME Review Article TABLE 3 A Summary of Prenatal Management Recommendations Prenatal Management Recommendations 1. Detailed sonographic midtrimester evaluation of fetal anatomy/ ‘‘level II ultrasound evaluation’’ 2. Fetal karyotype assessment 3. Fetal echocardiogram at 22–24 wk 4. Third trimester inpatient fetal surveillance at 28 wk 5. Neonatology consultation 6. Consideration of delivery at 32 wk
assessment with fetal echocardiography in this setting (Table 3). In reviewing all of the published literature, the risk for IUFD in pregnancies complicated by umbilical artery aneurysm is calculated to be 46%, at least half of which had normal chromosomes.1,2,4–13 Thrombosis and compression of neighboring vessels have been suggested as potential causes of IUFD in reported cases of umbilical artery aneurysm.5,6,8 It is difficult to speculate whether antenatal monitoring has the ability to prevent IUFD in the setting of umbilical artery aneurysm. In our department, the 2 patients we have cared for with fetal umbilical artery aneurysms were admitted for inpatient antenatal monitoring with nonstress tests 3 times daily, starting at viability, which is defined at our institution as 24 weeks, or at the time of diagnosis. In monoamniotic twins, where cord accidents are also a cause of a high rate of IUFD, this frequency of fetal monitoring has been used successfully and has served as a template for application to this clinical circumstance.14 Including this case report, 3 of the 4 surviving neonates were delivered by cesarean delivery between 28 weeks and 32 weeks 2 days of gestation.9–11 The gestational age at which IUFD was recognized varied significantly in the limited number of case reports available, occurring from 26 to 36 weeks.2,4–8 There are 3 reported cases of IUFD in fetuses with normal karyotype; these occurred at 26, 32, and 34 weeks of gestation. At our institution, a multidisciplinary team convened to discuss the timing of delivery. Given the significant risks associated with this condition, we suggest counseling patients in conjunction with neonatology to ensure that patients make informed decisions that balance the risks of prematurity with the possibility of fetal demise. On the basis of the previously presented information, the patient was offered and
163
accepted delivery at 32 weeks by repeat cesarean delivery. In patients without prior cesarean delivery, consideration should be made regarding route of delivery. Although there is a trend toward cesarean delivery in the limited number of cases with antenatal diagnosis of umbilical artery aneurysm, it is unclear whether this mode of delivery improves fetal outcomes. Although a rare antenatal finding, the serious risk for umbilical artery aneurysm requires a specialized approach to pregnancy management, the foundations of which include karyotype assessment, and heightened antenatal surveillance with early delivery to attempt to avoid IUFD. This article has provided an overview as to the diagnosis and management of patients whose fetuses may have umbilical artery aneurysm. REFERENCES 1. Sepulveda W, Corral E, Kottmann C, et al. Umbilical artery aneurysm: prenatal identification in three fetuses with trisomy 18. Ultrasound Obstet Gynecol. 2003;21:292–296. 2. Fortune D, Ostor A. Umbilical artery aneurysm. Am J Obstet Gynecol. 1978;131:339–340. 3. Ghosh A, Woo J, MacHenry C, et al. Fetal loss from umbilical cord abnormalities—a difficult case for prevention. Eur J Obstet Gynecol. 1984;18:183–198. 4. Jeffrey I. Intrapartum asphyxia and birth trauma. In: Keeling J. Fetal Neonatal Pathol. 3rd ed. London: Springer; 2001;237–259. 5. Sentilhes L, Vivet-Lefebure A, Patrier S, et al. Umbilical artery aneurysm in a severe growth-restricted fetus with normal karyotype. Prenat Diagn. 2007;27:1059–1061. 6. Shen O, Reinus C, Baranov A, et al. Prenatal diagnosis of umbilical artery aneurysm: a potentially lethal anomaly. J Ultrasound Med. 2007;26:251–253. 7. Siddiqi T, Bendon R, Schultz D, et al. Umbilical artery aneurysm: prenatal diagnosis and management. Obstet Gynecol. 1992;80:530–533. 8. Weber M, Sau A, Maxwell D, et al. Third trimester intrauterine fetal death caused by arterial aneurysm of the umbilical cord. Pediatr Dev Pathol. 2007;10:305–308. 9. Hill J, Strong T, Elliot J, et al. Umbilical artery aneurysm. Obstet Gynecol. 2010;116:559–562. 10. Myosorekar V, Dandekar C, Sundari N. Umbilical artery aneurysm: report of a rare case. Singapore J Obstet Gynecol. 2002;33:5153. 11. Olog A, Thomas J, Petersen S, et al. Large umbilical artery aneurysm with a live healthy baby delivered at 31 weeks. J Prenat Med. 2011;29:331–333. 12. Sherer D, Anyaegbunam A. Prenatal ultrasonographic morphologic assessment of the umbilical cord: a review. Part I. Obstet Gynecol Surv. 1997;52:506–514. 13. Sherer D, Anyaegbunam A. Prenatal ultrasonographic morphologic assessment of the umbilical cord: a review. Part II. Obstet Gynecol Surv. 1997;52:515–523. 14. Heyborne K, Porreco R, Garite T, et al. Obsetertrix/Pediatrix Research Study Group. Improved perinatal survival of monoamniotic twins with intensive inpatient monitoring. Am J Obstet Gynecol. 2005;192:96–101.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
CME REVIEW ARTICLE
8
CHIEF EDITOR’S NOTE: This article is part of a series of continuing education activities in this Journal through which a total of 36 AMA PRA Category 1 Credits TM can be earned in 2014. Instructions for how CME credits can be earned appear on the last page of the Table of Contents.
Umbilical Artery Aneurysm: A Case Report, Literature Review, and Management Recommendations Pooja Doehrman, MD,* Brenna J. Derksen, BS,† Jordan H. Perlow, MD,‡ William H. Clewell, MD,§ and Harris J. Finberg, MD} *Obstetrics and Gynecology Resident, Department of Obstetrics and Gynecology, Banner Good Samaritan Medical Center, Phoenix, AZ; †Medical Student, University of Arizona College of Medicine, Phoenix, AZ; ‡Director, Maternal-Fetal Medicine, Banner Good Samaritan Medical Center, Phoenix, AZ; §Maternal-Fetal Medicine and Director of Obstetric Ultrasound, Banner Good Samaritan Medical Center, and Clinical Professor of Obstetrics and Gynecology, University of Arizona College of Medicine, Phoenix, AZ; }Diagnosic Radiology, Banner Good Samaritan Medical Center, Phoenix, AZ Background: Umbilical artery aneurysm is a rare and often lethal condition frequently associated with fetal anomalies, fetal demise, and neonatal complications. Case: We report a case of umbilical artery aneurysm discovered at 21 weeks 2 days of gestation in a fetus of normal karyotype. Maternal hospitalization occurred at 28 weeks for antenatal testing, betamethasone administration, and monitoring for expansion of the aneurysm. Delivery of a live neonate by repeat cesarean delivery was performed at 32 weeks 2 days. Pathology confirmed a 3-vessel cord with an umbilical artery aneurysm. Neonatal course was complicated by respiratory distress of the newborn, hyperbilirubinemia, anemia, difficulty feeding, and cardiac defects. The newborn was discharged from the neonatal intensive care unit on day of life 19. Conclusions: Umbilical artery aneurysm is highly associated with fetal complications including trisomy 18, single umbilical artery, cardiac anomalies, and intrauterine fetal demise. A normal karyotype, antenatal monitoring, and early delivery have been suggested to impact the likeliness of survival. Antenatal management strategies include consideration of nonstress testing 3 times daily, serial ultrasound assessments, testing to identify intrauterine growth restriction, and delivery by planned cesarean delivery between 32 and 34 weeks. We recommend that patients be counseled on the high risks associated with umbilical artery aneurysm and be included in discussions regarding antenatal management and delivery planning. Target Audience: Obstetricians and gynecologists, family physicians Learning Objectives: After completing this CME activity, physicians should be better able to diagnose umbilical artery aneurysm using ultrasound and manage pregnant women whose fetuses have umbilical artery aneurysm.
All authors and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interests in, any commercial organizations pertaining to this educational activity. Correspondence requests to: Jordan H. Perlow, MD, Department of Obstetrics and Gynecology and Maternal-Fetal Medicine, Banner Good Samaritan Medical Center, 1111 E McDowell Rd, Phoenix, AZ 85006. E-mail: [email protected].
A 30-year-old Native American, G3P1011, at 21 weeks 2 days of gestation was found on routine second trimester obstetrical ultrasound to have a hypoechoic mass of the fetal umbilical cord measuring 2.7 4.0 3.4 cm. The patient was referred to a maternal fetal medicine group for further evaluation of the mass. At 26 weeks 6 days, a detailed level II ultrasound was performed for assessment of the
www.obgynsurvey.com | 159
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
160
Obstetrical and Gynecological Survey
FIG. 1. The umbilicus is to the left within the image, with 2 umbilical arteries straddling the fetal bladder. The cord widens 3 cm from the umbilicus, with the umbilical arteries splayed around a masslike region of mixed echogenicity.
fetal anatomy; it demonstrated a 3 5-cm enlargement of the umbilical cord beginning approximately 3 cm from the cord insertion at the umbilicus. During the ultrasound assessment, color Doppler flow demonstrated a swirling pattern of blood within the umbilical aneurysm and normal systolic to diastolic ratio (Figs. 1–3). The outer third of the cord defect at the level of the aneurysm appeared solid with echogenic heterogeneous material absent of vascularity. This solid material was hypothesized to be either a mural thrombus or focal thickening of Wharton jelly. Ultrasound examination raised the possibility of other abnormalities including membranous ventricular septal defect, overriding aorta, and calcifications adjacent to the umbilical vein at the level of hepatic
FIG. 3. Umbilical artery aneurysm of a neonate 3 cm from the cord insertion at the umbilicus on DOL 1.
insertion. A Doppler waveform obtained of the umbilical artery near the aneurysm demonstrated a normal systolic to diastolic ratio of 2.6. The patient was counseled regarding the association between umbilical artery aneurysm and aneu ploidy, specifically trisomy 18.1 She was also counseled on the significantly increased risk for intrauterine fetal demise (IUFD).2–8 After counseling the patient in regard to the variety of management options, the patient was hospitalized at 28 weeks 3 days. Inpatient management consisted of daily fetal movement assessments, nonstress testing 3 times daily, weekly fluid measurements, weekly umbilical artery Doppler assessments, and growth ultrasounds every 3 weeks. A fetal echocardiogram was performed at
FIG. 2. A, The cord mass has a well-circumscribed cystic area and an adjacent solid area of somewhat heterogeneous echogenicity, consistent with thrombus abutting the liquid blood portion of the aneurysm. B, Color flow mapping demonstrates the presence of swirling blood within the aneurysm.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
US at 30 wk
US at 35 wk
After delivery
US at 22 wk
US at 22 wk
US at 27 wk
2002
2002
2002
2006
2006
2007
2009
2011
2013
Weber
Sentilhes et al Hill et al
Olog et al
Our case
32 wk 2 d
31 wk 3 d
28 wk
34 wk
32 wk
26 wk
38 wk
36 wk
40 wk
37 wk
35 wk 5 d 27 wk
36 wk
Age at Delivery
CS
CS
CS
IUFD
IUFD
IUFD
NSVD
CS
NSVD
NSVD
IUFD IUFD
IUFD
Delivery Method
3VC
3VC
2VC
2VC
2VC
2VC
3VC
2VC
2VC
2VC
2VC NA
3VC
No. Vessels
PDA, normal variant PFO vs ASD
None
None
Cardiomegaly
Multicystic dysplastic kidney Cardiomegaly
None
Multiple
Multiple
Multiple
Cardiomegaly IUGR
None
Fetal Anomalies
Normal Normal
46,XX
Oligohydramnios
Oligohydramnios
NA
46,XY
46,XY
Oligohydramnios
Normal
46,XY
46,XY
Normal
Polyhydramnios
Polyhydramnios
Polyhydramnios
Oligohydramnios
NA
Amniotic Fluid
46,XY
47, XX+18
47, XX+18
47, XX+18
N/A N/A
N/A
Karyotype
Live born
Live born
Live born
IUFD
IUFD
IUFD
Live born
Death DOL 1
Death DOL 18
Death DOL 50
IUFD IUFD
IUFD
Outcomes
5
8
3
2.5
2.5
2.6
4
3
2.3
1.9
5 NA
3
UAA Size, cm
At placental insertion At placental insertion At placental insertion At placental insertion 3 cm from fetus
12 cm from fetus At placental insertion At placental insertion At placental insertion At placental insertion Midlength of cord N/A
N/A
UAA Location
N/A
Yes
No
Yes
Yes
Yes
Yes
No
No
No
No No
No
UAA Thrombus
ASD, atrial septal defect; CS, cesarean section; GA, gestational age; IUGR, intrauterine growth restriction; N/A, not available; NSVD, normal spontaneous vaginal delivery; PDA, patent ductus arteriosus; PFO, patent foramen ovale; UAA, umbilical artery aneurysm; VC, vessel cord.
US at 21 wk 2 d
US at 27 wk 2 d
At time of IUFD
US at 32 wk
1992 2001
2002
US at 30 wk NA
1978
Fortune and Ostor Siddiqi et al Jeffrey
Sepulveda et al: 1 Sepulveda et al: 2 Sepulveda et al: 3 Myosorekar et al Shen et al
At delivery
Year
Reference
GA Discovery of UAA
TABLE 1 Case Reports and Umbilical Artery Aneurysm Features
Umbilical Artery Aneurysm • CME Review Article 161
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
162
Obstetrical and Gynecological Survey
28 weeks 4 days, demonstrating low velocity flow within the umbilical artery aneurysm but no evidence of ventricular septal defect or other cardiac abnormalities. After informed consent was obtained, genetic amniocentesis was performed, confirming normal fetal karyotype. Her antenatal course was complicated by maternal obesity (body mass index, 35.2), gestational diabetes, and urinary tract infection. In addition, the patient and male partner reportedly had a consanguineous relationship of unknown degree. An interdisciplinary committee including neonatologists, maternal fetal medicine specialists, obstetrician gynecologists, nurses, and case managers convened to consider the risk for IUFD and benefits of various management strategies for this relatively rare condition. The decision on the timing and mode of delivery was made after careful review of the limited literature available. Twenty days after maternal hospitalization, the neonate was delivered by scheduled repeat cesarean delivery, at which time the estimated gestational age was 32 weeks 2 days. The birth weight of the neonate was 2180 g (48th percentile); Apgar scores were 6 at 1 minute and 8 at 5 minutes. Neonatal complications included respiratory distress of the newborn, hyperbilirubinemia of prematurity, anemia of prematurity, and difficulty feeding. Neonatal calcifications of the left portal vein were found on ultrasound and were hypothesized to be a calcified thrombus possibly related to the aneurysm. Neonatal echocardiogram showed a large patent ductus arteriosus and small atrial septal defect that possibly represented a normal variant of patent foramen ovale. These findings were likely related to prematurity given the gestational age at delivery. The newborn continued to do well and was discharged home from the neonatal intensive care unit on day of life (DOL) 19 in good condition with no significant morbidity either associated with premature delivery or as a result of the umbilical artery aneurysm. REVIEW OF UMBILICAL ARTERY ANEURYSM DIAGNOSIS AND MANAGEMENT A review and summary of the world literature were performed. The terms fetal and umbilical and artery and aneurysm were entered into the PubMed database. A total of 12 case reports were identified (Table 1).1,2,4–13 Including the case presented herein, there are only 4 live births reported with survival, 6 intrauterine fetal deaths, and 3 neonatal deaths. With the addition of this case, 2 of the 4 cases of surviving newborns were born at our institution.9 On the
basis of our acknowledged limited experience of this relatively rare condition, we propose consideration of the following with respect to antenatal management. Once the prenatal ultrasound occurs, karyotype should be considered. Trisomy 18 is associated in 3 (23%) of the 13 cases.1 Level II ultrasound to identify other coexisting congenital anomalies should also be used to aid in antenatal management. A single umbilical artery is a relatively common finding occurring in 0.2% of all births and of limited significance compared with the exceedingly rare finding of umbilical artery aneurysm. However, single umbilical artery is the most commonly associated congenital anomaly in fetuses with umbilical artery aneurysm and normal karyotypes; in our review of the literature, it was present in 4 cases (67%) (Table 2).5,6,8,9 There is no definitive evidence on the influence of a single umbilical artery on the development of an umbilical artery aneurysm. It has been speculated that, in cases of a single umbilical artery, the artery may undergo a compensatory increase in size resulting in thinning of the arterial wall and aneurysm formation.1,9 Four cases, including the one describe herein, also had cardiac abnormalities.5,7,8 Three cases of cardiomegaly have been reported in fetuses with umbilical artery aneurysm.5,7,8 Given our limited understanding of this rare congenital anomaly, it is difficult to assess the role of umbilical artery aneurysm on development of cardiomegaly. Our recommendation, based on the limited number of cases managed at our institution and review of the literature, is to perform antenatal TABLE 2 Umbilical Artery Aneurysm Features and Outcomes Outcomes (N = 13)
Delivery by cesarean delivery Delivery by vaginal delivery Delivery after 32 wk Delivery at 32 wk or earlier Trisomy 18 Normal karyotype Prenatal diagnosis Diagnosis at delivery 2-vessel cord 3-vessel cord Abnormal AFI Thrombosis
Live Born, n (%)
Neonatal Death, n (%)
IUFD, n (%)
3 (23%)
1 (8%)
0 (0%)
1 (8%)
2 (15%)
0 (0%)
2 (15%) 2 (15%)
3 (23%) 3 (23%)
3 (23%) 0 (0)
0 3 3 1 1 3 1 2
3 0 3 2 3 0 3 0
0 3 3 0 4 1 3 3
(0%) (23%) (23%) (8%) (8%) (23%) (8%) (15%)
(23%) (0%) (23%) (15%) (23%) (0%) (23%) (0%)
(0%) (23%) (23%) (0%) (31%) (8%) (23%) (23%)
AFI, amniotic fluid index; IUFD, intrauterine fetal demise.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Umbilical Artery Aneurysm • CME Review Article TABLE 3 A Summary of Prenatal Management Recommendations Prenatal Management Recommendations 1. Detailed sonographic midtrimester evaluation of fetal anatomy/ ‘‘level II ultrasound evaluation’’ 2. Fetal karyotype assessment 3. Fetal echocardiogram at 22–24 wk 4. Third trimester inpatient fetal surveillance at 28 wk 5. Neonatology consultation 6. Consideration of delivery at 32 wk
assessment with fetal echocardiography in this setting (Table 3). In reviewing all of the published literature, the risk for IUFD in pregnancies complicated by umbilical artery aneurysm is calculated to be 46%, at least half of which had normal chromosomes.1,2,4–13 Thrombosis and compression of neighboring vessels have been suggested as potential causes of IUFD in reported cases of umbilical artery aneurysm.5,6,8 It is difficult to speculate whether antenatal monitoring has the ability to prevent IUFD in the setting of umbilical artery aneurysm. In our department, the 2 patients we have cared for with fetal umbilical artery aneurysms were admitted for inpatient antenatal monitoring with nonstress tests 3 times daily, starting at viability, which is defined at our institution as 24 weeks, or at the time of diagnosis. In monoamniotic twins, where cord accidents are also a cause of a high rate of IUFD, this frequency of fetal monitoring has been used successfully and has served as a template for application to this clinical circumstance.14 Including this case report, 3 of the 4 surviving neonates were delivered by cesarean delivery between 28 weeks and 32 weeks 2 days of gestation.9–11 The gestational age at which IUFD was recognized varied significantly in the limited number of case reports available, occurring from 26 to 36 weeks.2,4–8 There are 3 reported cases of IUFD in fetuses with normal karyotype; these occurred at 26, 32, and 34 weeks of gestation. At our institution, a multidisciplinary team convened to discuss the timing of delivery. Given the significant risks associated with this condition, we suggest counseling patients in conjunction with neonatology to ensure that patients make informed decisions that balance the risks of prematurity with the possibility of fetal demise. On the basis of the previously presented information, the patient was offered and
163
accepted delivery at 32 weeks by repeat cesarean delivery. In patients without prior cesarean delivery, consideration should be made regarding route of delivery. Although there is a trend toward cesarean delivery in the limited number of cases with antenatal diagnosis of umbilical artery aneurysm, it is unclear whether this mode of delivery improves fetal outcomes. Although a rare antenatal finding, the serious risk for umbilical artery aneurysm requires a specialized approach to pregnancy management, the foundations of which include karyotype assessment, and heightened antenatal surveillance with early delivery to attempt to avoid IUFD. This article has provided an overview as to the diagnosis and management of patients whose fetuses may have umbilical artery aneurysm. REFERENCES 1. Sepulveda W, Corral E, Kottmann C, et al. Umbilical artery aneurysm: prenatal identification in three fetuses with trisomy 18. Ultrasound Obstet Gynecol. 2003;21:292–296. 2. Fortune D, Ostor A. Umbilical artery aneurysm. Am J Obstet Gynecol. 1978;131:339–340. 3. Ghosh A, Woo J, MacHenry C, et al. Fetal loss from umbilical cord abnormalities—a difficult case for prevention. Eur J Obstet Gynecol. 1984;18:183–198. 4. Jeffrey I. Intrapartum asphyxia and birth trauma. In: Keeling J. Fetal Neonatal Pathol. 3rd ed. London: Springer; 2001;237–259. 5. Sentilhes L, Vivet-Lefebure A, Patrier S, et al. Umbilical artery aneurysm in a severe growth-restricted fetus with normal karyotype. Prenat Diagn. 2007;27:1059–1061. 6. Shen O, Reinus C, Baranov A, et al. Prenatal diagnosis of umbilical artery aneurysm: a potentially lethal anomaly. J Ultrasound Med. 2007;26:251–253. 7. Siddiqi T, Bendon R, Schultz D, et al. Umbilical artery aneurysm: prenatal diagnosis and management. Obstet Gynecol. 1992;80:530–533. 8. Weber M, Sau A, Maxwell D, et al. Third trimester intrauterine fetal death caused by arterial aneurysm of the umbilical cord. Pediatr Dev Pathol. 2007;10:305–308. 9. Hill J, Strong T, Elliot J, et al. Umbilical artery aneurysm. Obstet Gynecol. 2010;116:559–562. 10. Myosorekar V, Dandekar C, Sundari N. Umbilical artery aneurysm: report of a rare case. Singapore J Obstet Gynecol. 2002;33:5153. 11. Olog A, Thomas J, Petersen S, et al. Large umbilical artery aneurysm with a live healthy baby delivered at 31 weeks. J Prenat Med. 2011;29:331–333. 12. Sherer D, Anyaegbunam A. Prenatal ultrasonographic morphologic assessment of the umbilical cord: a review. Part I. Obstet Gynecol Surv. 1997;52:506–514. 13. Sherer D, Anyaegbunam A. Prenatal ultrasonographic morphologic assessment of the umbilical cord: a review. Part II. Obstet Gynecol Surv. 1997;52:515–523. 14. Heyborne K, Porreco R, Garite T, et al. Obsetertrix/Pediatrix Research Study Group. Improved perinatal survival of monoamniotic twins with intensive inpatient monitoring. Am J Obstet Gynecol. 2005;192:96–101.
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
