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UOG Journal Club: February 2013
   Impact of cord entanglement on perinatal outcomes of
  monoamniotic twins: a systematic review of the literature

                     AC Rossi and F Prefumo
      Volume 41, Issue 2, Date: February 2013, pages 131–135




                 Journal Club slides prepared by Dr Aly Youssef
                 (UOG Editor for Trainees)
•   Monoamniotic pregnancies represent about 1% of cases of
    monozygotic twins

•   Monoamniotic twins are at high risk of fetal death and neonatal
    morbidity, secondary to congenital anomalies, premature birth and
    also presumably to umbilical cord entanglement

•   Cord entanglement is an almost universal finding at delivery in
    monoamniotic twins (Dias et al. 2010). However, a relatively recent
    review including literature from as early as 1990s showed that this
    was only documented on scan in 22% of cases (Roque et al. 2003)

•   Because of advances in obstetric ultrasound resolution, it is likely
    that prenatal diagnosis of cord entanglement has improved
    significantly over the last decade
                                             Dias T et al. Ultrasound Obstet Gynecol. 2010;35:201-4.
                                         Roque F et al. J Matern Fetal Neonatal Med 2003;13:414-21.
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013




 The aim of this meta-analysis was to review the literature
   published in the last decade on perinatal outcomes
    of monoamniotic pregnancies with documentation
               of cord entanglement at birth
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                                  Rossi and Prefumo, UOG 2013

                                         Methods
       Search of the literature between January 2000 and December 2011
           for relevant articles that described perinatal outcomes of
       monoamniotic twin pregnancies complicated by cord entanglement

                                                 Exclusion criteria
Inclusion criteria

•   Studies which included monoamniotic          1) Higher-order multiple
    twins with documented cord                      pregnancies, selective
    entanglement at the time of delivery            feticide, pregnancies complicated by twin
    and perinatal outcomes reported as              reversed arterial perfusion
    proportional rates rather than graphs or        sequence, conjoined twins and non-
    percentages                                     English language publications
                                                 2) Case reports and case series with <4
                                                    cases
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013




 Perinatal outcomes consisted of:
 1. Overall survival rates
 2. Number of surviving twins for each pregnancy,
 3. Intrauterine demise
 4. Neonatal mortality and morbidity*

 *Neonatal morbidity was defined as at least one of the following: an
 Apgar score <7 at 5 min, respiratory distress syndrome, admission to the
 NICU, congenital heart block, necrotizing enterocolitis and neurological
 impairment.
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                               Rossi and Prefumo, UOG 2013
                                      Results
                     78 potentially relevant studies concerning cord
                      entanglement in monoamniotic pregnancies

                                                        16 studies excluded based on title or abstract


                    62 articles retrieved for more detailed evaluation

                                                       40 studies excluded an inappropriate

                   22 articles potentially appropriate to be included in
                                  the systematic review

                                                       13 studies not meeting the inclusion criteria


              9 studies with relevant information included in the systematic
                   review. Final analysis included 114 monoamniotic
                     twin sets with cord entanglement (228 fetuses)
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                                Rossi and Prefumo, UOG 2013

 Results: outcome of pregnancies with cord entanglement at birth and at
                         prenatal ultrasound

                               Cord entanglement                  Cord entanglement
                                     at birth*                        at ultrasound
                          (9 articles, 114 pregnancies)       (4 articles, 60 pregnancies)
Overall survival rate               202/228                             105/120
   n/fetuses (%)                    (88.6%)                              (88%)

    No survivors                      8/114                              4/60
  n/pregnancies (%)                  (7.0%)                              (7%)

   Single survival                   10/114                               7/60
  n/pregnancies (%)                  (8.8%)                              (12%)
   Double survival                    96/114                             49/60
  n/pregnancies (%)                  (84.2%)                             (81%)

*At least 1 survivor was present in 106/114 (93.0%) pregnancies cord entanglement at birth
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013

       Perinatal outcome of pregnancies complicated by cord
                      entanglement vs controls

                         Overall survival rate




    No significant difference in overall survival rate was observed between
            pregnancies with and without cord entanglement at birth
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013

       Perinatal outcome of pregnancies complicated by cord
                      entanglement vs controls

                         Neonatal morbidity




          Controls showed a higher rate of neonatal morbidity
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013


                              Findings
• Cord entanglement appears to be a minor complication of monoamniotic
  twin pregnancies

• This review confirms that both neonatal morbidity and mortality of
  monoamniotic pregnancies do not depend on the presence of cord
  entanglement. Other factors, such as TTTS, prematurity and congenital
  anomalies are the main contributers to poor outcome

• Prenatal management of monoamniotic pregnancies should focus on the
  detection of sonographic signs of TTTS, structural anomalies or risk of
  preterm birth, rather than the presence of cord entanglement
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013


                             Limitations
• Most of the reviewed articles did not provide specific data on Doppler
  findings, therefore the authors could not comment on whether they were
  associated with neonatal morbidity and mortality

• Pooled data did not provide sufficient information on the benefit of
  inpatient vs outpatient prenatal management of women with prenatal
  diagnosis of cord entanglement

• The present review did not have sufficient power to demonstrate the
  effect of any particular antenatal management protocol on pregnancy
  outcome
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013



                      Future perspectives

  • Further studies are needed to investigate whether elective delivery
    at a specific gestational age is effective in improving perinatal
    outcome in pregnancies with prenatal diagnosis of cord
    entanglement

  • Ideally, such studies should be randomized controlled trials of
    monoamniotic pregnancies free of other complications. However,
    the rarity of monoamniotic pregnancy may preclude their completion.
Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a
                     systematic review of the literature
                          Rossi and Prefumo, UOG 2013



                        Discussion points

• How frequently should monoamniotic (MA) pregnancies be monitored
  and from which gestational age?
• Should MA pregnancies have inpatient or outpatient monitoring?
• Should cord entanglement be routinely searched for in MA pregnancies?
• At what gestational age should women with MA pregnancies with and
  without prenatal diagnosis of cord entanglement be delivered?
• Are there any specific Doppler findings associated with umbilical vessels
  narrowing due to cord entanglement?

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Impact of cord entanglement on perinatal outcomes in monoamniotic twins

  • 1. UOG Journal Club: February 2013 Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature AC Rossi and F Prefumo Volume 41, Issue 2, Date: February 2013, pages 131–135 Journal Club slides prepared by Dr Aly Youssef (UOG Editor for Trainees)
  • 2. Monoamniotic pregnancies represent about 1% of cases of monozygotic twins • Monoamniotic twins are at high risk of fetal death and neonatal morbidity, secondary to congenital anomalies, premature birth and also presumably to umbilical cord entanglement • Cord entanglement is an almost universal finding at delivery in monoamniotic twins (Dias et al. 2010). However, a relatively recent review including literature from as early as 1990s showed that this was only documented on scan in 22% of cases (Roque et al. 2003) • Because of advances in obstetric ultrasound resolution, it is likely that prenatal diagnosis of cord entanglement has improved significantly over the last decade Dias T et al. Ultrasound Obstet Gynecol. 2010;35:201-4. Roque F et al. J Matern Fetal Neonatal Med 2003;13:414-21.
  • 3. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 The aim of this meta-analysis was to review the literature published in the last decade on perinatal outcomes of monoamniotic pregnancies with documentation of cord entanglement at birth
  • 4. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Methods Search of the literature between January 2000 and December 2011 for relevant articles that described perinatal outcomes of monoamniotic twin pregnancies complicated by cord entanglement Exclusion criteria Inclusion criteria • Studies which included monoamniotic 1) Higher-order multiple twins with documented cord pregnancies, selective entanglement at the time of delivery feticide, pregnancies complicated by twin and perinatal outcomes reported as reversed arterial perfusion proportional rates rather than graphs or sequence, conjoined twins and non- percentages English language publications 2) Case reports and case series with <4 cases
  • 5. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Perinatal outcomes consisted of: 1. Overall survival rates 2. Number of surviving twins for each pregnancy, 3. Intrauterine demise 4. Neonatal mortality and morbidity* *Neonatal morbidity was defined as at least one of the following: an Apgar score <7 at 5 min, respiratory distress syndrome, admission to the NICU, congenital heart block, necrotizing enterocolitis and neurological impairment.
  • 6. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Results 78 potentially relevant studies concerning cord entanglement in monoamniotic pregnancies 16 studies excluded based on title or abstract 62 articles retrieved for more detailed evaluation 40 studies excluded an inappropriate 22 articles potentially appropriate to be included in the systematic review 13 studies not meeting the inclusion criteria 9 studies with relevant information included in the systematic review. Final analysis included 114 monoamniotic twin sets with cord entanglement (228 fetuses)
  • 7. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Results: outcome of pregnancies with cord entanglement at birth and at prenatal ultrasound Cord entanglement Cord entanglement at birth* at ultrasound (9 articles, 114 pregnancies) (4 articles, 60 pregnancies) Overall survival rate 202/228 105/120 n/fetuses (%) (88.6%) (88%) No survivors 8/114 4/60 n/pregnancies (%) (7.0%) (7%) Single survival 10/114 7/60 n/pregnancies (%) (8.8%) (12%) Double survival 96/114 49/60 n/pregnancies (%) (84.2%) (81%) *At least 1 survivor was present in 106/114 (93.0%) pregnancies cord entanglement at birth
  • 8. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Perinatal outcome of pregnancies complicated by cord entanglement vs controls Overall survival rate No significant difference in overall survival rate was observed between pregnancies with and without cord entanglement at birth
  • 9. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Perinatal outcome of pregnancies complicated by cord entanglement vs controls Neonatal morbidity Controls showed a higher rate of neonatal morbidity
  • 10. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Findings • Cord entanglement appears to be a minor complication of monoamniotic twin pregnancies • This review confirms that both neonatal morbidity and mortality of monoamniotic pregnancies do not depend on the presence of cord entanglement. Other factors, such as TTTS, prematurity and congenital anomalies are the main contributers to poor outcome • Prenatal management of monoamniotic pregnancies should focus on the detection of sonographic signs of TTTS, structural anomalies or risk of preterm birth, rather than the presence of cord entanglement
  • 11. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Limitations • Most of the reviewed articles did not provide specific data on Doppler findings, therefore the authors could not comment on whether they were associated with neonatal morbidity and mortality • Pooled data did not provide sufficient information on the benefit of inpatient vs outpatient prenatal management of women with prenatal diagnosis of cord entanglement • The present review did not have sufficient power to demonstrate the effect of any particular antenatal management protocol on pregnancy outcome
  • 12. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Future perspectives • Further studies are needed to investigate whether elective delivery at a specific gestational age is effective in improving perinatal outcome in pregnancies with prenatal diagnosis of cord entanglement • Ideally, such studies should be randomized controlled trials of monoamniotic pregnancies free of other complications. However, the rarity of monoamniotic pregnancy may preclude their completion.
  • 13. Impact of cord entanglement on perinatal outcomes of monoamniotic twins: a systematic review of the literature Rossi and Prefumo, UOG 2013 Discussion points • How frequently should monoamniotic (MA) pregnancies be monitored and from which gestational age? • Should MA pregnancies have inpatient or outpatient monitoring? • Should cord entanglement be routinely searched for in MA pregnancies? • At what gestational age should women with MA pregnancies with and without prenatal diagnosis of cord entanglement be delivered? • Are there any specific Doppler findings associated with umbilical vessels narrowing due to cord entanglement?