Polyhydramnios/Oligohydramnios in Twin Pregnancy John D. Yeast NeoReviews 2006;7;e305-e309 DOI: 10.1542/neo.

7-6-e305

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Article

neonatology

Polyhydramnios/ Oligohydramnios in Twin Pregnancy

John D. Yeast, MD*

Objectives
1. 2. 3. 4.

After completing this article, readers should be able to:

Author Disclosure Dr Yeast did not disclose any nancial relationships relevant to this article.

Describe the morbidity and mortality associated with monochorionic twin gestations. List the causes of polyhydramnios/oligohydramnios in twins. Recognize the complex pathophysiology of twin-to-twin transfusion syndrome. Delineate the treatment options currently available for polyhydramnios/ oligohydramnios and the relative value of each. 5. Know the importance of prenatal understanding of zygosity and placentation in twin gestations.

Introduction
The discovery of increased amniotic uid in the gestational sac of one twin and decreased amniotic uid in the amniotic sac of the second twin should generate an immediate clinical suspicion of twin-twin transfusion syndrome (TTTS). This relatively uncommon condition occurs when twins sharing a single placenta develop signicant vascular anastomoses that result in a unidirectional shunt from one twin to the other. Although independent diagnoses could be affecting each twin simultaneously, leading to separate abnormalities in amniotic uid volume, the possibility of such clinical coincidence is rare. Indeed, if the clinician recognizes that the placentation is monochorionic, the leading consideration in the differential diagnosis must be TTTS.

Incidence and Frequency

TTTS is unique to monozygotic (one egg), monochorionic (one placenta) twin gestations. It has been recognized for some time that monochorionic twins experience greater morbidity and fetal loss than do dichorionic twins. Fortunately, only 30% of twins are monochorionic (Table 1). Of those, up to 15% may demonstrate some degree of TTTS. Thus, for all spontaneous twin gestations, the risk of TTTS is 4.5%. It is estimated that 15% of perinatal mortality for twins is due to TTTS. Death may occur in either the donor (oligohydramnios) twin or the recipient (polyhydramnios) twin. Although perinatal mortality was nearly 100% with this diagnosis as recently as 25 years ago, improvements in outcome have resulted from better management options. In addition, earlier diagnosis via ultrasonography allows better obstetric management. Mortality remains signicant, however, with 20% to 30% loss experienced in many centers. In addition, signicant morbidity may be seen in the surviving twin, with a high risk of neurologic abnormalities as well as cardiac and renal disorders.

Diagnosis
In many cases, the diagnosis of TTTS is suspected only after the incidental nding of polyhydramnios/oligohydramnios sequence (POS) during routine ultrasonography of twins. In some instances, the mother may present with symptoms of a rapid increase in uterine size, changes in perceived fetal movement, or uterine contractions. These presentations are less common, however, than an asymptomatic presentation. When any twin gestation is diagnosed, it is important to determine chorionicity as early as possible. Classication of placentation is easier early in gestation. If the twins are concordant for sex, placental location should be conrmed. If two separate placentas are
*Professor and Vice Chairman, Department of Obstetrics and Gynecology, University of Missouri-Kansas City School of Medicine; Director of Medical Affairs, Saint Lukes Hospital of Kansas City, Kansas City, Mo. NeoReviews Vol.7 No.6 June 2006 e305

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The Frequency of Placentation in Twin Pregnancies

Table 1.

tient. This has resulted in the term stuck twin and conrms the diagnosis of TTTS.

Type of Placentation Dichorionic/Diamnionic, fused* Dichorionic/Diamnionic, separated* Monochorionic/Diamnionic** Monochorionic/Monoamnionic**

*Monozygotic or dizygotic **Monozygotic only

Frequency 34.0% 35.2% 29.6% 1.2%

Cause

noted, with a thick septum or membrane between each sac, a dichorionic gestation exists. If a single placental bed is seen, careful examination of the membranes should help determine chorionicity. At the junction of the membrane into the placental bed, the membrane appears thick, with the twin peak sign of a widened insertion when the placentation is dichorionic. With a monochorionic gestation, the membrane is thin, with no intervening chorionic layer. Determination of a monochorionic twin gestation always should alert the clinician to the additive risks seen in TTTS. Ultrasonography examinations should be performed every 3 weeks in monochorionic twin gestations to assure that amniotic uid volumes and fetal size remain concordant. It is important to recognize that methods of measuring amniotic uid volume in twins are less precise than methods advocated for singletons. Some centers use the amniotic uid index (AFI); others use the single deepest pocket. In either instance, the sonographer must pay careful attention to the volumes of uid and changes between serial scans. Although there is no consensus on the best method to measure amniotic uid volumes, one recognized method is to consider POS if the deepest pocket in the reducedvolume twin is less than 2 cm and the deepest pocket in the increased-volume twin is greater than 8 cm. In many instances, no uid is seen in the sac of the donor twin at the time of diagnosis. Occasionally, monoamniotic twin gestation is diagnosed erroneously when the sonographer sees twins without an apparent septum. The clinician always should consider that this nding may represent TTTS with severe oligohydramnios in one sac. The membrane, or sac wall, may be almost adherent to the fetus, creating the illusion of a monoamniotic gestation. Observation via ultrasonography usually helps to achieve the correct diagnosis. The twin that has oligohydramnios will not move following repositioning or ambulation of the pae306 NeoReviews Vol.7 No.6 June 2006

As noted previously, TTTS is a pathologic condition that essentially only occurs in monochorionic twin gestations. An isolated case has been reported of vascular anastomoses and TTTS in a dichorionic set of twins, but the exact cause is not well understood. For clinical purposes, only monozygotic twins that have a monochorionic placenta are at risk. The most common pathologic nding is the development in the placenta of both supercial and deep vascular anastomoses between the circulations of each twin. The anastomoses may be artery to artery (A-A), artery to vein (A-V), or vein to vein (V-V). Anastomoses, especially A-A and V-V, can be demonstrated in nearly every monochorionic twin placenta, but imbalance of the anastomoses seems to be key to the development of TTTS. Specically, a dominance of A-V anastomoses leads to an unbalanced shunt from one fetus to the other. In addition, more complex changes in the placenta contribute to the condition. Signicant asymmetry is apparent in the placenta due to unequal partitioning of the placenta that may arise as early as implantation. Thus, the exact anatomic ndings are complex, and the precise origins are not yet understood. Clinicians at centers that have substantial experience treating TTTS have noted signicant maternal metabolic differences compared with other patients who are pregnant with twins. The women who have TTTS nearly all have signicant anemia, hypoproteinemia, and low colloid osmotic pressures. In addition, maternal weight gain, when corrected for the weight associated with the polyhydramnios, is below norms for twins. Clinically, the patients have signicant extravascular uid compartmentalization and are at risk for pulmonary edema due to low colloid pressures. The combination of the vascular ndings, placental development, and maternal metabolic changes results in the unique ndings in this condition that may include:

Polyhydramnios in the recipient twin Polycythemia in the recipient twin Oligohydramnios in the donor twin Anemia and growth restriction in the donor twin Developmental disorders in either twin

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Table 2.

Criteria for Intervention in Twin-Twin Transfusion Syndrome

Less than 25 weeks gestational age Monochorionic placenta Polyhydramnios (>8 cm deepest pocket) in one gestational sac Oligohydramnios (<1 cm deepest pocket) or stuck twin in one gestational sac Probable polyuria in one twin (distended bladder) and oliguria (empty bladder) in other twin

should exclude other causes that rarely can be present with similar ndings. These include certain congenital malformations, uteroplacental insufciency, and intrauterine infections.

Treatment
A variety of interventions is available, and considerable discussion within the perinatal literature weighs the risks/benets of each. The choice of treatment method often is limited by availability; not all perinatal centers can offer each of the available options. Repeated amnioreduction is one straightforward method to attempt to improve intrauterine function and delay delivery. In many ways similar to amnioreduction in a singleton gestation with polyhydramnios, uid is suctioned via an 18-gauge spinal needle and a controlled method of negative pressure. Attempts are made to reduce the amniotic uid in the polyhydramnios sac to near-normal levels. Serial procedures often are necessary because the uid reaccumulates rapidly. Interestingly, reducing the uid in one sac may change the vascular anastomoses and normalize blood ow between shunts. The fetus that has oligohydramnios also can benet, and both fetuses can normalize amniotic uid volumes. Fetoscopic laser ablation is a highly specialized procedure to identify surface vessels on the placenta that demonstrate anastomoses and ablate them via laser. The procedure requires highly experienced endoscopists using specialized equipment to approach the placenta and membranes. Identication of the anastomoses allows laser photocoagulation and, hopefully, normalization of vascular ow. Septostomy is the creation of an articial window in the membrane separating each fetus with a needle at the time of amniocentesis. The creation of an opening in the membranes allows uid to ow from the larger, higherpressure sac into the smaller, lower-pressure sac. This, in turn, helps to normalize some of the pressure gradients and reduce shunted blood ow. Unfortunately, it also eliminates assessment of uid volume as a measure of fetal well-being because amniotic uid can ow to and from either sac. Medical therapy to reduce amniotic uid volume in the twin who has polyhydramnios has been advocated in some centers. Prostaglandin inhibitors, such as indomethacin, have been shown to reduce amniotic uid volume in the recipient twin, but may worsen the condition of the donor twin. Other studies have evaluated digoxin in treating this condition. No large studies have supported widespread use of either modality. Selective termination of one fetus can stop shunted
NeoReviews Vol.7 No.6 June 2006 e307

Adapted from Hecher et al. Eur J Obstet Gynecol Reprod Biol. 2000; 92:135.

Ischemic injury in either twin Neurologic development disruption or injury in either twin

The compound ndings noted previously may result in fetal death, preterm labor and delivery, and signicant risk of prolonged morbidity in survivors.

Evaluation
The nding of POS in a twin gestation and the presumptive diagnosis of TTTS requires prompt evaluation and development of a management plan. The ndings that support some form of intervention, as summarized by Hecher and associates, are listed in Table 2. Basically, diagnosis at an early gestational age, such as less than 25 weeks gestation, and severe oligohydramnios in the donor twin necessitate some form of intervention. Referral to a perinatal center that has high-resolution ultrasonography and experience in the management of TTTS is strongly recommended. Some centers characterize TTTS by clinical ndings. The following stages are based on ultrasonographic and obstetric ndings:

Stage 1: POS present, with the fetal bladder still visible in the donor Stage 2: POS with no bladder visible in the donor, but normal Doppler study results Stage 3: POS with no bladder seen in donor and at least one abnormal Doppler ow study result in the donor or recipient Stage 4: POS and hydrops (donor or recipient) Stage 5: Fetal demise of one or both twins

Although observation of POS in monochorionic twins is largely diagnostic for TTTS, a clinician and sonographer

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blood ow and may allow restoration of normal umbilical circulation in the surviving twin. Rarely, this may be the preferred method of management, especially when the twins are discordant for malformation. The procedure is accomplished by mechanically obstructing the ow in one cord with ligation, coil placement, or cautery and requires high-resolution ultrasonography, possibly fetoscopy, and signicant experience on the part of the operators. Prospective, randomized studies of treatment options are rare with unusual conditions such as TTTS. A recent study (Moise) compared serial amnioreduction with septostomy in TTTS and supported septostomy. Although outcome results were similar, the study demonstrated that only one amniocentesis was necessary with septostomy versus the potential of multiple procedures with amnioreduction. Only one randomized study (Senat) has compared laser ablation with serial amnioreduction. The investigators showed signicant improvement in survival, with reduced morbidity in laser-treated patients versus those having amnioreduction. The overall survival with laser ablation was 57% versus 41% with amnioreduction. More than 50% of the survivors had no neurologic abnormality compared with only 31% of the amnioreduction survivors. It must be recognized that overall mortality and morbidity is high with both methods of treatment. Other studies have reported outcomes with single methods of treatment or used historic controls. As a result, ascertainment bias and selection criteria inuence the results of many published series. It is difcult to provide a precise template for which patients benet from which treatment. However, development of TTTS at less than 24 weeks gestational age requires thorough perinatal evaluation and treatment. Otherwise, outcome is dismal. Following delivery, it is important to study the placenta and membranes carefully to conrm the prenatal diagnosis. Experienced pathologists often can demonstrate vascular anastomoses to help conrm the clinical suspicion. Documentation should be conrmed via photographs or pathology report. Careful obstetric management is important in affected pregnancies, including use of betamethasone to reduce the risk of respiratory distress in survivors, fetal surveillance via electronic fetal monitors and ultrasonography, and a high suspicion for jeopardy with the capability of prompt delivery. Despite aggressive management, however, perinatal mortality remains high, and fetal death can immediately follow reassuring fetal surveillance.
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Suggested Reading
Baldwin VJ. Pathology of Multiple Pregnancy. New York, NY: Springer-Verlag; 1994:199 Barrea C, Alkazaleh F, Ryan G, et al. Prenatal cardiovascular manifestations in the twin-to-twin transfusion syndrome recipients and the impact of therapeutic amnioreduction. Am J Obstet Gynecol. 2005;192:892902 Bendon RW. Twin transfusion: pathological studies of the monochorionic placenta in liveborn twins and of the perinatal autopsy in monochorionic twin pairs. Pediatr Pathol Lab Med. 1995;15: 363376 Benirschke K, Masliah E. The placenta in multiple pregnancy: outstanding issues. Reprod Fertil Dev. 2001;13:615 622 Bromley B, Frigoletto FD, Estroff JA, Benacerraf BR. The natural history of oligohydramnios/polyhydramnios sequence in monochorionic diamniotic twins. Ultrasound Obstet Gynecol. 1992;2:317320 Chescheir NC, Seeds JW. Polyhydramnios and oligohydramnios in twin gestations. Obstet Gynecol. 1988;71:882 884 De Lia JE, Kuhlmann RS, Cruikshank DP, OBee LR. Current topic: placental surgery: a new frontier. Placenta. 1993;14: 477 485 Fesslova V, Villa L, Nova S, Mosca F, Nicolini U. Fetal and neonatal echocardiographic ndings in twin-twin transfusion syndrome. Am J Obstet Gynecol. 1998;179:1056 1062 Fisk NM, Borrell A, Hubinont C, Tannirandorn Y, Nicolini U, Rodeck CH. Fetofetal transfusion syndrome: do the neonatal criteria apply in utero? Arch Dis Child. 1990;65:657 661 Fox C, Kilby MD, Khan KS. Contemporary treatments for twintwin transfusion syndrome. Obstet Gynecol. 2005;105: 1469 1477 Gaziano EP, De Lia JE, Kuhlmann RS. Diamnionic monochorionic twin gestations: an overview. J Matern Fetal Med. 2000;9: 89 96 Glinianaia SV, Pharoah PO, Wright C, Rankin JM, Northern Region Perinatal Mortality Survey Steering Group. Fetal or infant death in twin pregnancy: neurodevelopmental consequence for the survivor. Arch Dis Child Fetal Neonatal Ed. 2002;86:F9 F15 Hecher K, Diehl W, Zikulnig L, Vetter M, Hackeloer BJ. Endoscopic laser coagulation of placental anastomoses in 200 pregnancies with severe mid-trimester twin-to-twin transfusion syndrome. Eur J Obstet Gynecol Reprod Biol. 2000;92:135139 Jain V, Fisk NM. The twin-twin transfusion syndrome. Clin Obstet Gynecol. 2004;47:181202 Lopriore E, Nagel HT, Vandenbussche FP, Walther FJ. Long-term neurodevelopmental outcome in twin-to-twin transfusion syndrome. Am J Obstet Gynecol. 2003;189:1314 1319 Mari G, Roberts A, Detti L, et al. Perinatal morbidity and mortality rates in severe twin-twin transfusion syndrome: results of the International Amnioreduction Registry. Am J Obstet Gynecol. 2001;185:708 715 Moise KJ, Dorman K, Lamvu G, et al. A randomized trial of amnioreduction versus septostomy in the treatment of twintwin transfusion syndrome. Am J Obstet Gynecol. 2005;193: 701707 Nicolini U, Poblete A, Boschetto C, Bonati F, Roberts A. Complicated monochorionic twin pregnancies: experience with bipolar cord coagulation. Am J Obstet Gynecol. 2001;185:703707

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Porreco RP, Barton SM, Haverkamp AD. Occlusion of umbilical artery in acardiac, acephalic twin. Lancet. 1991;337:326 327 Quintero RA, Bornick PW, Allen MH, Johnson PK. Selective laser photocoagulation of communicating vessels in severe twin-twin transfusion syndrome in women with an anterior placenta. Obstet Gynecol. 2001;97:477 481 Quintero RA, Dickinson JE, Morales WJ, et al. Stage-based treatment of twin-twin transfusion syndrome. Am J Obstet Gynecol. 2003;188:13331340 Robyr R, Quarello E, Ville Y. Management of fetofetal transfusion syndrome. Prenat Diagn. 2005;25:786 795 Saade GR, Belfort MA, Berry DL, et al. Amniotic septostomy for the treatment of twin oligo-polyhydramnios sequence. Fetal Diagn Ther. 1998;13:86 93 Senat MV, Deprest J, Boulvain M, Ville Y. Fetoscopic laser surgery versus serial amniodrainage in the management of severe twinto-twin transfusion syndrome at midgestation: a randomized, controlled trial. Am J Obstet Gynecol. 2003;189:S56 Senat MV, Deprest J, Boulvain M, Paupe A, Winer N, Ville Y. Endoscopic laser surgery versus serial amnioreduction for severe

twin-to-twin transfusion syndrome. N Engl J Med. 2004;351: 136 144 Sutcliffe AG, Sebire NJ, Pigott AJ, Taylor B, Edwards PR, Nicolaides KH. Outcome for children born after in utero laser ablation therapy for severe twin-to-twin transfusion syndrome. Br J Obstet Gynecol. 2001;108:1246 1250 Taylor MJ, Denbow ML, Tanawattanacharoen S, Gannon C, Cox PM, Fisk NM. Doppler detection of artero-arterial anastomoses in monochorionic twins: feasibility and clinical application. Hum Reprod. 2000;15:16321636 Taylor MJ, Govender L, Jolly M, Wee L, Fisk NM. Validation of the Quintero staging system for twin-twin transfusion syndrome. Obstet Gynecol. 2002;100:12571265 Wee LY, Taylor M, Watkins N, Franke V, Parker K, Fisk NM. Characterisation of deep arterio-venous anastomoses within monochorionic placentae by vascular casting. Placenta. 2005; 26:19 24 Yeast JD. Multiple gestations. In: Evans AT, ed. Manual of Obstetrics. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2006; in press

NeoReviews Quiz
7. Twin-twin transfusion occurs when placental vascular anastomoses result in a unidirectional shunt from one twin to the other. Of the following, the twin-twin transfusion is unique to twins that are: A. B. C. D. E. Dizygotic, dichorionic. Dizygotic, monoamnionic. Dizygotic, monochorionic. Monozygotic, dichorionic. Monozygotic, monochorionic.

8. A twin pregnancy is complicated by polyhydramnios in one twin and oligohydramnios in the other twin at an estimated gestational age of 29 weeks. You suspect twin-twin transfusion. Fetal ultrasonography and placental blood ow studies show that the donor twin has developed hydrops. Of the following, the most appropriate stage of twin-twin transfusion in this patient is: A. B. C. D. E. Stage Stage Stage Stage Stage 1. 2. 3. 4. 5.

9. Several interventions have been explored for the treatment of twin-twin transfusion. In the absence of large randomized trials, however, the safety/efcacy prole of each intervention remains unestablished, making it difcult to dene a template for evaluation and treatment. Of the following, the most commonly used intervention for the treatment of twin-twin transfusion is: A. B. C. D. E. Amniotic membrane septostomy. Fetoscopic laser ablation. Repeated amnioreduction. Selective termination. Treatment with prostaglandin inhibitors.
NeoReviews Vol.7 No.6 June 2006 e309

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Polyhydramnios/Oligohydramnios in Twin Pregnancy John D. Yeast NeoReviews 2006;7;e305-e309 DOI: 10.1542/neo.7-6-e305

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