Notification



Notification Issue Date:



Medical Policy Bulletin


Title:Treatment of Twin-Twin Transfusion Syndrome (TTTS)

Policy #:11.00.14e

This policy is applicable to the Company’s commercial products only. Policies that are applicable to the Company’s Medicare Advantage products are accessible via a separate Medicare Advantage policy database.


The Company makes decisions on coverage based on Policy Bulletins, benefit plan documents, and the member’s medical history and condition. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

This Medical Policy Bulletin document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy Bulletin will be reviewed regularly and be updated as scientific and medical literature becomes available. For more information on how Medical Policy Bulletins are developed, go to the About This Site section of this Medical Policy Web site.



Policy

Coverage is subject to the terms, conditions, and limitations of the member's contract.

MEDICALLY NECESSARY

Fetoscopic laser photocoagulation of placental anastomoses, or serial amnioreduction combined with fetoscopic laser photocoagulation, is considered medically necessary and, therefore, covered as a treatment for twin-twin transfusion syndrome (TTTS) when all of the following criteria are met:
  • Gestational age less than 26 weeks
  • Ultrasonographic examination shows a single, monochorionic placenta
  • Oligohydramnios (reduced amniotic fluid) in the donor with the deepest vertical pocket less than 2 cm
  • Polyhydramnios (excessive amniotic fluid) of the recipient twin with deepest vertical pocket of amniotic fluid 8 cm or more
  • Discordant fetal bladders with markedly enlarged bladder in the recipient twin and very small or non-cycling bladder in the donor twin during most of the exam

Serial amnioreduction is considered medically necessary and, therefore, covered as a treatment for TTTS when the criteria for fetoscopic laser photocoagulation are not met.

All other uses for serial amnioreduction, fetoscopic laser photocoagulation of placental vessels, or serial amnioreduction combined with fetoscopic laser photocoagulation as a treatment for TTTS are considered experimental/investigational and, therefore, not covered because their safety and/or effectiveness cannot be established by review of the available published peer-reviewed literature.

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the service.
Guidelines

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable benefit contract, serial amnioreduction, fetoscopic laser photocoagulation of placental anastomoses, or serial amnioreduction combined with fetoscopic laser photocoagulation as a treatment for twin-twin transfusion syndrome (TTTS) is covered under the medical benefits of the Company’s products when the medical necessity criteria listed in this medical policy are met.
Description

Twin-twin transfusion syndrome (TTTS) occurs in cases of monochorionic (one placenta) diamniotic twins. In all monochorionic pregnancies, there are vascular communications (anastomoses) between the fetuses. These communications can consist of artery-artery, vein-vein and artery-vein. Though not yet fully understood, TTTS results when there is a threshold reached of artery-venous anastomoses that creates an imbalance in the net direction of blood flow within the placenta. This results in a state of hypervolemia (increased blood flow), with resultant polyhydramnios (excessive amniotic fluid) and hypertrophic cardiomyopathy (thickened heart muscle) in one fetus (recipient) and a state of hypovolemia (decreased blood flow) with resultant growth restriction, oligio- or anhydramnios (reduced or no amniotic fluid), and the potential for a restrictive cardiomyopathy (weakened heart muscle) in the other fetus (donor). If left untreated, progressive TTTS is fatal in up to 90% of cases, either before birth or from complications of prematurity.

TTTS can occur any time within a monochorionic gestation but is associated with significant increases in both morbidity and mortality when it occurs before 26 weeks of gestation.

Contemporary treatment options for TTTS include fetoscopic laser photocoagulation of the placental anastomoses, serial amnioreduction, and, in severe cases, umbilical cord occlusion. In fetoscopic laser photocoagulation, vascular anastomoses on the surface of the placenta are identified endoscopically under ultrasound guidance and ablated using a laser. This separates the two fetal circulations, eliminating the underlying abnormality. Serial amnioreduction is a variant of amniocentesis in which amniotic fluid is repeatedly removed in order to restore normal fluid volume. Serial amnioreduction has been used to reduce maternal discomfort and control polyhydramnios with the hope of preventing preterm delivery and maintaining the pregnancy until the extreme risks of prematurity lessen.

Based on outcomes of comparative studies, there is prevailing expert agreement that fetoscopic selective laser photocoagulation presents the best treatment option for cases of TTTS presenting prior to 26 weeks gestation. In this situation, overall survival rates of 75%-80%, and survivals of at least one twin have been documented. A minority of cases may be affected with neurologic impairment, found to be 6% at birth and 11% at long-term follow-up. Serial amnioreduction remains a treatment option for those not meeting laser surgery criteria, or for whom the procedure is not feasible.

In contrast to serial amnioreduction and fetoscopic laser photocoagulation of the placental anastomoses, umbilical cord occlusion is considered only for very early, severe cases. The rationale for umbilical cord occlusion is to prolong gestation and maximize the outcome for the surviving twin.

The alternative to prenatal intervention is conservative management, which is associated with a fetal mortality rate between 90 percent and 100 percent.
References

Akkermans J, Peeters SHP, Klumper FJ, et al. Twenty-five years of fetoscopic laser coagulation in twin-twin transfusion syndrome: A systematic review. Fetal Diagn Ther. 2015;38:241-253.


American College of Obstetricians and Gynecologists (ACOG). ACOG Practice Bulletin No. 169: Multifetal Gestations: Twin, Triplet, and Higher-Order Multifetal Pregnancies. Obstet Gynecol. 2016;128(4):e131-46.

American College of Obstetricians and Gynecologists (ACOG). ACOG Practice Bulletin No. 144: Multifetal Gestations: Twin, Triplet, and Higher-Order Multifetal Pregnancies. Obstet Gynecol. 2014;123(5): 1118–1132. (Replaced by Practice Bulletin 186).

American College of Obstetricians and Gynecologists (ACOG). ACOG Practice Bulletin No. 56: Complicated twin, triplet, and high-order multifetal pregnancy. Obstet Gynecol. 2004;104:869–83. (Reaffirmed 2009; replaced by Practice Bulletin 144).

Assistance Publique - Hôpitaux de Paris. Randomized controlled trial comparing a conservative management and laser surgery (TTTS1). NCT01220011. In: ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2000- [Last Updated 12/21/2016]. Available from: https://clinicaltrials.gov/ct2/show/NCT01220011. Accessed January 9, 2018.

Barrea C, Debauche C, Williams O, et al. Twin-to twin transfusion syndrome: perinatal outcome and recipient heart disease according to treatment strategy. J Paediatr Child Health. 2013;49:E28-E34.

Baschat AA, Barber J, Pedersen N, et al. Outcome after fetoscopic selective laser ablation of placental anastomoses vs equatorial laser dichorionization for the treatment of twin-to-twin transfusion syndrome. Am J Obstet Gynecol. 2013;209(3):234.e1-8.

Baschat A, Chmait RH, Deprest J, et al. Recommendations and guidelines for Perinatal practice: Twin-to-twin transfusion syndrome (TTTS) J Perinat Med. 2011;39:107-112.

Baud D, Windrim R, Keunen J, et al. Fetoscopic laser therapy for twin-twin transfusion syndrome before 17 and after 26 weeks gestation. Am J Obstet Gynecol. 2013;208:197.e1-7.

Bebbington M. Twin-to-twin transfusion syndrome: current understanding of pathophysiology, in-utero therapy and impact for future development. Semin Fetal Neonatal Med. 2010;15:15-20.

Bebbington M, Tiblad E, Huesler-Charles M, et al. Outcomes in a cohort of patients with stage 1 twin-to-twin transfusion syndrome (TTTS). Ultrasound Obstet Gynecol. 2010;36(1):48-51.

Bebbington MW, Danzer E, Moldenhauer J, et al. Radio frequency ablation vs. bipolar umbilical cord coagulation in the management of complicated monochorionic pregnancies. Ultrasound Obstet Gynecol. 2012;40(3):319-24.

Chalouhi GE, Essaoui M, Stirnemann J, et al. Laser therapy for twin-to-twin transfusion syndrome (TTTS). Prenat Diagn. 2012.31:637-646.

Chang YL, Chao AS, Chang SD, et al. The neurological outcomes of surviving twins in severe twin-twin transfusion syndrome treated by fetoscopic laser photocoagulation at a newly established center. Prenat Diagn. 2012;32:893-896.

Children's Hospital of Philadelphia (CHOP): Center for fetal diagnosis and treatment. Twin-twin transfusion syndrome (TTTS). [CHOP Web site]. Available at: http://www.chop.edu/service/fetal-diagnosis-and-treatment/fetal-diagnoses/twin-twin-transfusion-syndrome-ttts.html. Accessed January 9, 2018.

Cincotta RB, Gray PH, Gardener G, et al. Selective fetoscopic laser ablation in 100 consecutive pregnancies with severe twin-twin transfusion syndrome. Aust N Z J Obstet Gynaecol. 2009;49(1):22-27.

Crombleholme TM. The treatment of twin-twin transfusion syndrome. Semin Pediatr Surg. 2003;12(3):175-181.

D’Antonio F, Khalil A, Dias T, et al. Early fetal loss in monochorionic and dichorionic twin pregnancies: analysis of the Southwest Thames Obstetric Research Collaborative (STORK) multiple pregnancy cohort. Ultrasound Obstet Gynecol. 2013; 41(6):632-6.

Duryea EL, Happe SK, McIntire DD, et al. The natural history of twin-twin transfusion syndrome stratified by Quintero stage. J Matern Fetal Neonatal Med. 2016;29(21):3411-5.

Ek S, Halvorsen CP, Kublickas M, et al. Establishing a national program for fetoscopic guided laser occlusion for twin-to-twin transfusion syndrome in Sweden. Acta Obstet Gynecol Scand. 2012 Oct;91(10):1196-200.

El Kateb A, Ville Y. Update on twin-to-twin transfusion syndrome. Best Pract Res Clin Obstet Gynaecol. 2008;22(1):63-75.

Emery SP, Bahtiyar MO, Moise KJ. The North American Fetal Therapy Network Consensus Statement: Management of complicated monochorionic gestations. Obstet Gynecol. 2015;126(3):575-584.

Finneran MM, Pickens R, Templin M, et al. Impact of recipient twin preoperative myocardial performance index in twin-twin transfusion syndrome treated with laser. J Matern Fetal Neonatal Med. 2017;30(7):767-771.

Gil Guevara E, Pazos A, Gonzalez O, et al. Doppler assessment of patients with twin-to-twin transfusion syndrome and survival following fetoscopic laser surgery. Int J Gynaecol Obstet. 2017;137(3):241-245.

Graeve P, Banek C, Stegmann-Woessner G, et al. Neurodevelopmental outcome at 6 years of age after intrauterine laser therapy for twin-twin transfusion syndrome. Acta Paediatr. 2012;101:1200-1205.

Gray PH, Ward C, Chan FY. Cardiac outcomes of hydrops as a result of twin-twin transfusion syndrome treated with laser surgery. J Paediatr Child Health. 2009;45(1-2):48-52.

Habli M, Lim FY, Crombleholme T. Twin-to-twin transfusion syndrome: a comprehensive update. Clin Perinatol. 2009;36(2):391-416.

Hayes, Inc. Hayes Medical Technology Directory. Fetoscopic laser surgery for twin-twin transfusion syndrome. June 2002. Report archived Jan 01, 2008.

Illagan JG, Wilson RD, Bebbington MW, et al. Pregnancy outcomes following bipolar umbilical cord cauterization for selective termination in complicated monochorionic multiple gestations. Fetal Diag and Therapy. 2008;23:153-158.

Johnson A, Papanna R. Twin-twin transfusion syndrome: management and outcome. 05/08/2017. UpToDate. [UpToDate Web site]. http://www.uptodate.com/home/index.html. [via subscription only]. Accessed January 9, 2018.

Khalek N, Johnson MP. Management of prenatally diagnosed lung lesions. Semin Pediatr Surg. 2013;22(1):24-9.

Khalek N, Johnson MP, Bebbington MW. Fetoscopic laser therapy for twin-to-twin transfusion syndrome. Semin Pediatr Surg. 2013;22:18-23.

Kuller JA, Norton ME. Expanding the gestational age limits for laser treatment for twin-twin transfusion. Editorials. Am J Obstet Gynecol. 2013;208:165-166.

Kumar S, Paramasivam G, Zhang E, et al. Perinatal- and procedure-related outcomes following radiofrequency ablation in monochorionic pregnancy. Am J Obstet Gynecol. 2014;210(5):454.

Lopriore E, Nagel HT, Vandenbussche FP, Walther FJ. Long-term neurodevelopmental outcome in twin-to-twin transfusion syndrome. Am J Obstet Gynecol. 2003;189(5):1314-1319.

Malshe A, Snowise S, Mann LK, et al. Preterm delivery after fetoscopic laser surgery for twin-twin transfusion syndrome: etiology and risk factors. Ultrasound Obstet Gynecol. 2017;49(5):612-616.

Maschke C, Diemert A, Hecher K, et al. Long-term outcome after intrauterine laser treatment for twin-twin transfusion syndrome. Prenat Diagn. 2011;31:647-653.

Merz W, Tchatcheva K, Gembrush U, et al. Maternal complications of fetoscopic laser photocoagulation (FLP) for treatment of twin-twin transfusion syndrome (TTTS). J Perinat Med. 2010;38:439-443.

Mosquera C, Miller RS, Simpson LL. Twin-twin transfusion syndrome. Semin Perinatol. 2012;36:182-189.

Odibo AO, Caughey AB, Grobman W, et al. Selective laser photocoagulation versus serial amniodrainage for the treatment of twin-twin transfusion syndrome: a cost-effectiveness analysis. J Perinatol. 2009;29(8):543-7.

Oliver ER, Coleman BG, Goff DA, et al. Twin reversed arterial perfusion sequence: a new method of parabiotic twin mass estimation correlated with pump twin compromise. J Ultrasound Med. 2013;32(12):2115-23.

Patel S, Randolph LM, Benirschke K. Prevalence of noncardiac structural anomalies in twin-twin transfusion syndrome. J Ultrasound Med. 2012;31:550-560.

Peralta CF, Molina FS, Gómez LF, et al. Endoscopic laser dichorionization of the placenta in the treatment of severe twin-twin transfusion syndrome. Fetal Diagn Ther. 2013;34(4):206-10.

Pruetz JD, Schrager SM, Wang TV, et al. Blood pressure evaluation in children treated with laser surgery for twin-twin transfusion syndrome at 2-year follow-up. Am J Obstet Gynecol. 2015;213:417.e1-7.

Roberts D, Gates S, Kilby M, Neilson JP. Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol. 2008;31(6):701-711.

Roberts D, et al. Interventions for the treatment of twin-twin transfusion syndrome. Cochrane Database Syst Rev. 2014;30: 1:CD002073.

Roberts D, Gates S, Kilby M, Neilson JP. Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol. 2008;31(6):701-711.

Rossi AC. Treatment of twin-twin transfusion syndrome: septostomy. Medscape website. Available at: http://www.medscape.org/viewarticle/772183_3. Accessed January 9, 2018.

Rossi AC, D’Addario V. Survival outcomes of twin-twin transfusion syndrome stage I: a systematic review of literature. Am J Perinalol. 2013;30:5-10.

Rossi AC, Vanderbilt D, Chmait RH. Neurodevelopmental outcomes after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 2011;118:1145-1150.

Ruano R, Rodo C, Peiro JL, et al. Fetoscopic laser ablation of placental anastomoses in twin-twin transfusion syndrome using 'Solomon technique'. Ultrasound Obstet Gynecol. 2013;42(4):434-9.

Salomon LJ, Ortqvist L, Aegerter P, et al. Long-term developmental follow-up of infants who participated in a randomized clinical trial of amniocentesis vs. laser photocoagulation for the treatment of twin-to-twin transfusion syndrome. Am J Obstet Gynecol. 2010;203:444e1-444e7.

Sananès N, Gabriele V, Weingertner AS, et al. Evaluation of long-term neurodevelopment in twin-twin transfusion syndrome after laser therapy. Prenat Diagn. 2016;36(12):1139-1145.

Senat MV, Deprest J, Boulvain M, et al. Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med. 2004;351:1360144.

Skupski DW, Gurushanthaiah K, Chasen S. The effect of treatment of twin-twin transfusion syndrome on the diagnosis-to-delivery interval. Twin Res. 2002;5(1):1-4.

Skupski DW, Luks FI, Walker M, et al. Preoperative predictors of death in twin-to-twin transfusion syndrome treated with laser ablation of placental anastomoses. Am J Obstet Gynecol. 2010;203(4):388.e1-388.e11.

Slaghekke F, Lewi L, Middeldorp JM, et al. Residual anastomoses in twin-twin transfusion syndrome after laser: the Solomon randomized trial. Am J Obstet Gynecol. 2014;211(3):285.e1-7.

Slaghekke F, Lopriore E, Lewi L, et al. Fetoscopic laser coagulation of the vascular equator versus selective coagulation for twin-to-twin transfusion syndrome: an open-label randomised controlled trial. Lancet. 2014;383(9935):2144-51.

Snowise S, Mann LK, Moise KJ Jr, et al. Preterm prelabor rupture of membranes after fetoscopic laser surgery for twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 2017;49(5):607-611.

Society for Maternal-Fetal Medicine. Twin-twin transfusion syndrome. Am J Obstet Gynecol. 2013;208:3-18. Reaffirmed August 1, 2014. Available at: http://www.ajog.org/article/S0002-9378(12)01980-1/fulltext. Accessed January 9, 2018.

Spruijt M, Steggerda S, Rath M, et al. Cerebral injury in twin-twin transfusion syndrome treated with fetoscopic laser surgery. Obstet Gynecol. 2012;120:15-20.

Swiatkowska-Freund M, Pankrac Z, Preis K. Results of laser therapy in twin-to-twin transfusion syndrome: our experience. J Matern Fetal Neonatal Med. 2012;25(10):1917-20.

Szaflik K, Nowak P, Bielak A, et al. Treatment of twin to twin transfusion syndrome – comparison of two therapeutic methods – amnioreduction and laser therapy. Ginekol Pol. 2013;84:24-31.

Tosello B, Blanc J, Haumonté JB, et al. Short and medium-term outcomes of live-born twins after fetoscopic laser therapy for twin-twin transfusion syndrome. J Perinat Med. 2014;42(1):99-105.

Townsend CM Jr, Beauchamp RD, Evers BM, et al. Sabiston Textbook of Surgery. 19th ed. St Louis, MO: Elsevier; 2012.

Twin to Twin Transfusion Syndrome Foundation. What are the available treatments for TTTS? Available at: http://www.tttsfoundation.org/help_during_pregnancy/what_are_the_treatments.php. Accessed January 9, 2018.

Vanderbilt DL, Schrager SM, Llanes A, et al. Predictors of 2-year cognitive performance after laser surgery for twin-twin transfusion syndrome. Am J Obstet Gynecol. 2014;211(4):388.e1-7.

Vanderbilt DL, Schrager SM, Llanes A, et al. Prevalence and risk factors of cerebral lesions in neonates after laser surgery for twin-twin transfusion syndrome. Am J Obstet Gynecol. 2012;207;320.e1-6.

Van Klink JM, Koopman HM, van Zwet EW, et al. Cerebral injury and neurodevelopmental impairment after amnioreduction versus laser surgery in twin-twin transfusion syndrome: a systematic review and meta-analysis. Fetal Diagn Ther. 2013;33:81-89.

Walsh CA, McAuliffe FM. Recurrent twin-to-twin transfusion syndrome (TTTS) after selective fetoscopic laser photocoagulation: a systematic review of the literature. Ultrasound Obstet Gynecol. 2012;40: 503-512.

Wee LY, Fisk NM. The twin-twin transfusion syndrome. Semin Neonatol. 2002;7(3):187-202.

Weisz B, Hoffmann C, Ben-Baruch S, et al. Early detection by diffusion-weighted sequence magnetic resonance imaging of severe brain lesions after fetoscopic laser coagulation for twin twin transfusion syndrome. Ultrasound Obstet Gynecol. 2014;44(1):44-9.

Wilson RD, Johnson A, Ryan G. Current controversies in prenatal diagnosis 2: Should laser ablation of placental anastomoses be used in all cases of twin to twin transfusion? Prenat Diagn. 2009;29(1):6-10.

Xianggun L, Morokuma S, Fukushima K, et al. Prognosis and long-term neurodevelopmental outcome in conservatively treated twin-to-twin transfusion syndrome. BMC Pregnancy and Childbirth. 2011;32.

Zack T and Ramus, R. Twin-to-Twin Transfusion Syndrome. [E-Medicine Web site]. 01/29/10. Updated 12/30/2015. Available at: http://emedicine.medscape.com/article/271752-overview. Accessed January 9, 2018.




Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

59001, 59072, 76998


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

O43.021 Fetus-to-fetus placental transfusion syndrome, first trimester

O43.022 Fetus-to-fetus placental transfusion syndrome, second trimester

O43.023 Fetus-to-fetus placental transfusion syndrome, third trimester



HCPCS Level II Code Number(s)

S2411 Fetoscopic laser therapy for treatment of twin-twin transfusion syndrome


Revenue Code Number(s)

N/A

Coding and Billing Requirements


Cross References


Policy History

Revisions from 11.00.14e:
02/15/2018This policy has undergone a routine review, and no revisions have been made.
Version Effective Date: 04/20/2016
Version Issued Date: 04/20/2016
Version Reissued Date: 02/15/2018

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