Sunflower Health Plan Fetal Surgery in Utero for Prenatally Diagnosed Malformations (PDF) Form

This policy describes the medical necessity requirements for performing fetal surgery. This becomes an option when it is predicted that there will be severe disability, or if it is predicted that the fetus will not live long enough to survive delivery or after birth.8 Therefore, surgical intervention during pregnancy on the fetus is meant to correct problems that would be too advanced to correct after birth. Policy/Criteria I. It is the policy of health plans affiliated with Centene Corporation® that in-utero fetal surgery (IUFS) is medically necessary for any of the following: A. Sacrococcygeal teratoma (SCT): SCT resection or a minimally invasive approach; B. Lower urinary tract obstruction without multiple fetal anomalies or chromosomal abnormalities: urinary decompression via vesico-amniotic shunting; C. Congenital pulmonary airway malformation (CPAM) and extralobar bronchopulmonary sequestration (BPS), with high risk tumors: resection of malformed pulmonary tissue, or placement of a thoraco-amniotic shunt; D. Placement of a thoraco-amniotic shunt for pleural effusion with or without secondary fetal hydrops; E. Twin-twin transfusion syndrome (TTTS): treatment approach is dependent on Quintero stage, maternal signs and symptoms, gestational age and the availability of requisite technical expertise and include either: 1. Amnioreduction; or 2. Fetoscopic laser ablation, with or without amnioreduction when pregnancy is between 16 and 26 weeks gestation; F. Twin-reversed-arterial-perfusion sequence (TRAP): ablation of anastomotic vessels of the acardiac twin (laser, radiofrequency ablation); G. Myelomeningocele: repair when all of the following criteria are met: 1. Singleton pregnancy; 2. Upper boundary of myelomeningocele located between T1 and S1; 3. Evidence of hindbrain herniation; 4. Gestational age 19.0 to < 26 weeks; 5. Normal fetal karyotype; 6. None of the following: a. Severe kyphosis; b. Risk of preterm birth (e.g., short cervix or previous preterm birth); c. Placental abruption; d. Maternal body mass index of ≥ 35; e. Previous hysterotomy in the active uterine segment. Page 1 of 9 CLINICAL POLICY Fetal Surgery in Utero II. It is the policy of health plans affiliated with Centene Corporation that all repeat utero fetal surgery procedures require secondary review. III. It is the policy of health plans affiliated with Centene Corporation that current evidence does not support the use of utero fetal surgery for any of the following indications: A. Open or endoscopic fetal surgery for congenital diaphragmatic hernia (CDH), including temporary tracheal occlusion; B. Surgery for heart block, pulmonary valve, or aortic obstruction; C. Tracheal atresia or stenosis; D. Cleft lip and palate; E. In-utero stem cell transplantation; F. In-utero gene therapy; G. Amnioexchange procedure for gastroschisis. Background Maternal–Fetal Surgery Maternal–fetal surgery is a major procedure for the mother and her fetus, and it has significant implications and complications that could occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. For the fetus, safety and effectiveness are variable, and depend on the specific procedure, the reasons for the procedure, and the gestational age and condition of the fetus. Often babies who have been operated on in this manner are born pre-term. Therefore, it should only be offered at facilities with the expertise, multidisciplinary teams, services, and facilities to provide the intensive care required for these patients.8 Fetal surgery approaches can be divided into two categories: • Open fetal surgery is considered when the fetal condition is life threatening, and the intervention is felt to be the only option for fetal survival. A hysterotomy is performed, the fetus is partially removed to expose the area that needs surgery, the fetal abnormality is corrected, and the fetus is returned to the uterus where it continues to develop until delivery. • Fetoscopic surgery employs minimally invasive techniques and uses small fiberoptic telescopes and instruments to enter the uterus through small surgical openings to correct congenital malformations without major incisions or removing the fetus from the womb. This interim procedure is less traumatic, reduces the chances of preterm labor, and allows the fetus to remain in utero until it has matured enough to survive delivery and neonatal surgical procedures. Sacrococcygeal germ cell tumors The prenatal diagnosis of sacrococcygeal teratoma (SCT) typically occurs during the second trimester during routine sonography. Prenatal diagnosis and close monitoring have improved outcomes for fetal SCT, but overall perinatal mortality remains high. The major goal is to identify fetuses at increased risk of fetal demise because of hydrops fetalis and intervene as appropriate. Hydrops fetalis is a condition of excess fluid accumulation in the fetus that results in significant fetal demise and neonatal mortality. Criteria for open fetal surgery varies, but most centers include fetuses with high-risk SCT and hydrops that have developed at a gestational age too early for appropriate delivery and neonatal care. Type III or IV Altman type tumors, severe Page 2 of 9 CLINICAL POLICY Fetal Surgery in Utero placentomegaly, cervical shortening, and maternal medical issues are all contraindications for open fetal surgery for SCT.9 Lower Urinary Tract Obstruction The prenatal diagnosis of lower urinary tract obstructions typically occurs during the first or second trimester during routine sonography. Outcomes range from clinically insignificant to in- utero fetal demise. Vesicoamniotic shunts can be a temporizing measure and provide a survival advantage in a select cohort of fetuses with urinary tract obstruction.19 Congenital pulmonary airway malformation (CPAM) CPAM is one of the most common lung lesions diagnosed prenatally, although the birth prevalence is quite low. Prenatal diagnosis is typically made by ultrasonography. CPAMs presenting prenatally are classified as macrocystic or microcystic based on ultrasound appearance. Approximately 50% of the masses resolve before delivery, while the remainder persists until delivery. Hydrops can develop with either micro or macrocystic lesions due to compression of lymphatic structures or due to hemodynamic alterations from vena cava obstruction or cardiac displacement/compression. The presence of hydrops is a sign for impending fetal demise (risk of death approaches 100% in the absence of intervention), and thus it is an indication for fetal intervention. For hydropic fetuses over 32 to 34 weeks of gestation, early delivery with immediate postnatal resection is a reasonable option. Ex utero intrapartum therapy (EXIT) has been used to stabilize fetuses with large lesions expected to have difficulty breathing at delivery. In EXIT, the fetus is partially delivered and intubated without clamping the umbilical cord. Uteroplacental blood flow and gas exchange are maintained by using inhalational agents to provide uterine relaxation and amnioinfusion to maintain uterine volume. This provides time for resection of the lung mass prior to complete delivery of the infant. For hydropic fetuses between 20 and 32 weeks of gestation, the choice of the best invasive approach depends on the type of anomaly (macro- versus microcystic). Drainage procedures are used for CPAMS with dominant cysts, while solid masses are treated by resection or ablation.10 Twin-twin transfusion syndrome (TTTS) TTTS occurs in approximately 10–15% of monochorionic–diamniotic twin pregnancies and results from the presence of arteriovenous anastomoses in a monochorionic placenta. In the affected pregnancy, there is an imbalance in the fetal–placental circulations, whereby one twin transfuses the other. It usually presents in the second trimester. Once the diagnosis of TTTS has been made, the prognosis depends on gestational age and severity of the syndrome. Staging is commonly performed via the Quintero staging system, and treatment is by laser coagulation or amnioreduction, often in collaboration with an expert in TTTS diagnosis and management.18 Twin reversed arterial perfusion (TRAP) TRAP sequence is a rare unique serious complication of monochorionic twin pregnancy in which a twin with an absent or a nonfunctioning heart (“acardiac twin”) is perfused by its co-twin ("pump twin") via placental arterial anastomoses. The acardiac twin usually has a poorly developed heart, upper body, and head. The pump twin is at risk of heart failure and problems related to preterm birth. Current treatment modalities target occlusion of the umbilical cord of Page 3 of 9 CLINICAL POLICY Fetal Surgery in Utero the acardiac twin and include laser coagulation, bipolar cord coagulation, and radiofrequency ablation.12 Myelomeningocele Per the American College of Obstetricians and Gynecologists (ACOG) and the Society for Maternal–Fetal Medicine (SMFM), open maternal–fetal surgery for myelomeningocele repair has shown improvement in pediatric outcomes, but poses procedure-associated maternal and fetal risks. According to ACOG and SMFM recommendations for myelomeningocele repair, women who meet specific criteria for in utero repair should be counseled about all management options, including open maternal-fetal surgery. A referral for additional assessment and consultation to a fetal therapy center should be completed for candidates interested in fetal myelomeningocele repair. These centers have the expertise, resources, and multi-disciplinary team to provide the information and intensive care needed for patients choosing to undergo open maternal-fetal surgery.8 Coding Implications This clinical policy references Current Procedural Terminology (CPT®). CPT® is a registered trademark of the American Medical Association. All CPT codes and descriptions are copyrighted 2020, American Medical Association. All rights reserved. CPT codes and CPT descriptions are from the current manuals and those included herein are not intended to be all-inclusive and are included for informational purposes only. Codes referenced in this clinical policy are for informational purposes only. Inclusion or exclusion of any codes does not guarantee coverage. Providers should reference the most up-to-date sources of professional coding guidance prior to the submission of claims for reimbursement of covered services. CPT® Codes 59001 59076 59897 HCPCS Codes S2401 S2402 S2403 S2404 S2405 S2409 S2411 Amniocentesis; therapeutic amniotic fluid reduction (includes ultrasound guidance) Fetal shunt placement, including ultrasound guidance Unlisted fetal invasive procedure, including ultrasound guidance, when performed Repair, urinary tract obstruction in the fetus, procedure performed in utero Repair, congenital cystic adenomatoid malformation in the fetus, procedure performed in utero Repair, extralobar pulmonary sequestration in the fetus, procedure performed in utero Repair, myelomeningocele in the fetus, procedure performed in utero Repair of sacrococcygeal teratoma in the fetus, procedure performed in utero Repair congenital malformation of fetus, procedure performed in utero, not otherwise classified Fetoscopic laser therapy for treatment of twin-to-twin transfusion ICD-10-CM Diagnosis Codes that Support Coverage Criteria ICD 10 CM Code Neoplasm of uncertain behavior of spinal cord D43.4 - - Page 4 of 9 Conjoined twin pregnancy [twin reversed arterial perfusion (TRAP)] Maternal care for known or suspected fetal abnormality and damage CLINICAL POLICY Fetal Surgery in Utero - Maternal care for hydrops fetalis Twin pregnancy, monochorionic/diamniotic Fetus-to-fetus placental transfusion syndrome ICD 10 CM Code - O30.021 through O30.029 O31.031through O31.039 O35.0XX0 through O35.9XX9 O36.20X0 through O36.23X9 O43.021 through O43.029 Q05.0 through Q05.9 Q33.0 Q33.2 Q33.3 Q33.6 Q34.0 through Q34.9 Other congenital malformations of respiratory system Q62.31 through Q62.39 Q64.2 Q64.31through Q64.39 Q89.4 Q89.8 Spina Bifida Congenital cystic lung Sequestration of lung Agenesis of lung Congenital hypoplasia and dysplasia of lung Congenital posterior urethral valves Other atresia and stenosis of urethra and bladder neck Conjoined twins Other specified congenital malformations Other obstructive defects of renal pelvis and ureter Reviews, Revisions, and Approvals Policy adopted from HN NMP344 Fetal Surgery in Utero for Prenatally Diagnosed Malformations. Removed gestational age requirements from sections: IA.1, treatment of sacrococcygeal teratoma and IA.3 congenital pulmonary airway malformation or bronchopulmonary sequestration. Removed specific criteria from IA.2, Vesico-amniotic shunting as a treatment of lower urinary tract obstruction to allow procedure per the discretion of the treating surgeon. Updated background with more recent recommendations from ACOG committee opinion. Code updates. Reworded section I.A# 1-5 for clarification. Removed EXIT procedure from I.A. as the procedure is an “ex utero procedure.” Updated background information. Removed I.B,to defer to the discretion of the treating surgeon. References reviewed and updated. Myelomeningocele repair: clarified that “no history of previous hysterotomy in the active uterine segment should be “history of previous hysterotomy in the active uterine segment. Revision Date 09/16 Approval Date 10/16 09/17 10/17 08/18 08/18 10/18 Page 5 of 9 CLINICAL POLICY Fetal Surgery in Utero Reviews, Revisions, and Approvals SCT: removed requirement for hydrops and included option for minimally invasive approach. CPAM/BPS: removed requirement for hydrops. Specialist review. References reviewed and updated. Annual review. References reviewed and updated. Coding reviewed. Changed “review date” in the header to “date of last revision” and “date” in the revision log header to “revision date." Replaced all instance of “member” with “member/enrollee.” Added, “D. Placement of a thoraco- amniotic shunt for pleural effusion with or without secondary fetal hydrops,” to criteria set I. Added criteria set, “II. It is the policy of health plans affiliated with Centene Corporation that all repeat utero fetal surgery procedures require secondary review.” Reviewed by specialist. Annual review. updated with no impact on criteria. Background updated with no impact on criteria. References reviewed and updated. Revision Date 08/19 Approval Date 08/19 07/20 07/21 07/20 07/21 07/22 07/22