DESCRIPTION
Fetal surgery is used for specific congenital abnormalities associated with a poor postnatal prognosis. Prenatal surgery involves either opening the gravid uterus (with a Cesarean surgical incision) to correct the abnormality, or through a single or multiple fetoscopic port incision. This policy addresses fetal surgery performed for the following clinical conditions:l
Fetal Urinary Tract Obstruction
Although few cases of prenatally diagnosed urinary tract obstruction require prenatal intervention, bilateral obstruction can lead to distention of the urinary bladder and is often associated with serious disease such as pulmonary hypoplasia secondary to oligohydramnios. Fetuses with bilateral obstruction, oligohydramnios, adequate renal function reserve, and no other lethal or chromosomal abnormalities may be candidates for fetal surgery. The most common surgical approach is decompression through percutaneous placement of a shunt or stent. Vesicoamniotic shunting bypasses the obstructed urinary tract, permitting fetal urine to flow into the amniotic space. The goals of shunting are to protect the kidneys from increased pressure in the collecting system and to assure adequate amniotic fluid volume for lung development.
Congenital Cystic Adenomatoid Malformation (CCAM) or Bronchopulmonary Sequestration (BPS)
CCAM and BPS are the two most common congenital cystic lung lesions and share the characteristic of a segment of lung being replaced by abnormally developing tissue. CCAMs can have connections to the pulmonary tree and contain air, while BPS does not connect to the airway and has blood flow from the aorta rather than the pulmonary circulation. CCAM lesions typically increase in size in mid-trimester and then in the third trimester either involute or compress the fetal thorax, resulting in hydrops in the infant and sometimes mirror syndrome (a severe form of pre-eclampsia) in the mother. Mortality is close to 100% when lesions are associated with fetal hydrops (abnormal accumulation of fluid in two or more fetal compartments). These individuals may be candidates for prenatal surgical resection of a large mass or placement of a thoracoamniotic shunt to decompress the lesion.
Sacrococcygeal Teratoma
Sacrococcygeal teratoma (SCT) is a rare tumor that develops at the base of the spine. Large sacrococcygeal teratomas can cause a variety of complications before and after birth. They can grow rapidly in the fetus and require very high blood flow resulting in fetal heart failure, a condition known as hydrops.
Myelomeningocele
Myelomeningocele occurs when abnormal closure of the spinal cord results in exposure of the meninges and neural tube to the intrauterine environment. This neural tube defect results in varying degrees of deformities and functional disabilities (e.g., spine, limbs, bladder, bowel, sexual dysfunction, learning disabilities and neurologic deformities). Although the exact cause of the neurologic deficits is unknown, possible cause is attributed to either the primary defect in closure of the neural tube or secondary injury to exposed neural tissue throughout gestation by amniotic fluid and mechanical trauma.
Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) results from abnormal development of the diaphragm, which permits abdominal viscera to enter the chest, frequently resulting in hypoplasia of the lungs. CDH can vary widely in severity, depending on the size of the hernia and the timing of herniation. Fetal surgery for CDH originally involved open fetal surgery and repair of the diaphragmatic defect; however, as advances were made in neonatal care, the postnatal survival rate among babies undergoing standard care (without fetal intervention) improved. Temporary tracheal occlusion or fetal endoscopic tracheal occlusion (FETO) is being investigated for the treatment of congenital diaphragmatic hernia. This procedure uses a small balloon to occlude the trachea, resulting in a build-up of secretions in the pulmonary tree. This purportedly increases the size of the lungs and gradually pushes abdominal viscera out of the chest cavity and into the abdominal cavity. Additional study with longer follow-up is also needed to evaluate morbidity (e.g., neurologic and pulmonary outcomes) in survivors for the FETO procedure.
Cardiac Malformations
In utero interventions are being investigated for several potentially lethal congenital heart disorders, including aortic stenosis with hypoplastic left heart syndrome (HLHS), and pulmonary stenosis or pulmonary atresia. In utero aortic balloon valvuloplasty has been suggested as a way to relieve aortic stenosis in an attempt to preserve left ventricular growth and halt the progression to HLHS. Evidence related to fetal interventions for congenital heart defects is limited. Although postnatal repair/correction of these severe cardiac defects is associated with very high morbidity and mortality, further studies are needed to demonstrate that health outcomes are improved with fetal interventions.
POLICY
Fetal surgery for prenatally diagnosed malformations is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Fetal surgery for the treatment of other conditions/diseases, including, but not limited to, the following is considered investigational:
Temporary tracheal occlusion as a treatment of congenital diaphragmatic hernia
Congenital heart defect
MEDICAL APPROPRIATENESS
Fetal surgeries for prenatally diagnosed malformations are considered medically appropriate for ANY ONE of the following indications:
Vesicoamniotic shunting for the treatment of urinary tract obstruction if ALL of the following criteria are met:
Evidence of hydronephrosis due to bilateral urinary tract obstruction
Progressive oligohydramnios
Adequate renal function
Absence of other lethal abnormalities or chromosomal defects
Open in utero resection or placement of thoracoamniotic shunt for the treatment of malformed pulmonary tissue if ALL of the following criteria are met:
Congenital cystic adenomatoid malformation or bronchopulmonary sequestration is identified
The fetus is at 32 weeks’ gestation or less
There is evidence of ANY ONE of the following:
Fetal hydrops
Placentomegaly
Symptoms of severe preeclampsia (i.e., the maternal mirror syndrome) in the mother
In utero removal of sacrococcygeal teratoma if ALL of the following criteria are met:
The fetus is at 32 weeks’ gestation or less
There is evidence of ANY ONE of the following:
Fetal hydrops
Placentomegaly
Symptoms of severe preeclampsia (i.e., maternal mirror syndrome) in the mother
In utero repair of myelomeningocele if ALL of the following criteria are met:
The fetus is less than 26 weeks’ gestation
Upper boundary located between T1 and S1 with evidence of hindbrain herniation
ABSENCE of ALL the following:
Other fetal anomaly unrelated to myelomeningocele
Severe fetal kyphosis
Risk of preterm birth (e.g., short cervix or previous preterm birth)
Maternal body mass index greater than or equal to 35 kg/m2
IMPORTANT REMINDERS
Any specific products referenced in this policy are just examples and are intended for illustrative purposes only. It is not intended to be a recommendation of one product over another and is not intended to represent a complete listing of all products available. These examples are contained in the parenthetical e.g., statement.
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the medical policy and a health plan or government program (e.g., TennCare), the express terms of the health plan or government program will govern.
ADDITIONAL INFORMATION
For indications considered investigational, additional studies are needed to identify appropriate candidates and to evaluate longer term outcomes compared with postnatal management.
SOURCES
American College of Obstetricians and Gynecologists / Society for Maternal-Fetal Medicine. (2017, September; reaffirmed 2022). ACOG committee opinion #720: maternal-fetal surgery for myelomeningocele. Retrieved October 30, 2015 from http://www.acog.org.
Araujo, J., Tonni, G., Chung, M., Ruano, R., & Martins, W. (2016). Perinatal outcomes and intrauterine complications following fetal intervention for congenital heart disease: systematic review and meta-analysis of observational studies. Ultrasound in Obstetrics and Gynecology, 48 (4), 426-433. (Level 2 evidence)
Deprest, J., Brady, P., Nicolaides, K., Benachi, A., Berg, C., Vermeesch, J., et al. (2014). Prenatal management of the fetus with isolated congenital diaphragmatic hernia in the era of the TOTAL trial. Seminars in Fetal & Neonatal Medicine, 19 (6), 338-348. Abstract retrieved October 29, 2015 from PubMed database.
European Reference Network for Rare Kidney Diseases. (2022, February). Consensus statement: definition, diagnosis, and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group. Retrieved February 23, 2023 from http://www.erknet.org/guidelines-pathways/obstructive-uropathies/fetal-lower-urinary-tract-obstruction.
Grivell, R., Anderson, C., Dodd, J. (2015, November). Prenatal interventions for congenital diaphragmatic hernia for improving outcomes. The Cochrane Database Systematic Reviews, 2015 (11), CD008925, doi: 10.1002/14651858.pub2. (Level 2 evidence)
Jeong, B., Won, H., Lee, M., Shim, J, Lee, P., & Kim, A. (2015). Perinatal outcomes of fetal pleural effusion following thoracoamniotic shunting. Prenatal Diagnosis, 35 (13), 1365-1370, doi: 10.1002/pd.4709. (Level 4 evidence)
Kabagambe, S., Jensen, G., Chen, Y., Vanover, M., & Farmer, D. (2018). Fetal surgery for myelomeningocele: a systematic review and meta-analysis of outcomes in fetoscopic versus open repair. Fetal Diagnosis and Therapy, 43 (3), 161-174, doi: 10.1159/000479505. (Level 2 evidence)
Litwińska, M., Litwińska, E., Janiak, K., Piaseczna-Piotrowska, A., & Szaflik, K. (2020). Percutaneous intratumor laser ablation for fetal sacrococcygeal teratoma. Fetal Diagnosis and Therapy, 47 (2), 138–144, doi: 10.1159/000500775. Abstract retrieved February 23, 2023 from PubMed database.
Morris, R. & Malin, G. (2013). Percutaneous vesicoamniotic shunting versus conservative management for fetal lower urinary tract obstruction (PLUTO): a randomized trial. Lancet, 382 (9903), 1496-1506. (Level 2 evidence)
National Institute for Health and Care Excellence. (2006, December; last update December 2022). Fetal vesico-amniotic shunt for lower urinary tract outflow obstruction. Retrieved February 22, 2023 from https://www.nice.org.uk/guidance/.
National Institute for Health and Care Excellence. (2018, May). Percutaneous balloon valvuloplasty for fetal critical aortic stenosis. Retrieved January 11, 2022 from https://www.nice.org.uk/guidance/.
National Institute for Health and Care Excellence. (2020, January). Fetoscopic prenatal repair for open neural tube defects in the fetus. Retrieved March 30, 2021 from https://www.nice.org.uk/guidance/ipg667.
Peranteau, W., Adzick, N., Boelig, M., Flake, A., Hedrick, H., Howell, L., et al. (2015). Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases. Journal of Pediatric Surgery, 50 (2), 301-305. Abstract retrieved October 9, 2017 from PubMed database.
Ruano, R., Sananes, N., Sangi-Haghpeykar, H., Hernandez-Ruano, S., Moog, R., Becmeur, F., et al. (2015). Fetal intervention for severe lower urinary tract obstruction: a multicenter case-control study comparing fetal cystoscopy with vesicoamniotic shunting. Ultrasound in Obstetrics and Gynecology, 45 (4), 452-458. (Level 4 evidence)
Van Mieghem, T., Al-Ibrahim, A., Deprest, J., Lewi, L., Langer, J.C., Baud, D., et al. (2014). Minimally invasive therapy for fetal sacrococcygeal teratoma: case series and systematic review of the literature. Ultrasound in Obstetrics and Gynecology, 43(6), 611-619. (Level 2 evidence)
Winifred S. Hayes, Inc. Medical Technology Directory. (2018, July; last update search August 2022). Fetal surgery for congenital diaphragmatic hernia. Retrieved February 22, 2023 from www.hayesinc.com/subscribers. (23 articles and/or guidelines reviewed)
Winifred S. Hayes, Inc. Medical Technology Directory. (2018, July; last update search July 2022). Fetal surgery for myelomeningocele. Retrieved February 22, 2023 from www.hayesinc.com/subscribers, (33 articles and/or guidelines reviewed)
ORIGINAL EFFECTIVE DATE: 7/14/2007
MOST RECENT REVIEW DATE: 6/13/2024
ID_BT
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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