BlueCross BlueShield of Tennessee Medical Policy Manual

Fetal Surgery for Prenatally Diagnosed Malformations

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. Fetal surgery for many congenital conditions, including congenital diaphragmatic hernia (CDH) and heart defects, has not been shown to improve health outcomes compared with postnatal treatment.

This policy addresses fetal surgery performed for the following clinical conditions:

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 the most common tumor of the newborn and generally carries a good prognosis in infants born at term. However, in utero fetal mortality approaches 100% with large or vascular tumors, which may become larger than the rest of the fetus. In this small subset, SCT is associated with fetal hydrops, which is related to high output heart failure secondary to arteriovenous shunting.

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

MEDICAL APPROPRIATENESS

IMPORTANT REMINDERS

ADDITIONAL INFORMATION

There is a lack of published evidence based studies to demonstrate that fetal tracheal occlusion and aortic valvuloplasty provide improved health outcomes. For these and other applications of fetal surgery that are currently considered investigational, additional studies are needed to identify appropriate candidates and to evaluate longer term outcomes compared with postnatal management.

SOURCES

Agency for Healthcare Research and Quality (AHRQ). (2011). Technical Brief #5: Maternal-fetal surgical procedures. Retrieved May 30, 2014 from http://www.effectivehealthcare.ahrq.gov

American College of Obstetricians and Gynecologists, Committee on Ethics & American Academy of Pediatrics Committee on Bioethics (2011, August). Maternal - fetal intervention and fetal care centers. Retrieved May 30, 2014 from http://www.acog.org/.

American College of Obstetricians and Gynecologists. (2003; reaffirmed 2013). ACOG practice bulletin. Neural tube defects. Retrieved May 30, 2014 from http://www.guideline.gov.

American College of Obstetricians and Gynecologists. (2013; reaffirmed 2015). Committee on Obstetric Practice. 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. Abstract retrieved October 6, 2017 from PubMed database.

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.

Grivell, R., Anderson, C., Dodd, J. (2015, November) Prenatal interventions for congenital diaphragmatic hernia for improving outcomes. Cochrane Database Systemic Review. Retrieved October 13, 2016 Abstract from PubMed database.

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. Abstract retrieved October 9, 2017 from PubMed database.

Kabagambe, S., Jensen, G., Chen, Y., Vanover, M., & Farmer, D. (2017). Fetal surgery for myelomeningocele: a systematic review and meta-analysis of outcomes in fetoscopic versus open repair. Fetal Diagnosis and Therapy, doi: 10.1159/000479505. [Epub ahead of print]. Abstract retrieved October 6, 2017 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 (2011, January) Thoracoscopic repair of congenital diaphragmatic hernia in neonates. Retrieved October 12, 2016 from http://www.nice.org.uk/guidance/ipg379. 

National Institute for Health and Care Excellence. (2006; reaffirmed 2012). Percutaneous fetal balloon valvuloplasty for pulmonary atresia with intact ventricular septum. Retrieved October 6, 2017 from https://www.nice.org.uk/guidance/ipg176.

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. Abstract retrieved October 9, 2017 from PubMed database.

Winifred S. Hayes, Inc. Medical Technology Directory. (2012, October; last update search August 2016). In utero fetal surgery for congenital diaphragmatic hernia. Retrieved October 29, 2015 from: www.hayesinc.com.  (117 articles and/or guidelines reviewed)

Winifred S. Hayes, Inc. Medical Technology Directory. (2012, October; last update search August 2016). In utero fetal surgery for myelomeningocele. Retrieved October 13, 2016 from: www.hayesinc.com.  (111 articles and/or guidelines reviewed)

ORIGINAL EFFECTIVE DATE:  7/14/2007

MOST RECENT REVIEW DATE:  10/26/2017

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.

This document has been classified as public information.