Aetna considers in utero fetal surgery medically necessary for any of the following indications:
Ablation of anastomotic vessels in acardiac twins;
Insertion of pleuro-amniotic shunt for fetal pleural effusion;
Laser ablation of anastomotic vessels in early, severe twin-twin transfusion syndrome;
Removal of sacrococcygeal teratoma;
Repair of myelomeningocele;
Resection of malformed pulmonary tissue, or placement of a thoraco-amniotic shunt as a treatment of either of the following:
Congenital cystic adenomatoid malformation; or
Extralobar pulmonary sequestration;
Vesico-amniotic shunting as a treatment of urinary tract obstruction.
Aetna considers the following applications of in utero fetal surgery experimental and investigational because its effectiveness for these indications has not been established:
Fetal tracheal occlusion for congenital diaphragmatic hernia;
Treatment of congenital heart disease;
Treatment of gastroschisis.
Aetna considers in utero stem cell transplantation, in utero gene therapy, and other applications of in utero surgery experimental and investigational because their effectiveness has not been established.
Fetal surgery in-utero has been attempted for various congenital anomalies including congenital diaphragmatic hernia (CDH), spina bifida and urinary tract abnormalities.
Congenital diaphragmatic hernia is a defect in the diaphragm of a developing fetus, which results in abdominal viscera protrusion into the chest, displacing the lungs and heart in the thoracic cavity. Congenital diaphragmatic hernias are usually repaired after delivery; however, 2 primary methods for treating CDH in-utero have emerged in an attempt to overcome pulmonary hypoplasia and persistent pulmonary hypertension in infants who are more severely affected: (i) surgical repair of the herniated diaphragm, and (ii) ligation of the fetal trachea, with subsequent stimulation of lung growth.
Surgical treatment of spina bifida usually occurs within 24 hours of birth; however, in- utero repair has been used as a method to decrease nerve damage and improve outcomes at birth. Lower urinary tract obstruction has a significant impact on neonatal and child health. Pulmonary hyperplasia and renal impairment could be direct or indirect consequences of this condition leading to significant morbidity and mortality. Vesico-amniotic fetal shunting, open fetal surgery and more recently endoscopic fetal surgery for this condition are available as possible options of fetal intervention. Vesico-amniotic shunting has the advantage of bypassing the obstruction, however it is often associated with complications. Open fetal surgery is not usually recommended because of the complications and high fetal loss rate. Endoscopic surgery to visualize and treat the cause of lower urinary tract obstruction has been tried. Fetal endoscopic surgery is in its infancy and endoscopic procedures are limited to a few groups.
Twin-twin transfusion syndrome is the most common complication of monochorionic pregnancies affecting between 5 and 15 % of such pregnancies and accounts for 15 to 77 % of perinatal mortality in twins. Twin-twin transfusion syndrome is believed to occur as the result of uncompensated arteriovenous anastomoses in a monochorionic placenta, which lead to greater net blood flow going to one twin at the expense of the other. No single therapy is associated with a uniformly improved outcome for the involved twins and success is primarily related to gestational age and severity at diagnosis. A variety of therapies have been attempted, but serial therapeutic amniocenteses of the recipient twin's amniotic sac is most frequently used (ACOG, 2005). This therapy is believed to work by favorably changing intraamniotic pressure and, thus, placental intravascular pressure, allowing redistribution of placental blood flow and normalization of amniotic fluid volumes in each sac. More aggressive therapies, which usually are considered only for very early, severe cases, include abolishing the placental anastomoses by endoscopic laser coagulation or selective feticide by umbilical cord occlusion (ACOG, 2005). Clinical studies have shown that, in very early (less than 26 weeks gestation), severe cases of twin-twin transfusion syndrome, selective laser coagulation, when compared to serial amniocentesis, results in improved survival rates in at least one twin, less neurologic morbidity in survivors, and improved gestational age at time of delivery (Senat et al, 2004).
Twin reversed-arterial-perfusion (TRAP) sequence is a serious complication of monozygotic twin pregnancies, affecting 1% of monozygotic twins, or 1 in 35,000 births (James, 1997). It has been hypothesized that in the presence of artery-to-artery anastomoses in a monozygotic placenta, blood is perfused by the hemodynamically advantaged twin (“donor” or "pump" twin) to the other twin ("recipient" twin) by means of reversed arterial flow (Quintero et al, 1994; van Allen et al, 1984). Inadequate perfusion of the recipient twin is responsible for the development of a characteristic and invariably lethal set of anomalies, including the acardius fetal malformation (acardiac twins) and acephalus. Typically, the pump twin is structurally normal, but it is at risk for in-utero cardiac failure and without treatment dies in 50 to 75 % of cases, particularly if the recipient twin weighs more than half as much as the pump twin (Quintero et al, 1994).
Acardiac twinning is usually recognized by early fetal echocardiography. One approach to management is interruption of the vascular anastomosis between the donor and recipient twin. This is accomplished using endoscopic laser coagulation in pregnancies 24 weeks gestation or ligation of the umbilical cord using endoscopic or sonographic guidance at a greater gestational age (Arias et al, 1998). In a 1998 study of 7 pregnancies treated with laser therapy, the rate of death in the normal twin was 13.6 %, compared to an expected death rate reported in the literature of 50 % in the pump twin when the pregnancy is managed expectantly.
Another approach is the use of radiofrequency ablation to obliterate the blood supply of the acardiac twin. Tsao et al (2002) reported on the results of selective reduction of the abnormal twin in 13 consecutive cases of monochorionic twin gestation with TRAP sequence. The radiofrequency ablation needle was percutaneously inserted through the maternal abdominal wall into the intrauterine fetal abdomen at the level of the cord insertion site of the acardiac twin. The investigators reported that all 13 pump fetuses had been delivered, and that 12 of 13 infants are alive and well. One infant was delivered at 24.4 weeks gestation and subsequently died from complications of prematurity. The investigators reported that the average gestational age at delivery was 36.2 weeks.
Another approach to acardiac twins is expectant management. Although death rates of 50 % in the pump twin have been reported with expectant management of acardiac twins, Sullivan et al (2003) found that outcomes in expectantly managed cases may be better than reported due to increased antenatal diagnosis. Sullivan et al ascertained all cases of antenatally diagnosed acardiac twins delivered in the Salt Lake community between 1994 and 2001. All were managed expectantly. Of the 10 cases were identified, 9 women delivered a healthy pump twin. There was 1 neonatal death. The mean gestational age at delivery was 34.2 weeks. The mean weights of the pump and acardiac twins were 2,279 g and 1,372 g, respectively.
Other congenital anomalies that are amenable to in-utero treatment include myelomeningocele, cystadenomatoid malformation of the lung and saccrococygeal teratoma, shunts for uropathies and thoracic fluids.
In-utero hematopoietic stem cell transplantation is a promising approach for the treatment of a potentially large number of fetuses affected by congenital hematological disorders. Expansion of clinical application will depend on improved understanding of the biological barriers to engraftment in the fetus as well as on the development of effective clinical strategies based on the hematopoietic biology of individual disorders (Hayashi and Flake, 2001).
Findings of recent studies indicated that the effectiveness of in-utero approach in treating CDH has not been established. Downard and Wilson (2003) noted that antenatal maternal steroid administration and fetal surgery are not proven interventions for CDH. Adzick and Kitano (2003) stated that fetuses diagnosed with left CDH before 26 weeks' gestation with associated liver herniation and a low right lung to head circumference ratio have a reduced prognosis with conventional therapy after birth, but in-utero therapeutic approaches have yet to show a comparative survival benefit. Adzik and Kitano stated that a prospective randomized trial is required to critically evaluate the efficacy of fetal tracheal occlusion for severe diaphragmatic hernia. Heerma et al (2003) reported on comparative autopsy in 16 cases of congenital diaphragmatic hernia with fetal intervention (12 cases tracheal occlusion; 4 cases hernia repair) with 19 cases of congenital diaphragmatic hernia without fetal intervention. The investigators concluded that tracheal occlusion did not prevent development of lung pathology associated with pulmonary hypoplasia.
A prospective randomized controlled trial (RCT) of fetal tracheal occlusion for CDH found no differences in outcomes between subjects assigned to fetal endoscopic tracheal occlusion or standard care (Harrison et al, 2003). Enrollment was stopped after 24 women carrying fetuses with severe CDH had been enrolled because of the unexpectedly high survival rate with standard care and the conclusion of the data safety monitoring board that further recruitment would not result in significant differences between the groups. Eight of 11 fetuses (73 % in the tracheal-occlusion group and 10 of 13 (77 %) in the group that received standard care survived to 90 days of age. The authors concluded that tracheal occlusion did not improve survival or morbidity rates in this cohort of fetuses with CDH. In an accompanying editorial, Wenstrom (2003) argued that there are several reasons why antenatal tracheal occlusion may not result in a better outcome than conventional therapy. Wenstrom reasoned that, with new diagnostic technologies, congenital diaphragmatic defects of varying degrees of severity, from mild to severe, are now routinely identified antenatally, and affected neonates receive care at tertiary centers that offer highly specialized treatments for respiratory disease, including extracorporeal membrane oxygenation, high-frequency oscillatory ventilation, inhaled nitric oxide, exogenous surfactant, and others. As a result, the current survival rate for all cases of isolated CDH -- from mild to severe -- approaches 70 % without fetal surgery, and neonates who do not require extracorporeal life support (approximately 50 % of those with isolated CDH) have a survival rate of at least 80 %. Wenstrom argued that another reason why antenatal intervention may not result in a better outcome than conventional therapy is that any potential benefit may be negated by the substantial fetal morbidity associated with the surgical procedure itself. Most pregnancies subjected to antenatal fetal surgery end in preterm delivery. Wenstrom noted that, in the study by Harrison et al, premature rupture of the membranes and preterm delivery occurred in 100 % of those receiving antenatal treatment. The mean age at delivery was 30.8 weeks in the treated group, an age at which morbidity related to prematurity is likely. In addition, because birth occurred, on average, just 6 weeks after the procedure, appropriate catch-up lung growth may not yet have occurred. Wenstrom concluded that “[t]he study by Harrison et al also illustrates the critical importance of randomized clinical trials in evaluating new therapies – even heroic procedures performed in only a small fraction of neonates – before they are adopted as part of standard practice.”
In a RCT, Keller et al (2004) concluded fetal tracheal occlusion for severe CDH resulted in modest improvements in neonatal pulmonary function that, according to the investigators, were of questionable clinical significance.
There is considerable scientific and clinical interest in the potential use of hematopoietic stem cells before birth to treat congenital disease. In theory, stem cell transplantation in utero offers a number of possible advantages. First, intervention in utero will permit "correction" of a disorder before clinical manifestations have developed. Second, because the fetal immune system has not yet developed, it will not reject foreign cells. Unlike bone marrow transplantation after birth, there is no need to match donor cells. The fetus will become "tolerant" to the foreign cells allowing for further treatment after birth, again without the risk of rejection.
Current evidence for in-utero stem cell transplantation comes from animal models and from a small number reported cases of in utero transplantations of unmodified bone marrow progenitor cells in human fetuses involving such disorders as X-linked severe combined immune deficiency and hemoglobinopathies (e.g.,alpha thalassemia, sickle cell anemia and beta thalassemia). Although there is some evidence for success of in- utero stem cell transplantation in X-linked severe combined immunodeficiency syndrome, there is no proven clear advantage over post-natal stem cell transplantation for this indication. Regarding other potential uses, thus far, in utero stem cell transplantation has been unsuccessful in target disorders such as hemoglobinopathies where there is not a selective advantage for donor cells (Muench and Barcena, 2004; Flake, 2004).
In-utero gene therapy (i.e., the genetic modification of somatic cells in utero) has been propsed as most appropriate in disorders which result in irreversible illness or death in the pre- or post-natal period. Examples may include Gaucher’s disease, Krabbe’s disease, Hurler’s disease, etc. Currently evidence is limited to animal models that certain genetic conditions can be corrected in-utero using gene therapy using virus vectors. In addition to the need for evidence of the effectiveness of gene therapy in- utero in humans, it has been argued that 2 key issues need to be addressed before such an intervention is considered; that there must be a clear advantage over post-natal gene therapy; and that there must be an advantage over therapy with unmodified cells.
Strumper et al (2005) noted that chronically compromised uterine perfusion may lead to placental insufficiency and subsequent intra-uterine growth restriction (IUGR). Various interventions such as the use of vasodilators/low-dose aspirin, intravenous glucose infusion, as well as hemodilution are often of limited effectiveness. The use of local anesthetics has been demonstrated to improve placental blood flow in pre-eclamptic women. In a pilot study (n = 10), these researchers examined whether epidural administration of local anesthetics might improve outcome in IUGR independent of the underlying cause. Women presenting with oligohydramnios and IUGR were included in the study. In addition to the standard protocol (magnesium, glucose, betamethasone), each patient received an epidural catheter (T10/T12) with continuous infusion of bupivacaine 0.175 % at a rate of 5 ml/hour. Uteroplacental circulation was monitored by Doppler sonography and the amount of amniotic fluid was estimated daily. Epidural insertion and infusion was performed without complications. Four patients continued to deteriorate rapidly, amniotic fluid volume did not change and uterine artery pulsatility index (PI) tended to increase. In the remaining 6 patients the clinical status stabilized, amniotic fluid volume tended to increase and uterine artery PI tended to decrease during treatment. This improvement was associated with a prolonged interval to cesarean section and increased infant birth weight. The authors concluded that even if the underlying cause of IUGR is not pre-eclampsia, epidural infusion of local anesthetic might improve placental blood flow and be beneficial in a subgroup of patients. They stated that a clinical trial to test this hypothesis appears warranted.
Gardiner (2008) noted that the concept of fetal therapy is well-established for many disorders diagnosed before birth; but practical issues regarding its introduction into clinical practice are more difficult. Cardiac malformations are common, with major lesions affecting about 3.5 per 1,000 pregnancies; however, only a small proportion of these is likely to benefit from an intra-uterine intervention. In addition, there are no good animal models of human cardiac disease and knowledge of the underlying mechanisms is at best sketchy. This combination of factors has resulted in slow progress in developing effective therapies for the intra-uterine management of cardiac disease. The author stated that recent research and clinical developments have included percutaneous valvuloplasty for severe aortic and pulmonary stenosis, perforation of the closed or restrictive inter-atrial septum and pacing for complete heart block. Progress in these endeavours has been variable; but overall shows promise for treatment of the human fetus.
Adzick (2010) stated that myelomeningocele (MMC) is a common birth defect that is associated with significant lifelong morbidity. Little progress has been made in the post-natal surgical management of the child with spina bifida. Post-natal surgery is aimed at covering the exposed spinal cord, preventing infection, and treating hydrocephalus with a ventricular shunt. I n-utero repair of open spina bifida is now performed in selected patients and presents an additional therapeutic alternative for expectant mothers carrying a fetus with MMC. It is estimated that about 400 fetal operations have now been performed for MMC worldwide. Despite this large experience, the technique remains of unproven benefit. Preliminary results suggested that fetal surgery results in reversal of hind-brain herniation (the Chiari II malformation), a decrease in shunt-dependent hydrocephalus, and possibly improvement in leg function, but these findings might be explained by selection bias and changing management indications. A prospective, randomized study (the MOMS trial) is currently being conducted by 3 centers in the United States, and is estimated to be completed in 2010. The author stated that further research is needed to better understand the pathophysiology of MMC, the ideal timing and technique of repair, and the long-term impact of in-utero intervention.
Jani and colleagues (2009) examined operative and peri-natal aspects of fetal endoscopic tracheal occlusion (FETO) in CDH. It was a multi-center study of singleton pregnancies with CDH treated by FETO. The entry criteria for FETO were severe CDH on the basis of sonographic evidence of intra-thoracic herniation of the liver and low lung area to head circumference ratio (LHR) defined as the observed to the expected normal mean for gestation (o/e LHR) equivalent to an LHR of 1 or less. Fetal endoscopic tracheal occlusion was carried out in 210 cases, including 175 cases with left-sided, 34 right-sided and one with bilateral CDH. In 188 cases, the CDH was isolated and in 22 there was an associated defect. Fetal endoscopic tracheal occlusion was performed at a median gestational age of 27.1 (range of 23.0 to 33.3) weeks. The first 8 cases were done under general anesthesia, but subsequently either regional or local anesthesia was used. The median duration of FETO was 10 (range of 3 to 93) mins. Successful placement of the balloon at the first procedure was achieved in 203 (96.7 %) cases. Spontaneous preterm prelabor rupture of membranes (PPROM) occurred in 99 (47.1 %) cases at 3 to 83 (median of 30) days after FETO and within 3 weeks of the procedure in 35 (16.7 %) cases. Removal of the balloon was pre-natal either by fetoscopy or ultrasound-guided puncture, intra-partum by ex-utero intra-partum treatment, or post-natal either by tracheoscopy or percutaneous puncture. Delivery was at 25.7 to 41.0 (median of 35.3) weeks and before 34 weeks in 65 (30.9 %) cases. In 204 (97.1 %) cases, the babies were live born and 98 (48.0 %) were discharged from the hospital alive. There were 10 deaths directly related to difficulties with removal of the balloon. Significant prediction of survival was provided by the o/e LHR and gestational age at delivery. On the basis of the relationship between survival and o/e LHR in expectantly managed fetuses with CDH, as reported in the ante-natal CDH registry, these researchers estimated that in fetuses with left CDH treated with FETO the survival rate increased from 24.1 % to 49.1 %, and in right CDH survival increased from 0 % to 35.3 % (p < 0.001). The authors concluded that FETO in severe CDH is associated with a high incidence of PPROM and preterm delivery but a substantial improvement in survival. They also stated that these findings need to be tested in a RCT.
Gastroschisis is associated with inflammatory changes in the exposed bowel that leads to intestinal dysmotility following post-natal repair. In a retrospective study, Heinig et al (2008) followed a case-series of fetuses with isolated gastroschisis to evaluate if small-bowel dilatation may be indicative for emerging obstetric complications. The secondary objective of the study was to establish preliminary normative curves for the external diameter and wall thickness of eventerated fetal small bowel in gastroschisis during the 2nd and 3rd trimester of pregnancy. A total of 14 fetuses with isolated gastroschisis were followed at a single center. Repeated ultrasound examinations for fetal surveillance with measurement of fetal small-bowel diameter and wall thickness over the course of pregnancy until delivery were performed. Longitudinal data analysis showed significantly increasing bowel diameter and wall thickness of eventerated small bowel with advancing gestation. Dilatation of small bowel more than 25 mm in the 3rd trimester of pregnancy was associated with an increased risk of short-term pre-natal complications as fetal distress or intra-uterine fetal death (positive predictive value 100 %; 95 % confidence interval [CI]: 29.2 % to 100 %, negative predictive value 100 %; 95 % CI: 71.5 % to 100%). The authors concluded that dilatation of the extra-abdominal fetal small bowel in the 3rd trimester may allow identifying fetuses with increased risk of fetal distress requiring closer monitoring of fetal well-being or delivery in a short interval to prevent impending fetal death.
Cohen-Overbeek et al (2008) studied in infants with gastroschisis whether outcome is different when comparing a pre-natal diagnosis with a diagnosis only at birth with the intention to develop a pre-natal surveillance protocol. Intestinal atresia established after birth and preterm versus term delivery were studied as risk factors. A total of 24 fetuses and 9 infants diagnosed with gastroschisis and were studied retrospectively. The infants of the pre-natal subset were delivered at the authors' tertiary center and 18 survived. There were 2 pregnancy terminations, 3 intra-uterine deaths at 19, 33 and 36 weeks, respectively, and 1 neonatal death. All 9 infants in the post-natal subset survived -- 8 were out-born and 1 was delivered at the authors' tertiary center. Pre-natal bowel dilatation did not correlate with outcome. Between the pre-natal and post-natal subset, no significant difference in outcome of live-born infants was established. For 4 infants with intestinal atresia a significant difference was demonstrated for induction of preterm labor (p < 0.05), duration of parenteral nutrition (p < 0.01), number of additional surgical procedures (p < 0.001) and length of hospital stay (p < 0.01). The 15 infants born prior to 37 weeks of gestation spent a significantly longer period in hospital compared to those delivered at term. When the cases with bowel atresia were excluded this difference was no longer present. Five of the 33 cases were diagnosed with associated anomalies which mainly involved the urinary tract. The authors concluded that neonatal outcome of live born infants following a pre-natal diagnosis of gastroschisis is not different from a diagnosis at birth. The presence of intestinal atresia is the most important prognostic factor for morbidity. The supplemental value of pre-natal diagnosis to the outcome of infants with gastroschisis may be in the prevention of unnecessary intra-uterine death and detection of intestinal complications. A proposed surveillance protocol for fetuses with gastroschisis focused on intra-uterine signs of pending distress such as a dilated stomach, intra-abdominal bowel dilatation with peristalsis, notches in the umbilical artery Doppler signal, development of polyhydramnios and an abnormal cardiotocography registration may improve outcome. Currently, in-utero repair of gastroschisis is being studied in the sheep model (Stephenson et al, 2010). Thus, this approach is not ready for clinical use.
Adzick et al (2011) compared outcomes of in-utero repair for myelomeningocele with standard postnatal repair. These investigators randomly assigned eligible women to undergo either prenatal surgery before 26 weeks of gestation or standard postnatal repair. One primary outcome was a composite of fetal or neonatal death or the need for placement of a cerebrospinal fluid shunt by the age of 12 months. Another primary outcome at 30 months was a composite of mental development and motor function. Inclusion criteria were a singleton pregnancy, myelomeningocele with the upper boundary located between T1 and S1, evidence of hind-brain herniation, a gestational age of 19.0 to 25.9 weeks at randomization, a normal karyotype, U.S. residency, and maternal age of at least 18 years. Major exclusion criteria were a fetal anomaly unrelated to myelomeningocele, severe kyphosis, risk of preterm birth (including short cervix and previous preterm birth), placental abruption, a body-mass index (the weight in kilograms divided by the square of the height in meters) of 35 or more, and contraindication to surgery, including previous hysterotomy in the active uterine segment. The trial was stopped for efficacy of pre-natal surgery after the recruitment of 183 of a planned 200 patients. This report was based on results in 158 patients whose children were evaluated at 12 months. The first primary outcome occurred in 68 % of the infants in the prenatal-surgery group and in 98 % of those in the postnatal-surgery group (relative risk, 0.70; 97.7 % CI: 0.58 to 0.84; p < 0.001). Actual rates of shunt placement were 40 % in the prenatal-surgery group and 82 % in the postnatal-surgery group (relative risk, 0.48; 97.7 % CI: 0.36 to 0.64; p < 0.001). Prenatal surgery also resulted in improvement in the composite score for mental development and motor function at 30 months (p = 0.007) and in improvement in several secondary outcomes, including hind-brain herniation by 12 months and ambulation by 30 months. However, prenatal surgery was associated with an increased risk of preterm delivery and uterine dehiscence at delivery. The authors concluded that prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associated with maternal and fetal risks.
In an editorial that accompanied the afore-mentioned study, Simpson and Greene (2010) stated that "[t]o what extent can these results be generalized? Caution is necessary here. For the decade of this trial, all cases nationwide were funneled to the 3 study centers, which by now should have developed near-optimal prowess. With the trial complete, other U.S. centers are likely to initiate their own programs, diluting experience and necessitating individual center-specific learning curves. Fetal results may not be as good as those in MOMS, and maternal complications could be increased. In addition, most women who expressed interest in the trial were either ineligible or declined to participate, with only 15 % participation of those who were screened. This percentage may or may not increase as access extends beyond the 3 centers. Earlier diagnosis of myelomeningocele and the performance of open fetal surgery earlier than that performed in MOMS might further improve outcomes, but the potential benefits of even earlier intervention must be weighed against the greater likelihood of maternal complications and possibly increased difficulty of fetal repair. More work is also needed to determine whether baseline characteristics could predict which fetuses would be more or less likely to benefit from prenatal surgery. But surely the greatest benefit would derive from a less traumatic approach. Our job as physicians is to communicate options and available data to patients as lucidly as possible while assiduously adhering to the principles of non-directive genetic counseling. For many women, the 20 % absolute improvement in ambulation at the age of 3 years and the decreased need for shunting may be perceived as sufficient to justify the increased risk of maternal complications, but it should be recognized that outcomes after prenatal surgery were less than perfect in MOMS. Couples who do not elect to terminate a pregnancy unavoidably feel pressured “to do everything possible” and hence may be inclined to interpret even marginal benefit favorably. It is also human nature to over-estimate the likely benefit for one's own fetus and to under-estimate the associated risks. Counseling should involve not only precise quantitative statements comparing outcomes of prenatal versus postnatal surgery on the basis of this report but also the provision of information on center-specific experience. The degree to which intrauterine repair will transform outcomes for fetuses with myelomeningocele remains unclear. The study by Adzick et al is a major step in the right direction, but the still suboptimal rates of poor neonatal outcome and high maternal risk necessitate the use of less invasive approaches if such procedures are to be widely implemented".
Guidance from the National Institute for Health and Clinical Excellence (NICE, 2006) concluded that current evidence on the safety and efficacy of pleuro-amniotic shunts to drain fetal pleural effusions appears adequate. The guidance noted, however, that there are uncertainties about the natural history of fetal pleural effusion and about patient selection. Therefore, this procedure should not be used without special arrangements for consent and for audit or research.
CPT Codes / HCPCS Codes / ICD-9 Codes
CPT codes covered if selection criteria are met:
Other CPT codes related to the CPB:
HCPCS codes covered if selection criteria are met:
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, 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 syndrome
HCPCS codes not covered for indications listed in the CPB:
Repair, congenital diaphragmatic hernia in the fetus using temporary tracheal occlusion, procedure performed in utero
ICD-9 codes covered if selection criteria are met:
Neoplasm of uncertain behavior of bone and articular cartilage
Neoplasm of uncertain behavior of other specified sites
Unspecified pleural effusion
741.00 - 741.93
Congenital cystic lung
Agenesis, hypoplasia, and dysplasia of lung
Other specified anomalies of respiratory system
753.20 - 753.29
Obstructive defects of renal pelvis and ureter
Atresia and stenosis of urethra and bladder neck
Placental transfusion syndrome
ICD-9 codes not covered for indications listed in the CPB:
Known or suspected fetal central nervous system malformation in fetus affecting management of the mother, antepartum condition or complication
Known or suspected chromosomal abnormality in fetus affecting management of the mother, antepartum condition or complication
Unspecified known or suspected fetal abnormality affecting management of the mother, antepartum condition or complication
Other specified anomalies of spinal cord
745.0 - 746.9
Bulbus cordis anomalies and anomalies of cardiac septal closure and other congenital anomalies of the heart
Anomalies of diaphragm
776.0 - 776.9
Hematological disorders of fetus and newborn
Other ICD-9 codes related to the CPB:
Other fetal abnormality causing disproportion, antepartum condition or complication
Other known or suspected abnormality, not elsewhere classified, affecting management of the mother, antepartum condition or complication
Other specified congenital anomalies [acardia]
The above policy is based on the following references:
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