Obstentrics_Williams

Obstentrics_Williams. In a review of more than 50 cases of mirror syndrome, Braun (2010) found that approximately 90 percent of women had edema, 60 percent had hypertension, 40 percent had proteinuria, 20 percent had liver enzyme elevation, and nearly 15 percent had headache and visual disturbances. Based on these indings, it is reasonable to consider mirror syndrome a form of severe preeclampsia (Espinoza, 2006; Midgley, 2000). Others, however, have suggested that it is a separate disease process with hemodilution rather than hemoconcentration (Carbillon, 1997; Livingston, 2007).


Obstentrics_Williams

Obstentrics_Williams. Some reports describe the same imbalance of angiogenic and antiangiogenic factors that is observed with preeclampsia, and this suggests a common pathophysiology (Espinoza, 2006; Goa, 2013; Llurba, 2012). hese indings, which include elevated concentrations of soluble fms-like tyrosine kinase-1 (sFlt-l), decreased placental growth factor (PIG F) levels, and elevation of soluble vascular endothelial growth factor receptor-I (s VEG FR-1) concentrations, are discussed further Chapter 40 (p. 716).


Obstentrics_Williams

Obstentrics_Williams. In most cases with mirror syndrome, prompt delivery is indicated and followed by resolution of maternal edema and other findings (Braun, 2010). However, in isolated cases of fetal anemia, supraventricular tachycardia, hydrothorx, and bladder outlet obstruction, successful fetal treatment resulted in resolution of both fetal hydrops and maternal mirror syndrome (Goa, 2013; Livingston, 2007; Llurba, 2012; Midgley, 2000). Normalization of the angiogenic imbalance has also been described following fetal transfusion for parvovirus B 19 infection. Fetal therapy for these conditions is reviewed in Chapter 16. Given the parallels to severe preeclampsia, delaying delivery to efect fetal therapy should be considered only with caution. If the maternal condition deteriorates, delivery is recommended. American Academy of Pediatrics, American College of Obstetricians and Gynecologists: Guidelines for Perinatal Care. 8th ed. Elk Grove Village, MP, 201


Obstentrics_Williams

Obstentrics_Williams. American Academy of Pediatrics, American College of Obstetricians and Gynecologists: Guidelines for Perinatal Care. 8th ed. Elk Grove Village, MP, 201 American College of Obstetricians and Gynecologists: Management of alloimmunization during pregnancy. Practice Bulletin No. 75, August 2006, Reairmed 2016


Obstentrics_Williams

Obstentrics_Williams. American College of Obstetricians and Gynecologists: Prevention ofRh 0 alloimmunization. Practice Bulletin No. 181, August 20o17 Bellini C, Hennekam RC: Non-immune hydrops fetalis: a short review of etiology and pathophysiology. Am] Med Genet 158A(3):597, 2012 Bellini C, Hennekam RC, Fulcheri E, et al: Etiology of nonimmune hydrops fetalis: a systematic review. Amo] Med Genet A 149A(5):844, 2009 Bellini C, Donarini G, Paladini 0, et al: Etiology of non-immune hydrops fetalis: an update. Am ] Med Genet 167 A: 1082, 2015 Bellussi F, Perolo A, Ghi T, et al: Diagnosis of severe fetomaternal hemorrhage with fetal cerebral Doppler: case series and systematic review. Fetal Diagn Ther 41(1):1,o2017 Berkowitz L, Kolb A, McFarland ]G, et al: Parallel randomized trials of risk-based therapy for fetal alloimmune thrombocytopenia. Obstet Gynecol 107(1):91,o2006 Bollason G, Hjartardottir H, Jonsson T, et al: Red blood cell alloimmunization in pregnancy during the years 1996-2015 in Iceland: a


Obstentrics_Williams

Obstentrics_Williams. fetal alloimmune thrombocytopenia. Obstet Gynecol 107(1):91,o2006 Bollason G, Hjartardottir H, Jonsson T, et al: Red blood cell alloimmunization in pregnancy during the years 1996-2015 in Iceland: a nation-wide population study. Transfusion 57(1o1):2578, 2017


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Obstentrics_Williams. Bowmano]: Rh-immunoglobulin: Rh prophylaxis. Best Pract Res Clin HaematoIo19(1):27,o2006 Bowman ]M: Controversies in Rh prophylaxis: who needs Rh immune globulin and when should it be given? Am ] Obstet Gynecol 151 :289, 1985 Bowman ]M: he prevention of h immunization. Transfus Med Rev 2: 129, 1988 Braun T, Brauer M, Fuchs I, et al: Mirror syndrome: a systematic review of fetal associated conditions, maternal presentation, and perinatal outcome. Fetal Diagn Ther 27(4):191,o2010 Bussel ]B, Berkowitz L, Lynch L, et al: Antenatal management of alloimmune thrombocytopenia with intravenous gamma-globulin: a randomized trial of the addition oflow-dose steroid to intravenous gamma-globulin. Am ] Obstet GynecoIo174(5):1414, 1996 Bussel ]B, Zabusky MR, Berkowitz L, et al: Fetal alloimmune thrombocytopenia. N Englo] Med 337:22, 1997


Obstentrics_Williams

Obstentrics_Williams. Bussel ]B, Zabusky MR, Berkowitz L, et al: Fetal alloimmune thrombocytopenia. N Englo] Med 337:22, 1997 Carbillon L, Oury ]F, Guerin ]M, et al: Clinical biological features of Ballantyne syndrome and the role of placental hydrops. Obstet Gynecol Surv 52(5):310, 1997 Chambers E, Davies L, Evans S, et al: Comparison of haemoglobin F detection by the acid elution test, low cytometry and high-performance liquid chromatography in maternal blood samples analysed for fetomaternal haemorrhage. Transfus Med 22(3):199, 2012 Choavaratana R, Uer-Areewong S, Makanantakocol S: Fetomaternal transfusion in normal pregnancy and during delivery. ] Med Assoc Thai 80:96, 1997 Corcoran 0, Murphy 0, Donnelly], et al: The prevalence of maternal F cells in a pregnant population and potential overestimation of foeto-maternal haemorrhage as a consequence. Blood Transfus 12:570,o2014


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Obstentrics_Williams. Daniels G: Variants of RhO-current testing and clinical consequences. Br ] Haematol 161(4):461,o2013 de Almeida V, Bowman ]M: Massive fetomaternal hemorrhage: Manitoba experience. Obstet Gynecol 83:323, 1994 de Haas M, Thurik FF, Koelewijno]M et al: Haemolytic disease of the fetus and newborn. Vox Sang 109(2):99, 2015 de Haas M, Thurik FF, van der Ploeg CP, et al: Sensitivity of fetal RHO screening for safe guidance of targeted anti-D immunoglobulin prophylaxis: prospective cohort study of a nationwide programme in the Netherlands. BM] 89,o2016 Espinozao], Romero R, Nien ]K, et al: A role of the anti-angiogenic factor sVEGFR-1 in the "mirror syndrome" (Ballantyne's syndrome). ] Matern Fetal Neonatal Med 19(10):607,o2006 Fernandes B], von Dadelszen P, Fazal I, et al: Flow cytometric assessment of feto-maternal hemorrhage; a comparison with Betke-Kleihauer. Prenat Diagn 27(7):641, 2007


Obstentrics_Williams

Obstentrics_Williams. Fernandes B], von Dadelszen P, Fazal I, et al: Flow cytometric assessment of feto-maternal hemorrhage; a comparison with Betke-Kleihauer. Prenat Diagn 27(7):641, 2007 Garratty G, Glynn SA, McEntire R, et al: ABO and Rh(D) phenotype frequencies of diferent racial/ethnic groups in the United States. Transfusion 44(5):703, 2004 Giacoia GP. Severe fetomaternal hemorrhage: a review. Obstet Gynecol Surv 52:372, 1997 Giannina G, Moise K] ]r, Dorman K: A simple method to estimate the volume for fetal intravascular transfusion. Fetal Diagn Ther 13:94, 1998 Goa S, Mimura K, Kakigano A, et al: Normalisation of angiogenic imbalance after intra-uterine transfusion for mirror syndrome caused by parvovirus B19. Fetal Diagn her 6,o2013 Hachisuga K, Hidaka N, Fujita Y, et al: Can we predict neonatal thrombocytopenia in ofspring of women with idiopathic thrombocytopenic purpura? Blood 49(4):259,o2014


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Obstentrics_Williams. Hachisuga K, Hidaka N, Fujita Y, et al: Can we predict neonatal thrombocytopenia in ofspring of women with idiopathic thrombocytopenic purpura? Blood 49(4):259,o2014 Hackney ON, Knudtson E], Rossi KQ, et al: Management of pregnancies complicated by anti-c isoimmunization. Obstet Gynecol 103:24, 2004 Heinonen S, Ruynamen M, Kirkinen P: Etiology and outcome of second trimes ter nonimmunological fetal hydrops. Scando] Obstet Gynecol 79: 15, 2000 Howard H, Mardew V, McFadyen I, et al: Consequences for fetus and neonate of maternal red cell allo-immunization. Arch Dis Child Fetal Neonat Ed 78:F62, 1998 Johnson ]A, MacDonald K, Clarke G, et al: No. 343-Routine non-invasive prenatal prediction of fetal RHO genotype in Canada: the time is here. ] Obstet Gynaecol Can 39(5):366, 2017 Kamphuis MM, Paridaans N, Porcelijn L, et al: Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia: systematic review. B]OG 117(1o1):1335,o2010


Obstentrics_Williams

Obstentrics_Williams. Kamphuis MM, Paridaans N, Porcelijn L, et al: Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia: systematic review. B]OG 117(1o1):1335,o2010 Kleihauer B, Braun H, Betke K: Demonstration of fetal hemoglobin in erythrocytes of a blood smear. Klin Wochenschr 35(12):637, 1957 Knight M, Pierce M, Allen 0, et al: The incidence and outcomes of fetomaternal alloimmune thrombocytopenia: a UK national study using three data sources. Br] HaematoIo152(4):460, 2011 Koelewijn ]M, Vrijkotte TG, van der Schoot CE, et al: Efect of screening for red cell antibodies, other than anti-D, to detect hemolytic disease of the fetus and newborn: a population study in the Netherlands. Transfusion 48:941,o2008 Lazar L, Harmath AG, Ban Z, et al: Detection of maternal deoxyribonucleic acid in peripheral blood of premature and mature newborn infants. Prenat Diagn 26(2):168, 2006


Obstentrics_Williams

Obstentrics_Williams. Lazar L, Harmath AG, Ban Z, et al: Detection of maternal deoxyribonucleic acid in peripheral blood of premature and mature newborn infants. Prenat Diagn 26(2):168, 2006 Liao C, Weio], Li Q, et al: Nonimmune hydrops fetal is diagnosed during the second half of pregnancy in Southern China. Fetal Diagn Ther 22(4):302,o2007 Liley A W: Liquor amnii analysis in management of pregnancy complicated by rhesus sensitization. Amo] Obstet GynecoIo82:1359, 1961 Lindenburg I, van Kamp I, van Zwet E, et al: Increased perinatal loss after intrauterine transfusion for alloimmune anaemia before 20 weeks of gestation. B]OG 120:847,o2013 Lindenburg IT, Smits-Wintjens VE, van Klink ]M, et al: Long-term neurodevelopmental outcome after intrauterine transfusion for hemolytic disease of the fetus/newborn: the LOTUS study. Amo] Obstet Gynecol 206:o141. e1,o2012


Obstentrics_Williams

Obstentrics_Williams. Livingston ]C, Malik M, Crombleholme TM, et al: Mirror syndrome: a novel approach to therapy with fetal peritoneal-amniotic shunt. Obstet Gynecol 110(2 Pt 2):540,o2007 Llurba E, Marsal G, Sanchez 0, et al: Angiogenic and antiangiogenic factors before and after resolution of maternal mirror syndrome. Ultrasound Obstet Gynecol 40(3):367, 2012 Lockwood q, Nadel AS, King ME, et al: A 32-year old pregnant woman with an abnormal fetal ultrasound study. Case 16-2009. N Englo] Med 360(21):2225,o2009 MacKenzie IZ, Roseman F, Findlayo], et al: he kinetics of routine antenatal prophylactic intramuscular injections of polyclonal anti-D immunoglobulin. B]OG 113:97, 2006 Mari G, Deter L, Carpenter RL, et al: Noninvasive diagnosis by Doppler ultrasonography of fetal anemia due to maternal red-cell alloimmunization. N Englo] Med 342:9, 2000 Martin ]A, Hamilton BE, Sutton PD, et al: Births: final data for 2003. Natl Vital Stat Rep 54(2): 1, 2005


Obstentrics_Williams

Obstentrics_Williams. Martin ]A, Hamilton BE, Sutton PD, et al: Births: final data for 2003. Natl Vital Stat Rep 54(2): 1, 2005 Midgley DY, Hardrug K: The mirror syndrome. Euro] Obstet Gynecol Reprod Bioi 8:201, 2000 Moise K]: Fetal anemia due to non-Rhesus-D red-cell alloimmunization. Semin Fetal Neonatal Med ,o2008 Moise K], Argoti PS: Management and prevention of red cell alloimmunization in pregnancy. A systematic review. Obstet Gynecol 120(5): 1132, 20o12 Moise K], Gandhi M, Boring NH, et al: Circulating cell-free DNA to determine the fetal RHD status in all three trimesters of pregnancy. Obstet Gynecol 128(6):1340, 2016 Neunert C, Lim W, Crowther M, et al: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 11 (16):4190,o2011 Nicolaides KH, Warenski ]c, Rodeck CH: The relationship of fetal plasma protein concentration and hemoglobin level to the development of hydrops in rhesus isoimmunization. Am] Obstet Gynecol 152:341, 1985


Obstentrics_Williams

Obstentrics_Williams. Oepkes 0, Seaward PG, Vandenbussche FP, et al: Doppler ultrasonography versus amniocentesis to predict fetal anemia. N Engl] Med 355: 156, 2006 Pacheco LD, Berkowitz RL, Moise K], et a1: Fetal and neonatal alloimmune thrombocytopenia. A management algorithm based on risk stratification. Obstet Gynecol 118(5):1157,2011 Queenan ]T, Thomas PT, Tomai TP, et al: Deviation in amniotic fluid optical density at a wavelength of 450 nm in Rh isoimmunized pregnancies from 14 to 40 weeks' gestation: a proposal for clinical management. Am ] Obstet Gynecol 168:1370, 1993 Ratanasiri T, Komwilaisak R, Sittivech A, et al: Incidence, causes, and pregnancy outcomes of hydrops fetalis at Srinagarind Hospital, 1996-2005: a 10-year review.o] Med Assoc hai 92(5):594, 2009 Rink BD, Gonik B, Chmait RH, et al: Maternal hemolysis after intravenous immunoglobulin treatment in fetal and neonatal alloimmune thrombocytopenia. Obstet GynecoIo121o(2):47o1, 20o13


Obstentrics_Williams

Obstentrics_Williams. Rink BD, Gonik B, Chmait RH, et al: Maternal hemolysis after intravenous immunoglobulin treatment in fetal and neonatal alloimmune thrombocytopenia. Obstet GynecoIo121o(2):47o1, 20o13 Risson DC, Davies MW, Williams BA: Review of neonatal alloimmune thrombocytopenia.o] Pediatr Child Health 48(9):816,o2012 Rubod C, Deruelle P, Le Gouef F, et al: Long-term prognosis for infants after massive fetomaternal hemorrhage. Obstet Gynecol 110(2 pt 1),o200 Sandler SG, Flegel WA, Westhof CM, et al: It's time to phase-in RHD genotyping for patients with a serological weak D phenotype. Transfusion 55(3):680,o2015 Sandler SG, Queenan ]T: A guide to terminology for Rh immunoprophylaxis. Obstet Gynecol 130(3):633,o2017 Santo S, Mansour S, Thilaganathan B, et al: Prenatal diagnosis of non-immune hydrops fetalis: what do we tell the parents? Prenat Diagn 31: 186, 2011 Santolayao], Alley 0, Jafe R, et al: Antenatal classiication of hydrops fetalis. Obstet 9:256, 1992


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Obstentrics_Williams. Obstet 9:256, 1992 Society for Maternal-Fetal Medicine, Mari G, Norton ME, et a1: Society for Maternal-Fetal Medicine (SMFM) Clinical Guideline #8: he fetus at risk for anemia-diagnosis and management. Am] Obstet Gynecol 212(6):697, 20o15a Society for Maternal-Fetal Medicine, Norton ME, Chauhan SP, et al: Society for Maternal-Fetal Medicine Clinical Guideline # : Nonimmune hydrops fetalis. Amo] Obstet Gynecol 212(2):127, 2015b Sohan K, Carroll SG, De La Fuente S, et al: Analysis of outcome in hydrops fetalis in relation to gestational age at diagnosis, cause, and treatment. Acta Obstet Gynecol Scand 80(8):726, 2001 Storry]R, Castilho L, Daniels G, et a1: International Society of Blood Transfusion Working Party on red cell immunogenetics and blood group terminology: Cancun report (2012). Vox Sang 107(1): 90, 2014


Obstentrics_Williams

Obstentrics_Williams. Suwanrath-Kengpol C, Kor-anantakul 0, Suntharasaj T, et a1: Etiology and outcome of non-immune hydrops fetalis in southern Thailand. Gynecol Obstet Invest 59(3): 134, 2005 Tannirandorn Y, Rodeck CH: New approaches in the treatment of haemolytic disease of the fetus. Ballieres Clin Haematol 3(2):289, 1990 Tiller H, Kamphuis MM, Flodmark 0, et al: Fetal intracranial hemorrhages caused by fetal and neonatal alloimmune thrombocytopenia: an observational cohort study of 43 cases from an international multicentre registry. BM] 3:e002490, 20o13 Van den Veyver lB, Moise K]: Fetal RhO typing by polymerase chain reaction in pregnancies complicated by rhesus alloimmunization. Obstet Gynecol 88:1061, 1996


Obstentrics_Williams

Obstentrics_Williams. Van den Veyver lB, Moise K]: Fetal RhO typing by polymerase chain reaction in pregnancies complicated by rhesus alloimmunization. Obstet Gynecol 88:1061, 1996 Van Kamp IL, lumper F], Bakkum RS, et al: he severity of immune fetal hydrops is predictive of fetal outcome after intrauterine treatment. Am ] Obstet Gynecol 185:668,o2001 Vivanti A, Benachi A, Huchet FX, et al: Diagnostic accuracy of fetal rhesus 0 genotyping using cell-free fetal DNA during the first trimester of pregnancy. Am] Obstet GynecoIo215:606.e1, 2016 Weiner CP, Pelzer GO, Heilskov], et al: he efect of intravascular transfusion on umbilical venous pressure in anemic fetuses with and without hydrops. Amo] Obstet Gynecol 161:1498, 1989 Weinstein L: Irregular antibodies causing hemolytic disease of the newborn: a continuing problem. Clin Obstet Gynecol 25(2):321, 1982


Obstentrics_Williams

Obstentrics_Williams. Weinstein L: Irregular antibodies causing hemolytic disease of the newborn: a continuing problem. Clin Obstet Gynecol 25(2):321, 1982 Welsh K], Bai Y, Education Committee of the Academy of Clinical Laboratory Physicians and Scientists: Pathology consultation on patients with a large h immune globulin dose requirement. Amo] Clin Pathol 145: 44, 2016 Woelfer B, Schuchter K, ]anisiw M, et al: Postdelivery levels of anti-D IgG prophylaxis in mothers depend on maternal body weight. Transfusion 44:512, 2004 Wylie B], D'Alton ME: Fetomaternal hemorrhage. Obstet Gynecol 115(5): 1039,o2010 Wyszynski OF, Carmen J, Cantor AB, et al: Pregnancy and birth outcomes among women with idiopathic thrombocytopenic purpura. ] Pregnancy 2016:8297407,o2016


Obstentrics_Williams

Obstentrics_Williams. Wyszynski OF, Carmen J, Cantor AB, et al: Pregnancy and birth outcomes among women with idiopathic thrombocytopenic purpura. ] Pregnancy 2016:8297407,o2016 Zimmerman R, Carpenter R] ]r, Durig P, et al: Longitudinal measurement of peak systolic velocity in the fetal middle cerebral artery for monitoring pregnancies complicated by red cell alloimmunization: a prospective multicenter trial with intention-to-treat. B]OG ):746,o2002 Zipursky A, Bhutani VK: Impact of Rhesus disease on the global problem of bilirubin-induced neurologic dysfunction. Semin Fetal Neonatal Med 20(1):2,o2015 Zwiers C, Lindenburg IT, Klumper F], et al: Complications of intrauterine intravascular blood transfusion: lessons learned ater 1678 procedures. Ultrasound Obstet Gynecol 50(2):180, 2017 MEDICAL THERAPY ....e.....e..e.... ...e......e...e..315 SURGICAL THERAPY ..e........e.... ...e....e..e..e... 318 OPEN FETAL SURGERY .....e....e..e.=...........e...319 FETOSCOPIC SURGERY ...........e.........e...... 321


Obstentrics_Williams

Obstentrics_Williams. SURGICAL THERAPY ..e........e.... ...e....e..e..e... 318 OPEN FETAL SURGERY .....e....e..e.=...........e...319 FETOSCOPIC SURGERY ...........e.........e...... 321 PERCUTANEOUS PROCEDURESe. . .. .............. 324 EX-UTERO INTRAPARTUM TREATMENT ............e. 327 Minor grades of hydramnios rarey require active treatment. On the other han, when the abdomen is immensey distended and respiration is seriousy hampere, the termination of pregnancy is urgenty indicate. In such cases, the symptoms can be prompty relieved by peorating the membranes through the cervix, after which the amniotic luid drains of and labour pains set in.


Obstentrics_Williams

Obstentrics_Williams. -J. Whitridge Williams (1903) he concept of fetal therapy-even amniocentesis-was not considered by Williams in his irst edition. Aside from a few destructive procedures to aid vaginal delivery, any type of fetal treatment is not mentioned as even a remote possibility. Again, fast forward to this 25th edition, when interventions developed during the past three decades have dramatically altered the course of selected fetal anomalies and conditions. Reviewed in this chapter are fetal disorders amenable to treatment with either maternal medication or surgical procedures. he management of fetal anemia and thrombocytopenia is reviewed in Chapter 15, and treatment of some fetal infections is discussed in Chapters 64 and 65. Fetal pharmacotherapy uses medications administered to the mother and then transported transplacentally to the fetus. s described here, it can be used to treat narray of serious conditions.


Obstentrics_Williams

Obstentrics_Williams. Fetal pharmacotherapy uses medications administered to the mother and then transported transplacentally to the fetus. s described here, it can be used to treat narray of serious conditions. Fetal cardiac rhythm disturbances may be broadly categorized as tachyarrhythmias, heart ratesi> 180 beats per minute (bpm); bradyarrhythmia, heart rate < 110 bpm; and ectopy, typically premature atrial contractions. If these are identiied, fetal M-mode sonography is performed to measure the atrial and ventricular rates and to clariY the relationship between atrial and ventricular beats, thereby diagnosing the type of rhythm disturbance.


Obstentrics_Williams

Obstentrics_Williams. This is by far the most common arrhythmia and is identiied in 1 to 2 percent of pregnancies (Hahurij, 2011; Strasburger, 2010). Generally a benign inding, premature atrial contractions represent immaturity of the cardiac conduction system, and they typically resolve later in gestation or in the neonatal period. If the premature atrial contraction is conducted, it sounds like an extra beat when auscultated with handheld Doppler or fetoscope. However, premature atrial contractions are more commonly blocked and sound like dropped beats.


Obstentrics_Williams

Obstentrics_Williams. In general, premature atrial contractions are not associated with major structural cardiac abnormalities, although they sometimes occur with an atrial septal aneurysm. As shown in Figure 10-34 (p. 205), M-mode evaluation demonstrates that the dropped beat is a compensatory pause following the premature atrial contraction. They may occur as frequently as every other beat, known as blocked atrial bigeminy. This results in an auscultated fetal ventricular rate as low as 60 to 80 bpm. Unlike other causes of bradycardia, atrial bigeminy is benign and does not require treatment (Strasburger, 2010).


Obstentrics_Williams

Obstentrics_Williams. Approximately 2 percent of fetuses with premature atrial contractions are later found to have a supraventricular tachycardia (Copel, 2000; Srinivasan, 2008). Given the importance of identiying and treating supraventricular tachyarrhythmias, a fetus with premature atrial contractions is often monitored with heart rate assessment every 1 to 2 weeks until the ectopy resolves. This requires neither sonography nor fetal echocardiography, as the rate and rhythm may be easily ascertained with handheld Doppler.


Obstentrics_Williams

Obstentrics_Williams. The two most common tachyarrhythmias are supraventricular tachycardia (ST) and atrial lutter. SVT is characterized by an abrupt increase in the fetal heart rate to 180 to 300 bpm with 1: 1 atrioventricular concordance. he typical range is 200 to 240 bpm. ST may develop secondary to an ectopic focus or to an accessory atrioventricular pathway leading to a reentrant tachycardia. Atrial lutter is characterized by a much higher atrial rate, generally 300 to 500 bpm, with varying degrees of atrioventricular block. As a result, the ventricular rate in a fetus with atrial flutter may range from below normal to approximately 250 bpm (Fig. 16-1). In contrast, fetal sinus tachycardia typically presents with a gradual heart rate rise to a rate that is only slightly above normal. With this, readily discernible causes may be maternal fever or hyperthyroidism, or rarely, fetal anemia or infection.


Obstentrics_Williams

Obstentrics_Williams. If a fetal tachyarrhythmia is identified, it is important to determine whether it is sustained-deined as present for at least 50 percent of the time. It may be necessary to monitor the fetal heart rate for 12 to 24 hours upon initial detection, and then periodically to reassess (Srinivasan, 2008). Unsustained or intermittent tachyarrhythmias generally do not require treatment, provided that fetal surveillance is reassuring.


Obstentrics_Williams

Obstentrics_Williams. Sustained fetal tachyarrhythmia with ventricular rates exceeding 200 bpm impairs ventricular illing to a degree that the risk for hydrops is significant. With atrial flutter, lack of coordinated atrioventricular contractions may further compound this risk. Maternal administration of antiarrhythmic agents that cross the placenta may convert the rhythm to normal or lower the baseline heart rate to forestall heart failure. Therapy may require dosages at the upper end of the therapeutic adult range. Thus, a maternal electrocardiogram is obtained before and during therapy.


Obstentrics_Williams

Obstentrics_Williams. Antiarrhythmic medications most commonly used include digoxin, sotalol (Betapace), flecainide (Tambocor), and procainamide (Pronestyl). heir selection depends on the type of tachyarrhythmia as well as provider familiarity and experience with the drug. Traditionally, digoxin has been the initial preferred treatment, although it may poorly transfer to the fetus after hydrops has developed. Many centers now use lecainide or sotalol as first-line therapy Gaeggi, 2011; Shah, 2012). In many cases, additional agents are needed, particularly if hydrops has developed. SVT is generally more likely than atrial flutter to convert to a normal rhythm. With either arrhythmia, however, the overall neonatal survival rate now exceeds 90 percent (Ekman-Joelsson, 2015; Jaeggi, 2011; van der Heijden, 2013).


Obstentrics_Williams

Obstentrics_Williams. FIGURE 16-1 Atrial flutter. In this M-mode image at 28 weeks' gestation, calipers mark the ventricular rate, which is approximately 225 bpm. There are two atrial beats (A) for each ventricular beat (), such that the atrial rate is approximately 450 bpm with 2:1 atrioventricular block. The most common etiology of pronounced fetal bradycardia is congenital heart block. Approximately 50 percent of cases occur in the setting of a structural cardiac abnormality involving the conduction system. hese include heterotaxy, in particular ltatrial isomerism; endocardial cushion deect; and less commonly corrected transposition of the great vessels (Srinivasan, 2008). he prognosis of heart block secondary to a structural cardiac anomaly is extremely poor, and fetal loss rates exceed 80 percent (Glatz, 2008; Strasburger, 2010).


Obstentrics_Williams

Obstentrics_Williams. In a structurally normal heart, 85 percent of atrioventricular block cases develop secondary to transplacental passage of maternal anti-SSA/Ro or anti-SSB/La antibodies (Buyon, 2009). Many of these women have, or subsequently develop, systemic lupus erythematosus or other connective tissue disease (Chap. 59, p. 1142). he risk of third-degree heart block with these antibodies is small-only about 2 percent. But, the risk may reach 20 percent if a prior infant has been afected. Immune-mediated congenital heart block confers a mortality rate of 20 to 30 percent, requires permanent pacing in two thirds of surviving children, and also poses a risk for cardiomyopathy (Buyon, 2009). If associated with efusions, bradyarrhythmias, or endocardial ibroelastosis, neonatal status may progressively worsen after birth (Cuneo, 2007). Initial research eforts focused on maternal corticosteroid therapy to potentially reverse fetal heart block or forestall it.


Obstentrics_Williams

Obstentrics_Williams. Initial research eforts focused on maternal corticosteroid therapy to potentially reverse fetal heart block or forestall it. Friedman and colleagues (2008, 2009) conducted a prospective multicenter trial of pregnancies with anti-SSA/Ro antibodies the PR Interval and Dexamethasone (PRIDE) study. Weekly sonographic surveillance was performed, and heart block was treated with maternal oral dexamethasone 4 mg daily. Unfortu nately, progression from second-to third-degree block was not prevented with maternal dexamethasone therapy, and third degree atrioventricular block was irreversible. In rare cases, there block. However, irst-degree block did not generally progress even without treatment. In a subsequent review of 156 preg nancies with isolated second-or third-degree fetal heart block, sion, need for pacemaker in the neonatal period, or overall sur vival rates (Izmirly, 2016). hus, dexamethasone use cannot be recommended for this indication.


Obstentrics_Williams

Obstentrics_Williams. More recent eforts have turned to potential therapy with hydroxychloroquine (Plaquenil), a mainstay of treatment for systemic lupus erythematosus (Chap. 59, p. 1142). In a multi center review of more than 250 pregnancies in women whose prior pregnancies had been complicated by neonatal lupus, recurrence of congenital heart block was signiicantly lower if the woman had been treated with hydroxychloroquine during pregnancy (Izmirly, 2012). Research in this area is ongoing. Maternal terbutaline has also been given to increase the fetal heart rate in cases with sustained bradycardia of any cause in which the fetal heart rate is below 55 bpm. Reversal of hydrops with this therapy has been reported (Cuneo, 2007, 2010).


Obstentrics_Williams

Obstentrics_Williams. Several autosomal recessive enzyme deiciencies cause impaired fetal synthesis of cortisol from cholesterol by the adrenal cortex. his results in congenital adrenal hyperplasia (CAH). CAH is the most common etiology of androgen excess in females with 46,X disorders of sex development, formerly female pseudohermaphroditism (Chap. 3, p. 41). Lack of cortisol stimulates adrenocorticotrophic hormone (ACTH) secretion by the anterior pituitary, and the resulting androstenedione and testosterone overproduction leads to virilization of female fetuses. Sequelae may include formation of labioscrotal folds, persistence of a urogenital sinus, or even creation of a penile urethra and scrotal sac.


Obstentrics_Williams

Obstentrics_Williams. More than 90 percent ofCAH cases are caused by 21-hydroxylase deficiency, which is found in classic and nonclassic forms. he incidence of classic CAH approximates 1: 15000 births overall and is higher in selected populations. For example, it has been reported in approximately 1 :300 Yupik Eskimos (Nimkarn, 2010). Among those with classic CAH, 75 percent are at risk for salt-wasting adrenal crises and require postnatal treatment with mineralocorticoids and glucocorticoids to prevent hyponatremia, dehydration, hypotension, and cardiovascular collapse. The remaining 25 percent with classic CAH have the simple virilizing ype and also require glucocorticoid supplementation. As discussed in Chapter 32 (p. 614), all states mandate newborn screening for CAH.


Obstentrics_Williams

Obstentrics_Williams. he eicacy of maternal dexamethasone treatment to suppress fetal androgen overproduction and either obviate or ameliorate virilization of female fetuses has been recognized for more than 30 years (David, 1984; New, 2012). Prenatal corti costeroid therapy is considered successful in 80 to 85 percent of cases (Miller, 2013; Speiser, 2010). The alternative is con sideration of postnatal genitoplasty, a complex and somewhat controversial surgical procedure (Braga, 2009). he tpical preventive regimen is oral dexamethasone given to the mother at a dosage of 20 Lg/kg/d-up to 1.5 mg per day, divided in three doses. The critical period for external genitlia development is 7 to 12 weeks' gestation, and treatment to pre vent virilization should be initiated by 9 wees-bore it is known whether the etus is at risk. Because this is n autosomal recessive condition, ffected females mke up only 1 in 8 at-risk conceptions.


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Obstentrics_Williams. Typically, carrier parents are identified after the birth of an afected child. Molecular genetic testing is clinically available, initially using sequence analysis of the CP21A2 gene, which encodes the 21-hydroxylase enzyme (Nimkarn, 2016). If this is uninformative, gene-targeted deletion/duplication analy sis is performed, and additional testing such as whole exome sequencing may be considered (Chap. 13, p. 272). A goal of prenatal diagnosis is to limit dexamethasone expo sure in males and in unafected females. Prenatal diagnosis villi-at 10 to 12 weeks' gestation-or on amniocytes after 15 weeks. Cell-free DNA testing of maternal serum has potential to replace invasive tests such as chorionic villus sampling and amniocentesis for CAH (Chap. 13, p. 273). Determination of (Devaney, 2011). In the research setting, cell-free DNA testing using hybridization probes lanking the CP21A2 gene can be efective as early as 5617 weeks' gestation (New, 2014).


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Obstentrics_Williams. Maternal treatment with dexamethasone has become a topic of signiicant controversy. The Endocrine Society recommends that treatment be given only in the context of research protocols (Miller, 2013; Speiser, 2010). It should be noted that if therapy is initiated shortly before 9 weeks, the dose of dexamethasone used is not considered to have significant teratogenic potential because organogenesis of major organs has already taken place (McCullough, 2010). Ongoing concerns, however, focus on the potential efects of either excess endogenous androgens or excess exogenous dexamethasone on the developing brain. Although maternal dexamethasone has been used for many years to prevent virilization of female fetuses with CAH, longterm safety data are relatively limited.


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Obstentrics_Williams. Sonographically, this malformation is a well-circumscribed lung mass that may appear solid and echogenic or may have one or multiple variably sized cysts (Fig. 10-24, p. 199). Lesions with cysts � 5 mm are termed macrocystic, whereas microcystic lesions have smaller cysts or appear solid (Adzick, 1985). Also called congenital pulmonary airway malformation (CPAM), it represents a hamartomatous overgrowth of terminal bronchioles. Therapy for macrocystic congenital cystic adenomatoid malformation (CCAM) is discussed later (p. 324).


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Obstentrics_Williams. Occasionally, a microcystic CCAM may demonstrate rapid growth, generally between 18 and 26 weeks' gestation. he mass may become so large that it causes mediastinal shift, which may compromise cardiac output and venous return, resulting in hydrops (Cavoretto, 2008). A CCAM-volume ratio (CVR) has been used to quantiy size and risk for hydrops in these severe cases (Crombleholme, 2002). his ratio is an estimate of the CCAM volume (length X width X height X 0.52) divided by the head circumference. In a series of 40 pregnancies with microcystic CCAM, the mean CVR was 0.5 at 20 weeks' gestation, peaking in size at 1.0 at 26 weeks, followed by a pronounced decline prior to delivery (Macardle, 2016). A third of fetuses had no increase in mass size. In the absence of a dominant cyst, a CVR exceeding 1.6 is associated with a hydrops risk as high as 60 percent. However, CCAM growth resulting in hydrops develops in fewer than 2 percent of cases if the initial CVR is below 1.6 (Ehrenberg-Buchner,


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Obstentrics_Williams. 1.6 is associated with a hydrops risk as high as 60 percent. However, CCAM growth resulting in hydrops develops in fewer than 2 percent of cases if the initial CVR is below 1.6 (Ehrenberg-Buchner, 2013; Peranteau, 2016). Importantly, a CVR in the range of 1.6 indicates that the mass essentially fills the thorax, and thus it is not unexpected that ascites or hydrops may develop.


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Obstentrics_Williams. If the CVR exceeds 1.6 or if signs of hydrops develop, corticosteroid treatment has been used in an efort to improve outcome. Regimens include dexamethasone-6.25 mg every 12 hours for four doses, or betamethasone-12.5 mg intramuscularly every 24 hours for two doses. Following a single course of corticosteroids, hydrops resolved in approximately 80 percent of cases, and 90 percent of treated fetuses survived (Loh, 2012; Peranteau, 2016). Recently, multiple courses of steroids-generally twohave been advocated for fetuses with large CCAM lesions and with persistent or worsening hydrops or ascites despite a single course of medication (Derderian, 2015; Peranteau, 2016).


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Obstentrics_Williams. Identification of fetal thyroid disease is rare and usually prompted by sonographic detection of a fetal goiter. If a goiter is found, determination of fetal hyper-or hypothyroidism is essential, and thyroid hormone levels may be measured in amnionic luid or fetal blood. Traditionally, fetal blood sampling, described in Chapter 14 (p. 294), is preferred to amniocentesis for guiding treatment, although data are limited (Abuhamad, 1995; Ribault, 2009). Goals of therapy are correction of the physiological abnormality and diminished goiter size. The goiter may compress the trachea and esophagus to such a degree that severe hydramnios or neonatal airway compromise may develop. Hyperextension of the fetal neck by a goiter can create labor dystocia.


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Obstentrics_Williams. Untreated fetal thyrotoxicosis may present with goiter, tachycardia, growth restriction, hydramnios, accelerated bone maturation, and even heart failure and hydrops (Huel, 2009; Peleg, 2002). The cause is usually maternal Graves disease with transplacental passage ofIgG thyroid-stimulating immunoglobulins. Fetal blood sampling may confirm the diagnosis (Duncombe, 2001; Heckel, 1997; Srisupundit, 2008). Conirmed fetal thyrotoxicosis is followed by maternal antithyroid treatment. During this, if the mother develops hypothyroidism, she is given supplementalilevothyroxine (Hui, 2011). In a woman receiving medication for Graves disease, transplacental passage of methimazole or propylthiouracil may cause etal hypothyroidism (Bliddal, 2011 a). Other potential causes of fetal hypothyroidism resulting in goiter include transplacental passage of thyroid peroxidase antibodies, fetal thyroid dyshormonogenesis, and maternal overconsumption of iodine supple ments (Agrawal, 2002; Overcash, 2016).


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Obstentrics_Williams. Goitrous hypothyroidism may lead to hydramnios, neck hyperextension, and delayed bone maturation. If the mother is receiving antithyroid medication, discontinuation is generally recommended, along with intraamnionic levothyroxine injection. Numerous case reports describe intraamnionic levothyroxine treatment. However, optimal dosage and frequency have not been established, and reported dosages range from 50 to 800 �g every 1 to 4 weeks (Abuhamad, 1995; Bliddal, 2011b; Ribault, 2009) .


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Obstentrics_Williams. Also called maternaletal surgery, these procedures are ofered for selected congenital abnormalities in which the likelihood of fetal deterioration is so great that delaying treatment until after delivery would risk fetal death or substantially greater postnatal morbidity. Open fetal surgef) is a highly specialized intervention performed at relatively few centers in the United States and for only a few fetal conditions. Criteria for consideration of fetal surgery are listed in Table 16-1. In many cases, data regarding the safety and eicacy of these procedures are limited. TABLE 16-1. Guiding Principles for Fetal Surgical Procedures Accurate prenatal diagnosis for the defect is available, with staging if applicable The defect appears isolated, with no evidence of other abnormality or underlying genetic syndrome that would significantly worsen survival or quality of life


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Obstentrics_Williams. The defect appears isolated, with no evidence of other abnormality or underlying genetic syndrome that would significantly worsen survival or quality of life The defect results in a high likelihood of death or irreversible organ destruction, and postnatal therapy is inadequate The procedure is technically feaSible, and a multidisciplinary team is in agreement regarding the treatment plan Maternal risks from the procedure are well documented and considered acceptable There is comprehensive parental counseling It is recommended that there be an animal model for the defect and procedure Data from Deprest, 201s0; Harrison, 1982; Vrecenak, 2013; Walsh, 201s1. TABLE 16-2. Selected Fetal Abnormalities Amenable to Fetal Surgery Twin-twin transfusion: laser of placental anastomoses Diaphragmatic hernia: fetal endoscopic tracheal occlusion Posterior urethral valves: cystoscopic laser Congenital high airway obstruction: vocal cord laser Amnionic band release


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Obstentrics_Williams. Posterior urethral valves: cystoscopic laser Congenital high airway obstruction: vocal cord laser Amnionic band release Posterior-urethral valves/bladder outlet obstruction Pleural effusion: chylothorax or sequestration Dominant cyst in CCAM Twin-reversed arterial perfusion (TRAP) sequence Monochorionic twins with severe anomaly in 1 twin Chorioangioma Aortic or pulmonic valvuloplasty for stenosis Atrial septostomy for hypoplastic left heart with EXIT-to-resection: resection of fetal thoracic or mediastinal mass EXIT-to-extracorporeal membrane oxygenation (E(MO): congenital diaphragmatic hernia he Agency for Healthcare Research and Quality stresses that when considering fetal surgery, the overriding concern must be maternal and fetal safety. Accomplishing the fetal goals of the procedure is secondary (Walsh, 2011).


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Obstentrics_Williams. Some abnormalities amenable to fetal surgical treatment, antepartum or intrapartum, are shown in Table 16-2. An overview of these procedures, their indications, and complications is provided here to assist with initial patient evaluation and counseling. Additional content is also found in Cunningham and Gilstrap s Operative Obstetrics, 3rd edition.


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Obstentrics_Williams. These procedures require extensive preoperative counseling and multidisciplinary care. he mother must undergo general endotracheal anesthesia to suppress both uterine contractions and fetal responses. Using intraoperative sonographic guidance to avoid the placental edge, a low-transverse hysterotomy incision is made with a stapling device that seals the edges for hemostasis. To replace amnionic fluid losses, warmed luid is continuously infused into the uterus thorough a rapid infusion device. he fetus is gently manipulated to permit pulse oximetry monitoring and to establish venous access, in case luids or blood are emergently needed. The surgical procedure is then performed. After completion, the hysterotomy is closed and tocolysis begun. T ocolysis typically includes intravenous magnesium sulfate for 24 hours, oral indomethacin for 48 hours, and, at some centers, oral nifedipine until delivery (Wu, 2009). Prophylactic antibiotics are also administered and generally continued for 24


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Obstentrics_Williams. sulfate for 24 hours, oral indomethacin for 48 hours, and, at some centers, oral nifedipine until delivery (Wu, 2009). Prophylactic antibiotics are also administered and generally continued for 24 hours following the procedure. Cesarean delivery is needed later in gestation and for all future deliveries.


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Obstentrics_Williams. Morbidities associated with fetal surgery are well characterized. In a review of 87 open procedures, Golombeck and coworkers (2006) reported the following morbidities: pulmonary edema-28 percent, placental abruption-9 percent, blood transfusion-13 percent, premature rupture of membranes-52 percent, and preterm delivery-33 percent. Wilson and associates (2010) reviewed subsequent pregnancy outcomes following open fetal surgery and reported that 14 percent of women experienced uterine rupture and 14 percent had uterine dehiscence. Morbidities identiied in the recent Management of Myelomeningocele Study (MOMS) are shown in Table 16-3 (Adzick, 2011). Other potential risks include maternal sepsis and fetal death during or following the procedure, particularly if hydrops is present.


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Obstentrics_Williams. Even with postnatal repair, children with myelomeningocele generally have varying degrees of paralysis, bladder and bowel dys function, developmental delays, and brainstem dysfunction from the Arnold-Chiari II malformation (Chap. 10, p. 193). Damage is postulated to result from abnormal embryonic neurulation and from ongoing exposure of neural elements to amnionic luid (Adzick, 2010; Meuli, 1995, 1997). Fetal myelomeningocele meets the criteria listed in Table 16-1 and is the first nonlethal birth defect for which fetal surgery has been ofered (Fig. 16-2).


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Obstentrics_Williams. In preliminary reports, infants following antepartum defect repair were more likely to have reversal of the Arnold-Chiari II malformation and were less likely to require ventriculoperitoneal shunt placement (Bruner, 1999; Sutton, 1999). Spurred by this, the randomized, multicenter MOMS trial was conducted (Adzick, 2011). Criteria for trial participation included: (1) a singleton fetus at 19.0 to 25.9 weeks' gestation; (2) an upper myelomeningocele boundary between T1 and S 1 conirmed by fetal magnetic resonance (MR) imaging; (3) evidence of hindbrain herniation; and (4) a normal karyotype and no evidence of a fetal anomaly unrelated to the myelomeningocele. Women at risk for preterm birth or placental abruption, those with a contraindication to fetal surgery, and women with body mass indexi> 35 kg/m2 were excluded.


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Obstentrics_Williams. he MOMS indings demonstrated improved early childhood outcomes in the prenatal surgery cohort (see Table 16-3). Children who had undergone prenatal surgery were twice as likely to walk independently by 30 months. hey had signiicantly less hindbrain herniation and were only half Composite developmental scorea,b TABLE 16-3. Benefits and Risks of Fetal 68% 98% <0.001 40% 82% <0.001 149 ± 58 123 ± 57 0.007 64% 96% <0.001 20% 48% <0.001 42% 21% 0.01 6% 0 0.03 6% 0 0.03 9% 1% 0.03 21% 4% 0.001 34 ± 3 37 ± 1 <0.001 79% 15% <0.001 aEach primary outcome had two components. The perinatal death components of the primary outcomes as well as the Bayley Mental Development Index at 30 months did not differ between the two study cohorts. bScore derived from Bayley Mental Development Index and difference between functional and anatomical level of lesion (30 months). Data from Adzick, 201s1s.


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Obstentrics_Williams. FIGURE 16-2 Fetal myelomeningocele surgery. A. With the edges of both the laparotomy and hysterotomy incisions retracted, the skin around the defect is incised. Subsequently, the neural placode is sharply dissected from the arachnoid membrane. B. The dural membrane is reflected to the midline to cover the neural placode and is reapproximated using suture. In some cases a patch is needed (not shown). The fetal skin incision is subsequently sutured. Last, hysterotomy and laparotomy are then closed. (Reproduced with permission from Shamshirsaz AA, Ramin, SM, Belfort MA: Fetal therapy. In Yeomans ER, Hoffman BL, Gilstrap LC III, et al: Cunningham and Gilstrap's Operative Obstetrics, 3rd ed. New York, McGraw-Hili Education, 201o7.) as likely to undergo ventriculoperitoneal shunting by the age of 1 year. A primary outcome was a composite score that was derived from the Bayley Mental Development Index and from the diference between the functional and anatomical level of the lesion at 30


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Obstentrics_Williams. age of 1 year. A primary outcome was a composite score that was derived from the Bayley Mental Development Index and from the diference between the functional and anatomical level of the lesion at 30 months. This primary outcome was also signii cantly better in the prenatal surgery group.


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Obstentrics_Williams. When counseling prospective families, however, results are placed in perspective. For example, despite improvements in the proportion with independent ambulation, most children who received fetal surgery were not able to ambulate inde pendently, and nearly 30 percent were not able to ambulate at all. Prenatal surgery did not confer improvements in fetal or neonatal death rates or in the Bayley Mental Development Index score at age 30 months. And, as shown in Table 16-3, surgery was associated with a small but significant risk for pla cental abruption and maternal pulmonary edema. Moreover, nearly half were delivered before 34 weeks, which signiicantly increased the risk for respiratory distress syndrome (Adzick, 201l). Long-term surveillance data have only recently become cele repair prior to the MOMS trial. At a median follow-up of 10 years, these children have higher rates of behavioral problems and adverse executive functioning compared with population norms (Danzer, 2016).


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Obstentrics_Williams. Since publication of the MOMS findings, fetal myelomeningocele surgery rates have grown. Expansion of centers ofering this procedure has raised concerns about the importance of training and ongoing experience, adherence to the MOMS research criteria, and need for a registry to ensure that future eforts have similar success rates (Cohen, 2014; Vrecenak, 20l3).


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Obstentrics_Williams. In the past, if hydrops developed in a fetus with a large pulmonary sequestration or cystic adenomatoid malformation without a dominant cyst, open fetal surgery with lobectomy was the only treatment available other than preterm delivery. Most thoracic masses are small and have a benign prognosis, and larger masses are generally treated with corticosteroids (p. 318). Fetal surgery is generally reserved for cases prior to 32 weeks in which hydrops is developing, and in selected cases, the survival rate following open lobectomy approximates 60 percent (Vrecenak, 20l3). Use of the ex-utero intrapartum treatment procedure in the treatment of fetal lung masses at delivery is discussed later on page 327.


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Obstentrics_Williams. This germ cell tumor has a prevalence of approximately 1 per 28,000 births (Derikx, 2006; Swamy, 2008). Sonographically, a sacrococcygeal teratoma (SCT) is a solid and/or cystic mass that arises from the anterior sacrum (Fig. 16-3). Fetal MR imaging can aid evaluation of the extent of the internal tumor component. he mass may grow rapidly, usually extending inferiorly and externally (Fig. 10-18, p. 196). Hydramnios is common, and hydrops may develop from high-output cardiac failure, either as a consequence of tumor vascularity or secondary to bleeding within the tumor and resultant anemia. Mirror syndrome-maternal preeclampsia developing along with fetal hydrops-may occur in this setting (Chap. 15, p. 312). FIGURE 16-3 Fetal surgery for sacrococcygeal teratoma resection. Following laparotomy and hysterotomy, the caudal portion of the fetus has been delivered onto the surgical field. The tumor is held by the surgeon's hand. (Used with permission from Dr. Timothy M. Crombleholme.)


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Obstentrics_Williams. The perinatal mortality rate for cases of SCT diagnosed prenatally approximates 40 percent (Hedrick, 2004; Shue, 20l3). Poor prognostic factors include a solid component comprising more than 50 percent of the tumor mass and a tumor volume-to-fetal weight ratio (tumor volume divided by estimated fetal weight) exceeding 12 percent prior to 24 weeks' gestation (Akinkuotu, 2015). Fetal loss rates approach 100 percent if hydrops or placentomegaly develop (Vrecenak, 20l3). The group at the Children's Hospital of Philadelphia recommends consideration of open fetal surgery for SCT only in cases in which the tumor is completely external (Type I) and in which high cardiac output with early hydrops has developed in the second trimester (Vrecenak, 2013). For excision, hysterotomy is performed, and the external component is resected. he coccyx and any deep tumor are left in place for postnatal removal. Because tumor debulking interrupts the pathological vascular steal, normal fetal physiology may


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Obstentrics_Williams. external component is resected. he coccyx and any deep tumor are left in place for postnatal removal. Because tumor debulking interrupts the pathological vascular steal, normal fetal physiology may be restored.


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Obstentrics_Williams. As with open fetal surgeries, these procedures are performed at highly specialized centers, and some are considered investigational. To accomplish them, iberoptic endoscopes only 1 to 2 mm in diameter are used to penetrate the maternal abdominal wall, the uterine wall, and membranes. Instruments such as lasers fit through 3-to 5-mm cannulas that surround the endoscope. vIorbidities are generally lower than with open fetal surgery, but they still may be formidable, particularly if maternal laparotomy is required for access (Golombeck, 2006). Examples of some conditions treated by fetoscopy are listed in Table 16-2. Indications and Technique. As discussed in Chapter 45 (p. 879), fetoscopic laser ablation of placental anastomoses is the preferred management for severe twin-twin transfusion syndrome (TTTS). It is generally performed between 16 and


Obstentrics_Williams

Obstentrics_Williams. FIGURE 16-4 Selective laser photocoagulation for twin-twin transfusion syndrome. The fetoscope is inserted into the recipienttwin sac and positioned over the vascular equator, which lies in between the two placental cord insertion sites. Arteriovenous anastomoses along the placental surface are individually photocoagulated using the laser. (Reproduced with permission from Shamshirsaz AA, Ramin, SM, Belfort MA: Fetal therapy. In Yeomans ER, Hoffman BL, Gilstrap LC III, et al: Cunningham and Gilstrap's Operative Obstetrics, 3rd ed. New York, McGraw-Hili Education, 201o7.) 26 weeks' gestation for monochorionic-diamnionic twin pregnancies with stage II to stage IV TTTS. These categories of the Quintero Staging System are described in Chapter 45 (p. 879) (Quintero, 1999; Society for Maternal-Fetal Medicine, 2013).


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Obstentrics_Williams. For the procedure, a fetoscope is used to view the vascular equator that separates the placental cotyledons supplying each twin (Fig. 16-4). Arteriovenous anastomoses along the placental surface of the vascular equator are photocoagulated using a 600-�m diameter diode laser or a 400-�m neodymium:yttriumaluminum-garnet (Nd:YAG) laser (Fig. 16-5). he procedure is typically performed under epidural or local analgesia. At the end, amnioreduction is performed to decrease the single deepest pocket of amnionic luid to below 5 cm, and antibiotics are injected into the amnionic cavity.


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Obstentrics_Williams. With selective laser photocoagulation, anastomoses crossing between the twins along the vascular equator are individually coagulated (Ville, 1995). Unfortunately, residual anastomoses remain in up to a third of cases and may lead to TTTS recurrence or to the development of twin-anemia polycythemia sequence (TAPS). The latter is a feto-fetal transfusion characterized by large diferences in hemoglobin concentrations between a pair of monochorionic twins. To address these complications, the Solomon technique was developed. With this, after selective photocoagulation, the laser is used to coagulate the entire vascular equator, from one edge of the placenta to the other (Slaghekke, 2014a). he Solomon technique lowers the proportion of pregnancies with recurrent TTTS and TAPS in multiple trials. Also, placental dye-injection studies confirm a significant reduction in the number of residual anastomoses (Ruano, 2013; Slaghekke, 2014b).


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Obstentrics_Williams. Complications. Families should have reasonable expectations of procedural success and potential complications. Without treatment, the perinatal mortality rate for severe TTTS is 70 to 100 percent. Following laser therapy, the anticipated perinatal mortality rate approximates 30 to 50 percent, with a 5-to 20-percent risk for long-term neurological handicap (Society for Maternal-Fetal Medicine, 2013). Cystic periventricular leukomalacia and grade III to IV interventricular hemorrhage are identiied neonatally in up to 10 percent of laser-treated cases (Lopriore, 2006).


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Obstentrics_Williams. Procedure-related complications include preterm prematurely ruptured membranes in up to 25 percent, placental abruption in 8 percent, vascular laceration in 3 percent, amnionic band syndrome resulting from laser laceration of the membranes in 3 percent, and TAPS in 16 percent with photocoagulation and 3 percent with the Solomon modification (Habli, 2009; Robyr, 2006; Slaghekke, 2014b). Finally, most laser-treated TTTS pregnancies deliver before 34 weeks.


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Obstentrics_Williams. FIGURE 16-5 Fetoscopic photograph of laser photocoagulation for twin-twin transfusion syndrome. A. Vascular anastomoses (arrows) are shown before photocoagulation is performed. B. The ablation sites appear as blanched yellow-white areas (arrows). (Used with permission from Dr. Timothy M. Crombleholme.) he prevalence of congenital diaphragmatic hernia (CDH) is approximately 1 in 3000 to 4000 births, and the overall sur vival rate is 50 to 60 percent. Associated anomalies occur in 40 percent of cases and confer a considerably lower survival rate. he main causes of mortality among those with isolated CDH are pulmonary hypoplasia and pulmonary hypertension. And, the major risk factor is liver herniation, which complicates at least half of cases and is associated with a 30-percent reduction in the survival rate (vfullassery, 2010, Oluyomi-Obi, 2017).


Obstentrics_Williams

Obstentrics_Williams. Because of maternal and fetal risks associated with fetal surgical intervention, eforts have focused on identiying those least likely to survive with postnatal therapy alone. Fetuses with associated anomalies are typically excluded, as are those with out liver herniation. Prediction is further hampered because of improvements in neonatal care for newborns with CDH. These include permissive hypercapnia, "gentle ventilation" to avoid barotrauma, and delayed surgery.


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Obstentrics_Williams. Lung-to-Head Ratio. This sonographic ratio was developed to improve prediction of survival in fetuses with isolated leftsided CDH diagnosed before 25 weeks' gestation (Metkus, 1996). he lung-to-head ratio (LHR) is a measurement of the right lung area, taken at the level of the four-chamber view of the heart, divided by the head circumference (Fig. 10-23, p. 198). Investigators found that the survival rate was 100 percent if the LHR wasi> 1.35, and there were no survivors if it was <0.6. Nearly three fourths of pregnancies had values between 0.6 and 1.35, and prediction was diicult in this large group because the overall survival rate approximated 60 percent (Metkus, 1996).


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Obstentrics_Williams. As of 2017, trials underway have selected a threshold LHR of < 1.0 or an observed-to-expected LHR <25 percent for study inclusion. An observed LHR is obtained sonographically from the afected fetus, whereas rhe expected LHR is an established reference value from normal fetuses (Peralta, 2005). In a recent metaanalysis, the odds ratio for survival with an LHR < 1.0 was only 0.14 (Oluyomi-Obi, 2017). Similarly, with an observed-to-expected LHR <25 percent, survival rates ranged from 13 to 30 percent. In contrast, an observed-to-expected LHR > 35 percent was associated with survival rates ranging from 65 to 88 percent.


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Obstentrics_Williams. Magnetic Resonance Imaging. This has been used to estimate the total volume of lung tissue, both ipsilateral and contralateral to the diaphragmatic hernia, which may then be compared with a gestational age-matched reference. Mayer and coworkers (2011) performed a metaanalysis of 19 studies involving more than 600 pregnancies in which isolated CDH was evaluated with feral MR imaging. Factors signiicantly associated with neonatal survival included the side of the defect, total fetal lung volume, observed-to-expected lung volume, and fetal liver position.


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Obstentrics_Williams. Fetal MR imaging has also been used to quantiy the volume of herniated liver (Fig. 10-57, p. 219). Two reasons underlie the rationale for assessing liver volume. First, liver herniation is perhaps the strongest predictor of outcome in fetuses with isolated CDH. Second, liver volume might be a more reliable predictor because lungs are inherently more compressible than liver. Indeed, these MR parameters-lung volumes and degree of liver herniation-correlate well with postnatal survival rates and may be more useful predictors than sonographic parameters (Bebbington, 2014; Ruano, 2014; Worley, 2009).


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Obstentrics_Williams. Tracheal Occlusion. Early attempts to treat severe diaphragmatic herniation used open fetal surgery to reposition the liver into the abdomen, which unfortunately kinked the umbilical vein and led to fetal demise (Harrison, 1993). Knowledge that lungs normally produce luid and that fetuses with upper airway obstruction develop hyperplastic lungs formed the rationale for tracheal occlusion. The idea was to "plug the lung until it grows" (Hedrick, 1994). Initial eforts focused on occluding the trachea with an external clip (Harrison, 1993). Subsequently, a detachable silicone balloon was placed within the trachea endoscopically (Fig. 16-6).


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Obstentrics_Williams. The balloon technique-termed etal endoscopic tracheal occlusion (FETO)-uses a 3-mm operating sheath and fetoscopes as small as 1 mm (Depresr, 2011; Ruano, 2012). he procedure is generally performed between 27 and 30 weeks' gestation, with the goal of removing the balloon at approximately 34 weeks, either through a second fetoscopic procedure or by ultrasound-guided puncture Gimenez, 2017). If these are unsuccessful, the balloon is removed during an ex-utero intrapartum treatment procedure at delivery (p. 327). FIGURE 16-6 Fetoscopic tracheal occlusion (FETO). The endoscope enters the fetal oropharynx and advances down the trachea. Inset: The balloon is inflated to occlude the trachea, and then the endoscope is removed. (Reproduced with permission from Shamshirsaz AA, Ramin, SM, Belfort MA: Fetal therapy. In Yeomans ER, Hoffman BL, Gilstrap LC III, et al: Cunningham and Gilstrap's Operative Obstetrics, 3rd ed. New York, McGraw-Hili Education, 2017.)


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Obstentrics_Williams. In 2003, a randomized trial of the FETO procedure in pregnancies with isolated CDH, liver herniation, and LHR < 1.4 did not identiY a benefit from fetal therapy (Harrison, 2003). Survival rates 90 days after birth were unexpectedly high in both groups and approximated 75 percent. Following this study, however, enthusiasm for the technique continued, particularly outside the United States. Using a lower LHR threshold of 1.0 in addition to liver herniation as prerequisites for inclusion, signiicantly higher postnatal survival rates have been reported. Rates improved from <25 percent with postnatal therapy to approximately 50 percent with FETO Gani, 2009; Ruano, 2012). In a recent metaanalysis of five trials that included 21i1 pregnancies, those treated with FETO were 13 times more likely to survive (Al-Maary, 2016). At present, FETO is available in the United States only in research trials.


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Obstentrics_Williams. After publication of the MOMS indings, research eforts focused on whether maternal morbidities associated with open fetal myelomeningocele repair might be mitigated if the procedure was accomplished endoscopically. Araujo Junior and associates (2016) conducted a systematic review that included 456 open cases and 84 endoscopic surgeries. he endoscopic procedures were generally performed by inserting instruments through the maternal abdominal wall and then through the uterine wall, with partial carbon dioxide insuiation of the uterus. he rate of maternal myometrial dehiscence or attenuation was only 1 percent following endoscopy compared with 26 percent following open procedures. However, endoscopy was associated with significantly increased rates of preterm delivery before 34 weeks-80 versus 45 percent, and of perinatal mortality-14 versus 5 percent.


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Obstentrics_Williams. Belfort and colleagues (2017) recently described their outcomes in 22 pregnancies with fetal myelomeningocele using a technique in which the maternal abdomen was opened, the uterus exteriorized, and the procedure then performed endoscopically using warmed carbon dioxide insuiation. In contrast with earlier endoscopic reports, most treated pregnancies were delivered at term, with no perinatal losses. Further, the proportion of infants requiring hydrocephalus treatment prior to 1 year of age-approximately 40 percent-was similar to that with open fetal surgery in the MOMS trial (Adzick, 2011; Belfort, 2017). Research eforts in this area will undoubtedly continue.


Obstentrics_Williams

Obstentrics_Williams. Sonographic guidance can be used to permit therapy with a shunt, radiofrequency ablation needle, or angioplasty catheter. With these procedures, desired instruments cross the maternal abdominal wall, uterine wall, and membranes to reach the amnionic cavity and fetus. Risks include maternal infection, preterm labor or prematurely ruptured membranes, and fetal injury or loss. A shunt placed from the fetal pleural cavity into the amnionic cavity may be used to drain pleural luid (Fig. 16-7). A large efusion may cause a significant mediastinal shift, resulting in pulmonary hypoplasia or in heart failure and hydrops. he most common etiology of a primary efusion is chylothoraxcaused by lymphatic obstruction. Pleural efusions may also form secondary to congenital viral infection or aneuploidy, or they may be associated with a malformation such as pulmonary


Obstentrics_Williams

Obstentrics_Williams. FIGURE 16-7 Thoracoamnionic shunt placement. A. A large, right-sided fetal pleural effusion (asterisks) and ascites were identified at 18 weeks' gestation. The effusion was drained but rapidly reaccumulated. The xanthochromic fluid contained 95-percent lymphocytes, consistent with chylothorax. B. A double-pigtail shunt (arrow) was inserted under ultrasound gUidance. Following shunt placement, the efusion and ascites resolved.


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Obstentrics_Williams. FIGURE 16-8 Vesicoamnionic shunt placement. A. After amnioinfusion is performed, a trocar is inserted into the distended fetal bladder under sonographic guidance. The pigtail catheter is threaded into the trocar. B. The double-pigtail shunt has been deployed down the trocar, and the trocar has been removed. The distal end of the shunt is coiled within the fetal bladder, and the proximal end is draining into the amnionic cavity. (Reproduced with permission from Shamshirsaz AA, Ramin, SM, Belfort MA: Fetal therapy. In Yeomans ER, Hoffman BL, Gilstrap LC III, et al: Cunningham and Gilstrap's Operative Obstetrics, 3rd ed. New York, McGraw-Hili Education, 201o7.) sequestration. Yinon and associates (2010) reported aneuploidy cent of cases.


Obstentrics_Williams

Obstentrics_Williams. Typically, the efusion is first drained using a 22-gauge needle with sonographic guidance. Tests for aneuploidy and infection are performed, as well as a cell count. A pleural-fluid cell count with greater than 80-percent lymphocytes, in the absence of infection, is diagnostic of chylothorax. If the luid reaccumulates, a trocar and cannula may be inserted through the fetal chest wall, and a double-pigtail shunt may be placed to drain the efusion. If the efusion is right-sided, the shunt is placed in the lower third of the chest to permit maximum expansion of the lung. If left-sided, the shunt is placed along the upper axillary line to allow the heart to return to normal position (Mann, 2010). he overall survival rate is 70 percent, and that for hydropic fetuses approximates 50 percent (Mann, 2010; Yinon, 2010). Shunt displacement into the amnionic cavity is not uncommon. If the shunt remains in place, it must be clamped immediately upon delivery of the newborn to avoid pneumothorax.


Obstentrics_Williams

Obstentrics_Williams. Shunts can also drain a dominant cyst in fetuses with macrocystic congenital cystic adenomatoid maormation. However, cysts rarely are large enough to pose a risk for hydrops or pulmonary hypoplasia. Shunt placement may improve survival to 90 percent in the absence of hydrops and to more than 75 percent with hydrops (Liwinska, 2017).


Obstentrics_Williams

Obstentrics_Williams. Vesicoamnionic shunts are used in selected fetuses with severe bladder-outlet obstruction in which diminished amnionic fluid portends a grim prognosis (Fig. 16-8). Distal obstruction of the urinary tract occurs more often in male fetuses. The most common etiology is posterior urethral valves, followed by urethral atresia and by prune bely syndrome, which is also called Eagle-Barrett syndrome. Sonographic indings include dilation of the bladder and proximal urethra, termed the "keyhole" sign, along with bladder wall thickening (Fig. 10-45, p. 210). Associated oligohydramnios before midpregnancy leads to pulmonary hypoplasia. Unfortunately, postnatal renal function may be poor even when amnionic fluid volume is normal.


Obstentrics_Williams

Obstentrics_Williams. Evaluation includes a careful search for concurrent anomalies, which may coexist in 40 percent of cases, and for aneuploidy, which has been reported in 5 to 8 percent of cases (Hayden, 1988; Hobbins, 1984; Mann, 2010). Fetal urine sampled at vesicocentesis may be used to perform genetic studies. As with other structural fetal abnormalities, chromosomal micro array analysis is recommended. Because visualization may be limited due to lack of amnionic fluid, counseling should include the increased likelihood that associated anomalies may be missed sonographically.


Obstentrics_Williams

Obstentrics_Williams. Potential candidates are fetuses without other severe anomalies or genetic syndromes and without sonographic features that confer poor prognosis, for example, renal cortical cysts. herapy is generally ofered only if the fetus is male, because in females, the underlying anomaly tends to be even more severe. Serial bladder drainage-vesicocentesis-performed under sonographic guidance at approximately 48-hour intervals is used to evaluate fetal urine electrolyte and protein content. Fetal urine is normally hypotonic due to tubular resorption of sodium and chloride, whereas isotonic urine in the setting of obstruction suggests renal tubular damage. Serial assessment permits classiication of the renal prognosis as good or poor and helps guide candidate selection (Table 16-4). TABLE 16-4. Fetal Urinary Analyte Values with Bladder Outlet Obstruction Good or poor prognosis is based on values from serial gestation, using the last specimen obtained. Data from Mann, 2010.


Obstentrics_Williams

Obstentrics_Williams. TABLE 16-4. Fetal Urinary Analyte Values with Bladder Outlet Obstruction Good or poor prognosis is based on values from serial gestation, using the last specimen obtained. Data from Mann, 2010. Shunt placement allows urine to drain from the bladder into the amnionic cavity. When successful, this often prevents pulmonary hypoplasia, however, renal function is not reliably preserved. Before shunting, amnioinfusion of warmed lactated Ringer solution is generally performed to aid catheter placement. Amnioinfusion also aids sonographic evaluation of fetal anatomy. A small trocar and cannula are then inserted into the fetal bladder under sonographic guidance. he shunt is placed as low as possible within the bladder to avoid dislodgement after bladder decompression. A double-pigtail catheter is used. The distal end lies within the fetal bladder, and the proximal end drains into the amnionic cavity.


Obstentrics_Williams

Obstentrics_Williams. Complications include displacement of the shunt out of the fetal bladder in up to 40 percent of cases, urinary ascites in about 20 percent, and development of gastroschisis in 10 percent (Freedman, 2000; Mann, 2010). Preterm delivery is common, and neonatal survival rates range from 50 to 90 percent (Biard, 2005; Walsh, 201l). A third of surviving children have required dialysis or renal transplantation, and almost half have respiratory problems (Biard, 2005). In one randomized trial, vesicoamnionic shunt placement was compared with conservative management in 31 cases (Morris, 2013). hose receiving shunts had higher survival rates. However, only two children had normal renal function at age 2 years.


Obstentrics_Williams

Obstentrics_Williams. With this procedure, high-frequency alternating current is used to coagulate and desiccate tissue. Radiofrequency ablation (RFA) has become a favored modality for treatment of twin-reversed arterial peusion (TAP) sequence, also known as acardiac twin (Chap. 45, p. 880). Without treatment, the mortality rate for the normal or pump twin in severe TRAP sequence exceeds 50 percent. he procedure is also used for selective termination with other monochorionic twin complications (Bebbington, 2012).


Obstentrics_Williams

Obstentrics_Williams. Under sonographic guidance, a 17-to 19-9auge RF A needle is directed into the base of the umbilical cord of the acardiac twin and inserted into its abdomen. After a 2-cm area of coagulation is achieved, color Doppler sonography is applied to veriy absent low into the acardius. In several centers, survival rates for the normal twin following RF A have signiicantly improved (Lee, 2007; Livingston, 2007). RF A was performed at approximately 20 weeks' gestation in 98 pregnancies with TRAP sequence reported by the North American Fetal Therapy Network (NAFTNet). The median gestational age at delivery was 37 weeks, and the neonatal survival rate was 80 percent. The major complication was prematurely ruptured membranes and pre term birth. Twelve percent were delivered at approximately 26 weeks (Lee, 2013).


Obstentrics_Williams

Obstentrics_Williams. RFA has generally been ofered for TRAP sequence when the volume of the acardiac twin is large. In the NFTNet series cited above, the median size of the acardius relative to the pump twin was 90 percent (Lee, 2013). Considering procedure-related risks, expectant management with close fetal surveillance is instead considered if the estimated weight of the acardius is below 50 percent of the pump twin Jelin, 2010). Finally, acardiac twins are more likely to complicate monoamnionic gestations. In one recent series, pump twin survival following RFA was 88 percent in monochorionic diamnionic pregnancies but only 67 percent in monoamnionic pregnancies (Sugibayashi, 2016).


Obstentrics_Williams

Obstentrics_Williams. Selected fetal cardiac lesions may worsen during gestation, further complicating or even obviating options for postnatal repair. Severe narrowing of a cardiac outflow tract may result in progressive myocardial damage in utero, and a goal of fetal intervention is to permit muscle growth and preserve ventricular function (Walsh, 2011). These innovative procedures include aortic valvuloplasy for critical aortic stenosis; atrial septostomy for hypoplastic left heart syndrome with intact interatrial septum; and pulmonary valvuloplasy for pulmonary atresia with intact interventricular septum.


Obstentrics_Williams

Obstentrics_Williams. Fetal aortic valvuloplasty is the most commonly performed cardiac procedure, accounting for 75 percent of cases reported by the International Fetal Cardiac Intervention Registry (MoonGrady, 2015). It is ofered for selected cases of critical aortic stenosis in which the left ventricle is either normal sized or dilated. he goal is to prevent progression to hypoplastic left heart and to permit postnatal biventricular repair (McElhinney, 2009). Under sonographic guidance, an I8-gauge cannula is inserted through uterus and fetal chest wall and into the left ventricle. Although the procedure is ideally performed percutaneouslythrough the maternal abdominal wall-laparotomy may be needed if the fetal position is unfavorable. The cannula tip is positioned in front of the stenotic aortic valve, and a 2.5-to 4.5-mm balloon catheter is then guided into the aortic annulus and inlated. Fetal bradycardia requiring treatment complicates a third of cases, and hemopericardium requiring drainage afects


Obstentrics_Williams

Obstentrics_Williams. a 2.5-to 4.5-mm balloon catheter is then guided into the aortic annulus and inlated. Fetal bradycardia requiring treatment complicates a third of cases, and hemopericardium requiring drainage afects approximately 20 percent (Moon-Grady, 2015).


Obstentrics_Williams

Obstentrics_Williams. From the first 100 cases at Boston Children's Hospital, 85 children survived, 38 of whom achieved biventricular circulation (Freud, 2014). Despite these successes, the mortality rate and risk for neurodevelopmental impairment in childhood appear to be similar to cases treated with postnatal repair (Laraja, 2017; Moon-Grady, 2015). Fetal atrial septostomy, also using a percutaneous balloon catheter, is ofered in select cases of hypoplastic left heart with an intact or highly restrictive interatrial septum. This condition has a postnatal mortality rate of nearly 80 percent (Glantz, 2007). In an efort to ensure patency, atrial septal stent placement has TABLE 16-5. Components of the Ex-Utero Intrapartum Treatment (EXIT) Procedure Comprehensive preoperative evaluation: specialized sonography, fetal echocardiography, magnetic resonance imaging, Uterine relaxation with deep general anesthesia and tocolysis


Obstentrics_Williams

Obstentrics_Williams. Comprehensive preoperative evaluation: specialized sonography, fetal echocardiography, magnetic resonance imaging, Uterine relaxation with deep general anesthesia and tocolysis Intraoperative sonography to confirm placental margin and fetal position and to visualize vessels at uterine entry Placement of stay-sutures followed by use of uterine stapling device to decrease uterine entry bleeding Maintenance of uterine volume during the procedure via continuous amnioinfusion of warmed physiological solution to help prevent placental separation Delivery of the fetal head, neck, and upper torso to permit access as needed Fetal injection of intramuscular vecuronium, fentanyl, and atropine Fetal peripheral intravenous access, pulse oximeter, and cardiac ultrasound Following procedure, umbilical lines placed prior to cord clamping Uterotonic agents administered as needed Data from Moldenhauer, 2013.


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Obstentrics_Williams. Data from Moldenhauer, 2013. also been performed. Of37 cases of atrial septostomy, survival to hospital discharge was almost 50 percent (Moon-Grady, 2015). Fetal pulmonary valvuloplasty has been ofered in cases of pulmonary atresia with intact interventricular septum to prevent development of hypoplastic right heart syndrome. Although success is achieved in approximately two thirds of cases, it is not yet clear whether outcomes are improved compared with standard postnatal repair (Arzt, 2011; McElhinney, 2010).


Obstentrics_Williams

Obstentrics_Williams. This procedure allows the fetus to remain perfused by the placenta after being partially delivered, so that lifesaving treatment can be performed before completing the delivery. The technique was irst developed to obtain an airway with fetal tumors involving the oropharynx and neck (Catalano, 1992; Kelly, 1990; Langer, 1992). An ex-utero intrapartum treatment (EXIT) procedure is performed by a multidisciplinary team, which may include an obstetrician, maternal-fetal medicine specialist, pediatric surgeon(s), pediatric otolaryngologist, pediatric cardiologist, anesthesiologists for the mother and fetus, and neonatologists, as well as specially trained nursing personnel. Components of the procedure are shown in Table 16-5.


Obstentrics_Williams

Obstentrics_Williams. Selected indications are listed in Table 16-2. EXIT is the preferred procedure for intrapartum management oflarge venolymphatic malformations of the neck such as the one shown in Figure 16-9. At the Children's Hospital of Philadelphia, criteria for EXIT with a cervical venolymphatic malformation include compression, deviation, or obstruction of the airway by the mass, and also involvement of the floor of the mouth (Laje, 2015). In a review of 112 pregnancies with fetal cervical venolymphatic malformations, only about 10 percent met these criteria. Other indications for EXIT include severe micrognathia and congenital high airway obstruction sequence (CHAOS), which are discussed in Chapter 10 (Figs. 10-20 and 10-26, p. 197). Criteria for an EXIT procedure for micrognathia include a fetal jaw measurement below the 5th percentile along with indirect evidence of obstruction, such as hydramnios, an absent stomach bubble, or glossoptosis (Morris, 2009b). Case selection for EIT procedures is


Obstentrics_Williams

Obstentrics_Williams. measurement below the 5th percentile along with indirect evidence of obstruction, such as hydramnios, an absent stomach bubble, or glossoptosis (Morris, 2009b). Case selection for EIT procedures is generally based on fetal 1R imaging findings (Chap. 10, p. 219).


Obstentrics_Williams

Obstentrics_Williams. FIGURE 16-9 Ex-utero intrapartum treatment (EXID procedure for a venolymphatic malformation. A. Upon delivery of the head, placental circulation was maintained and an airway was established over the course of 20 minutes by a team of pediatric subspecialists that included a surgeon, anesthesiologist, and otolaryngologist. B. Following a controlled intubation, the fetus was ready for delivery and transfer to the neonatal intensive care unit team. (Used with permission from Drs. Stacey Thomas and Patricia Santiago-Munoz.)


Obstentrics_Williams

Obstentrics_Williams. In some cases, an EIT procedure has been used as a bridge to other procedures. For example, resection of large thoracic masses may be accomplished by fetal thoracotomy performed with intact placental circulation. In a series of 16 fetuses with CCM volume ratiosi> 1.6 or hydrops, all of whom had mediastinal compression, Cass and colleagues (2013) reported that nine infants undergoing EIT-to-resection survived. In contrast, there were no survivors with urgent postnatal surgery alone. Similarly, Moldenhauer (2013) reported that 20 of 22 newborns treated with EXIT -to-resection for lung masses survived. he EIT procedure has also been used as a bridge to extracorporeal membrane oxygenation-EIT-to-ECMO-in pregnancies with severe congenital diaphragmatic hernia. However, it has not been found to clearly confer survival benefit in such cases (Morris, 2009a; Shieh, 2017; Stofan, 2012).


Obstentrics_Williams

Obstentrics_Williams. Counseling prior to an EIT procedure includes procedurerelated risks such as hemorrhage from placental abruption or uterine atony, need for cesarean delivery in future pregnancies, higher risk for subsequent uterine rupture or dehiscence, possible need for hysterectomy, and fetal death or permanent neonatal disability. Compared with cesarean delivery, the EIT procedure is associated with greater blood loss, a higher incidence of wound complications, and a longer operating time-approximately 40 minutes longer depending on the procedure (Noah, 2002). Abuhamad Z, Fisher DA, Warsof SL, et al: Antenatal diagnosis and treatment of fetal goitrous hypothyroidism: case report and review of the literature. Ultrasound Obstet Gynecol 6:368, 1995 Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in utero intervention. Semin Fetal Neonatal Med 15(1):9,o2010


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Obstentrics_Williams. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in utero intervention. Semin Fetal Neonatal Med 15(1):9,o2010 Adzick NS, Harrison MR, Glick PL, et al: Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. ] Pediatr Surg 20:483, 1985 Adzick NS, Thon A, Spong CY, et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Englo] Med 364(11):993, 2011 Agrawal P, Ogilvy-Stuart A, Lees C: Intrauterine diagnosis and management of congenital goitrous hypothyroidism. Ultrasound Obstet GynecoIo19:501, 2002 Akinkuotu Ae, Coleman A, Shue E, et al: Predictors of poor prognosis in prenatally diagnosed sacrococcygeal teratoma: a multiinstitutional review. ] Pediatr Surg 50(5):771,o2015 Al-Maary ], Eastwood MP, Russo FM, et al: Fetal tracheal occlusion for severe pulmonary hypoplasia in isolated congenital diaphragmatic hernia. A systematic review and meta-analysis of survival. Ann Surg 264(6):929,o2016


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Obstentrics_Williams. Araujo Junior A, Eggink A], van den Dobbelsteeno], et al: Procedure-related complications of open vs endoscopic fetal surgery for treatment of spina bifida in an era of intrauterine myelomeningocele repair: systematic review and meta-analysis. Ultrasound Obstet GynecoIo48(2):151, 2016


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Obstentrics_Williams. Jani ]C, Nicolaides KH, Gratacos E, et al: Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol 34:304, 2009 ]elin E, Hirose S, Rand L, et al: Perinatal outcome of conservative management versus fetal intervention for twin reversed arterial perfusion sequence with a small acardiac twin. Fetal Diagn her 27:138,o2010


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