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Michael R Harrison - One of the best experts on this subject based on the ideXlab platform.
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REMOVED: Maternal-Fetal Surgery: History and General Considerations
Clinics in perinatology, 2012Co-Authors: Eveline H. Shue, Michael R Harrison, Shinjiro HiroseAbstract:This article reviews Fetal Intervention for congenital anomalies, which has evolved from a mere concept to a medical specialty over the past 3 decades. Advances in surgical techniques have paralleled developments in Fetal imaging, Fetal diagnosis, and the advent of maternal tocolysis to prevent preterm labor. Fetal Intervention has become an important option for fetuses who would otherwise not survive gestation or who would endure significant morbidity and mortality after birth. However, there were many trials and tribulations as Fetal surgery developed into a medical specialty.
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Long-term outcomes after Fetal therapy for congenital high airway obstructive syndrome
Journal of pediatric surgery, 2012Co-Authors: Payam Saadai, Jody A. Farrell, Larry Rand, Ruth B. Goldstein, Shinjiro Hirose, Tippi C. Mackenzie, Eric B. Jelin, Amar Nijagal, Samuel C. Schecter, Michael R HarrisonAbstract:Abstract Background/Purpose Congenital high airway obstructive syndrome (CHAOS) is a rare and devastating condition that is uniformly fatal without Fetal Intervention. We sought to describe Fetal treatment and long-term outcomes of CHAOS at a single referral center. Methods The medical records of patients with Fetal CHAOS evaluated at our center between 1993 and 2011 were reviewed. Maternal history, radiographic findings, antenatal management, and postnatal outcomes were compared. Results Twelve fetuses with CHAOS were identified. Eleven had concomitant hydrops at diagnosis. Six were electively terminated, and 2 had intra- or peripartum demise. Four patients underwent Fetal Intervention. Two underwent delivery via ex utero intrapartum treatment (EXIT) procedure with tracheostomy placement only, and 2 underwent Fetal bronchoscopy with attempted wire tracheoplasty followed by EXIT with tracheostomy at delivery. All 4 patients who underwent EXIT were alive at last follow-up. One patient was ventilator and tracheostomy free and feeding by mouth. Conclusion Long-term and tracheostomy-free survival is possible with appropriate Fetal Intervention even in the presence of hydrops. Fetal Intervention earlier in pregnancy may improve long-term outcomes, but patient selection for Intervention remains challenging. Magnetic resonance imaging may help select those patients for whom Fetal Intervention before EXIT delivery may be beneficial.
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The making of Fetal surgery.
Prenatal diagnosis, 2010Co-Authors: Jan Deprest, Mark P Johnson, Alan W. Flake, N. Scott Adzick, E Gratacos, Yves Ville, Kurt Hecher, Kypros H. Nicolaides, Francois I. Luks, Michael R HarrisonAbstract:Fetal diagnosis prompts the question for Fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical Intervention. This paper reviews historically important steps in the development of Fetal surgery. The first invasive Fetal Intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate Fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for Fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many Fetal Interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making Fetal surgery a clinical reality in a number of Fetal therapy programmes. The safety of Fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as Fetal Intervention for CDH, is currently being investigated in randomised trials. Copyright © 2010 John Wiley & Sons, Ltd.
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Fetal Intervention for mass lesions and hydrops improves outcome: a 15-year experience
Journal of pediatric surgery, 2007Co-Authors: Erich J. Grethel, Michael R Harrison, Amy J. Wagner, Matthew S. Clifton, Raul A. Cortes, Diana L. Farmer, Kerilyn K. Nobuhara, Hanmin LeeAbstract:The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with Fetal mass lesions can be halted and even reversed with Fetal Intervention and treatment of the underlying defect. We examined our patients with Fetal mass lesions to evaluate survival after Intervention. Institutional approval was obtained by the Committee on Human Research. A retrospective review was performed of 294 fetuses evaluated over 15 years with large mass lesions. All patients were evaluated for evidence of Fetal hydrops using ultrasound criteria. Patients were divided according to type of Intervention. Primary outcome measure was 30-day survival after birth. (1) Patients without Fetal hydrops did not undergo Fetal Intervention and survived to 30 days after birth (167/172, 97%). (2) Patients with Fetal mass lesions that developed hydrops fared poorly with no Intervention (1/33 survival, 3%), whereas fetuses undergoing prenatal Intervention fared much better (15/30 open, 50%; 3/10 percutaneous, 30%). (3) Four patients with hydropic congenital cystic adenomatoid malformation (n = 3) or pulmonary sequestration (n = 1) received steroids in preparation for surgery but underwent no Intervention, and the patients survived the neonatal period. Fetuses with prenatal diagnoses of masses not associated with hydrops have excellent prognosis with survival higher than 95%. Nonimmune hydrops associated with prenatal diagnosis of a Fetal mass is a devastating complication with less than 5% survival. Open resection of a mass causing hydrops resulted in 50% survival, with reversal of hydrops in a group with near-uniform fatality. Further investigation is warranted regarding the use of minimally invasive prenatal therapies including steroid administration for hydropic fetuses.
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Fetal Intervention for mass lesions and hydrops improves outcome a 15 year experience
Journal of Pediatric Surgery, 2007Co-Authors: Erich J. Grethel, Michael R Harrison, Amy J. Wagner, Matthew S. Clifton, Raul A. Cortes, Diana L. Farmer, Kerilyn K. Nobuhara, Hanmin LeeAbstract:Abstract Purpose The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with Fetal mass lesions can be halted and even reversed with Fetal Intervention and treatment of the underlying defect. We examined our patients with Fetal mass lesions to evaluate survival after Intervention. Methods Institutional approval was obtained by the Committee on Human Research. A retrospective review was performed of 294 fetuses evaluated over 15 years with large mass lesions. All patients were evaluated for evidence of Fetal hydrops using ultrasound criteria. Patients were divided according to type of Intervention. Primary outcome measure was 30-day survival after birth. Results (1)Patients without Fetal hydrops did not undergo Fetal Intervention and survived to 30 days after birth (167/172, 97%). (2)Patients with Fetal mass lesions that developed hydrops fared poorly with no Intervention (1/33 survival, 3%), whereas fetuses undergoing prenatal Intervention fared much better (15/30 open, 50%; 3/10 percutaneous, 30%). (3)Four patients with hydropic congenital cystic adenomatoid malformation (n = 3) or pulmonary sequestration (n = 1) received steroids in preparation for surgery but underwent no Intervention, and the patients survived the neonatal period. Conclusion Fetuses with prenatal diagnoses of masses not associated with hydrops have excellent prognosis with survival higher than 95%. Nonimmune hydrops associated with prenatal diagnosis of a Fetal mass is a devastating complication with less than 5% survival. Open resection of a mass causing hydrops resulted in 50% survival, with reversal of hydrops in a group with near-uniform fatality. Further investigation is warranted regarding the use of minimally invasive prenatal therapies including steroid administration for hydropic fetuses.
Wayne Tworetzky - One of the best experts on this subject based on the ideXlab platform.
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reversible ductus arteriosus constriction due to maternal indomethacin after Fetal Intervention for hypoplastic left heart syndrome with intact restrictive atrial septum
Fetal Diagnosis and Therapy, 2010Co-Authors: Melanie Vogel, Doff B. Mcelhinney, Audrey C. Marshall, Carol B. Benson, Virginia Silva, Louise Wilkinshaug, Wayne TworetzkyAbstract:Objective: Fetal cardiac Intervention (FCI) has been performed at our center in selected fetuses with complex congenital heart disease since 2000. Most Interventions are performed in
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Reversible Ductus Arteriosus Constriction due to Maternal Indomethacin after Fetal Intervention for Hypoplastic Left Heart Syndrome with Intact/Restrictive Atrial Septum
Fetal diagnosis and therapy, 2009Co-Authors: Melanie Vogel, Doff B. Mcelhinney, Audrey C. Marshall, Louise Wilkins-haug, Carol B. Benson, Virginia Silva, Wayne TworetzkyAbstract:Fetal cardiac Intervention (FCI) has been performed at our center in selected fetuses with complex congenital heart disease since 2000. Most Interventions are performed in fetuses with a ductus arteriosus (DA)-dependent circulation. Indomethacin promotes closure of the DA in newborns and in Fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease. We reviewed our experience with FCI with a focus on the frequency, features, and clinical course of ductal constriction. Fetuses undergoing FCI receive comprehensive pre- and postoperative cardiac and cerebral ultrasound evaluation, approximately 24 hours before and after the procedure, including imaging of DA flow and Doppler assessment of the umbilical artery and vein, ductus venosus, and, since 2004, the middle cerebral artery. Among 113 fetuses that underwent FCI, 24 of which were older than 28 0/7 weeks gestation, 2 were found to have DA constriction due to indomethacin therapy within 24 hours of Intervention. Both of these were 30-week fetuses with hypoplastic left heart syndrome and restrictive or intact atrial septum. The DA was stenotic by spectral and color Doppler, and middle cerebral and umbilical artery pulsatility indexes were depressed. After discontinuation of indomethacin, the Doppler indices improved or normalized. Close echocardiographic monitoring of Fetal Doppler flow velocities is very important after Fetal Intervention and indomethacin treatment, as the consequences of DA constriction in a fetus with hypoplastic left heart syndrome are potentially lethal. Sonographic evaluation should include measurement of cerebral and umbilical arterial flow velocities as well as color and spectral Doppler interrogation of the DA. Copyright 2009 S. Karger AG, Basel.
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hypoplastic left heart syndrome with intact or highly restrictive atrial septum surgical experience from a single center
The Annals of Thoracic Surgery, 2007Co-Authors: Vladimiro L Vida, Emile A Bacha, Alesandro Larrazabal, K Gauvreau, Ravi Thiagaragan, Francis Fynnthompson, Frank A Pigula, John E Mayer, Pedro J Del Nido, Wayne TworetzkyAbstract:Background The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors. Methods We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006. Chart review enabled analysis of patient and procedural variables. Results All 32 patients underwent left atrial decompression in utero or postnatally before surgery. Fourteen patients (44%) underwent Fetal Intervention, either atrial septoplasty (n = 9) or aortic valvuloplasty (n = 5). Twenty-nine of the 32 patients had postnatal left atrial hypertension and underwent transcatheter atrial septoplasty as neonates before surgery; 3 did not require postnatal atrial septoplasty after successful Fetal atrial septoplasty. After stage I, hospital survival was 69% (22 of 32). Need for shunt revision (p = 0.02) and for extracorporeal membrane oxygenation use (p Conclusions Surgical outcome for patients with HLHS and I/HRAS continues to improve. Prenatal decompression of the left atrium may be associated with greater hospital survival. Proposed effects of Fetal Intervention on lung pathology and longer-term survival are subjects for future study in this unique group of patients.
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Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome patient selection for Fetal Intervention
Circulation, 2006Co-Authors: Kaarin Makikallio, Doff B. Mcelhinney, Jami C. Levine, Gerald R. Marx, Steven D. Colan, Audrey C. Marshall, James E. Lock, Edward Marcus, Wayne TworetzkyAbstract:Background— Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for Fetal Intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation Fetal AS to HLHS. Methods and Results— Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at ≤30 weeks’ gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the fora...
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Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for Fetal Intervention
Circulation, 2006Co-Authors: Kaarin Mäkikallio, Doff B. Mcelhinney, Jami C. Levine, Gerald R. Marx, Steven D. Colan, Audrey C. Marshall, James E. Lock, Edward Marcus, Wayne TworetzkyAbstract:Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for Fetal Intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation Fetal AS to HLHS. Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS. In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for Fetal Intervention to prevent the progression of AS to HLHS.
Rodrigo Ruano - One of the best experts on this subject based on the ideXlab platform.
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Ethical considerations of maternal-Fetal Intervention in a twin pregnancy discordant for anomalies.
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine the Federation of Asia and , 2019Co-Authors: Kirsten A. Riggan, Rodrigo Ruano, Christopher A. Collura, Siobhan T. Pittock, Kevin J. Whitford, Megan AllyseAbstract:Background: Recent evidence suggests prenatal fetoscopic tracheal occlusion (FETO) may improve the survival and long-term morbidity of neonates with congenital diaphragmatic hernia, yet little guid...
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Fetal surgery for lower urinary tract obstruction: the importance of staging prior to Intervention.
Minerva pediatrica, 2017Co-Authors: Elizabeth Ann L. Enninga, Rodrigo RuanoAbstract:Fetal lower urinary tract obstruction (LUTO) is a heterogeneous pathology associated with a high morbidity and mortality due to pulmonary hypoplasia. Previously, when a fetus was diagnosed on ultrasound with LUTO, expectant care or termination was the only option; this has changed because of Fetal surgical Intervention. Vesicoamniotic shunts and cystoscopy are the current methods utilized to treat LUTO; however, it remains difficult to determine whether Fetal prognosis favors Intervention and long-term outcome follow-up has been limited. This review covers the history and current challenges of Fetal Intervention for obstructive uropathies. We also present the Ruano's LUTO staging system which integrates information from Fetal urine analysis with ultrasound findings to assess whether a specific Fetal Intervention is recommended. Utilizing a standard staging system that is readily adopted by providers carrying out Fetal surgery is crucial for determining the true impact on outcomes Intervention has on fetuses diagnosed with LUTO.
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report on the society for Fetal urology panel discussion on the selection criteria and Intervention for Fetal bladder outlet obstruction
Journal of Pediatric Urology, 2017Co-Authors: Marie Klaire Farrugia, Rodrigo Ruano, Michael C. Braun, Craig A Peters, C D HerndonAbstract:Summary Introduction The Society for Fetal Urology panel section at the 2016 Fall Congress featured a multidisciplinary discussion on appropriate patient selection, the conservative versus surgical management, and postnatal renal outcome of fetuses with lower urinary tract obstruction (LUTO). Selection criteria for Intervention Rodrigo Ruano shared his experience of prenatal Intervention, presenting the outcome of 111 fetuses with severe LUTO treated with vesicoamniotic shunting (VAS) ( n = 16), cystoscopy ( n = 34) or no Intervention ( n = 61) in a non-randomized series. Multivariate analysis at the 6-month follow-up suggested a significantly higher probability of survival with Fetal Intervention versus no Intervention. A clear trend for normal renal function was present in the Fetal cystoscopy group, but not in the VAS group. In cases in which there was a postnatal diagnosis of posterior urethral valves ( n = 57), Fetal cystoscopy was effective in improving both the 6-month survival rate and renal function, while VAS was associated with an improvement in the 6-month survival rate. In an attempt to better define which fetuses would benefit from Intervention, Michael Braun explained the proposed LUTO classification system that incorporates: (1) Fetal urinary biomarkers of renal injury; (2) amniotic fluid levels as a surrogate for the severity of obstruction; and (3) imaging studies to identify signs of renal dysplastic or cystic changes. Intervention was not recommended in patients at low risk of either renal disease or pulmonary hypoplasia (Stage 1). Vesicoamniotic shunting was performed in patients at high risk of either progressive renal injury or pulmonary hypoplasia without evidence of severe pre-existing renal damage (Stage 2). For those patients, who at the time of evaluation had evidence of severe renal disease (Stage 3), Fetal Intervention was individualized and often based on bladder capacity and bladder refilling after vesicocentesis. He went on to present the nephrologic outcome of fetuses managed over the last 3 years utilizing the selection criteria. Craig Peters supported the concept of selective criteria and discussed the cautious viewpoint, namely: (1) the procedure may be unnecessary, as it is possible for patients to do well, in spite of severe prenatal obstruction; and (2) the risk of giving partial treatment by allowing the baby to survive to delivery with the daunting postnatal journey of renal and pulmonary insufficiency. Conclusion Standardized patient selection utilizing a staging system is undoubtedly the way forward and will enable comparable long-term renal and bladder functional outcome studies.
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Lower urinary tract obstruction: Fetal Intervention based on prenatal staging
Pediatric nephrology (Berlin Germany), 2017Co-Authors: Rodrigo Ruano, Timothy N. Dunn, Michael C. Braun, Joseph Angelo, Adnan SafdarAbstract:The authors present an overview of lower urinary tract obstruction (LUTO) in the fetus with a particular focus on the insult to the developing renal system. Diagnostic criteria along with the challenges in estimating long-term prognosis are reviewed. A proposed prenatal LUTO disease severity classification to guide management decisions with Fetal Intervention to maintain or salvage in utero and neonatal pulmonary and renal function is also discussed. Stage I LUTO (mild form) is characterized by normal amniotic fluid index after 18 weeks, normal kidney echogenicity, no renal cortical cysts, no evidence of renal dysplasia, and favorable urinary biochemistries when sampled between 18 and 30 weeks; prenatal surveillance is recommended. Stage II LUTO is characterized by oligohydramnios/anhydramnios, hyperechogenic kidneys but absent renal cortical cysts or apparent signs of renal dysplasia and favorable Fetal urinary biochemistry; Fetal vesicoamniotic shunting (VAS) or Fetal cystoscopy is indicated to prevent pulmonary hypoplasia and renal failure. Stage III LUTO is oligohydramnios/anhydramnios, hyperechogenic kidneys with cortical cysts and renal dysplasia and unfavorable Fetal urinary biochemistry after serial evaluation; Fetal vesicoamniotic shunt may prevent severe pulmonary hypoplasia but not renal failure. Stage IV is characterized by intrauterine Fetal renal failure, defined by anhydramnios and ultrasound (US) findings suggestive of severe renal dysplasia, and is associated with death in 24 h of life or end-stage renal disease (ESRD) within the first week of life; Fetal vesicoamniotic shunt and Fetal cystoscopy are not indicated.
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Fetal lower urinary tract obstruction: proposal for standardized multidisciplinary prenatal management based on disease severity.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology, 2016Co-Authors: Rodrigo Ruano, N Sananes, Adnan Safdar, Clark Wilson, Chester J. Koh, Patricio C. Gargollo, Alireza A. Shamshirsaz, Jimmy Espinoza, Amirhossein MoaddabAbstract:To present a single center experience of a standardized prenatal multidisciplinary management protocol for Fetal lower urinary tract obstruction (LUTO) and to propose a classification of Fetal LUTO based on disease severity. This was a retrospective cohort study of 25 consecutive Fetal patients with prenatal diagnosis of primary LUTO. Fetal Intervention was offered after evaluation by a multidisciplinary team. Analyses were conducted using Bayesian methodology to determine predictors of survival at 6 months postpartum. Odds ratios (ORs) with 95% credibility intervals are reported. Fifteen (60.0%) of the 25 patients referred for assessment survived to postnatal evaluation. Fetal vesicoamniotic shunt was placed in 14 (56.0%) patients with 12 survivors. Multivariable analysis suggested that Fetal Intervention (OR, 6.97 (0.88-70.16), Pr(OR > 1) = 96.7%), anhydramnios (OR, 0.12 (0.04-0.35), Pr(OR < 1) = 99.9%), favorable Fetal urine analysis (OR, 3.98 (0.63-25.15), Pr(OR > 1) = 92.7%) and absence of renal cortical cysts (OR, 3.9 (0.66-24.2), Pr(OR > 1) = 93.3%) were predictors of survival. Fetal Intervention and Fetal renal function were independently associated with postnatal survival of fetuses with LUTO. A classification based on the severity of disease is proposed. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.
N. Scott Adzick - One of the best experts on this subject based on the ideXlab platform.
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The making of Fetal surgery.
Prenatal diagnosis, 2010Co-Authors: Jan Deprest, Mark P Johnson, Alan W. Flake, N. Scott Adzick, E Gratacos, Yves Ville, Kurt Hecher, Kypros H. Nicolaides, Francois I. Luks, Michael R HarrisonAbstract:Fetal diagnosis prompts the question for Fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical Intervention. This paper reviews historically important steps in the development of Fetal surgery. The first invasive Fetal Intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate Fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for Fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many Fetal Interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making Fetal surgery a clinical reality in a number of Fetal therapy programmes. The safety of Fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as Fetal Intervention for CDH, is currently being investigated in randomised trials. Copyright © 2010 John Wiley & Sons, Ltd.
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Antenatal diagnosis and management of congenital cystic adenomatoid malformation
Seminars in fetal & neonatal medicine, 2007Co-Authors: Stephanie Mann, R. Douglas Wilson, N. Scott Adzick, Michael Bebbington, Mark P JohnsonAbstract:One of the most enigmatic pulmonary lesions encountered in the prenatal period is the congenital cystic adenomatoid malformation (CCAM). This review presence current thinking on pathogenesis, prenatal assessment, Fetal Intervention, and management for this pulmonary malformation. Careful delivery planning by utilizing a multidisciplinary approach will optimize neonatal outcomes.
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Sacrococcygeal teratoma: prenatal assessment, Fetal Intervention, and outcome.
Journal of pediatric surgery, 2004Co-Authors: Holly L. Hedrick, Mark P Johnson, Alan W. Flake, Timothy M. Crombleholme, Lori J. Howell, R. Douglas Wilson, N. Scott AdzickAbstract:To understand the natural history and define indications for Fetal Intervention in sacrococcygeal teratoma (SCT), the authors reviewed all cases of Fetal SCT presenting for evaluation. Prenatal diagnostic studies including ultrasound scan, magnetic resonance imaging (MRI), echocardiography and pre- and postnatal outcomes were reviewed in 30 cases of SCT that presented between September 1995 and January 2003. The mean gestational age (GA) at presentation was 23.9 weeks (range, 19 to 38.5) with 3 sets of twins (10%). Overall outcomes included 4 terminations, 5 Fetal demises, 7 neonatal deaths, and 14 survivors. Significant obstetric complications occurred in 81% of the 26 continuing pregnancies: polyhydramnios (n = 7), oligohydramnios (n = 4), preterm labor (n = 13), preeclampsia (n = 4), gestational diabetes (n = 1), HELLP syndrome (n = 1), and hyperemesis (n = 1). Fetal Intervention included cyst aspiration (n = 6), amnioreduction (n = 3), amnioinfusion (n = 1), and open Fetal surgical resection (n = 4). Indications for cyst aspiration and amnioreduction were maternal discomfort, preterm labor, and prevention of tumor rupture at delivery. Although 15 SCTs were solid causing risk for cardiac failure, only 4 fetuses met criteria for Fetal debulking based on ultrasonographic and echocardiographic evidence of impending high output failure and favorable anatomy at 21, 23.6, 25, and 26 weeks' gestation. Intraoperative events included maternal blood transfusion (n = 1), Fetal blood transfusion (n = 2), chorioamniotic membrane separation (n = 2), and Fetal arrest requiring successful cardiopulmonary resuscitation (CPR) (n = 1). In the Fetal resection group, 3 of 4 survived with mean GA at delivery of 29 weeks (range, 27.6 to 31.7 weeks), mean birth weight of 1.3 kg, hospital stay ranging from 16 to 34 weeks, and follow-up ranging from 20 months to 6 years. Postnatal complications in the Fetal surgery group included neonatal death (n = 1, secondary to premature closure of ductus arteriosus with cardiac failure), embolic event (n = 1, resulting in unilateral renal agenesis, jejunal atresia), chronic lung disease (n = 1), and tumor recurrence (n = 1). For Fetal SCT, the rapidity at which cardiac compromise can develop and the high incidence of obstetric complications warrant close prenatal surveillance. Amnioreduction, cyst aspiration, and surgical debulking are potentially life-saving Interventions.
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A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for Fetal Intervention.
Pediatrics, 2002Co-Authors: Natalie E Rintoul, Leslie N Sutton, Anne M Hubbard, Brian M Cohen, Jeanne Melchionni, Patrick S Pasquariello, N. Scott AdzickAbstract:Previous reports have suggested that 80% to 85% of patients who have a myelomeningocele (MMC) and undergo surgical repair after birth develop hydrocephalus and require the placement of a ventricular shunt. However, the rate of shunting as a function of spinal level is not well established. We sought to determine the distribution of postnatally repaired MMC lesions as characterized by both functional and radiologic assessment, as well as the incidence of shunting when patients were categorized according to these 2 methods. A retrospective chart review of 297 patients who were born with open MMCs and followed in the spina bifida clinic at the Children's Hospital of Philadelphia was performed. The presence or absence of a shunt was determined for each patient. Functional spinal level was determined by the best-recorded neurologic examination and vertebral level by spine radiographs. The overall rate of ventricular shunting was 81%. The level of the lesion significantly affected the incidence of shunting, with more cephalad lesions correlating with higher rates. This was true both for functional and radiologic categorizations. A significantly higher shunt rate was found among patients with sacral lesions when categorized by radiologic rather than functional criteria. In 86% of patients, the functional level was found to be equal to or higher (worse) than the radiologic level. This study describes the natural history of ventricular shunting in MMC patients with relation to both radiologic and functional criteria. Fetal MMC closure is being performed in some centers in an attempt to decrease the incidence of shunting and to improve leg function in selected patients. The present data may serve as a comparison group and aid in the design and analysis of a prospective trial to assess the efficacy of this new procedure.
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The current status and future potential of Fetal Intervention: image is everything.
Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society, 1999Co-Authors: Alan W. Flake, Timothy M. Crombleholme, N. Scott AdzickAbstract:Fetal imaging is integral to the past, present, and future of Fetal Intervention. In the early history of Fetal Intervention, the role of prenatal ultrasound was primarily to identify a Fetal lesion, provide a correct anatomic diagnosis, and exclude other anatomic defects. We now depend on ultrasound, and other imaging studies, not only to provide anatomic information, but to provide prognostic, physiologic and functional information prior to, during, and after surgery for optimal selection and management of Fetal surgery patients. In the future improvements in imaging and non-invasive technology will drive further expansion of indications and therapeutic options for Fetal Intervention.
Alan W. Flake - One of the best experts on this subject based on the ideXlab platform.
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The making of Fetal surgery.
Prenatal diagnosis, 2010Co-Authors: Jan Deprest, Mark P Johnson, Alan W. Flake, N. Scott Adzick, E Gratacos, Yves Ville, Kurt Hecher, Kypros H. Nicolaides, Francois I. Luks, Michael R HarrisonAbstract:Fetal diagnosis prompts the question for Fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical Intervention. This paper reviews historically important steps in the development of Fetal surgery. The first invasive Fetal Intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate Fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for Fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many Fetal Interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making Fetal surgery a clinical reality in a number of Fetal therapy programmes. The safety of Fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as Fetal Intervention for CDH, is currently being investigated in randomised trials. Copyright © 2010 John Wiley & Sons, Ltd.
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sacrococcygeal teratoma prenatal assessment Fetal Intervention and outcome
Journal of Pediatric Surgery, 2004Co-Authors: Holly L. Hedrick, Mark P Johnson, Alan W. Flake, Timothy M. Crombleholme, Lori J. Howell, Douglas R Wilson, Scott N AdzickAbstract:Abstract Purpose To understand the natural history and define indications for Fetal Intervention in sacrococcygeal teratoma (SCT), the authors reviewed all cases of Fetal SCT presenting for evaluation. Methods Prenatal diagnostic studies including ultrasound scan, magnetic resonance imaging (MRI), echocardiography and pre- and postnatal outcomes were reviewed in 30 cases of SCT that presented between September 1995 and January 2003. Results The mean gestational age (GA) at presentation was 23.9 weeks (range, 19 to 38.5) with 3 sets of twins (10%). Overall outcomes included 4 terminations, 5 Fetal demises, 7 neonatal deaths, and 14 survivors. Significant obstetric complications occurred in 81% of the 26 continuing pregnancies: polyhydramnios (n = 7), oligohydramnios (n = 4), preterm labor (n = 13), preeclampsia (n = 4), gestational diabetes (n = 1), HELLP syndrome (n = 1), and hyperemesis (n = 1). Fetal Intervention included cyst aspiration (n = 6), amnioreduction (n = 3), amnioinfusion (n = 1), and open Fetal surgical resection (n = 4). Indications for cyst aspiration and amnioreduction were maternal discomfort, preterm labor, and prevention of tumor rupture at delivery. Although 15 SCTs were solid causing risk for cardiac failure, only 4 fetuses met criteria for Fetal debulking based on ultrasonographic and echocardiographic evidence of impending high output failure and favorable anatomy at 21, 23.6, 25, and 26 weeks’ gestation. Intraoperative events included maternal blood transfusion (n = 1), Fetal blood transfusion (n = 2), chorioamniotic membrane separation (n = 2), and Fetal arrest requiring successful cardiopulmonary resuscitation (CPR) (n = 1). In the Fetal resection group, 3 of 4 survived with mean GA at delivery of 29 weeks (range, 27.6 to 31.7 weeks), mean birth weight of 1.3 kg, hospital stay ranging from 16 to 34 weeks, and follow-up ranging from 20 months to 6 years. Postnatal complications in the Fetal surgery group included neonatal death (n = 1, secondary to premature closure of ductus arteriosus with cardiac failure), embolic event (n = 1, resulting in unilateral renal agenesis, jejunal atresia), chronic lung disease (n = 1), and tumor recurrence (n = 1). Conclusions For Fetal SCT, the rapidity at which cardiac compromise can develop and the high incidence of obstetric complications warrant close prenatal surveillance. Amnioreduction, cyst aspiration, and surgical debulking are potentially life-saving Interventions.
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Sacrococcygeal teratoma: prenatal assessment, Fetal Intervention, and outcome.
Journal of pediatric surgery, 2004Co-Authors: Holly L. Hedrick, Mark P Johnson, Alan W. Flake, Timothy M. Crombleholme, Lori J. Howell, R. Douglas Wilson, N. Scott AdzickAbstract:To understand the natural history and define indications for Fetal Intervention in sacrococcygeal teratoma (SCT), the authors reviewed all cases of Fetal SCT presenting for evaluation. Prenatal diagnostic studies including ultrasound scan, magnetic resonance imaging (MRI), echocardiography and pre- and postnatal outcomes were reviewed in 30 cases of SCT that presented between September 1995 and January 2003. The mean gestational age (GA) at presentation was 23.9 weeks (range, 19 to 38.5) with 3 sets of twins (10%). Overall outcomes included 4 terminations, 5 Fetal demises, 7 neonatal deaths, and 14 survivors. Significant obstetric complications occurred in 81% of the 26 continuing pregnancies: polyhydramnios (n = 7), oligohydramnios (n = 4), preterm labor (n = 13), preeclampsia (n = 4), gestational diabetes (n = 1), HELLP syndrome (n = 1), and hyperemesis (n = 1). Fetal Intervention included cyst aspiration (n = 6), amnioreduction (n = 3), amnioinfusion (n = 1), and open Fetal surgical resection (n = 4). Indications for cyst aspiration and amnioreduction were maternal discomfort, preterm labor, and prevention of tumor rupture at delivery. Although 15 SCTs were solid causing risk for cardiac failure, only 4 fetuses met criteria for Fetal debulking based on ultrasonographic and echocardiographic evidence of impending high output failure and favorable anatomy at 21, 23.6, 25, and 26 weeks' gestation. Intraoperative events included maternal blood transfusion (n = 1), Fetal blood transfusion (n = 2), chorioamniotic membrane separation (n = 2), and Fetal arrest requiring successful cardiopulmonary resuscitation (CPR) (n = 1). In the Fetal resection group, 3 of 4 survived with mean GA at delivery of 29 weeks (range, 27.6 to 31.7 weeks), mean birth weight of 1.3 kg, hospital stay ranging from 16 to 34 weeks, and follow-up ranging from 20 months to 6 years. Postnatal complications in the Fetal surgery group included neonatal death (n = 1, secondary to premature closure of ductus arteriosus with cardiac failure), embolic event (n = 1, resulting in unilateral renal agenesis, jejunal atresia), chronic lung disease (n = 1), and tumor recurrence (n = 1). For Fetal SCT, the rapidity at which cardiac compromise can develop and the high incidence of obstetric complications warrant close prenatal surveillance. Amnioreduction, cyst aspiration, and surgical debulking are potentially life-saving Interventions.
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The ex-utero intrapartum treatment.
Current opinion in pediatrics, 2002Co-Authors: Tippi C. Mackenzie, Timothy M. Crombleholme, Alan W. FlakeAbstract:Advances in prenatal diagnosis, combined with a better understanding of the natural history of prenatally diagnosed anomalies, are providing increasing opportunities to consider Fetal Intervention in selected cases of life-threatening malformations. Accurate prenatal diagnosis can now accurately identify Fetal pathophysiology that poses an immediate threat to the life of the newborn infant on separation from the placental circulation. In this circumstance, the ex-utero intrapartum treatment (EXIT) procedure, which maintains intrapartum uteroplacental support, can be life saving. The most common indications for the EXIT procedure are Fetal lesions causing extrinsic or intrinsic airway obstruction. However, fetuses with other anomalies that may compromise neonatal resuscitation can also benefit from this approach. The EXIT procedure differs significantly from a cesarean delivery, and caution must be taken to avoid maternal morbidity. As with all endeavors involving maternal-Fetal Intervention, a team approach is crucial to ensure accurate diagnosis and optimal perinatal management.
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The current status and future potential of Fetal Intervention: image is everything.
Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society, 1999Co-Authors: Alan W. Flake, Timothy M. Crombleholme, N. Scott AdzickAbstract:Fetal imaging is integral to the past, present, and future of Fetal Intervention. In the early history of Fetal Intervention, the role of prenatal ultrasound was primarily to identify a Fetal lesion, provide a correct anatomic diagnosis, and exclude other anatomic defects. We now depend on ultrasound, and other imaging studies, not only to provide anatomic information, but to provide prognostic, physiologic and functional information prior to, during, and after surgery for optimal selection and management of Fetal surgery patients. In the future improvements in imaging and non-invasive technology will drive further expansion of indications and therapeutic options for Fetal Intervention.