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Fetal Surgery Nowadays - Fetal Surgery in the Current Context

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Fetal Surgery Nowadays - Fetal Surgery in the Current Context Rev Med (São Paulo). 2018 mar.abr.;97(2):216-25. doi: http://dx.doi.org/10.11606/issn.1679-9836.v97i2p216-225 Fetal surgery nowadays - Fetal surgery in the current context Cirurgia fetal no contexto atual Luana S. N. da Rocha, Antonio G. de Amorim Filho, Victor Bunduki, Mario Henrique B. de Carvalho, Marco Antonio B. Lopes, Marcelo Zugaib, Rossana P. V. Francisco, Lisandra S. Bernardes Rocha LSN, Amorim Filho AG, Bunduki V, Carvalho MHB, Lopes MAB, Zugaib M, Francisco RPV, Bernardes LS. Fetal surgery nowadays - Fetal surgery in the current context / Cirurgia fetal no contexto atual. Rev Med (São Paulo). 2018 mar.-abr.;97(2):216-25. ABSTRACT: Fetal surgery is now a reality in large centers that RESUMO: A cirurgia fetal é, hoje, uma realidade em grandes specialized in the field of fetal medicine. Fetal surgery emerged centros especializados na área da medicina fetal. Surgiu por volta in the 1960s, primarily from the need to better identify and da década de 1960, inicialmente atrelada à necessidade de melhor understand fetal pathologies, and its exponential development conhecer e entender as patologias fetais, e teve desenvolvimento was driven by improvements in prenatal diagnostic techniques. exponencial impulsionado pelo aprimoramento das técnicas diagnósticas pré-natais. Foi aos poucos se consolidando e Fetal surgery gradually became the main treatment for specific tornando-se o tratamento de escolha para algumas patologias fetal pathologies, changing the course of these diseases. This específicas, mudando definitivamente o curso dessas doenças. study summarizes the history of the fetal surgeries that are Nesse estudo, encontra-se um breve histórico das cirurgias most commonly performed worldwide, how they started and fetais mais comumente realizadas em todo o mundo, como elas how they evolved over time, the main validation trials and surgiram e como foram evoluindo com o passar do tempo, os the most widely used surgical technique in each case. The principais estudos que as validaram e a técnica cirúrgica mais main surgeries include laser photocoagulation of placental amplamente utilizada em cada caso. Entre as principais cirurgias, anastomoses in twin-to-twin transfusion syndrome, in utero repair são citadas a fotocoagulação a laser de anastomoses placentárias of fetal myelomeningocele and fetal endotracheal occlusion in na síndrome de transfusão feto-fetal, a correção intrauterina a céu cases of congenital diaphragmatic hernia. Other surgeries and aberto da mielomeningocele fetal e a oclusão endotraqueal fetal por balão nos casos de hérnia diafragmática congênita. Também procedures, whose benefits are less clear and whose results are são abordados cirurgias e procedimentos cujos benefícios são still somewhat controversial, such as urinary interventions in menos evidentes e os resultados ainda um tanto controversos, cases of lower urinary tract obstruction, cardiac procedures in como as intervenções urinárias nos casos de obstrução ao trato cases of critical aortic stenosis, thoracoamniotic shunts for the urinário inferior, procedimentos cardíacos em casos de estenose treatment of massive pleural effusions and intrauterine resection aórtica crítica, derivações tóracoamnióticas para tratamento de of sacrococcygeal teratomas and pulmonary masses, are also cited. derrames pleurais compressivos, além de ressecções intrauterinas The future of fetal surgery is also assessed. de teratomas sacrococcígeos e de massas pulmonares. Também é feita uma avaliação a respeito do futuro da cirurgia fetal. Keywords: Fetus/surgery; Fetoscopy. Descritores: Feto/cirurgia; Fetoscopia. Grupo de Apoio Integral à Gestantes e Familiares de Fetos com Malformação (GAI) da Clínica Obstétrica e Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Disciplina de Obstetrícia, Departamento de Obstetrícia e Ginecologia, Faculdade de Medicina da Universidade de São Paulo. ORCID: Bernardes LS - 0000-0003-2367-2849; Francisco RPV - 0000-0002-9981-8069. E-mail: [email protected]. br; [email protected]; [email protected]. Correspondence: Lisandra Stein Bernardes. Av. Dr. Enéas de Carvalho Aguiar, 255, 10 andar, Obstetrícia. São Paulo, SP, Brasil. E-mail: lisbernardes@ usp.br 216 Rocha LSN, et al. Fetal surgery nowadays - Fetal surgery in the current context. INTRODUCTION need3 because some congenital diseases were long known to possibly cause progressive and irreversible damage etal medicine is part of modern obstetrics and while the fetus was still in the intrauterine environment. Fmay be defined as the science that studies Accordingly, the hypothesis that the antenatal repair of the development of the fetus and its physiology, the some lesions could prevent or reverse tissue damage, pathophysiology of malformations and congenital disorders restore normal development and allow quality survival and the diagnosis and treatment of these abnormalities was proposed. in the antenatal period, when possible1. Fetal medicine Michael Harrison is recognized as the father of fetal emerged in the 1960s and was initially focused more on surgery. In the early 1980s, he created the Fetal Treatment diagnosing fetal changes and prenatal counseling than on Center at University of California, San Francisco (UCSF), treating the fetus itself. where he pioneered open surgical treatments for fetal Until approximately 40 years ago, developing lower urinary tract obstruction, resection of congenital fetuses were poorly accessible to obstetricians within the cystic adenomatoid malformation (CCAM) and repair of pregnant uterus. However, over the years, this paradigm congenital diaphragmatic hernia (CDH). Together with shifted considerably thanks to breakthroughs in diagnostic his trainees, he established fetal treatment centers in many technologies, particularly in imaging examinations, from of the major pediatric hospitals in the United States of the 1970s and 1980s, which furthered the knowledge and America (USA)4. He defined the criteria and indications for 2 understanding of fetal pathologies . fetal surgeries in 1982, which were adapted and approved A brief history of the emergence of fetal surgery in 1991 by the International Fetal Medicine and Surgery Society (IFMSS)5, as shown in Table 1. Fetal surgical procedures emerged from clinical Table 1 - Criteria for fetal surgery according to Harrison (Harrison, 1991) 1. Accurate diagnosis and staging possible, with the exclusion of associated anomalies; 2. Natural history of the disease is documented and prognosis established; 3. No effective postnatal therapy is available; 4. In utero surgery demonstrated as feasible in animal models, reversing deleterious effects of the condition; 5. Interventions performed in specialized fetal treatment centers within strict protocols and approval of the local ethics committee with the informed consent of the mother or both parents. Another pioneer in the history of fetal surgeries some of the main invasive fetal therapies that are now is Julian De Lia. In the early 1980s, he developed an routinely performed. innovative technique for the treatment of twin-to-twin transfusion syndrome (TTTS): laser photocoagulation of Main Fetal Surgical Procedures placental anastomoses4,6,7. At that time, he also predicted that this would be the most performed procedure for treating I) Laser photocoagulation of placental sick fetuses6. This procedure was initially tested in animal anastomoses in twin-to-twin transfusion syndrome models and then in humans through fetoscopy, accessing TTTS is a complication in approximately 10-15% the uterine cavity by laparotomy and hysterotomy. of all monochorionic twin pregnancies8, and its general In the same period, in Europe, open surgeries for 9 the treatment of fetal anomalies caused maternal morbidity mortality rate reaches 73-100% . TTTS results from at very high rates, which triggered the search for less uneven blood flow between twins due to the predominance invasive alternatives. A major milestone at the time was of unidirectional arteriovenous (AV) anastomoses in the modification of the technique used to treat fetuses the placenta, with an absent or decreased caliber of diagnosed with TTTS. Ville and colleagues, together with arterio-arterial (AA) or venovenous (VV) anastomoses10. the team of Dr. Kypros Nicolaides, in London, adopted Consequently, one of the twins has reduced amniotic the technique of De Lia, accessing the amniotic cavity fluid (donor), and the other has increased amniotic fluid percutaneously but without requiring laparotomy, thereby reducing the morbidity of the open procedure4,6. (receptor). This condition may lead to a number of fetal Thus, new fetal medicine centers emerged, boosted changes and eventually to the death of one or both twins. by the great advances made in Europe and in the USA. The main and most accepted TTTS classification was The collaboration between these centers enabled the proposed by Quintero et al in 199911, who divided the development of multicenter clinical trials that validated syndrome into 5 stages using ultrasound criteria. 217 Rev Med (São Paulo). 2018 mar.-abr.;97(2):216-25. Table 2 - Quintero classification of twin-to-twin transfusion syndrome (TTTS) Oligohydramnios in the donor twin (single deepest pocket ≤ 2 cm) and polyhydramnios in the receptor twin (single Stage I deepest pocket ≥ 8 cm), and the donor twin bladder remains visible in the ultrasound Stage II Donor twin bladder is not visible on the ultrasound Abnormal arterial or venous Doppler in one
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