DOCSLIB.ORG

Mirror Syndrome After Fetoscopic Laser Treatment - a Case Report Síndrome Do Espelho Após Tratamento Laser Por Fetoscopia - Caso Clínico

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Mirror Syndrome After Fetoscopic Laser Treatment - a Case Report Síndrome Do Espelho Após Tratamento Laser Por Fetoscopia - Caso Clínico THIEME 576 Case Report Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Síndrome do espelho após tratamento laser por fetoscopia - caso clínico Ana Maria Simões Brandão1 Ana Patrícia Rodrigues Domingues1 Etelvina Morais Ferreira Fonseca1 Teresa Maria Antunes Miranda1 José Paulo Achando Silva Moura1 1 Obstetrics Unit A, Maternidade Dr. Daniel de Matos, Centro Address for correspondence Ana Brandão, Medical Resident, Rua Hospitalar e Universitário de Coimbra (CHUC), Faculdade de Miguel Torga, 3030-165 Coimbra, Portugal Medicina, Universidade de Coimbra, Portugal (e-mail: [email protected]; [email protected]). Rev Bras Ginecol Obstet 2016;38:576–579. Abstract Mirror syndrome is a rare disease with unknown pathophysiology that can be present in different diseases that can cause fetal hydrops. The prognosis is usually bad with a high Keywords perinatal mortality. We report an unusual form of mirror syndrome that manifested ► mirror syndrome itself only after a successful treatment for fetal hydrops (caused by twin-twin ► fetoscopic laser transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was treatment controlled by medical treatment, and despite the usually bad prognosis seen in these ► twin-twin transfusion cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, syndrome resulting in the birth of 2 live infants. Resumo Asíndromedoespelhoéumadoençarara,defisiopatologia desconhecida, que se manifesta em situações obstétricas responsáveis pela presença de hidrópsia fetal. Habitualmente o prognóstico é reservado, uma vez que se associa a elevadas taxas de Palavras-chave mortalidade perinatal. O presente caso clínico trata de uma situação de síndrome do ► síndrome do espelho espelho que se manifestou, atipicamente, após o tratamento eficazparaahidrópsia ► tratamento a laser fetal associada à síndrome de transfusão feto-fetal. fetoscópico Apesar do mau prognóstico associado a estas situações, conseguiu-se controlar a ► síndrome de situação apenas com tratamento médico e, desta forma, prolongar a gravidez durante transfusão feto-fetal 12 semanas. Introduction maternal edema virtually mirrors the edema of the fetus. The incidence is not clear since it is a rare disease, often – Mirror syndrome, also called Ballantyne’s or triple edema underdiagnosed.1 3 syndrome, was described in 1982 by John Ballantyne as a Fetal hydrops of any etiology, immune and nonimmune combination of maternal edema, fetal and placental hydrops. (►Table 1), can be associated with mirror syndrome, which This syndrome is a complication of fetal hydrops in which the appears when fetal symptoms become severe. Nevertheless, received DOI http://dx.doi.org/ Copyright © 2016 by Thieme-Revinter May 19, 2016 10.1055/s-0036-1593895. Publicações Ltda, Rio de Janeiro, Brazil accepted ISSN 0100-7203. September 27, 2016 Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Brandão et al. 577 Table 1 Different causes associated with mirror syndrome Immune Rh iso immunization Nonimmune Viral infections Parvovirus B19 Human parvovirus Coxsackie virus Structural fetal or placental malformations Sacrococcygeal teratoma Aneurysm of Galen’svein Ebstein’sanomaly Placental chorioangioma Multiple pregnancy Twin-twin transfusion syndrome Fetal arrhythmia the syndrome’s pathogenesis and pathophysiology are still essentially hemodynamic, with implications for the cardio- unknown, and it is associated with a high perinatal vascular systems of both twins, presenting itself more se- – mortality.1 5 verely in the recipient twin, who usually has a progressive According to a few cases described in the literature, the cardiomyopathy as an adaptive fetal response to hemody- gestational age of appearance of this syndrome ranges from namic, hormonal and biochemical stressors.12,13 22 to 29 weeks.2,4 However, other reports suggest a sporadic Fetoscopic laser ablation of the placental anastomoses is earlier or later gestational age of manifestation.2,6 the primary treatment of TTTS, having a deep effect on fetal Symptoms of maternal edema include weight gain; ele- hemodynamics and on the reversal of cardiac dysfunction vated blood pressure with or without proteinuria; oliguria; and valvulopathy, even in severe cases.5,9,13 When mirror tachycardia and tachypnea; acute respiratory failure due to syndrome complicates TTTS, the laser treatment also has the pulmonary edema; and laboratorial signs of hemodilution potential to reverse this complication.8 So far, there have with mild anemia and elevated liver enzymes, thus resem- been very few cases reported in the literature in which bling other complications such as preeclampsia or HELLP mirror syndrome occurred after laser therapy for syndrome.1,3,7 It has been suggested that the presence of TTTS.5,11,14 We report one such unusual case in which mirror hemodilution might be an important criterion to better syndrome presented itself only after fetoscopic laser therapy differentiate mirror syndrome from preeclampsia, which is was done for fetal hydrops in a twin gestation. associated with hemoconcentration.3,8 The similarity between these diseases has led some Case Report authors to designate mirror syndrome as a preeclampsia- like syndrome.7 Several studies and case reports have A 38 year-old woman with a spontaneous monochorionic, emerged in which authors have focused on a possible pla- diamniotic twin pregnancy was referred to our institution at cental dysfunction, where upregulation of antiangiogenic 9 weeks for a multiple pregnancy follow-up. Her personal factors seems to be responsible for the preeclampsia-like medical history included ankylosing spondylitis and hyper- – clinical pictures seen in mirror syndrome.3,4,7 9 somnia, a B Rh-positive blood type, and a previous forceps Mirror syndrome can be reversible when the underlying and breech delivery (gesta 3 para 2). An amniocentesis was factors are identified and corrected.3 Improvement in the performed for advanced maternal age, and it revealed a maternal condition after treating the cause of fetal hydrops normal karyotype in both fetuses. The first ultrasounds has been reported.10 When it is not possible to reverse the were normal, although on the 16th week, a minimal differ- main factor, the prognosis of the syndrome is usually poor, ence in amniotic fluid was found between both fetuses. This with high perinatal mortality, and prompt termination of difference was maintained on subsequent weeks (maximum pregnancy is often recommended.11 Delivery, of course, vertical pocket of 2.4 versus 7.4 cm), but without the criteria should be in accordance with gestational age and maternal of a TTTS. stability.3 Support therapy (diuretics and antihypertensive At 23 weeks of gestation, an ultrasound showed acute fetal therapy) for maternal stability is crucial in preventing the deterioration with oligohydramnios versus polyhydramnios, deterioration of the mother’s condition. discordant bladders, and the recipient twin presented severe Mirror syndrome is also a rare complication of twin-twin hydrops and cardiomegaly (►Fig. 1). The diagnosis of TTTS in transfusion syndrome (TTTS), a severe complication associ- Quintero stage IV was made. Umbilical artery Doppler flow ated with monochorionic twin pregnancies due to the un- indices were abnormal (altered pulsatility index) in the balanced flow of blood from one twin (donor) to its co-twin recipient twin, and his echocardiogram showed a cardiome- (recipient) through arteriovenous anastomoses deep within galy with reduced cardiac ejection fraction and a narrow the shared placental lobules.5,8 It is a progressive disease, aorta. Rev Bras Ginecol Obstet Vol. 38 No. 11/2016 578 Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Brandão et al. Fig. 1 Ultrasound findings of the fetus with hydrops (A and B), cardiomegaly (C) and polydramnios (D). Since our institution does not have experience with laser The postnatal echocardiogram of the ex-recipient twin treatment for TTTS, we contacted and sent the gravid to the showed biventricular hypertrophy and distal dilatation of Clínic Barcelona Hospital Universitari, where a successful the left ventricle with contraction anomaly and hypertrabe- fetoscopic laser therapy was performed. The mother and the culation. Twenty months later, this twin presents no cardiac fetuses responded well on the first hours after this proce- symptoms and has a normal cardiac auscultation, with an dure. However, on the day after, the mother presented an echocardiogram revealing lateral and apical trabeculation acute shortness of breath in a context of intense generalized but a normal ejection fraction. The ex-donor twin has no edema and oliguria, without signs of hypertension or pro- cardiac problem. teinuria. Analytically, she presented a progressive dilutional Despite the physical development being good in both anemia (hemoglobin [Hgb] ¼ 8.3 g/dL; hematocrit [Htc] twins, they are manifesting some delayed motor develop- ¼ 25.7%), normal renal function (Naþ ¼ 134; creatinine ment: sitting without support only at 13 months and walking ¼ 0.47), normal hepatic function, and negative protein to at 17 months. creatinine ratio in urine (0.3). Diuretics (furosemide three ’ times a day ) were introduced into the mother stherapy. Discussion Four days afterward, she was discharged and arrived clinically stable at our obstetrics unit, where diuretics treat- Mirror syndrome is a rare condition, where maternal edema ment, clinical and analytical controls were
Load more

Recommended publications
  • Diagnosis, Treatment and Follow Up
    DOI: 10.1002/jimd.12024 REVIEW International clinical guidelines for the management of phosphomannomutase 2-congenital disorders of glycosylation: Diagnosis, treatment and follow up Ruqaiah Altassan1,2 | Romain Péanne3,4 | Jaak Jaeken3 | Rita Barone5 | Muad Bidet6 | Delphine Borgel7 | Sandra Brasil8,9 | David Cassiman10 | Anna Cechova11 | David Coman12,13 | Javier Corral14 | Joana Correia15 | María Eugenia de la Morena-Barrio16 | Pascale de Lonlay17 | Vanessa Dos Reis8 | Carlos R Ferreira18,19 | Agata Fiumara5 | Rita Francisco8,9,20 | Hudson Freeze21 | Simone Funke22 | Thatjana Gardeitchik23 | Matthijs Gert4,24 | Muriel Girad25,26 | Marisa Giros27 | Stephanie Grünewald28 | Trinidad Hernández-Caselles29 | Tomas Honzik11 | Marlen Hutter30 | Donna Krasnewich18 | Christina Lam31,32 | Joy Lee33 | Dirk Lefeber23 | Dorinda Marques-da-Silva9,20 | Antonio F Martinez34 | Hossein Moravej35 | Katrin Õunap36,37 | Carlota Pascoal8,9 | Tiffany Pascreau38 | Marc Patterson39,40,41 | Dulce Quelhas14,42 | Kimiyo Raymond43 | Peymaneh Sarkhail44 | Manuel Schiff45 | Małgorzata Seroczynska29 | Mercedes Serrano46 | Nathalie Seta47 | Jolanta Sykut-Cegielska48 | Christian Thiel30 | Federic Tort27 | Mari-Anne Vals49 | Paula Videira20 | Peter Witters50,51 | Renate Zeevaert52 | Eva Morava53,54 1Department of Medical Genetic, Montréal Children's Hospital, Montréal, Québec, Canada 2Department of Medical Genetic, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia 3Department of Human Genetics, KU Leuven, Leuven, Belgium 4LIA GLYCOLAB4CDG (International
    [Show full text]
  • Case Report Anesthetic Management of a Parturient with Mirror Syndrome: a Case Report
    Int J Clin Exp Med 2015;8(8):14161-14165 www.ijcem.com /ISSN:1940-5901/IJCEM0009837 Case Report Anesthetic management of a parturient with mirror syndrome: a case report Zhendong Xu, Yan Huan, Yueqi Zhang, Zhiqiang Liu Department of Anesthesiology, Shanghai First Maternity and Infant Hospital, Tongji University School of Medicine, Shanghai 200040, China Received May 3, 2015; Accepted June 23, 2015; Epub August 15, 2015; Published August 30, 2015 Abstract: Mirror syndrome is a rare clinical entity consisting of fetal and placental hydrops with maternal edema. It is associated with an increase in fetal mortality and maternal morbility. We describe the anesthetic management of a parturient with Mirror syndrome complicated by HELLP syndrome and massive postpartum hemorrhage, who required general anesthesia for cesarean delivery. Keywords: Anesthesia, mirror syndrome Introduction and severe edema of vulva for 1 week was admitted to our hospital at 31 weeks and four Mirror syndrome is a rare obstetric entity that days’ gestation. The patient’s body weight was occurs in pregnant women and is secondary to 54.5 kg and height was 158 cm. She had no fetal and placental hydrops. The name was significant medical history and was healthy derived from the maternal signs and symptoms before the pregnancy. The baby was conceived that “mirror” those of the hydropic fetus and naturally and the patient underwent regular placenta [1, 2]. Patients often also have hemo- prenatal examinations. Ultrasonography per- dilutional anemia, hypertension, hypoprotein- formed in the second semester revealed a sin- emia and pulmonary edema [1]. Mirror syn- gleton with normal fetal umbilical blood flow but drome is not yet well recognized in clinical prac- thickening of the right ventricular myocardium.
    [Show full text]
  • Lessons Learned from Two Missed Prenatal Cases of Hemoglobin
    tics: Cu ne rr e en G t y R r e a Wu et al., Hereditary Genetics 2015, S7 t s i e d a e r r c DOI: 10.4172/2161-1041.S7-003 e h H Hereditary Genetics ISSN: 2161-1041 Case Report Open Access Lessons Learned from Two Missed Prenatal Cases of Hemoglobin Bart’s Hydrops Fetalis Until Second Trimester Despite a Nationwide Screening Program Wu WJ1,2#, Ma GC1,3#, Wu PC4, Huang KS5, Liu YL5, Chang SP1, Ginsberg NA6 and Chen M1,2,7,8* 1Department of Genomic Medicine, and Department of Genomic Technology and Science, Changhua Christian Hospital, Changhua, Taiwan 2Department of Obstetrics and Gynecology, Changhua Christian Hospital, Changhua, Taiwan 3Institute of Biochemistry, Microbiology and Immunology, Chung-Shan Medical University, Taichung, Taiwan 4Taiji Fetal Medicine Center, Taipei, Taiwan 5Department of Obstetrics and Gynecology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan 6Department of Obstetrics and Gynecology, Feinberg School of Medicine, Northwestern University Medical Center, Chicago, IL, USA 7Department of Obstetrics and Gynecology, College of Medicine, and Hospital, National Taiwan University, Taipei, Taiwan 8Department of Life Science, Tunghai University, Taichung, Taiwan #These authors contributed equally to this study Abstract Hemoglobin (Hb) Bart’s hydrops fetalis is the most severe form of alpha-thalassemia and has high mortality. It is caused by deletion of all four α-globin genes leading to a severe deficiency in α-globin and to the production of γ-globin tetramers, resulting in ineffective tissue oxygen delivery. Current preconceptional or prenatal screening policies for this disorder are comprehensive, but not all pregnancies with Hb Bart’s disease can be detected early before hydrops become apparent.
    [Show full text]
  • Mirror Syndrome After Fetoscopic Laser Treatment - a Case Report Síndrome Do Espelho Após Tratamento Laser Por Fetoscopia-Casoclínico
    THIEME 576 Case Report Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Síndrome do espelho após tratamento laser por fetoscopia-casoclínico Ana Maria Simões Brandão1 Ana Patrícia Rodrigues Domingues1 Etelvina Morais Ferreira Fonseca1 Teresa Maria Antunes Miranda1 José Paulo Achando Silva Moura1 1 Obstetrics Unit A, Maternidade Dr. Daniel de Matos, Centro Address for correspondence Ana Brandão, Medical Resident, Rua Hospitalar e Universitário de Coimbra (CHUC), Faculdade de Miguel Torga, 3030-165 Coimbra, Portugal Medicina, Universidade de Coimbra, Portugal (e-mail: [email protected]; [email protected]). Rev Bras Ginecol Obstet 2016;38:576–579. Abstract Mirror syndrome is a rare disease with unknown pathophysiology that can be present in different diseases that can cause fetal hydrops. The prognosis is usually bad with a high Keywords perinatal mortality. We report an unusual form of mirror syndrome that manifested ► mirror syndrome itself only after a successful treatment for fetal hydrops (caused by twin-twin ► fetoscopic laser transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was treatment controlled by medical treatment, and despite the usually bad prognosis seen in these ► twin-twin transfusion cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, syndrome resulting in the birth of 2 live infants. Resumo Asíndromedoespelhoéumadoençarara,defisiopatologia desconhecida, que se manifesta em situações obstétricas responsáveis pela presença de hidrópsia fetal. Habitualmente o prognóstico é reservado, uma vez que se associa a elevadas taxas de Palavras-chave mortalidade perinatal. O presente caso clínico trata de uma situação de síndrome do ► síndrome do espelho espelho que se manifestou, atipicamente, após o tratamento eficazparaahidrópsia ► tratamento a laser fetal associada à síndrome de transfusão feto-fetal.
    [Show full text]
  • Non-Immune Fetal Hydrops
    Non-Immune Fetal Hydrops (NIHF) MATY090 Type: Guideline HDSS Certification Standard: Issued by: Maternity PPG Group Version: 1 Applicable to: Maternity, Childrens, Radiology Contact person: O & G SMO Lead DHB: HVDHB Level: Hutt Maternity Policies provide guidance for the midwives and medical staff working in Hutt Maternity Services. Please discuss policies relevant to your care with your Lead Maternity Carer. Purpose: To provide guidance and a consistent approach for the accurate diagnosis and management of people and babies presenting with non-immune fetal hydrops in the Secondary Care setting. Scope: For the purposes of this document, staff will refer to: All staff within Hutt Valley DHB. This includes staff not working in direct contact with patients/consumers. Staff are taken to include anyone engaged in working to the Hutt Valley DHB. This may include but is not limited to: o Employees irrespective of their length of service o Agency workers o Self-employed workers o Consultants o Third party service providers, and any other individual or suppliers working in Hutt Maternity, including Lead Maternity Carers, personnel affiliated with third parties, contractors, temporary workers and volunteers o Students Definitions: SDP Single deepest pool SVT Supraventricular tachycardia SLE Systemic lupus erythematosus TTTS Twin-to twin-transfusion syndrome CPAM Congenital pulmonary airway malformation G6PD Glucose -6-phosphate dehydrogenase MCA Middle cerebral artery PSV Peak systolic velocity PTL Preterm labour TOP Termination of pregnancy Background Document author: O&G SMO Authorised by: Maternity PPG Group Issue date: May 2021 Review date: May 2023 Date first issued: May 2021 Document ID: MATY090 Page 1 of 6 CONTROLLED DOCUMENT – The electronic version is the most up to date version.
    [Show full text]
  • Department of Obstetrics, Gynecology, and Reproductive Sciences
    University of Pittsburgh School of Medicine DEPARTMENT OF OBSTETRICS, GYNECOLOGY, AND REPRODUCTIVE SCIENCES ANNUAL REPORT – ACADEMIC YEAR 2019 Tatomir, Shannon DEPARTMENT OF OBSTETRICS, GYNECOLOGY, AND REPRODUCTIVE SCIENCES UNIVERSITY OF PITTSBURGH SCHOOL OF MEDICINE ANNUAL REPORT Academic Year 2019 July 1, 2018 – June 30, 2019 300 Halket Street Pittsburgh, PA 15213 412.641.4212 1 TABLE OF CONTENTS YEAR IN REVIEW MISSION STATEMENT ..................................................................................................................... 3 CHAIR’S ADDRESS ........................................................................................................................... 4 RECRUITMENTS ............................................................................................................................... 6 DEPARTURES ................................................................................................................................... 7 DEPARTMENT PROFESSIONAL MEMBERS ………………………………………………………………………………...8 DIVISION SUMMARIES OF RESEARCH, TEACHING AND CLINICAL PROGRAMS DIVISION OF GYNECOLOGIC SPECIALTIES .................................................................................... 11 DIVISION OF GYNECOLOGIC ONCOLOGY ..................................................................................... 24 DIVISION OF MATERNAL FETAL MEDICINE .................................................................................. 34 DIVISION OF REPRODUCTIVE ENDOCRINOLOGY AND INFERTILITY ...........................................
    [Show full text]
  • Sacrococcygeal Teratoma Associated with Maternal-Mirror Syndrome
    Bahrain Medical Bulletin, Vol. 41, No.3, September 2019 Sacrococcygeal Teratoma associated with Maternal-Mirror Syndrome Amal Ali Hassani, CABOG, Mmed, MPHE* Dalya Al Hamdan, MD** Fatema A. Redha Hasan, ABOG*** Sacrococcygeal Teratoma (SCT) is one of the most common congenital tumors in newborns. The majority of the cases are type I and II; therefore, the diagnosis of SCT is mostly made in prenatal period especially during second-trimester ultrasound. This is the first case reported in Bahrain of a pregnant lady in her 20th week of gestation, carrying a fetus with a rapidly growing tumor in its sacrococcygeal area. The woman developed a rare condition of severe pre-eclampsia, cardiomegaly, bilateral bronchopneumonia and pleural effusion suggestive of maternal mirror syndrome (MS). Early prenatal diagnosis of SCT with early detection of maternal mirror syndrome is extremely challenging. Bahrain Med Bull 2019; 41(3): 184 - 187 Sacrococcygeal teratoma (SCT) is considered one of the most The ultrasound had a provisional diagnosis of sacrococcygeal common congenital tumors in newborns. In a study review, mass of the fetus. The possibility of termination of pregnancy most of the cases showed benign nature irrespective of size, was raised due to the progressive growth of the mass up to age, Altman classification or age at diagnosis; recurrence rate is double the size of the fetus; the fundal height of 36 weeks was 11% after surgery1. SCT is originally a germ cell tumor (GCT) associated with polyhydramnios and placentomegaly. The which commonly occurs in infants and young children2. A patient reported having difficulty in passing urine for 5 days.
    [Show full text]
  • Mirror Syndrome: a Novel Cause of Shortness of Breath in the Third Trimester of Pregnancy
    Case Report Annals of Clinical Case Reports Published: 03 Apr, 2017 Mirror Syndrome: A Novel Cause of Shortness of Breath in the Third Trimester of Pregnancy Emilie J Calvello Hynes*1 and Rouda Nuaimi2 1Department of Emergency Medicine, University of Colorado, USA 2Department of Internal Medicine and General Surgery, Dubai Medical College, UAE Abstract Mirror syndrome is a rare association of fetal and placental hydrops with maternal pre-eclampsia and edema. We report a case of a 32 years old pregnant female at 28 weeks of gestation with hydrops fetalis who presented to our Emergency Department with severe shortness of breath, elevated blood pressure and bilateral lower limb edema. The patient underwent bedside ultrasound, which showed pulmonary edema and bilateral pleural effusions with normal cardiac function. The patient was diagnosed with Mirror Syndrome and underwent an emergency cesarean section delivery. The case illustrates a novel cause of shortness of breath in the third trimester that is an indication for emergent obstetric consult and delivery. Keywords: Mirror Syndrome; Preeclampsia; Hydrops fetalis; Edema Introduction Dyspnea during pregnancy is a common complaint; approximately 60% -70% of women will have dyspnea with no previous history of cardiopulmonary disease [1,2]. The mechanical effect of 20 the enlarging gravid uterus and the superior displacement of diaphragm, is counterbalanced OPEN ACCESS by hormonally induced increased excursion of the rib cage [1-4]. Progesterone mediated hyperventilation is thought to be due to the increased sensitivity of respiratory center to PaCO2, *Correspondence: which increases the minute ventilation and the tidal volume [2,3,5]. The pregnant woman Emilie J Calvello Hynes, Department subjectively perceives this hyperventilation as shortness of breath [5].
    [Show full text]
  • Prenatal Counseling Series Sacrococcygeal Tumors
    American Pediatric Surgical Association Prenatal Counseling Series Sacrococcygeal Tumors from the Fetal Diagnosis and Treatment Committee of the American Pediatric Surgical Association Editor-in-Chief: Ahmed I. Marwan, MD Special thanks to: Amanda Jensen, MD, Erin Perrone, MD, and Jill Stein, MD ©2018, American Pediatric Surgical Association American Pediatric Surgical Association Prenatal Counseling Series Sacrococcygeal Tumors Sacrococcygeal Tumors • Sacrococcygeal tumors (SCT) are one of the most common congenital neoplasms of the newborn period with a prevalence of 1:27-40,000 live births. • They arise from a totipotent stem cell in the coccyx (Henson’s node) and are generally benign in fetal and early neonatal life. • Incidence is 4 times more common in females. • Sacrococcygeal tumors are classified into four categories : • Complications related to prenatally diagnosed SCTs may include polyhydramnios, fetal cardiac failure, fetal hydrops, placentomegaly, maternal mirror syndrome, tumor hemorrhage and prematurity. • Prenatally diagnosed SCTs have 3 times the mortality rate compared to postnatally diagnosed neonates with a mortality rate ranging from 15-35%. • Approximately 15-30% have associated congenital defects including nervous, cardiac, gastrointestinal, genitourinary and musculoskeletal. • Local abnormalities such as rectovaginal fistula, urethro-vaginal fistula, urethral atresia and imperforate anus are directly related to tumor growth. 2 American Pediatric Surgical Association Prenatal Counseling Series Sacrococcygeal Tumors Sagittal MRI images of a fetus with a large pre sacral mass composed of mixed cystic and solid components. The majority of the mass is exophytic with a small component located within the pelvis (type 1). Color Doppler ultrasound image shows internal blood flow within the solid components of the mass.
    [Show full text]
  • Twin to Twin Transfusion Syndrome
    1529 Review Article on Fetal Surgery Twin to twin transfusion syndrome Jena L. Miller^ The Johns Hopkins Center for Fetal Therapy, Department of Gynecology and Obstetrics, Johns Hopkins University, Baltimore, MD, USA Correspondence to: Jena L. Miller, MD. Assistant Professor, The Johns Hopkins Center for Fetal Therapy, Department of Gynecology and Obstetrics, 600 N Wolfe St, Nelson 228, Baltimore, MD 21287, USA. Email: [email protected]. Abstract: Twin to twin transfusion syndrome (TTTS) is a common complication that typically presents in the second trimester of pregnancy in 10–15% of monochorionic twins due to net transfer of volume and hormonal substances from one twin to the other across vascular anastomoses on the placenta. Without recognition and treatment, TTTS is the greatest contributor to fetal loss prior to viability in 90–100% of advanced cases. Ultrasound diagnosis of monochorionicity is most reliable in the first trimester and sets the monitoring strategy for this type of twins. The diagnosis of TTTS is made by ultrasound with the findings of polyhydramnios due to volume overload and polyuria in one twin and oligohydramnios due to oliguria of the co-twin. Assessment of bladder filling as well as arterial and venous Doppler patterns are required for staging disease severity. Assessment of fetal cardiac function also provides additional insight into the fetal cardiovascular impacts of the disease as well as help identify fetuses that may require postnatal follow up. Fetoscopic laser ablation of the communicating vascular anastomoses between the twins is the standard treatment for TTTS. It aims to cure the condition by interrupting the link between their circulations and making them independent of one another.
    [Show full text]
  • Resolution of Maternal Mirror Syndrome After Succesful Fetal
    Chimenea et al. BMC Pregnancy and Childbirth (2018) 18:85 https://doi.org/10.1186/s12884-018-1718-0 CASE REPORT Open Access Resolution of maternal Mirror syndrome after succesful fetal intrauterine therapy: a case series Angel Chimenea1, Lutgardo García-Díaz1, Ana María Calderón1, María Moreno-De Las Heras1 and Guillermo Antiñolo1,2* Abstract Background: Mirror syndrome (MS) is a rare obstetric condition usually defined as the development of maternal edema in association with fetal hydrops. The pathogenesis of MS remains unclear and may be misdiagnosed as pre-eclampsia. Case presentation: We report a case series of MS in which fetal therapy (intrauterine blood transfusion and pleuroamniotic shunt) resulted in fetal as well as maternal favourable course with complete resolution of the condition in both mother and fetus. Conclusions: Our case series add new evidence to support that early diagnosis of MS followed by fetal therapy and clinical maternal support are critical for a good outcome. Keywords: Mirror syndrome, Ballantyne syndrome, Parvovirus B19, Hydrops fetalis, Fetal therapy Background pre-eclampsia, delaying diagnosis and therapy of MS and Mirror syndrome (MS) is a rare complication of fetal worsening fetal and maternal condition. MS does not hydrops appearing as a triple edema (fetal, placental as usually present with hypertension, but blood pressure can well as maternal) [1], in which the mother “mirrors” the be elevated and proteinuria can appear, resembling the hydropic fetus. This syndrome was first described in 1892 clinical features of pre-eclampsia [3, 16]. Like in pre- by the Scottish obstetrician John William Ballantyne [2]. eclampsia, laboratory findings may include proteinuria, There have been multiple feto-placental diseases related low platelet count and elevation of creatinine, hepatic to MS, that can be classified into diverse groups based on enzymes and uric acid levels [3].
    [Show full text]
  • Beating the Odds: a Case Report on the Successful Management of a Non-Immune Hydrops Fetalis Due to Hemoglobin Bart's Disease
    Beating the odds: A case report on the successful management of a non-immune hydrops fetalis due to hemoglobin Bart’s disease* By Maria Jane Ellise S. Javier, MD; Maria Rosario C. Cheng, MD, FPOGS and Marinella Agnes G. Abat, MD, FPOGS Department of Obstetrics and Gynecology, The Medical City ABSTRACT Hemoglobin Bart’s hydrops fetalis, characterized by a deletion of all four α-globin genes is the most severe and lethal form of Thalassemia disease. Mortality rate usually ranges from 60-100% of cases.1-5 Given the poor overall prognosis, most countries resort to pregnancy termination or expectant management as the only options to offer affected pregnancies.5 This paper presents a case of the successful management of a primigravid, diagnosed with hydrops fetalis at 29 4/7 weeks age of gestation. She delivered successfully to a live, preterm, baby boy who was later found out to have hydrops fetalis due to Hemoglobin Bart’s disease, and currently, continues to thrive past eight months of age. This report aims to improve the clinicians’ knowledge regarding the work up and management of pregnant patients diagnosed with hydrops fetalis, and increase the clinician’s awareness on the epidemiology, importance of targeted screening, and diagnosis of Alpha-Thalassemia in Filipino patients. Keywords: Alpha-Thalassemia, Hemoglobin Bart’s, Hydrops Fetalis, Philippines INTRODUCTION In the Philippines, over 5% of the population are carriers of α0-thalassemia trait1, characterized by ydrops fetalis is a serious fetal condition two α-gene deletion, and most often from the same characterized by abnormal accumulation of fluid chromosome (heterozygous α-thalassemia 1).
    [Show full text]