DEVELOPMENTAL ABNORMALITIES of MULLERIAN DUCT- a REVIEW Kowsalya.R.G1, Padmasaritha.K2, Ramesh M3
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Te2, Part Iii
TERMINOLOGIA EMBRYOLOGICA Second Edition International Embryological Terminology FIPAT The Federative International Programme for Anatomical Terminology A programme of the International Federation of Associations of Anatomists (IFAA) TE2, PART III Contents Caput V: Organogenesis Chapter 5: Organogenesis (continued) Systema respiratorium Respiratory system Systema urinarium Urinary system Systemata genitalia Genital systems Coeloma Coelom Glandulae endocrinae Endocrine glands Systema cardiovasculare Cardiovascular system Systema lymphoideum Lymphoid system Bibliographic Reference Citation: FIPAT. Terminologia Embryologica. 2nd ed. FIPAT.library.dal.ca. Federative International Programme for Anatomical Terminology, February 2017 Published pending approval by the General Assembly at the next Congress of IFAA (2019) Creative Commons License: The publication of Terminologia Embryologica is under a Creative Commons Attribution-NoDerivatives 4.0 International (CC BY-ND 4.0) license The individual terms in this terminology are within the public domain. Statements about terms being part of this international standard terminology should use the above bibliographic reference to cite this terminology. The unaltered PDF files of this terminology may be freely copied and distributed by users. IFAA member societies are authorized to publish translations of this terminology. Authors of other works that might be considered derivative should write to the Chair of FIPAT for permission to publish a derivative work. Caput V: ORGANOGENESIS Chapter 5: ORGANOGENESIS -
Pregnancy in Non-Communicating Unicornuate Uterus
THIEME 640 Case Report Pregnancy in Non-Communicating Unicornuate Uterus: Diagnosis Difficulty and Outcomes – aCaseReport Gestação em útero unicorno não comunicante: dificuldadediagnósticaedesfechos– relato de caso Camila Silveira de Souza1 Gabriela Gindri Dorneles1 Giana Nunes Mendonça1 Caroline Mombaque dos Santos1 Francisco Maximiliano Pancich Gallarreta1 Cristine Kolling Konopka1 1 Department of Gynecology and Obstetrics, Universidade Federal de Address for correspondence Cristine Kolling Konopka, MD, PhD, Santa Maria, Santa Maria, Rio Grande do Sul, Brazil Universidade Federal de Santa Maria, Avenida Roraima, 1000, prédio 26, sala 1333, Camobi, 97105-900, Santa Maria, RS, Brazil Rev Bras Ginecol Obstet 2017;39:640–644. (e-mail: [email protected]). Abstract Approximately 1 in every 76,000 pregnancies develops within a unicornuate uterus with a rudimentary horn. Müllerian uterus anomalies are often asymptomatic, thus, the diagnosis is a challenge, and it is usually made during the gestation or due to its complications, such as uterine rupture, pregnancy-induced hypertension, antepartum, Keywords postpartum bleeding and intrauterine growth restriction (IUGR). In order to avoid ► uterus unnecessary cesarean sections and the risks they involve, the physicians should ► abnormalities consider the several approaches and for how long it is feasible to perform labor ► pregnancy induction in suspected cases of pregnancy in a unicornuate uterus with a rudimentary ► parturition horn, despite the rarity of the anomaly. This report describes a case of a unicornuate ► pregnancy uterus in which a pregnancy developed in the non-communicating rudimentary horn complications and the consequences of the delayed diagnosis. Resumo Aproximadamente 1 em cada 76 mil gestações se desenvolvem em útero unicorno sem comunicação com o colo uterino. -
Successful Uterovaginal Anastomosis in an Unusual Presentation Of
JSAFOMS Successful Uterovaginal Anastomosis in an Unusual Presentation10.5005/jp-journals-10032-1056 of Congenital Absence of Cervix CASE REPORT Successful Uterovaginal Anastomosis in an Unusual Presentation of Congenital Absence of Cervix 1Nusrat Mahmud, 2Naushaba Tarannum Mahtab, 3TA Chowdhury, 4Anjan Kumar Deb ABSTRACT Source of support: Nil Cervical agenesis or dysgenesis (fragmentation, fibrous cord Conflict of interest: None and obstruction) is an extremely rare congenital anomaly. Conser vative surgical approach to these patients involves uterovaginal anastomosis, cervical canalization and cervical INTRODUCTION reconstruction. In failed conservative surgery, total hysterec- Primary amenorrhea is defined as absence of menstrua- tomy is the treatment of choice. We report what we believe to be the first successful end-to-end uterovaginal anastomosis of tion by the age of 14 years in the absence of secondary an unusual case of congenital cervical agenesis. A 25-year- sex characteristics or the absence of periods by the age of old female presented complaining of primary amenorrhea 16 years regardless of appearance of secondary sex and primary subfertility for the same duration. At laparoscopy, complete separation between the cervix and the body of the charac ters. In our last study, a series of total 108 cases uterus was found and hanging from surrounding supports. of primary amenorrhea were reviewed. It was found Both ovaries and fallopian tubes were anatomically positioned. that 69.4% were due Müllerian dysgenesis, 19.4% due to There was another muscular tissue of 2 cm in diameter at the gonadal dysgenesis, 2.7% male pseudohermaphroditism pouch of Douglas which was attached with lateral pelvic wall 13 by transverse cervical ligament. -
Pregnancy in a Unicornuate Uterus with Non-Communicating Rudimentary Horn: Diagnostic and Therapeutic Challenges
Contents lists available at Vilnius University Press Acta medica Lituanica ISSN 1392-0138 eISSN 2029-4174 2020. Vol. 27. No 2, pp. 84–89 DOI: https://doi.org/10.15388/Amed.2020.27.2.6 Pregnancy in a Unicornuate Uterus with Non-Communicating Rudimentary Horn: Diagnostic and Therapeutic Challenges Ratko Delić Department of Obstetrics and Gynecology, General and Teaching Hospital Celje, Slovenia Abstract. Unicornuate uterus with non-communicating rudimentary horn is a type of congenital uterine abnormality that occurs as a consequence of the arrested development of one of the two Müllerian ducts. Patients with unicornuate uterus have increased incidence of obstetric and gynaecological complications. We present a report of a clinical case of a 28-years-old female, who was referred to the hospital for evalu- ation of her infertility. The patient reported primary infertility and inability to conceive after 3-year period of regular unprotected intercourse. Transvaginal ultrasound, along with the preoperative evaluation were completed; however, no anomalies or irregularities were reported. Combined diagnostic simultaneous laparoscopy and hysteroscopy were performed to establish the diag- nosis of unicornuate uterus with non-communicating rudimentary horn. The patient conceived spontaneously after diagnostic laparoscopy and hysteroscopy. During and after pregnancy, our patient and her child experienced numerous complications (cervical incompetence, acute chorioamnionitis, acute fetal distress, pneumonia, septic shock) and procedures (cer- vical cerclage, urgent cesarean section, intensive care unit treatment) without significant fetal or maternal compromise. Keywords: infertility, unicornuate uterus, pregnancy, cervical incompetence, sepsis Nėštumas vienaragėje gimdoje su rudimentiniu nesusijungusiu ragu Santrauka. Vienaragė gimda su rudimentiniu nesusijungusiu ragu yra įgimta makšties anomalija, atsiradusi sutrikus vieno iš dviejų Miulerio latakų vystymuisi. -
Cervical and Vaginal Agenesis: a Novel Anomaly
Cervical and Vaginal Agenesis: A Novel Anomaly Case Report Cervical and Vaginal Agenesis: A Novel Anomaly Irum Sohail1, Maria Habib 2 1Professor Obs/Gynae, 2Postgraduate Trainee, Wah Medical College, Wah Cantt Address of Correspondence: Dr. Maria Habib, Postgraduate Trainee, Wah Medical College, Wah Cantt Email: [email protected] Abstract Background: Cervical agenesis with vaginal agenesis is an extremely rare congenital anomaly. This mullerian anomaly occurs in 1 in 80,000-100,000 births. It is classified as type IB in the American Fertility Society Classification of mullerian anomalies. Case report: We report a case presented to POF Hospital, Wah cantt with primary amenorrhea and cyclic lower abdominal pain. She was diagnosed to have cervical agenesis associated with completely absent vagina. Conservative surgical approach to these patients involve uterovaginal anastomosis and cervical reconstruction. Creation of neovagina is necessary in these cases. Due to high failure rate and potential for complications, total hysterectomy with vaginoplasty is the treatment of choice by many authors. Conclusion: A thorough investigation of the patients with primary amenorrhea is necessary and total hysterectomy with vaginoplasty is feasible and should be considered as a first-line treatment option in cases of cervical and vaginal agenesis. Key words: Primary amenorrhea, Cervical agenesis, Vaginal agenesis, Hysterectomy. Introduction The female reproductive organs develop from the Society of Human Reproduction and Embryology fusion of the bilateral paramesonephric (Müllerian) (ESHRE)/European Society for Gynaecological ducts to form the uterus, cervix, and upper two- Endoscopy (ESGE) classification system of female thirds of the vagina.1 The lower third of the vagina genital anomalies is designed for clinical orientation develops from the sinovaginal bulbs of the and it is based on the anatomy of the female urogenital sinus.2 Mullerian duct anomalies (MDAs) genital tract. -
Genetic Syndromes and Genes Involved
ndrom Sy es tic & e G n e e n G e f Connell et al., J Genet Syndr Gene Ther 2013, 4:2 T o Journal of Genetic Syndromes h l e a r n a DOI: 10.4172/2157-7412.1000127 r p u y o J & Gene Therapy ISSN: 2157-7412 Review Article Open Access Genetic Syndromes and Genes Involved in the Development of the Female Reproductive Tract: A Possible Role for Gene Therapy Connell MT1, Owen CM2 and Segars JH3* 1Department of Obstetrics and Gynecology, Truman Medical Center, Kansas City, Missouri 2Department of Obstetrics and Gynecology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 3Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA Abstract Müllerian and vaginal anomalies are congenital malformations of the female reproductive tract resulting from alterations in the normal developmental pathway of the uterus, cervix, fallopian tubes, and vagina. The most common of the Müllerian anomalies affect the uterus and may adversely impact reproductive outcomes highlighting the importance of gaining understanding of the genetic mechanisms that govern normal and abnormal development of the female reproductive tract. Modern molecular genetics with study of knock out animal models as well as several genetic syndromes featuring abnormalities of the female reproductive tract have identified candidate genes significant to this developmental pathway. Further emphasizing the importance of understanding female reproductive tract development, recent evidence has demonstrated expression of embryologically significant genes in the endometrium of adult mice and humans. This recent work suggests that these genes not only play a role in the proper structural development of the female reproductive tract but also may persist in adults to regulate proper function of the endometrium of the uterus. -
Septate Uterus As Congenital Uterine Anomaly: a Case Report
em & yst Se S xu e a v l i t D c i s u o Reproductive System & Sexual Moghadam et al., Reprod Syst Sex Disord 2014, 3:4 d r o d r e p r e DOI: 10.4172/2161-038X.1000141 s R ISSN: 2161-038X Disorders: Current Research Case Report Open Access Septate Uterus as Congenital Uterine Anomaly: A Case Report Abas Heidari Moghadam1,2, Zahra Jozi1, Shapoor Dahaz1 and DarioushBijan Nejad1* 1Department of Anatomical Sciences, Faculty of Medicine, Ahvaz Jundishapour University of Medical Sciences (AJUMS), Ahvaz, Iran 2Diagnostic Imaging Center of Ahvaz Oil Grand Hospital, Ahvaz, Iran *Correspondingauthor: Darioush Bijan Nejad, Assistant Professor, Department of Anatomical Sciences, Faculty of Medicine, Ahvaz Jundishapour University of Medical Sciences (AJUMS), Ahvaz, Iran, Tel: +98 918 343 4253; Fax: +98 611 333 6380; E-mail:[email protected] Received: June 14, 2014; Accepted: August 01, 2014; Published: August 08, 2014 Copyright: © 2013 Moghadam AH, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Abnormal fusion of Mullerian duct in embryonic life is the origin of variety of malformations which may alter the reproductive outcome of the patients. Septate uterus is caused by incomplete resorption of the Mullerian duct during embryogenesis. Here, we report a case of septate uterus that was initially diagnosed by ultrasound scan and confirmed by Magnetic Resonance Imaging (MRI) technique. Keywords: Septate uterus; Mullarian ducts; Ultrasound; MRI Case Report A 29 year old lady came to the imaging diagnostic center of Ahvaz Introduction Oil Grand Hospital. -
Uterine Conserving Surgery in a Case of Cervicovaginal Agenesis with Cloacal Malformation
International Journal of Reproduction, Contraception, Obstetrics and Gynecology Mishra V et al. Int J Reprod Contracept Obstet Gynecol. 2017 Mar;6(3):1144-1148 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20170604 Case Report Uterine conserving surgery in a case of cervicovaginal agenesis with cloacal malformation Vineet Mishra1*, Suwa Ram Saini2, Priyankur Roy1, Rohina Aggarwal1, Ruchika Verneker1, Shaheen Hokabaj1 1Department of Obstetrics and Gynecology, IKDRC, Ahmedabad, Gujarat, India 2Department of Obstetrics and Gynecology, S. P. Medical College, Bikaner, Rajasthan, India Received: 30 December 2016 Accepted: 02 February 2017 *Correspondence: Dr. Vineet Mishra, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Cervico-vaginal agenesis (MRKHS) with normally formed uterus along with cloacal malformation is a very rare mullerian anomaly. We report a case, of a 13-year-old girl who was admitted at our tertiary care center with complaints of primary amenorrhea and cyclical lower abdominal pain for 3 months. Clinical examination and radiological investigations revealed complete cervico-vaginal agenesis with normal uterus with hematometra with horse shoe kidney. Vaginoplasty was done by McIndoe’s method with uterovaginal anastomosis and neocervix formation. Malecot’s catheter was inserted in uterine cavity. Vaginal mould was kept in the neovagina. Mould was removed after 10 days under anaesthesia and repeat hysteroscopy with insertion of a small piece of malecot’s catheter was performed under hysteroscopic guidance into the uterine cavity through neocervix and lower end fixed to the vagina. -
Female Infertility: Ultrasound and Hysterosalpoingography
s z Available online at http://www.journalcra.com INTERNATIONAL JOURNAL OF CURRENT RESEARCH International Journal of Current Research Vol. 11, Issue, 01, pp.745-754, January, 2019 DOI: https://doi.org/10.24941/ijcr.34061.01.2019 ISSN: 0975-833X RESEARCH ARTICLE FEMALE INFERTILITY: ULTRASOUND AND HYSTEROSALPOINGOGRAPHY 1*Dr. Muna Mahmood Daood, 2Dr. Khawla Natheer Hameed Al Tawel and 3 Dr. Noor Al _Huda Abd Jarjees 1Radiologist Specialist, Ibin Al Atheer hospital, Mosul, Iraq 2Lecturer Radiologist Specialist, Institue of radiology, Mosul, Iraq 3Radiologist Specialist, Ibin Al Atheer Hospital, Mosu, Iraq ARTICLE INFO ABSTRACT Article History: The causes of female infertility are multifactorial and necessitate comprehensive evaluation including Received 09th October, 2018 physical examination, hormonal testing, and imaging. Given the associated psychological and Received in revised form th financial stress that imaging can cause, infertility patients benefit from a structured and streamlined 26 November, 2018 evaluation. The goal of such a work up is to evaluate the uterus, endometrium, and fallopian tubes for Accepted 04th December, 2018 anomalies or abnormalities potentially preventing normal conception. Published online 31st January, 2019 Key Words: WHO: World Health Organization, HSG, Hysterosalpingography, US: Ultrasound PID: pelvic Inflammatory Disease, IV: Intravenous. OHSS: Ovarian Hyper Stimulation Syndrome. Copyright © 2019, Muna Mahmood Daood et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Citation: Dr. Muna Mahmood Daood, Dr. Khawla Natheer Hameed Al Tawel and Dr. Noor Al _Huda Abd Jarjees. 2019. “Female infertility: ultrasound and hysterosalpoingography”, International Journal of Current Research, 11, (01), 745-754. -
AMENORRHOEA Amenorrhoea Is the Absence of Menses in a Woman of Reproductive Age
AMENORRHOEA Amenorrhoea is the absence of menses in a woman of reproductive age. It can be primary or secondary. Secondary amenorrhoea is absence of periods for at least 3 months if the patient has previously had regular periods, and 6 months if she has previously had oligomenorrhoea. In contrast, oligomenorrhoea describes infrequent periods, with bleeds less than every 6 weeks but at least one bleed in 6 months. Aetiology of amenorrhea in adolescents (from Golden and Carlson) Oestrogen- Oestrogen- Type deficient replete Hypothalamic Eating disorders Immaturity of the HPO axis Exercise-induced amenorrhea Medication-induced amenorrhea Chronic illness Stress-induced amenorrhea Kallmann syndrome Pituitary Hyperprolactinemia Prolactinoma Craniopharyngioma Isolated gonadotropin deficiency Thyroid Hypothyroidism Hyperthyroidism Adrenal Congenital adrenal hyperplasia Cushing syndrome Ovarian Polycystic ovary syndrome Gonadal dysgenesis (Turner syndrome) Premature ovarian failure Ovarian tumour Chemotherapy, irradiation Uterine Pregnancy Androgen insensitivity Uterine adhesions (Asherman syndrome) Mullerian agenesis Cervical agenesis Vaginal Imperforate hymen Transverse vaginal septum Vaginal agenesis The recommendations for those who should be evaluated have recently been changed to those shown below. (adapted from Diaz et al) Indications for evaluation of an adolescent with primary amenorrhea 1. An adolescent who has not had menarche by age 15-16 years 2. An adolescent who has not had menarche and more than three years have elapsed since thelarche 3. An adolescent who has not had a menarche by age 13-14 years and no secondary sexual development 4. An adolescent who has not had menarche by age 14 years and: (i) there is a suspicion of an eating disorder or excessive exercise, or (ii) there are signs of hirsutism, or (iii) there is suspicion of genital outflow obstruction Pregnancy must always be excluded. -
Orphanet Report Series Rare Diseases Collection
Marche des Maladies Rares – Alliance Maladies Rares Orphanet Report Series Rare Diseases collection DecemberOctober 2013 2009 List of rare diseases and synonyms Listed in alphabetical order www.orpha.net 20102206 Rare diseases listed in alphabetical order ORPHA ORPHA ORPHA Disease name Disease name Disease name Number Number Number 289157 1-alpha-hydroxylase deficiency 309127 3-hydroxyacyl-CoA dehydrogenase 228384 5q14.3 microdeletion syndrome deficiency 293948 1p21.3 microdeletion syndrome 314655 5q31.3 microdeletion syndrome 939 3-hydroxyisobutyric aciduria 1606 1p36 deletion syndrome 228415 5q35 microduplication syndrome 2616 3M syndrome 250989 1q21.1 microdeletion syndrome 96125 6p subtelomeric deletion syndrome 2616 3-M syndrome 250994 1q21.1 microduplication syndrome 251046 6p22 microdeletion syndrome 293843 3MC syndrome 250999 1q41q42 microdeletion syndrome 96125 6p25 microdeletion syndrome 6 3-methylcrotonylglycinuria 250999 1q41-q42 microdeletion syndrome 99135 6-phosphogluconate dehydrogenase 67046 3-methylglutaconic aciduria type 1 deficiency 238769 1q44 microdeletion syndrome 111 3-methylglutaconic aciduria type 2 13 6-pyruvoyl-tetrahydropterin synthase 976 2,8 dihydroxyadenine urolithiasis deficiency 67047 3-methylglutaconic aciduria type 3 869 2A syndrome 75857 6q terminal deletion 67048 3-methylglutaconic aciduria type 4 79154 2-aminoadipic 2-oxoadipic aciduria 171829 6q16 deletion syndrome 66634 3-methylglutaconic aciduria type 5 19 2-hydroxyglutaric acidemia 251056 6q25 microdeletion syndrome 352328 3-methylglutaconic -
Management of Reproductive Tract Anomalies
The Journal of Obstetrics and Gynecology of India (May–June 2017) 67(3):162–167 DOI 10.1007/s13224-017-1001-8 INVITED MINI REVIEW Management of Reproductive Tract Anomalies 1 1 Garima Kachhawa • Alka Kriplani Received: 29 March 2017 / Accepted: 21 April 2017 / Published online: 2 May 2017 Ó Federation of Obstetric & Gynecological Societies of India 2017 About the Author Dr. Garima Kachhawa is a consultant Obstetrician and Gynaecologist in Delhi since over 15 years; at present, she is working as faculty at the premiere institute of India, prestigious All India Institute of Medical Sciences, New Delhi. She has several publications in various national and international journals to her credit. She has been awarded various national awards, including Dr. Siuli Rudra Sinha Prize by FOGSI and AV Gandhi award for best research in endocrinology. Her field of interest is endoscopy and reproductive and adolescent endocrinology. She has served as the Joint Secretary of FOGSI in 2016–2017. Abstract Reproductive tract malformations are rare in problems depend on the anatomic distortions, which may general population but are commonly encountered in range from congenital absence of the vagina to complex women with infertility and recurrent pregnancy loss. defects in the lateral and vertical fusion of the Mu¨llerian Obstructive anomalies present around menarche causing duct system. Identification of symptoms and timely diag- extreme pain and adversely affecting the life of the young nosis are an important key to the management of these women. The clinical signs, symptoms and reproductive defects. Although MRI being gold standard in delineating uterine anatomy, recent advances in imaging technology, specifically 3-dimensional ultrasound, achieve accurate Dr.