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Long-term urologic and gynecologic follow-up in anorectal anomalies: The keys to success
Published in Alejandra Vilanova-Sánchez, Marc A. Levitt, Pediatric Colorectal and Pelvic Reconstructive Surgery, 2020
Geri Hewitt, Daniel G. DaJusta, Christina B. Ching
Preconceptual counseling is particularly important in patients with ARM. Like all women, ideally their health should be optimized and their pregnancy planned. Patients with imperforate anus may have additional VACTERL abnormalities. Müllerian anomalies can impact fertility, risk of preterm labor, and fetal malpresentation. Cardiac anomalies require careful management with cardiology, particularly at the time of delivery, and require fetal echocardiography. Renal function may worsen and if impaired at baseline may increase the risk of preeclampsia. Spinal abnormalities may limit the utility of regional anesthesia.
Müllerian Anomalies
Published in Juan Luis Alcázar, María Ángela Pascual, Stefano Guerriero, Ultrasound of Pelvic Pain in the Non-Pregnant Female, 2019
Betlem Graupera, Jean L. Browne
Müllerian anomalies are a heterogeneous group of congenital anomalies caused by isolated or complex alterations happening at different stages of embryological development, whether in the formation, channeling, fusion, or absorption of the Müllerian ducts. They can occur as single defects or as a combination of defects, the latter resulting in so-called complex anomalies.1–3
Preterm labour
Published in Louise C Kenny, Jenny E Myers, Obstetrics, 2017
Congenital müllerian anomalies are often unrecognized but are estimated to occur in up to 4% of women of reproductive age. They occur as a consequence of abnormal embryologic fusion and canalization of the müllerian ducts and result in an abnormally formed uterine cavity, which can range from an arcuate uterus, which results in minimal fundal cavity indentation, to complete failure of fusion resulting in uterine didelphys (Figure 8.9). They are associated with adverse pregnancy outcome in up to 25% of women, including first and second trimester miscarriage, PPROM, preterm birth, FGR, breech presentation and caesarean section.
OHVIRA syndrome presenting with acute abdomen findings treated with minimally invasive method: three case reports
Published in Acta Chirurgica Belgica, 2022
Reyhan Gündüz, Elif Ağaçayak, Mehmet Sıddık Evsen
The combination of obstructed hemi-vagina and uterus didelphys was first reported in 1922 [1]. Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital defect of the Müllerian ducts, which are developmental precursors to the fallopian tubes, uterus, cervix, and upper vagina [1]. Because of the obstructed hemi-vaginal and ipsilateral renal agenesis (OHVIRA) which are parts of its characteristics, HWWS is also known as OHVIRA syndrome [1]. Obstructive Müllerian anomalies are estimated to affect approximately 0.1–3.8% of women [2]. Anatomically, HWWS is characterized by three anomalies of the female reproductive system: uterus didelphys, obstructed hemi-vagina, and ipsilateral renal agenesis. The presence of normal external genitalia generally delay the diagnosis until after menarche [3]. It is most commonly diagnosed at puberty due to pelvic and abdominal pain, worsening dysmenorrhoea, pelvic mass, and the ipsilateral renal agenesis. More rarely, it can be found in neonates or adults with primary infertility, pyometra, urinary obstruction, and ischiorectal swelling [4]. The diagnosis is made with history and physical examination together with appropriate imaging tests; diagnostic laparoscopy often is not required [5]. Early diagnosis and surgical intervention are important to relieve symptoms, prevent complications and preserve future fertility in OHVIRA syndrome [6].
Functioning left uterine horn with cervico-vaginal atresia and ovarian maldescent – an unclassified Müllerian anomaly treated with horn-vaginal anastomosis
Published in Journal of Obstetrics and Gynaecology, 2018
Rashmi Bagga, Tanuja Muthyala, Pradeep Kumar Saha, Jasvinder Kalra, Rimpi Singla, Aashima Arora, Tulika Singh
Uterine cervical agenesis is a rare congenital malformation and is classified as class II b variant in the ASRM classification. These girls present with primary amenorrhoea, symptoms of outflow tract obstruction and around half of them have partial or complete vaginal agenesis (Fujimoto et al. 1997). History, loco-regional examination, sonography and MRI help to diagnose obstructive Müllerian anomalies and plan surgery. Reconstructive surgery in the form of cervical canalisation has been performed using an abdomino-perineal approach and a neo-ostium is created by drilling through the atretic cervix to establish a communication between the endometrial cavity and the vagina. Its patency is maintained by stents with or without an overlying skin graft, but fibrotic obliteration occurs in 40–60%. Post-operative adverse effects such as fibrosis of the tract, infection and sepsis have led to recommend hysterectomy for this anamoly (Fujimoto et al. 1997).
Evaluation of the correlation between insulin like factor 3, polycystic ovary syndrome, and ovarian maldescent
Published in Gynecological Endocrinology, 2018
The embryologic scenario explaining maldescent of the ovary is still not clear, but it had been suggested to be due to the complete or partial failure of the normal caudal embryogenic descent or impeded growth of a segment of the urogenital ridge, which would give rise to the ovary later on, or both mechanisms were proposed as the underlying causes. Moreover, Mullerian anomalies are typically thought to be a multifactorial process leading to one or more embryological insult at 6–9 weeks. A similar process might occur at 12 weeks leading to ovarian maldescent [7,8].