Creation and maintenance of neo-vagina with the use of vaginal dilators as first line treatment: Results from a quaternary paediatric and adolescent gynaecology service in Australia.

BACKGROUND: Vaginal agenesis is an uncommon condition with an estimated incidence of 1/5000 live female births. There are surgical and non-surgical treatment options for neo-vagina creation. Vaginal dilatation has been used to treat vaginal agenesis for over 80 years, but outcome data to date have had poor definitions for 'success'. The optimal method for neo-vagina creation varies with vaginal dilator therapy being the most successful first line therapy in women with Mayer-Rokistansky-Kuster-Hauser syndrome, with little literature to guide best practice in women with more complex congenital conditions. There is a paucity of data from Australian and New Zealand paediatric and adolescent gynaecology services on management of women requiring a functional vagina, for any underlying aetiology. AIMS: To determine if creation and maintenance of a functional neo-vagina for vaginal agenesis with non-surgical management (via vaginal dilators) should be the first line treatment. MATERIALS AND METHODS: Retrospective observational study between January 2005 and June 2015. RESULTS: Of the 23 women, 21 (91.3%) achieved a successful vaginal length defined as achieving vaginal length >6 cm and maximum width using the largest dilator if not sexually active, or ability to have sexual intercourse without discomfort. CONCLUSION: Vaginal dilator therapy remains an effective first line treatment for neo-vagina creation and maintenance and can be achieved in an outpatient setting with appropriate support.

Rotational thromboelastometry (ROTEM® ) in gestational diabetes mellitus and coagulation in healthy term pregnancy: A prospective observational study in Australia.

BACKGROUND: Rotational thromboelastometry (ROTEM® ) is a point-of-care test of coagulation. ROTEM® -defined hypercoagulability has been identified in pregnant women and in non-pregnant patients with diabetes mellitus. Pregnancy is known to be a hypercoagulable state, but the influence of gestational diabetes mellitus (GDM) on coagulation is unknown. AIM: The aim of this study was to assess the combined effect of pregnancy and GDM on coagulation using ROTEM® and to compare this to healthy pregnant women presenting for elective caesarean delivery. MATERIALS AND METHODS: Ethics approval was granted for recruitment of women presenting for elective caesarean delivery. Women with pre-existing conditions affecting coagulation were excluded. Group N included health pregnant women at term and Group G included pregnant women at term with GDM. Data regarding GDM management and glycaemic control were collected. Poor glycaemic control was defined by markers of accelerated fetal growth and elevated fasting or postprandial blood glucose levels. The ROTEM® parameters (extrinsically activated thromboelastometric test (EXTEM) / fibrin polymerisation test (FIBTEM) amplitude at five minutes, coagulation time, maximum clot firmness and clot formation time) were compared between the two groups using Student's t-test. RESULTS: There were 75 women in Group N and 21 women in Group G. Mean age and median body mass index values were comparable for both groups. There were no statistical differences found between the EXTEM and FIBTEM parameters analysed for the two groups. CONCLUSIONS: There was no association between GDM and increased hypercoagulability as demonstrated by ROTEM® parameters in healthy pregnant women presenting for elective caesarean delivery at term.

The influence of obesity on coagulation in healthy term pregnancy as assessed by rotational thromboelastometry.

BACKGROUND: Rotational thromboelastometry (ROTEM® ) is a point-of-care coagulation test which has been used to demonstrate hypercoagulability in pregnant populations and obese populations. AIM: The aim of this study was to assess the combined effect of pregnancy and obesity on coagulation using ROTEM® in healthy pregnant women of varying body mass indices (BMIs) presenting for elective caesarean delivery. MATERIALS AND METHODS: Ethics approval was granted for recruitment of women presenting for elective caesarean delivery. Women with any condition affecting coagulation were excluded. The ROTEM® parameters of extrinsically activated thromboelastometric test / fibrin polymerisation test (EXTEM/FIBTEM) amplitude at five minutes (A5), coagulation time (CT), maximum clot firmness (MCF) and clot formation time (CFT) were compared between three different groups: normal weight, overweight and obese women. RESULTS: One hundred and eighty-five women presenting for elective caesarean delivery met inclusion criteria and were divided into three groups; normal weight (BMI < 25 kg/m2 , n = 86), overweight (BMI 25-29.9 kg/m2 , n = 54) and obese (BMI ≥ 30 kg/m2 , n = 45). They had a mean (SD) age of 32.7 ± 5.0 years and the median (interquartile range) BMI of 21.9 kg/m2 (20.5-23.0), 27.0 kg/m2 (26.0-28.5), 36.0 kg/m2 (32.2-41.8) for the normal weight, overweight and obese groups respectively. Forty-one (22.2%) women were nulliparous. Across the three groups for FIBTEM A5 (P = 0.018), FIBTEM MCF (P = 0.032), FIBTEM CFT (P = 0.047) and EXTEM MCF (P = 0.015) there was evidence of increasing coagulability with increasing BMI. However, following Bonferroni correction, this was no longer significant. CONCLUSIONS: There is no association between BMI and ROTEM® parameters in pregnant women presenting for elective caesarean delivery at term.

Dinoprostone vaginal pessary for induction of labour: safety of use for up to 24 h.

BACKGROUND: Cervidil(®) (dinoprostone) intravaginal pessaries are used for induction of labour and maintain serum prostaglandin levels for up to 24 h. The Therapeutic Goods Administration approves Cervidil(®) for 12-h use. However, twenty-four-hour use of Cervidil(®) is supported in Europe, New Zealand, America and some Australian hospitals. AIM: To assess the safety of Cervidil(®) use for up to 24 h for induction of labour in nulliparous women. METHODS: A retrospective cohort study of 269 consecutive women receiving Cervidil(®) at the Royal Brisbane and Women's Hospital (RBWH) between July 2007 and December 2008 was performed. The primary outcome measures were frequency of, and time to, uterine tachysystole with or without fetal heart rate (FHR) changes. Secondary outcome measures included frequency of maternal (intrapartum temperature, postpartum haemorrhage) and neonatal (low Apgars, resuscitation, nursery admission) morbidity. Morbidity outcomes of those who received Cervidil(®) for less than or equal to 12 h were compared with those who received Cervidil(®) for more than 12 h. RESULTS: Uterine tachysystole occurred in 9.3% of patients receiving Cervidil(®) , with a mean time to tachysystole of 10 h. The majority of cases (68%) occurred within 12 h of use. There was no increase in maternal or neonatal morbidity for those who received Cervidil(®) for longer than 12 h. CONCLUSION: Twenty-four-hour use of Cervidil(®) is likely as safe as 12-h use for induction of labour in nulliparous women.

Transition to Parenthood: Antenatal Education Promotes Perinatal Mental Health When Collaboratively Delivered by Midwives, Mental Health Peer Workers and Perinatal and Infant Mental Health Professionals.

Transition to Parenthood is a two-session (antenatal and postnatal) module for inclusion in a birth and parenting education course, designed to proactively support perinatal and infant mental health. In this pilot study, 299 mothers and 241 fathers/partners participated in the whole module, with 35 mothers completing pre- and post-program measures of depression, anxiety, stress, and parenting confidence. Statistically significant improvements were found on all four measures with high effect sizes. Participant ratings of learning and satisfaction were high and persisted over time. These results provide support for the usefulness of group-based birth and parenting education that focuses on perinatal and infant mental health, with mental health peer workers co-delivering the program.

Large loop excision of transformation zone procedures used in the management of cytological abnormalities of the cervix.

BACKGROUND: National Health and Medical Research Council (NHMRC) guidelines published in 2005 changed the management of cervical squamous intraepithelial lesions. Asymptomatic low-grade abnormalities may be treated conservatively as many regress spontaneously. Cervical changes reflect infection with the human papilloma virus (HPV). Risk factors for acquiring HPV are the same as those for other sexually transmitted infections (STIs). AIMS: To perform a comparison of the Papanicolaou (PAP) smear, histology results of the cervical biopsy and large loop excision of transformation zone (LLETZ) to determine what proportion were over treated, and conversely what proportion would have been under treated in relation to the 2005 NHMRC guidelines. This group of patients was reviewed also for history of STI or concurrent infection. METHODS: Retrospective chart audit of LLETZ procedures and related histology in a population of women under 25 years between 1999 and 2003 presenting to a colposcopy clinic at a regional hospital. RESULTS: Two hundred and fifty-one asymptomatic women underwent LLETZ procedures. Of them, 27.6% reported a history of STI and 9.2% had a positive test result or history of Chlamydia. Based on the 2005 NHMRC guidelines, which require high-grade squamous intraepithelial lesions (HSIL) on PAP or biopsy, 34% of women had no indication for LLETZ. Of these, 65% were over treated having no HSIL on LLETZ histology and 35% would have been under treated. CONCLUSIONS: The 2005 NHMRC guidelines should result in 33.9% fewer LLETZ procedures being performed in the younger age group. This has benefits for their future fertility needs. STI screening would be beneficial in this group when seen at colposcopy clinics, as they have significant rates of prior or current infection.

Idiopathic hypogonadotropic hypogonadism: a rare cause of primary amenorrhoea in adolescence-a review and update on diagnosis, management and advances in genetic understanding.

Idiopathic hypogonadotropic hypogonadism (IHH) refers to a family of genetic disorders that affect the production and/or action of gonadotropic-releasing hormone, resulting in reduced serum levels of sex steroids. This condition has a prevalence of 1-10 cases/100 000 births and is characterised by the absence of spontaneous pubertal development. In women, the condition is characterised by the onset of normal adrenarche, with the absence of thelarche and menarche. Pubertal induction for breast development and uterine growth with oestradiol, and sequential maintenance of a normal menstrual cycle and adequate oestrogen for bone health, with an oestrogen and progesterone, is considered first-line treatment. Pregnancy can be achieved in patients who have received and responded to treatment with ovulation induction with exogenous gonadotrophins. Advances in genetic testing have led to increased research and understanding of the underlying genetics of IHH with gene mutations described in up to 50% of all IHH cases.

Hyperandrogenism, insulin resistance and acanthosis nigricans (HAIR-AN syndrome): an extreme subphenotype of polycystic ovary syndrome.

HAIR-AN-a syndrome of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN)-is a specific subphenotype of polycystic ovary syndrome (PCOS), and it is seen in almost 5% of all women with hyperandrogenism. An adolescent girl aged 11 years old was referred with adrenarche, hyperandrogenism and obesity commencing at age 8. Clinical and biochemical investigations confirmed significant hyperandrogenism and insulin resistance, and a diagnosis of HAIR-AN syndrome was made after exclusion of other differential diagnoses. HAIR-AN syndrome is an important diagnosis for the adolescent gynaecologist to be aware of, and it requires a multidisciplinary approach, including endocrinology input, for optimal management. Weight loss, lifestyle modification and combined hormonal pill and metformin are considered first-line treatment.

The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad.

The triad of obstructed hemivagina, renal anomaly and uterus didelphys is an uncommon cause of abdominal pain and menstrual discomfort in adolescent girls. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Adolescent girls presenting with these symptoms should have a baseline pelvic ultrasound scan to establish uterine anatomy. In those with ultrasound findings of Müllerian anomalies appropriate follow up would include a magnetic resonance imaging, preferably at a tertiary centre with expertise in interpretation of Müllerian anatomy, as well as early consultation and referral to a centre with experience in the management of these rare conditions. Appropriate surgery would be a single stage procedure to either excise or completely divide the obstructing septum. There is currently no consensus on concurrent laparoscopy. We present a case series of four patients with the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis managed between 2005-2009 at a tertiary centre for paediatric and adolescent gynaecology.

Bleeding Disorders in Adolescents with Heavy Menstrual Bleeding: The Queensland Statewide Paediatric and Adolescent Gynaecology Service.

STUDY OBJECTIVE: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: The study was a retrospective review of 124 female adolescents aged 8 to 18 years with HMB who presented to the Queensland Paediatric and Adolescent Gynaecology Service, Brisbane, Australia. The primary outcome measure was diagnosis of a bleeding disorder, with secondary outcomes including iron deficiency and/or anemia and treatment modalities. RESULTS: Screening for bleeding disorders was performed in 77/124 (62.1%) of patients with HMB. Twenty-seven adolescents were diagnosed with a bleeding disorder, giving a prevalence of 27/124 (21.7%) in those with HMB, and 27/77 (35%) with HMB who were screened. Of these 35%, von Willebrand disease was the most common bleeding disorder, found in 14/27 (51.6%), followed by inherited platelet function disorders diagnosed in 9/27 (33.3%), thrombocytopenia (inherited or acquired) in 3/27 (11.1%), and Factor IX deficiency in 1/27 (3.7%). Iron deficiency and/or anemia was diagnosed in 53/107 (49.5%) of patients with HMB who were screened for this, and 19/27 (70.3%) of those diagnosed with a bleeding disorder. CONCLUSION: Adolescents with HMB who present to a tertiary pediatric and adolescent gynecology service should be screened for bleeding disorders, because of the considerably high prevalence in this at-risk population.

Successful Resuscitation of Maternal Cardiac Arrest With Disseminated Intravascular Coagulation Guided by Rotational Thromboelastometry and Transesophageal Echocardiography: A Case Report.

We present a case of maternal cardiac arrest during an elective cesarean delivery. Transesophageal echocardiography identified a large pulmonary artery mass, and guided resuscitation efforts. After return of spontaneous circulation, the patient developed disseminated intravascular coagulation with massive hemorrhage. Blood product selection and volume replacement were guided by rotational thromboelastometry and transesophageal echocardiography, respectively. Correction of coagulopathy was observed clinically and confirmed by rotational thromboelastometry. The patient fully recovered without neurological deficit.

Partial Cervical Agenesis and Complete Vaginal Atresia.

BACKGROUND: The objective of this study was to report 2 cases of the combined congenital anomalies of complete vaginal atresia and partial cervical agenesis, and highlight the limitations of magnetic resonance imaging for definitive initial diagnosis, and consequently the importance of early definitive management, to avoid life-threatening sepsis. Herein we provide a retrospective case audit of two patients with congenital abnormalities between 2005 and 2013 who were treated in a quaternary statewide pediatric and adolescent gynecology center. CASES: Two patients with the combined congenital anomalies of complete vaginal agenesis and partial cervical agenesis highlight the difficulties encountered with the limitations of magnetic resonance imaging in accuracy of diagnosis, as well as development of life-threatening sepsis that requires hysterectomy. Both patients were initially imaged as having distended endometrial cavities and cervical canals with what was thought to be an obstructive upper vaginal septum and absent lower vagina. Both required initial neovagina creation, however the cervices were never clinically or surgically visualized. SUMMARY AND CONCLUSION: Partial cervical agenesis is a relatively rare form of Müllerian abnormality which, if not diagnosed and definitively treated early, can have significant morbidity and mortality. Although magnetic resonance imaging is the diagnostic imaging gold standard for Müllerian abnormalities, it is important to recognize the limitations of this modality, the potential sequelae of these limitations, and to appreciate the importance of early accurate diagnosis and treatment of this condition. Importantly, if the imaging diagnosis does not completely correlate with the clinical and surgical findings, then a high suspicion of complete or partial cervical agenesis is prudent, because the consequences of nondefinitive early treatment can be life-threatening and potentially fatal.

Incidental gonadal tumors at the time of gonadectomy in women with Swyer syndrome: a case series.

BACKGROUND: Swyer syndrome (46XY complete gonadal dysgenesis) is an uncommonly encountered condition in our population. Gonadectomy is recommended upon diagnosis due to a significant risk of malignant transformation of the dysgenetic gonads, typically to dysgerminoma. CASES: We present 3 cases of women who underwent gonadectomy following a diagnosis of Swyer syndrome. Two of these patients had dysgerminoma confirmed on histopathology. In particular we discuss the macroscopic appearance of the affected gonads and the further management of each case. SUMMARY AND CONCLUSION: Individuals with Swyer syndrome require gonadectomy upon diagnosis of their condition, as part of their multidisciplinary management. For treatment of early stage dysgerminoma, surgical resection of the involved gonad and fallopian tube is curative, again highlighting the need for early intervention.

Hereditary angioedema with an estrogen trigger in a 12-year-old.

BACKGROUND: Hereditary angioedema (HAE) is rare autosomal dominant genetic disorder, commonly affecting girls around the menarche, which manifests clinically as recurrent episodes of angioedema. Laryngeal edema can lead to asphyxiation and death. Traditionally hormones have been avoided in the management due a reported association with flares in the literature. This case describes an alternative management with a progestin. CASE: A 12 year old HAE sufferer failed to receive relief from her symptoms of angioedema with standard treatment. A trial of depot medroxyprogesterone acetate has resulted in resolution of her symptoms for the last 14 months. CONCLUSION: Although estrogens and progestins have been avoided in the management of HAE in the past, the reasons for this are based only on a small number of case reports. In this case, successful treatment with depot medroxyprogesterone acetate indicates that progestins, as an alternative management for estrogen-triggered HAE, certainly warrants further research.

Curved versus straight scissors to avoid 3rd and 4th degree perineal tears: a randomised feasibility study.

BACKGROUND: Severe perineal tears sustained during childbirth cause significant distress and morbidity amongst women. The objective of this study was to compare the use of straight scissors for cutting an episiotomy with the use of curved scissors, which are designed to curve away from the anal sphincter. METHODS: We used a single-centre, randomised feasibility trial. The intervention was the use of curved scissors. Women were recruited during a prenatal visit and randomised in the delivery suite, when it became clear that an episiotomy was required. The feasibility outcomes were the proportion of women able to be recruited, randomised and followed up. We also calculated the incidence of obstetric anal sphincter injury when either straight or curved scissors were used to cut an episiotomy. Other outcomes assessed were pain, length of hospital stay, perineal infection and perineal dehiscence. RESULTS: Of the 155 patients recruited in the prenatal period, only 20 (12.9%) were eventually randomised at birth. The main reasons for the high loss were that women either did not have a vaginal delivery (38, 24.5%), or they did not need an episiotomy (72, 46.5%). Rates of obstetric anal sphincter injury and other outcomes were similar between groups. DISCUSSION: Anal sphincter injury during childbirth remains an important problem. Although the use of curved scissors provides a theoretical solution, we found that the high attrition rate made feasibility of conducting a suitably powered, randomised trial using the current design untenable. Alternative strategies have been suggested to make any future study more viable.