Pseudo (platelet-type) von Willebrand disease in pregnancy: a case report.

BACKGROUND: Pseudo (platelet-type)-von Willebrand disease is a rare autosomal dominant bleeding disorder caused by an abnormal function of the glycoprotein lb protein; the receptor for von Willebrand factor. This leads to an increased removal of VWF multimers from the circulation as well as platelets and this results in a bleeding diathesis. Worldwide, less than 50 patients are reported with platelet type von Willebrand disease (PT-VWD). CASE PRESENTATION: We describe the management of platelet type von Willebrand disease in pregnancy of a 26 year old Caucasian primigravida. The initial diagnosis was made earlier following a significant haemorrhage post tonsillectomy several years prior to pregnancy. The patient was managed under a multidisciplinary team which included obstetricians, haematologists, anaesthetists and neonatologists. Care plans were made for the ante- natal, intra-partum and post-partum periods in partnership with the patient. The patient's platelet count levels dropped significantly during the antenatal period. This necessitated the active exclusion of other causes of thrombocytopenia in pregnancy. A vaginal delivery was desired and plans were made for induction of labour at 38 weeks of gestation with platelet cover in view of the progressive fall of the platelet count. The patient however went into spontaneous labour on the day of induction. She was transfused two units of platelets before delivery. She had an unassisted vaginal delivery of a healthy baby. The successful antenatal counselling has encouraged the diagnosis of the same condition in her mother and sister. We found this to be a particularly interesting case as well as challenging to manage due to its rarity. Psuedo von Willebrand disease in pregnancy can be confused with a number of other differential diagnoses, such as gestational thrombocutopenia, idiopathatic thrombocytopenia, thrombotic thrombocytopenic purpura and pre-eclampsia; all need consideration during investigations even in a case such as this where the diagnosis of platelet type von Willebrand disease was known before pregnancy. CONCLUSION: Management of pseudo von Willebrand disease in pregnancy involves the co-operation of multidisciplinary teams, regular monitoring of platelet levels and factor VIII and replacement as appropriate. This case report highlights this rare condition and the need to exclude all the other differential diagnoses of thrombocytopenia in pregnant women with thrombocytopenia.

Prospective evaluation of combined local bupivacaine and steroid injections for the management of chronic vaginal and perineal pain.

PURPOSE: Vaginal/perineal pain is common following obstetric trauma or vaginal surgery for prolapse and may have a serious impact on sexual function and quality of life. Local injections of corticosteroids, local anaesthetic and hyaluronidase are treatment options for chronic pain; however, there are no published studies to support their efficacy. The objective of this study was to evaluate prospectively the efficacy of perineal/vaginal injections for chronic localised pain following childbirth or vaginal surgery. METHODS: Consecutive women with chronic vaginal/perineal pain were recruited in this prospective series (audit). Pain severity and sexual function were determined using a visual analogue scale (VAS 0-10) and the abbreviated sexual function questionnaire (ASFQ) respectively. Patients underwent local injections with a combination of 0.5% bupivacaine (10 ml), hydrocortisone (100 mg) and hyaluronidase (1,500 IU). Follow-up was undertaken at four-weekly intervals. Further injections were performed as clinically indicated. RESULTS: Fifty-three women underwent ≥1 injections [mean: 1.86 (range: 1-4)]. Mean interval from index childbirth [43/53 (81%)] or surgical intervention [10/53 (19%)] was 8 months (range 12 weeks-20 years). Twenty-seven women (51%) were sexually active. All reported dyspareunia. Fifteen (28%) women required 1 and 31(59%) two injections. Pre treatment VAS pain scores were 6.1 versus 4.1 after first injection (p = 0.0002, 95% CI 1.01-3.05) and mean ASFQ scores increased from 18.1 to 29.1 (p = 0.01, 95% CI -17.2 to -2.3) 4 weeks post-injection. There were no adverse events or morbidity. 24/27 (89%) sexually active women with dyspareunia resolved and 18/26 (69%) sexually inactive women resumed satisfactory sexual activity 8 weeks post-injection. CONCLUSION: In our series, this treatment was well tolerated and significant improvements in pain scores and sexual function were observed.

Metformin or insulin: logical treatment in women with gestational diabetes in the Middle East, our experience.

OBJECTIVE: The debate still continues about the preferred modality of treatment of gestational diabetes requiring pharmacological treatment. Insulin was previously considered as the gold standard, but the National Institute of Health and Care Excellence now recommend metformin as the first line drug of choice. The pharmacological management of gestational diabetes mellitus in the Middle East with its high risk population has not been widely published. We aim to evaluate the safety and efficacy of using metformin in comparison to insulin, in our group of patients, and to study key associated morbidities. RESULTS: A total of 291 women registered in the clinic during the study period. One hundred and twenty-one (121) were women with gestational diabetes Mellitus requiring medical therapy. Among them, 107 delivered at term. Ninety (84%) women received metformin. Additional insulin was required in 32% of these patients. There was a significant difference in the birth weight of babies in the metformin with insulin group of 207 g (p value 0.04) in favour of metformin. There was no significant difference in maternal or neonatal morbidities between the groups. Metformin was thus found to be a safe, practical and cost effective medication to be offered to our population.

Transvaginal postpartum manual removal of a prolapsed submucous leiomyoma, initially diagnosed hybrid predominantly intramural.

We present a rare case of a large infarcted submucous fibroid removed manually after a normal vaginal delivery. This patient was known to have a large postero-fundal hybrid predominantly intramural uterine fibroid, measuring 11 x 10 cm in diameter and diagnosed 2 years prior to last conception. Thirty-five days postpartum, she presented with severe lower abdominal pain and foul smell per vaginum. Abdominal examination revealed a very tender bulky uterus at 24 weeks size, and vaginal examination revealed a big fleshy smelly mass with friable surface just bulging from a dilated cervix. Examination under anaesthesia revealed a very foul smelly large pedunculated submucous fibroid that was felt through a dilated cervix. It was deliverable vaginally, so the submucous leiomyoma was removed manually, a procedure similar to manual removal of placenta. Histopathology examination confirmed an infarcted massive fibroid.

Word balloon catheter for Bartholin's cyst and abscess as an office procedure: clinical time gained.

BACKGROUND: Around 2% of women develop a Bartholin's cyst or abscess at least once in their life time. The use of Word balloon catheter as an office procedure for the management of Bartholin's cyst and abscess has been well described and indicates high patient acceptance, low short-term recurrence rates and reduced cost. In most of the reported studies, the reduced costs are attributed to savings from equipment cost, operating theatre costs and health personnel costs. An evaluation of the actual clinical time gained with this office procedure has not been reported and hence the rationale for this study. This study was conducted from December 2011 to January 2014 on 35 patients. An initial retrospective clinical audit of 14 cases of marsupialization under general anesthetic between December 2011 and December 2012 was performed. The findings were compared with a subsequent prospective observational service evaluation of 21 consecutive patients between December 2012 and January 2014. RESULTS: Compared to marsupialization under general anesthetic, the mean clinical time gained from admission to insertion of Word balloon catheter as an office procedure is 15 h and 40 min and the mean clinical time gained from admission to discharge is at least 24 h. There were very few minor complications and no major complications in the Word catheter group compared to the marsupialization group. CONCLUSIONS: There is a clinically significant time gained with the use of Word balloon catheter as an office procedure compared to marsupialization under general anesthetic for Bartholin's cyst and abscess. The findings from our study could assist other units that want to adopt this procedure justify the efficiency savings in terms of clinical time gained when a business case is submitted. Further studies are needed to investigate and address the underlying causes for the delays encountered when marsupialization under general anesthetic is chosen by patients.

Undiagnosed rectal tumour--a rare cause of obstructed labour in the second stage.

Colorectal cancer presenting in pregnancy is extremely rare. Here the authors present the case of a 25-year-old woman who was diagnosed with second-stage obstructed labour secondary to a large rectal tumour. Decision for emergency caesarean section was made for labour dystocia. Histology later confirmed villous adenocarcinoma of the rectum. The patient died from metastatic rectal cancer within 2 years of diagnosis.

Safer childbirth: a rights-based approach.

The Millennium Development Goals (MDGs) set very high targets for women's reproductive health through reductions in maternal and infant mortality, among other things. Reductions in maternal mortality and morbidity can be achieved through various different approaches, such as the confidential review of maternal deaths, use of evidence-based treatments and interventions, using a health systems approach, use of information technology, global and regional partnerships, and making pregnancy safer through initiatives that increase the focus on human rights. A combination of these and other approaches can have a synergistic impact on reductions in maternal mortality. This paper highlights some of the current global efforts on safer pregnancy with a focus on reproductive rights. We encourage readers to do more in every corner of the world to advocate for women's reproductive rights and, in this way, we may achieve the MDGs by 2015.