The incidence of intracranial haemorrhage (ICH) in newborns with haemophilia is unknown. Retrospective studies, estimate the incidence to be around 3%. Because of this uncertainty, we analysed the largest inpatient database in the USA, the Nationwide Inpatient Sample (NIS), to better approximate the incidence of ICH in these patients. ICD-9 coding data were used to reference NIS entries of haemophilia (A, B or C) or von Willebrand's disease (VWD), with intraventricular (IVH), subarachnoid (SAH), subdural (SDH) and/or intraparenchymal (IPH) haemorrhage. Of 9.2 x 10(7) hospitalizations from 1988 to 2001, 11% or 1 x 10(7) were newborns. Of these, 0.00527%, or 580 were diagnosed with haemophilia or VWD. Twenty of 580, or 3.4%, experienced an ICH. The ICH rate in non-haemophilic newborns was 0.11% (P value: <0.0001). The rate of ICH among term haemophilic newborns without sepsis, respiratory distress syndrome (RDS) or congenital heart disease (CHD), delivered without vacuum assist was 1.9%. One death occurred on the day of birth in a term neonate with haemophilia C. The mean length of stay for ICH patients with haemophilia was 28 days (median 28, range: 6-143 days). The mean hospital charges for the group were 102,072 dollars (median 67,551 dollars, range: 9624-467,132 dollars). These data add credence to the estimates of ICH in haemophilic newborns and may guide treatment strategies around the time of their birth. Further, uncomplicated delivery of term, otherwise healthy haemophilic newborns may carry a lesser risk of ICH.
Cervical dysgenesis is a rare cause of obstructive amenorrhoea. We are presenting a case of a 15-year-old girl presented with primary amenorrhoea diagnosed with cervical dysgenesis with left ectopic kidney and endometriosis. The patient underwent laparoscopy followed by exploratory laparotomy with total abdominal hysterectomy with left salpingectomy followed by neovaginal creation and right ureteric D-J stenting and vaginal mould insertion. Cervical agenesis treatment should aim at the re-establishment of patency of the outflow tract. A fistulous tract with stent placement has been done in some cases but eventually, hysterectomy is required in view of recurrent and severe pelvic infection is a common problem. Ovarian function can be preserved if a hysterectomy is performed timely.
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