Mayer-Rokitansky-Kuster-Hauser Syndrome diagnosed by Magnetic Resonance Imaging. Role of Imaging to identify and evaluate the uncommon variation in development of the female genital tract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a spectrum of Müllerian duct anomalies characterized by congenital aplasia of the uterus and of the upper part (2/3) of the vagina, in young women presenting otherwise with normal endocrine status. The ovaries and fallopian tubes are present. It is one of the most common causes of primary amenorrhea and affects at least 1 out of 4500 women. Its penetrance varies, as does the involvement of other organ systems and itcan be isolated (type I) or associated with other malformations (type II). The MRKH syndrome usually remains undetected until the patient presents with primary amenorrhea despite normal development of secondary sexual characteristics, so imaging evaluation can demonstrate in one setting, non invasively, the anomalies in development of genital tract. We report a case of MRKH syndrome in a 16-year-old woman who presented with primary amenorrhea, stressing the role and benefit of imaging in the differential diagnosis

Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study.

The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients. 70 patients newly diagnosed with IPF were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 patients was used for confirmation. Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS > 3, 6-min walking distance ≤ 72% predicted and composite physiologic index > 41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low diffusing coefficient of the lung for carbon monoxide. Results were confirmed in an independent cohort of patients. In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression

Chronic obstructive pulmonary disease (COPD) patients with osteoporotic vertebral compression fractures (OVCFs): improvement of pulmonary function after percutaneous vertebroplasty (VTP)

Objectives: To investigate the changes of respiratory function in patients affected by chronic obstructive pulmonary disease (COPD) with single dorsal osteoporotic vertebral compression fractures (OVCFs) treated with vertebroplasty (VTP). Methods: Forty-five patients affected by COPD and single dorsal OVCF underwent VTP (29 men, 16 women; mean age 71.4 years, range 65-77 years). Inclusion criteria were magnetic resonance findings of bone marrow oedema, without intracanal bone fragments and refractory pain to medical treatment for at least 3 months. Osteoporosis was assessed by bone densitometry. Spirometry was performed before and after treatment. Results: A significant VAS-score decrease was observed 1 week after VTP, with a subsequent decrease over time; vital capacity (VC) and forced vital capacity (FVC) improved over time, reaching a plateau at 3 months. Forced expiratory volume at 1 s (FEV1) did not significantly differ between the pre-VTP values and follow-up values. A significant correlation was observed between VAS-score values and VC, and VAS-score values and FVC. No significant correlation was observed between VAS-score values and FEV1 values. Conclusions: VTP improves restrictive ventilatory impairment in patients with moderate and severe COPD affected by single thoracic OVCFs. We recommend this treatment in the management of these patients. Key Points: • Osteoporosis is a major comorbidity in chronic obstructive pulmonary disease (COPD) patients. • Pain due to osteoporotic vertebral compression fractures worsens respiratory failure in COPD. • Vertebroplasty improves ventilatory impairment in COPD patients with osteoporotic vertebral compression fractures. © 2014 European Society of Radiology