Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.

Rationale: Conventional positive-pressure ventilation delivers pressure to the airways; in contrast, negative pressure is delivered globally to the chest and abdomen. Objectives: To test the hypothesis that ventilation with negative pressure results in better oxygenation and less injury than with positive pressure. Methods: Anesthetized, surfactant-depleted rabbits were ventilated for 2.5 hours in pairs (positive or negative). Tidal volume was 12 ml · kg−1, normocapnia was maintained by adjusting respiratory rate, and FiO2 was 1.0. Measurements and Main Results: Lung injury was assessed with histologic scoring, perfusion using thermodilution (global perfusion), and injected intravascular microspheres (regional perfusion); and dynamic computed tomography was used to determine inflation patterns. Negative pressure was associated with a higher PaO2, a lower Pa–PetCO2 gradient (despite identical minute ventilation), and less lung injury. Lung perfusion (global and regional) was similar with positive and negative pressure. Positive end-expiratory pressure applied to the airway was more efficiently transmitted to the pleural space than comparable levels of negative end-expiratory pressure applied to the chest wall; however, the oxygenation associated with any level of end-expiratory lung volume was greater when achieved by negative versus positive pressure. Dynamic computed tomography suggested that lung distension achieved with negative pressure is characterized by greater proportions of normally aerated lung (with less atelectasis) during inspiration and at end-expiration. Conclusions: Negative-pressure ventilation results in superior oxygenation that is unrelated to lung perfusion and may be explained by more effective inflation of lung volume during both inspiration and expiration.