| Main clinical features
|
characterized by inverted facial musculature and congenital obstructive uropathy
hydronephrosis, hydroureter and a peculiar facial expression, mainly when smiling or crying
distorted face, dysfunctional voiding, such as repeated episodes of urinary tract infection, dysuria or incontinence, frequency, urgency, or enuresis, is the life-threatening component of the syndrom; gradually progresses to severe upper tract damage and the development of bladder trabeculation, vesicoureteral reflux (VUR), hydroureternephrosis, and subsequent renal failure (Pang 2010) |