Torsion of a non-gravid leiomyomatous uterus in a patient with myotonic dystrophy complaining of acute urinary retention: anaesthetic management for total abdominal hysterectomy.

Torsion of a pregnant uterus is rare, but torsion of a non-pregnant uterus is extremely rare. Abdominal pain is the major symptom. Other symptoms include vaginal bleeding, urinary tract symptoms and gastro-intestinal manifestations. We present a case of a 37-year-old white nullipara who presented at the emergency room with acute urinary retention. Medical history revealed that the patient carried the disease of myotonic dystrophy, which was diagnosed two years before. Physical examination revealed a tender, distended bladder, which was easily catheterized, draining 900 ml of clear urine. The abdomen was soft with no muscle guarding or rebound tenderness. A palpable large dense mass occupying the cul-de-sac was found during bimanual examination. Abdominal ultrasound examination revealed a large intramural leiomyoma approximately 10 cm in diameter, in the posterior wall of the uterus, which repelled the bladder. In neurological examination the muscular tone and reflexes were reduced in the lower extremities. Myotonic phenomenon was not found. The patient was thought to suffer from myotonic dystrophy and therefore the possibilities for pulmonary and cardiac complications or malignant hyperthermia had to be kept in mind during the anaesthetic management. The patient underwent an exploratory laparotomy and the uterus was found to have undergone a 60 degrees rotation along the corpus and the cervix uteri transition line. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was perfomed. The intra- and postoperative course of the patient was uneventful. In conclusion, in this patient the uterine pathology (large leiomyoma) in combination with the disease of myotonic dystrophy seemed to be the predisposing factors for the torsion of the non-pregnant uterus. Also, the anaesthetic implications for total abdominal hysterectomy in myotonic dystrophy are discussed and the international literature is reviewed.