Congenital rotated uterus

  Rotated uterus is extremely rare, and two cases are reported in this summary.  Case 1: 15 years old, with progressive worsening of dysmenorrhea for 10 months after menarche, admitted to the hospital on December 15, 2010. The patient had her first menstrual period 10 months prior to admission, and her menstruation cleared once a month for 4 to 5 days each time thereafter, with progressive worsening of dysmenorrhea. Her last menstrual period was on December 1, 2010, and she was referred to our hospital with unclear diagnosis because she had a week-long period and abdominal pain. On examination: the vulva was well developed, a cystic mass of about 6cm×5cm×5cm could be palpated in the right anterior rectum on anal examination, which could be pushed, with obvious tenderness, the lower end of the mass was about 5cm from the hymen, and the uterus and adnexa could not be palpated. Pelvic ultrasound: double uterus, right uterine body 4,5cm×2,2cm, uterine cavity separation of about 1,8cm, a non-echoic area of about 7,5cm×5,0cm was detected below the uterus, dotted weak echogenicity was seen inside, which was connected to the uterine cavity; left uterine body 3,9cm×2,0cm, fresh endometrial line; left ovary size 2,7cm×1,2cm, right ovary 2,7cm×1,7cm. 7cm, the left fallopian tube was about 1,6cm wide with poor internal translucency, no obvious abnormal echogenicity was seen in the right adnexal area; the right kidney was absent and the left kidney was compensatory enlarged. After completing laboratory tests, a vaginal exploration was performed under lumbar anesthesia: a 7cm×5cm×5cm cystic mass was palpated in front of the right side of the vagina at a depth of about 5cm, protruding and blocking the vagina, with a smooth surface and no rupture, and old blood was drawn by puncturing the cystic cavity. The luminal surface was flattened with a complex epithelium, which was confirmed to be vaginal septum tissue. After excision of most of the oblique vaginal septum and drainage of the posterior septal cavity, the left uterine cervix was smaller on the left posterior side of the vaginal apex, while the right uterine cervix was slightly larger and located on the right anterior superior side, with an oblique septum between the two uterine cervixes. On postoperative review of pelvic ultrasound: scanning down the two uterine bodies, the confluence showed dilatation, with the left uterine cervix being about 2 or 3 cm long and the right uterine cervix being about 1 or 9 cm long. The patient had no further dysmenorrhea at postoperative follow-up.  Case 2: The patient was 30 years old, pregnant, with regular menstruation and dysmenorrhea. She was diagnosed with “longitudinal uterine septum” by ultrasound for 7 years and had normal sexual intercourse without contraception for 2 years without pregnancy. She was in good health. Ultrasound: the uterus was anteriorly positioned, slightly large, with a wide transverse diameter, the uterine cavity was separated by a hypoechoic zone of cords, the endometrium was 0.3 cm thick, the uterine wall was uniformly echogenic, the ovaries were of normal size bilaterally, and there was no special echogenicity, suggesting a completely longitudinal uterus. One day before surgery, one dilating rod (5mm diameter) was inserted into the cervical canal, and laparoscopic and ultrasound-monitored hysteroscopic electrosurgery was performed under general anesthesia. Laparoscopically, the anterior and posterior diameters of the uterus were slightly smaller, with a saddle-shaped depression in the middle of the uterine fundus, and the left uterine angle was larger than the right and had a spherical protrusion. The uterine cavity was probed to a depth of 7.5 cm. hysteroscopically, the cervical canal was divided into two anterior and posterior chambers by a septum 2.5 cm from the external cervical opening. After removal of the “septum” about 1.5 cm, it was found that the septum shifted from coronal to sagittal position, and the sagittal septum divided the uterine body into two halves, and the coronal and sagittal septum were connected by an oblique septum. This uterine septum resembles an “S” shape. The longitudinal septum was continued to be removed until the cut site was about 1.0 cm from the plasma membrane of the uterine fundus, and the longitudinal septum of the uterine cavity was basically removed under ultrasound. After the operation, one intrauterine device was placed in the uterine cavity. The excised longitudinal tissue was sent for pathological examination: the septum was a patch of smooth muscle tissue and part of the surface was covered with endometrium.  Discussion: 1. Diagnostic problems: Case 1 was characterized by double uterine bodies, double uterine cervix, right vaginal nonporous oblique septum combined with posterior septal cavity hemorrhage and ipsilateral renal agenesis, accompanied by clockwise rotation of the cervix and anterior-posterior misalignment of the cervix, diagnosed as congenital nonporous vaginal oblique septum combined with rotational uterine malformation, which is a special type of vaginal oblique septum; Case 2 was characterized by single cervix, double uterine cavity, counterclockwise rotation of the cervix However, according to the method of distinguishing longitudinal uterus from double uterus or double-horned uterus introduced by Lei Zhenwu, it still belongs to the category of single uterine base, i.e. longitudinal uterus, and is diagnosed as complete longitudinal uterus combined with rotational uterine malformation, which is a special type of longitudinal uterus. Since these patients are associated with anterior and posterior misalignment of the cervix or cervical canal, they are collectively classified as rotational uterus. Up to now, it has been found that rotational uterus occurs in double uterus and completely longitudinal uterus with two cervical rotations and anterior-posterior misalignment or with sagittal longitudinal uterine body and coronal longitudinal uterine cervix, there are three types as follows: ① completely undivided double uterus combined with uterine rotation: single uterine fundus, double uterine cavity, double uterine cervix with anterior-posterior misalignment of uterine cervix; ② completely separated double uterus combined with uterine rotation. Double uterine body, double uterine cavity, double uterine cervix, with anterior-posterior misalignment of the cervix; ③ completely longitudinal uterus combined with uterine rotation: single uterine fundus, double uterine cavity, single uterine cervix, with two cervical canal cavities and anterior-posterior misalignment.  2. Occurrence of disease: the position of the internal organs is in a mirror image type with left-right symmetry, and the formation process is called internal inversion. In other words, the initial occurrence of the internal organs is opposite to the normal direction, resulting in the final positioning of each organ on the opposite side of the initial position. Visceral retroposition can occur only in the thoracic cavity, the abdominal cavity, or in a single organ. During the sexually differentiated intersexual period at embryonic week 6, the mesonephric and paramedian ducts of all embryos coexist for a short time and are in an undifferentiated, primitive state. At this time, the foregut, midgut and hindgut are also developed and formed. At nearly 8 weeks of embryonic life, the midgut grows rapidly, protrudes into the umbilical cavity, and rotates counterclockwise on the axis of the superior mesenteric artery, completing a 270° rotation by 20 weeks; at the same time, the foregut (stomach) also rotates 90° clockwise along its long axis because the enlarged liver in the ventral mesentery (lesser omentum) moves to the left and the dorsal mesentery (greater omentum) protrudes to the left; after 8 weeks of embryonic life, the mesonephros begins to disappear and the mesonephric duct After 8 weeks of embryonic life, the mesonephros begins to disappear, the mesonephric duct degenerates, the hilum at the caudal end of the mesoderm in the pelvis faces anteriorly, and the smooth, convex posteriorly facing posterior kidney begins to rotate up to the posterior abdominal wall, rotating 90°, so that the vessels at the renal pelvis and hilum face medially. At 6 weeks of embryonic age, the caudal end of the paramedian tubule emerges anterior to the lateral mesonephric duct and is closely adjacent to it. At 6-7 weeks of embryonic age, the caudal end of the mesonephric duct has descended to the genitourinary sinus, and after 8 weeks of embryonic age, the caudal ends of the paramedian duct on both sides are connected to the genitourinary sinus. During the descent and fusion and resorption of the fused part of the paramedian ducts at 6-9 weeks of embryonic life, it may be affected by the rotation of the foregut, midgut, and posterior kidney, resulting in abnormal rotation and anterior-posterior misalignment if affected by adverse factors. In such patients, the septum is not absorbed after fusion of the bilateral paramedian tubes. The abnormal rotation may be a factor in the incomplete resorption of the uterine septum. The rotation of the fused part of the paramedian ducts may be clockwise or counterclockwise, depending on the time frame of involvement and the impact, and may manifest clinically as a clockwise or counterclockwise rotation of both uterine cervical lumens. These patients may also have a combination of a renal agenesis and a vaginal oblique septum. Whether or not there is inversion of other internal organs remains to be observed in the collection of clinical cases.  Treatment: Patients with rotated uterus often do not have any conscious symptoms and are mostly found incidentally during gynecological examination, pelvic ultrasound, abortion, prenatal examination or even delivery. If the vaginal septum is combined, there may be primary dysmenorrhea and blockage of the vagina by the subuterine mass, or a small amount of brown or pus-blood discharge from the vagina during menstruation with a foul odor. In cases of recurrent miscarriage due to complete longitudinal uterus combined with rotated uterus, hysteroscopy under ultrasonic surveillance and indirect resection (preservation of the isthmus and the longitudinal part of the cervical canal) should be performed to remove the longitudinal uterus in order to protect the cervical function from being affected and to reduce intraoperative bleeding. During delivery, the cervical mediastinum is pressed to one side by the descending fetal head, which usually does not increase the incidence of obstructed labor. An 8-gauge Foley catheter can be inserted through the cervical canal on one side, a hysteroscopic tube is placed into the body cavity of the other uterus, and after dilating the uterine cavity with dilating fluid, the longitudinal uterine septum is electrically removed with the electrosurgery scope starting at the internal cervical os until the catheter is visible, followed by upward removal and withdrawal of the catheter.