A new treatment for Robert’s uterus with adenomyosis: a case report and review of the literature

Abstract

Purpose

To provide a method for the differential diagnosis of Robert’s uterus with adenomyosis, a rare uterine malformation, and determine the best course of treatment.

Methods

A patient who had Robert’s uterus with adenomyosis was admitted to our hospital in December 2022. We analyzed and summarized her case .

Results

Our patient complained of progressively worsening primary dysmenorrhea over the course of 3 years and lower abdominal pain lasting for 2 days. Her carbohydrate antigen 125 (CA125) level was 372.10 U/mL. Examinations conducted by several hospitals indicated that she had a single-horned uterus and a residual horned uterus, and our hospital’s examination identified Robert’s uterus. This malformation was corrected by open abdominal surgery. For the procedure, pelvic adhesions were first isolated, after which the closed uterine cavity and adenomyosis were resected. Subsequently, the left ovarian endometriosis cyst was resected and right tubal ligation was performed. After surgery, three injections of gonadotropin-releasing hormone A (GnRH-A) were administered, which lowered the patient’s CA125 level to 14 U/mL and normalized her condition.

Conclusion

We pioneered a new therapeutic approach for the treatment of Robert’s uterus with adenomyosis. Some valuable references are provided for clinical practice.

Introduction

Robert’s uterus was first reported in 1970 [1]. It is a rare asymmetrical obstruction with malformation of the uterine septa, including primary dysmenorrhea of varying degrees and the features of a single-horn uterus as revealed by hysterosalpingography (HSG) or the absence of one fallopian tube opening by hysteroscopy. Our patient’s urinary system was normal, but during an open cesarean section procedure, the uterus was found to be slightly bicorned, which was consistent with Robert’s uterus [2, 3]. At present, there are very few reported treatment plans for Robert’s uterus combined with adenomyosis. Through this report, we hope to improve clinicians’ understanding of the disease to aid in its early detection and ensure the implementation of an appropriate treatment. Furthermore, this case presents a new approach to the treatment of Robert’s uterus with adenomyosis, which is expected to be helpful to clinicians.

Case presentation

The patient, a 33-year-old woman from Cambodia, was admitted to our hospital on December 6, 2022. Her chief complaint was “progressive aggravation of primary dysmenorrhea for 3 years and lower abdominal pain for 2 days.” Her previous medical history included menarche at age 13, primary dysmenorrhea, and continuing dysmenorrhea, which had gradually become worse over the previous three years. The patient also reported abdominal pain for 3 to 4 days during menstruation that required oral painkillers. Reports from other hospitals all suggested a “single-horn uterus combined with a residual-horn uterus.” In addition, owing to “a pelvic abscess and high fever,” laparoscopic left salpingectomy and lysis of pelvic adhesions were performed at another hospital on September 21, 2022. During the operation, extensive dense adhesions were found in the pelvic cavity. The intestinal tube and uterus were adhesive and wrapped together in such a way that the full uterine shape and pyosalpinx on the left side could not be exposed. After separation and resection of the left fallopian tube, no other treatment was performed. Postoperatively, the patient’s temperature was normal and the abdominal pain had been relieved. However, the patient’s symptoms of dysmenorrhea remained severe. At the inception of her last menstrual period, on November 14, 2022, she experienced gradually worsening lower-left-sided abdominal pain for which no explanation could be found. She therefore came to our department, where a transvaginal ultrasound indicated “a mass in the left lateral wall of the uterus,” pointing to the possibility of double horns or residual horns accompanied by a uterine hematoma. Over the course of her disease, the patient manifested no cold or fever, no abnormal vaginal discharge, no nausea or vomiting, and no weight loss. Her bladder and bowel function remained normal, and she was otherwise in generally good health.

Patient’s medical history: The patient had undergone three cesarean sections. The first was performed in another hospital in July 2006, resulting in the delivery of an immature fetus that did not survive. In 2008, she had a second cesarean section at a different hospital, where she delivered a healthy, full-term baby. The most recent cesarean section took place at our hospital in 2020, resulting in the delivery of another healthy, full-term infant. Her family history did not include any individuals suffering from diseases such as endometriosis or adenomyosis. The physical examination after admission yielded the following information: she had a height of 150 cm, a weight of 60 kg, and a normal body temperature. She exhibited a notable tenderness upon palpation of her left lower abdomen. Gynecologic examination showed some positive signs: pain with cervical lift, a uterus of normal size but that was difficult to move, and sharp pain upon palpation of the left side of the uterus and the left ovary area. Imaging studies were subsequently performed. The first was a two-dimensional transvaginal ultrasound conducted on December 4, 2022, which revealed a mass on the left lateral wall of the uterus, suggesting the possibility of a double or residual horn with possible uterine effusion, possibly suggesting hematosis. A subsequent transvaginal three-dimensional ultrasound (Fig. 1a and b) revealed the uterine body size to be 55 × 48 × 46 mm, with traces of fluid in the right uterine cavity and a mass on the left lateral wall measuring 50 × 44 × 48 mm in size. This was accompanied by a fluid-like echo measuring 28 × 19 × 14 mm inside the uterus, indicative of uterine hemaceloma with a residual horn. In addition, the left mixed structure of the uterus was 40 × 34 × 38 mm, somewhat similar to the ovarian structure found inside. A color ultrasound of the urinary system, which was conducted outside our hospital, showed normal results. Laboratory examinations revealed an increase of 17.05 × 10⁹/L in the white blood cell count, alongside an increase of 29.5 mg/L in the C-reactive protein (CRP) level. Furthermore, the carbohydrate antigen 125 (CA125) level had increased to 372.10 U/mL.

Fig. 1

(a) Three-dimensional ultrasound showing two uterine echoes. (b) Three-dimensional ultrasound showing that the left uterine cavity was not communicating with the vagina

After the third cesarean section, which was performed at our hospital in 2020, physicians noted that the appearance of the uterus was roughly normal, with a slight double-cornered shape, and that the left fallopian tube was twisted and adherent to the left corner. The right ovaries and fallopian tubes appeared normal. Based on this description, after discussing the findings with the radiologist, the patient was finally diagnosed with Robert’s uterine malformation complicated by adenomyosis, in addition to acute pelvic inflammatory disease. After 3 days of treatment with cefoperazone sodium and sulbactam (2.0 g q8h), the patient’s C-reactive protein level and routine blood test results returned to normal. After 7 days of continued consolidation and anti-inflammatory treatment, we performed an open operation on the patient. During the operation, the pelvic adhesions were first isolated, after which the closed uterine cavity and adenomyosis were resected. Finally, the left ovarian endometrial cyst was resected and right tubal ligation was performed (Fig. 2a and b, and 2c). In addition, the bladder was found to be adherent to the anterior wall of the uterus, the intestine was tightly wrapped around and adherent to the posterior and lateral walls of the uterus, and the depression between the uterus and rectum was closed; no obvious abnormalities were observed in the shape of the bottom of the uterus. A cyst with a diameter of about 4 cm was detected in the left ovarian area, and the left myometrium was hard and showed changes due to adenomyosis. The left uterine cavity was then aspirated to determine the location of the uterine cavity on the side of the atresia; upon aspiration, dark red hematosis was observed. The adhesions in the pelvic cavity were subsequently lysed and the adenomyotic lesions along the outer margin of the left uterine cavity were removed. Moreover, the left ovarian cyst was removed, and a coffee-colored liquid was found in the cyst. The right fallopian tube was dilated and tissue was removed for routine pathologic examination. The postoperative diagnosis was as follows: (1) Robert’s uterine malformation complicated by adenomyosis, (2) left ovarian ectopic cyst, (3) pelvic inflammatory disease, (4) intestinal adhesions, (5) cesarean section, and (6) laparoscopic left fallopian tube resection and lysis of pelvic adhesions. Postoperative pathology (Fig. 3a and b) revealed a deformed uterus consistent with Robert’s uterus with adenomyosis; in addition, a left ovarian mass suggestive of an endometrial cyst and chronic salpingitis on the right side were observed.

Fig. 2

(a) The shape of the uterus, as seen in the pelvic cavity, was normal. (b) The uterus as it appeared after the left atresia uterine cavity and focus of adenomyosis had been removed. (c) Excisional adenomyosis and left-sided atresia of the uterine cavity

Fig. 3

(a) Pathology consistent with uterine adenomyosis. (b) Endometrial implantation cyst

The patient was hospitalized for 4 days post-surgery, during which cefoperazone sodium and sulbactam were continued for anti-infective treatment, while Pituitrin was administered intravenously to promote uterine contraction. Additionally, the patient received three injections of gonadotropin-releasing hormone analogues (GnRH-a) to prevent the recurrence of endometriosis.

We followed up with her after the return of her first postoperative menses. The patient had no dysmenorrhea symptoms during menstruation and her CA125 levels had dropped to 14 U/mL. Two-dimensional B-ultrasonography indicated that the uterine body size was 49 × 40 × 42 mm, no obvious uterine structural abnormalities were detected, and no mass was observed in the bilateral appendages (Fig. 4a and b).

Fig. 4

(a) The uterus was 49 × 40 × 42 mm in size and regular in shape, and the endometrial thickness was 6.9 mm. (b) The left ovary was not clearly visualized, and the right ovary was 31 × 19.8 mm in size

Discussion

The intrauterine diaphragm of Robert’s uterus is usually biased to one side of the uterine cavity, which is completely closed by the diaphragm, and the closed uterine cavity is not connected to the vagina or the contralateral uterine cavity [4], There are normal ovaries and fallopian tubes on this side, and this condition often presents clinically with varying degrees of primary dysmenorrhea. Robert’s uterus is often misdiagnosed in the clinic as a residual-horn uterus (type II) with an unconnected single-horn uterus, a bicorn uterus, a complete mediastinal uterus, or as vaginal oblique septal syndrome. Especially for inexperienced clinical and imaging doctors, it is difficult to make a clear diagnosis at the first visit, and the misdiagnosis rate is extremely high.

only one side of the paramesonephric tube of the single-horned uterus may have developed normally to form the single-horned uterus, thereby allowing the ipsilateral ovary to function normally. On the other side, the accessory mesonephric tube may be completely undeveloped or may not have formed a tube, and the undeveloped side of the ovary, as well as the fallopian tube and kidney on that side, will often be absent. These features are commonly combined with the residual-horn uterus, while a normal fallopian tube and ovary can typically be seen on the side of the residual-horn uterus; however, this is often accompanied by malformation of the ipsilateral urinary organ.The residual-horn uterus can be divided into three types: (1) a residual-horn uterus with a uterine cavity that communicates with the single-horn uterus, (2) a residual-horn uterus with a uterine cavity that does not communicate with the residual-horn uterus, and (3) a residual-horn uterus that is a nonuterine entity connected to the single-horn chamber by only a fibrous band. A bicorned uterus can be detected by palpable depression at the base of the uterus, and it can be classified as either a complete bicorned uterus or an incomplete bicorned uterus, based on the degree of incomplete horizontal fusion of the uterine floor. Generally, there are no clinical symptoms of a bicorned uterus and it does not affect fertility. The complete mediastinal uterus refers to the end of the mediastinum reaching or exceeding the inner opening of the cervix, resembling a double cervix. Vaginal oblique septum syndrome is often accompanied by developmental abnormalities of the ipsilateral urinary system, primarily in the form of a double uterine body, double cervix, and absent kidney on the oblique septum. The latter can be divided into type 1 perforated oblique septum, type 2 perforated oblique septum, and type 3 perforated oblique septum combined with cervical fistula [5]. A differential diagnosis can be made with the help of gynecologic examination, color ultrasound, HSG, magnetic resonance imaging (MRI), uterine laparoscopy, and other examinations [6, 7]. At present, ultrasound is widely used in the diagnosis of uterine malformations; in particular, three-dimensional ultrasound can clearly show whether the internal structure and external shape of the uterine cavity is abnormal, thereby demonstrating high diagnostic value. Using HSG can easily result in the misdiagnosis of Robert’s uterus as a single-horn uterus with or without a residual horn. Pelvic MRI examination has several advantages in the diagnosis of uterine malformations; in the MRI results of Robert’s uterus, the external contour of the uterus is normal, two uterine lumens of different sizes can be seen, and one uterine lumen is not connected to the vagina; sometimes there is also hematosalpinx on the closed side, or there may be abnormal cysts in the adnexal area, but there is no abnormality on the opposite side. Timely MRI examination can thus reduce the probability of misdiagnosis. In the present case, hysteroscopy showed a single-horn uterus or barrel shape, only one uterine horn, and a fallopian tube opening. Laparoscopy showed a normal uterine shape or distended uterine horn on one side, tubal thickening or a normal tube on the distended side, as well as other possible pelvic abnormalities. A previously published article reported a case of acute appendicitis that, upon laparoscopic surgical exploration, resulted in discovery of salpingitis caused by Robert’s uterus on the obstructed side. Combined laparoscopy and hysteroscopy can improve diagnostic accuracy. Currently, laparoscopy combined with hysteroscopy is the gold standard for diagnosing Robert’s uterus [8].

Adenomyosis and congenital uterine anomalies (CUAs) can compromise reproductive potential and may coexist in the same patient, especially in cases of infertility. Adenomyosis can be found in both syndromic and nonsyndromic CUAs, which can arise from several etiologies. One hypothesis is that obstructions in CUAs increase uterine pressure and promote the development of adenomyosis [9]. In our case, Robert’s uterus caused a pelvic infection, adenomyosis, an endometriotic cyst, and other complications. However, our hypothesis is as follows: When the ipsilateral fallopian tube of Robert’s uterus is open, menstrual blood flows back into the pelvic cavity along the ipsilateral fallopian tube and stimulates the pelvic peritoneum, which can result in chemical peritonitis. Meanwhile, blood provides a good medium for bacteria. When the body’s resistance is reduced or exogenous operations, such as artificial abortion or cesarean section, occur, the chances of bacterial invasion are increased. As a result, pelvic inflammatory disease occurs, which harms the pelvic environment and causes blockage of the fallopian tube. If the fallopian tube is blocked on the blocked side, the menstrual blood fails to flow back into the abdominal cavity and instead accumulates in the uterine cavity on the blocked side, infiltrating the myometrium over time and leading to the formation of adenomyotic lesions on the blocked side, as well as causing more serious symptoms of dysmenorrhea. A long-term countercurrent of menstrual blood can lead to endometriosis and pelvic infection [10].

At present, surgery is the only effective way to treat Robert’s uterus [4, 11,12,13]. A review of the literature search revealed that five surgical procedures were commonly undertaken. (1) Open or laparoscopic incision of the closed uterine cavity and ligation of the ipsilateral fallopian tube. (2) Open or laparoscopic hemihysterectomy with ipsilateral tubal ligation. Both of these first two procedures are suitable for patients whose embryos have stopped developing and who need to preserve their reproductive function. (3) Excising the endometrium of the closed cavity. This operation is suitable for a closed uterine cavity and obstruction of the ipsilateral fallopian tube. (4) Resection of the oblique septum of Robert’s uterus. This approach is applicable to an abdominal cavity with endometriosis and other coexisting lesions, and it can be performed through laparoscopy or open surgery. (5) Hysteroscopic electric resection (or cutting) of the diaphragm of Robert’s uterus. This is currently the recommended first-line treatment for patients with fertility requirements.

Treatment for adenomyosis should be individualized [14]; specifically, it should be tailored to symptoms (i.e., heavy menstrual bleeding, pain, and/or infertility) and include medical options (e.g., non-steroidal anti-inflammatory drugs, tranexamic acid, combined oral contraceptives, levonorgestrel intrauterine system, dienogest, other progestins, or gonadotropin-releasing analogues), interventional options (e.g., uterine artery embolization), and surgical options (e.g., endometrial ablation, excision of adenomyosis, or hysterectomy).

In our case, due to infection, the patient’s fallopian tube was blocked on the atresia side and the menstrual blood failed to regress into the abdominal cavity, resulting in its accumulation in the uterine cavity on the atresia side; it then infiltrated the myometrium over time, resulting in the formation of adenomyotic lesions on the blocked side, resulting in more serious symptoms of dysmenorrhea. Hysteroscopic septomotomy, for example, can only solve the problem of menstrual blood drainage on the atresia side, but it cannot effectively relieve the symptoms of dysmenorrhea caused by adenomyosis. If the patient has no fertility requirements, the purpose of the operation is not only to restore the normal form of the uterus to drain menstrual blood, but also to solve the dysmenorrhea caused by adenomyosis. In fact, the uterine cavity of the closed side is equivalent to a large adenomyoma. The guiding principle behind removing the adenomyoma is to deal with the lesions and blind blood accumulation, which can effectively alleviate symptoms. In our case, after admission, the empirical antibiotic treatment was effective, the routine blood test results and C-reactive protein levels quickly dropped back to normal, the abdominal pain was relieved, and the operation was performed 10 days after anti-inflammatory treatment. The patient had passed the acute inflammatory period, the bleeding and damage had been reduced after the pelvic adhesions were separated by reoperation, and the timing of the operation was suitable. Previously, the patient had undergone three cesarean sections and one laparoscopic operation for abscess. The laparoscopic operation, conducted at another hospital, resulted in the detection of dense pelvic adhesions. We selected laparotomy for this patient to ensure that more elaborate separation and suturing could be performed, thereby reducing the risk of periprocedural organ injury. The patient’s postoperative recovery was good and her symptoms of dysmenorrhea were relieved, indicating that the surgical approach was correct. When an infection of this kind cannot be effectively controlled through antibiotic treatment and the clinical symptoms are serious, timely surgery should be carried out.

Summary

The patient had a history of dysmenorrhea for many years, a history of laparoscopic surgery, and three cesarean sections, but we could not accurately diagnose the type of uterine deformity. The rarity of this malformation has led to a lack of knowledge regarding it among clinicians. Imaging examination plays an important role in the diagnosis of Robert’s uterus. For patients with progressive and worsening dysmenorrhea and a two-dimensional ultrasound showing a septoid structure in the uterine cavity, three-dimensional ultrasound should be performed as quickly as possible before surgery, along with MRI if needed. The early and accurate diagnosis and treatment of Robert’s uterus is very important, and individualized surgical treatment should be planned according to the patient’s age, fertility requirements, and whether there is endometriosis or adenomyosis. In this case, we adopted a new approach that not only ensured the integrity of the uterus, but that also removed the adenomyotic lesion to maximize the relief of dysmenorrhea. This approach provides a new method for the treatment of such diseases.