Robotic-assisted laparoscopic excision of rudimentary uteri for chronic pelvic pain in Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

An 18-year-old female patient presented with recurrent abdominal pain of more than 12 months’ duration and a medical history of MRKH syndrome. The chronic abdominal and pelvic discomfort was predominantly left-sided, without related gastrointestinal symptoms. Besides primary amenorrhea, the patient’s medical background was unremarkable. Physical examination revealed normal secondary sexual characteristics, including the normal appearance of the external genitalia. Nevertheless, an imperforate hymen was noted, with no continuation to a vaginal cavity. Abdominal palpation combined with rectal examination identified a small, rudimentary uterus with abdominopelvic tenderness. Pelvic ultrasound revealed a small, rudimentary hemi-uterus with normal ovaries. Magnetic resonance imaging (MRI) confirmed a uterine congenital malformation. The ovaries were normal; however, the cervix and vaginal cavity were absent (Fig. 1 ). Additional abdominal MRI excluded urinary tract malformations. The MRI results led to the radiological diagnosis of total cervicovaginal aplasia with uterine malformation. Laboratory tests upon admission showed a normal hematocrit of 0.388 L/L and a serum hemoglobin level of 13.9 g/dL, with a normal platelet count. Figure 1. Preoperative T1-weighted MRI image reveals the rudimentary uterus in the lower abdominal cavity (yellow arrow). Preoperative T1-weighted MRI image reveals the rudimentary uterus in the lower abdominal cavity (yellow arrow). Following patient consent, an elective robotic-assisted laparoscopy was performed under general anesthesia. The Da Vinci Xi system (Intuitive Surgical, Inc., Sunnyvale, CA, USA) was used. The French supine position with 30 degrees reverse Trendelenburg was adopted. The arms were fixed at the sides of the trunk. The surgical assistant was positioned between the open legs of the patient. Standard port placement was employed, with the Xi system docked from the left side of the patient. The setup involved four robotic ports and one laparoscopic port strategically placed in the abdomen. In this phase, standard robotic instruments were used: a bipolar instrument (a long fenestrated bipolar forceps or a Maryland bipolar forceps), Cadiere forceps, monopolar curved scissors, a robotic Hem-o-lok applier, and a needle driver. The assistant port was used for suction, insertion of sutures, sponges, and laparoscopic graspers. Throughout the robotic procedure, the hemi-uteri remained small, without signs of hematometra, and no visible cervix was present. The ovaries were normal, as were the fallopian tubes. Intraperitoneal blood clots were not observed. A complete excision of both rudimentary uteri was performed with bilateral salpingectomy (Fig. 2 ). The surgery lasted 88 minutes, and the estimated blood loss was minimal. Pathological examination of the excised specimen verified the presence of an atypical, epithelialized, rudimentary uterus with mural fibrosis. Bilateral, atypical, epithelialized fallopian tubes and fimbrial funnels, as well as a single paratubal cyst, were present. No evidence of malignancy was detected (Fig. 3 ). The patient’s postoperative course was uneventful, and prophylactic antibiotic therapy was considered unnecessary. She demonstrated satisfactory recovery and was discharged from the hospital on the second postoperative day. Laboratory tests at discharge showed a normal hematocrit of 0.352 L/L and a serum hemoglobin level of 12.0 g/dL, with a normal platelet count. Follow-up at 3 and 6 months postoperatively demonstrated an asymptomatic course and satisfactory clinical outcomes. Figure 2. Intraoperative view of the rudimentary hemi-uteri (black arrow) with the bilateral tubes (yellow arrow) and normal ovaries, along with the prepared specimen: rudimentary uterus (black arrow) with the tubes (yellow arrow). Figure 3. Representative microphotograph of the tube cross-section (3a): dysplasia-free tube cross-section and of the rudimentary uterus (3b): uterine rudiment with non-atypical luminal columnar cell epithelium and a wall of smooth muscle tissue (hematoxylin and eosin stain, 100×). Intraoperative view of the rudimentary hemi-uteri (black arrow) with the bilateral tubes (yellow arrow) and normal ovaries, along with the prepared specimen: rudimentary uterus (black arrow) with the tubes (yellow arrow). Representative microphotograph of the tube cross-section (3a): dysplasia-free tube cross-section and of the rudimentary uterus (3b): uterine rudiment with non-atypical luminal columnar cell epithelium and a wall of smooth muscle tissue (hematoxylin and eosin stain, 100×).

Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome represents a spectrum of congenital anomalies affecting the female reproductive tract, with variable degrees of vaginal and uterine underdevelopment stemming from incomplete Müllerian duct (MD) differentiation during fetal life [ 1 , 2 ] . Epidemiological data indicate an occurrence rate of approximately 1 in 4500 to 5000 live female births [ 3 ] . The clinical presentation varies considerably: Type I manifests exclusively with reproductive tract involvement, whereas Type II encompasses associated anomalies affecting the kidneys, skeletal system, heart, or nervous system [ 4 ] . A specific variant, termed MURCS association, describes the triad of MD aplasia, unilateral renal absence or malposition, and vertebral segment abnormalities [ 5 ] . HIGHLIGHTS This case underscores the rarity and clinical significance of Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome and emphasizes the potential for chronic pelvic pain in such patients. We present the case of an 18-year-old woman with vaginal aplasia, cervical atresia, and a rudimentary uterus. Regardless of the surgical approach, referral to specialized centers with expertise in managing MRKH syndrome is recommended. In cases of complete vaginal atresia, total hysterectomy is often the preferred treatment to prevent future complications; robotic-assisted surgery offers a safe and effective therapeutic option for selected patients with MRKH syndrome. HIGHLIGHTS This case underscores the rarity and clinical significance of Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome and emphasizes the potential for chronic pelvic pain in such patients. We present the case of an 18-year-old woman with vaginal aplasia, cervical atresia, and a rudimentary uterus. Regardless of the surgical approach, referral to specialized centers with expertise in managing MRKH syndrome is recommended. In cases of complete vaginal atresia, total hysterectomy is often the preferred treatment to prevent future complications; robotic-assisted surgery offers a safe and effective therapeutic option for selected patients with MRKH syndrome. This case report details an innovative therapeutic approach for addressing intractable pelvic symptoms in an adolescent with MRKH syndrome. Although this condition does not pose immediate health threats, affected individuals often struggle with diminished well-being due to the emotional burden of reproductive anomalies, particularly when the diagnosis emerges during the vulnerable teenage years. We believe this represents the inaugural description of robot-assisted uterine removal in a young MRKH patient, specifically targeting pain relief. Standard laparoscopic techniques pose particular challenges in such anatomically distorted surgical fields, with an elevated risk of inadvertent injury to neighboring structures, underscoring the potential value of robotic technology. This report emphasizes how robotic precision can address symptomatic presentations while highlighting the broader clinical implications of this rare disorder. The SCARE reporting framework guided this manuscript [ 6 ] .

Structural reproductive tract abnormalities occur in roughly 4% of women [ 7 ] . Among these, MRKH-associated uterine absence constitutes the predominant form. Isolated vaginal absence with preserved uterine tissue occurs less frequently, affecting approximately 9% of cases with partial anomalies. From a developmental perspective, the paramesonephric ducts generate the tubal structures, uterine body, and proximal vaginal segment, while the distal vaginal portion derives from urogenital sinus tissue. Paramesonephric duct maldevelopment at the caudal end produces vaginal atresia [ 8 ] . Absent menstruation typically brings patients to clinical attention first, with cyclical pelvic discomfort being the next most common presenting complaint. Contemporary nomenclature, developed jointly by ESHRE and ESGE, provides a systematic framework categorizing anomalies of the uterus, cervix, and vagina [ 9 ] . Within this schema, our patient’s anatomy corresponds to the U4/C4/V4 designation (Table 1 ) [ 10 ] . Chromosomal studies in MRKH consistently reveal standard female 46,XX karyotypes [ 1 ] . Ovarian function remains preserved, enabling normal pubertal development with appropriate secondary sexual characteristics. The hallmark symptom remains failure of menarche secondary to utero-vaginal hypoplasia, while sexual intercourse difficulties due to vaginal shortening represent another prevalent concern. Most affected women (exceeding 90%) retain uterine remnant tissue, typically appearing as myometrial masses that may or may not contain endometrial elements [ 2 , 3 ] . When functional endometrium exists within these remnants, persistent pelvic symptoms frequently develop. Table 1 Classification of uterine anomalies by ESHRE/ESGE. Uterine anomaly Cervical/vaginal anomaly Main class Sub-class Co-existent class U0 Normal uterus C0 Normal cervix U1 Dysmorphic uterus T-shaped C1 Septate cervix Infantilis C2 Double normal cervix Others C3 Unilateral cervical aplasia U2 Septate uterus Partial C4 Cervical aplasia Complete V0 Normal vagina U3 Bicorporeal uterus Partial Complete Septate V1 Longitudinal non-obstructing vaginal septum U4 Hemi-uterus With rudimentary cavity V2 Longitudinal obstructing vaginal septum Without rudimentary cavity V3 Transverse vaginal septum and/or imperforate hymen U5 Aplastic With rudimentary cavity V4 Vaginal aplasia Without rudimentary cavity U6 Unclassified malformations U C V Classification of uterine anomalies by ESHRE/ESGE. T-shaped Infantilis Others Partial Complete Partial Complete Septate With rudimentary cavity Without rudimentary cavity With rudimentary cavity Without rudimentary cavity Recognition of MRKH typically occurs around the age of 14 years, with reported ages spanning from 10 to 22 years [ 11 ] . Zayed and colleagues documented a comparable age distribution of 14–21 years in their series [ 12 ] . Our patient’s presentation at 18 years represents a somewhat delayed diagnosis. Abdominal symptoms, particularly those with cyclical patterns, predominate clinically. Tian et al. examined 79 MRKH patients experiencing chronic or periodic pelvic symptoms who ultimately required surgical excision of their rudimentary uterine tissue. Histological analysis revealed endometrial differentiation in nearly half (43%) of specimens [ 13 ] . Comparable findings emerged from Rall and colleagues’ examination of 42 uterine remnants, where 40.4% demonstrated endometrial epithelium with characteristic CD10-positive stromal cells [ 14 ] . Additional research by Brucker’s group documented compromised decidualization capacity in endometrial stromal cells harvested from 39 MRKH patients [ 15 ] . Clinical examination typically reveals unremarkable external anatomy and secondary sex characteristics, though inspection commonly identifies a vaginal indentation without cervical identification [ 4 ] . Sonographic evaluation serves as the accessible, economical initial investigation, revealing absent or hypoplastic uterine structures with patent vaginal channels. Crucially, ovarian tissue appears normal owing to its distinct embryological derivation [ 9 ] . Three-dimensional ultrasound demonstrates strong concordance with MRI findings, ranging from 88.9% to 96.3% [ 16 ] . MRI represents the definitive diagnostic modality, particularly valuable for operative planning [ 17 ] . Its superior tissue contrast enables identification of subtle uterine remnants and precise characterization of genital tract malformations. MRI also permits recognition of endometrial tissue within remnants, showing strong correlation with surgical pathology. Direct visualization via laparoscopy, while invasive, typically serves therapeutic rather than diagnostic purposes. Therapeutic options encompass both conservative and operative interventions. Progressive vaginal stretching techniques enjoy first-line status given their high efficacy and favorable safety profile. Surgical neovagina creation becomes appropriate when dilation proves unsuccessful. For women desiring genetic offspring, uterine transplantation and gestational carrier arrangements represent emerging possibilities. Management of hormonally active uterine remnants warrants particular attention – an estimated 40% of MRKH patients harbor functional endometrium that may remain quiescent for extended periods before manifesting obstructive phenomena [ 1 , 7 ] . Surgical removal provides definitive treatment for obstruction and serves as prophylaxis against secondary endometriosis. Given the strong association between chronic symptoms and functioning endometrial tissue within remnants, hysterectomy offers substantial pain relief. Symptomatic patients, therefore, benefit from surgical referral. Robotic surgical platforms have witnessed tremendous growth in utilization. These systems offer enhanced patient outcomes and improved surgical execution, with demonstrated applicability across multiple subspeciality domains, including adolescent reproductive surgery. Comparative analyses of conventional laparoscopic versus robotic approaches have documented superior complication profiles with robotic technology, alongside abbreviated convalescence and reduced hospitalization duration [ 18 ] . The robotic interface delivers exceptional precision with minimally invasive access and optimized visualization. Operating surgeons experience meaningful ergonomic advantages, with documented reductions in cognitive demands and physical fatigue [ 19 ] . Patients without acute symptoms warrant ongoing surveillance, with consideration for delayed intervention should obstructive manifestations emerge. The timing of surgical intervention requires careful deliberation – premature operative intervention risks poor compliance and suboptimal outcomes. ACOG guidance emphasizes proceeding only when the patient demonstrates adequate emotional readiness and expresses an autonomous preference for treatment [ 20 ] . MRKH carries a substantial psychological burden, frequently manifesting as diminished self-worth, depressive symptoms, and anxiety. Incorporating psychological services into treatment algorithms meaningfully enhances therapeutic success. The additional burden of chronic pain further compromises quality of life, and persistent pain syndromes independently contribute to deteriorating mental and physical health. Consequently, a safe, minimally traumatic intervention, such as robot-assisted hysterectomy, may confer particular benefits for optimizing outcomes in this vulnerable population.

Comprehensive evaluation of MRKH patients demands coordinated input from pediatric gynecologists, orthopedic specialists, nephrologists, imaging experts, and mental health professionals. Published series demonstrate variable frequencies of extra-reproductive malformations, potentially reflecting population-specific differences. Beyond clinical assessment and basic sonography, pelvic MRI proves indispensable for characterizing any endometrial component within rudimentary structures. Cross-sectional imaging remains the benchmark for definitive diagnosis and detailed mapping of complex female genital anomalies, enabling visualization of retained menstrual products within malformed structures. Chronic pelvic symptoms commonly occur among individuals with residual uterine tissue and may substantially compromise daily function. Referral to institutions with dedicated expertise is strongly advisable – surgeon experience proves particularly important, given that initial procedures typically achieve better results than revision operations. This report presents an illustrative case demonstrating how surgical management prevented anticipated complications that would have occurred with expectant management. We aim to enrich the existing literature and support future investigations by emphasizing the importance of individualizing therapy to each patient’s emotional, social, and family context.