{"paper_id":"62235703-5796-478b-a8d6-c625d9d4c365","body_text":"Review began\n 11/11/2020 \nReview ended\n 12/14/2020 \nPublished\n 12/15/2020\n© Copyright \n2020\nWazir et al. This is an open access article\ndistributed under the terms of the\nCreative Commons Attribution License\nCC-BY 4.0., which permits unrestricted\nuse, distribution, and reproduction in any\nmedium, provided the original author and\nsource are credited.\nMassive Leiomyomata and Severe Endometriosis\nResulting in a Frozen Pelvis in an Asymptomatic\nPatient\nHersh Wazir \n \n, \nMolly S. Jain \n \n, \nEnkhmaa Luvsannyam \n \n, \nMichael Rayalu \n \n, \nCharles Alston \n1.\n Medicine, All Saints University College of Medicine, Toronto, CAN \n2.\n Medicine, Saint James School of Medicine, Park\nRidge, USA \n3.\n Medicine, California Institute of Behavioral Neurosciences and Psychology, Fairfield, USA \n4.\n Medicine,\nAmerican University of Integrative Sciences, Atlanta, USA \n5.\n Obstetrics and Gynecology, Roseland Community\nHospital, Chicago, USA\nCorresponding author: \nHersh Wazir, \nhershwazir@gmail.com\nAbstract\nLeiomyomas, also known as uterine fibroids, are the most common benign uterine tumors in women. The\nmost frequently reported symptoms are uterine bleeding and abdominal and/or pelvic pressure; however,\nmost cases are asymptomatic and may be found incidentally. Endometriosis is a condition where the\nendometrium proliferates outside of the uterine cavity. Extrauterine endometrial implants are usually found\nin the ovaries, pelvis, and peritoneum, but can extend anywhere throughout the body. Women with\nendometriosis may exhibit dysmenorrhea, dyspareunia, dyschezia, and infertility. Inflammation caused by\nendometriosis may lead to fibrosis, scarring, and adhesions. We report a case of an asymptomatic 36-year-\nold African-American woman with increasing abdominal girth, consistent with a 28-week gestation,\npresenting to her obstetrician/gynecologist for her annual exam, who on further investigation is found to\nhave multiple large fibroids, bilateral ovarian cysts, and widespread endometriosis with several adhesions\nultimately leading to a frozen pelvis.\nCategories:\n Obstetrics/Gynecology, Preventive Medicine, General Surgery\nKeywords:\n endometriosis, leiomyoma, fibroids, frozen pelvis, adhesions, ovarian cyst\nIntroduction\nUterine fibroids are the most common gynecologic tumors in women of reproductive age and are seen in\nmore than 77% of all women \n[1]\n. Fibroids are benign tumors arising from myometrial smooth muscle cells\nand can be large, numerous, and found in various locations. Fibroids located in the muscular wall of the\nuterus are commonly called intramural fibroids and those located within the outer lining or serosa of the\nuterus are known as subserosal fibroids. Moreover, fibroids inside the interior lining of the uterus are called\nsubmucosal fibroids. Symptomatic tumors present with pelvic pain or pressure, abnormal uterine bleeding,\nanemia, dysmenorrhea, bladder or bowel dysfunction, sexual dysfunction, compression of adjacent pelvic\norgans, and reproductive disorders such as infertility and recurrent pregnancy loss \n[2]\n. The risk factors\nassociated with uterine fibroids are age, early menarche, alcohol, high body mass index (BMI), polycystic\novary syndrome, and race \n[3,4]\n. Uterine fibroids are three times more frequently seen in African American\nwomen than in Caucasian women \n[5]\n. \nUterine fibroids are diagnosed by transabdominal ultrasound and symptomatic fibroids can be managed\nmedically or surgically. Medical treatment is the preferred option for patients who want to maintain future\nfertility or patients at high risk for surgery. If symptoms are severe and refractory to medical treatment,\nmyomectomy is the gold standard surgical procedure for women who desire to maintain fertility \n[2]\n. Up to\n50% of women with fibroids develop severe symptoms requiring surgery \n[6]\n. Hysterectomy is reserved for\npatients who present with severe symptomatic fibroids and do not wish to become pregnant. Uterine\nfibroids remain to be the most common indication for hysterectomy in the United States with more than\n200,000 hysterectomies performed annually \n[7]\n. Despite the high prevalence of uterine fibroids and their\nmajor complications, there is still not a complete understanding of the pathogenesis of these tumors.\nEndometriosis is a complex clinical disease characterized by ectopic implantation of endometrial glands in\nextrauterine tissues. Endometriosis is seen in 6% to 10% of women of reproductive age and is the most\ncommon cause of chronic pelvic pain \n[8]\n. Endometriosis is associated with the estrogen-dependent\ninflammatory process; however, the pathophysiology is not fully understood. The most frequently reported\nclinical features are chronic pelvic pain, dysmenorrhea, deep dyspareunia, dyschezia, and infertility \n[9]\n.\nThree major forms of endometriosis are peritoneal endometriosis, deep infiltrating endometriosis found in\nthe pouch between the vagina and rectum (pouch of Douglas), and ovarian endometriomas, which present as\novarian cysts filled with blood, commonly known as “chocolate cysts” \n[10]\n. \nInitially, the presumptive diagnosis of endometriosis is medically managed with nonsteroidal anti-\ninflammatory drugs (NSAIDs) and oral contraceptive pills (OCPs), progestins, or gonadotropin-releasing\n1\n2\n3\n4\n5\n \n Open Access Case\nReport\n \nDOI:\n 10.7759/cureus.12097\nHow to cite this article\nWazir H, Jain M S, Luvsannyam E, et al. (December 15, 2020) Massive Leiomyomata and Severe Endometriosis Resulting in a Frozen Pelvis in an\nAsymptomatic Patient. Cureus 12(12): e12097. \nDOI 10.7759/cureus.12097\n\nhormone (GnRH) agonists \n[9]\n. When symptoms do not improve with medical management, direct\nlaparoscopic visualization is required for definitive diagnosis and resection of implants \n[9]\n. Delay in\ndiagnosis is a major problem for the management of endometriosis due to nonspecific symptoms and lack of\ndiagnostic tools. Therefore, the treatment is often not initiated until the disease has progressed at least 8 to\n10 years \n[8]\n.\nThe chronic inflammation caused by the endometrial glands and stroma found outside the uterine cavity\ncauses remodeling of the surrounding tissues which may lead to fibrosis, scarring, and pelvic adhesions \n[10]\n.\nExtensive adhesions can lead to a frozen pelvis, which is characterized by fibrosis, reduced tissue elasticity,\ndistortion of anatomical structures, severe adhesions, and replacement of pelvic soft tissue by abnormal\ntissue \n[11]\n.\nIn this report, we present a case of a patient with severe adhesions due to widespread endometriosis and\nlarge fibroids leading to an asymptomatic frozen pelvis.\nCase Presentation\nA nulliparous 36-year-old African-American female, with a past medical history of a recurrent Bartholin cyst\nand irritable bowel syndrome (IBS), presented to her obstetrician/gynecologist’s (OB/GYN) office for her\nannual gynecological exam in September 2015. Prior to this, the patient was compliant with her annual\nappointments which revealed no significant abnormalities. The patient was sexually active with one male\npartner, with whom she had been trying to get pregnant for two years without success. Both she and her\nfamily had noted that her abdomen had increased in girth significantly over the span of six months despite\nno change in her diet, physical activity, or menstrual cycle, which then prompted her to schedule her annual\nvisit to her OB/GYN office. Her menstrual cycle was regular at 28-day intervals with menses lasting for four\ndays, “like clockwork,” per the patient. The patient stated no irregularities with her menses at this time,\ndenying any menorrhagia, dysmenorrhea, dyspareunia, dyschezia, or dysuria. At the time of her visit, she\nstated that her abdomen felt firm and her last bowel movement was three days prior, which was thought to\nbe due to her IBS. On abdominal physical exam, firmness was felt across all quadrants, with tenderness in\nthe right lower quadrant upon palpation. The patient described the pain as dull and reported a history of this\npain intermittently for two years. She was found to have intra-abdominal and pelvic swelling with findings\nof a mass and lump at an unspecified site. The remainder of the physical exam revealed no abnormalities.\nBlood work was drawn at this visit and results were normal aside from an elevated carcinoembryonic antigen\n(CEA). A computerized tomography (CT) of the abdomen and pelvis was ordered promptly. The results of the\nCT showed mild hydronephrosis of the right kidney, severe hydronephrosis of the left kidney, a massive\nfibroid uterus with two dominant subserosal fibroids, and two enlarged ovarian cysts, the left being\nmultiloculated (Figures \n1\n-\n2\n).\nFIGURE\n 1: Pelvic CT showing massive fibroid uterus measuring 19 cm\nin length and 15 cm in width with one large subserosal fibroid\nprojecting superiorly to the right measuring 13 x 12 cm, another\nprojecting superiorly to the left measuring 10 x 8 cm, evidenced by\ncoronal view (A) and again by transverse view (B).\n2020 Wazir et al. Cureus 12(12): e12097. DOI 10.7759/cureus.12097\n2\n of \n5\n\nFIGURE\n 2: \nPelvic CT showing an ovarian multiloculated cyst on the left\nmeasuring 8.8 x 6.8 cm (A), and another cystic process on the right\nmeasuring 5.1 x 4.1 cm (B).\nThe patient was provided with the option to manage the fibroids medically, with Lupron for six months, or\nsurgically via a hysterectomy. After a discussion of these options, their risks, benefits, and their respective\nprognoses, the patient agreed to a total abdominal hysterectomy as she did not desire to maintain fertility.\nIn December 2015, a total abdominal hysterectomy with bilateral salpingo-oophorectomy with lysis of\nmultiple adhesions of the omentum, intestines, pelvis, ovaries, and rectosigmoid was performed. During the\nprocedure, it was found the uterus was frozen to the pelvic floor, along with several additional fibroids that\nwere not visible on CT (Figure \n3\n). A general surgeon was called in to assist with lysis of numerous adhesions\nto free the frozen pelvis and colon. The uterus, fallopian tubes, and ovaries were removed. Histopathology\nrevealed chronic cervicitis, proliferative endometrium with endometrial polyps, multiple subserosal\nleiomyomata, the largest measuring 19 x 16 x 10.5 cm, a patent fallopian tube attached to another\nsubserosal leiomyoma, bilateral ovaries with cysts, one with multiple follicular cysts and the other with a\nhemorrhagic corpus luteal cyst, and a large leiomyoma weighing 577 g and measuring 13.5 x 11 x 8.5 cm with\nfocal degenerative changes. The patient recovered with no complications from the procedure. Currently, the\npatient is doing well and takes no medications.\nFIGURE\n 3: Perioperative images showing a 28-week-gestation sized\nfibroid uterus during hysterectomy and bilateral salpingo-oophorectomy\n(A) and massive fibroid uterus with additional subserosal fibroid and\nbilateral ovarian cysts after resection (B).\nDiscussion\n2020 Wazir et al. Cureus 12(12): e12097. DOI 10.7759/cureus.12097\n3\n of \n5\n\nUterine fibroids are benign tumors in women of childbearing age and the etiology is primarily dependent on\nhormones, specifically estrogen. The clinical significance of leiomyomas pertains to their size and location\nin the pelvis. Large fibroids compressing other organs can present with severe symptoms in women causing\nsignificant distress to the patient’s quality of life. Surprisingly, our patient did not present with any of the\ncommon symptoms and remained asymptomatic despite the later findings of multiple large and heavy\nfibroids. However, since she had subserosal fibroids, it appears that she remained infertile at least partly due\nto this reason. Since subserosal fibroids are located outside of the uterus, they can obstruct the pathway to\nthe cervix or fallopian tubes, thereby blocking the journey of sperm towards the fertilized egg for conception\n[12]\n. \nSome of the risk factors for the development of uterine leiomyomas are hereditary, others include race,\nobesity, vitamin D deficiency, and early menarche \n[13]\n. Racially, black women are more likely to develop\nfibroids than women of other races \n[12]\n. Moreover, African American women develop fibroids at earlier ages\nand present with larger tumors with more significant symptoms than Caucasian women \n[7]\n. Aside from\nultrasound, other imaging modalities include CT, MRI, hysterosonography, hysterosalpingography, and\nhysteroscopy to locate submucosal leiomyomas and to check the degree of patency of the fallopian tubes\n[13]\n. Treatment protocol of uterine fibroids is based on patient symptoms, pregnancy status, and other\ncomorbidities. Current medical treatment for symptomatic fibroids includes NSAIDs, OCPs, high-dose\nprogestins, tranexamic acid, and GnRH agonists \n[2]\n. However, medical treatments may cause undesirable\nside effects, and also cause the tumor to rebound once treatment is stopped \n[14]\n. Some minimally invasive\nprocedures such as uterine artery embolization, radiofrequency ablation, laparoscopic myomectomy,\nhysteroscopic myomectomy, and endometrial ablation can destroy fibroids without their surgical excision\n[13]\n. In complicated fibroids, which are larger in size and heavier in weight, surgical procedures are usually\nrequired. Due to the size of this patient’s fibroids, the compression of the bilateral ureters, multiple bilateral\novarian cysts, and extensive adhesions, a total abdominal hysterectomy with bilateral salpingo-\noophorectomy was performed. This procedure renders an individual infertile but is the permanent solution\nto complicated fibroids as seen in this case.\nFurthermore, while the hysterectomy was being performed, there was an unexpected finding of severe\nendometriosis of the pelvis, ovary, uterus, and rectosigmoid colon with multiple adhesions of the omentum.\nThe patient was peri-operatively diagnosed with a severe frozen pelvis as a result of the aforementioned\ncomplications. The endometriotic implants could be grown anywhere from the ovaries and bowel to any\ntissues lining the pelvis. The pathophysiology behind endometriosis is quite complex, and there are still\nmany theories. The retrograde menstruation theory states that the endometrial cells flow backward into the\npelvic cavity through the fallopian tubes during menses, implant onto various abdominal organs and\nperitoneum, grow, and cause chronic inflammation causing adhesions \n[9]\n. The severity of the disease is\nassociated with the amount of menstrual flow, estrogen dependence, progesterone resistance, inflammation,\nand genetic and environmental factors \n[9]\n.\nDiagnosing endometriosis involves taking a detailed history, performing pelvic exams, implementing\ntransvaginal/abdominal ultrasound, and finally, carrying out laparoscopy \n[15]\n. Diagnosis and medical\nmanagement of endometriosis involve enacting a similar approach to that of leiomyomata. There are four\nstages of endometriosis: minimal, mild, moderate, and severe. The severe stage of endometriosis is apparent\nwhen the implants are quite deep within the pelvic cavity and ovaries. The culmination of the severe stage\nmay result in a frozen pelvis; this occurs when the pelvic organs, such as the rectum, bladder, ureters, large\nand small bowels, and/or ovaries become densely adhered to one another via adhesions \n[16]\n. The organs that\nare densely adherent may have nerves that develop within the adhesions, leading to neuropathic pain which\nmay be masked by the pain of endometriosis \n[15]\n. Frozen pelvis requires prompt surgery to alleviate the\naforementioned adhesions and symptoms.\nThe highlight of this case is the asymptomatic presentation of the patient even though she had multiple life-\nthreatening gynecological disorders including multiple large fibroids along with severe endometriosis\nleading to a frozen pelvis. The patient was not taking any medications and felt nothing abnormal in spite of\nhaving such severe pelvic conditions. The patient faced infertility which could be attributed to the combined\neffect of both the fibroids and endometriosis as well as the adhesions. This case is rare, as it points to the\ninfrequent presentation of multiple common gynecological conditions. It also highlights the importance of\nregular physical exams and follow-ups to prevent severe complications such as infertility, bowel\nobstructions, kidney injury, and even hemorrhage.\nConclusions\nOur understanding of uterine leiomyomas and endometriosis often involves symptomatic review, as well as\nour knowledge that patients differ in severity. It is thus a rare case where a patient is severely affected but\noddly asymptomatic. The woman in this case presented with moderate distention while CT showed\nmultiple, massive fibroids that would normally present with severe symptoms. Additionally, when clinically\nappraised, the patient was found to have further pelvic adhesions and multiple foci of endometriosis\nthroughout her pelvic organs and on her bowel. The question could be asked, which gynecologic pathology\narose first? A more important question is asking, how can we adapt to what amounts to very individual\nexperiences in patient symptomatology? This case highlights the importance of doing so and how\n2020 Wazir et al. Cureus 12(12): e12097. DOI 10.7759/cureus.12097\n4\n of \n5\n\nparamount it is for clinicians to pay attention to early diagnosis regardless of type or severity of symptoms,\nespecially in patients such as this with multiple risk factors.\nAdditional Information\nDisclosures\nHuman subjects:\n Consent was obtained by all participants in this study. \nConflicts of interest:\n In\ncompliance with the ICMJE uniform disclosure form, all authors declare the following: \nPayment/services\ninfo:\n All authors have declared that no financial support was received from any organization for the\nsubmitted work. \nFinancial relationships:\n All authors have declared that they have no financial\nrelationships at present or within the previous three years with any organizations that might have an\ninterest in the submitted work. \nOther relationships:\n All authors have declared that there are no other\nrelationships or activities that could appear to have influenced the submitted work.\nAcknowledgements\nWe thank the patient for allowing us to publish the details of her case and for helping to advance the\nknowledge of her condition; we also thank Ebuka Ibuoka for his extensive help with this paper.\nReferences\n1\n. \nCramer SF, Patel A: \nThe frequency of uterine leiomyomas\n. 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