{"paper_id":"8732924b-c695-42d9-9acc-2ebdc215cbc5","body_text":"This article has been accepted for publication and undergone full peer review but has n ot been \nthrough the copyediting, typesetting, pagination and proofreading process which may lead to \ndifferences between this version and the Version of Record. Please cite this article as doi: \n10.1002/uog.22173 \n \nImaging in gynecological disease: clinical and ultrasound characteristics of \naccessory cavitated uterine malformations  \n \n \nJ. Naftalin\n1, E. Bean1, E. Saridogan1, P. Barton-Smith2, R. Arora3, D. Jurkovic1 \n \n \n1Institute for Women’s Heath, University College Hospital, London, UK \n \n2The Endometriosis Clinic, London, UK \n \n3Department of Cellular Pathology, University College London NHS Trust, London, \nUK \n \n \nCorresponding author: \nJoel Naftalin, Institute for Women’s Health, EGA wing, University College Hospital, \n25 Grafton Way, London, WC1E 6DB, UK \nEmail: joel.naftalin@nhs.net\n \n \n \n \nRunning head: Ultrasound diagnosis of ACUM \n \n \nKeywords: ACUM, accessory uterus, juvenile cystic adenomyoma, Mullerian \nanomaly, painful periods, ultrasound, diagnosis \n \nThis article is protected by copyright. All rights reserved.\n\n\nContribution \n \nWhat are the novel findings of this work? \n \nACUMs are a relatively rare abnormality of the uterus and there is little \ninformation about the criteria that should be used for  diagnosis, either on \nimaging or during surgery. In this largest case series so far we provide a \nsummary of ultrasound features which could be used to diagnose this \ncondition with more confidence in the future \n \nWhat are the clinical implications of this work? \n \nThis study should further clinician knowledge of this under recognised \ncondition. It also describes a non-excisional interventional procedure, that has \nnot been widely described as being used for this condition in the literature. \nThus a greater number of women will receive a diagnosis and have a greater \nnumber of treatment options.\nThis article is protected by copyright. All rights reserved.\n\n\nAbstract \nObjective \nTo describe the clin ical and ultrasound characteristics of accessory cavitated \nuterine malformations (ACUMs). \n \nMethods \nThis was a single center observational case series of consecutive patients \ndiagnosed with ACUMs who were retrospectively identified from medical \nrecords. We identified 20 patients with an ACUM after having undergone an \nultrasound examination by an experienced ultrasound examiner between \nJanuary 2013 and May 2019.   ACUMs were diagnosed when a cavitated \nlesion with a myometrial mantle and echogenic contents w as seen within the \nanterior lateral wall of the myometrium beneath the insertion of the round \nligament.  In all women,  presenting symptoms and clinical histories were \nrecorded along with detailed descriptions of the lesions and any concomitant \npelvic abnormalities. \n \nResults \nThe median age of the 20 women diagnosed with ACUMs was 29 (inter -\nquartile range: 25- 36). None of the women were pre- menarchal or post -\nmenopausal. Twelve of the ACUMs were in the right anterior lateral \nmyometrium and eight were in the left anterior lateral myometrium. All of the \nwomen reported painful periods or pelvic pain and none of them reported \nsubfertility. Surgical excision was carried out in eight  cases and the diagnosis \nwas confirmed on histopathological examination in all of them. \n \nConclusions \nACUMs are a uterine abnormality with a dist inct ultrasound appearance , \nwhich are associated with dysmenorrhoea and chronic pelvic pain.  Its typical \nappearance on ultrasound scan could facilitate early detection and treatment. \nThere are several treatment options  ranging from simple analgesia to \ncomplete excision. Further prospective and longitudinal studies are required \nto study the prevalence and natural history and of this condition. \nThis article is protected by copyright. All rights reserved.\n\n\nIntroduction \n \nAccessory cavitated uterine malformations or masses (ACUM) are recently \ncoined names for a previously described myometrial lesion considered to be a \nsignificant and treatable cause of pelvic and menstrual pain. We prefer the \nterm malformation, which better reflects the nature of this abnormality , rather \nthan mass , which is less specific and is more likely to have s inister \nconnotations for women. This abnormality has been almost exclusively \ndescribed in young women\n1. Possible early descriptions of these anomalies \ndate back to the early 20 th century2 and up until as recently as 2012 , they \nwere termed ‘juvenile cystic adenomyomas’ (JCA) and were treated as a rare \nform of adenomyosis3.  \nThey are now considered to be a Mullerian uterine anomaly1 and treatment, in \nthe form of complete surgical excision,  is thought to be curative 4. \nNevertheless, the condition is likely to be significantly under -diagnosed, in \npart due to lack of clinician knowledge of the lesion but also because of a lack \nof agreed diagnostic criteria. The aim of this study i s to increase knowledge \nand facilitate better detection of ACUM s in the future by describing  their \nclinical symptoms and ultrasound characteristics. \n \nEpidemiology \nThe current literature on ACUMs is limited and  there are currently no \npopulation-based studies looking at ACUMs , with the published literature \nconsisting entirely of case reports and case series. The prevalence of ACUMs \nis unknown, but they are generally considered to be a rare abnormality. \nOne epidemiological feature of  ACUMs is the age of women in which they \nhave been diagnosed, with some authors stipulating that they could only be \nseen in women ≤30 years 4. There have;  however, been descri ptions of \nACUMs in women over the age of 301. \n \nMicroscopy \nMicroscopically, the cavity of the lesion is lined with functional endometrium \nconsisting of glands and stroma 4,5 and blood may be seen within the \ncavitation (Figure 1a). The endometrial tissue within the ACUMs of all patients \nin two studies was positively stained for CD10, estrogen receptors (ER) and \nprogesterone receptors (PR), which are markers of normal endometrium 4,5. In \nthe same study, t he myometrial mantle of the ACUMs contained irregularly \narranged smooth muscle cells that stained positive for desmin, ER, and PR 4. \nThe myometrium surrounding the cavitated lesion  may be hypertrophic and \nwill often contain foci of adenomyosis6.  \n \nMacroscopy \nMacroscopically, ACUMs are isolated cavitated lesions ( Figures 1b & c ) \nlocated within the lateral aspect of the myometrium, beneath the attachment This article is protected by copyright. All rights reserved.\n\n\nof the round ligament to the uterus. Their mean overall diameters have been \nreported as ranging from 25mm to 42mm and their mean inner cavity as \nranging from 10mm to 23mm. In the same study, t he weight of resected \nspecimens ranged from 3.5g to 11.5g\n4. At s urgery or on hysterectomy \nspecimen, they often appear  as a subtle bulge on the lateral surface of the \nuterus near the attachment of the round ligament  (Figure 1d) and could easily \nbe missed if not looked for. The cavities contain altered blood (Figure 1e).  \n \nClinical symptoms \nSevere pelvic pain or dysmenorrhoea, often ipsilateral to the side of the lesion \nis considered to be ubiquitous  in most studies. There is no evidence \nsuggesting ACUMs  are associated with heavy menstrual bleeding or \nsubfertility although there are no published studies that have attempted to  \nlook for such an association. There are several cases of ACUMs found in \nparous women1,2,4,6. \n \nPrognosis \nThere are no longitudinal or observational studies on ACUMs. All case series \nand most of the case reports  describe surgical excision with alleviation  of \nsymptoms and so the natural history of ACUMs  remains unknown. Studies \ndescribe an unsatisfactory  response to conservative treatments  in women \nwith ACUMs. In Acien’s case series, all four patients had inadequate pain \nrelief with NSAIDs, the oral contraceptive pill or both,  and all patients \nultimately had surgical  resection6. Takeuchi et al  described NSAIDs as \nineffective for symptom relief, the oral contraceptive pill or GnRH analogues \nas somewhat effective for pain relief  and again, all patients ultimately ended \nup having surgical resection4. There are no reports of ACUM recurrence post-\nresection. \nThis article is protected by copyright. All rights reserved.\n\n\nMethods \n \nThis was an observational case series of consecutive patients diagnosed with \nACUMs who were retrospectively reviewed from  medical records . Following \nthe coining of the term ACUM in 2012 6, we started actively looking for  cases \nof ACUM s in our general gynaecology clinic (University College London \nHospitals, London). ACUMs were diagnosed if cavitated lesions with a \nmyometrial mantle and echogenic contents , were seen in the anter ior lateral \nwall of the myometrium beneath the insertion of the round ligament  (Figures \n2, 3 & 4) . Ruling out obstructive congenital anomalies , such as \ncommunicating and non- communicating horns,  was considered crucial to \ndiagnosis. ACUMs can be differentiated from obstructive congenital \nanomalies by their absence of a connection to either the uterine cavity or to \nthe Fallopian tubes. T herefore an uninterrupted endometrial echo and two \nnormal interstitial portions of Fallopian tube s were always visualised to be \ncertain of the diagnosis  (Video 1) . Twenty women were diagnosed with \nACUMs between March 2013 and April 2019. In all cases , the diagnosis was \nconfirmed by one of two level 3 expert ultrasound examiners (JN & DJ) 7, \neither by real -time scanning or offline assessment of stored images. All the \nwomen had demographic data recorded and a detailed clinical history was \ntaken prior to undertaking the ultrasound scan. This included the history of the \npresenting complaint along with age, pregnancy history, smear history, \nsurgical and medical history.\n \n \nAll the women then underwent a conventional clinical ultrasound examination  \nby a gynaecologist with a special interest in gynaecological ultrasound, as \npart of a referral for diagnosis and/or management. All women were examined \nusing either transvaginal (TVUS) or transrectal (TRUS) ultrasound, using a 4–\n9 MHz  probe with three- dimensional facility (Voluson E8, GE Medical \nSystems, Milwaukee, WI, USA). The overall diameters of the ACUMs and \ntheir cavities were measured in three orthogonal planes and a 3D scan of the \nuterus was performed in order to classify the uterine morphology . Any \nconcomitant pelvic abnormalities diagnosed on ultrasound were recorded. \n \nSome of the women were referred for di agnosis only. In these cases we \nrequested the subsequent treatment and outcomes  from the referring \nclinicians. Where women were referred for diagnos is and management, their \ntreatments and subsequent outcomes were recorded. All clinical and \nultrasound information was entered into a dedicated Excel file ( Microsoft \nOffice Excel 2003, Redmond, WA, USA ). IRB approval was not deemed \nnecessary as there was no randomisation, no deviation from standard clinical \ncare and the study was descriptive. As the study w as retrospective, no written \npatient consent was deemed necessary. \nThis article is protected by copyright. All rights reserved.\n\n\nResults \n \nMorphology and diagnosis \nDuring the study period 20  ACUMs were identified,  all of which were found \nwithin uteri that were either normal or arcuat e according to both CUME & \nASRM8. All were isolated cavitated lesions located in the anterolateral aspects \nof the myometrium, beneath the insertion of the round ligament on the uterine \nhorn.  There were 12/20 (60%) lesions on the right and 8/20 (40%) on the left, \nwhich were all clearly delineated from the surrounding myometrium . Having \nboth a myometrial mantle and a fluid–filled cavitation, were considered to be a \ndefining features on ultrasound. T he fluid contained within the cavitation was \neither echogenic with a ‘ground glass’ appearance, identical to the classical \nappearance of the altered blood seen within endometriotic cysts (Figure 5) or \nhyperechoic (Figure 6). They were all spherical in shape ( Figure 7). The \nDoppler flow seen in the outer rim was not markedly different to that of the \nsurrounding myometrium and the contents of the cavity , being fluid, were \navascular on Doppler examination (Figure 8). \n \nThe mean outer cavity diameter of the ACUMs was 22.8mm (95%CI: \n20.9mm-24.8mm) and the mean internal cavity diameter of the ACUMs  was \n14.1mm (95%CI: 12.2mm-16.1mm). In all cases, the interstitial portions of \nboth Fallopian tubes were identified to exclude misclassification of an \nobstructive uterine anomaly as an ACUM . Table 1 outlines the other \ndiagnoses made at the time  of ultrasound  scan, including three women \ndiagnosed in early pregnancy having attended with bleeding in early \npregnancy. \nTwo women, who had only had transabdominal (TAUS) scans at their first \nattendance, were diagnosed with ACUMs at their second attendance. In both \nwomen, the ACUM was diagnosed at their first TVUS or TRUS.  \n \nSymptoms \nAll 17 of the non -pregnant patients diagnosed with ACUMs had been referred \nfor investigation of pelvic pain or painful periods. El even of them reported \nunilateral pain that was ipsilateral to the side where the ACUM was located \nand the remaining 6 patients reported more generalised pain.  None of the \npatients were referred for heavy periods or subfertility. \n \nTreatments \nThe three women who were pregnant at the time of diagnosis were managed \nexpectantly. One woman w as lost to follow up. Of the remaining sixteen  \nwomen, six opted for hormonal treatments ( 3 progesterone-only pill, 2 \ncombined hormonal contraceptive pill, and 1  Levonorgestrel-containing \nintrauterine contraceptive system ). Four women opted for transvaginal \nThis article is protected by copyright. All rights reserved.\n\n\nultrasound-guided alcohol sclerotherapy. In all four cases, cytology confirmed \nthat the ACUM contents were haemorrhagic. \n \nSix women opted for primary surgical treatment. In addition, surgery was \ncarried out in 1  woman with failed medical management and 1 in whom \nsymptoms recurred six months after sclerotherapy . In all  eight women who \nopted for surgical treatment, the excision was completed laparoscopically and \nin all cases, histopathology confirmed the preoperative diagnosis of ACUM.  \n \nHistopathology \nAll eight ACUMs that were excised were described on histopathological \nexamination as cavitated smooth muscle lesions lined by functioning \nendometrium. The myometrial mantle comprised smooth muscle fibres with a \nwell-ordered concentric orientation.  This concentric orientation can help \ndifferentiate ACUMs from adenomyosis, which will tend to have a more \ndisordered orientation of muscle fibres. \n \nDifferential diagnoses \nSeveral women that  were ultimately considered not to have ACUMs, were \nsuspected of having ACUMs by clinicians who referred to us for a second \nopinion. Figures 9, 10 & 11 illustrate examples of this and detail the r easons \nwhy they were ultimately  not diagnosed as ACUMs. In these cases, the \nlesions were thought to be focal adenomyosis, cystic adenomyomas  or a \nlateral uterine fibroid. \n \nDemographics \nThe median age of the women diagnosed with ACUMs was 29.2 (inter -\nquartile range: 25- 35.8). None of the women were pre- menarchal or post -\nmenopausal and i n three cases the ACUMs were diagnosed in the first \ntrimester of pregnancy. All of the women in whom ACUMs were diagnosed  \noutside pregnancy had been referred for investigation of pelvic pain and all \nthree of the women who were diagnosed during  pregnancy described a \nhistory of painful periods or had previously been investigated or treated for  \npelvic pain. Of the 20 women  in the study , 13 were nulligravid and 16 were \nnulliparous. Table 2 outlines the obstetric history of the 5 women who had had \npregnancies prior to diagnosis.  \n \nPregnancy \nNone of the patients who were pregnant at the time of diagnosis  reported a \nhistory of difficulty conceiving , with one pregnancy being unplanned. The two \nwomen with ACUMs who had planned conceptions both had an \nThis article is protected by copyright. All rights reserved.\n\n\nuncomplicated antenatal course and had vaginal deliveries at term. The \npatient with an unplanned pregnancy had a first trimester miscarriage. \nThis article is protected by copyright. All rights reserved.\n\n\nDiscussion \nThis study represents the largest case series of ACUMs in the literature to \ndate. The morphological appearance of the ACUMs  in this series are \nconsistent with what is already described in the literature, in that they were all \nisolated cavitated lesions with a myometrial mantle and echogenic contents, \nlocated in the anter ior lateral aspects of the myometrium , beneath the \ninsertion of the round ligament.   ACUMs appear to have a distinctive and \nconsistent morphological appearance dating back to what appears to be their \noriginal description in 19122.  \n \nTerminology \nACUMs are also referred to in the literature as juvenile cystic adenomyomas \n(JCAs). As discussed by A cien et al in their 201 0 paper, we would consider \nthese names synonymous 1. There are several reasons why we use ACUM \ninstead of JCA. The word ‘juvenile’ to describe a lesion that can be found in \nadults could be a cause of confusion. While there is clear overlap between \nACUMs and adenomyosis with both being myometrial pathologies where \nthere are endometrial glands and stroma within the myometrium , there are \nalso many features of ACUMs  that make them distinct  from adenomyosis. \nACUMs have a very consistent appearance, in contrast to the highly variable  \nmorphological appearances  of adenomyosis , and ACUMs appear to be  \npresent in a markedly different demographic to adenomyosis.  While there is \nno irrefutable evidence to confirm Acien et al ’s supposition that ACUMs \nrepresent a uterine malformation, we feel it has more in common with a \nmalformation than it does with adenomyosis 6. We favour the term \nmalformation rather than mass because mass can take on a sinister meaning \nfor patients  and therefore we feel that  Accessory Cavitated Uterine \nMalformation is a better term that describes what the lesion is, where it is and \nits likely aetiology. \n \nDiagnosis \nOur ultrasound findings mirror the descriptions of ACUMs seen in Magnetic \nresonance i maging ( MRI) studies5. While MRI can of course be used to  \ndiagnose ACUMs, we did not use or require MRI to diagnose any of the \nACUMs in our series. This may reflect our greater experience and confidence \nin using ultrasound, as gynecologists with a particular interest in ultrasound . \nGiven that ACUMs tend to occur in younger women ther e is likely  to be a \nproportion of patients with ACUMs who have never had penetrative vaginal \nintercourse and who therefore would not tolerate a TVUS . Transrectal \nultrasound can offer equivalent views to TVUS  in this circumstance but not all \npatients will consent to such an examination.  Acien et al state that MRI and \nhysterosalpingography ( HSG) are an essential part of the work -up for \nThis article is protected by copyright. All rights reserved.\n\n\nACUMs6. With regards to the use of HSG in the diagnosis of ACUMs, its role \nwas to prevent the misdiagnosis of obstructive anomalies as ACUMs. We feel \nthat these can be excluded on standard or 3D TVUS  and they therefore have \nno role in the diagnosis of ACUMs. \n \n \n \nSymptoms \nAll the patients in our series had a history of severe pelvic pain or painful \nperiods, with many of the subjects having had multiple surgeries to either \ninvestigate or treat their pain. This is entirely consistent with the literature \nwhere pain sy mptoms are always present in women who have been \ndiagnosed with ACUMs. The likely mechanism for this is the build- up of \nmenstrual fluid and blood in a cavitated, and therefore enclosed space, \ncausing substantially increased pressure and pain. This mechanis m for \ncausing pain is also seen in women and girls with obstructive anomalies. \nACUMs do not appear to impact on the ‘normal’ uterine cavity or the Fallopian \ntubes. There is therefore no obvious reason they should cause heavy \nmenstrual bleeding or subfertil ity. Consistent with this, none of the patients in \nour study reported heavy periods or subfertility alongside their pain \nsymptoms.   \n \nAge \nThe mean age in our series was 29.2 years of age (inter -quartile range: 25-\n35.8). This is higher than the mean age reported in other studies with \nTakeuchi et al  reporting a mean age of 25years 4. Some authors have \nsuggested diagnostic criteria should only include women up to the age of 30  \nyears, but our findings do not support that as half of our patient s were aged \n≥30 years. \n \nThere may be a variety  of reasons why the literature reports so few women \nbeing diagnosed with ACUMs after the age of 30 years.  Women are more \nlikely to develop other uterine pathologies as age increases such as uterine \nfibroids or adenomyosis 9,10. The presence of these pathologies is likely to \nmake diagnosis of ACUMs more challenging as they might obscure ACUMs \nor become of the focus of the ultrasound examination.  Pregnancy may also \nchange their appearance in a similar manner, although many c ase series \ncontain parous women \n \nAssociated abnormalities \nThis article is protected by copyright. All rights reserved.\n\n\nIn contrast to other series who reported no major gynecological pathologies in \ntheir populations of women with ACUMs 5,6, many of the women in our series \nhad concommitant pathologies, as previously detailed in Table 1.  Only one \nwoman in our study was diagnosed with endometriosis at the time of her  \nACUM diagnosis . Obstructive anomalies that cause increased retrograde \nmenstruation are associated with a higher prevalence of endometriosis . In \nACUMs however, the anomaly is cavitated and so the menstrual fluid is  \ncompletely contained within the lesion. There is therefore no currently known \nreason why there should be an increased likelihood of endometriosis  in \nwomen with ACUMs.   \n \n \n \nTreatment \nIn contrast to the published literature, where all women with ACUMs ultimately \nunderwent surgical excision,  only 8/20 ( 40%) of the patients in our series \nunderwent surgical excision and subsequent histopathological analysis of the \nACUM. Nevertheless, in the 8 patients who underwent surgical excision, the \nexcised lesions were all confirmed to be ACUMs  on histological examination. \nWe used medical treatment more liberally than other authors .  One fifth of the \nwomen in our study underwent alcohol sclerotherapy during the study period \nand none of them had intraoperative or postoperative complications. Alcohol \nsclerotherapy offers a low risk, day case intervention 11,12 that attempts to \ndestroy the functioning endometrium within the ACUM, thereby reducing the \nmonthly build-up of menstrual fluid within the ACUM cavity. All of the women \nreported a significant reduction in their pain symptoms although one of them \nsubsequently opted for surgical excision as their pain returned to its pre -\ntreatment levels 6 months after tr eatment. We found that the majority of  \npatients were keen to avoid surgery in the first instance when given the option  \nof conservative management. \n \nIn our series, all the women who underwent surgical excision had it completed \nlaparoscopically and without c omplication. Some authors have expressed a \npreference for performing open excision, the logic being that it is easier to find \nthe surgical planes of the ACUM  at open surgery6. In our experience, finding \nthe correct surgical planes laparoscopically can be a challenge and in a \nnumber of our cases, we used intraoperative transvag inal ultrasound to aid \nexcision. None of the women in our study managed non- surgically nor those \nwho had their ACUMs excised have subsequently conceived so we have no \ndata on which we could base discussions about management of future \npregnancy. Nevertheless, the procedure of ACUM excision, as mentioned \nabove, is similar to resection of a moderately  sized subserous or superficial \nThis article is protected by copyright. All rights reserved.\n\n\nintramural fibroid and extrapolation from this group would not be \nunreasonable until ACUM-specific data becomes available. \n \nHistology \nWhile histopathological examination can confirm findings consistent with an \nACUM, there are challenges  to considering histopathology as definitive in the \ndiagnosis of ACUM s. Firstly the location of ACUMs within the uterus is \nfundamental to their diagnosis but histopathological examination of an excised \nlesion is done without certain knowledge of where the lesion was excised \nfrom.  \n \nSecondly there is substantial overlap in hist ological appearance between \nACUMs and cystic adenomyomas, which also represent a cavitated \nmyometrial lesion that can be lined with endometrium. Pre -operative imaging \ntherefore becomes crucial to the diagnosis of ACUMs as it can determine the \nexact location of the lesion within the uterus. Peyron et al  report that cystic \nadenomyomas tend to involve or be in close proximity to the endometrial -\nmyometrial junction, in contrast to ACUMs , which will be more distinct from \nthe uterine cavity5. Cystic adenomyomas can be sagittal whereas ACUMs , as \nalready mentioned, are always in the lateral aspect of the myometrium.  \n \nConclusion \nIn this paper, we describe the unique morphological ultrasound appearance of \nan important but under recognised gynaecological condition. There appears to \nbe strong correlation between the presence of these morphological features \nand symptoms of severe menstrual pain and pelvic pain. We also describe a \nvariety of different treatments, which can lead to partial or complete resolution \nof sympt oms. Increased recognition and understanding of the condition has \nthe potential to help an enormous  number of women of reproductive age  \nworldwide who would otherwise suffer debilitating pelvic pain.  Further work is \nneeded to determine the prevalence and c linical impact of this condition and \nto determine the efficacy of current treatments. \nThis article is protected by copyright. All rights reserved.\n\n\nAcknowledgment \nThe authors acknowledge Miss Jackie Ross MRCOG, who provided the \naccompanying videoclip. \nThis article is protected by copyright. All rights reserved.\n\n\nReferences \n1. Acien P, Acien M, Fernandez F, Mayol MJ, Aranda I. The cavitated \naccessory uterine mass. A Mullerian anomaly in women with an \notherwise normal uterus. Obstet Gynecol 2010;116:1101-1109 \n2. Oliver J. An accessory uterus distended with menstrual fluid \nenucleated from the substance of the right broad ligament. The Lancet  \n1912; 179(4633):1609 \n3. Fisseha S, Smith Y, Kumetz L, Mueller G, Hussain H, Quint E. Cystic \nmyometrial lesion in the uterus of an adolescent girl. Fert Steril  \n2006;86(3):716-718 \n4. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, Jinushi M. \nDiagnostic, laparoscopic management, and histopathologic findings of \njuvenile cystic adenomyoma: a review of nine cases. Fert steril  \n2010;94(3):862-868 \n5. Peyron N, Jacquemier E, Charlot M, Devouassoux, Raudrant D, Golfier \nF, Rousset P. Accessory cavitated uterine mass: MRI features and \nsurgical correlations of a rare but under -recognised entity. Eur Radiol  \n2019;29(3):1144-1152 \n6. Acien P, Bataller A, Fernandez F, Acien M, Rodriguez JM, Mayol M. \nNew cases of accessory and cavitated uterine masses (ACUM): a \nsignificant cause of severe dysmenorrhea and recurrent pelvic pain in \nyoung women. Hum Reprod 2012:27(3):683-694 \n7. European and practical standards Committee. European Federation of \nSocieties for Ultrasound in Medicine and Biology (EFSUMB). \nUltraschall Med 2006: 27(1): 79-105 \n8. Ludwin A, Martins WP, Nastri CO, Ludwin I, Coelho Neto MA, Leitão \nVM, Acién M, Alcazar JL, Benacerraf B, Condous G, De Wilde RL, \nEmanuel MH, Gibbons W, Guerriero S, Hurd WW, Levine D, Lindheim \nS, Pellicer A, Petraglia F, Saridogan E. Congenital Uterine \nMalformation by Experts (CUME): better criteria for distinguishing \nbetween normal/arcuate and septate uterus? Ultrasound Obstet \nGynecol 2018 Jan;51(1):101-109 \n9. Naftalin J, Hoo, W, Pateman K, Mavrelos D, Holland T, Jurkovic D. \nHow common is adenomyosis? A prospective study of prevalence \nusing transvaginal ultrasound in a gynaecology clinic. Hum Reprod  \n2012:27(12):3432-3439 \n10. Wegienka G, Baird  DD, Hertz-Picciotto I, Harlow SD, Steege JF, Hill \nMC, et al. Self -reported heavy bleeding associated with uterine \nleiomyomata. Obstet Gynecol. 2003;101(3):431–7 \nThis article is protected by copyright. All rights reserved.\n\n\n11. Fuseini NM, Shlansky -Goldberg RD, Neff PM. Ultrasound- guided \ndrainage and sclerosis of a cystic myometrial mass. J Obstet \nGynaecol. 2017;37(1)127-8 \n12. Deblaere L, Froyman W, Van den Bosch T, Van Rompuy AS, Kaijser J, \nDeprest J,  Timmerman D. Juvenile cystic a denomyosis: A case report \nand a review of the literature. Austral J Ultrasound Medicine  \n2019:22(4)295-300 \nThis article is protected by copyright. All rights reserved.\n\n\nFigure legends \n \nFigure 1a Microscopy of a histological slice through an ACUM showing \nsmooth muscle mantle (pink) and the inner cavity lined with endometrium \n(purple). Blood is seen in the lateral aspect of the cavitation. \n \nFigure 1b  Macroscopy of an intact ACUM after laparoscopic excision \n \nFigure 1c Macroscopy of serial histological slices through a laparoscopically \nresected ACUM \n \nFigure 1d Laparoscopic image of the uterus before excision of an ACUM \n(yellow arrow). Note the ACUM bulging anterior to the right round ligament \nand its location inferior to the right Fallopian tube \n \nFigure 1e Laparoscopic image of the uterus during exci sion of the ACUM, \nafter opening of the round ligament. Note the presence of altered blood on the \nanterior surface of the ACUM \n \nFigure 2 Transverse view of uterus on transvaginal ultrasound showing \nlocation of ACUM (thick yellow arrow) relative to uterine cavity (thin yellow \narrow) and interstitial portion of the right Fallopian tube (thin white arrow) \n \nFigure 3 3D rendered coronal view of uterus on transvaginal ultrasound \nshowing location of ACUM (A) relative to uterine cavity (UC) and interstitial \nportion of the Fallopian tubes (I) \n \nFigure 4 Hand drawn illustration of uterus and ACUM from original description \npublished in the Lancet in 1912 by Oliver \n \nFigure 5 Transverse transvaginal ultrasound image of a uterus and ACUM \nwith echogenic contents of ‘groundglass’ appearance seen within the ACUM \n \nFigure 6 Transverse transvaginal ultrasound image of a uterus and ACUM \nwith hyperechoic contents seen within the ACUM \n \nFigure 7 Measurement of an ACUM in three orthogonal planes illustrating a \nspherical shape \n \nFigure 8 Transverse view of the uterus on transvaginal ultrasound showing \nDoppler examination of an ACUM, illustrating the presence of vascularity \nwithin the myometrial mantle but not within the cavitated part of the lesion \n \nThis article is protected by copyright. All rights reserved.\n\n\nFigure 9 Sagittal view of a re troverted uterus on transvaginal ultrasound \nshowing cystic adenomyosis at the fundus. Note central fundal location of \nlesion rather than the anterior lateral location typical of an ACUM. \n \nFigure 10 Sagittal view of anteverted uterus on transvaginal ultrasound \nshowing cystic adenomyoma in the anterior myometrium. Note lack of \nmyometrial mantle, direct proximity to uterine cavity, location in central \nanterior aspect of uterus and anechoic contents of lesion \n \nFigure 11 Transverse view of uterus on transvaginal  ultrasound showing \nsubserous fibroid at left posterior aspect of the uterus. Note lack of myometrial \nmantle and lack of cavitation within the fibroid \n \n \nSupplementary videoclip: Video of 3D volume acquisition of a left sided \nACUM and an otherwise morphologically normal uterus \nThis article is protected by copyright. All rights reserved.\n\n\nTable 1 Table detailing the concurrent ultrasound diagnoses made at the time of \nACUM diagnosis \nUltrasound diagnoses at the time of ACUM diagnosis Number \nACUM only 12 \nIntrauterine pregnancy 3 \nUterine fibroids 2 \nPolycystic ovarian morphology 2 \nDeep infiltrating endometriosis 1 \nThis article is protected by copyright. All rights reserved.\n\n\n \nTable 2 Table detailing the obstetric history of the five parous women diagnosed with \nACUMs \nStudy number First pregnancy Second pregnancy Third pregnancy \n1 Miscarriage Miscarriage Vaginal delivery \n3 Caesarean section Caesarean section  \n9 Caesarean section   \n13 Surgical \ntermination of \npregnancy \nVaginal delivery Vaginal delivery \n17 Miscarriage Tubal pregnancy  \nThis article is protected by copyright. All rights reserved.\n\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nA \nUC \nI \nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.\n\nAccepted Article\nThis article is protected by copyright. All rights reserved.","source_license":"public-domain-us","license_restricted":false}