{"paper_id":"bdc43f9c-4b9a-4cb2-a5eb-38aa3870de13","body_text":"CASE REPORT\nLaparoscopic resection of cystic adenomyosis in a teenager\nwith arcurate uterus\nElizabeth Ball & Masoud Ganji & Grace Janik &\nCharles Koh\nReceived: 28 May 2009 / Accepted: 29 June 2009 / Published online: 17 July 2009\n# Springer-V erlag 2009\nAbstract Whereas diffuse adenomyosis is a common finding\nin parous women, cystic adenomyosis causing severe dysme-\nnorrhoea is very rarely reported and possibly under-diagnosed,\nparticularly in adolescents. We report a case of juvenile cystic\nadenomyosis in a 19-year-old nulliparous patient presenting\nwith secondary dysmenorrhoea and non-cyclical pain. A\n20-mm adenomyotic cyst in th e fundal myometrium was\nsuccessfully excised laparoscopically by modified myomec-\ntomy. It is mandatory to take persistent primary and early\nsecondary dysmenorrhoea seriously, especially after poor\nresponse to medical treatment and to have a low threshold for\nfurther investigations, because a diagnosis of cystic adenomyo-\nsis requires targeted therapeutic intervention.\nKeywords Juvenile cystic adenomyosis . Adenomyoma .\nUterine cyst . Laparoscopic surgery . Arcuate uterus .\nModified myomectomy\nIntroduction\nDiffuse adenomyosis is typically present in up to 20% of\nhysterectomy specimens of parous women over the age of\n30, but circumscribed forms, such as solid or cystic\nadenomyoma, also termed cystic adenomyosis, are less\nfrequent. Cystic adenomyosis can be divided into an adult\nform, which is present in 5 –7% of hysterectomy fibroid\nuteri [ 1] and often associated with the diffuse subtype, and\na juvenile form seen in nulliparous women between 13 and\n20 years of age, for which the true incidence is not known;\nless than ten cases have been reported to date [ 2–9], most of\nthem in Japan. Although the aetiology is still a cause for\ndebate, the adult form appears to result from traumatic\nbreech of the interface between the endometrium and the\nmyometrium, for instance after uterine instrumentation\nfollowed by bleeding into dispersed endometrial glands,\nwhich become dilated and turn into cystic spaces of\ntypically less than 5 mm in diameter. In contrast, the\njuvenile form is thought to result from a congenital defect\nof the development of the Müllerian duct, which appears to\nlead to larger cystic spaces lined with endometrium between\n1a n d3c mi nd i a m e t e r[6]. The early presentation of pain\nsoon after menarche may mislead clinicians into making the\ndiagnosis of functional, primary dysmenorrhoea; further\ndifferential diagnoses include ovarian endometriomata, de-\ngenerative uterine fibroids and bicornuate uterus with a non-\ncommunicating uterine horn.\nCase\nA 19-year-old nulliparous woman presented with an\n18 months’ history of severe and worsening dysmenorrhoea\nwith cramps, which were insufficiently relieved by non-\nsteroidal anti-inflammatory medication, oral narcotics and\nthe combined oral contraceptive pill. The worst pain\noccurred on the first day of her menses. In addition, she\nalso complained of non-cyclical left-sided pelvic pain. She\nwent through the menarche when she was 13 years old.\nE. Ball ( *)\nDepartment of Obstetrics and Gynaecology,\nBart’s and the London NHS Trust, The Royal London Hospital,\nWhitechapel High Street,\nWhitechapel, London E11BB\ne-mail: Elizabeth.ball@bartsandthelondon.nhs.uk\nM. Ganji\nDepartment of Histopathology, Columbia St. Mary ’s Hospital,\nMilwaukee, WI, USA\nG. Janik\n: C. Koh\nReproductive Specialty Center, Columbia St Mary ’s Hospital,\nSuite 707, 2015 East Newport Avenue,\nMilwaukee, WI 53211, USA\nGynecol Surg (2009) 6:367 –370\nDOI 10.1007/s10397-009-0505-3\n\nPelvic examination revealed a normal vagina, vulva and\nadnexae and a normal-sized anteverted uterus containing a\n2-cm firm fundal mass.\nTrans-vaginal ultrasound performed immediately before\nthe operation demonstrated a 2-cm myometrial lesion in the\nleft lateral fundal area (Fig. 1a). Interestingly, a trans-\nvaginal ultrasound scan 3 months before showed a fluid\nlevel within the lesion. In fact, changing morphology with\nintermittent visualisation of a fluid level in cystic adeno-\nmyoma has been reported before [ 10]. Both ovaries\nappeared normal.\nHysteroscopy and operative laparoscopy were performed\non day12 of her menstrual cycle under general anaesthesia.\nLaparoscopy was carried out via direct entry. A 10-mm\nExcel Optiview® port was introduced intra-umbilically and\na further three 5-mm lateral ports were placed under vision,\none on the left side and two on the right with 6-cm distance\nin between. The lower ports were placed 1 cm medial to the\nanterior iliac spines. Operative findings included an\narcurate uterus with clearly visible ostia and bilaterally\npatent fallopian tubes, thus ruling out a non-communicating\nuterine horn. A 20-mm nodular lesion with an external\nappearance similar to that of a fibroid (Fig. 1b) was\nembedded in the uterine myometrium and located in the\nfundus towards the left side just caudally to the round\nligament. The rest of the pelvis appeared entirely normal.\nAfter vasopressin (Abraxis, Schaumburg, IL, USA) at a\ndilution of 1 U in 50 ml of normal saline was injected\nsuperficially into the lesion, a Harmonic Ace® scalpel was\nused to incise the visceral peritoneum overlying the lesion\nand the adjacent parametrium transversely. During enucle-\nation of the lesion, it was difficult to establish a clear plane\nand thus the tumour was divided and drained of brown fluid\nreminiscent of an ovarian endometrioma (Fig. 1c). There was\nFig. 1 a Trans-vaginal ultra-\nsound scan of uterus showing a\nspherical 2-cm lesion with mid-\nlevel echoes suggestive of a\nlongstanding blood collection.\nb Adenomyoma in situ prior to\nexcision. Apparent blanching\ncan be seen secondary to\nvasopressin injection. There is\nno external discoloration as\ntypically seen in prominent\nendometriotic lesions. c Intra-\noperative image of uterus show-\ning the enucleation using a\nmodified myomectomy ap-\nproach. Altered blood, similar to\nendometrioma fluid, can be seen\ndraining from cystic lesion.\nd Repair of the site of the\nresection site with interrupted\n2/0 PDS sutures\nFig. 2 a Section of adenomy-\notic cyst wall stained with\nhaematoxylin and eosin. A layer\nof inactive endothelium\n(arrows) lines the cystic space\n(×100 magnification).\nb Haematoxylin and eosin-\nstained section of myometrium\n(×400 magnification) showing\nabundant haemosiderin-laden\nhistiocytes ( arrows)\n368 Gynecol Surg (2009) 6:367 –370\n\nno continuity with the pelvic cavity. Further, careful excision\nwith the Harmonic scalpel was employed to remove the cyst\nand the surrounding fibrosis. Haemostasis was achieved with\nbipolar diathermy and the cavity was closed with a layer of\ndeep intramural and parametrial intracorporeal continuous\n2/0 PDS sutures on a CT1 needle and a superficial layer\nincluding the superficial muscularis and serosa using 3/0\nPDS sutures (Fig. 1d). The bisected specimen was removed\nthrough the umbilical port. Surgery lasted 90 min and the\noperative blood loss was less than 20 ml.\nThe patient was discharged home after 24 h. Eighteen\nmonths following the procedure, the patient remained pain\nfree and does not require any medication. Histopathology\nshowed a cystic cavity with endometrial and stromal lining,\nconfirming the presumptive diagnosis of cystic adenomyo-\nsis. In the surrounding tissue, there were no other foci of\nadenomyosis (Fig. 2a) and abundant haemosiderin within\nhistiocytes was visualised (Fig. 2b)\nDiscussion\nSince the first case report of juvenile cystic adenomyosis in\n1996 [ 2], several other cases have recently drawn attention\nto this rarely diagnosed condition. However, increased\nawareness of this condition and improved access to trans-\nvaginal ultrasound scanning, MRI and laparoscopy may\nlead to better recognition of juvenile cystic adenomyosis in\nthe future. This case emphasises the importance of linking\nthe clinical findings with repeated ultrasound imaging and\nto explore the pelvis lapa roscopically. None of the\npreoperative investigations in isolation, including MRI,\nw o u l dh a v eh e l p e dt or e a c had e f i n i t i v ed i a g n o s i s ;\nmoreover, one-off ultrasound imaging alone may have led\nto the misdiagnosis of ovarian endometrioma or intramural\nfibroid. In fact, diagnostic laparoscopy revealed a lesion\nthat was identical in external appearance with a uterine\nfibroid, and without the ultrasound scan that had demon-\nstrated a fluid level, it could have been labelled as a fibroid\nand would probably not have been removed at the time of\nsurgery. In this context, it is important to remember that\nfibroids rarely cause dysmenorrhoea [ 11], thus a painful\n‘fibroid’ may reveal itself as an adenomyoma only during\nthe operation or on histological examination.\nIt is important to state that this young woman was not\noperated on, on the basis of a radiological image, but after\n18 months of incapacitating pain, unresponsive to medication.\nThis pain is similar to pain caused by cryptomenorrhoea\nassociated with a non-communicating uterine horn. Postoper-\natively, the patient is completely pain free and successfully\npursuing her career. This patient derived optimal benefit from\nsurgery, which she would not have had from medical\ntreatment.\nIt can be tempting to label dysmenorrhoea in adolescents as\n‘functional’ and commence medical treatment. However, apart\nfrom a single patient, who responded well to combined oral\ncontraceptives [ 5], all patients, including the present,\nresponded poorly to medical treatment such as non-steroidal\nanti-inflammatory medication or gonadotropin-releasing-\nhormone analogues. In juvenile cystic adenomyosis, pelvic\nexamination may be unremarkable, and thus lack of response\nto medical treatment should trigger further investigations,\nincluding ultrasound scanning, laparoscopy and hysteroscopy.\nIn contrast to the present patient, who complained of\nsecondary dysmenorrhoea, most investigators reported primary\ndysmenorrhoea. The present case illustrates that a pre-existing\ndevelopmental cyst within the myometrium may require a\nperiod of repetitive bleeding in order to become symptomatic\nas evidenced by the histological examination revealing\nnumerous haemosiderin-laden histiocytes.\nIt is of particular interest that the present patient was\nsuffering from juvenile cystic adenomyosis in addition to\nhaving an arcuate uterus, supporting developmental aetiol-\nogy of juvenile cystic adenomyosis.\nIf there is no response to medical treatment, the optimal\ntreatment for juvenile cystic adenomyosis appears to be\nresection. The laparoscopic route is preferable to laparotomy,\nif technically feasible, in order to avoid a negative impact on\nfuture fertility through adhesions.\nIn conclusion, this case draws attention to a little known\nbut not uncommon cause for both primary and secondary\ndysmenorrhoea, which can affect young nulliparous women\nand should be included in the differential diagnosis of\ndysmenorrhoea in young women, which have been reviewed\nby Drosdzol et al. [12]. It is best investigated with ultrasound\nscanning and MRI and can be treated laparoscopically with\nthe same approach as used for myomectomy.\nAcknowledgments Dr Ball ’s Fellowship in minimal access surgery\nwas supported by the British Society for Gynaecological Endoscopy,\nUK and the Endometriosis Millennium Fund, UK.\nThe authors would like to thank Mr Jeremy Carver for preparation\nof the illustrations.\nConflict of interest There is no actual or potential conflict of\ninterest in relation to this article.\nReferences\n1. 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Ho ML et al (2008) Adenomyotic cyst of the uterus in an\nadolescent. Pediatr Radiol 38(11):1239 –1242\n9. Dogan E et al (2008) Juvenile cystic adenomyosis mimicking\nuterine malformation: a case report. Arch Gynecol Obstet 278\n(6):593–595\n10. Troiano RN, Flynn SD, McCarthy S (1998) Cystic adenomyosis\nof the uterus: MRI. J Magn Reson Imaging 8(6):1198 –1202\n11. Lippman SA et al (2003) Uterine fibroids and gynecologic pain\nsymptoms in a population-based study. Fertil Steril 80(6):1488–1494\n12. Drosdzol A, Skrzypulec V (2008) Dysmenorrhea in pediatric and\nadolescent gynaecology. Ginekol Pol 79(7):499 –503\n370 Gynecol Surg (2009) 6:367 –370","source_license":"CC0","license_restricted":false}