To the Editor:
We read with great interest the article by Takeuchi et al. (1), which reports the largest series, 9 patients, who all underwent laparoscopic excision of the juvenile cystic adenomyoma (JCA) with 2- to 3-layer closure of the myometrium.
JCA is a condition characterized by the focal presence of ectopic endometrial glands and stroma within the uterine myometrium. Takeuchi et al. defined additional diagnostic criteria including an age range of menarche to age 30 with mean presenting age of 25.2 years. They also pointed out that the diagnosis should be confirmed by imaging modality of TVUS or MRI, excluding other diagnoses of obstructive uterine anomaly, adenomyosis with internal bleeding and acquired cystic lesions (1).
We want to add a new JCA case to the existing literature that was operated laparoscopically with robotic-assistance. A 15-year-old adolescent female was referred to our clinic for a six month history of intermittent episodic, right-sided periumbilical pain and severe dysmenorrhea. Studies obtained prior to her presentation included an abdominal and pelvic computed tomography scan which revealed a cystic structure measuring 2.5 cm in the right uterine wall. Concurrently, a transabdominal pelvic ultrasound demonstrated a predominantly anechoic 2.5 cm mass surrounded by myometrium.
She was initially followed conservatively and then lost to follow up for two years. A transvaginal pelvic ultrasound demonstrated a 4.76 x 3.35 x 3.0 cm round circumscribed cystic area in the right lateral wall of the uterus with hypoechoic contents consistent with the initial diagnosis of a cystic adenomyoma (figure 1).
Medical suppression with aromatase inhibitors, progestins, oral contraceptives, or GnRH agonists did not adequately control her pain for two more years; she elected to undergo robotic-assisted laparoscopic removal of the cyst.
Laparoscopically, the cyst was identified as a 4 cm protrusion from the right anterior fundal portion of the uterus (figure 2). The dissection through a 4 cm incision from the right anterior wall of the uterus was
resumed until the cyst wall was completely removed from the myometrium. The cyst was then removed in pieces through a 12-mm trocar. The uterus was closed in 4 layers (figure 3). Pathologic evaluation confirmed the diagnosis of the lesion as uterine cystic adenomyoma.
In our experience and in the literature, medical treatment was of limited usefulness (2-5). We agree that surgical excision via laparotomy or laparoscopy appears to be the most reliable method for treating JCA.
To our knowledge, this is the first case treated with robotic assistance. The robotic assistance allows higher degree of motion within the patient while offering an optimal view during dissection. We feel that this method allows for better and more efficient multilayer closure of the hysterotomy and therefore allows for increased safety in future pregnancies.
There is certainly a need in worldwide for report of more cases of juvenile cystic adenomyomas. This rare entity needs to be more clearly defined in order to develop specific treatment protocols. Currently, with such limited number of cases, it is difficult to define future clinical implication for patients specifically regarding their future reproductive potential.
Munire Erman Akar, M.D.a,b
Kristen H. Leezer, M.D.a
Tamer M. Yalcinkaya, M.D.a
aDepartment of Obstetrics and Gynecology
Wake Forest University School of Medicine
Winston Salem, North Carolina
bDepartment of Obstetrics and Gynecology
Akdeniz University School of Medicine
1. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, Jinushi M. Diagnosis, laparoscopic management and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases. Fertil Steril (2009) doi:10.1016/j.fertnstert.2009.05.010.
2. Koga K, Osuga Y, Hiroi H, Oishi H, Kugu K, Yano T, Taketani Y. Images in reproductive medicine. A case of giant cystic adenomyosis. Fertil Steril (2006)85:748-49.
3. Imaoka I, Kaji Y, Kobashi Y, Wada A, Honjo G, Hayashi M, Yoshida M, Matsuo M. Br J Radiol. (2005)78(930):558-61.
4. Kamio M, Taguchi S, Oki T, Tsuji T, Iwamoto I, Yoshinaga M et al. Isolated adenomyotic cyst associated severe dysmenorrhea. J Obstet Gynecol Res(2007):33:388-91.
5. Wang JH, Wu RJ, Xu KH, Lin J. Single large cystic adenomyoma of the uterus after cornual pregnancy and curettage. Fertil Steril (2007);88:965-7.
Published online in Fertility and Sterility doi:10.1016/j.fertnstert.2010.06.001
The Authors Respond:
We would like to express our thanks to Dr. Munire Erman Akar et al. for their interest in our previous study. They reported a case of juvenile cystic adenomyoma (JCA) in a young woman with pharmacotherapeutic refractory dysmenorrhea and with a cystic uterine lesion, which was imaged with pelvic ultrasound; a favorable result was obtained by excision of the lesion via robotic-assisted laparoscopic surgery. Interestingly, their methodology for the treatment of JCA closely resembles ours.
In our previous report, we defined the diagnosis of JCA with the following: 1) age 1 cm in diameter independent of the uterine lumen and covered by hypertrophic myometrium on diagnostic images; and 3) associated with severe dysmenorrhea (1). Although a non-communicating uterine anomaly, adenomyosis uteri, and an acquired cystic lesion of the uterus should be ruled out, it is possible to distinguish JCA from these disease solely via ultrasound (including diagnosis of presence or absence of a unilateral kidney) if the physician has identified the characteristics of JCA mentioned above. In addition, when supplementary images such as magnetic resonance image, hysterosalpingography and intravenous pyeloureterography are performed, it is thought that the differential diagnosis is simplified.
As was described by Dr. Munire Erman Akar et al., we suggest that pharmacotherapy has a minimal effect on dysmenorrhea associated with JCA and that significant improvement of symptoms is ensured by surgical excision of the cystic lesion. Moreover, it is notable that recurrences after surgical excision are extremely rare. Laparoscopic surgery is useful from the viewpoint of cosmetic results and postoperative adhesions, and this method is desirable for young women who desire to have children in the future. Although we do not have therapeutic experience with robotic surgery, we believe that the robotic surgery performed by Munire Erman Akal et al. facilitated the operator’s technique and that favorable postoperative results can frequently be obtained.
Although the diagnosis and management of JCA can be readily demonstrated, the condition remains a rather confusing disease or syndrome. A prime reason for this confusion is that the etiology of JCA is currently unclear because of its rarity. More cases must be evaluated in order to clarify the etiology; however, a MEDLINE search of the disease for similar cases, which we defined in a previous report, is difficult at present because of inconsistency in the nomenclature as well as various hypotheses regarding the etiology presented in previous reports (2-4). Therefore, further research is needed in order to define the disease and to unify nomenclature from the histopathological and clinical aspects.
Jun Kumakiri, M.D.
Mari Kitade, M.D.
Iwaho Kikuchi, M.D.
Satoru Takeda, M.D.
Department of Obstetrics and Gynecology
Juntendo University School of Medicine
1. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, Jinushi M. Diagnosis, Laparoscopic management and histopathological findings of Juvenile Cystic Adenomyoma: A review of Nine Cases. Fertil Steril. In press.
2. Tamura M, Fukaya T, Takaya R, Takaya R,IpCW, Yajima A: Juvenile Adenomyotic Cyst of the Corpus Uteri with Dysmenorrhea. Tohoku J Exp Med 1996; 178:339-44.
3. Potter DA, Schenken RS: Noncommunicating accessory uterine cavity. Fertil Steril 1998; 70:1165-6.
4. Fisseha S, Smith YR, Kumetz LM, Mueller GC, Hussain H, Quint EH. Cystic myometrial lesion in the uterus of an adolescent girl. Fertil Steril; 2006;86: 716-8.
Published online in Fertility and Sterility doi:10.1016/j.fertnstert.2010.06.002