Surgical Management of a Juvenile Cystic Adenomyoma: A Unique Presentation of Adenomyosis

Endometriosis is characterized by the presence of endometrial glands and stroma in ectopic locations within the peritoneal cavity. More commonly, the le- sions can be found on areas such as the cul-de-sac and other pelvic parietal surfaces. Endometrial glands and stroma infiltrating the myometrium characterizes Ad- enomyosis [1,2]. Adenomyosis is usually characterized by diffuse foci of endometrial glands scattered through- out the myometrium. Meanwhile, a less common type of adenomyosis, in which the lesions are focal or local - ized in nature is referred to as anadenomyoma [3, 4]. An adenomyoma is described as a circumscribed nod - ule of hypertrophic and distorted endometrium within the myometrium [3,5]. Only a few cases in the literature have been reported with histologically confirmed endo- metrioma within the myometrium of the uterus [4, 6]. Such patients may primarily present with chronic pelvic pain among the wide spectrum of clinical syndromes in patients with endometriosis. On the other hand, there are a few case reports in the literature describing juve- nile cystic adenomyoma as a unique uterine pathology [4,6,7]. Juvenile cystic adenomyoma is basically an ade- nomyoma with a cystic component that occurs in young ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410162 • Page 2 of 3 • Afaneh et al. Obstet Gynecol Cases Rev 2020, 7:162 Figure 1: Ultrasound view of juvenile cystic adenomyoma. Figure 2: MRI view of juvenile cystic adenomyoma. Figure 3: Chocolate-like material exiting from the juvenile cystic adenomyoma encased on the posterior aspect of uterus. device placed intraoperatively. About one month fol- lowing her surgery, her pain recurred. Repeat imag- ing with transvaginal ultrasound scan (TVUS) and MRI (Figure 1 and Figure 2) suggested recurrence of her endometrioma. MRI suggested a clearly demarcated approximately 3 cm × 3 cm mass located within the myometrium. Decision was made for a second sur- gery in conjunction with a Reproductive Endocrinol- ogy and Infertility specialist. On second laparoscopy, the uterine endometrioma was excised and tech- niques included the use of monopolar energy, sharp and blunt dissection (Figure 3 and Figure 4). Choco- late-like material was noted coming out of the cavity of the endometrioma (Figure 3). The uterine cavity was ultimately not entered (Figure 5). Repair of the defect in the myometrium was closed in a two-layer closure using 2-0 and 3-0 V-lock suture (Figure 6). The Figure 4: Excision of juvenile cystic adenomyoma. Figure 5: Adenomyoma cyst bed, following excision, with no evidence of entry of the endometrial cavity. ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410162 • Page 3 of 3 • Afaneh et al. Obstet Gynecol Cases Rev 2020, 7:162 < 1 cm in diameter independent of the uterine lumen and covered by hypertrophic myometrium on diag- nostic images; and 3) associated with severe dysmen- orrheal” [5 ]. Kriplani A, et al. (2011) described lapa- roscopic management of 4 patients of juvenile cystic adenomyoma and reviewed the other case reports on the topic [3 ]. Our case report and supplementary video aims to increase awareness for such an unusu- al pathology and options for minimally invasive sur- gical management. In view of a report of 2 patients who had rupture uterus at 32 and 37 week gestation, meticulous uterine closure and avoid use of cautery should be emphasized [9 ]. In addition, such patients should be managed with maternal Fetal Medicine specialist when pregnant with a plan for an elective cesarean section.

1. Pelvic Mass. In: Hoffman BL, Schorge JO, Bradshaw KD, Halvorson LM, Schaffer JI, et al. Williams Gynecology. (3 rd edn), McGrawHill, New York. 2. Endometriosis. In: Jones Howard, Rock JA (2015) Te- Lindes Operative Gynecology. (11 th edn), Lippincott, Wil- liams & Wilkins, Philadelphia. 3. Kriplani A, Mahey R, Agarwal N, Bhatla N, Yadav R, et al. (2011) Laparoscopic management of juvenile cystic adeno- myoma: Four cases. JMIG 18: 343-348. 4. Osama Zaghmout, Omar Abuzeid, John Hebert, Mostafa Abuzeid (2017) Endometrioma embedded within the myo- metrium. American Journal of Obstetrics & Gynecology (AJOB) 34. 5. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, et al. (2010) Diagnosis, laparoscopic management, and his- topathologic findings of juvenile cystic adenomyoma: A re- view of nine cases. Fertil Steril 94: 862-868. 6. Abhishek Trehan (2014) Endometrioma contained within the broad ligament. BMJ Case Rep. 7. Younes G, Tulandi T (2018) Conservative surgery for ade- nomyosis and results: A systematic review. JMIG 25: 265- 276. 8. Tamura M, Fukaya T, Takaya R, Ip CW, Yajima A (1996) Juvenile adenomyotic cyst of the corpus uteri with dysmen- orrhea. Tohoku J Exp Med 178: 339-344. 9. Koo YJIK, Kwon YS (2011) Conservative surgical treatment combined with GnRH agonist in symptomatic uterine ade- nomyosis. Pak J Med Sci 27: 365-370. duration of surgery was 90 minutes and the estimat- ed blood loss was approximately 40 cc. The patient was discharged home on postoperative day 1 and her post-operative period was uneventful. The pathology report confirmed the presence of fragments of myo- metrium and adenomyosis. Therefore, the diagnosis of juvenile cystic adenomyoma was considered as a primary diagnosis. Post-operatively, the patient did have relief of her pain. Six weeks later, repeat trans- vaginal ultrasound revealed no evidence of recur- rence of the juvenile cystic adenomyoma. She had a follow-up appointment 8 months following her sec- ond surgery, and she continues to have relief of her previous symptoms.

Juvenile cystic adenomyoma of the uterus is a rare entity of focal adenomyoma that occurs in young pa- tients. It usually causes severe and debilitating pain. The first report describing juvenile cystic adenomy- oma was published by Tamura M, et al. in 1996 [8 ]. Takeuchi H, et al. (2010) indicated that 30 cases of juvenile cystic adenomyoma (including those in their series) have been reported in the Japanese-language publications [5 ]. They suggested the following diag- nostic criteria: “1) age < 30 years; 2) Cystic lesion of Figure 6: Two-layer repair was carried 2-0 V-lock suture.