Shah N*, Paranjpe SH**, Puri J***.
(* Consulting Gynecologist/Obstetrician, Railway Hospital (Byculla) and Honorary Endosopic Surgeon, Wadia Hospital, ** Director, Velankar Hospital & Paranjpe Maternity Home, Chembur, *** Consulting Gynecologist/Obstetrician.)
Juvenile cystic adenoma is an uncommon type of adenomyosis. We present here a case, which was misdiagnosed pre-operatively as a hematometra in a non-communicating horn of the unicornuate uterus. A differential of a degenerated myoma or a chocolate cyst was considered. However, upon laparoscopy, the complete excision of the lesion was done and the findings were consistent with juvenile cystic adenomyoma.
Adenomyoma is a rare tumor of the uterus. It consists of smooth muscles and endometrial glands within it. It is not conclusively known regarding the mechanism of invasion of myometrium by the endometrium. It mainly affects perimenopausal women. Also, it affects the whole uterus presenting as a diffuse disease, but may also present as a focal lesion. Juvenile cystic adenomyoma are usually seen in 13 to 20 years age group and the real incidence is still unknown. A conventional hysterosalpingogram can be considered but a magnetic resonance imaging (MRI) is usually done for confirmation.
An 18 year old girl presented to us with severe dysmenorrhea since the prior one year. She had attained menarche at the age of 15, after which the cycles were irregular for the first year but regularized later with normal flow. Dysmenorrhea during the last one year was gradually progressive, and present throughout the menstrual cycle. Medical management in the form of antispasmodics did not help in reduction of symptoms. Her systemic examination was within normal limits. On sonographic evaluation, a mass with central cystic degeneration was seen along the right lateral aspect of the uterus and medial to the right ovary. The differential diagnosis given were hematometra in the non-communicating rudimentary horn of unicornuate uterus, endometriosis, degeneration of myoma and torsion of ovarian mass. An office hysteroscopy, which showed the uterine cavity was normal and both ostia were seen.
She was posted for laparoscopy. A 10 mm supraumbilical port was used, and 3 and 5 mm ancillary ports were also inserted. On inserting the laparoscope, uterus was bulky, but normal shape with a 5 cm globular mass protruding from the uterus near the right cornual structures beside the round ligament.(figure 1) Both fallopian tubes and ovaries were normal in appearance. Diluted vasopressin was injected into the myometrium on the uterus and a transverse incision was given over the swelling with a harmonic scalpel.
Figure 1. Globular mass seen just below the round ligament.
Figure 2. Injecting vasopressin and lifting up the mass.
Figure 3. Thick chocolate material drained.
Figure 4. Complete removal of the entire adenomyoma.
Figure 5. Uterus after removal of the mass.
Presence of cysts within the myometrium is considered unusual, and cystic adenomyoma is a differential diagnosis. The exact incidence of the disease is unknown because of the rarity of the disease, underreporting, and unapproved diagnostic criteria. Recently, these adenomyomas are categorized under accessory and cavitated uterine masses. Acién et al have proposed that these type of juvenile cystic adenomyomas (JCA) should be considered as a new type of congenital mullerian anomaly because of its juvenile onset and its peculiar location which is always on the anterior wall of the uterus near the origin of the round ligament. This may be explained by the duplication of mullerian tissue at the level of the insertion of the round ligament which in turn maybe because of a defect in the female gubernaculum.
It is also suggested that these lesions should be separately classified from the rest of the mullerian anomalies since uterine cavity contour is preserved. Takeuchi et al have provided a diagnostic criteria which include complaints of severe dysmenorrhea, age less than 30 years, and a cystic lesion of greater than 1 cm independent of the uterine lumen but covered with myometrium seen by diagnostic imaging technique or intraoperatively.
In our case, sonography was suggestive of a hematometra in the non-communicating horn of the unicornuate uterus. Hysteroscopic evaluation with visualization of both ostia ruled this possibility out. Medical management for juvenile cystic adenomyoma include nonsteroidal anti-inflammatory drugs for pain relief, GnRH analogs and continuous oral contraceptive pills. Although medical management can be started, it only provides temporary and a partial relief.
The gold standard treatment for a juvenile cystic adenomyoma is complete excision of the lesion, and use of minimally invasive method has been described.
Juvenile cystic adenoma is probably more common than previously believed to be. It should be considered as a differential diagnosis in Mullerian anomalies, especially those with rudimentary horn. MRI can be used for a non-invasive diagnosis. Operative laparoscopy in the hands of an experienced surgeon is helpful. Early surgical treatment should be considered the treatment of choice as it will completely eliminate the cause of pain.
- Azziz R. Adenomyosis: Current perspectives. Obstet Gynecol Clin North Am 1989; 16(1): 221-35
- Tamai K, Koyama T, Umeoka S, Saga T, et al. Spectrum of MR features in adenomyosis. Best Pract Res Clin Obstet Gynecol 2006; 20(4) :583-602
- Buerger PT, Petzing HE. Congenital cysts of the corpus uteri. Am J Obstet Gynecol 1954;67: 143-51.
- Acién P, Acién M, Fernández F, José Mayol M, Aranda I. The cavitated accessory uterine mass: a Müllerian anomaly in women with an otherwise normal uterus. Obstet Gynecol. 2010; 116 :1101–9.
- 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. 2010; 94:862–8
- Kriplani A, Mahey R, Agarwal N, Bhatla N, Yadav R, Singh MK. Laparoscopic management of juvenile cystic adenomyoma: four cases. J Minim Invasive Gynecol. 2011; 18:343–8.
Shah N, Paranjpe SH, Puri J. Juvenile Cystic Adenomyoma Managed Laparoscopically. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/juvenile-cystic-adenomyoma-managed.html