Figure 1
Figure 3
Oblique, transabdominal US image of the pelvis depicting the uterine lesion and the normal appearing right adnexal region. A small amount of fluid is seen in the Douglas sac.
Juvenile cystic adenomyosis
SectionGenital (female) imaging
Case TypeClinical Cases
Authors
I. Santiago1; F. Cavalheiro2; P. Coelho3; M. J. Noruégas3; M.C. Sanches3
1 Hospital Infante D. Pedro - Aveiro, Portugal
2 Hospitais da Universisdade de Coimbra, Portugal
3 Hospital Pediátrico de Coimbra, Portugal
Connected authors
18 years, female
Clinical History
An 18-year-old female patient (gravida 0, para 0) presented with complaints of chronic pelvic pain and dysmenorrhoea of 4 years duration, relieved partially with NSAIDs and oral contraceptives. Patient denied vaginal discharge, gastrointestinal or urinary symptoms. Physical examination was unremarkable. Laboratory analysis revealed microcytic hypochromic anaemia, low serum iron and ferritin.
Imaging Findings
Transabdominal US revealed a 2.4cm cystic lesion in the right lateral wall of the uterus, with a fluid-fluid level (Figs 1-3). The adnexal regions were normal (Figs 3, 4). There was a small amount of fluid in the Douglas sac (Fig 3).
A pelvic MR was performed for additional characterisation. The lesion was located within the right lateral wall of the body of the uterus and was surrounded by hypointense (hypertrophied) myometrium (Figs 5, 6). Its fluid content was double-layered. The dependent layer was intermediate SI on T2WI and high SI on T1WI, representing protein-rich fluid. The nondependent layer was high SI on T2WI and intermediate SI on T1WI, representing serous fluid (Figs 5-7). These findings were compatible with subacute haemorrhage. No communication with the endometrial cavity was observed. The morphology of the uterus was preserved and two normal uterine horns were found (Fig 6).
A pelvic MR was performed for additional characterisation. The lesion was located within the right lateral wall of the body of the uterus and was surrounded by hypointense (hypertrophied) myometrium (Figs 5, 6). Its fluid content was double-layered. The dependent layer was intermediate SI on T2WI and high SI on T1WI, representing protein-rich fluid. The nondependent layer was high SI on T2WI and intermediate SI on T1WI, representing serous fluid (Figs 5-7). These findings were compatible with subacute haemorrhage. No communication with the endometrial cavity was observed. The morphology of the uterus was preserved and two normal uterine horns were found (Fig 6).
Discussion
Adenomyosis is a benign condition characterised by the invasion of ectopic tissue from the basal layer of the endometrium into the myometrium, resulting in hyperplasia of adjacent smooth muscle. Although usually resistant to hormonal stimulation, functional activity is variable and internal haemorrhage may be seen.[1] Possible causes include iatrogenic implantation, endomyometrial invagination and oestrogen stimulation of Mullerian rests within the myometrium.[1]
The disease is most frequently found in multiparous women over 30 years old [2] and classically involves the uterus in either focal or diffuse forms. Haemorrhagic cysts may be found in both classic forms of presentation but are typically smaller than 5 mm.[2] Adenomyosis presenting as a large haemorrhagic cyst is rare in adults and ever rarer in adolescents.[2] These juvenile lesions present clinically as severe dysmenorrhoea, usually soon after menarche, as seen in this patient, symptoms being attributed to intracystic bleeding and stretching of the cystic cavity. [2] Other symptoms reported include pelvic pain and menorrhagia. [2, 3] The presence of symptoms soon after menarche has led some authors to believe that it represents a congenital malformation caused by a defect in the development of mullerian structures.[3]
On US, lesions may present as homogeneous or heterogeneous cystic lesions, sometimes assumed to be adnexal.[1]
On MR, cystic adenomyosis usually presents as a complex cystic lesion with high SI on T1WI and intermediate to high SI on T2WI due to haemorrhagic or proteinaceous content. A fluid-fluid level may also be found. The inner cyst wall may present with a thin rim of low SI on both T1WI and T2WI due to haemosiderin deposition. Lesions are surrounded by T2-hypointense myometrium due to reactive hypertrophy.[1]
The differential diagnosis of juvenile cystic adenomyosis includes uterine malformations, namely types IIb (unicornuate uterus with non-communicating rudimentary horn) and Va (complete septate uterus) with non-communicating hemicavity, and leiomyoma with haemorrhagic degeneration. MR, with its high anatomic detail, is crucial for differentiation. A regular uterine morphology with normal uterine horns and no septa excludes the presence of a uterine malformation. In the case of a haemorrhagic leiomyoma, methemoglobin accumulates in obstructed veins at the periphery of the mass, producing a hyperintense on T1WI and hypointense on T2WI rim, distinct from the rim that is sometimes found on cystic adenomyomas.[1]
Medical therapy involves hormonal supression with oral contraceptives, GnRH agonists or danazol, but is only marginally effective. Surgery is usually required, conservative, minimally invasive procedures being preferred.[2, 3]
The disease is most frequently found in multiparous women over 30 years old [2] and classically involves the uterus in either focal or diffuse forms. Haemorrhagic cysts may be found in both classic forms of presentation but are typically smaller than 5 mm.[2] Adenomyosis presenting as a large haemorrhagic cyst is rare in adults and ever rarer in adolescents.[2] These juvenile lesions present clinically as severe dysmenorrhoea, usually soon after menarche, as seen in this patient, symptoms being attributed to intracystic bleeding and stretching of the cystic cavity. [2] Other symptoms reported include pelvic pain and menorrhagia. [2, 3] The presence of symptoms soon after menarche has led some authors to believe that it represents a congenital malformation caused by a defect in the development of mullerian structures.[3]
On US, lesions may present as homogeneous or heterogeneous cystic lesions, sometimes assumed to be adnexal.[1]
On MR, cystic adenomyosis usually presents as a complex cystic lesion with high SI on T1WI and intermediate to high SI on T2WI due to haemorrhagic or proteinaceous content. A fluid-fluid level may also be found. The inner cyst wall may present with a thin rim of low SI on both T1WI and T2WI due to haemosiderin deposition. Lesions are surrounded by T2-hypointense myometrium due to reactive hypertrophy.[1]
The differential diagnosis of juvenile cystic adenomyosis includes uterine malformations, namely types IIb (unicornuate uterus with non-communicating rudimentary horn) and Va (complete septate uterus) with non-communicating hemicavity, and leiomyoma with haemorrhagic degeneration. MR, with its high anatomic detail, is crucial for differentiation. A regular uterine morphology with normal uterine horns and no septa excludes the presence of a uterine malformation. In the case of a haemorrhagic leiomyoma, methemoglobin accumulates in obstructed veins at the periphery of the mass, producing a hyperintense on T1WI and hypointense on T2WI rim, distinct from the rim that is sometimes found on cystic adenomyomas.[1]
Medical therapy involves hormonal supression with oral contraceptives, GnRH agonists or danazol, but is only marginally effective. Surgery is usually required, conservative, minimally invasive procedures being preferred.[2, 3]
Differential Diagnosis List
Juvenile cystic adenomyosis
Uterine malformation Type Va (complete septate uterus) with non-communicating hemicavity
Uterine malformation type IIb (unicornuate uterus with non-communicating rudimentary horn)
Uterine leiomyoma with haemorrhagic degeneration
Final Diagnosis
Juvenile cystic adenomyosis
References
[1] M-L Ho et al (2008) Adenomyotic cyst of the uterus in an adolescent. Pediatr Radiol 38:1239-1242 (PMID: 18679611)
[2] E. Dogan; F. Gode; B. Saatli; M. Seçil (2008) Juvenile cystic adenomyosis mimicking uterine malformation:a case report. Arch Gynecol Obstet 278:593-595 (PMID: 18335227)
[3] A. Takeda; K. Sakai; T. Mitsui, H. Nakamura (2007) ) Laparoscopic management of juvenile cystic adenomyoma of the uterus: Report of two cases and review of the literature. Journal of Minimally Invasive Gynecology 14:370-374 (PMID: 17478374)
[4] F. Örs; A.S. Lev-Toaff; D. Gergín (2009) Laparoscopic management of juvenile cystic adenomyoma of the uterus: Report of two cases and review of the literature. Cystic adenomyoma: transvaginal ultrasound and MRI findings 3:68-70
Case information
| URL: | https://www.eurorad.org/case/9066 |
| DOI: | 10.1594/EURORAD/CASE.9066 |
| ISSN: | 1563-4086 |
Figure 2
Figure 4
Oblique, transabdominal US image of the pelvis depicting the normal appearing left adnexal region.
Figure 3
Figure 5
Pelvic FSE T2-weighted oblique coronal MR image, showing a cyst with a dependent, intermediate-SI layer and a nondependent high-SI layer, located within the right lateral wall of the uterine body, surrounded by hypointense myometrium.
Figure 4
Figure 6
Pelvic FSE T2-weighted oblique coronal MR image depicting the two normal uterine cornua, independent from the cystic lesion.
Figure 5
Figure 7
Pelvic TSE oblique coronal T1-weighted MR image depicting the myometrial cyst, which presents a dependent, high SI layer and a nondependent intermediate SI layer, surrounded by isointense myometrium.
Figure 6
Figure 1
Axial, transabdominal US image of the pelvis depicting a 2.4 cm heterogeneous cystic lesion within the myometrium of the right lateral wall of the uterus.
Figure 7
Figure 2
Sagital, transabdominal US image of the pelvis showing the same lesion as in Figure 1 but in the sagital plane. The normal appearing cervix is also depicted.
Figure 3
Oblique, transabdominal US image of the pelvis depicting the uterine lesion and the normal appearing right adnexal region. A small amount of fluid is seen in the Douglas sac.
Figure 4
Oblique, transabdominal US image of the pelvis depicting the normal appearing left adnexal region.
Figure 5
Pelvic FSE T2-weighted oblique coronal MR image, showing a cyst with a dependent, intermediate-SI layer and a nondependent high-SI layer, located within the right lateral wall of the uterine body, surrounded by hypointense myometrium.
Figure 6
Pelvic FSE T2-weighted oblique coronal MR image depicting the two normal uterine cornua, independent from the cystic lesion.
Figure 7
Pelvic TSE oblique coronal T1-weighted MR image depicting the myometrial cyst, which presents a dependent, high SI layer and a nondependent intermediate SI layer, surrounded by isointense myometrium.
Figure 1
Axial, transabdominal US image of the pelvis depicting a 2.4 cm heterogeneous cystic lesion within the myometrium of the right lateral wall of the uterus.
Figure 2
Sagital, transabdominal US image of the pelvis showing the same lesion as in Figure 1 but in the sagital plane. The normal appearing cervix is also depicted.