Uterine rupture due to adenomyosis in an adolescent: A case report and review of literature

A 16-year-old female visited the emergency department in hypovolemic shock. The patient was obese (150 cm, 78 kg, body mass index 34.7 kg/m 2 ) and complained of dysmenorrhea that suddenly occurred a month ago. The patient was suffering from irregular menstrual period, heavy menstrual bleeding and was on ferrous sulfate medication for anemia (hemoglobin levels 9.2 g/dL). She had no history of taking other medications, including oral contraceptives or hormonal agents. The patient was a virgin. She had no other significant personal or family history or previous surgical history. Menarche began at the age of 12. Physical examination revealed a distended abdomen with diffuse abdominal tenderness. A palpable mass was not detected. Laboratory findings showed decreased hemoglobin levels (6 g/dL). LDH (1476 U/L), CA-125 (1063 U/mL), and CA19-9 (1347 U/mL) levels were significantly increased. An hCG blood test was negative. An enhanced computed tomography (CT) scan of the entire abdominal pelvic cavity revealed a 13 cm × 12.5 cm × 10 cm heterogeneously enhancing mass in the uterine corpus, suggesting a uterine malignancy (sarcomatous change of uterine myoma with lung metastasis), such as rhabdomyosarcoma or leiomyosarcoma (Figure 1 ). The chest CT also revealed enhancing lesions in the right lower lung (3.5 cm) and left lower lung (1.5 cm), suggesting metastatic lesions. Radiologic findings. A: Chest computed tomography scan revealed two nodules, suggesting metastatic lesions; B-D: Abdominal computed tomography scan demonstrated a huge mass in the uterine corpus, suggesting a uterine malignancy (B: Axial; C: Coronal; D: Sagittal).

The preoperative differential diagnosis was uterine malignancy, such as rhabdomyosarcoma or leiomyosarcoma, with lung metastasis. However, there was no histologic evidence of malignancy in the resected surgical specimen. The final diagnosis was diffuse adenomyosis with extensive hemorrhage. Histopathology after a wedge resection confirmed pulmonary endometrioma of both lung nodules.

All elevated blood tests normalized after surgery. Following surgery, radiologic studies revealed no specific abnormalities. The patient is currently taking Diengest (Visanne) and is doing well without any side effects or recurrence.

The patient underwent a total hysterectomy with bilateral salpingectomy and wedge resection of the lung nodules. Intraoperative findings revealed a 4 L blood-filled abdominal cavity, a 15 cm sized huge necrotic tumor filling the uterus with blood, and multiple uterine perforations (Figure 2 ). Operative findings. Intraoperative findings revealed a huge necrotic mass with multiple uterine perforations. Macroscopic examination of the resected surgical specimen demonstrated an enlarged uterus with extensive hemorrhagic necrosis and hematoma. There were no definite mass-like lesions in the uterine corpus. Under low-power microscopy, the uterus showed extensive necrosis from the endometrium to the entire myometrium due to hemorrhage (Figure 3 ). Pathologic evaluation revealed diffuse adenomyosis with hemorrhagic necrosis and glandular and stromal dissociation. Microscopic findings. A: Under low-power microscopy, the uterus showed diffuse adenomyosis with extensive hemorrhagic necrosis [Hematoxylin-and-eosin stain (H&E), × 20]; B: At a higher magnification, pathologic evaluation revealed glandular and stromal dissociation with necroinflammatory exudates (H&E, × 100); C: Histology of lung nodules demonstrated Müllerian type epithelium surrounded by endometrial stroma (H&E, × 100); D: Immunohistochemistry revealed positivity for ER in the tumor cells (immunohistochemistry, × 100). While there was no evidence of malignancy in the uterine specimen, histology of the two lung nodules showed hemorrhagic cystic lesions lined by the cuboidal epithelium and surrounded by hemosiderin-laden macrophages. The cuboidal epithelium showed no cytologic atypia or mitotic activity. Immunohistochemistry was positive for CD10, PAX8, ER, and PR and negative for TTF-1. Ectopic endometrial glands outside the uterine cavity confirmed a final diagnosis of pulmonary endometriosis.

Here we present an extraordinary case of spontaneous uterine rupture due to adenomyosis in a nulliparous adolescent. Uterine rupture should be considered for all female patients with adenomyosis, regardless of gestational status and history. It should be distinguished from other neoplastic conditions and early detection may lower the risk of adverse obstetric outcomes, including uterine rupture.

Adenomyosis is a gynecologic disorder, associated with a high risk of obstetric complications and adverse pregnancy outcomes[ 12 ]. Uterine rupture is an obstetric emergency with a high incidence of morbidity and mortality. It mostly occurs during the third trimester of pregnancy or delivery, with a prevalence rate of 0.05% in pregnant women[ 13 ]. A history of surgery, such as a cesarean section or myomectomy, is the most common risk factor for uterine rupture[ 14 ]. Advanced maternal age, multiparity, uterine malformation, excessive uterine pressure, and rare intrauterine manipulation are other important risk factors that may precipitate uterine rupture[ 15 ]. Spontaneous uterine rupture of an unscarred primigravid uterus is an extremely rare event[ 13 , 16 ]. Nikolaou et al [ 8 ] reported that nine of 12 cases of spontaneous uterine rupture were associated with adenomyosis. Uterine adenomyosis involves the endometrial tissue growing into the uterine muscle wall of the uterus. This can cause painful menstrual periods, heavy bleeding, and pelvic pressure or discomfort. While adenomyosis mostly occurs in adult life, it can also involve adolescents in a mild to moderate form[ 17 - 23 ]. The exact cause of adenomyosis is unknown, but hormonal imbalances, uterine abnormalities, and certain medical conditions may increase the risk of this condition. Exacoustos et al [ 17 ] suggested using ultrasound as a diagnostic tool for adenomyosis could avoid the need for histologic diagnosis and facilitate appropriate management. Adenomyosis treatment may include medication or surgery in severe cases[ 1 , 22 , 24 , 25 ]. Obesity is associated with a higher risk of endometriosis and adenomyosis, although the exact relationship is unclear. The increasing incidence of adenomyosis and endometriosis in adolescents may be due to obesity[ 26 ]. Adenomyosis and endometriosis may increase the risk of obstetric complications[ 12 ]. Obesity can increase the risk of uterine rupture by increasing uterine pressure and may complicate the diagnosis of adenomyosis due to increased estrogen levels. Hormonal imbalances and inflammation may play a role in the development of both of these conditions in obese individuals[ 27 ]. However, hormones could cause different reactions to the adenomyotic stroma, especially in pregnant individuals. The adenomyotic stroma has two response patterns to pregnancy-related hormones. One is the superficial foci of adenomyosis, which are located within the endometrium’s basal layer with no to minimal decidualization. In the second pattern, deeper adenomyosis foci could exhibit prominent decidualization[ 3 ]. Higher expression of progesterone receptors in the stromal components of adenomyosis is likely related to stromal decidualization, supporting the theory that adenomyosis is a response to progesterone during pregnancy[ 8 ]. Furthermore, abundant decidual transformation of stromal cells in adenomyosis results in atrophy and necrosis of muscle cells[ 7 ]. Necrosis of the uterine muscles causes atony and muscle cell separation, leading to life-threatening rupture[ 28 ]. This could explain the spontaneous uterine rupture due to adenomyosis in pregnant women. About 15 cases of spontaneous uterine rupture due to adenomyosis have been reported to date (Table 1 ). Azziz[ 3 ] reviewed 11 cases of uterine rupture, seven of which were associated with adenomyosis. Uccella et al [ 29 ] reviewed the literature and found that 1 in 25 reported cases of prelabor spontaneous uterine rupture involved adenomyosis. Mueller et al [ 5 ] reported a primigravida woman who experienced spontaneous uterine rupture at 18 wk of gestation due to heavily decidualized adenomyosis. Nikolaou et al [ 8 ] reported a case of rupture of an unscarred uterus caused by multiple foci of adenomyosis with a marked decidual reaction in the adenomyotic stroma. Indraccolo et al [ 9 ] also reported a woman with uterine rupture caused by adenomyosis. Cases of spontaneous uterine rupture due to adenomyosis NA: Not available. However, all previously reported cases were related to pregnancy, and the current case in a nulligravida juvenile patient is the first reported to date. The spontaneous uterine rupture in this nulligravida adolescent girl may be due to increased uterine pressure and changes in estrogen due to her obesity. We believe that transmural adenomyotic foci with significant hemorrhage and subsequent splaying of the myometrial smooth muscle fibers may have weakened the myometrium, ultimately rupturing the uterus. Uterine rupture, a rare adenomyosis complication, can be fatal if not treated immediately. It can be difficult to diagnose adenomyosis, as the symptoms are similar to those of other conditions, such as endometriosis[ 30 , 31 ]. A preoperative diagnosis of spontaneous uterine rupture is also challenging, especially in a juvenile patient with nulligravida. Unrecognized adenomyosis is particularly problematic in younger patients[ 32 ]. The standard workup for all women who present with severe dysmenorrhea and heavy menstrual bleeding should include an evaluation for adenomyosis, regardless of their age and health conditions. Regular monitoring is key to managing the risks associated with adenomyosis, especially in obese, nulliparous teenagers. Early detection may lower the risk of associated infertility and adverse obstetric outcomes, including uterine rupture[ 11 ]. The present case highlights the importance of considering a spontaneous uterine rupture diagnosis in women with a history of adenomyosis, regardless of their parity. Extensive adenomyosis may contribute to uterine wall weakness and increase the risk of uterine rupture, even in women who are not pregnant. If adenomyosis is detected early, fertility can be preserved with medical treatment and hysterectomy avoided.

Adenomyosis is a commonly encountered estrogen-dependent disease in women across the lifespan, causing heavy menstrual bleeding, intense pelvic pain, and infertility[ 1 ]. Adenomyosis is associated with an increased risk of many obstetrical complications, such as uterine rupture, postpartum hemorrhage and fetal growth restriction[ 2 ]. Spontaneous uterine rupture due to adenomyosis in an adolescent and non-gravida female is extremely rare, with no cases reported in the literature. Here, we describe a unique case of uterine rupture due to adenomyosis with coexisting pulmonary endometriosis and review previously reported, similar cases[ 3 - 11 ].

We are grateful to the patient for allowing us to use her medical records in our case report.