Rare complication of adenomyosis: acute purulent peritonitis and septicaemia in a young nulligravida

Adenomyosis is a benign gynaecological condition in multiparous women during their middle age commonly presenting as pelvic pain and menorrhagia. We report a case of a 27-year-old nulligravida of Asian origin from the Indian subcontinent who presented with a huge adenomyotic uterus with mild splenomegaly, and portal cavernoma having gross ascites that developed life-threatening peritonitis and septicaemia. Endometrial aspiration done showed no atypia or malignancy and upper gastrointestinal tract endoscopy done showed no dilated varices. With increasing abdominal distension, she developed tachycardia, tachypnoea, septicaemia and oliguria. Exploratory laparotomy done revealed purulent loculi, omentum and bowel adhesion around the uterus. Adnexa was not distinctly identifiable. Hysterectomy with left salpingo-oopherectomy was performed. She required ventilatory support and intensive unit care postoperatively. Histopathology examination showed adenomyosis with suppuration, right ovary endometriotic cyst and left ovary non-specific inflammation. The patient was discharged in stable condition on day 21. Adenomyosis may rarely occur in young nulligravida women as life-threatening manifestations with purulent peritonitis and septicaemia. Early exploration is crucial for diagnosis and recovery.

obstetrics, gynaecology and fertility, reproductive medicine

Adenomyosis is a benign gynaecological condition in multiparous women during their middle age commonly presenting as pelvic pain and menorrhagia. Diagnosing adenomyosis preoperatively poses a challenge owing to confounding clinical symptoms coupled with other frequently coexisting pelvic pathologies such as myomas, polyps, endometrial hyperplasia and even endometrial cancer. A rapidly growing uterine mass with the presence of ascites suggests malignancy.1 Both MRI and transvaginal ultrasound are valuable in characterising adenomyosis as they can identify myometrial abnormalities like cysts, distorted and heterogeneous myometrial echotexture and poorly defined foci of abnormal myometrial echotexture.2 Adenomyosis and leiomyomas commonly coexist in the same uterus,3 which causes diagnostic difficulty. Abscess developing in an adenomyotic uterus presenting as acute abdomen, being a rare presentation, has only been in very few reports so far.4–8 Acute abdomen caused by the rupture of an ovarian endometrial cyst has often been encountered.3 We report a rare case of adenomyosis with suppuration of the uterus presenting as acute abdomen, septicaemia and renal failure. Case presentation A 27-year-old nulliparous woman of Asian origin from the Indian subcontinent reported to our gynaecology outpatient department having dull aching pain and menorrhagia for the last 1 year. She had a fixed, ill-defined abdominal mass of 26 weeks’ size. On history, she had dull aching pain and menorrhagia for the last 1 year with no history of pathology or surgical intervention. She had menarche at the age of 14 years and had regular cycles with moderate bleeding. For the last 3 years, she was anxious to conceive. She had visited a local hospital 1 month before for evaluation of primary subfertility where she was investigated. Her scan showed uterine mass (leiomyoma with heterogeneous myometrium), presence of mild splenomegaly, and portal cavernoma (multiple collaterals noted around portal hilum) with moderate to gross ascites, confirmed by CT scan. With the above history and reports, we further evaluated her. All routine investigations like total white cell count, platelet count, and renal function test were normal except for mild anaemia and mildly raised cancer antigen-125 (value: 61.6 U/mL). MRI revealed an enlarged uterus with a big heterogeneous mass at fundus involving anterior and posterior wall (9×10.3×11.6 cm) causing significant mass effect (figure 1). On the right side of the uterus, haemorrhagic collection was noted of size 5.3×5.2×4 cm which was separate from the uterus. Gross-free fluid with multiple internal septations in the peritoneal cavity was present. Both ovaries were visualised with multiple hyperdense follicles. Few bilateral external iliac and inguinal lymph nodes were enlarged (largest 1.5×1.4 cm). Endometrial aspiration with pipelle was taken, but the histopathology report showed no evidence of malignancy and atypia. Upper gastrointestinal endoscopy done because of portal cavernoma revealed no dilated varices. Her family history was insignificant. During the evaluation, the patient requested for discharge for a few days. She reported back within 10 days of the discharge as she had developed acute pain of the abdomen, breathlessness and decreased urine output for the last 3 days. On examination, the patient had tachycardia (pulse: 130/min), tachypnoea (respiratory rate: 30/min) and decreased oxygen saturation (93% on room air) with normal chest auscultation with occasional basal crept. The abdomen was markedly distended until xiphisternum with rapidly increasing distension having tense ascites. Per speculum examination was unremarkable. On bimanual examination, a fixed abdominal mass of 26 weeks’ size was palpable. Bilateral fornices were full with abdominal distension. Adnexa could not be palpated separately. The patient’s condition deteriorated in the next 2 days as she had a low-grade fever (max 100°F), her abdominal distension increased tachycardia, tachypnoea persisted, her urine output decreased in the range of 18–20 mL/hour over 24 hours and pedal oedema developed. Preoperatively, injection of amoxicillin, potassium clavulanate and metronidazole intravenously 8 hourly was given. Ascitic tapping was done, cytology report had total leucocyte count of 24 x 109/L and polymorphs were 90%. Lactate dehydrogenase levels were high (2346 U/L) (table 1). With rising total white cell count, high C reactive protein titre (245 mg/L) and raised serum creatinine levels, oliguria was progressing. The treating gynaecologist explained to the patient’s relatives of high-risk state and our plan for emergency exploratory laparotomy. They agreed and gave consent for the lifesaving surgery and hysterectomy if needed. Table 1. | Investigation/day of admission | Preoperative report | Surgery | Postoperative report | ||| | Day 1 | Day 2 | Day 3 (day of surgery) | Day 4 | Day 10 | Day 21 | | | Haemoglobin (g/L) | 9.7 | 7.9 | 8.8 | 9.8 | 9.6 | 9.1 | | White cell count (per mm3) (109/L) | 20 310 | 32 480 | 53 120 | 46 280 | 15 490 | 5530 | | Platelet count(109/L) | 143 000 | 119 000 | 100 000 | 116 000 | 308 000 | 266 000 | | Serum creatinine (mg/dL) | 1.34 | 1.60 | 1.8 | 1.2 | 0.46 | 0.5 | | Blood urea (mg/dL) | 45.07 | 63.76 | 77.10 | 56.71 | 31.41 | 24.63 | | Ascitic fluid analysis | |||||| | Glucose (mg/dL) | 27.26 | ||||| | Protein (g/dL) | 2.94 | ||||| | Albumin (g/dL) | 1.63 | ||||| | Lactate dehydrogenase | 2436.7 U/L | 115.3 U/L | |||| | Adenosine deaminase activity | 22.1 U/L | ||||| | Ascitic fluid culture and sensitivity report | Enterococcus faecium grew in culture | ||||| | Carbohydrate antigen 19-9 | 20.2 U/mL | ||||| | Cancer antigen-125 | 61.6 U/mL | ||||| | Carcinoembryonic antigen | 1.93 ng/mL | ||||| | Beta human chorionic gonadotropin | 0.19 mIU/mL | ||||| | C reactive protein | 245 mg/L | Intraoperatively, upon opening parietal peritoneum by sharp dissection, it had inflammatory features and was densely adherent to the anterior surface of the uterus with pus-filled loculi over the uterus (figures 2–4). There were multiple pus and fluid-filled loculi of about 2000 mL seen up to the lesser omentum and the liver. Left side of the ovary seen anterior to the uterus had pus exuding from it, thus left salpingo-oophorectomy was done. The uterus showed dense adhesion of omentum and bowel on its anterior surface, multiple irregular pus-draining sites and trabecular myometrial mass protruding out of the perforations. As the uterus was deemed unsalvageable, consent for hysterectomy was taken and a total abdominal hysterectomy performed. The right ovary was not separately visualised, plastered to the adjacent structures. Abdominal layers were closed with an in situ intraperitoneal drain. Postoperatively, she was managed at the intensive care unit with inotropic support and assisted ventilation for 7 days. She underwent dialysis and plasma ultra-filtration three times as her urine output was markedly low in the first 3 days after surgery. She received a total of five blood transfusions and four doses of intravenous 20% albumin (serum albumin was 1.6 g/dl postoperatively). Higher antibiotics meropenem, teicoplanin and vancomycin (renal-adjusted doses) were started. Injection of colistin based on sensitivity report was started after the culture report of the intraperitoneal fluid showed Acinetobacter baumannii. There were three episodes of fever during the postoperative period. All cultures were negative except for blood culture that again suggested A. baumannii. On day 12 of surgery, suture removal was done that had serosanguinous discharge and gaping of the lower half of the wound. The culture sent from the wound also reported A. baumannii sensitive to colistin. Resuturing was done after the wound got healthy granulation tissue on day 18, and the patient was discharged on day 21. Stitches were removed 10 days later and the stitch line was healthy. Histopathology report (figure 5) stated chronic cervicitis, endometrium with changes due to hormone (exogenous/endogenous) myometrium–adenomyosis along with suppuration, right ovary had an endometriotic cyst, left ovary with non-specific inflammation, pus-like material specimen and decidualised stroma with inflammation. The patient was discharged in stable condition on day 21 and is doing well on follow-up until 6 months. Differential diagnosis The preoperative diagnosis of uterine mass was assumed to be malignant. Leiomyosarcoma was our most likely primary provisional diagnosis. Gross-free fluid in the peritoneal cavity with few external iliac and inguinal lymph nodes enlargement was reported. Although acute pain and abdominal distension with septicaemia following a cascade of events were causing diagnostic dilemmas, uterine malignancy in a young woman is rare. The possibility of ovarian malignancy was less as MRI had revealed normal-sized ovaries. Leiomyoma with secondary infection was thought of as another differential diagnosis as a big heterogeneous mass was present along with features of sepsis. Treatment As exploratory laparotomy was done, pus-filled loculi in the peritoneal cavity were unexpected findings. As she was a young nulligravida, the crucial decision for hysterectomy was taken on the table as the uterus was unsalvageable, to save her life. The postoperative period also was crucial as she was on the ventilator and vasopressor support for 7 days and required dialysis. Her white cell count was very high (53×109/L) indicating severe sepsis. Considering the deranged renal profile, giving higher antibiotics (meropenem, teicoplanin, vancomycin, colistin) in renal doses to combat severe sepsis was a big challenge. The expertise of the gynaecologist, anaesthetist, critical care specialist, physician and their teamwork saved the patient’s life. Outcome and follow-up The patient was discharged in stable condition on day 21 and is doing well on follow-up for last 6 months.

Adenomyosis has been characterised as the benign invasion of the endometrium into the myometrium producing a diffusely enlarged uterus that microscopically exhibits ectopic endometrial glands and stroma surrounded by the hypertrophic and hyperplastic myometrium.1 It has been a disease of the middle-aged and multiparous women (particularly with prior uterine surgeries/curettage). Adenomyosis is frequently presented as chronic pelvic pain, dysmenorrhoea, menorrhagia and sometimes incidentally diagnosed during the evaluation of subfertility.1–3 This case report highlights a rare presentation of acute abdomen in a case of adenomyosis secondary to suppurative inflammation of the endomyometrial layers. Our patient wanted to conceive and primarily came for infertility treatment but unexpected sudden complications occurred that changed the treatment priorities to lifesaving ones. Preoperatively, we suspected malignancy but postoperative histopathology reported as adenomyosis. After a thorough literature search done to date, we have come across very few similar case reports and found that our case was a nullipara and the youngest of all cases reported until now. Our patient presented with acute abdomen tachycardia, tachypnoea and a deranged renal profile with oliguria. The compression due to mass effect and septicaemia features together contributed to the clinical presentation of respiratory distress and the renal profile got deranged. In a study reported recently on infertile women, adenomyosis prevalence was 24.4% in women at least 40 years old and 22% in women less than 40 years old.4 Recently, the role of tissue injury and repair as the primary mechanism of myometrial invasion is hypothesised. Chronic peristaltic myometrial contractions may induce continuous microtrauma to the junction zone (JZ), causing inflammation which in turn promotes local increased oestrogen production, inducing a vicious cycle. A positive feedback mechanism is generated, and repeated cycles of auto-traumatisation occur due to chronic hyperperistalsis in the JZ.5 Only a few cases have been mentioned in the literature stating acute abdomen due to adenomyosis. Erguvan et al described a 54-year-old postmenopausal woman who had a 53×43 mm abscess in adenomyosis, who presented with inguinal pain, night sweats and hot flashes, and this was the first report on abscess formation in adenomyosis.6 Sahin et al reported a multiparous, perimenopausal woman with vaginal discharge and abnormal vaginal bleeding, and a 53-year-old multiparous perimenopausal woman with abnormal vaginal bleeding and pelvic pain, and hysterectomy material revealed micro-abscess formation in adenomyotic foci.7 Weng et al 8 described a case of micro-abscess in adenomyosis with sepsis (raised C reactive protein) in a nulliparous globular enlarged uterus. They hypothesised that the micro-abscess lacked a response to the antibiotics due to suboptimal concentration of antibiotics because of poor perfusion. The antibiotic therapy was ineffective, so total abdominal hysterectomy was performed. Weng et al further stated that in these cases, surgical intervention seems to be the choice for effective diagnosis and treatment when medical treatment fails.8 Cheung and Cheung have reported a case of pyoadenomyosis as a complication of adenomyosis.9 Fujino et al reported a rare case of adenomyosis having a clinical presentation of acute abdomen secondary to intraperitoneal bleed.10 Mohd Noor et al have published about the unusual presentation of the abdominal cocoon in association with adenomyosis and leiomyomata of the uterus along with endometriotic cyst; they mentioned sclerosing encapsulating peritonitis or abdominal cocoon formation involving pelvic organs and bowels of unknown aetiology. The aetiopathology in this condition is peritonitis leading to sclerosis with membrane formation. The pathological diagnosis in these patients was adenomyosis and leiomyomata of the uterus and ovarian endometriotic cyst.11 Hence, we conclude that adenomyosis, though usually considered as a disease of middle-aged perimenopausal women presenting with pain and menstrual irregularities, can also have unusual clinical presentations. This case report and discussion elucidate the unusual clinical manifestations. Patient’s perspective. Hindi language transcription “मैं ज़ैनब बोल रही हूँ। मेरी एम्स हस्पताल में तबियत ख़राब थीं। मैं एडमिट थीं। मेरे पेट में पानी भरा हुआ था, मेरे पेट में गठान थीं, एम्स हस्पताल में मेरा ईलाज़ चला, मेरे पेट में गठान थी डॉक्टरों ने मेरा ऑपरेशन किया और मैं सात रोज़ ई सी यू में भर्ती रहीं और हस्पताल में एडमिट तीन हफ़्ते रहीं, सभी डॉक्टरों को थैंक यू । मेरा ऑपरेशन करने से मेरी जान बच गई।” English language translation I am Zainab speaking. I was ill at AIIMS hospital. I was admitted there. My abdomen was full of fluid, I had a lump in my abdomen, I was treated at AIIMS hospital, I had a lump in my abdomen, doctors operated on me and I was admitted at ICU for seven days and admitted at the hospital for three weeks, thank you to all the doctors. My life was saved by doing operation upon me. Learning points. Adenomyosis is a benign gynaecological disease with an ill-defined clinical presentation, but can manifest as life-threatening complications. Gross ascites in a case of uterine mass commonly raises suspicion for underlying malignancy but could represent loculated pus collection secondary to suppurative inflammation of the uterine/bowel pathology. The decision for early exploratory laparotomy in the presence of uterine mass with gross-free fluid, acute abdomen presentation, increasing septicaemia and renal failure can clinch the diagnosis and treat the pathology. Acknowledgments We acknowledge Dr Ashwani Tandon, Additional Professor, Pathology and Lab Medicine at AIIMS Bhopal and Dr Radha Sarawagi Gupta, Additional Professor, Radiodiagnosis Department of AIIMS Bhopal for their contribution in this case. Footnotes Contributors: KP, as the head of the team and senior gynaecologist, played a key role in treatment decisions and patient care, and reviewing the manuscript. RK, as associate faculty, provided patient care, and did review of the literature, case report writing, critical reviewing and revision, as the corresponding author. BS, as faculty team member/assistant surgeon, provided patient care and reviewed the manuscript. AD, as the senior resident, was involved in the patient care, and assisted in drafting the case report, literature search and revising the manuscript. Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy. Competing interests: None declared. Provenance and peer review: Not commissioned; externally peer reviewed. Ethics statements Patient consent for publication Consent obtained directly from patient(s).

- 1. Garcia L, Isaacson K. Adenomyosis: review of the literature. J Minim Invasive Gynecol 2011;18:428–37. 10.1016/j.jmig.2011.04.004 [DOI] [PubMed] [Google Scholar] - 2. Taran FA, Stewart EA, Brucker S. Adenomyosis: epidemiology, risk factors, clinical phenotype and surgical and interventional alternatives to hysterectomy. Geburtshilfe Frauenheilkd 2013;73:924–31. 10.1055/s-0033-1350840 [DOI] [PMC free article] [PubMed] [Google Scholar] - 3. Boyd CA, Riall TS. Unexpected gynecologic findings during abdominal surgery. Curr Probl Surg 2012;49:195–251. 10.1067/j.cpsurg.2011.12.002 [DOI] [PMC free article] [PubMed] [Google Scholar] - 4. Puente JM, Fabris A, Patel J, et al. Adenomyosis in infertile women: prevalence and the role of 3D ultrasound as a marker of severity of the disease. Reprod Biol Endocrinol 2016;14:60. 10.1186/s12958-016-0185-6 [DOI] [PMC free article] [PubMed] [Google Scholar] - 5. Vannuccini S, Petraglia F. Recent advances in understanding and managing adenomyosis. F1000Res 2019;8. 10.12688/f1000research.17242.1. [Epub ahead of print: 13 03 2019]. [DOI] [PMC free article] [PubMed] [Google Scholar] - 6. Erguvan R, Meydanli MM, Alkan A, et al. Abscess in adenomyosis mimicking a malignancy in a 54-year-old woman. Infect Dis Obstet Gynecol 2003;11:59–64. 10.1155/S1064744903000085 [DOI] [PMC free article] [PubMed] [Google Scholar] - 7. Sahin B, Demirtas O, Karabulut A. Micro abscess formation in adenomyotic focus, a rare case. J Fam Med 2014;1:2 https://austinpublishinggroup.com/family-medicine/fulltext/jfm-v1-id1004.php [Google Scholar] - 8. Weng S-F, Yang S-F, Wu C-H, et al. Microabscess within adenomyosis combined with sepsis. Kaohsiung J Med Sci 2013;29:400–1. 10.1016/j.kjms.2012.11.009 [DOI] [PubMed] [Google Scholar] - 9. Cheung KW, Cheung VYT. Pyoadenomyosis: a rare complication of adenomyosis. J Obstet Gynaecol Can 2016;38:789. 10.1016/j.jogc.2016.04.098 [DOI] [PubMed] [Google Scholar] - 10. Fujino T, Watanabe T, Shinmura R, et al. Acute abdomen due to adenomyosis of the uterus: a case report. Asia Oceania J Obstet Gynaecol 1992;18:333–7. 10.1111/j.1447-0756.1992.tb00328.x [DOI] [PubMed] [Google Scholar] - 11. Mohd Noor NH, Zaki NM, Kaur G, et al. Abdominal cocoon in association with adenomyosis and leiomyomata of the uterus and endometriotic cyst : unusual presentation. Malays J Med Sci 2004;11:81–5. [PMC free article] [PubMed] [Google Scholar]