{"paper_id":"db5d9ffa-1e78-4775-963a-087db7da171f","body_text":"CC BY-NC 4.0 Licence  ●  Copyright © 2024 EMJ   ●   December 2024  ●  EMJ\n83\nArticle\nAbdominal Cocoon and Recurrent \nHaemorrhagic Ascites, Unexpected \nFindings in Endometriosis: A Case \nReport and Review of Literature\nAuthors: *Rosheen Jamil,1 Maheen Jamil2\n1. Mayo Hospital, King Edward Medical University, Lahore, Pakistan\n2. Allama Iqbal Medical College, Jinnah Hospital, Lahore, Pakistan\n*Correspondence to rosheen.jamil.20@gmail.com\nDisclosure: The authors have declared no conflict of interest.\nReceived: 24.08.24 \nAccepted: 14.11.24\nKeywords: Abdominal cocoon, case report, endometriosis, hemoperitoneum, \nhaemorrhagic ascites, loculated ascites, recurrent massive  \nascites, sclerosing encapsulating peritonitis. \nCitation: EMJ. 2024;9[4]:83-88. https://doi.org/10.33590/emj/YRVF4649.\nAbstract\nIntroduction: The presentation of endometriosis as massive haemorrhagic ascites and the \npresence of concurrent encapsulating sclerosing peritonitis is extremely rare in medical \nliterature. This presentation warrants a strong suspicion for endometriosis especially in a \nnulliparous, reproductive-age woman. \nCase Report: A 22-year-old woman presenting with nonspecific abdominal discomfort, \ndistension, massive ascites, dysmenorrhea, anaemia, and mild weight loss is reported here.\n \nDiagnosis: Based on biopsy results at laparotomy, negative Tuberculosis cultures, a poor \nresponse to the anti-tuberculosis therapy, and, finally, an excellent response  to combined oral \ncontraceptive pills, endometriosis was confirmed as the diagnosis of exclusion in this case.\nInterventions and outcomes: The patient received medical treatment for endometriosis and \nhad an excellent response to treatment.\nMethods: The team conducted a thorough literature review on PubMed/MEDLINE, Cochrane, \nand Science Direct and shortlisted 13 highly relevant articles for the case report. The patient \nprovided informed written consent for the publication of this case report, with no patient-\nidentifying information included in the article. The figures in the case report have not been \npublished elsewhere, so the authors did not require copyright permission.\nConclusion: This is one of the few cases reported in the literature in which endometriosis \npresented with haemorrhagic ascites and sclerosing peritonitis. Endometriosis should be \nconsidered a differential diagnosis in a nulliparous, reproductive-age female who presents \nwith massive recurrent haemorrhagic ascites.\nArticle\n\n84\nEMJ  ●  December 2024  ●  Copyright © 2024 EMJ   ●   CC BY-NC 4.0 Licence\nINTRODUCTION\nEndometriosis is defined as the presence of \nendometrial glands and stroma outside the \nuterine cavity, which commonly presents \nwith subfertility and chronic pelvic pain in \nreproductive-age women. It affects roughly \n10% of the global reproductive-age women’s \npopulation. However, this percentage is \nsignificantly higher in women with chronic \npelvic pain at about 75% and in those \nwith infertility at about 40%.1 Since Brews \ndescribed the first case of endometriosis \npresenting as haemorrhagic ascites (HA)  \nin 1954, less than 100 cases have  \nbeen documented so far.2 \nThe first presentation of endometriosis as \nrecurrent HA is rare in the literature and \nwarrants strong clinical suspicion, especially \nin young, nulliparous, reproductive-age \nwomen of African descent. The criterion \nfor HA is the presence of more than 10,000 \nred blood cells per microliter. Furthermore, \nonly six cases have been reported in which \nwomen presented with both endometriosis-\nrelated ascites and encapsulating \nperitonitis, both of which were present \nin this case. Encapsulating peritonitis, \nabdominal cocoon, or frozen ascites is \ncharacterised by a fibrin membrane  \nthat entraps the bowel loops.3 \nThe authors present the case of a \n22-year-old woman with massive \nrecurrent haemorrhagic ascites, first \ndiagnosed on an abdominal ultrasound \nscan for occasional abdominal discomfort, \nabdominal distension, mild weight loss, and \na thin fibrinous membrane encasing the \nbowel loop on laparotomy. Endometriosis \nwas subsequently confirmed as the \ndiagnosis of exclusion because of this \npatient’s excellent response to the \ncombined oestrogen-progesterone oral \ncontraceptive pills. This case provides \nvaluable insights for the consideration of \nendometriosis as an important differential \ndiagnosis in the evaluation of nulliparous \nwomen of childbearing age presenting with \nmassive HA. \nCASE PRESENTATION\nThe case presented here is of a 22-year-\nold Pakistani woman who presented with \nabdominal distension, intermittent dull \nabdominal pain and discomfort, and 8 \nkg unintentional weight loss in 2 years. \nMenarche was at age 11 years. She was \nnot sexually active. She had primary \ndysmenorrhoea but regular menstrual \ncycles and no history of cyclic pelvic pain. \nThe patient had a past surgical history of \nan open laparotomy and appendectomy \nat age 12 years for acute abdominal pain; \nintra-operatively, a thin fibrinous membrane \nencasing the bowel loops was found. Based \non tuberculous suspicion, histopathology \nwas done then, but no acid-fast bacilli \nwere detected. The surgeon reported no \nsuspicion or findings of endometriosis \nduring this laparotomy. \nApart from past surgical history, there was \nno significant medical history, and she was \nnot on any regular medication apart from \nthe occasional use of ibuprofen for primary \ndysmenorrhea. On presentation, physical \nexamination revealed generalised distension \nbut no tenderness. The percussion note \nwas dull, particularly in the lower quadrants.  \nKey Points\n1. Overall, 10–15% of all reproductive-age women and 70% of women with chronic pelvic pain have  \nendometriosis. Haemorrhagic ascites as the first presentation of endometriosis is rarely reported.\n2. This is a case report describing a young woman with recurrent haemorrhagic ascites and an abdominal cocoon \nfound during laparotomy who was diagnosed with endometriosis after 18 months of anti-tuberculosis treatment.\n3. The distinct presentation of endometriosis should be considered in the differential diagnosis  \nof women of reproductive age who present with massive recurrent haemorrhagic ascites.\nArticle\n\nCC BY-NC 4.0 Licence  ●  Copyright © 2024 EMJ   ●   December 2024  ●  EMJ\n85\nArticle\nINVESTIGATIONS\nBlood analysis showed microcytic anaemia \nwith a haemoglobin of 11.5 g/dL. An \nultrasound scan of the abdomen and pelvis \nrevealed moderate to significant ascites \nin the pelvis, extending to the right and \nleft paracolic gutters. The uterus and right \nadnexa were normal; however, there was a \nhaemorrhagic cyst with internal echoes in \nthe left adnexa and the left hydrosalpinx. \nAn ultrasound-guided ascitic tap revealed \nchocolate-coloured haemorrhagic fluid. \nCytological analysis of the ascitic fluid \nshowed numerous red blood cells and \nsome lymphocytes, but there was no \nevidence of malignant cells. Fluid was \nexudative with a decreased serum ascitic-\nalbumin gradient. Cultures were negative, \nand no acid-fast bacilli were detected.  \nSerum tumour markers demonstrated no \nsignificant increase in CA-125 levels. CT of \nthe abdomen and pelvis showed moderate \nto significant loculated ascites, more \nmarked in the pelvis and extending to the \nright and left paracolic gutters. The decision \nfor exploratory laparotomy was made to \nobtain tissue biopsy, as an imaging-guided \nattempt for biopsy was considered difficult \ndue to the loculated nature of ascites \nand the presence of peritoneal adhesions \nbecause of previous laparotomy. \nFindings at laparotomy included \nmultiple adhesions, approximately 4 L \nof haemorrhagic ascites, a thin fibrinous \nmembrane encasing bowel loops, and \na normal-appearing uterus and ovaries. \nMultiple biopsies of the omentum and \novarian lining were obtained. Histopathology \nrevealed many hemosiderin-laden \nmacrophages and benign-appearing \nmesothelial cells. There was no evidence  \nof malignant cells. Cultures were negative, \nand acid-fast bacilli were not detected. \nTREATMENT\nBased on clinical suspicion and the \nincreased burden of disease in the \nregion, peritoneal tuberculosis (TB) was \nsuspected, and empiric treatment of TB \nwas started. The first-line anti-TB therapy \n(ATT) regimen continued for 1.5 years. \nHowever, after 8 months of ATT initiation, \nimaging, as shown in Figure 1, confirmed \nthe recurrence of ascites, which was \ndrained. After completing the course of \nATT, a follow-up MRI of the pelvis revealed \na left-sided large hematosalpinx with left \nparacolic haemorrhagic fluid. There was  \na small endometrioma in the left ovary  \nand bilateral haemorrhagic follicles.  \nThe uterus was unremarkable.  \nAn alternative diagnosis of endometriosis \nwas considered due to the ineffective \nresponse to ATT, the lack of objective proof \nof TB, and an indication of endometriosis \non MRI. The patient was started on \ncontinuous oestrogen-progesterone  \noral contraceptive pills. \nOUTCOME AND FOLLOW-UP\nThe patient responded well to combination \noral contraceptive pills (COCP), and \nimaging showed decreasing trends in \nintrabdominal fluid collection. The patient \nreported no active complaints and was \nasymptomatic. Continuous COCPs were \ncontinued for 1 year, and the patient \nremained amenorrhoeic. Follow-up  \nimaging revealed no evidence of ascites \nrecurrence; the patient discontinued using \nCOCPs for 3 months and began to have \nregular menstruation again. However, then \nshe started experiencing discomfort and \npain in the left lower quadrant. As shown \nin Figure 2, the abdominal ultrasound \nconfirmed the re-accumulation of fluid in \nthe left paracolic gutter, which measured \naround 370 mL. She was then placed \nback on continuous COCP and has \nreported no active complaints. She is now \nunder periodic surveillance to look for a \nrecurrence of ascites. The patient is not \ncurrently planning for a family; however, \nshe was counselled regarding potential \nfertility and pregnancy complications in  \nthe future. She was also counselled on  \nthe possibility of IVF and surgery if needed. \nOther medical or surgical treatment options \ncan also be considered depending  \non the patient’s preference.  \n\n86\nEMJ  ●  December 2024  ●  Copyright © 2024 EMJ   ●   CC BY-NC 4.0 Licence\nDISCUSSION \nEndometriosis is a benign condition in which \ncells lining the uterus, or endometrium, \ndeposit outside the uterus, causing \npain and infertility. Overall, 10–15 % of \nall reproductive-age women and 70% \nof those with chronic pelvic pain have \nendometriosis.4 Endometriosis usually \npresents with chronic, cyclical pelvic pain, \ndeep dyspareunia, and subfertility; however, \nrecurrent HA as an initial presentation of \nendometriosis is extremely rare.5  \nEndometriosis presenting as massive HA is \noften initially mistaken for peritoneal TB or \novarian or primary peritoneal malignancy as \nits symptoms of weight loss and decreased \nappetite often mimic the symptoms in these \ngynaecological malignancies.6  Additionally, \nfibroids, Meigs syndrome, benign ovarian \ntumours, and ovarian hyperstimulation \nsyndrome are among the benign \ngynaecologic diseases that have been \nlinked to ascites, which makes it challenging \nto arrive at a final diagnosis.7 In the most \nrecently published systematic review and \nmeta-analysis, the highest prevalence \nof HA was noted in nulliparous women \nof African origin. Abdominal distension, \nweight loss, abdominal pain, and abnormal \nuterine bleeding were the most common \nsymptoms. In contrast, pelvic mass was the \nmost common physical finding.8 Due to the \nunusual presentation, it is often dealt with \nas a diagnostic dilemma, leading to delayed \ntreatment and adding to the patient’s \ndistress. This case emphasises the need for \nits early recognition and treatment. \nThe exact cause of endometriosis is not \nentirely understood; however, various \ntheories exist to explain its pathophysiology. \nThe most postulated theory is that \nretrograde menstruation of oestrogen-\nsensitive endometrial cells implanting on \nperitoneal surfaces elicits an inflammatory \nresponse accompanied by angiogenesis, \nadhesions, fibrosis, scarring, and organ \ndistortion, leading to pain and infertility.7 \nFurthermore, the precise pathophysiology  \nof endometriosis causing HA and \nFigure 1: An ultrasound image of the abdomen and pelvis shows a small amount of fluid in the pelvis  \nafter laparotomy and the drainage of ascites. \nArticle\n\nCC BY-NC 4.0 Licence  ●  Copyright © 2024 EMJ   ●   December 2024  ●  EMJ\n87\nArticle\nencapsulating peritonitis is unknown; \nhowever, according to Bernstein, it may \nbe caused by irritation of the peritoneum \nby free blood released from ruptured \nendometrioma, which further enhances \nfibrosis and inflammation.9 Bloody ascites \nmay be the result of enhanced angiogenesis \nand friable soft tissue erosions on serosal or \nperitoneal surfaces, causing micro or frank \nbleeding. Bloody pleural effusions could be \nanother common finding in patients with  \nHA due to endometriosis, and the most  \nlikely cause for this is anatomical  \nabnormalities in the diaphragm.10 \nThe presentation of massive haemothorax \nis almost always in conjunction with \nmassive ascites. Many of them have \ndiaphragmatic and pleural lesions that \nneed to be surgically repaired.11 The gold \nstandard for diagnosing diaphragmatic \nendometriosis is video laparoscopy; for \nthoracic endometriosis, it is video-assisted \nthoracoscopic surgery.12 Endometriosis \nrisk factors include early age at menarche, \nshorter menstrual length, and taller height; \nrisk factors associated with a lower risk \ninclude smoking, parity, and higher BMI.13  \nA study by Kaabachi et al.14 showed a \nstatistically significant increase in the \nexpression of IL-37 mRNA in peritoneal \nfluid in women with endometriosis \ncompared to healthy controls. Moreover, \nlevels of IL-37 mRNA were directly \ncorrelated with disease severity.14 \nEncapsulating sclerosing peritonitis or \nabdominal cocoon syndrome is defined \nas a fibro-collagenous membrane \nsurrounding the small bowel or cocoon-\nlike appearance.15 According to a recent \nsystematic review by Magalhães et al.16 \non endometriosis-related ascites and \nencapsulating peritonitis, only six cases of \nendometriosis-associated encapsulating \nperitonitis can be found in the literature. The \nmost common primary cause of abdominal \ncocoon syndrome is idiopathic. However, its \nsecondary causes include endometriosis, \nperitoneal dialysis, ventriculoperitoneal \nor peritoneo-venous shunts, liver \ntransplantation, recurrent peritonitis,  \nand familial Mediterranean fever.17 A case \nreport by Yılmaz on a young female patient \nFigure 2: An ultrasound image of the abdomen and pelvis shows re-accumulation of fluid in the left paracolic \ngutter after completing the course of anti-tuberculosis therapy for 18 months, prompting a change in treatment to        \ncombination oral contraceptive pills, considering endometriosis as the primary diagnosis. \n\n\n88\nEMJ  ●  December 2024  ●  Copyright © 2024 EMJ   ●   CC BY-NC 4.0 Licence\non haemodialysis revealed diffuse peritoneal \nthickening and cocoon formation, with \nrecurrent hemoperitoneum corresponding \nwith her menstrual cycles. Endometriosis \nwas further confirmed on biopsy.18 Both \nendometriosis and encapsulating peritonitis \nwere present in the authors’ case. \nCONCLUSION\nDespite its rarity, this case highlights the \nimportance of the unique presentation of \nendometriosis that should be considered  \nin the differential diagnosis of women  \nof reproductive age presenting with  \nmassive haemorrhagic ascites.19\nReferences\n1. Gonzalez A et al. Endometriosis \npresenting as recurrent haemorrhagic \nascites: a case report and \nliterature review. Int J Fertil Steril. \n2020;14(1):72-5.\n2.  Gungor T et al. A systematic review: \nendometriosis presenting with ascites. \nArch Gynecol Obstet. 2011;283(3):513-8.\n3.  Machado NO. Sclerosing encapsulating \nperitonitis: review. Sultan Qaboos  \nUniv Med J. 2016;16(2):e142-51.\n4.  Ashkenazi MS et al. The clinical \npresentation of endometriosis and \nits association to current surgical \nstaging. Journal of Clinical Medicine. \n2023;12(7):2688.\n5.  Morgan TL et al. Endometriosis \npresenting with hemorrhagic ascites, \nsevere anemia, and shock. Am J \nEmerg Med. 2013;31(1):272.e1-3.\n6.  Ahmed U, Chughtai N. Endometriosis \npresenting as relapsing haemorrhagic \nascites in a South Asian woman: \na case report. J Pak Med Assoc. \n2024;74(1):165-8. \n \n \n \n7.  Giudice LC. Clinical practice. \nEndometriosis. N Engl J Med. \n2010;362(25):2389-98.\n8.  Wang X et al. Endometriosis presenting \nwith recurrent massive hemorrhagic \nascites and diagnosed by core needle \nbiopsy: a case report. Medicine \n(Baltimore). 2019;98(19):e15477.\n9.  Bernstein JS et al. Massive ascites  \ndue to endometriosis. Digest Dis  \nSci. 1961;6:1-6.\n10.  Chan-Tiopianco M et al. Clinical \npresentation and management of \nendometriosis-related hemorrhagic \nascites: a case report and systematic \nreview of the literature. Cureus. \n2021;13(6):e15828.\n11.  Ezemba N et al. Thoracic \nendometriosis syndrome in Nigeria:  \na single-centre experience.  \nInteract Cardiovasc Thorac  \nSurg. 2021;32(2):256-62.\n12.  Patrucco Reyes S et al. A case of \nhemothorax as manifestation of \nthoracic endometrial syndrome. J \nInvestig Med High Impact Case Rep. \n2021;9:23247096211052191.\n13.  Parasar P et al. Endometriosis: \nepidemiology, diagnosis and clinical \nmanagement. Curr Obstet Gynecol \nRep. 2017;6(1):34-41.\n14.  Kaabachi W et al. Interleukin-37 \nin endometriosis. Immunol Lett. \n2017;185:52-5.\n15.  Pokharel P et al. Abdominal cocoon \nsyndrome. J Nepal Health Res  \nCounc. 2019;17(2):264-6.\n16.  Magalhães TF et al. Ascites \nand encapsulating peritonitis in \nendometriosis: a systematic review \nwith a case report. Rev Bras Ginecol \nObstet. 2018;40(3):147-55.\n17.  Çolak Ş, Bektaş H. [Abdominal cocoon \nsyndrome: a rare cause of acute \nabdomen syndrome]. Ulus Travma Acil \nCerrahi Derg. 2019;25(6):575-9.  \n(In Turkish)\n18.  Yılmaz F et al. A case of cyclic \nhemoperitoneum in a hemodialysis \npatient: Encapsulated peritoneal \nsclerosis complicated by \nendometriosis and fungal peritonitis. \nSemin Dial. 2021;34(2):176-9.\n19.  Bignall J et al. Endometriosis: a rare \nand interesting cause of recurrent \nhaemorrhagic ascites. BMJ Case Rep. \n2014;2014:bcr2013010052.\nArticle\nFOR REPRINT QUERIES PLEASE CONTACT:   INFO@EMJREVIEWS.COM","source_license":"CC0","license_restricted":false}