{"paper_id":"33c8bcc2-53f9-46b3-815e-079b876a961d","body_text":"Endometriosis is characterized by endometrial glands\nand stroma outside the uterine cavity. Endometriosis is a\ncommon condition that occurs in 6-10% of all reproductive\nage women ( 1 - 3 ). This number increases to approximately\n40% in women with infertility and nearly 75% in women\nwith complaints of chronic pelvic pain ( 4 ,  5 ). The pathogenesis of endometriosis is still debated. A well-founded\ntheory postulates that it could be caused by retrograde\nmenstruation of hormone-sensitive endometrial cells and\ntissues, which implant on peritoneal surfaces and cause an\ninflammatory response ( 6 ). The most common complaints\nassociated with endometriosis are dysmenorrhea and pelvic\npain; however, patients often present without pain and only\nwith complaints of infertility, or there is an incidental finding of an ovarian mass on imaging ( 7 ). One exceedingly\nrare, and interesting, presentation is haemorrhagic ascites.\nSince its first description in 1954 by Dr. Brews, less than\n100 cases of haemorrhagic ascites associated with endometriosis have been documented ( 8 ).\nThis article aims to present a case of a 32 year-old\nwoman who presented with recurrent haemorrhagic ascites. We will discuss the patient’s clinical course and surgical findings. A comprehensive review of the literature\non medical/surgical management of patients with this rare\nfinding will be presented.\n\nA 32-year-old nulligravida Hispanic female was referred\nto our department with complaints of general malaise, abdominal distention, loss of appetite, diffuse abdominal\npain and difficulty breathing that had worsened over the\nlast few days. She was known to have endometriosis that\nwas diagnosed at the time of an exploratory laparotomy\ndue to massive haemorrhagic ascites performed two years\nbefore. She was started on oral contraceptives at that time\nwith poor response and was subsequently treated with\nmonthly 3.75 mg leuprolide IM (Lupron®) but she selfdiscontinued the treatment due to the desire to conceive.\nThe patient provided consent for publication of the case\nreport. The IRB was consulted and the IRB committee at\nHospital Pedro Mallo, Buenos Aires, Argentina deemed\nthis work exempt of approval.\nInitial imaging with ultrasound and computed tomography (CT) scan revealed a large amount of intraperitoneal\nfluid. A paracentesis was performed that obtained 5 litres\nof thick bloody peritoneal fluid with a red blood cell count\nof >50000/µL that was negative for bacteria or malignant\ncells. The patient had symptomatic relief and was discharged\nhome after the procedure. She then returned eight days later\ncomplaining of recurrence of the same symptoms. A repeat\nultrasound was performed along with magnetic resonance\nimaging (MRI), which revealed massive ascites (Figes .1 ,  2 ).\nShe was taken to the operating room for diagnostic laparoscopy and drainage of the hemoperitoneum. Upon entry of\nthe peritoneal cavity, a large amount of bloody peritoneal\nfluid was identified. We removed ten litres of hemoperitoneum ( Fig .3 ). Extensive pelvic adhesions with complete\nobliteration of surgical planes was noted ( Fig .4 ). The pelvis was described as “frozen” due to encapsulating peritonitis that prevented the creation of surgical planes ( Fig .5 ).\nMultiple peritoneal biopsies were taken which revealed endometriotic implants ( Fig .6 ). The patient had an uneventful\npostoperative recovery and was treated with the gonadotropin-releasing hormone (GnRH) agonist triptorelin (3.75 mg\nintramuscular injection prior to discharge. At three months\nof the postoperative course, the patient was asymptomatic\nwithout recurrence of the disease.\nA large amount of intraperitoneal fluid is visualized on computed\ntomography (CT) of the abdomen and pelvis.\nMassive ascites with small intestine floating inside the peritoneal\ncavity visualized on transabdominal ultrasound.\nBloody ascites filling the abdominopelvic cavity. Note necrotic\nomental adhesions on the anterior abdominal wall.\nNote the complete obliteration of the vesicouterine space. The\nuterus is encapsulated from dense inflammatory plastic peritonitis and\ndensely adheres to the pelvic side walls.\nThe liver is encapsulated by a dense parietal peritoneal inflammation. The liver is densely adherent to the anterior abdominal wall and the\ngallbladder is not visualized.\nPeritoneal biopsy confirming the diagnosis of endometriosis. The\nendometriosis glands with periglandular endometriotic stroma that contain blood vessels are visualized.\n\nMassive ascites associated with endometriosis is extremely rare with less than 100 cases described in the literature ( 9 ). Endometriosis is a common challenging condition of reproductive-age women. The spectrum of the\ndisease ranges from asymptomatic to complete debilitation, which requires both aggressive surgical and medical\nintervention. As stated above, the most common presenting symptoms of endometriosis are dysmenorrhea and\npelvic pain. Our case of haemorrhagic ascites represents\nan incredibly rare complication associated with endometriosis. Patients with haemorrhagic ascites typically present with weeks to months of increasing abdominal pain,\nanorexia/weight loss, abdominal pain and dysmenorrhea.\nThis presentation often leads to a workup for malignancy\nas ovarian cancer was the suspected diagnosis in more\nthan half of the patients who presented with haemorrhagic\nascites ( 8 ).\nWhile the majority of patients with haemorrhagic ascites present with a gradual onset of symptoms, reports\nof acute onset of symptoms have been published. A 2013\ncase report described a 27-year-old who presented with a\none day onset of neck and flank pain, abdominal distention, light-headedness and palpitations. She was initially\nstable, but progressively decompensated and required\ntransfusion of numerous units of packed red blood cells.\nUltimately, a diagnostic paracentesis was performed and\n4.5 litres of grossly bloody ascitic fluid was removed ( 10 ).\nOur patient who presented with an acute recurrence following drainage via paracentesis provided evidence of\nhow quickly the hemoperitoneum can accumulate.\nPatients with haemorrhagic ascites often pose a difficult diagnostic dilemma on initial presentation. The different diagnosis must include large haemorrhagic ovarian\ncyst rupture, ovarian cancer, ectopic pregnancy, endometriosis, Meigs’ syndrome, trauma, or other processes that\ncould cause large hemoperitoneum. If necessary, initial\nstabilization measures with IV fluids and possible transfusion of blood products should be performed. As this presentation is so rare, no agreed upon workup is in place, but\nshould be focused on ruling out the more common causes\nof hemoperitoneum. In a review of the literature, laboratory analyses that include complete blood count (CBC),\nbasic metabolic panel (BMP), urine pregnancy and Ca-\n125 were typically performed, along with basic imaging\nwith either ultrasound, CT scan or MRI ( 8 ). Choice of\nimaging is often physician dependent; however, MRI is\nbeing used more frequently in evaluation for patients with\nthis presentation ( 11 ).\nHaemorrhagic ascites has been treated both medically\nand surgically. Medical management was attempted in\n97% of patients with the use of hormonal therapy (e.g.,\nGnRH agonist, danazol, progesterone, combination oral\ncontraceptive pills or a combination of these) ( 8 ). These\nmedications aim to inhibit ovarian functions and have\nbeen well documented to successfully treat endometriosis. Although medical management was attempted, 89%\nof patients ultimately underwent a surgical procedure ( 8 ).\nThe average volume of ascites was 4470 ± 2625 mL ( 12 ).\nA review of numerous case reports showed that patients\nunderwent a variety of surgical procedures, which varied\nfrom exploratory laparotomy with excision of an adnexal\nmass, total abdominal hysterectomy, oophorectomy, ovarian wedge biopsy, lysis of adhesions, or a combination of\nthese. Newer case reports have also been published that\nshow successful management via a laparoscopic approach,\nand one via diagnostic and therapeutic paracentesis ( 9 ,  10 ,\n 13 ). Improvements were seen with both medical and surgical management; however, as in our patient, recurrence\nis possible. The most successful treatments were bilateral\nsalpingo-oophorectomy or ovarian suppression therapy.\nBoth treatments had no recurrence of ascites ( 12 ).\nThe exact cause of haemorrhagic ascites in patients with\nendometriosis is unknown. It has been suggested that the\nascites is caused by a ruptured endometrioma or by exudation of widespread pelvic endometriosis. However, Ussia\net al. ( 12 ) reported that endometriomas were only seen in\n65% of cases, and that widespread superficial pelvic endometriosis was only associated with a minimal increase\nin peritoneal fluid and not with massive ascites. They\nhave stated that the pathophysiology is ovarian in nature\nand due to excessive ovarian transudation (e.g., similar to\nMeigs’ syndrome and Pseudo-Meigs’ syndrome). Meigs’\nsyndrome is based on the triad of an ovarian fibroma,\npleural effusion and ascites with resolution of symptoms\nafter resection of the fibroma. Pseudo-Meigs’ syndrome is\nassociated with a benign pelvic mass and a typical rightsided pleural effusion without ascites ( 14 ,  15 ). Their case\nis strengthened by a 50% recurrence rate in the setting\nof unilateral oophorectomy or cystectomy compared to\nno recurrences when a bilateral oophorectomy was performed. Patients placed on ovarian suppression therapy\nwith a GnRH agonist also had no recurrence during the\ntime they were taking the medication.\nManagement needs to take into account a patient’s\nage, surgical history, medical history and future fertility\nplans. In patients who have no desire for future fertility\nand desire definitive surgical treatment, a bilateral salpingo-oophorectomy would be most effective. Subtotal\nsurgical management (e.g., unilateral oophorectomy or\ncystectomy) alone should be avoided as the recurrence\nrate is high. Medical management with GnRH agonists\nare proven to be highly effective and should be used with\na patient who desires future fertility, and for those who\nwant to avoid surgical intervention.\n\nHaemorrhagic ascites is a poorly understood and rare\nmanifestation of pelvic endometriosis. The differential\ndiagnosis includes a variety of benign conditions, but\nmalignant pathology must be ruled out. There are no\nspecific protocols for the treatment of this rare condition. Current theories regarding the pathophysiology\npoint to the ovary and excessive ovarian transudation.\nManagement therefore involves surgical removal of\nbilateral ovaries or medical management with ovarian\nsuppression. Patients who desire future fertility should\nbe managed with a GnRH agonist. Clinicians should\nconsider endometriosis in the differential diagnosis on\nfemale patients of reproductive age who present with\nhaemorrhagic massive ascites.","source_license":"CC0","license_restricted":false}