Case Report
Spontaneous Ruptured Uterus in an Adolescent With Polycystic Ovarian Syndrome and Endometrial Hyperplasia Mary Anne Baquing, MD, and Joy Brotherton, MD, FACOG* From the Department of Obstetrics and Gynecology, Harbor-UCLA Medical Center, Torrance, California (both authors).
ABSTRACT Uterine diverticula and rudimentary horns are rare forms of uterine anomalies that occur during embryogenesis. They can communicate with the endometrial cavity and may have the potential to develop pathology. This case report presents an obese, anovulatory adolescent with polycystic ovarian syndrome who was admitted with acute abdominal pain and found to have radiological findings that were concerning for a ruptured mass contiguous with the uterine cavity, which was likely a uterine horn or diverticulum. Further evaluation revealed simple hyperplasia without atypia on endometrial sampling, supporting the surgical resection and subsequent medical management of this young patient. Journal of Minimally Invasive Gynecology (2015) 22, 1109–1112 Ó 2015 AAGL. All rights reserved. Keywords:
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Adolescent; Endometrial hyperplasia; Polycystic Ovary Syndrome; Uterine horn; Uterine diverticula; Uterine rupture
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Acute pelvic pain from adnexal and uterine masses is a condition that frequently requires emergent gynecologic consultation. However, the presence of a fluid-filled mass contiguous with the uterine cavity has not been described as the cause of acute pelvic pain. These masses, usually found to be M€ ullerian anomalies, such as uterine horns and uterine diverticula, are lined with endometrial tissue, and therefore, can theoretically develop pathology, particularly in obese, anovulatory women. Engle and Rushovic described a true uterine diverticulum as ‘‘an exceedingly rare entity,’’ when they described the second case of a nulliparous patient with a uterine diverticulum. Although uterine diverticulum are not well defined in the literature, they went on the describe ‘‘true uterine diverticulum probably arise from a localized duplication of the distal Mullerian duct on one side’’ [1].
Corresponding author: Dr. Joy Brotherton, MD, FACOG, Department of Obstetrics and Gynecology, Harbor-UCLA Medical Center, 1000 W. Carson Street, Box 3A, Torrance, CA 90509. E-mail:
[email protected] Submitted February 19, 2015. Accepted for publication May 17, 2015. Available at www.sciencedirect.com and www.jmig.org 1553-4650/$ - see front matter Ó 2015 AAGL. All rights reserved. http://dx.doi.org/10.1016/j.jmig.2015.05.014
We report on a case of a patient with polycystic ovarian syndrome (PCOS) who presented with an acute abdomen caused by a balloon-like mass communicating with the uterine cavity that was thought to have ‘‘ruptured’’ out of the fundus on imaging. Surgical and pathological evaluation revealed a likely large uterine diverticula. Case reports are considered exempt by the institutional review board by LA BioMed, our governing body. Case Report A 19-year-old virginal gravida 0 presented to the emergency department with sudden onset left lower quadrant abdominal pain associated with nausea and vomiting. The patient’s menstrual history was significant for oligomenorrhea with heavy bleeding during her infrequent menses. Her last menses was reported as being .6 months previously. She denied a history of dysmenorrhea or previous pelvic pain. The patient had a body mass index of 40 kg/m2. In addition to an acute abdomen, physical examination was significant for hirsutism, central obesity, and acanthosis nigricans in the groin and axillary areas. The patient denied any past medical or surgical history. Computed tomography (CT) of the abdomen and pelvis revealed a heterogeneous
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fluid-filled uterus with cystic pockets in the myometrium and rupture of the myometrial wall with pelvic fluid (Figs. 1 and 2). The patient’s pain was reported as 10/10; other vital signs were within normal limits. Her hemoglobin was 14.0, and a pregnancy test was negative. Due to the patient’s acute abdominal pain and imaging consistent with uterine rupture through the fundus of the uterus, a decision was made to proceed with emergent surgical management. Laparoscopic ports were placed in a traditional fashion, including a 5-mm port in the umbilicus and the left lower quadrant, approximately 3 fingerbreadths above and 2 fingerbreadths medial to the anterior superior iliac spines. A 12-mm port was placed similarly in the right lower quadrant. Diagnostic laparoscopic evaluation revealed a large, dilated thin-walled structure at the left cornual region consistent with what appeared to be a rudimentary uterine horn containing abundant hematometrium (Fig. 3). On further assessment, the thin-walled structure was inadvertently ruptured, and the dark blood contained within was evacuated with the suction irrigator (Fig. 4). The base of this balloon-like structure had a direct communication with the fundus of the uterus, and brisk bleeding was noted. The bleeding structure was then ligated by threading 0-Vicryl suture around the base. Extracorporeal knots with a closed knot pusher were then tied to compress the base. Once the bleeding was controlled, the structure was excised at the base using the Harmonic Ace (Ethicon Endo-Surgery, Inc. Cincinnati, OH). Attempts were made to imbricate the edges of the tissue with sutures, but the uterus was very soft, friable, and bled easily. Therefore, several Endoloops (Ethicon EndoSurgery, Inc. Cincinnati, OH) were placed to further debulk the base of the structure (Fig. 5). The remaining exposed tissue was presumed to be endometrium. This exposed endometrium was cauterized with the RoBi Bipolar device
Fig. 2 Computed tomography of abdomen and pelvis, coronal view with arrow indicating fluid-filled mass communicating with uterine fundus.
(Karl Storz Endoscopy America, Inc., El Segundo, CA) to prevent future bleeding into the abdomen with subsequent menses. The excised structure was removed through an Endo Catch (Covidien, Mansfield, MA) and sent for pathological evaluation. Enlarged, polycystic ovaries bilaterally and outpouchings on the uterine serosa (Fig. 6) were also noted on laparoscopy. Because of the patient’s history of long periods of anovulation and abundant material in the endometrial cavity present on the CT scan, an endometrial biopsy was performed while the patient was under anesthesia. Pathology of the excised structure revealed a thin uterine wall with a thin endometrial lining. The findings were thought to be consistent with a rudimentary uterine horn. Endometrial biopsy showed polypoid fragments with simple endometrial hyperplasia without atypia.
Fig. 1 Computed tomography of abdomen and pelvis, sagittal view with arrow indicating point of ‘‘rupture’’ at the uterine fundus.
Fig. 3 Large, thin-wall structure containing abundant hematometium attached to left cornual region.
Baquing and Brotherton.
Spontaneous Uterine Rupture in an Adolescent
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Fig. 4
Fig. 6
Deflated balloon-like structure communicating with endometrial cavity, connected at left cornual region.
Stump of uterine mass at end of procedure. Arrow points to 1 of the numerous outpouchings seen on the serosa of the uterus.
Postoperatively, several laboratory values, including glycosylated hemoglobin, thyroid-stimulating hormone, and prolactin were obtained and found to be within normal limits. The patient was started on medroxyprogesterone acetate (Depo Provera, Pfizer, New York, NY) for temporary cessation of menses. This was considered the best option by the surgeons and reproductive endocrinology specialist. There was concern that the defect created by the resected uterine horn needed to heal and could potentially bleed into the abdominal cavity if the patient menstruated. The patient had a normal renal collecting system on CT scan. Considering the pathological findings of simple hyperplasia without atypia, and the clinical diagnosis of PCOS based on history and clinical findings, the patient was sent for nutritional counseling and advised to lose weight. The pa-
tient declined oral progesterone therapy. The patient also declined a Levonorgestrel intrauterine device, and ultimately it was thought best to allow the defect in the myometrium to heal before inserting a foreign body into the uterine cavity. She instead opted to continue the medroxyprogesterone acetate. Nine months after her initial surgery, unable to tolerate an examination in clinic, the patient was taken to the operating room and underwent diagnostic hysteroscopy with dilation and curettage to further monitor her hyperplasia. Diagnostic hysteroscopy showed polypoid endometrium and multiple defects in the endometrium, which could best be described as uterine diverticula. Endometrial curettings were consistent with secretory endometrium. To date, the patient has lost 20 pounds and continues to be managed with medroxyprogesterone.
Fig. 5 Uterine fundus after structure was ligated and excised.
Discussion Congenital uterine malformations are the most common types of M€ullerian defects. The true incidence is unknown because most patients are asymptomatic, but they are more prevalent in patients with infertility [2]. Uterine horns form with abnormal fusion of paramesonephric ducts during embryogenesis. These accessory structures may or may not communicate with the uterus and can also have some functional endometrium [3,4]. Women with functional endometrium have a higher incidence of endometriosis and dysmenorrhea, as well as renal anomalies [2,3,5]. Given that uterine horns may contain functional endometrium and may or may not communicate with the uterine cavity, there is a potential risk for entrapment of hematometria and subsequent rupture of these structures. In this rare case, it was difficult to determine if the structure found in our patient was a uterine horn or an enlarged uterine diverticulum. The lack of any renal anomalies and the
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unusual findings of multiple outpouchings or diverticula seen later on hysteroscopy may favor the diagnosis of large, thinwalled uterine diverticula. Case reports exist in the literature, describing large balloon-like structures originating from the uterus in pregnant patients [6]. The described large balloonlike structures appear to be very similar to the structure seen in our patient. These case reports suggest that the balloon-like structures are actually large uterine diverticula, a rare anomaly that also results from incomplete fusion of the M€ ullerian ducts during embryogenesis. Differential diagnoses in the setting of pregnancy include uterine sacculation, a more common, thicker outpouching of the uterus, which may contain fetal or placental parts. However, in our nongravid patient, this seems a less likely differential finding. Ultimately, it is difficult to assign a definitive label to the mass resected in our patient: uterine horn versus diverticulum. At the time of laparoscopy, the serosa of the uterus had multiple irregularities. In retrospect, those irregularities were likely the outpouchings from smaller diverticula seen later on hysteroscopy (Fig. 5). Thus, the thin-walled nature of the structure and the multiple diverticuli seen on hysteroscopy may favor the diagnosis of diverticulum in our patient. Another important aspect of this case highlights the dilemma of obesity and anovulation in this young woman. Endometrial hyperplasia is defined as endometrial thickening with proliferation of irregular glands that have the potential to become malignant. Excessive exposure to estrogen, resulting from unopposed estrogen therapy or obesity, is associated with development of hyperplasia. Excess estrogen affects the hypothalamic-pituitary-ovarian axis, causing anovulation. Without a corpus luteum, there is no progesterone withdrawal–induced shedding of the endometrial lining, which therefore results in continual endometrial proliferation [3]. The true incidence of hyperplasia is difficult to quantify because of the changing prevalence of risk factors. However, the incidence of simple hyperplasia without atypia is estimated to be 142 per 100,000 woman-years [7]. In young, reproductive age women, (ages 19–39 years), PCOS is the most common cause of ovulatory dysfunction; therefore, such patients have increased risks of developing hyperplasia. Although
the overall risk of endometrial cancer is very low in this age group (1.6%), those who do have prolonged periods of unopposed estrogen should undergo endometrial evaluation [8]. The treatment for simple hyperplasia without atypia is progestin therapy. Due to the risk factors for endometrial hyperplasia in our patient, it is important to realize that the functional endometrium in rudimentary uterine horns or uterine diverticula have the potential to develop pathology. Cystic pelvic masses arising from the uterus found on imaging studies (ultrasound, magnetic resonance imaging, or CT) in patients with significant risk factors for endometrial hyperplasia should be evaluated. The acute nature and risk of rupture of these balloon-like structures suggest that if found on imaging, resection should be strongly considered. Furthermore, endometrial hyperplasia or cancer could presumably develop in such structures with functional endometrium, further supporting resection. Because endometrial hyperplasia is more likely to develop in obese patients, it is important to realize, as demonstrated by this case, that a minimally invasive approach is preferable and feasible.
References 1. Engel G, Rushovic AM. True uterine diverticulum, a partial Mullerian duct duplication? Arch Pathol Lab Med. 1984;108:734–736. 2. Jayasinghe Y, Rane A, Stalewski H, Grover S. The presentation and early diagnosis of the rudimentary uterine horn. Obstet Gynecol. 2005;105: 1456–1467. 3. Hoffman BL, Schorge J, Schaffer J, et al. Williams Gynecology. 2nd ed. Beijing, China: McGraw Hill Medical. p. 481–503. 4. Speroff L, Fritz MA. Clinical Gynecologic Endocrinology and Infertility. 8th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2011. 5. Rackow BW, Arici A. Reproductive performance of women with M€ullerian anomalies. Curr Opin Obstet Gynecol. 2007;19:229–237. 6. Rajiaj P, Eastwood KL, Gunn MLD, Manjiri D. Uterine diverticulum. Am J Obstet Gynecol. 2009;113:525–527. 7. Reed SD, Newton KM, Clinton WL, et al. Incidence of endometrial hyperplasia. Am J Obstet Gynecol. 2009;200:678.e1–678.e6. 8. Committee on Practice BulletinsdGynecology. Practice bulletin no. 136: management of abnormal uterine bleeding associated with ovulatory dysfunction. Obstet Gynecol. 2013;122:176–185.