We report on hemorrhagic shock due to intracoital vaginal rupture in a sporadic case of gonadal dysgenesis with uterine and bilateral oviduct aplasia (46 XX) verifi ed during laparoscopy. Resuscitation with primary wound sutures was performed with full recovery.Prikazana je bolesnica s hemoragičnim šokom zbog intrakoitalnog razdora rodnice u rijetkom slučaju gonadalne disgeneze i maternične i obostrane aplazije jajovoda (46 XX), što je pronađeno tijekom laparoskopije. Učinjena je reanimacija i primarne šavi ozljede s potpunim oporavkom

DUBRAVKO HABEK

KREŠIMIR ŽIVKOVIĆ

MARKO DRAGIĆ

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527Case reportActa Med Croatica, 72 (2018) 527-529INTRODUCTIONLife-threatening conditions in urgent adolescent gy-necology, such as hemorrhagic shock, may be due to acute intra-abdominal hemorrhage with acute abdo-men syndrome (ectopic pregnancy, corpus luteum hemorrhage) or spontaneous or violent external hem-orrhage (metrorrhagia, intracoital rupture), and re-quire surgical therapy with resuscitation (1).Gonadal dysgenesis (GD) is one of the main causes of primary amenorrhea and is most commonly caused by Turner’s or Swyer syndrome. Ovarian, gonadal dysgenesis is a disorder of sexual development. GD is characterized by incomplete or defective formation of the gonads due to either structural or numerical anomalies of sex chromosomes or mutations in the genes involved in the development of the gonad with complex clinical fi ndings (2). müllerian duct anoma-lies are very rare in GD, such as partial or total geni-tal aplasia or complex Rokitansky-Hauser syndrome, sporadically described in the literature (3,4).We report on hemorrhagic shock due to intracoital vaginal rupture in a sporadic case of gonadal dysgene-sis with uterine and oviduct aplasia (46 XX). CASE REPORTA 21-year-old amenorrheic patient was admitted to the emergency gynecologic department because of severe vaginal bleeding. Her medical history was un-remarkable. Upon arrival, her clothes were covered with blood, as were her legs and abdomen. Th e patient was alert, with somnolence, hypotension (BP 90/50), tachycardia (140 /min), with massive arterial colpor-rhagia. Initial approach included intravenous fl uids (1000 mL Ringer lactate and 500 mL colloid solution containing 10% hydroxyethyl starch) and tranexamic acid 2 g intravenously. She reported coitus two hours before and bleeding since then. She had never men-struated and had only one gynecologic examination in her life at the age of 20 years, which was suspicious of uterine agenesis. Gynecologic pelvic examination was impossible due to pelvic pain and patient distress. Emergency ultrasound examination did not show ad-nexa and uterus but showed some fl uid in the pelvis. Physical examination showed no pubic and armpit hair and stage I mammary development on Tanner scale. She was 170 cm tall weighing 49 kg without any typical features of Turner’s syndrome. Laboratory fi ndings revealed acute severe secondary posthemor-rhagic anemia (erythrocytes 1.2x1012/L, hemoglobin 64 g/L, hematocrit 0.16 L/L) and normal coagulation parameters.HEMORRHAGIC SHOCK DUE TO INTRACOITAL VAGINAL RUPTURE IN CASES OF GONADAL DYSGENESIS (46 XX)DUBRAVKO HABEK, MARKO DRAGIĆ and KREŠIMIR ŽIVKOVIĆSveti Duh Clinical Hospital, Croatian Catholic University, Department of Obstetrics and Gynecology, Zagreb, CroatiaWe report on hemorrhagic shock due to intracoital vaginal rupture in a sporadic case of gonadal dysgenesis with uterine and bilateral oviduct aplasia (46 XX) verifi ed during laparoscopy. Resuscitation with primary wound sutures was performed with full recovery.Key words: vaginal rupture, hemorrhagic shock, gonadal dysgenesis, uterine and oviduct aplasia, ovarian hypoplasiaAddress for correspondence: Professor Dubravko Habek, MD, PhD University Department of Obstetrics and Gynecology  Sveti Duh Clinical Hospital Croatian Catholic University Sveti Duh 64  10 000 Zagreb, Croatia E-mail: dubravko.habek@os.t-com.hrD. Habek, M. Dragić, K. ŽivkovićHemorrhagic shock due to intracoital vaginal rupture in cases of gonadal dysgenesis (46 XX)Acta Med Croatica, 72 (2018) 527-529528Considering heavy vaginal bleeding and hemorrhag-ic shock, we suggested urgent operation, which was accepted by the patient. Our initial diagnosis was in-tracoital vaginal rupture due to rudimentary vagina as part of Mayer-Küstner-Hauser-Rokitansky syndrome according to patient history and primary fi ndings. Af-ter endotracheal anesthesia, we performed vaginal ex-amination, which revealed rupture of the right vaginal wall 6x2 cm with arterial hemorrhage (Fig. 1).Fig. 1. Intracoital vaginal rupture. Contrary to our expectation, vagina was normal in size (9 cm) and two transverse fi ngers in diameter. External genitals were normal. We placed sutures on vaginal wall, which stopped bleeding. Aft er optimal hemostasis was achieved, laparoscopy revealed some blood in the pelvis, uterine aplasia, fallopian tube apla-sia and hypoplastic ovaries (U5b C4 V0 by ESHRE classifi cation) (5) (Fig. 2). During the operation, the patient received 2500 mL of crystalloid solution and 750 mL of erythrocyte concentrate.Aft er initial postoperative period, we obtained de-tailed patient medical history. She had never menstru-ated, was generally of good health. She had only one gynecologic examination but she had no follow up or recommended testing aft er her fi rst visit. Her family history was inconspicuous. Her mother did not take any diethylstilbestrol during pregnancy nor received chemo- or radiotherapy. Th e patient did not suff er any hearing loss or other body anomalies. Considering uterine agenesis with hypoplastic ovaries and normal vagina, we opted to do laboratory tests and peripheral blood karyotyping (46 XX). Hormonal profi le showed hypergonadotropic hypogonadism (Table 1). We rec-ommended hormone replacement therapy, supple-mentation with vitamin D and higher Ca intake for prevention of osteoporosis. We also recommended diagnostic biopsy of streak gonads, but the patient re-fused any further investigation and treatment. On the third postoperative day, she left  the hospital healthy.Fig. 2. Laparoscopic fi ndings of uterine and bilateral oviduct aplasia.Table 1. Postoperative laboratory fi ndings Estrogen 102 pmol/L LowProgesterone 1.31 nmol/L LowProlactin 310 mIU/L NormalTestosterone 2.18 nmol/L NormalDHEA-S 11.1 μmol ElevatedFSH 46.42 U/L ElevatedLH 13.06 U/L ElevatedDHEA-S = dehydroepiandrosterone sulfate; FSH = follicle-stimu-lating hormone; LH = luteinizing hormoneDISCUSSIONVerifi ed normal karyotype (46 XX) with hypergo-nadotropic hypogonadism and U5b C4 V0 classifi ed anomaly indicated diagnosis of sporadic ovarian dys-genesis, also known as pure ovarian dysgenesis with primary amenorrhea and müllerian duct anomalies in our case (3,5). Anecdotally, intraoperative (for vital in-dications) fi ndings verifi ed uterine and fallopian tube aplasia with hypoplastic ovaries. Th e association of gonadal dysgenesis with aplastic or hypoplastic mülle-rian duct anomalies is extremely rare (3,4).D. Habek, M. Dragić, K. ŽivkovićHemorrhagic shock due to intracoital vaginal rupture in cases of gonadal dysgenesis (46 XX)Acta Med Croatica, 72 (2018) 527-529529Th ere are very few case reports of XX 46 gonadal dys-genesis and most of them are related to familial gonad-al dysgenesis. According to Pertusa and Palacios, the most important issues are long-term lack of estrogen and sterility (6). Sterility in most cases remains un-solved issue. Lack of estrogen is preventable with hor-mone therapy, however, early osteoporosis remains an important issue. According to Ropke et al., important part of further management is ovarian biopsy because ovarian tissue can have diff erent karyotype than pe-ripheral blood karyotype, thus carrying an increased risk of dysgerminoma (7). Th e highest risk of ovari-an dysgerminoma is in 46 XY ovarian dysgenesis be-cause of Y region. Th e risk of ovarian dysgerminoma in XX 46 ovarian dysgenesis is unknown and rarely described due to very few cases reported. To conclude, there is no evidence-based recommendation for early gonadectomy in 46 XX ovarian dysgenesis, however, a reasonable approach would be to perform ovarian biopsy and ultrasound follow up once a year (8,9). Possible concomitant hypoplasia of other female gen-italia can be the cause of intracoital rupture of vagina and hemorrhagic shock development, although in this case the vagina was of normal dimensions. A rare case of concomitant genital tract anomalies with ovarian dysgenesis is presented. R  E  F  E  R  E  N  C  E  S1. Habek D, Karša M, Kalajžić T. Clinical evaluation of pae-diatric and adolescent gynecology in Clinical Hospital Osijek. Gynaecol Perinatol 2005; 14: 145-8.2. McCann-Crosby B, Mansouri R, Dietrich JE et al. State of the art review in gonadal dysgenesis: challenges in diagnosis and management. Int J Pediatr Endocrinol 2014; 2014 (1): 4. doi: 10.1186/1687-9856-2014-4. Epub 2014 Apr 14.3. Oppelt PG, Lermann J, Strick R et al. Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). Reprod Biol Endocrinol 2012; 57: 4-7. 4. Bousfi ha N, Errarhay S, Saadi H, Ouldim K, Bouchikhi C, Banani C. Gonadal dysgenesis 46, XX associated with May-er-Rokitansky-Küster-Hauser syndrome: one case report. Ob-stet Gynecol Intern 2010; 1-3. doi:10.1155/2010/8473705. Di Spiezio Sardo A, Campo R, Gordts S et al. Th e ESH-RE/ESGE consensus on the classifi cation of female genital tract congenital anomalies. Hum Reprod 2015; 30(5): 1046-58. 6. Pertusa S, Palacios A. 46 XX pure gonadal dysgenesis: an infrequent cause of primary amenorrhoea. BMJ Case Reports 2009; doi: 10.1136/bcr.07.2008.0485 7. Ropke A, Pelz A, Volleth M et al. Sex chromosomal mo-saicism in the gonads of patients with gonadal dysgenesis, but normal female or male karyotypes in lymphocytes. Am J Obstet Gynecol 2004; 190: 1059-62.8. Zangen D, Kaufman Y, Zeligson S et al. XX ovarian dys-genesis is caused by a PSMC3IP/HOP2 mutation that abolishes coactivation of estrogen-driven transcription. Am J Hum Gen-et 2011; 89(4): 572-9. doi: 10.1016/j.ajhg.2011.09.0069. Kanagal DV, Prasad K, Rajesh A et al. Ovarian go-nadoblastoma with dysgerminoma in a young girl with 46, XX karyotype: a case report. J Clin Diagn Res 2013; 7(9): 2021-2. doi: 10.7860/JCDR/2013/6412.3393Prikazana je bolesnica s hemoragičnim šokom zbog intrakoitalnog razdora rodnice u rijetkom slučaju gonadalne disgeneze i maternične i obostrane aplazije jajovoda (46 XX), što je pronađeno tijekom laparoskopije. Učinjena je reanimacija i primar-ne šavi ozljede s potpunim oporavkom.Ključne riječi: razdor rodnice, hemoragični šok, gonadalna disgeneza, aplazija maternice i jajovoda, hipoplazija jajnikaS A Ž E T A KHEMORAGIČNI ŠOK ZBOG RAZDORA RODNICE U BOLESNICE S GONADALNOM DISGENEZOM (46 XX)D. HABEK, M. DRAGIĆ i K. ŽIVKOVIĆKlinička bolnica Sveti Duh, Hrvatsko katoličko sveučilište, Klinika za opstetriciju i ginekologiju, Zagreb, Hrvatska

HEMORAGIČNI ŠOK ZBOG RAZDORA RODNICE U BOLESNICE S GONADALNOM DISGENEZOM (46 XX)

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