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CASE REPORT Ectopic pregnancy in an undescended fallopian tube: a diagnostic challenge Catarina Pardal, Luís Braga, Belisa Vides Gynecology and Obstetrics Department, Hospital de Braga, Braga, Portugal Correspondence to Dr Catarina Pardal, catarinapardal_84@hotmail. com Accepted 25 January 2016 To cite: Pardal C, Braga L, Vides B. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/bcr-2015- 213058 SUMMARY Uterine malformations are the most frequent anomalies of the Müllerian ducts, but undescended ovaries and fallopian tubes are very rare congenital defects. Pregnancy in these misplaced organs may occur, frequently posing a diagnostic challenge. A case of a ruptured ectopic pregnancy in an undescended fallopian tube associated with other genital malformations is presented. This case provides evidence for the recognised phenomenon of peritoneal gametes or embryo transmigration. BACKGROUND Development of the female genital tract is a complex process. It is dependent on a series of events such as cellular differentiation, migration, fusion and canalisation. Failure of any of these pro- cesses may result in a congenital anomaly. Uterine abnormalities are the most frequent anomalies of the Müllerian ducts, occurring in 24% of fertile women, 1 while structural anomalies of the fallopian tubes and ovaries are exceedingly rare. 2 Some uterine malformations, in particular unicornuate uterus, 1 are known to be associated with renal agenesis, ectopic ovaries and undescended tubes, and, although rare, pregnancy in such misplaced organs can occur. These ectopic pregnancies are frequently difcult to diagnose and are associated with important maternal morbidity and mortality. CASE PRESENTATION A 28-year-old woman presented at our emergency department, with a history of 9 weeks of amenor- rhoea, mild vaginal bleeding and pelvic pain. She reported a positive urine pregnancy test. The patients history was signicant for primary infertil- ity. The subsequent evaluation included a diagnostic laparoscopy, which revealed a left unicornuate uterus with no rudimentary horn (Buttram and Gibbonsclassication class IIB or European Society of Human Reproduction and Embryology-European Society for Gynaecological Endoscopy (ESHRE/ESGE) classication class U4b) and an absent right fallopian tube and ovary. On admission, the patient was pale; her blood pressure was 107/76 mm Hg, and she had a heart rate of 90 bpm and body temperature of 36.4°C. Findings on physical examination were mild uterine enlargement with no adnexal mass; cervical motion and abdominal tenderness. INVESTIGATIONS A transvaginal ultrasound (TVUS) demonstrated a unicornuate uterus with no rudimentary horn, a thickened homogeneous endometrium (16 mm), no signs of pregnancy (intrauterine or extra uterine) and scarce free peritoneal uid. No right adnexa were identied. The measurement of the serum β subunit human chorionic gonadotrophin (β-HCG) was positive (38 485.9 mUI/mLhigh above the discriminatory zone). An abdominal ectopic preg- nancy was suspected and the ultrasound (US) con- rmed the location of a gestational sac in the right upper quadrant of the abdomen, near what appeared to be a right ectopic ovary. Given the previous information of absent right adnexa, a laparoscopy was proposed to clarify both the diagnosis and treatment. The main pelvic nd- ings included (video 1) a small volume haemoperi- toneum and a unicornuate uterus with normal left adnexa and no right rudimentary horn. The abdominal inspection revealed a right undescended ovary located above the pelvic bream and adherent to the parietal peritoneum, with a juxtaposed bluish sac-like formation 5 cm in diameter, corre- sponding to the ectopic pregnancy. No corpus luteum was identied. Histological examination conrmed the diagnosis of ruptured tubal pregnancy. DIFFERENTIAL DIAGNOSIS Amenorrhoea and abdominal pain are common symptoms of early pregnancy complications (such as uterine enlargement, threatened abortion or rup- tured corpus luteum cyst), as well as ectopic preg- nancy. If in the context of uterine bleeding, another possible diagnosis is gestational trophoblastic disease. The TVUS plus the high level of serum β-HCG suggested an ectopic pregnancy of unknown loca- tion, which was conrmed by the abdominal US and diagnostic laparoscopy. TREATMENT Laparoscopic right total salpingectomy was per- formed (video 1). Because the patient had a history of infertility, the ectopic right ovary was not removed. OUTCOME AND FOLLOW-UP The patients postoperative course was uneventful, and she was discharged 2 days after surgery. Pardal C, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2015-213058 1 Reminder of important clinical lesson

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  • CASE REPORT

    Ectopic pregnancy in an undescended fallopiantube: a diagnostic challengeCatarina Pardal, Luís Braga, Belisa Vides

    Gynecology and ObstetricsDepartment, Hospital deBraga, Braga, Portugal

    Correspondence toDr Catarina Pardal,[email protected]

    Accepted 25 January 2016

    To cite: Pardal C, Braga L,Vides B. BMJ Case RepPublished online: [pleaseinclude Day Month Year]doi:10.1136/bcr-2015-213058

    SUMMARYUterine malformations are the most frequent anomaliesof the Müllerian ducts, but undescended ovaries andfallopian tubes are very rare congenital defects.Pregnancy in these misplaced organs may occur,frequently posing a diagnostic challenge. A case of aruptured ectopic pregnancy in an undescended fallopiantube associated with other genital malformations ispresented. This case provides evidence for therecognised phenomenon of peritoneal gametes orembryo transmigration.

    BACKGROUNDDevelopment of the female genital tract is acomplex process. It is dependent on a series ofevents such as cellular differentiation, migration,fusion and canalisation. Failure of any of these pro-cesses may result in a congenital anomaly. Uterineabnormalities are the most frequent anomalies ofthe Müllerian ducts, occurring in 2–4% of fertilewomen,1 while structural anomalies of the fallopiantubes and ovaries are exceedingly rare.2 Someuterine malformations, in particular unicornuateuterus,1 are known to be associated with renalagenesis, ectopic ovaries and undescended tubes,and, although rare, pregnancy in such misplacedorgans can occur. These ectopic pregnancies arefrequently difficult to diagnose and are associatedwith important maternal morbidity and mortality.

    CASE PRESENTATIONA 28-year-old woman presented at our emergencydepartment, with a history of 9 weeks of amenor-rhoea, mild vaginal bleeding and pelvic pain. Shereported a positive urine pregnancy test. Thepatient’s history was significant for primary infertil-ity. The subsequent evaluation included a diagnosticlaparoscopy, which revealed a left unicornuateuterus with no rudimentary horn (Buttram andGibbons’ classification class IIB or EuropeanSociety of Human Reproduction andEmbryology-European Society for GynaecologicalEndoscopy (ESHRE/ESGE) classification class U4b)and an absent right fallopian tube and ovary.On admission, the patient was pale; her blood

    pressure was 107/76 mm Hg, and she had a heartrate of 90 bpm and body temperature of 36.4°C.Findings on physical examination were milduterine enlargement with no adnexal mass; cervicalmotion and abdominal tenderness.

    INVESTIGATIONSA transvaginal ultrasound (TVUS) demonstrated aunicornuate uterus with no rudimentary horn, athickened homogeneous endometrium (16 mm), nosigns of pregnancy (intrauterine or extra uterine)and scarce free peritoneal fluid. No right adnexawere identified. The measurement of the serum βsubunit human chorionic gonadotrophin (β-HCG)was positive (38 485.9 mUI/mL—high above thediscriminatory zone). An abdominal ectopic preg-nancy was suspected and the ultrasound (US) con-firmed the location of a gestational sac in the rightupper quadrant of the abdomen, near whatappeared to be a right ectopic ovary.Given the previous information of absent right

    adnexa, a laparoscopy was proposed to clarify boththe diagnosis and treatment. The main pelvic find-ings included (video 1) a small volume haemoperi-toneum and a unicornuate uterus with normal leftadnexa and no right rudimentary horn. Theabdominal inspection revealed a right undescendedovary located above the pelvic bream and adherentto the parietal peritoneum, with a juxtaposedbluish sac-like formation 5 cm in diameter, corre-sponding to the ectopic pregnancy. No corpusluteum was identified. Histological examinationconfirmed the diagnosis of ruptured tubalpregnancy.

    DIFFERENTIAL DIAGNOSISAmenorrhoea and abdominal pain are commonsymptoms of early pregnancy complications (suchas uterine enlargement, threatened abortion or rup-tured corpus luteum cyst), as well as ectopic preg-nancy. If in the context of uterine bleeding, anotherpossible diagnosis is gestational trophoblasticdisease.The TVUS plus the high level of serum β-HCG

    suggested an ectopic pregnancy of unknown loca-tion, which was confirmed by the abdominal USand diagnostic laparoscopy.

    TREATMENTLaparoscopic right total salpingectomy was per-formed (video 1). Because the patient had a historyof infertility, the ectopic right ovary was notremoved.

    OUTCOME AND FOLLOW-UPThe patient’s postoperative course was uneventful,and she was discharged 2 days after surgery.

    Pardal C, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2015-213058 1

    Reminder of important clinical lesson

    http://crossmark.crossref.org/dialog/?doi=10.1136/bcr-2015-213058&domain=pdf&date_stamp=2016-02-08http://casereports.bmj.com

  • DISCUSSIONCongenital malformations of the female genital tract are definedas deviations from normal anatomy resulting from embryo-logical maldevelopment of the Müllerian ducts. They representa rather common benign condition with a prevalence of 4–7%.3

    Uterine anomalies occur in 2–4% of fertile women,1 but undes-cended ovaries and fallopian tubes are very rare congenitalabnormalities of the Müllerian ducts.4 They are not mentionedin the widely used classification of Müllerian anomalies pro-posed by Buttram and Gibbons in 1979, nor are they includedin the new classification proposed by ESHRE/ESGE.

    Embryologically, the fallopian tube is the most cephalicportion of the Müllerian duct. During the sixth week of gesta-tion, it can be seen as a shallow depression in the coelomic epi-thelium lateral to the mesonephric duct. Starting as a groove,the tube forms, a lumen develops and the Müllerian duct growscaudally. The ovary, however, starts as a thickening of thegenital ridge during the fifth week. Germ cells found near theallantois migrate towards the genital ridge. The ovary then ‘des-cends’ from its position near the kidney towards the truepelvis.2 Failure of descent of the ovaries and tubes may occur,and pregnancy in such misplaced organs may pose importantmaternal risks.2 Only a few case reports are found in the litera-ture. Granat and co-authors reported the first case of pregnancyin ectopic adnexa.2 Other authors have described such a condi-tion,2 4 5 varying the site of fallopian tube in the abdomen andsite of corpus luteum.

    Our case illustrates the interesting anatomic and physiologicalfeatures of the female genital tract and demonstrates the phe-nomenon of transperitoneal gamete/embryo migration.Furthermore, this case underlines the importance of careful USexamination of both adnexa when an extra uterine pregnancy is

    suspected, even when a right ovary agenesia and a left unicornu-ate uterus would appear to exclude the involvement of one side.

    Some authors4 have proposed the removal of undescendedfallopian tubes in order to decrease the risk of a tubal preg-nancy, and laparoscopic treatment appears to be a relatively easyand safe procedure.

    In summary, an ectopic pregnancy in an undescended fallo-pian tube is a rare condition frequently difficult to diagnose.There is no simple screening technique for this condition and,therefore, clinicians and radiologists should be aware of it forsuch a diagnosis.

    Learning points

    ▸ Uterine abnormalities are the most frequent congenitalanomalies of the Müllerian ducts, while structural anomaliesof the fallopian tubes are very rare.

    ▸ Ectopic pregnancy in undescended fallopian tubes mayoccur, posing a diagnostic challenge.

    ▸ Pregnancy in undescended fallopian tubes providessupportive evidence for the phenomenon of transperitonealgamete/embryo migration.

    Contributors CP managed the case, provided the design and conception of thearticle, performed the literature review and is the guarantor. LB managed the case.BV managed the case and revised the article.

    Competing interests None declared.

    Patient consent Obtained.

    Provenance and peer review Not commissioned; externally peer reviewed.

    REFERENCES1 Iverson R, DeCherney A, Laufer M. Clinical manifestations and diagnosis of

    congenital anomalies of the uterus, Uptodate, 20152 Seoud M, Khayyat H, Mufarrij IK. “Ectopic pregnancy in an undescended fallopian

    tube: an unusual presentation”. Obstet Gynecol 1987;69(Part 2):455–7.3 Grimbizis GF, Gordts S, Di Spiezio Sardo A, et al. The ESHRE/ESGE consensus on the

    classification of female genital tract congenital anomalies. Hum Reprod2013;28:2032–44.

    4 Timmerman D, Lewis B. Laparoscopic treatment of an ectopic pregnancy in anundescended fallopian tube. J Obstet Gynaecol 1993;13:291–2.

    5 Gabriel B, Fischer DC, Sergius G. Unruptured pregnancy in a non-communicatingheterotopic right fallopian tube associated with left unicornuate uterus: evidence fortransperitoneal sperm and oocyte migration. Acta Obstet Gynecol Scand2002;81:91–2.

    Copyright 2016 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visithttp://group.bmj.com/group/rights-licensing/permissions.BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.

    Become a Fellow of BMJ Case Reports today and you can:▸ Submit as many cases as you like▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles▸ Access all the published articles▸ Re-use any of the published material for personal use and teaching without further permission

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    Video 1 Laparoscopic evaluation of the abdomen.

    2 Pardal C, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2015-213058

    Reminder of important clinical lesson

    http://dx.doi.org/10.1093/humrep/det098http://dx.doi.org/10.3109/01443619309151868

    Ectopic pregnancy in an undescended fallopian tube: a diagnostic challengeAbstractBackgroundCase presentationInvestigationsDifferential diagnosisTreatmentOutcome and follow-upDiscussionReferences