{"paper_id":"307dd148-b7f9-4326-a833-ff1baddbc605","body_text":"Intramural ectopic pregnancy is described as a pregnancy that is partially or\ncompletely located within the myometrium of the uterine wall, without connection to\nthe fallopian tubes or endometrial cavity. 1  It is characterized by trophoblastic invasion that extends beyond the\nendometrial-myometrial junction, with invasion into the myometrium. 2  Diagnosis requires visualization of trophoblastic invasion into the\nmyometrium, most commonly performed with transvaginal ultrasound or magnetic\nresonance imaging (MRI). 3  Intramural pregnancy is a rare diagnosis, accounting for less than 1% of all\nectopic pregnancies. 4\nThere is limited evidence to guide management of intramural ectopic pregnancy.\nMedical treatment involves using localized methotrexate with or without potassium\nchloride and systemic methotrexate, while surgical encompasses procedures to remove\nthe pregnancy tissue such as uterine wedge resection or hysterectomy. However, the\nmanagement pathway will vary depending on location, extend of myometrial\ninvolvement, gestational age at diagnosis, viability, and the patient’s desire to\nconserve the pregnancy and wishes for future fertility. 5\n\nA 34-year-old multiparous woman re-presented for review with vaginal discharge and\npain in the right iliac fossa on a background of a positive β-HCG. She had been\nreviewed 1 year previously in the gynecological outpatient clinic for opinion about\nan incidental finding of a benign asymptomatic fibroid discovered on a pelvic\nultrasound performed by her local doctor for investigation for gastric symptoms.\nUltrasonography performed with her local doctor revealed a 63 × 60 × 56 mm\nintramural fibroid in the right lateral posterior uterine wall and a smaller 58 × 30\n× 19 mm fibroid adjacent to the external cervical os. Despite extensive counselling\nagainst surgical management, the patient underwent an open myomectomy privately.\nShe re-presented 1 year post open myomectomy with vaginal discharge and pain in the\nright iliac fossa with a 12-week pregnancy by her last menstrual cycle. This\npregnancy was spontaneously conceived, and her past obstetric history included 2\nnormal vaginal deliveries. On review, she was clinically well and a transvaginal\nultrasound was performed, which revealed a live intramural ectopic pregnancy, with a\nthin 3-mm layer of myometrium surrounding the pregnancy ( Figures 1  and  2 ). Placental invasion was also seen, thought\nto be over the previous myomectomy site. An MRI was performed following the\nultrasound to help aid management and determine if fertility sparing intervention\noptions could be considered. MRI revealed a gestational sac (8.0 × 7.9 × 7.0 cm)\ncontaining a mobile fetus within the myometrium of the right uterine cornua, with\nmarked thinning of the overlying myometrium to 3 mm, with no clinical features of\nhemoperitoneum ( Figure\n3 ).\nTransvaginal ultrasound with the live intramural pregnancy seen.\nTransvaginal ultrasound with the live intramural pregnancy seen.\nSagittal views on magnetic resonance imaging of the abdomen demonstrating an\nintramural pregnancy.\nInitial management options that were considered included medical management with\nintra-sac and multidose methotrexate, uterine wedge resection, or hysterectomy. The\npatient’s desires to conserve fertility were considered, and hence, all conservative\nmanagement options were explored at multidisciplinary clinical meetings.\nSubspecialty experts in gynecological surgery and ultrasound were involved in this\nclinical decision-making process. Unfortunately, medical management with intra-sac\nand multidose methotrexate was deemed inappropriate due to the advanced gestation\nage of the pregnancy. Wedge resection of the uterus was also excluded as a viable\nmanagement option as the location and size of the intramural ectopic pregnancy would\nresult in a large amount of uterine tissue needing to be excised. Senior clinicians,\ntogether with the patient, made a uniform decision that it would be safest to\nproceed with hysterectomy.\nA midline laparotomy, total abdominal hysterectomy, and bilateral salpingectomy was\nperformed. Blood loss was minimal, and the patient remained well postoperatively.\nShe was discharged home 3 days later after an uneventful recovery.\n\nThis case leaves open for discussion many issues associated with care in women of\nreproductive age. The patient underwent surgical management of a benign asymptomatic\nfibroid in the year prior, which increased her risk of future complications,\nincluding ectopic pregnancy, placental adhesive disorders, and uterine dehiscence in\nfuture pregnancies. 6  Surgical management of benign asymptomatic fibroids is controversial, with\nthe general consensus being against surgery if patients are asymptomatic. 7\nA review of the literature demonstrates less than 30 published cases of intramural\nectopic pregnancy of various etiologies. Cases associated with previous myomectomy\nspecifically are even more uncommon. Bannon et al 6  described a similar case to the one here. The patient presented at 6 weeks\ngestation, having undergone an open myomectomy 3 years previously. She was diagnosed\nwith a missed abortion and underwent suction dilatation and curettage. The pathology\nrevealed decidua with foci of necrosis and portions of gestational endometrium, but\nno placental villi was identified. A subsequent transvaginal ultrasound and computed\ntomography scan were performed, with an intramural pregnancy diagnosed at the site\nof the previous myomectomy scar. A single dose of systemic methotrexate was\nadministered; however, a 5-cm avascular intramural pregnancy with possible fistulous\ntract persisted, and the patient subsequently underwent laparoscopic removal of the\nintramural pregnancy. In this case, the incorrect initial diagnosis of missed\nabortion complicated the clinical timeline and delayed the diagnosis. It is\nimportant to recognize that intramural pregnancy is often difficult to distinguish\nfrom other pathologies; however, performing ultrasonography together with MRI may\nassist in making an accurate diagnosis and exclude other diagnostic probabilities. 7\nAs described, surgical procedures such as myomectomy, salpingectomy, hysteroscopy,\nand dilatation and curettage are all thought to contribute to the risk of intramural implantation. 5  Other predisposing factors include assisted reproductive technologies and adenomyosis. 8  Intramural pregnancy often presents with nonspecific clinical symptoms,\nincluding mild vaginal bleeding and abdominal pain; however, some patients may be\nasymptomatic. Early diagnosis is key in preventing complications, including uterine\nrupture. Failure to diagnose an intramural pregnancy can result in catastrophic\nhemorrhage due to the proximity of the gestational sac to the intramyometrial\narcuate vasculature. 9\nThe pathophysiology of intramural pregnancy is not entirely clear and many hypotheses\nexist. Previous uterine surgery may lead to the formation of myometrial defects and\nfacilitate intramural implantation. 5  It is thought that the embryo implants into the myometrium through a\nmicroscopic fistula, created through previous uterine surgery, like myomectomy but\nalso as a consequence of previous caesarean section. 10  In a similar way, the embryo may implant, together with endometrial tissue,\ninto the myometrium during the development of adenomyosis. 11  Furthermore, artificial implantation of the embryo during assisted\nreproductive technologies may also result in development of an intramural pregnancy. 2  The myometrial defect potentially created from these procedures is thought to\nallow trophoblast invasion into the myometrium, which may enable intramural implantation. 5\nTransvaginal ultrasound is considered the first-line imaging technique for diagnosis\nof ectopic pregnancy, 7  with a diagnostic accuracy of 90.9%. 12  The other imaging modality alternatively used, MRI, has a diagnostic accuracy\nof 96%. 13  This case report utilized both imaging techniques as diagnostic tools and for\nsurgical planning, with both playing an important role in constructing the overall\nclinical picture. In cases of unusual or rare pregnancies, the use of such\ndiagnostic tools early in the gestation has allowed management to shift\npredominately from radical surgical management, to more conservative, minimally\ninvasive interventions. 14\nIn patients who present clinically well, without signs of hypovolemic shock with\nsuspected uterine rupture, medical or surgical management options can be considered.\nA recent study by Ramkrishna et al 13  has shown that the use of systemic methotrexate and or local intra-sac\nmethotrexate (with intra-sac KCl if embryonic heart activity is present) is a\nsuccessful intervention for management of nontubal ectopic pregnancies, especially\nin those women wishing to preserve fertility. If diagnosis is made at an early\ngestation, prior to rupture, conservative options can be considered. 15  The median gestational age of successful medical management within the study\nby Ramkrishna et al 13  in all ectopic pregnancy sites was less than 8 weeks gestation. Medical\nmanagement can be considered at early gestations in cases when the patient is\nclinically stable. Given the advanced gestational age and presence of a fetal\nheartbeat in this case, the use of systemic or local injection of methotrexate did\nnot seem appropriate, and hence, surgical management was required. Surgical options\ncan include excision of the intramural pregnancy or definitive hysterectomy, these\ncan be done laparoscopically or open. 16\nThis report adds to the literature and explores some of the diagnostic and management\nchallenges uncommon ectopic implantation sites can pose. Women often present with\nnonspecific clinical symptoms, which makes diagnosis difficult. Previous uterine\nsurgical procedures have been shown to increase the risk of fertility complications,\nincluding intramural pregnancy, and as such, patients with known risk factors should\nseek medical attention early in their pregnancy. Transvaginal ultrasound plays a\npivotal role, with MRI also adding to diagnostic accuracy. Overall, diagnosis and\ntreatment should be tailored to individual patient factors, with multidisciplinary\nteam management playing a pivotal role.","source_license":"CC-BY-4.0","license_restricted":false}