A Case Series of Rare Ectopic Pregnancies - Clinical Presentation and Management at Vani Vilas Hospital

 Introduction

Ectopic pregnancy is a master chameleon and can have a variety of presentations.¹ It is a leading cause of mortality and morbidity among pregnant women. It can be managed by medical or surgical methods depending on the age of the patient, reproductive history and status of the fallopian tube. The majority of ectopic pregnancies are located within the fallopian tube. Nevertheless, pregnancies have been reported to implant in the ovary, interstitial tubal segment, C-section scar and at various abdominal sites.²

The incidence of ectopic pregnancy is 2% of all reported pregnancies. The incidence is rising in the recent past due to increased risk of sexually transmitted infections and early diagnosis owing to advancements in technology. Most of the ectopic pregnancies present with a classical triad of pain in abdomen, bleeding per vagina and amenorrhoea. A meticulous history and thorough clinical examination is a must, followed by ultrasound, serum beta Human Chorionic Gonadotropin (HCG) doubling time.¹

The early diagnosis and treatment of unusual implantation sites present a challenge for clinical as well as radiological diagnosis and there is a tendency to overlook its possibility. In this study, we illustrate and discuss the diagnosis and optimal treatment of rare ectopic pregnancies based on our experience with eight rare types of ectopic pregnancy cases from Vani Vilas Hospital, Bengaluru. The successful management of these cases hinged on a strong index of suspicion, early detection, and timely laparotomy, which proved life saving. An alert clinician who makes timely decisions can avert life-threatening complications in such pregnancies.³

Case Series

Source of data

Rare ectopic pregnancies reported to the Department of Obstetrics and Gynaecology (OBG) in Vani Vilas Hospital, BMCRI.

Methods of Collection of Data

A. Study design: Case series

B. Study period: One year (March 2023 – March 2024)

C. Place of study: Vani Vilas Hospital, BMCRI

Inclusion criteria

1. Extra uterine pregnancies, except tubal pregnancy

2. Uterine, but ectopic location in the uterus

Exclusion criteria

1. Tubal pregnancy

2. Intrauterine pregnancy

After obtaining approval and clearance from the institutional ethics committee, patients fulfilling the criteria were included in the study.

Pregnant women diagnosed with rare ectopic pregnancies and admitted to the Department of OBG were included in this study after providing informed consent. A detailed history was taken, followed by clinical evaluation, ultrasound examination, and serum beta HCG testing. Additionally, blood investigations were performed.

All records and registers maintained during the study period in our tertiary care hospital were utilized for this purpose.

The data regarding intraoperative findings were collected from the OT register. The various methods adopted for the surgical management of each of the cases were noted and analyzed.

The following cases are discussed in this paper: three cases of interstitial pregnancy, two cases of Caesarean scar pregnancy, two cases of ovarian pregnancy, and one case of heterotopic pregnancy.

Case 1

A 24-year-old primigravida with a history of 1.5 months of amenorrhea presented with pain in abdomen for one week, predominantly on the right side.

• Examination findings: Pulse rate: 100 beats/min, Blood pressure: 100/70 mmHg. On abdominal examination, mild tenderness in right lower quadrant was noted. Right fornix tenderness was observed on bimanual examination.

(i) Investigations:

• Urine pregnancy test (UPT) - Positive.
• beta Human Chorionic Gonadotropin - 15,000 mIU/mL.
• Ultrasounds showed seven weeks of gestation with pregnancy outside the uterine cavity in the right interstitial part of fallopian tube with a Crown rump length (CRL) of 9.1 mm.

    (ii) Diagnosis: Right interstitial ectopic pregnancy

   (iii) Management

• Intraoperative finding - An interstitial pregnancy measuring 5 x 5 cm was noted on the right side.
• Right partial isthmic excision was done (Figure 1).

      Case 2

A 38-year-old, Gravida 4 Para 3 Living 3, with 1.5 months of amenorrhea and a positive UPT, presented with abdominal pain since one day.

On examination, pulse rate was 106 beats/min, blood pressure was 110/70 mmHg with diffuse abdominal tenderness, predominantly on the left lower side, with left fornix tenderness noted on bimanual examination.

Ultrasound showed left, unruptured ectopic of 3 x 3 cm, at the cornual end of fallopian tube.

On laparotomic exploration:

Left sided ectopic pregnancy measuring 3 x 3 cm was noted at the cornua medial to round ligament. Vasopressin 20 units in 200 mL dilution was injected around the mass. Horizontal incision was made and left salpingostomy was done along with ligation of the feeding vessel.

Case 3

A 28-year-old, Gravida 3 Para 1 Living 0 Abortion 1, with seven weeks period of gestation was referred to Vani Vilas Hospital for right ectopic pregnancy. On examination, tachycardia with a pulse rate of 110 beats/min, blood pressure of 100/60 mmHg, and pallor were noticed. On abdominal examination, tenderness was noted on the right side, near iliac fossa. On bimanual examination, right fornix tenderness was noted.

Ultrasound evaluation conducted at the hospital showed an ectopic on the right side of interstitial part of the tube.

Intraoperatively, a right interstitial pregnancy at the cornual end was identified and excision was done. Additionally, one pint of packed red blood cells was transfused. Post operative period was uneventful.

Case 4

A 35-year-old female, presented with a history of 1.5 months of amenorrhea and pain in abdomen since two days, with a positive UPT status. On examination, mild pallor with tachycardia with a pulse rate of 104 beats/min was observed. On per abdomen examination, tenderness was noted in the right iliac fossa. On bimanual examination, uterus was noted to be bulky with right sided fornix tenderness. Ultrasound evaluation showed a heterogeneous hyperechoic lesion with multiple cystic areas within the right adnexa, abutting the uterus medially with minimum flow in doppler, with mild to moderate fluid in peritoneal cavity. A provisional diagnosis of a right ruptured ectopic pregnancy was made. On laparotomic exploration, hemoperitoneum of 600 mL was noted with normal right fallopian tube. Right ruptured ovarian ectopic was found and excision was performed. The specimen was sent for histopathological examination, which confirmed the ovarian ectopic gestation of right ovary (Figure 2).

Case 5

A 30-year-old woman, Gravida 3 Para 1 Living 1 Abortion 1, with a previous history of lower segment caesarean (LSCS) prior to three years presented with amenorrhea for two months, and c/o bleeding pv and pain in abdomen since one day.

On examination: Urine pregnancy test was positive with a pulse rate of 110 beats/min and blood pressure of 110/70 mmHg. On per abdomen examination, mild tenderness was noted at the scar site. On bimanual examination, uterus was found to be bulky with bilateral fornices free and non-tender. Her β-HCG was 38,342 mIU/mL.

An ultrasound was performed which showed an empty uterus and cervical canal with gestation sac in the anterior wall of uterine isthmus.

On laparotomic exploration, an incision was made over the previous C-section scar site and the gestational sac was found at the scar site. An area of 3 x 3 cm was excised and sent for histopathological examination, which confirmed the ectopic pregnancy (Figure 3).

Case 6

A 22-year-old female, Gravida 3 Para 2 Living 1, with a gestational age of 10 week 6 days, and a history of two previous LSCS, was referred for evaluation of a C-section scar pregnancy, which was detected on a dating scan. She had no complaints of abdominal pain or bleeding per vagina.

On examination, her pulse rate was 98 beats/min and blood pressure was 110/80 mmHg.

The abdomen was soft, non-tender with Pfannenstiel scar noted in the lower abdomen. On bimanual examination, uterus was noted to be normal in size with bilateral fornices free and non-tender. Her β-HCG was 43,563 mIU/mL.

On ultrasound evaluation: Gestational sac was found in the lower uterine segment with thinning of adjacent anterior myometrial wall, suggestive of a C-section scar pregnancy.

Management: The patient underwent uterine artery embolization followed by manual vacuum aspiration. The histological examination confirmed the presence of decidual proliferation. Post procedure was uneventful and the patient was discharged on the third day. Her β-HCG was repeated after one week, which was less than 5 mIU/mL. The patient was reviewed after a month and was found to be asymptomatic.

Case 7

A 28-year-old woman, Gravida 3 Para 2 Living 2, presented with amenorrhea for one month, and pain in abdomen, bleeding per vagina since one day. UPT was positive. On examination, pallor was noted with tachycardia of 120 beats/min and a blood pressure of 80/60 mmHg. Immediately, IV cannula was placed and intravenous fluids were started. Blood samples were drawn for haemoglobin estimation, blood grouping, and cross matching. Her haemoglobin was found to be 6 gm/dL. Two units of packed red blood cells (PRBC) were arranged and the patient was immediately shifted to OT.

Intraoperatively, a hemoperitoneum of 500 mL was noted, with 200 cc removed. Adhesions were observed between the left adnexa, omentum and pouch of Douglas. A ruptured left ovarian ectopic pregnancy was detected, and a left salpingectomy was performed. A right ovarian cyst measuring 2 x 3 cm was noted and punctured. The tissue was sent for histopathological examination, which confirmed the presence of ovarian ectopic tissue (Figure 4).

Case 8

A 29-year-old, Gravida 2 Para 1 Living 1, with a history of 1.5 months of amenorrhea, and a positive urine pregnancy test presented with pain in abdomen since one day.

On examination, her pulse rate was 90 beats/min with a blood pressure of 110/70 mmHg. Per abdomen was soft, non-tender. Uterus was noted to be bulky, with no fornix tenderness noted on bimanual examination.

An ultrasound evaluation revealed an intrauterine gestation of five weeks, with a thick-walled gestational sac like structure in the right adnexa, and a moderate collection in the pelvic/peritoneal cavity. A diagnosis of heterotopic pregnancy was made.

On laparotomic exploration, right ectopic tubal abortion was noted and right salpingectomy was performed.

Post-operative scan was repeated on day 3, which showed intrauterine gestation and the patient was started on oral progesterone along with folic acid supplements.

Discussion

Ectopic pregnancy is a condition in which a fertilized egg gets implanted at a site other than the uterine cavity. They are estimated to occur in 1-2% of all pregnancies.⁴ It is a life threatening condition causing significant mortality. The rarer types of ectopic pregnancies include, ovarian ectopic, Caesarean scar ectopic, interstitial and heterotrophic pregnancies, each presenting unique challenges in diagnosis, management and outcomes.

Interstitial Ectopic Pregnancy

This occurs when the embryo implants in the proximal segment of fallopian tube. It represents 2-4% of ectopic pregnancies and is dangerous due to the rich blood supply in this region. The diagnosis before rupture is difficult. It is mistaken with lateral flexion of gravid uterus,pregnancy associated with fibroid.⁵ The ultrasound criteria for diagnosis include:

a) Absence of intrauterine pregnancy.

b) A chorionic sac separate and at least 1 cm from the lateral edge of uterine cavity.

c) A thin layer of myometrium surrounding the gestational sac measuring less than 5 mm.

d) Interstitial sign - An echogenic line extending from endometrial cavity to the uterine cornua, adjacent to the gestation sac.

An MRI, β-HCG, and high-resolution transvaginal ultrasound can aid in early diagnosis. Timely diagnosis can prevent excessive intraperitoneal bleeding. Treatment includes immediate laparotomy with salpingectomy, wedge resection of cornua, reconstruction of uterine wall if needed. In cases of torrential bleeding, hysterectomy may be warranted to prevent mortality.

Ovarian Ectopic Pregnancy

It is an uncommon form of ectopic pregnancy where fertilised egg implants directly on the ovary. It accounts for 0.5-3% of all ectopic pregnancies, making it one of the rarest forms. The diagnosis is often difficult due to its similarities to ovarian cysts in appearance on imaging. Spiegelberg described four criteria for the diagnosis of ovarian pregnancy which include:

a) The fallopian tube on the affected side must be intact.

b) The foetal sac must occupy the position of ovary.

c) The ovary must be connected to uterus by the ovarianligament.

d) The ovarian tissue must be located in the sac wall.

Laparotomy is considered as a gold standard for definitive diagnosis, along with histopathological confirmation. Management is essentially surgical with early diagnosis and intervention to prevent morbidity and mortality.

Caesarean Scar Ectopic Pregnancy

Here, the embryo implants within the fibrous tissue of a previous C-section scar; a rare but increasingly concerning condition as the global rate of caesarean deliveries rises. This type of ectopic pregnancy is particularly dangerous due to the risk of early uterine rupture, which can occur even in the first trimester, leading to a massive haemorrhage. Diagnosis is based on ultrasound criteria such as:

a) An empty uterus and cervical canal.

b) A gestational sac in the anterior wall of the uterine isthmus.

c) Absent or diminished myometrium between gestational sac and bladder.

d) Presence of colour doppler flow surrounding gestational sac.

Treatment options include systemic or local administration of methotrexate to stop pregnancy, dilatation and curettage (D&C), uterine artery embolization or surgical excision of the scar tissue. The treatment depends on gestational age, the extent of myometrial involvement and the patient’s reproductive needs.

Heterotopic Pregnancy

Spontaneous heterotopic pregnancy is a rare clinical and a potentially dangerous condition in which intrauterine and extra uterine pregnancies occur at the same time.6 Early diagnosis of heterotopic pregnancy is difficult as clinical symptoms are often lacking. Most common presentation is abdominal pain. Other features such as adnexal mass, peritoneal irritation with enlarged uterus aids in the diagnosis of heterotrophic pregnancy. Around 56% patients can be diagnosed at 5-6 weeks with a transvaginal scan and around 10% cases are diagnosed much later than 11 weeks when ruptured.

Conclusion

The management of rare ectopic pregnancies, such as interstitial, ovarian, C-section scar, heterotopic, requires prompt and accurate diagnosis to prevent life threatening complications. Ovarian ectopic pregnancies mimic ovarian cysts, making surgical intervention the primary treatment. Cesarean scar ectopic pregnancies pose a significant risk of uterine rupture and hemorrhage, necessitating early diagnosis and careful selection of medical or surgical treatment to preserve fertility. Heterotrophic pregnancies, particularly prevalent in artificial reproductive technique patients, require a high index of suspicion with timely intervention to manage the ectopic pregnancy, while protecting the intrauterine pregnancy. Interstitial ectopic pregnancies with their risk of late rupture demand early diagnosis and appropriate medical or surgical intervention for preventing catastrophic hemorrhage. These rare ectopic pregnancies highlight the importance of individualized management strategies and the critical role of early detection in improving patient outcomes and preserving fertility.

Conflicts of Interest

None