Volume 4 Issue 7, July 2017

Editorial
Parulekar SV

Cesarean Scar Ectopic Pregnancy - A Perplexing Presentation
Mehta N, Parulekar SV.

Degenerated Uterine Leiomyoma Masquerading As An Ovarian Cyst
Parulekar SV

Scar Ectopic Pregnancy Managed Laparoscopically
Shah NH, Paranjpe S, Shah VN.

Management of secondary post partum hemorrhage by uterine artery embolization
Deshpande PS, Prasad M, Gupta AS.

Successful Management Of Pregnancy With Portal Cavernoma With Splenomegaly With Bicytopenia
Deshmukh P, Tiwari N, Chauhan AR.

Pregnancy Complicated With Sjögren Syndrome
Sharma K, Chauhan AR.

Twisted Paraovarian Cyst
Saini N, Chauhan AR, Paghdiwalla K.

Large Hydrosalpinx Mimicking An Ovarian Cyst
Rane V, Samant PY, Honavar P.

Pregnancy After Repair Of Bladder Exstrophy and Epispadias Complicated by Uterine Prolapse
Sikarwar R, Hatkar PA, Satia MN.

Editorial

Parulekar SV

Historically Apollo was identified as the father of healing as well as music by the ancient Greeks. But there is no mention of his combining healing and music. Perhaps he devoted separate times to the two jobs. ‘Hallelujah to the healer’ was played as part payment for medical services around 4000 BC. Dr Evan O'Neil Kane from Pennsylvania first played music on a gramophone in the operation theater (OT) in 1914. He believed that soft and soothing music helped surgical patients relax. This practice was soon taken up by many surgeons. No scientific studies were done to prove merits and demerits of playing music in the OT. Thus one can say that the widespread use of the trend was not evidence based. Just as the surgeon was considered the master of the OT for the surgical treatment of the patient, the music part of the treatment was also at his discretion, and the others in the OT heard what the surgeon wanted to listen to. One anesthesiologist was bold enough to voice his disgust saying “same songs, same order, every single time, every Thursday!” Of course the surgeons do it not for self, but for the patients. When an operation is done on an awake patient, music of the patient’s choice is supposed to relax the patient, reduce the amount of sedation required and reduce not only anxiety but also pain. The music is supposed to help the OT personnel relax, improve their cognitive function, and elevate their mood. The logic is that the patient benefits when the healthcare providers are feeling good. There are claims that specific types of music help surgeons perform specific types of operations best. Slow and melodic music is supposed to help perform critical steps, while fast music is supposed to help perform wound closures faster and better. Improvement is surgical efficiency is also claimed to lower healthcare costs. Now we understand that the surgeon is not the only person who matters in OT. Each member of the team does a specific job and proper performance of that job is critical for the outcome of the operation. Since there are attendants, nurses, anesthesiologists and surgeons involved in that chronological order, perhaps the music needs to be changed to suit the person and task at a given time. It must be noted that the task of a person does not end when that of another one begins, and all persons continue to be involved throughout the surgery. Hence music for one may not be suitable for the other, and if ten persons are involved at one time, one might require ten different tracks to be played simultaneously, but in such a way that each one hears what he or she prefers or there would be white noise. Added to that is the cacophony of the monitoring equipment signals and alarms, the electrocautery noise and error signals, and people talking professionally or otherwise. The talkers do not respect the music being played and keep quiet. They actually speak louder so as to be heard. If the surgeon is hard of hearing, as he is likely to be with age or after having listened to music while operating over a period of many years, he will keep the volume of the music high. It becomes rather difficult to manage. A study done in a 2007 study showed that noise levels in OTs are often more than 120 decibels, which is louder than on a highway at peak hours. I wonder if anyone has considered the auditory trauma to persons who work in OT all day, six days a week, and not just when they have a slot for operating like the surgeons do. Perhaps a study to find out the time of onset and degree of deafness in such personnel is due.

The surgical team members have to be in constant communication with one another. The surgeon has to tell his assistants, the nurse and the anesthesiologist what he needs. The assistants have to tell him of any difficulties they are facing, or any errors that might not be noticed by the surgeon. The anesthesiologist may need some input from the surgeon and the nurse. The nurse may have to ask another nurse or an attendant for things. The music may make this communication difficult. Either the person may be so engrossed with the music that he does not realize that he is being told to do something, or the music may be so loud that he may not hear what is said. One may be irritated by the music so much that he cannot concentrate on his work. There are concerns expressed that music may make one’s attitude casual, It may make one lose focus and make the results of the operative work less than optimum.

Operative work is not just physical work. Scrubbing and preparing the operation site are mechanical jobs that do not require application of the mind. Some operations are mechanical and can be performed without much thinking. The surgeon may be so experienced that he does not require any thinking while operating. However most people would not fall in that category. For those, and for a surgeon who has to perform difficult and critical operations, concentration and thinking while operating are very important. No matter what management gurus might claim, multitasking produces inferior results for the tasks performed. If the mind is busy with one thing, it cannot be engaged effectively with another thing. Whenever I am operating, I cannot listen to any music because it does not register. If there is any music, I cannot recall which tracks were played in the time between the beginning and the end of the operation. They claim the subconscious registers the music and that achieves the wonderful results which are claimed. This is another statement that is not evidence based. So the music is probably wasted on me when I operate. It could be true for a lot of other people.

The debate on whether to play music in OT will continue, because many persons are involved and their ideas differ widely. We do not want to do something that will be harmful to the patient. But we also do not want to avoid doing something that will improve results. We must satisfy all parties involved as far as possible. So the best solution seems to be to let each person decide whether he wants to listen to music or not while working in the OT. If he does, he can play tracks of his own choice on a personal music player, like a phone or mp3 player, and listen to it using an earphone. He may use a single bud, leaving the other ear open to listen to instructions, answers, signals, alarms and patient’s complaints if awake. If it is found that he cannot concentrate, loses focus and does not perform well while working, his listening to music can be stopped. This personal music playing will also do away with the problem of the institute having to pay for the music which is not royalty free, as each individual becomes liable for possession and playing of his own music.

Cesarean Scar Ectopic Pregnancy - A Perplexing Presentation

Author Information

Mehta N*, Parulekar SV**.

(* Third Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

A cesarean scar ectopic pregnancy, albeit a rare finding, has a rising incidence, due to increasing cesarean and repeat cesarean delivery rates. In addition to this, the diagnosis is often difficult, and a missed diagnosis may lead to major complications, including severe hemorrhage, uterine rupture and hysterectomy.  

Introduction

A cesarean scar ectopic pregnancy refers to implantation of the gestational sac within the myometrium of a previous cesarean delivery scar. There are various theories for explaining its occurrence, but the most plausible one seems to be that the blastocyst enters into the myometrium through a microscopic dehiscent tract, created through the trauma of cesarean section.[1] The incidence ranges at about 1:1800 to 1:2216 normal pregnancies. The mean gestational age at diagnosis is 7.5 ± 2.5 weeks, and the most frequent symptom is painless vaginal bleeding.[2] We present a case which complied with the above mentioned diagnostic symptom, but in which the diagnosis was missed, leading to complications of uterine rupture and formation of an organized hematoma in the pelvis containing fetal parts. The patient eventually required a hysterectomy.

Case Report

A 27-year-old woman, married for 4 years, with previous 1 lower segment cesarean section done 1 year ago, came with complaints of pain in abdomen and bleeding per vaginum for 1 month. The pain in abdomen was continuous in nature, more in the right lower abdomen.

The patient has no other significant medical or surgical history, no bowel or bladder complaints, no complaints of hematuria. The patient’s last menstrual period was on 13th February, 2017.

When she did not get menses in March, she did a urine pregnancy test (UPT) at home, which was positive. The patient gave a history of taking oral combination contraceptive pills regularly prior to that pregnancy, in spite of which she had conceived. She consumed MTP pills (mifepristone followed by misoprostol), procured without a check-up from a local private clinic on 18th March 2017. She started bleeding per vaginum and getting pain in abdomen from beginning of April. An emergency check curettage was done at a private hospital on 17th April 2017. Ultrasonography (USG) was not done prior to the procedure. Her complaints of pain in abdomen and bleeding per vaginum persisted after the curettage. UPT done in early May was positive, and repeat check curettage was done on 20th May 2017. Vaginal bleeding and pain in the right lower abdomen persisted post procedure. The patient got a USG done which was suggestive of uterine dehiscence with macerated fetal parts with a collection/hematoma in the abdominal cavity with hemoperitoneum. The uterus was bulky,11.2 x 6.5x 4.2 cm with a breach in the anterior myometrium of the uterus at the scar site with hypoechoic heterogeneous collection of 10.9 x 4.7 cm with fetal spine and limbs seen within it extending to the right adnexa. At that time she presented to our hospital. On examination her general condition was fair, and vital parameters normal. Abdominal examination showed tenderness in the right iliac fossa. On vaginal examination, a 10 x 12 cm tender mass was felt in the right fornix. The uterus was bulky and the cervical os was closed. Mild bleeding was seen from the os. Her hemoglobin was 8.8, total leucocyte count 11700/cmm. Coagulation profile was normal. Intravenous administration of amoxy-clav, gentamycin and metronidazole was started. One unit of packed cell transfusion was given, and then the patient was taken up for an emergency exploration with consent for obstetric hysterectomy if required.

Intraoperatively, a foul-smelling mass of 10 x 8 cm with a long limb bone and spine of the fetus was seen over the right anterolateral part of the uterus and right adnexa, with omentum and cecum adherent over it. The hematoma and mass with fetal parts was evacuated, and sent for culture sensitivity and histopathology. Uterine rent of approximately 3 cm was seen over the right anterolateral wall of the uterus, with necrotic thick fibrosed edges. A necrotic mass was seen at base of the cecum. Bowel and omentum were separated by the general surgeons’ team. Rest of the bowel was normal. Left anterior surface of uterus was adherent to the anterior abdominal wall, which was separated by sharp dissection. Adhesions between anterior uterine wall and posterior wall of bladder were present. Bladder was pushed down slightly, but could not be separated further due to adhesions. Subtotal obstetric hysterectomy was done. Part of the rent in the lower segment that could not be removed with the specimen was sutured by figure of 8 stitches with vicryl no 0. There was no evidence of any injury to or leak from the bladder or the ureters. Intraoperative estimated blood loss was 2.3 liters. Intraoperatively, 2 units blood and 2 units fresh frozen plasma were transfused. An intraperitoneal drain was kept in the pouch of Douglas, which was removed on the 5th postoperative day. Postoperatively, the patient was vitally stable, and received 3 units of blood transfusion. The specimen sent for culture, grew E coli sensitive to gentamycin. She was given injectable gentamycin for a total of 7 days. She made an uneventful recovery. A computerized tomography (CT) was done prior to discharge from the hospital. It confirmed that intraabdominal structures were normal and no fetal parts were left behind. Histopathology of the hysterectomy specimen confirmed the diagnosis of cesarean scar ectopic pregnancy.

Figure 1. CT scan of abdomen and pelvis showing fetal spine (arrow).

Figure 2. CT scan of abdomen and pelvis showing fetal long limb bone (arrow).

Figure 3. Operative findings: a mass is seen anterior and to the right of the uterus.

Discussion

A cesarean scar pregnancy may be misdiagnosed as a threatened abortion; or incomplete abortion, as in this case. Differentiating features include a greater volume of bleeding in an abortion, and cramp like abdominal pain.[3] Transvaginal USG is the first line tool for diagnosis of a cesarean scar ectopic pregnancy. Magnetic resonance imaging should be done if the USG results are equivocal or inconclusive.[4] Sonographic criteria include an empty uterus, empty cervical canal, development of the gestational sac in the anterior part of the lower uterine segment or uterine isthmus and an absence of healthy myometrium or presence of thinned out myometrium between the bladder wall and the gestational sac.[5] Additional criteria are presence of placenta or gestational sac embedded in the uterine scar, a triangular (< 8 weeks) or rounded or oval (> 8 weeks) gestational sac that fills the niche of the scar, a closed and empty cervical canal, the presence of a prominent or rich vascular pattern at or in the area of the scar in the presence of a positive pregnancy test.[6]

Treatment guidelines are lacking. Hysterectomy is the first line of management in patients not wishing to preserve fertility, or with heavy uncontrolled bleeding. For patients wishing to preserve fertility, systemic or locally injected methotrexate is used; or procedures such as visually guided suction curettage or transvaginal aspiration, hysteroscopic removal or isthmic resection are done. Medical and surgical options may be carried out in conjugation with each other or alone. Uterine artery embolization may also be done with any of the above procedures.[4] Some believe that USG-guided methotrexate injection is the treatment of choice to terminate cesarean scar pregnancy.[3]

The case presented was intriguing. The patient took MTP pills without a check-up and prescription, which could have resulted in the rupture of the cesarean scar ectopic pregnancy. It is possible that there was not disruption of the site of implantation sufficient to cause fetal demise, so that it continued to grow. It is also possible that the scar gave way with the use of the drugs, and the pregnancy got extruded through the rent and continued to grow in the disrupted scar. The curettage that was done subsequently was not preceded by an USG. Hence the diagnosis was missed. It appears that it missed the scar ectopic pregnancy too, and the fetus continued to grow. The second curettage was also done without a prior USG. In case repeated curettages are required, it is essential that one obtains a pelvic USG first, so that unusual conditions like a scar ectopic pregnancy are not missed. It is probable that removal of most of the fetus was done at this time, so that only the fetal vertebral column and a long limb bone were left behind. An infection was inevitable due to repeated surgeries and incomplete removal of the products of conception. The size of the fetal spine and the limb bone were suggestive of a fetal age of about 10-12 weeks. That fits the probable clinical course described.

A differential diagnosis is that there could have been a uterine perforation during the curettage, following which the pregnancy grew outside the uterine cavity, with or without secondary implantation. A third possibility is that the patient may have mistaken dates, and a late first or early second trimester suction curettage resulted in uterine perforation, with an incomplete curettage and partial extrusion of the foetal parts through the defect in the uterine wall. The incidence of uterine perforations during elective first trimester abortions is 0.08%. [7] The foetal spine and limb bones were found, which indicates that the gestational age of the fetus would have been at least 10-12 weeks. [8] 

This incident cannot emphasize enough the need for routine USG before medical or surgical MTP to make the procedure safer for the woman.

Acknowledgment

We thank Dr Durga Valvi for performing the operation.

References

1. Maymon R, Halperin R, Mendlovic S, Schneider D, Vaknin Z, Herman A, Pansky M. Ectopic pregnancies in Caesarean section scars: the 8 year experience of one medical centre. Human Reproduction. 2004 Feb 1;19(2):278-84.
2. Rotas MA, Haberman S, Levgur M. Cesarean scar ectopic pregnancies: etiology, diagnosis, and management. Obstetrics & Gynecology. 2006 Jun 1;107(6):1373-81.
3. Seow KM, Huang LW, Lin YH, Yan‐Sheng Lin M, Tsai YL, Hwang JL. Cesarean scar pregnancy: issues in management. Ultrasound in obstetrics & gynecology. 2004 Mar 1;23(3):247-53.
4. Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, et al. Ectopic Pregnancy. Williams Obstetrics, 24th ed. Pp 391-92
5. Godin PA, Bassil S, Donnez J. An ectopic pregnancy developing in a previous caesarian section scar. Fertility and sterility. 1997 Feb 1;67(2):398-400.
6. Timor-Tritsch IE, Monteagudo A, Santos R, et al. The diagnosis, treatment, and follow-up of cesarean scar pregnancy. Am J Obstet Gynecol 2012;207:44.e1-13.
7. Chen LH, Lai SF, Lee WH, Leong NK. Uterine perforation during elective first trimester abortions: a 13-year review. Singapore medical journal. 1995 Feb;36:63-7.
8. Callen PW. The Foetal Musculoskeletal System. Ultrasonography in Obstetrics and Gynaecology, 5th ed. New Delhi: Elsevier,2008. Pp 421.

Citation

Mehta N, Parulekar SV. Cesarean Scar Ectopic Pregnancy - A Perplexing Presentation. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/cesarean-scar-ectopic-pregnancy.html

Degenerated Uterine Leiomyoma Masquerading As An Ovarian Cyst

Author Information

Parulekar SV. Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.

Abstract

A cystic pelvic mass is most likely to be an ovarian cyst. Other possible diagnoses include a leiomyoma with cystic degeneration, paraovarian cysts, endometriomas, pelvic hematocele, encysted ascites, hydatid cyst and full bladder. It may be difficult to distinguish the conditions from one another clinically and sometimes even on ultrasonography. A case is presented here in which the clinical and ultrasonographic diagnosis was ovarian cyst, while it turned out to be a leiomyoma with cystic degeneration.

Introduction

Uterine leiomyoma is the commonest benign tumor in the female pelvis. It occurs in up to 30% of women in the reproductive age group.[1,2] Most of the leiomyomas are solid.[3] Some of them undergo cystic degeneration. It is the second commonest type of degeneration in a leiomyoma and the commenest one during pregnancy.[3] A cystic degeneration needs to be differentiated from other cystic masses in the pelvis. Ultrasonography (USG) is a very useful method of differentiating pelvic cystic masses. However it can be in error at times. An unusual case of a cystic degeneration in a   subserosal leiomyoma is presented.

Case Report

A 36-year-old, asymptomatic woman presented to our outpatient clinic with a USG report showing a loculated cyctic tumor in the pelvis. It had been detected by a private practitioner during evaluation of chronic mild pelvic pain. It was closely related to the top of the uterus. Its size was 7 x 6 x 5.5 cm. It was loculated and contained clear fluid. There was no solid area within the tumor, or on its surface. The right ovary was normal, while the left ovary was not seen. The remaining intraperitoneal structures were normal. A diagnosis of a benign ovarian tumor was made.

She was a second para with two normal deliveries. Both children were well. Her chief complaint was mild chronic pelvic and lower abdominal pain for 4 months. There were no aggravating or relieving factors for that pain. Her menstrual cycles were regular, painless, with moderate flow for 3-4 days every month. Her last menstrual period had been 10 days ago. Her past history was not contributory. Her general and systemic examination revealed no abnormality. Findings of speculum examination of the vagina were normal. Abdominal examination revealed normal findings. Bimanual pelvic examination showed a normal sized uterus, and a 5-6 cm diameter nontender, cystic mass above the uterus. Lateral fornices were empty. A provisional diagnosis of an ovarian cystic tumor was made. Reports of hemogram, fasting and postprandial plasma sugar levels, liver and renal function tests, chest radiograph and electrocardiogram were normal. Her CA 125 level was 18 units/ml. USG performed at our center confirmed the findings of USG done prior to presenting to our center.

The patient opted for a laparotomy rather than laparoscopic surgery. At laparotomy, a kidney-shaped loculated lump measuring about 6 x 5 x 5 cm was found above the uterus, obscuring it. It was cystic and the fluid within it was clear. Further examination revealed that both the ovaries were normal and the tumor was connected by a pedicle measuring 2 mm x 2 mm to the center of the uterine fundus. A diagnosis of cystic degeneration in a subserous pedunculated leiomyoma of the uterus was made. The pedical was electrocauterized and cut. The uterus did not show any other leiomyoma. Hence no further surgery was done. The patient made an uneventful recovery. Histopathology of the mass confirmed the diagnosis of cystic degeneration in a leiomyoma.

Figure 1. Ultrasonography of pelvis showing bilobed cystic mass (yellow arrows) above the uterus (U).

Figure 2. Laparotomy findings: A bilobed cystic tumor is seen above the uterus. The pedicle of the tumor (POT) is very small. LO: left ovary; RO: right ovary.

Figure 3. Leiomyoma with cystic degeneration.

Discussion

A leiomyoma is a smooth muscle tumor.[2,4] Owing to a compromise of its blood supply, it tends to undergo degeneration.[4] Different types of degeneration seen in a leiomyoma include hyaline, cystic, myxomatous and calcific. A degeneration is seen more commonly in the center of large intramural leiomyomas, where the blood supply is less than at the periphery. When the pedicale of a subserous or submucous leiomyoma elngates and thins, the blood supply to the leiomyoma may get compromised and it may undergo degeneration. In the case presented, the pedicle of the fundal subserous leiomyoma was very small and thin, and there was virtually no blood supply to the leiomyoma. It had not become adherent to any other intraperitoneal structure like bowel, its mesentary or omentum, from which it could have derived its blood supply. It was likely that the pedicle would have broken some time soon and the leiomyoma would have become a free intraperitoneal leiomyoma.

A USG is very useful to distinguish between various conditions that present as cyctic pelvic masses, such as paraovarian cysts, endometriomas, pelvic hematocele, encysted ascites, hydatid cyst and chronic retention with overflow of urine.[4,5,6,7] Performed carefully a USG would have detected the ovaries separate from the mass, and possibly the connection between the uterus and the mass in the case presented. Then a correct diagnosis could have been made preoperatively. It was not considered necessary to perform a computerized tomography or magnetic resonance imaging, because the diagnosis seemed correct. Retrospectively thinking, it is felt that it would not have mattered much, as the mass was benign and needed to be removed, whether ovarian or uterine in origin.

Conclusion

A cystic mass close to the uterus in the female pelvis is not always an ovarian tumor. Though most of the conditions are benign, it is still necessary to make a correct diagnosis of the condition, so that appropriate treatment can be planned. Imaging techniques like USG, CT and MRI are useful for differentiating cystic pelvic masses from one another in an asymptomatic woman.

References

1. Prayson RA, Hart WR. Pathologic considerations of uterine smooth muscle tumors. Obstet Gynecol Clin North Am 1995;22:637-57.
2. Cramer SF, Patel A The frequency of uterine leiomyomas. Am J Clin Pathol 1990;94:435-8.
3. Murase E, Siegelman ES, Outwater EK, Perez-Jaffe LA, Tureck RW. Uterine Leiomyomas: Histopathologic Features, MR Imaging Findings, Differential Diagnosis and Treatment. Radiographics 1999;19:1179-97.
4. Vitiello D, McCarthy S. Diagnostic imaging of myomas. Obstet Gynecol Clin N Am 2006;33:85-95.
5. Cohen JR, Luxman D, Sagi J, et al. Ultrasonic "honeycomb" appearance of uterine submucous fibroids undergoing cystic degeneration. J Clin Ultrasound. 1995;23:293–6.
6. Reddy NM, Jain KA, Gerscovich EO. A degenerating cystic uterine fibroid mimicking an endometrioma on sonography. J Ultrasound Med. 2003;22:973–6.

Citation

Parulekar SV. Degenerated Uterine Leiomyoma Masquerading As An Ovarian Cyst. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/degenerated-uterine-leiomyoma.html

Scar Ectopic Pregnancy Managed Laparoscopically

Author Information

Shah NH*, Paranjpe S**, Shah VN***.

(* Director, Vardann Multispeciality Hospital, ** Director, Velankar Hospital and Paranjpe Maternity Home, *** Consultant Anesthetist, Vardann Multispeciality Hospital, Mumbai, India.)

Abstract

Intramural pregnancy with implantation in a previous cesarean section (CS) scar is probably the rarest location for ectopic pregnancy. The true incidence of pregnancy occurring in a uterine scar has not been determined because so few cases have been reported in the literature. However, the incidence of such cases seems to be on the rise. Here we present a case of a 28-year-old woman with a history of previous 1 CS 5 years ago, who presented with a history of 2 months amenorrhea with positive urine pregnancy test and pain in abdomen. USG was suggestive of scar ectopic, which we managed laparoscopically.

Introduction

Any form of ectopic pregnancy is one of the leading causes of mortality and morbidity in women of child bearing age group. Among ectopic pregnancies, fallopian tubal ectopic is the commonest. Apart from the fallopian tube, an ectopic pregnancy can also occur in the cervix, ovary, previous cesarean scar, or abdomen. An intramural pregnancy with implantation occurring in the previous cesarean scar is probably the rarest location for all ectopic pregnancies.[1] The exact incidence of ectopic pregnancy occurring in previous cesarean scar has not yet been determined because of small number of cases reported. However, incidence is on the rise.[1] The diagnosis can be done by ultrasonography and confirmed by magnetic resonance imaging (MRI) or by laparoscopy/ laparotomy.

Case Report

A 28-year-old woman came to our OPD with complaints of pain in abdomen with a history of 2 months amenorrhea. A urine pregnancy test was done and was positive. Patient was second gravida with a history of previous one LSCS done 5 years back. An ultrasonography was done which showed a regular endometrial cavity with a gestational sac at the anterior border of the myometrium corresponding to 7 weeks pregnancy suggestive of a scar ectopic pregnancy. On examination, patient had tenderness over the lower abdomen. On vaginal examination uterus was 6-8 weeks size. External os was closed.

Figure 1. Sonography showing scar ectopic pregnancy.

A laparoscopy was done, which showed bladder and anterior wall adhesions to the previous uterine scar. A bulge was seen at the previous uterine scar. Keeping in mind the severity of bleeding, bilateral uterine arteries were ligated at the origin beforehand.

Figure 2. Uterine artery ligation done beforehand.

Local injection of vasopressin was done around the lower uterine scar area to minimize the bleeding. The adhesions over the previous uterine scar were separated. Bladder peritoneum was dissected off the uterus. After separating the bladder peritoneum off the uterus, a clear blue bulge was visible protruding from the uterine scar. The thinned out uterine wall over the protruding sac was incised. The gestational sac was exposed and was enucleated completely along with the fetus and the choriodecidual tissue and were removed in an endo-bag. The previous uterine scar was excised. After confirming hemostasis, the uterine incision was closed with polyglactin No. 1. Postoperative period was uneventful and patient was discharged on 3rd day.

Figure 3. Enucleation of sac being performed.

Figure 4. Uterus sutured after removal of contents.

Discussion

A scar ectopic pregnancy is a rare form of ectopic pregnancy whose incidence is rising due to increasing number of cesarean sections and increasing diagnostic sensitivity. The incidence of scar ectopic has gone to 1 in 2000 pregnancies.[2] The commonly accepted theory for the formation of a scar ectopic pregnancy is that either the embryo implants through the wedge defect over the lower uterine segment or it passes through a microscopic fistula extending into the scar.[3] Other causes of myometrial implantation also include adenomyosis, previous dilatation and curettage, manual removal of placenta and in vitro fertilization.[4]

Clinical presentation of a scar ectopic pregnancy can vary from being asymptomatic to vaginal bleeding, abdominal tenderness or even hypovolemia and shock in case of rupture.[5] Barring a few exceptions, most of the cases of scar ectopic pregnancy are diagnosed and managed in the first trimester only.[2] A differential diagnosis may include cervical ectopic pregnancy and placenta accreta.[6]

The time interval of a scar implantation from the previous cesarean section ranges from 5 months to 12 years. The first laparoscopic surgical management of a scar ectopic pregnancy was done by Lee et al.[4] Principles of laparoscopic management include:

• Scar ectopic pregnancy is removed in an endo- bag
• Bleeding is minimized by locally injecting vasopressin
• Hemostasis is achieved by bipolar cautery
• Uterine incision is closed by endo- suturing

Primary open laparotomy may be considered in cases presenting late, or in shock or non-availability of endoscopic expertise and instruments. Various other treatment modalities have also been used in cases of scar ectopic pregnancies which include systemic or local methotrexate, or hysteroscopic evacuation of ectopic pregnancy. A simple dilatation and curettage has also been done but in a review of literature by Arslan [7], out of the 9 women who underwent dilatation and curettage, 8 women required surgical intervention due to complications of heavy bleeding during dilatation and curettage.

Our patient underwent a successful laparoscopic approach with minimal amount of bleeding and no intra -operative complications.

Conclusion

With increasing number of cesarean operations, we can expect more number of scar ectopic pregnancies in the future, wherein we have to keep in mind the possibility of a scar ectopic in patients with even 1 previous cesarean section. A transvaginal sonography is a fairly reliable modality of diagnosing a scar ectopic pregnancy. There is no general consensus for management of a scar ectopic but is usually decided upon factors such as gestational age, scar rupture, expertise of obstetrician and facilities available.

References

1. Fylstra DL, Pound-Chang T, Miller MG, Cooper A, Miller KM. Ectopic pregnancy within a cesarean delivery scar: a case report. Am J Obstet Gynecol. 2002; 187(2):302–4.
2. Rotas MA, Haberman S, Levgur M. Cesarean scar ectopic pregnancies: etiology, diagnosis, and management. Obstet Gynecol. 2006; 107(6):1373–81.
3. Vial Y, Petignat P, Hohlfeld P. Pregnancy in a cesarean scar. Ultrasound Obstet Gynecol. 2000; 16(6): 592–3. 
4. Lee CL, Wang CJ, Chao A, Yen CF, Soong YK. Laparoscopic management of an ectopic pregnancy in a previous caesarean section scar. Hum Reprod. 1999; 14(5):1234–6.
5. Graesslin O, Dedecker F Jr, Quereux C, Gabriel R. Conservative treatment of ectopic pregnancy in a cesarean scar. Obstet Gynecol. 2005: 105(4): 869–71.
6. Ofili-Yebovi D, Ben-Nagi J, Sawyer E, Yazbek J, Lee C, Gonzalez J et al. Deficient lower-segment cesarean section scars: prevalence and risk factors. Ultrasound Obstet Gynecol. 2008; 31: 72–7. 
7. Arslan M, Pata O, Dilek TU, Aktas A, Aban M, Dilek S. Treatment of viable cesarean scar ectopic pregnancy with suction curettage. Int J Gynecol Obstet. 2005; 89(2):163–6.

Citation

Shah NH, Paranjpe S, Shah VN. Scar Ectopic Pregnancy Managed Laparoscopically. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/scar-ectopic-pregnancy-managed.html

Management Of Secondary Postpartum Hemorrhage By Uterine Artery Embolization

Author Information 

Deshpande PS*, Prasad M**, Gupta AS***

(*Third year Resident, **Assistant Professor, ***Professor and Head of the Unit, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai.)

Abstract

In modern obstetrics, the use of vascular interventional radiology has proven to be of great benefit in situations where blood loss is anticipated or has occurred. Here we present a case of secondary postpartum hemorrhage that was successfully managed by uterine artery embolization thus reducing the surgical morbidity that the patient would otherwise be subjected to.

Introduction

Uterine artery embolization is a radiological procedure where the uterine arteries are selectively catheterized under fluoroscopy guidance and small particles are delivered to block the flow through them. This conservative technique of reducing blood flow to the uterus in conditions where massive blood loss is expected has been used successfully perioperatively in fibroids, adenomyosis, dysfunctional uterine bleeding, placenta accreta, uterine artery pseudo aneurysms, uterine arterio-venous malformations, and for other indications.[1] Use of uterine artery embolization for controlling postpartum hemorrhage (PPH) was first reported in 1979.[2] Since then this procedure is gaining popularity as a non-invasive option for treatment of PPH.

Case Report

A 24 year old, primigravida was referred to our tertiary care center on day 8 of emergency LSCS done for twin pregnancy at term. The twins weighed 2.1 and 1.1 kg respectively, and were doing well at the time of admission. She was referred because she had persistent abdominal pain and fever; and ultrasonography suggested ascites. On arrival, she was febrile, pulse and blood pressure were 100 beats per minute and 110/70 mm of Hg respectively. Cardiovascular and respiratory system were normal. Pallor was present. Abdomen was soft with diffuse tenderness, and uterus was just palpable on abdominal examination.  Pfannenstiel scar had healed well. There was no foul smelling lochia. Uterus was well retracted. Hemoglobin was 6.1 gm%. Complete blood count was 12,500 cells/cc. Differential counts were within normal range. Neutrophils, lymphocytes and eosinophils were 76, 20, and 4 % respectively. Liver and renal function parameters were normal. Coagulation profile was normal. Prothrombin time was 14.4 and the control was 13.1. International normalized ratio was 1.0. One unit of packed red blood cells was transfused. Ultrasonography (USG) showed moderate amount of hemoperitoneum. An angiography by contrast enhanced computerized tomography (CECT) was done. It also showed hemoperitoneum. A blush, suggestive of active bleeder, near the left uterine artery was seen, in the arterial phase. In view of relative hemodynamic stability of the patient, conservative mode of treatment was preferred and interventional radiologist team was involved. They suggested angiography, localization of the bleeder, embolization or coil placement for hemostasis. Written, valid, informed consent was taken from the patient and her husband after explaining the risks, success rate, need for emergency exploration in case of failure. The procedure was done under antibiotic cover and local anesthesia. Catheterization of the femoral artery was done by Seldinger technique and a 5 French catheter with guide wire was inserted into the aorta. A non-selective aorto-iliogram (figure 1) was done to look for the pelvic bleeder. Dye was seen oozing out near the left uterine artery area with tortuous collateral's.(figure 2) Selective uterine artery catheterization (by Roberts uterine artery catheter) was done on left side and 500 micron size polyvinyl alcohol particles were injected mixed with equal amount of contrast. Similar procedure was repeated on the right side. Post embolization repeat angiography performed showed significant reduction in extravasation of the dye from the site of initial leak. The procedure took approximately 90 minutes. She recovered uneventfully and subsequently maintained a good hemodynamic status. Strict immobilization was implemented for 12 hours and puncture site was checked after 2 days. Clinical and laboratory parameters remained normal and she was discharged on day 7 of the procedure.

Figure 1. Aorto-iliogram.

Figure 2. Active blush in the left uterine artery region.

Figure 3. Stent in the uterine artery.

Figure 4. Digital subtraction angiography. Post-embolization image showing reduced vascularity.

Discussion 

Secondary or late postpartum hemorrhage is defined as bleeding 24 hours to 12 weeks after delivery. It is mostly seen within 1-2 weeks and in 1% cases. Apart from sub-involution of the uterus and retained products of conception, sepsis, uterine artery pseudoaneurysm with bleeding from the tortuous uterine plexus are other causes of secondary PPH.[3] Our case had two high risk factors for secondary PPH; twin pregnancy and cesarean section. Selective arterial embolization is a minimally invasive, non surgical technique of uterine devascularisation. Management options in our case included surgical exploration with step-wise devascularisation procedures or use of vascular interventional radiology. 

Debating about the two available options, subjecting the patient to re-surgery increases the morbidities associated with the same. Also placing hemostatic sutures in the presence of sepsis which is inadvertently going to be there can be hazardous. Due to the intricate pelvic architecture of arteries and veins, if it is not possible to locate the bleeder, then the chances of hysterectomy are high. This problem is solved using a real time diagnostic modality like angiography which can accurately locate the bleeder. In addition, interventional radiology procedures are done under local anesthesia and hence the regional/ general anesthesia complications are also avoided.

Thus, in a tertiary care center, with available interventional radiology facilities, hemodynamically stable case, with no signs of peritoneal irritation like guarding, rigidity and rebound tenderness and USG suggestive of moderate amount of hemoperitoneum which could resolve over time, a conservative mode of management seems to be a better option.

Touboul et al in 2008 described selective arterial embolization in 102 patients with life-threatening PPH with an overall success rate of 71.5%.[4] In 2009, Kirby et al published a retrospective multi center study evaluating 43 patients who underwent arterial embolization for PPH and found clinical success of approximately 80%.[5]  Kim et al in 2013 published a retrospective study of 257 consecutive patients who underwent pelvic arterial embolization for PPH and confirmed this procedure to be a safe and effective alternative to surgical intervention. [6]

A review conducted previously had concluded that among the various options available, namely arterial embolization and iliac artery ligation, no particular procedure was better than the other. It was also acknowledged that performing randomized controlled trials in such situations may not be feasible.

A recent review by Sathe et al have stated evidence regarding long term problems like infertility is not sufficient. Also, since only a small number of patients have been reported (2100 in their review), even to this date, evidence is insufficient to choose any one particular procedure in precedence of the other. Clinical decision making should be based on severity of the situation and the available facilities. 

Secondary PPH usually indicates presence of infection. There is no last word in the literature discussing the pros and cons of uterine artery embolization in the presence of overt or covert infection as randomized controlled trials are difficult to design due to various causes of the same.

Conclusion

This case is presented to highlight the fact that even in resource-constrained countries like India; PPH can be effectively managed in tertiary care center using uterine artery embolization. Appropriate patient selection for the same is of utmost importance, and avoids surgical exploration and hysterectomy. 

Acknowledgement

Dr. Dev Thakkar, Assistant Professor, Department of Radiology, KEM Hospital, Mumbai for performing the procedure and Dr. Hemant Deshmukh, Head of the Department of Radiology, KEM Hospital, Mumbai) for allowing us to publish this case.

References

1. Desai PK. Uterine Artery Embolisation. Radiopedia.org [Internet Journal]. Available from: https://radiopaedia.org/articles/uterine-artery-embolisation
2. Wee L, Barron J, Toye R. Management of severe postpartum haemorrhage by uterine artery embolization. Br J Anaesth. 2004; 93(4):591–4.
3. Cunningham FG. The Puerperium. In Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BS, editors. Williams’ Obstetrics. 24th ed. New York: Mc Graw hill 2014; pg 670.  
4. Touboul C, Badiou W, Saada J, Pelage J-P, Payen D, Vicaut E, et al.  Efficacy of Selective Arterial Embolisation for the Treatment of Life-Threatening Post-Partum Haemorrhage in a Large Population. PLoS ONE. 2008; 3(11): e3819. 
5. Kirby JM, Kachura JR, Rajan DK, Sniderman KW, Simons ME, Windrim RC, et al. Arterial embolization for primary postpartum hemorrhage. J Vasc Interv Radiol. 2009;20(8):1036-45. 
6. Kim TH, Lee HH, Kim JM, Ryu AL, Chung SH, Seok LW. Uterine artery embolization for primary postpartum hemorrhage. Iran J Reprod Med. 2013;11(6):511–8.
7. Doumouchtsis SK, Papageorghiou AT, Arulkumaran S. Systematic review of conservative management of postpartum hemorrhage: what to do when medical treatment fails. Obstet Gynecol Surv. 2007;62(8):540-7
8. Sathe NA, Likis FE, Young JL, Morgans A, Carlson-Bremer D, Andrews J. Procedures and Uterine-Sparing Surgeries for Managing Postpartum Hemorrhage: A Systematic Review. Obstet Gynecol Surv. 2016;71(2):99-113.

Citation

Deshpande PS, Prasad M, Gupta AS. Management Of Secondary Postpartum Hemorrhage By Uterine Artery Embolization. JPGO 2017. Volume 4 No.7. Available from:
http://www.jpgo.org/2017/07/management-of-secondary-postpartum.html

Successful Management Of Pregnancy With Portal Cavernoma With Splenomegaly With Bicytopenia

Author Information

Deshmukh P*, Tiwari N**, Chauhan AR***.

(* Third Year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India).

Abstract

We present a case of 24 year old woman with chronic portal vein thrombosis with splenomegaly with extrahepatic portal venous hypertension with bicytopenia, with a successful maternal and neonatal outcome.

Introduction

Pregnancy is a hypercoagulable state.[1] This may predispose to high chances of portal vein thrombosis (PVT), where a clot may partially or completely occlude the lumen of the portal vein. Management of pregnancy in portal cavernoma is difficult due to various co-morbidities. Mother and fetus have increased morbidity and mortality. Patient should be managed in higher center with multidisciplinary approach.

Case Report

A 24-year-old primigravida with spontaneous conception presented to us at full term in early labor. She was a case of primary infertility under evaluation and on routine investigations, she had low platelet and WBC counts. Patient had no history of hematemesis, petechiae or any major illness. Her coagulation profile, fasting and postprandial sugar levels, thrombophilia profile, liver and renal function tests were normal. Bone marrow aspiration was suggestive of iron deficient reactive marrow and trephine bone biopsy was suggestive of hypercellular reactive marrow. Her abdominal ultrasonography was suggestive of gross splenomegaly with dilated portal vein with multiple collaterals, most probably due to extrahepatic portal vein hypertension. There was chronic portal vein thrombosis with large portal vein formation. Upper gastroscopy done was suggestive of small varix in esophagus with normal duodenum and pylorus. During pregnancy, she was started on tablet propranolol with iron and folic acid tablets.

During labor, platelet count was 35,000 /cu mm and WBC count 2100/cu mm. Hepato-portal Doppler was suggestive of portal cavernoma with splenomegaly and epigastric collaterals. Obstetric doppler was suggestive of early fetoplacental insufficiency. Her coagulation profile was deranged -her initial INR was 1.3, which further deteriorated to 2.2, hence 4 units of fresh frozen plasma (FFP) were given in early labor. During active stage of labor 8 units platelet and 4 units FFP were given and patient was monitored. Gastroenterologist and hematologist opinion was taken. Her labor progress was uneventful. She delivered a female child of 2.12 kg. Her postpartum period was uneventful. Post-delivery, platelet count was 61,000/ cu mm and TLC was 8000/cu mm. Propranolol was continued. Patient was discharged on day 6 post-delivery and was advised endoscopy and regular follow up with gastroenterologist.

Discussion

Portal vein thrombosis (PVT) is classified into acute and chronic types. Acute portal vein thrombosis presents within 60 days with symptoms like abdominal pain or distention, diarrhea, nausea, vomiting, anorexia and fever with absence of portal carvernoma and portal hypertension. Chronic PVT is mostly asymptomatic and associated with portal cavernoma and/or complications of portal hypertension. PVT is also classified into complete and partial types, and each is subclassified based on the presence or absence of portal cavernoma. Patients with complete PVT have higher frequency of cavernous transformation than those with partial PVT. The common causes of PVT include cirrhosis, neoplasm, myeloproliferative disorders, inflammatory disorders like pancreatitis and thrombophilias. PVT is a relatively common complication of liver cirrhosis. With the stoppage of the portal venous blood flow, there is a compensatory mechanism of venous rescue due to rapid development of collaterals to bypass the obstruction which is called “cavernomatous transformation”.[2] Because of cirrhosis and advanced liver disease pregnancy occurs rarely in these patients, but in non-cirrhotic portal hypertension, pregnancy can occur as liver function is preserved and patients are not infertile. Complications associated with these pregnancies are jaundice, jaundice with ascites, and gastrointestinal bleeding.[3] In a normal pregnancy many physiological and hormonal changes lead to spider naevi due to hyperdynamic circulation which is similar to decompensated chronic liver disease.[4] 

Fetal and maternal outcomes are favorable for most pregnancies reaching gestation week 20. High platelet counts appear to increase the risk for unfavorable outcome. Pregnancy should not be contraindicated in stable PVT patients.[5] Maternal outcome is excellent on anticoagulation due to limited thrombosis recurrence. However, in patients treated with anticoagulation, the rate of miscarriage and preterm birth appears to be increased. Also, post-cesarean section bleeding can be severe. Vaginal delivery and brief interruption of anticoagulation around delivery may reduce bleeding in these patients.

Prognosis of pregnancy with portal hypertension depends on the underlying cause and the degree of derangement of liver function. Termination of pregnancy should be considered in patients with recurrent hematemesis, deranged liver functions and decompensated cirrhosis, especially with abnormal coagulation profile. Maternal mortality in these patients ranges between 2 and 18% and is attributed to hematemesis, hepatic coma, postpartum hemorrhage; it is maximum with cirrhosis.

Conclusion

In pregnant PVT patients, maternal and fetal outcome is favorable with proper management, and pregnancy outcome is good in patients whose disease is well controlled prior to pregnancy.[5] A multidisciplinary approach seems to be key to management. Elective cesarean section may be necessary in cases with digestive varices. However, vaginal delivery, with a passive second stage, seems to be relatively safe and less morbid when maternal and fetal tolerance permits.[6] 

References

1. Hosley CM, McCullough LD. Acute neurological issues in pregnancy and the peripartum. Neurohospitalist. 2011;1(2): 104–16.
2. Bayraktar Y, Tuncer ZS, Kabukçu A, Uzunalimoğlu B, Ayhan A. Pregnancy complicated by congenital hepatic fibrosis with cavernous transformation of the portal vein: a case report. Am J Obstet Gynecol. 1997; 177(2): 459–61.
3. Aggarwal N, Negi N, Aggarwal A, Bodh V, Dhiman RK. Pregnancy with portal hypertension. J Clin Exp Hepatol. 2014; 4(2):163–71.
4. Westbrook RH, Dusheiko G, Williamson C. Pregnancy and liver disease. J Hepatol. 2016; 64(4): 933–45.
5. Hoekstra J, Seijo S, Rautou PE, Ducarme G, Boudaoud L, Luton D, et al. Pregnancy in women with portal vein thrombosis: Results of a multicentric European study on maternal and fetal management and outcome. J Hepatol. 2012; 57(6):1214–9.
6. Ducarme G, Plessier A, Thuillier C, Ceccaldi PF, Valla D, Luton D. Pregnancy and delivery in patients with portal vein cavernoma. Gynecol Obstet Invest 2009; 68(3):196–8.

Citation

Deshmukh P, Tiwari N, Chauhan AR. Successful Management Of Pregnancy With Portal Cavernoma With Splenomegaly With Bicytopenia. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/successful-management-of-pregnancy-with.html

Pregnancy Complicated With Sjögren Syndrome

Author Information

Sharma K*, Chauhan AR**.

(* Senior Resident, ** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India).

Abstract

This is a case of pregnancy complicated with Sjögren syndrome (anti Ro antinuclear antibodies) with a past history of hypokalemic paralysis and distal renal tubular acidosis (dRTA). Patient was successfully managed by a multidisciplinary approach, had a preterm delivery and was discharged with a healthy baby.

Introduction

Sjögren syndrome is an autoimmune disease characterized by the presence of autoimmune antibodies which cause destruction of salivary and lacrimal glands, primarily resulting in dry eyes and dry mouth.[1]  If Sjögren syndrome occurs primarily it is called primary Sjögren syndrome, and if it occurs in association with other connective tissue disorders it is called secondary Sjögren syndrome.[2]

Case Report

Our patient 28-year-old primigravida with 32 weeks gestation presented to the emergency room with pain in abdomen. Pregnancy up to this point was uneventful. Patient’s general examination was normal. On per abdomen examination, uterus was 30 weeks gestation and relaxed with normal liquor and a normal regular fetal heart rate. On per vaginal examination, os was closed, posterior and uneffaced.

Patient was diagnosed in 2013 as a case of Sjögren syndrome when she had an episode of hypokalemic paralysis and facial palsy, at which time her potassium level was 2.9 mEq/ l (normal value 3.5- 5 mEq/ l). On investigation at that time, it was found that hypokalemia was due to distal renal tubular acidosis (dRTA). USG done showed left renal stone which was due to hypercalciuria and hyperphosphaturia which is a feature of dRTA. Patient was found to have hypothyroidism, Raynaud’s phenomena and dRTA with presence of antinuclear antibody (ANA) and anti SS-A Ro and anti SS-B La antibodies pointing towards diagnosis of Sjögren syndrome. She was treated in the ICU and was on ventilator support. She was advised confirmatory tests like Schimer’s test and lip biopsy but she did not get them done. She recovered completely but was lost to follow up and stopped all her medications on her own. 

In the current pregnancy, patient was admitted and investigated; case was reviewed by physician, endocrinologist, rheumatologist and obstetrician. In her present reports, VBG (venous blood gas) showed a pH of 7.35 (normal 7.35-7.45), potassium level was 3.9 mEq/ l, bicarbonate was 13 mEq/ l (normal 7-13 mEq/ l) and urine calcium to creatinine ratio was 0.26 (normal <0.2). Her USG abdomen showed no renal stones. Her urine routine and vaginal swab were normal ruling out any source of infection. Her 2 D echocardiography was normal and ENT and ophthalmological examination were normal ruling out systemic complications if any. ANA blot was done which was strongly positive for anti SSA Ro antibodies. Her obstetric USG showed a single live intrauterine pregnancy of 30 weeks with normal liquor and no IUGR, with normal Doppler. The fetal 2D echocardiography was normal with no conduction defects; anomaly scan was also normal. She was started on tablet aspirin 75 mg daily, tab hydrochloroquine 200 mg daily, tab thyroxine 75 µg daily, syrup potassium chloride 10 ml thrice a day, along with calcium, vitamin D and hematinics.  She was given steroids for fetal lung maturity. She was advised to have coconut water and fruit juices which are potassium rich. Patient remained asymptomatic and was discharged on request with the instructions for medications and weekly serum potassium monitoring.  She presented in active labor at 35 weeks gestation and delivered vaginally a preterm male child with birth weight of 1.68 kg.  Baby was admitted in NICU and discharged on day 6, along with the mother. On discharge patient was reviewed by various departments and advised to continue syrup potassium chloride and calcium supplements.

Discussion

Sjögren syndrome is basically a genetic defect which is precipitated by environmental triggers like viral and bacterial infections. It is more common in females. Estrogen in females might affect immune system in body increasing susceptibility to this syndrome.[3]  The diagnosis of Sjögren syndrome is based on symptomatology.  Antibodies like ANA and Rh factors which are indicative of autoimmune disorders are present in this syndrome. ANA is the most commonly detected antibody and anti SSB LA is the most specific. A lip or salivary gland biopsy that reveals lymphocytic infiltration around salivary gland is diagnostic of the condition. In our patient ANA and anti Ro and La antibodies were positive. Lip biopsy was proposed but patient refused the same. Sjögren syndrome does not impair fertility but patients experience more complications during pregnancy compared to normal controls. The course and outcome of pregnancy has not been extensively studied as the syndrome occurs mostly in perimenopausal and post-menopausal women. This syndrome may sometimes be complicated with pulmonary hypertension specially during pregnancy and post-partum period. Effect of auto immune disease on pregnancy depends on various factors like disease activity, severity of organ damage, antibody profile and drug treatment.[4]  There is an increased risk of spontaneous abortions and fetal loss.[5]  The rate of preterm deliveries and IUGR is also higher in these patients.[6]  IUGR and fetal distress increases the rate of cesarean delivery in affected cases.[7] These adverse fetal effects are due to presence of immunological factors like SSA, SSAB and APLA antibodies. Neonatal lupus and congenital heart block (CHB) are the most common fetal manifestations. CHB results from damage of atrioventricular node caused by anti SSA and SSB antibodies. Incidence of CHB in anti SSA affected pregnancy is 1- 2 %. Cases of Sjögren syndrome affected pregnancies are managed by multidisciplinary approach.[8]  Prenatal counseling of affected women must be done explaining the maternal and fetal manifestations of the disease. Ideally disease should be well in control 3 to 6 months prior to conception. During antenatal period, serial 2 D echocardiogram should be done starting from 16- 20 weeks to detect CHB as early as possible. Monitoring of fetal wellbeing by serial USG is advised for early detection of IUGR and other fetal manifestations. 

Fluorinated corticosteroids like dexamethasone and betamethasone are the most commonly used drugs. These decrease the load of maternal antibodies which in turn reduces placental transfer of these antibodies. These steroids can also cross the placenta to reach the fetus and reduce inflammatory damage and fibrosis of atrioventricular node thus preventing CHB.[9] Renal involvement in the mother is seen in 4.2 to 50 % cases. Distal RTA is the most common renal manifestation seen in 25 % cases of Sjögren syndrome.[10]  Hypokalemia is the most common electrolyte disorder caused by increased potassium secretion by distal tubular cells in setting of diminished H+ ion secretion.  Hypokalemia in distal RTA is usually not very severe but in a few cases can cause hypokalemic periodic paralysis and hypokalemic cardiac arrhythmias. Hypokalemic paralysis, though very rarely, can present as primary manifestation of Sjögren syndrome as seen in our case. There is hypercalciuria and hyperphosphatemia in distal DTA due to reduction in calcium absorption secondary to chronic acidosis and due to increased calcium phosphate release from bones due to bone buffering of excess acid. All these can result in recurrent renal stones, bone pain and osteomalacia. RTA and hypokalemia are treated with potassium citrate and sodium bicarbonate. Our case is one of the very rare cases of pregnancy with Sjögren syndrome complicated with distal RTA and hypokalemia. Our patient was treated with potassium syrup and recovered completely without any recurrence of symptoms.

References

1. Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X et al. Sjögren syndrome. Nat Rev Dis Primers. 2016; 2:16047.
2. Tincani A, Andreoli L, Cavazzana I, Doria A, Favero M, Fenini MG et al. Novel aspects of Sjögren’s syndrome in 2012. BMC Med. 2013; 11:93.
3. Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjögren's syndrome. Nat Rev Rheumatol. 2010; 6(9): 529–37.
4. Carvalheiras G, Faria R, Braga J, Vasconcelos C. Fetal outcome in autoimmune diseases. Autoimmun Rev. 2012; 11(6–7): A520–30.
5. Sandhya P, Jeyaseelan L, Scofield RH, Danda D. Clinical characteristics and outcome of primary Sjogren’s syndrome: a large Asian Indian cohort. Open Rheumatol J. 2015; 9: 36–45.
6. Priori R, Gattamelata A, Modesti M, Colafrancesco S, Frisenda S, Minniti A et al. Outcome of pregnancy in Italian patients with primary Sjögren syndrome. J Rheumatol. 2013; 40(7): 1143–7.
7. Hussein SZ, Jacobsson LT, Lindquist PG, Theander E. Pregnancy and fetal outcome in women with primary Sjogren’s syndrome compared with women in the general population: a nested case-control study. Rheumatology (Oxford) 2011; 50(9): 1612–7.
8. Brucato A, Cimaz R, Caporali R, Ramoni V, Buyon J. Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clin Rev Allergy Immunol. 2011; 40(1): 27–41.
9. Yang CH, Chen JY, Lee SC, Luo SF. Successful preventive treatment of congenital heart block during pregnancy in a woman with systemic lupus erythematosus and anti-Sjögren’s syndrome A/Ro antibody. J Microbiol Immunol Infect. 2005; 38(5): 365–9.
10. Arman F, Shakeri H, Nobakht N, Rastogi A, Kamgar M. A case of kidney involvement in primary Sjögren's syndrome. Am J Case Rep. 2017; 18:622-626.

Citation

Sharma K, Chauhan AR. Pregnancy Complicated With Sjögren Syndrome. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/pregnancy-complicated-with-sjogren.html

Twisted Paraovarian Cyst

Author Information

Saini N*, Chauhan AR**, Paghdiwalla K**.

(* Third Year Resident, ** Honorary Consultant, Saifee Hospital, Mumbai, India).

Abstract

Torsion of adnexa may present as acute abdominal pain in the reproductive age group. Torsion of ovarian masses are common, but isolated torsion of paraovarian cyst is rare. If timely diagnosis and intervention are not done, it can lead to irreversible damage to the ipsilateral tube and ovary. We report a case of a twisted paraovarian cyst in a 33-year-old female causing acute abdominal pain, which was managed laparoscopically.

Introduction

Parovarian cysts represent approximately 5% of adnexal cysts.[1] Most of them are the vestigial remnants of Wolffian duct in the mesosalpinx or arise from mesothelium of the broad ligament or from tubal epithelium. These cysts are unilocular, the wall is thin and they contain clear fluid. They are mostly small and asymptomatic but occasionally they become large enough to cause acute pain in abdomen.[2] Physicians need to maintain a high index of suspicion for this rare and difficult to diagnose cause of pain abdomen.[3] They are difficult to be differentiated from ovarian cysts on imaging.[4] They should be surgically removed and if possible, fallopian tube and ovary should be preserved. Complications include torsion, rupture, hemorrhage, secondary infection and neoplastic transformation. We present a case of paraovarian cyst torsion with two twists which presented as a mass in the pouch of Douglas (POD).

Case Report

A 33-year-old female para 1 living 1, previous LSCS, married for 5 years presented with complaints of sudden onset pain in lower abdomen and vomiting 1-2 episodes since 2 days. The pain was sharp, non-radiating and was relieved on giving analgesics. There was no history of fever, abnormal vaginal bleeding, altered bowel or bladder habits or any other illness. Her last menstrual period was 20 days ago, and was scanty. On examination, patient was well oriented, afebrile, pulse and blood pressure were normal, no pallor or any signs of dehydration were seen; rest of the systemic examination was normal. Per abdomen, there was tenderness in the hypogastrium, no guarding or rigidity, and no palpable lump was felt. Speculum examination was normal. On per vaginal examination, a mass of 4 -5 cm diameter was felt in posterior fornix, which was smooth, firm and tender. Uterus was anteverted and normal size. Our initial impression was ovarian cyst torsion or ectopic pregnancy.

Her blood counts and urine investigations were within normal limits, and β HCG was within the non-pregnant range. Her first USG revealed a hypoechoic lesion of about 4 cm diameter in the pouch of Douglas, with low level internal echoes suggestive of endometrioma. Ca-125 was 11 U/ml (normal). As the clinical picture and her severe pain was discordant with USG findings, a repeat USG with Doppler and CT scan with contrast was done to rule out any bowel etiology. The second USG revealed a thick walled cystic mass 4.6 x 3 cm in cul-de-sac suggestive of either a paraovarian or mesenteric cyst. Doppler showed normal blood flow to both ovaries. CT scan showed a cystic lesion in the posterior cul-de-sac 5.1 x 4 cm, suggestive of paraovarian cyst; both ovaries were seen separately from the lesion.

Figure 1. Contrast enhanced CT scan showing heterogenous cystic lesion in the pouch of Douglas.

An operative laparoscopy was performed. An enlarged hemorrhagic left paraovarian cyst 4 x 5 cm was found in the POD which had undergone 2 twists; left fallopian tube appeared edematous and left ovary was normal and seen separate from the cyst. Right tube and ovary, and uterus were normal. The cyst was untwisted, the pedicle was cauterized and cut.

Figure 2. Intraoperative view of mass in POD.

Figure 3. Intraoperative view of torsion of paraovarian cyst.

Posterior colpotomy was done and cyst was retrieved vaginally and sent for histopathology. Surface of the left fallopian tube appeared normal; apart from mild congestion, there were no signs of rupture or bleeding. Tube was free, not adherent to the surrounding structures and tubo-ovarian relationship was maintained, hence it was preserved. Post operatively, patient had an uneventful recovery. Histopathological examination confirmed a benign hemorrhagic cyst with no evidence of endometriosis.

Discussion

The distinction between paratubal and paraovarian is sometimes arbitrary, with interchangeable usage of terms, as these cysts are present in the broad ligament between the tube and the ovary. A paraovarian cyst arises from mesothelium of broad ligament, also from the para mesonephric tissues and rarely mesonephric remnants.[5] Mostly, they are an incidental finding during surgery and should be prophylactically excised because of their propensity to torsion and rapid enlargement.[6] Torsion of the paraovarian cyst is three times more common in pregnancy.[3] These cysts are mostly unilateral, but bilateralism has also been reported. The same sided fallopian tube and ovary being close to the paraovarian cyst, may also undergo torsion with it. Differentiation between ovarian and paraovarian cysts is difficult, not only clinically, but also on imaging. USG with color Doppler is an important tool to diagnose the viability of adnexa, by showing the blood flow in the twisted pedicle and the central venous flow. An early intervention is recommended to save the ipsilateral tube and ovary.

Previously there were concerns that untwisting of adnexa might cause increased chances of thromoembolism. But at present, we have evidence that unwinding the adnexa to watch for tissue reperfusion is safe.[7] McGovern et al reported a 0.2 % incidence of pulmonary embolism in cases of adnexal torsion, these were linked with adnexal excision and not to detorsion of adnexa.[4] It is possible to preserve the adnexa irrespective of its appearance after de-torsion. Subsequent successful pregnancies have been reported in 95 % of cases, with no post-operative morbidity.[8]

Acknowledgment

We acknowledge Dr Zuber Kazi, consultant radiologist for input on CT scan.```

References

1. Atal R. Torsion of paraovarian cyst resulting in secondary torsion of ovary and fallopian tube. International Journal of Obstetrics and Gynaecology Research (IJOGR). 2016; 3(8): 432-7.
2. Steinback F, Kauppila A. Development and classification of paraovarian cysts: an ultrastructural study. Gynecol Obstet Invest. 1981; 12 (1):1-10.
3. Puri M, Jain K, Negi R. Torsion of para ovarian cyst: a cause of acute abdomen. Indian J Med Sci. 2003; 57(8):361-2.
4. Gopal K, Lim Y, Dobson M, Keating P, Stringfellow H. A case of torted parafimbrial cyst on MRI: case report and review of literature. Br J Radiol. 2006;79(948): e208-10. 
5. Kiseli M, Caglar GS, Cengiz SD, Karadag D, Yılmaz MB. Clinical diagnosis and complications of paratubal cysts: review of the literature and report of uncommon presentations. Arch Gynecol Obstet. 2012 Jun; 285(6):1563-9.
6. Genardy R, Parmley T, Woodruff JD. The origin and clinical behavior of the paraovarian tumour. Am J Obstet Gynecol. 1977; 129(8): 873-80.
7. Ghossain MA, Braidy CG, Kanso HN, Farah K, Klein-Tomb L, Trak-Smayra V et al. Extraovarian cystadenomas: ultrasound and MR findings in 7 cases. J Comput Assist Tomogr. 2005; 29(1):74-9.
8. Barloon TJ, Brown BP, Abu-Yusuf MM, Warnock NG. Paraovarian and paratubal cysts: preoperative diagnosis using transabdominal and transvaginal sonography. J Clin Ultrasound. 1996; 24(3):117-22.

Citation

Saini N, Chauhan AR, Paghdiwalla K. Twisted Paraovarian Cyst. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/twisted-paraovarian-cyst.html

Large Hydrosalpinx Mimicking An Ovarian Cyst

Author Information

Rane V*, Samant PY**, Honavar P***.

(* Second Year Resident, ** Additional Professor, *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

A 45 years old, perimenopausal, multiparous, diabetic woman presented with chronic pelvic pain. Her imaging studies were suggestive of left ovarian cyst but on exploration we found a large left hydrosalpinx with normal ovaries. Left hydrosalpinx excision with right salpingectomy with bilateral oophorectomy was performed. Here, we discuss this unusual case of an atypical hydrosalpinx mimicking an ovarian cyst.

Introduction

An ovarian cyst is a common finding on pelvic examination and ultrasonography (USG), of which 70 % are benign cysts, 24 % are functional and 6 % are malignant.[1] We frequently rely on USG features to confirm the diagnosis with regards to the risk of pelvic malignancy on the basis of characteristics like size, laterality, complex appearance with solid component (with Doppler flow present in solid areas), mural nodules  and ascites. Rarely, ultrasonically diagnosed complex ovarian cyst may turn out to be large hydrosalpinx as in our case. Misdiagnosis may be due to the tube’s large dimensions, very thin walls, septations and spherical appearance. Loculated ascites may also look like an ovarian cystic mass on USG. Hydrosalpinx due to distal tube occlusion may be secondary to pelvic inflammatory disease, endometriosis, fimbrial serosal obstruction following an adjacent inflammation like appendicitis and previous surgery (either tubal, pelvic or abdominal) or tubal ligation near fimbrial end.

Case Report

A 45 years old multiparous woman presented with gradual onset of dull aching pain in left iliac fossa for the last six months. She had history of severe pain with vomiting one month before presentation which was treated conservatively. Abdominopelvic ultrasound in private clinic showed a cystic adnexal mass for which she was sent to our institute. She had no complaints of burning micturition or vaginal discharge, fever, anorexia or weight loss suggestive of any genitourinary or systemic infection.  She had no other urinary or bowel complaints due to pressure of pelvic mass.  She had no complaints of pedal edema, chest pain, paroxysmal nocturnal dyspnea, palpitations, syncope or seizures. She had previous three cesarean sections, with tubal ligation done at the time of last section 18 years ago. She had irregular menses with amenorrhea for 3 months before presentation to our outpatient department. Urinary pregnancy test was negative and she had been given progesterone for 5 days for withdrawal bleeding. On physical examination, she was afebrile. Pulse was normal and blood pressure was raised (140/90 mm Hg). Her BMI was 27 kg/m2. Chest auscultation revealed normal heart sounds with ejection systolic murmur, respiratory system was clear. 

On per abdominal examination, patient had both midline vertical scar and Pfannensteil scar with a 24 weeks size mass felt in lower abdomen more on right side arising from pelvis. Patient had mild tenderness in left iliac fossa and suprapubic region.  Per speculum examination showed healthy cervix and vagina. On per vaginal examination, uterus was retroverted, soft, firm, mobile, normal in size. Mass was not well appreciated due to obesity. Both fornices were free and non-tender. 

Her investigations revealed raised blood sugars, raised HbA1c (7.17). She was started on metformin and sitagliptin by endocrinologist. Her cholesterol levels were normal. Her hemogram, liver and renal function tests were normal. She was seronegative for syphilis, hepatitis B, C and HIV. Tumor markers AFP, CEA, Beta HCG, CA 125 were normal, LDH was elevated (624 IU/liter). Repeat LDH was 503 IU/liter. 2D echocardiogram showed atrial septal defect with large ostium secundum and left to right shunt. Pap smear showed inflammatory cells.

USG was suggestive of a thin walled, avascular, multiloculated, anechoic lesion measuring 11.2 x 9.9 x 8 cm in left ovary most likely complex ovarian cyst. Right ovary was normal, and no adnexal mass was seen. Computerized tomography scan (CT scan) showed thin walled multiloculated cystic lesion in pelvis measuring 9.6 x 8.4 x 10.2 cm representing ovarian cyst probably from left ovary. There was no ascites or lymph node enlargement. Chest radiograph showed patchy opacities in left mid zone suggestive of infective etiology; bilateral costophrenic angles were clear. 

Patient was counseled for total abdominal hysterectomy with bilateral salpingo-oophorectomy SOS omentectomy and peritoneal fluid cytology. Exploratory laparotomy was done after taking cardiology, endocrinology and chest medicine references for fitness for surgery.

Intraoperatively, peritoneal fluid was sent for cytology. Uterus was normal in size. Urinary bladder was found to be densely adherent to the anterior uterine wall.  Left hydrosalpinx of about 7 x 8 cm adhered to the omentum was noted (figure 1). 

Figure 1. Left hydrosalpinx.

Left ovary was normal with 3 x 2.5 cm simple cyst. Right ovary and fallopian tube looked normal. Hysterectomy could not be done due to dense adhesions between bladder and anterior uterine wall. Left hydrosalpinx with left ovary was excised with right salpingo-oophorectomy after informing the patient and relatives about anticipated difficulty in hysterectomy and risk of bladder injury. Postoperatively, patient was given deep venous thrombosis prophylaxis with strict monitoring of blood sugars.

Cytology of peritoneal fluid revealed degenerative changes with many erythrocytes and a few monolayered sheets of degenerated mesothelial cells. No malignant cells were seen. Histopathology report was suggestive of normal ovarian stroma, corpus albicans and follicular cyst identified. Fallopian tube section showed features of hydrosalpinx.

Discussion

Chronic pelvic pain is a common problem seen in gynecologic outpatient department. Gynecological causes of chronic pelvic pain can be endometriosis, inflammatory or post-operative adhesions, ovarian cysts, tubo-ovarian mass, adenomyosis, myoma or pelvic congestion. Of these, ovarian cysts and large hydrosalpinx may be difficult to differentiate both clinically and on imaging studies. Hydrosalpinx after pelvic inflammatory disease is often a sequela of chlamydial infection which affects fallopian tube epithelial transporters and ion channels particularly cystic fibrosis transmembrane conductance regulator. This results in increased epithelial secretion, decreased fluid absorption, hence accumulation of fluid.[2] Ultrasound is useful for adnexal pathology but requires high index of suspicion to rule out various similar appearing causes of pelvic pain. 

Simple cysts appear unilocular with lack of cyst wall papillae, but hemorrhagic cysts have echogenic content which may be homogenous or heterogenous. A resolving blood clot may show fibrin strands (cobweb/ reticular/ lace like pattern) mimicking septations. Dermoid cysts are easy to identify due to hyperechoic bone, teeth, hair in hypoechoic cyst. Para ovarian cyst can be correctly diagnosed when ovary is seen separately from the cyst. Endometrioma shows uni- or multiloculated homogenous echoes and ground glass appearance.[3] Malignancy may be detected by Doppler study. A typical hydrosalpinx appears as a tubular retort shaped cystic mass with incomplete septation or indentations along its walls (cogwheel/ waist sign), mural nodules may give it a “beads on string” appearance.[4] In our case, it appeared large with spherical shape and hence was mistaken as ovarian cyst. Hydrosalpinx with menstrual cycle dependent changes due to fallopian tube endometriosis has been reported.[5] Hydrosalpinx can also mimic ovarian malignancy as both present as adnexal mass. Transabdominal sonography may be non-discriminative. Elevated tumor marker levels like CA125, CEA or LDH should clinch the diagnosis.[6] In some cases of hydrosalpinx, the tumor markers also may be raised.[7] CA 125 may be high in tuberculosis. In our case, LDH was high.  We planned total abdominal hysterectomy with bilateral salpingo-oophorectomy for ovarian neoplasia. On laparotomy, it was large left hydrosalpinx which was excised along with right fallopian tube and both ovaries, uterus being densely adherent to bladder could not be removed. Risk reducing salpingo-oophorectomy without hysterectomy is a known modality of treatment in cases of BRCA carriers.[8]

Conclusion

It is important to remember that hydrosalpinges can have an atypical appearance due to significantly large size along with rise in tumor markers mimicking a malignant ovarian cyst.

References

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5. Osuga Y, Koga K, Hirata T, Hiroi H, Takesani Y. A case of hydrosalpinx associated with the menstrual cycle. Fertil Steril. 2008; 90(1):199.
6. Manidakis LG, Angelakis E, et al. Genital tuberculosis can present as disseminated ovarian carcinoma with ascites and raised CA125: A case report. Gynecol Obstet Invest. 2001; 51(4):277-9. 
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Citation

Rane V, Samant PY, Honavar P. Large Hydrosalpinx Mimicking An Ovarian Cyst. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/large-hydrosalpinx-mimicking-ovarian.html