Volume 6 Number 4

Editorial
Parulekar SV

A Rare Case Of A Large Asymptomatic Cervical Leiomyoma
Mathews N, Parulekar SV.

An Unusual Case Of A Large Leiomyoma Complicating Pregnancy
Joshi S, Parulekar SV.

Absence Of Ipsilateral Adnexa And Its Implications
Rane P, Samant P.

Torsion Of Hematosalpinx In A Case Of Unicornuate Uterus
Jha N, Chaudhari HK, More V.

Subserosal Fibroid Misdiagnosed As Ovarian Mass In Pregnancy: Even MRI Is Not 100% Diagnostic
Verma ML, Sachan R, Singh A, Kumari M, Singh U, Sankhwar PL.

Vaginal Pull Through Procedure To Treat Isolated Lower One Third Vaginal Agenesis
Saxena A, Pardeshi S, Gupta AS.

Alloimmunisation With Rare Blood Group In Twin Pregnancy
Singhania N, Vaidya A, Gupta AS.

Successful Management Of Essential Thrombocytosis In Pregnancy
Tagad M, Tiwari N, Chaudhari HK.

Remembering Past Greats: Grigoris Lambrakis
Prasad M, Venkatesh S.

Editorial

Parulekar SV

Obesity during pregnancy is on the rise, and so are the conditions that go with obesity. Obstructive sleep apnea (OSA)  is one such condition. It is characterized by repetitive episodes of obstruction of the upper airway during sleep, leading to absence or severe reduction in airflow despite respiratory effort. The gravida experiences hypoxemia and recurrent arousal from sleep. Its prevalence is directly proportional to maternal age and weight independent of each other. Physiological changes of pregnancy like reduced diameter of the oropharynx, nasal obstruction due to mucosal edema and increase in Mallampati grade lead to increased resistance of the upper airway and greater negative intra-pharyngeal pressure, which in turn lead to snoring and obstructed breathing in sleep. The increase in the circulating blood volume causes adverse effect on the upper airway function in recumbency (sleep) and risk of OSA. The true incidence of OSA in pregnancy is not known, but is probably much more than what is believed, because women tend to report it less often. Questionnaires are not found to be very useful to detect which gravidas suffer from it. An objective test is more likely to detect OSA. A full-night, attended, in-laboratory polysomnogram is the best method of diagnosing OSA. An unattended, home sleep apnea testing is more convenient, but is not cleared for use in pregnancy by American Academy of Sleep Medicine. When apnea hypopnea index (average number of apneas plus hypopneas per hour slept) is 5 to 15, 15-30, and more than 30 events per hour, OSA is said to be mild, moderate and severe respectively. It must be kept in mind that OSA is not the only cause of disturbance of sleep in pregnancy, and there are causes related to pregnancy, e.g. leg cramps, fetal movements, urge for urination and dyspepsia. If OSA is not treated, risk of complications like fatigue, excessive daytime sleepiness, lack of attention, systemic hypertension, cerebrovascular disease, cardiac arrhythmias, ischemic heart disease, cardiomyopathy, pulmonary embolism,  insulin resistance, gestational diabetes, preeclampsia, gestational hypertension, fetal growth restricition, effect on neurological growth, preterm labor and fetal death. Pregnancy may exacerbate OSA. Thus treating OSA during pregnancy is important. All gravidas with moderate or severe disease are treated. The treatment includes use of oral mandibular repositioning appliances, continuous positive airway pressure and behavior modification. Modafinil, which is used to treat OSA, is a category C drug. It is also found in the breast milk. Hence its use is not recommended in pregnancy and lactation.  Upper airway surgery like uvulopalatopharyngoplasty is not recommended in pregnancy. Complications listed above need to be watched for and managed aggressively. Continuous pulse oximetry is used in labor and after childbirth. Any hypoxemia is evaluated and treated energetically, maintaining oxygen saturation at least 96%. Regional anesthesia for labor pain management is preferred to opioids. As the awareness of this condition during pregnancy increases, more and more cases will come to light. We hope to create this awareness in our readers through this editorial.

A Rare Case Of A Large Asymptomatic Cervical Leiomyoma

Author Information

Mathews N*, Parulekar SV**.
(* Second Year Resident **Professor and Head, Obstetrics and Gynaecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Leiomyomas are most common benign tumors of the uterus. Most of them are situated in the body of the uterus and rarely, in about 1-2% of cases, they are located in the cervix and cause bowel and bladder pressure related symptoms owing to their proximity to these structures. We report an unusual case of a 33 year old female with a large cervical leiomyoma with no associated bowel and bladder symptoms where myomectomy followed by total abdominal hysterectomy was done.

Introduction

Cervical leiomyomas are rare tumors accounting for only 1-2% of all uterine leiomyomas.[1] They may arise from supravaginal or vaginal part of the cervix and may be anterior, posterior, lateral or central depending upon their location.[2] Owing to their location, cervical leiomyomas often lead to pressure related bowel and bladder symptoms.

Case Report

A 33 year old woman, married for 13 years, who had undergone two lower segment cesarean sections in the past and had one living 11 year old male child, presented to the outpatient department with dull aching lower abdominal pain for 2 months. There was no history of altered bowel and bladder habits, weight loss, decreased appetite, menstrual irregularities, menorrhagia or dysmenorrhea. There was no relevant medical or surgical history.  On examination, her general condition was fair and vital parameters were within normal limits. General and systemic examination revealed no abnormality. On per abdominal examination, one Pfannensteil and one vertical midline scar were seen. There was no guarding, tenderness, rigidity or any mass. On per speculum examination, the cervix and vagina were healthy. On bimanual pelvic examination, a 7-8 cm diameter mass was felt continuous with the uterus. Ultrasonography was suggestive of a large subserosal leiomyoma of size 10.5X5X7.5 cm arising from the posterior wall of the uterus extending into the left adnexa with multiple areas of cystic degeneration within. On account of the above findings and the patient’s desire for not preserving fertility, a plan for total abdominal hysterectomy with bilateral salpingectomy was made. All investigations for fitness for anesthesia were within normal limits. Her PAP smear report was normal. An exploratory laparotomy was performed under spinal anesthesia. Intraoperatively a large cervical leiomyoma arising from the left side of the posterior part of the cervix was seen, extending into the pouch of Douglas and anteriorly up to the right lateral uterine wall with dense adhesions anteriorly with the bladder (figure 1). Both ovaries were normal. Enucleation of the leiomyoma was done by anterior left broad ligament approach. This approach was preferred because the leiomyoma was extraperitoneal, though a part of its surface was covered by peritoneum. A myomectomy was done prior to hysterectomy, because it was considered safer to restore normal anatomy first, so as to be able to clamp the uterine vessels properly and avoid injury to the ureters. Dense adhesions with the bladder were released by sharp dissection. Total abdominal hysterectomy with bilateral salpingectomy was done. Ureters were visualized again after the hysterectomy and any injury was ruled out. The specimen was sent for histopathological examination. Her recovery was uneventful. Histopathological examination confirmed the diagnosis of a cervical leiomyoma.

Figure 1. Intraoperative findings. The leiomyoma is being enucleated after cutting its pseudocapsule.

Figure 2. Surgical specimen. U: uterus, C: cervix, L: leiomyoma.

Discussion

Cervical leiomyomas are rare tumors. Large cervical leiomyomas seldom remain asymptomatic and are often associated with bowel and bladder symptoms and menstrual irregularities and dyspareunia. Proximity of a large cervical leiomyoma to ureters and urinary bladder poses an increased risk of injury to these structures. Difficulties during surgical removal of a cervical leiomyoma include anatomical displacement of the uterine vessels and ureters, and cranial displacement of the urinary bladder.[3]

In our case, a large cervical leiomyoma was seen arising from the left supravaginal cervix, extending into the Pouch of Douglas and forwards towards the right anterior surface of the uterus, being adherent densly to the urinary bladder. Such trans-midline and circumuterine growth of a leiomyoma is quite unusual, and may be missed on both clinical examination as well on imaging like ultrasonography, computed tomography and magnetic resonance imaging. Despite its large size and extent, the patient had no complaints with respect to bowel and bladder function and menstrual bleeding or pain which was unusual. Enucleation of the large leiomyoma was done through an approach through the left anterior leaf of the broad ligament, because that was the safest and easiest approach to the extraperitoneal space where the leiomyoma was present. Myomectomy helped visualize the uterine vessels and ureters, so that the vessels could be clamped properly and injury to the ureters was avoided.  Intracapsular enucleation of cervical leiomyomas is the best approach to avoid injury to ureters and urinary bladder.

Acknowledgment

We thank Dr Sana Bijapur for taking operative photographs.

References

1. Buttram VC Jr, Reiter RC. Uterine Leiomyomata: etiology, symptomatology and management. Fertil Steril 1981;36:433.
2. Kumar P, Malhotra N. Tumors of the corpus uteri. In: Jeffcoat’s Principles of Gynaecology 7th edition; p 487-516.
3. Monaghan JM, Lopes AB, Naik R. Total hysterectomy for cervical and broad ligament fibroids. In: Huxley R, Taylor S, Chandler K. Bonney’s gynaecological surgery 10th edition; pp. 74-86.

Citation

Mathews N, Parulekar SV. A Rare Case Of A Large Asymptomatic Cervical Leiomyoma. JPGO 2019. Vol 6 No. 4. Available from: https://www.jpgo.org/2019/04/a-rare-case-of-large-asymptomatic.html

An Unusual Case Of A Large Leiomyoma Complicating Pregnancy

Author Information

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

Abstract

Uterine leiomyomas are common benign smooth muscle tumors. Their incidence during pregnancy approximates 2 percent. The range varies with frequency of routine sonography and the population characteristics. Presence of a leiomyoma may complicate a pregnancy in various manners. We discuss one such case of a pregnancy complicated by a large uterine leiomyoma.

Introduction

Uterine leiomyomas are usually asymptomatic during pregnancy but may occasionally be complicated by cystic degeneration, red degeneration or torsion. Leiomyomas increase the risk of spontaneous abortions, preterm labour, premature rupture of membranes, intrauterine growth restriction, antepartum haemorrhage, malpresentations, obstructed labour, need for cesarean section, retained placenta, and postpartum hemorrhage.[1-4] Factors most important in determining the morbidity during pregnancy are the size, number and location of the leiomyomas.

Case Report

A 37 year old woman, ninth gravida 9, third para, with 2 living issues, 1 neonatal death, 1 ectopic gestation and 4 spontaneous abortions in the past, presented with 37 weeks of amenorrhea. She had undergone exploratory laparotomy with right salpingectomy in view of a ruptured ectopic gestation. She had 3 full term normal vaginal births, and 4 spontaneous abortions following which check curettage was done in all 4 cases in view of incomplete abortion. She had two ultrasound reports during antenatal period showing posterior intramural leiomyoma with increase in size during pregnancy, from 1.8x1.8 cm on 20/7/18 to 4.8x 4.6 cm on 5/9/18. She was hospitalized because she was hypertensive. An obstetric ultrasonography was done on 19/12/18, which showed a single live intrauterine gestation in breech presentation with gestational age of 33 week 2 days with intrauterine growth restriction, severe oligohydramnios (amniotic fluid index 0-1) and fetoplacental insufficiency with brain sparing effect. The pregnancy was terminated by a lower segment cesarean section in view of breech presentation with above mentioned ultrasonographic changes. After delivering the baby, the posterior wall intramural leiomyoma of approximately 8x8 cm in size was observed. There was no active bleeding. The leiomyoma was kept undisturbed and uterine closure was done and hemostasis was achieved. Postoperatively patient had 5 episodes of atonic postpartum hemorrhage over 2 hours, each episode with clots of 70-80 ml approximately. Bleeding was not controlled with intravenous Oxytocin infusion, rectal Misoprostol and intramuscular 15S, 15 CH3 PGF2alpha. On vaginal examination the cervix was found to be open and the leiomyoma was felt through the open cervix. A postpartum hysterectomy was done in view of resistant atonic postpartum hemorrhage. A posterior intramural leiomyoma of size 8x8 cm was found (figure 1). The patient made an uneventful recovery.

Figure 1. Intraoperative findings: the leiomyoma is seen to be bulging in the uterine incision for cesarean section.

Figure 2. Surgical specimen: the leiomyoma is seen to be bulging in the uterine cavity after bisection of its anterior wall.

Discussion

Leiomyomas are present in 2% of pregnant women. They are usually asymptomatic in pregnancy. The obstetric adverse events include spontaneous abortions, preterm labor, fetal growth restrictions, malpresentations, placenta previa, placental abruption, dysfunctional labor and atonic postpartum hemorrhage.[5] The need for a cesarean section is also increased in view of some of the adverse events. Other than malpresentations, the adverse events are associated with intramural or submucous leiomyomas, more with the latter. Our patient had fetal growth restriction, breech presentation, she required a cesarean section and had atonic postpartum hemorrhage.

In our patient the leiomyoma was large (8x8 cm), was situated in the posterior wall, bulging into the uterine cavity. It was sessile and had a large base. It was not removed during cesarean section because it was not anywhere near the uterine incision and there was no indication for the myomectomy. Serious hemorrhage could have resulted if an attempt was made to remove the leiomyoma at that time, because it was largely intramural. That would have forced us to give massive transfusion of blood and blood products and perform uterine artery embolization and hysterectomy.[6-11] The plan was to perform a myomectomy after puerperium, if required.[12] Obstetric hysterectomy was not done immediately after the cesarean section, because bleeding from the placental bed was well controlled.

Clinical judgement and foresight are required for anticipating possible future complications and need for repeat surgery before making a decision about cesarean hysterectomy for uterine leiomyomas.

References

1. Terry KL, De Vivo I, Hankinson SE, Missmer SA. Reproductive characteristics and risk of uterine leiomyomata. Fertil Steril 2010; 94:2703.
2. Katz VL, Dotters DJ, Droegemeuller W. Complications of uterine leiomyomas in pregnancy. Obstet Gynecol 1989; 73:593.
3. Klatsky PC, Tran ND, Caughey AB, Fujimoto VY. Fibroids and reproductive outcomes: a systematic literature review from conception to delivery. Am J Obstet Gynecol 2008; 198:357.
4. Hasan F, Arumugam K, Sivanesaratnam V. Uterine leiomyomata in pregnancy. International journal of gynaecology and obstetrics. 1991;34(1):45–48.
5. Qidwai GI, Caughey AB, Jacoby AF. Obstetric outcomes in women with sonographically identified uterine leiomyomata. Obstet Gynecol 2006; 107:376.
6. Sparić R, Kadija S, Stefanović A, Spremović RS, Likić LI, Popović J.et al. Cesarean myomectomy in modern obstetrics: More light and fewer shadows. J Obstet Gynaecol Res 2017;43:798–804.
7. Kwon DH, Song JE, Yoon KR, Lee KY. The safety of cesarean myomectomy in women with large myomas. Obstet Gynecol Sci 2014; 57: 367–372.
8. Mehmet BS, Mesut P, Ozan D, Çiğdem P, Oğuz DY, Resul K et al. Outcome of caesarean myomectomy: is it a safe procedure? Geburtshilfe Frauenheilkd. 2017 Nov; 77(11): 1200–1206.
9. Park RC, Duff WP: Role of cesarean hysterectomy in modern obstetric practice. Clin Obstet Gynecol 23:(2):601, 1980.
10. Wittich AC, Salminen ER, Yancey MK, Markenson GR. Myomectomy during early pregnancy. Mil Med 2000; 165:162.
11. Celik C, Acar A, Ciçek N, et al. Can myomectomy be performed during pregnancy? Gynecol Obstet Invest 2002; 53:79.
12. Hee Joong Lee, Errol R Norwitz, and Julia Shaw, Contemporary Management of Fibroids in Pregnancy. Rev Obstet Gynecol. 2010;3(1): 20–27.

Acknowledgment

We thank Dr Sarika Solanke for taking operative photographs.

Citation

Joshi S, Parulekar SV. An Unusual Case Of A Large Leiomyoma Complicating Pregnancy. JPGO 2019. Vol 6 No. 4. Available from: https://www.jpgo.org/2019/04/an-unusual-case-of-large-leiomyoma.html

Absence Of Ipsilateral Adnexa And Its Implications

Author Information

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

Abstract

Absent ovary and fallopian tube with normal uterus has been reported in the literature. Such a finding is most likely due to ischemic necrosis following torsion. Such a situation creates a dilemma when contralateral oophorectomy is contemplated. In young women, loss of both ovaries is catastrophic. We report a case of absent unilateral ovary and tube in a young woman detected at laparotomy.

Introduction

Unilateral absent fallopian tube and ovary is seen extremely rarely. Two possible mechanisms proposed are; adnexal torsion and defective development of both Müllerian and Mesonephric systems.[1] Besides fertility concerns, premature menopause is also important. If a tubal stump is present, ectopic pregnancy implantation on the stump may cause risk to life.[2]

Case Report

A 30 year old multipara was referred from a peripheral hospital for right iliac fossa pain radiating to the back for a period of 3 days. The pain had aggravated on the day of admission. There was no history of nausea, vomiting, fever, or bowel/ bladder complaints. She was planned for an exploratory laparotomy with oophorectomy as her ultrasonography had diagnosed a dermoid cyst of the right ovary. Her preoperative electrocardiogram was suggestive of a ischemic cardiac condition, so she was referred to our center as an emergency case for intensive care facilities. Her last menses were about 14 days before her presentation to us, and her cycles were regular. She was para 3, living 3 and had one abortion. She was using barrier contraception. She had no significant surgical or medical history.
On examination, her vital parameters were normal. There was no pallor. Abdomen was not guarded. There was mild suprapubic and right iliac fossa tenderness. Speculum examination was normal. On vaginal examination, uterus was normal in size, with right forniceal tenderness and fullness. A 3 cm cystic mass was palpable in the right fornix. It was not adherent to the uterus.
A transvaginal sonogram done in a private hospital showed a 2.5x1.8 cm hyperechoic mass in the right adnexa, closely abutting the right ovary, probably a dermoid ovarian cyst. Uterus and left ovary were reported as normal. Sonography in our center showed a bulky right ovary of 4.3x4.1x3 cm with multiple peripherally located follicles. The vascularity of the pedicle was normal probably with detorsion. The left ovary was obscured probably by gas shadow.
Her laboratory reports were all normal. Hemoglobin was 11 gm%. Total count was 7,600/ mm3.  Blood sugar, renal and liver function tests were within normal limits. INR was normal. CA 125 was 11.9 units/ ml. She was considered at high risk for perioperative cardiac event on cardiological assessment. Exploratory laparotomy was performed for persistent pain and tenderness. The couple was asked about sterilization but they declined consent. Intraoperatively, there was no evidence of torsion of the right adnexa. The right ovary was bulky with a follicular cyst of about 1.5 cm in diameter. The tube was normal. Contra lateral ovary was absent. There was only a long tubal stump on the contralateral side. There were no adhesions. Her appendix was suspected to be inflamed, but the  surgeon who was called ruled out the same any need for intervention. No ovarian or tubal structure was seen in the pelvic cavity or abdomen when her bowels were inspected in detail by the surgeon.

Figure 1. Intra operative findings of absent ipsilateral  adnexa. The arrows point to the left tubal stump and empty left ovarian space.

Figure 2. The tubal stump, the ovary on the left side is not visualized.

Abdomen was closed after explaining the findings to the family members. She was started on broad spectrum antibiotics with metronidazole for 5 days.  On retrospective enquiry, she denied any episode of acute abdominal pain in the past. She made good recovery and was discharged after 5 days. She was counseled about early consultation in case of pregnancy to rule out stump ectopic pregnancy.

Discussion

Fertility implications for the woman with unilateral absence of fallopian tube and ovary are a major concern. In his study of fertility potential in women with a single ovary, Lass found that fertility was lower in women due to low ovarian reserve to begin with; but otherwise their fertility potential was normal.[3] For avoidance of premature surgical menopause and fertility preservation, Barsky proposes preemptive oophoropexy in women detected to have absent contralateral ovary or elongated utero-ovarian ligament.[1] An ectopic pregnancy in a tubal stump after salpingectomy is reported; the site usually is isthmic or interstitial and these cases have a higher mortality rate.[2] Po Chun et al reported a series of stump ectopic pregnancies in women who had conceived through assisted reproductive technology after salpingectomy for tubal pathology.[4] Hence it is important to counsel these patients about early reporting in case of amenorrhea.

Conclusion

In case of unanticipated absent tube and ovary, an attempt should be made to search for the ovarian remnant in the pelvis and the retroperitoneum. Risk of stump ectopic pregnancy has to be borne in mind during counseling these patients.

References

1. Barsky M, Beaulieu AM, Sites CK. Congenital Ovarian-Fallopian Tube Agenesis Predisposes to Premature Surgical Menopause: A Report of Two Cases. J Androl Gynaecol. 2015;3(1):3.
2. Lakhotia S, Yussof SM, and Aggarwal I. Recurrent ectopic pregnancy at the ipsilateral tubal stump following total salpingectomy case report and review of literature. Clin Med Invest, 2016;1(2): 35-38.
3. Lass A. The fertility potential of women with a single ovary. Human Reptoduction Update 1999;5(5):546-550.
4. Ko PC, Liang CC, Lo TS, Huang HY. Six cases of tubal stump pregnancy: complication of assisted reproductive technology? Fertil Steril. 2011;95(7):2432.e1-4.

Citation

Rane P, Samant P. Absence Of Ipsilateral Adnexa And Its Implications. JPGO 2019. Volume 6 No.4. Available from: https://www.jpgo.org/2019/04/absence-of-ipsilateral-adnexa-and-its.html

Torsion Of Hematosalpinx In A Case Of Unicornuate Uterus

Author Information

Jha N*, Chaudhari HK**, More V***.
(* Third Year Resident, ** Associate Professor, *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical college and K E M Hospital, Mumbai, India.)

Abstract

Congenital anomalies of the uterus and vagina caused by alterations in development or fusion of the Mullerian ducts are associated with multiple obstetric problems.  Here we present a case of a 21year old unmarried female with unicornuate uterus with a non-communicating horn. She came in an emergency with severe abdominal pain and ultrasound was suggestive of large left ovarian cyst with ovarian torsion. Intraoperatively there was torsion of hematosalpinx. She was also a known case of bicytopenia with non-cirrhotic portal fibrosis.

Introduction

Hematosalpinx is a medical condition involving blood collection into cavity of fallopian tubes. Its various causes include ruptured tubal abortion, endometriosis, cryptomenorrhoea, tubal carcinoma. Torsion in hematosalpinx is extremely rare affecting 1 in 1.5 million women.[1] This condition is rarely diagnosed pre-operatively and by the time surgery is undertaken, the vascular supply of the affected adnexa is usually damaged. Making a differential diagnosis between an ovarian and a tubal torsion is very difficult and a definitive diagnosis is always made after a laparoscopic or exploratory surgery.

Case Report

A 21year old unmarried female came to the emergency room with complaints of severe pain in abdomen since 2 days. On abdominal examination, 14 weeks size cystic mass was felt arising from the pelvis which was mobile and tender. On per rectum examination, 10x10 cm cystic mass was felt anterior to the uterus with mild tenderness. Ultrasound was suggestive of right ovarian torsion of a 10x10 cm ovarian cyst and also presence of bicornuate uterus with hematometra with hematosalpinx was documented. There were no renal anomalies. She was a known case of bicytopenia with non-cirrhotic portal fibrosis. In view of ovarian torsion, she was posted for emergency exploratory laparotomy with cystectomy SOS salphingo-oophorectomy. Pre-operative investigations were sent. Liver function tests showed raised total serum bilirubin of 2.67 mg/dl and alanine transaminase was mildly raised to 73.40 IU/l. Hemoglobin was 8.2 gm%, platelet count was 42,000/ cm3 and total leucocyte count was 1700/ cm3. Her coagulation profile was also deranged with INR of 1.73 and plasma fibrinogen of 115 mg/dl. Hematology and gastrointestinal medicine opinion was taken. Four units of fresh frozen plasma, 4 units of platelets and one unit of packed cell volume were transfused as per hematology advise pre- operatively. Gastro intestinal medicine advised to proceed with the surgery as it was an emergency and asked the her to follow up post procedure. Exploratory laparotomy was done under general anesthesia. Intraoperatively, unicornuate uterus with right non-communicating horn with right hematometra was noted. Also, right hematosalpinx of 10x12 cm was seen with four turns of torsion. A hemorrhagic cyst of about 3 cm was seen in the right ovary. Left fallopian tube and ovary were normal. Right hematometra of around 5 cc volume was seen which was drained. Right salpingectomy followed by excision of right non-communicating horn of uterus was done. Base was sutured with polyglactin 910 no. 1 in multiple figure of eight fashion. Right ovarian cystectomy was done with ovarian reconstruction with polyglactin 910 2-0. Postoperatively she was stable and discharged on day 5 of surgery after an uneventful course. Histopathology report was suggestive of endometriotic cyst in right ovary with hematosalpinx with unicornuate uterine horn with small foci of adenomyosis.

Figure 1. Unicornuate uterus with right rudimentary horn.

Figure 2. Around 10 cm diameter right  hematosalpinx with right hemorrhagic cyst.

Discussion

Congenital uterine anomalies are estimated to occur in 0.1 to 0.5 % of women.[2] Women with Mullerian duct anomalies can have reproductive problems like infertility, first trimester abortions, obstructed Mullerian systems that present with hematocolpos, hematosalpinx and hematometra. This patient presented with right hematometra and ipsilateral hematosalpinx with torsion of the hematosalphinx.  The first line investigation is ultrasonographic evaluation. Newer three-dimensional ultrasound can offer an added advantage. MRI is considered the investigation of choice for imaging uterine anomalies.
EAC classification of congenital anomalies of uterus proposed by Parulekar is a comprehensive classification of congenital malformations of the female genital tract that is based on embryological aberration.[3] According to this classification our case can be classified as follows.

Fallopian tubes FaR1 10 cm, FaL1 4 cm
Uterus Ut R1 4cm/Fn/Obst, UtL1 4 cm/Fn/ No Obst
Cervix Cx1 3cm/Fn/No Obst
Vagina 1 8 cm/Fn/No Obst
Hymen Hy
Clitoris Cl
Labium major NA
Labium minor NA
Introitus In

According to ESHRE ESGE classification system, our case comes under U4 class hemi uterus with rudimentary non-communicating horn.
American Fertility Society classification of Uterovaginal Anomalies classifies Mullerian anomalies into four classes.[4] According to this classification, this case is an example of obstructed asymmetric lateral fusion defect leading to unicornuate uterus with non-communicating right uterine horn.
Most rudimentary horns are non-communicating. Urinary tract anomalies are often associated with a unicornuate uterus like horseshoe or pelvic kidney. But this case did not have any renal anomaly.
Because most cases of unicornuate uterus have a non-communicating rudimentary horn on the opposite side, there is danger of pregnancy in the rudimentary horn from transperitoneal migration of sperm or ovum from the opposite side. Also, it is important to make the diagnosis as soon as possible, because if the lumen of the tube communicates with the endometrial cavity of the rudimentary uterus, then retrograde menstruation and pelvic endometriosis may occur. In this case, non-communicating right rudimentary horn with retrograde menstruation probably led to the development of right hydrosalpinx and endometriotic cyst of the ovary.
Management of this uterine anomaly is mainly surgical consisting of excision of functioning non-communicating horn followed by uterine reconstruction.[5]

References

1. Shukla R. Isolated torsion of the hydrosalpinx: a rare presentation. Br J Radiol. 2004; 77:784–86.
2. Speroff L. The uterus. In: Mitchell C, ed. Clinical Gynecologic Endocrinology and Infertility. 7th ed. Philadelphia,Pa: Lippincott Williams & Wilkins; 2005.p 113.
3. Parulekar SV. Classification of Congenital Malformations of The Female Genital Tract. JPGO 2015;2(4). Available from: http://www.jpgo.org/2015/04/eac-classification-of-congenital.html.
4. American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal    tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, mullerian anomalies and intrauterine adhesions. Fertil Steril 1988;49: 944.
5. Strassman EO. Operations for double uterus and endometrial atresia. Cln Obstet Gynecol 1961; 4:240.

Citation

Jha N, Chaudhari HK, More V. Torsion Of Hematosalpinx In A Case Of Unicornuate Uterus. JPGO 2019. Volume 6 No.4. Available from: https://www.jpgo.org/2019/04/torsion-of-hematosalpinx-in-case-of.html

Subserosal Fibroid Misdiagnosed As Ovarian Mass In Pregnancy: Even MRI Is Not 100% Diagnostic

Author Information

Verma ML*, Sachan R**, Singh A***, Kumari M****, Singh U**, Sankhwar PL**.
(* Assistant Professor, ** Professor, *** Senior resident, **** Associate Professor.
 Department of Obstetrics and Gynecology, KGMC, Lucknow, India.)

Abstract

Even MRI may not clear diagnostic dilemma in big fibroids with cystic or hyaline degeneration. We present a 24 years old primigravida who was diagnosed to have a right adnexal mass of ovarian origin. She had a successful vaginal delivery. At 2 months postnatal, laparotomy revealed this mass to be a subserosal fibroid with hyaline degenerative changes.

Introduction

MRI seems to be a better modality for giving accurate diagnosis for fibroids where ultrasonography (USG) findings are inconclusive. However, in fibroids with cystic and hyaline degenerative changes, MRI may also not be 100 % accurate. Once there is a diagnosis of ovarian mass with pregnancy in second trimester, patients are considered for laparotomy. However, since our patient had presented at 37 weeks, she was given a trial of labor and she delivered vaginally successfully.

Case Report

A 24 years old primigravida was admitted to our emergency department at 37 weeks of gestation with chief complaints of intermittent lower abdominal pain and vaginal discharge since last one day. She had been married for 4 years and this pregnancy was a spontaneous conception. On general examination she had mild pallor and BP was 150/100 mm of Hg. On abdominal examination, abdomen was overtly distended. Huge tense abdominal swelling around 20 x 10 cm located towards the right side of the uterus was felt. Uterus of around 34 weeks size was deviated towards the left. Abdominal findings were suggestive of pregnancy with a huge adnexal mass. She had also got one MRI done at 30 weeks of gestation which was suggestive of a huge multiloculated solid cystic space occupying lesion of ovarian origin measuring 18 x 12 x 11 cm. A single live intrauterine fetus of 29 weeks of gestation with placenta maturity grade 3 and adequate liquor was also reported (figure 1). Decision was taken for pre-induction cervical ripening with prostaglandins in view of poor bishop score. Examination findings showed that the adnexal mass was not obstructing the lower uterine segment. An indentation could be felt between the mass and uterine contour supporting that mass per se would not cause labor problems or complications. Internal examination on admission revealed unripe cervix hence induction with misoprostol by per vaginal route was decided and dose of 25 microgram four hourly was started. She entered into active labor after 4 doses. She had outlet forceps vaginal delivery of female baby. Indication of forceps was poor maternal bearing down efforts. The baby had a good Apgar score of 8/10 and 9/10 at 1 minute and 5 minutes respectively. Her birth weight was 2.4 kg. Labor and postpartum period were uneventful. She was discharged on 4th post-natal day with both mother and baby in satisfactory condition.

Figure1. Antenatal MRI showing solid cystic space occupying lesion with fetus.

Figure 2. Peroperative images of subserosal fibroid.

Figure 3. Histopathology showing leiomyoma.

In postpartum period, all tumor markers were done which were within normal limits. She was closely followed up in the postpartum period. Two months after delivery she underwent a laparotomy and per operatively a huge subserosal fibroid was detected with multiple small fibroids (figure 2). Myomectomy was done. Leiomyoma was confirmed on histopathological examination (figure 3).

Discussion

The average incidence of adnexal masses with pregnancies is 1 in 200.[1] There is  literature available about laparotomy in second trimester of pregnancy with adnexal mass or in third trimester according to presentation. Similarly, there are case reports of removal of mucinous cystadenomas weighing 6 kg after cesarean section by Yenicesu GI et al and 6.3 kg by Qublan HS et al.[2,3] However there is paucity of data about whether patients with asymptomatic huge adnexal mass with pregnancy, should be given trial of vaginal delivery or not. One case similar to our case with a huge ovarian mass who delivered vaginally was reported by Dipak et al and her vaginal delivery occurred without any intrapartum and postpartum complications.[4] If asymptomatic adnexal masses with pregnancy in third trimester present in emergency, but they do not cause obstruction of the lower uterine segment, then trial of vaginal delivery should be given or not, is still not very clear. However, in our case our patient was induced with all preparations for emergency cesarean delivery if required but finally she had successful vaginal delivery. However, in the postpartum period these masses should be followed up vigilantly and treatment provided. Degenerative changes in fibroids may result in heterogeneous or unusual presentations that may lead to a diagnostic dilemma.[5] MRI plays a crucial role in determining the origin and nature of a pelvic mass in cases with inconclusive USG features.[6] MRI appearances of leiomyomas vary widely and may present a diagnostic problem and even MRI may not be 100% accurate.[7] Although MRI helps in cases where USG findings are inconclusive but when fibroids undergo cystic or hyaline degenerative changes, even MRI may not give the correct diagnosis.

References

1. Katz VL, Watson WJ, Hansen WF, Washington JL Massive ovarian tumor complicating pregnancy. A case report. J Reprod Med 1993; 38(11): 907-10.
2. Yenicesu GI, Cetin M, Arici S. A huge ovarian mucinous cystadenoma complicating pregnancy: a case report. Cumhuriyet Medical Journal. 2009;31:174–7.
3. Qublan HS, Al-Ghoweri AS, Al-Kaisi NS, Abu-Khait SA. Benign mucinous cystadenoma with stromal luteinization during pregnancy: a hormonally responsive tumor and a rare cause of fetal intrauterine growth restriction. J Obstet Gynaecol Res. 2002;28(2):104–7.
4. Mandi D, Mondal RC, Bhar D., Maity AK, Nandi MK, Singh K. Successful Vaginal Delivery despite a Huge Ovarian Mucinous Cystadenoma Complicating Pregnancy. A Case Report.  Iran J Med Sci.2013;38(4):339-42.
5. Ahamed KS, Raymond GS. Answer to case of the month #103. Large subserosal uterine leiomyoma with cystic degeneration presenting as an abdominal mass. Can Assoc Radiol J. 2005;56(4):245–7.
6. Maizlin ZV, Vos PM, Cooperberg PL. Is it a fibroid? Are you sure? Sonography with MRI assistance. Ultrasound Q. 2007;23(1):55-62.
7. Hricak H, Tscholakoff D, Heinrichs L, Fisher MR, Dooms GC, Reinhold C, et al. Uterine leiomyomas: correlation of MR, histopathologic findings, and symptoms. Radiology. 1986;158(2):385-91.

Citation

Verma ML, Sachan R, Singh A, Kumari M, Singh U, Sankhwar PL. Subserosal Fibroid Misdiagnosed As Ovarian Mass In Pregnancy: Even MRI Is Not 100% Diagnostic. JPGO 2019. Volume 6 No.4. Available from: https://www.jpgo.org/2019/04/subserosal-fibroid-misdiagnosed-as.html

Vaginal Pull Through Procedure To Treat Isolated Lower One Third Vaginal Agenesis

Author Information

Saxena A*, Pardeshi S**, Gupta AS***.
(* Junior Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Vaginal agenesis is a rare condition. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome or Mullerian agenesis is the most common etiology which is described as congenital absence of uterus and vagina with female genotype, normal secondary sexual characteristics and ovaries. Absence or agenesis of various segments is usually uncommon. We are presenting one case of an adolescent girl who developed massive hematocolpos due to absence of the lower one third of the vagina.

Introduction

Vaginal agenesis has an incidence of 1 in 4,000 to 1 in 10,000 females.[1] It may occur rarely as an isolated finding.[2, 3]. Here we are reporting one such case of vaginal agenesis, who presented with abdominal pain and an abdominal mass in our emergency receiving room and was managed surgically to drain the hematometra and create an outflow tract, while keeping her menstrual and reproductive function intact.

Case Report

A 15 years old, unmarried, student of grade VII, who had not attained menarche, came to our receiving room at 1:00 am with complaints of pain in abdomen. She was apparently alright till 3 days ago, when she had sudden pain in abdomen and her mother felt an abdominal mass. She was immediately taken to a private hospital and investigations were carried out. Amongst routine investigations, hemoglobin was 7.8 gm%, total leucocytes were 9,480/mm3, platelets were 1,93,000/mm3 and creatinine was 2.9 mg%. She was given symptomatic treatment and referred to our hospital.
On examination, per abdominally, a huge cystic mass arising from the pelvis and going up to the right hypochondrium with mild tenderness was felt. Infraumbilical, the mass was in the midline and in the upper abdomen the mass was felt more on the right side of the midline. It was fusiform to cylindrical in shape. On local examination, labia majora, labia minora and urethral and anal opening were normal but the vaginal opening was blind and flat. On per rectal examination, cystic collection was felt approximately 6 cms above the anal opening. Fecal matter was felt further. Abdominal movements of the mass were transmitted to its lower end. An impression of cervical atresia, with a functioning uterus with hematometra and hematosalpinx with renal compromise was made.
Ultrasonography of the abdomen and pelvis was suggestive of a very large cystic lesion with homogeneous internal echoes. The mass measured 21x10 cm and was seen extending from pelvis to the epigastric region. Uterine shadow was not seen separately. MRI was done which was suggestive of hematometra, hematocolpos and bilateral hydrosalpinx with blind ending cervical canal, which gave the impression of cervical atresia. Bilateral hydronephrosis and hydroureter were also probably due to extrinsic compression. Right kidney was found to be small, measuring 4.9x3.3 cms. Left kidney was of normal dimension.
Opinions of urologists and nephrologists were taken. She was catheterized and after fitness from the urologist, nephrologist and anesthetist an exploratory laparotomy with consent for possible hysterectomy and excision of the hematosalpinx was done.
Exploratory laparotomy was done under spinal and epidural anesthesia, through an infra umbilical midline incision. In vitro, large hematocolpos and hematometra were seen extending up to the right hypochondrium, just under the liver surface. Aspiration of the hematocolpos was done. After draining 100 cc of old blood, a 2-inch vertical incision was made on the anterior surface of the mass.  Almost 2 liters of blood from the hematocolpos and hematometra were drained after which the uterus was exteriorized. On inspection ovaries were normal, both tubes were stretched and had a retort shape.They were distended to  due to hematosalpinx. Uterus was about 10 weeks in size even after drainage of the old blood. An over distended vagina was seen. A bimanual digital palpation through the anterior vaginal wall incision towards the blind introitus, revealed a distance of 3 cms from the vaginal dimple.
In view of the grossly elongated vagina (empty vagina was just above the umbilicus) a decision to pull through the vagina and creation of an introitus by fixing the lower end of the vaginal mucosa to the perineal skin was made. Lithotomy position was then given and under all aseptic precautions, a cruciate incision was made below the urethral opening at the site of the vaginal dimple. Careful dissection between the bladder anteriorly and the rectum posteriorly was guided by palpating the digit of the surgeon who was at the abdominal end of the procedure. Vagina was distended abdominally with 100 cc of methylene blue to detect opening of the blind upper vagina into the dissected space. A long Kelly’s forceps from above was passed till it indented the roof of the dissected space and was felt from below. Area checked to confirm it was away from the bladder anteriorly and rectum posteriorly. Incision was taken on the tip of the Kelly’s forceps and the incision was then extended laterally. The vaginal edges were held all around and then undermined to prevent tension on the vagina. Fascia in the dissected space was incised laterally on both sides to circumvent possibility of vaginal stricture. Hemostasis in the bed was confirmed. The vaginal mucosal edges were then sutured with simple, interrupted sutures to the fascia radially with 0 no. polyglactin sutures. Fascia was then reinforced and sutured to the perineal skin from lower end of labia minora to the other labia minora. Anteriorly this was sutured to the tissue below the urethral meatus. One finger could easily be passed through the neovaginal opening. Abdominal anterior vaginotomy was sutured with simple, interrupted sutures 0 no. polyglactin sutures. Hematosalpinx was left untouched to allow natural healing. She tolerated the procedure well and got her menses after 4 days.

Figure 1. MRI. Yellow arrow shows hematocolpos which was mistaken for the atretic cervix. Yellow and green arrow is the hematometra.

Figure 2. Yellow and green arrow mark the distended, drained vagina. Blue and yellow arrow is the site of anterior colpotomy.

Figure 3. Blind Vagina

Figure 4. Dissection in the blind vagina roof to reach the upper cannalized part of the vagina.

Figure 5. Tip of the Kelly’s forceps (Yellow Arrow)

Figure 6. Anastomosed upper patent vagina to the introitus.

Daily vaginal digital examinations were done to check and maintain the patency of the vagina. After 2 weeks of the procedure, she and her mother were taught the correct usage of glass dilators and she was discharged with instructions to use the vaginal dilator daily. Weekly follow ups were advised but she was lost to follow up for almost a month.
When she finally followed up in OPD, a vaginal stricture had formed even though her mother gave history of regular use of glass dilators. On local examination, a vaginal stricture at about 3 cm from the introitus was seen. No epithelialization was seen in the 3 cm area. Opening was identified with a uterine sound and an artery forceps could be introduced. Clear fluid flowed out but tip of finger couldn’t be admitted.
She was posted for a second procedure where dilation of the stricture with radial incision and with placement of amnion graft in the lower one third of the vagina with Styrofoam vaginal mold was planned. Stricture was dilated with Hegar dilators upto no.20 till a opening of 2 cm in diameter was created. Radial incisions at 10’o clock, 2’o clock, 4’o clock and 8’o clock were taken to release the stricture and the flaps thus created were sutured anteriorly to the vestibule. Posteriorly a flap could not be raised due to lack of epithelization. An amnion graft was placed over the Styrofoam mold into the vagina so as to ensure the raw area had contact with amnion graft. Introitus was closed. Foley’s catheter was inserted in the bladder and left in situ. After a week, the mold was changed under all aseptic precautions. The soft mold was changed again after a week and replaced by a prosthetic mold which she or her mother could change as and when required. She was discharged with instructions to wear the mold every night till she becomes sexually active and to follow up as instructed. Her monthly followup shows a patent vagina. She is menstruating at regular intervals and wearing the mold at night regularly.

Figure 7. Placing vaginal mold with amnion graft (arrows).

Figure 8. Vagina closed after inserting mold.

Discussion

Vaginal agenesis is the result of complete failure of the vaginal plate to canalize. Mullerian agenesis may be of varying degrees, distal to proximal vagina, cervix or uterus or near total absence of all mullerain structures with associated degrees of Mullerian aplasia.[4]
The diagnosis may be unsettling for the patient and her family. Therefore, attention has to be paid not only to the correction of the anatomical abnormality but also to the psychosocial issues.[5] Counseling about future fertility and sexual function is also extremely important. In patients with isolated vaginal agenesis with a functioning uterus and cervix, normal fertility can be expected in future. However, infertility issues may occur due to hematosalpinx, endometriosis and the surgery itself.

The timing for both nonsurgical and surgical correction of this anomaly is purely elective unless the patient is symptomatic.[5] As such in patients with intact uterus experiencing cyclic pelvic pain as a result of obstructed menstrual fluid flow, surgical correction at the earliest is mandatory at the time of diagnosis. In the literature, although more than 10 surgical procedures for neovaginal reconstruction have been described so far, an ideal approach has not yet been identified. Emran et al reconstructed the vagina using a sigmoid colon pedicle flap successfully. The proximal part of the created vagina was connected to the cervix and a Foley’s catheter was left in situ for 7 days to prevent obstruction.[6] Beksak described a procedure in a 14 year old girl with a normal female genotype and secondary sexual characteristics with complains of primary amenorrhea and cyclic pain in the pelvis. A vaginal dimple without a normal vaginal orifice was seen on local examination and an enlarged uterus was felt on bimanual recto-abdominal examination. A transverse incision at the site of vaginal dimple was taken. Blunt dissection was done to create a potential space between the bladder and urethra in front and rectum behind. The surgeon palpated the catheter in front and assistant’s finger in rectum to avoid injury while dissecting. A neovagina was obtained which was 9-10 cms long and 3-4 cms wide. in spite of this, the collection couldn’t be reached and an abdominal approach was required to drain the hematometra. An 8-french Foleys catheter was placed in its central lumen with its tip located in the uterine cavity. Its balloon was insufflated with 3 mL saline. Endometrium and myometrium were closed separately. To maintain the mold in position, it was sutured to the labia majora. Hyalobarrier gel was applied between the mold and the neovaginal walls.[7] Many a times it is not possible to drain a hematometra or hematocolpos through a vaginal approach and an abdomino-perineal approach is often required.

We primarily decided an abdominal procedure as the hematometra reported on MRI was upto the right hypochondrium and differential diagnosis was of cervical atresia or hematocolpos. Since it was an over distended vagina containing more than 2 liters of blood which we drained we realized that we would be able to pull through this stretched vagina and fix it to the introitus thus resulting in a vaginal reconstruction with normal vaginal epithelium. We also used an abdomino-perineal approach to align the upper vagina into the dissected space and its fixation to the introitus. This was successfully performed and the daily digital vaginal dilatation maintained it patent. However, though the vaginal dilatation was taught to the girl’s mother and also confirmed, the upper vagina retracted from the introital sutures. Hence in the second sitting we had to excise the constriction ring of about 1cm in width between junction of upper vagina and lower vagina and place a graft on it.
Many complications are associated with the procedure. Bowel and bladder injury are serious complications that have to be explained to the family before surgery. Vaginal molds which are used to prevent restenosis may be associated with many problems. An unsatisfactory reconstruction due to poor drainage, graft maceration, sloughing and graft detachment may occur due to non ideal vaginal molds. Using an appropriate vaginal mold is the key to achieving good results.
Although multiple options are available for allografts, amniotic membrane was used by us as an allograft in vaginal reconstruction as it is inexpensive, easily available and has low antigenicity.

Conclusion

Various grafts have been used to line the neovagina. Pull through of the upper vagina upto the introitus should theoretically prevent stricture formation provided the upper vagina does not retract back. However, daily wearing of the vaginal mold will still have to be continued till the girl maintains an actice sexual life.

Acknowledgement

We would like to thank Dr. S. V. Parulekar for his valuable intraoperative inputs.

References

1. Evans TN, Poland ML, Boving RL. Vaginal malformations. American Journal of Obstetrics and Gynecology. 1981;141(8):910-920.
2. Current evaluation of amenorrhea. Practice Committee of the American Society for Reproductive Medicine. Fertility and Sterility. 2006;86(5 Suppl 1):148-155.
3. Müllerian agenesis: diagnosis, management, and treatment. ACOG Committee Opinion No. 728. American College of Obstetricians and Gynecologists. Obstet Gynecol 2018;131:e35–42.
4. Miller RJ, Breech LL. Surgical correction of vaginal anomalies. Clin Obstet Gynecol. 2008;51(2):223-36.
5. Laufer MR. Congenital absence of the vagina: in search of the perfect solution. When, and by what technique, should a vagina be created? Curr Opin Obstet Gynecol. 2002;14(5):441-4.
6. Erman-Akar M, Ozkan O, Ozkan O, Yucel S, Dolay K, Ertugrul F, et al. Uterine preservation and vaginal reconstruction in a patient with congenital vaginal agenesis presenting with cyclic menouria. J Minim Invasive Gynecol. 2011;18(5):682-5.
7. Beksac MS, Salman MC, Dogan NU. A new technique for surgical treatment of vaginal agenesis using combined abdominal-perineal approach. Case Reports in Medicine. 2011;2011:1-6.

Citation

Saxena A, Pardeshi S, Gupta AS. Vaginal Pull Through Procedure To Treat Isolated Lower One Third Vaginal Agenesis. JPGO 2019. Volume 6 No.4. Available from: https://www.jpgo.org/2019/04/vaginal-pull-through-procedure-to-treat.html

Alloimmunisation With Rare Blood Group In Twin Pregnancy

Author Information

Singhania N*, Vaidya A**, Gupta AS***.
(* Junior Resident, ** Senior Resident, *** Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

A blood group is called rare when it is found in a frequency of 1:1000 samples of a given population. Rare blood groups are those who lack high frequency antigens. Anti-Rh17 is a rare alloantibody produced after an immune stimulus like blood transfusion or pregnancy,   by individuals who lack C/c and E/e antigens of Rh blood group on their red cells. This rare blood group is denoted as D- -. We report diagnosis and management of a pregnant lady with anemia, monchorionic diamniotic twins and presence of anti-Rh17 alloantibodies.

Introduction

Rare blood groups are those who lack high frequency antigen in relation to blood transfusion and they occur with a frequency of 1:1000 samples of a given population.[1] Rare blood type may vary from country to country and therefore a blood type rare in one country may not be considered rare in another. The following rare blood types were encountered over a period in India; “Bombay” (Oh) phenotype, D -/- D -, In(a+b-), Co(a-b-), weaker variants of A, B and H antigens, I-i-, CdE/CdE (ryry) and Mg.[2]  Anti-Rh17 is a rare alloantibody.[3] This rare blood group is designated as D- - and was first described by Race and Sanger in 1950.[4]

Case Report

A 25 year old primigravida, a nurse by profession with monchorionic diamniotic twins registered in the antenatal OPD at 10 weeks of pregnancy. She had regular antenatal visits. Her booking hemoglobin (Hb) was 11.4 mg/dl. At 24.5 weeks of gestation she was admitted in view of threatened preterm labor. Tocolysis was given. In her routine investigations, she was found to be anemic with Hb of 8.5 mg/dl, total leucocyte count (TLC) was 10,700/mm3, and platelet count was 3.2 lacs/cu mm. Serum ferritin was 10.48 ng/ml. Serum bilirubin was within normal limits. Serum LDH was 660 IU/liter. Peripheral smear was suggestive of anisocytosis, poikilocytosis, hypochromia, microcytosis, macrocytosis, target RBC’s with Rouleax formation. Hb electrophoresis showed no abnormal hemoglobin’s.  All other investigations were within normal limits. Parenteral iron therapy was initiated but there was no improvement even after 2 weeks. Hemoglobin was persistently low. The blood grouping report was suggestive of autoantibodies. Hematologist opinion was taken and was advised to do husband’s blood group. Extended blood group typing and antibody panel with temperature sensitivity was also advised. Husband’s blood group was B positive. Extended blood grouping suggested ABO blood group to be B with Rh group to be D--/D--. All her family members were tested for this rare blood group and only her younger teenage sibling was found to have similar group. No donor was available for her. Hemoglobin was serially monitored every 2 weeks and fetal surveillance was done to rule out fetal anemia by serial ultrasonography for MCA PSV. She was continued on parenteral iron, vitamin B12 and folic acid therapy. Her hemoglobin raised to 12.4 mg/dl. Serial ultrasonography for MCA PSV done every 2 weekly showed a rising trend but was always less than 1.5 mean over median (MOM). At 30.3 weeks of gestation she went into inevitable preterm labor. Tocolysis was stopped. Transfusion medicine consultant advised not to transfuse any products containing RBC’s. Plasma products like FFP, platelets and cryoprecipitate can be given. She delivered vaginally with first twin, male child of 1.586 kg by vertex presentation and second twin, male child of 1.620 kg by breech extraction. Apgar score was 9/10 for both babies. Postpartum hemorrhage prophylaxis with oxytocin and injection methylergometrine were given. There was no postpartum hemorrhage and no blood transfusion was required. Babies were kept in NICU for low birth weight. There blood groups were B positive (both of them). Later on the babies were discharged from NICU uneventfully.

Discussion

Rh17 is a sub type of Rhesus blood group. The Rh blood group system has two Rh genes (D and CE). Four combinations are possible by CE. These can be CE, Ce, cE or ce. Absence of this cDe is referred to as Rh17. It is a high frequency antigen negative blood. Anti-Rh17 (anti-Hro) is a monospecific IgG alloantibody, which reacts with a common determinant on the Rh-CE protein, generally found in patients with history of blood or blood products transfusion or pregnancy. The genetic events producing the D-- phenotype are not yet understood.  According to a case report published by Huang CH et al, recombination of portions of the Rh-D gene with the Rh-CE gene leads to overexpression of Rh-D antigens and the lack of Rh-CE antigens, thus producing Rh17. The same case report also stated that this particular phenotype can be inherited by compound heterozygosity also, apart from consanguineous homozygosity, hence all blood relatives of the patient should be screened for a similar blood group.[5] A total of 3 cases of anti-Rh17 allo-immunisation have been reported in English literature and 40 cases in Japanese literature till now. According to a study by Yun JW et al even trivial amounts of RBCs in platelet concentrates can also trigger sensitization to the highly immunogenic C, c, E, or e antigens and induce the formation of anti-Rh17 and other Rh antibodies.[6] Since our patient is nurse by profession we speculate that needle prick injury could be the source for exposure to trivial amount of RBCs, thus triggering sensitization in our patient. Surgery is usually contraindicated in such cases but if required, intraoperative blood saving units should be used or appropriate donors having same blood group or Rh-null RBC’S can be transfused. A few cases have been reported using exchange transfusion postpartum to decrease the number of antibodies but it did not prove to be very efficacious.[7] Past literature reviews have concluded that Anti-Rh17 are known to produce hemolytic disease of the new-born which ranges from mild to fatal. Fetal anemia should be monitored throughout pregnancy and if required intrauterine transfusions should be given with frozen rare donor blood or maternal blood.[8]

References

1. Joshi SR, Vasantha K. A profile of rare bloods in India and its impact in blood transfusion service. Asian J Transfus Sci. 2012;6(1):42-3.
2. Reesink HW, Engelfriet CP, Schennach H, Gassner C, Wendel S, Fontão-Wendel R, et al. Donors with a rare phenol (geno) type. Vox Sang. 2008;95(3):236–53.
3. Reid ME, Oyen R, Morsh WL. Summary   of   the clinical   significance   of   blood   group   alloantibodies. Seminars in Hematology April 2000; 37(2):197-216.
4. Race RR, Sanger R. Blood Groups in Man. 6th ed. Oxford: Blackwell Scientific Publications; 1975.
5. Huang CH, Peng J, Chen HC, Chen YX, Lin DT, Lin SW, et al. RH locus contraction in a novel Dc-/D-- genotype resulting from separate genetic recombination events. Transfusion 2004;44(6):853-85.
6. Yun JW, Kang E-S, Ki C-S, Koh KC, Kim DW. Sensitization to Multiple Rh Antigens by Transfusion of Random Donor Platelet Concentrates in a -D- Phenotype Patient. Ann Lab Med 2012;32(6):429-32.    
7. Rock G, Lafreniere I, Chan L, McCombie N. Plasma exchange in the treatment of hemolytic disease of the newborn. Transfusion. 1981;21(5):546-51.
8. Whang DH, Kim HC, Hur M, Choi JH, Park JS, Han KS. A successful delivery of a baby from a D--/D-- mother with strong anti-Hr0. Immunohematology 2000:16(3):112-4.

Citation

Singhania N, Vaidya A, Gupta AS. Alloimmunisation with rare blood group in twin pregnancy. JPGO 2019. Volume 6 No.4. Available from: https://www.jpgo.org/2019/04/alloimmunisation-with-rare-blood-group.html

Successful Management Of Essential Thrombocytosis In Pregnancy

Author Information

Tagad M*, Tiwari N**, Chaudhari HK***.
(* Junior Resident, ** Assistant Professor, *** Associate Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Patients with essential thrombocythemia (ET) are mostly women. Some of them are diagnosed at childbearing age. Decision making on pregnancy is a common issue in the clinical management of women with ET. There is limited information regarding the outcome of pregnancy in patients with ET. We report here one case with ET in a pregnant woman at our institute.

Introduction

Essential thrombocythemia (ET) is a chronic myeloproliferative disorder with an increased risk of vascular complications.[1] It is a clonal expansion of multipotent stem cells.Thrombocytosis is a part of ET and is also found in 50 % of patients with polycythemia vera and 35 % patients of chronic myeloid leukemia. Similar to idiopathic thrombocytopenia of pregnancy, ET is also a diagnosis of exclusion once other reasons for thrombocytosis such as reactive thrombocytosis, myelodysplastic syndrome (MDS) and other myeloproliferative neoplasms (MPN) have been eliminated. Pregnancy with essential thrombocythemia are at high risk of spontaneous abortion and other pregnancy complications. Live birth rate of pregnancy is 50 %-70 % and abortion rate is 25- 50 %.[2]

Case Report

A 30 years old gravida 4 with 1 prior spontaneous abortion and 1 prior sudden term intrauterine fetal demise at 36 weeks of gestation with bad obstetric history diagnosed with essential thrombocytosis due to JAK2 mutation was admitted with us. Bone marrow  examination done in view of bad obstetrics history in first trimester was suggestive of    JAK2 mutation and she was started on tablet aspirin 75 mg daily and injection low molecular weight heparin 0.6 cc subcutaneous (s.c.) daily in the first trimester. Injection Interferon α -2 β (3mu) s.c. once a week was advised from second trimester of pregnancy if platelet count was less than 4.5 lakhs/ μl. Her general condition was fair. On per abdomen examination uterus was 36 weeks, cephalic presentation, FHS were present and uterus was relaxed On per vagina examination her cervical os was closed.
Hematologist was consulted for peripartum management. She was advised complete hemogram. Her platelet count was 4.01 lakhs/ μl. Her leucocyte count was 10,100 cells/ cu.mm. Her coagulation profile was normal. Thombophilia profile was negative. Her obstetrics scan at term was suggestive of mild IUGR and mild oligohydramnios. She was advised to stop tablet aspirin at 37 weeks of gestation but continue injection LMWH 0.6 cc s.c. daily till 24 hours prior to delivery subsequently to restart it after achieving complete hemostasis post delivery till 6 weeks postpartum. Injection Interferon α 2 β 3 mu s.c. once a week was continued weekly. She went in labor spontaneously at 39 weeks of gestation. Injection LMWH was stopped. Labor was monitored as per protocols. She delivered a female child of 3.3 kg uneventfully. Injection LMWH was restarted as per advice of hematologist after complete hemostasis.

Discussion

In ET, platelet count or leucocyte count are not high risk factors for pregnancy complications. The JAK2 mutation was found in 50 % of patients. The JAK2 mutation assessment is a key tool in the diagnostic work-up of patients with chronic myeloproliferative disorders.[1,2] Women having the mutation have higher risk of complication during pregnancy; like abortion, intrauterine fetal retardation and still birth. However, pregnancy is not contraindicated in a patient with essential thrombocytosis.
Complete hemogram is must in these cases. Bone marrow examination is the diagnostic test. Thrombophilia profile is recommended in the evaluation of a woman of childbearing age with ET for therapeutic interventions that are aimed at improving pregnancy outcome.[3]
Regarding treatment of ET during pregnancy, cytoreduction therapy is used to reduce the risk of bleeding. Hydroxyurea is the first-line medication for cytoreduction but it should be avoided in the first trimester because of its known teratogenicity.[5,6]  Interferon is the agent of choice in pregnant women with ET.[5]  Low-dose aspirin during pregnancy has been shown to be safe for the fetus without an increased risk of bleeding for the mother.[7] Aspirin is a very common drug used in patients with ET who do not have a history of bleeding.[8] Interferon inhibits the growth of megalokaryocytic progenitors in patient with essential thrombocythemia resulting in platelet reduction. Aspirin is also helpful for the same.

Conclusion

In patient’s with essential thrombocytosis, pregnancy may evolve uneventfully in most of these patient’s with proper management. Women with JAK2 mutation can have various adverse events during pregnancy like growth restriction, fetal loss and maternal complications like gestational diabetes, pregnancy induced hypertension, bleeding, and   abruptio placentae

References

1. Campbell PJ, Green AR.The myeloproliferative disorders. N Engl J Med. 2006; 355(23):2452-66.
2. Gangat N,  Wolanskyj  AP,  Schwager  S, Tefferi A. Predictors of pregnancy outcome in essential thrombocythemia: a single institution  study of 63 pregnancies. Eur J Haematol. 2009;82(5):350-3.
3. Hoffman R, Prchal JT, Samuelson S, Ciurea SO, Rondelli D. Philadelphia chromosome-negative myeloproliferative disorders: biology and treatment. Biol Blood Marrow Transplant. 2007;13(1 Suppl 1):64-72.
4. Bockenstedt PL. Management of hereditary hypercoagulable disorders. Hematology Am Soc Hematol Educ Program. 2006;444-9.
5. Harrison C. Pregnancy and its management in the Philadelphia negative myeloproliferative diseases. Br J Haematol. 2005;129(3):293-306.
6. Vantroyen B, Vanstraelen D. Management of essential thrombocythemia during pregnancy with aspirin, interferon alpha-2a and no treatment. A comparative analysis of the literature. Acta Haematol. 2002;107(3): 158-69.
7. CLASP: a randomised trial of low-dose aspirin for the prevention and treatment of pre-eclampsia among 9364 pregnant women: CLASP (Collaborative Low-dose Aspirin Study in Pregnancy) Collaborative Group. Lancet. 1994; 343(8898): 619-29.
8. Schafer AI. Molecular basis of the diagnosis and treatment of polycythemia vera and essential thrombocythemia. Blood . 2006; 107(11):4214-22.

Citation

Tagad M, Tiwari N, Chaudhari HK. Successful management of Essential thrombocytosis in pregnancy. JPGO 2019. Volume 6 No.4. Available from:  https://www.jpgo.org/2019/04/successful-management-of-essential.html

Remembering Past Greats: Grigoris Lambrakis

Author Information

Prasad M*, Venkatesh S**.
(*Assistant Professor, **Professor and Head of Department, Department of Obstetrics and Gynecology, Vydehi Institute of Medical Sciences and Research Centre, Whitefield, Bengaluru, India.)

Grigoris Lambrakis (3rd April 1912 – 27th May 1963) was an eminent Greek obstetrician-gynecologist. However, he appeared to have become more famous for his involvement in politics than his other notable achievements in many fields.[1]
He was also an athlete, and held the national record for the long jump competition for 23 long years, even after he had stopped active participation in athletic competitions. In the peak of his athletic participations, he won several gold medals in the Balkan athletic games.[2]
He was educated in medicine in Athens, Berne and Munich and having served under the mentorship of Ernst Sauerbrunch, he gained surgical dexterity which was far superior than his gynecologist peers. The gynecological fraternity of Greece had fondly called him ‘artist with a scalpel’.
He became a lecturer in the public maternity hospital by the age of 38, which was an achievement in itself. As he gained public acceptance, he ran many small private clinics for the downtrodden and was praised widely for the same.[3]

Part of his medical education consisted of tutoring under some eminent endocrinologists of their time, and hence Lambrakis forayed into gynecological endocrinology, an otherwise untouched subject in those times. His doctoral research was on relationship between changes in potassium, calcium and glucose during labour and its correlation with uterine inertia. Though he became a pioneer in the field of gynecological endocrinology in Greece, it was beset by problems. His attempt to open a branch of a Germany based clinic specialised in research in endocrinology, in Greece led to some controversies. By 1949, he had published a book titled ‘Diabetes and Pregnancy’.[2]
During the second world war, he was drawn into activism and politics, and had organised many events as a part of the Greek resistance to the war. Due to his political leanings, he was identified as a ‘pacifist’, one who astutely opposed to war or violence in any form. Despite his political activism, he always made time to see patients and continued to oversee activities of his clinics. Eventually, he became a prominent figure in Greek politics, and was unfortunately assassinated by political rivals. His popularity as a public figure was revealed upon his death, when large protests broke out, and large scale changes in the political leadership of the country followed.[1]
He left behind a rich legacy and recently even a documentary film was made on him titled ‘Marathon of an unfinished spring- G Lambrakis’.[4]
To summarize, Grigoris Lambrakis was an all-rounder and positively impacted his country. His life gives us the inspiration that we gynecologists have the power to make the world a better place to be in.

References

1. Grigoris Lambrakis. Available from: https://en.wikipedia.org/wiki/Grigoris_Lambrakis 
2. Gkegkes ID, Karamanou M, Iavazzo PE, Gkegke XE, Androutsos G, Iavazzo C. Grigoris Lambrakis (1912-1963) - a Greek obstetrician and world renowned activist. Acta Med Hist Adriat. 2016 Aug;14(1):177-84.
3. Gkotzaridis. A Pacifist's Life and Death: Grigorios Lambrakis and Greece in the Long Shadow of Civil War. Newcastle upon Tyne. Cambridge Scholars publishing; 2016
4. London Greek Film festival 2014. Available from: http://www.londongreekfilmfestival.com/lgff2014filminfo/MarathonofanunfinishedspringGrigorisLambrakis.htm

Citation

Prasad M, Venkatesh S. Remembering Past Greats: Grigoris Lambrakis. JPGO 2019.Volume 6. No.4. Available from: https://www.jpgo.org/2019/04/remembering-past-greats-grigoris.html