Archived Volumes of Past Issues

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Volume 6 Number 1

Editorial
Parulekar SV

Recurrent Hemihematometra In Herlyn–Werner–Wunderlich Syndrome
Parulekar SV

Very Early Primary Abdominal Ectopic Pregnancy
Parulekar SV

Huge Extraperitoneal Anterior Abdominal Wall Fibroid
Paranjpe S, Paranjpe A, Paranjpe HE.

Laparoscopic Management Of Ruptured Ectopic Pregnancy With Massive Hemoperitoneum
Shah N, Joshi A, Mourya S.

A Rare Presentation Of Bilateral Dermoid Cysts With Unilateral Torsion In An Adolescent Girl
Shah N, Joshi A, Mourya S.

Successful Pregnancy In a Factor X Deficient Woman
Saxena A, Pardeshi S, Gupta AS.

A Rare Case Of Portal Vein Cavernoma In An Antenatal Patient
Malani K, Samant PY, Thakur HS, Bharthi S.

Broad Ligament Ectopic Pregnancy Diagnosed And Managed Laparoscopically
Shah NH, Mourya S, Paranjpe S.

Remembering Past Greats: Kermit Krantz
Prasad M.


Editorial

Parulekar SV

Hydrocephalus is a condition characterized by excessive cerebrospinal fluid due to disturbed circulation. Its incidence is between 0.2 and 1 per 1000 live births. It is a serious disorder which can cause neonatal death, infant death, mental retardation and morbidity due to associated genetic defects and congenital malformations. It is due to a number of clinicopathological conditions. A large percentage of cases are associated with other intracranial anomalies, like aqueductal stenosis, other neural tube defects, Chiari malformations, Dandy-Walker syndrome, posterior fossa cysts, alobar oloprosencephaly and polymicrogyria. It may be associated with other anomalies, like craniofacial anomalies (cleft lip and/or cleft palate, optic atrophy, low set ears, acrocephalosyndactylia and facial bone anomalies), gastrointestinal anomalies, cardiovascular anomalies, skeletal anomalies, genitourinary anomalie. It may be a part of syndromes like Meckel-Gruber syndrome, Miller-Dieker syndrome and Joubert syndrome. Chromosomal anomalies may be seen in about 20% of cases, such as trisomy 21 and triploidy. X-linked hydrocephalus is reported. Viral infections like tomegalovirus, small pox, rubella, chickenpox, coxsackievirus and herpes simplex can also cause hydrocephalus. Syphilis and toxoplasmosis can also cause the condition. Now Vanessa van der Linden et al have reported a strong association between Zika virus infection and development of hydrocephalus. Maternal diabetes mellitus can be a predisposing factor and needs to be corrected before any intervention is done.

It is essential to detect the disease early with use of ultrasonography and magnetic resonance imaging, so that the progress of the condition and damage to cerebral cortex can be arrested by fetal surgery like ventriculoamniotic shunt under ultrasonographic guidance. The prognosis depends on the underlying cause and presence of associated anomalies. The outcome is better when it is associated with arachnoid cyst, corpus callosum agenesis, atresia of Monro, and fetal intracranial hemorrhage. The outcome is not good when it is associated with encephalocele, holoprosencephaly,  syndromic hydrocephalus, chromosomal errors, and fetal virus infections. Prognosis is better when fetal hydrocephalus begins in the last trimester of gestation as compared to when it develops at the beginning of gestation. These factors need to be borne in mind when making a decision on whether to perform surgery for correction of congenital hydrocephalus. It may be necessary to deliver the woman by a cesarean section in view of fetopelvic disproportion due to large size of the fetal head. In countries where facilities for prenatal diagnosis are not easily available and fetal surgery is not possible, and even antenatal care may not be available to all cases, fetal hydrocephalus may present late in pregnancy or even in labor. Such cases are likely to develop fetal dystocia and maternal complications like uterine rupture. Fetal cerebral tissue may be in the form only a thin mantle and chances of fetal survival are rather poor. The only therapeutic option in such cases may be tapping the hydrocephalus under ultrasonographic control to reduce the size of the very large fetal head and achieve a vaginal delivery.

Our institute has facilities for prenatal diagnosis by ultrasonography and magnetic resonance imaging. Interventional ultrasonography is also possible. On the other hand, we continue to get referred cases with advanced fetal hydrocephalus diagnosed very late. I hope our students and residents benefit from this editorial so that they can manage patients belonging to either group. I also thank all of our contributors and readers, without whose enthusiastic support we would not have been able to complete five years of publication of this journal punctually and successfully.

Recurrent Hemihematometra In Herlyn–Werner–Wunderlich Syndrome

Author Information

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

Abstract

The Herlyn–Werner–Wunderlich syndrome is characterized by uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra. It usually presents normal onset of menarche, followed by progressively severe primary dysmenorrhea. A high degree of suspicion is necessary to detect it and treat it in time. A case of spontaneous resolution of hemihematometra which recurred and resolved is presented.

Introduction

The Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra.[1] Because menstrual blood from one uterus continues to drain out cyclically through one patent vagina, attention is not drawn to the condition. There is severe primary dysmenorrhea due to hemihematometra.[2] If not treated in time, it may lead to pelvic endometriosis. A case in which the hemihematometra drained into vagina spontaneously only to recur and resolve is presented. This is the first case of this type in the world literature.

Case Report

A 14-year-old girl, presented with severe dysmenorrhea and a lump in lower abdomen. She had undergone ultrasonography (USG) prior to presenting to us. It showed uterus bicornis bicollis, with normal vagina on the right side and a blind pouch of vagina on the left side. There was hemihematometra on the left side, along with hemihematocolpos of upper vagina on the right side. The right kidney was absent. Her medical and surgical history was not contributory. She was not sexually active. Her general and systemic examination revealed no abnormality. There was a tender lump in the suprapubic region reaching 2 cm above the pubis. Her external genitals were normal and hymen was intact. A rectal examination showed a tense bulging cystic lump low in the right part and center of the pelvis, measuring about 5-6 cm in diameter. The uterus was felt separately from the mass. There was no other abnormality. A computerized tomographic (CT) scan of the abdomen and pelvis was done. It showed uterus bicornis bicollis, with normal vagina on the right side and a blind pouch of vagina on the left side. There was hemihematometra on the left side, along with hemihematocolpos of upper vagina on the right side. The right kidney was absent.

Investigations for fitness for anesthesia showed normal reports. She was scheduled to undergo surgical connection of the hematocolpos to the lower vagina. But when she reported to the hospital for admission, there was no pelvic lump. On enquiry she stated that she had menses three days ago, and had passed a lot of blood, which was mainly dark blackish in color. Pelvic ultrasonography was done. It showed no blood in the right hemiuterus and vagina. She was counselled that her condition had resolved by itself and was advised to follow up. She had regular menses subsequently. Her right hemihematometra recurred twice and resolved spontaneously by draining into lower vagina. She was counselled to undergo surgical enlargement of the opening of the hemihematometra into the lower vagina electively. She was subsequently lost to follow up.

Discussion

Anomalies of the Mullerian (paramesonephric) duct result from non- or faulty development or failure of fusion of the mullerian ducts between the sixth to ninth weeks of fetal gestation.[3,4] The uterus, cervix and the upper four-fifths of the vagina develop from the paramesonephric ducts. The lower one-fifth of the vagina develops from the sinovaginal bulbs.[5] Anomalies of the genital tract and urinary tract (which develops from the mesonephric duct) are often associated. Renal agenesis is the commonest such anomaly. HWW syndrome shows uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra. It is seen twice as often on the right side than the left side. Since one uterus is menstruating normally and its outflow tract (cervix and vagina) is patent, the girl menstruates apparently normally, and attention is not drawn to the condition. She has severe dysmenorrhea, which is often treated symptomatically, while she continues to develop hemihematocolpos, followed by hemihematometra. If it is not detected early, she may develop hematosalpinx on the same side and pelvic endometriosis. An USG is easy to perform, noninvasive and diagnostic. The diagnosis can be confirmed by CT scan or magentic resonance imaging.[6] Treatment is excision of the tissue between the hematocolpos and the lower blind vagina and connect the two to each other. There is no treatment for unilateral absence of one kidney, but it is important to know that the girl has that condition, so that adequate precautions can be taken in treatment of her future illnesses so as not to harm the single kidney.

Sheih et al reported two girls with unilateral hematocolpos and ipsilateral vaginal ectopic ureter in whom there was spontaneous rupture of uterovaginal septum. However they did not have intermittent expulsion of the blood/pus.[7] The case presented here was unique in that there was a spontaneous rupture of the right hemihepatocolpos into the lower vagina, so that the hemihematocolpos and hemihematometra drained. Unfortunately the opening was small, and hence it got plugged and the condition recurred. Luckily it opened up again and resolved. In view

References
  1. Kabiri D, Arzy Y, Hants Y. Herlyn-Werner-Wuderlich syndrome: Uterus didelphys and obstructed hemivagina with unilateral renal agenesis. Isr Med Assoc J. 2013;15(1):66.1.
  2. Jeong J-H, Kin Y-J, Chang C-H, et al. A case of Herlyn–Werner–Wunderlich syndrome with recurrent hematopyometra. J Womens Med 2009;2:77–80.
  3. Ballesio L, Andreoli C, De Cicco ML, et al. Hematocolpos in double vagina associated with uterus didelphus: US and MR findings. Eur J Radiol 2003;45:150–3.
  4. Jindal G, Kachhawa S, Meena GL, et al. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis. J Hum Reprod Sci 2009;2:87–9.
  5. Troiano RN. Magnetic resonance imaging of mullerian duct anomalies of the uterus. Top Magn Reson Imaging 2003;14:269–79.
  6. Orazi C, Lucchetti MC, Schingo PM, et al. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007;37(7):657–65.
  7. Sheih CP, Li YW, Huang TS, Liao YJ, Chen WJ. Spontaneous rupture of the uterovaginal septum in 2 girls with unilateral hematocolpos and ipsilateral vaginal ectopic ureter. J Urol. 2000 Jun;163(6):1947-8.

Citation

Parulekar SV. Recurrent Hemihematometra In Herlyn–Werner–Wunderlich Syndrome. JPGO 2019. Vol 6 No. 1. Available from: https://www.jpgo.org/2019/01/recurrent-hemihematometra-in.html

Very Early Primary Abdominal Ectopic Pregnancy

Author Information                                                                                                                 Image

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

Abstract

A primary abdominal pregnancy is a very rare variety of extrauterine pregnancy. It can occur in a number of intraperitoneal sites. Its appearance has been documented only once in world English literature. Image of another case of primary abdominal pregnancy is presented here.

Introduction

A primary abdominal pregnancy is a very rare variety of extrauterine pregnancy. Abdominal pregnancies occur at the rate of 1 in 10,000.[1] These cases include both primary and secondary types. Many of such early pregnancies are not diagnosed because they get resorbed. An image of such a pregnancy has been reported only once in the English literature.[2] Such a diagnosis is usually not suspected before and is co-incidental, at the time of a laparoscopy performed for some other indication. Image of another case of primary abdominal pregnancy is presented here.

Case Report

A 22 year old woman, married for 3 years, presented for evaluation of primary infertility. She was cohabiting for 3 years without use of any contraceptives, at a rate of 4-5 times a week. Her menstrual cycles were regular, every 28 to 35 days, with moderate flow and mild dysmenorrhea. Her last menstrual period had been 31 days after the preceding period, but was associated with scanty menstrual flow for only 2 days. Her medical and surgical history was not contributory. Her general and systemic examination revealed no abnormality. Gynecological examination showed normal abdominal findings, normal cervix and vagina, and normal uterus in anteverted position. Her husband's semen examination showed normal findings. Her investigations for fitness for anesthesia gave normal results.

Hysteroscopy and laparoscopy were performed. Hysteroscopy showed normal findings. Laparoscopy showed a normal sized and shaped uterus, normal fallopian tubes and ovaries, normal bowel and omentum (figure 1). There was an oval red elevated lesion on the back of the uterine corpus. It measured about 1 cm x 0.5 cm. It was elevated and had a flat surface. It had a pale band along its periphery, measuring 1-2 mm in width. A primary abdominal ectopic pregnancy was suspected from its appearance, being similar to that of another case described by the same author in the past.[2] A grasping forceps was passed through a second port and the lesion was elevated from the uterine surface. It could be peeled off easily, without any bleeding from its bed. The surface of the red lesion showed fine processes resembling early chorionic villi. It was fixed in fomalin and sent for histopathology. The patient made an uneventful recovery. She had menstrual flow one week after the laparoscopy. She bled for 4 days, using 3 to 4 pads per day.


Figure 1. Laparoscopic appearance of the primary abdominal ectopic pregnancy. U: back of uterus, P: pouch of Douglas, primary abdominal ectopic pregnancy (arrows).

Histological examination showed  sheets of large polygonal cells with abundant cytoplasm, minimal atypia. There was no chorionic tissue. Considering the scanty amount of tissue in the original biopsy and the fragile nature of the tissue resembling early chorionic villi, it was felt that chorionic villi had possibly been lost in the fixative by detachment from the primary biopsy. A request was made to centrifuge the fixative in the container in which the biopsy was sent. Residue after centrifugation was subjected to histological examination. It showed a few chorionic villi.

Discussion

The case was that of a primary abdominal ectopic pregnancy as per Studdiford's criteria - both fallopian tubes and ovaries were normal, there was no uteroperitoneal fistula, the pregnancy was related exclusively to the peritoneal surface and was early enough to exclude a secondary implantation.[3] It also satisfied Friedrich and Rankin's criteris of a primary abdominal pregnancy - it was less than 12 weeks of gestation and was related solely to the peritoneal surface.[4] Such a pregnancy can occur with the use of assisted reproductive techniques.[5] But the case presented had not received any treatment for her infertility prior to presenting to our center.

Documentation of image of such an early pregnancy is very rare because most of such cases are diagnosed by serial b-hCG assays, and are managed either by observation or administration of methotrexate.[2] This case is reported so as to help build up database of images of very early primary abdominal ectopic pregnancies.

Acknowledgment

I thank Dr. Shruti Panchbudhe for taking photo of laparoscopic findings in this case.

References
  1. Yildizhan R, Kurdoglu M, Kolusari A, Erten R. Primary omental pregnancy. Saudi Med J. 2008;29:606–9.
  2. Parulekar SV. Face to face: Misplaced intrauterine device and abdominal ectopic pregnancy. JPGM 2011;57:223-224.
  3. Studdiford WE. Primary peritoneal pregnancy. Am J Obstet Gynecol. 1942;44:487–91.
  4. Friedrich EG, Rankin CA. Primary pelvic peritoneal pregnancy. Obstet Gynecol. 1968;31:649–53.
  5. Rojansky N, Schenjer JG. Heterotopic pregnancy and assisted reproduction: An update. J Assist Reprod Genet. 1996;13:594–601.
Citation

Parulekar SV.Very Early Primary Abdominal Ectopic Pregnancy. JPGO 2019. Vol 6 No. 1. Available from:  https://www.jpgo.org/2019/01/very-early-primary-abdominal-ectopic.html

Huge Extraperitoneal Anterior Abdominal Wall Fibroid

Author Information

Paranjpe S,* Paranjpe A**, Paranjpe HE**
(*Director, **Consulting Gynecologist/Obstetrician. Velankar Hospital & Paranjpe Maternity Home, Mumbai, India.) 

Abstract

Fibroid is a common benign tumor of the reproductive age having a 20% incidence rate.[1] Extra uterine fibroids are usually benign and also cause a dilemma in the diagnosis. We present here an interesting case of a 45 year old lady with an extraperitoneal fibroid in the anterior abdominal wall. Patient had no concomitant fibroid in the uterus or anywhere else. She also had no history of previous open or laparoscopic fibroid surgery.  Histopathology confirmed a benign fibroid.

Introduction

Fibroids are the commonest benign tumors of the uterus. Extra uterine fibroids always pose a diagnostic dilemma because of its unusual positions. Other sites where fibroids can arise from may be the broad ligament, round ligament, fallopian tube, anterior abdominal wall or sometimes even the bowel. The fibroids which arise from these unusual locations are thought to arise due to seeding, after a previous fibroid removal surgery. These fibroids are associated with a previously morcellated uterus or a fibroid hence sometimes are also known as parasitic fibroids.

Case Report

A 45 year old lady para 2 living 2, presented with a lump in abdomen till the umbilicus. She was not aware of the lump until a sonography was done by her family doctor. She was of the opinion that her weight and abdominal fat was increasing due to sedentary lifestyle and had also started sessions of exercises for the same to reduce the fat. She had no pain in abdomen.  Only other associated complaint she had was dysmenorrhea and irregular cycles. She had previous 2 cesarean sections for her previous 2 deliveries about 20 years back.  On examination, the mass was around 22 weeks in size arising from the pelvis, non mobile and firm in consistency. Her hematological work up was within normal limits. CA 125 was 19 U/ml; also within normal limits. Ultra sonography showed a large mass arising from the pelvis probably a fundal fibroid. Both ovaries were normal. She was scheduled for exploratory laparotomy. A vertical midline incision was taken and abdomen was opened in layers till the rectus muscle. After separating the rectus muscle, the mass was visible before opening the peritoneum. The fibroid was completely separated from all sides before opening the peritoneum. The mass was completely removed with the peritoneum still intact. The consistency of the mass was similar to that of a fibroid and weighed about 2.2 Kg. After opening the peritoneum, the uterus was seen which was bulky but free from any fibroid. In view of the previous 2 cesarean deliveries, there were a lot of bladder and bowel adhesions. A subtotal hysterectomy could be carried out due to thick dense bladder adhesions. Bilateral salpingectomy was also done. While closing the peritoneum, a clear pedicle was visible. It was a small stalk like pedicle attached to the peritoneum. Hence a small portion of the peritoneum along with the pedicle was excised. The defect could be easily closed and the abdomen was closed in layers. The mass was sent for histopathology and was confirmed to be a leiomyoma with no necrosis, mitosis or atypia.

Figure 1. After separation of rectus muscle, tumor could be seen

Figure 2. High vascularity and some peritoneal attachment seen.


Figure 3. After removal of the fibroid and the uterus

Discussion

The incidence of fibroids in a premenopausal woman ranges from 20 to 30 percent as discussed earlier. But the incidence decreases sharply after menopause as it is believed that hormonal changes help in spontaneous resolution of fibroids. Fibroids outside of the uterus are a rare occurrence and are caused mostly due to seeding of small particles after a previous fibroid surgery and more after a laparoscopic morcellation.[2]  A theory has been postulated that fibroids in the anterior abdominal wall can be spontaneously formed by the formation of smooth muscle cell tumors originating from the cells of the vessel wall in the anterior abdominal wall.[3] It is suggested that fibroids can grow from smooth muscle cell of the uterus or rarely from smooth muscle of the intestine or a vessel wall. It is formed by a somatic mutation of the cells and a symbiotic action of various hormones and a deranged lipoid metabolism and growth factors.[4] Also, it has been recently found that fat cells play a role in the initiation and growth of a fibroid by a pro inflammatory cytokine called TNF-α.[3,4] Other tumors arising from the anterior abdominal wall may be well differentiated tumors originating from fat cells or muscle or mesothelial cells or fibrous tissue. Most of the smooth muscle cell tumors are benign. A malignant transformation is found in dispersed cases.[5] Here, the diagnosis of a de Novo anterior abdominal wall fibroid cannot be made as she has a history of previous 2 cesarean sections 20 years back. But there was no history of any fibroid removal surgery nor there was any concomitant uterine fibroid. There are reports of formations of fibroids in old scars as old as 30 years.[6] Also, in literature, Parasitic fibroids are described to be found in the retro peritoneum as well as in the pre peritoneum. It has been postulated that the original fibroid must have grown from the uterus , but over time it receives blood supply from some other location and is cut off from the original site like a parasite.[7] Thus as discussed , there was no gynecological surgery done in this patient. But it can always be said that it could be possible that some seeding must have occurred during the cesarean section while closing of the uterine incision which was done 20 years ago.

Conclusion

It is not yet established that any hormonal changes or local factors can form a de nova fibroid in the anterior abdominal wall. But, a differential diagnosis of a Fibroid should always be kept in mind while dealing with tumors of the anterior abdominal wall.

References
  1. Kjerulff KH, Langenberg P, Seidman JD, Stolley PD, Guzinsky GM. Uterine leiomyomas. Racial differences in severity, symptoms and age at diagnosis. J Reprod Med. 1996;41(7):483–90
  2. Schindl M, Birner P, Lösch A, Breitenecker G, Joura EA. Preperitoneal lipoleiomyoma of the abdominal wall in a postmenopausal woman. Maturitas. 2000 Nov 30;37(1):33-6.
  3. Nair S, Al-Hendy A. Adipocytes enhance the proliferation of human leiomyoma cells via TNF-α proinflammatory cytokine.Reprod Sci. 2011 Dec;18(12):1186-92.
  4. Porter KB, Tsibris JC, Nicosia SV, Murphy JM, O'Brien WF, Rao PS, et al.Estrogen-induced guinea pig model for uterine leiomyomas: do the ovaries protect? Biol Reprod. 1995 Apr;52(4):824-32.
  5. Wada-Hiraike O, Yamamoto N, Osuga Y, Yano T, Kozuma S, Taketani Y. Aberrant implantation and growth of uterine leiomyoma in the abdominal wall after laparoscopically assisted myomectomy. Fertil Steril. 2009 Nov;92(5):1747.e13-5.
  6. Kumar S, Sharma JB, Verma D, Gupta P, Roy KK, Malhotra N. Disseminated peritoneal leiomyomatosis: an unusual complication of laparoscopic myomectomy. Arch Gynecol Obstet. 2008 Jul;278(1):93-5.
  7. Yamaguchi T, Imamura Y, Yamamoto T, Fukuda M. Leiomyomatosis peritonealis disseminata with malignant change in a man.Pathol Int. 2003 Mar;53(3):179-85.
Citation

Paranjpe S, Paranjpe A, Paranjpe HE. Huge Extraperitoneal Anterior Abdominal Wall Fibroid. JPGO 2019. Volume 6  No.1. Available from: https://www.jpgo.org/2019/01/huge-extraperitoneal-anterior-abdominal.html

Laparoscopic Management Of Ruptured Ectopic Pregnancy With Massive Hemoperitoneum

Author Information

Shah N*, Joshi A**, Mourya S**.
(* Director , ** Fellow, Vardann Multispeciality Hospital, Mumbai, India.)

Abstract

Ruptured ectopic pregnancy is an obstetric emergency which can have catastrophic outcomes if timely management is not administered. Laparoscopic management of an ectopic has numerous benefits with a competent and experienced team. We present a case of massive hemoperitoneum due to a ruptured ectopic managed effectively by laparoscopy.

Introduction

Ectopic pregnancy is an extrauterine pregnancy. The incidence of ectopic pregnancies is about 1-2%.[1] A ruptured ectopic may lead to life threatening complications and needs immediate surgical management. In the current era, advanced development in the surgical techniques and equipment has made laparoscopy the surgery of choice. However, massive hemoperitoneum can pose a great challenge in managing patients laparoscopically.

Case Report

A 28 year old, primigravida presented with 2 months amenorrhea and intermittent spotting per vaginum since 3-4 days. On examination, general condition was moderate, pulse was 112 beats/ minute, blood pressure was 80/60 mm of Hg, pallor was present. Cardiovascular and respiratory system examination was normal. On per abdomen examination, abdomen was soft with full flanks and tenderness in iliac region. On per speculum examination os was closed with slight bleeding noted through the os.
Her urine pregnancy test was positive. A provisional diagnosis of ectopic pregnancy was made and colpopuncture was done. It revealed dark colored blood. Blood was drawn for investigations and cross matching, after immediate access of an intravenous line was obtained. She was started on crystalloids and colloids while awaiting for blood and blood products. An urgent transvaginal ultrasound was done which also suggested a ruptured tubal ectopic pregnancy. Laboratory investigations revealed a hemoglobin of 6.4 gm%, total leucocyte count  was 5500/cu.mm, platelet count  was 1,14,000 /cu.mm and INR was 1.4.
A decision for surgery via laparoscopy was taken. She was given general anesthesia and ketamine was used as the anesthetic agent. Three ports were inserted; 10 mm supraumbilical primary port and two 5 mm secondary ports on the left side. Intraoperatively, massive hemoperitoneum was noted with a left ruptured ampullary pregnancy. The opposite adnexa and uterus were normal. The hemoperitoneum and clots were evacuated by suction and blood loss amounted to 3 liters. Around 15 degree of head low position was given to clear the blood from the visual field and enable better visualization. Left side salpingectomy was done with the help of a harmonic and bipolar cautery. Copious peritoneal lavage was given and the ports were sutured with non-absorbable suture material. She was transfused 4 units whole blood and 3 units of fresh frozen plasma.

Figure 1. Image seen after entry of the primary port, confirming hemoperitoneum

Figure 2. Image showing presence of massive hemoperitoneum with blood clots

Figure 3. Image of the ruptured left sided tubal ectopic with arrow pointing towards the site of rupture.

Figure 4. Image showing the process of left salpingectomy.

Figure 5. Image depicting the final view after salpingectomy and suction and irrigation

She was kept in the ICU for observation for 24 hours. There was no requirement for inotropic support throughout the procedure due to proper fluid management and timely administration of blood and blood products. Her post-operative period was uneventful. She was started on orals after 24 hours and discharged on day 3. She came for follow up after one week, on examination vitals were stable and port sites had healed well.

Discussion

Ectopic pregnancy is a common cause of first trimester bleeding. A timely diagnosis and surgical management of a ruptured ectopic is crucial and often life saving. The choice for mode of surgery (laparotomy versus laparoscopy) generally depends upon the hemodynamic condition of the patient and the expertise of the operating surgeon. Evidence has shown that laparoscopy is effective in managing patients with massive hemoperitoneum in experienced surgical hands.[2]
The choice of anesthesia in these patients is always general. Ketamine may prove to be beneficial in these patients due to its sympathomimetic action. It leads to an increase in blood pressure and cardiac output which has a beneficial role during surgery. Besides the positive action on the cardiovascular system, it also has a profound analgesic effect which is effective in pain management.[3]
Carbon dioxide is the most common gas used for creating pneumo-peritoneum. Hypercarbia can have significant effect on the cardiovascular system. The intra-abdominal pressure should be maintained below 10 mm of Hg to avoid the myocardial depressant effect of the gas at higher intra-abdominal pressure. At pressure between 8-10 mm of Hg, it has a sympathomimetic effect which increases both the heart rate and blood pressure which may prove beneficial in such cases.[4]
A proper positioning of the patient during surgery may also prove to be an effective measure. The Trendelenberg position (head low) helps keep the visualization in the surgical field better and avoids pooling of blood in the dependent areas of the pelvis. Besides, this position has been commonly adopted for improving venous return in patients with hypotension to improve cardiac output.
Immediate intervention by an experienced team, timely availability of blood and blood products and vigilant monitoring helped manage this case laparoscopically. The use of harmonic instrument also greatly helped to decrease the operative time. Laparoscopy has also shown to have better fertility outcomes in the future with lesser rate of recurrence for ectopic. [5]

Conclusion

Laparoscopy provides major benefits over conventional laparotomy in terms of faster recovery and ambulation, lesser blood loss and decreased requirement of postoperative analgesia and shorter operative time. Managing patients with massive hemoperitoneum due to ruptured ectopic laparoscopically is a great challenge. In expert hands, this method is safe and can emerge as the method of choice given the benefit of carbon dioxide insufflation in these group of patients.

References
  1. Lehner R, Kucera E, Jirecek S, Egarter C, Husslein P. Ectopic pregnancy. Arch Gynecol Obstet. 2000;263(3):87–92.
  2. Odejinmi F, Sangrithi M, Olowu O. Operative laparoscopy as the mainstay method in management of hemodynamically unstable patients with ectopic pregnancy. J Minim Invasive Gynecol . 2011;18(2):179–83.
  3. Kurdi MS, Theerth KA, Deva RS. Ketamine: Current applications in anesthesia, pain, and critical care. Anesth essays Res. 2014 ;8(3):283–90.
  4. Srivastava A, Niranjan A. Secrets of safe laparoscopic surgery: Anaesthetic and surgical considerations. J Minim Access Surg. 2010;6(4):91-4.
  5. Yao M, Tulandi T. Current status of surgical and nonsurgical management of ectopic pregnancy. Fertil Steril 1997;67(3):421- 33.
Citation

Shah N, Joshi A, Mourya S. Laparoscopic Management Of Ruptured Ectopic Pregnancy With Massive Hemoperitoneum. JPGO 2019. Volume 6 No.1. Available from:https://www.jpgo.org/2019/01/laparoscopic-management-of-ruptured.html

A Rare Presentation Of Bilateral Dermoid Cysts With Unilateral Torsion In An Adolescent Girl

Author Information

Shah N*, Joshi A**, Mourya S**
(* Director , ** Fellow, Vardann Multispeciality Hospital, Mumbai, India.)

Abstract

Bilateral mature cystic teratomas are a rare occurrence especially in young women. Often, they may undergo torsion or rupture leading to complications if not detected timely. We present a case of an adolescent girl diagnosed to have bilateral dermoid cysts with unilateral adnexal torsion, managed laparoscopically.

Introduction

Mature cystic teratomas are common in women in the reproductive age group. They are generally benign, however rarely in 2% cases they may undergo malignant changes.[1] Adnexal torsion occurs in about 3.5% of benign cystic teratomas and is the most common associated complication.[2] Repeated torsion and detorsion events can lead to ischemic changes and in severe cases may lead to permanent damage to ovarian tissue.

Case Report

A 19 year old, unmarried girl presented with complaints of vague pain in the abdomen on and off since one week. There were no other associated symptoms. Her menstrual cycle was regular. On examination vital parameters were normal. On examination of cardiovascular and respiratory system, no abnormality was detected. On per abdomen examination, abdomen was soft with mild tenderness in left iliac region. On per rectal examination, a tender mass was felt in the left adnexa. Her blood investigations were normal. Ultrasound imaging revealed bilateral complex ovarian masses around 5-6 cm with mixed echogenicity suggestive of dermoid cysts with a possibility of torsion. In view of the clinical and radiological findings, a decision for laparoscopic surgery was taken.
She was given general anesthesia and total four laparoscopic ports were introduced. Intraoperative findings were suggestive of bilateral ovarian cysts approximately 6 cm with torsion of the left side adnexa. The left side ovarian cyst appeared congested, edematous and dark with two twists over the infundibulo-pelvic ligament. The torsion was unwound and vascularity was restored in the pedicle, hence only a cystectomy was done. The ovarian tissue was conserved. Similarly, a cystectomy was done on the contralateral side too. Considering the possibility of a mature cystic teratoma, both cysts were retrieved in a surgical glove with aspiration and mechanical morcellation. The contents of the cysts contained tufts of hair and sebaceous material suggestive of a dermoid cyst which was confirmed on histopathology.
There was no spillage of the cyst contents intraperitoneally. Her postoperative course was uneventful and she was discharged on day 2 post surgery.

Figure 1. Image showing bilateral ovarian cysts (yellow arrow) with the uterus in between the two cysts.

Figure 2. Image showing enucleation of the left sided ovarian cyst

Figure 3. Image showing enucleation of the contralateral ovarian cyst.

Discussion

Dermoid cysts are the most common germ cell tumors. They comprise about 20-25 % of all ovarian tumours. They are mostly unilateral however bilateral presentation has been noted in 10-15% cases.[3] Torsion is the most common complication of a dermoid cyst. Some factors predisposing to torsion are a large size of the cyst (generally >5 cm) or elongated infundibulopelvic or utero-ovarian ligaments.
A study reported that, torsion was incidentally diagnosed in about 2-15 % of women who underwent operative treatment for adnexal masses. Most patients are asymptomatic and diagnosis is often ascertained by radiological modalities. Ultrasonography has a low sensitivity for diagnoses ranging from 40-75 %.[4] Doppler studies reveal blood flow patterns and may play a role in screening but is not diagnostic. However, owing to direct visualization, laparoscopy remains the best method for diagnosing as well treating.
Laparoscopy, being minimally invasive offers numerous benefits over conventional laparotomy. However, substantial surgical skill is required to ensure that the cyst remains unruptured during retrieval. Oophorectomy was the treatment of choice in the past in cases with a large cyst, those with torsion or where occult malignancy was suspected. However, preservation of ovarian tissue is extremely critical in young patients. Conservation of the ovarian tissue despite visible necrosis in cases of torsion has been recently recommended.[5] Multiple studies have reported that vascularity is regained in the apparently necrosed appearing ovaries on detorsion, the color changes and edema however resolves subsequently over a period of time. The recurrence rate after surgery remains 4.2 %.[6] Hence a close surveillance and follow up is necessary which has been enabled by the advent of better imaging modalities over the past few decades.

Conclusion

Dermoid cysts are often asymptomatic or may present with vague symptoms. Bilateral large cysts have a tendency to undergo torsion. Timely detection and management of these cysts is essential especially in young adolescent to preserve ovarian function and fertility.

References
  1. Patni R. Squamous cell carcinoma arising in mature cystic teratoma of ovary. J Midlife Health. 2014;5(4):195–7.
  2. Oelsner G, Shashar D. Adnexal torsion. Clin Obstet Gynecol. 2006;49(3):459–63.
  3. Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: Tumor types and imaging characteristics. Radio Graphics. 2001;21(2):475–90.
  4. Mashiach R, Melamed N, Gilad N, Ben-Shitrit G, Meizner I. Sonographic diagnosis of ovarian torsion:accuracy and predictive factors. J Ultrasound Med. 2011;30(9):1205.
  5. Spinelli C , Pucci V , Buti I , Liserre J , Messineo A , Bianco F, et al. The role of tumor markers in the surgical approach of ovarian masses in pediatric age: A 10-year study and a literature review. Ann Surg Oncol. 2012;19(6):1766–73.
  6. Pepe F, Panella M, Pepe G, Panella P, Pennisi F, Arikian S. Dermoid cysts of the ovary. Eur J Gynaecol Oncol. 1986;7(3):186–191.
Citation

Shah N, Joshi A, Mourya S. A Rare Presentation Of Bilateral Dermoid Cysts With Unilateral Torsion In An Adolescent Girl. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/a-rare-presentation-of-bilateral.html

Successful Pregnancy In A Factor X Deficient Woman

Author Information

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

Abstract

Out of many coagulation disorders, factor X deficiency is one of the rarest disorders. Positive pregnancy outcomes in these patients have been rarely reported. Here we are presenting a case report of such a patient who throughout her pregnancy meticulously followed up with hematology and obstetrics department of our hospital.

Introduction

Factor X deficiency is a rare disorder, with autosomal recessive mode of inheritance. It has an incidence of 1 in 1,000,000.[1] It presents with excessive bleeding if levels of factor X fall below 10%. Homozygus individuals can have severe bleeding episodes whereas heterozygus individuals, though usually asymptomatic, can also present with varying degrees of bleeding. Women with factor X deficiency (FXD) who want to become pregnant face uncertain risks to themselves and to an unborn infant from hemorrhagic complications during pregnancy and parturition. This is a case report of our patient, a 27 years old, primigravida, married since 2.5 years who was referred to our OPD at 11 weeks of gestation with a rare coagulation disorder of factor X deficiency.

Case Report

A primigravida, 27 years old, at 11 weeks of gestation was referred to our ANC OPD for further management of her pregnancy. At 11 years of age she had attained her menarche. Due to complaints of menorrhagia not responding to medical management, she was investigated for coagulation disorders and was diagnosed with Factor X deficiency. Since then she had been taking combined oral contraceptive pills (OCP’s) to avoid menses. Intermittently when she tried to stop OCP’s she had heavy bleeding during menses and hence she took them everyday thereafter. She was also advised to get her husband evaluated for any bleeding disorder so as to plan pregnancy in future. Accordingly her husband was evaluated and there was no evidence of any hematological disorder.  She stopped consuming the pills when she wanted to conceive.

We referred her to hematology department, where she was counseled regarding the high risk related to pregnancy, increased risk of spontaneous abortions, need for monitoring of any bleeding episode and increased risk of morbidity and mortality related to bleeding.
She had regular follow ups with the hematologist and the obstetrician. At 24 weeks of gestation she had an episode of hematuria for which she came to our emergency services department. No active intervention was required and bleeding resolved on its own. Near term, she was advised to arrange for approximately 70 units of fresh frozen plasma (FFPs).
At the onset of labor, she came to our receiving room with term gestation. She was admitted. Hemoglobin on admission was 11.9 gm %. Availability of 70 FFPs were confirmed. Vital parameters were monitored. When she entered the active phase of labor, as per hematologist advice 7 units of FFPs (at the rate of 15 ml/kg) were transfused. At the onset of second stage of labor 4 more units of FFPs were transfused. Due to non-descent of the fetal head for an hour in the second stage of labor, an emergency lower segment cesarean section was performed. A healthy male baby was born. Neonatologist’s evaluated the neonate and found no evidence of any bleeding in the neonate. Neonatal evaluation for bleeding disorders was deferred for 6 months. Total blood loss was estimated to be 700 ml. Post-operative hemoglobin was 9.6 gm%. As per hematologist advice 4 more FFP’s were transfused after 12 hours of delivery. Vital parameters remained stable throughout the post-operative period. No abnormal bleeding was noted. No postpartum hemorrhage occurred. As per hematologist advice she was again transfused 4 units of FFP’s on day 3 and another 4 on day 6. Her factor 10 levels done on day 3 were >25%. She was discharged on day 7. She was advised double barrier method for contraception and later on resumption of hormonal contraception.

Discussion

FXD is one of the rare disorders of coagulation. Factor X gets converted into factor Xa in the final cascade of coagulation pathway.[2] FXD can be divided into two types, Type 1, a quantitative defect and Type 2, a qualitative defect with near normal antigen levels but reduced FX activity.
As FXD, is extremely rare, not many successful pregnancies have been reported in literature. Complications like miscarriage, abruptio placentae, premature labor, fetal death and bleeding episodes in the baby are commonly encountered.
Fertility rate among women with FXD is unknown. Most women with factor X deficiency are encountered with complains like menorrhagia, prolonged menses, hemorrhagic ovarian cyst, post coital bleeding. In case of severe FXD, prolonged menorrhagia and post coital bleeding reduce the frequency of coitus thus hindering their fertility.[3]
Vijakupar reported that five of the eight women with severe FXD had refractory menorrhagia making conception difficult. One patient required hysterectomy, the other two couldn’t be weaned off OCPs, one did conceive but had an abortion and one was declared infertile.[4]
Mamopoulos et al has described pregnancy in a 34 year old patient with FXD.[1] At 40 weeks, she was admitted with false labor and was transfused FFPs with a loading dose of 20 ml/kg of body weight. The same dose was repeated after 4 days when she spontaneously went into labor. She underwent an emergency LSCS due to a non reassuring cardiotocogram. A healthy baby delivered and no bleeding problems were encountered during the operation. Two more units of FFP were administered to maintain FX levels above 20%.
Another case report published in 1994, describes the outcomes of 4 pregnancies in a patient with factor X deficiency.[5] In her first 2 pregnancies she was transfused FFPs at the time of delivery in view of acute bleeding episodes. She delivered at 21 and 25 weeks of gestation respectively and babies died in their neonatal period due to severe prematurity. In the next 2 pregnancies she was transfused earlier in pregnancy and the outcome of both these pregnancies was good, one being at 34 weeks and the second at 32 weeks.

Romagnolo et al reported a pregnancy in a 30 year old, nulliparus, homozygus patient, where an elective cesarean section was planned.[6] Prior to cesarean section, she was given an intravenous concentration of Vitamin K dependent factors, one hour prior to surgery to prevent post partum hemorrhage. She was given the complex for another 4 days after her delivery, but her prothrombin time value fell to 7% of the normal, the next day. Teixeira published a case report about a 33 year old nulliparus patient.[7] An ultrasound done at approximately 8 weeks of gestation showed detachment of the smooth chorion. It regressed completely with bed rest and 1000 IU of prothrombin complex concentrate. An elective cesarean section was done at 38 weeks. Prophylaxis of bleeding using prothrombin complex concentrate was given pre-operatively and for three consecutive days postpartum.The post operative period was uneventful. FXD is an autosomal recessive disorder and follows Mendelian inheritance patterns. Majority cases of FXD are caused by missense mutations, duplications and partial deletions.[8] In pregnancy, there is a modest physiological rise of factor X. Also there is an increase in FVIII, VWF and fibrinogen levels and overall there is a prothrombotic state in normal pregnancy. Circulating FX levels rise in pregnant states to roughly 1.3 times the non-pregnant values and return to baseline post delivery.[9,10] For women with very low baseline levels of FX activity, this rise may be only a few points or none at all. The risk for preterm labor appears to be 2.5 times higher in FXD women.[11] Amongst 9 of the 24 pregnancies reporting preterm deliveries, four died and three were extremely premature. In advanced countries, due to easy availability of factor X concentrates, prophylaxis as well as treatment of any peripartum bleeding can be effectively managed, whereas in developing countries due to difficulty in getting factor X, FFP is used for the same.

Conclusion

Though little is known about the pregnancy outcomes in factor X deficient states due to the rarity of the disorder itself, however with early antenatal registration at tertiary care centers, regular follow ups, early anticipation of complications and prophylactic measures, a good pregnancy outcome can be expected in these patients.

References
  1. Mamopoulos A, Vakalopoulou S, Lefkou E, Fileli A, Garipidou V, Mavromatidis G et al. Pregnancy in a patient with severe factor X deficiency. Haemophilia. 2009;15(6):1351-1353.
  2. Menegatti M, Peyvandi F. Factor X Deficiency. Seminars in Thrombosis and Hemostasis. 2009;35(04):407-415.
  3. Nance D, Josephson NC, Paulyson-Nunez K, James AH. Factor X deficiency and pregnancy: preconception counselling and therapeutic options. Haemophilia. 2012;18(3):e277-e285.
  4. Vijapurkar M, Mota L, Shetty S, Ghosh K. Menorrhagia and reproductive health in rare bleeding disorders: a study from the Indian subcontinent. Haemophilia. 2009;15(1):199-202.
  5. Kumar M, Mehta P. Congenital coagulopathies and pregnancy: Report of four pregnancies in a factor X-deficient woman. American Journal of Hematology. 1994;46(3):241-244.
  6. Romagnolo C, Burati S, Ciafonni S, Fattori E, Franchi M, Zanon E et al. Severe factor X deficiency in pregnancy: case report and review of literature. Hemophilia. 2004;10(5):665-8
  7. Teixeira PS, Oliveira PS, Guerra JCC, Hamerschlak N, Colombini MP, Kalil R. Factor X deficiency and pregnancy: case report and counselling. Haemophilia. 2012;18(1):e11-e12.
  8. Uprichard J, Perry DJ. Factor X deficiency. Blood Reviews. 2002;16(2):97-110.
  9. Condie RG. A Serial Study Of Coagulation Factors XII, XI And X In Plasma In Normal Pregnancy And In Pregnancy Complicated By Pre-Eclampsia. BJOG: An International Journal of Obstetrics and Gynaecology. 1976;83(8):636-639.
  10. Szecsi PB, Jørgensen M, Klajnbard A, Andersen MR, Colov NP, Stender S. Haemostatic reference intervals in pregnancy. Thrombosis and Haemostasis. 2010;103(04):718-727.
  11. Martin JA, Hamilton BE, Osterman MJ, Driscoll AK, Mathews TJ.Births: Final Data for 2015. Natl Vital Stat Rep. 2017;66(1):1.
Citation

Saxena A, Pardeshi S, Gupta AS. Successful Pregnancy In a Factor X Deficient Woman. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/successful-pregnancy-in-factor-x.html

A Rare Case Of Portal Vein Cavernoma In An Antenatal Patient

Author Information

Malani K*, Samant P**, Thakur H S***, Bharthi S*.
(* Junior Resident, ** Academic Professor , *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Portal vein cavernoma is a rare disease which results from extrahepatic portal vein thrombosis and development of collateral venous circulation. An antenatal patient with this disease poses a challenge to the obstetrician because physiological hemodynamic changes associated with pregnancy that are needed to meet demands of the growing fetus may worsen the condition of the patient and put her at risk of life threatening complications. Here we present a case of successful vaginal delivery of a patient with portal vein cavernoma and chronic thrombocytopenia.

Introduction

Portal cavernoma described first as phenomenon by Balfour and Stewart is thrombosis and varicose dilatation of portal vein leading to splenomegaly and ascites. The causes of which can be divided into two categories; local (70%) and systemic(30%).[1] The most common presenting symptom is variceal bleeding followed by thrombosis, abdominal pain, jaundice, and splenomegaly. Pregnancy being a hypervolumeic state causes increase in portal flow, which in turn increases portal pressure, which gets transmitted to collateral circulation and leads to variceal bleeding.[2] We are reporting this case for its rarity and successful outcome of pregnancy.

Case Report

A 37 year old G5P2L2MTP1SA2 previous FTND was admitted at 24 weeks of gestation with chief complaints of giddiness, anorexia, generalized weakness and easy fatigability. She was investigated for the same, platelet count of 14,000/mm3 was detected on her routine investigations. She was transfused two units of random donor platelets. She was advised complete hemogram, bone marrow aspiration and bone marrow biopsy if platelet count was  persistently below 30,000/mm3 by the hematologist. Bone marrow aspiration was done. It reported a normocellular marrow with increased megakaryocytes. Bone Marrow Biopsy detected a normocellular marrow with hematopoietic cells of all three series, consistent with megakaryocytic thrombocytopenia.
She was started on Injection methylprednisolone 1 g O.D. for three days followed by oral prednisolone and tapering of the dose was done every week by 5mg.
On obstetric ultrasonagraphy (USG) incidental finding of portal cavernoma of 3.4x4.5 cm was noted. Gastroenterologist was consulted and an upper G.I. scopy was performed. It diagnosed severe portal hypertension with gastropathy.  Liver function tests were within normal limits. She had complaint of small joint pain for which β2 glycoprotein, ACLA, APLA IgG and IgM, ANA, anti–dsDNA were tested. Results were negative for each one of them.
She was transfused 4 units platelets in addition to previous two and was discharged at platelet count of more than 50,000/mm3. She was advised to follow up regularly with the hematologist, gastroenterologist and obstetrician.
USG for uteroplacental Doppler was done at 35 weeks of gestation. It was suggestive of fetoplacental insufficiency with oligohydrominos and increased S/D ratio. USG Abdomen was repeated. It showed an increased size of cavernoma of 7x6 cm, altered liver echotexture and mild splenomegaly, but there was no evidence of mesenteric vein thrombosis.
On examination she was clinically asymptomatic, hemodynamically stable with pulse of 88/min, B.P. Of 110/80 mm of Hg, and respiratory rate of 18/min. Obstetric examination indicated intrauterine growth retardation. FHS were within normal limits, and regular.
Her hemoglobin was 9.4 g/dl, platelet count was 40,000/mm3, and liver function tests were within normal limits.
Hematologist reviewed her and advised to continue oral prednisolone. They opined that there was no need of anticoagulation as she was already thrombocytopenic and did not have any history of prior anticoagulation. Gastroenterologist advised to monitor LFT, RFT and INR.
The possibility that large size of cavernoma (7x6 cm) at the time of Valsalva manuever in active labour may lead to rupture  was informed by the fact that increase in intra-cavitatory  pressure of caveroma will be redistributed to collaterals and hence the necessity of mode of termination of pregnancy only  via caesarean section was ruled out.
The possibility of rupture of the large sized cavernoma (7x 6 cm) at the time of Valsalva maneuver in active labor was considered but the fact that increase in intra-cavitatory pressure of the caveroma would be redistributed to its collaterals thus preventing its rupture was concluded and hence the necessity of mode of termination of pregnancy only via cesarean section was ruled out. Alternate day NST done for fetal well being was reactive. In view of platelets being in the range of 40,000-45,000 /mm3, she was started on Injection Methylprednisolone 1 gm intravenous daily for 3 days. Complete hemogram showed an increase in the platelet count to 80,000 /mm3. She went into spontaneous labor and delivered vaginally a male child of 2.32 kg with an APGAR score of 9/10. She was transfused 4 units of random donor platelets intrapartum.
From the day of safe confinement till discharge as LFT’s  remained within normal limits the gastroenterologist did not advise any additional intervention. Platelets at the time of discharge was 1.17 lacs/mm3. She was discharged on oral prednisolone on day 5 of normal delivery.

Discusssion

Portal vein cavernoma is a rare disease resulting from extra hepatic portal vein thrombosis. Due to cessation of portal venous blood flow, liver doesn’t get two thirds of its blood supply which is compensated by development of collateral blood vessels.[3]
Cavernous transformation from portal vein thrombosis requires first 6 to 20 days after acute thrombosis of portal vein and completes within 3 to 5 weeks. Amusingly this occurs much more frequently in patients without an underlying liver disease and leads to portal hypertension because collaterals are not able to handle splenic and mesenteric blood flow.[4]
Conversely, liver cirrhosis has rare chance of cavernoma formation because stasis of portal venous flow prevents formation of collaterals around the portal venous thrombus.
Common factors leading to cavernoma formation are liver cirrhosis, portal hypertension, prothrombotic tendencies like myeloproliferative disorders, antiphospholipid antibodies, anticardiolipin antibodies, thrombophilias, pregnancy, post partum period, and use of oral contraceptive pills.[5]

Pregnancy being a prothrombotic condition predisposes to development of portal vein thrombosis and eventually to cavernoma formation.
Acute portal vein thrombosis presents with abdominal pain, nausea, fever, mostly reflecting the consequences of mesenteric venous thrombosis and resulting bowel ischemia. Chronic portal venous thrombosis presents with esophageal or gastric varices. Variceal bleeding may not be as common as believed because the increased blood volume flows within uteroplacental circulation without affecting portal system and cavernoma significantly. Hyperspleenism  and consequent pancytopenia, severe anemia, hepatic decompensation leading to progressive hepatic and renal failure, hepatic encephalopathy, splenic artery aneurysm rupture, ascites and post partum hemorrhage can be seen in chronic portal venous thrombosis.[6]
Treatment is either surgical or medical depending on symptoms.  American association for liver disease (AASLD) recommends screening endoscopy in second trimester as that is the time of maximal increase in portal pressure in these patients.[7]
For esophageal varices shunts and band ligation can be done. Medical treatment includes use of beta blockers to decrease flow in the portal circulation. Anticoagulation should be started if diagnosis is made early in pregnancy as it causes recanalization in 80% of cases. Maternal as well as fetal outcome is better with anticoagulation, but should be stopped before term to reduce the chances of excessive bleeding.[8]

Conclusion

Pregnancy with portal cavernoma is a rare condition with very few reported cases. Regarding pregnancy outcome, if diagnosed and treated at a tertiary care hospital, both the fetal and maternal outcomes are good.[8] There is no role of elective termination of pregnancy nor the need to terminate the pregnancy via cesarean section. Cesarean section is indicated only for obstetric or fetal indication. Second stage of labor does not increase chances of gastrointestinal bleeding. In patients with high chances of variceal bleeding, second stage can be cut short with operative vaginal delivery.[9] Pregnancy is not a contraindication, however, periconceptional counseling should be done for such patients and maternal and fetal risks, consequences and complications should be explained.[7]

References
  1. Bayraktar Y, Harmanci O. Etiology and consequences of thrombosis in abdominal vessels. World J Gastroenterol. 2006;12(8):1165–1174.
  2. 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. Journal of Hepatology. 2012;57(6):1214-1219.
  3. Aggarwal N, Chopra S, Raveendran A, Suri V, Dhiman RK, Chawla YK. Extra hepatic portal vein obstruction and pregnancy outcome: largest reported experience.J Obstet Gynaecol Res. 2011;37(6):575-80.
  4. Sogaard KK, Astrup LB, Vilstrup H, Gronbaek H. Portal vein thrombosis; risk factors, clinical presentation and treatment. BMC Gastroenterol. 2007;7:34.
  5. Ponziani FR, Zocco MA, Campanale C, Rinninella E, Tortora A,  Di Maurizio L, et al. Portal vein thrombosis: Insight into physiopathology, diagnosis, and treatment. World J Gastroenterol. 2010; 16(2): 143–155.
  6. Northup PG, Sundaram V, Fallon MB, Reddy KR, Balogun RA, Sanyal AJ, et al. Hypercoagulation and thrombophilia in liver disease. J Thromb Haemost. 2008;6(1):2-9.
  7. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey W. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology. 2007;46(3):922-38.
  8. Ghike S, Gawande M, Mitra K, Jain S, Senani S, Ratnaparkhi C. A rare case of portal vein cavernoma with pregnancy. J South Asian Feder Obst Gynae. 2013;5(1):37-39
  9. 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

Malani K, Samant PY, Thakur HS, Bharthi S. A Rare Case Of Portal Vein Cavernoma In An Antenatal Patient. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/a-rare-case-of-portal-vein-cavernoma-in.html

Broad Ligament Ectopic Pregnancy Diagnosed And Managed Laparoscopically

Author Information

Shah NH*, Mourya S**, Paranjpe S***.
(* Consulting Gynecologist/Obstetrician & Hon. Endosopic Surgeon Wadia Hospital & Railway Hospital, ** Consulting Gynecologist/Obstetrician, *** Director: Velankar Hospital & Paranjpe Maternity Home, Mumbai, India.)

Abstract
Ectopic pregnancy causes significant maternal morbidity and also maternal mortality. Broad ligament pregnancy is very rare type but has high maternal mortality. Diagnosis of this condition is seldom done with imaging techniques. Definitive diagnosis is usually done intraoperatively either during  exploratory laparotomy or by minimally invasive surgeries especially in a young stable patient. We are presenting a case of a 28 year old female with clinical suspicion of right sided tubal ectopic pregnancy. Diagnosis of right broad ligament pregnancy was done during laparoscopy.

Introduction

Ectopic pregnancy means implantation of the fertilized ovum outside the uterine body. Most of the ectopic pregnancies occurs in the fallopian tube. Abdominal pregnancy accounts for 1% of which broad ligament pregnancy is very rare.[1] Here, we are presenting a case of broad ligament ectopic pregnancy, in which laparoscopy played an  important role in diagnosis and its subsequent management.

Case Report

A 28 year old female Gravida 2 Para 1 Living 1 presented to emergency ward with complaints of pain in abdomen since 2 days, and one episode of spotting per vaginum. She had 2 months of amenorrhea. Her urine pregnancy test was positive. She had previous vaginal delivery. There was no previous history of surgery or significant medical history. No other significant past history was obtained.
On general examination, she was conscious, afebrile. Her blood pressure was 110/60 mm of Hg, and pulse was 86/min. Mild tenderness in right iliac fossa was present on abdominal examination. Per speculum examination revealed minimal blood discharge through the os. On vaginal examination  ill-defined mass in the right fornix was felt with positive cervical motion tenderness. Size of the uterus could not be assessed due to tenderness.
Transvaginal ultrasound (TVS) revealed empty uterus with thickened endometrium. There was an ill-defined mass measuring 3*2 cm on the right side of the uterus suggestive of an unruptured right tubal ectopic pregnancy. There was no free fluid collected in the pouch of douglas. Hematological examination showed hemoglobin level of 8.8 gm/dl and white blood cell count of 12,500/ mm3. Serum β HCG measured 26,198 mIU/ml.
From this clinical and ultrasonographic findings, diagnosis of right ectopic pregnancy was made but the exact location was uncertain. As she was hemodynamically stable, a decision for laparoscopy was taken for definitive diagnosis and management. A 3*2 cm irregular mass in the right broad ligament most probably ectopic pregnancy was found with minimal blood in the POD. Uterus, both the fallopian tube and ovary appeared normal.


Figure 1. Mass seen protruding from the right broad ligament.


Figure 2. Distended tube seen above the mass.

Figure 3. Removal of the mass.

Figure 4. View after removal of the complete mass.

Figure 5. Final view after giving wash.

The Broad ligament was opened and excision of mass was done. Hemostasis was achieved by bipolar cautery and bleeding could be easily controlled. Also, right salpingectomy was done. The tissue was completely removed and sent for histopathological examination. Ureter peristalsis was confirmed again after giving wash and right ureter was visualized.  Histopathology report confirmed the tissue as products of conception. Postoperative course was uneventful and she was discharged on 4th postoperative day.

Discussion
Ectopic pregnancy is a type of pregnancy that occurs outside the normal uterine cavity. Fallopian tube is the most common site of ectopic pregnancy and abdominal pregnancy accounts for 1%.[1] It can occur in any part but it is most common in POD and rare in the broad ligament.
Broad ligament ectopic pregnancy is also known as interligamentous pregnancy. In broad ligament pregnancy the fetus or gestation sac develop within the leaves of the broad ligament. The incidence of broad ligament pregnancy is 1 in 189,300 pregnancies.[2] Broad ligament pregnancy is very rare, and maternal mortality rate is as high as 20%.[1] It can be due to primary implantation of the zygote on the broad ligament or followed by secondary implantation in the fallopian tube, ovary or peritoneal surface. In secondary broad ligament pregnancy, ovum first implants in the fallopian tube. Due to fimbrial abortion or rupture of the fallopian tube it gets implanted in the broad ligament.
Risk factors include a history of pelvic inflammatory disease, previous ectopic pregnancy, previous salpingectomy, abdominal tuberculosis, endometriosis and use of assisted reproductive techniques. There was no risk factor in this case.
There are no specific clinical features for this rare form of ectopic pregnancy to enable diagnosis to be made preoperatively. The clinical presentation of broad ligament ectopic pregnancy is highly variable and can range from asymptomatic early ectopic pregnancy to rupture in labor at term.  However, dull lower abdominal pain during early pregnancy and vaginal bleeding are common presentations. Pain has been attributed to placental separation, tearing of broad ligament and small peritoneal hemorrhage’s. Vaginal bleeding occurs due to breakdown of decidual cast. They usually present in the first trimester with pain and vaginal bleeding but live birth is also reported in literature.
In this case, she presented with severe abdominal pain with guarding and rigidity and no vaginal bleeding, raising the possible differential diagnosis of ruptured ectopic pregnancy, torsion of ovarian cyst or any surgical cause of acute abdomen. As her urine pregnancy test was positive, so the provisional diagnosis of ectopic pregnancy was made.
Ultrasound is usually the preferred investigation. Ectopic pregnancy can be diagnosed with the help of high resolution transvaginal sonography combined with serum Beta HCG with sensitivity of 93% and a specificity of 99%.[3] Broad ligament pregnancy is very rarely diagnosed prior to surgery. Definitive diagnosis is usually intraoperative.
Conservative management or medical management is not recommended for broad ligament ectopic if the diagnosis is certain. Usually exploratory laparotomy is considered as the choice of surgical management as the patients are unstable. Our patient was managed laparoscopically as she was stable and it was a small broad ligament pregnancy. Laparoscopy played important role not only in diagnosis but also in management of the case in the same setting. Laparoscopy also has advantages of lower morbidity, mortality, less blood loss, and faster recovery.

Conclusion

Being a rare condition, diagnosis of it is challenging. One needs a high index of suspicion for such a rare diagnosis. Laparoscopy should be considered for diagnosis and management whenever in doubt in appropriately selected patient.

References
  1. Sharma S, Pathak N, Goraya SPS, Mohan P. Broad ligament ectopic pregnancy. Sri Lanka J Obstet Gynaecol 2011;33:60–2.
  2. Sheethal CH, Powar A. Full term viable secondary broad ligament pregnancy – A rare case. Case Rep Womens Health. 2016;13:4-5
  3. Nayar J, Nair SS. Broad Ligament Pregnancy - Success Story of a Laparoscopically Managed Case. J Clin Diagn Res. 2016;10(7):QD04-5.
Citation

Shah NH, Mourya S, Paranjpe S. Broad Ligament Ectopic Pregnancy Diagnosed And Managed Laparoscopically. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/broad-ligament-ectopic-pregnancy.html

Remembering Past Greats: Kermit Krantz

Author Information

Prasad M
(*Assistant Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai)

Kermit Edward Krantz (1923 - 2007) was an outstanding American obstetrician and gynecologist. Born in Illinois into a family of six siblings, and himself being one of the twins, he lost his parents at the young age of 13. He is believed to have worked multiple jobs simultaneously, and self-funded his education. One of his jobs as a teenager was as a helper in the anatomy museum of the university he would go on to study.[1]

He went on to the Northwestern university, where he completed a Masters in Science and in Anatomy. His early interests in anatomy formed the foundation to his later successes in gynecological surgery. After serving clinical residency in Cornell, he joined the university hospital at Arkansas, where he became the head of the department of obstetrics/gynecology, at a rather young age of 36. One of his significant contributions was the modernization of the maternity ward, including making specific divisions for patients who required specific care. He was also instrumental in popularizing laser based procedures in gynecology and did substantial research on premalignant lesions of the cervix.[2] He also served as dean of the medical school at Arkansas and held various top posts at the American College of Obstetricians and Gynecologists. 

His pioneering achievement was the procedure of abdominal urethral suspension. Along with his colleagues, Marshall and Marchietti, he held patent to the procedure which was popularly referred to as the MMK procedure. It was first published in 1949,[3] and it has been hailed as a landmark article.[4]  Many urogynecological surgeons adopted this procedure, and for three decades, its utility was repeatedly proven.[5-8] The MMK procedure was the result of sound medical scientific collaboration. The anatomical analysis was provided by Krantz and the physiological basis was provided by the other two collaborators.  Krantz went on to perform around 5000 of these operations, with a long term success rate of more than 75 %.

Throughout his career, he was a champion of equal rights for patients and mitigation of discrimination based on race and religion, and had been honored extensively. Perhaps the most inspiring honour was that the anatomy museum in Northwestern university where he started of a teenaged helper boy, went on to be renamed after him.[9] The life and achievements of Prof Krantz shall remain an inspiration for generations of gynecologists to come.

References
  1. Kermit E Krantz. Available from https://en.wikipedia.org/wiki/Kermit_E._Krantz
  2. Biographies. In O’Dowd MJ, Philipp EE, editors. The History of Obstetrics and Gynecology. 1st ed. Lancs: Parthenon Publishing Group 2000; pp. 633-4
  3. Marshall VF, Marchetti AA, Krantz KE. The correction of stress incontinence by simple vesicourethral suspension. Surg Gynecol Obstet. 1949 Apr;88(4):509-18.
  4. Marshall VF, Marchetti AA, Krantz KE. The correction of stress incontinence by simple vesicourethral suspension. 1949. J Urol. 2002 Oct;168(4 Pt 1):1326-31.
  5. McDuffie RW Jr, Litin RB, Blundon KE. Urethrovesical suspension (Marshall-Marchetti-Krantz). Experience with 204 cases. Am J Surg. 1981 Feb;141(2):297-8.
  6. Hegarty PK, Power PC, O'Brien MF, Bredin HC. Longevity of the Marshall-Marchetti-Krantz procedure. Ann Chir Gynaecol. 2001;90(4):286-9.
  7. Strittmatter HJ, Neises M, Wischnik A, Melchert F. [Marshall-Marchetti-Krantz operation or fasciaplasty in therapy of recurrent urinary incontinence in women]. [Article in German] Geburtshilfe Frauenheilkd. 1993 Sep;53(9):630-4.
  8. Kjer JJ, Hebjørn S, Jaszczak P. A follow-up study of the Marshall-Marchetti-Krantz vesicourethral operation for female incontinence. Zentralbl Gynakol. 1983;105(22):1468-71.
  9. Obituaries. Kermit E. Krantz. Available from: https://web.archive.org/web/20070927201324/http://www.legacy.com/KansasCity/DeathNotices.asp?Page=LifeStory&PersonID=91819956
Citation

Prasad M. Remembering Past Greats: Kermit Krantz. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/remembering-past-greats-kermit-krantz.html