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Volume 5 Number 10

Editorial

Parulekar SV

There is a new kid on the contraceptive block - Annovera - a long-acting contraceptive system developed by Population Council and approved by the U.S. FDA in August 2018. It is the sixth one developed by the Council, the previous ones being ParaGard (a copper intrauterine device), Mirena (an intrauterine progestin releasing device), Norplant and Jadelle (contraceptive implants) and Progering (a vaginal ring for lactating women). It does not mean the previous ones were not good enough or caused serious problems. A medicated device makes changes in body physiology and like any drug, can be associated with adverse reactions. Still newer contraceptives keep getting developed, in order to make greater choices available to couples and hoping to make more effective, better tolerated and safer contraceptives. The needs and choices of different women differ and a wide choice helps ensure that contraceptive coverage of population is wider.

Annovera is a soft, flexible and reusable silicone ring measuring 5.715 cm in diameter containing ethinyl estradiol and segesterone acetate. While ethinyl estradiol is an old and well known estrogen, segesterone acetate is quite new. It is a synthetic 19-norprogesterone derivative progestin without a CH3 group radical in position 6. Its progestational activity is very high, 100 times that of Progesterone. It binds selectively to progesterone receptors and has no androgenic, estrogenic or anabolic activity.

Annovera is inserted and removed by the woman who is using it. This may be a detractor, when handling of the genitals is disliked by the user. When she is comfortable with the insertion and removal process, it is totally under her control, which is important in keeping the pregnancy rates low, especially when the male partner cannot be relied on to use a condom consistently. It is kept in the vagina continuously for 3 weeks, followed by its removal for one week. It need not be kept in a refrigerator, which is important in resource poor populations. It gives protection for one year (thirteen cycles), which is quite useful because the user does not have to visit the provider again and again, especially when the family members of the woman may not be very supportive. But it does need training for its use, which may be difficult to arrange in populations with few healthcare providers. US FDA approved its use after studying data of 17 clinical trials, which included 2 phase 3 efficacy and safety studies. Its success rate was 97.3%. Satisfaction rate was 89%. It was found to be easy to use. The device was not felt by most of the couples during its use. No serious effects due to over dosage were reported. In studies on rats over 2 years, no tumors developed with its use, while mice showed an increased incidence of adenocarcinoma and lobular hyperplasia in the breast with a supratherapeutic dose (30 mg/kg/day). No mutagenic or clastogenic effect was seen with the use of segesterone acetate. The risk of serious cardiovascular adverse events is as with other estrogen-progestin contraceptives. Segestrone acetate's metabolism can be increased or decreased when combined with different drugs, which may have an effect on its contraceptive efficacy. While Annovera is available for use in USA, it is not yet available in many countries. It may be a disadvantage, but one may find solace in the thought that a post marketing surveillance is being carried out in another country and when it is found to be safe, women may embrace its use freely.

Resection Of A Large Subserosal Leiomyoma By Minilaparotomy

Author Information                                                                                                     Innovation

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

Abstract

The term leiomyoma defines a benign tumour composing mainly of uterine smooth muscle cells with varying amount of fibrous connective tissue. They are the most common tumours of uterus and female pelvis. There are mainly of 3 types: intramural (arising in the myometrium), submucosal or subserosal. Here we present an innovative method of myomectomy of a large symptomatic subserosal leiomyoma in an unmarried woman by a minilaparotomy incision and morcellation.

Introduction

All leiomyomas arise from the myometrium to begin with i.e. they are intramural/interstitial. As they grow, they could extend inwards towards the endometrial cavity or outwards towards the peritoneal cavity and give rise to submucosal or subserosal leiomyomas respectively. (1) Large symptomatic leiomyomas warrant surgical excision.

Case Presentation

A 35 years old, unmarried nulligravida, presented to the outpatient clinic with complaints of persistent dull aching lower abdominal pain for 2 years and a palpable mass per abdomen for 1 year. The pain was progressively increasing in intensity. There were no aggravating or relieving factors for the pain. There was no history of menstrual abnormalities. The patient had visited a private hospital 1 year back and a transabdominal ultrasonography (USG) was done, which showed a 7x 8 cm sized subserosal leiomyoma located at the fundus. She was advised conservative management with non steroidal antiinflammatory agents for pain relief and serial USG for follow-up. However, her symptoms continued to worsen. Hence she was referred to us. On examination, her general and systemic examination showed no abnormality. Abdominal examination showed a 24 weeks' size mobile, nontender mass. There was no tenderness, guarding or rigidity. Per speculum and per vaginal examinations were not done considering her unmarried status. A rectal examination showed the cervix was in continuity with the mass. USG was done which showed a 12 x 13 x 11 cm sized pedunculated subserosal fundal leiomyoma. Bilateral adnexal structures were normal. There was no evidence of hydroureter or hydronephrosis formation. Investigations for fitness for anesthesia showed normal results. Myomectomy was planned through a mini laparotomy incision. A minilaparotomy was done through a 5 cm long infra-umbilical midline vertical incision. Intraoperative findings showed a 15 x 12 cm large pedunculated subserosal leiomyoma, connected to the uterine fundus by a broad pedicle with leash of vessels within it. Bilateral fallopian tubes and ovaries were normal. There were no adhesions between the leiomyoma and the omentum or bowel, and no torsion of the pedicle. A rolled up gauze mop was passed around the pedicle and traction was made on the two ends of the mop, so that the pedicle was drawn forwards and fixed.  The leiomyoma was grasped with a tenaculum near the pedicle. The pedicle was held with three Allis' forceps applied close to the uterine serosa, and divided above the grips of the forceps. The Allis' forceps were replaced by sutures of No. 1-0 polyglactin in two layers. Hemostasis achieved. Thus the leiomyoma was separated from the uterus and its blood supply was cut off, so that there would not be any blood loss during morcellation of the leiomyoma. Morcellation procedure was performed with a scalpel using wedge resection technique to reduce the size of the leiomyoma so that it could fit through the smaller incision. Fifteen wedges of different sizes were resected to facilitate the delivery of the leiomyoma through the small abdominal incision. During each wedge resection, the leiomyoma was pulled closer to the skin with the help of a tenaculum and long Allis’ forceps, so that a wedge could be cut out only from the surface which was visible through the incision and no tissue inadvertently spilled into the peritoneal cavity. This also made the use of any containment retrieval bag for the specimen unnecessary. The patient made an uneventful recovery. The leiomyoma weighed 625 g. Its histopathological confirmed the diagnosis of a leiomyoma.


Figure 1. Findings at the time of the laparotomy: L - leiomyoma, U - uterus, P - pedicle of the leiomyoma held with Allis' forceps, G - roll of gauze mop passed behind the pedicle of the leiomyoma.


Figure 2. The pedicle of the leiomyoma is being cut.


Figure 3. Divided pedicle of the leiomyoma is held with Allis' forceps and traction is made to reveal the uterine fundus (U).


Figure 4. The cut edges of the pedicle are being sutured with a continuous stitch of No. 1-0 polyglactin.


Figure 5. The leiomyoma (L) is held with a tenaculum.


Figure 6. A wedge (W) of the leiomyoma is being removed.


Figure 7. Another wedge (W) of the leiomyoma is being removed.


Figure 8. Another wedge (W) of the leiomyoma is being removed.


Figure 9. Another wedge (W) of the leiomyoma is being removed.


Figure 10. Another wedge (W) of the leiomyoma is being removed.

Figure 11. The residual leiomyoma (U) is being delivered through the abdominal incision.


Figure 12. Specimen showing wedges of the leiomyoma and residual leiomyoma.

Discussion

Leiomyomas are smooth muscle tumors – commonest tumors of uterus. Most of them are small and asymptomatic, they just need observation by frequent pelvic examinations and ultrasonography to look for any rapid enlargement in size. Few may produce symptoms like abdominal pain – because of red degeneration, ulceration and infection or torsion of a large subserosal leiomyoma, dyspareunia, dysmenorrhea, menstrual irregularities – menorrhagia/menometrorrhagia which is more common with submucosal variety, postmenopausal bleeding, pressure symptoms leading to urinary retention/increased urinary frequency, infertility, spontaneous abortions and very rare complications like intravenous leiomyomatosis and sarcomatous change. Sarcomatous change is less common in subserosal variety as it contains more fibrous tissue. According to US FDA, chances of leiomyosarcoma are 1:458 grossly in uterine leiomyomas.[2]

Expectant management can be done in asymptomatic leiomyomas and leiomyomas without any complications, where one is certain of its origin and benign nature of the mass. Symptomatic leiomyomas need surgical excision. Hysterectomy or myomectomy can be done after weighing the risks and benefits of each option.  Myomectomy can be done especially when the patient desires her reproductive function. Myomectomy for subserosal leiomyomas can be done by an abdominal route (exploratory laparotomy/laparoscopically or robotic assisted). Laparoscopic or mini laparotomy routes have lot of advantages over an exploratory laparotomy because of which these approaches are preferred by most patients and surgeons - minimal postoperative discomfort, lesser chances of adhesions, hernia, less hospital stay, faster healing, better cosmesis.[3 to 6] But the complications associated with morcellation techniques also have to be considered - uterine perforation, bowel injury and direct trauma to other surrounding organs, dissemination of benign leiomyoma tissue leading to recurrence of ectopic or parasitic leiomyomas causing ‘Leiomyomatosis peritonealis disseminata’ or dissemination of an occult sarcoma.[7] The most serious complication of abdominal intraperitoneal morcellation is the dissemination of an occult leiomyosarcoma, which will increase the stage of cancer and worsen its prognosis and patient  5 year survival rate,[8] maximally seen with laparoscopic power morcellator.[9,10] The FDA has reported 1:350 chance of uterine sarcoma in patients undergoing hysterectomy or myomectomy for leiomyomas, but this is based only on a review of 9 retrospective single institution studies; rest of the studies show a much lower risk.[8] FDA has issued warnings on the patient selection while considering morcellation to increase patient safety :
A) Laparoscopic power morcellators are contraindicated for removal of uterine tissue containing suspected leiomyomas in patients who are peri- or post-menopausal, or are candidates for en bloc tissue removal, for example through the vagina or mini-laparotomy incision. (Note: These groups of women represent the majority of women with leiomyomas who undergo hysterectomy.[8]
B) Laparoscopic power morcellators are contraindicated in gynecologic surgery in which the tissue to be morcellated is known or suspected to contain malignancy.[8]
Containment techniques are advised where different retrieval bags can be used to retrieve the sample and minimize dissemination of tissue and related complications.[11,12]

In our case, a small incision was used, the total length of which was equal to the cumulative lengths of the four abdominal incisions that would be required for laparoscopic myomectomy. Use of conventional technique reduced the cost tremendously as compared to laparoscopic surgery. Use of a rolled up gauze mop to make traction on the pedicle and trap it just below the abdominal wall incision was an innovative idea. It kept the pedicle trapped until it could be held with Allis’ forceps and cut. It also fixed the leiomyoma in position under the anterior abdominal wall until the pedicle was divided. Removing wedges of leiomyoma only from the part projecting out of the abdominal incision was another innovative idea. It prevented any unintentional, unanticipated spill of parts of the leiomyoma into the peritoneal cavity and resultant dissemination of occult malignancy (leiomyosarcoma) which is the greatest fear during any morcellation technique. The patient had a shorter hospital stay, cosmetically better scar, less postoperative pain as compared to a laparotomy.
Treatment option has to be individualized for each patient considering patient’s age, symptoms, size of the leiomyoma, suspicion of any cancerous changes and preference of the patient.

Acknowledgment

We thank Dr Girija Swaminathan for taking intraoperative photographs.

References
  1. Buttram VC, Reiter RC. Uterine leiomyomata : etiology, symptomatology, and management. Fertil Steril 1981;36:433.
  2. US Food and Drug Administration. UPDATED Laparoscopic Uterine Power Morcellation in Hysterectomy and Myomectomy: FDA Safety Communication.http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm424443.htm.
  3. Nieboer TE, Johnson N, Lethaby A, et al. Surgical approach to hysterectomy for benign gynaecological disease. Cochrane Database Syst Rev 2009;(3): CD003677.
  4. ACOG Committee Opinion No. 444: choosing the route of hysterectomy for benign disease. Am Cong Obst Gynecol 2009;114(5):1156–8.
  5. Worldwide AAMIG. AAGL position statement: route of hysterectomy to treat benign uterine disease. J Minim Invasive Gynecol 2011;18(1):1–3.
  6. Wiser A, Holcroft C, Tulandi T, Abenhaim H. Abdominal versus laparoscopic hysterectomies for benign disease: evaluation of morbidity and mortality among 465,798 cases. Gynecol Surg 2013;10:117–22.
  7. Naumann RW, Brown J. Complications of electromechanical morcellation reported in the manufacturer and user facility device experience (MAUDE) database. J Minim Invasive Gynecol 2015;22(6):1018e21.
  8. Administration FaD. UPDATED Laparoscopic Uterine Power Morcellation in Hysterectomy and Myomectomy: FDA Safety Communication http://www.fda.gov/medicaldevices/safety/alertsandnotices/ucm424443.htm2014 [updated November 24, 2014; cited 2015 February 18].
  9. Leren V, Langebrekke A, Qvigstad E. Parasitic leiomyomas after laparoscopic surgerywith morcellation. Acta Obstet Gynecol Scand 2012;91:1233-6.
  10. Seidman MA, Oduyebo T, Muto MG, Crum CP, Nucci MR, Quade BJ. Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms. PLoS One2012;7:50058.
  11. Solima E, Scagnelli G, Austoni V, Natale A, Bertulessi C, Busacca M, et al. Vaginal uterine morcellation within a specimen containment system: a study of bag integrity. J Minim Invasive Gynecol 2015; 22:1244–1246.
  12. Winner B, Porter A, Velloze S, Biest S. Uncontained compared with contained power morcellation in total laparoscopic hysterectomy. Obstet Gynecol 2015; 126:834–838.
  13. George S, Barysauskas C, Serrano C, Oduyebo T, Rauh-Hain JA, Del Carmen MG, et al. Retrospective cohort study evaluating the impact of intraperitoneal morcellation on outcomes of localized uterine leiomyosarcoma. Cancer 2014.
Citation

Parulekar M, Parulekar SV. Resection Of A Large Subserosal Leiomyoma By Minilaparotomy. JPGO. 2018 Vol 5 No. 10. Available from: http://www.jpgo.org/2018/10/resection-of-large-subserosal-leiomyoma.html

Pregnancy Outcome With An Amniotic Band

Author Information

Singhania N*, Joshi 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 multigravida woman presented to us with a fetal MRI showing amniotic band towards the left lateral uterine wall with no gross anomaly. There was a progressive reduction in the thickness of the amniotic band over the antenatal period. She went into spontaneous labor at term and delivered, a healthy male child of 3.436 kg, with no limb or any other gross anomalies. Pregnancy with amniotic band is a rare occurrence; one such case has been reported here.

Introduction

Amniotic band is a rare clinical entity which can present with a wide range of features in the fetus from multi organ involvement ranging from complete absence of an extremity to rarely no abnormality at all. Here, we present a case of a multigravida with confirmed amniotic band on fetal MRI with no specific fetal defects. The incidence of amniotic bands, associated dilemmas and the role of fetal MRI will be discussed. 

Case Report

A 29 year old woman, married since 3 years, Gravida 3, MTP 1, Abortion 1 registered in our hospital at 8.3 weeks of gestation. She had regular antenatal follow up. At 16 weeks of gestation routine obstetric imaging was done suggestive of a small synechiae on left side of the uterine wall in the antero-posterior and cephalo-caudal direction measuring 2.5 cm in length, partially dividing the amniotic cavity. Fetal parts and placenta were seen on either side of it. She was advised a fetal MRI, which was suggestive of a linear T2 hypointense band towards left lateral wall at mid-corpus extending between anterior and posterior myometrium. A 1-2 mm thick amniotic band was going from placenta to anterior wall of gestational sac, one fetal foot was seen lateral to it (Figure 1). However, limb movements were normal. She was followed up regularly in the antenatal period. A repeat ultrasonography done at a later date showed no obvious fetal limb mal-positioning or gross anomaly. All other routine investigations were within normal limits. Her antenatal course remained uneventful. She went into spontaneous labor at term and delivered at 39.5 weeks. An outlet forceps was applied in view of prolonged second stage of labor. She delivered a male child of 3.436 kg with an APGAR score of 9/10 at 1 minute and 5 minute. Baby had good cry with no limb or any other gross abnormalities detected. She was discharged 48 hours post-delivery.

Figure 1. Fetal MRI with arrow pointing to the amniotic band.

Discussion

Amniotic band syndrome (ABS) is a collection of congenital anomalies which can range from minor constriction bands to major deformities which are incompatible with life.[1] This entity has remained a dilemma and has been extensively studied for years. The incidence ranges from 1:1200 to 1:15000 live births and 178:10000 spontaneous abortions.[2] The organs affected depend upon the location of the amniotic band. There are several different terminologies used to describe this entity, the commonly used terminologies include amnion rupture sequence, aberrant tissue band syndrome, ADAM (amniotic deformity, adhesions, mutilations) complex, constriction band syndrome and Streeter's dysplasia.[3] The exact etiopathogenesis of ABS is still unknown. ABS is not of a genetic origin since it has not been found to occur in siblings.[4]  Maternal trauma, oophorectomy during pregnancy, amniocentesis, intrauterine devices and connective tissue disorder (Ehler Danlos Syndrome) are found to play some role in its etiopathogenesis.[5,6,7,8]
There have been numerous theories proposed for the etiopathogenesis of this condition. Two of the most widely acknowledged theories are the‘Extrinsic’ and ‘Intrinsic’ Theory. Amongst the two, the most widely accepted theory is the extrinsic theory (amniotic disruption), proposed by Torpin in 1965.[9]  According to this theory, the occurrence of an amniotic rupture leads to loss of amniotic fluid with extrusion of all or some parts of the fetus into the chorionic cavity. This subjects the fetus to compression leading to entanglement of fetal limbs or other body parts in the remnants of the amnion. The type of anomalies that occur depend on the timing of amniotic rupture during pregnancy as well as the site and the degree of compression of the underlying structures. According to Higginbottom et al, the damage that takes place before 45 days of gestation leads to severe facial clefts and severe defects of the brain and calvarium, while damage after 45 days of gestation resulted in limb involvement without facial and central nervous involvement.[10] Whereas Streeter's intrinsic (embryonic dysplasia) theory suggests that fetal disruptions result from imperfect histogenesis.[11]
The most common fetal part affected are the limbs, these present as digital amputations, constriction rings or acrosyndactyly.[3] Other uncommon defects noted include talipes equinovarus, scoliosis, craniofacial abnormalities, such as cleft lip or cleft palate, orbital defects, corneal abnormalities, body wall defects, and internal organ abnormalities.[3,12] It has been noted that even a single constriction band can lead to digital or extremities amputation or may affect the growth of a particular body part.[13] An amniotic band can be diagnosed on ultrasonography (USG) as early as 12 weeks of gestation. The USG diagnostic criteria for amniotic bands are appearance of linear echoes floating in amniotic fluid, constriction rings on extremities and irregular limb amputations. A fetal MRI is more sensitive and helpful in diagnosing difficult cases.[14] The treatment of amniotic bands is surgical which should have a multidisciplinary approach and should be individualized for each case. Prenatal treatment of ABS has also been attempted in the form of fetoscopic laser cutting of amniotic bands, before their compression causes malformation.[15]

Conclusion

Amniotic band is a rare occurrence in pregnancy. The exact mechanism of its occurrence still remains unanswered. Owing to the wide range of clinical presentations, this condition warrants need for proper patient education and routine antenatal follow-up to ensure a timely detection. 

References
  1. Ciloglu NS, Gumus N. A rare form of congenital amniotic band syndrome: total circular abdominal constriction band. Arch Plast Surg. 2014;41(3):290–291.
  2. Richardson S, Khandeparker RV, Pellerin P. Amniotic constriction band: a report of two cases with unique clinical presentations. J Korean Assoc Oral Maxillofac Surg.  2017;43(3):171–7.
  3. Goldfarb CA, Sathienkijkanchai A, Robin NH. Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation.  The Journal of Bone and Joint Surgery. 2009;91(Suppl 4):68–75.
  4. Verma A, Mohan S, Kumar S. Late presentation of amniotic band syndrome: A case report.  J Clin Diag Res. 2007;1(2):65-68.
  5. Tanaka O, Koh T, Otani H. Amniogenic band anomalies in a fifth-month fetus and in a newborn from maternal oophorectomy during early pregnancy. Teratology 1986;33:187–93
  6. Csécsei K, Szeifert GT, Papp Z. Amniotic bands associated with early rupture of amnion due to an intrauterine device. Zentralbl Gynakol. 1987;109(11):738-41.
  7. Kohn G. The amniotic band syndrome: a possible complication of amniocentesis. Prenat Diagn 1987;7(4):303–5.
  8. Young ID, Lindenbaum RH, Thompson EM, Pembrey ME. Amniotic bands in connective tissue disorders. Arch Dis Child 1985;60(11):1061–3.
  9. Torpin R. Amniochorionic mesoblastic fibrous strings and amnionic bands: associated constricting fetal malformations or fetal death. Am J Obstet Gynecol. 1965;91:65–75.
  10. Higginbottom MC, Jones KL, Hall BD, Smith DW. The amniotic band disruption complex: timing of amniotic rupture and variable spectra of consequent defects. J Pediatr. 1979;95(4):544–549.
  11. Streeter GL. Focal Deficiencies in Fetal Tissues and Their Relation to Intra-Uterine Amputation. Contributions to Embryology. 1930;22:1–44.
  12. Doi Y, Kawamata H, Asano K, Imai Y. A case of amniotic band syndrome with cleft lip and palate. J Maxillofac Oral Surg. 2011;10(4):354–356.
  13. Kashyap S, Shanker V, Sharma N. Amniotic band: a rare presentation. Indian J Dermatol. 2015;60(2):200–202.
  14. Merrimen JL, McNeely PD, Bendor-Samuel RL, Schmidt MH, Fraser RB. Congenital placental-cerebral adhesion: an unusual case of amniotic band sequence. Case report. Neurosurgery 2006;104(5):352-355.
  15. Quintero RA, Morales WJ, Phillips J, Kalter CS, Angel JL. In utero lysis of amniotic bands. Ultrasound Obstet Gynecol 1997;10(5):316-20.
Citation

Singhania N, Joshi A, Gupta AS. Pregnancy outcome with an amniotic band. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/pregnancy-outcome-with-amniotic-band.html

Pregnancy Complicated By Essential Thrombocythemia: Risks And Challenges

Author Information

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

Abstract

Essential thrombocythemia (also known as essential thrombocytosis) is a rare blood disorder characterized by excessive production of platelets by megakaryocytes of the bone marrow. Management of pregnancy in such patients is a challenge and needs a multidisciplinary team approach with a hematologist. Here we present a case of a patient incidentally diagnosed of this condition in her second pregnancy.

Introduction

Essential thrombocythemia is a form of chronic myeloproliferative disorder. The annual incidence of cases reported worldwide varies between 0.59 to 2.53 per 1,00,000 individuals. It is predominantly a disorder of the older age with a median occurrence around 65-70 years. However, with the advent of automated platelet counting the disease has been diagnosed in asymptomatic middle age group patients with a preponderance noted in the female population.[1]

Case Report

A 25 year old, G2P1L1 with previous lower segment cesarean section, a known case of essential thrombocytosis registered for antenatal care in our hospital at 20 weeks of gestation. She had registered previously at 3 months of gestation in a private hospital. On routine investigations, her platelet count was found to be elevated (11 lac/cubic mm) while hemoglobin and white blood cell count was normal. She had no complaints and there was no significant medical illness in the past. She was referred for a hematology opinion. She visited a private hematology consultant who advised iron studies, vitamin B12 levels, LDH, ANA, hemoglobin electrophoresis, erythropoietin levels and genetic studies (JAK2, CALR and MPL mutations). On evaluation, her ANA levels were found to be weakly positive, rest all tests were normal and the gene mutations were all negative. She was started prophylactically on 75 mg aspirin daily.
She then registered at our institute and had regular follow up visits with our institutional hematologist. On reviewing her reports, a provisional diagnosis of essential thrombocytosis was made. She was advised to undergo a bone marrow aspiration to confirm the diagnosis and the plan was to start on low molecular weight heparin and interferon if the result came positive. Bone marrow aspiration biopsy revealed a normocellular bone marrow with increase in erythroid as well as megakaryocytic series. Final impression was essential thrombocythemia. She was started on injection LMWH 0.4 ml subcutaneous once a day, interferon alpha 3 million units twice a week subcutaneous to be continued throughout pregnancy. Aspirin was to be continued throughout pregnancy and routine follow up with monthly complete hemogram reports was advised. Her hemogram reports showed a gradual decline in the platelet count on treatment from 11 lacs/cc to 8 lacs/cc, 6 lacs/cc and finally 4 lacs/cc in labor. She had regular antenatal follow up. On routine blood investigations she was diagnosed to be hypothyroid and treatment was started for the same. Rest all investigations were normal. Her antenatal course was uneventful.
She had a previous LSCS done 3 years back in view of failure of induction. In this pregnancy she was willing for a vaginal birth after cesarean section. Hence, the plan of action for her was to await for spontaneous onset of labor till term. Injection LMWH was to be stopped 12 hours prior to delivery and it was to be restarted 12 hours after delivery.
She came in labor at 40 weeks. On examination her general condition was fair, she was afebrile, had a pulse rate of 88 beats/ min, and blood pressure was 110/70 mm of Hg. Cardiovascular and respiratory system examination was within normal limit. On per abdomen examination uterus was full term, Vertex in left occipito anterior position was 3/5th palpable, fetal heart rate was 144 bpm and uterine activity was 1/10/10-15. On per vaginal examination os was 2.5 cm dilated, 40% effaced, station -1, membranes present and show was present. Her night dose of LMWH was omitted. She progressed spontaneously and had an uneventful vaginal delivery. She delivered a male child of 2.56 kg. Her postnatal course was uneventful. LMWH was restarted 24 hours post-delivery. A hematology review was taken postpartum and she was advised to continue LMWH for 6 weeks postpartum and to continue aspirin and interferon treatment lifelong.

Discussion

Essential thrombocythemia is a rare variety of myeloproliferative blood disorder. The diagnosis is confirmed when other secondary causes of thrombocythemia are excluded. The modified criteria by British Committee for Standards of Hematology suggest the presence of the following criteria:[2]
A sustained platelet count ≥ 450,000/ microliter.
Presence of acquired pathogenic mutations (like JAK2, MPL)
No other myeloid malignancies (like polycythemia vera, primary myeloproliferative fibrosis)
No reactive cause for thrombocytosis with normal iron stores.
Bone marrow aspirate biopsy showing increased megakaryocyte numbers associated with predominant large megakaryocytes with hyper-lobulated nuclei and abundant cytoplasm.
The presence of 1+2 or 1+3,4,5 is required to diagnose the patient as a case of essential thrombocytosis. The association of this condition with pregnancy poses an increased risk of thrombosis and hemorrhage. 
The clinical presentation of the patient may vary from vague common symptoms to serious potentially life-threatening manifestations. Generalized headaches, paresthesia, fatigue, easy bruising and bleeding tendencies are some of the common symptoms. However, some patients may have major thrombotic events in the form of stroke, transient ischemic attacks, infarction and pulmonary embolism or major hemorrhagic complications in terms of severe bleeding episodes.
Abruptio placenta, intrauterine fetal death and preterm labor are the commonest complications encountered with this condition. These are predominately seen in the later half of pregnancy. Thromboembolic complications were more common than hemorrhagic complications. Also, fetal complications were encountered more frequently than maternal complications. These include pregnancy loss (abortion, stillbirth) and intrauterine growth restriction. First trimester pregnancy loss was noted to be more common. The live birth rate noted by Brière et al was merely 50-57%.[1]  The increased platelet count can cause micro-infarctions in the placental bed and lead to activation of platelet function which is largely responsible for the adverse fetal effects. Hemorrhagic manifestations are few and occur primarily due to proteolytic reduction of the von Willebrand Factor (vWF) multimers which results in a state of acquired von Willebrand disorder leading to bleeding tendencies. The vWF is a protein required for platelet adhesion and effective coagulation. Hemorrhage occurs rarely and especially in patients with very high platelet count, hence the need for cytoreduction. [3]
Most of the patients are incidentally diagnosed on a routine laboratory investigation. This can be attributed to the indolent course of the disease. Investigations mainly deal with ruling out conditions that can lead to secondary thrombocytosis, these include chronic inflammatory conditions, infections, iron deficiency anemia, protracted bone marrow disorders and malignancy. An evaluation of genetic mutations especially JAK2, CALR and MPL is also done. The presence of a genetic mutation is seen in almost 60% of the cases, JAK-2 being the commonest one forming almost 50% of those cases.[4] Studies have shown a better pregnancy outcome for patients with CALR mutations as compared to JAK2 mutations.[5] Being a somatic mutation, newborns are generally not tested for this gene. However, for patients with a positive family history of hereditary thrombocytosis, a genetic mutation testing maybe carried out for the offspring.[6]
The main goal of treatment is to avoid thrombotic or hemorrhagic complications. Hence, the aim of the therapy is cytoreduction. Interferon alpha is an antiproliferative agent which inhibits the progenitor cells of megakaroytes in the bone marrow, thereby decreasing the platelet count in the peripheral blood. Multiple studies have demonstrated that the drug is effective in as many as 90% of the patients. Although interferon alpha is FDA category C drug, it is the cytoreductive agent of choice since other drugs like hydroxyurea are teratogenic. Its pegylated formulation is an emerging choice due to its longer half-life. However, owing to the limited data on safety profile of the pegylated formulation, it is not yet preferred in pregnancy.
The increased thrombotic tendency in these patients leads to repeated placental infarcts and chorionic villi fibrosis thereby increasing the tendency for abortions. Here the role of acetylsalicyclic acid (ASA) and low molecular weight heparin (LMWH) is of utmost importance.[7] Low dose ASA acts by selectively decreasing the thromboxane A2 levels which in turn decreases the rate of placental infarcts, leading to a positive pregnancy outcome. A study by Grieshammer et al showed a live birth rate of almost 68% in patients treated with ASA proving the beneficial role of this drug.[8]
The routine use of LMWH in all cases is controversial since the average risk of venous thromboembolism (VTE) in antenatal period is only 1-3%. However, for patients with a past history of VTE or DVT, antenatal prophylaxis with heparin is a must. In other cases, LMWH can act as a prophylactic measure to counteract the enhanced coagulation status of pregnancy. There have been no randomized controlled trials by far to determine the adjunct role of LMWH in these patients. These lack of controlled studies makes it difficult to define a standard form of therapy.

Conclusion

Patients of essential thrombocythemia with pregnancy need multi modal management. Strict vigilance of clinical and laboratory parameters throughout pregnancy is vital. Improved diagnostic methods and discovery of newer medications has led to better pregnancy outcomes over the past decade.

References
  1. Brière JB. Essential thrombocythemia. Orphanet J Rare Dis. 2007;2:3.
  2. Harrison CN, Butt N, Campbell P, Conneally E, Drummond M, Green AR, et al. Modification of British Committee for Standards in Haematology diagnostic criteria for essential thrombocythaemia. Br J Haematol. 2014;167(3):421–3.
  3. Landolfi R, Cipriani MC, Novarese L. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: Pathogenetic mechanisms and prevention. Best Pract Res Clin Haematol. 2006;19(3):617–33.
  4. Harrison CN, Bareford D, Butt N, Campbell P, Conneally E, Drummond M, et al. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol.2010;149(3):352–75.
  5. Rumi E, Bertozzi I, Casetti IC, Roncoroni E, Cavalloni C, Bellini M, et al. Impact of mutational status on pregnancy outcome in patients with essential thrombocytemia. Haematologica. 2015;100(11):e443–5.
  6. Teofili L, Giona F, Torti L, Cenci T, Ricerca BM, Rumi C, et al. Hereditary thrombocytosis caused by MPLSer505Asn is associated with a high thrombotic risk, splenomegaly and progression to bone marrow fibrosis. Haematologica. 2010 Jan ;95(1):65–70.
  7. Radaelli F, Colombi M, Maiolo AT. Essential thrombocythemia in pregnancy: report of four cases. Haematologica; 1994;79(4):360–3.
  8. Griesshammer M, Grünewald M, Michiels JJ. Acquired Thrombophilia in Pregnancy: Essential Thrombocythemia. Semin Thromb Hemost 2003;29(2):205–12.
Citation

Joshi A, Gupta AS. Pregnancy Complicated By Essential Thrombocythemia: Risks And Challenges. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/pregnancy-complicated-by-essential.html

Management Of A Rare Case Of Glanzmann`s Thrombesthenia In Pregnancy

Author Information

Chouhan T*, Tiwari N**, Chaudhari HK***.
(* Junior Resident, ** Assistant Professor, *** Associate Professor. Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)

Abstract

Glanzmann`s thrombasthenia is an autosomal recessive bleeding disorder where there is excessive bleeding inspite of normal platelet count due to platelet function defect. Here the defect lies in the human glycoprotein (Gp/gp) IIb/IIIa receptors present on the surface of platelets responsible for platelet aggregation. Patients usually present with menorrhagia, epistaxis, gum bleeding. Haematuria and GI bleeding occurs rarely. It is specially important in pregnancy as it can result in postpartum hemorrhage. We are reporting a case of Glanzmann’s thrombasthenia in pregnancy where the patient, who was a known case of Glanzmann thrombasthenia was managed successfully, and post-partum hemorrhage was prevented .

Introduction

Glanzmann thrombasthenia is a platelet function defect where there is prolonged bleeding time due to defective platelet aggregation.[1] Since it is an autosomal recessive disorder it is transmitted only when mutation is present in both the parents. The defect is in the genes that code for the glycoprotein Gp IIbIIIa receptors present on the surface of platelets responsible for platelet aggregation.
Presenting features includes easy bruising, epistaxis, bleeding from gums, menorrhagia, postpartum hemorrhage, abnormal bleeding following surgery, hematuria, hematemesis, gastrointestinal bleed or genitourinary bleed.[2] This condition is more problematic in women due to bleeding during menstruation and childbirth.

Case Report     

A 30 year old primigravida, a known case of Glanzmann thrombasthenia, 29 weeks of gestation presented in the emergency with complaints of spotting per vagina and with a ultrasound (USG) scan showing a placenta previa. She was admitted.  Placenta  previa was excluded by a repeat USG. Hematologist was consulted for Glanzmann thrombasthenia. She was diagnosed as a case of Glanzmann thrombasthenia at menarche when he was 13 years old as she had severe menorrhagia. Flow cytometry was used to diagnose the condition. She gave history of repeated platelet transfusions since then for menorrhagia. She also had complaints of 4 – 5 episodes of haematuria. She was on cyclical estrogen progesterone combined pills (Ovral G). She consulted a hematologist and a gynecologist before conception. Hematologist advised to keep platelets ready for her and to give her NovoSeven® [recombinant human coagulation Factor VIIa (rFVIIa)] 30 mcg/kg injection prior to delivery. She was regularly cared for by the hematologist and obstetrician during this pregnancy as an outdoor case.

She was admitted at 36.3 weeks of pregnancy. USG showed gross hydrocephalus in the baby with dilatation of the 3rd, 4th and the lateral ventricle with blood clots and internal echos within the ventricles. There was blood clot in retro cerebellar region also. Poor prognosis was explained by pediatric surgeons and evaluation of the mother for mutation was advised. Platelet count of newborn was also advised. She went into spontaneous labor. All investigations were sent hemoglobin was 13.1 gm %, platelet count was1.7 lacs/ ml with normal coagulation profile. Cephalocentesis was advised for the baby in as the biparietal diameter was 11 cms. Pre-procedure 1 unit of SDP (single donor platelet) was transfused. Cephalocentesis was done were 190 ml of cerebrospinal fluid (CSF) was drained for gross hydrocephalus. She progressed normally in labor. Before delivery 4 injections of Novoseven 4 mcg was given and 6 units of RDP (random donor platelets) were transfused. She had assisted breech delivery. Baby was fresh still birth. Post-delivery vaginal pack was kept in situ to control bleeding from a cervical laceration after suturing it. A 20 IU of Pitocin drip was given and per rectally 800 mcg of misoprostol was kept. One unit of SDP (single donor platelet ) was transfused post-delivery after 12 hours followed by 6 units of RDP (random donor platelet) the next day and another SDP on day 3. Vaginal pack was removed after 6 hours. She was stable and did not have excessive bleeding post-delivery. She was discharged after hematology opinion. Contraceptive counseling was done.

Discussion

Glanzmann thrombasthenia is an autosomal recessive condition which does not manifest in heterozygous carrier states. It is estimated that one in 1,000,000 individuals have Glanzmann thrombasthenia with slight predominance in woman.[3] Fertility is not affected in case of Glanzmann thrombasthenia. There is an increased risk of post-partum hemorrhage. New born thrombocytopenia is occasionally severe but always transitory. There is problem in platelet aggregation due to defect in glycoprotein IIbIIIc resulting in prolonged bleeding time and defective clot retraction. Patients will have normal platelet count and coagulation profile but with increased bleeding time. Platelet aggregation in presence of collagen and ADP will be impaired as this requires fibrinogen whereas the one with ristocin will be normal.[4]
Glanzmann thrombasthenia is further divided into type I where < 5 % receptors (GpIIbIIIa) are present and aggregation of platelets will be absent, type II where 5-20% receptors are present and function of platelets is impaired but not absent and type III where receptors are present but function is defective. Most common presentation in  non pregnant women is menorrhagia which is controlled initially with tranexamic acid and oral contraceptive pills. Injection testosterone or injection leuprolide is helpful in severe cases but specific management includes platelet transfusion. Obstetric patients have high risk of developing post-partum hemorrhage. Definitive diagnosis is based on flow cytometry where CD 41 and CD 61 receptors for GpIIb and GpIIIa and fibrinogen receptors are absent.[4] For preventing post-partum hemorrhage adequate platelets should be kept ready before delivery. Most patients will have history of multiple platelet transfusion which result in development of antibodies and risk of alloimmunization increases with successive pregnancies. Repeated platelet transfusion is required in patients to control bleeding. Use of antiplatelet drugs like aspirin and other NSAIDs are contraindicated in such patients. Other control measures include use of oral contraceptives to control menorrhagia and use of Novoseven. This activated factor Novoseven is reserved for people who have already developed antibodies after several platelet transfusions.[2]

Novoseven which is an activated factor is helpful in this condition.[5] Novoseven was initially used in management of hemophilia but its role has increased in management of Glanzmann thrombasthenia. It is specially useful in this case as due to platelet transfusion these patients have antibodies against platelets and therefore a directly acting activated factor 7 is more effective. Intramuscular injection should be avoided. Patients with mutations detected should be adequately counseled about risk of transmission to the fetus. Pregnant patients develop bleeding post-partum and even during puerperium which can be controlled by use of uterotonics followed by platelet transfusion.These patients have increased risk of maternal and fetal bleeding and high chances of neonatal deaths.[6]

Conclusion

Prognosis of Glanzmann thrombasthenia is good when proper supportive care is given. Post-partum bleeding can be serious and requires timely intervention. This case highlights the need of timely replacement of platelets and proper measures to prevent post-partum hemorrhage for managing a patient with Glanzmann thrombasthenia.

References
  1. Pittman MA, Graham JB Glanzman’s Thrombopathy: an autosomal recessive trait in one family. Am J Med Sci. 1964;247:293-303.
  2. Poon M-C. Clinical use of recombinant human activated factor VII (rFVIIa) in the prevention and treatment of bleeding episodes in patients with Glanzmann’s thrombasthenia. Vasc Health Risk Manag. 2007; 3(5): 655–664.
  3. Sundqvist S-B, Nilsson IM, Svanberg L, Cronberg S. Pregnancy and parturition in a patient with severe Glanzmann’s thrombasthenia. Scand J Haematol. 1981;27(3):159-64.
  4. Sharp WJ, Khanduri UD, Christie BS. Rapid heterozygote detection in Glanzmann’s thrombasthenia. Br J Haematol 1998;101(1): 66 –9
  5. Fernandes G M, de Melo RM, Plens G, Pontes EM, Silva MM, da Rocha JC. Surgical and clinical management of a patient with Glanzmann thrombasthenia: a case report. Quintessence Int. 2004;35(8):617-20.
  6. Balci YI, Karabulut A, Sibel Kabukcu S, Sari I, Keskin A. Intensive Menstrual Bleeding Successfully Treated With Recombinant Factor VIIa in Glanzmann Thrombasthenia. Clin Appl Thrombo Hemost. 2011;17(4):320:2.
  7. Fiore M, Nurden AT, Nurden P, Seligsohn U. Clinical utility gene card for: Glanzmann thrombasthenia. Eur J Hum Genet. 2012;20(10):1102.
Citation

Chouhan T, Tiwari N, Chaudhari HK. Management Of a Rare Case Of Glanzmann`s Thrombesthenia In Pregnancy. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/management-of-rare-case-of-glanzmanns.html

Submucosal Fibroid: A Diagnostic And Management Challenge In A Case Of Missed Abortion

Author Information

Uppal M*, Samant PY**, Jagtap J***, Agarwal P*
(* Junior Resident, ** Additional Professor, *** Senior Resident. Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)

Abstract

We present an interesting case of suspected uterine perforation and omental prolapse into the vagina during surgical evacuation of missed abortion. The patient required two step surgical procedures to remove a submucosal fibroid that had undergone ischemic necrosis and partial expulsion. The case was a surgical challenge due to high chance of uterine injury, inversion and septicemia. It also highlights imaging errors.

Introduction

Submucosal fibroids are a risk factor for a spectrum of pregnancy related problems from early pregnancy loss, abruption, growth restriction, need for cesarean section, postpartum hemorrhage and inversion.[1] Women with submucosal fibroid seeking surgical abortion often present a unique challenge. When the uterine cavity is significantly enlarged or distorted, it makes surgical evacuation difficult or impossible. Possible complications in such cases could be error in clinically estimating gestational age, pregnancy loss, incomplete evacuation, uterine perforation, hemorrhage, bowel/ bladder injury, cervical laceration and hematometra.

Case Report

A 25 year old G3P2L2SA1 was referred to our institute on the 6th post operative day after curettage with suspected uterine perforation and omental prolapse. She had 2 full term normal deliveries in the past. She had undergone dilatation evacuation with Cu-T insertion for missed abortion of 8 weeks and 6 days pregnancy. On the 4th post evacuation day, she complained of abdominal pain, fever and multiple episodes of vomiting. Pelvic ultrasonography (USG) from the private hospital was suggestive of bulky uterus with rent in the left posterolateral wall at the junction of the body of the uterus and cervix with prolapse of omentum into the uterine cavity and vagina. She was referred to our hospital for further management with this report.

On examination her general condition was stable. There was mild pallor. She was afebrile, with pulse rate of 86/ minute, and blood pressure of 110/70 mm of Hg. Systemic examination was unremarkable. Abdomen was soft with no guarding, rigidity or tenderness. There was no abdominal distension and peristaltic sounds were normal. There was suprapubic fullness. On speculum examination, a necrotic polypoidal structure was seen through the cervical canal (figure 1)  In addition a shaggy necrotic foul smelling pale pinkish flimsy tissue was seen from the left lateral surface between the polyp and the cervical wall. Cu-T was found in the vagina.  On bimanual examination, uterus was 12 weeks in size, anteverted, with a firm polypoidal structure felt in the cervical canal. Cervical swab was collected for culture and antibiotic sensitivity. Detached part of the shaggy tissue was sent for histopathological examination. No forniceal fullness or tenderness was appreciated. Foley’s catheterization was done and 200 ml of clear urine was drained out. USG was repeated at our institute. It was suggestive of a 2 cm rent within the uterus in the left posterior lateral aspect with multiple air specs within the uterine cavity. CT Scan was suggestive of multiple air specs and heterogeneous content in the uterine cavity. The suspicious rent in the wall of uterus was not well appreciated on CT scan.  Her laboratory reports were unremarkable. On considering her abdominal complaints and after confirming availability of blood and products, exploratory laparotomy was performed under antibiotic cover. Intraoperatively, peritoneal cavity was clean without any visceral injury. Uterus was bulky with variegated consistency. As the exploratory laparotomy was negative she was given a lithotomy position and a speculum examination was performed. The cervical os was open and a fibroid polyp was hanging out, uterus was 14 weeks in size. An additional intracavitary fibroid was felt. Uterine sound could not be passed easily around it. Fibroid polyp was removed and sent for histopathological examination. She tolerated the procedure and anesthesia well. Two units of packed cells were transfused perioperatively. Enterococcus was grown on culture and she was treated with intravenous linezolid.

In further evaluation, USG was suggestive of a large fibroid occupying the endometrial cavity. MRI diagnosed the lesion as retained products of conception. After completion of  linezolid course, polypectomy with curettage if required was planned. A large Fibroid, ovoid in shape about 5x7 cm (figure 2) that had extruded out of the cervical canal was excised and sent for histopathological examination. Stump was checked for bleeding. Uterine tamponade was done with Foley’s catheter as the stump kept oozing despite ligatures (figure 3). She tolerated the procedure well. The Foley catheter was removed after 24 hours. Histopathological report confirmed the uterine mass as leiomyoma with hemorrhagic infarction. She was discharged uneventfully with advice about contraception.

Figure 1. Omentum like structure at the cervical os.


Figure 2.  Submucosal fibroid protruding from the cervix.


Figure 3. Foley catheter tamponade.

Discussion

On retrospection, our patient did not give any history of dysmenorrhea, abnormal uterine bleeding attributed to fibroids. Detailed history would probably have avoided the infection and the unnecessary laparotomy. Laparoscopy would have been a preferred alternative to laparotomy but it was not available during the emergency hours.
Submucosal fibroids cause numerous fetal complications in addition to fibroid enlargement and degeneration.[1,2,3] Surgical evacuation poses a challenge in cases of missed abortion. Omental extrusion with perforation of uterus is a known complication and depending on presence or absence of bowel herniation, ischemia or perforating injury the morbidity and mortality increases.[4] 
Spontaneous expulsion of a degenerated submucosal fibroid is known in post partum period.[3] However, our patient did not complain of pain indicating extrusion; hence we were misled to trust the imaging reports. If the bleeding after polypectomy is not torrential, tamponade with catheter balloon saves unnecessary interventions like embolization or hysterectomy.[5]
With infection noted at initial presentation, excision of intrauterine fibroid would have caused severe bleeding needing hysterectomy or interventional radiological procedure. So we opted for a two-step procedure. Omentum like degenerated fibroid caused concern. Erroneous USG, CT scan and MRI reports cost the patient an unnecessary laparotomy.

Conclusion

Cautious history taking, examination under anesthesia, judicious use of imaging modalities and careful surgical approach helps in avoiding unnecessary hysterectomies.

References
  1. Lee HJ, Norwitz ER, Shaw J. Contemporary Management of Fibroids in Pregnancy. Reviews in Obstetrics and Gynecology. 2010;3(1):20-27
  2. Radhika BH, Naik K, Sreelatha S, Vana H. Case Series: Pregnancy Outcome in Patients with Uterine Fibroids. Journal of Clinical and Diagnostic Research. 2015;9(10):QR01-QR04
  3. De Cure N, Sullivan T, Robertson M, Hallam L, Whale K. Spontaneous expulsion of large submucosal uterine fibroid without embolisation – a case study. AJUM. 2013; 16 (1):37-40
  4. Chandi A, Jain S, Yadav S, Gurawalia J. Vaginal evisceration as rare but a serious obstetric complication:A case series. Case Reports in Women's Health. 2016; 10:4–6
  5. Saridogan E. Surgical Treatment of Fibroids in Heavy Menstrual Bleeding.Women’s Health. 2016;12(1):53-62.
Citation

Uppal M, Samant PY, Jagtap J, Agarwal P. Submucosal Fibroid: a Diagnostic And Management Challenge In a Case Of Missed Abortion. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/submucosal-fibroid-diagnostic-and.html

Mesentric Cyst Masquerading As An Ovarian Cyst

Author Information

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

Abstract

Abdomino-pelvic cystic tumors are encountered quite frequently and are often diagnosed early due to various radiological modalities available. These masses are usually diagnosed incidentally due to imaging modalities available. Therefore presentation of huge tumors has become rare. But sometimes these patients present late with huge abdominopelvic mass and diagnosis is possible only at laparotomy and after histopathological examination as was seen in our patient. We present a case report of a 50 year woman who had a past history of hysterectomy, who underwent exploratory laparotomy for ovarian cyst but intraoperatively the cyst was found to be occupying the mesentery of descending colon with mucinous content, thus a probable diagnosis of mesocolon cyst was made, although a final diagnosis of mucinous-cystadenoma was made on histopathology.

Introduction

Mucinous cystic neoplasms (MCN) usually arise in the ovary and can arise at various extra-ovarian sites as they share a common pathway of development.[1] MCN are rare tumors of uncertain histogenesis and should be considered in the differential diagnosis of abdomino-pelvic cystic masses. The rarest type of MCN is of the mesocolon which can present as a abdomino-pelvic mass in the spectrum of mesenteric cysts.[2] MCN are usually diagnosed incidentally on radiological imaging and sometimes can present with nonspecific or chronic symptoms as was seen in our case. A mesocolonic MCN masqurading as an ovarian tumor is presented here.

Case Report

A 50 year old woman, married for 30 years came to the gynecological outpatient department with dull aching  pain in abdomen and heaviness in the abdomen for 6 months. She had also noticed a lump in the lower abdomen which had gradually increased in size. There was no history of acute pain in abdomen, loss of appetite or weight loss. There was a history of total abdominal hysterectomy done in 2015 for a fibroid uterus. She had a normal vaginal delivery 23 years back. She was diagnosed case of  myotonic dystrophy and was on  phenytoin 100 mg PO q12h. She had been investigated by gastroenterologist for deranged liver function tests and presence of a subhepatic cyst on magnetic resonance scan. Her liver biopsy had been found to be normal, and  no obvious cause was found for deranged liver enzymes. Her general and systemic examination findings were normal. Abdominal examination showed an infraumbilical vertical midline scar of previous hysterectomy and a 24 weeks' size mass arising from the pelvis, which was non tender, cystic with restricted side to side mobility. On speculum examination vaginal vault was healthy and on vaginal examination a 24 weeks mass was felt above the center of the vault. Her investigations for fitness for anesthesia, including liver function tests, showed normal results. Her ultrasonography (USG) of the abdomen and pelvis was done which showed a 14×12×10 cm well defined cystic lesion arising from left adnexa till umbilicus with few internal echoes, left ovary was not seen separately and right ovary was obscured and uterus was not visualized due to postoperative status. There was also evidence of right moderate hydronephrosis and proximal hydroureter and left mild hydronephrosis probably due to compression by the cyst. She also underwent Computerized tomography (CT) of abdomen and pelvis which showed a complex left ovarian cyst of 13.5×15×14 cm with septations and calcification, fat planes were preserved and presence of calcified lymph nodes in the mesentery. There was bilateral hydronephrosis and hydroureter due to extrinsic compression of the ureters, iliac vessels were compressed by the cyst but fat planes were preserved. Her serum CA- 125 was 23U/ml. Bilateral ureteric catheterization was done prior to an exploratory laparotomy.  Laparotomy was done through a vertical midline incision. It showed a 15×15×10 cm cystic mass occupying the mesentery of the descending colon,  which was seen stretched circumferentially over the cyst. The cyst was adherent to the posterior parietal peritoneum. In view of these findings a surgeon were called. He found the cyst to be attached to the descending colon. He separated it from the colon, dissected and enucleated it. The cyst was accidentally ruptured at the time of dissection and mucinous material was drained from the cyst which was sent for cytological examination. No malignant cells were found in the fluid. Frozen section of the cyst was suggestive of mucinous cystadenoma. A 6 to 7 cm long serosal tear in the descending colon at the site of separation of the cyst was noted and was sutured with No. 2-0 black silk with simple interrupted sutures. Hemostasis was achieved. The right ovary was found to be atrophic. There was a 2×2 cm sized  simple cyst in it. The right ovary and  lateral one third of the right fallopian tube were found to be  densely adherent to the vault and sigmoind colon and rectum. There were dense adhesions over the position of the left ovary and fallopian tube, and they could not be identified. An intraperitoneal drain was kept and the abdomen was closed. The patient was kept nil by mouth for 48 hours and was given intravenous fluids in that period. She made an uneventful recovery. The final histopathology report revealed that the cyst was a mucinous cystadenoma.


Figure 1. Laparotomy findings – the cyst is in the center (black arrows) with descending colon (green arrows) along with its mesentry completely encasing the cyst.


Figure 2. Serosal tear  in the descending colon (green arrows).


Figure 3. Repair of the serosal tear with simple interrupted sutures of No. 2-0 black silk (green arrows).

Discussion

MCN can arise in the ovary and various other extra-ovarian sites like the appendix, retroperitonium, spleen, bowel, lung, hepatobiliary tract, breast and the mesentery.[3] MCN are predominately found in adult females. The ovarian and extra-ovarian MCN have a common pathway of development. They are usually diagnosed incidentally or present with nonspecific and chronic symptoms like abdominal distension, pain, a palpable mass or symptoms of gastrointestinal or urinary obstruction.[2] There is often diagnostic dilemma in the diagnosis of MCN and its origin due to nonspecific symptoms, absence of any particular radiological features or specific biochemical markers.[4,5] In the case presented here, despite the use of imaging modalities like USG and CT, diagnosis of descending mesocolon cyst was made on laparotomy and final diagnosis of mucinous cystadenoma was made on histology. The important hypothesis which is postulated in the pathogenesis for extra ovarian MCN are celomic mucinous metaplasia of epithelial cells or peritoneal invaginations in the course of ovarian decent.[6] The various other proposals given in literature have been attributed to the ectopic or implanted or supernumerary ovary.
Our case adds to one of the rare cases of MCN which was found in the mesocolon. The diagnosis could not be achieved preoperatively and surgery was performed jointly by surgeon and gynecologists due to the involvement of descending mesocolon and preoperative misdiagnosis of the cyst as an ovarian cyst.  It also highlights the importance of intraoperative frozen section which aided in our management. Surgery is the mainstay of treatment and can be performed by laparotomy or laparoscopically. The surgical treatment of choice is complete excision. If it is not possible, marsupialization may be done, though it can lead to development of pseudomyxoma peritonei at a later date.

Acknowledgment

We thank Dr. Girja Swaminathan for taking operative photographs.

References
  1. Shiono S, Suda K, Nobukawa B, Arakawa A, Yamasaki S, Sasahara N, et al. Pancreatic, hepatic, splenic, and mesenteric mucinous cystic neoplasms (MCN) are lumped together as extra ovarian MCN. Pathol Int. 2006;56:71–77.
  2. Metaxas G, Tangalos A, Pappa P, Papageorgiou I. Mucinous cystic neoplasms of the mesentery: a case report and review of the literature. World Journal of Surgical Oncology 2009 May 19;7:47. doi: 10.1186/1477-7819-7-47.
  3. Nakayama Y, Kato Y, Okubo S, Takahashi D, Okada R, Nishida Y, et al. A case of mucinous cystic neoplasm of the liver: a case report. Surg Case Rep. 2015 Dec; 1: 9. doi: 10.1186/s40792-014-0007-z
  4. Izumo A, Yamaguchi K, Eguchi T, Nishiyama K, Yamamoto H, Yonemasu H, et al. Mucinous cystic tumor of the pancreas: immunohistochemical assessment of "ovarian type stroma." Oncol Rep 2003;10:515-525.
  5. Shaco-Levy R, Tsodikov V, Levy I. Mucinous cystadenoma of the ascending colon: a novel presentation. Scand J Gastroenterol. 2003 Nov;38(11):1193-5.
  6. Payan HM, Gilbert EF: Mesenteric cyst-ovarian implant syndrome. Arch Pathol Lab Med 1987, 111(3):282-4.
Citation

Panchbudhe SA, Parulekar SV. Mesentric Cyst Masquerading As An Ovarian Cyst. JPGO. 2018 Vol 5 No. 10. Available from: http://www.jpgo.org/2018/10/mesentric-cyst-masquerading-as-ovarian.html

Papillary Hidradenoma Of Vulva

Author Information

Swathi HV*, Thakur HS**, Kothari K***, Samant PY****
(* Junior Resident, ** Assistant Professor, **** Additional Professor, Department of Obstetrics and Gynecology, *** Associate Professor, Department of Pathology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Vulvar papillary hidradenoma arises from specialized anogenital apocrine glands in and around the intralabial sulcus. These are benign lesions, which present as slow growing, circumscribed cystic masses especially in reproductive age. Here we discuss a case of papillary hidradenoma of vulva.

Introduction

Vulvar lesions are classified as per the tissue of origin. These can be hidradenoma, syringoma, condyloma acuminatum and acrochordon which are of epithelial origin; leiomyoma, lipoma, fibroma, neurofibroma and granular cell tumor which are of mesenchymal origin; and hemangioma and lymphangioma which are of vascular origin. Glandular blockage causes Bartholin’s cysts. Rare lesions like endometriosis can also occur.  It is important to distinguish a benign lesion like hidradenoma from a rare hidradenocarcinoma or even an aggressive malignancy like merkel cell cancers for appropriate treatment, follow up and counseling of the patient.[1]

Case Report

A 35 year old lady presented to the gynecology outpatient with complaints of a painless, gradually enlarging swelling in the vulvar region since 2 months. It was not associated with any discharge or skin changes. There was no history of any urinary complaints.  Clinical examination revealed a 2 x 2 x 3 cm para clitoral cystic non-tender swelling with smooth surface (figure 1).

Figure 1. Image of vulval swelling.

Figure 2. Image of excised mass.
Provisional diagnosis of lipoma was made and she was posted for excision of the cyst. Preoperative investigations including pap smear were normal except that she was incidentally found to be VDRL positive with a titre of 1: 16. After completing three doses of benzathine penicillin 2.4 MU, each dose given one week apart, excision of the para clitoral cyst was done under general anesthesia. A subdermal hemorrhagic cyst of 2 x 2 x 3 cm was separated by sharp and blunt dissection and removed. (figure 2) The incision was closed by polyglactin no 1-0. She was discharged on oral antibiotic and anti-inflammatory medication, local antiseptic cream and was adviced personal hygiene. Histopathological examination revealed a diagnosis of hidradenoma papilliferum, a benign tumor of apocrine sweat glands. (figure 3) At follow up, the wound was well healed. She was explained the benign nature of the disease and a rare chance of recurrence. 

Figure 3. Histopathology image with green arrows pointing to papillae lined by bilayered epithelium with uniform epithelial and myoepithelial cells. Yellow arrow points to epithelium showing apocrine snouts and decapitation secretions.

Discussion

The term hidradenoma denotes tumor of the sweat gland. Papillary hidradenoma is a rare, benign, cystic, sweat gland tumor. This has originally been reported in adult Caucasian women. It arises from the anogenital glands, with apocrine differentiation. Estrogen and progesterone receptors are present in anogenital sweat glands and are markers to differentiate these glands from other usual sweat glands.[2] Due to estrogen and progesterone receptor positivity, it is believed that hormonal stimulation may play a role in the pathogenesis of hidradenomas in women.[2,3] Our patient did not have any hormonal abnormality.
Androgen receptor positivity in some papillary hidradenomas is documented in luminal cells. This bears similarity with breast proliferative disorders. Hence it is considered that hidradenomas grow in specialized anogenital glands around the intralabial sulcus, which is located in the mammary line. These tumors are mostly single, small in size and do not cause symptoms.  Most of the lesions occur in the labia majora, appearing as a raised mass. It usually takes the color of the overlying skin. The incidence of multiple lesions is reported to be around 5%.[4] Our patient had a swelling with typical description, as described in literature and had only a single swelling.
Ectopic papillary hidradenoma is slightly more frequent in women, and can be seen in any age group.[3]  Ectopic papillary hidradenoma can occur in any location including head and neck region, extremities, external ear, upper eyelid and chest wall. Differential diagnoses includes those conditions mentioned above but rarely, a malignancy.[1] Our patient did not have any other similar swellings.

Vazmitel et al reported a case of ductal carcinoma in-situ arising in papillary hidradenoma, with human papilloma virus 16 association.[5] Kazakov et al in their study of 18 cases found human papilloma viral particles in four cases. Some cases had multiple strains.[6] However, a causative association has not been established. In our patient, HPV was not tested. For treatment, simple surgical excision is adequate. When there is coexisting Bartholin’s cyst, wide surgical excision may be considered. A careful follow-up is recommended since the recurrence rate is not clearly known.[4] There are no reliable prognostic markers for hidradenomas at present.[7]

Conclusion

Papillary hidradenoma is a benign slow growing anogenital tumor. It should be included in the differential diagnosis of a vulval swelling. Surgical excision and regular follow-up are recommended.

References
  1. Horbelt D, Delmore J. Benign Neoplasms of the Vulva. Glob Libr Women's Med.  2008. Available from: https://www.glowm.com/section_view/heading/Benign%20Neoplasms%20of%20the%20Vulva/item/4
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Citation

Swathi HV, Thakur HS, Kothari K, Samant PY. Papillary Hidradenoma Of Vulva. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/papillary-hidradenoma-of-vulva.html

Paraovarian Cyst Of 36 Weeks Size Managed Laparoscopically

Author Information

Shah NH*, Paranjpe SH**, Gosalia K***
(* Consulting Gynecologist/Obstetrician, Hon. Endosopic Surgeon Wadia Hospital & Railway Hospital, ** Director, Velankar Hospital & Paranjpe Maternity Home, *** Consulting Gynecologist/ Obstetrician, Vardann Hospital, Mumbai, India.)

Abstract

Paraovarian cysts contribute to about 10 % of all adnexal masses. They are usually benign and small cysts. Very rarely, they can grow up to large sizes.  Here we present a case of a 21 year old woman with a paraovarian cyst, reaching up to the xiphisternum, about 36 weeks in size, that was managed laparoscopically.

Introduction

The lining of the peritoneal mesothelium or remnants of mesonephric or paramesonephric tissue are the possible origin of the paraovarian cysts.[1] Usually, microscopy shows a single layer of columnar or flattened cells. They are fluid filled sacs of usually small size arising beside the ovary but never attached to the ovary. Very few cases have been described in the literature about huge paraovarian cysts. Some paraovarian cysts, owing to its large size may undergo torsion or rupture as a complication.[2] Here we present an unusual case of a paraovarian cyst which grew up to 36 weeks size and was excised laparoscopically.

Case Report 

A 21 year old unmarried woman presented to the outpatient department with a slowly increasing abdominal swelling since about one year. She was unaware of any abnormal pathology and was undergoing weight loss measures for the same. Abdominal swelling was associated with some non-specific symptoms like pain in the right hypochodrium and occasional dysmenorrhea. She had no history of colicky pain, vomiting or other gastric disturbances. There was no weight loss or weakness. Menstrual cycles were regular, with dysmenorrhea.
Systemic examination was normal. Abdominal examination showed large tense, smooth cystic mass arising from the pelvis reaching up to the xiphisternum (figure 1). It was tense, non-mobile and non-tender. Ultrasonography and MRI confirmed the findings of a large cystic swelling arising from pelvis reaching upto the xiphisternum occupying the whole abdomen. Ovaries were visualized and were normal in size and shape. Uterus was also normal in size and shape. Preoperative investigations and tumor markers (CA-125 value and beta-hCG value) were normal. A laparoscopic approach was chosen due to the benign nature of the lesion.

Figure 1. Preoperative image showing the size of the cyst .
Figure 2. Image showing cyst with clear fluid.
Through a supraumbilical approach, a 10 mm trocar was directly inserted into the peritoneal cavity. The cyst showed clear fluid (Figure 2). The trocar and cannula were then inserted into the cyst, and a suction device was used to drain the cyst fluid.  The volume was around 2.5 liters (figure 3).
Figure 3. Image depicting amount of fluid drained.
After draining, the suction was removed and laparoscope was re-inserted, and lateral ports were placed. The drained cyst was easily visualized and its origin was traced to the paraovarian region separate from the ovary (figure 4). The cyst wall was excised and removed from the lateral port. Uterus was visualized and was in normal size. Both ovaries were seen normal. Unilateral salpingectomy had to be done as the tube was overtly stretched and elongated (figure 5). There was significant and appreciable change in the external contour of the abdomen (figure 6). The specimen obtained (figure 7) was sent for histopathological examination and it confirmed a paraovarian cyst. Postoperative course was uneventful.

Figure 4. Image showing normal uterus, normal left adnexae, normal right ovary and right paraovarian cyst wall.

Figure 5. Image showing the view of pelvis after cystectomy.

Figure 6. Image showing decompressed abdomen with lateral ports.

Figure 7. Final specimen after removal.

Discussion

Most paraovarian cysts are asymptomatic and are incidental findings, but rarely they give rise to clinical symptoms due to their enlargement or torsion. Hydronephrosis is also a complication of large cysts which is caused by the pressure on the ureters.[3] Our patient did not have any urinary complaints.
These cysts can be examined by abdominal palpation, vaginal bimanual examination or rectal examination. Ultrasound is most commonly used to diagnose the mass and its location. But rarely in such large masses the point of origin cannot be determined. Hence to clarify the diagnosis, sometimes computerized tomography or MRI are used. MRI is preferred over CT as to prevent radiation to the ovaries especially in a young girl. In our patient, since the cyst was visualized separately from both the ovaries, it was reasonably ascertained that it is of paraovarian origin.
Traditionally, midline vertical incision is used to remove such large cysts. However, we chose a laparoscopic route since there has been positive experience with minimally invasive techniques in abdominal procedures.[4] Due to direct visualization, the laparoscopic differentiation of paraovarian cysts from that of ovarian cysts is also very easy.  Oophrectomy or salpingectomy may sometimes be required.[5,6] In our case, salpingectomy was required.

Conclusion

Paraovarian cysts can be huge, sometimes reaching upto xiphisterum. Successful removal with laparoscopic methods is possible.

References
  1. Athey PA, Cooper NB. Sonographic features of paraovarian cysts. Am J Rad. 1985; 144:83–86.
  2. Lurie S, Golan A, Glezerman M. Adnexal torsion with a paraovarian cyst in a teenage girl. J Am Assoc Gynecol Laparosc. 2001; 8(4): 597–9.
  3. Leanza V, Cassaro N, Di Prima F. Hydronephrosis and vault prolapse. Urogynaecologia International Journal.2009;23(2):129–132.
  4. Barbaros U, Dinççağ A, Sümer A, Vecchio R, Rusello D, Randazzo V, Issever H, Avci C. Prospective randomized comparison of clinical results between hand-assisted laparoscopic and open splenectomies. Surg Endosc. 2010;24(1):25–32.
  5. Azzena A, Quintieri F, Salmaso R. A voluminous paraovarian cyst. Case report. Clin Exp Obstet Gynecol.1994;21(4):249–52.
  6. Darwish AM, Amin AF, Mohammad SA. Laparoscopic management of paratubal and paraovarian cysts. JSLS. 2003 Apr-Jun;7(2):101-6.
Citation

Shah NH, Paranjpe SH, Gosalia K. Paraovarian Cyst Of 36 Weeks Size Managed Laparoscopically. JPGO 2018. Volume 5 No.2. Available from: http://www.jpgo.org/2018/10/paraovarian-cyst-of-36-weeks-size.html