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Volume 5 Number 12

Editorial

Madhva Prasad

The placenta is an exciting organ. No other organ is as temporary as the placenta. It serves as a pivotal part of the pregnancy. It leads to major problems to the fetus, even if there is slight alteration in the location or function. Despite the excessive dependence and overriding importance of this tissue, not many really bother about what happens to it once delivery takes place. The placenta is just discarded. Its life is akin to a soldier’s. Placentae, like soldiers are “disposable heroes”. Way back in 1986, the American based alternative music group-Metallica, wrote a song titled “Disposable heroes”, themed on the lives of soldiers. Writing the lyrics verbatim here, the song goes “…I was born for dying. Life planned out before my birth, nothing could I say. Had no chance to see myself, molded day by day. Looking back I realize, nothing have I done. Left to die..”  If the placenta could speak, these words appear absolutely appropriate too.

Wells et al (2014) have explored Indian traditional birth practices and had identified that one unique feature of the birthing process was the importance attached to the mode of disposal of the placenta. In some communities, it has been accorded a good deal of respect and is given befitting burial also.
Burns et al (2014) have explored the importance of the placenta in an Australian context. An in-depth interview of 51 patients revealed that they did acknowledge that after births have to be given more importance than what is usually given. They identified three methods which are commonly practiced.
First is placentophagy. It is the consumption of the placenta by the members of the family, and thereby imbibing it into each one’s body.

Second is burial. The placenta is cleaned and is buried in a safe spot deep under the soil, and saplings or seeds are planted above it. The growing tree is considered a life long symbolism of the placenta, which supported their progeny during the time of need.

Third is the lotus birth.  Clamping and cutting of the umbilical cord is done almost immediately after birth. The established medical methods are “delayed” cord clamping and “early” cord clamping. The delayed clamping is by far the most advantageous (Fogarty 2018).   However, an entity called “physiological cord clamping” method – synonymous with Lotus birth has been reported. 
Lotus birth is an approach wherein the umbilical cord is not cut. The placenta is expelled spontaneously and its complete expulsion is ensured. The placenta is wrapped in a clean sterile moist cloth and periodically, moistness is ensured by irrigating with sterile water and salt is poured. Sterility is maintained at all times. It has been observed consistently that by the 5th or 6th day of postnatal life, the umbilical cord separates on its own. No major health issues had been observed in the neonates undergoing lotus birth. (Zinsser, 2018) Whether it can be adopted in all settings is yet to be looked into.

Viewed from an Indian mythological context, the Lotus birth seems natural. The human body form attached to a placenta through a cord is quite popular, with one ancient text stating that the universe took physical form from the Umbilicus of the Lord (Nabhyaa aaseedantariksham) and the term “One with lotus like umbilicus” used as a common description of a commonly worshipped deity (Padmanaabha).

Coming back to more modern versions, the term “cut the umbilical cord” can refer to moving out of house thereby ending of a long relationship between a young adult and a supportive parent. In medical education terms, it can refer to the completion of either an undergraduate/ postgraduate medical course. The student is akin to the fetus. The medical college and its faculty are akin to the placenta, serving as the most critical part for growth. The medical education which the student resident received is akin to the nutrition received. However, every trainee doctor has to eventually become independent, move forth and enter the world to serve humanity.
It is envisioned and encouraged that just like there is so much reverence attached to the placenta, those providing medical education will also be respected and revered by all passing-out doctors for their entire careers. 
With this, we bring to you the journal’s last issue for this year. We hope that you enjoyed reading the articles of this year.

Breast Cancer Metastasis In Therapeutic Salpingo-Oophorectomy Specimen

Author Information

Parulekar SV*, Kothari K**.
(* Professor and Head, Obstetrics and Gynecology, ** Associate Professor of Pathology, Seth G S Medical College & K E M Hospital, Mumbai, India.)

Abstract

Patients with breast cancer with estrogen receptor  ≥1% need to be treated with suppression or ablation of ovarian function. Suppression can be achieved with tamoxifen. Other endocrine therapy options include aromatase inhibitors ( letrozole, anastrazole and exemestane) and progestogens.  Ablation is achieved with GnRH analogs or therapeutic oophorectomy. We report a case of metastatic breat cancer who was found to have left ovarian metastasis when there was no suspicion of the same on clinical examination as well as on inspection of the ovary during her operation.

Introduction

Premonopausal women with hormone-sensitive breast cancer benefit from endocrine therapy.[1]  Salpingo-oophorectomy is more effective than tamoxifen as adjuvant therapy.[2] Such salpingo-oophorectomy is distinct from that performed as a debulking procedure in case of ovarian metastases from a breast cancer. We present an unusual case of microscopic ovarian metastasis in the left ovary of a case of metastatic breast cancer.

Case Report

A 35 year old woman, para 2 living 2, was referred to us from an oncology center for bilateral salpingo-oophorectomy in view of metastatic breast carcinoma which was being treated with combination chemotherapy. She had menarche at the age of 13 years. Her past and present menstrual cycles were every 28-32 days, regular and with moderate flow. Her last menstrual period had been 2 weeks ago. She had two normal deliveries, 9 and 6 years ago. She had successfully breastfed both babies for a year each. She was a known case of breast carcinoma. She was receiving cyclophosphamide-methotrexate-5-fluorouracil chemotherapy. Being estrogen receptor positive, she was advised to undergo  bilateral salpingo-oophorectomy.

Her general condition was fair. Her general and systemic examination revealed no abnormality clinically. Abdominal and bimanual pelvic examination showed normal findings. Her liver was mildly enlarged. Chest radiograph showed small metastases bilaterally. Ultrasonography of the abdomen showed mild hepatomegaly, small calcified granulomas in the liver and normal intraabdominal and pelvic organs. Her liver and renal function tests showed normal results. Investigations for fitness for anesthesia showed normal results.

The patient opted for minilaparotomy for  bilateral salpingo-oophorectomy rather than laparoscopy. It was performed under spinal anesthesia. Both fallopian tubes and the right ovary were normal. The left ovary was mildly enlarged, but otherwise normal in external appearance. Its cut surface showed a yellowish nodule measuring 0.8 cm in diameter.

The patient made an uneventful recovery from the surgery. Histopathological examination of the surgical speciment showed normal fallopian tubes and  right ovary, and the left ovary showed infiltrating ductal carcinoma. The patient went back to the oncology center for continuation of her treatment.


Figure 1. Histopathology: Scan - Normal ovarian parenchyma in the periphery with a circumscribed nodule ( HE x40).


Figure 2. Histopathology: Low - Tumor cells arranged in nests, cords and small tubules amidst cellular stroma (HE x 100).

Figure 3. High:  Tumor cells have high nuclear:cytoplasmic ratio, scanty cytoplasm and occasional prominent nucleoli (HE x400).

Discussion

George Thomas Beatson ( 1895) first performed a bilateral oophorectomy for breast cancer. He reported three patients treated in this manner.[3] The addition of ovarian ablation or suppression to any systemic therapy for breast carcinoma is associated with a significant reduction in recurrence and death.[4] It improves both disease-free survival and overall survival of premenopausal women as compared to treatment without adjuvant therapy.[5.6] Premenopausal women with estrogen receptors in the tumor benefit from ovarian suppression by both GnRH analogs and  bilateral salpingo-oophorectomy. The latter has the advantages of much lower cost and absence of adverse effects of GnRH analogs. Chemotherapy can cause ovarian failure, but that effect depends on the patient's age - younger women tend to develop permanent ovarian failure less often. Thus one cannot rely on that effect and must achieve ovarian suppression or ablation.

Ovarian metastases are found in 10-20% autopsies done in cases of ovarian carcinomas. They are found in 30% cases of therapeutic oophorectomy specimens of breast carcinoma cases.[7,8,9] Ovarian metastasis from breast carcinoma can appear many years after the initial diagnosis, the median time being five years.[10] Early diagnosis and treatment of ovarian metastases is important in improving survival of these cases.[11] These can be detected when they cause gross lesions. In the case presented, the metastasis was not detectable clinically and would have been missed had bilateral salpingo-oophorectomy not been performed therapeutically to aid treatment of the breast carcinoma. Thus it was not only curious to find it, but was also in patient's interest – it served the purpose of ovarian ablation and also reduced the tumor burden so that chemotherapy would work better.[12] It also improved prognosis as the metastasis would have been detected later when it had grown enough to be detectable grossly on imaging. Usually metastatic tumors from breast carcinoma are small in size, as in our case.[13]

References
  1. Aebi S, Davidson T, Gruber G, Cardoso F; ESMO Guidelines Working Group. Primary breast cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2011 Sep;22 Suppl 6:vi12-24.
  2. American College of Obstetricians and Gynecologists. Elective and risk-reducing salpingo-oophorectomy. Washington (DC): ACOG; 2008 Jan. (ACOG practice bulletin; no. 89). Obstet Gynecol 2008 Jan;111(1):231-41.
  3. Love RR, Philips J. Oophorectomy for breast cancer: history revisited. J Natl Cancer Inst. 2002 Oct 2; 94(19):1433-4.
  4. LHRH-agonists in Early Breast Cancer Overview group., Cuzick J, Ambroisine L, Davidson N, Jakesz R, Kaufmann M, Regan M, Sainsbury R. Use of luteinising-hormone-releasing hormone agonists as adjuvant treatment in premenopausal patients with hormone-receptor-positive breast cancer: a meta-analysis of individual patient data from randomised adjuvant trials. Lancet. 2007 May 19; 369(9574):1711-23.
  5. Early Breast Cancer Trialists’ Collaborative Group. Effects of adjuvant tamoxifen and of cytotoxic therapy on mortality in early breast cancer: an overview of 61 randomized trials among 28,896 women. N Engl J Med. 1988;319:1681–1692. doi: 10.1056/NEJM198812293192601.
  6. Early Breast Cancer Trialists’ Collaborative Group. Effects of chemotherapy and hormonal therapy for early breast cancer on recurrence and 15-year survival: an overview of the randomised trials. Lancet. 2005;365:1687–1717.
  7. Petru E, Pickel H, Heydarfadai M, Lahousen M, Haas J, Schaider H, et al. Nongenital cancers metastatic to ovary. Gynecol Oncol. 1992;44:83–86.
  8. Mazur MT, Hsueh S, Gersell DJ. Metastases to the female genital tract. Analysis of 325 cases. Cancer. 1984;53:1978–1984.
  9. Hann LE, Lui DM, Shi W, Bach AM, Selland DL, Castiel M. Adnexal masses in women with breast cancer: US findings with clinical and histopathologic correlation. Radiology 2000;216:242–247.
  10. Bigorie V, Morice P, Duvillard P, Antoine M, Cortez A, Flejou JF, et al. Ovarian metastases from breast cancer: report of 29 cases. Cancer 2010;116:799–804.
  11. Jiang R, Tang J, Cheng X, Zang RY. Surgical treatment for patients with different origins of Krukenberg tumors: outcomes and prognostic factors. Eur J Surg Oncol 2009;35:92–97.
  12. Kim WY, Kim TJ, Kim SE, Lee JW, Lee JH, Kim BG, et al. The role of cytoreductive surgery for non-genital tract metastatic tumors to the ovaries. Eur J Obstet Gynecol Reprod Biol 2010;149:97–101.
  13. Koyama T, Mikami Y, Saga T, Tamai K, Togashi K. Secondary ovarian tumors: spectrum of CT and MR features with pathologic correlation. Abdom Imaging 2007;32:784–795.
Citation

Parulekar SV, Kothari K. Breast Cancer Metastasis In Therapeutic Salpingo-Oophorectomy Specimen. JPGO 2018. Vol 5 No. 12. Available from: https://www.jpgo.org/2018/12/breast-cancer-metastasis-in-therapeutic.html

An Unusual Case Of Cervical Pseudo Broad Ligament Leiomyoma

Author Information

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

Abstract
Cervical leiomyomas are rare tumors accounting for 2% of all leiomyomas. We present a case of a large posterior cervical pseudo broad ligament leiomyoma where myomectomy was performed by approaching it by opening the anterior leaf of the right broad ligament.

Introduction
Cervical leiomyomas account for 2% of all leiomyomas and they can originate from either supra-vaginal or vaginal portion of cervix.[1] The former may be anterior, posterior, lateral or central depending on their site of origin. It projects into the vagina if it arises from the portio vaginalis. A leiomyoma arising from lateral aspect of the supravaginal cervix can grow into the broad ligament of the same side, producing a pseudobroad ligament leiomyoma.[2] We present an unusual case in which the leiomyoma arose from posterior part of the supravaginal cervix and grew into the right broad ligament.

Case Report
A 35 years old woman, married for 15 years, para 1 living 1, presented to our outpatient clinic with complaints of pain in the lower abdomen for 2 months. She had no menstrual complaints. She had one male child 12 years back and was delivered by lower segment cesarean section. She was diagnosed to have hypertension 1 year ago and was on Amlodipine 5 mg PO qd. She had no other surgery done in the past. On general examination her general condition was fair and vital parameters were in normal limits. On per abdomen examination there was a infraumbilical vertical midline scar of previous lower segment cesarean section. The abdomen was soft with no tenderness, guarding or rigidity. On per speculum examination the cervix was high up and healthy. On bimanual examination the uterus was 14 to 16 weeks' size and firm. It had a mass of 10 ×8 cm in the posterior wall. Her Pap smear and investigations for fitness for anesthesia gave normal results. On ultrasonography of the pelvis  the uterus measured 13 cm in length and showed a large, ovoid, heterogeneous, mildly hypoechoic 8×7×5.9 cm sized leiomyoma in central and predominately posterior location in the lower body and cervix, with mild increase in intra- and peripheral vascularity. Both ovaries were normal. In view of above findings and patient's desire of retaining her fertility, a myomectomy was performed through an infraumbilical midline incision under spinal anesthesia. Dense adhesions were encountered between the anterior abdominal wall and omentum, which were separated by sharp dissection. The uterus was of normal size. The right fallopian tube and both ovaries were normal. The left cornual portion of the uterus and anterior aspect of the left adnexal structures were adherent to the anterior abdominal wall. These adhesions were separated to facilitate mobility of the uterus. The left fallopian tube couild not be located in the adhesions. A posterior cervical leiomyoma of 10×8 cm was felt posteriorly and was growing into the right broad ligament, stretching the posterior leaf of the broad ligament and reaching the pelvic floor inferiorly. The right ureter was found to be of normal size, away from the leiomyoma. The right round ligament was divided between two hemostats in its central portion and the hemostats were replaced by transfixion ligatures of No. 1 polyglactin. The upper surface of the pseudocapsule of the leiomyoma was exposed by dissecting the peritoneum of the broad ligament away from it. The pseudocapsule was cut longitudinally in its upper part. The leiomyoma was dissected off the pseudocapsule and enucleated after clamping and dividing a leash of blood vessels entering its lower portion. Hemostasis was achieved in the bed of the leiomyoma, redundant part of the pseudo-capsule was excised, the dead space occluded with purse-string sutures at the bottom of the cavity and then the pseudocapsule was closed with sutures of No. 1 polyglactin. The anterior leaf of the right broad ligament was reconstructed by a continuous suture of No. 1-0 polyglactin after approximating cut ends of the right round ligament. The patient made an uneventful recovery. Histopathological examination of the tumor confirmed the diagnosis of a leimyoma.


Figure 1. Right round ligament has been divided (arrows) to expose the leiomyoma (green arrows) covered by a number of blood vessels. U: uterus, L: posterior surface of the leiomyoma.


Figure 2. The upper surface of the leiomyoma (L) is exposed.


Figure 3. The superior part of the pseudo-capsule is divided (arrows) to expose the leiomyoma (L) within it.


Figure 4. The leiomyoma (L) is being enucleated from within its pseudocapsule.


Figure 5. The leiomyoma has been enucleated. The bed of the leiomyoma is seen (arrows).


Figure 6. End result. The suture line in the right broad ligament is seen.
Discussion

The presentation of cervical leiomyoma usually depend on the location. It can present with symptoms like menstrual irregularities, constipation, frequency of urination, urinary retention, dyspareunia and postcoital bleeding.[3,4] A large size of a cervical leiomyoma can cause anatomical distortion of ureter, bladder and uterine vessels, thereby increasing the risk of injury to these structures.[5] The progression in the number, size and growth of cervical leiomyomas is unpredictable. They usually do not become become cancerous. They are less likely to shrink  on their own until after menopause.

In our case it was a posterior cervical leiomyoma which was arising from the supravaginal part of the cervix. Two unusual features of this leiomyoma were its growth into the right broad ligament and stretching of only the posterior leaf of right broad ligament over it. Leiomyomas arising from the posterior part of the supravaginal cervix usually do not grow into the broad ligament which lies lateral to it. Once a leiomyoma grows in the broad ligament, it stretches both leaves of the broad ligament over it. Both of these features were absent in our case. Due to this unusual location the ureter was expected to lie not only lateral to the leiomyoma but also on the top of it. Luckily it was on the lateral pelvic wall in its upper pelvic part and below the leiomyoma in its lower pelvic wall. This leiomyoma was reached by opening the anterior leaflet of right broad ligament by dividing the right round ligament, which was the safest approach for avoiding the right ureter. Adequate exposure of the leiomyoma was possible without injuring the right fallopian tube when the broad ligament was dissected off its upper surface of the leiomyoma, displacing the right fallopian tube posteriorly. Dissection within the pseudocapsule was the key principle used to avoid ureteric injury.

References
  1. Bhatla N. Tumors of the corpus uteri. In: Jeffcoates Principles of Gynaecology. 6th ed. London: Arnold Publisher; 2001. p. 470.
  2. Khan AT, Shehmar M, Gupta JK. Uterine fibroids: current perspectives. Int J Women's Health. 2014; 6: 95-114.
  3. Buttram VC Jr., Reiter RC. Uterine leiomyomata: etiology, symptomatology, and management. Fertil Steril 1981;36:433
  4. Parker WH. Uterine myomas: An overview of development, clinical features, and management. Obstet Gynecol 2005;105:216-7.
  5. Rajput DA, Gedam JK. Broad ligament fibroid: A case series. IJSS Case Rep Rev 2015;1:8-11.
Citation

Panchbudhe SA, Parulekar SV. A Unusual Case Of Cervical Pseudo Broad Ligament Leiomyoma. JPGO 2018. Vol 5 No. 12. Available from: https://www.jpgo.org/2018/12/a-unusual-case-of-cervical-pseudo-broad.html

Hypersensitivity Reaction With Methylene Blue: A Rare Case

Author Information

Khadke B*, Koshewara P**, Gupta A S***

(* Junior Resident, ** Senior Resident, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Methylene blue associated anaphylactic reaction is rare. We are presenting a 25 year old nulliparous patient, who suffered from acute hypersensitivity reaction with 1% methylene blue dye during tubal patency test. Importance of awareness about the entity, early detection of symptoms, its management, and its possible outcomes are presented here.

Introduction

Methylene blue is methylthioninium chloride. It is commonly used as a dye in diagnostic procedures such as detection of fistula and for patency of fallopian tubes; it can also produce methemoglobinemia in susceptible individuals.[1] Methylene blue dye hypersensitivity reaction is commonly associated with dyspnea, precordial pain, restlessness, and apprehension, while tremor, hemolytic anemia, pulmonary edema, and even death can be associated with its anaphylactic reaction. Patients suffering from Glucose-6-phosphate dehydrogenase (G6PD) deficiency may produce methemoglobinemia when methylene blue dye is used because of their inability to reduce methylene blue to leukomethylene blue, hence use of methylene blue dye should be avoided in such patients.[2]

Case Report

A 25 year old nulliparous lady, married for 5 years presented to outpatient department with complaints of something coming out per vaginum for 2 years. She had one abortion two years prior and was anxious to conceive. The mass per vaginum aggravated on coughing, sneezing and straining at stools. Her medical & surgical history was unremarkable. On further evaluation, there was no history of asthma, allergies or any drug hypersensitivity reaction. On examination, her vital parameters were stable, systemic examination was within normal limits and clinical examination was suggestive of second degree uterine prolapse. Investigations were sent and anesthetic evaluation was performed. She was scheduled for abdominal sling surgery (Khanna’s sling) and assessment for infertility.
As is usual practice, prior to the day of surgery, a test dose of injection amoxycillin and clavulanic acid and injection xylocaine was performed and there was no allergic reaction observed. For surgical prophylaxis injection amoxycillin and clavulanic acid 1.2 g, injection metronidazole 500 mg and injection gentamycin 80 mg intravenously were administered. Spinal anesthesia was induced with injection bupivacaine that was given for subarachnoid block, and intravenously injections of 1 mg midazolam and 30 mg fentanyl were given for sedation.
Tubal patency evaluation was done through a trans cervical injection of 40 ml of methylene blue dye via a Leech Wilkinson’s cannula. The methylene blue dye was injected transcervically prior to the preparation of the abdomen and thighs with povidone iodine. To start with she was catheterized and then the vagina was painted with povidone iodine and as it was a second degree prolapse the Leech Wilkinson’s cannula was easily attached and transcervically the methylene blue dye 40 ml was injected. Presence of the dye in the peritoneal cavity and blue discoloration of the entire fallopian tubes seen on subsequent opening of the peritoneal cavity was the end point to be used to determine tubal patency. Soon after transcervical injection of methylene blue dye eruption of rash and urticarial wheals on the abdomen and thighs were noted. A possible anaphylactic reaction alert was sounded. At that point of time her heart rate was 92 beats per minute and blood pressure was 100/60 mm Hg, and her chest was clear bilaterally. Her oxygen saturation was 100% on nasal oxygen. Immediately, intravenously hydrocortisone 100 mg and injection dexamethasone 4 mg were administered. This resulted in slight reduction of the erythematous wheals over the abdomen. Other parts of the body were also examined, and no other allergic site was found. After 15 minutes she was reevaluated. There were no further allergic phenomena and her vitals parameters remained stable. Abdomen from xiphisternum to the knee was scrubbed, prepared and painted with povidone iodine scrub and solution after she stabilized from her urticaria. Khanna’s sling surgery was performed uneventfully and methylene blue dye indicating patent tubes was seen in the pelvic and peritoneum cavity. Her subsequent post-operative period was uneventful.

Discussion

Methylene blue reaction is an IgE mediated reaction.[3] The immediate manifestations of IgE mediated reaction include urticaria, and rashes, accounting for more than 70% of the cases. Skin manifestations are the most common manifestations.[4] Spillage into the peritoneal cavity may have resulted in the urticaria occurring on the abdominal wall. Lymphatic and intravascular extravasation fortunately did not occur (uterus and fallopian tubes did not show any evidence of extravasation of the dye ) or was minimal or else a more severe reaction was possible. In this case, she developed an allergic reaction, which was unlikely due to any intravenous medication, because the urticarial eruptions noticed were localized to the abdomen and thighs only and were not generalized. Also, antibiotic and anesthetic drugs sensitivity test were done one day prior to the surgery; and these had tested negative for any allergic reactions. The time period between injection of methylene blue and the occurrence of reaction, and the location of the rash indicate the inciting agent to be methylene blue. Abdomen from xiphisternum to the knee was scrubbed, prepared and painted after the patient stabilized from her urticaria hence this excludes the reaction caused by local antiseptics. Even though very few cases of allergic reactions are observed with antiseptics some authors have reported anaphylactic reactions with povidone iodine as well as with chlorhexidine and these are also mentioned in the US FDA safety guidelines.[5,6] Prompt identification and systemic medications (corticosteroids) probably led to prevention of a severe reaction, or systemic effects.
A similar case was reported by Milio et al, wherein a 30 year old woman with primary infertility who under went laparoscopic chromopertubation with methylene blue dye  developed fatal pulmonary edema.[1] Another case of chromopertubation with use of methylene blue dye was reported by Bilgin et al in a woman with G6PD deficiency which precipitated the methemoglobinemia.[2]
Dewachter et al stated that, severe immunoglobulin E-mediated hypersensitivity reaction is associated with the use of 1% methylene blue for detection of tubal patency even under anesthesia. This requires the need for systematic investigation of potentially allergenic drugs and substances administered during the perioperative period.[3] Breast cancer surgery had been abandoned when a woman developed angioedema and urticaria on injection of methylene blue dye to detect the sentinel lymph node.[7] In our case she also developed urticaria and rash, but it was manageable with antihistaminic drug and steroid and surgery could be completed successfully.
In 1996, Trikha et al observed that, injection of methylene blue resulted in development of pulmonary oedema in an ASA grade-1 patient with change in patient oxygen saturation.[8] However, fortunately our case was non-severe and respiratory system was unaffected throughout the procedure. Similar case of pulmonary edema following laparoscopic chromopertubation with methylene blue was reported by Cm V et al.[9] The cardinal allergic sign and symptoms of tachypnoea and hypotension were found, which required immediate intervention with steroids, inotropes and intensive unit care while our patient was stable after administration of steroids and did not require any intensive care.
According to standardized procedures as recommended by the French Society of Anesthesiology and Critical Care Medicine, sensitivity test with methylene blue can be done preoperatively and to confirm methylene blue hypersensitivity. In vitro leucocytes histamine tests can also be done for the same purpose.[3] As the average incidence of reactions to blue colored dyes can range from 7 patients in 10, 000 to 2.7 patients per 100 it is difficult to say whether a policy/ guideline to do sensitivity test for all patients who will need to use these vital blue dyes be recommended. However, methylene blue has the best safety profile amongst all the vital blue dyes.

Conclusion

Although methylene blue dye is used commonly in many diagnostic procedures, hypersensitivity reaction to methylene blue should always be kept in to mind. Although it is rare, awareness about its occurrence is key to early diagnosis and management.

References
  1. Millo T, Misra R, Girdhar S, Rautji R, Lalwani S, Dogra TD. Fatal pulmonary oedema following laparoscopic chromopertubation. Natl Med J India. 2006;19(2):78-79.
  2. Bilgin H, Özcan B, Bilgin T. Methemoglobinemia induced by methylene blue perturbation during laparoscopy. Acta Anaesthesiol Scand. 1998;42(5):594-595.
  3. Dewachter P, Mouton-Faivre C, Tréchot P, Lleu JC, Mertes PM. Severe anaphylactic shock with methylene blue instillation. Anesth Analg 2005;101(1):149-150.
  4. Katayama H, Yamaguchi K, Kozuka T, Takashima T, Seez P, Matsuura K. Adverse reactions to ionic and nonionic contrast media. A report from the Japanese Committee on the Safety of Contrast Media. Radiology 1990;175(3):621–8
  5. Rahimi S, Lazarou G. Late-onset allergic reaction to povidone-iodine resulting in vulvar edema and urinary retention. Obstet Gynecol. 2010 Aug;116 Suppl 2:562-4.
  6. Abdallah C. Perioperative chlorhexidine allergy: Is it serious? J Anaesthesiol Clin Pharmacol. 2015; 31(2): 152–154.
  7. Hamelin A, Vial-Dupuy A, Lebrun-Vignes B, Francès C, Soria A, Barete S. [Acute blue urticaria following subcutaneous injection of patent blue dye]. Ann Dermatol Venereol. 2015;142(11): 670-674.
  8. Trikha A, Mohan V, Kashyap L, Saxena A. Pulmonary edema following intrauterine methylene blue injection. Acta Anaesthesiol Scand. 1996;40(3):382-384.
  9. Cm V, Joshi S D, Yr M. A Rare Case of Delayed Pulmonary Oedema due to Methemoglobinemia Following Laparoscopic Chromopertubation with Methylene blue. J Clin Diagn Res. 2014;8(6):OD05-06.
  10. Bézu C, Coutant C, Salengro A, Daraï E, Rouzier R, Uzan S. Anaphylactic response to blue dye during sentinel lymph node biopsy.Surg Oncol. 2011;20(1):e55-9.
Citation

Khadke B, Koshewara P, Gupta AS. Hypersensitivity reaction with methylene blue: A rare case. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/hypersensitivity-reaction-with.html

A Case Of Pregnancy With Hereditary Spherocytosis

Author Information

Khadke B*, Prasad M**, 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

Hereditary spherocytosis (HS) is a common inherited erythrocytic membranopathy, mostly presenting as hemolytic anemia. Here we are presenting a multigravida woman, a diagnosed case of hereditary spherocytosis requiring multiple blood transfusions in the antenatal period.

Introduction

Hereditary spherocytosis (HS) is a common type of hemolytic anemia. In an unselected population, the frequency is around 1 in 5000. The problem lies in the red cell membrane proteins; spectrin, ankyrin, band 3 protein and protein 4.[1] The abnormal red cell membrane proteins give rise to an unusual susceptibility to lysis. Osmotic fragility is a hallmark of hereditary spherocytosis. Peripheral smear shows spherocytes. Most individuals have mild or only moderate disease. Typical cases present with family history and in 75% of patients, inheritance is autosomal dominant. Folate supplements are adequate for mild cases while splenectomy is required for severe cases. The disease can affect pregnancy in many ways.

Case Report

A 33 year old patient, gravida 3 para 1 living 1 abortion 1, married for 5 years registered in antenatal outpatient department at 9 weeks of gestation with a hemoglobin of 8.5 gm %.  She was diagnosed as a case of hereditary spherocytosis 8 years back. She was icteric. Her mother and brother both were diagnosed cases of HS and both of them had undergone splenectomy. Hematologists were consulted and they advised continuation of 1000 mcg of folic acid and regular monitoring of red blood cell indices.  
Her first pregnancy was a full term vaginal delivery with no history of blood transfusion, and a healthy female child of 3.5 kg was born. Second pregnancy was a spontaneous abortion at the third month of gestation, for which emergency curettage was done. The current pregnancy was a spontaneous conception. At 30 weeks of gestation, she presented with complaints of breathlessness and excessive fatigue. On examination, pallor was present, and pulse was around 110 bpm, with a respiratory rate of 30 cycles per minute. Abdominal examination showed splenomegaly, and a live fetus with no uterine activity. She was admitted to the intensive care unit with severe anemia, and heart failure. Hemoglobin was 2.6 gm % and platelet count was 80,000/ cc and serum bilirubin was elevated. She was stabilized with diuretics, and non-invasive ventilation. The diagnosis of hemolytic crisis was considered and she was transfused with 5 unit packed red cells over ten days. Post transfusion hemoglobin was 7.7 gm %. She was then discharged. She followed up in the antenatal outpatient department once in two weeks, and was otherwise asymptomatic.
At 36 weeks of gestation, she came with pain in abdomen and leaking per vaginum. Abdominal examination showed live fetus with regular heart rate pattern and good uterine activity. Vaginal examination showed 6 cm dilated internal os with a vertex presentation. She progressed spontaneously and delivered a female child of 1.26 kg with APGAR score of 9/10. Neonate was shifted to NICU for weight gain and screening. Mother was given one unit packed red cells peripartum. Postpartum period was otherwise uneventful and she was discharged on day 4. 

Discussion

Our patient did not have an autosomal dominant mode of transmission. Her first child was unaffected by the disease. Pregnancy in HS is usually uneventful.[2,3] However, some specific problems can arise. In our patient, there was one prior term birth and one spontaneous abortion. In the case series by Pajor et al, they had noticed a 68% rate of term birth and 21% rate of spontaneous abortion.[4]
In our patient, there was one episode of hemolytic crisis in the 30th week of gestation, necessitating multiple blood transfusion. Pregnancy is a well known trigger for hemolytic crisis, as shown by HO-Yen et al.[5]
In the case series by Pajor et al, hemolytic crises was seen in 30% of the cases. In our patient, apart from anemia and resultant heart failure, there were no major problems. In the case series by Maberry et al also, the main problem that was encountered was anemia.[6]
Our patient developed cardiac failure. The causes of cardiac failure in spherocytosis can be severe anemia,[7] and rarely due to cardiac hemosiderosis, as described by Fujino et al.[8]
Our patient overcame the hemolytic crisis without requirement for splenectomy. However, two cases of hemolytic crisis necessitating splenectomy have also been described in literature.[9] Brabek et al and Pajor et al [3,4] have mentioned that pregnancies with HS, who have undergone splenectomy have lesser problems compared to those without.
The neonate in our case had intrauterine growth restriction, but no features of hydrops fetalis. Association between hydrops fetalis and spherocytosis has also been described.[10] Though the neonate in our case needed a prolonged NICU stay, overall neonatal outcomes are good, as opined by Maberry et al.[6]

References
  1. Luzzatto L. Hemolytic Anemias and Anemia Due to Acute Blood Loss. In: Longo DL, Kasper DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J, Eds. Harrison’s Principles of Internal Medicine.18th Ed. New York. McGrawHill. 2012. pp 1032-34.
  2. Gallagher PG. The Red Blood Cell Membrane and Its Disorders: Hereditary Spherocytosis, Elliptocytosis, and Related Diseases. In Kaushansky K, Lichtman MA, Beutler E, Kipps TJ, Seligsohn U, Prchal JT. 8th ed. California. McGraw-Hill 2010. pp 897-9.
  3. Brabec V, Cermák J, Petrtýlová K, Jarolím P. [Pregnancy in patients with hereditary spherocytosis]. [Article in Czech] Vnitr Lek. 1999;45(4):220-3
  4. Pajor A, Lehoczky D, Szakács Z. Pregnancy and hereditary spherocytosis. Report of 8 patients and a review. Arch Gynecol Obstet. 1993;253(1):37-42.
  5. Ho-Yen DO. Hereditary spherocytosis presenting in pregnancy. Acta Haematol. 1984;72(1):29-33.
  6. Maberry MC, Mason RA, Cunningham FG, Pritchard JA. Pregnancy complicated by hereditary spherocytosis. Obstet Gynecol. 1992;79(5 (Pt 1)):735-8.
  7. Morita M, Hashizume M, Kanematsu T, Sugimachi K, Makizumi K. Hereditary spherocytosis with congestive heart failure: report of a case. Surg Today. 1993;23(5):458-61.
  8. Fujino T, Inoue S, Katsuki S, Higo T, Ide T, Oda Y, et al. Fatal Cardiac Hemochromatosis in a Patient with Hereditary Spherocytosis. Int Heart J. 2018;59(2):427-30.
  9. Moore A, Sherman MM, Strongin MJ. Hereditary spherocytosis with hemolytic crisis during pregnancy. Treatment by splenectomy. Obstet Gynecol. 1976;47(1):19S-21S.
  10. Hannah DM, Tressler TB, Taboada CD. Nonimmune hydrops fetalis due to autosomal recessive hereditary spherocytosis. Case Rep Womens Health. 2017;16:4-7.
Citation

Khadke B, Prasad M, Gupta AS. A Case Of Pregnancy With Hereditary Spherocytosis. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/a-case-of-pregnancy-with-hereditary.html

Posterior Vaginal Wall Cyst

Author Information

Parihar AS*, Warke HS**, Shah R***
(* Junior Resident, ** Associate Professor, *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Benign cystic lesions of the vagina are frequently encountered. These are usually found incidentally during gynecological examination. This case deals with the diagnosis and management of posterior vaginal wall cyst.

Introduction

Vaginal cysts may be embryological derivatives, inclusion tissues or occur due to urological abnormalities.[1] Vaginal cysts are most common in the third and fourth decades of life, and rarely in the prepubertal girls. Various vaginal wall cysts are classified according to the lining epithelium determined by histology.[2] Mullerian cyst is the commonest constituting 30%, Bartholin duct cyst 27.5%, inclusion cyst 25% and rest 17.5%  are constituted by Gartner’s cyst, endometriotic cyst and unclassified variety.[3] These are usually incidental findings but may present with symptoms of mass per vaginum, bladder complaints, dyspareunia, pain or vaginal discharge.[2]

Case Report

A 28 year old woman, married since two years, nulligravida, came with complaints of dyspareunia since two years. General examination was unremarkable. There was no mass palpable per abdomen. On per speculum examination vaginal wall cyst of around 9x7 cm arising from the right lateral vaginal wall, extending to the posterior vaginal wall was seen. Cervix was not visualized. On per vaginal examination 9x7 cm vaginal wall cyst arising from the right vaginal wall extending upto posterior vaginal wall was felt. Uterus was normal in size, smooth, firm and mobile. Bilateral fornices were normal.


Figure 1. Vaginal wall cyst

On investigation, biochemical and serological investigations were in the normal range. Hemoglobin was 11.7 gm%, leucocyte count was 7800/ cmm, platelet count was 3,97,000/ cmm, BUN was 10.0 mg %, serum creatinine was 1.1 mg%, SGOT was 18 U/L, SGPT was 12 U/L, and TSH was 0.55 µIU/ml. On transvaginal sonography, a well defined cystic lesion with anechoic contents measuring 4.5 x 4.1 x 5 cm in the upper vagina arising from the right lateral wall, above the level of the symphysis pubis was noted, suggestive of Gartner’s cyst. No evidence of any solid components was detected. Uterus was anteverted with no focal lesion. Bilateral ovaries were normal. Bilateral adnexae were clear. No evidence of free fluid in pelvis was seen. MRI pelvis revealed a well defined cystic lesion measuring 2.9x4.3x6.1 cm which had homogeneous T2 hyper intense and T1 hypo intense signals, in the right lateral and posterior aspect of the vagina in its upper part protruding into the right fornix. The cystic lesion had a well defined T2 hypo intense wall throughout. The most likely diagnosis was that of a Gartner’s cyst. Visualized rectum and anal canal were normal.

She was taken for vaginal cyst excision under spinal anesthesia. Initially the cyst was separated from the vaginal mucosa by sharp dissection but in the process, the cyst ruptured. Cyst wall was separated posteriorly from the recto-vaginal extra pelvic fascia and it was noted that cyst was in very close proximity to the rectum. Cyst wall was separated posteriorly till its upper half and the redundant cyst wall (lower half) was excised. Cyst wall was sent for histopathological examination. Tissue overlying the rectum was sutured in the mid-line with polyglactin 2-0 interrupted sutures. On per rectal examination rectal mucosa was found to be intact. Remainder of the cyst in its upper extent was marsupilised with vaginal mucosa using polyglactin 2-0 sutures. Visualized cervix was normal. She tolerated the procedure and anesthesia well.

Postoperative period was uneventful. She had no urinary or bowel complaints and was discharged on day 10. Histopathology report was suggestive of a benign vaginal cyst. The gross specimen had smooth grey white surface. Sections showed a cyst wall comprising of fibromuscular tissue. No epithelium was seen.

Discussion

Vaginal wall cysts present in a variety of ways. True vaginal wall cyst arises from the vaginal tissues but lesions that arise from the urethra and surrounding tissues can also present as a cystic lesion in the vagina. History and physical examination alone can make appropriate diagnosis in most cases. Other cases are diagnosed only after excision and pathological examination of the tissue. Through physical examination, the lesion should be assessed for location, motility, tenderness, definition and consistency. The presence of malignancy must always be considered. Prolapse of pelvic organs must be ruled out, as they may mimic a vaginal cyst. Imaging by means of ultrasound, CT, MRI may be required to further categorize the lesion. MRI is found to be invaluable in diagnosing vaginal cyst and various other genitourinary abnormalities.

Mullerian and mesonephric remnants tend to be located along the anterolateral aspect of the vagina, however they can be found in any part of the vaginal wall. Mullerian cysts are lined predominantly by mucinous epithelium but may be lined by any epithelium of mullerian origin. These cysts are usually small and asymptomatic and require no treatment.[4] Gartner’s cysts are usually small but may enlarge to a point where they are mistaken for cystocele or urethral diverticulum. Abnormalities of the metanephric urinary system are also seen with Gartner’s cyst. MRI is especially useful for evaluation of the characteristics of the cyst and of the urinary tract. Cyst excision is only indicated when the cyst is large and symptomatic. Gartner’s duct cysts are found to be lined by cuboidal or low columnar, nonciliated, nonmucinous cells.

Bartholin’s duct cysts typically range from 1 to 4 cm in diameter. The majority are unilateral non-tender cystic masses located in the lateral introitus medial to the labia minora. Cysts arising from the main duct are lined by transitional or squamous epithelium, whereas cysts originating in an acinus are lined by mucinous columnar epithelium.
Epidermal inclusion cysts are the most common non embryological type of vaginal cysts. Inclusion cysts vary in size. Location correlates with an area of previous surgery. On histological examination inclusion cysts are found to be lined with stratified squamous epithelium and they contain sebaceous appearing material that represents desquamated epithelial cells.
Nodules of endometriosis are located at the posterior fornix and appear red-blue to yellow-brown. The patient may complain of cyclical enlargement, dysmenorrhea, dyspareunia, pelvic pain or dysuria. Histologically, 2 of the following 3 characteristics must be seen to make the diagnosis; endometrial glands, stroma and or hemosiderin laden macrophages. Foreign body giant cells may also be found.

Pelvic organ prolapse can present as a vaginal cyst. History and physical examination helps in diagnosis. It is important to determine if a vaginal cyst arises from the vaginal wall or if it is of urethral origin, as excision of certain urethral lesions requires post-operative catheter drainage to prevent fistula formation. A urethral caruncle is seen as a solitary red polypoid lesion protruding from one segment of the urethral meatus. Histopathology consist of a core of loose connective tissue and blood vessels covered by transitional or squamous epithelium. Urethral diverticula are located along the distal two-thirds of the urethra, presenting as anterior vaginal cyst. Histologically, diverticula consist mostly of fibrous tissue, and an epithelial lining is usually absent. If present, the epithelial lining may be transitional or squamous epithelium.
Thus, familiarity with the different diagnosis is essential for any clinician involved in gynecological or female urological practice to arrive at the correct diagnosis and treatment plan.

References
  1. Eilber KS, Raz S. Benign cystic lesions of the vagina: a literature review.  J Urol. 2003; 170(3):717-22
  2. Samal S, Mahapatro A, Poorkodi B. Posterior vaginal wall cyst of Mullerian origin: a case report. Int J Reprod Contracept Obstet Gynecol. 2015;4(1):245-6.
  3. Kondi-Pafiti A, Grapsa D, Papakonstantinou K, Kairi-Vassilatou E, Xasiakos D. Vaginal cysts: a common pathologic entity revisited. Clin Exp Obstet Gynecol. 2008; 35(1):41-4.
  4. Hwang JH, Oh MJ, Lee NW, Hur JY, Lee KW, Lee JK. Multiple vaginal mullerian cysts: a case report and review of literature.Arch Gynecol Obstet. 2009;280(1):137-9.
Citation

Parihar AS, Warke HS, Shah R. Posterior Vaginal Wall Cyst. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/posterior-vaginal-wall-cyst.html

A Case Of Mayer-Rokitansky-Kuster-Hauser Type II Syndrome – Murcs Variant

Author Information

Honavar PU*, Samant PY**.
(* Assistant Professor, ** Additional Professor and Head of unit, Department of Gynecology and Obstetrics, Seth G S Medical College and K E M Hospital, Mumbai, India.)
Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is aplasia of uterus and upper part of vagina. Their presentation is around puberty with primary amenorrhea. It can involve the urinary, skeletal, rarely cardiac or auditory system. The incidence and presentation of each association are varied. Approach to these patients should be pan systemic to diagnose other morbidity and to manage accordingly. One such variant of MRKH syndrome is presented here
Introduction

MRKH syndrome is characterized by congenital aplasia of the uterus and upper part of the vagina with 46XX karyotype and normal secondary sexual characteristics. The incidence is around 1: 4,500.[1] MRKH type II is associated with renal and vertebral problems. Mullerian duct aplasia-Renal agenesis-cervicothoracic somite dysplasia is abbreviated and referred to as MURCS syndrome. It can have cardiac or hearing defects.[2] GRES Syndrome, the association of Genital Renal And Ear abnormalities has an incidence of 1: 50,000.[3] Cardiac and auditory defects are less frequent with variable severity.[4
Case Report

A seventeen year old girl presented to the gynaecology outpatient department with complaints of primary amenorrhea. She also had occasional dull aching pain abdomen, which was not cyclical in nature. She was born of a nonconsanguineous marriage. None of her siblings had any significant medical or surgical history. She was averagely built and nourished. Tanner stage V breast development was noted. She had discrete facial dysmorphia with short neck (figure 1) not characterizing any known genetic syndrome. Neck movements were unrestricted.

Figure 1. Photograph of patient showing dysmorphic facies.
Figure 2. Photograph of patient showing lateral view with reconstructed pinna

Genital inspection revealed normal clitoris, labia and urethral meatus and a small blind pouch at the introitus. No thinned out/ bluish tinged bulging membrane noted. On rectal examination, no palpable uterus or mass was felt in the midline or laterally. Medial aspect of right thigh had a scar mark of previous skin graft harvest. She had undergone surgical construction of right pinna in childhood. This explained the child covering her right ear with locks of hair. On uncovering the right ear, a reconstructed auricle was seen. (Figure 2). Left sided pinna appeared normal. On ultrasonography of the abdomen and pelvis, uterus and ovaries were not appreciated. Karyotyping was 46 XX. MRI abdomen and pelvis was suggestive of a small uterus seen to the left of the midline. Reported endometrial thickness was 16 mm. An atretic cervix could not be ruled out, and bilateral ovaries were seen. Both kidneys were normal. A diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome was made. Her medical records revealed profound deafness right ear and otherwise normal left ear hearing. There was no deformity in the spine. The family was explained about the syndrome and management options.

After a consent, she was posted for a diagnostic laparoscopy with uterovaginal anastomoses or hysterectomy as per findings. Plastic surgeons evaluated and counseled the patient for vaginoplasty at a later date. Examination under anaesthesia confirmed a blind vaginal pouch of 3 cm. Laparoscopy revealed hypoplastic right fallopian tube. Bilateral ovaries were normal looking. A small hemi uterus of about 2 x 2 x 3 cm was noted with left sided round ligament and fallopian tube tube near the left pelvic wall (Figure 3). No cervix, connecting tissue or fibrous band between the uterus and vagina was noted. In situ findings of complete cervical agenesis was explained to the relatives. The procedure of anastomoses was deferred in view of huge anatomical gap between uterus and vagina. Hysterectomy in a rudimentary uterus was currently unindicated as she did not have cyclical abdominal pain which would have indicated of a functional endometrium.

Figure 3. Intraoperative laparoscopic image. B: Bladder, RO: Right Ovary, LO: Left Ovary, RL: Round Ligament, FT: Fallopian Tube, U: Uterus.

The patient and her parents were counselled about the menstrual, sexual and fertility issues in her case. Need for hysterectomy later, psychosexual counselling and later the need for vaginal dilatation before marriage and surrogacy options for conception were explained. The patients family has been explained about the need for follow up to monitor each of the above issues
Discussion

Although genetics of MRKH syndrome is not exactly known, most cases are sporadic.[5] However when familial, autosomal dominant inheritance with an incomplete penetrance is noted.[6] As variable presentation is seen, prevalence of this syndrome is underestimated. Type I MRKH is less frequent than MURCS association.[7] This explains the need for evaluation of every case of Mullerian agenesis for other systemic affections and vice versa.  Symmetric or asymmetric uterine hypoplasia with unilateral or bilateral tubal hypoplasia or aplasia is the characteristic feature of type II MRKH syndrome.[8] Our case had asymmetrical, left hypoplastic uterus with hypoplasia of right tube.

The anomalies with Type II mainly involve spine[9], less frequently, face and extremities. Facial asymmetry has been described by Pillay K et al[10], and was also noted in our case. Middle ear malformations or sensorineural defects of varying severity are associated with 10 to 25% of MURCS patients.[11,12] With MRKH type II syndrome our patient was also advised cardiac and skeletal evaluation.
Conclusion
Clinicians must include evaluation for renal, skeletal, facial and cardiac malformations in patients with primary amenorrhea. This case brings out the association of MRKH syndrome with other systemic abnormalities, highlighting the possibility of predicting the diagnosis in childhood, before the onset of amenorrhoea, especially when other malformations are noted.

References
  1. Morcel K, Watrin T, Pasquier L, Rochard L, Le Caignec C, Dubourg C, et al. Uterus-vaginal aplasia (Mayer-Rokitansky-Küster-Hauser syndrome) associated with deletions in known DiGeorge or DiGeorge-like loci. Orphanet J Rare Dis. 2011; 6: 9.
  2. Hofstetter G, Concin N, Marth C, Rinne T, Erdel M, Janecke A. Genetic analyzes in a variant of Mayer-Rokitansky-Kuster-Hauser syndrome (MURCS association). Wien Klin Wochenschr. 2008; 120 (13-14): 435-9.
  3. Balusamy SL, Kumar D, Subrahmanian SR. MURCS and VACTERL association in a 27 year old female. J Obstet Gynaecol. 2009; 29 (8): 762-3.
  4. Morcel K, Camborieux L, Guerrier D; Program of Recherchessur les Aplasies Müllérienes (PRAM). Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2007; 2:13.
  5. Carson SA, Simpson JL, Malinak LR, Elias S, Gerbie AB, Buttram VC, Jr., Sarto GE. Heritable aspects of uterine anomalies. II. Genetic analysis of Mullerian aplasia. Fertil Steril. 1983;40: 86–90.
  6. Pavanello Rde C, Eigier A, Otto PA. Relationship between Mayer-Rokitansky-Kuster (MRK) anomaly and hereditary renal adysplasia (HRA) Am J Med Genet. 1988; 29:845–9.
  7. Strubbe EH, Cremers CW, Willemsen WN, Rolland R, Thijn CJ. The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome without and with associated features: two separate entities? Clin Dysmorphol. 1994; 3:192–9.
  8. Strubbe EH, Willemsen WN, Lemmens JA, Thijn CJ, Rolland R. Mayer-Rokitansky-Kuster-Hauser syndrome: distinction between two forms based on excretory urographic, sonographic, and laparoscopic findings. Am J Roentgenol. 1993; 160:331–4.
  9. Pittock ST, Babovic-Vuksanovic D, Lteif A. Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005; 135:314–6.
  10. Pillay K, Matthews LS, Wainwright HC. Facio-auriculo-vertebral sequence in association with DiGeorge sequence, Rokitansky sequence, and Dandy-Walker malformation: case report. Pediatr Dev Pathol. 2003; 6:355–360.
  11. Cremers CW, Strubbe EH, Willemsen WN. Stapedial ankylosis in the Mayer-Rokitansky-Kuster-Hauser syndrome. Arch Otolaryngol Head Neck Surg. 1995; 121:800–3.
  12. Wulfsberg EA, Grigbsy TM. Rokitansky sequence in association with the facio-auriculo-vertebral sequence: part of a mesodermal malformation spectrum? Am J Med Genet. 1990; 37:100–2.
Citation

Honavar PU, Samant PY. A Case Of Mayer-Rokitansky-Kuster-Hauser Type II Syndrome – MURCS Variant. JPGO 2018. Volume 5 No.12. Available from:https://www.jpgo.org/2018/12/a-case-of-mayer-rokitansky-kuster.html

Scar Integrity Of A Hysterotomy Scar

Author Information
Jha N*, More V**, Chaudhari HK***.
(* Junior Resident, ** Assistant Professor, *** Associate Professor, Department of Gynecology and Obstetrics, Seth G S Medical College and K E M Hospital, Mumbai, India.) 
Abstract
Hysterotomy is rarely required as a procedure for first trimester abortion. Here we present a case of a Gravida 5 Para 2 Living 2 MTP 2 with three prior scars on uterus. In the current pregnancy, pregnancy was evacuated with suction evacuation, with direct visualization of the uterus under laparotomic guidance to prevent any breach in uterine integrity.
Introduction

Hysterotomy is a surgical technique where abdominal access into the gravid uterus is attained with the aim of evacuating the conceptus before viability. Hysterotomy is rarely required as a procedure for abortion. The scar of hysterotomy has almost similar complication profile as with upper segment cesarean section. Some of the complications include injury to the bladder base and haemorrhage, overstretching of the bladder to a higher anatomical position during covering of incision resulting in increased risk of bladder injury in subsequent surgeries and increased risk of uterine rupture in subsequent pregnancies. One case where difficulty was encountered in management due to a prior hysterotomy scar is presented here.
Case Report
A 28 year old female married since 10 years gravida 5 para 2 living 2 MTP 2, with 6 weeks gestation was admitted for medical termination of pregnancy with bilateral tubal ligation. The couple was using barrier method of contraception and was not keen on having further children. There was no significant history of medical or surgical illness. Her first two pregnancies were both uneventful lower segment cesarean sections, resulting in live births, both alive and well. Her third was an induced abortion, done by medical method. In her fourth pregnancy, at 3rd month of gestation, she wanted termination of pregnancy and was posted for surgical termination. During the procedure, a false passage was created with an anterior forniceal tear. Hence, an exploratory laparotomy was done with suturing of anterior vaginal wall to uterus. A vertical incision on the uterus was necessitated and products of conception were evacuated. Uterine incision was closed in polygalactin. Contraception counseling was done. However, she conceived again, and came with 6 weeks pregnancy for medical termination of pregnancy. Abdomen was soft with obesity. Pfannensteil incisions of prior cesarean sections and vertical midline incision of previous hysterotomy were present. On speculum examination, cervix high up. On vaginal examination, uterus was anteverted and bulky. The exact size could not be assessed due to obesity. There was anterior vaginal puckering.

Ultrasonography showed a single live intrauterine gestation of 7.2 weeks and there was no evidence of implantation at scar site. MRI also ruled out caesarean scar site implantation. However, scar thickness was only 5 mm on the posterior wall of uterus. In view of multiple scars on the uterus, there was a dilemma about the surgical procedure for termination of pregnancy. There were high chances of perforation with suction and evacuation, since MRI was suggestive of scar thinning. After due deliberation, she was posted for suction evacuation with tubal ligation under laparotomic vision in presence of surgeon.

Surgeons were called over, and abdominal wall was opened in layers. Multiple flimsy bowel adhesions were found and adhesiolysis was done. After this, the uterine contour was easily visualised. With the uterus in constant vision, the products of conception were aspirated with MVA syringe. Following this, all walls of uterine cavity was curetted under vision. During this step, an assistant stabilized the fundus and the posterior wall of uterus. The previous hysterotomy scar was seen on anterior wall of uterus extending on the fundus and posterior wall of uterus. Though the hysterotomy scar was thinned out and approximately 2 mm in thickness, a perforation was avoided due to constant visualization. Though tubal ligation was planned, bilateral salpingectomy was necessitated due to multiple adhesions. Patient tolerated the procedure well and post-operative course was uneventful. She was discharged on day 14 after suture removal.
Discussion
Hysterotomy is a surgical technique where abdominal access into the gravid uterus is attained with the aim of evacuating the conceptus before viability. Hysterotomy is rarely required as a procedure for abortion in second trimester. Inability to negotiate the internal os may necessitate hysterotomy in the first trimester also.  Complications of hysterotomy include increased risk of uterine rupture in subsequent pregnancies and increased risk of bowel and bladder adhesion and injuries. There is little literature on scar integrity of previous hysterotomy incision.[1]  In uterus with previous scars, vaginal procedures like dilatation, suction and evacuation, hysteroscopies can lead to creation of false passages. There is also substantial risk to continue pregnancy after previous hysterotomy. In this case, a false passage was created during first trimester medical termination of pregnancy, for which hysterotomy was performed with evacuation of products of conception by vertical incision on uterus. Moreover, there were already two scars on the uterus. The dilemma in the current pregnancy was about the procedure due to high chances of perforation with suction and evacuation, and radiological investigation suggestive of scar thinning.

Hysterotomy should be best avoided, due to substantial risk of perforation of the uterus.[2] Other risk factors for creation of false passage include nulliparous cervix, scarred uterus,[3] stenotic cervix, menopausal flushed cervix and obesity.[4] It usually occurs when instrument enters in wrong direction or in uterine cavity during adhesiolysis when dissection is done in wrong plane and intramyometrial space is created. It can be prevented by proper cervical traction, use of misoprostol 200 mcg 8 hrs before surgery, use of USG guidance and laparoscopic assistance.[5,6]
In both laparoscopic assistance and laparotomy, perforation is prevented by direct visualisation of the uterine contours. However, in laparotomy, there is a possibility of tactile sensation also, which we used in our case, to our advantage. Salpingectomy could also be performed in a quick manner.
Conclusion
Uterine perforation should be avoided while attempting medical termination of pregnancy in uteri which are scarred. While ultrasound guided and laparoscopy are also options, direct laparotomy remains a good option to avoid uterine perforation and bowel injury.
References
  1. Song D, Xia E, Xiao Y, Li TC, Huang X, Liu Y. Management of false passage created during hysteroscopic adhesiolysis for Asherman's syndrome. J Obstet Gynaecol. 2016;36(1):87-9
  2. Heys RF. Pregnancy after hysterotomy. Br Med J. 1973;1(5854):681-2
  3. Frick AC, Drey EA, Diedrich JT, Steinauer JE.Effect of prior cesarean delivery on risk of second-trimester surgical abortion complications.Obstet Gynecol. 2010 Apr;115(4):760
  4. Lederle L, Steinauer JE, Montgomery A, Aksel S, Drey EA, Kerns JL.Obesity as a Risk Factor for Complications After Second-Trimester Abortion by Dilation and Evacuation.Obstet Gynecol. 2015 Sep;126(3):585-92.
  5. Fritz RB, Rosenblum N, Gaither K, Sherman McCalla A. Successful Laparoscopically Assisted Transcervical Suction Evacuation of Interstitial Pregnancy following Failed Methotrexate Injection in a Community Hospital Setting. Case Reports in Obstetrics and Gynecology. 2014. 2014: 695293. Available from: http://dx.doi.org/10.1155/2014/695293
  6. Anagani M, B Radhika, P Vandana. Role of Laparoscopy in Management of Post Abortion Haemorrhage. SciFed Obstetrics & Women Healthcare Journal. 2017. Available from: http://scifedpublishers.com/fulltext/role-of-laparoscopy-in-management-of-post-abortion-haemorrhage-case-reports/21492
Citation
Jha N, More V, Chaudhari HK. Scar Integrity Of A Hysterotomy Scar. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/scar-integrity-of-hysterotomy-scar.html

Paravulvar Lipoma Masquerading As Vulvar Lipoma

Author Information

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

Abstract

A paravulvar lipoma is a very rare tumor. It can be mistaken for a vulvar lipoma. We present a case of a large paravulvar lipoma in a young woman.

Introduction

Lipomas are widely disseminated benign tumors of soft tissues. Their etiology is unknown. Trauma seems to be one of the causes.[1,2] It is common over the neck, upper back, shoulders, abdomen, buttocks, and proximal portions of the limb [3]. It rarely present in vulva [4,5]. In this paper, we report the case of a paravulvar lipoma that was diagnosed in a 20 year-old woman. Our patient was young without any history of trauma. We discuss the clinical features and management options of this pathology with the histopathological evaluation of all excised lesions. A review of the literature is also presented.

Case Report

A 20 year-old woman presented with a painless and slow-growing soft movable swelling of private parts. She reported a sudden uncomfortable feeling in the    perineum. The mass had grown gradually over one year. There was no significant family history. She had no history of change in color or any discharge from the mass, the main reason to seek medical attention was the cosmetic disfigurement. Her previous menstrual and obstetrical history was unremarkable. A physical examination done by unit head  revealed a single soft and pasty mass in her right paravulvar region (figure 1 ). The mass could be moved freely under her skin. The dimensions of the mass measured was 10 cm X 8 cm. There was no visible palpable cough impulse or inguinal lymphadenopathy. Her physical pelvic examination was normal. Ultrasonography was performed which demonstrated a homogenous and hyperintense mass with a well-defined contour in our patient’s right labium majus of vulva in the subcutaneous plane. All her laboratory tests were normal. In lithotomy position and under general anesthesia a longitudinal incision along the most prominent area of the right labium majus was taken. The fatty lump was identified and mobilized. The mass including 1- cm width of redundant skin on the incision line was excised carefully (figure 2). There was no difficulty in surgery because the mass was well-encapsulated. After securing hemostasis, the skin was closed with interrupted sutures of No. 3-0 monofilament polyamide (figure 3). The postoperative course was uneventful and the patient left hospital on the fourth postoperative day. The excised specimen measured 10 × 8 × 3.8 cm (figure 4) and it was surrounded by a fibrous capsule. Serial histological sections showed lobulated yellow tissue and homogeneous mature adipocytes without any hemorrhage or necrosis. The histopathologic diagnosis of a lipoma was confirmed.


Figure 1. Image showing the vulva mass. local examination.


Figure 2. Intraoperative image showing excision of the paravulvar mass.


Figure 3. Image showing closure of skin incision with non-absorbable sutures.


Figure 4. Image showing excised specimen.

Discussion

The most common areas for lipomas are the upper back, neck, shoulder and abdomen. Lipomas are usually seen between 40 and 60 years of age.[1,2] They are  the most common benign tumor in soft tissues. Solitary lipomas are more common in women. Multiple lipomas are more common in men. Variants seen include spindle cell lipomas, pleomorphic lipomas, angiolipomas and adenolipomas. It is derived from mesenchymal cells.[4] Congenital childhood lipomas are mostly inherited and rare.[5]
Lipomas usually present as single or multiple, not causing pain, gradually increasing in size and mobile swelling. The tumor shows ill-defined, or pedunculated aspects that are not adherent to the overlying skin. These pecularities provide correct diagnosis in most cases by clinical examination.[7,8,9,10,11] However, various differential diagnosis are liposarcomas, cystic swellings of Bartholin’s gland and Nuck’s canal, and inguinal hernia, especially in children.[3,4,6] Other rare tumors such as aggressive angiomyxoma,[12] benign lipoblastoma-like tumors,[13] granular cell tumors and,[14]liposarcoma,[15,16] must be differentiated from a simple lipoma. In  our case it was a large swelling. Diagnosis was difficult. It was arising from genitocrural fold  and grown downwards. Its origin was not vulvar but paravulvar.
In developing countries, ultrasound is recommended instead of expensive imaging investigation because of its availability and cost-effectiveness.[3] Vulvar lipoma ultrasound demonstrates a non-specific homogenous echogenic mass with lobular structures consistent with fat deposition.[3,9,10] Computed tomography and MRI are useful for evaluating tumor extensions and anatomical connections with surrounding structures.[4,9,10] MRI is a supportive tool to differentiate vulvar lipomas from liposarcoma.[3,6,11] Therefore, soft lipomatous lesions with thin septa that are not enhanced on MRI could be diagnosed as lipoma.[6,17]
Conservative treatment for a lipoma includes steroid injection and liposuction and is done commonly. However, complete surgical excision is the treatment of choice for vulvar lipomas.[3] Surgery also allows for excluding malignant nature by performing a histological study.[3,4] Typically, a histological study shows a thin peripheral capsule surrounding a lobular proliferation of adipocyte.[6] Recurrence is possible; short-term recurrence should draw the attention of clinicians to possible malignant nature of the tumor.

Conclusion

Paravulvar lipomas share very similar clinical and imaging profiles with liposarcomas. Recently, CT and MRI have been used with some success to differentiate the two.[18] To exclude the possibility of malignant tumors, biopsy should be performed by surgical excision. The final diagnosis should be based on histopathological evaluation. This case report will help students to distinguish a vulvar swelling from paravulvar swelling.

References
  1. Signorini M, Campiglio GL. Posttraumatic lipomas: where do they really come from? Plast Reconstr Surg. 1998; 101:699–705
  2. Copcu E. Sport-induced lipoma. Int J Sports Med. 2004; 25:182–5
  3. Odoi AT, Owusu-Bempah A, Dassah ET, Darkey DE, Quayson SE. Vulvar lipoma: is it so rare? Ghana Med J. 2011, 45: 125-127.
  4. Jung HL, Seung MC: Large vulvar lipoma in an adolescent: a case report. J Korean Med Sci. 2008, 23: 744-746. 
  5. Jourjon R, Dohan A, Brouland JP, Guerrache Y, Fazel A, Soyer P. Angiolipoma of the labia majora: MR imaging findings with histopathological correlation. Clin Imaging. 2013, 37: 965-968.
  6. Aust MC, Spies M, Kall S, Gohritz A, Boorboor P, Kolokythas P, Vogt PM. Lipomas after blunt soft tissue trauma: are they real? Analysis of 31 cases. Br J Dermatol. 2007, 157 (1): 92-9
  7. Salam GA. Lipoma excision. Am Fam Physician. 2002, 65 (5): 901-904.
  8. Jung TO, Seung HC, Sung GA, Myung JK, Woo IY, Seok JH. Vulvar lipomas in children: an analysis of 7 cases. J Pediatr Surg. 2009, 44: 1920-1923.
  9. Ogasawara Y, Ichimiya M, Nomura S, Muto M. Perineal lipoma in a neonate. J Dermatol. 2001, 28: 165-167.
  10. Ohguri T, Aoki T, Hisaoka M, Watanabe H, Nakamura K, Hashimoto H, et al. Differential diagnosis of benign peripheral lipoma from well-differentiated liposarcoma on MR imaging: is comparison of margins and internal characteristics useful? Am J Roentgenol. 2003, 180: 1689-94.
  11. Mandai K, Moriwaki S, Motoi M. Aggressive angiomyxoma of the vulva. Report of a case. Acta Pathol Jpn 1990; 40: 927-934.
  12. Atallah D, Rouzier R, Chamoun ML. Benign lipoblastomalike tumor of the vulva: report of a case affecting a young patient. J Reprod Med 2007; 52: 223-224.
  13. Raju G, Naraynsingh V. Granular cell tumours of the vulva. Aust N Z J Obstet Gynaecol 1987; 27: 349-352.
  14. Nucci M, Fletcher C. Liposarcoma (atypical lipomatous tumors) of the vulva: a clinicopathologic study of six cases. Int J Gynecol Pathol 1998; 17: 17-23.
  15. Murphey MD, Carroll JF, Flemming DJ, Pope TL, Gannon FH, Kransdorf MJ. Benign musculoskeletal lipomatous lesions. Radiographics. 2004, 24: 1433-1466.
Citation

Tiwari N, Chaudhari HK. Paravulvar Lipoma Masquerading As Vulvar Lipoma. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/paravulvar-lipoma-masquerading-as.html

Remembering Past Greats: Thomas Spencer Wells

Author Information

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

Sir Thomas Spencer Wells (1818-1897) was an excellent 19th century medical practitioner. He started training as a doctor at a rather young age (for those times) of 17. Within two years, he had risen to the role of main assistant to leading obstetricians in Leeds. He then spent varying amounts of time being trained at Leeds University, Trinity College, Dublin and St Thomas Hospital, London. Upon earning the Royal College of Surgeons membership, he joined the Royal Navy and served in the British colony, Malta, for many years.[1]

His mentor during the Navy days, William Burnett had been described as a ruthless professional,[2] and had significant influence over his future career. It appears that Sir Wells picked up a lot of nuances about the importance of asepsis during the stint in the Navy,[3] and implemented them well during future practice also. His ability to describe pathological processes in detail and correlate with autopsy findings are exemplified in a publication, whose archive is still available.[4]
Gynecological pathology evolved under him, and a contemporary Lawson Tait, who pioneered the correlation of pathological specimens removed at surgery rather than excessive reliance on autopsy specimens, as chronicled by Young.[5] His enthusiasm for strategies to prevent diseases among sailors, and to develop and popularize vaccines,[6] paved way for much research in this aspect.
Despite all these contributions, the ones he is most popular are the operations on the ovary. He is believed to have performed over 1200 ovariotomies for various indications and attempted pathological correlation with various gynecological conditions, which was a pioneering achievement for the 19th century. Most of this work was done in the Samaritan Hospital for Women and Children in London.  He also designed a clamp, which is now commonly used in vaginal hysterectomy. He had initially described it for the ovarian pedicle.[1]

To summarize, Spencer Wells was a leading medical figure of his times, and his memory is sure to kindle enthusiasm among all of us to be excellent gynecologists.

References
  1. Biographies. In O’Dowd MJ, Philipp EE, editors. The History of Obstetrics and Gynecology. 1st ed. Lancs: Parthenon Publishing Group 2000; pp. 656-7.
  2. Penn C. Sir William Burnett (1779-1861), professional head of the Royal Naval Medical Department and entrepreneur. J Med Biogr. 2004;12(3):141-6.
  3. Smith EJ. 'Cleanse or Die': British Naval Hygiene in the Age of Steam, 1840-1900. Med Hist. 2018 Apr;62(2):177-198.
  4. Martin W, Wells TS. Report of Cases Treated in the Royal Naval Hospital, Malta. Edinb Med Surg J. 1844;61(159):350-390.
  5. Young RH. The history of British gynaecological pathology. Histopathology. 2009;54(2):144-55.
  6. Cook GC. Thomas Spencer Wells, Bt FRCS (1818-97) and his contributions to naval medicine. J Med Biogr. 2007;15(2):63-7.
Citation

Prasad M. Remembering Past Greats: Thomas Spencer Wells. JPGO 2018. Volume 5 No.12. Available from: https://www.jpgo.org/2018/12/remembering-past-greats-thomas-spencer.html

Volume 5 Number 11