Volume 3 Issue 4, April 2016

Editorial
Parulekar SV

A Case Of Primary Amenorrhoea With A Rare Mullerian Anomaly
Lothe S, Panchbudhe S, Warke HS, Satia MN.

 Intramyometrial Pseudocyst
Chawla T, Parulekar SV, Fernandes G, Rojekar A.

 Cornual Leiomyoma And Patent Tube
Jagtap V, Valvi D, Parulekar SV.

Takayasu Arteritis Presenting With Severe Preeclampsia and Intrauterine Fetal Demise
Madhva Prasad S, Puri J, Gupta AS.

Congenital Granular Cell Tumor (Epulis) Diagnosed Antenatally On 3d Ultrasound And Its Postnatal Management
Chhatrapati AK, Asarkar TJ, Mishra I, Jassawalla MJ.

Acoustic Schwannoma In A Full Term Pregnant Patient
Dwivedi JS, Gupta AS.

Successful Outcome Of Pregnancy In A Patient With Myasthenia Gravis
Patil S, Tiwari N, Shah A, Chauhan AR.

Pituitary Macroprolactinoma with Acromegaly in Pregnancy: A Diagnostic Challenge
Ganapathi T, Daigavane M, Samant P.

Successful Pregnancy Outcome In A Case Of Sickle Cell Crisis
Lothe S, Hatkar P, Satia MN.

Editorial

Parulekar SV

Maternal mortality and morbidity are higher than they should be, in both most of the developed countries as well as developing countries. They need to be kept low, because pregnant women are young women, most of them healthy, and they have a long life ahead of them. Pregnancy and childbirth are physiological processes, not illnesses and should not result in serious outcomes for the mother and the baby. The mothers are the persons who look after the families, especially the children, who grow up to form pillars of the society. Death or serious illness of a mother leaves a deep imprint on the health of the family. A large number of such occurrences are entirely avoidable, provided they are anticipated and detected early. Healthcare providers have always used methods to meet this goal.

Workers in the developed world have recommended the use of maternal early warning tools for this purpose. Shields et al described use of one such tool called Maternal Early Warning Trigger (MEWT) tool. It was described as a clinical pathway-specific tool that addressed the four most common areas of maternal morbidity – infection, cardio-pulmonary dysfunction, hemorrhage and hypertension. Any single value of the following, sustained for more than 20 minutes was considered positive – maternal heart rate above 130 bpm, respiratory rate above 30/min, mean arterial pressure below 55 mm Hg, or concern by the nurse. Other parameters were also considered to be positive if there were two abnormal values - heart rate above 110 or below 50 bpm, temperature above 38 or below 36° C, blood pressure above 160/110 or below 85/45 mm Hg, respiratory rate above 24 or  below 10/min, oxygen saturation below 93%, fetal heart rate above 160 bpm, altered maternal mental status, or disproportionate pain. The study was done on a large number of pregnant women. It involved a control group too, in which these measures were not used. Use of this tool and addressing the condition detected resulted in significant reduction in maternal morbidity.

Other tools have been recommended and used in the past. In Great Britain The modified early obstetric warning system (MEOWS) has been proposed in UK and the maternal early warning criteria (MERC) has been recommended in USA by National Council for Patient Safety. MEOWS uses a score attributed to the parameters such as temperature, systolic blood pressure, diastolic blood pressure, heart rate, respiratory rate, level of consciousness using AVPU scale and urine output. AVPU is short for A - Alert (Alert and conscious), V - Voice   (Responds to voice), P - Pain   (Responds to pain), and U - Unresponsive    (No response to voice or pain). These are charted on a graph paper. A score of 3 or higher is an indication for initiating action based on predefined algorithm which recommends change in monitoring pattern, referral, review, or therapeutic action.

General opinion on the use of these tools is that they have not been tested widely enough to prove statistically that they are effective. In the meantime, more and more of such tools are likely to be proposed, each worker or group of workers inspired to find a tool that would prove to be more useful that those which have been described before. In general a scoring system is not a very good system to detect an abnormality because the same score can be reached by different combinations of values of different variables, all of which do not carry the same degree of significance. Besides, on receiving an alert, the obstetrician has to evaluate all parameters again in order to determine which one is abnormal, so that the underlying cause can be sought. It would be a lot easier, faster and more efficient to inform him about the abnormal parameter itself. Nurses and doctors in the developing world have a very large number of pregnant women to treat at any given time, and cannot afford to spend time developing scores from charts and then evaluating the scores to find the cause. We screen all pregnant women and decide which ones are likely to develop particular complications during labor or any problems related to the pregnancy. The high risk ones are monitored more intensively. We have been using the chart of vital parameters, record of vaginal bleeding and nurse’s concern over anything that she believes is abnormal over the last thirty six years. A woman in labor or with an acute pregnancy complication is monitored every half hour (more frequently if critically ill, but we are considering early warning here) and the nurse informs the obstetrician if the temperature rises above 370 C, heat rate rises above 120/min or falls below 60/min, respiratory rate rises above 30/min or falls below 14/min, blood pressure rises above 140 mm Hg systolic or 90 mm Hg diastolic, or falls below 90 mm Hg systolic or 60 mm Hg diastolic, urine output falls below 60 ml in 2 hours (in patients likely to develop renal insufficiency), significant vaginal bleeding occurs, or any serious event occurs that alarms or baffles the nurse. Then the obstetricians checks the patient and finds out the cause of the abnormal parameter. Based on the diagnosis, appropriate action is taken as per management guidelines. We have been able to detect almost all conditions that cause maternal morbidity and mortality (hemorrhage, hypertension, sepsis and their complications) using this system. We cannot detect some like amniotic fluid embolism in early phase, but then no tool described so far can do so. We appreciate that some workers are developing tools to reduce maternal morbidity and mortality, because they mean well. But we prefer to be practical and take clinical actions at the earliest hint of a developing abnormality rather than spend time and energy developing scores using cumbersome tools which do not achieve results any better than ours.

A Case Of Primary Amenorrhoea With A Rare Mullerian Anomaly

Author Information

Lothe S*, Panchbudhe S**, Warke HS***, Satia MN****.
(* Second Year Resident, ** Assistant Professor, *** Associate Professor, ****Professor, Department of Obstetrics and Gynacology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

Few problems in gynecology are as challenging and taxing to the physician as amenorrhea and also those associated with Mullerian duct anomalies. Mullerian anomalies include a wide spectrum of abnormalities. They can result from dysregulation or interruption at various stages of development of the paramesonephric duct. Various classifications have been given to group these anomalies. Although the most widely accepted system is the American Fertility Society classification - 1988 (AFS) which categorizes Mullerian defects into 7 classes according to major uterine anatomic defect, it is associated with various limitations in effectively categorizing the mullerian anomalies. We present a rare case of a 25 year old female having two uterine horns, both non communicating with each other, left horn functioning and communicating to a single stenosed cervix with hematometra and right horn non functioning, not communicating to the cervix. This defect is not covered under AFS classification of mullerian anomalies but can be classified by European Society Of Human Reproduction And Embryology (ESHRE/ESGE)  and EAC classification of congenital mullerian anomalies.

Introduction

Mullerian duct anomalies have been reported since 16th century and the incidence varies from 0.1 to 3.5%.[1] They are being diagnosed early over the last few decades due to better imaging modalities. Fusion of Mullerian duct occurs at around 6-11 weeks of gestation in utero and this fusion and absorption is unidirectional from caudal to cephalad end.[2] This unidirectional theory forms the basis of the current AFS classification which is the most broadly used classification till date. 90% anomalies are explained under this classification however some rare cases do not fit into this. ESHRE/ESGE classification for mullerian anomalies is also a quite complex system and does not cover complex anomalies. EAC classification has overcome these fallacies and is more comprehensive and easy to use. Mullerian anomalies have a wide spectrum of varieties and management also differs according to the defect. The case described here is a Mullerian anomaly with two uterine horns, one functioning with hematometra and communicating to the single stenosed cervix & the other horn non functioning and not communicating to the cervix. This represents failure of fusion of two uterine horns that could have lead to the formation of a bicornuate uterus. The defect cannot be classified as uterus didelphys as both the horns are attached to each other by an adhesive band and one of the uterine horn was lacking the cervix. The presence of both the fallopian tubes and ovaries excludes this case from the unicornuate variety.

Case Report

A 25 year old woman married since 3 years, a case of primary amenorrhea was admitted from the outpatient department with complaints of continuous pain in the abdomen since 5 to 6 months. Patient gave history of cyclical pain in the abdomen since 17 years of age. Initially the pain was cyclical for 4 to 5 days followed by continuous pain throughout since last 5 to 6 months. There was no history of any other medical and surgical illness in the past. Her general examination was normal and secondary sexual characters were well developed. Breast development was Tanners stage 5 and pubic hair was Tanners stage 4. On general examination thyroid gland was normal. There was no stigmata like lymphadenopathy. No muculoskeletal deformities were associated. On examination abdomen was soft with no evidence of palpable lump. Per speculum examination revealed single pin point cervix and on per vaginal examination there was a right forniceal solid  mass of 3×3 cm. On the left side a mass of 3×2 cm was felt which probably was the uterus or the left horn. Clinical impression was bicornuate uterus. She had a contrast enhanced computed tomography (CECT) from a private hospital. It showed right adnexal mass of 5.2×4×3.4 cm that was solid cystic and left adnexal solid lesion suggestive of tubo-ovarian mass of 2.3×4.5×3.5 cm. Ultrasound (USG) pelvis done at our institute was suggestive of uterus 3.5x2x2 cm, hypoplastic with endometrial thickness (ET) of 1 cm with central thin hypo echoic endometrial collection within. Right ovary was 5.5x3 cm with a large heterogeneous lesion having central vascularity and few cystic areas. Left ovary of 3.4x3.1 cm had a solid lesion with similar architecture with central echogenic cystic areas, and increased vascularity. A diagnosis of hypoplastic uterus with solid ovarian tumor was given. Tumor markers were done in view of the CT scan findings. They were normal. Karyotype was done which was 46 XX. Her hormonal profile was normal. MRI pelvis was advised. MRI showed a small hypoplastic uterus of 3.7x2.1x2.2 cm with no endometrial collection. Right ovary was bulky measuring 5.5x3.6 cm with multiple tiny cystic areas showing T1 hyper-intense contents, There was no torsion of the right side pedicle seen. Left ovary was also bulky measuring 3.2x3.4 cm, showing thick wall T1W/ T2W hyper-intense cystic lesion and showed blood fluid levels with thick septations. MRI impression was hypoplastic uterus with bilateral tubo-ovarian mass. Kidneys and ureters were normal. Diagnostic laparoscopy was done in view of recurrent intractable pelvic pain.  Bicornuate uterus with both horns non-communicating, attached to each other by a thick band of adhesion was seen. Left horn was 3 3 cm and right horn was 5x4 cm. Bilateral ovaries and Fallopian tubes were normal. Dilatation of the cervix was tried but was not possible. Post procedure analgesics were given, patient was symptomatically better and was discharged.
Patient was readmitted after a month with severe abdominal pain. USG was suggestive of an increase in the size of the right horn to 6.3x2.9x4.5 cm and left horn of 6x2.8x4 cm. In view of above findings and excruciating pain, exploratory laparotomy with excision of the non communicating horn with dilatation of stenosed cervix was planned. Consent regarding the same with consent for abdominal hysterectomy if required was taken. Under anesthesia attempt was made to dilate the cervix. However the internal cervical Os could not be negotiated. Intra-operative findings were suggestive of two uterine cavities both non-communicating with a stenosed single cervix with left sided hematometra and right sided cavity non-functioning with evidence of flimsy adhesions between the two horns. Right horn was excised and pedicle sutured. From the left horn 5 cc of chocolate colored fluid was aspirated suggestive of menstrual blood. Incision was taken at the isthmus of the left horn so as to enter the endometrial cavity. Retrograde dilation of the cervix through the cavity was tried which failed suggesting cervical stenosis. Hence decision of subtotal hysterectomy was taken. Cut section of both the horns showed adenomyotic changes with chocolate colored fluid in the left horn. Patient was stable and relieved of pain in post-operative period and was discharged on 7th postoperative day. Couple was counseled regarding adoption. Histopathology report was suggestive of two horns of the uterus with severe adenomyosis with benign endometrial glands in proliferative phase.


Figure 1. Both the horns of the uterus as seen during exploratory laparotomy. Green arrow shows left horn (3x3 cm) of the uterus with fallopian tube and ovary, Yellow arrow shows right horn (4x4 cm) with right fallopian tube and ovary.


Figure 2. Vertical cut section of both the horns of the uterus. Yellow arrow shows vertical cut section of the right non functioning horn and green arrow shows cut section of left functioning horn with hematometra.

Discussion

Mullerian duct anomalies have multifactorial and polygenic etiology.[3] Most widely accepted AFS classification categorizes Mullerian defects into 7 classes according to major uterine anatomic defect.[4] It is based on unidirectional theory of mullerian duct development. Mullerian duct anomalies account for 10% of the primary amenorrhea cases. Mullerian duct anomalies include a vast array of structural defects that range from complete agenesis, defective fusion, failure of canalization to failure of absorption of septum. Presentation varies from patient to patient according to the defect. These defects are mostly manifested during adolescence or early adulthood. Frequently the cases are asymptomatic and are missed during gynecological examination. Complaints of primary amenorrhea, pelvic pain following menarche, dysmenorrhea, and palpable abdominal lump bring these cases to the notice of the clinician.
Diagnostic modalities include hysterosalpingography, transvaginal ultrasonography, 3D USG which is more sensitive and equal to or better than MRI. Nowadays the gold standard is MRI.[5] Management also differs according to the type of the defect, the clinical scenario and the patient’s wish to preserve reproductive functions. The aim of operative intervention should be to retain healthy sexual life and successful reproductive outcome. However in some cases it is not possible and decisions are taken for symptomatic relief. In our case preserving the reproductive potential was not possible and hence subtotal hysterectomy was done in order to avoid morbidity, financial and psychological burden.
This case does not come under any of the seven classes of AFS system. Under ESHRE/ESGE classification it is classified as U4b/C3/V0.[6] According to EAC classification based on embryological aberrations, anatomical abnormalities and clinical features proposed in 2015 it is classified as UtL 3cm Fn Obs, UtR 4cm No Fn Obs, Cx 1cm Obs.[7]
Similar to the above case, DA Potter et al reported a case with non-communicating accessory uterine cavity.[8] M. Saleh reported a case with unilateral non-communicating cervical atresia with uterus didelphys and unilateral renal agenesis.[9] According to European Society of Human Reproduction and Embryology published in March 2015, a study conducted from 1988 to January 2014 revealed 140 cases that are not described under AFS classification.[10] This case too is not included under AFS classification but can be classified according to EAC classification. It could be suggested that more comprehensive classification like EAC classification should be used for classifying mullerian anomalies to overcome fallacies of AFS classification. Mullerian anomalies consist of a vast array of defects which vary from patient to patient and hence management must be individualized taking into account the anatomical and clinical variations as well as the patient’s wishes.

References
  1. Speroff L. The uterus. In: Mitchell C, editor. Clinical Gynecologic Endocrinology and Infertility. 7th ed. Philadelphia: Lippincott Williams and Wilkins; 2005. p. 113.
  2. Dunn R, Hantes J. Double cervix and vagina with normal uterus and blind cervical pouch: a rare mullerian anomaly. Fertil Steril. 2004 Aug; 82(2): 458-9. 
  3. Troiano RN, Mccarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology. 2004 Oct; 233(1): 19-34. 
  4. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions.Fertil Steril. 1988 Jun;49(6):944-55.
  5. Berger A, Batzer F, Lev-Toaff A, Berry-Roberts C.Diagnostic imaging modalities for Müllerian anomalies: the case for a new gold standard. J Minim Invasive Gynecol. 2014 May-Jun; 21(3): 335-45.
  6. Grimbizis GF, Gordts S, Sardo AD, Brucker S,  De Angelis C, Gergolet M,  et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies.Hum Reprod. 2013 Aug; 28(8): 2032–2044. 
  7. Parulekar SV. Classification Of Congenital Malformations Of The Female Genital Tract. JPGO 2015. Volume 2 No. 4, Available from: http://www.jpgo.org/2015/04/eac-classification-of-congenital.html
  8. Potter DA, Schenken RS. Noncommunicating accessory uterine cavity. Fertil Steril. 1998 Dec; 70(6): 1165-6. 
  9. Saleh M, Badawy SZA. Unilateral Non-communicating Cervical Atresia in a Patient with Uterus Didelphys and Unilateral Renal Agenesis Online Only. 2010 Oct;23(5): e137–e140
  10. Di Spiezio Sardo A, Campo R, Gordts S, Spinelli M, Cosimato C, Tanos V, et al.The comprehensiveness of the ESHRE/ESGE classification of female genital tract congenital anomalies: a systematic review of cases not classified by the AFS system. Hum Reprod. 2015 May;30(5):1046-58.
Citation

Lothe S, Panchbudhe S, Warke HS, Satia MN. A Case Of Primary Amenorrhoea With A Rare Mullerian Anomaly. JPGO 2016. Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/a-case-of-primary-amenorrhoea-with-rare.html

Intramyometrial Pseudocyst

Author Information

Chawla T*, Parulekar SV**, Fernandes G***, Rojekar A****.
(*Second year Resident; ** Professor and Head, Department of Obstetrics and Gynecology, *** Associate Professor, **** Assistant Professor, Department of Pathology; Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

There are a number of causes of intramyometrial cysts, of which cystic degeneration in leiomyoma is the most common one. Uterine leiomyomas are predominantly composed of smooth muscle cells surrounded by a pseudocapsule. An imbalance between oxygen demand and supply is observed when leiomyomas enlarge, causing areas of degeneration. Among the degeneration types observed, the hyaline, myxoid, red and cystic degenerations are the most common. Intramyometrial cyst formation is observed with cystic degeneration. Here we describe a case of marked cystic degeneration of a uterine fibroid, presenting as a large intramyometrial cyst.

Introduction

Intramyometrial cysts can be due to cystic degeneration in a leiomyoma, adenomyosis, congenital anomalies in the form of uterine cysts, cervical nabothian cysts, and echinococcal cysts.[1,2,3,4,5] Uterine leiomyomas are the most common benign solid tumors of the female genital system, composed mainly of smooth muscle cells containing various amounts of fibrous connective tissue. These tumors affect 20% to 30% of women at childbearing age, and more than 40% of women above 40 years of age. Cystic degeneration occurs in areas of hyaline degeneration when extensive. It causes formation of a cysts within the myometrium.[3,4,5] A case of a large intramyometrial cyst due to cystic degeneration in an intramural leiomyoma is presented.

Case Report

A 43 year old female married for 22 years, para 2 living 2 presented with complaints of menorrhagia associated with dysmenorrhoea since 3 months. She would soak of 5-6 pads/day for 3-4 days during menstrual flow. She had undergone laparoscopic tubal ligation 19 years ago. Her medical or surgical history was not contributory. Her general and systemic examination findings revealed no abnormality. The abdomen was soft with a laparoscopic tubal ligation scar. The uterus was enlarged uniformly to 16 weeks' size. A per speculum examination showed a nabothian follicle on the cervix at 6 o'clock position, a small rectocoele and lax perineum. There was no forniceal tenderness or mass. Her biochemical preoperative investigations and chest radiography were normal. Her hemoglobin was 6.5 g/dl for which she received 4 units of packed cells transfusion, after which her hemoglobin became 10.1g/dL. Abdominal ultrasonography revealed an anechoic lesion in the posterior uterine wall, suggestive of a cystic collection, measuring 5.0 × 4.0 cm in size. A total abdominal hysterectomy was performed under spinal anaesthesia. On further dissection of the specimen an approximately 6x4 cm of fibroid was seen in the posterior uterine wall. When cut open it revealed a unilocular cavity with amber colored fluid stained with blood. A provisional diagnosis of cystic degeneration of the fibroid was made with differential diagnosis of endometrial cyst. Post-operative period being uneventful.
The comprehensive histopathological examination of the specimen revealed a large leiomyoma with extreme hyaline and cystic degeneration. The leiomyoma was partly lined by endometrium on its external surface. There was no evidence of any lining epithelium on the inner cystic surface.


Figure 1. Showing gross specimen of uterus cut open showing leiomyoma. The pseudocapsule of the leiomyoma is indented by pressure of the handle of a scalpel, demonstrating fluid contents of the leiomyoma.


Figure 2. The leiomyoma with cystic degeneration has been cut open. Blood stained fluid is seen escaping from the opening.


Figure 3. Showing gross specimen of uterus cut open with partially enucleated leiomyoma, which has been cut open to reveal a smooth, shiny inner surface of its cavity.


Figure 4. Showing a leiomyoma with extensive areas of hyalinization (red arrows). No lining epithelium is seen on the inner surface of the cavity of the leiomyoma (hollow arrow). (H&E x100)


Figure 5. Wall of pseudocyst without any lining epithelium (arrows). (H&E x400)

Discussion

Leiomyomas are the most common uterine neoplasms. They occur in 20-30% of women in the reproductive age group.[3,4,5] They are composed of smooth muscle and fibrous tissue and are benign in nature.[3] Based on their location within the uterine wall, leiomyomas are classified into submucosal/subendometrial, intramural/myometrial or subserosal leiomyomas. Leiomyomas may outgrow their blood supply when they enlarge, causing various types of degeneration; these include hyaline, cystic, myxoid, fatty, infection, red degeneration, dystrophic calcification.[5,6] Hyaline degeneration occurs in 60% of leiomyomas.[3] Cystic degeneration is seen in 4% of leiomyomas. It results in formation of cystic areas with solid contents, or a cavity with irregular walls. Usually these areas are small and scattered. A single large cystic cavity is unusual.[7,8] Such a cavity is not lined by any epithelium and cannot be called a cyst. Hence we call it a pseudocyst, though it is called as a cyst in published scientific literature. Adenomyosis is another important cause of intramyometrial cysts. Histopathology is diagnostic. Congenital anomalies in the form of uterine cysts and echinococcal cysts are uncommon. The former can be diagnosed on histopathology, while the latter can be diagnosed on gross examination as well as histopathology.[7,8] Nabothian cysts are diagnosed clinically by their location in the cervix and gross appearance, and the diagnosis can be confirmed histopathologically, if they are removed at all. The diagnosis of cystic degeneration of a leiomyoma was confirmed on histopathological examination in our case.

Conclusion

Cystic lesions are uncommon in the uterus. Cystic degeneration of leiomyoma and adenomyosis are common causes of this condition. Usually these are cysts are small, but occasionally they can be large.

Acknowledgement

We than Dr Vijyeta Jagtap for the gross images of the specimen.

References
  1. Chopra S, Lev-Toaff AS,Ors F, Bergin D. Adenomyosis: Common and Uncommon Manifestations on Sonography and Magnetic Resonance Imaging. Journal of Ultrasound in Medicine. 2006,25(5):617-627.
  2. Kumar A, Kumar A. Myometrial cyst. Journal of Minimally Invasive Gynecology. 2007;14(4):395–396.
  3. Low SCA, Chong CL. A case of cystic leiomyoma mimicking an ovarian malignancy. Ann Acad Med Singapore. 2004;33:371–4.
  4. Maria Luisa C Fogata, Kiran A Jain. Case report: Degenerating cystic uterine fibroid mimics an ovarian cyst in a pregnant patient. J Ultrasound Med. 25:671-674–0278-4297. [PubMed]
  5. Okizuka H, Sugimura K, Takemori M, Obayashi C, Kitao M, Ishida T. MR detection of degenerating uterine leiomyomas. J Comput Assist Tomogr. 1993;17:760–6. 
  6. Karim S Ahamed, Gregory S Raymond. Answer to case of the month #103 large subserosal uterine leiomyoma with cystic degeneration presenting as an abdominal mass. Can Assoc Radiol J. 2005;56:245–7.
  7. Murase E, Siegelman ES, Outwater EK, et al. Uterine leiomyomas: histopathologic features, MR imaging findings, differential diagnosis, and treatment. Radiographics. 1999;19:1179–97.
  8. Prayson RA, Hart WR. Pathologic considerations of uterine smooth muscle tumors. Obstet Gynecol Clin North Am 1995; 22:637-657.
Citation

Chawla T, Parulekar SV, Fernandes G, Rojekar A. Intramyometrial Pseudocyst. JPGO 2016. Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/intramyometrial-pseudocyst.html

Cornual Leiomyoma And Patent Tube

Image
Author Information

Jagtap V*, Valvi D**, Parulekar S V***
(*Third Year resident, **Assistant Professor, ***Professor and Head, Department of Obstetrics and Gynecology, Seth G. S. Medical college and KEM hospital, Mumbai, India.)

Abstract

Leiomyomas are the commonest tumors of benign origin in females. Usually a leiomyoma does not cause infertility, unless it obstructs the fallopian tubes. We present a case in which the fallopian tube was patent despite the presence of a cornual leiomyoma.

Introduction

Leiomyomas are benign monoclonal tumors originating from smooth muscle cells of myometrium of uterus.[1] Its incidence varies from 35% to 80% depending upon age and ethnicity.[2] Leiomyomas may be seen in up to 10% of women with infertility. However only up to 2.5% cases of infertility show leiomyoma as the only cause of infertility with all other possible factors ruled out.[3] Usually a leiomyoma does not cause infertility, unless it obstructs the fallopian tubes. This may occur with a large leiomyoma or multiple leiomyomas which alter the normal pelvic anatomy, or a cornual location which causes obstruction of a fallopian tube, the other fallopian tube being obstructed due to any other reason. We present a case of infertility in which in which a fallopian tube was patent despite the presence of a leiomyoma exactly medial to the origin of the tube.


Case Report

A twenty eight year old woman, married since four years presented for management of primary infertility. She had regular and normal menstrual cycles with no medical or surgical comorbidity. All her pre-operative investigations for fitness for anesthesia, thyroid function test, serum prolactin levels, Pap smear and her husband’s semen analysis were within normal limit. Hence she was posted for diagnostic hystero-laparoscopy with chromopertubation and uterine curettage. Hysteroscopy showed normal findings and on laparoscopy uterus, bilateral ovaries and fallopian tubes were normal. Only incidental finding was a small 1.5×2 cm cornual leiomyoma on left side. On chromopertubation dye spillage was present bilaterally. The patient made an uneventful recovery.


Figure 1. Left cornual leiomyoma (arrows). Remaining pelvic findings are normal.

Discussion

Leiomyoma may cause infertility due to obstruction of the fallopian tubes, as with large or multiple leiomyomas which alter the pelvic anatomy, or cornual location of the leiomyoma which can obstruct only one fallopian tube. A cornual leiomyoma may also cause tubal ectopic pregnancy. Other causes of infertility due to uterine leiomyomas include distortion of the uterine cavity, inflammation of endometrium, interference with implantation, altered tubal contractility, cervical obstruction or due to obstruction of proximal tubal end.[4] Lower fertility rates are seen with submucous leiomyomas but not with subserosal or intramural leiomyomas.[5]
A cornual leiomyoma can be dignosed with ltrasonography, aided by hysterosalpingography or sonohysterography. Comuterized tomography or magnetic resonance imaging are not necessary. The diagnosis can be confirmed by laparoscopy, and the tubal patency can be tested at that time with chromopertubabtion, as in the case presented. This patient had a cornual leiomyoma and the fallopian tube appeared to arise right from its lateral aspect. The leiomyoma was large enough to obstruct the fallopian tube. However the tube was found to be patent on chromopertubabtion. This highlights the point that the mere presence of a cornual leiomyoma should not lead to the presumption that the fallopian tube on that side would be obstructed. This patient remains at risk of development of a tubal ectopic pregnancy on that side. No active management is required in such a case. She was counseled about the risk of development of an ectopic pregnancy and advised to report if she missed a period and developed lower abdominal pain, fainting, or vaginal bleeding. Other treatment options for cornual leiomyomas include GnRH agonists to reduce size of the fibroid,[6] myomectomy with tubal reimplantation (more of historical importance) and in vitro fertilization and embryo transfer, in case the other fallopian tube is obstructed too.

Acknowledgments

We thank Dr Rashmi Prasad for taking the operative photograh.

References
  1. Leppert PC, et al. A new hypothesis about the origin of uterine fibroids based on gene expression profiling with microarrays. Am J Obstet Gynecol 2006;195:415-420.
  2. Day Baird D, Dunson DB, et al. High cumulative incidence of uterine leiomyoma in black and white women: ultrasound evidence. Am J Obstet Gynecol 2003; 188:100-107.
  3. Buttram VC Jr, Reiter RC. Uterine leiomyomata: Etiology, symptomatology and management. Fertil Steril. 1981;36:433–445.
  4. ASRM Practice Committee. Myomas and Reproductive functions. Fertil Steril. 2008;90(3)Suppl 3: S126-130.
  5. Pritts E, Parker W, et al. Fibroids and infertility: an updated systematic review of the evidence. Fertil Steril 2009,91:1215-1223.
  6. Gardner RL, Shaw RW. Cornual fibroids: a conservative approach to restoring tubal patency using gonadotropin releasing hormone agonist(goserelin) with successful pregnancy. Fertil Steril 1989,52(2):332-4.
Citation

Jagtap V, Valvi D, Parulekar SV. Cornual Leiomyoma And Patent Tube.  JPGO 2016. Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/cornual-leiomyoma-and-patent-tube.html

Takayasu Arteritis Presenting With Severe Preeclampsia and Intrauterine Fetal Demise

Author Information

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

Abstract

Takayasu arteritis (TKA) is a vasculitic disorder characterized by involvement of aortic arch and its branches. In pregnant patients, it is commonly associated with preeclampsia, intrauterine fetal demise (IUFD) and increased morbidity. A multigravid patient presenting with severe pre-eclampsia and IUFD, who was found to have Takayasu arteritis is reported here.

Introduction

Preeclampsia presents in myriad ways. In a patient who presents in the index pregnancy with severe preeclampsia with no prior medical evaluation, a thorough initial examination is imperative, and can lead to detection of hereto unrecognized systemic disturbances.

Case Report

A 30-year-old female married since 17 years G4P2L2A1, with eight months gestation presented to our emergency room with chief complaints of abdominal pain, giddiness and vomiting. She was unregistered, non-immunized, and was apparently asymptomatic till the day of presentation. Headache started in the occipital region and was throbbing in nature. There were no complaints of vaginal bleeding, convulsions or decreased urine output. There was no previous significant major medical or surgical history. She had two normal, uneventful home deliveries.
Patient was conscious and well oriented, with a pulse of 88 beats per minute, recorded in right radial artery and blood pressure of 170/110 mm Hg recorded in the right brachial artery. Pulses in left radial and brachial artery were not palpable, while other peripheral pulsations on left side, namely femoral, and dorsalis pedis were palpable. There were no ischemic changes over the left upper limb. History was reviewed and patient did not complain of any pain, claudication or weakness of the left upper limb. Neurological assessment did not reveal any abnormality. A physician was consulted and Doppler studies were advised. Cardiovascular and respiratory systems were unremarkable. On abdominal examination uterus was relaxed, 26 weeks size, and fetus in vertex presentation. Fetal heart sounds could not be heard on Doppler, and ultrasonography confirmed IUFD. Internal cervical os was closed and uneffaced. Premonitory symptoms were present in the form of headache, vomiting and epigastric pain, hence magnesium sulfate by Pritchard regimen was started.
A few hours later, patient developed a syncopal attack, from which she improved within a few minutes. Magnesium sulfate was discontinued and injectable phenytoin was started after physician consultation. Through the course of her stay in the hospital for around 6 days, she developed 4 episodes of syncope. History was further reviewed and patient reported having similar episodes of syncope occasionally, which had never been reported or evaluated before.
Investigations revealed mild thrombocytopenia, elevated liver enzymes (SGOT 244 and SGPT 178U/l) and otherwise normal hematological and biochemical profile. Pre-induction ripening of cervix was done with Foley catheter. Labor was augmented with oxytocin and a fresh still born female of 1024 grams was delivered. Lactation suppression was initiated.
Upper limb Doppler flows were studied which showed monophasic waveform in left ulnar, radial, brachial arteries, suggestive of occlusion. CT angiogram done post delivery confirmed long segmental complete occlusion of left subclavian and left axillary artery, with presence of calcification. However, there was no evidence of thrombosis. Cardiac echogram showed structurally normal heart without any valvular lesions or pulmonary artery hypertension. Doppler of both renal arteries did not show any evidence of renal artery stenosis.
Serum TSH was high (9.14) hence l 50 microgram of levothyroxine was started. Autoimmune markers were sent, but were negative for ANA, Anti-dsDNA, LA, aCL antibodies (IgM & IgG). Further evaluation, steroids and stenting of the subclavian artery will be planned on follow up after the puerperal period.


Figure 1. CT angiography (arrow pointing to subclavian stenosis) .

Discussion
The initial presentation of the patient was similar to classic presentation of severe preeclampsia, except that the asymmetric pulse and blood pressure recording pointed towards a peripheral arterial problem. However, the diagnosis of Takayasu arteritis could be ascertained only after the results of the imaging features was suggestive of subclavian stenosis. The occurrence of syncopal attacks also strongly supported the diagnosis. Ours being a tertiary academic center, such diagnosis could be reached with multi-disciplinary consultation.
Takayasu's arteritis is a chronic, relapsing, stenotic, inflammatory disease of medium and large-sized arteries with a propensity for the aortic arch and its branches. Incidence is around 2.5 cases per million and is more prevalent among young women and in Asians.
Hypertension is seen in up to 90% of patients with TKA.[1] However, since the patient had not received any prior antenatal care, it is postulated that hypertension was present but not detected due to lack of medical attention; her presentation was one of hypertension with superimposed preeclampsia.
Though vasculitis, i.e. histopathologic demonstration of inflamed vessels is the gold standard, it is rarely required for establishing the diagnosis, and was not done in our patient.[1] The classical CT angiographic findings include transmural calcific changes and luminal narrowing,[2] both of which were present in our patient (Figure 1). Narrowing of the arterial lumen occurs with thrombosis; however CT scan in our patient did not show the presence of any thrombus.
Pregnancies with Takayasu arteritis have been reported to have a 13-fold higher rate of obstetric complications compared to normal pregnancies. [3] Our patient presented with a blood pressure of 170/110 mmHg and intrauterine fetal demise. Pre-eclampsia, premature delivery and IUFD have been reported to be of higher incidence in patients with TKA.[4, 5, 6]
There were no cardiac events or electrocardiographic abnormalities in our patient. Cardiovascular events are a common feature in this condition. However the occurrence of the same during pregnancy has been reported to be rare,[7] as was in our case. Pulmonary hypertension is a common feature,[8] but it was ruled out in our case on cardiac echogram. The occurrence of renal artery stenosis along with concomitant subclavian stenosis detected in pregnancy has also been reported by Nalini et al.[9]
Our patient was also evaluated objectively for antiphospholipid antibody syndrome (APLA). ANA, Anti-ds DNA, aCL, LA, which were all negative. However, Anti-beta 2 glycoprotein antibody could not be done due to financial constraints. Hence, concomitant APLA syndrome cannot be ruled out. It is interesting to note that aortoarteritis has been postulated to be a precursor to APLA syndrome.[10, 11]
The treatment principles include glucocorticoids, control of hypertension, prevention of renal failure, and arterioplastic approach to stenosed vessels.[1]
To conclude, a vigilant initial general examination led to an early suspicion of a concomitant systemic cause in this patient, a rare vasculitic syndrome. Like in this case, grave medical disorders could first be recognized during pregnancy, and the opportunity should be used for thorough investigation and management. Control of blood pressure appears to be the cornerstone in the management of this disease in pregnancy. A multidisciplinary approach is needed to ensure good obstetric and neonatal outcome. This case is being presented due the novel occurrence of the rare vasculitic condition in pregnancy.

References
  1. Langford AC, Fauci AS. The Vasculitis Syndromes. In: Longo LD, Kasper DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J. Eds. Harrison’s Principles of Internal Medicine. 18th edition. McGraw Hill.
  2. Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis: imaging spectrum at multidetector CT angiography. Br J Radiolog. 2012 Dec; 85(1020): e1282–92.
  3. Comarmond C, Mirault T, Biard L, Nizard J, Lambert M, Wechsler B, et al. Takayasu Arteritis and Pregnancy. Arthritis Rheumatol. 2015 Dec; 67(12): 3262–9
  4. Mandal D, Mandal S, Dattaray C, Banerjee D, Ghosh P, Ghosh A, et al. Takayasu arteritis in pregnancy: an analysis from eastern India. Arch Gynecol Obstet. 2012 Mar; 285(3): 567–71.
  5. De Jesús GR, d’Oliveira IC, dos Santos FC, Rodrigues G, Klumb EM, de Jesús NR, et al. Pregnancy may aggravate arterial hypertension in women with Takayasu arteritis. Isr Med Assoc J. 2012 Dec;14(12): 724–8
  6. Suri V, Aggarwal N, Keepanasseril A, Chopra S, Vijayvergiya R, Jain S. Pregnancy and Takayasu arteritis: a single centre experience from North India. J Obstet Gynaecol Res. 2010 Jun; 36(3): 519–24.
  7. Tanaka H, Tanaka K, Kamiya C, Iwanaga N, Yoshimatsu J. Analysis of pregnancies in women with Takayasu arteritis: complication of Takayasu arteritis involving obstetric or cardiovascular events. J Obstet Gynaecol Res. 2014 Sep; 40(9): 2031–6.
  8. Wang X, Dang A, Chen B, Lv N, Liu Q. Takayasu arteritis-associated pulmonary hypertension. J Rheumatol. 2015 Mar; 42(3): 495–503.
  9. Nalini S, Santa SA. Takayasu Arteritis with Bilateral Renal Artery Stenosis and Left Subclavian Artery Stenosis in Pregnancy. J Clin Diagn Res. Sep; 9(9):QD07-8. doi: 10.7860/JCDR/2015/14371.6485. Epub
  10. Dhaon P, Das SK, Saran RK, Parihar A. Is aorto-arteritis a manifestation of primary antiphospholipid antibody syndrome? Lupus. 2011 Dec; 20(14):1554–6.
  11. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006 Feb; 4(2): 295–306.
Citation
Madhva Prasad S, Puri J, Gupta AS. Takayasu Arteritis Presenting With Severe Preeclampsia and Intrauterine Fetal Demise.  JPGO 2015. Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/takayasu-arteritis-presenting-with.html

Congenital Granular Cell Tumor (Epulis) Diagnosed Antenatally On 3d Ultrasound And Its Postnatal Management

Author Information

Chhatrapati AK*, Asarkar TJ **, Mishra I *, Jassawalla MJ***
(* Assistant Professor, *** Honorary Professor, Department of Obstetrics and Gynecology, Nowrosjee Wadia Maternity Hospital and Seth G.S. Medical College, ** Senior Registrar, E.N.T., Bai Jerbai Wadia Children's Hospital, Mumbai, India)

Abstract

Congenital Granular Cell Tumor (GCT), commonly called congenital epulis or Neumann’s tumor is a rare benign tumor of the maxillary or mandibular gingival margin. It may be detected antenatally or can present at birth. If large in size, it can lead to respiratory obstruction or difficulty in swallowing. Surgical excision is needed in most cases with favorable outcome. We present a case of antenatally diagnosed congenital epulis which was surgically excised with no complication at 4 month follow up of baby.

Introduction

Epulis is a growth on alveolar mucosa. Congenital epulis also known as granular cell tumor was first described by Neumann in 1871. Although it is a very rare tumor it is common in Caucasians, with female preponderance (about 8-10 times more than male infants).[1,2] Diagnosis may be antenatal, as in our case, or postnatal.

Case report

A 26 year old lady, married since 3 years, non consanguineous marriage, a 3rd gravida with previous two miscarriages, with 1.5 months amenorrhea was booked in our hospital at 6 weeks gestation. On obstetric history, she had two previous spontaneous incomplete abortions at 4 months and 3 months respectively, each followed by check curettage. Her blood investigations, dating scan and scan at 12 weeks 5 days for nuchal translucency and nasal bone were normal. Her anomaly scan done at 18 weeks 1 day was normal, with cervical length of 3.5 cm.
At 30 weeks of gestation on her routine growth scan, patient was diagnosed to have a single live intrauterine fetus with gestational age 30 weeks 3 days, with adequate liquor of 18 cm, with a 2 x 1.6 x 1.1 cm echo- poor mass, seen protruding from the region of fetal oral cavity, in the midline. The mass was probably seen arising from mandible or epignathus. She underwent 3D scan, in which a 2 cm x 2 cm well defined hypoechoic lesion was seen protruding from fetal mouth, arising from mandibular alveolus with normal fetal tongue and maxilla, as seen in figures 1 and 2.


Figure 1: 2D/3D antenatal USG showing mandibular mass.


Figure 2: 3D antenatal USG showing mandibular mass on lateral view.

Antenatally, pediatric surgery reference was taken; they opined that the mode of delivery would not affect the postnatal outcome, and that the mass would be surgically excised after delivery. Glucose tolerance test was repeated and values were, fasting: 76 mg/dl, 1 hour: 92 mg/dl, 2 hour: 84 mg/dl. Patient was called for weekly follow up for fetal well being and growth monitoring. At 38 weeks gestation, patient came with spontaneous labor pains and delivered uneventfully a female child of 2.8 kg with an Apgar score at of 8/10 at 1 minute. Baby was admitted in NICU for evaluation of the mandibular mass and was given Ryle's tube (RT) feeds as the mass was obstructing normal swallowing. There was no respiratory obstruction.


Figure 3: The newborn baby with mandibular mass.

On day 2 of life, ENT evaluation and USG was done. USG was suggestive of a pedunculated hypoechoic lesion 2 x 1.2 cm originating from lower alveolar mucosa, containing both arterial and venous component, suspected to be neoplastic.
On day 10, injection triamcinolone 0.1 mg per kg and injection dexamethasone 0.4 mg per kg was given for sclerosing the vascular component. Bleomycin was not given as the mass was flush to mandible and could cause mandibular necrosis and later pulmonary fibrosis in the baby. After 2 weeks, the tumor shrunk to 50% of its size and on day 24 of life, laser- assisted excision of the tumor was done. Wound was left open for mucosal healing. After 1 week the wound healed completely. Trial with breast feeding was attempted on day 32 of life (before that only RT feeds were given). Baby successfully established suckling and swallowing. Baby was discharged on 10th post operative day. Histopathology revealed congenital epulis (granular cell tumor) with clear margins.


Figure 4: Post operative gingival margin.


Figure 5: Gross appearance of the mass post excision.


Figure 6: Histopathology showing squamous epithelium.


Figure 7: Histopathology showing stromal polygonal cells with eosinophilic cytoplasm and blood vessels

Discussion

In cases of epulis, the upper gingival margin, that is maxilla is three times more affected than mandible. On gross external examination, it is seen as a pink coloured growth on alveolar margin with smooth surface; often it is pedunculated. It generally affects canine or incisor area.
On histopathological examination, it originates from the epithelial cells of dental lamina (odontogenic origin). Sometimes epulis originates from fibroblasts, pericytes, myocytes, smooth muscle cells, histiocytes and neural crest cells. In a recent study by Aparna et al on immuno-profiling of the epulis, cells of mesenchymal origin with myofibroblastic features were identified. They were contractile fibres and produced collagen, and eventually underwent auto-phagocytosis.[3]
In immunohistochemical study on the histogenesis performed by Leocata, markers for lysozyme, macrophage marker CD68, and HLA-DR were present. Epithelial markers and S-100 protein, which are specific for Schwann cells were absent.[4] Although there is female preponderance, the tumor does not have estrogen receptors and is hormone independent.[1][5] Malignant transformation in the mass is also not found. [6][7]
Diagnosis can be done antenatally by ultrasonography.[8] In intrauterine life, a big mass in oral cavity can hamper fetal swallowing and result in polyhydramnios.[9,10] The tumor is rarely detected before third trimester.[11] Antenatally other oral lesions as dermoids, lymphatic malformations, osteogenic or chondrogenic sarcomas, rhabdomyosarcoma can have similar ultrasound features and should be ruled out postnatally.[12]
If the lesion is huge and can potentially obstruct neonatal airway during a vaginal delivery, due to mechanical obstruction or by traumatic disruption of tumor, elective lower segment cesarean section with ex- utero intrapartum treatment (EXIT) can be attempted.[13] EXIT includes elective intubation and mechanical ventilation of the neonate before placental separation and clamping of the cord, so that hypoxia can be avoided.[14]
Postnatally, surgical excision is the preferred form of treatment for large granular cell tumors as they interfere with breathing, suckling and swallowing.[15] Smaller lesions can be monitored for spontaneous regression.[16,17] If an alveolar defect is created during surgical excision, normal dental development can be affected. In such cases, subperiosteal undermining with gingivoperiosteal flap suturing should be done. Carbon dioxide laser excision is another good option which was done in our institute. [18]

Conclusion

Congenital epulis can be diagnosed in an antenatal ultrasound mostly in third trimester. A multidisciplinary approach and an institutional delivery with good NICU care is advocated. Confirmation of the diagnosis can be done only after histopathological examination. Postnatal USG and MRI can guide the extent of penetration. Small tumors can be monitored as they may regress in size but larger ones need surgical excision as they can cause mechanical obstruction to respiratory and oral tract. Even on long term follow up, recurrence or malignant transformation of the tumor mass is not seen.

References

  1. Olson JL, Marcus JR, Zuker RM. Congenital epulis. J Craniofac Surg 2005; 16(1):161-4.
  2. Eghbalian F, Monsef A. Congenital epulis in the newborn, review of the literature and a case report. J Pediatr Hematol Oncol. 2009 Mar; 31(3):198-9.
  3. Aparna HG, Jayanth BS, Shashidara R, Jaishankar P. Congenital epulis in a newborn: a case report, immunoprofiling and review of literature. Ethiop J Health Sci. 2014; 24(4): 359-62.
  4. Leocata P, Bifaretti G, Saltarelli S, Corbacelli A, Ventura L. Congenital (granular cell) epulis of the newborn: A case report with immunohistochemical study on the histogenesis. Ann Saudi Med 1999; 19(6):527-9.
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  18. Dash JK, Sahoo PK, Das SN. Congenital granular cell lesion "congenital epulis”-report of a case. J Indian Soc Pedod Prev Dent 2004; 22(2):63-7.
Citation

Chhatrapati AK, Asarkar TJ, Mishra I, Jassawalla MJ. Congenital Granular Cell Tumor (Epulis) Diagnosed Antenatally On 3d Ultrasound And Its Postnatal Management. JPGO 2015.  Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/congenital-granular-cell-tumor-epulis.html