Volume 5 Number 6

Editorial
Madhva Prasad Sarvothaman

Spontaneous Cervical Perforation
Kaviya .S, Parulekar SV.

Pregnancy With Chronic Renal Anemia
Khadke B, Prasad M, Gupta AS.

Transverse Lie And Dystocia Due To Asherman Syndrome
Desai A, Parulekar SV.

A Case Of Pregnancy With Coexisting Systemic Sclerosis And Paucibacillary Leprosy
Nasare P, Prasad M, Gupta AS.

Chronic Nonpuerperal Inversion Of Uterus Secondary To Submucous Myoma
Jain N, Chaudhari H, More V.

Giant Sacrococcygeal Teratoma
Parihar A, Warke HS.

Cesarean Myomectomy
Malani K, Jagtap S, Swaminathan G, Samant PY.

Scar Endometriosis
Nebhani M, Shah R, Warke HS.

Remembering Past Greats: James Marion Sims
Pai K.

Editorial

Madhva Prasad Sarvothaman

As the adage goes, “the biggest sophistication is simplicity”. Many a time we prefer to advocate modern and advanced methods of treatment, but overlook simple solutions. One may recall that the WHO definition of health states it not to be merely the absence of disease. In the context of the twenty first century, lack of physical activity, and life-style disorders are threatening to setback all medical achievements. No medical professional should neglect this aspect of a woman’s life - physical activity. Though it appears trivial, the relevance of this aspect to women’s health is profound.

A brief discussion on the inter-relationship of physical activities, physical fitness, exercise and sports, with women’s health is presented here. Physical activity usually gains importance during the first two decades, where many females may enrol in sporting activities. It may be noted that till the turn of the 20th century, there was a Victorian-era belief that sports was exclusive for men. For example, though modern Olympics started many years prior, athletics and gymnastics were allowed for women only in 1928.
Brings us to the problems faced by female athletes. A recent meta-analysis (Teixeira, 2018) showed that a 36% prevalence of urinary incontinence among female athletes. This is much higher than the average population. Similarly, it has been reported (Nygaard, 2016) that there is a higher likelihood of anal incontinence among women engaging in intense activity, when compared with sedentary women. It is a matter of responsibility of the trainers, coaches and other caregivers of these athletes to sensitize them about possible urogynecological problems. Further research in this matter should be encouraged (Goldstick, 2014).
The female athlete triad consisting of eating disorders (low energy availability), osteopenia and oligo/amenorrhea is a peculiar condition among competitive athletes with lifelong gynaecological consequences. The knowledge of this triad among specialty physicians has been demonstrated to be low (Curry, 2015) and there is need for improving awareness about the same. 
Moving onto the reproductive age group, the first condition that comes to mind in relation to physical activity is PCOS. It is to be noted that while hormonal manipulation treats menstrual issues and hyperandrogenism, a regular structured effective physical activity serves to improve cardiac, metabolic and pulmonary profile of these patients (Orio, 2016). The overall advantages of physical activity in PCOS is imperative. Moving on to fertility issues, it has been shown that lifestyle with adequate physical activity in the year preceding the artificial reproduction therapy, favourably impacted outcomes in IVF (Evenson, 2014).
In today’s context, what kind of physical activity a pregnant woman can engage in, forms a part of any antenatal counselling session. It has been shown that moderate-intensity walking resulted in improvement of the levels of physical fitness in pregnant women, who are otherwise healthy, but previously led sedentary lifestyles. Simple benefits include reduced occurrence of pelvic girdle pain during pregnancy. It was also shown that such activity did not have any detrimental effect on fetal growth or fetoplacental blood flow (Oliveira Melo, 2012). Similarly, there is data to show that supervised prenatal exercises minimizes the chance of having a large new born, without altering the chance of delivering a small newborn (Wiebe, 2015). There has been consensus that those engaged in the healthcare of pregnant women should encourage them to undertake moderate exercise (Barakat, 2015). Reduction of preeclampsia risk has been noted (Wolf, 2014).  In the Indian context, structured physical activity has also resulted in reduction of the risk of GDM and improved outcomes (Uma, 2017).
It is noteworthy that published structured guidelines for physicians regarding how to advice patients on physical activity are freely available (ACOG, 2017). One study has also evaluated and concluded that following such structured guidelines were particularly beneficial (Davari Tanha, 2014). Despite the availability of such resources, there appears to be a gap between the existing knowledge and actual practice by physicians in this aspect (Watson, 2015). An introspection into whether we can add encouragement of moderate physical activity to all our antenatal clientele is called for.

It is important to know the contraindications to physical activity in pregnancy. The absolute contraindications are haemodynamically significant heart disease, restrictive lung disease, incompetent cervix/cerclage, multiple gestation, persistent second or third trimester bleeding, placenta praevia after 26 weeks, premature labour and pregnancy induced hypertension. Other relative contraindications exist.  Physical activity and exercise promoting methods in labour such as “birthing ball” have also been tried, but such options are in the nascent stage of research. (Luces Lago 2014)

Moving back briefly to sports, there are some interesting areas where little knowledge is available. “Should oral contraceptives be offered to reduce ligament injury?” One study showed 20% reduction in the risk of anterior cruciate ligament injury among women on OCPs. (Herzberg, 2017) “Is it safe for women who are pregnant to undergo scuba diving/ parachuting?” While some opine that a pregnancy test is mandatory, generating reliable evidence in such matters can be a real squabble. (Damnon, 2016; Ebner, 2014)

Next on the common gynecological symptomatology. Women who present with pelvic organ prolapse needing surgery are more likely to give history of heavy work when compared to the average population. On the other hand, mild to moderate physical activity actually reduces the risk of developing urinary and fecal incontinence (in contrast to sportswomen who have a higher risk). Hence it is clear that the nature and amount of physical activity impacts the gynaecological symptomatology. A balance between apt amount to avoid sedentary and obesity risks, and excessive amount which may cause gynaecological issues appears necessary.

Menopause is one aspect where exercise training has consistently been shown to reduce symptomatology such as hot flushes, blood lipid levels and body weight. (Mendosa, 2016) Finally, a trial – REWARD (Revving-up Exercise for sustained Weight loss by Altering neurological Reward and Drive) has noted beneficial effects of physical activity among women being treated for endometrial cancer.
As is seen above, the beneficial effects of appropriate moderate amount of physical activity on women’s health is obvious. The onus lies on the healthcare provider to advocate this simple tool to significantly improve women’s health.

With this, we welcome you to yet another interesting edition of this journal, featuring a variety of cases. One article is dedicated to the enigmatic and controversial Dr James Marion Sims. We hope that you will enjoy this month’s fare.

Pregnancy With Chronic Renal Anemia

Author Information

Khadke B*, Prasad M**, Gupta AS***.
(* First year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

A case of pregnancy associated with chronic renal disease, management of pregnancy and possible outcomes in women with chronic kidney disease, managed with erythropoietin injection is presented here.

Introduction

Chronic kidney disease is classified in five stages. All five stages are associated with increased risk of preterm labor, pre-eclampsia and intra uterine growth restriction, 86% preterm deliveries occur in women with serum creatinine above 2.5 mg/dl and more than 40% pregnancies are associated with pre-eclampsia. Pregnant woman with high level of serum creatinine have more decline in renal function than non-pregnant woman with the same level of creatinine. Approximately 30 % have increased risk of fetal loss when serum creatinine ranges between 1.6 to 5.5 mg/dl, 50% women with serum creatinine more than 1.5mg/dl have decline in glomerular filtration rate, and among them 20% developed end stage renal disease in the postpartum period. 

Case Report

A 19 year old primigravida married for 1 year presented to the outpatient department at 28 weeks of gestation with diagnosed chronic kidney disease. Approximately 2 months before conception, she had developed an episode of fever which was diagnosed as enteric fever by the positive Widal test showing infection with Salmonella typhi O and H. She was treated with antibiotics for the same. Ultrasonography (done by a practitioner in a remote rural area) revealed hepato-splenomegaly, but no specific comment on any abnormality in the kidneys was mentioned in the report. However, in view of continuing abdominal pain, vomiting, low grade fever and multiple joint pains, she presented to our tertiary care hospital for further evaluation, 2 months after this initial episode. Investigations showed raised serum creatinine of 2.33 mg/ dl, raised blood urea of 86.7 mg % and raised blood urea nitrogen as 40.35 mg % for which she was evaluated. Ultrasonography showed renal parenchymal disease (grade III in right kidney and grade II in left kidney) and left moderate hydronephrosis. Investigations also showed anemia (hemoglobin 8.8 gm%) and proteinuria (2+) on urine routine examination. Diagnosis of chronic kidney disease with renal anemia was made, and she was managed on outpatient basis by nephrology department. There was no requirement for dialysis, but was advised close follow up.
She was given injection iron carboxymaltose 1000 mg and injection erythropoietin 2000 IU two doses; tablet sodium bicarbonate 1 gram thrice a day and multivitamin tablets twice a day. She was advised further work up but did not comply and was lost to follow up. Five months later she visited the nephrology department and reported amenorrhea for the prior 5 months. Pregnancy was diagnosed and she was referred for antenatal registration. She and her relatives were counseled about risk involved in pregnancy with kidney disease and was advised admission. However, she got admitted two months later, at 33 weeks. She was comfortable, mild pallor was present, there was no facial puffiness, pedal edema, anasarca or ascites. Blood pressure was 140/90 mm Hg, urine albumin was 1+, systemic examination was otherwise unremarkable and uterine size was corresponding to period of gestation.
Nephrologist reviewed her. Salt intake of only 2 grams per day, protein intake of 0.8 grams per day, maintaining an input corresponding to the output, abdominal girth monitoring and daily weight monitoring were initiated. Tablet acetylsalicylic acid 75 mg once a day, tablet sodium bicarbonate 1 gm thrice a day, calcium and iron supplements were started and continued. Her serum TSH value was 5.31 IU/ml and tablet levothyroxine 50 mcg was started.
As part of evaluation of multiple joint pains, levels of ANA, serum PTH, vitamin D were checked and found to be normal. Radiological evaluation of joints was also normal. Ophthalmologist excluded chronic changes in her retina. The investigations at the time of admission were blood urea nitrogen of 41 mg%, creatinine of 2.5 mg%, SGOT 32 U/L, SGPT 22 U/L, sodium 132 mEq/l, potassium 4.2 mEq/l, chloride 107 mEq/l, and 24 hour urine protein of 760 mg. In view of persistent anemia, which was of renal origin, nephrologists advised injection erythropoietin 2000 IU twice a week for a total of 3 weeks; which was given. Multivitamin injections were also continued.
Fetal growth parameters were monitored and the fetal growth was appropriate for gestational age. At 37 weeks, she went into spontaneous labor and LSCS was required due to thick meconium stained amniotic fluid with fetal distress in the first stage of labor. General anesthesia was given and intraoperative blood pressure rose to 180/120 mm of Hg that was controlled by intravenous labetalol. Male child of 2.316 kg was delivered, and postoperative period was otherwise uneventful. The neonate was evaluated and was found to have absent right kidney, but was discharged since there was no decrease in urine output. Outpatient follow up with pediatric nephrologist was advised.
The mother was continued on levothyroxine, iron and calcium supplements and sodium bicarbonate.

Discussion

Our patient did not know that she was suffering from chronic renal disease just prior to pregnancy. How long she had renal disease prior to its identification during pregnancy is not known. Webster et al opine that pregnancy is an opportunity to identify renal disease.[1] Despite advice of regular follow-up, she did not comply.  It has been shown that accurate diagnosis and intensive follow-up with specialist nephrologist increases the maternal survival and neonatal outcomes.[2] Despite poor follow-up, our patient had a blood urea nitrogen level of 40 mg%, which is within the target; 50 mg% is preferred among patients with chronic kidney disease and on dialysis.[3]
Our patient had anemia with hemoglobin of 8.8 gm% which did not improve with oral hematinics.  The incidence of anemia in patients with chronic renal disease can be as high as 80 %. A hemoglobin level of less than 9 gm% despite three weeks of oral hematinics is considered an indication for erythropoietin.[4]
A total of 6 doses of erythropoietin (2000 IU each dose) was required in our patient during pregnancy. The requirement of erythropoietin in pregnancy is higher than that required in non-pregnant state. The accurate dose of erythropoietin during pregnancy has not been definitively determined.  Other factors such as nutritional status, duration of disease and type of vascular access ports appear to be determinants. Erythropoietin does not cross the placenta, and is a FDA category C drug, approved for use in pregnancy when indicated for maternal indication. The therapeutic value of this drug lies in compensating for the reduced renal production of erythropoietin in renal disease.[5,6]
Our patient developed severe intrapartum hypertension. The occurrence of hypertension can be related to the kidney disease itself, or it may be secondary to the use of erythropoietin, and this is difficult to distinguish.  However, the hypertension was transient.[7,8]
Our patient had hypothyroidism, which was also detected during pregnancy, but did not appear to be the main cause of renal disorder. There have been case reports of severe hypothyroidism being the sole cause of renal disorder in pregnancy.[9]
The neonate weighed 2.3 kilograms. The occurrence of IUGR in mothers with kidney disease is well known. However, we would like to highlight the fact that recent research is being conducted to look at determinants of future chronic kidney disease. Newsome et al have recently opined that being born as an IUGR baby is in itself a risk factor for future development of renal disease as an adult, consistent with the theory of “fetal origin of adult disease”.[10]
In our patient, the neonate, surprisingly had a single kidney. An entity of hereditary renal dysplasia/ aplasia which has the features of absent kidney in the fetus and renal problems in the mother had been initially described by Buchta et al.[11] Though evaluation was being planned on a later date, the significance of this is not known.

Conclusion

Chronic renal disease may be first identified during pregnancy. There is a need to emphasize and counsel patients about rigorous follow-up. Anemia during pregnancy in renal disease may necessitate erythropoietin injection, and its use is safe. 

References

1. Webster P, Webster LM, Cook HT, Horsfield C, Seed PT, Vaz R, et al. A Multicenter Cohort Study of Histologic Findings and Long-Term Outcomes of Kidney Disease in Women Who Have Been Pregnant. Clin J Am Soc Nephrol. 2017;12(3):408–16.
2. Reddy SS, Holley JL. The importance of increased dialysis and anemia management for infant survival in pregnant women on hemodialysis. Kidney Int. 2009; 75(11):1133–4.
3. Sachdeva M, Barta V, Thakkar J, Sakhiya V, Miller I. Pregnancy outcomes in women on hemodialysis: a national survey. Clin Kidney J. 2017; 10(2):276–81.
4. Cyganek A, Pietrzak B, Kociszewska-Najman B, Sanko-Resmer J, Paczek L, Wielgos M. Anemia Treatment With Erythropoietin in Pregnant Renal Recipients. Transplant Proc. 2011 Oct; 43(8):2970–2.
5. Wolfson GH, Vargas E, Browne VA, Moore LG, Julian CG. Erythropoietin and Soluble Erythropoietin Receptor: A Role for Maternal Vascular Adaptation to High-Altitude Pregnancy. J Clin Endocrinol Metab. 2017; 102(1):242–50.
6. Ifudu O. Patient characteristics determining rHuEPO dose requirements. Nephrol Dial Transplant. 2002; 17 Suppl 5:38–41.
7. Piccoli GB, Cabiddu G, Castellino S, Gernone G, Santoro D, Moroni G, et al. A best practice position statement on the role of the nephrologist in the prevention and follow-up of preeclampsia: the Italian study group on kidney and pregnancy. J Nephrol. 2017; 30(3):307–17.
8. Kashiwagi M, Breymann C, Huch R, Huch A. Hypertension in a pregnancy with renal anemia after recombinant human erythropoietin (rhEPO) therapy. Arch Gynecol Obstet. 2002; 267(1):54–6.
9. Bora A, Madhva Prasad S, Gupta AS. Severe hypothyroidism causing minimal change nephropathy in early pregnancy. JPGO 2017. Volume 4 No.9. Available from: http://www.jpgo.org/2017/08/severe-hypothyroidism-causing-minimal.html
10. Newsome AD, Davis GK, Ojeda NB, Alexander BT. Complications during pregnancy and fetal development: implications for the occurrence of chronic kidney disease. Expert Rev Cardiovasc Ther. 2017; 15(3):211–20.
11. Rodriguez MM. Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT). Fetal Pediatr Pathol. 2014; 33(5–6):293–320.

Citation

Khadke B, Prasad M, Gupta AS. Pregnancy With Chronic Renal Anemia. JPGO 2018. Volume 5 No.6. Available from: http://www.jpgo.org/2018/06/pregnancy-with-chronic-renal-anemia.html

Transverse Lie And Dystocia Due To Asherman Syndrome

Author Information

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

Abstract

Intrauterine adhesions or uterine synechiae are bands of fibrous tissue that form in the endometrial cavity and sometimes the cervix. The degree of scarring can vary from thin strips of tissue to complete obliteration of the endometrial cavity. It can cause infertility or a number of obstetric complications. We present a case of uterine synechiae which caused a transverse lie and difficulty during delivery of the fetus during a cesarean section.

Introduction

The incidence of uterine synechiae is 0.14% to 0.47%.[1,2] It is commonly associated with dilatation and curettage, endometritis secondary to infections like tuberculosis, uterine surgery like myomectomy and cesarean section and and prolonged use of intrauterine device.[4,5] Patients who undergo dilatation and curettage for retained products of conception after a miscarriage or retained placenta are more likely to develop uterine adhesions. The effects range from menstrual disturbances to infertility, recurrent pregnancy loss and placental abnormalities, malpresentations and premature rupture of membranes.[6]

Case Report

A 25 year old gravida 4 para 1 living 1 spontaneous abortion 2,  registered at our tertiary care center at 27 weeks of gestation. She was referred from a private hospital in view of her ultrasonography (USG) finding suggestive of uterine synechiae on the lower uterine segment and placental insertion on it. She had one normal delivery in the past followed by two spontaneous abortions at 2 and 1.5 months of amenorrhea respectively for which she had undergone check curettage at a private hospital. Her subsequent menstrual periods had been irregular and the menstrual flow was scanty, associated with severe dysmenorrhea. She did not remember the date of her last menstrual period. USG at our center confirmed the findings of her previous USG. She was admitted at 39 weeks of gestation in antenatal ward in view of transverse lie and intrauterine fetal growth restriction for elective lower segment cesarean section. Her recent scan was suggestive of an intrauterine gestation of 35 weeks with transverse lie with fetal head towards maternal right and an intrauterine longitudinal adhesion band of 3 cm thickness, like a uterine septum. The placenta was attached to it. She underwent an elective LSCS with tubal ligation and delivered female child of 2.6 kg with an APGAR of 8/10. The baby had to delivered by a breech extraction. There was difficulty while delivering the head because it was held back by the intrauterine adhesions. A band of adhesion was noted  connecting the anterior, posterior and right lateral uterine walls (figures 1 and 2). The placenta was not attached to the band. The  band was clamped, cut and transfixed using polyglactin No.1 and tissue sent for histopathological examination. Rest of the operative course was uneventful. Patient had an uneventful postoperative course in the ward and was discharged on day 5. The histopathology showed hypertrophied smooth muscle covered with sheets of decidual stroma on one surface and no trophoblastic infiltration.

Figure 1. Intraoperative image showing the uterine band of adhesion extending from the inner surface of anterior (white arrow) to right lateral (green arrows) walls of the uterus.

Figure 2.  Intraoperative image showing the uterine band of adhesion extending from the inner surface of anterior (white arrow) to posterior (black arrows) and right lateral (green arrows) walls of the uterus

Discussion

Uterine cavity is lined by endometrium which is divided into the functional endometrium that is shed during menses and the basal layer which is involved in regeneration of endometrium. It is believed that trauma to the basal layer during surgical procedure like dilatation and curettage or due to infection can lead to development of adhesions which could be flimsy or dense. Dense adhesions could further obliterate the uterine cavity and the cervix. The risk of developing synechiae increases with the number of check curettage.[3] The incidence of uterine adhesions is equal in all races thus ruling out genetic predisposition for development of the same however uterine adhesions developing secondary to chronic endometritis is more common in developing countries due to high prevalence of tuberculosis.[7] Hysteroscopy can be used for diagnosis as well as treatment of the same. Myometrial adhesions are more common. They show a thin layer of endometrium with glandular ostia on the surface. Histology of thick bands of fibrous adhesion shows dense fibrous tissue without any overlying endometrium.[8] Diagnosis of the condition can be made based on the history and a high index of suspicion in a patient with menstrual complaints or infertility following history of curettage or any uterine surgery. Tests like Saline infusion sonograghy, hysterosalpingography or magnetic resonance imaging can be used for confirming the diagnosis.[9,10]  The treatment depends on the presenting complaints of the patient. Those seeking medical attention for infertility could be treated with hysteroscopic adhesiolysis followed by hormonal treatment with estrogen to stimulate endometrial growth and prevent recurrence of adhesion formation.[11] The adhesions may also lead to obstruction of outflow tract leading to cyclical pain during menses and obstruction to menstrual flow may cause endometriosis.[12] Hence prompt diagnosis and treatment are necessary. As recently pregnant uterus is soft and under hormonal influence it is easily injured during curettage. As prevention is better than cure, medical alternative for evacuation of products of conceptus like mifepristone and misoprostol could be considered as better alternative.[Zhang J, Gilles JM, Barnhart K, Creinin MD, Westhoff C, Frederick MM (August 2005). A comparison of medical management with misoprostol and surgical management for early pregnancy failure. The New England Journal of Medicine. 2005;353(8):761–9.] The risk of preterm premature rupture of membranes, placental abruption, and malpresentation requiring cesarean section is significantly increased with uterine synechiae.[13-15] Cho reported intrauterine fetal death due to horizontal intrauterine synechiae.[An intrauterine fetal death due to horizontal uterine synechia.[16]
Our patient had intrauterine restriction of fetal growth, probably due to reduced blood supply to the placental site due to fibrosis. The fetus was in a transverse lie, which was also due to intrauterine synechaie. The band trapped the fetal head during delivery by cesarean section and made the delivery difficult. The band was myometrial, as its histopathology showed smooth muscle in them. The development of this form of adhesion is difficult to explain as myometrial tissue of anterior and posterior wall would adhere to each other by fibrous tissue and not smooth muscle. It is possible if there is detachment of some part of the myometrium from one wall which gets adherent to another wall. But that could happen with major surgery, while our patient had not undergone any major surgery in the past.

Conclusion

Uterine synechiae are a reversible and potentially preventable cause of infertility and further pregnancy complications. In the event of a pregnancy successfully proceeding to full term, mode of delivery can be decided based on the lie and presentation.

References

1. Sato M, Kanenishi K, Ito M, Tanaka H, Takemoto M, Hata T. Antenatal 3-D sonographic features of uterine synechia. J Obstet Gynaecol Res 2013;39:395e8.
2. Tuuli MG, Shanks A, Bernhard L, Odibo AO, Macones GA, Cahill A. Uterine synechiae and pregnancy complications. Obstet Gynecol 2012;119:810e4.
3. Friedler S, Margalioth EJ, Kafka I, Yaffe H. Incidence of post-abortion intra-uterine adhesions evaluated by hysteroscopy--a prospective study. Human Reproduction. 1993;8 (3): 442–4.
4. March CM. Intrauterine adhesions. Obstet. Gynecol. Clin. North Am. 1995;22 (3): 491-505.
5. Klein SM, García CR. Asherman's syndrome: a critique and current review. Fertil. Steril. 1973;24 (9): 722-35.
6. Berman JM. Intrauterine adhesions. Semin. Reprod. Med. 2008;26 (4): 349-55.
7. Sharma JB, Roy KK, Pushparaj M, Gupta N, Jain SK, Malhotra N, Mittal S (January 2008). Genital tuberculosis: an important cause of Asherman's syndrome in India. Archives of Gynecology and Obstetrics. 2008;277(1): 37–41.
8. Al Inany H: Intrauterine adhesions. An update. Acta Obstet Gynecol Scand 2001;80:986-993.
9. Ball RH, Buchmeier SE, Longnecker M. Clinical significance of sonographically detected uterine synechiae in pregnant patients. J Ultrasound Med. 1997;16 (7): 465-9.
10. Letterie GS, Haggerty MF. Magnetic resonance imaging of intrauterine synechiae. Gynecol. Obstet. Invest. 1994;37 (1): 66-8.
11. Valle RF, Sciarra JJ. Intrauterine adhesions: hysteroscopic diagnosis, classification, treatment, and reproductive outcome. Am. J. Obstet. Gynecol. 1988;158: 1459-70.
12. Palter SF. High Rates of Endometriosis in Patients With Intrauterine Synechiae (Asherman’s Syndrome). Fertil Steril September 2005;84:S471.
13. Tuuli MG, Shanks A, Bernhard L, Odibo AO, Macones GA, Cahill A. Uterine synechiae and pregnancy complications. Obstet Gynecol. 2012;119(4):810-4.
14. Bavelloni A, Piazzi M, Raffini M, Faenza I, Blalock WL. Prohibitin 2: At a communications crossroads. IUBMB Life. 2015;67(4):239-54.
15. Lazebnik N, Hill LM, Many A, et al: The effect of amniotic  sheet orientation  on  subsequent  maternal  and  fetal complications. Ultrasound Obstet Gyneco18:267, 1996.
16. Cho JH An intrauterine fetal death due to horizontal uterine synechia. Taiwanese Journal of Obstetrics & Gynecology 56 (2017) 406-7.

Citation

Desai A, Parulekar SV. Transverse Lie And Dystocia Due To Asherman Syndrome. JPGO. 2018 Vol 5 No. 6. Available from: http://www.jpgo.org/2018/06/transverse-lie-and-dystocia-due-to.html

A Case Of Pregnancy With Coexisting Systemic Sclerosis And Paucibacillary Leprosy

Author Information

Nasare P*, Prasad M**, Gupta AS***
(* Third year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

The clinical characteristics of women who conceive during leprosy and systemic sclerosis and the association between it are not well known. A case of coexisting systemic sclerosis in which paucibacillary lepromatous leprosy developed secondarily, and its successful management in pregnancy is described here. To the best of our search of available literature, no cases of pregnancy with coexisting systemic sclerosis and paucibacillary leprosy were found. This appears to be the 1st case of pregnancy with coexisting systemic sclerosis and paucibacillary leprosy that is being reported.

Introduction

Systemic sclerosis is an autoimmune disorder with a wide variety of presentation, of which dermatological manifestation in just one component. Paucibacillary leprosy is an infective disorder caused by Mycobacterium leprae with a predominantly dermatological presentation. The occurrence of leprosy has drastically reduced in India. While the occurrence as an individual disease itself is low, its occurrence along with an autoimmune disorder like systemic sclerosis is even rarer. Successfully management of such a case encountered in pregnancy is described here.

Case Report

 A 28 year old married woman gravida 2 para 1 living 1 presented to our outpatient department at 36 weeks of gestation. She had registered elsewhere in the end of first trimester and was on regular antenatal follow-up, compliant to hematinics/ calcium and other medications. Her first pregnancy was a spontaneous conception with a full term LSCS performed for transverse lie and she delivered a healthy 3 kg male child. At that time, she did not have any medical disorders. One year after completion of her first pregnancy, she noticed tightening of skin over arms, an approximately 3*4 centimeter plaque on right cheek, increased sensitivity to light, redness of skin, decreased opening of mouth following which she consulted a dermatologist. Investigations were done, of which ANA was positive and ANA blot showed Scl-70 positivity with a titer of 1:320 and a diagnosis of systemic sclerosis was reached. X-ray of bilateral hands had been done which was suggestive of acro-osteolysis. Spirometry was suggestive of mild restrictive lung disease. She was started on tablet prednisolone (initially 15 mg once a day tapered and maintained at 10 mg), tablet hydroxychloroquine 200 mg once a day and moisturizing ointments for local application.  After 6 months of diagnosis she was started on tablet nifedipine 10 mg once a day for sclerodermic ulceration of digits and tablet pentoxyfylline 400 mg thrice a day was added.
Around five months prior to the current pregnancy, a skin biopsy was taken from the right cheek circular lesion as it was not resolving with ongoing treatment. It showed paucibacillary lepromatous leprosy. She was started on multidrug treatment regimen which included tablet dapsone (100 mg OD), rifampicin (600 mg OD), clofazimine (300 mg OD). Tablet prednisolone was stopped and tablet azathioprine and nifedipine were continued.
She came to know about pregnancy status at around 12 weeks of gestation and registered at the same time in outside hospital. She stopped antimycobacterial treatment on her own but continued sclerosis medication. Ultrasonography for fetal malformation was normal. Blood pressure was always within normal limits.
In our hospital she registered at 36 weeks of gestation. She was advised admission for further management but she did not get admitted. As per dermatologists’ advise, she was restarted on multidrug treatment for paucibacillary leprosy. Cardiologists opinion was taken which showed normal echocardiography and pulmonologists suggested chest physiotherapy.
She was admitted at 41 weeks of gestation for induction of labor. On examination general condition was fair, afebrile, pulse was 80 per minute and blood pressure recorded was 120/80 millimeter of mercury. Cardiovascular and respiratory examination was within normal limits. Abdominal examination showed full term relaxed gravid uterus in vertex presentation with regular fetal heart sounds. Cervical internal os was closed and uneffaced. Preinduction cervical ripening was done with Foley’s catheter, following which labor was augmented.
She developed fetal distress and an emergency LSCS was done during first stage of induced labor. She delivered a healthy male child of 3.2 kilogram birth weight with Apgar score of 9/10. Post-delivery breast feeding was prohibited as per advice given by neonatologist.

Discussion

Hansen's disease (HD) or Leprosy is a chronic granulomatous infectious disease caused by Mycobacterium leprae affecting the skin, mucous membranes and peripheral nerves. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of it are extensive which can mimic symptoms of many rheumatic diseases. It is rarely reported during pregnancy but can be exacerbated during pregnancy. It is important to treat leprosy during pregnancy as without treatment it can permanently damage the skin, nerves and eyes.[1]
Systemic sclerosis is a multi-systemic autoimmune disorder affecting predominantly the skin, lungs, gut and kidneys. Pregnancy in women diagnosed with systemic sclerosis is not a common condition but it has a favorable outcome according to the most recent studies.[2]
Our patient initially presented with gradual sclerotic skin change that is thickening of skin over forearms and sclerodactyly diagnosed on radiological imaging which is frequently observed in systemic sclerosis. Diagnosis was confirmed on doing immunological blood test. Later on a skin biopsy with acid-fast stain was done for a non-resolving skin lesion over face which confirmed lepromatous leprosy.
Systemic sclerosis in pregnancy can have complications like dyspnea, hypertension, gastroesophageal reflux disease and renal complications. The most common maternal complication is worsening of gastroesophageal reflux, and the most severe complications are renal crisis and flaring of arterial pulmonary hypertension.[3] But in our patient, none of the above has occurred during antenatal period. Obstetric complications include placental insufficiency, and these patients may benefit with aspirin, low dose anticoagulants or even nitric oxide donors. Our patient was started on aspirin and tablet nifedipine was continued.
The most important and interesting part of our case was the co-existence of systemic sclerosis and lepromatous leprosy.
Immunodeficient state is a risk factor for leprosy in pregnancy.[4] Our patient was on steroids and autoimmune medications, which is in an immunodeficient state.
Lepromatous leprosy, in general, has been reported to be a condition which is commonly misdiagnosed.  Systemic sclerosis is a condition with which it is commonly confused.[5,6] Salvi et al have reported a case series of leprosy cases occurring in a rheumatological settings, and had termed it at a challenging mimic, which is difficult to expose.[7]
Despite a thorough literature search, we have not been able to find any case report of a parturient suffering from a combination of systemic sclerosis and leprosy. We believe this may be the first such case report, and is hence reported here.
The patient in our case had an otherwise uneventful postpartum period. Breastfeeding is not allowed for those on anti-mycobacterial medications like dapsone.[4]
Though the neonate in our case had no problems, rare case reports exists which have recorded neonatal methemoglobinemia due to transplacental transfer of dapsone.[8]

Conclusion

The occurrence of lepromatous leprosy in pregnancy is rare. The occurrence of this in combination with systemic sclerosis is even rare. One such case is presented here.

References

1. Ozturk Z, Tatliparmak A. Leprosy treatment during pregnancy and breastfeeding: A case report and brief review of literature. Dermatologic Therapy. 2017; 30(1): e12414.
2. Josselin-Mahr L, Carbonne B, Cabane J. Systemic sclerosis and pregnancy. Internal Medicine Journal 2011; 32(6):363–8.
3. Grygiel-Górniak B, Puszczewicz M. [The complications of systemic sclerosis in pregnancy - diagnostic and therapeutic difficulties]. Polish medical Journal. 2016;40(235):61-5.
4. Gimovsky AC, Macri CJ. Leprosy in pregnant woman, United States. Emerg Infect Dis. 2013; 19(10):1693–4.
5. Yaghoobi R, Feily A, Ranjbari N, Lotfi J, Yaghoobi E. Lepromatous Leprosy: A Commonly Misdiagnosed Disease. Sci World J [Internet]. 2010;10:2348–9.
6. Lee JY, Park SE, Shin SJ, Kim CW, Kim SS. Case of lepromatous leprosy misdiagnosed as systemic sclerosis. J Dermatol. 2014; 41(4):343–5.
7. Salvi S, Chopra A. Leprosy in a rheumatology setting: a challenging mimic to expose. Clin Rheumatol. 2013; 32(10):1557–63.
8. Kabra NS, Nanavati RN, Srinivasan G. Neonatal methemoglobinemia due to transplacental transfer of dapsone. Indian Pediat. 1998; 35(6):553–5.

Citation

Nasare P, Prasad M, Gupta AS. A Case Of Pregnancy With Coexisting Systemic Sclerosis And Paucibacillary Leprosy. JPGO 2018. Volume 5 No.6. Available from: http://www.jpgo.org/2018/06/a-case-of-pregnancy-with-coexisting.html

Chronic Nonpuerperal Inversion Of Uterus Secondary To Submucous Myoma

Author Information

Jain N*, Chaudhari H**, More V***.
(* Second Year Resident, ** Associate professor, *** Assistant Professor. Department of Obstetrics and Gynecology, Seth G.S. Medical college and KEM hospital , Mumbai, India.)

Abstract

Uterine inversion is defined as descent of the uterine fundus to or through the cervix. Chronic, non puerperal, postmenopausal uterine inversion is an extremely rare entity. Here we are presenting a case of a 60 year old, postmenopausal woman who presented as a case of prolapse but who had chronic non puerperal uterine inversion secondary to multiple uterine submucosal pedunculated leiomyoma, She was managed by vaginal myomectomy followed by correction of inversion of the uterus by Kustner’s  method followed by abdominal hysterectomy.

Introduction

Uterine inversion is defined as descent of uterine fundus to or through the cervix. Uterine inversion can present as an acute or puerperal and chronic or non puerperal type. Chronic inversion is rare as compared to acute inversion.  Incidence of  chronic non puerperal inversions are one sixth of all inversion cases.[1,2] Chronic non puerperal postmenopausal uterine inversion  is extremely rare condition. It is reported that 97.4 % chronic non puerperal uterine inversions were associated with tumors out of which only 20% were malignant.[3] Most common predisposing factor is fundal submucous leiomyoma as seen in our case. Others are endometrial polyp, and uterine sarcoma.[4] We present a rare case of chronic uterine condition in non puerperal stage managed surgically.

Case Report

A 60 years old, postmenopausal woman, para 4 living 1, all full term normal vaginal  home deliveries, came with complaints of something coming out from vagina since 1 month which was non reducible. She had foul smelling vaginal discharge since 2 months.  She was a known case of hypertension since 2 years; well controlled on medication.  Her general condition was fair and vital parameters were stable. Abdomen was soft. On local examination there was a large, elongated, congested, firm mass of approximate 15 x 10 cm present, mass was irreducible, 2 areas of ulcerations of size 3x3 cm covered with foul smelling slough were seen. Two submucosal fibroids were present of size approximate 4x4 cm on right lateral wall near right cornua and 3x3 cm on left lateral wall behind left cornua. Cervical ring was seen and felt. Clinical impression was chronic non puerperal infected uterine inversion associated with multiple fibroid. Other differential diagnosis was uterine procidentia.

Figure 1. The prolapsed mass with fibroid. Leiomyoma is pointed out by arrow.

She was hospitalized. Pap smear was severe atrophic and inflammatory. Ultrasonography  (USG) of the pelvis showed that the uterus was not seen in the pelvis. MRI was suggestive of complete uterine inversion grade four with 3x4.5x4.5 cm pedunculated posterior wall submucous fibroid, 2x2 cm intramural fibroid over fundus and a 4.8x3.4x3.5 cm anterior wall submucosal fibroid with degeneration and ulceration.

Figure 2. MRI scan showing inverted fundus.  Yellow arrow marks inverted fundal cup, Green arrow shows the adnexa, Pink arrow depicts the inverted endometrium ‘F’ is the fibroid.

All blood investigations except blood sugar were within normal limit. She was diagnosed with diabetes mellitus after hospitalization and started on oral hypogycemic drugs. Daily twice dressing of inverted uterine mass was done using povidine iodine, and the prolapsed mass was covered with glycerine and acroflavin dressing. She had an episode of transient stroke in the ward. Neurologist advised a CT scan of the brain but no abnormality was detected.  She was started on anticoagulants. 2D Echo done was within normal limit with ejection fraction of 60%. She also had urinary tract infection which was treated according to culture sensitivity report.  Corrective Surgery was postponed as she was unfit. She was planned for surgery after getting fitness from anesthetist, endocrinologist and neurologist.  Procedure performed was vaginal myomectomy followed by correction of inversion of uterus by Kustner’s method followed by abdominal hysterectomy. Surgery was performed under general anesthesia. Posterior colpotomy was done and diagnosis of inversion was confirmed.

Figure 3. Kustner's operation. Posterior pouch opened.

Submucosal fibroid was enucleated. Reposition of the uterine inversion was done by Kustner’s method. Cervical ring was divided vertically in the midline posteriorly. Edges of cervix were held with Alli’sforceps. There was still difficulty in reposition hence the incision was extended upto the isthmus and posterior uterine wall. Following which uterine inversion was corrected, which was confirmed on per speculum examination. Abdominal hysterectomy was done by standard method. Histopathology report showed a leiomyoma. Uterus and cervix had no evidence of malignancy.

Figure 4. Kustner's operation. Incision of the posterior cervical ring and identification of the edges of the cervix.

Figure 5. Kustner's operation. Incision extended to the posterior wall of the uterus

On day 3 of surgery She developed signs of sepsis. She was shifted to intensive care unit. Higher antibiotics were started. Coagulation profile was deranged and correction with fresh frozen plasma was given as per hematologist’s advice. Her condition deteriorated and she succumbed on post operative day 16  .

Discussion

Uterine inversion is a condition where the uterus turns inside out, the fundus prolapsing through the cervix. It may be classified as puerperal and non puerperal. Chronic non puerperal inversion is very rare. They represent 1/6th of all cases of uterine inversion.[1,2] Causes of non puerperal chronic inversion are incomplete obstetric inversions unnoticed or uncared for following failure to reduce for a period of 4 weeks or more, submucosal fundal myomatous polyp can cause chronic inversion due to traction effect, sarcomatous changes of fundal fibroma and senile inversion. Most common cause of non puerperal chronic inversion is a uterine leiomyoma in approximately 78 to 85% of the cases,as in our case.[5]  Two types of chronic inversion have been described. Incomplete inversion in which fundus protrude through the cervix but lies inside the vagina. Second is complete type in which whole of the uterus including the cervix are inverted, vagina may also be involved in the process. Our patient had complete inversion in which vagina was involved. Uterine inversion can be classified into four stages. Stage 1 is when the inverted fundus remains in the uterine cavity. In stage 2 complete inversion of the fundus occurs through the cervix. In stage 3 the inverted fundus protrudes through the vulva and in stage 4 inversion the uterus and vaginal wall protrude out of the vulva.

They present with symptoms of something coming out from vagina, irregular vaginal bleeding and offensive vaginal discharge.[6, 7] Chronic inversion is some times confused  with fibroid polyp. Our patient presented with something coming out from vagina, with foul smelling discharge and multiple ulceration. USG is first diagnostic modality of choice. MRI and CT scan are also useful diagnostic tools.[8, 9]
Rectification surgery is definitive line of treatment. Unlike acute inversion which requires immediate correction, chronic inversion is to be treated electively.  Preservation and/ or removal of the uterus along with rectification is determined by factors like age, parity, and associated complicating factors. Since our patient was postmenopausal, rectification surgery followed by hysterectomy was done. Many operative procedures for the treatment of chronic inversion are described amongst these the most common abdominal procedures are Huntington’s and Haultain’s  and  vaginal procedures are mainly Spinelli’s and Kustner’s operations.[10,11,12] Spinelli’s and kustner’s operation are both  performed vaginally, are similar with the exception that in Spinelli’s surgery the approach to divide the cervical ring is anterior whereas in Kustner’s procedure the  posterior approach is used and the incision is placed on the posterior cervical ring.[13] Kustner’s approach was suitable here as the risk of damaging the bowel was small, because of the deep reflection of the pouch of Douglas whereas anterior approach included  danger of damaging the bladder during separation, risk being more due to complete inversion. In Haultain’s  procedure, done transabdominally the constricting ring is incised posteriorly by a vertical incision and then the inversion is corrected. In Dobbin’s procedure, the cervical ring is divided in midline anteriorly by an abdominal approach and then the inversion is corrected.

Conclusion

Once a diagnosis is established the surgical correction can be done by choosing the best option from the different available ones as described in literature.

Acknowledgment

We thank Dr S V Parulekar for his intraoperative guidance and Dr. Lalita Kambagatti for the intraoperative images.

References

1. Katdare P, Valecha SM, GandhewarM, Dhingra D. Chronic Non-Puerperal Uterine Inversion: Recommendations for Diagnosis and Management. Global Journal of Medical Research Gynecology and Obstetrics. 2013; 13(2):45-47.
2. Takano K, Ichikawa Y, Tsunoda H, Nishida M. Uterine inversion caused by uterine sarcoma: a case report. Jpn J Clin Oncol. 2001;31(1):39-42.
3. Mwinyoglee J, Simelela N, Marivate M. Non-puerperal uterine inversions: a two case report and review of literature. Central African J Med. 1997;43(9):268-71
4. Kagne SS, Thawal YA, Tambe SG. An extremely rare case of chronic non-puerperal uterine inversion treated by myomectomy preceding vaginal hysterectomy. Journal of evolution of medical and dental sciences. 2013;46: 8976-8979.
5. Lupovitch A, England ER, Chen R. Non-puerperal uterine inversion in association with uterine sarcoma: case report in a 26-year-old and review of the literature. Gynecol Oncol. 2005;97(3):938-41.
6. Krenning RA, Dörr PJ, de Groot WH, de Goey WB. Non-puerperal uterine inversion. Case report. Br J Obstet Gynaecol. 1982;89(3):247-9.
7. Gowri V. Uterine inversion and corpus malignancies: a historical review. Obstet Gynecol Surv. 2000;55(11):703-7.
8. Salomon CG, Patel SK. Computed tomography of chronic nonpuerperal uterine inversion. Journal of Computer Assisted Tomography.1990;14(6):1024-1026.
9. Lewin JS, Bryan PJ. MR imaging of uterine inversion. J Comput Assist Tomogr. 1989; 13: 357-359.
10. Lascarides E, Cohen M. Surgical management of nonpuerperal inversion of uterus. Obstet Gynaecol. 1968; 32:376-381.
11. Haultain FWN. The treatment of chronic uterine inversion by abdominal hysterectomy, with a successful case. Br Med J. 1901; 2(2127): 974-976.
12. Huntington JL, Irving FC, Kellogg FS. Abdominal reposition in acute inversion of the puerperal uterus. Am J Obstet Gynecol. 1928; 15(1): 34-40.
13. Bakre T, Gupta AS, Hira Priya, Parulekar SV. Chronic Non Pueperal Inversion Of Uterus Secondary To Submucosal Fundal Myoma. Available from http://www.jpgo.org/2014/10/chronic-non-pueperal-inversion-of.html

Citation

Jain N, Chaudhari H, More V. Chronic Nonpuerperal Inversion Of Uterus Secondary To Submucous Myoma. JPGO 2018. Volume 5 No.6. Available from:http://www.jpgo.org/2018/06/chronic-nonpuerperal-inversion-of.html

Giant Sacrococcygeal Teratoma

Author Information

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

Abstract

One of the most common tumours in the newborns is sacrococcygeal teratoma. We discuss the management and prognosis of antenatally diagnosed sacrococcygeal teratoma.

Introduction

Sacrococcygeal teratoma is a tumour that arises from all the three germ layers. It accounts for 44% of all germ cell tumours. The incidence is somewhere in between 1 per 20,000 to 1 per 40,000 live births.[1] Females are more frequently affected .[2]

Case Report

A 24 year old G3P2L2 with previous two vaginal deliveries presented to the outpatient department of our tertiary care centre at 28 weeks of gestation. A prior ultrasonography, done at 23 weeks of gestation showed a 8.3 x 6.1 cm solid cystic mass, with vascularity was noted at the caudal end of spine. Antenatally, pediatric surgeons opined that the patient should deliver in a tertiary care centre, and the neonate will need to be evaluated immediately post-delivery. This progressed to around 12.9 x 11.2 cm sized with volume of around 1096cc with polyhydramnios at 28 weeks. A week later, the dimensions were noted to be 14.5 x13.2 cm with volume of 1502cc, suggestive of rapid progression. 

 At 32 weeks of gestation she presented to the emergency in established active labour. In view of the large mass, emergency lower segment cesarean section was performed. She delivered a female child of 3.5 kg with Apgar score of 3/10 and 9/10. The external features of the teratoma in the neonate (Figure 1) was consistent with the radiological examination. The neonate was shifted to neonatal intensive care unit on controlled positive pressure ventilation as the baby was not able to maintain saturation. Pediatric surgeons advised MRI after stabilization. However, the neonates general condition continued to deteriorate, requiring further ventilatory support and soon needed inotropic support also. Due to ongoing bleeding and oozing of fluid from the teratoma, a picture of hypovolemic shock persisted and one unit packed cell transfusion was given. Further hemorrhage continued and the neonate succumbed on the fourth day of life due to hemorrhagic shock.

Figure 1. Sacrococcygeal teratoma.

Discussion

One of the most common tumors in newborns is sacrococcygeal teratoma. Tumor arises from Hensen node, located in the coccyx. Two dimensional ultrasound is used to make the diagnosis of sacrococcygeal teratoma antenatally. In the first trimester, diagnosis of these tumors may be missed owing to its small size. However, large, rapidly growing tumors with solid and vascular components, may be diagnosed earlier. Also, those diagnosed before 30 weeks of gestation, are associated with poor prognosis. In our case tumor was increasing rapidly in size, had solid and vascular components and was diagnosed at 23 weeks of gestation. Over few weeks, the tumour grew almost double in size.  Ultrasound doppler helps to know the amount of blood flow to the tumor.

In rapidly growing tumours, large amount of blood is directed towards the tumour, leading to heart failure and sick and hydropic foetus. Hence, a fetal echocardiogram is recommended.  Our patient was planned for a fetal echocardiogram but went into preterm labour. For detailed evaluation, fetal MRI is an option.
Small, relatively avascular cystic tumour are usually treated by aspiration. Various procedures can be done, the options being vascular procedures like embolisation of the tumour, balloon occlusion, thermocoagulation, sclerosis, endoscopic snaring of the tumor neck, laser/radio-frequency ablation. Radiofrequency ablation may result in large soft-tissue defects that require postnatal surgery. Debulking of the tumor can be done in some cases in the midgestation period, but experience is limited. An EXIT procedure (Ex-Utero Intrapartum Treatment Procedure) for resection of a massive sacrococcygeal teratoma can also been performed.
The morbidity and mortality of the sacrococcygeal teratomas can be reduced if they are diagnosed prenatally and the surgical management is performed during the neonatal period.[3] Other possible complications include hemorrhage within the tumour, polyhydramnios, pre-term labour and intrauterine fetal demise.
In our patient, there was polyhydramnios, ongoing hemorrhage inside the mass and preterm labour. In severe cases, sacrococcygeal teratomas with hydrops are associated with maternal risk for development of mirror syndrome, which is a severe form of preeclampsia and is associated with placentomegaly. This was not seen in our case.

A planned cesarean delivery is necessary for large tumors greater than 5 cm, with provisions for immediate resection if necessary. Delivery of the fetus through a cesarean section prevents dystocia, tumor rupture and profuse tumor bleeding.[4] An upper segment vertical uterine incision may be required.[5]  However, in our patient a lower segment cesarean scar was adequate for delivery. There were no intraoperative problems. When the diameter of the neoplasm exceeds 10 cm, the risk of rupture of sacrococcygeal teratoma is increased during the ablative tumour resection. In our case, size of the tumour was 14.5 X 13.2 cm with volume of 1502cc. Though there was no external rupture, the ongoing internal hemorrhage probably led to hemorrhagic shock, and eventually mortality.

Conclusion

Sacrococcygeal teratoma is associated with high perinatal morbidity and mortality. Despite early recognition, fetal salvage may not be possible when the tumour size is large. The best chance for the fetus lies when the delivery is an tertiary care hospital with close coordination between obstetricians, neonatologists and pediatric surgeons.

References

1. Winderl LM, Silverman RK. Prenatal identification of a completely cystic internal sacrococcygeal teratoma (type IV). Ultrasound Obstet Gynecol. 1997 9(6):425-8.
2. Gucciardo L, Uyttebroek A, De Wever I, Renard M, Claus F, Devlieger R, et al. Prenatal assessment and management of sacrococcygeal teratoma. Prenat Diagn. 2011  31(7):678-88.
3. Saygili-Yilmaz ES, Incki KK, Turgut M, Kelekci S. Prenatal diagnosis of type I sacrococcygeal teratoma and its management. Clin Exp Obstet Gynecol 2008; 35(2):153-5.
4. Lahdes-Vasama TT, Korhonen PH, Seppänen JM, Tammela OK, Iber T. Preoperative embolization of giant sacrococcygeal teratoma in a premature newborn. J Pediatr Surg 2011; 46(1): e5-8.
5. Aktepe Keskin E, Arikan Onaran Y, Derbent A, Ayrim A, Kafali H. Prenatal diagnosis and follow-up of giant sacrococcygeal teratoma. Taiwan J Obstet Gynecol 2011; 50(2):242-4.

Citation

Parihar A, Warke HS. Giant Sacrococcygeal Teratoma. JPGO 2018. Volume 5 No.6. Available from:http://www.jpgo.org/2018/06/giant-sacrococcygeal-teratoma.html

Cesarean Myomectomy

Author Information

Malani K*, Jagtap S**, Swaminathan G**, Samant PY***.

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

Abstract

Cesarean myomectomy has been conventionally discouraged in view of possibility of uncontrollable hemorrhage and maternal morbidity. However, it is recently becoming popular with some researchers concluding that it is safe. We present a case of an unavoidable caesarean myomectomy with uneventful post-operative course.

Introduction

From the time of Victor Bonney, cesarean myomectomy was strongly discouraged for the torrential blood loss it may cause. But in the last 20 years, numerous case reports and case control studies have supported cesarean myomectomy as safe and even cost effective. Nevertheless, some authors suspect that complications are under reported. A case of cesarean myomectomy is presented here. 

Case Report

Twenty nine year old Gravida 3 Para 1 Abortion 1 Living 1 with a previous cesarean section was diagnosed with an uterine intramural fundal fibroid of 5.7 x 4.5 x 5 cm in the first trimester. The diagnosis was reconfirmed in second trimester with no significant change in size. She had an uneventful antenatal period. Most of this time, she was following up in a primary centre. She had been diagnosed of hypothyroidism since 2 years and was on thyroid supplements. She was detected with gestational diabetes mellitus in the current pregnancy and was controlled on medical nutrition therapy. She was compliant with medications and diet. In view of cephalopelvic disproportion, she was advised elective lower segment caesarean section at 38 weeks, but did not get admitted. She came to emergency room in early labor and was prepared for immediate cesarean section.  Her hemoglobin was normal and blood was cross matched and kept ready.
Intraoperatively, fibroid was not palpable. Baby’s head was deflexed. After delivery of the baby and placenta, an 8 x 8 x 4 cm intramural fibroid was seen protruding from the upper edge of the uterine incision (figure 1). This location of fibroid was least expected since antenatal ultrasonographic scans were suggestive of fundal fibroid. After careful examination of the fibroid size and position, decision of caesarean myomectomy was made, which if not done would make closure of uterine incision impossible. Required consent was sought and documented. Blunt and sharp dissection was done to free the myoma of its pseudocapsule. (Figure 2) Myoma specimen was of salmon pink colour and non hemorrhagic and sent for histopathological examination. Multiple figure of eight interrupted sutures were taken with no. 1 delayed absorbable sutures in the myoma bed and hemostasis was achieved. After this, it was possible for closure for the uterine incision with no difficulty. (Figure 3) Uterine incision closure was performed in single layer with continuous interlocking sutures. Injection tranexamic acid 1 gram intravenous infusion was started intraoperatively and oxytocin infusion was continued for 6 hours post-delivery. Post-operative recovery was uneventful. In view of estimated blood loss of around 1200 ml, she was transfused one unit of packed red cells post operatively.  Hemoglobin done on third post-operative day was 8.6 gm %, and was discharged on oral iron. There were no wound complications.

Figure 1. Arrow pointing to intramural fibroid in lower uterine segment, just above uterine incision.

Figure 2. Arrow pointing to fibroid being removed.

Figure 3. Uterine incision after removal of fibroid, with arrows pointing to the upper and lower parts of the incisions.

Discussion

Uterine fibroids are seen in 0.3 to 3 % pregnancies.[1] They are associated with  complications like intra uterine growth restriction, fetal malpresentations, premature rupture of membranes, preterm delivery, obstructed labor, retained placenta, severe pain in case of red degeneration and in rare cases; placental abruption due to location under the placental site.[2] Antepartum management is essentially conservative, but for extreme reasons, antepartum myomectomy has been reported.[3] Obvious reasons of avoiding excision of fibroid at time of cesarean section are high chances of torrential hemorrhage, atonic uterus and resultant hysterectomy. But sometimes, cesarean myomectomy becomes unavoidable like in case of fibroids in the lower uterine segment posing difficulty in baby delivery or uterine closure. In our case, there was difficulty in closure of uterine incision, which necessitated myomectomy. A study comparing uterine artery embolization followed by cesarean myomectomy with only cesarean section showed that short and long term results were comparable.[4] The average blood loss in one study ranged between 200 to 1000 ml with an average of around 500 ml.[1] Based on a large retrospective case control study with more than 1200 patients, it was seen that myomectomy during cesarean section is a safe procedure from point of view of blood loss, post-operative recovery and fever.[5] In our case also, there was no need for massive blood transfusion, there was no febrile morbidity and postoperative recovery was otherwise uneventful. According to Akkurt et al long term outcomes for subsequent pregnancy in cesarean myomectomy are favorable.[6]
In this case, the earliest ultrasonography reported the fibroid to be fundal and intramural. Later done obstetric scans did not comment on any change on either the size or the location of the fibroid. In case the anterior low lying location was known prior, it may have helped in some preparation regarding the uterine incision in order to avoid a myomectomy.  The decision of enucleation is made on the basis of tumor size, location, proximity to vascular areas and severity of symptoms. Large fundal intramural fibroids, intramural fibroids located near cornual region, isthmus region and seedling fibroids and fibroids in cornual region are best left untouched.[7] Our patient had an obvious, easily accessible fibroid, which made the performance of myomectomy relatively easy.

Although ultrasound is a gold standard, dynamic MRI can give vital information about vascular supply or myometrial involvement in patients with fibroid related post-partum bleeding.[8] Rarely complications like pyomyoma may create diagnostic dilemma in postpartum period.[9] They can manifest postpartum and rarely, even months later.[10] None of these complications were noted in our patient.  As data on long term outcomes in women undergoing cesarean myomectomy are lacking, the procedure should be undertaken very cautiously.[11]

Conclusion

Cesarean myomectomy is no more a rare procedure, but there is no unanimity of opinion on its safety. Prior to proceeding with cesarean section for any indication, it is advantageous to know the location of the fibroid. This case is presented to highlight that even if there was no preoperative plan, there may be an intraoperative need for a decision of cesarean myomectomy.

References

1. Awoleke JO. Myomectomy during Caesarean Birth in Fibroid-Endemic, Low-Resource Settings. Obstet Gynecol Int. 2013 Nov; 2013:520834.
2. Lee H, Norwitz ER, Shaw J. Contemporary Management of Fibroids in Pregnancy. Rev Obstet Gynecol. 2010 Winter; 3(1): 20–27.
3. Bhatla N, Dash BB, Kriplani A, Agarwal N. Myomectomy during pregnancy: A feasible option. J Obstet Gynaecol Res 2009; 35(1):173–5.
4. Lin JY, Lee WL, Wang PH, Lai MJ, Chang WH, Liu WM. Uterine artery occlusion and myomectomy for treatment of pregnant women with uterine leiomyomas who are undergoing cesarean section. J Obstet Gynaecol Res 2010; 36(2):284-90.
5. Myomectomy during cesarean section (query bank). RCOG. Available from: https://www.rcog.org.uk/en/guidelines-research-services/guidelines/myomectomy-during-caesarean-section---query-bank/
6. Akkurt MO, Yavuz A, Eris Yalcin S, Akkurt I, Turan OT, Yalcin Y, et al. Can we consider cesarean myomectomy as a safe procedure without long-term outcome? J Matern Neonatal Med. 2017 Aug ; 30(15):1855–60.
7. Kamal P, Bala R, Nagpal M, Kaur H. Cesarean myomectomy: Practical issues. Indian Journal of Obstetrics and Gynecology Research 2017;4(3):249-253
8. Torrance S, Muhn N, Ellis S, Rebello R, Ramanna R. Role of dynamic MRI in surgical decision-making for a postpartum woman with a prolapsed degenerating uterine leiomyoma. J Obstet Gynaecol Can. 2009 May; 31(5):446-51.
9. Bhave N, Shah P, Chaudhari H. An unusual presentation of Degenerating Fibroid.Int J Reprod Contracept Obstet Gynecol 2016; 5 :582- 4
10. Sirha R, Miskin A, Abdelmagied A. Postnatal pyomyoma: a diagnostic dilemma. BMJ Case Rep [Internet]; 2013: bcr2013201137.
11. Sparić R, Kadija S, Stefanović A, Spremović Radjenović S,2, Likić Ladjević I, Popović  Cesarean myomectomy in modern obstetrics: More light and fewer shadows. J Obstet Gynaecol Res. 2017 May; 43(5):798-804.

Citation

Malani K, Jagtap S, Swaminathan G, Samant PY. Cesarean Myomectomy. JPGO 2018. Volume 5 No.6. Available from:http://www.jpgo.org/2018/06/cesarean-myomectomy.html

Scar Endometriosis

Author Information

Nebhani M*, Shah R**, Warke HS***.
(* Junior Resident, ** Assistant Professor, *** Associate Professor, Department of Gynecology and Obstetrics, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Endometriosis is a benign disease characterized by the presence of endometrial glands at sites other than the uterus. Scar endometriosis is a rare site for development of endometriosis. The differential diagnoses include a few skin or surgical conditions which may cause a delay in the management.  A woman with previous one lower segment cesarean section done four years prior, and diagnosed with scar endometriosis is presented here.

Introduction

The overall prevalence of endometriosis. Endometriosis is a disease of reproductive age and the overall estimated prevalence is 3-10%. The mean age of patients is 25 to 35 years. Endometriosis usually presents in the pelvic sites. Extrapelvic endometriosis can rarely be seen in sites such as the nervous system, thorax, gastrointestinal tract and rarely in the skin scars such as episiotomy scars.[1] One of the rare extra-pelvic site for endometriosis i.e. surgical scar is called scar endometriosis. The incidence of scar endometriosis after cesarean section is 0.03-0.45 %.[2] A case of scar endometriosis is described here.

Case Report

A 24 year old woman presented with a painful nodular swelling over the right side of the abdominal scar since 1 year with progressive increase in pain and size of swelling especially during menstruation. She also complained of serous discharge from it during menstruation. She had undergone a lower segment caesarean section four years prior. General examination was within normal limits. On local examination there was a approximately 2 x 2 cm tender, immobile nodular subcutaneous swelling in the right iliac fossa, 2 cm lateral and 3 cm above the scar. A provisional diagnosis of scar endometriosis was made. Ultrasound showed a 2.1x1.6 cm well defined hypoechoic lesion in the paramedian region in the subcutaneous plane with peripheral vascularity on colour Doppler suggestive of scar endometriosis.(Figure 1) MRI abdomen pelvis done suggested an oval shaped T1 hypointense and T2 heterogenous hyperintense lesion seen at the anterior abdominal wall on the right side measuring approximately 2.2 x 2 cm suggestive of scar endometriosis, and was posted for operative procedure in the post-menstrual period.

Figure 1. Ultrasound image of scar endometriosis showing vascularity on Color Doppler
Intraoperatively a mass of 3 x 2 cm, bluish in colour and hard in consistency was identified. The mass was situated below and adherent to the rectus sheath with rectus muscle below. The nodular mass was dissected from the rectus sheath and muscle to ensure that the entire mass was widely excised with surrounding clear margins and sent for histopathological examination. (figures 2 and 3) The layers of abdominal wall were reconstructed and sutured.

Histopathological examination revealed dense fibrocollagenous tissue with embedded endometrial glands surrounded by endometrial stroma suggestive of scar endometriosis. Patient was followed up after surgery for a period of 3 months. She did not have any complaints and was completely cured after the surgical excision.

Figure 2. Intraoperative picture- Endometriotic nodule adherent to rectus sheath and muscle.

Figure 3. Specimen of endometriotic nodule after excision.

Discussion

Rokitansky gave the first description of endometriosis in 1860 with the most common site as pelvis. Asymptomatic endometriosis is seen in 1-7% patients of tubal ligation, 12-32% in women presenting with pelvic pain between age of 15-45 years, 9-50% in infertility and as much as 50% in adolescents with chronic pelvic pain or dysmenorrhea.[l] Scar endometriosis is a rare type of endometriosis occurring in extrapelvic sites that usually happens after surgery on the uterus and tubes. In our case, it was a cesarean section.
Scar endometriosis can mimck other common surgical and gynecological conditions. The diagnosis achieved at a preoperative stage is correct in only 20-50% patients.[3] The differential diagnoses include stitch granuloma, lipoma,  inguinal hernia, cyst, incisional hernia, abscess, sarcoma, desmoid tumor, lymphoma and primary or metastatic cancer.[4] Therefore, the clinician needs to rule out all of the above conditions before making the final diagnosis, for which a biopsy is imperative.

One theory states that endometrial tissue may also approach the surgical scar by vascular and lymphatic channels causing this disease. Hence, one must ensure secure closure of the parietal and preferably also visceral peritoneum during caesarean section and reduce dissemination of endometrial cells as it may also be associated with endometriosis at the surgical scar.[5] The complaint of cyclic pain in an incisional nodule or lump usually at caesarean scar is almost classical for scar endometriosis. This was seen in our patient also. Review of literature suggests that efforts should be made to narrow down the diagnosis with the help of imaging techniques and FNAC. In our patient, radiological examination was adequate. Sonography with color Doppler shows characteristic appearance of scar endometriotic lesions which is a hypoechoic, vascular, and solid mass with some cystic changes.[6] Computerized Tomography would show a well-defined soft tissue nodule with heterogenous post contrast enhancement and surrounding tissue showing streakiness. However, MRI is the most sensitive imaging modality and has ability to localize the lesion accurately in relation to the caesarean scar with the signal characteristics similar to background endometriosis. Various methods of hormonal therapy such as oral contraceptives, progesterones, gonadotropin-releasing hormone analogues, danazol have been used but they give only temporary relief and have a higher side effect profile. Thus, the treatment of choice is a wide surgical excision to healthy margins. Similar cases have been reported recently.[7,8] Sometimes, mesh placement may be required. Residual endometrial tissue may cause recurrences. Histopathology shows glands and stromal cells of endometriosis with the background of altered blood and foamy macrophages which is confirmatory for the diagnosis.
Histopathological evaluation is also important to make sure there is no malignant transformation. Although malignant change of scar endometriosis is rare, malignant changes may occur in long-standing recurrent scar endometriosis. Clear-cell carcinoma and endometrioid carcinoma are the common types.[9]
As mentioned above, certain surgical techniques such as closure of the uterovesical fold, parietal peritoneum, saline peritoneal washes prior to closure and using separate instruments and suture materials for closure of uterus and the abdominal wall, are options for prevention of future scar endometriosis. The usefulness of such practices is yet to be proved by studies and further research is needed in this matter which is difficult owing to the rarity of scar endometriosis cases.

Conclusion

Endometriosis involving scars is a rare condition that should be considered in reproductive age women with the classical clinical presentation of cyclical painful nodule at the scar site having previous obstetrical surgery. The amount of pain and size of mass may vary with the menstrual cycle. Surgical wide excision after diagnosing clinically and by radiological investigations is the treatment of choice and histopathological evaluation is the gold standard of diagnosis.

References

1. Jubanyik KJ and Comite F. Extrapelvic endometriosis. Obstetrics and Gynecology Clinics of North America.1997; 24(2): 411–440. 
2. Wolf Y, Haddad R, Werbin N, Skornick Y, and Kaplan O. Endometriosis in abdominal scars: a diagnostic pitfall. American Surgeon.1996; 62(12) : 1042–1044.
3. Horton JD, Dezee KJ, Ahnfeldt EP, Wagner M. Abdominal wall endometriosis: a surgeon’s perspective and review of 445 cases. Am J Surg. 2008; 196: 207–12.
4. Blanco RG, Parithivel VS, Shah AK, Gumbs MA, Schein M. Gerst PH. Abdominal wall endometriomas. The American Journal of Surgery.2003; 185(6): 596–598.
5. Steck WD and Helwig EB. Cutaneous endometriojsis. Clinical Obstetrics and Gynecology.1966; 9(2): 373–383.
6. Kinkel K, Frei KA, Balleyguier C, Chapron C.Diagnosis of endometriosis with imaging: a review. European Radiology. 2006; 16(2): 285–298.
7. Madhva Prasad S, Puri J, Gupta AS. Surgical scar endometriosis. JPGO 2015. Volume 2 Number 11. Available from: http://www.jpgo.org/2015/11/surgical-scar-endometriosis.html
8. Shaikh A, Mayadeo MN, Warke HS. Scar Endometriosis: a Rare Case. JPGO 2017. Volume 4 No.8. Available from: http://www.jpgo.org/2017/08/scar-endometriosis-rare-case.html
9. Taburiaux L, Pluchino N, Petignat P, Wenger JM.  Endometriosis-Associated Abdominal Wall Cancer: A Poor Prognosis? Int J Gynecol Cancer. 2015 ; 25(9):1633-8.

Citation

Nebhani M, Shah R, Warke HS. Scar Endometriosis. JPGO 2018. Volume 5 No.6. Available from:http://www.jpgo.org/2018/06/scar-endometriosis.html

Remembering Past Greats: James Marion Sims

Author Information

Pai K.

(Asst Hon.,Department of OBGYN, Pandit Madan Mohan Malviya Shatabdi Municipal General Hospital, Mumbai, India.)

Dr. J. M. Sims ((1813-1883) is widely considered as ‘Father of modern gynecology’. The first instrument taught to undergraduates is the Sims speculum and ‘Enumerate the contributions of Sims to the field of OBGYN’ is one of the favorite questions amongst undergraduate and postgraduate examiners. The recent news of the statue of James Marion Sims being moved from a prominent location at Central Park, opposite the New York Academy of Medicine to the location of Sims’ grave in Brooklyn prompted me to write this article to review whether he rightly fits the space under ‘Past Greats’. 

The New York Public Design commission considered Dr. Sims to be a ‘symbol of hate’. Dr. Sims performed medical experiments on enslaved black women using no anesthesia. He not only invented the Sims speculum but also devised an operation for vesico-vaginal fistula. The stories of his experiments done at an 8-bedded hospital in the slave-trading district of Montgomery, Alabama placed among white plantation settlements, whose owners treated black women as slaves, were well documented in his autobiography. Was Sims simply one of the men of his time who conformed to the social norms existent then? He was one of the few men of those times who treated females when they were considered not even worthy of examination. The use of anesthesia was also uncommon among many surgeons of those times. So should he be given the benefit of the doubt? He perfected his ‘art’ of vesico-vaginal fistula repair on these helpless black enslaved women before using them on white women with use of anesthesia. Sometimes he performed as many as 30 surgeries on a single woman. Sims has himself described the experiments he tried on Lucy, Anarcha and Betsey; the three women whose names are inked in history.[1]

The story of Sims is important and relevant in present times when consent and adherence to ethical guidelines (Declaration of Helsinki) are sacrosanct. The race to greatness and fame for a clinician/ research should not be at the cost of human suffering. The ‘guinea pig’ approach to learning surgery and other aspects of medicine needs to change. The Drug Technical Advisory Board (DTAB), has endorsed adoption of GCP guidelines for streamlining the clinical studies in India which would be useful to Research Institutions, Investigators, Institutional Ethics Committee (IEC) in providing desired direction, protection of rights and welfare of human subjects of biomedical research.[2] 

References

1. https://www.history.com/news/the-father-of-modern-gynecology-performed-shocking-experiments-on-slaves
2. Good Clinical Practice (GCP) Guidelines for Clinical Trials on Pharmaceuticals Products in India Central Drugs Standard Control Organization (CDSCO), DGHS, Ministry of Health and Family Welfare, New Delhi, December 2001. 

Citation

Pai K. Remembering Past Greats: James Marion Sims. JPGO 2018 Volume 5 Number 6. Available from: http://www.jpgo.org/2018/06/remembering-past-greats-james-marion.html