Volume 3 Issue 5, May 2016

Editorial
Gupta AS

Scar Ring Formation Due To Lower Segment Caesarean Sections
Wagh PT, Parulekar SV.

Conservative Management Of Cesarean Section Scar Ectopic Gestation And Lithokelyphos Formation
Mehta D, Parulekar SV.

Endoscopic Retrograde Cholangiopancreatography In Pregnancy
Kale KG, Kalappa SB, Chauhan AR.

Reed’s Syndrome: Rare Tumor Disarray
Pandey I, Gupta AS.

Uterine Perforation With Omental Incarceration During Suction Evacuation
Lothe S, Mali K, Warke HS, Satia MN.

Rare Case of Bilateral Dermoid Cysts Managed Laparoscopically
Shah NH,  Shah VN, Paranjpe SH.

Laparoscopic Management of Right Ovarian Ectopic Pregnancy With Left Ovarian Torsion Following IVF
Shah NH, Bandolkar D, Jilla M, Kale KG.

A Rare Case Report Of Cystic Struma Ovarii With Co-Existing Serous Cystadenoma
Rojekar AV, Fernandes GC.

Vasa Previa
Raut VS, Jain N, Jain P, Velankar K.

Editorial

Gupta AS

Skin diseases in non pregnant women are usually co incidental and not specific to pathology of the reproductive system. A gynecologist is not required to manage dermatological manifestations in women. An obstetrician does frequently encounter various dermatological conditions in their pregnant patients. The pregnant woman consults her obstetrician regarding all dermatological manifestations in pregnancy rather that visiting a dermatologist primarily. The obstetrician should therefore be aware about the various skin manifestations that may manifest in the gravid woman. These dermatological conditions may be either pre-existing, hormone induced or pregnancy specific.

Hormone induced changes that are commonly required to be dealt by the obstetricians include striae gravidarum, chloasma or melasma, skin pigmentation. The hyper-pigmentation tends to occur on the breast, vulva, linea alba, face, etc. These are usually physiological effects of pregnancy and they resolve or reduce in the post postpartum phase but may recur in future pregnancies. These are very distressing to the pregnant patient. Vascular changes under the effect of estrogen also tends to occur in some women like spider nevi, telangiectasias, palmar erythema, hemorrhoids, and varicosities.

Pre existing dermatological manifestations can include infections like candidiasis, tinea; eczema's, psoriasis, atopic dermatitis, etc. Conditions like atopic dermatitis or fungal infections worsen in pregnancy whereas diseases like psoriasis improve in pregnancy. Sometimes a condition may remain unaltered by pregnancy.

Pregnancy specific skin conditions are also known as dermatoses of pregnancy. These are seen only in pregnancy and are a heterogeneous, poorly defined, itchy, inflammatory cutaneous eruptions. These usually are seen in the antenatal period and usually settle down in the puerperium. These are intrahepatic cholestasis of pregnancy, pemphigoid gestationis, polymorphic eruptions of pregnancy and 3 conditions clubbed under atopic eruption of pregnancy. These 3 conditions included in atopic eruption of pregnancy are eczema in pregnancy, prurigo of pregnancy and pruritic folliculitis of pregnancy. The woman is distressed with persistent and repeated itching. Intrahepatic cholestasis of pregnancy and pemphigoid gestationis can adversely affect the fetus. In these pregnancies, fetal monitoring is needed to reduce the risk of fetal distress, morbidity and mortality.

When an obstetrician encounters a woman with skin lesions, a consultation with the dermatologist should always be sought in order to correctly establish a diagnosis and plan the treatment. Many of these women will require steroid based cutaneous preparations and some of them may even need systemic anti histaminic and steroid therapy. Patients with intrahepatic cholestasis of pregnancy will require treatment with bile acid binding substance like ursodeoxycholic acid. These women should not have a prolonged pregnancy and induction of labor may also be considered besides closer fetal surveillance.

At times an obstetrician also is required to be aware about the skin manifestations seen in the newborn. Most of the time these are insignificant occurrences and settle down, however, some of the skin conditions may be a pointer towards more serious inheritable conditions like epidermolysis bullosa.

An evaluation of the patient by a dermatologist is the best option and the patient should always be advised to consult a dermatologist.

We present one case in this issue where the gynecologist was called upon to manage a case of uterine fibroids which was actually a part of the Reed’s syndrome or the MCUL syndrome. In our previous issues the readers have read cases related to dermatological manifestations in pregnancy and in future also we will publish some more interesting cases associated with skin lesions which I am sure will be appreciated by our readers.With this I present the May issue to our esteemed readers.

Scar Ring Formation Due To Lower Segment Caesarean Sections

Author Information

Wagh PT*, Parulekar SV**

(* Senior Resident, ** Professor and Head, Department of Obstetrics and Gynecology,  Seth G S Medical college and KEM Hospital, Mumbai, India.) 

Abstract

Cesarean section scars may cause difficulties during future pregnancies and any surgery the patient has to undergo. We present a case report of an unusual problem during a first trimester medical termination of pregnancy (MTP) due to previous cesarean section scars.

Introduction

Some of the commonly known and faced long term complications due to lower segment cesarean section (LSCS) are uterine scar dehiscence or rupture and intra-abdominal adhesions.[1,2] Other problems like scar endometriosis, adherent placenta, and scar ectopic pregnancy may also be seen.[1,2] In our case, previous caesarean section scar had formed an incomplete ring like constriction around the isthmus of the uterus, which posed difficulty during cervical dilatation and curettage.

Case Report

A 35 year old gravida 3, para 2, living 2 patient with previous 2 LSCS came to us with 7 weeks intrauterine gestation for medical termination of pregnancy. She gave a history of receiving 15-S-15-methyl PGF2alpha intramuscularly followed by an attempt at dilatation and curettage in a private hospital, which had to be abandoned as the cervix could not be dilated. Then tablet misoprost 800 µg was kept per rectally but that also failed to cause any bleeding or cervical dilatation. Hence she was referred to our hospital.

Her medical and surgical history was not contributory. Her last cesarean section had been performed 2.5 years ago. Her general and systemic examination revealed no abnormality. Abdominal examination showed a Pfannenstiel scar and other normal findings. On per vaginal examination the uterus was 7-8 weeks' size, acutely retroflexed and retroverted (cochleate). The fornices were nontender, without any mass. The results of her investigations for fitness for anesthesia were normal. Due to the risk of uterine rupture, we decided not to use pre-procedure misoprostol for cervical dilatation in this patient. She was put in lithotomy position under general anesthesia. the cervix was held with vulsellum to give outward and upward traction to straighten the axis of uterus, uterine sounding was done and dilatation of cervix with Hegar’s dilators was done. Dilatation posed difficulty as we had to negotiate the dilators first in horizontal and then upward and backward direction. There was resistance at two levels – the first at the level of the internal os, and the other about 1 cm above it. Cervical dilatation to 8 mm could be achieved with difficulty. Suction evacuation was performed and check curettage was done. During check curettage, a ring like ridge was felt above the level of the cervix anteriorly (in the area where an LSCS scar would be expected to be present) and laterally. It was thought to be due to previous two caesarean sections that the patient had undergone. The patient made an uneventful recovery after the procedure. After 2 days of procedure we performed ultrasonography on the patient to check for the ring like constriction that was felt during surgery and it was found to be LSCS scar.

Figure 1. Pelvic ultrasonogram showing thickened LSCS scar.

Discussion

Difficulty may be encountered during rapid dilatation of the cervix for first trimester MTP.[3] It may be due to nulliparity or no vaginal delivery in the past in a parous woman (due to deliveries being by LSCS). Scar formation is a process of healing of wounds, including surgical wounds. The lower uterine segment contains more fibrous tissue than smooth muscle, and its incisions heal with fibrosis. Usually the LSCS scar is of the same thickness as the wall of the lower segment when the healing is good, or thinner if the healing is not good due to any reason. A scar thicker than the wall of the lower segment has not been reported in literature so far. In our case there was a scar in the lower segment that produced a ridge projecting into the cavity of the lower segment. That might be explained by presence of two scars close to each other, though such an occurrence has not been reported so far, even in cases with more than two previous LSCS. Another unusual feature in this case was presence of the thick scar on the lateral aspects of the lower segment too. It might have been due to extension of the lower segment incision onto the lateral walls, possibly to overcome difficulty in delivery of the baby. Presence of thick scar tissue on three sides of the lower segment must have caused constriction of the lower segment, which would not yield during rapid dilatation of the cervix. Such a scar may be seen during antenatal ultrasonography, so that difficulty during dilatation for MTP can be anticipated.[4] Cervical resistance to rapid dilatation may be prevented by prior use of misoprostol in a woman who has not delivered any baby vaginally in the past.[5] However there is no method of avoiding difficulty due to a constricting scar in the lower segment during performance of the procedure, and it has to be dealt with if it occurs.

References

1. Hakim-Elahi E, Tovell HM, Burnhill MS. Complications of first-trimester abortion: a report of 170,000 cases. Obstet Gynecol 1990; 76:129-35.
2. Ferris LE, McMain-Klein M, Colodny N, Fellows GF, J Lamont J. Factors associated with immediate abortion complications. CMAJ 1996; 154:1677-1685.
3. Kaunitz AM, Rovira EZ, Grimes DA, Schulz KF. Abortions that fail. Obstet Gynecol 1985; 66:533-37.
4. Basic E, Basic-Cetkovic V, Kozaric H, Rama1 A. ULTRASOUND EVALUATION OF UTERINE SCAR AFTER CESAREAN SECTION. Acta Inform Med. 2012 Sep; 20(3): 149–153.
5. Schulz KF, Grimes DA, Cates W Jr. Measures to prevent cervical injury during suction curettage abortion. Lancet 1983; 1:1182-85.

Citation

Wagh PT, Parulekar SV. Scar Ring Formation Due To Lower Segment Caesarean Sections. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/scar-ring-formation-due-to-lower.html

Conservative Management Of Cesarean Section Scar Ectopic Gestation And Lithokelyphos Formation

Author Information

Mehta D*, Parulekar SV*.

(* First Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)

Abstract

Cesarean scar ectopic pregnanacy is one of the rarest form of ectopic pregnancy. it is presumed as a result of migration of embryo through defect in scar. Here we present Such a case managed conservatively. It ended in formation of a lithokelyphos. This is the first case of lithokelyphos formation in a cesarean section scar in the world literature.

Introduction

Ectopic pregnancy in cesarean section (LSCS) scar is the rarest form of ectopic pregnancy.[1] It is characterized by the gestational sac being completely separated from the uterine cavity and completely surrounded by myometrium and LSCS scar. Its incidence seems to be on the rise, either because of an increase in rate of cesarean sections or an increase in evaluation of early pregnancy by transvaginal ultrasonography(USG).[2] Its clinical presentation varies a lot - painless or painful vaginal bleeding, lower abdominal pain, or severe hemorrhagic shock. Some cases may be asymptomatic. If not treated, there can be complications like rupture and severe hemorrhage. In some cases the pregnancy may continue to grow and the placenta maybe morbidly adherent in the lower segment.[3,4] There are different modes of management of  LSCS scar ectopic pregnancies, medical or surgical.[5,6,7,8,9] We present a case that was successfully managed by observation alone. It progressed to formation of a lithokelyphos. This is the first case of lithokelyphos formation in a cesarean section scar in the world literature.

Case Report

A 24 year old woman, married for 3 years, para presented with a complaint of 3 months of amenorrhea and spotting per vaginum for 1.5 months. There was no history of abdominal pain. She had undergone a cesarean section in past. Her medical and surgical history was not contributory. She was not using any contraception. She gave a history of consumption of mifepristone and misoprostol for achieving a medical abortion 1.5 months ago. She had not passed any products of conception after that. Her vital parameters were normal. Her general and systemic examination revealed no abnormality. Abdominal examination showed a Pfannenstiel scar but no tenderness, guarding, rigidity or lump. Bimanual examination showed the uterus to be mildly enlarged and soft. There was no tenderness or vaginal bleeding. She had reports of transvaginal ultrasonography (USG) and magnetic resonance imaging (MRI) performed 5 and 4 prior to presenting at our hospital. The USG showed a 4x3 cm mass without any fetal pole with decidual reaction reaching up to serosa. The MRI showed a 48x44x21 mm irregular gestational sac in lower uterine segment with diffuse thinning of anterior myometrium and decidual reaction reaching up to serosa. USG performed in our hospital showed a heterogenous mass measuring 3.5x25x3.1 cm in lower uterine segment, with scar thickness 3.5 mm. A diagnosis of cesarean section (LSCS) scar ectopic pregnancy was made. Her hemoglobin was 8.5 g/dl. Remaining hemogram, urinalysis, liver and renal function tests were normal. Serum βhCG level was  3867 mIU/ml. It fell to 2711 mIU/ml after 8 hours. She was counselled about various treatment options. She opted for observation for spontaneous resolution of the LSCS scar ectopic pregnancy. She was give a transfusion of two units of packed red blood cells. Then she was discharged from the hospital with instructions to report immediately in the event of vaginal bleeding or acute pain in abdomen. Serum βhCG levels were 427, 127, 47, and not detectable 1, 2, 3, and 7 weeks later. However her pelvic USG did not show any resolution of the lesion in this period. The mass remained of a size 3.5x25x3.1 cm. It showed deposition of calcium in its walls during the last of the three USG scans performed on her. She was prescribed hematinics for a month and counseled about the risk of rupture of the scar in a future pregnancy at the site of the ectopic pregnancy with litokelyphos formation. She opted not to undergo any surgical treatment for its removal and not to have any more pregnancies.

Figure 1. MRI showing LSCS scar ectopic pregnancy (arrow).

Discussion

The first case of LSCS scar ectopic pregnancy was reported by Larsen and Solomon.[10] The risk of it development is increased further by past myomectomy, manual removal of placenta, dilatation and curettage, in vitro fertilization and embryo transfer, and adenomyosis.[11] Early evaluation by transvaginal USG of a woman with previous LSCS is important in making an early diagnosis of LSCS scar ectopic pregnancy. Diagnostic features include absence of trophoblastic tissue in the uterine cavity and cervical canal, presence of a gestational sac between the anterior wall of the uterus and adjacent wall of the urinary bladder, and thin or absent myometrial layer between the urinary bladder and the gestational sac. If it is less than 8 weeks’ size, a triangulas mass fills the niche of the scar. If it is more than 8 weeks’ size, the shape is rounded. Trophoblastic activity is confirmed by Doppler study.[11]

Medical treatment is preferred so as to preserve fertility. It is with systemic or local methotrexate administration. Surgical treatment options include rapid cervical dilatation and evacuation by suction and curettage, hysteroscopic removal, laparoscopic removal, and uterine artery embolization, and removal by laparotomy. Use of high energy focused ultrasound combined with curettage has also been reported.[12] Abdominal hysterectomy may have to be performed as a last resort in some cases to control hemorrhage in an advanced, ruptured LSCS scar ectopic pregnancy. In our case the patient had ingested mifepristone and misoprostol without medical prescription and supervision. Fortunately the ectopic gestation did not rupture and cause a life threatening hemorrhage. These drugs must have caused fetal demise. So her serial serum βhCG levels were found to be falling. That suggested that her ectopic pregnancy might resolve without and intervention. However that did not happen. The mass remained of the same size, and its walls got calcified, forming a lithokelyphos. A problem with observation or medical treatment is that the defect in the lower segment scar remains, and is likely to rupture in a future pregnancy. With the formation of a lithokelyphos, the risk is possibly increased further. Since cesarean scar ectopic pregnancy and its treatment conservatively are relatively recent, follow up of patients so treated is essential over a few years to see if this risk is real.

References

1. Fylstra DL. Ectopic pregnancy within a caesarean scar: a review. Obstet Gynecol Surv 2002;57:537-43.
2. Timor-Tritsch I, Monteagudo A. Unforeseen Consequences of the Increasing Rate of Cesarean Deliveries: Early Placenta Accrete and Cesarean Scar Pregnancy. A Review. American Journal of Obstetrics & Gynecology. 2012;207:14-29.
3. Ben-Nagi, J., Ofili-Yebovi, D., Marsh, M. and Jurkovic, D. First Trimester Cesarean Scar Pregnancy Evolving into Placenta Previa/Accreta at Term. Journal of Ultrasound in Medicine.2005: 24,1569-1573.
4. Jurkovic D. Cesarean Scar Pregnancy and Placenta Accreta. Ultrasound in Obstetrics & Gynecology. 2014;43, 361-362.
5. Ravhon A, Ben - Chetrit A, Rabinowitz R, Neuman M, Beller U. Successful methotrexate treatment of a viable pregnancy within a thin uterine scar. Br J Obstet Gynaecol 1997;104:628-9.
6. Shufaro Y, Nadjari M. Implantation of a gestational sac in a cesarean section scar. Fertil Steril 2001;75:1217.
7. Lam PM, Lo KW. Multiple - dose methotrexate for pregnancy in a cesarean section scar. A case report. J Reprod Med 2002;47:332-4.
8. Ghezzi F, Laganà D, Franchi M, Fugazzola C, Bolis P. Conservative treatment by chemotherapy and uterine arteries embolization of a cesarean scar pregnancy. Eur J Obstet Gynecol Reprod Biol 2002;103:88-91.
9. Fylstra DL, Pound - Chang T, Miller MG, Cooper A, Miller KM. Ectopic pregnancy within a cesarean delivery scar: a case report. Am J Obstet Gynecol 2002;187:302-4.
10. Larsen JV, Solomon MH. Pregnancy in a uterine scar sacculus - an unusual cause of postabortal haemorrhage. A case report. S Afr Med J 1978;53:142-3.
11. Jurkovic  D, Hillaby  K, Woelfer B, Lawrence A, Salim R, Elson CJ. First trimester diagnosis and management of  pregnancies implanted into the lower uterine caesarean section scar. Ultrasound Obstet Gynecol 2003;21:220-7.
12. Huang L, Du Y, Zhao C. High intensity focused Ultrasound   combined with Dilatation and curettage for Cesarean scar pregnancy. Ultrasound Obstet and Gynaecol 2014;43:98-101.

Citation
Mehta D, Parulekar SV. Conservative Management Of Cesarean Section Scar Ectopic Gestation And Lithokelyphos Formation. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/conservative-management-of-cesarean.html

Endoscopic Retrograde Cholangiopancreatography In Pregnancy

Author Information

Kale KG *, Kalappa SB **, Chauhan AR ***

(* Assistant Professor, ** First Year Resident, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India)

Abstract

Symptomatic choledocholithiasis in pregnancy, though rare, is an emergency requiring prompt treatment to prevent fatal maternal and fetal complications. Surgery, in the form of cholecystectomy with common bile duct exploration has high rate of fetal loss, hence is not preferred. Recently, therapeutic endoscopic retrograde cholangiopancreatography (ERCP) has been evaluated and documented as a safe and effective alternative to surgery. However, there still exist concerns about fetal radiation exposure during ERCP.  We present a case of symptomatic choledocholithiasis in second trimester of pregnancy treated with therapeutic ERCP leading to a good maternal and fetal outcome.

Introduction

Pregnancy is a "lithogenic" state. Marked elevation in the serum levels of estrogen and progesterone in pregnancy leads to increase in the biliary cholesterol concentration, decreased bile acid secretion and decreased gall bladder motility, thus favoring formation of gall stones.[1] The prevalence of gall stone disease in pregnancy is estimated to be 3 - 12% and symptomatic choledocholithiasis complicates 1 in every 1000 - 1200 pregnancies.[2]  Choledocholithiasis often leads to cholangitis and/ or gallstone pancreatitis, both of which may prove fatal to the mother and the fetus, hence should be treated promptly.[3] Therapeutic endoscopic retrograde cholangiopancreatography (ERCP) is considered as the current standard of care in the treatment of choledocholithiasis in pregnancy.[4] Although the safety of ERCP has been established by various case series till date,[2- 9] there still exist concerns about the risk of radiation exposure to the fetus. 

Case Report

A 22 years old female, gravida 2 presented at 24 weeks' of gestation with moderate to severe colicky pain in right hypochondrium, nausea and vomiting. On physical examination, jaundice was evident in the sclera and tenderness was present over the right hypochondrium. Uterus corresponded to 24 weeks' size without any uterine activity. Fetal heart sounds were regular on Doppler. On per vaginal examination, cervical os was closed. 

Laboratory investigations showed normal hemogram, elevated total and direct bilirubin and elevated liver enzymes (total bilirubin- 2.4mg/dL, direct bilirubin- 1.36 mg/dL, AST- 88 U/L, ALT- 106 U/L, alkaline phosphatase- 212 U/L, GGT- 46 U/L). Abdominal ultrasonogram revealed multiple small gall bladder calculi, a 9 mm calculus in the distal common bile duct (CBD) and dilatation of proximal CBD with diameter of 14 mm. Obstetric ultrasound revealed single live intrauterine fetus corresponding to 23 weeks 5 days. An emergency ERCP was planned. ERCP was performed under intravenous sedation, with the patient in supine position, by an experienced endoscopic surgeon. Fetal heart was monitored pre and post operatively. Lead shielding of lower abdomen was done to minimize fetal dosing. CBD was cannulated and cholangiogram obtained which confirmed multiple stones in biliary tree including the CBD. Sphincterotomy was performed and a 9 mm CBD stone was removed using a balloon catheter followed by placement of a 10 Fr stent. No hard copy radiographs were taken during the procedure and fluoroscopy was used for as short a time as possible (2 minutes 10 seconds). There were no procedure related complications and patient had a rapid post-operative recovery with normalization of laboratory parameters. 

Patient was regularly followed up in antenatal and gastroenterology OPD. Patient carried the pregnancy to term without any further complications or recurrence of symptoms and delivered a healthy female child of 3.08 kg vaginally. Apgar scores at 1 and 5 minutes were 8 and 9 respectively. No obvious congenital malformations were found. Patient was discharged along with the newborn on day 4 postpartum and was advised to follow up in gastroenterology OPD for definitive management. 

Discussion

Symptomatic choledocholithiasis with cholangitis and/or gallstone pancreatitis is a surgical emergency with a grave prognosis for both the mother and the fetus if not treated promptly.[3] Traditional approach consisting of cholecystectomy with CBD exploration has high incidence of preterm labor and fetal loss; hence should be avoided. [2] Therapeutic ERCP with its well documented safety and efficacy for both the mother and the fetus, is currently the treatment modality of choice for symptomatic choledocholithiasis in pregnancy.[2 - 9] 

The primary concern with ERCP is the fetal exposure to ionizing radiations causing intrauterine growth retardation, fetal anomalies, intrauterine fetal death and childhood cancers.[7] The threshold conceptus dose for these fetal effects is estimated to be 100 - 200 mGy which is much larger than the average dose delivered during a routine ERCP. [2] The average fetal radiation exposure during ERCP in various studies ranges from 0.1 to 11 mGy [5-7] with a mean fluoroscopy time ranging from 8 seconds to 3.8 minutes. [3,5,6] According to the American College of Obstetricians and Gynecologists (ACOG), a radiation exposure of < 50 mGy is not associated with an appreciable increased rate of fetal loss or anomalies.[10] Thus, with minimal radiation exposure, the measurement of radiation exposure for routine ERCP procedures appears unnecessary.[8] The importance of various measures taken to minimize radiation exposure to the fetus, as was done in our case, has been stressed upon in the literature. These measures include limiting fluoroscopy time, lead shielding of abdomen, using low dose settings, avoiding hard copy radiographs and appropriate patient positioning to minimize fetal dosing.[2, 4-8]  

The risk of preterm delivery after therapeutic ERCP is less, with a term pregnancy rate ranging from 89.8 % to 94.4 % in various studies.[3, 7, 9] Tang in 2009 reported that term pregnancy rate was lowest for patients who underwent an ERCP in first trimester with high preterm delivery rate of approximately 20 %.[9] Second trimester appears to be the safest period to perform ERCP.[4] No reported cases of intra procedure fetal distress [2], or procedure-related perinatal deaths, stillbirths or fetal malformations have been documented. [2,3,9] The 5 minute Apgar scores of babies born to mothers who have undergone ERCP was ≥ 8.[2,5,7] The longest follow up (6 years) by Gupta et al[3] have found no developmental or congenital abnormalities in these children. The incidence of other procedure related complications, viz. post- ERCP pancreatitis and post- ERCP bleed was similar to that seen in non-pregnant population.[7]  

In an effort to eliminate radiation exposure, few authors have tried various modifications of therapeutic ERCP. Akcakaya et al[11] and Sharma et al[12] implemented cannulation of CBD using a guidewire and confirmed its position by aspiration of bile (fluoroscopy was not used). This was followed by sphincterotomy and balloon sweeping of the duct with/without stent placement. This approach however has various disadvantages: Firstly, there is risk of inadvertently cannulating the cystic duct. Secondly, complete stone clearance is not achieved due to lack of visualization of biliary tree which may lead to recurrence.[8] To overcome these disadvantages, Shelton et al[12] have recommended the use of real-time percutaneous ultrasonography, intraductal ultrasonography, linear echoendoscopy and choledochoscopy during the performance of non-radiation ERCP. However, greater degree of experience is required with these novel techniques of non- radiation ERCP before they can be routinely employed.

Conclusion

Therapeutic ERCP appears safe and effective in the management of symptomatic choledocholithiasis in pregnancy. Second trimester appears to be the safest period for performance of the procedure with high term delivery rates and good fetal outcome. All measures to reduce fetal radiation exposure should be undertaken. Newer techniques of non radiation ERCP should be evaluated further for possible use in future.

References

1. de Bari O, Wang TY, Liu M, Paik CN, Portincasa P, Wang DQ. Cholesterol cholelithiasis in pregnant women: pathogenesis, prevention and treatment. Ann Hepatol. 2014 Nov-Dec; 13(6):728-45.
2. Daas AY, Agha A, Pinkas H, Mamel J, Brady PG. ERCP in pregnancy: is it safe? Gastroenterol Hepatol (N Y) 2009; 5(12): 851-5.
3. Gupta R, Tandan M, Lakhtakia S, Santosh D, Rao GV, Reddy DN. Safety of therapeutic ERCP in pregnancy - an Indian experience. Indian J Gastroenterol 2005; 24(4): 161-3.
4. Al-Hashem H, Muralidharan V, Cohen H, Jamidar PA. Biliary disease in pregnancy with an emphasis on the role of ERCP. J Clin Gastroenterol. 2009; 43(1):58-62.
5. Tham TC, Vandervoort J, Wong RC, Montes H, Roston AD, et al. Safety of ERCP during pregnancy. Am J Gastroenterol. 2003; 98(2):308-11.
6. Kahaleh M, Hartwell GD, Arseneau KO, Pajewski TN, Mullick T, et al. Safety and efficacy of ERCP in pregnancy. Gastrointest Endosc. 2004; 60(2):287-92.
7. Fine S, Beirne J, Delgi-Esposti S, Habr F. Continued evidence for safety of endoscopic retrograde cholangiopancreatography during pregnancy. World J Gastrointest Endosc 2014; 6(8): 352-8.
8. Smith I, Gaidhane M, Goode A, Kahaleh M. Safety of endoscopic retrograde cholangiopancreatography in pregnancy: Fluoroscopy time and fetal exposure, does it matter? World J Gastrointest Endosc 2013; 5(4): 148-153.
9. Tang SJ, Mayo MJ, Rodriguez-Frias E, Armstrong L, Tang L, Sreenarasimhaiah J, Lara LF, Rockey DC. Safety and utility of ERCP during pregnancy. Gastrointest Endosc 2009; 69(3 Pt 1): 453-61.
10. ACOG Committee on Obstetric Practice. ACOG Committee Opinion. Number 299, September 2004. Guidelines for diagnostic imaging during pregnancy. Obstet Gynecol. 2004;104: 647-651.
11. Akcakaya A, Ozkan OV, Okan I, Kocaman O, Sahin M. Endoscopic retrograde cholangiopancreatography during pregnancy without radiation. World J Gastroenterol 2009; 15(29): 3649-3652.
12. Sharma SS, Maharshi S. Two stage endoscopic approach for management of choledocholithiasis during pregnancy. J Gastrointestin Liver Dis 2008; 17(2): 183-5.
13. Shelton J, Linder JD, Rivera-Alsina ME, Tarnasky PR. Commitment, confirmation, and clearance: new techniques for nonradiation ERCP during pregnancy (with videos). Gastrointest Endosc 2008; 67(2): 364-8.

Citation

Kale KG, Kalappa SB, Chauhan AR. Endoscopic Retrograde Cholangiopancreatography In Pregnancy. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/endoscopic-retrograde.html

Reed’s Syndrome: Rare Tumor Disarray

Author Information

Pandey I*, Gupta AS**

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

Abstract

Reed’s syndrome is an inherited disorder where unusual skin manifestations coexist with uterine leiomyomas (MCUL- multiple cutaneous and uterine leiomyomatosis). Leiomyomas are benign tumors, originated from smooth muscle cells. Cutaneous leiomyomas originate from the erector pili muscle of the hair follicle. These are uncommon, unrecognized and under- diagnosed tumors. Morphologically skin lesions are multiple, small, oval, reddish-brown tumors around hair follicles. Histopathological examination confirms the diagnosis. We report a case of an elderly woman who presented with symptomatic, recurrent uterine fibroid requiring surgical intervention, with multiple, occasionally painful skin lesions. 

Introduction

Reed’s syndrome is a condition known for the coexistence of benign smooth muscle tumor in the uterus and the skin.[1] An autosomal dominant pattern of inheritance established by Reed et al in 1973.[2,3] Several synonyms have been used for this condition like multiple cutaneous and uterine leiomyomatosis (MCUL). This syndrome may be associated with papillary type 2 renal cell carcinoma, and termed as hereditary leiomyomatosis and renal cell cancer (HLRCC).[4] Mutation in a disease predisposing gene "fumarate hydratase", an important gene of our mitochondrial cellular energy metabolism, has been identified. Our patient presented with menorrhagia and was detected to have multiple uterine fibroids. A supracervical hysterectomy was only possible due to dense adhesions of the cervix to the sigmoid colon. Multiple, small, reddish-brown, painful skin lesions were present around hair follicles; they were biopsied.

Case Report

A 45 year old woman unsuccessfully treated for primary infertility presented to our department with complaints of menorrhagia and pelvic pain. Clinical examination, pelvic ultrasonography (USG), contrast-enhanced CT scan (CECT), and PET scan detected multiple uterine fibroids. She had similar complaints at the age of 28 years; subsequently she underwent myomectomy for uterine fibroid. She was on treatment for hypertension and diabetes mellitus; both were well controlled. CECT detected incidentally a benign adrenal adenoma. A PET scan confirmed the diagnosis. An endocrine evaluation with ACTH, basal cortisol, plasma free and urine free metanephrins, and 18-hydroxycortisol was done and all levels were within normal range. Her complete blood counts, liver and renal function test, chest X ray, and ECG were normal. On examination there were multiple groups of skin colored to red colored papules and nodules present on the back, thigh, and gluteal region. Mild tenderness was present over these lesions. She had these lesions since 15 years and they had increased in number. Dermatologic opinion was taken. A 6 mm deep biopsy of the nodules was taken and sent for histopathology examination. Histopathology showed smooth muscle cell proliferation, intermixed with collagen fibers and few lymphocytes suggestive of leiomyoma of skin. A total hysterectomy was planned; however only a supracervical hysterectomy was possible due to dense adhesions of the cervix to the sigmoid colon. These adhesions could not be released by the general surgeons also. Intraoperatively multiple fibroids with bosselation on the surface of uterus were seen. These multiple fibroids expanded the fundus and uterine body transversely. They extended from the right side broad ligament posteriorly, covering the bladder field anteriorly, and partly into the left side of the broad ligament. The anatomy of the uterus was distorted.  Specimen was sent for histopathology, which showed multiple leiomyomata of uterus with one of them showing features of symplastic leiomyoma. The mitotic figure were less than 1/10 HPF. Uterus size was 13 x 12 x 9 cm, corpus was 8 cm and 3 cm of cervix. Largest fibroid measured 8 x 6 x 5 cm in size. Endometrial cavity was normal. No evidence of malignancy was found.  No tumor necrosis was seen. On direct questioning, she said that her mother also underwent a hysterectomy for similar complaints but was never evaluated properly. Patient has been asked to follow up regularly.

Figure 1.  Contrast enhanced CT scan. Yellow arrows have outlined the enlarged uterus with multiple fibroids.

Figure 2.  Multiple, small, oval, reddish-brown papules and nodules in a group on the back.

Figure 3. Gross surgical specimen.

Discussion

Reed’s syndrome is a rare hereditary neoplastic condition. It is associated with uterine leiomyomas and multiple cutaneous leiomyomas. In some cases it may be associated with type 2 papillary renal cell cancer and about 16% of patients may develop aggressive renal cell cancer.[5] It has an autosomal dominant inheritance of mutation in the loss of function of  fumarate hydratase enzyme.[6] The gene for this enzyme is located on chromosome 1q42.3-q43, called MCUL 1 locus.[7] Though the exact pathologic mechanism has not been understood, the cells which are deficient in function of fumarate hydratase enzyme have defective Kreb’s cycle.[8] Hypoxic state created due to it may be responsible for cellular transformation and tumorigenesis.[9] Fumarate hydratase enzyme gene may act as tumor suppressor gene, but its consequence is not yet identified.[10]   Mostly symptoms appear in young to middle age patients. Though approximately 80% of the patients have multiple or single cutaneous leiomyomas, a small proportion may present simply with uterine leiomyomas, may or may not be associated with an underlying renal cell carcinoma.[5]  Multiple uterine and cutaneous leiomyomatosis in our patient established the diagnosis of Reed Syndrome. Our patient did not have features of renal cell carcinoma even on PET scan. She had one symplastic leiomyoma wherein the mitotic figure were less than 1/10 HPF. Symplastic /bizarre leiomyoma contains multinucleated tumor cells with moderate to severe atypia. Usually these have a benign course. They are associated with the use of progesterone.[11] Some patients may present with linear configuration of  single cluster of cutaneous leimyomas along the Blaschko’s line due to genetic mosaicism.[12] The lesions of this type may be misdiagnosed as herpes zoster infection.[13] No specific diagnostic criteria has been established, and diagnosis is generally made by practical clinical criteria.[14] The definite diagnosis of Reed syndrome can be established by identifying the fumarate hydratase enzyme gene mutation.[6] Early detection and treatment of associated renal cell carcinoma is the most important aspect. One of the suggested screening programmes is first renal imaging at third decade of life, followed by biannual renal ultrasound and annual MRI.[7] 

Based on the size, number of uterine leiomyoma and the severity of symptoms, surgical management including hysterectomy are generally required.[15,16] Moreover, alternative treatment modality for uterine leiomyoma includes myomectomy, uterine artery embolization, or medical management with gonadotropin releasing hormone agonists. Skin lesions are usually benign. Solitary tumors may require surgical excision, cryoablation. Multiple painful tumors can be treated with nitroglycerol, calcium channel blockers, and alpha-adrenorecepter blockers to relieve pain.[17] Genetic counseling may be recommended as the condition is inherited.

Conclusion

Reed’s syndrome mainly includes benign tumors of skin and uterus. Moreover, renal cell carcinomas that may be found in a subset of cases are very aggressive in clinical course. Hence, early diagnosis and appropriate surveillance is needed. Genetic analysis should be performed depending on the feasibility and affordability.

References

1. Emer JJ, Solomon S, Mercer SE. Reed’s Syndrome. A Case of Multiple Cutaneous and Uterine Leiomyomas. J Clin Aesthet Dermatol. 2011; 4(12):37-42. 
2. Reed WB, Walker R, Horowitz R. Cutaneous leiomyomata with uterine leiomyomata. Acta Derm Venereol. 1973; 53(5):409–416.
3. Kloepfer HW, Krafchuk J, Derbes V, Burks J. Hereditary multiple leiomyoma of the skin. Am J Hum Genet. 1958;10(1):48–52.
4. Kiuru M, Launonen V. Hereditary leiomyomatosis and renal cell cancer (HLRCC). Curr Mol Med. 2004; 4(8):869–875.
5. Pithukpakorn M, Toro JR. “Hereditary leiomyomatosis and renal cell cancer,” in: Gene Reviews [Internet], Editors: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A,  Bean LJH,  et al., University of Washington,  Seattle, Washington, USA, 2006. http://www.ncbi.nlm.nih.gov/ books/NBK1252/.
6. Smit DL, Mensenkamp AR, Badeloe S, Breuning MH,  Simon ME,  Van Spaendonck KY, et al. Hereditary leiomyomatosis and renal cell cancer in families referred for fumarate hydratase germline mutation analysis. Clin Genetics. 2011; 79 (1): 49–59.
7. Alam NA, Bevan S, Churchman M, Barclay E, Barker K, Jaeger EE, et al. Localization of a gene (MCUL1) for multiple cutaneous leiomyomata and uterine fibroids to chromosome 1q42.3-q43. Am J Hum Genet. 2001; 68(5):1264–9.
8. Badeloe S, van Geel M, van Steensel MA, Bastida J, Ferrando J, Steijlen PM, et al. Diffuse and segmental variants of cutaneous leiomyomatosis: novel mutations in the fumarate hydratase gene and review of the literature. Exp Dermatol. 2006; 15(9):735–741.
9. Sudarshan S, Pinto PA, Neckers L, Linehan WM. Mechanisms of Disease: hereditary leiomyomatosis and renal cell cancer—a distinct form of hereditary kidney cancer.  Nat Clin Pract Urol. 2007; 4(2):104–110.
10. Alam NA, Rowan AJ, Wortham NC, Pollard PJ, Mitchell M, Tyrer JP, et al. Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency. Hum Mol Genet. 2003; 12(11):1241-52.
11. Downes KA, Hart WR. Bizzare leiomyomas of the uterus: a comprehensive pathologic study of 24 cases with long-term follow-up. Am J Surg Pathol. 1997; 21(11):1261-70.
12. Hernandez C, Rivera CM. Multiple painful cutaneous facial papules. South Med J. 2008; 101(11):1180–2. 
13. Agarwalla A, Thakur A, Jacob M, Joshi A, Garg VK, Agrawal S. Zosteriform and disseminated lesions in cutaneous leiomyoma. Acta Derm Venereol. 2000 Nov-Dec; 80(6):446.
14. Smit DL, Mensenkamp AR, Badeloe S, Breuning MH, Simon ME, van Spaendonck KY et al. Hereditary leiomyomatosis and renal cell cancer in families referred for fumarate hydratase germline mutation analysis. Clin Genet. 2011 Jan; 79(1):49-59.
15. Alam NA, Barclay E, Rowan AJ, Tyrer JP, Calonje E, Manek S, et al. Clinical features of multiple cutaneous and uterine leiomyomatosis: an underdiagnosed tumor syndrome. Arch Dermatol. 2005 Feb; 141(2):199-206.
16. Stewart L, Glenn GM, Stratton P, Goldstein AM, Merino MJ, Tucker MA, et al. Association of germline mutations in the fumarate hydratase gene and uterine fibroids in women with hereditary leiomyomatosis and renal cell cancer. Arch Dermatol 2008; 144(12): 1584–1592.
17. Ritzmann S, Hanneken S, Neumann NJ, Ruzicka T, Kruse R. Type 2 segmental manifestation of cutaneous leiomyomatosis in four unrelated women with additional uterine leiomyomas (Reed's Syndrome). Dermatology. 2006; 212(1):84-7.

Citation

Pandey I, Gupta AS. Reed’s Syndrome: Rare Tumor Disarray. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/reeds-syndrome-rare-tumor-disarray.html

Uterine Perforation With Omental Incarceration During Suction Evacuation

Author Information

Lothe S*, Mali K**, Warke HS***, Satia MN****

(* Second Year Resident, ** Assistant Professor, *** Associate Professor, **** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

Suction evacuation for missed or incomplete abortion is one of the commonly performed procedures in obstetrics worldwide. Incomplete abortion, excessive postabortal bleeding, uterine perforation and infection are some of the complications of the procedure. Uterine perforation is one of the rare but serious complication of the procedure. Factors leading to perforation include training level of a care-giver, retroverted uterus, infections such us pelvic inflammatory disease advanced gestational age, previous operations  on the uterus eg. caesarean section. Many cases remain asymptomatic and require only close observation, whereas some cases undergo short and long term complications requiring early management. We present a case of 28 year old female referred to us in view of uterine perforation during suction evacuation for missed abortion with omentum extruding out through the cervix which was managed successfully in our institute.

Introduction

Suction evacuation is an optimal  surgical procedure for missed abortion as it is safe, quick, easy to perform and associated with minimal blood loss. Uterine perforation is one of the complication of the procedure. Incidence is underestimated as many cases are  unrecognised. Reported incidence is 5% in pregnant and peripartum women.[1] Most commonly associated instruments causing uterine perforation are cervical dilators and the uterine sound. Most commonly perforated area is the relatively avascular fundus.[2] Perforation can lead to injury to the surrounding viscera and blood vessels. Incidence generally depends upon self reporting by the surgeon and is underestimated as many cases are not recognized or confirmed. Diagnosis is made by surgeon’s suspicion of perforation when no tissue is obtained or the instrument is inserted deeper than expected, or dragging of abdominal contents through cervix. Ultrasonography, computed tomography, X-ray abdomen may be done to confirm the diagnosis. Management depends upon the cause and the extent of the perforation and the clinical presentation of the patient. If the perforation is caused by a blunt instrument and the patient is asymptomatic, monitoring for 24 hours is done. In most of these cases no intervention is required. Surgical repair is indicated in cases where signs of perforation are present or injury to the abdominal contents is evidenced. There are case reports describing small intestine,[2,3] appendix[4], omentum,[5] and ovary[6] entering the uterine cavity because of perforation. In our case, omentum was seen extruding out through the cervix for which exploratory laparotomy was performed.

Case Report

28 years old female, married since 13 years G4 P2 L2 A1 with previous two full term normal deliveries had visited a private consultant with an ultrasonography report suggestive of missed abortion. Patient was posted for suction evacuation. During the procedure, uterine perforation occurred and omentum was sucked out and visualised through the cervix. Post procedure patient was immediately transferred to our institute for further management. On general examination she was pale and her vital parameters were stable. On abdominal examination there was minimal tenderness on deep palpation and no guarding or rigidity. On speculum examination omentum was seen coming through the cervix. On vaginal examination uterus was anteverted, corresponded to 6 weeks, os was closed with minimal bleeding and bilateral forniceal tenderness was present. USG was done which indicated a suspicious rent at the fundus on the right side with mild hemoperitoneum and blood clots in the pelvis highly suggestive of  uterine perforation. Computed tomography scan of abdomen and pelvis was done which was suggestive of a 3 mm wide defect in the myometrium in the fundus with periuterine collection with pockets of air. Upper abdominal viscera was normal. 

In view of above findings the patient was taken for exploratory laparotomy with uterine rent repair. Intra-operatively there was small 2 x 1 cm cornual perforation on right side with  small hematoma of 0.5 x 1 cm seen  around the uterine perforation. Omentum was seen at the site of perforation going in to the uterine cavity and coming out at the cervix. Blood clots of approximately 160 grams were evacuated from the peritoneal cavity. Omentum inside the uterine cavity was removed by blunt dissection, the omental part of around 3 cm which had  extruded inside the uterine cavity was excised and the rest omentum reduced back into the peritoneal cavity. Small bowel was traced and no injury was seen, as confirmed by surgeons. Uterine rent on right cornual side was sutured with chromic catgut no 2 in continuous interlocking manner. Haemostasis was achieved. Intraoperatively one unit of whole blood transfusion was given. Patient tolerated the procedure well and postoperative course was uneventful. Patient was discharged on day 8 postoperatively.

Figure 1. Sutured uterine rent after removal of omentum from the uterine cavity.

Discussion

Suction evacuation is a commonly performed surgical procedure in obstetrics with overall low complication rates, and can be performed as inpatient as well as outpatient procedure. Uterine perforation is one of the rare complications and can prove serious at times. Acutely retroverted uterus, previous cesarean scar, previous abortion, advanced gestational age, infection, factors altering the strength of myometrium like breastfeeding and pregnancy, and cervical stenosis which makes endometrial cavity access difficult, are some of the factors contributing to the occurrence of this complication. Consequences vary widely from totally asymptomatic postoperative course to fatal outcomes due to severe hemorrhage and sepsis. Short term complications include severe hemorrhage and long term complications such as perforation in the subsequent pregnancy can occur. Other complications include extremely thin myometrium, sacculation and dehiscence. Most of the times diagnosis is made by the surgeon’s suspicion of a perforation and sometimes by observing the extrusion of the abdominal contents through the cervix.  

In most cases patients may remain asymptomatic and do not require any intervention. Observation for vaginal bleeding, changes in vital parameters and signs of peritonitis for 24 hours is mandatory. In cases where uterine perforation has led to injury to the abdominal organs, patient may present with nausea, vomiting, severe bleeding, abdominal pain, fever, tachycardia and in such cases operative intervention is indicated. Ultrasonography may be used to confirm the diagnosis. Complete blood count, serum electrolytes are done to detect the presence of infection and coagulation profile is done to exclude disseminated intravascular coagulation. X-ray abdomen may reveal presence of air in abdominal cavity. Computed tomography and magnetic resonance imaging may be done in some cases for confirmation. Finally laparoscopy or in some cases laparotomy may have to be done to confirm the diagnosis. Management depends upon the instrument causing the perforation and the extent of perforation. In cases where abdominal organ injury is confirmed, laparoscopy to confirm the site and extent of the perforation is performed and if the bleeding is minimal then first procedure is completed under laparoscopic guidance. In cases with severe bleeding exploratory laparotomy with surgical repair of uterine rent is needed. Cases have been reported where total abdominal hysterectomy had to be performed in cases of uterine perforation. In case where facilities are not available the patient should be immediately transferred to higher centre for definitive management as in our case. 

Preoperative cervical ripening using misoprostol may reduce the morbidity of the surgical termination procedure including hemorrhage, cervical trauma, incomplete evacuation and uterine perforation.[7]

References 

1. McElin TW, Bird CC, Reeves BD, Scott RC. Diagnostic dilatation and curettage. A 20- year survey. Obstet Gynecol. 1969; 33(6): 807-12. 
2. Coughlin LM, Sparks DA, Chase DM, Smith J. Incarcerated small bowel associated with elective abortion uterine perforation. Journal of Emergency Medicine. 2013; 44(3): e303-e306.  
3. Augustin G, Majerović M, Luetić T. Uterine perforation as a complication of surgical abortion causing small bowel obstruction: a review. Arch Gynecol Obstet. 2013; 288(2): 311-323.
4. Dignac A, Novellas S, Fournol M, Caramella T, Bafghi A, Chevallier P. Incarceration of the appendix complicating a uterine perforation following surgical abortion: CT aspects. Emerg Radiol. 2008; 15(4): 267-269.
5. Ozaki K, Suzuki S. Uterine perforation with omentum incarceration after dilatation and evacuation/curettage. Arch Gynecol Obstet. 2013; 287(3): 607-8.
6. Su S, Tao G, Dong B, Shi L, Dong J. Delayed presentation of uterine perforation with ovary migration after dilatation and curettage. Int J Clin Exp Med. 2015; 8(4):6311-4.
7. Grimes DA, Schulz KF, Cates WJ. Prevention of uterine perforation during curettage abortion. JAMA 1984; 251(16): 2108-11.

Citation

Lothe S, Mali K, Warke HS, Satia MN. Uterine Perforation With Omental Incarceration During Suction Evacuation. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/uterine-perforation-with-omental.html

Rare Case of Bilateral Dermoid Cysts Managed Laparoscopically

Author Information 

Shah NH *,  Shah VN **, Paranjpe SH ***

(* Hon. Endosopic Surgeon Wadia Hospital & Railway Hospital, Byculla, ** Anesthesiologist, *** Director: Velankar Hospital & Paranjpe Maternity Home, Chembur, Mumbai, India.)

Abstract 

Mature cystic teratomas are mostly benign. 0.1-0.2% of cases may undergo malignant transformation. They are usually asymptomatic but sometimes may present with acute abdomen due to torsion of cyst, infection, or cyst rupture. Here we present a case of a 23 year old woman non gravid with previous one abortion who came to us with lower abdominal pain for last 6 months, with bilateral dermoid cysts of ovary, which is a rare presentation. Both the dermoid cysts were enucleated laparoscopically and placed in an endobag and retrieved by morcellation. Six month follow up showed no evidence of recurrence.

Introduction 

An ovarian dermoid cyst or a benign cystic mature teratoma is a benign tumor arising from the germinal cells.[1] In approximately 80 % of the cases, it occurs in young women  aged 20 to 30 years and accounts for 18% of all benign ovarian tumors. Most of the time, dermoid cysts are unilateral, but they are bilateral in 10 to 15% of cases.[2] Laparotomy  has  been   the  preferred  procedure  for  the  management  of  dermoid  cysts because  of  the  risk  of  chemical  peritonitis  occuring  from spillage of its contents.[3] We have done morcellation  in  an  endobag,  which  preserves  the  benefits of the laparoscopic  approach while maintaining  the safety of non spillage of contents as achieved in a laparotomy.

Case Report 

A  23 year old  non gravid woman with previous one abortion presented to us with pain in the  lower abdomen for the past 6 months. The pain was as dull  aching, non radiating. There was  no  associated  vaginal  discharge, increased urinary frequency, fever, or bowel symptoms. Her  menses  were regular with a 30 day cycle,  and  no  history  of  associated dysmenorrhea  or  menorrhagia. No significant medical or surgical history was present. On examination, her abdomen was soft with no guarding, rigidity or tenderness. Per speculum examination showed no   vaginal discharge. Per vaginal  examination  showed a normal-sized uterus with bilateral adnexal masses of 5 x 4 cm on the left and 4 x 6 cm on the right. The ultrasound scan was suggestive of a bilateral dermoid cyst. The  uterus was normal (58 x 32 x 29 mm) with endometrial thickness of   8 mm. All preoperative investigations including CA 125 were within normal limits.  

A laparoscopic bilateral cystectomy was done with “in bag” morcellation. Histopathology report confirmed mature cystic teratomas. A 6 months follow-up of this patient was done and there was no recurrence or any signs of granulomatous peritonitis, and she had regular menses after surgery.

Figure 1. Bilateral dermoid cysts seen.

Figure 2. After enucleation of the dermoid cysts.

Discussion 

Dermoid cysts may be formed by elements arising from all three of the germinal  layers, but in ovarian dermoids, ectodermic discrimination frequently occurs. Usually, the diameter of an ovarian dermoid is <10 cm and is rarely >15 cm. On histology, lipidic substance, hair, sebaceous secretions, hair follicles, and bone calcifications are seen in half of the cases; organoid morphology (teeth, bone) are seen in 30% of the cases. [3] Surti et al [4] theorized 5 mechanisms of origin of a dermoid cyst which were: 

• Error of meiosis 1

• Error of meiosis 2 

• End reduplication of an haploid egg cell

• Premiotic  germ cell undergoing mitotic division

• Fusion of two ova.

This being said, the few bilateral cases which had been studied, did not show preponderance of any mechanism.

Usually, the symptoms arise severely with pelvic pain, and in a few of the cases, the symptoms are related with menstrual irregularities. Torsion is the most common complication, whereas rupture and infection are rare.[5] Malignant transformation occurs in 1 to 2% of the cases, commonly arising from squamous epithelial cells. The diagnosis of ovarian dermoid can be made confidently on an ultrasound.  In one study, experienced radiologists achieved 100 % positive predictive value in cases where ≥ 2 characteristic sonographic features were present.[6] It has also been reported that women with bilateral or multiple dermoid cysts have more predisposition for development of ovarian germ cell neoplasms in later life.[7]

Literature suggests that dermoid cysts be removed by laparotomy only, because of the presence of the risk of spillage of sebaceous and hairy material in the peritoneum, causing granulomatous peritonitis in some cases. As of now, laparoscopic management of a dermoid cyst with a morcellation bag is considered a safe option. 

The only concern is the skill of the operating surgeon. In the presence of a skilled laparoscopic surgeon, appropriately assisted by well-trained assistants, the procedure maintains the basic conventions of the abdominal approach. Furthermore, reduced tissue handling and drying, which is distinctive  in laparoscopy, may contribute to reducing adhesion formation after laparoscopy. However in case intra- operative spillage occurs, cyst contents should be removed immediately from the peritoneal cavity and repeated washing and aspiration should be done.[8]

To conclude, laparoscopic management of bilateral, mature cystic teratomas by  an  experienced  surgeon could yield results as good as laparotomy.

References

1. Bazot M, Cortez A, Sananes S, Boudghene F, Uzan S, Bigot JM. Imaging of dermoid cysts with foci of immature tissue. J Comput Assist Tomogr 1999; 23(5):703-6.
2. Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: Tumor types and imaging characteristics. Radiographics. 2001; 21(2):475-90.
3. Campo S, Garcea N. Laparoscopic conservative excision of ovarian dermoid cysts with or without an endobag. J Am Assoc Gynecol Laparosc.1998; 5(2):165–70.
4. Surti U, Hoffner L, Chakravarti A, Ferrell RE. Genetics and biology of human ovarian teratomas. I. Cytogenetic analysis and mechanism of origin. Am J Hum Genet. 1990; 47(4): 635–43
5. Comerci JT Jr, Licciardi F, Bergh PA, Gregori C, Breen JL. Mature cystic teratoma: a clinicopathologic evaluation of 517 cases and review of the literature. Obstet Gynecol. 1994; 84(1):22–8.
6. Patel MD, Feldstein VA, Lipson SD, Chen DC, Filly RA. Cystic teratomas of the ovary: diagnostic value of sonography. AJR Am J Roentgenol.1998;171(4):1061-1065.
7. Anteby EY, Ron M, Revel A, Shimonovitz S, Ariel I, Hurwitz A. Germ cell tumors of the ovary arising after dermoid cyst resection: a long term follow up study. Obstet Gynecol.1994; 83(4): 605–8.
8. Sinha RY, Joshi K, Warty NR, Frey B. Morcellation in the bag: the superior solution  to  avoid  spillage. Gynaecol Endosc. 2001; 9:103–6.

Citation

Shah NH,  Shah VN, Paranjpe SH. Rare Case of Bilateral Dermoid Cysts Managed Laparoscopically. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/rare-case-of-bilateral-dermoid-cysts.html

Laparoscopic Management of Right Ovarian Ectopic Pregnancy With Left Ovarian Torsion Following IVF

Author Information

Shah NH*, Bandolkar D**, Jilla M **, Kale KG ***.

(* Hon. Endosopic Surgeon Wadia Hospital and Railway Hospital (Byculla), ** Consulting gynecologist and infertility specialist, *** Fellow Endosopic Surgeon, Surabhi Hospital ,  Andheri, Mumbai, India.)

Abstract

Both ovarian torsion and ovarian ectopic pregnancy are rare entities and can be easily missed unless one has a high degree of suspicion. The incidence of ectopic pregnancies after an in- vitro fertilization (IVF) procedure is 3 to 5 times higher than that of the general population and is reported to be in the range of 1 - 5 %.  We present here a case of a 35 year old female who underwent an IVF procedure following which she had an ovarian ectopic in the right ovary and a left bulky ovary which underwent torsion at the same time. Both these complications were picked up early and successfully tackled laparoscopically conservatively. 

Introduction  

Despite the fact that the incidence of ectopic pregnancy is increasing, occurrence of ovarian pregnancy following in vitro fertilization (IVF) and embryo transfer (ET) is uncommon. Ovarian ectopic contributes to 0.5-3% of all ectopic pregnancies[1] while the incidence of ovarian ectopic following an IVF procedure has been reported to be 0.3 %.[2] Torsion of a stimulated unilateral ovary occurring after IVF is an unusual event.[3] Ovarian torsion should be suspected in all females undergoing ovulation induction for IUI or IVF, who present with severe abdominal pain. Delay in diagnosis and management may lead to necrosis of ovary.

Case Report

The patient was 35 year old with primary infertility of 5 years. She had regular cycles with normal hormonal profile. Her husband had mild oligo-asthenozoospermia. She had five attempts at ovulation induction with intrauterine insemination which were unfruitful, after which controlled ovarian stimulation with IVF was planned. After luteal phase down regulation with leuprolide acetate, stimulation with recombinant follicle stimulating hormone was done. Oocyte retrieval was carried out on day 14 of cycle. Right ovary was 7 × 7 cm and left ovary was 6 × 7 cm. She had moderate ovarian hyperstimulation syndrome (OHSS) managed conservatively with intravenous albumin, fluids and high-protein diet. 

On day 15 after ET, her β hCG was 626.8 IU/l and on day 18, it increased to 4782.4 IU/l. A transvaginal scan showed a thickened endometrium of 14 mm with multiple hemorrhagic small cysts in the right adnexa and few follicles in the left ovary. No intra- or extrauterine gestational sac could be demonstrated. Patient was kept under close observation for 24 hours as she had no complaints and the rise in β hCG appeared adequate, if the possibility of multiple gestations were considered. 

On day 18 after ET, she presented with severe right sided abdominal pain with increasing severity in next 1 hour. On examination, she had tachycardia 110/min, blood pressure of 100/70, respiratory rate of 22/min. She had abdominal distension, severe guarding and tenderness and abdominal girth 100 cm. Ultrasound was done which suggested enlarged bilateral ovaries: right ovary 8 × 10 cm and left ovary 8 × 7 cm with multiple cysts, moderate fluid in the pelvis; intra uterine gestation sac was not visualized.

Patient underwent an emergency laparoscopy and the findings were as follows: there was collection of blood and blood clots, approximately 150 ml, in the peritoneal cavity. Right ovary was bulky and stuck to the right ovarian fossa. An ectopic pregnancy was seen, which was bleeding after separating from the ovarian fossa. The ovary was held with grasper forceps and a wedge resection of the ovary along with the ectopic pregnancy was done using harmonic scalpel. Remaining ovarian tissue was observed and hemostasis confirmed. 

The left ovary was bulky and had one twist over the pedicle. The ovary was not yet gangrenous and hence only de-twisting was done. Histopathological examination confirmed an ovarian pregnancy. Patient was uneventful postoperatively and is planned for IVF again.

Figure 1. Right ovarian ectopic.

Figure 2. Final view after left ovarian detorsion and right ovarian wedge resection.

Discussion 

The presence of two different rare pathologies, i.e. ovarian ectopic pregnancy on one side and ovarian torsion on the other side, presenting at the same time makes this case an unusual one. The ovarian ectopic could be because of reverse migration of the embryo due to deep deposition of the embryo into the uterus.[4] It is suggested that an ovarian ectopic may also be the result of using a large volume of culture fluid during the transfer.[5] Other factors predisposing an ovarian ectopic include pelvic inflammatory disease and tubal pathologies. 

Ovarian torsion is defined as "partial or complete rotation of the ovarian vascular pedicle which causes obstruction to venous outflow and arterial inflow".[6] Torsion after stimulation of ovary is rarer. Ovarian torsion can occur in females at any age but usually pregnancy and infertility treatment predisposes its occurrence. Symptoms are nonspecific and they classically present as sharp localized pelvic pain and tenderness with a palpable mass with peritoneal signs. Conventional USG combined with Doppler is the gold standard for imaging of lower abdominal pain in females.[7] But since in our patient showed signs of an emergency, we could not send her for an ultrasound.

During laparoscopy we could identify easily the ovarian ectopic and the twisted ovarian pedicle of the other side. Although the twisting had occurred, there were no signs of necrosis and de-twisting was done. As selective excision of the gestational sac in the ovary is the treatment of choice,[8] we did a wedge resection of the ovary including the ectopic pregnancy. Hemostasis was achieved successfully.

There are some reports showing the use of systemic methotrexate for an ovarian ectopic. Successful treatment with 50 mg methotrexate injected directly into the ectopic sac of ovarian pregnancy at laparoscopy has also been reported.[9] Because of the limited reports available, in contrast to tubal pregnancies, the factors which predict success of methotrexate in management of ovarian ectopic pregnancy are not well defined.

It is our belief that laparoscopic management is the standard for diagnosis as well as treatment of ovarian pregnancy and/or ovarian torsion.

References  

1. Raziel A, Golan A, Pansky M. Ron-El R, Bukovsky I, Caspi E. Ovarian pregnancy: A report of twenty cases in one institution. Am J Obstet Gynecol. 1990;163:1182–5.
2. Marcus SF, Brinsden PR. Analysis of the incidence and risk factors associated with ectopic pregnancy following in vitro fertilization and embryo transfer. Hum Reprod. 1995;10(1):199-203.
3. Chew S, Ng SC. Laparoscopic treatment of a twisted hyperstimulated ovary after IVF. Singapore Med J. 2001;42(5): 228–9.
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Citation

Shah NH, Bandolkar D, Jilla M, Kale KG. Laparoscopic Management of Right Ovarian Ectopic Pregnancy With Left Ovarian Torsion Following IVF. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/laparoscopic-management-of-right.html

A Rare Case Report Of Cystic Struma Ovarii With Co-Existing Serous Cystadenoma

Author Information

Rojekar AV *, Fernandes GC **.

(* Assistant Professor, ** Associate Professor, Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India)

Abstract 

Struma ovarii is a very unique and rare form of mature teratoma constituting about 3% of all teratomas. Struma ovarii concurrently occurring with other ovarian epithelial tumors has been rarely reported. Concomitant struma ovarii and serous cystadenoma has been mentioned in only two case reports in the extensively searched medical literature. We report a rare occurrence of coexisting struma ovarii along with a serous cystadenoma in a 55 year old woman.

Introduction

Struma ovarii is a very rare but most common and specialized form of monodermal teratoma. Boettlin, in 1889, was the first to report and describe this entity, after observing the presence of thyroid follicular tissue in ovary. The term struma ovarii is reserved for only those monodermal teratomas when the proportion of thyroid tissue present must be more than 50% of the overall tissue. [1,2,3] Coexistence of this rare variant with surface epithelial tumors is very rare. To the best of our knowledge only two cases of concomitant struma ovarii and serous cystadenoma have been reported in English literature [4,5]. Here we present a rare case of concomitant cystic struma ovarii with serous cystadenoma.

Case Report 

A 55 year old post menopausal female presented with lower abdominal lump since two months. There was no history of pain or bleeding or any other systemic complaints. On per abdominal examination, there was a mass of 20 - 22 week size in pelvis. Abdomen and pelvic ultrasonography showed right sided cystic ovarian mass possibly ovarian teratoma. Intraoperatively right sided cystic ovarian mass (10 x 8 cm) was found with stretched fallopian tube of the same side. Left sided ovary, fallopian tube and uterus cervix were unremarkable. She underwent total abdominal hysterectomy with bilateral salphingo-ophorectomy. 

Gross examination of tumor revealed right sided unilocular ovarian cyst with smooth external surface, which measured 8.5 x 6.5 cm. Cut surface showed multiple cystic nodules ranging from 0.5 to 2.5 cm filled with greenish brown gelatinous material resembling colloid filled thyroid tissue. Approximately 40 % of the cyst wall was smooth, wrinkled and grayish white in color. No areas of papillary projections, solid areas, hairs, bone or cartilage were seen. 

Multiple sections from ovarian cyst were studied. The cystic wall with multiple nodules showed predominantly colloid filled follicles of varying sizes (> 50%) lined by benign thyroid follicular epithelium. Sections from the flat wrinkled areas showed a cyst wall of fibro-collagenous tissue lined by low columnar epithelium with morphology of serous cystadenoma. No other element of mature cystic teratoma or any other malignant tissue was recognized.

Figure 1. Multiple colloid filled follicles of varying sizes. 

Figure 2. Colloid filled follicles are lined by benign thyroid follicular epithelium. 

Figure 3. The cyst wall of fibro-collagenous tissue lined by low columnar epithelium. 

Bilateral fallopian tubes, contra-lateral ovary and uterus with cervix showed normal histology. Immunohistochemistry with TTF-1 (Thyroid Transcription Factor – 1) was done and showed strong positivity in follicular epithelium while the areas with appearance of serous cystadenoma were absolutely negative Hence the diagnosis of concomitant cystic struma ovarii with serous cystadenoma has been confirmed.  

Figure 4. TTF-1 is negative in follicular lining epithelium.

Discussion

Very rare occurrence of concomitant struma ovarii with serous cystadenoma has been reported only in two case reports. Struma ovarii is defined as a monodermal highly specialized mature teratoma composed either exclusively or predominantly of thyroid tissue or in which thyroid tissue can be recognized macroscopically. They may have features similar to thyroid adenoma or carcinoma (malignant struma ovarii) or both.[1,2,3]

Struma ovarii accounts for 0.3–1% of all ovarian tumors and for 3 % of all mature teratomas. Approximately 5-15 % of mature cystic teratomas show variable proportion of thyroid tissue; however proportion of thyroid should be more than 50 % to classify it as struma ovarii.[1,2,3] Struma ovarii have also been reported with coexisting non-germinal epithelial tumors such as mucinous cystadenoma, Brenner’s tumor, and serous cystadenofibroma.[6,7]   

Struma ovarii is commonly seen in older patients than for those with common mature teratomas.[8] Although these patients may experience variety of symptoms similar to all other ovarian tumors, abdominal pain and a palpable lump are the most common symptoms observed. Rarely vaginal bleeding, ascitis (up to 33% cases) and pseudo Meig's syndrome (< 10 cases) are also reported in the literature. [1,2,8] The patient described in this report presented with abdominal mass which is the most common symptom and was 55 years old. 

Macroscopically, typically the tumor is brown or green-brown, predominantly solid and gelatinous. A presence of a green to brown glairy fluid is a clue to the diagnosis.

Cystic struma ovarii is very rare.[1,2,8] Recently Szyfelbein, Young and Scully described a series of 20 cases of cystic struma ovarii. Cystic variety of struma ovarii is extremely rare, which may make its identification difficult.[9] In our case, typical morphology and absence of TTF-1 in lining serous epithelial cells in contrast to positivity in follicular epithelium confirmed the diagnosis.  

Till now only two cases have been reported of coexisting struma ovarii with serous cystadenoma in the English literature. In both these cases, it was unilateral and confirmed by IHC. Etiopathogenesis of this pathologic process has not been clear; metaplasia of serous epithelium can be the possible explanation.[4,5] 

Usually non-functional struma ovarii may present with symptoms and signs of hyperthyroidism, due to autonomous activation of the thyroid tissue, seen in only 8 % of patients. Struma ovarii may demonstrate all pathologic patterns that are seen in the thyroid, including malignancy in 5 -10% of cases in the form of papillary and follicular carcinoma.[1,2,3,10,11]

Conclusion

Knowledge of these rarely associated coexisting pathologies and extensive thorough sampling of cyst along with IHC is required for the accurate diagnosis in this otherwise benign condition as they are difficult to diagnose before surgical intervention.   

References

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9. Szyfelbein WM, Young RH, Scully RE. Struma ovarii simulating ovarian tumors of other types. A report of 30 cases. Am J Surg Pathol 1995;19(1):21-9. 
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Citation

Rojekar AV, Fernandes GC. A Rare Case Report Of Cystic Struma Ovarii With Co-Existing Serous Cystadenoma. JPGO 2015. Volume 3 No. 5. Available from: http://www.jpgo.org/2016/05/a-rare-case-report-of-cystic-struma.html