Volume 3 Issue 6, June 2016

Editorial
Chauhan AR

Large Broad Ligament Leiomyoma
Chaudhari HK,  Parulekar SV.

Unilateral Segmental Absence Of Fallopian Tube
Pednekar R, Parulekar SV.

Colporrhexis During Cesarean Section
Shukla M, Shende D, Mayadeo NM.

Laparoscopic Excision Of The Functional Noncommunicating Horn Of The Unicornuate Uterus In a 15 Years Old Postmenarcheal Adolescent Girl
Shah NH, Khadkikar R, Jethwani L, Sahu S.

OHSS Versus Hyperreactio Luteinalis: A Diagnostic Dilemma
Madhva Prasad S, Gupta AS.

Epidermolysis Bullosa In The Newborn –a Rare Clinical Entity
Singhal N, Qureshi S, Gupta AS.

Vaginal Varix in Pregnancy- A Therapeutic Dilemma
Dharmadhikari M, Samant PY, Pai K.

Successful reproductive outcome in a young female following a mixed germ cell tumor
Satia MN, Madhavi J, More V.

Myomectomy during Cesarean Section
Tiwari P, Chauhan AR.

Editorial

Chauhan AR

Fibroids in pregnancy are increasingly common due to factors like delayed childbearing and advances in infertility treatment, and are more often diagnosed due to better imaging techniques.  They may remain asymptomatic, or complicate pregnancy in any trimester; miscarriage, increase in size of fibroid, pain due to red degeneration, malpresentation, preterm labor, obstructed labor and postpartum hemorrhage (PPH) may occur in 10 - 30 % of these patients. The incidence of cesarean section (CS) in patients with fibroids is as high as 73 %, however surgical removal of the myoma has traditionally been discouraged due to the high risk of PPH and postoperative morbidity. Cesarean myomectomy (CM) is the term used to describe myomectomy at cesarean section. Previously CM was resorted to only when the fibroid was obstructing the lower uterine segment or line of incision, or inadvertent entry into the fibroid when the placenta was implanted over it, or when closure of the uterus was not possible without its removal. This article examines recent literature on the safety of CM.

The safety and efficacy of CM was evaluated by Hassiakos in 47 women who underwent simultaneous CS and myomectomy, compared with 94 women with fibroids who underwent only CS. He found that myomectomy added only 15 minutes to the operative time, no patients required blood, no patient required hysterectomy, and length of hospital stay was similar. Pre- and postoperative hemoglobin fall was statistically significant but did not differ among the two groups. He concluded that despite a traditional reluctance to do two procedures simultaneously, myomectomy at CS could be recommended.

Similar findings have been shown by Park, where he retrospectively studied 97 women who had CM as compared to 60 women with fibroids who had only CS. There were no differences in intra or postoperative morbidity; when the size of the fibroid was more than 6 cm, the operative time was longer in the CM group. Kwon studied the safety of CM in large myomas and found no differences in operative outcomes in patients with myomas > 5 cm as compared to those with smaller ones.

In a meta- analysis of available data, Song et al reviewed 9 case - control studies which included more than 1000 women with fibroids, of whom 41 % underwent CM and 59 % underwent CS alone. They found no major differences in safety parameters like intra-operative blood loss, need for transfusion, surgical time or postoperative morbidity. However they concluded that though CM is a reasonable option for some women, no definite conclusions can be drawn as the data was of low quality.  

Principles of myomectomy that are routinely followed apply for CM as well: intracapsular myomectomy, sharp dissection or use of electrocautery, careful attention to hemostasis, obliteration of dead spaces to prevent postoperative hematoma in the myoma bed, good approximation of the myometrium with 2 or 3 layered closure with delayed absorbable sutures. Additional techniques to prevent blood loss like stepwise devascularization and preoperative placement of balloons in the uterine arteries have also been described.

Two interesting recent articles in 2015 looked at surgical decision -making. Sparic analyzed the  intraoperative decision to perform CM in 102 of 185 women with myomas who underwent CS, and found that CM was mostly performed in younger women, and usually by experienced surgeons. The decision was based more on type and location of the fibroid rather than number or size; myomectomy was done more often when the fibroids were subserous or pedunculated.  Topcu in his retrospective series of 76 women who underwent CM compared to 60 women who had CS alone, also found that surgeons were more likely to remove subserous fibroids and that size of the myoma did not affect the decision process. Both authors concluded that CM is a safe procedure.  

Turgal evaluated postoperative adhesion formation between uterus and omentum, adnexal adhesions, incision site adhesions and adhesions causing surgical difficulty in women who had previously undergone CM for small subserosal, pedunculated or intramural fibroids, and were undergoing repeat CS 1 to 5 years later. He found no statistical difference in the adhesions between these patients and controls who had not undergone myomectomy during their previous CS.   

So, is it time to rethink CM in low - resource settings? In a recent 2013 review of CM in Africa, Awoleke supports the argument for CM citing that it could eliminate multiple surgeries for both indications. He emphasises that careful selection of the patient, thorough preoperative counselling especially when patient requests removal of previously diagnosed fibroids at CS, an experienced surgeon and facilities for postoperative management, will increase the safety of the procedure. However he cautions that large RCTs are required before any recommendations. Similarly, Mumtaz from Kerala and Ramesh Kumar from Karnataka have published their experience of CM in 26 and 21 cases respectively with encouraging results. Both authors cite potential advantages of CM as: avoiding a second surgical procedure or "interval myomectomy" with its attendant risks of anesthesia and surgical difficulties due to previous CS, better obstetric outcome in subsequent pregnancies as known complications of pregnancy with fibroids will be negated, increased chances of VBAC in subsequent pregnancies, and cost saving by combining two surgeries. 

Sir Victor Bonney, the father of myomectomy, cautioned the bold surgeon against a "misguided policy" of combining CS with myomectomy. Even today, only a small number of surgeons practice CM and most of the published data is a retrospective analysis of cases; more robust data and larger studies are needed before advocating CM as standard practice. This issue of the journal carries two vastly different reports of fibroid in pregnancy which we hope will be of interest to our readers. 

Large Broad Ligament Leiomyoma

Author Information

Chaudhari HK *,  Parulekar SV**. 

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

Abstract

The broad ligament is the commonest extrauterine site for the occurance of a leiomyoma. We report a case of a 30 year old woman with a 26 weeks’ size broad ligament leiomyoma. At laparotomy it was found to be multilobulated, measuring 33x19x10 cm  and weighing 3200 g.   

Introduction

Leiomyoma is the most common pelvic tumor of the uterus. The broad ligament is the commonest extrauterine site for the occurrence of leiomyoma.[1] Extrauterine leiomyomas may develop in the broad ligament or at other sites where smooth muscle exists.[1,2] Common symptoms of leiomyomas include menstrual disturbances, dysmenorrhea, and symptoms related to pressure caused by the mass.[3] Incidence of broad ligament fibroid is less than 1%.[4]

Case Report

A 30 year old woman, para 1 living 0, presented with a chief complaint of pain in abdomen for3 days. She had no history of any bladder or bowel complaints. Her menstrual cycles were regular with moderate flow and mild pain. She had a normal delivery 12 years ago. Her general and systemic examination revealed no abnormality. Abdominal examination showed a soft, nontender mass of 26 weeks’ size arising from the pelvis. On speculum examination the cervix was high up in position. The anterior cervical lip was effaced out. A mass was arising from the posterior lip of cervix measuring 7 cm in length, and 5 cm in diameter, projecting downward into the vagina. It was continuous with the abdominal mass which had also filled the pouch of Douglas. Abdominopelvic ultrasonography showed a  large heterogenous lesion of 20x18 cm in the uterine fundus, suggestive of a leiomyoma. A computed tomography of the abdomen and pelvis showed the presence of multiple leiomyomas in the uterine fundus and body of the uterus, ranging in size from 8.3 to 11 cm (figure 1). Excretory urography showed a lateral displacement of the  left ureter and normal functioning kidneys. There was no hydronephrosis. Her hemogram showed hemoglobin to be 9.5 g/dl. Her urinalysis, plasma sugars, renal and liver function tests, chest radiograph and electrocardiogram showed no abnormality. The patient opted to undergo a myomectomy. One unit of packed red cells was transfused. At laparotomy a multilobulated  mass measuring 33x19x10 cm was seen in the left broad ligament, arising from the myometrium on the left side (figure 2). The left fallopian tube, left ovary and left infundibulopelvic ligament were stretched over its upper surface. The mass extended laterally into the iliac fossa, and posteriorly behind the cervix occupying pouch of Douglas. The sigmoid colon was adherent to the broad ligament covering the leiomyoma laterally. The uterus was normal size, elevated and displaced to the right side. The right tube and ovary were normal. The left broad ligament was opened by dividing the left round ligament between clamps. The pseudocapsule of the leiomyoma was divided on its upper surface and the leiomyoma was enucleated by sharp and blunt dissection. The sigmoid colon got separated from the mass without any harm when the pseudocapsule and overlying broad ligament were separated from the mass. The left fallopian tube and the left infundibulopelvic ligament were slipped posteriorly off the surface of the mass. The leiomyoma was found to be passing to the right side of the uterus behind the cervix, and inferiorly. It was pushed to the left during dissection, and when it was removed, it was found to include the part of the mass that had presented vaginally. There was no need to push the vaginal mass upward during the dissection. The left ureter was found to be below and lateral to the mass, unharmed due to dissection entirely within the pseudocapsule of the leiomyoma. The left broad ligament was closed and the divided left round ligament was reconstructed. The surgically resected mass weighed 3.2 kg. Cut surface showed multiple large masses with cystic areas with few areas of hemorrhage. On histopathological examination, a leiomyoma with cystic and hyaline degeneration was seen. The patient made an uneventful recovery.

Figure 1. CT scan of abdomen-pelvis –suggestive of multiple leiomyomas in the fundus and body of uterus.

Figure 2. Intraoperative findings: uterus yellow arrow, left fallopian tube (green arrow), and left ovary (white arrow) stretched over the large left broad ligament leiomyoma.

Figure 3. Gross appearance of broad ligament leiomyoma.

Discussion 

A tumors in the broad ligament sometimes attains a  large size because it is often asymptomatic. It distends the broad ligament so that the fallopian tube is stretched and lies on their upper surface. It sometimes pushes its way upwards stripping the peritonium of the  lateral wall of pelvis and iliac fossa and on left side it may burrow between the layers of the sigmoid mesocolon, the  bowel itself lying upon the tumor. The ureter can be in danger and great care is necessary not to damage this structure during surgery. A pseudo broad ligament leiomyoma arises from the lateral wall of the uterine corpus. Hence the ureter is displaced downward and laterally. A true broad ligament leiomyoma can develop anywhere in relation to the ureter, and hence must be localized before any structure is cut. Preoperative assessment of the location of the leiomyoma with contrast enhance computed tomography or excretory urography is useful for this purpose. Preoperative catheterization of the ureter is also recommended for localizing the ureter during surgery.

Our patient had some unique features. The leiomyoma was unusually large. Though it was arising on the left side, it had grown to the right side behind the supravaginal cervix, so that it appeared to be in the right broad ligament too during laparotomy. It had grown downward too, so that it appeared in the vagina like a polyp, but actually had its surface covered by vagina of the posterior fornix, which had stretched over its surface. The distortion of the normal anatomy was quite unusual, and careful attention to detail helped restoration of normal anatomy after myomectomy and reconstruction.

Conclusion 

A diagnosis of extrauterine fibroid should be considered  in a case of pelvic masses with the normal uterus. During surgery one should be careful about the ureteric course and surrounding organs.

Acknowledgment

We thank Dr Sreshthha Mahanti for taking the intraoperative pictures.

Referances

1. Bakari, F. , Sulayman, H. , Avidime, S. , Ameh, N. and Adesiyun, A. (2015) Huge Broad Ligament Leiomyoma: A Case Report. Case Reports in Clinical Medicine, 4, 55-59. doi: 10.4236/crcm.2015.42013.
2. Kumar P, Malhotra N. Jeffcoate's Principles of Gynaecology. 7th ed. New Delhi: Jaypee Brothers; 2008. Tumors of the corpus uteri; p. 492.
3. Barek JS. Novack's Gynaecology. 15th ed. New Delhi: Lippincott Williams and Wilkins, Wolters Kluwer (India); 2007. Benign diseases of the female reproductive tract; p. 470.
4. Rajput DA, Gedam JK. Broad Ligament Fibroid: A Case Series. IJSS Case Reports & Reviews 2015;1(11):8-11.

Citation

Chaudhari HK,  Parulekar SV. Large Broad Ligament Leiomyoma. JPGO 2015. Volume 3 No. 6. Available from: http://www.jpgo.org/2016/06/large-broad-ligament-leiomyoma.html

Unilateral Segmental Absence Of Fallopian Tube

Author Information

Pednekar R*, Parulekar SV**.
(* Assistant Professor, ** Professor and Head, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India.)

Abstract

This paper presents an incidental finding of segmental absence of mid portion of left fallopian tube in a 32 years old multigravida during a lower segment cesarean section with tubal ligation. Around 1 cm stump of left fallopian tube seen at the cornu  and a well-defined fimbrial end was seen in the fold of broad ligament laterally. The middle portion of the fallopian tube between these two ends could not be traced and found to be absent. There was no other abnormal finding in the anatomy of uterus, right fallopian tube and ligaments around the uterus. There are very few cases reported in a literature. The possible etiology of the condition is discussed here.

Introduction

Fallopian tube is a 8 to 14 cm long tubular structure extending from uterine cornu on each side, into the broad ligament, which is then called mesosalpinx. The fallopian tube is divided into an interstitial portion, isthmus, ampulla, and infundibulum or fimbrial extremity. The embryological development of the fallopian tube is from paramesonephric duct or mullerian duct. Unilateral partial or complete absence of fallopian tube is a rare condition. It may be congenital or acquired. One such case is presented here and its pathogenesis is discussed.

Case Report

A 32 years old woman, married for 13 years, gravida 4, para 3, living 3, previous all full term normal vaginal deliveries, rpesented at 39 weeks of gestation in labor. Her past medical and surgical history was not contributory. Her past menstrual cycles were regular. There was no history suggestive of pelvic inflammatory disease or endometriosis. There had been no episode of acute pelvic pain in the past, which had settled on its own. She had not undergone any pelvic surgery in the past.  Her general and systemic examination was normal. Abdominal examination showed a full term uterus with single longitudinal lie in vertex presentation, with uterine activity, and normal fetal heart sounds. On per vaginal examination the cervix was 2 cm dilated and 40% effaced. The station was - 4. She subsequently required an emergency lower segment cesarean section for fetal distress. She also wanted a sterilization operation. It was found that the uterus and the right fallopian tube were normal, while the middle portion of the left fallopian tube was absent. There was a 1 cm long stump of the tube at the cornu and well defined infundibulum and fimbrial end (figure 1). The two parts were connected by a free fold of the mesosalpinx. Pomeroy’s sterilization operation was done on the right fallopian tube, while the lateral end of the left tubal stump was cut and ligated along with fimbriectomy after the cesarean section. The patient made an uneventful recovery.

Figure 1. Intraoperative view of left fallopian tube, showing absent medial portion (yellow line), cornual 1 cm of stump (white arrows), infundibulum and fimbrial end (black arrows).

Discussion

Structural abnormalities of the fallopian tube like partial or complete absence are very rare.[1,2,3] Other congenital anomalies of the fallopian tube include complete absence, duplication, multiple lumina, accessory ostia , or segmental deletion.[4,5,6,7] Nawroth et al reported only 18 patients with partial atresia of the fallopian tubesinn a comprehensive survey of the literatue in 2006.[4] Segmental atresia could occur unilaterally or bilaterally, but most cases (14/18) reported displayed unilateral; partial tubal atresia. Several cases of partial tubal atresia (4/18) were associated with the uterine anomalies of bicornuate or unicornuate uterus.
This rare condition may be due to congenital malformation or torsion. Congenital absence is frequently associated with developmental alteration of mesonephric and paramesonephric ducts. The mesonephric ducts appear in the embryo at approximately 5 weeks, while the paramesonephric ducts, located do so at 7 weeks, lateral to mesonephric ducts. The paramesonephric duct grows caudally, crosses the mesonephric duct and fuses with the paramesonephric duct of the other side. The upper and the middle segments of paramesonephric duct form the fallopian tube.  An absence of a uterine horn, fallopian tube, kidney and ureter on the affected side is seen with combined congenital absence of mesonephric or paramesonephric ducts. Partial defects of a fallopian tube are due to a failure of development of a paramesonephric duct, possibly due to inadequate blood supply.[7,8]
Torsion of the uterine adnexa is not uncommon condition that causes acute pelvic pain. Symptoms may, however be mild or even absent, especially when occurring during pregnancy.[7] Torsion of the fallopian tube with /or without that of the ovary may occur in utero or in childhood.[9]  Asymptomatic torsion consequently leads to avascular necrosis and resorption of the tissue (fallopian tube and/or ovary).[7,10]  Isolated torsion of the fallopian tube is reported to occur in one in 1.5 million women.[11] It may be due to excessive length or spiral course of fallopian tube, hematosalpinx, neoplasm, or long mesosalpinx, and extrinsic causes like a paraovarian cyst, uterine enlargement by pregnancy or neoplasm, adhesion, pelvic congestion leading to spiraling of the veins of the mesosalpinx, and hypermobility of the fallopian tube.[13] For most of the cases reported to date, the true etiologies of partial absence of the fallopian tube remain unclear. If there are other anomalies of the genital tract or the urinary system, the etiology is likely to be congenital. Absence of other anomalies suggests torsion as the cause. If the uterus and the other fallopian tube are normal, the fertility of the woman is unlikely to be affected. In our patient, there was neither a history of acute pelvic pain or past surgery. But there were no other anomalies too. Thus the cause of partial absence of her fallopian tube cannot be stated with certainty, though it may have been due to asymptomatic torsion of the adnexa.

References

1. Semmens JP. Congenital anomalies of the female genital tract. Functional classification based on review of 56 personal cases and 500 reported cases. Obstet Gynecol, 19 : 328-350, 1962.
2. Woolf RB, Allen WM. Concomitant malformations : the frequent simultaneous occurrence of congenital malformations of the reproductive and urinary tracts. Obstet Gynecol, 2 :236-265, 1953. 
3. Yazawa H, Yabe M, Endo S, Hayashi S. A case of congenital unilateral partial absence of fallopian tube. Fukushima J Med Sci. 2010 Jun;56(1):44-9.
4. Nawroth F, Nugent W, Ludwig M. Congenital partial atresia of fallopian tube. Reprod Biomed Online, 12 : 205-208, 2006.
5. Beyth Y, Kopolovic J. Accessory tubes, a possible contributing factor in fertility. Fertile Sterile, 38 : 382-383, 1982.
6. Daw E. Duplication of uterine tube. Obstet Gynecol, 42 :137-138, 1973.
7. Eustace DL. Congenital absence of fallopian tube and ovary. Eur J Obstet Gynecol Reprod Biol, 46 : 157-159, 1992.
8. Paternoster DM, Costantini W, Uglietti A, Vasile C, Bocconi L. Congenital or torsion-induced absence of fallopian tubes. Two case reports. Minerva Ginecol. 1998 May;50(5):191-4.
9. Dresler S. Antenatal torsion of a normal ovary and  fallopian tube. Am J Dis Child, 131 : 236, 1977
10. Uckuyu A, Ozcimen EE, Ciftci FCS. Unilateral congenital ovarian absence with twisted tube. J Minim Invasive Gynecol, 16 : 126-127, 2009.
11. Hansen OH. Isolated torsion of fallopian tube.  Acta Obstet Gynecol Scand, 49 : 3-6, 1970.
12. Sankaran S, Shahid A, Odejinmi F. Autoamputation of the fallopian tube after chronic adnexal torsion. J Minim Invasive Gynecol, 16 : 219-221, 2009.
13. Antoniou N, Varras M, Akilivis C, Kitsiou E, Stefanki S, Salanalekis E. Isolated torsion of the fallopian tube : a case report and review of the literature. Clin Exp Obstet Gynecol, 31 : 235-238, 2004.

Citation

Pednekar R, Parulekar SV. Unilateral Segmental Absence Of Fallopian Tube. JPGO 2015. Volume 3 No. 6. Available from: http://www.jpgo.org/2016/06/unilateral-segmental-absence-of.html

Colporrhexis During Cesarean Section

Author information

Shukla M *, Shende D **, Mayadeo NM ***.

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

Abstract

Uterine leiomyoma are the most common pelvic tumors in women of reproductive age. Pregnancy can take place in spite of the presence of fibroids and can continue uneventfully. However, when the fibroids are multiple, large or if they are located in the lower uterine segment or cervix, they can adversely affect the obstetric outcome as described in our case of colporrhexis during cesarean section. The objective of this case report is to highlight the importance of surgical preparedness in multiple uterine fibroids with malpresentation of fetus.

Introduction

Incidence of uterine fibroids is 20 - 25% and 2% of pregnant women have fibroids.[1,2] Most fibroids are asymptomatic but complications may be found in 10 - 30% of women with uterine fibroids.[3] Severe localized abdominal pain may occur if a fibroid undergoes "red degeneration", torsion (most commonly seen with a pedunculated subserosal fibroid), or impaction.[4] Uterine fibroids are associated with increase rate of spontaneous miscarriage, preterm delivery, placental abruption, malpresentation, labor dystocia, cesarean delivery, postpartum haemorrhage, obstetric hysterectomy and rarely colporrhexis.[5,6]

Case Report

A 32 year old primigravida with 38 weeks of gestational age was taken for elective cesarean section in view of multiple uterine fibroids with fetus in transverse lie. On clinical examination uterus was 36 weeks size with a left lateral fibroid measuring 7 x 6 cm. Fetus was in transverse lie with breech felt in right flank. On per speculum examination, vagina was healthy and cervix could not be seen. On per vaginum examination, fetal parts could not be felt and 7 x 7 cm posterior lower uterine segment fibroid extending to cervix was felt. Ultrasound suggested multiple fibroids with fundal fibroid measuring 73 x 71 mm and lower segment posterior fibroid measuring 52 x 50 mm. 

Intra-operative findings were multiple uterine fibroids with levorotated uterus. Levorotation was corrected and incision was taken on lower uterine segment. The uterine incision was inadvertently extended towards right lateral fornix and posterior vaginal wall. Baby delivery was difficult due to both left lateral fibroid and head on that side; and was delivered by breech extraction. While trying to look for uterine angles and lower edge of incision, it was realised that cervix was avulsed and incision was at posterior vagina. Hence colporrhexis was immediately diagnosed. With the help of senior obstetrician, right-sided uterine incision was sutured with polyglactin no.1 in continuous interlocking fashion. Avulsed cervix was sutured with polyglactin no. 2-0 in simple interrupted fashion. Bleeding was promptly controlled with uterotonics and patient received one unit blood transfusion. Post-operative course was uneventful, mother and baby went home on day 7 of cesarean section.

Figure 1. Colporrhexis diagnosed intraoperatively. Avulsed cervix can be seen through the uterine incision.

Figure 2. Avulsed cervix sutured with Vicryl 2-0 with simple interrupted sutures.

Figure 3. Uterus after suturing of colporrhexis and uterine incision.

Discussion

Colporrhexis is a laceration causing separation of the cervix from the vaginal fornix.[7] It can be primary or secondary. Primary colporrhexis is a vaginal vault tear which is not associated with cervical or uterine extension while secondary colporrhexis cannot be differentiated from a rupture originating in the uterus and then extending to involve the vagina.[8] Incomplete colporrhexis involves rupture of the vaginal epithelium and the muscularis, whereas complete rupture includes the overlying peritoneum as well.[8] Colporrhexis is known to occur both in non-pregnant and pregnant state. In non-pregnant state, forceful coitus and foreign bodies are known to cause such injuries. These are more common in an already weakened vagina because of postmenopausal atrophy and previous surgery. Those related to pregnancy mostly occur during labor, spontaneously or due to trauma.[9,10] The traumatic varieties are usually the result of complications of forceps or manual removal of placenta.[10] Our case fits the description of secondary traumatic complete colporrhexis. The cause of colporrhexis in this case can be inferred to be the multiple fibroids, which distorted the uterine anatomy and led to levorotation.

Conclusion

Localization of the fibroids and fetal lie in a pregnant uterus are the key steps in deciding the mode of delivery. Also, fetal malpresentation should be expected in a case of multiple uterine fibroids. During cesarean section, it is important to restore the uterus to its normal anatomy, by correcting any rotation, before taking an incision on the uterus. The diagnosis of low-lying placenta and multiple uterine fibroids enabled us to anticipate blood loss. Blood and blood  products were thus kept ready for this patient and helped save precious time when the patient had postpartum hemorrhage. Iatrogenic complications, such as this case, can occur with anybody, especially with young surgeons. However the important learning point is to diagnose such complications promptly and to immediately call for help.

References

1. Katz VL, Dotters DJ, Droegemeuller W. Complications of uterine leiomyomas in pregnancy. Obstet Gynecol. 1989;73(4): 593–6.
2. Wallach EE, Vu KK. Myomata uteri and infertility. Obstet Gynecol Clin North Am. 1995; 22(4):791–9.
3. Klatsky PC, Tran ND, Caughey AB, Fujimoto VY. Fibroids and reproductive outcomes: a systematic literature review from conception to delivery. Am J Obstet Gynecol. 2008; 198(4): 357-66.
4. Lee HJ, Norwitz ER, Shaw J. Contemporary Management of Fibroids in Pregnancy. Reviews in Obstetrics and Gynecology. 2010; 3(1):20-27.
5. Sheiner E, Bashiri A, Levy A, Hershkovitz R, Katz M, Mazor M. Obstetric characteristics and perinatal outcome of pregnancies with uterine leiomyomas. J Reprod Med. 2004; 49(3):182-6. 
6. Sharma KK, Frith KM. Primary complete colporrhexis in primigravida. Br J Obstet Gynaecol 1993; 100(12): 1142-4.
7. Wijesinghe PS. Primary spontaneous complete colporrhexis. Ceylon Medical J. 2002; 47(1): 29.
8. Osterling DL, McCreedy PA, McFee JG, Thompson HE. Primary spontaneous colporrhexis. A case report. Obstet Gynecol. 1971; 38(1): 96-8
9. Brander JH, Buchman MI. Rupture of the vagina during spontaneous delivery. Obstet Gynecol. 1964; 24:151-4
10. Uchil D, Kumakech W, Jolaoso A. Spontaneous vaginal delivery through an unusual posterior cervical tear. J Obstet Gynaecol. 2006; 26(3): 263-4.

Citation

Shukla M, Shende D, Mayadeo NM. Colporrhexis During Cesarean Section. JPGO 2016. Volume 3 Number 6. Available from: http://www.jpgo.org/2016/06/colporrhexis-during-cesarean-section.html

Laparoscopic Excision Of The Functional Noncommunicating Horn Of The Unicornuate Uterus In a 15 Years Old Postmenarcheal Adolescent Girl

Author Information

Shah NH*, Khadkikar R,* Jethwani L**, Sahu S*, 

(* Consultant, ** Senior Resident, Vardann Multispeciality Hospital, Mumbai, India.)

Abstract

A 15-year-old girl came to our outpatient clinic with complaints of lower abdominal pain and dysmenorrhea. She had no past surgical or medical illness. Menarche had occurred two years ago. She suffered from dysmenorrhea since past one and half years and continuous right sided pelvic pain and heaviness since past three to four months. The symptoms were partially relieved by analgesics. Ultrasonography revealed hemorrhagic cyst in the right ovary in a case of suspected bicornuate uterus. Laparoscopic excision of right sided hematosalpinx and non-communicating functional horn with hematometra of the unicornuate uterus was performed under general anesthesia. The postoperative period was uneventful. Laparoscopy results in anatomical outcome equivalent to that offered by a laparotomy approach, and has additional advantages such as; less risk of formation of adhesions, better cosmetic results and early recovery in the postoperative period; which are important for a very young patient.

Introduction

Type and time of presentation of Mullerian anomaly make these cases challenging and management differs from case to case. The incidence of Mullerian anomaly reported is between 0.1- 3.5%.[1] These anomalies develop due to disruption of mullerian fusion during 8th week of intrauterine development. Many patients with this condition are asymptomatic. Of the various mullerian malformations, unicornuate uterus with a rudimentary horn is very uncommon with an incidence of 0.06%.[2] Unicornuate uteri are classified into four groups by the American Society of Reproductive Medicine (ASRM) as: 1. A unicornuate uterus with a communicating rudimentary horn; 2. A unicornuate uterus with a non-communicating rudimentary horn; 3. A non-cavitated unicornuate uterus with a non-communicating rudimentary horn; and 4. An isolated unicornuate uterus. The unicornuate uterus with non-communicating rudimentary horn is the most common type.[3] Complications include endometriosis, primary infertility, hematometra. The symptoms do not appear until menarche and they may be nonspecific; the diagnosis is often made late. Here, we present a case of laparoscopic excision of the rudimentary horn of a unicornuate uterus in a 15 year old postmenarcheal girl.

Case Report

A 15-year-old girl presented with complaints of dysmenorrhea and right-sided lower abdominal pain. She had menarche at 13 years of age. Her menstrual cycle was of 30 days interval, with a moderate amount of bleeding lasting for 3-4 days but with progressively increasing dysmenorrhea. She developed spasmodic right lower abdominal pain and heaviness which increased in severity since last four months. The pain responded only temporarily to the antispasmodic analgesic medications. The patient weighed 39 kg, with blood pressure of 100/60 mm Hg and heart rate of 96 beats per minute. Her respiratory rate was 20 breaths per minute, and body temperature was 37.5°C. Secondary sexual characters were normal with normal external genitalia.  Per abdomen, there was marked tenderness in the right iliac fossa. However, distinct mass could not be palpated.  White blood cell count was 10,020/ mm3 and  hemoglobin concentration was 10.2 g/dl, with a normal platelet count. Ultrasonography of abdomen and pelvis showed hemorrhagic cyst of 7.5x6.3 cm in the right ovary in a case of suspected bicornuate uterus.

Laparoscopy was done under general anesthesia. A unicornuate uterus was noted with a right sided non-communicating rudimentary horn measuring approximately 6x5 cm. A right hematosalpinx was noted measuring 6x3 cm. Right ovary, left fallopian tube and left ovary were normal.

Figure 1. Panoramic view showing unicornuate uterus with rudimentary horn and right hematosalpinx.

Right ureter was traced and was found to be well away from the rudimentary horn and hematosalpinx. First, hematosalpinx was excised with help of bipolar cautery and scissors. Then the same incision was continued transversely to excise the rudimentary horn. The horn contained approximately 50 ml of chocolate like material in its cavity and had no communication with the main uterus. 

Figure 2. After excision of hematosalpinx and rudimentary horn.

The remaining defect in the serosa and part of myometrium of the uterus was sutured in double layer with number1 polyglactin sutures. 

Figure 3.After suturing remaining defect in the uterus.

As the horn was well above the level of the isthmus and uterine vessels, uterovesical fold of peritoneum was not dissected. Hemostasis was confirmed and specimen was retrieved.  Hysteroscopy was not performed as the patient was a virgin.

Postoperative period was uneventful and she was discharged on third day. She was followed up regularly and had significant pain relief after the surgery. Histopathology confirmed hematometra and hematosalpinx.

Discussion 

Unicornuate uterus develops due to failure of development of one of the mullerian ducts and is commonly associated with various degrees of rudimentary horns when one of the ducts develops only partially. It is often associated with renal, spinal or cloacal abnormalities.  Diagnosis of this condition can be made incidentally during surgical procedure because of acute abdomen as seen in our case or during LSCS. Causes of abdominal pain in these patients can be due to distention of the uterus because of accumulated blood in the non-communicating rudimentary horn, pyometra, ruptured ectopic pregnancy in the rudimentary horn and torsion of the hematosalpinx. 

Presence of a cavitatory rudimentary horn especially carries poor prognosis due to frequent occurrence of endometriosis, cornual ectopic pregnancy and spontaneous abortion.[4] Endometriosis is a dreaded complication resulting from retrograde menstruation. It causes severe dysmenorrhea, chronic pelvic pain, dyspareunia and infertility. Three dimensional ultrasonography and MRI can confirm the diagnosis but diagnostic laparoscopy is considered gold standard. Surgical excision of the non-communicating horn should be performed at the earliest as it may contain functional endometrium, to prevent endometriosis and pregnancy complications. Operative laparoscopy is minimally invasive, has lesser morbidity and should be the method of choice in the surgical management of a rudimentary horn.[5]

Conclusion

Secondary dysmenorrhea should be thoroughly evaluated in adolescent girls considering the possibility of mullerian anomaly. Minimal access surgery is the preferred mode of surgical management. Early diagnosis and prompt treatment is of importance to avoid future gynecological and obstetric complications.

References

1. Speroff L. The uterus. In: Speroff L, Fritz MA ed. Clinical Gynecologic Endocrinology and Infertility. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2005:113.
2. Raga F, Bauset C, Remohi J, Bonilla-Musoles F, Simón C, Pellicer A. Reproductive impact of congenital mullerian anomalies. Hum Reprod. 1997;12(10):2277–81.  
3. Heinonen PK. Unicornuate uterus and rudimentary horn. Fertil Steril. 1997;68(2):224–30. 
4. Jayasinghe Y, Rane A, Stalewski H, Grover S.The presentation and early diagnosis of the rudimentary uterine horn. Obstet Gynecol. 2005;105(6):1456–67. 
5. Zapardiel I, Alvarez P, Perez-Medina T, Bajo-Arenas JM. Laparoscopic management of a cavitated noncommunicating rudimentary uterine horn of a unicornuate uterus: a case report. Journal of Medical Case Reports.2010; 4:215.

Citation

Shah NH, Khadkikar R, Jethwani L, Sahu S. Laparoscopic Excision Of The Functional Noncommunicating Horn Of The Unicornuate Uterus In a 15 Years Old Postmenarcheal Adolescent Girl. JPGO 2016. Volume 3 Number 6. Available from: http://www.jpgo.org/2016/06/laparoscopic-excision-of-functional.html

OHSS Versus Hyperreactio Luteinalis: A Diagnostic Dilemma

Author Information

Madhva Prasad S*, Gupta AS**.

(* Assistant Professor, ** Professor, Department of Gynecology and Obstetrics, Seth GS medical college and KEM hospital, Mumbai, India.)

Abstract

Adnexal masses in pregnancy pose a challenging clinical scenario.  Hyperreactio luteinalis and ovarian hyperstimulation syndrome are two such conditions, which cause bilateral ovarian masses. These two entities have been described in literature as conditions in continuum. The diagnostic dilemma between the seemingly similar entities and an attempt to distinguish the two is reported here.

Introduction

Ovarian hyperstimulation syndrome (OHSS) is a condition that is characterized by massive enlargement of ovaries, features of fluid shift and varying effects on the end organ systems. In the mild form, the patient presents with discomfort, pain, nausea, abdominal distension, enlarged ovaries and normal hematological and biochemical profiles.   In its severe form, this can include hydrothorax, marked ascites, liver dysfunction, renal shut down or venous thromboembolism.[1]

Case Report

A 25 year old lady with married life of 2 years, primigravida with a gestational age of 13 weeks and 3 days confirmed by early sonography was admitted with complaints of multiple episodes of vomiting.  She had complaints of abdominal pain, but no complaints of vaginal spotting or bleeding. Patient had been admitted to a different hospital prior where she did not respond to symptomatic management. Apart from irregular menstrual cycles, for which no specific treatment was taken; she did not report any major medical or surgical illnesses in the past. She denied any history of receiving medications for purpose of conception or regularization of menses in the past 6 months. A review of pre-pregnancy investigations showed a scan dated 14 months prior to admission, showing polycystic ovaries with ovarian volumes of 23 cc and 14 cc and also a left para-ovarian cyst measuring 2.5x2.1 cm. However, patient did not give history of any weight gain, irregular menstrual cycles or facial hair. 

Examination showed signs of dehydration, pulse of 120 beats per minute, normal blood pressure, unremarkable cardiovascular and respiratory system examination. Abdominal examination showed a uterus of 14-16 weeks size and closed internal cervical os. Bilateral forniceal fullness was present.  

Ultrasonography confirmed a single live intrauterine gestation of corresponding gestational age and showed bilateral simple ovarian cysts in both ovaries—the ovaries measured 10.5x6.2x5.8 cm and 7x6.2x5.1 cm. The bilateral cysts showed normal vascularity. 

Patient was admitted to the ward and was treated with intravenous fluids, anti-emetics and multi-vitamin infusion. Urine output was monitored and was adequate. Vomiting gradually reduced in frequency and severity.  Investigations showed normal hemoglobin, WBC and platelet count. Liver function tests showed normal levels of protein and albumin. Slightly elevated AST with value of 56 IU/ml normalized in few days. Renal function tests showed elevated blood urea of 44 mg%, elevated creatinine of 2.1 mg%, serum sodium of 126 meq/l and serum potassium levels of around 2.5 meq/l. There was no decrease in urine output, which was maintained strictly.   Daily monitoring of renal function tests showed improvement in blood urea and creatinine and returned to normalcy within 4 days of admission. Electrolyte abnormality persisted for duration of almost 10 days despite treatment with intravenous potassium in normal saline.

β HCG value was 450667 mIU/ml which reduced to 395012 mIU/ml. The upper value of reference range at 13 weeks of gestation being 254,000 mIU/ml. Coagulation profile was normal. Serum TSH was 0.03 IU/ml but Free T3 (normal range 1.4-4.4 pg/ml)  and Free T4 ( normal range 0.84-2.00 ng/dl) were normal and hence no treatment was started. The serum TSH repeated 3 months later showed normal value of 2.15 IU/ml. Ultrasonography was repeated 10 days after admission and showed single live intrauterine gestational age with corresponding gestational age and persistence of multiple large simple cysts with a left ovarian volume of 109 cc and right ovarian volume of 40 cc. 

Upon symptomatic improvement and reduction of vomiting, patient was discharged after 23 days of indoor hospital stay; and advised regular outpatient antenatal visits. Ultrasonography done around 3 months and 5 months (at 34 weeks) after the admission also showed persistence of the simple cysts in the ovary. 

At full term gestation, patient went into spontaneous active labor. LSCS was performed in view of non-progress of labor and a female child of 3.39 kg was delivered uneventfully. Adnexae were inspected and showed bilateral mildly enlarged ovaries with a paraovarian cyst of 1x1 cm on the left side (figure 1). No intervention was done for the cyst. Patient had an uneventful postoperative course. The patient did not have any signs of virilization and the female child was also normal.

Post-delivery, venous blood sample of the patient was collected and sent for FSH receptor analysis. This did not reveal any mutation. 

Figure 1. Intraoperative picture showing ovarian cysts (red arrow) and para-ovarian cyst (yellow arrow).

Discussion

OHSS is predominantly described as an iatrogenic event in the context of controlled ovarian hyperstimulation for management of infertility. Being a possibly life-threatening complication, many classifications have been proposed and many strategies have been developed to reduce the incidence and complications of OHSS.  However, recently it has been recognized that OHSS can occur even without the use of ovulation induction or gonadotropic agents.  This entity is now termed as “spontaneous OHSS” (sOHSS).[2] 

A clinico-radiological algorithm has also been described recently which differentiates the four etiological types of sOHSS (Type I-- FSH receptor mutation; Type II – elevated β HCG due to twins/ molar pregnancy; Type III—hypothyroidism; Type IV- FSH/LH secreting adenomas).[3] 

Our patient had abdominal pain, vomiting, tachycardia, elevated creatinine, hyponatremia, hypokalemia, multiple bilateral ovarian cysts, elevated β HCG and mildly elevated liver enzymes. The absence of history of ovulation induction allows a diagnosis of sOHSS, and clinical criteria match that of mild OHSS. All of these clinical parameters improved with conservative management including intravenous hydration and directed symptomatic management, which is in line with the guidelines available.[1]

A close differential diagnosis of such a clinical condition is hyperreactio luteinalis (HL). The condition is characterized by multiple cysts in the ovary (theca lutein cysts), transient hyperthyroidism, elevated β HCG, ascites and virilization of the mother and fetus, if female. In the past aggressive management including exploratory laparotomy with cystectomy or ovarian removal were in vogue due to possibility of coexisting malignant pathology.  However, significant long term sequelae on the mother or the fetus have not been described. A recent review also suggests that conservative management is most preferred, for the condition.[4]

Multiple reports have appeared in the past, which have suggested the similarity of the two conditions, but the need to distinguish the two. Foulk et al had reported a case which details about the need to differentiate the two conditions. The distinction was made on the fact that there were no features of virilization and conservative management was followed.  No other specific effort was made to distinctly characterize the conditions.[5] Coccia et al have described hyperreactio luteinalis occurring in a woman with preexisting renal failure, hyperthyroidism and with twin pregnancy, but the distinction was not clearly made.[6] Gherman et al reported a case of intractable hyperemesis and transient hyperthyroidism ultimately resulting in a small for gestation age fetus. Interestingly our patient also had severe hyperemesis and transient hyperthyroidism initially, but IUGR was not present.[7] In their two-case comparison between sOHSS and hyperreactio luteinalis, Suzuki et al opined that the distinction between the conditions is difficult.[8] Haimov-Kochman in 2004 suggested that sOHSS and HL are entities in a continuum.[9]  

Clearly, our case represents the diagnostic dilemma between spontaneous OHSS and Hyperreactio luteinalis. Since the patient does fit into sOHSS, it was reviewed if the patient fits into any of the types of sOHSS etiologically (as mentioned above)[3]. It was a singleton pregnancy and there were no features of gestational trophoblastic disease, so type II is not considered. Our patient had transient hyperthyroidism and a later euthyroid state for the duration of pregnancy, and hence is not type III. Since the patient did not have any neurological or ophthalmological manifestations, it is unlikely to be type IV.  

Hence, a possibility that remained to be ruled out was Type I sOHSS, which is FSH receptor mutation. A recent case report has described a novel FSH receptor mutation occurring in a patient with severe sOHSS with successful pregnancy outcome.[10] Hence the possibility of FSH receptor mutation was considered in our patient and analysis done for the purpose. However, no mutation was found.     

This case is being reported because a specific attempt was made to distinguish the two seemingly similar entities by testing for FSH receptor mutations. As mentioned in the literature reviewed above, and is presented in our case; hyperreactio luteinalis and sOHSS are entities which have a continuum and continue to be difficult to distinguish.

Acknowledgement

Dr Swati Achrekar, Division of Structural Biology, National Institute for Research in Reproductive Health, Parel, Mumbai, Maharashtra, India is thanked for performing the FSH receptor analysis for mutations. 

References

1. Jenkins JM, Drakeley AJ, Mathur RS, editors. The Management of Ovarian Hyperstimulation Syndrome. RCOG Green Top Guideline no. 5, 2006. Reconfirmed 2011. Available from: https://www.rcog.org.uk/globalassets/documents/guidelines/gtg5_230611.pdf.
2. Panagiotopoulou N, Byers H, Newman WG, Bhatia K. Spontaneous ovarian hyperstimulation syndrome: case report, pathophysiological classification and diagnostic algorithm. Eur J Obstet Gynecol Reprod Biol.  2013; 169(2): 143–48
3. Mittal K, Koticha R, Dey AK, Anandpara K, Agrawal R, Sarvothaman MP, et al. Radiological illustration of spontaneous ovarian hyperstimulation syndrome. Pol J Radiol. 2015 Jan;80:217–27. 
4. Malinowski AK, Sen J, Ser mer M. Hyperreactio Luteinalis: Maternal and Fetal Effects. J Obstet Gynaecol Canada [Internet]. 2015 Aug [cited 2016 Apr 10];37(8):715–23. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26474228  
5. Foulk RA, Martin MC, Jerkins GL, Laros RK. Hyperreactio luteinalis differentiated from severe ovarian hyperstimulation syndrome in a spontaneously conceived pregnancy. Am J Obstet Gynecol. 1997 Jun;176(6):1300-4. 
6. Coccia ME, Pasquini L, Comparetto C, Scarselli G. Hyperreactio luteinalis in a woman with high-risk factors. A case report. J Reprod Med . 2003 Feb;48(2):127–9. 
7. Gherman RB, Mestman JH, Satin AJ, Goodwin TM. Intractable hyperemesis gravidarum, transient hyperthyroidism and intrauterine growth restriction associated with hyperreactio luteinalis. A case report. J Reprod Med. 2003 Jul;48(7):553–6. 
8. Suzuki S. Comparison between spontaneous ovarian hyperstimulation syndrome and hyperreactio luteinalis. Arch Gynecol Obstet . 2004 Mar;269(3):227–9. 
9. Haimov-Kochman R, Yanai N, Yagel S, Amsalem H, Lavy Y, Hurwitz A. Spontaneous ovarian hyperstimulation syndrome and hyperreactio luteinalis are entities in continuum. Ultrasound Obstet Gynecol . 2004 Nov;24(6):675–8. 
10. Chauhan AR, Prasad M, Chamariya S, Achrekar S, Mahale SD, Mittal K. Novel FSH receptor mutation in a case of spontaneous ovarian hyperstimulation syndrome with successful pregnancy outcome. J Hum Reprod Sci. Jan;8(4):230–3.

Citation

Madhva Prasad S, Gupta AS. OHSS Versus Hyperreactio Luteinalis: A Diagnostic Dilemma. JPGO 2016. Volume 3 Number 6. Available from: http://www.jpgo.org/2016/06/ohss-versus-hyperreactio-luteinalis.html

Epidermolysis Bullosa In The Newborn –a Rare Clinical Entity

Author Information

Singhal N*, Qureshi S**, Gupta AS***.
(* Second Year Postgraduate Student, ** Assistant Professor, *** Professor. Department of Obstetrics and Gynecology, Seth G.S. medical College and KEM hospital, Mumbai, India.)

Abstract

Epidermolysis bullosa (EB) is a group of hereditary skin disorders characterized by blister formation in varying degree of severity and can severely incapacitate the life of the affected person. The severity can range from mild localized disease to widespread devastating process. We report an interesting case of a couple whose 3 out of 4 children were born with Epidermolysis bullosa , two of them died in the neonatal period. This case is reported to highlight this rare disorder and the importance of prenatal diagnosis in couples at risk of recurrence of an inherited disorder.

Introduction

Epidermolysis bullosa is a group of inherited skin disorders characterized by extreme fragility of skin and mucous membrane which lead to the formation of blisters following trivial trauma. As it develops in the area more prone to pressure or friction they are also known as mechanobullous disorder. There are four major types; epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa, Kindler syndrome.  Epidermolysis bullosa simplex(EBS) is most common among them and per se has an incidence of only 10-30 cases per million worldwide. [1] EBS generally manifest at birth or during the neonatal period. We report a case of Epidermolysis bullosa simplex in a full term baby at birth.

Case Report

A 40 year old patient G4P3L1D2, in a non consanguineous marriage presented at 40 weeks of gestation with the chief complaints of pain in abdomen. The patient was in labor, she progressed and delivered a male child of 2.886 kg, appropriate for gestational age, with apgar score of 9/10. The male neonate had skin lesions at birth.

Patient got registered in our antenatal clinicat 13 weeks of gestation. Her menstrual cycles were regular. In her first pregnancy she had delivered a full term male child normally in a hospital. Baby had skin lesions at birth which gradually spread to the whole body involving the mucosa of the oral cavity. Baby got admitted in neonatal intensive care unit (NICU) for the same and died at 2 months of age in NICU due to pneumonia. In her second pregnancy she again delivered a full term male child normally. Baby is 4 years, alive and well and had no skin lesions from birth till date. In her third pregnancy she delivered a full term male child normally. Baby had skin lesions like the first child and was admitted to NICU. Baby died at one month of age due to pneumonia. Patient was referred to Genetic lab and advised genetic counseling but patient did not follow up. She has no other significant medical or surgical illness. Her antenatalprofile was within normal limits.  There was no history of any skin disorder in patient’s family but her husband himself had fluid filled lesions at birth which gradually resolved with time. He gave the history of one month NICU stay after which he was discharged. He had skin lesions till 12 years of age which healed by minimal scarring. Now he gives the history of blistering of the skin in trauma prone areas. None of the parents or any member of the extended family of the husband had history of blistering.

On examination her vital parameters were stable; there were no lesions on perineum or any other site on the skin. Labor progressed spontaneously and patient delivered a male baby of 2.886 kg, appropriate for gestational age with apgar score of 9/10. At birth baby had superficial erosions of skin on the medial aspect of both right and left ankle. Oral cavity, conjunctiva, cornea, nails, scalp and genitalia were normal.  Soft non adhesive dressing was applied and baby was shifted to NICU. In NICU baby sepsis screen was done which was negative. Fresh lesions developed over the buttocks on day 2 of life. Skin reference was done, biopsy that was taken diagnosed epidermolysis  bullosa simplex. The neonate was discharged on day 18.

Figure 1. Lesions on the lower extremity

Figure 2. Lesions on the back 

Discussion

Epidermolysis bullosa (EB) is a heterogeneous group of hereditary disorders characterized by extreme fragility of skin and mucous membrane which gives rise to formation of blisters and ulcers following minor trauma. These disorders are associated with wide range of complications from localized skin fragility to neonatal death. There are 4 major types of Epidermolysis bullosa based on the affection of the exact microscopic site that splits resulting in  blistering.

1) Epidermolysis bullosa simplex

2) Junctional Epidermolysis bullosa

3) Dystrophic Epidermolyis bullosa

4) Kindler Syndrome

Out of four types, Epidermolysis bullosa simplex (EBS) is the most common type and has an incidence of 10-30 cases per million. EBS is an autosomal dominant disorder although mode is recessive in some subtypes. In EBS blisters are commonly seen at birth or in the neonatal period. Site of predilection are hand, feet, elbow, knees, legs and scalp. Intraoral lesions can also be present. The blisters typically heal with minimal to no scarring or milia formation and do not result in atrophy of the skin. The complications are secondary infection, sepsis, deformities, anemia, dehydration, skin cancer. Most common cause of death in EBS is sepsis followed by respiratory failure.[2] The propensity to blister formation decreases with age.

The diagnosis of Epidermolysis bullosa can be made based on clinical picture of the patient like age of onset ;size, frequency and location of blisters in trauma prone area; family history of blistering and the patient’s geographic and racial ancestry. However absence of affected family does not necessarily mean that the mode of inheritance is autosomal recessive but can be due to spontaneous mutation or incomplete penetrance of an autosomal trait.

There are various laboratory studies for confirmation of the diagnosis of EB like transmission electron microscopy, immunoflorescence mapping, and genetic testing. Electron microscopy helps to directly visualize and quantify specific ultra structural features and has the sensitivity and specificity of 71% and 81% respectively when compared with genetic results. Immunoflorescence mapping (IFM) determines the level of cleavage by determining binding sites for a series of antibodies. IFM has the sensitivity and specificity of 97% and 100% respectively when compared with genetic results. [3]

Skin biopsy helps to diagnose Epidermolysis bullosa. In histology, electron microscopy reveals the abnormal epidermal keratin filaments in epidermolysis bullosa simplex (EBS), abnormal dermal anchoring fibrils in Dystrophic epidermolyis bullosa and defective hemidesmosomes in Junctional Epidermolysis bullosa. Kindler syndrome is added recently as the fourth major type of epidermolysis bullosa with a unique clinical phenotype –photosensitivity.

The differential diagnosis includes familial benign pemphigus, toxic epidermal necrolysis, herpes simplex infections, syphilis, and staphylococcal pyoderma. There is presently no definitive cure for EBS.  Treatment is supportive and aims to relieve the symptoms. Once blistering has occurred, blister should be punctured with a sterile needle keeping in mind to leave the roof of the blister intact and by covering the affected area with white petroleum impregnated gauze. Open wounds should be covered with non adherent dressing such as petroleum impregnated gauze, hydrogels, fenestrated silicone dressings. Psychological support for parents and family members is vital. Corrective gene therapy is the ideal therapy for EB but the research is still going on before it can be used in clinical practice.

Nowadays, prenatal diagnosis is offered for couples at risk of recurrence of severe inherited skin disorder. Initially it was based on fetal skin biopsy sampling but has largely been superseded by DNA based analysis mostly using foetal DNA derived from chorionic villus sampling taken at 10-12 weeks of gestation. [4]

In our patient her first and third pregnancies resulted in babies who had skin lesion at birth and died soon after. Second child was normal. In her fourth pregnancy she delivered a baby with skin lesions which was provisionally diagnosed as EBS. We suspect that other two babies also had EBS though no diagnosis was made at that time. Thus, this case report highlights the importance of proper diagnosis as well as preconception and prenatal testing for couples at risk of recurrence of severe inherited skin disorder.

References

1. Sarkar R, Bansal S, Garg VK. Epidermolysis bullosa: Where do we stand? Indian J Dermatol Venereol Leprol 2011;77:431-8.
2. Fine JD, Johnson LB, Weiner M, Suchindran C. Cause-specific risks of childhood death in inherited epidermolysis bullosa.J Pediatr. 2008 Feb;152(2):276-80.
3. Fassihi H, McGrath JA.Prenatal diagnosis of epidermolysis bullosa. Dermatol Clin. 2010 Apr;28(2):231-7 
4. Yiasemides E, Walton J, Marr P, Villanueva EV, Murrell DF. A comparative study between transmission electron microscopy and immunofluorescence mapping in the diagnosis of epidermolysis bullosa. Am J Dermatopathol. 2006 Oct;28(5):387-94.

Citation

Singhal N, Qureshi S, Gupta AS. Epidermolysis Bullosa In The Newborn –a Rare Clinical Entity. JPGO 2016. Volume 3 Number 6 Available from: http://www.jpgo.org/2016/06/epidermolysis-bullosa-in-newborn-rare.html

Vaginal Varix in Pregnancy - A Therapeutic Dilemma

Author Information

Dharmadhikari M*, Samant PY**, Pai K***

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

Abstract

Vaginal varices during pregnancy is a rare condition. The vulval varicosities seen in pregnancy are very rare. Vaginal varix can rupture from trauma during the second stage of labor, leading to a  hematoma with subsequent extravasation into the tissues. We report a case of vaginal varices detected on routine examination in early labor.

Introduction

Genital varicosities occur in approximately 4% of pregnant women, & usually develop during third or fourth month of gestation.[1] They mostly regress spontaneously after parturition. Pregnancy is associated with dilatation of the vascular system, increased blood volume, and uterine pressure on pelvic veins.[2] Increased levels of oestrogen & progesterone causing venous distension along with  the scarcity of valves in the pelvic veins contribute to varicosities in pregnancy. Most genital varices are asymptomatic, but a few are associated with pelvic pain and spontaneous bleeding per vaginum. This pelvic congestion syndrome comprises of pelvic pain, dyspareunia, dysmenorrhea, dysuria and vulval/ perivulvar varices.[3,4]  Patients have discomfort during walking. It is essential to understand the underlying causes of vaginal varicosities formation in pregnancy, site of origin and change in course of varicosities  during vaginal delivery so that the mode of delivery can be planned in patients with vaginal varicosities.

Case Report

A 35 years old female,  non-smoker multigravida with previous vaginal delivery, with 38 weeks of gestation presented in early labor. There was no history of any major medical and surgical illness. The full term uterus with cephalic presentation had minimal activity. On speculum examination,  about 4x3 cm size plexus of engorged, tortuous veins was seen on anterior vaginal wall 3 cm below urethral meatus suggestive of vaginal varicosities. Internal os was 1.5 cm dilated, 30% effaced with intact membranes. There were no limb varicosities.

Figure 1: Preoperative vaginal varix.

The patient and family were counseled about cesarean section in view of risk of rupture of vaginal varicosities during delivery. Ultrasound with color Doppler and MRI could not be performed as patient was in labor. A lower segment cesarean section (LSCS) was performed uneventfully. There was no extraordinary pelvic congestion. On postoperative day 4, speculum examination showed a significant decrease in the size of vaginal varicosity to 2x2 cm suggestive of spontaneous regression of vaginal varicosities.

Figure 2: Postoperative day 4.

Discussion

A patient of vaginal varicosities should be examined completely to exclude possibility of associated conditions like leg varices, venous malformation of the labia, clitoral area, or vagina and associated vascular malformation on limbs (Klippel-Trenaunay syndrome).[5] The Klippel-Trenaunay syndrome comprises of skin capillary malformations, varicosities and soft tissue hypertrophy. In cases of Klippel-Trenaunay syndrome, long term treatment with anticoagulant and elastic stockings may be required to prevent thromboembolic events Any two of these three features are diagnostic. The few cases reported in the literature, manifested vaginal variceal bleeding due to portal hypertension.[6,7]

Vaginal varicosities can rupture from trauma leading to vaginal hematoma or thrombus during second stage of labor. They regress spontaneously postpartum. Occasionally there may be pelvic pain and dyspareunia. Transvaginal ultrasonography and Doppler study can confirm diagnosis as well as the extent and anatomy of the varices. Doppler studies can also rule out haemangioma and angiosarcoma. Magnetic resonance imaging helps in anatomical understanding of vaginal varicosities in pregnancy. Conservative measures like firm pelvic support usually relieves symptoms. If symptoms persist for more than 3 months postpartum, sclerotherapy may be used.[8] In cases of Kippel-Trenaunay syndrome, long term  treatment with anticoagulant and elastic stockings may be required to prevent thromboembolic events after delivery.

In case of huge vaginal varicosities, massive bleeding may occur at vaginal delivery. There is paucity of data to predict the risk of rupture during a vaginal delivery. ‘Huge’ vaginal varix itself has not been defined. Furuta et al[9] observed that vulval and vaginal varicose veins markedly diminished at the end of the second-stage of labor with compression by fetal head.[9] This may suggest that extensive vulvovaginal varicosities will decrease in size irrespective of their size or extent during labor and delivery.[9] However in practice, it is important to avoid accidental laceration of the vaginal varicosities at this stage. Careful delivery may minimize but cannot completely eliminate the risk of trauma and rupture of varicosities. A case with fetal death from significant maternal bleeding due to  vaginal variceal rupture, was reported by Purslow et al.[10] Watermeyer et al. reported a 21-year-old primigravida with a massive plexus of varicosities filling the lower uterine segment that required classical cesarean section. In some cases, engorged vessels may bleed periodically on increased intraabdominal pressure.[5]

Conclusion

There is paucity of evidence to recommend either mode of delivery in cases of vulval varicosities. There are no definite guidelines on management of vaginal varices in intra- and postpartum period. The cases are dealt with as per the amount of bleeding encountered and there are no recommendations for or against cesarean section as a preventive measure.

Pelvic varices may surprise an obstetrician even at LSCS  On the other hand, vaginal varicosities may cause extensive hemorrhage during labor.[11] Optimal mode of delivery can only be decided after evaluating the cause of the vaginal varicosity. Hence an antenatal Doppler and MRI studies are advisable.

References

1. Bell D, Kane PB, Liang S, Conway C, Tornos C. vulval varices: an uncommon entity in surgical pathology. Int J Gynecol Pathol 2007;26(1):99-101.
2. Cunningham FG. Maternal physiology. Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, et al editors. In Williams obstetrics. 24th.ed. New York: McGraw-Hill Education; 2010; 79-80.
3. Scultetus AH, Villavicencio JL, Gillespie DL, Kao TC, Rich NM. The pelvic venous syndromes: analysis of our experience with 57 patients. J Vasc Surg 2002;36(5):881-8.  
4. Hobbs JT. Varicose veins arising from the pelvis due to ovarian vein incompetence. Int J Clin Pract 2005;59:1195-203.
5. Watermeyer SR, Davies N, Goodwin RI. The Klippel-Trenaunay syndrome in pregnancy. BJOG 2002;109:1301-2.
6. Orlando G, Goffette P, Geubel A, Lerut J. Vaginal bleeding complicating portal hypertension: a particular entity-report of two cases and review of the literature. Transplant International 2005;18(12):1382-5.  
7. McHugh PP, Jeon H, Gedaly R, Johnston TD, Depriest PD, Ranjan D. Vaginal varices with massive hemorrhage in a patient with nonalcoholic steatohepatitis and portal hypertension: Successful treatment with liver transplantation. Liver Transpl. 2008;14:1538-40.
8. Hobbs JT. The treatment of vulval and pelvic varices. In: Bergan JJ, Yao JS, editors. Venous disorders.1st ed. Philadelphia (PA): W.B. Saunders Co; 1991. p.250-7. 
9. Furuta N, Kondoh E, Yamada S, Kawasaki K, Ueda A, Mogami H, Konishi I. Vaginal delivery in the presence of huge vulval varicosities: a case report with MRI evaluation.European Journal of Obstetrics & Gynecology and Reproductive Biology 2013; 167(2): 127-131
10. Purslow CE, Branson GJ. Severe Ante-Partum Haemorrhage Due to Spontaneous Rupture of Vaginal Varix. Br Med J 1910;1:319-20.  
11. Reich WJ, Nechtow MJ. Rupture of a vulval varix with massive and extensive hemorrhage following a normal delivery. Am J Obstet Gynecol. 1951;61:1374-1375.

Citation

Dharmadhikari M, Samant PY, Pai K. Vaginal Varix in Pregnancy- A Therapeutic Dilemma. JPGO 2016. Volume 3 No. 6. Available from: http://www.jpgo.org/2016/06/vaginal-varix-in-pregnancy-therapeutic.html

Successful Reproductive Outcome In A Young Female Following A Mixed Germ Cell Tumor

Author Information

Satia MN*, Madhavi J**, More V***

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

Abstract

Ovarian tumors in adolescent female is more often benign than malignant. Below the age of 20 years, 60% of the tumors are of germ cell origin. Also, increased incidence of ovarian tumors in reproductive age group is attributed to the improvements in diagnostic modalities and hence, there is more need for fertility preserving surgeries. We report an interesting case of a young girl with a mixed germ cell tumor. It was an embryonal cell carcinoma with yolk sac remnants who underwent a fertility preserving surgery and later had a successful pregnancy outcome twice and then came back to us for medical termination of pregnancy with tubal ligation.

Introduction 

Germ cell malignancies account for fewer than 5% of ovarian cancers in western countries and around 15% in Asian countries.[1] Twenty to 25 % of all ovarian neoplasms are of germ cell origin[2], out of which 3% cases are malignant. Mixed germ cell tumors account for 10% of germ cell tumors. They are composed of two or more germ cell elements such as dysgerminoma, teratoma, yolk sac tumor, choriocarcinoma, or embryonal carcinoma. Our patient had a tumor comprising of embryonal cell carcinoma with yolk sac elements. Embryonal cell carcinoma is an extremely rare germ cell tumor, contributing to 3% of all germ cell tumors.[3] It affects very young patients between 4 to 28 years of age. The treatment challenge lies in preservation of fertility, as the tumor affects very young females in the reproductive age group.

Case Report 

A 29 year old woman, married for 7 years, gravida 3, para 1 and abortion 1 with 6 weeks of pregnancy came to antenatal outpatient department. At 16 years she had presented with acute abdomen to the emergency department. Ultrasonography done was suggestive of right ovarian mass with torsion. A decision of emergency exploratory laparotomy was taken. Intraoperatively there were two and half turns of torsion, hence right salpingo oophorectomy was performed. The tumor was solid, well capsulated, around 8-10 cm in size. There was no ascites. Patient had an uneventful post operative stay. Histopathology report was suggestive of mixed germ cell tumor; embryonal cell carcinoma with elements of yolk sac tumor. Hence she was referred to an oncologist for further management. Tumor markers were sent. Serum β HCG was 0, α-fetoprotein was 3 ng/ml and CA-125 was 66 IU/ml. CT abdomen and pelvis did not detect any abnormality or any metastatic focus. She had six monthly follow up with the oncologist and her tumor markers were always in the normal range. The table below depicts the regular follow up of the patient over 10 years. No adjuvant therapy was required. She conceived immediately after marriage but had missed abortion for which dilatation and evacuation was done. She conceived spontaneously after an year, continued uneventfully till term and a lower segment cesarean section was performed for breech presentation. During present pregnancy she was antenatally registered at our hospital since 6 weeks of pregnancy. She developed severe pre-eclampsia at 35 weeks of gestation for which antihypertensive therapy was given. Lower segment cesarean section was performed for breech presentation with previous LSCS and impending eclampsia. She delivered a male child of 3.2 kg with apgar at 1 minute of 9/10. Intraoperatively, right sided ligated tubal stump was seen and the left sided fallopian tube and ovary was normal. Cesarean section was uneventful. Post operative course was uneventful and patient was discharged on day 7 of surgery with advise to use depot medroxyprogesterone as contraceptive once in three months. A year after the birth of her second child the patient presented with six weeks of pregnancy. She underwent medical termination of pregnancy and left sided tubal ligation through a minilaprotomy.

Discussion 

In the adolescent age group, 80 % of the malignant ovarian tumors are germ cell tumors, and are usually diagnosed between 16-20 years of age.[4,5] According to WHO in 1973, germ cell tumors are classified as dysgerminomas or non-dysgerminomas.[6] Dysgerminomas, considered as the female counterparts of testicular seminomas are the most common (50%) germ cell tumors. Non dysgerminomas include endodermal sinus tumor, embryonal cell carcinoma, polyembryoma, choriocarcinoma, teratomas (immature, mature, and monodermal), mixed and gonadoblastoma. Yolk sac tumors or endodermal sinus tumors are the commonest after dysgerminomas accounting for 20% of the cases, affecting young females around 19 years of age.[7] Embryonal cell carcinoma of the ovary is an extremely rare tumor that is composed of undifferentiated cells. This lesion is a progenitor of other germ cell tumors. As already mentioned these tumors are seen in very young females, the median age at diagnosis being 14 years.[8] These tumor cells synthesize both α fetoprotein and β-HCG, that can be later used to monitor the response to chemotherapy. As compared to slower growing epithelial ovarian tumors, germ cell tumors grow rapidly and are characterized by pelvic pain due to capsular distension, hemorrhage or necrosis. The rapidly enlarging mass can lead to pressure symptoms due to compression on bladder or rectum. Menstrual irregularities have been reported in menarcheal patients. This is attributed to the estrogen secretion seen in embryonal cell carcinoma.[9] Patients can also present with torsion of the ovarian mass, as was seen in our patient and rarely, rupture of the adnexal mass. The primary lesions are large and are usually confined to ovary at the time of diagnosis. Primary modalities of diagnosis include ultrasonography (USG) and computed tomography (CT) of the abdomen and pelvis. CT chest is also recommended as germ cell tumors are known to metastasize to lungs or mediastinum. Tumor markers are also done to evaluate for the nature of the ovarian tumor. As our patient presented with an acute abdomen, these investigations were done retrospectively after confirmation with the histopathological diagnosis. Grossly, embryonal cell carcinomas are poorly defined with areas of hemorrhage and necrosis.[10] Microscopically, they have indistinct cell borders with a variable architecture, composed of solid, glandular and papillary type of cells.

As germ cell tumors affect young reproductive females, preservation of fertility and ovarian function is important, although it is a controversial issue in gynecological oncology. Treatment for stage I cancers is surgical resection alone with a unilateral salpingo-oophorectomy and staging. Ipsilateral tube is removed as there is abundant lymphovascular connection between tube and ovary. Surgical resection requires a diligent follow up care with serial pelvic examinations and follow up with tumor markers is necessary if resection is preferred as the only treatment of choice. The follow up of ovarian germ cell malignancies is usually done with α-fetoprotein (AFP) and β human chorionic gonadotrophin.[11] Our patient had a regular follow up for 10 years (Table 1).

Weinberg et al described 8 patients with spontaneous pregnancies ending in 11 live full-term births in a series of twenty two patients ( fertility rate of 80%).[12] A wedge biopsy of the contralateral ovary was previously recommended to rule out metastasis; but is not recommended now as germ cell tumors are good responders to chemotherapy and also, the fear of adhesions in the ovary after wedge biopsy that might affect future fertility. 

Adjuvant treatment in the form of radiotherapy and chemotherapy is recommended for tumors beyond stage Ia. Radiotherapy has been replaced by chemotherapy owing to the long term complications associated such as sterility and early menopause.[13] For those patients requiring chemotherapy, the regimen comprises of Bleomycin, Etoposide and Cisplatin for 3-4 cycles. Germ cell tumors respond well to platinum based chemotherapy regimens. The efficacy of chemotherapy has now allowed conservative surgery including only a unilateral salpingo-oophorectomy preserving fertility in grade I tumors.[14]

Date	AFP ng/ml	β HCG IU/l
10/12/2002	2.7	0
26/3/2003	4.0	0
10/6/2003	3.1	0
23/9/2003	3.0	0
13/1/2004	3.1	0
28/7/2004	2.9	0
8/2/2005	3.6	0
15/9/2005	1.84	0
16/3/2006	3.51	0
8/1/2008	4.11	<1.20
3/6/2008	3.99	<1.20
4/12/2008	3.93	<1.20
2/8/2010	5.10	<1.20
10/8/2011	4.98	<1.20

References

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Citation

Satia MN, Madhavi J, More V. Successful reproductive outcome in a young female following a mixed germ cell tumor. JPGO 2016. Volume 3 No. 6. Available from: http://www.jpgo.org/2016/06/successful-reproductive-outcome-in.html