Volume 4 Issue 4, April 2017

Editorial
Parulekar SV

46XY Pure Gonadal Dysgenesis
Valvi Durga, Parulekar SV.

Posterior Isthmic Leiomyoma in a Post-Hysterectomized Woman
Mehta N, Parulekar SV.

Omental Abscess Encasing A Lost Multiload
Deshpande PS, Pardeshi SH, Gupta AS.

Uterine Graveyard :Rare cause For Secondary Infertility
Jaybhaye SB

Vulvar Abscess in Autoimmune Disorder - A Clinical Challenge
Rane V, Samant PY, Kamat S, Daigavane MM.


Use Of Immunosuppressive Drugs For Systemic Lupus Erythematosus In Pregnancy
Satia M, Shilotri M, Panchbudhe S.

Pendulous Abdomen Causing Levorotation of Uterus And Transverse Lie
Prabhu S, Mishra N, Savani G, Tintoiya I.

Pregnancy In Müllerian Anomaly: A Diagnostic Dilemma To Fetal Outcome
Shetty A, Thosar MA, Gupta AS.

High Dose Hook Effect With Urine Pregnancy Test
Goel A, Amin K, Chauhan AR.

Editorial

Parulekar SV

Torsion of the gravid uterus is not a very common condition. Some degree of dextrorotation is usually seen in pregnancy due to the presence of the sigmoid colon on the left and posterior aspect of the uterus. It is also the normal direction of the uterine muscle fibers. Usually the rotation is mild (less than 40 degrees) and asymptomatic. Rotation greater than 45 degrees is uncommon. Of those cases, about 5, 25 and 70% cases occur in the first, second, and third trimester respectively. The earliest occurrence of uterine torsion in pregnancy has been at 6 weeks, and the latest at 43 weeks. There is a predisposing factor in about 20% cases, such as uterine leiomyoma, bicornuate uterus, adnexal mass or pelvic adhesions due to a past pelvic surgery or other causes. Congenital weakness at the junction of the uterine corpus and cervix has also been proposed as a cause. Vehicular accidents and external cephalic version have also been listed as possible causes. Chronic torsion is seen much more often than acute or subacute torsion. Levorotation of the uterus is less common. It would be seen in case of situs inversus with the sigmoid colon on the right and posterior aspect of the uterus and any of the other conditions listed above. A very high degree of suspicion in the antenatal period may bring to attention a few cases. Acute symptoms like abdominal pain, nausea, vomiting, diarrhea,  vaginal bleeding, dystocia, urinary symptoms like urgency, frequency, nocturia and hematuria may draw attention to the presence of the condition. However chronic asymptomatic type is far more common than the acute or subacute type. On palpation of the maternal abdomen, the round ligament may be felt passing across the abdomen. Uterine artery pulsations may be palpable in the anterior fornix. A speculum examination may show a distortion of the vagina and cervix.  The diagnosis of the chronic type can be confirmed by performing a magnetic resonance imaging scan, which would show an X-shaped vagina rather than the usual H-shaped one. However such cases are extremely rare. Most of the asymptomatic rotations escape detection, since there is no way of diagnosing them when the women deliver vaginally. The condition comes to light only when a cesarean section is performed. In those cases the rotation must be corrected manually before making uterine incision, or the broad ligament and its contained structures would get cut in case of rotation of 90 degrees, and posterior wall of the uterine lower segment in case of rotation of 180 degrees. Such cases have been reported periodically over the years. The maternal morbidity in such cases is serious and entirely unjustifiable.

Pendulous abdomen due to poor muscle tone and divarication of recti is another condition that can affect the obstetric outcome. It can cause transverse lie, face presentation, dystocia, and injury to the fetal neck. It is related to excessive stretching of abdominal wall during past pregnancies and lack of abdominal wall exercises subsequently. Concurrent occurrence of levorotation of the uterus and pendulous abdomen causing transverse lie is extremely uncommon. We have one such case reported in this issue. What makes it even more interesting is that the authors have included photographs of the operative findings, which are not found in the literature published so far. I believe that our readers will find this case report interesting as well as educative.

46XY Pure Gonadal Dysgenesis

Author Information

Valvi Durga*, Parulekar S V**

(* Assistant Professor; ** Professor and Head; Department of Obstetrics and Gynaecology, Seth G S Medical College and K.E.M Hospital, Mumbai, India.)

Abstract

We report a case of 46 XY pure gonadal dysgenesis with complete female phenotype. who presented to us with complaint of primary amenorrhea with absence of secondary sexual characters.

Introduction

Swyer syndrome is a disorder of sexual development (DSD) caused by 46,XY complete gonadal dysgenesis. Swyer first reported two cases of this syndrome in 1955.[1] It is a rare disorder seen in 1 in 80000 births.[2] It is the result of mutations of the SRY gene or microdeletion in the SRY gene.  Due to defect in gene,  the indifferent gonads fail to differentiate into testis in an XY (genetically male) fetus. Absence of testes results in non production of testosterone  and antimüllerian hormone (AMH). Without testosterone the Wolffian ducts fail to develop and internal male organs are not formed and external genitalia do not virilize, resulting in development of normal female genitalia. Without AMH, the Müllerian ducts develop into normal internal female organs (uterus, fallopian tubes, cervix and upper third of the vagina). The streak gonads are at risk of developing  gonadoblastoma or dysgerminoma.[3,4] We present a case of Swyer syndrome.

Case Report

A 22 year old female presented in the outpatient clinic with a complaint of primary amenorrhea and absence of development of breasts. She had not consulted earlier due to family history of late onset of menses. She was the tallest person in the family. Her siblings were normal. On examination, her breast development was Tanner stage 1, axillary and pubic hair was in Tanner stage 2-3. her height was 174 cm, arm span was 185 cm, lower segment was 98 cm, upper to lower segment ratio was 0.77, transeverse diameter of chest was 35 cm and internipple distance was 25 cm.  Her father's height was 172 cm and mother's height was 150 cm.  Abdominal examination was normal, external genitaila were as in a normal female. Vaginal opening was seen. Laboratory investigations showed FSH 77.76 mIU/ml, LH 30.64 mIU/ml, thyroid function tests, serum prolactin serum testesterone, and basal cortisol levels normal. Her karyotype was 46XY, Ultrasonography of pelvis showed a small sized uterus and streak gonads.

Vaginoscopy was done. It showed adequate length of vagina and a small cervix at top of the vagina. Laparoscopy showed rudimentary uterus and fallopian tubes, and bilateral streak gonads in the position of the ovaries. Bilateral gonadectomy was done by operative laparoscopy. Histopathology report showed mesonephric duct remnants and residual ovarian stroma. She was advised hormone replacement therapy.

Figure 1. Cervix: anterior lip (black arrow), external os (red arrow).

Figure 2. Uterus (arrow). Fallopian tubes are seen on two sides of the uterus.

Figure 3. Right fallopian tube (RFT) and right streak gonad (RO).

Figure 4. Left fallopian tube (LFT) and right streak gonad (LO).

Discussion

Various causative genes responsible for development of 46,XY DSD gonadal dysgenesisare ARX, ATRX, CBX2,  DHH,  DMRT1,  GATA4,  MAMLD1, MAP3K1, NR0B1, NR5A1, SOX9, SRY, WNT4, WT1 and WWOX.[5] This DSD may be a part of a genetic syndrome like campomelic dysplasia, Denys–Drash   syndrome and   Frasier   syndrome.[6] About 25% cases have associated anomalies of heart or central nervous system.[7]

The patient is a phenotypic female at birth and presents with delayed puberty and primary amenorrhea. The stature is tall. Breast development is delayed, but axillary and pubic hair is developed. Since less than 5% cases have a sibling with that condition, early suspicion and karyotyping is not possible. So the diagnosis may be delayed. Serum FSH level is high and karyotype is 46XY. Pelvic imaging shows streak gonads and normal or underdeveloped uterus and fallopian tubes. An early diagnosis is essential because the risk of gonadal neoplasia is increased,  early sex hormone administration has to be given for inducing and maintaining female pubertal development, and for achieving adequate bone mass. Gonadoblasomas can develop in one or both of the dysplastic gonads.[2,8,9] Though they are benign, they can lead to development of germ cell malignancy like dysgerminoma, endodermal sinus tumor, embryonal  carcinoma and teratoma. Hence when the diagnosis is made, bilateral gonadectomy should be done at the earliest, instead of performing a biopsy first. Then cyclical  estrogen  and progestin replacement therapy should be given up to the age of 50 years. Counseling need to be done to explain that menstruation and normal reproduction will not be possible, but sexual function will be possible. A pregnancy may be possible with ovum donation if the uterus is well developed.[10-13] If the vagina is not of adequate depth, a vaginoplasty will be required.

References

1. Swyer GI. Male pseudohermaphroditism: a hitherto undescribed form. Br Med J 1955; 2:709–712.
2. Michala  L,  Goswami  D,  Creighton  SM,  Conway  GS.  Swyer  syndrome: presentation and outcomes. BJOG 2008; 115:737–741.
3. Zheng E, Weili L. A familial 46 XY gonadal dysgenesis and high incidence of embryonic gonadal tumors. Chinese journal of cancer research.1994;6(2):144-148. doi: 10.1007/BF02997250.
4. Ben Temime R, Chachial A, Attial L, Ghondbanel I, Makhloufl T, Koubaal A, et al. 46 XY pure gonadal dysgenesis with gonadoblastoma and dysgerminoma. Tunis Med 2008;86(7):710-3.
5. Ono M, Harley VR. Disorders of sex development: new genes, new concepts. Nat Rev Endocrinol 2013; 9:79–91.
6. Ostrer H. Disorders of sex development (DSDs): an update. J Clin Endocrinol Metab 2014; 99:1503–1509.
7. Cox K, Bryce J, Jiang J, Rodie M, Sinnott R, Alkhawari M, et al. Novel associations in disorders of sex development: findings from the I-DSD Registry. J Clin Endocrinol Metab 2014;99:E348–E355.
8. Liu AX, Shi HY, Cai ZJ, Liu A, Zhang D, Huang HF, Jin HM. Increased risk of gonadal malignancy and prophylactic gonadectomy: a study of 102 phenotypic female patients with Y chromosome or Y-derived sequences. Hum Reprod 2014; 29:1413–1419.
9. Juniarto AZ, Setyawati BA, Miranti IP, Santosa A, Hersmus R, Stoop H, et al.  Gonadal  malignancy  in  13 consecutive  collected  patients  with  disorders  of  sex  development  (DSD) from Semarang (Indonesia). J Clin Pathol 2013; 66:198–204.
10. Poláková M, Alexander D, Sulc J, Zetová L, Vlk R, Křepelová A, Zantová D. Pregnancy and delivery in a patient with pure  46,XY  karyotype.  Summary  of  actual  knowledge  about  XY  women. Ceska Gynekol 2013; 78:443–447.
11. Frydman, R., Parneix, I., Fries, N., Testart, J., Raymond, J., Bouchard, P. Pregnancy in a 46,XY patient. Fertil Steril 1988; 50:813–814.
12. Kan AK, Abdalla HI, Oskarsson T. Two successful pregnancies in a 46,XY patient. Hum Reprod 1997; 12:1434–1435.
13. de Santis M, Spagnuolo T, Barone D, Licameli A. Successful twin pregnancy in a 46,XY pure gonadal dysgenesis. J Obstet Gynaecol 2013; 33:737–738.

Citation

Valvi Durga, Parulekar SV. 46XY Pure Gonadal Dysgenesis. JPGO 2017. Volume 4 No. 4. Available from: http://www.jpgo.org/2017/04/46xy-pure-gonadal-dysgenesis.html

Posterior Isthmic Leiomyoma In A Post-Hysterectomized Woman

Author Information

Mehta N*, Parulekar SV**
(* Second Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G. S. Medical College and KEM Hospital, Mumbai, India.)

Abstract

We present an interesting case of a 55 year old post-hysterectomized woman who had a 20-22 weeks’ size mass arising from the pelvis. On imaging studies it was suggestive of a broad ligament leiomyoma, but on exploratory laparotomy it turned out to be an isthmic leiomyoma.

Introduction

A leiomyoma is the commonest benign tumor of the uterus in the reproductive age. It can develop in any part of the uterus or its ligaments. It may also develop in the broad ligaments. If a subtotal hysterectomy is performed, the cervix and sometimes the isthmus are left behind. They can develop any condition that can develop at that site. Thus if the operation has been done for uterine leiomyomas, a leiomyoma may develop there, either by growth of a seedling leiomyoma which had been present at the time of the first operation but could not be seen, or it may develop as a new growth due to predisposing factors which caused the development of leiomyomas in the first place. We present an unusual case of isthmic leiomyoma following subtotal abdominal hysterectomy.

Case Report

A 55 year old woman presented with complaints of pain in lower abdomen for 8 days. The pain was continuous, moderate, and nonradiating. There were no aggravating or relieving factors. There were no bowel or bladder symptoms. She gave history of abdominal hysterectomy having been done three years ago in view of uterine leiomyomas. She had no episode of vaginal bleeding after that operation. She had three normal deliveries. She had a viral infection in the past, after which she had developed bilateral lower limb weakness. The details of that illness were not available.  On examination, her general condition was fair, vital parameters were within normal limits and general and systemic examination revealed no abnormality. On abdominal palpation, there was a 20-22 weeks’ size mass arising from the pelvis, which was non-tender, solid, and mobile from side to side. Per speculum examination revealed a flat, 2 x 1 cm polypoid projection from near the left angle on the vault, covered with smooth, flat epithelium. It could not be assessed well because her lower limbs had reduced power and dorsal position could not be maintained well. On vaginal examination there was a 20-22 weeks’ size solid, non-tender mass with side to side mobility, occupying the pelvis and lower abdomen. On ultrasonography (USG), there was a heterogeneous hyperechoic well defined mass with internal whorled appearance measuring 15 x 12.6 x 14.2 cm in the lower abdomen, with no internal vascularity, suggestive of left broad ligament leiomyoma. Computerized tomography (CT) scan revealed a well-defined mass of 18.6 x 14.6 x 13.5 cm in the left adnexa, with no evidence of ascites or lymphadenopathy, suggestive of a broad ligament leiomyoma. Both ureters were passing posterior to the mass, with no compression effect. Her investigations for fitness for anesthesia showed normal results. The lower limb weakness was assessed by neurologists, who advised that the condition was static, and would be evaluated after the abdominal lump was treated by us.

Though the imaging techniques suggested that the leiomyoma was in the broad ligament, the side to side mobility of the lump ruled out that diagnosis, as broad ligament leiomyomas are extraperitoneal and hence not mobile. A tentative diagnosis of left adnexal leiomyoma, possibly in the uteroovarian ligament or residual round ligament was made. A differential diagnosis of left ovarian tumor was also considered. An exploratory laparotomy was done. A large leiomyoma measuring 25-30 cm in diameter was found to be arising from posterior part of the isthmus of the uterus. The uterine corpus and round ligaments were absent. The ovaries, fallopian tubes, cervix and uterosacral ligaments were present. The cervix was long. A diagnosis of post subtotal hysterectomy uterine isthmic leiomyoma was made. The leiomyoma was excised. The cervical stump was not removed as the relatives did not wish her to have that additional surgery. The anterior and posterior walls of the upper end of the cervix were sutured to each other with No. 1-0 polyglactin.  It was covered with the peritoneum raised during excision of the leiomyoma to prevent postoperative adhesions. After closure of the abdominal incision, a speculum examination was done. The tag seen preoperatively was found to be a flat polyp arising from the anterior lip of the cervix. It was excised. The patient made an uneventful recovery. The weight of the isthmic mass was found to be 2.5 kg. Histopathological examination of the mass showed it to be a leiomyoma. The cervical polyp was confirmed to be a cervical polyp.

Figure 1. Isthmic leiomyoma: Tumor - leiomyoma, LO - left ovary, RO - right ovary, Cervix - supravaginal cervix.

Figure 2. Cervical polyp, held with sponge holding forceps.

Discussion

A leiomyoma is the commonest benign tumor of the uterus.[1,2] It is found in 25% of women in the reproductive age. It is a monoclonal tumor arising from uterine smooth muscle. It can develop in any part of the uterus, its ligaments, and even in the broad ligaments. Broad ligament leiomyomas either develop from the adjacent part of the uterus or from paramesonephric remnants in the broad ligaments. Development of broad ligament leiomyomas after hysterectomy is possible, but has not been reported. On the other hand, leiomyomas can develop from the isthmus or cervix of the uterus after a subtotal abdominal hysterectomy.[3-8] Various cervical conditions that may develop after a subtotal hysterectomy include cysts, leiomyomas, prolapse, adhesions, and endometriosis. It is recommended that a total hysterectomy be performed when it is being done for a benign condition of the uterus, so as to prevent of cervical disease at a later date.[9,10]

Our patient had probably not been counseled adequately prior to and/or after her subtotal hysterectomy, or she had not understood what she had been told.. So she was not aware that her uterine corpus had been removed while the isthmus and cervix had been left behind. She had lost the records of her past treatment. So these details could not be found out. Owing to her neurological condition affecting her lower limbs, an adequate speculum examination could not be done, and hence the cervix which had been pulled upwards by the large leiomyoma could not be visualized. Her USG and CT scans further made an erroneous diagnosis of a broad ligament leiomyoma. If a diagnosis of isthmic or upper cervical leiomyoma had been made preoperatively, the cervix could have been removed along with the leiomyoma, with adequate counseling preoperatively. Now she has been advised to have Pap smears as per the screening protocol for detection of cervical cancer, and an annual gynecologic examination in order to detect development of any disease that may develop in the cervix.

Conclusion

Adequate counseling is essential prior to hysterectomy. In case any difficulty is encountered that prevents a total hysterectomy and a subtotal hysterectomy has to be performed, the patient and her relatives must be informed about the same, and advised to retain all documents related to the operation and have periodic follow-up examinations for development of some disease in the uterine cervix and isthmus. Imaging techniques, though helpful, cannot be totally relied upon when making a diagnosis of the nature of an abdominopelvic lump.

References

1. VC Buttram Jr, RC Reiter. Uterine leiomyomata: Etiology, symptomatology, and management [abstr]. Fertil Steril 1981;36:433.
2. SF Cramer, A Patel. The frequency of uterine leiomyomas [abstr]. Am J Clin Pathol 1990;94:435.
3. Adamian LV, Kulakov VI, Dzhabrailova SSh. Characteristics of surgical treatment of pathologic conditions of the cervical stump. Akush Ginekol 1991; 5: 60-62.
4. Bojahr B, Lober R, Romer T, Schwesinger G. Large cervix myoma after supra-cervical hysterectomy. Zentralbl Gynakol 1996; 118: 468-470.
5. Nezhat CH, Nezhat F, Roemisch M, et al. Laparoscopic trachelectomy for persistent pelvic pain and endometriosis after supracervical hysterectomy. Fertil Steril 1996; 66: 925-928. (Medline) 
6. Okaro EO, Jones KD, Sutton C. Long term outcome following laparoscopic supracervical hysterectomy. BJOG 2001; 108: 1017-1020. (Medline) 
7. Pelosi MA 3rd, Pelosi MA, Rudelli RD. Symptomatic cervical macrocyst as a late complication of subtotal hysterectomy. A case report. J Reprod Med 1999; 44: 567-570. (Medline) 
8. van der Stege JG, van Beek JJ. Problems related to the cervical stump at follow-up in laparoscopic supracervical hysterectomy. JSLS 1999; 3:5-7.
9. Markowska J, Markowska A. Total or subtotal hysterectomy? Ginekol Pol 2000; 71: 34-38. 
10. Munro MG. Supracervical hysterectomy: ... a time for reappraisal. Obstet Gynecol 199; 89:133-139.

Acknowledgement

We thank Dr Maitreyee Parulekar for taking photograph of the leiomyoma and Dr Durga Valvi for taking photograph of cervical polyp.

Citation

Mehta N, Parulekar SV. Posterior Isthmic Leiomyoma In A Post-Hysterectomized Woman. JPGO 2017. Volume 4 No. 4. Available from: http://www.jpgo.org/2017/04/posterior-isthmic-leiomyoma-in-post.html

Omental Abscess Encasing A Lost Multiload

Author Information 

Deshpande PS*, Pardeshi SH**, Gupta AS***.

(* First year Resident, ** Assistant Professor, *** Professor and Head of the Unit, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Various methods of contraception are available. Each method has its own merits and demerits but the essence of any method is good instructions to the patient and meticulous follow up. Here we present a case of a patient who had a Multiload inserted following 2 cesarean sections. It perforated into the abdominal cavity and was detected at term during her third pregnancy. Omentum had encased it and formed a sterile abscess. It was removed during her third cesarean section. 

Introduction

Intrauterine devices are in top most tier of WHO classification as the most effective method of contraception with the failure rates on perfect and typical use being comparable to permanent methods of sterilization.[1] One time out patient visit is needed for their insertion with their contraceptive benefits for periods ranging from 3 to 10 years. However, these devices do come with their share of complications and one needs quick diagnosis and prompt management of the same.[2]

Case Report

A 25 year old gravida 4, para 2, living 2, medical termination of pregnancy (MTP) 1, at 36 weeks of gestation with previous 2 cesarean sections (LSCS) was referred to our hospital in view of previous 2 LSCS with ultrasonography (USG) suggestive of possibly morbid adherent placenta with an intrauterine contraceptive device, partly in the uterus and partly outside. She had thalassemia trait. Her hemoglobin was 8.1 gm/dl.

Her first LSCS was done for nonprogress of labor and the second was done for previous LSCS. Three months post delivery she had an interval, post menstrual IUCD inserted  in a private hospital. Vaginal palpation of the thread of the IUCD post insertion was not taught to her. She was not instructed regarding follow up nor was she called for any specific follow-up. On redirect questioning she confirmed that the insertion was painful. Post insertion she had menses for one cycle. In the next cycle she conceived and desired an MTP. At the time of the MTP that was done in a private hospital, the IUCD thread was not seen, felt or found on vaginal examination, USG or with the products of conception post procedure. She was told that she must have expelled the IUCD unknowingly. Radiographic examination of the entire abdomen was not performed. No further investigations were done to locate the missing device. She was started on oral contraceptive pills (OC) for contraception. Three years later she stopped OC pills and conceived spontaneously. 

In the present pregnancy she was registered in first trimester and had 4-5 ANC visits. USG was done in first and second trimester with no mention about the IUCD. On routine USG in third trimester, the missing IUCD was reported as being partly in the uterus and partly outside along with a morbidly adherent placenta. She was admitted and a USG was done of the abdomen and pelvis for the IUCD which was suggestive of a 2.8 cm hyperechoic lesion surrounded by a 3 cm collection in the sub hepatic region suggestive of the IUCD. MRI pelvis was done and showed a foreign body with a peripherally loculated fluid collection in the subhepatic recess which could be an IUCD. On discussion with the radiologists, it was concluded that the device was not embedded in the uterus but was in the subhepatic region. No evidence of placenta accreta/percreta was noted. All serological and biochemical investigations were normal. After one packed cell transfusion she was scheduled for elective LSCS with tubal ligation and IUCD removal.

Under antibiotic cover, LSCS was done by a midline vertical incision. No adhesions or advancement of bladder was seen. LSCS was done uneventfully. She delivered a healthy, 2.4 kg female baby. Uterus, cervix and fallopian tubes did not show any area suggestive of old healed scar of perforation. On bimanual palpation of the uterus with one hand in the cavity and one on the surface of the uterus, no localized area of thinning or scar of suspected perforation site was felt. Uterine incision was closed. Bilateral tubal ligation was done. The subhepatic region was explored carefully and a localized thickening of the omentum with IUCD thread was seen. The thickened part of the omentum was excised and ligated (figure 1). Rest of the procedure was uneventful.  

The excised omentum was opened and a Multiload was found encased in a collection of pus. The whole pus filled cavity was completely sealed off in the omentum forming an omental abscess. Pus was sent for culture and sensitivity which showed no organisms (figure 2 and 3). Her postoperative course was normal.

Figure 1. Excision of the omental abscess (OA) along with the encased IUCD (thread seen marked by the arrow). U is the uterus

Figure 2. Excised omental abscess with pus draining out.

Figure 3. Cut section of the omental abscess with the encased multiload IUCD. The threads of the IUCD are seen separate from the IUCD.

Discussion 

The common complications of IUCD’s are expulsion, perforation, infections, irregular bleeding and failure of the device resulting in pregnancy even with the device in situ.[1] 

In our case, user was not taught to check the thread after insertion. In a properly counseled user, a missing thread is the first sign of something amiss. Sometimes the thread can just be curled up in the cervix and diagnosed on probing with a brush or artery forceps but it can also mean expulsion or perforation. In such cases, USG would aid the diagnosis. The protocol for a missing IUCD was not followed in our patient.[3]

She subsequently conceived and was seen by a doctor for medical termination of that pregnancy. Expulsion of the device was just assumed and the ACOG recommended algorithmic approach for the management of lost IUCD was not followed to locate the device.[3] In the present pregnancy, the patient had 2 USG examinations done but since they were done in early pregnancy; they could not pick up the IUCD as they scanned the pelvic region only. Subsequently as the pregnancy advanced, the USG done in the third trimester scanned the upper part of the abdominal cavity and hence detected it, located in the sub-hepatic region. MRI whole abdomen confirmed the diagnosis of copper IUCD being outside the uterine cavity.

A painful insertion in a lactating and post partum woman had mostly led to a perforation during insertion of the device. Besides, failure of the contraceptive method she remained asymptomatic and the perforation remained silent for nearly 3 years as the omentum, so rightly labeled as the policeman of the abdomen, caught this foreign body and kept it safely away, from causing any complications till we accidentally discovered it. Fortunately, the site of uterine perforation did not compromise the integrity of the uterine wall. She underwent an MTP and a term pregnancy after this perforation without the perforation site giving way.

Perforation by an IUCD and its migration into the peritoneum is a known complication and occurs in 1/1000 insertions.[2] Usually painful insertion and missing thread are the first signs.[4] The precipitating factors for perforation are insertion in the post partum status and or a lactating mother.[4] Perforation of the uterus by an IUCD can be either primary( at the time of insertion) or secondary( due to contractions).[5] Perforation can also be classified as partial or complete. Partial is when the IUCD remains either within the cavity or the myometrium (Type 1 and 2) and complete is when the IUCD is in the peritoneal cavity (Type 3).[6] A type 3 perforated IUCD can intrude into the surrounding organs including the bladder, small intestine, sigmoid colon or the omentum.[7-9] This migration can either be completely asymptomatic (as in our case) or can cause range of symptoms right from abdominal pain, gastro-intestinal  bleeding, intestinal or ureteric obstruction and even bladder stones.[11,12] Massive hemoperitoneum on attempted removal of a perforated Cu-T has also been reported.[9] The copper in the Cu-T, is known to cause a tissue reaction resulting in dense adhesions and chemical peritonitis that was prevented in our case due to encasement of the device by the omentum.

The definitive management of such a perforated device is either open or laparoscopic removal.[9,13] But all studies and reports indicate that prompt removal of the device is a must upon detection.

Conclusion

Any method of contraception comes with its share of complications. However we, as health care providers, armed with the latest diagnostic techniques at our disposal, need to keep in mind and treat these complications accordingly. We need to constantly strive to not only reduce, detect and treat the complications but also prevent the complications by proper training for method use and counseling of users so that the usefulness of the method does not suffer.

References

1. Cunningham FG. Contraception. In Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, et al editors. Williams Obstetrics. 24th ed. USA: MC Graw Hill Education 2010; pp.1447-1448.
2. Clinical challenges of long-acting reversible contraceptive methods. Committee Opinion No. 672. American College of Obstetricians and Gynecologists. Obstet Gynecol 2016;128:e69–77
3. Julia Shefras with GRG Group, Management of lost intrauterine device threads.  Oxfordshire Clinical Commissioning Group.September 2012. http://www.ouh.nhs.uk/services/referrals/womens/gynaecology/documents/occg-lost-coil-threads-pathway.pdf
4. Heinonen PK, Merikari M, Paavonen J. Uterine perforation by copper intrauterine device. Eur J Obstet Gynecol Reprod Biol. 1984;17(4):257-61
5. Cederqvist LL, Lindhe BA, Fuchs F. Perforation of the uterus by the copper-t and copper-7 intrauterine contraceptive devices. Acta Obstet Gynecol Scand. 1975;54(2):183-9
6. Zakin D, Stern WZ, Rosenblatt R. Complete and partial uterine perforation and embedding following insertion of intrauterine devices. I. Classification, complications, mechanism, incidence, and missing string. Obstet Gynecol Surv. 1981;36(7):335-53.
7. Ikechebelu JI, Onwusulu DN. Missing IUCD string: prevalence, diagnosis and retrieval in Nnewi, Nigeria. Niger J Med. 2009;18(3):303-5.
8. Yang X, Duan X, Wu T. Ureteric Obstruction Caused by a Migrated Intrauterine Device. Urol Case Rep. 2016;10:33-35. eCollection 2017.
9. Chen CP, Hsu TC, Wang W. Ileal penetration by a Multiload-Cu 375 intrauterine contraceptive device. A case report with review of the literature. Contraception. 1998;58(5):295-304.
10. Eli SF, Olisa E, Abam DS, Enyindah CE. Transmigration of intra-uterine device, exploratory laparotomy, retrieval and repair of perforated uterus. Niger J Med. 2015;24(3):273-6.
11. Johri V, Vyas KC. Misplaced intrauterine contraceptive devices: common errors; uncommon complications. J Clin Diagn Res. 2013;7(5):905-7
12. Goldman JA, Feldberg D, Dicker D. Massive hemoperitoneum due to IUCD. Eur J Obstet Gynecol Reprod Biol. 1983;14(4):239-40.
13. Gardyszewska A, Niewiadomska-Kowalczyk M, Szymańska B, Roszkowski P, Czajkowski K. Extrauterine mislocated IUCD. Ginekol Pol. 2009 Dec;80(12):942-5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20120941

Citation

Deshpande PS, Pardeshi SH, Gupta AS. Omental Abscess Encasing A Lost Multiload. JPGO 2017. Volume 4 No.4. Available from: http://www.jpgo.org/2017/04/omental-abscess-encasing-lost-multiload.html

Uterine Graveyard :Rare cause For Secondary Infertility

Author Information 

Jaybhaye SB

(Consultant Obstetrician And Gynecologist, Department Of Advanced Gynaec. Endoscopy. Om Sai Hospital, Kamothe, Panvel, Maharashtra, India.)

Abstract

Intrauterine retention of fetal bones is a rare complication these days due to availability of good quality medicines for termination of pregnancy. But still there are areas in India where the procedures like aspirotomy or dilatation and evacuation is being tried for termination of pregnancy in advanced gestational age (12 to 20 weeks). There is a very high possibility of missing some bony fragments of the fetus inside the uterine cavity during this procedure. Such retained fragments can lead to formation of intrauterine adhesions, hypomenorrhea, pelvic inflammatory disease (PID), that in turn can lead to fertility issues like infertility. We present one such case managed at our center where patient presented for secondary infertility. Severe Asherman’s syndrome as well as retained fetal bony fragments were detected on evaluation. Surgical correction of these problems eventually corrected infertility.

Introduction

Retention of fetal bones inside the uterine cavity during medical termination of pregnancy, although a rare event to occur, can sometimes pose serious fertility concerns to the patient in question. The event goes unrecognized in most of the cases as patients do not exhibit any symptoms in the immediate postoperative period. Later on after few years many of these patients present with various complains like dysmenorrhea, chronic pelvic pain, and hypomenorrhea and sometimes with an inability to conceive. Infertility is one of the most unfortunate consequences of retained fetal bones. Proposed etiopathogenesis of infertility in such cases is either development of asherman’s syndrome as a reaction to retained bones [1] or the bones themselves acts like intrauterine contraceptive devises to prevent pregnancy.[1,2]

Case Report

A 32 year old woman P1L1A1 presented with secondary infertility since 6 years. She was  anxious to conceive and had scanty menses with severe dysmenorrhea since the last 2 to 3 years. In her obstetric history, she underwent an LSCS 10 years back. She underwent a medical termination of pregnancy (MTP) in her second pregnancy at 14 to 16 weeks of gestation for anencephaly 6 years back. Detail medical record of the procedure adopted for MTP were not available with her but as per history given by her a dilatation and evacuation was done to terminate pregnancy at some hospital in rural area of Jalgaon district In her menstrual history she has a regular 28 day cycle but bleeds scantily for 2 days and menstruation is painful. Her last menstrual period was 5 days prior to presentation.She gave history of 3 failed attempts for conception after ovulation induction and planned relations despite of documented ovulation on follicular study. Her past medical history was insignificant. On clinical examination her general and systemic examinations were normal. On local examination her abdomen was soft. On speculum examination cervix and vagina were healthy. On bimanual vaginal examination uterus was anteverted, normal in size, and adnexa was free.

Her serological, biochemical and day 3 hormonal profile were within normal range, Ultrasound (USG) of the pelvis showed thick calcified endometrium. Her husbands semen analysis was normal. A consent for diagnostic and if required operative hysterolaparoscopy to evaluate for the causes of secondary infertility was obtained from her and her husband. On hysteroscopic examination, impacted multiple bony fragments were detected in the endocervical canal and endometrial cavity.[Figure 1,2] Endometrial cavity was found to be obliterated with severe adhesions leading to asherman’s syndrome. [Figure 3] All bony fragments were removed with the help of hysteroscopic grasper’s [Figure 4, 5]] and adhesiolysis was done with hysteroscopic scissors. Laparoscopy that was done at the same time revealed normal findings with free spillage of dye from both fallopian tubes. Intrauterine Foleys’ catheter was kept for 5 days and she was started on estrogen supplementation for endometrial regeneration. As the hysteroscopically retrieved pieces of bones were grossly looking like fetal bones the histopathological confirmation was not done . She was advised to wait for spontaneous conception for at least 6 months and it was planned to start treatment in case she failed to conceive in 6 month period. She reported with a positive urine pregnancy test (UPT) in the 4th month following hysterolaparoscopy.

Figure 1. Bony fragments (arrow) in the endocervical canal. 

Figure 2. Bony fragments (arrow) in the endometrial cavity

Figure 3. Intrauterine adhesions

Figure 4. Retrieval of bones by hysteroscopic grasper

Figure 5. Retrieved bones 

Discussion 

Uterine and tubal factors are known to be the leading causes of infertility across the world especially more so in developing countries. Abortion has been known to be associated with future fertility problems in few cases. The proposed mechanism is likely to be result of damage to the fallopian tubes from infections or sometimes as a result of damage to the endometrial lining resulting in endometrial adhesions or synechiae formation leading to development of asherman's syndrome.[1]  One of the significant, although rare cause of uterine factor of infertility is retention of fetal bones left during a previous mid-trimester dilatation and evacuation. Besides secondary infertility, retained fetal bones may also present with irregular vaginal bleeding, dysmenorrhea, dyspareunia and chronic pelvic pain. It has been postulated that the fetal bones that are left behind in the uterine cavity following medical termination of pregnancy acts like an intrauterine contraceptive device (IUCD).[1,2] Sometimes they may also act like an adhesiogenic factor leading to development of intrauterine adhesions which in turn lead to secondary infertility. The exact incidence of retained intrauterine fetal bones is unknown. Most of the times it’s an incidental finding during routine hysteroscopic evaluation in a case of secondary infertility. A high index of suspicion is needed for the preoperative diagnosis to be made. Imaging modalities like 4 dimensional ultrasound, hysterosalphingography ( HSG) and hysteroscopy may make the diagnosis fairly simple. Filling defects on HSG may sometime give an indication of possibility of the diagnosis of this condition as a differential diagnosis to asherman’s syndrome. Simple removal of the retained  intrauterine fetal bones under hysteroscopic guidance is the gold standard for treatment of this condition.[2] Success rate in terms of resumption of normal fertility and the resolution of other symptoms can be very high as reported in majority of the studies.[1,2,3,4] 

References

1. Srofenyoh EK, Addison M, Dortey B, PA Kuffour PA. Intrauterine Retained Fetal Bones as a Cause of Secondary Infertility. Ghana Med J. 2006; 40(3): 105–109.
2. Tulandi T, Sammour A. Retained fetal bones in the uterine cavity. The Journal of the American Association of Gynecologic Laparoscopists. 2001; 8(2):179-180
3. Moon HS, Park YH, Kwon HY, Hong SH, Kim SK. Iatrogenic secondary infertility caused by residual intrauterine fetal bone after midtrimester abortion. Am J Obstet Gynecol. 1997;176(2):369-70.
4. Graham O , Cheng LC ,  Parsons JH. The ultrasound diagnosis of retained fetal bones in West African patients complaining of infertility. BJOG: An International Journal of Obstetrics & Gynaecology. 2000;107(1):122–124.

Citation

Jaybhaye SB. Uterine Graveyard :Rare cause For Secondary Infertility. JPGO 2017. Volume 4 No.4. Available from: http://www.jpgo.org/2017/04/uterine-graveyard-rare-cause-for.html

Vulvar Abscess in Autoimmune Disorder - A Clinical Challenge

Author Information

Rane V*, Samant PY**, Kamat S***, Daigavane MM****.

(* First year resident, ** Professor Additional, *** Second year resident, **** Assistant Professor. Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

                                                          

Abstract

A 36 year old woman was diagnosed with autoimmune disorder with multi system involvement. She presented with a large vulvar abscess extending to the groin, and perineum and also had inguinal lymph adenitis. Abscess was drained and dressings were followed by secondary suturing. The large abscess healed. Here we discuss autoimmune disease and extreme presentation of inflammatory pathology with reference to other autoimmune conditions with similar predilection.

Introduction

Vulvar abscess is a common gynecological problem. These abscesses most commonly originate as simple infection in the vulvar skin or subcutaneous tissue superficial to the fascia. Spread of the infection located in the vulvar area may occur easily due to the loose areolar tissue in the subcutaneous layers into adjoining fascial planes of the groin and anterior abdominal wall. The response to treatment in immunocompetent patients differs from those in patients with autoimmune disorders and immunodeficient cases. Such cases may require special investigations. We report a case of large vulvar abscess in a patient of rheumatoid arthritis.

Case Report

A 36 years old multiparous woman presented with sudden onset of severe pain in vulvar region, rapidly growing swelling over the left labium majus over a few days and fever with chills for a day. She did not report any significant injury, vaginal discharge preceding the occurrence of swelling. There was no history of similar lesions in the past anywhere else on the body. She also reported having joint pains and swelling of her wrists, knees and ankles for 3 years. She was taking ayurvedic medications for the same on and off. Her menstrual history was unremarkable. Her last menses were 4 days before presentation. She did not have diabetes mellitus. Her both deliveries were by cesarean section.

On physical examination, she was pale but afebrile. Pulse and blood pressure were normal. Local examination showed 10 x 4 cm tender and tense left labial swelling with erythema extending to perineal and perianal region and left inguinal lymphadenopathy. There was no vaginal extension. Bimanual examination was difficult. The affected joints were swollen, but there was minimal deformity of one wrist. Her hemoglobin was 5.9 gm%, and ESR was 45 at the end of 1 hour. Blood sugar, liver and renal function tests were within normal range. She was seronegative for syphilis, hepatitis B, hepatitis C and HIV. Her TSH was 9.48 mIU/ ml. Her anti TPO was < 0.8 U/ ml (normal up to 60 U/ ml). She was advised by endocrinologists to repeat TSH after 3 months and follow up. Local ultrasound showed ill-defined heteroechoic lesion in her perineal region, suggestive of early local abscess. Multiple enlarged inguinal lymph nodes on the left side were also seen.

Figure 1. Left labial swelling.

In view of anemia, three packed cell transfusions were given. Tetanus toxoid was administered. Incision and drainage of the abscess under saddle block was done after administering prophylactic amoxicillin-clavulanic acid and metronidazole. Extremely foul smelling pus was drained which was sent for culture and antibiotic sensitivity. 

Rheumatology opinion was taken for multiple joint pain and swelling. RA levels were  > 64 IU/ml. Hydroxylchloroquin 300 mg once a day and calcium supplements were started as per advice of the rheumatologist. In addition to prophylactic antibiotics, injection amikacin was started as per pus culture, which grew E. coli. It was given for 10 days. Anti-inflammatory drugs were given. Daily dressing of the wound was done with povidone iodine. Secondary suturing was done 2 weeks after abscess drainage when the wound looked healthy. A week after suturing, minimal discharge was seen from the suture line. Repeat wound swab culture was suggestive of insignificant polymicrobial growth. Suture removal was done after a week. Wound had healed well and looked healthy.  At 6 weeks follow up, she had vaginitis unrelated to surgery and on examination, minimal edema of the labium around the scar was noted. Vaginitis was duly treated.  

Figure 2. Healed wound.

Discussion 

Vulvar abscesses are not uncommon. Causes of vulvar abscess can range from skin and hair follicle infection to vulvar carcinoma. Bartholin’s gland cysts can progress to an abscess. Hidradenitis suppurativa of vulva may progress to an abscess. Bartholin’s gland cysts can progress to an abscess. Hematoma following an episiotomy may develop infection to form an abscess. Vulvar cancer can be mistaken for a vulvar abscess with acute onset of redness, swelling and severe pain.[1] If cancer is suspected, biopsy should be done. Many vulvar abscesses have mixed polymicrobial infection, which include staphylococcus aureus, streptococci, E. coli, methicillin resistant staphylococcus aureus.[2,3] In our case, pus grew E. coli. Immunocompetent hosts clear the infections more readily and recover with no sequelae. Immunocompromised hosts fail to mount an immune response, which results in infectious process getting exacerbated. Lesions that initially look innocuous may progress to possibly fatal necrotizing fasciitis.[4] If the patient shows predilection for recurrent abscesses, underlying immune mediated pathology should be investigated. Elevated IgE levels may indicate atopy and very high levels may be suggestive of hyper IgE syndrome. White cell phenotyping, Interferon pathway analysis and other specialized investigations may be required for diagnosis of underlying immunological conditions.[5] In rare cases even after surgical drainage, infection may progress to necrotising fasciitis; a condition with high mortality.[6]                                      Our patient had symptomatic rheumatoid arthritis; but was not on any medication for a long time. She also had a raised TSH level and severe anemia without menorrhagia or any other predisposing factor; probably of autoimmune origin. Rheumatoid arthritis is known to have a host of cutaneous manifestations like vasculitis, granulomatous dermatosis and neutrophilic dermatosis. These are caused by activation of complement cascade by immune complexes. Neutrophils and lymphocytes are recruited and vasculitis of subcutaneous small vessels may cause recurrent ulcers. Suppurative hidradenitis may be one of the cutaneous manifestations in PASH (Pyoderma gangrenosum, acne and suppurative hydradenitis) syndrome in rheumatoid arthritis.[7]

Conclusion

It is important to bear in mind possibility of underlying immunological condition in cases with atypical presentation of abscesses. Timely treatment of the immunological condition can help in reducing morbidity.    

References

1. Nayak AU,  Sundari N., Nandini G. Wolf in lamb's skin: Vulval carcinoma mimicking bartholin gland abscess. Indian J Sex Transm Dis. 2009; 30(1): 46–47.
2. Bhide A, Nama V, Patel S, Kalu E. Microbiology of cysts/ abscesses of Bartholin's gland: review of empirical antibiotic therapy against microbial culture. J Obstet Gynaecol 2010; 30(7): 701-3.
3. Thurman AR, Satterfield TM, Soper DE. Methicillin-resistant Staphylococcus aureus as a common cause of vulvar abscesses. Obstet Gynecol 2008;112(3):538-44
4. Stephenson H, Dotters DJ, Katz V, Droegemueller W. Necrotizing fasciitis of the vulva. Am J Obstet Gynecol 1992;166(5):1324-7.
5. Johnston SL. Clinical Immunology Review Series: An approach to the patient with recurrent superficial abscesses. Clin Exp Immunol 2008; 152(3): 397–405.
6. Kdous M, Hachicha R, Iraqui Y, Jacob D, Piquet PM, Truc JB. Necrotizing fasciitis of the perineum secondary to a surgical treatment of Bartholin's gland abscess. Gynecol Obstet Fertil 2005;33(11):887. 
7. Marzano A.V. Cutaneous manifestations in rheumatoid arthritis. Clinical Dermatology 2013;1(2): 53-59.

Citation

Rane V, Samant PY, Kamat S, Daigavane MM. Vulvar Abscess in Autoimmune Disorder - A Clinical Challenge. JPGO 2017. Volume 4 No. 4. Available from: http://www.jpgo.org/2017/04/vulvar-abscess-in-autoimmune-disorder.html

Use Of Immunosuppressive Drugs For Systemic Lupus Erythematosus In Pregnancy

Author Information

Satia M*, Shilotri M**, Panchbudhe S***.

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

                                            

Abstract

Systemic lupus erythematosus (SLE) is a chronic disease which causes inflammation of the connective tissues. Women are affected during their childbearing period. Pregnancy in patients with SLE was discouraged not only due to concern regarding the poor fetomaternal outcomes but also due to the increase in disease activity during pregnancy. Drugs like cyclophosphamide, methotrexate, steroids & mycophenolate are used for the treatment of SLE. Several of these are contraindicated in pregnancy due to their teratogenicity and they should be stopped at least three months prior to conception. We report a case of SLE who had received cyclophosphamide during the first trimester of pregnancy and hence underwent termination of pregnancy.

Introduction

Systemic lupus erythematosus (SLE) is a type of autoimmune and chronic inflammatory disorder affecting multiple organ systems and is characterized by exacerbation's and remissions. It is thought to be caused by polyclonal B-cell activation that leads to antinuclear antibody production against native tissue. These antibodies can cause direct damage to tissues. There is also widespread deposition of immune complexes in vascular beds with impairment of T- cell regulation causing a failure to remove these immune complexes.[1] This leads to activation of the complement system and inflammation. Thus, the mainstay of treatment of SLE is with immunosuppressive and anti-inflammatory drugs. The drug of choice depends on the disease activity and the patient’s response to therapy. A wide spectrum of anti-inflammatory and immunosuppressive drugs like non-steroidal anti-inflammatory drugs (NSAIDs), steroids, methotrexate, mycophenolate mofetil, cyclophosphamide, azathioprine and cyclosporine are used for the treatment of SLE. Most of these drugs cross the placenta and have teratogenic potential. Women with SLE should be seen in the pre-pregnancy period not only to ascertain the suitability of conception depending on the level of the disease activity but also to check whether they are on any anti-inflammatory or immunosuppressive drugs.

Case Report

A 26 year old gravida 3, para 1, living 1, abortion 1 with 7.6 weeks gestation was referred to our antenatal outpatient department by the rheumatologist for further management. She was a known case of beta thalassemia trait, systemic lupus erythematosus with antiphospholipid antibody syndrome, and  autoimmune hemolytic anemia on pulse cyclophosphamide therapy. On examination, her general condition was fair, pulse and blood pressure were normal. She had no pallor, icterus, cyanosis or pedal edema. Cardiovascular and respiratory system examination were unremarkable. The abdomen was soft, there was no guarding, rigidity or tenderness and the Pfannenstiel scar of a previous lower segment cesarean section was seen. On per speculum examination the cervix and vagina were healthy. On per vaginal examination the uterus was 6-8 weeks in size, anteverted, os was closed and bilateral fornices were clear. She had received the last dose of pulse cyclophosphamide therapy around the time of conception; 2 weeks after her last menstrual period. She had also taken atorvastatin and telmisartan in the first trimester.

She was diagnosed to have systemic lupus erythematosus with autoimmune hemolytic anemia with beta thalassemia trait 1 year prior during the evaluation of severe anemia and jaundice. She had presented with hemoglobin of 2.2 gm % and bilirubin of 2.3 mg/ dl. Her anti-nuclear antibodies (ANA),  lupus anticoagulant and antiphospholipid antibodies were all positive. Bone marrow aspiration and biopsy showed erythroid hyperplasia with hemolytic process. She was transfused with 8 units of packed red cells and started on pulse methylprednisolone therapy, pulse cyclophosphamide therapy, oral hydroxychloroquine, aspirin, atorvastatin and telmisartan. The DEXA scan was suggestive of osteoporosis and hence she was started on vitamin D and calcium supplements. Her hematological parameters improved gradually. She conceived despite her and her husband being counseled about the need to avoid pregnancy during the immunosuppressant therapy.

As per the rheumatologist’s advice, a complete evaluation was done to determine her disease activity. Complete blood count, liver and renal function tests, fasting, postprandial blood sugars, serum complement (C3, C4) levels, serum C-reactive protein (CRP) levels, serum lipids, thyroid function tests and 24 hour urine protein levels, were all in their respective normal ranges. A cardiology evaluation and an echocardiogram were done to rule out any cardiac lesions due to autoimmunity and cyclophosphamide therapy. The echocardiogram was normal. The couple was counseled regarding the risk of adverse effects on the fetus of the cyclophosphamide therapy received in the periconceptional period and the available modalities for screening of fetal anomalies. They opted for termination of pregnancy. First trimester medical termination of pregnancy was done by suction evacuation under total intravenous anesthesia. The procedure was uneventful. Post-procedure she was referred to the rheumatologist for further plan of action. The couple was counseled to avoid conception until the end of immunosuppressant therapy and they chose to use barrier contraception.

Discussion

Pregnancy in patients with SLE is a high risk condition. This condition is associated with an increased risk of maternal and perinatal morbidity and mortality. Active SLE in the early gestational period is associated with adverse pregnancy outcomes. The longer the woman is in remission, the better is the pregnancy outcome. Hence counseling prior to conception is mandatory so that pregnancies can be planned during periods of disease remission. Also, a detailed drug history is necessary in order to take a decision of continuation or changeover of the drugs depending on disease activity and teratogenicity of the drugs. There is a small to moderate risk of birth defects due to NSAIDs such as cleft lip/ palate, neural tube defect, eye defects, pulmonary valve stenosis and limb defects. However, there is no major risk for their use in early pregnancy.[2] Hydroxychloroquine is an antimalarial drug that has anti-inflammatory properties and helps maintain disease remission. It does not cause congenital malformations and its continuation through pregnancy is recommended.[3] Exposure to steroids should be limited to a minimum during pregnancy as higher doses are associated with an increased risk of diabetes, hypertension, pre-eclampsia and premature rupture of membranes. There are no major congenital malformations associated with steroids but risk of oral clefts and intrauterine growth restriction is increased.[4,5] The immunosuppressant drug azathioprine is one of the few agents that is safe during pregnancy and is used in the dose of 2 mg/kg/day, to avoid risk of fetal cytopenias and immune suppression.[6] The fetal liver does not have adequate levels of an enzyme inosinatopyrophosphorylase, which is required for the conversion of azathioprine to its active form, and therefore is protected from its harmful effects.[7] Other immunosuppressive drugs like the calcineurin inhibitors, tacrolimus and cyclosporine, are not reported to have any increase in fetal risk.

Cyclophosphamide is proven teratogenic and cyclophosphamide embryopathy includes ear and facial abnormalities, defects of the calvaria, absence of digits and hypoplastic limbs.[8] It also causes growth restriction and suppression of neonatal hematopoiesis. In some cases, mycophenolate is the only treatment that achieves disease stability and patients should be counseled regarding the fetal risks such as external auditory canal atresia with and without microtia, tracheo-esophageal atresia, severe hydronephrosis, atrial septal defect and myelomeningocele. Drugs such as cyclophosphamide, methotrexate and mycophenolate are contraindicated during pregnancy and should be stopped at least 3 months before conception.[6] The half-life of these drugs is very long and their effects may be seen up to a few weeks to months after discontinuation. Thus, women need to be counseled regarding the need for contraception during the period of immunosuppressant therapy and for 3 months thereafter. Barrier contraception and progesterone only pills or depot progestogens are safe in SLE. Low dose combined oral contraceptive pills (COCs) may be used with caution in selected cases of SLE.[9] In case a woman conceives while on teratogenic immunosuppressant drugs, she should be counseled regarding the measures that can be taken to antenatally detect any fetal anomalies. Ultrasound for nuchal translucency and nasal bone, detailed anomaly scan and fetal 2D echo, double, triple and quadruple markers, amniocentesis, chorionic villus sampling are the methods that can be used. However, she must be told that these measures are not foolproof. If the tests show that the fetal condition is untreatable or associated with significant handicap then termination of pregnancy may be suggested. Alternatively, the antenatal management, time, place and mode of delivery may be planned in order to ensure the optimal prognosis of the neonate.

Conclusion

Management of pregnancy with SLE requires a multidisciplinary approach involving the obstetrician and rheumatologist. Stress should be laid on pre-pregnancy counseling and history of use of drugs, especially during the first trimester of pregnancy. The need to use contraception must be emphasized while patients are taking teratogenic medications.

References

1. Mok CC, Lau CS. Pathogenesis of systemic lupus erythematosus. J Clin Pathol. 2003; 56(7):481-490.
2. Hernandez RK, Werler MM, Romitti P, Sun L, Anderka M. Nonsteroidal antiinflammatory drug use among women and the risk of birth defects. Am J Obstet Gynecol 2012; 206(3):228:e221-8.
3. Parke A, West B. Hydoxychloroquine in pregnant patients with systemic lupus erythematosus. J Rheumatol. 1996; 23(10):1715–1718.
4. Rodriguez-Pinilla E, Martinez-Frias ML. Corticosteroids during pregnancy and oral clefts: a case-control study. Teratology 1998; 58(1):2–5.
5. Reinisch JM, Simon NG, Karow WG, Gandelman R. Prenatal exposure to prednisone in humans and animals retards intrauterine growth. Science 1978; 202(4366):436–8.
6. Østensen M, Khamashta M, Lockshin M, Parke A, Brucato A, Carp H et al. Anti-inflammatory and immunosuppressive drugs and reproduction. Arthritis Res Ther 2006; 8(3):209.
7. Polifka JE, Friedman JM. Teratogen update: azathioprine and 6-mercaptopurine. Teratology 2002; 65(5):240–61.
8. Kirshon B, Wasserstrum N, Willis R, Herman GE, McCabe ER. Teratogenic effects of first-trimester cyclophosphamide therapy. Obstet Gynecol 1988; 72(3 Pt 2):462–4.
9. Lateef A, Petri M. Hormone replacement and contraceptive therapy in autoimmune diseases. J Autoimmun 2012; 38(2-3):J170-6.

Citation

Satia M, Shilotri M, Panchbudhe S. Use Of Immunosuppressive Drugs For Systemic Lupus Erythematosus In Pregnancy. JPGO 2017. Volume 4 No.4. Available from: http://www.jpgo.org/2017/04/use-of-immunosuppressive-drugs-for.html

Pendulous Abdomen Causing Levorotation of Uterus And Transverse Lie

Author Information 

Prabhu S*, Mishra N**, Savani G***, Tintoiya I****

(* Senior Consultant, ** Head of Department, *** Consultant, **** Resident Medical Officer Department of Obstetrics and Gynecology, Bhabha Atomic Research Centre Hospital, Mumbai, India.)

Abstract

Torsion of gravid uterus is a rare and a ‘once in a lifetime’ diagnosis for a gynecologist. We present an asymptomatic, antenatally undiagnosed case of uterine torsion that was detected at elective lower segment cesarean section (LSCS). It was well managed surgically avoiding the usually expected complications resulting in a good maternal and neonatal outcome.

Introduction

Rarely does a gynecologist get to see a case of uterine torsion, for many it is once in a life time opportunity, for others they never see this condition.[1]  Minor degrees of rotation of gravid uterus about its longitudinal axis are physiological.[2] Uterine torsion is considered when pathological rotation occurs on its long axis of more than 45 degrees.[3] Though the phenomenon of gravid uterine torsion is commonly seen in animals, it is rare in humans. We report a case of uterine torsion which remained asymptomatic and was detected only at elective LSCS.

Case Report

A 30 year old G2 P1 L1 with previous LSCS was registered for antenatal care at our institute. On her ANC visit at 37 weeks, patient had no complaints. Her vital parameters were normal. Obstetric examination revealed pendulous abdomen with poor tone, and a Pfannenstiel scar of previous LSCS. Empty Pawlik’s grip suggested transverse lie of the fetus. Fetal heart sounds were regular at 146 beats/minute. Uterus was relaxed and there was no scar tenderness. She had history of LSCS performed 4 years back for non-progress of labor and fetal distress. Her current pregnancy was otherwise uncomplicated. Her ANC investigations were normal and 3 obstetric ultrasonographic scans (USG) performed in each trimester were normal.

The clinical findings of transverse lie were confirmed by USG which showed fetus in transverse lie with cephalic pole in the right hypochondrium (right dorsoinferior position). Liquor was adequate and placenta was anterior. Elective LSCS was planned at 38 weeks in view of previous LSCS with transverse lie. At LSCS, abdomen was opened with Pfannenstiel incision. After opening the parietal peritoneum, tortuous vessels were visible in the center on the lower part of the uterus. There were no adhesions due to previous surgery. Prior to planning uterine incision, routine anatomical delineation of round ligaments was performed, as palpation of round ligaments and centralization of uterus is our routine practice in all LSCS cases. We noticed that the cornual structures of the right side were more towards the left crossing the mid-line. Right ovarian ligament was seen superiorly in the midline, tortuous vessels seen were identified as the right mesosalpingeal vessels and right sided round ligament was seen inferiorly. Considering the right lateral wall of the uterus facing anteriorly, pathological rotation of the uterus and uterine torsion was suspected. (Figure 1). The fetus was in transverse lie with cephalic pole in the right hypochondrium and dorso-inferior position. (Figure 2)

Figure 1. The right sided adnexal structures; ovarian ligament (yellow arrow), fallopian tube (black arrow), round ligament (blue arrow) seen at LSCS.

Figure 2. Diagrammatic representation of fetus in transverse lie (dorso-inferior) with head in right hypochondrium.

Uterine position was corrected manually without difficulty. After correction of uterine torsion the fetal presentation changed from transverse lie to longitudinal lie (floating vertex). Utero-vesical fold of the peritoneum was identified, opened transversely and bladder was retracted with Doyens' retractor. Lower uterine transverse incision was taken. Liquor was clear. A 3.415 kg, male child was delivered by vertex. Baby cried immediately after birth and 1 minute APGAR was 9/10.

Figure 3. Post LSCS view of uterus showing normal cornual structures, anterior lower  uterine segment incision with well dissected utero-vesical fold of peritoneum

The placenta which was detected to be located anteriorly on preoperative USG was found to be fundo-posterior during LSCS. After uterine closure, bilateral tubal ligation was performed as the couple had opted for it and abdomen was closed in layers. Post operative period was uneventful. Both mother and baby were discharged on post operative fifth day.

Discussion

Physiological, slight degree of rotation of the pregnant uterus is seen to the right in 80% and towards the left in 20% cases.[3] Uterine torsion is considered when rotation of the uterus occurs on its long axis of more than 45 degrees.[3] Predisposing factors may include uterine anomalies, fibroids, adhesions, abnormal pelvis, placenta previa or malpresentation, especially transverse lie.[3] In 20% cases, no causative factor is apparent.[3] 

In our case, though the patient had previous history of cesarean section, there were no adhesions at the time of present LSCS. On evaluating intra operative findings in our case we presume that acute anteversion of the uterus followed by pathological torsion towards left must have occurred as the right sided round ligament was seen inferiorly and ovarian ligament was seen superiorly crossing the midline towards the left side. (Figure 1)

Acute anteversion of the gravid uterus occurs with a pendulous belly. Malpresentations are naturally more common in these cases. The torsion does not occur in case of an anatomically normal uterus.[2]

On evaluating the cause of torsion in our case, we realized pendulous maternal abdomen must have caused acute anteversion of the gravid uterus leading to transverse lie, which further must have contributed as predisposing factor for the torsion. We think transverse lie was consequence and not the cause; and pendulous abdomen was the possible cause of uterine torsion. 

Acute torsion compromises the uterine circulation, hence can present as acute abdominal crisis with pain, shock, bleeding as in case of abruptio placentae. Torsion during labor can lead to obstructed labor and its severe complication of uterine rupture.[3]

The overall maternal mortality rate (MMR) associated with the torsion of gravid uterus is about 13 %. It is directly related to the duration of gestation and degree of twisting. Before 5 months it is 0 % while at term it reaches 18.5 %. In 1951, MMR was 7.4% with 90-180 degrees which rose to 50% with 180-360 degrees of torsion. After 1976, there is no reported maternal mortality.

The perinatal mortality also increases with degree of torsion. Between 90-180 degrees it is 20-24 % and may reach as high as 75 % when rotation is more than 180 degrees. Fetal mortality rate reported by Jensen between 1876-1992 was 12 % (212 cases) while between 1996-2006 was 18 % (38 cases).[4]

Our patient was asymptomatic and as the degree of torsion was 90-135 degrees to the left (levorotation), there was no compromise of uterine blood supply. Hence, maternal and neonatal outcomes were unaffected. Antenatal diagnosis by imaging USG is difficult. Nicholson et al have suggested X-shaped configuration of upper vagina on MRI as a sign to diagnose uterine torsion, which is normally visualized as a ‘H’ shaped structure.[5]

An unexpected uterine torsion at term was found during cesarean section by Kremer et al. In their reported case, a changing placental localization on ultrasound from the left to the right side was recorded just before the operation. They suggested this may be a useful sign to diagnose uterine torsion earlier.[1]

As our patient was registered for antenatal care, she had 3 ultrasonographies performed in each trimester. Retrospectively, we analyzed placental localization. It was posterior till mid-trimester (18 weeks) USG, which then was noted anteriorly at and after 28 weeks scan. This finding and its significance was overlooked antenetally, as USG’s were performed by different sonologists. We can now consider this as the time of torsion; after mid-trimester.

Usually uterine torsion is found on laparotomy or at cesarean section. If  recognized prior to fetal delivery then manual correction of torsion followed by delivery of fetus is the treatment option.[6] Wilson D et al reviewed the literature of uterine torsion during pregnancy and found posterior hysterotomy is usually performed inadvertently due to non-recognition of torsion or deliberately due to impossible detorsion. The anatomical land marks should be defined prior to uterine incision, to prevent injuries to other organs and blood vessels.

In our present case, inadvertent injury to the blood vessels and hysterotomy at sites other than the anterior lower uterine segment was prevented by routine practice of palpation of bilateral round ligaments making us aware of this condition and allowing the correction of rotation of the uterus prior to the uterine incision. 

As the couple opted for permanent method of sterilization the chance of recurrence of torsion in future pregnancy is ruled out. 

Conclusion

Gravid uterine torsion is rare in humans. The clinical presentation of the condition is variable from asymptomatic to acute abdomen. Clinical examination and ultrasonography may not be sufficient for establishing the diagnosis antenetally. Most of the time the diagnosis is made at the time of laparotomy. But it is very important to identify the uterine torsion prior to uterine incision for reducing intraoperative complications, maternal and fetal morbidity and mortality. Incorporating routine practice of palpation of bilateral round ligaments and correction of rotation of uterus prior to uterine incision at LSCS can diagnose pathological degrees of uterine rotation and prevent inadvertent injury to the blood vessels, other organs and hysterotomy at sites other than the anterior lower uterine segment respectively. Hence, we strongly recommend this (palpation and correction) technique as routine practice at the time of LSCS.

References

1. Kremer JA, van Dongen PW, Torsion of the pregnant uterus with a change in placental localization on ultrasound; a case report. Eur J Obstet Gynecol Reprod Biol. 1989; 31(3):273-5.
2. Donald I. Local abnormalities. Donald I. Practical Obstetric Problems.5th ed.New Delhi: B.I.Publications Pvt.Ltd; Reprinted 1998, pp 262-63.
3. Pascali D. Uterus and vagina. Posner GD, Jessica DY, Black AY, Jones GD. eds. In Oxorn -Foote. Human Labor and Birth, 6th ed. India; McGraw Hill Education. 2013; Pg 34-5.
4. Goran A. Torsion of the gravid uterus. Goran A. ed. In Acute abdomen during pregnancy. Springer International Publishing Switzerland; 2014. pg 533.
5. Nicholson WK, Coulson CC, McCoy MC, Semelka RC. Pelvic magnetic resonance imaging in the evaluation of uterine torsion. Obstet Gynecol 1995;85(5Pt2):888-90.
6. Wilson D, Mahalingham A, Ross S. Third trimester uterine torsion: case report. J.Obstet Gynecol Can. 2006; 28(6):531-5.

Citation

Prabhu S, Mishra N, Savani G, Tintoiya I. Pendulous Abdomen Causing Levorotation of Uterus And Transverse Lie. JPGO 2017. Volume 4 No.4. Available from: http://www.jpgo.org/2017/04/pendulous-abdomen-causing-levorotation.html