Volume 1 Issue 10, October 2014

Editorial
Parulekar SV

Paraganglioma of Bladder in Pregnancy
Fernandes G, Parulekar SV.

Hemoperitoneum from Ruptured Leiomyoma
Mirchandani A, Parulekar SV, Dalvi P.

Chronic Non Pueperal Inversion Of Uterus Secondary To Submucosal Fundal Myoma
Bakre T, Gupta AS, Hira P, Parulekar SV.

Successful Pregnancy Outcome In Severely Immunocompromised Patient
Prasad M, Chamariya S, Khadkikar K, Chauhan AR.

Placental Site Nodule
Mali K, Fernandes F, More V, Satia MN.

Torsion of Paraovarian Cyst in Pregnancy
Valvi D, Parulekar SV, Fernandes G.

Posthysterectomy Fimbrial Prolapse
Patil A, Parulekar SV, Fernandes G.

Prolapsed Leiomyoma Or Uterine Inversion: Radiology Doesn't Always Help
Mayadeo NM

Fimbrial Prolapse: Diagnostic Clinical Test
Parulekar SV

Quiz October 2014

Editorial

Parulekar SV

Nonpuerperal chronic inversion of the uterus is as much a rarity as a curiosity. Most of the clinicians go through a busy lifetime without ever seeing a case. There are fewer than a couple of hundred case reports in the world literature. It is curious that there were enough cases to go round in the past that Hippocrates wrote about it, Themison (50 BC) advised amputation of the bleeding and sloughing inverted corpus of the uterus, Soranus actually did it 150 years later, and methods were described for surgical correction of the inversion by stalwarts like Aran, Marion Sims, Barnes, Thomas, Browne, Kustner, Piccoli, Morisani, Spinelli, Haultain, Dobbin, and Huntigton. One wonders if any of them had sufficient number of cases to show the merits and demerits of any given method. It is equally doubtful if anyone in modern times has sufficient experience to recommend one method over another. The conditions which predispose to nonpuerperal chronic inversion of the uterus have not changed over years, because they are gynecological conditions which are not preventable, as can be said about obstetric inversions. Hence incidence of this condition has remained more or less constant over years, while obstetric inversions have declined. The diagnosis of this condition is not easy. In fact, the dictum ‘what looks like a chronic inversion of the uterus clinically will be anything but that’ is true even today. It will most probably be a leiomyomatous polyp, with or without uterine prolapse. With advances in imaging like computerized tomography and magnetic resonance imaging, the diagnosis of a chronic inversion is more likely to be made accurately. In this issue we present two cases, one of nonpuerperal chronic inversion which was diagnosed accurately with such imaging, and another which was falsely diagnosed so. Opinion is divided over the best method to treat such cases. I feel that a vaginal technique is preferable to an abdominal technique for a number of reasons. The first reason is that a chronic inversion is often associated with a neoplasm of the uterine fundus, most commonly a leiomyoma. It needs to be removed first, so that the inverted uterus can be put back in original shape and position. That can be done best by the vaginal route. The second reason is that the constricting of the cervix is low down and is approached more easily vaginally than abdominally. One ends up cutting a lot of wall of the uterine corpus before dividing the ring by the abdominal route. The third reason is that a trained gynecologist is more comfortable by the vaginal route and resorts to the abdominal route only when vaginal surgery cannot be done. Amongst the vaginal operations, Kustner’s operation is safer, because it involves dividing the cervical ring posteriorly, which is a lot safer than any operation that divides the ring anteriorly, with the inverted urinary bladder so close. Modern surgeons want to do everything by the endoscopic method, and there are a few reports of laparoscopic correction of chronic inversions. The vaginal route has the same advantages over the laparoscopic route as over the abdominal route. The only advantage of the laparoscopic route would be to make a correct diagnosis before cutting into any tissues, in case the imaging techniques have not helped make a correct diagnosis.
We have added one more feature to the journal for the benefit of postgraduate students and those readers interested in continuing medical education. A quiz is added at the end of the journal. There are multiple choice questions of the best one answer out of four type. They are based on the articles published in the current issue of the journal. The answers will be given in the next issue. We request the readers not to send us their answers, but check them themselves.

Paraganglioma of Bladder in Pregnancy

Author Information

Gwendolyn Fernandes*, SV Parulekar**

(* Associate Professor, Department of Pathology, ** Professor and Head, Department of Obstetrics & Gynecology, Seth GS Medical College & KEM Hospital, Parel, Mumbai, India.)

Abstract

A paraganglioma of the urinary bladder is a rare tumor that develops from the chromaffin tissue of the sympathetic nervous system situated outside the adrenal medulla. It is even more rare in pregnancy. We present a rare case that became symptomatic only in successive pregnancies.

Introduction

A paraganglioma, also known as extra-adrenal pheochromocytoma, is an extra-adrenal tumor. It develops from the chromaffin tissue of the sympathetic nervous system  outside the adrenal medulla. It can cause severe hypertension and its deleterious effects onthe mother and fetus both. Diagnosis and management of a paraganglioma of the urinary bladder may be more difficult in pregnancy.

Case report

A 33 year old woman, married for 3 years, gravida 2 para 1 living 0, with 3 months of amenorrhea, presented with frank hematuria and occasional micturition syncope during the last 6 days. There was no history of any pain in abdomen or from loin to groin, and no dysuria. She gave a history of similar occurrence during her last pregnancy a year ago, which had been complicated by severe hypertension, convulsions, and had ended in a stillbirth. The symptoms had resolved after the childbirth, and had not recurred until 6 days ago. Her general condition was fair, pulse rate 68/min, respiratory rate 18/min, and blood pressure 120/70 mm Hg. Systemic examination revealed no abnormality. The uterus was of 12-14 weeks’ size. Speculum examination showed no abnormality. Vaginal examination showed a closed cervix, and a 3x1.5x1.5 cm transverse, nontender mass in the area of the urinary bladder, behind the pubes. A diagnosis of a bladder mass was made. Abdominal and pelvic ultrasonography (USG) showed a 6.5x5.5 cm sized mass in the inferior wall of the bladder, a 10.8x5.6 cm hematoma within the bladder and an intrauterine gestation of 12 weeks. Magentic resonance imaging (MRI) of the abdomen and pelvis showed a 5.3x4.8x5.4 cm mass in the left and inferior to the urinary bladder, a blood clot in the bladder, and an intrauterine gestation of 12 weeks. There was no mass in the adrenal glands. A self retaining urinary catheter was passed into the bladder. Plasma metanephrine level was found to be 126 picogram/ml. Hemogram, fasting and postprandial blood sugar levels, liver and renal function test results were normal, except that her hemoglobin level was 9 g/dL.

Her blood pressure showed episodic elevation to 149/90 mm Hg over a number of days. She was administered amlodipine 5 mg qd, prazosin 2.5 mg qd by the endocrinologist as a preventive measure, along with adequate hydration and salt intake. 17 alpha hydroxyprogesterone caproate 250 mg deep IM was administered to prevent an abortion triggered by operation on the bladder. Two units of packed red cells were transfused. Transurethral biopsy of the tumor was done. Its histopathology showed it to be a paraganglioma of the bladder. MRI brain showed no metastases. Partial cystectomy along with excision of the paraganglioma was done. After insertion of DJ stents into the ureters and placing a suprapubic drain, the bladder was reconstructed. The abdomen was closed after placing two intraperitoneal drains. The patient’s blood pressure rose to 190/110 mm Hg during surgery, but came down to 90/60 mm Hg after surgery. She had to be administered a dopamine infusion to maintain her blood pressure for 12 hours. She made an uneventful recovery thereafter.

The tumor was unencapsulated, measuring 8x6x4 cm with a dark brown color. Smooth muscle fibres of the urinary bladder could be identified at the periphery. The formalin fixative used to fix the specimen had a light brown color (figure 1). Histology showed the tumor to be composed of large sheets of basophilic polygonal neoplastic cells situated amidst the muscularis propria of the urinary bladder. The sheets had a focal Zell Ballen pattern i.e. nest of cells surrounded by a thin fibrovascular stroma. Individual cells had moderately pleomorphic round nuclei with prominent nucleoli and granular basophilic cytoplasm. The diagnosis was a paraganglioma of the urinary bladder (figures 2, 3, and 4).

Figure 1. Paraganglioma of the urinary bladder. Characteristic dark brown colored tumor measuring 8 x 6 x 4 cm. Lighter colored smooth muscle fibers of the urinary bladder are seen in the lower portion.

Figure 2. Large sheets of basophilic chromaffin tumor cells surrounded by muscle fibers. (H & E X 100)

Figure 3. Another microphotograph showing nests of basophilic tumor cells splaying the muscularis propria of the urinary bladder. (H & E X 100)

Figure 4. High power view of the chromaffin cells showing characteristic Zell Ballen pattern. i.e. small nest of cells showing alveolar arrangement. (H & E X 400)

The patient received adequate antenatal care. She had some incontinence of urine up to term, which was explained to be due to reduced capacity of the bladder and urge incontinence. She underwent a cesarean section at 38 weeks of gestation for a breech presentation and uteroplacental insufficiency, delivering a baby weighing 2.8 kg. Postoperatively she was put on darifenacin 7.5 mg qd. She was advised regular follow up examinations to detect any recurrence.

Discussion

A paraganglioma, also known as extra-adrenal pheochromocytoma, is an extra-adrenal tumor that develops from the chromaffin tissue of the sympathetic nervous system situated outside the adrenal medulla.^[1] It accounts for 6% of all paragangliomas and less than 0.05% of all bladder tumors.^[2] It is more common in women than in men ( 3:1), mainly in the age group 30-50.^[3] About 17% of them are hormonally inactive.^[4]

Its clinical features include headache, palpitations, sweating, and fainting (especially with micturition), paroxysmal hypertension and hematuria. USG detects it in 8% cases, showing a submucosal homogeneous, well delineated, vascular mass, sometimes with   necrosis at its center. MRI in diagnostic in almost 100% cases, also defining its extension accurately.^[5,6] Cystoscopy both shows the mass and permits transurethral resection. A biopsy is not recommended, as it can cause serious hemorrhage, hypertensive crisis, and its diagnostic rate is low. The histological features of the tumor are as described in this case. About 5-15% cases are malignant. Histology cannot distinguish between benign and malignant tumors, and only local invasion or distant metastases are diagnostic of malignancy.^[7,8] Transurethral resection is adequate if the tumor is less than 2-3 cm in size and does not infiltrate deep.^[9] In all other cases open surgery is preferred. Pregnancy does not contraindicate the diagnostic and therapeutic approaches discussed. It is a unique situation in that the hypertension caused by the tumor may be mistaken for toxemia of pregnancy.^[10] Our case was unique in that she had hematuria suggestive of the presence of a bladder mass even in her past pregnancy associated with hypertension, presumably due to the paraganglioma, which did not cause any symptoms in between pregnancies. This then would be the first case of a paraganglioma of the bladder that became manifest during successive pregnancies only. This patient did not have any family history of such a disorder. However she was counseled to have the family members screened inview of about 10% cases being familial or genetic in nature.^[11] She was also advised to have at least one follow-up every year, since recurrences as late as 10 years are known to occur.^[12]

References

1.        Elder EE, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol. 2005;89:193-201.

2.        Pastor-Guzmán JM, López-García S, Giménez-Bachs JM, Ruíz-Mondejar R, Cañamares-Pabolaza L, Atiénzar-Tobarra M, et al. Paraganglioma of the bladder: controversy regarding treatment. Urol Int. 2004;73:270–275.

3.        Yadav R, Das AK, Kumar R. Malignant non-functional paraganglioma of the bladder presenting with azotemia. Int Urol Nephrol. 2007;39:449–451.

4.        Kontani K, Okaneya T, Takezaki T. Recurrent granular cell tumour of the bladder in a patient with von Recklinghausen’s disease. BJU Int. 1999;84:871–872.

5.        Vahidi K, Joe BN, Meng M, Coakley FV, Yeh BM. Review of atypical pelvic masses on CT and MRI: expanding the differential diagnosis. Clin Imaging. 2007;31:406–413.

6.        Vyas S, Kalra N, Singh SK, Agarwal MM, Mandal AK, Khandelwal N. Pheochromocytoma of urinary bladder. Indian J Nephrol. 2011;21:198–200.

7.        Dahia PL. Evolving concepts in pheochromocytomas and paraganglioma. Curr Opin Oncol. 2006;18:1-8.

8.        Piédrola G,  López E,  Rueda MD,  López R,  Serrano J,  Sancho M.  “Malignant  pheochromocytoma  of  the  bladder:  current controversies,” European Urology 1997;31:122–125.

9.        Pastor-Guzmán JM, López-García S, Giménez-Bachs JM, Ruíz-Mondejar R, Cañamares-Pabolaza L, Atiénzar-Tobarra M, et al. Paraganglioma of the bladder: controversy regarding treatment. Urol Int. 2004;73:270–275.

10.    Demirkesen O, Cetinel B, Yaycioglu O, Uygun N, Solok V.Unusual cause of early preeclampsia: bladder paraganglioma. Urology. 2000;56(1):154.

11.    Young WF., Jr Paragangliomas: clinical overview. Ann N Y Acad Sci. 2006;1073:21–29.

12.    Maddocks RA, Fagan WT Jr. Paraganglioma of bladder with recurrence ten years later. Urology. 1976;7:430–432.

Citation

Fernandes G, Parulekar SV. Paraganglioma of Bladder in Pregnancy. JPGO 2014. Volume 1 Number 10. Available from: http://www.jpgo.org/2014/10/paraganglioma-of-bladder-in-pregnancy.html

Hemoperitoneum from Ruptured Leiomyoma

Author information

Mirchandani Anil*, Parulekar SV**, Dalvi Parita***

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

Abstract

Leiomyomas are the commonest benign tumors in women. They are known to have varied symptoms depending upon the location and size of the leiomyoma. Development of a hemoperitoneum from the surface of a subserous leiomyoma is the least common mode of presentation of a leiomyoma of the uterus. We report such a case with life threatening intraperitoneal hemorrhage occurring from rupture of veins on the surface of a subserous leiomyoma.

Introduction

Leiomyoma of uterus happens in 30% of all women. Most of the leiomyomas are asymptomatic. The commonest symptom of leiomyoma uterus is abnormal uterine bleeding in the form of menorrhagia. Complications that a woman with uterine leiomyoma may present with include acute cystic degeneration, torsion, and acute red degeneration of the leiomyoma. One of the rarest complications of leiomyoma uterus which happens in subserous leiomyoma is rupture of the surface vessels over subserous leiomyoma. We report a case of such a complication in a middle aged patient.

Case Report

A 39 year old woman, married for 15 years, with previous 3 normal deliveries, tubal ligation surgery done in the past, presented with complaints of left lower abdominal pain for 3 days. The pain was progressively increasing in intensity. There was history of giddiness and easy fatigability for 3 days. There was no history of any bowel or urinary bladder related symptoms. There was no history of missed periods or any other menstrual complaint. There was no history of any other major medical or surgical illness except incidental gall stones. Her clinical examination revealed tachycardia of 102 beats per minute, moderate pallor, blood pressure of 100/60 mm of Hg. General and systemic examination revealed no abnormality. There was tenderness in the right iliac fossa, hypogastrium and hypochondrium, Horse shoe shaped dullness was present on abdominal percussion, suggestive of free fluid in the abdomen.  The uterus was of normal size. There was 8 week sized firm cystic mass arising from uterine fundus. There was no cervical motion tenderness but there was left forniceal tenderness. Her hemoglobin was 5g/dL. Pregnancy test on urine was negative. Abdominopelvic ultrasonography (USG) showed a fundal leiomyoma measuring 6 x 8 cm, and free fluid in the abdomen and pelvis with internal echoes, suggestive of a hemoperitoneum, Gall Bladder was mildly distended. Liver and spleen were normal. Computerized tomography confirmed these findings. A colpopuncture yielded free flow of blood which did not clot. In view of absence of any features of an alternative source of hemoperitoneum and presence of a subserous leiomyoma, a provisional diagnosis of rupture of a surface vessel of a uterine subserous leiomyoma was made. An exploratory laparotomy was performed. There was a 7x7 cm subserous leiomyoma with bleeding surface veins. Both ovaries and fallopian tubes were normal. Hemoperitoneum of 500 ml was present along with clots weighing 100 g. A myomectomy was performed. Redundant serosa with the bleeding vessels was removed. The bed of the leiomyoma was closed with interrupted sutures of No. 1 polyglactin and covered with the serosal flap of the leiomyoma. Patient was transfused with 4 units of blood. The patient made an uneventful recovery. Histopathology report confirmed benign leiomyoma.

Figure 1. Uterine fundal leiomyoma (white arrow) with rupture of surface veins. The uterus (black arrow) is held with Shirodkar's uterus holding forceps.

Figure 2. The veins on the surface of the leiomyoma are seen to be bleeding.

Discussion

Life threatening hemoperitoneum is an extremely rare complication of a uterine leiomyoma. The most common cause of it is bleeding from the ruptured surface vessels on the subserous leiomyoma.^[1] The hemorrhage is likely to be more acute and more severe if the ruptured vessel is an artery than when it is a vein. The other causes may be avulsion or torsion of, or blunt trauma to the leiomyoma. The first such reported case was way back in 1861 by Von Rokitansky in the autopsy of a girl who had died of intraperitoneal hemorrhage from the subserous venous rupture over leiomyoma uterus.^[2] Venous congestion and increased vascularity in leiomyoma is known to cause sudden rupture of superficial veins over leiomyoma, especially during a pregnancy.^[2,3] It may also occur if the patient is on anticoagulant therapy for any unrelated indication.^[4] The hemorrhage is likely to be less severe when the cause is torsion of a subserous pedunculated leiomyoma, because the vessels are usually occluded. Our patient had no antecedent history of blunt trauma or anticoagulation, she was not pregnant and rupture of its surface veins was spontaneous in nature. Exploratory laparotomy becomes the diagnostic and life saving therapeutic modality of treatment in such cases. Similar treatment can be given with laparoscopic surgery.^[5] The aim of this case report is to make the readers aware of this differential diagnosis while dealing with a case of acute abdomen and shock in gynecologic case of subserous leiomyoma uterus.

References

1.      Buttery BW. Spontaneous hemoperitoneum complicating fibromyomata. Aust N Z J Obstet Gynaecol 1972; 12 : 210-3.

2.      Wong L, Ching TW, Kok TL, Koon TH. Spontaneous hemoperitoneum from a uterine leiomyoma in pregnancy. Acta Obstet Gynecol Scand 2005;84:1208–1209.

3.      Mattison DR, Yeh SY, Hemoperitoneum from rupture of uterine vein overlying a leiomyoma. Am J Obstet 1980; 136: 415:16.

4.      Matsuda M1, Watanabe Y, Tonosu N, Nabeya Y, Arima H, Matsuzaki H, et al. Hemoperitoneum secondary to exophytic leiomyoma: report of a case. Surg Today. 2000;30(5):448-50.

5.      Estrade-Huchon S1, Bouhanna P, Limot O, Fauconnier A, Bader G.Severe life-threatening hemoperitoneum from posttraumatic avulsion of a pedunculated uterine leiomyoma. J Minim Invasive Gynecol. 2010;17(5):651-2..

Citation

Mirchandani A, Parulekar SV, Dalvi P. Hemoperitoneum from Ruptured Leiomyoma. JPGO 2014 Volume 1 Number 10. Available from: http://www.jpgo.org/2014/10/hemoperitoneum-from-ruptured-leiomyoma.html

Chronic Non Pueperal Inversion Of Uterus Secondary To Submucosal Fundal Myoma

Author Information

Bakre Tejashree*, Gupta AS**, Hira Priya***, Parulekar SV.****

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

Abstract

A case of  chronic inversion of uterus secondary to a large submucous fundal fibroid in a 40 year old multiparous woman with previous 2 lower segment cesarean sections (LSCS) is presented. Kustners' procedure was used to correct the inversion. Correction was preceded by vaginal myomectomy and followed by hysterectomy.

Introduction

Non puerperal uterine inversion is a very rare clinical entity. Almost all non puerperal cases are chronic and only around 10% have acute presentation.^[1] Chronic non puerperal inversion of uterus is associated with uterine tumors in about 90% of cases, most common being submucous fibroid.^[2]

Case Report

A 40 year old woman, para 2 living 2 with history of previous 2 lower segment cesarean sections, presented with complaints of something coming out of the vagina accompanied with bleeding since 15 days and lower abdominal heaviness. There were no voiding difficulties. She had a history of menometrorrhagia for past 2 months. There were no other surgical and medical high risk factors. On examination she had severe pallor and tachycardia. Abdomen was soft. There was a Pfannenstiel scar. On local examination a large, elongated, congested, red, soft mass 15 x 4 x 4 cm in size, with a firm, well demarcated, white lesion about 6 x 5 cm in dimension was seen at the end of the elongated mass. It was devoid of any punctum and was lying outside the introitus. The mass could be incompletely reduced  into the vagina.

Figure 1. The Prolapsed mass with the fibroid, as seen in the outpatient clinic

Figure 2. The Prolapsed mass with the fibroid, as seen in the operation theater. The leiomyoma is pointed out by blue arrows and the cervical ring by solid black arrows.

On vaginal examination a cervical rim without any depth could be felt all around except posteriorly. A provisional diagnosis of uterine inversion with a submucous fibroid polyp was made. The prolapsed part  was reposited back in the vagina and restrained by a vaginal tampon soaked in glycerin acriflavin solution.  However, it could not be kept reduced in the vagina.  The patient developed  fever 48 hours after admission. Pus was seen oozing from the inverted endometrium or the presumed stalk of the fibroid. A wound swab was sent for culture and sensitivity. It grew Pseudomonas areuginosa sensitive to Levofloxacin. The patient was given oral Levofloxacin for 10 days. Daily dressings with povidone iodine were performed.  The fever settled within 72 hours of  starting Levofloxacin. Her serological and biochemical preoperative investigations, chest radiography and electrocardiogram were done.  Hemoglobin was 6 g/dL.  All other investigations were in their normal range. The patient was transfused with 3 units of packed cells over 3 days. Ultrasonography revealed indentation of fundus with fundal fibroid (4.2 x 3.5 cm). Computerized tomography (CT) scan showed  cupping of fundus with inversion of uterus and a submucous fundal fibroid 5.5 cm in diameter. Magnetic resonance imaging (MRI) confirmed the findings showing U shaped uterine cavity on saggital images with complete inversion , a submucous fundal fibroid 5.5 cm in diameter with ovaries present in midline at site of inversion but without bladder involvement.

Figure 3. CT Scan showing the inverted fundus, ovaries in the cup and the cervical ring.

Figure 4. MRI Scan confirming CT scan findings.

The patient was operated under spinal anesthesia.  The cervical ring was very vascular anteriorly. Spinelli's surgery was not considered due to this vascularity, chances of presence of bladder in the inverted mass and adhesions between the uterus and bladder due to the previous surgeries. Kustner’s procedure was performed. The cervical ring was divided vertically in midline posteriorly. Divided edges of the cervix were held with Allis’ forceps. The myoma was enucleated from its bed in the fundus because the inverted uterus could not be reposited with the myoma in situ. The incision was extended in the isthmus and the uterine body posteriorly to facilitate correction of the uterine inversion.  The correction of the inversion was confirmed by the visualization of the cervix in central position and the anterior and posterior  fornices in front of and behind it respectively. Vaginal hysterectomy was then done by the standard method. Postoperative period was uneventful and patient was discharged on day 7. The perioperative and the post operative period was covered by antibiotics.

Figure 5. Kustner's operation: the posterior ring of the cervix is defined (arrow).

Figure 6. Kustner's operation:. Incision of posterior cervical ring and identification of the edges of the cervix (arrows).

Figure 7. Kustner's operation: myomectomy.

Figure 8. Kustner's operation: excised myoma (M) and its bed (B).

Figure 9. Kustner's operation: reformation of the lips of the cervix. A: anterior lip of cervix, P: two ends of the divided posterior lip of cervix.

Discussion

Uterine inversion is very uncommon.^[3,4] Most of the cases occur in the puerperal period. Non puerperal cases are mostly chronic and account for only 15% of all cases of inversion.^[1]  Over 90% of these are associated with uterine tumors, of which 20% are malignant.^[1]

Chronic inversion is of two types, incomplete and complete. Incomplete is the type where fundus protrudes through the cervix but lies inside the vagina and complete is one in which whole of the uterus including the cervix are inverted and lie outside the introitus. Vagina may also be involved in latter type.^[5] Predisposing factors for uterine inversion include submucous fibroid, endometrial polyp and uterine sarcoma.^[2] Most cases occur secondary to  prolapsed fibroid, with some reports stating that the fibroid was infected and necrotic.^[4]  The most common presenting symptoms of chronic inversion are chronic vaginal discharge and irregular vaginal bleeding leading to anemia.^[5] In this case the patient presented with a mass coming out per vaginum with foul smelling discharge from the mass and fever secondary to necrosis and inflammation of fibroid and surrounding endometrium. As the entity is rare a high index of suspicion is required for diagnosing uterine inversion. It can be mistaken for other conditions like prolapsed uterine fibroid, uterine sarcoma, uterovaginal prolapse and endometrial polyp.^[3,4,5]   Many a times the diagnosis is only made intraoperatively.^[6]  This leads to surgical difficulties, increase in complication rates and the surgeon may not be able to choose the most appropriate surgical method. The recto abdominal examination is often the most diagnostic clinical method wherein on bimanual palpation dimpling of uterine fundus is felt. Clinical diagnosis is often aided by imaging modality. USG is often the first modality for diagnosing an inversion of the uterus. Indentation of the fundal area, a depressed longitudinal grove extending from the uterus to the center of the inverted fundus and target sign with hyperechoic fundus surrounded by a hypoechoic rim, suggesting fluid occupying the space between the inverted uterine fundus and the vaginal wall are signs described in relation to the chronic uterine inversion.^.[5]  MRI and CT scan are useful diagnostic tools. They also help in understanding the extent of involvement of the bladder and ovaries in the inverted tissues.^[1] Signs indicative of uterine inversion on MRI are U shaped uterine cavity and a thickened and inverted uterine fundus on a sagittal image and a “bulls-eye” configuration on an axial image.^[1,3,4] Surgical treatment of chronic uterine inversion depends upon patient's fertility, stage of inversion and associated pathology.^[3] There are many abdominal and vaginal surgical approaches  to correct inversion.^[4] The vaginal and abdominal approaches have their own advantages and disadvantages. Hence opinion is divided regarding the management of chronic inversion of the uterus. As a leiomyoma was  present and uterine inversion was complete the vaginal approach was preferred in the case described. Vaginal myomectomy was done to debulk the uterine mass prior to  the correction of the inversion. The divided cervix is sutured after the correction of the inversion if the uterus is to be conserved. If a hysterectomy is to be performed, reposition is always done first, because hysterectomy on the inverted uterus is associated with difficulty in identification of the peritoneal pouches, and also associated with a great risk of injury to the urinary bladder and ureters.^[2]

Spinelli’s and Kustner ‘s operations are similar vaginal procedures, the only difference being that Spinelli's approach is anterior and involves division of the cervical ring, while Kustner's is a posterior approach with incision on the posterior cervical ring.^[2,3] Kustner’s approach was preferred here as there is little risk of injuring the bowel, because of the deep reflection of the pouch of Douglas and anterior approach involved danger of damaging the bladder during separation, risk being more due to complete inversion.^[7] The risk was even more in our case due to fibrosis and loss of tissue planes due to previous cesarean scars. Surgical reposition can also be done abdominally by Huntington’s procedure wherein round ligaments are pulled on and the inverted part of the uterus is pulled back into the peritoneal cavity by pulling on a series of Allis’ forceps applied progressively at lower levels. In Haultain’s  procedure, done transabdominally the constricting ring is divided posteriorly by  a vertical incision and then the inversion is corrected.^[3] In Dobbin’s procedure, the cervical ring is divided in midline anteriorly by an abdominal approach and then the inversion is corrected.^[3]

Conclusion

These rare cases pose diagnostic and management challenges. A high index of suspicion by the clinician and the radiologist can detect the true nature of the condition.  Once a diagnosis is established the surgical correction can be done by  adopting the best option from the various techniques described in literature. As the clinical entity of chronic inversion is rare definite management guidelines cannot  be recommended and management of each case has to be individualized.

References

1. Ashraf-Ganjooie T: Nonpuerperal uterine inversion: A case report. Archives of Iranian Medicine 2005;8(1):63-66  .
2. Kagne SS, Thawal YA, Tambe SG. An extremely rare case of chronic non-puerperal uterine inversion treated by myomectomy preceding vaginal hysterectomy. Journal of evolution of medical and dental sciences. 2013;46: 8976-8979.
3. Shabbir S, Ghayasuddin M, Younus SM, Baloch K. Chronic non puerperal uterine inversion secondary to sub-mucosal fibroid: JPMA 2014;64:586-588.
4. Kilpatrick CC, Chohan L and Maier RC. Chronic nonpuerperal uterine inversion and necrosis: a case report. J Med Case Reports 2010;4: 381.
5. Jain S, Aherwar R, Joshi P. Chronic Non-Puerperal Uterine Inversion; Fibromyoma Uteri as a Cause- A Case Report.  Sch J Med Case Rep 2014;2(2):100-102.
6. Sharma JB, Kumar S, Rahman SM, Roy KK, Malhotra N. Non-puerperal incomplete uterine inversion due to large sub-mucous fundal fibroid found at hysterectomy: a report of two cases. Archives of Gynecology and Obstetrics 2009;279(4):565-567.
7. Culiner A, Charlewood GP. The surgical management of chronic inversion of the uterus. S. A. Medical Journal May 1954; 459-60.
8. Crossen HS.Chronic inversion of the uterus. In Operative Gynecology. 1st ed.  St. Louis: CV Mosby Co; 1917: Pp. 151-160.

Citation

Bakre T, Gupta AS, Hira P, Parulekar SV. Chronic Non Pueperal Inversion Of Uterus Secondary To Submucosal Fundal Myoma. JPGO 2014. Volume 1 Number 10. Available from: http://www.jpgo.org/2014/10/chronic-non-pueperal-inversion-of.html

Successful Pregnancy Outcome In Severely Immunocompromised Patient

Author Information

Madhva Prasad*, Sumit Chamariya*, Rashmi Khadkikar**, A. R. Chauhan***

(* Third Year Resident, ** Assistant Professor, *** Additional Professor. Department of Obstetrics & Gynecology, Seth GS Medical College & KEM Hospital, Parel, Mumbai, India.)

Abstract

Maternal HIV infection leads to immunocompromised state and adverse maternal and perinatal outcomes. With institution of ART and a multi-disciplinary approach, improved outcomes are possible even with severe immune compromise. Successful pregnancy outcome in a patient with CD4 count of only four is highlighted here.

Introduction

HIV infection is a major global health problem; an estimated 35.3 million people were living with HIV in 2012 but deaths due to HIV/ AIDS have decreased to 1.6 million people. To “eliminate HIV infection among children and reduce maternal deaths” is a key element described by WHO. This is possible by good anti-retroviral therapy (ART) coverage, which has reached 62% in 2012.¹ In this context of improved survival among mothers with HIV/AIDS, a successful pregnancy outcome in a severely immunocompromised case is presented here.

Case Report

A 25 year old primigravida, separated from husband, urban resident in Western India, middle socio-economic status, with 19 weeks’ gestation was referred for severe anemia. Patient had complaints of decreased appetite, fever and breathlessness. Though detected to be HIV positive six months earlier and advised ART, she had defaulted and was not on any treatment. She had been transfused one unit blood prior to referral.

She was conscious, severely pale, and afebrile, with mild tachycardia (pulse 104 beats / minute). She was cachexic, with a BMI of 15.3. On general examination, she had palpable cervical lymph nodes and extensive bilateral crepitations on respiratory system auscultation.  Abdominal examination revealed a relaxed uterus of 18 weeks’ with positive external ballotment and regular fetal heart sounds; non-tender hepato-splenomegaly and minimal ascites. She had candidasis and an erythematous rash on speculum examination; cervical os was closed. There was no other evidence of opportunistic infections.

Investigations revealed severe anemia (hemoglobin 5.6 gm/ dl), total WBC count of 5600/ mm³, thrombocytopenia of 40000/ mm³. CD4 count was only 4. The normal value of CD4+ T helper cells ranges between 410 and 1590/ µL. The AIDS surveillance and case definition system utilizes this CD4 count:  any count < 200 is sufficient to label the patient as having AIDS.² Ultrasonography showed a single live fetus of 19 weeks’gestation with oligohydramnios and fetal hydrocephalus. Rest of the fetal anatomical survey was normal. Abdominal scan showed multiple lymph nodes, hepato-splenomegaly and altered liver echotexture suggestive of abdominal tuberculosis.  Chest X- ray was normal. Sputum for acid fast bacilli was negative. Other viral markers were negative.

Correction of severe anemia was initiated with two units of blood transfusion. ART was instituted in consultation with the physician; patient was started on HAART (Highly Active Anti-Retroviral Therapy) with daily tablets of tenofovir/ lamivudine and efavirenz. In view of extremely low CD4 count, she was also empirically started on weekly tablets of secnidazole and azithromycin. Candidiasis was treated with cotrimoxazole vaginal pessary. Category 1 anti-tuberculous therapy was started in view of abdominal tuberculosis as per the recommended standard regimen; patient was discharged and asked to follow-up on outpatient basis.

However, her follow- up was irregular and she next presented at 32 weeks gestation with threatened preterm labor. Examination revealed a relaxed uterus of 30 weeks’ with regular fetal heart sounds and closed internal os. Ultrasonography revealed a single live fetus of 30 weeks 2 days with moderate oligohydramnios and estimated weight of 1.2 kg; Doppler flows were suggestive of fetoplacental insufficiency. Corticosteroids were given for fetal lung maturity. Doppler two days later was suggestive of fetoplacental and uteroplacental insufficiency with severe oligohydramnios and incidentally detected bilobulated psoas abscess of 60 ml volume in maternal pelvis.  No active intervention was done for this abscess as patient remained asymptomatic. In view of abnormal Doppler and oligohydramnios, labor was induced with prostaglandin E2 gel. Patient delivered a male child of 1.425 kg with Apgar score of 9/10. The mother opted to top- feed. The baby was admitted to neonatal intensive care, was tested to be HIV negative. Zidovudine prophylaxis for 6 weeks was started as per standard recommendations. Detailed ultrasonography ruled out any major neurological problem. Once appropriate weight gain was achieved, mother and baby were discharged on day 11 on life.

Discussion

A comprehensive approach to manage anemia, including micronutrient supplementation and infectious disease control is warranted in HIV-infected women in resource-limited settings, as seen in our patient, who received blood transfusion in early pregnancy.^[3] 

CD4 count during pregnancy is dependent on gestational age, micronutrient status and seasonal influences. However, these do not appear to influence clinical practices.^[4] Though our patient had alarmingly low CD4 count of 4, she never required ICU care; intensive care admission itself is an adverse outcome predictor in HIV positive patients.^[5] Institution of ART is the most important step to improving maternal outcomes in these patients.^[6] However, use of HAART regimen itself is associated with low birth weight and other poor obstetric outcomes^[7]; this has been confirmed in a study from a similar geographic location.^[8] Studies have found an association between maternal HIV and adverse pregnancy outcomes including spontaneous abortions, preterm delivery, low birth weight and perinatal loss. Low CD4 count and opportunistic infections increase the propensity of the same.^[9-13]   Pregnancy-related mortality in HIV infected women is unlikely to be due to a higher risk of direct obstetric complications and reducing this mortality will require non- obstetric interventions involving access to ART in pregnant women.^[14]

Our patient progressed from a symptomatically ill 20 weeks’ pregnant patient to a stable, compliant patient with successful maternal and neonatal outcome despite advanced maternal HIV disease. The cornerstone of the management included institution of ART and a multi-disciplinary approach. With the advent of ART, health care providers can expect increasing numbers of mothers with advanced maternal HIV disease and can expect a consistent improvement in outcomes.

References

1.      Global report: UNAIDS report on the global AIDS epidemic 2013. Revised and reissued, November 2013

2.      Lane HC, Fauci AS in Human immunodeficiency virus: AIDS and related infections: Introduction.  In:  Longo DL, Kasper DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J, Eds. Harrison’s Principles of Internal Medicine.18^th Ed. McGraw-Hill; 2012

3.      Finkelstein JL, Mehta S, Duggan CP, Spiegelman D, Aboud S, Kupka R. Predictors of anemia and iron deficiency in HIV-infected pregnant women in Tanzania: a potential role for vitamin D and parasitic infections. Public Health Nutr. 2012 May; 15(5): 928-37.

4.      Gomo E, Vennervald BJ, Ndhlovu P, Kaestel P, Nyazema N, Friis H. Predictors and reference values of CD4 and CD8 T lymphocyte counts in pregnancy. A cross sectional study among HIV negative women in Zimbabwe. Cent Afr J Med. 2004 Jan-Feb; 50(1-2):10-9

5.      Ngene NC, Moodley J, Songca P, von Rahden R, Paruk F, Onyia CO. Maternal and fetal outcomes of HIV-infected and non-infected pregnant women admitted to two intensive care units in Pietermaritzburg, South Africa. S Afr Med J. 2013;103(8):543-8.

6.      van der Merwe K, Hoffman R, Black V, Chersich M, Coovadia A, Rees H. Birth outcomes in South African women receiving highly active antiretroviral therapy: a retrospective observational study. J Int AIDS Soc. 2011;14:42.

7.      Gibb DM, Kizito H, Russell EC, Chidziva E, Zalwango E. Pregnancy and Infant Outcomes among HIV-Infected Women Taking Long-Term ART with and without Tenofovir in the DART Trial. PLoS Med 9(5): e1001217. . doi:10.1371/journal.pmed.1001217

8.      Darak S, Darak T, Kulkarni S, Kulkarni V, Parchure R, Hutter I. Effect of highly active antiretroviral treatment (HAART) during pregnancy on pregnancy outcomes: experiences from a PMTCT program in western India. : AIDS Patient Care STDS. 2013;27(3):163-70.

9.      Ezechi OC, Gab-Okafor CV, Oladele DA, Kalejaiye OO, Oke BO, Ohwodo HO. Pregnancy, obstetric and neonatal outcomes in HIV positive Nigerian women. Afr J Reprod Health. 2013; 17(3):160-8.

10.  Turner AN, Tabbah S, Mwapasa V, Rogerson SJ, Meshnick SR, Ackerman WE 4^th. Severity of maternal HIV-1 disease is associated with adverse birth outcomes in Malawian women: a cohort study.  J Acquir Immune Defic Syndr. 2013;64(4):392-9.

11.  Kim HY, Kasonde P, Mwiya M, Thea DM, Kankasa C, Sinkala M, et al. Pregnancy loss and role of infant HIV status on perinatal mortality among HIV-infected women. BMC Pediatrics 2012;12:138

12.  Cailhol J, Jourdain G, Coeur SL, Traisathit P, Boonrod K, Prommas S. Perinatal HIV Prevention Trial Group. Association of low CD4 cell count and intrauterine growth retardation in Thailand. J Acquir Immune Defic Syndr. 2009;50(4):409-13.

13.  Zaba B, Calvert C, Marston M, Isingo R, Nakiyingi-Miiro J, Lutalo T. Effect of HIV infection on pregnancy-related mortality in sub-Saharan Africa secondary analyses of pooled community-based data from the network for Analysing Longitudinal Population-based HIV/AIDS data on Africa (ALPHA). Lancet 2013;381(9879):1763-71.

14.  Calvert C, Ronsmans C. The contribution of HIV to pregnancy-related mortality: a systematic review and meta-analysis. AIDS. 2013 Jun 19; 27(10): 1631-9.

Citation

Prasad M, Chamariya S, Khadkikar K, Chauhan AR.. Successful Pregnancy Outcome In Severely Immunocompromised Patient. JPGO 2014. Volume 1 Number 10. Available from: http://www.jpgo.org/2014/10/successful-pregnancy-outcome-in.html

Placental Site Nodule

Author Information

Kimaya Mali*, Fernandes G**, Vibha More*, M. N. Satia***

(* Assistant Professor, *** Professor, Department of Obstetrics & Gynecology, ** Associate Professor, Department of Pathology, Seth GS Medical College & KEM Hospital, Parel, Mumbai, India.)

Abstract

Placental site nodule is a rare, well-circumscibed, benign, generally asymptomatic lesion of chorionic type intermediate trophoblastic origin, which is often detected several months to years after the pregnancy from which it resulted. We report a case of placental site nodule which was incidentally diagnosed on premenstrual dilatation and curettage in a patient who presented with abnormal uterine bleeding three years after a spontaneous abortion.

Introduction

Placental site nodule (PSN) is a benign lesion which is rare and represents remnants of intermediate trophoblast from a previous gestation. It generally represents a retained noninvoluted placental site. Placental site nodule is diagnosed in many cases, years after tubal ligation which suggests that it develops as an unrecognized pregnancy or is retained in the endometrium for a long time. These lesions many a times are diagnosed incidentally after curettage or in hysterectomy specimens done for abnormal uterine bleeding, post coital bleeding or abnormal cervical smears.

Case Report

A 38 year old woman, married for 20 years, multipara, presented to  out  patient  department with complaints of menorrhagia for 1 year. She had had 5 normal deliveries and one spontaneous abortion. Her last pregnancy resulted in spontaneous abortion for which curettage was done 3 years ago. She or her spouse had not undergone sterilization procedure. On examination her vital parameters were stable. On speculum examination her cervix was hypertrophied and on bimanual examination the uterus was anteverted, 6-8 weeks in size, and firm. Pelvic ultrasonography showed a  bulky  uterus with  endometrial thickness of 10 mm and an anterior wall leiomyoma of 10x9 mm. Her Pap smear was normal. A premenstrual dilatation and curettage was performed for evaluation of abnormal uterine bleeding. Moderate amount of endometrium was obtained which was sent for histopathological examination. Histopathological examination showed secretory endometrium with tortuous glands showing secretory changes. The stroma was loose and edematous with spiral arterioles and decidualization around them. A prominent well circumscribed nodular, eosinophilic lesion was seen. It was composed of single and nests of cells within an abundant extracellular hyaline material. These cells were of intermediate trophoblastic origin, had pleomorphic hyperchromatic bizarre nuclei and plenty of vacuolated eosinophilic cytoplasm. No mitosis, decidua or chorionic villi were seen. A diagnosis of a placental site nodule in a background of secretary endometrium was made.  Her β-human chorionic gonadotropin (β-hCG) levels were not elevated (less than 2 mIU/ml), when checked after the histological diagnosis was made..

Figure 1. Well circumscribed eosinophilic nodule composed of intermediate trophoblastic cells embedded in a hyalinized extracellular matrix. Endometrial glands and stroma are seen around the nodule. (H and E × 100)

Figure 2. Higher magnification of the nodule showing large vacuolated pleomorphic trophoblastic cells amidst eosinophilic extracellular matrix. (H and E × 400)

Discussion

A placental site nodule is a well circumscribed hyalinized lesion composed of chorionic-type intermediate trophoblastic cells. Placental site nodule represents a retained noninvoluted placental site tissue which may have remained in the uterus for several years after the pregnancy from which it resulted. The interval from the recent pregnancy till the detection of tumor averages around 3 years with range of around 1 month to 8 years.^[1] Placental site nodule is seen in endocervix in 40% of cases, 56% in endometrium and 4% in fallopian tube and very rarely in ovary. Placental site nodule should be differentiated from other lesions like placental site trophoblastic tumor and epitheliod trophoblastic tumor. ^[1,2] The patients’ ages range from 20 to 47 years at diagnosis and the mean age is in early thirties.^[3] It is usually an incidental finding diagnosed during surgical evaluation  for metro-menorrhagia, hypermenorrhoea, dysmenorrhoea, recurrent abortions, post-coital bleeding, abnormal cervical smear, or infertility.^[2,3]

Placental site nodule mainly is diagnosed microscopically, but sometimes it is grossly visible in the endometrium or superficial myometrium as a yellow, tan or a hemorrhagic nodule varying in size from 1 mm to 1 cm in diameter. Microscopically it has a discrete, well circumscribed , lobulated border sometimes showing cells projecting into the surrounding  tissue .The intermediate trophoblastic cells within placental site nodule are embedded in abundant  eosinophilic  fibrillar extracellular matrix protein which is the most prominent feature of placental site nodule. The cells are small to large in size with hyper chromatic nuclei and often vacuolated cytoplasm.

The placental site nodule is differentiated from other intermediate trophoblast tumors like placental site trophoblastic tumor, epitheloid tumor, and certain nontrophoblastic lesions like invasive keratinizing squamous cell carcinoma of the cervix. The small size of the  lesion, sharp circumscribed borders,  extensive eosinophilic extracellular matrix, bland and low or absent  mitotic activity, lack of necrosis, lower ki-67 index (<10%) and lack of association with current or recent pregnancy helps to differentiate a placental site nodule from placental site trophoblastic tumors ( PSTT)  and epitheliod  trophoblastic tumors (ETT).  PSTT show trophoblastic infiltration of the muscle fibers, vasculotropism, extensive deposition of fibrinoid material, atypical nuclei,  frequent mitoses, and necrosis. ETT show larger lesions with substantial necrosis, are more cellular, have atypical cells with frequent mitoses. A squamous cell carcinoma, a nontrophoblastic lesion, may also be confused with placental site nodule. Larger size, greater cytological atypia with mitosis and presence of keratinized cells are  features  of squamous carcinoma.

The importance of recognizing this lesion is because it is benign, does not recur and no specific treatment or follow-up is required. It needs to be differentiated from other gestational trophoblastic tumors which are aggressive lesions.^[4] However there are some reports in which PSN transformed into a malignant epithelioid trophoblastic tumor.^[4] However there are some reports in which PSN transformed into a malignant epithelioid trophoblastic tumor.^[5,6]

References

1.      Young RH, Kurman RJ, Scully RE. Placental site nodules and plaques: A clinicopathologic analysis of 20 cases. Am J Surg Pathol 1990;4:1001-9.

2.      Shih IM, Seidman JD, Kurman RJ. Placental site nodule and characterization of distinctive types of intermediate trophoblast. Hum Pathol 1999;30:687-94.

3.      Huettner PC, Gersell DJ. Placental site nodule: a clinicopathologic study of 38 cases. Int J Gynecol Pathol 1994;13(3):191-8.

4.      Shih IM, Kurman RJ. Ki-67 labeling index in the differential diagnosis of exaggerated placental site, placental site trophoblastic tumor, andchoriocarcinoma: a double immunohistochemical staining technique using Ki-67 and Mel-CAM antibodies. Hum Pathol 1998;29:27-33.

5.      Tsai HW, Lin CP, Chou CY, Li CF, Chow NH, Shih IM, Ho CL. Placental site nodule transformed into a malignant epithelioid trophoblastic tumour with pelvic lymph node and lung metastasis. Histopathology 2008;53:601–604. doi: 10.1111/j.1365-2559.2008.03145.x

6.      Bo-Jung C, Chien-Jui C, Wei-Yu C.Transformation of a post-cesarean section placental site nodule into a coexisting epithelioid trophoblastic tumor and placental site trophoblastic tumor: a case report.Diagnostic Pathology 2013,8:85. doi:10.1186/1746-1596-8-85

Citation

Mali K, Fernandes G, More V, Satia MN. Placental Site Nodule. JPGO 2014. Volume 1 Number 10. Available from: http://www.jpgo.org/2014/10/placental-site-nodule.html