Volume 2 Issue 12, December 2015

Editorial
Chauhan AR

A Massive Ovarian Mucinous Cystadenoma Managed by Surgical Removal and Abdominal Plastic Reconstruction
Goel B, Goel S.

Innovative Method Of Hysteroscopic Retrieval Of A Broken Metal Suction Cannula Tip During MTP
Shah NH, Shah VN, Paranjpe SH.

Sacral Giant Cell Tumor In Pregnancy: A Rare Occurrence
J Madhavi, Warke HS, Satia MN.

Subacute Intestinal Obstruction Due To Classical Cesarean Section Scar Rupture.
Deshpande PS, Maurya N, Ansari M, Gupta AS.

Ovarian Torsion at Term Pregnancy- A Rare Case Report.
Sharma G, Supe P, Kore SJ, Nandanwar YS.

Chronic Idiopathic Thrombocytopenic Purpura Causing Neonatal Thrombocytopenia.
Goel A, Jain P, Shende D, Chauhan AR.

Successful Management Of Recurrent Peripartum Cardiomyopathy.
Amin K, Shende D, Dhokia T, Chauhan AR.

Primary Amenorrhea Of Combined Etiology.
Prasad R, Parulekar SV.

Classical Cesarean Section In A Case Of Advanced Carcinoma Cervix With Twin Pregnancy.
Karve N, Gupta AS.

Editorial

Chauhan AR

Ovarian cysts in pregnancy are fairly uncommon; most data on incidence is derived from case reports and case series, and ranges from 0.1 to 2.4% depending on the gestational age. The vast majority are physiological, functional and benign and the risk of malignancy is low (1 to 6 % of all ovarian masses in pregnancy). Incidental diagnosis of simple cysts in asymptomatic women in early pregnancy should be managed expectantly as these usually resolve spontaneously and do not cause any adverse effect on the pregnancy. Ideally all women undergoing first trimester sonography should have their ovaries scanned and in case cysts are found, they should undergo repeat sonography at 6 to 8 weeks, and further follow -up. In case of detection of more complex masses, color Doppler is indicated.

The most common pregnancy-associated ovarian masses are functional cysts (corpus luteum cyst or hematoma), which usually resolve by 16 weeks' pregnancy. Masses which persist beyond this time are usually non- functional; the commonest is the dermoid which accounts for almost half the total cases, followed by endometrioma. They are usually asymptomatic, or patients may present with pain or acute abdomen in the mid or third trimester, mainly due to hemorrhage or torsion. Epithelial ovarian neoplasms in pregnancy are usually benign; cystadenomas account for almost half of these, with serous cystadenoma being the commonest. These cysts are usually thin walled, range from 5 to 20 cm and may be multiloculated, and bilateral in almost 20 % of cases. Non- functional ovarian masses and cystadenomas do not usually resolve, and they need close follow up for size and differentiation from malignant ovarian neoplasms. 

The obstetrician should carefully weigh the decision for surgery versus expectant management in pregnancy; retrospective reviews of conservation have shown higher fetal morbidity and mortality, and higher chance of spontaneous rupture of cysts with spillage, and/ or torsion.  Hence most authors are in agreement that cyst size in excess of 6 cm after 16 weeks' gestation should be surgically removed in the second trimester, as too early an intervention may carry the risk of miscarriage. In case of laparotomy, a midline incision with minimal uterine handling is the preferred approach. Laparoscopy is feasible and safe during pregnancy and has many advantages over laparotomy. However specific guidelines like avoidance of Veress needle and preference for open laparoscopy method, primary trocar placement (supra umbilical),  avoidance of Trendelenberg tilt or lateral tilt, size of the mass relative to the size of the uterus, speed and skill of the surgeon, should be followed. Dermoids should be removed via an endobag during laparoscopic surgery to avoid spillage. In a September 2015 review of more than 500 ovarian masses in pregnancy in the American Journal of Perinatology, Webb et al commented that laparotomy and laparoscopy were both comparable in terms of pregnancy complications like spontaneous abortion, vaginal bleeding and intrauterine fetal death, but incidence of preterm contractions was significantly higher in the laparotomy group and those who were operated in emergency settings. Percutaneous transabdominal aspiration for large simple cysts in second and third trimesters has been used by some authors; this may obviate the need for surgery and also avoid potential issues of preterm labor and fetal problems.

A Massive Ovarian Mucinous Cystadenoma Managed by Surgical Removal and Abdominal Plastic Reconstruction

Author Information

Goel B, Goel S.
(Obstetrics and Gynaecology, Kamla Nagar Hospital, Jodhpur, India.)

Abstract

A rare case of massive ovarian mucinous cystadenoma managed by surgical removal and abdominal plastic reconstruction.

Introduction

Ovarian mucinous cystadenoma, second most common epithelial tumour of the ovary is a benign tumour.[1] It originates from the surface epithelium of the ovary. It is a multilocular cyst that tends to be huge in size.[2] In India, ovary is the third most common site of cancer among women and sixth worldwide. The most frequent complications of benign ovarian cysts are torsion, haemorrhage and rupture.[3] The most dangerous complication is pseudomyxoma peritonei. The rupture of cyst causes mucinous depositions leading to adhesions in the abdomen. Its recurrence is found to be rare after complete excision.[4]

Case Presentation

A 50-year-old married Rajasthani woman, para 6 presented at the gynecology outpatient clinic of Kamla-Nagar Multi Speciality Hospital, Jodhpur, with a massive abdominal distension progressed over 2 years and respiratory discomfort. The patient had hypertension since 1 year and no previous medical diseases or surgical operations. Her menarche was at the age of 13 years with subsequent regular cycles. On General examination the lady was found to be emaciated, in distress. She had blood pressure 170/100 mm of Hg and rest normal vital signs other than moderate tachypnea. Her body weight was 87 kg, her height was 162 cm and her abdominal circumference was 180 cm and 90 cm from xiphisternum to pubic symphysis.

Figure 1 showing a massive ovarian mucinous cystadenoma

On abdominal examination, a huge ill-defined pelvi-abdominal mass was noticed, extended up to xiphisternum, with evident veins, mimicking triplet term pregnancy. On palpation her abdomen was cystic. There was no tenderness or shifting dullness. On Pelvic examination the uterus was normal sized, firm with fullness in the cul-de-sac. On per speculum examination the cervix was not seen. On transabdominal ultrasonography a huge multilocular cyst with no solid components and mild ascites was seen. The laboratory investigations including complete blood picture, blood biochemistry, cervical cytology and cancer antigen (CA-125) were within normal range. A plain chest radiograph in erect position was also normal. MRI was impossible due to large size of the tumor.

She was planned for laparotomy. Abdomen was opened by a paramedian incision. Huge cyst was present reaching up to xiphisternum. Blackish mucinous fluid was suctioned out. Cyst was multiloculated. Intra operatively 65 litres of fluid approximately was aspirated from the mucinous cyst of the left ovary. After aspiration of the fluid, the cyst could not be delivered as it was adherent to mesentery and lateral pelvic wall. After doing adhesiolysis total abdominal hysterectomy plus bilateral salpingo-oophorectomy was done. Uterus and right adnexa were found th be normal. The abdominal cavity was explored. The liver, spleen, and intestines were normal. Abdominal plastic reconstruction was done by plastic surgeon. Four drains were inserted – in pelvic pouch of Douglas, right sub-diaphragmatic space, under rectus sheath and subcutaneous plane. The patient was managed in intensive care unit on a ventilator for 4-5 days.

Figure 2 showing surgical specimen of cyst wall.

Postoperative period was uneventful and patient was transfused 5 units of packed red blood cells. She weighed 42 kg postoperatively. She was discharged on the postoperative day 12. She was advised to follow up after 6 weeks

Discussion

Giant mucinous ovarian tumors are detected early during routine examinations. Hence they have become rare entity to be found in clinical practice.[1] These giant tumors are associated with pressure symptoms like respiratory embarrassment, urinary tract changes, defecation difficulties and debilitation. The frequent complications are torsion, hemorrhage and rupture of these giant mucinous cysts. Its rupture causes mucinous depositions on abdomen. Their recurrence is rare after complete excision. On histopathological examination it shows non-ciliated columnar cells with mucin at apical part. Our case had epithelium of intestinal-like cells. Management of these cases depends on the age of the patient, symtoms, size, its histopathology and complications of giant cysts.[4] It requires taking care of both the complications and problems arising with sudden decompression of these huge cysts.[2]

Conclusion:

Even though the malignant form is less common than other gynecological cancers, they are still the gynecologists greater test challenge because of its mortality is one of the highest of gynecological neoplasm.

References

1. Vizza E, Galati GM, Corrado G, Atlante M, Infante C, Sbiroli C et al. Voluminous mucinous cystadenoma of the ovary in a 13-year-old girl. J Ped Adoles Gynecol. 2005; 18 (6) : 419–422. doi: 10.1016/j.jpag.2005.09.009.
2. Ozgun MT, Turkyilmaz C et al. A giant ovarian mucinous cystadenoma in an adolescent: a case report. Arch Med Sci. 2009;5(2):281–283.
3. Yenicesu GI, Cetin M, Arici S. A huge ovarian mucinous cystadenoma complicating pregnancy: a case report. Cumhuriyet Medical Journal. 2009;31:174–7.
4. Alobaid, A.S. Mucinous cystadenoma of the ovary in a 12-year-old girl. Saudi Medical Journal 2008;29,:126-128.

Citation

Goel B, Goel S. A Massive Ovarian Mucinous Cystadenoma Managed by Surgical Removal and Abdominal Plastic Reconstruction. JPGO 2015. Volume 2 No. 12. Available from: http://www.jpgo.org/2015/12/a-massive-ovarian-mucinous-cystadenoma.html

Innovative Method Of Hysteroscopic Retrieval Of A Broken Metal Suction Cannula Tip During MTP

Author Information

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

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

Abstract

The breaking of the tip of a metal suction cannula while performing a suction evacuation procedure is a rare and almost unheard of the complications. Usually such a complication is managed by removing that broken tip, with a ring forceps or an artery forceps, both of which are blind procedures. Here we present a case of a 22 year old woman while undergoing an MTP, wherein, the tip of the metal suction cannula was broken during the procedure and was retained inside the uterus. We removed that broken tip with an innovative method using a laparoscopic 5 mm tenaculum passed alongside the hysteroscope. Thus establishing a safe and under vision, method of the removal of any foreign body from the uterus, under direct vision, as a better option than a blind procedure and the need for new innovative hysteroscopic instruments, for retrieving heavier and larger objects.

Introduction

A review of literature shows very few cases of breaking of the metal tip of a cannula during suction and evacuation. As such foreign body in uterus is a very rare condition. The usual foreign bodies that are found are intrauterine devices. Other foreign bodies found in the uterus are broken laminaria tents, tips of curettes, and non-absorbable suture materials. Very rarely fetal bones after an incomplete abortion have also been written about. Hysteroscopy is always a better option for foreign body removal as direct vision reduces the chances of complications than that of a blind procedure. It also helps to see whether the foreign body is removed in whole and completely, as the entire cavity can be scanned under vision. 

Case Report

A 29 year old female gravida 3 para 2 living 2 with 6 weeks of pregnancy came to us for a medical termination of pregnancy. All routine blood examinations were sent and her hemoglobin, platelets and urine routine were within normal range. While doing a suction and evacuation with a metal vacuum aspiration cannula, the tip of the cannula broke inside the uterus and did not come out. The suction cannula was checked prior to the procedure but probably due to the wear and tear it broke inside while performing the procedure. An attempt was made to try to remove the tip doing a curettage and also of a blind procedure by using a ring forceps and an Allis’ forceps, both of which failed due to improper grasp on the broken tip, probably due to a change in position of the broken tip.

Figure 1. Metal tip and tenaculum seen during hysteroscopy.

Figure 2. Position of tenaculunm to remove tip vertically.

A decision of hysteroscopic removal of the broken tip was taken. Normal saline was used as a distension media. The outflow track of the hysteroscpe was intermittently opened due to which the vision was not hampered. But the hysteroscopic grasper would have been too small to catch heavy and large broken metal tip. Thinking that the hysteroscopic grasper would also break while trying to hold a larger and heavier object like the metal tip, the idea was abandoned.

Hence an innovative technique was used wherein we used a laparoscopic 5 mm tenaculum.

The tenaculum was passed along side the hysteroscope. There was minor leakage from alongside the hysteroscope but did not compromise the distension nor the vision. And due to the direct vision, we were able to grasp the hollow part of the broken tip with the laparoscopic 5 mm tenaculum due to which the tip could be removed vertically with the least diameter.

Discussion

Early case reports in the literature date to the mid 60’s and the management of in-utero foreign bodies’ cases have previously been blind dilatation and removal with a ring forceps or a hysterectomy.[1] Verma et al reported their experience with ultrasound-guided removal of retained IUDs, as safe and cost-effective, and it could be performed in an office setting.[2] Roy et al. reported removal of an intrauterine wooden stick retained for 12 years from the uterine fundus, under ultrasound guidance.[3] Yazicioglu et al. removed the tip of number 6 Karman’s cannula hysteroscopically from the subvesical space.[4]

But as we can see from this case that holding the broken tip at a specific site and reducing the possibility of injuring any surrounding areas was only possible under direct vision.

Also a larger instrument like a laparoscopic tenaculum was needed to retrieve a large and heavy object like the broken metal tip.

Operative hysteroscopy is an evolving branch as thus we conclude that removing the broken tip under vision was a better option than a blind or under ultrasonography guidance. Also there is a  need to develop new larger innovative hysteroscopic instruments for different operative procedures.

References

1. De Brux J, Palmer R, Ayoub-Despois H () Les ossifications de l'endometre. Gynecol Obstet (Paris) 1956;55:494–497.
2. Verma U, Astudillo-Davalos FE, Gerkowicz SA. Safe and cost-effective ultrasound guided removal of retained intrauterine device: our experience. Contraception. 2015 Feb 21;12:52-3.
3. Roy KK, Mittal S, Verma A. Removal of an intrauterine foreign body retained for 12 years. Int J Obstet Gynecol 1996;54:185-6
4. Yazicioglu HF, Yasar L, Dulger O. Hysteroscopic removal of a foreign body from the subvesical space. Int J Obstet Gynecol 2004;86:48-9. 

Citation

Shah NH, Shah VN, Paranjpe SH. Innovative Method Of Hysteroscopic Retrieval Of A Broken Metal Suction Cannula Tip During MTP. JPGO 2015. Volume 2 No. 12. Available from: http://www.jpgo.org/2015/12/innovative-method-of-hysteroscopic.html

Sacral Giant Cell Tumor In Pregnancy: A Rare Occurrence

Author Information

J Madhavi*, Warke HS**, Satia MN***.
(* Second Year Resident, ** Associate Professor, *** Professor; Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

Abstract

The occurrence of malignancy in pregnancy is rare, with an incidence of 0.07-0.1% of all malignant tumors based on epidemiological studies. [1,2] Giant cell tumors (GCT), better known as Osteoclastomas are aggressive benign neoplasms of the bone commonly affecting the ends of long bones. Sacrum is the most common site for the tumor in the axial skeleton, accounting for 6% of all GCTs. [3] It is unusual to find a giant cell tumor during pregnancy. We present a case of a primigravida with sacral giant cell tumor diagnosed during pregnancy.

Introduction

Giant cell tumors are common bone neoplasms affecting the ends of long bones. The most common sites being the distal femur, proximal tibia and distal radius in order of occurrence. The sacrum ranks fourth in the order of occurrence [4]. The most common primary tumor of the sacrum is chordoma, second being GCTs. Although considered benign tumors, they can be locally aggressive and can also metastasize. Treatment options include radiation therapy, surgical management in the form of intralesional curettage and filling with bone cement, wide excision by complete or partial en bloc sacrectomy, surgery plus adjuvant treatment and serial arterial embolization. Other drugs that are being used as adjuvant treatment modalities for recurrent and metastatic tumors are bisphosphonates, denosumab and interferons. Our patient underwent elective Cesarean section at 34 weeks gestation following which she was treated with serial arterial embolization and denosumab therapy.

Case Report

A 36 year old primigravida, married since one year, 29.3 weeks of gestation was referred to our hospital from a specialized cancer institute in view of sacral giant cell tumor in pregnancy requiring Cesarean Section to prevent any neurological deficit to the mother. Patient had complaints of severe backache since third month of gestation with radiating pain to both legs, difficulty in walking and complaints of constipation and increased frequency of micturition. The pain was initially considered to be due to pregnancy and no further examination was performed. She was given conservative treatment for two months, after which on consultation with a private orthopaedician, an MRI lumbar spine was done which revealed a well-defined lobulated irregular mass lesion in the sacrum, measuring 6.5cm x 6.8cm x 4.7cm, obliterating sacral spinal canal and extending anteriorly into the presacral space, representing a neoplastic lesion. Fine needle aspiration cytology of the mass done at the cancer institute was suggestive of sacral giant cell tumor. Hence, patient was referred to us for termination of pregnancy as patient needed urgent angioembolisation and bisphosphonate therapy which could not be initiated during pregnancy. She was admitted in antenatal ward, her vital parameters were stable with blood pressure ranging from 130/90mmHg to 160/100mm Hg despite complete bed rest and no proteinuria. Respiratory and cardiovascular examination was normal. Knee jerks were normal and she had no pre monitory symptoms. On per abdomen examination, uterus was corresponding to 24 weeks size gestation with external ballottement present with oligohydromnios.  She was started on tablet alpha methyl dopa 250 mg qid for hypertension and tablets morphine and gabapentin for pain relief as advised by the cancer specialists. All her blood investigations were normal. Ultrasonography was suggestive of a single live intrauterine gestation of 25.2 weeks with moderate oligohydromnios with no obvious congenital anomalies. Doppler was within normal limits. Orthopedic reference was taken, advised symptomatic pain relief and elective Caesarean Section in view of maternal interest. Patient was taken up for elective Cesarean Section under general anaesthesia at 34 weeks of gestation  in view of  severe intrauterine growth restriction and excruciating pain in her back and lower limbs  despite morphine and gabapentin. She delivered a female baby of 1.148 kg with an Apgar score of 9/10. Baby was admitted in neonatal intensive care unit for further management. Post-operative course in our hospital was uneventful. Patient was discharged on day 14 after suture removal and referred to the cancer institute for further management. Repeat MRI pelvis was done which was suggestive of a mass occupying lower four sacral vertebrae involving the erector spinae muscle posteriorly and pyriformis laterally and encasing the sacral nerve roots, with no involvement of sacro iliac joints. Patient underwent serial arterial embolization twice at 6 weeks interval. Embolization of feeding vessels from bilateral internal iliac arteries and sacral arteries was done using 100-300 um embospheres. Post procedure angiograms showed significant reduction in tumor vascularity. After two sessions of angioembolisation, patient was started on weekly injections of denosumab (120mg) given subcutaneously. Patient has taken four doses till now and is now asymptomatic and she has been advised the same injection once a month and has been asked to continue follow up at the cancer institute 6 weekly with MRI spine.

Figure 1: MRI at the time of diagnosis. Black arrow shows the tumor in the sacrum.

Figure 2: MRI image after delivery. Black arrow shows tumor in the sacrum, red arrow shows the lumbar vertebrae.

Discussion

Giant cell tumors are most common in the third and fourth decade of life with a predilection towards females in the ratio of 1.2:1. [5] Histologically, these tumors are benign with a vascularized network of spindle shaped stromal cells surrounding multinucleated giant cells. Sacral GCTs are a rare complication during pregnancy and are clinically silent in their initial stages and cause few symptoms until they achieve an extremely large size. Hence the detection of the tumor may be delayed since the pain is misinterpreted as a symptom of pregnancy, as in this case. Although histologically benign, these tumors are known to be locally invasive (<16%) and associated with pulmonary metastasis (1-9%) [6,7]. The local recurrence rate in the sacrum is higher than that of other skeletal locations. Investigation of choice is Lumbosacral magnetic resonance imaging (MRI). Sacral GCTs are difficult to treat because of their location and aggressive behavior. Wide resection of the tumor from the sacrum is associated with significant morbidity and life threatening intraoperative bleeding [8] and may lead to neurological damage and bowel and bladder dysfunction. Intralesional curettage, burring of the cavity and packing with bone cement is also one of the treatment options with good functional outcome and a local recurrence rate of 15% but is not feasible in sacral tumors as these infiltrate the spinal canal. [9] Hence treatment of pelvic GCTs by arterial occlusion has been preferred as a primary modality. This technique devascularizes tumors, causes reduction in their size, calcification of its margins and relieves pain. It requires an initial angiogram before embolization to visualize the feeding vessels and embolization is done using gel foam, stainless steel coils or polyvinyl alcohol[10], followed by reassessment with CT scan or MRI to evaluate the success of treatment. Serial arterial embolization is performed at regular intervals until there is decreased flow on angiography. In our case angioembolisation of bilateral internal iliac arteries and sacral arteries was done. Denosumab, a human monoclonal antibody is an efficient drug against RANK (Receptor activator of nuclear factor-kappa beta) ligand, which inhibits osteoclast function and helps in rapid suppression of bone turnover in these patients. It is used in unresectable or recurrent tumors and it is associated with inhibited disease progression (99%) [11] and reduced need for surgery. 90% of tumor necrosis has been reported with the use of denosumab. [12]. As our case involved extensive involvement of sacral canal with soft tissue involvement, denosumab was initiated in our patient. Calcium and Vitamin-D supplements need to be given to counteract side effects such as hypocalcaemia, hypophosphatemia. Other modality of treatment of sacral GCT is en bloc spondylectomy which is also associated with recurrence, incidence being less than wide resection. [13, 14] Surgery with adjuvant radiotherapy has a limited role since the chances of malignant transformation are higher than that of cure. [15]

Conclusion

In comparison to GCTs of long bones, tumors of the axial skeleton are more aggressive and need to be considered as a severe disease. Embolization deserves consideration as a primary and possibly the sole treatment of giant-cell tumors of the sacrum. Denosumab is also a promising drug in treatment and prevention of disease progression in GCT. Treatment requires close clinical and radiological follow-up, a multidisciplinary approach and a full commitment on the part of the patient and surgeon.

References

1. Nieminen U, Remes N. Malignancy during pregnancy. Acta Obstet Gynecol Scand 1970; 49: 315‐319.
2. Maxwell C, Barzilay B, Shah V, Wunder JS, Bell R, Farine D. Maternal and neonatal outcomes in pregnancies complicated by bone and soft‐tissue tumors. Obstet Gynecol. 2004;     104: 344‐348.
3. Bloem JL, Reidsma II. Bone and soft tissue tumors of hip and pelvis. Eur J Radiol. 2012; 81: pp. 3793–3801.
4. Turcotte RE, Sim FH, Unni KK. Giant cell tumor of the sacrum. ClinOrthop. 1993; 291: 215-221.
5. Balke M, Schremper L, Gebert C, Ahrens H, Streitbuerger A, Koehler G, et al. Giant cell tumor of bone: treatment and outcome of 214 cases. Journal of Cancer Research and Clinical Oncology 2008; 134(9): 969–978.
6. Guo W, Ji T, Tang X, Yang Y. Outcome of conservative surgery for giant cell tumor of the sacrum. Spine. 2009; 34: 1025–1031.
7. Althausen PL, Schneider PD, Bold RJ, Gupta MC, Goodnight JE, Jr, Khatri VP. Multimodality management of a giant cell tumor arising in the proximal sacrum: case report. Spine 2002; 27: E361–E365.
8. Wuisman P, Lieshout O, Sugihara S, van Dijk M. Total sacrectomy and reconstruction: oncologic and functional outcome. Clin Orthop Relat Res. 2000; 38: 192–203.
9. Mendenhall WM, Zlotecki RA, Scarborough MT, Gibbs CP, Mendenhall NP. Giant cell tumor of bone. American Journal of Clinical Oncology 2006; 29: 96–99.
10. Wallace S, Granmayeh M, De Santos LA, et al. Arterial occlusion of pelvic bone tumors. Cancer 1979; 43: 322-328.
11. Blay J, Chawla SP, Martin Broto J, et al. Denosumab safety and efficacy in giant cell tumor of bone (gctb): interim results from a phase ii study. J Clin Oncol. 2011; 29.
12. Chakarun CJ, Forrester DM, Gottsegen CJ, Patel DB, White EA, Matcuk GR. Giant cell tumor of bone: review, mimics, and new developments in treatment. Radiographics 2013; 33: 197–211.
13. Martin C and McCarthy EF. Giant cell tumor of the sacrum and spine: series of 23 cases and a review of the literature. The Iowa Orthopaedic Journal 2010; 30: 69–75.
14. Fidler MW. Surgical treatment of giant cell tumours of the thoracic and lumbar spine: report of nine patients. European Spine Journal 2001: 10(1): 69–77.
15. Shikata J, Yamumuro T, Shimizu K, Shimizu K, Kotoura Y. Surgical treatment of giant-cell tumors of the spine. Clin Orthopedics 1992; 278: 29-36.

Citation

J Madhavi, Warke HS, Satia MN. Sacral Giant Cell Tumor In Pregnancy: A Rare Occurrence. JPGO 2015 Volume 2 Number 12. Available from: http://www.jpgo.org/2015/12/sacral-giant-cell-tumor-in-pregnancy.html

Subacute Intestinal Obstruction Due To Classical Cesarean Section Scar Rupture

Author Information

Deshpande PS*, Maurya N**, Ansari M***, Gupta AS****.

(* First Year Resident, ** Fourth Year Resident, *** Assistant Professor, **** Professor; Department of Obstetrics & Gynecology, Seth G.S.Medical College & KEM Hospital, Mumbai, India.)

Abstract

Uterine scar dehiscence and subsequently rupture is one of the most dreaded complications encountered in obstetrics and is every obstetricians’ nightmare.  The varied presentation of the phenomenon of dehiscence and rupture from being silent to outright dramatic and life threatening poses a challenge to the obstetrician in terms of diagnosis and management. We present a case of uterine classical scar rupture, clinically simulating intestinal obstruction till we opened the Pandora’s box and got to the right diagnosis.

Introduction

Acute abdomen due to obstetric cases must be precisely distinguished from acute abdomen due to other causes as the approach to management and treatment differs widely in the two. The most common non obstetric cause of acute abdomen in pregnancy is appendicitis.[1] Commonest causes of acute abdomen in pregnancy are  ruptured ectopic in early pregnancy followed by red degeneration of myoma and ruptured ovarian cysts.[2] Intestinal obstruction presenting as acute abdomen in pregnancy is most commonly caused due to adhesions.[2] Only 0.05% patients who underwent surgery for gynecologic or obstetric causes had small bowel obstruction due to adhesion.[3]

Case Report

A 29 yrs old gravida 3, para 1, omtrauterine fetal death 1, abortion 1 with non isoimmunized Rh negative pregnancy with previous history of lower segment cesarean section with 34 weeks of gestation was referred from a private hospital as a case of preterm labor with suspected intestinal obstruction. She had no previous case records. The only information received from the patient was that cesarean section was done for prolonged preterm labor at 8 months of gestation in Uttar Pradesh 3 years ago. The patient was evaluated for the same and as she had no complaints of vomiting, distension of abdomen or obstipation, intestinal obstruction was excluded clinically. Her abdomen was soft to touch and had no signs of tenderness, guarding, or rigidity. Pfannestiel scar of previous cesarean section was seen. Uterine size corresponded to 34 weeks of gestational age. Minimal uterine activity was observed. There was no scar tenderness. On vaginal examination cervical os was closed and there was no show or amniotic fluid leak. The patient was admitted and treated for threatened preterm labor with tocolytics. She responded and was then discharged after 2 days of observation. She again presented after 2 weeks at 36 weeks of gestation with abdominal pain. She had no uterine activity, scar tenderness or vaginal bleed. An abdominal para-umbilical defect with herniated bowel loop was noted. Peristalsis in the herniated bowel loop was felt. Bowel loop returned back after reposition. She was kept under observation. On the second day, her pain increased in severity and she had 2-3 episodes of vomiting. The defect had increased and the bowel loop with persistent peristalsis could not be reduced. Surgeons evaluated the patient, detected the hyperperistaltic irreducible bowel loop and suspected intestinal obstruction. Decision for emergency laparotomy and lower segment cesarean section was taken. The surgery was performed jointly by the obstetrician and the surgeons. Surgery was done under general and epidural anesthesia. The abdomen was opened by a midline vertical incision. Dense adhesions were encountered between the rectus muscle and the anterior surface of the uterus. A bowel loop was found adherent to the anterior surface of uterus. After releasing the adhesions a full length classical cesarean section scar rupture was observed.

Figure 1. Adherent, traumatized ileal loop (yellow arrows) with classical cesarean incision rupture (blue arrows).

Figure 2: Full length classical cesarean section scar rupture (yellow arrows highlight the entire uterine rent).

Near term fetus with 9/10 Apgar scores was delivered through the previous ruptured classical scar. Approximately 5 cm loop of ileum was densely adhered to the ruptured scar. This ileal loop had a spiral, irregular rent measuring about 3 cm that was seen when it was separated from the edge of the ruptured scar after delivering the fetus. The lower edge of the opened scar did not reach the visceral peritoneum of the lower segment. The previous scar was excised and the uterus was repaired in two layers with polyglactin No.1.  Four inches of the bowel was resected and side to side anastamosis was done. Proximal diverting loop ileostomy was done and a stoma bag was attached. Postoperatively the patient had sepsis that settled with broad spectrum antibiotics. Stoma closure was advised after 3 months and proper stoma care was explained. Suture removal was done after 1 week. The patient improved clinically and was discharged. Couple was counseled about hazards of further pregnancies and the risk of performing any more surgery on the patient in view of permanent contraception and hence the patient’s husband was advised to undergo vasectomy to limit family size.

Discussion

Uterine rupture can be classified as complete or incomplete. It is complete when all the layers of the uterine wall are separated and incomplete when the visceral peritoneum is intact but the myometrial fibers give way.[4] The greatest risk factor for either form is previous cesarean delivery.[4] Metaanalysis of 25 studies from 1976 to 2012 indicate the overall incidence of pregnancy related uterine rupture is 0.07%.[5] Incidence of uterine rupture in an unscarred uterus varies demographically from 0.01% to 0.1%.[5] Incidence of uterine rupture in cases of previous cesarean section is 0.3%. Classifying further, risk of rupture in a previous case of low transverse uterine scar varies from 0.2 to 0.9% and it shoots up to 2-9% in a case of previous classical cesarean section.[4] An astonishing 10 fold increase in risk!  Classical cesarean section in today’s times is performed rarely and accounts for 0.5% of all births.[5] The probability for the performance of a classical cesarean delivery is inversely proportional to the gestational age at the time of delivery. The rate of classical cesarean sections in a study was 20% at 24 weeks. 5% and 1% at 30 weeks and at term respectively [6].

Our patient initially presented with abdominal pain and as uterine activity was present she was provisionally diagnosed and treated as a case of threatened preterm labor. She had no documents of the previous cesarean section. Verbally her previous medical practitioner had told her that cesarean delivery was done for prolonged labor at preterm gestational age. In the absence of any previous case records, operation notes, discharge summaries it was impossible for us to assume that a classical cesarean section had been performed as this is against the practice of standard care. Furthermore, presence of the Pfannestiel scar also pointed towards a lower segment uterine incision rather than an upper segment uterine incision. The access and the exposure of the upper segment to perform a classical section is not adequate with a Pfannensteil incision in the 8^th month of pregnancy. Hence the probability of a classical section and its consequence like scar thinning or rupture in the antenatal period was not contemplated. The patient presented again after 2 weeks with aggravation of the same symptoms. The presence of the fixed, irreducible, hyperperistaltic bowel loop through an abdominal right paramedian defect raised the suspicion of subacute intestinal obstruction due to adhesion. Exploratory laparotomy clinched the diagnosis. The hyperperistaltic loop that was adherent to the previous section scar had a spiral tear and its edges were not bleeding. This adherent loop formed a barrier and prevented the uterus from expelling out the fetus into the peritoneal cavity after the scar rupture. The placenta that was implanted posteriorly was not injured and uteroplacental blood supply was not compromised thus saving the fetus. Retrospectively, the scar thinning and rupture had probably set in 2 weeks back. Thus it was the surgical management of intestinal obstruction that led us to the final diagnosis and subsequent treatment. Performing a classical cesarean operation without proper indication, not adequately documenting the same and warning the patient of subsequent consequences should be strongly condemned as this not only increases the morbidity but also increases maternal and perinatal mortality and suboptimal care in subsequent pregnancies.  All the subsequent pregnancies have to be monitored with a hawk’s eye, with good paper work and antenatal care to pick up the minutest abnormalities from as early as 20 weeks to prevent any such catastrophes.

Conclusion

Cases of acute abdomen in pregnancy must be addressed seriously. Especially cases of previous cesarean section should be evaluated in detail if they present with complaints of acute abdomen. All the possible obstetric and nonobstetric causes must be thought of and the patient investigated accordingly. Surgical causes deserve a special mention in cases of previous cesarean section especially adhesions. Combine this with the fact that in many developing countries, patients do not maintain the records of the previous surgeries, like in our case; one must be ready for any kind of surprises.

References

1. Kameoka S, Ogawa S. Acute Abdomen in Pregnancy. Japan Medical Association Journal. 2001 44(11):496-500.
2. Taylor D, Perry RL. Acute Abdomen and Pregnancy. Available from: emedicine.medscape.com/article/195976-overview#a2
3. Al-Took S, Platt R, Tulandi T.Adhesion-related small-bowel obstruction after gynecologic operations. Am J Obstet Gynecol. 1999 Feb; 180(2 Pt 1):313-5.
4. Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL,et al. Prior Cesarean Delivery. Williams Obstetrics.24th ed. New Delhi: McGraw Hill Education 2014; pp.609-624
5. Nahum GG, Pham KQ. Uterine Rupture in Pregnancy. Available from: http://reference.medscape.com/article/275854-overview#a1
6. Chauhan SP. Prior classical cesarean delivery-counseling and management. Contemporary OB/GYN, 2012 Available from: http://contemporaryobgyn.modernmedicine.com/contemporary-obgyn/news/modernmedicine/modern-medicine-feature-articles/prior-classical-cesarean-del 

Citation

Deshpande PS, Maurya N, Ansari M, Gupta AS. Subacute Intestinal Obstruction Due To Classical Cesarean Section Scar Rupture. JPGO 2015. Volume 2 No. 12. Available from: http://www.jpgo.org/2015/12/subacute-intestinal-obstruction-due-to.html

Ovarian Torsion at Term Pregnancy- A Rare Case Report

Author Information

Sharma G*, Supe P**, Kore SJ***, Nandanwar YS****.
(* Specialty Medical Officer, ** Assistant Professor, *** Additional Professor, **** Professor and Head, Department. of Obstetrics and Gynecology, LTMMC and LTMGH, Mumbai, India.)

Abstract 

Ovarian torsion is rare in the second and third trimester of pregnancy. Diagnosis is hampered by non-specific symptoms common in pregnancy (nausea, vomiting). Ovarian torsion can be managed conservatively if diagnosed at an earlier gestational age. Characteristic clinical presentation along with the ultrasound evidence of adnexal mass helps in establishing the diagnosis. We report a case of 20 year gravida 2 para 1 living 1 (G2P1L1) with 36 weeks of pregnancy with huge right ovarian cyst. The diagnosis was made on abdominal ultrasound. She underwent elective lower segment caesarean section (LSCS) with removal of right gangrenous ovary and tube at 37 weeks.

Introduction

Pregnancy is associated with 5 times increased risk of ovarian torsion. Incidence is 5 per 10000 pregnancies as quoted by many studies.[1]  Most common adnexal masses associated with pregnancy are functional cysts, dermoid and serous cystadenomas.[1] Majority of the adnexal torsions (around 2/3) are right sided as the mobility of the left ovary is limited by the sigmoid colon. Most common risk factor for ovarian torsion is ovarian stimulation, as found during early pregnancy or infertility treatment.[2]   The symptoms of ovarian torsion like nausea, vomiting and pain in abdomen are nonspecific. These symptoms can be confused with other acute abdominal conditions such as appendicitis, ureteral or renal colic, cholecystitis and bowel obstruction.[1] Missed diagnosis of ovarian torsion can threaten the pregnancy. Adnexal torsion should be ruled out in a pregnant patient with ultrasound suggestive of ovarian mass.

Case Report

A 20 year old woman; married for 3 years, G2P1L1 with spontaneous conception was referred to our antenatal outpatient department  (OPD) at 36 weeks and 4 days of gestation with ultrasound report showing right ovarian cyst 18 x 10 x 10 cm without septations. She had no complaints of pain in abdomen, fever or vomiting. Her earlier scans did not show presence of adnexal mass. She had history of laparoscopic right ovarian cyst aspiration done in 2012. She had a previous normal vaginal delivery. On examination, her vital parameters were normal. There was no pallor. There was no tenderness, guarding or rigidity on abdominal examination. Uterine fundal height was corresponding with 34 – 36 weeks of gestation. The fetus was in longitudinal lie with cephalic presentation. Internal os was closed. Blood investigations were normal. Cancer Antigen 125 (CA 125) level was 15 U/ml. An obstetric ultrasound with color flow Doppler was repeated and it showed a single live intrauterine gestation corresponding to 36 weeks with fetal weight of 2.4 kg. Large anechoic cystic lesion in right adnexa 16 x 8 x 11 cm with few internal echoes within and without septation was seen.Color Doppler showed a healthy fetus with normal uteroplacental and fetoplacental blood flows. Large anechoic adnexal cyst with vascularity was seen. Decision for LSCS with removal of adnexal mass was taken. In situ findings – 20 x 18 cm gangrenous right ovarian cyst. The cyst had undergone torsion. Left side tube and ovary were normal. There was no hemoperitoneum. Baby was delivered by LSCS and gangrenous right ovarian cyst with tube was removed. Postoperative course was uneventful. Histopathology report confirmed gangrenous tube and ovary with hemorrhagic follicular cyst.

Figure 1. Ovarian Torsion in a case of full term pregnancy.

Discussion

Ovarian cysts associated with pregnancy can lead to following complications - torsion of the cyst, rupture, malignancy, and malpresentations of the fetus.[3] The risk of adnexal torsion rises by 5-fold during pregnancy. Diagnosis on pure clinical grounds is usually difficult.  Color Doppler plays an important role in establishing the diagnosis. Possibility of adnexal torsion should always be kept in mind during management, even if Doppler study shows presence of blood flow. MRI can be used as an adjunct to color Doppler.[4] Role of CA 125 in ovarian cyst in pregnancy is not clear. However according to American College of Obstetricians and Gynecologists (ACOG) guidelines, level of CA 125 antigen reaches the highest elevation in 1st trimester and then gradually declines. Thus CA 125 can be valuable in 2nd and 3rd trimester to differentiate between benign and malignant ovarian masses.[5] Surgical management is offered for cysts larger than 10 centimeters as they are associated with increased risk of malignancy, rupture or torsion.3 Surgery can be done by either laparoscopy or laparotomy. Laparoscopy becomes more difficult after second trimester
In our patient an ovarian cystectomy rather than cyst aspiration in the previous surgery might have prevented recurrence. It seems that the torsion in this case must have happened at an earlier gestation since patient was clinically asymptomatic and hemodynamically stable and also torsion is less likely with term pregnant uterus. Also diagnosis at an earlier gestation might have helped conserve our patient's ovary. Possibility of ovarian torsion should not be ruled out despite normal color Doppler findings.

References

1. Mohammed Khairy Ali,, Ahmed Y. Abdelbadee , Sherif A.Shazly , Ahmed M. Abbas. Adnexal torsion in the first trimester of pregnancy: A case report. Middle East Fertility Society Journal. 2013;: 284–286.
2. Case Report: Ovarian torsion in pregnancy- diagnosis and management. J Emerg Med.2013;45 (3):348 – 51. Doi: 10.1016/ J.Jemermed.2012.02.089
3. Lee CH, Raman S, Sivanesaratnam V. Torsion of ovarian tumors: a clinicopathological study. Int J Gynaecol Obstet 1989;28:21–25. doi: 10.1016/0020-7292(89)90539-0.
4. Born C, Wirth S, Stäbler A, Reiser M. Diagnosis of adnexal torsion in the third trimester of pregnancy: a case report. Abdom Imaging. 2004;29:123–127. doi: 10.1007/s00261-003-0079-x
5. Lisa Graham. ACOG Releases guidelines on Management of adnexal masses. Am Fam Physician. 2008 May 1;77(9):1320-1323. Available from www.aafp.org/afp/2008/0501/

Citation

Sharma G, Supe P, Kore SJ, Nandanwar YS. Ovarian Torsion at Term Pregnancy- A Rare Case Report. JPGO 2015. Volume 2 No. 12.Available from: http://www.jpgo.org/2015/12/ovarian-torsion-at-term-pregnancy-rare.html

Chronic Idiopathic Thrombocytopenic Purpura Causing Neonatal Thrombocytopenia

Author Information

Goel A*, Jain P**, Shende D***, Chauhan AR****.

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

Abstract

Idiopathic thrombocytopenic purpura (ITP) in pregnancy is uncommon. It usually presents in first trimester of pregnancy but may present at any point of gestation. A case of chronic ITP in pregnancy leading to neonatal thrombocytopenia is presented here.

Introduction

Thrombocytopenia complicates 10% of all pregnancies.[1] The most common cause of isolated thrombocytopenia in first and second trimesters of pregnancy is ITP, which accounts for 5% of all pregnancy associated thrombocytopenia. Incidence of ITP is 1-2/1000 pregnancies.[2] Making a diagnosis is complex and challenging as time of onset of thrombocytopenia and its clinical manifestations usually overlap. ITP may lead to maternal and neonatal complications. Its management requires collaboration between obstetrician and hematologist.

Case Report

A 35 year old female, diagnosed case of chronic ITP, Gravida 2 Para 1 with previous neonatal death, registered antenatally at 6 weeks of gestation with severe thrombocytopenia (platelet count of 10000/μL). She was diagnosed as a case of chronic ITP 4 years ago, when she was started on corticosteroids, azathioprine and dapsone. As she did not respond to medical treatment, she underwent splenectomy. She was then started on penicillin prophylaxis (tablet penicillin G 400 mg twice daily) and corticosteroids (tablet dexamethasone 40 mg for four days every month). She conceived 2 years later, and received 18 platelet transfusions and intravenous immunoglobulins during peripartum period. She delivered a male child of 2.5 kg by outlet forceps application. However, the baby died on day 1 of life due to unknown cause.

She was antenatally registered with us in this pregnancy and had regular follow-up. She was admitted at 34 weeks of gestation as she developed steroid induced diabetes mellitus. On examination, her vital parameters were normal. On abdominal examination the uterus was 34 weeks of gestation and relaxed, the presentation was cephalic, fetal heart sounds were regular (140 bpm). Vaginal examination was unremarkable.

Hematological investigations showed normal hemoglobin of 11.7 g/dL, normal total and differential white blood cell counts, but abnormality in the form of isolated thrombocytopenia (platelet count of 10,000/μL) and  anisocytosis, poikilocytosis, crenated red blood cells on peripheral smear. Her fasting and postprandial blood sugars were elevated FBS- 103 mg/dl, PLBS- 124 mg/dl, oral glucose tolerance test (100 g - Carpenter and Coustan) 99/121/102/93, Serum LDH was elevated (777 U/L). Renal, thyroid and liver function tests were within normal limits.

She was treated with tablet metformin and injection human insulin after endocrinologist’s opinion. Blood sugar levels and blood pressure monitoring was done. The treatment plan was to increase the platelet count prior to anticipated delivery. In view of her persistently low platelet counts, she was started on injection methyl prednisolone 500 mg IV for 3 days, which raised her platelet count to only 40000/μL. Injection dexamethasone 40 mg in 100 ml normal saline once daily for 4 days was given intravenously, which raised her platelet count to 86,000/μL. Intravenous immune globulin (IVIg) 1 g/kg body weight was transfused and her platelet count rose to 1,20,000/μL; hence induction was planned. However, she went into spontaneous labor a day prior to planned induction and delivered a male child of 2.6 kg vaginally. On day 2 of delivery, her platelet counts started to decline again (platelet count- 95,000 /μL).

In view of maternal history and previous neonatal loss, serial platelet counts of the neonate were done, and the baby developed neonatal thrombocytopenia (platelet count- 20,000/μL). The neonate was given 2 doses of injection methyl prednisolone intravenously on day 2 of life. Neonatal thrombocytopenia resolved spontaneously after a few days and both mother and child were discharged on day 7 after delivery.

Discussion

ITP may be subdivided into primary and secondary types. Secondary ITP may be due to autoimmune disorder, HCV, HIV, H. pylori infection. It is most commonly due to clearance of platelet coated by IgG antiplatelet antibodies in spleen and less commonly due to direct activation of complement system, diminished production, and alteration in regulatory T cells.[3,4,5] ITP is likely to occur if there is a history of thrombocytopenia in prior pregnancy, underlying autoimmune disease or severe thrombocytopenia especially in first trimester of pregnancy.[6]

According to American Society of Hematology (ASH) [7] and British Committee for Standards in Hematology[8] guidelines, treatment is required for women with platelet count <10,000/μL at any time during pregnancy or <30,000/μL in second or third trimester or when thrombocytopenia is associated with bleeding.

Corticosteroids are the first line of management during pregnancy. Prednisone and prednisolone are preferred in pregnancy. Therapeutic dose of prednisolone is 1 mg/kg (based on pre pregnancy weight) which needs to be titrated to lowest effective dose after achieving a response. However steroids may cause gestational diabetes, weight gain, hypertension, acceleration of bone loss, placental abruption and preterm labor, orofacial clefts in the first trimester. Our patient developed steroid-induced diabetes mellitus. High dose IVIg (2 mg/kg over 2-5 days) is required to increase the platelet count rapidly. Multiple courses of IVIg are required as the response is usually transient; as this is expensive its use should be just prior to anticipated delivery. Combination of steroids and IVIg is used for refractory cases. Laparoscopic splenectomy may be considered in second trimester in cases who do not respond to steroids or IVIg. Anti-D immunoglobulin is relatively contraindicated but may be given in third trimester to refractory cases.[9] Cytotoxic drugs and immunosuppressive drugs are avoided in pregnancy. 

Mode of delivery should be determined by obstetric indications. Cesarean section should be done for obstetric indication only. According to ASH guidelines, platelet count should be > 50,000/μL for vaginal delivery and cesarean section. But according to BCSH guidelines, platelet count should be >50,000/μL for vaginal delivery and > 80,000/μL for cesarean section and epidural anesthesia, which is currently followed in our institute. Corticosteroids can be started 10 days prior to anticipated delivery for a patient whose platelet count is <80,000/μL and has not required therapy during pregnancy, as we did in our case. When the delivery is imminent, IVIg should be considered. Platelet transfusion is generally not useful in ITP. In emergency cases, platelet transfusion along with IVIg can be considered.

5% of the offspring develop fetal thrombocytopenia. Nadir in the infant platelet count occurs 2 to 5 days after delivery which spontaneously rises by day 7 of delivery. Most reliable predictor of neonatal thrombocytopenia is history of thrombocytopenia in prior sibling at delivery.[10] Risk of intracranial hemorrhage in neonate is below 1%. [11]

Conclusion

ITP can complicate pregnancy and its management. More aggressive treatment is required for preparing the patient for labor and delivery. The outcome of pregnancy is generally good. Other causes of thrombocytopenia need to be ruled out.

References

1. Provan D, Newland A. Idiopathic thrombocytopenic purpura in adults. J Pediatr Hematol Oncol 2003;25(Suppl 1):S34.
2. McCrae, K. Thrombocytopenia in Pregnancy. In: Michelson, A., editor. Platelets. New York, NY: Elsevier; 2006.
3. Chang M, Nakagawa PA, Williams SA, et al. Immune thrombocytopenic purpura (ITP) plasma and purified ITP monoclonal autoantibodies inhibit megakaryocytopoiesis in vitro. Blood 2003;102(3):887.
4. McMillan R, Wang L, Tomer A, et al. Suppression of in vitro megakaryocyte production by antiplatelet autoantibodies from adult patients with chronic ITP. Blood 2004;103(4):1364.
5. McMillan R. The pathogenesis of chronic immune thrombocytopenic purpura. Semin Hematol 2007;44(4 Suppl 5):S3.
6. Gill KK, Kelton JG. Management of idiopathic thrombocytopenic purpura in pregnancy. Semin Hematol 2000;37(3):275.
7. Gernsheimer T, James AH, Stasi R, How I treat thrombocytopenia in pregnancy. Blood. 2013;121(1):38-47; and Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Jr., Crowther MA. The American Society of Hematology 2011 evidence based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190- 4207; and Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood.2010;115(2):168-186.
8. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. British Committee for Standards in Haematology General Haematology Task Force. Br J Haematol. 2003 Feb;120(4):574-96.
9. Michel M, Novoa MV, Bussel JB. Intravenous anti-D as a treatment for immune thrombocytopenic purpura (ITP) during pregnancy. Br J Haematol 2003;123(1):142-146.
10. Christiaens GC1, Nieuwenhuis HK, Bussel JB. Comparison of platelet counts in first and second newborns of mothers with immune thrombocytopenic purpura. Obstet Gynecol. 1997 Oct;90(4 Pt 1):546-52.
11. Nationwide study of idiopathic thrombocytopenic purpura in pregnant women and the clinical influence on neonates.Fujimura K1, Harada Y, Fujimoto T, Kuramoto A, Ikeda Y, Akatsuka J, Dan K, Omine M, Mizoguchi H. Int J Hematol. 2002 May;75(4):426-33.

Citation

Goel A, Jain P, Shende D, Chauhan AR. Chronic Idiopathic Thrombocytopenic Purpura Causing Neonatal Thrombocytopenia. JPGO 2015. Volume 2 No. 11. Available from: http://www.jpgo.org/2015/12/chronic-idiopathic-thrombocytopenic.html

Successful Management Of Recurrent Peripartum Cardiomyopathy

Author Information

Amin K*, Shende D**, Dhokia T***, Chauhan AR****.
(* Third Year Resident, ** Assistant Professor, *** Second Year Resident, **** Additional Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)

Abstract

Cardiomyopathy is a life threatening form of cardiac failure which usually occurs in late pregnancy or early peurperium. The incidence of cardiomyopathy in pregnancy is rare and seen in 1:1485 to 4000 live births.[1,2] The chances of recurrence in subsequent pregnancy is around 21-80%.[3] We present a case of recurrent peripartum cardiomyopathy despite rapid return of normal cardiac function after the first pregnancy.

Introduction

Cardiomyopathy is characterized by its rapid clinical course and probability of spontaneous recovery. There is a risk of recurrence of cardiomyopathy and even death in subsequent pregnancies if the ejection fraction is persistently reduced. However if ventricular size and function have returned to normal in the postpartum period, the risk of recurrence is low.

Case Report

A 34 years old patient with multiple high risk factors and bad obstetric history (BOH) was referred to our tertiary care center at 34 weeks of gestation for evaluation of persistent breathlessness. She was a known case of type 2 diabetes mellitus. She was obese (weight 109kgs), had BOH (Gravida 7, Para1, with one living issue and 5 spontaneous abortions) with one previous full term lower segment cesarean section in view of transverse lie, with gestational hypertension in this pregnancy.  She was on injection insulin and anti-hypertensive drugs. She presented to us with dyspnea on exertion (NYHA II), palpitations and decreased urine output since 15 days.
In the present pregnancy her general examination findings were suggestive of raised blood pressure of 140/90 mm of Hg with bilateral pitting pedal edema grade III. On abdominal examination, uterus was over-distended, abdominal wall edema was present, with single live fetus in longitudinal lie with cephalic presentation, no uterine activity, Pfannensteil scar of previous cesarean section with no scar tenderness. On per vaginal examination cervical os was closed.
Her hematological investigations were normal. Her 2-D echocardiogram done elsewhere 1 month prior to presentation in view of breathlessness was within normal limits. Ultrasound was suggestive of polyhydramnios with an amniotic fluid index of > 25 cm. After all other investigations, therapeutic amniocentesis was done as no other cause was found for her breathlessness; around 1 L of amniotic fluid was drained but patient was still symptomatic.
Hence cardiology opinion was sought and 2-D echocardiogram was done which was suggestive of left ventricular ejection fraction of 30% with moderate mitral regurgitation, tricuspid regurgitation and severe pulmonary hypertension. Diagnosis of peripartum cardiomyopathy was made. The patient was shifted to cardiac intensive care unit and was started on digoxin and intravenous frusemide drip along with insulin and antihypertensive drugs. While explaining to the patient about her heart condition, she revealed that in previous pregnancy she had peripartum cardiomyopathy for which she was managed in intensive care unit; she did not reveal this history as she thought the problem had been resolved.
After stabilization in ICU, emergency lower segment cesarean section with copper T insertion was done under general anesthesia in view of severe pulmonary hypertension and moderate maternal condition. Female child of 3.4 kg was delivered with good Apgar score. Patient was monitored in cardiac ICU. Her postoperative recovery was uneventful and she was discharged on day 7.

Discussion

In 1971, Demakis and colleagues first defined peripartum cardiomyopathy as idiopathic heart failure which occurs in the absence of any preexisting heart disease in the last month of pregnancy or during the first 5 months postpartum.[4] There are multiple risk factors for cardiomyopathy such as maternal age >35yrs, gestational hypertension, preeclampsia, multiple pregnancy, multiple gestations, obesity, and family history.
Chances of recurrence in subsequent pregnancy is around 21-80%. The risk of recurrence of cardiomyopathy is highest in patients who have persistent cardiac dysfunction. However the  risk is lowest in those whose cardiac functions have been returned to normal & this can be detected by performing dobutamine stress test. Recurrence has also been reported in patients whose ventricular size and function have returned to normal as in our case.[5,6] They usually present with dyspnea on exertion, cough, orthopnea and paroxysmal nocturnal dyspnea and often mimic left ventricular failure (LVF). Nonspecific symptoms like palpitations, fatigue, malaise and abdominal pain may be present in 50% of cases.[5,7] Gallop rhythm, engorged neck veins, tachycardia and pedal edema are commonly found.
Diagnosis can be established by excluding common causes of cardiac failure such as toxins, infection, and ischemic, metabolic or valvular heart disease and it requires high degree of suspicion. Many features of cardiomyopathy resembles that of advanced pregnancy making the diagnosis difficult for doctor.[1] Electrocardiogram, chest radiograph and Doppler echocardiograph are most commonly used diagnostic modalities. Doppler echocardiography is the gold standard diagnostic tool for assessing the severity and prognosis of the disease. Criteria used to establish diagnosis are: left ventricular ejection fraction (LVEF) 45% or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m2. Common differential diagnoses include accelerated hypertension, preeclampsia, idiopathic cardiomyopathy, pulmonary embolism, anemia and thyrotoxicosis.[8] Complications such as arrythmias, thromboembolism, multiorgan failure, increased incidence of abortion (4-25%), premature delivery (11-50%), low birth weight and small for date babies, intrauterine growth retardation and fetal deaths are associated with it.[9] Congenital fetal anomalies can occur in 4-6% of cases.
Digoxin, diuretics, vasodilators, β blockers and anticoagulants are used for routine medical management. Immunosuppressive drugs, immunoglobulin and pentoxifylline can be considered in resistant cases. Severe cases may need intensive management, including mechanical circulatory support and heart transplant.
A multidisciplinary approach involving an obstetrician, cardiologist, anesthesiologist and neonatologist may be required for management of such patients. Induction of delivery should be considered if a patient's condition worsens despite maximal medical management.[5,6] Regional techniques are safer for both labor analgesia and anesthesia. Second stage of labor should be cut short using outlet forceps or vacuum to minimize cardiac stress. Cesarean section to be considered for obstetric indications and in situations were normal delivery can deteriorate further condition of patient as in our case. Following delivery, these patients should be monitored in an intensive care unit for prompt detection and management of uterine autotransfusion-induced pulmonary edema.[10,11]. Mortality rate is around 50%.[6]  Future pregnancy is better avoided in patients with persistent cardiac failure.

References

1. Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, et al. Peripartum cardiomyopathy: National Heart, Lung and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendation and review. JAMA 2000;283:1183–1188.
2. Whitehead SJ, Berg CJ, Chang J. Pregnancy-related mortality due to cardiomyopathy: United States, 1991-1997. Obstet Gynecol 2003;102:1326–1331.
3. Fett JD, Carraway RD, Dowell DL, King ME, Pierre R. Peripartum cardiomyopathy in the Hospital Albert Schweitzer District of Haiti. Am J Obstet Gynecol 2002;186:1005–1010. 
4. Demakis JG, Rahimtoola SH, Sutton GC, Meadows WR, Szanto PB, Tobin JR, et al. Natural course of peripartum cardiomyopathy. Circulation. 1971;44:1053–1061.
5. Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy. Lancet. 2006;368:687–693.
6. Ro A, Frishman WH. Peripartum cardiomyopathy. Cardiol Rev 2006;14:35–42.
7. Sliwa K, Skudicky D, Bergemann A, Candy G, Puren A, Sarelil P. Peripartum cardiomyopathy: analysis of clinical outcome, left ventricular function, plasma levels of cytokines and Fas/Apo-1. J Am Coll Cardiol 2000;35:701–705. 
8. Ray P, Murphy GJ, Shutt LE. Recognition and management of maternal cardiac disease in pregnancy. Br J Anaesth 2004;93:428–439.
9. Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, et al. Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005;111:2050–2055. 
10. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003;24:761–781. 
11. Ray P, Murphy GJ, Shutt LE. Recognition and management of maternal cardiac disease in pregnancy. Br J Anaesth 2004;93:428–439.

Citation

Amin K, Shende D, Dhokia T, Chauhan AR. Successful Management Of Recurrent Peripartum Cardiomyopathy. JPGO 2015. Volume 2 No. 12. Available from: http://www.jpgo.org/2015/12/successful-management-of-recurrent.html

Primary Amenorrhea Of Combined Etiology

Author Information

Prasad R*, Parulekar SV**.

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

Abstract

A girl can present with primary amenorrhea, which may be due to cryptomenorrhea due to an obstruction of the genital outflow tract. In presence of normal genital tract, there can be a number of causes. We present a case in which there was cervical and vaginal aplasia combined with an end organ (endometrial) defect. This is the first case of this type in the world literature.

Introduction

Primary amenorrhea can be due to a number of causes. Cryptomenorrhea could be a cause of a primary absence of menstruation associated with an outflow tract obstruction of the genital tract. In presence of normal anatomy of the genital tract, primary amenorrhea can occur due to genital tuberculosis. We present a case in which there was aplasia of the vagina and the cervix, normal uterine corpus, normal appearance endometrial shadow on ultrasonography (USG) and no cryptomenorrhea. Possible etiology of the condition is discussed.

Case Report

An 18 year old unmarried woman presented with primary amenorrhea. She had a history of cyclical lower abdominal pain for 3 to 4 years. It was experienced every month, lasting for 2 to 3 days every time. There was no history of any bleeding per vaginum or leucorrhea. There were no bladder or bowel complaints. The breast development had occurred at the age of 13 years, and axillary and pubic hair development had occurred at the age of 12 years. There was no history suggestive of any medical disorder including tuberculosis, or any endocrine disorder. Her mother had menarche at the age of 12 years. Her vital parameters were normal and general and systemic examination revealed no abnormality. Breasts development of Tanner's stage 5. There was no galactorrhea. Axillary and pubic hair development was stage 5. Thyroid was normal. There was no hirsutism. There was no abnormality on abdominal examination. Local examination showed an absence of vagina. There was a shallow dimple at the position of the expected vaginal opening. External genitals were normal. Rectal examination showed a normal sized uterus. There was no pelvic tenderness or mass. Abdominopelvic ultrasonography showed uterus measuring 6x3.9x2.8 cm, central endometrial echo measuring 8.1 mm, 2x1.7 cm sized cyst in the right ovary, 2.3x1.9 cm cyst in the left ovary, normal kidneys, and no fluid in the pelvis or uterine cavity. There was no vagina. Her hemogram, thyroid, liver and renal function tests, serum prolactin level,and chest radiograph were normal. 

Figure 1. USG of the pelvis showing normal uterus with endometrial echo, and absence of cervix and vagina.

A laparoscopy was performed under general anesthesia. The uterus was of normal size and shape. The fallopian tubes and ovaries were normal. Uterine ligaments were normal. Cervix was not seen producing an elevation on the anterior wall of the pouch of Douglas, between the uterosacral ligaments. Mild inflammatory fibrosis and a thin band of adhesion was seen around the left ovary. No endometriosis was seen anywhere. Bowel, bladder, omentum and liver were normal. There was no evidence of tuberculosis. The patient made an uneventful recovery. TB Gold test was performed on her postoperatively. Its result was negative. The woman was counseled and offered vaginoplasty and creation of an opening in the lower end of the uterus into the neovagina. She opted to undergo that when she planned to get married.

Discussion

In a woman with an outflow tract obstruction in the form of imperforate hymen, vaginal agenesis or atresia, cervical atresia or agenesis, the patient suffers from cryptomenorrhea.[1,2] The menstrual blood collects behind the site of the obstruction and distends the genital tract, producing hematocolpos (if the obstruction is in the vagina or at the introitus),  hematocervix (if the obstruction is below the level of the internal os), and hematometra. Usually the patient presents early, with cyclical lower abdominal pain, and often retention of urine due to elevation of the bladder neck and elongation of the urethra. The endometrium is functional, and recovery is complete when the obstruction is relieved surgically. There can be residual complication in the form of pelvic endometriosis. Primary amenorrhea can be a presenting feature of genital tuberculosis, though secondary amenorrhea is far more common.[3,4,5] The amenorrhea is due to an end organ failure, the endometrium being destroyed by tuberculous inflammation and often being replaced by fibrous tissue.[6]

In the case presented, the woman had cervical and vaginal agenesis, while the uterine corpus was normally developed and had normal endometrial shadow on ultrasonography. Thus she should have been menstruating and had cryptomenorrhea. But there was no fluid in the uterine cavity. This would be possible only if there was end organ defect in the form of endometrial destruction due to tuberculosis. If she had contacted the disease in early childhood, then the genital infection could have manifested before menarche, and then she would have primary amenorrhea, but no cryptomenorrhea. But all investigations failed to reveal the presence of tuberculosis. It is possible that she still had tuberculosis in the endometrium, which could not be sampled for histopathological examination and TB-PCR test. The other possibility was Leon Israel syndrome. It is a rare disorder, with only seven cases in the world literature. The condition is characterized by normal uterus, normal ovulation, primary amenorrhea, and successful pregnancies without any treatment. The defect is believed to be an absence of a proteolytic enzyme in the endometrium, which prevents the normal breakdown of the endometrium at the time of the menstruation, which gets resorbed and then grows again in the next cycle.[7,8] Owing to rarity of the condition, the patient presented is more likely to have genital tuberculosis rather than Leon Israel syndrome. The chronic inflammatory lesions on the left side adnexa would support the diagnosis. The final diagnosis may be possible when the woman plans to get married and undergoes a vaginoplasty, with creation of an opening in the lower end of the uterus communicating with the neovagina. The endometrium can be curetted at that time and studied histologically as well as by TB-PCR test.

References

1. Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Brolmann H, Gianaroli L, Campo R. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod 2013;28:2032–2044.
2. Rock JA, Breech LL. Surgery for Anomalies of the Mullerian Ducts. In Te Linde's Operative Gynecology, 10th Edition. 10th Edition. Rock JA, Jones HW III. Eds 2008 Lippincott Williams & Wilkins. pp. 539-584.
3. Samal S1, Gupta U, Agarwal P. Menstrual disorders in genital tuberculosis. J Indian Med Assoc. 2000;98(3):126-7, 129.)
4. Schaefer G. Female genital tuberculosis. Clin Obstet Gynecol 1976;19:223-39.
5. Reiss, HE.  Primary amenorrhoea as a manifestation of pelvic tuberculosis. BJOG: An International Journal of Obstetrics & Gynaecology, 1958;65: 734–738. doi: 10.1111/j.1471-0528.1958.tb08863.x
6. Malkani PK: Epidemiology of genital tuberculosis in India. In Rippman ET, Wenner R (eds): Latent Genital Tuberculosis. Basal, S Karger, 1966. pp 27, 35.
7. Parulekar SV, Bhattachryya M. Pregnancy in Leon Israel syndrome (Journal of Obstetrics and Gynecology of India 1986;36:6-7.
8. Israel SL. Menstrual disorders and sterility. Fifth ed New York. Harper and Row. 1967. p 269.

Citation

Prasad R, Parulekar SV. Primary Amenorrhea Of Combined Etiology. JPGO 2015. Volume 2 Number 12. Available from: http://www.jpgo.org/2015/12/primary-amenorrhea-of-combined-etiology.html