Volume 2 Issue 3, March 2015

Editorial
Chauhan AR

Dilatation And Curettage In A Patient Of Eisenmenger’s Syndrome: Anaesthesia Implications
Fernandes  S, Mali A, Ritika Deo R.

Pregnancy Complicated By A Large Leiomyoma: A Case Report.
Shukla H, Kakade AS.

Unruptured Pregnancy In Rudimentary Horn Of Unicornuate Uterus.
Nikam P, Jain A, Pardeshi S, Mayadeo NM.

A Case Of Postpartum Pubic Diastasis.
Mhaske N, Madhva Prasad S, Kharat D, Fonseca MN.

Pregnancy Termination In Case Of Hemophilia B
Shukla H, Dudhe M, Channawar S, Chauhan AR.

Intraplacental Hematoma: A Rare Cause Of Non-Immune Hydrops.
Parekh NA, Agrawal A, Badade A, Satoskar P.

OEIS Complex
Patel A, Gupta AS.

A Rare Case Of Youssef’s Syndrome
Panchbudhe S, Poonia S, . Satia MN, Pathwardhan S.

Bilateral Ovarian Sertoliform Endometrioid Carcinoma
Malviya K, Fernandes G, Satia MN.

Quiz March 2015

Editorial

Chauhan AR

Pregnant women are vulnerable to many communicable and non- communicable diseases; either the susceptibility to or severity of infections increases due to pregnancy. Vaccination programmes are one of the most important and cost effective health interventions. Fear of adverse effects makes clinicians and mothers hesitant to vaccinate in pregnancy and the obstetrician is only attuned to administering tetanus toxoid (TT); this article addresses the recent recommendations for vaccination of pregnant and postpartum women with Td (tetanus and diphtheria), Tdap (Tetanus toxoid, reduced diphtheria toxoid and acellular pertussis vaccine), influenza and other vaccines.
General guidelines for vaccination of pregnant women include evaluation for the possibility of pregnancy before immunization and immunization history. As a rule, live viral vaccines are contraindicated during pregnancy; however this risk is largely theoretical. The benefits of vaccinating pregnant women are usually greater than the potential risks when the possibility of exposure to that particular infection is high, when the infection would possibly harm the mother or fetus or when the vaccine is unlikely to harm them. Inactivated viral vaccines, bacterial vaccines and toxoids are safe in pregnancy. Women who have inadvertently received live vaccine during pregnancy should not be counseled to terminate the pregnancy for teratogenic risk; however non pregnant women who have received live vaccine should delay pregnancy for at least 4 weeks. This is a change from the previous recommendation of avoidance of pregnancy for 3 months. Another important point of intervention is the postpartum period, where breast feeding women can be immunized safely.

Td

Both tetanus and diphtheria toxoids (Td) and TT vaccines have been used extensively in pregnant women worldwide to prevent neonatal tetanus; their administration has not been shown to be teratogenic. Current recommendation is that everyone should be given a booster shot for tetanus and diphtheria every 10 years after first being immunized; hence Td during pregnancy has replaced TT in many parts of the world.

Tdap

Pertussis is a highly contagious respiratory infection caused by Bordetella pertussis which colonises respiratory tract mucous membranes, produces toxins that damage mucosa and induce systemic effects; the typical spasms of coughing ending with a “whoop”. Both the incidence and mortality are underestimated and underreported. Older individuals, especially parents, represent a reservoir of infection transmitting disease to unvaccinated or partially vaccinated infants. Despite generally high coverage with childhood pertussis vaccines, it is one of the leading causes of deaths worldwide. In recent years, a decrease in diphtheria– pertussis–tetanus (DPT) vaccine compliance has been reported from different parts of India.
“Cocooning” is the strategy of vaccinating parents, siblings, grandparents, and health workers who are likely to have close contact with an infant aged <12 months. These contacts, especially pregnant women in the later part of pregnancy or immediately postpartum should receive Tdap to reduce the risk for transmission of pertussis to infants, ideally at least 2 weeks prior to the anticipated contact. Tdap is a combination vaccine that contains tetanus (T), diphtheria (d) and acellular pertussis (ap) in a single injection, and provides protection against all three; it is approved for adolescents from the age of 11 (younger contacts should receive DTaP) and adults ages 19 to 64. There is no live vaccine component in Tdap. Some studies have suggested that maternal antibodies to pertussis can inhibit production of active pertussis-specific antibody after administration of DTaP vaccine to infants of mothers vaccinated with Tdap during pregnancy, referred to as “blunting”. However, the benefit of protection given by maternal antibodies in newborn infants is much higher than the risk of shifting the burden of the disease to later in infancy.
It is recommended to give one dose of Tdap during each pregnancy irrespective of the patient’s prior history of receiving Tdap. Although Tdap may be given at any time during pregnancy, the ideal time for administration is between 27 and 36 weeks’ of gestation, which maximizes the maternal antibody response and transfer of antibodies to the neonate. The first dose of TT should be replaced with Td and the second, with Tdap. If Tdap is not administered during pregnancy, it should be administered soon after delivery. Along with several international agencies like Advisory Committee on Immunization Practices (ACIP) of CDC, Indian Academy of Pediatricians (IAP) in their 2013 guidelines also strongly recommend Tdap to pregnant women.

Influenza

Influenza is a highly contagious acute respiratory illness caused by infection with influenza viruses (Orthomyxoviridae) which affects the upper and lower respiratory tracts and produces systemic signs and symptoms. Three types, Influenza A, B and C are determined by nuclear material. Influenza A subtypes are further divided based on H and N surface glycoproteins.
“Flu” is often mistaken for the common cold, but has serious and far reaching complications. Global pandemics and epidemics cause a huge disease burden; though pregnancy does not increase susceptibility to influenza infection, pregnant women in the second and third trimesters of pregnancy are at increased risk for hospitalization, severe illness, serious complications like pneumonia and death. Maternal influenza infection has also been associated with an increased risk of schizophrenia (which is higher with first trimester exposure) in the offspring. There is also an increased risk of preeclampsia in mothers and preterm labor, low birth weight, lower Apgar scores at birth and stillbirth. It is critical to vaccinate against influenza before the season begins; however this cannot be predicted accurately due to geographical and other variations. Hence routine influenza vaccination is recommended for all women who are or will be pregnant (in any trimester) during influenza season. In case of outbreaks of influenza prior to the season, vaccination should be given to all pregnant women. A single dose of the trivalent inactivated vaccine (TIV) intramuscularly is recommended; the live attenuated nasal spray is contraindicated in pregnancy. Immunization to the mother affords protection to the infant for at least the first 6 months of life. ACOG guidelines state that “preventing influenza during pregnancy is an essential element of prenatal care, and the most effective strategy for preventing influenza is annual immunization”. This is endorsed by many other agencies including WHO, ACIP (CDC) and Indian Association of Physicians (API).

Hepatitis B

Hepatitis B poses a serious risk to infants at birth and universal screening with HBsAg for all pregnant women is recommended. Pregnant women who are considered as being at risk of developing HBV infection during pregnancy (more than one sex partner during the preceding 6 months, evaluated or treated for an STI, recent or current intravenous drug abuse, or an HBsAg-positive sex partner) should be tested with antibody to surface antigen (anti –HBs) and be vaccinated if negative. Vaccination should be given only if clearly needed and possible advantages outweigh the possible risks. There are limited data which suggest that developing fetuses are not at risk for adverse events following hepatitis B vaccine. Babies should get HBIG at birth and first dose of vaccine within 12 hours of life.

HPV vaccine

HPV vaccines are not recommended for use in pregnant women. If a woman gets pregnant after initiating the vaccination series, no intervention is needed but the remainder of the 3-dose series should be delayed until completion of pregnancy.

Postpartum vaccination

The puerperal period is an excellent opportunity for vaccination and promotion of positive health advice. It is safe for a woman to receive routine vaccines after birth and in the lactation period. A woman who has not received Tdap or influenza vaccine should be vaccinated right after delivery and a woman who is not immune to measles, mumps and rubella and/or varicella (chicken pox) should be vaccinated before she leaves the hospital. HPV vaccine is also safe during the lactation period.
In conclusion, maternal vaccination is a cost-effective and targeted strategy to improve pregnancy outcomes in developed and developing countries. Many national organizations like IAP, Indian Association of Physicians (API) and FOGSI, and all international public health agencies recommend vaccination during pregnancy for influenza and Tdap. Lack of awareness of benefits and concerns about vaccine safety in pregnancy are common barriers to vaccination. The real challenge is to educate health professionals and make these vaccinations routine.

Dilatation And Curettage In A Patient Of Eisenmenger’s Syndrome: Anaesthesia Implications

Author Information

Fernandes  S*, Mali A**, Ritika***, Deo R****.
(Additional Professor, Assistant  Professor, Second Year Resident,
Department ofAnaesthesia, T N Medical College and BYL Nair Hospital, Mumbai, India.)

Abstract

The Eisenmenger’s syndrome consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular or aortopulmonary level. The pathophysiological effects usually worsen during pregnancy increasing the risk of a poor maternal outcome.[1] There are numerous reports of patients with Eisenmenger’s syndrome posted for non-cardiac surgeries. Both general and regional anaesthesia have been successfully used in these patients. We discuss the  anaesthetic goals and  intra-operative management of a primigravida undergoing dilatation and curettage under total intravenous anaesthesia.

Introduction

Maternal mortality in patients with Eisenmenger’s syndrome is as high as 30-50% and these women are usually advised to avoid conception. The increased blood volume of pregnancy is accommodated due to vasodilation in the pulmonary and the systemic vascular bed. Patients with Eisenmenger’s are at risk of right ventricular strain due to the irreversible increase in pulmonary vascular resistance. The decrease in systemic vascular resistance that normally occurs during pregnancy can exaggerate the right to left shunt thus worsening the hypoxemia.

Case-Report

A 23year old  female gravida 1,para 0 , weighing 30 kg, known case of Eisenmenger’s syndrome was admitted with pain in the abdomen since 3 days and bleeding per vaginum since 8days.She gave history of consuming an abortifacient at a private clinic two weeks ago. At three years of age she was advised surgery for ventricular septal defect but did not undergo the procedure due to financial constraints. She had been advised to avoid strenuous work as she suffered  dyspnoea Grade II . She was hospitalised for chest pain, syncope and dyspnoea Grade IV  two years ago. Electro-encephalography did not reveal any epileptiform focus. She was advised Tab Sildenafil 25 mg BD.

Pre-operative physical examination revealed Grade II clubbing, pulse rate of 90/min with occasional missed beats, blood pressure of 100/70mmHg and room air saturation of 95% .No cyanosis, peripheral oedema or rise in jugular venous pressure was observed. Lungs were clear to auscultate, there was a pansystolic murmur  over the left sternal border and a loud P2. ECG showed rSR | in I, aVL, R in V1 and RS in V1-V3 with occasional ventricular premature complexes. Two dimensional echocardiography with colour Doppler revealed a large midmuscular VSD with bidirectional shunt, good biventricular function and Pulmonary artery systolic pressure (PASP) by Tricuspid regurgitation (TR) jet of 110mmHg. Her haemoglobin was 12.5 g/dL with a hematocrit of 40% and a platelet count of 212000/cmm. Prothrombin time 19.6 sec, Prothrombin index 71.43 and International Normalised Ratio  (INR) 1.51. Ultrasonography  showed a intrauterine sac but no yolk sac or foetal pole. She had received Tab Misoprost 200mcg sublingually 4 hourly. Infective endocarditis prophylaxis was given. Monitoring included Electrocardiography, Noninvasive  Blood Pressure, Capnometry and Pulse-oximetry.  Intravenous line was secured with a 18 G intracath  with special precaution to avoid any air entrapment that could predispose to paradoxical air embolism. We did not place an arterial line and central venous catheter since it was a minor procedure. The  obstetrician had planned dilatation and endometrial curettage  under a paracervical  block but the patient was extremely anxious.She was given i.v midazolam 0.5mg, i.v fentanyl 70 μg and i.v. ketamine 30 mg. She maintained a saturation of 99% breathing spontaneously with face mask. Intra-operatively she continued to have Venticular Premature Contractions (VPCs) but remained hemodynamically stable. Post-operative course was uneventful and patient was discharged on day 3.

Discussion

Eisenmenger’s syndrome a term coined by Paul Wood is defined as Pulmonary Vascular Obstructive Disease that develops as a consequence of a large pre-existing left to right shunt such that pulmonary artery pressures  approach systemic levels and the direction of flow becomes bilateral  or right to left. Congenital heart defects that can result in Eisenmenger’s syndrome include simple defects such as Atrial septal defect (ASD), Ventricular septal defect (VSD)  and Patent ductus arteriosus (PDA) as well as complex defects such as Atrioventricular septal defect, truncus arteriosus, aortopulmonary window and univentricular heart. The high pulmonary vascular resistance (PVR)  is usually established   by the age of  2 years  except in ASD and is sometimes present from birth.[2]  In the absence of  complications, un-operated patients generally have an excellent to good functional capacity up to their third decade and thereafter usually experience a progressive decline in their physical abilities.  Most patients survive to adulthood with a reported 77% and 42% survival rate at 15 and 25 yr of age respectively.[3]
Thromboembolic phenomena are responsible for as many as 43% of all maternal deaths with many occurring as late as 4-6 weeks after delivery.[4] These women are often unable to respond to the increased demands for oxygen during pregnancy. The decrease in PVR seen in normal pregnancy does not occur in these women because PVR is fixed. The decrease in Systemic vascular resistance (SVR) associated  with pregnancy tends to exacerbate the severity of the right to left shunt. The pregnancy associated decrease in Functional residual capacity (FRC) also may predispose to hypoxemia.[5]
Primary goals of anesthesia management are: to maintain adequate SVR, maintain intravascular volume and venous return, avoid aortocaval compression, prevent pain,myocardial depression, hypoxemia, hypercarbia and  acidosis which may cause an increase in PVR. Several disadvantages are associated with the use of general anesthesia. Positive pressure ventilation results in decreased venous return which compromises cardiac output.The volatile anesthetic agents can cause myocardial depression and decreased SVR. Hofland et al have successfully conducted a laproscopic cholecystectomy by the use of xenon anaesthesia and obtained cardiovascular stability.[6]   Induction agents such as thiopental or propofol  decrease both contractility and SVR which may exacerbate a right to left shunt.  Ketamine  has been recommended as drug of choice for induction because it has little effect on pulmonary and  systemic vascular resistance,  maintains spontaneous respiration and intact  laryngopharygeal reflexes.[7]
Pretreatment with phenylephrine to prevent changes in SVR is also recommended although it may cause similar vasoconstriction in both pulmonary and systemic circulations and therefore have only little effect on shunt direction.[8] Increase in SVR will cause increased left to right shunting with a further increase in pulmonary pressure and right ventricular failure. Volume overload has the same effect and hence fluids have to be administered judiciously.[9] Tsutsumi et al performed a paracervical block using 1% lidocaine 12ml under 6 L oxygen inhalation through a face mask.[10]
Although some physicians have stated that a Pulmonary artery catheterisation (PAC) is essential for the management of pregnant women with eisenmengers,[11] there are some who believe that a PAC may be relatively contraindicated for several reasons.[12]   In the presence of a large intracardiac shunt, measurement of cardiac output by thermodilution may not be accurate, these patients may not tolerate arrhythmias , Pulmonary artery pressure (PAP) monitoring rarely yields useful information in the presence of severe fixed pulmonary hypertension and the PAC may predispose to pulmonary thromboembolism. To avoid to the risk of invasive cardiac output monitoring in Eisenmenger syndrome, transthoracic bioimpedence cardiography has been used.[13] Weber et al discussed the role of low dose heparinization to prevent thromboembolism,[14] however this was not considered  in our patient as it was a short procedure and she was ambulatory soon.
A multidisciplinary approach, judicious perioperative monitoring and knowledge of pathophysiology of Eisenmenger’s syndrome is essential for a successful outcome.

References
  1. Lieber S, Dewilde P, Huyghens L, Traey E, Gepts E. Eisenmenger syndrome and pregnancy. Acta Cardiol 1985;40:421-4
  2. Gary D Webb, Jeffrey F. Disease of the heart , Pericardium and Pulmonary Vasculature.  In: Braunwald’s Heart  Disease : A Textbook of Cardiovascular Medicine , Edn.Philadelphia  .W.B.Saunders 19. Libby Borrow Mann Zipes pg 1829-1853.  Saunders 1600 John F Kennedy B oulevard Suite 1800 Philadelphia PA 19103-289
  3. Saha A, Balakrishnan KG, Jaiswal PK, Venkitachalam CG, Tharakan J, Titus T, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol. Jul 1994;45(3):199-207.
  4. American College of Obstetrics and Gynecology Cardiac Disease in     Pregnancy.    ACOG Technical Bulletin No 168,1992
  5. Harnett M , Mushlin P , Camann W . Cardiovascular Disease  In :Chestnut DH ,Obstetric Anesthesia  . Principles and Practice pg 709-710 .Third Edition , Elsevier  Philadelphia, Pennsylvania 19106 .
  6. J.Hofland, I.Gultuna, R Tenbrinck  Xenon anaesthesia for laproscopic cholecystectomy in a patient with Eisenmengers syndrome. Br J Anaesth (2001) 86(6): 882-886
  7. Tweed WA, Minuck M, Mymin D(1972) Circulatory responses to ketamine anaesthesia. Anesthesiology 37: 613-619
  8. Lyons B, Motherway C, Casey W, Doherty P. The anaesthetic Management of the child with eisenmengers syndrome. Can J Anaesth 1995; 42:904-9
  9. Young.D and Mark,H. Fate of the patient with the Eisenmenger syndrome. Am J Cardiol 1971; 28:658-669
  10. Tsutsumi Y, Mizuno J, Takada S, Morita S.  Paracervical block for dilatation and curettage in a parturient with eisenmenger syndrome. Masui 2010 Mar; 59(8): 379-82
  11. Spinnato JA, Kraynack BJ,  Eisenmengers  syndrome in pregnancy: Epidural analgesia for elective caesarean section. N Engl J Med 1981; 304: 1215-7
  12. Pollack KL, Chestnut DH, Wenstrom KD. Anesthetic Management of a parturient with Eisenmengers syndrome. Anesth Analg 1990;70:212-4
  13. Cole PJ, Cross M H, Dresner M  Incremental spinal anaesthesia for elective caesarean in a patient with Eisenmengers syndrome. Br J Anaesth: 2001,86(5): 723-6
  14. Weber,RK, Buda,AJ and Levene,D.L.General anesthesia in Eisenmenger’s syndrome. Can Med Assoc J, 1977;117:1413-1414.
Citation

Fernandes  S, Mali A, Ritika Deo R. Dilatation And Curettage In A Patient Of Eisenmenger’s Syndrome: Anaesthesia Implications. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/dilatation-and-curettage-in-patient-of.html

Pregnancy Complicated By A Large Leiomyoma: A Case Report

Author Information

Shukla H*, Kakade AS**.
(* Second Year Resident, ** Associate Professor. Department of Obstetrics & Gynecology, Bharati Vidyapeeth University Medical College, Bharati Hospital & Research center, Pune, India.)

Abstract

Uterine leiomyomas are seen in 1.6 to 4 % of pregnancies. They affect pregnancy in many ways and subsequently the pregnancy also affects the leiomyoma in different ways. We present such case of large intramural leiomyoma encountered during antenatal period and the challenges faced in the antenatal, intra-partum and postnatal period.

Introduction

Uterine leiomyomas are seen in 1.6 to 4 % of pregnancies.[1] They affect pregnancy in many ways and subsequently the pregnancy also affects the leiomyoma in different ways. With the advances in imaging techniques more and more leiomyomas are diagnosed. They provide a challenge to the treating obstetrician during the pregnancy. A vigilant approach with an outlook of the possible complications can lead to a successful pregnancy outcome.

Case report

A 32 years old Gravida 2, Abortion 1, known to have a prenatal large leiomyoma in the uterus, visited the antenatal outpatient department for routine care at 10 weeks of gestation. She did not have any presenting complaint. She had a spontaneous abortion one year ago for with a dilatation and evacuation was performed. She has excessive bleeding after that and was transfused with one unit of packed cell volume. She was married for two years and was not using any form of contraception.
Her hemoglobin was 10.8 g/dL and her plasma glucose 2 h after oral load of 75 g glucose was 127 mg/dL. Ultrasound revealed a large leiomyoma of 9 X 10.2 X 7 cm in the left lateral wall of the uterus on the lower side and compressing the pregnancy. There was a single live intrauterine pregnancy of around 18 weeks and no detectable fetal anomalies. She had one admission at 14 weeks for threatened abortion which was treated with micronized progesterone and bed rest.
The patient and her relatives were counseled regarding the risk of leiomyoma in pregnancy and effect of pregnancy on the leiomyoma. She was asked to follow up every two weekly with an advice to follow up immediately in case of any bleeding per vaginum or pain in abdomen. She was started on oral micronized progesterone. The pregnancy was monitored by every 4 weekly ultrasounds with Doppler analysis till 37 completed weeks.
At term the leiomyoma was found to be abutting the lower fetal pole pushing the uterus and the fetus to the right side of the abdomen. There was mild fetal growth restriction. She was posted for elective cesarean section. Two units of packed cell were kept reserved. She delivered a baby girl of weight 2.9 kg. Intra-operatively there was a large fundal and isthmic fibroid (figure 1). The third stage was managed by giving inj. oxytocin 10 u intravenously at the delivery of anterior shoulder. She had atonic post-partum hemorrhage which was controlled using injection 15-S-15-methyl PGF2α 250 micrograms intramuscularly. She required one unit of packed cell volume and injection of iron ferrous carboxymaltose post operatively. Her post-operative period was uneventful and she did not require any blood transfusions. She was discharged on 7th day after suture removal.


Figure 1. Arrow pointing towards fibroid after delivery of baby.

Discussion

Leiomyomas are the most frequently encountered gynecological tumors during pregnancy. Increasing women are delaying pregnancy until their late thirties and this is also the likely time for most fibroids to occur. Most of the myomas remain asymptomatic during the tenure of pregnancy. Ultrasonography has improved the detection of these tumors and helped in the evaluation of possible complications.  Leiomyoma can lead to multiple complications like spontaneous abortion, antepartum hemorrhage, preterm delivery, premature rupture of membranes, in coordinate uterine activity. It can lead to malpresentations, dystocia and increased incidence of caesarean deliveries.[2] They are known to cause postpartum hemorrhage and sub-involution of the uterus.
Pregnancy can lead to increase in the size of the fibroid, make it more vascular, soft and cause red degeneration in the fibroid. The risk and type of complication appear to be related to the size, number and location of the myoma.[3] Thus pregnancy with a fibroid is a challenging situation for the treating obstetrician.
The reported case had an early antenatally detected fibroid of size 10 cm. She had threatened abortion and it was a challenge to continue the pregnancy till term. She was labeled as a high risk pregnancy and was counseled regarding the possible complications, their presenting features, need of fetal surveillance, and institutional delivery. She did not report any other untoward event during the antenatal period. She underwent a cesarean section for the leiomyoma obstructing the passage. The anticipated risk of postpartum hemorrhage was tackled with prophylactic uterotonic agent, active management of third stage and packed cell volume transfusion. Many a cases of leiomyomas in pregnancy have a turbulent antenatal period with unfavorable outcome. The reported case had a huge fibroid where all the complications were anticipated and prevented.
Leiomyomas complicating pregnancies are on the rise due to delayed age of conception in women and the increasing use of ultrasonography. leiomyomas can cause complications in all the trimesters of pregnancy and during the intrapartum and postpartum period also. Vigilant approach during pregnancy can lead to successful outcome in these cases.

References
  1. Ardovino M, Ardovino I, Castaldi MA, Monteverde A, Colacurci N, Cobellis L. Laparoscopic myomectomy of a subserous pedunculated fibroid at 14 weeks pregnancy: a case report. Journal of Medical Case Reports 2011;5: 545.
  2. Muram D, Gillieson M, Walters JH. Myomas of the uterus in pregnancy: ultrasonographic follow-up. Am J Obstet Gynecol 1980;138: 6-9.
  3. Phelan JP. Myomas and Pregnancy . Obstet Gynecol Clin North Am 1995;22: 801-805. 
Citation

Shukla H, Kakade AS. Pregnancy Complicated By A Large Leiomyoma: A Case Report. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/pregnancy-complicated-by-large.html

Unruptured Pregnancy In Rudimentary Horn Of Unicornuate Uterus

Author Information

Nikam P*, Pardeshi S**, Jain A***, Mayadeo NM****
(* Second Year Resident, ** Assistant Professor, *** Third Year Resident, **** Professor. Department of Obstetrics and Gynecology, Seth G. S. Medical College and KEM Hospital, Mumbai, India.)

Abstract

Pregnancy in non communicating rudimentary horn of a uterus is an extremely rare form of ectopic gestation. Rudimentary horn is a rare type of mullerian duct anomaly, difficult to diagnose and carries grave consequences for the mother and fetus. It consists of relatively normal appearing uterus on one side with rudimentary horn on other side. We present a case report of  a 22 year old  primigravida with failed attempts at termination of pregnancy in rudimentary horn at 16 weeks of gestational age for which a laparotomy was done and the rudimentary horn was excised. The need for a high index of suspicion and the role of ultrasonography in the accurate diagnosis is highlighted.

Introduction

Unicornuate uterus is a rare mullerian congenital anomaly occurring due to fusion defects. 40-50% cases have associated urinary tract anomaly. The First case of rudimentary horn was described by Manricean in 1669.[1] The incidence of unicornuate uterus in general population is about 5%. Rudimentary  horn pregnancy (RHP) occurs in approximately 1/76000 to 1/150000 pregnancies.[2] Conception in rudimentary horn is rare, the possible explanation for RHP to occur is transperitoneal migration of fertilized ovum or sperm from contralateral tube through abdominal cavity, in case of a noncommunicating horn.[3,5,6] RHP in 80-90% cases presents as rupture of pregnant horn during second or third trimester with life threatening hemoperitoneum, in 10% continues till term and in 2% presents as salvageable fetus. It is associated with high rate of spontaneous abortion, preterm labour (in case of communicating horn), intrauterine growth restriction, intraperitoneal hemorrage and uterine rupture.[4] Because of varied muscular constitution in thickness and distensibility of the wall of the rudimentary horn, pregnancy is accommodated for a variable period of gestation.

Case Report

A 22 year old primigravida, with 4 months of amenorrhea, was referred to our emergency department from some private clinic for failure of induction of abortion with misoprostol. She presented with no complaints of pain in abdomen or bleeding per vaginum despite the administration of misoprostol. A transabdominal ultrasound (USG) scan revealed a 16 weeks intrauterine fetal demise in right horn of uterus. On Examination, her vital parameters were stable with pulse 88/min and blood pressure 120/80 mm Hg. Per abdominally her uterus was just palpable with no tenderness. On per speculum examination cervix and vagina were healthy. On vaginal examination two horns of uterus were felt with the right horn of 14-16 weeks’ size. All investigations for fitness for anesthesia were found to be within normal limits. An exploratory laparotomy was done which revealed pregnancy in an intact right rudimentary horn of size 15X15 cm. There was no haemoperitoneum.The horn appeared non communicating with  the uterine cavity. The rudimentary horn with right tube was excised. Myometrial suturing was done at the site of resection of the horn. Both kidneys were normal in size on palpation. Complete hemostasis was achieved and the abdomen was closed in layers. The cut section of horn showed a macerated fetus of about 14-16 weeks, with cord and placenta. The postoperative course was uneventful and the patient was discharged a week later.


Figure 1. Right rudimentary horn pregnancy, anterior view..


Figure 2. Right rudimentary horn pregnancy, posterior view. The apparently large gap between the ovary and the fallopian tube on the right side is due to the right fallopian tube being stretched over the upper surface of the gravid horn (not seen in this view).

Discussion

Difficulty in diagnosis of RHP during early pregnancy is quite common as there are no definite signs to distinguish this abnormal implantation from normal intrauterine pregnancy. Accurate diagnosis is possible only after laparotomy. Sonographic criteria for early diagnosis of RHP include a pseudopattern of an asymmetrical bicornuate  uterus, absent visual continuity between the cervical canal and lumen of the pregnant horn, and presence of myometrial tissue surrounding the gestational sac.[1] These criteria can help differentiate suspected RHP from cornual pregnancy, intrauterine pregnancy and pregnancy in a bicornuate  uterus. Magnetic resonance imaging may have a major contribution to the diagnostic evaluation when pregnancy in a rudimentary horn is suspected. It offers multiplanar images without the hazards of ionizing radiation, is non-invasive and is able to show both the internal and external uterine structure. Thus early diagnosis of RHP remains challenging.
The management of RHP is laparotomy and surgical removal of the pregnant horn to prevent rupture and recurrences. The fallopian tube on the side of the rudimentary horn must be removed in order to avoid tubal pregnancies. There are instances of modern techniques for management of rudimentary horn pregnancy like laparoscopic excision of rudimentary horns.[7,8] Laparoscopic management is the most accurate diagnostic tool that allows efficient surgical management thereby avoiding laparotomy. Medical management with methotrexate provides another treatment option and it can be a useful adjunct to surgical intervention, provided beta-hCG level is not more than 6000 mIU/ml.[9,10]

Conclusion

Non communicating rudimentary horn pregnancy is a rare entity associated with life threatening consequences. Early diagnosis and early interventions will avoid maternal morbidity and mortality. These patients are advised to be screened for urinary tract anomalies with preoperative intravenous pyelography. A complete USG examination should be performed on the aspect of the pregnancy and the pelvic anatomy. If USG remains inconclusive, the use of magnetic resonance imaging is suggested. Non communicating horn should be suspected whenever difficulty is encountered during termination of pregnancy. It is easy to miss this condition both clinically and radiologically. Above case highlights the need for high index of suspicion to diagnose rudimentary horn pregnancy.

References
  1. Tsafrir A, Rojansky N, Sela HY, Gomori JM, Nadjari M. Rudimentary horn pregnancy: first-trimester prerupture sonographic diagnosis and confirmation by magnetic resonance imaging. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 2005;24(2):219-23.
  2. Okonta PI, Abedi H, Ajuyah C, Omo-Aghoja L. Pregnancy in a noncommunicating rudimentary horn of a unicornuate uterus: a case report. Cases journal. 2009;2:6624.
  3. Jayasinghe Y, Rane A, Stalewski H, Grover S. The presentation and early diagnosis of the rudimentary uterine horn. Obstetrics and gynecology. 2005;105(6):1456-67.
  4. Ahmed S. Elagwany HHE, Tamer M. Abdeldayem. Ruptured ectopic pregnancy in non- communicating right rudimentary horn: A case report. Apollo medicine. 2013.
  5. Scholtz M. A full-time pregnancy in a rudimentary horn of the uterus. The Journal of obstetrics and gynaecology of the British Empire. 1951;58(2):293-6.
  6. Dicker D, Nitke S, Shoenfeld A, Fish B, Meizner I, Ben-Rafael Z. Laparoscopic management of rudimentary horn pregnancy. Human reproduction. 1998;13(9):2643-4.
  7. Sharma D, Usha MG, Gaikwad R, Sudha S. Laparoscopic resection of unruptured rudimentary horn pregnancy. Int J Reprod Contracept Obstet Gynecol. 2013;2(1):95-8.
  8. Samuels TA, Awonuga A. Second-trimester rudimentary uterine horn pregnancy: rupture after labor induction with misoprostol. Obstetrics and gynecology. 2005;106(5 Pt 2):1160-2.
  9. Edelman AB, Jensen JT, Lee DM, Nichols MD. Successful medical abortion of a pregnancy within a noncommunicating rudimentary uterine horn. American journal of obstetrics and gynecology. 2003;189(3):886-7.
Citation

Nikam P, Pardeshi S, Jain A, Mayadeo NM. Unruptured Pregnancy In Rudimentary Horn Of Unicornuate Uterus. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/unruptured-pregnancy-in-rudimentary.html

A Case Of Postpartum Pubic Diastasis

Author Information

Mhaske N*, Madhva Prasad S**, Kharat D***, Fonseca MN****.
(* Second Year Resident, ** Fourth Year Resident, *** Assistant Professor, ****  Professor. Department of Obstetrics and Gynecology, Department of Obstetrics/Gynecology, LTMMC and LTMGH, Mumbai, India)

Abstract

A home-delivered postpartum patient presented on day 5 with pubic diastasis. The patient was successfully managed conservatively.

Introduction

With increasing emphasis on institutional delivery, the focus is shifting from reduction of maternal mortality to prevention of maternal morbidity.[1] In this setting, a case of home delivery with postpartum pubic diastasis is reported.

Case Report

A 23 year old primipara who was unregistered and unimmunized throughout pregnancy, presented to our hospital, on the fifth day following home delivery, with fever, hip pain and purulent discharge per vaginum. The home delivery was assisted by an untrained traditional birth assistant, and the patient reported that she was having labor pains for about 12 hours. She did not report any major difficulty in delivery, such as need for fundal pressure or baby needing to be pulled out. The patient had delivered a healthy female weighing 3500 g, which cried immediately after birth. Following delivery, she noticed difficulty in walking and getting up. Upon worsening of symptoms, she presented to the hospital. There was no urinary incontinence. There was no significant past medical or surgical illnesses. 
The patient was clinically stable with unremarkable systemic examination. Right paraurethral region was ulcerated with slough, and the pubic bones were grossly visible. Digital examination confirmed separation of the pubic symphysis and exposure of supporting connecting tissue (figure 1). There was no perineal tear and speculum examination revealed no cervical tear. Pervaginal examination revealed an involuting uterus. Per rectal examination was unremarkable. Hemoglobin was 6 g/dL, for which blood transfusion was given, and total white blood cell count was raised. Pelvic ultrasonography was normal. Radiography was suggestive of pubic diastasis (figure 2). Magnetic resonance imaging showed diastasis fracture of pubic symphysis with air speck seen in the pubic symphysis which was extending cranially in subcutaneous plane in anterior abdominal wall. Specialist orthopedic and urology opinion advised conservative line of management, which consisted of intravenous antibiotics, urethral catheterization, daily dressing and pelvic hip binder. With reduction in symptoms, and formation of healthy granulation tissue, the patient was discharged after three weeks.  After one year, though there was evidence of pubic diastasis on radiography, the patient wass asymptomatic and had a normal gait. 



Figure 1. (a) showing diastasis of pubic bone; (b) showing separation of urethra from its supporting tissue.


Figure 2. Arrow shows diastasis with fracture of pubic symphysis.

Discussion

Global met need for Emergency Obstetric Care (EmOC) is about 45%,[2] and it is estimated that about 53% of deliveries in India are conducted by Traditional Birth Attendants. [3] Pubic diastasis is a drastic complication of unattended deliveries and is more common than estimated. The risk factors include nulliparity, macrosomia, shoulder dystocia, fundal or suprapubic pressure, cephalopelvic disproportion and twin gestation[4]. The clinical symptoms include pain, restricted mobility, urinary incontinence and there can be chronic problems like prolapse, dyspareunia and psychosexual dysfunction. [4] Management options include conservative treatment, while surgical interventions like external fixation may be necessary, when bone separation is more than 3 cm.[5,6,7]  Osteomyelitis, urinary incontinence, retropubic hematoma and bladder entrapment are possible surgical complications.[6,8,] Physiotherapy is helpful.[10] Despite appropriate management chronic morbidities persist.[4]
This case highlights the catastrophic nature of complications that can occur when labor is not supervised by trained personnel. This case serves to emphasize the need for encouragement of institutional delivery.[3] It is opined that all such occurrences be reported, so as to contribute to the improvement of maternal outcomes and prevention of obstetric morbidity.

References

  1. Pandey A, Das V, Agarwal A, Agrawal, Misra D, Jaiswal N. Evaluation of Obstetric Near Miss and Maternal Deaths in a Tertiary Care Hospital in North India: Shifting Focus from Mortality to Morbidity. J Obs Gyn Ind 2014 Nov Dec ; 64(6):394–399
  2. Holmer H, Oyerinde K, Meara J, Gillies R, Liljestrand J, Hagander L. The global met need for emergency obstetric care: a systematic review. BJOG 2015 Jan ;122(2):183–9.
  3. Satishchandra DM, Naik VA, Wantamutte AS, Mallapur MD, Sangolli HN. Impact of Training of Traditional Birth Attendants on Maternal Health Care: A Community-based Study. J Obst Gyn India. Nov Dec 2013 63(6):383–387
  4. Yoo JJ, Ha Y-C, Lee Y-K, Hong JS, Kang B-J, Koo K-H. Incidence and risk factors of symptomatic peripartum diastasis of pubic symphysis. J Korean Med Sci. 2014 Feb;29(2):281–6.
  5. Aggarwal S, Bali K, Krishnan V, Kumar V, Meena D, Sen RK. Management outcomes in pubic diastasis: our experience with 19 patients. J Orthop Surg Res. 2011 Jan;6:21. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3108341/
  6. Chang JL, Wu V. External fixation of pubic symphysis diastasis from postpartum trauma. Orthopedics. 2008 May ;31(5):493
  7. Dunivan GC, Hickman AM, Connolly A. Severe separation of the pubic symphysis and prompt orthopedic surgical intervention. Obstet Gynecol. 2009 Aug; 114(2 Pt 2):473–5.
  8. Shippey S, Roth J, Gaines R. Pubic symphysis diastasis with urinary incontinence: collaborative surgical management. Int Urogynecol J. 2013 Oct;24(10):1757–9.
  9. Geracci JJ, Morey AF. Bladder entrapment after external fixation of traumatic pubic diastasis: importance of follow-up computed tomography in establishing prompt diagnosis. Mil Med .2000 Jun;165(6):492–3
  10. Shim JH, Oh DW. Case report: Physiotherapy strategies for a woman with symphysis pubis diastasis occurring during labour. Physiotherapy. 2012 Mar; 98(1):89–91.
Citation

Mhaske N, Madhva Prasad S, Kharat D, Fonseca MN. A Case Of Postpartum Pubic Diastasis. JPGO 2015. Volume 2 No. 3. Available from:  http://www.jpgo.org/2015/03/a-case-of-postpartum-pubic-diastasis.html

Pregnancy Termination In Case Of Hemophilia B

Author Information

Shukla H*, Dudhe M**, Channawar S***, Chauhan AR****.
(* Second Year Resident, ** Third Year Resident, *** Assistant Professor, **** Additional Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

Hemophilia B also called Factor IX deficiency or Christmas disease, is a genetic disorder caused by the missing or defective clotting protein, Factor IX. We present a case of Hemophilia B who underwent second trimester medical termination of pregnancy (MTP). The focus of the discussion is management of an obstetric case with bleeding disorder.

Introduction

Hemophilia is an X-linked recessive disorder. Males are predominantly affected because of the presence of single X chromosome. Women are usually asymptomatic carriers because of the presence of the other normal X chromosome. Incidence of hemophilia B is 1 in 30,000 live births. [1]

Case Report

Our patient a 29 year old primigravida with 19 weeks’ gestation, known case of Hemophilia B was admitted for medical termination of pregnancy in view of ultrasonography (USG) suggestive of fetal congenital malformations (bilateral renal agenesis, hydrops fetalis, Ebstein’s anomaly) with anhydramnios. Patient was asymptomatic with no complaint of bleeding during this pregnancy. General examination findings were normal. On abdominal examination, uterus was 18 to 20 weeks’ size. Her baseline routine investigations and coagulation profile were within normal limits.
Patient had been diagnosed with hemophilia B at 13 years of age when she presented with puberty menorrhagia; she received blood transfusion at that time. She also had a tendency of easy bruising. There was a family history of haemophilia in maternal uncle.
After a valid well informed consent, decision of second trimester medical termination of pregnancy was taken in view of substantial risk that if the child were born, it would suffer from such physical or mental abnormalities to be seriously handicapped. Pregnancy was terminated using tablet Misoprostol per vaginally 400 µg repeated four hourly for 5 doses (total dose of 2000 µg).
The case was jointly managed with hematologist; their opinion was sought for measures to control excessive blood loss during MTP. Accordingly, they advised Factor IX 3600 IU intravenously loading dose prior to the initiation of MTP, followed by 1800 IU twice daily during the procedure, which was given. The abortion was complete; there was only mild bleeding immediately post abortion which was managed successfully with oxytocin infusion, with cover of factor IX throughout the process of abortion. She received another dose of 4800 IU of Factor IX after abortion on day 2. As advised by hematologist, recombinant Factor VIIa was kept in reserve for excessive bleed loss in spite of Factor IX therapy; however the patient did not require the same.
The patient was asymptomatic after the procedure with no further bleeding episode. She was discharged on day 3 of procedure, and was advised to follow up.

Discussion

Hemophilia is inherited in an X-linked recessive manner. It is of two types, hemophilia A which occurs due to deficiency of factor VIII, and hemophilia B which occurs due to deficiency of factor IX. Hemophilia B is of three types, mild, moderate and severe, according to the severity and presence of level of factor IX. Mild - level of 6 to 30%, moderate - 1 to 5% and severe - < 1%.[1] Hemophilia presents as spontaneous haemorrhages, hemarthrosis, unexpected bruising and bleeding from trivial trauma, menorrhagia, post- menopausal bleeding, dysmenorrhea, excessive bleeding following minor procedure such as dilatation and curettage, tooth extraction, circumcision, following vaginal delivery and vacuum or forceps deliveries. All bleeding disorders including hemophilia increase the risk of postpartum hemorrhage. Prolonged labour should be avoided; instrumental deliveries like vacuum extraction pose highest risk of intracranial bleeding hence should be avoided.[2,3,4] In cases of excessive bleeding or to prevent excess blood loss, transfusion with Factor IX is recommended.
Prenatal screening of hemophilia can be done by chorionic villous sampling at 11-13 weeks of gestation or by amniocentesis at 15-18 weeks of gestation.[1,5] These patients usually have low hemoglobin, normal partial thromboplastin time and increased activated thromboplastin time. Factor IX assay is done to determine the level of Factor IX in blood and to confirm the diagnosis. The definitive treatment of hemophilia B is to give concentrated factor IX; antifibrinolytics like tranexemic acid can be given as supportive treatment. Genetic tests such as mutation analysis can be done to look for the altered gene responsible for hemophilia, but it is costly and not available in all centres.[1] Preconceptional counseling plays an important role to educate patients about the high risk pregnancy.

Conclusion

Pregnancy in women with inherited bleeding disorders should be managed with multidisciplinary approach with consideration for obstetric and bleeding risk factors. Women with bleeding disorders of moderate to severe types or rare bleeding disorders like hemophilia should be managed in a tertiary care centre.

References
  1. “Carriers and women with hemophilia”. 1st ed. Montreal: World Federation of Hemophilia, 2012; pp 3-6.
  2. Gekas J, Broermann L, Heidenreich W, Z Geburtshilfe. Outcome of pregnancy in patients with haemophilia B -two case reports. Neonatol. 2007Apr; 211(2):90-2.
  3. Yang MY, Ragni MV. Clinical manifestations and management of labor and delivery in women with factor IX deficiency. Haemophilia . 2004 Sep;10(5):483-90.
  4. Huq FY, Kadir RA. Management of pregnancy, labour and delivery in women with inherited bleeding disorders. Haemophilia. 2011 Jul;17 Suppl 1:20-30.
  5. Young JH, Wang JC, Gau JP, Hu HT. Prenatal and molecular diagnosis of hemophilia B. Am J Hematol.1996 Aug;52(4):243-7.
Citation

Shukla H, Dudhe M, Channawar S, Chauhan AR. Pregnancy Termination In Case Of Hemophilia B JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/pregnancy-termination-in-case-of.html


Intraplacental Hematoma: A Rare Cause Of Non-Immune Hydrops

Author Information

Parekh NA*, Agrawal A**, Badade A***, Satoskar P****.
(* Assistant Professor, *** Honorary Ultrasonologist, Nowrosjee Wadia Maternity Hospital; ** Senior Resident, **** Professor Department of Obstetrics and Gynecology, Seth GS Medical College &, Nowrosjee Wadia Maternity Hospital, Mumbai, India.)

Abstract

We present a case of an incidental intraplacental hematoma that was discovered when the placenta was examined post-delivery in order to identify the cause of a non-immune hydrops.

Introduction

Hydrops fetalis is defined as the presence of excessive fluid accumulation in at least two fetal body cavities.  Two major groups can be differentiated: immune and nonimmune hydrops fetalis. Nonimmune hydrops fetalis occurs in 1 in 1500- 4000 pregnancies.With the routine immunization of Rh negative pregnancies, the percentage of immune hydrops has significantly decreased. Presently about 80% of cases of hydrops are due to nonimmune causes.

Case Report

A 32 year old Primigravida with spontaneous conception was referred at 31 weeks of gestation with ultrasonography report suggestive of fetal hydrops [figure 1]. She did not have any complaints in this pregnancy. On examination, she weighed 100 kg with an abdominal girth of 126 cm. She was normotensive. She had an overdistended uterus, abdominal wall edema and oozing pedal edema. Fetal heart rate was 140/min. USG showed generalized fetal edema with no malformations and placental thickness of 8 cm. Amniotic fluid index was 35 cm with estimated fetal weight of 2.029 kg. Color Doppler showed increased middle cerebral artery peak systolic velocity (MCA PSV) of 57 cm/sec, >1.5 MoM. Fetal echocardiography showed a thin rim of pericardial effusion with no structural abnormalities.


Figure 1. Figure 1. USG suggestive of generalized fetal anasarca- skin edema, ascites with polyhydramnios.

The patient’s blood group was AB positive and the indirect Coomb’s test negative. VDRL and, serology for toxoplasmosis, parvovirus B19 and cytomegalovirus were negative. Her glucose tolerance test with 75 g glucose values were 89/150/137 mg/dl. Hepatic viral markers were negative. SGOT (66 IU/ml), SGPT (85 IU/ml), Serum Creatinine (1.2 mg/dl) and serum uric acid (9.3 mg/dl) were elevated. Her hemoglobin was 12.2 g/dL, white blood cell and platelet count,  and serum electrolytes were normal normal. Urine culture did not show any growth. The patient was given steroids for fetal lung maturation and 3 doses of vitamin K (10 mg).
Fetal anemia with non-immune hydrops with maternal mirror syndrome was diagnosed. Fetal transfusion was planned. On cordocentesis fetal hemoglobin was low (10 g/dL). 60 ml of double packed O Negative blood was transfused. On repeat Doppler MCA PSV declined to 40 cm/sec. Cord blood sample testing revealed normal fetal karyotype and was negative for thalassemia.
The patient continued to have severe polyhydramnios with severe discomfort. Hence USG guided amnioreduction was done (1.1 L drained). At 32 weeks, the patient had preterm premature rupture of membranes with footling presentation. Emergency cesarean delivery was performed. At delivery, female baby weighed 2.5 kg and had an Apgar score 2/10 at 1 minute and 5/10 at 5 minutes The baby was immediately intubated and ventilated in neonatal intensive care unit. During cesarean delivery, significant maternal atrial ectopic beats were noted. For hyperkalemia (6.2 mEq/l) neutral human insulin in dextrose and diuretics were given, which corrected it within 2 days. The baby had generalized edema and abdominal distension. Pleural effusion had resolved. She was ventilated and started on diuretics and inotropes. Hemoglobin at birth was 13 g/dL. Gradually the baby was weaned off from ventilator. No exchange transfusion was required.
The placenta weighed 1.2 kg and had a diameter of 21 cm. It was congested with pale areas and thickened membranes. There was no hematoma on gross examination. Microscopy showed mild deciduitis, intervillous fibrin, marked intervillous hemorrhage and intraplacental hematoma with infarcts (figure 2).


Figure 2. Histopathological slide showing intraplacental hematoma.

Healthy mother with healthy baby were discharged on day 15 of delivery; on discharge the baby weighed 1.7 kg.

Discussion

Hydrops fetalis is defined as the presence of excessive fluid accumulation in at least two fetal body cavities; including ascites, pleural effusion, pericardial effusion, and skin edema; often associated with polyhydramnios and placental edema. Ascites is the most common finding on ultrasonography.[1] Normal mechanism for the distribution of interstitial fluid is through lymphatic return. Imbalance in this basic mechanism is postulated as the cause of fetal hydrops. The causes of non-immune hydrops can be grouped in broad categories  like cardiovascular (21.7%), chromosomal (13.4%), hematologic (10.4%), infections (6.7%), placental (5.6%), syndromic, skeletal conditions, lymphatic dysplasia, inborn errors of metabolism, thoracic, urinary tract malformations, extra-thoracic tumors, gastrointestinal causes, Prune belly syndrome, CNS anomalies, infant of diabetic mother, feto-maternal hemorrhage and twin-twin transfusion (donor).[2] In fetal anemia the peak systolic velocity of the middle cerebral artery on color Doppler exceeds 1.5 MoM. Early diagnosis is important because prompt intrauterine transfusion is highly effective and long term outcome is unimpaired in these babies. Parvovirus infection and fetomaternal hemorrhage are self-limited causes of fetal anemia. Intra-placental hematoma is an indicator of feto-maternal hemorrhage (FMH). FMH can begin anytime from the mid-first trimester onward. It is presumed to result from a breach in the integrity of the placental circulation. The cause of FMH is unknown in 82% of cases. In most spontaneous FMH cases, microscopic areas of placental capillary damage may result from third-trimester uterine activity. Hydrops fetalis is associated with severe FMH. Diagnosis of fetomaternal hemorrhage could have been made by Kleihauer-Betke Test in the case presented. Disruptions of the fetomaternal circulation may be spontaneous or related to tumors (choriocarcinoma, chorioangioma), trauma, or partial placental abruption. The perinatal mortality rate of non-immune hydrops is high, treatment options are limited and thus the need for precise diagnosis. Establishing the cause is also helpful in treating the infant at birth. If prenatally diagnosed, mother should be referred to a high-risk center with a good neonatal intensive care unit for further management and multidisciplinary counseling.[3,4] Aggressive management of fetal anemia in non-immune hydrops is associated with an excellent prognosis for baby. Resuscitation of hydrops fetalis poses difficulties. Amniotic fluid and/or fetal cells for future genetic testing, as well as autopsy in case of fetal/ neonatal death, should be offered.

References
  1. Lin SM, Wang CH, Zhu XY, Li SL, Lin SM, Fang Q. [Clinical study on 156 cases with hydropsfetalis].Zhonghua Fu Chan KeZaZhi. Dec 2011; 46(12):905-10.
  2. Bellini C, Hennekam RC. Non-immune hydropsfetalis: a short review of etiology and pathophysiology. Am J Med Genet A. Mar 2012; 158A(3):597-605.
  3. Désilets V, Audibert F. Investigation and management of non-immune fetal hydrops. J ObstetGynaecol Can. Oct 2013; 35(10):923-38.
  4. [Guideline] Leduc L, Farine D, Armson BA, et al. Stillbirth and bereavement: guidelines for stillbirth investigation. J ObstetGynaecol Can. Jun 2006;28(6):540-52.
Citation

Parekh NA, Agrawal A, Badade A, Satoskar P. Intraplacental Hematoma: A Rare Cause Of Non-Immune Hydrops. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/intraplacental-hematoma-rare-cause-of.html

OEIS Complex

Author Information

Patel A*, Gupta AS**.
(* Second Year Resident, ** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

An interesting case of multiple congenital anomaly diagnosed prenatally with very poor compatibility with life is presented here. The neonate was detected to have OEIS [Omphalocele, Extrophy of bladder, imperforate anus and Spinal defect] complex. Prenatal magnetic resonance imaging (MRI) confirmed the abnormality that was suspected on ultrasound scan (USG).

Introduction

A case of OEIS complex was first described and published by Littre in 1709.[1] Carey et al firstly used the term OEIS complex in 1978.[2] OEIS complex is a severe form of extrophy epispadias complex [EEC]. It has an incidence of 1 in 200000 to 1 in 400000 pregnancies [2,3].  

Case Report

A 26 year old primigravida was referred to us at 30 weeks of gestation with an obstetric ultrasound (USG) scan showing a singleton live pregnancy with a composite gestational age of 30 weeks but with absence of the lower abdominal wall, and a cystic mass. This was considered to be extrophy of the bladder. The umbilical cord had a two vessel structure. An MRI of the fetus was done. It showed a midline umblical and an infraumblical anterior abdominal wall defect. Herniation of bowel loops along with peritoneal covering and a partially filled bladder were seen in that sac. Distal bowel loops and the rectum appeared mildly dilated probably due to the imperforate anus. A defect was seen in the lower lumbar region with herniation of cerebrospinal fluid into the sac. Its dimensions were 12 x 7 mm. Left hydronephrosis and hydroureter were also seen. This was suspected to be a part of OEIS [Omphalocele, Extrophy of bladder, imperforate anus and Spinal defect] complex on prenatal magnetic resonance imaging of the fetus.
The patient was counselled by the pediatric surgeons and the poor prognosis and almost nil life expectancy were explained. The patient gave no history of consumption of any drugs in the 1st trimester of pregnancy. Her plasma fasting and postprandial sugars were normal. The woman was allowed to go into spontaneous labor. She delivered a full term malformed live baby weighing 2.7 kg with ambiguous genitalia as shown in the figures 1 and 2. She had cloacal extrophy with caudal dysgenesis and lumbosacral meningomyelocele. Anterior abdominal wall showed ruptured peritoneal covering and bowel loops in the umbilical region. External genital examination showed two distinct openings widely separated as shown in figure 1.



Figure 1. Bladder (cloaca), omphalocele and imperforate anus are seen. The external genitalia are ambiguous with two openings. Pediatric Ryles' tubes are seen inserted through both the orifices. A: On insertion urine drained out so it was considered to be the urethra. B: On insertion it come through cloacal mucosal opening suggestive of some fistulous connection, Baby passed meconium from the perineal opening (entry of tube B). One was considered to be the urethral opening and the other was an anal opening.


Figure 2. Sacral defect is seen.


Figure 3. Anteroposterior radiograph of the neonate.


Figure 4. Lateral radiograph of the neonate.

Neonate had all components of OEIS complex. Pediatric surgeons decided against any surgical repair due to poor results. The parents were counselled and the neonate was discharged on day 3 of birth. On follow up the mother stated that the neonate expired on day 7 after birth and during this period the neonate passed urine and fecal matter through the two orifices.

Discussion

Fetal OEIS defect affects the midline inferior part of the body. It may develop as early as the blastogenesis period. Failure of the fusion of the cephlo-caudal and the lateral embryonic folds may result in the defects. Cloaca is an embryonic structure in which the distal end of the urinary, digestive and the reproductive tract terminates. OEIS complex may result from a single defect in early blastogenesis or due to the defect in mesoderm migration during primitive streak period. This mesoderm later on contributes to the formation of infra umbilical mesenchyme, cloacal septum and vertebral body [4, 5].
Etiology of OEIS complex is unknown but there are many associations like teratogenic effect of diazepam and diphenylhydantoin.[6] Mutation in homeobox gene HLXB9.[6] There is also a higher incidence in monozygous twins which suggests a genetic involvement.[7]
OEIS complex if untreated has almost nil or very short life expectancy and as it has no particular etiological factors prevention is not possible. Anomalous baby with such defect causes strong social stigma so early diagnosis by USG and timely intervention are necessary. These neonates require multidisciplinary approach in specialized centers. The neonate has to undergo multiple reconstructive surgeries that involves neurosurgical closure of the spinal defects, closure of the omphalocele, creation of a colostomy, a new bladder with adequate capacity and continence, pull through operations, and construction of an anal orifice with adequate sphincter mechanisms. Surgeries to establish continence are the most challenging due to weak sphincter muscles and neurological defects.

Conclusion

Complex multiple congenital anomalies are challenges not only to the medical fraternity but also stresses the social, financial fabric of the society and compromises the quality of life of the individual and the family. Early diagnosis may allow the option of termination of pregnancy or early referral to specialized centers. Expertise in such repairs also are not widely available as incidence of the OEIS complex is uncommon.

References
  1. Smith NM, Chambers HM, Furness ME, Haan EA. The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. J Med Genet. 1992;29(10):730-2.
  2. Carey JC, Greenbaum B, Hall BD. The OEIS complex (omphalocele, exstrophy, imperforateanus, spinal defects). Birth Defects Orig Artic Ser. 1978;14(6B):253-63.
  3. Källén K1, Castilla EE, Robert E, Mastroiacovo P, Källén B. OEIS complex--a population study. Am J Med Genet. 2000; 92(1):62-8.
  4. Maizels M. 1998. Normal and anomalous development of the urinary tract. In: Walsh PC, Retnik AB, Vaughan Ed, Wein AJ, editors. Campbell’s urology, 7th edition. Philadelphia:W.B. Saunders Co. pp 1545–600.
  5. Martinez-Frias ML, Bermejo E, Rodriguez-Pinilla E, Frias JL. Exstrophy of the cloaca and exstrophy of the bladder: Two different expressions of a primary developmental field defect.Am J Med Genet. 2001; 99(4):261-9.
  6. Shanske AL, Pande S, Aref K, Vega-Rich C, Brion L, Reznik S, Timor-Tritsch IE Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) in triplet pregnancy after IVF and CVS. Birth Defects Res A Clin Mol Teratol. 2003; 67(6):467-71.
  7. Noack F, Sayk F, Gembruch U. Omphalocele-exstrophy-imperforate anus-spinal defects complex in dizygotic twins. Fetal Diagn Ther. 2005; 20(5):346-8.
  8. Stadtler C, Cremer R, Boemers TM. Spinal dysraphism associated with OEIS complex: aspect of diagnosis and treatment. Cerebrospinal Fluid Res. 2010;7(Suppl 1): S6.
Citation

Patel A, Gupta AS. OEIS Complex. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/oeis-complex.html