Volume 4 Issue 12, December 2017

Editorial
Parulekar SV

Uterine Angioleiomyoma
Parulekar SV

Balloon Catheter Dilatation Of Vaginal Stenosis Post Vaginal Septum Excision
Parulekar SV

Bartter Syndrome – a Rare Cause Of Maternal Polyhydramnios
Sakpal V, Gupta AS.

Obstetric Challenges With Previous Unrepaired Third Degree Perineal Tear
Joshi AV, Pradhan M, Gupta AS.

Rupture Uterus In Unscarred Uterus: A Rare Case Report
Shaikh A,  Mayadeo NM, Warke HS.

Early Diagnosis Of Heterotopic Pregnancy Resulted From Assisted Reproductive Techniques By Three Dimensional Ultrasonography
Mamta D,  Kadambari , Donthi S, Tolani AD.

Sickle Cell Disease In Pregnancy
Parihar AS, Warke HS, Mali K.

Torsion Of A Huge Round Ligament Fibroid With Parasitic Omental Blood Supply
Shah NH, Adsul SD, Paranjpe S.

Adnexal Torsion In Pregnancy
Bano A,  Pardeshi S.

Editorial

Parulekar SV

Bartter syndrome was originally described by Dr. Frederic Bartter et al. in 1962. It is characterized by hypokalemia, hypochloremia, metabolic alkalosis and hyperreninemia with normal blood pressure. I recall a case of recurrent polyhydramnios in three successive pregnancies due to fetal Bartter's syndrome, which I published in 1991. It was believed to be a  salt-losing tubulopathy. Now advances in molecular diagnostics have shown that Bartter syndrome is an autosomal recessive disorder due to mutations in many genes which affect the function of ion channels and transporters responsible for salt reabsorption in the distal renal tubules. Based on genetic changes, six types of Bartter syndrome have been described. Type 1 is antenatal Bartter syndrome due to mutations in SLC12A1 (the sodium-chloride-potassium cotransporter gene). Type 2  is antenatal-neonatal Bartter syndrome due to mutations in the ROMK gene. Type 3  is the classic Bartter syndrome due to mutations of the chloride CLCNKB gene. Type 4 is Bartter syndrome associated with sensorineural deafness due to mutations in BSND. Type 5 is Gitelman syndrome due to mutations in SLC12A3 (the sodium-chloride cotransporter). Type 6 is a severe but transient X-linked disorder showing polyhydramnios, prematurity due to mutations in MAGED2. Bartter's syndrome needs to be differentiated from Gitelman syndrome, congenital adrenal hyperplasia, nephrogenic diabetes insipidus, pseudohypoaldosteronism, cystic fibrosis, chronic accidental intake of loop-acting or thiazide diuretics, pyloric stenosis,  hyperprostaglandin E syndrome, congenital chloride diarrhea, familial hypomagnesemia and Gullner syndrome.

The obstetric problems are polyhydramnios, preterm labor, fetal growth restriction, placenta abruption from uncontrolled escape of amniotic fluid after spontaneous rupture of membranes, growth restriction. Without treatment there can be neonatal dehydration nephrocalcinosis, hypokalemia, hypochloremia, metabolic alkalosis, hyperreninemia, failure to thrive and  developmental delay. Electrolyte imbalance may cause cardiac arrhythmia and sudden death. Bone mineral density decreases significantly over time. Sensorineural deafness may be seen. Slowly progressive chronic renal failure may occur due to interstitial fibrosis.

Antenatal diagnosis is based on findings of polyhydramnios without fetal anatomical defects and uncontrolled maternal diabetes mellitus, fetal growth restriction and elevation of amniotic chloride and and aldosterone levels. Laboratory tests used to diagnose Bartter's syndrome baby after birth include  serum levels of electrolytes including magnesium, renin, aldosterone, and urinary levels of prostaglandin E2, sodium and potassium. A genetic analysis to detect abnormal gene function like loss-of-function, frame-shift, missense and large deletion mutations is a good method of assessment, but is not easily available in most parts of the world and is quite expensive too. Ultrasonography of an older child may show diffuse increase in echogenicity, hyperechoic pyramids and interstitial deposition of calcium.

The condition is not curable (except type 6) and supportive treatment is required lifelong. Conventional treatment of Bartter syndrome includes maintaining fluid and electrolyte balance and indomethacin to prevent the production of prostaglandin 2. Recent advances in treatment include renin-aldosterone-angiotensin system antagonists (including aldosterone antagonists, angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers). Growth hormone is used to treat growth restriction. Calcium and magnesium may have to be given for tetany. Sensorineural deafness can be managed by cochlear implants. Stressful situations like trauma, surgery and infection tend to aggravate electrolyte imbalance and aggressive electrolyte balance is required. Renal transplant can be done for end stage renal disease. It not only improves renal function, but also corrects many of the associated endocrine abnormalities.

We have an interesting case of Bartter syndrome reported in this issue of the journal. I hope the readers benefit from it.

Uterine Angioleiomyoma

Author Information

Parulekar SV.

(Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

Abstract

Uterine angioleiomyoma is a very rare tumor, usually seen in 40-60 years old women. It is made of smooth muscle cells and thick-walled blood vessels. An unusual case of uterine angioleiomyoma is presented.

Introduction

Angioleiomyoma is an extremely rare variant of uterine leiomyoma.[1] Its etiology is unclear. It is estrogen dependent. Chronic venous insuffficiency and minor injury play a role in its genesis. Its clinical presentation is usually like that of a leiomyoma and the diagnosis is made only after histopathological examination. There are only 42 cases of an angioleiomyoma of the uterus in the world literature. We present 43rd case of uerine angioleiomyoma (UAL) in the world literature.

Case Report

A 21 year old woman, married for 2 years, presented with a complaint of heavy menstrual bleeding for 6 months,  severe secondary dysmenorrhea for 1.5 years and primary infertility. She had menarche at the age of 12 years. Her menstrual cycles were regular, every 28-30 days, with blood flow for 3 to 4 days. Her past cycles had been painless. Her past medical and surgical history was not contributory. Her general and systemic examination showed no abnormality. Findings of abdominal examination were normal. Speculum examination showed a normal vagina and cervix. Bimanual pelvic examination showed uterus of 8 weeks’ size, with a posterior wall leiomyoma measuring about 3 cm in diameter. There was no tenderness. There was no other abnormality. Abdominopelvic ultrasonography (USG) showed an intramural leiomyoma in the posterior wall of the uterus measuring 3.5X2.4X1.4 cm. Counseling was done about her treatment options. In view of her severe dysmenorrhea not responding to treatment, she opted for myomectomy rather than management of her infertility and postponing myomectomy until after her childbirth. Her investigations for fitness for anesthesia showed normal results. Myomectomy was done. A leiomyoma measuring about 3 cm in diameter was removed and the uterus was reconstructed. The patient withstood the procedure well and made an uneventful recovery.

Gross examination of the leiomyoma showed it to be of size 3.5X2.5X1.5 cm. Its external surface was whitish and rough. Its cut surface was whitish and whorled. Microscopic examination showed intersecting bundles of smooth muscle cells. Thick walled blood vessels were found amidst and around the smooth muscle cells. There were swirling spindle cells (well-differentiated smooth muscle cells) arising from vascular walls. Some of the vessels were hyalinized. A diagnosis of angioleiomyoma of the uterus was made.

Figure 1. Angioleiomyoma is seen being enucleated (arrows).

Figure 2. Histopathological appearance of the angioleiomyoma. (100X)

Figure 3. Histopathological appearance of the angioleiomyoma. (400X)

Discussion

Angioleiomyoma is a very rare variant of uterine leiomyoma. It accounts for 0.34-0.40% cases of UAL.[2] It is usually seen in 40-60 years old women. Handler et al. Reported 11 cases between 1966 and 2007.[3].  Sharma et al. reported 16 cases in 2014[4]. Garg et al. Reported one more case in 2014.[5] Diwaker et al. Reported another series of 15 cases in 2015.[6] Most of the cases are those subjected to hysterectomy, and very few are of myomectomy. Our case was unusual in that she was only 21 years old. The variant develops as an estrogen dependent tumor, just like simple leiomyoma.[7] Angiogenesis is involved, which may be due to hypoxia, development of shearing forces which activate endothelial cells, growth factor coming from surrounding macrophages.[8,9] The genetic basis of UAL is different from that of a leiomyoma as cytogenetic clonal changes in the karyotype in UAL are not seen in conventional leiomyoma.[10]

Clinical features of UAL include abnormal uterine bleeding (menorrhagia) and dysmenorrhea. The enlarged uterus feels just like one with a conventional leiomyoma does. Thus it is not possible to distinguish between the two clinically. Investigations like USG, computerized tomography and magnetic resonance imaging are not very useful in making this distinction.[5] The diagnosis is most often made only on histopathological examination. Histopathologically UAL is of capillary type, venous type or cavernous type.[11] The capillary type shows a dense structure of narrow vessels and thick bundles of smooth muscles. The venous type shows thick vessels and bundles of smooth muscles. The cavernous type shows wide vessels and less of smooth muscle. Our case was of the venous type. UAL does not show any atypia, pleomorphism, mitosis, or necrosis. When in doubt, immunohistochemical studies are used. Tumors like fibroma, angiolipoma, angiofibroma, and angiomyofibroblastoma are positive for  desmin and vimentin but negative for smooth muscle actin.[12] 

It is sufficient to remove the tumor by total hysterectomy. No recurrence has been reported after that. But if the patient is young, a myomectomy is required. Since the tumor is benign, this should be sufficient. It is desirable to keep a long follow up in such cases.[13]  We are treating our patient for infertility and have counseled her to have annual follow up examinations subsequently for many years.

Acknowledgement

I thank Dr Gwndolyn Fernandes for providing the photographs of histopathology slides.

References

1. Sahu L, Tempe A, Agrawal A. Angioleiomyoma of uterus. J Obstet Gynaecol. 2012;32:713–714.
2. Sikora-Szczęśniak DL. Uterine angioleiomyoma – a rare variant of uterine leiomyoma: review of literature and case reports. Przegla̜d Menopauzalny = Menopause Review. 2016;15(3):165-169. doi:10.5114/pm.2016.63496.
3. Handler M, Rezai F, Fless KG, et al. Uterine angioleiomyoma compicaded by consumptive coagulopathy. Gynecol Onkol Case Rep. 2012;2:89–91. [PMC free article] [PubMed]
4. Sharma C, Sharma M, Chander B, et al. Angioleiomyoma uterus in a adolescent girl: a highly unusual presentation. J Pediatr Adolesc Gynecol. 2014;27:e69–71. [PubMed]
5. Garg G, Mohanty SK. (2014) Uterine Angioleiomyoma: A Rare Variant of Uterine Leiomyoma. Archives of Pathology & Laboratory Medicine 2014;138:1115-1118.
6. Diwaker P, Pradhan D, Garg G. Uterine angioleiomyoma: a rare variant of uterine leiomyoma – a case report and literatur ereview. J Can Res Ther. 2015;11:49. [PubMed]
7. Stewart EA, Nowak RA. Leiomyoma-related bleeding: a classic hypothesis updated for the molecular era. Hum Reprod Update. 1996 Jul-Aug;2(4):295-306.
8. Van Royen N, Piek JJ, Schaper W, et al. Arteriogenesis mechanisms of collateralartery development. J Nucl Cardiol. 2001;8:687–693.
9. Rajotte D, Arap W, Hagedorn M, et al. Molecular heterogeneity of the vascular endothelium revealed by in vivo phage display. J Clin Invest. 1998;102:430–437.
10. Hennig Y, Caselitz J, Stern C, et al. Karyotype evolution in a case of uterine angioleiomyoma. Cancer Genet Cytogenet. 1999;108:79–80.
11. Matsuyama A, Hisaoka M, Hashimoto H. Angioleiomyoma: a clinicopathological and immunohistochemical re with particular reference to the correlation myopericytoma. Hum Pathol. 2007;38:645–651.
12. Culhaci N, Ozkara E, Yksel H, et al. Spontaneously ruptured uterine angioleiomyoma. Pathol Oncol Res. 2006;12:50–51.
13. Hsieh CH, Lui CC, Huang SC, et al. Multiple uterine angioleiomyomas in a woman persenting with severe menorrhagia. Gynecol Oncol. 2003;90:348–352.

Citation

Parulekar SV. Uterine Angioleiomyoma. JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/uterine-angioleiomyoma.html

Balloon Catheter Dilatation Of Vaginal Stenosis Post Vaginal Septum Excision

Innovation

Author Information

Parulekar SV

(Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

Abstract

A transverse vaginal septum causes an obstruction to the outflow of menstrual blood and results in formation of hematocolpos, hematometra and hematosalpinx in that order. Excision of the septum relieves the obstruction. However if the newly created opening closes, the obstruction recurs. If the closure is partial, leaving behind a narrow channel, the collected menstrual blood may escape with difficulty and considerable pain, still producing hematocolpos and hematometra. Such a case with unique presentation and innovative management is presented.

Introduction

 A transverse vaginal septum occurs in about 1:30000 to 1:80000 women. [1.2] It can be at different level in the vagina; about 45% percent being in the upper part, 35-40% in the middle part and the rest in the lower part.[3] If the septum has a hole, it does not cause any symptoms until sexual activity is started, and even that may not be experienced if the septum is in the upper third of the vagina. A complete septum produces cryptomenorrhea, cyclical lower abdominal and pelvic pain in premarital period, and dyspareunia if the septum is in the middle or lower third of the vagina. Complete excision of the vagina is usually adequate management. But if it is partially excised, and efforts are not made to keep the vagina patent postoperatively, the opening may close.[4] A case of this type with unusual features and innovative management is presented.

Case Report

A 16 year old unmarried girl presented with complaints of purulent discharge from an abdominal opening for 6 months and severe lower abdominal and pelvic pain for 3-4 days every month cyclically for 5 months. She had been diagnosed to have cryptomenorrhea due to a transverse vaginal septum in the past and had undergone surgical excision of the same at a healthcare facility elsewhere 7 months ago. That operation had been followed by an exploratory laparotomy for unknown indication. Operative findings were a ruptured hemorrhagic cyst in the left ovary, intense adhesions between the ovary and small intestine and 1 L of purulent fluid in the peritoneal cavity. Abdominal adhesions were separated at that time. Then the abdomen was closed after placing an intraperitoneal drain. The drain was removed on day 3 after the operation. She started discharging purulent fluid from drain site. A magnetic resonance imaging scan was performed on her, which showed a bicornuate uterus with stenosis of the distal cervical or vaginal canal, hematocolpos, and a fistulous tract opening on the anterior abdominal wall. She had menses every month subsequently. The flow was preceded by intense lower abdominal and pelvic pain for 3-4 days.

When she presented at our center, her general condition was good and vital parameters were in the normal range. Her general and systemic examination revealed no abnormality. Abdominal examination showed an infraumbilical midline vertical scar. The site of colostomy had healed except at one place where 5 mm x 5 mm part was open, discharging mucoid material. There was some fibrosis around that opening. The remaining abdomen was normal. Local examination showed normal external genitals. The vaginal depth was 5 mm. A 1 cm diameter opening was seen at the center of the pouch of vagina. Rectal examination showed scanty tissue between the anterior rectal wall and the posterior wall of the vagina. A mildly tender cystic lump measuring about 6-8 cm in diameter was felt high in the pelvic cavity. An MRI scan of the abdomen and pelvis was done (figure 1). It showed a bicornuate uterus, single vagina distended with blood, and a small hematosalpinx on each side. A sinogram was obtained, which showed a sinus tract leading from the anterior abdominal wall into the transverse colon (figure 2). A diagnosis of coloabdominal fistula was made. It was confirmed by performing a CT sinogram (figure 3). Gastrointestinal surgeon advised surgical treatment of the fistula after 6 months, giving the tissues to get consolidated.

Figure 1. MRI scan of the abdomen and pelvis. It shows right horn of the uterus (RH), left horn of the uterus (LH), left hematosalpinx (LHS) and urinary bladder (UB).

Figure 2. Sinogram showing cannula for injection (C), sinus tract (ST) and colon (CO).

Figure 3. CT sinogram showing  sinus tract (ST) and colon (CO).

The patient had menses while in the ward. The flow was a drop at a time, accompanied by intense pain n the lower abdomen. Rectal examination showed the pelvic lump to have enlarged and become more tender. A fibrous tract was felt between the center of the top of the vaginal pouch and the pelvic lump. The length of the tract was about 4 cm. A diagnosis of extreme stenosis of the vagina was made. The patient and her parents were counseled about two treatment options – excision of the closed vaginal septum and skin grafting over the new vagina or dilatation of the stenotic vagina and a vaginoplasty at a later date when the patient was to get married. They opted for vaginal dilatation. The patients investigations for fitness for anesthesia showed normal findings. Dilatation of the stenosed vagina was initiated under general anesthesia. However the opening was too narrow to allow passage of even No. 3/6 Hegar’s dilator. The direction of the tract could also not be ascertained. So a finger was placed in the rectum and a thin probe was passed through the opening along the tract, remaining parallel to the anterior rectal wall so as to avoid perforating it. When the probe was passed successfully into the upper vagina distended with blood, a series of Hegar’s dilators were passed similarly. A dilator of size 5/8 could be passed, but not one larger than that. A decision was made to pass a No. 12 Foley’s catheter through the vagina. It could be passed easily. The balloon was inflated with 10 ml of normal saline. The catheter was replaced by another catheter of size 16 F a week later, and a third one of size 22 another week later. The collected menstrual blood drained over 4-5 days after the passage of the first catheter. After 3 weeks, the patient presented with foul discharge vaginally. Ascending infection was diagnosed and the catheter was removed. Vaginal irrigation was done twice a day with povidone iodine (5%) solution for one week. The pus discharging from the vaginal opening grew acinatobacter sensitive to ofloxacin. She was given ofloxacin for 1 week and the infection was controlled. The vaginal opening was wide enough to allow menstrual flow but did not permit passage of a little finger. The patient presented after a month with mentruation which was painless and the flow was normal. She was keen to go back to her native place so as to be able to resume school. So she was sent home with instructions to follow up if the menstrual flow was obstructed again, foul discharge occurred vaginally. She reported successful menstruation for 3 months subsequently.

Figure 4. A probe is passed into the lower end of the stenotic vagina, with an index finger in the rectum to guide it.

Figure 5. The stenotic vagina is being dilated with Hegar’s dilator.

Figure 6. A Foley’s catheter is being passed into the dilated vagina.

Discussion

Excision of a transverse vaginal septum is not an operation performed routinely by a gynecologist. It can result in an injury to the urinary bladder or the rectum if the operation is done without exercising due caution.[5] Fear of injuring the bladder and/or the rectum makes the gynecologist operate cautiously, so that the end result is often leaving a large part of the septum behind. This can result in closure of the opening partially or completely. If the septum is thick, its excision leaves behing a large raw area, which needs to be covered by partial thickness skin graft or amniotic membrane graft. If that is not done, the newly created opening can close again. A lot of fibrosis often develops around it, making future operations difficult. Reoperating in such cases can be difficult and more likely to cause the complications described above.[6,7] In this case the patient already had an enterocutaneous fistula as a complication of the abdominal operation. She had a partial closure of vagina in the area from which a vaginal septum had been excised. The length of the tract indicated that a septum 4 cm in length had been excised. It closed probably because no graft had been placed over it. There was a significant degree of fibrosis around it, so that dilatation was difficult. Placing self retaining catheters of progressively increasing sizes was an innovative technique which was noninvasive, painless and effective. It did not create a vaginal canal of normal dimensions, but it did relieve the obstruction to flow. Since the girl was just 16 and unlikely to get married for a number of years, a vaginoplasty at that stage would not have been useful anyway. The vagina would have closed as there would not be any means of keeping it open, other than wearing a mould at night. Doing that for years was not very practical. Ascending sepsis was an unavoidable complication of the procedure. But the resultant morbidity was much less than what would have perhaps occurred after a major operation for reexcision of the septum and placement of a graft.

Conclusion

Continuous placement of self retaining urinary catheters in stenotic vagina, increasing the size of the catheter every week is a useful and effective method of dilating vaginal stenosis after a failed excision of transverse vaginal septum.

Acknowledgement

I thank Dr Durga Valvi for taking intraoperative pictures.

References

1. Rock JA, Azziz R (1987) Genital anomalies in childhood. Clin Obstet Gynecol 30: 682-696.
2. Nahum GG. Uterine anomalies. How common are they, and what is their distribution among subtypes? J Reprod Med 1998;43: 877-887.
3. Acién P. Incidence of Müllerian defects in fertile and infertile women. Hum Reprod 1997;12: 1372-1376.
4. Rock JA, Reeves LA, Retto H, Baramki TA, Zacur HA, Jones HW., Jr. Success following vaginal creation for Mullerian agenesis. Fertil Steril. 1983;39:809–813.
5. Suidan FG, Azoury RS The transverse vaginal septum: a clinicopathologic evaluation. Obstet Gynecol 1979;54:278-283.
6. Robson S, Oliver GD: Management of vaginal agenesis: Review of 10 years’ practice at a tertiary referral centre. Aust N Z Obstet Gynaecol 2000;40:430-433.
7. Roberts CP, Haber MJ, Rock JA. Vaginal creation for mullerian agenesis. Am J Obstet Gynecol. 2001;185:1349–52; discussion 1352-3.

Citation

Parulekar SV. Balloon Catheter Dilatation Of Vaginal Stenosis Post Vaginal Septum Excision. JPGO 2017. Volume 4 No. 12. Available from: http://www.jpgo.org/2017/12/balloon-catheter-dilatation-of-vaginal.html

Bartter Syndrome – a Rare Cause Of Maternal Polyhydramnios

Author Information

Sakpal V,  Gupta AS**

(*Senior resident, **Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Unexplained polyhydramnios posses a diagnostic challenge to the treating obstetrician. We present a rare case of Bartter syndrome [BS] causing polyhydramnios.

Introduction

Polyhydramnios is pathological increase in the amount of amniotic fluid. There are various maternal and fetal causes of polyhydramnios. Bartter syndrome is a renal salt wasting disorder causing fetal polyuria that can result in polyhydramnios.

Case Report

A 28 year old woman married since 8 years G2P1L1A0  with  spontaneous conception and a non consanguineous marriage, registered with a private practitioner and was on regular follow up. Her previous pregnancy was uneventful. She delivered vaginally a healthy male child 6 years back. Her hemoglobin was 12.3 gm% , serology for HIV,  HbsAg  and  VDRL were negative . Fasting blood sugar was 79 mg% and blood group was O positive. First trimester ultrasound (USG) at 9 weeks of gestation and quadruple marker test at 16 weeks were normal. However, ultrasonography at 20 weeks showed elevated liquor with amniotic fluid index ( AFI) of 21 cm. There were no structural anomalies. USG scan done two weeks after this showed a rise in liquor volume with an AFI of 31 cm. Hence, she was referred to a higher center for further management. 

In our hospital, she presented at 25 weeks of gestation. Examination showed over distended abdomen with full flanks, and uterus of 30 weeks size and presence of polyhydramnios. Fetal heart rates were regular and there was no appreciable uterine activity. Cervical internal os was closed . Abdominal girth was 114 cm and fundal height was 50 cm. Since she was asymptomatic and respiratory rate was within normal limits, she was considered for outpatient management and was advised serum TSH, OGTT and to follow up with reports, or whenever she becomes symptomatic. However, she presented to the emergency department 2 weeks later with complaints  of  abdominal pain. There was no leaking or bleeding per vaginum. Examination parameters were within normal limits and fundal height was 52 cm and abdominal girth was 116 cm. Uterus was relaxed. On per speculum examination there was no leak. On per vaginal  examination os was closed and uneffaced. OGTT with 75gm of glucose showed values of 78 mg%, 142 mg%, 124 mg% and 103 mg% as fasting, end of first hour, second hour and third hour respectively. Serum TSH was 2.6 uIU /ml and HbA1C was 4.5%.  Fetal echocardiogram was normal.  In view of no obvious detectable cause, she was managed as a case of idiopathic polyhydramnios. Tablet indomethacin was started with an initial dose of 50 mg followed by 25 mg four times. Her pain subsided. Ultrasound done showed  AFI of 70 cm .

Serial fetal 2 D echo was done weekly to monitor ductus arteriosus. Indomethacin was to be continued till there were signs of narrowing of ductus arteriosus. Fortunately there was no narrowing of ductus arteriosus.  Injection  Dexamethasone  6 mg 12 hourly,  total 4 doses were  given, hematinics and calcium were continued.  Blood sugar was repeated which was also normal. Daily fundal height and abdominal girth was monitored which was almost constant at 52 cm and 115 cm respectively. During her stay in hospital she never developed respiratory distress, hence therapeutic amniocentesis was not considered for this patient. At 33 weeks and 6 days, due to technical reasons fetal 2D echo could not be done further and hence decision to stop oral indomethacin was taken.  Just one day later at 34 weeks of gestation she developed preterm premature rupture of membranes. Urgent ultrasound showed breech presentation, hence she underwent an emergency cesarean section. Intraoperatively almost 7 liters of amniotic fluid was  drained Four suction bottles of 2 liter capacity each almost filled up (figure 1).  A female neonate of 1.9 kg was delivered with APGAR score of  9/10. Neonate was shifted to NICU in view of low birth weight.  There was no abruption or postpartum hemorrhage. 

In its course in NICU as neonate was losing her weight and had salt wasting polyuria an electrolyte report was sent on day one of life. Serum sodium and potassium were 126 meq/l 2.3 meq/l respectively. A sample sent few hours later for reconfirmation showed values of serum sodium, potassium and chloride as 122 meq/l,  2.8 meq/l and 95 meq/l respectively. Her serum sodium level were in range of 118 mEQ/l. Sodium correction was done. On clinical basis and investigations baby was diagnosed to have Bartter’s syndrome. Neonate received electrolyte and fluid correction. She was started on indomethacin at the dose of 1mg/kg. Neonate was discharged on indomethacin, syrup potassium chloride and follow up. Follow up would be for weight gain and electrolyte balance. Genetic testing for confirmation was offered, however due to financial constraints it was not carried out. 

Figure 1. Suction Bottles completely filled with drained amniotic fluid 

Discussion 

Polyhydramnios, a condition characterized by excess of amniotic fluid is associated with significant fetal morbidity and mortality.[1,2]  It requires a thorough antenatal workup and meticulous monitoring.  Even the rare causes need to be anticipated. BS is an autosomal recessive disorder where the chloride transport is defective across the thick ascending loop of Henle and or distal convoluted tube.[3,4]  It results in loss of Na+, K+, Cl- and Ca++ ions and excess water loss. Clinical features include fetal polyuria causing polyhydramnios, preterm delivery, postnatal polyuria, failure to thrive. Biochemical parameters show hypokalemia, metabolic alkalosis and hyperaldosteronism.[3,4] 

Antenataly this condition can be diagnosed by biochemical analysis of amniotic fluid which shows high chloride levels.[5]  Postnatally electrolyte and fluid correction are the mainstay of  treatment. Recommended treatment is Indomethacin in the dose of 1-5 mg/kg/day.[6,7,8]  It inhibits prostaglandin synthatase, decreases renal salt loss and hence reduce urine output. If diagnosis is made antenatally then indomethacin is the drug of choice. By decreasing fetal urine output it controls polyhydramnios. However it can cause closure of ductus arteriosus, hence its use mandates frequent monitoring of fetal ductus arteriosus for signs of narrowing. In our case we had started indometthacin to control polyhydramnios. Just one day after indomethacin was stopped  patient had preterm premature rupture of membranes, thus proving the tocolytic effect of indomethacin . 

Long term complications involve nephrocalinosis, growth failure, renal insufficiency. Parents need to be educated  regarding treatment and need of follow up for best outcome. Being autosomal recessive there is 25% risk of recurring in the subsequent pregnancy and prenatal diagnostic tests should be offered to the parents.

Conclusion

Unexplained  polyhydramnios is a real challenge for the treating obstetrician. A high index of suspicion  of  this entity is warranted  in case of  increasing polyhydramnios  without apparent fetal abnormalities or maternal  factors. Antenatal diagnosis of this syndrome is possible by subjecting the amniotic fluid to biochemical analysis. Its treatment with indomethacin and therapeutic amniocentesis if required can prolong pregnancy and improve the fetal outcome. It is recommended that parents should be offered prenatal diagnosis in subsequent pregnancies.

References

1. Golan A, Wolman I, Sagi J, Yovel I, David MP. Persistence of polyhydramnios during pregnancy--its significance and correlation with maternal and fetal complications. Gynecol Obstet Invest. 1994;37(1):18-20.
2. Many A, Hill LM, Lazebnik N, Martin JG. The association between polyhydramnios and preterm delivery. Obstet Gynecol. 1995;86(3):389-91.
3. Bartter FC, Pronove P, Gill Jr. JR, MacCArdle RC.  Hyperplasia of the juxtaglomerular complex with hyperaldosteronism and hypokalemic alkalosis a new syndrome. The American Journal of Medicine. 1962;33(6):811-828.
4. Dell KM, Avner ED. Bartter-Gitelman syndromes and other inherited tubular transport abnormalities. In: Kleigman RM, Behrman RE, Jenson HB, Stanton BF, editors. Nelson Textbook of Pediatrics. 18th edition. Philadelphia, Pa, USA: Saunders; 2007. pp. 2201–2202.
5. Bhat YR, Vinayaka G, Vani R, Prashanth KA, Sreelakshmi K. Antenatal Bartter syndrome: a rare cause of unexplained severe polyhydramnios. Ann Trop Paediatr. 2011;31(2):153-7.
6. Rodriguez-Soriano J. Tubular disorders of electrolyte regulation. In: Avner E,        Harmon W, Niaudet P, editors. Pediatric Nephrology. 5th edition. Philadelphia: Lippincott Williams and Wilkins; 2004. pp. 729–756.
7. Konard M, Leonhardt A, Hensen W, Seyberth H, Kockerling A. Prenatal and postnatal management of Hyperprostaglandin E syndrome after genetic diagnosis from amniocytes. Pediatrics.1999;103(3):678-683.
8. Amirlak I, Dawson KP. Bartter syndrome: an overview. QJM. 2000; 93(4):207-215.

Citation

Sakpal V, Gupta AS. Bartter Syndrome – a Rare Cause Of Maternal Polyhydramnios.  JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/bartter-syndrome-rare-cause-of-maternal.html

Obstetric Challenges With Previous Unrepaired Third Degree Perineal Tear

Author Information

Joshi AV*,  Pradhan M**, Gupta AS***

(* Specialty Medical Officer, ** First Year resident, **Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Obstetric anal sphincter injuries (OASIs) are a severe form of perineal lacerations commonly encountered with difficult vaginal deliveries. They can severely affect the patient’s daily routine and quality of life. This case report highlights the management dilemma that an obstetrician encounters in a patient with a deficient perineal body, sustained during previous vaginal birth.

Introduction

The incidence of obstetric anal sphincter injuries following a vaginal delivery is presently around 3-4%.[1] These can predispose the patients to long term morbidities like dysparenuia, fecal or urinary incontinence, pelvic organ prolapse etc.  A term patient managed elsewhere who presented in active labour is described here. 

Case Report

A 22 year old G2P1L0A0 at 40 weeks of gestation was referred in view of meconium stained amniotic fluid.  Referral note also mentioned high risk factor of “deficient perineal body”. She was antenatally registered at a private hospital in this pregnancy at 5th month of gestation. She had two antenatal visits, antenatal profile was normal. She does not give history of any bowel or bladder complaints in the antenatal period.

The first delivery outcome was a neonatal death that occurred 2 years back. It was a full term vaginal delivery at a primary health center with a birth weight of 4.5 kg. Baby did not cry at birth and died in transit to a higher center for want of NICU care. She complained of symptoms like fecal urgency, inability to control flatus and fecal incontinence in the immediate postpartum period. However, since the symptomatology significantly improved, she did not follow up for these complaints. Upon questioning, she reported that she gradually achieved fecal continence by 6 months post-delivery without any active interventions. 

On examination, vital parameters were stable. Uterus was full-term, vertex (2/5th palpable) with an activity of 3/10/30. Fetal heart sounds were 120 beats per minute with persistent variable decelerations till 80 beats per minute. On per vaginal examination cervix was fully dilated, fully effaced, station at zero, occiput at 3’0 clock with presence of caput and liquor was meconium stained. On local examination, perineal body was absent. There was a thin band of fibrous tissue formed by the posterior vaginal wall and torn external anal sphincter suggestive of an old third-degree tear. A decision for emergency lower segment cesarean section was taken in view of severe fetal distress in second stage of labor. Instrumental delivery was not done as station was at zero. She delivered a male child of 3.6 kg.

She required a cesarean section in this pregnancy for a fetal indication. Surprisingly, she remained asymptomatic following this childbirth. We had planned for a repair of the deficient perineal body after her puerperium, however she was reluctant since she was asymptomatic.

Figure 1: Image showing an old third degree perineal tear with arrow pointing towards area of fibrosis

Discussion

The perineal body is a fibromuscular structure that provides strength to the pelvic floor. It is the junction formed by the following muscles: external anal sphincter, bulbospongiosus muscle, superficial and deep transverse perineal muscles, anterior fibers of levator ani and the external urinary sphincter. Perineal injuries are classified into four degrees of tear. Injuries to the perineal body are generally categorized into grade three which are further subdivided depending upon the degree of involvement of the external or internal anal sphincter. Obstetric anal sphincter injuries primarily refer to the third and fourth degree perineal lacerations which involve the anal sphincter and rectal mucosa respectively. 

The risk factors for such injuries include young primipara, induction of labour, advanced gestational age, obesity, operative vaginal delivery and infant birth weight >3500 grams. The clinical presentation of the patient depends upon the degree of neuromuscular and connective tissue damage. These include urinary and/ or anal incontinence, anal incontinence being more common. Anal incontinence can further be categorized as inability to control flatus, fecal urgency or fecal incontinence. 

The long term morbidities associated with such injuries include chronic pelvic pain, dyspareunia, pelvic organ prolapse, rectovaginal fistulas and recurrence in subsequent pregnancies.

A study by Brincat et al showed an increase in fecal incontinence at 6 weeks postpartum which gradually subsided at 6 months and then again showed an increase at 1 year post-delivery.[2] This warrants a need for close follow up in such patients despite relief of symptoms. The improvement noted at 6 months post delivery is usually a result of restoration of the neuromuscular as well as connective tissue elements of the pelvic floor. The resurfacing of symptoms at 1 year was attributed to lifestyle practices, bowel habits, diet, medications. Women with a 3rd or 4th degree perineal tear in the first vaginal delivery are at 3-4 times increased risk of experiencing a recurrent laceration in the subsequent pregnancy.[3] 

This case is being reported to highlight the interesting facts and dilemmas that crossed our minds while managing this patient. When she presented to us in the emergency room, we intended to deliver her vaginally. When she developed severe intra partum fetala distress instrumental delivery was not done as the vertex remained at station zero even when she was reassessed in the operation theater immediately prior to the cesarean section.

The first dilemma: Had the patient delivered vaginally, would an episiotomy be required? Technically, considering the absence of the perineal body, there would have been no resistance offered from the pelvic floor. Hence there is no role of a prophylactic episiotomy in this case. However, the requirement of episiotomy should be individualized. Evidence in this matter is lacking. 

The next challenge would have been in the event of a laceration being sustained while delivering her vaginally. Do such patients need an immediate repair of the tear? Or is surgical repair after 6 weeks preferred? Again, literature in this subject is lacking. Every case must be individualized and managed as per the treating doctor’s clinical acumen. Immediate repair of such injuries carry a risk of fecal contamination and breakdown of the wound as bowel preparation is not done in spontaneous labors. Repair immediate post delivery would entail application of surgical principles for a complete perineal repair in a gynecological patient. Pre existing fibrous excision would be needed prior to the layered repair. As the bowel preparation is not done in spontaneous labors fecal contamination will occur. When such patients present in latent labor, bowel preparation may be possible to enable immediate repair post delivery. The mode of delivery in these patients in subsequent pregnancies remains a challenge due to lack of robust evidence. Additional factors like history of failure of previous repair, persistent anal incontinence and larger estimated birth weight of the fetus may go in favor of an elective cesarean section.[4] However, the risk of recurrence of a perineal tear with instrumental delivery or greater birth weight in subsequent pregnancies for such patients was lesser, possibly due to the stretching of perineum in previous pregnancy. In well-equipped centers an anal manometry can further guide to decide the mode of delivery. Asymptomatic patients can undergo a vaginal delivery in presence of a skilled obstetrician.

Conclusion

The primary approach to a patient with deficient perineal body depends on the nature and severity of her symptoms and the time of presentation. The mode of delivery in these patients remains controversial. A decision for the same, must be made after a thorough clinical examination and if possible an anal manometry. Patients must be made aware of the associated complications with any mode of delivery and an informed consent must be obtained for the same. Timely detection and repair of perineal injuries is vital to ensure a good quality of life.

References

1. Friedman AM, Ananth CV, Prendergast E, D'Alton ME, Wright JD. Evaluation of third-degree and fourth-degree laceration rates as quality indicators. Obstet Gynecol. 2015 Apr;125(4):927-37
2. Brincat C, Lewicky-Gaupp C, Patel D, Sampselle C, Miller J, Delancey JOL, et al. Brincat C, Lewicky-Gaupp C, Patel D, Sampselle C, Miller J, Delancey JOL, et al. Fecal incontinence in pregnancy and postpartum. Int J Gynaecol Obstet. 2009;106(3):236–8.
3. Lowder JL, Burrows LJ, Krohn MA, Weber AM. Risk factors for primary and subsequent anal sphincter lacerations: a comparison of cohorts by parity and prior mode of delivery. Am J Obstet Gynecol 2007;196(4): 344.e1-5.
4. Edozien LC, Gurol-Urganci I, Cromwell DA, Adams EJ, Richmond DH, Mahmood TA, et al. Impact of third- and fourth-degree perineal tears at first birth on subsequent pregnancy outcomes: A cohort study. BJOG. 2014;121(13):1695–703.

Citation

Joshi AV, Pradhan M, Gupta AS. Obstetric Challenges With Previous Unrepaired Third Degree Perineal Tear.  JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/obstetric-challenges-with-previous.html

Rupture Uterus In Unscarred Uterus: A Rare Case Report

Author Information

Shaikh A*, Mayadeo NM**, Warke HS***.

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

Abstract

Rupture of a pregnant uterus is a rare and catastrophic obstetrical complication. Its incidence is higher in patients with previous scar on uterus but it can also be present in patients with unscarred uterus. It leads to higher maternal and perinatal morbidity and mortality. Here we present a case of a 27 year old woman G3P1L1MTP1 with 19 weeks of pregnancy with previous normal delivery with full thickness uterine rupture at the fundus with en sac fetus lying in the abdominal cavity. The placenta was attached to the serosa of the uterine fundus. Around 1.2 liters of hemoperitoneum was drained and 600 grams of clots were evacuated. Subtotal hysterectomy was done with successful outcome. Histopathology revealed placenta percreta and her post-operative recovery was uneventful.

Introduction

Rupture uterus is a life threatening obstetric complication which must be treated promptly. It is an uncommon occurrence. When compared with general population; patients with previous scar have a higher incidence of uterine rupture especially those with a vertical, T or J shaped uterine scars. Patients without prior cesarean scars but with previous surgical procedures like removal of fibroids, curettage, previous abortions; excessive use of uterotonics, prolonged labor, with a big size baby can also lead to rupture uterus.[1]  Spontaneous rupture of an unscarred uterus` is rare. Rupture uterus may involve adjacent organs mainly the urinary bladder which is an acute obstetric emergency.

Case Report

A 27 year old woman G3P1L1MTP1 with 19 weeks of pregnancy with previous normal delivery was referred from a private hospital with chief complaints of pain in abdomen since 1 day, breathlessness (NYHA Grade 2) and vomiting since two hours with septic shock with acidosis with suspected concealed abruption with severe anemia. She had a history of a full term normal delivery of a female child 3 years back and also a medical termination of pregnancy at 2 and a half months with uterine curettage done 1 and a half year back. She had a cervical cerclage done 1 month back. No medical records of the same were available. On admission her general condition was moderate to poor, peripheral pulses were feeble, tachycardia and tachypnea were present. Blood pressure was 100/60 mm of Hg. Her per abdomen findings were normal and uterus was 20 weeks in size corresponding to gestation age. On per vaginal examination os was closed and cervical knot was felt. Clear urine around 200 cc was in the urine bag. On admission her hemoglobin was 3.9 gm%, WBC count was 24000/ cmm, platelet count of 1.2 lakh/ mm3, INR was 1.2, aPTT test was 26 second with a control of 32 seconds. Her ultrasound (USG) was suggestive of liver parenchymal disease, IUFD and moderate hemoperitonuem. Hemoperitoneum was confirmed on USG guided abdominal tapping. Knots of cervical cerclage were cut and removed; no active bleeding occurred. CT scan of the abdomen and pelvis on surgeons recommendations was done to know the definitive cause of haemoperitoneum. It was suggestive of IUFD and suspected blush from the left uterine artery and hemoperitoneum. Consent for Exploratory laparotomy and obstetric hysterectomy (if needed) was taken from the patient after adequate information and counseling. Intra-operative findings showed a full thickness uterine rupture at the fundus. Fetus en sac was lying in the abdominal cavity but was attached to the placenta that was attached to the serosa of the uterine fundus. Placenta seemed to be invading the serosa of the fundus of the uterus suggestive of rupture uterus with placenta percreta. Subtotal obstetric hysterectomy was done as uterine repair was not possible. Around 1.2 liters of hemoperitoneum was drained and 600 grams of clots were evacuated. She was transfused with a total of 5 units of whole blood and 9 units of fresh frozen plasma. DIC profile was normal post correction. She was monitored in an ICU setup where she was successfully extubated. Her rest of the postoperative hospital stay was uneventful. Histopathology report showed chorionic villi invading the myometrium through the serosa consistent with diagnosis of placenta percreta. She was discharged on day 10 of surgery. 

Figure 1. Operative findings - the fundus of the uterus shows the site of the rupture.

Figure 2. Specimen of subtotal hysterectomy - fundal rupture of the uterus and placenta extruding from the uterine rent are seen.

Discussion

Rupture uterus is a rare entity. It has a significant effect on reproductive function of women. The clinical presentation of rupture uterus include abdominal pain, vaginal bleeding, and maternal shock. Uterine rupture in a term patient can lead to non-reassuring fetal heart rate, loss of presenting part on pelvic examination, loss of uterine contraction. The risk factors include multiparity, uterotonic drugs, placenta percreta , cephalopelvic disproportions, malposition and malpresentations. Scarred uterus following operations such as cesarean section, myomectomy have a higher incidence of rupture uterus.[2] Other procedures such as curettage, instrumental delivery, perforation of uterus during abortions also have a higher incidence of rupture uterus with placenta percreta. Placenta percreta is the rarest form of placenta accreta. Placenta percreta is rare and life threatening complication of pregnancy hazardous, for both the mother and the fetus. Placenta percreta is a state in which the chorionic villi completely penetrates the uterus and adjacent structures such as urinary bladder and bowel.  Placenta percreta leading to spontaneous rupture of uterus in early pregnancy is rare.[3] Its incidence varies from 0.3% to 1.7% in women with previous scar and 0.03% to 0.08% in women with unscarred uterus.[4]

Diagnosis of placenta percreta at earlier weeks of gestation is difficult as a routine ultrasound at 18 weeks of gestation does not include detailed examination of localization and implantation of placenta. USG is definitive. However MRI can also be done. First trimester higher values of PAPP-A and free beta hCG MoM values can be related to placenta accreta in patients with placenta previa.[5] In patients with severe hemorrhage and shock requiring hysterectomy, operative time and exposure to anesthesia are vital factors, and a quick subtotal hysterectomy should be resorted to.

Conclusion

Rupture uterus is a life threatening obstetric emergency. These are non specific which makes diagnosis even more difficult. Delay in management can lead to significant morbidity and mortality.[6] The high maternal morbidity, mortality and fetal mortality can be prevented through integrated efforts. Early antenatal care, early diagnosis of high risk cases, timely referral to a higher center, increased awareness of patients on birth spacing can prevent unwanted outcomes.

References

1. Landon MB. Vaginal Birth after Cesarean Section. In John QT, Spong CY, Lockwood CJ. Queenan’s Management of High-risk pregnancy: An Evidence-based Approach. 6th ed. West Sussex: Wiley- Blackwell, Chichester.2012; pg. 414
2. Berghella V, Airoldi J, O'Neill AM, Einhorn K, Hoffman M. Misoprostol for second trimester pregnancy termination in women with prior caesarean: a systematic review. BJOG. 2009;116(9):1151-7.
3. Kazandi M. Placenta percreta: report of two cases and review of the literature.
4. Venkatesh KV, Harsha B. Uterine rupture at the fundus during pregnancy: a case report. Int J Reprod Contracept Obstet Gynaecol 2015;4(6):2072-3.
5. Buke B, Akkaya H, Demir S, Sagol S, Simsek D, Basol G, Barutcuoglu B. Relationship between first trimester aneuploidy screening test serum analytes and placenta accreta. J Matern Fetal Neonatal Med. 2018 Jan;31(1):59-62
6. Cunningham FG.Prior Cesarean Delivery. In  Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, et al. Williams Obstetrics. 24th ed. New York: McGraw-Hill Education. 2014; pg. 609.

Citation

Shaikh A,  Mayadeo NM, Warke HS. Rupture Uterus In Unscarred Uterus: A Rare Case Report. JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/rupture-uterus-in-unscarred-uterus-rare.html

Early Diagnosis Of Heterotopic Pregnancy Resulted From Assisted Reproductive Techniques By Three Dimensional Ultrasonography

Author Information

Mamta D*,  Kadambari **, Donthi S***, Tolani AD****.

(* Clinical director, ** Obstetrician and Gynecologist, Micro surgeon, *** Research Associate, **** Clinical consultant and Scientific In-Charge. Infertility Institute and Research Centre, Hyderabad, India)

Abstract

The term heterotopic pregnancy describes the presence of two embryos implanted in two different places including the one in the uterine cavity. Timely diagnosis is crucial and is needed to avoid the life threatening complications. Three dimensional ultrasonography has become an important tool in the field of reproductive medicine. It offers the benefits over other techniques in prompt diagnosis of heterotopic and ectopic pregnancies. In this case report we present a heterotopic pregnancy resulted from assisted reproductive technology and its early diagnosis using three dimensional transvaginal ultrasonography (3D-TVS). We could locate the 5 weeks heterotopic pregnancy where one was left ectopic with a gestational sac and yolk sac and the another showed an intrauterine sac. The patient was advised for surgery. She then underwent laparoscopic left salpingectomy with harmonic scalpel and then recovered remarkably. 

Introduction

Heterotopic pregnancy is a condition where implantation of embryos occurs one in the uterine cavity and other in the fallopian tube. Although the frequency of heterotopic pregnancy is less in spontaneous conception, the incidence is quite high with assisted reproductive technology.[1, 2]  Early diagnosis offers benefits over life threatening complications from rupture of ectopic pregnancy. Most of the heterotopic cases are asymptomatic and difficult to identify at early stages. In most heterotopic pregnancies, presence of an intrauterine sac caused the sonographer to overlook the ectopic pregnancy. Hence it is always a diagnostic and therapeutic challenge for a clinician and a sonographer. A through and comprehensive screening is essential, particularly in women undergoing IVF embryo transfer with two or more embryos. Hence in such suspected cases it’s mandatory to search for the second sac even after visualizing the intrauterine sac. Three-dimensional Ultrasonography (3D USG) offers diagnosis of heterotopic pregnancies at early stages. In this case report we present a heterotopic pregnancy resulted from in vitro fertilization and our holistic approach to screen the location of pregnancies using 3D trans-vaginal ultrasonography.

Case Report

A 37 year old lady with BMI 24, a known case of dextrocardia, consulted our institution with her husband for infertility treatment. She was diagnosed poor ovarian reserve and possible grade 1 endometriosis. The couple was suggested for in vitro fertilization which they underwent subsequently. Two early blast embryos, one at 2AA and another at 2BB stage were transferred under ultrasound guidance to mid cavity, 7 mm from the fundus. Her beta HCG levels after 14 days were initially 499 mIU/ml which increased to 780 mIU/ml after 48 hours, 2690 mIU/ml after 48 hours and 5991 mIU/ml after another 48 hours. Then we performed a 3D trans vaginal ultrasonography (3D TV-USG) to locate the pregnancy. 3D-TV-USG (Voluson TM  E 10, GE Healthcare, Austria. GmbH & Co OG) with endovaginal probe (GE-RIC5-9-D) showed a 5 weeks heterotopic pregnancy where one was left ectopic with a gestational sac and yolk sac and another was an intrauterine sac. A transvaginal grey-scale two-dimensional ultrasound image was obtained (Figure 1) and subsequently three-dimensional volumes were applied. We carefully chose the section of interest to include both the implantation structures. Volume rendering was also performed (Figure-2). She was asymptomatic, however in view of the diagnosis made she was referred to a tertiary care unit for follow-up and management. There she was further diagnosed to be a case of heterotopic pregnancy where one was left sided live ectopic pregnancy and the other was non-viable intrauterine pregnancy. The couple was offered surgical treatment options which they agreed and consented. Laparoscopic left salpingectomy was done. An unruptured 2x2 cm ectopic pregnancy was noted in the left tube. The ovaries and upper abdomen appeared normal. Left salpingectomy was done with harmonic scalpel. The intrauterine non-viable pregnancy aborted spontaneously following week after surgery. Histopathology of the salpingectomy specimen was done. Microscopic examination of the section revealed ruptured fallopian tube with dysmorphic edematous villi with scalloped outlines and with focal trophoblastic proliferation embedded in hemorrhage. Cisterns and nuclear atypia was not observed. After the surgery she recovered remarkably and was again planning for another IVF cycle.

Figure 1. Two dimensional (grey-scale) transvaginal ultrasonography image showing an intrauterine sac and a left ectopic gestational sac. 

Figure 2. Three dimensional volume rendered image of heterotopic pregnancy of 5 weeks.

Discussion

Heterotopic pregnancy is simultaneous gestation in more than one implantation sites that includes one in the uterine cavity whilst another implanted outside of the cavity ectopically. First heterotopic pregnancy was found in 1708 during autopsy.[3] The clinical diagnosis of this condition is crucial. The incidence rate is higher in assisted reproductive technology (ART) particularly after ovulation induction.[4,5] Multiple factors contribute for the heterotopic pregnancy in ART including the hydrostatic forces generated during embryo transfer, previous tubal surgeries and pelvic inflammations.[1,4,6] Prompt evaluation and early diagnosis is demanded to diminish the morbidity and mortality. Transvaginal ultrasonography and magnetic resonance imaging (MRI) would be the current beneficial diagnostic tools to screen the condition. However, MRI being an expensive tool and not readily available in all the centers, ultrasonography offers the benefits over it. Hence, 3D ultrasound is an effective alternative of MRI for its easy availability and better comfortability to the patients. Three dimensional ultrasound and Power Doppler are relatively new technologies and offer benefits over 2D ultrasound. 3D ultrasound enables us to acquire the images in three planes such as coronal, sagittal and frontal therefore accurate configuration of the mass can be mapped out.[7] Evaluating the heterotopic pregnancy from adjoining ovarian pathologies such as corpus luteum and other hematologic cyst which are common in early pregnancies can be easily comprehended with 3D ultrasound. Heterotopic pregnancies are seen occasionally in ART treatments and they may be obscured especially in presence of cysts. In such situations 3D ultrasound can be used to differentiate the heterotopic pregnancy from other adnexal pathologies. In the present case 2D and 3D ultrasonography enabled us to screen the heterotopic pregnancy at early stages and helped us to suggest the possible treatment strategies to the patient, preventing the life threatening complications. 

References

1. Govindarajan MJ, Rajan R. Heterotopic pregnancy in natural conception. J. Hum. Reprod. Sci. 2008;1(1):37–38. 
2. Barrenetxea G, Barinaga-Rementeria L, Lopez de Larruzea A, Agirregoikoa JA, Mandiola M, Carbonero K. Heterotopic pregnancy: two cases and a comparative review. Fertil Steril. 2007;87(2):417.e9-15.
3. Varras M, Akrivis C, Hadjopoulos G, Antoniou N. Heterotopic pregnancy in a natural conception cycle presenting with tubal rupture: a case report and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2003;106(1):79-82.
4. Honarbakhsh A, Khoori E, Mousavi S. Heterotopic pregnancy following ovulation induction by Clomiphene and a healthy live birth: a case report. J Med Case Rep. 2008;2:390.
5. Glassner MJ, Aron E, Eskin BA. Ovulation induction with clomiphene and the rise in heterotopic pregnancies: A report of two cases. J Reprod Med. 1990;35(2):175–8.
6. Gruber I, Lahodny J, Illmensee K, Losch A. Heterotopic pregnancy: Report of three cases. Wien Klin Wochenschr. 2002;114(5-6):229–32.
7. Chiou Li Ong. The current status of three-dimensional ultrasonography in gynaecology. Ultrasonography. 2016; 35(1): 13–24.

Citation

Mamta D,  Kadambari , Donthi S, Tolani AD. Early Diagnosis Of Heterotopic Pregnancy Resulted From Assisted Reproductive Techniques By Three Dimensional Ultrasonography.  JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/early-diagnosis-of-heterotopic.html

Sickle Cell Disease In Pregnancy

Author Information

Parihar AS*, Warke HS**, Mali K***.

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

Abstract

Sickle cell is the most common inherited condition worldwide. Pregnancy exacerbates the complications associated with sickle cell disease while sickle cell disease in pregnancy is associated with increased risk of obstetric complications. We present a case of successful pregnancy in a case of sickle cell disease.

Introduction

Sickle cell disease is a group of inherited disorders, caused by mutation in the ‘sickle’ gene, inherited in an autosomal recessive manner, resulting in abnormal structure of haemoglobin.

Sickle cell disease includes sickle cell anemia (HbSS) and the heterozygous conditions of hemoglobin S and other clinically abnormal hemoglobins including hemoglobin C, D, E, O,  Arab as well as combination with beta thalassemia. The abnormal hemoglobin undergoes polymerization under hypoxic conditions leading to abnormal rigid and fragile sickle shaped red cells causing haemolytic anemia and occlusion of small vessels thereby leading to painful crisis.

Sickle cell disease is associated with increased maternal and fetal complications.[1] There is an increased risk of spontaneous abortions, infection, thromboembolic events, antepartum hemorrhage, premature labor, antenatal hospitalizations, increased need for operative intervention and acute painful crisis during pregnancy. Some studies have also shown increased risk of pre-eclampsia and pregnancy induced hypertension [2, 3] while some studies have shown no association.[4, 5] Sickle cell disease is associated with fetal complications such as intra-uterine fetal growth restriction, premature births, abnormal fetal heart rate and increased incidence of perinatal mortality.[6]

A multidisciplinary approach, high vigilance of the health care providers and compliance of the patient is required to effectively manage the pregnancy in a case of sickle cell disease.

Case Report

A 26 year old, G4P2L1MTP1 with 32 weeks of gestation came to emergency department with complaints of gastroenteritis and fever with chills. She was a known case of sickle cell disease diagnosed in 2006 (HbSS 76.3%) with history of previous two lower segment cesarean sections. First cesarean section was done at term for meconium stained liquor. Baby was diagnosed with microcephaly at birth. Baby died of unknown cause at 7 years of age and was not investigated for hemoglobinopathy. Second cesarean section was done at term for fetal distress in a baby with intrauterine growth retardation. Baby was later diagnosed to have sickle cell trait. She had required multiple exchange transfusions in previous two pregnancies.

  

She was antenatally registered at 6 weeks of gestation with us. She was on tablet hydroxyurea which she stopped 2 months prior to this conception. Amniocentesis was done at 15 weeks of gestation for prenatal diagnosis. Amniotic fluid DNA analysis suggested the presence of sickle cell trait in the fetus. She had history of admission at fifth month of gestation for H1N1 infection with anemia which was managed conservatively with parenteral antibiotics and hydration. She received four packed cell transfusions during this admission.

At 32 weeks of gestation, she came with fever with chills and generalized body ache since one day. On examination, she was febrile (temperature of 39 degree celsius), pulse rate was 100 beats/ minute and blood pressure was 110/70 mm Hg. On auscultation, chest was clear, S1S2 were normal. On abdominal examination uterus was relaxed, 30-32 weeks in size, cephalic presentation, FHS were 140 beats per minute. She was admitted for the above complaints in medical unit and was managed. She was treated conservatively by the physician with injection ceftriaxone and metronidazole for 14 days and adequate hydration. Peripheral smear showed no evidence of malarial parasite, tests for malarial antigen, IgG, IgM antibodies for dengue, tests to detect NS1 antigen, IgG, IgM antibodies for leptospirosis and Widal test were negative. Urine culture showed no growth. Her hemoglobin (Hb) on admission was 6.8 gm%, WBC counts were 24,100/ cumm and platelets were 2 lakhs cumm. Her liver function tests were mildly deranged with SGOT 92 U/L, SGPT 27 U/L, total bilirubin 4.7 mg%, direct bilirubin 2.8 mg% and INR 1.03 seconds. Her renal function tests were normal. Ultrasound (USG) abdomen was suggestive of moderate splenomegaly. Hematologist opined to maintain Hb between 8-9 gm% and exchange transfusion to be considered only if new infiltrate on chest X-ray/ hypoxia/ breathlessness / chest pain occurred.  Two units of packed cells were transfused. Liver function tests and INR were monitored every third day. They were in the decreasing trend. Deranged liver function tests with moderate splenomegaly was due to hemolysis. She was closely monitored for development of maternal and fetal risk factors. Two doses of 12 mg injection betamethasone were given. Serial USG scans were done to detect early evidence of fetal/ maternal compromise. USG doppler was suggestive of severe oligohydramnios (AFI 2) without materno-fetal insufficiency. Fetal well being was monitored by performing biweekly non stress test and weekly doppler. Two weeks later, she again developed fever spikes. She was started on injection piperacillin/ tazobactum for 7 days and artisunate was given for 3 days. Peripheral smear showed no evidence of malarial parasite, tests for malarial antigen, IgG, IgM antibodies for dengue, tests to detect NS1 antigen, IgG, IgM antibodies for leptospirosis and Widal test were negative. Urine culture showed no growth. She went into spontaneous onset of labor at 34 weeks of gestation. Her Hb was 8.6 gm%, WBC counts were 10,400/ cumm, platelets were 2.35 lakhs/ cumm, SGOT was 51 U/L, SGPT was 24 U/L, total bilirubin was 2.2 mg%, direct bilirubin of 1.0 mg%, and INR was 0.93.  An emergency lower segment cesarean section was done and she delivered a male child of 2.002 Kg with Apgar score of 9/10. One unit packed cell was transfused intraoperatively. Post cesarean  section, she was continued on parenteral piperacillin/ tazobactum for 14 days, metronidazole for 5 days and gentamycin for 3 days. She was closely monitored for symptoms of sickle cell crisis post cesarean section. She had fever spikes of 39 degree celsius on day 3 postpartum and she also complained of breathlessness. She was managed conservatively on same antibiotics, adequate hydration and nasal oxygen to maintain optimal oxygen saturation. She received one packed cell transfusion post-delivery on Hb 7.6 gm%. She was monitored for two weeks postpartum and was discharged in consultation with the hematologist.

Discussion

Sickle cell disease is characterized by formation of abnormal hemoglobin structure leading to development of fragile sickle cells. These fragile sickle cells are prone to breakdown thereby leading to hemolytic anemias. Sickle cell disease is associated with increased risk of maternal and foetal complications. Our patient suffered from infections several times during her pregnancy. She also went into premature labor.

Women with sickle cell disease should undergo preconceptual screening regarding advice about partner screening, vaccinations, medications and avoidance of crisis. If a partner is a carrier or is affected by a major hemoglobinopathy, the couple must be explained about the risk of having affected offspring and also the choice regarding termination of pregnancy.  As our patient was regularly following up with the hematologist, husband’s electrophoresis was done prior to this conception which revealed no evidence of hemoglobinopathy in the partner. 

Penicillin prophylaxis is beneficial for women with sickle cell disease as they are hyposplenic and are at greater risk of infection with capsulated organisms.[7] Our patient suffered from H1N1 infection at fifth month of gestation and was managed conservatively. Women with sickle cell disease should receive influenza and swine flu vaccine annually.[7] 

Hydroxyurea, a drug used to decrease the incidence of painful crisis[8]should be stopped 3 months prior to conception. Hydroxyurea has been found to be teratogenic in animals, however there are published reports that show no adverse effects in babies of women who have taken hydroxyurea throughout their pregnancy.[9,10] Termination of pregnancy is not indicated based on exposure to hydroxurea alone, however a level 3 ultrasound must be performed to detect structural abnormalities.[11] Our patient stopped the drug two months prior to this conception, however she continued with the drug in her previous two pregnancies. Low threshold for seeking medical help is advisable for women with sickle cell disease. Antenatal care must be provided by a multidisciplinary team including an obstetrician, hematologist and a general physician. Precipitating factors of sickle cell crisis like extreme temperature, hypoxia, and dehydration must be avoided by pregnant women. Iron supplementation is recommended for them only if there is laboratory evidence of iron deficiency. Low dose aspirin (75 mg OD) must be provided from 12 weeks of gestation as they are at mild risk of pre-eclampsia.[12] Administration of low molecular weight heparin is also recommended in these women during their antenatal admissions to avoid thromboembolism.[13] Blood pressure and urinalysis must be done at every antenatal visit, midstream urine for culture should be done monthly. Serial growth scans should be done at every 4 weeks after 24 weeks of gestation to detect early fetal growth restriction. The role of prophylactic blood transfusion in pregnant women with sickle cell disease is inconclusive.[14] Some studies have shown prophylactic transfusion to decrease the maternal painful crisis however it has not shown to influence maternal or fetal outcome.[15] The decision for blood transfusion must be taken after correlating with clinical findings and after consulting experienced obstetrician and hematologist. Prophylactic transfusion must be considered in multiple gestation as they are associated with higher complication rate. Transfusion must be continued in pregnancy for women who are on a transfusion regimen before pregnancy for prevention of severe disease complications. Top up transfusion is recommended for women with previous serious medical, obstetric or fetal complications and to treat acute anemia. Exchange transfusion is the accepted method to manage acute chest syndrome and acute stroke.[7, 16]. Blood should be cross matched for extended phenotype as alloimmunization is common in women with sickle cell disease and it renders women untransfusable. Acute painful crisis should be treated with appropriate analgesia. Mild pain is treated with paracetamol and NSAIDS. Recommended use of NSAIDS is between 12 and 28 weeks of gestation for mild to moderate pain. Weak opioids are used for moderate pain whereas stronger opioids like morphine are used for severe pain. Pethidine should be avoided as it is associated with seizures in women with sickle cell disease.[17] Fluid of at least 60 ml/ kg/ 24 hours should be ensured. Oxygen supplementation is recommended if saturation drops below 95%. Women should be assessed for infection. Therapeutic antibiotics must be started if febrile or there is clinical suspicion of infection. Thromboprophylaxis is given if patient is admitted with painful crisis.

Vaginal delivery between 38 to 40 weeks is the recommended mode of delivery for women with sickle cell disease.[18] Cesarean section should be reserved for obstetric indication. Our patient underwent cesarean section as she had undergone LSCS in previous two pregnancies. Regional anesthesia is recommended for cesarean section. Blood should be cross matched. Concerned hematologist, physician as well as anesthetists must be informed while taking the patient for delivery. Patient must be kept warm and well hydrated during labor. Continuous fetal monitoring should be done as there are high chances of fetal distress. Prophylactic antibiotics is not recommended but the threshold to start broad spectrum antibiotics should be low and the patient should be closely monitored for increase in temperature.

Same level of care and vigilance should be given to women in the post-partum period as the risk of sickle cell crisis remains. Adequate hydration and maternal saturation above 94% is required. Thromboprophylaxis is recommended for 7 days postpartum following vaginal delivery and for 6 weeks postpartum following cesarean section. In women with high risk of sickle cell disease in newborn, early testing for sickle cell disease must be offered. Hematology opinion was taken for the newborn and they advised to do Hb electrophoresis at six months of age. Women need to be counseled regarding modes of contraception. Progesterone only pill and injection DMPA are recommended for women with sickle cell disease.[19, 20]

Expert knowledge of the condition, proper intervention at the time of crisis and appropriate pre-conceptional, antenatal and postpartum management can result in successful maternal and fetal outcome in sickle cell disease in pregnancy. 

References

1. Boulet SL, Okoroh EM, Azonobi I, Grant A, Hooper WC. Sickle cell disease in pregnancy: maternal complications in a Medicaid-enrolled population. Matern Child Health J. 2013;17(2):200-7.
2. Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199(2):125.e1–5.
3. Chakravarty EF, Khanna D, Chung L. Pregnancy outcomes in systemic sclerosis, primary pulmonary hypertension, and sickle cell disease. Obstet Gynecol 2008; 111(4):927–34.
4. Afolabi BB, Iwuala NC, Iwuala IC, Ogedengbe OK. Morbidity and mortality in sickle cell pregnancies in Lagos, Nigeria: a case control study. J Obstet Gynaecol 2009; 29(2):104–6.
5. Serjeant GR, Loy LL, Crowther M, Hambleton IR,Thame M. Outcome of pregnancy in homozygous sickle cell disease. Obstet Gynecol 2004; 103(6):1278–85.
6. Desai G, Anand A, Shah P, Shah S, Dave K, Bhatt H, et al. Sickle cell disease and pregnancy outcomes: a study of the community-based hospital in a tribal block of Gujarat, India. J Health Popul Nutr. 2017; 36: 3.
7. Sickle Cell Society. Blood Transfusion. Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. London: Sickle Cell Society; 2008; pg 69-80
8. Charache S,Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Effect of hydroxyurea on the frequency of painful crisis in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995; 332(20):1317–22.
9. Ballas SK, McCarthy WF, Guo N, DeCastro L, Bellevue R, Barton BA, et al; Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia. J Natl Med Assoc. 2009; 101(10):1046-51.
10. Diav-Citrin O, Hunnisett L, Sher GD, Koren G. Hydroxyurea use during pregnancy: a case report in sickle cell disease and review of the literature. Am J Hematol 1999; 60:148–50.
11. Byrd DC, Pitts SR,Alexander CK. Hydroxyurea in two pregnant women with sickle cell anemia. Pharmacotherapy 1999; 19(12):1459–62.
12. National Institute for Health and Clinical Excellence.Hypertension in pregnancy. The management of hypertensive disorders during pregnancy. NICE clinical guideline 107. London: NICE; 2010.
13. Royal College of Obstetricians and Gynaecologists. Reducing the risk of thrombosis and embolism during pregnancy and the puerperium. Green-top Guideline No. 37a. London: RCOG; 2015.
14. Okusanya BO, Oladapo OT. Prophylactic versus selective blood transfusion for sickle cell Disease in pregnancy. Cochrane Database Syst Rev. 2016 Dec 22; 12:CD010378.
15. Elenga N, Adeline A, Balcaen J, Vaz T, Calvez M, Terraz A, et al. Pregnancy in sickle cell disease is a very high-risk situation: an observational study. Obstetrics and Gynecology International. 2016, Article ID 9069054. 5 pages
16. Styles LA, Abboud M, Larkin S, Lo M, Kuypers FA.Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2. Br J Haematol 2007; 136(2):343–4.
17. Rees DC, Olujohungbe AD, Parker NE, Stephens AD,Telfer P, Wright J; British Committee for Standards in Haematology General Haematology Task Force by the Sickle Cell Working Party. Guidelines for the management of acute painful crisis in sickle cell disease. Br J Haematol 2003; 120(5):744–52.
18. Bick DRM. Caesarean Section. Clinical Guideline. National Collaborating Centre for Women's and Children's Health:Commissioned by the National Institute for Clinical Excellence. Worldviews on Evidence-Based Nursing.2004; 1(3):198–199
19. Legardy JK, Curtis KM. Progestogen-only contraceptive use among women with sickle cell anemia: a systematic review. Contraception 2006; 73(2):195–204.
20. Manchikanti Gomez A, Grimes DA, Lopez LM, Schulz KF. Steroid hormones for contraception in women with sickle cell disease (Review). Cochrane Database of Systematic Reviews 2007, Issue 2. Art. No.: CD006261.

Citation

Parihar AS, Warke HS, Mali K. Sickle cell disease in pregnancy.  JPGO 2017. Volume 4 No.12. Available from: http://www.jpgo.org/2017/12/sickle-cell-disease-in-pregnancy.html