Volume 4 Issue 3, March 2017

Editorial

 Pregnancy With Moyamoya Disease
Dalvi P, Parulekar SV.

Interpretation Of An Atypical Pattern On Non-stress Test
Mahanti S, Parulekar SV.

A Case Of Syndrome Of Inappropriate Anti diuretic Hormone Secretion In Pregnancy
Harsha A, Sharma K, Gupta AS.

Successful Pregnancy Outcome with Pseudo Diaphragmatic Hernia in the Gravida
Bora A, Madhva Prasad S, Gupta AS.

Cephalocentesis In Fetal Hydrocephalus – An Alternative To Cesarean Section For Obstructed Labor
Shah S, Mayadeo NM.

 Innovative Technique of Using 5 mm Laparoscopic Tenaculum Alongside Bipolar Saline Resectoscope in Hysteroscopic Myomectomy
Shah NH, Khadkikar R, Paranjpe S.

 Mullerian Anomaly With Pregnancy
Dhokia T, Shende D, Shah A, Chauhan AR.

Angiomyolipoma Of Vulva
Daruwale R, Kale K, Waghmare T, Chauhan AR.
Dhanawat J, Kumbhar P, Honavar P, Samant PY.

Editorial

Chauhan AR

Obstructed labor is still seen in developing countries and is an important preventable cause of maternal and perinatal morbidity and mortality. Potential risks of obstructed labor are uterine rupture, traumatic and/ or atonic postpartum hemorrhage, sepsis and death; it is responsible for 8% of all maternal deaths. In rare situations with obstructed labor and a dead baby, the obstetrician is faced with the dilemma of a difficult second stage cesarean section (CS) with its attendant maternal morbidity versus saving the mother a surgery and performing a destructive, or more accurately, a reductive procedure.
Literature search of studies from India shows that destructive operations account for a very small proportion of obstetric cases, mainly carried out for obstructed labor with intrauterine fetal demise (IUFD). A 25 year analysis by Sikka in a tertiary care center in Chandigarh found that destructive operations accounted for 0.26 % of all obstetric cases, with craniotomy being the most common in 87.8%, followed by decapitation, evisceration and cleidotomy.  In a retrospective analysis of 7 years by Singhal et al, craniotomy was the commonest destructive procedure. They reported complications like PPH, genital tract injuries and sepsis in as many as 45% of their cases.  
What is the concept of modern obstetrics? It is evidence or fact -based, keeping the best interests of the mother and newborn as the focus. In cases where the baby is already dead, maternal safety is of prime concern. If patients of obstructed labor present early to well equipped healthcare facilities, irrespective of fetal status, it is prudent to perform CS. But this concept of "modern obstetrics" is an oxymoron in many parts of rural India, which supports the argument in favor of destructive procedures: in remote parts of the country, where healthcare expertise and transport facilities are lacking or poor, destructive procedures may be life saving; for example, with a few instruments and local anesthesia, simple tapping of the hydrocephalus head may aid vaginal delivery and obviate the need for CS and attendant operative morbidity. 
Though horrific barbaric instruments like cephaloclast and cranioclast, saws, hooks and perforators should be relegated to the museum, cephalocentesis may have a role to play in certain cases. Hydrocephalus is one of the common congenital malformations, easily diagnosed with USG. Traditionally, most cases especially those with gross hydrocephalus and severe cortical loss have poor perinatal outcomes. On the other hand, cephalocentesis is associated with fetal demise in majority of cases. The scenario is however changing, and fetal survival is possible hence the parents should be counseled regarding the pros and cons of cephalocentesis and CS, and tapping should be done only with consent of the patient.   Reduction in the size of the head can be simply achieved by cephalocentesis, a term used to describe puncturing or tapping the fontanelle and draining the cerebrospinal fluid. In most cases this is achieved per vaginum after at least half to 3/4th dilatation using a long wide bore spinal needle. In cases when the large hydrocephalic head fails to engage, suprapubic tapping, after ensuring that the bladder is empty, can help in reduction of the fetal skull diameters and affect a vaginal delivery. Similarly, in breech presentation, after delivery of the limbs and trunk, the aftercoming head can be decompressed vaginally or abdominally. Immediate complications are genital tract trauma and injury to the bladder and rarely, rectum.
Can we do away with destructive procedures? The answer lies in the continued occurrence of obstructed labor and is a sad commentary on the state of healthcare in developing countries. Till the incidence of obstructed labor is not decreased or ceases to exist, obstetricians will need this skill -set to tackle neglected and poorly managed cases.
This issue carries an interesting case of USG guided cephalocentesis which prompted this article on the role of destructive procedures in modern obstetrics; we hope that both will be of interest to the reader.

Pregnancy With Moyamoya Disease

Author Information

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

Abstract

Moyamoya disease  is a progressive cerebrovascular disease characterized  by bilateral occlusion of the arteries supplying the circle of Willis and resulting in a prominent arterial collateral circulation, the vessels of which are fragile. The disease is characterized by thickening of the intima in the walls of the internal carotid arteries bilaterally. Intraventricular, subarachnoid, and intracerebral hemorrhage are known to occur in these patients. We present a case of a woman with Moyamoya disease  who underwent an emergency lower segment cesarean section. 

Introduction

The meaning of Japanese word “Moyamoya” is hazy or like “a puff of smoke in the air.” The term was originally used to describe the angiographic appearance of the affected vascular collateral network.[1] 
The exact etiology of the disease is not known, but the incidence is higher in the Japanese and other Asian people. A familial inheritance occurs in around 10 percent of cases.[2] It is twice as often seen in women as in men. A considerable number of women with Moyamoya disease may be in the child bearing age group. Clinical features include cerebrovascular events such as transient ischemic attacks (50-75%), hemorrhagic stroke (10-40%), and infarction. Diagnosis is made by demonstration of stenotic or occlusive lesions in the distal internal carotid arteries and in the arteries proximal to the circle of Willis with vascular imaging with magnetic resonance (MR), computerized tomographic (CT), or conventional cerebral angiography.

Case Report

A 35 year old woman,  married for 1.5 years, second gravida with one prior stillbirth was referred to us at 39 weeks of gestation for breech presentation and two loops of cord around the neck on ultrasonography. She was a known of Moyamoya disease diagnosed at the time of her previous pregnancy on CT angioangiography . She had history of  severe preeclampsia in her first pregnancy which had to be terminated at 6 months’ gestation for maternal indications.  Her previous pregnancy had been further complicated by intracerebral frontoparietal hemorrhage which had been managed conservatively by the neurosurgery department.   There was no history of any other major medical or surgical illness. She was on tablet labetalol 100 mg twice a day for three months. She had also been taking tablet aspirin 75 mg until 3 months ago. There was no history of any bowel or urinary bladder related symptoms. Her clinical examination revealed a pulse of 90 beats per minute, blood pressure was 100/80 mm of Hg, no pallor no edema. The uterus was full term, the fetus in a breech presentation, fetal heart rate 140 per minute, regular. General and systemic examination revealed no abnormality. The uterus was relaxed. There was no bleeding or leaking per vaginum. The cervix was 2.5 cm dilated, 40% effaced, station was -1, membranes intact and the pelvis was adequate. Her hematological and biochemical investigations for fitness for anesthesia were normal. Neurosurgeons found her to fit for undergoing surgery. A decision for an emergency lower segment caesarean section (LSCS) was taken in view of breech presentation of the fetus.  LSCS was done under general anesthesia and a female child of 2.6 kg with Apgar 9/10 at 1 and 5 minutes was delivered. Intraoperative and postoperative course was uneventful. The patient made an uneventful recovery.

Discussion

Moyamoya disease has no definitive treatment other than palliation. Individuals experiencing TIAs and strokes are prescribed aspirin, vasodilators, or anticoagulants to reduce the risks of future recurrent neurological sequelae.[3]. A large number of cases in literature have initially presented as an intracranial hemorrhage with neurological dysfunction.[4,5,6] The control of blood pressure is of utmost importance. Risk of thrombosis is also increased in pregnancy; thus, aspirin may even be more essential during this period. Maintenance of hemodynamic stability during pregnancy is important in avoiding neurological events. It depends on adequacy of analgesia, maintaining fluid balance, avoiding fluctuations in blood pressure, and reducing Valsalva's maneuver. Many neurosurgeons believe that vaginal delivery has more risk than cesarean section without data supporting the same. To avoid problems at delivery, scheduled cesarean section is preferred in by some workers for fear of litigation. 
The preferred mode of delivery remains unclear. LSCS was chosen in the majority of previously reported cases. This has been suggested to better control changes in blood pressure and potential variations in cerebral blood flow that may occur during the active and second stages of labor. Women with Moyamoya disease having had vaginal deliveries without neurological sequelae have been reported.[4,7]
Lee et al reported the use of  single­photon emission computed tomography to assess cerebral hemodynamic status to help determine women at greater risk of development of neurological events during pregnancy and puerperium. They postulated that revascularization surgery prior to conception may help avoid neurological events during pregnancy and puerperium.[8]
General anesthesia may provide stable systemic condition, but hypertension, vomiting at the time of endotracheal intubation may occur. Spinal and epidural anesthesia may cause hypotension and the patient may present hyperventilation or hypertension due to anxiety. Epidural anesthesia can provide good pain control in the post-delivery period.

Conclusion

There is no consensus regarding the safest method of delivery, or anesthesia.  So the best approach would be the one with which obstetricians, anesthesiologists and neonatologists are familiar. Adequacy of anesthesia and maintenance of hemodynamic status seem to be more important than the mode of delivery. LSCS under epidural anesthesia is a good option. Alternatively vacuum or obstetric forceps assisted vaginal delivery can also be achieved with due care.

References
  1. Suzuki J, Kodama N. Moyamoya disease: a review. Stroke. 1983;14(1):104–109. 
  2. Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology. 2000;20(supplement):S61–S64.
  3. Cunningham FG, Leveno KJ, Bloom SL. Williams Obstetrics. New york, NY, USA: 2010. Neurological and psychiatric disorders; pp. 1167–1169.
  4. Komiyama M, Yasui T, Kitano S, Sakamoto H, Fujitani K, Matsuo S. Moyamoya disease and pregnancy: case report and review of the literature. Neurosurgery. 1998;43(2):360–369. 
  5. Sucholeiki R. Moyamoya Disease. Emedicine. 2006, Available at: http://www.emedicine.medscape.com/article/1180952-overview.
  6. Kato R, Terui K, Yokota K, Nakagawa C, Uchida J, Miyao H. Anesthetic management for cesarean section in moyamoya disease: a report of five consecutive cases and a mini-review. International Journal of Obstetric Anesthesia. 2006;15(2):152–158. 
  7. Amin-Hanjani S, Kuhn M, Sloane N, Chatwani A. Moyamoya disease in pregnancy: a case report. AmJ Obstet Gynecol 1993;169:395–396.
  8. Lee SU, Chung YS, Oh CW, Kwon OK, Bang JS, Hwang G, et al. Cerebrovascular Events During Pregnancy and Puerperium Resulting from Preexisting Moyamoya Disease: Determining the Risk of Ischemic Events Based on Hemodynamic Status Assessment Using Brain Perfusion Single-Photon Emission Computed Tomography. World Neurosurg. 2016 Jun;90:66-75.
Citation

Dalvi P, Parulekar SV. Pregnancy With Moyamoya Disease. JPGO 2017. Volume 4 No. 3. Available from: http://www.jpgo.org/2017/03/pregnancy-with-moyamoya-disease.html

Interpretation Of An Atypical Pattern On Non-stress Test

Author Information

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

Abstract

Fetal well being can be assessed by a variety of tests including the fetal heart rate (FHR) monitoring. The FHR pattern is assessed with respect to the baseline as well as changes related to various events. It is done in the antenatal period using a non stress test (NST). We present a case in which the NST showed periodic accelerations with fetal movements, the long term variability being maintained during the accelerations. Such a pattern has not been described in the world literature. 

Introduction

NST is the most widely used and accepted test of fetal well surveillance that involves electronic fetal monitoring and tracing fetal heart rate patterns over 20 minutes when the patient is not in labor.[1] The baseline FHR is assessed with respect to its value, as well as short term and long term variabilities. Presence of accelerations and decelerations is also noted and analyzed. Various patterns encountered are due to specific physiological and pathological processes. We describe a new pattern in which baseline long term variability was maintained during FHR accelerations.

Case Report

A 31 year old, third gravida first para with a cesarean section and a  spontaneous abortion in the past was referred to us at 37 weeks of gestation in view of FHR tracing showing a baseline tachycardia. Patient complained of intermittent minimal pain abdomen only. She was a known case of gestational hypothyroidism on tablet levothyroxine 12.5 micrograms once a day. Her other medical and surgical history was not contributory. On examination her vital parameters were normal, general and systemic examination revealed no abnormality. Obstetric examination showed a full term fetus and vertex presentation. FHR was regular at 150 bpm. The FHR tracing done by the referring obstetrician is shown in figure 1. It showed baseline FHR of 140 bpm, baseline short term variability of 2-3 bpm, long term variability of 5-10 bpm, and 7 episodes of FHR accelerations up to 180 bpm, each one associated with fetal movements  and lasting for 1.5 to 2 minutes. One lasted for 2.5 minutes, associated with continuous fetal movements. There was no baseline tachycardia as was stated in the referral note. The NST was done again at our center, 3.5 hours after the first NST. Its results are shown in figure 2. It showed a baseline FHR of about 130 bpm, short term baseline variability of 2-3 bpm, long term variability of 5-10 bpm, and accelerations with fetal movements reaching 150 bpm, lasting for 0.5 -1 minute. Long term variability was not seen to be maintained during the accelerations. The patient was reassured and sent home. She followed up regularly in the antenatal clinic of the referring doctor and had a cesarean section after 2 weeks for nonprogress of labor, delivering a baby weighing 3.2 kg, with 1 and 5 minute Apgar scores of 9/10. The baby was normal.


Figure 1. NST done by referring obstetrician.


Figure 2. NST done at our center.

Discussion

Normal fetal heart rate ranges between 110 bpm and 160 bpm. It is not fixed for a given fetus, but keeps changing under the combined effects of sympathetic and parasympathetic activity, arterial baroreceptors and chemoreceptors, circulating levels of catecholamines, and the state of the myocardium and its conducting system.[2,3,4,5] Central stimuli act by sending impulses through direct neuronal pathways to sinoatrial node of the fetal heart and bring about rapid change in FHR. Low pH and oxygen level act through chemoreceptor stimulation and bring about slower changes. Changes in fetal heart rate from one beat to the next are due to the neuronal mechanism, while the long term variation in FHR is due to the chemoreptor mechanism. Fetal breathing and body movements act though the central pathways to medulla oblongata and from there to the sympathetic and parasympathetic systems. Factors which depress fetal central nervous system suppress FHR variability. Sympathetic blockade causes a reduction in long-term variability.[6,7] Parasympathetic blockade causes a reduction in short-term variability.[8] Baseline FHR variability is usually reduced with baseline FHR tachycardia (FHR above 160 bpm). FHR acceleration is a sudden increase in FHR above baseline of at least 15 bpm lasting for at least 15 seconds after 32 weeks of gestation, and of of at least 10 bpm lasting for at least 10 seconds before 32 weeks of gestation. A prolonged acceleration lasts for more than 2 minutes. FHR accelerations are seen with fetal movements, scalp stimulation, and sometimes with uterine contractions. Acceleration of 10 minutes or longer is considered to be a change in the baseline. The short and long term variabilities of FHR are not seen during FHR acceleration.[9,10] An acceleration is an abrupt increase in FHR above baseline with onset to peak of the acceleration less than < 30 seconds and less than 2 minutes in duration. The duration of the acceleration is defined as the time from the initial change in heart rate from the baseline to the time of return to the FHR to baseline.

In the case presented, the fetus was quite active, and 7 episodes of repeated fetal movements produced FHR accelerations lasting for up to 2.5 minutes, which was misinterpreted by the referring obstetrician as baseline tachycardia. The fact that the FHR reached the baseline of 130 bpm periodically indicates that it was not baseline tachycardia and was periodic acceleration of FHR. The curious finding in this case was that the long term variability of the baseline FHR was maintained during the accelerations, which is not normally seen. Normally the stimulus that causes FHR accelerations acts on the central neural pathway and produces a strong progressive increase in FHR which overrides long term variation in the baseline. In this case the fetal movements were frequent, and the accelerations in FHR were large and sustained. In spite of that the long term variability persisted, which could not be explained. The pattern was not seen in other women undergoing NST at that center, thereby ruling out machine error. It was also not seen in the same patient at our center, when the machine used was different. The only factor left was the state of the myocardium of the baby, which has its own intrinsic effect on the FHR variability. However the interval between the two NSTs was only 3.5 hours, and the fetal myocardium would not show any change of such a magnitude in that interval. The pattern was not due to random mixture of bigger and smaller accelerations related to stronger and weaker fetal movements, because such a relationship does not exist.

Conclusion

The Interpretation of FHR patterns is believed to be standardized as all possible types of patterns have been described and analyzed. However we have described a new pattern, which needs further study. Any such occurrences need to be reported so that adequate amount of data can be collected and analyzed.

References
  1. Evertson LR, Gauthier RJ, Schifrin BS, Paul RH Antepartum fetal heart rate testing. I. Evolution of the nonstress test.Am J Obstet Gynecol. 1979 Jan 1;133(1):29-33.
  2. Renou P, Warwick N, Wood C :Autonomic control of fetal heart rate. Am J Obstet Gynecol 1969;105:949.
  3. Gagnon R, Campbell K, Hunse C, Patrick J Patterns of human fetal heart rate accelerations from 26 weeks to term. Am J Obstet Gynecol. 1987 Sep;157(3):743-8. 
  4. Electronic fetal heart rate monitoring: research guidelines for interpretation. National Institute of Child Health and Human Development Research Planning Workshop. Am J Obstet Gynecol. 1997 Dec;177(6):1385-90.
  5. Paul RH, Suidan AK, Yeh S, Schifrin BS, Hon EH Clinical fetal monitoring. VII. The evaluation and significance of intrapartum baseline FHR variability. Am J Obstet Gynecol. 1975 Sep 15;123(2):206-10.
  6. Wakatsuki A, Murata Y, Ninomiya Y, Masaoka N, Tyner JG, Kutty KK. Autonomic nervous system regulation of baseline heart rate in the fetal lamb. Am J Obstet Gynecol. 1992 Aug;167(2):519-23.
  7. Kleinhout J, Stolte LA, Janssens J, Knoop AA. The fetal autonomic nervous system, the fetal heart rate and the beat-to-beat irregularity. Eur J Obstet Gynecol Reprod Biol. 1977;7(6):373-6.
  8. Dalton KJ, Dawes GS, Patrick JE. The autonomic nervous system and fetal heart rate variability. Am J Obstet Gynecol. 1983 Jun 15;146(4):456-62.
  9. American College of Obstetricians and Gynecologists. ACOG Practice Bulletin No. 106: Intrapartum fetal heart rate monitoring: nomenclature, interpretation,  and  general  management principles.  Obstet Gynecol. 2009;114(1):192-202.
  10. Macones GA, Hankins GD, Spong CY, Hauth J, Moore T. The 2008 National Institute of Child Health and Human Development workshop report on electronic fetal monitoring: update on definitions, interpretation, and research  guidelines. Obstet Gynecol 2008;112(3):661-666.
Citation

Mahanti S, Parulekar SV. Interpretation Of An Atypical Pattern On Non-stress Test. JPGO 2017. Volume 4 No. 3. Available from: http://www.jpgo.org/2017/03/interpretation-of-atypical-pattern-on.html

A Case Of Syndrome Of Inappropriate Anti diuretic Hormone Secretion In Pregnancy

Author Information

Harsha A*, Sharma K**, Gupta AS***.
(* First Year Resident, ** Specialty Medical Officer, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.) 

Introduction

Hyponatraemia is a common electrolyte imbalance disorder. Cases of hyponatremia in pregnancy are less common, but can have major consequences for both the mother and child. It is important to diagnose hyponatremia, recognize its symptoms and its causes and commence prompt treatment.

Abstract

Hyponatremia in pregnancy can have serious ill effects on mother and fetus. It is important to measure plasma sodium concentration, plasma osmolality, urine sodium concentration and urine osmolality to distinguish between various pathophysiological causes of hyponatremia. In this article we present a case where hyponatremia caused convulsions in a primigravida in early pregnancy and how syndrome of inappropriate anti diuretic hormone secretion (SIADH) was diagnosed as the cause of hyponatremia.

Case Report

A 26 year old, primigravida, married since 2 months, came to emergency with complaint of pain in abdomen since four days, which had increased gradually in intensity and frequency. She also had few episodes of vomiting. She had her last menstrual period 37 days prior to the day of presentation. Urine pregnancy test was done which was faintly positive. Ultrasound showed no evidence of any gestational sac like structure in the uterine cavity or the adnexa.  She continued to have vomiting, non bilious, non blood stained. Her liver and renal function tests and serum electrolytes were normal with sodium levels of 137 mEq/L  which was also within normal limits. Serum β HCG levels were 378.19 mIU/mL. She was kept on antiemetics and serum β HCG was being monitored to look for effective rise in β HCG levels. Her vomiting had stopped after admission. On day 4 of admission she had 2 episodes of  generalized tonic  clonic seizures involving all four limbs and frothing from the mouth. She did not regain consciousness in between two episodes of seizures. Each episode lasted for 30 seconds with an interval of 1 minute between the two seizures. She was drowsy after the episodes, responding only to deep painful stimuli. A sudden unexplained drop in her serum sodium levels from normal to 108 mEq/L was found indicating hyponatremia as the cause of seizures. There was no history of convulsions or any other central nervous system (CNS) disease in the past.  She had a history of abdominal tuberculosis 4 years back which was completely treated. Her serum osmolality was 226 mOsmol/kg (normal: 275-295 mOsmol/kg) while urine osmolality was 282 mOsmol/kg (average value: 200 – 500 mOsmol/kg). Her urea levels were 6 mg/dl and creatinine was 0.9 mg/dl. The adrenocorticotropic hormone level in plasma was raised to a value of 68.4 pg/mL, with a normal serum cortisol level and normal thyroid hormone levels. Physician  and nephrologist were consulted. She was managed in medical intensive care unit (MICU) for 13 days. In view of serum hypo osmolality, urine hyper osmolality with normal cortisol and thyroid levels a diagnosis of  SIADH was made. Her MRI brain showed an old calcified granuloma in right parietal parafalcine lobe. A possibility was that it could be a sequelae of the old tuberculous infection. A contrast enhanced computerized tomography  (CECT) would have further clarified the nature of the lesion in the CNS that could have been the cause of  her SIADH. Her sodium levels, were corrected by infusion of 3% normal saline, following which her serum sodium levels returned to normal. Her sodium levels were monitored and remained within normal limits. The β HCG levels on the other hand kept showing an increasing trend, upto a level of 748.82 mIU/mL and 1110.62 mIU/ mL on day 4 and 6 of her admission (day 40 and 42 of her LMP). An intrauterine pregnancy of 6weeks 1day was confirmed on a repeat scan done on day 53 of her LMP (7 weeks, 4 days by dates). She was discharged on steroids with advise of follow up for two weekly serum electrolytes monitoring and was advised a post delivery CECT to look for any CNS cause of SIADH. She is following regularly for antenatal care. She presently of oral Prednisolone  and has had no further episodes of hyponatremia induced symptoms till date.

Discussion

Hyponatremia is a condition in which sodium levels in blood are less than normal ( <135mEq/l ).[1] Symptoms of mild hyponatremia are headaches, nausea, confusion and poor balance.[2] Severe hyponatremia when the sodium  level is below 120 mEq/L causes intracerebral osmotic fluid shifts and brain edema which leads to convulsions and coma.[3] Hyponatremia can be classified based on volume status into hypovolemic, euvolemic and hypervolemic. While vomiting, diarrhea and excess use of diuretics can cause hypovolemic hyponatremia; hypervolemic hyponatremia may be caused by heart failure, cirrhosis and renal failure. Glucocorticoid deficiency, hypothyroidism and the condition of our interest; SIADH  are causes of euvolemic hyponatremia. Our patient had severe hyponatremia with normal cardiac, renal and liver parameters. Though she had vomiting, the hyponatremia presented long after the vomiting had ceased and no signs of dehydration were present. Our patient was thus a case of euvolemic hyponatremia. It is necessary to rule out renal disease and insufficiency of thyroid, pituitary, and adrenal glands, before attributing the hyponatremia to SIADH in euvolemic states. This was done in our case. Thus SIADH is mostly a diagnosis of exclusion. SIADH is defined by inappropriate secretion and function of anti diuretic hormone which reduces water excretion excessively despite a normal or increased plasma volume resulting in hyponatremia and hypo osmolality.[4] During pregnancy, physiologic changes occur in intravascular volume and osmoregulation that aggravate the problem of SIADH. Mean plasma osmolality reduces by 5-10 mmol, and serum sodium levels reduce by 5mmol/l in a normal pregnancy.[5]  This drop in plasma osmolality is caused by a ‘reset osmostat’ phenomenon: that is ADH-release and a thirst stimulus start at a comparatively lower serum osmolality level in pregnancy.[6] As early as 6 weeks of pregnancy there is vasodilation resulting in a decrease in effective circulating volume subsequently decreasing blood pressure which trigger non-osmotic arginine vasopressin release.[7] Common causes of SIADH are neurological and pulmonary disorders and certain psychiatric medication. However pregnant women experience pain, anxiety and nausea very frequently and these become more common causes of increased ADH production in pregnancy. Bartter and Schwartz in 1967 laid down criteria to define SIADH which are valid till today.[8] The criteria includes serum hyponatremia with corresponding hypoosmolality,  urinary hyperosmolality, an absence of features of volume depletion (normal skin turgor, normal blood pressure), absence of other causes of hyponatremia (adrenal insufficiency, hypothyroidism, cardiac failure, renal disease with salt wastage, hepatic disease, and drugs) and correction of hyponatremia by fluid restriction. Hyponatremia (serum Na+ <135 mEq/L), serum hypo osmolality (serum osmolality <280 mOsm/kg) and high urine osmolality characterizes SIADH. ADH secretion is suppressed in serum hypoosmolality to permit the excess water to be excreted in urine causing urine osmolality to be less than 100 mOsm/kg. Therefore, in a case of plasma hypoosmolality if urinary osmolality is more than 100 mOsm/kg, ADH excess is confirmed. In our case hyponatremia of 108 mEq/L, low serum osmolality of 226 mosm/kg and high urine osmolality of 282 mosmol/Kg suggested SIADH. In SIADH total body water increases in which blood urea gets diluted resulting in lower levels of blood urea nitrogen (BUN), usually below 10 mg/dl. In our case urea levels were always < 6 mg/dl. In SIADH, serum potassium concentration generally remains unchanged because potassium moves from intra cellular to extra cellular space which prevents its dilution. In our case potassium was 3.4 mEq/L. During second trimester and delivery, it can be difficult to differentiate between hyponatraemia and impending eclampsia as both present with symptoms of headache, nausea, dizziness, drowsiness, coma and seizures. Aggressive treatment of hyponatremia is indicated in patients who have severe symptoms (like convulsions, stupor, coma) and who have an acute episode of moderate to severe hyponatremia of a duration of less than 48 hours. In our case correction with 3% hypertonic saline was given which caused sodium levels to return to normal. Sodium levels were then serially monitored which remained normal without any further treatment. Hyponatremia correction rate was monitored as too aggressive correction can cause central pontine myelinolysis (CMP). In pregnancy and lactation, vaptans (ADH receptor antagonists) are contraindicated as these are category C drugs. Diuretics are also avoided as there is a risk of IUGR. Hypertonic saline and fluid restriction are main modalities of treatment in pregnancy. During labor, use of oxytocin should be avoided and if used sodium levels should be monitored. Case has been reported of patients with SIADH developing hyponatremia due to oxytocin use in labor.[9]  Oxytocin has a structure and function similar to ADH and causes water reabsorbtion and hyponatremia. Chances of developing hyponatremia are less when oxytocin is dissolved in NaCl 0.9 % or Ringers Lactate and chances are more if oxytocin is used with Glucose 5%.[10] Hyponatraemia in pregnancy is a significant problem as it can cause severe symptoms and even mortality. Hyponatremia in mother can cause low sodium levels in the child both in utero and during immediate postpartum because sodium exchanges take place in the placenta.[11] Thus sodium levels need to be maintained in normal range for benefit of both mother and fetus. SIADH ,which is an important cause accounting for almost one third cases of hyponatremia in pregnancy, should be promptly diagnosed and treated.

References
  1. Lee JJY, Kilonzo K, Nistico A, Yeates K. Management of hyponatremia. Canadian Medical Association Journal  2014; 186(8):E281-6.
  2. Henry DA. In The Clinic: Hyponatremia. Ann Intern Med 2015; 163(3):ITC1-19.
  3. Williams DM, Gallagher M, Handley J, Stephens JW. The clinical management of hyponatraemia. Postgrad Med J. 2016; 92(1089):407–11.
  4. Cuesta M, Thompson CJ. The syndrome of inappropriate antidiuresis (SIAD). Best Pract Res Clin Endocrinol Metab. 2016;30(2):175–87.
  5. Lindheimer MD, Barron WM, Davison JM. Osmoregulation of thirst and vasopressin release in pregnancy. Am J Physiol. 1989 Aug; 257(2 Pt 2):F159-69
  6. Lindheimer MD, Davison JM. Osmoregulation, the secretion of arginine vasopressin and its metabolism during pregnancy. Eur J Endocrinol 1995; 132(2):133–43.
  7. Schrier RW. Water and sodium retention in edematous disorders: role of vasopressin and aldosterone. Am J Med 2006;119(7 Suppl 1):S47–53.
  8. Bartter FC, Schwartz WB. The syndrome of inappropriate secretion of anti diuretic hormone. Am J Med 1967 May;42(5):790–806.
  9. Jellema J, Balt J, Broeze K, Scheele F, MW. Hyponatraemia during pregnancy. Internet J Gynecol Obstet. 2008;12(1).
  10. Stratton JF, Stronge J, Boylan PC. Hyponatraemia and non-electrolyte solutions in labouring primigravida. Eur J Obstet Gynecol Reprod Biol 1995 Apr 59(2):149–51
  11. Ravid D, Massarwa LE, Biron-Shental T, Fejgin MD. Hyponatraemia and preeclampsia. J Matern Fetal Neonatal Med 2005;18(1):77-8.
Citation

Harsha A, Sharma K, Gupta AS. A Case Of Syndrome Of Inappropriate Anti diuretic Hormone Secretion In Pregnancy. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/a-case-of-syndrome-of-inappropriate.html

Successful Pregnancy Outcome with Pseudo Diaphragmatic Hernia in the Gravida

Author Information

Bora A*, Madhva Prasad S**, Gupta AS***
(* First Year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Diaphragmatic hernia occurs due to defect in the diaphragm causing herniation of the intra-abdominal organs into the thoracic cavity. While obstetricians commonly encounter diaphragmatic hernia in the unborn fetus, it is rare to encounter the same in the mother. A successfully managed pregnancy in a patient with a known diaphragmatic hernia but which was actually a diaphragmatic eventration is presented here.

Introduction

Diaphragmatic hernia is the herniation of intraabdominal contents into the thoracic cavity due the presence of a defect in the diaphragm. When associated with pregnancy, the condition might worsen as pregnancy causes increased intraabdominal pressure.

Case Report

A 31 year old fifth gravida second para with two abortions and two living issues, presented to us for antenatal registration at 12 weeks of gestation. Her first two pregnancies were uneventful.  In her 3rd pregnancy she developed gradually worsening breathlessness around 7 month of gestation. She was admitted under the pulmonology department and evaluated in detail. X ray chest (done  with abdominal shielding) was suggestive of bronchiectatic changes and peribronchial cuffing with cystic changes suggestive of chronic bronchitis; and relative abnormal position of the two hemidiaphragms.  MRI was done showing a defect in the left hemidiaphragm with herniation of left kidney along with colon and part of small bowel into the thorax. Kidney was malrotated and hilum was facing infero-laterally. Renal vasculature was maintained.  She was managed with nebulisation, levosalbutamol, ipratropium bromide and budesonide. She delivered in a tertiary care hospital under close observation at 9 months of gestation, a female child of 2.75 kg.  She was asymptomatic in the inter pregnancy intervals and she had no episode of breathlessness. In the present (5th) pregnancy, her above obstetric history and reports were seen. Upon further enquiry, she gave history of some surgery done in early childhood, but no documentation was available. Presence of a transverse scar extending from right hypochondrium to the epigastric area suggested that it could have been a congenital diaphragmatic hernia which was repaired. In view of cough and dyspnea on exertion, she required admission in the pulmonology ward at 28 weeks of gestation. She was managed with supplemental oxygen and bronchodilators and was discharged upon improvement. At 37 weeks, she was readmitted with similar complaints and improved symptomatically. She developed mild gestational hypertension and her blood pressure was 140/90 mm of Hg. Investigations including complete blood counts, liver, renal parameters and coagulation profile were all within normal limits. She went into spontaneous labor at 38 weeks of gestation, and had a precipitate labor. She delivered a male child of 2.416 kilograms. Intrapartum, patient was continued on nebulisation with levosalbutamol, ipratropium bromide and budesonide. Post-delivery her CT scan was done which was suggestive of focal eventration of left dome of diaphragm. There was no evidence of herniation. CT scan report stated fibrocavitary destruction in the right upper lobe, emphysematous bilateral lungs, fibrocalcific infiltrates in the left lung, and focal posterior eventration of the left dome of diaphragm (figure1). Post-delivery X ray was suggestive of abnormal radio densities and lucencies in the left lower zone in the retro cardiac region. Dome of diaphragm was continuous over the opacity in the lateral view. Fibrocalcific scars in the right upper zone and lower zone were suggestive of post Koch’s sequel (figure 2).


Figure 1. 1.Absent stomach bubble shadow. 2. Homogeneous radiodense opacity suggestive of soft tissue, above the diaphragm. 3. Peak of elevated dome of diaphragm.


Figure 2. CT scan film showing 1. Air in the bowel loops.


Figure 3. CT scan film showing 1. Homogeneous soft tissue shadow (hernia sac).

Discussion

The diaphragm that develops during 4th to 12th week of pregnancy acts is a dome-shaped partition between the thoracic and abdominal cavities. It develops from 4 major structures namely the septum transversum, the pleuroperitoneal membranes, the dorsal esophageal mesentery and the body wall.[1] Congenital diaphragmatic hernia (CDH) is a developmental anatomical defect in the diaphragm that allows herniation of intra-abdominal organs into the chest cavity.  Diaphragmatic eventration refers to incomplete muscularization of the diaphragm resulting in a thin membranous sheet of tissue.[2] While the neonates with diaphragmatic hernia usually present with respiratory insufficiency, adults may be completely asymptomatic till late, as was seen in our patient. They may manifest new onset of respiratory or gastrointestinal symptoms due to herniation of abdominal contents into the thoracic cavity through the defect. Various conditions can predispose to this, out of which pregnancy is one such condition. Imaging is necessary to detect diaphragmatic hernia as the symptoms are nonspecific. Gas above the diaphragm or the fluid filled viscera above the diaphragm suggest that herniated organ can be stomach or the bowel. It is difficult to diagnose if non-gastrointestinal tissue has herniated. Some features of diaphragmatic hernia were seen in our patient also, but were not severe enough to cause symptomatology necessitating operative intervention. The magnetic resonance imaging done in the previous pregnancy (of which images were not available) was suggestive of a defect in the left hemidiaphragm with herniation of left kidney along with colon and part of small bowel into the thorax. During that time also, patient only had respiratory complaints such as breathlessness and no gastrointestinal symptoms. The computerized tomography imaging  in present postpartum period showed focal eventration of left dome of diaphragm but no evidence of herniation. It also showed fibrocavitary destruction in right upper lobe, emphysematous bilateral lungs and fibrocalcific infiltrates in the left lung. Considering these imaging reports, it appears that our patient may not have had a true recurrence of diaphragmatic herniation, but may have had only eventration. This explains the patient not requiring a surgical management during the course of her previous pregnancy and the current pregnancy. The x ray report suggesting that the dome of the diaphragm was continuous over the opacity in the lateral view also supports this. The combined effect of diaphragmatic eventration, fibrocavitary destruction and emphysematous bilateral lungs resulted in restrictive lung disease, explaining the patients’ respiratory symptomatology. Though patient required admission twice, she responded to conservative management and operative intervention was not required. Diaphragmatic hernia complicating pregnancy is rare. Chen et al had reported that only 30 reports had been published over 50 years in the English medical literature.[3] Previously, Dumont had mentioned that more than half of the patients with diaphragmatic hernia in pregnancy succumb during pregnancy. This highlights the importance that needs to be attached to this condition when it is encountered in pregnancy.[4] In pregnancy increased intra-abdominal pressure and gravid uterus leads to herniation of abdominal contents into the thorax. In the first trimester, maternal diaphragmatic hernia can present similar to hyperemesis gravidarum, probably due to strangulation of the abdominal contents in the thoracic cavity. Hence a high index of suspicion should be kept to include maternal diaphragmatic hernia in the differential diagnosis; especially among women who have history of some abdominal surgery.[5] Our patient did not have any specific features of hyperemesis during the first trimester of pregnancy. However, our patient presented with respiratory symptomatology in the second trimester. A few cases have been reported wherein the severity necessitated operative intervention.  Eglinton et al in their three case series concluded that consideration should be given to repair even asymptomatic hernias in the second trimester of pregnancy.[6] Hernia can be repaired through thoracic or abdominal route. Primary repair can be done if the diaphragmatic defect is small whereas for large defects prosthetic patch or grafts are used to avoid tension after repair. Repair of hernia in pregnancy gets complicated by reduction of intra-abdominal space and decreased visualization.[7,8] The repair of hernia in pregnancy carries risk of preterm labor, fetal jeopardy due to a possible decrease in uterine blood flow or due to intrauterine injury. While the above authors have approached the diaphragmatic hernia in an open surgical manner, laparoscopic method has also been attempted successfully in pregnancy in the second trimester and even at 27 weeks of gestation.[9,10] Julien et al successfully repaired diaphragmatic hernia in pregnant patient laparoscopically using a novel right lateral position.[11] Chen et al  reported a case of simultaneous repair of irreducible hernia along with cesarean section.[3] Obstetric management of maternal diaphragmatic hernia continues to be debatable. Dumont had advocated to avoid bearing down and vaginal delivery. However, use of forceps application to avoid intra-abdominal pressure was advocated.[4] On the contrary, Genc et al had achieved successful outcome after induction of labor at 39 weeks.[8] Being a rare scenario, no controlled studies can be conducted and only literature reviews can help in the matter. In our case, patient went into spontaneous precipitate labor and delivered successfully as she was a case of pseudo diaphragmatic hernia. It is suggested that all such cases be reported so that some consensus would emerge regarding the best approach to management. 

References 
  1. Pansky B. Development of The Diaphragm. Review of MEDICAL EMBRYOLOGY. New Jersey, United States: Prentice Hall (a Pearson Education Company). Macmillan USA. 1982; pg 48.
  2. Pober BR, Russell MK, Ackerman KG. Congenital Diaphragmatic Hernia Overview. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, et al, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle 2006;1993-2017.
  3. Chen Y, Bai J, Guo Y, Zhang G. The simultaneous repair of an Irreducible Diaphragmatic Hernia while carrying out a Cesarean Section. Int J Surg Case Rep; 2013;4(9):771–2.
  4. Dumont M. Diaphragmatic hernia and pregnancy. J Gynecol Obstet Biol Reprod. 1990; 19(4):395–9.
  5. Ting JYS. Difficult diagnosis in the emergency department: Hyperemesis in early trimester pregnancy because of incarcerated maternal diaphragmatic hernia. Emerg Med Australas 2008; 20(5):441–3.
  6. Eglinton T, Coulter GN, Bagshaw P, Cross L. Diaphragmatic hernias complicating pregnancy. ANZ J Surg. 2006;76(7):553-7.
  7. Ngai I, Sheen JJ, Govindappagari S, Garry DJ. Bochdalek hernia in pregnancy. BMJ Case Rep. 2012;2012. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22967686
  8. Genc MR, Clancy TE, Ferzoco SJ, Norwitz E. Maternal congenital diaphragmatic hernia complicating pregnancy. Obstet Gynecol. 2003;102(5 Pt 2):1194–6.
  9. Palanivelu C, Rangarajan M, Senthilkumaran S, Parthasarathi R. Safety and efficacy of laparoscopic surgery in pregnancy: experience of a single institution. J Laparoendosc Adv Surg Tech A. 2007; 17(2):186–190
  10. Wieman E, Pollock G, Moore BT, Serrone R. Symptomatic Right-Sided Diaphragmatic Hernia in the Third Trimester of Pregnancy. J Soc Laparoendosc Surg. 2013; 17(2):358–60
  11. Julien F, Drolet S, Lévesque I, Bouchard A. The right lateral position for laparoscopic diaphragmatic hernia repair in pregnancy: technique and review of the literature. J Laparoendosc Adv Surg Tech. 2011;21(1):67–70.
Citation

Bora A, Madhva Prasad S, Gupta AS. Successful Pregnancy Outcome with Pseudo Diaphragmatic Hernia in the Gravida. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/successful-pregnancy-outcome-with.html

Cephalocentesis In Fetal Hydrocephalus – An Alternative To Cesarean Section For Obstructed Labor

Author Information

Shah S*, Mayadeo NM**.
(* Second Year Resident, ** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

With the availability of imaging modalities at most institutions, there is still a place of destructive procedure in few selected cases. The authors present a case of ultrasound guided transabdominal cephalocentesis in a fetus with gross hydrocephalus and myelomeningocele. This fetus was delivered vaginally and thereby avoided cesarean section for a baby which had poor prognosis and ensured a better obstetric future and morbidity outcome.

Introduction

Cephalocentesis is a procedure by which the excess cerebrospinal fluid is drained from a fetal skull with hydrocephalus thereby permitting vaginal delivery. It is a rare procedure offered to pregnant patients having a hydrocephalic fetus which is expected to have a poor prognosis after delivery, such as a still birth or an early neonatal death.[1]

Case Report

A 30 year old primigravida, with history of infertility treatment for past 10 years was registered at our hospital at 13 weeks of gestation. Present pregnancy was a spontaneous conception. At 21 weeks of gestation she was diagnosed to have a malformed fetus. Ultrasound report was suggestive of moderate degree of hydrocephalus with Arnold Chiari type II malformation and a lumbar meningomyelocele. The prognosis was explained to the patient and relatives. Patient was lost to follow up thereafter. At 36 weeks of gestation patient presented to our emergency department in active labor. On examination she was hemodynamically stable. On per abdomen examination uterus was full term with breech presentation. Fetal heart sounds were present 140 beats per minute, regular, and uterine activity was 1/10/10-15. On per vaginum examination membranes were absent, cervix was 3cm dilated 40% effaced with buttocks as presenting part at -2 station. Urgent ultrasound was repeated for biparietal diameter and head circumference. USG report was suggestive of single live intrauterine gestation with breech presentation and BPD of 10.6 cm and head circumference of 41.1cm. Patient and relatives requested a vaginal trial. Decision was made to do a cephalocentesis. A liberal episiotomy was taken and the breech was delivered in the labor room. Cephalocentesis was attempted per vaginum which failed (figure 1).


Figure 1: Attempted cephalocentesis per vaginum using spinal needle.

Hence decision was made to perform ultrasound guided transabdominal cephalocentesis. Ultrasound probe was used to visualize the fetal skull and brain. An 18 guage spinal needle was inserted transabdominally under vision into the occipital/posterior horn of lateral ventricle near lambdoid sutures away from placenta and 250 ml cerebrospinal fluid was aspirated taking care not to damage any maternal tissue (figure 2 and figure 3).


Figure 2. USG guided transabdominal cephalocentesis.


Figure 3. USG showing needle placement.

The delivery was uneventful and a fresh still born male of 2.460 kg was delivered. The cervix was traced; there was no evidence of any cervical tear. Cesarean section along with its complications was hence avoided in a primigravida with a fetus which had poor prognosis post delivery.

Discussion

Arnold Chiari type II malformations have an incidence of 1:1000 total live births.[2] Studies have shown that this condition has poor prognosis and it is a life threatening abnormality. In survivors, 15% of newborns die by the age of 3 years and one third have permanent neurological disability.[3] If diagnosed before 20 weeks of gestation, as it involves serious associated malformations which are incompatible with life then termination of pregnancy is an option.
After 20 weeks of gestation fetal surgical procedures can be offered. These include intrauterine fetal surgery for repair of myelomeningocele either by open or an endoscopic surgery. These surgeries require expertise and resources which are not available in developing countries.[4]
At term elective cesarean delivery can be offered to patients in whom fetal malformations are likely to cause dystocia or where immediate neonatal surgery will result in better neonatal outcome. Hydrocephalus is the most common congenital malformation that can lead to obstructed labor. Obstructed labor can be managed either by cesarean section or an instrumental delivery. Cesarean section as compared to vaginal delivery has a increased risk of hemorrhage and infection. Unnecessary cesarean uterine scar is given to the patient, and hence in her successive pregnancies she is a high risk patient with a previously scarred uterus. In a malformed fetus where the poor prognosis has been explained, vaginal delivery can be done by vaginal cephalocentesis if it has a vertex presentation and transabdominal cephalocentesis in case of breech presentation as it is in our present case. In cephalocentesis a spinal needle is inserted in the uterus under ultrasound guidance and then advance further into the cerebral space to drain the excess CSF which will eventually reduce the size of head after which it is possible to conduct a successful vaginal delivery.[1]

References
  1. Chasen S, Chervenak FA, McCullough LB. The role of cephalocentesis in modern obstetrics. Am J Obstet Gynecol 2001; 185(3): 734-6.
  2. Gollop TR, Eigier O. Early prenatal ultrasound diagnosis of fetal hydrocephalus. Rev Bras Genet 1987; 3: 575-80.
  3. McLone DG. Continuing concepts in the management of spina bifida. Pediatr Neurosurg. 1992;18(5-6):254-6.
  4. Kahn L, Mbabuike N, Valle- Giler EP, Garces J, Moore RC, Hilaire HS, et al. Fetal Surgery: The ochsner experience with in utero spina bifida repair. Oschner J. 2014; 14(1):112-8.
Citation

Shah S, Mayadeo NM. Cephalocentesis In Fetal Hydrocephalus – An Alternative To Cesarean Section For Obstructed Labor. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/cephalocentesis-in-fetal-hydrocephalus.html

Innovative Technique of Using 5 mm Laparoscopic Tenaculum Alongside Bipolar Saline Resectoscope in Hysteroscopic Myomectomy

Author Information

Shah NH*, Khadkikar R**, Paranjpe S***.
(* Director, ** Consulting Obstetrician and Gynecologist, Vardann Multispeciality Hospital, Mumbai, *** Director, Velankar Hospital and Paranjpe Maternity Home, Mumbai, India.)

Abstract

Hysteroscopic myomectomy is the procedure of choice nowadays in cases of symptomatic submucous fibroids causing infertility, recurrent reproductive loss or abnormal vaginal bleeding. Hysteroscopic resection of a large submucous fibroid has remained a challenging procedure. Need of using glycine as distension medium and possible thermal damage with monopolar resectoscope are two important disadvantages. We present here our innovative technique of complete removal of submucous fibroid with the combination of bipolar saline resectoscope and 5 mm laparoscopic tenaculum. With this technique, we completely removed intramural portion of the fibroid and retrieved the separated fibroid from the uterus in a patient with primary infertility.

Introduction

Uterine fibroids are the most common benign solid tumors found in the female genital tract with incidence of 20–25 % in women of reproductive age. They arise from the muscular layer of the uterus. They usually grow towards lower resistance, thus becoming subserous when they grow towards the abdominal cavity, or submucous when they grow inside uterine cavity (5– 10 % of fibroids). Submucous fibroids may present with menorrhagia, colicky dysmenorrhea (as the uterus tries to expel the fibroids), and are believed to cause reproductive failure. Furthermore, these types of fibroids can cause chronic endometritis; they may have a greater risk for malignant change. Obstetric complications may include preterm labor, abnormal presentation, postpartum hemorrhage and puerperal sepsis. Most commonly submucous fibroids are fundal, other sites being anterior, posterior or lateral uterine walls. Small fibroids may arise from the cornual regions, thus obstructing the tubal ostia. Some of them are located at the cervical canal occupying the part of vagina and presenting as fibroid polyps. Hysterectomy and excision by laparotomy were the only modalities used in the past for treatment of symptomatic submucous fibroids. Conservative surgery requires opening the uterine cavity which itself is one of the factors responsible for altering subsequent fertility. Moreover, pelvic adhesions can form hampering tubal or ovarian function. In future, this approach has obstetric repercussions in the form of need to perform cesarean section. Development of hysteroscopy has made submucous fibroids accessible and completely resectable without performing laparotomy.

Case Report

A 30 year old woman, married since two and half years presented with primary infertility and irregular menses. Her menstrual cycles were irregular since 2 years, at intervals of 15 to 20 days with heavy bleeding at times. She had severe dysmenorrhea. Hormonal profile was within normal limits. Husband’s semen analysis report was normal. On examination, mild pallor was noted. Per abdomen examination revealed normal findings. On per speculum examination, external cervical os was seen as partially opened, otherwise cervix and vagina were normal. On per vaginal examination, uterine size was approximately 8 to 10 weeks and tip of finger could be inserted through the cervix. Uterus was firm, mobile and globular in shape. Her hemoglobin was 9.5 gm % and WBC count was 10,700/ cm3. Other routine biochemical and serological investigations were normal. Ultrasonography showed a well-defined hypoechoic mass within the endometrium measuring 6.7 × 5.4 × 2.3 cm having a small vascular stalk suggestive of submucous fibroid. She underwent hysteroscopic myomectomy under general anesthesia. The cervix was dilated up to No 10 Hegar, and we used bipolar saline resectoscope with normal saline as distension medium; total volume of saline used was 2 liters.. Hysteroscopic finding was that of a type 1 submucous fibroid with attachment at the anterior uterine wall.


Figure 1. Hysteroscopic view of submucous fibroid. 

We used a combination technique wherein we resected more than 60 – 70 % of the fibroid with the bipolar saline resectoscope and when the uterine wall was approached, we introduced 5 mm laparoscopic tenaculum by the side of the hysteroscope to completely remove the intramural portion of submucous fibroid. With the same 5 mm laparoscopic tenaculum by the side of the hysteroscope, bits of the fibroid were removed from the uterine cavity. The total procedure of removing the myoma from its bed as well as its retrieval went smoothly, requiring about 15 minutes. No complications like infection, cervical laceration or hemorrhage occurred.


Figure 2. Fibroid being removed with bipolar saline resectoscope.


Figure 3. Five mm laparoscopic tenaculum by the side of the hysteroscope.


Figure 4. Intramural portion of the fibroid being removed with laparoscopic tenaculum.

Postoperative course was uneventful. Histopathology report confirmed fibroid.

Discussion

Early case reports in the literature date to the mid 60’s and the treatment of submucous fibroids cases have previously been blind dilatation and attempted removal with a ring forceps or a hysterectomy.[1] Verma et al reported ultrasound-guided removal of retained IUDs, as safe and it could be performed in an office setting. [2] Yazicioglu removed the broken tip of Karman canula hysteroscopically from the subvesical space.[3] Hysteroscopic myomectomy using bipolar resectoscope uses normal saline as distension medium instead of glycine which has to be used with monopolar energy. Glycine is a hypotonic fluid which if used in excess can lead to dangerous hyponatremia. This complication can be avoided by using bipolar saline resectoscope. Numerous published reports exist about complications due to glycine extravasation with a rate between 0.1- 2.5 %. [4] This can be a serious complication, especially in older patients or those with heart or renal disease.
Hysteroscopic myomectomy using resectoscope can cause cervical trauma and also damage the underlying and surrounding endometrium. This is avoided by using the 5 mm laparoscopic tenaculum as we approach the uterine wall, avoiding thermal damage to the endometrium. The remaining part of the fibroid can be twisted and removed completely from its capsule with the use of the same tenaculum causing less bleeding. This is of utmost importance in infertile patients as endometrial injury can be caused by the resectoscope while removing the base of the fibroid causing damage to the underlying and surrounding endometrium. Thus, a possibility of Ashermann syndrome is averted. Also it significantly reduces the remote possibility of perforation of the uterine fundus with consequent omental and bowel injury. Using a 5 mm laparoscopic tenaculum by the side of the hysteroscope helps to remove the separated bits smoothly.  Minor leakage occurs alongside the hysteroscope while using tenaculum but that does not compromise the distension or the vision. We are able to grasp the pieces of resected fibroid lying in the cavity with 5 mm laparoscopic tenaculum due to which they could be removed vertically with the least diameter. 5 mm laparoscopic tenaculum is the best instrument available as it ensures firm and continuous grip over the fibroid pieces. Myoma screw cannot be used as it is difficult to maneuver inside the uterine cavity and it cannot grasp small pieces of fibroid. Similarly ovum forceps is also not very useful as retrieval is incomplete, tiresome and it is not under direct vision. Previously the same authors have used the 5 mm laparoscopic tenaculum to retrieve the broken tip of metal suction canula.[5] To conclude, while removing a fibroid hysteroscopically, considering all the advantages, we find it best that most of the fibroid (approximately 70 %) can be removed with a bipolar saline resectoscope with ease. The remaining firmly attached part of the fibroid can be removed with the use of laparoscopic 5mm tenaculum passed alongside the hysteroscope. Also it can be done faster and complete removal is possible using less fluid for distension and lesser chances of pulmonary edema.

References 
  1. De Brux J, Palmer R, Ayoub-Despois H. Ossifications of the endometrium. Gynecol Obstet (Paris) 1956; 55(5): 494–504.
  2. Verma U, Astudillo-Davalos FE, Gerkowicz SA. Safe and cost-effective ultrasound guided removal of retained intrauterine device: our experience. Contraception. 2015; 92(1): 77-80.
  3. Yazicioglu HF, Yasar L, Dulger O. Hysteroscopic removal of a foreign body from the subvesical space. Int J Gynaecol Obstet. 2004; 86(1):48-9. 
  4. Hart R, Molnar G, Magos A. Long term follow up of hysteroscopic myomectomy assessed by survival analysis. Br J Obstet Gynaecol. 1999; 106(7):700–5.
  5. Shah NH, Shah VN, Paranjpe SH. Innovative Method Of Hysteroscopic Retrieval Of A Broken Metal Suction Cannula Tip During MTP. JPGO 2015. Volume 2 No. 12.
Citation

Shah NH, Khadkikar R, Paranjpe S. Innovative Technique of Using 5 mm Laparoscopic Tenaculum Alongside Bipolar Saline Resectoscope in Hysteroscopic Myomectomy. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/innovative-technique-of-using-5-mm.html

Mullerian Anomaly With Pregnancy

Author Information

Dhokia T*, Shende D**, Shah A***, Chauhan AR****.
(* Third Year Resident, ** Assistant Professor, *** Second Year Resident, **** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Mullerian duct anomalies (MDA) are a group of entities that result from the dysgenesis, defective vertical or lateral fusion, or resorption failure of the Mullerian (paramesonephric) ducts, with an incidence in the general population of 0.001–10%.[1] The theory of unidirectional caudal to cephalad fusion of the Mullerian ducts cannot explain the presence of a single unified uterine fundus with partial septum, two cervices and  a partial longitudinal vaginal septum. Such an anomaly reinforces the alternative bidirectional theory.[2] Most patients with this anomaly are reported to have infertility, dysmenorrhea and recurrent pregnancy loss. Few such cases have been described in literature; however we present a rare case with this anomaly that had a successful obstetric outcome.

Introduction

MDAs are a group of developmental disorders that involve the internal female reproductive tract. Fusion of Mullerian duct occurs at around 11-13 weeks’ of gestation in utero and this fusion and absorption is unidirectional from caudal to cephalad end as stated by Crosby and Hill in 1962.[3] The case described here is a Mullerian anomaly with partial septate uterus with double cervix and a partial longitudinal vaginal septum. Some cases may be asymptomatic whereas other cases in the past have presented with unilateral obstruction, pelvic mass, dysmenorrhea, infertility, poor reproductive outcome. In such anomalies, fetal survival rates ranges from 10% to 75%; premature birth rates range from 9% to 33%, and spontaneous abortion rates range from 26% to 94%.[4] Our case had a successful pregnancy outcome by cesarean section at 40 weeks of gestation with no significant intra or postpartum complications.

Case Report

A 22 year old, Gravida 2 Para 1, with one living issue, was admiited at 40 +1 week of gestation in early labor. She had a previous full term cesarean section in view of meconium stained amniotic fluid but no details were available. On examination, vital parameters were stable, on abdominal examination uterus was full term, cephalic presentation with FHS 140/min and activity of 1/10/10-15; Pfannensteil scar of previous LSCS was present. On vaginal examination a longitudinal septum was present in vagina, bicollis cervix present, and the right cervix was 2.5 cm dilated, 50% effaced, vertex presentation, station -2, membranes present. The left cervix admitted tip of the finger and was poorly effaced. In view of the vaginal septum, speculum examination was done; a partial longitudinal vaginal septum was visualized. 2 cervices were seen, one on each side of the septum, fetal head was seen through the right cervix. Patient was taken for emergency LSCS in view of Mullerian anomaly with previous LSCS. Permanent sterilization by bilateral tubectomy was also done.
Intraoperative findings were suggestive of smooth uterine contour (Figure 1) with partial uterine septum arising from the fundus of the uterus not reaching the isthmus (Figure 2). Fetus was present in longitudinal lie in the left half of the uterine cavity. Two cervical openings were seen. Both the fallopian tubes and ovaries were normal. No intraoperative or postoperative complications occurred and patient and baby were discharged on day 5.


Figure 1. Smooth uterine contour.


Figure 2. Partial uterine septum.

Discussion

Uterine development results from the fusion of the Mullerian ducts during the 11-13th weeks of embryonic life. Septal resorption follows thereafter, starting at any point of fusion and moving in either or both directions.[3] The presence of septate uterus with double cervix and longitudinal vaginal septum challenges this theory.  The case reported by us cannot be explained by this theory, because the presence of two cervixes and vaginas is suggestive of a failure of caudad fusion while a partial septate uterus indicates normal cephalad fusion with failure of septal resorption superiorly.
Our case can be explained by an alternative embryologic mechanism hypothesis which propounds that fusion of the Mullerian ducts begins at the isthmus of the uterus and then moves both cranially and caudally, simultaneously. Midline resorption also begins at the isthmus and first moves caudally, to unify the cervix and vagina, and later cephalad to resorb the uterine septum. The dual cervix and vagina present in our case could be explained by failure of fusion of ducts in the caudal direction, beginning at the uterine isthmus. The partial septate uterus with normal fundus would result from failure of septal resorption superiorly after normal fusion.
The anomaly in our case does not fit into the Mullerian classification systems described by American fertility Society. However as per the ESHRE/ ESGE classification the anomaly can be classified as U2a – partial septate uterus, C2 – double cervix, V1 – longitudinal non-obstructing vaginal septum [5].
Patients with this type of anomaly described in literature have presented with a variety of symptoms, including severe dysparuenia, dysmenorrhea, infertility and recurrent pregnancy loss; some have been asymptomatic. Diagnosis of such anomalies is made by careful examination, followed by imaging. MRI has been the best non-invasive method for diagnosis of Mullerian anomalies. Treatment is usually planned in symptomatic patients which may include resection of the vaginal septum. Hysteroscopic resection of uterine septum with laparoscopic assistance can be performed. Some patients may however require metroplasty.[6]

Conclusion

As Mullerian anomaly in our patient was incidental finding and patient opted for permanent sterilization method of tubectomy no further treatment of septum resection was required. Only symptomatic patient of Mullerian anomalies should be treated.

References
  1. Green LK, Harris RE. Uterine anomalies. Frequency of diagnosis and associated obstetric complications. Obstet Gynecol. 1976; 47(4): 427–9.
  2. Chang AS, Siegel CL, Moley KH, Ratts VS, Odem RR. Septate uterus with cervical duplication and longitudinal vaginal septum: a report of five new cases. Fertil Steril. 2004; 81(4):1133-6. 
  3. Crosby WM, Hill EC. Embryology of the Mullerian duct system. Review of present day theory. Obstet Gynecol 1962; 20:507-15.
  4. Junqueira B, Allen LM, Spitzer RF, Lucco KL, Babyn PS, Doria AS. Mullerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. RadioGraphics. 2009; 29(4):1085-1103.
  5. Grimbizis GF, Di Spiezio Sardo A, Saravelos SH, Gordts S, Exacoustos C, Van Schoubroeck D. The Thessaloniki ESHRE/ ESGE consensus on diagnosis of female genital anomalies. Gynecol Surg. 2016;13:1-16.
  6. Saygili-Yilmaz ES, Erman-Akar M, Bayar D, Yuksel B, Yilmaz Z. Septate uterus with a double cervix and longitudinal vaginal septum. J Reprod Med. 2004; 49(10): 833-6.
Citation

Dhokia T, Shende D, Shah A, Chauhan AR Mullerian Anomaly With Pregnancy. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/mullerian-anomaly-with-pregnancy.html

Angiomyolipoma Of Vulva

Author Information

Daruwale R*, Kale K**, Waghmare T***, Chauhan AR****.
(* Senior Registrar, ** Assistant Professor, **** Professor, Department of Obstetrics and Gynecology, *** Assistant Professor, Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

Angiomyolipomas (AMLs) are benign tumors composed of blood vessels, smooth muscle cells and fat cells. Most commonly found in the kidney, they are also located in the liver and less commonly in the fallopian tubes, ovaries, spermatic cord, palate and colon. They are strongly associated with the genetic disease tuberous sclerosis. Vulval angiomyolipomas are extremely rare and to the best of our knowledge, only one case has been reported in the literature till date.

Introduction

Angiomyolipomas(AMLs) are benign tumors composed of three main tissue elements, namely mature adipose tissue, thick walled blood vessels, and smooth muscles cells. AMLs are most commonly found in the kidney with an incidence of 0.13%.[1] Few cases of extra renal AML have been reported affecting the liver, retroperitoneum, colon, fallopian tubes, uterus, ovaries, vagina, penis and abdominal wall.[2] We present a rare case report of vulval angiomyolipoma diagnosed on histopathological examination.

Case Report

A 38 year old female patient para 2, living 2 came with complaint of swelling in the perineal region since 3 months. The swelling was insidious and progressive, and intermittently painful. She had regular menstrual cycles with no menstrual complaints. Per-abdomen examination revealed no abnormality. Local examination of the vulva revealed 5 x 4 cm ovoid swelling on the right side between upper two third and lower one third of labia minora, approximately 2 cm above the fourchette. It was soft in consistency and minimally tender. Per-speculum examination revealed healthy cervix and vagina. On per-vaginal examination, uterus was anteverted, slightly bulky with bilateral fornices free and non tender. All biochemical investigations were within normal limits. Pelvic ultrasound showed a normal sized anteverted uterus with normal endometrial thickness. Bilateral ovaries were normal in size and echotexture and no adnexal pathology was seen. Patient was managed conservatively with antibiotics and analgesics. Pain subsided but due to persistent and uncomfortable swelling, cyst excision was performed. The excised cyst was sent for histopathological examination.
Histopathology report showed non-encapsulated benign neoplasm composed of blood vessels, smooth muscle fibers with interdisposed benign adipose tissue. The blood vessel component showed thin to thick wall, small to medium sized blood vessels. The muscle fibers were arranged encircling the blood vessels forming sheets and at places forming vague fascicles. The fatty tissues component showed clusters of mature adipocytes interspersed in the muscle fibers and vessels. There was no evidence of necrosis, atypical mitosis or malignancy. Immunohistochemistry showed strong diffuse positivity for desmin and smooth muscle actin. The final diagnosis was angiomyolipoma of right side of vulva. Patient was advised CT abdomen and pelvis to look for lesions at other sites; however, she did not comply and was subsequently lost to follow up.


Figure 1.Hematoxylin –eosin stained slide (100X) shows mature adipose tissue intermixed with smooth muscle fibers.


Figure 2.Hematoxylin –eosin stained slide (400 X) shows many thin walled blood vessels with proliferating smooth muscle fibers and adipose tissue. It also shows scanty lymphocytic infiltrate.


Figure 3.Hematoxylin –eosin stained slide (400 X) shows intermixed proliferating blood vessels, smooth muscle fibers and adipose tissue.


Figure 4.Strong and diffuse positivity for desmin and smooth muscle actin on immunohistochemistry.

Discussion

In 1951, the term “angiomyolipoma” was first used by Morgan to describe a benign renal tumor composed of blood vessels, smooth muscle, and adipose tissue.[3] It has an overall incidence of 0.3-3%. Broadly, there are two types of AMLs: isolated AML and AMLs associated with tuberous sclerosis. Isolated angiomyolipoma occurs sporadically and accounts for 80% of the angiomyolipomas, whereas those associated with tuberous sclerosis are caused by mutations in TSC1 or TSC2 genes (which regulate cell growth and proliferation).[4,5]  The mean age at presentation of patients with isolated angiomyolipoma is 40 years. It is about 4 times more common in women than in men. Although most common in the kidney,  these tumors have been reported in the liver, retroperitoneum, colon, fallopian tubes, uterus, ovaries, vagina, penis and abdominal wall.[2]  A thorough search of literature revealed only one case report of isolated vulval AML.[6]
Angiomyolipomas are generally asymptomatic. Sometimes cases with retroperitoneal AML present with abdominal pain and increasing abdominal girth.[7] Renal and extra renal AMLs can present with complications like degenerative necrosis, hematoma or sudden torrential bleeding due to spontaneous rupture of vessels within, though very rare. Although considered benign, they have an uncertain malignant potential which is an area of active research.  
The diagnosis of AML is based on the combination of clinical history, imaging tests, and histological examination. Imaging studies are useful in cases of renal and extra-renal AMLs. On histopathological examination, the gross appearance of tumor is yellow to gray with sizes ranging from few centimeters to upto 20 cm. On microscopic examination, presence of adipocytes, blood vessels and smooth muscle components confirms the diagnosis of AML. The smooth muscle component is mainly comprised of spindle cells and epithelioid cells. In most cases, the tumors stain positive for desmin, muscle specific actin, and HMB45 which helps in accurate diagnosis.HMB45 may be absent in extra renal AML.[6]  AML may be confused with lipomatous variant of angiomyofibroblastoma (LAMF). However, on histopathology, these tumors show epithelioid cells in nests, which is not a feature of AML. Also, LAMF stain uniformly positive with vimentin, but only occasionally for desmin and very rarely for smooth muscle actin.[7,8]
Although angiomyolipomas are universally regarded as benign lesions, their malignant potential should not be underestimated. Tumors with features of hemorrhage, necrosis, local invasiveness and high mitotic activity are likely to have poor outcome. Management options for AMLs include conservative management and surgical excision. Surgical excision is considered for tumors that are symptomatic and those with size greater than 4 cm. If opting for conservative management, meticulous follow up is necessary owing to their unknown malignant potential.[7]

Conclusion

Angiomyolipoma though rare, should be considered as one of the differential diagnosis in patients presenting with vulval mass. Usually considered benign, more research is needed to determine their malignant potential.

References
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  2. da Silva AA, Carlos R, Contreras E, de Almeida OP, Lopes MA, Vargas PA. Angiomyolipoma of the upper lip: case report and review of the literature. Med Oral Patol Oral Cir Bucal. 2007; 12 (2): E101 4.
  3. Martignoni G, Pea M, Rigaud G, Manfrin E, Colato C, Zamboni G, et al. Renal angiomyolipoma with epithelioidsarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions. Am J SurgPathol. 2000; 24(6): 889-94.
  4. Bissler JJ, Henske EP. Renal Manifestations of Tuberous Sclerosis Complex. In: Kwiatkowski DJ, Wiittlemore DJ, Thiele EA, Editors. Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics. Wiley-VCH Verlag GmbH; 2010. pp. 321–325.  
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  6. Garg M, Duhan A, Bindroo S, Kaur J, Mahajan NC. Isolated angiomyolipoma of vulva: A case report of an uncommon tumor at an uncommon site. J Cancer Res Ther 2015; 11(3): 645.
  7. Matsukuma S,  Koga A, Suematsu R,  Takeo H,  Sato K. Lipomatous angiomyofibroblastoma of the vulva: A case report and review of the literature. Mol Clin Oncol. 2017; 6(1): 83–7.
  8. Magro G, Righi A, Caltabiano R, Casorzo L, Michal M. Vulvovaginal angiomyofibroblastomas: morphologic, immunohistochemical, and fluorescence in situ hybridization analysis for deletion of 13q14 region. Hum Pathol. 2014; 45(8): 1647-55.
Citation

Daruwale R, Kale K, Waghmare T, Chauhan AR. Angiomyolipoma Of Vulva. JPGO 2017. Volume 4 No.3. Available from: http://www.jpgo.org/2017/03/angiomyolipoma-of-vulva.html