Volume 3 Issue 1, January 2016

Editorial

Parulekar SV

I am happy that JPGO has successfully completed two years of publication. It has been possible only because of the enthusiasm and efforts of the authors and the encouragement received from all over the world. In fact, JPGO gets more readers from countries other than the country of its origin and publication. Maintaining the quality and regularity of publication was not easy in face of self funding and entirely voluntary work of the editors and reviewers. I am happy that we achieved what we set out to do two years ago. I am also satisfied that we could share the clinical experience, innovations and original ideas of our contributors with the world without asking anyone to pay for it.

Urinary stress incontinence (SUI) is a condition in which there is an involuntary loss of urine on Valsalva’ maneuver in the form of effort, physical exertion, sneezing, or coughing. It  is a far more common problem in the lives of women than meets the eye. More than 50% of all women are affected by urinary incontinence at some time in their lives. At any given time it affects about 15% of all adult women. Among the women affected by SUI, about 75% have bothersome symptoms, and of these women, about 30% have moderate to extremely severe symptoms. The effects of SUI on a woman’s life can be varied. The smell of urine around her can cause a disruption of social relationships. The anxiety, embarrassment, and lowered self-esteem associated with SUI can cause significant psychological distress. There can be disruption of sexual life due to urinary incontinence occurring during sexual activity. Skin ulceration and urinary infection associated with severe SUI may necessitate hospitalization for management. Many cases go unreported and untreated because of the women’s embarrassment to discuss the issue and failure of the treating physicians to ask specific questions aimed at detecting SUI.


There are different pathophysiological mechanisms that cause SUI. Weakening of the pelvic fascial support under the urethra is the commonest cause. It may be due to childbirth trauma to the fascia or weakening due to postmenopausal atrophy. Childbirth trauma can also cause damage to the pudendal nerve, weakening the muscular component of the support. In such cases there is no resistance to downward displacement of the urethra with Valsalva’s maneuver, intraabdominal pressure gets transmitted to the bladder but not the urethra which has been displaced below the level of the urogenital diaphragm, intravesical pressure exceeds intraurethral pressure and SUI occurs. Another cause of SUI is a failure of the urethral sphincter to close properly as a result of a weak striated muscles around the bladder neck. In a case of SUI of any etiology, the symptoms may be masked if the woman develops pelvic organ prolapse (POP) later on. If the prolapse if of long duration, she may forget that she had SUI. If the gynecologist does not ask her about a history of SUI in past, the condition remains undetected, and will manifest after surgical repair of the POP. This is entirely unavoidable. In this issue we have an article on SUI, in which a so far unreported cause of development of SUI – a vaginal wall cyst -  which caused manifestation of SUI after surgery, is discussed. I hope it helps prevent postoperative SUI in some women who suffer from such a condition.

Large Cervical Fibroid With Cardiomyopathy: A Challenge

Author Information

Agarwal S*, Warke HS**, Satia MN***.
(* Third Year Resident, ** Associate Professor, Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

We present a case report of a 45 year old female with a large cervical fibroid in a known case of dilated cardiomyopathy with cardiac arrhythmias with an ejection fraction of 30 % who presented with severe anemia due to menorrhagia. Prior to surgery the patient underwent uterine artery embolization to decrease the vascularity of the large cervical fibroid in view of suspected large intraoperative hemorrhage. She was planned for surgery within 48 hours of the procedure but developed persistent ventricular premature beats on the day of surgery for which surgery was deferred after cardiac opinion. She presented 2 weeks later with a prolapsed fibroid through the vagina with necrosis and infection for which antibiotics were started. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and had an uneventful postoperative recovery.

Introduction

Dilated cardiomyopathy is a group of diseases that primarily affects the myocardium. It is the most common form of non-ischemic cardiomyopathy.[1] It has a prevalence of 36 per 100000 and is commonly seen between 20 and 60 years of age.[2] Dilated cardiomyopathy may cause congestive cardiac failure in about one in three cases.[1] There is dilatation of a part of myocardium, the etiology of which is unclear.

Case Report

A 45 year old woman post-menopausal since 1 year had come to our outpatient department (OPD) with complaints of lump in the abdomen which had increased to the present size gradually over 2 years with excessive bleeding per vaginum since 3 days. On examination she was extremely pale with pulse rate of 110/min, blood pressure of 100/60 mm Hg and 2x2 cm thyroid nodule with a 32-34 weeks’ size mass arising from the pelvis, hard in consistency with restricted mobility. Her Hb was 6 g/dl, PCV of 21 with hypochromasia and microcytosis. Ultrasonography was suggestive of multiple uterine fibroids, intramural and submucosal in location with largest anterior wall fibroid measuring 18x15x15 cm. She was a case of trivial tricuspid regurgitation, trivial mitral regurgitation and global hypokinesia with ejection fraction of 30% with dilated cardiomyopathy. She was started on intravenous tranexamic acid and was given 5 units of packed cell volume transfusion. CT scan revealed multiple uterine fibroids, largest measuring 18x15x15 cm arising from anterior lower uterine segment compressing the sigmoid colon, lower ureters and bladder bilaterally with mild hydronephrosis on both sides with well-defined fat planes. Serial ECGs were suggestive of ventricular bigeminy with ventricular premature beats. Chest radiograph indicated mild cardiomegaly with bilateral prominent hilar shadows suggestive of pulmonary hypertension. Her serum calcium was 6.9 mg/dl and hypocalcemia was treated with 20 ml of 20% calcium gluconate followed by oral calcium supplements. Cardiac stress test was done which showed ill sustained monomorphic ventricular tachycardia in stage 1 with recovery in stage 2 with return to baseline sinus rhythm in stage 3 suggestive of need of electrophysiological studies followed by radiofrequency ablation. Cardiac MRI was suggestive of dilated non infiltrative cardiomyopathy. USG thyroid was suggestive of enlarged thyroid gland with a benign thyroid nodule in the right lobe. Her serum TSH was 2.71 Miu/L  on tablet Thyronorm 50 μg OD .Endocrine opinion was taken and the drug was continued.
Bilateral uterine artery embolization was done with fine polyvinyl alcohol 300 particles so as to reduce intraoperative blood loss. Excessive blood loss in this patient with an ejection fraction of 30% would be life threatening. She was posted for total abdominal hysterectomy with bilateral salpingo-oophorectomy with moderate cardiac risk. She had persistent ventricular premature beats on operation table which did not subside.  Serum electrolytes sent were within normal limits. After discussion with anesthetist and cardiologist surgery was deferred. She was started on oral frusemide 20 mg 1-1-0, carvedilol 3.125 mg1-0-1, ramipril 2.5 mg 0-0-1 and spironolactone 25 mg 0-1-0 and cardiologists advised to postpone surgery for 4 weeks. Patient was started on tablet medroxyprogesterone acetate 10 mg tds so as to control vaginal bleeding and was discharged. She presented 2 weeks later in our out patients department with heavy bleeding per vaginum and a prolapsed mass outside the introitus. On examination vital parameters were stable with a 30 weeks’ mass arising from pelvis and a 10x10 cm fleshy mass extruding from the introitus with necrosis and foul smell.  This probably was due to necrosis of the fibroid following the procedure of uterine artery embolization.
 Intravenous ceftriaxone, metronidazole and analgesics were started. Swab for culture sensitivity was sent from fleshy mass which showed Pseudomonas aeruginosa sensitive to levofloxacin and polymyxin B. Intravenous levofloxacin and polymyxin B ointment was given for local application over the mass. She was posted for surgery with consent for requirement of intraoperative and perioperative intensive cardiac care and ventilator.



Figure 1: Sloughed off necrosed fibroid outside the introitus.

Intraoperatively uterus was 30 weeks’ size with multiple fibroids. There was a large cervical fibroid of size 22x20 cm. Bilateral fallopian tubes were edematous and bilateral ovaries were normal. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done under general and epidural anesthesia. Foul smelling pus was present at the base of the cervical fibroid which was sent for culture and sensitivity.


Figure 2: Large cervical fibroid of 18x15x15 cm.

Intraoperatively and postoperatively her vital parameters were stable. Two units packed cell volume transfusion was given intra-operatively. Swab sent for antibiotic culture sensitivity suggested Escherichia coli sensitive to levofloxacin which was continued along with injectable ceftriaxone, metronidazole and gentamicin postoperatively. She was discharged on day 10 of surgery after suture removal. The scar site was healthy with no pain, discharge and induration. Histopathology report was suggestive of benign leiomyoma with hyaline changes. She was advised to follow up in cardiology OPD for electrophysiological studies and further management.

Discussion

20-30% women in reproductive age group have leiomyoma uteri, the usual anatomical location being the body of the uterus and only 1-2% are confined to the cervix.[3] These women often present with an abdominal mass without any other symptoms, retention of urine, menstrual abnormalities, constipation or may mimic an ovarian tumor.[4] Our patient presented with a 32 weeks’ size mass with heavy bleeding per vaginum. She was a case of dilated cardiomyopathy with global hypokinesia with ejection fraction of only 30% with ECG changes showing ventricular bigeminy with ventricular premature beats. Dilated cardiomyopathy is characterized by progressive cardiac dilatation leading to impaired ventricular function.[1] There is global decrease in myocardial contractility which reduces left ventricular ejection fraction. According to Frank–Starling relationship, initially compensation occurs with enlargement of the left ventricular cavity leading to an increase in stroke volume with associated increase in the force of contraction.[1] Eventually these compensatory mechanisms fail, cardiac output decreases, resulting in left ventricular failure. In severe cases, there is combined systolic and diastolic dysfunction with impaired relaxation and elevated left ventricular end-diastolic pressures (LVEDP). This can lead to congestive cardiac failure and arrhythmias if not managed optimally. Medical therapy with various drugs used as disease modifying agents includes angiotensin-converting enzyme inhibitors (ACEIs), angiotensin II inhibitors and diuretics. Other drugs used are β-blockers, spironolactone, digoxin, biventricular pacing, and anticoagulants.
Our patient was started preoperatively with oral frusemide 20 mg 1-1-0, carvedilol 3.125 mg 1-0-1, ramipril 2.5 mg 0-0-1 and spironolactone 25 mg 0-1-0 to improve the postoperative outcome. Optimal management with these drugs can reduce the chances of development of arrhythmias and worsening of cardiac function. From anesthesia point of view the main aim was to prevent hypotension so as to avoid myocardial hypoperfusion. The goals of anesthesia are to avoid tachycardia, minimize the effects of negative inotropic agents, especially anesthetic drugs, to prevent increases in afterload and to maintain adequate preload in the presence of elevated LVEDP.
Preoperatively uterine artery embolization (UAE) was done to decrease the vascularity of the fibroid. It is a minimally invasive technique in which small polyvinyl alcohol (PVA) particles are used to occlude the blood supply to the fibroids. These particles stick in the lumen of the blood vessels forming a clot, thus occluding the blood supply. Eventually the fibroids reduce in size, and the symptoms get reduced or disappear. Preoperative embolization of the uterine arteries is also useful to decrease perioperative bleeding complications. It causes ischemia and necrosis of fibroids while preserving the uterus. UAE is technically successful in 95-99% of patients. It is successful in controlling menorrhagia in 85-95%. Transcervical sloughing of the necrosed fibroid can occur in about 5% of patients. 20-25% of patients with dominant submucosal fibroids can undergo sloughing.[5] This may resolve with spontaneous passage of tissue or may require surgical evacuation of the uterus. Other adverse effects of UAE include septicemia resulting in multiple organ failure,[6] endometritis[7], uterine necrosis[8], misembolisation from microspheres of PVA particles leading to ovarian failure,[9] infertility, menopause, loss of menstruation. Post embolization syndrome may occur, leading to chronic pain, nausea, vomiting, malaise, fever, night sweats. There can be fibroid expulsion, vaginal discharge containing pus and blood with foul odor coming from the infected necrotic tissue which remains inside the uterus, unsuccessful fibroid expulsion with fibroid being trapped in the cervix causing infection requiring surgical removal. Our patient presented with fibroid expulsion with foul vaginal discharge with bleeding and infection 2 weeks after uterine artery embolization requiring surgical intervention after a course of antibiotics.
This was an extremely high risk case from surgical, medical and anesthesia point of view. There was an increased risk of excessive intraoperative hemorrhage with difficult surgical technique due to the large size and location of the cervical fibroid. The management of the case was a challenge to the anesthetist and cardiologist due the ejection fraction being 30% with ventricular arrhythmias. She was managed with a multidisciplinary approach with involvement of gynecologist, cardiologist and anesthetist working as a team.

References

  1. Stevenson LW, Loscalzo J. Cardiomyopathy and myocarditis. In Jameson JN, Kasper DL, Harrison TR, Braunwald E, Fauci AS, Hauser SL, Longo DL, editors. Harrison's principles of internal medicine. 18th ed. New York: McGraw-Hill Medical Publishing Division 2015; pp. 1951-1970.
  2. Schoen FJ, Mitchell RN. The Heart. In Robbins SL, Kumar V, Cotran RS, editors. Robbins basic pathology. 8th ed. Philadelphia: Saunders 2015; pp. 240-246.
  3. Bhatla N. Tumours of the corpus uteri. In Pratap K, Narendra M, editors. Jeffcoate’s Principles of Gynaecology. 7th ed. New Delhi: Jaypee brothers Medical publisher; 2008; pp. 487-502.
  4. Suneja A, Taneja A, Guleria K, Yadav P, Agarwal N. Incarcerated procidentia due to cervical fibroid: An unusual presentation. The Australian and New Zealand Journal of Obstetrics and  Gynaecology 2003;43:252-253.
  5. Radeleff B, Eiers M, Bellemann N, Ramsauer S, Rimbach S, Hans-Ulrich Kauczor, Goetz M. Richter GM. Expulsion of dominant submucosal fibroids after uterine artery embolization. European Journal of Radiology. 2010; 57–63.
  6. de Block S, de Bries C, Prinssen HM . Fatal Sepsis after Uterine Artery Embolization with Microspheres. Journal of Vascular and Interventional Radiology. 2003;14:779–783.
  7.  Rajan DK, Beecroft JR, Clark TW, Asch MR, Simons ME, Kachura JR, et al. Risk of intrauterine infectious complications after uterine artery embolization. J Vasc Interv Radiol. 2004 Dec;15(12):1415-21.
  8. Gabriel H, Pinto CM, Kumar M, Nikolaidis P, Miller FH, Weinrach DM et al, MRI detection of uterine necrosis after uterine artery embolization for fibroid. AJR Am J Roentgenol. 2004;183:733–736.
  9. Spies JB, Spector A, Roth AR, Baker CM, Mauro L, Murphy-Skrynarz K. Complications after uterine artery embolization for leiomyomas. Obstet Gynecol. 2002; 100: 873–880.
Citation

Agarwal S, Warke HS, Satia MN. Large Cervical Fibroid With Cardiomyopathy: A Challenge. JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/large-cervical-fibroid-with.html

Uteroovarian Ligament Leiomyoma

Author Information

Valvi D*, Parulekar SV**.
(* Assistant Professor, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G. S. Medical College and KEM Hospital, Mumbai, India.)

Abstract

Uterine leiomyomas are far more common than extrauterine leiomyomas. Of the latter, the uteroovarian ligament is one of the least common sites of development of a leiomyoma. We present an unusual case of uteroovarian ligament leiomyoma.

Introduction

Leiomyomas are benign smooth muscle tumors of the uterus. They can arise from extra-uterine structures attached to the uterus, such as the broad ligament, vagina, round ligament, uteroovarian ligament etc. Extrauterine leiomyomas are far less common than uterine leiomyomas. Of the extrauterine leiomyomas, are not as common as uterine fibroids. They may arise in the broad ligament or at other sites where smooth muscle exists. The uteroovarian ligament leiomyomas are amongst the least common of the extrauterine leiomyomas. We present an unusual case of a uteroovarian ligament leiomyoma and its surgical management.

Case Report

A 33 years old woman came to our outpatient clinic  for treatment of primary infertility and uterine leiomyoma. She was married and cohabiting since 12 years but did not conceive. Her menstrual history was normal. She had been diagnosed to have a leiomyoma of the uterus by another clinician elsewhere. Her medical and surgical history was not contributory. General and systemic examination revealed no abnormality. On gynecological examination; uterus was of 10 weeks' size, retroverted, cochleate, displaced to forward and left side, and with a nodular posterior surface.  Approximately 7× 6 cm firm fibroid was felt on right lateral wall of uterus. A diagnostic hystero-laparoscopy done 7 years ago showed normal findings. Her husband's semen analysis was normal.  Her hemogram, plasma sugar levels, thyroid, liver and renal function tests, serological tests for HIV, hepatitis B, and syphilis, and serum levels of FSH, LH, and prolactin were normal.  Her follicular study showed follicular development and spontaneous rupture indicating ovulation.  Ultrasonography of pelvis showed a leiomyoma measuring 7.6× 7.1 cm in right lateral wall of the uterus.  As per the patient's desire and clinical findings, a decision to perform a myomectomy was made.  On exploration, approximately 7× 8cm degenerated leiomyoma with scattered calcification on the surface was seen arising from right utero-ovarian ligament.  It was firmly adherent to the right half of the posterior wall of uterus, anterior wall and floor of the pouch of Douglas, and the rectum.  It had no pseuadocapsule. There was no plane of dissection and hence it could not be dissected off easily.  It was first separated from the ovary by division of the uteroovarian ligament between the ovary and the leiomyoma. Then its anterior surface was dissected free from above downwards. Then it was separated from the floor of the pouch of Douglas, passing backwards up to the rectum. This left about 1-2 cm wide raw area in the area dissected. The serosal edges of the raw portion on the back of the uterus could be approximated easily with a continuous suture of No. 1-0 polyglactin, the adjacent part of the broad ligament being used for that purpose.  The ovary was sutured to the upper part of this suture line for suspension as well as for covering the scar to prevent adhesions. There was a defect in rectal serosa 5 mm wide, whenre the leiomyoma had been dissected off. Rectal injury was ruled out by injection of air through a tube passed in through the anus, and filling the pouch of Douglas with saline to see if any air bubbles escaped through a possible rent. The defect in the peritoneum on floor of the pouch of Douglas and upper 0.5 cm of the anterior surface of the rectum was not sutured, as that would possibly kink the rectum. The abdomen was closed after leaving an intraperitoneal drain in the pouch of Douglas. The patient made an uneventful recovery. Histopathological examination of the rectum confirmed the diagnosis of a leiomyoma with degeneration.


Figure 1. Operative findings. The uterus (U), the right fallopian tube (RFT) and ovary (RO), and the leiomyoma (L) arising from the right uteroovarian ligament (between arrows) are seen.


Figure 2. Leiomyoma.

Discussion

Uterine leiomyomas are benign smooth muscle tumors, that usually arise within the myometrium.[1] They may remain within the myometrium, or may grow towards its external or internal surface to become subserous and submucous leiomyomas respectively. Sometimes they arise from structures attached to the uterus, such as the broad ligaments, round ligaments, vagina, and the uteroovarian ligaments. Subserous pedunculated leiomyomas may become adherent to vascular structures like bowel and omentum and derive their blood supply from them.[1] When such leiomyomas lose their connection with the uterus, they become wandering leiomyomas. This occurs when their blood supply from the uterus diminishes and cannot sustain them. A similar occurrence is seen with leiomyomas which arise from relatively avascular structures like the round ligaments and uteroovarian ligaments.[2,3,4] The right uteroovarian ligament leiomyoma in the case presented probably outgrew its blood supply through the ligament. Hence it became adherent to the back of the uterus, the anterior wall and the floor of the pouch of Douglas, and rectum. A diagnosis of its origin could not be made preoperatively because it was firmly adherent to the uterus and mimicked intramural leiomyoma. It was considerably larger than the uterus, but was not responsible for the infertility of the woman. Since it had not developed from the myometrium, it did not have a pseudocapsule, and hence a plane could not be found to shell it out. It had degenerated as a result of reduction in its blood supply, and hence could not be dissected off easily. The first logical step was to separate it from the ovary. Then its anterior surface could be accessed for dissection. After its anterior surface was free, it could be separated from the floor of the pouch of Douglas, passing backwards up to the rectum. Thus the rectum could be protected during its separation from the leiomyoma. The serosal edges of the raw portion on the back of the uterus could be approximated easily. The ovary was sutured to the upper part of this suture line, covering it so as to prevent postoperative adhesions.[5] The defect in the peritoneum on floor of the pouch of Douglas and upper 0.5 cm of the anterior surface of the rectum was not sutured, as that would possibly kink the rectum. Placement of a drain locally ensured keeping the area dry and allow serosal healing.

References
  1. Breech LL, Rock JA. Leiomyomata uteri and myomectomy. In Rock JA, Jones HW III, editors. Te Linde’s Operative Gynecology. 10th ed. New Delhi: Wolters Kluwer Health – Lippincott Williams & Wilkins 2008; pp. 687-726.
  2. Yoldemir T, Atasayan K, Eraslan A. A giant extra-uterine fibroma originating from an utero-ovarian ligament initially diagnosed as an ovarian tumour. Marmara Medical Journal 2014; 27: 132-3 DOI: 10.5472/MMJ.2014.03084.1
  3. Buttram VC Jr, Reiter RC. Uterine leiomyomata: etiology, symptomatology, and management. Fertil Steril 1981; 36:433-45.
  4. Fasih N, Prasad Shanbhogue AK, Macdonald DB, et al. Leiomyomas beyond the uterus: Unusual locations, rare manifestations. RadioGraphics 2008; 28:1931-48.
  5. Parulekar SV. Practical Gynecology and Obstetrics. 5th ed. Mumbai: Vora Medical Publications; 2011.
Citation

Valvi D, Parulekar SV. Uteroovarian Ligament Leiomyoma. JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/uteroovarian-ligament-leiomyoma.html

Ischemic Uretero-Vaginal Fistula After Manchester- Fothergill’s Surgery

Author Information

Gupta AS
(Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

Urinary tract complications after various gynecology surgeries mainly hysterectomy are well documented. However, uretero vaginal fistula after Manchester - Fothergill’s (MF) repair for prolapse is not reported. This case of uretero vaginal fistula that developed 3 weeks after surgery reflects the dangers involved if the surgeon deviates from the recommended surgical steps.

Introduction

Manchester- Fothergill surgery is a conservative surgery for  uterine  prolapse . It comprises of amputation of the elongated cervix , shortening of the uterosacral mackenrodt’s liagments by their anterior fixation to the cervical stump, creation and dilatation of the neo cervix, anterior colporrhapy and posterior colpoperineorrhapy.[1]  Iatrogenic ureterovaginal fistulas have been reported in various gynecological surgeries. Hysterectomy mainly abdominal  and cesarean sections are the leading gynecology and obstetric reasons for these uretero vaginal fistulas. An incidence of 0.4 to 2.5% of ureterovaginal fistuals has been reported for gynecology surgeries that were performed for benign conditions.[2]

Case Report

A 35 year old G3P1L1A2, resident of Mumbai , presented to our OPD with vaginal discharge of fluid since a couple of days. She had undergone a Manchester - Fothergill’s repair for prolapse 3 weeks back for uterovaginal descent.  On presentation with vaginal fluid leak, her pre-operative  and operative case record’s were reviewed. The pre- operative notes had a mention of her having a right sided unicornuate uterus but there was no confirmatory report supporting the same.  A notification regarding no desire for further child bearing was present in her discharge summary. Her general and systemic examination records were all within normal range.  Her local examination findings prior to surgery that were documented showed that she had a normal appearing vagina but presence of infra vaginal elongation of the cervix, though the cervix appeared normal. Uterus and cervix were central. No deviation was present. She had no cystocoele but rectocoele was present. Bimanual pelvic examination found a less than normal size, anteverted, anteflexed uterus, and normal fornices. Her documented utero-cervical length was  4” and of that the cervical length was 2 and a 1/2’’. The infravaginal portion of the cervix measured 1 and a 1/2”. Her biochemical, serological, radiological investigations were well within normal range. She underwent Manchester- Fothergill’s repair with posterior colpoperineorrhaphy in her proliferative phase of her menstrual cycle. Surgery  notes were reviewed. Surgery was  done under combined spinal and epidural anesthesia. The salient points of surgery were as follows. Pre operative uterocervical length was confirmed. Internal Os was dilated upto 6.5 Hegar dilator. Vagina was reflected circumferentially from the portiovaginal cervix. Pouch of Douglas peritoneum was opened. Bilateral uterosacral, mackenrodt’s ligament complex was ligated and detached from the cervix. Bilateral descending cervical arteries were ligated. Suture material used was Polyglactin 910 throughout the surgery.  A sufficient portion of the portiovaginal part of the cervix was amputated.  Three Fothergill stitches were taken. The interesting part of the surgery was the performance of the Sturmdoff suture. Four Sturmdoff sutures were taken to refashion the cervix. One anterior, one posterior and two lateral, one on the left and one on the right. The patient was examined in the dorsal position with adequate light. Speculum examination showed the presence of the four Sturmdoff sutures. One anterior, one posterior and two lateral, one on the left and one on the right as mentioned by the surgeon. A trickle of fluid was seen from underneath the suture in the right fornix.


Figure 1. The post MF neocervix. The yellow arrow marks the external Os and the 3 gray arrows mark the right , posterior and the left dimples of the sturmdoff sutures.


Figure 2. The post MF neocervix. The artery points the uretero vaginal fistula and the leak.


Figure 3. The post MF neocervix. The artery has lifted the suture end of the right sturmdoff suture to show the fistula and leak coming from underneath the suture (yellow arrow).

It was odorless. There was no perineal excoriation or urine smell. The portiovaginal cervix was not even 1 cm in length. The cervix appeared flushed to the vagina. On redirect questioning patient had normal voiding sensation and she was passing adequate amount of urine through the urethra. A three swab methylene blue test was performed. None of the swabs turned blue. Upper most swab was found to be moist. The patient was admitted and evaluated for suspected uretero-vaginal fistula. An intravenous pyelography (IVP) was done. The left kidney and ureter were normal. There was right sided hydronephrosis and hydroureter. The right dilated ureter in its terminal portion before entering into the bladder had its continuity interrupted and there was presence of small quantity of radio opaque dye in the vagina, suggestive of right sided uretero vaginal fistula.


Figure 4. The 20 minute IVP. It shows the right hyrdoureter, hydro nephrosis, The terminal hydro ureter is marked by the yellow arrow and the pink arrow marks the dye in the vagina.


Figure 5.  The 45 minute IVP Film (Oblique film). The yellow arrows show the entry of the ureter in the bladder. Left yellow arrow shows entry of left normal ureter and the right arrow shows the dilated ureter that is not entering the urinary bladder (red hollow arrow).


Figure 6. The 1 hour IVP Film.


Figure 7. The Post IVP Film

The patient was seen by a urologist and a cystoscopy was done. An attempt at cannulating the right ureter was unsuccessful. She subsequently underwent an implantation of the right ureter (neoureterocystostomy) in the bladder 10 weeks after her primary surgery and a DJ stent was placed for 3 months. The DJ stent was removed after 12 weeks and there was no leak after the stent removal.

Discussion

Manchester- Fothergill surgery is a conservative surgery for young patients of utero vaginal prolapse who desire to retain their menstrual function. It is not the best surgery for women desiring child birth.  A pubmed, medline and a google search failed to bring up any reported ureterovaginal fistula following a Manchester- Fothergill surgery for uterine descent in english literature. Literature search highlighted the causes of uretero vaginal fistuals to be hysterectomy, post cesarean section, and obstructed labor.  Occurrence of ureterovaginal fistula was maximum after abdominal rather than with laparoscopic or vaginal hysterectomy.  It was least after vaginal hysterectomies. [3,4,5,6,7,8]
Various studies have evaluated the complications associated with this surgery. Alkis et al has   studied 49 cases who underwent Manchester- Fothergill surgery over a period of 5 years. Of these 49  one patient had bladder injury, one patient  developed recurrent prolapse and one patient had post operative urinary retention.[9] Ayhan et al analyzed the data of 204 women who had  Manchester- Fothergill surgery performed on them. He studied women operated between 1985 to 2004, a period of 19 years. None of them had any urological injuries. Urinary retention, cervical stenosis were the main complications.[10] Eighty one patients of MF were evaluated by De Boer, urinary retention was the commonest complication. There were no cases of urological injuries in their reported series.[11] Ninety eight patients studied by Thys and colleagues also reported no urological tract injuries.[12] All the above authors after analyzing data from over 450 patients have not reported a single uro vaginal fistula. There was only one patient with bladder injury. All the above studies concluded that the MF procedure for uterine prolapse is low on morbidity and mortality.
MF procedure consists of amputation of the elongated cervix , shortening of the uterosacral mackenrodt’s liagments by their anterior fixation to the cervical stump, creation and dilatation of the neo cervix, anterior colporrhapy and posterior colpoperineorrhapy. The neo amputated cervix is covered with sturmdoff sutures. Two sturmdoff sutures, one covering the anterior and another covering the posterior lip of the cervix is the norm. The surgery notes documented that four instead of two sturmdoff sutures were placed. One anterior, one posterior and two lateral. I believe that the right lateral sturmdoff suture went through the right ureter and caused ischemic necrosis resulting in the uretero-vaginal fistula.  The original cervical length was 2 and a 1/2’’ and the infravaginal portion of the cervix measured 1 and a 1/2”. It was observed that the portio vaginalis cervix was almost flushed to the fornix and barely 1 cm in length. The post surgery utero cervical length was 2”. This suggests that only half an inch of the cervix was left behind, excising the entire infra vaginal and half the supra vaginal portion of the cervix. The distance between the external and the internal Os was only half an inch.  The ureter that goes about 1 and a 1/2 cm below and lateral to the uterine artery at the level of the isthmus would now be directly susceptible to trauma in the lateral fornix. So while passing the lateral sturmdoff suture through the cervical canal and bringing it out laterally at the right lateral angle of the vagina, the surgeon possibly injured the ureter at this point due to generous amputation of the cervix. It is also possible to injure the uterine vessels and cause a retroperitoneal hemorrhage. Prior to performing the IVP, the possibility of an aberrant course of the right ureter being the cause for this trauma was entertained as one of her old papers mentioned a right unicornuate uterus. But after the IVP the normal course of the ureter was seen ruling out developmental reason for the ureteric trauma.
Surgeons, when they deviate from the  recommended steps have to realize that they expose the patients to morbidity and subject them to prolonged surgical, medical treatment, immense emotional and financial burdens. A procedure with inherently low rate of serious complication was converted into a highly morbid condition.

References
  1. Hopkins MP, Devine JB, DeLancey JOL. Uterine problems rediscovered after presumed hysterectomy: The Manchester operation revisited. Obstetrics & Gynecology. May 1997; 89 (5, part 2); Supplement : 846-848.
  2. Drake MJ, Noble JG. Ureteric trauma in gynecologic surgery. Int Urogynecol J Pelvic Floor Dysfunct. 1998;9(2):108-17.
  3. Demirci U, Fall M, Göthe S, Stranne J, Peeker R. Urovaginal fistula formation after gynaecological and obstetric surgical procedures: clinical experiences in a Scandinavian series.S cand J Urol. 2013 Apr;47(2):140-4. doi: 10.3109/00365599.2012.711772. Epub 2012 Aug 8.
  4. Murtaza B, Mahmood A, Niaz WA, Akmal M, Ahmad H, Saeed S. J Pak Med Assoc. 2012 Oct;62(10):999-1003.
  5. Shaw J, Tunitsky-Bitton E, Barber MD, Jelovsek JE. Ureterovaginal fistula: a case series. Int Urogynecol J. 2014 May;25(5):615-21. doi: 10.1007/s00192-013-2272-y. Epub 2013 Dec 18.
  6. Biswas A, Bal R, Alauddin M, Saha S, Kundu MK, Mondal P. Genital fistula--our experience. J Indian Med Assoc. 2007 Mar;105(3):123-6.
  7. Rafique M, Arif MH. Management of iatrogenic ureteric injuries associated with gynecological surgery. Int Urol Nephrol. 2002;34(1):31-5.
  8. Benchekroun A, Lachkar A, Soumana A, Farih MH, Belahnech Z, Marzouk M, et al. Uretero-vaginal fistulas. 45 cases. Ann Urol (Paris). 1998;32(5):295-9.
  9. Alkış I, Karaman E, Han A, Gülaç B, Ark HC.The outcome of Manchester-Fotergill operation for uterine decensus repair: a single center experience.Arch Gynecol Obstet.ﾧ 2014 Aug;290(2):309-14. doi: 10.1007/s00404-014-3200-1. Epub 2014 Mar 18
  10. Ayhan A, Esin S, Guven S, Salman C, Ozyuncu O. The Manchester operation for uterine prolapse. Int J Gynaecol Obstet. 2006 Mar;92(3):228-33. Epub 2006 Jan 20.
  11. De Boer TA, Milani AL, Kluivers KB, Withagen MIJ, and Vierhout ME. The effectiveness of surgical correction of uterine prolapse: cervical amputation with uterosacral ligament plication (modified Manchester) versus vaginal hysterectomy with high uterosacral ligament plication  Int Urogynecol J Pelvic Floor Dysfunct. 2009 Nov; 20(11): 1313–1319.
  12. Thys SD, Coolen A-L, Martens IR, Oosterbaan HP,  Roovers J-P WR , Mol B-W, et al. A comparison of long-term outcome between Manchester Fothergill and vaginal hysterectomy as treatment for uterine descent. International Urogynecology Journal September 2011, Volume 22, Issue 9, pp 1171-1178. 
Citation

Gupta AS. Ischemic Uretero-Vaginal Fistula After Manchester- Fothergill’s Surgery JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/ischemic-uretero-vaginal-fistula-after.html

A Case Of Unilateral Complete Absence Of Fallopian Tube And Ovary

Author Information

Maurya NR*, Gupta AS**.
(* Senior Registrar, ** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

A 32 yrs old multigravida with 39 weeks of gestation, while undergoing a cesarean section, was incidentally discovered to have unilateral complete agenesis of right fallopian tube and ovary. No other abnormal findings were present.  Unilateral absence of fallopian tube and ovary has rarely been reported in the literature The associations and etiology, developmental and otherwise are discussed.

Introduction

Unilateral absence of fallopian tube and ipsilateral ovary is a rare event with very few mentions in the literature. The etiology is usually auto amputation of the adnexa due to a silent, or sub-acute torsion with resorption of the necrortic adnexa or very rarely may be associated with a congenital malformation early in intrauterine life which coexists with a unicornutae uterus, ipsilateral renal malformations and also absence of the ipsilateral utero ovarian and round ligament.

Case Report

A 32 yrs old Gravida 4 Para 2 Living 1 NND 1 with 39 weeks of gestation was referred from a private hospital in view of deranged liver function tests for further management.  She had previous two vaginal deliveries. On abdominal examination, uterus was full term with increasing uterine contractions. There were no surgical scars either of laparotomy or laparoscopy on the anterior abdominal wall. Cervical internal Os was 3 cms dilated and after rupture of membranes moderate meconium stained liquor was seen. Emergency lower segment cesarean section was performed, delivering a 3.16 kg female child with 9/10 Apgar scores at birth. 
Intra-operatively, the uterus was eventerated. It was normal in size and shape. The left Fallopian tube and ovary were present. Surprisingly, the right sided Fallopian tube and ovary were absent. Both the round ligaments and infundibulo-pelvic ligaments were present.  No stump of the Right Fallopian tube or any utero-ovarian ligament attached to the uterus could be found. 
Figure 1 and 2. 
There was no ovary in the Right ovarian fossa. In the post operative period the patient was questioned regarding any previous episodes of abdominal pain either in childhood or adulthood that was conservatively managed. She was unable to recall any such episodes. Post operative abdominal ultrasonography showed normal kidneys. Patient had an uneventful postoperative period.


Figure 1: Posterior view of the uterus.


Figure 2: Right lateral view. Bald right lateral surface of the uterus.

Discussion

The uterus and the Fallopian tubes develop from the Mullerian ducts, while the ovaries are derived from the gonadal ridge [1]. Development starts in the embryo during the  6th week when the two Mullerian ducts converge towards the mid-line and meet. This results in the formation of the mid-line luminal structures, the uterus and usually the upper part of the vagina. Cranially, the Mullerian ducts form Fallopian tubes. Various Mullerian anomalies develop if this process is disrupted or altered [1].Even though the Fallopian tubes and ovaries are anatomically adjacent and functionally related, their embryological origins are diverse. Ovarian development is from the gonadal ridge. Developmental abnormalities that have been observed in the female genital tract more commonly include organs that have their embryological origin in the Mullerian ducts. The incidence of Mullerian anomalies in general population is 0.5% [2] while that of adnexal malformation is 1:11240 [3]. The absence of both Fallopian tube and ovary, thus, warrants further inquiry.
Unilateral absences  are usually asymptomatic. They are  incidentally detected during a laparoscopy or laparotomy performed for gynecological or obstetric conditions. The first such case was reported by Danreuther in 1923 [4].  Agenesis of both the ipsilateral ovary and Fallopian tube is rare but it has been reported and it is often associated with malformations of the uterus and/or urinary tract, including a unicornuate uterus and ipsilateral renal agenesis [5]. Simultaneous developmental disorder involving the Mullerian system and the genital ridge is very unlikely.  The true etiology of unilateral absence of ovary and/or Fallopian tube is yet to be elucidated. The most likely causes of ipsilateral ovarian and coexisting tubal absence are conjectured to be an asymptomatic torsion of the adnexa with consequent organ ischemia and reabsorption [6][7], or a defect in the development of the Mullerian and gonadal structures due to underlying vascular anomalies.[5] Torsion can occur at anytime after birth or may be even in the intrauterine life.

In our patient these findings were discovered after exteriorizing the uterus. Our patient did not have a unicornuate uterus nor were their any coexisting renal anomalies to suggest a developmental cause. Both the round ligaments and the utero ovarian ligaments were present in our patient. Embryogically, the primitive undifferentiated gonad is attached and supported to its upper and the lower pole with bands or gubernaculum and these bands subsequently form the suspensory ligament or the infundibulo pelvic ligament of the uterus and the utero ovarian ligament which then continues as the round ligament respectively. [8]. Presence of both these ligaments in our patient, absence of uterine and renal malformations indicate to the unlikely-hood of a developmental anomaly. It is more likely that our patient had an asymptomatic adnexal torsion and resorption during the past that went unnoticed.

Routine inspection of adnexal structures during all cesarean sections and laparotomies is recommended. Such patients may also be discovered during workup for infertility or chronic pelvic pain. Once diagnosed with the condition, the patient must be further investigated to find associated renal anomalies by ultrasonography, IVP and/or MRI. 

References
  1. Hoffman BL, Schorge JO, Schaffer JI, Halvorson LM, Bradshaw KD, Cunningham FG. Antomic disorders. Williams Gynecology. 2nd ed. New Delhi: McGraw Hill 2012: pp. 481-505.
  2. Nahum GG: uterine anomalies. How common are they, and what is their distribution among subtypes? J Reprod Med, 1998: 43(10):877.
  3. Sivanesaratnam, V. Unexplained unilateral absence of ovary and Fallopian tube. European Journal of Obstetrics, Gynecology, and Reproductive Biology. 1986:  22(1-2), 103–5.
  4. Alexander HD. True unicornuate uterus and total absence of left broad ligament, round ligament, salphinx, ovary, kidney and ureter. Can Med Assoc J. 1947;56:539.
  5. Chen B., Yang C., Sahebally Z., Jin H. Unilateral ovarian and Fallopian tube agenesis in an infertile patient with a normal uterus. Experimental and Therapeutic Medicine, 2014: 8(3), 831–835.
  6. Dahan M. H., Burney R., Lathi R. Congenital interruption of the ampullary portion of the Fallopian tube. Fertility and Sterility, 2006: 85(6), 1820–1.
  7. Gupta A, Parulekar SV. Silent autoamputation of an adnexa. Bom. Hosp. J. 1996:38(4),900-2.
Citation

Maurya NR, Gupta AS. A Case Of Unilateral Complete Absence Of Fallopian Tube And Ovary JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/a-case-of-unilateral-complete-absence.html

Urinary Stress Incontinence Due To Anterior vaginal Wall Cyst

Author Information

Parulekar SV
(Professor and Head, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India.)

Abstract

Urinary stress incontinence can be caused by loss of urethral support due to childbirth trauma, postmenopausal atrophy of connective tissue, and chronic stress on the pelvic floor by lifting heavy weights, chronic cough, constipation, or obesity. It may be due to pudendal neuropathy caused by childbirth trauma or ageing. A vaginal wall cyst. has not yet been reported to cause this condition. A first case of urinary stress incontinence caused by an anterior vaginal wall cyst is presented here.

Introduction

Urinary stress incontinence affects 4 to 35 percent of women.[1,2] It is caused by a lack of support under the bladder neck and urethra, such that the urethra is not compressed during Valsalva's maneuver. It may be due to anatomical conditions like childbirth trauma, postmenopausal atrophy of connective tissue, and chronic stress on the pelvic floor by lifting heavy weights, chronic cough, constipation, or obesity.[2,3] It may also be due to pudendal neuropathy caused by childbirth trauma or ageing.[2,4] A case of urinary stress incontinence caused by an anterior vaginal wall cyst is presented here. It is the first case of its type in the world literature.

Case Report

A 28 year old woman, gravida 2 para 2, presented with a complaint of something coming out per vaginum for two months. She had no history of lifting heavy weights, chronic cough, constipation, or obesity. She had no history of urinary stress incontinence in past. Her menstrual cycles were regular, every 28 days, painless, and with moderate flow. She had two full term normal deliveries which had been uneventful. Her last menstrual period had been one week ago. Her medical and surgical history was not contributory. There were no bowel complaints. Her general and systemic examination revealed no abnormality. A speculum examination of the lower genital tract showed the presence of an anterior vaginal wall cyst on the right side of the urethra and the bladder neck. It was 5 cm in diameter, nontender, soft and cystic. The cervix and vagina were normal. There was no genital prolapse or urinary stress incontinence. Bimanual pelvic examination showed normal sized uterus and no pelvic abnormality.

Surgical removal of the mass was done under spinal anesthesia. A number 14 Foley's catheter was passed into the urinary bladder through the urethra. A longitudinal incision was made on the anterior vagina over the undersurface of the cyst. The cyst was dissected away from the bladder and urethra, which were not traumatized in any way. Hemostasis was achieved by electrocoagulation. Redundant part of the vaginal walls was excised. The edges of the vagina were approximated with interrupted sutures of No. 1-0 polyglactin, occluding the dead space in the cavity left behind after excision of the cyst. The urinary catheter was removed after 24 hours. The patient made an uneventful recovery and was discharged after 3 days.

The patient came for a follow up examination after 15 days. She complained of urinary stress incontinence which had started two days after the operation. There were no symptoms suggestive of urinary tract infection. An examination showed the presence of urinary stress incontinence which was confirmed by Bonney's test. Sensations in the area of distribution of the pudendal nerve were normal. Postvoid residual urine volume was 10 ml. Her urinalysis was normal and microbiological test of urine showed no growth of any bacteria. She was counseled on the treatment options. She chose to perform Kegel's perineal exercise and not undergo any surgical treatment. She was well six months after the operation. Her symptom had decreased considerably, but not totally controlled. She opted to continue Kegel's perineal exercise.


Figure 1. Vaginal wall cyst below and to the right of the bladder and the urethra.


Figure 2. The collapsed cyst is held with Babcock's forceps during dissection.


Figure 3. Dead space in the bed of the cyst after its excision is being closed by approximation of fascia with interrupted sutures of No. 1-0 polyglactin.


Figure 4. The dead space in the bed of the cyst has been occluded. Redundant portion of the vagina has been excised.


Figure 5. Vaginal edges have been approximated with interrupted sutures of No. 1-0 polyglactin.

Discussion

Urinary continence is maintained when the intraurethral pressure remains higher than the intravesical pressure. If the two pressures equalize or intravesical pressure exceeds the intraurethral pressure, urinary incontinence occurs. During Valsalva's maneuver the urethra gets compressed against the vagina and subvaginal fascia, which maintains the intraurethral pressure. If this support is weakened by anatomical factors (e.g. by childbirth trauma, postmenopausal atrophy of connective tissue, and chronic stress on the pelvic floor by lifting heavy weights, chronic cough, constipation, or obesity.) or neurological damage to the muscles (e.g. with pudendal neuropathy caused by childbirth trauma or ageing), urinary stress incontinence can occur.[4,5]
In the case presented here, there was none of the factors listed above causing damage to the fascia between the vagina and the urethra, nor was there any neurological damage to the pudendal nerve. She had no urinary incontinence in the past. Her urinary incontinence manifested first time 2 days after the operative removal of the vaginal wall cyst, 1 day after removal of her urinary catheter. There had been no damage to the bladder neck, urethra, or the fascia under them. Thus it appears that she had fascial defect prior to the operation, and urinary stress incontinence manifested after removal of the vaginal wall cyst which was supporting the urethra till the time of the operation. It could not be anticipated as there have been no such cases reported in the world literature.
It is recommended that suburethral fascial buttressing be done after removal of large anterior vaginal wall cysts, if the fascia in that area appears to be deficient. Routine use of a transobturator tape insertion in such cases cannot be recommended until more cases of this type are reported.

References
  1. Abrams P, Cardozo L, Fall M, Griffiths D, Rosier P, Ulmsten U, et al. Standardisation Sub-committee of the International Continence Society. The standardisation of terminology of lower urinary tract function: report from the Standardisation Sub-committee of the International Continence Society. Neurourol Urodyn. 2002;21(2):167-78.
  2. Luber KM. The definition, prevalence, and risk factors for stress urinary incontinence. Rev Urol 2004; 6 Suppl 3:S3.
  3. DeLancey JO. Structural support of the urethra as it relates to stress urinary incontinence: the hammock hypothesis. Am J Obstet Gynecol 1994; 170:1713.
  4. American College of Obstetricians and Gynecologists. Urinary incontinence in women. Obstet Gynecol 2005; 105:1533.
  5. Rogers RG. Clinical practice. Urinary stress incontinence in women. N Engl J Med. 2008 Mar 6. 358(10):1029-36.
Citation

Parulekar SV. Urinary Stress Incontinence Due To Anterior vaginal Wall Cyst, JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/urinary-stress-incontinence-due-to.html

Darier’s Disease In Pregnancy

Authors Information

Jagtap V*, Samant P **, Parulekar SV***.
(*Third Year Resident, **Additional Professor, ***Professor and Head, Department of Obstetrics and Gynecology, Seth G. S. Medical college and KEM hospital, Mumbai, India)

Abstract

Darier’s disease (keratosis follicularis or Darier’s White disease) is a rare autosomal dominant disease.[1] We report a case of a 22 year old primigravida woman with full term pregnancy and Darier’s disease since childhood.

Introduction

Darier’s disease is a rare autosomal condition with variable penetrance. Its incidence is about 1 case in 30000 with worldwide distribution. Males and females are equally affected. Its onset is usually in adolescence.[1] The disease occurs due to mutation of ATP2A2 gene located on chromosome 12q.[2] This gene codes for sarcoplasmic ER Ca2+ ATPase protein which is important for maintaining desmosomal protein attachments.[3] Though prenatal diagnosis is possible, prenatal counseling is difficult due to variable penetrance and inability to predict severity of disease in an individual.[4]

Case Report

A 22 year old primigravida was registered with us antenatally. She was a known case of Darier’s disease since childhood. She had warty papular lesion with hyperkeratosis all over the body sparing axilla, perineum, nails and mucosae. She was following up regularly with a dermatologist and was not on any medications. The disease had no progression or regression during this pregnancy. Her medical and surgical history was not contributory. There was no family history of this illness. Her general and systemic examination revealed no abnormality. There was a single fetus in vertex presentation, whose growth was appropriate. Her biochemical, serological  and hematological tests showed normal results. Obstetric ultrasonography showed no abnormality. She did not go into spontaneous labor until 41 weeks. A nonstress test was reactive. Labor was induced. She developed intrapartum fetal distress, for which a cesarean section was done under spinal anesthesia. The newborn was normal. She made an uneventful recovery. Suture removal was done on postoperative day 14. The wound healed well. Thus our patient had a normal outcome. Follow up examination after 2 weeks and 3 months showed no change in the lesions. The lactation was successful.


Figure 1. Lesions on the lower back.


Figure 2. Lesions on the chest.


Figure 3. Lesions on the back.


Figure 4. Appearance of the healed wound of cesarean section.

Discussion

Darier’s disease is a rare condition affecting skin, nails and mucous membranes characterized by hyperkeratotic, greasy, warty papules/plaques on seborrhoeic areas of trunk and face and limbs. Crusted lesions may lead to painful fissuring. Nails and mucous membranes may also be involved.[5] Differential diagnosis includes acrokeratosis verruciformis, familial benign pemphigus (Hailey-Hailey Disease), seborrheic dermatitis, and transient acantholytic dermatosis (Grover’s disease).
Definitive diagnosis requires a skin biopsy. A skin biopsy is diagnostic. It shows dyskeratosis and acantholysis and corps ronds and grains.[5] On electron microscopy there is a loss of desmosomal protein attachments.[5] About 50% of the cases may have mood disorders. Such cases are prone to develop postnatal depression.
Association of mental retardation, epilepsy and schizophrenia has been reported. 30% cases may have depression and some may also have suicidal tendencies.[6] Obstetric problems associated with this condition are traumatic vaginal birth due to inelasticity of skin leading to traumatic vaginal birth, difficulty in giving regional anesthesia, cesarean scar related complications, infective folliculitis (group B streptococcus or MRSA or secondary viral infections), and an inability to breast feed the baby due to lesions over breast with or without infection. Bacterial skin infection of the mother may lead to neonatal sepsis. There is up to 50% risk of the offspring being affected due to the autosomal dominant pattern of. inheritance means that 50% of offspring. A woman with mild disease can give birth to a baby with severe disease. The patient and her spouse need to be counseled about the risk and offered prenatal diagnosis. In our case it could not be done because the facilities were not available.[7] Mild cases may be treated with moisturizers and sunscreen lotion. Severe cases are treated with topical/oral retinoids which are contraindicated in pregnancy. Topical steroids show poor efficacy in treating these lesions. Fusidic acid and 5% potassium permanganate solution are used for topical application. Antibiotics are used for secondary bacterial infections and acyclovir in case of herpes simplex virus infection.  Dermabrasion, laser ablation or surgical excision of lesions may be done if required.
 
References
  1. Cooper SM and Burge SM. Darier’s disease: epidemiology, pathophysiology, and management. Am J Clin Dermatol. 2003; 4:97-105.
  2. Sakuntabhai A, Ruiz-Perez V, Carter S, Jacobsen N, Burge S, Monk S, et al. Mutations in ATP2A2, encoding a Ca2+ pump, cause Darier disease. Nat Genet. 1999 Mar;21(3):271-7.
  3. Celli A1, Mackenzie DS, Zhai Y, Tu CL, Bikle DD, Holleran WM, et al. SERCA2-controlled Ca²+-dependent keratinocyte adhesion and differentiation is mediated via the sphingolipid pathway: a therapeutic target for Darier's disease. J Invest Dermatol. 2012 Apr;132(4):1188-95.
  4. Munro CS. The phenotype of Darier's disease: Penetrance and expressivity in adults and children. Br J Dermatol 1992;127: 126-30.
  5. Parker DC, Morris RJ, Solomon AR. Nonneoplastic Diseases of the Skin. In Mills, SE, editor 5th Edition Sternberg's Diagnostic Surgical Pathology. 2010 Lippincott Williams & Wilkins. Pg 17.
  6. Jacobsen NJ, Lyons I, Hoogendoorn B, Burge S, Kwok PY, O'Donovan MC, et al. ATP2A2 mutations in Darier's disease and their relationship to neuropsychiatric phenotypes. Hum Mol Genet. 1999 Sep. 8(9):1631-6.
  7. Shimizu H and Suzumori K. Prenatal diagnosis as a test for genodermatoses: its past, present and future. J Dermatol Sci. 1999; 19:1-8.
Citation

Jagtap V, Samant P, Parulekar SV. Darier’s Disease In Pregnancy. JPGO 2015. Volume 3 No. 1. Available from: http://www.jpgo.org/2016/01/dariers-disease-in-pregnancy.html