Volume 5 Number 8

Editorial
Gupta AS

Bilateral Gonadectomy In A Case Of Complete Androgen Insensitivity Syndrome
Swaminathan G, Parulekar SV.

Excision Of Collapsed Vaginal Wall Cyst With Old Rupture
Parulekar SV.

Successful Management Of Pregnancy Complicated With Guillain Barre Syndrome
Venkateswaran S, Hatkar PA, Prasad M.

Uterine Rupture And Colporrhexis After Forceps Delivery And Fundal Pressure
Nasare P, Joshi A, Pardeshi S, Gupta AS.

Good Pregnancy Outcome In A Case Of Previous B-Lynch Suture
Nasare P, Prasad M, Gupta AS.

Juvenile Cystic Adenomyoma Managed Laparoscopically
Shah N, Paranjpe SH, Puri J.

Termination Of Pregnancy In A Case Of Giant Leiomyoma
Ganapati T, Chaudhari HK.

Quadruplets with Acute fatty liver of pregnancy
Sikhawar R, Hatkar P, Desai G.

Remembering Past Greats: Francois Mauriceau
Prasad M


Editorial

Gupta AS

Maternal morbidity and mortality is a serious concern for both developed and developing countries. Post partum hemorrhage (PPH) is a major cause of maternal mortality and morbidity globally. About 15 to 25 % women in India are lost due to PPH.
Four ‘T’’s are commonly taught to the medical students to remember the types and the causes of PPH. These are “Tone”, “Trauma”, “Thrombin”, and “Tissue”. It is essential for the obstetrician to identify the high risk factors that predispose to the various types of PPH.
Trauma may be spontaneous or iatrogenic. Today I will be highlighting the iatrogenic cause of traumatic PPH mainly fundal pressure. Injuries to the birth canal can cause crippling debilities or even death of the fetus, mother or severe birth asphyxia leading to long term sequelae. Instrumental deliveries, application of fundal pressure, improper management of the delivery wherein large diameters are allowed to pass the birth canal, internal podalic version, extension of episiotomy are some of the iatrogenic reasons for trauma to the birth canal and traumatic PPH. Methods like high and mid forceps, cervical incisions (Duhrssen), vaginal birth of a breech by total extraction are no longer recommended.
Fundal pressure given in the second stage of labor synchronous with the uterine contraction and along with maternal bearing down efforts is a controversial issue. There is no scientific evidence to prove that it reduces the duration of labor or the need for instrumental or cesarean deliveries. Literature regarding it is scarce. It is only in the form of case reports, review articles but hardly any well designed prospective randomized controlled trials are available regarding its efficacy and safety. Serious adverse events after its use have been documented by few authors but probably that reporting is only the tip of the ice berg as most adverse events do not get reported due to apprehension of litigation. The use of this method is almost never documented in the case records. The use of this method is never discussed with the patient nor her explicit consent for the same obtained.
Adverse maternal outcomes like extension of episiotomy, 3rd and 4th degree perineal tears, colporrhexis, rupture uterus, increased pressure on the inferior vena cava causing maternal hypotension and serious fetal asphyxia, fractured ribs, liver injuries, abdominal soft tissue injuries and uterine inversion all have been attributed to this method by several authors.
Adverse fetal outcomes reported as caused by fundal pressure are non reassuring fetal heart rate, severe bradycardia due to excessive pressure on the fetal skull, raised fetal intracranial tension, severe birth asphyxia, intracranial hemorrhages, nerve palsy, and shoulder dystocia.
Proponents of this method should first of all counsel the woman for the same, obtain her consent, document its use, the direction in which the pressure was given, the number of times it was given, the duration of each fundal pressure, the maternal injuries, neonatal outcomes in detail should be all recorded. No documentation in the patients medical records suggest that the users are aware about its risks, and want to do something dangerous and not get caught.
Unless these methods are documented and well designed scientific studies are conducted and evidence proving its efficacy and safety is established this controversial method should not be used either alone or in conjunction with instrumental delivery.
We bring to our readers in this issue one such case of uterine rupture and colporrhexis caused by fundal pressure and forceps delivery used concomitantly.

Bilateral Gonadectomy In A Case Of Complete Androgen Insensitivity Syndrome

Author Information

Swaminathan G*, Parulekar SV**.
(* Specialty Medical Officer, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.)

Abstract

Androgen insensitivity syndrome(AIS), previously known as testicular feminization syndrome, is an X-linked recessive disorder of sexual development due to a mutation of androgen receptor coding gene at locus Xq11-12. It is the most common cause of male pseudohermaphroditism. Patients usually have a female phenotype with a karyotype of 46XY and primary amenorrhea. We present a case of 17 year old girl who presented with primary amenorrhea. She had sufficient breast development but scanty pubic and axillary hair. On gynecological examination, she had a blind vaginal pouch of length around 5 cm. On ultrasonography, the uterus was hypoplastic and bilateral around 3 cm size gonads were present. Her karyotype was 46XY. Laparoscopy was performed which revealed no internal genitalia except bilateral gonads. A small low transverse incision was taken and open Bilateral gonadectomy was done in our case.

Introduction

Androgen insensitivity syndrome, which was previously known as testicular feminization syndrome is an X-linked recessive disorder of sexual development due to a mutation of androgen receptor coding gene at locus Xq11- q12.[1] It is the most common cause of male pseudohermaphroditism. Patients usually have a female phenotype with a karyotype of 46XY and primary amenorrhea.[2[ The prevalence of this syndrome is estimated to be about 1 in 20000 births.[3] The complete form is a more common, occurring in 1 in 20,000 to 64,000 male births. The importance of this syndrome is the development of testicular tumors, especially seminomas after puberty.[1] Gonadal malignancies like yolk sac tumor, Sertoli cell tumor, unclassified sex cord stromal tumors and embryonic teratomas are rare in these patients.[1,4) The risk of the occurrence of gonadal tumors is rare before the age of 25.[5] The risk of developing testicular tumors is 3.6% at an age of 25 years and 33% at 50 years of age.[4] The diagnosis is often based on the absence of a cervix, uterus and fallopian tubes, presence of nondysgenetic testes and a vagina of variable length.[6] In this report we present and discuss a case of androgen insensitivity syndrome who underwent bilateral gonadectomy.

Case Report

A 17-year old girl  came to our outpatient department with the complaint of primary amenorrhea. Her height was 160 cm and weight 50 kg. Her breast development was Tanner stage 5. Pubic and axillary hair were scanty. She had no abnormal finding in her general and systemic examination. Gynecological examination showed normal perineum and vulva and a blind vaginal pouch of length around 5 cm. Rectal examination did not show any palpable uterus or pelvic mass. A transabdominal pelvic ultrasonography (USG) was suggestive of a hypoplastic uterus measuring 2.1x0.7x1.3 cm. Bilateral gonads were present measuring around 3 cm each, located in the position of ovaries. She had no other abnormal finding in her abdominal USG. The hormonal analysis were as follows: serum FSH:16.88 mIU/ml, LH:28.81 mIU/ml, prolactin 14.17 ng/ml, free testosterone 15 pg/ml which was consistent with normal male values. In cytogenetic examination, karyotype was determined to be 46XY. After preoperative preparations and anesthesia fitness, a diagnostic laparoscopy was performed under general anesthesia. Pelvic and abdominal inspection revealed the presence of bilateral gonads with some intervening tissue which could be the suspected hypoplastic uterus which was reported on ultrasonography. On the right side, the gonad was elongated and extending up to the lateral pelvic wall, passing very close to and under the appendix and cecum. Bilateral gonadectomy was done along with removal of intervening tissue, through a small abdominal infraumbilical incision. The patient made an uneventful recovery. The histopathologic report revealed a testicle on the left measuring 2.5x1.5x0.5 cm, with a cyst identified at one pole measuring 1.8x1.8 cm (possibly epididymal). The right testicle measured 3x2.5x0.5 cm. It had a tubular structure, possibly epididymis, measuring 1.5x1.5 cm. Both testes showed immature seminiferous tubules with immature Sertoli cells. There were prominent Leydig cell micronodules in the interstitium of the testes. No spermatogenesis seen. Histopathological diagnosis was bilateral gonads showing bilateral immature testis with Sertoli cells.


Figure 1. Left testis (T). G: Gubernaculum.


Figure 2. Relations of left testis: cecum (C), appendix (white arrow) and right testis (green arrows) are seen.


Figure 3. Right testis.


Figure 4. Surgical specimen.

Discussion

Androgen insensitivity syndrome is the most frequent cause of the male pseudohermaphrotidism and the third most frequent cause of primary amenorrhea (approximately 10% of the primary amenorrhea). [2,7] Three different types of AIS have been reported. [2,7] The three AIS phenotype classifications are: complete androgen insensitivity syndrome (CAIS), also called as testicular feminization syndrome, partial androgen insensitivity syndrome (PAIS), and mild androgen insensitivity syndrome (MAIS) also called as under-virilized male syndrome.[8] CAIS, the typical mode of presentation is in an adolescent female who has well developed breasts with a pubertal growth spurt but has no menarche and no or scanty growth of axillary and pubic hair. CAIS may also present in early infancy with the appearance of bilateral labial or inguinal swellings. Bilateral inguinal hernias are rare in girls and it has been estimated that 1-2% of such cases actually  have CAIS. On the other hand if a female child shows inguinal hernia, then  CAIS must be suspected every time.[6] PAIS is one category of intersex. The prototypic phenotype for PAIS is characterized by micropenis, perineo-scrotal hypospadias and a bifid scrotum. The testes may also remain undescended. The most severe form of PAIS usually presents as isolated clitoromegaly.[8] MAIS as a category of AIS was first diagnosed following investigations for male factor infertility which suggested a defect in androgen action with oligospermia and a normal level of testosterone. Diagnosis of CAIS is usually with the absence of the female internal genital organs on physical examination and pelvic ultrasonography, karyotyping, molecular genetic testing of the androgen receptor gene mutations (chromosomal locus Xq11-q12), and elevated levels of testosterone and luteinizing hormone.[6,9] In our case the diagnosis of AIS was made based on history, physical and gynecologic examination, ultrasonography, the karyotype and laparoscopy. Gonadal tissue may be located in the inguinal canal or anywhere in the abdomen. Magnetic resonance imaging and laparoscopic examination have proven value for localizing nonpalpable undescended testes.[10,11].There is an increased risk of malignancy in dysgenetic gonads which can be as high as 30%. In contrast to the other forms of gonadal dysgenesis, the incidence of malignant tumors in AIS cases is rare before puberty and significantly higher after the age of 35 years.[2,8] Kriplani et al reported that two out of seven male pseudohermaprodite cases (28.6%) who underwent laparoscopic gonadectomy had gonadal malignancies (12). For patients with AIS, prophylactic gonadectomy is necessary in the post pubertal period for the risk of malignancy of the gonads. Gonadectomy is performed after puberty, to allow the development of the secondary sex characteristics during puberty.[1,4] The laparoscopic gonadectomy has many advantages compared to laparotomy i.e minimal blood loss, rapid recovery and shorter hospital stay. Laparoscopy also has a better visualization of abdomen and pelvis compared to the laparotomy.[12] The operational time is similar in laparoscopy and laparotomy, but the recovery time and the duration of the hospital stay is much less with laparoscopy as compared with laparotomy.[13,14] In our case, there was a need for laparotomy as the right gonad was very close to the appendix and cecum and partially adherent to it. The patients with AIS need to be treated with long term hormonal replacement therapy mainly estrogen after gonadectomy.[2,7] The androgen supplementation treatment will not be beneficial in these patients due to the absence of functional androgen receptors.[15]

Conclusion

In conclusion androgen insensitivity syndrome should be suspected in cases with primary amenorrhea, confirmed by genetic studies and gonadectomy should be performed after puberty due to the risk of development of gonadal malignancy in future.

Acknowledgment

We thank Dr. Ashwini Desai for taking surgical photographs.

References
  1. Collins GM, Kim DU, Logrono R. Pure seminoma arising in androgen insensitivity syndrome (testicular Feminization syndrome): a case report and review of literature. Mod Pathol 1993;6(1):89-93.
  2. Creatsas GK. Hermaphroditism-Intersexual Disorders,Modern Obstetrics and Gynecology. Athens: Pashalidis Publishers, 1998;34-42.
  3. Barthold JS, Kumasi-Rivers K, Upadhyay J, Testicular position in the androgen insensitivity syndrome: implications for the role of androgens in testicular descent. J Urol 2000;164(2):497-501.
  4. Handa N, Nagasaki A, Tsunoda M. Yolk sac tumor in a case of testicular feminization syndrome. J Pediatr Surg 1995;30(9):1366-1368.
  5. Velidedeoglu HV, Coskunfirat OK, Bozdogan MN. The surgical management of incomplete testicular feminization syndrome in three sisters. British Journal of Plastic Surgery 1997;50(3):212-216.
  6. Solari A, Groisman B, Bidondo MP. Complete androgen insensitivity syndrome: diagnosis and clinical characteristics. Arch Argent Pediatr 2008;106(3):265-268.
  7. Speroff L, Fritz MA. Normal and abnormal sexual development in Clinical Gynecologic Endocrinology and Infertility. 7th ed. Philadelphia: Williams and Wilkins Publishers, 2005;319-359.
  8. Hughes IA, Deeb A. Androgen resistance. Best Pract Res Clin Endocrinol Metab 2006;20(4):577-598.
  9. Ahmed SF, Cheng A, Dovey L. Phenotypic features, androgen receptor binding, and mutational analysis in 278 clinical cases reported as androgen insensitivity syndrome. J Clin Endocrinol Metab 2000;85:658-665.
  10. Christensen JD, Dogra VS. The undescended testis. Semin Ultrasound CT MR 2007;28(4):307-316.
  11. Ishida K, Harada Y, Tei K. Laparoscopic examination of the nonpalpable testis. Hinyokika Kiyo 2007;53(11):795-799.
  12. Kriplani A, Abbi M, Ammini AC. Laparoscopic gonadectomy in male pseudohermaphrodites. Eur J Obstet Gynecol Reprod Biol 1998;81(1):37-41.
  13. Kallipolitis GK, Milingos SD, Creatsas GK. Laparoscopic gonadectomy in a patient with testicular feminization syndrome. J Pediatr Adolesc Gynecol 2000;13(1):23-26.
  14. Chantilis SJ, Mc Quitty DA, Priminger GM. Laparoscopic removal of gonads containing an occult seminoma in a woman with complete androgen resistance. J Am Assoc Gynecol Laparosc 1994;1(3):277- 282.
  15. Slob AK, van der Werfften Bosch JJ, van Hall EV. Psychosexual functioning in women with complete testicular feminization: Is androgen replacement therapy preferable to estrogen? J Sex Marital Ther 1993;19(3):201-9.
Citation

Swaminathan G, Parulekar SV. Bilateral Gonadectomy In A Case Of Complete Androgen Insensitivity Syndrome. JPGO. 2018 Vol 5 No. 8. Available from: http://www.jpgo.org/2018/08/bilateral-gonadectomy-in-case-of.html

Excision Of Collapsed Vaginal Wall Cyst With Old Rupture: Innovative Approach

Innovation
Author Information

Parulekar SV.
(Professor and Head, Obstetrics and Gynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.)

Abstract

A vaginal wall cyst sometimes ruptures spontaneously and may form a chronic discharging sinus. Excision of such a collapsed vaginal wall cyst is difficult as it is often empty and collapsed. An innovative method of excising such a cyst is presented.

Introduction

Vaginal wall cysts are not very uncommon in a busy gynecological practice. Many of them are diagnosed incidentally while the patient presents for some other condition. Large cysts may present with dyspareunia or symptoms of genital prolapse. Spontaneous rupture of a cyst and formation of a sinus has been reported only once in the world literature.[1] That patient refused surgical treatment. This is the second such case in the world literature. An innovative method was used to excise the cyst.

Case Report

A 34 year old woman presented with a complaint of chronic intermittent vaginal discharge of mucus, dating from spontaneous rupture of a swelling in her vagina 3 months ago. Turbid looking fluid had been discharged at that time. She had two normal deliveries in the past, the youngest child being 6 years old. Her menstrual history was normal. Her medical and surgical history was not contributory. Her general and systemic examination revealed no abnormality. A speculum examinaion of the vagina showed a bulging of the anterior vagina, which was folded up like a collpased structure. There was no expansion of the vagina on coughing, ruling out a cystocele. Pressure on the collapsed wall produced a drop of mucus from a very small opening in the posterior aspect.The remaining vagina was normal. The uterus was of normal size and shape. There were no pelvic lumps. A diagnosis of spontaneously ruptured anterior vaginal wall cyst which had led to formation of a chronic discharging sinus was made. Her investigations for fitness for anesthesia were normal. A cystoscopy was performed. It showed no abnormality, ruling out any connection between the cyst and the urinary bladder or the urethra.

The patient was placed in lithotomy position under spinal anesthesia. Aseptic and antiseptic technique was used. A size 14 Foley's catheter was passed into the urinary bladder. The opening of the sinus was identified by pressure on the collapsed cyst, which caused escape of a drop of mucus through the opening. The vaginal wall was held near the opening and then the opening was enlarged by serial passage of the tip of a small curved hemostat followed by Hegar's dilators from size 3/6 to 5/8. Then a No. 14 Foley's catheter was passed into the cyst through the dilated sinus track. Its balloon was inflated with normal saline until the cyst distended (about 10 ml). Then a circumferential incision was made in the vaginal mucosa around the opening of the cyst, and the incision was extended cranially and caudally for 1 cm each. The vaginal wall was dissected off the cyst wall, and then the cyst wall was dissected off the surrounding structures, like the urinary bladder posteriorly and the ischiocavernosus laterally on the right side. The opening through which the Foley's catheter had been passed expanded sopntaneously during dissection when the vagina was released on all sides around it. Finayy the cather balloon came out. A finger was passed into the cyst to define its limit. The cyst was found to be 5-6 cm in diameter. After removal of the cyst, hemostasis was achieved in its bed. Excess of vagina was excised and vaginal edges were sutured with interrupted sutures of No. 1-0 polyglactin. The patient made an uneventful recovery. Histopahology showed the cyst to be a benigh mucus secreting cyst.


Figure 1. Collpased anterior vaginal wall cyst. Opening of the cyst is seen (arrow).


Figure 2. Tip of a small curved hemostat is passed into the opening of the cyst.


Figure 3. The opening is dilated with passage of a Hegar's dilator.



Figure 4. A Foley's catheter is passed into the cyst through its opening.

Figure 5. The anterior vagina is dissected partly off the cyst wall. A part of the balloon of the Foley's catheter is seen through the enlarged opening of the cyst (arrow).


Figure 6. The anterior vagina has been dissected partly off the cyst wall. The edges of the vagina are shown by black arrows, and the limits of the cyst are shown by green arrows.



Figure 7. The balloon of the Foley's catheter is seen being expressed through the enlarged opening in the cyst.

Figure 8. A finger is passed into the cyst through the opening in its wall.


Figure 9. The cyst has been separated almost completely. The edges of the vagina are shown by black arrows, and those of the cyst are shown by green arrows.
Discussion

The cyst in this case was a mucous cyst. Such a cyst is often very thin walled and is more likely to rupture than other cysts of the vagina.[2,3] In case of a spontaneous rupture of the cyst, if the site of rupture heals, the cyst fills up again over a period of time. But if the epithelium lining the cyst heals with the vaginal epithelium over the edges of the opening caused by the rupture, a discharging sinus forms, which periodically empties its contents into the vagina. Surgical excision of such a cyst is difficult when it is collpapsed. If it is filled to some extent, dissection becomes easier because the cyst can be seen well and risk of accidental injury to adjacent structures like the urinary bladder and rectum is reduced. The cyst was never filled to adequately in the case presented, and only a few drops of mucus could be expressed at any time. In order to delineate its limits during surgery, it was necessary to fill it up. Distending it with saline was not an option, because its opening could not be closed and the saline would leak out during dissection. Then the cyst would collapse again. Filling it with a viscous liquid like lubricant or lignocaine gel would also not work out for the same reason, and operative field would become messy too. Hence an innovative idea was used. After dilating the opening of the cyst, a Foley's catheter was passed into it and its balloon was inflated so as to distend the cyst. Subsequent operative steps were as for excision of an anterior vaginal wall cyst. This maneuver reduced the risk of intraoperative injury to the urinary bladder and urethra, since the cyst wall was well defined and dissection was easier.

Conclusion

Distension of a collapsed vaginal wall cyst due to chronic rupture and sinus formation can be achieved with a balloon catheter. This makes dissection of the cyst easier and significantly reduces the risk of injury to adjacent structures.

References
  1. Parulekar SV. Vaginal Sinus Due To Rupture Of Posterior Vaginal Wall Cyst. JPGO 2015 Volume 2 Number 6. Available from: http://www.jpgo.org/2015/06/vaginal-sinus-due-to-rupture-of.html
  2. Sahnidt WN. Pathology of the vagina – Vaginal cysts. In: Fox H, Wella M, editors. , eds. Haines and Taylor Obstetrical and Gynecological Pathology. Vol. 1, Fifth edition New York, NY: Churchill Livingstone; 2003:180–3.
  3. Pradhan S, Tobon H. Vaginal cysts: a clinicopathological study of 41 cases. Int J Gynecol Pathol 1986;5:35-46.
Citation

Swaminathan G, Parulekar SV. Bilateral Gonadectomy In A Case Of Complete Androgen Insensitivity Syndrome. JPGO. 2018 Vol 5 No. 8. Available from: http://www.jpgo.org/2018/08/excision-of-collapsed-vaginal-wall-cyst.html

Successful Management Of Pregnancy Complicated With Guillain Barre Syndrome

Author Information

Venkateswaran S*, Hatkar PA**, Prasad M***.
(* Third year Resident, ** Associate Professor, *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Vomiting in pregnancy is usually considered to be hyperemesis gravidarum. However, when persistent, it may be the initial manifestation of some sinister diagnosis. A case of Guillain Barré syndrome (GBS) which initially presented as hyperemesis gravidarum is described here. Recovery after ICU care and intravenous immunoglobulin (IvIg) is also described.

Introduction

GBS is an acute polyradiculoneuropathy with variable presentation. The disease could have life-threatening complications and hence accurate criteria for diagnosis are required. GBS consists of a spectrum of neuropathic disorders that have various underlying pathogenesis and clinical presentations. We present a case of an 18 week G2A1 presenting as hypokalemic paralysis which was later diagnosed as GBS.

Case Report

A 26 year old G2A1 at 18 weeks of gestation was referred to our center for persistent vomiting for 2 weeks. She also complained of weakness of both lower limbs which was progressive in nature. She was unable to sit up without support. She did not have any complains of pain in abdomen, bleeding or leaking per vaginum. There was no history of fever or loose stools. On examination, she was well oriented and co-operative but had no signs of dehydration. She found difficulty in standing up and getting on to the examination table, for which she needed help. There was no tachycardia, blood pressure was normal, cardiovascular examination and respiratory system was normal. Abdomen was soft and non-tender, uterus was 18 weeks in size and relaxed. On vaginal examination, os was closed.
Owing to late presentation of vomiting, a condition unrelated to pregnancy was considered and she was sent for physician’s evaluation. Ultrasonography showed single live intrauterine gestation, rest abdominal organs were normal. Investigations revealed normal hemogram and liver parameters. However, surprisingly her serum electrolytes revealed hypokalemia with results showing sodium level of 135mEq/L, potassium level of 2.6mEq/L and chloride level of 105 mEq/L.
Detailed neurological examination showed power of 5/5 in both upper limbs, but < 3/5 in both lower limbs. Deep tendon reflexes were blunted in both upper limbs and absent in both lower limbs. Truncal weakness was present. Initial impression by neurologist was hypokalemic paralysis and potassium correction was instituted immediately. Despite this, she complained of breathlessness at rest and arterial blood gas analysis showed carbon dioxide retention. She was transferred to intensive care unit and observed for progression of symptoms. Invasive ventilation was not required.  There was a brief episode of glycosuria. Fasting and postprandial blood sugar levels were within normal limits and diabetic ketoacidosis was excluded. Symptomatology persisted despite correction of potassium levels and hence further evaluation was done. Electromyogram and nerve conduction studies were done, and results of these tests was suggestive of early Guillain-Barré syndrome. Intravenous immunoglobulin was started for the same. She reported significant improvement in respiratory symptoms but only marginal improvement in motor activities. Active physiotherapy was instituted after which truncal weakness resolved. Though she could sit up, she required help for activities of daily living, and had to be mobilized on a wheelchair. She was discharged after six days in the hospital. She was following regularly in the outpatient department with a residual amount of weakness of the lower limbs.  Apart from medications for usual antenatal care, she was also on multivitamin supplementation and physiotherapy for limb exercises.
At 36 weeks of gestation, She went into labor and delivered a female child of 2.3 kg by emergency lower segment cesarean section that was done for acute fetal distress. The surgery was   uneventful.
She recovered well postoperatively and was discharged with the advice to continue physiotherapy.

Discussion

Guillain Barré Syndrome (GBS) is an acute polyneuropathy. It may lead to severe weakness in many cases. It is an immune mediated, post-infectious disorder. Incidence in general population is 1.2 to 2.3 per 100,000 per year.[1,2]
Despite instituting treatment (consisting of parenteral immunoglobulins and plasma exchange) at the earliest, about 20% patients have difficulty in walking/ performing activities of daily living after 6 months. Thus, GBS is a severe disease with great impact on social life of the affected patients. Such a scenario was seen in our patient. Though the initial episode occurred at around 18 weeks, even at term gestation she had some residual weakness. Hence, any delay in instituting the treatment is unacceptable.
The main clinical feature of GBS is rapidly progressive muscular weakness. Motor symptomatology is rapid, bilaterally symmetrical weakness. This weakness may variably involve cranial nerve innervated muscles, respiratory muscles and sensory disturbances. There may be pain preceding the weakness, and autonomic disturbances may cause diagnostic difficulties. Typical history was seen in our patient also.
Artificial ventilation may be required, due to respiratory muscle weakness. However, our patient did not required ventilation at any point in time.
Patients who are able to walk with support are labeled as “mild” patients, and the ones who are unable to walk are labeled as “severe” patients.[2] Using this definition, our patient could be classified as “severe”.
In our case, the patient presented with extreme lower limb weakness and inability to walk, respiratory difficulty, truncal weakness, absent reflexes in both lower limbs. There was autonomic dysfunction in the form of vomiting which then led to hypokalemia.
Eikoundi et al have described recently a similar case with similar features, including hypokalemia and neurological signs, which was presumed to be GBS, but was eventually detected to be Gitelman syndrome.[3] In their case, the patient presented with similar complains of weakness and vomiting. Serum electrolytes revealed hypokalemia but the diagnostic feature of Gitelman syndrome which is metabolic alkalosis, hypocalcemia, hypomagnesemia in the absence of hypertension were what confirmed their diagnosis. A case of GBS diagnosed in a preexisting case of diabetic ketoacidosis has been described by Affes et al. However, our patient did not have any other preexisting medical conditions.[4]

Our patient however was being treated for hypokalemia with potassium supplementation and supportive care. Ongoing respiratory distress and persistence of autonomic symptoms even after administering potassium supplementation prompted us to ask for an electromyogram (EMG). Absent ‘h’ reflexes bilaterally on EMG clinched the diagnosis of GBS. While a variety of criteria for diagnosis of GBS are available, many include examination of cerebrospinal fluid by a lumbar puncture.[5] However, our patient did not undergo the same. No specific validated criteria in pregnancy appear to exist.
In our patient, intravenous immunoglobulin did not cause any maternal or fetal side effect. The European League Against Rheumatism has summarized the safety and side-effect profile of intravenous immunoglobulins in pregnancy.[6]

The key to management was prompt institution of intensive care which prevented potential mortality. There is a reported maternal mortality of 7 %, which is slightly higher than that of non-pregnant women.[7].

It was possible to institute prompt management due to the availability of specialist neurologist in our tertiary care hospital. Hence, it is advisable to manage such patients with lower limb weakness at a higher centre for adequate deliberation on the differential diagnosis and correct management of the condition. Detailed outcomes regarding obstetric outcomes in GBS is not well known. This case being reported to add to the available literature on GBS in pregnancy.

Conclusion

Successful maternal and fetal outcome can be achieved in GBS with a multidisciplinary approach involving the obstetrician, intensivist, neurologist and physiotherapist.

References
  1. Hughes RA, Cornblath DR. Guillain–Barré syndrome. Lancet. 2005; 366(9497):1653–66.
  2. van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol. 2008; 7(10):939–50.
  3. Elkoundi A, Kartite N, Bensghir M, Doghmi N, Lalaoui SJ. Gitelman syndrome: a rare life‐threatening case of hypokalemic paralysis mimicking Guillain–Barré syndrome during pregnancy and review of the literature. Clinical Case Reports. 2017;5(10):1597-1603.
  4. Affes L, Elleuch M, Mnif F, Kacem FH, Salah D B, Mnif M, et al. Guillain Barré syndrome and diabetic acido-ketotic decompensation during pregnancy: a case report and review of the literature. Pan Afr Med J. 2017;26:86.
  5. Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria. Brain. 2014; 137(Pt 1):33–43.
  6. Götestam SC, Hoeltzenbein M, Tincani A, Fischer-Betz R, Elefant E, Chambers C, et al. The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation. Ann Rheum Dis. 2016;75(5):795–810.
  7. Furara S, Maw M, Khan F, Powell K. Weakness in pregnancy-expect the unexpected. Obstetric Medicine. 2008;1(2):99–101.
Citation

Venkateswaran S, Hatkar PA, Prasad M. Successful Management Of Pregnancy Complicated With Guillain Barre Syndrome. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/successful-management-of-pregnancy.html

Uterine Rupture And Colporrhexis After Forceps Delivery And Fundal Pressure

Author Information

Nasare P*, Joshi A**, Pardeshi S***, Gupta AS****.

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

Abstract

Uterine rupture is a rare but catastrophic complication of instrumental delivery. Forceps delivery is considered to cause more major maternal injuries (like uterine rupture, colporrhexis, perineal tears) as compared to vacuum delivery. Here, we present a case of uterine rupture in a gravida who not only had a forceps delivery but also received fundal pressure to aid delivery.

Introduction

Uterine rupture leading to postpartum hemorrhage (PPH) is a rare but serious complication. PPH is a leading cause of maternal morbidity and mortality. Traumatic PPH is the second most common cause for PPH. Instrumental delivery being the most common cause resulting in this complication. Early detection and prompt intervention can save a life. A multidisciplinary approach with effective teamwork, availability of blood and blood products and well-equipped intensive care management are crucial in determining the outcome.

Case Report

A 34 year old gravida 3 para 2 living 2 abortion 1, day one of full term forceps delivery was referred to the emergency medical services in view of sudden onset abdominal pain and breathlessness post-delivery. On a detailed history it was learnt that the she had been admitted one day prior in labor. The details regarding progress of labor were not available. However she required forceps application in view of fetal distress in second stage of labor. She also mentioned that while the forceps delivery was ongoing another person in the labor room whom she could not identify gave fundal pressure on her upper abdomen and she was told to bear down simultaneously. She delivered a male baby weighing 2.8 kilograms but baby did not cry immediately. After resuscitation, baby was shifted to NICU for further monitoring.
She developed sudden onset pain in abdomen and breathlessness post-delivery, hence she was transferred to another center for further management. After reaching the transferred center, blood investigations were sent and an ultrasonography abdomen was done, which was suggestive of mild ascites and mild left sided pleural effusion. On a complete hemogram report, hemoglobin was 6.5 gram% and WBC and platelet count were within normal limit. Hence she was transfused one packed cell volume and immediately transferred to our center. On examination her general condition was moderate, she was conscious and well oriented, clinically severe pallor was present, pulse rate was 110 per minute, low volume, blood pressure was 90/60 millimeter of mercury. Her respiratory rate was 30 per minute and saturation on pulse oximetry was 97 % on oxygen inhalation by mask. The cardiovascular and respiratory system examination revealed no abnormality. On abdominal examination gross distension was noted, exact uterine contour could not be made out. A baseline abdominal girth was measured. On per vaginal examination a vaginal pack was noted in situ.
An intravenous access was obtained and intravenous fluids were started for the initial resuscitation while cross matched blood was being arranged. An ultrasonography of abdomen was done, which revealed a probable uterine rupture in anterior wall of lower uterine segment with moderate fluid in pelvic cavity with internal echoes. On abdominal paracentesis blood that did not clot was aspirated confirming the clinical suspicion of uterine rupture. A decision for exploratory laparotomy and obstetric hysterectomy if needed was taken. Since her blood group was ‘A’ negative, a major hurdle occurred due to acute shortage of the same. A senior blood transfusion medicine officer was consulted regarding transfusing O negative blood. Since it was a life-saving surgery, it was advised to transfuse O negative blood but with the precaution that ‘A’ negative blood if subsequently procured should not be transfused in the next 48 hours to avoid major transfusion reactions.
Meanwhile, her vital parameters were monitored strictly. After all necessary arrangements were made, she was taken for exploratory laparotomy. Immediately after starting the first blood transfusion, the procedure was begun. In lithotomy position, the vaginal pack was removed, a cervical tracing was done. One sutured cervical tear was located at 10 o’clock position however the apex of the tear could not be identified and it seemed to extend beyond the fornix. Abdominal exploration was done through a midline vertical incision. On opening the parietal peritoneum, hemoperitoneum of around 500 ml was suctioned out. A uterine rent was noted in its right lateral aspect almost up to the right sided cornua and its lower extension was in continuation with the vaginal wall colporrhexis. The nature of the rupture warranted the need for an obstetric hysterectomy.
A total obstetric hysterectomy was done. Owing to the close relation of the ureters to the vaginal vault and colporrhexis which was present, the urologists were called to trace the ureters and rule out any bladder injury. Ureters on both sides were found to be normal. As the entire lower extent of the rent could not be seen abdominally a digital examination done with full aseptic precautions helped in identifying the lower end of the rent which was not seen on the prior vaginal examination. This rent was then closed with polygalactin no 2-0. The bladder muscularis was found to be thinned out on the right posterolateral aspect of bladder (approximately 2 cm in length) and the bladder mucosa was protruding out. This was repaired with polygalactin 3-0 and was further reinforced by placing an omental patch. Methylene blue test was performed at the end, which revealed no leak of the dye from the urinary bladder. An intra-peritoneal drain was kept and the abdomen was closed in layers.


Figure 1. Complete rupture of the right lateral uterine wall (yellow arrow). Black arrow shows the right round ligament.




Figure 2. Rupture uterus specimen showing the entire right lateral wall rupture extending from just below the cornua to the external os.

She required a total 4 units of blood transfusion (1st A-ve and the subsequent 3 units were O –ve cross matched blood units) and 4 units of fresh frozen plasma. Post procedure she was shifted to the ICU for the first 24 hours after which she was shifted to the ward. The intraperitoneal drain was removed on the third post operative day. Urologist advised continued catheter drainage for three weeks. Catheter was removed after 3 weeks. She had no urinary complaints after removing the transurethral Foley’s catheter. Her postnatal course was uneventful. Her baby was discharged from NICU after 7 days where he was kept for birth asphyxia.

Discussion

Uterine rupture is an obstetric emergency which is life threatening both to the mother as well as the fetus. It is defined as a breach in the integrity of the myometrial wall. Depending upon the extent of injury, there are two types of uterine rupture, complete and incomplete. In a complete rupture the contents of the uterus may spill into the peritoneal cavity or the broad ligament. While in an incomplete rupture the visceral peritoneum is intact. 
The most common cause for uterine rupture is a previous scarred uterus. Rupture of an unscarred uterus is a rare entity with an incidence of 1 in 8000-15,000 deliveries.[1] It occurs mainly due to vigorous use of oxytocin or prostaglandins for induction or augmentation of labor, inappropriate use of instrumental delivery and fundal pressure during delivery. Other ancillary factors that contribute to this risk include grand multiparity, obstructed labor, larger birth weight, shoulder dystocia, higher body mass index (BMI), uterine anomalies or manual removal of the placenta.[2] In our patient, the identified risk factors were multiparity, fundal pressure and instrumental delivery.
A study conducted in Mali showed that, out of the total uterine ruptures that presented during the study course, 87.4% were noted in an unscarred uterus while 12.6% occurred in a scarred uterus. The observed risk factors for primary uterine rupture in their study were: dystocia coupled with oxytocin use (12.6%), malpresentations (12.4%), contracted pelvis (12.0%), fetal macrosomia (9.7%), and a contracted pelvis with macrosomia (3.4%). Grand multiparity comprised of 12.4% of all uterine ruptures while short inter-pregnancy interval was observed in 12.0% of the uterine ruptures.[3]
In our case, the patient was a multipara with previous uneventful vaginal delivery. In the present pregnancy, a forceps delivery was done for fetal distress in the second stage of labor. Details regarding the progress of labor, use of uterotonic agents, examination findings at the time of forceps delivery were not available. A detailed history was suggestive of use of fundal pressure for delivery which probably was a most significant contributory factor leading to uterine rupture. Fundal pressure involves applying a steady pressure over the uterine fundus. It is a controversial maneuver with no proven documented benefit, but few adverse events have been reported in association with its use.[4]
The uterine rupture can occur antepartum, intrapartum or postpartum. The most common time of presentation is intrapartum. Intrapartum rupture is usually detected after a sudden increase in maternal pulse rate and a decrease in blood pressure with vaginal bleeding and abdominal pain accompanied by fetal bradycardia [5]. However, in the postpartum period, a clinical diagnosis is difficult and it requires high index of suspicion. Literature search revealed a case report of a postpartum uterine rupture which was diagnosed 4 days post-delivery. It described a patient who had complaints of lower quadrant abdominal pain for 4 days post vaginal delivery.[6]
Treatment of uterine rupture mainly depends upon the prompt detection of the rupture, the extent of the rent, hemodynamic condition of the patient, feasibility of repair and expertise of the operating surgeon. In most of the cases an obstetric hysterectomy may be required as a life-saving procedure. In our case also obstetric hysterectomy was performed as the uterine rent was extensive and uterus was not in a salvageable condition. Besides this the tear had gone beyond the vaginal fornix, was very ragged, edges were not identifiable hence suturing of the rupture was not done. It is observed that perinatal mortality is higher than maternal mortality in uterine rupture.[7,8] As the baby was live born but severely asphyxiated, it is suspected that this rupture might have occurred while the fundal pressure and forceps traction were being given..

Conclusion

Uterine rupture in an unscarred uterus is an avoidable complication. Fundal pressures have no benefit and only cause harm and should be avoided. Even an instrumental delivery that is forcibly performed results in grave maternal and fetal injuries and complications. Iatrogenic uterine rupture cannot be justified for any reasons and practices that cause it can only be condemned.

References

  1. Pan HS, Huang LW, Hwang JL, Lee CY, Tsai YL, Cheng WC. Uterine rupture in an unscarred uterus after application of fundal pressure. A case report. Journal of Reproductive Medicine. 2002;47(12):1044–6.
  2. Wang PH, Yuan CC, Chao HT, Yang MJ, Ng HT. Posterior uterine wall rupture during labour. Human Reproduction. 2000; 15(5): 1198–1199.
  3. Teguete I, Traore Y, Sissoko A, Djire MY, Thera A, Dolo T, et al. Determining factors of cesarean delivery trends in developing countries: lessons from point G National Hospital (Bamako-Mali). INTECH Open Access. 2012; pp. 161–202.
  4. Wei SC, Chen CP. Uterine Rupture due to Traumatic Assisted Fundal Pressure. Taiwan Journal of Obstetric and Gynecology . 2006;45(2):170–2.
  5. Sweeten KM, Graves WK, Athanassiou A. Spontaneous rupture of the unscarred uterus. Am J Obstet Gynecol. 1995;172(6):1855-6.
  6. Hruska KM, Coughlin BF, Coggins AA, Wiczyk HP. MRI diagnosis of spontaneous uterine rupture of an unscarred uterus. Emergency  Radiology. 2006;12(4):186–188.
  7. Cisse CT, Faye EO, de Bernis L, Diadhiou F. [Uterine rupture in Senegal. Epidemiology and quality of management]. Medecine Tropicale: Revue Du Corps de Sante Colonial  [Med Trop (Mars)] 2002;62 (6):619-22.
  8. Vangeenderhuysen C, Souidi A. [Uterine rupture of pregnant uterus: study of a continuous series of 63 cases at the referral maternity of Niamey (Niger)]. Med Trop (Mars). 2002 ;62(6):615–8.
Citation

Nasare P, Joshi A, Pardeshi S, Gupta AS. Uterine Rupture And Colporrhexis After Forceps Delivery And Fundal Pressure. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/uterine-rupture-and-colporrhexis-after.html

Good Pregnancy Outcome In A Case Of Previous B-Lynch Suture

Author Information

Nasare P*, Prasad M**, Gupta AS***.
(* Third year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Postpartum hemorrhage (PPH) is still one of the leading causes of maternal mortality and an important cause for morbidity in the developing counties. Uterine compression sutures, though recently introduced, have become popular and effective in the surgical management of PPH. However, long term outcomes after this procedure is largely unknown. Pregnancy outcome in a patient who had undergone B-lynch suturing in the previous pregnancy is presented here. 

Introduction

Uterine compression sutures involve the use of an absorbable suture material that tightly compresses the uterus to prevent atony and hemorrhage. The major advantage is that it allows uterine conservation. While short term complications such as failure of procedure, need for re-laparotomy have been described, intermediate term and long term complications are not well documented. A pregnancy in a patient with previous B-Lynch suture is described here.

Case Report

A 24 year old woman G2P1L1 presented to the outpatient department at 21 weeks of gestation for antenatal registration. Her first pregnancy outcome was reviewed. It was a lower segment cesarean section done at 39 weeks of gestation in view of failure to progress in labor. She had been in labor for a total duration of 18 hours. Her operative notes were available and the procedure was done under spinal anesthesia. Male child of 2.6 kg had been delivered uneventfully, following which there was intraoperative hemorrhage due to uterine atony. Due to lack of response to medical management, uterine compression suture (B-Lynch) was taken with catgut no 0. This was accompanied by one unit blood transfusion, and no further procedure.
Following her antenatal registration, she had regular visits, and was compliant to antenatal care, which was uneventful. In view of fasting blood sugar of 99 mg % and postprandial blood sugar of 108 mg %, oral glucose tolerance test (100 mg) was performed which showed values of 100/209/164/81 mg % at zero hour (fasting), 1, 2 and 3 hours respectively. She was diagnosed as a case of gestational diabetes mellitus, and medical nutrition therapy and tablet metformin 500 mg once daily was started. Following this, sugars were controlled. Other antenatal evaluation was within normal limits.

In view of previous cesarean section and non-willingness for a vaginal trial, an elective cesarean section at 38 weeks of gestation was scheduled. Anticipating a recurrence of postpartum hemorrhage, adequate blood and blood products were kept ready. Lower segment cesarean section was performed under spinal anesthesia. Intraoperative there were no adhesions, and a healthy male child of 2.8 kg was delivered. Placenta and membranes were expelled spontaneously and completely. Oxytocin drip was started, uterine tone was maintained and there was no excessive hemorrhage. Uterus was eventrated and evidence of a previous compression suture was found in the form of a depression on the anterior and posterior surface of the uterus on either side.(figure 1) This scarring was very subtle. No other abnormalities were found. Postoperative recovery was uneventful and she was discharged on 5th postoperative day. Suture removal was done on day 14 and she chose barrier contraception.


Figure 1. Intraoperative image. Yellow arrow is pointing to an area on the anterior uterine surface showing subtle indentation (suggestive of prior compression suture).

Discussion

Recurrence of postpartum hemorrhage is well documented in medical literature. If postpartum hemorrhage occurs in a pregnancy, there is a 3-fold chance of recurrence of a similar event in the subsequent pregnancy. This was confirmed in a large population based cohort study by Oberg et al.[1] The risk is particularly higher if the woman is more than 35 years of age, has diabetes mellitus or has had two pregnancies with postpartum hemorrhage. In a similar study by Ford et al, the recurrence rate was pegged at around 15 %. They had opined that in every multiparous woman, a history of postpartum hemorrhage in previous pregnancy should be specifically sought for, and if present, should be advised to deliver only in a center with adequate blood banking facilities.[2]  In our case, the patient was not elderly, and it was only her second pregnancy. Though she was diagnosed with gestational diabetes mellitus, there was no recurrence of postpartum hemorrhage in the current pregnancy. Our patient had undergone prior conservative management of PPH with a uterine compression suture. She did not appear to have had any immediate complications. Uterine necrosis as a complication of uterine brace sutures, though rare is well documented.[3]
The first successful pregnancy outcome after a previous uterine compression suture was described as recently as 2009.[4] One early review had concluded that there were no adverse fertility outcomes following uterine compression sutures.[5] Gizzo et al conducted a review to assess the reproductive outcomes after the two main types of conservative uterine procedures for PPH; vessel embolization and compression sutures. It was noted that use of compression sutures resulted in a higher risk of subsequent need for cesarean section and the recurrence of PPH.[6]
In our case, the patient conceived around one and half years after the prior procedure, and it was a spontaneous conception. Hence, our case is in sync with available literature that fertility is not affected. Though the immediate advantages of uterine compression sutures are obvious, few case reports have reported long term adverse outcomes. Development of severe Asherman syndrome following B-Lynch suturing has been reported by Goojha et al.[7] In our case, patient had no complaints suggestive of hypomenorrhea or oligomenorrhea.  The most important finding in our case was that there were no adhesions, and only a minimal depression (scar) caused by compression identifying the prior B Lynch suture was seen. This is very much in contrast to the case reported by Begum J et al.[8] They had described a case where there were extensive adhesions, and there was uterine disfigurement.
Obstetric outcome in patient with prior uterine compression suture assumes importance because, it is well established that prior uterine vascular embolization increases the chances of repetition of similar procedure.[9] Hence, we are reporting this case, mainly to add to the existing literature regarding pregnancy outcomes with prior uterine compression sutures.

Conclusion

A case of pregnancy with prior B-lynch suture is presented. There was no recurrence of PPH. There were no intraoperative adhesions. While there was a subtle evidence of prior compression sutures, uterine contour was well-preserved.

References
  1. Oberg AS, Hernandez-Diaz S, Palmsten K, Almqvist C, Bateman BT. Patterns of recurrence of postpartum hemorrhage in a large population-based cohort. Am J Obstet Gynecol. 2014; 210(3): 229.e1-8.
  2. Ford JB, Roberts CL, Bell JC, Algert CS, Morris JM. Postpartum haemorrhage occurrence and recurrence: a population-based study. Med J Aust. 2007; 187(7):391–3.
  3. Gottlieb AG, Pandipati S, Davis KM, Gibbs RS. Uterine necrosis: a complication of uterine compression sutures. Obstet Gynecol. 2008; 112(2, Part 2):429–31.
  4. Sentilhes L, Gromez A, Trichot C, Ricbourg-Schneider A, Descamps P, Marpeau L. Fertility after B-Lynch suture and stepwise uterine devascularization. Fertil Steril. 2009; 91(3): 934.e5-934.e9.  
  5. Fotopoulou C, Dudenhausen JW. Uterine compression sutures for preserving fertility in severe postpartum haemorrhage: An overview 13 years after the first description. J Obstet Gynaecol. 2010; 30(4):339–49.
  6. Gizzo S, Saccardi C, Patrelli TS, Di Gangi S, Breda E, Fagherazzi S, et al. Fertility rate and subsequent pregnancy outcomes after conservative surgical techniques in postpartum hemorrhage: 15 years of literature. Fertil Steril. 2013; 99(7):2097–2107.
  7. Goojha CA, Case A, Pierson R. Development of Asherman syndrome after conservative surgical management of intractable postpartum hemorrhage. Fertil Steril. 2010; 94(3): 1098.e1-1098.e5.
  8. Begum J, Pallave P, Ghose S. B-lynch: a technique for uterine conservation or deformation? A case report with literature review. J Clin Diagn Res. 2014 Apr; 8(4): OD01-3.
  9. Cho GJ, Shim J-Y, Ouh Y-T, Kim LY, Lee TS, Ahn KH, et al. Previous uterine artery embolization increases the rate of repeat embolization in a subsequent pregnancy. PLoS One. 2017;12(9): e0185467.
Citation

Nasare P, Prasad M, Gupta AS. Good Pregnancy Outcome In A Case Of Previous B-Lynch Suture. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/good-pregnancy-outcome-in-case-of.html

Juvenile Cystic Adenomyoma Managed Laparoscopically

Author Information

Shah N*, Paranjpe SH**, Puri J***.
(* Consulting Gynecologist/Obstetrician, Railway Hospital (Byculla) and Honorary Endosopic Surgeon, Wadia Hospital, ** Director, Velankar Hospital & Paranjpe Maternity Home, Chembur, *** Consulting Gynecologist/Obstetrician.)

Abstract

Juvenile cystic adenoma is an uncommon type of adenomyosis. We present here a case, which was misdiagnosed pre-operatively as a hematometra in a non-communicating horn of the unicornuate uterus.  A differential of a degenerated myoma or a chocolate cyst was considered. However, upon laparoscopy, the complete excision of the lesion was done and the findings were consistent with juvenile cystic adenomyoma.

Introduction

Adenomyoma is a rare tumor of the uterus. It consists of smooth muscles and endometrial glands within it. It is not conclusively known regarding the mechanism of invasion of myometrium by the endometrium. It mainly affects perimenopausal women.[1] Also, it affects the whole uterus presenting as a diffuse disease, but may also present as a focal lesion.[2] Juvenile cystic adenomyoma are usually seen in 13 to 20  years age group and the real incidence is still unknown. A conventional hysterosalpingogram can be considered but a magnetic resonance imaging (MRI) is usually done for confirmation.

Case Report

An 18 year old girl presented to us with severe dysmenorrhea since the prior one year. She had attained menarche at the age of 15, after which the cycles were irregular for the first year but regularized later with normal flow. Dysmenorrhea during the last one year was gradually progressive, and present throughout the menstrual cycle. Medical management in the form of antispasmodics did not help in reduction of symptoms. Her systemic examination was within normal limits. On sonographic evaluation, a mass with central cystic degeneration was seen along the right lateral aspect of the uterus and medial to the right ovary. The differential diagnosis given were hematometra in the non-communicating rudimentary horn of unicornuate uterus, endometriosis, degeneration of myoma and torsion of ovarian mass. An office hysteroscopy, which showed the uterine cavity was normal and both ostia were seen.
She was posted for laparoscopy. A 10 mm supraumbilical port was used, and 3 and 5 mm ancillary ports were also inserted. On inserting the laparoscope, uterus was bulky, but normal shape with a 5 cm globular mass protruding from the uterus near the right cornual structures beside the round ligament.(figure 1) Both fallopian tubes and ovaries were normal in appearance.  Diluted vasopressin was injected into the myometrium on the uterus and a transverse incision was given over the swelling with a harmonic scalpel.


Figure 1. Globular mass seen just below the round ligament.


Figure 2. Injecting vasopressin and lifting up the mass.


Figure 3. Thick chocolate material drained.


Figure 4. Complete removal of the entire adenomyoma.


Figure 5. Uterus after removal of the mass.
After incising, thick chocolate material drained out. A thick myometrial cyst was present which was removed completely. After removal, the base of the cavity was checked and there was no opening into the uterus. Hemostasis was confirmed and the cavity was sutured in layers with vicryl no.1. She had no complication and was discharged the next day.

Discussion

Presence of cysts within the myometrium is considered unusual, and cystic adenomyoma is a differential diagnosis.[3] The exact incidence of the disease is unknown because of the rarity of the disease, underreporting, and unapproved diagnostic criteria. Recently, these adenomyomas are categorized under accessory and cavitated uterine masses. Acién et al have proposed that these type of juvenile cystic adenomyomas (JCA) should be considered as a new type of congenital mullerian anomaly because of its juvenile onset and its peculiar location which is always on the anterior wall of the uterus near the origin of the round ligament. This may be explained by the duplication of mullerian tissue at the level of the insertion of the round ligament which in turn maybe because of a defect in the female gubernaculum.[4]
It is also suggested that these lesions should be separately classified from the rest of the mullerian anomalies since uterine cavity contour is preserved. Takeuchi et al have provided a diagnostic criteria which include complaints of severe dysmenorrhea, age less than 30 years, and a cystic lesion of greater than 1 cm independent of the uterine lumen but covered with myometrium seen by diagnostic imaging technique or intraoperatively.[5]
In our case, sonography was suggestive of a hematometra in the non-communicating horn of the unicornuate uterus. Hysteroscopic evaluation with visualization of both ostia ruled this possibility out. Medical management for juvenile cystic adenomyoma include nonsteroidal anti-inflammatory drugs for pain relief, GnRH analogs and continuous oral contraceptive pills. Although medical management can be started, it only provides temporary and a partial relief.[5]
The gold standard treatment for a juvenile cystic adenomyoma is complete excision of the lesion, and use of minimally invasive method has been described.[6]

Conclusion

Juvenile cystic adenoma is probably more common than previously believed to be. It should be considered as a differential diagnosis in Mullerian anomalies, especially those with rudimentary horn. MRI can be used for a non-invasive diagnosis. Operative laparoscopy in the hands of an experienced surgeon is helpful. Early surgical treatment should be considered the treatment of choice as it will completely eliminate the cause of pain.

References
  1. Azziz R. Adenomyosis: Current perspectives. Obstet Gynecol Clin North Am 1989; 16(1): 221-35
  2. Tamai K, Koyama T, Umeoka S, Saga T, et al. Spectrum of MR features in adenomyosis. Best Pract Res Clin Obstet Gynecol 2006; 20(4) :583-602
  3. Buerger PT, Petzing HE. Congenital cysts of the corpus uteri. Am J Obstet Gynecol 1954;67: 143-51.
  4.  Acién P, Acién M, Fernández F, José Mayol M, Aranda I. The cavitated accessory uterine mass: a Müllerian anomaly in women with an otherwise normal uterus. Obstet Gynecol. 2010; 116 :1101–9.
  5. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, Jinushi M. Diagnosis, laparoscopic management, and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases. Fertil Steril. 2010; 94:862–8
  6.  Kriplani A, Mahey R, Agarwal N, Bhatla N, Yadav R, Singh MK. Laparoscopic management of juvenile cystic adenomyoma: four cases. J Minim Invasive Gynecol. 2011; 18:343–8.
Citation

Shah N, Paranjpe SH, Puri J. Juvenile Cystic Adenomyoma Managed Laparoscopically. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/juvenile-cystic-adenomyoma-managed.html

Termination Of Pregnancy In A Case Of Giant Leiomyoma

Author Information

Ganapati T*, Chaudhari HK**
(* Ex Third year Resident, ** Associate Professor. Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

Uterine fibroids are the most common tumors of the uterus.  They are benign growth and are also called as leiomyomas. Generally women with huge uterine fibroids are symptomatic. They present with menorrhagia, infertility or with pressure symptoms. Our patient  primarily sought medical attention for termination of pregnancy.

Introduction

Leiomyoma is known to grow in response to hormone stimulation and their prevalence is more in the reproductive years, affecting 20 % to 30 % of women of reproductive age group.[1] A genetic component of the pathogenesis of uterine fibroids has been suggested. High-frequency mutations involving chromosomes 6, 7, 12, and 14 have been reported in uterine leiomyomas. It is not known, however, how these mutations initiate the cascade of events that eventually leads to the formation of a fibroid. Some theories suggest that intrinsic myometrial anomalies and endometrial injury play important roles.[2] The clinical presentation of symptomatic uterine leiomyomas may include irregular uterine bleeding, pelvic pain, and pressure symptoms, such as urinary frequency/ urgency or infertility. Our patient who came for termination of pregnancy was incidentally diagnosed with giant leiomyoma.

Case Report

A 26 year old G2P1L1 married since 5 years presented to the OPD with 4 months of amenorrhea, 15 weeks by date for medical termination of pregnancy in view of failure of contraception and associated mass per abdomen. She noticed mass per abdomen 4 months ago which was insidious in onset and gradually increasing in size which she attributed to pregnancy. She had history of excessive menstrual flow with dysmenorrhea for 3-4 cycles prior to amenorrhea. She had no complaints of pain in abdomen, difficulty in micturition and defecation. No history of weight loss or loss of appetite. Her general and systemic examination were normal. On abdominal examination uterus was 28 to 30 weeks in size, fetal parts were not felt, and fetal heart sounds could not be heard. On speculum examination  anterior and posterior vaginal walls were bulging resulting in reduced vaginal cavity, cervix could not be seen. On vaginal examination a hard mass was felt in the anterior and posterior vagina obliterating the fornices, cervix was deviated to the right and seemed compressed between anterior and posterior vaginal wall. Bilateral fornices were full and non tender. Her blood investigations were normal. Ultrasound examination of the abdomen and pelvis revealed an intrauterine pregnancy of 14 weeks with cardiac activity present. Pregnancy was situated in the fundus above the fibroid. Uterus showed multiple fibroids. They were intramural, subserosal and submucosal in location, with largest fibroid being intramural measuring 10x12 cm with degenerative changes within it. Subserosal fibroid in the lower segment measured 7x5 cm, submucosal fibroid in the posterior uterine wall was 5x5cm in size. Corpus luteum was seen in the left ovary. Right ovary was normal.  Liver, spleen, and pancreas were normal. CECT scan that was performed after consent confirmed the above mentioned findings, there was compression of bilateral ureters causing hydronephrosis and hydroureter in the upper 1/3rd segment. Bladder neck was compressed due to pressure. There was no evidence of lymph node enlargement. Small bowel was displaced to one side and large bowel were normal, liver and gallbladder was normal. She and her relatives were counseled regarding the need for hysterotomy for 2nd trimester MTP due to location of fibroid in the lower segment and difficult vaginal delivery. They were also counseled regarding the need for an obstetric hysterectomy in the event of post abortal hemorrhage.  She and her husband did not want to continue pregnancy in view of failure of contraception  and opted for hysterotomy. Prior to hysterotomy ureteric stenting was done. Hysterotomy was done at 16 weeks of gestation.
On opening the abdomen uterus was 28 weeks in size, had multiple bosselations revealing multiple intramural fibroids of various sizes all over the lower segment of the uterus. Uterovesical fold was pulled up. Sharp dissection was done to separate the bladder from the lower uterine segment. Incision was taken over it to open the uterine cavity.  Fetus was delivered, placenta and membranes delivered completely. Uterus did not contract and retract resulting in atonic PPH. Oxytocics were given, bilateral uterine artery was ligated. She was transfused 4 units of packed cells due to excessive blood loss. As the bleeding could not be controlled a decision of hysterectomy was taken. Total abdominal hysterectomy was done. The lower uterine fibroids prevented application of clamps and few myomas coming in the way of the clamp were enucleated. After confirmation of hemostasis closure was done.  Weight of the uterus with fibroids was 1.8 kg. Postoperatively she required 2 more units of blood transfusion on Hb of 6 g%. She was stable postoperatively. She was discharged after stabilization. Histopathology report revealed leiomyoma of the uterus with no evidence of leiomyosarcoma.
Figure 1. Amniotic sac seen through the hysterotomy incision.

Figure 2. Giant leiomyoma occupying whole of pelvis. The arrows mark the extent of the fibroid extending to the lateral pelvic walls.

Discussion

Early menarche, exposure to exogenous estrogen, obesity, and pregnancy usually influence fibroid growth.[1]
Large uterine leiomyomas can result in mechanical obstruction of the pelvic ureters, may cause renal impairment, with hydroureters and hydronephrosis. Prognosis in patients with obstruction is good with normal renal function tests. A previous study noted an incidence of 14.35% mechanical obstruction of ureters in patients with uterine fibroids. Most of these cases are recognized incidentally at ultrasonography.[3] Rarely, a fibroid may cause acute retention of urine by kinking of the urethra.[4]  Rare presentations of uterine leiomyoma include asymptomatic fibroids, associated polycythaemia, infertility, hypercalcaemia and hyperprolactinaemia.[5]
Surgical termination of pregnancy in the presence of multiple large uterine fibroids may be technically difficult resulting in reduced efficacy of the termination procedure.[6] Medical termination in a grossly distorted uterus due to fibroids is a very difficult case to manage. No literature provides guidelines regarding medical termination of pregnancy in 2nd trimester with a giant leiomyoma.
Treatment of fibroids can be divided into medical and surgical or both.
Surgery should be considered when there is multiple large fibroid like in our case.  Our patient with multiple enlarged fibroids had a increased risk of excessive bleeding and a risk of requiring hysterectomy at the time of the operation which was already discussed with the relatives. Therefore, blood should be cross matched preoperatively in every patient with fibroid uterus and the patient should consent for hysterectomy should the need arise during the procedure.[7]
With a large myoma in the lower segment of the uterus, myomectomy may be inevitable and there appears to be no absolute contraindication to myomectomy during cesarean section or hysterectomy. Whereas small fibroid < 2-3 cm or single fibroids, myomectomy during cesarean section or hysterotomy probably is not indicated especially when it is asymptomatic. With an adequate experience in myomectomy during cesarean section or hysterotomy and use of high dose oxytocin infusion, severe hemorrhage which is the most serious complication can be controlled.[8]
Cases of myomectomy during cesarean section that has been reported reveal few cases that were complicated by severe hemorrhage necessitating hysterectomy.[9] They concluded that the decision to perform hysterectomy during cesarean section should be made with caution because of risk of hemorrhage. On the other hand, other studies have showed a high incidence of hysterectomy for post-partum hemorrhage at delivery and puerperium period and post-partum sepsis in which the myoma was not removed.[10] So removal of the myoma during cesarean section seems to be logical if required.

Conclusion

Thus we conclude that management of giant leiomyoma should be individualized for every women. Surgical management should be done with caution keeping in mind the excessive blood loss and need for hysterectomy. More research is essential in patients undergoing termination of pregnancy having fibroid uterus.

References
  1. Nayak S, Dash SP, Khatua M. Uterine Sub Mucosal Leiomyoma (Fibroid) – A Case Report. IOSR Journal of Dental and Medical Sciences (IOSR-JDMS). 2014;13(12)(12):17-21.
  2. Ligon AH, Morton CC. Genetics of uterine leiomyomata. Genes Chromosomes Cancer. 2000;28(3):235–245
  3. Fletcher HM, Wharfe G, Williams NP, Gordon-Strachan G, Johnson P. Renal impairment as a complication of uterine fibroids: a retrospective hospital-based study. J Obstet Gynaecol 2013; 33(4):394–8 .
  4. Munk B, Rasmussen KL. [Acute urinary retention caused by incarcerated fibromyoma in the 8th week of pregnancy]. Ugeskr Laeger 1988;150(32):1937–8.
  5. Hadidi SA, Mohammed TS, Bachuwa G. Unusual presentation of uterine leiomyoma. BMJ Case Rep. 2015; 2015.bcr2014208995.
  6. Fenwick DK, Divers MJ. Medical pregnancy termination in the presence of a massive uterine fibroid. Br J Clin Pract. 1995;49(6):332-3.
  7. Olufowobi O, Sharif K, Papaionnou S, Neelakantan D, Mohammed H, Afnan M. Are the anticipated benefits of myomectomy achieved in women of reproductive age? A 5-year review of the results at a UK tertiary hospital. J Obstet Gynaecol. 2004;24(4):434-40.
  8. Ghaemmaghami F, Karimi-Zarchi M, Gharebaghian M, Kermani T. Successful Myomectomy during Cesarean Section: Case Report & Literature Review. Int J Biomed Sci. 2017; 13(2): 119–121.
  9. Exacoustos C, Rosati R. Ultrasound diagnosis of uterine myomas and complications in pregnancy. Obstet. Gynecol. 1993;82(1):97–101
  10. Davis JL, Ray-Mazumder S, Hobel CJ, Boley K, Sassoon D. Uterine leiomyomas in pregnancy: A prospective study. Obstet. Gynecol. 1990;75(1):41–44.
Citation

Ganapati T, Chaudhari HK. Termination Of Pregnancy In A Case Of Giant Leiomyoma. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/termination-of-pregnancy-in-case-of.html

Quadruplets With Acute Fatty Liver Of Pregnancy

Author Information

Sikawar R*, Hatkar P**, Desai G***.
(* Third Year Resident, ** Associate Professor, *** Assistant Professor, Seth G S Medical College and K E M Hospital, Mumbai, India.)

Abstract

An interesting case of quadruplet pregnancy in a morbidly obese patient, with acute fatty liver of pregnancy (AFLP) at 33 weeks of gestation is presented. Early delivery and need for aggressive management which resulted in survival of the mother, and survival of one of the neonates is described. Maternal survival despite the severity of disseminated intravascular coagulation (DIC) is the highlight of the case. The first case of quadruplet pregnancy with acute fatty liver in pregnancy is being reported.

Introduction

AFLP is a rare catastrophic disease, mainly occurring in the third trimester of pregnancy. It is a diagnosis of exclusion, with a rare incidence of 1 in 7000 to 1 in 16000 deliveries.[1] A strong index of suspicion can lead to timely diagnosis and management. Delay in diagnosis is frequently associated with high maternal and fetal morbidity and mortality. The estimated rate of maternal mortality is around 18 % and can reach upto 80 % if complicated with DIC. The neonatal mortality rate is also high, ranging from 7 % and almost 65 %, in few studies.[2]

Case Report

A 24 year old woman G2A1 with spontaneously conceived quadruplet pregnancy presented to our tertiary care center at 33 weeks of gestation. The immediate reason for referral was hyperbilirubinemia, total bilirubin being 8.5 mg/ dl. She had complaints of generalised weakness since the last two weeks. There were no complaints of fever, diarrhea, vomiting, or pain in abdomen or itching in palms or soles. She was a diagnosed case of hypothyroidism and was on tablet levothyroxine 75 ug once a day. She was diagnosed with gestational hypertension 15 days prior for which she was started on tablet labetalol 100 mg twice a day. She did not have any other major medical or surgical illness.  On examination, icterus was present. She was conscious, well-oriented and afebrile. Her pulse was 80/ min, with a blood pressure of 90/ 60 mm Hg. Respiratory and cardiovascular systems were unremarkable. There was no pallor, but bilateral pedal edema was present. She was morbidly obese with a body mass index of 46.6 kg/ m2. There was abdominal wall edema with an overdistended uterus (figure 1) and multiple fetal parts felt.  Only one fetal heart sound was localized on Doppler, which was regular and 140 beats per minute. Per vaginal examination showed a closed internal os which was uneffaced. There were no premonitory symptoms or signs of impending eclampsia, bilateral deep tendon reflexes were normal and urine albumin was nil. She was admitted and investigated.

Figure 1. Image showing grossly distended abdomen.

Viral markers were negative. Ultrasonography was performed which showed 4 live fetuses, the lightest being 1.4 kg and the heaviest being 2.1 kg. Ultrasonography also showed mild hydronephrosis with mild hydroureter of right side, grade I fatty liver, no free fluid in the abdomen. There was no splenomegaly. She was shifted to medical intensive care unit, an opinion of gastroenterologists was taken, which suggested the diagnosis to be AFLP. She fitted into six parameters as per the Swansea criteria for AFLP. Hence AFLP was considered higher as a possibility than preeclampsia related complications.  The investigations are summarised in Table 1. She was started on vitamin K injections, antibiotics, Injection dextrose and syrup lactulose to maintain glycemia and for prevention of encephalopathy. She was advised to go for earliest possible termination of pregnancy for maternal interest. Injectable steroids (betamethasone intramuscular 12 mg 24 hours apart) was given. In view of coagulation abnormality, requiring induction of labor, hematologists advised correction. Fresh frozen plasma at 15 ml/ kg, one unit cryoprecipitate per 10 kg of body weight were advised. It was advised to monitor coagulation profile for further need for correction. Decision of induction of labor was taken after correction of coagulation profile. Over the course of two days, she was transfused with 11 units of FFPs and 11 units of cryoprecipitate for the abnormal coagulation profile (Table 1).
Table 1. Investigation Results

Parameter
Day of admission
Day of labor induction
Day of delivery
Post delivery
Hb (g/ dl)
10.8
10.7
8.8
7.1
WBC (/ cumm)
21,000
20,600
23,700
24,000
Platelet (lacs/ cu mm)
2.5
1.2
08
08
PT-INR
2.05
1.52
1.84
1.75
APTT/Control
85/28
59/31
47/29
31/28
Fibrinogen (mg/ dl)
71
207
134
217
FDP (mg/ l)
>320
>320
>320
150-320
Bilirubin(T/D)(mg/dL)
17.7/11.1
15/10.6
20/14.1
18.2/16
SGOT (IU/ml)
97
-
85
80
SGPT (IU/ml)
197
-
263
223
Creatinine (mg/ dl)
1.8
2.3
3.0
4.0
BUN (mg/ dl)
12.9
14.8
18
23
Na (mEq/ l)
123
138
134
135
K (mEq/ l)
3.1
4
3.5
4.1
Cl (mEq/ l)
97
105
96
110


Pre-induction ripening of cervix was done using dinoprostone gel, and a repeat dose was required 6 hours later. Labor was augmented using injection oxytocin 5 IU in 500 ml ringer lactate. 1st, 2nd, 3rd babies were delivered using vacuum in view of maternal exhaustion and the 4th baby was delivered by assisted breech delivery. Out of the four neonates, 3 were male and one was a female of which one male fetus was a still birth (1.2 kg). Among the three born alive, two required intubation, respiratory support and inotropic support, while one did not require any significant support. The baby weights of the latter 3 were 1.4 kg, 1.4 kg and 1.8 kg respectively. The two neonates requiring inotropic support succumbed within one week of ICU stay. One neonate was discharged on day 21 of life with an otherwise normal growth pattern.

Figure 2. Image showing quadriamniotic quadrichorionic placentation.

All measures were taken to prevent postpartum haemorrhage (Injection oxytocin drip and tablet misoprotol 800 ug kept per rectally). Correction of coagulation status was continued antepartum, intrapartum and postpartum depending on coagulation profile results every 12 hours. She required transfusion that comprised of 66 units of FFPs, 40 units of   cryoprecipitates, 23 random donor platelets units and 7 units of packed red cell over 7 days.Though she had tremors (hepatic flaps) suggestive of of grade I hepatic encephalopathy, she was oriented to place and person. Worsening of grade of hepatic encephalopathy was prevented by monitoring of blood sugar values, maintaining a high carbohydrate diet, injection dextrose 5 times a day and syrup lactulose. High WBC counts necessitated stepping up antibiotics to imipenem 250 mg once a day and vancomycin once a day. Gradually, bilirubin reduced and coagulation profile improved. There were no major postpartum complications. On day 21 postdelivery, she chose to get discharged, against medical advice.
Discussion

It is important to diagnose cause of jaundice in pregnancy accurately. The differential diagnosis includes HELLP Syndrome, cholestasis of pregnancy, acute fatty liver of pregnancy and acute viral hepatitis. AFLP is a diagnosis of exclusion.  Although the etiology of AFLP is not known, there is predilection for nulliparous women, multiple gestation, and pregnancy with male fetus.[2] It is believed to be due to mitochondrial dysfunction in long chain fatty acid oxidation, leading to hepatocyte accumulation of toxic metabolites. Women with AFLP have long chain 3 hydroxyacyl conenzyme A dehydrogense (LCHAD) enzyme deficiency.[3]  Liver biopsy is diagnostic of AFLP but cannot be done in most of the cases because of DIC. However, it can be strongly suspected when patient fulfils 6 of the following clinical and laboratory criteria. These are vomiting, polydypsia/ polyuria, abdominal pain, encephalopathy, high bilirubin (>15 µmol/L), hypoglycaemia (< 72 mg/L), high uric acid (> 340 µmol/L), leucocytosis (> 11x106/cu mm), ascites or echogenic liver on ultrasound, elevated SGOT/SGPT (> 42 µmol/ L), high ammonia (> 47), serum creatinine (> 150 µmol/L), coagulation abnormality (PT>14 sec), microvesicular steatosis on liver biopsy.  These are collectively called as Swansea criteria.[4] Though useful, it has not been fully validated. Our patient had complaint of abdominal pain with serum bilirubin of 20 umol/L. She had recurrent episodes of hypoglycaemia, persistently raised WBC count > 20,000/ cmm, SGOT 97 µmol/L and SGPT 195 µmol/L, serum creatinine increased to 4 µmol/L,  and PT was 35 seconds against control of 13 and INR was 2.5. Her USG abdomen was suggestive of grade I fatty liver. She fulfilled 8 of the Swansea criteria and hence diagnosis of AFLP was made. Though HELLP syndrome is more frequently encountered, there is hemolysis with elevated liver enzymes and thrombocytopenia. Thrombocytopenia occurred late in our patient, secondary to DIC.  AFLP can be distinguished from cholestasis of pregnancy in which patient has complaint of itching in palm and sole with raised alkaline phosphatase and bile acids. Management of AFLP comprise of rapid delivery of fetus with supportive care and correction of DIC. In most of the cases liver dysfunction and DIC starts improving post- delivery. High mortality has been reported due to cerebral edema, gastro intestinal hemorrhage, renal failure, coagulopathy, and sepsis. Intensive management of patients has reduced the mortality significantly.[5] Recent studies and cases do report a higher survival of patients with AFLP and DIC. A retrospective study by Dwivedi et al, which included 7 cases of AFLP, 4 developed DIC. However, there was only one mortality. There were three twin gestations. There was a male predilection too.[6]  In our case also this was seen with 3 males and one female fetus. Another case of multiple gestation with AFLP with complicated DIC but survival after needing intensive care support has also been described by Philip et al.[7] Only one case of triplet gestation with AFLP has been described.[8] Though multiple gestation is surely associated with AFLP, cases of higher order (quadruplets) were not found in contemporary literature, despite a diligent search. Similar to these cases described above, maternal survival despite the severity of hyperbilirubinemia and DIC is the highlight of this case.

Conclusion

The Swansea criteria can help in raising suspicion of AFLP. DIC is a major complication of AFLP. With good intensive care support and multi-disciplinary approach, maternal survival is possible.

References
  1. Bacq Y. The liver in pregnancy. In Schiff ER, Madddrey WC, Sorrell MF, editors. Schiff’s Disease of Liver. 11th ed. Philadelphia: Wiley-Blackwell 2007; pp 476-8.
  2.  Rajasri AG, Srestha R, Mitchell J. Acute fatty liver of pregnancy (AFLP)--an overview. J Obstet Gynaecol. 2007 Apr;27(3):237-40.
  3. Bellig LL. Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants. Adv Neonatal Care. 2004 Feb;4(1):26-32.
  4. Mackillop L, Williamson C. Liver disease in pregnancy. Postgrad Med J. 2010 Mar;86(1013):160-4.
  5. Usta IM, Barton JR, Amon EA, Gonzalez A, Sibai BM. Acute fatty liver of pregnancy: an experience in the diagnosis and management of fourteen cases. Am J Obstet Gynecol. 1994 Nov;171(5):1342-7.
  6. Dwivedi S, Runmei M. Retrospective study of seven cases with acute Fatty liver of pregnancy. ISRN Obstet Gynecol. 2013:730569. doi: 10.1155/2013/730569.
  7. Philip S, George S, Sunil G. AFLP complicating a twin pregnancy. Indian J Anaesth. 2014; 58(1): 73–5.
  8. Davidson KM, Simpson LL, Knox TA, D'Alton ME. Acute fatty liver of pregnancy in triplet gestation. Obstet Gynecol. 1998 May;91(5 Pt 2):806-8.
Citation

Sikhawar R, Hatkar P, Desai G. Quadruplets With Acute Fatty Liver Of Pregnancy. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/quadruplets-with-acute-fatty-liver-of.html