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Volume 2 Issue 9, September 2015

Editorial
Chauhan AR

Giant Congenital AV Malformation Causing High Output Cardiac Failure.
Shetty A, Gupta AS.

Accessory Ovary: An Incidental Finding
Atre R, Gupta AS.

Situs inversus: a rare congenital anomaly. Situs inversus: a rare congenital anomaly.
Madhu Kumari, Mulchandani D, Thakur V, Chavan NN.

Recurrent Hematometra And Hematocolpos Due To Cervical Agenesis.
Amin K, Khadkikar R, Saxena N, Chauhan AR.

Hysterectomy at Teenage in Turners Syndrome.
Kokate P. Humne A, Fusey S.

Successful Pregnancy Outcome In A Patient With Ebstein’s Anomaly.
Changede P, Gupta S, Chavan N.

Torsion Of The Normal Adnexa.
Mehta N, Gupta AS, Thakur H, Samant PY.

Microcephalus In A Neonate Caused By Cytomegalo Virus (CMV)
Mehta V, Gupta AS.

Suture granuloma of the neocervix post Fothergill’s surgery.
Deshpande PS, Thakur HS, Gupta AS.

Editorial

Chauhan AR
Adnexal torsion is seen in approximately 2 -3 % of adolescents and young women in the reproductive age, though prepubertal and postmenopausal cases have also been reported. Ovarian torsion is defined as partial or complete rotation of the ovarian pedicle to such a degree as to occlude or compromise its vascular and lymphatic drainage. “Twisted ovarian cyst” presents as a medical emergency and may imply torsion of the ovary or adnexa; less commonly, paraovarian torsion or isolated torsion of only fallopian tube may be encountered.
Classic clinical features include sudden onset lower abdominal pain (right sided more common as the colon on the left is protective and does not give enough room for torsion to occur), nausea and vomiting. The pain may sometimes be colicky or episodic when the torsion is intermittent or partial. The commonest differential diagnosis is ectopic pregnancy; tubo- ovarian mass due to PID, acute appendicitis and renal colic should also be kept in mind. Ultrasonography is the diagnostic modality of choice, where commonly an enlarged ovary or ovarian cyst, and free fluid in the pelvis are seen. The “whirlpool sign” (twisted vascular pedicle) if seen, has a high sensitivity for diagnosing torsion. Though Doppler may reveal the absence of blood flow in the ovarian pedicle with high specificity, Doppler USG is not a sensitive modality and has not been found to improve the diagnostic accuracy. In fact, many surgically proven cases of torsion show the presence of blood flow on Doppler. CT scan and MRI should be reserved for those patients where the diagnosis is in doubt. Misdiagnosis and diagnostic delay can lead to the loss of the ovary, fallopian tube or both; prompt diagnosis is essential to reduce the risk of complications and increase the chances of ovarian conservation.
Ovarian masses associated with torsion are usually benign. A long utero- ovarian ligament rather than the size of the cyst determines whether torsion will occur. Torsion of the ovarian vascular pedicle results in venous congestion, hemorrhage, ischemia and eventually necrosis, believed to cause loss of ovarian function. It was for this reason that historically all torsed adnexae which appeared necrotic were surgically removed; emergency laparotomy with salpingo- oophorectomy was the standard treatment.
Laparoscopy has taken over from laparotomy in the last three decades and laparoscopic detorsion of the twisted adnexae with ovarian preservation is now the recommended surgical treatment, even in the presence of complete vascular occlusion or hemoperitoneum. Numerous studies in literature have evaluated laparotomy or laparoscopy with ovarian preservation with favorable results. Even if the ovary is dark, ischemic or necrotic in appearance, many authors emphasize that detorsion is the only surgery which should be performed; this is relatively easy and safe to do. As the tissue is extremely edematous and friable, care should be exercised; gentle handling and non –traumatic instruments should be used. The return of color after detorsion, and normal blood flow may not resume immediately but this should not discourage the surgeon. Surgeons usually excise necrotic tissue but the temptation to bivalve the ovary or perform cystectomy should be avoided as it can cause hemorrhage and undue removal of ovarian tissue as there is usually no clear plane of cleavage, eventually leading to oophorectomy. Though there are risks of sepsis (due to liberation of toxins), peritonitis and emboli, these are largely theoretical and have not been reported.
Oophoropexy, the surgical fixation or suspension of the ovary, is also easily performed via the laparoscope. It has previously been performed to prevent repeat ovarian torsion, and on the contra-lateral normal ovary to prevent torsion, but its efficacy is debatable and hence is not recommended. Current evidence has shown that ovarian function is preserved in as many as 88 - 100% of cases. Obviously, better outcomes are achieved if the surgical intervention occurs within 36 hours, with some reports suggesting that the best results are achieved when the time of onset of torsion to surgery is 8 hours. Many studies have analyzed subsequent ovarian follicular development, and shown restoration of ovarian function in > 80% of these ovaries. Laparotomy performed for unrelated causes has also shown macroscopically normal ovaries. In patients undergoing IVF treatment, oocytes have been retrieved from the previously detorsed ovaries and successfully fertilized. Hence preservation of ovarian function and fertility should be paramount and cases of twisted adnexae should be treated laparoscopically with detorsion and ovarian conservation. The September issue of our journal carries a case report on ovarian conservation in a case of adnexal torsion, which should interest and help the readers.

Giant Congenital AV Malformation Causing High Output Cardiac Failure

Author Information

Shetty A*, Gupta AS**.
(* First Year Resident, ** Professor. Department of Obstetrics & Gynecology, Seth G.S.Medical College & KEM Hospital, Mumbai, India.)

Abstract

Congenital vascular abnormal arterial and venous connections with immense potential for growth are Arteriovenous malformations (AVMs).[1] We report a case of cardiomegaly and hypoplastic lungs detected on prenatal ultrasound scan (USG) that turned out to be a huge arteriovenous malformation of the upper limb presenting as secondary high output cardiac failure in the neonate at birth.

Introduction

AVMs in neonates are very rare. The pathogenesis of these is not very well understood. Early and accurate diagnosis will help in delivering the neonate in a tertiary care set up where interventional radiology support is available and congestive cardiac failure can be managed effectively.

Case Report

A 22 year old primigravida with spontaneous conception out of non consanguineous marriage for 1 year presented to us in early labor. She was referred to us in view of a malformed fetus (cardiomegaly with hypoplastic lungs ) as detected on USG. She was asymptomatic throughout her pregnancy except for history of varicella infection at 20 weeks of pregnancy for which she was treated with acyclovir for 5 days. She also had papular erythematous coalescing plaques with fine scaling at periphery on upper and lower limbs for which she was treated with an antihistaminic by a private practitioner. Patient had no other co morbid surgical or medical illness .
Her general and systemic examination were normal. On abdominal examination, the uterus was full term, contracting once in every 10 minutes, and contraction lasting for 15 seconds. The fetus was in the LOA position. Fetal heart sounds were 144 beats /minute and regular . All her serological and biochemical investigations were within normal limits except for the third trimester USG scan showing fetal cardiomegaly with dilated SVC and pulmonary artery due to volume overload and hypoplastic lungs .There was a hetero echoic lesion 67 x 68 x 50 cm in the left forearm with significant internal vascularity and predominantly slow venous flow. Pediatric surgery consultation as part of prenatal fetal medicine was taken and a very poor fetal prognosis in view of cardiomegaly and hypoplastic lungs was explained to the patient’s relatives. Skin consultation was taken and patient was diagnosed with inverse pityriasis rosea as seen in Figure 1and was treated symptomatically with oral Azithromycin and local calamine lotion application.


Figure 1: Inverse pityriasis rosea on mother’s forearm.

Patient progressed spontaneously into active labour and delivered a 2.704 kg female neonate with Apgar score of 9/10. Neonate had a giant vascular lesion extending on whole of the left forearm. It was erythematous, warm and pulsatile with bruit and thrill, overlying skin had dystrophic changes probably due to tissue necrosis. All the features were suggestive of a huge Arterio venous malformation as shown in Figure 2, 3 and 4. Neonate was immediately shifted to NICU and was diagnosed to be in secondary congestive high output cardiac failure in decompensated state.


Figure 2: A, B, C. Giant vascular AV malformation with dystrophic chnages.

Cardiomegaly and the apparently hypoplastic lungs seen in the USG scan earlier were due to secondary high output congestive cardiac failure with volume overload because of AV malformation. Neonate was immediately started on anti congestive measures with maximum doses of frusemide and digitalised. Maintenance doses of digoxin were given. Interventional radiology support to repair the AV malformation which was the cause of CCF was taken. But due to poor general condition of the neonate and very high anaesthetic risk , immediate repair could not be attempted. Neonate succumbed to congestive cardiac failure on day 4 of birth.

Discussion

AVMs are among the rare congenital vascular anomalies. It is however very rare for an AVM to present in neonatal period. In series of 138 AVMs only one presented at birth.[2] If systemic blood pressure falls below the pulmonary pressure, neonate can develop a right to left shunt through foramen ovale and ductus arteriosus. In some cases this leads to early death.[3] Thus emphasizing the need for an urgent referral to an appropriately experienced team to care for these neonates. A clinical staging system introduced by Schobinger in 1990 is very useful for documenting presentation and evolution of an AVM.[4]
Stage I (quiescence): pink-bluish stain, warmth, and arteriovenous shunting by way of Doppler examination.
Stage II (expansion): Enlargement, tense/ tortuous veins, thrill, bruit and pulsations in addition to stage I.
Stage III: same as stage II, plus dystrophic skin changes, ulceration, (destruction) tissue necrosis, bleeding, or persistent pain ( pseudo Kaposi sarcoma )
Stage IV: cardiac failure (decompensation) in addition to stage III.
Management includes surgery, catheter embolisation, and direct percutaneous sclerotheraphy.[5] Distal ischaemia is a feature of extremity AVM and any intervention may decrease the distal circulation even further and it must be carefully planned taking into account the future treatment. Ideally surgery is to be carried out within 72 hours after embolisation. The results of managing large AVMs can be disappointing.[6] Symptomatic AVMs rarely present in neonates. When the lesions are extensive like in our case the neonate can present with high output congestive cardiac failure.[7] Urgent care by a multidisciplinary team is necessary for medical stabilization. Ideal treatment should be embolisation or sclerotheraphy followed by early surgery which in our case could not be attempted. But even with aggressive management recurrence is very frequent and associated with high risk of amputation . Research to develop of newer treatment options for AVMs is required. Surgical removal and intravascular embolization are palliative methods for controlling AVM’s but they are not always curative. This case once again reinforces the fact the peripheral AVM can be a cause of fetal cardiomegaly with a apparently hypoplastic lungs.[8]

References
  1. Richter GT,Suen JY. Pediatric extra cranial arteriovenous malformations. Curr Opin Otolaryngol Head Neck Surg. 2011 Dec;19(6):455-61 available from http://www.ncbi.nlm.nih.gov/pubmed/22552742.
  2. Young AE. Arteriovenous malformations. In: Mulliken JB, Young AE, eds. Vascular Birthmarks: Hemangiomas and Malformations Philadelphia: WB Saunders, 1988: 228-245.
  3. Pernot, C. Malignant brachial arteriovenous fistula in a newborn child: a case report. Angiology. 1979;30:640–644.
  4. Mulliken JB. Vascular anomalies. In: Aston SJ, Beasley RW, Thorne CHM, editors. Grabb and Smith’s Plastic Surgery. 5th ed. Philadelphia Lippincott- Raven; 1997: 191-204.
  5. Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg 1998; 102(3):643-54.
  6. Webb JB, O'Brien M, John PR, Nishikawa H. Early presentation of an extremity Arteriovenous malformation. JPRAS 2004; 57(8) : 785–788 available from http://www.jprasurg.com/article/S0007-1226(04)00216-4/
  7. Peter m waters, Donald s bae Paediatric hand and upper limb surgeries : practical guide Springhouse Publishing Co ,U.S.; 1 edition (1 March 2012) page 66
  8. Waters PM, Bae D. Pediatric Hand and Upper Limb Surgery: A Practical Guide. 1st ed. USA: Springhouse Publishing Co; 2012
  9. Knipe H, Radswiki, et al. Fetal cardiomegaly available from http://radiopaedia.org/articles/fetal-cardiomegaly
Citation

Shetty A, Gupta AS. Giant Congenital AV Malformation Causing High Output Cardiac Failure. JPGO 2015. Volume 2 Number 9. Available from: http://www.jpgo.org/2015/09/giant-congenital-av-malformation.html

Accessory Ovary: An Incidental Finding

Author Information

Atre R*, Gupta AS**.
(* Fourth Year Resident, ** Professor. Department of Obstetrics & Gynecology, Seth G.S.Medical College & KEM Hospital, Mumbai, India.)

Abstract

Anatomical aberration in the development of organs leads to their development at an abnormal anatomical site. Such aberrations are seen in various organs resulting in development of accessory breasts, accessory pancreas, accessory spleen, accessory ovaries and so on. All these are rare entities, Accessory ovary being extremely rare. Accessory ovary may either develop at the time of embryogenesis due to separation during migration or it may develop in a normal adult ovary due to trauma caused by an inflammatory process or during surgery.[1,2] Our patient is a case of bilateral ovarian cysts who at the time of exploratory laparotomy was incidentally found to have an accessory ovary like tissue near the normal left ovary but separate from it.

Introduction

Accessory ovary is defined as a part of ovarian tissue situated close to but connected (at least by the vascularity) to the normal ovary. It is a rare condition with incidence of 1:29,000-700,000 in indoor patients. Anomalies of other organs can coexist with an accessory ovary. In the genital system defects seen are accessory Fallopian tubes, uterus didelphys, septate uterus, and unicornuate uterus. Urinary system anomalies seen are bladder diverticulum, agenesis of unilateral kidney, ureter, accessory suprarenal gland. Gastrointestinal system may show presence of a lobulated liver or accessory pancreas.[3] Incidence of such an association is 36%. Accessory ovaries are usually detected incidentally at laparotomies done for other pathologies as they themselves are most often asymptomatic.[4]

Case Report

A 24 year old nulligravida, married since one year, came to the gynecology outpatient department with complaints of pain in lower abdomen and an ultrasononography report suggestive of bilateral ovarian cysts. She had regular menses which lasted for 4-5 days each cycle and came every 28-30 days with moderate flow without any associated dysmenorrhea. On examination, general and systemic examination was within normal limits. On abdominal examination, a 16week size mass was palpable in the left iliac fossa, cystic in consistency, mobile, and non tender. On vaginal examination, a 16-18wk size mass was palpable, well differentiated from the uterus, non tender, and mobile. Patient was admitted for evaluation. A repeat pelvic ultrasound was suggestive of a large cystic lesion measuring 6.0 cm * 5.5 cm in the right adnexa with a fat containing component measuring 4 cm * 3.5 cm within the cyst with calcification. There was no evidence of torsion. All these features suggested a dermoid cyst arising from the right ovary. Another large clear cyst measuring 11 cm * 9.9 cm was noted in the pelvis adjacent to the above cyst. Left ovary was normal. Right ovary was not identified. A computed tomography examination of the pelvis confirmed the presence of a right ovarian dermoid cyst and a left ovarian cyst. An exploratory laparotomy with left ovarian cystectomy and right ovarian dermoid enucleation with bilateral ovarian reconstruction was done under spinal plus epidural anesthesia. Intraoperative findings revealed a 10 cm * 11 cm * 12 cm paraoophoron cyst in the left adnexa which was seen anterior to the uterus as shown in Fig 1. Uterus was normal in size and left Fallopian tube was stretched over the cyst. Left ovary was normal. Right ovary, with a right ovarian dermoid cyst of about 4 cm * 5 cm * 5 cm was also noted which was seen posterior to the uterus. A small 2 cm * 1 cm * 1 cm ovarian like tissue was seen in the left ovary at the junction of the fimbriae and the left paraoophoron cyst. It was seen separately from the normal left ovarian tissue suggestive of an accessory ovary as shown in the figure 1.


Figure 1: Laparotomy Findings: Left Paraoophoron Cyst (P), accessory ovary (Blue arrow), Stretche Fallopian Tube (T) right sided Dermoid cyst (D) and the uterus (U and the yellow arrow).

A biopsy was taken from this ovarian like tissue and sent for histopathological examination which revealed mature ovarian parenchyma with stroma thus confirming the finding of an accessory ovary. Post operative period was uneventful.

Discussion

Accessory ovary, as defined above was first described by Grobe in 1864. Development of an accessory ovary during embryogenesis as a result of separation during migration was explained. Lachman and Berman had an alternative point of view and said accessory ovaries are actually implants caused by trauma and are not of embryological origin.[2] Tumors arising in an accessory ovary are extremely rare but reports of various tumors like cystadenoma (serous or mucinous), dermoid cyst, Brenner tumor, steroid cell tumor are available. This suggests that the various types of tumours seen in an accessory ovary are similar to those seen in a normal ovary.[5] In our case, accessory ovary was an incidental finding and was detected due to the symptomatic bilateral ovarian cysts. We did not find any abnormalities in the Fallopian tubes, uterus. The accessory ovary did not appear to be associated with any other anomalies in other systems. In summary, accessory ovary is a rare under reported condition which should be looked for at exploratory laparotomies and if noticed we should be vigilant about any other anomalies associated with it. This condition is associated with a high risk of pelvic and renal anomalies and should lead to further evaluation in order to advise patients about future reproductive function and management of congenital anomalies. These accessory ovary can be a source for the development of neoplasm. The clinicians should document their presence for future reference.

References
  1. Lim MC, Park SJ, Kim SW, Lee BY, Lim JW, Lee JH, et al.Two dermoid cysts developing in an accessory ovary and an eutopic ovary.J Korean Med Sci. 2004 Jun;19(3):474-6.
  2. Lachman MF, Berman MM. The ectopic ovary. A case report and review of the literature. Arch Pathol Lab Med. 1991 ;115(3):233-5.
  3. Sharatz SM, Treviño TA, Rodriguez L, and West JH. Giant serous cystadenoma arising from an accessory ovary in a morbidly obese 11-year-old girl: a case report. J Med Case Reports. 2008; 2: 7.
  4. Vendeland LL, Shehadeh L.Incidental finding of an accessory ovary in a 16-year-old at laparoscopy. A case report. J Reprod Med. 2000;45(5):435-8.
  5. Kim AR, Sung WJ, Kim MJ. A Fibroma with Cystic Change Developing in an Accessory Ovary- A Brief Case Report - The Korean Journal of Pathology 2011; 45: 319-321.
Citation

Atre R, Gupta AS. Accessory Ovary: An Incidental Finding. JPGO 2015. Volume 2 Number 9. Available from: http://www.jpgo.org/2015/09/accessory-ovary-incidental-finding.html

Situs Inversus: A Rare Congenital Anomaly

Author Information

Madhu Kumari*, Mulchandani D**, Thakur V**, Chavan NN***.
(* Speciality Medical Officer, ** Registrar, *** Additional Professor. Department of Obstetrics & Gynaecology, LTMM College & General Hospital, Mumbai, India.)

Abstraxt

Situs inversus is a rare congenital anomaly where there is a reversal placement of various thoracic and abdominal organs from their normal anatomical location. 5-6 % of people with Situs inverses have heart defect & 20 - 25 % are associated with Kartagener's Syndrome. In most of the cases patients of these conditions are apparently normal and detected accidentally. However, due to presence of pre-existing cardiovascular or respiratory problems these individuals are prone to catch various infections. These individuals also have risk for sudden death. Here we present a rare case of situs inversus totalis detected accidentally in second full-term pregnancy.

Introduction

Situs inversus is a rare congenital anomaly in which there is reversal placement of various thoracic and abdominal organs. In Situs inversus totalis, mirror image of the anatomic location of all the viscera occurs while in dextrocardia only the apex of the heart points to right and is present in the right hemi thorax. [1] When Situs inversus is associated with sinusitis and bronchiectasis, it constitutes Kartagener’s syndrome.[2]

Case Report

A 31 years old patient, married since 10 years, gravida 4, living 1, spontaneous abortion 2, with 40 weeks of gestation with previous lower segment cesarean section done, was admitted in our tertiary care hospital in latent phase of labour. On admission patient was complaining of pain in abdomen with no complaints of bleeding or leaking per vaginum. She had a female child of 5 years, delivered at term by emergency lower segment caesarean section (LSCS) done in view of cephalopelvic disproportion. There was no history of intraoperative or post-operative complication. Second and third pregnancies ended as spontaneous abortions. Dilatation and curettage was not done both the time. Gravida 4 - present pregnancy, patient was registered at peripheral hospital and she had no history of any complications in this pregnancy. On admission – vital parameters were normal, cardiovascular system- both heart sounds were heard more prominently on right fifth intercostal space, no murmur was heard, Respiratory system - clear, air entry bilaterally equal. Per abdomen-uterus full term, vertex presentation, fetal heart rate 140 bpm, was getting minimal uterine activity, previous lower segment pfannenstiel scar was present, and no scar tenderness was noted. Per vagina - cervical os - one finger loose, 30 % effaced, vertex presentation, with bilateral convergent wall suggestive of mid pelvic contraction. Decision of emergency lower segment caesarean section (LSCS) was taken in view of cephalopelvic disproportion after cardiac evaluation. Twelve lead Electrocardiogram was done suggestive of inversion of T waves in aVL, v 1- v 6. Cardiac reference was done and 2D-ECHO was advised. Echocardiography confirmed dextrocardia with normal cardiac valve areas and normal size ventricle and atria with ejection fraction of 60%, no PAH, no evidence of vegetation. Fitness for emergency lower segment caesarean section was given by cardiologist. Ultrasonography of abdomen was advised and was suggestive of situs inversus-large lobe of liver in left hypochondria, gallbladder in left hypochondria, and spleen in right hypochondria. Emergency LSCS was done under spinal anaesthesia, no intraoperative complication occurred. Intraoperative large lobe of liver in left hypochondria was noted. Patient was monitored postoperatively, no complication occurred. In Postoperative period X-ray chest of mother and baby was done, mother’s X-ray was again suggestive of situs inversus (pic-1). X-ray chest of baby was normal. Patient was discharged on day 5 of LSCS. Patient was informed regarding the complication that can occur with situs inversus and screening of other family member was advised.


Figure 1. Chest radiograph showing dextrocardia with fundic bubble of stomach on right side.

Discussion

The exact incidence of situs inverses is not known because most of the patient remains asymptomatic. It is 0.01% approximately.
Types of sites inverses:
Situs inverses totals: Generally, there are no complications associated with situs inversus totalis because the organs are just reversed. Patient can remain asymptomatic but situs inversus totalis may also be associated with some other rare conditions. 5 - 6 % people with situs inversus totalis suffers from congenital heart disease. 20 - 25 %of patients with situs inversus totalis have Kartagener Syndrome. Patient with Kartagener Syndrome also have to face problems of abnormal bronchioles of the lungs and male infertility.
Dextrocardia is a congenital condition in which only heart is located on the right side of the chest. Left lung is smaller than the right lung. People may live normally, however few may suffer from additional congenital heart abnormalities like transposition of the great arteries. [3]
Situs ambiguous occurs in one in 20,000 births. In situs ambiguous, both liver and stomach develops in centre, stomach remains behind the liver. [4]
In case if situs inversus is diagnosed at autopsy, [5] then other family members should be counselled and are advised for screening, as they are prone to have various cardiac abnormalities.
If it is diagnosed during lifetime, the dangers of smoking and fumes should be told as they are at high risk for development of various respiratory infections. These patient should be advised to take vocational guidance. Cardiac abnormality and respiratory complications decreases the life span and thus increases the rate of sudden death in patients of situs inversus.

Conclusion

Situs inversus is a rare congenital anomaly with reversal placement of various thoracic and abdominal organs. Usually detected accidentally, are susceptible to catch various infection and are prone to sudden death.

References
  1. Narayan Reddy KS. Death and its cause. In the Essentials of Forensic Medicine &
  2. Toxicology. Pub. by K. Sugana Devi, Hyderabad, 22nd Ed., 2003 : p. 417.
  3. Kobzik L. The Lung. In Robbin's Pathologic Basis of Disease ed. by Cotran, Kumar and
  4. Collins. Pub. by W.B. Saunders Co., Philadelphia, In India by Harcourt India Pvt.Ltd., New Delhi. 6th Ed. 2000: p.716.
  5. Chugh SS, Kelly KL, Titus JL: Sudden Cardiac Death with apparently normal heart.
  6. Circulation, 2000; 102 : 649-654.
  7. Adults:Ann S. Fulcher, MD, and , Mary Ann Turner, MD From the Department of Radiology, Medical College of Virginia of Virginia Commonwealth University, 401 N 12th St, Rm 3-407B, Richmond, VA 23298-0615.
  8. Hangartner JRW, Marley NJ, Whitehead A, Thomas AC and Davies MJ. The assessment
  9. of cardiac hypertrophy at autopsy. Histopathology, 1985; 9: 1295-1306.
Citation

Madhu Kumari, Mulchandani D, Thakur V, Chavan NN. Situs inversus: a rare congenital anomaly. Situs inversus: a rare congenital anomaly. JPGO 2015. Volume 2 No 9. Available from: http://www.jpgo.org/2015/09/situs-inversus-rare-congenital-anomaly.html

Recurrent Hematometra And Hematocolpos Due To Cervical Agenesis

Author Information

Amin K*, Khadkikar R**, Saxena N***, Chauhan AR****.
(*Third Year Resident, ** Assistant Professor, *** Ex-Third Year Resident, **** Additional Professor. Department of Obstetrics and Gynaecology, Seth G S Medical College and KEM Hospital. Mumbai, India.)

Introduction

Cervical agenesis or aplasia is a rare Mullerian anomaly with incidence of 1 in 80,000 to 1,00,000 live births.[1] It is classified as Class IB Mullerian anomaly under American Fertility Society Classification.[2] The cervix is absent, and the lower uterine segment narrows to terminate in the peritoneal sleeve at a point above the normal communication with the vaginal apex. It is often associated with absence of portion or all of the vagina. We report a case of recurrent hematometra and hematocolpos due to cervical agenesis.

Case Report

A 19 year old unmarried girl was admitted to our tertiary center two years ago in view of primary amenorrhea and cyclical abdominal pain. General examination findings were normal and secondary sexual characteristics were well developed. On abdominal examination, a cystic mobile mass of 16-18 weeks' size was palpable. On local examination, pin point vaginal orifice was noted with no features suggestive of imperforate hymen. Ultrasound was suggestive of hematometra with right ovarian chocolate cyst of 6 × 5 cm. Magnetic Resolution Imaging (MRI) was suggestive of hematometra probably due to cervical stenosis with 5.2 cm right ovarian chocolate cyst. Tentative diagnosis was made as transverse vaginal septum. Resection of transverse vaginal septum through a very narrow introitus, with drainage of uterine collection (hematometra) was done. Cervix was not felt on examination. Foley's catheter was inserted into the cavity of hematometra and was distended to keep the cavity patent. One week later, examination under anesthesia was done with removal of Foley's catheter. On per vaginal examination, vaginal opening of one finger size was felt through vaginal septum. Uterus was felt but cervix was not palpable. Patient and her parents were counseled about poor prognosis in view of cervical stenosis and were asked to follow up as the definitive plan in this case was hysterectomy.
However patient did not follow up and presented two years later with progressively increasing dysmenorrhea, and lower abdominal pain in between the cycles. She gave history of menstrual cycles occurring every 15 - 20 days, lasting for 5 to 7 days. General examination was unremarkable. On abdominal examination, mild tenderness was present but no mass was felt. On per rectal examination, uterus was 6 weeks' size, anteverted, with restricted mobility. No forniceal mass was felt. Her baseline routine investigations were normal. MRI revealed hematometra with right sided distal hematosalpinx measuring 3.2 cm in diameter. Bilateral kidneys were normal.


Figure 1. MRI showing hematometra and hematosalpinx.

Exploratory laparotomy with subtotal hysterectomy was done. Intraoperatively, uterus was 10 weeks' size, bilateral fallopian tubes were normal, left ovary was normal. A right ovarian endometriotic cyst of 4 × 4 cm was seen with omental adhesions. This was enucleated, fulgurated and the ovary was reconstructed. Upper vagina was distended with blood (hematocolpos) ending blindly, indicative of partial vaginal agenesis. During hysterectomy, after applying clamps at the junction of cervix and vagina, uterine cavity was opened and 20 ml of chocolate coloured fluid was drained from uterine cavity and upper vagina. Cavity was examined. Findings were suggestive of absence of cervix and presence of blind and ballooned out vaginal pouch.


Figure 2. Surgical findings.

Histopathological report was suggestive of endometrium in proliferative phase, unremarkable myometrium with absence of ectocervix and focal thinning of endometrium at endocervix. Post operative course was uneventful. GnRH agonist injection Luprolide acetate 3.75 mg intramuscularly was given at monthly interval for three months. Patient has been counseled and advised a vaginoplasty prior to marriage.

Discussion

Cervical agenesis is the absence of cervix. Milder forms, in which the cervix is deformed and nonfunctional, are known as cervical atresia or cervical dysgenesis. These anomalies (complete agenesis and dysgenesis) are classified as type IB in the American Fertility Society Classification of Mullerian anomalies. According to EAC classification, it is classified as "Cx no fn, obst". [3]
Cervical atresia occurs due to defect in the elongation and canalization of Mullerian duct. Diagnosis is based on history, clinical examination, ultrasonography; confirmed by magnetic resonance imaging. Patients affected by this anomaly have normal secondary sexual development, and usually present at the time of puberty with primary amenorrhoea. As these cases have a functional uterus, they present with cyclical abdominal or pelvic pain due to hematometra. They may also have history of recurrent surgeries, as illustrated in this case. Vaginal atresia and renal tract anomalies such as renal agenesis, ectopic kidneys and malrotation are commonly associated.[4]
Conservative management in the form of laparoscopic uterovaginal anastomosis is considered as the first line of management but it should be done in highly specialized unit with the help of experts in laparoscopic surgery and management of complex Mullerian anomalies.[5] This was not possible in our case due to complete cervical agenesis. Also the procedure to make a channel in the blind vagina was tried previously in our case, but failed, leading again to hematocolpos, hematometra and endometriotic cyst in the ovary; hence definitive management that is hysterectomy was done instead of conservative management like uterovaginal anastomosis. Preoperative urography should be done to rule out associated anomalies in the kidney. Complications like endometritis, pelvic inflammatory disease, persistent pelvic pain, bowel and bladder injury, repeated surgeries and even death have been reported in the literature following reconstructive surgeries. Creation of the new vagina and cervix is a complex procedure associated with high morbidity in the form of sepsis and resultant restenosis due to fibrosis . In nearly 50% of cases cervical agenesis is associated with vaginal atresia and it is difficult to make a satisfactory fistulous tract, as in our case. Our patient developed hematometra and hematocolpos secondary to fibrosis and closure of the original surgical opening created between uterus and blind vaginal pouch. Several factors such as size of the created channel, duration of its stenting, presence of rudimentary endocervical glands and native vagina adjacent to the created channel and the number of menses allowed to flow through the stented channel may influence the surgical outcome. [6]
To conclude, though a reconstructive procedure in the form of cervicovaginal anastamosis is preferable, it can result in repeat hematometra formation ultimately requiring hysterectomy. Hysterectomy is warranted in extreme cases like this, as it saves repeated morbid procedures as well as financial and psychological burden.

References
  1. Suganuma N, Furuhashi M, Moriwaki T, Tsukahara S, Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril 2002;77:1071–3.
  2. American Fertility Society (1988) The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions. Fertil Steril 49: 944–955.
  3. Parulekar SV. Classification Of Congenital Malformations Of The Female Genital Tract. JPGO 2015. Volume 2 No. 4
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Citation

Amin K, Khadkikar R, Saxena N, Chauhan AR. Recurrent Hematometra And Hematocolpos Due To Cervical Agenesis. JPGO 2015. Volume 2 Number 9. Available from: http://www.jpgo.org/2015/09/recurrent-hematometra-and-hematocolpos.html