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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 887-889
Two unique cases of atypical mullerian duct development


Radiology Department, PDU Medical college, RAJKOT: 360001, Gujarat, India

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Date of Submission05-Apr-2006
Date of Acceptance10-Nov-2006
 

Keywords: Bicornuate uterus, Renal agenesis, USG, CT scan

How to cite this article:
Trivedi A V, Vaishya K B, Dodiya C K, Gandhi S P, Pandya A J. Two unique cases of atypical mullerian duct development. Indian J Radiol Imaging 2006;16:887-9

How to cite this URL:
Trivedi A V, Vaishya K B, Dodiya C K, Gandhi S P, Pandya A J. Two unique cases of atypical mullerian duct development. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:887-9. Available from: http://www.ijri.org/text.asp?2006/16/4/887/32376

   Introduction Top


Congenital anomalies of uterus provide fascinating subject for study. Uterine malformations are usually detected during routine Ultrasound examination and Hysterosalpingography, and are confirmed by laparoscopy and surgery. The majority of mullerian duct anomalies are considered to be sporadic or multifactorial in nature, however polygenic and genetic patterns of inheritance have been described in expression of these anomalies. Extrautrine and intrauterine environmental factors, such as exposure to ionizing radiation, intrauterine infections and drugs with teratogenic effects can also produce defects in development of fetal genital tracts.

Here we report two cases of uterine anomalies associated with unilateral renal agenesis.


   Case reports Top


Case 1:

A 13 year old unmarried female presented with lower abdominal lump since one year associated with burning micturation. Her menstrual cycles were irregular and were accompanied with dysmennorhoea. On examination, a lobular cystic mass was felt in the suprapubic region, felt in pouch of Douglas on per rectal examination, and cervix was not [3] felt separately from it. Ultrasound examination showed a large multiloculated cystic lesion with internal echoes of approximately 10 X 12 cm in size [Figure - 1]. Left kidney was not visualized in left renal fossa. On laparotomy bicornuate bicollis uteri with left sided haematometra and haematosalpinx with absent left kidney was observed. Both ovaries were normal.

Case 2:

A 18 year old unmarried female with history of delayed menarche presented with secondary amennorhoea after two to three normal cycles associated with lower abdominal pain and lump with dysuria. On examination the vagina was single and a single cervical os was observed. Ultrasound showed a multiloculated lesion of approximately 20 X 12 cm in pelvis more towards left side causing elevation of uterus and bladder [Figure - 2]. Left kidney was absent in left renal fossa. Intravenous pyelogram showed absent left kidney. CT Scan showed a lobulated collection adjacent to uterine cavity suggesting that collection was within second cornu and that sided  Fallopian tube More Details [Figure - 3].

On laparoscopy, bicornuate unicollis uterus with haematometra and haematosalpinx involving left cornu and left fallopian tube was observed.

CLASSIFICATION OF MULLERIAN DUCT ANOMALIES:

Uterine anomalies are of different types and are classified in to following ways [Figure - 4] [1].

CLASS I : MULLERIAN AGENESIS OR HYPOPLASIA

Usually results from the non development or rudimentary development of both mullerian duct.

CLASS II: UNICORNUATE UTERUS:

Non development or rudimentary development of a mullerian duct. Rudimentary horn may be communicating or non-communicating with uterus.

CLASS III: UTERUS DIDELPHYS:

Two separate normal sized uteri and cervices are demonstrated with two uteri widely separated with preservation of myometrial and endometrial width.

CLASS IV: BICORNUATE UTERUS:

Partial fusion of mullerian duct with dividing septum composed of myometrium only.

CLASS V: SEPTATE UTERUS:

Here there is incomplete resorption of final fibrous septum between two uteri.

Septum may be partial or complete and may extend up to external cervical os.


   Discussion Top


Embryologically uterus, upper two third of vagina and fallopian tube are derived from paired mullerian (paramesonephric) ducts. Since 1967, Muller's theory about embryonic formation of female genital system has remained almost unchanged [2]. In 1957,Crosby et al proposed that fusion of the two Mullerian ducts starts at their caudal ends in Muller's tubercle, and proceeds cranially up to the fundus [3]. Failure of ducts to develop leads to various types of [5] uterine, cervical or vaginal agenesis while absent or incomplete fusion results in uterus didelphys. These congenital anomalies occur in 1-15% of women associated with renal anomalies especially agenesis or ectopia.

The association between the uterine and the renal anomalies can be described on the basis of the MAYER ROKITANSKY KUSTER HAUSER SYNDROME where the uterine agenesis associated with renal agenesis is attributed to the additional disturbances in other mesodermal structures. The renal system arises from the Wolffian(mesonephric) ducts and associated disturbances in these structures leads to renal agenesis or ectopia. Vertebral bodies are also derived from the mesodermal elements but there disturbances are not clinically significant.

Hence a hypothesis can be put that the associated uterine and renal abnormalities can be attributed to the mal or non development of the mesodermal structures from which both of these organs are developed.

For the evaluation of mullerian duct anomalies along with physical examination, other investigations available are Ultrasound and hysterosalpingography [4].

However these two are inconclusive as in first case and laparoscopy and surgery are often necessary for diagnosis and classification of these anomalies.

However with the advent of MRI it is possible to go for accurate diagnosis and precise classification of anomalies and demonstration of its complications [5]. But in countries like India it is overruled by cost and ability.

 
   References Top

1.The American Fertility Society classifications of adnexal adhesions, distal tubal obstruction, tubal occlusion secondary to tubal ligation, tubal pregnancies, mullerian anomalies and intrauterine adhesions. Fertil Steril 1988; 49:944-955.   Back to cited text no. 1    
2.Muller P, Musst A, Sobal A, Winroud JC, Gillet JY. Appareil urinaaire chez les povteues de malformation uterines : etude de 1333 observation press Med 1967; 10: 113-8.  Back to cited text no. 2    
3.Crosby WM, Hill EC. Embryology of Müllerian duct system: review day theory. Obstet Gyn 1929: 49: 799-805.   Back to cited text no. 3    
4.Blask AR, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. II. Teenagers. Radiology 1991; 179:84-88.  Back to cited text no. 4    
5.Fedele L, Dorta M, Brioschi D, Giudici MN, Candiani GB. Magnetic resonance imaging in Mayer-Rokitansky-Kuster-Hauser Syndrome. Obstet Gynecol 1990; 76:593-596.  Back to cited text no. 5  [PUBMED]  

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Correspondence Address:
A V Trivedi
20/25, New Jaganath, "Ashish", Rajkot: 360001, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32376

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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