mullerian duct anomalies ( mda ) , though rare , can be a treatable cause of pelvic pain and infertility .
the spectrum of mullerian duct anomalies is a continuum rather than distinct entities , and some complex anomalies have features of more than one class .
since there are no precise clinical or imaging criteria to enable specific categorisation of such anomalies , there is ambiguous classification of these anomalies by various radiologists and clinicians .
we report a case of a rare complex mda , diagnosed as unicornuate uterus with cervical dysgenesis and cavitated , noncommunicating rudimentary horn .
there has been no similar case reported in the literature till date , adding to the spectrum of complex anomalies .
a nulliparous , 30-year - old female , presented to the infertility clinic with complaints of primary amenorrhoea and infertility , with chronic pelvic pain for more than ten years duration .
the patient had prior been investigated several times , including sonography of pelvis , outside our institution , with all inconclusive reports .
the patient underwent ultrasound in the radiology department of our institute , which demonstrated the presence of a normal sized anteverted uterus , oriented more toward the left side of pelvic cavity .
a complex mass comprising of solid and cystic components was found on the right side .
the patient was advised pelvic magnetic resonance imaging ( mri ) to better characterise the ultrasound findings .
mri of the pelvis showed presence of two asymmetric uterine horns , smaller on the right side with thin myometrium and normal sized left with normal myometrium .
both endometrial cavities were distended with hemorrhagic contents and showed no communication to each other .
were normally developed , however there was no demonstrable communication between the endometrial cavity and the endocervical canal .
a diagnosis of unicornuate uterus with cervical dysgenesis and a cavitated , noncommunicating rudimentary horn on the right side , was made [ figure 1 ] .
axial t1w ( a ) and t2w ( b ) images showing unicornuate uterus ( u ) with rudimentary right horn ( r ) .
sagittal t2w images ( c , d ) showing well formed cervix ( arrow ) with no communication between endometrial cavity and endocervical canal .
coronal t1w image ( e ) showing hematometra in unicornuate uterus ( u ) and rudimentary horn ( r ) with left hematosalpinx ( arrow ) the patient was taken up for laprohysteroscopy .
the cervix was well developed but the hysteroscope could not be negotiated beyond the isthmus , consistent with cervical dysgenesis .
dense adhesions were found in the pelvic cavity , likely due to retrograde menstruation and stage iv endometriosis .
the fallopian tubes on both sides were dilated with thick , oedematous walls and were found congested .
due to delayed presentation of the patient , advanced endometriosis , dense pelvic adhesions and a failed attempt to recanalize / reconstruct cervix , a decision for hysterectomy was taken after obtaining patient 's consent [ figure 2 ] .
mullerian duct anomalies ( mda ) , though rare , may present in different ways from infancy to young adulthood , with mucocolpos , hematocolpos , hematometra , primary amenorrhea , pelvic pain , infertility , or repeated pregnancy loss .
an understanding of the differences between these uterovaginal anomalies is crucial in understanding the respective clinical manifestations , different treatment regimens and prognosis .
many classifications of uterine anomalies exist including the buttram and gibbons and the american fertility society ( afs ) classification .
though these classifications are important in the treatment of infertility and symptoms arising from obstruction or deformity , it is important to realise that these classification systems serve merely as a framework and not all anomalies will fit completely into one of these categories .
it is more important to accurately describe the different components of the complex anomalies so that appropriate management can be planned .
the modified afs classification by rock and adam embraces a broader collection of uterine and vaginal anomalies without conflicting observations or over simplicity encountered in other classifications .
this classification correlates anatomic anomalies with embryologic arrests , classifying uterovaginal anomalies as dysgenesis disorders or vertical or lateral fusion defects .
class iv of this classification is a useful addition , embracing any possible unusual configurations or combination of defects , since genital tract aberrations do not necessarily follow any defined and consistent pattern .
a unicornuate uterus with a rudimentary horn is the most uncommon uterine anomaly , representing approximately 20% of all mda .
a cavitated , non - communicating rudimentary horn in a unicornuate uterus represents approximately 4.4% of all mda .
congenital cervical anomalies , including agenesis and dysgenesis are even rarer , with less than 200 cases of cervical agenesis being reported till date .
many authors have recommended hysterectomy as an initial procedure for a patient with cervical dysgenesis / agenesis with a functioning uterine corpus .
considering the small potential for pregnancy , some authors advocate procedures of recanalisation or reconstruction of cervix , especially in cases of cervical dysgenesis .
imaging plays an important role in detection and classification of mda , thereby guiding appropriate management .
although many of these anomalies may be initially diagnosed at hysterosalpingography or sonography , further imaging by mri is often required for definite diagnosis and elaboration of secondary findings .
complex anomalies and secondary findings of endometriosis along with renal anomalies are optimally characterised noninvasively .
the present case is unique , comprising of combination of two rare anomalies , a unicornuate uterus with cervical dysgenesis and a cavitated noncommunicating rudimentary horn .
this anomaly can be assigned to class iv of modified afs classification by rock and adam .
the review of literature did not reveal a similar case , though many authors have emphasized the possibility of complex anomalies in a patient with features of more than one class .
in the present case , the diagnosis was delayed due to complex nature of the anomaly and absence of an integrated clinico - radiological classification scheme for specific diagnosis of such anomalies .
to conclude , various complex mda may exist with combined features of more than one class .
an integrated clinical and radiological classification and familiarity with rare and complex mda is essential .
early surgery offered to the patient may reduce patients suffering , help restore a patent outflow tract and may preserve fertility in some cases .
 mullerian duct anomalies , though rare , can be a treatable cause of pelvic pain and infertility . 
 various complex mullerian duct anomalies may exist with combination of features of more than one class . 
 since there are no precise clinical or imaging criteria to enable specific categorisation , there is ambiguous classification of these anomalies by various radiologists and clinicians . a young female presented with complaints of chronic pelvic pain , primary amenorrhoea and infertility . 
 the patient was evaluated by sonography and magnetic resonance imaging and diagnosed as case of complex mullerian duct anomaly , a unicornuate uterus with cervical dysgenesis and cavitated , noncommunicating , rudimentary right horn . 
 the findings were confirmed on laprohysteroscopy and the patient underwent hystertectomy . 
 there should be an integrated clinico - radiological classification scheme and familiarity with rare and complex anomalies for appropriate diagnosis and management of complex mullerian duct anomalies . 
INTRODUCTION
CASE REPORT
DISCUSSION