Uterus Didelphys - SUNY Downstate Medical Center¼llerian malformations True duplication of the uterus is rare Failed resorption of the common medial wall of the paired mullerian ducts

Uterus Didelphys

Maria Georgiades, MDJune 24, 2010

Morbidity and Morbidity

downstatesurgery.org

Case Presentation

• 13 year old female presented with dull, intermittent abdominal pain x 2 days in the lower abdomen.

• The pain initially relieved with Motrin

• Similar pains in 12/2009.


Presenter

Presentation Notes

Pain was 6/10 and non radiating.

History continued..

PMH: left renal agenesis, pneumonia

PSH: Exploratory laparotomy, excision of cyst wall, drainage of intraperitoneal blood and appendectomy- 12/2009 (Brooklyn Hospital)

All immunizations were up to date

SH: non - contributory


Physical Exam

• VS: T 98.9 BP 119/78 HR 83 O2 sat 100%

• General: well nourished, AAO x 3, no acute distress

• HEENT: NCAT, no conjuctival pallor, PEERL

• CV: RRR, S1S2 normal

• Pulm: CTA bilaterally; no rales, rhonchi, crackle

• GI: BS present, soft nondistended, TENDER IN THE LLQ, no rebound tenderness

• Rectal: good sphincter tone, no gross blood


Labs

CBC : 9.2/13/41/325

BMP: 139/5 101/26 12/0.8 <80

LFTs: 9.7/7.1/29/11/180/0.1


Differential Diagnosis

Ruptured ovarian cyst

Hematosalpinx

Ureterovaginal malformation

Uterus didelphys


Radiologic Studies

Abdominal ultrasound: absent left kidney

Pelvic US: mass in LLQ

MRI (5/4): uterus didelphys with left hematocolpos and distended left sided endometrium and distended endocervix

MRI (5/6): right uterine horn and cervix extending into single vagina; divergent rudimentary left uterine horn with fusion abnormality of left mullerian duct


Pelvic Ultrasounddownstatesurgery.org

Pelvic MRI -1downstatesurgery.org

Pelvic MRI-2downstatesurgery.org

Hospital Course

• Taken for EUA, vaginoscopy and possible vaginal aspiration on HD # 2

• Patient was and taken for an exploratory laparotomy on HD #3

• Intra-operative findings: didelphys uterus– Right: normal uterine system with a right ovary and a

cervix opening into the vagina– Left: hematosalpinx with a distended uterus filled with

dark blood and normal ovary.


Presenter

Presentation Notes

Wanted to make sure there was no duplicated vagina with a blind vaginal opening on the left side

Intra-Operative Imagingdownstatesurgery.org

Pathologydownstatesurgery.org

Postoperative Course

Pathology: weak proliferation of endometrium; endocervix with hematosalpinx with paratubal

adenomatoid tumor Foci of endometriosis

Diet was advanced on POD # 1

Discharged on POD#2


Normal Uterusdownstatesurgery.org

Paradenomatoid tumordownstatesurgery.org

Calretinin immunostaindownstatesurgery.org

Outline

Uterovaginal development

Mullerian malformations

Congenital fusion abnormalities

Herlyn-Werner-Wunderlich Syndrome


Figure 106-23 Development of uterus and vagina. During the 10th week, the paramesonephric ducts fuse at their caudal ends to establish a common channel and come into contact with a thickened portion of the posterior urogenital sinus called the sinovaginal bulb. This is followed by development of the vaginal plate, which elongates between the 3rd and 5th months and becomes canalized to form the inferior vaginal lumen. (Modified from Sadler TW: Langman's Medical Embryology. Baltimore, Williams & Wilkins, 1985.)

DEVELOPMENT downstatesurgery.org

Presenter

Presentation Notes

Nephric (wolffian) ducts degenerate Paramesonephric (mullerian) ducts give rise to the fallopian tubes, uterus, and upper 2/3 of the vagina -- As soon as the fused paramesonephric ducts connect to the sinusal tubercle they fuse in a caudal to cranial direction forming a tube with a single lumen. --endodermal tissue of the sinusal tubercle in the post urogenital sinus continues to thicken forming pair of swellings called the sinovaginal bulbs. These structures give rise to the lower third of the vagina.

Müllerian malformations

True duplication of the uterus is rare

Failed resorption of the common medial wall of

the paired mullerian ducts

More than 50 cases of uterus didelphys with a

unilateral imperforate vagina

Renal and axial skeletal abnormalies


Presenter

Presentation Notes

#2 result in 2 uteri and cervices Embryologically the mullerian ducts are derived from mesoderm, and the urogenital sinus is derived from endoderm. -Disruption of the local mesoderm development and its contiguous structions sometimes accounts for some of the associated axial skeletal abnormalities seen in mullerian anomalies.

Mullerian Ductal Defects

a. Agenesis and hypoplasia

b. Vertical fusion defects

c. Lateral fusion defects

Incidence: 1 of 200-600 fertile women


Congenital fusion abnormalities

Bicornuate uterus - 37%

Arcuate or incomplete septum - 28%

Complete septum – 9%

Didelphic uterus – 11%

Unicornuate uterus – 4%


Presenter

Presentation Notes

Arcuate: concave twd th fundus, incomplete reabsorption Unicornuate: one mullerian duct develops

Figure 129-12 Fusion anomalies of the müllerian system. Upper left to lower right, bicornuate uterus (partial), bicornuate uterus, uterine duplication, complete uterine and vaginal duplication, duplication of the uterus and cervix with a unilateral imperforate vagina. (From Yerkes EB, Rink RC: What urologists should know about pediatric gynecologic abnormalities. Contemp Urol 2002;14:12.)


Herlyn-Werner-Wunderlich Syndrome

Didelphic uterus, obstructed hemivagina and ipsilateral renal and ureteral agenesis

Failure of both lateral fusion and vertical fusion

Theory: Damage to the caudal portion of the wolffian duct


Presenter

Presentation Notes

Lateral fusion: between both uteri Vertical fusion: with the urogenital sinus that eventually forms the distal vagina ***wolffian and mullerian duts anatomically closer in the more caudal region and the mullerian duct may be guided by the wolffian duct in its descent toward the urogenital sinus -causes delay in diagnosis because menstruation is normal

Anatomical defect is a failure of lateral fusion of mullerian ducts combined with failure of vertical fusion between mullerian duct and urogenital sinus (hemivaginal septum ipsilateral to the side of renal ageneis. (Journal of Ped Surgery 2006. 41, 987-992)


Associated anomalies

Ipsilateral renal agensis is commonly seen

o Inferred that an early teratogenic process active in 4th

week of gestation resulted in arrested growth of one

mesonephric ductAgenesis of the ureteric bud

IVC duplication, intestinal malformation, ovarian

malposition


On history and physical…

Sx: asymptomatic until menarche cyclic or chronic pelvic pain Does not result in primary amenorrhea

PE: Unilateral abdomino-pelvic mass that

terminates in a bluish bulge in lateral vaginal mass


Presenter

Presentation Notes

Menstrual outflow obstruction results in retrograde flow, hematometra, hematosalpinx and endometriosis

Diagnosis

Imaging: MRI: two uterine horns usually widely

separated, with preservation of the endometrial and myometrial widths

US: renal anomalies on the side ipsilateral to the obstructed system


Complications

• If left untreated:

– Endometriosis

– Pelvic adhesions

– Pyosalpinx or pyocolpos

• Spontaneous abortion – 40%

• Fertility is comparatively good


Presenter

Presentation Notes

Patients do carry a pregnancy to term often and have no obstretric outcomes 34/36 females had at least one pregnancy and 89% had at least one living infant europena journal of ob gyn in 2000

Management

Diagnostic Laparoscopy

Treatment: Wide incision of vertical vaginal septum to

release the entrapped menstrual blood Resection of the vaginal septum Marsupialization of vaginal margins Hemihysterectomy and salpingo-oophorectomy


Presenter

Presentation Notes

---- reveal the type of uterine malformation but the presence of any complications like endometriosis, hematometra, hematosalpinx or ascending inflammatory process Main goal of treatment is for the patient to have successful reproductive outcomes and normal sexual relations Prompt and accurate diagnosis is needed in order to prevent cryptomenorrhea and endometriosis

Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis

(Herlyn-Werner-Wunderlich syndrome

80 patients with uterine and vaginal abnormalities 12 patients with HWWS Preferred surgical approach: full excision and

marsupialization of the vaginal septum

Hemihysterectomy with or without salpingooophoretomy is rarely indicated

J Pediatr Surg. 2006 May;41(5):987-92.


References

Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). Gholoum S, Puligandla PS, Hui T, Su W, Quiros E, Laberge JM. J Pediatr Surg. 2006 May;41(5):987-92.

Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis Jindal G, Kachhawa S, Meena GL, Dhakar GJ Hum Reprod Sci. 2009 Jul;2(2):87-9

Wein: Campbell-Walsh Urology, 9th ed.; Chapter 129 - Surgical Management of Intersexuality, Cloacal Malformation, and other Abnormalities of the Genitalia in Girls > ... > Obstructive Genital Anomalies

Adam: Grainger & Allison's Diagnostic Radiology, 5th ed.

Uterus didelphys and longitudinal vaginal septum coincident with an obstructive transverse vaginal septum. Moawad NS - J Pediatr Adolesc Gynecol - 01-OCT-2009; 22(5): e163-5


Thank you

Special Thanks To Dr. Velcek.


Documents

Uterus Didelphys - SUNY Downstate Medical Center¼llerian malformations True duplication of the uterus is rare Failed resorption of the common medial wall of the paired mullerian ducts