Please enable JavaScript.
Coggle requires JavaScript to display documents.
DEVELOPMENT AND MALFORMATIONS OF GENITAL ORGANS (VAGINAL AGENESIS…
DEVELOPMENT AND MALFORMATIONS OF GENITAL ORGANS
MÜLLERIAN ANOMALIES
~ 4% in the general population
Six major anatomic types
COMPLICATIONS:
Infertility
+/- Endometriosis
MAIN TYPES OF DEFECTS
AGENESIS/HYPOPLASIA
VERTICAL FUSION
(canalization abnormalities)
LATERAL FUSION
(duplication)
RESORPTION
(septum)
CAUSES:
GENETIC MUTATION
DEVELOPMENTAL ARREST
ENVIRONMENTAL INSULTS
COMMON SYMPTOMS:
(PRIMARY) AMENORRHOEA
ACUTE +/- CHRONIC PAIN
ABNORMAL VAGINAL BLEEDING
FOUL-SMELLING VAGINAL DISCHARGE
DIAGNOSTIC
ULTRASONOGRAPHY (US)
(all cases)
MAGNETIC RESONANCE IMAGNG (MRI)
HYSTEROSALPINGOGRAPHY (HSG)
(patency of Müllerian system, complex communication with the urologic or colorectal system)
CYSTOSCOPY, VAGINOSCOPY, LAPAROSCOPY, HYSTEROSCOPY
HYMENAL ABNORMALITIES
Failure of hymen to perforate during perinatal period
PRESENTATION
PUBERTAL GIRL WITH PERSISTENT PELVIC PAIN AND ABDOMINAL MASS OR PERINEAL BULGING DUE TO HEMATOCOLPOS
(+)
URINARY RETENTION OR/AND CONSTIPATION
DIAGNOSIS
EXAMINATION OF EXTERNAL GENITALIA+DIGITAL RECTAL EXAMINATION+US
TREATMENT HYMENOTOMY+DRAINAGE/
HYMENECTOMY
TRANVERSE VAGINAL SEPTUM
Failure in fusion and/or canalization of the urogenital sinus and Müllerian ducts
UPPER VAGINA 46%
MIDDLE VAGINA 40%
LOWER VAGINA 14%
DIFFERENTIAL DIAGNOSIS: CERVICAL AGENESIS (MRI)
COMPLICATIONS:
VAGINAL STENOSIS
TREATMENT:
DEFINITIVE SURGERY
RESECTION + END-TO-END ANASTOMOSIS
LONGITUDINAL VAGINAL SEPTUM
complex anomaly (+ septate uterus, uterine didelphi, bicornuate uterus, anorectal malformations or renal abnormalities – 20%)
PRESENTATION
WOMAN WITH DYSPAREUNIA, PERSISTENT BLEEDING OR ASYMPTOMATIC WOMAN
DIAGNOSIS
VAGINOSCOPY (PREPUBERTAL) OR PELVIC EXAM (REPRODUCTIVE AGE)
TREATMENT
RESECTION OF THE SEPTUM
VAGINAL AGENESIS (MÜLLERIAN APLASIA - MAYER-VON ROKITANSKY-KÜSTNER-HAUSER SYNDROME)
Congenital absence of the vagina
In 1/3 to 1/2 patients complex abnormalities
(genitourinary + skeletal + auditory)
(ovarian function preserved)
3 ELEMENTS
CERVICAL
UTERINE (TOTAL OR IN MAJOR PART)
VAGINAL (UPPER
DIAGNOSIS
US + BIMANUAL GENITAL EXAM + RECTOABDOMINAL EXAM
MRI
Laparoscopy
GOAL OF TREATMENT
CREATION OF FUNCTIONAL VAGINA
TREATMENT:
NONSURGICAL
SURGICAL
1) FRANK METHOD (vaginal dilators – I choice, with 85%-90% successful outcomes; (–) several years of treatment)
2) ABBE-MCINDOE (harvesting a split-thickness skin graft from the buttocks and placing it over a vaginal mold; place is created from the vaginal dimple to the level of peritoneum – II choice; 80% success rate)
3) LAPAROSCOPIC TECHNIQUES
4) BOWEL VAGINOPLASTY (10 CM BOWEL – SIGMOID OR SMALL INTESTINE)
5) VULVOVAGINOPLASTY (FROM LABIA MAJORA, M. GRACILIS, RECTUS ABDOMINIS OR PUDENDAL TIGH FASCIOCUTANEUS FLAPS)
VAGINAL ATRESIA/DISTAL VAGINAL AGENESIS/SEGMENTAL VAGINAL AGENESIS
PRESENTATION
AMENORRHOEA (+ often HEMATOCOLPOS)
PAIN
PELVIC/ABDOMINAL MASS
NORMAL II st. DEVELOPMENT (except dimple in the vaginal introitus)
DIAGNOSIS
US
MRI
TREATMENT
INCISION, IDENTIFICATION OF MUCOSA, PULL-THROUGH PROCEDURE
OR
GRAFTS (COLON) IF THE DISTANCE FROM THE UPPER VAGINA TO THE INTROITUS IS TOO GREAT
UTERUS DEFECTS (SEPTATE UTERUS)
1) COMPLETE UTERINE SEPTUM,
2) BICORNUATE UTERUS,
3) UNICORNUATE UTERUS.
SYMPTOMS
PAIN
RECURRENT MISCARRIAGE
INFERTILITY
TREATMENT
HYSTEROSCOPY OR VAGINAL APPROACH IF CONCOMITANT VAGINAL SEPTUM
NO TREATMENT
HYSTERECTOMY (EXCISION OF THE HORN)