Diagnostic Ultrasound - Abdomen and Pelvis
Hematometrocolpos TERMINOLOGY Abbreviations • Hematometra (HM) • Hematocolpos (HC) • Müllerian duct anomaly (MDA) • Cloacal malformation (CM) Definitions • HM: Distension of uterine cavity by blood products • HC: Distension of vagina by blood products • Hematometrocolpos: Distension of uterus and vagina by accumulated blood IMAGING General Features • Best diagnostic clue ○ Echogenic fluid within distended uterus ± vagina Ultrasonographic Findings • Grayscale ultrasound ○ Distended uterine &/or vaginal cavities – HM appears thick-walled due to surrounding myometrium – HC is lower in pelvis and appears thin-walled compared to HM ○ Mixed echogenicity material within uterine &/or vaginal cavities – Blood products of varying age ○ Fetal diagnosis reported – Thin bulging membrane separating labia – Distended vagina – May be associated with ascites attributed to uterine reflux via fallopian tubes versus associated distal urinary obstruction • Color Doppler ○ No flow: Presence of flow should raise concern for mass • 3D allows better sonographic evaluation of uterine fundal contour ○ Vital for diagnosis of MDA MR Findings • Distended uterus &/or vaginal cavities ○ T1WI: Isointense to hyperintense material ○ T2WI: Hyperintense, but less than simple fluid • T1 C+: No enhancement of endometrial/vaginal contents CT Findings • Nonspecific: Requires further work-up with US or MR • Enlarged distended uterus ○ May mimic a fluid collection, distended rectum, or mass Imaging Recommendations • Best imaging tool ○ US for initial evaluation or to confirm CT findings ○ MR best to confirm blood products, absence of solid mass ○ MR best to clarify anatomy, relationship of pelvic organs • Protocol advice ○ Ultrasound – Consider use of translabial scans – Some reports of transrectal sonography ○ MR – Include renal images on coronal scout views – True coronal images of uterus to evaluate fundal contour for MDA – Distend vagina with Surgilube if possible, helpful to inject even if tiny perineal orifice – Contrast necessary if there is concern for an underlying mass DIFFERENTIAL DIAGNOSIS Pyometra • Associated with fever, elevated white cell count • Clinical diagnosis, imaging cannot distinguish pus vs. blood • Does not involve vagina Endometritis • Seen after childbirth, uterine instrumentation • Look for gas bubbles within endometrial cavity • Not associated with amenorrhea • Does not involve vagina Muco/Hydrometrocolpos • Uterus/vagina distended with mucous secretions, not blood • Most commonly associated with imperforate hymen • Hymenal membrane appears white ○ HC/HM hymenal membrane appears bluish due to accumulated blood products Gestational Trophoblastic Disease • Uterus distended by complete mole, has typical snowstorm appearance, not echogenic fluid • Invasive mole typically hypervascular mass invading myometrium ○ Myometrium may be thinned in HM but is intact • Does not involve vagina Retained Products of Conception • History of recent delivery • Solid perfused tissue • Retained clot is hypoechoic, nonvascular, smaller in volume than that seen with HM • Does not involve vagina Complex Adnexal Mass • Always identify organ of origin of adnexal mass ○ Most are ovarian: Normal uterus can be identified separately ○ Pedunculated fibroids with cystic degeneration can be confusing – Not associated with amenorrhea – Look for vessels from myometrium to mass – Often other fibroids in uterine corpus in addition to pedunculated ○ If normal uterus is not identified could "mass" be abnormal uterus? • May require MR to visualize normal ovaries ○ If large complex pelvic mass ○ If vaginal sonography not possible Diagnoses: Female Pelvis 753
Hematometrocolpos Diagnoses: Female Pelvis PATHOLOGY General Features • Etiology ○ Imperforate hymen – Most frequent cause of vaginal outflow obstruction ○ Müllerian duct anomaly – Vaginal septum: Transverse or vertical – Vaginal agenesis – Cervical agenesis – Uterus didelphys with obstructed hemivagina is most confusing as normal menstruation occurs through nonobstructed side ○ Cloacal malformation – Confluence of rectum, vagina, and urethra into single common channel – Often septated or bilobed due to müllerian duplication – Up to 50% present with hydrocolpos at birth – Obstruction due to vaginal stenosis after reconstruction, stenosis of persistent urogenital sinus (no previous reconstruction), or cervical stenosis ○ Cervical/vaginal stenosis – Post radiation therapy for gynecologic/colorectal malignancies – Post reconstructive surgery – Vaginal stenosis described in chronic graft-vs.-host disease • Associated abnormalities ○ Renal anomalies ○ Endometriosis CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Primary amenorrhea ○ Cyclical pelvic pain/pressure ○ Low back pain • Other signs/symptoms ○ Urinary retention secondary to pressure on bladder/ureters ○ Constipation secondary to pressure on rectum ○ Presents at puberty if associated with MDA – Uterus didelphys → duplicated vagina → normal menses through unobstructed side with progressive distension of obstructed side ○ Presents later if associated with cervical cancer – Average age at diagnosis of CxCA is 50 years – Radiation therapy induced cervical/vaginal stenosis develops within a year of therapy Demographics • Epidemiology ○ Imperforate hymen 1:1,000 ○ Vaginal agenesis 1:5,000 ○ CM occurs exclusively in females, 1:20,000 live births ○ Vaginal stenosis occurs in up to 88% of cervical cancer patients treated with radiation therapy Natural History & Prognosis • Depends on underlying etiology ○ Imperforate hymen easily corrected ○ CM requires complex repair with multiple surgeries ○ MDA repair varies with malformation: Simple septal resection to more complex vaginal reconstruction • Associated with endometriosis ○ Increased incidence of infertility/ectopic pregnancy ○ Chronic pelvic pain Treatment • Avoid aspiration as risk of infection → pyocolpos/pyometra • Imperforate hymen ○ Cruciate incision with marsupialization of edges to vaginal wall ○ Simple incision inadequate → does not guarantee complete drainage → risk of infection • MDA ○ Incision/removal of vaginal septum ○ Creation of perineal opening/vaginoplasty ○ Uterine surgery may also be required to improve chances of successful pregnancy • Radiation therapy related ○ Topical estrogen, anti-inflammatory ointment ○ Serial vaginal dilators • CM ○ Surgical challenge is to create 3 perineal openings with functional vagina, bladder/bowel control ○ Individual anatomy will direct reconstructive approach DIAGNOSTIC CHECKLIST Consider • HM/HC should be considered in young female patients with lower abdominal symptoms or back pain Image Interpretation Pearls • Distended uterine &/or vaginal cavities with heterogeneous avascular material SELECTED REFERENCES 1. Sakhel K et al: Begin with the basics: role of 3-dimensional sonography as a first-line imaging technique in the cost-effective evaluation of gynecologic pelvic disease. J Ultrasound Med. 32(3):381-8, 2013 2. Behr SC et al: Imaging of müllerian duct anomalies. Radiographics. 32(6):E233-50, 2012 3. Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging. 36(6):756-64, 2011 4. Drakonaki EE et al: Hematocolpometra due to an imperforate hymen presenting with back pain: sonographic diagnosis. J Ultrasound Med. 29(2):321-2, 2010 5. Junqueira BL et al: Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 29(4):1085-103, 2009 6. Dane C et al: Imperforate hymen-a rare cause of abdominal pain: two cases and review of the literature. J Pediatr Adolesc Gynecol. 20(4):245-7, 2007 7. Sherer DM et al: Acquired hematometra and hematotrachelos in an adolescent with dysfunctional uterine bleeding. J Ultrasound Med. 25(12):1599-602, 2006 8. Prada Arias M et al: Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg. 15(6):441-5, 2005 9. Warne SA et al: Long-term gynecological outcome of patients with persistent cloaca. J Urol. 170(4 Pt 2):1493-6, 2003 754
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Hematometrocolpos<br />
Diagnoses: Female <strong>Pelvis</strong><br />
PATHOLOGY<br />
General Features<br />
• Etiology<br />
○ Imperforate hymen<br />
– Most frequent cause of vaginal outflow obstruction<br />
○ Müllerian duct anomaly<br />
– Vaginal septum: Transverse or vertical<br />
– Vaginal agenesis<br />
– Cervical agenesis<br />
– Uterus didelphys with obstructed hemivagina is most<br />
confusing as normal menstruation occurs through<br />
nonobstructed side<br />
○ Cloacal malformation<br />
– Confluence of rectum, vagina, <strong>and</strong> urethra into single<br />
common channel<br />
– Often septated or bilobed due to müllerian<br />
duplication<br />
– Up to 50% present with hydrocolpos at birth<br />
– Obstruction due to vaginal stenosis after<br />
reconstruction, stenosis of persistent urogenital sinus<br />
(no previous reconstruction), or cervical stenosis<br />
○ Cervical/vaginal stenosis<br />
– Post radiation therapy for gynecologic/colorectal<br />
malignancies<br />
– Post reconstructive surgery<br />
– Vaginal stenosis described in chronic graft-vs.-host<br />
disease<br />
• Associated abnormalities<br />
○ Renal anomalies<br />
○ Endometriosis<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Primary amenorrhea<br />
○ Cyclical pelvic pain/pressure<br />
○ Low back pain<br />
• Other signs/symptoms<br />
○ Urinary retention secondary to pressure on<br />
bladder/ureters<br />
○ Constipation secondary to pressure on rectum<br />
○ Presents at puberty if associated with MDA<br />
– Uterus didelphys → duplicated vagina → normal<br />
menses through unobstructed side with progressive<br />
distension of obstructed side<br />
○ Presents later if associated with cervical cancer<br />
– Average age at diagnosis of CxCA is 50 years<br />
– Radiation therapy induced cervical/vaginal stenosis<br />
develops within a year of therapy<br />
Demographics<br />
• Epidemiology<br />
○ Imperforate hymen 1:1,000<br />
○ Vaginal agenesis 1:5,000<br />
○ CM occurs exclusively in females, 1:20,000 live births<br />
○ Vaginal stenosis occurs in up to 88% of cervical cancer<br />
patients treated with radiation therapy<br />
Natural History & Prognosis<br />
• Depends on underlying etiology<br />
○ Imperforate hymen easily corrected<br />
○ CM requires complex repair with multiple surgeries<br />
○ MDA repair varies with malformation: Simple septal<br />
resection to more complex vaginal reconstruction<br />
• Associated with endometriosis<br />
○ Increased incidence of infertility/ectopic pregnancy<br />
○ Chronic pelvic pain<br />
Treatment<br />
• Avoid aspiration as risk of infection → pyocolpos/pyometra<br />
• Imperforate hymen<br />
○ Cruciate incision with marsupialization of edges to<br />
vaginal wall<br />
○ Simple incision inadequate → does not guarantee<br />
complete drainage → risk of infection<br />
• MDA<br />
○ Incision/removal of vaginal septum<br />
○ Creation of perineal opening/vaginoplasty<br />
○ Uterine surgery may also be required to improve chances<br />
of successful pregnancy<br />
• Radiation therapy related<br />
○ Topical estrogen, anti-inflammatory ointment<br />
○ Serial vaginal dilators<br />
• CM<br />
○ Surgical challenge is to create 3 perineal openings with<br />
functional vagina, bladder/bowel control<br />
○ Individual anatomy will direct reconstructive approach<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• HM/HC should be considered in young female patients with<br />
lower abdominal symptoms or back pain<br />
Image Interpretation Pearls<br />
• Distended uterine &/or vaginal cavities with heterogeneous<br />
avascular material<br />
SELECTED REFERENCES<br />
1. Sakhel K et al: Begin with the basics: role of 3-dimensional sonography as a<br />
first-line imaging technique in the cost-effective evaluation of gynecologic<br />
pelvic disease. J <strong>Ultrasound</strong> Med. 32(3):381-8, 2013<br />
2. Behr SC et al: Imaging of müllerian duct anomalies. Radiographics.<br />
32(6):E233-50, 2012<br />
3. Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging.<br />
36(6):756-64, 2011<br />
4. Drakonaki EE et al: Hematocolpometra due to an imperforate hymen<br />
presenting with back pain: sonographic diagnosis. J <strong>Ultrasound</strong> Med.<br />
29(2):321-2, 2010<br />
5. Junqueira BL et al: Müllerian duct anomalies <strong>and</strong> mimics in children <strong>and</strong><br />
adolescents: correlative intraoperative assessment with clinical imaging.<br />
Radiographics. 29(4):1085-103, 2009<br />
6. Dane C et al: Imperforate hymen-a rare cause of abdominal pain: two cases<br />
<strong>and</strong> review of the literature. J Pediatr Adolesc Gynecol. 20(4):245-7, 2007<br />
7. Sherer DM et al: Acquired hematometra <strong>and</strong> hematotrachelos in an<br />
adolescent with dysfunctional uterine bleeding. J <strong>Ultrasound</strong> Med.<br />
25(12):1599-602, 2006<br />
8. Prada Arias M et al: Uterus didelphys with obstructed hemivagina <strong>and</strong><br />
multicystic dysplastic kidney. Eur J Pediatr Surg. 15(6):441-5, 2005<br />
9. Warne SA et al: Long-term gynecological outcome of patients with<br />
persistent cloaca. J Urol. 170(4 Pt 2):1493-6, 2003<br />
754