O+G Flashcards
Bartholin cysts are
Cystic dilatation of Bartholins glands.
Clinical: Most patients asx. Cyst formation in reproductive years. Secondary infection and abscess not uncommon. Rarely, malginancy can arise.
Bartholin cyst imaging characteristics:
Smooth marginated round vulvular cyst
Thin wall without significant inflammatory change
Thin septations may be present
Posterolateral aspect of the vaginal introitus, within superficial perineal pouch, below perineal membrane/PS
Typically solitary, can be multiple/bilateral
CT: Low density, non enhancing.
Uniformly thin wall, minimal enhancement
MRI
T1: Hypointense, but dependant on protein/haemorrhage
T2: Homogenous high signal, heterogenous if complicated
PCT1: no central enhancement. Thickened enhancing wall suggests infection. Nodular enhancement ?malignant transformation (rare)
US: thin walled anechoic vulvular cyst
no colour flow
Bartholin cyst embryology
Glands arise from urogenital sinus
Analogous to male Cowper (bulbourethral) glands
Bartholin cyst differentials
Bartholinitis: infection of the cyst, thickened enhancing wall with adjacent inflammatory change
Skene gland cyst: cystic dilatation of a Skene gland
Positioned anteriorly in vaginal introitus at the external urethral meatus
Gartner duct cyst: Simple cyst arising from anterolateral vaginal wall
Typically above the level of the pubic symphysis/perineal membrane
Epidermal inclusion cyst: SC lesion, most commonly from labia majora. often secondary to trauma or surgery
Urethral diverticulum: Cystic lesion margin of the mid urethra. May communicate with the lumen. Above the level of PS/perineal membrane
Adenocarcinoma of Bartholin’s gland: Significant enhancing soft tissue component
Periurethral collagen injection
Nabothian cyst: Endocervical canal
Urethral caruncle: Benign excrescences of urethral mucosa, postmenopausal
Prolapsed utererocoele: childhood mass eccentric to urethral meatus
Bartholin cyst clinical issues
Presentation: Asymptomatic, palpable vulvar mass, dyspareunia, pain/signs of infection
Demographics: Reproductive years, 20-30s. Glands involute post
2% of women in their lifetime
Prognosis: Resolve spontaneously, secondary infection
Malignant transformation: 40% adeno, 40% squamous cell. New cyst after 40 is suspicious
Treatment: Small asx; no treatment
If symptomatic; marsupialisation
other: resection, fistulisation, aspiration, ablation
Bartholinitis is
Infection of dilated/obstructed Bartholin gland, leads to abscess formation
located along the posterolateral vaginal introitus in superficial perineal pouch, below level of the pubic symphysis/perineal membrane
Can be bilateral, multilocular
Bartholinitis imaging characteristics
CT: Low density, rim enhancement
Peripheral enhancement may be slightly irregular
May see thin internal enhancing septation
Adjacent inflammatory fat stranding
MRI
T1: Well marginated, variable intensity
T2: High signal intensity uni or multilocular cyst
T1C+FS: thickened irregular enhancing wall with surrounding inflammation
US: introital cyst transperineal/translabial US
Increased peripheral Doppler flow
May have septations, internal debris
Nabothian cysts are
Mucinous endocervical gland cysts arising as a result of obstruction secondary to overgrowth of the squamous epithelium at their neck.
Tunnel clusters: complex multicystic dilation of endocervical glands
Nabothian cyst imaging characteristics
Circumscribed, unilocular, superficial cysts of the cervix
Typically simple and superficial but can be complex and invade cervical stroma
Most are few mm in diamter
Round or oval, single or multiple, can be numerous
CT: non-enhancing, hypodense to cervical lesion
MRI:
T1: Intermediate to hyperintense (mucinous)
T2: hyperintense, circumscribed, superficial
US: Anechoic, circumscribed lesion with posterior acoustic enhancement
Nabothian cyst differentials
Adenoma malignum: low-grade mucinous carcinoma affecting deep endocervical glands. Multilocular cystic masses. Enhancing solid components. Deeply penetrating. Copious watery vaginal discharge
Squamous cell carcinoma: Solid mass of cervix. Necrotic regions but solid elements predominate
Nabothian cysts clinical
usually asymptomatic
Tunnel clusters almost always occur in multigravid women >30
The majority require no treatment
Symptomatic; cyst drainage, cryosurgery, conization
Endocervical polyp imaging characteristics
Small pearl shaped mass
Feeding vessel in stalk on colour imaging
Originates from cervical canal, may protrude through external os and prolapse into canal
Usually between 2-30mm
Giant polyps are rare
CT
Soft tissue mass, similar attenuation to myometrium
MR
T1: low intensity within cystic spaces of polyp
T2: low intensity mass surrounded by high intensity fluid. large multicystic with high signal intensity fluid filling canal
DWI: no restriction
C+: Brisk enhancement, similar to myometrium
US
Echogenic mass within canal
Thickening of canal +/- cystic change
Central feeding vessel in stalk
Well defined echogenic structure in endocervix
Surrounding fluid may be present
May have cystic spaces
Sonohystogram: Smoothly marginated mass projecting off stalk
Endocervical polyp differentials
Cervical malignancy: Cannot differentiate from benign vs malignant containing polyp.
Cervical malignancy may invade underlying cervical tissue
Cervical leiomyoma: 10% of fibroid. Usually submucosally or subserosally but may be polypoid
Blood clot: no vascularity, will not enhance. Should pass over time.
Endometrial polyp or fibroid: Large enough to prolapse through external cervical os. tend to be polypoid with broad base.
Sarcoma botryoides: extremely rare, adolescents
Mullerian adenosarcoma: extremely rare
Uterine epithelioid endometrial stromal sarcoma: also rare
Endocervical polyp pathology
Etiology
Tamoxifen use
Other: multiparity, chronic cervicitis, foreign bodies, estrogen secretion
Focal hyperplastic protrusions of endocervical folds
Develop dysplasia and in situ or invasive carcinoma <1%
Gross
Usually pedunculated, may be sessile
Soft, smooth, red or purple
Few to 30mm
Microscopic
Classified according to the preponderance of tissue component: mucosal, fibrous, vascular, mixed endocervical/endometrial, mesodermal stromal
Cystically dilated endocervical glands
Large no. of blood vessels at surface
Inflammatory infiltrate 80%
Cervical carcinoma clinical
Third most common gynae malignancy
Typically presents in younger women with an average age of onset around 45 years
Risk factors:
HPV (not for clear cell or mesonephric)
Multiple sexual partners or male partner with multiple partners
Young age of first intercourse
High parity
Immunosuppression
HLA subtypes
Oral contraceptives
nicotine/smoking (not adeno)
Presentation:
Vaginal bleeding, discharge, abnormal screening
Cervical carcinoma pathology
Thought to arise from the transformation of cervical intraepithelial neoplasia
Histological types
Squamous: vast majority, HPV exposure
Adenocarcinoma: rarer. Subtypes: clear cell, endometrioid, mucinous, serous, mesonephric
Neuroendocrine: small cell, rare
Adenosquamous, rare
Squamous arise from the squamocolumnar junction. Situated at the ectocervix in younger patients and regresses into the endocervical canal with age
Adenocarcinomas arise from the endocervix
Prognosis
5 yr survival 92% stage 1 and 17% stage IV
Cervical carcinoma imaging characteristics
US
Hypoechoic, heterogeneous mass
May show increased vascularity on colour doppler
CT
Useful in assessing advanced disease, particularly adenopathy
also monitoring mets, planning of radiation port placement, guiding bx
Can be hypo or isoenhancing to normal stroma
MRI
Normal low signal cervical stroma provides intrinsic contrast for the high signal tumour
T1: usually isointense to pelvic muslces
T2: Hyperintense relative to stroma
C+: not routine, can be helpful for small tumours. high signal relative to stroma
Cervical carcinoma staging
FIGO or AJCC https://radiopaedia.org/articles/cervical-cancer-staging-1
FIGO 2018
1. confined to the cervix
- 1a1 0-3mm depth
- 1a2 3-5
- 1b1 5-20
- 1b2 20-40
- 1b3 >40
- beyond the uterus but not extended to lower third of the vagina or pelvic wall
- 2a1 upper 2/3 of vagina without parametrial <40
- 2a2 same >40
- 2b with parametrial but not wall - involves lower third of the vagina, +/- pelvic wall +/- hydronephhrosis +/- pelvic/paraaortic nodes
- 3a lower third no wall
- 3b pelvic wall and/or hydronephrosis
- 3c pelvic or paraaortic nodes - beyond true pelvis or mucosa of bladder or rectum
- 4a1 adjacent organs
- 4a2 distant organs
Mullerian duct anomalies are
Congenital abnormalities that occur when Mullerian/paramesonephric ducts dont develop correctrly.
Result may be complete agenesis, defective vertical or lateral fusion, or resorption failure
1-5% of women
majority asymptomatic, can present with miscarriages and infertility
Obstruction - abdominal mass and dysmenorrhoea
Delayed treatment - potentially infertility
Mullerian anomaly subtypes
Agenesis 10%
Arcuate 7%
Unicornuate 15%
Duplication
Didelphys 7.5%
Bicornuate 25%
Septate 45%
Mullerian anomaly associations
Renal anomalies; agenesis, crossed fused ectopic, duplex
Uterine agenesis is
Class 1 Mullerian duct anomaly where there is the complete absence of uterine tissue above the vagina
Agenesis-hypoplasia spectrum accounts for 10-15% of MDA
Uterine agenesis pathology
Clinical
Primary amenorrhea, normal hormonal levels with fully functional gonads
Path
Complete absence of the Mullerian ducts; Mayer Rokitansky Kuster Hauser MRKH syndrome
Associations
Renal tract anomalies
Vertebral anomalies
Unicornuate uterus is
Class 2 Mullerian duct anomaly characterised by a banana-shaped uterus usually draining into a single fallopian tube
Unicornuate uterus pathology
Failure of one of the ducts to elongate while the other develops naturally. Predominance on the right. May or may not have a rudimentary horn
Subclassification
A: Rudimentary horn
- A1: endometrium
- - A1a: communicating 10%
- - A1b: non communicating 22%
- A2: Controlateral horn has no endometrial cavity 33%
B: No horn 35%
Associations
Renal abnormalities; more common with unicornuate than the others, 40% of cases. Always ipsilateral to the rudimentary horn
Cryptomenorrhea
Primary infertility
Treatment and prognosis
Second worst obstetric outcome, with septate worst
Spontaneous abortion rates 41-62%
Unicornuate uterus imaging characteristics
HSG:
Fusiform banana shape
May have a small cavitation in type a
Drains to single fallopian tube
US
Difficult to detect on US, may be tapering
MRI
Curved and elongated uterus; banana shaped external contour
Reduced volume
Asymmetric configuration
Normal myometrial zonal anatomy
May be difficult to tell from bicorunate with only one side cannulated
Arcuate uterus is
Mild variant shape of the uterus, mild indentation of the endometrium at the uterine fundus. Least associated with reproductive failure.
Arcuate uterus pathology
Mild fundal indentation of the endometrium. Most common anomaly, 3.9% population. Exists on a spectrum with septate uterus, from least to most resorption of the uterovaginal septum
Arcuate uterus imaging characteristics
Normal fundal contour
No horn division
Smooth indentation of the fundal endometrial canal, <1cm
Increased transverse diameters
Fluoroscopy
Opacification of the cavity, single cavity, broad saddle shaped indentation of the uterine fundus
US
Normal external contour
Broad smooth indentation on the fundal endometrium
MRI
Normal external uterine contour. Myometrial fundal indentation is smooth and broad. Isointense to normal myometrium
Arcuate uterus differentials
Septate: Exist on a spectrum from most to least resorptive
Bicornuate: Arcuate has normal or slightly indented external fundal contour. Bicornuate has a more marked fundal indentation <5mm above the level of the uterine horns
Uterus didelphys is
Class III Mullerian duct anomaly where there is complete duplication of uterine horns as well as duplication of the cervix with no communication
Uterine didelphys pathology
Associations
Renal agenesis (helyn werner wunderlich)
Vaginal septum, including transverse septum
Along with unicornuate, greatest impact on reproductive performance
Pathology
Failed ductal fusion between 12-16th week of pregnancy
Characterised by two symmetric widely divergent uterine horns and two cervices
Uterine volume in each is reduced
Increased incidence of fertility issues; pregnancy to term 20%, third aborted, half premature. Only 40% result in living infants
Uterine didelphys imaging characteristics
Two widely spaced uterine corpora, each with a single Fallopian tube. Separate horns with large fundal cleft (distinct from septate)
HSG
Two separate endocervical canals that open into separate fusiform endometrial cavities with no communication between horns
If associated with longitudinal vaginal septum only one cervical os may be depicted and it may be cannulated with the endometrial configuration mimicking a unicornuate uterus
US
Separate divergent uterine horns identified with a large fundal cleft. Endometrial cavities are uniformly separate with no evidence of communication. Two separate cervices need to be documents.
MRI
Two separate uteri with widely divergent apices. Two separate cervices. Usually an upper vaginal longitudinal septum. Normal zonal anatomy is preserved.
Bicornuate uterus is
Class IV Mullerian duct anomaly, type of duplication anomaly. Concave or heart shaped external contour, fundal cleft >1cm deep. Caudally fused symmetric uterine cavities with some degree of communication.
Bicornuate uterus pathology
Clinical
Incidentally often
Early pregnancy loss and cervical incompetence
Associations
Longitudinal vaginal septum in 25%
Abnormalities of the renal tract
Pathology
Abnormal development of the paramesonephric ducts. Partial failure of fusion resulting in uterus divided into two horns
Subtypes; according to cervical canal
Bicollis: two cervical canals; central myometrium extends to the external cervical os
Unicollis: one cervical canal; central myometrium extends to the internal cervical os
Treatment and prognosis
In recurrent pregnancy loss; strassman metroplasty could be considered
In cervical incompetence; placement of cervical cerclage may increase fetal survival rates
Bicornuate uterus imaging characteristics
External contour is concave or heart-shaped, horns are divergent
Fundal cleft is typically more than 1cm deep and the intercornual distance is widened
Uterus seen as comprising caudally fused symmetric uterine cavities with some degree of communication, usually at the isthmus. Angle between the horns usually more than 105 degrees
HSG
Divided uterus, difficult to differentiate between septate and bicornuate since the uterine fundal contour is not visible
MRI
Helps confirm anatomy by showing a deep >1cm fundal cleft in the outer uterine contour and an intercornual distance of >4cm. Normal zonal anatomy
Septate uterus is
Class V Mullerian duct anomaly. Type of duplication anomaly resulting from partial or complete failure of resorption of the uterovaginal septum after fusion of the paramesonephric ducts.
Septate uterus pathology
Commonest. Type V.
Most common anomaly associated with subfertility, preterm labour, reproductive failure.
Partial or complete failure of resorption of the uterovaginal septum after fusion of the paramesonephric ducts. Septum is usually fibrous but can have some muscle
Associations: concurrent renal anomalies
Treatment
Metroplasty
Septate uterus subtypes
Partial: endometrial canal but not the cervix.
Complete: extends either to internal or external os
Septate uterus/vagina: extends into vagina
Septate uterus imaging characteristics
Variable external contour, convex, flat or mildly (<1cm) concave.
Acute angle between uterine cavities.
Endometrial canals are completely separated by tissue isoechoic to myometrium with extension to endocervical canal.
HSG:
Inaccurate for septate vs bicornuate. Angle of less than 75 between the uterine horns suggestive of septate, 105 suggestive of bicornuate
US
Endometrial stripe separated at the fundus by the intermediate echo septum. Extends to cervix in complete type. May show vascularity in septum.
MRI
Normal uterus size. Each endometrial cavity smaller than normal cavity. Variable septum signal
Septate uterus differentials
Bicornuate: shape of external contour
Arcuate: small myometrial indentation with normal contour
Thick adhesion
Haematometrocolpos is
Distension of the uterus and vagina with blood (metra uterus colpo ovary)
Haematometrocolpos imaging characteristics
Echgenic fluid within distended uterus +/- vagina
US
distended uterus/vaginal cavities. HM thick walled, HC thin walled.
Mixed echogenicity
No flow (if flow mass)
3D ?MDA
MR
T1 iso to hyper
T2 Hyper
Haematometrocolpos differentials
Pyometra: fever WCC. clinical dx
Endometritis: Post instrumentation/childbirth. Gas bubbles in endometrial cavity. Not associated with amenorrea, does not involve vagina
Muco/hydro metrocolpos
Gestational trophoblastic disease: Complete mole snowstorm appearance, not echogenic fluid. Invasive mole typically hypervascular and invading myometrium. Does not involve vagina
RPOC
Complex adnexal lesion
Haematometrocolpos causes
Imperforate hymen (most common)
MDA: vaginal septum (TV/Vert), vaginal agenesis, cervical agenesis, uterus didelphys with obstructed hemivagina
Cloacal malformation: confluence of rectum, vagina, urethra. Often septated/bilobed.
Cervical/vaginal stenosis: post radiation therapy, post recon surgery, chronc GVHD
Endometritis is
Ascending polymicrobial infection of the cervic and uterus
Endometritis imaging characteristics
Increasing fluid and gas in endometrial cavity in postpartum patient with fever and pelvic tenderness. Findings can be normal frequently
CT
Thickening, fluid gas
parametrial inflmmation/collection/pyosalpinx
MRI
Thickened endometrial cavity, fluid or gas
Contrast enhanced MR for collections
US
uterus may be enlarged, tender
thickened heterogenous endometrium. Endometrial fluid and echoes.
Adnexal collections
Increased flow
Endometritis pathology
Post partum usually polymicrobial
Not related to pancrea; chlamydia, gonorrhea, genital mycoplasms, aerobic/anaerobic vaginal flora
Clinical
Enlarged, tender post partum uterus. Fever WCC/
Non post partum: lower abd pain, dysparuenia, fever, back pain and vomiting. Adnexal tenderness on bimanual
Endometritis differentials
RPOC: Echogenic endometrial mass. High vel low resistance flow doppler.
Clot and debris
Normal gas in cavity
Gartner duct cyst is
Embryologic mesonephric duct remnant. Simple anterolateral upper vaginal wall cyst.
Gartner duct imaging characteristics
Simple appearing cyst. Anterolateral upper vaginal wall. Above PS/perineal membrane
US
Simple anechoic vaginal wall cyst.
May have internal echoes/debris
Peripheral Doppler flow with inflammation/infection
CT
Low attenuation non enhancing
MR
T1 hypo
T2 hyper
May be atypical if infected, haemorrhagic, proteinaceous
Nodular enhancement suggests rare malignant transformation
Urethral diverticulum are
Uni or multilocular lesions adjacent to and often surrounding urethra.
Majority are acquired arising from infected/inflamed periurethral skene glands
Usually asymptomatic and incidental. May cause UTI sx, dribblings, dyspareunia
Urethral diverticula imaging characteristics
MR
T2 hyperintense
T1 hypointense
Diverticular neck may be visualised
Irregular wall enhancement or mass like components may suggest infection/malignancy
US
Well marginated anechoic periurethral cystic lesions
peripheral doppler flow suggests infection
CT
hypointense periurethral cystic lesion
may opacify post void
may see complicating stones
Fluoro
Urethral diverticula differentials
Bartholin cyst - posterolateral vaginal introitus
Skene gland cyst - anterior vaginal introitus at external meatus
Gartner duct cyst - classically anterolateral vaginal wall above the pelvic diaphragm
Urethral tumour - solid, expands urethra
Vaginal fistula is
an epithelially lined communication between the vaginal lumen and adjacent pelvic organs
types; vesico, colo/entero, recto/ano
Simple (single tract) or complex (multiple tracts).
Vaginal fistula causes
Obstetric trauma
Gynae/urologic procedures
Inflammation (Crohns)/ infection
Pelvic malignancy (bladder, cervical, endometrial)
Radiation therapy, 20 years post
Vaginal fistula imaging characteristics
Fluoroscopy
communication between the vaginal lumen and other pelvic organ, specific to type
CECT
Enteric contrast in vagina with bowel associated
Vesico or ureterovaginal fistula confirmed with contrast in vagina on CT cystography or urography
MR
Superior modality owing to multiplanar capabilities and soft tissue contrast
Abnormal T2/STIR linear hyperintensity
Surrounding low T2 fibrous wall
Low signal intensity tract with enhancing wall on T1+C
Cervical stenosis is
Canal narrowing from benign or iatrogenic source.
When severe, results in hydrometra, pyometra or haematometra.
Cervical canal narrowing <2.5-3.5mm
Cervical stenosis pathology
Any process that results in inflammation, erosion, repair and regeneration of cervical mucosa
In post menopausal women usually age related atrophy
Recognised complication following D/C, radiation therapy, cone biopsy and cervical amputations, radical tracheloectomy
Cervical stenosis imaging
Thickened cervix, fluid within endometrial canal
May see ancillary signs to suggest etiology eg thickened bowel post radiotherapy
May see dilated blood filled fallopians
Loss of normal cervix zonal architecture if radiation or old
Cervical stenosis differentials
Obstructed uterus secondary to malignancy
Obstructed uterus secondary to mass effect - cervical or submucosal leiomyoma or other pelvic mass causing compression/obstruction
Congenital abnormalities - imperforate hymen, complete transverse vaginal septum, cervical atresia, vaginal atresia
May have associated haemtocolpos and haematometra
Kidneys should be evaluated
Mayer Rokitansky Kuster Hauser syndrome is
congenital anomaly characterised by vaginal agenesis associated with spectrum of other GU tract abnormalities.
Two forms
A: congenital absence of the uterus and upper 2/3 vagina with normal ovaries and fallopian tubes
B: includes associated abnormalities of the ovaries and fallopians rubes, as well as renal anomalies
Mayer Rokintansky Kuster Hauser syndrome pathology
Arrested development of the paramesonephric ducts at 7 weeks.
Normal external genitalia and absence or reduced development of the uterus and upper two thirds of the vagina
Upper vagina, uterus, cervix and fallopian tubes from Mullerian ducts from 8-12w.
KUB concomitantly at 6-12 w.
Assoc: vertebral anomalies, renal anomalies (agenesis, ectopic kidney, fused kidney, renal hypoplasia and horseshoe kidney
Adenoma malignum is
Subtype of mucinous adenocarcinoma of cervix , termed malignum due to virulent and fatal progression
Adenoma malignum pathology
Well-differentiated endocervical glands that extend from surface to deeper portion of the cervical wall.
Presents with cluster of cysts, deceptively benign on histo
Associated with
Peutz jeghers syndrome
Mucinous ovarian tumours
Ovarian sex cord tumours with annular tubules
Endometrial hyperplasia is
Excessive proliferation of endometrial glands with increased ratio of glands to stroma
Endometrial hyperplasia imaging
Endometrial thickening with well defined myometrial interface
Focal or diffuse
May have cystic change
MRI
T1: usually not seen
T2: diffuse thickening of striple, iso/hypo to normal endometrium. May hve cystic change
C+FS: hypo to myometrium early, iso to hyper to myometrium late
US
Diffusely thickened, homogenous
May show cystic swiss cheese change
If atypical hypoechoic/heteerogenous areas
Multiple vessels, sparse vasculairty
Endometrial hyperplasia cutoffs
Premenopausal
>8mm proliferative
>16mm secretory
Post menopausal with bleeding >5mm
Postmenopausal without bleeding >8-11mm
Endometrial hyperplasia differentials
Secretory endometrium
Endometrial carcinoma - may coexist. irregular thickening/mass. ill-defined margins. myometrial invasion.
Endometrial polyp - may coexist. sessile polyps may mimic focal. Separate endometrial lining. Single feeding vessel in pedunculated. Fibrous stalk MR.
endometritis - hypervascular, fluid in cavity, adnexal changes of PID
Submucosal leiomyoma - focal hypoechoic thickening of endometrial echo complex. MR can easily differentiate.
Endometrial hyperplasia pathology
Unopposed estrogen; chronic anovulatory, exogenous, tamoxifen, obesity, secreting tumours
Assoc; endometrial polyp, endometrial cancer
post menopausal bleeding
Divided into hyperplasia with and without atypia. 25% with have or will have carcinoma.
Endometrial cancer epidemiology
Most common gynae cancer. Peak incidence 6th decade.
Risk factors: estrogen exposure; replacement, PCOS, tamoxifen, obesity, early menarche/late menopause, nulliparity, secreting tumours, DM
Assoc: HNPCC, complex hyperplasia
Endometrial cancer pathology
Majority adenocarcinoma
Divided into type 1 and 2
Type 1: 80%. unopposed estrogen and endometrial hyperplasia. Obese mid 50s women. Well diff, slow progression. PTEN gene mutation 3-80%
- endometrioid carcinoma
Type 2: 20%. Endometrial atrophy. 70ish. p53 mutation 50%. Less differntiation and spreads early
- papillary serous carcinoma
- clear cell carcinoma
- adenosquamous carcinoma
- adenocarcinoma with squamous diff
= undifferentiated/small cell
Endometrial cancer imaging
US
Thickening or polypoid mass
premenopausal; varies with cycle. >16 secretory, >8 prolif
post menopausal >5, or 8 if HRT/tamox
Suggestive US features; heterogenous, irregular. polypoid mass. intrauterine fluid. frank myometrial invasion. Disruption of subendometrial halo
CT
Mets
MRI
T1: hypo to iso to normal endometrium
C+: hypoenhancing. delayed for stromal invasion.
t2: iso to hypo relative to normal. heterogenous. hypointense to myometrium
DWI: restricted
Endometrial cancer FIGO
stage 1: tumour confined to the uterus
stage 1a: an invasion of less than half of the myometrium
stage 1b: an invasion of the outer half of myometrium
stage 2: tumour extends to the cervical stroma
stage 3: tumour extends beyond the uterus
stage 3a: tumour invades the serosa or adnexa
stage 3b: tumour invades the vagina or parametrium
stage 3c: pelvic/para-aortic lymph node involvement
3c1: pelvic lymph node involvement
3c2: para-aortic lymph node involvement\
stage 4: bladder/rectal invasion or distant metastases
stage 4a: tumour extends into adjacent bladder or bowel
stage 4b: distant metastases
Endometrial cancer differentials
Benign proliferation
endometrial hyperplasia
endometrial polyp
Submucosal uterine leiomyoma
DDX advanced
Uterine sarcoma - endometrial sarcoma, leiomyosarcoma, malignant mixed Mullerian tumour
Uterine lymphoma
Cervical cancer with invasion
Mets
Fibroids are
Benign tumours of uterine smooth muscle cells
Fibroid types
Location
Intramural: most common, surrounded by myometrium
Subserosal: deep to and abutting serosa. sessile or pedunculated. May grow laterally through broad lig folds. Can tort and infarct.
Submucosal: deep to endometrium. Sessile or pedunculated. Stretches over endometrium or projects into cavity. Can pass through cervix. Odten symptomatic, irregular bleeding and infertility
Fibroid imaging
HSG
Mass effect on endometrium
CT
Hypo or homogenous enhancement
can calcify
MRI
T1 iso to myometrium
T2: homogenous, hypointense to myometrium. pseudocapsule. hyperintense rim, edema,lymph, veins
C+: solid enhancing, variable, enhancing halo
US
homogenous, hypoechoic, shadowing
peripheral flow, decreased central flow
have have stalk vessel in pedunculated or bridging vessel sign in subserosal
Fibroid differentials
Adenomyoma: poorly marginated and intermediate T2. Punctate hyperintense T1/T2 foci. Ill defined endometrial/myometrial junction and contiguous with junctional zone
Malignant uterine neoplasm; leimyosarcoma. irregular and indistinct. heterogeneous t2 and post con signal. rapid growth
Contraction
Ovarian fibroma
Fibroid degeneration
Hyaline; focal or generalized
Cystic
Myxoid
Red/carneous; due to haemorrhagic infarct
Not degenerative but other types
parasitic; subserosal torted off
broad lig and cervical
diffuse
lipoleiomyoma
pyomyoma - suppurative
Pyomyoma is
Suppurative leimyoma. Presents with sepsis, bactaraemia, leiomyoma.
Pyomyoma imaging
Gas and internal debris. Heterogenous in attenuation with regions of degeneration associated with parametrial inflammation
Intramural/submucosal/subserosal
MPR helps identify pyomyoma rupture with discontinuity of wall
Uterine AVM pathology (etiology and assoc)
Etiology:
Congenital (rare)
Acquired; post traumatic or infectious
Risk factors: D/C, IUD, pelvic surgery, infection, GTD, carcinoma, diethylstilbestrol exposure
Assoc:
pseudoaneurysm
Uterine AVM imaging
US
Small anechoic spaces distributed uniformly producing spongy myometrial echotexture
No mass effect
2 mosaic patterns of colour; flow reversal and colour aliasing
High flow low resistance arterial flow
Prominent parametrial vessels
MR
Bulky appearance, focal or diffuse disruption of the junctional zone
Multiple serpentine flow related signal voids
No well defined mass or margins
MRA: enlarged arteries, vascular network, early venous filling
Angiographic
Complex tangle of vessels
Hypertrophied feeding uterine arteries
Early venous drainage
Uterine AVM ddx
Gestational trophoblastic disease - positive bhcg, overlapping features, may coexist
Endometrial carcinoma - neovascularity, low volume, high velocity
RPOC - bhcg, endometrial based mass, overlapping doppler characteristics
Pelvic varicosities - normal venous waveforms
Uterine haemangiomas - phleboliths
Ovarian torsion is
Twisting of the vascular pedicle on axis of infundibulopelvic and uteroovarian ligaments. Leads to venous and lymphatic congestion, arterial obstruction and infarction.
Torsion imaging
Enlarged oedematous ovary displaced
>4cm in one dimension or >20cc premeno 10cc post meno
Heterogenously echogenic appearance of the ovary
US
enlarged, heterogenous, hyperechoic
peripherally displaced follicles
displaced to midline
cystic or solid lesion as lead point
twisted vascular pedicle
free fluid/haematoma
probe pain
Doppler
whirlpool along pedicle
pulsed doppler variable findings
CT
Enlarged cyst or mass may be present
Twisted vascular pedicle is specific
Free fluid
May have subacute haemorrhage
MRI
T1 hypointense. can be hyper if subacute haemorrhage, or haemorrhagic lesion
T2 increased due to oedema. hyperintense follicles
C+ variable
Torsion ddx
Haemorrhagic cyst; highly variable appearance. typicall avascular with reticular fishnet pattern. can have retractile clot, fluid level, debris
Ectopic pregnancy; positive bhcg. Tubal mass with thick echogenic ring
Isolated fallopian tube torsion; FT thickened walls,internal haemorrhage
PID; complex fluid collection with pseudo solid appearing components. hyperaemia.
Torsion pathology
Follicular cyst most common
Mature cystic teratoma most common tumour
Presentation
acute onset sharp pelvic pain, n/v
Demographics
reproductive years, 20% pregnant
2-3% gynae emergencies
increases with ovarian stimulation
Prognosis
If untreated, infarction
Spontaneous torsion detorsion leading to ovarian oedema
Treatment
surgery; detorse vs remove
Follicular cyst - definition, imaging, ddx, clinical
Hormone dependent functional ovarian cyst. Arrested follicular development with subsequent cyst formation.
Imaging
Well marginated round with thin wall
2-8cm solitary
Peripheral rim of compressed ovarian parenchyma, often with other smaller developing follicles
US: anechoic, avascular, simple apearing. varied if haemorrhagic
MR: T1 low T2 high. No central enhancement. Thin smooth enhancing wall without nodularity of papillary projections. Varied central signal if haemorrhagic.
CECT: fluid density, no central enhancement
DDX
endometrioma
CL cyst
Paraovarian cyst
Surface epithelial tumour
Clinical
Majority of simple cysts in premenopause are follicular and regress in 2 cycles
Corpus luteal cyst - definition, pathology, imaging, ddx, clinical
Cystic dilatation of normal physiological corpus luteum
Path
Round yellow with lobulated margins
Thickened and irregular
Serous or serosanguinous internally
Wall becomes luteinised; granulosa lutein, theca lutein and k cells
Menstrual cycle:
Follicular - FSH, dominant follicle, estradiol, LH surge, ovulation
Luteal - Ovulation, CL, progesterone, CL increases then involutes to corpus albicans
Imaging
Unilocular cyst wih irregular thick crenelated vascular wall
Mimic solid when collapsed
MR
thickened irregular enhancing wall
Variable signal due to haemorrhage
No internal enhancing papillary projections or mural nodularity
US
anechoic with thickened echogenic wall
posterior enhancement
variable due to haemorrhage
ring of fire vascularity
typically <3cm
DDX
Ectopic
Endometrioma
Primary ovarian neoplasia
Ovarian abscess
Clinical
Asx, can have rupture and haemorrhage
Majority regress in 2 months
Theca lutein cysts - defintion, imaging, ddx, clinical
Multiple theca lutein cysts in associated with increased levels of or abnormal ovarian respone to bhcg
Imaging
Bilaterally enlarged ovaries with multiple cysts of variable sizes
Hypervascular central uterine mass if molar
typically 6-12cm
preservation of underlying ovary
thin walled cysts
No nodules or solid components
Spoke wheel ovaries
DDX
ovarian epithelial neoplasm
PCOS
OHSS
Clinical
Usually asx, can rupture or tort
typically regress after causative factor
Haemorrhagic cyst - definition, imaging, ddx, clinical
Haemorrhage into cystic space in ovarian pathology
Imaging
Avascular hypoechoic ovarian mass with fine, lacy internal echoes
Most resolve 6-12 weeks
Mass like complex adnecal without vascularity
If ruptures, haemoperitoneum
DDX
Endometrioma - more uniform echoes
Solid ovarian mass - papillary projections more likely than angular fragments
Torsion - can be painful, haemorrhagic
Clinical
Most asx and resolve
Larger - pain, acute abdo
Surgery for severe - rupture or torsion
PCOS IS
Polycystic ovarian morphology with clinical and endocrine dysfunction. Must exclude other etiologies of hyperandrogenism and menstrual disturbance.
PCOS imaging
> 25 follicles, >10mL volume
Usually bilateral, may be unilateral
Other: >9follicles per section. String of pearls appearance.
MRI
T1 low follicles, stroma isointense to myometrium
T2 small subcapsular hyperintense follicles. low signal ovarian cortex and central tissue.
C+ follicle rim enhancement. enhancement of vascularised stroma.
US
FNPO >25, sesn 85 sp 94
volume 10cc
Increased stromal volume and echogenicitiy
Endometrial thickening - homogeneous or heterogenous, can have cystic change. due to unopposed estrogen.
Increased stromal blood flow and vessel calibre, decreased RI PI
PCOS ddx
Normal ovaries with multiple functional cysts
Polycystic ovaries. absent clinical and biochemical features
Multifolliclar ovaries - incomplete pulsatile gonadotropin stimulation. assoc w hyperprolactin, hypothalamic anovulation, weight related amenorrhea. Normal LH and T, decreased FSH. Mid to late normal puberty. Fewer follicles.
Pelvic congestion - prominent ovaries, polycystic pattern to clusters of 4-6. Enlarged uterus thick endometrium.
OHSS
Dermoid cysts are
Congenital cystic tumour composed of well differentiated derivations from at least 2/3 germ cell layers. Classified as benign ovarian germ cell tumour.
Dermoid cyst imaging
CT
Fat is diagnostic
Teeth or calc in half
May see floating mass of hair
Dermoid plug in wall
Enhancement of solid component may occur in benign
MRI
T1 - heterogenous. Bone low, fat high. Can have chemical shift artefact.
T1FS - suppression is diagnostic
T2 - variable
SWI - restricting, due to keratinoid substance
C+ - enhancement can occur in benign
US
Dependant on size of plug, calcific elements and histo
May be echogenic or cystic
3 most common;
- cystic with echogenic shadowing nodule projecting inward (Rokitansky)
- Tip of iceberg; Echogenic with sound attenuation owing to sebaceous material or hair within cavity
- Dermoid mesh; multiple thin dot dash caused by hair
Shadowing calcific structures
Fluid fluid level with sebaceous and serous
Floating nodules
Complications
- torsion
- rupture
- malignant transformation
- pseudomyxoma peritonei
- infection
- paraneoplastic encephalitis
Dermoid cyst differentials
Endometriomas
Bowel - gas/faeces may mimic rokitansky
Haemorrhagic cyst
Pedunculated lipoleiomyoma
Immature teratoma - predominantly solid that contain fatty elements, irregular calcifications and numberous cysts
Dermoid cyst pathology
Cavity filled with sebaceous material liquid/semisolid
Surrounding firm capsule
Usually unilocular
1 or more rokintansky nodule
Micro
Well differentiated derivatives of 3 germ layers
Orderly arrangement of tissues
Squamous epithelium lined walls of cyst
Compressed ovarian stoma
Hair, skin, muscle within wall
Strumi ovarii
Anembryonic pregnancy is
Form of failed early pregnancy with a gestational sac but the embryo does not form
Anembryonic pregnancy pathology
Blastocyst formed from fertilised ovum
Fetal pole never develops
bHCG formed due to syncytiotrophoblast invasion into endometrium
Anembryonic pregnancy imaging
No embryo in gestational sac MSD >25mm
OR
No embryo on follow up scan
- 11 days after showing sac with yolk sac but no embryo, or
- 2 weeks after scan showing gestational sac with no yolk sac or embryo
Ancillary
- Absent yolk sac MSD >8mm
- poor decidual reaction
- irregular sac shape
- low sac position
Anembryonic pregnancy differentials
Early pregnancy
Pseudogestational sac
Gestational trophoblastic disease
Ectopic pregnancy pathology
Epidemiology
- 1-2% pregnancies.
- 18% of 1st trimester w bleeding
- assoc with IVF
Locations
- tubal (ampullary, isthmal, fimbrial)
- Interstitial/cornual
- Ovarian
- Cervical
- Scar ectopic
- Abdominal ectopic
RF
- IVF
- Prior ectopic
- Tubal surgery/injury
- PID
- SIN
- endometrial injury
- IUD
- Endometriosis
- Previous placenta previa
- Congenital uterine anomalies
- smoking
- maternal age
bHCG increases at a slower rate, 50% or less in 48 hours
Ectopic pregnancy imaging
Empty uterus
pseudogestational sac
decidual cast
thick echogenic endometrium
Complex adnexal cystic mass 95% with empty uterus
Simple 10
Ring of fire
Live extrauterine
Free pelvic fluid or haemoperitoenum
- positive bhcg, fluid and empty uterus 70%
Special ectopic key features (interstitial, ovarian, cervical and c scar)
Interstitial
- 2-4%
- higher risk of rupture, massive haemorrhage, uterine rupture
- interstitial line sign (echogenic line from mass to endometrial echo complex
- eccentric gestational scar, <5mm myometrium in all planes
Ovarian
- <3%
- Wide echogenic outer ring
- on or in ovary
- Unable to separate on pressure
Cervical
- <1%
- sac within the distended cervix, hour glass appearance of the uterus
- abnormally low sac position
- hyperechoic reaction
- internal os usually closed
- consider miscarriage in progress; no HR, open internal os, sliding sac sign, loss on repeat us
C section
- empty uterus and cervical canal
- anterior lower part of uterine segment
- absence of myometrium between bladder wall and gestational sac
- methotrexate injections, high surgical morbidity
- uterine rupture and massive haemorrhage
- ddx; anterior cervical ectopic, prominent c scar tissue, miscarriage in progress
Pregnancy unknown location definition and differentials
Neither uterine or ectopic prengnancy identified on TVUS in setting of positive BHCG
DDX
- early
- non viable not detected
- complete miscarriage
- unidentified ectopic
Failed pregnancy criteria
Diagnostic:
MSD>25 - no embryo
CRL >7 - no FHR
Absence of embryo with HB >14 days after GS no YS
Absence of embryo with HB > 11 days after GS with YS
Sac, no embryo, MSD <12mm that fails to double >14 days
Sac no embryo and MSD >12mm with no FHR >7 days
Embryo no FHR and no FHR >7days
Cessation of previous FHR
Suspicious:
CRL <7mm no FHR
MSD 16-24 no embryo
Absent embryo with FHR 7-13 post GS wo YS
Absence of embryo with FHR 7-10 post GS w YS
No embryo >6weeks LMP
No embryo when amnion visible
Embryo present with amnion visible but no FHR
Small GS in relation to embryo <5mm
Enlarged YS >7mm
Complete miscarriage is
cessation of vaginal bleeding with no RPOC or GS in women with previous IUP
Inevitable miscarriage is
Open internal os, bleeding, 1st trimester. Most often intracervical contents. Migration on serial scans. Progression of a threatened miscarriage once cervix opens
Perigestational haemorrhage is
Haemorrhage around fetus during gestation. Spectum includes
- chorionic; separation of chorion from endometrium
- periplacental; subchorionic/preplacental, retroplacental
- placental
Epidemiology
2% pregnancies <10weeks
20% of those have vaginal bleeding <10 weeks
Prognosis
- 90% success rate if living and small
- large >50%, 25% loss rate
- haemorrhage with embryonic bradycardia 80% loss rate
Subchorionic haemorrhage
perigestation between uterine wall and chorionic membrane
US
crescentic collection and elevation of chorionic membrane
variable echotexture
extension toward placental margin
Small <30%, medium 20-50, large >50%
Prognosis
increased risk of abruption and preterm labour
poor prognosis if extension to internal os
ddx
retroplacental
marginal can mimic twin gestational sac
chorioamniotic separation
Physiological gut herniation
Natural phenomenom from 6-13 weeks
Intestine elongates and moves outside of embryonic abdomen into base of umbilical cord at 8 weeks
Midgut rotates 90 CC
10-11 weeks intestines return, then rotate additional 180 CC
Physiological gut herniation imaging
bowel outside anterior abdominal wall at base of umbilical cord
not seen after 12-13 weeks
no other organs
extent of hernia relatively small
DDX
gastroschisis - right side of umbilical cord
omphalocoele - midline, membrane, small AC, direct UC insertion
Nuchal translucency is
Normal fluid filled space at the back of the fetal neck 11 weeks to 13 weeks 6 days.
Increased NT thoguht to be related to dilated lymph channels. Non specific sign of generalised getal abnormality.
Thickened NT associations
Aneuploidy
- trisomies
- turners
Non aneuploidy structural defects and syndromes
- congenital heart disease
- Noonan syndrome
- CDH
- Omphalocoele
- skeletal dysplasias
- Smith lemli opitz syndrome
- VACTERL assoc
Miscarriage or fetal demise
Intrauterine infections; Parvo B19
NT technique
Midsag
- VC down
- tip of nose/nasal bone, hard palate, diencephalon
Magnificantion
- only head and upper chest
No extension or flexion
Free floating fetus
Calipers inside hyperechoic edges, widest part measured
NT assessment
CRL between 45 and 84, 11 weeks 3 days to 13 weeks 6 days
No septations
<2.2-2.8mm normal, needs to be age/CRL matched
Interpretation
<2mm <1% risk
3.4mm 7% risk
3.5-4.4mm 20% risk
5.5-6.4mm 50% risk
>8.5mm 75% risk
Correlate with
bhcg
afp
PAPP A
oestrriol
Further workup
Increased risk of less than 1/300, further on patients desrire after counselling
Amniocentesis and or chorionic villus sampling
fetal echo
Hydrops fetalis is
excessive extravasation of fluid into the third space of a getus which could be due to heart dailure, overload, decreased onc pressure or increased permeability.
At least two components of;
- pleural effusion
- pericardial effusion
- ascites
- generalised body oedema
- placental enlargement
- polyhydramnios
- hepatomegaly
Hydrops fetalis pathology
Immune or non immune cardiac failure
Immune
- 10%
- fetomaternal incompatibility, including rhesus
Non immune
- chromosomal anomalies
- cardiac causes (rate, congenital anomalies, tumours)
- twin related complications (transfusion syndrome, reversed arterial perfusion sequence)
- in utero infections (TORCH, PB19, coxsackie)
- fetal tumours (sacrococc teratoma, hepatic haemangioendothelioma, placental chorioangioma)
- in born errors of metabolism (gaucher, niemann pick)
- fetal hypoprotein states
- congenital anaemia
- skeletal dysplasia
- high output flow states (vein of galen aneurysmla malformation)
- thoracic/pulmonary (primary hydrothorax, CPAM, CDH, PS)
Placenta praevia is
abnormally low lying placenta to the internal cervical os. common cause of antenatal haemorrhage
Placenta praevia risk factors
Previous PP
Previous CS
Increased maternal age
Increased parity
Large placentas
Smoking
Assisted conception
Placenta praevia association
abnormal placental villous adherences
Placenta praevia classification
1: low lying - 20-5mm
2: marginal - reaches internal os, doesnt cover
3: partial - partially covers
4: complete - completely covers
Placental abruption is
premature separation of normally implanted placenta after 20 w and before 3rd stage of labour. Potentially fatal. Cause of 3rd trimester bleeding.
Placental abruption risk factors
Preeclampsia
Prior abruption
PROM
Maternal age
Maternal trauma
Smoking
Cocaine/amphetamine
Thrombophilia
Chorioamnionitis
Short cord
Multiparity
Placental abruption clinical
Board like abdo tone
Bleeding, can be concealed
Uterine contractions
lumbar pain
Maternal/fetal compromise
Placental abruption pathology
Likely rupture of a spiral artery with haemorrhage into the decidua basalis leading to separation.
Classification:
Marginal
Retroplacental
Preplacental
Placental abruption imaging
US:
Retroplacental haematoma
Interplacental anechoic areas
Separation and rounded edges
Placental thickening
Thickening of retroplacental myometrium
Disrupted placental circulation
Intra-amniotic echos
Blood in fetal stomach
Intermembranous clot (twins)
Placental abruption complications
IUGR
Fetal demise
Maternal exsanguination
Placental abruption ddx
Isoechoic:
Contraction
Placentomegaly
Hypoechoic:
leiomyoma
Subplacental space
Placental variations
Single lobe, bilobe, succenturiate, circumvallate, circummarginate, placenta membranacea, placenta fenestrata, zonary, annular, ring, shorse shoe
Bilobed placeta
Two near equal sized lobes. If smaller, succenturiate.
Assoc: velamentous cord insertion, increased risk vasa previa II, post partum haemorrhage
Succenturiate lobe
Smaller accessory lobe. Check for vascular connection over os (vasa previa)
Complications: type II vasa previa, post partum haemorrhage
Circumvallate placenta
Small chorionic plate, inward insertion of membranes from the edge to the centre (placental shelf)
Complications; increased risk abruption, IUGR
ddx: amniotic shelf, amniotic band close to placenta
Circummarginate placenta
Similar to circumvallate but not inward rolling. Chorionic membranes insert inward from margin of placental edge.
Placenta membranacea/diffusa
placenta develops as thin membrane around entire periphery of chorion
associations; abnormal adherence
complications; praevia, IUGR, antepartum haemorrhage, miscarriage, fetal demise, post partum haemorrhage
Types of cord insertion
Central
Eccentric - lateral, >2cm
Maringal - <2cm
Velamentous - on chorioamniotic membranes
Marginal cord insertion is
insertion of the cord <2cm from placental margin. also known as battledore.
7% single preg but 25% twins
Marginal cord associations
IUGR, monchorionic twins, abruption, preeclampsia, nuchal cord entanglement, preterm
can progress to velamentous
Velamentous cord insertion pathology
Epidemiology
1% single and 9-15% twins
More common in praevia
Path
Cord inserts into the chorioamniotic membranes outside the margin then travels within membranes to the placenta. Thought to be remodelling of placenta as response to factors that affect blood flow (trophotropism). May evolve from marginal.
Associations
bilobed, twin preg, uterine anomalies, IUCD, single umbilical artery, praevia
Complications
Type 1 vasa previa
IUGR
twin growth discordance
Twin twin transfusion
Vasa praevia is
aberrant fetal vessels crossing over or in proximity to internal cervical os, ahead of presenting fetus, within membranes without support of placenta. Unsupported by Whartons jelly and risk of labour rupture.
Vasa praevia types
1: vessels connect velamentous cord insertion to placenta
2: vessels connect portions of bilobed of succenturiate lobe placenta
Vasa praevia risk factors
multiple gestations
low lying
succenturiate lobe or bilobed
ivf preg
Vasa praevia imaging
vessels over os
Placental lakes are
formation of hypoechoic cystic spaces centrally within placenta. Can be abnormal if diffuse or in very early pregnancy
Placental lake imaging
well defined hypoechoic regions
low velocity intraplacental laminar flow
ddx; chorioangioma; marked internal vascularity
Gestational trophoblastic disease is
Abnormal proliferation of trophoblastic tissue
Encompasses:
Hydatiform mole
- complete
- partial
- coexistent
Invasive mole
Choriocarcinoma
Placental site trophoblastic tumour
Epithelioid triphoblastic tumour
Hydatidform mole pathology
Subtypes
Complete or partial, absence or presence of a fetus
complete; absence
partial; with an abnormal fetus or fetal demise
Can coexist
Chromosomal composition
Complete 46XX diploid chromosomal pattern. single sperm egg lost chromosomes
Partial 69XXY egg two sperm
Location
Uterine cavity, rarely fallopian tubes or ovaries
Cluster of grapes, chorionic villi into mass of clear vesicles
bHCG exceeding those in pregnancy
can become invasive or turn to choriocarcinoma
Complete mole summary
Commonest, benign GTD
Absence of fetal parts
Non invasive diffuse swelling of the chorionic villa
most have 46XX diploid chromosomal pattern, some 4XY
elevated bhcg
US
enlarged uterus
intrauterine mass with cystic spaces and no fetal parts
snowstorm, bunch of grapes
bilateral theca lutein cysts
high velocity low impedance
MR
heterogenous mass with cystic spaces distending uterus
fetal parts absent
uterine zonal anatomy often distorted
Partial mole summary
Focal distribution, slower transformation, embryo presence and triploid karyotype
most 69XXY, then 69XXX then 69XYY
US:
enlarged placenta relative to uterine cavity
cystic spaces in placenta
amniotic cavity, empty or amorphous small fetal echoies
well formed but growth retarded fetus, either dead or alive with hydropic degeneration
can be similar to complete mole or missed abortion
high velocity low impedance
ddx
fetal demise with placental hydropic change
twin pregnancy with coexistent mole
placental mesenchymal dysplasia
Invasive mole is
tumours growth associated with gestation, under GTD spectum. Locally invasive, non metastasising neoplasm.
Invasive mole path
10-20% post molar evac and infrequently after other. penetrates and perforates uterine wall
Path
arise from hydatidiform moles. invasion of the myometrium by hydropic chorionic villa, accompanied by the proliferation of trophoblast. locally destructive and may invade parametrial tissue and vessels
bhcg markedly elevated
Invasive mole imaging
US
echogenic vascular mass invading myometrium. high velocity low impedance
MR
poorly defined mass that deeply invades myometrium. complete or partial disruption of the junctional zone.
T1 isointense to myometrium with foci of high signal haemorrhage
T2 mixed
IUGR is
EFW and/or AC below the 3rd percentile
OR
AC below the 10th percentile with deranged Doppler parameters
IUGR aetiology
Maternal conditions; smoking, alcohol, diabetes, malnutrition, vascular conditions
Placentral insufficiency
Single umbilical artery
Fetal; multifetal, infection, chromosomal, mosaicism, syndromic anomalies, in utero exposure
IUGR imaging features
Non Doppler features:
Reduced AC and or EFW
- AC and or EFW <3rd
- AC and or EFW <10 with deranged Dopplers
Oligohydramnios without membrane rupture
Increases HC to AC ratio
Advanced placental grade
Doppler features:
UA PI >95th, absent or reversed diastolic flow
UV pulsatility
Uterine A PI >95th, notching in mid to late
CP ratio <5th
IUGR complications
Antepartum
- stillbirth
- iatrogenic prematurity
- abruption
- perinatal stroke
Intrapartum
- abnormal fetal status
- asphyxia
- emergenct c section
- active neonatal resus
- perinatal stroke
Neonatal
- hypothermia
- hypoglycaemia
- hypocalcaemia
- ploycythemia
- sepsis
- coagulopathy
- hepatocellular dysfx
- RDS
- NEC
- IV haemorrhage
- HIE
Paediatric
- risk of short, palsy, delay, emotional problems, lower IQ, chronic lung disease
Symmetric IUGR is
IUGR when all fetal biometric parameters tend to be less than expected for the given age
May present at an earlier stage in gestation
Aetiology;
- aneuploidy
- infections
- Other; topical fluoro glucocorticoid, external agents (nicotine, alcohol, heroin, radiation)
Normal HC AC and FL AC ratios
Asymmetric IUGR is
IUGR when some parameters are disproportionately lower than others. Classically AC.
Tends to present later, and is commoner
Relative preservation of the fetal brain due to fetal head sparing theory, characterised by an increased brain to liver ratio. Decreased SC fat. Rarely maternal coke has HC out of proportion.
Aetiology
Placental insufficiency
Pre-eclampsia
Assoc; Russell Silver syndrome.
Concurrent karyotopic abnormalities is low esp if detected late
Features
AC clasically reduced out of proportion and below 10th
BPD and HC may be normal
Increased HCAC ratio
Fetal tachycardia may be present
Oligohydramnios may be present
Fetal macrosomia is
EFW >90th percentile
Macrosomia features
Often truncal obesity and AC first parameter to increase
Macrosomia treatment and prognosis
Early delivery or elective c section.
Complications related to issues with delivery and include; birth truam, birth asphyxia, neonatal hypoglycaemia and meconium aspiration
Fetal aenamia causes
Haemolytic
- ABO incompatibility
- Rh incompatability
Infection
- PB19
Haemopoetic
- homozygous alpha thalassemia
Syndromes
- Aase
- Fanconi
Tumours
- placental chorioangioma
- infantile haemngioendothelioma
- sacrococcygeal teratoma
Fetal anaemia imaging
US
- hepatomegaly and/or splenomegaly
- CV compromise and development of hydrops
Doppler
- increased MCA time average mean velocity
- increase in MCA PSV
- changes in doppler mca 88% sensitive and 82% specific
Oligohydramnios imaging
AFI <5
Max vert pocket <2
Two diameter pocket <15cmsq
Oligohydramnios causes, associations, complications
DRIPPC
Demise
Drugs (indometacin)
Renal abnormalities
IUGR
PROM
Post dates
Chromosomal
Associations:
Potter sequence
Underlying fetal hypoxia and CV compromise
Twin pregnancy related complications
Maternal dehydration
Poor prognostic marker
Amnioinfusion can be attempted if severe and indicated
Complications; pulmonary hypoplasia, limb contractures
Polyhydramnios is
When amniotic fluid is more than expected for age
AFI >25cm
MVP > 10cm
AFV >1500-2000mL
Two diameter pocket >50cmsq
Classification
Mild: AFI 25-30
Moderate: 30-35
Severe >35
Polyhydramnios pathology
Path; icnreased production or decreased removal of amniotic fluid. Vast variety of disorders.
Aetiology:
Idiopathic - majority
Maternal
- 25-30%
- diabetes
- CHF
Fetal
- 10-20%
- CNS lesions
- Proximal GI obstruction
- fetal cervicothoracic abnormalities (cervical teratoma/epignathus, fetal goitire, CPAM, CHAOS, CDH)
- Fetal CV anomalies
- twin preg related
- hydrops
- skeletal abnormalities
Treatment
- diabetes control
- c section
- amnioreduction
- indomethacin
Complications
- maternal dyspnea
- PROM
- preterm
- abnormal presentation
- cord prolapse
- post partum haemorrhage
The spectrum of abnormal placental villous adherence describes
the degree to which there is invasion of chorionic villi into the myometrium due to defect in devidua basalis
Abnormal placental villous adherence pathology
Pathology
- accreta; mildest, attached to myomatrium but doesnt invade muscle
- increta; intermediate, partially invade myometrium
- percreta; severest, penetrate entire myometrium or beyond serosa
Abnormal placental villous adherence imaging
US
May have praevia
May have placental lacunae; moth eaten/swiss cheese appearance with turbulent flow
Abnormal colour doppler; turbulent flow, increased vascularity
Loss of retroplacental clear space
Reduced myometrial thickness
MRI
Praevia
Uterine bulging
Heterogenous signal
Dark intraplacental bands
Focal myometrial interruptions
Tenting of urinary bladder
Direct invasion
Cystic hygroma is
congenital macrocystic lymphatic malgormation at the cervico facial region, particularly posterior triangle
Cystic hygroma pathology
Fetal/infantile/paediatric . 0.2-3%.
Pathology
- maldevelopment of the lymphatic system and communication with the venous system of the neck
- endothelial lined cavernous spaces
- can be mixed with other vascular malformations
Most commonly occur at the neck, termed nucal hygroma.
Also axilla, mediastinum
Associations
Aneuploidy 65%
Non aneuploidy
- congenital cardiac; coarctation, hypoplastic left heart
- pentalogy cantrell
- apert syndrome
Cystic hygroma imaging
usually well circumscribed, fluid density. Maybe be infiltrative, uni or multilocular. Can be mixed density.
US
Nuchael cyst, septations, evidence of fetal anasarac/hydrops
CT
ill defined hypoattenutating mass
MRI
T1 low unless hamorrhage
T2 high
C+ no enhancement
Omphalocele is
a congenital midline abdominal wall defect at the base of the umbilical cord insertion with herniation of the gut and other structures out of the fetal abdomen
Omphalocele associations
Chromosomal anomalies
- trisomy 18 most common
- trisomy 13
- trisomy 21
- turners
- klinefleters
- pallister killian
Other syndromes
- Beckwith Wiedemann
- pentalogy of cantrell
- OEIS complex (omph, exstrophic bladder, imperf anus, spinal anom)
- lethal omphalocele celft palate syndrome
Other GI anomalies
CNS/cardiac/GU/MSK anomalies
- bladder/cloacal exostrophy
- omphalocele radial ray complex
Omphalocele imaging features
Multiple bowel loops in membrane covered defect
Direct insertion of cord into sac
Smaller AC
Polyhydramnios
Allantoic cyst
DDX
Pseudo omphalocele
Gastroschisis (usually right of midline)
Physiological gut herniation
Limb body wall complex (usually left of midline)
Umbilical hernia
Limb body wall complex features
Abdominoschisis, usually left sided
Ectopia cordis
Lower limb anomalies
Scoliosis
Exencephaly
Beckwth Wiedemann features
Macroglossia
Otic dysplasia
Omphalocoele
Localised giagantism
Hemihypertrophy
Cardiac anomalies
Pancreas islet cell hyperplasia
Organomegaly
Pentalogy of Cantrell features
Omphalocoele
Ectopia cordis
Diaphragmatic defect
CV malformations
Gastroschisis is
Extra abdominal herniation of fetal or neonatal bowel loops through a para umbilical wall defect
Gastroschisis pathology
Usually right side, no membrane, small bowel. Bowel lacks normal rotation and fixation to the posterior wall. Other structures might herniate.
Compromised vascular supply to the area may be causative, or possibly incomplete regression of hte right umbilical vein
Associated with other bowel problems. Possibly IUGR.
Gastroschisis imaging
herniated content right side of cord
AC smaller than expected
Bowel appears free floating
Accompanying oligo or polyhydramnios
Gastroschisis prognosis and diffys
Condition of bowel most important prognostic.
Intrauterine mortality 10-15%
Surgically managed after birth
Complications:
Obstruction
Perforation
Mecnoium peritonitis
Motility dysfunction
NEC
Short gut syndrome
Fistula
GORD
DDX
Omphalocele
Physiological herniation
Duodenal atresia is
a congenital malformation of the duodenum requiring prompt correction neonatally. Common, 1 in 5-10000
Duodenal atresia clinical
Early life, duodenal obstruction
Distension, vomiting, absent bowel movements
Usually just distal to ampulla, so bilious vomiting but can be non bilious
Duodenal atresia pathology
Failure of recanalisation of the bowel lumen that is a solid tube in early fetal life 11-12 weeks
Duodenal atresia associations
Down syndrome
VACTERL
Annular pancreas
Other atresias
Duodenal atresia ddx
XR
Duodenal stenosis
Duodenal web
Internal malrotation and midgut volvulus
These should show distal gas
US
Choledochal cyst
Omental cyst
Enteric duplication cyst
Oesophageal atresia is
Absent continuity of the oesophagus due to inappropriate division of the primitive foregut into the trachea and oesophagus. Most common oesophageal congenital anomaly
Oesophageal atresia clinical
Inability to swallow
Failure to pass NGT
Oesophageal atresia pathology, subtypes, associations
Failure of the primitive foregut to divide into the trachea and oesophagus 4th week.
Pathogenesis includes teratogenic effects of early antithyroid drugs in pregnancy
Mostly sporadic
Subtypes:
Proximal atresia with distal fistula
Isolated atresia
isolated fistula H type
Double fistula with intervening atresia
Proximal fistula with distal atresia
Assoc:
Other atresias
Annular pancreas
Pyloric stenosis
VACTERL
CHARGE syndrome
chromosomal anomalies such as tri 18 and 21
Oesophageal atresia imaging
XR
Dilated pharyngeal pouch
Presence of air distally implied fistula
Tube looping
Fluoro:
Particularly good for h type
US
Unexpected stomach position and shape
Other VACTERL anomalies
Polyhydramnios
Growth restriction
Jejunal atresia is
A congenital anomaly characterised by obliteration of the lumen of the jejnum. Site can be anywhere from lig of Trietz to the jejnuoileal junction. Can be more than one segment.
Jejunal atresia imaging
XR
Clasically triple bubble
More loops of air the more distal it is
Fluoro
Microcolon
US
Dilated proximal bowel loops
In utero bowel perforation
Polyhydramnios
Jejunal atresia diffys
XR
Malrotation with midgut volvulus
Contrast enema
Total colonic Hirschsprungs
Meconium ileus
Ileal atresia is
a congenital abnormality where there is stenosis or absence of a portion of the ileum. Increased with chromosomal abnormalities.
Ileal atresia imaging
Dilated loops of proximal bowel
Microcolon on fluoro
US: polyhydramnios, proximal dilated segments
Anal atresia is
spectrum of anorectal abnormalities ranging from membranous separation to complete absence
Anal atresia path and association
No anal opening. High or low depending on the location of the atretic portion to levator ani. Mostly sporadic.
Associations:
Other atresias
VACTERL
Caudal regression syndrome: LL hypoplasia, sacral agenesis
Currarinos triad: Sacral anomalies and presacral mass
Fistulous tracts to urethra or vagina
Anal atresia imaging
XR
Variable due to site, mecnoium impaction, physiological efects
May have multiple dilated loops with absent rectal gas
Air within urinary bladder suggests high type
Calcified meconium in the bowel loops would suggest high type
Coin on butt and baby flip
- >2cm high type
- <2cm low type
Fluoro
?rectourinary/vaginal/perineal fistula
?above or below pubococcygeal line
US
Echogenic spot at level of perineum
Bowel dilatation
Renal anomalies
Spinal anomalies
Intestinal malrotation is
congenital anatomical anomaly resulting in abnormal gut rotation as it returns to cavity during embryogensis, Predisposes to midgut volvulus and internal hernias
Malrotation associations
GI tract malformations
Biliary system malformations
Pancreatic malformations
CDH
Heterotaxy
Choanal atresia
hypospadias
Malrotation pathology
During normal embryogensis
- bowel herniates into base of umbilical cord
- rapidly elongates
- undergoes complex 270 CC rotation resulting in DJ flexure to the left at L1 and terminal ileum in the RIF
- resultant broad mesentery running obliquely
Malrotation
- does not occur, resulting in short root mesentery acting as a pedicle around which volvulus can occur
Non rotation
- subtype in which the small bowel is mainly in the right and caecum in the left. Risk of volvulus is lower as patients effectively have the anatomy of patients who have undergone Ladd procedure.
Embyrological delay in rotation of the DJ loop and CC loop
Malrotation imaging
XR
Right jejunal markings
Absence of RLQ stool
Features of midgut volvulus
US
Inverted SMA SMV relationship with SMA on the right and SMV on the left
No retromesenteric D3 segment duodenum
CT
Absent retromesenteric D3
Reversed SMA SMV
Large bowel mostly on left and small mostly on right
fluoro
Abnormal DJ location
Midgut volvulus is
a complication of malrotated bowel resulting in proximal bowel obstruction with resultant ischaemia if untreated
Midgut volvulus pathology and associations
Complication of intestinal malrotation
Associations
Gastroschisis
Omphalocele
CDH
Atresias
Midgut volv imaging
XR
variable
bowel obstruction or free air late
Fluoro
Corkscrew sign
Beaking of bowel
Malrotated configuation
US
whirlpool sign
abnormal mesenteric vessels
- inverted SMV SMA
- Hyperdrynamic pulsating SMA
- truncated SMA
- inapparent SMA
Abnormal bowel
Free fluid
Similar CT findings
Meconium peritonitis is
sterile chemical peritonitis due to intrauterine bowel perforation and spillage of fetal meconium into peritoneal cavity
Meconium peritonitis pathology, classification and associations
Path
Sterile chemical reaction resulting from bowel perforation in utero. Usually perforates due to obstruction from atresias or meconium ileus. Results in ascites, fibrosis, calcifications and cyst formation.
Classification
Fibroadhesive
Cystic
Generalised
Healed
Associations:
CF, usually doesnt calcify
Atresias
Polyhydramnios
Meconium peritonitis imaging
XR
Intraabdominal calcifications
mass containing calcification if cyst
Calc in scrotum if patent processus vaginalis
US
calcification may give snowstorm appearance
Ascites and or polyhydramnios
Increased AC
Dilated bowel
Dilated stomach
Congenital hepatic haemangioma differentials
Hepatoblastoma
- solid, echogenic
- pseudocapsule
- spoke wheel appearance
- less vascular
- elevated AFP
Mesenchymal harmatoma
- predominantly cystic
- septations give swiss cheese appearance
Metastatic neuroblastoma
Infantile/Congenital hepatic haemangiomas are
benign endothelial neoplasms in soft tissue or viscera, usually liver. Most common beign hepatic tumour of infants.
CH: perinatal period, does not proliferate beyond birth
Rapidly involuting RICH and non-involuting NICH subtypes.
IH: not present at birth. Proliferating phase over few months follows by involuting phase from 12 months over years.
Infantile/Congenital hepatic haemangiomas imaging
Focal, multifocal or diffuse
- Focal = true CH. Large solitary well defined.
- Multifocal = IH. Multiple small round lesions. Diffuse, liver enlarged and completely replaced.
XR
Hepatomegaly. RUQ calcification. Heart failure features.
NCCT
Well defined hypodense
CH: hyperdense foci of haemorrhage or calc
CECT
CH: Confluent early nodular peripheral enhancement. No central fill in
IH: Early peripheral with complete central fill in
MRI
T1
hypointense
CH hyperintese foci
T2
CH: heterogenous
IH: homogenous hyperintense
C+
Similar enhancement to CT
MRA/MRV
Enlargement of abdominal arteries and veins including at periphery of lesion
US
CH: prenatal, heterogenous, vascular, hydrops, solitary
IH: Post natal, multiple round well defined lesions, variable
Doppler
CH: peripheral vascularit and AV shunts, little central
IH: variable, low resitance waveforms
Infantile/Congenital hepatic haemangiomas clinical
CH: anaemia, consumptive coagulopathy (usually not kassabach merritt, seen with tufted angioma and kaposiform haemnagioendohelioma)
IH, diffuse: hypothyroid, liver failure, compartment syndrome. Can have kassabach meritt syndrome
AFP not elevated
Demographics:
CH usually perinatal
IH first few weeks
CH MF 1:1
IH MF 3:1
IH common in white
Natural history
RICH involute by 14 months
NICH may grow
IH grow over first year, gradually involute
higher mortality for diffuse
Hepatocellular adenoma is
Benign neoplasm arising from hepatocytes. Rare malignant potential but risk of rupture and hamorrhage.
Different subtypes
Inflammatory 30-50%
HNF1a mutated 35-40%
b catenin activated 10-20%
Undifferentiated 5-10%
Hepatocellular adenoma imaging
US
Well demaracted, solitary, hetergenous
variable echogenicity
Hypoechoic halo can be seen
Doppler
Perilosional sinusoids
CT
Variable dependant on fresh haemorrhage and fat content
If fatty liver can be hyperattenuating
Typically well marginated, isoattenuating
Transient homogenous arterial enhancement
Isoenhancing to liver on PV and delayed
Calcification in 5-10%
MRI
T1 variable, most often hyper
T2 mildly hyper
In/out: should have signal drop out
C+ early arterial, isointense on delayed
Hepatocyte specific: hypointense
Can be heterogenous everywhere due to blood and products
NM
Photopaenic with increased rim on Tc99 sulphur colloid (uptake in 23%)
Increased on HIDA
No uptake on gallium
Hepatocellular adenoma epidemiology
Typically young women OCP
Anabolic steroids men
Glycogen storage diseases
- Type 1 von gierke
- Type III cori or forbes
Obesity
Metabolic syndrome
DM
Hepatocellular adenoma differentials
HCC
- demographics
- washout tends to render it hypointense to liver
- persistent pseudocapsule enhancement
FLHCC
- central scar
- more calcification
- LN enlargement
FNH
- T2 bright scar with late enhancement
- retains hepatocyte spec contrast on delayed
Hepatic metastases
- T1 hypo T2 hyper
- fat and haemorrhage less common
Haemangioma
- typically fills in
- flash filing follows blood pool
Multicystic dysplastic kidney is
A non inheritable paediatric cystic renal disease resulting in multiple renal cysts forming in utero
Multicystic dysplastic kidney epidemiology
1:2500-4000
Slightly higher for males in uni and females in bilateral
Multicystic dysplastic kidney pathology
Affected kidney or renal segment has no functional tissue and is replaced by multiple cysts.
Type main types:
1. pelviinfundibular
- most common
- multiple small non communicating renal cysts representing dilated calyces
- atresia of the ureter and pelvis
- may regress spontaenously
2. Hydronephrotic obstructive
- dominant cyst in the renal pelvis
Genetic
Sporadic and non familial.,
Rarely autosomal dominant forms.
Associations:
Associated renal tract anomalies is common including - - VUR
- PUJ obstruction
- ureteral ectopia
- VEUJ obstruction
- urterocele
Sydnromes
- Meckel gruber
- zellweger
- joubert syndrome related disorders
Multicystic dysplastic kidney imaging
Often made antenatally with multiple small cysts becoming evident as early as the 15th week of gestation. May change dramatically over time.
US
Lobulated contour with multiple internal cysts of variable sizes
Parenchyma usually fibrous and echogenic with small hilar vessels
Cysts usually cluster and are non communicating
MRI
T2 bright well demarcated.
NM
MAG3 or DTPA: generally a void with no excretion
Renal agenesis is
congenital absence or one or both kidneys. If bilateral (classic potter) than its fatal.
Renal agenesis pathology and associations
Cause unknown in many cases. Can be early vascular insult to ureteric bud.
Results from failure of the proper development of the metanephros resulting in complete absence. Thought to occur early weeks 6-7. May also have internal genital malformation due to failure of the wolffian and mullerian ducts to develop or involute.
Associations
Chromosomal
- 21. 22, 7 and 10.
- Turner
Mullerian duct anomalies
Congenital heart disease
Skeletal anomalies
Potter syndrome/sequence
VACTERL
Adrenal agenesis
Renal agenesis imaging
Antenatal US:
Absent kidney
Absent renal artery
Contralateral hypertrophy
Lying down adrenal sign
Bilateral: oligo or anhydramnios, non visualised bladder
Renal ectopia is
Congenital renal anomaly characterised by the abnormal location of one or both kidneys.
Can occur as cross fused, thoracic or pelvic
Renal ectopia pathology
During normal embyrological development there is cephalic migration. Ectopic is arrested migration.
Renal ectopia associations
MCDK
Ureterocele
Patent urachus
VUR
Vaginal agensis
Hypospadias
PUJO
Other renal, vertebral, GI, GU and spinal anomalies
Horseshoe kidney is
most common type of renal fusion anomaly. renders the kidney susceptible to trauma and increase risk of calculi/cancer (TCC, wilms, carcinoid).
Horseshoe kidney pathology
Fusion across midline of two functioning kidneys. Isthmus can be fibrous or parenchymal.
Normal ascent restricted by IMA which hooks over isthmus. Normal renal axis is reversed with inferior poles pointed medially.
Renal vascular anomalies are common
Duplex collecting system is
one of the most common renal tract congenital abnormalities, characterised by incomplete fusion of the upper and lower moieities resulting in a variety of complete or incomplete duplications of the collecting systems. Can be complicated by VUR, obstruction or urterocele.
Duplex collecting system pathology
Occurs when two separate burds arise from a single wolffian duct.
Variable, exist on a spectrum from two pelvises draining to single ureter, to two complete separate tracts.
unilateral or bilateral
associated with a varietyy of other congenital abnormalities
assoc fanconi anaemia
Duplex collecting system classification
duplex kidney: two pelvicalyceal systems draining a single parenchyma
duplex collecting; duplex kidney draining into
- singler ureter
- bifid ureter
- double ureter
bifid collecting system: duplex kidney with two separate collecting systems uniting at the puj or as bifid ureters
duplicated ureters: two ureters that drain separately into the bladder or genital tract
Duplex collecting system imaging
duplicated ureters
obstruction of the upper pole moiety, usually with ureterocele
VUR into lower pole moiety
ectopic insertion of the upper pole moiety
fluoro
inferiorly displaced lower pole moiety dropping lily appearance
non excreting upper
Crossed fused renal ectopia is
an anomaly where the kidneys are fused and located on the same side of the midline
Crossed fused renal ectopia subtypes
Inferior cross fusion
sigmoid kidney
lump kidney
disc kidney
L shaped kidney
superiorly crossed fused
left to right ectopy three times more common
Crossed fused renal ectopia imaging
90% at least partial fusion
10% unfused
Characteristic anterior or posterior notch between the two fused kidneys
PUJ obstruction pathology
Commonly unilateral, left sided predilection
PUJ forms around the fifth week and the initial tubular lumen of the ureteric bud becomes recanalised by 10-12 weeks.
PUJ last area to recanalise. Inaequaecy here is thought to be the main explanation of PUJO. Extrinsic obstruction also commonly encountered.
Etiology:
Congenital
- idiopathic. proposed causes include
- abnormal muscle arrangement
- anomalous collagen collar
- ischaemic insult
- urothelial ureteral fold
- extrinsic compression or encasement
Adult
- Trauma
- calculus
- pyelitis with scarring
- malignancy
- extrinsic compresssion
PUJ obstruction differentials
Congenital megacalectasis
Extrarenal pelvis
Parapelvic cyst
Fetal pyelectasis pathology and associations
Majority physiological and spontaneously resolving.
May heraled tract pathology including;
- PUJO
- VUJO
- Urethral obstr such as posterior valves
- VUR
- Duplex kidney
Associations
- soft marker trisomy 21
Fetal pyelectasis imaging
AP measurment of the renal pelvis axial plane
>4mm up to 28 weeks
>7mm at or after 38 weeks
Note any calyceal dilatation, ureteric dilatation, parenchymal appearance, bladder appearance and oligohydramnios
Vesicoureteric reflux is
abnormal flow of urine from the bladder into the upper urinary tract and is typically encountered in young children
Vesicoureteric reflux clinical
Predisposes to pyelonephritis
May be isolated or associated with other anomalies including
- congenital posterior urethral membrane
- bulbar urethral obstruction
- ureteral partial obstruction
- duplex collecting system
Vesicoureteric reflux pathology
Majority of cases is the result of primary maturation abnormality of the VUJ resulting in a short distal ureteric submucosal tunnel. Normal pinch cock action when bladder pressure increases is impaired allowing urine to pass retrogradely.
Vesicoureteric reflux imaging
VCUG
- confirm presence and grade
- occurence during micturition or during filling
- presence of associated anatomical anomalies
- length of the ureteric tunnel
- width of the lower ureter
US
- assess renal parenchyma for scarring or anatomical anomalies
NM
- can also grade it but cant see bladder disease, visualised male urethra as well and lacks spatial resolution
Vesicoureteric reflux treatment
Low grade - abx
Surgical reimplantation for higher grades
Endoscopic treatment injceting bulking agent at VUJ may be used
Vesicoureteric reflux grading
- limited to ureter
- up to renal pelvis
- mild dilatation of ureter and pelvicalyceal
- tortuous ureter with moderate dilatation, blunted fornices but preserved papillary impressions
- tortuous ureter with severe dilatation. loss of fornices and papillary impressions.
Posterior urethral valves are
the most common congenital obstructive lesion of the urethra and a common cause of obstructive uropathy in children. Only seen in males.
Posterior urethral valves clinical
Variable, may not be apparent until early infancy if mild. If severe, oligohydramnios and SGA. UTIs are common
Posterior urethral valves pathology and associations
Formation of thick valve like membrnae from Wolffian duct origin (failure of regression of the mesonephric duct) that courses obliquely from the verumontanum to the most distal portion of the prostatic urethra.
Vast majority sporadic
Associations:
Chromosomal abnormalities
Bowel atresias
Craniospinal defects
Posterior urethral valves types
Young classification
1. most common. Two mucosal folds extend anteroinferiorly from verumontanum and fuse anteriorly at lower level
- rare, considered a normal variant. Mucosal folds extend along posterolateral urethral wall from ureteric orifice to verumontanum
- circular diaphragm with central opening in membranous urethra. Located below the verumontanum and occurs due to abnormal canalisation of urogenital membrane. Sometimes referred to as Cobbs collar.
Posterior urethral valves imaging
Antenatal:
- distention and hypoertrophy of the baldder
- may have hydroureteronephrosis
- oligohydramnios and renal dysplasia
- keyhole sign
Post natal US
- Similar to antenatral
- posterior urethral diameter >6mm
- can ruture collecting system resulting in urinoma around kidney or rupture bladder causing free fluid
VCUG
- keyhole sign
- linear radiolucent band
- VUR
- bladder trabeculae and diverticula
Posterior urethral valves treatment and prog
Antenatal treatment is vesicoamniotic shunting
Post natally transurethral ablation of valve
Prognosis by degree and duration of obstruction. Severe cases with renal dysplasioa, oligohydramnios and pulmonary hypoplasia can be fatal.
Bladder exstrophy is
herniation of the bladder through an anterior abdominal wall defect. Severity is variable. Male predilection. Mostly sporadic.
Bladder exstrophy imaging
Soft tissue mass extending from a large infra umbilical anterior abdominal wall defect
Absence of the normal bladder and low lying umbilical cord insertion
Failure of the pubic bones to meet in the midline (manta ray sign)
Hurley stick appearance of the distal ureters
Fetal enteric duplication cyst pathology
Result from abnormal recanalisation of the gastrointestinal tract. Comprise of two layer smooth muscle wall and internal epithelium of a respiratory or intestinal type.
May or may not communicate with GI tract.
Cystic or tubular.
Can be anywhere but predilection of the ileal region.
Associations
Most commonly vertebral
Fetal enteric duplication cyst imaging
Anechoic cystic lesion within the abdomen separate from the normal hollow structures such as the bladder and the stomach.
Characteristic signs
- double wall sign
- gut signature sign (echoic club sandwich)
Can be echogenic (likely haemorrhage)
Fetal enteric duplication cyst differential
Fetal omental cyst
Fetal mesenteric cyst
Meconium pseudocyst
Fetal ovarian cyst
Mesenteric lymphatic malformation is
Subtype of congenital slow flow vascular malformation due to error of lymphatic vessel formation. Results in well defined cyst like (macro or micro) mass of abnormal lymph channels focally or diffusely. Lacks normal communication. Not neoplastic.
Synonymous with mesenteric or omental cyst, lymphangioma
Mesenteric lymphatic malformation imaging
Macrocystic
- well defined, lobulated, uni or multilocular fluid filled mass in mesentery
- thin septations with minmal enhancement
- simple or complex fluid, can have layering fluid fluid levels
Microcystic
- more solid appearing, infiltrative
- may be intermixed with macrocystic components
Mesenteric lymphatic malformation clinical
Generally slow growing. May rapidly enlarge due to haemorrhage, infection, hormonal.
Complications include obstructive, volvulus, infection, haemorrhage
Most present under 2 yo
Simple excision, partial bowel resection, sclerotherapy. Can use sirolimus
Fetal adrenal hemorrhage pathology
unknown. associated with birth trauma, perinatal asphyxia, sepsis and congenital infections (classicaly syphilis)
uni or bilateral
Fetal adrenal hemorrhage imaging
US
mass above kidney
demonstrate changing characteristics on sequential imaging
- hyper to iso to hypo to anechoic
with or without septations
no intrinsic vascularity
Fetal adrenal hemorrhage differential
Neuroblastoma (vascularity)
Subdiaphragmatic pulmonary sequestration (left predilection, aorta supply
fetal adrenal cyst
Fetal neuroblastoma pathology
May represent temporary defect in growth of normal fetal adrenal neuroblastic nodules which involute over time.
Most sporadic with 1-2% familial. Autosomal dominant with incomplete penetrance. Germline mutations in ALK and PHOX2B
Derive from primordial neural crest cells. Cystic change may indicate involution.
May mature to neuroblastoma, ganglioneuroblastoma, ganglioneuroma.
Biologic marcker; MYCN amplication (protooncogene on chromosome 2p, multiple copies in aggresive). DNA index (>1 more favourrable)
90% arise in the adrenal glands. Usually seen third trimester. Slight right predilection.
Fetal neuroblastoma staging
International neuroblastoma staging sytem
1. confined to adrenal
2. extension beyond adrenal but not across midline
3. extension across midline
4. mets
4s. skin/liver and <10% bone marrow, good prog
Risk group staging
L1: lororegional without image defined risk factors
L2: locoregional with 1 or more IDRF
M: distal mets
Ms: similar to 4s
Fetal neuroblastoma imaging
Complex cystic mass in suprarenal location. Diffuse vascularity rather than feeding vessel.
Fetal neuroblastoma Clinical
Most comon congnital malignancy
Variable fetal course. May resolve spontaneously. Most remain stable without complication. Minority progress to hydrops and death.
Most have a favourable stage and biologic markers with excellent prognosis.
Fetal neuroblastoma diffys
Extralobar sequestration - usually left sided, more solid, feeding vessel from aorta.
Adrenal haemorrhage - no colour
Duplex collecting
Mesoblastic nephroma
Teratoma
Persistent right umbilical vein path and associations
Normally obliterates between 4th and 7th weeks. With PRUV, left obliterates. Intra or extrahepatic.
Associations:
Single UA
Chromosomal anomalies, 18
Noonan
Situs anomalies
Congenital cardiac, renal and GIT
Vertebral anomalies
Cloacal malformation is
a spectrum of congenital hindgut and GU anomalies resulting from failure of cloacal division early in embryogenesis.
Cloacal dysgenesis - complete absence of perineal openings
Classic - convergence of urinary, genital and hindgut structures into single channel
Urogenital sinus - common channel draining urethra and vagina at expected location of normal urethra with normally positioned anus
Posterior cloaca - urogenital sinus deviates, draining into anterior wall of rectum
Cloacal variant - urogenital sinus with anteriorly displaced anus
Cloacal malformation pathology
Arrested development. Cloaca present until 5th week when urorectal septum divides urogenital sinus from anorectum. Urogenital sinus further divides into bladder/urethra and vagina.
Cloacal malformation imaging
Cystic mass posterior to bladder (hydrocolpos) with fluid fluid or fluid debris level.
Uterine and vaginal duplication is observed in 80% of cases with hydrocolpos.
Additional anomalies in GU, bowel, lumbosacral
Abnormal genitalia and absent anal dimple
Absence of normal meconium filled t1 hyperintense rectum on MR
Hepatoblastoma is
the most common primary malignant liver tumour in children under four years. Abdominal mass and raised AFP. Rare with slight male predilection.
Hepatoblastoma pathology
Well circumscribed large masses.
Notable subtypes
- epithelial (most common). Includes fetal, embryonal and small cell undiff
- Mixed: epithelial and mesenchymal. more calcifications.
AFP frequently elevated.
Major and minor histo categories
Major:
Epithelial (fetal, embryonal, macrotrabecular)
Mixed
Small cell undifferentiated
Rhabdoid
Minor:
cholangioblastic
keratinising squamous epithelium
interstinal glandular epithelium
teratoid
rhabdomyoblastic
chondroid
osteoid
Hepatoblastoma associations
Beckwith WIedemann
Hemihypertrophy
FAP
FAS
Gardner syndrome
Glycogen storage disease
Biliary atresia
Hepatoblastoma imaging
XR
RUQ mass may have calcifications
US
Echogenic soft tissue mass, can be heterogenous. Can have calcifications
CT
Well defined heterogenous mass, usually hypoattenuating. Areas of necrosis and haemorrhage. Chunky calcifications.
MRI
T1 hypo
C+ heterogenous enhancement
T2 generally hyper, can be heterogenous
Hepatoblastoma differentials
hepatic mesenchymal hamartoma
infantile hemangioendothelioma
hepatic mets
HCC
rhabdomyosarcoma of biliary tree
Congenital talipes equinovarus is
the most common anomaly affecting the feet.
Synonymously used with the term club foot although that is more encompassing and includes
- talipes equinovarus (forefoot adduction, inversion of heel and plantar flexion of ankle)
- talipes calcneovalgus (dorsal flexion of the forefoot with plantar surface facing laterally)
- metatarsus varus (inversion and adduction of the forefoot alone)
Congenital talipes equinovarus pathology and associations
Deformity involving the ankle and subtalar joints. Fixed by joint ligamentous and tendinous contractions.
May be a familial predilection.
Associations (fuckloads)
Chromosomal
- 18q deletion
- trisomy 18
- Wolf Hirschhorn
Other syndromes
- Freeman Sheldon
- Meck gruber
- Roberts
Renal anomalies
- prune belly
- renal agenesis
Connective tissue disorders
- Marfan
- Ehlers danlos
Spinal anomalies
- caudal regression
- diastematomyelia
- spina bfida
Skeltal dysplasia
- diastophic
Congenital talipes equinovarus imaging
hindfoot equinus: lateral talocalcaneal angle <35
hindfoot varus: talocalcaneal angle less than 20
Metatarsus adductus: adduction and varus deformity of the forefoot, talus to first MT angle >15
talonavicular subluxation
US
tib and fib in sam image as medially deviated foot, foot may appear plantarflexed
Autosomal recessive polycystic kidney disease is
one of the paediatric cystic renal diseases. Enlarged echogenic kidneys with multiple small cysts. May have liver involvment with featrures of hepatic fibrosis
One of the commonest inheritable infantile cystic renal diseases but far less common than ADPKD which affects adults.
Autosomal recessive polycystic kidney disease pathology and associations
Mutation in PKHD1 gene on chromosome 6p.
Bilateral symmetric microcystic disease in distal convulted tubles and collecting ducts.
Perinatal type - mopst common. oligohydramnios and pulmonary hypoplasia. Minimalhepatic fibrosis
Neonatal type - minmal hepatic fibrosis
Infantile type - moderate periportal fibrosis
Juvenile type - gross hepatic fibrosis
Associations
Carolis
Congenital hepatic fibrosis
Multiple biliary hamartomas
Autosomal recessive polycystic kidney disease imaging
US
Oligohydramnios
cysts initially too small to resolve, rarely exceed 1-2cm
Enlarged and echogenic kidneys
Medullary pyramids intially hypoechoic, gives a peripheral halo
CM differentiation eventually lose
liver changes; carolis, coarse echotexture, portal hypertension
MRI
large kidneys, increased T2 signal
oligohydramnios
Autosomal recessive polycystic kidney disease ddx
ADPKD
Beckwith wiedemann
Laurence moon beidl
Meck Gruber
Renal dysplasia with trisomy 13
Polydactyly is
more than the usual number of digits. Broadly catergorised into preaxial (radial), post axial (ulnar), and central
Pre axial polydactyly assoc
Down syndrome
VATER association
Holt-Oram syndrome
Greig cephalopolysyndactyly syndrome
Carpenter syndrome
Laurin-Sandrow syndrome
Fanconi anaemia
Post axial polydactyly assoc
trisomy 13
Meckel Gruber syndromee
oral-facial-digital syndrome (OFDS)
skeletal dysplasias
Ellis-van Creveld syndrome
asphyxiating thoracic dysplasia - Jeune syndrome
Syndactyly is
congenital fusion of two or more digits. May be soft tissue or bone.
Syndactyly assoc
Fuckloads. Can be isolated.
Aneuploidic syndromic
triploidy: tend to affect the 3rd and 4th digits of the hands
Non-aneuploidic syndromic
acrocephalosyndactylies
type I: Apert syndrome
type II: Crouzon syndrome
acrocephalopolysyndactylies
amniotic band syndrome
Gorlin syndrome
Pallister-Hall syndrome
Poland syndrome
prune belly syndrome
VACTERL association
Down syndrome
neurofibromatosis typ
Non-syndromic
isolated polydactyly, a.k.a. polysyndactyly
isolated brachydactyly, a.k.a. brachysyndactyly
isolated ectrodactyly
Clindactyly is
radial angulation at an IPJ, typically fifth finger
Clindactyly assoc
Often isolated and normal.
aneuploidic syndromic
Down syndrome: may be seen in up to 60% of infants with Down syndrome 3
Klinefelter syndrome
trisomy 18
Turner syndrome
non-aneupliodic syndromic
Cornelia de Lange syndrome
Feingold syndrome
Roberts syndrome
Russell-Silver syndrome
Fanconi anaemia
non-syndromic
macrodystrophia lipomatosa
brachydactyly type A3
Achondroplasia is
a congenital genetic disorder resulting in rhizomelic dwarfism. Most common skeletal dysplasia.
Achondroplasia pathology
mutation in the fibroblast growth factor gene 3 on 4p16.3
Causes abnormal cartilage formation.
Gain of function mutation with constitutive activation of an inhibitroy signal. All bones form by endochondral ossification affected. Membranous ossification not affected.
Achondroplasia imaging
Antenatal
- short femur length
- trident hand (2/3/4 finger separation)
- frontal bossing
- depressed nasal bridge
Cranial
- large cranial vault with small skull base
- frontal bossing and depressed nasal bridge
- narrow FM
- cervicomedullary kink
- elevation of the brainstem
- communicating hydrocephlus
- large anterior fontanelle
Spinal
- posterior vert scalloping
- decreasing interpedicular distance
- gibbus (thoracolumbar kyphoisis with bullet shaped vert)
- short pedicle canal stenosis
- laminar thickneing
- widening of the intervertebral discs
- increased sacral lumbar angle
Chest
- anterior flaring of ribs
- anteropoasterior narrowing of the ribs
Pelvis/hips
- decreased acetabular angle
- tombstone iliac wings
- trident acetabulum
- champagne glass pelvic inlet
- short sarcoiliac notches
Limbs
- metaphyseal flaring, trumpet
- rhizomelic shortening
- long fibula
- boweing to mesial segment
- trident hand
- chevron sign
- short proximal phalanges
Achondroplasia DIFFYS
achondrogenesis
campomelic dysplasia
thanatophorix dysplasia
chondroectodermal dysplasia
osteogenesis imperfecta is
heterogenous group of congenital non sex linked genetic disorders of collagen type 1 production
hallmark feature is osteoporosis and fragile bones, blue sclera, dental fragility and hearing loss
variable depending on type
assoc with congen cataracts
osteogenesis imperfecta pathology
disturbance of type 1 collagen synthesis, predominant protein of extracellular matrix of most tissues
mutation in one of the two genes for type 1 collagen. COL1A1 and COL1A2 which encode the a2 and a2 polypeptide chains.
can be sporadic or autosomal. most commonly autosomal dominant
osteogenesis imperfecta imaging
Prenatal
- decreased calvarial ossification
- evidence of fractures
- presence of polyhydramnios
head/neck/spine
- basilar invagination
- wormian bones
- kyphoscoliosis
- vertebral compression fractures
- codfish vert
- platyspondyly
Chest
- excavatum or carinatum
- accordian ribs
Pelvis
- protrusio acetabuli
- coxa vara
General
- OP
- gracile overtubulated bones
- cortical thinning
- hyperplastic callues
- popcorn calcification
- zebra striple sign (bisphosphanates)
- pseudoarthoroses at fractures
Fetal hydrocephalus is
extension of fetal ventriculomegaly where dilatation is more severe. Lateral ventricle diameter >15mm.
Fetal hydrocephalus pathology and associations
Obstructive or non obstructive. Can have X linked.
Central nervous system anomalies: (more common and reported in more than 80% of cases):
- aqueductal stenosis: one of the commonest causative associations
- Chiari malformations
- neural tube defect(s)
- Dandy-Walker malformation
- encephalocele
- alobar holoprosencephaly
Non-central nervous system anomalies:
craniofacial
acrocephalosyndactylia
congenital cardiovascular anomalies
gastrointestinal anomalies
genitourinary anomalies
congenital renal fusion
skeletal anomalies
clubfeet
Syndromes:
- Meckel-Gruber syndrome
- Miller-Dieker syndrome
Chromosomal anomalies: may be present in ~20% of cases
- trisomy 21 8
- triploidy 8
Fetal hydrocephalus imaging
Ventriculomegaly
Lateral ventricles >15mm
Parenchymal thinning
3mm between choroid and ventricle margin
Aqueduct stenosis is
most common cause of congenital obstructive hydrocephalus
Aqueduct stenosis pathology
Congenital
- webs or diaphragms
- gliosis
Acquired
- extrinsic compression (tumours, avms)
- intrinsic (infection, haemorrhage, idiopathic)
Aqueduct stenosis imaging
Hydrocephalus
Near normal posterior fossa
Secondary thinning of the cortical mantle and macrocephaly
Choroid plexus cysts are
benign, often transient typically resulting from an infolding of the neuroepithelium
Choroid plexus cysts (antenatal) associations
Soft marker
Trisomy 18, 21
Klinefelter syndrome
Aicardi syndrome
Choroid plexus cysts (antenatal) imaging
Sonolucent cysts about the lateral ventricles 2nd trimester
should go by 26-28weeks
Warrants closer surveillance, particularly if large, bilateral, multiple, abnormal maternal screening
Fetal intracranial haemorrhage pathology
- mechanical trauma
- severe fetal hypoxia
- fetal thrombocytopaenia
- maternal thrombocytopaenia (alloimmune, idiopathic, vonwillebrand, medications)
- tumour
- twin twin transfusion
- demise of a co twin
Fetal intracranial haemorrhage imaging
variable depending on location and age
- can be seen as irregular hyperechoic mass
- as it matures, porencephalic cyst formation or fetal intracranial calcification
Anencephaly is
the most severe neuralt tube defect, complete absence of cortical tissue. Ranges from holocrania to merocrania.
Anencephaly pathology and associations
Failure of closure of the antral end of the neural tube, happens at day 24.
Markers
- highly eelvated MSAFP
Anencephaly imaging and ddx
US100% accurate at 14 weeks.
- no tissue above the orbits and absent calvarium
- If small amount of tissue then exencephaly
- low CRL
- Frog eye, mickey mouse
- polyhydramnios
Ddx
- severe microcephaly
- amniotic band syndrome
Encephalocoele is
a form of NTD where brain tissue and overlying meninges herniate through a defect in the cranium
Encephalocoele pathology and associations
Form of NTD, due to failure of fusion of the cartilginous neurocranium, membranous neurocranium or viscerocranium.
Associations
- Chiari malformations
- Dandy walker malformations
- Meckel Gruber
Encephalocoele classification
- Occipital (75%)
- Sincipital (frontoethmoidal, interfrontal, craniofacial clefts)
- Convexity
- basal (10%, intrasphenoidal, temporal, transsphenoidal or whatever)
Encephalocoele imaging
Antenatal US
- may be purely cystic or echoes from brain tissue
CT/MRI
- better for defining anatomy and contents
Microcephaly is
a term for a small head. Usually defined as HC <3rd centrile or lower than 2 SD below the mean. May be primary (never forms) or secondary (normal develoment arrested by some insult)
Fetal toxoplasmosis clinical
The majority of infants (~75%) are asymptomatic. For those symptomatic, the severity of symptoms is related to the trimester of pregnancy when transmission occurred 11:
first trimester: fetal death
second trimester: retinochoroiditis, microcephaly, and intellectual disability
third trimester: lymphadenopathy, hepatosplenomegaly, eye injuries, and brain calcifications
Serologic
- PCR on amniotic fluid
- cordocentesis for igM antibodies
Complications
- hydrops
- after birth sequelae
Fetal toxoplasmosis imaging
- hydrocephalus
- IC calcification
- microcephaly
- intrahepatic calc
- hepatosplenomegaly
- ascites
Congenital cerebral toxoplasmosis imaging
- multiple calcifications
- cerebral/cerebellar atrophy
- encephalomalacia
- ventriculomegaly
- micro or macrocephaly, hydrocephalus
- cortical abnorm, rare
- chorioretinitis
Congenital rubella clinical
Features
- sensorineural deafness
- cataracts
- cardiac anomalies
- VSD/ToF
- intellectual disability
- microcephaly
- IUGR
Worst during 1st trimester
Serology
- maternal rubella specific igG and IgM
Congenital rubella imaging
- hydrops, if severe
- cardiac anomaly
- IUGR
- subependymal cysts
- small HC
- periventricular calc
- white matter hypo
- ventriculomegaly
non specific
Congenital CMV clinical
most common
mostly asx
possible features
- jaundice/hepatosplenomegaly
- microcephaly
- sensorineural deafness
- chorioretinitis
- petechiae
- blueberry muffin rash
- intellectual disability
- seizures
complications
- encephalitis
- hydrops
Congenital CMV imaging
- intracranial calv
- hydrocephalus
- heterogenous parenchyma
- microcephaly
- intraventricular adhesiosn
MRI
- white matter lesions
- delayed myelination
- cysts, periventricular and temporal pole
- migrational abnormalities
other
- intrahepatic calc
- hepatomegaly
- IUGR
- echogenic bowel
Spinal dysraphism is
broad group of malformations affecting the spine and surrounding structures. Form of NTD
Spinal dysraphism pathology
NTD formed by lengthwise closure of the nural plate in the embryo dorsum
Upper part forms forebrain, midbrain and hindbrain. Lower part forms spinal canal.
Dysraphism is when teh neural palte does fuse completely in the lower segment.
Spinal dysraphism classification
Open: when cord and covering communicat ith outside
- myelomeningocoele
- myelocoele
- hemimyelomeningocoele and myelocoele
Closed: cord is covered by other nromal mesenchymal elements
Closed with SC mass
- lipoma with dural defect (lipomyelomeningocoele, lipomyelocoele)
- terminal myelocystocele
- meningocoele
- limited dorsal myeloschsis
Closed without SC mass
- posterior spina bfida
- intradural lipoma
- filar lipoma
- tight filum terminale
- persistent terminal ventricle
- disorders of midline notochordal integration (dermal sinus, enteric fistual, neuroenteric cyst, split cord malformations)
- disorders of notochorodal formation (caudal regression 1 and 2, segmental dysgenesis)
Caudal regression syndrome
represents a spectrum of structural defects of the caudal region, varying from isolated partial coccyx agensis to lumbosacral agenesis
Caudal regression syndrome imaging
variable
- LS vertebral body dysgenesis/hypoplasia
- usually below L1, most often limited to sacrum
- truncated blunt spinal cord terminating above expected level; wedge or cigar shaped conus
- severe canal narrowing rostral to last intact vertebra
- assoc anomalies
Antenatal
- as above
- shield sign; opposed iliac bones
- low CRL
MRI
-as above. two groups
- group 1; blunt conus, dilated central canal or cyst. usually major deformity
- group 2; conus elongated and tethered by thick filum or lipoma, lower than expected level. neurologic sx worse.
Sacrococcygeal teratoma is
commonest congenital tumour in fetus and neonate. female predilection.
Sacrococcygeal teratoma classification
Pathology base
- benign (mature); more common
- malignant (immature)
Location based
1. only outside the fetus, may have small pre sacral component
2. extra fetal with intrapelvic presacral extension
3. extra fetal with extension through pelvis into abdomen
4. tumour developing entirely in the fetal pelvis
Sacrococcygeal teratoma imaging
XR
- large mass
- may have calc
CT
- heterogenous, bone fat calc etc
US
- more cystic with anechoic components
- echogenic solid parts/type
- marked hypervascularity
MRI
T1: fat, low calc
T2: high signal fluid, calc low
T2*: calc
C+: enhancing solid components
Sacrococcygeal teratoma complications
high output cardiac failure, hydrops
ureteric obstruction
GIT obstruction
nerve compression
anaemia
dystocia
tumour rupture
Sacrococcygeal teratoma differentials
chordoma
myelocystocoele
meningocoele
low lying neuroblastoma
low lying rhabdomyosacroma
small round blue cell tumour
enteric cyst
OHSS is
a complication of ovarian stimulation for IVF consisting of ovarian enlargement, extravascular accumulation and intravascular depletion.
OHSS path
massive cystic ovarian enlargement and fluid shift from intravascular compartment into peritoneal, pleural or pericardial spaces due to increased capillary permeability of mesothelial surfaces. Vasoactive factors from multiple corpora lutea.
very rare sporadic forms assoc with precocious puberty and hypothyroidism (van wyk grumback)
OHSS imaging
bilateral enlarged ovaries, multiple cysts, spoke wheel. ascites, effusions
OHSS grading
Modified Golan
Mild - ovarian enlargement
1. distension
2. nvd, ovaries 5-12cm
Moderate - assoc ascites
3. mild ohss + ascites
severe - hypovolaemia
4. moderate + ascites/hydrothorax
5. change in blood volume, coags etc
herlyn werner wunderlich syndrome is
mullerian duct anomaly with assocaited mesoneprhic duct anomaly
triad of
- uterus didelphys
- renal agenesis (or other renal anomalie)
- obstructed hemivagina
