O+G Flashcards

Question

Unicornuate uterus pathology

Answer 1

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%

Answer 2

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

Answer 3

Mild variant shape of the uterus, mild indentation of the endometrium at the uterine fundus. Least associated with reproductive failure.

Answer 4

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

Answer 5

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

Answer 6

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

Answer 7

Class III Mullerian duct anomaly where there is complete duplication of uterine horns as well as duplication of the cervix with no communication

Answer 8

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

Answer 9

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.

Answer 10

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.

Answer 11

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

Answer 12

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

Answer 13

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.

Answer 14

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

Answer 15

Partial: endometrial canal but not the cervix. Complete: extends either to internal or external os Septate uterus/vagina: extends into vagina

Answer 16

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

Answer 17

Bicornuate: shape of external contour Arcuate: small myometrial indentation with normal contour Thick adhesion

Answer 18

Distension of the uterus and vagina with blood (metra uterus colpo ovary)

Answer 19

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

Answer 20

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

Answer 21

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

Answer 22

Ascending polymicrobial infection of the cervic and uterus

Answer 23

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

Answer 24

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

Answer 25

RPOC: Echogenic endometrial mass. High vel low resistance flow doppler. Clot and debris Normal gas in cavity

Answer 26

Embryologic mesonephric duct remnant. Simple anterolateral upper vaginal wall cyst.

Answer 27

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

Answer 28

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

Answer 29

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

Answer 30

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

Answer 31

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).

Answer 32

Obstetric trauma Gynae/urologic procedures Inflammation (Crohns)/ infection Pelvic malignancy (bladder, cervical, endometrial) Radiation therapy, 20 years post

Answer 33

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

Answer 34

Canal narrowing from benign or iatrogenic source. When severe, results in hydrometra, pyometra or haematometra. Cervical canal narrowing <2.5-3.5mm

Answer 35

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

Answer 36

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

Answer 37

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

Answer 38

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

Answer 39

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

Answer 40

Subtype of mucinous adenocarcinoma of cervix , termed malignum due to virulent and fatal progression

Answer 41

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

Answer 42

Excessive proliferation of endometrial glands with increased ratio of glands to stroma

Answer 43

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

Answer 44

Premenopausal >8mm proliferative >16mm secretory Post menopausal with bleeding >5mm Postmenopausal without bleeding >8-11mm

Answer 45

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.

Answer 46

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.

Answer 47

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

Answer 48

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

Answer 49

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

Answer 50

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

Answer 51

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

Answer 52

Benign tumours of uterine smooth muscle cells

Answer 53

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

Answer 54

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

Answer 55

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

Answer 56

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

Answer 57

Suppurative leimyoma. Presents with sepsis, bactaraemia, leiomyoma.

Answer 58

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

Answer 59

Etiology: Congenital (rare) Acquired; post traumatic or infectious Risk factors: D/C, IUD, pelvic surgery, infection, GTD, carcinoma, diethylstilbestrol exposure Assoc: pseudoaneurysm

Answer 60

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

Answer 61

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

Answer 62

Twisting of the vascular pedicle on axis of infundibulopelvic and uteroovarian ligaments. Leads to venous and lymphatic congestion, arterial obstruction and infarction.

Answer 63

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

Answer 64

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.

Answer 65

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

Answer 66

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

Answer 67

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

Answer 68

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

Answer 69

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

Answer 70

Polycystic ovarian morphology with clinical and endocrine dysfunction. Must exclude other etiologies of hyperandrogenism and menstrual disturbance.

Answer 71

>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

Answer 72

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

Answer 73

Congenital cystic tumour composed of well differentiated derivations from at least 2/3 germ cell layers. Classified as benign ovarian germ cell tumour.

Answer 74

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

Answer 75

Endometriomas Bowel - gas/faeces may mimic rokitansky Haemorrhagic cyst Pedunculated lipoleiomyoma Immature teratoma - predominantly solid that contain fatty elements, irregular calcifications and numberous cysts

Answer 76

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

Answer 77

Form of failed early pregnancy with a gestational sac but the embryo does not form

Answer 78

Blastocyst formed from fertilised ovum Fetal pole never develops bHCG formed due to syncytiotrophoblast invasion into endometrium

Answer 79

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

Answer 80

Early pregnancy Pseudogestational sac Gestational trophoblastic disease

Answer 81

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

Answer 82

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%

Answer 83

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

Answer 84

Neither uterine or ectopic prengnancy identified on TVUS in setting of positive BHCG DDX - early - non viable not detected - complete miscarriage - unidentified ectopic

Answer 85

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

Answer 86

cessation of vaginal bleeding with no RPOC or GS in women with previous IUP

Answer 87

Open internal os, bleeding, 1st trimester. Most often intracervical contents. Migration on serial scans. Progression of a threatened miscarriage once cervix opens

Answer 88

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

Answer 89

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

Answer 90

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

Answer 91

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

Answer 92

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.

Answer 93

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

Answer 94

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

Answer 95

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

Answer 96

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

Answer 97

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)

Answer 98

abnormally low lying placenta to the internal cervical os. common cause of antenatal haemorrhage

Answer 99

Previous PP Previous CS Increased maternal age Increased parity Large placentas Smoking Assisted conception

Answer 100

abnormal placental villous adherences

Answer 101

1: low lying - 20-5mm 2: marginal - reaches internal os, doesnt cover 3: partial - partially covers 4: complete - completely covers

Answer 102

premature separation of normally implanted placenta after 20 w and before 3rd stage of labour. Potentially fatal. Cause of 3rd trimester bleeding.

Answer 103

Preeclampsia Prior abruption PROM Maternal age Maternal trauma Smoking Cocaine/amphetamine Thrombophilia Chorioamnionitis Short cord Multiparity

Answer 104

Board like abdo tone Bleeding, can be concealed Uterine contractions lumbar pain Maternal/fetal compromise

Answer 105

Likely rupture of a spiral artery with haemorrhage into the decidua basalis leading to separation. Classification: Marginal Retroplacental Preplacental

Answer 106

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)

Answer 107

IUGR Fetal demise Maternal exsanguination

Answer 108

Isoechoic: Contraction Placentomegaly Hypoechoic: leiomyoma Subplacental space

Answer 109

Single lobe, bilobe, succenturiate, circumvallate, circummarginate, placenta membranacea, placenta fenestrata, zonary, annular, ring, shorse shoe

Answer 110

Two near equal sized lobes. If smaller, succenturiate. Assoc: velamentous cord insertion, increased risk vasa previa II, post partum haemorrhage

Answer 111

Smaller accessory lobe. Check for vascular connection over os (vasa previa) Complications: type II vasa previa, post partum haemorrhage

Answer 112

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

Answer 113

Similar to circumvallate but not inward rolling. Chorionic membranes insert inward from margin of placental edge.

Answer 114

placenta develops as thin membrane around entire periphery of chorion associations; abnormal adherence complications; praevia, IUGR, antepartum haemorrhage, miscarriage, fetal demise, post partum haemorrhage

Answer 115

Central Eccentric - lateral, >2cm Maringal - <2cm Velamentous - on chorioamniotic membranes

Answer 116

insertion of the cord <2cm from placental margin. also known as battledore. 7% single preg but 25% twins

Answer 117

IUGR, monchorionic twins, abruption, preeclampsia, nuchal cord entanglement, preterm can progress to velamentous

Answer 118

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

Answer 119

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.

Answer 120

1: vessels connect velamentous cord insertion to placenta 2: vessels connect portions of bilobed of succenturiate lobe placenta

Answer 121

multiple gestations low lying succenturiate lobe or bilobed ivf preg

Answer 122

vessels over os

Answer 123

formation of hypoechoic cystic spaces centrally within placenta. Can be abnormal if diffuse or in very early pregnancy

Answer 124

well defined hypoechoic regions low velocity intraplacental laminar flow ddx; chorioangioma; marked internal vascularity

Answer 125

Abnormal proliferation of trophoblastic tissue Encompasses: Hydatiform mole - complete - partial - coexistent Invasive mole Choriocarcinoma Placental site trophoblastic tumour Epithelioid triphoblastic tumour

Answer 126

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

Answer 127

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

Answer 128

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

Answer 129

tumours growth associated with gestation, under GTD spectum. Locally invasive, non metastasising neoplasm.

Answer 130

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

Answer 131

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

Answer 132

EFW and/or AC below the 3rd percentile OR AC below the 10th percentile with deranged Doppler parameters

Answer 133

Maternal conditions; smoking, alcohol, diabetes, malnutrition, vascular conditions Placentral insufficiency Single umbilical artery Fetal; multifetal, infection, chromosomal, mosaicism, syndromic anomalies, in utero exposure

Answer 134

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

Answer 135

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

Answer 136

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

Answer 137

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

Answer 138

EFW >90th percentile

Answer 139

Often truncal obesity and AC first parameter to increase

Answer 140

Early delivery or elective c section. Complications related to issues with delivery and include; birth truam, birth asphyxia, neonatal hypoglycaemia and meconium aspiration

Answer 141

Haemolytic - ABO incompatibility - Rh incompatability Infection - PB19 Haemopoetic - homozygous alpha thalassemia Syndromes - Aase - Fanconi Tumours - placental chorioangioma - infantile haemngioendothelioma - sacrococcygeal teratoma

Answer 142

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

Answer 143

AFI <5 Max vert pocket <2 Two diameter pocket <15cmsq

Answer 144

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

Answer 145

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

Answer 146

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

Answer 147

the degree to which there is invasion of chorionic villi into the myometrium due to defect in devidua basalis

Answer 148

Pathology - accreta; mildest, attached to myomatrium but doesnt invade muscle - increta; intermediate, partially invade myometrium - percreta; severest, penetrate entire myometrium or beyond serosa

Answer 149

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

Answer 150

congenital macrocystic lymphatic malgormation at the cervico facial region, particularly posterior triangle

Answer 151

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

Answer 152

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

Answer 153

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

Answer 154

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

Answer 155

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

Answer 156

Abdominoschisis, usually left sided Ectopia cordis Lower limb anomalies Scoliosis Exencephaly

Answer 157

Macroglossia Otic dysplasia Omphalocoele Localised giagantism Hemihypertrophy Cardiac anomalies Pancreas islet cell hyperplasia Organomegaly

Answer 158

Omphalocoele Ectopia cordis Diaphragmatic defect CV malformations

Answer 159

Extra abdominal herniation of fetal or neonatal bowel loops through a para umbilical wall defect

Answer 160

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.

Answer 161

herniated content right side of cord AC smaller than expected Bowel appears free floating Accompanying oligo or polyhydramnios

Answer 162

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

Answer 163

a congenital malformation of the duodenum requiring prompt correction neonatally. Common, 1 in 5-10000

Answer 164

Early life, duodenal obstruction Distension, vomiting, absent bowel movements Usually just distal to ampulla, so bilious vomiting but can be non bilious

Answer 165

Failure of recanalisation of the bowel lumen that is a solid tube in early fetal life 11-12 weeks

Answer 166

Down syndrome VACTERL Annular pancreas Other atresias

Answer 167

XR Duodenal stenosis Duodenal web Internal malrotation and midgut volvulus These should show distal gas US Choledochal cyst Omental cyst Enteric duplication cyst

Answer 168

Absent continuity of the oesophagus due to inappropriate division of the primitive foregut into the trachea and oesophagus. Most common oesophageal congenital anomaly

Answer 169

Inability to swallow Failure to pass NGT

Answer 170

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

Answer 171

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

Answer 172

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.

Answer 173

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

Answer 174

XR Malrotation with midgut volvulus Contrast enema Total colonic Hirschsprungs Meconium ileus

Answer 175

a congenital abnormality where there is stenosis or absence of a portion of the ileum. Increased with chromosomal abnormalities.

Answer 176

Dilated loops of proximal bowel Microcolon on fluoro US: polyhydramnios, proximal dilated segments

Answer 177

spectrum of anorectal abnormalities ranging from membranous separation to complete absence

Answer 178

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

Answer 179

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

Answer 180

congenital anatomical anomaly resulting in abnormal gut rotation as it returns to cavity during embryogensis, Predisposes to midgut volvulus and internal hernias

Answer 181

GI tract malformations Biliary system malformations Pancreatic malformations CDH Heterotaxy Choanal atresia hypospadias

Answer 182

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

Answer 183

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

Answer 184

a complication of malrotated bowel resulting in proximal bowel obstruction with resultant ischaemia if untreated

Answer 185

Complication of intestinal malrotation Associations Gastroschisis Omphalocele CDH Atresias

Answer 186

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

Answer 187

sterile chemical peritonitis due to intrauterine bowel perforation and spillage of fetal meconium into peritoneal cavity

Answer 188

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

Answer 189

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

Answer 190

Hepatoblastoma - solid, echogenic - pseudocapsule - spoke wheel appearance - less vascular - elevated AFP Mesenchymal harmatoma - predominantly cystic - septations give swiss cheese appearance Metastatic neuroblastoma

Answer 191

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.

Answer 192

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

Answer 193

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

Answer 194

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%

Answer 195

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

Answer 196

Typically young women OCP Anabolic steroids men Glycogen storage diseases - Type 1 von gierke - Type III cori or forbes Obesity Metabolic syndrome DM

Answer 197

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

Answer 198

A non inheritable paediatric cystic renal disease resulting in multiple renal cysts forming in utero

Answer 199

1:2500-4000 Slightly higher for males in uni and females in bilateral

Answer 200

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

Answer 201

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

Answer 202

congenital absence or one or both kidneys. If bilateral (classic potter) than its fatal.

Answer 203

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

Answer 204

Antenatal US: Absent kidney Absent renal artery Contralateral hypertrophy Lying down adrenal sign Bilateral: oligo or anhydramnios, non visualised bladder

Answer 205

Congenital renal anomaly characterised by the abnormal location of one or both kidneys. Can occur as cross fused, thoracic or pelvic

Answer 206

During normal embyrological development there is cephalic migration. Ectopic is arrested migration.

Answer 207

MCDK Ureterocele Patent urachus VUR Vaginal agensis Hypospadias PUJO Other renal, vertebral, GI, GU and spinal anomalies

Answer 208

most common type of renal fusion anomaly. renders the kidney susceptible to trauma and increase risk of calculi/cancer (TCC, wilms, carcinoid).

Answer 209

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

Answer 210

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.

Answer 211

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

Answer 212

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

Answer 213

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

Answer 214

an anomaly where the kidneys are fused and located on the same side of the midline

Answer 215

Inferior cross fusion sigmoid kidney lump kidney disc kidney L shaped kidney superiorly crossed fused left to right ectopy three times more common

Answer 216

90% at least partial fusion 10% unfused Characteristic anterior or posterior notch between the two fused kidneys

Answer 217

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

Answer 218

Congenital megacalectasis Extrarenal pelvis Parapelvic cyst

Answer 219

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

Answer 220

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

Answer 221

abnormal flow of urine from the bladder into the upper urinary tract and is typically encountered in young children

Answer 222

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

Answer 223

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.

Answer 224

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

Answer 225

Low grade - abx Surgical reimplantation for higher grades Endoscopic treatment injceting bulking agent at VUJ may be used

Answer 226

1. limited to ureter 2. up to renal pelvis 3. mild dilatation of ureter and pelvicalyceal 4. tortuous ureter with moderate dilatation, blunted fornices but preserved papillary impressions 5. tortuous ureter with severe dilatation. loss of fornices and papillary impressions.

Answer 227

the most common congenital obstructive lesion of the urethra and a common cause of obstructive uropathy in children. Only seen in males.

Answer 228

Variable, may not be apparent until early infancy if mild. If severe, oligohydramnios and SGA. UTIs are common

Answer 229

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

Answer 230

Young classification 1. most common. Two mucosal folds extend anteroinferiorly from verumontanum and fuse anteriorly at lower level 2. rare, considered a normal variant. Mucosal folds extend along posterolateral urethral wall from ureteric orifice to verumontanum 3. 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.

Answer 231

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

Answer 232

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.

Answer 233

herniation of the bladder through an anterior abdominal wall defect. Severity is variable. Male predilection. Mostly sporadic.

Answer 234

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

Answer 235

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

Answer 236

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)

Answer 237

Fetal omental cyst Fetal mesenteric cyst Meconium pseudocyst Fetal ovarian cyst

Answer 238

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

Answer 239

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

Answer 240

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

Answer 241

unknown. associated with birth trauma, perinatal asphyxia, sepsis and congenital infections (classicaly syphilis) uni or bilateral

Answer 242

US mass above kidney demonstrate changing characteristics on sequential imaging - hyper to iso to hypo to anechoic with or without septations no intrinsic vascularity

Answer 243

Neuroblastoma (vascularity) Subdiaphragmatic pulmonary sequestration (left predilection, aorta supply fetal adrenal cyst

Answer 244

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.

Answer 245

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

Answer 246

Complex cystic mass in suprarenal location. Diffuse vascularity rather than feeding vessel.

Answer 247

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.

Answer 248

Extralobar sequestration - usually left sided, more solid, feeding vessel from aorta. Adrenal haemorrhage - no colour Duplex collecting Mesoblastic nephroma Teratoma

Answer 249

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

Answer 250

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

Answer 251

Arrested development. Cloaca present until 5th week when urorectal septum divides urogenital sinus from anorectum. Urogenital sinus further divides into bladder/urethra and vagina.

Answer 252

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

Answer 253

the most common primary malignant liver tumour in children under four years. Abdominal mass and raised AFP. Rare with slight male predilection.

Answer 254

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

Answer 255

Beckwith WIedemann Hemihypertrophy FAP FAS Gardner syndrome Glycogen storage disease Biliary atresia

Answer 256

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

Answer 257

hepatic mesenchymal hamartoma infantile hemangioendothelioma hepatic mets HCC rhabdomyosarcoma of biliary tree

Answer 258

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)

Answer 259

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

Answer 260

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

Answer 261

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.

Answer 262

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

Answer 263

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

Answer 264

ADPKD Beckwith wiedemann Laurence moon beidl Meck Gruber Renal dysplasia with trisomy 13

Answer 265

more than the usual number of digits. Broadly catergorised into preaxial (radial), post axial (ulnar), and central

Answer 266

Down syndrome VATER association Holt-Oram syndrome Greig cephalopolysyndactyly syndrome Carpenter syndrome Laurin-Sandrow syndrome Fanconi anaemia

Answer 267

trisomy 13 Meckel Gruber syndromee oral-facial-digital syndrome (OFDS) skeletal dysplasias Ellis-van Creveld syndrome asphyxiating thoracic dysplasia - Jeune syndrome

Answer 268

congenital fusion of two or more digits. May be soft tissue or bone.

Answer 269

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

Answer 270

radial angulation at an IPJ, typically fifth finger

Answer 271

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

Answer 272

a congenital genetic disorder resulting in rhizomelic dwarfism. Most common skeletal dysplasia.

Answer 273

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.

Answer 274

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

Answer 275

achondrogenesis campomelic dysplasia thanatophorix dysplasia chondroectodermal dysplasia

Answer 276

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

Answer 277

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

Answer 278

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

Answer 279

extension of fetal ventriculomegaly where dilatation is more severe. Lateral ventricle diameter >15mm.

Answer 280

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

Answer 281

Ventriculomegaly Lateral ventricles >15mm Parenchymal thinning 3mm between choroid and ventricle margin

Answer 282

most common cause of congenital obstructive hydrocephalus

Answer 283

Congenital - webs or diaphragms - gliosis Acquired - extrinsic compression (tumours, avms) - intrinsic (infection, haemorrhage, idiopathic)

Answer 284

Hydrocephalus Near normal posterior fossa Secondary thinning of the cortical mantle and macrocephaly

Answer 285

benign, often transient typically resulting from an infolding of the neuroepithelium

Answer 286

Soft marker Trisomy 18, 21 Klinefelter syndrome Aicardi syndrome

Answer 287

Sonolucent cysts about the lateral ventricles 2nd trimester should go by 26-28weeks Warrants closer surveillance, particularly if large, bilateral, multiple, abnormal maternal screening

Answer 288

- mechanical trauma - severe fetal hypoxia - fetal thrombocytopaenia - maternal thrombocytopaenia (alloimmune, idiopathic, vonwillebrand, medications) - tumour - twin twin transfusion - demise of a co twin

Answer 289

variable depending on location and age - can be seen as irregular hyperechoic mass - as it matures, porencephalic cyst formation or fetal intracranial calcification

Answer 290

the most severe neuralt tube defect, complete absence of cortical tissue. Ranges from holocrania to merocrania.

Answer 291

Failure of closure of the antral end of the neural tube, happens at day 24. Markers - highly eelvated MSAFP

Answer 292

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

Answer 293

a form of NTD where brain tissue and overlying meninges herniate through a defect in the cranium

Answer 294

Form of NTD, due to failure of fusion of the cartilginous neurocranium, membranous neurocranium or viscerocranium. Associations - Chiari malformations - Dandy walker malformations - Meckel Gruber

Answer 295

- Occipital (75%) - Sincipital (frontoethmoidal, interfrontal, craniofacial clefts) - Convexity - basal (10%, intrasphenoidal, temporal, transsphenoidal or whatever)

Answer 296

Antenatal US - may be purely cystic or echoes from brain tissue CT/MRI - better for defining anatomy and contents

Answer 297

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)

Answer 298

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

Answer 299

- hydrocephalus - IC calcification - microcephaly - intrahepatic calc - hepatosplenomegaly - ascites

Answer 300

- multiple calcifications - cerebral/cerebellar atrophy - encephalomalacia - ventriculomegaly - micro or macrocephaly, hydrocephalus - cortical abnorm, rare - chorioretinitis

Answer 301

Features - sensorineural deafness - cataracts - cardiac anomalies - VSD/ToF - intellectual disability - microcephaly - IUGR Worst during 1st trimester Serology - maternal rubella specific igG and IgM

Answer 302

- hydrops, if severe - cardiac anomaly - IUGR - subependymal cysts - small HC - periventricular calc - white matter hypo - ventriculomegaly non specific

Answer 303

most common mostly asx possible features - jaundice/hepatosplenomegaly - microcephaly - sensorineural deafness - chorioretinitis - petechiae - blueberry muffin rash - intellectual disability - seizures complications - encephalitis - hydrops

Answer 304

- 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

Answer 305

broad group of malformations affecting the spine and surrounding structures. Form of NTD

Answer 306

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.

Answer 307

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)

Answer 308

represents a spectrum of structural defects of the caudal region, varying from isolated partial coccyx agensis to lumbosacral agenesis

Answer 309

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.

Answer 310

commonest congenital tumour in fetus and neonate. female predilection.

Answer 311

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

Answer 312

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

Answer 313

high output cardiac failure, hydrops ureteric obstruction GIT obstruction nerve compression anaemia dystocia tumour rupture

Answer 314

chordoma myelocystocoele meningocoele low lying neuroblastoma low lying rhabdomyosacroma small round blue cell tumour enteric cyst

Answer 315

a complication of ovarian stimulation for IVF consisting of ovarian enlargement, extravascular accumulation and intravascular depletion.

Answer 316

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)

Answer 317

bilateral enlarged ovaries, multiple cysts, spoke wheel. ascites, effusions

Answer 318

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

Answer 319

mullerian duct anomaly with assocaited mesoneprhic duct anomaly triad of - uterus didelphys - renal agenesis (or other renal anomalie) - obstructed hemivagina

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