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Trunk and lower extremity > Genital embryology, anatomy, congenital and acquired reconstruction > Flashcards

Genital embryology, anatomy, congenital and acquired reconstruction Flashcards

1
Q

indicate what each germ layer differentiates into for sexual differentiation

A
  • ectoderm: external genitalia
  • mesoderm: internal genitalia (gonads/ovaries/testes); spermatic cord; uterus etc
  • endoderm: cloaca (membrane btwn rectum & bladder/vagina)
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2
Q

how is gonadal sex determined

A

SRY gene on Y chromosome

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3
Q

compare male to female differentiation

A
  • SRY gene induces gonads to become testes
  • testes release testosterone (Leydig cells) and breaks down into DHT and both influence mesonephric duct formation into internal and external (DHT) genitalia
  • testes sertoli cells release mullerian inhibitor substance which causes regression of paramesonephric duct (female stuff)
  • Whereas w female, this is default, therefore SRY gonads become ovaries
  • no testosterone means mesonephric duct does not form
  • no sertoli cells means no MIS and therefore paramesonephric duct develops - internal and upper 1/3 of vagina
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4
Q

Compare the predecessors of external genitalia from female to male

A

Starts 10-11 wks

Male

DHT/androgens from testes sertoli cells induce male ext genitalia

Female

  • placental/fetal estrogen induces female external genitalia

Genital tubercle

Penis

Clitoris

Genital fold

Ventral penis and raphe

Labia minora

Labioscrotal swelling

Scrotum and raphe

Labia majora & mons

  • paramesonephric duct – upper 1/3 vagina
  • UG sinus: lower 2/3 vagina (vaginal plate apoptosis complete @ 22wks)
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5
Q

Describe the arterial supply to the perineum

A
  • anterior triangle - femoral system (medial side CFA) - superficial and deep external pudendal arteries
    • anastomose w internal pudendal system
  • posterior triangle - internal iliac system
    • internal pudendal artery gives rise to perineal artery (scrotal / labial artery)
    • common penile artery has 3 branches
    • whereas dorsal clitoral artery has 1 branch
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6
Q

Compare layers in abdominal wall to layers in scrotum

A
  • skin –> skin
  • subQ, scarpa –> dartos muscle & colle’s fascia
  • external oblique aponeurosis –> external spermatic fascia
  • internal oblique muscle –> cremaster muscle
  • internal oblique fascia –> cremasteric fascia
  • transversalis fascia –> internal spermatic fascia
  • peritoneum –> tunica vaginalis
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7
Q

what is persistent mullerian duct syndrome

A
  • mullerian duct = mesonephric duct = female ductal system
  • genetic male, external genitalia male, internal has testes (descended) as well as small paired ovaries & uterus
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8
Q

what is intersex / androgen insensitivity?

A
  • genetic male
  • phenotypically external genitalia female, although often lack secondary sexual differentiation - ie breast development
  • no internal female characteristics abnormal male duct system including undescended testes
  • due to insensitivity or abnormality to androgen, androgen receptor, 5 alpha-reductase
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9
Q

what is female pseudohermadrotism / intersex?

A
  • genetic female
  • virilization of external female genitalia to appear male
  • female internal duct system (ovaries, uterus)
  • due to congenital adrenal hyperplasia / defect in cortisol synthesis leads to increased testosterone production
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10
Q

what is the etiology of perineal and genital defects?

A
  • congenital
    • male: bladder extrophy (both), hypospadias/epispadias, microphallus
    • female: congenital absence of vagina, congenital labia minora hypertrophy, imperforate hymen
  • acquired
    • trauma
      • blunt, sharp, avulsion, burn
    • tumour - benign/malignant/metastatic
    • infection - ex fournier’s gangrene
    • iatrogenic - radiation injury
    • penile curvatures
    • miscellaneous
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11
Q

what is hypospadius?

A
  • abnormal ventral urethral opening
    • anywhere from corona to base
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12
Q

how is hypospadius classified?

A
  • by locaiton
    • proximal third: penoscrotal, scrotal, perineal
    • middle third: penile shaft
    • distal third: glanular, coronal, subcoronal
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13
Q

what procedure do you want to avoid in hypospadius patients?

A
  • circumcision
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14
Q

what are associated anomalies w hypospadius

A
  • upper urinary - ex: kidney
  • hernia
  • undescended testes
  • meningomyelocele
  • adrenal hyperplasia; hypogonadism
  • imperforate anus
  • down syndrome
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15
Q

what are goals of hypospadius repair?

A
  • release chordee
  • create new urethra
  • advance meatus to tip
  • timing: 6 mos to 3 years to avoid psychosocial sequalle
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16
Q

What are operations for coronal meatal openings in hypospadius

A
  • meatal advancement & glanuloplasty
  • urethral advancement
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17
Q

what are options for distal vs. proximal hypospadius

A
  • indicate never seen procedure as generally performed by urologists / pediatric urologists in my region
  • distal
    • tubularized incised plate (tip) ureteroplasty
    • flip flap technique
  • proximal
    • full thickness graft urethroplasty
    • preputial flap urethroplasty
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18
Q

what is epispadius?

A
  • urethral meatal opening on dorsal surface of penis
  • less common than hypospadius
  • often occurs w/ bladder extrophy
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19
Q

what characterizes an epispadius

A
  • short / flat / wide penis
  • cleft glans
  • dorsal curvature
  • dorsal meatus
20
Q

what are goals of epispadius repair?

A
  • lengthen penis
  • repair dorsal cord
  • urethroplasty
  • dorsal penile skin coverage
21
Q

what is bladder extrophy?

A
  • separation of pubic symphysis
  • abnormality of lower anterior abdominal wall
  • anterior bladder wall is absent and bladder is everted
22
Q

Describe a treatment plan for bladder extrophy

A
  • manage w urology
  • excision of bladder, urinary divesion via ilial conduit
  • abdominal wall clsoure w local flaps
  • reconstruction of neobladder
  • immediate or delayed reconstruction of external genitalia
23
Q

what syndrome is defined by congenital absence of vagina?

A
  • Meyer Rotikansky syndrome
  • congenital abnormality in mullerian duct / paramesonephric duct system
    *
24
Q

what are the clinical findings associated w congenital absence of vagina?

A
  • Two types
  • both have genetic female, absence of vagina,
  • Type I may or may not have non-functioning or absent uterus
  • type II has absent / non-functioning uterus, has also abnormal other lower urinary tract, associated skeletal abn (rib, vertebrae), GIT abnormalities
25
Q

when being consulted to reconstruct vagina in congenital absence of vagina, what will your workup include

A
  • focussed history including ante/peri/post natal history
  • focussed physical exam including pelvic, rectal
  • IVP (intravenous pyelogram) or abdo/pelvis US to rule out LUT abnormality and define reflux
  • skeletal survey including spine XR
  • karyotype
    *
26
Q

What are the treatment options for patient with congenital absence of vagina?

A
  • timing when patient is mature and motivated; approaching age for intercourse
  • nonsurgical = Frank method - graduated vaginal dilators
  • Surgical
    • McIndoe technique dissects the vaginal pocket, uses split or full thickness skin graft, then a long-standing stent x 6 mos then routine long term stenting
    • Local flaps: bilateral singapore (internal pudendal artery) flaps
      • other: posteiror thigh
    • Regional flaps: gracilis (MC), VRAM,
    • Intra-abdominal: colonic, ileal
27
Q

what are the advantages / disadvantages of the surgical options for congenital vaginal absence?

A
  • McIndoe technique involves dissecting the vaginal pocket and lining w F/STSG (usually harvested from suprapubic region)
    • adv: easy, no-minimal donor site morbidity
    • disadv: 6/12 wear mold post op then long-term use of dilators, high failure rate, stenosis, /fistula, dyspareunia
  • Fasciocutaneous flaps: Singapore/internal pudendal
    • adv: local, minimal donor morbidity or visibility, sensate, bilateral, spare abdo
    • disadv: hair-bearing, less bulk
    • other Fasciocutaneous - psoterior thigh
  • Musculocutaneous - VRAM
    • adv: reliable, sufficient length and bulk for total vaginal reconstruction, unilateral
    • disadv: abdominal scar, hernia w/ tunnel (if no laparotomy), recovery, abdo weakness
  • vs MC gracilis - adv: no abdo morbidity
    • disadv: requires bilateral for total, scar, distal skin not reliable, less favourable arc of rotation
  • Intra-abdominal - colon or small bowel
    • adv: low risk of stricture, adequate length, lubrication
    • disadv: laparotomy/laparoscopy, adhesions, permanent vaginal discharge/soilage
28
Q

What are the goals of labiaplasty?

A
  • reduction of labia minor to address functional and cosmetic complaints
  • preserve neurovascular supply
  • preserve introitus
  • achieve optimal colour/texture match along labial edge
  • minimally invasive
29
Q

What are the general considerations when making a plan for genital/perineal reconstruction?

A
  1. Defect / recipient site considerations
    1. Adequacy of local blood supply
    2. Radiation to site
    3. Size of defect and need for dead space obliteration
    4. Invovled (ligated, excised) structures including local vessels/pedicles, ureters, bladder, anorectum, external genitalia
    5. Desire to restore penis/vagina for sexual intercourse
  2. Donor site considerations
    1. scars across donor site / other factors that limit donor availability
  3. Surgical considerations
    1. Patient position during surgery
    2. Patient post-op positioning
    3. Need for diverting urinary or fecal stream
    4. Operative approach: laparotomy / laparoscopy / isolated perineal
    5. Consider entire reconstructive ladder: primary closure, skin graft, local random flap, local pedicle flap, regional flap, free tissue transfer
30
Q

What are general goals of genital / perineal reconstruction?

A
  • Restore pain-free sexual function
  • Effective wound healing to allow timely chemo / rads
  • Phallus
    • structure: sufficient length, width and position
    • function: rigidity for penetration, erogenous and tactile sensation, durable skin for penetration
  • Vagina
    • structure: sufficient stability in depth and breadth
    • function: erogenous and tactile sensation, durable lining capable for intercourse
  • Scrotum
    • structure: sufficient skin to cover testes
    • function: ideally extra-peritoneal position for temperature control for spermatogensis (and avoid malignant degeneration)
  • Restore pelvic floor to prevent herniation of pelvic contents or fistula formation
  • Maintain micturition
  • Maintain proper fecal evacuation
  • obliteration of dead space
  • primary closure in single stage
31
Q

What are options for reconstruction of surface defects of vulva?

A
  • primary closure (vascularity and laxity of surrounding skin)
  • skin graft (when wound bed is well vascularized)
  • local random flaps - rotation, advancement, transposition
  • local named flaps - internal pudendal artery / singapore flap
  • would rarely require regional flaps
  • would almost never require free flaps
32
Q

what are options for reconstruction of vagina or reconstruction of large perineal defect in female?

A
  • skin graft - these are generally not amenable to skin grafting bc the wound bed is not graftable in post-traumatic or post-ablative defects
  • common locoregional fasciocutaneous flaps
    • internal pudendal a / singapore flap (often bilateral)
    • posteiror thigh flap (off IGA)
    • groin SCIA (less commonly d/t limitations w/ arc of rotation)
  • common myogenous/myocutaneous flap
    • rectus abdominus (typically VRAM, TRAM, TRAMP - w/ peritoneum)
    • gracilis
  • free tissue transfer
    • rarely needed due to availability of locoregional options
33
Q
  • list the advantages and disadvantages of your preferred myocutaneous flap for vaginal reconstruction.
  • compare to advantages and disadvantages of an alternate myocutaneous flap.
A

My preferred flap is VRAM

  • adv: reliable, well vascularized muscle and skin paddle, sufficient length and bulk, modifiable paddle, many ablative defects require a laparotomy - therefore abdominal incision would be there anyway, easy to transfer through pelvic when lap, easy/generous arc of rotation even when no lap, fewer recipient site complications
  • disadv: incision/scar for those not needing lap; hernia when pedicled subcutaneous, abdominal weakness, recovery time, may require mesh, more morbidity when obese, thoughtful planning when ostomy required

Alternate choice is gracilis myocutaneous

  • adv: no abdominal morbidity (especially important when lap not required), less recovery and donor site mobidity vs VRAM; perhaps this is better for total vaginal recon (bilateral)
  • disadv: distal 1/3 skin unreliable, often require bilateral for total vagina, overall skin paddle dissection more care vs. VRAM, scar on leg, more difficult when obese, more limited arc of rotation
34
Q
  • list the advantages and disadvantages of your preferred fasciocutaneous flap for vaginal reconstruction
  • contrast to advantages and disadvantages to an alternate fasciocutaneous option
A

My preferred FC flap would be internal pudendal artery flap

  • adv: local, well tolerated donor w minimal morbidity, can harvest bilateral with little consequence, sensate
  • disadv: hair-bearing, less bulk, wihtin the radiation field

An alternative is psoterior thigh flap off IGA

  • adv: sensate, less mrobidity vs. a VRAM
  • disadv: less bulk, less generous arc of rotation, donor site scar and morbidity
35
Q

how are acquired vaginal defects classified and what reconstructive approach is propsed based on this classification

A
  • cordiero et al in 2002
  • 1A: anterior wall - singapore FC flap (uni, bilateral)
  • 1B: posterior wall - VRAM
  • 2A: upper 2/3 vagina - rolled (tubed) VRAM vs. coloplasty
  • 2B: total circumferential vagina - bilateral gracilis mc flaps
36
Q

outline a general reconstructive appraoch to external genital defects in male

A
  • superfical defects of shaft
    • full/split thickness skin graft
  • total / near-total penile reconstruction
    • locoregional flaps are too bulky
    • free tissue transfer preferred
      • tubed (vs cricket paddle) radial forearm preferred
      • others: alt, lateral arm, fibula osteocutaneous
37
Q

list and compare reconstructive options for scrotal reconstruction

A
  • meshed stsg
    • adv: easy, readily available, little to no donor morbidity, well-matched aesthetic result, extraperitoneal position for appropriate temperature
    • disadv: difficult bolster, require tunica albuginea as wound bed
  • medial thick subcutaneous pockets
    • adv: ideal when NV or spermatic cord exposed or absent tunica or non-graftable testes, no immobilization
    • disadv: could be involved in acquired defect, non-anatomic position, non aesthetic reconstruction, theoretically increase temperature / spermatogenesis
38
Q

list options for reconstruction of male perineal defects

A
  • FC
    • singapore
    • posterior thigh
  • MC
    • vram
    • gracilis
39
Q

what is the operative approach to penile replantation?

A
  • start on amputated part
    • identify and dissect the 2 dorsal arteries, 2 nerves, 1 vein
  • identify and dissect proximally the dorsal arteries, vein, nerves on wound base
  • divert urinary stream (suprapubic catheter)
  • repair urethra over foley
  • repair tunica albuginea over corpora
  • undertake arterial, venous anastomoses and nerve coaptations
  • repair dartos fascia & skin
40
Q

What are complications after reconstruction of female or male acquire genital/perineal defect?

A
  • Early
    • post op infection: cellulitis, superficial collection, deep pelvic abscess
    • delayed wound healing &/or dehiscence; common at distal tip
    • partial necrosis & total flap failure
    • Delay in adjuvant therapy
  • Late
    • rectovaginal, urethrovaginal fistula
    • urethral stenosis
    • abdominal bulge/hernia
    • fat necrosis
    • psychologic factors pertaining to reconstruction, intimacy
    • vaginal: stenosis, dryness, fistula, need for repeat stenting/dilatation, dyspareunia, excess tissue
    • male: inability to obtain/maintain erection; insufficient rigidity to permit penetration
41
Q

What are the advantages, disadvantages and potential modifications of the free radial forearm flap for total penile reconstruction?

A
  • advantages
    • reliable, well vascularized flap
    • long vascular pedicle
    • thin
    • large skin paddle permits double-tube (tube wihtin tube) design
    • sensate
    • may not be large enough to permit graft for rigidity
  • disadvantages
    • donor morbidity
    • potential for hair-bearing skin within urethra
  • modifications
    • for rigidity: autogenous options - vasc bone (partial radius O-C flap), costal cartilage, NVBG, alloplast
    • cricket bat flap (longitudinally oriented over radial artery, distal narrower component is tubed over foley and folded 180’ to wihtin the more proximal paddle (shaft)
42
Q

indicate which donor vessels go to which recipient vessels for free radial forearm flap for total penile reconstruction

A
  • radial artery –> LCFA or PFA (ETS)
  • cephalic vein –> greater saphenous
  • LABC –> internal pudendal vs. ilioinguinal
43
Q

what are complications after total penile reconstruction using free tissue transfer?

A
  • insufficient rigidity for penetration
  • lack of erogenous / tactile sensation; inability to obtain erection
  • urethral stricture
  • fistula
  • flap loss
  • hair
44
Q

what are the overarching goals of surgery in gender reassignment?

A
  • single stage
  • aesthetically please
  • erogenous sensibility
  • minimal morbidity
45
Q

over-arching steps for male to female

A
  • hormone therapy, introduction to society as female
  • psychiatric clearance
  • surgical interventions
    • breast augmentation
    • rhinoplasty
    • thyroid cartilage reduction
    • epilation of male pattern hair
    • feminizing genital surgeries: penectomy, penile inversion, skin grafts, instestinal substitution
46
Q

over arching steps for female to male gender reassignment

A
  • hormone therapy and introduction to society as male
  • pyschiatric clearance
  • surgeries
    • breast ampuation or reduction
    • hysterectomy/oopherectomy
    • penile construction
    • neoscrotal reconstruction

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