HYPOSPADIASEPISPADIAS Dr Enivwenae O A NITMED TUTORIALS OUTLINE

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HYPOSPADIAS/EPISPADIAS Dr Enivwenae O. A. © NITMED TUTORIALS

HYPOSPADIAS/EPISPADIAS Dr Enivwenae O. A. © NITMED TUTORIALS

OUTLINE: INTRODUCTION EPIDEMIOLOGY RELEVANT ANATOMY/ EMBRYOLOGY CLINICAL PRESENTATION INVESTIGATIONS TREATMENT COMPLICATIONS FOLLOW-UP

OUTLINE: INTRODUCTION EPIDEMIOLOGY RELEVANT ANATOMY/ EMBRYOLOGY CLINICAL PRESENTATION INVESTIGATIONS TREATMENT COMPLICATIONS FOLLOW-UP

INTRODUCTION: -Congenital defects of the external genitalia. -Disturbing for Parent/patient -Surgery : Challenging/Technically demanding

INTRODUCTION: -Congenital defects of the external genitalia. -Disturbing for Parent/patient -Surgery : Challenging/Technically demanding -Changing epidemiologic trends

HYPOSPADIAS: • Most common male ext. genitalia anomaly • Abnormal urethral opening on the

HYPOSPADIAS: • Most common male ext. genitalia anomaly • Abnormal urethral opening on the ventral aspect of the penis • 1 in 125 to 250 male births • Rare in females (1 per 1, 000)

AFRICA: • • Unawareness Deleterious cultural/religious beliefs Late referral Already circumcised/Mutilation Suboptimal theatre Lack

AFRICA: • • Unawareness Deleterious cultural/religious beliefs Late referral Already circumcised/Mutilation Suboptimal theatre Lack of fine instruments/sutures High infection rates

EMBRYOLOGY: • • • Genital tubercle-> Glans Urethral folds-> Penile urethra Genital swellings-> Scrotum

EMBRYOLOGY: • • • Genital tubercle-> Glans Urethral folds-> Penile urethra Genital swellings-> Scrotum Urethral folds fuse cranio-caudally Arrest/Failure of complete fusion of plate >Hypospadias

 • 3 rd WK gestation – Cloacal folds formation • 4 th WK

• 3 rd WK gestation – Cloacal folds formation • 4 th WK gestation – Genital tubercle (Cloacal folds anteriorly) – Urethral folds (Caudally/anteriorly)Distal urethra - last to fuse

 • Genital swellings->Scrotal swelling • 6 th WK – Genital tubercle elongation->Phallus (Androgen)

• Genital swellings->Scrotal swelling • 6 th WK – Genital tubercle elongation->Phallus (Androgen) • Formation of urethral plate

NORMAL PENILE ANATOMY • 2 Corpora Cavernosa, • Corpus Spongiosum Enclosed In A Fascial

NORMAL PENILE ANATOMY • 2 Corpora Cavernosa, • Corpus Spongiosum Enclosed In A Fascial Sheath- Tunica Albuginea • Bucks Fascia, Thick Fibrous Envelope • Connective Tissue, Dartos Fascia

Associated anomalies • Genital anomalies – Chordee – Torsion – Corporal disproportion – Undescended

Associated anomalies • Genital anomalies – Chordee – Torsion – Corporal disproportion – Undescended testes • Upper urinary tract • Others

AETIOLOGY: • Hypospadias (Unknown) – Multifactorial – Endocrine(Andr. synth. /receptor/h. CG response) – Genetic(Familial-7%)

AETIOLOGY: • Hypospadias (Unknown) – Multifactorial – Endocrine(Andr. synth. /receptor/h. CG response) – Genetic(Familial-7%) – Environmental contamination (Insecticides, Pharmaceuticals/ Plant estrogen)-> Worldwide ^

HYPOSPADIAS Risk factors q 8% of patients have father with hypospadia q 14% of

HYPOSPADIAS Risk factors q 8% of patients have father with hypospadia q 14% of patients have male siblings with hypospadias q. If child with hypospadia risk to next child q 12% risk with negative family history q 19% if cousin or uncle with hypospadias q 26% if father or sibling

HYPOSPADIAS Risk factors • Familial inheritance-5 alpha reductase type II mutation • Assisted reproductive

HYPOSPADIAS Risk factors • Familial inheritance-5 alpha reductase type II mutation • Assisted reproductive technology • Advanced maternal age • Paternal subfertility

PATHOLOGY: • Hypospadias – Structure(Ventral meatus may appear stenotic) – Urethral plate(Atretic distal urethra/Corpus

PATHOLOGY: • Hypospadias – Structure(Ventral meatus may appear stenotic) – Urethral plate(Atretic distal urethra/Corpus Spong. – Splayed spade-like glans – Prepuce with a ventral defect(Dorsal hood)

 • Chordee – Skin tethering over atretic corpora spong. – Fibrous band deep

• Chordee – Skin tethering over atretic corpora spong. – Fibrous band deep to urethra – Short urethra – Corporal disproportion

CLASSIFICATION: • Hypospadias: • Standard (based on location of meatus) – Anterior/Middle/Posterior – Proximal/Distal

CLASSIFICATION: • Hypospadias: • Standard (based on location of meatus) – Anterior/Middle/Posterior – Proximal/Distal – Mild/Moderate/Severe • Isolated/Syndromic

ASSOC. ANOMALIES: • Hypospadias: – Undescended testes (9. 3%) – Inguinal hernia(9%-Ant. /17%-Post. )

ASSOC. ANOMALIES: • Hypospadias: – Undescended testes (9. 3%) – Inguinal hernia(9%-Ant. /17%-Post. ) – – Opitz syndrome Smith-Lemli-Opitz syndrome Wolf-Hirschhorn syndrome Denys-Drash syndrome

Clinical features: • Abnormal urethral opening at birth • Family history. Co-morbidities • Assess

Clinical features: • Abnormal urethral opening at birth • Family history. Co-morbidities • Assess meatal location, glans configuration, skin coverage and chordee • Chordee apparent or only visible during erection • Bifid scrotum, transposition of penis.

INVESTIGATIONS: • • Diagnosis usually clinical Abd. USS Preoperative work up (+Urine m/c/s) Hormonal

INVESTIGATIONS: • • Diagnosis usually clinical Abd. USS Preoperative work up (+Urine m/c/s) Hormonal assay

INVx: • Urinalysis • MCUG • Karyotype

INVx: • Urinalysis • MCUG • Karyotype

Management: • Counsel • Definitive treatment(Surgery) – Single stage/Multistage – Inpatient/Outpatient – Use of

Management: • Counsel • Definitive treatment(Surgery) – Single stage/Multistage – Inpatient/Outpatient – Use of penile tissue/Substitutes

Surgical Correction Approach: • Staged – Correction of chordee – Widening or creating urethral

Surgical Correction Approach: • Staged – Correction of chordee – Widening or creating urethral plate – Later reconstruction • Single stage

Surgical Correction Timing: • Best done at age > 6 mths • If phallus

Surgical Correction Timing: • Best done at age > 6 mths • If phallus too small, may be helped by: – Testosterone cream application – Controlled i/m testosterone given monthly for not >3 months.

Selection of technique: • Best decided at surgery, after intraoperative re-evaluation of abnormality. –

Selection of technique: • Best decided at surgery, after intraoperative re-evaluation of abnormality. – Site of meatus – Mobility of urethra – Furrowing of glans – Quality of perimeatal skin (should be pliable) – Chordee – Prepuce – Ventral shaft skin

AIMS OF Tx: • COSMESIS-Conically shaped symmetrical glans - Slit-like ext urethral meatus -

AIMS OF Tx: • COSMESIS-Conically shaped symmetrical glans - Slit-like ext urethral meatus - Straight penile shaft without torsion NORMAL VOIDING -straight flow of urine stream - no straining - no post void dribbling continence SEXUAL FUNCTION- Painless erection - Normal insemination - Satisfactory sexual function

STEPS: • • Assessment Chordee correction Urethroplasty Protective intermediate layer Meatoglanuloplasty Scrotoplasty Skin cover

STEPS: • • Assessment Chordee correction Urethroplasty Protective intermediate layer Meatoglanuloplasty Scrotoplasty Skin cover

PRINCIPLES: • • • Timing/Optimization Preop hormonal Tx Instruments Magnification Haemostasis

PRINCIPLES: • • • Timing/Optimization Preop hormonal Tx Instruments Magnification Haemostasis

PRINCIPLES • • • Suture materials & technique Stents & catheters Dressings Postop analgesia

PRINCIPLES • • • Suture materials & technique Stents & catheters Dressings Postop analgesia Antibiotics

INTRAOP CHORDEE ASSESSMENT: • Artificial erection test(Gittes & Mc. Laughlin) – Normal saline •

INTRAOP CHORDEE ASSESSMENT: • Artificial erection test(Gittes & Mc. Laughlin) – Normal saline • Penile traction

Type of hypospadias Anterior Glanular Subcoronal no chordee, good perimeatal skin poor ventral skin,

Type of hypospadias Anterior Glanular Subcoronal no chordee, good perimeatal skin poor ventral skin, stenosis Type of surgery MAGPI Mathieu TIP(Snodgrass) TPIF Onlay, TIP Middle No chordee Chordee Island flap onlay, TIP Island flap tubularized Posterior Island flap tubularised, combinations, staged Circumcised, re-do Combinations of techs, mucosal grafts; staged

Surgical Correction correction of chordee • Skin chordee – Mobilisation of penile shaft skin

Surgical Correction correction of chordee • Skin chordee – Mobilisation of penile shaft skin – Byars flaps • Fibrous chordee – Complete excision • Corporeal disproportion/short urethra – Dorsal plication Ventral excision of defective segment of tunica albuginea + skin grafting Confirm adequate correction by artificial errection.

Modified MAGPI

Modified MAGPI

Mathieu Repair

Mathieu Repair

Surgical Options Mathieu technique (Flip-flap)

Surgical Options Mathieu technique (Flip-flap)

Transverse preputial island flap of Duckett

Transverse preputial island flap of Duckett

Tubularized incised plate (Snodgrass)

Tubularized incised plate (Snodgrass)

 • Early – – – Complications of repair: Penile oedema Bleeding and haematoma

• Early – – – Complications of repair: Penile oedema Bleeding and haematoma Wound infection Complete dehiscence Dysuria and urinary retention • Late – – – Urethroctaneous fistula 3 -45% Redundant foreskin Meatal stenosis Persisting chordee Penile torsion

Outcome: • With current methods of treatment, results are good. • First time is

Outcome: • With current methods of treatment, results are good. • First time is best. • Each repeat operation increases risk of complication and fistula formation.

Epispadias: • • • Rare 1 in 120, 000 male live births 1 in

Epispadias: • • • Rare 1 in 120, 000 male live births 1 in 500, 000 female live births M: F , 2. 3 -5: 1 Urethra opens on dorsum of the penis(Male) Fissure in upper wall of urethra (Female)

AETIOLOGY: • Epispadias – Explained by defective migration of paired primordia of genital tubercle

AETIOLOGY: • Epispadias – Explained by defective migration of paired primordia of genital tubercle to fuse in midline 5/52 – Considered varying degrees of a single disorder of Epispadias-Bladder exstrophy complex

 • Epispadias: • Boys-Wide open/ healthy urethral plate along dorsum of penis on

• Epispadias: • Boys-Wide open/ healthy urethral plate along dorsum of penis on variable length , hood on ventral surface • Girls-Bifid clitoris, wide open urethral plate , normal vagina

CLASSIFICATION: • Epispadias: • Based on the location of the meatus – Glanular/Balanic-5% –

CLASSIFICATION: • Epispadias: • Based on the location of the meatus – Glanular/Balanic-5% – Penile-20% – Penopubic-75%

PRINCIPLES: • • Timing of repair-12 -18 months Use of testosterone may be necessary

PRINCIPLES: • • Timing of repair-12 -18 months Use of testosterone may be necessary Adequate mobilisation of urethral plate Chordee correction Corporal lengthening/approximation Inferior displacement of the urethra Avoid tension repairs

SURGICAL METHODS: • Cantwell-Ransley procedure • Total penile disassembly

SURGICAL METHODS: • Cantwell-Ransley procedure • Total penile disassembly

 • Cantwell-Ransley procedure • Total penile disassembly

• Cantwell-Ransley procedure • Total penile disassembly

Cantwell - Ransley repair

Cantwell - Ransley repair

Total penile disassembly

Total penile disassembly

POSTOP COMPLICATIONS • Fistula • Hypospadias • (Complete breakdown)

POSTOP COMPLICATIONS • Fistula • Hypospadias • (Complete breakdown)