Learn — Reproductive & genitourinary (adult)

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Hypospadias

Hypospadias is a congenital anomaly in which the external urethral meatus opens somewhere on the ventral (under) surface of the penis — anywhere from the glans to the perineum — because the urethral folds fail to fuse completely between the 8th and 14th weeks of gestation. It matters perioperatively because the hooded foreskin is the reconstructive material for the repair, so the baby must never be circumcised, and because the repair is timed for infancy, before the child develops genital awareness or is toilet-trained to void standing.

Classified by the site of the meatus after the chordee has been straightened: glanular, coronal, distal penile, midshaft, proximal penile, penoscrotal and perineal; about 70% are distal and carry the best functional result.
The triad is a ventrally placed meatus, chordee (ventral curvature of the penis, worst on erection) and a dorsally hooded prepuce that is deficient ventrally.
Do not circumcise, and stop any planned ritual circumcision — the prepuce supplies the skin for urethroplasty and ventral cover, and removing it converts a straightforward repair into a graft procedure.
Associated anomalies: undescended testis in about 10% and inguinal hernia; hypospadias with bilateral impalpable testes is a disorder of sex development until karyotype, 17-hydroxyprogesterone and endocrine review say otherwise.
Repair is done between 6 and 18 months of age (commonly 6 to 12 months), when the anaesthetic risk is low, the penis is large enough to operate on and the child is still before the genital awareness stage.
Common operations: MAGPI for a glanular meatus, tubularised incised plate (Snodgrass) urethroplasty for distal defects, and staged repair with preputial or buccal mucosa grafts for proximal and severe chordee cases.
After repair a urethral stent, drip-stent or Foley diverts urine for about 5 to 14 days; the dressing is left undisturbed until the surgeon removes it, the catheter is secured and kept unkinked and freely draining, and an anticholinergic such as oxybutynin plus antibiotic prophylaxis is given for bladder spasm.
The commonest complications are urethrocutaneous fistula, meatal stenosis, urethral stricture and glans dehiscence; a fistula shows as urine escaping from a second opening and is repaired only after at least 6 months, when the tissues have softened.
Function, not appearance, is the reason for surgery: an untreated proximal hypospadias gives a downward spraying stream that cannot be directed standing, and uncorrected chordee interferes with intercourse and insemination later.

Ectopia vesicae

Ectopia vesicae, or bladder exstrophy, is a midline closure defect in which the infra-umbilical abdominal wall and the anterior wall of the bladder are both absent, so the posterior bladder wall lies open and everted on the lower abdomen with urine dribbling from the visible ureteric orifices. It is the middle of the exstrophy–epispadias complex and always comes with epispadias and a wide pubic diastasis, and it matters from the first minutes of life because the exposed mucosa must be protected from drying, abrasion and latex until staged reconstruction can be done.

Incidence is about 1 in 30,000 to 50,000 live births, with boys affected two to three times as often as girls.
The spectrum runs from epispadias alone, through classic bladder exstrophy, to cloacal exstrophy in which bowel is exstrophied as well; classic exstrophy shows an open bladder plate, a low-set umbilicus, wide pubic diastasis and a short, upward-pointing epispadiac penis or a bifid clitoris with a wide vagina.
Delivery-room care decides the outcome of the plate: cover it with clear plastic wrap (cling film), never with gauze or paraffin dressings that stick and strip the mucosa when removed, irrigate with warm saline at each nappy change, and tie the cord with a silk ligature instead of a plastic clamp that abrades the bladder.
All care is latex-free from birth, because these children face repeated operations and have a high rate of latex sensitisation and anaphylaxis.
Reconstruction is either the modern staged repair — bladder and posterior urethral closure with or without pelvic osteotomy in the first 48 to 72 hours or delayed, epispadias repair at 6 to 12 months, and bladder-neck reconstruction at about 4 to 5 years when the child can cooperate with continence training — or complete primary repair in a single operation; osteotomy allows the pubic bones to be brought together and takes tension off the closure.
Postoperative nursing is immobilisation as prescribed (modified Bryant's or spica traction after osteotomy), meticulous care of the ureteric stents and suprapubic tube, strict output charting from each tube separately, and prevention of abdominal distension that could burst the repair.
Long-term problems are vesicoureteric reflux and recurrent urinary infection in almost all, incontinence needing bladder-neck surgery or augmentation, genital and fertility issues, and a raised lifetime risk of bladder adenocarcinoma — so follow-up is lifelong.

Undescended testis

Undescended testis (cryptorchidism) is failure of the testis to complete its normal descent into the scrotum, so it lies anywhere along the path from the posterior abdominal wall to the superficial inguinal pouch and cannot be brought into the scrotum and left there. It is the commonest genital anomaly of boys — about 3 to 5% of term and up to 30% of preterm male newborns — and it matters because spontaneous descent stops by 3 to 6 months of age, after which germ cells are progressively lost and orchidopexy must be scheduled.

Spontaneous descent is almost complete by 3 months of corrected age and never happens after 6 months, so a testis still undescended at 6 months is a surgical problem, not a wait-and-see one.
A retractile testis has a brisk cremasteric reflex, can be milked down into the scrotum and stays there — it is a normal variant needing only annual review; a true undescended testis cannot be brought down or springs straight back, and an ectopic testis lies off the line of descent (superficial inguinal pouch, femoral, perineal or prepubic).
Orchidopexy is done between 6 and 12 months of age and by 18 months at the latest: earlier surgery preserves more germ cells and places the testis where it can be examined for a tumour.
Untreated risks are impaired fertility (much worse when bilateral), a five- to ten-fold increase in testicular malignancy — usually seminoma, and slightly raised in the normally descended contralateral testis too — torsion, trauma against the pubic bone, and an associated patent processus vaginalis causing indirect inguinal hernia in most cases.
Imaging is not indicated for a non-palpable testis: ultrasound cannot reliably find or exclude an intra-abdominal testis, so diagnostic laparoscopy is the standard, with a staged Fowler–Stephens orchidopexy when the testis is high in the abdomen.
Hormonal treatment with hCG or GnRH is no longer recommended as primary therapy — success rates are low and there is no proven fertility benefit; the treatment is surgical.
Bilateral non-palpable testes at birth, particularly with hypospadias, is a disorder of sex development until karyotype, 17-hydroxyprogesterone, electrolytes and endocrine review say otherwise.
After day-case orchidopexy: scrotal support and regular analgesia, keep the wound dry, no straddle toys, cycling or rough play for 2 to 4 weeks, and teach monthly testicular self-examination from puberty for life because the tumour risk remains after the testis is brought down.

Testicular torsion

Testicular torsion is twisting of the spermatic cord on its own axis, which first occludes venous drainage and then arterial inflow, so the testis swells, becomes ischaemic and infarcts. It is the most time-critical emergency in urology: salvage approaches 90 to 100% if the cord is untwisted within 6 hours of the onset of pain, falls to around 50% by 12 hours and to under 10% after 24 hours, so the diagnosis is made clinically and the boy goes to theatre.

There are two peaks — the neonatal period, where the torsion is extravaginal (the whole tunica twists and the testis is usually already lost), and 12 to 18 years, where it is intravaginal on a bell-clapper deformity in which the tunica vaginalis invests the testis completely and leaves it swinging free.
The history is sudden severe unilateral scrotal pain, often waking the boy from sleep or coming on after sport or minor trauma, with nausea and vomiting and pain referred to the lower abdomen — every boy with acute abdominal pain must have his scrotum examined.
Signs: a high-riding testis lying transversely, a swollen, exquisitely tender hemiscrotum, an absent cremasteric reflex on that side (the single most useful sign), and a negative Prehn's sign — elevating the testis does not relieve the pain, whereas it does in epididymo-orchitis.
Colour Doppler ultrasound shows absent or reduced intratesticular flow and a whirlpool sign in the cord, but imaging must never delay exploration when the history and signs are classic, and normal flow does not exclude an intermittent or early torsion.
The differential is epididymo-orchitis (gradual onset, fever, dysuria, cremasteric reflex present, positive Prehn's sign), torsion of the appendix testis (localised upper-pole tenderness with a blue dot), idiopathic scrotal oedema, incarcerated hernia and trauma.
Treatment is emergency scrotal exploration: the cord is untwisted, the testis wrapped in warm saline packs and observed, and a viable testis is fixed with three-point non-absorbable sutures — the opposite testis is fixed at the same operation because the bell-clapper deformity is bilateral; a black, non-viable testis is removed.
Manual detorsion is only a holding measure while theatre is prepared: the testis usually rotates medially, so it is rotated laterally like opening a book; relief of pain and lengthening of the cord suggests success, but exploration and fixation still follow.
Nursing preparation from the moment torsion is suspected: nil by mouth, intravenous access and bloods, consent and theatre informed, adequate analgesia (it does not mask the diagnosis), and no ice packs or repeated manipulation of the scrotum.

Scrotum — conditions

The scrotal swellings and pains are a short list that is distinguished almost entirely at the bedside by four questions: is it painful, does it transilluminate, can you get above it, and is the testis itself involved. Getting the answer right matters because one member of the list — testicular torsion — is a surgical emergency with a salvage window measured in hours, while another, a painless testicular mass, is a curable cancer that is ruined by the wrong biopsy.

Testicular torsion is the emergency: sudden severe pain with nausea and vomiting, usually in a boy or young man, with a high-riding testis lying transversely, a swollen tender hemiscrotum, an absent cremasteric reflex (the most useful single sign) and pain that is not relieved by elevating the testis (negative Prehn sign). Salvage is close to 90 to 100% within 6 hours and falls to almost nothing beyond 24 hours.
Torsion is treated by immediate surgical exploration with untwisting and bilateral orchidopexy — the other side is fixed too because the underlying bell-clapper deformity is bilateral; a Doppler ultrasound is done only if it does not delay theatre, and a normal scan never overrides the clinical picture.
Epididymo-orchitis is the main mimic: pain builds over days with fever, dysuria and urethral discharge, the cremasteric reflex is present, and elevating the testis eases the pain (positive Prehn sign). The organism is chlamydia or gonococcus under about 35 years and coliforms above it, so the treatment differs and partner treatment is needed in the younger group.
Hydrocele is a painless collection of fluid in the tunica vaginalis that transilluminates brilliantly, and you can get above it; a filarial hydrocele is common in endemic parts of India, and a hydrocele in a young man that appears rapidly must prompt an ultrasound for an underlying tumour.
Varicocele feels like a bag of worms above the testis, is more prominent on standing and disappears on lying down, and is on the left in about 90% of cases because the left testicular vein drains at a right angle into the left renal vein; a right-sided varicocele or one that does not empty on lying flat suggests a retroperitoneal or renal tumour and demands imaging.
Testicular tumour is a painless, hard, non-tender mass arising from the testis itself, which does not transilluminate; markers are alpha-fetoprotein (raised in yolk sac and embryonal tumours and never in a pure seminoma), beta-hCG and LDH.
A suspected testicular tumour is never biopsied through the scrotum, because that breaches the lymphatic drainage and seeds the inguinal nodes; the operation is a high inguinal orchidectomy, and sperm banking is offered before chemotherapy.
Inguinoscrotal hernia is the swelling you cannot get above, with a cough impulse and bowel sounds over it; sudden pain with irreducibility, vomiting and absent bowel sounds means strangulation and is a surgical emergency.
Undescended testis is corrected by orchidopexy between 6 and 12 months of age (and no later than about 18 months) to protect fertility; the cancer risk remains raised even after surgery, but the testis becomes examinable.
Fournier gangrene is necrotising fasciitis of the perineum and scrotum, typically in a diabetic or immunosuppressed man, with pain out of proportion to the findings, crepitus, dusky skin and systemic toxicity; it needs resuscitation, broad-spectrum antibiotics and immediate radical debridement.

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