Pediatric Surgery 66 Genitourinary system

• The ectopic testis probably occurs due to rupture of the main scrotal tail of the gubernaculum testis. According to Lockwood, the gubernaculum testis has five tails. 
• 1. The scrotal tail - which is the main one 
• 2. Pubic tail - attached to the pubic tubercle 
• 3. Perineal tail - attached to perineum 
• 4. Inguinal tail - attached to the front of the inguinal canal 
• 5. Femoral tail - Attached to the saphenous opening.

The scrotal tail is the strongest and the other tails normally disappear and that is why the testis normally descends on the scrotum. In case one of the four accessory tails becomes stronger, the testis is drawn towards the attachment of that tail and then the testis is called as ectopic testis.

• Testis develops at the level of L1 vertebra in the retroperitoneum at the 2nd month of intrauterine life.
• During 2nd to 3rd month it traverses the retroperitoneum and reaches right iliac fossa at the end of third month.
• Reaches the deep inguinal ring by 7th month of intrauterine life.
• It traverses the inguinal canal between 7th to 9th month.
• It reaches the bottom of the scrotum at the end of 9th month.

• Cryptorchidism is a condition in which one testis or both testes fail to descend into the scrotum before birth.
• Some undescended testes eventually descend by 1 year of age.
• The undescended testis is associated with histologic and morphologic changes as early as 6 months of age; atrophy of Leydig cells, decrease in tubular diameter, and impaired spermatogenesis can occur by 2 years of age.

A retractile testis is a normally descended testis that retracts into the inguinal canal but can be brought down into the scrotal sac during the examination. It is thought to result from a hyperreflexive cremasteric muscle contraction and does not require operative intervention.

Retractile testis can be be differentiated from ectopic testis by chair test (Orr).

Ectopic testes have had an aberrant path of descent; these can be found in perineal, femoral canal, and suprapubic regions.

Clinical examination

The recommended timing of orchidopexy is  6 months to 1 year of age.

For a unilateral palpable testis in the inguinal canal, standard dartos pouch orchidopexy is performed.

• For unilateral non-palpable testis - perform diagnostic laparoscopy.
• - If the testicular vessels are seen exiting the internal ring - open inguinal orchidopexy
• - If Blind-ending vessels - diagnosis is Monorchia. Excision of remnent can be done.
• - If intraabdominal testis - Perform double stage orchidopexy or single stage orchidopexy.

• For bilateral non-palpable testis - a hCG stimulation test is carried out to confirm the presence of functioning testes.
• - If no response, diagnose as Anorchia.
• - If Increased testosterone (and increased FSH, LH), diagnostic laparoscopy is performed to identify the testes and to determine surgical therapy.

• Although orchidopexy does not decrease the malignancy risk associated with undescended testes, it allows earlier detection.
• Nonseminomatous germ cell tumors are usually associated with undescended testes

Fowler-Stephens orchidopexy is a two stage orchidopexy in which, testicular vessels are ligated as a first stage to allow collateral circulation from vas and cremesteric vessels to develop for 6 months before orchidopexy is performed as a second stage of the procedure.

It is an emergency condition of the testis, wherein the testis twists (rotates) in its axis compromising its blood supply. If not intervened and rectified within 12-24 hours, testicular gangrene may occur.

• Extravaginal torsion is more common in neonates, in whom there can be torsion of the spermatic cord along its course outside the tunica vaginalis.
• Intravaginal torsion is associated with bell clapper deformity, in which the suspended testis can twist to torsion.

• • Occurs in children and adolescents.
• • Presents with sudden onset of pain in the scrotum, groin and lower abdomen.
• • Vomiting due to pylorospasm.
• • On examination, the testis that has undergone torsion may be high-riding, edematous, and significantly tender.
• – Deming’s sign: Affected testis is positioned high because of twisting of cord and spasm of cremaster muscle.
• – Angell’s sign: Opposite testis lies horizontally because of the presence of mesorchium

• Acute epididymo-orchitis—elevation of the scrotum relieves the pain of acute epididymo-orchitis but aggravates in case of torsion testis (Prehn’s sign).
• Urinary tract symptoms, such as frequency, urgency, dysuria, and fever, tend to occur more frequently with infectious or inflammatory conditions, such as epididymitis; however, in no way does this rule out testicular torsion.

• Ultrasonography may be helpful to determine vascular
• flow to the testicles.

• Emergency exploration of the scrotum and untwisting of the torsion testis is done. Then viability of testis is checked. Once confirmed, the testis is fixed to the scrotal sac using 2-3 interrupted nonabsorbable sutures (Prolene).
• If surgery is done within 12-24 hours, viability of the testis may be retained. If delayed, testis becomes gangrenous and orchidectomy is done after taking informed consent.
• When in doubt, testis is retained and orchidectomy can be postponed to later period so as to give a chance to have a viable testis.
• In all cases, the contralateral testis should also be fixed in the scrotum.

• • Normal flow of urine is always from ureter to bladder through ureteric orifice. It is called as efflux.
• • If urine enters the ureter from the bladder through ureteric orifice, it is called as reflux. It is always pathological.

• • Congenital: It is commonly associated with posterior urethral valve.
• • Acquired: Trauma, after surgery or intervention.

Vesicoureteric reflux is graded depending on the severity of the reflux

• 1 Into a nondilated ureter
• 2 Into the pelvis and calyces without dilatation
• 3 Mild to moderate dilatation of the ureter, renal pelvis, and calyces with minimal blunting of the fornices
• 4 Moderate ureteral tortuosity and dilatation of the pelvis and calyces
• 5 Gross dilatation of the ureter, pelvis, and calyces; loss of papillary impressions; and ureteral tortuosity

• • Features of recurrent urinary tract infection.
• • Renal failure in bilateral cases.
• • Palpable kidney mass in the loin.

• • Urine microscopy and C/S.
• • Blood urea and serum creatinine.
• • IVU.
• • Micturating cystourethrogram (MCU).

• • Tailoring of the ureter with ureteric re-implantation.
• • Antibiotics.
• • In severe cases nephrectomy, renal transplantation are required.

• • They are congenital symmetrical valves in the posterior urethra, just below the verumontanum.
• • It allows the passage of catheter without obstructing its ingress. But it obstructs the outflow of urine.
• • Clinical features range from newborns with life-threatening renal and pulmonary conditions to older children with minor voiding dysfunction
• • Proximal urethra is enormously dilated with obstructive pathology in the bladder (sacculations and diverticula formation).
• • Bladder wall is thickened and  hypertrophied so much so that it is palpable in supra pubic region as a firm swelling (cricket–ball bladder).
• • There is poor urinary stream, with hydronephrosis, often with infection.
• • Child finds difficult to pass urine.
• • Often it is associated with vesico-ureteral reflux.
• • Renal failure and atrophy of kidney occurs due to back pressure.
• • Oligohydramnios leading to Pulmonary hypoplasia is the most common cause of mortality in valve patients.

• • The cystogram may show vesicoureteral reflux and the severe trabeculations of long-standing obstruction, and the voiding cystourethrogram often demonstrates elongation and dilatation of the posterior urethra, with a prominent bladder neck
• • U/S abdomen - to detect hydronephrosis, hydroureter, and bladder distention
• • Blood urea and serum creatinine.
• • IVU.
• • Antenatal US shows fetal urinary tract dilatation.

• Initial management of all patients with posterior urethral valves requires the immediate establishment of urinary catheter drainage from the bladder.
• The ill newborn with posterior urethral valves is usually in the hands of the neonatal service for the management of the most threatening issues such as pulmonary hypoplasia and renal insufficiency.
• After successful initial bladder drainage and when the patient’s medical condition has stabilized, the next step is to permanently destroy the valves.
• If the infant is too small to instrument safely for valve ablation, then a cutaneous vesicostomy can be performed as a temporary measure.
• Initial management of hydronephrosis and reflux is valve ablation and observation with antibiotic prophylaxis.
• Bladder and renal function are often unstable in valve patients and usually change throughout life, requiring life long monitoring

In hypospadias, the urethral meatus opens on the ventral side of the penis proximal to the tip of the glans penis

• Sexual differentiation and urethral development begin in utero at approximately 8 weeks and are complete by 15 weeks.
• The urethra is formed by the fusion of the urethral folds along the ventral surface of the penis, which extends to the corona on the distal shaft.
• The glandular urethra is formed by canalization of an ectodermal cord that has grown through the glans to communicate with the fused urethral folds. Hypospadias results when fusion of the urethral folds is incomplete.

• • Glandular: Meatal opening in glans. It is the most common.
• • Coronal - opening at the coronal sulcus
• • Penile.
• • Peno-scrotal.
• • Perineal with split scrotum. This is associated with bilateral undescended testes.

• • Absence of urethra and corpus spongiosum distal to abnormal urethral orifice.
• • Abnormal (hooded) appearance of the penis, caused by deficient or absent ventral foreskin.
• • Bowing or bending of penis distal to abnormal urethral opening (chordee), with poorly developed prepuce over inferior aspect.
• • Urine soakage over the scrotum with dermatitis and infection.
• • Associated congenital anomalies are known to exist.

• Karyotype may help categorize hypospadias as syndromic when there are other nongenital anomalies.
• Children with penoscrotal and perineal hypospadias - Urethroscopy and cystoscopy are of value to determine whether internal male sexual organs are normally developed. Excretory urography is also indicated in these patients to detect additional congenital anomalies of the kidneys and ureters.
• For more distal types of hypospadias - routine excretory urography seems to be of little value in the more distal types of the disorder, because there appears to be no increased incidence of upper urinary tract anomalies.

For psychological reasons, hypospadias should be repaired before the patient reaches school age; in most cases, this can be done before age 2.

I. Cordee correction (Orthoplasty) - At 1.5 years. Orthoplasty is done by Heineke-Mikulicz technique.

II. Urethroplasty - At 5-7 years, done using prepucial skin (ideal) or scrotal skin if the patient has been circumcised.

• Some urologists prefer one stage repair with foreskin island flaps and incised urethral plate.
• Buccal mucosa grafts are more advantageous than others and should be considered the primary grafting technique when indicated.
• In hypospadias, circumcision is contraindicated as prepucial skin is required for future urethroplasty.

• Hypospadias repair after chordee release 
• 1. Glanular - Double Y Glanulomeatoplasty (DYG),  Meatal advancement glandular Plasty Incorporated  (MAGPI)
• 2. Distal Penile - Tubularised incised plate (TIP), Slit like Adjusted Mathieu (SLAM)
• 3. Proximal - Lateral Based Flap (LAB)
• 4. Perineal - Two staged repair

Perineal urethrostomy is done for  diversion and should be there until reconstruction of urethra takes up well (Denis-Browne  procedure).

Exstrophy of the bladder is a rare congenital anomaly with complete ventral defect of the urogenital sinus and the overlying skeletal system.

• The lower central abdomen is occupied by the inner surface of the posterior wall of the bladder, whose mucosal edges are fused with the skin. Urine spurts onto the abdominal wall from the ureteral orifices.
• The rami of the pubic bones are widely separated. The pelvic ring thus lacks rigidity, the femurs are rotated externally, and the child “waddles like a duck.”
• The rectus muscles are widely separated from each other caudally. A hernia, made up of the exstrophic bladder and surrounding skin, is present.
• Epispadias is almost always associated. Undescended testicles may be seen, and the anus and vagina located anteriorly.
• Urinary tract infection and hydronephrosis are common.

Prenatal diagnosis is difficult. Obvious anomaly of exposed bladder mucosa makes diagnosis easy at birth.  X-ray studies will reveal separation of pubic bones.

Steps for satisfactory repair of bladder exstrophy

• (1) bladder closure with sacral osteotomy in order to close the pelvic ring at the pubic symphysis, plus lengthening of the penis;
• (2) antiureteral reflux procedure and bladder neck reconstruction; and
• (3) repair of the epispadiac penis. Most common procedure is Cantwell Ransley Procedure. 

Treatment needs to be initiated prior to the fibrosis of the bladder mucosa in order to repair this anomaly completely. When the bladder is small, fibrotic, and inelastic, functional closure becomes inadvisable, and urinary diversion with cystectomy is the treatment of choice.

Common complications after surgical repair include incontinence, infertility, vesicoureteral reflux, and urinary tract infection. Complete primary closure appears to be the best choice for improved continence. Adenocarcinoma of the bladder and rectum are noted in higher frequency.