Common Disorders of Pediatric Urology and Surgical Approach DR. H. Murat MUTUŞ

Pediatric Urology 1% of all pregnancies, there is a congenital anomaly 50% Central nervous system (Brain and spinal chord) 20-30% Genitourinary system (Kidneys and urinary tract) 15% Gastrointestinal system (Stomach and bowels) 8% Cardiovascular system (Heart and vessels)

Urinary Anomalies Urinary system anomalies present in 5 main forms: Visible anomalies Urinary tract infections (UTI) Voiding problems Mass Kidney failure

Symptoms Prenatal Postnatal Hydronephrosis Oligohydramniosis Recurrent urinary and kidney infections Abdominal pain Kidney stones Hypertension Findings of mass Kidney failure Most frequent Most frequent

Visible anomalies Bladder extrophy Hypospadias Epispadias Disorders of Sexual Development (DSD) Rare anomalies

Hypospadias One of the most common anomalies of boys (1-2%) Urethral meatus opens to ventral surface of penis instead of the tip. Underlying pathology is development failure of corpus spongiosum. According to the position of meatal opening, classiefied as distal, mid, or proximal hypospadias. Ventral curvature of penis, called as «chordee deformity» often accompanies hypospadias. There is no ventral preputial skin tissue. Treatment: The time of operation is around 1 year of age. The aim is to create a tube carrying the meatus to the tip, and correcting the ventral chordee deformity. This may be achieved in one single stage or as a multistaged operation. The role of family physician: 3 points should be noted: Urine caliber. The meatus sometimes can be very stenotic By the end of 1st month, a urinary system USG may reveal other anomalies As prepuce may be used as graft in surgery, these babies should not be circumcised under any circumstances.

Epispadias Urethral meatus opens to penile dorsum. It may be isolated, or seen as a component in bladder extrophy. When bladder neck is not involved, these cases are continent for urination. The role of family physician: The family should be assured that this is a correctable anomaly, and surgery would be performed at the age of 6 months. Upper urinary tract may be imaged for the presence of other anomalies

Disorders of Sexual Development (DSD)

DSD What is Sex? Factors determining sex: Genetic structure Sex hormones and their effects Genital organ structure Environmental factors

SRY gene in Y Chromosome DSD Testosterone & MIS testis SRY gene in Y Chromosome

Ambiguous genitalia of the newborn and child DSD Ambiguous genitalia of the newborn and child Cliteromegaly hyperpigmentation Palpable Inguinal/labial mass Posterior labial fusion

Conditions of Ambiguous genitalia DSD Conditions of Ambiguous genitalia 1. Female Pseudohermaphroditism (Female DSD) 2. Male Pseudohermaphroditism (Male DSD) 3. True hermaphroditism 4. Gonadal dysgenesis

Female DSD Most common cause of female DSD is Congenital Adrenal Hyperplasia (CAH) About 90-95 % of all CAH cases, the cause is 21-Hydroxylase Deficiency 25% of 21-OH Deficiency are simple virilizing type, 75% are salt loosing type. Salt loosing type may be fatal if not properly treated.

Female DSD Feminizing Genitoplasty Reduction clitoroplasty Posterior flap vaginoplasty

Imaging in Pediatric Urology USG SCINTIGRAPHY DMSA DTPA and MAG3 VOIDING CYSTOURETHROGRAM (VCUG) MR RENOGRAM (UROGRAPHY) IVP

HYDRONEPHROSIS Hydroneprosis is the most common genitourinary system anomaly seen in kidneys Hydronephrosis may coexist with hydroureter (Hydroureteronephrosis) May be bilateral according to underlying pathologic condition

Renal Pelvic AP Diameter Measured by USG When renal pelvic AP diameter is > 10 mm, the risk of renal damage is high Pelvic AP diameter Rate of operation; •> 20 mm % 94 •10-15 mm % 50 •< 10 mm % 3

Causes of Hydronephrosis Obstructive UP Junction Obstruction Ureteral Valves Duplex System UV Junction Obstruction Ectopic Ureter Ureterocele PUV Urolithiasis Non obstructive Cystic diseases VUR Neurogenic bladder Prune Belly syndrome

Obstructive Causes

Uretero Pelvic Junction Obstruction (UPJO) 40-64% of newborn hydronephrosis cases are caused by UPJO 1/ 1250 live births Majority of obstructive uropathies are seen in males. M/F ratio is 3-4/1 More common on left side and in males. 15-25 % are bilateral UPJO are generally sporadical, but familial cases are also reported

UP junction obstruction UPJO Most common site of obstruction in urinary system. Most common prenatal urinary anomaly UP junction obstruction Intrinsic causes fibrotic stenosis Intraureteric polyp Ureteral valves Extrinsic causes aberrant vessels adhesions ureter outlet anomalies Adynamic segment

UPJO The role of family physician: In prenatally diagnosed cases, postnatal monthly USG should be obtained to evaluate pelvic AP diameter to make sure that there is note sudden increase in severity of obstruction. Renal scintigraphies, static one (DMSA) to evaluate the renal function and scarring, dynamic ones (DTPA or MAG-3) to evaluate pelvic clearance in every 3-6 months periods. The decision of surgery is given by surgeons.

(Anderson Hynes Dismembered) UPJO PYELOPLASTY (Anderson Hynes Dismembered) Decision for surgery is given individually !!!

Ureteral Duplication Most common anomaly of ureter Seen 1 in 125-150 of population More common in females Urinary infection is generally the first manifestation In complete duplications there may be scar formation in the relevant kidney unit

Ureteral Duplication In complete duplications, mainly vesicoureteral reflux (VUR), and other anomalies like ureteral ectopia or ureterocele may commonly accompany

Ureteral Duplication Weigert – Meyer Law ????

Ectopic Ureter Normalde üreteral orifisler mesane trigonu üzerinde yer almalıdır. Üreter orifisinin mesane boynuna veya trigon dışında bir noktaya açılmış olmasına ektopik üreter denir. Üreteral ektopi 2000 kişide 1 sıklığında görülür. Kızlarda ektopik üreter %80 duplikasyonla birlikteyken, erkeklerde daha çok tekli üreterin ektopisi görülür. Ektopilerin %20’si iki taraflıdır.

Ureterocele Üreterin intravezikal parçasının distal kısmının kistik dilatasyonuna üreterosel denir. üreterosel

Ureterocele Üreterosel sıklıkla dupleks sistemlerde üst kutbu drene eden üreterin (%80) veya ektopik bir üreterin ucunda (%60) yer alır. 5000-12.000’de 1’dir. Kızlarda erkeklerden 3-7 kez daha sık görülür. Sol tarafta sağa göre biraz daha sık, olguların % 10-15’inde de iki taraflıdır.

Ureterocele Üreterosel sıklıkla dupleks sistemlerde üst kutbu drene eden üreterin (%80) veya ektopik bir üreterin ucunda (%60) yer alır. 5000-12.000’de 1’dir. Kızlarda erkeklerden 3-7 kez daha sık görülür. Sol tarafta sağa göre biraz daha sık, olguların % 10-15’inde de iki taraflıdır.

UV DARLIK KONJENİTAL MEGAÜRETER Üreter çapının 7 mm den fazla olmasıdır. 3 tipi vardır Reflüsüz obstruktif Reflülü obst yok Reflüsüz obst yok Üreter alt uçundaki kasların anormalliği ile oluşur...

ÜRETEROPLASTİ

POSTERİOR URETRAL VALV Alt üriner sistemin en sık obst. nedenidir.. Erkeklerde İdrar yapma şikayetleri Posterior üretra veru montanum

SİSTOSKOPİ

VUR VUR idrarın üst üriner traktusa geri kaçmasıdır.. İYE ile karşılıklı bir ilişki söz konusudur. Gelişmekte olan ülkelerdeki son evre böbrek yetmezliği nedenleri arasında en sıktır. Sağlıklı çocuklarda görülme insidansı % 0.5 VURlu çocukların kardeşlerinde ortaya çıkma oranı 10-20 kat fazladır.

Antireflü mekanizmasının bozukluğu Primer VUR Antireflü mekanizmasının bozukluğu Sekonder reflü İYE Nörojen mesane PUV Travmatık üretra tıkanıklıkları

İYE Rezidu idrar İntra renal reflü Kronik pyelonefrit DMSA SKAR

Tedavi 1- medikal 2- cerrahi sting açık cerrahi (üreteroneositostomi)