Surgery II - Block 7.4 Pediatrics Urology PDF

7.4 UROLOGY: Pediatrics Urology Dr. ALEJANDRO RiverA – [01/28/2025] | [1:30pm – 3:30pm] Bulcase, DELFIN, LUNASPE, PADILLA, RODRIGUEZ AND SANTACERA | ASSESSOR: PADILLA OUTLINE Emb...

7.4 UROLOGY: Pediatrics Urology Dr. ALEJANDRO RiverA – [01/28/2025] | [1:30pm – 3:30pm] Bulcase, DELFIN, LUNASPE, PADILLA, RODRIGUEZ AND SANTACERA | ASSESSOR: PADILLA OUTLINE Embryology of Nephric System 1. Embryology of Nephric Systems a. Pronephros Pronephros b. Mesonephros  Earliest nephric stage in humans c. Metanephros  Vestigial structure d. Embryology of the Gut  Develops at the 3rd week 2. Development of the Reproductive System  Disappears by the 4th-5th week of embryonic a. Important events life b. Derivatives of Ductal System c. Anomalies of the Nephric System Mesonephros d. Anomalies of position  Also a transient excretory organ i. Horsehoe kidney ii. Duplicated ureter  Mesonephric tubules develop iii. Ureterocele o cuplike outgrowths (bowman’s) iv. Congenital Uteropelvic Junction o knot of capillaries (glomerulus) (UPJ) Stenosis  Gives rise to the ureteral bud 3. Vesicoureteral Reflux (VUR)  Mesonephric duct (Wolffian) gives rise to the a. International Reflux Classification male reproductive system. i. Secondary VUR  The paramesonephric duct (Mullerian) gives ii. Spectrum of Chronic VUR rise to the female reproductive ductal system b. Posterior Uretheral Valves (PUV) c. Potters’s Syndrome Metanephros 4. Embryology of Vesicoureteral Unit  Gives rise to the mature Kidney a. Urogenital Sinus  Requires the stimulation from the ureteral bud i. Important events  Migration is an essential step in development. b. Embryology of Vesicouretheral Unit c. Anomalies of Vesicouretheral Unit d. Persistent Cloaca e. Urachal Abnormalities i. Urachus f. Epispadias g. Hypospadias 5. Embryogenesis of Testicular Descent a. Scrotal Problems b. Cryptorchidism i. Absent testis ii. Undescended testis c. Inguinal Hernia d. Hydrocele e. Testicular Torsion i. Manual detorsion f. Epididymo-Orchitis g. Differential Diagnosis of Abdominal Mass in a Child h. Wilm’s tumor i. Neuroblastoma j. Pediatric Urologic Oncology Notes: Embryology of Gut  Paramesonephric Important Sequences o Upper vagina o Uterus  Union of a single ureteral bud and the o Fallopian tubes metanephric blastema  Remnant in males  Migration and rotation of the metanephric o Prostatic utricle blastema until it matures into the kidney Anomalies of the Nephric System  Renal Agenesis Development of the Reproductive System  Ectopic Kidney Important Events  Malrotated kidney  Testis secretes a Mullerian inhibiting substance.  Horseshoe kidney  MIS causes regression of the mullerian ducts.  Bifid Kidney  The Testosterone from the Leydig cells causes  Duplicated Ureter development of the WOLLFIAN DUCT  Supernumerary kidneys SYSTEM Anomalies of Position Derivatives of the Ductal System  Mesonephric o Efferent ductules of the testes o Vas deferens o Epididymis o Remnant of females  Gartner’s duct  Paraophoron  Epoopohoron 2 Ref: lecture slides, shs trans, audio recording Notes: Horseshoe kidney  Outcome: o Hydronephrosis of the upper pole I o Reflux of lower pole  Treatment: o Common sheath reimplantation o Ureteropyelostomy o Heminephrectomy Ureterocele  Cause o Delayed canalization of the ureteral bud.  Types: o Intravesical o Ectopic  usually associated with duplex  Consequences: Duplicated Ureter o Recurrent infection  Incomplete duplex o Bladder outlet obstruction o usually of no clinical consequence o Incontinence o Incidental finding  Diagnosis o Warrants no intervention o unless mag obstruct siya,advice lang ang patient for a better lifestyle to prevent stones  Double Collecting System o Weigert- Meyer Law  Double Ureters always cross  Upper pole drains into the lower lower and more medial part of the ureteral meatus.  Lower pole drains into the upper and more lateral part of the meatus.  Possible consequences of a duplex collecting system: o The upper moiety may drain into an  Treatment ectopic ureter and is commonly  Endoscopic incision of ureterocele obstructed o This however may convert an obstructed o The lower moiety is usually refluxing renal segment to a refluxing unit. 3 Ref: lecture slides, shs trans, audio recording Notes:  It can become refluxing, then you have to  There should be low intravesical monitor the reflux, and how severe it is. pressure. Sometimes they reimplant, you cut the ureter,  UV junctionmust occlude the then implant it somewhere else. distal ureter during bladder filling and contraction. Congenital UteropelvicJunction (UPJ) Stenosis  Sa anatomy ta, ang anti-retrograde mechanism. So dapat antegrade na ang flow sang urine, wala na garetrograde  “Flap-Valve” effect of the Uterovesical Lunction (UVJ) o Characteristics of the UVJ:  Oblique course  muscular attachments  Adequate submucosal length  During bladder filling and Contraction: o The muscle layer occludes the intramural segment of the ureter thereby preventing “retrograde” flow of urine to the upper collecting system. Vesico-Ureteral Reflux (VUR)  Most common cause of obstructive  “Retrograde” flow of urine into the upper hydronephrosis in children. collecting system.  Pathology:  Cause of recurrent UTI in children o True stenosis is rarely found o clue mo is failure to thrive, they dont o Aperistatltic segment always present with UTI. o Aberrant vessel  Types: o It is functionally obstructed. Waay siya o Primary gafunction kag waay siya ga peristalsis. o Secondary  Treatment  Primary VUR o Dismembered pyeloplasty o Congenital o Resection of pathologic segment o Deficiency of longitudinal muscles of o Reduction of the redundant pelvis intravesical ureter o Transposition of the UPJ anterior to the  Short intramural segment abberant vessel.  Normal ratio 5:1 - (if lip-ot ang  Physiology of Antegrade Urine antireflux mechanism,then o Anatomic considerations pigado)  Unilateral peristalsis is necessary to propel urine like a bolus. 4 Ref: lecture slides, shs trans, audio recording Notes:  Secondary VUR o Caused by bladder obstruction  Anatomical  Functional o High intravesical pressure o Posterior Urethral valves  Consequences o Renal insufficiency o Renal failure o Scarring  Treatment o Medical Therapy  Control Infection  Lower intravesical pressure  Prophylactic antibiotics  Indications - Grade I-IV o Surgical  Ureteral Reimplantation  Deflux (Dextranomer with sodium hyaluronan) Secondary VUR  Endoscopic tissue  Pathogenesis augmentation o Decreased bladder wall compliance  Induce fibroblast and o Detrusor decompensation collagen deposition o Incomplete bladder emptying  Disappears within one o Attenuation of the trigone week but endogenous o Damage of the UVJ complex tissue augmentation  Can be: remains. o Anatomical  Indication - Grade V or o Functional Parenchymal scarring Anatomical Functional International Reflux Classification Posterior Urethral Neurogenic Bladder Valves Myelodysplasia Spina bifida Tethered cord syndrome Spinal dysraphism Ectopic  Non-neurogenic Bladder Ureterocoele  dysfunction  Bladder instability  Uninhibited bladder  contractions Meteal Stenosis  Bladder-sphincter dyssynergia 5 Ref: lecture slides, shs trans, audio recording Notes: Spectrum of Chronic VUR  Cystoscopy o Valves are visualized  Hydronephrosis  Diagnostic  Recurrent UTI o Ablation with electric cautery  Parenchymal Scarring  Therapeutic  Chronic Hypertension  Chronic Renal failure Posterior Uretheral Valves (PUV)  Clinical Presentation o Distended bladder o Hydronephrosis o Neonatal Ascites o Respiratory distress (pulmonary hypoplasia) o Oligohydramnios Potter’s Syndrome Diagnosis  Dysmorphicfacial features  Fetal growth retardation  Voiding Cystourethrogram (VCUG)  Limb deformities  GOLD STANDARD o Irregular bladder outline  Pulmonary hypoplasia o Dilated Prostatic urethra o VUR  Ultrasonography o Thickened bladder wall o Bilateral hydronephrosis o Bilateral dilated ureters  P- ulmonary hypoplasia  O- ligohydramnios  T- wisted (wringkly) skin  T- wisted (Potter) face  E- xtremities defects  R- enal agenesis 6 Ref: lecture slides, shs trans, audio recording Notes:  Mullerian ducts fuse proximal to the tubercle Embryology of Vesicouretheral Unit and form the upper vagina, uterus, and fallopian tube.  The pelvic part forms the vaginal vestibule and lower vagina. Embryology of VUU  CLOACA o Expanded portion of the caudal end of the hindgut.  Two divisions: Anomalies of VUU o Ventral - Urogenital sinus  Persistent Cloaca o Dorsal – Rectum  Urachal abnormalities Urogenital Sinus o Urachal Fistula o Urachal Cyst Important sequences o Urachal Diverticulum  Separation of the cloaca into two compartments  Epispadias by the migration of the urorectal fold.  Bladder Exstrophy  In males,  Hypospadias o Epithelial outgrowths develop into the Persistent Cloaca prostate gland. o Ventral part widens to form the bladder. o Pelvis part narrows and becomes tubular. o Urethral folds unite to form the urethra. o Mesonephric duct forms the vas deferens, epididymis and seminal vesicles.  In females, o Ventral part widens to form the bladder. o The Mullerian tubercle is important landmark. 7 Ref: lecture slides, shs trans, audio recording Notes: Urachal Abnormalities  Components o Dorsal hood o Ventral Penile curvature o Abnormal ventral location of the urethral meatus. Classification Urachus  Fate of Urachus o Remnant of the allantoic duct extending from the anterior bladder wall to the urachus. o The tract obliterates by the end of the third trimester.  Presentation o Neonate with wet umbilicus. o Worsens while crying  Other symptoms: o Fever o Pain o Frequency, Urgency and Dysuria Epispadias  Urethral opening is located dorsally  Results from caudal development of the corpora cavernosa Hypospadias  Pathogenesis o Incomplete closure of the urethral folds o associated with chordee, shortening of  Consequences the penis since short man ang urethra o Voiding in squat position  Etiology o No projectile ejaculation o Abnormal androgen production by the  Goals of treatment hypospadias fetal testis o Correct the ventral curvature o Limited androgen sensitivity  Release of chordee o Premature cessation of androgen o Transpose urethra to the most distal stimulation portion o Premature involution of the Leydig cells o Use a vascular tissue flap for coverage. 8 Ref: lecture slides, shs trans, audio recording Notes: Absent Testis Embryogenesis of Testicular Descent  Factors affecting descent of the gonad o Gubernaculum (“helm” or “rudder”)  one that pulls the testicles down o Intact androgen stimulation  to encourage migration o Increased intraabdominal pressure  a factor that affects the descent Scrotal Problems  Absent Testis (Undescended)  Inguinal Hernia  Hydrocele Undescended Testis  Testicular Torsion  Causes:  Epididymo-orchitis o Primary Gonadotropin Deficiency Cryptorchidism o Androgen Deficiency o Androgen Insensitivity Syndromes  Terms: o Abnormalities of the Gubernaculum o “Hidden testis”  Diagnosis: o May be used interchangeably with o Laparoscopy undescended testis  Non-palpable o Abnormally placed testis.  Blind ending vas  Pathogenesis: o CT Scan o Absence of gubernaculum  Intraabdominal o Androgen deficiency o Inguinal/Abdominal exploration  Gonadotropin deficiency  Leydig cell dysplasia Inguinal Hernia  Androgen receptor insensitivity syndromes  Presentation  Isolated Cryptorchidism o Reducible, non-tender scrotal mass o affecting upward of 3% full-term male o (-) Transillumination newborns  Treatment o Unilateral > Bilateral o Ligation of the hernial sac Cryptorchidism Case 1: Name: BBM CC: Absent Testis “Walang bayag” DOI: Jan 01, 2025 HPI: Always absent during Debate 9 Ref: lecture slides, shs trans, audio recording Notes: Hydrocele Manual Detorsion  Presentation:  Should be attempted only when a diagnosis is o Painless enlargement of the scrotum confirmed. o Non-tender  Orchiopexy should be done after a successful o (+) Transillumination manual detorsion  Treatment: o To do your detorsion, kung early pagid o Ligation of the patent processus kag sure ka nga it’s torsion, so just “open vaginalis a book” motion while ara ka sa tiilan o sang patient. o If left, of course sa left lateral mo sya iturn, if right to the right lateral naman. Testicular Torsion  Presentation o Sudden onset of painful scrotum at rest o Inflamed Scrotum Epididymo-Orchitis  Treatment:  Differentiating Torsion vs Orchitis o Manual Detorsion o Immediate exploration, orchiopexy o If non-viable, orchiectomy  Prehn’s Sign is positive for both  Doppler scan is very important when in doubt.  Scintigraphy would take too long, but results are helpful.  Isa pa ka clue, ang torsion ga balabag kg taas. Often patients come in, sobra na ka edematous, hindi kna ka check properly. 10 Ref: lecture slides, shs trans, audio recording Notes: Differential Diagnosis of Abdominal Mass in Child  Hepatomegaly Malignant Tumors  Hemihypertrophy  Wilm’s Tumor Lacking growth  Neuroblastoma – (important: most common)  WAGR  Rhabdomyosarcoma  Wilms’  Hepatoblastoma  Aniridia  Lymphoma  Genital anomalies  Renal Cell Carcinoma  Retardation Benign Abdominal Masses  Multicystic Dysplastic Kidney  Hydronephrosis  Polycystic Kidneys  Renal Abscess  Congenital Mesoblastic Nephroma  Mesenteric Cyst  Choledochal Cyst  Intestinal Duplication  Splenomegaly Wilms Tumor  Arises from the nephrogenic rest of the metanephric blastema. Treatment  Other names  Mainstay of Treatment: Radical Nephrectomy o Renal embryoma o Dapat iremove gd. After that, pwde o Nephroblastoma radiation, pwde chemo. Kung dako gd,  Most common primary malignant renal tumor of pwde neo-adjuvant. childhood  3-drug chemotherapy o Median age 3.5 years (diagnosed