PEDI GU REVIEW-perinatal emergencies i


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PEDI GU REVIEW-perinatal emergencies i

  1. 1. PERINATAL EMERGENCIES Pediatric GU Review UCSD Pediatric Urology George Chiang MD Sara Marietti MD Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007 (not for reproduction, distribution, or sale without consent)
  2. 2. Antenatal Period <ul><li>Intervention may include: medical treatment, termination, early induction, surgical intervention </li></ul><ul><li>Ethical issues </li></ul><ul><li>Initial screening sonograms in obstetric office </li></ul>
  3. 3. Urinary tract obstruction with oligohydramnios <ul><li>1 in 30,000 pregancies may be a candidate for antenatal intervention </li></ul><ul><li>Induction of early delivery after lung maturity is established </li></ul><ul><li>Treat the mother with corticosteroids to accelerate lung maturity </li></ul>
  4. 4. Urinary tract obstruction with oligohydramnios <ul><li>Urethral atresia </li></ul><ul><ul><li>Most common cause of early/severe hydroureteronephrosis </li></ul></ul><ul><li>Posterior urethral valves-early vs. late </li></ul><ul><li>Ureterocele obstructing bladder neck more comon in females </li></ul>
  5. 5. Urinary tract obstruction with oligohydramnios <ul><li>Only patients with oligohydramnios are intervention candidates </li></ul><ul><li>Other severe abnormalities should be ruled out </li></ul><ul><li>Evidence of recoverable renal function </li></ul><ul><ul><li>Serial urine samples </li></ul></ul><ul><li>Infusion of fluid into amniotic space to enhance sonographic analysis </li></ul><ul><li>Chromosomal analysis </li></ul><ul><li>Increasing dilatation of renal pelvis corresponds with greater likelihood of obstruction </li></ul>
  6. 6. Urinary tract obstruction with oligohydramnios <ul><li>Complications with antenatal intervention </li></ul><ul><ul><li>Amniocentesis  1% incidence of spontaneous abortion </li></ul></ul><ul><ul><ul><li>Fetal hemorrhage, cord laceration, direct fetal injury, uterine injury, chorioamnionitis </li></ul></ul></ul><ul><ul><li>Chorionic villus sampling  fetal loss 3.5% </li></ul></ul><ul><ul><li>Surgical intervention for fetal urinary tract, myelo, fetal diaphragmatic hernia, fetal cystic adenomatoid malformation, and fetal sacrococcygeal teratoma </li></ul></ul><ul><ul><ul><li>Uterine contractions  premature delivery </li></ul></ul></ul>
  7. 7. Antenatal Period <ul><li>Classic bladder exstrophy </li></ul><ul><ul><li>No visualization of the bladder, lower abdominal bulge, small penis and anteriorly placed scrotum, low-set umbilicus, and abnormal widening of the iliac crests </li></ul></ul><ul><li>Omphaloceles, gastroschisis </li></ul><ul><li>Use of MRI </li></ul><ul><li>Counseling for parents </li></ul>
  8. 8. Antenatal Period <ul><li>Neural tube defects </li></ul><ul><ul><li>Routine screening of materal serum alpha-fetoprotein and targeted sonography </li></ul></ul><ul><ul><li>Decreasing incidence by use of folic acid and increased termination </li></ul></ul><ul><ul><li>Neurologic abnormalities may be related to prolonged neural tube exposure to amniotic fluid </li></ul></ul><ul><ul><ul><li>VP shunting less likely with intrauterine repair </li></ul></ul></ul><ul><ul><ul><li>Despite surgery, care should be the same </li></ul></ul></ul>
  9. 9. Antenatal Period <ul><li>Solid masses </li></ul><ul><ul><li>Adrenal hemorrhage </li></ul></ul><ul><ul><li>Neuroblastoma </li></ul></ul><ul><ul><li>Congenital mesoblastic nephroma </li></ul></ul><ul><ul><li>Wilms’ tumor </li></ul></ul><ul><ul><li>Pulmonary sequestration </li></ul></ul><ul><ul><li>Teratoma </li></ul></ul><ul><ul><li>Sacrococcygeal teratoma </li></ul></ul><ul><ul><li>Hepatic lesions </li></ul></ul><ul><ul><li>Rhabdomyosarcoma </li></ul></ul><ul><li>These should be evaluated thoroughly after delivery </li></ul>
  10. 10. Antenatal Period <ul><li>Intersex disorders </li></ul><ul><ul><li>Amniocentesis, chorionic villus sampling, and sonography can allow early ID of many intersex disorders </li></ul></ul><ul><ul><li>Provide prenatal counseling </li></ul></ul><ul><ul><li>Treat fetus in hopes of preventing androgen effects </li></ul></ul><ul><ul><ul><li>Most effective with family history </li></ul></ul></ul><ul><ul><ul><ul><li>Genetic sampling with history </li></ul></ul></ul></ul><ul><ul><li>If genetic sex doesn’t correspond to ultrasound genotype, then consider intersex </li></ul></ul><ul><ul><li>Prenatal treatment needs to star before 6 weeks whereas chorionic villus sampling for fetal DNA can’t be done until 10-12 weeks </li></ul></ul><ul><ul><li>Risk of having an infant with CAH for a mother with classic 21-hydroxylase deficiency is 1 in 8 </li></ul></ul>
  11. 11. Intersex 46 XX Female pseudohermaphrodite Male pseudohermaphrodite True hermaphrodite Gonadal dysgenesis 46 XY Male pseudohermaphrodite True hermaphrodite 46 XX/XY True hermaphrodite 45, X, 45,X/46,XX, 45,X/46,XY and others Gonadal dysgenesis Mixed gonadal dysgenesis Male pseudohermaphrodite True hermaphrodite
  12. 12. Congenital anomalies noted at birth <ul><li>Anorectal malformations </li></ul><ul><ul><li>High (above levator); low (inferior to levator) </li></ul></ul><ul><ul><li>Correction via a posterior sagittal approach </li></ul></ul><ul><ul><li>Urological abnormalities in up to 20% with low lesions and 60% with high lesions </li></ul></ul><ul><ul><ul><li>VUR, renal agenesis, neuropathic bladder (up to 50%), cryptorchidism </li></ul></ul></ul><ul><ul><ul><li>Should be studied with UDS with high lesions and/or spinal abnormalities (tethered cord) </li></ul></ul></ul>
  13. 13. Congenital anomalies noted at birth <ul><li>Exstrophy </li></ul><ul><ul><li>Typically male </li></ul></ul><ul><ul><li>Exstrophic bladder, abdominal wall defect, epispadias, pelvic diastasis, VUR after bladder closure, bilateral inguinal hernias </li></ul></ul><ul><ul><li>Suture instead of umbilical clamp </li></ul></ul><ul><ul><li>Cellophane non-adherent dressing </li></ul></ul><ul><ul><li>Staged versus 1 stage procedures </li></ul></ul>
  14. 14. Congenital anomalies noted at birth <ul><li>Prune belly syndrome </li></ul><ul><ul><li>Deficient abdominal wall musculature, cryptorchism, urinary tract dilation </li></ul></ul><ul><ul><li>Presumptive diagnosis can be made at 12 weeks </li></ul></ul><ul><ul><li>Spectrum of disease is wide; most severe is urethral obstruction </li></ul></ul><ul><ul><li>Postnatal eval includes VCUG and renal scan </li></ul></ul><ul><ul><li>Antibacterial prophylaxis and vesicostomy or upper tract diversion </li></ul></ul><ul><ul><li>Treatment of UDT similar to ither patients with intra-abdominal testes </li></ul></ul>
  15. 15. Congenital anomalies noted at birth <ul><li>Myelodysplasia </li></ul><ul><ul><li>Level of lesion does not correlate with neurological defect because some nerve roots may be intact </li></ul></ul><ul><ul><li>Arnold-Chiari malformation can affect the brainstem and pontine micturition center </li></ul></ul><ul><ul><li>Changing UDS over age </li></ul></ul><ul><ul><li>In general, patients with high thoracic lesions have intact sacral reflex; lesions at S1 or below can have normal bladder function </li></ul></ul><ul><li>Lipomeningocele </li></ul><ul><ul><li>Intradural lipoma </li></ul></ul><ul><ul><li>Few outward physical abnormalities and may require UDS </li></ul></ul><ul><ul><ul><li>Detrusor hyperreflexia and arreflexia </li></ul></ul></ul><ul><li>Sacral agenesis </li></ul><ul><ul><li>Present at older age with failure to be continent </li></ul></ul><ul><ul><li>Stable neurologic lesion that does not progress with growth </li></ul></ul><ul><ul><li>UDS is variable </li></ul></ul>
  16. 16. Scrotal Masses <ul><li>Eval includes: onset/severity of pain; orientation of testis </li></ul><ul><li>Differential </li></ul>Hydrocele Incarcerated hernia Torsion of testis Appendage torsion Testis tumor Epididymitis Epididymal cyst Epididymal tumor Paratesticular tumor Varicocele HSP Idiopathic edema Hemangioma Funiculitis Patent processus
  17. 17. Scrotal Masses <ul><li>Neonatal scrotal masses </li></ul><ul><ul><li>Hydrocele </li></ul></ul><ul><ul><li>Incarcerated hernia </li></ul></ul><ul><ul><li>Torsion </li></ul></ul><ul><ul><li>Patent processus vaginalis (mecomium mass, intra-abd hemorrhage, tumor seeding) </li></ul></ul><ul><ul><li>Meconium (rupture of bowel during development and peritonititis) </li></ul></ul><ul><ul><li>Testis tumors (yolk sac, gonadal stromal, granulosa cell) </li></ul></ul><ul><ul><li>Trauma </li></ul></ul><ul><li>Postivite transillumination can help, plain abdominal film can diagnose bowel gas in the scrotum or calification suggesting meconium </li></ul><ul><li>Neonatal torsion: controversy over mechanism, risk of bilaterality, and timing for contralateral fixation </li></ul><ul><ul><li>Non-tender, discolored, scrotal mass </li></ul></ul><ul><ul><li>Most often unilateral but over 33 cases bilateral </li></ul></ul>
  18. 18. Scrotal Masses <ul><li>Pediatric scrotal masses </li></ul><ul><ul><li>Torsion has bimodal distribution in perinatal period and at or before puberty </li></ul></ul><ul><ul><ul><li>Cremasteric is generally absent, testis is tense to palpation and rides high in the scrotum </li></ul></ul></ul><ul><ul><ul><li>Usually intravaginal and secondary to bell-clapper deformity </li></ul></ul></ul><ul><ul><ul><li>Should be relieved within 4 hrs; by 24 hrs there is almost uniform atrophy </li></ul></ul></ul><ul><ul><ul><ul><li>Spermatogonia are more sensitive to ischemia than Sertoli and Leydig cells </li></ul></ul></ul></ul><ul><ul><ul><li>Contralateral testis should be fixed at time </li></ul></ul></ul><ul><ul><ul><li>16% of patients presenting to an ER with acute scrotum will have torsion </li></ul></ul></ul><ul><ul><ul><li>hCG may cause torsion in the UDT </li></ul></ul></ul><ul><ul><li>Epididymitis presents as scrotal swelling with gradually increasing pain </li></ul></ul><ul><ul><ul><li>Elevation of testes may improve pain (Prehn’s sign) </li></ul></ul></ul><ul><ul><ul><li>Etiologies include trauma, infection, reflux of sterile urine </li></ul></ul></ul><ul><ul><ul><li>Treatment with scrotal elevation, bed rest, and antibiotics if UA positive </li></ul></ul></ul><ul><ul><ul><li>In sexually active teenagers, chlamydia should be treated </li></ul></ul></ul><ul><ul><ul><li>With bacterial epidiymitis, RUS and VCUG </li></ul></ul></ul>
  19. 19. Scrotal Masses <ul><li>Pediatric Scrotal Tumors </li></ul><ul><ul><li>Benign lesions in prepubertal children can be removed by partial orchiectomy </li></ul></ul>Mature Teratoma Teratocarcinoma Yolk Sac Tumor Paratesticular rhabdomyosarcoma Gonadal stromal tumors Granulosa cell tumors Canvernous hemangioma Gondoblastoma Metastatic tumors
  20. 20. Intersex disorders <ul><li>CAH is the most common cause </li></ul><ul><ul><li>21-hydroxylase, 11B-hydroxylase, 20,22 desmolase, and 3B-hydroxysteroid dehydrogenase </li></ul></ul><ul><ul><li>21-hydroxylase deficiency can lead to lack of mineralocorticoid and glucocorticoid with elevated potassium and low sodium with dehydration and arrhythmias-->treat with replacement therapy </li></ul></ul><ul><ul><li>Virilization of the female infant </li></ul></ul><ul><ul><ul><li>Varying degrees </li></ul></ul></ul><ul><ul><ul><li>Gonads are symmetrical and typically non-palpable </li></ul></ul></ul><ul><ul><ul><li>Pelvic u/s identifies uterus and ovaries </li></ul></ul></ul><ul><ul><ul><li>Mullerian inhibiting substance is undetectable </li></ul></ul></ul><ul><ul><ul><li>46 XX </li></ul></ul></ul><ul><ul><ul><li>Serum studies include electrolytes, glucose, gonadotropins, dihydrotestosterone, testosterone, adrenal steroid levels </li></ul></ul></ul>
  21. 21. Intersex disorders <ul><li>Evaluation </li></ul><ul><ul><li>Physical exam should concentrate on palpating gonads </li></ul></ul>No gonads palpable One Gonad Palpable Both Gonads palpable <ul><ul><li>Female pseudohermaphrodite (FH), </li></ul></ul><ul><ul><li>gonadal dysgenesis (GD), </li></ul></ul><ul><ul><li>male pseudohermaphrodite (MPH), </li></ul></ul><ul><ul><li>true hermaphrodite (TH) </li></ul></ul>Mixed gonadal dysgenesis (MGD) TH MPH MPH TH
  22. 22. Intersex disorders <ul><li>Mesurement of:serum electrolytes, 17-hydroxyprogesterone, testosterone, and dihydrotestosterone and karyotype </li></ul><ul><li>Screening sonography and genitography </li></ul><ul><li>Checking 11-deoxycortisol levels can differentiate between 21-hydroxylase and 11-hydroxylase deficiencies </li></ul><ul><li>Always consider maternal virilizing hormone with normal evaluation </li></ul><ul><li>MPH </li></ul><ul><ul><li>Testicular failure, dysgenetic testes, steroid deficiency (male CAH), androgen insensitivity, 5 reductase def </li></ul></ul><ul><li>Pure gonadal dysgenesis-->both streak gonads </li></ul><ul><li>Mixed gonadal dysgenesis-->streak gonad and testis </li></ul><ul><li>True hermaphrodite-->both a testes and ovary or ovotestes </li></ul>
  23. 23. PUV and lower urinary tract <ul><li>Antenatal u/s findings include a distended/thick-walled bladder, bilateral hydronephrosis, and oligo (sometimes) </li></ul><ul><li>Outcomes of patients diagnosed antenatally may be worse than those diagnosed postnatally (greater severity of disease) </li></ul><ul><li>Catheter drainage and prophylactic antibiotics at birth </li></ul><ul><ul><li>Catheter drainage should be maintained until nadir creatinine; if <1 then valve ablation and then monitoring of renal fxn/bladder fxn/and reflux </li></ul></ul><ul><ul><li>If creatinine doesn’t decrease then vesicostomy </li></ul></ul><ul><ul><li>Little evidence exists that upper tract diversion really helps unless infection is present and it complicates reconstruction </li></ul></ul><ul><li>Older children may require anticholinergics and/or bladder augmentation for valve bladder with high storage pressures </li></ul>
  24. 24. Circumcision Injuries <ul><li>600,000-700,000 performed annually </li></ul><ul><li>Circumcision may be recommended with prenatal hydro and reflux </li></ul><ul><li>Older children-->persistent phimosis, paraphimosis, and recurrent balanitis </li></ul><ul><li>Success rates of 70-90% with betamethasone </li></ul>
  25. 25. Circumcision Injuries <ul><li>Early acute complications </li></ul><ul><ul><li>Bleeding </li></ul></ul><ul><ul><li>Infection </li></ul></ul><ul><ul><li>Laceration </li></ul></ul><ul><ul><li>Amputation </li></ul></ul><ul><ul><li>Urinary retention </li></ul></ul><ul><ul><li>Skin loss </li></ul></ul><ul><ul><li>Necrosis </li></ul></ul>
  26. 26. Circumcision Injuries <ul><li>Non-acute complications </li></ul>Skin excess Skin asymmetry Skin bridges Skin chordee Inclusion cysts Buried penis Phimosis Meatal stenosis UC fistula Hypospadias discovery Plastibell specific lymphedema
  27. 27. Circumcision injuries <ul><li>Prominent fat pad, penoscrotal webbing, prematurity are associated with complications </li></ul><ul><li>Mogen clamp can cause laceration/ amputation </li></ul><ul><li>Amputation can be fixed with up to 18 hr delay </li></ul><ul><li>Penile skin loss can be managed conservatively </li></ul><ul><li>Megameatus with intact prepuce should abort circ </li></ul><ul><li>Plastibell has higher incidence of delayed wound infection and retained plastic cuff </li></ul><ul><li>Ring block>dorsal block; EMLA is equivalent to dorsal </li></ul>
  28. 28. Abdominal masses in the infant <ul><li>Hydronephrotic lesions </li></ul><ul><ul><li>UPJ most common cause </li></ul></ul><ul><ul><li>Bilateral in up to 20-40% </li></ul></ul><ul><ul><li>Postnatal management with antibiotics and sonography; evaluate with VCUG and renogram </li></ul></ul><ul><ul><ul><li>Antibiotics can be stopped when reflux and obstruction excluded </li></ul></ul></ul><ul><ul><li>When obstructed kidney may have minimal function, eval of parenchyma may be difficult and require perc and scintigraphy </li></ul></ul><ul><ul><li>Treatment of UPJ depends on degree of obstruction and laterality </li></ul></ul>
  29. 29. Abdominal masses in the infant <ul><li>Renal cystic lesions </li></ul><ul><ul><li>Unilateral lesions </li></ul></ul><ul><ul><ul><li>MCDK second most common cause of abdominal mass in infants and may impede feeding/respiration </li></ul></ul></ul><ul><ul><ul><ul><li>High incidence of contralateral lesions (up to 25%) with UPJ and VUR (26% but usually low grade) </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Diagnosis with DMSA </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Ureteral atresia is associated with a higher incidence of contralateral abnormalities than is renal pelvic atresia </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Serial ultrasonography </li></ul></ul></ul></ul><ul><ul><li>Bilateral cystic lesions </li></ul></ul><ul><ul><ul><li>ARPKD with 1:20,000 </li></ul></ul></ul><ul><ul><ul><ul><li>Children present with massively enlarged kidneys and in severe cases with oligo and Potter’s facies </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Kidneys exhibit ectasia and cystic dilation of collecting tubules </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Asociated with pulmonary hypoplasia and hepatic fibrosis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Up to 30% die as neonates with 20-45% with ESRD by age 15 </li></ul></ul></ul></ul>
  30. 30. Abdominal masses in the infant <ul><li>Renal cystic lesions </li></ul><ul><ul><li>Bilateral cystic lesions </li></ul></ul><ul><ul><ul><li>ADPKD has incidence of 1 in 500 to 1 in 1000 </li></ul></ul></ul><ul><ul><ul><li>Chromosome 16 (PKD1) and 4 (PKD2) </li></ul></ul></ul><ul><ul><ul><li>May initially present unilaterally or with asymmetry </li></ul></ul></ul><ul><ul><ul><li>If identified in utero, 43% of cases die before 1st year and 67% develop HTN by age 3 </li></ul></ul></ul><ul><ul><ul><li>Later in life present with HTN, impaired renal function, proteinuria or hematuria </li></ul></ul></ul><ul><ul><ul><li>Associated with hepatic/pancreatic cysts and cerebral aneurysms </li></ul></ul></ul>
  31. 31. Abdominal masses in the infant <ul><li>Renal cystic lesions </li></ul><ul><ul><li>Midline cystic abdominal lesions </li></ul></ul><ul><ul><ul><li>Hydrometrocolpos-distension of the vagina with mucosa and blood; produced in response to maternal estrogens </li></ul></ul></ul><ul><ul><ul><ul><li>Vaginal obstruction from imperforate hymen, transverse vaginal septum, or vaginal atresia </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Presents as midline abdominal mass and/or interlabial mass; diagnosis with sonography; mass is anterior to the rectum and u/s will demonstrate lesion behind bladder </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Patients can have lower urinary tract obstruction and hydronephrosis which will resolve after incision and draimage </li></ul></ul></ul></ul><ul><ul><ul><li>Ovarian cysts are the third most common cause of an intra-abdominal mass in an infant; 30% of neonates </li></ul></ul></ul><ul><ul><ul><ul><li>Majority of cysts regress </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Large cysts may compromise bowel/respiratory function </li></ul></ul></ul></ul><ul><ul><ul><li>Megacystis-can be associated with PUV, megacystis-microcolon, prune belly </li></ul></ul></ul>
  32. 32. Abdominal masses in the infant <ul><li>Midline cystic abdominal lesions </li></ul><ul><ul><li>Urinary ascites 30% of all cases of ascites </li></ul></ul><ul><ul><ul><li>PUV, urethral stenosis, distal ureteral stenosis, obstructed/ hydronephrotic kidneys </li></ul></ul></ul><ul><ul><ul><li>Umbilical artery catheterization with bladder perforation </li></ul></ul></ul><ul><ul><ul><li>KUB may show ground-glass appearance with floating loops of bowl </li></ul></ul></ul><ul><ul><ul><li>Rim of contrast around kidney on IVP </li></ul></ul></ul><ul><ul><ul><li>Findings include hyponatremia, hyperkalemia and elevated creatinine </li></ul></ul></ul><ul><ul><ul><li>Treat with urethral catheter and antibiotics </li></ul></ul></ul><ul><ul><ul><li>Non-urological causes include erythroblastosis fetalis, cardiac or hepatic abnormalities, sepsis, and GI perforation </li></ul></ul></ul>
  33. 33. Abdominal masses in the infant <ul><li>Solid lesions </li></ul><ul><ul><li>Solid lesions detected antenatally are followed before delivery and then after birth imaged with CT or MRI </li></ul></ul><ul><ul><li>Sacrococcygeal teratoma 1 in 40,000 with majority females </li></ul></ul><ul><ul><ul><li>Evaluated by CT or MRI </li></ul></ul></ul><ul><ul><ul><li>May cause obstruction of urinary tract with hydronephrosis </li></ul></ul></ul><ul><ul><li>Neuroblastoma 1 in 7,000; 40% diagnosed in <1 year </li></ul></ul><ul><ul><ul><li>Many tumors detected prenatally regress </li></ul></ul></ul><ul><ul><ul><li>Neonatal screening unlikely to affect mortality </li></ul></ul></ul><ul><ul><ul><li>Firm, fixed lesion </li></ul></ul></ul><ul><ul><ul><li>Bulky metastatic disease with palpable liver/SQ nodules usually regresses </li></ul></ul></ul><ul><ul><ul><li>Diagnosis made be confused with pulmonary sequestration </li></ul></ul></ul><ul><ul><ul><li>Most cause elevation of urinary catecholamines </li></ul></ul></ul>
  34. 34. Abdominal masses in the infant <ul><li>Solid lesions </li></ul><ul><ul><li>Wilm’s tumor </li></ul></ul><ul><ul><ul><li>Infrequent in neonatal period although most common renal tumor </li></ul></ul></ul><ul><ul><ul><li>Associated with fetal hydrops and polyhydramnios </li></ul></ul></ul><ul><ul><ul><li>Children borth with aniridia, WAGR, Denys-Drash, or Beckwith-Wiedemann syndrome develop tumors at a youngerage and are more likely to have bilateral tumors; require surveillance u/s </li></ul></ul></ul><ul><ul><ul><li>Vincristine,doxorubicin,dactinomycin </li></ul></ul></ul><ul><ul><li>Congenital mesoblastic nephroma </li></ul></ul><ul><ul><ul><li>Most common solid renal mass in children <6 months of age (50%) </li></ul></ul></ul><ul><ul><ul><li>Associated with polyhydramnios </li></ul></ul></ul><ul><ul><ul><li>80% discovered in 1st month of life </li></ul></ul></ul><ul><ul><ul><li>Pathologicall: classic vs. cellular </li></ul></ul></ul><ul><ul><ul><ul><li>Classic doesn’t metastasize; cellular has potential for local recurrences and mets </li></ul></ul></ul></ul><ul><ul><ul><li>In-utero treatment is observation; postnatally nephrectomy and chemo if needed; hypercalcemis usually resolves after Nx </li></ul></ul></ul>
  35. 35. Abdominal masses in the infant <ul><li>Solid lesions </li></ul><ul><ul><li>Renal vein thrombosis </li></ul></ul><ul><ul><ul><li>Enlarged kidney, hematuria, proteinuria </li></ul></ul></ul><ul><ul><ul><li>Risk factors include dehydration, sepsis, birth asphyxia, maternal diabetes, protein C deficiency, twin-twin transfusion and presence of umbilical catheter </li></ul></ul></ul><ul><ul><ul><li>Diagnosed via u/s and initially lesion appears echogenic streaks within kidney </li></ul></ul></ul><ul><ul><ul><li>Treatment is supportive unless bilateral (thrombolysis) </li></ul></ul></ul><ul><ul><li>Renal artery thrombosis </li></ul></ul><ul><ul><ul><li>Doesn’t commonly present as a neonatal mass </li></ul></ul></ul><ul><ul><ul><li>Supportive measures and thombolytics </li></ul></ul></ul>
  36. 36. Abdominal masses in the infant <ul><li>Solid lesions </li></ul><ul><ul><li>Neonatal adrenal hemorrhage </li></ul></ul><ul><ul><ul><li>Traumatic or difficult labor, neonatal hypoxia, septicemia, or coagulopathy </li></ul></ul></ul><ul><ul><ul><li>1.9 per 1,000 births with R sided predominance and 10% bilaterality </li></ul></ul></ul><ul><ul><ul><li>Diagnosis by u/s and can be difficult to differentiate from adrenal neuroblastoma [CT and MRI] and negative urine catecholamines </li></ul></ul></ul><ul><ul><ul><li>No treatment; rarely transfusion or steroid replacement for adrenal insufficiency </li></ul></ul></ul><ul><ul><li>Cystic nephroma </li></ul></ul><ul><ul><ul><li>Rare and benign neoplasm </li></ul></ul></ul><ul><ul><ul><li>Partial nephrectomy </li></ul></ul></ul><ul><ul><li>Rhabdomyosarcoma </li></ul></ul><ul><ul><ul><li>Bladder, prostate, vagina, or uterus </li></ul></ul></ul><ul><ul><ul><li>Presentation is varied and treatment is multimodal </li></ul></ul></ul><ul><ul><li>Germ cell tumors are rare and can occur as ovarian mass or mass in UDT </li></ul></ul><ul><ul><ul><li>Surgery and platinum based therapy </li></ul></ul></ul>
  37. 37. Intraoperative consultations <ul><li>Often during hernia repair </li></ul><ul><ul><li>In males, finding of female organs=peristent Mullerian duct syndrome [failure of Sertoli cell production of MIS] </li></ul></ul><ul><ul><ul><li>Excision of female structures secondary to future malignancy risk </li></ul></ul></ul><ul><ul><li>In phenotypic females, finding of male structures suggests testicular feminization </li></ul></ul><ul><ul><ul><li>If removed early, then exogenous steroids; if left in situ until puberty, the testis must be removed because of risk of gonadoblastoma </li></ul></ul></ul><ul><li>During trauma, never explore the flank when an expanding hematoma is present until normal function of contralateral kidney confirmed </li></ul><ul><li>Beware of a pelvic mass that is actually an ectopic or pelvic kidney </li></ul>
  38. 38. Intraoperative consultations <ul><li>Organs drawn into the hernia sac </li></ul><ul><ul><li>Appendix </li></ul></ul><ul><ul><li>Bladder </li></ul></ul><ul><ul><li>Gonad </li></ul></ul><ul><ul><li>Meckel’s diverticulum </li></ul></ul><ul><ul><li>Omentum </li></ul></ul><ul><ul><li>Bowel </li></ul></ul><ul><ul><li>Pelvic kidney </li></ul></ul><ul><ul><li>Ureter </li></ul></ul><ul><ul><li>uterus </li></ul></ul>
  39. 39. Intraoperative consultations <ul><li>Aberrant rests </li></ul><ul><ul><li>Adrenal </li></ul></ul><ul><ul><li>Ectopic renal tissue </li></ul></ul><ul><ul><li>Accessory spleen </li></ul></ul><ul><li>Inflammatory lesions </li></ul><ul><ul><li>Abscess </li></ul></ul><ul><ul><li>Funiculitis </li></ul></ul><ul><ul><li>Granulomas </li></ul></ul><ul><ul><li>Sarcoidosis </li></ul></ul><ul><ul><li>Meconium granuloma </li></ul></ul><ul><ul><li>Parasitic disease </li></ul></ul><ul><ul><li>Peritonitis </li></ul></ul><ul><ul><li>Polyarteritis nodosa </li></ul></ul><ul><ul><li>Vasitis nodosa </li></ul></ul><ul><ul><li>Xanthogranuloma </li></ul></ul>
  40. 40. Intraoperative consultations <ul><li>Tumors/Benign lesions </li></ul><ul><ul><li>Adrenal tumor </li></ul></ul><ul><ul><li>Dermoid cyst </li></ul></ul><ul><ul><li>Epidermoid cyst </li></ul></ul><ul><ul><li>Epididymal cysts and nodules </li></ul></ul><ul><ul><li>Calculi </li></ul></ul><ul><ul><li>Hemangioma </li></ul></ul><ul><ul><li>Lymphangioma </li></ul></ul><ul><ul><li>Mesenchymoma </li></ul></ul><ul><ul><li>Neuroblastoma </li></ul></ul><ul><ul><li>Paratesticular cyst/rhabdomyosarcoma </li></ul></ul><ul><ul><li>Spermatic cord tumors </li></ul></ul><ul><ul><li>Spermatocele </li></ul></ul><ul><ul><li>Tunica albuginea cyst </li></ul></ul><ul><ul><li>Tunica vagnialis cyst </li></ul></ul><ul><ul><li>Venous thrombosis </li></ul></ul><ul><ul><li>Wilms’ tumor </li></ul></ul>
  41. 41. Intraoperative consultations <ul><li>Lesions secondary to patent processus </li></ul><ul><ul><li>Adrenal hemorrhage </li></ul></ul><ul><ul><li>Endometriosis </li></ul></ul><ul><ul><li>Meconium </li></ul></ul><ul><ul><li>blood </li></ul></ul>
  42. 42. QUESTIONS
  43. 43. <ul><li>A newborn boy has not voided for the first 18 hours of life. A renal ultrasound is obtained. The next step is: </li></ul><ul><li>Observation </li></ul><ul><li>VCUG </li></ul><ul><li>MAG-3 Renal Scan </li></ul><ul><li>Urethral Catheter </li></ul><ul><li>Serum Creatinine </li></ul>
  44. 44. <ul><li>A 2 month-old uncircumcised boy has an E.Coli UTI with a temperature of 104 degrees. Physical exam is normal except for a phimotic prepuce. He responds well to antibiotics. The next step is: </li></ul><ul><li>Circumcision </li></ul><ul><li>Circumcision and cystoscopy </li></ul><ul><li>Renal ultrasound </li></ul><ul><li>VCUG and ultrasound </li></ul><ul><li>DMSA renal scan </li></ul>
  45. 45. <ul><li>A 24 hour old neonate has a left scrotal mass which does not transilluminate. The most likely diagnosis is: </li></ul><ul><li>Extravaginal torsion </li></ul><ul><li>Intravaginal torsion </li></ul><ul><li>Incarcerated hernia </li></ul><ul><li>Endodermal sinus (yolk sac) tumor </li></ul><ul><li>Torsion of a testicular appendage </li></ul>
  46. 46. <ul><li>A 6-week old boy was born at 27 weeks gestation. His postnatal course has been complicated by respiratory distress, bronchopulmonary dysplasia, and a PDA. He has required long-term diuretic therapy. A KUB reveals calcifications in the mid and upper regions consistent with bilateral renal calculi. The most likely mechanism for the formation of the stones is: </li></ul><ul><li>Hypercalciuria </li></ul><ul><li>Hyperuricosuria </li></ul><ul><li>Obstructive uropathy </li></ul><ul><li>Type I RTA </li></ul><ul><li>Type II RTA </li></ul>
  47. 47. <ul><li>A newborn undergoes a circumcision with a Gomco clamp. Three days later he has 2 separate urinary streams. The next step is: </li></ul><ul><li>Observation </li></ul><ul><li>Antibiotics </li></ul><ul><li>Testosterone cream </li></ul><ul><li>VCUG </li></ul><ul><li>RUG </li></ul>
  48. 48. <ul><li>A 30 year-old woman wishes to become pregnant following renal transplantation. Her serum creatinine is 1.2. She should be counseled that pregnancy will likely be associated with: </li></ul><ul><li>Fetal genetic abnormality </li></ul><ul><li>Preterm delivery </li></ul><ul><li>Allograft rejection </li></ul><ul><li>Preeclampsia </li></ul><ul><li>Intrauterine growth retardation </li></ul>
  49. 49. <ul><li>A newborn baby had bilateral hydroureteronephrosis. Postnatal ultrasound reveals bilateral moderate caliectasis and markedly dilated ureters. Serum creatinine is 0.4. VCUG is normal. A diuretic renogram shows prompt uptake of radionuclide but the washout is indeterminate. The next step is: </li></ul><ul><li>Whitaker test </li></ul><ul><li>Ureteral reimplantation with tailoring </li></ul><ul><li>Temporary cutaneous ureterostomies </li></ul><ul><li>Observation </li></ul><ul><li>Prophylactic antibiotics </li></ul>
  50. 50. <ul><li>Nephrocalcinosis and/or renal calculi that occur in pre-term infants on diuretics: </li></ul><ul><li>Is usually unrelated to hypercalciuria </li></ul><ul><li>Usually leads to chronic renal failure </li></ul><ul><li>Is often associated with aminoaciduria </li></ul><ul><li>May completely disappear </li></ul><ul><li>Usually accompanies obstructive uropathy </li></ul>
  51. 51. <ul><li>A one week old boy with prenatally diagnosed left hydronephrosis underwent a sonogram that showed moderate left hydronephrosis. The VCUG was normal. A Mag-3 diuretic renal scan should not be performed now since: </li></ul><ul><li>Administration of radionuclide can be hazardous </li></ul><ul><li>Renal vascular resistance is diminished </li></ul><ul><li>Venous access is difficult </li></ul><ul><li>Restricted oral intake is not tolerated </li></ul><ul><li>GFR is lower in the first week of life </li></ul>
  52. 52. <ul><li>The most important predisposing factor for candidemia in newborns is:: </li></ul><ul><li>IV catheters </li></ul><ul><li>Steroid administration </li></ul><ul><li>Blood dyscrasia </li></ul><ul><li>Antibiotic therapy </li></ul><ul><li>Immunosuppressive therapy </li></ul>
  53. 53. <ul><li>Renal function in the newborn is similar to that in the adult with regard to: </li></ul><ul><li>Free water clearance </li></ul><ul><li>Urinary concentration </li></ul><ul><li>GFR </li></ul><ul><li>Bicarbonate reabsorption </li></ul><ul><li>Urinary acidification </li></ul>
  54. 54. <ul><li>A 28 year old woman with asymptomatic bacteriuria is 27 weeks pregnant. A urine culture is positive for E. Coli. The most frequent complication of failure to treat bacteriuria: </li></ul><ul><li>Acute renal failure </li></ul><ul><li>Premature birth </li></ul><ul><li>Perinephric abscess </li></ul><ul><li>Eclampsia of pregnancy </li></ul><ul><li>Spontaneous abortion </li></ul>
  55. 55. <ul><li>Which of the following suggest an increased risk of genitourinary abnormality in the neonate: </li></ul><ul><li>Newborn serum creatinine of 1.2 </li></ul><ul><li>Maternal insulin-dependent diabetes </li></ul><ul><li>Maternal hypertension </li></ul><ul><li>No urine from the infant in the first 20 hrs </li></ul><ul><li>Maternal history of MCKD </li></ul>
  56. 56. <ul><li>A full-term boy had antenatal hydronephrosis identified during in-utero ultrasound. U/S and VCUG 4 hours post-natally are normal. The next step is: </li></ul><ul><li>No further evaluation </li></ul><ul><li>MAG-3 renal in 3 months </li></ul><ul><li>U/S in 3 months </li></ul><ul><li>Repeat u/s in 24 hours </li></ul><ul><li>IVP in 3 months </li></ul>
  57. 57. <ul><li>A newborn infant is circumcised with a Gomco clamp after administration of a local anesthetic containing 1:1,000 epinephrine. Blanching of the penile skin and glans penis is observed. Physical exam demonstrates mild peripheral vasoconstriction of the hands and feet, blood pressure of 90/60 mm Hg, and a pulse of 160. The next step is: </li></ul><ul><li>IV papaverine </li></ul><ul><li>IV propranolol </li></ul><ul><li>SubQ phentolamine </li></ul><ul><li>Caudal sympathetic block </li></ul><ul><li>Oral nifedipine </li></ul>