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8 Urologic Emergencies in Children: Special Considerations

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8 Urologic Emergencies in Children: Special Considerations Chapter 8 Urologic Emergencies in Children: 8 Special Considerations A. Cook, A.E. Koury fore cover common emergent and urgent pediatric uro- 8.1 Introduction 73 logic consultations encountered from birth through 8.2 Adrenal 73 childhood. Prenatal diagnoses and their respective 8.2.1 CAH and Intersex 73 8.2.2 Adrenal Hemorrhage 75 management options (such as fetal obstructive uropa- thy) will not be considered, as they are beyond the 8.3 Kidney 75 8.3.1 Anomalies and Masses 75 scope of this chapter and do not necessarily reflect the 8.3.2 Cystic Renal Lesions 76 typical urologic conditions encountered in the emer- 8.3.3 Solid Renal and Juxtarenal Lesions 78 gency department. The chapter will progress via an an- 8.3.4 Pyelonephritis and Pyonephrosis 80 atomical top-down approach, emphasizing various 8.3.5 Trauma 82 conditions from adrenal disorders to scrotal and testic- 8.3.6 Calculi 83 ular pathology. 8.4 Bladder 84 8.4.1 Exstrophy 84 8.4.2 Bladder Trauma 86 8.4.3 Bladder Rupture Postaugmentation 87 8.2 8.4.4 Urinary Retention 87 Adrenal 8.5 External Genitalia 89 8.2.1 8.5.1 Penis 89 8.5.1.1 Circumcision Injuries 89 CAH and Intersex 8.5.1.2 Paraphimosis and Phimosis 90 8.5.1.3 Urethral Trauma 91 Although sexual ambiguity in the newborn can be a 8.5.2 Scrotum 93 very distressing condition for the new parents of an af- 8.5.2.1 The Acute Scrotum 93 fected child, investigations attempting to elucidate the 8.5.2.2 Testicular Torsion 93 underlying etiology for ambiguity must be undertaken 8.5.2.3 Neonatal Torsion 93 rapidly in order to avoid potentially fatal complica- 8.5.2.4 Pediatric Torsion 94 8.5.2.5 Epididymitis 94 tions. Intersexuality results from either the genital 8.5.2.6 Idiopathic Scrotal Edema 95 masculinization of a female fetus or the arrest thereof 8.5.2.7 Henoch-Schönlein Purpura 96 in a male during development. This usually occurs be- 8.5.2.8 Scrotal Masses 96 8.5.2.9 Testis Tumors 96 References 97 8.1 Introduction Pediatric urologic emergencies fortunately remain rare occurrences within the emergency department of a hospital or ambulatory care center. More commonly, congenital anomalies noted at birth, or benign lesions that prompt significant parental anxiety (such as be- nign scrotal conditions), often result in a visit to the emergency department for evaluation. These urgencies nonetheless require both the appropriate investiga- tions and management in order to allay patient and pa- Fig. 8.1. Phenotypic appearance of a newborn 46, XX female rental concern. The objective of this chapter is to there- with moderate virilization secondary to CAH 74 8 Urologic Emergencies in Children: Special Considerations Table 8.1. Classification of intersex disorders Category Gonadal histology Genotype Phenotype Female pseudohermaphro- Histologically normal ovaries 46, XX Variable degrees of virilization ditism True hermaphroditism Ovarian and testicular tissue Variable, most com- Variable appearance, depending on monly mosaic 46,XX/ amount of viable testicular tissue 46,XY or 47,XXX/46XY Male pseudohermaphro- Histologically normal testes 46, XY Partial or complete failure of mas- ditism culinization Pure or mixed gonadal Dysplastic gonads with abun- Usually both XY and Variable appearance, depending on dysgenesis dant fibrous stroma XO cell lines amount of viable testicular tissue Fig. 8.2. Steroidogenic cascade demonstrating the various adrenal enzymes involved in miner- alocorticoid, glucocorticoid, and androgen production tween the 7th and 14th weeks of gestation and, depend- births (Perry et al. 2005) (Fig. 8.2). Although numerous ing on the underlying etiology, may have profound ef- enzymatic defects have been identified, deficiencies in fects on the ultimate phenotypic appearance of the gen- 21-hydroxylase account for more than 90% of cases italia (Fig. 8.1). (Forest 2004). Deficient 21-hydroxylase activity leads to Intersexuality is most conveniently classified ac- an overproduction of the weak adrenal androgens, an- cording to gonadal histology. Four main categories drostenedione and dehydroepiandrosterone, while have been identified that effectively enable the appro- preventing appropriate mineralocorticoid and gluco- priate diagnosis and prognosis of these children with corticoid production. These weak androgens are subse- respect to sexual assignment, potential fertility as well quently converted to testosterone and dihydrotestoste- as future gender identity (Table 8.1). rone, resulting in virilization in the female fetus. The Congenital adrenal hyperplasia (CAH) is the most salt-wasting form affects two-thirds of CAH patients, common underlying cause of sexual ambiguity and, if where mineralocorticoid and glucocorticoid deficien- not recognized, may lead to death in the neonatal period. cies lead to severe dehydration, hyponatremia, and hy- Although other causes of intersexuality, such as mixed perkalemia. This in turn can lead to fatal cardiac ar- gonadal dysgenesis or true hermaphroditism, may cause rhythmias and hypovolemic shock (Lee and Donahoe distress to the family in the newborn period secondary 1997). If not recognized immediately postpartum, pa- to inability to assign appropriate gender, only patients tients with the salt-wasting form typically present with CAH affected by the salt-wasting form represent a 7–10 days following birth with lethargy, poor feeding, true urologic emergency. Consequently, the scope of this vomiting, or even subsequent to near-miss SIDS epi- section will concentrate on the management of the infant sodes (Gassner et al. 2004). with CAH, bearing in mind that the initial evaluation is Other potential causes of female pseudohermaphro- similar for all infants with ambiguous genitalia. ditism include maternal ingestion of androgenic medi- CAH is caused by an inherited defect in cortisol me- cations during pregnancy, placental aromatase defi- tabolism occurring in 1 in 10,000 to 1 in 15,000 live ciency,or,rarely,hormonallyactivematernalovarian 8.3 Kidney 75 Fig. 8.4. Urogenital sinogram demonstrating the confluence of the vagina posteriorly and the anteriorly oriented urethra, bladder neck, and bladder andvaginaaswellasassessforvesicoureteralreflux (Fig. 8.4). Fig. 8.3. Severe virilization of a 46, XX female with 21-hydroxy- These patients require lifelong mineralocorticoid lase deficiency. As do most CAH patients with significant viri- and glucocorticoid replacement and their optimal lization, this patient presented with the salt-wasting variety management involves a multidisciplinary health care team consisting of pediatric urology, gynecology, en- or adrenal tumors (Ludwig et al. 1998; McClamrock and docrinology, genetics, as well as psychiatry and social Adashi 1992). Therefore, a careful history, particularly work. As the vast majority with CAH can appropriately ascertaining potential maternal drug exposure, is im- be reared as females (46, XX) with potential fertility, portant for elucidating the potential underlying etiolo- lifelong follow-up of these unique patients is impera- gy for virilization. A family history of neonatal death or tive in order to address not only medical, but psychoso- sexual ambiguity may also identify potential cases. cial issues as well, which may arise during growth into Acute resuscitative measures are obviously indicat- adulthood. ed for those infants presenting with signs of shock and dehydration. Physical examination will reveal various 8.2.2 degrees of virilization, from mild clitoral hypertrophy, Adrenal Hemorrhage to a fully developed phallus, rugated scrotum and im- palpable gonads (Fig. 8.3). In general, patients with Adrenal hemorrhage uncommonly presents as an adre- salt-wasting tend to present with more severe virilizati- nal mass. Most are incidentally discovered on imaging on. Laboratory investigations are directed toward de- and are associated with birth trauma, neonatal asphyx- termining the underlying cause and typically include ia, septicemia, or coagulopathies. Expectant manage- serum electrolytes, glucose, gonadotropin, testoster- ment and serial US are usually all that are required; one, dihydrotestosterone, androstenedione, dehydroe- however, pathologic jaundice may necessitate a course piandrosterone,aswellaschromosomalanalysis.As of phototherapy or even transfusion (Sherer et al. the vast majority of females with CAH will harbor a de- 1994). Subsequent adrenal insufficiency very rarely oc- fectinthe21-hydroxylaseenzyme,theywilldemon- curs and usually necessitates temporary steroid re- strate significantly elevated levels of that enzyme’s sub- placement only (Velaphi and Perlman 2001). strate, namely 17-hydroxyprogesterone. If 17-hydroxy- progesterone levels are not elevated, other rare enzy- matic defects may be present such as 11-betahydroxy- 8.3 lase, 20,22-desmolase, or 3B-hydroxysteroid dehydro- Kidney genase (Dacou-Vouteakis et al. 2001). 8.3.1 Abdominal and pelvic ultrasound (US) is important Anomalies and Masses todocumentthepresenceofauterusandovariesas well as rule-out any upper urinary tract anomalies. A number of lesions (both of GU and non-GU origin) Furthermore, fluoroscopic genitography will demon- may present as a palpable abdominal mass in the infant strate the confluence of the urogenital sinus, urethra, or young child and require assessment in the emergen- 76 8 Urologic Emergencies in Children: Special Considerations Table 8.2. Classification of palpable abdominal masses Cystic Solid renal/ Midline Nongenitourinary renal juxtarenal lesions lesions lesions lesions Hydro- CMN Urinary Gastrointestinal nephrosis
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