Alexander J.C. Mittnacht, MD

• Director, Pediatric Cardiac Anesthesia
• Associate Professor
• Department of Anesthesiology
• Mount Sinai Medical Center
• New York, New York

Like patients with a posterior urethral valve or neurogenic bladder heart attack 51 best buy altace, lifetime urologic care will be needed (Wymer et al arrhythmia etiology generic altace 1.25 mg on line. Bladder Duplication Duplication of the bladder and urethra can be complete or incomplete with variable orientation blood pressure up altace 10 mg on-line. Abrahamson (1961) attempted to classify the various bladder duplication anomalies and found complete duplication in the sagittal plane the most common blood pressure medication and ed cheapest altace. In incomplete duplications blood pressure cuff purchase generic altace canada, the two bladder halves communicate and are usually drained by a single urethra blood pressure low pulse high cheap altace 5mg without prescription. Although the size and quality of each entity can be different, they are usually supplied with their own ureter and are drained by an individual urethra and external meatus (Esham and Holt, 1980). This leads to ipsilateral renal dysplasia via complete obstruction (Cheng and Maizels, 1996). Association with other nonurologic congenital anomalies are more frequent in sagittal than coronal duplications. In duplication variations of the classic cloacal-bladder exstrophy complex, patients have an exstrophic bladder and urethra in addition to a closed regular intra-abdominal bladder (Perren and Frey, 1998). However, many children are not diagnosed until recurrent infections or incontinence initiates a urologic workup. Although similarities exist, there are many variations necessitating individual management. Complete preoperative diagnostic evaluations with karyotype, ultrasound, videourodynamic studies, genitogram, and cross-sectional imaging are useful to understand the anatomic relationships. Often the final treatment plan has to be deferred until the time of endoscopic and surgical exploration of the malformation. Initial treatment is directed toward renal preservation and prevention of infections by relieving possibly obstructed genitourinary tracts. Long-term goals include achieving continence and reconstructing the internal and external genitalia. Incomplete duplications may not require surgical procedures if both bladder halves are sufficiently drained by a common urethra. If one is incompetent, the corresponding bladder neck can be closed and the connected urethra excised. Small, asymptomatic congenital diverticula detected during unrelated workups can be treated conservatively. He attributed it to higher voiding pressures even without the presence of infravesical obstruction. These diverticula are often found in children with generalized connective tissue diseases, such as Ehlers-Danlos, Williams, or Menkes syndrome, but are occasionally seen in the absence of a known genetic syndrome (Babbitt et al. Interestingly, pathological analysis does not necessarily show the classic herniation of mucosa through a bladder muscle defect but instead an attenuated muscle wall, allowing laxity in the absence of obstruction (Garat et al. If the child is asymptomatic, then close observation is recommended, especially in the setting of the earlier-mentioned connective tissue disorders. Unfortunately, recurrence or formation of new diverticula is common in syndromic patients, so surgical treatment must be carefully weighed with quality-of-life considerations. In some cases, urinary diversion with vesicotomy or suprapubic drainage may be preferred. Urodynamics should be pursued postoperatively to assess for any obstruction or high-pressure voiding that could expedite recurrence. The urogenital duplications also can be left uncorrected if the patient is asymptomatic; Gastol reported two successful pregnancies in a 26-year-old woman (Gastol et al. Because of the rarity of the disease and the large variety of manifestations, the surgeries must be individualized and should be performed in centers experienced in complex urogenital reconstruction. Congenital Diverticula Diverticula typically develop as herniation of bladder mucosa between defects of bladder smooth muscle fibers. The neck of the resulting diverticulum depends on the size of the muscular defect. The true incidence is likely higher as symptomatic diverticula are rare and undiscovered. Nuclear renal studies can be used to obtain information concerning anatomy, kidney function, and ureteral obstruction if hydroureteronephrosis is present. Radiographic studies may have to be repeated in cases of continuous clinical suspicion. Paraureteral diverticula are by far the most common congenital diverticula and categorized as primary or secondary by Hutch in his landmark article (Hutch, 1961). Primary paraureteral diverticula are seen in smooth-walled bladders, occur isolated with no other diverticula, can be intermittent on imaging, and happen in children with no infravesical obstruction. Secondary paraureteral diverticula are found in trabeculated bladders as one of many diverticula in the bladder, are always present on imaging, and are caused by infravesical obstruction. Primary diverticula arise as a localized herniation of bladder mucosa through the ureteral hiatus between the intravesical ureter and the roof of the ureteral hiatus. Some authors have implied an isolated defect in the Waldeyer sheet; however, congenital Urachal Anomalies the treatment of a symptomatic urachal remnant in a child typically requires complete surgical excision with a bladder cuff. In the left panel, the voiding cystourethrogram demonstrates the large superior diverticulum (A) and a smaller one posteriorly near the bladder neck (B). In right panel, postvoid film reveals the residual urine volume within the diverticulum. It is the asymptomatic incidental urachal remnant that presents a clinical conundrum. Although urachal adenocarcinoma in a child is unreported, malignant transformation in an adult can be devastating with a high mortality and a much more complicated surgical excision. Patent urachus is explained by nondescent of the bladder or, more commonly, failure of the epithelial-lined urachal canal to obliterate. Bladder obstruction during fetal development has been blamed for the urachus remaining tubular. The fact that urachal patency is often absent with severely obstructed bladders in utero, however, casts doubt on this theory. In addition, only up to 14% of patients with a patent urachus have postnatal confirmation of in utero bladder obstruction (Mesrobian et al. Obliteration of the urachus may be independent from the level of bladder distention. Therefore retubularization, rather than primary patency, may be the cause for urinary drainage from the umbilicus. This theory is supported by reports of umbilical urinary fistulas in acquired bladder obstructions later in life (Berman et al. A patent urachus is suspected in the neonatal period by continuous or intermittent drainage of fluid from the umbilicus. The most common organisms cultured from the umbilical drainage include Staphylococcus aureus, Escherichia coli, Enterococcus, Citrobacter, and, rarely, Proteus species (Mesrobian et al. Additional manifestations include an enlarged or edematous umbilicus and delayed healing of the cord stump (Razvi et al. It is important to differentiate the condition from a patent omphalomesenteric duct. Management of an infected urachus with abscess formation includes initial drainage under antibiotic coverage. Once the infection has subsided, complete excision of the patent urachus, including a bladder cuff, is required (Nix et al. This avoids recurrences or stone formation and prevents the rare event of later transformation into a malignant adenocarcinoma (Ashley et al. Traditionally, the patent urachus is surgically excised using a transverse or midline infraumbilical incision. In infants, a small transverse subumbilical incision is often possible because the bladder dome is still high. A feeding tube or small catheter is placed into the patent urachus for better intraoperative identification. The urachus can be incised circumscriptly, without removing the umbilicus for cosmetic Chapter 30 reasons. The rectus fascia is incised in a longitudinal fashion, muscles spread apart, and the dome of the distended bladder identified. The dissection continues extraperitoneally toward the umbilicus until the previously incised umbilical portion is free and can be pulled into the surgical field. A bladder cuff including the urachal insertion is marked and excised using electrocautery. Khurana and Borzi (2002) described their laparoscopic experience with 4 children between 5 months and 10 years and found the procedure to be safe in children of all ages. Technically, they suggested a three-port approach, with the camera port in the midline between the umbilicus and the xiphoid and two working ports on either side in the upper quadrant. Early in their series they inserted the camera port at the umbilicus with working ports into the right and left upper abdomen. Later they preferred to place the camera into the left lower abdominal wall, with 2-mm working ports at the left lower and upper abdomen for better visualization of the urachus at the umbilicus. They reported no intraoperative or postoperative complications, no recurrences, and a median operating time of 35 minutes. The advantage of the laparoscopic technique is the good visualization of the course of the urachus and the bladder dome. However, laparoscopic techniques require an intra-abdominal approach and pose the potential risk for spilling infected or malignant material into the abdominal cavity as well as intraperitoneal urinary extravasation. Robotic-assisted removal and single-port laparoscopy also have been suggested (Ahmed et al. In the umbilical-urachus sinus, the urachus obliterates at the bladder level but remains open at the umbilical site, causing a continuously draining sinus. The caudal part of the urachus is filled with desquamated epithelial cells, and no connection to the bladder can be identified. These structures can be very difficult to differentiate from an umbilical-urachus sinus because no connection to the bladder or bowel can be seen on sinugram. However, the surgical approach to both anomalies requires the complete excision of all tissue. Unlike urachal structures, omphalomesenteric remnants can show gastric or small bowel mucosa on histologic examination. However, the fluid-filled cyst can drain through the umbilicus or into the bladder intermittently. Urachal cysts are found more commonly in the distal part of the urachus and manifest more commonly in adults than in infants or children (Cilento et al. These cells can become infected; Staphylococcus aureus has been identified as the most common organism (Mesrobian et al. Once infected, urachal cysts can manifest as umbilical abscess formation or bladder infections. Additional symptoms include localized lower abdominal pain, voiding symptoms, or even a painful palpable mass. The diagnosis is confirmed by ultrasound, demonstrating the localized cyst between the anterior abdominal wall and the peritoneum. If unrecognized, the infected cyst can perforate into the bladder (Maruschke et al. The urachus obliterates almost completely, except at the level of the bladder apex. These lesions are usually asymptomatic and found Bladder Anomalies in Children 525 incidentally on nonrelated radiographic workups. Although the diverticula can enlarge in the case of urinary obstruction, this rarely causes problems because they tend to have a large opening and drain into the bladder well. Stone formation and urinary tract infections have been reported, especially in the case of a narrowed neck causing the need for intervention. After bladder outlet resistance is normalized, diverticulectomy may become unnecessary because of natural remodeling of the bladder. If symptomatic, the diverticulum should be excised with ipsilateral ureteral reimplantation if it is near or included in the diverticulum. This can be successfully performed intravesically or extravesically and open or laparoscopically/ robotically (Christman and Casale, 2012; Jayanthi et al. Secondary paraureteral diverticula are acquired and develop as a result of existing infravesical obstruction or a neurogenic bladder as originally described by Hutch (1961). The resulting increased infravesical pressure forces the bladder mucosa to bulge between the muscle fibers. These diverticula are usually just one of many pop-off mechanisms that can occur throughout the bladder. These diverticula also can be caused by weakening in the bladder muscle by infection (Barrett et al. The increasing diverticulum pulls the intravesical ureter out of its anchored position, causing dysfunction of the ureterovesical junction. Eventually, the enlarged diverticulum can become responsible for ureteral obstruction. This even has been associated with renal dysplasia (Amar, 1972; Livne and Gonzales, 1985). Paraureteral diverticula or posterolateral diverticula can become so large that they compress the bladder neck or posterior urethra. The resulting bladder outlet obstruction starts a vicious circle by continuously filling and expanding the diverticulum. This increases the obstruction and subsequently causes complete urinary retention (Sheldon and Essig, 1994; Zia-Ul-Miraj, 1999). This can be seen with the congenital diverticular syndromes discussed previously, leading to surgical intervention.

Syndromes

• Megaloblastic anemia
• Thyroid function tests
• Limiting dairy products if you have problems digesting dairy fats. Try low-lactose cheeses, such as Swiss and cheddar, and an enzyme product, such as Lactaid, to help break down lactose.
• Before meals: 70 - 130 milligrams per deciliter (mg/dL)
• Complete blood count (CBC) to check for anemia or low blood count
• Bad breath or loss of smell

Bartter syndrome is an autosomal recessive disorder pulse pressure of 96 order altace 1.25 mg line, which usually presents in the first year of life with failure to thrive blood pressure chart numbers order generic altace from india. Typically blood pressure 160 100 buy altace from india, patients have a prenatal history of polyhydramnios and prematurity arrhythmia future cure cheap altace online, and polydipsia and polyuria as infants and young children blood pressure effects 5 mg altace visa. Hypokalemic metabolic alkalosis is the defining feature of Bartter syndrome (Seyberth and Schlingmann pulmonary hypertension xanax cheap altace 5 mg mastercard, 2011). Blood pressure is typically normal, but plasma renin and aldosterone levels are elevated as a result of volume depletion. Bartter syndrome is commonly caused by mutations in the Na/K/2Cl cotransporter or in Barttin, which is associated with sensory neural deafness. Treatment of patients with Bartter syndrome is centered on replacement of urinary solute loss and management of polyuria. Gitelman syndrome is an autosomal recessive disorder that also presents with hypokalemic alkalosis and can be confused with Bartter syndrome (Seyberth and Schlingmann, 2011). Patients with Gitelman syndrome typically do not have failure to thrive, and there is no antenatal history of polyhydramnios. Hypomagnesemia is nearly universal in Gitelman syndrome, and urinary calcium excretion is low in Gitelman syndrome as opposed to Bartter syndrome, in which urinary calcium excretion is elevated. Many patients with Gitelman syndrome have a history of tetany and muscle cramps secondary to hypomagnesemia, which is an uncommon feature of Bartter syndrome. The keystone of treatment for Gitelman syndrome is magnesium and potassium supplementation to replace urinary loss. Many patients are diagnosed prenatally or during the evaluation of hypertension, gross or microscopic hematuria, abnormal renal function, polyuria, or incidentally as part of an evaluation of abdominal pain or constipation. Often a specific diagnosis can be determined by imaging alone or by family history. Currently there are no disease-specific treatment options that can alter disease progression or correct the underlying disorders. Serial monitoring of renal function, blood pressure, and renal ultrasonography is generally recommended at an interval of 3 months for the first year of life and then every 6 months up to involution, or at least up to 5 years. As a result, there is growing consensus that these patients should be monitored annually until well into adulthood. The kidneys are bilaterally enlarged as a result of fusiform cysts arising from the collecting ducts. Neonatal presentation with oligohydramnios/anhydramnios has a reported mortality rate of 25% to 50%. However, hypertension and urinary tract infections should be treated aggressively to limit disease progression. Although increasingly less common in the United States, stones may be identified as part of the evaluation of urinary tract infections. It is not unusual for nephrolithiasis to be noted incidentally as part of a diagnostic evaluation for nonspecific complaints such as recurrent abdominal pain or failure to thrive. In the United States, the majority of stones are found in the kidney, and presentation with bladder stones is more common in those with underlying urologic abnormalities. Hypercalciuria is the most common risk factor identified in children with urolithiasis, and the vast majority have idiopathic hypercalciuria. Systemic hypercalcemia is less common and is associated with hyperparathyroidism, immobilization hypercalcemia, thyroid disease, idiopathic hypervitaminosis D, corticosteroid use, Williams syndrome, and rarely, mutation of the calcium-sensing receptor. Children with hypercalciuria are much more commonly normocalcemic, and with the exception of those on loop diuretics, most will have idiopathic hypercalciuria. There is, however, an increasing understanding of the genetic underpinnings of tubular dysfunction as a cause of hypercalciuria. Hyperoxaluria is a less common cause of urolithiasis and can be either primary or secondary. Secondary causes include enteric hyperoxaluria, excess vitamin C intake, and oxalate-rich diets. Uric acid stones caused by hyperuricosuria can be either a primary disorder seen with inborn errors of purine metabolism (Lesch-Nyhan syndrome) or, more commonly, a secondary process associated with hematologic malignancies, gout, high purine intake, tubular disorders, or diabetes mellitus. The evaluation of nephrolithiasis should begin with a history focused on symptoms associated with stones and predisposing factors including recurrent urinary tract infection, familial history of nephrolithiasis, gout, and conditions that increase enteric oxalate absorption: inflammatory bowel disease, short gut syndrome, or cystic fibrosis (Edvardsson et al. A through dietary history should be taken including sodium, calcium, fluid intake, and vitamin supplementation. In the presence of hypercalciuria, hypercalcemia, or hypophosphatemia, serum parathyroid hormone and vitamin D levels should be obtained. If the stone is available, it should be sent for analysis by infrared spectroscopy or radiographic diffraction. Timed urine collections for calcium, oxalate, urate, citrate, and sodium should recommended in the pediatric population unless there are clinical signs or symptoms that warrant imaging. In patients with suspected recessive nephronophthisis, comprehensive genetic analysis is warranted as this can help to predict both complications and disease progression, and importantly can help with caregiver education. Currently there are more than 100 distinct genes that have been associated with nephronophthisis. Mutations in the gene encoding uromodulin/Tamm-Horsfall protein have been linked with one of the two types of medullary cystic kidney disease (type 2). In addition, glomerulocystic kidney disease is characterized by multiple small cortical cysts that result from cystic dilation of the Bowman space; the lack of tubular involvement distinguishes it from other forms of cystic disease. And medullary sponge kidney is a rare congenital cystic disorder in which there is ectasia of cortical ducts within the inner medulla of the kidney, resulting in a spongelike appearance on radiograph and is associated with nephrolithiasis and urinary tract infection. Although not as common as in the adult population, it is estimated that nephrolithiasis accounts for between 1 in 600 and 1 in 800 pediatric hospital admissions in the United States. Recent data suggest that the incidence of urolithiasis in the adolescent population has increased significantly over the past 10 years from roughly 20 per 100,000 in the late 1980s to more than 50 per 100,000 in 2014; this number is likely to be an underestimate as urolithiasis remains under-diagnosed because many children are asymptomatic at the time of diagnosis (Braun et al. Concurrent with the increase in the frequency of stone disease has been a shift in the types of stones seen at presentation. There has been a marked drop in the incidence of infectious stones from more than 60% before the 1980s to now less than 20%; there are similar reported reductions in the frequency of uric acid and ammonium acid urate stones. The reasons behind these shifts are not clear, however in the United States, it has been proposed that changes in dietary practices, changes in types of fluids used for hydration, and the obesity epidemic underlie the rise in pediatric stone disease. Reported rates for stone recurrence in the pediatric population are variable, ranging from 15% to 20%, but may be as high as 30% in individuals with underlying metabolic disorders. The clinical presentation of nephrolithiasis in children is varied and includes microscopic or gross hematuria, urgency, dysuria, Chapter 21 be performed for any child with stones if possible. For children in whom 24-hour urine collections are not feasible, spot ratios of urine solute to urine creatinine can be substituted. It is important to remember that normative data for urinary solute excretion vary with age. For example, a normal urine calcium-to-creatinine ratio for a 2-month-old child is in the 0. One of the primary goals of the initial evaluation is to identify patients with underlying metabolic disease for whom specific therapies will be beneficial and to determine which individuals are at risk for recurrent disease. In terms of imaging, to limit radiation exposure, ultrasonography is commonly employed in children for initial screening (Smith-Bindman et al. Standard abdominal radiographs remain useful to determine stone burden and for longitudinal assessment of response to therapy. Genetic testing is an increasingly important part of the evaluation of pediatric stone disease, as monogenic disorders may underlie urolithiasis or nephrocalcinosis in 20% of children. Medical therapy should be directed toward the underlying metabolic cause, if one has been identified, to prevent recurrence and growth of existing stones (Hernandez et al. With hypercalciuria, salt restriction will reduce urinary calcium losses by promoting reabsorption of sodium and calcium. Avoiding excessive protein intake to reduce the acid load is also recommended in recurrent stone formers. A thiazide diuretic can be used in the normocalcemic hypercalciuric patient to increase distal tubular reabsorption of calcium. For primary hyperoxaluria, the goal of therapy is to decrease oxalate production, increase urine calcium oxalate solubility, and decrease crystal deposition in the kidney. Potassium citrate, orthophosphate, or magnesium citrate formulations are used to decrease urinary calcium oxalate supersaturation. In individuals with hypocitraturia, a diet rich in fruits and vegetables, and/or supplemental potassium citrate is recommended. Sodium citrate should be avoided because the excess salt may increase hypercalciuria. If stone formation continues despite high fluid intake and alkalinization of the urine, D-penicillamine or tiopronin can also be used. In hyperuricosuria, allopurinol, alkalinization of urine, and avoidance of protein excess are recommended. Struvite calculi often require surgical removal and prolonged treatment with antibiotics. For children who present with acute renal colic, analgesia and adequate hydration with appropriate monitoring of fluid and electrolyte status are the mainstays of therapy. Radiologic evaluation should be performed, and in the setting of urinary obstruction, infection, acute kidney injury, or incontrollable pain, urologic intervention may be needed. For small ureteral stones (< 10 mm), management with observation with or without medical expulsive therapy is recommended. The choice of intervention is typically guided by stone size, composition, and location. Complete removal is important in children as residual small fragments are associated with regrowth and adverse outcomes. Medical expulsive therapy with an -blocker has been successful in the management of small ureteral stones. Percutaneous nephrolithotomy is the recommended treatment for large renal pelvic or caliceal stones (>20 mm). Ureteroscopy is increasingly becoming a first-line procedure of choice, Urologic Aspects of Pediatric Nephrology 351 especially for distal ureteral stones greater than 10 mm. Open surgery is usually reserved for very young children with complex stones, children with orthopedic issues that limit positioning, or those who have failed primary therapy. Urolithiasis has a high rate of recurrence, especially if an underlying metabolic disorder exists. Prevention with appropriate medical therapy and high fluid intake should be encouraged to limit morbidity. The estimated prevalence of hypertension in children is much lower than the prevalence seen in the adult population; however, data suggests the increase in the prevalence in hypertension seen in the pediatric population in recent years is closely linked to increasing obesity rates in children (Din-Dzietham et al. Weight-related disorders such as hypertension are now increasingly common in pediatric patients and are likely to account for the rise in the prevalence of hypertension from 2. An accurate measurement of blood pressure is essential to the diagnosis of hypertension (National High Blood Pressure Education Program, 2004). Systolic blood pressure is defined by the first Korotkoff sound, with the disappearance of the Korotkoff sounds defining diastolic blood pressure. As accurate auscultatory measurement of blood pressure is often difficult in infants and young children, the use of automated (oscillometric) blood pressure measurement devices is now widespread; however it should be noted that pediatric normative blood pressure data is based on auscultatory and not oscillometric standards. A recent clinical practice guidelines for management of hypertension in children and adolescents (Flynn et al. A major focus of this report was improving the recognition of hypertension by simplifying the initial screening blood pressure table. The blood pressure classification scheme has also been revised to align the classification system for adolescents with the absolute blood pressure values used in adult blood pressures guidelines (Redwine et al. The term prehypertension has been abandoned and replaced with elevated blood pressures to be consistent with the adult guidelines. Staging of hypertension is useful in determining which children require more immediate attention for diagnostic evaluation and management. Echocardiograms are no longer recommended as part of the initial screening of patients with confirmed hypertension. However, baseline echocardiography is now recommended at the initiation of pharmacologic therapy for all patients regardless of the etiology of their hypertension. For patients with stage 1 hypertension or those with primary (essential) hypertension without end-organ damage, therapeutic lifestyle changes are the initial intervention of choice (Lurbe et al. However, like all behavioral interventions, the long-term success of therapeutic lifestyle changes requires engagement not only of the patient, but of family members as well. Pharmacologic therapy should be considered in patients with primary (essential) hypertension who do not have improvement in their blood pressure after a trial of therapeutic lifestyle changes, or in patients with stage 2 hypertension, evidence of end-organ damage, symptomatic hypertension, or secondary hypertension (Flynn and Daniels, 2006). Unlike adult hypertension guidelines, there are no data or evidence-based guidelines regarding which drug to select when initiating pharmacologic therapy. Rather than standardized approaches to pharmacologic therapy, most practitioners individualize therapy with the underlying renal, cardiac, or endocrine conditions guiding the choice of the initial agent. Adverse effects of each agent and available formulations need to be considered, because many standard adult hypertensive medications are not available in liquid formulations or are in fixed ratios that are not suitable for the pediatric population. Once therapy is initiated, blood pressure should be monitored closely, including laboratory monitoring, both for efficacy of the antihypertensive medication and for potential side effects. Based on data suggesting that end-organ damage is present in patients with blood pressures greater than the 90th percentile but less than the 95th end-organ damage from hypertension. Two other clinically relevant designations for severity of hypertension are important: (1) hypertensive emergency, when a patient has end-organ injury such as encephalopathy, pulmonary edema, or cerebral hemorrhage or clinical symptoms like blurred vision, headache, or nausea, which can be associated with severely elevated blood pressure; and (2) hypertensive urgency, when blood pressure is severely elevated without clear evidence of end-organ involvement or overt symptoms.

Bellah reported a relatively high (8/15) tendency to progressive renal failure in his population of pseudo prune patients blood pressure levels women purchase altace 5 mg otc. This may be partially attributed to a delay in diagnosis in the absence of the obvious abdominal musculature deficiency and therefore a tendency to experience recurring urinary tract infections (Bellah et al hypertension from stress order altace toronto. Although there have been isolated reports of adults with no history of urinary tract infections heart attack lyrics sum 41 buy generic altace 2.5 mg on-line, most who present in adulthood eventually develop urinary tract infections from the chronic urinary stasis associated with the syndrome (Culp and Flocks blood pressure ranges by age buy cheap altace on line, 1954) heart attack grill buy discount altace. As noted arteria recurrens order 2.5mg altace otc, neonates can be categorized on the basis of their spectrum of disease (see Table 32. In particular, intervention in the urinary tract is not indicated beyond simple bladder drainage because the fate of these patients usually cannot be altered (Woodard and Smith, 1998). Children in this category require regular monitoring of urinary tract dilation (ultrasonography) and renal function (serum creatinine), as well as urinary tract infection. Some of these patients have persistent vesicoureteral reflux that may require surgical treatment on mid- to long-term follow-up, if symptomatic. Evaluation of renal function or renal drainage, or both, is required in those with renal insufficiency. In the presence of poor renal function, assessment of renal outflow obstruction by nuclear scan techniques may be limited; therefore selective use of the Whitaker antegrade perfusion test may be helpful. Compilation of the cases reported in the literature between 1950 and 1970 by Waldbaum and Marshall (1970) revealed that 86% of the 56 accurately traceable patients had died, with or without surgical intervention. With the recognition that infection and progressive renal insufficiency are the factors that most often pose the greatest threat to quality of life and survival, surgical reconstruction to normalize the anatomy and function of the genitourinary tract was advocated. Early reconstruction of the urinary system to reduce stasis and eliminate reflux or obstruction has included ureteral shortening, infolding, and vesicoureteral reimplantation as well as reduction cystoplasty. Although rarely, eventual dysplastic or hydronephrotic kidneys with severely reduced function may require removal if symptomatic. Reconstruction is best delayed until the child is at least 3 months of age to allow for pulmonary maturation. This approach has been successful in achieving anatomic and functional improvement as evidenced by stable radiographic studies, stable creatinine values, and a reduced occurrence of infection (Jeffs et al. Early urinary tract reconstruction can be done comprehensively with orchiopexy, abdominoplasty, and circumcision without increasing the morbidity of the procedure. In a report of comprehensive reconstructive experience, 35 patients who have undergone urinary tract reconstruction have maintained a normal creatinine level, whereas 5 demonstrated moderate to severe renal insufficiency, and 3 required renal transplantation in a follow-up ranging from 15 months to 26 years (Lopes et al. Proponents advocate close surveillance with medical management of bacteriuria and surgical intervention only in patients with proven obstruction or intractable infection. There is no reason to believe that reflux in this population is any less important, and correction of high-grade reflux seems prudent. Of these 11 patients, who were monitored from infancy, 9 remained well except for a few urinary tract infections for periods of up to 24 years. Spontaneous improvement in ureteral appearance and function may occur with normal growth and elongation of the ureters (Duckett et al. Also, some patients with gross abnormalities of the urinary collecting system have survived decades without medical attention (Asplund and Laska, 1975; Lee, 1977; Texter and Koontz, 1980). Urinary Tract Urinary tract reconstruction is generally reserved for children with progressive or severe hydroureteronephrosis, high-grade vesicoureteral reflux, recurrent upper tract infections, true obstructive uropathy, and progressive renal failure. Temporary urinary diversion also has a role in the very young or the very ill child. In some patients the occurrence of repeated upper tract infections or deterioration of renal function dictates the need for temporary urinary diversion. Although cutaneous vesicostomy usually provides adequate upper tract drainage and decompression, in rare instances more proximal diversion is indicated because of ureteropelvic or ureterovesical junction obstruction. When temporary urinary diversion is indicated, a cutaneous vesicostomy is the procedure of choice. This is best done by the Blocksom technique as described by Duckett (1974) and Duckett et al. The goal of remodeling and reimplantation is to reduce urinary stasis and correct massive reflux. The key to success relies on meticulous surgical technique and preservation of the upper few centimeters of proximal ureter, which are often less dilated, for reconstruction. Even then, tailoring or infolding of these segments may be necessary for adequate reimplantation into the abnormal bladder. This step can be difficult, in that the creation of a submucosal tunnel in the abnormal bladder for these ureters can be challenging (Woodard and Trulock, 1986). In cases with associated secondary ureteropelvic junction obstruction, proximal ureterolysis without compromising ureteral vascularization may decompress the renal pelvis. In those with true mechanical obstruction, a nondismembered ureteropyelostomy between the dilated pelvis and a normal upper ureteral segment may normalize urinary drainage. However, they no longer recommend such extensive reconstructive surgery before the age of 3 to 6 months. This leads to the concept of reducing the size of the bladder and remodeling it into a more spheric shape to better direct the contractible forces (Perlmutter, 1976). A variety of approaches have been proposed, from simple excision of the urachal diverticulum to the excision of redundant mucosa with the creation of overlapping between flaps to improve contractibility (Williams and Parker, 1974; Woodard and Trulock, 1986). Over time, however, high bladder capacity and residual volumes seem to recur (Bukowski and Perlmutter, 1994). It seems therefore that reduction cystoplasty would be justified only to remove the larger urachal diverticulum or as part of a more extensive urinary tract reconstruction. In some patients, intermittent catheterization through the urethra or through an appendicovesicostomy channel is likely to afford better long-term bladder emptying with reduction of residual urinary volumes until the patient is able to achieve better voiding pressures with age or as a result of abdominoplasty (Joseph, 1999). The normal resistance of the urinary sphincter has been implicated in "unbalanced" urethrovesical function, contributing to large postvoid residuals. Snyder and Cukier proposed lowering urethral resistance by internal urethrotomy to improve bladder emptying (Cukier, 1977; Snyder et al. In patients studied by urodynamic flow rate profilometry, improved flow rates with reduced residual urine and improvement in the radiographic appearance of the upper tracts were found (Snyder et al. Interestingly, internal urethrotomy did not result in incontinence in this population. Despite these early reports, no further experience with urethrotomy has been described. It is difficult to imagine this procedure being performed in infants and small children currently. Patients with this abnormality may survive without any intervention but frequently require some form of treatment to improve bladder emptying. Surgical correction of megalourethra in a patient with the prune-belly syndrome: (A) Penile deglovement. The redundant urethra can be excised or infolded to provide support and reconstructed over an appropriately sized catheter. Alternatively, some of the urethra can be used to reinforce the urethroplasty because in either form of megalourethra the spongiosum is deficient. Note vesicostomy and double-J stent through the urethra for progressive urethral dilation. Transabdominal orchidopexy with and without preservation of the vascular pedicle in patients with the prune belly syndrome. These factors, along with the potential risk of testicular carcinoma (Massad et al. Because the testes are uniformly located in the abdomen, most commonly on a broad mesorchium overlying the iliac vessels (Coplen et al. Woodard, Parrott, and other researchers noted that if an orchidopexy is done in the neonatal period and up to 6 months of age by a transabdominal approach, adequate spermatic vessel mobilization can usually be achieved for scrotal placement (Fallat et al. This approach is often used in conjunction with other abdominal surgeries, either a vesicostomy, urinary tract reconstruction, or abdominal wall reconstruction. In the absence of need for other abdominal surgeries, this can be accomplished laparoscopically (Philip et al. When successful transabdominal orchidopexy cannot be accomplished in the first few months of life, the other options that may be considered are (1) FowlerStephens orchidopexy (Boddy et al. A meta-analysis report by Docimo (1995) indicated comparative success rates of 67% and 77% for standard and staged Fowler-Stephens techniques, respectively. Operative photograph showing increased vascularity along vas deferens 4 months after first-stage Fowler-Stephens orchidopexy. Children with mild degrees of abdominal muscular deficiency may show improvement in the abdominal wall laxity as they mature. However, most others with moderate to severe degrees of abdominal wall laxity are left with a potentially physically and psychologically crippling defect (Ehrlich et al. An elasticized corset can improve the external appearance when the patients are fully clothed as well as improve the respiratory and gastrointestinal problems, even in the neonatal period (Satar et al. Nevertheless, these corsets may become inconvenient to use in the long term, particularly in regions with warm climate. There is general agreement about the cosmetic benefit of abdominal wall reconstruction. However, whether it improves bladder, bowel, and pulmonary function is controversial (Smith et al. Potential effects include a more effective cough and improvement Chapter 32 in defecation. The timing of abdominal wall reconstruction should be dictated by the need for other surgical interventions, particularly if upper urinary tract remodeling is necessary. If upper tract remodeling is not anticipated, the abdominal wall can be addressed at any time and has been done as young as 6 months of age along with transabdominal orchidopexy (Smith and Woodard, 2002). However, if the procedure is done in infancy, the infant may have to be placed on a respirator postoperatively for a period of time. This technique makes a transverse incision from the 12th rib to the pubic symphysis to the opposite 12th rib with a full-thickness removal of the skin, lower abdominal musculature, and peritoneum. The healthy fascia is then approximated to the anterior iliac spines, pubic tubercle, and inferior fascia. Although this technique is successful in establishing a waistline, lateral abdominal bulging often persists. Of the 16 patients reported, 9 had excellent cosmetic results and there was some residual protuberance in 7 (Fallat et al. The technique described by Ehrlich used a vertical midline incision and allows preservation of the umbilicus on a vascular pedicle from the inferior epigastric artery (Ehrlich et al. The skin and subcutaneous tissues are elevated off the muscle and fascial layers, and an overlapping, vest-over-pants advancement of each side to the contralateral flank is performed, preserving the less affected lateral muscles and fascia. The skin and subcutaneous tissue are dissected off the attenuated fascia and muscle with the dissection extending laterally to the anterior axillary line. Vertical fascial incisions are made lateral to the superior epigastric arteries, leaving a central fascial bridge. If intra-abdominal surgery is necessary, excellent exposure to the urinary tract or abdominal testes is afforded through these lateral fascial incisions. Recently incorporated modifications, such as the use of diagnostic laparoscopy to define the topography of the abdominal wall defect, initial midline rather than elliptical skin incision to defer retailoring of the skin coverage until the final step of the procedure, varying the width of the central plate to correct side-to-side asymmetry in redundancy, plication of the central plate to reduce vertical redundancy and allow reposition of the umbilicus, and plication of focal areas of fascial weakness in the flank region, have improved the results of this abdominoplasty technique (Smith et al. Another modification of the Ehrlich and Monfort techniques was reported in which, after the fusiform longitudinal resection of the mid-abdominal skin and subcutaneous tissue with preservation of the musculo-aponeurotic fascia and umbilicus, one elliptical xyphopubic incision is made in the more flaccid side of the abdominal fascia, producing one wide and one narrow fascial flap, with the umbilicus kept intact in the wide flap. Closure is made by suturing the wide flap laterally to the inner side of the narrow flap, followed by vest-over-pants suture of this flap over the wide, now "inner" flap, with a buttonhole exposing the umbilicus, which is then fixed in place. Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation (Lopes et al. A modification of the Monfort technique uses laparoscopy to protect the abdominal contents (Franco, 2005). The patients have compromised physical, emotional, social, and school functioning, and the caregivers report an overall lower quality of life because of the burdens that families with chronically ill children often face (Arlen et al. Renal Function the nadir creatinine value during infancy has proven to be a useful predictor of long-term renal function. Reconstruction of the upper and lower urinary tract has been proven to maintain stabilized renal function in most patients (Lopes et al. Unfortunately, the risk of infection is constant in the setting of persistent urinary tract dilation and stasis. Furthermore, kidney dysplasia and polyuria may also play a deleterious role in the long-term renal function (Lopes et al. Long-term surveillance of the urinary tract is essential because functional dynamics can change over time. As consequence, some patients tend to retain large volumes in the bladder, which develops progressive capacity associated to significant postvoid residual (Snyder et al. The lack of abdominal support, the eventual association of polyuria, and the negative behavior of some patients in regard to timed voiding usually increase this tendency (Kinahan et al. Surgical technique for Monfort abdominoplasty and concomitant reconstruction of prune-belly uropathy. The bladder is closed in two layers, and ureteral stents (not shown) and a cystostomy tube are used. Skin is brought together in the midline, enveloping the previously isolated umbilicus. In the case of progressive unbalanced voiding, if such a program is not feasible or ineffective, intermittent bladder catheterization is mandatory, either per urethra or through a Mitrofanoff channel. Eventually, effective voiding is achieved with growth and improved proprioceptive sensation, as well as increased abdominal support. Rarely, some of these children reacquire normal voiding with good bladder emptying.

El Khader K blood pressure medications that start with l purchase cheapest altace, Ibn Attya A blood pressure below 100 generic altace 1.25 mg on line, Mamoun M hypertension vascular disease 5 mg altace visa, et al: Role of ultrasound lithotripsy in the treatment of lithiasis of the lower urinary tract pulse pressure 38 generic 5 mg altace amex. Huang Z hypertension grades order altace with american express, Fu F hypertension jnc 8 pdf buy altace 1.25mg without prescription, Zhong Z, et al: Flexible ureteroscopy and laser lithotripsy for bilateral multiple intrarenal stones: is this a valuable choice Kumar A, Nanda B, Kumar N, et al: A prospective randomized comparison between shockwave lithotripsy and semirigid ureteroscopy for upper ureteral stones <2 cm: a single center experience, J Endourol 2013. Miyazaki H, Hirano Y, Kato S, et al: Early experiences of contact laser vaporization of the prostate using the 980 nm high power diode laser for benign prostatic hyperplasia, Low Urin Tract Symptoms 2017. Philippou P, Volanis D, Kariotis I, et al: Prospective comparative study of endoscopic management of bladder lithiasis: is prostate surgery a necessary adjunct Yet it is only in the modern era that we have developed the technology to detect microscopic blood, the means to identify the source of hematuria, and the understanding of anatomy, physiology, and disease processes underlying this important sign. Today, hematuria is one of the most common indications for urologic evaluation (Mariani et al. Therefore knowledge of the differential diagnosis, principles of evaluation, and strategies for management of hematuria is critical. This characterization may give some indication of the source of hematuria, with initial hematuria most commonly emanating from a urethral source; terminal hematuria from the bladder trigone, bladder neck, or prostate; and total hematuria from the bladder or above (Sokolosky, 2001). Notably, myoglobinuria and other factors can cause false-positive chemical tests. After ruling out vaginal bleeding and mimics of hematuria, a urologic source must be suspected. In fact, a meta-analysis reported that the rate of malignancy detected among patients evaluated for a single positive urinalysis was 3. Requirement for Microscopic Evaluation the results of urine dipstick tests must be confirmed on urinalysis with microscopy and alone are considered insufficient to prompt an evaluation according to most authorities (Davis et al. Indeed, chemical tests for hematuria detect the peroxidase activity of hemoglobin using benzidine, and therefore conditions such as myoglobinuria can falsely activate the test (Mariani et al. If the urinalysis with microscopy is not confirmatory, but the clinician remains suspicious, repeat microscopic testing is reasonable with the frequency individualized based on provider judgment. Specimens collected immediately after prolonged recumbency (first void in morning) or after vigorous physical or sexual activity may be falsely positive for hematuria (Addis, 1926; Kincaid-Smith, 1982). The proper collection of a urine specimen and the details of urine dipstick testing and urinalysis are covered in Chapter 1. Unfortunately, uniform agreement does not exist on how to identify benign causes of hematuria. Presence of hematologic illness, medical renal illness or use of anticoagulants or antiplatelet agents does not preclude the need for a hematuria evaluation. In addition, patients who develop hematuria (microscopic or gross) who are taking anticoagulation or antiplatelet medications. In fact, it has been noted that these medications may unmask genitourinary lesions at an earlier stage (Antolak and Mellinger, 1969; Kraus et al. More recently, a population-based study in Ontario, Canada, showed that patients taking antithrombotic agents were more than twice as likely to be diagnosed with bladder cancer within 6 months after an episode of hematuria (Wallis et al. Specifically, the goal should be to identify causes that would warrant variation from the standard evaluation, such as infection, menstruation, recent vigorous exercise, known medical renal disease, acute viral illness, trauma, and the presence of foreign bodies in the urinary tract or recent urologic instrumentation. Current medication use, including anticoagulants and antiplatelet therapies, should be elicited, along with recent coagulation values and any concomitant medications that would potentiate the effects of blood thinners. Family history of nephritis, polycystic kidneys, and rare familial tumor syndromes of the kidney. In addition, the possibility of finding a tobacco-related illness, such as bladder cancer, makes this a potential "teachable moment" for tobacco users (Bassett et al. Thus smoking cessation counseling should be a standard component of the hematuria evaluation discussion for current smokers. Laboratory testing includes urinalysis (if not performed previously) to confirm the presence of hematuria and check for dysmorphic red cells, cellular casts, or proteinuria; urine culture if the urinalysis or clinical presentation suggests infection; renal function testing (serum creatinine) to determine whether concomitant nephrologic evaluation is indicated and to guide the selection of appropriate upper tract imaging; and prostate-specific antigen in the appropriate setting. If a benign cause of hematuria is discovered during the initial history and physical examination. Moreover, if a medical renal cause of hematuria is suspected based on the presence of renal insufficiency, hypertension, or abnormalities on urinalysis, nephrology evaluation is recommended, but the patient should still undergo urologic evaluation. Nevertheless, it is important to recognize that, despite the potential benefits of such risk-based approaches, analyses of contemporary practice patterns have noted that fewer than 25% of patients found to have hematuria are even referred for evaluation, and fewer than 10% undergo a complete evaluation with cystoscopy and imaging, including among patients at high risk for malignancy (Ark et al. Therefore continued research and education are necessary and may in fact be facilitated by the implementation of such systems as decision support tools in the electronic medical record, telemedicine consultation, and one-stop hematuria clinics (Liedberg et al. The Question of Screening for Hematuria and Bladder Cancer Bladder cancer is the sixth most commonly diagnosed cancer in the United States, and although no large-scale screening trials have been performed, most believe that the harms and costs of mass screening for bladder cancer would prove to outweigh the potential benefits (Chou and Dana, 2010; National Cancer Institute, 2018). Nonetheless, many primary care providers perform urinalysis as part of routine health examinations, creating numerous opportunistic screening events (Prochazka et al. The risk of malignancy diagnosis is greater in patients with higher degrees of hematuria, male gender, and/or risk factors for malignancy and lower in absence of these risk factors. Cystoscopy in the Diagnostic Evaluation of Hematuria Cystoscopy is a key component of the hematuria evaluation because it is the most reliable way to evaluate the bladder for the presence of bladder cancer and provides the opportunity to evaluate the urethra. Bladder cancer is rare (<1 per 100,000) among persons 35 years old or younger (National Cancer Institute, 2018; van der Molen and Hovius, 2012). Repeat anatomic evaluation within 3 to 5 years* or sooner, if clinically indicated. American Urological Association guideline algorithm for evaluation of adult patients with asymptomatic microhematuria. Again, other organizational guidelines differ slightly in criteria for cystoscopy (see Table 16. Potential risks of the procedure include discomfort, injury to the urethra, infection, and the need for additional procedures such as biopsy. Nevertheless, such an approach is not risk stratified and importantly has not been prospectively tested. Nevertheless, these studies contained large proportions of younger patients, many of whom did not undergo a complete workup at any time, raising the possibility of persistent occult urologic disease. The first two can be summarized as recommending following up annual urinalysis for 2 years after a complete negative hematuria workup and releasing the patient from care if the urinalyses confirm resolution of hematuria. Urine Cytology and Urinary Biomarkers in the Diagnostic Evaluation of Hematuria Urine cytologic examination is highly sensitive and specific for the detection of high-grade urothelial carcinoma, but sensitivity decreases significantly for low-grade urothelial carcinoma, resulting in an overall sensitivity of 15. Indeed, in a large study of patients with hematuria, the sensitivity and specificity of positive/suspicious/atypical cytology were 45. Here, eight bladder tumors were identified, of which seven were identified by the ImmunoCyt test, for a sensitivity of 87%. However, studies in the urothelial carcinoma follow-up setting have found a far more modest sensitivity (68. In a population of patients with hematuria (gross and microscopic) 50 years of age or older without diagnosis of bladder cancer, the sensitivity and specificity of this test were noted to be 87. In the setting of a symptomatic, culture-documented urinary infection presenting with hematuria, with documentation of hematuria resolution after treatment, a complete workup can be avoided. Specifically, cystoscopy is recommended in such patients regardless of age (Davis et al. Moreover, although routine cytology is not recommended as part of the routine evaluation for the asymptomatic patient with microscopic hematuria, cytologic examination is considered an option in the setting of irritative voiding symptoms, although cystoscopy should not be omitted even if the cytologic findings are negative (Davis et al. However, as the degree of hematuria increases, so Chapter 16 does the likelihood of finding clinically significant lesions during evaluation (Mariani, 1989). After initial stabilization, diagnostic evaluation should then proceed, with cause-specific management as outlined below. Unfortunately, patients in this situation are often elderly and infirm, with medical comorbidities that complicate plans for care. Hemorrhagic cystitis is characterized by diffuse inflammation and bleeding from the bladder mucosa (Rastinehad et al. Meanwhile, viral-induced hemorrhagic cystitis may affect children and immunosuppressed adults particularly. Treatment for viral hemorrhagic cystitis is primarily supportive, with hydration, diuresis, and bladder irrigation, although case reports of success with antiviral therapy exist (Rastinehad et al. Hemorrhagic cystitis also may result from exposure to the oxazaphosphorine class of chemotherapeutic agents, specifically cyclophosphamide and ifosfamide. Indeed, hemorrhagic cystitis has been reported to occur in 2% to 40% of patients treated with cyclophosphamide (Rastinehad et al. The onset of hematuria is typically within 48 hours of treatment (Cox, 1979; Stillwell and Benson, 1988). Nevertheless, 10% to 40% of patients will develop the condition despite preventive treatment (Shepherd et al. Meanwhile, radiation therapy for pelvic malignancy represents another predisposing factor to hemorrhagic cystitis. Indeed, moderate to severe hematuria has been reported in approximately 5% of patients after pelvic radiotherapy, depending in part on factors such as the radiation dosage and mode of delivery, with onset between 6 months and 10 years after treatment (Corman et al. Mechanistically, radiation damages the vascular endothelium, thereby inducing subsequent inflammation, fibrosis, and ischemia, with tissue necrosis and mucosal sloughing occurring through progressive obliterative endarteritis (Bevers et al. In the setting of such local vascular compromise, moreover, secondary infection frequently ensues, further compromising tissue healing (Del Pizzo et al. Importantly, receipt of both cyclophosphamide chemotherapy and pelvic radiation (Wallis et al.

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