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M C Adams

Publications and source records attributed to M C Adams.

At least 19 recordsLinked to original sources
PubMed2006

Continent catheterizable channels and the timing of their complications.

PURPOSE: We reviewed our experience with continent catheterizable channels with interest in the timing of conduit related complications. MATERIALS AND METHODS: A retrospective review was performed of the outcome of continent catheterizable channels in all patients between 1998 and 2003 who had undergone construction of an antegrade continence enema and/or a Mitrofanoff procedure using appendix, small bowel or continent cutaneous vesicostomy. We performed a total of 117 such stomas in 37 male and 41 female patients 2.5 to 20 years old (mean age 8.9). For the antegrade continence enema we used appendix in 92% of cases, an ileal Yang-Monti tube in 6% and a cecal tube in 2%. For the continent catheterizable channel we used appendix in 43% of cases, a Yang-Monti tube in 38% and continent cutaneous vesicostomy in 19%. RESULTS: Continence was achieved in 98% of patients. Followup was 6 to 71 months (mean 28.4). There were 27 channel related complications (23%). Stomal stenosis occurred in 7 antegrade continence enema procedures (14%) within 1 to 10 months (mean 6.2) and in 9 continent bladder channels (13%), including 5 continent cutaneous vesicostomies, within 1 to 24 months (mean 9.4) after surgery. False passages occurred in 5 antegrade continence enema procedures (10%) within 1 to 13 months (mean 3.6) and in 4 continent catheterizable channels (6%) within 1 to 13 months (mean 6.5) after surgery. Of patients with stomal stenosis 50% were treated with surgical revision, while the remainder was successfully treated with dilation. Most false passages were managed by catheter drainage alone. Reasons for revision were contained perforation, colovesical fistula and inability to catheterize. Patient noncompliance appeared to have a role in stomal stenosis. CONCLUSIONS: Continent catheterizable stomas help patients achieve bowel and bladder continence. Stomal incontinence after reconstruction is rare. In our experience most stoma related complications occurred in the first year after reconstruction. Experience with more patients and longer followup will help determine whether such problems continue to accumulate with time or whether continent stomas function well with time, particularly after the initial period of healing.

Adolescent↗
PubMed2005

Management of the failed pyeloplasty: a contemporary review.

PURPOSE: We reviewed our experience with open dismembered pyeloplasty, with specific focus on the presentation and management of failed pyeloplasty in the pediatric population. MATERIALS AND METHODS: We performed a retrospective review of patients who had undergone open dismembered pyeloplasty between 1998 and 2003. All patients with less than 6 months of followup were excluded from analysis. The patients were followed postoperatively with serial ultrasounds, with renograms reserved for those patients with prolonged, persistent or worsening hydronephrosis, or recurrent symptoms during followup. RESULTS: A total of 105 pyeloplasties were performed in 103 patients (71 males and 32 females) 1 to 204 months old (mean 60), with an overall success rate of 93.3%. Followup ranged from 6 to 69 months (mean 23). The 7 patients with treatment failure were males 1 to 204 months old (mean 125), who presented most commonly with pain within 3 to 38 months (mean 13.1) after initial surgery. Subsequent ultrasound revealed worsening hydronephrosis, and renography in these patients showed a pattern consistent with obstruction. Five patients underwent initial balloon dilation, in which 1 was successful. In addition, 1 of these patients underwent an unsuccessful antegrade laser endopyelotomy. Six patients (86%) underwent open surgery, consisting of ureterocalicostomy in 3 and reoperative dismembered pyeloplasty in 3. Dense scarring was seen in all patients, 2 patients had a redundant pelvis causing kinking and 2 patients had unrecognized crossing vessels. Overall salvage rate was 100% with continued followup of 3 to 50 months (mean 18). CONCLUSIONS: Dismembered pyeloplasty was successful in the vast majority of patients. In our series failures occurred as late as 3 years postoperatively, although most presented within 12 months of followup. Excluding routine postoperative nuclear renography did not seem to affect our ability to identify these cases. Although anatomical features such as renal pelvic size may have a role, failure is most likely secondary to technical issues, including missed crossing vessels and dependency of the anastomosis. In this series failed pyeloplasties did not respond well to balloon dilation, likely due to scar formation. Our current practice is to manage failures by open surgery, although endoscopic management by an incision may be an option.

Adolescent↗
PubMed2004

Signal analysis of total internal reflection fluorescent speckle microscopy (TIR-FSM) and wide-field epi-fluorescence FSM of the actin cytoskeleton and focal adhesions in living cells.

Fluorescent speckle microscopy (FSM) uses low levels of fluorescent proteins to create fluorescent speckles on cytoskeletal polymers in high-resolution fluorescence images of living cells. The dynamics of speckles over time encode subunit turnover and motion of the cytoskeletal polymers. We sought to improve on current FSM technology by first expanding it to study the dynamics of a non-polymeric macromolecular assembly, using focal adhesions as a test case, and second, to exploit for FSM the high contrast afforded by total internal reflection fluorescence microscopy (TIR-FM). Here, we first demonstrate that low levels of expression of a green fluorescent protein (GFP) conjugate of the focal adhesion protein, vinculin, results in clusters of fluorescent vinculin speckles on the ventral cell surface, which by immunofluorescence labelling of total vinculin correspond to sparse labelling of dense focal adhesion structures. This demonstrates that the FSM principle can be applied to study focal adhesions. We then use both GFP-vinculin expression and microinjected fluorescently labelled purified actin to compare quantitatively the speckle signal in FSM images of focal adhesions and the actin cytoskeleton in living cells by TIR-FM and wide-field epifluorescence microscopy. We use quantitative FSM image analysis software to define two new parameters for analysing FSM signal features that we can extract automatically: speckle modulation and speckle detectability. Our analysis shows that TIR-FSM affords major improvements in these parameters compared with wide-field epifluorescence FSM. Finally, we find that use of a crippled eukaryotic expression promoter for driving low-level GFP-fusion protein expression is a useful tool for FSM imaging. When used in time-lapse mode, TIR-FSM of actin and GFP-conjugated focal adhesion proteins will allow quantification of molecular dynamics within interesting macromolecular assemblies at the ventral surface of living cells.

Actins↗
PubMed2003

An in vitro model for investigating intestinal adhesion of potential dairy propionibacteria probiotic strains using cell line C2BBe1.

AIMS: The purposes of this study were to screen the adhesion properties of dairy propionibacteria strains and evaluate whether C2BBe1 could be used in the screening of potential probiotic strains. METHODS AND RESULTS: Thirteen dairy propionibacteria strains and two control strains, Lactobacillus acidophilus MJLA1 and Bifidobacterium lactis BDBB2, were tested for adhesion to C2BBe1. Electron microscopic observations demonstrated that the control strains, L. acidophilus MJLA1 and B. lactis BDBB2, had similar adhesive ability to C2BBe1 as had been previously shown to Caco-2. Only one of the 13 strains of dairy propionibacteria, strain P. jensenii 702, demonstrated adhesion to C2BBe1. CONCLUSIONS: C2BBe1 can provide an alternative to Caco-2 for assessing in vitro adhesion properties of probiotic strains. Adhesion properties of dairy propionibacteria were strain-dependent. SIGNIFICANCE AND IMPACT OF THE STUDY: C2BBe1 is highly suitable for application in bacterial adhesion studies, and was used successfully to select a new potential probiotic.

Animals↗
PubMed2001

Early evolution of bladder emptying after myelomeningocele closure.

OBJECTIVES: To analyze bladder emptying in newborns with spina bifida after closure of the myelomeningocele defect. Manipulation of the spinal cord and nerve roots results in temporary changes in bladder function in many settings. METHODS: We reviewed 62 consecutive newborn patients who underwent closure of a myelomeningocele defect at our institution from January 1990 to December 1997. We examined the catheterized urine volumes obtained before and after closure, radiographic and urodynamic studies, and the subsequent need for intermittent catheterization. Poor bladder emptying was defined as most catheterized urine volumes greater than 10 mL (20% of expected bladder capacity). Adequate follow-up was available for 54 patients for a period of 4 to 96 months (mean 42). One patient with posterior urethral valves was excluded from the study, leaving 53 for evaluation. RESULTS: Of the 53 patients, 4 with high outflow resistance and dyssynergy never emptied the bladder adequately before or after closure. Of the remaining 49 patients, 6 (12%) emptied well immediately after the myelomeningocele repair. In the other 43 (88%), the bladder did not empty as well after closure. The mean volume obtained with catheterization after closure was 20 mL, significantly higher (P <0.001) than the mean volume obtained before repair (6.5 mL) or after resumption of better spontaneous emptying (4 mL). Intermittent catheterization done for poor emptying was required for an average duration of 11 days (range 2 to 42). Of the 43 patients with poor emptying immediately after closure, 32 (74%) required catheterization for 2 weeks or less; the remaining 11 (26%) required catheterization for 2 to 6 weeks. Despite the relatively early resumption of good emptying, numerous patients (19 of 49) required additional intervention (clean intermittent catheterization or vesicostomy) before attempts to toilet train. CONCLUSIONS: A clinical pattern similar to spinal shock does occur in most newborns after closure of a myelomeningocele defect. Resumption of near-complete emptying usually occurs less than 2 weeks after repair but may require up to 6 weeks.

Female↗
PubMed2001

Is postoperative cystography necessary after ureteral reimplantation?

OBJECTIVES: To evaluate the patients in our practice to determine whether postoperative cystography was useful in monitoring the outcome after ureteroneocystostomy. Surgical repair of vesicoureteral reflux is the treatment of choice after medical therapy failure. Radiographic evaluation by ultrasonography or voiding cystourethrography (VCUG) is commonly used postoperatively to evaluate for urinary tract obstruction or persistent reflux. However, imaging modalities are not without cost, both in monetary terms and in terms of radiation exposure and trauma to the child. METHODS: We reviewed the records of all patients who underwent ureteroneocystostomy without ureteral tapering at our institution between January 1, 1996 and December 31, 1999 for primary vesicoureteral reflux. These records were evaluated with respect to the type of surgical procedure, preoperative and postoperative clinical course, and radiographic studies performed. RESULTS: We performed reimplantation on 267 renal units in 153 patients. The surgical technique was the Cohen cross-trigonal in 120 renal units (45%), Glenn-Anderson ureteral advancement in 92 (35%), and modified Leadbetter-Politano ureteral advancement in 55 (20%). All patients underwent imaging with ultrasonography within 6 weeks of surgery. The follow-up ranged from 4 to 42 months (average 14.2). Between 3 and 8 months postoperatively, 61 patients underwent imaging with VCUG. We identified persistent reflux in six renal units. Four of six had marked improvement in their reflux. All the patients with persistent reflux were asymptomatic, including 2 patients who were no longer receiving antibiotics. Four patients developed febrile urinary tract infections postoperatively. Three of the four underwent imaging with VCUG after treatment; all three had no evidence of reflux. CONCLUSIONS: In our population, the addition of VCUG to the postoperative evaluation did not allow us to identify those patients at risk of febrile urinary tract infections. Patients in whom persistent reflux was identified were all asymptomatic. We continue to monitor patients with ultrasonography, but believe that VCUG often provides little benefit to these children.

Adolescent↗
PubMed2001

Congenital anomalies of the kidney and urinary tract--role of the loss of function mutation in the pluripotent angiotensin type 2 receptor gene.

PURPOSE: Recent studies of the human genome and genetic engineering experiments in mice revealed that congenital anomalies of the kidney and urinary tract commonly seen in newborns in various anatomical forms are polygenic disease, that is a disease caused by simultaneous defects in multiple genes. We discuss some possible genetic explanations of the classic theories of the formation of congenital kidney and urinary tract anomalies. MATERIALS AND METHODS: We reviewed classic and current theories regarding urinary tract development. Included in our review are recent results from our laboratory evaluating the genetic role of normal and abnormal urinary tract development. RESULTS: We observed a genetic abnormality that may explain many classic anatomical theories of congenital kidney and urinary tract anomalies. One of the genes involved in urinary tract ontogenesis is the angiotensin type 2 receptor gene, which is the "other" angiotensin receptor. While the type 1 receptor mediates essentially all known actions of angiotensin, including its hypertensive effect, relatively little is known about the angiotensin type 2 receptor. Careful dissection studies in mutant mouse embryos selectively lacking the angiotensin type 2 receptor gene revealed that this gene is pleiotropic, that is its defect causes not only ectopic ureteral budding from the wolffian duct, but also disturbance in other subsequent ontogenic events that are critical for the normal growth of the kidney and urinary tract. CONCLUSIONS: Many congenital anomalies of the kidney and urinary tract appear to share a common genetic cause. While these anomalies are caused by various genetic hits, abnormalities in the angiotensin type 2 receptor gene are often involved in this anomalous development. This review article offers a better understanding of the genetics involved in urinary tract development and ties some of the newly emerging genetic theories with classic anatomical theories.

Angiotensin II↗
PubMed2001

The use of radiography, urodynamic studies and cystoscopy in the evaluation of voiding dysfunction.

PURPOSE: Children with dysfunctional voiding disorder often undergo radiological, cystoscopic or urodynamic evaluation to identify an anatomical or organic cause. We determined the role of these studies in the evaluation, management and ultimate outcome of a large patient population with voiding dysfunction at a single institution. MATERIALS AND METHODS: We retrospectively evaluated the records of 1, 153 children with dysfunctional voiding disorder treated from 1990 to 1999. A thorough history and physical examination with specific emphasis on voiding patterns were done and urinalysis was performed in all cases. Ultrasound of the urinary system and excretory urography were done in 1,050 (91%) and 24 (2%) patients, respectively, while voiding cystourethrography was performed in 672 (58%), including 564 with a history of nonfebrile urinary tract infection. Cystoscopy and a formal urodynamic study were performed in 61 (5%) and 40 (3.5%) cases, respectively. RESULTS: Mean patient age at referral was 6 years (range 3 to 14). Of the children 74% were girls and 26% were boys. Physical examination of the abdomen, back, genitalia and neurological system was unremarkable in all cases. Ultrasound of the upper urinary system was normal in 1,018 patients (97%) and showed insignificant pyelectasis in 32 (3%). All 24 excretory urography studies were normal and voiding cystourethrography was normal in 470 of 672 cases (70%). Unilateral and bilateral low grade, and unilateral high grade reflux was present in 108, 19 and 3 patients, respectively. Urodynamic studies were performed in 40 children who did not respond to standard treatment. We noted detrusor instability in 16 patients, detrusor-sphincter dyssynergia in 6 and sensory abnormality in 3, while the study was completely normal in 10. Cystoscopy revealed normal findings in 17 cases, trabeculations in 21, inflammation in 20 and type 1 posterior urethral valves in 2. CONCLUSIONS: The incidence of upper tract changes and positive anatomical findings in children with voiding dysfunction is too low to justify routine radiological evaluation and cystoscopy. However, in those who present with a nonfebrile urinary tract infection there remains an important role for voiding cystourethrography. We do not recommend routine urodynamics in children with voiding disorder because this study does not change therapy or influence the final outcome. Thorough history and physical examination lead to the correct diagnosis and treatment in the majority of children. A focus on correcting faulty voiding behavior with the judicious administration of antibiotics and anticholinergic therapy leads to a favorable outcome in most cases.

Algorithms↗
PubMed2001

Small intestinal submucosa as a urethral coverage layer.

PURPOSE: Urethrocutaneous fistula is the most common complication of hypospadias surgery. Numerous techniques have been used to decrease the incidence of this complication and the use of biocompatible materials in surgery has expanded the options in difficult situations. We hypothesized that porcine small intestinal submucosa may be used as a coverage layer after urethral surgery. We evaluated the histological changes associated with small intestinal submucosa when used as a coverage layer over the urethra in a rabbit model. METHODS AND METHODS: We performed urethral surgery in 16 New Zealand White rabbits divided into 4 animals each in groups 1-sham operation with penile degloving only, 2-penile degloving and small intestinal submucosa patch placement, 3-urethrotomy without a patch and 4-urethrotomy with a small intestinal submucosa patch. The graft edges were marked with permanent suture at surgery for later identification. All rabbits were maintained for 6 weeks before sacrifice. The urethra of each animal was then serially sectioned and examined histologically. RESULTS: Histological examination of animals with an small intestinal submucosa patch revealed a foreign body tissue reaction with an infiltrate of histiocytes, giant cells and lymphocytes in the area of graft placement. There was no histological evidence of remaining small intestinal submucosa patch in any sections. The urethral mucosa healed normally in all cases in which it was disrupted. There was no evidence of acute or chronic inflammation in any group 1 or 2 nonsmall intestinal submucosa animals and none in the animals with a small intestinal submucosa graft in areas other than the former graft site. There were also no urethrocutaneous fistulas in any of the 8 rabbits that underwent urethrotomy. CONCLUSIONS: Small intestine submucosa provides an adequate coverage layer in the rabbit penis after urethrotomy. Histologically the foreign material did not alter normal healing of the urethral mucosa, although it did appear to cause an infiltration of histiocytes, giant cells and lymphocytes. Small intestinal submucosa has previously been studied as a scaffold on which tissue may be remodeled or may regenerate. Our study shows that small intestinal submucosa did not interfere with normal tissue healing in this animal model. When used as a urethral coverage layer, it appears to provide extra tissue between the urethra and skin. Small intestinal submucosa may potentially decrease the incidence of urethrocutaneous fistula after urethral surgery.

Animals↗
PubMed2001

Angiotensin type II receptor expression and ureteral budding.

PURPOSE: Deletion of the angiotensin type II receptor gene (Agtr2) in mice results in a spectrum of urinary tract anomalies similar to that in humans. The mechanism behind this anomalous development is poorly understood. We evaluated Agtr2 expression as it relates to normal and abnormal ureteral budding. MATERIALS AND METHODS: A total of 400 wild type mice were inspected at birth for gross evidence of a urinary tract anomaly. In addition, the urinary tracts of 30 wild type embryos were evaluated at 11.0/11.5 and 13.5 weeks of gestation. These embryos were examined for ureteral budding site via section and whole mount in situ hybridization with c-ret probe and Agtr2 expression via in situ hybridization with Agtr2 riboprobe. There were 740 newborn mice homozygous for the null mutation of Agtr2 also evaluated along with 55 angiotensin type II knockout embryos at the aforementioned gestational ages. RESULTS: All wild type newborn animals were grossly normal. Of the angiotensin type II knockout newborns 23 (3.1%) had gross abnormalities of the urinary tract at birth. The predominant finding was a duplicated collecting system associated with a hydronephrotic upper pole moiety. These duplicated collecting systems fulfilled the Meyer-Weigert law. Interestingly, 25 (59.5%) of the knockout embryos showed abnormal ureteral budding. However, in wild type embryos Agtr2 was expressed at this "ectopic" cranial site between the wolffian duct and metanephric mesenchyme. CONCLUSIONS: Although not the sole regulator, angiotensin type II receptor expression may have a role in the embryological development of the urinary tract by inhibiting aberrant ureteral budding. A defect in this inhibitory process appears to cause ectopic ureteral budding, and may subsequently lead to renal dysplasia and other congenital anomalies of the kidney and urinary tract.

Animals↗
PubMed2000

The role of hypercalciuria in a subgroup of dysfunctional voiding syndromes of childhood.

PURPOSE: Idiopathic hypercalciuria is believed to be the cause of a variety of urinary tract complaints in clinical pediatrics, including urinary frequency, urgency, and/or dysuria, often associated with gross or microscopic hematuria. In children noncalculous manifestations of idiopathic hypercalciuria are reportedly more common than urolithiasis. We determine the use of the calcium-to-creatinine ratio for the evaluation of different subsets of functional voiding disorders. MATERIALS AND METHODS: We retrospectively evaluated 288 patients with functional voiding disorders during the last 8 years. Patients presenting with isolated urinary tract infection were not included in the study. A thorough history with emphasis on voiding patterns was elicited and a routine urinalysis was performed in all patients. Patients were divided into 5 groups of 22 with total gross hematuria and dysfunctional voiding pattern, 102 with microhematuria and dysfunctional voiding pattern, 66 with isolated childhood daytime frequency, 45 with the isolated dysuria syndrome and 53 with combined childhood frequency-urgency-dysuria syndrome. The season at presentation was noted to determine a seasonal pattern. In 149 patients urine was evaluated for a spot calcium-to-creatinine ratio. RESULTS: Mean age of the 95 (33%) males and 193 (67%) females was 6.1 years (range 2 to 14) and mean duration of symptoms was 10 months (1 week to 10 years). The incidence of idiopathic hypercalciuria was 28% in cases with gross hematuria and dysfunctional voiding, 30% with microscopic hematuria and dysfunctional voiding, 21% with pure childhood daytime frequency, 22% with pure dysuria, and 28% with frequency, urgency and dysuria. The patients were treated predominantly with behavioral therapy, correction of faulty voiding habits, anticholinergics and minor diet modification in some. Mean followup of 6.5 months (range 1 month to 10 years) was available for 153 patients (53%). Resolution of symptoms along with marked improvement in voiding habits was noted in 136 (89%) patients, moderate improvement in 10 (6.5%) and persistent symptoms with minimal improvement in 7 (4.5%). Treatment with thiazides was used in only 6 (2%) patients who had intractable symptoms and a markedly elevated urine calcium-to-creatinine ratio of whom 5 responded favorably. CONCLUSIONS: Idiopathic hypercalciuria may have a significant role in cases of functional voiding disorders. It affects the different subsets of voiding disorders with remarkable consistency but the exact mechanism remains unknown. Although a significant number of patients with voiding dysfunction have an elevated calcium-to-creatinine ratio, the majority respond to standard behavioral therapy and pharmacotherapy in the form of anticholinergics, and treatment directed toward hypercalciuria is not required in most cases.

Adolescent↗
PubMed2000

How well do patients with exstrophy actually void?

PURPOSE: Achieving continence and preserving renal function are goals in the care of patients with bladder exstrophy. The Young-Dees-Leadbetter bladder neck reconstruction should ideally provide continence and normal voiding dynamics without the need for intermittent catheterization. We review our experience with bladder neck reconstruction in this population with emphasis on voiding dynamics among those patients doing well. MATERIALS AND METHODS: We retrospectively analyzed all patients with the exstrophy-epispadias complex seen at our institutions since 1985. We reviewed staged reconstruction in 53 patients, including 31 with classic bladder exstrophy, 4 with exstrophy variants and 18 with incontinent epispadias. Patients with additional neurogenic dysfunction were excluded from study. Subjective and objective data regarding voiding function and complications were collected. RESULTS: Complete reconstruction for continence was performed in 38 cases, of which 11 that required bladder augmentation with bladder neck reconstruction or who had a different primary continence procedure were excluded from study. The remaining 27 patients treated with the Young-Dees-Leadbetter bladder neck reconstruction had 2 or more years of followup (mean 5.9). Dry intervals of at least 2 hours were achieved by 18 patients and all were considered by parents to void well. Despite near or total subjective continence and "good" voiding, 13 of these 18 patients (72%) have clinical problems related to emptying, which include recurrent urinary tract infections in 10, epididymitis in 2 and bladder calculi in 4. Objective urodynamic parameters confirm poor voiding in most patients. CONCLUSIONS: Bladder neck reconstruction in patients with exstrophy can achieve continence without intermittent catheterization. In our experience patients who achieve these goals have an alarming frequency of clinical and urodynamic problems related to emptying. One must question the normalcy of the voiding pattern and price to achieve continence among patients with exstrophy.

Bladder Exstrophy↗
PubMed2000

Y-to-I wrap: use of the distal spongiosum for hypospadias repair.

PURPOSE: Rather than resecting the distal spongiosum lateral to the open urethra for hypospadias, we mobilized this tissue with the urethral plate away from the corpora cavernosa, subsequently wrapping it around various types of urethroplasty to prevent fistula formation. MATERIALS AND METHODS: The distal spongiosum was preserved and used for coverage in 25 hypospadias repairs in 1 year. We initially applied it in this manner when the distal spongiosum persisted as a pillar of healthy erectile tissue but later when the distal tissue appeared more fibrous in nature. The wrap was used to cover various types of urethroplasty, including advancement in 6 cases, tubularization in 10, flip-flap repair in 6 and an island onlay pedicle graft in 3. RESULTS: All patients have at least 1 year of followup. There has been no fistula formation or residual chordee. In 1 patient minor meatal retraction did not require a secondary procedure. Cosmetic results have been good. CONCLUSIONS: A distal wrap of corpus spongiosum may be used to avoid fistula formation without causing residual or recurrent curvature. It re-creates a nearly normal urethra in some cases.

Adolescent↗
PubMed2000

The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation.

PURPOSE: Spinal dysraphism is the most common cause of neurogenic bladder dysfunction in newborns. Urodynamic findings in these patients include uninhibited bladder contractions, bladder areflexia, decreased compliance and detrusor-sphincter dyssynergia. Early urodynamic studies are recommended for spina bifida to help identify bladder characteristics that may cause a risk of upper tract deterioration. We recently evaluated a new early type of intervention involving closure of the neural tube defect during gestation in 25 patients at our institution. We hypothesize that this procedure results in decreased exposure of the spinal cord to amniotic fluid, which may improve neurological function. To date we have evaluated 16 of the 25 patients with video urodynamics. We compared the results to those in the literature on patients with myelomeningocele and without prenatal intervention. MATERIALS AND METHODS: We performed urodynamic testing in 16 patients with a mean age of 6.5 months, including cystometrography, fluoroscopic evaluation of filling and voiding, pelvic floor electromyography and post-void residual urine measurement. In addition, we retrospectively reviewed renal ultrasound, voiding cystourethrography, catheterization need, number of urinary tract infections and medication in these cases. RESULTS: Uninhibited detrusor contractions and an areflexic bladder were identified in 6% and 43% of patients, respectively, while 19% had decreased compliance and 75% had leak point pressure greater than 40 cm. water. Mean bladder capacity was 40 cc and 31% of patients had much lower capacity than expected for age. Previous renal ultrasound and voiding cystourethrography showed evidence of upper tract dilatation and reflux in 2 cases, respectively. Intermittent catheterization and anticholinergic therapy were required by 1 patient each and 1 had a significant urinary tract infection. CONCLUSIONS: Urodynamic findings in this population are comparable to those previously reported in the literature in patients with spina bifida without prenatal closure of the spinal defect. The lower incidence of urinary tract infection and reflux in our study probably represents more aggressive early urological management rather than neurological improvement. These urodynamic studies were performed early in life and future evaluation may ultimately reveal improved bladder function compared with that in others with myelodysplasia. However, at this time we recommend that patients who undergo spinal cord defect closure during gestation be evaluated and treated in the same manner as those with myelomeningocele but without fetal intervention.

Child↗
PubMed1999

Congenital dorsal penile curvature: a potential problem of the long phallus.

PURPOSE: We report the results of surgical correction of severe congenital dorsal penile curvature associated with a long slender phallus. MATERIALS AND METHODS: In the last 7 years we treated 16 boys with a mean age of 2 years 10 months who had severe dorsal penile curvature. The series included 5 patients with megameatus variant hypospadias and a full foreskin, 3 who presented with hypospadias and 8 who were referred when dorsal penile curvature was noticed by the family or primary physician. Mean penile length was 6.5 cm. without stretching. In each case length was greater than 2 standard deviations above the mean for patient age. Penile circumference decreased from a mean of 4.2 cm. at the base of the shaft to 4.1 cm. at the mid shaft and 3.7 cm. at the corona. Penile circumference was generally normal for patient age until corrected for length, when it was revealed to be small. Surgical correction of severe curvature was performed in 14 patients, while 2 who presented as newborns had remarkable spontaneous improvement in the first year of life. In each case curvature was due to corporeal disproportion. RESULTS: All patients have erections postoperatively. There has been no residual or recurrent curvature and cosmetic results are good. CONCLUSIONS: Congenital dorsal penile curvature is a potential problem of the long phallus. In severe cases surgical repair improves the appearance of the penis but it must also address the problem of corporeal disproportion.

Child↗
PubMed1999

Concealed penis in childhood: a spectrum of etiology and treatment.

PURPOSE: Concealed penis is an uncommon condition due to poor skin fixation at the base of the penis, cicatricial scarring after penile surgery and excessive obesity. The condition varies in severity and several surgical options are available, such as excision of previous scarring, degloving the penile shaft, reconstructing the penile shaft skin with flaps, fixing the penile skin at the penopubic and penoscrotal angles, and removing excess suprapubic fat. MATERIALS AND METHODS: We reviewed the records of 43 patients treated for concealed penis from 1993 to 1998. We categorized the cases as type 1-congenital concealed penis, type 2-concealed penis due to scarring from previous surgery and type 3-complex cases involving excessive obesity. Cases were reviewed in regard to surgical techniques and outcomes. We identified 18 type 1, 18 type 2 and 7 type 3 cases. Mean age of type 1 patients at surgery was 12.4 months with 1 patient presented at age 7 years. None had previously undergone penile surgery. All patients underwent complete penile degloving. To reconstruct the penile shaft flaps or Z-plasties with penile skin were used in 12 patients and scrotal skin flaps were used in 2. In 12 patients the penile skin was fixed at the penoscrotal and penopubic angles to maintain penile length and in 2 excess fat was excised. Mean age of type 2 patients at surgery was 19.8 months. All had previously undergone surgery, including hypospadias in 1 and circumcision in 17. All patients underwent complete penile degloving and the cicatricial scar that trapped the penis was excised. Penile skin flaps and Z-plasties were used in 12 cases, scrotal skin flaps were used for reconstruction in 2 and skin grafting was done in 1. In 10 patients the penile skin was fixed with sutures to maintain penile length. Mean age of type 3 patients at surgery was 15.8 years. Of the 7 boys 6 had previously undergone penile surgery. All required extensive scar excision and complex reconstruction involving penile skin flaps in 3, scrotal flaps in 5 and penile skin fixation in 6. Excessive suprapubic fat was removed in 5 patients, of whom 3 underwent liposuction. RESULTS: Surgical results were uniformly good in type 1 patients except in 1 who was believed to have excessive suprapubic fat. Results were good in 14 of the 18 type 2 patients, although 2 retained excessive suprapubic fat and 2 had some unsightly scarring. No type 1 or 2 patient required additional surgery. Of the 7 type 3 patients 6 had a good result and required no additional surgery. One patient has recurrent concealed penis after 2 procedures and awaits additional surgery. CONCLUSIONS: Concealed penis has a varied etiology and requires a flexible surgical approach. The common surgical options in all cases include complete penile degloving, excising the scarring due to previous surgery, removing excess suprapubic fat, reconstructing the penile skin with local flaps, and fixing the penile skin at the penopubic and penoscrotal angles.

Child↗
PubMed1999

Does every patient with prenatal hydronephrosis need voiding cystourethrography?

PURPOSE: Prenatal ultrasound has allowed early identification of urinary tract abnormalities that may require urological followup or early intervention. While all children with prenatal hydronephrosis should undergo ultrasound within the first few weeks of life, we believe that there is a subset of postnatal hydronephrosis for which voiding cystourethrography can be avoided if careful observation is continued. MATERIALS AND METHODS: For 5 years 175 infants with a history of prenatal hydronephrosis were evaluated by ultrasound. Of 60 infants with less than Society for Fetal Urology grade II unilateral or bilateral hydronephrosis 44 underwent voiding cystourethrography as part of the early evaluation and 16 were observed without voiding cystourethrography. Four infants for whom we would routinely obtain voiding cystourethrography were excluded from study due to severe prenatal hydronephrosis, renal duplication, hydroureter, ipsilateral small or echogenic kidney and grade II or higher hydronephrosis. RESULTS: Voiding cystourethrography was positive in 6 of the 40 infants (15%) with less than grade II hydronephrosis. Of these cases 3 had grade III or higher vesicoureteral reflux and 1 with high grade reflux required reimplantation. None of the 16 patients followed without voiding cystourethrography has required further evaluation or intervention. In all patients with negative or no voiding cystourethrography parenchyma was preserved and hydronephrosis stabilized or resolved. CONCLUSIONS: Prenatal and postnatal ultrasound in infants should be used to guide further urological evaluation. Among infants with less than grade II hydronephrosis postnatally 15% had reflux on voiding cystourethrography, which is significantly higher than that reported among normal children (approximately 1%). However, none of the 16 infants observed without voiding cystourethrography on short-term antibiotic prophylaxis had deleterious renal events with 6 months to 4.5 years of followup. Therefore, we question the actual significance of the reflux detected in the first cohort of infants. Voiding cystourethrography can provide a definitive answer. However, we also believe that it is not absolutely mandatory based on the outcome in the observed group. With careful counseling and followup most patients with less than grade II hydronephrosis can be observed without urological sequela.

Female↗