TL;DR: Endoscopic incision is appropriate as an emergency treatment or when dealing with a completely intravesical ureterocele, and both endoscopic puncture and upper pole partial nephrectomy frequently require additional surgery at the bladder level.
Abstract: Ureterocele is a cystic dilatation of the intravesical ureter that is most commonly observed in females and children, and usually affects the upper moiety of a complete pyeloureteral duplication. According to their position, ureteroceles are divided into intravesical, when the ureterocele is completely contained inside the bladder, and extravesical when part of the cyst extends to the urethra or bladder neck. Most ureteroceles are diagnosed in utero or immediately after birth during an echographic screening of renal malformations. Severe, febrile urinary tract infection is the most common postnatal presentation of ureteroceles, but they may, rarely, prolapse and acutely obstruct the bladder outlet. Once an ureterocele is identified sonographically, a voiding cystourethrogram to detect vesicoureteral reflux (VUR) and a 99m-technetium dimercapto-succinic acid renal scan to evaluate the function of the different portions of the kidney are mandatory. VUR in the lower pole is observed in 50% of cases and in the contralateral kidney in 25%. Simple endoscopic puncture of the ureterocele has recently been advocated as an emergency therapy for infected or obstructing ureteroceles and as an elective therapy for intravesical ureteroceles. The rate of additional surgery after elective endoscopic puncture of an orthotopic ureterocele ranges from 7 to 23%. Treatment of ectopic ureteroceles is more challenging and both endoscopic puncture and upper pole partial nephrectomy frequently require additional surgery at the bladder level. The reoperation rate after endoscopic treatment varies from 48 to 100%. It is 15 to 20% after upper pole partial nephrectomy if VUR was absent before the operation, but is as high as 50-100% when VUR was present. Thus, endoscopic incision is appropriate as an emergency treatment or when dealing with a completely intravesical ureterocele. Upper pole partial nephrectomy is the elective treatment for an ectopic ureterocele without preoperative VUR. In an ectopic ureterocele with VUR, no matter which type of primary therapy has been chosen, a secondary procedure at the bladder level, involving ureterocele removal and reimplantation of the ureter(s), should be anticipated.
TL;DR: A comprehensive literature review of various aspects of theVCUG is undertaken in order to determine the best way to perform the VCUG in modern paediatric radiology practice.
Abstract: The voiding cystourethrogram (VCUG) examination is a difficult investigation to perform and is a stressful experience for patients and their parents, as well as for the radiologists, technicians and paediatric radiology nurses involved in the examination. Despite the VCUG being one of the most commonly performed fluoroscopic procedures in paediatric radiology practice, there is no general consensus as to the best way to perform this investigation. This is particularly concerning when one considers the potentially high gonadal radiation dose children may receive. Because of this, we have undertaken a comprehensive literature review of various aspects of the test in order to determine the best way to perform the VCUG in modern paediatric radiology practice.
TL;DR: The goal of all therapeutic strategies in the management of newborn hydronephrosis is to select all infants with severe obstructive dilatation during serial follow-ups and to perform surgical repair before irreversible deterioration and functional renal damage occurs.
Abstract: The incidence of asymptomatic hydronephrosis in newborns is high. Despite extensive clinical and scientific research much controversy still exists about the assessment and management of hydronephrosis during infancy. Postnatal management demands a detailed prenatal history. Initial workup in newborns with unilateral hydronephrosis starts with a physical examination and sonography of the urinary tract on day 3-5. Grades 3 and 4 hydronephrosis are further investigated with a voiding cystourethrogram (VCUG) and diuretic renogram between weeks 4 and 6. An infant with an asymptomatic unilateral hydronephrosis of any grade, without urinary infection and stable washout, and stable normal differential function on serial controls can be managed conservatively. The goal of all therapeutic strategies in the management of newborn hydronephrosis is to select all infants with severe obstructive dilatation during serial follow-ups and to perform surgical repair before irreversible deterioration and functional renal damage occurs. Interdisciplinary cooperation between experienced pediatric urologists, nephrologist and radiologists is the basis for optimal decision making.
TL;DR: Renal cortical scintigraphic defects persisted in approximately one-quarter of young children after their first proven urinary tract infection and failed to predict scar formation, suggesting prevention of urinary tract infections from birth avoids post-natal scar acquisition or extension.
Abstract: Background: Renal cortical scintigraphic studies challenge the role of vesicoureteric reflux in renal scar development, emphasizing instead the part played by acute pyelonephritis. Objective: To determine the prevalence of renal cortical defects in a child cohort 2 years after the child’s first diagnosed urinary tract infection and to analyze the relationship of these defects with acute illness variables, primary vesicoureteric reflux and recurrent infections. Materials and methods: In a prospective cohort study, 193 children younger than 5 years with their first proven urinary tract infection underwent renal sonography, voiding cystourethrogram, and renal cortical scintigraphy within 15 days of diagnosis. Two years later, 150 of the 193 children, or 77.7%, had a further renal cortical scintigram, including 75, or 86.2%, of the 87 children who had acute scintigraphic defects. The relationship of cortical defects to age, gender, pre-treatment symptom duration, hospitalization, presence and grade of vesicoureteric reflux, and recurrent urinary tract infections was evaluated. Results: Overall, 20 of the 150 (13.3%; 95% confidence interval (CI) 8.3, 19.8) children had persistent defects 2 years after infection. This included 20 of 75 (26.7%; 95% CI 17.1, 38.1) with initially abnormal scintigrams. No new defects were detected. Although acute defects were more common in the young, those with persistent defects were older (median ages 16.4 vs. 6.8 months, P=0.004) than those with transient abnormalities. After adjustment for age, persistent defects were no longer associated with gender and were not predicted by acute illness variables, primary vesicoureteric reflux or recurrent infections. Conclusions: Renal cortical scintigraphic defects persisted in approximately one-quarter of young children after their first proven urinary tract infection. The associated clinical features, however, failed to predict scar formation. It is possible that some of the scintigraphic defects preceded the infection by arising from either previously undiagnosed acute pyelonephritis or from underlying congenital dysplasia. The etiology of scars may be best addressed by determining whether prevention of urinary tract infections from birth avoids post-natal scar acquisition or extension.
TL;DR: Renal transplantation is feasible with good results for children with abnormal LUT, however these children display a high incidence of urologic and infectious complications.
Abstract: Objective This retrospective study reports the outcomes as well as pre- and posttransplant urologic treatments of renal transplantation for children with an abnormal lower urinary tract (LUT). Methods Between March 1981 and December 2001, 195 children (≤18 years of age) received live-donor kidney transplants. The 15 recipients (14 boys and 1 girl, mean age 13.5 ± 3 years) who had lower urinary tract disorders included posterior urethral valves (PUV) with valve bladder ( n = 12) and neuropathic bladders secondary to meningomyelocele ( n = 3). These children were evaluated by voiding cystourethrogram, cystourethroscopy, and cystometry. The children with PUV were maintained on clean intermittent catheterization (CIC) and a detrusor relaxant at least 3 months before transplantation. Augmentation ileocystoplasty or continent cutaneous diversion were used in three patients. The graft and patient survivals as well as complications in this cohort was compared with a group of children with normal LUT, who underwent renal transplantation during the same period. Results One child died in the early posttransplant period due to rupture of the external iliac artery. Follow-up ranged from 6 months to 16 years (mean = 4.5 years). During the same period the graft and patient survival rates were comparable between the group of children with versus without abnormal LUT. Furthermore, mean serum creatinine and creatinine clearance values were also comparable. The group with an abnormal LUT showed a higher incidence of urinary fistula (3/14) and recurrent UTI and/or bacteremia (4/14). Conclusions Renal transplantation is feasible with good results for children with abnormal LUT. Pre- and posttransplant urologic management is critical for a successful outcome. However these children display a high incidence of urologic and infectious complications.
TL;DR: Fetal renal pelvic dilatation is a predictor of vesicoureteral reflux, but has low specificity, and fluctuation of the renal pelvis on sonography is a marker for persistent high-grade VUR and renal damage in girls and boys.
Abstract: Fetal renal pelvic dilatation is a predictor of vesicoureteral reflux (VUR), but has low specificity. The aim of this study was to determine the clinical significance of fluctuating size of the renal pelvis on sonography, particularly in relationship to VUR. We prospectively recorded fetal renal pelvic diameter >/=4 mm in 1,092 fetuses (692 boys), from May 1989 to December 1995, with a minimum follow-up of 7 years. Fluctuation, defined as size of renal pelvis changing by more than 4 mm during the course of obstetric (23), postnatal (128), and both pre- and postnatal (7) renal sonograms, was recorded prospectively in 159 (117 boys). Of the 1,092, 849 (593 boys) had a voiding cystourethrogram at a mean age of 7 weeks (range 3-20 weeks). Of the 31 (16 girls) with high-grade VUR, fluctuation was observed in 20 (9 girls). Fluctuation was strongly associated with high-grade VUR (odds ratio 11.1, P=0.0000003) and with renal damage (sensitivity 61%, positive predictive value 31%). Primary high-grade VUR was persistent, seen equally in boys and girls, and required surgery in the majority. Fluctuation was associated with renal duplication anomalies ( P=0.00009) and megaureter ( P<0.00000001). Fluctuation of the renal pelvis on sonography is a marker for persistent high-grade VUR and renal damage in girls and boys.
TL;DR: There was a trend for an increased risk in girls and those with recurrent febrile illnesses, and history of daytime dryness and/or constipation was not predictive of recurrent UTIs in this series.
TL;DR: A case of eosinophilic cystitis is reported in a boy who presented with severe irritative voiding symptoms and an infiltrative bladder lesion and was treated with 6 weeks of tapered prednisone and trimethoprim-sulfamethoxazole prophylaxis.
TL;DR: The endoscopic subureteral injection of polydimethylsiloxane in women with vesicoureteral reflux is an effective therapy with no associated morbidity.
TL;DR: Following an uncomplicated 38 weeks pregnancy, a normal labor, and delivery with Apgar scores 8 and 9 at 1 and 5 minutes, respectively, a newborn was delivered with a birth weight of 6 pounds 5 ounces and physical examination was unremarkable, except for complete absence of the penis.
Abstract: CASE. Following an uncomplicated 38 weeks pregnancy, a normal labor, and delivery with Apgar scores 8 and 9 at 1 and 5 minutes, respectively, a newborn was delivered with a birth weight of 6 pounds 5 ounces. The physical examination was unremarkable, except for complete absence of the penis. The scrotum appeared normal with bilateral palpable gonads of normal size. A voiding cystourethrogram demonstrated a normal bladder without uretero-vesical reflux; the contrast study revealed that urine partially emptied into the rectum and colon. The urethral meatus was positioned at the anterior anal verge. Karyotype was 46 XY. This is the third child for this couple. They have a 4-year-old boy and a 6-year-old girl. To which sex should this infant be assigned? Accompanying decisions concern disclosure of information to patient and family (what should be disclosed about the condition and its treatment and when?); surgery to have the genitalia match the sex assignment, or alternatively, female genital anatomy (what should be done and when?); psychological support of the patient and family (who should provide it and what model of care should be followed?); and involvement of other family members and friends (should they be told, and if so, what should they be told and when?).
TL;DR: A case of infectious perinephric urinoma in a 73‐year‐old woman who had a neurogenic bladder with vesico‐ureteral reflux is reported, the first case reported in an adult.
Abstract: We report a case of infectious perinephric urinoma in a 73-year-old woman who had a neurogenic bladder with vesico-ureteral reflux. The patient was admitted to our emergency room with right lumbago and high fever. Ultrasounds and computed tomography demonstrated a right large perinephric cystic mass, bilateral hydronephrosis and much residual urine. Percutaneous drainage of the cystic mass was performed with an indwelling urethral catheter. The content of the mass was urine infected with Escherichia coli. Antibiotic therapy was performed successfully and we then examined the cause of the urinoma. A urodynamic study demonstrated a low-compliance small bladder and detrusor-sphincter dyssynergia. A voiding cystourethrogram revealed right grade III vesicoureteral reflux. The patient was unable to be cleared with intermittent catheterization and had an indwelling urethral catheter inserted. In 1 year, the voiding cystourethrogram showed no vesicoureteral reflux and the patient was well with no evidence of recurrent urinoma without the urethral catheter. There have been only two reported cases of urinoma caused by neurogenic bladder with vesico-ureteral reflux in children and this is the first case reported in an adult.
TL;DR: It is concluded that VCUG should still be performed in hospitalized children with UTI, and the dilation of the renal pelvis did not predict all VUR precisely, especially severe VUR.
Abstract: BACKGROUND The aims of this study were to determine whether renal pelvis dilation on ultrasound was reliable in predicting vesicoureteral reflux (VUR) and to assess the relationship of other clinical information of VUR in children with urinary tract infection (UTI). METHODS We retrospectively reviewed clinical data, renal echo, and voiding cystourethrogram (VCUG) results in hospitalized children with their first episode of UTI, aged from 1 month to 5 years, during a 1-year period. RESULTS There were 114 children with 228 kidneys eligible for the study. Unilateral pelvis size greater than 8 mm had 2.4 (p = 0.049, 95% CI: 1.0-5.9) and 3.7 (p = 0.025, 95% CI: 1.2-11.3) times greater risk for VUR and severe reflux, respectively. The sensitivity in detecting severe reflux was 27.8%, and the specificity was 90.5%. The positive and negative predictive values in suggesting severe VUR were 20.0% and 93.6%, respectively. The sum of bilateral pelvis sizes greater than 16 mm had higher risk for VUR and severe reflux (4.1 and 4.6 times) and similar specificity and negative predictive value for severe reflux. Age, gender, C-reactive protein, leukocytosis, pyuria and acute pyelonephritis did not show significant relationships to the reflux. CONCLUSIONS Unilateral pelvis size greater than 8 mm or the sum of the bilateral pelvis sizes greater than 16 mm was associated with VUR, especially severe VUR. The possibility of severe reflux was lower than 10% when the reverse criteria were applied. However, the dilation of the renal pelvis did not predict all VUR precisely. We concluded that VCUG should still be performed in hospitalized children with UTI.
TL;DR: The anterior sagittal transrectal approach (ASTRA) was primarily developed for the treatment of anorectal malformations and was selected for fistula closure of a recurrent traumatic urethrovaginal fistula in girls after a mine explosion in Afghanistan.
TL;DR: It is suggested that UTI is an infrequent complication and that antibiotic prophylaxis cannot be considered the “standard of care” for VCUGs, and that only slightly more than half of pediatric nephrologists who responded to the question recommended prophYLaxis.
Abstract: Sirs, Most published data about the association of urinary catheterization and urinary tract infection (UTI) relate to long-term catheterization. In contrast, the risk of UTI from the one-time insertion of a catheter that is subsequently promptly removed appears to be low [1, 2], although at least one sepsis-related fatality is known to have occurred in a 6-week-old male following a voiding cystourethrogram (VCUG) (O. Mehls, personal communication). More recently, in a study of 123 adults who had undergone pressure flow studies, Logadottir et al. [3] reported that 4.1% had a positive urine culture and symptoms of infection 1 week after the procedure, and required antibiotic treatment. The authors concluded that routine antibiotic prophylaxis was not indicated after a study involving a brief catheterization of the bladder. This conclusion was supported by another study of 94 women who had undergone urodynamic testing involving two catheterizations. Those patients were randomly assigned to receive trimethoprim-sulfamethoxazole prophylaxis or a placebo. One week after the urodynamic testing, cleancatch urine samples showed UTI in 5.4% of the treated patients and 6.1% of the placebo patients (P=0.6) [4]. We were able to find only one study of the risk of UTI associated with VCUGs in children. In that retrospective study of 932 children who had undergone VCUGs, 18 patients (1.9%) developed a febrile UTI within 7 days of undergoing a VCUG. The risk was higher in boys, infants, patients with vesicoureteral reflux, and patients with other urinary tract abnormalities, but was not significantly lowered by antibiotic prophylaxis [5]. A few months ago, the occurrence of a UTI a few days after a VCUG in one of our patients prompted us to try to determine whether or not pediatric nephrologists routinely provide antibiotic prophylaxis to their patients who are scheduled to undergo a VCUG. To achieve this, we sent the following email to the subscribers of the PEDNEPH Internet Pediatric Nephrology List: UTI occurs in a small proportion of patients who undergo a VCUG. Do you prescribe prophylactic antibiotic treatment against that risk? Fifty-three responses were received. We included our own answers to the question for a total of 56 responses. Two respondents gave equivocal answers that were not included in the final count. One of those prescribed an antibiotic, after the procedure, only when the catheterization was difficult. The other prescribed prophylaxis for infants but not for older children. Of the remaining 54, 25 (46%) reported that they did not routinely provide prophylaxis and 29 (54%) reported that they did. There was a disparity in the practice of pediatric nephrologists working in the United States and those working in other countries. Of the 35 American respondents, 20 (57%) did not recommend prophylaxis and 15 (43%) did. Of the 19 non-American respondents, only 5 (26%) did not recommend prophylaxis, while 14 (74%) did. The difference between American and non-American nephrologists was statistically significant (chi-squared 4.71, P 0.05). In view of the absence of a clear-cut association between VCUG and post-VCUG UTI, after many years of performing the procedure, we suggest that UTI is an infrequent complication and that antibiotic prophylaxis cannot be considered the “standard of care.” This conclusion is supported by the fact that only slightly more than half of pediatric nephrologists who responded to our question recommended prophylaxis. What little evidence is available does not support antibiotic prophylaxis for VCUGs. B. Gauthier · M. Vergara · R. Frank · S. Vento · H. Trachtman Division of Nephrology, Schneider Children’s Hospital of the North Shore-Long Island Jewish Health System, Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, New York, USA
TL;DR: Anteroposterior and lateral X-ray images of the lumbosacral spine have been used to identify vertebral defects associated with nervous system anomalies such as sacral agenesis or spina bifida occulta.
TL;DR: Given that by fifteen months success rates were the same with either approach, the extravesical approach is comparable to the intravesical technique and is a viable option in terms of outcome and economics given the shorter length of hospital stay.
Abstract: Purpose: The purpose of our study was to examine outcomes and compare length of stay after extravesical and intravesical ureteral reimplantation at our institution. Materials and Methods: Retrospective review was performed of 30 patients (55 ureters) with vesicoureteral reflux who underwent either the Cohen (intravesical) cross-trigonal procedure or the extravesical (detrusorrhaphy) approach. Each patient had documented follow-up consisting of a postoperative renal ultrasound and/or a voiding cystourethrogram (VCUG). Inclusion criteria was the presence of primary vesicoureteral reflux. Exclusion criteria were patients who had undergone a previous repair and patients in whom results of neither the renal ultrasound nor the VCUG were available. Results: There were no significant cases of obstruction or wound infection with either approach. Two patients who underwent the extravesical approach had persistent reflux on VCUG three months postoperatively, but both resolved by fifteen months. Average length of stay was only 3.00 ± 1.33 days for the extravesical approach, compared to 5.36 ± 1.75 days for the intravesical approach ( P = .0003 ). Conclusions: Given that by fifteen months success rates were the same with either approach, the extravesical approach is comparable to the intravesical technique and is a viable option in terms of outcome and economics given the shorter length of hospital stay.
TL;DR: Echo-enhanced ultrasound with contrast medium in the bladder, performed by an experienced investigator, could replace the voiding cystourethrogram in follow-up studies of patients treated conservatively for vesicoureteric reflux.
Abstract: Purpose of review Newer techniques of molecular biology allow us to gain a better understanding of the molecular mechanism of primary urinary reflux. In recent years, diagnostic tools and treatment options for vesicoureteric reflux have dramatically changed. In this review, we want to focus on genetics, molecular biology, histology, clinical findings, diagnostic tools and newer surgical techniques for the treatment of vesicoureteric reflux published between 2003 and March 2004. Recent findings Recent findings have demonstrated that the renin-angiotensin-aldosterone system does not seem to be responsible for the development of primary vesicoureteric reflux. Echo-enhanced ultrasound with contrast medium in the bladder, performed by an experienced investigator, could replace the voiding cystourethrogram in follow-up studies of patients treated conservatively for vesicoureteric reflux. The sensitivity and specificity of ultrasound is in the range of 70-100% in some studies compared with the standard voiding cystourethrogram. Endoscopic treatment of vesicoureteric reflux using Defux or Macroplastique has gained more and more popularity, with success rates ranging between 60 and 86%. The surgery is performed on an outpatient basis with minimal morbidity. Long-term results after open operative techniques have demonstrated cure of reflux in over 95% of cases. Summary In the coming years, echo-enhanced ultrasound may replace the standard voiding cystourethrogram during follow-up and it may be used as an initial test in females with normal voiding patterns. Endoscopic reflux correction must be judged on the re-treatment rates and long-term success.
TL;DR: In this opinion, primary endoscopic ablation of the valves followed by a wait-and-see attitude is the most efficacious management of posterior urethral valves.
Abstract: Posterior urethral valve is a condition that leads to characteristic changes in the bladder and upper tracts. The bladder develops hypertrophic changes including muscular hypertrophy, dilatation of the prostatic urethra (keyhole appearance), and progressive hydroureteronephrosis. The voiding cystourethrogram confirms the diagnosis and documents vesicoureteral reflux and accompanying bladder changes. The follow-up of the serum creatinine level is a parameter for renal recovery. In our opinion, primary endoscopic ablation of the valves followed by a wait-and-see attitude is the most efficacious management of posterior urethral valves. The development of the bladder function is controlled by ultrasound and voiding cystourethrogram. Urodynamics provide a formal and objective means of assessing bladder function, but should be carefully applied in infants. Valve ablation in a neonate with significant reflux and a markedly trabeculated bladder can remodel itself remarkably within the 1st year of life. The persistence of hydronephrosis, bladder wall thickening and trabeculation, and persistent elevation of serum creatinine can all be harbingers that a degree of bladder outlet obstruction persists and one needs to rule out a persistent anatomic obstruction. At what point a functional obstruction occurs and which management is reasonable are still issues of debate and require the vigilance of a pediatric urologist to sort out. Dysfunctions of the bladder such as hyperreflexia, hypertonic, small capacity bladder, sphincter incompetence and/or myogenic failure should be adequately treated.
TL;DR: A 6-year-old girl was referred to the authors' department due to pyelonephritis and has persistent residual urine due to neurogenic voiding dysfunction, and is being treated with a regimen of frequent timed voiding to reduce urinary residual and urinary tract infection.
Abstract: A 6-year-old girl was referred to our department due to pyelonephritis. Voiding cystourethrogram (VCUG) revealed grade 4 vesicoureteral reflux (VUR) and urethral deformity (stenosis and lateral deviation). She had a history of sacrococcygeal teratoma resection in the newborn. Urodynamic study revealed a large-capacity hypotonic bladder and poor bladder emptying. Magnetic Resonance Imaging of the spine demonstrated no abnormal findings. Despite conservative therapy, there were no improvement of VUR. Then, urethral dilation and anti-reflux-surgery were performed. Six months after the operation, VCUG showed no VUR. However, she has persistent residual urine due to neurogenic voiding dysfunction, and is being treated with a regimen of frequent timed voiding to reduce urinary residual and urinary tract infection.
TL;DR: To determine the sensitivity, specificity, and predictive values of renal ultrasound findings for vesicouretral reflux (VUR) in febrile infant with urinary tract infection, retrospective review of ultrasound and voiding cystourethrogram results of 42 infants under 3 months of age admitted with their first episode of UTI over 18 months period is reviewed.
Abstract: Aims: To determine the sensitivity, specificity, and predictive values of renal ultrasound findings for vesicouretral reflux (VUR) in febrile infant with urinary tract infection. Patients and Methods: Retrospective review of the ultrasound and voiding cystourethrogram (VCUG) results of 42 infants under 3 months of age admitted with their first episode of urinary tract infection (UTI) over 18 months period. Ultrasound findings were considered suggestive of VUR if 'dilatation of the pelvi-calyces', 'dilatation of ureters" or "dilatation of the collecting system of one or both kidney" was reported. Results: A total of 49 patients were eligible for inclusion (medium age 28 days, 30 (71%) were male). Forty-two patients met all inclusion criteria. Prevalence of VUR on VCUCs was 62 % (26/42). Sensitivity of ultrasound for detection of VUR was 62 %, specificity 62 %, positive predictive value = 73, Negative predictive value = 50 %.
TL;DR: It is therefore imperative that genital examination for anomalies should be carried out in all children diagnosed with urinary tract infection and the presence of severe labial fusion may preclude bladder catheterization, which should be treated before a full urological work-up can be accomplished.
Abstract: Bladder catheterization for voiding cystourethrogram is a common paediatric procedure for the evaluation of urinary tract infection. Contraindications are rarely mentioned and are mostly related to the use of contrast or radiation. 1 The authors describe a case of failed examination in a young girl with severe labial fusion. The small labial aperture was mistaken as the urethral opening and the catheter ended up in the vaginal canal. The error was only recognized when the contrast examination revealed a female genital tract. A 21-month-old girl was to undergo a voiding cystourethrogram because of a recent history of urinary tract infection. The diagnosis was made 8 months earlier in another hospital from a suprapubic urine tap that grew Escherichia coli . Abnormality in the external genitalia had not been noted in the previous admission. When the patient was prepared for bladder catheterization, she voided spontaneously and urine was seen coming from a small ‘opening’. Thinking that this was the urethral meatus, the junior medical doctor inserted a size 5 Paediatric Feeding Tube (Pacific Hospital Supply Co., Ltd, Taiwan) through the opening. Afterwards, the patient was sent to the radiology department where the cystourethrogram was performed. It was then noticed that the vagina had been inadvertently catheterized and the genital tract was outlined (Fig. 1). When the patient was examined by the authors on the same day, she was found to have severe labial fusion all the way down to the posterior commissure. Only a small vulval opening was seen anteriorly just below the clitoris, from which urine could be seen coming out when the patient was asked to void. The underlying urethral opening could not be discerned. The child’s mother had no idea about the abnormality before the examination. The labial fusion resolved 4 weeks later after topical treatment with an oestrogen cream. An ultrasonography showed normal-sized kidneys without signs of obstruction. Labial fusion is a relatively common finding in young girls. In a prospective series of almost 2000 girls, it was found in 1.8% of subjects and occurred most often between 13 and 23 months of age. 2 As a female counterpart of phimosis, labial fusion is associated with vulvovaginitis and urinary tract infections. 3 Severe cases of urinary obstruction in relation to labial fusion have been described. A 6-month-old female presented with hydroureteronephrosis that resolved after the labial fusion was surgically treated. 4 Another 6-month-old girl presented with a palpably enlarged bladder in association with severe labial fusion. After the labial folds were separated, a moderately trabeculated bladder was seen on cystoscopy. 5 In both cases, catheterization of the bladder was considered impossible prior to the treatment of labial fusion. It is therefore imperative that genital examination for anomalies should be carried out in all children diagnosed with urinary tract infection. The presence of severe labial fusion may preclude bladder catheterization, which should be treated before a full urological work-up can be accomplished.
TL;DR: Following an uncomplicated 38-weeks pregnancy, a normal labor, and delivery with Apgar scores 8 and 9 at 1 and 5 minutes, respectively, a newborn was delivered with a birth weight of 6 pounds 5 ounces and physical examination was unremarkable.
Abstract: CASE. Following an uncomplicated 38 weeks pregnancy, a normal labor, and delivery with Apgar scores 8 and 9 at 1 and 5 minutes, respectively, a newborn was delivered with a birth weight of 6 pounds 5 ounces. The physical examination was unremarkable, except for complete absence of the penis. The scrotum appeared normal with bilateral palpable gonads of normal size. A voiding cystourethrogram demonstrated a normal bladder without uretero-vesical reflux; the contrast study revealed that urine partially emptied into the rectum and colon. The urethral meatus was positioned at the anterior anal verge. Karyotype was 46 XY. This is the third child for this couple. They have a 4-year-old boy and a 6-year-old girl. To which sex should this infant be assigned? Accompanying decisions concern disclosure of information to patient and family (what should be disclosed about the condition and its treatment and when?); surgery to have the genitalia match the sex assignment, or alternatively, female genital anatomy (what should be done and when?); psychological support of the patient and family (who should provide it and what model of care should be followed?); and involvement of other family members and friends (should they be told, and if so, what should they be told and when?).