Zoran Krstic

Impact of micturating cystourethrography and DMSA renal scintigraphy on the investigation scheme in children with urinary tract infection

ObjectiveThe relationships among urinary tract infection (UTI), vesicoureteral reflux (VUR), and permanent renal damage in children are not fully understood. The aim of this study was to evaluate the incidence of renal scarring in children with a history of UTI and to determine the change in the probability of permanent renal damage owing to the presence of VUR documented on micturating cystourethrography (MCU).MethodsWe analyzed 201 children (400 renal units, two children with solitary kidney). Seventy-four boys and 127 girls (aged 7 months to 7 years, median 2.5 years) with culture verified UTI were referred for technetium-labeled dimercaptosuccinic (DMSA) renal scintigraphy 4-6 months after acute UTI. MCU was also performed mostly 1 month prior to DMSA. Statistical analysis was performed using χ2 test or Fisher’s exact test. Likelihood ratio (LR) positive and negative, diagnostic odds ratio (DOR), and post-test probability of (no) disease were calculated for VUR on MCU versus scarring on DMSA.ResultsVesicoureteral reflux was found in 158 (39.5%) kidneys, and evaluated as grades I, II, III, IV, and V in 3, 70, 43, 25, and 17 refluxing renal units (RRU), respectively. Permanent renal damage according to DMSA was seen in 15.2% (61/400) kidneys. Scarring was shown in 29.7% (47/158) of kidneys with VUR and in 5.8% (14/242) kidneys without VUR (P < 0.0001). LR positive was 2.353 (95% CI 1.889, 2.865), LR negative 0.341 (95% CI 0.209, 0.523), and DOR 6.895 (95% CI 3.533, 14.093). Rate of scarring significantly increased with VUR of grades III, IV, and V. There was no significant difference in the incidence of scarring in kidneys without VUR and RRU with low-grade (I and II) VUR (P = 0.306). The presence of VUR on MCU increased the chance of renal damage on DMSA by about 15%, whereas negative MCU increased the chance of no-renal involvement by 9%.ConclusionsMicturating cystourethrography should not be used as a first-line test to rule out the permanent renal damage owing to UTI. The priority of imaging strategy should be focused on early identification of renal lesions to prevent further deterioration.

True hermaphroditism: 10 years' experience

Abstract True hermaphroditism (TH) is the rarest variant of intersex malformations. By definition, these patients have both ovarian and testicular tissue. Most present due to ambiguous genitalia and/or gynecomastia. From 1986 to 1996, we treated 97 patients with intersex disorders; there were 4 with TH. In all cases testicular and ovarian tissue was separate. In 3 patients the external genitalia were ambiguous and 1 had hypoplastic male genitalia. Three patients had a 46, XY and 1 a 46, XX karyotype. Three patients had been listed as males and 1 as a female. The number of operations required varied from 3 to 9 (mean 5.7 per patient). Two patients who had been raised as males finished puberty with hypogenitalism and hypoplastic testes. One married and could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a female after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that was deepened to 12 cm with bougienage, so that she was capable of having normal intercourse. It is felt at present that most patients with TH should be raised as females. This was confirmed in our patients as well, which underlines the significance of early diagnosis.

Surgical treatment of intersex disorders

Despite the progress made in understanding the factors regulating sexual differentiation, infants born with ambiguous genitalia face significant problems. The authors reviewed a group of 84 children with ambiguous genitalia managed surgically between 1986 and 1993. The most frequent condition was male pseudohermaphroditism (PM) (58%); 31% had female pseudohermaphroditism. Fifty-seven percent of patients were raised as males and 43% as females. In each group of patients, feminine and masculine reconstructive operations were performed. In only 31% of PM and 60% of PF cases was the diagnosis made within the first 2 months of life. In 41% of PF and 40% of PM patients, treatment was begun before the second year of life, which we consider an acceptable time. The timing and type of vaginoplasty were determined by the point of entry of the vagina into the urogenital sinus. Of the 29 patients reared as females, 22 required perineal vaginoplasty, had pull-through vaginoplasty, and 2 had colovaginoplasty. Since 1986, we have applied Mollards clitoroplasty, which preserves the neurovascular bundle and is important for experiencing orgasm. Seventeen percent of patients with feminization procedures experienced complications. The optimal time for masculinization procedures is 2 years of age, after obligatory testosterone treatment. If there is utriculus prostaticus (UP) type II or III, it is removed before urethroplasty. This is not done for UP types 0 and 1. In PM cases, the number of feminization and masculinization operations was 2.1 and 4.05 per patient, respectively. It is easier to make a vagina than a phallus, not taking into consideration dimensions, aesthetics, or capability of erection of the phallus.(ABSTRACT TRUNCATED AT 250 WORDS)

Expression profiling of the AT2R mRNA in affected tissue from children with CAKUT

OBJECTIVES Congenital anomalies of the kidney and urinary tract (CAKUT) are common causes of chronic renal failure in children. The angiotensin II receptor type 2 (AT2R) is one of proposed candidate genes for CAKUT, but the expression was never explored in humans. The aim was to establish the AT2R gene expression in human CAKUT concerning -1332A/G polymorphism, which might affect alternative splicing. DESIGN AND METHODS Forty-eight patients with CAKUT constitute the basis of this study. Genotyping for -1332A/G, RT-PCR for AT2R gene expression and confirmation sequencing were performed. RESULTS The expression of Ex 1/2/3 and Ex 1/3 transcript splice variants of the AT2R mRNA were detected in human CAKUT tissue. The pattern was observed independently of A to G transition. CONCLUSIONS The expression of AT2R mRNA in human CAKUT was established for the first time and was not affected by -1332A/G polymorphism in children with CAKUT.

Severe renovascular hypertension in an infant with congenital solitary pelvic kidney

Renal artery stenosis (RAS) is one of the most common causes of severe arterial hypertension in infants. Its management is very difficult, especially when present in a single kidney. We report a case of severe hypertension caused by RAS of congenital single pelvic kidney in a 4-month-old boy. The patient presented with cardiorespiratory insufficiency that was first treated as acute fulminate myocarditis. Medical treatment of arterial hypertension was disappointing, as it had to be balanced between congestive cardiac failure and acute renal failure. Percutaneous transluminal angioplasty (PTA) done by coronary balloon dilatation catheters through the left axillary access was successful. Following dilatation of the renal artery, blood pressure decreased and its good control was possible by only one drug. With improved medical blood pressure control and normal growth development, the reassessment of clinical therapy options adjusted to a larger vessel size would be possible. Renovascular hypertension due to RAS in infants with a solitary kidney is difficult to control by medical treatment alone. PTA should be considered as a viable option in infants with refractory hypertension due to renal artery stenosis in a solitary kidney, since it has the potential of improving hypertension while preserving renal function.

Vestibular fistula: the operative dilemma — cutback, fistula transplantation or posterior sagittal anorectoplasty?

Vulvovestibular fistulas usually have a good prognosis. Most pediatric surgeons use operations such as cutback, fistula transposition without colostomy, and lately, posterior sagittal anorectoplasty with colostomy. Since 1978, the authors have been involved in the surgical treatment of 18 patients with vestibular fistulas without colostomy using a modified Santulli technique. Our results are satisfactory. We discuss voluntary bowel movements, soiling, constipation, and cosmetic effects. Comparisons are made between the three techniques with a discussion of advantages and disadvantages. We personally prefer anal transposition without colostomy.

Surgical treatment of cloacal anomalies

Abstract From 1989–1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1–8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry.PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Objective Long-Term Evaluation after Bladder Autoaugmentation with Rectus Muscle Backing

PURPOSE Bladder autoaugmentation with rectus muscle backing is an efficient surgical technique for bladder augmentation. We evaluated long-term outcomes to determine the value of this procedure. MATERIALS AND METHODS Between August 1999 and June 2004 autoaugmentation was performed in 16 girls and 7 boys 4 to 13 years old (median age 8). The indication was neurogenic bladder with small capacity and poor compliance due to myelomeningocele in 18 patients, tethered cord in 3 and sacral agenesis in 2. Detrusorectomy usually involved the whole upper half of the bladder. The prolapsed bladder urothelium was hitched to the 2 rectus muscles to prevent retraction and provide easier bladder emptying with voluntary muscle contractions. RESULTS At the median early followup of 27 months (range 9 to 49) bladder volume had increased significantly in all 23 patients (median 338 ml, range 190 to 462). At the current median long-term followup of 134 months (range 94 to 159) bladder volume continued to be significant compared to median bladder capacity preoperatively (median 419 ml, range 296 to 552). Voluntary voiding was achieved in 14 patients without post-void residual urine. Nine patients used clean intermittent catheterization, of whom only 4 could not empty the bladder voluntarily and relied only on clean intermittent catheterization. CONCLUSIONS Detrusorectomy with a rectus muscle hitch and backing is a minimally invasive, completely extraperitoneal, simple and safe procedure. However, the technique is indicated only in select cases without anterior abdominal wall anomalies.

The use of recombinant activated factor VII during major surgery in a child with Kasabach-Merritt syndrome.

muscular blockade. Patient recovered without any complications. In our institute we do pyeloplasty also in prone position as posterior approach gives easy and quick access to the renal pedicle(1). Other advantages are small incision and less requirement of analgesia. The I-gel airway is a novel supraglottic device made up of thermoplastic elastomer (styrene ethylene butadiene styrene)(2). A supraglottic airway device without an inflatable cuff has potential advantages including easier insertion, minimal risk of tissue compression and stability after insertion. It has been widely used for gynecological and other surgeries(3). I-gel is similar to proseal laryngeal mask airway (PLMA) without inflatable cuff. The PLMA has been used for prone position surgery(4), so we used I-gel for prone position surgery because the noninflatable cuff is semirigid and cannot be folded over, thus diminishing the risk of airway obstruction. The tensile properties of the noninflatable cuff and stability after insertion make the Igel less displacement. Another advantage is nitrous oxide cannot diffuse into the cuff causing problems as with laryngeal mask airway. We did not use any prophylactic antisialogue for prevention of device displacement but it was tied with tape. In our case there were no leak, hypoxia and displacement and regurgitation during the prone position but the end tidal carbon dioxide was progressively increasing. Rising end tidal carbon dioxide has been reported previously with other supraglottic devices(5). At the middle of the surgery the surgeons were giving pressure over the back, so the peak pressure increased and tidal volume decreased for transient period but rest of the time ventilatory parameters were within the acceptable range. Although we did not check the position of the device with fiberoptic bronchoscope as low peak airway pressure and absence of signs of airway obstruction suggested that the device was correctly positioned. The problems of using I-gel for prone position surgery including displacement, if occur, repositioning, and securing the airway may be difficult. Other problems are regurgitation, light plane of anesthesia may produce soft tissue trauma to the airway and laryngospasm. We had informed the surgeon before starting the case and arranged everything for if any airway disaster occurs. Device displacement can be managed because the gravity facilitates both device insertion and facemask ventilation by pulling the tongue forward to create a large pharyngeal space for insertion. Igel has been compared with other supraglottic devices for ease of insertion training manikins and was found to be the best performing device tested (6). Aspiration should be less likely in the prone position as gravity draws any regurgitated fluid away from the lungs. We placed a suction catheter in the drain tube and abdomen was freely moving without any compression, both might have reduced the gastric regurgitation. But regurgitation has been reported even in supine position with adequately fasted patient(3). The gastric tube was left in situ in view of gastric drainage and guide to reinsertion in the event of displacement. Use of I-gel for prone position surgery has not been reported in the literatures. In conclusion I-gel can be used for prone position surgery; further studies are needed to enhance the patient safety. M. Senthil Kumar Ravinder Pandey Puneet Khanna Department of Anaesthesiology & Intensive Care Unit, All India Institute of Medical Sciences, New Delhi, India (email: josenthil@gmail.com)

Severe Penile Injuries in Children and Adolescents: Reconstruction Modalities and Outcomes

OBJECTIVE To review our experience with severe penile trauma, mechanism of injury, and their treatment modalities in 16 children younger than 18 years. Management of penile trauma poses diverse challenges to the reconstructive urologist, as injuries vary from abrasions to total emasculation. METHODS Analysis of 16 patients with severe penile injuries referred to us between 2002 and 2011 was undertaken. The median age at surgery was 13 years (range, 5-17). Etiology of penile trauma and choice of treatment were evaluated. The management included a wide variety of surgical techniques that were tailored to the individual patient. Results were analyzed to define etiology, that is, mechanism of penile injury and to estimate modalities of surgical management and postoperative outcomes. Also, postoperative questionnaire was used, which included questions on functioning and esthetical appearance of participating patients and overall satisfaction. RESULTS The causes of penile injury in these series were traffic accidents (2), iatrogenic trauma (5), self-amputation (1), electrocution (1), burns (3), dog bite (2), zipper injury (1), and mothers hair strangulation (1). The mean follow-up was 46 months (range, 14-122), and examinations were uneventful, except for 2 fistulae formation after neophallic urethral reconstruction. CONCLUSION The main goal of reconstructive surgery is to have a penis with normal appearance and functions. Severe penile injuries should be treated on a case-by-case basis using the most propitious techniques.