Massimiliano Silveri

The changing urodynamic pattern from infancy to adolescence in boys with posterior urethral valves.

Objective To determine whether bladder dysfunction in boys with posterior urethral valves (PUV) changes from a uniform pattern of hypercontractility during infancy to the hypocontractility found in adolescence, by reviewing serial urodynamic studies.

Occult spinal dysraphism: neurogenic voiding dysfunction and long-term urologic follow-up

From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days - 21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days–21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.

DETRUSOR HYPOCONTRACTILITY EVOLUTION IN BOYS WITH POSTERIOR URETHRAL VALVES DETECTED BY PRESSURE FLOW ANALYSIS

PURPOSE We evaluated the natural evolution of detrusor voiding contractility in boys who underwent posterior urethral valve ablation using pressure flow analysis, which is a mathematical computerized analysis of pressure flow studies. MATERIALS AND METHODS Among 30 boys with posterior urethral valves who were being prospectively followed, even if asymptomatic on serial pressure flow studies, 11 were included in our study. These 11 patients had had at least 2 evaluations performed between ages 5 and 15 years, a minimum interval of 4 years between the first and last examination, and all pressure flow studies records available for mathematical analysis of voiding contractility. The first examination had been done at ages 5 to 10 years (average 7 +/- 2.04) and the last one at ages 9 to 15 (12.5 +/- 2.5), including 6 evaluated after puberty. All but 1 patient underwent valve endoscopic resection as a newborn and none received urinary diversion. Voiding symptoms, post-void residual, cystometric bladder capacity and bladder instability were considered. Voiding phase maximal detrusor pressure and flow rate were evaluated and detrusor contractility was calculated by the pressure flow analysis parameters of contraction velocity, detrusor contractile power expressed as watt factor and Schafers nomogram. Contraction velocity and contractile power factor were considered low if below 2 standard deviations of previously determined normal values. True hypocontractility was diagnosed when at least 2 pressure flow analysis parameters were low. RESULTS True hypocontractility was detected in 3 of the 11 boys at the first examination and in 8 at the last pressure flow analysis. The remaining 8 and 3 cases of first and last examinations, respectively, were considered to have normal contractility even if 4 of the 8 and 1 of the 3 had 1 low pressure flow analysis parameter (covert hypocontractility). Detrusor contractility worsened in 6 patients, hypocontractility was detected at the first pressure flow analysis in 2, hypocontractility changed to normal in 1 and pressure flow analysis remained normal in 2. Of the 6 boys followed through puberty 5 had hypocontractility, including 3 with cystometric bladder capacity greater than 700 ml., high post-void residual and strained voiding. Of the 11 patients 8 had detrusor instability, including 7 with urge symptoms, at first evaluation which was not found at last examination. CONCLUSIONS Pressure flow analysis extensively used in men has been confirmed as a useful tool to assess voiding contractility in children. The majority of boys with posterior urethral valves have progressive impairment of detrusor contractility at voiding many years after relief of obstruction. The pattern of hypocontractility, which is detected early on pressure flow analysis, follows a prolonged phase of instability in many cases and leads to an over distended bladder in most patients followed after puberty. Questions arise if this evolution may be prevented by early (pharmacological or rehabilitative) treatment and if it is partially determined by extensive use of drugs acting against unstable detrusor contractions.

Surgery for ulcerative colitis in pediatric patients: functional results of 10-year follow-up with straight endorectal pull-through

Children and adolescents affected by ulcerative colitis (UC) frequently require colectomy because of refractory or chronic symptoms. The aim of this paper is to present our experience and 10-year follow-up results of 28 patients who underwent endorectal pull-through (ERPT) as surgical treatment for UC, with special regard to surgical complications, stooling patterns (frequency of defecation, stool consistency, urgency period), fecal incontinence, and quality of life. A retrospective chart review of these patients was performed to evaluate age at colectomy, indication for surgical treatment, operative procedures, technical details, and early or late complications. Frequency of defecation was less than twice per day in two patients, between three and five times per day in nine patients, and more than six times per day in 10 patients. Stool consistency was normal in 14 patients, loose in five, and liquid in only two cases. Urgency period was normal (minutes) in 14 patients, short (seconds) in four, and absent in three. Ten patients (47%) have perfect or good fecal continence, whereas 11 (52%) patients present moderate to total incontinence. The self-reported emotional health was good in most of the patients. A large number are progressing well at school and are coping with their operations. Studies of quality of life in UC patients who underwent surgical treatment in childhood or adolescence, comparing as well the results according to the surgical technique adopted, must be encouraged.

Persistent cloaca: are we ready for a correct prenatal diagnosis?

Cloacal malformations are rare and can present in variable aspects. The importance of ultrasound in detecting these anomalies is well known. Sonographic features vary in accordance with the type of malformation and the gestational age. A positive diagnosis is not possible because of the lack of specific ultrasound findings, which can show similar aspects to other abnormalities. We present 3 cases of prenatal diagnosis of this malformation, emphasizing that in the presence of a plurilobed cystic pelvic fetal mass with associated malformations, such as cardiac, renal, and vertebral anomalies, a persistent cloaca can reasonably be suspected.

Surgery in disabled children: general gastroenterological aspects.

UNLABELLED Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70-75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation. CONCLUSION This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.

Neurovesical dysfunction in children after treating pelvic neoplasms

To evaluate 10 years of experience, and thus define the occurrence and causes, of neurogenic lower urinary tract dysfunction in children with pelvic neoplasms treated by surgery.

The impact of perinatal urinoma formation on renal function: our experience and review of the literature.

Introduction:  A urinoma is a fluid mass consisting of extravasated urine in the perirenal space. Its impact on renal function was analysed.

Ullrich-Turner Syndrome and Tumor Risk: Is There Another Chance to Early Gonadectomy in Positive TSPY and SRY Patients?

The presence of the Y chromosome in the karyotype of patients with disorders of sex differentiation is significantly associated with an increased risk to develop specific types of malignancies, predominantly type II germ cell tumors (GCTs). Gonadoblastoma in the gonads without an obvious testicular differentiation and intratubular germ cell neoplasia of unclassified type in testicular tissue are the precursor lesions of most GCTs. Gonadal dysgenesis, the characteristic feature of Ullrich-Turner syndrome (UTS), further contributes to increase this tumor risk. The reported incidence of Y chromosome material in UTS is 6 to 8% and in these cases an early gonadectomy is strongly recommended to prevent the risk of a malignancy. The aim of this work was to retrospectively analyze the clinical outcome and the histopathological and cytogenetic findings of our UTS patients who underwent gonadectomy to establish strict selection criteria aimed at promoting an organ-sparing surgery.

Analysis of readability and quality of web pages addressing both common and uncommon topics in pediatric surgery

INTRODUCTION The quality medical information on Internet is highly variable. The aim of this study is to determine if Web pages addressing four common pediatric surgical topics (CT) and four uncommon pediatric surgical topics (UT) differ significantly in terms of quality and/or characteristics. MATERIALS AND METHODS We performed an Internet search regarding four CT, addressing more frequent clinical conditions with an incidence≤1:1.500 children (inguinal hernia, varicocele, umbilical hernia, and phimosis) and four UT addressing less frequent clinical conditions with an incidence≥1:1.500 children (anorectal malformation, intestinal atresia, gastroschisis, and omphalocele), using a popular search engine (Google). We evaluated readability with the Flesch reading ease (FRE) and the Flesch-Kincaid grade (FKG) and quality of content using the site checker of the HON Code of Conduct (HON code) for each website. RESULTS In this study, 30/40 websites addressing CT versus 33/50 addressing UT responded to our criteria. No differences statistically significant in advertisements between the two groups were found (15 vs. 16%) (p>0.05). No differences were found in terms of time from last update, owner/author type, financial disclosure, accreditation, or advertising. CT had higher quality level according to the HON code (6.54±1.38 vs. 5.05±1.82) (p<0.05). Mean FRE was 47.38±14.27 versus 46.24±14.56, respectively, for CT and UT (p>0.05). The mean FKG was 8.1±1.9 for CT versus 8±1.9 for UT (p>0.05). CONCLUSIONS Websites devoted to pediatric surgical topics have higher readability and quality information for disease diagnosis and natural history. Otherwise, the quality of pediatric surgical information on the Internet is high for CT and UT. A high reading level is required to use these resources.