Daniel M. Weber

The Penile Perception Score: An Instrument Enabling Evaluation by Surgeons and Patient Self-Assessment After Hypospadias Repair

PURPOSE Studies of the outcome of hypospadias repair must document quality, including assessment of complications and appraisal of appearance. To our knowledge the Pediatric Penile Perception Score is the first validated instrument for the outcome assessment of hypospadias repair in prepubertal males by surgeons and patients. We validated the instrument for adult genitalia. MATERIALS AND METHODS Standardized photographic documentation was prepared for 19 men after hypospadias repair and 3 with normal genitalia after circumcision. This was sent to 21 urologists, who rated the outcome with a questionnaire comprising items on the penile meatus, glans, shaft skin and general appearance. Each item was rated with a 4-point Likert scale. The Penile Perception Score is a sum score of all items. Patients were asked to provide a self-assessment with the same instrument. RESULTS When calculated with the ICC and the rank correlation using Kendall W, concordance among urologist scores was fair and good (0.46 and 0.64, respectively, p <0.001). Instrument stability was 0.78, indicating good reproducibility. Using the Spearman rank correlation coefficient general appearance correlated well with single items, including the meatus (r = 0.93, p = 0.000), glans (r = 0.92, p = 0.000) and shaft skin (r = 0.89, p = 0.000). No significant differences were found between patient and urologist Penile Perception Scores. CONCLUSIONS The Penile Perception Score is a reliable instrument for urologist assessment and self-assessment of postpubertal genitalia after hypospadias repair. The instrument can be recommended for all age groups because it was previously validated for the pediatric population.

Is there an ideal age for hypospadias repair? A pilot study.

OBJECTIVE To assess whether repairing hypospadias before or after 18 months affects psychological adjustment, health-related quality of life (HRQoL) and surgical outcome. PATIENTS AND METHODS Seventy-seven boys aged 6-17 years were assigned to one of two groups, according to whether they had a hypospadias repair before or after the age of 18 months. The surgical outcome was assessed using the pediatric penile perception score by non-involved urologists. A psychologist interviewed the patients to assess HRQoL and gender-role behavior. The childs psychological adjustment was assessed with a questionnaire for parents. RESULTS The surgical outcome and complication rate were not significantly different between groups. A covariance analysis of HRQoL, gender-role behavior and psychological adjustment as a function of age at the last operation with current age as covariant was performed, but differences did not reach significance. CONCLUSION This study does not provide evidence to support recommendations concerning the ideal age for hypospadias repair. In the absence of evidence of a benefit of early surgery, anesthesia-related risk factors must be considered when operating in very early infancy. Large, prospective studies, measuring surgical and psychological outcome with similar instruments to those presented may reveal whether there is a true ideal age for hypospadias surgery.

Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: Uro-nephrological outcome

OBJECTIVE To describe the urological and nephrological long-term outcome of patients born with classical bladder exstrophy treated with bilateral ureterosigmoidostomies in early childhood. PATIENTS AND METHOD Out of 42 patients born with bladder exstrophy in Switzerland between 1937 and 1968, 25 participated in this study; seven had died, seven were lost to follow up and three refused consent. Assessment included chart review, clinical examination, and assessment of renal function and morphology. RESULTS After a follow-up period of 37-69 years ((mean 50 years), 13 of the 25 participants (52%) had their ureterosigmoidostomy still in place. All others had different forms of urinary diversions. Fifteen (60%) patients had normal renal function or mild chronic kidney disease as assessed by estimated glomerular filtration rate. Three patients were on renal replacement therapy. MRI (n=16) showed 10 morphologically normal kidneys. One patient suffered from adenocarcinoma of the colon, five had benign colonic polyps, one urethral papillary carcinoma and 18 no evidence of tumor. CONCLUSION The majority of our patients have normal or mildly impaired renal function and a well functioning ureterosigmoidostomy. This is remarkable, given the fact that ureterosigmoidostomies are considered to be refluxing high-pressure reservoirs at risk of renal injury and malignancy.

Ureteral obstruction following injection of dextranomer/hyaluronic acid copolymer: An infrequent but relevant complication

PURPOSE To report our experience with ureteral obstruction after injection of dextranomer/hyaluronic acid copolymer (Dx/Ha) to treat vesicoureteral reflux, and analyze its possible causes, management and outcome. MATERIALS AND METHODS Retrospective review of patients undergoing injection of Dx/Ha. The charts of patients with clinically relevant ureteral obstruction were evaluated for indications, prior interventions, technique of injection and volume injected. Video recordings obtained during injection were analyzed to detect possible technical errors. RESULTS Fifty-four patients (87 ureters) were treated with Dx/Ha injection in a 5-year period. Five ureters (5.7%) in five patients (9.3%) developed significant ureteral obstruction requiring intervention. Manifestations of obstruction included pain in two patients, urinary tract infections in one and loss of function in one. Increased serum creatinine was observed in a patient with a transplanted kidney. Four obstructions resolved spontaneously (two after percutaneous nephrostomy, two after placement of a ureteral stent) and one required reimplantation. Review of the videos did not reveal any deviation from the usual technique. The volumes injected in the obstructed cases (0.7-1.2 ml) were in the usual range. CONCLUSIONS In this series, the incidence of post Dx/Ha ureteral obstruction was higher than previously reported. Although 4/5 cases resolved spontaneously, they required drainage to relieve symptoms or to improve renal function. Surgeons need to be aware of this complication and include its possible occurrence in the informed consent obtained prior to injection.

Repeated Botulinum-A toxin injection in the treatment of neuropathic bladder dysfunction and poor bladder compliance in children with myelomeningocele.

To evaluate the efficacy of intradetrusor injection of Botulinum‐A toxin (BoNT/A) in children with neuropathic bladder dysfunction and poor bladder compliance, due to meningomyelocele.

Long-Term Followup (37 to 69 Years) in Patients With Bladder Exstrophy Treated With Ureterosigmoidostomy: Psychosocial and Psychosexual Outcomes

PURPOSE We describe the long-term psychosocial and sexual outcomes of patients born with bladder exstrophy treated with ureterosigmoidostomy at our institution. MATERIALS AND METHODS Of 42 patients born with bladder exstrophy between 1937 and 1968, 28 were located, of whom 25 agreed to participate in this study. Evaluation consisted of studying pediatric and adult medical records, medical examination, and reports of sexual and social history. RESULTS Four female and 21 male patients with a mean age of 50 years (range 39 to 67) were evaluated. Of the patients 21 (84%) were married or lived in a stable relationship, 6 (24%) had several children after normal conception and an additional 2 (8%) became parents with assisted reproductive technology. Nine patients (36%) reported no sexual activity or were unable to engage in sexual intercourse. All patients except 2 are professionally and socially successful. CONCLUSIONS The long-term followup of this study of 37 to 68 years provides valuable information on patients who live with bladder exstrophy during a long period. Most followed patients who were born with bladder exstrophy and treated with ureterosigmoidostomy were able to lead satisfactory lives socially and professionally. However, at least 36% of the patients are severely restricted in their sexual lives.

Health-related quality of life in men with corrected hypospadias: An explorative study

OBJECTIVE The aim of the study was to evaluate health-related quality of life (HRQoL) of men with a corrected hypospadias in comparison to circumcised controls. Furthermore, determinants of HRQoL were examined. PATIENTS AND METHODS In a cross-sectional study, HRQoL of 45 men with corrected hypospadias (mean age: 26.2 years +/- 5.1) was compared with a control group of 46 circumcised men (mean age: 25.5 years +/- 4.9). Participants answered three questionnaires: The Medical Outcomes Study Short Form-36 item questionnaire (SF-36), the International Index of Erectile Function (IIEF), and the Penile Perception Score (PPS). RESULTS Physical and mental dimensions of HRQoL were not impaired among men with corrected hypospadias if compared to circumcised men. Neither socio-demographic and medical variables nor erectile function (EF) predicted HRQoL. However, a negative genital self-perception (PPS) and a lower orgasmic function (OF) were risk factors for an impaired mental HRQoL. CONCLUSIONS Our data suggest that the HRQoL of adult hypospadias patients is comparable to that of circumcised men. However, patients should be supported in developing a positive genital self-perception, because poor genital self-perception correlated with an impaired mental HRQoL. Since studies with non-operated men suggest that some adapt well to their penile condition, further studies should also include non-operated hypospadias.

To Cohen and Forget? Evaluation of Postoperative Imaging Studies after Transtrigonal Ureteric Reimplantation for Vesicoureteric Reflux in Children

Objectives: To determine the clinical significance of routine postoperative voiding cystourethrography (VCUG) and renal functional studies in the postoperative management of children after a transtrigonal ureteric reimplantation. Methods: A retrospective record review of 126 consecutive patients undergoing transtrigonal ureteric reimplantation. Inclusion criteria included primary reflux and >5 years of follow-up. Follow-up imaging studies consisted of serial renal ultrasounds (US) and one VCUG and intravenous urogram (IVU) each. Results: Of 126 patients, 2 required a reoperation for contralateral reflux and pyelonephritis. In all other patients the results of the VCUG did not alter management. Dilatation seen in IVU was always visible in the renal US as well and always resolved spontaneously. No new dilatation was observed after 1 year of follow-up. Conclusions: Routine postoperative VCUG and renal functional studies are not warranted in asymptomatic patients after transtrigonal reimplantation. Only in patients with postoperative pyelonephritis did the imaging studies alter the treatment. In the majority of patients, follow-up with an early and 1-year renal US may suffice. Elimination of routine VCUG and functional studies will decrease morbidity and cost after ureteric reimplantation.

Conservative treatment of stable volar plate injuries of the proximal interphalangeal joint in children and adolescents: a prospective study.

Objectives: The purpose of this study was to assess a standard conservative management for stable volar plate injuries of the proximal interphalangeal joint in children and adolescents. No study is available regarding this subject for this age group. Methods: A prospective study was performed on 37 consecutive patients (aged between 9 and 15 years; mean, 13 years) with stable acute volar plate injuries of the proximal interphalangeal joints, including 22 avulsion fractures without dislocation. All patients had a standardized conservative treatment consisting of a dorsal aluminum extension block splinting at a 15-degree flexion for 10 days, followed by a spontaneous mobilization and taping to adjacent fingers for sports only. At follow-up visits, active and passive ranges of motion, swelling of the affected joints, analgesic intake, and pain perception by the patient were recorded. Regular follow-up consisted of standardized assessments at 2, 6, and 12 weeks, with additional consultations if symptoms persisted. Results: Healing was uneventful, and hand therapy was not necessary to regain full range of motion in 32 of 33 children with a regular follow-up. The only flexion contracture observed responded well to splinting. Of the 33 patients, 31 had an excellent outcome and 2 had a good outcome. Conclusions: Dorsal extension block splinting was an efficient, simple, well-tolerated treatment for stable volar plate injuries of the proximal interphalangeal joint in a preliminary series of patients younger 16 years. Flexion contractures were rare and responded well to dynamic splinting.

Primary reconstruction of fingernail injuries in children with split-thickness nail bed grafts.

INTRODUCTION Failure to detect and treat partial or complete avulsions of the nail bed may lead to severe nail deformity that predisposes to repeat injuries and is cosmetically inacceptable. Treatment of these injuries with split-thickness nail bed grafts (STNBGs) is controversial and no pediatric series has been published. METHODS A retrospective, single center case series of nine fingers with complex nail bed injuries that were reconstructed primarily with STNBGs was performed. Surgical outcome and patient satisfaction were assessed. RESULTS For six nail bed reconstructions, the nail bed graft was harvested from the injured finger, and for the remaining three from the great toe. Harvesting of the great toes nail bed could be performed without removal of the nail plate by only lifting it up distally. Insufficient vascularization required primary flap coverage in six cases with three Moberg flaps, two palmar V-Y flaps, and one thenar flap. No flap was lost and all nail bed grafts had a 100% take. Only one patient required reoperation due to a hook- and split-nail deformity. All other patients were satisfied or very satisfied and the surgical outcome was least satisfactory in all but two patients. CONCLUSIONS Primary reconstruction of complex nail bed injuries with STNBGs usually gives good cosmetic and functional results in children and prevents secondary nail growth disturbances reliably.