Laparoscopic selective clipping of upper moiety vasculature and ureter without partial nephrectomy: A novel technique for pediatric urinary incontinence due to ectopic ureter associated with poor functioning upper renal moiety.

OBJECTIVE: The aim of this study was to present a novel laparoscopic technique for persistent urinary incontinence in pediatrics due to ectopic ureter associated with poor functioning upper renal moiety. METHODS: This technique consisted of laparoscopic clipping of the upper moiety artery and vein. The ectopic ureter was also clipped afterwards without upper pole partial nephrectomy. RESULTS: The patient was a seven-year-old girl with persistent urinary incontinence and confirmation of duplex kidney with poor functioning upper moiety in pre-operative investigations. The upper moiety ureter was ectopically drained to the vaginal cavity. She was immediately dry after surgery and discharged on the second postoperative day. During the follow-up period of 14 months, she was continent and symptom-free. Hydronephrosis was not visualized in follow-up ultrasonography. CONCLUSIONS: This laparoscopic upper renal moiety vascular and ureteral clipping without partial nephrectomy could serve as a promising, safe and simple alternative in the treatment of patients with ectopic ureter associated with poor functioning renal moiety. Also, ipsilateral normal functioning moiety would not be associated with potential morbidity in this technique.

Outcomes of seromuscular bladder augmentation versus standard ileocystoplasty: A single institution experience over 14 years.

INTRODUCTION: Ileocystoplasty is the standard technique used for bladder augmentation, and has been used widely for decades. However, it is known to be associated with complications such as stone formation, mucus production, metabolic acidosis, urinary tract infections, intestinal obstruction, and a long-term risk of bladder cancer. Seromuscular bladder augmentation (SMBA) is an alternative to the standard ileocystoplasty, and has been associated with a lower incidence of bladder stones. Few reports have been published on intermediate outcomes of SMBA. Herein, we report long-term outcomes of SMBA from a single institution compared with standard ileocystoplasty. METHODS: After Institutional Review Board approval, a retrospective chart review of all patients who underwent bladder augmentation at our institution over a 14-year period was performed. The status of patients after SMBA (10 patients) was compared according to age, sex, and diagnosis with patients who underwent traditional ileocystoplasty (30 patients). Parameters such as demographic information, pre- and postoperative bladder capacity as assessed by urodynamic studies, urinary tract infections (UTIs), bladder calculi, incontinence, need for secondary surgical procedures, and spontaneous bladder perforation were compared in the two groups. All the patients were on a clean intermittent catheterization (CIC) regimen. RESULTS: Over the study period, 10 patients underwent SMBA and 30 patients (according to age, sex, and diagnosis) underwent standard ileocystoplasty; the average age at surgery was 10.3 and 10 years respectively, with a mean follow up of 6.7 years in the SMBA group and 6 years in the ileocystoplasty group. There were no statistically significant differences in the rate of UTIs, urinary incontinence, subsequent surgery, or spontaneous bladder perforation. The mean bladder capacity increased significantly for both groups as assessed by pre- and postoperative urodynamic studies, although the difference in the rate of bladder calculi between the two groups (0 [0%] vs. 8 [27%], p = 0.06) did not reach statistical significance (Table). CONCLUSIONS: SMBA is safe and efficacious and may result in a lower rate of stone formation than standard ileocystoscopy. SMBA should be considered as a viable alternative to standard ileocystoplasty.

Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome.

BACKGROUND: PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction. CASE PRESENTATION: Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring. CONCLUSIONS: Large perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

The long-term management and outcomes of cloacal anomalies.

Cloacal anomalies occur when failure of the urogenital septum to separate the cloacal membrane results in the urethra, vagina, rectum and anus opening into a single common channel. The reported incidence is 1:50,000 live births. Short-term paediatric outcomes of surgery are well reported and survival into adulthood is now usual, but long-term outcome data are less comprehensive. Chronic renal failure is reported to occur in 50 % of patients with cloacal anomalies, and 26-72 % (dependant on the length of the common channel) of patients experience urinary incontinence in adult life. Defaecation is normal in 53 % of patients, with some managed by methods other than surgery, including medication, washouts, stoma and antegrade continent enema. Gynaecological anomalies are common and can necessitate reconstructive surgery at adolescence for menstrual obstruction. No data are currently available on sexual function and little on the quality of life. Pregnancy is extremely rare and highly risky. Patient care should be provided by a multidisciplinary team with experience in managing these and other related complex congenital malformations. However, there is an urgent need for a well-planned, collaborative multicentre prospective study on the urological, gastrointestinal and gynaecological aspects of this rare group of complex conditions.

Sacral nerve stimulation is a valuable diagnostic tool in the management of anorectal and pelvic malformations.

BACKGROUND: We hypothesize that the current practice of sphincter assessment in anorectal malformations (ARMs) by direct muscle stimulation can be improved by sacral nerve stimulation (SNS). Focusing on the specific adjustments for infants, we highlight the anatomical and neurophysiologic basis of SNS and its further diagnostic and therapeutic implications. PATIENTS AND METHODS: We examined 20 patients: 12 patients with ARM, 3 with Hirschsprung disease, 3 with sacrococcygeal teratoma, and 2 with cloacal exstrophies. Under general anesthesia and ultrasound guidance, percutaneous needle electrodes were placed within the sacral neural foramina. Electrical stimulation was applied to assess the presence, pattern, and extent of the neuromuscular response of the external anal sphincter. RESULTS: We successfully modified the traditional method used in adults replacing radiographic controls by ultrasound guidance. In 20 SNS procedures with no complications, we found a muscle response on stimulation of the third and/or fourth sacral nerves in 18 of 20 patients (anal sphincter contraction and/or ipsilateral plantar flexion). CONCLUSION: We demonstrated the feasibility of SNS for intraoperative sphincter mapping and detection of primary innervation abnormalities in ARM as well as for the assessment of secondary deficits in postoperative follow-up. Sacral nerve stimulation broadens the spectrum of intraoperative information concerning the sacral innervation pattern by a direct assessment of the sacral nerves. Furthermore, electrophysiologic data may allow prospective criteria for sphincter function to be established. These may improve the accuracy of the present classification-based prognosis as well as the understanding of sphincter physiology in general.

[The surface anesthesia in outpatient pediatric department].

The combination of even parts lidocaine and prilocaine in crème substance was used for surface anesthesia in 73 children, aged 1,5-16 years. Such surgical interventions as molluscum contagiosum eradication, laser wart resection and prepuce synechia dissection were performed. The described anesthetic was highly effective in all areas, but the nasolabial triangle. Resection of warts larger then 0,5 sm required either additional infiltrative anesthesia or step-wise resection. Local allergic reaction was registered in one case, no systemic reactions were noticed. Generally, the used method of surface anesthesia proved to be highly appropriate in pediatric practice.