Continuous caudal epidural analgesia and early feeding in delayed bladder exstrophy repair: a nine-year experience.

BACKGROUND: Children undergoing primary closure of bladder exstrophy experience blood loss and significant fluid shifts and require protracted periods of postoperative immobilization to avoid compromising the repair. Suboptimal anxiolysis and pain management is associated with increased morbidity. There is a lack of consensus on the optimal analgesic technique and studies have not previously described analgesic management in delayed bladder exstrophy closure. In exstrophy management, opioid infusions and benzodiazepine sedation are commonplace but are associated with dose-dependent respiratory and gastrointestinal side-effects. We present nine years of caudal epidural anaesthesia in delayed bladder exstrophy repair and describe its facilitation of early extubation and early feeding (within 12 h) without surgical complication. METHODS: We retrospectively evaluated consecutive infants with classic bladder exstrophy undergoing delayed primary closure with anterior pelvic osteotomies between November 2007 and January 2016. Outcomes and complications were evaluated in terms of postoperative comfort (using the FLACC score-Face, Legs, Activity, Cry, Consolability), epidural failure rate, re-intubation rate and gastrointestinal complications. RESULTS: Forty-four infants had average age of 5.8 months (range 1.6-17.1 months) and weight of 7.0 kg (range 3.5-11.8 kg), and their duration of surgery was 9.5 h (range 6.9-14.3 h). Forty-two of 44 (95.5%) patients received caudal epidural catheters. At 24 h, 15 of 42 (35.7%) caudal epidurals required supplementation with intravenous opioids. Fewer patients with optimally functioning epidurals required postoperative ventilation (1/27 [3.7%] compared with 3/15 [20.0%]). None of the patients with caudal epidural catheters required re-intubation. Pain scores were lower in infants with isolated caudal epidurals catheters than those with caudal epidurals supplemented by intravenous opioids (day 1 [18 vs 53; P = 0.008]; day 2 [8 vs 15; P > 0.05] and overall [32 vs 65; P = 0.014]). Infants with intravenous opioids experienced higher complications: pruritus (25% [95% confidence interval {CI}: 5%-57%] vs 0% [95% CI: 0%-13%]; P = 0.026) and nausea and vomiting (25% [95% CI: 5%-57%] vs 8% [95% CI: 1%-25%]; P = 0.30) requiring treatment. Nineteen of 44 (43.1%) infants were fed early (within 12 h of surgery). Infants who were fed early had lower pain scores than infants feeding late (day 1 [17.5 vs 31; P > 0.05]; day 2 [5.5 vs 15; P = 0.045]; overall [26 vs 55.5; P = 0.015]) without increase in complications (nausea and vomiting [6.3% vs 20.0%; P = 0.06]; ileus [0.0% vs 0.0%]; aspiration [0.0% vs 0.0%] and re-intubation aspiration [0.0% vs 0.0%]). CONCLUSIONS: Caudal epidural analgesia facilitates postoperative extubation in infants undergoing delayed exstrophy repair. Early feeding (within the first 12 h) in delayed bladder exstrophy repair is likely to improve patient comfort and consolability without increasing the incidence of gastrointestinal complications. Intravenous opioid may be associated with increased postoperative complications that may influence peri-operative outcomes.

Management of psychogenic non-epileptic seizures: a multidisciplinary approach.

The International League against Epilepsy (ILAE) proposed a diagnostic scheme for psychogenic non-epileptic seizure (PNES). The debate on ethical aspects of the diagnostic procedures is ongoing, the treatment is not standardized and management might differ according to age group. The objective was to reach an expert and stakeholder consensus on PNES management. A board comprising adult and child neurologists, neuropsychologists, psychiatrists, pharmacologists, experts in forensic medicine and bioethics as well as patients' representatives was formed. The board chose five main topics regarding PNES: diagnosis; ethical issues; psychiatric comorbidities; psychological treatment; and pharmacological treatment. After a systematic review of the literature, the board met in a consensus conference in Catanzaro (Italy). Further consultations using a model of Delphi panel were held. The global level of evidence for all topics was low. Even though most questions were formulated separately for children/adolescents and adults, no major age-related differences emerged. The board established that the approach to PNES diagnosis should comply with ILAE recommendations. Seizure induction was considered ethical, preferring the least invasive techniques. The board recommended looking carefully for mood disturbances, personality disorders and psychic trauma in persons with PNES and considering cognitive-behavioural therapy as a first-line psychological approach and pharmacological treatment to manage comorbid conditions, namely anxiety and depression. Psychogenic non-epileptic seizure management should be multidisciplinary. High-quality long-term studies are needed to standardize PNES management.

Antegrade sclerotherapy in adolescent varicocele patients.

INTRODUCTION: In the 1970s, Tauber described the antegrade sclerotherapy technique to treat varicoceles, and reported a 10% recurrence rate. The present study aimed to evaluate paediatric success rates and the effect of modifications to the surgical technique. METHODS: A prospective study was performed of all adolescent patients undergoing antegrade sclerotherapy surgery. Each patient had an idiopathic varicocele with spontaneous venous reflux on Doppler examination, and underwent cannulation of a pampiniform plexus vein via a scrotal incision under general anaesthetic. Aethoxysklerol® 3% (2 ml/kg) maximum 3 ml was injected into the pampiniform plexus vein under fluoroscopic monitoring. Success was assessed by clinical examination and Doppler ultrasound 3, 6 and 9 months after surgery. Data were presented as median (interquartile range). Patients were split into three groups: Group A - liquid sclerotherapy with Y connector; Group B - liquid sclerotherapy direct to cannula; and Group C - foam sclerotherapy direct to cannula. Fisher's exact test was used to compare the success rates in each group. RESULTS: A total of 91 patients underwent antegrade sclerotherapy. The median age was 14.8 years (range 13.7-15.5). Eleven persistent varicoceles occurred and were diagnosed by Doppler ultrasound. The success rate of surgery was 58% in Group A, 90% in Group B and 96% in Group C. Success was significantly higher in Group B and Group C compared with Group A. No testicular atrophy occurred; two wound infections, two haematomas and one hydrocele were recorded (Table). CONCLUSION: Introduction of antegrade sclerotherapy in the adolescent population resulted in a safe and cost-effective method for the management of adolescent varicocele. Several modifications to the technique have been introduced to achieve a high success rate (96%) with minimal complications.

Alternative ileal flap for bladder augmentation if mesentery is short.

PURPOSE: To date the clam ileocystoplasty is the preferred method of bladder augmentation in children when the urodynamic problem is non-compliance and/or detrusor overactivity. The key to this technique is the incision of the bladder wall deep into the pelvis down to the trigone in order to avoid a diverticulum like neobladder and to provide adequate margin for augmentation. The detubularised ileum flap therefore has to reach to the bottom of the divided bladder on a reliable vascular pedicle without significant tension. A short ileal mesentery caused by previous surgery, peritonitis, peritoneal dialysis or ventriculo-peritoneal shunt may complicate surgery and compromise outcome. We hypothesized we can rely on the communication of the intramural vessels within the intestine and can detubularise the ileum adjacent to the mesentery rather than along the antimesenteric line and this could be combined with ligation of some vasa recta (VR) in order to create alternative ileum flaps, which reach further into the pelvis. Our aim was to assess the viability of the alternative flaps detubularised along the paramesenteric line and measure how many VR could be sacrificed beyond the tertiary arcades. MATERIALS AND METHODS: After ethical approval adjacent ileal segments were detubulirased along the antimesenteric line (Group 1) and along the paramesenteric line (Group 2) in 5 minipigs in general anaesthesia. Ligation of 0,1,2,3 and 4 VR has been performed starting from the free end of the segments. The length of the ileal flaps was recorded. The microcirculation of flap edges was detected by in vivo microscopy using orthogonal polarising spectral imaging (Cytoscan A/R Cytometrics, PA, USA). Clam ileocystoplasty was performed with the ileum detubularised along the paramesenteric line without ligation of VR. Specimens of the augmented bladder were obtained after 4 weeks and stained with Hematoxilin + Eosin. RESULTS: No alteration in capillary red blood cell velocity (RBCV) and perfusion rate (PR) was observed after paramesenteric detubularisation. The flaps in Group 2 reached 20.25 ± 0.5 mm longer vs. CONTROL: This is 98% of the mean bowel width (20.5 ± 0.57 mm) measured in the animals. Ligation of each VR further increased the length of both flaps (mean: 10.59 ± 3.18 mm) however ligation of more than 2 VR gradually decreased the microcirculation in both groups. All animals augmented with alternative flap survived, there was no urine leak or suture break down. Histology confirmed viable bowel flaps. CONCLUSION: Paramesenteric detubularisation of the ileum is fully tolerated and results in longer reaching ileal flap vs. CONTROL: Only limited ligation of VR is tolerated. DISCUSSION: This study showed the first time that clam ileocystoplasty is feasible with ileal flap detubularised along the paramesenteric line. The use of the animal model and the relative short postoperative observation are the main limitations of this study.

Sperm banking in the United Kingdom is feasible in patients 13 years old or older with cancer.

PURPOSE: Assisted reproductive technologies are increasingly being used to treat infertility. Male adolescents with cancer are particularly encouraged to bank semen to preserve fertility before beginning chemotherapy or radiotherapy. We evaluated the feasibility of semen preservation in 12 to 17-year-old patients with cancer. MATERIALS AND METHODS: We retrospectively collected data from the sperm banking database at our institution for the years 1995 to 2009. Outcomes measured were histological diagnosis, success rate, sperm concentration and sample volume. RESULTS: A total of 180 patients with a mean age of 16.1 years (range 13.2 to 17.9) were referred for cryopreservation during the study period. Underlying diagnoses included lymphoma (64 patients), leukemia (50), bone tumors (18), testicular tumors (13), soft tissue sarcoma (13), brain tumor (6), germ cell tumors (6) and other cancers (10). Of the patients 119 (66%) successfully banked sperm. A total of 26 patients did not attend their appointment. Of those who attended 15 (10%) were unable to provide a sample and 20 (13%) had azoospermia. A total of 20 patients died after banking sperm and their specimens were subsequently destroyed. CONCLUSIONS: Cryopreservation of semen of acceptable quality for future use in assisted conception is feasible for most adolescents from age 13 years onward.

Antenatal diagnosis of bladder/cloacal exstrophy: challenges and possible solutions.

OBJECTIVE: To identify the pitfalls in accurate antenatal diagnosis of bladder exstrophy (BE) and cloacal exstrophy (CE), and thus understand the challenges for antenatal counselling. METHODS: A prospectively maintained bladder exstrophy database of antenatal and live born referrals for BE/CE was used to identify patients. Data were collected about the antenatal scan findings and the outcome of pregnancy. RESULTS: Between 2003 and 2009, 40 new babies with BE/CE were referred and of them 10 had an antenatal diagnosis. Five patients did not have a diagnosis despite suspicious findings noted on antenatal scans and another three had a wrong diagnosis of BE/CE. Of the 16 referrals with antenatal suspicion of BE/CE, 5 opted for termination. At the 20-week scan, it was possible to identify the gender of the fetus in 3/16 cases only. CONCLUSIONS: Only a quarter of the babies born with BE/CE had received an antenatal diagnosis. Raising awareness about the condition amongst radiographers, and facilitating further scanning by a specialist fetal management unit if suspicious findings are noted, is crucial for improving the rate of detection. An antenatal diagnosis may not be reliable, and difficulty in identifying gender at the 20-week scan adds to the complexity of antenatal counselling. Magnetic resonance imaging and karyotyping may provide additional helpful information.

Complete primary repair of bladder exstrophy: a single institution referral experience.

PURPOSE: The reasons for referral and treatment strategies in patients who underwent complete primary repair of bladder exstrophy elsewhere in the newborn period were evaluated. MATERIALS AND METHODS: An institutionally approved database identified patients who underwent complete primary repair of exstrophy and were subsequently referred for continued care. RESULTS: A total of 10 females and 55 males were referred for treatment between 1996 and 2010. Six females and 23 males were referred for complications following initial complete primary repair of exstrophy. Female complications included dehiscence (3 patients), prolapse (2) and stricture (1). Male complications included dehiscence (10 patients), prolapse (9), pubic separation (1) and stricture (3). Nine males had posterior urethral loss and 13 had major penile soft tissue injuries. Grade V vesicoureteral reflux and severe hydronephrosis were seen in the 4 patients with urethral strictures. Patients with dehiscence or prolapse underwent successful reclosure with osteotomy. Cases with posterior urethral loss or strictures were repaired with grafts. Four females and 32 males were referred for incontinence. Nine of these patients had poor bladder capacity, of whom 5 underwent bladder augmentation with continent stoma and 4 are awaiting further bladder growth. A total of 27 patients underwent bladder neck repair, with 15 (56%) subsequently continent, 5 (19%) daytime continent with nocturnal incontinence and 7 (26%) continuously incontinent. At initial closure osteotomy had been performed in all patients who were continent following bladder neck reconstruction and in 4 of 5 who were daytime continent. CONCLUSIONS: Complications of complete primary repair of bladder exstrophy can result in undesired upper urinary tract changes and penile soft tissue loss. Surgical reconstruction of such complications and acceptable continence are attainable in select cases.

Sexual function and fertility issues in cases of exstrophy epispadias complex.

In patients with EEC, the issues such as sexuality, sexual function and fertility gain more importance once theses patients advance from puberty to adulthood. The aim of this review is to critically examine the available evidence on these issues. A systemic literature search was performed in Medline over the last 25 years using the key words: Exstrophy, sexual function and pregnancy. Search results were limited to studies of patients with exstrophy published in English literature. A total of 1500 publications were found and subsequently screened by title and when appropriate by abstracts. Of these, 40 publications pertinent to the subject were included for the analysis. The publications were supplemented by an additional 15 publications obtained from their bibliographies. The studies were rated according to the guidelines published by the US department of health and human services. Heterosexuality is usually expressed in both the sexes and most of them have adequate sexual function. Urinary diversion in some series seems to result in better ejaculatory hence fertility outcome in male patients. Recent series have shown equally good results with primary reconstruction. Most of the female patients have normal fertility while male patients have significantly low fertility. Most of the male and female patients with EEC have adequate sexual function. Most of the female patients have normal fertility while most of the male patients have significantly low fertility.

Correlation between high-resolution ultrasound and surgical/pathological findings in patients with suspected appendicitis.

AIM: Clinical diagnosis of appendicitis in children is often difficult. The aim of this study was to evaluate the usefulness of ultrasound with graded compressed technique in cases with suspected appendicitis. METHODS: A radiological classification of appendicitis was formulated associated with the surgical/histological degree. Afterwards, 92 patients with suspected appendicitis enrolled in this study were prospectively evaluated and managed to follow a new protocol based on the clinical and radiological experiences. In this study, the ultrasonography was considered positive when the diameter of the wall of the appendix was larger than 7 mm and vascularization was increased or absent. RESULTS: Of these 92 patients, 54 patients underwent surgery while 38 were treated conservatively. Of the treated group, 12 patients had a perforated appendicitis, 3 had a gangrenous appendicitis, 36 patients demonstrated a phlegmonous appendix while 3 patients had a catarrhal appendix. Blood cell counts and CRP levels were significantly higher in patients with appendicitis (P<0.05) and WBC and CRP levels were higher for patients with perforated appendicitis compared with patients with simple appendicitis (P<0.05). A CRP value higher than 17 mg/dL was a strong predictor for the presence of infection. Three patients of the conservative group underwent surgery later for recurrent abdominal pain (within 2 weeks); two had a catarrhal appendix while one patient had a normal appendix (confirmed by histology) CONCLUSIONS: Patients with suspected appendicitis could be managed with ultrasound, suggesting an early approach. The selection of patients for surgery prevent complication and unnecessary surgery.

Microsurgical spermatico-epigastric venous anastomosis in the treatment of varicocele in children: assessment of long-term patency.

AIM: To demonstrate the long-term patency of microsurgical anastomoses between the internal spermatic vein and the inferior epigastric vein, constructed in the treatment of essential varicocele in paediatric patients to supplement ligation of the spermatic veins. MATERIALS AND METHODS: We submitted 66 patients to inguinopelvic colour-flow Doppler ultrasonography. The patients had been treated 18 - 36 months earlier for essential varicocele by microsurgical inguinal ligation of the testicular venous pedicle and anastomosis between the internal spermatic vein and the inferior epigastric vein. RESULTS: Preoperatively, the 66 patients operated on at ages ranging from 10 to 16 years (13 +/- 1.4) presented with Dubin and Amelar grade II (14 patients) or grade III (52 patients) left varicocele with ipsilateral testicular hypotrophy. The postoperative follow-up showed 2 cases of persistence of disease and 3 cases of persistence of "medium" spermatic vein reflux without clinical evidence of varicocele. Seven patients developed left hydrocele which resolved spontaneously in 5 cases, whereas in 2 cases it proved necessary to perform an eversion of the tunica vaginalis of the testis. The results obtained in patients treated for Coolsaet type I varicocele (64 patients) were as follows: long-term patency of the anastomosis was observed in 58/64 patients (90.6 %); in 4 patients (6.2 %) the left inferior epigastric vein presented a position in relation to the homologous artery that prevented adequate sampling and thus made it impossible to assess the patency of the anastomosis; in 2 patients (3.1 %) the anastomosis was closed. In the two patients who had submitted to anastomosis for Coolsaet type III varicocele, colour-flow Doppler failed to identify the shunt. CONCLUSIONS: The results of this study demonstrate that microsurgical anastomosis between the internal spermatic vein, and the inferior epigastric vein remains competent in the long term, thus confirming the validity of this technique for the treatment of essential varicocele in children.

Treatment of neonatal chylous ascites using a modified Denver peritoneovenous shunt: a case report.

Chylous ascites is rarely observed in newborns. Its etiology is not always readily identifiable. The authors report a patient with idiopathic chylous ascites resistant both to conservative treatment with total parenteral nutrition plus diuretics and to dietary treatment (milk with a low-fat content and medium-chain triglycerides). Surgical correction of an associated intestinal malrotation and an annular pancreas failed to resolve the ascites and we therefore placed a modified Denver peritoneovenous shunt. Six months after placement of the shunt, the patient was in good general condition, with restoration of normal oral feeding and regular growth in terms of height and weight. Clinical and ultrasonographic follow-up examinations have not revealed the presence of ascitic fluid in the peritoneal cavity.

Oxidative stress, vitamin A and vitamin E behaviour in patients submitted to conservative surgery for complicated Crohn's disease.

AIMS: To assess whether plasma peroxidation and plasma levels of antioxidant compounds are correlated with clinical and biochemical activity in complicated Crohn's disease patients, and to evaluate whether the relief of obstructive complication by conservative surgery has any effect on the oxidative stress. PATIENTS AND METHODS: From May 1998 to May 2000, 20 Crohn's disease patients were studied. Basal peroxidative state (basal thiobarbituric acid reactive substances), peroxidative state after stimulation with copper sulfate (stimulated thiobarbituric acid reactive substances], lag time of plasma peroxidation susceptibility, plasma levels of vitamin E and A, C reactive protein, erythrocyte sedimentation rate and Crohn's disease activity index, were determined, before surgery, then 2 months and 1 year after surgery. A group of 134 healthy volunteers were used as controls. All patients were treated by conservative surgical procedures (i.e., strictureplasty and/or minimal resections). Student t test for paired and unpaired data and Spearman R correlation coefficient were calculated. RESULTS: Peroxidative plasma levels, as well as inflammatory indices, are significantly reduced 2 months and 1 year after surgery (p < 0.005), but basal levels of peroxidation and antioxidant scavengers seem to be disregulated in Crohn's disease patients compared to those in controls (p < 0.005). A correlation was found between basal thiobarbituric acid reactive substances, lag-time and erythrocyte sedimentation rate (R:0.51; p < 0.05. R:0.56; p < 0.05) and C reactive protein (R:0. 6; p < 0.005. R:0. 65; p < 0.005). CONCLUSIONS: An imbalance between pro- and antioxidant mechanisms, due to chronic gut inflammation, is present in complicated Crohn's disease, and an excess of lipid peroxidation is probably an important pathogenetic factor Conservative surgery can reduce the oxidative stress avoiding repeated or extended resections that could lead to intestinal malabsorption and short bowel syndrome.

Congenital true pancreatic cyst in pediatric age: case report.

Congenital pancreatic cysts are rare lesions in pediatric age. We present a case of congenital true pancreatic cyst in otherwise asymptomatic boy aged 4 years. Clinical history, laboratory, imaging, immunohistological findings and surgical treatment are also described.