Effectiveness of unilateral sequential video-assisted sympathetic chain blockage for primary palmar hyperidrosis in children and adolescents.

Introduction: Primary palmar hyperhidrosis (PPH) is a severely debilitating condition that can affect patients of any age. Thoracoscopic sympathectomy provides a definitive treatment for PPH. Aim of this study is to investigate the effectiveness of unilateral sequential video-assisted thoracic sympathetic chain clamping (VATSCC) by clips application in pediatric population. Methods: The surgical procedure was done in the semi-sitting position, under general anesthesia with orotracheal intubation. Mean operation time was 23 ± 6 min (range 12-45). Two 5 mm ports were inserted at the level of the middle axillary line in the second and fourth intercostal space respectively. The sympathetic chain was identified, and two clips were applied, the first one at the level of the third and the second one, at the level of the fourth rib. No chest tube was used. Resolution of symptoms, complications, recurrence rate, onset and duration of compensatory hyperhidrosis were analyzed. Results: From August 2017 to September 2021, 58 patients (male:female ratio 32:26), mean age 16.5 years (range 14-19), with PPH underwent unilateral sequential VATSCC by clips application, starting on the dominant hand. The contralateral side was operated 2 months after. All patients except one (transient pneumothorax) were discharged on the first post-op day. No immediate or late complications have been recorded. Mean follow-up was 32 months (range 6-41). All patients except one (1,7%), affected by Raynaud's disease, showed a complete resolution of the symptom. Seven patients (12%) developed transient moderate compensative hyperhidrosis (CH) that spontaneously disappeared in the postoperative period. Conclusions: Unilateral sequential thoracoscopic sympathetic chain clamping for PPH in pediatric patients is a safe and very effective procedure with a low complication rate and low incidence of postoperative CH that, in our experience, resolved spontaneously in the postoperative period, after the second surgery leading to an improvement in the quality of life.

Changes in Total Homocysteine and Glutathione Levels After Laparoscopic Sleeve Gastrectomy in Children with Metabolic-Associated Fatty Liver Disease.

PURPOSE: Paediatric obesity is a well-known risk factor for metabolic-associated fatty liver disease (MAFLD). The aim of this study was to evaluate the effects of laparoscopic sleeve gastrectomy (LSG) on the levels of total homocysteine (tHcy) and total glutathione (tGSH) plasma levels in children with MAFLD. MATERIAL AND METHODS: Twenty-four children with severe obesity who underwent LSG were included in the study. The metabolic parameters, systemic inflammatory markers, one-carbon metabolism products, ultrasound and histological improvement were evaluated at baseline (T0M) and after 12 months from LSG (T12M). RESULTS: The patients exhibited a significant amelioration of several metabolic parameters at T12M. A significant reduction of steatosis was observed at ultrasound (from 72.7% of moderate-severe grade to 0% severe steatosis), accompanied by a statistically significant improvement of ballooning, portal and lobular inflammation and fibrosis. A statistically significant decrease of tumour necrosis factor circulating levels was also observed (T0M median = 290.3, IQR = 281.0-317.0 pg/mL; T12M median = 260.4, IQR = 240.0-279.0 pg/mL; p < 0.0001). After 12 months from LSG, a significant increase of mean plasma levels of tHcy(T0M mean = 15.7 ± 4.1 µmol/L; T12M mean = 21.1 ± 9.3 µmol/L; p = 0.0146) was also observed. The increase of tHcy showed no causal link with the improvement of MAFLD-related inflammatory, metabolic and histological pattern. CONCLUSION: LSG in children with obesity induces an improvement of MAFLD-related metabolic derangement and liver damage, but also a mild hyperhomocysteinemia that should be avoided to prevent cardiovascular risk.

Long-term efficacy of percutaneous tibial nerve stimulation for different types of lower urinary tract dysfunction in children.

PURPOSE: We evaluated the efficacy of percutaneous tibial nerve stimulation for different types of pediatric lower urinary tract dysfunction. MATERIALS AND METHODS: A total of 14 children with idiopathic overactive bladder, 14 with dysfunctional voiding, 5 with underactive bladder, 4 with underactive valve bladder and 7 with neurogenic bladder resistant to conventional therapy underwent percutaneous tibial nerve stimulation weekly for 12 weeks. The stimulation effect was evaluated by comparing bladder diary, flowmetry and urinalysis before and after treatment. Improved patients were followed by bladder diary and urinalysis. Followup data at 1 and 2 years were compared with those obtained after stimulation. Data were analyzed using Fisher's exact test. RESULTS: Symptom improvement was significantly greater in nonneurogenic than in neurogenic cases (78% vs 14%, p <0.002). Of patients 18% with underactive bladder and 50% with underactive valve bladder were unresponsive. Of 14 overactive bladder cases 12 and all 14 of dysfunctional voiding were improved (p not significant). Of improved patients 5 of 12 with overactive bladder and 12 of 14 with dysfunctional voiding were cured (p <0.01). On uroflowmetry voided volume and post-void residual urine became normal in a greater number of dysfunctional voiding than overactive bladder cases (57% vs 20% and 57% vs 25%, each p not significant). At 1 year of followup the cure rate was greater in dysfunctional voiding than in overactive bladder cases (71% vs 41%) and it remained the same at the 2-year evaluation. Chronic stimulation was necessary to maintain results in 29% of dysfunctional voiding and 50% of overactive bladder cases. CONCLUSIONS: Percutaneous tibial nerve stimulation is reliable and effective for nonneurogenic, refractory lower urinary tract dysfunction in children. Efficacy seems better in dysfunctional voiding than in overactive bladder cases. There is evidence that percutaneous tibial nerve stimulation should be part of the pediatric urology armamentarium when treating functional incontinence.

Bladder after total urogenital mobilization for congenital adrenal hyperplasia and cloaca--does it behave the same?

PURPOSE: Followup of total urogenital mobilization for persistent urogenital sinus is well established anatomically and functionally. Nevertheless, studies comparing bladder function in different subsets of patients with urogenital sinus, such as congenital adrenal hyperplasia and cloaca, are scant. MATERIALS AND METHODS: We reviewed the records of patients with congenital adrenal hyperplasia and cloaca who underwent total urogenital mobilization and urodynamics in the last 10 years. Those with a short urogenital sinus (less than 2.5 cm) not requiring an abdominal approach and without spinal dysraphism were selected for study. Urodynamics were performed postoperatively before and after toilet training, and compared between patients with congenital adrenal hyperplasia and cloaca. Methods, definitions and units conformed to International Continence Society/International Children's Continence Society standards. For the emptying phase we defined bladder outlet obstruction as maximum detrusor pressure greater than 70 cm H(2)O and underactive detrusor as maximum detrusor pressure less than 20 cm H(2)O plus post-void residual urine greater than 25 ml. RESULTS: Six patients with congenital adrenal hyperplasia and 6 with cloaca met study criteria. Three patients with congenital adrenal hyperplasia and 4 with cloaca underwent urodynamics before and after toilet training at a median age of 2 (range 2 to 4) and 5 years (range 3 to 8), respectively. Urodynamics were done in 1 patient with congenital adrenal hyperplasia before toilet training, and in 2 with congenital adrenal hyperplasia and 2 with cloaca after toilet training. All patients had normal urodynamics except 1 with congenital adrenal hyperplasia and detrusor overactivity, which normalized after toilet training. In all cloaca cases urodynamics were abnormal. Before toilet training bladder outlet obstruction was found in 2 patients, detrusor underactivity was found in 1 and detrusor overactivity was found in the remaining 1. After toilet training a detrusor underactivity pattern was found in 4 patients and bladder outlet obstruction was found in 2. All patients except 1 with cloaca had post-void residual urine before and after toilet training (median 100 ml, range 25 to 200). After toilet training all patients with congenital adrenal hyperplasia became spontaneously dry and all with cloaca were placed on clean intermittent catheterization. CONCLUSIONS: In the long term patients with cloaca show bladder outlet obstruction or underactive/acontractile detrusor patterns, which are not noted in patients with congenital adrenal hyperplasia. Therefore, in patients with cloaca urogenital sinus length may not be as good an indicator of functional results as it is in patients with congenital adrenal hyperplasia. Whether additional rectal dissection and repositioning surgical procedures in cloaca cases may have a role in explaining such a difference remains to be clarified.

Glossopexy as an alternative to aortopexy in infants with repaired esophageal atresia and upper airway obstruction.

BACKGROUND/PURPOSE: Clinical manifestations of airway obstruction in infants with repaired esophageal atresia or tracheoesophageal fistula (EA/TEF) are attributed conventionally to tracheomalacia. In the current study, the authors tested the hypothesis that a retrodisplacement of the tongue (glossoptosis), by causing a functional upper airway obstruction (obstructive apnea/hypopnea), may play a role in the pathogenesis of the respiratory problems. METHODS: The records of 5 infants with repaired EA/TEF and respiratory symptoms treated by glossopexy, which serves to anchor the tongue forward, were reviewed. Prompt improvement after glossopexy in clinical manifestations, in blood gas exchange, and in weight velocity was used to establish a primary cause-effect relationship between glossoptosis and respiratory problems. RESULTS: Before glossopexy, all infants presented with severe respiratory distress. Three infants had life-threatening events requiring recurrent or chronic airway intubation. Four infants had severe tracheomalacia, diagnosed at bronchoscopy. All infants presented obstructive apnea/hypopnea with desaturation. Body weight was below the third percentile in all infants. After glossopexy, 3 presented a marked clinical improvement associated with significant increase in mean oxygen saturation (88% +/- 0.4 SE v 95% +/- 0.3 SE; P <.001). In another infant, glossopexy allowed permanent decannulation but did not relieve the problem during crying or excitement when obstructive hypopnea with desaturation required supplemental oxygen. After glossopexy and despite subsequent aortopexy, the last infant could not be extubated and died later of mycotic sepsis. Autopsy results showed a vascular ring. In the 4 surviving infants, glossopexy was followed promptly by significant catch-up growth and subsequent normal growth velocity (P =.03). CONCLUSIONS: Present findings support the concept that, in some infants with EA/TEF, respiratory problems are mainly caused by recurrent obstructive apnea/hypopnea, which may be treated successfully by lip-tongue adhesion procedures. Severe airway obstruction, not relieved by glossopexy, requires aortopexy to reduce the respiratory load imposed by tracheomalacia.