Localized intravascular coagulopathy (LIC) has been described in adults with venous malformation (VM) but rarely reported in children. This study aims to determine the prevalence of LIC in children with VM and associated risk factors. Patients younger than 18 years with VM from 2010 to 2014 were reviewed. Diagnosis was confirmed by Doppler ultrasound and/or magnetic resonance imaging. Demographics data and VM characteristics including volume, site, extension, painful symptoms, and palpable phleboliths were studied. Plasma D-dimer level of greater than 500 ng/mL was considered as abnormal. Total 24 children were included, of whom 8 were boys. Median age of presentation was 9 months (range: 0-12 years). Head-and-neck VM occurred in 17 (70.8%) patients and 3 (12.5%) had multifocal lesions. Seven (29.2%) patients had VM volume greater than 10 mL. Five (20.8%) patients had painful symptoms. Palpable phleboliths were found in two patients. Plasma D-dimer was raised in eight cases (33.3%). One patient with Klippel-Trenaunay syndrome (KTS) had D-dimer level of 5,000 ng/mL. Raised D-dimer was found in 23.5% of small VM (volume < 10 mL) and 57.1% of large VM ( = 0.167). D-dimer was significantly raised in multifocal VM ( = 0.028) and showed increasing trend in lesions with palpable phleboliths ( = 0.101). All patients had sclerotherapy performed with indications (cosmesis 41.7%, enlarging lesion 29.2%, pain 20.8%, bleeding 8.3%). Perioperatively, bolus intravenous fluid and mannitol were given to selected patients. All patients had VM volume reduction after sclerotherapy. There were no major thromboembolic complications. LIC with raised D-dimer level occurred in one-third of pediatric VM. It was more common in large, multifocal VM and in those with palpable phleboliths or KTS.
Objective: Idiopathic constipation in children can be difficult to manage with conventional therapies. The present study aimed to evaluate the efficacy of adjunctive botulinum toxin injection in children attending our regional centre with refractory idiopathic constipation associated with internal anal sphincter hypertonicity. Methods: Children suffering refractory constipation after a minimum of 3 months bowel training, dietary and laxative treatments were evaluated by anorectal manometry. Those with idiopathic anal sphincter hypertonicity (resting pressure > 60 mmHg with normal recto-anal reflex) were recruited for botulinum toxin injection. Pretreatment evaluation also included bowel-function score and the degree of megarectum as measured transpubically by ultrasound. Each child received 60 units of Botox ® intrasphincterically under general anaesthesia. Follow-up evaluations were carried out at 6 weeks and 6 months. Results: Eleven Chinese children, mean age 6.1 years, underwent botulinum toxin treatment. The mean sphincter pressure decreased from 73 mmHg to 55 mmHg and the mean symptom score improved from 2.5 to 6.3 (P = 0.003, Wilcoxon signed rank-sum test). The mean rectal diameter decreased from 3.7 cm to 3.4 cm. Findings were sustained at 6 months with no treatment complications. Conclusion: Botulinum toxin intrasphincteric injection is a safe, non-invasive and effective adjunct to conventional therapies in managing children with refractory idiopathic constipation associated with sphincter hypertonicity. Timely definitive therapy to facilitate pain-free habitual defecation plays an important role in these children in their potty training, as demonstrated by the sustained 'sphincterotomy' effects outlasting the transient toxin paralysis.
Aim: Urodynamic study is a functional assessment of the lower urinary tract. It has an essential role in the evaluation of paediatric patients with lower urinary tract symptoms, be it due to neurogenic or nonneurogenic causes. Since 2013, we have introduced wireless ambulatory form of the study. In the present study, we performed a baseline review of our paediatric urodynamic service within a 6-year period to determine its indications, outcomes and complications. Patients and Methods: A retrospective review of all urodynamic studies performed between August 2011 and April 2017 in patients <18 years of age was performed. Demographics, including age and sex, and indications were collected. Urodynamic parameters, including type of catheter used, presence of detrusor overactivity, bladder compliance, end-fill detrusor pressure, maximum voiding detrusor pressure, postvoid residual bladder volume and bladder emptying efficiency, were collected. Complications from either catheter insertion or the urodynamic study itself were documented. Results: Sixty-seven studies were done between the study period in 55 patients (16 females and 39 males). Fifty-eight ambulatory studies with natural and conventional fill were performed since 2013. The median age at study was 2.1 years (range 0.1-17 years). Indications included myelodysplasia (61 per cent, n = 41 studies), bilateral high-grade vesicoureteric reflux (18 per cent, n = 12), dysfunctional voiding (15 per cent, n = 10) and patient with posterior urethral valve (6 per cent, n = 4). Suprapubic catheters were used in 62 studies in patients with urethral sensation, and transurethral catheters were used in five studies. Overall, 53 studies were considered abnormal (79.1 per cent). No complications resulted from suprapubic catheter insertion or urodynamic studies. Conclusion: Ambulatory urodynamic studies using our unit's protocol remain safe and child friendly, with minimal complications and high patient and parent satisfaction.
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