Trigeminal neuralgia in children and adolescents: Experience of a tertiary pediatric headache clinic.

OBJECTIVES: To describe the characteristics and treatment of trigeminal neuralgia in children attending a dedicated pediatric headache clinic. BACKGROUND: Data on trigeminal neuralgia as a cause of headache are largely derived from adult studies. Little is known about the etiology, symptoms, treatment, and outcome of the disorder in children and adolescents. METHODS: A case series study was undertaken. The database of a headache clinic within a tertiary, university-affiliated, pediatric medical center was searched for all patients aged 3-18 years presenting with clinical and epidemiological features of trigeminal neuralgia or trigeminal neuropathy from January 2015 to December 2019. The diagnosis was revised for the present study according to the criteria of the International Classification of Headache Disorders, third edition. Data on demographic parameters, clinical symptoms, treatment, and outcome were collected from the medical files. RESULTS: Of the 1040 patients who presented to our clinic during the study period, five (0.5%) were diagnosed with trigeminal neuralgia. Mean patient age was 15.1 ± 3.0 years (range 9.5-17.5; 95% CI 10.8-18.9). All had idiopathic type: purely paroxysmal in one and with concomitant continuous pain in four. Findings on herpes serology in all five and magnetic resonance imaging were normal in four patients. In the fifth, a vascular ring was noted from the superior cerebellar artery around the right trigeminal nerve without radiologic evidence of vascular nerve compression. All patients were initially treated with carbamazepine: one reported partial relief, two did not respond, and two had severe adverse effects. Three patients were switched to gabapentin but only one responded well. Three patients were treated with nerve block. CONCLUSION: Trigeminal neuralgia accounted for only a small proportion of patients seeking treatment for headache in pediatric headache clinic over a 5-year period. Unlike findings in adults, vascular compression was not the underlying mechanism in any of our patients. The response to pharmacologic treatment was poor. Nerve block may serve as an alternative when pharmacologic treatment fails.

Small-fiber neuropathy associated with autoinflammatory syndromes in children and adolescents.

INTRODUCTION: Small-fiber neuropathy is rare in children. It has been associated with several autoimmune disorders, but there are no reports of an autoinflammatory etiology. METHODS: The data of four children/adolescents presenting with erythromelalgia and neuropathic pain from 2014 to 2019 were collected retrospectively from the electronic database of a pediatric medical center. RESULTS: Results of clinical and/or electrophysiological evaluation excluded large nerve fiber involvement. Skin biopsy results confirmed small-fiber neuropathy. According to genetic analysis, two patients were heterozygous and one was homozygous for mutations in the familial Mediterranean fever (MEFV) gene. Behcet disease was diagnosed in the fourth patient. Treatment with anti-interleukin-1 agents, intravenous immunoglobulin, and glucocorticoids was beneficial. DISCUSSION: The diagnosis of small-fiber neuropathy should be considered in children/adolescents presenting with erythromelalgia. A thorough investigation is required to reveal the underlying disorder. Clinicians should be alert to the peripheral neurological manifestations of autoinflammatory syndromes because effective treatments are available.

Neuropathic pain other than CRPS in children and adolescents: incidence, referral, clinical characteristics, management, and clinical outcomes.

BACKGROUND AND OBJECTIVES: Chronic pain in children and adolescents is common, but proportion of neuropathic pain (NP), a heterogeneous group of diseases with major impact on health-related quality of life, significant economic burden, and limited treatment options, is unclear. Many studies have focused only on complex regional pain syndrome (CRPS). Our aim was to examine the incidence, clinical features, management, and outcome of non-CRPS NP in patients referred to a chronic pediatric pain clinic (CPPC) at a tertiary-care hospital. METHODS: Retrospective analyses of the patient's files with non-CRPS NP from 2008 until 2012. RESULTS: Twenty patients (9.9-22.0 years; 10.7% of new referrals) were treated with non-CRPS NP (postoperative 8/20, trauma-related 5/20, disease-related 7/20). The number of consultations performed and the number of medications used before CPPC were significantly higher than in CPPC (Z = 0.75, P = 0.005; Z = 1.68, P = 0.003; respectively, Wilcoxon test). The number of diagnostic procedures was not statistically significant. Invasive treatments were used in 50% of patients. Full/partial recovery was accomplished in 95%. anova with repeated measures yielded a highly significant difference between the initial and final visual analog scale (VAS) scores (8.2 ± 1.3; 1.19 ± 2.01, respectively; P < 0.001), and no effect of age, gender, time needed for referral to CPPC, and patient's categories on the change in VAS. CONCLUSIONS: Better understanding of the medical profile of pediatric patients with non-CRPS NP is crucial to timely and correct diagnosis and effective management, but even children with delayed diagnosis still have a good outcome. The management of this condition by an experienced team is recommended.

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases.

OBJECTIVE/AIM: To compare hemodynamic parameters in infants with congenital heart disease (CHD) undergoing noncardiac surgery (NCS) under awake spinal anesthesia (SA) with controls without CHD also undergoing SA. BACKGROUND: NCS poses a twofold higher mortality risk in infants with CHD. SA might be a good alternative to general anesthesia (GA) in this setting. METHODS: The files of 84 infants were reviewed; 42 had CHD and 42 were controls without CHD. Primary outcome measures were percent decrease in mean arterial pressure (%MAP decrease) and heart rate (%HR decrease)from baseline to the lowest intraoperative value. One-way analysis of variance(ANOVA), ANOVA with repeated measures, Pearson chi-square test, Fisher's exact test, and Pearson correlation were used for statistical analysis.Time to discharge was analyzed with the nonparametric Mann­Whitney U-test. RESULTS: There were no significant between-group differences in %MAP decrease and %HR decrease; no significant associations and correlations between %MAP decrease or %HR decrease and other variables; and no correlation between %MAP decrease and %HR decrease. A %MAP decrease of >20% was documented in 11 patients with CHD (26.2%) and 10 controls (23.8%); a lowest intraoperative HR of <100 b.min)1 was recorded in two study patients (4.8%) and four controls (9.5%) (P = NS for both). There were no cases of high SA or conversion to GA and no need for mechanical ventilation or inotropic support intra/postoperatively. CONCLUSIONS: These preliminary findings show that hemodynamic parameters in infants with CHD undergoing NCS under awake SA are not different from controls without CHD and that SA appears to be safe in infants with CHD.

Duloxetine contributing to a successful multimodal treatment program for peripheral femoral neuropathy and comorbid 'reactive depression' in an adolescent.

In the United States, duloxetine has been approved for the treatment of major depressive disorder, diabetic peripheral neuropathic pain and fibromyalgia in the adult population. Data regarding the use of duloxetine in the pediatric population, however, are very limited. Femoral nerve injury is a rare complication of cardiac catheterization. In the case described, duloxetine contributed to a successful multimodal treatment program for peripheral neuropathic pain due to femoral neuropathy in an adolescent with 'reactive depression' and conversion symptoms. To the best of the authors' knowledge, the present article is only the third such report on this dual use of duloxetine in children and adolescents, and the first report of such treatment following femoral neuropathy induced by cardiac catheterization.

Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome.

Providing anesthesia to patients with Brachmann-de Lange syndrome (BdLS) may be challenging, mainly because of intubation difficulty, gastroesophageal reflux disease, and aspiration complications. The use of spinal anesthesia (SA) in this population has not been reported. We report the uneventful administration of awake SA to a 7-month-old girl with BdLS who was scheduled for rectal biopsy. The current literature is reviewed to discuss the indications for SA in those patients.

Sub-Tenon's ropivacaine block for pain relief after primary strabismus surgery.

PURPOSE: Pain is the main cause of patient distress/dissatisfaction after strabismus surgery. The aim of the study was to evaluate the effect of sub-Tenon's block with ropivacaine at the end of strabismus surgery on post-operative pain. METHODS: A prospective trial was conducted in 79 patients (age 1.0-65 years) scheduled for outpatient primary strabismus surgery with fixed sutures under general anesthesia (GA) at a major tertiary hospital. Half the patients were randomly allocated to receive sub-Tenon's block with ropivacaine 0.2% at conclusion of the operation. Primary outcome measures were visual analog scale (VAS) scores at arrival to the post-anesthesia care unit (PACU), at discharge 3 hr later, 12-16 hr post-operatively, and 24 hr post-operatively. Supplemental analgesia requirements and patient satisfaction were recorded as well. Data were presented as median (range). Mann-Whitney test, Pearson chi(2)-test or Fisher's exact test was used for statistical analysis; p

Impact of spinal anesthesia for open pyloromyotomy on operating room time.

PURPOSE: When pyloromyotomy for hypertrophic pyloric stenosis (HPS) is performed under general anesthesia, metabolic abnormalities and fluid deficits coupled with residual anesthetics may increase the risk of postoperative apnea, thereby, prolonging operating room time and delaying extubation. Spinal anesthesia has been found to reduce the rate of postoperative apnea in high-risk infants. The aim of the study was to evaluate the effect of spinal vs general anesthesia on operating room time in infants undergoing open pyloromyotomy. METHODS: Data for 60 infants who underwent pyloromyotomy under spinal (n = 24) or general (n = 36) anesthesia at a tertiary pediatric medical center were derived from the computerized database. Primary outcome measures were total operating room time, procedure duration, anesthesia release time, wake-up time, and anesthesia control time (anesthesia release plus wake-up). Nonparametric Mann-Whitney test was used for statistical analysis, and Levene's test was used to assess the equality of variances in samples; P

A vein entry indicator device for facilitating peripheral intravenous cannulation in children: a prospective, randomized, controlled trial.

BACKGROUND: Vascular access is often technically difficult in children because of the small caliber and impalpability of the veins. In this study, we sought to determine if use of the Vein Entry Indicator Device (VEIDtrade mark) in children facilitates peripheral venous access. METHODS: Two-hundred-two healthy (ASA grade I and II) children scheduled for same-day surgery at a major tertiary hospital in Israel were randomly allocated to undergo VEID-assisted or standard peripheral venous cannulation. All cases involved the insertion of a 22-gauge cannula into an upper limb vein. Primary outcome measures were number of attempts to successful cannulation, rate of success at first attempt, and time required for insertion. The data were presented as mean (sd). Analyses of variance and Pearson chi(2) test or Fisher's exact test were used to compare the groups; forward stepwise logistic regression was used to identify the three variables (age, vein assessment category, use of the VEID) significantly associated with a successful first attempt. A P value of < or =0.05 was considered significant. RESULTS: Successful cannulation was achieved at the first attempt in the majority of patients in both groups. Two attempts were needed in 8% of the VEID group and 28% of the control group, and 3 attempts were needed in 1% and 3%, respectively (P < 0.01). Analysis by vein assessment category yielded a similar rate of successful first-attempt cannulations in the two groups for easy veins. However, for the difficult and intermediate categories, the rate was 89.7% in the VEID group compared to 23.3% in the control group (P < 0.001). The fewer number of attempts in the study group was associated with a shorter time from the start of the search for an appropriate vein to successful cannulation (9.1 s versus 22.5 s in the control group). CONCLUSIONS: The VEID facilitates the insertion of peripheral venous cannulas in healthy children with intermediate/difficult veins undergoing same-day surgery, reducing the number of attempts and the overall time required.

Complex regional pain syndromes in children and adolescents.

BACKGROUND: The purpose of the present paper was to assess efficiency of treatment and long-term functional outcome of complex regional pain syndromes (CRPS) in children who were treated in the chronic pain clinic at a major tertiary hospital in Israel. METHODS: The files of 14 children with CRPS were analyzed retrospectively. Demographic data, initiating event, referring source, time needed for referral to pain clinic, clinical evaluation, treatment, recurrence and complications were recorded. RESULTS: Fourteen children with CRPS types I and II were included in the study. Girls were affected in 71%. Lower extremities were affected in 57%. The median time from onset of symptoms to seeking medical help was 4.46 weeks (range 2-82 weeks). The median time to referral to pain clinic was 24.51 weeks (range 1.2-94). In 45% the referral source was the pediatrician. A total of 85.8% of patients were referred to various consultations before the pain clinic. Most children had reduced pain and improved function on non-invasive treatment approach. Invasive treatments were used in 28.5%. Full or partial recovery was accomplished in 93%. Recurrence was observed in 29%. CONCLUSIONS: CRPS in children and adolescents is still underdiagnosed, although many of the epidemiologic features of pediatric CRPS are similar in different countries/cultures. Early recognition and management is the major factor in improving outcome and preventing resistant CRPS, but even children with delayed diagnosis still have a good outcome. The management of this disease by an experienced multidisciplinary team is recommended. Because psychosocial factors play an important role, it is recommended to provide psychological evaluation and cognitive behavioral treatment as soon as possible.

Spinal anesthesia in neonates and infants - a single-center experience of 505 cases.

BACKGROUND: Our aim was to assess the safety and efficacy of spinal anesthesia (SA) in newborns and infants undergoing surgery appropriate for this technique. METHODS: The files of 505 patients who underwent surgery under spinal anesthesia since 1998 at a major tertiary hospital in Israel were analyzed retrospectively. SA was performed with bupivacaine 5 mgxml(-1) by attending pediatric anesthesiologists or an anesthesia resident. Demographic data, prematurity history, comorbidities, technical data, cardiovascular stability, complications and supplementary drugs were documented. The surgeon assessed the quality of anesthesia at the end of surgery. RESULTS: Appropriate SA was achieved in 95.3% of cases; in 69.9% at the first attempt. The mean number of attempts per patient was 1.41 and mean dose of bupivacaine was 0.66 +/- 0.16 mgxkg(-1). Intravenous sedation, usually with midazolam (dose 0.1-0.2 mgxkg(-1)) was required in 28.1% of children because of crying/restlessness. Intraoperative conversion to general anesthesia was necessary in five patients (1.04%). The main side effect was bradycardia (<100 bxmin(-1)) without desaturation which occurred in nine patients (1.8%). In three patients (0.62%), high spinal block occurred without bradycardia and hypotension. None of the patients had postoperative meningitis. CONCLUSIONS: SA is safe and effective in newborns and infants undergoing low abdominal, perineal and orthopedic surgery. In order to save time, our advice is to attempt SA after the surgeon is scrubbed, and minimize surgical teaching activity. The need to deal with a small and sometimes sick patient independent of the type of anesthesia requires the presence of an experienced pediatric anesthesiologist.

Anesthesia management for the child with Sanjad-Sakati syndrome.

Sanjad-Sakati syndrome (SSS) is a rare genetic disorder characterized by congenital hypoparathyroidism, hypocalcemia and hyperphosphatemia, seizures, severe intrauterine and postnatal growth failure, dwarfism, mental retardation, dysmorphic features including retromicrognathia and abnormal dentition and increased susceptibility to infection. It is mainly confined to children in the Middle-East countries. We report the anesthesia management of a 12-year-old boy with SSS for dental treatment, and discuss the anesthesia implications of this disorder.

Spinal anesthesia in infants with ventriculoperitoneal shunt: report of five cases and review of literature.

We describe five cases of children with ventriculoperitoneal shunt who underwent abdominal and perineal procedures under spinal anesthesia. Four of them had been born prematurely, and all had suffered from severe neonatal complications. All of our patients suffered from severe respiratory impairment, that had required mechanical ventilation, and three of them suffered additionally from apnea of prematurity. Four patients had ventriculoperitoneal shunt inserted because of obstructive hydrocephalus and one because of congenital central nervous system anomalies. Two underwent subsequently shunt revision. The benefits of spinal anesthesia in this high-risk population are described. The risks of spinal anesthesia in the presence of a ventricular shunt device, especially infection and dural leakage, are discussed, and the literature about this topic briefly reviewed.