Sarcoid-Associated Bilateral Multifocal Choroiditis Secondary to Adalimumab.

PURPOSE: To report a rare paradoxical development of systemic sarcoidosis in a patient taking adalimumab manifesting as multifocal choroidal infiltrates and seventh nerve palsy. METHODS: This was a single patient case report. RESULTS: A 30-year-old man with a history of psoriatic arthritis on adalimumab presented with intermittent fevers and headaches. Initial infectious serology and initial ophthalmic examination were within normal limits. Over the next month, he developed a seventh nerve palsy, unilateral decreased visual acuity, and bilateral multifocal choroidal infiltrates. The patient was diagnosed with systemic sarcoidosis secondary to tumor necrosis factor alpha (TNFα) inhibitor use after a hilar lymph node biopsy. Upon treatment with high-dose oral corticosteroids, the patient's symptoms and choroidal lesions significantly improved. CONCLUSION: This case report illustrates a rare presentation of ocular, neurologic, and systemic sarcoidosis presenting as a bilateral multifocal choroiditis and seventh nerve paresis in a patient treated with adalimumab. We highlight the importance of obtaining an ophthalmic evaluation in the management of this rare adverse effect of TNFα inhibitors.

Sacral nerve stimulation allows for decreased antegrade continence enema use in children with severe constipation.

BACKGROUND: Sacral nerve stimulation (SNS) can be beneficial for children with constipation, but no studies have focused on children with constipation severe enough to require antegrade continence enemas (ACEs). Our objective was to evaluate the efficacy of SNS in children with constipation treated with ACE. METHODS: Using a prospective patient registry, we identified patients <21years old who were receiving ACE prior to SNS placement. We compared ACE/laxative usage, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), Fecal Incontinence Severity Index (FISI), and Vancouver Dysfunctional Elimination Syndrome Score (DES) at baseline and progressive follow-up time intervals. RESULTS: Twenty-two patients (55% male, median 12years) were included. Median ACE frequency decreased from 7 per week at baseline to 1 per week at 12months (p<0.0001). Ten children (45%) had their cecostomy/appendicostomy closed. Laxative use, GSS, FIQL, and DES did not change. FISI improved over the first 12months with statistical significance reached only at 6months (p=0.02). Six (27%) children experienced complications after SNS that required further surgery. CONCLUSIONS: In children with severe constipation dependent on ACE, SNS led to a steady decrease in ACE usage with nearly half of patients receiving cecostomy/appendicostomy closure within 2years. LEVEL OF EVIDENCE: IV.

Robotic vs. Laparoscopic Sleeve Gastrectomy in Adolescents; Reality or Hype.

BACKGROUND: The rising prevalence of childhood obesity and concomitant increase in comorbid disease pose significant challenges for the health care system. While mounting evidence demonstrates the safety and efficacy of bariatric surgery for severely obese adolescents, the potential role of robotic technology has not been well defined. OBJECTIVE: The aim of this study was to establish the safety and efficacy of robotic-assisted laparoscopic sleeve gastrectomy (RSG) in treating severe adolescent obesity. In addition, 30-day outcomes and hospital charges were compared to subjects undergoing RSG versus laparoscopic sleeve gastrectomy (LSG). METHODS: A retrospective analysis of 28 subjects (14 LSG vs. 14 RSG) at a single institution was conducted. Data collection included demographics, body mass index, comorbidities, hospital length of stay (LOS), operative time, 30-day outcomes, and hospital charges. Analysis was performed using chi-square, Fisher's exact, and nonparametric Wilcoxon rank sum tests. RESULTS: There were no differences in subject demographics or comorbidities. While median operative time was longer for RSG vs. LSG (132 vs. 100 min, p = 0.0002), the median LOS for RSG compared to LSG was shorter (69.6 vs. 75.9 h, p = 0.0094). In addition, RSG-related hospital charges were higher ($56,646 vs. $49,498, p = 0.0366). No significant differences in post-operative outcomes or complications were observed. CONCLUSIONS: RSG is equally safe and efficacious when compared to LSG among adolescents. Similar to studies in adults, LOS is shortened while hospital charges are higher. Larger prospective studies are needed to gain insight regarding cost benefit ratios.

Sacral nerve stimulation: a promising therapy for fecal and urinary incontinence and constipation in children.

PURPOSE: This study describes our series of children with bowel and bladder dysfunction (BDD) treated with sacral nerve stimulation in order to begin to identify characteristics associated with better outcomes and guide future therapies. METHODS: Between May 2012 and February 2014, 29 patients were evaluated before and after sacral nerve stimulator (SNS) placement. A prospective data registry was developed that contains clinical information and patient-reported measures: Fecal Incontinence Qualify of Life Scale, Fecal Incontinence Severity Scale, PedsQL Gastrointestinal Symptom Scale, and Vancouver DES Symptom Scale. RESULTS: The median age of patients was 12.1 (interquartile range: 9.4, 14.3) years and the median follow-up period was 17.7 (12.9, 36.4) weeks. 93% had GI complaints and 65.5% had urinary symptoms while 7% had urologic symptoms only. The most common etiologies of BBD were idiopathic (66%) and imperforate anus (27%). Five patients required reoperation due to a complication with battery placement. Six of 11 patients (55%) with a pre-SNS cecostomy tube no longer require an antegrade bowel regimen as they now have voluntary bowel movements. Ten of eleven patients (91%) no longer require anticholinergic medications for bladder overactivity after receiving SNS. Significant improvements have been demonstrated in all four patient-reported instruments for the overall cohort. CONCLUSIONS: Early results have demonstrated improvements in both GI and urinary function after SNS placement in pediatric patients with bowel and bladder dysfunction.

Carbon dioxide monitoring during laparoscopic-assisted bariatric surgery in severely obese patients: transcutaneous versus end-tidal techniques.

Various factors including severe obesity or increases in intra-abdominal pressure during laparoscopy can lead to inaccuracies in end-tidal carbon dioxide (PETCO2) monitoring. The current study prospectively compares ET and transcutaneous (TC) CO2 monitoring in severely obese adolescents and young adults during laparoscopic-assisted bariatric surgery. Carbon dioxide was measured with both ET and TC devices during insufflation and laparoscopic bariatric surgery. The differences between each measure (PETCO2 and TC-CO2) and the PaCO2 were compared using a non-paired t test, Fisher's exact test, and a Bland-Altman analysis. The study cohort included 25 adolescents with a mean body mass index of 50.2 kg/m2 undergoing laparoscopic bariatric surgery. There was no difference in the absolute difference between the TC-CO2 and PaCO2 (3.2±3.0 mmHg) and the absolute difference between the PETCO2 and PaCO2 (3.7±2.5 mmHg). The bias and precision were 0.3 and 4.3 mmHg for TC monitoring versus PaCO2 and 3.2 and 3.2 mmHg for ET monitoring versus PaCO2. In the young severely obese population both TC and PETCO2 monitoring can be used to effectively estimate PaCO2. The correlation of PaCO2 to TC-CO2 is good, and similar to the correlation of PaCO2 to PETCO2. In this population, both of these non-invasive measures of PaCO2 can be used to monitor ventilation and minimize arterial blood gas sampling.

Benign and precursor lesions in the esophagus.

The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on the role of salivary stimulation and esophageal secretion of protective factors in prevention of adenocarcinoma sequelae in gastroesophageal reflux disease; the pediatric conditions associated with esophageal cancer; the relationship of achalasia and pseudoachalasia with esophageal cancer; the potential for malignant transformation in eosinophilic esophagitis and overlap syndromes; the role of lymphocytic esophagitis as an overlapping phenotype; the role of Barrett's esophagus as a premalignant condition; the indications and type of treatment of premalignant conditions of the esophagus; and the decision for use of endoscopical procedures in premalignant conditions of the esophagus.

Ventilation during laparoscopic-assisted bariatric surgery: volume-controlled, pressure-controlled or volume-guaranteed pressure-regulated modes.

INTRODUCTION: Managing ventilation and oxygenation during laparoscopic procedures in severely obese patients undergoing weight loss surgery presents many challenges. Pressure-controlled ventilation, volume-guaranteed (PCV-VG) is a dual-control mode of ventilation and an alternative to pressure (PC) or volume (VC) controlled ventilation. PCV-VG features a user-selected tidal volume target, that is auto-regulated and pressure controlled. We hypothesized that PCV-VG ventilation would provide improved oxygenation and ventilation during laparoscopic bariatric surgery with a lower peak inflating pressure (PIP) than either PC or VC ventilation. METHODS: This was a prospective cross-over cohort trial (n = 20). In random sequence each patient received the three modes of ventilation for 20 minutes during the laparoscopic portion of the procedure. For all modes of ventilation the goal tidal volume was 6-8 mL/kg, and the respiratory rate was adjusted to achieve normocarbia. The PIP, exhaled tidal volume, respiratory rate, and oxygen saturation were recorded every five minutes. At the end of 20 minutes, an arterial blood gas was obtained. Data were analyzed using a paired t-test. RESULTS: PCV-VG and PC ventilation both resulted in significantly lower PIP (cmH2O) than VC ventilation (30.5 ± 3.0, 31.6 ± 4.9, and 36.3 ± 3.4 mmHg respectively; p < 0.01 for PCV-VG vs. VC and PC vs. VC). There was no difference in oxygenation (PaO2), ventilation (PaCO2) or hemodynamic variables between the three ventilation modes. CONCLUSIONS: In adolescents and young adults undergoing laparoscopic bariatric surgery, PCV-VG and PC were superior to VC ventilation in their ability to provide ventilation with the lowest PIP.

Adolescent bariatric surgery program characteristics: the Teen Longitudinal Assessment of Bariatric Surgery (Teen-LABS) study experience.

The number of adolescents undergoing weight loss surgery (WLS) has increased in response to the increasing prevalence of severe childhood obesity. Adolescents undergoing WLS require unique support, which may differ from adult programs. The aim of this study was to describe institutional and programmatic characteristics of centers participating in Teen Longitudinal Assessment of Bariatric Surgery (Teen-LABS), a prospective study investigating safety and efficacy of adolescent WLS. Data were obtained from the Teen-LABS database, and site survey completed by Teen-LABS investigators. The survey queried (1) institutional characteristics, (2) multidisciplinary team composition, (3) clinical program characteristics, and (4) clinical research infrastructure. All centers had extensive multidisciplinary involvement in the assessment, pre-operative education, and post-operative management of adolescents undergoing WLS. Eligibility criteria and pre-operative clinical and diagnostic evaluations were similar between programs. All programs have well-developed clinical research infrastructure, use adolescent-specific educational resources, and maintain specialty equipment, including high weight capacity diagnostic imaging equipment. The composition of clinical team and institutional resources is consistent with current clinical practice guidelines. These characteristics, coupled with dedicated research staff, have facilitated enrollment of 242 participants into Teen-LABS.

Cardiovascular recovery following bariatric surgery in extremely obese adolescents: preliminary results using Cardiac Magnetic Resonance (CMR) Imaging.

PURPOSE: Baseline cardiovascular characteristics and longitudinal changes following weight loss surgery (WLS) in adolescents are not well defined. Recent data suggest that the use of transthoracic echocardiography (TTE) for preoperative cardiac assessment may provide suboptimal imaging fidelity secondary to excessive upper body adiposity. High fidelity imaging using cardiac magnetic resonance (CMR) is an extremely useful diagnostic tool. We report the use of CMR in a cohort of extremely obese adolescents undergoing WLS. METHODS: A retrospective analysis of adolescent WLS patients at a single institution was conducted. Data collection included mean age, sex, body mass index (BMI), and CMR measurements of left ventricular (LV) mass, LV end-diastolic volume (LVEDV), ejection fraction (EF), and myocardial perfusion reserve index (MPRI). Comparison of CMR results to normative data derived from lean subjects was performed. RESULTS: Ten subjects (9 female), with a mean age and BMI of 17.4 ± 1.9 years and 50.33 ± 10.21 kg/m(2) respectively, were studied. When compared to age, gender, and height matched normal weight (NW) controls, the obese (OB) subjects had evidence of increased LV mass (122 ± 25 g vs. 101 ± 10 g, OB vs. NW respectively, p<0.05), and increased LVEDV (156 ± 25 mL vs. 109 ± 9 mL, p<0.05), with an average EF of 61.5% ± 5% (range 52% to 67% vs. 71% to 74% expected EF for males and females, respectively, p=0.003). In addition, 60% of the OB subjects (6/10) demonstrated adenosine-induced sub-endocardial ischemia at baseline, the majority of whom underwent WLS (n=5) resulting in complete normalization of ischemia in 60% (3/5) and partial improvement in 40% (2/5). A reduction in mean LV mass (range 2 to 12 g) following WLS was observed. CONCLUSION: Extreme adolescent obesity is associated with significant cardiovascular abnormalities that include LV hypertrophy (i.e. increased LV mass) and LV dilatation. These findings, considered to be well-recognized cardiovascular disease risk factors in adults, were shown to be reversible after WLS in the small group of subjects studied here. Additional large-scale investigations designed to examine obesity-related cardiovascular disease in severely obese adolescents are required.

Efficacy of permanent gastric electrical stimulation for the treatment of gastroparesis and functional dyspepsia in children and adolescents.

PURPOSE: Permanent gastric electrical stimulation (GES) has been performed in adults as a treatment for gastroparesis and refractory nausea and vomiting in patients who have failed medical therapy. We assessed the feasibility and clinical outcomes of permanent GES in children. METHODS: Permanent GES was performed in 16 children (10 females/6 males), median age 15 years (range 4-19 years). All patients had chronic nausea and vomiting refractory to medical therapy and met ROME III criteria for functional dyspepsia. Symptoms, route for nutrition, and satisfaction with procedure were recorded before and after permanent GES. Statistical analysis was performed using paired Student's t test. RESULTS: After permanent GES, there was significant improvement in severity of vomiting (p=0.0001), frequency of vomiting (p=0.0003), frequency of nausea (p<0.0001), and severity of nausea (p<0.0001). At the time of follow-up, 13/16 were on oral feeds exclusively, two patients on oral plus G-tube feedings, and one patient on oral plus G-tube plus intermittent TPN. CONCLUSIONS: 1). Permanent GES improved health in children with functional dyspepsia and gastroparesis who fail medical therapy. 2). No serious adverse effects of permanent GES were noted. 3). Long-term efficacy and safety of GES therapy in children need to be established.

Fibrous soft tissue tumors: factors predictive of recurrence.

BACKGROUND/PURPOSE: Fibrous soft tissue tumors (FSTT) of infancy and childhood present a challenge to surgeons due to ambiguous histological interpretation, propensity for local recurrence, and uncertainty regarding appropriate therapy. METHODS: Records were reviewed for patients diagnosed and treated for FSTT at our institution from 1992 to 2010. Data included demographics, tumor location, operative treatment, histopathology, margin status, mitotic index, and recurrence. RESULTS: Eighty-six patients underwent treatment of FSTT. Mean follow-up time was 62 months (range 1 to 201 months). There were 11 recurrences (13%), with a mean time to recurrence of 23 months. Extremity and digital lesions were most likely to recur with 21% of such lesions recurring compared to 7% of lesions located elsewhere. Recurrences were seen more frequently in older children. Despite the presence of positive margins in 92% of cases, margin status, histopathology, and mitotic index were not significant predictors of recurrence. CONCLUSIONS: Digital and extremity fibrous soft tissue tumors and tumors of older children have an increased risk for local recurrence. Margin status did not predict recurrence rates in this series. While the surgical goal is to obtain complete local resection with clear margins, this may be challenging in particular locations and may not ultimately impact recurrence.

Childhood obesity: a risk factor for injuries observed at a level-1 trauma center.

PURPOSE: Obesity is an independent risk factor in trauma-related morbidity in adults. The purpose of this study was to investigate the effect of obesity in the pediatric trauma population. METHODS: All patients (6-20 years) between January 2004 and July 2007 were retrospectively reviewed and defined as non-obese (body mass index [BMI] <95th percentile for age) or obese (BMI > or =95th percentile for age). Groups were compared for differences in demographics, initial vital signs, mechanisms of injury, length of stay, intensive care unit stay, ventilator days, Injury Severity Score, operative procedures, and clinical outcomes. RESULTS: Of 1314 patients analyzed, there were 1020 (77%) nonobese patients (mean BMI = 18.8 kg/m(2)) and 294 (23%) obese patients (mean BMI = 29.7 kg/m(2)). There was no significant difference in sex, heart rate, length of stay, intensive care unit days, ventilator days, Injury Severity Score, and mortality between the groups. The obese children were significantly younger than the nonobese children (10.9 +/- 3.3 vs 11.5 +/- 3.5 years; P = .008) and had a higher systolic blood pressure during initial evaluation (128 +/- 17 vs 124 +/- 16 mm Hg, P < .001). In addition, the obese group had a higher incidence of extremity fractures (55% vs 40%; P < .001) and orthopedic surgical intervention (42% vs 30%; P < .001) but a lower incidence of closed head injury (12% vs 18%; P = .013) and intraabdominal injuries (6% vs 11%; P = .023). Evaluation of complications showed a higher incidence of decubitus ulcers (P = .043) and deep vein thrombosis (P = .008) in the obese group. CONCLUSION: In pediatric trauma patients, obesity may be a risk factor for sustaining an extremity fracture requiring operative intervention and having a higher risk for certain complications (ie, deep venous thrombosis [DVT] and decubitus ulcers) despite having a lower incidence of intracranial and intraabdominal injuries. Results are similar to reports examining the effect(s) of obesity on the adult population.

V.A.C. Therapy in the management of paediatric wounds: clinical review and experience.

Usage of negative pressure wound therapy (NPWT) in the management of acute and chronic wounds has grown exponentially in the past decade. Hundreds of studies have been published regarding outcomes and methods of therapy used for adult wounds. This treatment is increasingly being used to manage difficult-to-treat paediatric wounds arising from congenital defects, trauma, infection, tumour, burns, pressure ulceration and postsurgical complications in children, although relatively few studies have been aimed at this population. Given the anatomical and physiological differences between adults and children, a multidisciplinary expert advisory panel was convened to determine appropriate use of NPWT with reticulated open cell foam (NPWT/ROCF) as delivered by Vacuum Assisted Closure (V.A.C. Therapy, KCI Licensing, Inc., San Antonio, TX) for the treatment of paediatric wounds. The primary objectives of the expert advisory panel were to exchange state-of-practice information on paediatric wound care, review the published data regarding the use of NPWT/ROCF in paediatric wounds, evaluate the strength of the existing data and establish guidelines on best practices with NPWT/ROCF for the paediatric population. The proposed paediatrics-specific clinical practice guidelines are meant to provide practitioners an evidence base from which decisions could be made regarding the safe and efficacious selection of pressure settings, foam type, dressing change frequency and use of interposing contact layer selections. The guidelines reflect the state of knowledge on effective and appropriate wound care at the time of publication. They are the result of consensus reached by expert advisory panel members based on their individual clinical and published experiences related to the use of NPWT/ROCF in treating paediatric wounds. Best practices are described herein for novice and advanced users of NPWT/ROCF. Recommendations by the expert panel may not be appropriate for use in all circumstances. Decisions to adopt any particular recommendation must be made by the collaborating medical team, including the surgeon and wound care specialist based on available resources, individual patient circumstances and experience with the V.A.C. Therapy System.

Pediatric thoracic outlet syndrome: a disorder with serious vascular complications.

BACKGROUND: Thoracic outlet syndrome (TOS), caused by compression of the neurovascular structures between the clavicle and scalene muscles, typically presents with neurologic symptoms in adults. We reviewed our experience with 25 adolescents and propose a diagnostic/treatment algorithm for pediatric TOS. METHODS: From 1993 to 2005, 25 patients were treated with TOS. A retrospective chart review was performed with institutional review board approval. Demographics, clinical presentation, diagnostic studies, and treatment were evaluated. RESULTS: Seven male (28%) and 18 female (72%) patients presented between the ages of 12 to 18 years. Thirteen (52%) had vascular TOS (11 venous, 2 arterial), 11 (44%) had neurologic TOS, and 1 had both. Vascular TOS included subclavian vein thrombosis (7), venous impingement (4), and arterial impingement (2). Three patients had hypercoagulable disorders, and 6 had effort thrombosis. Venography was diagnostic in 10 cases. Neurogenic TOS was diagnosed by clinical symptoms. Five patients with subclavian vein thrombosis underwent thrombolysis, with 3 maintaining long-term patency. Of 25 patients, 24 underwent transaxillary first rib resection. CONCLUSION: Vascular complications are more common in adolescents with TOS than in adults. A diagnostic/treatment algorithm includes urgent venography and thrombolysis for venous TOS and a workup for hypercoagulability. Neurogenic TOS is diagnosed clinically, whereas other studies are rarely beneficial.

Catheter-based drainage and agitation for definitive cytological diagnosis of a ciliated hepatic foregut cyst in a child.

Ciliated hepatic foregut cyst (CHFC) is an uncommon lesion, which rarely presents in the pediatric population. It is congenital in origin and manifests because of the migration of a bronchiolar bud of the foregut through the pleuroperitoneal canal. CHFC is accompanied by a broad list of differential diagnoses and is difficult to diagnose by means of radiologic evaluation. Therefore, fine-needle aspiration with cytology has been used in an attempt to diagnose this lesion. In previously reported cases, this approach has been moderately successful in the diagnosis of CHFC, while in others, no definitive diagnosis was achieved because of the retrieval of scattered, irregular cells insufficient for cytologic evaluation. We report a case of a 16-year-old girl who presented with a CHFC and discuss a promising alternative for obtaining large, intact cellular specimens to facilitate cytologic evaluation and definitive diagnosis.

Wound management with vacuum-assisted closure: experience in 51 pediatric patients.

BACKGROUND/PURPOSE: Soft tissue loss from infectious, vascular, and traumatic disorders often results in poor healing, painful wound care, and the need for repeated operations. This retrospective study evaluates a single-institutional experience with negative pressure therapy (NPT), using the vacuum-assisted closure (VAC) device in a group of children with diverse soft tissue problems. METHODS: The medical records of 51 patients treated with NPT from January 2000 to July 2003 were reviewed for demographics, diagnosis, duration of VAC therapy, wound closure, recurrent disease, and complications. RESULTS: Patients were classified by diagnosis: group 1: pilonidal disease (n = 21, primary = 6 and recurrent = 15); group 2: sacral and extremity ulcers (n = 9); group 3: traumatic soft tissue wounds (n = 9); and group 4: extensive tissue loss (n = 12) from the abdominal wall (n = 7), perineum (n = 2), thigh (n = 2), and axilla (n = 1). Group 1 had an average age of 16 years (range, 10-20 years), 67% were obese, and had an average length of follow-up of 13 months (range, 8-36 months). VAC was placed in the operating room in 95% with subsequent outpatient care that included dressing change 3 times weekly. Healing occurred in all patients with primary disease at an average of 37 days. For patients with recurrent disease, 12 healed at an average of 48 days and 3 developed recurrent sinuses. Group 2 was treated with VAC as a bridge to skin grafting or flap closure. All children in group 3 achieved healing without skin grafting at an average of 10 days and with acceptable cosmesis. Negative pressure therapy in group 4 was the only wound treatment in 10 patients and adjunctive to operative closure in 2. Complications from VAC occurred in 5 patients: retained sponge in 2 and device malfunction in 3. CONCLUSIONS: Negative pressure therapy offers a safe, cost-effective alternative to traditional complex wound care in children. Its advantages are less frequent dressing changes, outpatient management, resumption of daily activities including return to school, and a high degree of patient tolerance.

Current controversies in perinatal care: fetal versus neonatal surgery.

Fetal surgery has become a viable option for many parents whose unborn infants have congenital anomalies. However, this approach is best suited to specific circumstances and specific babies. Careful prenatal care and early diagnosis ensure that this option is available to those who can benefit from the intervention.