Impact of microscopic deposits of yolk sac tumor on recurrence of mature sacrococcygeal teratoma.

INTRODUCTION: Sacrococcygeal teratomas (SCT) with malignant histology frequently recur and are treated aggressively, but risk factors and surveillance protocols are less established for mature tumors. In particular, prior studies have not investigated whether microscopic deposits of yolk sac tumor (YST) in otherwise mature teratomas lead to higher recurrence rates. METHODS: We reviewed patients with mature SCTs resected at our institution from 2011 to 2021 and analyzed tumor characteristics, treatment, and outcomes. RESULTS: We identified 56 patients with mature SCT, of which 9 (16%) demonstrated microscopic YST. Following surgery, 7/56 (13%) patients developed local recurrence at a mean of 1.2 ± 0.7 years, while no patients developed metastases. Recurrence was more likely in patients with microscopic YST [5/9 (56%) vs. 2/47 (4%), p = 0.021] and positive margins [6/24 (35%) vs. 1/32 (3.1%), p = 0.030]. A solid tumor component tended to increase recurrence risk as well [6/29 (21%) vs. 1/27 (4%), p = 0.053]. Five patients demonstrated malignant recurrence and were all detected by a rising alpha-fetoprotein (AFP), while two patients demonstrated recurrence of mature teratoma and were detected on surveillance magnetic resonance imaging (MRI). CONCLUSIONS: Microscopic foci of YST may increase recurrence risk for patients with mature SCT. Such patients might benefit from closer postoperative surveillance with serial AFP measurements and MRI.

Percutaneous biopsy for the diagnosis, risk stratification, and molecular profiling of neuroblastoma: A single-center retrospective study.

PURPOSE: To determine whether percutaneous core needle biopsy (PCNB) is adequate for the diagnosis and full molecular characterization of newly diagnosed neuroblastoma. MATERIALS AND METHODS: Patients with newly diagnosed neuroblastoma who underwent PCNB in interventional radiology at a single center over a 5-year period were included. Pre-procedure imaging and procedure details were reviewed. Rates of diagnostic success and sufficiency for International Neuroblastoma Pathology Classification (INPC), risk stratification, and evaluation of genomic markers utilized in the Children's Oncology Group risk stratification, and status of the anaplastic lymphoma kinase (ALK) gene were assessed. RESULTS: Thirty-five patients (13 females, median age 2.4 years [interquartile range, IQR: 0.9-4.4] and median weight 12.4 kg [IQR: 9.6-18]) were included. Most had International Neuroblastoma Risk Group Stage M disease (n = 22, 63%). Median longest axis of tumor target was 8.8 cm [IQR: 6.1-12]. A 16-gauge biopsy instrument was most often used (n = 20, 57%), with a median of 20 cores [IQR: 13-23] obtained. Twenty-five specimens were assessed for adequacy, and 14 procedures utilized contrast-enhanced ultrasound guidance. There were two post-procedure bleeds (5.7%). Thirty-four of 35 procedures (97%) were sufficient for histopathologic diagnosis and risk stratification, 94% (n = 32) were sufficient for INPC, and 85% (n = 29) were sufficient for complete molecular characterization, including ALK testing. Biologic information was otherwise obtained from bone marrow (4/34, 12%) or surgery (1/34, 2.9%). The number of cores did not differ between patients with sufficient versus insufficient biopsies. CONCLUSION: In this study, obtaining multiple cores with PCNB resulted in a high rate of diagnosis and successful molecular profiling for neuroblastoma.

Long-term follow-up of patients with intermediate-risk neuroblastoma treated with response- and biology-based therapy: A report from the Children's Oncology Group study ANBL0531.

BACKGROUND: We previously reported excellent three-year overall survival (OS) for patients with newly diagnosed intermediate-risk neuroblastoma treated with a biology- and response-based algorithm on the Children's Oncology Group study ANBL0531. We now present the long-term follow-up results. METHODS: All patients who met the age, stage, and tumor biology criteria for intermediate-risk neuroblastoma were eligible. Treatment was based on prognostic biomarkers and overall response. Event-free survival (EFS) and OS were estimated by the Kaplan-Meier method. RESULTS: The 10-year EFS and OS for the entire study cohort (n = 404) were 82.0% (95% confidence interval (CI), 77.2%-86.9%) and 94.7% (95% CI, 91.8%-97.5%), respectively. International Neuroblastoma Staging System stage 4 patients (n = 133) had inferior OS compared with non-stage 4 patients (n = 271; 10-year OS: 90.8% [95% CI, 84.5%-97.0%] vs 96.6% [95% CI, 93.9%-99.4%], p = .02). Infants with stage 4 tumors with ≥1 unfavorable biological feature (n = 47) had inferior EFS compared with those with favorable biology (n = 61; 10-year EFS: 66.8% [95% CI, 50.4%-83.3%] vs 86.9% [95% CI, 76.0%-97.8%], p = .02); OS did not differ (10-year OS: 84.4% [95% CI, 71.8%-97.0%] vs 95.0% [95% CI, 87.7%-100.0%], p = .08). Inferior EFS but not OS was observed among patients with tumors with (n = 26) versus without (n = 314) 11q loss of heterozygosity (10-year EFS: 68.4% [95% CI, 44.5%-92.2%] vs 83.9% [95% CI, 78.7%-89.2%], p = .03; 10-year OS: 88.0% [95% CI, 72.0%-100.0%] vs 95.7% [95% CI, 92.8%-98.6%], p = .09). CONCLUSIONS: The ANBL0531 trial treatment algorithm resulted in excellent long-term survival. More effective treatments are needed for subsets of patients with unfavorable biology tumors.

Small-bowel plication prevents ileostomy prolapse in young children with inflammatory bowel disease.

PURPOSE: Young children with medically refractory very early-onset inflammatory bowel disease (VEO-IBD) sometimes benefit from ileostomy diversion alone or may be offered subtotal colectomy with ileostomy. Though generally well-tolerated, ileostomy complications are frequent. Prolapse is particularly frustrating as it can be difficult and painful to reduce, becomes a recurring problem is some patients, and often requires ostomy revision or bowel resection. METHODS: Over the course of the past 6 months, eight consecutive children with VEO-IBD underwent 10 creation or revision of a diverting ileostomy (two underwent subsequent colectomy with ileostomy revision). In each of these 10 cases, we plicated the ileum just proximal to the ileostomy for a distance of approximately 3 cm using a running permanent monofilament suture. RESULTS: No patient who underwent plication of bowel has developed ileostomy prolapse. There were no cases of ileostomy retraction, parastomal hernia or ostomy-level obstruction. One patient required a lysis of a single band adhesion for a more proximal small bowel obstruction. The stomas have functioned well and there have been no complications. CONCLUSION: Simple bowel plication appears to be a quick and effective way to prevent ileostomy prolapse in young children with VEO-IBD with an ileostomy who are at high risk for prolapse.

Guideline for Children With Cancer Receiving General Anesthesia for Procedures and Imaging.

Children with cancer and those undergoing hematopoietic stem cell transplantation frequently require anesthesia for imaging as well as diagnostic and therapeutic procedures from diagnosis through follow-up. Due to their underlying disease and side effects of chemotherapy and radiation, they are at risk for complications during this time, yet no published guideline exists for preanesthesia preparation. A comprehensive literature review served as the basis for discussions among our multidisciplinary panel of oncologists, anesthesiologists, nurse practitioners, clinical pharmacists, pediatric psychologists, surgeons and child life specialists at the Children's Hospital of Philadelphia. Due to limited literature available, this panel created an expert consensus guideline addressing anesthesia preparation for this population.

Intermittent boluses of balanced salt solution for post-operative intravenous hydration following elective major abdominal and thoracic surgery in children.

PURPOSE: Maintenance fluids following major operations in children are typically administered with a continuous rate. We hypothesized that administering fluids as intermittent boluses is more physiologic and could limit post-operative fluid volume, thereby avoiding harmful effects of excess fluid. METHODS: We retrospectively reviewed children aged 1-21 admitted after an elective major abdominal or thoracic operation from 2015 to 2021. We excluded non-elective operations and patients receiving peri-operative enteral or parenteral nutrition. We analyzed total fluid volume at 0-24, 24-48, 48-72, and 72-96 h, time to regular diet and discharge, and end-organ complications. RESULTS: We identified 363 patients, of which 108 received intermittent boluses and 255 continuous fluids. Bolus group patients received significantly less fluid up to 72 h post-operatively with average rates of 0.49 mL/kg/h vs 0.86 mL/kg/h at 0-24 h (p << 0.01), 0.57 mL/kg/h vs 1.46 mL/kg/h at 24-48 h (p << 0.01), and 0.50 vs 0.92 mL/kg/h at 48-72 h (p << 0.01). Additionally, the bolus group maintained adequate urine output, tolerated a regular diet sooner (2.08 days vs 2.51 days; p = 0.0023) and averaged a shorter hospital stay (3.12 vs 4.14 days; p = 0.004). There was no difference in adverse effects between the two groups. CONCLUSION: Utilizing intermittent boluses reduces the volume of maintenance fluids administered and may lead to a faster time to regular diet and discharge. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective review.

Surgical considerations for neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome: a series of 14 patients from a single institution.

INTRODUCTION: Neuroblastoma is a childhood cancer of neural crest cells occasionally associated with opsoclonus-myoclonus-ataxia syndrome (OMAS), a paraneoplastic process characterized by ataxia, rapid eye movements, and muscle twitching. OMAS treatment and outcomes are well studied, but prior reports do not detail how the presence of OMAS should impact surgical approach, particularly for tumors with image defined risk factors (IDRF). METHODS: We reviewed patients with neuroblastoma and OMAS at our institution from January 2009 to December 2020 and recorded tumor characteristics, operative details, OMAS therapies, and outcomes. RESULTS: We identified 14 patients with neuroblastoma and OMAS out of 212 patients referred for surgery. There were 11 gross total resections and three partial resections. Two patients with partial resections developed OMAS after initial resection. One patient with gross total resection developed tumor recurrence 10 years later with OMAS redevelopment signaling recurrence. Three patients were positive for IDRFs and the one receiving neoadjuvant therapy achieved a gross total resection. CONCLUSIONS: OMAS development after partial resection and OMAS recurrence following tumor recurrence indicates a correlation between tumor bulk and the paraneoplastic process. This justifies an aggressive resection even for low-risk tumors. Neoadjuvant therapy should be considered for potentially unresectable tumors with image defined risk factors. LEVEL OF EVIDENCE: IV.

Differentiating solitary brain metastases from high-grade gliomas with MR: comparing qualitative versus quantitative diagnostic strategies.

PURPOSE: To investigate the diagnostic efficacy of MRI diagnostic algorithms with an ascending automatization, in distinguishing between high-grade glioma (HGG) and solitary brain metastases (SBM). METHODS: 36 patients with histologically proven HGG (n = 18) or SBM (n = 18), matched by size and location were enrolled from a database containing 655 patients. Four different diagnostic algorithms were performed serially to mimic the clinical setting where a radiologist would typically seek out further findings to reach a decision: pure qualitative, analytic qualitative (based on standardized evaluation of tumor features), semi-quantitative (based on perfusion and diffusion cutoffs included in the literature) and a quantitative data-driven algorithm of the perfusion and diffusion parameters. The diagnostic yields of the four algorithms were tested with ROC analysis and Kendall coefficient of concordance. RESULTS: Qualitative algorithm yielded sensitivity of 72.2%, specificity of 78.8%, and AUC of 0.75. Analytic qualitative algorithm distinguished HGG from SBM with a sensitivity of 100%, specificity of 77.7%, and an AUC of 0.889. The semi-quantitative algorithm yielded sensitivity of 94.4%, specificity of 83.3%, and AUC = 0.889. The data-driven algorithm yielded sensitivity = 94.4%, specificity = 100%, and AUC = 0.948. The concordance analysis between the four algorithms and the histologic findings showed moderate concordance for the first algorithm, (k = 0.501, P < 0.01), good concordance for the second (k = 0.798, P < 0.01), and third (k = 0.783, P < 0.01), and excellent concordance for fourth (k = 0.901, p < 0.0001). CONCLUSION: When differentiating HGG from SBM, an analytical qualitative algorithm outperformed qualitative algorithm, and obtained similar results compared to the semi-quantitative approach. However, the use of data-driven quantitative algorithm yielded an excellent differentiation.

A safety and feasibility trial of 131 I-MIBG in newly diagnosed high-risk neuroblastoma: A Children's Oncology Group study.

INTRODUCTION: 131 I-meta-iodobenzylguanidine (131 I-MIBG) is effective in relapsed neuroblastoma. The Children's Oncology Group (COG) conducted a pilot study (NCT01175356) to assess tolerability and feasibility of induction chemotherapy followed by 131 I- MIBG therapy and myeloablative busulfan/melphalan (Bu/Mel) in patients with newly diagnosed high-risk neuroblastoma. METHODS: Patients with MIBG-avid high-risk neuroblastoma were eligible. After the first two patients to receive protocol therapy developed severe sinusoidal obstruction syndrome (SOS), the trial was re-designed to include an 131 I-MIBG dose escalation (12, 15, and 18 mCi/kg), with a required 10-week gap before Bu/Mel administration. Patients who completed induction chemotherapy were evaluable for assessment of 131 I-MIBG feasibility; those who completed 131 I-MIBG therapy were evaluable for assessment of 131 I-MIBG + Bu/Mel feasibility. RESULTS: Fifty-nine of 68 patients (86.8%) who completed induction chemotherapy received 131 I-MIBG. Thirty-seven of 45 patients (82.2%) evaluable for 131 I-MIBG + Bu/Mel received this combination. Among those who received 131 I-MIBG after revision of the study design, one patient per dose level developed severe SOS. Rates of moderate to severe SOS at 12, 15, and 18 mCi/kg were 33.3%, 23.5%, and 25.0%, respectively. There was one toxic death. The 131 I-MIBG and 131 I-MIBG+Bu/Mel feasibility rates at the 15 mCi/kg dose level designated for further study were 96.7% (95% CI: 83.3%-99.4%) and 81.0% (95% CI: 60.0%-92.3%). CONCLUSION: This pilot trial demonstrated feasibility and tolerability of administering 131 I-MIBG followed by myeloablative therapy with Bu/Mel to newly diagnosed children with high-risk neuroblastoma in a cooperative group setting, laying the groundwork for a cooperative randomized trial (NCT03126916) testing the addition of 131 I-MIBG during induction therapy.

MRI-based classification of the anatomical variants of the hippocampal head.

PURPOSE: The head of the hippocampus (H) is classically described as having two to four digitations both in ex vivo specimens and in vivo MR coronal images. The aim of this study was to develop and evaluate a new MR-based classification of the anatomical variants of the hippocampal head in a large sample population of healthy subjects. METHODS: MR images of the brain of 238 young healthy subjects (138 men and 100 women; age range 18-39) were analyzed. The head of the H was identified on coronal reformatted 3D T1 weighted MR images. The frequencies were reported for hemisphere and sex. Inter-rater reliability was assessed. RESULTS: Eight variants of the hippocampal head were described. Class 0 (11.4%) indicated a total absence of sulci. This class was further subdivided as follows: 0A (one digitation, 10.1%) and 0B (no digitations or "null variant", 1.3%). Class 1 (25.6%) presented a single sulcus and was further subdivided into four types according to the location and the width of the sulcus [1A (8.8%), 1B (12.8%), 1C (1.3%), and 1D (2.7%)]. Class 2 (63.0%, the most frequent and the classical variant) had two symmetrical sulci and three digitations. Statistically significant differences between the two hemispheres were observed only in women and overall. Differences in prevalence between sexes were not observed. CONCLUSIONS: The large study population allowed the description of a novel morphological classification of the different anatomical variants of normal H in the coronal plane. This classification could reduce the risk of misinterpreting normal anatomical variants as pathological.

In vivo and in vitro results of an automated preloaded delivery system for IOL implantation in cataract surgery.

PURPOSE: To compare the corneal tissue trauma after the use of an automated preloaded injector and a manual injector and assess scanning electron microscope (SEM) and atomic force microscope (AFM) features of both injector cartridges. SETTING: Ophthalmology Clinic and Laboratory of Stem Cells and Regenerative Medicine University "G. d'Annunzio" of Chieti-Pescara, Chieti, Italy; DESIGN: Prospective randomized clinical study METHODS: Forty eyes of 40 patients for phacoemulsification were divided into two groups: implantation of intraocular lens was performed with AutonoMe automated delivery system (AutonoMe group: 20 eyes) and Monarch III injector system (Monarch group: 20 eyes). In vivo confocal microscopy (IVCM) and anterior segment optical coherence tomography (AS-OCT) were performed before surgery, at 1 h, 1 day and 1 month post-operatively. In addition, SEM and AFM were performed on cartridges of both injector systems after injection of the IOL. RESULTS: A greater increase in central corneal thickness and corneal thickness at the incision site were observed in Monarch group versus AutonoMe group 1 h and 1 day post-operatively (p < 0.05). Endothelial cell count loss was significantly higher in Monarch group compared with AutonoMe group (p < 0.05) at 1 and 24 h. AS-OCT showed less endothelial misalignment at 30 days (p < 0.05), and IVCM showed less tunnel inflammation at all time points (p < 0.05) in AutonoMe group compared with Monarch group; roughness analysis at AFM of the AutonoMe cartridge was significantly lower compared to Monarch D cartridge (p < 0.05). CONCLUSIONS: The AutonoMe injector provided less corneal tissue trauma compared with Monarch III injector. The AutonoMe cartridge showed lower roughness at AFM compared to the Monarch D cartridge.

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs. >24 months postoperatively, respectively; P < 0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. CONCLUSION: This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).

Diagnosis of Beckwith-Wiedemann syndrome in children presenting with Wilms tumor.

Beckwith-Wiedemann syndrome (BWS) is a genetic syndrome associated with overgrowth and cancer predisposition, including predisposition to Wilms tumor (WT). Patients with BWS and BWS spectrum are screened from birth to age 7 years for BWS-associated cancers. However, in some cases a BWS-associated cancer may be the first recognized manifestation of the syndrome. We describe 12 patients diagnosed with BWS after presenting with a WT. We discuss the features of BWS in these patients and hypothesize that earlier detection of BWS by attention to its subtler manifestations could lead to earlier detection of children at risk for associated malignancies.

Successful treatment of pulmonary mucormycosis in two pediatric hematopoietic stem cell transplant patients.

Pulmonary mucormycosis diagnosed immediately after hematopoietic stem cell transplantation frequently portends a poor prognosis. However, here we describe two cases in children that were treated successfully to highlight the efficacy of a multidisciplinary approach. Despite diagnosis in the immediate post-transplant period and requirement for ongoing immunosuppression to prevent or treat GVHD, both are long-term survivors due to early surgical debridement with transfusion support and prompt initiation of targeted antifungal therapy. In the absence of evidence-based treatment guidelines, survival of pulmonary mucormycosis is achievable even in high-risk patients with a multidisciplinary team to guide management.

Single-site robotic-assisted laparoscopic cholecystectomy in children and adolescents: a report of 20 cases.

BACKGROUND: Single-site laparoscopy is increasingly popular for straightforward operations like appendectomy. Due to limited triangulation and maneuverability, single-site cholecystectomy is riskier and more difficult. Robotics offer to make it easier and safer. METHODS: Twenty children and adolescents underwent robotic-assisted single-site cholecystectomy at a large academic children's hospital. Patients were not randomized; patients were offered the option of robotic-assisted single-site (SSR) or standard four-incision laparoscopic (LAP) cholecystectomy. Demographics and perioperative details were compared with those of a comparable cohort who underwent LAP during the same period. RESULTS: The two groups were similar in physical characteristics and indications for operation. The robotic operations took longer but both groups received similar PRN doses of parenteral opiates. Patients in the SSR group were all discharged on the first postoperative day. There were no major complications in either group but a slightly higher incidence of minor wound complications in the SSR group. CONCLUSION: Robotic-assisted single-site cholecystectomy appears to be a safe alternative to standard laparoscopy with a similar postoperative pain profile, short postoperative lengths of stay, and, for some, a superior cosmetic result. Nevertheless, it comes with longer set-up and operative times, a higher incidence of minor wound complications, an unknown but possibly higher risk of incisional hernia, and higher costs.

ADULT-ONSET FOVEOMACULAR VITELLIFORM DYSTROPHY EVALUATED BY MEANS OF OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A Comparison With Dry Age-Related Macular Degeneration and Healthy Eyes.

PURPOSE: To investigate alterations of superficial and deep retinal vascular densities, as well as of choroidal thickness, in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD). METHODS: A total of 22 eyes (15 patients) affected by AOFVD were recruited in the study. Furthermore, 20 eyes of 20 healthy subjects and 20 eyes of 18 patients affected by intermediate dry age-related macular degeneration (AMD) were enrolled. All patients underwent a complete ophthalmologic examination, including optical coherence tomography angiography. Outcome measures were superficial vessel density, deep vessel density, and choroidal thickness. RESULTS: Parafoveal superficial vessel density was increased in patients with AOFVD compared with the AMD group (50.6 ± 4.3% and 46.3 ± 4.3%, respectively, P = 0.016). Parafoveal deep vessel density was 57.9 ± 6.4% in patients with AOFVD, 52.2 ± 3.8% in patients with AMD, and 52.7 ± 6.0% in healthy controls (P = 0.006 and P = 0.035, respectively, after comparison with the AOFVD group). CONCLUSION: We demonstrated that both superficial and deep vessel densities were significantly increased in patients with AOFVD, after the comparison with intermediate patients with AMD. These findings suggest that the pathogenic mechanisms in AOFVD are different from those in AMD and that optical coherence tomography angiography could be useful in differentiate early stages of these two diseases.

Microvascular changes after vitrectomy with internal limiting membrane peeling: an optical coherence tomography angiography study.

PURPOSE: To evaluate superficial capillary plexus (SCP) changes occurring after internal limiting membrane (ILM) peeling for the treatment of idiopathic epiretinal membrane (ERM). METHODS: A total of 15 eyes of 15 patients affected by idiopathic ERM (eight males and seven females; mean age 59.8 ± 9.6 years) were enrolled in the study. Patients were treated with pars plana vitrectomy followed by ERM and ILM peeling. Subjects were evaluated at baseline and at the week-1 and month-1 follow-up visits. At each visit, patients were evaluated with a complete ophthalmologic evaluation, which included imaging with optical coherence tomography angiography. RESULTS: Overall, the SCP vessel density was 43.0 ± 3.0% at baseline and was stable throughout the follow-up (40.0 ± 4.0% at week-1 and 41.0 ± 4.0% at month-1 follow-up visits; p = 0.087 and p = 0.426, respectively). Nevertheless, the SCP vessel density was reduced at week-1 visit in both the superior and inferior sectors. In these sectors, the superficial vessel density was still reduced at the month-1 follow-up visit. CONCLUSIONS: We observed a reduction in the SCP vessel density occurring after pars plana vitrectomy with ILM peeling. The reduction is referred to those areas where other changes (e.g., swelling of the arcuate nerve fiber layer) have been already described. In theory, superficial vessel density modifications may be due to the direct surgical trauma to the inner retina, where the superficial plexus is contained, during the ILM grasping.

Mortality and Morbidity after Laparoscopic Surgery in Children with and without Congenital Heart Disease.

OBJECTIVES: To determine the risk of morbidity and mortality after laparoscopic surgery among children with congenital heart disease (CHD). STUDY DESIGN: Cohort study using the 2013-2014 National Surgical Quality Improvement Program-Pediatrics, which prospectively collected data at 56 and 64 hospitals in 2013 and 2014, respectively. Primary exposure was CHD. Primary outcome was overall in-hospital postoperative mortality. Secondary outcomes included 30-day mortality and 30-day morbidity (any nondeath adverse event). Among 34?543 children who underwent laparoscopic surgery, 1349, 1106, and 266 had minor, major, and severe CHD, respectively. After propensity score matching within each stratum of CHD severity, morbidity and mortality were compared between children with and without CHD. RESULTS: Children with severe CHD had higher overall mortality and 30-day morbidity (OR 12.31, 95% CI 1.59-95.01; OR 2.51, 95% CI 1.57-4.01, respectively), compared with matched controls. Overall mortality and 30-day morbidity were also higher among children with major CHD compared with children without CHD (OR 3.46, 95% CI 1.49-8.06; OR 2.07, 95% CI 1.65-2.61, respectively). Children with minor CHD had similar mortality outcomes, but had higher 30-day morbidity compared with children without CHD (OR 1.71, 95% CI 1.37-2.13). CONCLUSIONS: Children with major or severe CHD have higher morbidity and mortality after laparoscopic surgery. Clinicians should consider the increased risks of laparoscopic surgery for these children during medical decision making.

Management of adrenal masses in patients with Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome, associated with both benign and malignant adrenal findings. Literature review and an institutional case series elucidate the wide spectrum of adrenal findings in BWS patients. The altered expression of the 11p15 region is likely related to adrenal gland hyperplasia and growth dysregulation. Given the absence of guidelines for managing adrenal findings in BWS, we propose a systematic approach to adrenal findings in BWS patients, to allow for maximum detection of potentially malignant pathology without posing additional risk to patients.