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The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Both are usually diagnosed prenatally with fetal ultrasonography, and affected patients are treated at a center with access to high-risk obstetric services, neonatology, and pediatric surgery. The main distinguishing features between the 2 are that gastroschisis has no sac and the defect is to the right of the umbilicus, whereas an omphalocele typically has a sac and the defect is at the umbilicus. In addition, patients with an omphalocele have a high prevalence of associated anomalies, whereas those with gastroschisis have a higher likelihood of abnormalities related to the gastrointestinal tract, with the most common being intestinal atresia. As such, the prognosis in patients with omphalocele is primarily affected by the severity and number of other anomalies and the prognosis for gastroschisis is correlated with the amount and function of the bowel. Because of these distinctions, these defects have different management strategies and outcomes. The goal of surgical treatment for both conditions consists of reduction of the abdominal viscera and closure of the abdominal wall defect; primary closure or a variety of staged approaches can be used without injury to the intra-abdominal contents through direct injury or increased intra-abdominal pressure, or abdominal compartment syndrome. Overall, the long-term outcome is generally good. The ability to stratify patients, particularly those with gastroschisis, based on risk factors for higher morbidity would potentially improve counseling and outcomes.

On initial examination, a male newborn has the finding shown in Fig 1. Figure 1. Initial presentation. ### Prenatal and Birth Histories ### Presentation (Day 1) The infant was placed nil per OS (NPO) and a large-bore nasogastric tube was placed to low-intermittent suction to decompress the stomach. Total parenteral nutrition was initiated for nutritional support. His glucose levels were monitored and found to be normal. The pediatric surgical team was consulted. Vital signs were as follows: ### Physical Examination (Day 1)

Management of complex esophageal problems in children is challenging. We report our experience with the use of esophageal stents in three children with esophageal strictures, leaks, or airway-esophageal fistulae refractory to conventional treatment. The stent played a key role in allowing extubation of a child with a large tracheo-esophageal-pleural fistula and in the resolution of pulmonary infection in a child with esophago-bronchial fistula, both followed by surgery. In the third child, with stricture, stents were complicated with migration, esophageal erosion, and esophago-bronchial fistula. In our experience, esophageal stents were useful mainly as a bridge to definitive surgical repair.

Purpose To evaluate technical and clinical success and report long-term outcomes of portal vein (PV) recanalization in pediatric orthotopic liver transplant (OLT) patients with chronic PV occlusion. Materials and Methods This is a retrospective review of 15 OLT patients (5 males) with chronic PV occlusion who underwent PV recanalization (33 procedures) between October 2011 and February 2018. Median age was 4.5 years (range, 1–16 years); median weight was 16.6 kg (range, 11.5–57.3 kg). Median time interval from OLT to first intervention was 3.25 years (range, 0.6–15.7 years). Clinical presentations included hypersplenism (n = 12), gastrointestinal bleeding (n = 9), and ascites (n = 3). One patient had incidental diagnosis of PV occlusion. Primary, primary-assisted, and secondary patency at 3, 6, 12, and 24 months were evaluated. Results Technically successful PV recanalization and reduction of PV pressure gradient to ≤ 5 mm Hg was performed in 13/15 patients (87%). Ten of 15 (67%) patients had successful recanalization with the first attempt. Clinical success, defined as improvement in signs and symptoms of portal hypertension, was achieved in 12/13 (92%) patients. Five of 33 (15%) major complications (Society of Interventional Radiology class C), including perisplenic hematoma (n = 2), hemoperitoneum (n = 2), and hepatic artery pseudo aneurysm (n = 1), were managed with pain medication and blood product replacement. Median follow-up was 22 months (range, 1–77 months). Median primary patency was 5 months. Primary patency at 3, 6, 12, and 24 months was 53.8%, 46.2%, 38.5%, and 30.8%, respectively. Primary-assisted patency was 84.6%, 76.9%, 53.8%, and 46.2%, respectively. Secondary patency was 92.3%, 84.6%, 53.8%, and 46.2%, respectively. Conclusions PV recanalization is a safe and effective minimally invasive option in the management of chronic PV occlusion after pediatric OLT.

Embolization is a well-established method of treatment of arteriovenous malformations. Most procedures are performed under general anesthesia due to the painful nature of some sclerosing agents, procedure duration, and need for immobilization to allow precise injection. Familiarity with the possible side effects of these agents is important for the anesthesiologist to facilitate timely and successful management of intra- and postprocedural complications. This case report describes an unusual presentation of systemic migration of glue-lipiodol mixture during embolization of a calf arteriovenous malformation in an adolescent that resulted in accelerated idioventricular rhythm and its successful management leading to complete resolution.

DenverRetrospective chart review of a 6-year-old girl with generalized lymphatic anomaly, who presented with refractory lymphorrhea from the labium majus, was performed. This was managed with innovative placement of the Denver shunt between a large abdominal wall cyst and the peritoneal cavity.There was progressive reduction in lymphorrhea with complete cessation at 11 months post shunt placement. At 33-month follow-up, the shunt remains patent with no further lymphorrhea.Denver shunts can be utilized for the creation of internal drainage pathways besides its traditional uses for draining peritoneal and pleural fluids.

Introduction: Pediatric chronic pancreatitis is not commonly diagnosed. Most patients with chronic pancreatitis have at least one gene mutation, often CFTR, PRSS1, or SPINK1.1 Other risk f...

Native esophagus is always the best, and all efforts should be made to preserve the native esophagus. In circumstances where the esophagus is not available, a suitable substitute is needed to perform the function of the esophagus. All the substitutes available have their advantages and disadvantages. The best substitute would be the one which allows close to normal swallowing with minimum reflux and reduced number of complications like strictures, leaks, and dilatation.

Patients with long gap EA, in which a primary repair is unable to be achieved without significant tension, encompasses a group of challenging patients in which there is no ideal management approach. Multiple operative strategies have been described for these patients. Many of these techniques require multiple procedures and may subject patients to prolonged operative times, sustained physiologic stress, and significant morbidity. Although esophageal replacement can be performed by a variety of methods if required, it is optimal to preserve the native esophagus if possible. There are a number of innovative techniques that have been described for esophageal lengthening in order to preserve the esophagus and create esophageal continuity in EA patients. These include new thoracoscopic approaches, the use of magnets, and lengthening devices.

Introduction: Metabolic syndrome is a well-known disease of multiple risk factors that has at its core, insulin resistance accompanying abnormal adipose deposition and function. Multiple studies demonstrate a potential relationship between Visceral Fat Thickness (VFT) and metabolic syndrome. Ultrasound provides a radiation free low-cost alternative, which is high reproducible in the quantification of visceral fat and can act as an effective screening modality. The simplicity of the method combined with its objectiveness makes it advantageous for accurate measurement of VFT. Aim: To correlate of sonographic measurement of VFT with existing metabolic syndrome. Materials and Methods: This cross-sectional study was conducted on all patients that underwent ultrasound at the study center between August 2016 to August 2019 and were clinically suspected of suffering from metabolic syndrome at the Endocrinology Outpatient Department (OPD). Collation of data into three categories based on VFT (10 cm) was done. The patients in each category underwent further testing and metabolic syndrome was either ruled out or diagnosed. Results: The study cohort comprised of 2022 patients. The VFT ranged between 4 and 16 with a mean value of 10.8 and standard deviation of 2.8. The percentage of patients with metabolic syndrome increased in proportion to the increased VFT. VFT >10 cm was found to have specificity and Positive Predictive Value (PPV) 92.52% and 92.53% respectively while VFT

A late preterm newborn presents with exposed bowel and abnormal upper limbs. ### Prenatal and Birth Histories ### Presentation The female newborn cried immediately after birth. The exposed bowel was placed immediately in a plastic bowel bag and the infant was positioned on her right side. A nasogastric tube was placed and attached to continuous suction. The neonatology team provided positive pressure ventilation because of poor respiratory effort. Supplemental oxygen was titrated to maintain her pulse oximetry as per Neonatal Resuscitation Program guidelines. She was transferred to the NICU on continuous positive airway pressure (CPAP). ### Vital Signs ### Physical Examination (Newborn Day)

Background: High altitude, an extremely rare cause of cortical venous thrombosis (CVT) has no literature review available signifying the relation between two as per date. The aim of this study is to establish the relation of exposure to high altitude and occurrence of CVT in properly acclimatised healthy individuals exposed to high altitude having no pre-existing morbidities.Methods: An observational type of prospective study was conducted at a tertiary care centre in North India. Patients who were sent back from a high-altitude area with CNS symptoms (headache to coma) were included in the study. Magnetic resonance imaging (MRI) and magnetic resonance venography (MRV), blood investigations including complete blood count and D-dimer were done as routine examinations in all these patients. MRI and MRV findings were recorded and analyzed for features of CVT.Results: Twenty-eight patients with an average age of 31.5 years (23-51 years) were included in the study. All patients had CNS symptoms; with headache being the common symptom. In the patients of CVT, there were MRI features of parenchymal infarct in 7 (25%), hemorrhagic infarct in 11 (41.6%), subarachnoid hemorrhage in 7 (25%) and mass effect in 3 (8.4%) patients. MRV revealed involvement of multiple sinus involvement more common than single sinus involvement, of which most commonly involved sinuses were Superior sagittal sinus and transverse sinuses. D-Dimer levels were significantly raised in 23 (83%) patients.Conclusions: High altitude though a rare cause, can be the single most important contributory factor in the development of CVT in healthy acclimatised individuals with no predisposing factors. The physicians dealing with such patients at high altitude should be well aware of the scenario as early diagnosis via imaging and prompt management can drastically reduce mortality in this potentially lethal but treatable conditions.

Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is characterized by formation of recurrent benign tumors described as PTEN hamartoma of soft tissue that may contain fast flow vascular anomalies (FFVA). The purpose of this study is to review the temporal evolution and management of FFVA in PHTS. A retrospective review of 22 patients (9 males), age 1 to 18 (median 9) years diagnosed with PHTS at a tertiary care pediatric hospital between October 2002 and August 2017 revealed 4 patients with FFVA. Imaging, management, and treatment complications were reviewed. During median follow-up of 8 (range: 4–13) years, ultrasound and magnetic resonance imaging performed for recurrent pain, showed progressive increase in the size of hamartomas and development of new FFVA in three-fourth patients. Medical management included pain medications, oral sirolimus, and physical and psychiatric therapy. Surgical excision of hamartoma (n = 1) resulted in recurrence within 3 months. Between 4 and 24 (average 1.5/year) embolizations were performed per patient. Pain related to FFVA responded well to embolization. Pain secondary to PTEN hamartoma responded poorly to percutaneous sclerosant injection, but demonstrated improvement with sirolimus. There was no correlation between serum sirolimus levels and frequency/timing of recurrence of FFVA/hamartoma. Complications included sclerosant migration into digital arteries (n = 1), subclavian vein stenosis due to glue migration (n = 1), oral mucositis (n = 4), and elevated triglycerides (n = 4). Patients with PHTS present with recurrent pain requiring life-long management with a multi-disciplinary team. Pain due to FFVA responds to embolization, and pain due to hamartoma responds to sirolimus. This improves quality of life, but does not prevent disease progression.

Femoral tunneled central line placement in the pediatric population offers an alternative means for intravenous (IV) access, but there is concern for higher complication and infection rates when placed at bedside. To describe the complications and infection outcomes of primary femoral tunneled central venous catheter placement in the interventional radiology suite compared to the portable bedside location at a single tertiary pediatric institution. We conducted a retrospective review comparing interventional radiology suites vs. bedside primary tunneled common femoral vein central line placement (January 2014 to December 2015). We identified 244 primary femoral placements in pediatric patients, ages 1 day to 18 years, using our electronic medical record and collected into a Research Electronic Data Capture. We compared categorical variables using the Fisher exact test. We compared continuous variables using the Wilcoxon rank test. In total, 2,375 pediatric patients received peripherally inserted and central lines; 244 of these were primary femoral tunneled central venous catheters (in 140 boys and 104 girls). In 140 children (mean age: 206 days), lines were inserted in the interventional radiology (IR) suite (technical success of 100%), with 14 (10.0%) complications including infection (n=7), malposition (n=2), bleeding (n=0), thrombosis (n=1) and line occlusion (n=4). The infection rate was 2.1 per 1,000 line days. In 104 children (mean age: 231 days), lines were placed at bedside (technical success 100%) with 14 (13.3%) complications including infection (n=3), malposition (n=5), bleeding (n=0), thrombosis (n=2) and line occlusion (n=4). The infection rate was 0.78 per 1,000 line days. The total line days were 7,109, of which 3,258 were in the IR suite and 3,851 in the bedside group. There was no statistical significance for complication rate (P=0.55) or infection rate (P=0.57) between bedside and interventional suite placements. In a cohort of children receiving primary femoral tunneled central venous catheters, the complication and infection rates in a bedside setting are not significantly increased compared to the lines placed in an IR suite. The perception of increased infection and complications from bedside-placed tunneled central venous catheters appears to be hyperbolized.

Background Lot of debate is centered around the prognostic value of coronary artery calcium scores in predicting coronary atherosclerosis. NICE Guidelines exist regarding the role of calcium scoring as a screening tool in patients with a low (10–29%) likelihood of suffering from coronary artery disease. 1 The aim of this study was to study the efficacy of calcium scores in predicting severity of coronary atherosclerosis in patients with moderate (30–60%) risk of coronary artery disease. Methods Retrospective review of 445 Coronary CT Angiograms done between August 2014 and October 2015 with the objective of correlating coronary calcium scores and the number of significantly stenosed coronary arteries. Results In our study CS = 0 had extremely high sensitivity and negative predictive value of 100% for ruling out any critical stenosis in both low and intermediate risk groups. Meanwhile in the intermediate risk group, CS >400 was 97.46% specific for critical stenosis with positive predictive value of 81.63%. Conclusion We propose that coronary artery calcium scoring may be considered for use as a primary screening modality in patients with moderate risk of coronary artery disease.

A single-incision technique for tunneled central venous access has been described. This study evaluates whether single-incision technique in children is comparable to the conventional method, with regard to procedure time, fluoroscopy time, and complication rate. This is a retrospective review of 303 internal jugular vein tunneled central catheter placements whose age ranged from newborn to 17 years (median 1.7 years) by pediatric interventional radiologists from January 2014 through December 2015. 223 catheters were placed (181 patients) using the single-incision technique, and 80 catheters were placed (72 patients) using the conventional two-incision technique. Data were obtained from electronic medical records and PACS including procedure time, fluoroscopy time, and complication rates which were compared for both single-incision and conventional techniques. Technical success for the single-incision and conventional technique groups was 99.1 and 98.8%, respectively. Early complication rate was 12.1% for the single-incision technique and 17.5% for the conventional technique (p = 0.254). Overall complication rate was 26% (3.8/1000 line days) for the single-incision technique and 37.5% (4/1000 line days) for the conventional technique (p = 0.085). Median procedure time was 25 min for the single-incision technique and 26 min for the conventional technique (p = 0.427). Median fluoroscopy time was 1.7 min in the single-incision group and 1.3 min in the conventional group (p = 0.085). The single-incision technique for central venous access has comparable procedure time and fluoroscopy time with no difference in complication rates between the two techniques in a pediatric population.

Global transcriptional profiling of individual cells represents a powerful approach to systematically survey contributions from specific cellular phenotypes to human disease states. Heart diseases are the leading causes of death globally, yet no protocols for robust single cell isolation from adult human hearts have been published. Here, we found that enzymatic dissociation of human heart resulted in low-quality cell suspensions across a range of protocols. We show that isolation of cardiac nuclei provides a robust alternative, and that nuclear transcriptomes reliably represent the cytoplasmic and whole-cell transcriptomes of major cardiac cell types. Furthermore, coupling of nuclear isolation to PCM1-gated flow cytometry facilitated specific cardiomyocyte depletion, expanding the cardiac transcriptome beyond bulk tissue transcriptomes which were strongly correlated with PCM1+ transcriptomes (r=0.8). We applied these methods to generate a transcriptional catalogue of human cardiac cells by droplet-based RNA-sequencing of 8,460 nuclei from which cellular identities were inferred. Reproducibility was explored in an independent biopsy (4,760 additional nuclei) from the same human heart. Our results provide a perspective of the cellular composition of the adult human heart and validate a framework for assessment of cellular individuality across cardiac disease states.

Objective Complicated lymphatic anomalies (CLAs) are chronic, progressive, and debilitating conditions that share clinical features, yet key elements for optimal evaluation and management have not been established. We aimed to formulate expert opinion consensus-based guidelines for comprehensive evaluation of CLAs. Study design Patient support groups dedicated to CLAs organized an international conference for vascular anomaly experts from 16 specialties to address the objective. Participants received a set of questions before the meeting and reviewed the literature. Data extracted from international lymphatic anomaly registries were presented and the group separated for panel discussions during the conference. The recommendations achieving consensus within the panel were presented to the entire audience. Open debate occurred until majority approval was achieved. Results The expert group was composed of 52 physicians who defined the clinical elements required to evaluate and diagnose a CLA. The radiology panel established the preferred anatomical and functional imaging methods for diagnosis and the elements required to be described during interpretation. Two medical panels compiled the metabolic and hematologic tests at diagnosis and also recommended functional studies. The surgical group recommended precautions for biopsy and the pathology panel provided biopsy specimen processing guidelines. Conclusions Patients with CLAs require a comprehensive and targeted diagnostic plan for appropriate management, prevention of complications, and conservation of resources. As this population is managed by diverse medical and surgical specialties, we offer an expert multidisciplinary consensus-based opinion on the current literature and on data extracted from international lymphatic anomaly registries.

Background Isolated coronary artery anomalies are usually clinically silent and mostly detected incidentally during angiography or autopsy. However, few of them may be implicated in cases of sudden cardiac death even in the absence of additional heart abnormalities. Prior knowledge of such variants and anomalies is necessary for planning various interventional procedures. Multiple detector computed tomography coronary angiography has proved a very useful non-invasive modality in this field given its superiority over conventional coronary angiography in providing detailed coronary artery anatomy. Methods A retrospective review of the coronary CT angiography studies was carried out at our center between August 2014 and December 2015 with the purpose of describing the coronary artery variants and anomalies that we came across in our cohort. Results In our cohort, about 77% (n = 391) of the patients had a right dominant system while left dominant and co-dominant systems were seen in 12% (n = 61) and 11% (n = 56) respectively. Coronary CT angiography was successful in visualizing smaller branches, such as the conus artery (96.25%, n = 489), the sinus node artery (83.07%, n = 422), and the septal branches (95.27%, n = 484). Coronary anomalies were observed in the 10.04% of our population (n = 51). Eleven anomalies of origin and course were found. Conclusion Coronary CT angiography gives us a good understanding of the variations and anomalies of the anatomy of the coronary arteries. This can be of immense help to the clinician planning interventional procedures.

Digital papillary adenocarcinoma is a rare malignant tumor of the sweat glands, most commonly encountered in adult males. Only few pediatric cases have been reported, with an apparent predominance of females and none on the ankle. We report a case of a 15-year-old girl with a slowly growing, non-tender mass on the right ankle presenting with difficulty in walking; clinically believed to be a keloid. Excision of the mass revealed a glandular neoplasm with largely eccrine and focally apocrine differentiation. Histologic features do not reliably predict biologic behavior. Because of the existing confusion over the nomenclature, we propose that such tumors be called 'papillary adnexal neoplasms of distal extremities'.

The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease.Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (30days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age2, and weight10kg. Univariate and multivariate analysis were performed.One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7±6.3years. Mean catheter life was similar in the laparoscopic and open groups (581±539days versus 574±487days, p=0.938). The late complication rate was higher for open procedures (57% versus 37%, p=0.013). Children age2 or weight10kg had higher re-operation rates (64% versus 42%, p=0.014 and 73% versus 40%, p=0.001, respectively). Adjusted for age and weight, open technique remained a risk factor for late complications (OR 2.44, 95% CI 1.20-4.95) but not re-operation.Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children2years age or10kg remain at risk for complications regardless of technique.

Neurocutaneous melanosis (NCM) is a rare congenital disorder. Most of the cases described in literature for this entity have involvement of the leptomeninges and other structures of brain such as brain stem, temporal lobes, and spinal meninges and no involvement of leptomeninges and presence of lesions in bilateral temporal lobes. NCM without the involvement of leptomeninges should be considered a distinct entity as the prognosis is favorable as compared to cases with leptomeningeal involvement who develop early hydrocephalus and multiple cranial nerve palsies.

Background Umbilical hernia repairs are one of the most commonly performed operations in children. The traditional repair involves an infraumbilical incision, which produces a visible scar. We report a novel technique of umbilical hernia repair through a transumbilical incision, which eliminates the scar by hiding it within the umbilicus. Methods We performed a retrospective chart review of 134 patients who had undergone a transumbilical hernia repair at a single institution between 2008 and 2016. Satisfaction with cosmesis and the presence of complications were assessed through parental interviews during follow up visit or by telephone survey. These data were compared to a large volume retrospective analysis of the standard infraumbilical approach. Results 121 of the 134 patients were evaluated in the clinic or by telephone interview. The overall complication rate was 7.44%. Parents of 118 patients reported satisfaction with the cosmetic result (97.52%). In comparison to the largest study of pediatric infraumbilical repair, there was an improvement in subjective cosmesis without a significant increase in complications. Conclusion Transumbilical hernia repair is a safe and cosmetically appealing technique for umbilical hernia repair in children. Level of evidence Treatment study, level III.

Introduction External angular dermoid cysts, or epidermoid inclusion cysts, are a common subcutaneous tumor of the head and neck. For the majority of these lesions, excision is relatively simple and performed through an incision immediately overlying the mass. Facial lesions in pediatric patients present a unique challenge in that a direct approach carries the potential for visible scar formation. Objective This article aims to detail our experience with subcutaneoscopic excision of external angular dermoid cysts in pediatric patients using endoscopic instrumentation. Materials and Methods Retrospectively, we reviewed 11 cases, between the ages of 4 months and 3 years with external angular dermoid cysts. An incision is made on the scalp above the hairline, then a tunneled working space is created underneath the skin. 3 mm laparoscopy instruments were then used for providing excellent visualization and precise subcutaneoscopic dissection. Results There were no complications apparent in any of the 11 cases (except cyst rupture in one case), with mean procedure duration at 61 minutes. Final results at follow-up revealed aesthetically pleasing and well-healed skin incisions, hidden from view behind the hairline. Conclusion The subcutaneoscopic technique utilizing endoscopic instrumentation has the advantage of improved visualization of the cyst, greater precision of dissection, and excellent cosmesis. One disadvantage of this procedure is that this involves learning a new technique while the majority of surgeons are already comfortable with the open approach. Given the safety and efficacy observed for this subcutaneoscopic procedure, this technique can have tremendous possibilities.

Purpose To determine long-term outcomes of fetuses with neck masses (NM), including functional and cosmetic results. Methods A retrospective review was performed of all fetuses evaluated for NM from November 2001 to March 2014. Quality of life (QOL) was evaluated using the validated PedsQL™ questionnaire. Results Of 35 fetuses evaluated, 9 died perinatally and 1 died late from tracheostomy complications. NM ranged from 4 to 20cm (mean, 9.1cm); 18 were delivered by EXIT. Of 25 surviving patients, 22 had mass resection, 7 requiring more than one procedure. Surviving patients with lymphatic malformations (LM) had the highest incidence of moderate and severe disfigurement and a higher rate of persistent/recurrent disease (100% vs. 31%, p=0.002) and cranial nerve dysfunction (50% vs. 0%, p=0.005) compared to those with non-LM diagnoses. Of 9 children attending school, 78% achieve grades of A/B's. QOL for 13 patients revealed a mean score of 83/100 for physical and 78/100 for psychosocial functioning. Median follow-up was 6years (7months–17years). Conclusion Unlike those with teratoma or other lesions, children with congenital cervicofacial LM are at high-risk for persistent disease, nerve dysfunction and moderate–severe disfigurement. There is substantial perinatal morbidity for fetuses with neck masses, but for those surviving, the long-term functional and cognitive outcomes are good.

To describe a dynamic magnetic resonance (MR) lymphangiography technique after intranodal injection of gadolinium-based contrast agent and to assess its feasibility for evaluation of the central conducting lymphatics (CCL) in patients with pathologic disorders that involve the CCL.A retrospective evaluation of experience with the dynamic MR lymphangiographic technique in six consecutive patients was performed after institutional review board approval. Written informed consent for the percutaneous procedure was obtained from the patient, parent, or the legally responsible guardian. The dynamic MR lymphangiographic technique involves ultrasonographically guided intranodal injection of gadolinium-based contrast material into the inguinal lymph nodes, combined with sequential imaging of the chest and abdomen with a three-dimensional sequence optimized for soft tissue with high spatial resolution that provides time-resolved imaging of lymphatic transit through the CCL. Qualitative assessment of the images was performed for reliability of CCL visualization and for associated findings that could explain the clinical symptoms, including lymphangiectasia, chylolymphatic reflux, and chylous leak.The procedure was technically successful in all six patients. The dynamic MR lymphangiographic findings confirmed the presence of normal CCL morphologic structure in two patients and provided a possible explanation for clinical manifestations in the remaining four patients. The dynamic MR lymphangiographic procedure led to a change in management in two patients, continuation of conservative treatment in three patients, and confirmation of an alternative nonlymphatic diagnosis in one patient. Image quality for visualization of the CCL was considered good in all cases by the two readers. There were no known adverse effects related to the procedure.The dynamic MR lymphangiographic technique with intranodal injection of gadolinium-based contrast material is feasible and can provide useful information in a variety of lymphatic flow abnormalities involving the CCL.

Objective To review the technical aspects and categorize the imaging findings of dynamic contrast enhanced magnetic resonance lymphangiography (DCMRL) and correlate the findings with patient management options. Materials and methods A retrospective review of patients who underwent DCMRL between June 2012 and August 2017 at a tertiary care paediatric hospital was performed. Twenty-five DCMRL studies were performed in 23 patients (9 males, 13 females, 1 ambiguous gender) with a median age of 4 years (range: 1 month–29 years). DCMRL imaging findings were reviewed, categorized and the impact on patient management was studied. Results DCMRL was technically successful in 23/25 (92%) studies. DCMRL findings were categorized based on the status of central conducting lymphatics (CCL) and alternate lymphatic pathways as follows: Type 1 – normal CCL with no alternate lymphatic pathways, Type 2 – partial (2a) or complete (2b) non-visualization of CCL with reflux of contrast into alternate pathways and Type 3 – normal CCL with additional filling of alternate pathways. Type 1 DCMRL patients (n = 5) were reassured and conservative management was continued, Type 2 patients (n = 10) had evidence of CCL obstruction hence thoracic duct ligation or embolization was avoided and other options such as lymphatic fluid diversion using Denver® shunt or lympho-venous anastomosis were used, and Type 3 patients (n = 8) were evaluated for elevated central venous pressure as a cause of lymphatic backflow in addition to Denver® shunt, lympho-venous anastomosis, thoracic duct ligation or embolization. Conclusion DCMRL is an evolving imaging technique for understanding abnormalities of the central conducting lymphatics. Categorization of imaging findings may be helpful in guiding selection of management options.

Three vascular lesions are commonly encountered in the oropharynx. These are hemangioma, venous malformation, and lymphatic malformation. A fourth type of vascular anomaly, arteriovenous malformation, may also occur in the oropharynx but only as an extension of a nearby larger lesion. As such, it is better considered outside of the anatomic subsection of the oropharynx. Each of these three types will be discussed separately with respect to phenotype, etiology, natural history, and treatment.

Not available.

Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology.

Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses.

Background: To assess the extent of Ultrasound (USS) abdomen findings in acute viral hepatitis and further assess the role of USS as a prognostic marker.Methods: From May 2013 to September 2016, a total of 220 patients of acute Enterogenic viral hepatitis were studied by routine USS within first seven days of onset of symptoms, followed by routine USS between 10 to 15 days and follow up scan after 12 weeks. Only patients with acute Enterogenic viral hepatitis (Hepatitis A and Hepatitis E) were included. All patients with chronic liver disease and other form of acute hepatitis i.e. Hepatitis B, C and D were excluded from the study.Results: Among 220 patients of acute viral hepatitis routine USS findings including hepatomegaly, bright liver and thickened GB wall and periportal adenopathy were in isolation or in combination up to varying degrees. The commonest routine USS finding in acute phase was thickened GB wall (80%). 14 patients exhibited triad of enlarged Portal Vein (PV), Splenomegaly and Ascites. These 14 patients had prolonged stay in hospital and 11 patients had deranged liver function tests at 12 wks. interval and three patients developed hepatic encephalopathy including one patient who went into fulminant hepatic failure. Out of the patients who did not exhibit this triad 72 patients still had deranged LFT at 12 weeks and maximum of these patients had a combination of USS markers at presentation.Conclusions: USS has very little if any role in the diagnosis of acute viral hepatitis. It can however play an important role as a prognostic marker during the acute phase where it can detect enlarged portal vein, splenomegaly and ascites. This triad of USS findings is suggestive of transient portal hypertension likely due to hepatic congestion. Also, other USS markers if seen in combination at presentation can reliably predict a poorer prognosis

Heterotopic gastric mucosa (HGM) has histologic features of gastric mucosa located outside of the stomach. The congenital form is a rare clinical entity that can present with GI bleeding, intussusception from a polypoid mass or intermittent obstruction. Ultrasound and upper GI series are typically used for diagnosis. We report a case of a young male with heterotopic gastric tissue presenting as numerous polyps causing intestinal obstruction. Keywords: Heterotopic gastric mucosa, Intestinal obstruction, Pediatric small bowel obstruction

Purpose To evaluate the role of multimodality imaging tools for intraprocedural guidance and outcome evaluation during sclerotherapy of low-flow orbital vascular malformations. Materials and Methods A retrospective review was performed of 17 consecutive patients with low-flow orbital malformations (14 lymphatic, two venous, and one venolymphatic) who underwent multimodality image-guided sclerotherapy between November 2012 and May 2015. Sclerotherapy technique, image guidance tools, and complications were recorded. Sclerotherapy outcome was evaluated using clinical response, magnetic resonance (MR) image–based lesion volumetry, and proptosis quantification. Results There were 22 sclerotherapy sessions performed. Intraprocedural ultrasound (US), fluoroscopy, cone-beam computed tomography (CT) and MR image fusion were used for image guidance with 100% technical success. Resolution of presenting symptoms was observed in all patients at 1-month follow-up. Four major sclerotherapy complications were successfully managed. Statistically significant reduction in lesion volume ( P = .001) and proptosis ( P = .0117) by MR image analysis was achieved in all patients in whom 3-month follow-up MR imaging was available (n = 13/17). There was no lesion recurrence at a median follow-up of 18 months (range, 8–38 mo). Conclusions Multimodality imaging tools, including US, fluoroscopy, cone-beam CT, and MR fusion, during sclerotherapy of low-flow orbital malformations provide intraprocedural guidance and quantitative image-based evaluation of treatment outcome.

Introduction Arterial pseudoaneurysms are well known iatrogenic complication of percutaneous angiographic or interventional vascular procedures. In the past, the definitive treatment option was open surgical repair which is a relatively invasive procedure. In the last few years, Ultrasound (US) guided percutaneous thrombin injection has been gaining popularity as definitive treatment of pseudoaneurysms. Aim To evaluate efficacy and safety of US guided percutaneous injection of thrombin for treatment of iatrogenic femoral pseudoanurysms at a tertiary care interventional radiological and cardiology centre. Materials and methods A retrospective analysis was conducted on 38 consecutive patients, diagnosed to have iatrogenic femoral artery pseudoaneurysms by Doppler study, in the period from Jan 2013 to Jun 2016. All these patients were treated by US guided percutaneous injection of thrombin solution inside the pseudoaneurysm sac till contents became echogenic and flow inside the pseudoaneurysm stopped completely. One month further follow up in all these patients was done. Results The dose of thrombin injected varied from 200-1000 IU (mean 300 IU). Immediate thrombosis was seen in all the pseusdoaneurysms. Follow up at 24-48 hours showed complete thrombosis and regression of pseudoaneurysm in all the patients except one in whom a small residual sac was seen which thrombosed completely on second thrombin injection. Further follow up at one month showed regression of aneurysms in all the cases. No significant post procedural clinically significant complications were seen in any of the patients. Conclusion US guided percutaneous thrombin injection is a highly successful and safe procedure for the treatment of iatrogenic femoral pseudoaneurysms.

Digital papillary adenocarcinoma is a rare malignant tumor of the sweat glands, most commonly encountered in adult males. Only few pediatric cases have been reported, with an apparent predominance of females and none on the ankle. We report a case of a 15-year-old girl with a slowly growing, non-tender mass on the right ankle presenting with difficulty in walking; clinically believed to be a keloid. Excision of the mass revealed a glandular neoplasm with largely eccrine and focally apocrine differentiation. Histologic features do not reliably predict biologic behavior. Because of the existing confusion over the nomenclature, we propose that such tumors be called 'papillary adnexal neoplasms of distal extremities'.

Laparoscopy has become the gold standard technique for appendectomy and cholecystectomy. With the emergence of newer laparoscopic instruments which are roticulating and provide 7 degrees of freedom it is now possible to perform these operations through a single umbilical incision rather than the standard 3-4 incisions and thus lead to more desirable cosmetic results and less postoperative pain. The newer reticulating telescopes provide excellent exposure of the operating field and allow the operations to proceed routinely. Recently, ports [Triports (Olympus surgery)/SILS ports] especially designed for single incision laparoscopic surgery (SILS) have been developed. We herein describe our experience with laparoscopic single port appendectomies and cholecystectomies in children using the Triport. This is a retrospective cohort study of children who underwent single incision laparoscopic surgery between May 2009 and August 2010 at Texas Children's Hospital and Ben Taub General Hospital in Houston Texas by a single surgeon. Charts were reviewed for demographics, type of procedure, operative time, early or late complications, outcome and cosmetic results. Fifty-four patients underwent SILS. A total of 50 appendectomies (early or perforated) and 4 cholecystectomies were performed using this new minimally invasive approach. The average operative time for SILS/LESS appendectomy was 54 min with a range between 25 and 205 min, while operative time for SILS cholecystectomy was 156 min with a range of 75-196 min. Only small percentage (4%) of appendectomies (mostly complicated) were converted to standard laparoscopy, but none were converted to open procedure. All patients were followed up in the clinic after 3-4 weeks. No complications were noted and all patients had excellent cosmetic results. Parents were extremely satisfied with the cosmetic results. SILS/LESS is a safe, minimally invasive approach for appendectomy and cholecystectomy in children. This new approach is performed in an acceptable operative time with good outcomes and great cosmetic result.

Background Placing a ventriculo-peritoneal shunt in children with hydrocephalus is the standard of care. Many of these children will require revision of this portion of the shunt for a variety of reasons. Previously, it was thought that in a child with multiple previous ventriculo-peritoneal shunt (VPS) revisions, laparoscopy was contraindicated. This study aims to show that laparoscopy can be used safely and effectively in children with multiple previous ventriculo-peritoneal shunt surgeries. Materials and methods Laparoscopically assisted placement of the peritoneal portion of the ventriculo-peritoneal shunt in children with multiple previous VPS revisions was performed in 8 consecutive children (4 female) with ages ranging from 7 months to 18 years between May 2003 and September 2007. All eight children had undergone more than two previous VPS operations. All shunts were placed in areas free of adhesions and flow was observed under direct visualization. Results All of the procedures were successful; none needed conversion to the standard mini-laparotomy approach. No obvious or occult injury to the abdominal components was noted during hospitalization or during follow-up. Six of 8 patients required lysis of adhesions at the time of the revision. Average length of hospital stay was 2.6 days and no revisions of the abdominal portion of the VPS have been required by any of the 8 patients after laparoscopic revision. Previously unknown complications of shunt surgery were corrected in 1 of 8 children. Conclusions Laparoscopic placement of the peritoneal portion of a ventriculo-peritoneal shunt can be done safely and effectively in children with multiple previous VPS revisions due to improved visualization and placement of the shunt tip in a virgin area of the abdomen. Additionally, any known or unknown complications from previous VPS surgeries can be corrected with the laparoscopic approach. When combined with the reduction in pain, shorter hospital stay, and fewer immediate and future complications, this is the procedure of choice for patients requiring revision VPS surgeries in our hospital.

Gastrostomy tube placement may be performed in numerous ways: percutaneous endoscopic gastrostomy (PEG), laparoscopic PEG, and laparoscopic gastrostomy tube placement. In this paper, we propose a new technique of laparoendoscopic gastrostomy tube (LEGT) placement, using a three-suture technique to pexy the stomach to the abdominal wall. This paper outlines the LEGT technique and its benefits over previously reported techniques.Five children were included in this study during 2007. Their ages ranged from 15 months to 14 years. Under general anesthesia, a gastroscope was placed in the stomach and laparoscopic visualization was obtained through a 5-mm umbilical port. Two small incisions were made transabdominally for working instruments. Using laparoscopic visualization, three sutures were placed around a proposed gastrostomy site in the stomach. These sutures were pulled externally, using a GraNee needle (R-Med, Inc., Oregon, OH). Under visualization by a gastroscope, the gastrostomy button was placed in the center of these three sutures at the proposed gastrostomy tube site. Once the gastrostomy balloon was inflated, the three sutures were pulled taught and tied subcutaneously to pexy the stomach to the abdominal wall. Inspection with the gastroscope and laparoscope ensured proper gastrostomy tube placement.All patients had no complications intra- or postoperatively and at 2 months of follow-up. An optimal cosmetic result was obtained, and there was no need for suture removal or a second procedure.The laparoscopic visualization of the LEGT avoids accidental gastrocolic fistula formation and allows primary placement of the gastrostomy button without need for subsequent procedures. The endoscopic visualization, if the LEGT ensures the gastrostomy tube, is placed within the gastric lumen. Additionally, the three-suture technique gives an optimal fixation of the stomach to the abdominal wall, gives outstanding cosmetic results, avoids accidental disruption of sutures, and has no need for suture removal at a postoperative visit. LEGT thus serves as a one-stop shopping approach for gastrostomy in children.

Cervicothoracic neuroblastoma arising from the stellate ganglion in children has always been a challenge to the pediatric surgeon. Localized thoracic neuroblastoma in children has a very good prognosis if excised completely even without adjuvant therapy. Several approaches have been described to resect cervicothoracic neuroblastoma arising from the stellate ganglion with limited success. The muscle and bone sparing transmanubrial transcostal approach which spares the clavicle and the sternomastoid muscle provides excellent exposure for the complete excision of the tumor and excellent functional outcome. We report a 2-year-old girl with cervicothoracic neuroblastoma who had an excellent outcome with this approach.

Low flow vascular malformations of the orbit are uncommon, yet vision threatening lesions, that are difficult to manage surgically by virtue of their infiltrative morphology and tendency to recur. With the recent availability of advanced image guidance tools in the angiography suite, sclerotherapy has emerged as a minimally invasive treatment option for these lesions. A multi-disciplinary approach involving oculoplastic surgery, ophthalmology and interventional radiology is recommended for optimal management.

Fibroadenoma is a common condition in adolescent girls. Most tumors are excised either through a periareolar approach or the inframammary approach. Both approaches produce visible scars in adolescent girls. We propose a new cosmetic approach to this lesion and report our experience with the transaxillary subcutaneouscopic approach for excision of the fibroadenoma of the breast. The purpose of this case report is to delineate an innovative surgical approach to resection of a breast fibroadenoma that yields an adequate resection without possible damage to the ductal system while optimizing cosmetic results by avoiding scars.We retrospectively reviewed the medical records of four adolescent girls who underwent the above approach for excision of fibroadenoma of the breast. The age range was 14-16 years.There were no complications in all 4 patients. The final result at the 3-month follow-up revealed an esthetically pleasing skin incision that healed well and was hidden by the natural skin fold of the axilla.Transaxillary subcutaneouscopic excision of fibroadenoma of the breast in children is a safe and effective technique and should be considered for excision of benign breast lesions in children.