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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. 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. 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. Transumbilical hernia repair is a safe and cosmetically appealing technique for umbilical hernia repair in children. Treatment study, level III. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background 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. Methods Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (> 30 days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age < 2, and weight < 10 kg. Univariate and multivariate analysis were performed. Results One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7 ± 6.3 years. Mean catheter life was similar in the laparoscopic and open groups (581 ± 539 days versus 574 ± 487 days, p = 0.938). The late complication rate was higher for open procedures (57% versus 37%, p = 0.013). Children age < 2 or weight < 10 kg 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. Discussion Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children < 2 years age or < 10 kg remain at risk for complications regardless of technique. [ABSTRACT FROM AUTHOR]

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 20 cm (mean, 9.1 cm); 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 6 years (7 months–17 years). 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. [ABSTRACT FROM AUTHOR]

Purpose: To report the results of a multicenter experience with the Viatorr expanded polytetrafluoroethylene-covered stent-graft for transjugular intrahepatic portosystemic shunt (TIPS) creation in which patency and clinical outcome were evaluated. Materials and Methods: One hundred consecutive patients with portal hypertension, with a mean age of 52 years (range, 22-86 years), underwent implantation of the Viatorr TIPS stent-graft at one of three hospital centers. The indications for TIPS creation were variceal bleeding (n = 81) and refractory ascites (n = 19). Twenty patients had Child-Pugh class A disease, 46 had class B disease, and 34 had class C disease. Eighty-seven patients underwent de novo TIPS placements, with 13 treated for recurrent TIPS stenosis. Sixty-two patients were available for follow-up portal venography and portosystemic pressure gradient (PSG) measurement commencing 6 months after Viatorr stent-graft placement. Results: The technical success rate was 100%. TIPS creation resulted in an immediate decrease in mean PSG (+/-SD) from 21 mm Hg +/- 6 to 7 mm Hg +/- 3. Acute repeat intervention (within 30 days) was required for portal vein thrombosis (n = 1), continued bleeding (n = 3), and encephalopathy (n = 1). The all-cause 30-day mortality rate was 12%. Two patients developed acute severe refractory encephalopathy, which led to death in one case. New or worsening encephalopathy was identified in 14% of patients. The incidence of recurrent bleeding was 8%. The cumulative survival rate at 1 year was 65%. Sixty-two patients available for venographic follow-up had a mean PSG of 9 mm Hg +/- 5 at a mean interval of 343 days (range, 56-967 days). There were four stent-graft occlusions (6%) and seven hemodynamically significant stenoses (11%), four within the stent-graft and three in the non-stent-implanted hepatic vein. The primary patency rate at 1 year by Kaplan-Meier analysis was 84%. Conclusions: This retrospective multicenter experience with the Viatorr stent-graft confirms the preliminary findings of other investigators of good technical results and improved patency compared with bare stents. Early mortality and symptomatic recurrence rates are low by historical standards. The theoretical increase in TIPS-related encephalopathy was not demonstrated. Longer-term follow-up will be required to determine whether the additional cost of the Viatorr stent-graft will be offset by reduced surveillance and repeat intervention. [ABSTRACT FROM AUTHOR]

Background Upper GI (UGI) studies are routinely ordered to screen for malrotation before routine placement of gastrostomy (G) tubes. However, the usefulness of this study is unknown. Methods A retrospective review of children with surgically placed G-tubes over a 2 year period (2011–2013) was performed. Patients with concomitant fundoplications were excluded. Results Three hundred ninety-three patients underwent G-tube placement. Of these, 299 patients (76%) had preoperative UGI, and 11 patients (3.7%) were identified with malrotation on UGI. Five (1.7%) patients underwent a Ladd’s procedure. The remaining 6 either had malrotation associated with gastroschisis (n = 5) or were lost to follow-up (n = 1). Children < 1 year did not have different rates of malrotation compared to older children (4.3% vs. 3.2%, p = 0.617). Likewise, children with neurologic impairment (NI) had similar rates of malrotation compared to neurologically normal (NN) children (2.6% vs. 3.8%, p = 0.692). The only significant difference in malrotation rate was between those with congenital gastrointestinal anomalies (24%) and those without (1.5%) (p < 0.001). Conclusion Preoperative screening UGI before routine G-tube placement led to an unexpected diagnosis of malrotation in only 1.7%. Given the added radiation risk associated with an UGI, our data suggest that an UGI is unnecessary prior to routine G-tube placement. A larger prospective study is warranted to validate these results. [ABSTRACT FROM AUTHOR]

Abstract: We report a case of iliopsoas abscess in a 13-days-old baby girl who presented with a diffuse, rapidly growing left flank, groin and upper thigh mass. Ultrasound scan (USS) and computed tomography (CT) were helpful in diagnosis. She was treated with extra-peritoneal surgical drainage as USS guided radiological intervention was unsuccessful. She was given systemic antibiotics. The clinical presentation, diagnosis and treatment of this rare condition with a brief review of the literature are presented in this report. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: To review the long term results and complications of children who underwent BAE in a single center. Materials and Methods: We present a retrospective review of patients who underwent BAE at a tertiary care children’s hospital over the last 13 years. After obtaining IRB approval, relevant data (demographics, indication, underlying disease, clinical presentation, preoperative imaging, bronchial artery anatomy, technique, results, complications and clinical outcome) was collected and analyzed. Results: 22 procedures were performed in 19 patients (12M, 7F), 9 months-21 years age. Indication for BAE was chronic intermittent hemoptysis (5-30 cc/day) in 54% and acute hemoptysis (200-500 cc) in 46%. Etiology of hemoptysis was: cystic fibrosis (90%), congenital heart disease (5%) and no apparent cause (5%). Most patients (95%) had a preoperative chest radiograph; 67% of which lateralized disease to one lung. Other preoperative imaging included CT chest (23%), CT angiogram (23%), cardiac catheterization (4%) and bronchoscopy (14%). The latter lateralized bleeding to one lung in 67%. A non-bronchial systemic supply was seen in 50% and spinal artery in 14%. A right intercosto-bronchial trunk was seen in 50%, common bronchial trunk in 41%, and bronchial artey aneurysm in 1. The most common embolic agent was PVA particles (64%) followed by embospheres (32%). Both PVA and embospheres were used in 1, gel foam in 2 and aneurysm coil embolization in 1. The procedure was uneventful in 68% with no major or minor complications. Chest pain requiring analgesia occurred after 9% of procedures. One patient each had the following complication: groin hematoma, pericardial effusion, transient pedal ischemia, mediastinal contrast extravasation and aortic intimal dissection. The median follow-up was 54 (1-142) months. 55% of procedures were successful with complete resolution of hemoptysis and no recurrence. Recurrent bleeding occurred after 41% with 3 of these patients requiring repeat BAE at 21, 32 and 39 months, respectively. Conclusion: BAE is effective in management of acute and chronic intermittent hemoptysis in children with low risk of major complications. Recurrence is frequent and can be managed with repeat embolization. [Copyright &y& Elsevier]

Abstract: While blunt abdominal trauma with associated liver injury is a common finding in pediatric trauma patients, hepatic artery transection with subsequent treatment by transarterial embolization has rarely been reported. We present a case of a child who suffered from a hepatic artery injury which was successfully managed by supraselective transarterial microcoil embolization, discuss management strategies in these patients, and provide a review of currently available literature. [Copyright &y& Elsevier]

Abstract: A 4-month-old female infant presented to our institute with recurrent pneumonia and was diagnosed with a right lower lobe esophageal bronchus. This congenital anomaly is extremely rare with very few cases reported in the literature. We describe the diagnostic workup and management that led to a successful outcome. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Background Microcystic lymphatic malformations (LM) are congenital birth defects that can cause severe functional or esthetic deformity. At this time, several treatment interventions are possible, but there is no ideal therapy. A recently published article noted a coincidental improvement in microcystic LMs with the use of sildenafil for pulmonary hypertension, but conclusive and reproducible data is lacking regarding its efficacy. Methods and results A prospective study was conducted to examine the subjective and objective results associated with sildenafil use in the treatment of microcystic LMs. Patients under the age of 18 were enrolled, and after evaluation with pre-intervention magnetic resonance imaging (MRI) studies, each was given a 6-week course of sildenafil. Subjective outcomes were obtained, and postoperative MRIs were used to objectively quantify changes. Five patients between the ages of 4 and 11 were enrolled in the study. All patients had microcystic LMs of the head and neck. All patients had undergone previous treatment interventions utilizing various modalities, and each was now seeking treatment for functional and cosmetic purposes. All patients had minimal to no response from a subjective standpoint. Comparison of pre- and post-treatment MRIs also demonstrated minimal to no response. Conclusions The use of sildenafil for the treatment of microcystic lymphatic malformations did not have any appreciable effect on our selected population of pediatric patients who had been resistant to previous therapies. Further studies would be necessary to determine if other cohorts may benefit. [ABSTRACT FROM AUTHOR]