Cold Therapy for Pain Control in Pediatric Appendectomy Patients: A Randomized Controlled Trial.

PURPOSE: Topical ice has been shown to reduce pain scores and opioid use in adults with midline abdominal incisions. This study was designed to evaluate the efficacy of a cold therapy system in children following laparoscopic appendectomy. METHODS: Patients 7 years and older who underwent laparoscopic appendectomy at our institution from December 2021-September 2022 were eligible. Patients were randomized to standard pain therapy (control) or standard plus cold therapy (treatment) utilizing a modified ice machine system with cool abdominal pad postoperatively. Pain scores on the first 3 postoperative days (PODs), postoperative narcotic consumption, and patient satisfaction were analyzed. RESULTS: Fifty-eight patients were randomized, 29 to each group. Average survey response rate was 74% in control and 89% in treatment patients. There was no significant difference in median pain scores or narcotic use between groups. Cold therapy contributed to subjective pain improvement in 71%, 74%, and 50% of respondents on PODs 1, 2, and 3 respectively. CONCLUSION: A majority of patients reported cold therapy to be a helpful adjunct in pain control after appendectomy, though it did not reduce postoperative pain scores or narcotic use in our cohort - likely due to this population's naturally expedient recovery and low baseline narcotic requirement. TYPE OF STUDY: Randomized Controlled Trial. LEVEL OF EVIDENCE: Level I.

Metastatic pulmonary calcifications after pediatric liver transplantation.

BACKGROUND: Pulmonary calcification (PC) is a rare clinical entity observed following liver transplantation (LT). Most often identified in adults or in patients with concomitant renal failure, PC is rarely reported in children. While the clinical course of PC is largely benign, cases of progressive respiratory failure and death have been reported. Additionally, PC may mimic several other disease processes making diagnosis and management challenging. Currently, little is reported regarding the diagnosis, management, and long-term outcomes of children with PC following LT. METHODS: We performed a retrospective chart review of patients undergoing LT at our institution between 2006 and 2023. We identified two patients who developed PC following LT. Their diagnosis, clinical course, and long-term outcomes are reported. A literature review of the presentation, diagnosis, management, and outcomes of adult and pediatric patients with PC post-LT was also performed. CONCLUSIONS: Pulmonary calcifications are a rare but notable complication after pediatric liver transplantation. Our case series adds to the limited literature on this clinical entity in children but also highlights the fact that effective diagnosis and treatment may be safely accomplished without the use of lung biopsy.

Autologous and synthetic pediatric iliofemoral reconstruction: a novel technique for pediatric iliofemoral artery reconstruction.

Pediatric lower extremity arterial catheterization-related injuries can result in significant long-term morbidity. Revascularization is challenging due to concerns for long-term patency and growth accommodation with synthetic grafts. We describe a novel technique for iliofemoral revascularization using common iliac artery transposition and bridging polytetrafluoroethylene grafts. We treated two children who underwent femoral catheterization resulting in lifestyle-limiting claudication. Both patients experienced immediate resolution of symptoms. Postoperative imaging demonstrated widely patent vasculature. ASPIRE (autologous and synthetic pediatric iliofemoral reconstruction) is a method of pediatric iliofemoral artery revascularization that allows for an autologous artery to span the hip joint, reducing graft thrombosis risk and accommodating patient growth.

Immediate extubation after pediatric liver transplantation: Update on a single-center experience.

PURPOSE: Immediate extubation (IE) following liver transplantation (LT) is increasingly common in adult patients. This study reviews our center's experience with IE in children following LT to determine characteristics predictive of successful IE and its effects on post-operative outcomes. METHODS: We performed a retrospective chart review of all patients who underwent LT at our institution between January 2005 and November 2022. Patients with concomitant lung transplants and chronic ventilator requirements were excluded. RESULTS: Overall, 235 patients met study criteria. IE was achieved in 164 (69.8%) patients across all diagnoses and graft types. Of IE patients, only two required re-intubation within 3 days post-transplant. IE patients exhibited significantly shorter ICU (2 [1, 3 IQR] vs. 4 [2, 4 IQR] days, p < .001) and hospital lengths of stay (17 [12, 24 IQR] vs. 22 [14, 42 IQR] days, p = .001). Pre-transplant ICU requirement, high PELD/MELD score, intraoperative transfusion, cold ischemia time, and pressor requirements were risk factors against IE. There was no association between IE and recipient age or weight. The proportion of patients undergoing IE post-transplant increased significantly over time from 2005 to 2022 (p < .001), underscoring the role of clinical experience and transplant team learning curve. CONCLUSION: Spanning 18 years and 235 patients, we report the largest cohort of children undergoing IE following LT. Our findings support that IE is safe across ages and clinical scenarios. As our center gained experience, the rate of IE increased from 40% to 83%. These trends were associated with lower ICU and LOS, the benefits of which include earlier patient mobility and improved resource utilization.

Attitudes Toward Surgical Innovation Research in the Pediatric Surgery Fellowship Match.

INTRODUCTION: Pediatric surgery applicants are increasingly pursuing research in non-traditional fields including surgical innovation. This study aims to evaluate the relative value that pediatric surgeons involved in fellow selection place on innovation experience compared to traditional research. METHODS: A cross-sectional web-based survey of American Pediatric Surgical Association members involved in the selection of pediatric surgical fellows was conducted. Respondents reported their own innovation experience and were asked to identify valuable traits of applicants who completed an innovation fellowship. They rated the value of traditional research metrics including publications, presentations, and advanced degrees compared to patents and other innovation-related metrics. Comparisons were made between those with and without innovation experience with respect to gender, years in practice, and institutional role. RESULTS: One hundred thirty respondents were involved in pediatric surgery fellow selection. Innovation work was felt to be equal to or more valuable than basic science by 75% of respondents (84% vs. clinical/outcomes, 93% vs. other non-traditional, 72% vs. other clinical fellowships). Commonly cited concerns included "fewer publications" (21%) and "preoccupation with financial reward" (19%). The most valuable innovation-related metrics were "developing a novel surgical procedure" (67%) and "developing a novel device" (58%). When asked if the respondent would advise a junior resident to pursue an innovation fellowship, 49% would, 9% would not, and 43% were unsure. Seventeen percent expressed concern for match success. CONCLUSION: Innovation experience is generally viewed positively by pediatric surgeons involved in fellow selection. However, applicants and mentors would benefit from focusing on traditional academic outputs to ensure competitiveness. TYPE OF STUDY: Cross-sectional observational study. LEVEL OF EVIDENCE: III.

Outcomes of laparoscopic gastrostomy in children with and without the use of a modified T-fastener technique.

BACKGROUND: Laparoscopic gastrostomy is commonly performed for durable enteral access in children. T-fasteners have been used intraoperatively to achieve a secure gastropexy, traditionally using external bolsters. We compare the safety profile of a modified paired T-fastener technique to standard laparoscopic-assisted suture gastropexy. METHODS: A retrospective matched case-control study was performed of pediatric patients who underwent laparoscopic gastrostomy at a single center from 2015 to 2021. In the paired T-fastener group, pairs of T-fasteners were passed into the stomach in a square configuration, allowing the suture pairs to be tied subcutaneously. This cohort was matched in a 1:2 fashion with age, sex, and body mass index or weight-matched controls who underwent laparoscopic gastrostomy with buried transabdominal gastropexy. RESULTS: Thirty patients underwent laparoscopic gastrostomy using the paired T-fastener technique and were matched to 60 controls. There was no significant difference in median operative time or 30-day complication rates between the groups, but the paired T-fastener technique significantly reduced the number of trocars required, and it was used for patients with thicker abdominal walls. CONCLUSION: We demonstrate the modified paired T-fastener technique as a safe, efficient means of gastropexy in pediatric laparoscopic gastrostomy. The paired T-fastener approach eliminates external bolsters, reduces additional trocars, and may be advantageous for thicker abdominal walls while maintaining a similar complication profile to standard laparoscopic gastrostomy.

Pediatric button battery ingestion: A single center experience and risk score to predict severe outcomes.

PURPOSE: The purpose of this study was to analyze the management and outcomes of primary button battery ingestions and their sequelae at a single high-volume center, and to propose a risk score to predict the likelihood of a severe outcome. METHODS: The medical record was queried for all patients under 21 years old evaluated at our institution for button battery ingestion from 2008 to 2021. A severe outcome was defined as having at least one of the following: deep/circumferential mucosal erosion, perforation, mediastinitis, vascular or airway injury/fistula, or development of esophageal stricture. From a selection of clinically relevant factors, logistic regression determined predictors of a severe outcome, which were incorporated into a risk model. RESULTS: 143 patients evaluated for button battery ingestion were analyzed. 24 (17%) had a severe outcome. The independent predictors of a severe outcome in multivariate analysis were location of battery in the esophagus on imaging (96%), battery size >/ = 2 cm (95%), and presence of any symptoms on presentation (96%), with P < 0.001 in all cases. Predicted probability of a severe outcome ranged from 88% when all three risk factors were observed, to 0.3% when none were present. CONCLUSION: We report the presentation, management, and complication profiles of a large cohort of BB ingestions treated at a single institution. A risk score to predict severe outcomes may be used by providers initially evaluating patients with button battery ingestion in order to allocate resources and expedite transfer to a center with pediatric endoscopic and surgical capabilities. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Clinical Research Paper.

Staple aneurysmorrhaphy and suture venoplasty for repair of large bilateral external iliac vein aneurysms in an adolescent.

Aneurysms of the iliac veins are very rare; thus, the best approach to management has not yet been defined. We have presented the case of a 17-year-old boy with incidentally identified large bilateral external iliac vein aneurysms. Given the risks of potentially fatal thromboembolism or rupture, he underwent definitive repair of his aneurysms using staple aneurysmorrhaphy combined with additional vein tailoring by suture venoplasty, a technique not previously described for these aneurysms. We have also discussed the etiology, presentation, and our surgical technique to manage this rare condition.

Outcomes of internal biliary diversion using cholecystocolostomy for patients with severe Alagille syndrome.

Alagille syndrome (AGS) is a disorder that leads to increased serum cholesterol and bile acids, which can result in debilitating xanthomas and pruritus. External biliary drainage and transplantation are effective treatments for AGS. Internal biliary diversion with Roux-en-Y cholecystocolostomy has been described for other biliary conditions, but not AGS. Three patients with severe pruritus due to AGS underwent Roux-en-Y cholecystocolostomy for internal biliary drainage. Retrospective analysis compared preoperative and post-operative lab values and symptom scores (0, none-4, severe). Three patients underwent cholecystocolostomy. All patients had at least three diagnostic criteria for AGS. Mean preoperative pruritus score was 3.33 (range, 2-4) and mean post-operative score was 1. Mean preoperative xanthoma score was 1.33 (range, 0-4) and post-operative score was 1 at 2-month follow-up. Roux-en-Y cholecystocolostomy can be considered for AGS, which is refractory to medical management. This procedure accomplishes internal biliary diversion without significant physiologic derangements.

Functional side-to-side splenorenal shunts to treat extrahepatic portal vein thrombosis in children.

BACKGROUND: Surgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV). METHODS: Pediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated. RESULTS: Twelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was -11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures. CONCLUSIONS: Surgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.

Triple gallbladder with heterotopic gastric mucosa: a case report.

BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain. CONCLUSIONS: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy.

Trends in Surgical Patents Held by Surgeons From 1993 to 2018.

OBJECTIVE: This study aims to quantify the number of patent-holding surgeons and determine their specialty demographics. SUMMARY BACKGROUND DATA: The number of intellectual property filings related to surgery has exponentially increased over the past 40 years, yet surgeon inventor status among these inventions remains poorly defined. METHODS: A query of the United States Patent and Trademark Office (USPTO) Patent Full-Text and Image Database was performed over the years 1993 to 2018. Patents related to surgery were defined as surgical devices, implantables, dressings, introducers, and sterilization equipment based on Cooperative Patent Classification (CPC) code. Inventor names were cross-indexed with names of active Fellows in the American College of Surgeons (FACS) as of 2019. Surgeon inventors were identified and differences between specialty and sex were evaluated. RESULTS: A total of 275,260 patents related to surgery were issued over the study period. The number of surgical patents has increased by 462% from 4593 per year to 21,241 per year. A total of 9008 patents were held by a total of 2164 surgeons (4% of FACS). This represents 3.3% of all surgical patents with a mean of 5 patents (range 1-346) per patent-holding surgeon. Specialties with the largest number of patent holders include neurosurgery (9%) and orthopedic surgery (8%). Ninety-seven percent of patent-holding surgeons were male. CONCLUSIONS: 3.3% of patents related to surgery involve a surgeon inventor, and although the number of surgical patents has shown an exponential increase, surgeon involvement in these inventions has grown minimally. Surgical innovation training may offer an opportunity to reduce these discrepancies and increase surgeon involvement as patent holders.

Midaortic syndrome and renovascular hypertension.

Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta with or without the involvement of branch vessels. The majority of cases are thought to be idiopathic though MAS has been associated with a number of conditions including granulomatous vasculitis, neurofibromatosis-1 (NF-1), Alagille Syndrome, fibromuscular dysplasia (FMD), and Williams syndrome. Patients typically present with hypertension due to decreased renal perfusion. Less common presentations include renal insufficiency, heart failure, claudication, stroke, and abdominal pain. Imaging modalities help establish the diagnosis of MAS including duplex ultrasound, computed tomography angiography (CTA), magnetic resonance angiography (MRA), and angiography. Initial therapy focuses on medical management with antihypertensives prior to intervention. Invasive interventions are indicated when there is evidence of end organ damage or dysfunction such as decreased renal function, poorly growing kidneys, cerebrovascular accident, left ventricular hypertrophy or frank cardiac failure. Endovascular interventions may assist in diagnosis and may treat some lesions although reintervention rates are high. Most patients require some type of surgical intervention, and a variety of surgical options are available based on anatomic findings. Renal revascularization may be accomplished by renal artery bypass, autotransplantation, or renal artery reconstruction. Aortic lesions may be repaired using patch angioplasty or aortoaortic bypass. Mesenteric arteries do not typically require reconstruction as they are rarely symptomatic. More novel options include the use of tissue expanders to lengthen the aorta to allow for primary aortic reconstruction (TESLA) or the use of the meandering mesenteric artery as an autologous aortic bypass graft (MAGIC).

The Paired T-Fastener Technique: A Bolster-Free Gastropexy for Laparoscopic Gastrostomy Tube Placement.

Purpose: Gastropexy during laparoscopic gastrostomy tube (GT) insertion can be technically challenging. T-fasteners are an effective method of gastropexy. However, the use of external bolsters requires an additional procedure for removal and may cause skin complications due to pressure necrosis. We describe our experience utilizing T-fasteners in a novel way that eliminates external bolsters during laparoscopic GT placement. Methods: Pediatric patients requiring enteral access who underwent gastrostomy at a single institution using the paired T-fastener technique were reviewed. Gastropexy was achieved by passing pairs of T-fasteners, under laparoscopic and/or endoscopic guidance, through single stab incisions into the stomach in a square configuration, allowing the suture from one T-fastener to be tied subcutaneously to its paired suture. This eliminates the need for external bolsters. Operative time and 30-day postoperative complications, including local wound infection, granulation tissue formation, bleeding, and tube replacement, are reported. Results: Thirty patients underwent gastrostomy placement using the paired T-fastener technique. Mean age was 9.2 years (standard deviation [SD] 6.9) and mean weight 29.9 kg (SD 21.0). Mean tube length was 2.2 cm (SD 0.71). Eight patients underwent an additional procedure at the time of gastrostomy. Mean operative time was 74.4 minutes (SD 39.7). Five patients developed a local wound infection requiring antibiotics. Five developed granulation tissue. Seven patients underwent tube replacement within 30 days for dislodgment or stem upsize. Conclusion: The paired T-fastener technique is a safe and efficient method for primary button gastrostomy placement. This method eliminates the need for additional trocars or external bolsters and may be helpful in patients with thick abdominal walls.

Utilization of the Boston Children's Hospital SRTR Cohort Visualization Tool to increase team understanding of reporting cohorts.

BACKGROUND: While reviewing outcomes metrics and data from the SRTR, it became apparent that prospective assessment of the SRTR reporting cohorts would be an important proactive strategy for internal quality control. It was particularly important to identify the number of patient deaths and graft failures within 1 year of transplant that would result in being flagged by the UNOS and the MPSC. METHODS: A simple Microsoft Excel line graph was created to visually display retrospective, current, and future SRTR cohorts. Data provided by the SRTR CUSUM (https://securesrtr.transplant.hrsa.gov/srtr-reports/cusum-charts/) Reports and the SRTR 1 Year Expected Survival Excel Worksheet (https://securesrtr.transplant.hrsa.gov/srtr-reports/current-release/) were leveraged to identify whether programs were in jeopardy of being flagged by UNOS/MPSC for outcomes. RESULTS & CONCLUSIONS: The creation of this visual tool has greatly improved team understanding of SRTR report cohorts, as well as the risk of being flagged by regulatory agencies, for adverse outcomes.

Prenatal diagnosis of rapidly enlarging choledochal cyst with gastric outlet obstruction.

Choledochal cysts are congenital malformations of the biliary tract that involve aberrant configurations of the pancreaticobiliary ductal system. The pathology exists on a spectrum from fusiform dilation of the common bile duct to multiple dilations involving the intra- and extrahepatic bile ducts with potential risks of malignant transformation and hepatic fibrosis. Advancements in ultrasound technology have increased the incidence of prenatal diagnosis of choledochal cysts. Here, we present the case of a prenatally diagnosed initially asymptomatic Type I choledochal cyst with rapid progression in the neonatal period to a complete gastric outlet obstruction within the first month of life. We demonstrate the feasibility of cyst resection and reconstruction with Roux-en-Y hepaticojejunostomy in the neonatal age group. Finally, we discuss management of the case based on evolving imaging findings and laboratory evidence of impending liver dysfunction.

Surgical management of pediatric renovascular hypertension and midaortic syndrome at a single-center multidisciplinary program.

OBJECTIVE: To evaluate the outcomes of various surgical approaches in the treatment of renovascular hypertension and midaortic syndrome (MAS) in children. METHODS: We performed a retrospective medical record review of patients who had undergone surgery for renovascular hypertension from 2010 to 2018 at our center under the care of a multidisciplinary team. The operative interventions included mesenteric artery growth improves circulation (MAGIC), tissue expander-stimulated lengthening of arteries (TESLA), aortic bypass using polytetrafluorethylene, renal artery reimplantation, and autotransplantation. The MAGIC procedure uses the meandering mesenteric artery as a free conduit for aortic bypass. The TESLA procedure is based on lengthening the normal distal aorta and iliac arteries by gradual filling of a retroaortic tissue expander for several weeks, followed by resection of the stenotic aorta and subsequent primary reconstruction. RESULTS: A total of 39 patients were identified, 10 with isolated renal artery stenosis, 26 with MAS, and 3 with systemic inflammatory vasculitis. The median age at presentation and surgery was 6.4 years (range, 0-16.3 years) and 9.3 years (range, 0-9.2 years), respectively. The MAS-associated syndromes included neurofibromatosis type 1 (15.4%) and Williams syndrome (5.1%), although most cases were idiopathic. At surgery, 33.3% had had stage 1 hypertension (HTN), 53.8% stage 2 HTN, and 12.8% normal blood pressure with a median of three antihypertensive medications. Follow-up of 37 patients at a median of 2.5 years demonstrated normal blood pressure in 86.1%, stage 1 HTN in 8.3%, and stage 2 HTN in 5.6%, with a median of one antihypertensive medication for the entire cohort. CONCLUSIONS: The patterns of vascular involvement leading to renovascular hypertension in children are variable and complex, requiring thoughtful multidisciplinary planning and surgical decision-making. The MAGIC and TESLA procedures provide feasible approaches for aortic bypass and reconstruction using autologous tissues and will result in normalization of blood pressure in 85% of children 2.5 years after surgery.

The re-occurrence of cardiomyopathy in propionic acidemia after liver transplantation.

Cardiomyopathy is a frequent complication of propionic acidemia (PA). It is often fatal, and its occurrence is largely independent of classic metabolic treatment modalities. Liver transplantation (LT) is a treatment option for severe PA as the liver plays a vital role in metabolism of the precursors that accumulate in patients with PA. LT in PA is now considered to be a long-lasting and valid treatment to prevent cardiac disease. The subject of this report had severe cardiomyopathy that largely disappeared prior to undergoing a LT. Three years following the transplant, there was recurrence of cardiomyopathy following a surgery that was complicated with a postoperative aspiration pneumonia. On his last hospital admission, he was presented with pulmonary edema and heart failure. He continued with episodes of intractable hypotension, despite maximum inotropic and diuretic support. He died following redirection of care. We conclude that lethal cardiomyopathy may develop several years after successful LT in patients with PA.

Transient elastography assessment of liver allograft fibrosis in pediatric transplant recipients.

TE measures liver stiffness to assess fibrosis. Its use in post-transplant patients was reported in few small pediatric studies. We evaluated TE ability to predict liver graft fibrosis in a large cohort while comparing it to the performance of APRI and FIB-4. We also investigated the effect of graft type on LSMs. Patients at Boston Children's Hospital who underwent LT and LSM ≤ 1 year from biopsy (2007-2018) were eligible. Ninety-four patients (45%M) aged 1-21 years (89% < 18 years; 13% < 2 years) were eligible. Median time between transplant/biopsy and LSM was 5.1 years and 52 days, respectively. Thirty-nine percent received whole-liver grafts, 54% TV grafts, and 6% as part of MV. At LSM, median ALT was 25 [IQR 16-33] IU/L. Twenty-one percent had METAVIR ≥ F2. LSM was statistically higher among those with significant fibrosis (METAVIR ≥ F2) compared to those with METAVIR F0/F1 (median [IQR] 7.5 [4.6, 13.6] vs 5.1 [4.0, 6.4] kPa, respectively) (P = .005 by Wilcoxon rank-sum test). APRI and FIB-4 distributions were not different across METAVIR stages. The AUROC for LSM was 0.71 (95% CI 0.56-0.85) with an optimal cut-point of 6.5 kPa. Graft type had no influence on the AUROC for LSM. TE is useful for assessing significant graft fibrosis in children and young adult LT recipients and performs better than APRI and FIB-4. TV grafts demonstrate similar correlation with histology as whole-liver grafts.